TO DO PAEDS PART 1 Flashcards

(206 cards)

1
Q

FOETAL CIRCULATION
What is the flow of foetal blood?

A
  • Oxygenated + nutrients at placenta for rest of body (umbilical vein) + disposes waste like CO2 + lactate (umbilical artery)
  • Umbilical vein > ductus venosus > RA > foramen ovale > LA > LV > rest of body > umbilical artery
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2
Q

FOETAL CIRCULATION
What are physiological (innocent flow) murmurs?

A

4S’s –
- Soft blowing murmur
- Symptomless
- left Sternal edge
- Systolic murmur only

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3
Q

FOETAL CIRCULATION
What are the main cyanotic heart diseases?

A

4Ts –
- ToF
- TGA
- Tricuspid atresia
- Truncus arteriosus
(Complete AVSD too)

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4
Q

ATRIAL SEPTAL DEFECT
What signs would you find on clinical examination in ASD?

A
  • Fixed + widely split S2 (split does not change with inspiration/expiration)
  • ES murmur at upper L sternal edge (pulmonary) as increased flow across pulmonary valve by L>R shunt
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5
Q

VSD
What are the features of the pansystolic murmur in VSD?

A
  • Left lower sternal edge
  • Loud murmur = smaller VSD (larger = quieter)
  • May have systolic thrill on palpation
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6
Q

PDA
What are the signs of PDA?

A
  • Collapsing or bounding pulse as increased pulse pressure
  • Continuous ‘machinery’ murmur heard loudest beneath the L clavicle
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7
Q

PDA
What is the management of PDA?

A
  • Monitor until 1y with ECHOs (treat early if Sx or heart failure)
  • NSAIDs (indomethacin) facilitates closure of PDA as inhibits prostaglandins
  • After 1y unlikely to resolve so trans-catheter or surgical closure to reduce IE risk
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8
Q

TOF
What abnormalities are described in tetralogy of fallot (TOF)?

A
  • Large VSD
  • Pulmonary stenosis (RV outflow obstruction)
  • RVH
  • Overriding aorta
    (If ASD present too = pentad of Fallot)
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9
Q

TOF
What is the management of a hyper-cyanotic tet spell in TOF?

A
  • Morphine for sedation + pain relief
  • IV propranolol as peripheral vasoconstrictor
  • IV fluids, sodium bicarbonate if acidotic
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10
Q

TOF
What is the management of TOF?

A
  • Neonates = prostaglandin infusion to maintain ductus arteriosus to allow blood to flow from aorta > pulmonary arteries
  • Early surgical repair with closure of VSD + correction of pulmonary stenosis at 6m
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11
Q

TGA
What are the investigations for TGA?

A
  • May be Dx antenatally, pre (R arm) + post duct (foot) sats
  • CXR may show narrow mediastinum with ‘egg on its side’ appearance
  • ECHO confirms Dx
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12
Q

TGA
What is the management of TGA?

A
  • Neonates = prostaglandin E1 infusion to maintain ductus arteriosus
  • Balloon atrial septostomy to create hole between 2 atria for mixing
  • Arterial switch procedure = open heart surgery, definitive Mx
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13
Q

COARCTATION OF AORTA
What is the clinical presentation of coarctation of aorta?
How may it present if severe?

A
  • Weak femoral pulses + radiofemoral delay
  • Systolic murmur between scapulas or below L clavicle
  • Heart failure, tachypnoea, poor feeding, floppy
  • LV heave (LVH)
  • Acute circulatory collapse at 2d as duct closes (duct dependent)
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14
Q

COARCTATION OF AORTA
What are the investigations for coarctation of the aorta?

A
  • 4 limb BP (R arm > L arm), pre + post-duct sats
  • CXR may show cardiomegaly + rib notching (often teens + adults)
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15
Q

COARCTATION OF AORTA
What is the management of coarctation of aorta?

A
  • ABCDE if collapse
  • Prostaglandin E1 infusion if critical
  • Stent insertion or surgical repair
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16
Q

EBSTEIN’S ANOMALY
What is Ebstein’s anomaly associated with?

A
  • Wolff-Parkinson-White syndrome + lithium in pregnancy
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17
Q

EBSTEIN’S ANOMALY
What is the clinical presentation of Ebstein’s anomaly?

A
  • Evidence of heart failure
  • SOB, tachypnoea, poor feeding, collapse or cardiac arrest
  • Gallop rhythm with S3 + S4
  • Cyanosis few days after birth if ASD when ductus arteriosus closes
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18
Q

EBSTEIN’S ANOMALY
What are the investigations for Ebstein’s anomaly?

A
  • ECG = arrhythmias, RA enlargement (P pulmonale), LAD + RBBB
  • CXR = cardiomegaly + RA enlargement
  • ECHO diagnostic
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19
Q

AORTIC STENOSIS
What is the normal clinical presentation of aortic stenosis?

A
  • Most asymptomatic with ejection-systolic murmur at upper right sternal edge (aortic area) radiating to neck (carotid thrill)
  • Ejection click before murmur
  • Palpable systolic thrill
  • Slow rising pulses + narrow pulse pressure
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20
Q

PULMONARY STENOSIS
What is the clinical presentation of pulmonary stenosis?

A
  • Ejection systolic murmur at upper left sternal edge with ejection click
  • ?RV heave due to RVH
  • Critical PS = duct-dependent pulmonary circulation so cyanosis in first few days of life
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21
Q

RHEUMATIC FEVER
How is rheumatic fever diagnosed?

A

Jones criteria –
- Evidence of recent strep infection plus 2 major or 1 major + 2 minor criteria

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22
Q

RHEUMATIC FEVER
What are the major criteria in rheumatic fever?

