Paeds Flashcards

Question

Management of acute asthma: Hospital

Answer 1

``` Assess severity Moderate - 2-10 puffs beta2 agonist via spacer (start at 2 puffs/2 min, increase dose by 2 puffs each time up to 10 puffs according to response) - 30-40mg oral prednisolone - reassess within 1 hour ``` Severe - O2 to get SpO2 up to 94-98% - B2 agonist 10 puffs via spacer OR nebulised salbutamol 2.5-5mg OR nebulised terbutoline 5-10mg - Oral prednisolone 30-40mg OR IV hydrocortisone 4mg/kg if vomiting - If poor response, nebulised ipatropium 0.25mg - Repeat B2 agonist AND ipatropium every 20-30 mins according to response Life threatening - Nebulised b2 agonist (sallbutamol 5mg OR terbitaline 10mg) AND nebulised ipatropium 0.25mg - Oral prednisolone 30-40mg OR IV hydrocortisone 4mg/kg if vomiting - Repeat bronchodilators every 20-30 mins - Contact senior (PICU or paediatrician) ``` Assess response with HR, RR, SpO2, PEF If responding: - Continue bronchodilators 1-4hrs PRN - Ensure stable on 4 hourly Rx - Oral pred 30-40mg for up to 3 days ``` When discharged - Ensure stable on 4 hourly Rx - Review inhaler technique - Review need for regular Rx and inhaled steroids - Provide written action plan for future attacks - Arrange follow-up according to local policy

Answer 2

Assess severity Moderate - 2-10 puffs b2 agonist via spacer (2 puffs, increase by 2 puffs every 2 mins up to max 10 puffs according to response) - Consider 30-40mg oral prednisolone - If poor response, arrange admission to hospital Severe - 10 puffs b2 agonist via spacer OR nebulised salbutamol 2.5-5mg OR nebulised terbutaline 5-10mg - Oral prednisolone 30-40mg - Assess response after 15 mins of b2 agonist. If poor response, repeat b2 agonist and arrange admission Life threatening - Nebulisers (salbutamol 5mg or terbutaline 10mg AND ipatropium 0.25mg) - Oral prednisolone 30-40mg OR IV hydrocortisone 100mg - Repeat b2 agonist w/O2 driven nebuliser - Arrange immediate hospital admission Good response - Continue B2 agonist via spacer or nebs up to every 4 hours - If symptoms not controlled, repeat b2 agonist and refer to hospital - 3 days oral prednisolone 30-40mg - Arrange follow-up appointment Poor response - Stay with patient until ambulance arrives - Send written referral info and assessment - O2 driven nebulised b2 agonist in abmulance Reduce threshold for admission if: - Late evening or night - Recent hospital admission or previous severe attack - Concern about social circumstances and ability to cope at home

Answer 3

1) SABA 2) SABA + low dose ICS (8 week trial) - If symptoms don't resolve, consider alternative diagnosis - If symptoms resolve and come back within 4 weeks of trial ending, start low dose ICS - If symptoms resolve and come back >4 weeks after, then repeat the trial 3) SABA + low dose ICS + LTRA 4) remove LTRA, refer for expert opinion ``` SABA = Short Acting B2 Agonist ICS = Inhaled CorticoSteroid LTRA = LeukoTriene Receptor Antagonist ```

Answer 4

1) SABA 2) SABA + low dose ICS 3) SABA + low dose ICS + LTRA 4) SABA + low dose ICS + LABA 5) SABA + MART (contains low dose ICS) 6) SABA + MART with moderate dose ICS 7) SABA + Either: - MART with high dose ICS - Another drug e.g. Theophylline - Referral to expert ``` SABA = Short Acting B2 Agonist (salbutamol) ICS = Inhaled CorticoSteroid (Beclometasone) LTRA = LeukoTriene Receptor Antagonist (montelukast) LABA = Long Acting B2 Agonist (Salmeterol) MART = MAintenance Reliever Therapy (Budenoside with formoterol) ```

Answer 5

