paeds Flashcards

Question

hw is reflex scored on apgar

Answer 1

2 = cries on stimaulation / coughs / sneezes 1 = grimace 0 = nil

Answer 2

7 or above

Answer 3

Apgar score is therefore 9. This is likely to be describing a case of acrocyanosis — a benign condition that causes peripheral cyanosis immediately after birth in healthy infants and resolves within 48 hours

Answer 4

Enterobius vermicularis

Answer 5

7–10 day course of either oral cephalexin or oral co-amoxiclav.

Answer 6

should be given a 3-day course of oral trimethoprim or oral nitrofurantoin

Answer 7

Hirschsprung disease

Answer 8

leukaemia = abnormal cells accumulate in blood or bone marrow lymphoma = form solid tumours in lypmh nodes, thmus, spleen

Answer 9

ALL = children more likely & Lymphadenopathy +++ CNS involvement +++ Testicular enlargement Thymic enlargement (mediastinum) AML = Lymphadenopathy less common Quick subtype facts: M3: Acute promyelocytic leukaemia 15;17– prone to DIC & bleeding M4+5: Monoblasts/monocytes - Skin / gum infiltration + hypokalaemia

Answer 10

ann-arbor staging Staging (Ann-Arbor) Stage 1 – one LN region (LN region can include spleen) Stage 2 – two or more LN regions on the same side of the diaphragm Stage 3 – two or more LN regions on opposite sides of the diaphragm Stage 4 – extra nodal sites (liver, BM) A: No constitutional symptoms B: Constitutional symptoms E.g. Stage 2a – patient with involvement in 3 LN regions above the diaphragm, pain after alcohol and SVC syndrome but no weight loss, night sweats etc.

Answer 11

salbutomol - burst therapy

Answer 12

mainly supportive humidifed oxygen

Answer 13

respiratory syncytial virus (RSV)

Answer 14

Ophthalmia neonatorum simply means infection of the newborn eye. Responsible organisms include Chlamydia trachomatis Neisseria gonorrhoeae Suspected ophthalmia neonatorum should be referred for same-day ophthalmology/paediatric assessment.

Answer 15

Pharmacological Treatment - if admission is not needed, prescribe an antibiotic if the onset of the cough is within 21 days. o < 1 month old = clarithromycin o >1 months old and not pregnant = azithromycin o If macrolides contraindicated, give co-trimoxazole

Answer 16

neonatal hypoglycaemia

Answer 17

cat 1 = 30 mins cat 2 = 75 mins

Answer 18

can be either

Answer 19

IgA vascultitis (henoch-schonlein purpura)

Answer 20

* Kasai hepatoportoenterostomy ideally within first 60 days of life

Answer 21

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 but low testosterone

Answer 22

Prostaglandin E1 infusion (alprostadil) this keeps ductus arteriosis open

Answer 23

toddler's diarrheoa

Answer 24

Management is with Ramstedt pyloromyotomy

Answer 25

The Kocher criteria for the diagnosis of septic arthritis: fever >38.5 degrees C non-weight bearing raised ESR raised WCC

Answer 26

* Prolonged course of antibiotics (initially IV for 2 weeks, followed by 4 weeks of oral antibiotics) o Neonate to <3 months:  IV cefotaxime o 3 months to /=6 years  IV flucloxacillin  If penicillin allergic, give clindamycin

Answer 27

o IV fluids, NBM, antiemetics, analgesia straight to surgery

Answer 28

kawasaki: - 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 mx = aspirin scarlet fever: - 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 (sandpaper rash)/fine punctate erythema mx= oral penicillin V for 10 days (also called phenoxymethylpenicillin)

Answer 29

kawasaki = 5-14 days after fever scarlet fever = 12-48 hours after fever

Answer 30

you give them together

Answer 31

Ophthalmia neonatorum (conjunctivitis in the neonate) Responsible organisms include * Chlamydia trachomatis * Neisseria gonorrhoeae Management * Mild bacterial conjunctivitis: o Chloramphenicol eye drops * Moderate-severe bacterial conjunctivitis: o Chlamydial (most common):  Oral erythromycin o Gonococcal:  Single dose of parenteral (IV or IM) cefotaxime/ ceftriaxone o Pseudomonal:  Gentamicin eye drops plus systemic antibiotics * Viral: no specific antiviral, may use topical antihistamine and artificial tears to relieve itching * For chlamydia or gonococcal infections, the mother and her sexual partner also require treatment

Answer 32

Prevention * Offer intrapartum benzylpenicillin (or vancomycin if allergic to penicillin) to pregnant women who have had: o a previous baby with an invasive group B streptococcal infection o group B streptococcal colonisation, bacteriuria, or infection in the current pregnancy o are in pre-term labour Neonatal infection management * Penicillin (IV Benzylpenicillin) and gentamicin * First-line antibiotics recommended in NICE NG195 guidelines, but local antibiotic policies may vary

Answer 33

o Resuscitation  A to E approach particularly if preterm, anaemic or hydropic(swelling/taking up fluid) o Phototherapy  if significant jaundice (test with transcutaneous bilirubin) o Exchange transfusion if:  Bilirubin rapidly rising (>8-10 μmol/l/hr) despite adequate phototherapy  Severe hyperbilirubinaemia insufficiently responsive to phototherapy and supportive care  Significant anaemia (Hb <100 g/l) o IVIG  Only for immune haemolysis; if bilirubin continues to rise by > 8.5mmol/L/hour

Answer 34

* Infants of mothers who are HBsAg positive should receive exposure immunization schedule: o Monovalent Hepatitis B vaccine within 24 hours of birth (also at 4 weeks and 1 year of age) o 6-in-1 vaccine (DT/aP/IPV/Hib/HepB) at usual times (8, 12 and 16 weeks) * HBIG should be given to the neonate if: o Mother is HBsAg positive (even if she is HBeAg negative) o Mother had acute hepatitis B during pregnancy o Mother had an HBV DNA level equal or above 1x10^6IUs/ml in any antenatal sample during the current pregnancy * HBIG should be ideally given simultaneously as initial Hep B vaccine, but at a different site * Acute Hep B infection: supportive care

Answer 35

hypoxic ischaemic encephalopathy: Babies born near or at term with HIE can benefit from therapeutic hypothermia

Answer 36

Management * Amoxicillin and gentamicin o If blood cultures or CSF comes back as positive for Listeria

Answer 37

meconium aspiration syndrome more common in post term delivery / also could have meconium stained amniotic fluid * If normal term infant with meconium-stained amniotic fluid but no history of GBS, observation is recommended * If there are risk factors or laboratory findings that are suggestive of infection, consider antibiotics o IV ampicillin AND gentamicin

Answer 38

Necrotising Enterocolitis Management * Stop enteral feeding and medications o TPN may be required if feeds stopped >24 hours o For confirmed NEC cases feeds stopped for 7 days * NG tube o Used to drain fluid and gas from the gut o Monitor hourly gastric aspirates * Broad-spectrum IV antibiotics o Must cover both aerobic and anaerobic organisms o For example: cefotaxime and vancomycin * IV Fluids o For cardiovascular support, may require addition of inotropes * Surgery o Indicated if:  Perforation  Failure to respond to medical treatment o Laparotomy with resection of necrosed bowel with either a primary anastomosis or a defunctioning stoma

Answer 39

necrotising enterocolititis

Answer 40

If pre-feed glucose <2mmol/L or symptomatic (BNFC) o Immediate glucose IV infusion if above this, just encourage feeding.

