paeds Flashcards

Question

Juvenile idiopathic arthritis: pauciarticular - features?

Answer 1

Describes arthritis occurring in someone who is less than 16 years old that lasts for more than 6 weeks. Pauciarticular JIA refers to cases where 4 OR LESS JOINTS ARE AFFECTED. It accounts for around 60% of cases of JIA Features of pauciarticular JIA (most common type of JIA) - joint PAIN, stiffness and swelling: usually medium sized joints (larger joints) e.g. knees, ankles, elbows - limp - fatigue - associated symptoms may include rash, fever, dry or gritty eyes - Family history of autoimmune disease (e.g.SLE) is a risk factor. - ANA may be positive in JIA - associated with anterior uveitis - have normal blood results

Answer 2

- Often anaemia, thrombocytosis, and leucocytosis - High spiking fevers-there must be 1 or 2 spikes of fever a day occurring daily for at least 2 weeks - Non-pruritic salmon coloured erythematous rashes are common also, usually on the trunk and proximal extremities -The rash often co-occurs with the fever.

Answer 3

Slipped capital femoral epiphysis is rare HIP condition seen in children, classically seen in OBESE BOYS. It is also is known as slipped upper femoral epiphysis. Basics - typically age group is 10-15 years - More common in obese children and boys - Displacement of the femoral head epiphysis postero-inferiorly - May present acutely following trauma or more commonly with chronic, persistent symptoms Features - hip, groin, medial thigh or knee pain - loss of INTERNAL ROTATION of the leg in flexion - bilateral slip in 20% of cases Investigation - AP and lateral (typically frog-leg) views are diagnostic Management - internal fixation: typically a single cannulated screw placed in the center of the epiphysis

Answer 4

- Short stature - Learning difficulties - characteristic like affect -Friendly, extrovert personality - Transient neonatal hypercalcaemia - Supravalvular aortic stenosis Associated with elfin facies

Answer 5

Micrognathia ( lower jaw is undersized) Low-set ears Rocker bottom feet Overlapping of fingers

Answer 6

- An overgrowth of tissue which occurs during the healing process of the umbilicus. - It is most common in the first few weeks of life. - On examination, a small, red growth of tissue is seen in the centre of the umbilicus. - It is usually wet and leaks small amounts of clear or yellow fluid. - It is treated by regular application of salt to the wound, if this does not help then the granuloma can be cauterised with silver nitrate.

Answer 7

- This condition consists of an infection of the umbilicus. - Infection with Staphylococcus aureus is the commonest cause. - The condition is potentially serious as infection may spread rapidly through the umbilical vessels in neonates with a risk of portal pyaemia (collection of pus in the portal venous system due to inflammation), and portal vein thrombosis. - Treatment is usually with a combination of topical and systemic antibiotics.

Answer 8

- This will typically present as an umbilical discharge that discharges SMALL BOWEL CONTENT. - Complete persistence of the duct is a rare condition. - Much more common is the persistence of part of the duct (Meckel's diverticulum). - Persistent vitello-intestinal ducts are best imaged using a contrast study to delineate the anatomy and are managed by laparotomy and surgical closure.

Answer 9

- Little or no head lag on being pulled to sit - Lying on abdomen, good head control - Held sitting, lumbar curve - Reaches for object - Holds rattle briefly if given to hand - Visually alert, particularly human faces - Fixes and follows to 180 degrees

Answer 10

- Lying on abdomen, arms extended - Lying on back, lifts and grasps feet - Pulls self to sitting - Held sitting, back straight - Rolls front to back - Holds in palmar grasp - Pass objects from one hand to another - Visually insatiable, looking around in every direction

Answer 11

Sits without support

Answer 12

- Pulls to standing - Crawls - Points with finger - Early pincer

Answer 13

Cruises Walks with one hand held Good pincer grip Bangs toys together

Answer 14

Walks unsupported (15 months) - makes a tower of 2 (15 months)- looks at book, pats page

Answer 15

Squats to pick up toy Drawing - circular scribble Book - turns pages, several at time

Answer 16

``` Runs Walks upstairs and downstairs holding on to rail Makes a tower of 6 Drawing - copies vertical line Book - turns pages, one at a time ```

Answer 17

Rides a tricycle using pedals Walks up stairs without holding on to rail Makes a tower of 9 Drawing - copies circle

Answer 18

Hops on one leg | Drawing - copies cross

Answer 19

Developmental dysplasia of the hip The Department of Health advises that all babies that were breech at any point from 36 weeks (even if not breech by time of delivery), babies born before 36 weeks who had breech presentation, and all babies with a first degree relative with a hip problem in early life, should be referred for ultrasound of the hips.

Answer 20

- female sex: 6 times greater risk - breech presentation - positive family history - firstborn children - oligohydramnios - birth weight > 5 kg - congenital calcaneovalgus foot deformity

Answer 21

(usually detected in neonates) The following infants require a routine ultrasound examination - first-degree family history of hip problems in early life - breech presentation at or after 36 weeks gestation, irrespective of presentation at birth or mode of delivery - multiple pregnancy All infants are screened at both the newborn check and also the six-week baby check using the Barlow and Ortolani tests

Answer 22

Clinical examination - Barlow test: attempts to dislocate an articulated femoral head - Ortolani test: attempts to relocate a dislocated femoral head Other important factors include: - symmetry of leg length - level of knees when hips and knees are bilaterally flexed - restricted abduction of the hip in flexion Ultrasound is used to confirm the diagnosis if clinically suspected Management - most unstable hips will spontaneously stabilise by 3-6 weeks of age - Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months - older children may require surgery

Answer 23

Turner's syndrome is a chromosomal disorder affecting around 1 in 2,500 FEMALES. It is caused by either the presence of only one sex chromosome (X) or a deletion of the short arm of one of the X chromosomes. Turner's syndrome is denoted as 45,XO or 45,X. Features - short stature - shield chest, widely spaced nipples - WEBBED NECK - bicuspid aortic valve (15%), coarctation of the aorta (5-10%) - primary amenorrhoea - cystic hygroma (often diagnosed prenatally) - high-arched palate - short fourth metacarpal - multiple pigmented naevi - lymphoedema in neonates (especially feet) - gonadotrophin levels will be elevated - hypothyroidism is much more common in Turner's - horseshoe kidney: the most common renal abnormality in Turner's syndrome

Answer 24

- Bicuspid aortic valve and aortic coarctation | - There is also an increased incidence of autoimmune disease (especially autoimmune thyroiditis) and Crohn's disease

Answer 25

- idiopathic - dehydration - low-fibre diet - medications: e.g. Opiates - anal fissure - over-enthusiastic potty training - hypothyroidism - Hirschsprung's disease - hypercalcaemia - learning disabilities

Answer 26

Advice on diet/fluid intake | MOVICOL paediatric plain

Answer 27

- Reported from birth or first few weeks of life - Passage of meconium >48 hours - 'ribbon' stools - Previously unknown or undiagnosed weakness in legs, locomotor delay - distended abdomen AMBER signs: - Faltering growth - Disclosure or evidence that raises concerns over possibility of child maltreatment

Answer 28

- movicol paediatric plain (1st line) - Add Stimulant laxative if disimpaction not occurred in 2 weeks - substitute a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric Plain is not tolerated - inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain

Answer 29

very similar to the faecal impaction regime, with obvious adjustments to the starting dose, i.e. - first-line: Movicol Paediatric Plain - add a stimulant laxative if no response - substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if stools are hard - continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce dose gradually

Answer 30

Leukotriene receptor antagonist

Answer 31

1. SABA 2. SABA + low dose inhaled corticosteroid (ICS) 3. SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA) 4. SABA + paediatric low-dose ICS + long-acting beta agonist (LABA) 5. SABA + switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a paediatric low-dose ICS 6. SABA + paediatric moderate-dose ICS MART OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA 7. SABA + one of the following options: increase ICS to paediatric high-dose, either as part of a fixed-dose regime or as a MART (maintenance and reliever therapy) a trial of an additional drug (for example theophylline) seeking advice from a healthcare professional with expertise in asthma

Answer 32

1. SABA 2. SABA + an 8-week trial of paediatric MODERATE-dose inhaled corticosteroid (ICS) After 8-weeks stop the ICS and monitor the child's symptoms: if symptoms did not resolve during the trial period, review whether an alternative diagnosis is likely if symptoms resolved then reoccurred within 4 weeks of stopping ICS treatment, restart the ICS at a paediatric low dose as first-line maintenance therapy if symptoms resolved but reoccurred beyond 4 weeks after stopping ICS treatment, repeat the 8‑week trial of a paediatric moderate dose of ICS 3. SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA) 4. Stop the LTRA and refer to an paediatric asthma specialist

Answer 33

Maintenance and reliever therapy (MART) - a form of combined ICS and LABA treatment in which a single inhaler, containing both ICS and a fast-acting LABA, is used for both daily maintenance therapy and the relief of symptoms as required - MART is only available for ICS and LABA combinations in which the LABA has a fast-acting component (for example, formoterol) It should be noted that NICE does not advocate changing treatment in patients who have well-controlled asthma simply to adhere to the latest guidance.

Answer 34

Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases. Epidemiology: - peak incidence at 6 months - 3 years - more common in autumn Features: - stridor - barking cough (worse at night) - fever - coryzal symptoms - Mild:Occasional barking cough No audible stridor at rest No or mild suprasternal and/or intercostal recession The child is happy and is prepared to eat, drink, and play - Moderate: Frequent barking cough Easily audible stridor at rest Suprasternal and sternal wall retraction at rest No or little distress or agitation The child can be placated and is interested in its surroundings - Severe: Frequent barking cough Prominent inspiratory (and occasionally, expiratory) stridor at rest Marked sternal wall retractions Significant distress and agitation, or lethargy or restlessness (a sign of hypoxaemia) Tachycardia occurs with more severe obstructive symptoms and hypoxaemia CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include: - < 6 months of age - known upper airway abnormalities (e.g. Laryngomalacia, Down's syndrome) - uncertainty about diagnosis (important differentials include acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation) Management - CKS recommend giving a single dose of oral DEXAMETASONE (0.15mg/kg) to all children regardless of severity - prednisolone is an alternative if dexamethasone is not available Emergency treatment - high-flow oxygen - nebulised adrenaline

Answer 35

- chronic knee problem - Pain after exercise - Intermittent swelling and locking

Answer 36

Haemophilus influenzae type B

Answer 37

Streptococcus pyogenes and Streptococcus pneumoniae.

Answer 38

rapid onset high temperature, generally unwell stridor drooling of saliva

Answer 39

Parainfluenza

Answer 40

16 months - 2 years

Answer 41

7-8 months

Answer 42

Intussusception describes the invagination of one portion of bowel into the lumen of the adjacent bowel (telescoping bowel), most commonly proximal or at the level of the ileo-caecal region. Intussusception usually affects infants between 6-18 months old. Boys are affected twice as often as girls Features: - paroxysmal abdominal colic pain (colicky pain) - during paroxysm the infant will characteristically draw their knees up and turn pale - vomiting - bloodstained stool - 'red-currant jelly' - is a late sign - sausage-shaped mass in the right upper quadrant Investigations: ultrasound is now the investigation of choice and may show a target-like mass Management: - the majority of children can be treated with reduction by AIR INSUFFLATION under radiological control, which is now widely used first-line compared to the traditional barium enema - if this fails, or the child has signs of peritonitis, surgery is performed

Answer 43

Is caused by absence of ganglion cells (aganglionic segment of bowel) from myenteric and submucosal plexuses. Occurs in 1/5000 births (rare). It is an important DD in childhood CONSTIPATION. Possible presentations: Hirschsprungs may present either with features of bowel obstruction in: 1. the neonatal period e.g. failure or delay to pass meconium 2. More insidiously during childhood. - constipation, abdominal distention - After the PR there may be an improvement in symptoms. - Full-thickness rectal biopsy for diagnosis - Treatment is with rectal washouts initially - After that an anorectal pull through procedure

Answer 44

Exomphalos | Diaphragmatic herniae

Answer 45

- High caecum at the midline - Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia - May be complicated by the development of volvulus, an infant with volvulus may have bile stained vomiting - Diagnosis is made by upper GI contrast study and USS - Treatment is by laparotomy, if volvulus is present (or at high risk of occurring then a Ladd's procedure is performed

Answer 46

- Usually delayed passage of meconium and abdominal distension - The majority have cystic fibrosis - X-Rays will not show a fluid level as the meconium is viscid, PR contrast studies may dislodge meconium plugs and be therapeutic - Infants who do not respond to PR contrast and NG N-acetyl cysteine will require surgery to remove the plugs

Answer 47

- Prematurity is the main risk factor - Early features include abdominal distension and passage of bloody stools - X-Rays may show pneumatosis intestinalis and evidence of free air - Increased risk when empirical antibiotics are given to infants beyond 5 days - Treatment is with total gut rest and TPN, babies with perforations will require laparotomy

Answer 48

- Associated with tracheo-oesophageal fistula and polyhydramnios - May present with choking and cyanotic spells following aspiration - VACTERL associations (VACTERL association is a disorder that affects many body systems. VACTERL stands for vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities.)

Answer 49

Rectal washouts/bowel irrigation (to allow the baby to pass meconium")

Answer 50

Males | Down's syndrome

Answer 51

Pathophysiology: - normally, in the foetus, there is an attachment between the vitellointestinal duct and the yolk sac. This disappears at 6 weeks gestation - the tip is free in the majority of cases - associated with enterocystomas, umbilical sinuses, and omphaloileal fistulas. - arterial supply: omphalomesenteric artery. - typically lined by ileal mucosa but ectopic gastric mucosa can occur, with the risk of peptic ulceration. Pancreatic and jejunal mucosa can also occur. Meckel's diverticulum is a congenital diverticulum of the small intestine. It is a remnant of the omphalomesenteric duct (also called the vitellointestinal duct) and contains ectopic ileal, gastric or pancreatic mucosa Rule of 2s occurs in 2% of the population is 2 feet from the ileocaecal valve is 2 inches long Presentation (usually asymptomatic) - abdominal pain mimicking appendicitis - rectal bleeding: Meckel's diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years - intestinal obstruction secondary to an omphalomesenteric band (most commonly), volvulus and intussusception Management: - removal if narrow neck or symptomatic. Options are between wedge excision or formal small bowel resection and anastomosis.

Answer 52

Urinary tract infections (UTI) are more common in boys until 3 months of age (due to more congenital abnormalities) after which the incidence is substantially higher in girls. At least 8% of girls and 2% of boys will have a UTI in childhood Presentation in childhood depends on age: - infants: poor feeding, vomiting, irritability - younger children: abdominal pain, fever, dysuria - older children: dysuria, frequency, haematuria - features which may suggest an upper UTI include: temperature > 38ºC, loin pain/tenderness NICE guidelines for checking urine sample in a child - if there are any symptoms or signs suggestive or a UTI - with unexplained fever of 38°C or higher (test urine after 24 hours at the latest) - with an alternative site of infection but who remain unwell (consider urine test after 24 hours at the latest) Urine collection method: - clean catch is preferable - if not possible then urine collection pads should be used - cotton wool balls, gauze and sanitary towels are not suitable - invasive methods such as suprapubic aspiration should only be used if non-invasive methods are not possible Management - infants LESS THAN 3 MONTHS OLD SHOULD BE REFERRED IMMEDIATELY TO A PAEDIATRICIAN - children aged more than 3 months old with an upper UTI should be considered for admission to hospital. If not admitted oral antibiotics such as cephalosporin or co-amoxiclav should be given for 7-10 days - children aged more than 3 months old with a lower UTI should be treated with oral antibiotics for 3 days according to local guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin. Parents should be asked to bring the children back if they remain unwell after 24-48 hours - antibiotic prophylaxis is not given after the first UTI but should be considered with recurrent UTIs

Answer 53

The majority of children achieve day and night time continence by 3 or 4 years of age. Enuresis may be defined as the 'involuntary discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous system or urinary tract' Nocturnal enuresis can be defined as either primary (the child has never achieved continence) or secondary (the child has been dry for at least 6 months before) Management: - look for possible underlying causes/triggers (e.g. Constipation, diabetes mellitus, UTI if recent onset) - advise on fluid intake, diet and toileting behaviour reward systems (e.g. Star charts). NICE recommend these 'should be given for agreed behaviour rather than dry nights' e.g. Using the toilet to pass urine before sleep - NICE advises: 'Consider whether an alarm or drug treatment is appropriate, depending on the age, maturity and abilities of the child or young person, the frequency of bedwetting and the motivation and needs of the family'. Generally: - an enuresis alarm is first-line for children under the age of 7 years - desmopressin may be used first-line for children over the age 7 years, particularly if short-term control is needed or an enuresis alarm has been ineffective/is not acceptable to the family

Answer 54

'those over 16 can consent to treatment, but cannot refuse treatment under 18 unless there is one consenting parent, even if the other disagrees'.

Answer 55

Ejection systolic murmur - due to bicuspid aortic valve, aortic valve stenosis and/or aortic coarctation - those with Turner's are prone to have these (Loudest over the aortic valve)

Answer 56

Automated otoacoustic emission test/ evoked otoacoustic emissions test - Newborn Hearing Screening Programme. A computer generated click is played through a small earpiece. The presence of a soft echo indicates a healthy cochlea

Answer 57

Croup | Systemically well- therefore rules out epiglottis

Answer 58

Necrotising enterocolitis is one of the leading causes of death among premature infants. Initial symptoms can include FEEDING INTOLERANCE, abdominal DISTENSION and BLOODY STOOLS, which can quickly progress to abdominal discolouration, perforation and peritonitis.

Answer 59

Abdominal x-rays are useful when diagnosing necrotising enterocolitis, as they can show: - dilated bowel loops (often asymmetrical in distribution) - bowel wall oedema - pneumatosis intestinalis (intramural gas- gas in the gut wall) - portal venous gas - pneumoperitoneum resulting from perforation - air both inside and outside of the bowel wall (Rigler sign) - air outlining the falciform ligament (football sign)

Answer 60

5 months - 12 months

Answer 61

Ultrasound scan | Shows a target sign

Answer 62

Alphas -1 antitrypsin deficiency | CF

Answer 63

Early surgical intervention (a Kasai procedure- hepatoportoenterostomy)- attempts to restore bile flow from the the liver to the proximal small bowel. It decreases hepatic damage, and avoids or delays the need for subsequent liver transplantation.

Answer 64

The cause of prolonged jaundice in this infant is biliary atresia, a condition involving either obliteration or discontinuity within the extrahepatic biliary system. Biliary atresia results in an obstruction in the flow of bile and the presentation of a cholestatic picture, including pale stools and dark urine.

Answer 65