``` Clinical Hx - Polydipsia, polyuria - Weight loss - Abdominal pain, vomiting - Weakness, confusion Signs - Assess hydration - Ketone smell on breath - Kussmaul breathing - Confused, lethargic, drowsy Biochemistry - Glucose >11, pH <7,3, ketones in blood or urine - Take blood for urea and electrolytes - Other tests if indicated CONFIRM DIAGNOSIS OF DKA, Call senior staff Assess hydration 1) Shock: Reduced peripheral pulse volume, reduced consciousness/coma - Airway +/- NG tube - Breathing: Aim for 100% SpO2 - Circulation: 10ml/kg 0.9% saline, repeat until haemodynamically stable, max 3 doses - This should then bring patient out of shock to just dehydrated (3) ``` 2) Barely dehydrated (<5%), clinically well, tolerating oral fluid - s/c insulin and oral fluids - If no improvement, go to (3), otherwise keep monitoring 3) Dehydrated >5%, clinically acidotic, vomiting - IV therapy: - 0.9% saline, aim to correct fluids over 48hrs - add 20mmol KCl per 500ml - 0.1U/kg/hr insulin infusion after 1hr fluids 4) Observations - Hourly blood glucose, ketones, fluid balance - Electrolytes 2hrs after IV begins, then every 4 hours - aim for glucose <14mmol/L New neuro signs (if absent, move on to (5) - E.g. headache, bradycardia, increased ICP, irritable, decreased consciousness - Exclude hypoglycaemia. Could be Cerebral Oedema - 5ml/kg 2.7% saline or 0.5-1g/kg mannitol - Call senior help, consider PICU - Reduce IV fluids by half - CT scan when stable Not improving (If improving, move on to (5) - Monitor fluid balance - Give IV therapy as per (3) - If acidosis continues, may need more fluid resus - Check insulin dose is correct 5) Glucose <14mmol/L - Add 5% glucose to 0.9% saline - Change to 5% glucose 0.45% saline after 12hrs - Monitor as above (4) - Reduce insulin to 0.05U/kg/hr if pH>7.3 6) Resolution of DKA - Clinically well, drinking and tolerating food - Blood ketones <1.0mmol or pH normal - Urine ketones may be high for a while 7) Insulin - Start sub cut, then stop IV 1 hr later

Answer 6

1) Airway, High flow O2, Don't Ever Forget Glucose 2) [5 mins after convulsions start] Vascular access? - Yes? --> IV/IO lorazepam 0.1mg/kg - No? --> Buccal midazolam 0.5mg/kg or rectal diazepam 0.5mg/kg 3) [10 mins later, still seizing] - IV/IO lorazepam 0.1mg/kg - Prepare phenytoin 4) [10 mins after (3)} Confirm it's an epileptic fit - Get senior help (Anaethetics/PICU) - IV phenytoin 20mg/kg over 20 mins - [5 mins later] If already on phenytoin, give IV/IO phenobarbitol 20mg/kg 5) [20 mins after (4)} Anaesthetics MUST be present - RSI with thiopentane

Answer 7

``` COLOUR Green - Normal; skin, lips and tongue Amber - Pallor reported by parent/carer Red - Pale, mottled, ashen or blue ACTIVITY Green - Responsive to social cues, awake or quickly aroused - Content/smiling, strong normal cry/not crying Amber - Not normal response to social cues - Wakes only upon prolonged arousal - Decreased activity/no smile Red - Unresponsive to coal cues. Looks unwell to HCP - Unable to awake or if roused, only awake for short time - Weak, high pitched or continuous cry RESPIRATORY Green - No amber or red signs Amber - Nasal flairing - Crackles on auscultation - RR>50 (<12m) or >40 if >12m. SpO2<95% on air Red - Grunting - Moderate or severe chest indrawing - RR>60 HYDRATION Green - Moist mucous membranes, normal skin and eyes Amber - Dry mucous membranes, cap refill >3s - Reduced urine output - Poor feeding in infants Red - Reduced skin turgor OTHER Green - No amber or red Amber - Fever > 5 days - New lump >2cm -Swelling of limb or joint - Not wiehgtbearing or using one limb RED - T>38 (0-3 months) or T>39 (3-6 months) - Bile stained vomiting - Non-blanching rash - Bulging fontanelle - Neck stiffness - Focal neurological signs - Focal seizure - Status epilepticus ```

Answer 8

``` Low risk (Green) - Test urine for UTI - Look for specific signs/symptoms f pneumonia - No need for bloods or CXR Intermediate risk (Amber) - Test urine for UTI - Routine bloods (FBC, CRP, Blood cultures) - Consider LP if <1YO - CXR if T>39 and WCC>20 High risk (Red) - Test urine for UTI - Routine bloods (FBC, CRP, Cultures) - LP in any age -CXR reguardless of temps or WCC - Serum electrolytes - Blood gases ```

