Paediatrics notes Flashcards

Question

What is the most common congenital infection?

Answer 1

90% are normal at birth and develop normally 5% will have clinical featuers (e.g. hepatosplenomegaly, petechiae) at birth and most of these babies will have neurodevelopmental disabilities, such as sensorineural hearing loss, cerebral palsy, epilepsy and cognitive impairment 5% develop problems later in life mainly sensorineural hearing loss

Answer 2

Infection of the pregnant woman is usually asymptomatic | Pregnant women are not screened for CMV and there is no vaccine

Answer 3

IV ganciclovir OR Oral valganciclovir

Answer 4

Most infected infants are asymptomatic- at risk of developing chorioretinitis in adulthood 10% will have clinical features: retinopathy (due to acute fundal chorioretinitis), cerebral calcification, hydroecephalus --> these infants usually have long-term neurological disabilities

Answer 5

1st line: Pyrimethamine + sulfadiazine + calcium folinate | Adjunct: prednisolone

Answer 6

If the mother develops chicken pox in the first half of pregnancy (<20 weeks), there is a <2% risk of the foetus developing severe scarring of the skin and also ocular and neurological damage and digital dysplasia If the mother develops chicken pox within 5 days before or 2 days after delivery, when the foetus is unprotected by maternal antibodies and viral dose is high, about 25% will develop a vesicular rash and mortality can be as high as 30%

Answer 7

Exposed susceptible mothers can be protected with VZIG and treated with aciclovir

Answer 8

Characteristic rash on soles of feet and hand and bone lesions

Answer 9

Before birth, the blood vessels to and from the lungs are constricted, so most of the blood from the right side of the heart will pass through the ductus arteriosus into the aorta, and some will flow across the foramen ovale Shortly before and during labour, lung liquid production is reduced During descent through the birth canal, the infant's chest is squeezed and some lung fluid is drained Several thermal, tactile and hormonal stimuli (mainly an increase in catecholamine) to initiate breathing The first breath takes place an average of 6 seconds after delivery Lung expansion occurs due to intrathoracic negative pressure Regular breathing is usually established after 30 seconds After a the infant gasps, the rest of the lung fluid is absorbed into the lymphatic and pulmonary circulation Pulmonary expansion leads to reduced pulmonary resistance and an increase in pulmonary blood flow The flow of oxygenated blood through the ductus arteriosus leads to closure of the duct IMPORTANT: in babies born via an elective Caesarean section, their chest would not have been squeezed so it may take longer for the fluid to be drained from the lungs

Answer 10

Some infants will not breathe at birth - this may be due to asphyxia (lack of oxygen during labour or delivery) If a foetus is deprived of oxygen in utero, the foetus will attempt to breathe BUT, as they are still in utero, this attempt will be unsuccessful - this is called primary apnoea During the primary apnoea, heart rate is maintained If oxygen deprivation continues, primary apnoea is followed by irregular gasping and then a second period of apnoea (secondary or terminal apnoea) During terminal apnoea, the heart rate and blood pressure will fall If the infant is delivered after the terminal apnoea, they will need help with lung expansion (e.g. positive pressure ventilation or tracheal tube) The foetus rarely experiences continuous asphyxia except in the case of placental abruption or complete occlusion of umbilical blood flow in a cord prolapse More commonly, asphyxia during labour and delivery is intermittent (e.g. due to frequent uterine contractions)

Answer 11

Weight or abdominal circumference lies on a lower centile than that of the head

Answer 12

When the placenta fails to provide adequate nutrition late in the pregnancy but brain growth is relatively spared at the expense of liver glycogen and skin fat

Answer 13

Utero-placental dysfunction secondary to maternal pre-eclampsia, multiple pregnancy and maternal smoking These infants tend to put on weight rapidly after birth

Answer 14

Head circumference is equally reduced

Answer 15

Prolonged period of poor intrauterine growth starting in early pregnancy

Answer 16

Usually due to a small but normal foetus Could be due to a foetal chromosomal disorder, congenital infection, maternal drug/alcohol abuse or a chronic medical condition These infants are more likely to be permanently small

Answer 17

Intrauterine hypoxia and unexplained intrauterine death Asphyxia during labour and delivery After birth, growth restricted babies are liable to: Hypothermia (Large surface area) Hypoglycaemia (low fat and glycogen stores) Hypocalcaemia Polycythaemia

Answer 18

>90th centile for weight for their age Birth asphyxia from a difficult delivery Breathing difficulty from an enlarged tongue in Beckwith-Wiedemann syndrome (overgrowth syndrome) Hypoglycaemia (due to hyperinsulinism) Polycythaemia

Answer 19

Prevent haemorrhagic disease of the newborn

Answer 20

Breech presentation | 6x more common in females

Answer 21

Splinting in abduction reduced long-term morbidity

Answer 22

For infants <2 months with normal physical examination without instabililty, observation is recommended with serial examinations and US on monthly basis Hip abduction orthosis (splint) in Pavlik harness if the dysplasia persists or worsens (serial follow up and plain X-ray evaluation is recommended after 6 months of age) Breech delivery: if no DDH at neonatal examination, arrange ultrasound scan at 6 weeks

Answer 23

Some may suffer from intracranial haemorrhage which could leave them permanently disabled

Answer 24

Anticonvulsants impair synthesis of vitamin-K dependent clotting factors

Answer 25

PKU Hypothryoidism Haemoglobinopathies (sickle cell and thalassemia) CF (measuring serum immunoreactive trypsin, which is raised if there is pancreatic duct obstruction --> DNA analysis also performed to reduced the false-positive rate) MCAD deficiency

Answer 26

Evoked Otoacoustic emission: earphone palced over the ear and a sound is emitted which evoked an echo or emission from the ear if cochlear function is normal. IF a normal result is not achieved, move on to: Automated Auditory Brainstem Response Audiometry: A computer will analyse the EEG waveforms evoked in response to a series of clicks

Answer 27

In perinatal asphyxia, gas exchange, either placental or pulmonary, is compromised leading to cardiorespiratory depression. This leads to hypoxia, hypercapnia and metabolic acidosis. Reduced cardiac output leads to hypoxic-ischaemic injury to the brain and other organs.

Answer 28

Failure of gas exchange across placenta (excessive or prolonged uterine contractions, placental abruption, ruptures uterus) Interruption of umbilical blood flow (e.g. cord compression including shoulder dystocia, cord prolapse) Inadequate maternal placental perfusion Compromised foetus (anaemia, IUGR) Failure of cardiorespiratory adaptation at birth (failure to breathe)

Answer 29

Clinical manifestations of of HIE start up to 48 hours after asphyxia and can be graded: MILD= infant is irritable and responds excessively to stimulation, may have staring eyes, hyperventilation and impaired feeding MODERATE= infant shows marked abnormalities of tone and movement, cannot feed and may have seizures SEVERE= No spontaneous movements or response to pain, tone in limbs may fluctuate between hypotonia to hypertonia, seizures are often prolonged and refractor to treatment, multi-organ failure present NB: the neuronal injury in HIE may be immediate from primary neuronal death or may be delayed from reperfusion injury causing secondary neuronal death

Answer 30

With mild HIE, full recovery can be expected Even infants with moderate HIE, who have recovered fully on neurological examination and are feeding normally by 2 weeks of age have an excellent long-term prognosis Sever HIE has a mortality of 30-40%. Of the survivors, 80% have neurodevelopmental disabilities, particularly cerebral palsy. If MRI shows significant abnormalities at 4-14 days, there is a very high risk of later cerebral palsy Although HIE usually occurs antenatally or during delivery/labour, it can occur postnatally or be caused by a neonatal condition (e.g. kernicterus)

Answer 31

Both are potential injuries sustained during birth Caput Succedaneum - bruising and oedema of the presenting part extending beyond the margins of the skull bones (resolves within a few days) Cephalhaematoma - haematoma from bleeding below the periosteum, confined within the margins of the skull sutures. Usually involves the parietal bone (resolves over several weeks)

Answer 32

Oedema and bruising from Ventouse delivery

Answer 33

``` Extracranial haemorrhage Chignon Bruising Abrasion Forceps marks Subaponeurotic haemorrhage Nerve palsies (brachial nerve palsies result from traction to the brachial plexus nerve roots. This can occur during breech delivery or shoulder dystocia. Upper nerve root (C5+C6) injury results in Erb's palsy- can be accompanied by a phrenic nerve palsy which results in an elevated diaphragm. facial nerve palsies may occur from compression of the facial nerve against the mother's spine. Most palsies will resolve spontaneously) Fractures ```

Answer 34

Clavicle= usually from shoulder dystocia. A snap may be heard during delivery and the infant may show reduced arm movement on the affected side. A lump may be noticed due to callous formation. Prognosis is excellent- no specific treatment needed. Humerus/Femur= Humerus fractures tend to occur with shoulder dystocia. Femoral fractures more likely with breech deliveries. Infants may show some deformity, reduced movement of the limb and pain on movement. Fractures heal rapidly with immobilisation.

Answer 35

Hyaline membrane disease

Answer 36

Surfactant deficiency--> widespread alveolar collapse and inadequate gas exchange

Answer 37

Preterm baby | Maternal diabetes

Answer 38

Glucocorticoids given antenatally to mother if preterm delivery is anticipated In babies born with RDS, artifical surfactant can be instilled into the lungs directly or with a tracheal tube

Answer 39

Seen within 4 hours of birth Tachypnoea (>60breaths/minute) Laboured breathing with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring Expiratory grunting (attempting to create positive airway pressure during expiration and maintain functional residual capacity) Cyanosis if severe Diffuse granular or 'ground glass' appearance of lungs and air bronchogram on CXR. Indistinct heart border/obscured heart border with severe disease. In RDS, air from overly distended alveoli may track into the interstitium, resulting in pulmonary interstitial emphysema (PIE)

Answer 40

Oxygen and ventilation CPAP or artificial ventilation via a tracheal tube may be necessary Other options: mechanical ventilation, high-flow humidified oxygen therapy

Answer 41

In about 10% of infants ventilated for RDS, air will leak into the pleural cavity and cause a pneumothorax

Answer 42

Increased oxygen demands Breath sounds and chest expansion reduced on affected side A pneumothorax can be demonstrated by transillumination

Answer 43

Immediate decompression + oxygen therapy + chest drain if tension pneumothorax To prevent pneumothoraces, infants should be ventilated with the lowest pressures that provide adequate chest movement and blood gases

Answer 44

Can occur spontaneously or be secondary to: Meconium aspiration Respiratory distress syndrome Complication of ventilation

Answer 45

Very common in very low birthweight infants until 32 weeks' gestation. Can occur when an infant stops breathing over 20-30 seconds or when breathing continues against a closed glottis. Tend sot be caused by immaturity or central respiratory control and breathing will usually start again after gentle physical stimulation Caffeine may also help stimulate breathing CPAP may be needed if apnoeic episodes are frequent

Answer 46

``` Hypoglycaemia Infection Anaemia Hypoxia Electrolyte disturbances ```

Answer 47

Increased energy consumption --> hypoxia, hypoglycaemia --> failure to gain weight and increased mortality

Answer 48

Large SA:volume ratio Thin skin that is heat permeable (transepidermal water loss) Little subcutaneous fat for insulation Often nursed naked and cannot conserve heat by curling up or generate heat by shivering Preterm babies are kept in incubators to closely control the temperature and humidity

Answer 49

Infants with respiratory distress syndrome

Answer 50

Can be asymptomatic Can cause apnoea and bradycardia Increased oxygen requirement Difficulty in weaning infant from artificial ventilation Bounding pulse (due to increased pulse pressure) Prominent precordial impulse Systolic murmur Echocardiography can be used to assess infant's circulation

Answer 51

Duct can be closed using: IV indomethacin Prostacyclin synthetase inhibitor Ibuprofen If pharmacological methods are unsuccessful, surgical ligation or percutaneous catheter device closure may be used

Answer 52

35-36 weeks Less mature infants will need feeding via an orogastric or nasogastric tube

Answer 53

Phosphate | May need supplementation of protein, calories and calcium

Answer 54

Central venous catheter (e.g. PICC line) NB: PICC lines carry a significant risk of septicaemia (and other risks such as thrombosis) NB: cows' milk formula increases the risk of necrotising enterocolitis

Answer 55

Iron is usually transferred to the foetus during the last trimester Iron supplements are usually started at several weeks of age

Answer 56

IgG is mainly transferred across the placenta in the last trimester Additionally, infection in and around the cervix is often a reason for preterm labour Most infections in preterm infants occur after several days and are nosocomial (e.g. indwelling catheters, artificial ventilation)

Answer 57

Ultrasound scans

Answer 58

Usually occur in the germinal matrix above the caudate nucleus (contains a fragile network of blood vessels)

Answer 59

More common following perinatal asphyxia and in infants with severe RDS Pneumothorax is a major risk factor for brain haemorrhage

Answer 60

The MOST SEVERE type of haemorrhage is a unilateral haemorrhagic infarction involving the parenchyma of the brain - usually resulting in hemiplegia A large intraventricular haemorrhage may impair the drainage and reabsorption of CSF, leading to an accumulation of CSF This may resolve spontaneously or may progress to hydrocephalus Hydrocephalus may lead to separation of cranial sutures, a rapid increase in head circumference and the anterior fontanelle to become tense A ventriculoperitoneal shunt may be required, but symptomatic relief by relieving CSF via a lumbar puncture or ventricular tap may be sufficient Around 50% of infants with progressive post-haemorrhagic ventricular dilatation have cerebral palsy Periventricular white matter brain injury may occur following infarction or inflammation and may occur in the absence of haemorrhage This may resolve spontaneously However, if cystic lesions become visible on ultrasound 2-4 weeks later, there is definite loss of white matter The presence of multiple bilateral cysts is called periventricular leukomalacia (PVL) - it is associated with an 80-90% risk of spastic diplegia Definition of Spastic Diplegia: a form of cerebral palsy that manifests as especially high tightness or stiffness in the muscles of the lower extremities NOTE: PVL and intraventricular haemorrhage could both occur in the absence of clinical signs

Answer 61

Associated with bacterial infection of ischaemic bowel wall | Preterm infants fed with cows' milk formula are more likely to develop this condition

Answer 62

``` Infant stops tolerating feeds Milk is aspirated from the stomach Vomiting (may be bile-stained) Abdominal distension Rectal bleeding (sometimes) ``` The infant may develop shock and require artificial ventilation Characteristic X-ray features: Distended loops of bowel Thickening of the bowel wall with intramural gas Gas in the portal tract The disease may progress to bowel perforation, which can be detected by X-ray or transillumination of the abdomen

Answer 63

Stop oral feeding Broad-spectrum antibiotics to cover both aerobic and anaerobic organisms Surgery if bowel perforation/necrosis Parenteral nutrition is always needed and artificial ventilation and circulatory support are often needed

Answer 64

Long-term consequences include the development of strictures and malabsorption if extensive bowel resection is necessary

Answer 65

Affects developing blood vessels at the junction of the vascular and non-vascularised retina, usually due to uncontrolled use of high concentrations of oxygen

Answer 66

May progress to retinal detachment, fibrosis and blindness

Answer 67

Infants who still have oxygen requirement at a post-menstrual age of 36 weeks are described as having bronchopulmonary dysplasia (or chronic lung disease) Lung damage occurs as a result of artificial ventilation, oxygen toxicity and infection CXR may show widespread areas of opacification, sometimes with cystic changes Subsequent pertussis or RSV infection could lead to respiratory failure

Answer 68

Some infants will need prolonged ventilation, but most will be weaned onto CPAP and then additional ambient oxygen Corticosteroids may facilitate earlier weaning (although there are concerns about abnormal neurodevelopment)

Answer 69

Additional iron supplementation needs to be given until 6 months corrected age ``` Increased risk of: Poor growth Pneumonia/wehezing/asthma Bronchiolitis from RSV infection Bronchopulmonary dysplasia Gastro-oesophageal reflux Complex nutritional and GI disorders (e.g. following necrotising enterocolitis) Inguinal hernias ``` About 5-10% of very low birthweight babies develop cerebral palsy, but the most common impairments are learning difficulties

Answer 70

Over 50% of newborns become visibly jaundiced, due to: Marked physiological release of haemoglobin from the breakdown of red blood cells because of the high Hb concentration at birth Red cell life span of newborn infants (70 days) is considerably shorter than that of adults (120 days) Hepatic bilirubin metabolism is less efficient in the first few days of life

Answer 71

May be a sign of another disease (e.g. haemolytic anaemia) | Unconjugated bilirubin can get deposited in the brain, particularly in the basal ganglia, causing kernicterus

Answer 72

Encephalopathy resulting from the deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei It may occur when the level of unconjugated bilirubin exceeds the albumin-binding capacity of the blood- free bilirubin is fat-soluble, so it can cross the blood-brain barrier.

Answer 73

Lethargy Poor feeding ``` In severe cases: Irritability Increased muscle tone causing the baby to lie with an arched back (opisthotonos) Seizures Coma ```

Answer 74

Infants who survive may develop choreoathetoid cerebral palsy (due to damage to the basal ganglia), learning difficulties and sensorineural deafness

Answer 75

Around 80mcM/L

Answer 76

Usually due to haemolysis: Rhesus haemolytic disease: affected infants are usually identified antenatally. Severely affected infants will have anaemia, hydrops and hepatosplenomegaly with rapidly developing jaundice ABO incompatibility: Most ABO antibodies are IgM and do not cross the placenta. However, some O group women have an IgG anti-A-haemolysin in the blood, which can cross the placenta and haemolyse the red cells of a group A infant. Jaundice doesnt tend to be as bad as with rhesus disease and hepatosplenomegaly is absent. Coombs test positive. G6PD deficiency: Parents of affected infants should be given a list of drugs that they should avoid that may precipitate haemolysis Spherocytosis Congenital infection

Answer 77

Physiological jaundice Breastmilk jaundice: Unconjugated Dehydration: Poor milk intake. In some infants, IV fluids may be needed. Infection: a baby with an infection may develop unconjugated hyperbilirubinaemia from poor fluid intake, haemolysis, reduced hepatic function and an increase in enterohepatic circulaiton. Bruising and polycythaemia can exacerbate jaundice Criggler-Najjar syndrome (deficient or absent UGT)

Answer 78

Biliary atresia Neonatal hepatitis syndrome However, most cases are unconjugated: breast milk jaundice is most common Infection Congenital hypothydroidism

Answer 79

Tends to start on the head and face and spread down the trunk and limbs

Answer 80

It is recommended that in the UK all babies should be checked clinically for jaundice in the first 72 hours of life. If clinically jaundiced, a transcutaneous measurement should be made.

Answer 81

The rate at which bilirubin rises tends to be linear until it plateaus, so serial measurements can be plotted on a chart and used to anticipate the need for treatment Drugs that displace bilirubin from albumin (e.g. sulphonamides and diazepam) should be avoided in newborn infants

Answer 82

``` Visually inspect baby in natural light Measure bilirubin (use serum bilirubin if jaundice developed in the first 24 hours of life or if the gestational age if <35 weeks. Use transcutaneous bilirubinometer if >35 weeks or with jaundice that develops after first 24 hours- if result >250micromol/L, measure serum bilirubin) ``` Assess risk of developing kernicterus: increased risk if serum bilirubin >340 in babies >37 weeks gestation, rapidly rising bilirubin >8.5 micromol/L per hour, clinical features of acute bilirubin encephalopathy Serum bilirubin should be measured every 6 hours until it drops below the treatment threshold or becomes stable/falling

Answer 83

Measure haematocrit Blood group of mother and baby Coombs- if mother is Rh -ve, find out whether mother received prophylactic anti-D immunoglobulin during pregnancy Consider: FBC and blood film (e.g. looking for hereditary spherocytosis) Blood G6PD levels (consider ethnic origins) Microbiological cultures of blood, urine and/or CSF (if suspected infection)

Answer 84

Phototherapy 1st line (light from blue-green band of visible spectrum converts conjugated bilirubin into harmless water-soluble pigment that is excreted in the urine) --> monitor baby's temperature during phototherapy and protect the baby's eyes. Phototherapy can be stopped once serum bilirubin is >50micromol/L below threshold for treatment. Check for rebound hyperbilirubinaemia by measuring serum bilirubin 12-18 hours after stopping phototherapy Exchange transfusion IVIG

Answer 85

Transient tachypnoea of the newborn, caused by delay in resorption of lung liquid Usually settles within the first day of life More common in birth by C-section

Answer 86

Meconium may be passed in response to foetal hypoxia- asphyxiated infants may start gasping before delivery and aspirate the meconium. Meconium is a lung irritant and will cause both mechanical obstruction and chemical pneumonitis, and it predisposes to infection

Answer 87

Lungs become overinflated and have patches of collapse and consolidation. There is a high incidence of air leak, leading to pneumothorax and pneumomediastinum Artificial ventilation is often needed Infants may develop persistent pulmonary hypertension

Answer 88

If normal term infant with meconium-stained amniotic fluid but no history of GBS, observation is recommended If there are risk factors or laboratory findings that are suggestive of infection, consider antibiotics (IV ampicillin and gentamicin) Oxygen therapy and non-invasive ventilation (e.g. (CPAP) may be used in more severe cases)

Answer 89

Prolonged rupture of membranes Chorioamnionitis Low birthweight

Answer 90

Broad-spectrum antibiotics are started early until the results of the infection screen are available NB: milk aspiration can also cause respiratory symptoms in the newborn

Answer 91

Birth asphyxia Meconium aspiration Septicaemia RDS Can occur as a primary disorder LIFE THREATENING

Answer 92

High pulmonary vascular resistance leads to right-to-left shunting of blood Cyanosis occurs soon after birth CXR will show a normal sized heart but there may be some pulmonary oligaemia

Answer 93

Urgent echocardiogram to make sure that the infant does not have a congenital cardiac defect

Answer 94

Most infants will require mechanical ventilation and circulatory support Inhaled nitric oxide is a potent vasodilator and can be beneficial Sildenafil has also been introduced recently High-frequency (oscillatory) ventilation may be useful ECMO (heart and lung bypass for several days) may be used for severe but reversible causes

Answer 95

Many are diagnosed during antenatal ultrasound screening Diagnosis is confirmed by X-ray

