General paeds - SJS Flashcards

Question

What is the management of NEC

Answer 1

**Basics** NBM (gut rest) NGT (drainage) Insert IV line for fluids and to take sample for FBE & blood cultures **Place and Person** Refer to paeds gastroenterology / surgery **Investigate and confirm diagnosis** AXR is gold standard Rationalise ABx once cultures back **Definitive management** Empirical antibiotic therapy: Metronidazole Ampicillin Gentamycin Only in stage 3B disease: Surgery to resect affected section with stoma diversion and abscess drainage if needed

Answer 2

Projectile, non-bilious vomiting First born, Caucasian male with a positive family history Hypochloraemic, hypokalaemic metabolic alkalosis (also get hyponatraemia too, but not included in the buzz word)

Answer 3

Usually between 2-6 weeks of life

Answer 4

A sudden onset of vomiting between 2 and 6 weeks of life. Before the onset of vomiting, these infants feed well and are thriving. The infant then loses weight (or inadequately gains weight) and becomes dehydrated Projectile, non bilious vomiting. May contain altered blood. “Hungry vomiters” – vomit immediately after feeding, and then are hungry again May be constipated (not absolute) **Examination** Palpable, mobile pyloric tumour in the midline of the epigastrium between the rectus abdominal muscles OR the right rectus and the liver edge (“olive”)

Answer 5

Behaviour/demeanour HR/BP Cap refill (check centrally) Sinking of the anterior fontanelle and eyes Tear production/Urine output Skin turgour Mucus membranes Weight loss

Answer 6

**Usually a clinical diagnosis** ## Footnote **Bedside** ABG --\> hypochloremic, hyponatremic, hypokalaemic, metabolic alkalosis BGL **Bloods and urine** FBE UEC --\> hydration Coags **Imaging** Ultrasound If very unsure you can do a barium meal (not usually needed)

Answer 7

It is particularly important to fully correct serum bicarbonate before theatre because of the risk of hypoventilation/ apnoeas post-operatively in the setting of metabolic alkalosis.

Answer 8

**Medical (not surgical) emergency** ## Footnote **Basics** IV access and fluid resusc if required and as directed by UEC/ABG results NBM NG tube if continuing to vomit **Place and person** Paediatric gastroenterology, admit **Definitive** Pyloromyotomy (Ramstedt operation).

Answer 9

10-20ml/kg of normal (0.9%) saline, which may be repeated

Answer 10

Malrotation + volvulus until proven otherwise!

Answer 11

**History** Presents day 1-3 of life (60–80% of all cases of malrotation present in the first month of life, mostly in the first week.) Bilious vomiting (malrotation until proven otherwise!) No dirty nappies once there is volvulus Rectal bleeding (a late symptom/sign) Abdominal pain **Examination** Abdominal distension (late sign) Peritonism (late sign)

Answer 12

AXR - non-specific, gas in bowel, can get "double bubble sign" **Barium swallow - corkscrew sign** USS - whirlpool sign

Answer 13

**Basics** NBM NGT – check aspirates for bile Insert IV and commence fluids **Place and person** Admit and refer to paediatric surgeon **Ix** ABG Septic screen Blood group and hold **Definitive management** Urgent surgical correction with follow up Ladd procedure: untwist counter-clockwise, divide Ladd’s band, widen mesentery attachment, appendicectomy, and replace the bowel in the right location. Where there is necrotic bowel second laparotomy performed in 24-48hrs later to see if viable gut can be saved – resection

Answer 14

**Gastro** FAMINE HIP GAIT **Neurological** Increased ICP **Infectious** Gastroenteritis Meningitis Pertussis (post tussive cough) **Endocrinological** DKA Errors of Inborn Metabolism **Psychogenic / Behavioural** Overfeeding in infants Bulaemia in adolescents Pregnancy in adolescents

Answer 15

FAMINEHIPGAIT

Answer 16

Oxygen therapy if hypoxic Assisted ventilation if increased work of breathing NG feeding if poor feeding NG or IV fluids if dehydrated

Answer 17

**Basics** DRSABCD NBM **Place and person** Transport to surgical unit via NETS **Ix** ECG, ABG, BGL FBE, UEC, LFTs, LDH, Coags, Group and hold AXR, CT, USS **Definitive** * *Drip and suck-* * NBM and NG tube suction - relieves any respiratory distress from abdominal distension (may also be caused by vomit aspiration) * Rapid IVF resuscitation with boluses of 10mL/kg given at 15 minute intervals if shocked otherwise give maintenance and mL by mL match for NGT losses. * Glucose- monitor BSLs and glucose solution should be given with IVF (glucose stores low, no absorption --\> anaerobic metabolism) * Correct Hypothermia * monitor temperature and have overhead heater * Correct acidosis correction of fluid loss, hypothermia and glucose will correct acidosis * Sepsis * if at risk administer IV Abx after cultures taken * Surgical correction if required- according to cause

Answer 18

Plus also ask about sleep

Answer 19

**SCOFF** Do you make yourself **Sick** because you feel uncomfortably full? Do you worry you have lost **Control** over how much you eat? Have you recently lost more than **One** stone (6.35kg) in a three month period? Do you believe yourself to be **Fat** when others say you are thin? Would you say **Food** dominates your life? **Plus to screen for bulimia nervosa:** Are you satisfied with your eating pattern? Do you ever eat in secret?

Answer 20

The 5 Hs 1. Height (growth retardation) 2. Heart (arrythmia, QT, bradycardia, hypotension) 3. Happy Bones (irreversible osteoporosis) 4. Hormones (osteporosis, hypothyroid, delayed puberty/amenorrheoa) 5. Heads (poor concentration, slowed mentation)

Answer 21

History provided not consistent with injury Delay in presentation Claims that injury caused by young sibling Denial that it is an injury Multiple injuries of different types and ages Injuries not consistent with developmental age Child brought in by person other than parent **Shaken baby syndrome --\> subdural haematoma + retinal haemorrhage**

Answer 22

Unless it is catastrophic epilepsy, early treatment does not change long term outcome Be hesitant to make a diagnosis because it will change a child's life forever Epilepsy will declare itself by progression or resolution.

Answer 23

**Time course** Red is raised ICP Green is migraine Blue is muscular **Severity** Best to ask what they did during the headache to determine severity (confined to dark room = severe)

Answer 24

**Relief by sleep** **Dizziness/pallor** Abdo pain Nausea and vomiting Prodrome Characteristic location of pain

Answer 25

**Rhinovirus** Adenovirus Influenza and parainfluenza RSV Enterovirus EBV Streptococci

Answer 26

* ear, nose, or throat infection * acute respiratory infection * gastroenteritis * urinary tract infection * meningitis * osteomyelitis / septic arthritis

Answer 27

Bacterial infection

Answer 28

Urine dipstick not useful in babies under 1 because they pee as soon as urine created which means not enough time for bacteria/WCC to accumulate Can cause false negative!

Answer 29

MR SARS EEK! * Measles * Rubella * Systemic JCA (Still’s disease) * Allergy * Roseola infantum (HHV6) * Scarlet fever * Erythema infectiosum (Parvovirus B19) * Erythema multiforme * Kawasaki disease

Answer 30

Mild, diffuse dilatation of coronary arteries usually begins on day 10 from fever onset. If left untreated, 25% of these patients progress to true aneurysms, with 1% becoming ‘giant aneurysms’ (\>8 mm internal diameter). These giant aneurysms have the worst prognosis, with risk of acute thrombosis and long-term risk of myocardial ischaemia * Carditis during the febrile phase * myocarditis with ST-T changes (25%) * pericardial effusions (20-40%) * valvular dysfunction (1-2%) * cardiac failure (~5%) * Coronary vessel abnormalities (occur in 20% of cases if untreated and * aneurysm formation * may lead to fatalities from * thrombosis, * rupture or * ischemia-related dysrhythmia

Answer 31

Rash from strep throat. Tx with phenoxymethylpenicillin (PO) or benzathine penicillin (IM) to prevent post strep GN or acute rheumatic fever

Answer 32

Hand, foot and mouth Chickenpox Herpes simplex (Remember: vesicular just means blistering)

Answer 33

Fever Stiff neck\*/Kernig's sign Bulging fontanelle Irritable Drowsiness Poor feeding/vomiting **Palpable non-blanching purpuric rash =\> meningococcal meningitis** Convulsions/coma/shock --\> consider meningitis **Stiff neck is not relevant in infants**

Answer 34

Transplacental transfer of IgG happens in the third trimester. Very premature babies can miss out on this process rendering them very vulnerable to infection NB: The process is actually active and means that the baby ends up with a higher concentration of IgG than their mum!

Answer 35

**\> 38.0 degrees** **INVESTIGATE IT!** FBE blood culture CRP Urine dipstick/Urine MCS CXR only if respiratory signs are present stool culture, if diarrhoea is present

Answer 36

Viral lower respiratory tract infection Up to 18 months

Answer 37

**_RSV_** Picornovirus Human metapneumovirus Adenovirus --\> bad because causes bronchiolitic obliterans! Parainfluenza Influenza

Answer 38

Exposure to cigarette smoke Maternal smoking during pregnancy Prematurity Chronic cardiorespiratory diseases (including congenital heart disease and cystic fibrosis) Immunodeficiency

Answer 39

None, clinical diagnosis However, nasopharyngeal aspirate is usually done because we like to know which viruses are doing the rounds in the community and to rule out pertussis (which is life threatening to infants)

Answer 40

**Basics** Oxygen therapy if hypoxic **Person and Place** Criteria for admission: * \<50% normal oral intake * Sats \<93% * Parental anxiety * Not yet day 2/3 and expected to worsen **Investigations and Diagnosis** Clinical diagnosis, NPA if required to isolate virus or exclude pertussis **Definitive Management** * NG feeds * IV fluids * O2 support * Low or high flow nasal prongs * CPAP * Intubation **Long-Term Management** Usually resolves after a week RSV is a highly infectious virus, ensure good hand hygiene to prevent spread. Ensure immunizations are up to date

Answer 41

Bronchiolitis / bronchitis Asthma / viral induced wheeze Viral pneumonitis Croup (can cause wheeze) Pneumonia Pertussis Bronchiomalacia/tracheomalacia Cardiac failure CF or other suppurative lung dz Recurrent aspiration Inhaled foreign body

Answer 42

6 months to 12 years of age, with a peak incidence at age 2

Answer 43

If you're thinking about hypoxia the croup is life threatening. Adequate SPO2 should not be reassuring – low oxygen saturations is a BAD sign.

Answer 44

Parainfluenza Influenza Adenovirus

Answer 45

* Begins with coryzal symptoms for 1-2 days * Fever is generally low grade (38-39) – no signs of toxicity * Barking cough – usually begins at night or early hours of morning. Peaks at day 2 and 3 of disease * It takes two or three days before the sub-glottic area narrows enough to cause the barking cough * It happens first at night, usually, because we have reduced upper airway tone when we sleep * Inspiratory stridor is heard as the disease progresses * The viral illness typically lasts 7-10 days, but the **typical croupy cough only lasts 2-3 nights** * May have associated widespread wheeze, increased work of breathing and hypoxia – child may become distressed and restless, cyanosis is a late and ominous sign * Recurrent spasmodic croup * ‘Upper airway hyperresponsiveness’ * No viral prodrome * Children are well when they go to bed, and wake up in the early hours of the morning with a barking cough and stridor * Fever is unusual in this form of croup * Children often have asthma or atopy, or a FHx of asthma and atopy

Answer 46

None, dont distress the kid!

Answer 47

**Basics** Minimal handling Oxygen is not usually required unless severe obstruction IV access should be deferred **Place and person** Grade 1 – manage at home Grade 2 – admit to hospital via ED Grade 3 – admit to ICU If having to administer oxygen, contact anesthetics! If having to administer a second dose of adrenaline, contact anaethetics! **Investigate and confirm diagnosis** Clinical diagnosis **Non-invasive management** Keep child relaxed **Definitive management** *Mild to moderate* Oral prednisolone 1mg/kg OR Oral dexamethasone 0.15mg/kg – 0.6mg/kg *Severe* * Oxygen * Nebulised adrenaline 1:1000 solution * Works by vasoconstriction * Beware of possible rebound effect after 2-3 hours – must observe * This is more symptomatic treatment to buy you time * Dexamethasone IV 0.2mg/kg * Follow with oral steroids * ETT if going into respiratory failure **Long term** Consider transfer when no improvement following nebulized adrenaline **Discharge requirements** Four hours post nebulized adrenaline Half hour post oral steroid Stridor free at rest **Recurrent-Spasmodic Croup** Usually don’t require treatment as attacks are very short-lasting. But can treat the same as acute viral croup. Some evidence to suggest that ICS may reduce frequency of attacks, but children will most likely grow out of this.

Answer 48

Most common age group in community is adolescents and young adults, but not dangerous for this group except for being vector Under 12 months deadly Under 6 months VERY deadly

Answer 49

Transmission: Direct contact with droplet discharges Highly contagious – spreads to 80-90% of susceptible individuals coming in contact with disease

Answer 50

**Catarrhal stage (7-14 days)** * Anorexia * Rhinnorrhoea * Conjunctivitis/lacrimation * Dry cough **Paroxysmal stage (about 4 weeks)** * Paroxysms of severe coughing with inspiratory ‘whoop’ * Vomiting (after coughing) * Coughing mainly at night * Lymphocytosis **Convalescent stage** * Chronic cough which may last for weeks

Answer 51

FBE --\> lymphocytosis CXR to exclude pneumonia Culture and/or PCR of nasopharygeal aspirate (within 1 week from onset of cough). Both of these tests have 70% sensitivity

Answer 52

**Basics** ABC **Place and person** If younger than 6 months – hospitalization School exclusion until at least 5 days of antibiotic use **Investigate and confirm diagnosis** Nasopharygeal aspirate FBE, IgA serology CXR – to exclude pneumonia **Non-invasive management** Good ventilation Avoid emotional excitement or distress Avoid overfeeding during paroxysmal stage **Definitive management** * Pertussis infection (Group B disease) requires written notification within five days of diagnosis. * Antibiotics minimize transmission but do not effect course of disease * Azithromycin 500 mg 5 days OR * Clarithromycin 7 days **Prophylaxis** Same treatment as above for household and other close contacts if commenced within 3 weeks of onset of cough in the patient **Prevention** * Acellular pertussis is part of the immunization schedule * Initial protection * 2, 4 and 6months * Booster (dTpa) * 4 years * 10-15 years * Parents or household contacts of newborns

Answer 53

Pneumonia Atelectasis Pneumothorax Seizure Asphyxia Apnoea/bradycardia Rib fracture Encephalopathy Otitis media

Answer 54

SABA PRN only, NO ROLE FOR PREVENTER IT IS NOT EFFICACIOUS

Answer 55

**Step 1: RELIEVER** SABA **Step 2: PREVENTOR** Low dose ICS (200 – 400mcg / day) OR LTRA OR inhaled cromone **Step 3: ADD OR INCREASE STEROID** Increase ICS (Double: 400 – 800mcg / day) LTRA / inhaled cromone + low dose ICS **Step 4: ALL PREVENTORS AND HIGHEST ICS DOSE** LTRA / cromone + ICS (800mcg) **Step 5: ADD SYMPTOM CONTROLLER / ORAL STEROIDS** LABA (not in children

Answer 56

Rrecurrent wheezing episodes which are responsive to salbutamol.

Answer 57

Boys generally have smaller airways than girls and tend to suffer more from asthma. However, after adolescence the prevalence is higher in females.

Answer 58

Very safe, almost not clinically significant long term side effects Hoarseness and pharyngeal candidiasis are not common Treatment beginning before puberty is associated with a small (mean approximately 1 cm) reduction in adult height

Answer 59

autosomal recessive

Answer 60

**MR PANCREAS** ## Footnote M - Meconium ileus (at birth) and distal intestinal obstruction (children and adults) R - Recurrent infections P - Pancreatic failure (exocrine and endocrine) A - Allergic bronchopulmonary aspergillosis N - nasal polyps and recurrent sinusitis C - Cirrhosis of hepatobiliary system E - Emotions (teenagers often depressed) and electrolyte disturbances (low Na+) A - Airway leaks (pneumothorax) S - Sterility in males

Answer 61

**Basics** NA **Place and person** Referral to * Respiratory physician * Chest physiotherapy * Dietitian **Investigate and confirm diagnosis** Sweat test and genotyping **Non-invasive management** * Chest physiotherapy * Monitoring and optimizing nutrition * High intake of dietary fat * Pancreatic enzyme supplements * Fat soluble Vitamin supplements **Definitive management** * For respiratory infections * S. aureus infection * Di/flucloxacillin * S. aureus and Haemophilus influenzae infection * Augmentum or trimethoprim and sulfamethoxazole * Initial pseudomonal infection * Ciprofloxacin PLUS * Tobramycin by nebulizer OR * Colistimethate sodium by nebulizer * Chronic pseudomonal exacerbation * Ciprofloxacin PLUS * Tobramycin by nebulizer OR * Colistimethate sodium by nebulizer OR * Gentamicin by nebulizer **Long term** * Respiratory * Daily chest physiotherapy * Aerosolised mucolytics * Dornase alfa * Nebulised hypertonic saline * Inhaled bronchodilators * Inhaled corticosteroids **Chronic pseudomonal infection** Azithromycin orally 3 times weekly **GORD** PPI

Answer 62

Exomphalos is a type of abdominal wall defect. It occurs when a child’s abdomen does not develop fully while in the womb. Tx = surgical correction in first 2 days of life

Answer 63

Hydrocele. It will self resolve. But you must make sure to rule out testicular torsion.

Answer 64

Gastroschisis Urgent surgical repair

Answer 65

Phimosis (the foreskin cannot be fully retracted over the glans penis). Most kids born with non-retractile foreskin. Normal up until puberty. If not loosening by age 4 prescribe steroid cream to promote loosening of the skin.

Answer 66

Paraphimosis (the foreskin becomes trapped behind the glans penis, and cannot be reduced). Management: Apply local or general anaesthetic, compress the oedema (may have to apply pressure for \>5 mins) to drain the fluid. If that fails contact paeds surg to puncture the skin with a needle and aspiration fluid. Then replace the foreskin.

Answer 67

**1,2,3,4,5 T’s** 1 vessels (Truncus arteriosus) 2 vessels swap (Transposition of the Great Arteries) 3 = Tricuspid atresia 4 = Tetralogy of Fallot 5 words (Total Anomalous Pulmonary Venous Return)

Answer 68

Septal defects cause L --\> R shunts ACEi, diuretics to reduce preload and strain on right heart plus feeding to increase baby's weight ready for surgical correction

Answer 69

Identified if infant experiences heart failure, or later in life because doctor hears machinery murmur or notices wide pulse pressure (caused by the run-off of blood flow from the aorta to the pulmonary artery during diastole). **What is the problem?** Blood flows from the high pressure aorta into the low pressure pulmonary artery, overloading pulmonary circulation and causing pulmonary hypertension and heart failure (dyspeneoa, FTT, recurrent infections). If not picked up until adulthood pulmonary hypertension can be a significant issue. Children with a small PDA are often asymptomatic, but may develop Infective Endocarditis.