Answer 9

Occur usually between 6 months old and 6 years old, commonest @ 18 months Simple vs Complex: Simple - <15 mins, tonic clonic, occurs once, <1YO Complex - >15mins, focal.partial seizure, occurs multiple times in one illness, not fully recovered after 1 hour Hx is important - Low Ca, Low glucose, UTI, pneumonia - Breath holding spells or reflex anoxic seizures General advice - Scary, but don't harm the child - don't case epilepsy (2% risk after one simple seizure vs 1% general population risk) - Approx 1/3 recover, antipyretics don't help - Admit if: <18mo, prolonged, concerns about coping at home or first time and unable to review in community Parental advice - Caused by rapid increase in temperature, not the peak temperature itself - Avoid cooling with cold water. minimise blankets and give cold drinks and early antipyretics - May be post-ictal after. [Give leaflets] - If recurrent or prolonged (>5min) seizures are a problem, give rectal diazepam or buccal midazolam to take home

Answer 10

Types of abuse - Physical violence/NAI - Neglect - Emotionalabuse - Sexual abuse - Factitious disorder (Munchausen's by proxy) Presentations where you'd consider abuse - Delay in presenting to healthcare - Differing/inconsistent stories - Poor emotional attachment to caregivers - Poor hygiene - Poor attendance or behaviour at school - Delay in speech and language development - Inappropriate reaction to situation from caregivers - Injury inappropriate to child's stage of development - Highly sexualised behaviour - Failure to thrive - Recurrent injury - Hx incompatible with bruising Bruising/injuries compatible with NAI - Bruising in pattern of fingerprints - Bruising behind ears - Bite marks/cigarette burns - Torn frenulum (force feeding) - Tears, bleeding or bruising around genitals, thighs, buttocks - Metaphyseal fractures or posterior rib fractures - Subdural or retinal haemorrhage (shaking)

Answer 11

- Follow local trust guidelines, are they already known to social servcies? - Be prompt in escalating to hospital or child protection lead - Full Hx and examination. Photograph any injuries - Decide if child needs immediate protection (may need legal enforcement if parents refuse hospitalisation) - Evaluation by social workers needed Convene child protection meeting: - Should they be put on child protection register - Should there be an application to court to protect the child - Follow-up

Answer 12

DEFINED as growth failure PLUS failure of emotional and developmental progress - usually weight gain faills first, followed by height and head circumference - Neglect can be a possible cause, need to involve social services and some children get removed Other causes of weight faltering and poor growth include: - CF (associated with recurrent chest infections & diarrhoea. do genetic analysis and treat appropriately - IUGR: low birthweight, if length and head size are also small, catch-up is less likely - Immunodeficiency (rare): e.g. HIV, SCID. Recurrent infections, ?parental Hx of infection - Genetic syndromes commonly cause low birthweight and dysmorphic features - Chronic illness - usually apparent - GORD: pain from oesophagitis, vomiting/cosseting, apnoea. Common in babues w/neurodevelopmental problems - Coeliac disease: Weight falls off when weaning (gluten introduced), diarrhoea, irritable - Endocrine dysfunction: Hypothyroid - Developmental delay. GH deficiency - very rare History - Nutrition - ROS - Developmental Hx - PMHx (low birth weight) - Family Hx of weight faltering Examination - General obs, Interaction with mum. Growth chart. Physical exam TESTS - FBC + Ferritin is useful, Iron deficiency is common - Other tests based on clinical findings

Answer 13

Refers to lack of quality of nutrition (e.g. specific things missing) rather than lack of food altogether (starvation) - Fe, Zn, Vit A, Vit D and Iodine are most common - Fe: Fatigue, anaemia, decreased cognitive function, headaches, glossitis, nail changes - Zn: anaemia, dwarfism, hepatosplenomegaly, hyperpigmentation and hypogonadism - Vit A: Night blindness, Xerophthalmia, poor growth, hair changes - Vit D: Poor growth, hypocalcaemia, Rickett's - Iodine: Goitre, developmental delay and mental retardation Rx: - Balanced diet - supplementation if needed - advice and education - More sinlight is important for Vit D deficiency

Answer 14

Steady growth on low centile - IUGR/low birth weight - Turner's XO (No pubertal signs, normal bone age) - Maturational delay (Delayed puberty, FHx, delayed bone age) - Constitutional (familial) short stature Falling through centiles - Chronic illness (often apparent, Crohn's and CKD can be more occult) - Hypothyroid (Gotre, low T4 and high TSH, decreased bone age) - sychosocial: Neglected appearance, behavioural problems. Growth tends to catch up when removed from home - Cushing's (rare), usually due to prescribed steroids - GH deficiency (rare), may be with other hormone deficiencies (e.g. hypopituitarism)