Answer 96

Usually presents with failure to respond to resuscitation or respiratory distress In most cases, there is a left-sided herniation of abdominal contents through the posterolateral foramen of the diaphragm- this will cause the apex beat and heart sounds to be displaced to the right, with poor air entry in the left

Answer 97

Once diagnosed, a large NG tube is passed and suction applied to prevent distension of the intrathoracic bowel. Once stabilised, the hernia will be surgically repaired

Answer 98

In most infants, the main consequence is pulmonary hypoplasia- compression by herniated loops of bowel throughout pregnancy prevents lung development in foetus Hypoplastic lungs are associated with high mortality

Answer 99

Heart failure (Femoral arteries must be palpated in all infants with respiratory distress, as coarctation of the aorta and interrupted aortic arch are important causes of heart failure in newborn infants)

Answer 100

Bacteria will have either ascended from the birth canal or invaded the amniotic fluid The foetus becomes infected because the foetal lungs are in direct contact with the infected amniotic fluid These infants will develop pneumonia and secondary septicaemia/bacteraemia On the other hand, with congenital viral infections and early-onset infection with Listeria moncytogenes, foetal infection is acquired via the placenta following maternal infection

Answer 101

Prolonged or premature rupture of membranes when chorioamnionitis is clinically evident (e.g. mother having fever during delivery)

Answer 102

``` Respiratory distress Apnoea Temperature instability Vomiting Poor feeding Abdominal distension Jaundice Neutropenia Shock Irritability Seizures ```

Answer 103

CXR Septic screen FBC to check for neutropenia Blood cultures NB: Acute phase proteins can take 12-24 hours to rise, so a single normal result doesnt rule out infection

Answer 104

Antibiotics are started immediately without waiting for culture results IV antibiotics should cover: Group B Strep Listeria monocytogenes Other Gram +ve organisms (usually with benzylpenicillin or amoxicillin) Gram -ve organisms (usually with an aminoglycoside such as gentamicin) If CRPs are negative and the infants appears to have recovered initially, antibiotics can be stopped after 48 hours If the blood culture is positive or there are any neurological signs, CSF must be examined and cultured

Answer 105

Often the infant's environment Common in NICU, due to sources of infection such as indwelling catheters, invasive procedures and tracheal tubes Most common pathogen is coagulase negative staphylococcus (Staph epidermidis)

Answer 106

Ampicillin + gentamicin/cefotaxime Flucloxacillin + gentamicin If resistant, specific antibiotics (e.g. vancomycin for coagulase-negative staphylococci or enterococci) or broad-spectrum antibiotics (e.g. meropenem) may be needed NB: The use of prolonged or broad-spectrum antibiotics predisposes to invasive fungal infections (e.g. candidiasis)

Answer 107

Serial measurements of CRP to measure response to therapy

Answer 108

Late signs: Bulging fontanelle Hyperextension of the neck and back (opisthotonos)

Answer 109

Ampicillin or penicillin and a 3rd generation cephalosporin (eg. cefotaxime)

Answer 110

Cerebral abscess

Answer 111

1) Supplemental oxygen 2) Gain IV or IO access and order blood cultures, blood glucose and arterial/venous/capillary gases 3) IV/IO broad-spectrum antibiotics 4) IV fluids (be cautious of fluid overload) 5) Experienced senior clinicians should be involved early 6) Vasoactive inotropic support (e.g. adrenaline) should be considered early- considered if normal parameters are not achieved after >40ml/kg of fluid resus

Answer 112

Respiratory distress Pneumonia May also cause septicaemia and meningitis

Answer 113

``` Preterm labour Prolonged rupture of membranes Maternal fever during labour Maternal chorioamnionitis Previously infected infant ``` Prophylactic intrapartum antibiotics given IV can prevent this infection

Answer 114

Targeted antibiotic therapy (Benzypenicillin or ampicilolin + gentamicin or cefotaxime or ceftriaxone) Supportive therapy

Answer 115

Transmitted to the mother through food (e.g. unpasteurised milk, soft cheeses, undercooked poultry)

Answer 116

mild influenza-like illness in mother Can pass to the foetus throuhg the placenta ``` Meconium staining of the amniotic fluid (unusual in preterm infants) Widespread rash Septicaemia Pneumonia Meningitis ```

Answer 117

Spontaneous abortion Preterm delivery Foetal/neonatal sepsis

Answer 118

Amoxicillin or co-trimoxazole (trimethoprim is contraindicated in pregnancy) If systemic infection: IV ampicillin or benzylpenicillin with gentamicin

Answer 119

Cleaning with saline and water is all that is required- will resolve spontaneously Discharge and redness is suggestive of staph or strep infection and can be treated with a topical ointment (e.g. neomycin) Gonococcal infection may cause purulent discharge, conjunctival infection and swelling of the eyelids. Discharge should be Gram-stained and cultured. Treatment (usually a 3rd generation cephalosporin) should be started immediately because loss of vision could occur Chlamydia infection presents with a purulent discharge and swelling of the eyelids. Treated with oral erythromycin for 2 weeks. Bacterial conjunctivitis treated with opthalmic azithromycin OR erythromycin OR polymyxin

Answer 120

Systemic antibiotics if skin around umbilicus becomes inflamed Sometimes, the umbilicus can be prevented from involuting bi an umbilical granuloma, which can be removed by applying silver nitrate or by applying a ligature around the stump

Answer 121

During passage through an infected birth canal or occasionally by ascending infection Often the mother does not know that she is infected More common in preterm infants

Answer 122

Infants present up to 4 weeks of age with: Localised herpetic lesions on the skin or eye Or encephalitis Or disseminated disease

Answer 123

If the mother is identified as having primary disease or delivery genital herpetic lesions at the time of devliery, C-section is indicated Aciclovir or valaciclovir can be given prophylactically to the baby during the at-risk period

Answer 124

Infants of mothers who are HbsAg positive should receive the hepatitis V vaccination shortly after birth to prevent vertical infection Babies are at highest risk of becoming chronic carriers when their mothers are e-antigen positive but have no e-antibodies

Answer 125

Mothers should receive antiviral monotherapy (Tenofovir disoproxil OR lamivudine) Babies at risk should receive passive immunisation with hepatitis B immunoglobulin AND hepatitis B vaccine

Answer 126

``` Particularly likely within the first 24 hours of babies with: IUGR Preterm Born to mothers with diabetes (infants undergo hyperplasia of the islet cells in the pancreas causing high insulin levels) Large-for-dates Hypothermia Polycythaemia Ill ```

Answer 127

``` Jitteriness Irritability Apnoea Lethargy Drowsiness Seizures ``` During the first 24 hours, many asymptomatic babies will have blood glucose below 2.6mmol/L (optimal level for desirable neurodevelopmental outcomes) Prolonged, symptomatic hypoglycaemia can cause permanent neurological disability

Answer 128

Can be prevented with early and frequent milk feeding Glucose can be given IV in refractory or severe hypoglycaemia Glucagon and hydrocortisone can also be given

Answer 129

Seizures are unstimulated and usually appear as repetitive, rhythmic movement of the limbs which persist despite restraint and are often accompanied by eye movements or changes in respiration

Answer 130

``` HIE Cerebral infarction Septicaemia/meningitis Hypoglycaemia Hypo/hypernatraemia Hypocalcaemia Hypomagnesaemia Inborn errors of metabolism Pyridoxine dependency ``` Whenever seizures are observed, hypoglycaemia and meningitis need to be excluded or treated urgently

Answer 131

A cerebral ultrasound can be performed to identify haemorrhage or cerebral malformations Ongoing or repeated seizures will be treated with anticonvulsants

Answer 132

Infarction in the territory of the middle cerebral artery

Answer 133

May present with seizures Seizures can be focal or generalised There are no other abnormal clinical features (unlike HIE)

Answer 134

Thrombotic (e.g. thromboembolism from placental vessles or inherited thrombophilia) Prognosis is relatively good (only 20% have hemiparesis or epilepsy)

Answer 135

May be unilateral or bilateral Cleft lip results from failure of fusion of the frontonasal and maxillary processes In bilateral cases, the pre-maxilla becomes anteverted Cleft palate result from failure of fusion of the palatine processes and the nasal septum These defects can occur as a part of a syndrome (e.g. chromosomal disorders) They are associated with maternal anticonvulsant therapy

Answer 136

Infants are prone to acute otitis media

Answer 137

Specialised feeding advice may be necessary Watch out for airway problems (e.g. Pierre-Robin sequence) Pre-surgical lip tapping, oral appliances or pre-surgical nasal alveolar moulding (PNAM) may be needed to narrow the cleft Definitive repair is by surgery

Answer 138

Associated with micrognathia (small jaw), posterior displacement of the tongue (glossoptosis) and midline cleft of the soft palate

Answer 139

Can lead to difficulty feeding As the tongue falls back, there is obstruction to the upper airways which may cause cyanotic episodes Infant is at risk of failure to thrive Due to the risk of airway obstruction, infants may need to lie on their front Eventually, as the mandible grows, these problems resolve

Answer 140

Usually associated with a trans-oesophageal fistula Associated with polydramnios during pregnancy Almost half of babies with oesophageal atresia will have other congenital malformations (e.g. VACTERL: verterbral, anorectal, cariac, trachea-oEsophageal, renal and radial limb)

Answer 141

If not diagnosed at birth, it typically presents with persistent salivaiton and drooling The infant will cough and choke when fed and may have cyanotic episodes They may aspirate saliva or milk into the lungs from the upper airways, and acid from the stomach

Answer 142

Passing a wide-calibre feeding tube through the mouth and checking, using an X-ray, to see if it reaches the stomach

Answer 143

``` Persistent vomiting (may be bile-stianed, unless obstruction is procximal to the ampulla of Vater: Abdominal distension is more prominent. High lesions will present soon after birth, whereas lower lesions may take a few days) ```

Answer 144

Atresia or stenosis of the duodenum (1/3 have Down syndrome) Atresia or stenosis of the jejunum or ileum Malrotation with volvulus (could lead to infarction of the entire midgut) Meconium ileus (almlost all affected neonates have CF) Meconium plug (a plug of congealed meconium causes lower intestinal obstruction)

Answer 145

Based on clinical features and abdominal X-ray

Answer 146

Atresia or stenosis is treated surgically A meconium plug will usually pass spontaneously A meconium ileus can be dislodged using Gastrograffin

Answer 147

Hirschsprung disease - absence of the myenteric nerve plexus in the rectum which may extend along the colon Rectal atresia - absence of the anus at the normal site

Answer 148

Often diagnosed antenatally Abdominal contents protrude through the umbilical ring and are covered by a transparent sac (formed by the amniotic membrane and peritoneum) Often associated with other major congenital abnormalities

Answer 149

Bowel protrudes through a defect in the anterior abdominal wall adjacent to the umbilicus and there is no covering sac It is not associated with any other congenital abnormalities Carries a much greater risk of dehydration and protein loss, so the abdomen of affected infants should be wrapped in several layers of clingfilm An NG tube is passed and asiprated frequently and an IV dextrose infusion is set up Colloid support is often required to replace lost protein Can be corrected with surgery

Answer 150

Posseting refers to small amounts of milk that often accompay the return to swallowed air (burp) Regurgitation describes larger, more frequent losses, and may indicate the presence of more significant gastro-oesophageal reflux Vomiting is the forceful ejection of gastric contents

Answer 151

Bile stained vomit= intestinal obstruction Haematemesis= oesophagitis, peptic ulceration, oral/nasal bleeding, oesophageal variceal bleeding Projectile vomiting in the first few weeks of life= pyloric stenosis Vomiting at the end of paroxysmal coughing = whooping cough Abdominal tenderness/abdominal pain on movement = surgical abdomen Abdominal distension= intestinal obstruction, including stragulated inguinal hernia Hepatosplenomegaly= chronic liver disease, inborn error of metabolism Blood in stool = intussusception, bacterial gastroenteritis Severe dehydration, shock = severe gastroenteritis, systemic infection (UTI, meningitis), DKA Bulging fontanelle or seizures = raised ICP Faltering growth = GOR, coeliac, other chronic GI conditions

Answer 152

Functional immaturity of lower oesophageal sphincter leading to inappropriate relaxation

Answer 153

Predominantly fluid diet Mainly horizontal posture Short intra-abdominal length of oesophagus

Answer 154

When it starts causing significant problems

Answer 155

Cerebral palsy and other neurodevelopmental disorders Preterm infants (especially those with bronchopulmonary dysplasia) Following surgery for oesophageal atresia or diaphragmatic hernia

Answer 156

``` Faltering growth from severe vomiting Oesophagitis Recurrent pulmonary aspiration (recurrent pneumonia, cough, wheeze) Dystonic neck posturing Apparent life-threatening events ```

Answer 157

24 hour oesophageal pH monitoring 24 hour impedance monitoring Endoscopy with oesophageal biopsies Upper GI contrast study Diagnosis usually clinical and no investigations are required. However, the investigations above may be used.

Answer 158

Referral SAME DAY referral if haematemesis, melaena or dysphagia present Arrange assessment by paediatrician if: Red flag symptoms Faltering growth Unexplained distress Unresponsive to medical therapy Feeding aversion Unexplained iron deficiency anaemia No improvement after 1 year of age Suspected Sandifer's syndrome Refer if there are complications: Recurrent aspiration pneumonia Unexplained apnoeas Unexplained epileptic seizure-like events Unexplained upper airway inflammation Dental erosion with neurodisability Recurrent acute otitis media Treatment Reassure It is very common Begins early (< 8 weeks) and may be frequent It usually becomes less frequent with time Treatment and investigation is not usually needed Review infant or child if: Projectile regurgitation Bile-stained vomit or haematemesis New concerns (e.g. faltering growth, feeding difficulties) Persistent, frequent regurgitation beyond the first year of life

Answer 159

Caused by hypertrophy of the pyloric muscle causing a gastric outlet obstruction

Answer 160

Presents at 2-8 weeks of age, irrespective of gestational age 4x more common in boys Vomiting (increases in frequency and forcefulness over time, ultimately becoming projectile) Hunger after vomiting (until dehydration leads to loss of interest in feeding) Weight loss if presentation is delayed Hypochloraemic metabolic alkalosis with low plasma sodium and potassium secondary to vomiting stomach contents

Answer 161

``` Gastric peristalsis may be seen as a wave moving from left to right across the abdomen Pyloric mass (feels like an olive) is usually palpable in the RUQ Ultrasound may be useful ``` NB: if the stomach is distended with air, it will need to be emptied using an NG tube to allow palpation

Answer 162

IV fluid resuscitation: This is essential to correct the fluid and electrolyte disturbance before surgery This should be provided at 1.5x maintenance rate with 5% dextrose and 0.45% saline Definitive treatment is by performing a Ramstedt pyloromyotomy (dividing the hypertrophied muscle down to but not including the mucosa- can be open or laparoscopic)

Answer 163

``` Infection (UTI, middle-ear, meningeal) Pan from an unrecognised fracture Oesophagitis Testicular torsion Severe nappy rash Constipation Teething ```

Answer 164

Paroxysmal, inconsolable crying or screaming often accompanied by drawing up the knees and passage or excessive flatus takes place several times a day NB: if severe and persistent, it may be due to a cows' milk protein allergy

Answer 165

Reassure parents that colic is a common problem that should resolve by 6 months of age Sources of information/support: NHS choices leaflet, health visitor (help with feeding techniques etc...) Strategies to soothe a crying infant: holidng the baby, gentle motion, white noise Encourage parents to look after themselves: get support form family and friends, meet other parents at a similar state (NCT), resting, putting the baby in a safe place to give yourself a time out Insufficient evidence for Infacol and Colief, so do not recommend

Answer 166

Testes Hernial orifices Hip joints

Answer 167

Acute appendicitis (although it is v uncommon <3 years)

Answer 168

Anorexia Vomiting Abdominal pain (initially central and colicky (appendicular midgut colic), but then localising to right iliac fossa (from localised peritoneal inflammation))

Answer 169

Fever Abdominal pain aggravated by movement Persistent tenderness wit guarding in the RIF (McBurney's point tenderness)- NB: with a retrocaecal appendix, localised guarding may be absent Perforation can occur rapidly in children because their omentum is less well developed and fails to surround the appendix

Answer 170

No laboratory investigation or imaging is consistently helpful in making a diagnosis Neutrophilia is not always present on the blood count WCC and organisms can be seen in the urine because the inflamed appendix may be adjacent to the ureter or the bladder Ultrasound may support the clinical diagnosis and show complications (e.g. abscess, perforation, appendix mass)

Answer 171

``` SURGICAL EMERGENCY Patient should be nil-by-mouth from the time of diagnosis IV fluids should be started Requires immediate hospital admission Treatment of choice is an appendicectomy ```

Answer 172

Refers to abdominal pain that resolves within 24-48 hours The pain is less severe than acute appendicitis Often accompanied by an URTI with cervical lymphadenopathy In some of these children, abdominal signs do not resolve and an appendicectomy is performed Mesenteric adenitis is often diagnosed in these children as enlarged mesenteric lymph nodes are seen at laparoscopy with a normal appendix

Answer 173

Invagination of proximal bowel into a distal segment | Most commonly involves ileum passing into caecum through the ileocaecal valve

Answer 174

3 months - 2 years It is the most common cause of intestinal obstruction in infants after the neonatal period

Answer 175

Stretching and constriction of the mesentery resulting in venous obstruction, causing engorgement and bleeding from the bowel mucosa, fluid loss, and, eventually, bowel perforation, peritonitis and gut necrosis Requires prompt diagnosis, immediate fluid resuscitation and urgent reduction of the intussusception

Answer 176

Paroxysmal, severe colicky pain with pallor (during episodes, the child will become pale, especially around the mouth, and draw their legs up. There is recovery in between episodes, but the child may become lethargic) Refusing feeds Vomiting (may be bile-stained depending on the location of the intussusception) Sausage-shaped mass is palpable in the abdomen Redcurrant jelly stool is a late sign Abdominal distension and shock (pooling of fluid in the gut, so IV fluid resuscitation is often necessary) Viral infection leading to enlargement of Peyer's patches may form the lead point of the intussusception Other possible lead points include a Meckel's diverticulum (more common >2 years)

Answer 177

X-ray may show distended bowel with no gas in the distal colon and rectum Abdominal ultrasound is useful to confirm the diagnosis and check the response to treatment (target sign)

Answer 178

Unless there are signs of peritonitis, reduction by rectal air insufflation, with fluoroscopy guidance, by radiologist (successful 75% of the time- other 25 may need operation) Clinically stable with no contraindications (peritonitis, perforation, hypovolaemic shock) to contrast enema reduction Fluid resuscitation Contrast enema (air or contrast liquid) Broad-spectrum antibiotics (Clindamycin + gentamicin OR tazocin OR cefoxitin + vancomycin) 2nd line: surgical reduction with broad-spectrum antibiotics If recurrent intussusception, consider investigating for a pathological lead point (e.g. Meckel's diverticulum)

Answer 179

Most cases are asymptomatic May present with severe rectal bleeding (neither bright red nor true melaena), causing an acute drop in Hb Intussusception Volvulus Meckel's diverticulitis (mimics appendicitis)

Answer 180

Technetium scan will demonstrate increased uptake by ectopic gastric mucosa in 70% of cases

Answer 181

Asymptomatic= incidental imaging finding- no treatment required. Detected during surgery for other reasons- prophylactic excision Symptomatic= Bleeding - excision of diverticulum with blood trasnfusion (if haemodynamically stable) Obstruction- excision of diverticulum and lysis of adhesions Perforation/peritonitis- excision of diverticulum or small bowel segmental resection with perioperative antibiotics

Answer 182

During rotation of the small bowel in foetal life, if the mesentery is not fixed at the duodenojejunal flexure or in the ileocaecal region, its base is shorter than normal and is predisposed to volvulus

Answer 183

Peritoneal bands that may cross the duodenum (often anteriorly)

Answer 184

Obstruction Obstruction with compromised blood supply Obstruction with bilious vomiting is the usual presentation within the first few days of life, but it can occur later If there are signs of vascular compromise, an urgent laparotomy is needed

Answer 185

Urgent upper GI contrast study to assess intestinal rotation

Answer 186

Ladd procedure- detorting the bowel and surgical dividing the Ladd bands (the bowel is placed in the non-rotated position with the duodenojejunal flexure on the right and the caecum and appendix on the left- appendix is usually removed to avoid diagnostic confusion in case the child presents again with an acute abdomen) Antibiotics (cefazolin)

Answer 187

Pain sufficient to interrupt normal activities and lasts for at least 3 months

Answer 188

Pain is typically periumbilical and children are generally well Constipation is a frequent cause of recurrent abdominal pain and should be excluded May occur as a manifestation of stress Vicious cycle of anxiety - parents demanding more invasive investigations etc. could worsen the perceived symptoms

Answer 189

Aim to identify any serious cause without subjecting the child to unnecessary investigations Examination includes inspection of the perineum for anal fissures and checking the child's growth Urine MC+S is important as UTIs may cause pain without other signs Abdominal US may be useful to exclude gallstones and pelvic ureteric junction obstruction Coeliac antibodies and TFTs should be checked (although these are rare causes of recurrent abdominal pain) IBS and functional dyspepsia are diagnoses of exclusion and should be explained simply to patients (e.g. "sometimes the insides of the intestines become so sensitive that some children can feel the food going around the bends")

Answer 190

Around 50% rapidly become free of symptoms IN around 25%, symptoms take months to resolve In around 35%, symptoms continue or return in adulthood as migraine, IBS or functional dyspepsia

Answer 191

Often associated with abdominal pain and headaches The pain is typically in the midline and is associated with vomiting and facial pallor There is often a personal or family history of migraine The history tends to describe long periods of no symptoms followed by a shorter period of non-specific abdominal pain and pallor, with or without vomiting Treatment with anti-migraine medication could help

Answer 192

Intra-abdominal infection

Answer 193

The disease has a significant psycho-social component (stress and anxiety) There is often a positive family history Some people with IBS will also have coeliac disease, so coeliac antibodies must always be tested

Answer 194

Non-specific abdominal pain (often periumbilical and relieved by defecation) Explosive, loose or mucousy stool Bloating Feeling of incomplete defecation Constipation (often alternating with loose or normal stools)