Answer 70

**Bedside tests** ECG – normal with small PDAs. Left ventricular hypertrophy with larger PDAs **Bloods and urine** Nil **Imaging** CXR – full pulmonary artery silhouette and increased pulmonary vascularity. Cardiomegaly may be present in a larger shunt. Echocardiography – visualise the PDA to confirm diagnosis **Special tests** Nil

Answer 71

**Premature infants** * IV indomethacin in patients with very low birth weight * IV indomethacin or ibuprofen of premature infants of larger weight * Treatment can be given before evidence of a PDA becomes evident * Prophylactic indomethacin might increase ductal closure and decrease the incidence of intraventicular haemorrhage **Term infants** * In asymptomatic or symptomatic patients, percutaneous catheter closure is the first-line treatment in order to treat heart failure and prevent elevated pulmonary pressures or endarteritis. * Infants who are symptomatic and too small to undergo percutaneous device closure need ACEi/diuretic treatment and feeding until they are large enough for surgical management

Answer 72

**When does it present?** If severe: acute circulatory collapse at 2 days of life after being previously well (duct closure). This is the usual scenario. If mild: Later in life, often discovered when pt is being investigated for refractory hypertension. The later the abnormality is discovered the harder the HTN is to treat. **How does it present?** Sick baby Hypertension Unequal BP between arms Absent femoral pulses Severe metabolic acidosis May have an ejection systolic murmur between the shoulder blades **MGMT?** ABC, prostaglandins, urgent surgery

Answer 73

The Norwood operation, which connects the aorta and the returning pulmonary veins to the R heart – a one chamber heart! Usually receives follow up operations at 3 months and 6 years

Answer 74

In the immediate newborn period blood flows through the 'Ductus' into the Descending Aorta and hence reaches the lower part of the circulation. Fortunately these babies usually have a VSD, meaning that the blood that flows through the pulmonary artery, though the ductus and into the descending aorta is at least partly oxygenated. As the ductus closes after birth blood pressure in the lower circulation becomes inadequate and severe symptoms develop. Most affected infants develop severe symptoms (difficulty breathing and impaired kidney function) in the first week of life and need urgent surgery **Mx = ABC, prostaglandins, surgery in days**

Answer 75

Presents early in life (usually D2 when duct closes) as the “shocked neonate” – dusky colour, weak or absent pulses, low BP, reduced UO, metabolic acidosis

Answer 76

duct dependent In the first week of life When the ductus closes

Answer 77

Small VSD – high pitched pan-systolic murmur, associated with a thrill Large VSD – Quiet pansystolic murmur, heave

Answer 78

VSD in both

Answer 79

Small --\> conservative management and monitoring Large --\> surgical repair

Answer 80

Secundem (most common) Primium (AVSD)

Answer 81

Most affected children are free of any major symptoms, but the risk of heart failure developing later in life makes closure desirable, unless the defect is very small. These defects may be repaired surgically or by using an expanding plug ("Device"), which can be inserted through a heart catheter without an operation (Device Closure)

Answer 82

OH CRAP THAT BABY IS BLUE! DONT SWEAR IN FRONT OF THE BABY! OH, I SAID, **CRIP** * *C**yanotic Heart Disease (1,2,3,4,5 Ts) * *R**espiratory disease (Meconium aspiration syndrome, pulmonary hypoplasia) * *I**nfection (GBS) * *P**ersistent pulmonary HTN of the newborn (PPHN)

Answer 83

Pulmonary stenosis Right ventricular hypertrophy Overriding aorta VSD Pentology --\> ASD

Answer 84

Murmur, cyanosis or congestive heart failure, typically develops over the first 6-12 weeks of life If mild disease not picked up until later childhood. Presentation at this stage is characterised by FFT, clubbing of the fingers, reduced exercise tolerance and ‘Tet spells’

Answer 85

Comfort the child Rest younger infants against the parents shoulders with knees tucked in Encourage older children to squat O2, IV fluids Beta-blockade and ICU support may be required IM morphine can reduce pain and venous return Phenylephrine is last line\* \*Used to increase systemic venous resistance by direct simulation of peripheral alpha-adrenergic receptors. The goal is to increase the systemic vascular resistance above the resistance of the pulmonary outflow tract so that blood in the right ventricle preferentially flows through the pulmonary circulation, rather than across the ventricular septal defect and into the systemic circulation.

Answer 86

Heart failure is rare. But cyanosis --\> polycythemia --\> thromboembolism can occur. Infective endocarditis and cerebral abscess too!

Answer 87

**CATCH** **22q11 (Di George Syndrome)** Tetralogy of Fallot is famously associated with the deletion of the long arm chromosome 22 Cardiac anomalies (tetra, transpos) Abnormal facies Thymic hypolasia (T cell deficiency) Cleft palate Hypocalcaemia/hypoparathyroidism

Answer 88

Prostaglandins Atrial baloon septostomy Arterial switch is definitive surgical correction at 6-9 months

Answer 89

Right ventricular hypertrophy Coarse ES murmur on ULSE

Answer 90

* This defect is a form of Tetralogy of Fallot * Blood cannot enter the pulmonary circulation via the pulmonary valve. * Blood entering the right ventricle moves into the left ventricle via an ASD/VSD or foramen ovale, is pumped via the left ventricle and then shunts back to the pulmonary circulation via the PDA. * Survival depends on the ductus remaining open in the early days of life * Cyanotic heart disease * Surgical correction with a shunt is required immediately, with complete repair performed after age 1.

Answer 91

A single ventricular outflow tract straddles a large VSD. It has an **abnormal** valve. Presents in newborn period with **mild** cyanosis and congestive heart failure. The abnormal valve is often stenosed (systolic murmur) and may be regurgitant (diastolic murmur). Urgent surgery

Answer 92

ASD If symptomatic or causing R ventricular dilation, catheter closure at 3-5 years Partial AVSD needs surgery at 3 years Complete AVSD: 3-5 months

Answer 93

Small VSD Usually no treatment required, spontaneous closure

Answer 94

**Large VSD** Diuretics, captopril Extra calories Surgery at 3-6 months

Answer 95

* Normally, the pulmonary valve closes a little after the aortic (A2, P2). * During inspiration, venous return in increased at the R ventricle takes a little longer to empty. * Blood pools in the lower pressures of the pulmonary circuit, reducing return to the left heart, which now finishes emptying a little sooner. * Therefore, the split normally widens on inspiration * During expiration, the right heart is given the chance to catch up a little and the split is narrowed

Answer 96

* Stabilise with diuretics, ACEIs and high caloric diet * Treat if: * Heart failure or failure to thrive on medical management * Pulmonary hypertension * Aortic valve prolapse * Infective endocarditis * Surgical and trans-catheter options exist

Answer 97

* Small VSD may have no abnormal findings * Larger VSDs may demonstrate cardiomegaly and pulmonary plethora of CXR * Biventricular hypertrophy can be shown on ECG * Diagnose with echo

Answer 98

High flow VSDs can suck in the nearby aortic leaflet by the Venturi effect and cause aortic regurgitation

Answer 99

Essentially all congenital heart abnormalities increase your risk, except for secundum ASDs

Answer 100

ALL until proven otherwise

Answer 101

Intracranial haemorrhage - 1% of ITP cases

Answer 102

* Pallor – conjunctival, palmar crease * Fatigue, listlessness * Poor growth * Flow murmur * Air hunger (severe) * Hypotension and cardiac failure (severe) * Iron deficiency can present with poor attention span, irritability and pica (eating dirt, clay, ice etc)

Answer 103

Malabsorption and occult blood loss (although rare) should be considered, but usually after failure of dietary measures. Polyps, haemangiomas, Meckel’s diverticula and hereditary telangectiasia are rare causes of GI blood loss.

Answer 104

One of the most common and serious complications of parvovirus B19 infection is transient aplastic crises in patients with chronic haemolytic anaemia such as sickle cell disease and hereditary spherocytosis. Pure red cell aplasia may also develop with a persistent infection of parvovirus B19 in immunocompromised individuals.

Answer 105

FBE Blood film Ferritin Reticulocyte count

Answer 106

A raised APTT in the absence of anticoagulant therapy suggests a deficiency in a factor or an inhibitor. An inhibitor is usually an antibody made against a factor, such as lupus anticoagulant or antibodies formed by haemophilia patients receiving recombinant factors

Answer 107

**Presentation** Clinical manifestations of platelet disorder develop **acutely** – mucosal and skin petechiae, epistaxis, rectal bleeding, haematuria, bleeding gums **Examination** Normal examination – no pallor, no lymphadenopathy or hepato-/spleno- megaly (exclude leukaemia) **Investigations** FBE --\> thrombocytopaenia, blood film shows isolated low platelets with no other findings A bone marrow aspirate is an invasive procedure with some morbidity in children who bruise easily, and is only necessary if the diagnosis is uncertain. It is rarely necessary in uncomplicated ITP. ***ITP is a diagnosis of exclusion, but an FBE and blood film may be sufficient for diagnosis if Hx and Ex consistent. It can be thought of as isolated thrombocytopenia with no other suggestive findings on Hx, Ex, FBE or blood film.***

Answer 108

Platelets consistently under 10

Answer 109

Untreated 75% in 6 weeks, 90% will resovle within 6 months With standard dose steroids 1 week With high dose steroids 2-4 days With IVIG 1-3 days

Answer 110

* **Safety precautions are key** * Allow for generous supervision, especially of young children * Avoid contact sports or any moderate-impact activity * Regular follow up * Parents must receive thorough education * Avoid NSAIDs and 5-ASA Splenectomy is a last line option. Often very effective but vaccinations and life-long antibiotic prophylaxis required.

Answer 111

**Neonates** * Often picked up on screening due to FMHx * Cord blood can be used for genetics and coagulation studies * Intracranial haemorrhage, severe birth bruising and excessive bleeding after circumcision can suggest the disese **Older children** * Haemarthroses tend to occur in walking children

Answer 112

* Mild-moderate haemophilia A can be treated with DDAVP after appropriate challenge test (\>5y/o) * Not adequate for major bleeding * Tranexamic acid can reduce mucosal bleeds and epistaxis * Severe disease can be treated with 25-40 units/kg of recombinant factor concentrates three times a week.

Answer 113

AML (less common than ALL)

Answer 114

* **CXR** is used to investigate the possibility of mediastinal masses (occurs in 50% of T-ALL). * **Lumbar punctures** are mandatory to detect occult CNS infiltration. They are often delayed until treatment has begun to reduce the risk of seeding into CNS. Also because often thrombocytopenic Many more tests are done. This is the realm of a paediatric oncologist. Tumour lysis bloods, liver and renal function, lactate dehydrogenase and coagulation studies all form part of the initial work-up at baseline.

Answer 115

It is commonly quoted that cure rates (in children) are 80%. The current rate is probably higher, especially in low risk disease. **Remission Induction** * Aims to eliminate 99% of the tumour cell population. This should achieve normal haematopoiesis and functional status. * Does the ‘heavy lifting’ --\> remaining treatment is about total eradication, relapse prevention and cure * High risk of tumour lysis in early chemo. * the breakdown products of dying cells causes hyperkalaemia, hyperphosphataemia (which causes hypocalcaemia), high blood uric acid and high urine uric acid and consequent acute uric acid nephropathy and acute kidney failure. * Tumour lysis prevention: * bicarb to alkylise urine and prevent uric acid crystal formation in kidney * allopurinol * fluid diuresis * monitor all electrolytes **CNS protection** * Most chemo penetrates the BBB poorly * Intrathecal and IV methotrexate can obviate the need for cranial irradiation (CI). CI is still used for overt CNS disease or high risk patients.

Answer 116

Strangulation/NAI Vomiting Coughing + other causes of petechiae (SHIELD)

Answer 117

Immature megakaryocytes in BM churning out platelets. The platelets are young/healthy/sticky. Vs kids with cancer with same low platelet count who will bleed because unhealthy platelets.

Answer 118

· **Oral** – paracetemol, ibuprofen, oxycodone · **Intranasal** – fentanyl (device that makes a mist out of it) · **IV** – morphine, ketamine · **IM** – (rare) ketamine, morphine · Local anaesthetic

Answer 119

* Wet nappies * Tears * Fontanelles * Skin turgor * Mucus membranes * Cap refill * HR/BP * Weight * Vomits/diarrheoa * Alertness

Answer 120

Tracheo-oesophageal fistula

Answer 121

pyloric stenosis (if age 2-7wks) duodenal atresia

Answer 122

A 'Slipped Upper Femoral Epiphysis' (also called a 'SUFE') is when the femoral epiphyseal plate is weak and the ball head of the femur slips downward and backward. The exact cause of this condition is not known. A 'SUFE' is not usually associated with an injury. The symptoms often develop slowly - over several months - and may seem like a pulled muscle in the hip, thigh or knee. It is important to get an early diagnosis and treatment before the slip gets worse and children may need to have the unaffected side treated as well to prevent future slipping. Clasically happens to obese teenage boys, can be bilateral True hip pathology causes GROIN pain! **Signs and symptoms** Groin, hip, thigh or knee pain. (Some people only have knee pain, even though the condition affects the hip) Limp Limited movement of the hip joint There may be slight shortening of the affected leg. **Diagnosis** Diagnosis is usually made by physical examination of hip movement and an x-ray. Attached X ray shows how in the abnormal side the line does not cut off the femoral head like it should **Management** Surgical correction with screw

Answer 123

Refusal to weight bear Fever (\>38.5) Raised WBC ESR \>40 CRP \>20

Answer 124

6 weeks, it gets better after that

Answer 125

Bring them back if: 1. They become dehydrated (because of vomiting/diarrheoa) with poor oral intake and no wet nappies 2. Become drowsy or not responsive to interactions 3. Puffing very hard or finding it difficult to breathe 4. Uncontrollably irritable, not able to be soothed

Answer 126

Nothing, vomiting is not a localising sign in a kid. Could be anything from UTI to meningitis.

Answer 127

Meningitis Pneumonia UTI Sepsis

Answer 128

**Febrile convulsions / seizures are seizures in which** * Fever \>38 degrees * No evidence of CNS infection * No history of afebrile seizures * No history of neurological deficits or developmental delay **Simple febrile seizures are** * generalized tonic and/or clonic seizures * Short in duration * Do not recur within the same febrile illness **Complex febrile seizures are** * focal features at onset or during the seizure * duration of more than 15 minutes * Recurrence within the same febrile illness * Incomplete recovery within 1 hour.

Answer 129

6 months to 6 years

Answer 130

3 in 100 babies

Answer 131

**Bedside tests** * BGL – this is the only investigation you should do if the child is \>6 months and the source of the fever can be identified * Urine dipstick and MC&S **Bloods and urine** * FBE * CRP * Blood culture * UEC / CMP * check for electrolyte derangement. * Feeding babies water bottles causes hyponatraemic seizures **Imaging** CXR if respiratory symptoms **Special tests** LP – low threshold if unknown source of fever. From RCH “consider LP if the child is less than 12 months and not up to date with immunisations, if they are clinically unwell, or if they are already on oral antibiotics that may mask meningitis.”

Answer 132

* Recurrence rate depends on the age of the child; * the younger the child at the time of the initial convulsion, the greater the risk a further febrile convulsion * (1 year old 50%; 2 years old 30%). * Risk of future afebrile convulsions (epilepsy) is increased by: * family history of epilepsy, * any neurodevelopmental problem, * atypical febrile convulsions (prolonged or focal). * With none of these risk factors risk of recurrence is similar to the rest of the population (1%). With 1 risk factor, risk is 2%, with \>1 risk factor, 10%.

Answer 133

* Fever control: * minimal clothing, nappy alone or light outer layer depending on ambient temperature * Tepid sponging, baths and fans are ineffective at lowering core temperature and are NOT recommended * Paracetamol has not been shown to reduce the risk of further febrile convulsions. It may be used for pain/discomfort associated with febrile illnesses (e.g otitis media),it should not be solely used to control fever * If the seizure recurs, roll him onto his side and allow him to fit, Seizure should not last longer than 3-5 minutes. Any longer and an ambulance should be called. * If Henry was to have recurrent or prolonged febrile seizures, prophylactic oral diazepam or rectal diazepam (rescue therapy) may be prescribed

Answer 134

No, not at all

Answer 135

Teething will not cause fever \> 38.5oC

Answer 136

**History** * Headache (if child can talk) * Photophobia (if child can talk) * Cough * Coryza * Diarrheoa * Vomiting * Abdominal pain * Joint symptoms * Dysuria/foul smelling urine * Travel history * Sick contacts * Immunisations **Exam**: * Vitals, general appearance, conscious state * Neck stiffness * ENT examination * Work of breathing * Abdominal exam * Skin rash * Joint swelling

Answer 137

* Sick child with fever * Vomiting * Red-yellow bulging TM * Discharge * Loss of TM landmarks * Persistent fever and pain after 3 days of conservative approach * Bilateral AOM *

Answer 138

Birth incidence is about 1 in 1000 **live** births. Overall incidence rises after maternal age of 35 years.

Answer 139

50% have congenital heart defects particularly AVSD

Answer 140

CVS = 1% above the background risk Amniocentesis = 0.5%

Answer 141

**Diagnosis** = herpes simplex gingivostomatitis **Management** = Pain relief (codeine may be required as it is very sore). Antivirals inneffective **Prognosis** = Child may have a fever and become irritable, a day or two later ulcers develop on the lips and gums. These are painful and often lead to drooling and a refusal to eat or drink, dehydration can occur. The pain normally subsides after 3-4 days, the blisters usually take from 10 -14 days to go away. The blisters never leave scars.

Answer 142

**Idiopathic Nephrotic Syndrome (90% of presenting children)** * Minimal change disease * Focal segmental glomerulosclerosis * Also called steroid-sensitive nephrotic syndrome as 80+90% of cases will respond to initial steroid therapy ******Non-idiopathic Nephrotic Syndrome (rare)** * Secondary * SLE * Henoch Schonlein Purpura * Membranoproliferative GN * Membranous nephropathy * Congenital nephrotic syndrome

Answer 143

* 80% of children with Minimal change will relapse * Steroid-responsive patients have little risk of chronic renal failure * Patients with FSGS may initially respond to steroids but later develop resistance * Many children with FSGS progress to end-stage kidney failure * Recurrence of FSGS occurs in 30% of children who undergo renal transplantation

Answer 144

**Membranoproliferative glomerulonephritis** Characterised by hypocomplementemia (low C3 and C4) with signs of glomerular renal disease 5 to 15% of children with primary nephrotic syndrome Typically persistent Has a high likelihood of progression to renal failure over time **Membranous nephropathy** Less than 5% of children with primary NS Seen most commonly in adolescents and children with systemic infections Hep B, syphilis, malaria and toxoplasmosis Or on specific medications Gold, penicillamine

Answer 145

**Usually neonates or teenagers** Sudden onset testicular pain and swelling; occasionally nausea, vomiting. Note: pain may be in the iliac fossa, may be associated with sports activity Discolouration of scrotum; exquisitely tender and swollen testis, riding high. Cremasteric reflex is usually absent. **Management**: Early surgical consultation is vital, as delay in scrotal exploration and detorsion of a torted testis will result in testicular infarction within 8-12 hours. Keep the child fasted.

Answer 146

* Perform a reduction manoeuvre (see below) * expect distress and pain * a click may be felt over the radial head * review after ten minutes * if reduction fails, consult with senior medical staff https://youtu.be/-0ROu4hCXwQ

Answer 147

Intussusception

Answer 148

**Primary – ages 3 months – 2 year olds** *Ileocaecal location* Idiopathic **Secondary – ages 4 years – 12 year olds** *Ileoileul location* Mesenteric lymph nodes HSP --\> pathological lead point of submucosal haemorrhage Meckels diverticula Haemophilia --\> submucosal haemorrhage

Answer 149

1. Fluid resuscitation 2. Analgesia 3. Air enema 4. Definitive surgery if necessary

Answer 150

Like a sock folding in on itself

Answer 151

99% in first day Rest in second day If not passed on 3rd day it's a sign of abnormality

Answer 152

In hirschprungs on rectal exam there is explosive discharge of flatus and faeces

Answer 153

Suction rectal biopsy

Answer 154

1/25 1/2500

Answer 155

Cardiac defects Down syndrome

Answer 156

Ask if either parent had pyloric stenosis when they were born.

Answer 157

20% chance

Answer 158

The vomiting may continue for 3 - 5 days, that is normal.