Answer 15

Puberty begins with testicular enlargement in boys and breast development in girls Precocious puberty is defined as before 8.5YO in girls and 9.5YO in boys - Can cause early growth plate seizure and hort stature - Can be central precocious puberty (CPP): Gonadotrophin dependent, early maturation of entire HPG axis - Precocious pseudopuberty: Increased sex steroids, not gonadotrophin dependent - Rx: GnRH analogues

Answer 16

Delayed puberty (defined as after 13 in girls and 14 in boys - Constitutional - High level of activity if female (e.g. athlete) - Chronic disease (Crohn's, CKD) - Malnutrition - Hypothalamic (Prader-Willi) or Puberty (hypopituitarism) or Gonadal (Turner's, Klinefelters)

Answer 17

Diagnosis - Usually discovered through 20 week anomily scan - If not, present as either cyanosis OR not cyanotic but SoB, Murmur, Shock or hypoxia Cyanotic causes - Truncus arteriosus - Transposition of great arteries (cyanotic, no murmur) - Tricuspid atresia - Tetralogy of fallot - Total anomalous pulmonary venous drainage - Severe pulmonary stenosis - Pulmonary atresia Non Cyanotic causes - SoB: VSD, PDA< Arterio-venous malformations - Murmur: Valve stenosis, VSD, PDA - Shock: Coarctation of aorta, Critical aortic stenosis, Interruption to aortic arch, hypoplastic left heart sndrome (single ventricle) Diagnose with echocardiogram. Refer to cardiology - Cardiac catheter with balloon can open stenosed valve - Most of these will need surgical correction at some point

Answer 18

Develops 1-5 weeks after bacterial throat infection (sore throat), with group A strep - Shorter incubation in recurrence Jones criteria (diagnosis) - Evidence of recent strep infection (Hx of scarlet fever, +ve throat swab, rising ASOT titre) + 2 major criteria or 1 major and 2 minor Major - Arthritis (Flitting or mogratory, affecting large joints, hot and red, reduced range of motion, fluctuates 1-2 joints @ a time) - Carditis (high temperature than expected, echo shows characteristic signs, aortic regurg murmur) - Chorea - Subcutaneous noduels - Erythema marginatum (or anulare): Pale-red macules or papuls, 1-3cm, trunk and proximal limbs Minor - Fever/raised ESR and CRP - Arthralgia (unless arthritis was the major) - Proonged PR (not if carditis is major) Management - Bed rest until inflammatory markers are normal, even if pt. feels well - Penicillin to eradicate the group A strep (erythromycin if allergic), WHO recommends IM - Aspirin, usually relieves arthritis - HF needs diuretics, ACEi and digoxin - Diazepam if needed for chorea

Answer 19

Absence of ganglion cells in bowel wall nerve plexus - Presents in newborn with delayed passage of meconium (>48hr), abdo distension (if only small part of bowel affected) - Can present later as constipation with failure to thrive - Many genes identified to be associated. also associated with Down's - Dx: Barium enema and rectal biopsy - Rx: Surgical resection of abnormal section of bowel

Answer 20

Congenital abnormality where contents of abdomen herniate into the umbilical cord (Exomphalos) or to the right of the umbilical cord (Gastroschisis) - Contents are all within a membrane (Exomphalos) or without (Gastroschisis) - Usually presents as a rise in AFP (higher in gastroschisis) in 2nd trimester, or an abnormality on USS - Also worth karyotyping as both are associated with other chomosomal abnormalities - USS can define the specific pattern RX: - Surgery as soon as suitably possible to put the contents back and repair the hernia - For Gastrpschisis, planned pre-term delivery may be beneficial

Answer 21

Occurs in first 2-8 weeks of life, most common indication for surgery - Increasingly intense projectile vomiting immediately after feeds - Vomit not bile staines, infant still hungry - May have Hx of constipation - Irritable (hungry) and dehydrated - Palpation after feed may show mobile, hard mass to the right of the epigastrium. Peristaltic waves seen over stomach - USS: thickened, elongated peristaltic muscle - Bloods: Decreased Na, Cl, K. Alkalosis (all secondary to vomiting Rx: Rehydrate carefully before surgery. Splitting pyloric muscle without cutting the mucosa (Ramstedt's pyloromyotomy)