Answer 41

serum bilirubin threshold graph 1. subthreshold no treatment 2. in between the 2 lines on graph - phototherapy 3. above top line --> exchange transfusion

Answer 42

Respiratory distress syndrome

Answer 43

Management * ABC resuscitation o Review history and examine baby to identify cause of respiratory distress * Respiratory support o Intubation and ventilation  Used when severe RDS o Endotracheal surfactant o Continuous positive airway pressure (CPAP)  Helps keep the lungs inflated o Supplementary oxygen  Aim between 91-95% for preterm neonates * Fluids * IV antibiotics o Broad spectrum combination  Such as benzylpenicillin and gentamicin (unless listeria in which case start amoxicillin and gentamicin) * CXR o Do ASAP unless mild respiratory distress where this can be delayed

Answer 44

Management of symptomatic babies * Refer to paediatric infectious diseases * Pyrimethamine + Sulfadiazine + Folinic acid o Continue all 3 for 1 year * Monitor LFTs and FBCs every 4-6 weeks * + Glucocorticoids (prednisolone) o If CSF protein >1g or active chorioretinitis threatens vision

Answer 45

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. mx --> largely observational

Answer 46

are Group B Streptococcus, Escherichia coli, and Listeria monocytogenes. The antibiotics used to treat early-onset sepsis may vary depending on the trust, but they typically include a penicillin (e.g. benzylpenicillin) and an aminoglycoside (e.g. gentamicin) to cover Gramnegatives

Answer 47

Classically causes a mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border with fixed splitting of the second heart sound

Answer 48

* 98% occur distal to the left subclavian artery, usually at the origin of the ductus arteriosus * Most common presentation is at 48 hours old when the ductus arteriosus closes Management * Prostaglandin E1 infusion o To maintain duct patency * Surgical repair o End-to-end anastomosis or arch reconstruction with patch placement or bypass graft o Older patients may require stent insertion or surgical resection

Answer 49

Maintain duct patency: key to early survival o Start prostaglandin E1 infusion

Answer 50

if cyanotic disease you need to keep it open so give prostaglandin E1 if not then close it up... give them: o IV Indomethacin – 1st line treatment o Prostacyclin synthetase inhibitor o Ibuprofen  Usually done in premature/VLBW infants * If pharmacological methods are unsuccessful, surgical ligation or percutaneous catheter device closure may be used

Answer 51

Rheumatic fever develops following an immunological reaction to a recent (2-4 weeks ago) Streptococcus pyogenes infection. Type 2 hypersensitivity reaction….

Answer 52

Diagnosis is based on evidence of recent streptococcal infection accompanied by: 2 major criteria 1 major with 2 minor criteria Evidence of recent streptococcal infection raised or rising streptococci antibodies, positive throat swab positive rapid group A streptococcal antigen test Major criteria erythema marginatum Sydenham's chorea: this is often a late feature polyarthritis carditis and valvulitis (eg, pancarditis) The latest iteration of the Jones criteria (published in 2015) state that rheumatic carditis cannot be based on pericarditis or myocarditis alone and that there must be evidence of endocarditis (the clinical correlate of which is valvulitis which manifests as a regurgitant murmur) subcutaneous nodules Minor criteria raised ESR or CRP pyrexia arthralgia (not if arthritis a major criteria) prolonged PR interval

Answer 53

Prophylaxis * Benzathine penicillin every 3-4 weeks Management 1. Naproxen 2. Anti-streptococcal antibiotics if persistent infection = e.g. penicillin V, benzathine benzylpenicillin, amoxicillin) 3. Symptomatic heart failure o Treated with diuretics and ACE inhibitors o Prednisolone may be required

Answer 54

1. vagal manouveres 2. adenosine 3. DC cardiovert / amiodarone if haemo unstable --> DC cardiovert

Answer 55

o Place the patient in the knee-to-chest position o Supplementary oxygen o IV fluids o Beta blockers (relax the right ventricle and improve flow to the pulmonary vessels) o Morphine (decrease the respiratory drive) o Sodium bicarbonate (counteracts any metabolic acidosis) o Phenylephrine infusion (increase systemic vascular resistance)

Answer 56

* If severe with worsening cyanosis: o Prostaglandin E1 infusion o Blalock-Taussig shunt surgery performed from 4 months of age onwards

Answer 57

Prostaglandin E1 infusion (alprostadil)

Answer 58

* Blalock-Taussig shunt insertion

Answer 59

loud pansystolic murmur heard best at the lower left sternal edge in the third and fourth intercostal spaces; quiet pulmonary second heart sound

Answer 60

ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur)

Answer 61

Whilst the VSD is present, bacterial endocarditis should be prevented by maintaining good dental hygiene o Prophylactic amoxicillin to patients at high risk of developing endocarditis e.g. during surgical procedures

Answer 62

whilst viral upper respiratory tract infections (URTIs) typically precede otitis media, most infections are secondary to bacteria, particularly Streptococcus pneumonaie, Haemophilus influenzae and Moraxella catarrhalis viral URTIs are thought to disturb the normal nasopharyngeal microbiome, allowing bacteria to infect the middle ear via the Eustachian tube

Answer 63

don't really need abx - normally resolves in 3 days if it doesnt the you can give abx  Amoxicillin - 5-7 days is first-line  Penicillin allergy: clarithromycin, erythromycin

Answer 64

Myringotomy is the most common surgical option for OME. It involves making an incision in the tympanic membrane to drain the fluid, followed by insertion of grommets which stay in place for 6–12 moths to prevent recurrence

Answer 65

haemophilus influenza type B

Answer 66

acute epiglottitis

Answer 67

* Secure airway, do NOT examine the throat o Direct rigid laryngoscopy and intubation is the most common approach * Take a blood culture abx = ceftriaxone

Answer 68

* Patients with rapidly developing angio-oedema without anaphylaxis: o Chlorphenamine and hydrocortisone * Patients with symptoms requiring treatment: o Cetirizine (or other non-sedating antihistamine such as fexofenadine, or loratadine) o Oral corticosteroid (for example prednisolone 40 mg daily for up to 7 days)

Answer 69

< 5 y/o 1. SABA 2. paed mod dose ICS 3. LTRA (montelukast) 4. stop LTRA --> refer to paeds 5-16 y/o 1. SABA 2. paed low does ICS 3. montelukast 4. stop montelukast, start LABA 5. stop ICS , stop LABA. start MART with paed low dose ICS 6. Increase ICS to moderate dose 7. refer to paeds

Answer 70

High resolution CT is the gold standard investigation to diagnose bronchiectasis o Radiological features indicative of bronchiectasis include: o Bronchial wall thickening o Diameter of bronchus larger than that of the bronchial artery (‘signet ring’ sign) o Visible peripheral bronchi

Answer 71

o Pseudomonas spp. Is characteristic of CF bronchiectasis

Answer 72

* 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

Answer 73

1. humidified o2 2. CPAP 3. upper airway suciton 4.fluids

Answer 74

regurgitation and vomiting diarrhoea urticaria, atopic eczema 'colic' symptoms: irritability, crying wheeze, chronic cough rarely angioedema and anaphylaxis may occur

Answer 75

breastfeeding mum: exclude cow's milk from her diet bottlefed: * Trial of extensively hydrolysed formula

Answer 76

* peak incidence at 6 months - 3 years * more common in autumn

Answer 77

* cough * barking, seal-like * worse at night * stridor * remember, the throat should be not examined due to the risk of precipitating airway obstruction * fever * coryzal symptoms * increased work of breathing e.g. retraction

Answer 78

every gets oral dexamethasone moderate croup can give oxyen severe croup can also give nebulsied adrenaline (1 in 1000)

Answer 79

Sweat test patient's with CF have abnormally high sweat chloride normal value < 40 mEq/l, CF indicated by > 60 mEq/l

Answer 80

 F508 delta

Answer 81

o Pulmonary – physiotherapy, mucolytics o Infection – prophylactic antibiotics, monitoring o Nutrition – enzyme tablets, high-calorie diet, monitor growth o Psychosocial – provide support for child and carers

Answer 82

Food challenge o After 6-12 months of being symptoms-free consider a food challenge  This involves administration of increasing quantities of the food allergen under medical supervision, starting with direct mucosal exposure (allergen contact with the lips), and then titrated oral ingestion as tolerated. If no symptoms are provoked, the test is negative and clinical allergy can be excluded

Answer 83

1. ecnourage cooughing 2. back blows 3. abdo thrusts in hosputal 1. flexi bronchoscopy / or rigid bronchoscopy 2. surgery / trachotomy

Answer 84

Laryngomalacia is the most common congenital laryngeal abnormality characterised by flaccidity of the supraglottic structures. The larynx is soft and floppy as a result and collapses during breathing. Features Inspiratory stridor: high-pitched and crowing. This is usually intermittent, occurring in the supine position e.g. when the child lies on its back, when feeding or when agitated Symptoms increase in severity during the first 8 months but tend to resolve by 18-24 months Respiratory distress, failure to thrive and cyanosis are rare

Answer 85

Group A haemolytic streptococci (usually Streptococcus pyogenes Pencillin V (phenoxymethylpenicllin)

Answer 86

* 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

Answer 87

FeverPAIN score (4 or 5) or Centor score (3 or 4)

Answer 88

sore throat is tonsilittis / pharingitis normally from GAS inection therefore treat with Penicilin V

Answer 89

* Urticaria Activity Score (UAS7) cetirizine (anti histamine)