Answer 159

Immediately after birth = duodenal atresia 3 days after birth = malrotation with volvulus First few days of life with failure to pass stools = MI or Hirschsprungs 3-4 weeks after birth = pyloric stenosis

Answer 160

Mild dehydration = No signs Moderate dehydration = 4-6% depleted - **Signs of dehydration** Severe dehydration is \>= 7% depleted - **Shock** *For the purposes of calculation of fluid requirements use:* Mild - 3% Moderate - 5% Severe - 10%

Answer 161

**First:** For shock give 10-20ml boluses of normal saline until signs of shock improve. **Second:** Replace deficit, so for a 6kg kid if they're moderately shocked that approx 5%, so their deficit is approx 300ml, give 300ml normal saline **Third**: Give maintenance fluids using normal saline + 5% glucose. Using the 4,2,1 rule for a 6 kg infant that is 24ml/hr of normal saline + 5% glucose. **Monitor electrolytes daily and consider adding 20mmol K**

Answer 162

Cryptosporidium Giardia Lamblia

Answer 163

dysentery w blood, pus, pain and tenemus

Answer 164

**Bedside** None **Bloods and urine** UECs and glucose a minimum if commencing any IV rehydration **Cultures** Faecal culture and MCS if significant abdominal pain, blood or mucus, or septic child Faecal ova, cysts and parasites – if considering parasitic causes C difficile toxin if recent use of antibiotics

Answer 165

Post-gastroenteritis lactase deficiency Mx - Lactose free diet for a few weeks

Answer 166

Coeliac disease!

Answer 167

**Bedside** NA **Bloods** * *(not anti TTG or IgA like normal as low sensitivity under 2 years)* * Anti Gliadin * HLA testing * Screen for T1DM and thyroid disease * FBE * May show microcytic or macrocytic anaemia from iron or folate deficiency * U&E * may reveal reduced concentrations of calcium, magnesium, total protein, albumin or vitamin D **Imaging** None **Special** Gastroscopy and small bowel biopsy

Answer 168

Exclusion of gluten (barley, rye, wheat, and oats) Dietary fortification – iron, folate, calcium and vitamin D

Answer 169

1/25 boys born

Answer 170

SpO2 \<93% Oral intake \<50% of normal Parental anxiety Or, if these criteria are not quite met but the condition is likely to worsen before it gets better (remember the worst symptoms are on day 2-4) then consider admission

Answer 171

What worries you the most?

Answer 172

Subacute onset, prominent cough, +/- headache, +/- sore throat Roxithromycin PO for 10 days

Answer 173

The response of fever to paracetemol is not significant, and is not an indicate of illness severity. However, if the child was tachycardic and the paracetemol does not bring their HR down, you would consider myocarditis. If it was myocarditis admit them for monitoring and supportive care.

Answer 174

* malrotation and volvulus * duodenal atresia (if obstruction distal to Ampulla of Vater 80% of cases) * jejunoileal atresia * meconium ileus * necrotising enterocolitis

Answer 175

The first stool is passed within 24 hours of birth in 99 percent of healthy full-term infants and within 48 hours in all healthy full-term infants. Failure of a full-term newborn to pass meconium within the first 24 hours should raise a suspicion of intestinal obstruction.

Answer 176

Exophalos (omphalocoele) - large defect at the umbilicus with herniation of bowel and liver into a sac covered by fused amniotic membrane and peritoneum. **Associations** Prematurity/IUGR 30% will have CVS defects Also associated with trisomy 13 and 18, renal abnormalities and other syndromes. **Diagnosis** Usually diagnosed on the 12 and 20 week scans. Aim is to then monitor them and deliver them in a tertiary centre. **Management**: * Glad wrap * NG decompression * IV Ab and fluids * Catheter to monitor fluid output * Heater * BSL * Look for associated malformations * Delayed operation (as often unstable initially). * appendix is removed and the goal is to get it all inside the abdomen. This is not always possible and thus a 'silo reduction' may be used.

Answer 177

Gastroschisis - defect immediately to the right of the umbilicus through which bowel (and sometimes gonads) herniate. There is no covering sac and the eviscerated small and large bowel is thickened and densely matted with exudate as the result of amniotic peritonitis before birth. **Associations** Prematurity/IUGR Related to maternal drug use. **Diagnosis** Usually diagnosed on the 12 and 20 week scans. Aim is to then monitor them and deliver them in a tertiary centre. **Management:** * Glad wrap * NG decompression * IV Ab and fluids * Catheter to monitor fluid output * Heater * BSL * Look for associated malformations * Immediate operation * appendix is removed and the goal is to get it all inside the abdomen. This is not always possible and thus a 'silo reduction' may be used.

Answer 178

The 9 S's of development ## Footnote Neonate 6 weeks 3 months 6 months 9 months 12 months 18 months 3 years 5 years

Answer 179

SHIELD + vWD vWD less likely to be acute diagnosis. The story is "abnormally prolonged bleeding when prompted (like teeth brushing)" versus SHIELD which is acute onset petechiae

Answer 180

Because remember that desmopressin/DDAVP is just synthetic ADH. ADH causes aquaporins to be plugged in to the distal collecting tubule allowing for resorbtion of water which can cause dilutional hyponatremia. The challenge test it to make sure the kid doesn't get hyponatraemic.

Answer 181

* Following infectious diarrhoea, infants may have temporary lactose intolerance. * This sequela of acute gastroenteritis used to be prominent in infants * This may be due in part to early oral feeding which aids mucosal recovery. * Clinical features of sugar intolerance include: * Persistently fluid stool * Excessive flatus * Excoriation of the buttocks * Typically, these infants appear well. Breast feeding should continue unless there are persistent symptoms with buttock excoriation and failure to gain adequate weight. Formula-fed infants should be placed on a lactose-free formula for 3–4 weeks.

Answer 182

A clinical syndrome characterised by chronic diarrhoea often with undigested food in the stools of a child who is otherwise well, gaining weight and growing satisfactorily. Stools may contain mucus and are passed 3–6 times a day; they are often looser towards the end of the day. Onset is usually between 8 and 20 months of age. **Management** Reassurance and explanation. No specific drug or dietary therapy has been shown to be of value in toddler diarrhoea.

Answer 183

* Headache/neck stiffness/photophobia/Rash * Cough, coryza, sore throat * Dysuria/frequency * Vomiting/diarrheoa/abdominal pain * Joint or elsewhere pain? * Contacts * Travel * Immunisations * Intake/vomiting/urine production

Answer 184

* **Tumour** * painless * **Henoch Schonlein purpura** * purpuric rash on testicle * **Varicocele** * bag of worms, left side, teenager * **Hydrocele** * transilluminates, soft, can feel testis * **Incarcerated inguinal hernia** * **Epididymoorchitis** * fever, nausea, vomiting, urinary symptoms * **Torsion of the appendix testis (hydatid of Morgagni)** * More gradual onset. Focal tenderness at upper pole of testis; "blue dot" sign - necrotic appendix seen through scrotal skin. * **Torsion** * Sudden onset testicular pain and swelling; occasionally nausea, vomiting. Discolouration of scrotum; exquisitely tender and swollen testis, riding high. Cremasteric reflex is usually absent. * **Idiopathic scrotal oedema** * It usually affects boys between 5-9 years of age. There is insidious onset of redness and swelling of perineum or inguinal region (groin), extending to scrotum. It may be itchy but is not often painful.

Answer 185

* observation of the patients gait and resting position * natural position of the testis in the scrotum while standing * presence or absence of cremasteric reflex (this is absent in torsion) * palpation of lower abdomen, inguinal canal and cord * palpation of scrotum and contents, compare with unaffected hemiscrotum * transillumination * Is the swelling reducible?

Answer 186

**Without failure to thrive** * Infectious * Bacterial - campylobacter, salmonella * Antibiotic induced - C difficile colitis * Parasitic - Giardia lamblia * Post-infectious - secondary lactase deficiency * Toddler's diarrhoea * Lactase deficiency (lactose intolerance) * Irritable bowel syndrome - a diagnosis of exclusion in an older child/adolescent **Chronic diarrhoea with failure to thrive** * GI causes * Coeliac disease * Milk protein allergy - cow's milk, soya protein, etc. * Inflammatory bowel disease * Pancreatic insufficiency --\> Cystic fibrosis * Others * Thyrotoxicosis * Food allergy

Answer 187

The classical presentation is of a profound malabsorptive syndrome at 6-24 months of age after the introduction of wheat-containing weaning foods (e.g. steatorrhoea, vomiting in some cases). * FTT with poor appetite * Steatorrhoea * Anorexia, nausea and vomiting, abdominal pain * Abdominal distention * Buttock wasting * General irritability, apathy and fatigue - consider Fe deficiency Presentation is highly variable, and may be less acute later in childhood.

Answer 188

Most commonly functional constipation (99%) - due to lack of fibre, poor fluid intake, behavioural things (especially around toilet training), and changes in diet (esp. when changing to cow's milk). **Management** Clean out + maintain with stool softeners and adequate fibre. Address any underlying problems. This may need to be done multiple times.

Answer 189

**Basics** * Keep breastfeeding * Regular small volume flood intake * Ondansetron **Place and person** Admit the following: * Infants with moderate-severe dehydration * Diagnosis in doubt * Those who are high risk of dehydration (keep in hospital for 4-6 hours): * Infants * Infants * High frequency diarrhoea (8 per 24 hours) * High frequency vomiting (\>4 per 24 hours) **Investigations** * Mostly not required. * UECs and glucose - if severe dehydration and needs resuscitation. * If there is significant abdominal pain, or blood or mucus in the stools, or if the child appears septic * Faecal culture * Faecal leukocytes or blood * Faecal ova, cysts and parasites - when considering parastic infections * C difficile toxin (faecal) - if recent use of antibiotics **Definitive** Oral, NG or IV rehydration There is no role for antibiotics in bacterial gastroenteritis. **Long term** Early feeding as soon as rehydrated reduces stool output and aids gut recovery. Anti-diarrhoeals and maxalon are not recommended.

Answer 190

The proportion of boys with retractile foreskins is: 40% at 1 year, 90% at 4 years and 99% at 15 years. **Management of true phimosis** * 0.05% betamethasone cream should be used twice daily for 2 to 4 weeks. * Gently retract foreskin without causing any discomfort and apply a thick layer of cream to the tightest part of the foreskin. * Circumcision (if significant phimosis and steroid creams fail)

Answer 191

Smegma = white/yellow lumps are called smegma and are made up of the cells that once attached the foreskin to the head of the penis. Smegma is normal and will eventually disappear. No treatment is needed. Balanitis: severe inflammation of the glans penis +/- foreskin is often due to infection and is usually termed balanitis. Tx is Soaking in a warm bath with the foreskin retracted plus topical steroid.

Answer 192

* Enterovirus * HSV * Other herpes viruses (EBV, CMV, HHV6, VZV) * Arboviruses. * Less commonly, encephalitis can be caused by bacteria, fungi or parasites.

Answer 193

**Below 2 months of age** * Group B streptococcus * E. coli and other Gram-negative organisms * Listeria monocytogenes * Plus the below.... *Use cefotaxime and ben pen* **Above 2 months of age** * Streptococcus pneumoniae * Neisseria meningitidis * Haemophilus influenzae type B (in unimmunised children) * As a result of immunisations Hib meningitis is now rare and there has been a reduction in the incidence of pneumococcal meningitis. *Use ceftriaxone and dexamethasone*

Answer 194

25% of people with autism will have normal/high IQ 75% will have some degree of intellectual disability The child's level of ability is often uneven, with areas of strength and weakness.

Answer 195

There are three problems: 1. Protrusion of the bowel through the processus vaginalis can obstruct it's blood supply causing necrosis/peritonitis/sepsis 2. Obstruction of the processus vaginalis can put pressure on and ultimately obstruct the blood supply to the testicles and the spermatic cord, resulting in death of the testis and/or reduced fertility 3. obviously there is the risk of bowel obstruction

Answer 196

Ballooning of the foreskin during urination due to physiological phimosis. In itself is not a concern, no need for management, will resolve as phimosis resolves.

Answer 197

4 years old, topical steroid.

Answer 198

* Hernia is reducable (unless strangulated, in which case it will be extremely painful) * Hydrocele is not reducable, it would take hours for you to squeeze the fluid back up the narrow patent processus vaginalisis * Hydrocele transilluminates, hernia does not * You can get above a hydrocele but not a hernia * Hydrocele doesn’t really ever have to be fixed, but you do for cosmesis after age 2.

Answer 199

* Pre-term baby, typically in the 2nd-3rd week of life * Infant stops tolerating feeds --\> vomiting (bilious sometimes) * Abdominal distention * Rapidly becomes shocked * Other features: * Bloody stools * Pneumatosis intestinalis - gas cysts in the bowel wall; diagnostic for NEC * Diminished bowel sounds * Signs of bowel perforation - sepsis, shock, peritonitis, DIC

Answer 200

Tracheo-oesophageal fistula in 85% of cases Diagnosis is confirmed by passing a large firm catheter through the mouth and finding that it cannot be passed more than about 10cm from the gums. A plain XRAY of the torso will show gas in the bowel (if there is distal TOF).

Answer 201

Slipped upper femoral epiphysis (SUFE) is the most common hip disorder in the adolescent age group. It occurs when weakness in the proximal femoral growth plate allows displacement of the capital femoral epiphysis. The metaphysis displaces anteriorly and superiorly, leading to the slipped state. There are a number of ways that SUFE can be classified **Stable vs unstable** The patient is able to weight bear on the affected leg (stable) versus unable to weight bear even with crutches (unstable). **Acute, chronic or acute on chronic** Acute = Sudden onset of severe symptoms and inability to weight bear Chronic = Gradual onset and progression of symptoms for more than 3 weeks, without sudden exacerbation. This is the most common presentation (85% of patients with SUFE) Acute on chronic = Sudden exacerbation of symptoms due to acute displacement of a chronically slipped epiphysis **Radiographical degree of displacement** ``` Type I (0 to 33% displacement) Type II (34 to 50% displacement) Type III (\>50% displacement) ``` The likelihood of complications increases with displacement

Answer 202

A very reliable sign of chronic SUFE is detection of obligatory external rotation during flexion of the hip. As the hip is flexed on the affected side, the thigh will automatically rotate and abduct

Answer 203

**Basics** Nil by mouth Depending on severity, pain management according to WHO pain management ladder. **Place and person** Urgent orthopaedic referral **Investigate and confirm diagnosis** AP and frog lateral pelvis x-rays of both hips **Non-invasive management** The patient needs to be kept non-weight bearing **Definitive management** Immediate surgical screw fixation across the physis (within 24 hours)

Answer 204

(1 - 50%) Oligoarticular JIA (peak age 2-3, rare after age 10, F\>M) (2 - 40%) Polyarticular JIA (dual peaks at 2-5 then 10-14 years, F\>M) (3 - 10%) Less common types like systemic JIA, psoriatic and enthesitis (HLAB27+) (anyone less than 17, M=F)

Answer 205

JIA diagnosed if age 6 weeks of symptoms **Systemic arthritis** * 1 or more joints * Preceded by at least 2 weeks of fever * Signs/symptoms include one or more of: * evanescent rash, * generalised lymphadenopathy, * hepato/splenomegaly, * serositis **Oligoarthritis** * Arthritis affecting 1-4 joints _during the first 6 months_ * Persistent oligoarthritis affects up to 4 joints throughout the course of the disease **Polyarthritis** * 5 or more joints during the first 6 months * RF+ disease is more aggressive than RF- **Psoriatic arthritis** * Diagnosed if there is arthritis and psoriasis, or * arthritis plus 2 or more of: * dactylitis, * nail pitting, * onycholysis, * fam hx of psoriasis **Enthesitis-related arthritis** * Diagnosed if there is arthritis and/or enthesitis with 2 or more of: * hx of sacroiliac joint tenderness * HLA B27 antigen, * onset of arthritis in a male over 6 years of age * acute anterior uveitis * Fhx of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter’s syndrome, or acute anterior uveitis in a first-degree relative

Answer 206

**Basics** Rule out septic arthritis **Place and person** Refer to specialist paediatric rheumatology multidisciplinary team **Ix and confirm diagnosis** * FBE (anaemia, thrombocytosis, WCC) * ESR/CRP * RhF, anti-CCP * ANA (for anterior uveitis risk) * HLA B27 genotyping if suspect enthesitis * Fe studies (reactive ferritin) * Xray **Non-invasive management** Physiotherapy and occupational therapy Swimming and cycling encouraged, weight-bearing activities for those at greater risk of low bone mineral density **Definitive management** Varies depending on type of JIA, sounds very specialized. * Pain relief with NSAIDs or corticosteroid injections * Methotrexate, sulfasalazine, steroids * Biologics (anti-TNFa) increasingly used

Answer 207

* 70% of patients will have minimal or no joint disease by age 15 * Oligoarticular disease has the best prognosis, with most patients achieving remission * RF+ve patients and those with systemic arthritis have a worse prognosis, with more continuing to have active disease after several years

Answer 208

**MAS – Macrophage activation syndrome** * Life-threatening emergency * Usually occurs early in the onset of systemic JIA * high fever, hepatosplenomegaly, lymphadenopathy, pancytopenia, liver dysfunction, disseminated intravascular coagulation, hypofibrinogenemia, hyperferritinemia, and hypertriglyceridemia * **Reduced ESR** * Tx with high dose steroids and cyclosporin

Answer 209

Now. You have 6 hours before bowel becomes ischaemic and patient dies

Answer 210

* Wide-calibre feeding tube is passed and checked by X-ray to see if it reaches the stomach * AXR shows gasless abdomen --\> absent gastric bubble * If not diagnosed at birth, presents clinically with: * Persistent salivation and drooling from the mouth after birth * Infant will cough and choke when fed, and have cyanotic episodes

Answer 211

Check for others, they tend to co-occur.... **VACTERL** V - Vertebral anomalies A - Anal atresia C - Cardiovascular anomalies T - Tracheoesophageal fistula E - Esophageal atresia R - Renal (Kidney) and/or radial anomalies L - Limb defects

Answer 212

Enterocolitis Decompression washout enemas, IV ABx and intensive care

Answer 213

* Surgery too hard in neonate, use colonic washout enemas until baby is 5-6kgs * Pull-through procedure * Involves resection of distal bowel to transition point (established via contrast enema X-ray), and mobilisation of proximal bowel to allow pull-through to anal anastomosis * Complications * Prone to dehydration as large bowel is responsible for absorption of sodium, potassium and fluid Increased frequency of bowel movements due to increased fluidity of stools * Often incontinent * Technically difficult procedure due to risk of damage to continence muscles, possibility of anastomotic breakdown, stricture * Prone to Hirschsprung’s Enterocolitis even years after corrective procedure * Requires IV antibiotics every time

Answer 214

**HAI! DONT TAP SAM** ## Footnote H - HSP A - Appendicitis I - Inguinoscrotal pathology/testicular torsion D- DDH (age 1-4) O- Osteomyelitis/septic arthritis N- NAI T- Toddler’s fracture (age 1-4) T- Transient synovitis A- Arthritis/JIA P- Perthes disease (age 4-10) S- SUFE (aged \>10) A- Articular or non-articular stress fracture M- Malignancy (ALL)

Answer 215

**Basics** Bed rest is important for children with transient synovitis. Analgesia; NSAID (eg ibuprofen) +/- paracetamol **Place and person** Review with local doctor within 3 days. **Ix** None **Definitive** Analgesia **Long term/follow up** Return to hospital if febrile, unwell or getting worse Patients with symptoms for greater than 4 weeks can be referred to rheumatology clinic.

Answer 216

* General appearance, temp * Gait - running may exaggerate a limp * Neurological examination - look for ataxia, weakness * Generalised lymphadenopathy (viral infection / haematological cause) * Excessive bruising or bruising in unusual places (NAI, haematological) * Abdomen, scrotum and inguinal area (masses) * Bony tenderness * All joints * knee pain can be referred from the hip, and thigh pain can be referred from the spine * Include sacro-iliac joints and spine in joint assessment - look for pain on flexion and/or midline tenderness which may be present in discitis * Exaggerated lordosis (discitis) * Hip abduction and internal rotation are often the most restricted movements in hip pathology

Answer 217

Staphylococcus aureus but can be caused by group A b-haemolytic streptococci and Haemophilus influenzae.

