Paeds Mx Flashcards
Signs, Ix and Management of ASDs?
Sx: Ejection systolic murmur (upper L sternal edge), fixed wide splitting of S2
Ix: (severe, long term cases)ECG: RAD, RBBB for secundum. partial AVSD - the AV node is displaced and therefore it conducts the ventricles superiorly giving a negative reflection of QRS in the AVF lead. Echo will show the abnormality.
Observation is main treatment, as it may close or shrink; measured by ratio of pulmonary to systemic blood flow, where <1.5 is of little prognostic importance. >1.5 = large enough to cause RV dilatation and will require surgical closure at 2-4 years of age.
Secundum ASDs - managed by cardiac catheterisation with the insertion of an occlusive device (percutaneous closure/endovascular closure) but may need
surgical closure under GA.
Partial AVSD - managed by surgical correction
Prophylactic amoxicillin for the first 6m after device insertion
Sx and Mx of VSD?
Signs:
• Pansystolic murmur (lower L sternal edge) (loud murmur = smaller defect) (will be soft/absent in large VSD)
• Quiet P2
• Large VSD will have apical mid-diastolic murmur from increased flow over the mitral valve
• Signs of HF if large VSD (hepatomegaly, SOB, failure to thrive, recurrent chest infections) (From 1 week old)
Ix: ECG will show biventriuclar hypetrophy if LVSD. (Upright t wave in V1 indicates pul HTN).
Management Depends on size! If smaller than AV node diagemeter (~ 3mm), they will close spontaneously, ascertained by the disappearance of the murmur and normal ECG/Echo. While the VSD is present, prevention of bacterial endocarditis is by maintaining dental hygiene. Proph amoxicillin if high risk.
More than 3mm: Drug therapy for HF is with diuretics furosemide, often combined with captopril and digoxin. Additional calorie input is required.
Surgery 3-6months is required to manage HF and prevent lung damage from pul HTN. This procedure (pulmonary artery banding) narrows the pulmonary artery to reduce the blood flow to the lungs. When the child is older, an operation is done to remove the band and fix the VSD with open-heart surgery if the opening is large.
Signs, IX and management of PDA?
Signs: Continuous murmur below L clavicle (cont. as Pa in pul artery always less than aorta)
ECG- Large L-R shunt will cause RVH (+ Pul HTN) and LVH and so indistinguishable from large VSD.
If a cyanotic disease is dependent on a PDAs (e.g. TGA), the patient should start a prostaglandin infusion to keep the PDA open until corrective surgery can be performed
• The duct can be closed using:
o IV Indomethacin – 1st line treatment
o Prostacyclin synthetase inhibitor
o Ibuprofen ( Usually done in premature/VLBW infants)
• If pharmacological methods are unsuccessful, surgical ligation or PCD closure may be used
• Term infants:
o Symptomatic >6mo, PCD closure ASAP
o Usually closed using a coil or occlusive device introduced through a cardiac catheter at ~1yo via femoral.
o Diuretics can be given if delay of closure, to manage symptoms
Acute diagnosis and management of cyanotic heart disease in neonate
Ix: Nitrogen washout test ( The infant is placed in 100% oxygen (headbox or ventilator) for 10 min. If the right radial arterial PaO2 from a blood gas remains low (<15 kPa, 113 mmHg) after this time, a diagnosis of ‘cyanotic’ congenital heart disease can be made if lung disease and persistent pul HTN of newborn have been excluded)
Mx: ABC, artificially ventilate if necessary. Start prostaglandin infusion (PGE, 5 ng/kg per min). Most infants with cyanotic heart disease presenting in the first few days of life are duct dependent; i.e. there is reduced mixing between the pink oxygenated blood returning from the lungs and the blue deoxygenated blood from the body. Maintenance of ductal patency is the key to early survival of these children. Observe for potential side-effects – apnoea, jitteriness and seizures, flushing, vasodilatation and hypotension
TOF Ix and Mx
Sx: loud systolic MURMUR FROM DAY1, murmur will shorten with time and cyanosis will increase. Clubbing in older children. Can present with severe cyanosis on d1 or much later with hypercyanotic spells (squatting on exs).
Ix: CXR: boot-shaped heart (RVH), pul artery ‘bay’ (concavity where the PulA should be). ECG normal at birth. RVH eventually.
Initial mx is medical, with definitive surgery at 6 months. It involves closing the VSD and relieving right ventricular outflow tract obstruction, sometimes with an artificial patch, which extends across the pulmonary valve.