Answer 90

wheeze salbutamol  Burst Therapy is often used for viral-induced wheeze

Answer 91

catarrhal phase * symptoms are similar to a viral upper respiratory tract infection * lasts around 1-2 weeks paroxysmal phase * the cough increases in severity * coughing bouts are 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 * lasts between 2-8 weeks * convalescent phase * the cough subsides over weeks to months

Answer 92

bordetella pertussis (gram neg coccobacillli) * Pharmacological Treatment - if admission is not needed, prescribe an antibiotic if the onset of the cough is within 21 days. o < 1 month old = clarithromycin o >1 months old and not pregnant = azithromycin o If macrolides contraindicated, give co-trimoxazole  Do not use in infants <6 weeks of age

Answer 93

Appendicitis is one of the most common acute surgical problems facing children. Diagnosis is often made difficult by a presentation which is far from the classically history of: central abdominal pain which later radiates to the right iliac fossa low-grade pyrexia minimal vomiting Children who are younger or have a retrocaecal/pelvic appendix are more likely to present in an atypical way Appendicitis is uncommon in children under 4 years old but in this group often presents with perforation

Answer 94

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

Answer 95

o Bulk-forming: fybogel, methylcellulose o Osmotic: lactulose, Movicol o Stimulant: Bisacodyl, senna, sodium picosulphate o Stool-softener: arachis oil, docusate sodium

Answer 96

Red Flag for Constipation - Ribbon stool - >24 hrs meconium at birth - Distension - any blood Amber Flag for Constipation - Faltering growth Ribbon stool pattern — may indicate anal stenosis (more likely to present in a child younger than 1 year of age)

Answer 97

Meconium should be passed ideally within the first 24 hours and less frequently within the first 48 hours

Answer 98

if impaction: 1. movicol (osmotic lax), can increase dose over 2 weeks, also diet advice. (movicol are satchet packets) 2. if unresponsive can give senna (stimulative laxative) 3. if stools are hard can also use lactulose/docusate if no impaction: 1. movicol 2. if movicol not tolerated can give senna (stimulative laxative) 3. if stools are hard can also use lactulose/docusate

Answer 99

The shift in guidance to start anti-TNF agents such as infliximab as a first-line treatment (‘top-down therapy’) as opposed to the previous ‘step-up’ model was informed by studies such as the TISKIDS and REACH trials where this approach was associated with much better outcomes for children

Answer 100

1. Inadequate nutritional intake. e,g, neglect, poverty, iron def anaemia, maternal malnurtiion if breastfeeding 2. Difficulty feeding - poor breastfeeding/suck, cleft lip, cleft palate, pyloric stenosis, 3. Malabsorption - crohns, coeliac, CF, perniciois anaemia 4. Increased energy requirements - CF, hyperthyroid, malignancy. HIV 5. Inability to process nutrition - type 1 diabetes, inborn errors of metabolism

Answer 101

* Breastfeeding: offer assessment → alginate therapy * Formula: review feeding history → smaller, more frequent feeds → thickeners → alginate therapy

Answer 102

Hirschsprung's disease is caused by an aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses. parasympathetic neuroblasts fail to migrate from the neural crest to the distal colon → developmental failure of the parasympathetic Auerbach and Meissner plexuses → uncoordinated peristalsis → functional obstruction

Answer 103

Possible presentations neonatal period e.g. failure or delay to pass meconium older children: constipation, abdominal distensionh

Answer 104

rectal biopsy

Answer 105

bowel irrigation surgery = anorectal pull through

Answer 106

* intermittent, severe, crampy, progressive abdominal pain * inconsolable crying * 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

Answer 107

abdo ultrasound target like mass

Answer 108

1. A-E 2. NG aspitation 3. IV fluids 4.  Clindamycin + gentamicin 5. rectal air insufflation surgery = if peritonitis / big problem

Answer 109

* If recurrent intussusception - consider investigating for a pathological lead point (e.g. Meckel's diverticulum)

Answer 110

meckel's diverticulum 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

Answer 111

Investigation * if the child is haemodynamically stable with less severe or intermittent bleeding then a 'Meckel's scan' should be considered * uses 99m technetium pertechnetate, which has an affinity for gastric mucosa * mesenteric arteriography may also be used in more severe cases e.g. transfusion is required

Answer 112

Ladd procedure - detorting the bowel and surgically dividing the Ladd bands

Answer 113

constipation abdominal bloating abdominal pain nausea/vomiting

Answer 114

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 pain can take 2-4 weeks to resolve

Answer 115

epigastric pain nausea duodenal ulcers more common than gastric ulcers epigastric pain when hungry, relieved by eating gastric ulcers epigastric pain worsened by eating

Answer 116

Pyloric stenosis typically presents in the second to fourth weeks of life with vomiting, although rarely may present later at up to four months. It is caused by hypertrophy of the circular muscles of the pylorus. NON BILOUS VOMITING * 'projectile' vomiting, typically 30 minutes after a feed * constipation and dehydration may also be present * a palpable mass may be present in the upper abdomen * hypochloraemic, hypokalaemic alkalosis due to persistent vomiting

Answer 117

pyloric stenosis or intussusecption

Answer 118

* Definitive treatment is by performing a Ramstedt pyloromyotomy

Answer 119

* Proctitis o 1st line - Topical aminosalicylate for 4 weeks, * Mild UC: o 5-ASA * Moderate UC: o Oral prednisolone for 2-4wks and taper * Severe UC: o Medical emergency o High dose IV Methylprednisolone o Surgical Treatment

Answer 120

ursodeoxycholic acid

Answer 121

Patients typically present in the first few weeks of life with: - Jaundice extending beyond the physiological two weeks - Dark urine and pale stools - Appetite and growth disturbance, however, may be normal in some cases Signs: Jaundice Hepatomegaly with splenomegaly Abnormal growth Cardiac murmurs if associated cardiac abnormalities present The perinatal form presents in the first two weeks of life, and the postnatal form presents within the first 2-8 weeks of life Biliary atresia occurs in 1 in every 10,000-15,000 live births

Answer 122

Ultrasound of the biliary tree and liver: May show distension and tract abnormalities Percutaneous liver biopsy with intraoperative cholangioscopy

Answer 123

paracetamol

Answer 124

* Nazar score or Kings Wilson score used to assess severity

Answer 125

Wilson's disease is an autosomal recessive disorder characterised by excessive copper deposition in the tissues. Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion. Wilson's disease is caused by a defect in the ATP7B gene located on chromosome 13. The onset of symptoms is usually between 10 - 25 years. Children usually present with liver disease whereas the first sign of disease in young adults is often neurological disease Features result from excessive copper deposition in the tissues, especially the brain, liver and cornea: - liver: hepatitis, cirrhosis neurological: - basal ganglia degeneration: in the brain, most copper is deposited in the basal ganglia, particularly in the putamen and globus pallidus speech, behavioural and psychiatric problems are often the first manifestations - also: asterixis, chorea, dementia, parkinsonism - Kayser-Fleischer rings green-brown rings in the periphery of the iris due to copper accumulation in Descemet membrane present in around 50% of patients with isolated hepatic Wilson's disease and 90% who have neurological involvement renal tubular acidosis (esp. Fanconi syndrome) haemolysis blue nails Investigations slit lamp examination for Kayser-Fleischer rings reduced serum caeruloplasmin reduced total serum copper (counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin) free (non-ceruloplasmin-bound) serum copper is increased increased 24hr urinary copper excretion the diagnosis is confirmed by genetic analysis of the ATP7B gene mx = penicillamine (chelates copper) has been the traditional first-line treatment

Answer 126

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

Answer 127

< 3 months = amoxicillin/ampicillin + cefotaxime > 3 months = ceftriaxone dexamethsone (don't give if < 3months) * Presenting in primary care: single dose of IM/IV benzylpenicillin all children should be followed up 4/6 weeks for hearing assessment

Answer 128

1. fever 2. drowsy, floppy, unresponsive 3. refusing food 4. vomiting 5. tense bulging fontanelle 6. unusual cry 7. rapid breathing 8. seizures

Answer 129

1. fever initially 2. itchy, rash starting on head/trunk before spreading. 3. Initially macular then papular then vesicular systemic upset is usually mild

Answer 130

mainly supportive treatment - fluids, smooth cotton frabirc, nails short - paracetamol, topical calamine lotion for itch - infective until lesions have crusted over so until this point no school, no pregnant ladies, no immunocompromised - admit to hospital if serious complications

