Paediatrics - QuesMed

Question 1

How does GORD present in an infant?

Answer 1

Gastro-oesophageal reflux affects up to 40% of children during the first 6 months of life. It is common as the gastro-oesophageal sphincter is still developing.

As acid refluxes back up the oesophagus, babies commonly present with:

Milky vomits after feeds.
Crying/irritability
Arching of the back
Drawing up of the knees into the chest

In some cases, these arching movements can be so strong that they can be mistaken for seizures.

Question 2

What is the management of GORD?

Answer 2

Management of GORD in infants can be:

• Conservative (advice on keeping baby upright and burping after feeds, put their cot on a slight incline for sleeping)
• Medical (infant gaviscon, omeprazole)
• mSurgical in severe cases (fundoplication)

Simple reflux does not warrant further investigations if the child is still feeding and gaining weight.

Question 3

What are the first signs of puberty in boys and girls?

Answer 3

Enlargement of testes >4ml in boys (usually at 9 years) and enlargement of breasts in girls (usually at 8.5 years).

Question 4

What is precocious puberty?

Answer 4

Precocious puberty should be considered whenever secondary sexual characteristic appear before 8 years of age in girls and before 9 years of age in boys. Left untreated it can lead to accelerated skeletal development and bone plate fusion resulting in a reduced final height. The early onset of the physical changes experienced can also have a significant impact on the affected child’s psychological wellbeing.

Question 5

What are some causes of Gonadotrophin-dependent precocious puberty (GDPP)?

Answer 5

• Idiopathic (>90%)
• Brain neoplasms
• Cranial radiotherapy
• Cranial disability conditions such as hydrocephalus, cerebral palsy or post-infection (meningitis)
• Traumatic head injury
• Associated with child adoption and sexual abuse

Question 6

What are some causes of Gonadotrophin independent precocious puberty (GIPP)?

Answer 6

• Ovarian causes: follicular cysts of the ovary, granulosa cell tumours, Leydig’s cell tumours, and gonadoblastoma.
• Testicular causes: Leydig’s cell tumours and a defect of luteinising hormone (LH) receptor function (testotoxicosis or familial GIPP).
• Adrenal causes: 21-hydroxylase congenital adrenal hyperplasia (CAH) in males results in GIPP. CAH in females presents with signs of virilisation (e.g., pubic and axillary hair and clitoromegaly) but no breast development.
• McCune-Albright syndrome (MAS)

Question 7

Summarise paediatric observations

Answer 7

Age	           HR (bpm)    RR (per/min) SBP (mmHg)
Birth	        110-170	25-60	60-100
12 months	105-165	25-55	65-115
1-5 years 	85-150	20-40	70-120
6-11 years	70-135	16-35	80-130
12-18 years	60-120	14-26	95-140

Question 8

What is tuberous sclerosis complex?

Answer 8

Tuberous sclerosis complex (TSC) is an autosomal-dominant, neurocutaneous, multi-system disorder characterised by cellular hyperplasia, tissue dysplasia, and multiple organ hamartomas (benign tumours).

The disease has 2 genetic loci: TSC1, found on chromosome 9q34; and TSC2, found on chromosome 16p13.The clinical phenotype can result from a mutation in either of these genes.

TSC can be identified in all ethnic groups and is equally identified in both sexes. Population studies have estimated a prevalence of 1 in 6000 to 9000 people.

Question 9

What may a child with tuberous sclerosis complex present with?

Answer 9

• Giant cell astrocytoma
• Sub-ependymal nodes
• Learning disabilities and autism spectrum -disorder
• Infantile spasms with hypsarrhythmia seen on electroencephalogram
• Epilepsy
• Ash leaf spots
• Angiomyolipomas (found in the kidneys)
• Papilloedema
• Cardiac rhabdomyomas
• Cystic replacement of lung parenchyma
• Facial angiofibromas

Question 10

Describe the presentation of parvovirus B19 infection

Answer 10

Parvovirus B19 presents in children with a prodrome of fever, coryza and diarrhoea. Following this, a diffuse ‘lace-like’ rash develops across the body with a characteristic bright red rash on the cheeks - hence the name, ‘slapped cheek syndrome’ (or ‘erythema infectiosum’, ‘fifth disease’).

In adults, Parvovirus B19 presents with arthralgias.

Question 11

What are the complications of parvovirus B19 infection?

Answer 11

Parvovirus infection also reduces erythropoiesis. This is not significant for most patients; however, in vulnerable groups like those with conditions like sickle cell anaemia and hereditary spherocytosis that rely on erythropoiesis, infection can precipitate a severe anaemia and an aplastic crisis

Infection in the first half of pregnancy can also cause severe foetal anaemia that can precipitate hydrops foetalis and subsequent miscarriage.