Answer 195

Reassure Encourage patient to identify sources of stress or anxiety in their lifestyle and any foods that may aggravate symptoms Recommend adequate fluid intake

Answer 196

It wakes them up at night Pain radiates to the back History of peptic ulceration in a first-degree relative

Answer 197

H. pylori causes nodular antral gastritis, which may be associated with abdominal pain and nausea It can be identified with gastric antral biopsies

Answer 198

Gastric antral biopsies (if suspected nodular antral gastritis) CLO test C-13 breath test (H.pylori produces urease) Stool antigen

Answer 199

In children with suspected peptic ulceration, it should be treated with proton-pump inhibitors If investigations suggest the presence of an H.pylori infection, eradication therapy should be given (amoxicillin AND metronidazole OR clarithromycin) If they fail to respond to treatment, an upper GI endoscopy should be performed (if this is normal --> functional dyspepsia is diagnosed- a variant of IBS)

Answer 200

As well as symptoms of peptic ulceration, children with functional dyspepsia will have other non-specific symptoms such as: Early satiety Bloating Post-prandial vomiting Delayed gastric emptying (due to gastric dysmotility)

Answer 201

An inflammatory condition affecting the oesophagus caused by activation of eosinophils within the mucosa and submucosa

Answer 202

Vomiting Discomfort when swallowing or bolus dysphagia (when food gets stuck) Asthma Eczema Hayfever

Answer 203

Endoscopy: Linear furrows and trachealisation of the oesophagus may be seen (looks like the trachea with rings protruding into th elumen) Microscopically, eosinophilic infiltration will be identified

Answer 204

Oral corticosteroids (fluticasone or viscous budesonide)

Answer 205

Rotavirus- particularly prevalent in winter and early spring Bacterial causes are less common in developed countries but may be suggested by presence of blood in stools Campylobacter jejuni is the most common cause of bacterial gastroenteritis in developed countries, and is often associated with severe abdominal pain

Answer 206

Shigella and some salmonellae produce a dysenteric type of infection, with blood and pus in the stool, pain and tenesmus Shigella infection may be accompanied by a high fever

Answer 207

Associated with profuse, rapidly dehydrating diarrhoea

Answer 208

Dehydration leading to shock

Answer 209

Infants (especially low birthweight): great SA:volume ratio, higher basal fluid requirements, immature renal tubular reabsorption If >/=6 diarrhoeal stools in the past 24 hours If vomited >/=3 times in past 24 hours If unable to tolerate extra fluids If they have malnutrition

Answer 210

Assess for features of dehydration and shock, features that may suggest an alternative diagnosis, and severity and possible cause of infection Consider hospital admission Give rehydration advice: Maintenance volumes: 0-10kg= 100ml/kg 10-20kg= 1000ml + 50ml/kg for each kg over 10 20+ kg= 1500ml + 20ml/kg for each kg over 20kg Modes of rehydration: <5 years= 50ml/kg for fluid deficit replacement over 4 hours as well as maintenance with oral rehydration solution 5+ years= 200ml ORS after each loose stool Perform stool sample analysis: if this reveals causative organism, seek specialist advice regarding antibiotic treatment Give advice on preventing spread and follow-up Notify local authority if a notifiable disease is diagnosed or suspected (e.g. cholera)

Answer 211

``` Appears unwell or deteriorating Altered responsiveness e.g. lethargic, irritable Sunken eyes Tachycardia Tachypnoea Reduced skin turgor ```

Answer 212

Degree of weight loss during diarrhoeal illness: No clinically detectable dehydration <5% BW loss Clinical dehydration 5-10% loss of BW Shock >10% loss of BW

Answer 213

When children with diarrhoeal illness drink a large amount of water --> hyponatraemic This makes water move from the extracellular compartment into the intracellular compartment. The increase in intracellular volume leads to an increase in brain volume, which may result in seizures The marked extracellular depletion leads to a greater degree of shock per unit of water loss

Answer 214

High insensible water loss (e.g. fever, or hot/dry environment) Profuse, low sodium diarrhoea More difficult to identify clinically, as signs of extracellular fluid depletion are less per unit of fluid loss

Answer 215

Water is drawn out of the brain--> cerebral shrinkage, which may lead to jittery movements, increased muscle tone, hyperreflexia, altered consciousness, seizures, multiple small cerebral haemorrhages Transient hyperglycaemia occurs in some patients, but this is self-correcting

Answer 216

``` Child appears septic Blood or mucus in stools Child is immunocompromised Recent foreign travel Diarrhoea has not improved in 7 days Diagnosis is uncertain ``` If antibiotics are started, a blood culture should be taken

Answer 217

Shock Deterioration Persistent vomiting

Answer 218

ORS If IV fluids are required, a rapid reduction in plasma sodium concentration and osmolality will lead to a shift of water into the cerebral cells and may result in seizures and cerebral oedema, so reduction in plasma sodium should be slow- at least 48 hours, with regular measurement of plasma sodium

Answer 219

Ineffective May prolong the excretion of bacteria in stools Can be associated with side-effects Adds unnecessarily to cost Focus attention away from oral rehydration

Answer 220

Suspected or confirmed sepsis Extra-intestinal spread of bacterial infection Salmonella gastroenteritis if <6 months Malnourished or immunocompromised children Specific bacterial or protozoal infections (e.g. C diff associated with pseudomembranous colitis, cholera, shigellosis, giardiasis)

Answer 221

SO supplementation may be necessary

Answer 222

Occasionally, after an episode of gastroenteritis, the introduction of a normal diet results in a return of the watery diarrhoea Oral rehydration therapy should be restarted

Answer 223

Abnormal stools Poor weight gain or faltering growth (not all cases) Specific nutrient deficiencies, either singly or in combination True malabsorption stool is difficult to flush and has a potent smell

Answer 224

Autosomal recessive metabolic disorder characterised by tge malabsorption of zinc, which results in: Diarrhoea Inflammatory rash around the mouth and/or anus Hair loss

Answer 225

Enteropathy in which the gliadin fraction of gluten in wheat, barely and rye provoke a damaging immunological response in the proximal small intestine--> massive increase in rate of migration of absorptive cells moving up the villi from the crypts, but this is insufficient to compensate for increased cell loss from villous tips--> villi become shorter and progressively absent--> flat mucosa

Answer 226

Depends on the age of introduction of gluten into the diet

Answer 227

``` Profound malabsorptive syndrome at 8-24 months of age after introduction of wheat into diet Faltering growth Abdominal distension Buttock wasting Abnormal stools General irritability Mild, non-specific GI symptoms Anaemia ```

Answer 228

Anti-tTG antibodies Endomysial antibodies Duodenal biopsy (required to confirm diagnosis) Catch up of growth following gluten withdrawal from the diet is also required to confirm diagnosis

Answer 229

All products containing wheat, rye and barley are removed from diet Monitor body weight, height and BMI to assess for signs of malnutrition Consider referral to dietician if there are problems with adhering to the diet Arrange annual review Non-adherence results in: micronutrient deficiency (especially osteopenia), small increased risk of bowel cancer (especially small bowel lymphoma)

Answer 230

Most common cause of persistent loose stools in preschool children Stools vary in consistency, are sometimes well formed, sometimes explosive and loose Affected children are well and thriving In some cases, it might result from undiagnosed coeliac disease or excessive ingestion of fruit juice (especially apple juice) Occasionally caused by temporary cows' milk allergy after gastroenteritis Once most probably causes have been excluded, most cases are probably a result of dysmotility of the gut (form of IBS) and fast-transit diarrhoea These tend to improve with age

Answer 231

Crohns Crohns can occur anywhere from mouth to anus, whereas in UC the inflammation is confined to the colon

Answer 232

Poor general health Growth restriction Psychological

Answer 233

``` Growth failure Puberty delayed Abdo pain Diarrhoea Weight loss Fever, lethargy (can present without GI symptoms, especially in older children) Oral lesions or perianal skin tags Uveitis Arthralgia Erythema nodosum ``` May be mistaken for psychological problems and can mimic anorexia nervosa

Answer 234

Raised inflammatory markers (platelet count, ESR, CRP) Iron deficiency anaemia Low serum albumin

Answer 235

Strictures and fistulae may develop

Answer 236

Distal ileum or proximal colon

Answer 237

Upper GI endoscopy Ileocolonoscopy Small bowel imaging May reveal narrowing, fissuring, mucosal irregularities and bowel wall thickening Diagnosis based on endoscopic and histological findings on biopsy

Answer 238

Presence of non-caseating epitheloid cell granulomata (though this is not idenitified in up to 30% at presentation)

Answer 239

Remission is induced with nutritional therapy: the normal diet is replaced by whole protein modular feeds (polymeric diet). Lasts 6-8 weeks. Effective in 75% of cases. Systemic steroids are required if this is ineffective. Relapse is common: Immunosuppressants (e.g. azathioprine, mercaptopurine, methotrexate) are almost always required to maintain remission. Anti-TNF agents (e.g. infliximab, adalimumab) may be needed if conventional treatments fail. Long-term supplemental enteral nutrition (eg. NG or gastrotomy feeds) may be needed to correct growth failure Surgery is necessary for complications such as: Obstruction Fistulae Abscess formation Severe localised disease that is unresponsive to medical treatment

Answer 240

Assess impact of symptoms on daily functioning (anxiety, depression) Provide information on Crohn's disease and offer sources of support (Crohn's and Colitis UK) Encourage stopping smoking (may reduce the risk of relapse) Assess risk of osteoporosis Medical Management Steroids (prednisolone) may be used to induce and maintain remission Immunosuppressive drugs (azathioprine, methotrexate) Biologic therapies (e.g. infliximab) Aminosalicylates (e.g. mesalazine) Using these medical therapies requires monitoring of certain biochemical measures (e.g. ferritin, B12, calcium and vitamin D) Educate about features of Crohn's flare up (e.g. unintended weight loss) NOTE: patients on immunosuppressive therapies should NOT have live vaccines. They are at increased risk of influenza and pneumococcal infection so should receive vaccines.

Answer 241

Recurrent, inflammatory and ulcerating disease involving the mucosa of the colon

Answer 242

``` Rectal bleeding Diarrhoea Weight loss Colicky pain Growth failure Erythema nodosum Enteric arthritis ```

Answer 243

Endoscopy and histology: Mucosal inflammation Crypt damage (cryptitis, architectural distortion, abscesses, crypt loss) Ulceration 90% of children have pancolitis (entire large intestine)

Answer 244

Severity in children is assessed using the Paediatric Ulcerative Colitis Activity Index (PUCAI) Severe > 65 points Mild-Moderate - 10-64 points Mild Proctitis or Proctosigmoiditis Consider either oral or topical aminosalicylates or a combination of the two If aminosalicylates are NOT tolerated or are contraindicated: Topical corticosteroid or oral prednisolone Mild Left-Sided and Extensive Ulcerative Colitis Oral aminosalicylate Consider adding topical aminosalicylate or oral beclometasone If these are NOT tolerated, consider oral prednisolone If there is no improvement 4 weeks after starting aminosalicylate therapy Consider adding oral prednisolone If this still does not produce an adequate response, consider adding oral tacrolimus Biological agents such as infliximab, adalimumab and golimumab Resistant disease may require surgery Maintaining Remission Aminosalicylates are mainly used for maintaining remission Consider oral azathioprine or oral mercaptopurine if aminosalycilates are inadequate/frequent exacerbations requiring steroids Severe fulminating disease - EMERGENCY Assess likelihood of needing surgery Increased likelihood of needing surgery if: Stool frequency > 8 per day Pyrexia Tachycardia AXR showing colonic dilatation Low albumin, low haemoglobin, high platelets or CRP Offer IV corticosteroids to induce remission Consider IV ciclosporin (if IV corticosteroids are contraindicated or ineffective) Surgical Treatment Colectomy with an ileostomy or ileojejunal pouch UC is associated with an increased risk of bowel cancer Regular colonoscopic screening performed after 10 years of diagnosis

Answer 245

Dehydration Reduced fluid intake Anal fissures Anxiety

Answer 246

``` Hirschsprung disease Lower spinal cord abnormalities Anorectal abnormalities Hypothyroidism Coeliac disease Hypercalcaemia ```

Answer 247

Failure to pass meconium within first 24 hours of life - Hirschsprung disease Failure to thrive/growth failure - hypothryoidism, coeliac disease, other causes Gross abdominal distension - Hirschsprung disease or other GI dysmotility Abnormal lower limb neurology or deformity e.g. talipes, or secondary urinary incotninence - Lumbosacral pathology Sacral dimple above natal cleft, over the spine: naevus, hairy patch, central pit, or discoloured skin - Spina bifida occulta Abnormal appearance/position/patency of the anus - abnormal anorectal anatomy Perianal bruising or multiple fissures - sexual abuse Perianal fistulae, abscesses or fissures - Perianal Crohn's disease

Answer 248

Constipation arising acutely in young children (e.g. after an acute febrile illness) will usually resolve spontaneously or with the use of maintenance laxative therapy and fluids In more long-standing constipation, the rectum becomes overdistended. with the subsequent loss of feeling the need to defecate Involuntary soiling may occur

Answer 249

Exclude red flag symptoms Reassure that underlying causes of constipation have been excluded Laxatives - may have to be taken for several months Check for faecal impaction - if present, recommend disimpaction regimen Start maintenance laxative treatment if impaction is not present/has been treated Advice behavioural interventions (scheduled toileting, bowel habit diary, reward system) Diet and lifestyle advice (adequate fluid intake) Follow up to assess adherence and response to treatment Fluid requirements: Infants 0–6 months of age: 700 mL, assumed to be from milk. Babies 7–12 months of age: 800 mL from milk and complementary foods and beverages, of which 600 mL is assumed to be water from drinks. Children 1–3 years of age: 1300 mL (900 mL from drinks). Children 4–8 years of age: 1700 mL (1200 mL from drinks). Children 9–13 years of age: Boys — 2400 mL (1800 mL from drinks). Girls — 2100 mL (1600 mL from drinks). Young people 14–18 years of age: Boys — 3300 mL (2600 mL from drinks). Girls — 2300 mL (1800 mL from drinks). Secondary behavioural problems are common Initial Aim: evacuate the overloaded rectum completely Achieved using a disimpaction regimen of stool softeners, initially with a macrogol laxative (e.g. polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain)) An escalating dose regimen is administered over 1-2 weeks or until impaction resolves If unsuccessful: a stimulant laxative (e.g. senna or sodium picosulphate) may also be required NOTE: if polyethylene glycol and electrolytes is not tolerated, an osmotic laxative (e.g. lactulose) can be used instead Disimpaction must be followed by maintenance treatment to ensure ongoing regular, pain-free defecation Polyethylene glycol with/without a stimulant laxative are the treatment of choice Dose should be reduced over a period of months in response to an improvement in stool consistency and frequency NOTE: dietary changes tend to have little effect in these situations (the addition of more fibre may make the stools larger and more difficult to pass) Children can be encouraged to sit on the toilet after mealtimes to utilise the physiological gastrocolic reflex and improve the likelihood of success Behavioural interventions (e.g. star chart) may be useful to aid motivation and keep a record MAINSTAY OF TREATMENT: Early, aggressive and prolonged use of laxative medication Should allow the passage of a large, soft stool at least once a day Emphasise that laxative use is safe, even in the long-term Underuse is the most common cause of treatment failure Occasionally, faecal retention may be so severe that evacuation is only possible using: Enemas Manual evacuation under anaesthetic Learnt from Firms It is quite common for neonates to not poo for up to 10 days because to begin with they are absorbing most of the milk and relatively little is going through

Answer 250

Absence of ganglionic cells from the myenteric and submucosal plexuses of the large bowel--> narrow and contracted segment of the large bowel The region of abnormal bowel will extend from the rectum for a variable distance proximally, and will end with a normally innervated, dilated colon In 75% of cases, the lesion is confined to the rectosigmoid

Answer 251

Failure to pass meconium within the first 24 hours of life Later on, abdominal distension and bile-stained vomiting will develop Rectal examination may reveal a narrowed segment and withdrawal of the examining finger often releases a gush of liquid stools and flatus Occasionally, infants may present with severe, life-threateninig Hirschsprung enterocolitis during the first few weeks of life, sometimes due to C.difficile infection In later childhood, Hirschsprung disease can present with chronic constipation, which is usually profound and associated with abdominal distension (child may also show signs of growth failure)

Answer 252

Demonstration of absence of ganglion cells, along with the presence of large acetylcholinesterase-positive nerve trunks on a suction full thickness rectal biopsy Anorectal manometry or barium studies may provide useful information about the length of the aganglionic segment

Answer 253

Surgical - usually involves an initial colostomy followed by anastomosing normally innervated bowel to the anus (anorectal pull-through) Initial management involves bowel irrigation

Answer 254

< 4 weeks of age: electronic thermometer in the axilla 4+ weeks to 5 years: electronic or chemical dot thermometer in the axilla OR infrared tympanic thermometer Fever: >37.5 degrees NB: axillary measurement tend to underestimate body temperatures by around 0.5 degrees

Answer 255

``` Fever or temperature instability or hypothermia Poor feeding Vomiting Apnoea and bradycardia Respiratory distress Abdominal distension Jaundice Neutropenia Hypo/Hyperglycaemia Shock Irritability Seizures Lethargy, drowsiness ``` Meningitis: Tense or bulging fontanell Head retraction (opisthotonos)

Answer 256

Blood culture FBC including differential WCC Acute phase proteins (e.g. CRP) Urine sample If indicated: - CXR - LP - Rapid antigen screen on blood/CSF/urine - Meningococcal and pneumococcal PCR on blood/CSF PCR for viruses in CSF (especially HSV and enteroviruses)

Answer 257

Illness of close contacts Specific illness prevalent in the community Lack of immunisations Recent travel abroad (consider malaria, typhoid, and viral hepatitis) Contact with animals (consider brucellosis, Q fever, HUS caused by E.coli O157) Increased susceptibility from immunodeficiency - THis may be secondary: -- Post-autosplenectomy in sickle cell disease -- Nephrotic syndrome --> increased susceptibility of encapsulated organisms (e.g. S. pneumoniae, H. influenzae and Salmonella species) -- Primary immunodeficiency (RARE)

Answer 258

``` Fever > 38 degrees if <3 months Fever > 39 degrees if 3-6 months Colour - pale, mottled Reduced consciousness, neck stiffness, bulging fontanelle, status epilepticus, focal neurological signs, seizures Significant respiratory distress Bile-stained vomiting Severe dehydration or shock ?rash ?focus for infection ```

Answer 259

Children who are NOT seriously ill can be managed at home with regular review by the parents as long as they are given clear instructions Children who are very unwell require admission to the paediatric assessment unit, A&E or children's ward A septic screen should be requested Parenteral antibiotics should be given to seriously unwell children - < 1 month who has been discharged from hospital: 3rd generation cephalosporin (e.g. cefotaxime) - Often ampicillin is added to cover Listeria infection - 1+ months: high dose ceftriaxone - Aciclovir may be given is herpes simplex encephalitis is suspected MANAGEMENT (NICE Guidelines): Assess for risk of serious underlying cause Recommend paracetamol or ibuprofen for children with a temperature > 38 degrees who are distressed or unwell - NOTE: do NOT give antipyretics if they are well or if you are trying to prevent a febrile convulsion - IMPORTANT: do NOT give both drugs simultaneously. You may switch from one to the other if the first is ineffective Advice for Parents: Look for signs of dehydration (poor urine output, dry mouth, sunken anterior fontanelle) Offer regular fluids Dress the child appropriately for their surroundings Check the child regularly Keep the child away from nursery or school whilst the fever persists and notify the nursery or school of the illness ``` SAFETYNET - seek help if: Signs of dehydration Seizure Non-blanching rash Fever lasts > 5 days Child becoming generally unwell Distressed or concerned that they cannot look after the child ```

Answer 260

Bacterial infection of the meninges usually follows bacteraemia Much of the damage to the meninges is inflicted by the host response to infection rather than the organism itself Release of inflammatory mediators, recruitment of inflammatory cells and endothelial damage leads to cerebral oedema, raised ICP and decreased cerebral blood flow Inflammatory responses below the meninges leads to a vasculopathy resulting in cerebral cortical infarction fibrin deposits may block the resorption of CSF by the arachnoid villi leading to hydrocephalusm

Answer 261

Neonatal-3months: GBS E.coli and other coliforms Listeria monocytogenes 1 month-6 years: Neisseria meningitidis Streptococcus pneumoniae Haemophilus influenzae >6 years: Neisseria meningitidis Streptococcus pneumoniae

Answer 262

The early signs and symptoms of meningitis are non-specific, especially in infants and young children NB: neck stiffness may be seen in some children with tonsillitis and cervical lymphadenopathy As children with meningitis may also have sepsis, signs like tachycardia, tachypnoea and hypotension should be explored IMPORTANT: purpura in a febrile child of any age should be assumed to be due to meningococcal sepsis, even if the child does not seem particularly ill at the time

Answer 263

Blood tests: - CRP - WCC - Blood culture - PCR to check for N. meningitidis LP to obtain CSF to confirm the diagnosis, identify the causative organism and antibiotic sensitivites (confirm there are no clinical signs of raised ICP before LP) If an LP is contraindicated, it should be postponed until the child's condition has stabilised In addition, rapid antigen screens can be carried out on urine and blood samples Throat swabs should also be obtained for culture and PCR A serological diagnosis can be made 4-6 weeks after the presenting illness if necessary

Answer 264

``` Bacterial meningitis: Turbid CSF Increased polymorphic WCCs Raised protein Decreased glucose ``` ``` Viral meningitis: Clear CSF Increased lymphocytes (initially may be polymorphs) Normal/raised protein Normal/decreased glucose ``` ``` TB meningitis: Turbid/clear/viscous CSF Increased lymphocytes Greatly raised protein Greatly reduced glucose ``` ``` Encephalitis: Clear CSF Normal/raised lymphocytes Normal/raised protein Normal/decreased glucose ```

Answer 265