Answer 131

Dengue fever fever headache (often retro-orbital) myalgia, bone pain and arthralgia ('break-bone fever') pleuritic pain facial flushing (dengue) maculopapular rash haemorrhagic manifestations e.g. positive tourniquet test, petechiae, purpura/ecchymosis, epistaxis 'warning signs' include: abdominal pain hepatomegaly persistent vomiting clinical fluid accumulation (ascites, pleural effusion) Severe dengue (dengue haemorrhagic fever) this is a form of disseminated intravascular coagulation (DIC) resulting in: thrombocytopenia spontaneous bleeding around 20-30% of these patients go on to develop dengue shock syndrome (DSS)

Answer 132

The classic triad of sore throat, pyrexia and lymphadenopathy

Answer 133

* Explain the expected course of the illness o Symptoms usually last for 2–4 weeks. o Tiredness is common o Exclusion from work or school is not necessary but tailor activities to what they find comfortable o Limit spread by avoiding kissing and sharing of eating utensils o Avoid heavy lifting and contact or collision sports for the first month of the illness - due to risk of splenic rupture supportive management (rest, paracemolm . iburpofen)

Answer 134

Features * high fever: lasting a few days, followed later by a * maculopapular rash * Nagayama spots: papular enanthem on the uvula and soft palate * febrile convulsions occur in around 10-15% * diarrhoea and cough are also commonly seen supportive mx (paraceotmol & ibrufpofen) risk of febrile seizures

Answer 135

The illness may consist of a mild feverish illness which is hardly noticeable. - noticeable rash which appears after a few days. - The rose-red rash makes the cheeks appear bright red, hence the name 'slapped cheek syndrome'. - The rash may spread to the rest of the body but unlike many other rashes, it only rarely involves the palms and soles. The child begins to feel better as the rash appears and the rash usually peaks after a week and then fades. Most children recover and need no specific treatment. School exclusion is unnecessary as the child is not infectious once the rash emerges. In adults, the virus may cause acute arthritis.

Answer 136

caused by either Staphylcoccus aureus or Streptococcus pyogenes. It can be a primary infection or a complication of an existing skin condition such as eczema (in this case), scabies or insect bites. Features 'golden', crusted skin lesions typically found around the mouth very contagious tx = topical fusidic acid

Answer 137

human parvovirus b19

Answer 138

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 mx = high dose aspirin / IVIG

Answer 139

* Stay away from school for at least 4 days after the development of the rash

Answer 140

prodromal phase irritable conjunctivitis fever Koplik spots typically develop before the rash white spots ('grain of salt') on the buccal mucosa rash starts behind ears then to the whole body discrete maculopapular rash becoming blotchy & confluent desquamation that typically spares the palms and soles may occur after a week diarrhoea occurs in around 10% of patients mx = supportive , stay away from sschool at least 4 days after developement of rash

Answer 141

* Stay away from school for 5 days after the development of parotitis

Answer 142

Clinical features fever malaise, muscular pain parotitis ('earache', 'pain on eating'): unilateral initially then becomes bilateral in 70%

Answer 143

Features prodrome, e.g. low-grade fever rash: maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day lymphadenopathy: suboccipital and postauricular

Answer 144

* Supportive care: IV fluids and antipyretics * Suspected: Ceftriaxone and azithromycin * Known: o Ciprofloxacin 7d treatment o If no response after 4/5d of treatment add azithromycin o If encephalopathic, add high-dose dexamethasone

Answer 145

* Stay away from school for at least 5 days after initial development of rash

Answer 146

Eczema herpeticum describes a severe primary infection of the skin by herpes simplex virus 1 or 2. It is more commonly seen in children with atopic eczema and often presents as a rapidly progressing painful rash. admit and tx = acyclovir

Answer 147

1. emolient 2. topical corticosteroids - hydrocortisone 3. Topical Calcineurin Inhibitors wet wraps / bandages non-sedating anti-histamine (cetirizine) tacrolimus infected eczema = flucoxacillin

Answer 148

Guttate psoriasis is more common in children and adolescents. It may be precipitated by a streptococcal infection 2-4 weeks prior to the lesions appearing. Features tear drop papules on the trunk and limbs gutta is Latin for drop pink, scaly patches or plques of psoriasis tends to be acute onset over days mx = phototherapy

Answer 149

Haemangioma Most haemangiomas will have disappeared completely by the age of five to seven years. * If there is functional impairment (near eyes, nose, mouth) or cosmetic disfigurement: o Beta-blocker: PO (propranolol) or topical (timolol) o Corticosteroid: PO or topical o Until theoretical involution or 12m o If cosmetically-sensitive or high-risk/ulceration, can additionally consider:  Pulsed laser dye  Surgical excision

Answer 150

Clinical features * mild systemic upset: sore throat, fever * oral ulcers * followed later by vesicles on the palms and soles of the feet 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 self limiting / symptomatic tx

Answer 151

* ‘Cradle-cap’ * Erythematous scaly eruption in first 2 months of life * Progresses to thick yellow adherent layer * Starts on scalp, spreads to the face, flexures and napkin area * Not itchy * Management – o Infants: baby shampoo/oil, barrier emollients, topical antifungal creams (clotrimazole, miconazole) o Children/adolescents: ketoconazole shampoo/creams, selenium sulphide shampoo, coal tar, salicylic acid ointment

Answer 152

* Itchy rash typically in face, trunk and flexor surfaces (age-dependent) * Common in first year of life * Associated with asthma and hay fever * Scratching causes excoriation, crusting, infection and eventually lichenification * Management – avoid precipitants, emollients, topical corticosteroids, occlusive bandages, antihistamine, antibiotics, immunomodulators

Answer 153

o Disposable nappies are better than towel nappies o Expose nappy area to air where possible o Apply barrier cream (e.g. Sudocrem) o Mild steroid cream (e.g. 1% hydrocortisone) in severe cases o Manage suspected candida nappy rash with topical imidazole (cease the use of barrier cream until candida has settled)

Answer 154

* Both causes and complicates napkin rashes * Spares flexures * Satellite pustules * Management – ensure good hygiene, topical antifungals

Answer 155

* Caused by a poxvirus * Pearly papules with central umbilication through which the infectious central core is shed * Usually disappear spontaneously * Management (if needed) – cryotherapy, topical antibacterials to prevent/treat secondary bacterial infection

Answer 156

* Caused by Sarcoptes scabiei * Severe itching worse at night and in warmth * Ask about family’s living situation * Between fingers and toes, axillae, palms, soles and trunk in younger children Treatment – treat child and whole family, 5% permethrin cream or 0.5% malathion lotion to whole body below neck, washed off after 12 hours

Answer 157

* Annular lesions with a crusted edge caused by dermatophyte fungi * Kerion – severe inflammatory pustular ringworm * Treatment – topical antifungals, systemic antifungal for severe infection, treat source (often animals)

Answer 158

* Target lesions, can also be vesicular or bullous * Caused by herpes simplex, Mycoplasma pneumonia and other infections and drugs * Usually resolves spontaneously within a few weeks

Answer 159

* Most commonly guttate psoriasis following strep/viral URTI * Small erythematous patches with silvery scale on trunk and upper limbs * Rare before 2 years, rarely chronic * Management – emollients, coal tar, dithranol (for resistant cases), calcipotriol (over 6 years of age) * Can be associated with arthritis

Answer 160

* Can occur 1 – 2 years before onset of puberty * Due to obstruction of sebum flow in sebaceous follicle * Open/closed comedones, papules, pustules, nodules, cysts on face, neck, back, shoulders * Can produce scarring * Treatment – topical – benzoyl peroxide, antibiotics, retinoids. Systemic – oral antibiotics (tetracyclines only licensed if over 12 years old), isotretinoin

Answer 161

* Tender discrete red nodules on the shins * Caused by Strep, primary TB, IBD, drugs * Treatment – treat underlying cause, compression, anti-inflammatories

Answer 162

* Caused by Neisseria meningitides septicaemia * Characteristic non-blanching purpuric lesions irregular in size and outline with a necrotic centre * Called ‘purpura fulminans’ if widespread * Often non-specific initial presentation – fever, vomiting, poor feeding, irritability, lethargy, seizures, reduced consciousness, shock; later bulging fontanelle, neck stiffness and opisthotonus * Typically headache, neck stiffness and photophobia * Diagnosis – LP (turbid fluid, polmorphs, high protein, low glucose) * Management – rapid initiation of antibiotics – 3rd generation cephalosporin e.g. ceftriaxone)

Answer 163

* Common infection caused by parvovirus B19 * Often asymptomatic * Rash – initially ‘slapped cheek’, progresses to maculopapular lace-like rash on trunk and limbs * Rash associated with fever, malaise, headache and myalgia * Infects erythroblastoid red cell precursors in BM, so causes aplastic crisis in sickle cell (↑ RBC turnover), also in immunocompromised * Maternal transmission can cause hydrops and foetal death due to severe anaemia * Paracetamol or ibuprofen and encourage adequate fluid intake * Secondary arthritis may be treated with ibuprofen

Answer 164

1. wet combing 2. dimeticone lotion (insecticide) 3.