Answer 218

A self-limiting disease of the femoral head comprising of necrosis, collapse, repair, and re-modelling that presents in the first decade of life and is more commonly seen in boys. **Presentation** * An occasional limp in the early phase * Knee pain * Worsening pain and limping as the disease progresses * Pain in the knee, thigh or groin on movement or when putting weight through the leg * Decreased movement * Uneven leg length – the affected leg may become shorter **Investigation** - Xray +/- MRI **Management** If the hip joint is only mildly affected, no immediate treatment may be required. If there is pain or stiffness, they will need to rest to relieve the hip joint from weight-bearing movements (no high-impact activities, like jumping or running, until the joint has recovered.) Low impact activities like swimming and gentle cycling are recommended. Ortho referral for consideration of surgical repair

Answer 219

**Acute** Gastro **Chronic** Coeliac Lactose intolerence (temporary after gastro or permanent) Pancreatic insufficiency from CF

Answer 220

It's an umbilical hernia. Common condition and \> 95% will resolve spontaneously by 2-3 years. In infants, hernia may become large and increase in size in the first 6 months. No treatment needed Refer to the OPD of General Surgery if still present after 2 years or if there is parental anxiety due to large size in infancy

Answer 221

None. This is an indirect inguinoscrotal hernia protruding through a patent processus vaginalis. If the duct was smaller it would just cause a hydrocele. There are **no tests indicated.** Ultrasound can not see anything.

Answer 222

**History** * *Microcytic anaemia -- Iron deficiency* * Intake * diet, meat, chicken, veges * Absorbtion * Screen for malabsorbtion * diarheoa, steatorheoa, poor growth, abdo pain * Family history or coeliac * Loss * blood loss * *Microcytic anaemia -- thalassaemia* * family history of thalassaemia * *Normocytic anaemia -- aplastic anaemia* * Weight loss, night sweats, predisposition to infection, bruising/petechiae * Slapped cheek picture or rash (transient erythroblastopenia of Parvovirus) * *Macrocytic anaemia -- B12 and folate* * adequate intake of folate and B12? **Exam** * Vitals * Growth charts * Pallor palmar creases/conjunctiva * Jaundice * Flow murmur? * Hepatosplenomegaly * Lymphadenopathy **Investigations** * FBE * Blood flm * Reticulocyte count * Fe studies

Answer 223

80% of preterm infants 60% of term infants

Answer 224

If \>15% of total bilirubin is conjugated or absolute conjugated bili of 15umol, that’s when to consider a hepatic/post-hepatic causes

Answer 225

1. Too early ( 2. Too high (More than 320) 3. Rising too quickly (Increasing at a rate of greater than 85 micromol per day) 4. Too late (more than 2 weeks)

Answer 226

Slera = 50 Body = 100 (progresses cephalocaudally)

Answer 227

**Before 24 hours** * Haemolysis due to blood group incompatibility * Rhesus incompatibility * ABO incompatibility * Minor blood group incompatibility * Haemolysis due to congenital infection/sepsis * Haemolysis due to RBC abnormality * RBC enzyme deficiency --\> G6PD * RBC membrane defect --\> hereditory spherocytosis

Answer 228

* Premature infants * Low albumin * Sepsis (esp meningitis) * Unconjugated bilirubin \>350 micromol/L * Asphyxia, acidosis, hypoxia and hypothermia

Answer 229

**Introduction** **Explanation (before, during and after)** * Phototherapy transforms your baby’s bilirubin into a less harmful chemical * Your baby is placed in an incubator, they are undressed and wear eye protection * Because they can dehydrate, you will need to feed your baby every 3-4 hours * This therapy will normally last 1-2 days until the bilirubin levels are rechecked are low enough to be safe **Benefits** Safe way to reduce the level of bilriubin and protect the brain **Risks** Overheating, water loss, diarrhea, rash, (theoretical) retinal damage **Alternatives** A bili-blanket is a pad that applies light directly to the skin. Can allow your baby to stay in the cot

Answer 230

**Depends whether it is conjugated or not…** *_Unconjugated_* * G6PD * Hypothyroidism * Breast mild jaundice * Sepsis *_Conjugated_* * Hepatitis due to TORCH infections * Alpha1 antitrypisin * Cholestasis due to biliary atresia or choledocal cyst

Answer 231

**Acute signs** * Lethargy/drowsiness * Poor feeding * Irritability * Temperature instability * Arching of the head, neck and back (opisthotonos) * Seizures **Long term** * Death * Permanent brain damage including: * Athetoid cerebral palsy * Deafness * Mental retardation

Answer 232

* Inflammation * Crohn’s, ulcerative colitis * Infection * C diff, giardia, enamoeba, shigella, other causes of gastro * Meckel’s * Milk * Cow’s milk protein intolerance is a common cause of rectal bleeding * Trial a non-milk (and non-soy) diet * Often resolves with age

Answer 233

Breast milk is high calories of low volumes --\> more enterohepatic circulation Don’t stop breast feeding! In fact, important to keep feeding to rehydrate and to interrupt enterohepatic circulation / ‘get the gut moving’

Answer 234

1. Determine the time course of the jaundice 1. early, high, accelerating, late 2. Ask after the underlying cause 1. Blood groups/anti-D 2. PPROM/GBS/infections 3. Family history of blood disorders 4. Instrumental delivery/cephalhaematoma 5. Dirty nappes/bowel obstruction 3. Ask about symptoms of kernicterus 1. irritability 2. decreased appetite 3. seizures 4. drowsiness 5. arching back 4. Screen for kernicterus risk factors 1. prematurity 2. low albumin 3. Sepsis, particularly meningitis 4. Asphyxia, acidosis, hypoxia and hypothermia

Answer 235

an X-linked recessive genetic condition that predisposes to hemolysis and resultant jaundice in response to a number of triggers, such as certain foods, illness, or medication. There is no treatment.

Answer 236

Frickety Fuck Bloody Mum Seems To Give Crappy Antibodies - Liver Ultrasound ## Footnote FBE/Retics/film Fractionated SBR Bloood group and hold Mothers blood type Septic screen Thyroid function tests G6PD deficiency Coombs Direct antibody test (detects IgG attached to RBCs suggesting autoimmune haemolysis) Alpha1 anti-trypsin LFTs Ultrasound

Answer 237

**Basics** Ensure adequate caloric intake / glucose (breast, formula) Ensure kept at warm temperature (incubator) **Place and person** Contact Paeds Admit to SCN / NICU **Investigate and confirm the diagnosis** *Frickety Fuck Bloody Mum Seems To Give Crap Antibodies – Liver Ultrasound* * FBE/Retics/film * Fractionated SBR * Bloood group and hold * Mothers blood type * Septic screen * Thyroid function tests * G6PD deficiency * Coombs Direct antibody test (detects IgG attached to RBCs suggesting autoimmune haemolysis) * Alpha1 anti-trypsin * LFTs * Ultrasound **Definitive management** * Treat cause if possible * Phototherapy * IVIG if immune mediated haemolysis * Exchange transfusion if anaemic * Low threshold for empirical ABx

Answer 238

* All the causes of early jaundice * Cephalhaematoma * Polycythemia (were they IUGR?) * Poor feeding * Bowel obstruction causing increased enterohepatic circulation

Answer 239

* Pallor – conjunctival, palmar crease * Fatigue, listlessness * Poor growth * Flow murmur * Air hunger (severe) * Hypotension and cardiac failure (severe) * Iron deficiency can present with poor attention span, irritability and pica (eating dirt, clay, ice etc)

Answer 240

* FBE * Blood film (e.g. spherocytosis) * Bilirubin * Reticulocytes * Direct Coombs test * G6PD assay

Answer 241

G6PD deficiency Common in Chinese, Mediterranean and African people X-linked defect in the enzyme that protects cells from oxidative damage Triggered by infections, fava beans and drugs (sulfurs, antimalarials)

Answer 242

**Hereditary Spherocytosis** * Fairly common * Usually associated with neonatal jaundice. * These RBCs have a membrane defect, that causes their funny shape and predisposed them to destruction in periods of illness. May present with jaundice, anaemia and splenomegaly throughout life (even in adult life with recurrent cholelithiasis!)

Answer 243

**MIDGUT MISHAP** Migraines - abdominal Intussception DKA Gastroenteritis UTI Testiicular Torsion Malignancy (Wilm’s, Leukaemia, Neuroblastoma) Inguinal hernia - stranulated Stuck poo (constipation) HSP Appendix + mimickers (mesenteric adenopathy / Meckel’s diverticulum) Pregnancy / ectopics / gynae

Answer 244

It's the common cold of the hip It affects kids age 3-10 It should **improve** within 3 days It should be **resolved** within 2 weeks

Answer 245

* Home care should involve complete rest and pain relief * Time off school until better * Avoidance of sport and strenuous activity until completely better * **Paracetemol** * NSAIDs should be used with caution in asthmatics * Aspirin should be avoided in children * Importantly, tell them to come back if: * High fevers * Swelling * Redness * Severe or worsening pain * Pain that does not improve after 3 days or significantly resolve in a week. As a side note the concern with aspirin in kids under 16 is Reyes Syndrome. It is a very rare condition of acute deadly noninflammatory encephalopathy and fatty degenerative liver failure.

Answer 246

**Basics** ABC Antiemesis Analagesia NBM **Place and person** Admit and refer to ortho for washout, abscess draining **Investigation** Blood – FBE, ESR, cultures Imaging – plain film Special – joint aspiration, bone scan (best test) **Definitive Management** Urgent aspiration +/- arthrotomy and washout Flucloxacillin IV **Follow up**

Answer 247

Breech presentation (10x) Female (4x) Oligiohydraminos (4x) Macrosomia (\>4kg) (2x) 1st born baby (2x) FMHx Essentially, anything that causes the foetus to be squished can cause hip development issues.

Answer 248

Infants with a suggestive clinical examination **OR** risk factors (breech birth, oligohydraminos, macrosomia and FMHx)

Answer 249

Before 4-6 weeks, the hip is largely cartilaginous --\> No test is useful **Formal investigation is often deferred until after 6 weeks** Before 4-6 months, ossification of the upper femur has not occurred --\> Ultrasonography is the best test. After 4-6 months, there is sufficient ossification to enable interpretable radiography --\> Plain film becomes the best test

Answer 250

**After 3 months, the Barlow and Ortolani tests are less useful** More meaningful signs include: * Asymmetrical hip abduction * Uneven leg length * Asymmetrical skin creases (weak sign) * High greater trochanter (dislocated) * Gait disturbance and chronic limp (older children)

Answer 251

**A radiologically subtle spiral fracture of the distal tibia that occurs in toddlers (9 months to 3 years). The periosteum remains intact and the bone is stable. These fractures occur as a result of a twisting injury.** Suspect when: * Unexpected pain or duration of limp after a relatively minor injury * Twisting one leg causes no pain, twisting the affected leg is very painful * Tender tibia on palpation **Management** * Above-knee plaster can give pain relief, but if the pain is minimal treat conservatively. * May continue to limp for 6 weeks.

Answer 252

Orthopaedics referral Normal recovery expected in 18-36 months Femoro-acetabular contact is key to normal growth Treatment depends on severity: Symptomatic --\> slings/braces --\> broomstick plaster --\> surgery

Answer 253

Notice that a line along the lateral edge of the femoral neck does not cross the epiphysis on the affected side.

Answer 254

This is a SUFE Order a plan x ray No weight bearing until definitive management Urgent orthopaedic review In situ pinning of the epiphysis to the femoral neck

Answer 255

* Pain * Warmth * Swelling * Erythema --\> usually suggests an infectious pathology! * Stiffness, worse in morning * Loss of function (reduced range of movement)

Answer 256

* Oligoarthritis * 4 or fewer joints affected _in the first 6 months of the disease_ * Broken down into normal and extended * Polyarthritis * 5 or more joints affected _in the first 6 months of the disease_ * RH - and RF + * RF + is basically just RA, bad prognosis * Systemic Arthritis * Formerly called ‘Still’s disease’ * Systemic symptoms like fever, rashes, lymphadenopathy and hepatosplenomegaly predominant the early illess * Beyond the joint * psoriatic * enthesitis (HLA B27+)

Answer 257

* **Osgood-Schlatter syndrome** is a painful knee condition that affects adolescents. * It often occurs during a growth spurt and is associated with physical activity. Symptoms include pain and local swelling in the front of one or both knees. * It is thought that the quadricepts tendon becomes tight and causes inflammation and micro-fractures in the tibial tuberosity. * Diagnosis * Clinical but get an Xray * Management * Physio referral for excercises * Rest * NSAID analgesia * Prognosis * The condition usually completely resolves within 12 months but the prominent tibial tuberosity will always be there.

Answer 258

Chronic inflammation can lead to hyperaemia of the affected joint and the local epiphysis. This can lead to abnormally increased growth and lengthening of the affected bone!

Answer 259

Oligo = no Poly = can be, particularly if RF+

Answer 260

* High spiking (\>39) fever, with resolution between spikes. May occur 1-2x daily. * 95% have a salmon pink rash on the limbs or trunk that forms during the fever spike. Rash may be urticarial. * The rash may be triggered by warm baths or skin irritation (Koebner phenomenon) * Lymphadenopathy, hepatosplenomegaly often occur * Pericarditis and pleuritis are common * There may be severe anaemia. * **Joint disease may manifest months to years after systemic presentation.**

Answer 261

\>6yo boys 10% progress to AnkSpond

Answer 262

Only a small proportion of polyarticular JIA. Associated with poor prognosis.

Answer 263

Increases risk of anterior uveitis Commonly positive in oligo and polyarticular JIA Anterior uveitis is often asymptomatic, opthal review 2-6 monthly is needed If untreated, can lead to cataract, adhesions in the anterior chamber and glaucoma. Treat with topical steroids, dilators (mydriatics) and maybe MTX

Answer 264

Jumping up and down leading to severe pain

Answer 265

Boot shaped heart Tetralogy of Fallot

Answer 266

Damage from intubation Presents with chronic fixed stridor Classically an ex-preterm infant who had long term ventilation May require endoscopic ablation to resolve

Answer 267

Palivizumab monoclonal antibody against RSV Super expensive, only for very high risk babies Give as monthly injections throughout RSV season

Answer 268

Around 9 months old - no pathological lead point - respond well to air enema Over 3 years - pathological lead point (Meckels, polyp, lymphoma, TB, HSP) - often require surgery

Answer 269

**Inheritance pattern =** autosomal recessive **Pathophysiology** = Enzyme defect leading to impaired production of glucocorticoids and mineralocorticoids by the adrenal glands. \>95% of cases are caused by a 21-hydroxylase deficiency. Reduced negative feedback to the hypothalamus and pituatary leads to increased ACTH secretion and adrenal stimulation. The adrenal gland can only respond with hyperplasia and increased sex steroid synthesis. **Forms =** simple virilising and salt-losing **Clinical presentation =** in females either form presents with ambiguous genitalia. If this is missed they may present with salt losing adrenal crisis in the following 1-2 weeks. Missing this can lead to death. In males the virilising form leads to signs of puberty as a toddler.

Answer 270

Long term mineralocorticoid and glucocorticoid replacement.

Answer 271

E. Coli. IV cephalosporin Why? In a child this young they cant present with dysuria so they present late when septic. In an older child trimethoprim would be fine?

Answer 272

If he's had one there's a 30-40% chance he'll have another one at some point. But they generally occur early in the course of a febrile illness. At day 3.5 it's less likely to occur now.

Answer 273

* Infantile spasms / West syndrome * catastrophic childhood epilepsy * Seizures start between 4 - 12 months * Urgent neuro referral * 1/20 mortality * Poor prognosis with developmental delay and long term serious epilepsy

Answer 274

**What is it?** Malabsorbtion due to severe disease or surgical removal of a significant proportion of the small intestine. **Causes** Although SBS may rarely be a congenital condition, most patients with SBS have undergone bowel resection for one of the following: * A congenital anomaly such as an omphalocele, gastroschisis, or intestinal atresia * Necrotizing enterocolitis associated with prematurity * Intestinal ischemia from malrotation and volvulus * Crohn disease (infrequent with current treatment) **Management** Usually dependent on TPN **Prognosis** Fairly catastrophic In newborn infants, the 4-year survival rate on parenteral nutrition is approximately 70%. In newborn infants with less than 10% of expected intestinal length, 5 year survival is approximately 20%

Answer 275

The inferior ureter can insert low down in the bladder leading to VU reflex and hydronephrosis

Answer 276

Tachycardia, Tachypnoea Metabolic acidosis. Lactate commonly high. Controversial: can cause VQ mismatch and **worsen** symptoms in very high doses

Answer 277

The lactate is high because of the large amounts of salbutamol the child would have received. The glucose is likely high because the kid has probably been given prednisolone. Metabolic acidosis and hypokalaemia are a sign of salbutamol toxicity. You would worry about this (stop salbutamol) but not so much the other stuff.

Answer 278

Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood and affects the small vessels. HSP is characterised by the classic tetrad of rash, abdominal pain, arthritis/arthralgia, and glomerulonephritis. **Age groups** Between the ages of 3 and 15 years. Males twice as commonly as females **Aetiology** The underlying cause of HSP remains unknown. It is an immune-mediated vasculitis, with a variety of infectious and chemical triggers having been proposed as a cause. Many cases of HSP occur after a URTI, especially streptococcal infections **Complications/other symptoms** * Nephritic syndrome and long term renal damage * Subcuteneous oedema * Intussusception * Pancreatitis * Sponteneous bowel perforation * bloody stools * Haematemesis * Painful scotal swelling

Answer 279

Uncomplicated abdominal pain often resolves spontaneously within 72 hours. However serious abdominal complications may occur including intussusception, bloody stools, haematemesis, spontaneous bowel perforation, and pancreatitis.

Answer 280

**Bedside** None **Bloods and urine** Urinalysis and urine microscopy (to quanitify haematuria) FBE UEC (elevated creatinine indicated renal Cx) Blood culture **Imaging** USS of scrotum of abdo if suspect intusscusception, testicular torsion or other **Special** Can biopsy lesions in legs if really need to

Answer 281

**Basics** Analgesia **Place and person** Any purpuric rash should be reviewed by at least a Reg Consider admission if renal complications, suspicion of abdominal complications or severe pain **Investigate and confirm diagnosis** Urinalysis and urine microscopy FBE/UEC Blood cultures **Definitive** * Document the child's blood pressure * Consider a surgical consult if abdominal features are prominent. Testicular torsion can be hard to differentiate from the pain of vasculitic testicular pain. * The use of prednisolone has *_not_* been shown to make clinically important improvements in the rate of long-term renal complications. It has been shown to reduce the duration of abdominal and joint pain and may reduce the risk of abdominal complications. It may be considered for use in patients with more than mild joint or abdominal pain. Consider prednisolone 1mg/kg while symptoms persist.

Answer 282

**Come back if...** if your child has increasing stomach pains or swelling, blood in the stools or urine, or if you are worried for any other reason. **What you need to do now....?** BP and urinalysis with GP monthly for 6 months to ensure that if a kidney problem does arise it is picked up early.

Answer 283

Foetal alcohol syndrome Most distintive features are thin upper lip and indistinct philtrum

Answer 284

**What is it?** Delayed relaxation of the pulmonary vascular bed at birth means that the baby maintains it's foetal circulation (right to left shunt across the ductus arteriosus and foramen ovale ). **What causes it?** PPHN is seen in association with many neonatal respiratory diseases including: * lung hypoplasia (eg diaphragmatic hernia) * meconium aspiration syndrome * pneumonia * surfactant deficiency _The syndrome may also be seen in the absence of triggering diseases._

Answer 285

Nitric oxide (NO)

Answer 286

No, meconium stained liquor suggests he could have mec in his oropharynx. Suction his oropharynx first before stimulating. Intubate immediately.

Answer 287

If they lose 10% or more of their birth weight you would worry.