``` Admit to hospital as emergency Administer single dose of IM/IV benzylpenicillin (NB: check for penicillin allergy, in which case you might consider chloramphenicol and vancomycin) Administer IV ceftriaxone: - H. influenzae: 10 days - S. pneumoniae: 14 days - N. meningitidis: 7 days ``` Dexamethasone may be given if the following are seen on CSF analysis: - Frankly purulent CSF - CSF WBC >1000/microlitre - Raised CSF WBC + protein concentration > 1g/L - Bacteria on Gram stain - NB: steroids should not be used in meningococcal septicaemia IV 0.9% saline may be required if there are signs of shock/dehydration (monitor fluid administration and urinary output) Discharge and follow-up: Discuss potential long-term effects and pattern of recovery - Complications: hearing loss, orthopaedic complications, skin complications, psychosocial problems, neurological and developmental problems, renal failure Offer formal audiological assessment Consider testing for complement deficiency if they have had more than one episode of meningococcal disease or an episode caused by a serogroup other than the common ones

Answer 266

Hearing impairment: Due to inflammatory damage to cochlear hair cells ALL CHILDREN who have had meningitis should have an audiological assessment done promptly Children with hearing impairment may benefit from hearing amplification or a cochlear implant Local vasculitis: May lead to cranial nerve palsies and other focal neuro signs Local cerebral infarction: May result in focal or multifocal seizures, which may result in epilepsy Subdural effusion: Particularly associated with H. influenzae and pneumococcal meningitis Confirm by CT or MRI Most resolve spontaneously Hydrocephalus: May result from impaired resorption of CSF (communicating) or blockage of CSF flow (non-communicating) ``` Cerebral abscess: Will result in the child's clinical condition deteriorating with or without the emergence of signs of space-occupying lesion Temperature will continue to fluctuate Confirmed by cranial CT or MRI Drainage of abscess required ```

Answer 267

Prophylactic treatment with rifampicin or ciprofloxacin to eradicate nasopharyngeal carriage is given to ALL household contacts for meningococcal meningitis and H. influenzae infection It is not given to the patient as 3rd generation cephalosporin will eradicate nasopharyngeal carriage anyway Household contacts of patients with group C meningococcal meningitis should be vaccinated with meningococcal group C vaccine

Answer 268

Children are often given oral antibiotics for non-specific febrile illness If they have early meningitis, this treatment may lead to diagnostic problems CSF will show markedly elevated white cells, but cultures will usually be negative Rapid antigen screens and PCR are helpful

Answer 269

Enteroviruses EBV Adenoviruses Mumps

Answer 270

Culture or PCR of CSF/stool/urine/nasopharyngeal aspirate/throat swabs Serology

Answer 271

Mycoplasma Borellia burgdorferi (Lyme disease) TB Fungal infections These uncommon organisms are particularly likely in children who are immunocompromised (e.g. immunodeficiency, chemotherapy) Recurrent bacterial infections may occur in immunodeficient children or in those with structural abnormalities of the skull or meninges Aseptic meningitis can occur in malignancy or autoimmune diseases

Answer 272

In encephalitis, the inflammation is in the brain substance, although the meninges are often affected

Answer 273

Direct invasion of the brain by neurotoxic virus (e.g. HSV) Delayed brain swelling following a dysregulated neuroimmunological response to an antigen, usually a virus (post-infectious encephalopathy)e.g. following chickenpox Slow virus infection, such as HIV or subacute sclerosing pan-encephalitis following measles

Answer 274

Most children will present with fever, altered consciousness and often seizures Initially, it may be impossible to distinguish clinically between encephalitis and meningitis, so treatment for both should be started

Answer 275

``` Enteroviruses Respiratory viruses (influenza viruses) Herpes viruses (HSV, VZV, HHV-6) ```

Answer 276

Mycoplasma Corellia burgdorferi (Lyme disease) Bartonella henselae (cat scratch disease) Rickettsial infections (e.g. Rocky mountain spotted fever) Arboviruses

Answer 277

PCR is used to detect HSV in the CSF HSV is a destructive infection so the EEG and CT/MRI may show focal changes NB: these tests can be normal initially, so should be repeated if the child is not improving Later confirmation of the diagnosis may be made from HSV antibody production in the CSF

Answer 278

Proven and suspected cases of HSV encephalitis should be treated with IV aciclovir for 3 weeks, as relapses ma occur after shorter courses If untreated, the mortality rate is >70% and survivors usually have serious neurological sequelae

Answer 279

Toxin-producing S. aureus | Group A streptococci

Answer 280

Fever > 39 degrees Hypotension Diffuse erythematous, macular rash The toxin acts as a superantigen and can cause organ dysfunction: Mucositis (conjunctiva, oral mucosa, genital mucosa) GI dysfunction (vomiting/diarrhoea) Renal impairment Liver impairment Clotting abnormalities and thrombocytopenia CNS (altered consciousness) NB: around 1-2 weeks after the onset of the illness, you will see desquamation of the palms, soles, fingers and toes

Answer 281

``` High flow oxygen IV access and take bloods Give IV or IO antibiotics Consider fluid resus Involve senior clinicians Consider ionotropic support early ```

Answer 282

Intensive care support is required for patients in shock Areas of infection should be surgically debrided Antibiotics often used: - 3rd generation cephalosporn (eg. ceftriaxone) - Clindamycin: this acts on the bacterial ribosome to switch off toxin production - IVIG: may be given to neutralise the circulating toxin

Answer 283

This is produced by <2% of S.aureus strains PVL-producing S.aureus causes recurrent skin and soft tissue infections They can also cause necrotising fasciitis and a necrotising haemorrhagic pneumonia following an infleunza-like illness These both carry a high mortality rate In children, the procoagulant state induced by the toxin can cause venous thrombosis

Answer 284

Rare, severe subcutaneous infection The area involved may enlarge rapidly, leaving poorly perfused necrotic areas of tissue at the centre It is accompanied by severe pain and systemic illness and usually requires intensive care

Answer 285

Staph aureus Group A streptococcus NB: these can exist with or without a synergistic anaerobic organism (ie. it can be a mixed infection)

Answer 286

SURGICAL EMERGENCY Debridement of all infected and devitalised tissues IV fluids Empirical IV antibiotics (vancomycin, linezolid, daptomycin, tedizolid phsophate, tazocin, meropenem, imipenem/cilasttatin, ertapenem) IV antibiotics alone are not sufficient to treat this condition Surgical intervention and debridement of necrotic tissue is necessary or the infection will continue to spread Any clinical suspicion of necrotising fasciitis requires urgent surgical consultation IVIG may also be given

Answer 287

Of the three main causes of bacterial meningitis, meningococcal septicaemia has the lowest-risk of long-term neurological sequelae with most survivors recovering fully Often accompanied by a purpuric rash, which may start anywhere on the body and spread The rash is characteristically non-blanching on palpation, irregular in size and outline, and may have a necrotic centre

Answer 288

ANY FEBRILE CHILD with a purpuric rash should be treated immediately with IM penicillin or IV 3rd generation cephalosporin before urgent admission to hospital

Answer 289

After the inclusion of the meningococcus C vaccine in th eUK, most cases of meningococcal septicaemia are caused by group B meningococci

Answer 290

``` Pharyngitis Otitis media Conjunctivitis Sinusitis Invasive disease (pneumonia, bacterial sepsis, meningitis): this mainly occurs in young infants as their immune system is weak against encapsulated organisms. It carries a high morbidity and mortality ```

Answer 291

Eg. hyposplenism Daily prophylactic penicillin to prevent infection

Answer 292

Usually caused by direct invasion of the organisms They can also cause disease by releasing toxins which act as superantigens - Normal antigens will stimulate only a small subset of T cells, which have a specific antigen receptor - Whereas, superantigens bind to part of the TCR which is shared by many T cells - This results in massive T cell proliferation and cytokine release

Answer 293

A localised, highly contagious, staph or strep skin infection, most common in infants and young children, or in children with pre-existing skin disease (eg. eczema) Lesions are usually found on the face, neck and hands They begin as erythematous macules which become vesicular/pustular or even bullous Rupture of the vesicles with exudation of fluid leads to honey-coloured crusted lesions Infection readily spreads to adjacent areas and other parts of the body by autoinoculation of the infected exudate

Answer 294

Advice Leaflets from British Association of Dermatologists (BAD) Reassure that impetigo usually heals without any scarring Hygiene is important: wash areas with soapy water, wash hands after touching lesions, avoid scratching affected areas and keep nails short, avoid sharing towels/bathwater etc. Children should avoid school until the lesions are dry and scabbed over Arrange follow-up if no improvement after 7 days Medical Treatment Localised Infection = topical fusidic acid (3-4/day for 7 days) Extensive Infection = oral flucloxacillin (4/day for 7 days) Clarithromycin can be used if penicillin allergy Bullous Infection = oral flucloxacillin or clarithromycin/erythromycin If no improvement after 7 days Review diagnosis Check compliance with treatment and hygiene measures Take a swab Consider oral antibiotics if fusidic acid was initially used IMPORTANT: affected children should NOT go to nursery or school until the lesions are dry

Answer 295

Infections of hair follicles or sweat glands, usually caused by S. aureus Recurrent boils may occur due to persistent nasal carriage in the child or the family acting as a reservoir for reinfection Rarely, recurrent boils can be a feature of underlying immunodeficiency

Answer 296

Systemic antibiotics and (occasionally) surgical incision

Answer 297

Characterised by fever with erythema, tenderness and oedema of the eyelid or other skin adjacent to the eye Almost always unilateral May follow trauma to the skin In older children, it can spread from paranasal sinus infectino or dental abscess

Answer 298

Should be treated promptyl with IV antibiotics (e.g. high-dose ceftriaxone) This is to prevent posterior spread of the infection which could cause orbital cellulitis Incision and drainage of perio-ocular abscess may be required

Answer 299

Characterised by proptosis, painful or limited ocular movement with/without reduced visual acuity It can be complicated by abscess formation, meningitis or cavernous sinus thrombosis CT/MRI should be performed to assess for posterior spread of infection

Answer 300

An exfoliative staphylococcal toxin It causes the separation of the epidermal skin through the granular cell layers

Answer 301

Infants and young children

Answer 302

Fever Malaise Purulent, crusting and localised infection around the eys, nose and mouth Subsequent widespread erythema and tenderness of the skin Nikolsky Sign: area of epidermis will separate on gentle pressure, leaving denuded areas of skin, which tend to heal without scarring

Answer 303

IV antibiotics (flucloxacillin) Analgesia Monitoring hydration and fluid balance

Answer 304

HHV-8 in HIV-infected individuals)

Answer 305

After primary infection, latency is established and there is long-term persistence of the virus within the host (usually in a dormant state): this can be reactivated after certain stimuli

Answer 306

Through mucous membranes or skin | The site of primary infection may be associated with intense local mucosal damage

Answer 307

HSV1: lip and skin lesions HSV2: genital lesions NB: both viruses can cause both types of disease

Answer 308

Paracetamol or ibuprofen may be given to help with pain and fever Aciclovir (DNA polymerase inhibitor) may be considered Most herpes simplex infections are asymptomatic

Answer 309

Most common form of primary HSV in children Usually occurs in 10 months-3 years of age Presents with vesicular lesions on the lips, gums and anterior surfaces of the tongue and hard palate They usually progress to extensive, painful ulceration and bleeding It is often accompanied by a high fever The illness can persist for up to 2 weeks Eating and drinking become painful, resulting in dehydration

Answer 310

Symptomatic | Severe cases may require IV fluids and aciclovir

Answer 311

Widespread vesicular lesions develop on eczematous skin | This can be complicated by secondary bacterial infection, which can then, in turn, result in septicaemia

Answer 312

Painful, erythematous and oedematous white pustules on the site of broken skin (usually on fingers) It is spread by autoinoculation form gingivostomatitis

Answer 313

HSV can cause blepharitis or conjunctivitis It can involve the cornea and cause dendritic ulceration This can result in corneal scarring and loss of vision ANY CHILD with herpetic lesions near of involving the eye require urgent ophthalmic assessment

Answer 314

May be focal (e.g. affecting the skin, eyes or encephalitis) May be disseminated High morbidity and mortality

Answer 315

May be severe Cutaneous lesions can spread to involve adjacent sites (e.g. oesophagitis, proctitis) Pneumonia and disseminated infections involving multiple organs are major complications

Answer 316

Secondary Bacterial Infection Mainly with staphylococci and group A streptococci Can lead to toxic shock syndrome and necrotising fasciitis Should be considered when there is onset of a new fever or persistent high fever after the first few days Encephalitis May be generalised Usually occurs early in the illness GOOD prognosis (as opposed to HSV encephalitis) Characteristically causes a VZV-associated cerebellitis The child becomes ataxic with cerebellar signs This usually occurs around 1 week after the onset of the rash Usually resolves within a month Purpura Fulminans Consequence of vasculitis in the skin and subcutaneous tissues Best known in relation to meningococcal disease (purpuric rash) Can lead to the loss of large areas of skin by necrosis Rarely, after VZV infection, antiviral antibodies can cross-react and inactivate inhibitory coagulation factors (protein S and protein C), resulting in increased risk of clotting, which often manifests as a purpuric rash If the patient is immunocompromised, primary VZV infection could result in severe progressive disseminated disease (up to 20% mortality) Vesicular eruptions may become haemorrhagic

Answer 317

Admit to hospital if there are serious complications (e.g. pneumonia, encephalitis, dehydration) In immunocompetent adolescents and adults, consider oral aciclovir 800 mg 5/day for 7 days if they present within 24 hours of the onset of the rash of if the chickenpox is severe This is NOT recommended for otherwise healthy children with chickenpox ADVICE Encourage adequate fluid intake Dress appropriately to avoid overheating or shivering Wear smooth, cotton fabrics Keep nails short Explain that the most infectious period is 1-2 days before the rash appears Infectivity continues until all the lesions are dry and crusted over (usually ~5 days after onset of the rash) During this time, patients should AVOID contact with: People who are immunocompromised Pregnant women Infants < 4 weeks Children should be kept away from school until the vesicles have crusted over Seek urgent medical advice if the condition deteriorates or they develop complications: Bacterial superinfection - sudden high-grade pyrexia with erythema and tenderness around the original chickenpox lesions Dehydration - e.g. reduced urine output, lethargy, cool peripheries Immunocompromised children Should be treated with IV aciclovir Oral valaciclovir may be substituted later on Prevention in Immunocompromised Patients Human varicella zoster immunoglobulin should be used in high-risk immunocompromised individuals with deficient T cell function following contact with chickenpox NOTE: this protection is NOT absolute

Answer 318

UNCOMMON in children Caused by reactivation of latent VZV Characteristically causes a vesicular eruption in the dermatomal distribution of the sensory nerves Most commonly affects the thoracic region Recurrent or multi-dermatomal shingles is strongly associated with underlying immunocompromise (e.g. HIV infection) NB: in immunocompromised patients, reactivation of the infection can also disseminate and cause severe disease

Answer 319

Infectious mononucleosis

Answer 320

Burkitt lymphoma Lymphoproliferative disease Nasopharyngeal carcinoma

Answer 321

EBV has a tropism for B lymphocytes and epithelial cells of the oropharynx It is transmitted by oral contact

Answer 322

Most infections are subclinical Older children (and sometimes younger children) may develop a syndrome with: Fever Malaise Tonsilitis/pharyngitis (often severe enough to limit food and fluid intake) Lymphadenopathy (prominent lymph nodes, often with diffuse lymphadenopathy elsewhere) Petechiae of the soft palate Splenomegaly Hepatomegaly Maculopapular rash Jaundice

Answer 323

Very clinical but can be supported by certain lab findings Atypical lymphocytes (numerous large T cells) Positive monospot test (detects the presence of heterophile antibodies- this test is often negative in young children with EBV) Seroconversion with production of 3 anitbodies: Viral capsid antibodies (VCA) IgG and IgM, EB nuclear antigen (EBNA) antibodies

Answer 324

Paracetamol or ibuprofen to relieve pain and fever Explain the expected course of the illness (2-3 week duration) They dont need to avoid work or school but should do it depending on how they feel Limit the spread of the disease Avoid collision/contact sport Corticosteroids may be considered if the airway is severely compromised (RARE) Seek medical help if: Stridor or respiratory difficulty Difficulty swallowing fluids or signs of dehydration Systemically very unwell Abdominal pain (e.g. due to splenic rupture)

Answer 325

Can cause a florid maculopapular rash in children infected with EBV

Answer 326

Usually transmitted via saliva, genital secretion or breastmilk

Answer 327

Causes mild or subclinical infection in normal paediatric and adult hosts About 50% of adults show serological evidence of past infection In the immunocompromised host and developing foetus (transmitted from the mother), CMV is an important pathogen that can cause considerable morbidity CMV causes an mononucleosis-like syndrome: Pharyngitis and lymphadenopathy are not as prominent as in EBV Patients will have atypical lymphocytes on blood film They will be heterophile antibody negative ``` In the immunocompromised host, CMV can cause: Retinitis Pneumonitis Bone marrow failure Encephalitis Hepatitis Oesophagitis Enterocolitis ```

Answer 328

CMV is particularly important following bone marrow and organ transplantation Transplant recipients are closely monitored for evidence of CMV reactivation by blood PCR Antiviral prophylaxis may also be used

Answer 329

CMV is self-limiting ``` If necessary, CMV can be treated with: IV ganciclovir Oral valganciclovir Foscarnet NB: these all have serious side-effects ```

Answer 330

HHV6 Most children are infected by HHV6 or 7 by the age of 2 years, usually from the oral secretions of a family member

Answer 331

These viruses classically cause exanthema subitum (aka roseola infantum): This is characterised by high fever and malaise lasting a few days This is followed by a generalised macular rash, which appears as the fever wanes Many children will have the fever without the rash and many will have a subclinical infection NB: this is often misdiagnosed as measles or rubella NB: infants in the febrile stage may be given antibiotics, and when the exanthema subitum rash appears, they can be misdiagnosed with an allergy to anitbiotics

Answer 332

The condition will resolve over a few days/ weeks Paracetamol or ibuprofen for symptomatic relief Advice to maintain adequate hydration Explain risk of febrile seizures

Answer 333

Via respiratory secretions or by vertical transmission from mother to foetus It can be transmitted via infected blood products Outbreaks are more common in spring

Answer 334

Asymptomatic infection: About 65% of adults have anitbodies against HPV-B19 Erythema infectiosum: Most common It has a viraemic phase of fever, malaise, headache and myalgia This is followed by a characteristic rash on the face (slapped-cheek), roughly a week later This progresses into a maculopapular, lace-like rash on the trunk and limbs Complications are rare in children In adults, it can cause arthralgia and arthritis Aplastic crisis: MOST SERIOUS Occurs in children with chronic haemolytic anaemias (e.g. sickle cell disease, thalassemia) Foetal disease: Can lead to foetal hydrops and death due to severe anaemia Most infected foetuses will recover

Answer 335

Paracetamol or ibuprofen and encourage adequate fluid intake | Secondary arthritis may be treated with ibuprofen

Answer 336

Coxsackie virus Echoviruses Polioviruses

Answer 337

Primarily transmitted via the faeco-oral and respiratory droplet routes Infections mainly occur in the summer and autumn

Answer 338

Over 90% of infections are asymptomatic or cause non-specific febrile illness It can sometimes cause a rash on the trunk that is blanching and consists of fine petechiae The child is not usually systemically unwell Hand, foot and moth disease: Painful vesicular lesions on hands, feet , mouth and tongue Systemic features are generally mild Disease subsides within days Herpangina: Vesicular and ulcerated lesions on the soft palate and uvula leads to anorexia, painful swallowing and fever Severe cases may require IV fluids and analgesia Meningitis/encephalitis: In developed countries, enteroviruses are the most common cause of viral meningitis Most cases make a full recvoery Pleurodynia (Bornholm disease): An acute illness with fever, pleuritic chest pain and muscle tenderness Recover within days Myocarditis/pericarditis: RARE Children may present with chest pain and/or heart failure assocaited with a febrile illness and evidence or myocarditis on ECG Enteroviral neonatal sepsis syndrome: RARE Occurs in the first few weeks of life Results from transplacental/intrapartum infection of the infants The symptoms are often sever, mimicking bacterial sepsis Affected infants may present with hypotension and multiorgan failure Intensive care support is required There are no antiviral drugs that are effective against enteroviruses and the use of IVIG remains controversial

Answer 339

Older children and adults tend to have more severe disease ``` Prodrome of fever Malaise Cough Coryza Koplik spots followed by maculopapular rash ``` Encephalitis: RARE Initial symptoms are headache, lethargy and irritability Thus proceeds to seizures and coma Up to 15% mortality Serious long-term sequelae include: seizures, deafness, hemiplegia, severe learning difficulties Subacute Sclerosing Panencephalitis (SSPE): RARE but devastating Occurs, on average, 7 years after measles infection Caused by a variant of the measle virus that persists in the CNS Present with loss of neurological function This progresses, over several years, to dementia and death ``` Complications: Otitis media Bronchopneumonia Laryngotracheobronchitis Diarrhoea ```

Answer 340

Immediately notify the local Health Protection Team Advise that measles is a self-limiting disease but it is likely to cause unpleasant symptoms such as rash, fever, cough and conjunctivitis Rest and drink plenty Paracetamol or ibuprofen can be used for symptomatic relief Stay away from school for at least 4 days after the development of the rash Seek urgent medical advice if they develop complications such as SOB, uncontrolled fever, convulsions or altered consciousness Encourage vaccinations once the acute episode has subsided Find out the immunisation status of close contacts Children should be isolated in hospital In immunocompromised patients, ribavirin may be of use Vitamin A is given in low-income countries

Answer 341

Malnutrition and vitamin A deficiency leads to impaired cell-mediated immunity

Answer 342

Spread by droplet infection to the respiratory tract where the virus replicates within epithelial cells The virus gains access to the parotid glands before further dissemination to other tissues

Answer 343

Incubation period: 15-24 days The disease begins with a fever, malaise and parotitis (this may be initially unilateral, but it will eventually become bilateral after a few days) In up to 300%, infection will be subclinical Parotitis is uncomfortable, and children may complain of earache or pain when eating/drinking Examination of the parotid duct may show redness and swelling The fever usually subsides after 3-4 days Plasma amylase levels are usually elevated due to parotid inflammation When associated with abdo pain, consider pancreatic involvement Infectivity lasts for up to 7 days after the onset of parotid swelling The illness is usually mild and self-limiting Hearing loss can rarely follow mumps, but is usually unilateral and transient

Answer 344

Notify the local Health Protection Unit Diagnosis is usually confirmed using an oral swab Advise that it is a self-limiting condition Advise patient to rest and take in adequate fluids Paracetamol and ibuprofen can be used for symptomatic relief Stay away from school for 5 days after development of parotitis Consider follow-up 1 week after the onset of parotitis Advise patient/parents to seek help if they experience symptoms suggestive of meningitis or epididymo-orhcitis Find out immunisation status of close contacts and tell them to watch out for symptoms of mumps

Answer 345

Viral meningitis and encephalitis: Lymphocytes will be seen in the CSF in about 50% of cases Typical meningeal signs are only seen in 10% Orchitis: MOST FEARED complications Usually unilateral Infertility is unusual after mumps orchitis Hearing loss

Answer 346

Respiratory route

Answer 347

May have a prodrome of low-grade fever or no symptoms at all A maculopapular rash is often the first sign of infection: it tends to initially appear on the face and spread centrifugally to cover the whole body. Fades in 3-5 days Lymphadenopathy, particularly suboccipital and postauricular nodes

Answer 348

Complications are rare in children Arthritis Encephalitis Thrombocytopenia Myocarditis

Answer 349

Contact the local Health Protection Unit Diagnosed using an oral fluid sample Advise that it is a self-limiting disease Rest and take in adequate fluids Consider admitting if there is a serious complication such as haemorrhagic complications (caused by thrombocytopenia) or encephalitis

Answer 350

Contact the local Health Protection Unit Diagnosed using an oral fluid sample Advise that it is a self-limiting disease Rest and take in adequate fluids Consider admitting if there is a serious complication such as haemorrhagic complications (caused by thrombocytopenia) or encephalitis

Answer 351

6 months to 4 years Young infants tend to be more severely affected and are more likely to have incomplete symptoms (ie. not the full list of cardinal features)

Answer 352

CRASH and burn Conjunctivitis Rash Adenopathy (usually cervical) Strawberry tongue Hand - swelling or erythema on the hands and feet Burn - fever (usually difficult to control)

Answer 353

High inflammatory markers with a platelet count that rises int eh 2nd week of the illness

Answer 354

Coronary arteries ar affected in about 1/3 children within the first 6 weeks of the illness This can lead to aneurysms which can be detected on echocardiography Subsequent narrowing of the vessels due to scarring can lead to myocardial ischaemia and sudden death

Answer 355

IVIG High-dose aspirin (reduced thrombosis risk) Other treatment options include corticosteroids, infliximab and plasma exchange Children with giant coronary artery aneurysms may require long-term warfarin and close follow-up Patients should, therefore, have a cardiovascular risk assessment Children with persistent inflammation and fever may require corticosteroids, infliximab or ciclosporin

Answer 356

Clinical features are often non-specific: Prolonged fever Malaise Anorexia Weight loss Focal signs of infection (e.g. lymph node swelling) Extra-pulmonary disease is relatively uncommon (e.g. TB lymphadenitis, osteoarticular TB, genitourinary TB, TB meningitis)

Answer 357

Sputum samples are unobtainable in children < 8 years old As children usually swallow sputum, gastric washings on 3 consecutive mornings can be used to identify M. tuberculosis using Ziehl-Nielsen stains or auramine stains and mycobacterial cultures (this is obtained by passing an NG tube and washing out secretions in the stomach with saline) PCR-based methods are increasingly used in parallel with mycobacterial cultures but these methods provide limited information about drug resistance Tuberculin Skin Test (TST) This is done by injecting purified protein derivative intradermally into the forearm and observing 48-72 hours later and measuring the induration in millimetres NOTE: PPD is a mixture of proteins, some of which are expressed by M. tuberculosis and the BCG, so a positive test result may be because of past BCG vaccination rather than latent TB Originally, previous vaccination was taken into account when interpreting the TST result NEW GUIDELINES: state that an induration of > 5 mm should be considered to be POSITIVE regardless of prior BCG vaccination Interferon-Gamma Release Assay (IGRAs) New blood-based test Assesses the response of T cells to in vitro stimulation with a small number of antigens that are expressed by M. tuberculosis but NOT BCG Therefore, a positive result will indicate TB infection rather than BCG HOWEVER, a negative result does NOT reliably rule out TB infection IMPORTANT: neither the IGRA or the TST can reliably distinguish between latent TB and active TB NOTE: with advanced immunocompromise (e.g. HIV) both TST and IGRA can produce false negatives BEWARE: do NOT attempt to diagnose TB based on CXR alone in HIV patients because lymphoid interstitial pneumonitis can have a similar appearance and occur in 20% of HIV-infected children All individuals with TB should be tested for HIV and vice versa

Answer 358

Arrange hospital admission if the patient has suspected active TB and is unwell If hospital admission is not needed, arrange urgent referral to specialist TB service It is a notifiable disease Medical management: Riafmpicin+ Isoniazid = 6 months Pyrazanamide + Ethambutol - first 2 months In adolescents, pyridoxine (vitamin B6) is given weekly to prevent peripheral neuropathy due to isoniazid In tuberculous meningitis, dexamethasone is given initially to reduce the risk of long-term sequelae IMPORTANT: asymptomatic children who are Mantoux or IGRA positive (ie. latently infected) should also be treated as it will decrease the risk of reactivation later in life

Answer 359

There are several in the environment Immunocompetent individuals rarely get affected by these organisms May occasionally cause persistent lymphadenitis in young children TREATMENT: complete lymph node excision or watchful waiting (in most cases, spontaneous resolution occurs) NOTE: these organisms are transmitted by soil and water so NO contact tracing is needed It can cause disseminated infection in immunocompromised individuals Mycobacterium avium intracellulare infections are particularly common in patients with advanced HIV These infections do NOT respond to conventional anti-TB treatment Patients with cystic fibrosis are particularly susceptible to these infections

Answer 360

There are several in the environment Immunocompetent individuals rarely get affected by these organisms May occasionally cause persistent lymphadenitis in young children TREATMENT: complete lymph node excision or watchful waiting (in most cases, spontaneous resolution occurs) NOTE: these organisms are transmitted by soil and water so NO contact tracing is needed It can cause disseminated infection in immunocompromised individuals Mycobacterium avium intracellulare infections are particularly common in patients with advanced HIV These infections do NOT respond to conventional anti-TB treatment Patients with cystic fibrosis are particularly susceptible to these infections

Answer 361

Sub-Saharan Africa

Answer 362

MOther-to-child transmission: Intrauterine (during pregnancy) Intrapartum (at delivery) Postpartum (through breastfeeding) Uncommon routes of transmission to children include infected blood products, contaminated needles and sexual abuse

Answer 363

Children > 18 months: detect antibodies against the virus Children <18 months: who are born to infected mothers will have transplacental maternal IgG HIV antibodies, so a positive antibody test will confirm HIV exposure but not infection: The most sensitive test in this age group is HIV RNA PCR ALL babies born to HIV-infected mothers should be tested, irrespective of whether they are symptomatic or not

Answer 364

Some HIV-infected infants will progress rapidly to symptomatic disease and AIDS within the first year of life Others will remain asymptomatic for months or years before manifesting clinical features Presentation depends on the degree of immunocompromise: Mild= lymphadenopathy or parotid enlargement Moderate= recurrent bacterial infections, candidiasis, chronic diarrhoea, lymphocytic interstitial pneumonitis Severe: PCP, severe growth faltering, encephalopathy, malignancy (RARE) IMPORTANT: an unusual constellation or symptoms (especially infectious) should make you consider HIV

Answer 365

Mothers with a high viral load are more likely to transmit HIV to their infant Avoidance of breastfeeding also reduces transmission IN high-income countries, perinatal transmission of HIV is <1% due to: Use of effective ART during pregnancy and intrapartum to achieve an undetectable maternal viral load at delivery Post-exposure prophylaxis given to infant Avoidance of breastfeeding Active management of labour/delivery to avoid prolonged rupture of the membranes and unnecessary instrumentation Pre-labour C-section if the mother's viral load is detectable close to the due date

Answer 366

Mothers with a high viral load are more likely to transmit HIV to their infant Avoidance of breastfeeding also reduces transmission IN high-income countries, perinatal transmission of HIV is <1% due to: Use of effective ART during pregnancy and intrapartum to achieve an undetectable maternal viral load at delivery Post-exposure prophylaxis given to infant Avoidance of breastfeeding Active management of labour/delivery to avoid prolonged rupture of the membranes and unnecessary instrumentation Pre-labour C-section if the mother's viral load is detectable close to the due date

Answer 367

Spirochaete Borrelia burgdorferi

Answer 368

Incubation period: 4-20 days Erythematous macule at the site of the tick bite enlarges to cause erythema migrans (descrubed as a painless, red expanding leison with a bright red outer spreading edge) ``` Early features: Fever (fluctuate over several weeks and resolve) Headache Malaise Myalgia Arthralgia Lymphadenopathy NB: dissemination of infection early is rare but can lead to cranial nerve palsies, meningitis, arthritis or carditis ``` Late features (weeks or months after initial infection): Neurological (meningoencephalitis, cranial and peripheral neuropathies) Cardiac (myocarditis, heart block) Joint (varies from brief migratory myalgia to acute asymmetric monoarthritis and oligoarthritis of the large joints) - occurs in 50% of patients; recurrent attacks of arthritis are common; chronic erosive joint disease can occur months/years after the initial attack in 10% of cases

Answer 369

Incubation period: 4-20 days Erythematous macule at the site of the tick bite enlarges to cause erythema migrans (descrubed as a painless, red expanding leison with a bright red outer spreading edge) ``` Early features: Fever (fluctuate over several weeks and resolve) Headache Malaise Myalgia Arthralgia Lymphadenopathy NB: dissemination of infection early is rare but can lead to cranial nerve palsies, meningitis, arthritis or carditis ``` Late features (weeks or months after initial infection): Neurological (meningoencephalitis, cranial and peripheral neuropathies) Cardiac (myocarditis, heart block) Joint (varies from brief migratory myalgia to acute asymmetric monoarthritis and oligoarthritis of the large joints) - occurs in 50% of patients; recurrent attacks of arthritis are common; chronic erosive joint disease can occur months/years after the initial attack in 10% of cases

Answer 370

Based on clinical and epidemiological features and serology Isolation of the organism is difficult If Lyme disease is suspected without erythema migrans, offer an ELISA for Lyme disease If the ELISA is positive, perform an immunoblot test If immunoblot is positive, start treatment with oral antibiotics

Answer 371

1st line: Doxycycline 2nd line: Amoxicillin 3rd line: Azithromycin IV ceftriaxone is needed for carditis or neurological disease Advice: Explain that it is a bacterial infection treated with antibiotics but it can take some time (around a month) to get better Some people can have a Jarish-Herxheimer reaction (antibiotics cause lysis of bacterial membranes leading to the release of toxins causing symptoms to worsen) Lyme disease does not give lifelong immunity

Answer 372