Answer 165

cefotaxime or clindamycin)

Answer 166

Tinea Corporis (body), Tinea Cruris (jock) o Mild infections - terbinafine cream, clotrimazole o If marked inflammation- hydrocortisone 1% cream o severe infections:  1st line: oral terbinafine  2nd line: oral itraconazole o Topical aluminium acetate (in some) * Tinea Capitis (head) o Systemic antifungal therapy (e.g. griseofulvin or terbinafine) o 2nd line: itraconazole or fluconazole o Adding topical antifungal shampoo is recommended in some patients (e.g. selenium sulfide or ketoconazole topical) * NOTE: any animal source of the infection will also need treatment * Advice o Wear loose-fitting cotton clothing o Wash affected areas of skin daily o Dry thoroughly after washing o Avoid scratching o Do not share towels o Wash clothes and bed linen frequently o No need for school exclusion

Answer 167

permethrin 5% cream apply to whole body

Answer 168

* Most children are dry by day and night by the age of 5 years o Children are dry by day only by the age of 4 years

Answer 169

o BED  Behavioral - Advise onreduced fluid intake before bed, diet and toileting behaviour & Reward systems (e.g. star charts)  Enuresis alarm  Desmopressin: may be used 1st line if > 7 years or if short-term control is needed (e.g. holiday)

Answer 170

IgA vascultitis HSP is an immune-mediated small vessel vasculitis. IgA and complement components (C3) are deposited in vessel walls leading to inflammation. The mechanism underlying this is not entirely clear, but HSP can follow an upper respiratory tract infection, so it is thought to be triggered by an abnormal immune system response. Features palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs abdominal pain polyarthritis features of IgA nephropathy may occur e.g. haematuria, renal failure

Answer 171

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

Answer 172

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

Answer 173

o Sexually transmitted infection * Age <35 yrs * 1+ sexual partner in last 12 months * Urethral discharge o Gonorrhoeal * Previous gonorrhoeal infection * Purulent urethral discharge * Men who have sex with men * Black ethnicity o Enteric organisms associated with UTIs * Age >35 yrs * Low risk sexual history * History of penetrative anal sex * Recent catheterisation o Consider testing for HIV, hepatitis, syphilis, trichomoniasis and mumps

Answer 174

* < 2 years (congenital hydrocoele) = most resolve spontaneously before the age of 2 so observation is appropriate unless there is bowel palpable in the groin and provided there is no evidence of underlying pathology * 2-11 years - hydrocele persists beyond 2 years o Open repair o Laparoscopic exploration o Bilateral repair o Abdominoscrotal hydrocoeles - require surgery through an abdominal incision * 11-18 years - commonly non-communicating hydrocoele o Idiopathic hydrocoele - observation is appropriate, however, surgery may be considered if it is large or uncomfortable o Hydrocoele after varicocelectomy - conservative management is the initial approach, surgery is considered in cases that do not resolve o Filarial-related hydrocoele (parasitic infection) - complete excision of the tunica vaginalis

Answer 175

pain is usually severe and of sudden onset the pain may be referred to the lower abdomen nausea and vomiting may be present on examination, there is usually a swollen, tender testis retracted upwards. The skin may be reddened cremasteric reflex is lost elevation of the testis does not ease the pain (Prehn's sign) management: * Presenting within 4-6 hours of symptom-onset has a greater likelihood of testicular viability Management * If suspected admit immediately to urology or paediatric surgery o IV fluids, NBM, antiemetics, analgesia

Answer 176

Surgery (nephrectomy) and chemotherapy (may be post-operative or pre-operative)

Answer 177

regular blood transfusions

Answer 178

o Bruises are on a child who is not yet independently mobile (crawling, cruising, or walking). o Bruises have indicative features - unusually large, multiple sites or in clusters, similar shape and size, patterned in the shape of a hand print, ligature, stick, tooth, grip, or implement (such as a belt). o Bruises are found in indicative places - any non-bony part of the body or face (including the eyes, ears, cheeks, back, abdomen, buttocks, arms, and genitalia). o Explanation for the bruising is implausible, inadequate, or inconsistent o Delay in presentation. o If suspicions of NAI, screen for other injuries and perform skeletal survey to investigate. Inform safeguarding team.

Answer 179

* Immunisation against encapsulated organisms (e.g. S. pneumoniae and H. influenzae type B) * Daily oral penicillin * Daily oral folic acid * Vaso-occlusive crises should be minimised by avoiding exposure to cold, dehydration, excessive exercise, undue stress or hypoxia

Answer 180

platelet= Site Skin, mucus membranes (epistaxis, gum, vaginal, GI) coagulation = Soft tissues, joints, muscles

Answer 181

1. large glycoprotein which forms massive multimers up to 1,000,000 Da in size 2. promotes platelet adhesion to damaged endothelium 3. carrier molecule for factor VIII Types type 1: partial reduction in vWF (80% of patients) type 2*: abnormal form of vWF type 3**: total lack of vWF (autosomal recessive) Investigation prolonged bleeding time APTT may be prolonged (it stablises factor 8) factor VIII levels may be moderately reduced defective platelet aggregation with ristocetin

Answer 182

Management of Acute Crises * Oral and IV analgesia (avoid morphine < 12 years) * Good hydration (oral or IV) * Infection should be treated with antibiotics * Oxygen (if reduced saturation) * Exchange transfusion is indicated for acute chest syndrome, priapism and stroke Management of Chronic Problems * Children who have recurrent hospital admission (>3 in 12 months) for acute chest syndrome or vaso-occlusive crises could benefit from hydroxycarbamide (stimulated HbF production) o Monitor for white blood cell suppression (side-effect of hydroxycarbamide) * Splenectomy with immunisation against encapsulated organisms * Bone marrow transplant may be considered in severe cases

Answer 183

Types type 1: partial reduction in vWF (80% of patients) type 2*: abnormal form of vWF type 3**: total lack of vWF (autosomal recessive)

Answer 184

paraphimosis

Answer 185

* Average age of diagnosis: 18 months * In propriety, goals of treatment: Save life, save eye, save vision * Frequent eye examinations under anaesthesia to assess the response to treatment Management * Gross vitreous seeding present (tumor cells floating within the vitreous cavity) o 1st LINE: Enucleation (surgical removal of the eye without resection of the lids or extraocular muscles o Infiltration of the iris, ciliary body or sclera: Adjuvant chemotherapy: carboplatin, etoposide, and vincristine * Minimal or no vitreous seeding present o 1st LINE: systemic chemotherapy (carboplatin, vincristine, etoposide) o PLUS: focal therapy (cryotherapy or laser therapy) * Family history of retinoblastoma/detected at birth o Usually treated by laser alone o Followed up with an examination under anaesthesia every month for at least 1 year * Vitreous seeding after chemotherapy and/or focal therapy o 1st LINE: external beam radiotherapy * Most patients are cured (90%) * However, many will be visually impaired * Significant risk of second malignancy (especially sarcoma) among survivors of hereditary retinoblastoma

Answer 186

* ↓ platelets, ↓ fibrinogen, ↑ PT & APTT, ↑ fibrinogen degradation products

Answer 187

* Parents should be advised on the signs of acute haemolysis (jaundice, pallor, dark urine)

Answer 188

* In 80% of children, the disease is acute, benign and self-limiting * It will resolve spontaneously within 6-8 weeks * Most children can be managed at home * Treatment is indicated if there is evidence of major bleeding (e.g. intracranial or gastrointestinal) or persistent minor bleeding that affects daily life (e.g. excessive epistaxis) Management * Life- or Organ-threatening bleeding o IVIG + corticosteroid + platelet transfusion o Antifibrinolytics (Aminocaproic and tranexamic acid) may be used * Newly Diagnosed Child o Asymptomatic or Minor Bleeding  Observation (most will achieve a normal platelet count eventually)  Most manifestations are limited to the skin o Major Bleeding  Corticosteroids  IVIG OR anti-D immunoglobulin * Child with Chronic Disease o Mycophenolate mofetil o Rituximab o Eltrombopag (thrombopoietin receptor agonist) o 2nd line: splenectomy (if persistent)