• Infants who are very cyanosed in the neonatal period require a shunt to increase pulmonary blood flow. This is usually done by surgical placement of an artificial tube between the subclavian artery and the pulmonary artery (a modified Blalock–Taussig shunt), or sometimes by balloon dilatation of the RV outflow tract. ±ECMO.
• Hypercyanotic spells are usually self-limiting and followed by a period of sleep. If prolonged (beyond about 15 min), they require prompt treatment with:
– sedation and pain relief (morphine is excellent)
– IV propranolol (or an α adrenoceptor agonist), which probably works both as a peripheral vasoconstrictor and by relieving the subpulmonary muscular obstruction that is the cause of reduced pulmonary blood flow
- bicarb to correct acidosis
Ix & TGA management
Signs: Cyanosis is the main sx and it is always present. Often presents on day 2 when the PDA closes with increased cyanosis. Presentation can be delayed if there are other SDs. Loud S2 that is single. Generally, NO MURMUR. (Can have systolic murmur from pul stenosis -> increased blood flow)
Ix: CXR can show ‘egg on the side’ on cardiac contour, ECG is normal, Echo shows the deformity.
Mx: In a cyanosed neonate, the key is to improve mixing.
• Maintain patency of DA with PG infusion is mandatory.
• A balloon atrial septostomy is a life-saving procedure which may need to be performed in 20% of those with TGA. A catheter, with an inflatable balloon at its tip, is passed through the umbilical or femoral vein and then on through the right atrium and foramen ovale. The balloon is inflated within the left atrium and then pulled back through the atrial septum. This tears the atrial septum, renders the flap valve of the foramen ovale incompetent, and so allows mixing of the systemic and pulmonary venous blood within the atrium.
• All pts need the arterial switch procedure in the neonatal period. In this operation, performed in the first few days of life, the pulmonary artery and aorta are transected above the arterial valves & switched over. coronary arteries have to be transferred across to the new aorta also.
Signs and management of cardiac outflow obstructions in the well infant
Well children (if asymptomatic -> f/u, no intervention) Aortic Stenosis - murmur on UR sternal edge, carotid thrill; Mx is Trans-catheter balloon dilatation (balloon valvulotomy) If severe, transcatheter aortic valve replacement (TAVR) with proph abx and anticoag.
Pul stenosis - Murmur UL sternal edge, no carotid thrill; Transcatheter balloon dilatation 1st line, valvulostomy 2nd line.
Coarctation -Systemic HTN, Stent insertion or surgery
Signs & Mx for outflow obstructions in the sick child
Sick infant (i.e. duct dependent) ALL PRESENT WITH COLLAPSE. ALL NEED ABC + PG.
Coarctations -> Collapse, absent FEMORAL pulses. Abc + PG infusion + Surgery.
Interruption of Aortic Arch - distal aortic arch is attached (via a duct) to pulmonary artery instead of aorta. VSD usually present. Syndromic associations e.g. DiGeorge. Present with collapse, absent LEFT BRACHIAL pulse. Surgical repair and closure of VSD in first few days of life.
Hypoplastic LH syndrome: (MV and AV is small/absent, coarctation, LV is v small -> no flow on left hand side of heart.) Present antenatally or post natally with collapse and severe acidosis. Absence of all peripheral pulses. Surgical management: Norwood procedure (neonatally) and then Glenn/hemi-Fontan (6 months) and Fontan (at 3 yo)
Cardiac arrhythmia management
It is important to differentiate normal and pathological arrhythmias:
Normal sinus arrhythmia are cyclical changes in HR with respiration. Acceleration in inspiration and slowing on expiration (HR can change by 30 beats/min)
SVT - most common arrhythmia (250-350 b/min) due to premature activation of atrium via accesspory pathway (rarely a structural issue), echo needed. Mx depends on severity and whether there are signs of HF on investigation e.g. T wave inversion on lateral precordial leads.
1) Circulatory and resp support, correct acidosis
2) Vagal stimulating maneouvers e.g. carotid sinus massage or cold ice pack to face (v successful)
3) IV adenosine is treatment of choice (safe, effective, induces AV block and terminates tachycardia by breaking re-entry circuit that is set up between AV node and accessory pathway) Given in incrementally in increasing doses
4) If adenosil fails, Electircal cardioversion with synchronised DC shock
Once sinus rhythm is restored -> Maintenance therapy using flecainide (na channel blocker) or sotalol (beta blocker). 90% of children have no further attacks. The eons that do can be treated with cryoablation of accessory pathway.
Rheumatic fever Mx
QUICK RECAP: Rare in developed countries, rare Cx of strep throat infection where pt develops fever, painful joints etc. After several attacks, in 50% of cases there is an autoimmune reaction towards GBS bacteria causing scarring and fibrosis of valve leaflets.