``` pregnancy sexually transmitted infections, recurrent UTIs sexually precocious behaviour anal fissure, bruising reflex anal dilatation enuresis and encopresis behavioural problems, self-harm recurrent symptoms e.g. headaches, abdominal pain ```

Answer 66

Before 8 years in females and 9 years in males

Answer 67

Acephalohaematoma may occur after a spontaneous vaginal delivery or following a trauma from the obstetric forceps or the ventouse. A haemorrhage results after the presidium is sheared from the parietal bone. - A swelling on the newborns head. It typically develops SEVERAL HOURS AFTER DELIVERY and is due to bleeding between the periosteum and skull. - The most common site affected is the parietal region. - Doesn't cross suture lines - the tense swelling is limited to the outline of the bone. Jaundice may develop as a complication. Takes up to 3 months to resolve.

Answer 68

- Swelling on the head of a newborn. - More common following prolonged, difficult deliveries - Managed conservatively

Answer 69

Caput succedaneum is a subcutaneous, extraperiosteal, collection of fluid caused by pressure on the fatal scalp during the birthing process. - results in a large oedematous swelling and bruising over the scalp. - Present at birth - typically forms over the vertex and crosses suture lines - Resolves within days (therefore treatment not required)

Answer 70

Intraventricular haemorrhage is a haemorrhage that occurs into the ventricular system of the brain. It is relatively rare in adult surgical practice and when it does occur, it is typically associated with severe head injuries. In PREMATURE neonates it may occur spontaneously. The blood may clot and occlude CSF flow, hydrocephalus may result. In neonatal practice the vast majority of IVH occur in the first 72 hours after birth, the aetiology is not well understood and it is suggested to occur as a result of birth trauma combined with cellular hypoxia, together the with the delicate neonatal CNS. Treatment - Is largely supportive - Hydrocephalus and rising ICP is an indication for shunting.

Answer 71

aka subgaleal haemorrhage | - rare and is due to a traumatic birth. It may result in the infant losing large amounts of blood

Answer 72

Kawasaki disease is a type of VASCULITIS which is predominately seen in children. Whilst Kawasaki disease is uncommon it is important to recognise as it may cause potentially serious complications, including coronary artery aneurysms. Features - high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics - conjunctival injection - bright red, cracked lips - strawberry tongue - cervical lymphadenopathy - red palms of the hands and the soles of the feet which later peel Kawasaki disease is a clinical diagnosis as there is no specific diagnostic test. Management - high-dose aspirin= Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye's syndrome aspirin is normally contraindicated in children - intravenous immunoglobulin - echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms Complications: coronary artery aneurysm

Answer 73

- maternal diabetes mellitus - prematurity - IUGR - hypothermia - neonatal sepsis - inborn errors of metabolism - nesidioblastosis - Beckwith-Wiedemann syndrome

Answer 74

transient hypoglycaemia in the first few hours to days after birth is very common. This is usually monitored and does not need any intervention or escalation. Mothers should be encouraged to feed their child early and at regular intervals.

Answer 75

Refer for same-day paediatric assessment (admit to hospital) A child aged < 3 months with a fever > 38ºC should be assessed as high risk of serious illness. (3 months old with a temperature > 38ºC is regarded as a 'red' feature in the new NICE guidelines)

Answer 76

Slipped capital femoral epiphysis is rare hip condition seen in children, classically seen in obese boys. It is also is known as slipped upper femoral epiphysis. Basics - typically age group is 10-15 years - More common in obese children and boys - Displacement of the femoral head epiphysis postero-inferiorly - May present acutely following trauma or more commonly with chronic, persistent symptoms Features hip, groin, medial thigh or knee pain loss of internal rotation of the leg in flexion bilateral slip in 20% of cases Investigation AP and lateral (typically frog-leg) views are diagnostic Management internal fixation: typically a single cannulated screw placed in the center of the epiphysis

Answer 77

The Apgar score is used to assess the health of a newborn baby.

Answer 78

``` Pulse RR Colour Muscle tone Reflex irritability ```

Answer 79

Palivizumab is a monoclonal antibody which is used to prevent respiratory syncytial virus (RSV) in children who are at increased risk of severe disease.

Answer 80

Those at risk of developing RSV include Premature infants Infants with lung or heart abnormalities Immunocompromised infants

Answer 81

Less than 24 hours old

Answer 82

Jaundice in the first 24 hrs is always pathological

Answer 83

Measure bilirubin within 2 hours

Answer 84

Pathological jaundice is due to physiological problems in the red blood cells or a cross-reaction with the maternal blood - rhesus haemolytic disease - ABO haemolytic disease - hereditary spherocytosis - glucose-6-phosphodehydrogenase

Answer 85

Jaundice in the first 24 hrs is always pathological. Jaundice in the neonate from the c. 2-14 days is common (up to 40%) and usually physiological. It is more commonly seen in breast fed babies

Answer 86

Jaundice screen is performed including: - conjugated and unconjugated bilirubin: the most important test as a RAISED CONJUGATED BILIRUBIN could indicate BILIARY ATRESIA which requires urgent surgical intervention - direct antiglobulin test (Coombs' test) - TFTs - FBC and blood film - urine for MC&S and reducing sugars - U&Es and LFTs

Answer 87

- biliary atresia - hypothyroidism - galactosaemia - urinary tract infection - breast milk jaundice - congenital infections e.g. CMV, toxoplasmosis

Answer 88

Hand, foot and mouth disease is a self-limiting condition affecting children. It is caused by the intestinal viruses of the Picornaviridae family (most commonly coxsackie A16 and enterovirus 71). It is very contagious and typically occurs in outbreaks at nursery Clinical features: - mild systemic upset: sore throat, FEVER - oral ulcers - followed later by VESICLES on the PALMS and SOLES of the feet Management: - symptomatic treatment only: general advice about hydration and analgesia - reassurance no link to disease in cattle - children do not need to be excluded from school - - the HPA recommends that children who are unwell should be kept off school until they feel better - - they also advise that you contact them if you suspect that there may be a large outbreak.

Answer 89

At with during routine baby -checks. detect loss of red reflex

Answer 90

Retinoblastoma is the most common ocular malignancy found in children. The average age of diagnosis is 18 months. Pathophysiology - autosomal dominant - caused by a loss of function of the retinoblastoma tumour suppressor gene on chromosome 13 - around 10% of cases are hereditary Possible features: - absence of red-reflex, replaced by a white pupil (leukocoria) - the most common presenting symptom - strabismus- cross-eyed visual problems Management - enucleation (the removal of the eye that leaves the eye muscles and remaining orbital contents intact) is not the only option - depending on how advanced the tumour is other options include external beam radiation therapy, chemotherapy and photocoagulation Prognosis - excellent, with > 90% surviving into adulthood

Answer 91

leukotriene receptor antagonist

Answer 92

Baby may show symptoms of alcohol withdrawal at birth e.g. irritable, hypotonic, tremors Features: - short palpebral fissure- (small eye opening) - thin vermillion border/hypoplastic upper lip - (thin) - smooth/absent philtrum - learning difficulties - microcephaly- (small head) - growth retardation - epicanthic folds - (An epicanthic fold is a skin fold of the upper eyelid that covers the inner corner (medial canthus) of the eye.) - cardiac malformations

Answer 93

Increased risk of miscarriage, stillbirth, pre-term labour and intrauterine growth retardation

Answer 94

``` Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years. ``` Features - high fever: lasting a few days, followed later by a - maculopapular rash (the high grade fever resolves before the onset of the rash. The rash typically starts abruptly after the temperature subsides, and usually starts on the trunk before spreading to the limbs. The rash is not itchy) - Nagayama spots: papular enanthem on the uvula and soft palate - febrile convulsions occur in around 10-15% - diarrhoea and cough are also commonly seen Other possible consequences of HHV6 infection - aseptic meningitis - hepatitis School exclusion is not needed.

Answer 95

typically starts as an itchy red papular rash which becomes vesicular in nature. This can occur on any part of the body.

Answer 96

Measles rash occurs alongside other systemic symptoms. It typically starts on the face before spreading to other parts of the body. The characteristic 'koplik spots' are classical of this illness.

Answer 97

Otoacoustic emission test

Answer 98

Auditory Brainstem Response test

Answer 99

6-9 months= Distraction test 18 months - 2.5 years = Recognition of familiar objects > 2.5 years =Performance testing > 2.5 years= Speech discrimination tests > 3 years=Pure tone audiometry

Answer 100

the fever is followed later by rash

Answer 101

Late autumn but can occur throughout the year

Answer 102

A enuresis alarm

Answer 103

X-linked recessive condition They are only passed on from mothers (carriers) to sons. A father with haemophilia A can only pass on the gene to his daughters who will become carriers and he cannot pass on the gene to his sons as they inherit the Y-chromosome from him.