Answer 189

* Recombinant factor VIII concentrate for haemophilia A o 10-15% of patients will develop antibodies to factor VIII treatment * Recombinant factor IX concentrate for haemophilia B Management * Acute bleeds are treated with factor concentrates and anti-fibrinolytics (e.g. aminocaproic acid, tranexamic acid) * NOTE: these should be given by prompt IV infusion whenever there is any bleeding * Analgesia and physiotherapy may be required for deep bleeds into muscles and joints * Orthopaedic and pain team review may also be necessary * In patients with haemophilia, the following should be AVOIDED: o IM injections o Aspirin o NSAIDs

Answer 190

Prophylaxis * Immunisation against encapsulated organisms (e.g. S. pneumoniae and H. influenzae type B) * Daily oral penicillin * Daily oral folic acid * Vaso-occlusive crises should be minimised by avoiding exposure to cold, dehydration, excessive exercise, undue stress or hypoxia Management of Acute Crises * Oral and IV analgesia (avoid morphine < 12 years) * Good hydration (oral or IV) * Infection should be treated with antibiotics * Oxygen (if reduced saturation) * Exchange transfusion is indicated for acute chest syndrome, priapism and stroke Management of Chronic Problems * Children who have recurrent hospital admission (>3 in 12 months) for acute chest syndrome or vaso-occlusive crises could benefit from hydroxycarbamide (stimulated HbF production) o Monitor for white blood cell suppression (side-effect of hydroxycarbamide) * Splenectomy with immunisation against encapsulated organisms * Bone marrow transplant may be considered in severe cases Prognosis * Can cause premature death due to complications * 50% of patients with the most severe form of sickle cell disease will die < 40 years

Answer 191

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

Answer 192

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 Imaging ultrasound is generally used to confirm the diagnosis if clinically suspected however, if the infant is > 4.5 months then x-ray is the first line investigation

Answer 193

Screening for DDH 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 (they need an ultrasound of the pelvis at 6 weeks) * 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 194

* Summary o Most unstable hips will resolve spontaneously by 3-6 weeks o Pavlik harness in children younger than 4-5 months o Older children may require surgery o Female sex at greater risk

Answer 195

Features of systemic onset JIA include * pyrexia * salmon-pink rash * lymphadenopathy * arthritis * uveitis * anorexia and weight loss REMEMBER YOU CAN GET A FEVER!! Investigations ANA may be positive, especially in oligoarticular JIA rheumatoid factor is usually negative Juvenile idiopathic arthritis (JIA) refers to persistent joint swelling lasting longer than 6 weeks in patients under the age of 16 years.

Answer 196

Management * Pharmacological management: o Simple analgesia e.g. Paracetamol o NSAIDs are useful for controlling pain and stiffness o Consider weak opioids e.g. codeine * Intra-articular, oral or IV corticosteroids are useful adjunctive agents (whilst waiting for second-line agents to have an effect) – avoided if possible due to risk of growth suppression and osteoporosis * DMARDs - used when the disease fails to respond to conventional treatments o 1st line: oral or SC methotrexate o 2nd line: sulfasalazine * Other treatments: Inflammatory cytokine blockade e.g. TNF-alpha inhibitors, interleukin receptor antagonists, anti-emetics

Answer 197

Osgood-Schlatter disease (tibial apophysitis) is a type of osteochondrosis characterised by inflammation at the tibial tuberosity. It is a traction apophysitis thought to be caused by repeated avulsion of the apophysis into which the patellar tendon is inserted Management is supportive * Seen in sporty teenagers (‘osGOOD at sports) Management * Advise about pain relief o Analgesia - paracetamol or NSAIDs o Intermittent application of ice packs over the tibial tuberosity (10-15 mins up to 3 times per day, including after exercise) o Protective knee pads (may relieve pain when kneeling) * Reassure the patient and parents that this will resolve over time but may persist until the end of a growth spurt * Reassure that usually stopping all sporting activity is not necessary o Reduce sporting activity (intensity, frequency or duration) o Change the type of exercise to limit the amount of running and jumping requiring powerful quadriceps contraction if they cannot tolerate normal activity o As symptoms decrease, they can gradually increase their exercise levels o Introduce low-impact quadriceps exercises (e.g. isometric quadriceps contractions, straight leg raises, cycling or swimming) * Reassure that usually stopping all sporting activity is not necessary * If symptoms do not improve or worsen OR symptoms persist into adulthood despite the above management, refer for specialist assessment by orthopaedic surgeon. o ADVICE  Victorian Paediatric Orthopaedic Network fact sheet on Osgood-Schlatter disease has an explanation of the condition as well as instruction on some useful stretches  Proper stretching before and after exercise may reduce symptoms

Answer 198

Osteochondritis Dissecans Osteochondritis dissecans (OCD) is a pathological process affecting the subchondral bone (most often in the knee joint) with secondary effects on the joint cartilage, including pain, oedema, free bodies and mechanical dysfunctions. It generally affects children and young adults. OCD may progress to degenerative changes if untreated. Patients typically present with a subacute onset of: Knee pain and swelling, typically after exercise Knee catching, locking and/or giving way: more constant and severe symptoms are associated with the presence of loose bodies Feeling a painful 'clunk' when flexing or extending the knee - indicating the involvement of the lateral femoral condyle Signs: Joint effusion Tenderness on palpation of the articular cartilage of the medial femoral condyle, when the knee is flexed Wilson's sign for detecting medial condyle lesion - with the knee at 90° flexion and tibia internally rotated, the gradual extension of the joint leads to pain at about 30°, external rotation of the tibia at this point relieves the pain Investigations: X-ray (anteroposterior, lateral and tunnel views) - may show the subchondral crescent sign or loose bodies MRI - used to evaluate cartilage, visualise loose bodies, stage and assess the stability of the lesion Management Early diagnosis is important Clinical signs may be subtle in the early stages hence there should be a low threshold for imaging and/or orthopaedic opinion. * Pain relief (paracetamol or ibuprofen) * Rest and quadriceps exercises * Sometimes surgical intervention is needed (to remove intra-articular loose bodies)

Answer 199

Chondromalacia patellae Chondromalacia patellae is defined as anterior knee pain caused by degeneration of the articular cartilage on the posterior surface of the patella. It is particularly common in young adults as a result of overuse in physical activities. The pain may be exacerbated by running, climbing stairs, and getting up from a chair. Passive movements are usually painless, but repeated extension may produce pain and a grating feeling, and crepitus or a small effusion may be palpable. Patients should receive physiotherapy to strengthen the quadriceps.

Answer 200

Reactive Arthritis can't see, can't pee, can't climb a tree

Answer 201

Symptoms joint pain limp fever systemically unwell: lethargy Signs swollen, red joint typically, only minimal movement of the affected joint is possible Investigations joint aspiration: for culture. Will show a raised WBC raised inflammatory markers blood cultures The Kocher criteria for the diagnosis of septic arthritis: fever >38.5 degrees C non-weight bearing raised ESR raised WCC * Prolonged course of antibiotics (initially IV for 2 weeks, followed by 4 weeks of oral antibiotics) o Neonate to <3 months:  IV cefotaxime o 3 months to /=6 years  IV flucloxacillin  If penicillin allergic, give clindamycin o Oral stepdown:  Co-amoxiclav  Flucloxacillin * Joint aspiration - affected joints should be aspirated to dryness as often as required (through closed needle aspiration or arthroscopically) * Washing out of the joint or surgical drainage may be required

Answer 202

Slipped upper femoral epiphysis Typically seen in obese male adolescents. Pain is often referred to the knee. Limitation to internal rotation is usually seen. Knee pain is usually present 2 months prior to hip slipping. Bilateral in 20%. X-rays will show the femoral head displaced and falling inferolaterally (like a melting ice cream cone) The Southwick angle gives indication of disease severity * Ensure patient remains non-weight-bearing, analgesia, immediate orthopaedic referral * Surgical repair * In situ screw fixation across the growth plate Viva Tips  What are the X-ray findings? o Trethowan’s sign: line of Klein does not intersect superior femoral epiphyses/asymmetry between line of Klein’s on either side