A

JONES –

  • Joint arthritis (migratory as affects different joints at different times)
  • Organ inflammation (pancarditis > pericardial friction rub)
  • Nodules (subcut over extensor surfaces)
  • Erythema marginatum rash (pink rings of varying sizes on torso + proximal limbs)
  • Sydenham chorea
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23
Q

RHEUMATIC FEVER
What are the minor criteria in rheumatic fever?

A

FEAR –

  • Fever
  • ECG changes (prolonged PR interval) without carditis
  • Arthralgia without arthritis
  • Raised CRP/ESR
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24
Q

RHEUMATIC FEVER
What are the investigations for rheumatic fever

A
  • Throat swab for MC&S
  • Anti-streptococcal antibodies (ASO) titres = anti-DNase B +ve indicates strep infection (repeat after 2w to check if negative)
  • Echo, ECG + CXR to check cardiac involvement
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25
RHEUMATIC FEVER What is the management of rheumatic fever?
- Prevention by treating strep infections with 10d phenoxymethylpenicillin - Specialist Mx (NSAIDs for joint pain, aspirin + steroids for carditis) - Prophylactic 1/12 IM benzathine penicillin most effective to prevent recurrence (if not daily PO penicillin)
26
PDA What are some risk factors of PDA?
Prematurity is key + association with maternal rubella
27
HEART FAILURE What are the causes of heart failure in infants?
High pulmonary blood flow (VSD, AVSD, large PDA)
28
TOF What are some risk factors?
- Rubella, - maternal age >40, - alcohol in pregnancy, - maternal DM
29
TGA What is it associated with?
Duct dependent lesion, associated with PDA, ASD + VSD
30
TRICUSPID ATRESIA How is it managed?
Shunt between subclavian + pulmonary artery with surgery later
31
CROUP What are the investigations for croup?
- Clinical but if CXR done PA view shows subglottic narrowing (steeple sign)
32
CROUP What is the management of croup?
- PO dexamethasone 0.15mg/kg 1st line, can repeat at 12h - Nebulised budesonide (steroid) - High flow oxygen + nebulised adrenaline (more severe/emergency cases) - Monitor closely with anaesthetist + ENT input, intubation rare
33
ACUTE EPIGLOTTITIS What is the investigation for acute epiglottitis?
- Clinical Dx but if CXR done lateral view show epiglottis swelling = thumb sign
34
ACUTE EPIGLOTTITIS What is the management of epiglottitis?
- Prevention HiB vaccine, rifampicin prophylaxis for close household contacts - Do NOT examine throat, anaethetist, paeds + ENT surgeon input - Intubation if severe, may need tracheostomy - IV ceftriaxone + dexamethasone given once airway secured
35
BRONCHIOLITIS What are some criteria for admission?
- Apnoea - Severe resp distress (RR>60, marked chest recession, grunting) - Central cyanosis - SpO2 < 92% - Dehydration - 50–75% usual intake
36
PNEUMONIA What is the management of pneumonia?
- Newborns = IV broad-spec Abx (amoxicillin) - Older = PO amoxicillin with broad-spectrum Abx (co-amoxiclav) if unresponsive or influenza - Macrolides (erythromycin) to cover for mycoplasma, chlamydia or if unresponsive
37
ASTHMA What is the purpose of spirometry?
- Obstructive pattern = FEV1 <80%, FEV1/FVC < 70% - Bronchodilator responsiveness = FEV1 ≥12% improvement
38
ASTHMA What is the stepwise management of chronic asthma in <5y?
1. SABA + low dose ICS (trial for 8-12 weeks) IF SYMPTOMS RESOLVE 2. stop SABA + low dose ICS for 3 months 3. if symptoms recur restart SABA + low-dose ICS and titrate up to moderate dose ICS as needed 4. consider further trial without treatment 5. SABA + moderate dose ICS + LTRA 6 stop LTRA + refer to specialist IF SYMPTOMS DO NOT RESOLVE 2. check inhaler adherence, review if alternative diagnosis is likely 3. refer to specialist
39
ASTHMA What is the stepwise management of chronic asthma 5-12yrs?
1. SABA + ICS 2. decide whether MART pathway or conventional pathway is more suitable MART PATHWAY 3. SABA + low dose MART 4. SABA + moderate dose MART 5. refer to specialist CONVENTIONAL PATHWAY 3. SABA + ICS + LTRA (trial for 8-12 weeks) 4. SABA + low dose ICS/LABA (+/- LTRA) 5. SABA + moderate dose ICS/LABA (+/- LTRA)
40
ASTHMA What are some reasons for failure to respond to treatment for asthma?
ABCDE – - Adherence (#1) - Bad disease (dose inadequate for severity) - Choice of drug/device (different pts respond differently) - Diagnosis (?correct) - Environment (?trigger)
41
ASTHMA What is classed as a severe asthma exacerbation?
- PEFR 33–50% predicted - Unable to complete full sentences - RR>50 (2-5y), or >30 (>5y) - HR >130 (2-5y) or >120 (>5y) - Signs of resp distress (chest recessions) - SpO2 <92%
42
ASTHMA What is classed as a life-threatening asthma exacerbation?
- PEFR 33% predicted - Exhaustion/cyanosis - Poor respiratory effort - Altered consciousness, hypotension - Silent chest (airways so tight no air entry) - SpO2 <92%
43
ASTHMA What is the management of severe exacerbations of asthma?
stepwise approach: 1. salbutamol inhalers via spacer with 10 puff every 2 hrs 2. nebulisers with salbutamol/ipratropium bromide 3. oral prednisolone (for 3 days) 4. IV hydrocortisone 5. IV magnesium sulphate 6. IV salbutamol 7. IV aminophylline 8. call ICU
44
CROUP What are the causes?
- Parainfluenza viruses (#1), less so RSV, metapneumovirus, influenza
45
CROUP When would you admit a patient to hospital?
- Mod-severe croup, - <3m old - upper airway issues (laryngomalacia)
46
CROUP How do you assess croup severity?
Westley score for severity (chest wall retractions, stridor, cyanosis, air entry + consciousness)
47
VIRAL INDUCED WHEEZE What is the management?
1st line = PRN salbutamol 2nd line = Montelukast or ICS or both
48
PNEUMONIA What are the common causes of pneumonia in infants + young children?
RSV most common, pneumococcus #1 bacterial, H. influenzae, Bordatella pertussis, chlamydia trachomatis (S. aureus rarely but = serious)
49
PNEUMONIA What are the common causes of pneumonia in children >5?
Pneumococcus, mycoplasma pneumoniae, chlamydia pneumoniae
50
ASTHMA what is the management of mild-moderate exacerbations of asthma?