Question 12

What is meconium aspiration?

Answer 12

Meconium aspiration is when a newborn breathes in a mixture of meconium and amniotic fluid. Meconium is the baby’s first stool.

It most commonly occurs in term/post-term babies, or babies with foetal distress.Meconium aspiration can present with respiratory distress and staining of the newborn skin.

Question 13

What are the complications of meconium aspiration?

Answer 13

Meconium aspiration syndrome is associated with high rates of air leak, pneumothorax and pneumomediastinum. The sudden development of profound respiratory distress suggests pneumothorax.

Other:

• Airway obstruction/collapse
• Surfactant dysfunction
• Pulmonary vasoconstriction
• Chemical pneumonitis
• Aspiration pneumonia

Question 14

What is the management of meconium aspiration?

Answer 14

Surfactant replacement
Ventilation
Antibiotics

Question 15

What are some concerning symptoms and signs pointing towards severe illness/sepsis?

Answer 15

• If a child is not rousable, or does not stay awake when roused
• Bradycardia or tachycardia for age
• Bradypnoea or tachypnoea for age
• Mottled skin
• Peripheral or central cyanosis
• Non-blanching rash

Question 16

What is aortic coarctation?

Answer 16

A narrowing of the aorta just before the ductus arteriosus. Coarctation of the aorta will divert blood into the brachiocephalic artery supplying the right arm, and constrict flow to the more distal branches of the aorta that supply the right leg. Coarctation is monitored with echocardiography and may require management with a stent insertion or surgical repair.

Question 17

How does aortic coarctation present?

Answer 17

There is a difference in the saturations of the limbs. Radio-femoral delay is caused by blood flowing through collateral arterial circulation in the chest walls, which delays flow to the lower extremities.

It may present with an ejection systolic murmur heard loudest posteriorly, between the shoulders, although heart sounds may be completely normal.

Coarctation of the aorta may be completely asymptomatic, particularly in younger patients. However, it may progress to cause left ventricular hypertrophy in teenagers and adults.

In teenagers or adults with coarctation, collateral circulation will develop with large intercostal arteries. These may be visible as ‘rib notching’ on a chest x-ray.

5% of babies with Turner syndrome are born with coarctation of the aorta. There will be clinical findings of a webbed neck, widely spaced nipples and coarctation of the aorta are most consistent with a diagnosis of Turner syndrome. Turner’s syndrome is the result of an XO karyotype. All of these babies are girls.

Question 18

What is an important complication of aortic coarctation?

Answer 18

Most commonly -> cerebral aneurysms.
This is a narrowing of the aorta, most often after the left subclavian artery’s branches off the aorta.

Reduced perfusion pressures distal to stenosis trigger the renin-angiotensin-aldosterone system with limited effect as this won’t overcome the stenosis. It however will increase perfusion pressures before the stenosis, including the carotids, resulting in higher cerebral perfusion pressures which can cause aneurysms to form.

Question 19

What are the doses of adrenaline IM needed in anaphylaxis for the different age ranges?

Answer 19

The dose of adrenaline varies according to age:
150 micrograms of 1:1000 is for children under 6.
300 micrograms is for children 6-12.
500 micrograms is for children over 12 and adults

Question 20

What are the BTS asthma guidelines?

Answer 20

Step 1: Inhaled SABA PRN
Step 2: Add inhaled corticosteroid 200-400micrograms/day
Step 3: Add LABA or LRTA
If good response: continue
If benefit but still inadequate: continue LABA and increase steroid to 400
No response to LABA: Stop LABA and increased corticosteroid to 400micrograms/day.
Step 4: Increased inhaled corticosteroid to 800 micrograms/day
Step 5: Use daily steroid tablet + refer to respiratory physician

Question 21

When are newborns assessed after birth?

Answer 21

As part of newborn screening, a newborn and infant physical examination is performed within the first 72 hours of birth, and again at 6-8 weeks.

This involves a thorough examination of the baby’s systems and anatomical features, in order to assess for any congenital abnormality or pathology.

Question 22

Explain how to calculate paediatric fluid maintenance?

Answer 22

1st 10kg of bodyweight at 100ml/kg/day
2nd 10kg of bodyweight at 50ml/kg/day
Remaining bodyweight at 20ml/kg/day
The fluid type routinely used is 0.9% NaCl + 5% dextrose with 10mmol KCl (all in the same bag)

Paediatric electrolyte replacement requirements: Sodium: 2-4mmol/kg/day
Potassium: 1-2mmol/kg/day

Question 23

How would you calculate fluid deficit?