Answer 288

15mg / kg So a 30kg 6 year old would get 450mg

Answer 289

**What is it?** An abnormal leaflet of tissue exists in the urethra, causing an obstruction to urine flow. It is a developmental anomaly, present before birth and only affects boys. **How common?** PUV is rare, occurring in 1 in 4000-6000 boys **Why is it a problem?** Urethral blockage near the bladder makes it hard for the bladder to expel urine, so the bladder has to push harder to try to empty. This increases pressure in the urinary tract. The pressure may push the urine back through the ureters to the kidney and cause the ureters, kidneys and bladder to dilate (expand).

Answer 290

**Investigations...** * UEC * Ultrasound * Micturating cystourethogram (MCU) * This is the definitive test for PUV. It is performed with a catheter placed through the urethra into the bladder. Contrast material is injected through the catheter to outline the bladder. An X-ray is taken which will show the shape of the bladder, whether there is any back flow up the ureters (reflux). Xrays are taken to identify the contrast outlining the urethra and any internal obstruction (valve). * Cystoscopy * This is both a confirmatory and therapeutic intervention. It is a surgical procedure, performed under anaesthesia. A small telescope is placed inside the urethra to identify the obstruction. Treatment of the valves can be performed at the same time. Other tests may be needed to check the function of the kidneys, the drainage, and to monitor treatment **Management** Catheter insertion then ablation via cystoscopy **Prognosis** About one third will develop kidney failure at some stage, requiring dialysis or renal transplant. About one third of patients will have a degree of renal impairment, managed with medication and diet. About one third of patients will have normal kidney function, but may have ongoing issues with urinary tract drainage, infection and bladder function.

Answer 291

An infant should gain 180g/week within the first three months of life

Answer 292

* **PATIOS** * Psychosocial * Absorption * CF * Coeliac * Milk allergy * Thyroid disease * Intake * Milk/formula supply * Sucking * GORD * Output --\> diarrheoa * Sick

Answer 293

An innocent murmur is a murmur arising from turbulent blood flow through a normal heart, for reasons which may be unclear. The 5 ‘S’s of an innocent murmur are: * innoSent * aSymptomatic * Soft * Systolic * left Sternal edge Innocent murmurs are very common and may be heard in around 50% of normal school aged children and approximately 80% of children in early infancy An important DDx from an innocent murmur is an atrial septal defect (ASD). Therefore must carefully listen for wide or fixed splitting of S2 before concluding that it is an innocent murmur. Innocent murmurs are made louder, and hence more easily detected, during an illness with a fever (as the heart works harder and this increases the turbulence in blood flow). If you hear the mumur during an illness wait until they've recovered then review it again.

Answer 294

Immature capillary bed lining the ventricles / vascular fragility of the germinal matrix. Occurs within 48 hours of birth. **Clinical presentation** * Often asymptomatic * Tense fontanelles * Floppy / limp * Seizure activity * Decerebrate posture **How is it diagnosed?** U/S between 5 and 7 days of life. Repeat at 28 days of life **Grades** Grade I - bleeding occurs just in the germinal matrix Grade II - bleeding also occurs inside the ventricles, but they are not enlarged Grade III - ventricles are enlarged by the accumulated blood Grade IV - bleeding extends into the brain tissue around the ventricles Grades I and II are most common, and often there are no further complications. Grades III and IV are the most serious and may result in long-term brain injury to the infant. After a grade III or IV IVH, blood clots may form which can block the flow of cerebrospinal fluid, leading to hydrocephalus and then cerebral palsy, epilepsy, hearing/visual problems. **Management** Grade 1 and 2 have no treatment. Grade 3 and 4 --\> VP shunt + follow up to monitor development

Answer 295

**What is it?** * Also known as hyaline membrane disease or surfactant deficiency syndrome * Due to immaturity of the type 2 pneumocytes --\> surfactant deficiency --\> alveolar collapse --\> stiff lungs --\> increased WOB --\> decreased tidal volume --\> hypoxaemia --\> respiratory acidosis * Lung surfactant is only adequate by 30-32 weeks gestation * RDS affects 50% of neonates with GA * RDS affects almost 100% of those born * Causes: * Prematurity * Perinatal asphyxia (hypoxia inhibits surfactant production) * Maternal diabetes (delays surfactant synthesis) * RDS affects about 1% of newborn infants and, along with NEC, is the leading cause of death in preterm infants **Clinical picture:** Signs of increased WOB (increased RR, nasal flaring, use of accessory muscles, grunting) Hypoxaemia, cyanosis Whether treated or not, the clinical course for the acute disease lasts about 2 to 3 days. During the first day the patient worsens and requires more support. During the second day the baby may be remarkably stable on adequate support and resolution is noted during the third day, heralded by a prompt diuresis. **Prevention** Antenatal betamethasone is used in the prevention of RDS, given to a mother expecting a premature baby (up to 24 hours before birth, should be given two courses. Unlikely to give beneficial effect if given \>2 weeks prior to birth) **Mx** * Oxygen * Nasal CPAP * Surfactant administration (can be given down ETT!) * Mechanical ventilation (severe) * Non respiratory * Attention to thermoregulation can reduce the severity of the condition * Antibiotics commence penicillin and gentamicin therapy after initial investigations. * Minimal handling * No feeding until resolved

Answer 296

Defined no respiratory effort for greater than 20 seconds. No respiratory effort for shorter periods of time may also be classified as apnoea if accompanied by cyanosis or bradycardia **Why does it happen/what are the types?** * Central apnoea:* (40%) Caused by decreased central nervous system stimuli to respiratory muscles. Both the respiratory effort and airflow cease simultaneously. (Absence of chest wall movement and airflow). * Obstructive apnoea:* (10%) Caused by pharyngeal instability / collapse, neck flexion or nasal obstruction. Absence of airflow in presence of inspiratory efforts. (Presence of chest wall movement but no airflow). * Mixed apnoea*: (50%) Has a mixed aetiology. Central apnoea is either preceded (usually) or followed by obstructed respiratory effort NB: Short episodes of apnoea are usually central whereas prolonged ones are often mixed

Answer 297

**When does it happen?** Onset is from days 2-7 of life. Apnoea beginning immediately after birth suggests another cause. **Management** * Monitoring in SCN/NICU * Positioning: * Ensure the neonate's head and neck are positioned correctly (head and neck in neutral position) to maintain a patent airway. * Tactile stimulation: * Gentle rubbing of soles of feet or chest wall is usually all that is required for episodes that are mild and intermittent. * Clear airway: * Suction mouth and nostrils. * Methyxanthines * caffeine or theophyllline * Improve diaphragm contraction and stimulates the respiratory centres * Nasal CPAP (reducing obstruction; reducing work of breathing) * Mechanical ventilation (if severe) **Prognosis** Since AOP is fundamentally a problem of the immaturity of the physiological systems of the premature infant, it is a self-limited condition that will resolve when these systems mature. It is unusual for an infant to continue to have significant problems with AOP beyond 42 weeks post-conceptual age.

Answer 298

In the fetus, the ductus venosus shunts a portion of the left umbilical vein blood flow directly to the inferior vena cava. Thus, it allows oxygenated blood from the placenta to bypass the liver. Compared to the 50% shunting of umbilical blood through the ductus venosus found in animal experiments, the degree of shunting in the human fetus under physiological conditions is considerably less, 30% at 20 weeks, which decreases to 18% at 32 weeks, suggesting a higher priority of the fetal liver than previously realized. **Clinical significance** You use the ductus venosis for venous access in resusc of the neonate.

Answer 299

If less than 7 you score it every 5minute until it gets to 7

Answer 300

The risk is much greater in preterm babies, but if you pick a random kid with CP 60% of them will have been born at term

Answer 301

HIE is the most common identifiable risk factor, but it contributes to less than a quarter of cases

Answer 302

non-independent walking non-independent feeding epilepsy blindness hearing problems Later then have learning difficulties and behavioural problems

Answer 303

Varies from 60-95% depending on the skill of the clinician. Make sure where you send them have a Certificate of Obstetric and Gynaecological Ultrasound (COGU).

Answer 304

Viral illnesses which occur in winter cause swelling of Peyer's patches in the bowel which serve as pathological lead points for intussusception

Answer 305

XO females * short stature * infertility * congenital heart defects * spatial awareness issues – problems with tasks such as maths **but otherwise normal IQ** * Amenorrhoea * Hearing problems * no breays * extra skin (‘webbing’) on the neck * puffy hands and feet * low hairline.

Answer 306

**Turner's syndrome XO females** * short stature * infertility * congenital heart defects * spatial awareness issues – problems with tasks such as maths **but otherwise normal IQ** * Amenorrhoea * Hearing problems * no breays * extra skin (‘webbing’) on the neck * puffy hands and feet * low hairline.

Answer 307

Eisenmenger's syndrome is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. Eisenmenger syndrome is a cyanotic heart defect characterized by a long-standing intracardiac shunt (caused by ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) that eventually reverses to a right-to-left shunt. This syndrome is less frequent today because of medical screening withechocardiography early in life. Prognosis is poor if early intervention doesn't happen. After pulmonary hypertension has developed, a heart–lung transplant or a lung transplant with repair of the heart is a possible option.

Answer 308

The VACTERL association (also VATER association) refers to the non-random co-occurrence of birth defects Vertebral anomalies, Anal atresia Cardiac defects Tracheoesophageal fistula Esophageal atresia Renal & Radial anomalies Limb defects. The reason it is called an association, rather than a syndrome is that while the complications are not pathogenically related they tend to occur together more frequently than expected by chance. In general, the etiology of "associations" are not defined.

Answer 309

* It's very common * Very likely will self resolve * However, it will probably get bigger when the baby starts crawling as gravity takes it's course * If it hasn't resolved by age 2 get a surgical consult to consider repair * Tell parents warning signs * can't reduce * skin changes * pain (when able to talk) * constipation/vomiting late signs

Answer 310

For kids under 6 = 6 puffs For kids over 6 = 12 puffs Give every 20mins as required, then stretch. Can discharge when stretched to 3 hourly

Answer 311

* 10-20ml/kg boluses of normal saline until not shocked * Maintenance fluids of normal saline + 5% glucose according to 4,2,1 rule * Slowly correct deficit * ignore bolus fluid volumes given * If severe shock assume 10% dehydrated * Give 10% of bodyweight in fluids * Give first half of this over the first 6 hours * Give the second half of this over the second 18 hours * You can do this more slowly if needs be

Answer 312

**Presentation** Itching around the anus (pruritus ani). The itchiness tends to be worse at night and can cause trouble sleeping. The itching is caused by an inflammatory reaction to the adult worm and eggs on the skin. **Diagnosis** Sticky tape the kid's bum at night, examine the tape for worms/eggs. **How are they spread?** Through swallowing the eggs. When someone with pinworm scratches their anus, the eggs get lodged beneath their fingernails, and are then deposited on clothing and furniture. Uninfested individuals can come in contact with pinworm eggs and ingest them unknowingly. Infested individuals continue to ingest the eggs (auto-infection). Other family members are at particular risk of pinworm infestation. **Management** * Pyrantel (Combantrin) or mebendazole (Banworm). * Very safe medications * Treat the entire household * Shower the night before taking and the morning after. Wash all sheets in very hot water and vacuum the bedroom. * Have the child wash his/her hands with warm soapy water for at least 10 seconds after going to the toilet. **Prognosis** Reinfection is very common It's a mild disease though so doens't really matter

Answer 313

4 to 14. It’s also possible for older teens and adults to have absence seizures, but it’s less likely.

Answer 314

**Either ethosuximide or sodium valproate** Ethosuximide is used commonly to treat absence seizures. It is better tolerated than sodium valproate but has the disadvnatage of not controlling tonic-clonic seizures. Treatment is usually for 2 years only in typical childhood absence epilepsy, with an expectation of seizure remission, and through puberty in juvenile absence epilepsy, with later appearance of tonic-clonic seizures being a risk.

Answer 315

* Attacks usually commence in the first or second year of life. * Crucial to the diagnosis is recognition that attacks are precipitated by either physical trauma, such as a knock or a fall, or emotional trauma such as fright, anger or frustration, the precipitants not always being significant and noticed. * Attacks usually commence with crying, but this may be brief or absent. Apnoea then occurs with cyanosis or pallor, and may then either terminate without apparent loss of consciousness, or progress with the child becoming unconscious, limp and sometimes briefly stiffening or jerking in response to the cerebral hypoxia. Incontinence may occur with such convulsive features. * Recovery is usually rapid, although some children are drowsy and lethargic after an attack with convulsive features. Attacks usually cease by the third or fourth year of life. * They are not a cause of death, epilepsy, intellectual disability or cerebral damage in later life. * The pathophysiology of breath holding attacks is not well understood,

Answer 316

**Salmon patch** Also known as nevus simplex or "angel kisses", these are a common capillary malformations that are present at birth. Eyelid spots generally fade over several months. Lesions on the glabella may take several years to resolve, and occasionally the outlines can be seen into adulthood, especially when the face is flushed.

Answer 317

Milia are keratin filled epithethial cysts which occur in up to 40% of newborns. Spontaneous exfoliation and resolution is expected within a few weeks. Parents will occasionally mistake these lesion for neonatal acne, but milia are present at birth and have no inflammatory component.

Answer 318

**Craniosynostosis** ## Footnote One or more of the fibrous sutures in an infant skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures. Sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ.

Answer 319

Anterior fontanelle closes at 18-24 months Posterior fontanelle closes at 3 months

Answer 320

**Layers** Under periosteum of skull bone but superficial to skull **Where on the head** Usually parietal bones, because this is where forceps attach **What do they look/feel like?** Margins are firm w/ a soft fluctant centre. They do not cross suture lines (help in by periosteum). **What causes them?** The usual causes of a cephalohematoma are a prolonged second stage of labor or instrumental delivery, particularly ventouse. **Management** Phototherapy may be necessary if blood accumulation is significant leading to jaundice. Rarely anaemia can develop needing blood transfusion. Do not aspirate to remove accumulated blood because of the risk of infection and abscess formation. The presence of a bleeding disorder should be considered but is rare. Skull radiography or CT scanning is also used if concomitant depressed skull fracture is a possibility. It may take weeks and months to resolve and disappear completely.

Answer 321

Caput succedaneum is a neonatal condition involving a serosanguinous, subcutaneous, extraperiosteal fluid collection with poorly defined margins caused by the pressure of the presenting part of the scalp against the dilating cervix (tourniquet effect of the cervix) during delivery. It involves bleeding below the scalp and above the periosteum. **Features on examination** Caput succedaneum presents as a scalp swelling that extends across the midline and over suture lines and is associated with head molding. **Management** Caput succedaneum does not usually cause complications and usually resolves over the first few days.

Answer 322

**What is it?** Subgaleal hemorrhage or hematoma is bleeding in the potential space between the skull periosteum and the scalp galea aponeurosis. It presents as a fluctuant boggy mass developing over the scalp (especially over the occiput) with superficial skin bruising. The swelling develops gradually 12–72 hours after delivery, although it may be noted immediately after delivery in severe cases. The hematoma spreads across the whole calvaria as its growth is insidious and may not be recognized for hours. If enough blood accumulates a visible fluid wave may be seen. **Cause** The majority (90%) result from applying a vacuum to the head at delivery (Ventouse assisted delivery). **Management** Management consists of vigilant observation over days to detect progression. **Complications** The subgaleal space is capable of holding up to 50% of a newborn baby's blood and can therefore result in acute shock and death. Fluid bolus may be required if blood loss is significant and patient becomes tachycardic. Transfusion and phototherapy may be necessary. Investigation for coagulopathy may be indicated.

Answer 323

Ankyloglossia May require surgery if severe

Answer 324

Pierre Robin Sequence microganthia and cleft palate can cause severe respiratory problems

Answer 325

Around 18 months

Answer 326

* Can't hold their head up * Feels floppy when picked up * Unusual posturing * Stiff muscles * Delayed developmental milestones * rolling * sitting * standing * walking

Answer 327

Dystonia: characterized by involuntary, sustained contractions Chorea: rapid, involuntary, jerky and fragmented motions Athetosis: slower, constantly changing, writhing or contorting movements Ataxia: loss of muscular coordination with abnormal force and rhythm and impairment of accuracy

Answer 328

**Spastic cerebral palsy:** - *Motor cortex lesion* (86%) * stiffness or tightness of muscles on movement * Monoplegia – single limb involvement * Hemiplegia: involvement of an ipsilateral upper and lower extremity * Diplegia: predominant involvement of both lower extremities, greater than upper limbs * Quadriplegia: involvement of all limbs and trunk. **Dyskinetic *-*** *Basal ganglia lesion* * involuntary, recurring and occasionally stereotyped movements with a varying muscle tone. * Dystonia: characterized by involuntary, sustained contractions * Chorea: rapid, involuntary, jerky and fragmented motions * Athetosis: slower, constantly changing, writhing or contorting movements **Ataxic** *- Cerebellar lesion* Loss of muscular coordination with abnormal force and rhythm and impairment of accuracy **Mixed**

Answer 329

1 in 500 Australian babies is diagnosed with cerebral palsy.

Answer 330

* Antenatal (70-80% of cases) * premature birth (less than 37 weeks) * low birth weight (small for gestational age) * maternal thrombophilia * placental abruption * TORCH infections * Perinatal * RH or A-B-O blood type incompatibility between mother and baby * Asphyxia during birth (contributes to less than 10% of cases) * Postnatal * hyperbilirubinaemia, * neonatal sepsis, * respiratory distress, * early-onset meningitis, * intraventricular haemorrhage, * head injuries prior to 3 years (including child abuse and shaken baby syndrome).

Answer 331

4 – 9 years Idiopathic but strong genetic component Absence seizures (can be up to 100s per day) It is rare for children to have other seizure types at first, but about 40% of children with childhood absence epilepsy develop tonic-clonic seizures as well. Ethosuximide and sodium valproate are equally effective, but ethosuximide is better tolerated. However, unlike sodium valproate, ethosuximide does not protect against associated generalised tonic-clonic seizures. Usually resolves two to five years after the seizures begin or when the child is a teenager

Answer 332

10 – 15 years Idiopathic but strong genetic component Absence seizures (less frequent that childhood absence epilepsy) 80% of children with juvenile absence epilepsy also have tonic-clonic seizures. These may begin later than the absence seizures. Some also have myoclonic seizures and absence status epilepticus. Ethosuximide and sodium valproate are equally effective, but ethosuximide is better tolerated. However, unlike sodium valproate, ethosuximide does not protect against associated generalised tonic-clonic seizures. We don’t have a lot of data. The seizures usually respond well to anti-epileptic drugs, but children with this syndrome may need to take medication to control seizures all their lives.

Answer 333

15-19 Myoclonic jerks that occur on awakening from sleep either in the morning or from a nap. They are typically described as shock-like, irregular and arrhythmic movements of both arms. Sometimes these movements are restricted only to the fingers making the patient or individual look clumsy or prone to dropping things. They ALL have tonic clonic seizures One third have absence seizures sodium valproate; however, avoidance of sleep deprivation and excessive alcohol may also be necessary to achieve complete freedom from seizures. Although full remission is unlikely and most patients require lifelong treatment, the prognosis for seizure control is very good. Most patients remain neurologically normal

Answer 334

2 - 13 Seizures usually occur during sleep and begin in the face or mouth, producing a typical glugging sound. Speech arrest is common. The partial (focal) seizure may become secondarily generalised. The EEG is typical and shows epileptiform discharges in the centrotemporal region. Carbamazepine, sodium valproate and sulthiame are the drugs of choice. Treatment is not always indicated. most children are seizure-free by early adolescence

Answer 335

Has the child had an URTI or other illness recently -- is this mesenteric adenitis!?

Answer 336

**PATIOS** * Psychosocial * Absorption * CF * Coeliac * Milk allergy * Thyroid disease * Intake * Milk/formula supply * Sucking * GORD * Output --\> diarrheoa * Sick

Answer 337

Breastfeeding every 2 to 3 hours for 15mins per breast May be less on formula. Around 60ml per time but massive variation. Around 800ml per day Start to introduce solids at 6 months

Answer 338

* Antenatal * Normal scans? * Complications during pregnancy? High blood pressure or sugars? * Blood type and anti D * GBS status * Birth * Gestational age * NVB or LUSCS * Instrumental (forceps/vacuum( * PROM? * Did baby need resus? Prolonged hospital stay? * Heel prick test done? * Prolonged jaundice?