``` Diptheria Tetanus Pertusis Polio Hib Hep B ```

Answer 373

2 months 3 months 4 months

Answer 374

2 months 4 months 12 months

Answer 375

2 months 4 months 12 months

Answer 376

2 months | 3 months

Answer 377

1 year | 3 years 4 months

Answer 378

12-13 years

Answer 379

Swelling and discomfort at the injection site with mild fever and malaise Anaphylaxis can occur but this is very rare Live vaccines should not be given to immunocompromised children (except HIV-positive children on ART) Vaccination should be postponed if the child has an acute illness

Answer 380

Recurrent (proven) bacterial infection Severe infections (e.g. meningitis, osteomyelitis, pneumonia) Infections that present atypically, are unusually severe or chronic or fail to respond to regular treatment Infections caused by an unexpected or opportunistic pathogen or a pathogen that the child has been immunised against Severe or long-lasting warts, generalised molluscum contagiosum Extensive candidiasis Complications following live vaccinations (disseminated BCG) Abscesses of internal organs; recurrent skin abscesses Prolonged or recurrent diarrhoea (often combined with faltering growth)

Answer 381

Antimicrobial prophylaxis - For T-cell and neutrophil defects: Co-trimoxazole to prevent PCP Itraconazole or fluconazole to prevent other fungal infections - For B-cell defects: Antibiotic prophylaxis (e.g. azithromycin) to prevent recurrent bacterial infections Antibiotic treatment Prompt treatment of infections Longer courses Low threshold for IV therapy Screening for end-organ disease E.g. CT scan in children with antibody deficiency to detect bronchiectasis Immunoglobulin replacement therapy For children with antibody deficiency Bone marrow transplantation E.g. for SCID, chronic granulomatous disease Gene therapy

Answer 382

Early phase: occurring within minutes of exposure to the allergen, caused by release of histamine and other mediators from mast cells. Causes urticaria, angioedema, sneezing, vomiting,bronchospasm and/or cardiovascular shock Late phase: may occur after 4-6 hours, especially in reacitons to inhalant allergens. This causes nasal congestion in the upper airway, and cough and bronchospasm in the lower airway Non-IgE allergic immune responses have a delayed onset of symptoms and a more varied clinical course

Answer 383

Objectively reproducible symptoms or signs following exposure to a defined stimulus (e.g. food, drug, pollen) at a dose that is usually tolerated by most people

Answer 384

A hypersensititivity reaciton initiated by specific immunological mechanisms. This can be IgE-mediated (e.g. peanut allergy) or non-IgE mediated (e.g. coeliac disease)

Answer 385

A personal and.or familial tendency to produce IgE antibodies in response to ordinary exposures to potential allergens, usually proteins. Strongly associted with asthma, allergic rhinitis, and conjunctivitis, eczema and food allergy

Answer 386

A serious allergic reaction with bronchial, laryngeal and cardiovascular involvement that is rapid in onset and may cause death

Answer 387

INFANCY Eczema Food allergy PRIMARY SCHOOL YEARS Allergic rhinitis Conjunctivitis Asthma The progression of allergic diseases from an initial diagnosis or, for example, eczema in infancy is called the 'allergic march'

Answer 388

Mouth breathing + history of snoring or obstructive sleep apnoea Allergic salute (rubbing the nose upwards - may be due to nose being itchy) Pale and swollen inferior nasal turbinates Hyperinfalted chest or Harrison sulci from chronic undertreated asthma Atopic eczema affecting the limb flexures Allergic conjunctivitis: may be prominent creases (Denne-Morgan folds), may be blue-grey discolouration below the lower eyelids Growth should be checked: in patients with food alleergy, where dietary restriction of malabsorption can lead to nutritional compromise. In patients being treated with high-dose corticosteroids

Answer 389

Food allergy should be considered in any children who do not respons adequately to treatment for: Atopic eczema GORD Chronic GI symptoms

Answer 390

Certain allergic diseases can be managed by specialists and GPs, however, because allergic diseases can co-exist, it should be considered a systemic disease Paediatricians should help with trigger avoidance and manage children with severe disease ``` Specific allergen immunotherapy can be used to treat: Allergic rhinitis and conjunctivitis Insect stings Anaphylaxis Asthma ``` During immunotherapy, solutions of an allergen to which the patient is allergic are injected SC or sublingulally on a regular basis for 3-5 years, with the aim of developing immune tolerance It can provide protection for many years It has a risk of inducing anaphylaxis so must be done under specialist supervision

Answer 391

A food allergy occurs when a pathological immune response is mounted against a specific food protein It is usually IgE mediated

Answer 392

A non-immunological hypersensitivity reaction to a specific food

Answer 393

Varies depending on age: Infants: most common causes are milk, eggs and peanut Older children: peanut, tree nut, fish, shellfish

Answer 394

Usually due to cross-reactivity between proteins present in fresh fruits/vegetable/nuts and those present in pollens E.g. a child who can eat apples may develop an allergy to apples when they are older if they develop an allergy to birch tree pollen, which has a very similar protein This is called pollen food allergy syndrome and can cause mild allergic reactions (e.g. itchy mouth)

Answer 395

IgE mediated food allergy will present with a history of alergic symptoms varying from urticaria to facial swelling to anaphylaxis This usually occurs 10-15 minutes after ingestion of a food Non-IgE mediated food allergy usually presents with diarrhoea, vomiting, abdominal pain and sometimes faltering growth - Colic or eczema may also be present - It may present with blood in the stools due to proctitis in the first few weeks of life or severe repetitive vomiting which can result in shock (food protein-induced enterocolitis syndrome)

Answer 396

Clinical history is key For IgE mediated food allergy, skin-prick tests are the most useful confirmatory test Patch testing - identify allergens that cause reactions through delayed contact hypersensitivity where T cells play a major role (the allergenic extract is held against the skin for 48 hours) Measurement of IgE antibodies in the blood is also useful Non-IgE mediated food allergies are harder to diagnose - more reliant on clinical history and examination Endoscopy and intestinal biopsy are sometimes performed when indicated The diagnosis of non-IgE mediated food allergy is supported by the presence of eosinophilic infiltrates

Answer 397

Exclusion of the relevant food under the dietician's supervision - This is followed by a double-blind placebo-controlled food challenge, which involves the child being subjected to increasing amounts of the food or placebo - It should be performed at hospital with full resuscitation facilities available

Answer 398

Avoidance of relevant food Educating the child and family about how to manage an allergic attack (allergy action plan) Provide written self-management plans and training Mild reactions (no cardiorespiratory symptoms) are treated with non-sedating antihistamines Severe reactions (with cardiovascular, laryngeal or bronchial involvement) require IM adrenaline (may be given by autoinjector (EpiPen)) Food allergy to cow's milk and egg often resolves in early childhood, so gradual reintroduction may be possible Food allergy to nuts and seafood usually persist through to adulthood

Answer 399

Abnormal reaction by the body's immune system to a protein found in cows' milk

Answer 400

IgE-mediated = immediate reaction | Non-IgE mediated = delayed reaction

Answer 401

GI symptoms: vomiting, abdo pain, blood in stools, diarrhoea Skin symptoms: hives, eczema Babies: wheezing, irritability, facial swelling, poor growth

Answer 402

Consider referral to secondary care for a skin prick and/or specific IgE antibody blood test Implement strict cows' milk elimination diet for at least 6 months or until the child is 9-12 months - Breastfed babies: advise mother to exclude cows' milk protein from her diet. Consider prescribing daily supplement of 1000mg of calcium and 10mcg of vitamin D - Formula-fed babies: advise replacement of cows' milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula) Weaned infants/older children: exclude cows' milk protein from diet Offer nutritional counselling with a paediatric dietician Regularly monitor growth Re-evaluate the child to assess for tolerance to cows' milk protein (every 6-12 months) - this involves re-introducing cows' milk protein into the diet - If tolerance is established, greater exposure of less processed milk is advised following a 'Milk Ladder' SUPPORT: British Dietetic Association has produced a useful tact sheet

Answer 403

Classified based on pattern and severity of symptoms: Pattern= intermittent or persistent Severity = mild, moderate or severe It can also be classfiied as seasonal or perennial

Answer 404

With a cough due to post-nasal drip or chronically blocked nose leading to sleep disturbance

Answer 405

Assess for symptoms of asthma Try to identify the most likely causative allergen Look for signs of chronic nasal congestion (e.g. mouth breathing, cough, halitosis) Examine the nose for nasal polyps, deviated nasal septum, mucosal swelling or depressed or widened nasal bridge For those who want 'as required' treatment for occasional symptoms: If aged 2-5 or preference for oral treatment= oral antihistamine (e.g. cetirizine) All others= intranasal azelastine (antihistamine) For those who want preventative treatment for frequent symptoms: Avoid the causative agent If main issue is nasal blockage or nasal polyps = intranasal corticosteroid (e.g. beclomethasone) If main issue is sneezing or nasal discharge = oral anthistamine or intranasal corticosteroid For people requiring rapid relief whilst waiting for preventive treatment to take effect: Intranasal corticosteroids for up to 7 days Consider adding oral anithistamine If symptoms are severe and/or impairing quality oflife, prescribe a 5-10 day course of prednisolone

Answer 406

Presents as hives or redness and results from local vasodilation and increased permeability of capillaries and venules This is dependent on activation of skin mast cells, leading to the release of various mediators including histamines It is itchy

Answer 407

``` Viral infection (rash lasts for days) Allergen exposure (rash lasts for hours) ```

Answer 408

Urticaria involving deeper tissues to produce swelling (angioedema), especially of the lips and soft tissues around the eyes

Answer 409

When allergy has caused urticaria, there is a risk of anpahylaxis

Answer 410

Usually non-allergic

Answer 411

Identify and manage underlying triggers Symptoms diaries can be useful Consider assessing severity using a tool such as Urticaria Activity Score (UAS7) Advise that urticaria is likely to be self-limiting If symptomatic: -Offer non-sedating antihistamine (e.g. cetirizine) for up to 6 weeks - If severe offer a short course of oral corticosteroid - If symptoms improve, consider giving treatment to take as required (if there were to be future episodes) or daily antihistamines for 3-6 months if symptoms are likely to be persistent Consider referring to a dermatologist In refractory cases, leukotriene receptor antagonists or anti-IgE antibody (omalizumab) may be used

Answer 412

Viral illness may cause a delayed rash, before which antibiotics are often erroneously given to the child (Allergy to antibiotics is common in children)

Answer 413

Bee and wasp stings

Answer 414

Mild: local swelling Moderate: generalised urticaria Severe: symptoms with wheeze or shock Those who have suffered from a severe reaction should be offered an EpiPen and allergen immunotherapy

Answer 415

``` Moderate: Tachypnoea Tachycardia Nasal flaring Use of accessory respiratory muscles Intercostal and subcostal recession Head retraction Inability to feed ``` Severe: Cyanosis Tiring because of increased work of breathing Reduced conscious level Oxygen saturation <92% despite oxygen therapy (NB: monitoring oxygen saturation is useful to detect hypoxaemia and to titrate the amount of additional oxygen required)

Answer 416

Ex-preterm infants with bronchopulmonary dysplasia Haemodynamically significant congenital heart disease or disorders causing muscle weakness CF Immunodeficiency