Answer 23

Fluids deficit replacement in children:

Firstly you should calculate the percentage dehydration by taking away the child’s dehydrated weight from their ideal weight then dividing by the child’s ideal weight and multiplying by 100%.

You can then work out the fluid deficit in ml by multiplying the % dehydration by their weight (kg) then multiplying that by 10.

Deficit replacement fluids should then be given spread out over a 24-48 hour period in children in addition to normal maintenance fluids.

Question 24

What is cerebral palsy?

Answer 24

Cerebral palsy (CP) is a group of permanent movement disorders that appear in early childhood. Signs and symptoms vary among people and over time. Often, symptoms include poor coordination, stiff muscles, weak muscles, and tremors. About two to three children out of every 1,000 have Cerebral palsy. It is estimated that 1 in 400 babies born in the UK have a type of cerebral palsy.

Periventricular damage (due to a hypoxic ischaemic event during a prolonged delivery due to the baby getting stuck) is the aetiology behind spastic diplegia. The gait is classically termed as scissor walking.

Question 25

What are the different types of cerebral palsy?

Answer 25

Spastic (90%): _ damage to pyramidal pathways (pyramidal weakness) _ General features: increased tone and reflexes, clasp knife, flexed hip and elbow, ankle plantar flexion. _ Spastic hemiplegia: Arm>leg, early hand preference _ Spastic quadriplegia: Most severe, Associated with seizures, leg>arm _ Spastic monoplegia: paralysis of 1 limb, usually an arm _ Spastic diplegia: Associated with periventricular damage Scissor walking Dyskinetic/ athetoid: Choreiform movements, Intellectually relatively unimpaired, Basal ganglia damage from kernicterus. Ataxic: Uncommon, Genetically determined, Uncoordinated movements, intention tremor

Question 26

What does the management of cerebral palsy involve?

Answer 26

Management requires specialist input and MDT management, often including physiotherapy, occupational therapy and speech and language therapy.

Question 27

What is Ewing's sarcoma and how does it present?

Answer 27

Ewing's sarcoma makes up 14% of all bone sarcoma diagnoses making it the second most common bone cancer in children and adolescents after osteosarcoma. It most commonly affects teenagers and young adults with the pelvis, thigh bone and shin bone being the most commonly affected areas. Bone pain particularly occurring at night A mass or swelling Restricted movement in a joint Ewing's sarcoma typically presented with the lamellated (onion skinning) periosteal reaction which is visible on x-ray. MRI typically shows a large mass with evidence of necrosis and on histology (after biopsy) small blue round cells are visible with clear cytoplasms on haematoxylin and eosin staining.

Question 28

What is whooping cough and how does it present?

Answer 28

Pertussis (whooping cough) is a severe URTI characterised by severe bouts of spasmodic coughing, which may lead to apnoea in infants, followed by characteristic gasping for breath. It is caused by bordetella pertussis and there has been a recent resurgence even in vaccinated countries due to lower vaccination uptake. ``` Symptoms seen in pertussis infection: Cough Inspiratory whooping Rhinorrhoea Post-tussive vomiting Decreased food intake ```

Question 29

What is the management of pertussis?

Answer 29

1st line treatment in children over 1 month of age is with azithromycin. 2nd line treatment in children over 1 month of age is with trimethoprim/sulfamethoxazole

Question 30

What are the complications of pertussis?

Answer 30

- Apnoea is a rare but life-threatening acute complication of pertussis - Pneumonia either due to bordetella pertusssis or secondary to another organism - Seizure triggered by cerebral hypoxia which can develop during severe cough paroxysms - Otitis media is the most common complication in pertussis and is often seen in the following few weeks

Question 31

What is hypoxic ischaemic encephalopathy and how does it present?

Answer 31

Hypoxic ischaemic encephalopathy (HIE) is the term for brain damage resulting from ante- or perinatal hypoxia. The lack of oxygen in the foetal circulation results in poor supply of oxygen to the brain. This ischaemia results in irreversible brain damage, both from primary neuronal death (immediate) and secondary reperfusion injury (delayed). Any event causing poor oxygenation can cause HIE, for example during labour (placental abruption), during delivery (cord compression), or after delivery (failure to breathe). The presentation of hypoxic ischaemic encephalopathy depends on the degree of neurological damage, ranging from mild (irritability) to severe (hypotonia, poor responses, prolonged seizures). The prognosis of HIE depends on the degree of neurological damage. Severe cases are strongly associated with chronic neurological disability (especially cerebral palsy) and HIE is fatal in approximately 1/3 of severe cases. The degree of damage can be assessed with MRI.

Question 32

How is HIE investigated and managed?