Answer 339

Cystic fibrosis transmembrane conductance regulator (CFTR) ΔF508 Chromosome 7

Answer 340

**_Measles_** **What are the symptoms?** * High fever * Irritability * Exudative conjunctivitis * Rhinorrhea * Otitis media * Exanthem = mobilloform, blotchy and raised * Enanthem = Koplik’s spots **Who gets it?** In developed countries --\> unimmunised In developing countries --\> Malnourished and Vit A deficient **Management** Report, isolate, supportive **Complications** Complications can include otitis media, pneumonia and encephalitis. Measels specific IgM or IgG

Answer 341

6 puffs every 20 minutes x 3 if \<6 yo 12 puffs every 20 minutes x 3 if \> 6 yo

Answer 342

**FIT CAMPSITES** ## Footnote F - Frequency and severity of attacks I - Interval symptoms? T - Technique using inhaler C - Compliance with preventer A - Atopy - personal or family history M - Medications, past and current P - Pets in the house S - School missed I - ICU or hospital admissions T - Triggers E - Exercise tolerance S - Smokers in the house

Answer 343

* Testicular torsion. * Inguinal hernias through the patent processus vaginalis * Lower fertility - the body temperature in the abdomen is higher than in the scrotum. This can affect sperm production in the testes. * Risk of testicular cancer. The risk is usually less than 1 in 100. * Poor self esteem.

Answer 344

**Cryptorchidism, pronounced kript-ork-id-iz-im** **Congenital** * In premature babies, the testes may not have dropped down yet (usually happens in the eight month of pregnancy). * Some hormone and genetic disorders can cause undescended testes * Usually idiopathic **Acquired - boys who develop undescended testes after birth.** * This can happen between 1-10 years of age. * The testes are in the scrotum at birth * As the boy grows the cord attached to the testes (spermatic cord), fails to grow at the same rate. It ends up too short and pulls the testes back up into the groin.

Answer 345

**Is he walking?** If no --\> How does it get around? If yes --\> does he walk steadily? Does he run? Does he jump? **Is he talking?** If no --\> does he babble? If yes --\> how many words? What does he say? **Does he point to things to show you?** If no --\> does he wave bye bye? **How does he hold small objects?** **Does he draw?**

Answer 346

**SHIRTFRONT LAD AJ** ## Footnote Sepsis Hypoglycaemia (\<2.6) Intraventricular haemorrhage Respiratory distress syndrome/Meconium ileus syndrome Transient tachypneoa of the newborn Feeding difficulties Retinopathy of prematurity Osteopaenia of prematurity NEC Temperature instability ``` Leukomalacia (periventricular) Anaemia / Fe deficiency Developmental problems (ID, cerebral palsy) ``` Apneoa of prematurity Jaundice

Answer 347

**Foetal** Chromosomal disorders Congenital anomalies Multiple gestation TORCH infection **Placental** Uteroplacental insufficiency Abnormal implantation Placental abruption **Maternal** Chronic illness Pre-eclampsia Early or advanced age Malnutrition Substance abuse (e.g. cigarettes, alcohol, narcotics, cocaine) Medications (e.g. warfarin, anticonvulsants)

Answer 348

The aetiology of NTDs is still debated. Polygenic inheritance and environmental and teratogenic factors have been implicated. It has been demonstrated unequivocally that vitamin supplementation with folic acid reduces the incidence of recurrence in high-risk populations. Dietary factors may therefore play a major part in low-risk populations.

Answer 349

* Edwards syndrome occurs in around one in 6,000 live births, and around 80% of those affected are female. * The majority of fetuses with the syndrome die before birth. * The incidence increases as the mother's age increases. * The syndrome has a very low rate of survival, resulting from heart abnormalities, kidney malformations, and other internal organ disorders.

Answer 350

It's a herpes virus. Almost all adults are infected. In healthy people, CMV infection causes nothing more than a flu-like illness that lasts a few days. In certain people, however, including transplant patients and pregnant women, the effects can be much more serious. Symptoms may vary depending on which organ is affected, but generally a person with CMV will experience lethargy, a high temperature and a drop in white blood cell levels. For most people, a CMV infection poses no real health threat. The symptoms are generally mild and flu-like, although some may feel unwell for a few weeks, rather than a few days. Some people experience no symptoms. Antiviral medications may be used in pregnant or immunocompromised people.

Answer 351

* How low down the back is it? (the higher the more likely it is to be pathological) * Is it hairy? * Does it have any skin tags? * How deep is it?

Answer 352

Laryngomalacia

Answer 353

Stridor in infancy, abnormal voice Close monitoring, 85% chance will resolve by age 2 as cartilage hardens. Laryngomalacia becomes symptomatic after the first few months of life( 2-3 months), and the stridor may get louder over the first year, as the child moves air more vigorously. Most of the cases resolve spontaneously and less than 15% of the cases will need surgical intervention.

Answer 354

SHIELD ## Footnote **Sepsis (meningococcal)** conscious state? fever? headache, photobia or neck stiffness? **Henoch-Schonlein Purpura/HUS** haematuria or bloody diarrhea arthritis abdominal pain vomits **Event (non-accidental injury or accident)** Was the event witnessed Any other injuries Did they present straight away? **ITP** Recent URTI? **Leukaemia** pale, SOB or tired fevers, nightsweats, weight loss severe, persistent, unusual or recurrent infections lumps or bumps **DIC** seriously unwell

Answer 355

Steroids can accelerate the recovery of platelets, BUT: * most children with recover spontaneously * Side effects are common and include (name at least 3) * Gastric irritation * Behaviour change irritability and sleep disturbance * Transient diabetes and weight gain * Immunosuppression * Moon face and buffalo hump fat distribution * Growth delay * Bone weakness

Answer 356

* Annie should avoid contact sports or anything that could induce bleeding * No aspirin or ibuprofen * Annie should be supervised if you feel she cannot look after herself * Level of activity should increase as platelets rise

Answer 357

**Naevus simplex** * Aka “salmon patch” or “stork mark” * Pale pink to bright-red capillary vascular malformations * Indistinct borders that blanch and become more prominent with crying and straining * Commonly affects the forehead, glabella, upper eyelids and nape * Prevalence 20-60% * Most spontaneously disappear between age 1-3 * Thought to be related to immaturity of the vasculature * Treatment usually not required

Answer 358

**Naevus flammeus - Aka “port wine stain”** * Capillary vascular malformations * Unilateral, homogenous, red-to-violaceous macules involving the skin and sometimes mucosa. * Darkens and thickens over time * Incidence 0.3% * Can occur anywhere but many involve the face and follow the distribution of the trigeminal nerve * Largely a cosmetic concern but may be a marker for ophthalmologic or neurologic structural abnormalities * Further investigation is warranted. * Sturge-Weber syndrome = facial naevus flammeus + an ipsilateral vascular malformation of the leptomeninges and eye. * Location of the naevus helps identify newborns at risk of underlying abnormalities. * Parents of patients without V1 involvement may be reassured and informed about vascular laser as an option for treatment.

Answer 359

* **Infantile haemangioma - Aka “strawberry naevus”** * Benign vascular tumours * Occur in 5-10% of newborns. More common in premature infants * Often unapparent at birth, they appear in the first few weeks of life, proliferate rapidly for several months, and then gradually fade over several years. * Segmental haemangiomas have a linear or geographic distribution and are more likely to have associated structural abnormalities * Those that are large or occupy the airway, eyes and vital organs can cause serious or life threatening complications. Prompt specialist referral is required. * Prognosis * Resolution is considered complete in 76% of children by age 7. * 50% will have minor residual changes such as telangiectasia or wrinkling. * Treatment goals – to prevent serious complications and permanent disfigurement. * 1st line therapy = systemic prednisolone during the proliferative phase. * 2nd line therapy = propanalol * Laser therapy can sometimes also be an option

Answer 360

**Café au lait macules** * Usually noted in infancy and childhood. * Solitary CALMs are common in up to 3% of healthy infants and 25% of children. * Multiple CALMS are rare in healthy children. * Majority of children with 6 or more CALMs will eventually be diagnosed with neurofibromatosis-1 (NF-1) potentially affecting multiple organ systems. * Children with 3 or more CALMs should be monitored for other features of NF-1 * CALMs may also be seen in multiple other rare syndromes