- salbutamol inhaler via spacer - give 1 puff every 30-60 seconds upto maximum 10 puffs if symptoms are not controlled, refer to hospital - oral prednisolone (for 3 days)
51
PYLORIC STENOSIS What is the management of pyloric stenosis?
1st line - nil-by-mouth and NG tube insertion (to decompress stomach) - IV fluids (rehydration + correct electrolyte imbalances) - ramstedt pyloromyotomy (laparoscopic)
52
MECKEL'S DIVERTICULUM What are the investigations for Meckel's diverticulum?
- Technetium scan will demonstrate increased uptake by ectopic gastric mucosa
53
CONSTIPATION What are some causes of constipation?
- Usually idiopathic - Meds (opiates) - LDs - Hypothyroidism - Hypercalcaemia - Poor diet (dehydration, low fibre) - Occasionally forceful potty training
54
CONSTIPATION What is the medical management of constipation?
- 1st = MACROGOL (osmotic) laxative like polyethylene glycol + electrolytes (Movicol) - 2nd = stimulant laxative if no effect like Senna, bisocodyl ± osmotic laxative (lactulose) or stool softener (docusate) if hard stools - 3rd = consider enema ± sedation or specialist manual evacuation - Continue for several weeks after regular bowel habit then gradual dose reduction
55
GORD What are some complications of GORD?
- Failure to thrive from severe vomiting - Oesophagitis = haematemesis, discomfort on feeding or heartburn, Fe anaemia - Aspiration > recurrent pneumonia, cough/wheeze - Sandifer syndrome = dystonic neck posturing (torticollis)
56
GASTROENTERITIS What are some complications of gastroenteritis?
- Isonatraemic + hyponatraemic dehydration - Hypernatraemic dehydration - Post-infective lactose intolerance (remove lactose + slowly reintroduce) - Guillain-Barré - Dehydration #1 cause of death
57
BILIARY ATRESIA What is the clinical presentation of biliary atresia?
SYMPTOMS - prolonged jaundice >2 weeks - pale stools - dark urine - irritability SIGNS - hepatomegaly - scleral icterus - failure to thrive - abdominal distention - signs of portal hypertension (if severe)
58
BILIARY ATRESIA What is the management of biliary atresia?
1st line - Kasai portoenterostomy - ursodeoxycholic acid 2nd line - liver transplant
59
NEONATAL HEPATITIS What are 4 main causes of neonatal hepatitis?
- Congenital infection - Alpha-1-antitrypsin (A1AT) deficiency - Galactosaemia - Wilson's disease
60
NEONATAL HEPATITIS What is galactosaemia?
- Deficiency of galactose-1-phosphate uridyltransferase (GALT) involved in galactose metabolism (lactose breaks down into galactose)
61
NEONATAL HEPATITIS What is the management of galactosaemia?
- Stop cow's milk, breastfeeding C/I - Dairy-free diet - IV fluids
62
NEONATAL HEPATITIS What is Wilson's disease?
- Reduced synthesis of caeruloplasmin (normally binds to copper + allows it to be excreted with bile)
63
NEONATAL HEPATITIS How does Wilson's disease present?
Sx of copper accumulation - Eyes (Kayser-Fleischer rings) - Brain (Parkinsonism + psychosis) - Kidneys (vit D resistant rickets) - Liver (jaundice)
64
FAILURE TO THRIVE How does NICE define faltering growth in children by weight?
- ≥1 centile spaces if birth weight was <9th centile - ≥2 centile spaces if birth weight was 9th–91st centile - ≥3 centile spaces if birth weight was >91st centile - Current weight is below 2nd centile for age, regardless of birth weight
65
CMPA What are the investigations for cows milk protein allergy (CMPA)?
- IgE mediated = skin prick tests + RAST for cow's milk protein - Gold standard if doubt = elimination diet under dietician supervision
66
CMPA What is the management for cows milk protein allergy (CMPA)?
FORMULA FED - 1st line = extensive hydrolysed formula milk - 2nd line = amino acid-based formula BREASTFED - continue breastfeeding - eliminate cows milk protein from maternal diet. (consider prescribing calcium supplements to mother) - use extensively hydrolysed milk when breastfeeding stops, until 12 months of age and at least for 6 months
67
CHOLEDOCHAL CYST How may it present?
- Cholestatic jaundice - abdominal mass - pain in RUQ - nausea and vomiting - fever
68
CHOLEDOCHAL CYST what are the investigations?
can be detected on ultrasound before the child is born after the baby is born, the parent's may notice lump in RUQ, the following tests are then done: - CT scan - cholangiography
69
CHOLEDOCHAL CYST What are the complications?
- Cholangitis - small risk of malignancy
70
CHOLEDOCHAL CYST What is the management?
Surgical cyst excision
71
CHOLEDOCHAL CYST what are the different types?
Type 1 - cyst of extrahepatic bile duct (most common) Type 2 - abnormal pouch/sac opening from duct Type 3 - cyst inside the wall of the duodenum Type 4 - cysts on both intrahepatic and extrahepatic bile ducts
72
HIRSCHSPRUNG'S DISEASE How is Hirschsprung associated enterocolitis (HAEC) managed?
Urgent Abx, fluid resus + decompression of obstructed bowel
73
MECKEL'S DIVERTICULUM What is the management of Meckel's diverticulum?
Surgical resection, may need transfusion if severe haemorrhage
74
GASTROENTERITIS what is the management for shigella infection?
severe = azithromycin or ciprofloxacin
75
GASTROENTERITIS What is hyponatraemic dehydration?
- Child with diarrhoea drinks large quantities of water, Na+ loss greater than water so fall in plasma Na+ – Fluid shifts from ECF>ICF + can result in convulsions
76
BILIARY ATRESIA What genetic mutation is biliary atresia associated with?
Associated with CFC1 gene mutations
77
NEONATAL HEPATITIS What is the cause of A1AT deficiency?
AR on chromosome 14
78
NEONATAL HEPATITIS What are the genetics for Wilson's disease?
AR on chromosome 13
79
FAILURE TO THRIVE What are some causes of inability to process nutrients properly?
- T1DM, - inborn errors of metabolism
80
CMPA What is cow's milk protein allergy (CMPA) associated with?
- More common in formula fed babies - those with personal or FHx of atopy
81
GASTROENTERITIS How does hypernatraemic dehydration present?
Jittery movements, increased muscle tone, hyperreflexia, convulsions, drowsiness/coma
82