Answer 32

Investigation of HIE is with EEG monitoring. Management of HIE depends on presentation, and includes respiratory support, anticonvulsant therapy, and careful fluid balance, electrolyte monitoring and inotropes. Cooling the baby to induce mild hypothermia can also prevent further damage by secondary reperfusion injury

Question 33

What are the risk factors for sepsis?

Answer 33

Risk factors for sepsis include: - Age less than 1 year - Impaired immune function (e.g. diabetes, splenectomy, immunosuppressant medication, cancer treatment) - Recent surgery in the last six weeks - Breach of skin integrity (burns/cuts/skin infections) - Presence of an indwelling catheter or line

Question 34

What are the investigations for sepsis?

Answer 34

First line investigations should include: ``` Bloods for FBC, CRP, U&E, Procalcitonin (inflammatory marker which is more specific for bacterial infection) Blood culture Creatinine Clotting screen A blood gas to measure lactate. ``` Physical examination and further investigations should be aimed at finding the source of infection: Urinalysis Chest x-ray Imaging of the abdomen and pelvis Lumbar puncture (if there are no contra-indications such as suspicion of raised intracranial pressure, focal neurological signs or fluctuating consciousness)

Question 35

What is the management for suspected sepsis?

Answer 35

If any high risk criteria are identified, and the source of sepsis is unconfirmed, a broad-spectrum antibiotic should be given within one hour of identification of sepsis. Other management will include intravenous fluids, if appropriate, and a discussion with critical/intensive care doctors may need to be held if the child is in a high risk group, has highly abnormal physical observations or a high lactate result, for consideration of central venous access, inotropes and vasopressors.

Question 36

How does sepsis present in a child?

Answer 36

Sepsis in children can present in a number of ways, many of which are non-specific, as a result of systemic inflammatory response to suspected or proven infection. Symptoms in children with sepsis may include: ``` Generally feeling unwell Lethargy Poor social interaction Altered conscious level Continuous crying in babies Pale or mottled skin Rash Decreased urine output Poor feeding or oral intake Cool peripheries ``` Signs may include: Increased respiratory rate Increased respiratory effort Bradycardia or tachycardia Fever or signs of dehydration (e.g. dry mucous membranes, decreased skin turgor, delayed capillary refill time). I It is important to note that fever is not necessarily always present.

Question 37

How does measles present and how is it treated?

Answer 37

Measles presents with a characteristic erythematous, blanching maculopapular rash all over the body, preceded by a fever, cough, runny nose or conjunctivitis and grey spots (Koplik's spots) inside the cheeks. The illness is self-limiting, but because of the possible complications, routine immunisation is encouraged. It is caused by the measles virus. Children are infectious from 4 days before the rash to around 4 days after.

Question 38

Describe the newborn resuscitation protocol

Answer 38

The newborn neonatal resuscitation pathway is as follows: Birth Dry baby and perform initial check- assess tone, breathing and heart rate If gasping or not breathing- open airway, give five inflation breaths and consider oxygen and ECG monitoring Reassess- look for improvement and response in heart rate and chest movement during inflation If chest not moving- optimise airway control, repeat inflation breaths, give oxygen (if not given already), consider ECG monitoring (if not already doing so) and look for a response If no increase in heart rate at this point, re-assess for chest movement and repeat above steps if necessary When chest is moving- if heart rate <60 bpm, ventilate for 30 seconds At this point, reassess heart rate- if heart rate still <60 bpm, commence chest compressions at a rate of 3:1 Continue this process, re-assessing every 30 seconds- if heart rate remains <60 bpm, consider venous or osseous access and administering drugs Throughout process: Keep the parents updated, and keep a record of time and timings of interventions

Question 39

What is acute otitis media?

Answer 39

Acute otitis media is middle ear inflammation. Acute otitis mediia often follows an URTI with an acute onset of pain (hence, this is why the child is irritable and tugging on his ear). The fluid behind the eardrum will build up and may burst the ear drum causing a discharge and a sudden relief of pain. The build-up of fluid behind the ear drum results in a conductive hearing loss (Rinne's test).

Question 40

What is the management of acute otitis media?

Answer 40

Management is analgesia as first line. Indications for antibiotics in children are: - Perforated eardrum - <2 years old and bilateral - Present for ≥4 days - <3 months old If there are recurrent episodes, refer for insertion of grommets (thin tubes sitting in the ear drum that allow fluid to pass from behind the ear drum to be discharged). Grommets usually fall out on their own. Insertion of grommets twice is an indication for tonsillo-adenelectomy. Glue ear (recurrent otitis media) is a common presentation of conductive hearing loss in children. The major concern is whether it affects the child's speech and language development, and whether there are any problems in school.

Question 41

What is an important complication of otitis media?