Answer 104

1. Ejection murmurs- Due to turbulent blood flow at the outflow tract of the heart 2. Venous hums- Due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just below the clavicles 3. Still's murmur-Low-pitched sound heard at the lower left sternal edge

Answer 105

- soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area - may vary with posture - localised with no radiation - no diastolic component - no thrill - no added sounds (e.g. clicks) - asymptomatic child - no other abnormality

Answer 106

``` Investigations= Contraindication to lumbar puncture (any signs of raised ICP): -focal neurological signs -papilloedema -significant bulging of the fontanelle -disseminated intravascular coagulation -signs of cerebral herniation ``` For patients with meningococcal septicaemia a lumbar puncture is contraindicated - blood cultures and PCR for meningococcus should be obtained. Management= 1. Antibiotics < 3 months: IV amoxicillin + IV cefotaxime > 3 months: IV cefotaxime 2. Steroids - NICE advise against giving corticosteroids in children younger than 3 months - dexamethsone should be considered if the lumbar puncture reveals any of the following: - -frankly purulent CSF - -CSF white blood cell count greater than 1000/microlitre - -raised CSF white blood cell count with protein concentration greater than 1 g/litre - -bacteria on Gram stain 3. Fluids treat any shock, e.g. with colloid 4. Cerebral monitoring - mechanical ventilation if respiratory impairment 5. Public health notification and antibiotic prophylaxis of contacts - ciprofloxacin is now preferred over rifampicin

Answer 107

respiratory distress | this is a red flag

Answer 108

Bronchiolitis is a condition characterised by acute BRONCHIOLAR INFLAMMATION. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases. Epidemiology - most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 3-6 months). Maternal IgG provides protection to newborns against RSV - higher incidence in winter Basics - respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases - other causes: mycoplasma, adenoviruses - may be secondary bacterial infection - more serious if bronchopulmonary dysplasia (e.g. Premature), congenital heart disease or cystic fibrosis Features - coryzal symptoms (including mild fever) precede: - dry cough - increasing breathlessness - wheezing, fine inspiratory crackles (not always present) - feeding difficulties associated with increasing dyspnoea are often the reason for hospital admission NICE recommend immediate referral (usually by 999 ambulance) if they have any of the following: - apnoea (observed or reported) - child looks seriously unwell to a healthcare professional - severe respiratory distress, for example grunting, marked chest recession, or a respiratory rate of over 70 breaths/minute - central cyanosis - persistent oxygen saturation of less than 92% when breathing air. NICE recommend that clinicians 'consider' referring to hospital if any of the following apply: - a respiratory rate of over 60 breaths/minute - difficulty with breastfeeding or inadequate oral fluid intake (50-75% of usual volume 'taking account of risk factors and using clinical judgement') - clinical dehydration. Investigation - immunofluorescence of nasopharyngeal secretions may show RSV Management is largely supportive - humidified oxygen is given via a head box and is typically recommended if the oxygen saturations are persistently < 92% - nasogastric feeding may be needed if children cannot take enough fluid/feed by mouth - suction is sometimes used for excessive upper airway secretions

Answer 109

Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis Features - characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms - this lasts only 1-2 seconds but may be repeated up to 50 times - progressive mental handicap Investigation - the EEG shows hypsarrhythmia in two-thirds of infants - CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis) Management - poor prognosis - vigabatrin is now considered first-line therapy - ACTH is also used

Answer 110

Cleft lip and palate affect around 1 in every 1,000 babies. They are the most common congenital deformity affecting the orofacial structures. Whilst they may be an isolated developmental malformation they are also a recognised component of more than 200 birth defects. Orofacial clefts are a common malformation with many associated risk factors which can be split into those due to events in pregnancy (smoking, benzodiazepine use, anti-epileptic use, rubella infection) and syndromic disorders affecting the baby (trisomies 18, 13 and 15). The commonest variants are: - isolated cleft lip (15%) - isolated cleft palate (40%) - combined cleft lip and palate (45%) Pathophysiology - polygenic inheritance - maternal antiepileptic use increases risk - cleft lip results from failure of the fronto-nasal and maxillary processes to fuse - cleft palate results from failure of the palatine processes and the nasal septum to fuse Problems - feeding: orthodontic devices may be helpful - speech: with speech therapy 75% of children develop normal speech - increased risk of otitis media for cleft palate babies Management - cleft lip is repaired earlier than cleft palate, with practices varying from repair in the first week of life to three months - cleft palates are typically repaired between 6-12 months of age

Answer 111

Intestinal malrotation | An urgent upper GI contrast study and ultrasound is required.

Answer 112

A history of projective vomiting and weight loss is a common story suggestive of pyloric stenosis. The vomit is often not bile stained. Diagnosis is further suggested by hypochloraemic metabolic alkalosis and a palpable tumour on test feeding.

Answer 113

Displaced apex beat and decreased air entry are suggestive of diaphragmatic hernia. The abdomen may well be scaphoid in some cases. The underlying lung may be hypoplastic and this correlates directly with prognosis.

Answer 114

Overview - a form of congenital heart defect - generally classed as 'acyanotic'. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis. - connection between the pulmonary trunk and descending aorta - usually the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance - more common in premature babies, born at high altitude or maternal rubella infection in the first trimester Features - left subclavicular thrill - continuous 'machinery' murmur - large volume, bounding, collapsing pulse - wide pulse pressure - heaving apex beat Management - indomethacin (inhibits prostaglandin synthesis) closes the connection in the majority of cases - if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair

Answer 115

whooping cough

Answer 116

Ebstein's anomaly is a congenital heart defect characterised by low insertion of the tricuspid valve resulting in a large atrium and small ventricle. It is sometimes referred to as 'atrialisation' of the right ventricle. Features -the posterior leaflets of the tricuspid valve are displaced anteriorly towards the apex of the right ventricle. - The creates tricuspid regurgitation (pan-systolic murmur) and tricuspid stenosis (mid-diastolic murmur). There is also enlargement of the right atrium. - present days after birth Associations - tricuspid incompetence (pan-systolic murmur, giant V waves in JVP) - Wolff-Parkinson White syndrome - This may lead to tricuspid regurgitation and in 50% of patients Wolff-Parkinson-White syndrome is seen, which is a pre-excitation syndrome caused by an accessory electrical pathway between the atria and the ventricles which may lead to an irregular heart rate Ebstein's anomaly may be caused by exposure to lithium in-utero - mother taking lithium during the first trimester

Answer 117

pan-systolic but not diastolic murmur

Answer 118

at 1-2 months of age rather than in the days after birth.

Answer 119

Mitral valve prolapse presents with a mid systolic click followed by a late systolic murmur but has no diastolic element.

Answer 120

- ventricular septal defects (VSD) - most common, accounts for 30% - atrial septal defect (ASD) - patent ductus arteriosus (PDA) - coarctation of the aorta - aortic valve stenosis VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents later.

Answer 121

- tetralogy of Fallot - transposition of the great arteries (TGA) - tricuspid atresia Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at around 1-2 months The presence of cyanosis in pulmonary valve stenosis depends very much on the severity and any other coexistent defects.

Answer 122

Mitral valve prolapse, pes planus, autism, memory problems and speech disorders.

Answer 123

``` Flat feet (pes planus) Normal lower limb variants in children. Typical age of presentation - all ages - Typically resolves between the ages of 4-8 years - Orthotics are not recommended - Parental reassurance appropriate ```

Answer 124

Normal lower limb variants in children Typically 1st year of life. Possible causes: - metatarsus adductus: abnormal heel bisector line. 90% of cases resolve spontaneously, severe/persistent cases may require serial casting - internal tibial torsion: difference the thigh and foot ankle: resolves in the vast majority -femoral anteversion: 'W' sign resolves in around 80% by adolescence, surgical intervention in the remaining not usually advised

Answer 125

Can occur at all ages - Common in early infancy and usually resolves by the age of 2 years Usually due to external tibial torsion Intervention may be appropriate if doesn't resolve as increases risk of patellofemoral pain

Answer 126

Typically occurs in the 1st to 2nd year of life. - Normal lower limb variants in children - Typically resolves by the age of 4-5 years

Answer 127

Typically occurs in the 3rd to 4th year of life. - Normal lower limb variants in children - Typically resolves spontaneously

Answer 128

Infestation with threadworms (Enterobius vermicularis, sometimes called pinworms) is extremely common amongst children in the UK. Infestation occurs after swallowing eggs that are present in the environment. Threadworm infestation is asymptomatic in around 90% of cases, possible features include: - perianal itching, particularly worse at night - It is also possible to see threadworms, described as small threads of slowly-moving white cotton either around the anus or in the stools. - girls may have vulval symptoms Diagnosis may be made by the applying Sellotape to the perianal area and sending it to the laboratory for microscopy to see the eggs. However, most patients are treated empirically and this approach is supported in the CKS guidelines. Management - CKS recommend a combination of anthelmintic with hygiene measures for all members of the household - mebendazole is used first-line for children > 6 months old. A single dose is given unless infestation persists. The risk of transmission in families is as high as 75%, and asymptomatic infestation is common. For this reason an anthelmintic drug (mebendazole) should be given as a single dose to all household members.

Answer 129

Hypothyroidism in children (juvenile hypothyroidism) is autoimmune thyroiditis.

Answer 130

- autoimmune thyroiditis - post total-body irradiation (e.g. in a child previous treated for acute lymphoblastic leukaemia) - iodine deficiency (the most common cause in the developing world)