Answer 361

``` First = **Measles** (because it is the first named virus in the MMR vaccine) Second = **Scarlett fever**, caused by Streptococcus (S,S,S) Third = **Rubella** aka ‘3 day measles’ (the third named virus in the MMR vaccine Fourth = **doesn’t exist** Fifth = **Parvovirus B19** Sixth = **Roseola** ```

Answer 362

Due to group A beta-haemolytic streptococci. An abrupt onset of fever, headache, vomiting, malaise and sore throat. Show a mobilliform exanthem and a “strawberry tongue” (bright red oral mucosa and palatal petechiae).

Answer 363

**Erythema toxicum neonatorum** * Most common rash in infants, seen in up to 50% of newborns * Begins as blotchy macular erythema * Progresses to pustular/papular rash over trunk, face & extremities * Usually appears on 2nd/3rd day of life, can appear as late as 2-3 weeks * Distinguished from infection by lack of tenderness, warmth, or induration * Cause unknown * Self-resolving, typically by 5-7 days after appearance, usually by 2 weeks age * Requires no treatment

Answer 364

**Diagnosis and forms** Impetigo is a _highly contagious_ superficial, contagious, blistering infection of the skin also called “school sores” because it most often affects children. It has 2 forms: non-bullous and bullous. Bullae are fluid-filled lesions of \>0.5 cm in diameter. **Causative organism** Impetigo in patients from affluent communities is most commonly caused by Staphylococcus aureus, and less commonly by Streptococcus pyogenes. In patients from socioeconomically disadvantaged areas, such as remote Indigenous communities in central and northern Australia, S. pyogenes is often the primary pathogen. **Management** * In non-remote community settings suspect S. aureus as the pathogen. * For localised skin sores, use: * mupirocin 2% ointment or cream * For multiple skin sores or recurrent infection, use: * di/flucloxacillin 500 mg * In remote community settings in central and northern Australia suspect S. pyogenes as the pathogen. * benzathine penicillin 900 mg

Answer 365

**Congenital talipes equinovarus (club foot)** 1 in 1000 births, congenital **Management** **Basics** Prenatal education, reassurance! **Place and person** Refer to ortho Treatment should commence in the first week of life. **Definitive management** * Ponseti technique * 4-6 weeks of toe to groin plaster casts * Changed weekly with gentle manipulation of the foot * Following casting, most cases (80%) require an Achilles tenotomy to correct residual deformity. This can be done under local anaesthetic by an orthopedic surgeon in the clinic. * Boots and bar abduction brace * Worn 23 hours a day for 3 months and then 14–16 hours, while asleep, until the child's fourth birthday. * Rarely, more aggressive surgical procedures need to be done – posterior medial release. This is more common in patients with comorbidities such as cerebral palsy. **Long term** The child is monitored every 3 to 6 months until 4 years of age. The child is then brought into the clinic every 1 to 2 years until reaching skeletal maturity.

Answer 366

1 in 1000 live births

Answer 367

RSV and influenza

Answer 368

**First line** Methylphenidate (*Ritalin* - short acting, *Concerta* - long acting) Dexamphetamine **Second line** Atomoxetine Vyvanse (lisdexamfetamine) **Response rates** 75% will respond to first drug, 95% will respond to 2nd or 3rd

Answer 369

Firstly, they need to have **inattention, hyperactivity and/or impulsivity** for more than **6 months** Two sets of DSM criteria, ADD/innattention set and ADHD/hyperactivity set **ADD/innatention set - 6 or more of ADHD DSM DX** * Absent-minded- forgetful in daily activities * Devil is in the details – overlooks details, inaccurate work * Hopeless at finding things – bit of forcing for the mnemonic * Derailable – starts things but gets sidetracked * Daily activities forgetfulness (chores, errands, appointments) * Sustained attention * Mind elsewhere when spoken to directly * Disorganised (sequential tasks, time maagement etc.) * Xternal stimuli is distracting **ADHD/hyperactivity set - 6 or more of PPQRRSSTT** * Pre-emptive conversation – doesn’t wait for appropriate turn in convos * Pushy – butts into conversations, doesn’t ask for permission, intrude into activities * Quiet play impossible * Restless – can’t stay still for long periods * Runs and climbs inappropriately * Squirms and fidgets * Seat leaving inappropriately * Taking turns is difficult * Talks excessively

Answer 370

* Is this global delay? Check gross/fine motor, social and other domains of development on the Denver chart * Is this an expressive, receptive or mixed picture? Receptive MUCH more concerning. * Is there a hearing issue? * Is this autism? Use an autism screening questionnaire to check * Is there a family history of delayed speech - highly heritable phenomenon

Answer 371

If you can afford it go the more expensive ones. There's no hard evidence but theoretically it helps brain function.

Answer 372

UTI. Urinary sepsis is a very common cause of this presentation and (annoyingly) the kids are often afebrile.

Answer 373

Swallowed blood from cracked nipples OR from the delivery "Apt test" to determine if the blood is foetal or maternal If maternal blood safe to ignore and discharge the bub as planned

Answer 374

**COMETH** said the lord, you say. C – Coeliac screening O – obesity, prevention of M – MSK\* E – eye exam and echo at birth and every 3 years T – TSH annually (high risk hypothyroidism) H – hearing test as newborn every 6 months till age 4 \*Education regarding atlanto-axial instability: avoid trampoline and high risk activities. Discuss positioning of the spine during anasthaesia/surgery. Low threshold for imaging although routine spinal xray is not recommended.

Answer 375

Meckel's diverticula

Answer 376

2mg/kg for Day 1 1mg/kg for Day 2 and 3

Answer 377

Size is calculated by the average of the anteroposterior and transverse dimensions On the first day of an infant's life, the normal ANTERIOR fontanel ranges from 0.6 cm to 3.6 cm, with a mean of 2.1 cm. At birth, the average size of the POSTERIOR fontanel is 0.5 cm

Answer 378

The most common causes of a large anterior fontanel or delayed fontanel closure are: * achondroplasia (a form of dwarism) * hypothyroidism, * Down syndrome, * increased intracranial pressure, and * rickets.

Answer 379

* Symptoms * Breathless during feeding * Excess sweating from sympathomimetic activation * Cold hands and feet * Recurrent chest infections from L-\>R shunt causing pulmonary congestion * FTT * Signs * Murmur * Hepatomegaly * Tachycardia and tachypneoa * Dependent oedema (need to press down for a while to appreciate it) * clubbing

Answer 380

* Renal * Hypo-osmotic oedema so affects areas with interstitial tissues such as face and groin * Worse in morning, gets better over the course of the day * Cardiac * Orthostatic oedema so affects dependent areas * Worse in the evening and after exertion * Typically resolves overnight

Answer 381

Different systems for describing the location of ventricular septal defects are used. Some are located in the lower portion of the septum called the muscular septum. Defects in this location are called muscular ventricular septal defects. Perimembranous ventricular septal defects (also called membranous VSD'S) are located in the membranous septum, a relatively small portion of the septum located near the heart valves.

Answer 382

VSD murmurs aren't audible on D1 of life. The reason babies have a six week check is to look for these kinds of mumurs. At day 1 there is no pressure differential between the L and R heart. This means that there is no flow between the two, which means that there is no murmur.

Answer 383

Murmur may be heard at future assessments Infection (chest infections from increased pulmonary circulation, cerebral abscess or IE) Decreased exercise tolerance Failure to thrive Other signs/symptoms of heart failure

Answer 384

Is there an ASD? In infancy this could just be a patent formen ovale that could self resolve. But ASDs typically don't self resolve. ASDs are typically less symptomatic than VSDs and are usually just picked up on clinical exam. They can however get chest infections. Do an echo to see if it's PFO or ASD. If it's still there at 1 year refer for surgical repair to avoid long term pulmonary hypertension.

Answer 385

Dilutional hyponatremia occurs because of shift of water from the intracellular to the extracellular compartment secondary to hyperglycemia and increased plasma osmolality. To "correct" sodium concentration, use the following formula: * *Corrected (i.e. actual) Na = measured Na + 0.3 (glucose - 5.5) mmol/l** i. e. 3 mmol/l of sodium to be added for every 10 mmol/l of glucose above 5.5 mmol/l. If Na is \> 160 mmol/l, discuss with senior doctor. If sodium does not rise as the glucose falls during treatment or if hyponatraemia develops, it usually indicates overzealous volume correction and insufficient electrolyte replacement. This may place the patient at risk of cerebral oedema.

Answer 386

UTI or STI

Answer 387

Presumed neonatal sepsis ABx are BenPen and Gentamycin

Answer 388

\>2 times per week is an indication for upping the preventer therapy

Answer 389

* Bilateral CXR changes, can mimic a viral picture * \>5 days of cough * Not THAT sick, but has systemic symptoms

Answer 390

* Very sick, unwell child * CXR shows abscess, empyema and/or effusion * CXR shows gas blebs

Answer 391

Generally speaking BenPen IV (or amoxycillin PO) But if suspect mycoplasma roxithromycin for 10 days If suspect Staph Fluclox plus Gentamycin If under 3 months add Gent

Answer 392

Pneumonia severe enough to cause increased WOB will be visible on CXR. Thus in this case you can essentially rule out pneumonia.

Answer 393

**SOCIAL INTERACTION DIFFICULTIES (with same age peer)** 1. Poor eye contact, or staring from unusual angle 2. Ignores when called, pervasive ignoring, not turning head to voice 3. Excessive fear of noises (vacuum cleaner); covers ears frequently 4. In his/her own world (aloof) 5. Lack of curiosity about the environment 6. Facial expressions don't fit situations 7. Inappropriate crying or laughing 8. Temper tantrums, overreacting when not getting his/her way 9. Ignores pain (bumps head accidentally without reacting) 10. Doesn't like to be touched or held (body, head) 11. Hates crowds, difficulties in restaurants and supermarkets 12. Inappropriately anxious, scared 13. Inappropriate emotional response (not reaching to be picked up) 14. Abnormal joy expression when seeing parents 15. Lack of ability to imitate **SPEECH AND LANGUAGE DELAY** 1. Loss of acquired speech 2. Produces unusual noises or infantile squeals 3. Voice louder than required 4. Frequent gibberish or jargon 5. Difficulty understanding basic things ("just can't get it") 6. Pulls parents around when wants something 7. Difficulty expressing needs or desires, using gestures 8. No spontaneous initiation of speech and communication 9. Repeats heard words, parts of words or TV commercials 10. Repetitive language (same word or phrase over and over) 11. Can't sustain conversation 12. Monotonous speech, wrong pausing 13. Speaks same to kids, adults, objects (can't differentiate) 14. Uses language inappropriately (wrong words or phrases) **ABNORMAL SYMBOLIC OR IMAGINARY PLAY** 1. Hand or finer flapping; self stimulation 2. Head banging 3. Self mutilation, inflicting pain or injury 4. Toe walking, clumsy body posture 5. Arranging toys in rows 6. Smelling, banging, licking or other inappropriate use of toys 7. Interest in toy parts, such as car wheels 8. Obsessed with objects or topics (trains, weather, numbers, dates) 9. Spinning objects, self, or fascination with spinning objects 10. Restricted interest, (watching the same video over and over) 11. Difficulty stopping repetitive "boring" activity or conversation 12. Attachment to unusual objects, (sticks, stones, strings, or hair) 13. Stubborn about rituals and routines; resists to change 14. Restricted taste by consistency, shape or form (refuses solids) 15. Savant ability, restricted skill superior to age group (reads early, memorizes books)

Answer 394

before 8 years in girls and 9 years in boys

Answer 395

Idiopathic: most common cause in females (90%) but rare in males (less than 10%) Causes in males are above our paygrade

Answer 396

gonadotrophin-releasing hormone (GnRH) agonist Continuous exposure to GnRH suppresses puberty, as it is only pulsatile exposure that triggers pubertal progression. GnRH agonists are the only effective treatment modality for GDPP. Drug is **goserelin**

Answer 397

Psychological problems and short stature Sex hormones directly stimulate the growth plate, resulting in the growth spurt. There is also an increase in GH secretion. Oestrogen, either from the ovary or aromatised from testicular testosterone, is the factor that mediates the increased GH response during puberty. The premature secretion of gonadal steroids in children results in the paradox of tall stature during childhood, due to an accelerated rate of linear growth, with eventual short stature as an adult, due to early fusion of the epiphyseal growth plates.

Answer 398

https://app.box.com/s/7rd57a3q4f8a6odrcgwtk23n9rbum73t

Answer 399

In the literature the presence of the cremaster reflex is 100% reassuring for testicular torsion. That is, the ABSENCE of a cremaster reflex is 100% sensitive for torsion so if the cremaster reflex is present you should feel reassured. In real life, you will probably send them for surgical exploration anyway.

Answer 400

**GASTRO** Gastro Appendicitis Sepsis uTi Raging HUS Obstruction of the bowel (early)

Answer 401

(On day 1 there is no pressure differential between the L and R circulations, therefore there is no flow through any defects and the only things that can cause a murmur are valvular) · Hyperdynamic flow murmur (anaemia, thyrotoxicosis) · Left outflow obstruction (coarctation, AS) · Mitral valve pathology · Tricuspid valve pathology · Right outflow obstruction

Answer 402

**‘ITCH HIS BITS’** ## Footnote I - Inguinal hernia\* T - Torsion – of testis, of testicular appendage\* C - Cancer H - Henoch-scholein purpura H - Hydrocele\* I - Infection – orchitis, epididymitis S - Spermatocele\* B - Blood (haematocele)/trauma I - Idiopathic scrotal odema\* T - Trauma S - Swollen veins (varicocele) \*affects neonates

Answer 403

* Occurs in boys of all ages but is most common in **young adolescents** * Hx of previously self-limiting episodes * Anatomical variation – bell clapper testes * If torsion is confirmed, always fix both testes because these variants may be bilateral * Undescended testes

Answer 404

Yes ## Footnote Surgery should always be performed, even if non-viability is likely because partial torsion or intermittent torsion may present similarly but have longer viability times (that is, the testis may have untangled for a bit and got some blood flow --\> it could still be alive!). The second reason is to prevent the formation of anti-sperm antibodies (theoretical risk). This is because the testes are a site of immune privelige but damage to them may allow the immune system to see them, forming antibodies which could attack the surviving testis.

Answer 405

Acute complications * Bleeding * Infections * Anaesthesia * Illioinguinal nerve damage causing allodynia Late complications * A single testis is usually sufficient to maintain normal sexual function and hormone levels. Some men will have reduced fertility.

Answer 406

An epididymal cyst filled with sperm Non-tender and transilluminates – the only other scrotal mass that does this beside hydrocele! Unlike hydrocele, the testis can be felt separately Treat only if symptomatic

Answer 407

6 months ## Footnote Studies have shown that if congenital hearing loss is identified, diagnosed and appropriate intervention begun by the age of 6 months, a child's spoken language development will progress in the same way as that of a normal hearing child. The intervention will consist of hearing aid fitting initially to allow all available sound to be delivered to the child's developing auditory system. For children with a severe-profound hearing loss, for whom hearing aids are insufficient, cochlear implantation should be considered and, where appropriate, carried out as early as is practicably possible.

Answer 408

**Diagnosis** Bacterial tracheitis * Rare but serious * Can complicate viral croup * Suspect if similar features to croup (URTI symptoms, barking cough, stridor) PLUS: * Acute temperature elevation * Acute increase in respiratory distress * Very sick (“toxic”) looking child **Management:** * Minimal examination/investigations * Secure the airway (first priority) – e.g. endotracheal intubation * Empirical IV antibiotics

Answer 409

Duchenne Muscular Dystrophy occurs in boys and presents with delayed walking that is, 18 months. Speech delay, cardiomyopathy, scoliosis and calf pseudohypertrophy are also features.

Answer 410

No Growth failure is masked by fluid retention and reduced urine volume which leads to inappropriate weight gain.

Answer 411

Noonan's syndrome (chromosome 12q24.1 defect) Congenital rubella Connective tissue disorders Williams syndrome

Answer 412

Birthweight

Answer 413

Maternal SLE

Answer 414

NAIT - neonatal alloimmune thrombocytopenia This is the equivalent of Rh disease but for platelets Thrombocytopaenia caused by transplacental passage of maternal platelet-specific alloantibody and rarely human leukocyte antigen (HLA) allo-antibodies (which are expressed by platelets) to fetuses whose platelets express the corresponding antigens.

Answer 415

**Faecal calprotectin** is a substance that is released into the intestines in excess when there is any inflammation there. Its presence can mean a person has an inflammatory bowel disease such as Crohn's disease or ulcerative colitis. These conditions can cause very similar symptoms to irritable bowel syndrome. Sensitivity is 95% to 100% at cut-off of 50 µg/g Specificities ranges from 44-93%.

Answer 416

Tension headache is still the band-like headache Migraine is across the forehead rather than unilateral like it is in adults If a child complains of point tenderness (particularly at the pterion) it is likely a MSK issue, most commonly in the knee, as the child is tensing all muscles on the affected side. Ask the child to walk, and if they aren't swinging the arm on the affected side it is almost a certain diagnosis

Answer 417

\<32 weeks

Answer 418

**Basics** Prevent preterm birth * Maternal cervical cerclage * Bed rest * Treatment of infections * Administration of tocolytic medications * Prevention of neonatal cold stress, birth asphyxia and hypovolaemia Minimal handling is important Airway management – place the unwell infant in the lateral or prone posture Oxygen through incubator or headbox **Place and person** Preterm infants should be in neonatal ICU **Investigate and confirm diagnosis** ABG, SpO2, CXR **Non-invasive management** Temperature control – hypo and hyperthermia may worsen symptoms **Definitive management** * Commence penicillin and gentamicin therapy after initial investigations (it is very difficult to differentiate between RDS and sepsis!) * Antenatal corticosteroid therapy * Given to pregnant women at risk for preterm labour * Intratracheal administration surfactant therapy * Can be administered repeatedly * Withold feeding * Enteral feeding cannot commence until the RR is less than 60, due to the risk of aspiration * Intubation and intermittent positive pressure ventilation * Indications * Cyanosis despite maximal oxygen therapy * Severe recurrent apnoea * Respiratory failure (pCO2\>70 and pH \<7.2 * Some hospitals will use CPAP first **Long term** Preterm babies will recover within days

Answer 419

Extremely premature babies need parenteral nutrition, while less premature need nasogastric until sucking-swallowing coordination developed (@ 36-37 weeks). Usually add pentavite and Fe supplementation to feed

Answer 420

Hep B vaccine given a week after birth, unless before 32 weeks (in which case give at 32 weeks) Future vaccines should not be adjusted for prematurity and given according to chronological age

Answer 421

Any fracture in a child \<18 months **Metaphyseal fractures** (more often than not related to abuse)

Answer 422

**CHILD WORD** C – Caregivers (who are all the people who look after the child) H – Hospital presentations previously? I – Injuries previously? L – Little brothers and sisters? (Relevant to their safety as well) D – Drug and alcohol use? W – Wanted? Was the child planned? ![]() – Crying, is the child often miserable? (can be cause OR effect of abuse) R – Resources, financial and social. How is the family going? D – Depression? Post natal or otherwise poor mental health? Plus a normal paeds history including antenatal, birth, developmental and growth histories.

Answer 423

* Autosomal recessive condition causing partial or total defect  of enzymes required for cortisol and aldosterone production * Occurs in 1/15,000 live births and is the most common cause of ambiguous genitalia   * The salt-wasting form of CAH is a medical emergency - babies can die of vomiting, dehydration and shock at 2-4 wks of age. **Etiology** * 21-hydroxylase deficiency causes 95% of CAH cases; this causes decreased cortisol and aldosterone with shunting toward overproduction of androgens   * Cortisol deficiency leads to elevated ACTH, which causes adrenal hyperplasia   **Clinical presentation** Depends on the specific deficiency and the cause - may present with shock and hyperkalemia if not suspected. Can be divided into: _Salt-Wasting 21-Hydroxylase Deficiency (2/3 of cases)_ Infants present with shock, FTT, low Na+, high K+, low Cl, low glucose, adrenal insufficiency, high ACTH, hyperpigmentation of genitals and areola and postnatal virilization   _Late-Onset 21-Hydroxylase Deficiency_   Allelic variant of classic 21-hydroxylase deficiency- mild enzymatic defect   Girls present with amenorrhea   Boys present with precocious puberty with early adrenarche, dehydration   _Simple Virilizing 21-Hydroxylase Deficiency_ Virilization in girls but not boys   _11-Hydroxylase Deficiency_   Sexual ambiguity in females  May have insidious onset; may present with hirsutism, occasionally hypertension   _17-Hydroxylase Deficiency_   Sexual ambiguity in males, hypertension _Investigations_   * Low Na+, high K+, low cortisol, high ACTH if both glucocorticoid and mineralocorticoid deficiency * increased serum 17-OH-progesterone (substrate for 21-hydroxylase)   * increased testosterone, DHEAS  **Treatment**   * glucocorticoid replacement to lower ACTH  * in salt-wasting type mineralocorticoids given as well  * spironolactone is used in late-onset 21-hydroxylase deficiency as anti-androgen * surgery to correct ambiguous genitalia  

Answer 424

No ultrasound in quad test, just four serum biomarkers. The quad test is free in Victoria, but less sensitive with a 5% false positive rate.

Answer 425

Risks to fetuses ## Footnote * PROM and Pre-term birth (PTB) * PTB is the biggest cause of multi fetal M&M * PTB at 24-32 weeks affects: * 1% of singletons * 5% DC twins * 10% MC twins * Cord Prolapse * Polyhydramnios, PPROM and malpresentation can lead to cord prolapse * Antepartum and post partum haemorrhage * Congenital malformation * Twin-twin transfusion syndrome (TTTS) * 10-15% of all monochorionic gestations * If untreated, severe early-onset TTTS is associated with a perinatal mortality rate of over 90%. Maternal complications * Hyperemesis gravidarum * Anemia (greater haemodilution) * Iron and folic acid supplementation recommended * Gestational Diabetes * Antepartum and post partum hemorrhage * Larger placental surface area, uterine over distension, * Higher rates of operative birth * Postnatal depression * Maternal mortality – Two fold higher in twin pregnancies.

Answer 426

* Call for senior help/call code * Don PPE/gloves * History * Gestational age * mec stained liquour * mothers GBS status * maternal fever * complications in labour? * Stimulate the baby for 30 secs, check HR and RR with stethoscope * If inadequate resps commence IPPV on **room air** whilst attaching Spo2 and telemetry * PIP (30 cmH2O for a term infant or 20 - 25 cmH2O for a preterm infant), PEEP 5cm * If no response after 30 seconds and HR * At this stage consider intubation, adrenaline and 10ml/kg normal saline through umblical artery * Titrate oxygen to time of life

Answer 427

* Most common in preschool aged children * Rapidly growing retroperitoneal tumour * Often asymptomatic mass * May rupture, bleed and cause pain (20%) * Haematuria and HTN occur in some patients. Can cause an aquired vWD. * Surgery and chemotherapy achieve 90% cure rates * Metastasize very late

Answer 428

* Most commonly arises from the adrenal glands * Variable biology: may spontaneously regress or be chemo-refratory * 75% metastatic disease at diagnosis * May infiltrate abdomen, thorax and spine. Lymph nodes and BM are common metastatic sites * May have interesting paraneoplastic effects: * Opsomyoclonus (dancing eyes, dancing feet syndrome) * Excessive catecholamines: flushing, tachycardia, HTN * VIP secretion – severe refractory diarrhea with FTT and low K+ * Diagnosis * Urinary cathecholamines are raised in 90% * Biopsy for tissue diagnosis and risk stratification * CT/MRI * BMAT * MIBG (radioisotope) scan can show areas of cathecholamine synthesis * Treatment * Surgery for low risk disease. More intensive chemo-/radio- therapy for worse disease.

Answer 429

The first trimester Fetuses don't make their own insulin until 14 weeks so hyperglycaemia before this is teratogenic

Answer 430

A - Fractured radius Metaphyseal fractures have a peak incidence during the adolescent growth spurt (girls aged 11-12 years, boys 12-13 years) due to weakening through the metaphysis with rapid growth.

Answer 431

Failure of a child's bone in compression results in a "buckle" injury, also known as a "torus" injury. These most commonly occur in the distal metaphysis, where porosity is greatest.

Answer 432

In an adult a FOOSH = radial fracture. In a kid FOOSH = suprcondylar fracture

Answer 433

Hepatoblastomaa

Answer 434

Edward’s Syndrome (Trisomy 18) The majority of fetuses with the syndrome die before birth.

Answer 435

Turner’s Syndrome 45XO Cystic hygromas are benign and can happen to anyone, but most common in context of Turner's syndrome

Answer 436

Achondroplasia A common cause of dwarfism. It occurs as a sporadic mutation in approximately 80% of cases (associated with advanced paternal age) or it may be inherited as an autosomal dominant genetic disorder.

Answer 437

**Shwachman-Diamond syndrome --\> a rare autosomal recessive disorder** After cystic fibrosis (CF), it is the second most common cause of exocrine pancreatic insufficiency in children. Recurrent infection is common due to neutropenia and pancytopaenia. Growth retardation occurs in more than 50% of cases.

Answer 438

Rare genetic condition that causes the bile ducts to be narrow, malformed, and reduced in number (bile duct paucity). This causes jaundice, hepatosplenomegaly. Also associated with Tetralogy of Fallot, skeletan and eye deformities and a characteristic facial appearance

Answer 439

An overgrowth disorder usually present at birth, characterized by an increased risk of childhood cancer and certain congenital features. Common features used to define BWS are: - macroglossia (large tongue), - macrosomia (above average birth weight and length), - midline abdominal wall defects (omphalocele/exomphalos, - umbilical hernia, diastasis recti), - ear creases or ear pits, and - neonatal hypoglycemia (low blood sugar after birth). Associated with increased risk of cancer, specifically Wilms' tumor (nephroblastoma), pancreatoblastoma and hepatoblastoma

Answer 440