Answer 203

Rickets is a term that describes inadequately mineralised bone in developing and growing bones. This results in soft and easily deformed bones. It is usually due to vitamin D deficiency. In adults, the equivalent condition is termed osteomalacia Predisposing factors dietary deficiency of calcium, for example in developing countries prolonged breastfeeding unsupplemented cow's milk formula lack of sunlight Features aching bones and joints lower limb abnormalities: in toddlers genu varum (bow legs) in older children - genu valgum (knock knees) 'rickety rosary' - swelling at the costochondral junction kyphoscoliosis craniotabes - soft skull bones in early life Harrison's sulcus Investigations low vitamin D levels reduced serum calcium - symptoms may results from hypocalcaemia raised alkaline phosphatase mx= * If calcium deficient rickets with vitamin D deficiency o Daily calcium AND o Ergocalciferol (vit D2) / colecalciferol (vit D3) * If pseudo-vitamin D deficiency (defect in 1-alpha hydroxylase) o Calcitriol / alfacalcidol * Phosphate salts are used in hypophosphataemic rickets * Dietary: oily fish, egg yolk

Answer 204

Transient synovitis is sometimes referred to as irritable hip. It generally presents as acute hip pain following a recent viral infection. It is the commonest cause of hip pain in children. The typical age group is 3-8 years. Features * limp/refusal to weight bear * groin or hip pain * a low-grade fever is present in a minority of patients * high fever should raise the suspicion of other causes such as septic arthritis

Answer 205

diagnosis of attention deficit hyperactivity disorder (ADHD) which requires the presence of symptoms for at least 6 months in at least two different environments. If not hitting criteria then watchful waiting is important

Answer 206

Classification spastic (70%) subtypes include hemiplegia, diplegia or quadriplegia increased tone resulting from damage to upper motor neurons dyskinetic caused by damage to the basal ganglia and the substantia nigra athetoid movements and oro-motor problems ataxic caused by damage to the cerebellum with typical cerebellar signs mixed

Answer 207

* Risk Factors o Antenatal: Chorioamnionitis, maternal respiratory or GU infection o Perinatal: Preterm birth, LBW, neonatal encephalopathy, neonatal sepsis, maternal infection (e.g. respiratory or genito-urinary) o Postnatal: Meningitis, head trauma prior to 3 years

Answer 208

(correct for gestational age)  Not sitting by 8 months  Not walking by 18 months  Hand preference before 1 year o Refer all children with persistent toe walking

Answer 209

* Summary o Physiotherapy – encourage movement, improve strength and stop muscles from losing range of motion o Speech therapy – improve language abilities o Occupation therapy – identify everyday tasks that may be difficult and help make these tasks more accessible o Medication  Stiffness – baclofen, diazepam  Sleeping – melatonin  Constipation – laxatives  Drooling – anticholinergic

Answer 210

3 months Little or no head lag on being pulled to sit Lying on abdomen, good head control Held sitting, lumbar curve 6 months Lying on abdomen, arms extended Lying on back, lifts and grasps feet Pulls self to sitting Held sitting, back straight Rolls front to back 7-8 months Sits without support (Refer at 12 months) 9 months Pulls to standing Crawls 12 months Cruises Walks with one hand held 13-15 months Walks unsupported (Refer at 18 months) 18 months Squats to pick up a toy 2 years Runs Walks upstairs and downstairs holding on to rail 3 years Rides a tricycle using pedals Walks up stairs without holding on to rail 4 years Hops on one leg

Answer 211

Age Milestone 3 months Quietens to parents voice Turns towards sound Squeals 6 months Double syllables 'adah', 'erleh' 9 months Says 'mama' and 'dada' Understands 'no' 12 months Knows and responds to own name 12-15 months Knows about 2-6 words (Refer at 18 months) Understands simple commands - 'give it to mummy' 2 years Combine two words Points to parts of the body 2½ years Vocabulary of 200 words 3 years Talks in short sentences (e.g. 3-5 words) Asks 'what' and 'who' questions Identifies colours Counts to 10 (little appreciation of numbers though) 4 years Asks 'why', 'when' and 'how' questions

Answer 212

The tables below summarises the major fine motor and vision developmental milestones Age Milestone 3 months Reaches for object Holds rattle briefly if given to hand Visually alert, particularly human faces Fixes and follows to 180 degrees 6 months Holds in palmar grasp Pass objects from one hand to another Visually insatiable, looking around in every direction 9 months Points with finger Early pincer 12 months Good pincer grip Bangs toys together Bricks Age Milestone 15 months Tower of 2 18 months Tower of 3 2 years Tower of 6 3 years Tower of 9 Drawing Age Milestone 18 months Circular scribble 2 years Copies vertical line 3 years Copies circle 4 years Copies cross 5 years Copies square and triangle Book Age Milestone 15 months Looks at book, pats page 18 months Turns pages, several at time 2 years Turns pages, one at time

Answer 213

Developmental milestones: social behaviour and play The table below summarises the major social behaviour and play milestones Age Milestone 6 weeks Smiles (Refer at 10 weeks) 3 months Laughs Enjoys friendly handling 6 months Not shy 9 months Shy Takes everything to mouth Feeding Milestone Age May put hand on bottle when being fed 6 months Drinks from cup + uses spoon, develops over 3 month period 12 -15 months Competent with spoon, doesn't spill with cup 2 years Uses spoon and fork 3 years Uses knife and fork 5 years Dressing Milestone Age Helps getting dressed/undressed 12-15 months Takes off shoes, hat but unable to replace 18 months Puts on hat and shoes 2 years Can dress and undress independently except for laces and buttons 4 years Play Milestone Age Plays 'peek-a-boo' 9 months Waves 'bye-bye' Plays 'pat-a-cake' 12 months Plays contentedly alone 18 months Plays near others, not with them 2 years Plays with other children 4 years

Answer 214

buccal midazolam

Answer 215

sodium valproate

Answer 216

ethosuximide

Answer 217

levetiracetam, lamotrigine

Answer 218

6 months - 5 years old

Answer 219

Any febrile illness can cause febrile seizures, but around 80% are viral. Common causes include: * Respiratory tract infections * Otitis media (this will be the kid who is touching his ear a lot – could ask about this in Hx) * Urinary tract infections * Influenza.

Answer 220

The majority of children (75%) present with a simple febrile convulsion. This is typically a generalised tonic-clonic seizure, presenting with muscle stiffness and jerking or shaking of the limbs, without focal features. Additional features may include: * Breathing difficulties * Pallor * Cyanosis * Loss of consciousness This is often followed by post-ictal drowsiness and confusion, which may last anywhere from several minutes to hours. Seizures rarely last longer than 10 minutes, occur once within 24 hours or within the same febrile illness, and resolve spontaneously, the vast majority with full recovery within 1 hour

Answer 221

febrile seizure lasting longer than 30 misn

Answer 222

red flag symptoms: o Waking at night or present on waking in the morning o Features of meningism o Vomiting or ataxia o Aggravated by coughing, sneezing, or bending down o Progressively worsens o Change in conscious level or pervasive lethargy o Within 5 days of head injury o Squint or failure of upward gaze o New onset cognitive dysfunction

Answer 223

An eyewitness history is key to establish the duration and nature of the seizure. Important features to ask about: Presence of fever * Onset * Peak temperature * Duration Details of seizure * Characteristics * Duration It is also important to clarify whether this is the first presentation of a seizure. Additional factors to note include developmental history and immunisation history, including any recent immunisations.

Answer 224

ventriculoperitoneal shunt

Answer 225

1. paracetoml / ibruprofen 2. nasal sumatriptan 3. combination of above ^

Answer 226

Sturge–Weber syndrome

Answer 227

neurofibromatosis type 1

Answer 228

neurofibromatosis type 2

Answer 229

* Step 1: (0 min) o Secure airway o Check ABC, high flow Oxygen2 if available o Check blood glucose o Confirm clinically that it is an epileptic seizure * Step 2: (5 min) o If IV access, IV lorazepam o If NO immediate IV access:  Buccal midazolam  Rectal diazepam * Step 3: (15 min) o if no response, give a second dose of IV lorazepam o Call for senior help o Start to prepare phenytoin for step 4 o Re-confirm it is an epileptic seizure * Step 4: (25 min) o Seek senior anaesthetist / inform ICU o Phenytoin 20 mg/kg by intravenous infusion over 20 mins  or (if on regular phenytoin): Phenobarbital 20 mg/kg intravenously over 5 mins o Consider rectal paraldehyde 0.8 ml/kg * Step 4: (45 min) o Rapid sequence induction of anaesthesia using thiopental sodium * Consider dexamethasone if vasculitis/cerebral oedema is possible * NOTE: treat reversible causes if identified (e.g. thiamine if malnourished or glucose if hypoglycaemic)