Answer 131

Micrognathia Low-set ears Rocker bottom feet Overlapping of fingers

Answer 132

``` There are several ultrasound markers which are suggestive of Edwards syndrome and should prompt further investigation. These include: - Cardiac malformations - Choroid plexus cysts - Neural tube defects - Abnormal hand and feet position: clenched hands, rocker bottom feet and clubbed feet - Exomphalos - Growth restriction - Single umbilical artery - Polyhydramnios - Small placenta ``` Whilst these are not specific to Edwards syndrome, they are highly suggestive and together increase the likelihood of diagnosis. In fetal medicine these are known as 'soft markers'.

Answer 133

karyotype analysis of placental (chorionic villus sampling) or amniotic fluid (amniocentesis).

Answer 134

Febrile convulsions are seizures provoked by fever in otherwise normal children. They typically occur between the ages of 6 months and 5 years and are seen in 3% of children. Clinical features - usually occur early in a viral infection as the temperature rises rapidly - seizures are usually brief, lasting less than 5 minutes - are most commonly tonic-clonic Types of febrile convulsion: 1. simple = <15 mins, generalised seizure, typically no recurrence within 24 hours, Should be complete recovery within an hour 2. complex =15 - 30 minutes,Focal seizure,May have repeat seizures within 24 hours 3. febrile status epilepticus = >30 mins Management following a seizure - children who have had a first seizure OR any features of a complex seizure should be admitted to paediatrics

Answer 135

- obesity - nasal problems: polyps, deviated septum, hypertrophic nasal turbinates - recurrent tonsillitis - Down's syndrome (This is due to the low muscle tone in the upper airways and large tongue/adenoids. There is also an increased risk of obesity which in people with Down's syndrome which is another predisposing factor to snoring.) - hypothyroidism

Answer 136

Is a cause of delayed puberty secondary to hypogonadotrophic hypogonadism.

Answer 137

Ultrasound

Answer 138

Hypotonia, or floppiness, may be central in origin or related to nerve and muscle problems. - An acutely ill child (e.g. septicaemic) may be hypotonic on examination. Hypotonia associated with encephalopathy in the newborn period is most likely caused by HYPOXIC ISCHAEMIC ENCEPHALOPATHY Central causes: - Down's syndrome - Prader-Willi syndrome - hypothyroidism - cerebral palsy (hypotonia may - precede the development of spasticity) Neurological and muscular problems: - spinal muscular atrophy - spina bifida - Guillain-Barre syndrome - myasthenia gravis - muscular dystrophy - myotonic dystrophy

Answer 139

Cystic fibrosis is NOT a cause of hypotonia in infancy

Answer 140

Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old. Associations - Beckwith-Wiedemann syndrome - as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation - hemihypertrophy - around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11 Features - abdominal mass (most common presenting feature) - palpable non tender mass - distended abdomen - PAINLESS haematuria - FLANK PAIN - other features: anorexia (reduction in appetite), fever - unilateral in 95% of cases - metastases are found in 20% of patients (most commonly lung) - Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell blastomatous tissues resembling the metanephric blastema Referral - children with an unexplained enlarged abdominal mass in children - possible Wilm's tumour - arrange paediatric review with 48 hours Management - nephrectomy - chemotherapy - radiotherapy if advanced disease - prognosis: good, 80% cure rate

Answer 141

Depending on their size often give rise to pain on palpation similar with polycystic kidney disease.

Answer 142

Phototherapy | Exchange transfusion

Answer 143

Laparotomy | is often initially managed medically

Answer 144

A target sign - is the side on view of multiple layers of bowel wall

Answer 145

Pneumatic reduction under fluoroscopic guidance (reduction using fluoroscopy with air)

Answer 146

The majority resolve by the age of 2. 90% of cases resolve by age 1, the rest resolve by age 2.

Answer 147

Pauciarticular juvenile idiopathic arthritis (JIA) - is defined as affecting up to four joints.

Answer 148

Echocardiogram - as coronary artery aneurysms are a complication

Answer 149

Tetralogy of fallot

Answer 150

Laryngomalacia should be suspected in an otherwise well infant with noisy breathing. Congenital abnormality of the larynx. It is caused by a congenital softening of the cartilage of the larynx, causing collapse during inspiration. Infants typical present at 4 weeks of age with: STRIDOR. (is the cause of stridor in approx 50% of neonatal cases) Laryngomalacia can present at birth, and worsens in the first few weeks of life. It usually self-resolves before 2 years of age.

Answer 151

AKA tibial apophysitis It is a traction apophysitis thought to be caused by repeated avulsion of the apophysis into which the patellar tendon is inserted - Seen in sporty teenagers - Pain, tenderness and swelling over the tibial tubercle - Unilateral (but may be bilateral in up to 30% of people). - Gradual in onset and initially mild and intermittent, but may progress to become severe and continuous. - Relieved by rest and made worse by kneeling and activity, such as running or jumping Management is supportive.

Answer 152

Whilst not a classic cause of macrocephaly, children with Fragile X syndrome tend to have a head larger than normal.

Answer 153

Microcephaly may be defined as an occipital-frontal circumference < 2nd centile Causes include - normal variation e.g. small child with small head - familial e.g. parents with small head - congenital infection - perinatal brain injury e.g. hypoxic ischaemic encephalopathy - fetal alcohol syndrome - syndromes: Patau - craniosynostosis

Answer 154

Perthes' disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Impaired blood supply to the femoral head causes bone infarction. Perthes' disease is 5 times more common in boys. Around 10% of cases are bilateral Features hip pain: develops progressively over a few weeks (can last for months) limp stiffness and reduced range of hip movement x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening Diagnosis plain x-ray technetium bone scan or magnetic resonance imaging if normal x-ray and symptoms persist Complications osteoarthritis premature fusion of the growth plates Catterall staging Stage 1-Clinical and histological features only Stage 2- Sclerosis with or without cystic changes and preservation of the articular surface Stage 3- Loss of structural integrity of the femoral head Stage 4-Loss of acetabular integrity Management To keep the femoral head within the acetabulum: cast, braces If less than 6 years: observation Older: surgical management with moderate results Operate on severe deformities Prognosis Most cases will resolve with conservative management. Early diagnosis improves outcomes.

Answer 155

babies dying between 0-28 days of birth

Answer 156

Meconium aspiration syndrome refers to respiratory distress in the newborn as a result of meconium in the trachea. It occurs in the immediate neonatal period. It is more common in post-term deliveries, with rates of up to 44% reported in babies born after 42 weeks. It causes respiratory distress, which can be severe. Higher rates occur where there is a history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse.

Answer 157

Located in the anterior triangle, usually in the midline and below the hyoid (65% cases) Derived from remnants of the thyroglossal duct Thin walled and anechoic on USS (echogenicity suggests infection of cyst)

Answer 158

- Six branchial arches separated by branchial clefts - Incomplete obliteration of the branchial apparatus may result in cysts, sinuses or fistulae - 75% of branchial cysts originate from the second branchial cleft - Usually located anterior to the sternocleidomastoid near the angle of the mandible - Unless infected the fluid of the cyst has a similar consistency to water and is anechoic on USS

Answer 159

large soft tissue opacity - | Sausage -shaped opacity

Answer 160

Target sign

Answer 161

delayed passage - specifically >48 hours (is a red flag associated with Hirschsprung's disease) -Meconium normally passes within the first 24 hours.

Answer 162

Transient tachypnoea of the newborn (TTN) is the commonest cause of respiratory distress in the newborn period. It is caused by delayed resorption of fluid in the lungs It is more common following Caesarean sections, possibly due to the lung fluid not being 'squeezed out' during the passage through the birth canal Chest x-ray may show hyperinflation of the lungs and fluid in the horizontal fissure Supplementary oxygen may be required to maintain oxygen saturations. Transient tachypnoea of the newborn usually settles within 1-2 days

Answer 163

Legally, children under the age of 13 are unable to consent to sex. Therefore - you should disclose that this child has even sexually active to the relevant authority

Answer 164

Causes of a napkin ('nappy') rash include the following: - Irritant dermatitis - Candida dermatitis - Seborrhoeic dermatitis - Psoriasis - Atopic eczema

Answer 165

nappy rash - The most common cause, due to irritant effect of urinary ammonia and faeces - Creases are characteristically spared

Answer 166

Typically an erythematous rash which involve the flexures and has characteristic satellite lesions

Answer 167

Erythematous rash with flakes. May be coexistent scalp rash

Answer 168

A less common cause characterised by an erythematous scaly rash also present elsewhere on the skin

Answer 169

Other areas of the skin will also be affected

Answer 170

- disposable nappies are preferable to towel nappies - expose napkin area to air when possible - apply barrier cream (e.g. Zinc and castor oil) - mild steroid cream (e.g. 1% hydrocortisone) in severe cases - management of suspected candidal nappy rash is with a topical imidazole. Cease the use of a barrier cream until the candida has settled

Answer 171

Acute lymphoblastic leukaemia (ALL)

Answer 172

Acute lymphoblastic leukaemia (ALL) is the most common malignancy affecting children and accounts for 80% of childhood leukaemias. The peak incidence is at around 2-5 years of age and boys are affected slightly more commonly than girls Features may be divided into those predictable by bone marrow failure: anaemia: lethargy and pallor neutropaenia: frequent or severe infections thrombocytopenia: easy bruising, petechiae And other features bone pain (secondary to bone marrow infiltration) splenomegaly hepatomegaly fever is present in up to 50% of new cases (representing infection or constitutional symptom) testicular swelling Types common ALL (75%), CD10 present, pre-B phenotype T-cell ALL (20%) B-cell ALL (5%) ``` Poor prognostic factors age < 2 years or > 10 years WBC > 20 * 109/l at diagnosis T or B cell surface markers non-Caucasian male sex ```

Answer 173

whooping cough

Answer 174

Whooping cough (pertussis) is an infectious disease caused by the Gram-negative bacterium Bordetella pertussis. It typically presents in children. There are around 1,000 cases are reported each year in the UK. Immunisation infants are routinely immunised at 2, 3, 4 months and 3-5 years. Newborn infants are particularly vulnerable, which is why the vaccination campaign for pregnant women was introduced neither infection nor immunisation results in lifelong protection - hence adolescents and adults may develop whooping cough despite having had their routine immunisations Features, 2-3 days of coryza precede onset of: - coughing bouts: usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis - inspiratory whoop: not always present (caused by forced inspiration against a closed glottis) - infants may have spells of apnoea - persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures - symptoms may last 10-14 weeks* and tend to be more severe in infants - marked lymphocytosis Diagnostic criteria Whooping cough should be suspected if a person has an acute cough that has lasted for 14 days or more without another apparent cause, and has one or more of the following features: Paroxysmal cough. Inspiratory whoop. Post-tussive vomiting. Undiagnosed apnoeic attacks in young infants. Diagnosis per nasal swab culture for Bordetella pertussis - may take several days or weeks to come back PCR and serology are now increasingly used as their availability becomes more widespread Management infants under 6 months with suspect pertussis should be admitted in the UK pertussis is a notifiable disease an oral macrolide (e.g. clarithromycin, azithromycin or erythromycin) is indicated if the onset of the cough is within the previous 21 days to eradicate the organism and reduce the spread household contacts should be offered antibiotic prophylaxis antibiotic therapy has not been shown to alter the course of the illness school exclusion: 48 hours after commencing antibiotics (or 21 days from onset of symptoms if no antibiotics ) Complications - subconjunctival haemorrhage - pneumonia - bronchiectasis - seizures

Answer 175

Usually self-resolve, but if large or symptomatic perform elective repair at 2-3 years of age. If small and asymptomatic peform elective repair at 4-5 years of age - due to the risk of incarceration .

Answer 176

Referral points - doesn't smile at 10 weeks - cannot sit unsupported at 12 months - cannot walk at 18 months Fine motor skill problems - hand preference before 12 months is abnormal and may indicate cerebral palsy Gross motor problems - most common causes of problems: variant of normal, cerebral palsy and neuromuscular disorders (e.g. Duchenne muscular dystrophy) Speech and language problems - always check hearing - other causes include environmental deprivation and general development delay

Answer 177

Streptococcus pneumoniae

Answer 178

- Amoxicillin is first-line for all children with pneumonia - Macrolides may be added if there is no response to first line therapy - Macrolides should be used if mycoplasma or chlamydia is suspected - In pneumonia associated with influenza, co-amoxiclav is recommended

Answer 179

Group B streptococcus infection

Answer 180

Neonatal sepsis occurs when a serious bacterial or viral infection in the blood affects babies within the first 28 days of life. Neonatal sepsis is categorised into early onset (within 72 hours of birth) and late onset (between 7-28 days of life) sepsis, with each category tending to have a distinct group of causes and common presentations. Neonatal sepsis account for 10% of all neonatal mortality and therefore must be promptly identified and managed to prevent significant consequences, as untreated, the mortality can be as high as 100%. Epidemiology Male:female incidence 1:1 Incidence of neonatal sepsis: 1-5 per 1000 live births Term neonates: 1-2 per 1000 live births Late pre-term infants: 5 per 1000 live births Birth weight <2.5kg: 0.5 per 1000 live births Black race is an independent risk factor for group B streptococcus-related sepsis Causes - Neonatal infection is present in 8 per 1000 live births (note: this is the incidence of neonatal infection, not yet necessarily progressed to neonatal sepsis), and is the key factor which leads to subsequent life-threatening neonatal sepsis - The overall most common causes of neonatal sepsis are group B streptococcus (GBS) and Escherichia coli, accounting for approximately two thirds of neonatal sepsis cases - Early-onset sepsis in the UK is primarily caused by GBS infection (75%) - -Infective causes in early-onset sepsis are usually due to transmission of pathogens from the mother to the neonate during delivery - Late-onset sepsis usually occurs via the transmission of pathogens from the environment post-delivery, this is normally from contacts such as the parents or healthcare workers - -Infective causes are more commonly coagulase-negative staphylococcal species such as Staphylococcus epidermidis, Gram-negative bacteria such as Pseudomonas aeruginosa, Klebsiella and Enterobacter, and fungal species ``` Other less common causes include: Staphylococcus aureus Enterococcus Listeria monocytogenes Viruses including herpes simplex and enterovirus ``` Risk factors - Mother who has had a previous baby with GBS infection, who has current GBS colonisation from prenatal screening, current bacteruria, intrapartum temperature ≥38ºC, membrane rupture ≥18 hours, or current infection throughout pregnancy - Premature (<37 weeks): approximately 85% of neonatal sepsis cases are in premature neonates - Low birth weight (<2.5kg): approximately 80% are low birth weight - Evidence of maternal chorioamnionitis Patients typically present with a subacute onset of: - Respiratory distress (85%) - -Grunting - -Nasal flaring - -Use of accessory respiratory muscles - -Tachypnoea - Tachycardia: common, but non-specific - Apnoea (40%) - Jaundice (35%) - Seizures (35%): if cause of sepsis is meningitis - Poor/reduced feeding (30%) - Abdominal distention (20%) - Vomiting (25%) - Temperature: not usually a reliable sign as the temperature can vary from being raised, lowered or normal - -Term infants are more likely to be febrile - -Pre-term infants are more likely to be hypothermic - The clinical presentation can vary from very subtle signs of illness to clear septic shock - Frequently, the symptoms will be related to the source of infection (e.g. pneumonia + respiratory symptoms, meningitis + neurological symptoms) Investigations: - Blood culture: this will usually establish the diagnosis, as the sensitivity for septicaemia is around 90%. Should usually obtain two blood cultures if possible to distinguish from contamination, however one culture is still sufficient depending on hospital protocol - Full blood examination: neonatal sepsis is often associated with abnormal neutrophil counts (both neutrophilia and neutropenia), however in neonates, parameters on full blood examination are usually not always useful for diagnosis, rather may help to exclude healthy neonates - C-reactive protein: not useful for diagnosis, but sequential assessment will help to guide management and patient progress with treatment. CRP will usually be raised, and a persistently normal level is likely to exclude neonatal sepsis - Blood gases: metabolic acidosis is particularly concerning for neonatal sepsis, particularly a base deficit of ≥10 mmol/L - Urine microscopy, culture and sensitivity: will show signs of infection (e.g. raised leukocytes, positive culture, haematuria, proteinuria) if urinary tract infection is the source of sepsis - Lumbar puncture: particularly if there is concern of meningitis as the source of sepsis based on clinical features, however many hospitals will require lumbar puncture as part of a septic screen in any baby below the age of 28 days Management - In neonatal sepsis, the most important part of management to improve outcomes is early identification and treatment - The NICE guidelines recommend use of intravenous benzylpenicillin with gentamicin as a first-line regimen for suspected or confirmed neonatal sepsis - -The exception to this is if microbiological surveillance data reveal local bacterial resistance patterns, in which case an alternative antibiotic should be considered - -CRP should be re-measured 18–24 hours after presentation in babies given antibiotics to monitor ongoing progress and guide duration of therapy - -The evidence suggests that antibiotics can be ceased at 48 hours in neonates who have CRP of <10 mg/L and a negative blood culture at presentation and at 48 hours - -In all other neonates, the duration of antibiotic treatment will depend on the ongoing investigations and clinical picture, this will involve specialist decision making. Normally, in neonates with culture-proven sepsis, duration will be approximately 10 days - Other important management factors to consider include: - -Maintaining adequate oxygenation status - -Maintaining normal fluid and electrolyte status: severely ill neonates may require volume and/or vasopressor support. Body weight needs to be measured daily for accurate assessment of fluid status - -Prevention and/or management of hypoglycaemia - -Prevention and/or management of metabolic acidosis

Answer 181

23-24 months

Answer 182

Oral mebendazole

Answer 183

Prader-Willi syndrome is an example of genetic imprinting where the phenotype depends on whether the deletion occurs on a gene inherited from the mother or father: - Prader-Willi syndrome if gene deleted from father - Angelman syndrome if gene deleted from mother Prader-Willi syndrome is associated with the absence of the active Prader-Willi gene on the long arm of chromosome 15. This may be due to: - microdeletion of paternal 15q11-13 (70% of cases) - maternal uniparental disomy of chromosome 15 Features - hypotonia during infancy - dysmorphic features - short stature - hypogonadism and infertility - learning difficulties - childhood obesity - behavioural problems in adolescence

Answer 184

before 12 months | May indicate cerebral palsy

Answer 185

Therefore, they have the right to agree to a treatment without a parent's consent, but if they choose to refuse it, you must weigh up their best interests. e.g. if the patient has diabetic ketoacidosis, the decision to leave would most likely be fatal and so it would be fair to make the decision that it is in her best interests. (The General medical council's ethical guidance states: 'Parents cannot override the competent consent of a young person to treatment that you consider is in their best interests. But you can rely on parental consent when a child lacks the capacity to consent.)

Answer 186

reduced perfusion to the hip

Answer 187

Age <6, the prognosis is good with no intervention - only observation

Answer 188

Surgical repair of Perthes' disease is only indicated in children age >6.

Answer 189

- face: upslanting palpebral fissures, epicanthic folds, Brushfield spots in iris, protruding tongue, small low-set ears, round/flat face - flat occiput - single palmar crease, pronounced 'sandal gap' between big and first toe - hypotonia - congenital heart defects (40-50%, see below) - duodenal atresia - Hirschsprung's disease

Answer 190

- multiple cardiac problems may be present - endocardial cushion defect (most common, 40%, also known as atrioventricular septal canal defects) - ventricular septal defect (c. 30%) - secundum atrial septal defect (c. 10%) - tetralogy of Fallot (c. 5%) - isolated patent ductus arteriosus (c. 5%)

Answer 191

- subfertility: males are almost always infertile due to impaired spermatogenesis. Females are usually subfertile, and have an increased incidence of problems with pregnancy and labour - learning difficulties - short stature - repeated respiratory infections (+hearing impairment from glue ear) - acute lymphoblastic leukaemia - hypothyroidism - Alzheimer's disease - atlantoaxial instability

Answer 192

Atlantoaxial instability - people with Down syndrome who participate in sports that may carry an increased risk of neck dislocation (e.g. trampolining, gymnastics, boxing, diving, rugby and horse riding)

Answer 193

it demonstrates a cone with dilated ganglionic proximal colon and the distal aganglionic bowel failing to distend.

Answer 194

A plain abdominal x ray will demonstrate dilated loops of bowel with fluid levels

Answer 195

X -Ray of the abdomen shows distended loops of intestine and gas bubbles may be seen in the bowel wall.

Answer 196

Premature infants

Answer 197

x Ray of the abdomen shows distended coils of bowel and typical mottled ground glass appearance. Fluid levels are scarce as the meconium is viscid.

Answer 198

Indicative of reduced respiratory effort in asthma as is, therefore, a life-threatening sign

Answer 199