Answer 41

The major complication is mastoiditis which requires imaging with CT. This presents with: - Pain on palpation of the mastoid process. - Laterally and inferiorly displaced pinna - Thick purulent discharge In an adult – diabetes mellitus is a risk factor for mastoiditis

Question 42

What is the other name for chicken pox?

Answer 42

Human herpes virus 3 (HHV3)

Question 43

What is the management for a choking child?

Answer 43

Management of choking in children depends on whether the child is conscious and whether they can cough. If they can cough, encouraging them to cough may help dislodge the obstruction. If they can't cough effectively but are conscious, then you should alternate between giving 5 back blows and 5 chest (infant)/abdominal (child) thrusts. If the choking child becomes unconscious, then 5 rescue breaths should be given before immediately starting CPR.

Question 44

What monitoring is required after giving phototherapy to a jaundiced child?

Answer 44

After commencing phototherapy, bilirubin levels should be monitored regularly (4-6 hours after first starting and then 6-12 hours when levels are stable or falling). Once bilirubin levels have fallen over 50 micromoles/L below treatment threshold, phototherapy can be stopped. Bilirubin should then be rechecked in 12- 18 hours to assess for rebound hyperbilirubinaemia. If at this time levels remain >50 micromoles/L, no further monitoring is needed. However, if bilirubin has risen to within 50 micromoles/L below treatment, levels should be rechecked again in 12 hours (and consider restarting phototherapy).

Question 45

What is jaundice?

Answer 45

Jaundice is a condition where the eyes and skin turn yellow due to build up of bilirubin. Newborns can become jaundiced if bilirubin is more than 80 umol/L.

Question 46

What are the causes of jaundice?

Answer 46

Causes <24 hours: -Haemolytic disorders (Rh incompatibility, ABO incompatibility, G6PD, spherocytosis), -Infection (TORCH Screen is indicated) Causes 24 hours- 14 days (Physiological jaundice): - Breast milk jaundice - Dehydration, infection - Haemolysis - Bruising - Polycythaemia - Crigler-Najjar Syndrome Causes >14 days (>21 if preterm): - Physiological jaundice - Breast milk jaundice - Infection (esp UTI) - Hypothyroidism - G6PD - Pyloric stenosis - Bile obstruction (biliary atresia) - Neonatal hepatitis

Question 47

What causes physiological jaundice?

Answer 47

Marked physiological release of Hb from breakdown of red cells due to high Hb at birth Red cell life span of newborns is shorter than adults Hepatic bilirubin metabolism less efficient in first few days of life

Question 48

What are the complications of jaundice?

Answer 48

Kernicterus is a rare but serious complication of untreated jaundice in babies. It's caused by excess bilirubin damaging the brain or central nervous system. Treatment for kernicterus involves using an exchange transfusion.

Question 49

What is status epilepticus and what is the management?

Answer 49

Status epilepticus (SE) is a life-threatening neurological condition defined as 5 or more minutes of either continuous seizure activity or repetitive seizures without regaining consciousness. - Supportive care + oxygen + check glucose - Buccal midazolam or IV lorazepam - IV lorazepam - IV Phenytoin and/or IV phenobarbitone - Rapid sequence induction with sodium thiopentone in coordination with anaesthetics or intensive care

Question 50

What are the complications of status epilepticus?

Answer 50

Diagnosis of non-convulsive SE can be difficult, but recognition is crucial because rapid termination helps to prevent serious brain injury, especially in patients with impaired consciousness.In most cases, treatment must precede any thorough investigation and must be initiated as soon as SE is suspected. Complications of status epilepticus: - Focal neurological deficit (normally temporary) - Memory loss - Behavioural problems - Hypoxic brain injury - Epilepsy - Encephalopathy

Question 51

What are some causes of right iliac fossa pain?

Answer 51

Gastro-intestinal causes: acute appendicitis, mesenteric adenitis, inflamed Meckel's diverticulum, diverticulitis (although this is more common on the left), and inflammatory bowel disease (particularly Crohn's disease). Gynaecological causes: ectopic pregnancy, ovarian torsion of cyst, and pelvic inflammatory disease. Male genital causes: testicular torsion. Urological causes: pyelonephritis, ureteric colic.

Question 52

What is the best way to confirm a Meckel's diverticulum?

Answer 52

A technetium scan, using radioactive metastable technetium-99, will highlight ectopic gastric mucosa in a symptomatic Meckels' diverticulum. This is the commonest cause of per rectal bleeding in a child.

Question 53

What are the levels of tanner staging for boys and girls?