GASTROENTERITIS How is hypernatraemic dehydration managed?
Slow rehydration over 48h
83
INTUSSUSSEPTION what are the risk factors?
- young children - male gender - preceding viral illness - henoch-schonlein purpura (HSP) - meckel's diverticulum - lymphoma
84
HSP What is Henoch-Schönlein purpura (HSP)?
- IgA mediated small vessel vasculitis leading to inflammation affecting the skin, joints, GI tract + kidneys
85
HSP What is the clinical presentation of HSP?
SYMPTOMS - joint pain - abdominal pain - bloody stools - haematuria SIGNS - palpable purpuric rash (typically on legs) - joint swelling - joint tenderness
86
HSP What are some investigations for HSP?
- serum U&Es = may have rising creatinine - serum clotting screen - urinalysis = proteinuria, haematuria + RBC cast cells - stool analysis to consider - skin biopsy - renal biopsy
87
HSP What is the management of HSP?
1st line: - supportive care (rest, hydration + monitoring for complications - NSAIDs (for pain relief + joint symptoms, use with caution in renal injury) 2nd line - corticosteroids - immunosuppressive agents (AZATHIOPRINE or CYCLOPHOSPHAMIDE)
88
HAEMOLYTIC URAEMIC SYNDROME What is haemolytic uraemic syndrome (HUS)?
- Thrombosis within small blood vessels throughout the body, usually triggered by a bacterial toxin (shiga)
89
HAEMOLYTIC URAEMIC SYNDROME What is the classic HUS triad?
- Microangiopathic haemolytic anaemia (due to RBC destruction) - AKI (kidneys fail to excrete waste products like urea) - Thrombocytopenia
90
HAEMOLYTIC URAEMIC SYNDROME What is the clinical presentation of HUS?
SYMPTOMS - bloody diarrhoea - fever - abdominal pain - vomiting - reduced urine output SIGNS - dehydrated (delayed CRT, tachycardic/hypotensive, mottled skin) - pyrexia - pallor
91
HAEMOLYTIC URAEMIC SYNDROME What are some investigations for HUS?
- FBC = anaemia, thrombocytopaenia - blood film = schistocytes due to microangiopathic haemolysis - LDH = raised - LFTs = raised bilirubin - urinalysis = microscopic haematuria + proteinuria - U&Es = raised creatinine + reduced eGFR, often hyperkalaemia - stool culture = e.coli 0157:H7 - PCR shiga toxin
92
HAEMOLYTIC URAEMIC SYNDROME What is the management of HUS?
SUPPORTIVE - IV fluids - red cell transfusion - dialysis (if refractory acidosis, hyperkalaemia, fluid overload or oliguria) 2ND LINE - antibiotics (only in non-e.coli HUS) - plasma exchange - eculizimab
93
HAEMATURIA What investigations for haematuria should all patients get?
- Urinalysis + urine MC&S - FBC, platelets, clotting + sickle cell screen - U+Es, creatinine, albumin, Ca2+, phosphate - USS kidneys + urinary tract
94
HAEMATURIA What investigations would you do if you suspected glomerular haematuria?
- ESR, C3/4 + anti-DNA antibodies - Throat swab + antistreptolysin O/anti-DNAse B titres - Hepatitis B/C screen - Renal biopsy if recurrent haematuria, abnormal renal function/complement levels or significant persistent proteinuria
95
UTI In terms of performing ultrasounds scans in UTI, what are the guidelines?
- USS within 6w if 1st UTI + <6m but responds well to Tx within 48h or during illness if recurrent or atypical bacteria
96
UTI Admission criteria for UTI?
- Admission if <3m, systemically unwell or significant risk factors
97
UTI What is the management of UTI for >3m with upper UTI?
?Admission for IV, if not PO co-amoxiclav or cefalexin for 7–10d
98
UT ABNORMALITIES Name 6 urinary tract abnormalities
- Renal agenesis - Multicystic dysplastic kidney - Polycystic kidney disease - Pelvic/horseshoe kidney - Posterior urethral valves - Prune-belly syndrome
99
UT ABNORMALITIES How can posterior urethral valves present in utero? What is a complication of posterior urethral valves?
- Oligohydramnios + potentially pulmonary hypoplasia - Risk of dysplastic kidneys, at its worse if bilateral could lead to potter syndrome
100
UT ABNORMALITIES What are the 2 first steps in management of urinary tract abnormalities? How is the management split after that?
- Antenatal Dx + start prophylactic Abx to prevent UTI - Bilateral hydronephrosis and/or dilated lower urinary tract in a male - Unilateral hydronephrosis in male or any anomaly in female
101
UT ABNORMALITIES What is the management of bilateral hydronephrosis and/or dilated lower urinary tract in a male?
- Bilateral seen in bladder neck obstruction or posterior urethral valves - USS within 48h of birth to exclude posterior urethral valves – Abnormal = MCUG + surgery if required (ablation during cystoscopy) – Normal = stop Abx, repeat USS after 2-3m
102
UT ABNORMALITIES What is the management of unilateral hydronephrosis in male or any anomaly in female?
- Unilateral seen in pelviureteric or vesicoureteric junction obstruction - Abnormal = further investigations - Normal = stop Abx, repeat USS after 2-3m
103
CHRONIC KIDNEY DISEASE What is the management of CKD?
- Diet + NG or gastrostomy feeding may be needed for normal growth - Phosphate restriction + activated vitamin D to prevent renal osteodystrophy - May need recombinant growth hormone - Recombinant erythropoietin to prevent anaemia - Dialysis + transplantation if in ESRF (GFR <15ml/min/1.73m^2)
104
VESICOURETERIC REFLUX how is it graded?
graded using International Reflux Study grading system
105
VESICOURETERIC REFLUX how is it diagnosed?
- micturating cystourethrogram: radiocontrast medium introduced to catheterised bladder, reflux is detected on voiding - indirect cystogram
106
NEPHROTIC SYNDROME what can cause minimal change disease?
- NSAIDs, - Hodgkin's lymphoma, - infectious mononucleosis
107
HAEMATURIA What are some non-glomerular causes of haematuria?
- Wilm's tumour, - trauma, - stones (esp if FHx), - sickle cell disease - other bleeding disorders
108
UTI What are the investigations for recurrent + atypical UTIs?
- USS within 6w in all children with recurrent UTIs - DMSA (dimercaptosuccinic acid) scan (renal scarring 4-6m) - Micturating cystourethrogram (<6m) if FHx of vesico-ureteric reflux, dilatation of ureter on USS, poor urinary flow (catheterise + inject contrast into bladder)
109