``` SpO2 < 92% PEF 33-50% best or predicted Too breathless to talk or feed Heart rate >125 (>5 years) >140 (1-5 years) Respiratory rate >30 breaths/min (>5 years) >40 (1-5 years) Use of accessory neck muscles ```

Answer 200

``` SpO2 <92% PEF <33% best or predicted Silent chest Poor respiratory effort Agitation Altered consciousness Cyanosis ```

Answer 201

IV amoxicillin + IV cefotaxime | give IV amoxicillin in addition to cefotaxime to cover for Listeria

Answer 202

Achondroplasia is an autosomal dominant disorder associated with short stature. It is caused by a mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene. This results in abnormal cartilage giving rise to: - short limbs (rhizomelia) with shortened fingers (brachydactyly) - large head with frontal bossing and narrow foramen magnum - midface hypoplasia with a flattened nasal bridge - 'trident' hands - lumbar lordosis In most cases (approximately 70%) it occurs as a sporadic mutation. The main risk factor is advancing parental age at the time of conception. Once present it is typically inherited in an autosomal dominant fashion. Treatment There is no specific therapy. However, some individuals benefit from limb lengthening procedures. These usually involve application of Ilizarov frames and targeted bone fractures. A clearly defined need and end point is the cornerstone of achieving success with such procedures.

Answer 203

Presenting features - neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice - recurrent chest infections (40%) - malabsorption (30%): steatorrhoea, failure to thrive - other features (10%): liver disease Whilst many patients are picked up during newborn screening programmes or early childhood, it is worth remembering that around 5% of patients are diagnosed after the age of 18 years. Other features of cystic fibrosis - short stature - diabetes mellitus - delayed puberty - rectal prolapse (due to bulky stools) - nasal polyps - male infertility, female subfertility

Answer 204

steatorrhoea due to fat malabsorption -usually due to insufficiency of pancreatic enzymes once the disease process has affected the pancreas Development of diabetes is evidence of pancreatic disease involvement as insulin production has been affected.

Answer 205

auditory brainstem response test as a newborn/infant This involves three small sensors being placed on the baby's head and neck, and soft headphones are used to play quiet clicking sounds; the sensors detect how your baby's brain and hearing nerves respond to the sound and a computer analyses the results.

Answer 206

Patau syndrome

Answer 207

Trisomy 13 - Microcephalic, small eyes - Cleft lip/palate - Polydactyly - Scalp lesions Like many of the chromosomal defects, physical and mental disability is common, in this case key distinguishing features to separate Patau's from other trisomy disorders include polydactyly, cleft lips and palates, microcephaly and microphthalmia. Many children die before within a year of birth but those who survive will often go on to show intellectual and motor disability.

Answer 208

Alpha-thalassaemia is due to a deficiency of alpha chains in haemoglobin Overview 2 separate alpha-globulin genes are located on each chromosome 16 Clinical severity depends on the number of alpha globulin alleles affected: If 1 or 2 alpha globulin alleles are affected then the blood picture would be hypochromic and microcytic, but the Hb level would be typically normal If are 3 alpha globulin alleles are affected results in a hypochromic microcytic anaemia with splenomegaly. This is known as Hb H disease If all 4 alpha globulin alleles are affected (i.e. homozygote) then death in utero (hydrops fetalis, Bart's hydrops)

Answer 209

Mesenteric adenitis is inflamed lymph nodes within the mesentery. It can cause similar symptoms to appendicitis and can be difficult to distinguish between the two. It often follows a recent viral infection and needs no treatment

Answer 210

The seizure pattern in west's syndrome are known as infantile spasms, and to the family they can appear to look like colic. In this case, it is important to understand there is a definitive pathology due to the abnormal EEG and MRI, with hypsarrhythmia being classical of west's syndrome.

Answer 211

Sudden infant death syndrome is the commonest cause of death in the first year of life. It is most common at 3 months of age. Major risk factors - putting the baby to sleep prone: the relative risk or odds ratio varies from 3.5 - 9.3. If not accustomed to prone sleeping (i.e. the baby usually sleeps on their back) the odds ratio increases to 8.7-45.4 - parental smoking: studies suggest this increases the risk up to 5 fold - prematurity: 4-fold increased risk - bed sharing: odds ratio 5.1 - hyperthermia (e.g. over-wrapping) or head covering (e.g. blanket accidentally moves) Following a cot death siblings should be screened for potential sepsis and inborn errors of metabolism.

Answer 212

- male sex - multiple births - social classes IV and V - maternal drug use incidence increases in winter

Answer 213

- breastfeeding - room sharing (but not bed sharing, which is a significant risk factor) - the use of dummies (pacifiers)

Answer 214

- Full blood count - Blood culture - C-reactive protein - Urine testing for urinary tract infection - Chest radiograph only if respiratory signs are present - Stool culture, if diarrhoea is present

Answer 215

Measles is now rarely seen in the developed world following the adoption of immunisation programmes. Outbreaks are occasionally seen, particularly when vaccinations rates drop, for example after the MMR controversy of the early 2000s. Overview - RNA paramyxovirus - spread by droplets - infective from prodrome until 4 days after rash starts - incubation period = 10-14 days Features - prodrome: irritable, conjunctivitis, fever - Koplik spots (before rash): white spots ('grain of salt') on buccal mucosa - rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent Investigations - IgM antibodies can be detected within a few days of rash onset Management mainly supportive admission may be considered in immunosuppressed or pregnant patients notifiable disease → inform public health Complications - otitis media: the most common complication - pneumonia: the most common cause of death - encephalitis: typically occurs 1-2 weeks following the onset of the illness) - subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness - febrile convulsions - keratoconjunctivitis, corneal ulceration - diarrhoea - increased incidence of appendicitis - myocarditis Management of contacts - if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced measles antibody develops more rapidly than that following natural infection) - this should be given within 72 hours

Answer 216

DSM-V defines ADHD as a condition incorporating features relating to inattention and/or hyperactivity/impulsivity that are persistent. Like many paediatric conditions, there has to be an element of developmental delay. For children up to the age of 16 years, six of these features have to be present; in those aged 17 or over, the threshold is five features. Diagnostic features : INATTENTION : doesn't follow through on instructions, reluctant to engage in mentally- intense tasks, easily distracted, finds it difficult ti organise tasks or activities. often forgetful in daily activities, often loses things necessary for tasks to activities, often does not seem to listen when spoken to directly HYPERACTIVITY/IMPULSIVITY - unable to play quietly, talks excessively, dos not wait their turn easily, will spontaneously leave their seat when expect to sit, if often 'on the go', often interruptive or intrusive to others, will answer prematurely - before a question has been finished, will run and climb in situations where it is not appropriate

Answer 217

NICE stipulates a holistic approach to treating ADHD that isn't entirely reliant on therapeutics. Following presentation, a ten-week 'watch and wait' period should follow to observe whether symptoms change or resolve. If they persist then referral to secondary care is required. This is normally to a paediatrician with a special interest in behavioural disorders, or to the local Child and Adolescent Mental Health Service (CAMHS). Here, the needs and wants of the patient, as well as how their condition affects their lives should be taken into account, to offer a tailored plan of action. Drug therapy should be seen as a last resort and is only available to those aged 5 years or more. Patients with mild/moderate symptoms can usually benefit from their parents attending education and training programmes. For those who fail to respond, or whose symptoms are severe, pharmacotherapy can be considered: - Methylphenidate is first line in children and should initially be given on a six-week trial basis. It is a CNS stimulant which primarily acts as a dopamine/norepinephrine reuptake inhibitor. Side-effects include abdominal pain, nausea and dyspepsia. In children, weight and height should be monitored every 6 months - If there is inadequate response, switch to lisdexamfetamine; - Dexamfetamine should be started in those who have benefited from lisdexamfetamine, but who can't tolerate its side effects. In adults: - Methylphenidate or lisdexamfetamine are first-line options; - Switch between these drugs if no benefit is seen after a trial of the other. All of these drugs are potentially cardiotoxic. Perform a baseline ECG before starting treatment, and refer to a cardiologist if there is any significant past medical history or family history, or any doubt or ambiguity. Like most psychiatric conditions, whether adult or paediatric, a thorough history and clinical examination are key, especially given the overlap of ADHD with many other psychiatric and physical conditions.

Answer 218

Distinguishing features of a cephalhaematoma are that they usually develop AFTER birth and do not cross the suture lines of the skull as the blood is confined between the skull and periosteum. Caput succedaneum is an extraperiosteal collection of blood therefore can cross over the suture lines and can be present AT birth.

Answer 219

Rotavirus is an oral, live attenuated vaccine - is given orally at 2 and 3 months of age - Two doses are required.

Answer 220

(means not passed meconium) - CF - Hirschsprung's disease - meconium plug syndrome.

Answer 221

Meconium ileus (in 20% of babies with CF)

Answer 222

Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old. Associations - Beckwith-Wiedemann syndrome - as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation - hemihypertrophy - around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11 Features - abdominal mass (most common presenting feature) - painless haematuria flank pain - other features: anorexia, fever - unilateral in 95% of cases - metastases are found in 20% of patients (most commonly lung) Referral - children with an unexplained enlarged abdominal mass in children - possible Wilm's tumour - arrange paediatric review with 48 hours Management - nephrectomy - chemotherapy - radiotherapy if advanced disease - prognosis: good, 80% cure rate

Answer 223

trisomy 18 or Edward's syndrome

Answer 224

Newborn babies are relatively deficient in vitamin K. This may result in impaired production of clotting factors which in turn can lead to haemorrhagic disease of the newborn (HDN). Bleeding may range from minor brushing to intracranial haemorrhages Breast-fed babies are particularly at risk as breast milk is a poor source of vitamin K. Maternal use of antiepileptics also increases the risk Because of this all newborns in the UK are offered vitamin K, either intramuscularly or orally

Answer 225

Infantile colic describes a relatively common and benign set of symptoms seen in young infants. It typically occurs in infants less than 3 months old and is characterised by bouts of excessive crying and pulling-up of the legs, often worse in the evening. Infantile colic occurs in up to 20% of infants. The cause of infantile colic is unknown. NICE Clinical Knowledge Summaries do not recommend the use of simeticone (such as Infacol®) or lactase (such as Colief®) drops.

Answer 226

Hypospadias is a congenital abnormality of the penis which occurs in approximately 3/1,000 male infants Hypospadias is characterised by: - a distal ventral urethral meatus - a hooded prepuce - chordee (ventral curvature of the penis) in more severe forms - the urethral meatus may open more proximally in the more severe variants. However, 75% of the openings are distally located. Hypospadias most commonly occurs as an isolated disorder. Associated urological abnormalities may be seen in up to 40% of infants, of these cryptorchidism is the most frequent (10%). Corrective surgery is performed before 2 years of age. It is essential that the child is not circumcised prior to the surgery as the foreskin may be used in the corrective procedure. In boys with very distal disease no treatment may be needed.

Answer 227

Otitis media

Answer 228

'6-1 vaccine' (diphtheria, tetanus, whooping cough, polio, Hib and hepatitis B) Oral rotavirus vaccine Men B

Answer 229

Innocent murmur They are Soft, Systolic, Short, Symptomless, Standing/Sitting (vary with position)

Answer 230

pansystolic murmur

Answer 231

triad of: 1. retinal haemorrhages, 2.subdural haematoma 3. encephalopathy This is caused by the intentional shaking of a child (0-5 years old). The diagnosis of shaken baby syndrome has often made the headlines due to the controversy amongst physicians as to whether the mechanism of injury is definitely an intentional shaking of a child. This has often resulted in difficulty for the courts to convict suspects of causing shaken baby syndrome to a child.

Answer 232

A benign murmur heard in children and sounds like a continuous blowing noise heard below the clavicles Due to the turbulent blood flow in the great veins returning to the heart

Answer 233

Venous hum

Answer 234

A single dose of oral dexamethasone (0.15 mg/kg) is to be taken immediately regardless of severity

Answer 235

A child still being drowsy after 1 hour is not consistent with a 'simple' febrile convulsion. should prompt referral to paediatrics

Answer 236

At 12 - 13 months

Answer 237

Plain X ray of BOTH hips (AP and frog-leg views) Since SUFE is bilateral in 20% of cases, both hips should be imaged to exclude involvement of the other hip, even if there are no suggestive symptoms currently. The diagnosis can be confirmed and graded if Klein's line (drawn along the superior edge of the femoral neck) intersects less of the femoral head.

Answer 238

Congenital disorder with an incidence of 1 in 7000 births. - Posterior nasal airway occluded by soft tissue or bone. - Associated with other congenital malformations e.g. coloboma - Babies with unilateral disease may go unnoticed. - Babies with bilateral disease will present early in life as they are obligate nose breathers. - In choanal atresia the episodes of cyanosis are usually worst during feeding. Improvement may be seen when the baby cries as the oropharyngeal airway is used. - Treatment is with fenestration procedures designed to restore patency.