Answer 417

Caused by extrathoracic airway narrowing | Predominantly inspiratory

Answer 418

Caused by intrathoracic airway narrowing | Predominantly expiratory

Answer 419

A rough inspiratory noise caused by variable partial upper airway obstruction (snoring)

Answer 420

COmmon cold (coryza) Sore throat (pharyngitis, including tonsilitis) Acute otitis media Sinusitis (relatively uncommon)

Answer 421

Difficulty feeding in infants because their noses are blocked Febrile seizures Acute exacerbations of asthma

Answer 422

Mostly viruses: Rhinoviruses Coronaviruses RSV

Answer 423

Reassure that the common cold is self-limiting Symptoms may peak after 2-3 days and they will typically resolve within 2 weeks Encourage rest, adequate fluid intake and a healthy diet Encourage the use of paracetamol or ibuprofen for symptomatic relief

Answer 424

``` Usually due to a viral infeciton: Adenoviruses Enteroviruses Rhinoviruses IN older children, group A b-haemolytic streptococcus is common ``` The pharynx and soft palate become inflamed and local lymph nodes are enlarged and tender

Answer 425

Tonsilitis is a form of pharyngitis characterised by intense inflammation of the tonsil, often with purulent exudate

Answer 426

Group A b-haemolytic streptococci | EBV

Answer 427

It is difficult to distinguish clinically between bacterial and viral tonsilitis However, marked constitutional disturbance (e.g. headache, apathy, abdominal pain, white tonsillar exudate and cervical lymphadenopathy) is more common with bacterial infection

Answer 428

Arrange hospital admission if: Difficulty breathing Clinical dehydration Peri-tonsillar abscess or cellulitis Signs of marked systemic illness or sepsis A suspected rare cause (e.g. Kawasaki disease, diphtheria) Specific cases to look out for: DMARDs- could cause immunodeficiency Carbimazole - can cause idiosyncratic neutropenia NB: take urgent FBC in both cases If bacterial tonsilitis is confirmed using rapid anitgen testing: Phenoxymethylpenicillin If penicillin allergy: clarithromycin ADVICE: Adequate fluid intake Paracetamol or ibuprofen when necessary Salt water gargling, lozenges or anaesthetic sprays (e.g. Difflam) may provide temporary relief of throat pain Children can return to school after fever has resolved and they are no longer feeling unwell and/or after taking antibiotics for 24 hours Patients with recurrent tonsilitis may require referral to ENT To eradicate the organism completely, 10 days of antibiotic treatment is required for pharyngitis and tonsilitis Rarely, children may be admitted to hospital for IV fluids and analgesia if they are unable to swallow solids or liquids

Answer 429

It may cause widespread maculopapular rash if the tonsitlitis is due to infectious mononucleosis

Answer 430

Group A streptococcal infection

Answer 431

Fever usually precedes the presence of headache and tonsilitis by 2-3 days Appearance of rash is variable The typical rash will be sandpaper-like maculopapular rash with flushed cheeks and perioral sparing The tongue is often white and coated and may be sore or swollen

Answer 432

Antibiotics - Phenoxymethylpenicillin (penicillin V) 4/day for 10 days - Azithromycin (if penicillin allergy) Paracetamol or ibuprofen can be given for symptomatic relief Scarlet Fever is a notifiable disease so let PHE centre know Symptoms should settle down after 1 week Stay away from nursery/school for 24 hours after starting antibiotics This is needed to prevent complications such as acute glomerulonephritis and rheumatic fever

Answer 433

Suppurative: otitis media, throat infection/abscess, acute sinusitis/mastoiditis, streptococcal pneumonia, meningitis, endocarditis, osteomyelitis, necrotising fasciitis, toxic shock syndrome Non-suppurative (autoimmune): rheumatic fever (arthritis, carditis, erythema marginatum), streptococal glomerulonephritis

Answer 434

Short, horizontal, poorly functioning Eustachian tubes

Answer 435

Ear pain and fever NB: every child with a fever must have their tympanic membrane examined In acute otitis media, the tympanic membrane is bright red and bulging with loss of the normal light reflex Occasionally, the tympanic membrane can perforate and pus can become visible in the external canal

Answer 436

``` RSV Rhinovirus Pneumococcus H. influenzae Moraxella catarrhalis ```

Answer 437

Mastoiditis | Meningitis

Answer 438

Admit if: Severe systemic infection Complications (e.g. meningitis, mastoiditis, facial nerve palsy) Children < 3 months with a temperature >38 degrees ADVICE: Advise that the usual course of acute otitis media is about 3 days but can last up to 1 week Advise regular doses of paracetamol or ibuprofen for pain There is no evidence to support the use of decongestants or antihistamines Medical management: - No antibiotic prescription: most cases will resolve spontaneously. Advise to seek help if the symptoms have not improved after 3 days or if the child deteriorates clinically - Back-up antibiotic prescription - advise that the antibiotic is NOT needed immediately but should be used if symptoms have not improved after 3 days or if they have worsened significantly at any time - Immediate antibiotic prescription - seek medical help if the symptoms worsen rapidly or the patient becomes systemically unwel (Amoxicillin 5-7 days as first line; clarithromycin or erythromycin can be used if penicillin allergy) NB: antibiotics marginally reduce the duration of pain but have no effect on the risk of hearing loss

Answer 439

Otitis media with effusion, resulting from recurrent ear infections

Answer 440

Children are usually asymptomatic except for possible reduced hearing The eardrum is dull and retracted, often with a fluid level visible This is common in 2-7 year olds Usually resolves spontaneously It may cause conductive hearing loss (most common cause in children) - This can interfere with normal speech development and result in learning difficulties In these children, ventilation tubes (grommets) are often inserted - The benefits do not last longer than 12 months - If problems recur after the extrusion of the grommet, reinsertion of grommets with adjuvant adenoidectomy is often advocated

Answer 441

Viral URTI (occasionally may get secondary bacterial infection)

Answer 442

The frontal sinuses do not develop until late childhood

Answer 443

Refer to hospital if there are symptoms and signs of: Severe systemic infeciton Intraorbital or periorbital problems (e.g. periorbital cellulitis, displaced eyeball, double vision) Intracranial complications (e.g. features of meningitis) Symptoms lasting < 10 days: Do not offer an antbiotic ADVISE: - Acute sinusitis is usually caused by a virus and takes 2-3 weeks to resolve (it can be complicated by a bacterial infection) - Symptoms, such as fever, can be managed using paracetamol or ibuprofen - Some may find some relief using nasal saline or nasal decongestants - Medical advice should be sought if symptoms worsen rapidly, or if they do not improve in 3 weeks or become systemically unwell Symptoms lasting > 10 days - Consider high-dose nasal corticosteroid for 14 days for adults and children > 12 years old (e.g. mometasone) - > may improve symptoms but unlikely to affect duration of illness - > Could cause systemic side-effects - Consider NO antitbiotic prescription or back-up prescription - > antibiotics are unlikely to change the course of the illness - > the back up prescription should be used if symptoms get considerably worse or if it still has not improved by 7 days - > 1st line: phenoxymethylpenicillin (clarithromycin if penicillin allergy) - > 2nd line: co-amoxiclav - Advise patients to seek medical advice if they develop complciations or their symptoms do not improve/worsen (consider alternative diagnoses such as dental infection)- NHS Choices information on sinusitis

Answer 444

``` Recurrent severe tonsilitis Peritonsillar abscess (quinsy) Obstructive sleep apnoea (adenoids will also be removed) ```

Answer 445

Recurrent otitis media with effusion with hearing loss | Obstructive sleep apnoea

Answer 446

COMMON: croup (viral laryngotracheobronchitis) - Mucosal inflammation and swelling can rapidly cause life-threatening obstruction of the airway in young children ``` RARE: Epiglottitis Bacterial tracheitis Laryngeal or oesophageal foreign body Allergic laryngeal angioedema Inhalaiton of smoke and hot fumes in fires Trauma to the throat Retropharyngeal abscess Hypocalcaemia Severe lymph node swelling (TB, infectious mononucleosis, malginancy) Measles Diphtheria Psychological (vocal cord dysfunction) ```

Answer 447

None Only on crying At rest Biphasic It can also be assessed based on the degree of chest retraction: None Only on crying At rest

Answer 448

Tachypnoea Tachycardia Agitation VERY SEVERE: central cyanosis, drooling, reduced level of consciousness

Answer 449

Oxygen saturation by pulse oximetry

Answer 450

Total obstruction of the upper airway can be precipitated by examination of the throat using a spatula, so you should avoid examining the throat of a child with upper airway obstruction unless full resuscitation equipment and personnel are on hand (lying the child down or performing a lateral neck X-ray should also be avoided)

Answer 451

Most common are parainfluenza viruses Other viruses: Rhinovirus RSV Inluenza

Answer 452

1-2 years | age range affected: 6 months - 6 years

Answer 453

Coryza and fever followed by: Hoarseness due to inflammation of the vocal cords A barking cough, like a sea lion, due to tracheal oedema and collapse Harsh stridor Variable degree of breathing difficulty with chest retraction Symptoms often start, and are worse, at night

Answer 454

NB: if upper airway obstruction if mild then the stridor and chest recession will disapper when the child is at rest, so can be managed at home Children <12 months old have a low threshold for hospital admission Categorise the severity: Mild: seal-like barking cough but no stridor or sternal/intercostal recession at rest Moderate: seal-like barking cough with stridor and sternal recession at rest, no agitation and lethargy Severe: seal-like barking cough with stridor and sternal/intercostal recession associated with agitation or lethargy Impending respiratory failure: increasing upper airway obstruction, sternal/intercotal recession, asynchronous chest wall and abdominal movement, fatigue, cyanosis/pallor, reduced consciousness, respiratory rate > 70 IMPORTANT: admit all children with croup that is worse than mild If hospital admission is not required: single dose of oral dexamethasone (0.15mg/kg) to be taken immediately ADVICE: Advise that croup normally resolves after 48 hours Advise to seek medical attention if stridor continues, skin between ribs is pulling in, if the child is restless or agitated Advise to call an ambulance if very pale, blue or grey for more than a few seconds, unusually sleepy or unresponsive, having a lot of trouble breathing pr generally unwell Paracetamol or ibuprofen can be used if the child has a fever and is distressed Advise good fluid intake Advise parents to check the child regularly at night Croup causing chest recession at rest: Oral dexamethasone OR oral prednisolone OR nebulised steroids (budesonide) These will reduce the severity and duration of croup and reduce the need for hospitalisaiton Severe upper airways obstruction: Nebulised adrenaline with oxygen by face mask (causes rapid but transient improvement) Children should be closely monitored

Answer 455

Intense swelling of the epiglottitis and surrounding tissues associated with septicaemia It is an emergency because of the high risk of respiratory obstruction

Answer 456

H. influenzae type b (Hib)

Answer 457

1-6 years NB: massively reduced incidence since introduction of Hib vaccine

Answer 458

Very acute: High fever in a very ill, toxic-looking child An intensely painful throat that prevents the child from speaking or swallowing; saliva drools down the chin Soft inspiratory stridor and rapidly increasing respiratory difficulty over hours The child sitting immobile, upright, with an open mouth to optimise airway

Answer 459

Cough is not a feature of acute epiglottitis, unlike viral croup

Answer 460

If acute epiglottitis is suspected, urgent hospital admission and treatment are required The child should be transferred to the intensive care unit Secure the airway and give supplemental oxygen Take a blood culture Start IV antibiotics (e.g. cefuroxime) In some patients, steroids and adrenaline may be used With appropriate treatment, most children will recover completely within 2-3 days As with other serious H.infleunzae infections, prophylaxis with rifampicin is offered to close household contacts

Answer 461

RARE but dangerous S.aureus Similar presentation to croup in that the child will have a high fever, appear very ill and have rapidly progressive airways obstruction with copious thick airway secretions

Answer 462

IV antibiotics and intubation and ventilation if required

Answer 463

Acute wheeze is due to parital obstruction of the intrathoracic airways This can be form mucosal inflammation and swelling (e.g. bronchiolitis) It can also be due to bronchoconstriction (e.g. asthma, mechanical obstruction)

Answer 464

1-9 months

Answer 465

RSV Remainder of the cases are due to parainfluenza, rhinovirus, adenovirus, influenza virus and human metapneumoniavirus

Answer 466

Coryzal symptoms preceding a dry cough and increasing breathlessness Feeding difficulty due to increasing dyspnoea Recurrent apnoea (SERIOUS complication)

Answer 467

Infants born prematurely who develop bronchopulmonary dysplasia or with underlying lung disease (e.g. CF) or congenital heart disease

Answer 468

``` Dry wheezy cough Tachypnoea and tachycardia Subcostal and intercostal recession Hyperinflation of the chest Fine end-inspiratory crackles High-pitched wheezes (expiratory>inspiratory) ```

Answer 469

``` Pulse oximetry (should be performed on all children with suspected bronchiolitis) CXR or blood gases are ONLY recommended if respiratory failure is suspected ``` ``` Hospital admission needed if: Apnoea Persistent oxygen saturation <90% on air Inadequate oral fluid intake (50-75% of usual volume) Severe respiratory distress: - Grunting - Marked chest recession - Respiratory rate > 70 breaths a minute ```

Answer 470

Humidified oxygen supplementation if saturation is persistently <92% Consider CPAP if impending respiratory failure Consider upper airway suction if there is evidence of increased airway secretions (this should definitely be performed if they are presenting with apnoea) Give fluids by nasogastric or orogastric tube if they cannot take enough by mouth Supportive IMPORTANT: RSV is highly infectious, so infection control measures are needed to prevent cross-infection Most infants will recover within 2 weeks RARELY, the illness may cause permanent damage to the airways (bronchiolitis obliterans)

Answer 471

Palivizumab (monoclonal antibody against RSV) reduces the number of admissions in high-risk preterm infants

Answer 472

Viral episodic wheezing (wheeze only in response to viral infections) Multiple trigger wheeze (wheeze in response to multiple triggers and is more likely to develop into asthma over time) Asthma

Answer 473

Thought to result from small airways being more likely to narrow and obstruct due to inflammation and aberrant immune responses to viral infection

Answer 474

Maternal smoking during and/or after pregnancy | Prematurity

Answer 475

Prescribe a salbutamol inhaler A spacer should be used (improves delivery of the drug) Encourage parents who are smokers to stop Instructions: Check expiry date of inhaler Shake it Insert it into the end of the spacer Administer one puff into the spacer Then ask the child to breathes slowly and deeply through the mouthpiece for 5-10 breaths (10 breaths in children <2 years) NB: if the spacer makes a whistling sound, it means they are breathing too quickly The spacer should be cleaned once per month using warm soapy water Dosing: When the child is wheezy/breathless, give them up to 10 puffs of salbutamol with the spacer up to every 4 hours Safety net: If they do not respond or improve after 10 puffs or they need it again, seek help If your child continues to be wheezy/breathless 48 hours after being discharged from hospital, seek help If they experience symptoms in between viral illness (interval symptoms) they are at increased risk of having asthma If the salbutamol inhaler is ineffective, intermittent LTRA or intermittent ICS or both may be considered

Answer 476

Eczema Rhinoconjunctivitis Food allergy

Answer 477

Asthma should be suspected in any child with wheezing on more than one occasion Wheezing should be described as "a whistling in the chest when your child breaths out" On auscultation, an asthmatic wheeze is a polyphonic noise coming from the airways, due to the presence of many airways of different sizes Symptoms worsen at night and in the early morning Symptoms that have non-viral triggers Interval symptoms (ie. symptoms between acute exacerbations) Personal or family history of atopic disease Positive response to asthma therapy Long-standing asthma may cause hyperinflation of the chest and generalised polyphonic expiratory wheeze with a prolonged expiratory phase Onset of the disease in early childhood can result in Harrison sulci (depressions at the base of the thorax associated with the muscular insertion of the diaphragm)

Answer 478

If < 5 years old, the diagnosis is clinical If > 5 years old, offer/consider - Spirometry: FEV1/FVC < 70% of expected is a positive result - Bronchodilator reversibility: consider in children with a positive spirometry result. An imporovement in FEV1 of > 12% if considered positive. - Peak expiratory flow variability: consider peak flow monitoring for 2-4 weeks in children if there is diagnostic uncertainty - FeNO test: consider if there is diagnostic uncertainty after initial assessment. A level >35 ppb is considered a positive test (this is a measure of airway inflammation) Skin-prick testing for common allergens may be performed (helps identify triggers and demonstrate atopy)

Answer 479

1) Offer a SABA (e.g. salbutamol) as a reliever therapy 2) consider an 8-week trial of paediatrics moderate dose ICS in children with: symptoms at presentation that clearly indicate the need for maintenance therapy (e.g. asthma-related symptoms > 3 times per week), or suspected asthma that is uncontrolled by SABA alone 3) After 8 weeks, stop ICS treatment and continue to monitor the child's symptoms: - If symptoms did not resolve, consider an alternative diagnosis - If symptoms resolved but then recurred within 4 weeks of stopping ICS, restart paedaitrics ICSM low dose as first line - If symptoms resolved but recurred beyond 4 weeks after stopping ICS, repeat the 8-week trial of paediatric moderate dose ICS 4) if suspected asthma is uncontrolled on paediatric low dose ICS as maintenance therapy, consider an LTRA (e.g. montelukast) in addition to the ICS 5) if still uncontrolled despite the preceding steps, stop the LTRA and refer the child to a healthcare professional with expertise in asthma

Answer 480

Raised ICP Coagulopathy Infection at site of LP Meningococcal septicaemia

Answer 481

``` Give high flow oxygen Obtain IV/IO access and take blood cultures (and blood gas, lactate, glucose) Give IV/IO antibiotics Consider fluid resuscitation Involve senior clinicians early Consider ionotropic support early ```

Answer 482

1) SABA (e.g. salbutamol) to be used as a reliever 2) Offer paediatric low dose ICS as first-line maintenance therapy to children with symptoms at presentation that clearly indicate the need for maintenance therapy (e.g. asthma-related symptoms > 3 times per week), or suspected asthma that is uncontrolled by SABA alone 3) Consider an LTRA in addition to the ICS and review in 4-8 weeks 4) Consider stopping the LTRA and starting a LABA 5) Consider changing the ICS and LABA maintenance therapy to a MART regimen, with a paediatric low maintenance ICS dose 6) Consider increasing ICS to a paediatric moderate maintenance dose 7) Refer to specialist care (they may consider using paediatric high dose ICS or a trial of an additional drug (e.g theophylline)

Answer 483

Assess patient's baseline asthma status (can be done using Asthma Control Questionnaire or a lung function test (e.g. spirometry)) Provide self-management education and a personalised asthma action plan (available from Asthma UK) Ensure child is up to date with routine immunisations Provide information about sources of support (Asthma UK) Advise about trigger avoidance (specific allergens, smoke, beta-blockers, NSAIDs) NOTE: parents should be advised to stop smoking Assess for the presence of anxiety and depression Ensure that the patient has their own peak flow meter Explain how to use inhalers

Answer 484

In severe persistent asthma that is unresponsive to conventional treatment, oral prednisolone (given on alternate days) may be used Anti-IgE therapy (omalizumab) is an injectable monoclonal antibody that acts against IgE (mediator of allergy) Antihistamines and nasal steroids may be useful in the treatment of allergic rhinitis

Answer 485

Absence of daytime or night-time symptoms, no limit on activities (including exercise), no need for reliever use, normal lung function and no exacerbations (need for hospitalisation or oral steroids) in the previous six months

Answer 486

Confirm adherence to medication Review inhaler technique Review if treatment needs to be changed Ask about occupational asthma and triggers

Answer 487

Inhaled beta-2 agonists are the most commonly used and most effective bronchodilator SABAs (e.g. salbutamol, terbutaline) have a rapid onset of action (maximum effects after 10-15 mins): Furthermore, they are effective for 2-4 hours and have few side-effects SABAs are used as required (PRN) ``` LABAs (e.g. salmeterol, formoterol): Effective for 12 hours NOT used in acute asthma Should NOT be used without an inhaled corticosteroid Useful in exercise-induced asthma ``` Ipratropium bromide: Anticholinergic bronchodilator Sometimes given to young infants if other bronchodilators are ineffective or to treat severe acute asthma

Answer 488

Inhaled Corticosteroids: Most effective prophylactic therapy They decrease airway inflammation leading to reduced symptoms, decreased frequency of asthma exacerbations and reduced bronchial hyperactivity They are often used in conjunction with an inhaled LABA or leukotriene receptor antagonist If used at low-doses, there are no serious side effects NOTE: it can cause a mild reduction in height velocity, however this is followed by catch-up growth in later childhood Possible systemic side-effects with high-dose use: Impaired growth Adrenal suppression Altered bone metabolism To avoid these side-effect, very low doses should be used Add-on Therapy 1st choice add-on therapy in: Children > 5 years = LABA Children < 5 years = leukotriene receptor antagonist (e.g. montelukast) NOTE: LRAs may also be used in older children if LABA fails to control symptoms Alternative add-on: slow-release oral theophylline NOTE: this has a high incidence of side-effects( e.g. vomiting, insomnia, headaches, poor concentration)

Answer 489

If, after high-dose inhaled bronchodilator therapy, the child is still: Not responded adequately clinically (ie. persisting breathlessness, tachypnoea) Becoming exhausted Still have a marked reduction in predicted peak flow rate or FEV1 (<50%) The child should be admitted

Answer 490

- Moderate= PEFR 50-75%, Normal speech - Severe = PEFR 33-50%, RR >25 (>12 years), RR > 30 (5-12 years), RR>40 (2-5 years) HR >110 (>12 years), HR > 125 (5-12 years), HR > 140 (2-5 years) Inability to complete sentences in one breath Accessory muscle use Inability to feed SpO2 > 92% - Life-threatening = PEFR <33% SpO2 < 92% Altered consciousness Exhaustion Cardiac arrhythmia Hypotension Cyanosis Poor respiratory effort Silent chest