UTI What is the management of children under 3m in UTI?
ALL children <3m + fever get immediate IV cefuroxime + full septic screen (blood cultures, FBC, CRP lactate, LP etc)
110
UTI What is the management of UTI for >3m with lower UTI?
3d PO trimethoprim, nitrofurantoin, amoxicillin or cephalosporin with follow-up if still unwell after 24-48h
111
PYELONEPHRITIS what are the risk factors?
- vesicoureteral reflux (VUR) = most common + most important - previous history of UTI - siblings with a history of UTI - female sex - indwelling urinary catheter - intact prepuce in boys - structural abnormalities of the kidneys and lower urinary tract
112
PYELONEPHRITIS what is the management?
- empirical antibiotics (co-amoxiclav or cefalexin) then targeted based on cultures - severe = hospitalisation and IV antibiotics
113
PYELONEPHRITIS how can it be prevented?
children <2yrs diagnosed with a UTI should have a renal USS
114
NOCTURNAL ENURESIS what are the causes?
- not waking to bladder signals - inadequate levels of vasopressin (ADH) - overactive bladder - constipation - UTIs - Family history - Anxiety/stress - poor bedtime routines
115
NOCTURNAL ENURESIS what is the presentation of inadequate levels of vasopressin?
- large volumes of urine passed at night - wet in the early part of the night - wet more than once per night
116
NOCTURNAL ENURESIS what is the presentation of an overactive bladder?
- damp patches that occur at night also occur during the day - the volume of urine passed is variable - children often wake after wetting at night
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NOCTURNAL ENURESIS what is the medical management?
- antibiotics for infection - laxatives for constipation - alarms - desmopressin - anticholinergic medications (oxybutynin + tolterodine) for detrusor relaxation
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ALPORT'S SYNDROME what are the 3 types?
X-linked Alport syndrome (XLAS) Autosomal recessive Alport syndrome (ARAS) Autosomal dominant Alport syndrome (ADAS)
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ALPORT SYNDROME what is the clinical presentation?
- haematuria - oedema - hypertension - loss of kidney function - progressive hearing loss - proteinuria - vision problems
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ALPORT SYNDROME what is the management?
ACE inhibitors dialysis kidney transplant
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RDS What are some risk factors of RDS?
- Prematurity #1 - Maternal DM - 2nd premature twin - C-section
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RDS What is the investigation for RDS?
CXR – - Reticular "ground-glass" changes - Heart borders indistinct - Air bronchograms
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NEC. ENTEROCOLITIS What are some risk factors for necrotising enterocolitis?
- Very LBW + premature - Formula feeds (breast milk protective) - RDS + assisted ventilation - Sepsis - PDA + other CHD
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NEC. ENTEROCOLITIS What are some investigations for necrotising enterocolitis?
- FBC = thrombocytopaenia and neutropenia - U&Es = deranged electrolytes - CRP = raised - blood gas = acidosis, raised lactate - abdominal x-ray = bowel wall thickening, gas filled loops, gaseous distention to consider - bowel USS = increased bowel wall thickness + echogenicity, free fluid collection, loss of bowel wall perfusion
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NEC. ENTEROCOLITIS What would an AXR show in necrotising enterocolitis?
- Dilated loops of bowel - Bowel wall oedema (thickened bowel walls) - Pneumatosis intestinalis (intramural gas) - Pneumoperitoneum (free gas in peritoneum = perf) - Football sign = air outlining falciform ligament - Rigler's sign = air both inside/outside bowel wall - Gas in portal veins
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NEC. ENTEROCOLITIS What is the management of necrotising enterocolitis?
STAGE 1 - conservative = NG tube, IV fluids, parenteral nutrition - medical = IV antibiotics (triple therapy), paracetamol +/- morphine STAGE 2 & 3 - conservative = NG tube, discuss with surgical team - medical = correct electrolytes, IV morphine, IV antibiotics (triple therapy) - surgical = laparotomy +/- bowel resection
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JAUNDICE What are some risk factors for jaundice?
- LBW - Breastfeeding - Prematurity - FHx - Maternal diabetes
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JAUNDICE Jaundice can be split into 3 aetiological time categories. What are these?
- <24h = always pathological, usually haemolytic disease - 24h–2w = common - >2w = also bad
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JAUNDICE What are some causes of jaundice 24h–2w after birth?
- Physiological + breast milk jaundice (common) - Infection (UTI, sepsis) - Haemolysis, polycythaemia, bruising - Crigler-Najjar syndrome (rare inherited disorder with no UGT enzyme)
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JAUNDICE What are some causes of jaundice >2w after birth?
- Unconjugated = physiological or breast milk, UTI, hypothyroid, high GI obstruction (pyloric stenosis), Gilbert syndrome - Conjugated (>25umol/L) = bile duct obstruction (biliary atresia), neonatal hepatitis
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JAUNDICE What is Gilbert's syndrome? How does it present?
- AR deficiency of UDP-glucuronyltransferase = defective bilirubin conjugation - Unconjugated hyperbilirubinaemia (not in urine), jaundice may only be present if ill, exercising or fasting
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JAUNDICE What investigations would you perform in neonatal jaundice?
- FBC + blood film (polycythaemia, G6PD, spherocytosis) - Bilirubin levels - Blood type testing of mother + baby for ABO/Rh incompatibility - Direct Coombs (antiglobulin) test for haemolysis - TFTs, LFTs + urine MC&S
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HIE What happens as a result of cardiorespiratory depression?