Answer 22

Telescoping of one part of bowel into another, usually ileum into caecum - Commonest in 3-24 months old, often follows viral infection - Episodic screaming, pallor. Well between episodes - Red current jelly stool in 75% (late sign) - Sausage shaped mass on abdominal exam - Abdo X ray shows rounded edge of intussuseption against gas filled ilium, signs of proximal bowel obstruction - USS to confirm (doughnut sign) - Rx: Often reduced w/air or barium enema. Failing this (or if peritonitis), laparotomy is needed

Answer 23

Can lead to volvulus - Severe abdo pain and bilous vomiting, can be seen on AXR - Urgent surgery needed to untwise and treat underling malrotation as bowel may otherwise infarct

Answer 24

Can get physiological jaundice in 50% of newborns, due to high RBC mass and delayed hepatic conjugation - All jaundice <24hrs or >14d needs investigating - Rx: Phototherapy or exchange transfusion if it gets bad enough Non physiological - Due to excess haemolysis or impaired conjugation (unconjugated) - Or obstruction of bile drainage (conjugated) Haemolytic disease of newborn - Maternal IgG crosses placenta and reacts with foetal RBCs (usually due to ABO incompatibility or RhD) - RhD -ve women now routinely given Anti-D at 28 and 32 weeks - Rx: Deliver baby before v. extensive haemolysis, then exchange transfusion (remove the IgG) and phototherapy (remove bilirubin) Kernicterus - Free bilirubin crosses the blood brain barrier and gets to basal ganglia - Acute encephalopathy (irritable, high pitched cry or coma) - Can lead to deafness or athetioid CP Breast milk jaundice - Dx of exclusion in well, breast fed babies with normal coloured urine and stool - Can prsist from day 4-7 for 3 months - Probably due to liver enzyme inhibition by breast milk Biliary atresia - Rare absense of intra or extrahepatic bile ducts --> conjugated bilirubinaemia and clay coloured stools - May develop liver failure and die if undiagnosed - If diagnosed within 6 weeks, hepatoportoenterostomy can achieve biliary drainage - if any baby is still jaundiced after 2 weks, check split bilirubin - If >20% conjugated, REFER URGENTLY TO PAEDS HEPATOLOGIST

Answer 25

Caucasian population: 1 in 25 carriers, 1 in 2500 affected - Autosomal recessive, deficit in cell membrane Cl channel --> excessively thick mucus in many systems - Also causes very salty sweat (>60mmol/L), used as diagnostic test (small electrical current across skin, collect sweat and measure osmolality) - DeltaF508 mutation on CFTR gene can also be looked for - Can also be diagnosed on newborn blood spot screening (IRT or Immune Reactive Tripsinogen) Presentation - 1 in 10 get meconium ileus - Otheers get failure to thrive and malabsorption in infancy or recurrent chest infections - Atypical cases can present much later Chest infections - Pseudomonas aeruginosa or Burkholderia cepacia are common, can be chronic. Causes rapid decrease in lung function - Must avoid transmission to other CF patients - Rx: Bronchodilators, antibiotics (oral, nebs or IV), steroids to decrease lung inflammation, Nebulised DNAse to break down mucus - Prevention: physio to help clear airway regularly, immunisation against pneumococcus and influenza Malabsorption - Poor absorption of fatty foods due to impact on pancreas. Vitamins A, D, E and K (fat soluble) are also low - Rx: Pancreatic enzymes with each meal, high kcal, high fat diet. Fat soluble vitamin supplements Diabetes Mellitus - 25% get impaired glucose tolerance. Better glucose control correlates with better lung function Infertility - Men have no vas deferens, assosted conception can help - Women may be sub-fertile. - Carrier testing of partners is useful too Salt loss - Salt replacement to replace excessive salt loss in sweat. Monitor carefully in infancy as excess salt is dangerous Liver disease - Slow bile flow --> biliary disease. Urodeoxycholic acid can help - Pseudo-obstruction of bowel, often mistaken for appendicitis, but just needs laxative or adjustment of pancreatic enzyme supplement

Answer 26

Bordatella pertussis. young infants or those not fully vaccinated - Paradoxical coughing spasms, followed by sharp intake of breath (whoop). Can cause apnoea in infants - Dx: Clinical. Lymphocytosis is siggestive - Rx: Supportive, coughing can continue for months

Answer 27

6mo-3yo, Parainfluenza virus. Infection of upper airways - Common in winter and can reoccur - Coryza, then stridor and barking cough, classically worse at night. Can also have hoarse voice - Milder cases, Rx: Observation and reassurance - Severe cases. Rx: intubation and ventilate - Oral dexamethasone also given - Signs of severity: Increased work of breathing, cyanosis, restlessness