Answer 230

* Affected females sometimes need corrective surgery for external genitalia (they have a uterus and ovaries so they are reared as girls) * Definitive surgical correction is usually delayed until puberty * Acute (salt losing crisis) o IV saline (0.9% sodium chloride), IV hydrocortisone 200mg, IV dextrose * Long Term o Life-long glucocorticoids (hydrocortisone) to suppress ACTH levels (and hence testosterone) o Mineralocorticoids (fludrocortisone) if there is salt loss o Monitoring growth, skeletal maturity, plasma androgens and 17α-hydroxyprogesterone levels o Additional hormone replacement at times of illness or surgery

Answer 231

21-hydroxylase deficiency (90%) impairs the conversion of 17-hydroxyprogesterone to 11-deoxycortisol, leading to cortisol deficiency and excess androgen production 11-beta hydroxylase deficiency (5%) results in hypertension due to excess deoxycorticosterone 17-hydroxylase deficiency (very rare) leads to mineralocorticoid excess with low androgen and estrogen levels

Answer 232

Infantile spasms 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 / high dose pregnisolone ACTH is also used

Answer 233

The osmotic pressure range between intravascular and brain cells during DKA treatment has also been proposed as a cause of cerebral edema. Diabetic ketoacidosis causes an increase in plasma osmolality, whereas osmolytes accumulate in brain cells, resulting in no osmotic pressure gap mannitol or hypertonic sodium chloride

Answer 234

* Discuss factors that led to this episode * Advice on how to manage intercurrent illness (e.g. viral infections leading to increased insulin demand)

Answer 235

o Three Types of Insulin Therapy  Multiple Daily Injection Basal-Bolus: injections of short-acting insulin or rapid-acting insulin analogue before meals, with 1 or more separate daily injections of intermediate acting insulin or long-acting insulin analogue  Continuous Subcutaneous Insulin Infusion (insulin pump therapy): programmable pump and insulin storage device that gives regular or continuous amounts of insulin (usually rapid-acting insulin or short-acting insulin) by a subcutaneous cannula  One, Two or Three Insulin Injections Per Day: injections of short-acting insulin or rapid-acting insulin analogue mixed with intermediate-acting insulin o Offer multiple daily injection basal-bolus insulin regimens from diagnosis o If this is inappropriate, consider continuous subcutaneous insulin infusion (CSII or pump therapy)

Answer 236

* 1st line: Medical - Carbimazole or propylthiouracil * 2nd line: Radioiodine treatment, Surgery (partial thyroidectomy)

Answer 237

* 1st line: Medical - Carbimazole or propylthiouracil * Important: both thionamides are associated with a risk of neutropaenia o Families should be safe-netted about seeking urgent medical attention and a blood count if a sore throat or fever occur whilst on treatment

Answer 238

* Management of Acute Symptomatic Hypocalcaemia o IV calcium gluconate * Management of Chronic Hypocalcaemia o Oral calcium o High dose vitamin D analogues o Important: avoid hypercalciuria because it can lead to nephrocalcinosis so urinary excretion should be monitored

Answer 239

CATS go numb Convulsions arrythmias tetany spasms and stridor numb = parathesia

Answer 240

McCune-Albright syndrome

Answer 241

Precocious puberty Definition 'development of secondary sexual characteristics before 8 years in females and 9 years in males' more common in females Some other terms thelarche (the first stage of breast development) adrenarche (the first stage of pubic hair development) May be classified into: 1. Gonadotrophin dependent ('central', 'true') due to premature activation of the hypothalamic-pituitary-gonadal axis FSH & LH raised 2. Gonadotrophin independent ('pseudo', 'false') due to excess sex hormones FSH & LH low Males - uncommon and usually has an organic cause 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 242

* Mild-to-Moderate Hypoglycaemia o Give fast-acting glucose by mouth (usually liquid carbohydrate (e.g. Lucozade)) o May need to be given in small amounts if vomiting o Recheck blood glucose within 15 mins and repeat fast-acting glucose if hypoglycaemia persists o As symptoms improve, give oral complex long-acting carbohydrate to maintain blood glucose levels * SEVERE Hypoglycaemia o Treat in hospital  Give IV 10% glucose (maximum dose of 500 mg/kg of bodyweight (5 ml/kg)) o If NOT in hospital: unresponsive or PO route cannot be used  IM glucagon or concentrated oral glucose solution (e.g. glucogel) * IM glucagon: 500 µg for < 8 years; 1 mg for > 8 years  Seek medical help if blood glucose remains low after 10 mins  Once symptoms improve, give oral complex long-acting carbohydrate * NOTE: alcohol is a risk factor for hypoglycaemia (they should eat carbohydrates before and after drinking)

Answer 243

Secretions, foreign body Stridor See-sawing Open airway – jaw thrust and position: * Infant – neutral position * Children – ‘sniffing’ position * NB. C-spine control Remove obstruction if safe

Answer 244

Respiratory rate Symmetry Wheeze Work of breathing Auscultate and monitor Oxygen Support breathing

Answer 245

Pulse rate and volume Blood pressure Capillary refill time Fluid/blood Chest compressions Defibrillation Look at the colour and temperature of peripheries Measure capillary refill time Auscultate the heart Insert 1 or 2 large bore cannulas Reassess every 5 mins Consider fluid bolus if hypotensive

Answer 246

Disability Consciousness Pupils Posture AVPU/GCS Collateral history re. seizures, trauma, poison, sepsis, diabetes

Answer 247

* Give IM adrenaline 1: 1000 (as per age-related guidelines) * Give high flow oxygen * Give IV fluids * Give IV chlorphenamine 10 mg + IV hydrocortisone 200 mg

Answer 248

* At birth, delayed cord clamping if possible * Dry the baby, remove any wet towels and covers and start the clock or note the time * Within 30 seconds: assess tone, breathing and heart rate * Within 60 seconds: if gasping or not breathing – open the airway and give 5 inflation breaths o Consider SpO2 and ECG monitoring * Re-assess: if no increase in heart rate, look for chest movement * If chest NOT moving: o Check mask, head and jaw position o Consider 2-person airway control o Consider suction, laryngeal mask/tracheal tube o Repeat inflation breaths and look for a response o Consider increasing inflation pressure * If NO increase in heart rate: look for chest movement * When chest is moving: if heart rate is not detectable or slow (< 60/min) ventilate for 30 seconds * Reassess heart rate: if still < 60 bpm o Start chest compressions with ventilation breaths (3:1) o Increase oxygen to 100% o Consider intubation if not already done or laryngeal mask if not possible * Reassess heart rate every 30 seconds: if heart rate is not detectable or slow (< 60/min): o Vascular access and drugs (e.g. atropine) o Consider other factors, e.g. pneumothorax, hypovolaemia, congenital abnormality

Answer 249

5 rescue breaths

Answer 250

Gillick Competence * At 16 years or older, a young person can be treated as an adult and be presumed to have capacity to decide * Under the age of 16 years, children may have capacity to decide, depending on their ability to understand what is involved * Where a competent child refuses treatment, a person with parental responsibility or the court may authorise investigation or treatment which is in the child’s best interests * Family Law Reform Act (1969) o Those over 16 can consent to treatment but CANNOT REFUSE TREATMENT under 18 years old, unless there is one consenting parent, even if the other disagrees

Answer 251

* Variety of Presentations o Bruising o Broken bones o Drowsiness (subdural haematoma) o Neglect (e.g. unkempt) o Failure to thrive * Is the child in DANGER? o Could the siblings or parents be in danger? o MAKE SURE THE CHILD IS IN A SAFE PLACE * Who to get involved? o Senior colleagues o Named doctor for child protection o Contact social services and make a formal referral o Consider contacting the police (Child Abuse Investigation Team (CAIT)) o Consider contacting Multi-Agency Safeguarding Hub (MASH)  This includes a variety of people that help manage different aspects of a child’s life * Investigations o Skeletal survey o CT +/- MRI head scan o Bloods and bone profile  Rule out leukaemia, ITP etc. o Ophthalmology referral (fundoscopy for retinal haemorrhages) * GENERAL RULE: if you are suspecting NAI, it is always safe to admit the child

Answer 252

24 hours after abx

Answer 253

48 hours after abx

Answer 254

4 days from onset of rash

Answer 255

5 days from onset of rash

Answer 256

once all lesions crusted over

Answer 257

once all lesions have crusted over

Answer 258

5 days from onset of swollen glands

Answer 259

30 – 40 110 – 160 70 – 90

Answer 260

until sx settled for 48 hours

Answer 261

20 – 30 95 – 140 80 – 100

Answer 262

15 – 20 80 – 120 90 – 110

Answer 263

12 – 16 60 - 100 100 - 120

paeds Flashcards

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