A rare, neurodegenerative, inherited disease causing severe disability. A-T affects many parts of the body: * It impairs certain areas of the brain including the cerebellum, causing difficulty with movement and coordination. * It weakens the immune system, causing a predisposition to infection. * It prevents repair of broken DNA, increasing the risk of cancer. It is associated with leukaemias and lymphomas

Answer 441

Peutz Jeghers syndrome, also known as hereditary intestinal polyposis syndrome, is an autosomal dominant genetic disease characterized by the development of: 1. benign hamartomatous polyps in the gastrointestinal tract and 2. hyperpigmented macules on the lips and oral mucosa Associated with cancer! While the hamartomatous polyps themselves only have a small malignant potential, patients with the syndrome have an increased risk of developing carcinomas of the pancreas, liver, lungs, breast, ovaries, uterus, testicles and other organs.

Answer 442

Tends to occur in younger (6-36 months) children with high fevers **preceding** a **sudden** rash that begins on the trunk. The rash can last from a few hours to 3 days Essentially no worrying complications, conservative management. Can cause febrile convulsions (like any febrile illness) or otitis media.

Answer 443

Rash / exanthem – not raised (c.f. measles), often starts on the face in children then spreads down the neck, trunk and extremities Enanthem – Forchheimer’s spots Lymphadenopathy is common Constitutional symptoms: conjunctivitis, arthralgia, malaise, fever, coyza

Answer 444

1. IUGR 2. Facial dysmorphisms (see photo) 3. Global cognitive or intellectual deficits representing multiple domains of deficit 4. Social problems

Answer 445

Epidermolysis bullosa (EB) is a group of inherited connective tissue diseases that cause blisters in the skin and mucosal membranes. It is a result of a defect in anchoring between the epidermis and dermis, resulting in friction and skin fragility. Its severity ranges from mild to lethal.

Answer 446

Aplasia cutis (sometimes called ‘aplasia cutis congenita’) is a condition where a newborn child is missing skin from certain areas.

Answer 447

Acrodermatitis enteropathica is a rare genetic disorder characterised by diarrhoea, an inflammatory rash around the mouth and/or anus, and hair loss. Acrodermatitis enteropathica is due to malabsorption of zinc through the intestinal cells. It is autosomal recessive

Answer 448

Linear epidermal nevus/Lines of Blaschko Epidermal naevi are due to an overgrowth of the epidermis. Lesions are present at birth (50%) or develop during childhood (mostly in the first year of life). The abnormality arises from a defect in the ectoderm. This is the outer layer of the embryo that gives rise to epidermis and neural tissue.

Answer 449

Incontinentia pigmenti --\> an dominant X-linked disease ## Footnote **ASSOIATED WITH TEETH, EYE AND CNS PROBLEMS**

Answer 450

**Cutis marmorata telangiectatica congenita (CMTC)** is a rare, deep purple, marble- or net-like birthmark. It is mostly cosmetic, and while it is present at birth, it fades considerably over a child's first year.

Answer 451

Ash lead macules --\> associated with tuberous sclerosis. Note the hypopigmented macules, in contrast to vitiligo where there is NO pigmentation

Answer 452

Congenital melanocytic naevus proliferations of benign melanocytes (pigment cells) that are present at birth or develop shortly after birth. The risk of melanoma is mainly related to the size of the congenital melanocytic naevus. Small and medium sized congenital melanocytic naevi have a very small risk, well under 1%. Melanoma is more likely to develop in giant congenital naevi (lifetime estimates are 5-10%), particularly in lesions that lie across the spine or where there are multiple satellite lesions.

Answer 453

**Umbilical granuloma** Common condition resulting from low-grade infection of umbilical stump. Presents soon after cord separation as red, friable granulation tissue in region of umbilicus. The key to treatment is early application of silver nitrate three times daily applied directly onto granulation tissue. Protect surrounding skin with Vaseline.

Answer 454

A **sebaceous naevus** is an uncommon type of birthmark. Present at birth, it is most often found on the scalp, but sebaceous naevi may also arise on the face, neck or forehead. It consists of overgrown epidermis (upper layers of the skin), sebaceous glands, hair follicles, apocrine glands and connective tissue. It is a type of epidermal naevus and is classified as a benign hair follicle tumour. A sebaceous naevus is also called an organoid naevus because it may include components of the entire skin. Monitor and refer to derm. Probably no Tx required.

Answer 455

Congenital dermoid cyst Common and easily resectable

Answer 456

**Haemangioma --\> occurs in 10% of babies, come up in first few weeks of life.** It grows grows rapidly in the first 6 months of life and then slows down. It looks like a strawberry, or if they arise deeper in the skin they can look like a blueish lump. The vast majority regress completely by age 3, but about 10% may take until age 9-10 to regress. They can ulcerate and become secondarily infected or bleed. **Because infantile haemangiomas are likely to improve or regress completely with time, there is no need for specific treatment in most cases.** Treatment should be considered in the following circumstances. * Very large and unsightly lesions * Ulcerating haemangiomas (up to 5-25% of lesions) * Lesions that impair vision, hearing, breathing or feeding * If they fail to resolve by school age

Answer 457

**Port Wine stain over the ophthalmic and maxillary branches of trigeminal nerve** ## Footnote Associated with **Sturge-Weber syndrome** --\> a rare disorder that is present at birth. A child with this condition will have a port-wine stain birthmark (usually on the face) and may have nervous system problems. Worry about epilepsy, paralysis and ID Most cases of Sturge-Weber are not life-threatening. The patient's quality of life depends on how well the symptoms (such as seizures) can be prevented or treated. Patients will need to visit an ophthalmologist at least once a year to treat glaucoma. They also will need to see a neurologist to treat seizures and other nervous system symptoms.

Answer 458

There is no evidence that IV steroids are any more effective than PO steroids for asthma exacerbations. Their role is just for kids who can’t tolerate PO meds (like if they’re vomiting).

Answer 459

Bronchiolitis is always worst on day 3, counting day 1 as the first day of respiratory distress (not day 1 of the coryza)

Answer 460

The keyhole sign is an ultrasonograhic sign seen in boys with posterior urethral valves. It refers to the appearance of posterior urethra which is dilated, and associated thick walled distended bladder which on ultrasound may resemble a key hole.

Answer 461

* Idiopathic * Vesicoureteric reflux with or without radiographically evident structural cause * Renal tract abnormality, eg: posterior urethral valves * Poor hygiene * Neurogenic bladder --\> incomplete emptying * Voiding dysfunction/constipation

Answer 462

**For infants and children aged 6 months and older with first-time UTI that responds to treatment, routine ultrasound is not recommended unless the infant or child has atypical UTI** * Children \<6 months should have a renal ultrasound within 6 weeks of diagnosis. It should be performed during the illness if the UTI is atypical or not responding to antibiotics within 48 hours. * Older children do not require and ultrasound post first UTI, but should have a renal ultrasound for recurrent UTI. **If indicated, a DMSA scan 4–6 months following the acute infection should be used to detect renal parenchymal defects. This is a nuclear med scan that looks for a renal scar that may have been caused by the UTI.**

Answer 463

**Types/causes** Congenital hydrocephalus – is present from birth and is associated with other birth defects such as spina bifida and Dandy-Walker syndrome. Acquired hydrocephalus – can be triggered by tumours, infection or bleeding within the brain that blocks the movement or absorption of CSF. **Key signs** of hydrocephalus in a newborn --\> increasing head circumference Sundowning (downward deviation of the eyes) occurs later on in infancy, as fontanelles close its easier for ICP to increase causing neuro Sx. **Treatment** Mainstay of treatment is therapeutic LP and/or VP shunt

Answer 464

**NF1 – most common neurocutaneous syndrome** Autosomal dominant – chromosome 17, loss of functional NF1 gene, which is a tumour suppressor gene * Lots of cutaneous neurofibromas * cafe au lait spots (\>6) * Axillary freckling * Lisch nodule in iris **NF2** Autosomal dominant – chromosome 22 Bilateral acoustic neuromas (think: NF2 for 2 ears!) **Schwannomatosis** Rare (1 in 40,000) Similar to NF1/2 but get multiple schwannomas rather than neurofibromas

Answer 465

**What is it?** * Autosomal dominant condition, 66% of cases due to de novo mutations * Occurs in 1 in 5,000 - 10,000 births * Disorder is primarily caused by development of benign hamartomas affecting brain, skin, heart, kidneys, eyes, lungs and heart **How is it diagnosed?** * Diagnosis is made clinically according to criteria (SLE/DSM style) **Buzz words/presentation** * Ash leaf spots (hypopigmented) * Periungal fibromas * Shagreen patches * Facial angiofibromas * Forehead fibrous plaques **Complications** * Epilepsy * Infantile spasms are the most common presenting seizure * Intellectual disability * Autism * Risk of invasive malignancy **Management** Symptomatic tx of seizures and ID MRI every 1-3 years for monitoring for new brain lesions Surgical treatment of brain lesions only undertaken if they are causing intractable epilepsy, hydrocephalus, haemorrhage, focal neurologic deficits etc.

Answer 466

**What is it?** Friedreich's ataxia is an autosomal recessive inherited disease that causes progressive damage to the nervous system. It manifests in initial symptoms of poor coordination such as gait disturbance; it can also lead to scoliosis, heart disease and diabetes, but does not affect cognitive function. The disease progresses until a wheelchair is required for mobility. Its incidence in the general population is roughly 1 in 50,000. **Symptoms** * Worsening ataxia * Distal leg wasting * Absent lower limb reflexes (preserved extensor plantar response) * Pes cavus (high arch foot) * Dysarthria * Impairment of proprioception, vibration sense * Often optic atrophy * Associated with evolving kyphoscoliosis and cardiomyopathy that can cause **cardiorespiratory compromise and death at 40-50 years.** * **No effect on cognition**

Answer 467

**What is it?** Tay-Sachs disease (TSD) is an inherited (genetic) condition common in some Ashkenazi Jews and French-Canadians. However, it can affect people of any nationality. A mutated gene stops the body from producing an enzyme needed for proper brain functioning. This leads to paralysis and death, usually before the age of five. **Symptoms/effect** Symptoms first appear at around six months of age in a previously healthy baby. Over a short period of time, the baby stops moving and smiling, becomes paralysed and eventually dies. Most children with TSD die before their fifth birthday. There is no cure. **Buzz words** Cherry red spot Hypotonia Developmental regression in infancy Death 2-5 years

Answer 468

An autosomal recessive disease in which glucocerebroside accumulates in cells and certain organs. The disorder is characterized by: * splenomegaly * bone marrow suppression * neurological degeneration * seizures Most common in Ashkenazi Jews

Answer 469

**What is it?** Niemann-Pick Disease is one of a group of lysosomal storage diseases that affect metabolism and that are caused by genetic mutations. **Presentation/features** 3-4 months feeding difficulties FTT Hepatosplenomegaly Developmental delay Hypotonia Deterioration of hearing and vision Cherry red spot in 50%, **Prognosis** Death by 4 years.

Answer 470

**What is it?** Autosomal recessive condition --\> degeneration of the anterior horn cells Second most common neuromuscular disease after Duchenne’s. There are four types, type 1 being the worst and most common. **Key clinical features** Severe onset in the first months of life Severe hypotonia – “frog-like posture” Characteristic “bright eyes” – reflects normal intellect Areflexia Weakness worse in legs than arms **Prognosis** Usually fatal by 2 yo from respiratory failure

Answer 471

**What is it?** A genetically and clinically heterogeneous group of inherited disorders of the peripheral nervous system characterised by progressive loss of muscle tissue and touch sensation across various parts of the body. **What are the clinical features?** Symptoms of CMT usually begin in early childhood or early adulthood, but can begin earlier. Usually, the initial symptom is foot drop early in the course of the disease. This can also cause **hammer toe**, where the toes are always curled. Wasting of muscle tissue of the lower parts of the legs may give rise to a "**stork leg**" or "**inverted champagne bottle**" appearance. Loss of touch sensation in the feet, ankles and legs, as well as in the hands, wrists and arms occur with various types of the disease. **High arched feet (pes cavus)** or flat arched feet (pes planus) are classically associated with the disorder.

Answer 472

coxsackie virus A

Answer 473

The measles rash starts as spots, which then begin to blend together. The rash begins around the ears and on the forehead at the hairline. Over three days, it spreads sequentially to cover the face, neck, trunk, arms, buttocks, and legs.

Answer 474

Chickenpox is generally a benign and self limiting disease but may be associated with complications including: * bacterial superinfection (particularly group A beta haemolytic streptococcus and Staph aureus), * pneumonia, * encephalitis, * cerebellitis, * hepatitis, * arthritis and * Reye syndrome.

Answer 475

The patient is infectious from one to two days before the onset of the rash until the lesions have fully crusted over. Children must be excluded from school until fully recovered (all lesions crusted over) or at least one week after the eruption first appears.

Answer 476

In immunocompetent children no specific therapy is indicated. Symptomatic treatment consists of Calamine lotion, cool compresses, possibly oral antihistamines at night to improve sleep. Keeping the skin cool may reduce the number of lesions. Scratching increases the risk of secondary bacterial infection - cut the child's nails short at the first sign of the disease. **Avoid aspirin --\> Reyes syndrome** Aciclovir is indicated in children with impaired immunity

Answer 477

**What is it ?** Eosinophilic oesophagitis is a recently recognised panoesophagitis in children, with diagnosis based on histological evidence of at least 15 eosinophils per high power field on oesophageal biopsies obtained at gastroscopy. It is closely associated with food allergy (including IgE and non-IgE mediated) and other atopic conditions such as eczema, allergic rhinitis, asthma or family history of atopy. **Classic presentation** * Eosinophilic oesophagitis can present at any age with nonspecific gastrointestinal symptoms, including regurgitation, vomiting, food refusal or dysphagia. * A classic infancy EO presentation includes irritability, feeding refusal and failure to thrive, which often overlaps with GORD presentation. * On the other hand, food bolus impaction is the most common EO presentation in school aged children and adolescents. This presents as dysphagia, or a sensation of choking/food getting stuck (often with meat) **Management** Management of suspected EO requires referral to a gastroentologist for diagnosis by endoscopy. Treatment usually consists of a trial of food allergen elimination (ie. empirically or based on allergy testing – with referral to an allergist) or swallowed inhaled corticosteroids.

Answer 478

Height and weight – failure to thrive Abdomen - palpable faeces Spine – deep sacral cleft or tuft of hair Neurology - assessment of lower limbs. Anal area – visually examine for fissures. Internal examination not required.

Answer 479

Mainstay of Tx of are osmotic laxatives --\> osmolax. Very safe. Other option is lactulose with is less good.

Answer 480

30g per day. For premmies 15-20g/day

Answer 481

**Plagiocephaly** is the most common craniofacial problem today. **Explain to parents** It is common for a newborn baby to have an unusually shaped head. This can be caused by the position of the baby in the uterus during pregnancy, or can happen during birth. Your baby's head should go back to a normal shape within about six weeks after birth. Sometimes a baby's head does not return to a normal shape and the baby may have developed a flattened spot at the back or side of the head. This condition is known as deformational plagiocephaly. Plagiocephaly does not affect the development of a baby's brain, but if not treated it may change their physical appearance by causing uneven growth of their face and head. **Management** Many children with deformational plagiocephaly do not need any treatment at all, because the condition can improve naturally as the child grows and begins to sit up. For children where treatment is necessary, it is important to see a specialist (plastic surgeon) between four and eight months of age. This is because the greatest amount of correction will occur before 12 months of age. Where needed, treatment may involve helmets or correctional positioning.

Answer 482

Vit D deficiency and hypothyroidism

Answer 483

Eyes should be one eye length apart

Answer 484

* Under 6 weeks -\> 2 days * Under 6 months -\> 2 weeks * Under 6 years -\> 2 months

Answer 485

ASD, VSD, TOF

Answer 486

AS and coarctation

Answer 487

HOCM, ASD, pulmonary stenosis

Answer 488

Most common in 2-4 year olds Clinical presentation usually related to marrow infiltration or occasionally mass effect, certain subtypes more likely to manifest in certain ways, e.g: **BPrecursor ALL**: extensive infiltration of marrow and lymphoid organs (bone pain, bleeding, bruising, neutropenia, lymphadenopathy and hepato- splenomegaly) **Mature-B ALL:** extramedullary masses in abdomen or head/neck , CNS involvement more common than in other types **T-cell ALL:** bulky mediastinal mass,

Answer 489

**NHL more common in children** NHL is on a continuum with some types of ALL T-cell malignancies can present as either an ALL or NHL, and mediastinal mass is a common feature to both Mature B-cell types tend to present more as NHL, with masses in the abdomen, and head/neck region **HL more common in adolescent and younger adults** B symptoms are less common Often a neck / upper body mass, although can occur any Very good overall survival rates in adolescence, even with disseminated disease Ann Arbour staging system

Answer 490

Tricuspid atresia --\> Absence of tricuspid valve AND of functioning RV. Systemic venous return is therefore shunted from the RA through the FO/ASD into the LA where it mixes with pulmonary venous return, resulting in semi-oxygenated blood output to the Aorta. If a VSD is present there will be a small hypoplastic RV and blood will enter the pulmonary arteries through this. (This is pictured) **If there is no VSD, the RV will be completely hypoplastic, pulmonary atresia will be present, and the pulmonary arterial circulation will be completely dependant on shunting of blood from the aorta to the pulmonary circulation via the DA.**

Answer 491

**Presentation**: * Paroxysmal episodes of palpitations * Rapid heart rate = poor cardiac output = hypotension which often causes: * Chest pain * Fatigue * Lightheadedness * (neonates with these symptoms may just be irritable, poor feeding, drowsy etc – difficult to Dx) * If SVT is prolonged or left undiagnosed for a long period it may cause CCF **Mx:** Confirm with Holter monitor Acutely: * Assess for haemodynamic compromise, if yes: * Chemical cardioversion: adenosine, verapamil * DC cardioversion Long term: * Beta-blocker (propranolol - infants, atenolol –older) or Flecainide * If refractory to medical therapy then consider RF Ablation, relatively dangerous procedure though given most SVT relatively harmless.

Answer 492

Profound hearing loss from birth plus long QT syndrome

Answer 493

boot shaped heart

Answer 494

This is called flat feet (**pes planus**) It is very common from age 1 to 2 As long as the arch comes back when they're when on tiptoes it's totally fine Orthotics do little, no management required **When to worry?** Most children develop an arch in standing by around age six. However, about one in five children never develop an arch and that CAN indicate Achilles tendon contracture or inflammatory arthtropathy --\> should be referred.

Answer 495

This is called Bow legs (genu varum) Self resolves by age 3 Just make 100% sure it's not rickets

Answer 496

Knock knees are common in children between the ages of three and five. In most children the legs gradually straighten with growth, and are usually in a normal position by the time they are eight years old. Because bow legs and knock knees usually correct naturally, special shoes, braces or exercises are not recommended. If you are concerned about the shape of your child’s legs, it may be helpful to take a photograph of your child every six months to show your doctor. Photograph your child standing with their knees facing forward.

Answer 497

Osteogenesis imperfecta = fragile bones

Answer 498

Osteochondritis dissecans (separation of bone and cartilage from medial femoral condyle following avascular necrosis) If complete separation of articular fragment loose body formation will give symptoms of knee locking or giving way Spontaneous healing is usual unless there is an unstable fragment, and treatment revolves around rest and immobilisation for up to a year

Answer 499

Chondromalacia patellae = softening of patella articular cartilage often affects adolescent females Rx with rest and PT for quad strengthening

Answer 500

Coarctation of the Aorta Horseshoe kidney Primary amenorrheoa/ovarian failure Increased risk of autoimmune disorders

Answer 501

Fetal alcohol syndrome

Answer 502

(Mainly sporadic) deletion in chromosome 7 (7q11.23)

Answer 503

* “Elfin” facies (a.k.a. “pixie-like”) * Cognitive profile consisting of impaired cognition and development accompanied by a friendly, social personality * Love for and skill at music * hyperacusis – extreme sensitivity to sound, which may include being startled by ordinary noise levels * Supravalvular aortic and/or pulmonary stenosis * Hypertension * Short stature * Endocrine abnormalities including hypercalcemia, diabetes mellitus, and subclinical hypothyroidism * Genitourinary abnormalities * MANY MANY MANY others

Answer 504

Edwards syndrome --\> Trisomy 18 \<10% live beyond 1 year

Answer 505

Trisomy 13 Structural defects of brain and scalp defects/Cleft lip and palate 8-10% survive beyond 1 year with severe learning disabilities, most likely due to mosaicism or partial trisomy 13

Answer 506

Vertebral anomalies e.g. hemivertebrae, scoliosis, spina bifida Anal malformations Cardiac anomalies (VSD most common) (clef lip) Tracheo-oesophageal fistula with oesophageal atresia Renal anomalies Limb anomalies - radial (partial or total failure) most common , polydactyly/oligodactyly

Answer 507

Fragile X (X linked means more common in men) ## Footnote **Facial dysmorphism** Macrocephaly/High forehead/Long face Big ears Large testes (post-pubertal) **Cognitive** Intellectual disability Autistim

Answer 508

Pierre-Robin syndrome Micrognathia/retrognathia --\> resp problems at birth, feeding problems, FTT, OSA, cleft palate Prognosis is okay once the anatomical problems are corrected

Answer 509

**Mild:** Amoxycillin 15 mg/kg (500 mg) po 8H or Roxithromycin 4 mg/kg (150 mg) po 12H If considering Mycoplasma **Moderate**: Benzylpenicillin 60 mg/kg (2 g) iv 6H and Roxithromycin 4 mg/kg (150 mg) po 12H If considering Mycoplasma **Severe or pneumatocoele:** Flucloxacillin 50 mg/kg (2 g) iv 4H and Gent 7.5 (6 if \>10y) mg/kg (360 mg) iv daily and Azithromycin 15 mg/kg (500 mg) iv stat then 5mg/kg iv daily If considering Mycoplasma

Answer 510

Clarithromycin or Azithromycin

Answer 511

**Sick, young or pyelonephritis** Benzylpenicillin 60 mg/kg (2 g) iv 6H and Gent 7.5 (6 if \>10y) mg/kg (360 mg) iv daily **Otherwise** Trimethoprim 4 mg/kg (150 mg) tablets po 12H or if syrup necessary then Co-trimoxazole (8/40 mg per mL) 0.5 mL/kg (20 mL) po 12H

Answer 512

Flucloxacillin 50 mg/kg (2 g) iv 4H and Cefotaxime 50 mg/kg (2 g) iv 6H

Answer 513

Flucloxacillin 50 mg/kg (2 g) iv 4H and Gent 7.5 (6 if \>10y) mg/kg (360 mg) iv daily

Answer 514

**Bedside Tests** BGL finger prick Ketone finger prick Urine dipstick Catheter to monitor UO **Bloods & Urine** Serum glucose Serum ketones VBG / ABG UEC Septic screen - FBE, blood cultures, urine MC+S, CXR **Imaging** Special Tests – autoimmune investigations (new cases) islet cell antibodies, insulin antibodies, GAD (confirm T1DM); IgA level; antiendomyseal, anti-gliadin and tissue transglutaminase antibodies; TFTs

Answer 515

Do the following whilst monitoring BGL (finger prick and serum), serum ketones, UEC, VBG regularly **DRSABC Resuscitation** **Commence IV fluids (slowly to prevent cerebral oedema)** Normal saline to begin, add in dextrose when BGL drops below 15 Commence a strict fluid balance chart **Give insulin infusion** to correct acidosis (insulin stops ketogenesis) one to two hours after starting intravenous fluids, but not before one hour Think of correcting hyperglycaemia as a secondary benefit. You control the BGL with the addition of dextrose to the fluids, not the insulin. 50 units ‘Actrapid’ in 49.5ml normal saline (i.e. 1 unit per ml) 0.1 units/kg/hr in newly diagnosed children and in those already on insulin therapy with a blood glucose of greater than 15mmol/ **Give potassium – add it to NaCl (+/- dextrose)** Only once K Only if not anuric! **Ensure the patient is nil by mouth** **Once stable, transition to SC insulin**

Answer 516

blood glucose \> 11 mmol/l pH less than 7.3 bicarbonate

Answer 517

Cerebral oedema The pathophysiology of this is complex and controversial. No one is sure how it happens.

Answer 518

Screening for complications should begin annually, 5 years after diagnosis ## Footnote **BP** **Urinary: albumin ratio** **Fundoscopy with dilated pupils by opthalmologist**

Answer 519

Congenital hypogonadotropic hypogonadism Delayed puberty/infertility and often anosmia

Answer 520

the onset of breast development

Answer 521

Complication of GORD in children causing neurological, seizure like episodes The classical symptoms of the syndrome are spasmodic torticollis and dystonia. Nodding and rotation of the head, neck extension, gurgling, writhing movements of the limbs, and severe hypotonia have also been noted. Diagnosis is made on the basis of the association of gastro-oesophageal reflux with the characteristic movement disorder. Neurological examination is usually normal. Management involves treating the GORD

Answer 522

Infants born with congenital hypothyroidism may show no effects, or may display mild effects that often go unrecognized as a problem: excessive sleeping, reduced interest in nursing, poor muscle tone, low or hoarse cry, infrequent bowel movements, exaggerated jaundice, and low body temperature. If fetal deficiency was severe because of complete absence (athyreosis) of the gland, physical features may include a larger anterior fontanel, persistence of a posterior fontanel, an umbilical hernia, and a large tongue (macroglossia). If untreated for several months after birth, severe congenital hypothyroidism can lead togrowth failure and permanent intellectual disability.