Answer 28

Life threatening, caused by Hib - Young kids, signs of sepsis, inability to talk or swalllow (drooling) - If suspected, DO NOT do ENT EXAM - Rush to theatre for intubation by experienced anaesthetist

Answer 29

Obstruction of small airways by RSV --> Acute wheeze and resp distress in infants - Epidemics in winter months and very easily spread - Adenovirus, Parainfluenxa and influenza can also cause it - Coryza, then cough ,esp distress and wheeze - O/E: Widespread wheeze, fine crackels and overexpansion of chest - Nasopharyngeal aspirate often reveals RSV with immunofluorescence - Rx: Most need none, asts 7-10 days but may have recurrent wheeze in infancy - If severe (poor feeding, apnoea, increased resp distress or needing O2), admit - If chronic lung disease or underlying congenital heart defect, needs PICU - Only effective Rx is bronchodilators, O2, support. Palivizumab against RSV can be given to at risk kids in winter

Answer 30

Potter's syndrome - Caused by structural kidney disease (often bilateral renal agenesis, can be polycystik kidney disease) - Causes oligohydramnios and pressure in uterus - BRA is incompatible with life, other causes have a chance of survival. - At birth ventilation and dialysis is needed Polycystic kidney disease - Enlarged cystic kidneys, recessive form from infancy, dominant form in adolescence - Dominant: Loin pain, nocturia, hypertension, enlarged kidneys, gross haematuria - Recessive: can present periatally or in cildhood. Earlier = more severe and less liver involvement - Perinatally has 75% mortality in 1 week, born with large abdomen - Childhod: 6mo-5yo, 80% survival to 15YO. Marked liver disease, hepatomegaly and splenomegaly varied - USS to diagnose - Rx: Refer to paediatric nephrologist. Transplant is choice for end stage renal disease Posterior urethral valve - Causes common UTIs - Persistent embryological fold across urethr, causes bladder hypertrophy, bilateral hydronephrosis and renal involvement - Rx: Surgical correction if often best, done cyctoscopically

Answer 31

10% of 6YO and 3% of 12YO wet at least one night a week - Primary or secondary - Exclude UTI, Renal USS to exclude abnormalities - Rx: - Daytime: Encourage toiletting at regular intervals. Oxybutynin to increase bladder muscle stability if small bladder and frequent urgency - Night: Behavioural (star charts, lifting, rewards), reduce drinks in evenings - Enuresis alarms from age 7 - Desmopressin, enuresis nurse support

Answer 32

Nephrotic syndrome - Proteinuria, low albumin, oedema, hyperlipidaemia - Increased glomerylar permeability, 85% due to Minimal Change disease - Rx: Steroids - May have Hx of recurrent UTI Nephritic sydrome - Haematuria, proteinuria (>3.5g/day), decreased urine output, fluid retention and oedema - HTNm uraemia, decreased kidney function - Often caused by past infection with group A strep or other infections, SLE, HSP, Coodpastures also cause it - Rx: Full Hx, uraemia and other symptoms, previous infections, BP, oedema, urine dip - If nephritic syndrome syspected, refer to secondary care - Rx depends on further tests to find underlying cause

Answer 33

IgA mediated vasculitis of childhood - Skin urpura, arthritis, abdo pain, GI bleeding, orchitis and nephritis - Can be triggered by group A strep, EBV, mycoplasma or some food/drug allergies, pesticides or cold exposure - Occurs mustly in widter, 50-90% following URTI - Symmetrical, erythematous rash on backs of legs, buttocks and ulnar side of arms. in 24hrs, progress to purpuric lesions like bruises - Renal disease affects 40%, few progress to end stage renal failure - Rx: NSAIDs may help w/joint pain. No Rx can shorten disease pathway. Supportive Rx

Answer 34

Caused in 1/3 of cases by anatomical abnormalities or pelviuriteric obstructions - USS to diagnose - Idiopathic, constipation =, poor hygiiene, neuropathic vladder, vesicoureteric reflux are all causes Rx: - <3 months, signs of sepsis: full septic screen, urine MC&S, IV antibiotics - 3mo-3yo: Urine MC&S, start antibiotics - >3yo: Urine dipstick - If nitrate +ve, Probable UTI. Urine MC&S and start antibiotics - Leucocyte -ve, nutrate positive: Maybe UTI, urine MC&S, no Abx unless good evidence - Leucocyte -ve, nitrate -ve. Not UTI