Answer 53

Tanner staging for females Females breast changes on a scale of B1-B5: ``` B1 Prepubertal B2 Breast bud B3 Juvenile with smooth contour B4 Areola and papilla project above breast B5 Adult Tanner staging for males ``` Male genital changes on a scale of G1-G5: G1 Prepubertal, testicular volume <1.5ml G2 Penis grows in length only, testicular volume 1.5-6ml G3 Penis grows further in length and circumference, testicular volume 6-12ml G4 Development of glans penis, darkening of scrotal skin, testicular volume 12-20ml G5 Adult genitalia, testicular volume >20ml Pubic hair changes on a scale of PH1-PH5: PH1 Pre-adolescent no sexual hair PH2 Sparse, pigmented, long, straight, mainly along labia or at base of penis PH3 Dark, coarser, curlier PH4 Filling out towards adult distribution PH5 Adult in quantity and type with spread to medial thighs in males

Question 54

Describe some paediatric syndromes with cardiac involvement

Answer 54

Trisomy 21: Septal defects are most common with atrioventricular septal defects (AVSD) being the most common - The phenotype described is that of trisomy 21. The most common cardiac defect associated with people with trisomy 21 is an atrio-ventricular septal defect (AVSD), which will have a pan-systolic murmur due to the ventricular septal defect (VSD). ``` Foetal alcohol syndrome: Ventricular septal defect DiGeorge syndrome (22q11.2 deletion): Aortic arch defects - e.g. interrupted aortic arch Turner syndrome (45XO): Bicuspid aortic valves and coarctation of the aorta Noonan syndrome: Pulmonary stenosis Edward's syndrome (Trisomy 18): Septal defects Patau syndrome (Trisomy 13): Dextrocardia ```

Question 55

What are the 3 shunts present in the foetal circulation?

Answer 55

There are 3 shunts present in the foetal circulation: Ductus venosus, connecting the umbilical vein to the inferior vena cava Foramen ovale, connecting the right atrium to the left atrium Ductus arteriosus, connecting the pulmonary artery to the aorta In the foetal circulation, the pulmonary circulation has a very high resistance as the lungs are not yet inflated. This high resistance diverts blood away from the lungs and into the aorta via the ductus arteriosus (right to left shunt). After birth, as the lungs expand the resistance in the neonate's lung capillary bed rapidly decreases. Blood then flows through the lung circulation. As oxygenated blood goes through the ductus arteriosus, it is triggered to close. However, if the ductus arteriosus does not close, there will be a left-to-right shunt due to the now higher pressures in the left atrium.

Question 56

What is the presentation and management of a PDA?

Answer 56

A persistent ductus arteriosus may be asymptomatic or present with signs of heart failure. There may also be a collapsing pulse from the increased pressure gradients and a continuous murmur from turbulent flow across the ductus arteriosus. Treatment of a persistent ductus arteriosus is only required if the baby is symptomatic. About 1/3 of patients with a patent ductus arteriosus require medical treatment with indomethacin. Indomethacin is an NSAID that inhibits prostaglandin synthesis. Inhibition of prostaglandin causes vasoconstriction which closes the ductus arteriosus. Around 8% of patients will require surgical ligation to close the ductus arteriosus. NSAIDs can be used to ensure duct latency in conditions e.g. transposition of great arteries

Question 57

What is HSP?

Answer 57

Henoch-Schonlein Purpura (HSP) is the most common small vessel vasculitis in children. It most commonly affects children aged 3-5. HSP is commonly preceded by a viral upper respiratory tract infection. HSP presents with purpura or petechiae on the lower limbs and abdominal pain, arthralgia, and nephritis (haematuria +/- proteinuria), due to immunoglobulin deposition in the gut, joints and kidneys respectively.

Question 58

What is the management and prognosis of HSP?

Answer 58

Children with HSP should be admitted for supportive management if their symptoms are severe or they are unable to tolerate sufficient oral fluids. NSAIDs are used for pain relief. Steroids are not used routinely, but can be used if pain does not respond to NSAIDs. The prognosis of HSP is generally good; the majority of cases of HSP recover completely. 1/3 of patients have a second episode of HSP. Long-term renal impairment occurs in about 1/5 patients with significant proteinuria. After an episode of HSP, children should have regular urine dips for 12 months to check for renal impairment.

Question 59

How would you estimate doses of medication in children?

Answer 59

Prescribing in paediatrics is challenging given most doses are weight-based Calculating paediatric doses using WETFLAG For emergency situations where a patient's weight is unknown, the WETFLAG mnemonic may help with dosing until an actual weight is obtained ``` Weight: (Age + 4) * 2 Energy: 4J/kg Tube size: (Age/4) + 4 Fluids: 20ml/kg normal saline bolus (10ml/kg if septic, trauma or in heart failure) Lorazepam: 0.1mg/kg Adrenaline: 10 micrograms/kg Glucose: 2ml/kg of 10% dextrose ``` Note 1: local protocols may vary within the UK and abroad but are similar to the above based on APLS guidelines. Note 2: For large children it is important to consider (1) whether an adult dose should be used, and (2) doses prescribed based on weight do not exceed the adult dose.