Answer 239

if admission is not needed should prescribe Abx Azithromycin or clarithromycin if the onset of cough is within the previous 21 days (for for children aged 1 month or older, and non-pregnant adults) As well as the antibiotics, patients should be advised to rest, take adequate fluid intake, and use paracetamol or ibuprofen for symptomatic relief

Answer 240

Younger than 6 months of age Have significant breathing difficulties (for example apnoea episodes, severe paroxysms, or cyanosis) Have significant complications (for example seizures or pneumonia).

Answer 241

2 factors (1) Liver position (2) Lung-to-head ratio If the liver has herniated into the chest, the disease is more severe and there is lower chance of survival. The lung-to-head ratio is a numeric estimate of the size of the foetal lungs, dependent on the amount of lung visible. A ratio >1.0 reflects a better outcome.

Answer 242

It is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm. This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth. Pathophysiology usually represents a failure of the pleuroperitoneal canal to close completely The most common type of CDH is a left-sided posterolateral Bochdalek hernia which accounts for around 85% of cases. Only around 50% of newborns with CDH survive despite modern medical intervention.

Answer 243

Group B streptococcus usually acquired from the mother at birth. More common in low birth weight babies and following prolonged rupture of the membranes Other causative organisms for a neonatal are: E. coli and other Gram -ve organisms Listeria monocytogenes

Answer 244

Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes). It is more common in children aged 2 - 6 years with the peak incidence being at 4 years. Scarlet fever is spread via the respiratory route by inhaling or ingesting respiratory droplets or by direct contact with nose and throat discharges, (especially during sneezing and coughing). Scarlet fever has an incubation period of 2-4 days and typically presents with: - fever: typically lasts 24 to 48 hours - malaise, headache, nausea/vomiting - sore throat - 'strawberry' tongue - rash - -fine punctate erythema ('pinhead') which generally appears first on the torso and spares the palms and soles - -children often have a flushed appearance with circumoral pallor. The rash is often more obvious in the flexures - -it is often described as having a rough 'sandpaper' texture - -desquamination occurs later in the course of the illness, particularly around the fingers and toes Diagnosis - a throat swab is normally taken but antibiotic treatment should be commenced immediately, rather than waiting for the results Management - oral penicillin V for 10 days - patients who have a penicillin allergy should be given azithromycin - children can return to school 24 hours after commencing antibiotics - scarlet fever is a notifiable disease Scarlet fever is usually a mild illness but may be complicated by: - otitis media: the most common complication - rheumatic fever: typically occurs 20 days after infection - acute glomerulonephritis: typically occurs 10 days after infection - invasive complications (e.g. bacteraemia, meningitis, necrotizing fasciitis) are rare but may present acutely with life-threatening illness

Answer 245

Face and trunk

Answer 246

Kawasaki disease

Answer 247

Turner syndrome

Answer 248

(5Ts) - Tetralogy of fallot - Transposition of great vessels (TGA) - Tricuspid atresia - Total anomalous pulmonary venous return - Truncus arteriosus

Answer 249

TGA generally presents within hours to days of the neonate's birth

Answer 250

prostaglandin E1 (alprostadil) - is infused to prevent closure of the patent ductus arteriosus until a surgical correction can be carried out. - This will allow mixing of deoxygenated and oxygenated blood so as to provide adequate systemic circulation. Antibiotics should also be given as prophylaxis for bacterial endocarditis.

Answer 251

nonsteroidal anti-inflammatory drugs (NSAID)

Answer 252

Peripheral cyanosis, for example of the feet and hands, is very common in the first 24 hours of life and may occur when the child is crying or unwell from any cause Central cyanosis can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds 5g/dl The NITROGEN WASHOUT TEST (also known as the hyperoxia test) may be used to differentiate cardiac from non-cardiac causes. The infant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease !!

Answer 253

- supportive care - prostaglandin E1 - -used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect

Answer 254

Acrocyanosis is often seen in healthy newborns and refers to the peripheral cyanosis around the mouth and the extremities (hands and feet) (picture 1). It is caused by benign vasomotor changes that result in peripheral vasoconstriction and increased tissue oxygen extraction and is a benign condition. Acrocyanosis is differentiated from other causes of peripheral cyanosis with significant pathology (eg, septic shock) as it occurs immediately after birth in healthy infants. It is a common finding and may persist for 24 to 48 hours.

Answer 255

No | There is no evidence that giving a pyrexial child antipyretics reduces the chance of a febrile convulsion

Answer 256

Acute onset Usually accompanies viral infections, but the child is well or has a mild fever More common in boys, aged 2-12 years

Answer 257

Urgent hospital assessment Urgent assessment should be arranged for a child < 3 years presenting with an acute limp. they are at higher risk of septic arthritis and child maltreatment. 'transient synovitis is rare in this age-group and the diagnosis should be made with extreme caution after excluding serious causes of limp. Urgent referral for assessment is advised because examination may be difficult and clinical signs subtle.'

Answer 258

CONDUCTIVE - secretory otitis media - Down's syndrome* - (*may have elements of sensorineural loss as well) SENSORINUERAL - hereditary - Usher syndrome, Pendred syndrome, Jervell-Lange-Nielson syndrome, Wardenburg syndrome - congenital infection e.g. rubella - acquired - meningitis, head injury - cerebral palsy - perinatal insult

Answer 259

Basic physiology - initially gonads in fetus are undifferentiated - on the Y chromosome there is a sex-determining gene (SRY gene) which causes differentiation of the gonad into a testis - if absent (i.e. in a female) then the gonads differentiate to become ovaries Most common cause in newborns is CONGENITAL ADRENAL HYPERPLASIA Other causes include: - true hermaphroditism - maternal ingestion of androgens

Answer 260

Following birth, the first step is to DRY THE BABY maintain temperature and start the clock.

Answer 261

No increased risk | no male-to-male transmission on X-linked recessive conditions

Answer 262

Management is through supportive measures, including calamine lotion to soothe the itch and paracetamol to control the fever.

Answer 263

glandular fever

Answer 264

Bruising at birth can lead to elevated bilirubin levels (due to hemolysis)

Answer 265

Whooping cough is infectious. | A child with whooping cough should be excluded from school for 48 hours following commencement of antibiotics

Answer 266

Methylphenidate (ritalin) - he first-line treatment for ADHD, to be initiated following a period of watchful waiting, behavioural advice and educational programme for the parents/carers.

Answer 267

pyloric stenosis

Answer 268

Hypochloremic hypokalemic metabolic alkalosis

Answer 269

Head lice (also known as pediculosis capitis or 'nits') is a common condition in children caused by the parasitic insect Pediculus capitis, which lives on and among the hair of the scalp of humans. Head lice are small insects that live only on humans, they feed on our blood. Eggs are grey or brown and about the size of a pinhead. The eggs are glued to the hair, close to the scalp and hatch in 7 to 10 days. Nits are the empty egg shells and are white and shiny. They are found further along the hair shaft as they grow out. Head lice are spread by direct head-to-head contact and therefore tend to be more common in children because they play closely together. They cannot jump, fly or swim! When newly infected, cases have no symptoms but itching and scratching on the scalp occurs 2 to 3 weeks after infection. There is no incubation period. Diagnosis - fine-toothed combing of wet or dry hair Management - treatment is only indicated if living lice are found - a choice of treatments should be offered - malathion, wet combing, dimeticone, isopropyl myristate and cyclomethicone - household contacts do not need to be treated unless they too are affected School exclusion is not advised for children with head lice

Answer 270

Surfactant deficient lung disease (SDLD, also known as respiratory distress syndrome and previously as hyaline membrane disease) is a condition seen in premature infants. It is caused by insufficient surfactant production and structural immaturity of the lungs The risk of SDLD decreases with gestation - 50% of infants born at 26-28 weeks - 25% of infants born at 30-31 weeks Other risk factors for SDLD include - male sex - diabetic mothers - Caesarean section - second born of premature twins Clinical features are those common to respiratory distress in the newborn, i.e. tachypnoea, intercostal recession, expiratory grunting and cyanosis Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border Management - prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation - oxygen - assisted ventilation - exogenous surfactant given via endotracheal tube

Answer 271

A strawberry tongue can be seen in both scarlet fever and Kawasaki disease.

Answer 272

Diagnostic features for Kawasaki disease requires a fever >5d with 4 of the following criteria: A) Conjunctival injection B) Mucous membrane changes (dry cracked lips, strawberry tongue) C) Cervical lymphadenopathy D) Polymorphous rash E) Red and oedematous palms/soles, peeling of fingers and toes.

Answer 273

Hyperactivity and short stature are associated with Perthes disease.

Answer 274

DTaP/IPV/Hib/Hep B + Men B (no rotavirus) (The immunisation schedule was changed in 2020 so that infants now receive 2 doses of the PCV vaccine at 3 months and 12 months, instead of the previous 3 doses at 2, 4 and 12 months.)

Answer 275

- Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day - Lymphadenopathy: suboccipital and postauricular

Answer 276

- Fever initially - Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular - Systemic upset is usually mild

Answer 277

- Prodrome: irritable, conjunctivitis, fever - Koplik spots: white spots ('grain of salt') on buccal mucosa - Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent

Answer 278

- Fever, malaise, muscular pain | - Parotitis ('earache', 'pain on eating'): unilateral initially then becomes bilateral in 70%

Answer 279

- Also known as fifth disease or 'slapped-cheek syndrome' - Caused by parvovirus B19 - Lethargy, fever, headache - 'Slapped-cheek' rash spreading to proximal arms and extensor surfaces

Answer 280

- Reaction to erythrogenic toxins produced by Group A haemolytic streptococci - Fever, malaise, tonsillitis - 'Strawberry' tongue - which is when the tongue is covered with a white coat through which red papillae may be seen. Later, the white covering disappears, leaving the tongue with a beefy red appearance - Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor)

Answer 281

- Caused by the coxsackie A16 virus - Mild systemic upset: sore throat, fever - Vesicles in the mouth and on the palms and soles of the feet

Answer 282

- Loss of consciousness lasting more than 5 minutes (witnessed) - Amnesia (antegrade or retrograde) lasting more than 5 minutes - Abnormal drowsiness - Three or more discrete episodes of vomiting - Clinical suspicion of non-accidental injury - Post-traumatic seizure but no history of epilepsy - GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department - Suspicion of open or depressed skull injury or tense fontanelle - Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign) - Focal neurological deficit - If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head - Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)

Answer 283

Exomphalmos should have a gradual repair (over 6-12 months) to prevent respiratory complications. The prognosis is good if operated on as soon as possible and whilst waiting the bowel should be protected with cling-film. Intestinal function will take time to normalise and thus the child may require TPN for a few weeks. Gastroschisis requires urgent correction

Answer 284

Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord In exomphalos the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum

Answer 285

most commonly caused by coxsackie A16 and enterovirus

Answer 286

Hand, foot and mouth disease is a self-limiting condition affecting children. It is caused by the intestinal viruses of the Picornaviridae family (most commonly coxsackie A16 and enterovirus 71). It is very contagious and typically occurs in outbreaks at nursery Clinical features - mild systemic upset: sore throat, fever - oral ulcers - followed later by vesicles on the palms and soles of the feet Management - symptomatic treatment only: general advice about hydration and analgesia - reassurance no link to disease in cattle - children do not need to be excluded from school - -the HPA recommends that children who are unwell should be kept off school until they feel better - -they also advise that you contact them if you suspect that there may be a large outbreak.

Answer 287

Herpes virus 6

Answer 288

Maculopapular rash which starts on the chest and spreads to the limbs

Answer 289

Congenital diaphragmatic hernia - Pulmonary hypoplasia in CDH occurs alongside the hernial development rather than as a direct result of it, as part of a sequence

Answer 290

Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs Causes include - oligohydramnios ( It is believed that oligohydramnios decreases the size of the intrathoracic cavity, thus preventing foetal lung growth. ) - congenital diaphragmatic hernia

Answer 291

In neonatal practice the vast majority of IVH occur in the first 72 hours after birth, the aetiology is not well understood and it is suggested to occur as a result of birth trauma combined with cellular hypoxia, together the with the delicate neonatal CNS. Is largely supportive, therapies such as intraventricular thrombolysis and prophylactic CSF drainage have been trialled and not demonstrated to show benefit. Hydrocephalus and rising ICP is an indication for shunting

Answer 292

Diastolic Machinery murmur at the upper left sternal edge

Answer 293

The murmur does not vary with position and hence is not an innocent murmu

Answer 294

cystic fibrosis

Answer 295

FISH studies

Answer 296

the development of secondary sexual characteristics before 9 years of age

Answer 297

12-15 months

Answer 298

Receive PCV at 3 months and 12 months (it has changed in 2020)

Answer 299

Kawasaki disease | Desquamated - means peeling

Answer 300

No exclusion

Answer 301