Answer 35

Unilateral or bilateral. More comon in preterm ababies - 5% undescended at term, 1% by 12 months - May be associated with inguinal hernia - Orchidoplexy by 1yo to avoid infertility, torsion and malignancy - Bilateral impalpable tests need very careful evaluation

Answer 36

External urinary meatus opens on ventral side of penis - Mild: No Rx - Severe: Surgical repair. Don't circumcise yet as that skin may be needed for surgery

Answer 37

- Tender, swollen scrotum & intense pain - Occurs in pubertal boys and also babies - Rx: Urgent surgery to save the testis

Answer 38

Male - Has benefits, easier hygiene, reduced penile Ca, reduced STDs - No medical indication to offer it routinely - Contraindications: FGHx of bleeding disorders - Less painful for infants Female - Generally condemned in UK, is illegal

Answer 39

High arching, stiff feed - Due to muscle imbalances (agonist vs antagonist) in the foot, for which there are many static or porgressive causes (CP or Charcot Marie Tooth Dsease) - X-ray of foot (weightbearing) - Rx: Physio and orthotic shoes w/padding. Some patients may need splints. Some may need surgery

Answer 40

Common in newborns as transient postural deformity - Rarely, a more fixed deformity - Rx: Physio and serial casting (ponseti procedure if severe)

Answer 41

Self limiting hip disorder, caused by varying degrees of ischaemia and necrosis of femoral head - Presents with pain in hip or knee and limp, 10-15% are bilateral - FBC and ESR. X ray shows widening of joint space - Rx: No consensus - Early diagnosis helps prevent or slow necrosis - >6yo or 50% femoral head necrosis needs surgical Rx

Answer 42

- benign, common in 2-8YO - Limp (resolved in 1-3 weeks), preceeded often by URTI - No systemic symptoms, normal investigations

Answer 43

- Young teenage boys mostly, more common in overweight - Cradual onset groin pain - Dx: X ray (lateral pelvis) - Rx: external in-situ pinning or open reduction and pinning.

Answer 44

1% if infants, acetabulum is shallow leading to hip joint being dislocatable - Risk factors: Breech. FHx, Female, Impaired limb movement, Associated with talipes - Dx: Barlow and ortolani tests on newborn baby exam. USS if increrased risk (at 6 weeks) or positive newborn exam test - Rx: Wearing harness or splint for several months to hold in flexion and abduction

Answer 45

Most commonly 4 or fewer joints (pauciarticular) - usually large joints - <4yo, girls, minima systemic symptoms - Rheumatoid factor -ve, ANA may be positive - Increased risk of chronic uveitis (esp ANA +ve), slit lamp exam to screen for this - Arthritis completely resolves in 80% Systemic onset (still's disease) - Spiking fever, severe malaise, salmon pink rash - Anorexia, wt loss, pericarditis, hepatosplenomegaly - Affects small and large joints, 25% get severe arthritis - Associated with HLA DR4 - 25% persist into adulthood. Long term prognosis improved with biological therapies Polyarticular - Symmetrical, small and large joints - Poor weight gain and mild anaemia - RhF -ve, ANA sometimes positive - 12% get severe arthritis, prognosis generally good

Answer 46

Clinical diagnosis - Tonic clonic seizures are most common - Tonic: Sudden loss of consciousness, tongue biting, limbs extended, arched back, stops breathing - Clonic: Jerking intermittently, irregular breathing, may urinate - Post-ictal: Sleepy and disorientated - Absence seizures can also happen - Temporal love (impaired consciousness, strange sensations, movements or hallucinations) can also happen Rx: - best drug for that seizure type, up to max dose, then add another drug iff needed - Most use Na Valproate or Carpamezapine - General safety measures, especially for younger kids, to prevent physical injury when having seizure

Answer 47

Clinical Diagnosis: based on abnormal tone, delayed motor development, abnormal movement patterns and persistent primitive reflexes Causes - Pre-natal: Cerebrall malformations, congenital infections, metabolic defects - Perinatal: Complications of prematurity, intrapartum trauma, hypoxic ischaemic insult - Post natal: NAI, meningitis/encephalitis, head trauma, cardiopulmonary upset Associated problems - Learning difficulties - Epilepsy - Visual impairment - Squint - Hearing loss - Speech disorder - Behaviour disorder - Undernutrition & poor growth - Respiratory issues Management - MDT approach (Physio, OT, SALT, Dietician, Co-ordinated by paediatrician)