- Hypoxia, hypercarbia + metabolic acidosis - Compromised cardiac output reduces tissue perfusion > hypoxic ischaemic injury to brain
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HIE What is used to stage the severity of HIE? What are the stages?
Sarnat staging – - Mild = poor feeding, generally irritable + hyperalert, resolves in 24h - Moderate = poor feeding, lethargic, hypotonic, seizures, can take weeks to resolve - Severe = reduced GCS, apnoeas, flaccid + reduced/absent reflexes, half die
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TORCH What are the TORCH conditions?
Main congenital conditions - Toxoplasmosis, - Other (HIV), - Rubella, - CMV, - Herpes + Syphilis
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TORCH What are the characteristic features of toxoplasmosis?
- Cerebral calcification, chorioretinitis + hydrocephalus
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TORCH What is the clinical presentation of CMV?
- 90% normal at birth - 5% = hepatosplenomegaly, petechiae at birth, growth issues, neurodevelopmental disabilities (cerebral palsy, epilepsy, microcephaly) - 5% = problems later in life, mainly sensorineural hearing loss
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TORCH How does syphilis present?
- Rash on soles of feet + hands - Hutchinson's triad = keratitis, deafness, small + pointed teeth
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MECONIUM ASPIRATION What are some risk factors for meconium aspiration?
- Post-term deliveries at 42w - Maternal HTN or pre-eclampsia - Smoking or substance abuse - Chorioamnionitis
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MECONIUM ASPIRATION What is a complication of meconium aspiration? What are some other risk factors for that complication?
- Persistent pulmonary HTN of the newborn due to high pulmonary vascular resistance - RDS, sepsis, congenital diaphragmatic hernia, maternal SSRI use, maternal NSAID use in 3rd trimester (early closure of DA)
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MECONIUM ASPIRATION | What is the management of meconium aspiration?
- Artificial (positive pressure) ventilation with oxygenation - Suction if no breathing
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GASTROSCHISIS What is the management of gastroschisis?
- May attempt vaginal delivery - Urgent repair (theatre within 4h)
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BRONCHOPULMONARY DYSPLASIA How can bronchopulmonary dysplasia be prevented?
- Corticosteroids to mothers in premature labour <34w - CPAP rather than intubation where possible - Use caffeine to stimulate resp effort - Do not over oxygenate
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DUODENAL ATRESIA What is the clinical presentation?
- most appear well at birth - when they atart to feed they are sick (vomit is green) - jaundice - not pass meconium in first day
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GROUP B STREP INFECTION which babies are at more risk of becoming infected with group B strep?
- preterm labour - premature rupture of membranes - a long time between rupture of membranes and birth - internal foetal monitor - fever - past pregnancy with baby who had strep B - african-american/hispanic - group B strep in urine during pregnancy
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GROUP B STREP INFECTION what are the investigations?
- blood cultures - FBC, CRP - blood gas - urine microscopy - lumbar puncture - sputum culture
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GROUP B STREP INFECTION? what is the management?
- IV antibiotics (IV benzylpenicillin with gentamicin) - NICU admission
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PREMATURITY What are some neuro complications of prematurity?
- Cerebral palsy, - hearing/visual impairment, - intraventricular haemorrhage
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PREMATURITY What are some metabolic complications of prematurity?
- Hypoglycaemia, - hypocalcaemia, - electrolyte imbalance, - fluid imbalance - hypothermia
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PREMATURITY What causes hypocalcaemia?
Kidneys + parathyroid not fully developed
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JAUNDICE How does kernicterus present? What are the outcomes?
- Lethargy, poor feeding > hypertonia, seizures + coma - Permanent damage = dyskinetic cerebral palsy, LD + deafness
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TORCH How is CMV managed?
No therapy so no screening
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TORCH How is syphillis managed?
- If fully treated ≥1m before delivery = no treatment - Any doubts = benzylpenicillin
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OESOPHAGEAL ATRESIA What is it associated with?
- Tracheo-oesophageal fistula + polyhydramnios
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EXOMPHALOS What is the management?
C-section at 37w, staged repair as primary closure difficult
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LISTERIA INFECTION what is the management?
ampicillin + aminoglycoside (gentamycin)
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HIE what are the risk factors?
MATERNAL - HTN - diabetes - substance abuse - infection OBSTETRIC - prolonged labour - meconium-stained amniotic fluid - placental abruption - umbilical cord prolapse INFANT - prematurity - low birth weight - congenital abnormalities
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HIE what are the investigations?
- blood gas - blood tests - cranial USS to consider - MRI - EEG
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SIDS what are the risk factors?
- not prone sleeping - parental smoking - prematurity - bed sharing - hyperthermia - head covering other risk factors - LBW - male sex - maternal drug use - multiple births - incidence increases in winter
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SIDS what are protective factors?
- breastfeeding - room sharing - use of dummies