Answer 491

``` Determine the severity of asthma: Examine the patient Measure peak flow if possible Measure oxygen saturation Measure pulse rate and respiratory rate Assess for signs of exhaustion, cyanosis and increased respiratory effort ``` Admit everyone with severe or life-threatening asthma Management whilst awaiting admission to hospital: Give supplemental oxygen to all hypoxic patients (Use Venturi mask or nasal cannulae; Adjust flow rate to maintain sats 94-98%) Treat with SABA: - Life-threatening asthma: nebulised salbutamol (ideally should be oxygen-driven= 5mg if>5 years, 2.5mg if 2-5 years)- if a nebuliser is unavailable, use a pressurised MDI with a large volume spacer If the SABA was ineffective, consider ipratropium bromide (30-40 mg if >5 years, 20 mg if 2-5 years, 10 mg if < 2 years) Monitor PEFR and oxygen saturation Follow-up: Follow up within 2 working days of discharge

Answer 492

Use SABA with a large-volume spacer to relieve acute symptoms - 1 puff ever 30-60 seconds up to 10 puffs - 5 tidal breaths should be taken per puff Prescribe a short course of oral prednisolone (3-7 days) Consider antibiotics if there is clinical suspicion of bacterial infection Once symptoms have subsided, advise patient to return to using their SABA PRN up to 4 times a day, not exceeding 4-hourly The dose of ICS does not need to be altered and ICS should not be used as an alternative for oral prednisolone Advise patients to monitor PEFR and to seek help if symptoms worsen/PEFR decreases Consider initiating motelukast in children > 2 years Follow-up within 48 hours of presentation

Answer 493

Magnesium sulphate has few side-effects and is considered the first choice IV agent Aminophylline needs to be infused slowly, as rapid infusion can cause seizures, sever vomiting and fatal cardiac arrhytmias IV salbutamol Antibiotics if there is clinical suspicion of bacterial infection NB: ECG should be monitored and blood electrolytes checked when using aminophylline and salbutamol

Answer 494

Before discharge, the following should be reviewed with the child and family: When drugs should be used (regularly or PRN) How to use the drug (inhaler technique) What each drug does (relief vs prevention) How often and how much can be used (frequency and dosage) What to do if asthma worsens (a written personalised asthma management action plan should be compiled) Inform the parents and child about features of poorly controlled asthma (e.g. cough, wheeze, breathlessness, difficulty walking/talking/sleeping, decreasing relief from bronchodilators) Measurement of peak flow at home will allow earlier recognition of deteriorating asthma

Answer 495

Growth and nutrition Peak flow/spirometry Chest for: Hyperinflation Harrison's sulcus Wheeze Are there other allergic disorders? Allergic rhinitis Eczema, etc. ``` If there is: Sputum Finger clubbing Growth failure — If present, other causes should be sought ``` ``` Monitor: Peak flow diary Severity and frequency of symptoms Exercise tolerance Interference with life, time off school Is sleep disturbed? Use of preventer and reliever medication — are they appropriate? Inhaler technique ``` ``` Consider triggers: Allergic rhinitis needing treatment? Allergens - animal dander, etc. Stress ```

Answer 496

Atypical pneumonia: Mycoplasma, Chlamydia and adenovirus pneumonia can cause wheezing Foreign body inhalation: Abrupt onset cough followed by wheeze in a previously well child is suggestive of foreign body inhalation CXR performed during expiration will show persistent hyperinflation of the lung distal to the obstruction Eventually, airway swelling causes complete obstruction and lobar collapse is seen Anaphylaxis: Suspect if acute urticaria, facial swelling, stridor or previous reaction to allergen

Answer 497

``` Conscious: Encourage coughing External manoeuvres Back blows Chest thrusts for infants Abdominal thrusts (Heimlich) - NOTE: these should NOT be done on infants and very young children ``` Removal of foreign body - Flexible bronchoscopy OR rigid bronchoscopy - > Rigid bronchoscopy is preferred in cases of stridor, asphyxia, radio-opaque object seen on CXR, a history of foreign body aspiration associated with unilateral decreased breath sounds, localising wheeze, obstructive hyper-inflation or atelectasis - > In all other cases, flexible bronchoscopy should be performed to confirm the diagnosis - 2nd line: surgery, thoracotomy Unconscious: Secure the airway IMMEDIATELY (endotracheal intubation) - Unless the foreign body can be seen and removed from the upper airway Removal of foreign body (as above)

Answer 498

A week of coryza (catarrhal phase) Followed by the development of a characteristic paroxysmal or spasmodic cough followed by a characteristic inspiratory whoop (paroxysmal phase) Spasms of cough are often worse at night and may cause vomiting During the paroxysm, the child goes red or blue in the face and mucus flows from the nose and mouth NB: the whoop may not be present in infants Nosebleeds and sunconjunctival haemorrhages can occur after vigorous coughing The paroxysmal phase can last up to 3 months The symptoms will eventually decrease (convalescent phase)

Answer 499

Pneumonia Seizures Bronchiectasis NB: infants and young children suffering from severe spasms of cough or cyanotic attacks should be admitted to hospital and isolated from other children

Answer 500

Organism can be identified from culture of a perinasal swab PCR is more sensitive Serology There is marked lymphocytosis

Answer 501

Admit if: <6 months or acutely unwell Significant breathing difficulties (e.g. apnoea, severe paroxysms, cyanosis) Significant complications (e.g. seizures, pneumonia) - inform the hospital about need to isolate Pharmacological treatment - if admission is not needed, prescribe an antibiotic if the onset of the cough is within 21 days (macrolide antibiotic is firs-line) - < 1 month = clarithromycin 1+ months/non-pregnant adult = azithromycin Pregnant adult = erythromycin (recommended from 36 weeks gestation to reduce risk of transmission to the newborn) - Co-amoxiclav can be used if macrolides are contraindicated or not tolerated (however, this is not allowed in pregnant adults or babies < 6 weeks) Advice: Advise rest, adequate fluid intake and the use of paracetamol or ibuprofen for symptomatic relief Inform the parents that, despite antibiotic treatment, the disease is likely to cause a protracted non-infectious cough that may take weeks to resolve Advise that children should avoid nursery until 48 hours of appropriate antibiotic treatment has been completed or until 21 days after the onset of the cough if it was not treated Once the acute illness has been dealt with, advise parents to complete any outstanding immunisations Siblings, parents and school contacts may have developed a similar cough, and close contacts should receive macrolide prophylaxis Immunisation reduces the risk of developing pertussis but does not provide absolute protection Re-immunisation of mothers during pregnancy reduces the risk of pertussis in her infant during the first few months of life (this is when it is particularly dangerous)

Answer 502

Most commonly caused by a series of respiratory tract infections Other causes: Following specific respiratory infections (e.g. pertussis, RSV, Mycoplasma) Asthma Persistent lobar collapse following pneumonia Suppurative lung diseases (e.g. cystic fibrosis, ciliary dyskinesia, immune deficiency) Recurrent aspiration (with or without GORD) Persistent bacterial bronchitis Inhaled foreign body Cigarette smoking (active or passive) Tuberculosis Habit cough Airway anomalies (e.g. tracheo-bronchomalacia, tracheo-oesophageal fistula)

Answer 503

A cough that lasts more than 8 weeks or one that has not improved after 3-4 weeks should be considered persistent in the absence of a recurrent URTI NB: in any child with a severe, persistent cough, TB should be considered

Answer 504

Viruses in younger children, bacteria in older children Newborn: Organisms from the mother's genital tract Particularly group B streptococcus Other causes include Gram-negative enterococci and bacilli Infants and Young Children: Respiratory viruses (particularly RSV) Bacterial causes include S. pneumoniae and H. influenzae Other, less common, causes include Bordatella pertussis or Chlamydia trachomatis Staphylococcus aureus is a RARE but SERIOUS cause of pneumonia Children > 5 years: Mycoplasma pneumoniae Streptococcus pneumoniae Chlamydia pneumoniae AT ALL AGES, Mycobacterium tuberculosis should be considered

Answer 505

``` Fever Cough Rapid breathing Lethargy Poor feeding Unwell child NB: localised chest, abdominal or neck pain is a feature of pleural irritation and suggests bacterial infection ``` Tachypnoea Nasal flaring Chest indrawing Increased respiratory rate is the most sensitive clinical sign of pneumonia in children O2 sats may be decreased (in young children, the classical pneumonia signs of consolidation with dullness on percussion, decreased breath sound and bronchial breathing are often absent)

Answer 506

CXR may confirm the diagnosis, but cannot distinguish between bacterial and viral A pleural effusion may be seen in some patients Some of these effusions may develop into empyema

Answer 507

Determine severity: Measure temperature Examine chest Record BP, HR and RR Note the degree of agitation and consciousness Note signs of exhaustion, cyanosis and accessory muscle use Assess hydration status (capillary refill, examining skin turgor, dry mucous membranes and urine output) Immediately REFER for hospital admission if: Persistent SpO2 < 92% on air Grunting, marked chest recession, RR > 60/min Cyanosis Child looks seriously unwell, does not wake, or does not stay awake if roused or does not respond to normal social cues Temperature > 38 degrees in a child < 3 months Consider admission if: dehydration, decreased activity, nasal flaring, predisposing diseases (e.g. chronic lung disease) Whilst awaiting hospital admission give controlled supplemental oxygen if SpO2 < 92% If hospital admission is NOT needed: Most children can be managed at home Prescribe antibiotics - IMPORTANT: all children with a clinical diagnosis of pneumonia should receive antibiotics because bacterial and viral pneumonia cannot be reliably distinguished from each other - NOTE: children < 2 yrs presenting with mild respiratory tract symptoms do NOT usually have pneumonia and do not usually require antibiotics - AMOXICILLIN is first-line for 7-14 days - Alternatives: co-amoxiclav, cefaclor or macrolides (e.g. erythromycin) - Macrolides can be added at any stage if there is no response to first-line treatment Advice: Paracetamol or ibuprofen if the child is distressed by the fever (should NOT be given simultaneously but may be interchanged) Advise adequate fluid intake Advise parents not to smoke at home Check on the child regularly through day and night Seek medical advice if they are unable to cope or the child's condition deteriorates (e.g. signs of increased respiratory effort/distress, reduced fluid intake, reduced responsiveness, worsening or unresolving fever) Small effusions may occur in some children but most will resolve with appropriate antibiotics However, a persistent fever despite 48 hours of antibiotics suggests a pleural collection that required drainage (with ultrasound guidance) NOTE: patients with evidence of lobar collapse or atelectasis should have a repeat CXR after 4-6 weeks to check whether the lung fields look normal

Answer 508

H. infleunzae Moraxella catarrhalis May be a precursor to bronchiectasis Bacterial growth from the sputum or bronchial lavage will confirm the diagnosis

Answer 509

Persistent bacterial bronchitis, where there is persistent inflammation of the lower airways due to chronic infection, is an increasingly recognised cause of chronic wet cough in children High-dose anitbiotics (e.g. co-amoxiclav) Physiotherapy

Answer 510

It can be generalised or restricted to a lobe ``` Generalised bronchiectasis can be due to: Cystic fibrosis Primary ciliary dyskinesia Immunodeficiency Chronic aspiration ``` Focal bronchiectasis can be caused by: Previous severe pneumonia Congenital lung abnormality Obstruction by a foreign body

Answer 511

Chest CT Bronchoscopy may be used in focal bronchiectasis to exclude a structural cause

Answer 512

Exercise and improved nutrition Airway clearance therapy (postural drainage, percussion, vibration, use of oscillatory devices) Inhaled bronchodilator (e.g. salbutamol) Inhaled hyperosmolar agent (e.g. hypertonic saline) Long-term oral macrolide (azithromycin) Lung transplantation or resection of the bronchiectatic areas

Answer 513

CF is a multisystem disorder resulting from abnormal ion transport across epithelial cell membranes This leads to a reduction in the airway surface liquid layer and impaired ciliary function and retention of mucopurulent secretions This will, in turn, result in infection by Pseudomonas aeruginosa Defective CFTR also leads to dysregulation of inflammation and defence against infection In the intestines, thick viscid meconium is produced, leading to meconium ileus in 10-20% of infants Meconium ileus is when an inspissated meconium causes intestinal obstruction This presents with vomiting, abdominal distension and failure to pass meconium in the first few days of life Surgery is usually required, but gastrografin enema may relieve the obstruction Pancreatic ducts can also get blocked by thick secretions leading to pancreatic enzyme deficiency and malabsorption Abnormal function of sweat glands leads to excessive sodium and chloride concentrations in the sweat

Answer 514

ALL NEWBORN INFANTS born in the UK are screened for CF Immunoreactive trypsinogen (IRT) is raised in newborn infants with CF and is measured in the routine heel-prick test - Samples with a raised IRT will be screened for common CF gene mutations - Infants with two mutations will have a sweat test to confirm the diagnosis MOST CHILDREN are identified through screening However, some may present clinically with different features depending on age: Newborn: Diagnosed though newborn screening Meconium ileus ``` Infancy: Prolonged neonatal jaundice Growth faltering Recurrent chest infections Malabsorption, steatorrhoea ``` ``` Young Child: Bronchiectasis Rectal prolapse Nasal polyp Sinusitis ``` Older Child and Adolescent: Allergic bronchopulmonary aspergillosis Diabetes mellitus Cirrhosis and portal hypertension Distal intestinal obstruction (meconium ileus equivalent) Pneumothorax or recurrent haemoptysis Sterility in males Chronic infection can also occur with specific bacteria - initially S. aureus with H. influenzae and subsequently with P. aeruginosa or Burkholderia species This will lead to damage to the bronchial wall, bronchiectasis and abscess formation The child will have a persistent, wet cough, productive of purulent sputum EXAMINATION: Hyperinflation of the chest due to air trapping Coarse inspiratory crackles Expiratory wheeze Finger clubbing (with established disease) 95% will die of respiratory failure 90% of children with CF will have pancreatic exocrine insufficiency This result in maldigestion and malabsorption If untreated, this can lead to faltering growth It also causes frequent large, pale and greasy stools (steatorrhoea) Pancreatic insufficiency can be diagnosed by demonstrating low faecal elastase

Answer 515

``` Underweight Meconium ileus Fat-soluble vitamin deficiency DIOS Male infertility Upper airway complications (e.g. nasal polyps) Chronic liver disease Diabetes Reduced BMD ```

Answer 516

CF can be diagnosed based on: Positive test result in people with no symptoms, for example infant screening (blood spot immunoreactive trypsin test) followed by sweat and gene tests for confirmation Clinical manifestations, supported by sweat or gene test results for confirmation Clinical manifestations alone (RARE) In CF, the concentration of chloride in sweat is markedly elevated 60-125 mmol/L in CF 10-40 mmol/L in normal children Sweating is stimulated by applying a low-voltage current to pilocarpine applied to the skin NOTE: diagnostic errors are common if an inadequate volume of sweat is collected Confirmation of the diagnosis can be made by testing for gene abnormalities in the CFTR

Answer 517

Service Configuration: Care should be provided by a specialist cystic fibrosis multidisciplinary team based at a specialist cystic fibrosis centre Patients should have annual review of their condition AND at least one other review per year by the specialist cystic fibrosis MDT, in addition to reviews by local paediatric teams ``` Members of the MDT (all should be specialists in CF): Paediatrician Nurses Physiotherapists Dieticians Pharmacists Clinical psychologists Social worker (provide support for people with CF and their families regarding employment, education, help adjusting to the condition, benefits, respite care) ``` RESPIRATORY Management: Pulmonary Monitoring - Review CHILDREN every 8 weeks - Review ADULTS every 3 months At each review: Perform a clinical assessment (including history, physical examination and exploration of medication adherence) Measure SpO2 Take respiratory secretion samples for investigation Spirometry Airway Clearance Techniques: Offer training for parents and carers (involve physiotherapist) These techniques should be done at least twice per day Regularly assess effectiveness and technique Consider non-invasive ventilation in patients who are unable to sufficiently clear their airways using conventional techniques Mucoactive Agents: Offer in patients with CF who have clinical evidence of lung disease First-line: rhDNase If clinical response with rhDNase is inadequate, consider BOTH rhDNase AND hypertonic saline Consider mannitol dry powder inhalation for children who cannot use the above agents New Agents: Lumacaftor and Ivacaftor are new agents known as potentiators and correctors that may be effective in treating CF caused by the F508 mutation Regular exercise is beneficial and should be encouraged ``` INFECTION Management: Common infections in CF patients: Staphylococcus aureus Pseudomonas aeruginosa Burkholderia cepacia complex Haemophilus influenzae Non-tuberculous mycobacteria Aspergillus fumigatus ``` Often, continuous prophylactic antibiotics (usually flucloxacillin) are recommended , with additional rescue oral antibiotics for any increase in respiratory symptoms or decline in lung function Persistent symptoms require prompt and vigorous IV therapy to limit lung damage, usually given for 14 days via a PIC line Chronic Pseudomonas infection is associated with a more rapid decline in lung function - This can be slowed by the use of daily nebulised anti-pseudomonal antibiotics Regular azithromycin (macrolide) decreases respiratory exacerbations, probably due to an immunomodulatory effect Bilateral sequential lung transplantation is the only therapeutic option for end-stage CF lung disease NUTRITIONAL Management: Oral enteric-coated pancreatic replacement therapy (with meals and snacks to account for pancreatic insufficiency) High-calorie diet (recommended intake is 150% of normal) To achieve this, overnight feeding via a gastrostomy may be used Most patients need fat-soluble vitamin supplements PSYCHOLOGICAL Management: Review the mental health and wellbeing of the patient and their family members Support: Cystic Fibrosis Trust TEENAGERS and ADULTS Management: As the life expectancy of CF has increased, other complications have come to the forefront Diabetes mellitus is increasingly common due to decreasing pancreatic endocrine function About 1/3 of adolescent patients will have evidence of liver disease, with hepatomegaly on liver palpation, abnormal LFTs or an abnormal ultrasound Regular ursodeoxycholic acid to improve flow of bile may be useful RARELY, the liver disease could progress to cirrhosis, portal hypertension and liver failure Liver transplant may be necessary Distal Intestinal Obstruction Syndrome (DIOS): - Viscid mucofaeculent material obstructs the bowel - Usually cleared by a combination of oral laxatives As the disease progresses in adolescents and adults, there may be an increase in chest infections, as well as complications such as pneumothorax and life-threatening haemoptysis Females have normal fertility and should be able to tolerate pregnancy well Males are virtually always infertile but can father children through intracytoplasmic sperm injection The psychological impact of this disease is immense, so the CF team should provide emotional and psychological support

Answer 518

``` The development of CFTR potentiators (ivacaftor) and CFTR correctors (Lumicaftor) have potential to improve outcomes in CF Potentiators help restore function of CFTR in class III and IV mutations Correctors partially restory CFTR numbers in class II defects ```

Answer 519

Congential abnormality in the structure or function of cilia lining the respiratory tract, resulting in impaired mucociliary clearance Affected children will have recurrent infections of the upper and lower respiratory tracts which, if untreated, can lead to severe bronchiectasis

Answer 520

Recurrent productive cough Purulent nasal discharge Chronic ear infections 50% of PCD patients will have dextrocardia and situs inversus (Kartagener syndrome)

Answer 521

Diagnosis is reached following examination of the structure and function of cilia from the nasal epithelial cells brushed from the nose

Answer 522

Daily physiotherapy Proactive treatment of infections with antibiotics Appropriate ENT follow-up

Answer 523

IgG deficiency --> polysaccharide-capsulated bacteria (e.g. S. pneumoniae) Cell-mediated immunodeficiencies --> opportunistic infections (e.g. Pneumocystis jirovecii and fungi) Neutrophil-killing defects --> staphylococcal infection

Answer 524

During REM sleep, the control of breathing becomes unstable and relaxation of voluntary muscles in the upper airway and chest make upper airway collapse likely

Answer 525

Airway obstruction Central hypoventilation Or a combination of both

Answer 526

Loud snoring Pauses in breathing (apnoea) Restlessness Disturbed sleep

Answer 527

Excessive daytime sleepiness or hyperactivity Learning and behaviour problems Faltering growth Pulmonary hypertension (in severe cases)

Answer 528

Adenotonsillar hypertrophy

Answer 529

Neuromuscular disease (e.g. Duchenne muscular dystrophy) Craniofacial abnormalities (e.g. Pierre Robin sequence) Dystonia of upper airway muscles (e.g. cerebral palsy) Severe obesity Down syndrome (causes anatomical upper airway restriction as well as hypotonia meaning that these patients are at particular risk)

Answer 530

Overnight pulse oximetry (most basic assessment that can be performed at home - allows quantification of the frequency and severity of periods of desaturation) Polysomnography is required for more complex cases (this should monitor heart rate, respiratory effort, airflow, CO2 measurement and video recording)

Answer 531

Children with adenotonsillar hypertrophy may need adenotonsillectomy which usually causes a dramatic improvement in symptoms Other children may benefit from CPAP or BiPAP to maintain their upper airway at night NB: congential central hypoventilation syndrome is a rare condition resulting in disordered central control of breathing as well as autonomic dysfunction

Answer 532

At birth, the skin is covered by a chalky-white greasy coat, called the vernix caseosa

Answer 533

Uncommon but potentially serious blistering form of impetigo

Answer 534

S. aureus Treated with systemic antibiotics (e.g. flucloxacillin)

Answer 535

Moles Large congenital naevi are benign but can be disfiguring Melanocytic naevi becomes increasingly common as children get older

Answer 536

Positive family history Having large numbers of melanocytic naevi Fair skin Repeated episodes of sunburn Living in a hot climate with chronic skin exposure to the sun

Answer 537

Due to a defect in biosynthesis and distribution of melanin

Answer 538

Oculocutaneous Ocular Partial

Answer 539

Failure to develop a fixation reflex

Answer 540

Failure to develop fixation reflex Pendulum nystagmus Photophobia (causes constant frowning) Albinism is an important cause of severe visual impairment

Answer 541

Correction of refractive errors and tinted contact lenses may be helpful Their pale skin is prone to sunburn and skin cancer, so hats should be worn and high-factor sun cream should be applied

Answer 542

RARE group of genetic conditions characterised by blistering of the skin and mucous membranes Autosomal dominant variants are milder and autosomal recessive variants may be severe or fatal

Answer 543

Can occur spontaneously or following minor trauma

Answer 544

In severe cases, the fingers and toes may become fused and contractures of the limbs develop from repeated blistering and healing

Answer 545

Based on avoiding minor injury and treating secondary infection

Answer 546

Oral ulceration and stenosis from oesophageal erosions

Answer 547

Rare manifestation of the inherited ichthyoses (a group of conditions in which the skin is dry and scaly)

Answer 548

Infants are born with a taut, shiny, parchment-like or collodion-like membrane

Answer 549

There is a risk of dehydration

Answer 550

Emollients are applied to moisturise and soften the skin

Answer 551