Question 60

What agents are used as prophylaxis in contacts of a child with meningitis?

Answer 60

This girl is highly likely to have meningococcal infection. All household or close contacts should receive ciprofloxacin as prevention of spread of the disease. A suitable alternative would be rifampicin.

Question 61

Describe the developmental milestones in hearing, communication and language?

Answer 61

What is the difference between speech and language? Speech is how we say and sound words it includes articulation, fluency and voice. Language refers to the specific words we use and how we use them to present our ideas and or get what we want. Normal hearing, communication and language milestones 6 weeks: Startles to noise 6 months: Turns head to loud sounds, understands 'bye bye' 9 months: Responds to own name 12 months: 50% of children will use 3 words by 13m 15 months: point to own body parts, 18m on a doll, understanding basic nouns 18 months: 1-6 different words Any child not able to say 1-3 words and unable to point to body parts at 18m would be a cause for concern with regards to developmental delay. 2 years: understanding verbs, can join 2 words together 2.5 years: understanding prepositions in/on, 3-4 words joined together 3 years: understands negatives, understand adjectives 3.5 years: understands comparatives, able to follow a simple instruction e.g. 'can you draw me a big circle please?' 4 years: understands complex instructions e.g. 'before you put the book down can you give the pen to mummy'

Question 62

What is vitamin K deficiency?

Answer 62

It is common for newborn babies to have Vitamin K deficiency. The features of Vitamin K deficiency are bruising more easily and if severe enough, there can be bleeding internally. Subcutaneous Vitamin K injections are routinely offered at birth to prevent haemorrhagic disease of the newborn. However, mothers are able to decline this injection. Haemorrhagic disease of the newborn is potentially life-threatening as newborn babies progress rapidly into haemorrhagic shock. Vitamin K does not pass well through the placenta and babies are normally born with a Vitamin K deficiency. Vitamin K is necessary in the clotting cascade (factors 10, 9, 7 and 2 – remember the date 1972). This is why IM Vitamin K is offered routinely to all newborn babies. Increased bleeding caused by deficiency can lead to jaundice through increased breakdown of these blood products. This is particularly true in this case because of the cephalohaematoma.

Question 63

Describe bronchiolitis, how it presents and its management

Answer 63

Cause: Respiratory syncytial virus (RSV) is responsible for 80% of cases. ``` Common symptoms of bronchiolitis: Cough Laboured breathing Wheeze Tachypnoea Intercostal recession, grunting, and nasal flaring Management ``` Prophylaxis: Palivizumab prophylaxis in high risk patients Supportive care Oxygen therapy which may escalate to mechanical ventilation

Question 64

Describe the different foetal presentations

Answer 64

Fetal presentation Presentation of the fetus refers to which part of the fetus is presenting first at the pelvic inlet. There are three main categories of fetal presentation: cephalic, breech and shoulder. Cephalic presentations include: Vertex (most common presentation) Brow Face Chin Breech presentations include: Frank – the legs are extended up to head, the buttocks are the presenting part Complete – the hips and knees are flexed, buttocks are the presenting part Incomplete – one or both hips are extended, knee or foot is the presenting part Footling (single/double) – one or both legs are fully extended, foot/feet is the presenting part Shoulder presentations include: Arm is leading part Shoulder is leading part Trunk is leading part

Question 65

Describe the causes of tonsillitis and the management of bacterial tonsillitis

Answer 65

Tonsillitis is a form of pharyngitis where there is intense acute inflammation of the tonsils, often with a purulent exudate in bacterial tonsillitis. Cause: Strep. pneumoniae is the most common causative organism especially in recurrent tonsillitis in children. EBV is another common cause. Differentiating between bacterial and viral tonsillitis. Bacterial tonsillitis is more associated with cervical lymphadenopathy. Viral tonsillitis is associated with headache, apathy and abdominal pain. CENTOR criteria give an indication of the likelihood of a sore throat being due to bacterial infection. They are as follows: ``` Tonsillar exudate Tender anterior cervical lymphadenopathy Fever over 38 Absence of cough Each of the Centor criteria score 1 point (maximum score of 4). ``` A score of 0, 1 or 2 is thought to be associated with a 3 to 17% likelihood of isolating streptococcus. A score of 3 or 4 is thought to be associated with a 32 to 56% likelihood of isolating streptococcus. Management of bacterial tonsillitis A Centor criteria score of 3/4 would warrant prescribing antibiotics or evidence of systemic upset/immunosuppression would warrent a course of antibiotics: 1st line: Penicillin V 500mg PO QDS for 5-10 days Alternative in pen allergy: Clarithromycin/Erythromycin 250-500mg PO BD for 5 days

Question 66

What are the different causes of proteinuria in children?