Answer 48

Failure of neural tube to close in early pregnancy. Folic acid reduces it by 75% Defects are always midline - Anencephaly: Cranial part of neural tube doesn't exist. Baby's brain cortex doesn't develop. baby dies - Myelmeningocele: Open lesion, cord covered by thin layer of meninges. Severe weakness of legs, bladder, bowel. Hydrocephalus. Causes severe learning disability - Meningocele: Spinal cord intact, smal sac of meninges which can rupture and cause meningitis - Spina bifida occulta: Hidden vertebral bodies fail to fuse properly, Clue (sometimes) is tuft of hair. Lipoma, naevus or deep sacral pit in midline over lower back. USS indicated

Answer 49

Rash, conjunctivitis, lymphadenopathy, cracked lips, strawberry tongue, skin peeling - Preceeded by a fever of >5 days - Raised inflammatory markers and platelets sometimes seen - Complications include coronary artery spasms or aneurysms (Echocardiogram) - Rx: Immunoglobulin and aspirin

Answer 50

Occurs in 1/4 West Africans or 1 in 10 Afro-carribeans - HbS. when deoxygenated, makes structured polymers --> britle, spiny RBCs which occlude vessels - Sickle cell trait is usually aspmypmatic but can cause issues with general anaesthetic - Acute, painful crises (homozygous): preciptated by dehydration, hypoxia, infection, acidosis. Painful swelling of hands is an early presentation. Rx: analgesia, antibiotics, fluids, warmth - Pneumococcal vaccines and prophylactic penicillin is recommended - Newborn blood spot is screen for haemoglobinopathies

Answer 51

- Beta thalassaemia is most common, affecting Asian and Mediterranean people - Beta thalassaemia trait: mild, hypovolaemic, macrocytic anaemia (often confused with iron deficiency) - Dx: Haemoglobin electrophoresis. (high HbA2 and HbF Rx: None needed - Alpha trait: mild, hypochromic, microcytic anaemia. Low HbA2 and HbF, no evidence of iron deficiency - For both trait, advise partner screening as kids may have more serious homozygous conditions - Beta homozygous: Severe haemolytic anaemia w/compensatory bone marrow hyperplasia (Bossing of facial and skull bones and dental abnormalities) - Marked hepatosplenomegaly - Rx: Regular blood transfusions, which can ausew iron overload, causing cardiomyopathy, diabetes and skin pigmentations - Need regular infusions of chelating agent desferrioxamine

Answer 52

Haemophilia A - Most common, due to inherited gene (many). Lack of Factor VIII - X-linked recessive (Xq28 - Often presents as neonatal bleeding or excess bleeding after procedures (Like tooth extraction) - APTT raised, Factor VIII:C ratio is reduced - Rx: Severe - prophylactic factor VIII infusions weekly - Acute bleed: Give factor VIII (In FFP or Cryoprecipitate if concentrate not available) - Desmopressin and antifibrinolytic agents can increase FVIII activity and reduce need for admission Haemophilia B - Due to lack of Factor IX. Xq27.1-27.2. levels rise after puberty (still less than normal) - Causes spontaneous haematomas and haemarthosis - PT normal, APTT high - Factor IC activity percentage is low - Rx: Acute - Transfusion required, recombinant F IX is first line, aim for 100% in severe haemorrhage. 50% otherwise - Give more F IX 24hrs later. Analgesia (not NSAIDs, they Cause GI haemorrhage) - Chronic: Bracelet, Hep A and B vaccine S/c not IM. Advice re: pros/cons of factor concentrates

Answer 53

Leukaemia - Commonest (35%) childhood malignancy - 80% of cases are ALL - Presents with malaise, anaemia, bruising, bone pain, lymphadenopathy - Chemotherapy to induce remission and prevent relapse - 85% survival for ALL, 60-70% for AML Lymphoma - Hodgkins & Non hodgkins - Presents with lymphadenopathy (mediastinal lymph node involvement is common) - Diagnostic node excision followed by chemotherapy

Answer 54

Rare, important cause of blindness - Presents in early years with white pupillary reflex and squint - Commonest tumour in infancy - 98% cure rate (surgery OR intra-ocular chemotherapy OR radiotherapy)

Answer 55

Usually in older kids, present with bone pain or mass, particularly in long bones Ewing's sarcoma - Primitive, neuroectodermal tumour (PNET) - Translocation at chromosome 11 and 22 - 65% 5 year survival Osteosarcoma - Commonest primary childhood bone tumour - 5yr survival is 65% Management - Chemo and surgery with bone prosthesis