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SIDS what measures can be taken to reduce the risk of SIDS?
- put baby on their back when not directly supervised - keep head uncovered - place feet at foot of the bed to prevent them sliding down under blanket - keep cot clear of toys and blankets - maintain a comfortable room temperature (16-20 degrees) - avoid smoking (avoid handling baby after smoking) - avoid co-sleeping (particularly on sofa or chair) - if co-sleeping avoid drugs, alcohol, sleeping tablets or deep sleepers
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HSV ENCEPHALITIS what is the cause?
- neonates = HSV-2 (genital herpes) - older children = HSV-1 (cold sores)
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HSV ENCEPHALITIS what are the complications?
- lasting fatigue + prolonged recovery - changes to personality or mood - changes to memory and cognition - learning disability - headaches - chronic pain - movement disorder - sensory disturbance - seizures - hormonal imbalance
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CONGENITAL HYPOTHYROIDISM what are the causes?
- primary congenital hypothyroidism - thyroid dysgenesis - dyshormonogenesis - secondary or central congenital hypothyroidism
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CONGENITAL HYPOTHYROIDISM what are the risk factors?
- medication use during pregnancy - maternal advanced age - family history of thyroid disease - low birth weight - preterm birth - multiple pregnancies
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CONGENITAL HYPOTHYROIDISM what are the clinical features?
- prolonged neonatal jaundice - poor feeding + weight gain - hypothermia - macroglossia - large fontanelle - distended abdomen with umbilical hernia - dry skin - hoarse cry - myxoedema - bradycardia
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CEREBRAL PALSY What are the causes of cerebral palsy?
- Antenatal (80%) = genetics, congenital malformations or infections - Intrapartum (10%) = hypoxic-ischaemic injury - Postnatal (10%) = IV haemorrhage (prems), meningitis/encephalitis, trauma (NAI), hydrocephalus, kernicterus
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CEREBRAL PALSY What are some early features of cerebral palsy?
- Abnormal limb/trunk tone + posture with delayed motor milestones - Feeding issues > oromotor incoordination, slow feeding, gagging + vomiting - Abnormal gait when walking achieved - Hand preference before 12m + primitive reflexes after 6m
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CEREBRAL PALSY What are the stages of the Gross Motor Function Classification System?
- I = walks without limitation - II = with limitation - III = handheld mobility device - IV = III with limitation - V = wheelchair
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VISION What are some causes of severe visual impairment?
Genetic – - Congenital cataracts - Albinism - Retinal dystrophy - Retinoblastoma
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HEARING What are some causes of conductive hearing loss?
- #1 = congestion behind eardrums (viral URTI) - Glue ear, ear wax, middle ear infection, perforated ear drum - Structural abnormality of the outer ear (syndromes)
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HEARING What are some causes of sensorineural hearing loss?
- Genetic or syndromes - Perinatal (trauma, infection, hypoxia) - Congenital infections (rubella, CMV) - Meningitis (pneumococcus can cause ossification of cochlear)
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HEARING What are some risk factors for conductive hearing loss?
- Down's syndrome, - craniofacial syndromes - cleft palate
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HEARING What 2 types of hearing tests are part of the newborn hearing screening programme (NHSP)?
- Evoked otoacoustic emission (EOAE) - Auditory brainstem response (ABR) audiometry if EOAE fails
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HEARING What is evoked otoacoustic emission? What are the pros? What are the cons?
- Earphone produces sound which evokes an echo from ear if cochlear function normal - Simple + quick - Misses auditory neuropathy, cochlear test not hearing, high false +ve in first 24h
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HEARING What is auditory brainstem response audiometry? What are the pros? What are the cons?
- Computer analysis of EEG waveforms evoked in response to auditory stimuli - Screens hearing pathway ear>brainstem, low false +ve rate - Affected by movement (time consuming), electrodes on infant's head, complex computerised gear
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HEARING What testing might be done in children 6–9m?
- Distraction testing - Relies on baby locating + turning appropriately to high + low frequency sounds out of field of vision - 2x trained staff
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HEARING What testing might be done in children 10–18m?
- Visual reinforcement audiometry - Hearing thresholds are established using visual rewards (illumination of toys) to reinforce the child's head turn to stimuli of different frequencies - First test that does single ear measures
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HEARING What hearing tests are done at... i) >2y? ii) >2.5y? iii) 4y?
i) Performance testing = child performs an action when hear a noise ii) Speech discrimination tests (McCormick toy test) iii) Pure tone audiometry at school entry = child responds to pure tone stimulus with headphones
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HEARING What does Rinne's test show you?
- Normal = louder at EAM - Conductive = louder on mastoid - Sensorineural = both decreased
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HEARING What does Weber's test show you?
- Normal = vibrations equal in both ears - Conductive = louder in abnormal ear - Sensorineural = louder in normal ear
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EPILEPSY What are 4 epilepsy syndromes seen in children?