Answer 66

Orthostatic proteinuria features: This is the commonest cause of proteinuria in children. It is generally benign and requires no active management. It typically presents in adolescence. Transient proteinuria features Children will often be found to have proteinuria incidentally when being investigated. Commonest causes are concurrent infections or a recent seizure. This is benign and usually self-limiting, with the proteinuria receding as the precipitant is treated. Nephrotic syndrome: When children have 3+ proteinuria on dipstick it is worth consider whether the patient has nephrotic syndrome. The commonest cause in children is idiopathic nephrotic syndrome and they should be started empirically on steroids. Type 1 diabetes mellitus: Just like in adults, poorly controlled diabetes can result in diabetic glomerulopathy. Good control of diabetes can limit its progression. Management For any child with proteinuria, at least two separate urine samples should be sent for protein:creatinine ratios with one sample being an early morning sample. All patients should have their blood pressure measured at rest to ensure they aren't hypertensive

Question 67

Describe the different types of enteroviruses

Answer 67

Enteroviruses includes a broad range of pathogens that are all transmitted via the faeco-oral route. They include coxsackie A, coxsackie B, polio and echoviruses: Coxsackie A causes hand-foot-mouth disease (vesicles on the hands, feet and buccal mucosa, and fever). Coxsackie B causes myocarditis. Polio is now eradicated in the western world. In unvaccinated individuals, polio most commonly is asymptomatic. About a quarter of people develop relatively mild symptoms of an upper respiratory tract infection, diarrhoea and vomiting. In 1% of infections, the virus affects the CNS which leads to paralysis. If severe and involving the diaphragm, children require ventilatory support (you may have seen pictures of 'iron lungs' used for respiratory support in the 1950s). Paralysis may persist if anterior horn cells are completely destroyed, leading to muscle wasting. Echovirus causes aseptic meningitis and a febrile illness in children.

Question 68

Describe the APGAR scoring system and its use

Answer 68

The APGAR score uses 5 components to quickly assess the state of a newborn baby. The APGAR score is a method of assessing the state of a neonate quickly after birth. There are 5 components to the APGAR score: Appearance, Pulse, Grimace, Activity, Respiration. Appearance relates to the colour of the child. A score of 2 is for a pink baby, 1 if the baby is blue peripherally but pink centrally, and 0 if the baby is blue all over. Pulse: a heart rate of >100 beats per minute scores 2, <100 scores 1, and a non-detectable heart rate scores 0. Grimace relates to the response to stimulation: crying on stimulation scores 2, a grimace scores 1, and no response scores 0. Activity: Flexed limbs that resists extension score 2, some flexion scores 1, and a floppy baby scores 0. Respiration: a strong cry scores 2, a weak cry scores 1, and no respiratory effort scores 0. Interpreting the APGAR score Scores for each component of the APGAR are added up to an overall score out of 10. A score of >6 is reassuring A score of 4-6 requires stimulation A score of <4 requires resuscitation The score is intended as a quick assessment of current status, and is not a long-term prognostic indicator, although long periods of low APGAR scores are correlated with reduced neurological function. Newborns are assessed at 1, 5 and 10 minutes.

Question 69

What is malrotation and what is the management?

Answer 69

Malrotation is a rare but extremely important diagnosis to make. In malrotation, early in development the midgut rotates and fixates in an abnormal position. This abnormal position makes the bowel more prone to volvulus (twisting around the mesentery) and compression of the duodenum by peritoneal bands (called Ladd bands). The resulting bowel obstruction in malrotation causes a buildup of gut contents, which results in profuse vomiting as nothing can get past the obstruction. As the obstruction is past the Sphincter of Oddi, the bowel contents are full of green bile and so the vomit is dark green. The diagnosis of malrotation is confirmed with an upper gastrointestinal contrast study, which will identify the point of the obstruction (as no contrast can pass distally from this location). The proximal bowel may be shaped like a corkscrew. Malrotation must be managed with urgent surgery to relieve the obstruction e.g. Ladd's - This is performed, either open or laparoscopically, by untwisting the midgut volvulus, fixing the malrotated bowel in the correct location, removing Ladd's bands (congenital adhesions) and performing an appendicectomy.

Question 70

What is an atypical UTI?

Answer 70

A UTI is classed as atypical if any of the following are present: ``` Poor urine flow Abdominal or bladder mass Raised creatinine Septicaemia Failure to respond to treatment with suitable antibiotics within 48 hours Infection with non-E. coli organisms ```