``` Colour: -Pale/mottled/ashen/blue No response to social cues Activity: - Appears ill to a healthcare professional - Does not wake or if roused does not stay awake - Weak, high-pitched or continuous cry Respiratory: - Grunting - Tachypnoea: respiratory rate >60 breaths/minute - Moderate or severe chest indrawing Circulation and hydration: - Reduced skin turgor Other: - Age <3 months, temperature >=38°C - Non-blanching rash - Bulging fontanelle - Neck stiffness - Status epilepticus - Focal neurological signs - Focal seizures ```

Answer 302

Haemophilia is a X-linked recessive disorder and would hence be expected only to occur in males. As patients with Turner's syndrome only have one X chromosome however, they may develop X-linked recessive conditions

Answer 303

Do not use corticosteroids in children younger than 3 months with suspected or confirmed bacterial meningitis

Answer 304

Seborrhoeic dermatitis is a relatively common skin disorder seen in children. It typically affects the scalp ('Cradle cap'), nappy area, face and limb flexures. Cradle cap is an early sign which may develop in the first few weeks of life. It is characterised by an erythematous rash with coarse yellow scales. Management depends on severity - mild-moderate: baby shampoo and baby oils - severe: mild topical steroids e.g. 1% hydrocortisone Seborrhoeic dermatitis in children tends to resolve spontaneously by around 8 months of age

Answer 305

Treated with oral azithromycin within the first 21 days of symptoms. If the patient had presented later than this, then no antibiotic therapy would have been needed Report to Public Health England - is a notifiable disease

Answer 306

Between fifth and ninth day of life

Answer 307

The following conditions are currently screened for: - congenital hypothyroidism - cystic fibrosis - sickle cell disease - phenylketonuria - medium chain acyl-CoA dehydrogenase deficiency (MCADD) - maple syrup urine disease (MSUD) - isovaleric acidaemia (IVA) - glutaric aciduria type 1 (GA1) - homocystinuria (pyridoxine unresponsive) (HCU)

Answer 308

A viral illness

Answer 309

``` Precocious puberty in males definition: development of secondary sexual characteristics before 8 years much less common than in females usually organic cause testicular size often provides a clue ``` May be classified into: 1. Gonadotrophin dependent ('central', 'true') - due to premature activation of the hypothalamic-pituitary-gonadal axis - FSH & LH raised - testes enlarged Causes of gonadotrophin dependent precocious puberty in males - CNS lesions: craniopharyngioma, hydrocephalus, neurofibroma, tuberous sclerosis - hCG secretion hepatoblastoma - primary hypothyroidism (increased TSH stimulates FSH receptors) 2. Gonadotrophin independent ('pseudo', 'false') - due to excess sex hormones - FSH & LH low - testes may be small (e.g. adrenal tumour or hyperplasia) or unilaterally enlarged e.g. Leydig cell tumour Testes - bilateral enlargement = gonadotrophin release from intracranial lesion - unilateral enlargement = gonadal tumour - small testes = adrenal cause (tumour or adrenal hyperplasia) Females - usually idiopathic or familial and follows normal sequence of puberty Organic causes - are rare, associated with rapid onset, neurological symptoms and signs and dissonance - e.g. McCune Albright syndrome

Answer 310

BCG vaccination against TB should be offered to babies with family history of TB or from high risk region/country with TB (defined as >40 cases/100000) as defined by WHO BCG vaccination is recommended for babies up to one year of age who: are born in areas of the UK with high rates of TB have a parent or grandparent who was born in a country with high rates of TB

Answer 311

Croup Acute epiglottitis Inhaled foreign body Laryngomalacia

Answer 312

Diphtheria, tetanus, polio + Men ACWY

Answer 313

(C)aput (S)uccedaneum= (C)rosses (S)uture lines

Answer 314

- bronchopulmonary dysplasia (e.g. Premature) - congenital heart disease - cystic fibrosis

Answer 315

Barlow manoeuvre: attempted dislocation of a newborns femoral head

Answer 316

- pale and cold extremities | - prolonged capillary refill time

Answer 317

``` EARLY shock blood pressure -normal heart rate - tachycardia respiration -tachypnoea extremities -pale or mottled urine output -reduced ``` ``` LATE shock BP- hypotension Heart rate- bradycardia respiration -acidotic (Kussmaul) extremities -blue urine output -absent ```

Answer 318

Hypotension is a late sign of shock. In early, compensated shock the blood pressure is maintained by increased heart rate and respiratory rate, redistribution of blood from venous reserve volume and diversion of blood flow from non-essential tissues (which explains why the peripheries will be cold and pale). In late or uncompensated shock, the compensatory mechanism fails, blood pressure falls and lactic acidosis increases.

Answer 319

Bronchopulmonary dysplasia most often affects preterm infants who were treated with supplemental oxygen and ventilation.

Answer 320

Conjugated bilirubin is elevated Liver transaminases and bile acids are both raised in biliary atresia, but this cannot distinguish biliary atresia from other causes of neonatal cholestasis.

Answer 321

Transient tachypnoea of the newborn (TTN) In most cases, if the baby is well, no further investigations or treatment is required. TTN will frequently resolve on its own and parents should be reassured.

Answer 322

On examination you would typically hear a ejection systolic murmur and fixed splitting of the second heart sound.

Answer 323

The majority of atrial septal defects (ASDs) are asymptomatic in children. If these congenital hearts defects are not picked up prenatally then symptomatic patients with severe ASD may experience shortness of breath, lethargy, poor appetite and growth and increased susceptibility to respiratory infections.

Answer 324

Ejection systolic murmur in the upper left sternal border

Answer 325

Crescendo-decrescendo murmur in the upper left sternal border

Answer 326

- nose picking (most common cause) - foreign body - upper respiratory tract infection - allergic rhinitis

Answer 327

Children under the age of 2 years as epistaxis is rare in this age group and may be secondary to trauma or bleeding disorders.

Answer 328

prescribe a short course of topical chlorhexidine and neomycin (is to reduce crusting and vestibulitis), and discourage the child from picking his nose.

Answer 329

Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into the ureter and kidney. It is relatively common abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI Pathophysiology of VUR - ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle - therefore shortened intramural course of ureter - vesicoureteric junction cannot therefore function adequately The table below summarises the grading of VUR Grade: I -Reflux into the ureter only, no dilatation II -Reflux into the renal pelvis on micturition, no dilatation III -Mild/moderate dilatation of the ureter, renal pelvis and calyces IV -Dilation of the renal pelvis and calyces with moderate ureteral tortuosity V -Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity Investigation - VUR is normally diagnosed following a micturating cystourethrogram - a DMSA scan may also be performed to look for renal scarring

Answer 330

Nephrotic syndrome is classically defined as a triad of 1. proteinuria (> 1 g/m^2 per 24 hours) 2. hypoalbuminaemia (< 25 g/l) 3. oedema In children the peak incidence is between 2 and 5 years of age. Around 80% of cases in children are due to a condition called minimal change glomerulonephritis. The condition generally carries a good prognosis with around 90% of cases responding to high-dose oral steroids. Other features include hyperlipidaemia, a hypercoagulable state (due to loss of antithrombin III) and a predisposition to infection (due to loss of immunoglobulins)

Answer 331

monitor weight and height every 6 months Methylphenidate, a stimulant, may suppress appetite and cause growth impairment in children. It is advised to monitor growth as well as blood pressure and pulse in these patients on a regular basis.

Answer 332

A macrolide e.g. erythromicin should be used for children with pneumonia if mycoplasma is suspected

Answer 333

pyloric stenosis. | Diagnosis is most commonly made by abdominal ultrasound.

Answer 334

oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy (for cerebral palsy)

Answer 335

dilated cardiomyopathy

Answer 336

- progressive proximal muscle weakness from 5 years - calf pseudohypertrophy - Gower's sign: child uses arms to stand up from a squatted position - associated with dilated cardiomyopathy - 30% of patients have intellectual impairment

Answer 337

Disordes of sex hormones Androgen insensitivity syndrome is an X-linked recessive condition due to end-organ resistance to testosterone causing genotypically male children (46XY) to have a female phenotype. Complete androgen insensitivity syndrome is the new term for testicular feminisation syndrome Features - 'primary amenorrhoea' - undescended testes causing groin swellings - breast development may occur as a result of conversion of testosterone to oestradiol (The key symptom here is the groin swellings, which combined with 'primary amenorrhoea' and no pubic hair points towards a diagnosis of androgen insensitivity (previously testicular feminisation syndrome). The groin swellings here are undescended testes. This is a condition in which the patient is genetically male (46XY), but phenotypically female. Feminisation is a result of increased oestradiol levels, which lead to breast development) Diagnosis buccal smear or chromosomal analysis to reveal 46XY genotype Management - counselling - raise child as female - bilateral orchidectomy (increased risk of testicular cancer due to undescended testes) - oestrogen therapy

Answer 338

Disorder of sex hormones Kallman's syndrome is a recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism. It is usually inherited as an X-linked recessive trait. Kallman's syndrome is thought to be caused by failure of GnRH-secreting neurons to migrate to the hypothalamus. The clue given in many questions is lack of smell (anosmia) in a boy with delayed puberty Features - 'delayed puberty' - hypogonadism, cryptorchidism - anosmia - sex hormone levels are low - LH, FSH levels are inappropriately low/normal - patients are typically of normal or above average height Cleft lip/palate and visual/hearing defects are also seen in some patients

Answer 339

Disorder of sex hormones Klinefelter's syndrome is associated with karyotype 47, XXY ``` Features often taller than average lack of secondary sexual characteristics small, firm testes infertile gynaecomastia - increased incidence of breast cancer elevated gonadotrophin levels ``` Diagnosis is by chromosomal analysis

Answer 340

``` 1. Primary hypogonadism (Klinefelter's syndrome) LH- High Testosterone - Low 2. Hypogonadotrophic hypogonadism (Kallman's syndrome) LH- Low Testosterone - Low 3. Androgen insensitivity syndrome LH- High Testosterone Normal/high 4. Testosterone-secreting tumour LH- Low Testosterone - High ```

Answer 341

APGAR scores are assessed at 1, 5 and 10 minutes of age

Answer 342

``` Appearance (colour) Pulse (heart rate) Grimace (reflex irritability) Activity (muscle tone) Respiratory effort ```

Answer 343

First-line treatment is short-acting bronchodilator therapy. If this is not successful then either oral montelukast or inhaled corticosteroids should be tried.

Answer 344

1. Plagiocephaly - parallelogram shaped head. Plagiocephaly is a skull deformity producing unilateral occipital flattening, which pushes the ipsilateral forehead ear forwards producing a 'parrallelogram' appearance. - the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the 'Back to Sleep' campaign -encourage babies to sleep on their back to reduce the risk of sudden infant death syndrome (SIDS) - The vast majority improve by age 3-5 due to the adoption of a more upright posture 2. Craniosynostosis - premature fusion of skull bones

Answer 345

does not require exclusion from a childcare setting or school

Answer 346

Maintenance of the ductus arteriosus with prostaglandins (such as alprostadil) to promote duct patency ) is the initial management for duct dependent congenital heart disease Note, ibuprofen should be avoided as it promotes duct closure and would worsen the condition. The initial action here is to prevent duct closure to allow the child to survive long enough to allow investigations to confirm the diagnosis and a subsequent attempt at surgical correction.