- Infantile spasms (West's syndrome) - Lennox-Gastaut syndrome - Juvenile myoclonic epilepsy - Benign Rolandic epilepsy = M>F, paraesthesia (unilateral face, tongue, twitching) during sleep, EEG shows centrotemporal focal spike waves
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EPILEPSY Who is affected by infantile spasms?
- Early life (4-6m), M>F, often secondary to serious neuro abnormality (tuberous sclerosis, encephalitis, birth asphyxia)
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EPILEPSY What are the 3 components to infantile spasms?
- Violent flexor spasms of head, trunk + limbs followed by extension of arms (salaam spasms) for 1-2s, can repeat up to 50 times - Progressive mental handicap - EEG shows hypsarrhythmia
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EPILEPSY What is Lennox-Gastaut syndrome? How does it present? Management?
- Can be extension of infantile spasms, 1-5y - Atypical absences, falls, jerks + 90% have mod-severe mental handicap - EEG shows slow spike, ketogenic diet may help
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EPILEPSY Who is juvenile myoclonic epilepsy more common in? How does it present? Management?
- Teens, F>M - Infrequent generalised seizures (often morning), daytime absences, sudden shock-like myoclonic seizures (can happen before seizures) - Good response to valproate
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EPILEPSY What is the management of generalised seizures?
- 1st line = sodium valproate - 2nd line = lamotrigine, carbamazepine (TC), clonazepam (myoclonic)
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EPILEPSY What is the management of focal seizures?
- 1st line = carbamazepine or lamotrigine - 2nd line = levetiracetam or sodium valproate
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EPILEPSY What is the management of absence seizures?
- Ethosuximide or sodium valproate
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DISORDERS Name 2 neurocutaneous disorders What is the mode of inheritance for these conditions?
- Neurofibromatosis (type 1 + 2) + tuberous sclerosis - AD
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NEUROFIBROMATOSIS What is the clinical presentation of neurofibromatosis 1?
- No intellectual problems but lots of skin involvement - >5 café-au-lait spots - Axillary freckling in skin folds - Iris hamartomas, scoliosis + pheochromocytomas - Peripheral neurofibromas
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NEUROFIBROMATOSIS What is the clinical presentation of neurofibromatosis 2?
- Hearing problems with no skin involvement - Bilateral vestibular schwannomas > sensorineural hearing loss then tinnitus + vertigo
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TUBEROUS SCLEROSIS What are the cutaneous features of tuberous sclerosis?
- Hypopigmented 'ash-leaf' spots which fluoresce under UV light - Roughened (Shagreen) patches of skin over lumbar spine - Angiofibromas (butterfly distribution over nose) - Subungual fibromata
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TUBEROUS SCLEROSIS What are some other features of tuberous sclerosis?
- Neuro = epilepsy (infantile spasms or partial), developmental delay + intellectual impairment - Retinal hamartomas, - polycystic kidneys, - rhabdomyomata of heart
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NEURAL TUBE DEFECTS What are 5 different types of neural tube defects?
- Spina bifida occulta (#1) - Meningocele - Myelomeningocele (most severe) - Anencephaly - Encephalocele
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GLUE EAR What is the management?
- Insertion of ventilation tubes (grommets) to drain excess fluid - Adenoidectomy as adenoids can harbour organisms + obstruct Eustachian tube so poor ventilation + drainage
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GLUE EAR What investigations would you do?
- Otoscopy (TM appears dull + retracted, often with visible fluid level) - Flat trace on tympanometry + evidence of conductive loss on pure tone audiometry (or reduced hearing on distraction test if younger)
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DEAFNESS what are the causes of sensorineural hearing loss?
Inherited/genetic - ushers syndrome, Waardenburg syndrome Acquired - perinatal - birth asphyxia, hyperbilirubinemia, congenital infection (rubella, CMV, syphilis) - postnatal - drugs, meningitis, head injury, labyrinthitis, acoustic neuroma
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DEAFNESS what are the causes of conductive hearing loss?
External - ear canal atresia/stenosis Middle ear - acute/chronic otitis media, glue ear
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EPILEPSY What is the management of infantile spasms?
Vigabatrin or corticosteroids (poor prognosis)
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EPILEPSY What is the management of myoclonic seizures?
- 1st line = sodium valproate - 2nd line = clonazepam
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'FUNNY TURNS' What is the clinical presentation of reflex anoxic seizures?
- Child becomes pale + falls to floor, - hypoxia may induce generalised tonic-clonic seizure which is brief + child RAPIDLY recovers
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'FUNNY TURNS' What are the investigations for reflex anoxic seizures?
- Ocular compression under controlled conditions often lead to asystole - paroxysmal slow-wave discharge on EEG
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TUBEROUS SCLEROSIS What are the investigations?
CT/MRI will detect calcified subependymal nodules + tubers from 2nd year of life
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DEVELOPMENTAL DELAY what are the referral points?
- doesn't smile at 10 weeks - cannot sit unsupported at 12 months - cannot walk at 18 months
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DEVELOPMENTAL DELAY what problems can indicate fine motor problems?
hand preference before 12 months - can indicate cerebral palsy