``` Common: Irritant (contact) dermatitis (most common) Infantile seborrheic dermatitis Candida infection Atopic eczema ``` Rare: Acrodermatitis enterohepatica Langerhans cell histiocytosis Wiskott-Aldrich syndrome

Answer 552

THis can occur if the nappy is not changed frequently but it can also happen even with regular cleaning of the nappy area It tends to be due to the irritant effects of urine on the skin of susceptible infants The flexures tend to be spared in irritant dermatitis causing nappy rash (this distinguishes it from other causes of nappy rash) The rash is erythematous and may have a scalded appearance

Answer 553

Mild cases respond to the use of protective emollient | More severe cases may require topical corticosteroids

Answer 554

It includes the flexures and may cause satellite lesions

Answer 555

Topical antifungal agent

Answer 556

Advise the parents/carers about self-management strategies: Consider using a nappy with high absorbency and ensure that it fits properly Leave nappy off as much as possible to help skin drying of the nappy area Clean the skin and change the nappy every 3-4 hours or as soon as possible after wetting/soiling, to reduce skin exposure to urine and faeces Use water, or fragrance-free or alcohol-free baby wipes Dry gently after cleaning Bath the child daily Do NOT use soap, bubble bath, lotions or talcum powder ADVICE: NHS choices leaflet on nappy rash If mild erythema and the child is asymptomatic Advise on the use of barrier preparation to protect the skin (available OTC) Apply thinly at each nappy change Options: Zinc and Castor oil ointment BP, Metanium ointment, white soft paraffin BP ointment If the rash appears inflamed and is causing discomfort If > 1 month = hydrocortisone 1% cream OD (max 7 days) If the rash persists and CANDIDAL INFECTION is suspected or confirmed on swab Advise against the use of barrier protection Prescribe topical imidazole cream (e.g. clotrimazole, econazole, miconazole) Frequency depends on preparation used If the rash persists or BACTERIAL INFECTION is suspected or confirmed on swab Prescribe oral flucloxacillin for 7 days If penicillin allergy: clarithromycin (7 days) Arrange to review the child

Answer 557

Tends to present in the first 3 months of life It tends to start on the scalp as an erythematous scaly eruption The scales form a thick yellow adherent layer, commonly called cradle cap The rash may spread to the face and then extend to the flexures and nappy area Unlike atopic eczema, this rash is not itchy and the child does not seem to be bothered by it

Answer 558

It is associated with a subsequent risk of developing atopic eczema

Answer 559

Reassure the parents (it is NOT a serious condition) It will spontaneously resolve over a few weeks/months If scalp is affected, advise: Regular washing of the scalp with baby shampoo, followed by gentle brushing with a soft brush to loosen scales and improve the condition of the skin Softening the scales with baby oil first, followed by gentle brushing, then washing off with baby shampoo Soaking the crusts overnight with white petroleum jelly or slightly warmed vegetable/olive oil, and shampooing in the morning Emulsifying ointment can be used if these measures don’t work If conservative measures are ineffective, prescribe topical imidazole cream (e.g. clotrimazol, econazole, meconazole) Use 2-3 times per day (depending on preparation) until symptoms disappear Consider specialist advice if it lasts > 4 weeks If other areas of skin are affected, advise bathing the infant at least once per day using an emollient as a soap substitute

Answer 560

Clinical diagnosis NB: if the disease is unusually severe, atypical or associated with unusual infections or faltering growth, an immune deficiency should be excluded

Answer 561

Itching (pruritus) is the main symptom at all ages - This leads to scratching and exacerbation of the rash - Excoriated areas become erythematous, weeping and crusted Distribution of the eruption changes with age Atopic skin is usually dry Prolonged scratching and rubbing can lead to lichenification (accentuation of normal skin markings)

Answer 562

Causes of exacerbations of asthma: Bacterial infection (usually Staphylococcus or Streptococcus) - Staphylococcus aureus, in particular, thrives on atopic skin and releases superantigens, which seem to worsen eczema Viral infection - HSV infection, although rare, is potentially very serious because it can spread rapidly on atopic skin causing an extensive vesicular reaction called eczema herpeticum Ingestion of an allergen (e.g egg) Contact with an irritant or allergen Environment (heat, humidity) Change or reduction in medication Psychological stress Unexplained Regional lymphadenopathy is common in active eczema and resolves when the skin improves

Answer 563

CLEAR - normal skin with no evidence of active eczema MILD - areas of dry skin and infrequent itching MODERATE - areas of dry skin, frequent itching and redness (with/without excoriation and localised skin thickening) SEVERE - widespread areas of dry skin, incessant itching and redness (with/without excoriation and localised skin thickening) INFECTED - eczema is weeping, crusting or there are pustules with fever and malaise IMPORTANT: remember to assess the psychological impact of eczema on the child Consider using questionnaires such as the Children's Dermatology Life Quality Index (CDLQI)

Answer 564

Prescribe generous amounts of emollients and recommend frequent and liberal use Consider prescribing a mild corticosteroid (e.g. hydrocortisone 1%) for areas of red skin This should be continued for 48 hours after the flare has been controlled Routine follow-up is not normally necessary Refer for a routine dermatology appointment if the diagnosis is uncertain, current management has failed to control eczema, there is facial eczema or there is a recurrent secondary infection

Answer 565

(emollients are used in all types of eczema) Prescribe a moderately potent topical steroid: betamethasone valerate 0.025% or clobetasone butyrate 0.05% Treatment should be continued for 48 hours after the flare has been controlled If there is severe itching or urticaria, consider prescribing a 1-month trial of a non-sedating antihistamine (e.g. cetirizine, loratidine, fexofenadine) Consider prescribing a maintenance regimen of topical corticosteroids to control areas of skin prone to frequent flares (not face, genitals or axillae) 2nd line: topical calcineurin inhibitors (e.g. tacrolimus) - only prescribed by a specialist Review regularly if the child is young and using a large amount of topical corticosteroids

Answer 566

(emollients are used in all types of eczema) Prescribe a potent corticosteroid: betamethasone valerate 0.1% to be used on inflamed areas - For delicate areas of inflamed skin (e.g. face and flexures) use a moderate potency steroid Occlusive dressings or dry bandages may be useful Consider non-sedating antihistamine if there is severe itching If the itching is affecting sleep, consider using a sedating antihistamine (e.g. chlorphenamine) If there is SEVERE, extensive eczema causing psychological distress, consider a course of oral corticosteroids Prescribe a maintenance regimen of topical corticosteroids

Answer 567

Swab the infected skin 1st line: flucloxacillin (erythromycin or clarithromycin if penicillin allergy) If the area of infection is localised, topical antibiotics should be effective NB: these should not be used for linger than 2 weeks

Answer 568

Explain the symptoms and signs of eczema herpeticum and infected eczema Explain that in many children, eczema improves over time but not all children will grow out of it Children with atopic eczema may develop asthma, hay fever and allergies Advise frequent and liberal use of emollients even when the skin is clear Advise avoidance of triggers (e.g. types of clothes, detergents, soaps, animals) Avoid scratching if possible (keep nails short, use anti-scratch mittens in infants) Information and Support - Itchywheezysneezy.co.uk - excellent website demonstrating how to apply emollients - British Association of Dermatologists (BAD) - has an information leaflet on atopic eczema - National Eczema Society - has fact sheets Dietary elimination: Food allergy should be suspected in young children with moderate/severe, particularly if associated with gut dysmotility (colic, vomiting, altered bowel habit) or faltering growth Eggs and cows' milk are the most common allergens resulting in eczema Allergen specific IgE antibodies in the blood and skin-prick may be helpful Dietary elimination for 4-6 weeks may be necessary to detect a response

Answer 569

Eczema herpeticum is acute and often widespread It is treated with systemic aciclovir

Answer 570

Fingers and soles (verrucae)

Answer 571

Most will disappear spontaneously over a few months | Treatment is only indicated if the lesions are painful or causing cosmetic problems

Answer 572

Daily administration of proprietary salicylic acid or lactic acid paint or glutaraldehyde lotion can be useful Cryotherapy with liquid nitrogen is effective but can be painful (should only be used in older children)

Answer 573

The lesions are small, skin-coloured, pearly papules with central umbilication They may be single but are often multiple

Answer 574

Topical antibiotics may be used to prevent or treat secondary bacterial infection Does NOT require treatment if immunocompetent (it is self-limiting) Spontaneous resolution usually occurs within 18 months Advise against squeezing mollusca to avoid the spread of infectious material and reducing risk of super-infections Avoid sharing towels, clothing and baths with uninfected people If eczema or infection develops arounds the lesions, treat appropriately (e.g. emollients and steroids or antibiotics) Chemical or physical destruction may be done by a specialist

Answer 575

A severely inflamed pustular ringworm patch is called a kerion

Answer 576

From dogs and cats | Causes scaling and patchy alopecia with broken hairs

Answer 577

Rapid diagnosis can be made from microscopic examination of skin scrapings for fungal hyphae Definitive identification of the fungus is by culture

Answer 578

Mild infections are treated with topical antifungals More severe infections will require systemic antifungals Tinea Capitis Systemic antifungal therapy (e.g. griseofulvin or terbinafine) 2nd line: itraconazole or fluconazole Topical antifungal shampoo is recommended in some patients (e.g. selenium sulfide or ketoconazole topical) Tinea Faciale, Tinea Corporis, Tinea Cruris or Tinea Pedis Topical antifungal (e.g. terbinafine, naftifine, butenafine) Topical aluminium acetate (in some) NOTE: any animal source of the infection will also need treatment

Answer 579

Infestation of the mite Sarcoptes scabiei- it burows along the stratum corneum

Answer 580

Severe itching occurs 2-6 weeks after infestations and is worse in warm conditions and at night In older children, burrows, papules and vesicles involve the skin between the fingers and toes, axillae, flexor aspects of the wrists, belt line and around the nipples, penis and buttocks In infants and young children, distributionoften includes the palms, soles and trunk

Answer 581

Clinically Burrows are pathomnemonic of scabies but may be hard to identify because of secondary infection due to scratching The diagnosis can be confirmed by microscopic examination of skin scrapings to identify mite, eggs and mite faeces

Answer 582

Excoriation can lead to secondary eczematous or urticarial reaction which masks the true diagnosis Secondary bacterial infection is common

Answer 583

Prescribe a topical insecticide (permethrin 5% cream) 2nd line: malathion aqueous 0.5% (if permethrin is contraindicated/not tolerated) The product should be applied to the whole body from the chin and ears downwards Particular attention should be paid to areas in between the fingers and under the nails It should be applied to cool, dry skin and allowed to dry before the patient dresses Permethrin should be washed off after 8-12 hours (malathion should be washed off after 24 hours) A second application is required, 1 week after the first application ADVICE Members of the household and other close contacts should be treated The bedding, clothing and towels of the patient (and any potentially infected contacts) should be decontaminated by washing at a high temperature and drying in a hot dryer Patients whose symptoms persist 2-4 weeks after the last treatment application should be advised to retreat Treat post-scabietic itch with crotamiton 10% cream (or topical hydrocortisone) Night-time sedative anti-histamine (e.g. chlorphenamine) may help reduce itching and improve sleep In babies, the face and scalp should be included but avoiding the eyes Alternative: benzyl benzoate emulsion can be applied below the neck and left on for 12 hours but it smells bad and has irritant action Special Cases If crusted scabies, seek specialist advice Seek specialist advice if < 2 months old

Answer 584

Itching of the scalp and nape of the neck Live lice may be idenitified on the scalp or nits on hairs (nits are empty egg cases that are seen on hairs) There may be secondary bacterial infection, sometimes leading to misdiagnosis of impetigo Suboccipital lymphadenopathy is common

Answer 585

Dimeticone 4% lotion or aqueous solution 0.5% is rubbed into the hair and scalp and left on overnight and the hair is shampooed the following morning Treatment should be repeated a week later Wet combing with a fine-tooth comb to remove lice every 3-4 days for 2 weeks is useful and safe

Answer 586

Streptococcal or viral sore throat or ear infection

Answer 587

Lesions are small, raindrop-like, round or oval erythematous scaly patches on the trunk and upper limbs An attack usually resolves over 3-4 months Most will get a recurrence in the next 3-5 years Chronic psoriasis with plaques or annular lesions is less common in children

Answer 588

Bland ointments - NB: coal tar preparations are useful for plaque psoriasis in > 6 years - Dithranol preparations are very effective in resistant plaque psoriasis - Calcipotriol, a vitamin D analogue, is ueful for plaque psoriasis in > 6 years

Answer 589

Occasionally, children with chronic psoriasis will develop psoriatic arthritis

Answer 590

An acute, benign, self-limiting condition Usually begins with a single round or oval scaly macule, called a herald patch (usually 2-5 cm in diameter and seen on the trunk, upper arm, neck or thigh. After a few days, several small dull pink macules develop on the trunk, upper arms and thighs). The rash tends to follow the line of the ribs posteriorly, described as a fir tree pattern The lesions can be itchy

Answer 591

No treatment is required and the rash resolves within 4-6 weeks

Answer 592

Hailress, single or multiple, non-inflamed smooth areas of skin, usually over the scalp Remnants of broken off hairs may be visible as exclamation mark hairs (these can be seen at the edge or active patches of hair fall) Regrowth often occurs after 6-12 months

Answer 593

Lesions are typically ringed (annular) with a raised fledh-soloured non-scaling edge (unlike ringworm which is scaling) Usually found over the bony prominences, especially on the hands and feet Lesions may be single or multiple and tend to be 1-3cm in diameter They disappear spontaneously but may take years to do so

Answer 594

May begin 1-2 years before the onset of puberty following androgenic stimulation of the sebaceous glands and an increased sebum excretion rate Obstruction to the flow of sebum in the sebaceous follicles initiates the process of acne

Answer 595

Initially, there will be open comedones (blakheads) or closed comedones (white heads), which can progress to papules, pustules, nodules and cysts More severe cystic and nodular lesions can cause scarring

Answer 596

Menstruation and emotional stress can cause exacerbations

Answer 597

Avoid over-cleaning the skin (may cause dryness and irritation - twice daily washing with gentle soap is adequate) If make-up, emollients and cleansers are used, non-comedogenic preparations are recommended with a pH close to the skin Avoid picking and squeezing scars to due to risk of scarring Treatments are effective but may take a while to work (up to 8 weeks) and may initially irritate the skin Maintain a healthy diet Support and information: NHS Choices leaflet British Association of Dermatologists

Answer 598

``` Consider a single topical treatment: Benzoyl peroxide Benzoyl peroxide + clindamycin (Duac) Adapalene (topicla retinoid) - Contraindicated in breastfeeding and pregnancy Azelaic acid ``` Creams and lotions are preferable in patients with dry skin

Answer 599

Consider oral antibiotics: (for a maximum of 3 months) Lymecycline Doxycyline NB: a topical retinoid or benzoyl peroxide should always be co-prescribed with oral antibiotics to reduce the risk of antibiotic resistance developing Change to an alternative antibiotic if no improvement after 3 months If not responding after 2 courses of antibiotics or if they are scarring, refer to dermatology for consideration of treatment with isotretinoin (Roaccutane) COCP in combination with topical agents can be used as an alternative to systemic antibiotics in girls (NB: progesterone only contraceptives or rogestin implants with androgenic activity may worsen acne)

Answer 600

Severe variant (e.g. acne conglobata or acne fulminans) Severe acne with scarring or risk of scarring Multiple treatments have failed Significant psychological distress Diagnostic uncertainty

Answer 601

Review each treatment step at 8-12 weeks - If there is adequate response, continue treatment for at least 12 weeks - If acne has cleared or almost cleared - consider maintenance therapy with topical retinoids or azelaic acid - If there if no response, consider adherence to treatment, adverse effects, progression to more severe acne and discuss the next steps in management

Answer 602

Facial rash in SLE or dermatomyositis Purpura over the buttocks, lower limbs and elbows in HSP Erythema nodosum and erythema multforme are both associated with systemic disorders Stevens-Johnson syndrome is a severe bullous form of erythema multiforme involving the mucous membranes - Often starts with URTI - Eye involvment may include conjunctivitis, corneal ulceration and uveitis - It can be caused by drug sensitivity (particularly anti-epileptics)

Answer 603

Flesh-coloured wheals

Answer 604

A delayed hypersensitivity reaction most commonly seen on the legs, following a bite from a flea, bedbug, animal or bird mite

Answer 605

Rare autosomal disorder caused by deficiency or dysfunction of C1-esterase inhibitor There is no urticaria, but subcutaneous swellings occur, often accompanied by abdominal pain Usually triggered by physical trauma or psychological stress Episodes develop over a few hours and subside over a few days Angioedema may cause respiratory obstruction Treated with a purified preparation of the inhibitor

Answer 606

Left-to-right shunts (breathless): Ventricular septal defect Persistent arterial duct Atrial septal defect Right-to-left shunts (blue): Tetralogy of Fallot Transposition of the great arteries ``` Common mixing (breathless and blue): Atrioventricular septal defect (complete) ``` Outflow obstruction in a well child (asymptomatic with a murmur): Pulmonary stenosis Aortic stenosis Outflow obstruction in a sick neonate (collapsed with shock): Coarctation of the aorta

Answer 607

In the foetus, the left atrial pressure is LOW because relatively little blood returns from the lungs The pressure in the right atrium is higher than the left, as it receives all the systemic venous return including blood from the placenta The flap valve of the foramen ovale is held open, so blood can flow across the atrial septum into the left atrium and then the left ventricle, before being pumped to the rest of the body With the first breaths, resistance to pulmonary blood flow falls and the volume of blood flowing through the lungs increases six-fold This leads to a rise in left atrial pressure At the same time, the volume of blood returning to the right atrium will fall as the placenta is excluded from the circulation The change in the pressure differences causes the flap valve of the foramen ovale to close The ductus arteriosus, which connects the pulmonary artery to the aorta in foetal life, will close within the first few hours or days Some babies with congenital heart lesions rely on the blood flow through the duct (duct-dependent circulation) - So, their clinical condition will deteriorate dramatically when the duct closes (usually around 1-2 days)

Answer 608

``` Antenatal cardiac ultrasound diagnosis Detection of a heart murmur Heart failure Shock Cyanosis ```

Answer 609

Checking the foetal heart is a routine part of the foetal anomaly scan - Performed between 18-20 weeks' gestation - Leads to diagnosis of 70% of infants requiring surgery in the first 6 months If an abnormality is detected, a paediatric cardiologist will perform detailed foetal echocardiography Children at increased risk (e.g. Down's syndrome, previous congenital heart conditions in the family) are also checked Depending on the diagnosis, some choose termination

Answer 610

Vast majority of children with murmurs have a normal heart | This is called an innocent murmur which can be heard at some point inn almost 30% of children

Answer 611

ASymptomatic Soft blowing murmur Systolic murmur only, not diastolic Let Sternal edge Normal heart sounds with no added sounds No parasternal thrill No radiation NB: innocent of flow murmurs are often heard best during a febrile illness or anaemia, due to increased cardiac output, so it is important to examine a child with a murmur when these other illnesses have been corrected

Answer 612

Pulmonary vascular resistance is still high Conditions like VSD and ductus arteriosus may only become apparent at several weeks of age, when the pulmonary resistance falls

Answer 613

Breathlessness (particularly on feeding or exertion) Sweating Poor feeding Recurrent chest infections ``` Poor weight gain or faltering growth Tachypnoea Tachycardia Heart murmur, gallop rhythm Hepatomegaly Cool peripheries ```

Answer 614

Neonates- obstructed (duct-dependent) systemic circulation: - Hypoplastic left heart syndrome - Critical aortic valve stenosis - Severe coarctation of the aorta - Interruption of the aortic arch Infants- high pulmonary blood flow - VSD - Atrioventricular septal defect - Large persistent ductus arteriosus Older children and adolescents - right or left heart failure - Eisenmenger syndrome (right heart failure only) - Rheumatic heart disease - Cardiomyopathy IN the first week of life, heart failure tends to result from left heart obstruction (e.g. coarctation of the aorta) If the obstruction is very severe, arterial perfusion may be mainly RIGHT-to-LEFT flow of blood via the arterial duct (duct-dependent systemic circulation) Closure of the duct rapidly leads to severe acidosis, collapse and DEATH (unless duct patency is restored) AFTER the first week of life, progressive heart failure is most likely due to left-to-right shunt Over the subsequent weeks, as pulmonary vascular resistance falls, there is a progressive increase in LEFT-to-RIGHT shunt and increasing pulmonary flow The symptoms of heart failure will typically increase up to the age of 3 months, but then improve as the pulmonary vascular resistance rises in response to the LEFT-to-RIGHT shunt If this is left untreated, children will develop Eisenmenger syndrome

Answer 615

A process in which long-standing Left-to-right shunt (caused by congenital heart defect) causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic Right-to-left shunt This causes cyanosis If Eisenmenger syndrome develops, the only option is heart-lung transplant

Answer 616

``` Aims: Reduce preload - Using diuretics (e.g. furosemide) or, more rarely, venous dilators (e.g. nitroglycerin) Enhance cardiac contractility - Using IV agents (e.g. dopamine) - Other options: digoxin, dobutamine, adrenaline, milrinone Reduce afterload - Oral ACE inhibitors - IV agents (e.g. hydralazine, nitroprusside, alprostadil) Improving oxygen delivery - Beta-blockers (e.g. carvedilol) Enhancing nutrition ``` If heart failure is thought to be due to a cardiac malformation: If cyanotic --> Prostaglandin infusion - This maintains a PDA in duct-dependent cyanotic heart disease and buys time before surgical correction can be performed Echocardiography to identify the underlying structural defect is necessaryt

Answer 617

Structural heart disease