Answer 347

``` jittery movements increased muscle tone hyperreflexia convulsions drowsiness or coma ```

Answer 348

- sausage-shaped mass in the right hypochondrium | - emptiness in the right lower quadrant (Dance's sign)

Answer 349

- renal parenchymal disease - renal vascular disease - coarctation of the aorta - phaeochromocytoma - congenital adrenal hyperplasia - essential or primary hypertension (becomes more common as children become older) In younger children secondary hypertension is the most common cause, with renal parenchymal disease accounting for up to 80%

Answer 350

Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl- cotransporter in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine causes of hypokalaemia such as Conn's, Cushing's and Liddle's syndrome which are associated with hypertension) Features - usually presents in childhood, e.g. Failure to thrive - polyuria, polydipsia - hypokalaemia - normotension - weakness

Answer 351

A low-grade fever is typical in bronchiolitis Consider a diagnosis of pneumonia instead if the child has: - high fever (over 39°C) and/or - persistently focal crackles.

Answer 352

Gastroschisis and omphalocele present similarly, but gastroschisis refers to a defect lateral to the umbilicus whereas omphalocele refers to a defect in the umbilicus itself.

Answer 353

Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes).

Answer 354

should be reviewed by a paediatric specialist

Answer 355

- severe immunosuppression - allergy to neomycin - children who have received another live vaccine by injection within 4 weeks - pregnancy should be avoided for at least 1 month following vaccination - immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are present)

Answer 356

the lower jaw is undersized (small chin)

Answer 357

Echocardiogram - Coronary artery aneurysms are a complication of Kawasaki disease

Answer 358

until all the lesions are dry and have crusted over (usually about 5 days after the onset of the rash)

Answer 359

- Meconium aspiration syndrome refers to respiratory distress in the newborn as a result of meconium in the trachea. - It occurs in the immediate neonatal period. It is more common in POST-TERM DELIVERIES, with rates of up to 44% reported in babies born after 42 weeks. - It causes respiratory distress, which can be severe. - Higher rates occur where there is a history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse

Answer 360

- In a child under 1-year-old, the only two places to check for a pulse are brachial and femoral. - In a child 1 and over, the best places to check are femoral and carotid.

Answer 361

- Webbed neck - Pectus excavatum - Short stature - Pulmonary stenosis

Answer 362

an adrenal cause of the symptoms- e.g. adrenal hyperplasia

Answer 363

Osgood-Schlatter disease is a type of osteochondrosis caused by inflammation (apophysitis) at the tibial tuberosity

Answer 364

high-dose aspirin - Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye's syndrome aspirin is normally contraindicated in children intravenous immunoglobulin

Answer 365

The most likely cause for constipation developing at this age is dietary. The most likely cause for constipation developing at this age is dietary.A laxative would also be very useful, and would be indicated due to the progressively worsening constipation. An osmotic laxative should be prescribed initially as the stool is likely to be hard. A stimulant laxative may also be required, but only once the stools are soft.

Answer 366

pO2 after 15 minutes of 100% oxygen is <15kPa. Therefore, this is a cyanotic heart defect

Answer 367

Transposition of the great arteries

Answer 368

No murmur but typically a loud single S2 is audible and a prominent right ventricular impulse is palpable on examination

Answer 369

Transposition of the great arteries (TGA) is a form of cyanotic congenital heart disease. It is caused by the failure of the aorticopulmonary septum to spiral during septation. Children of diabetic mothers are at an increased risk of TGA. Basic anatomical changes: - aorta leaves the right ventricle - pulmonary trunk leaves the left ventricle Clinical features: - cyanosis - tachypnoea - loud single S2 - prominent right ventricular impulse - 'egg-on-side' appearance on chest x-ray Management: - maintenance of the ductus arteriosus with prostaglandins - surgical correction is the definite treatment.

Answer 370

A cutaneous dermoid cyst may develop at sites of embryonic developmental fusion. They are most common in the midline of the neck, external angle of the eye and posterior to the pinna of the ear. They typically have multiple inclusions such as hair follicles that bud out from its walls. They may develop at other sites such as the ovary and in these sites are synonymous with teratomas. A desmoid tumour is a completely different entity and may be classified either as low grade fibrosarcomas or non aggressive fibrous tumours. They commonly present as large infiltrative masses. They may be divided into abdominal, extra abdominal and intra abdominal. All types share the same biological features. Extra abdominal desmoids have an equal sex distribution and primarily arise in the musculature of the shoulder, chest wall, back and thigh. Abdominal desmoids usually arise in the musculoaponeurotic structures of the abdominal wall. Intra abdominal desmoids tend to occur in the mesentery or pelvic side walls and occur most frequently in patients with familial adenomatous polyposis coli syndrome. - Dermoid cysts occur at sites of embryonic fusion and may contain multiple cell types. They occur most often in children.

Answer 371

neonatal hypoglycaemia.

Answer 372

The use of maternal labetalol for HTN is a risk factor and these babies must have their blood glucose measured

Answer 373

stunted growth The mechanism is thought to be through a decrease in appetite. Patients under the age of 10 should have their weight and height plotted at regular intervals. Other side effects of this drug include insomnia, weight loss, anxiety, nausea and pain.

Answer 374

Administer dexamethasone to the mother. Natural maternal glucocorticosteroids are very important for surfactant production in the foetus, and therefore synthetic steroids are the first line agents for preventing NRDS in pregnancies at risk of pre-term birth. Tocolytics are agents that can be used to suppress pre-term labour, however they are not routinely used. Since administration of maternal steroids takes one to two days to increase surfactant levels, tocolytics can be considered in certain situations to buy time.

Answer 375

``` growth hormone deficiency hypothyroidism Down's syndrome Cushing's syndrome Prader-Willi syndrome ```

Answer 376

- orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in bowing of the legs), musculoskeletal pains - psychological consequences: poor self-esteem, bullying - sleep apnoea - benign intracranial hypertension - long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease

Answer 377

endocarditis

Answer 378

Increase in testicular volume

Answer 379

Seborrhoeic dermatitis is a relatively common skin disorder seen in children. It typically affects the scalp ('Cradle cap'), nappy area, face and limb flexures. Cradle cap is an early sign which may develop in the first few weeks of life. It is characterised by an erythematous rash with coarse yellow scales. Management depends on severity - mild-moderate: baby shampoo and baby oils - severe: mild topical steroids e.g. 1% hydrocortisone Seborrhoeic dermatitis in children tends to resolve spontaneously by around 8 months of age

Answer 380

Undescended testis occurs in around 2-3% of term male infants, but is much more common if the baby is preterm. Around 25% of cases are bilateral. Complications of undescended testis: - infertility - torsion - testicular cancer - psychological Management 1. Unilateral undescended testis NICE CKS now recommend referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age Orchidopexy: Surgical practices vary although the majority of procedures are performed at around 1 year of age 2. Bilateral undescended testes Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

Answer 381

more common between 3-12 months

Answer 382

Necrotising enterocolitis (gas cyst pneumatosis intestinalis (intramural gas)? )

Answer 383

Peripheral cyanosis, for example of the feet and hands, is very common in the first 24 hours of life and may occur when the child is crying or unwell from any cause Central cyanosis can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds 5g/dl The nitrogen washout test (also known as the hyperoxia test) may be used to differentiate cardiac from non-cardiac causes. The infant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease Causes of cyanotic congenital heart disease - tetralogy of Fallot (TOF) - transposition of the great arteries (TGA) - tricuspid atresia Initial management of suspected cyanotic congenital heart disease - supportive care - prostaglandin E1 - -used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect

Answer 384

``` Seriously ill 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. ```

Answer 385

Fragile X | Rett's syndrome

Answer 386

All 3 of the following features must be present for a diagnosis to be made - global impairment of language and communication - impairment of social relationships - ritualistic and compulsive phenomena

Answer 387

As per the NICE guidelines if any of the following are observed after any degree of meconium, then baby must be assessed by the neonatal team; respiratory rate above 60 per minute the presence of grunting heart rate below 100 or above 160 beats/minute capillary refill time above 3 seconds temperature of 38°C or above, or 37.5°C on 2 occasions 30 minutes apart oxygen saturation below 95% presence of central cyanosis

Answer 388

micturating cystourethrogram

Answer 389

- Afro-Caribbean infants - Down's syndrome - mucopolysaccharide storage diseases

Answer 390

Reflex anoxic seizure describes a syncopal episode (or presyncope) that occurs in response to pain or emotional stimuli. It is thought to be caused by neurally-mediated transient asystole in children with very sensitive vagal cardiac reflexes. It typically occurs in young children aged 6 months to 3 years Typical features - child goes very pale - falls to floor - secondary anoxic seizures are common - rapid recovery There is no specific treatment and prognosis is excellent

Answer 391

- prone sleeping - parental smoking - bed sharing - hyperthermia and head covering - prematurity

Answer 392

Eczema occurs in around 15-20% of children and is becoming more common. It typically presents before 6 months but clears in around 50% of children by 5 years of age and in 75% of children by 10 years of age Features - in infants the face and trunk are often affected - in younger children eczema often occurs on the extensor surfaces - in older children a more typical distribution is seen, with flexor surfaces affected and the creases of the face and neck Management - avoid irritants - simple emollients: large quantities should be prescribed (e.g. 250g / week), roughly in a ratio of with topical steroids of 10:1. If a topical steroid is also being used the emollient should be applied first followed by waiting at least 30 minutes before applying the topical steroid. Creams soak into the skin faster than ointments. Emollients can become contaminated with bacteria - fingers should not be inserted into pots (many brands have pump dispensers) - topical steroids - in severe cases wet wraps and oral ciclosporin may be used

Answer 393

A congenital cyst found in the mouth. They are common on the hard palate, but may also be seen on the gums where the parents may mistake it for an erupting tooth. in neonates No treatment is generally required as they tend to spontaneously resolve over the course of a few weeks.

Answer 394

Coeliac disease is caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption. Children normally present before the age of 3 years, following the introduction of cereals into the diet Genetics incidence of around 1:100 it is strongly associated with HLA-DQ2 (95% of patients) and HLA-DQ8 (80%) Features may coincide with the introduction of cereals (i.e. gluten) - failure to thrive - diarrhoea - abdominal distension - older children may present with anaemia - many cases are not diagnosed to adulthood Diagnosis - jejunal biopsy showing subtotal villous atrophy - anti-endomysial and anti-gliadin antibodies are useful screening tests IgA TTG antibodies - investigation

Answer 395

ultrasound

Answer 396

the following infants require a routine ultrasound examination: - first-degree family history of hip problems in early life - BREECH presentation at or after 36 weeks gestation, irrespective of presentation at birth or mode of delivery - multiple pregnancy all infants are screened at both the newborn check and also the six-week baby check using the Barlow and Ortolani tests

Answer 397

USS screening required 6 weeks after birth All breech babies at or after 36 weeks gestation require USS for DDH screening at 6 weeks regardless of mode of delivery

Answer 398

Children aged over 12 have similar normal vital signs to an adult

Answer 399

Initial management is through the insertion of a nasogastric tube with the aim of keeping air out of the gut. Therefore for cyanosed pt the best way to assist breathing is to intubate and ventilate. The child needs definitive management in the form of surgical repair of the diaphragm. (if they used a facemark then air would get into the NGT) Would not do: BIPAP and CPAP are airway adjuncts used when the problem is keeping the airway open, such as COPD or respiratory distress syndrome. Facemask ventilation and nasal cannulae would only increase the risk of air entering the gut, the infant needs an artificial airway to ensure they are able to receive oxygen.

Answer 400

In the UK, the vast majority of DDH is diagnosed in newborns. In this age-group, an ultrasound scan of the hip is the first-line investigation. However, there are some cases in which a diagnosis of DDH is suspected in an older child. In a child >4.5 months, an x-ray is the first-line investigation. This is because ossification of the femoral head has occurred, meaning that x-rays are better able to visualise the joint.

Answer 401

CF - can CAUSE the development of diabetes Cystic fibrosis presents in childhood with respiratory symptoms, but as the disease progresses, further features begin to develop. If the pancreas becomes affected, then diabetes mellitus can develop. It can take time for the pancreas to be affected enough to cause diabetes, which is why children with cystic fibrosis may develop diabetes later in life.

Answer 402

It often follows a recent viral infection and needs no treatment

Answer 403

history of coryzal symptoms followed by violent coughing fits is highly suggestive of pertussis. Infants with pertussis may present with apnoeas rather than the classic whoop. Young infants who develop whooping cough often do not have the classic whoop as they are unable to take the large breath required for this after the coughing fit. They instead may have apnoeas with cyanosis

Answer 404

Acute epiglottitis is rare but serious infection caused by Haemophilus influenzae type B. Prompt recognition and treatment is essential as airway obstruction may develop. Epiglottitis was generally considered a disease of childhood but in the UK it is now more common in adults due to the immunisation programme. The incidence of epiglottitis has decreased since the introduction of the Hib vaccine. Features - rapid onset - high temperature, generally - unwell - stridor - drooling of saliva Investigations - chest x-ray - -a lateral view in acute epiglottis will show swelling of the epiglottis - the 'thumb sign' - -in contrast, a posterior-anterior view in croup will show subglottic narrowing, commonly called the 'steeple sign'

Answer 405

high-flow oxygen | nebulised adrenaline

Answer 406

30-60 breaths

Answer 407

anaemia soft systolic murmur and shortness of breath on exertion) - anaemia

Answer 408

ALL - acute lymphoblastic leukaemia

Answer 409

Early disease can be missed on x-ray. MRI is increasingly replacing bone scans as the second line investigation of choice.

Answer 410

aspiration pneumonia

Answer 411

Respiratory distress shortly after birth which usually worsens over the next few days.

Answer 412

Usually presents with tachypnoea shortly after birth and often fully resolves within the first day of life. In TTN the CXR depicts a heart failure type pattern (e.g. interstitial oedema and pleural effusions) but key distinguishing features from congenital heart disease are a normal heart size and rapid resolution of the failure type pattern within days.

Answer 413

urinary symptoms

Answer 414

Hand, foot and mouth disease

Answer 415

Koplik spots: white spots ('grain of salt') on buccal mucosa | In measles

Answer 416

desmopressin Desmopressin is a synthetic replacement for vasopressin or antidiuretic hormone (ADH). Treatment can be considered in children over 5 years, though this has to be considered in line with the child's maturity, motivation, frequency of bedwetting.

Answer 417

large volume, bounding, collapsing pulse

Answer 418

high-dose aspirin | intravenous immunoglobulin

paeds Flashcards

(450 cards)