Acute - anti-inflam and bed rest, aspirin in high dosage with monitoring. If inflammation doesn’t resolve-> corticosteroids.
Chronic -ACE inhibitors and diuretics if severe HF.
Anti-strep abx if any evidence of persisting infection
Maintenance: monthly injections of benzathine penicillin is is most effective prophylaxis. Most recommend to treat until 18-21.
Rheumatic fever diagnosis
JONES CRITERIA (2 major or 1 major and 2 minor) Major criteria Polyarthritis: large joints, usually starting in the legs and migrating upwards. Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. Erythema marginatum: reddish rash that begins on the trunk/arms as macules -> spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance. This rash typically spares the face and is made worse with heat. (RARE) Sydenham's chorea: series of involuntary rapid movements of the face and arms. This can occur very late in the disease for at least three months from onset of infection. Pancarditis: Myocarditis (Inflammation of the heart muscle- can leads to HF & death), Endocarditis (murmur + valve dysfunction), Pericarditis (pericardial friction rub + pericardial effusion) Minor criteria: Fever, polyarthralgia, hx rheumtaic fever, PR interval prolonged, raised acute phase reactant on FBC
Infective endocarditis management
Most common causative organism is alpha haemolytic strep (strep viridans). It is usually treated with a beta-lactam (e.g. high dose penicillin) and aminoglycoside e.g. genta (broad spec abx for g-ve) IV therapy for 6 weeks.
If the causative organism is Staph Aureus, treat as above + clindamycin.
If there is a prosthetic valves/VSD patches shunts etc. This might have been the cause and there is less chance of eradication- surgical removal might be required.
PROPHYLAXIS - good dental hygiene! strongly encouraged in all children with congenital heart disease. Proph abx no longer recommended. Avoid piercings.
Myocarditis /cardiomyopathy management
RECAP: Can be caused by either direct viral infection, secondary to metabolic disease or inherited - any child with enlarged heart and HF should be suspeted to have cardiomyopathy.
Mx: symptomtic with ACE inhibitors and carvedilol (a Beta-adrenoceptor blocking qagent)
Pul HTN Management
Key is to reduce pressure to prevent irreversible damage to the pulmonary vascular bed (which is not correctable other than herat-lung transplant)
Medications that target GMP pathway (inhaled NO) or cAMP pathway (IV prostacyclin) can cause vasodilation and allow transplant to be delayed for many years. Or endothelin antagonists (e.g. oral bosentan).
Causes of pul HTN
1) Arterial HTN (persisnt pul htn of newborn; post tricuspid shunts e.g. VSD, PDA, AVSD; HIV; idiopathic)
2) Venous HTN (L sided heart disease, pul vein stenosis)
3) Systemic HTN + resp diseae
4) Pulmonary thromboembolic disease
5) Pul. inflammatory or capillary disease
Presentations of congenital heat diseae
- BREATHLESS (or asymptomatic) - ASD, VSD, PDA
- BLUE (R-L shunts) TOF, TGA
- BREATHLESS AND BLUE (Common mixing, AVSD, complex congenital heart disease e.g. tricuspid atresia)
- ASYMPTOMATIC (AS, adult type coarc, PS)
- COLLAPSED WITH SHOCK (Coarcation, HLHS)
What is AVSD?
Complete AVSD:
1) Defect in atrial septum
2) Defect in ventricular septum
These in turn then result in the mitral/tricuspid valve being deformed, and is regarded as having one single five leaflet common valve which stretches across tha triovetircular junction that tends to leak. This results in pul HTN.
upper UTI/pyelonephritis management
NICE GUIDELINES:
<3 mnths - refer to hospital asap, IV abx therapy (eg cef)
> 3 mnths
1. Cefalexin (o) 12.5 mg/kg or 125 mg twice a day for 7 to 10 days (If older than a year its 3x a day)
- If can’t tolerate (o) or severely unwell:
IV Co-amoxiclav (only in combo or if culture results available and susceptible) 30 mg/kg three times a day (max 1.2 g 3 times a day)
IV Cefuroxime 20 mg/kg three times a day (max 750 mg/ dose), increased to 50 to 60 mg/kg three or four times a day (maximum 1.5 g per dose) for severe infections
lower UTI management
NICE GUIDELINES:
If under 3 months -> IV abx therapy (3rd gen ceph e.g. cefotaxime or ceftriaxone) and refer to paed specialist
> 3 mnths - oral abx:
Trimethoprim if low risk of resistance, (25 mg twice a day for 3 days)
Nitrofurantoin if eGFR>45ml/min (750 mcg/kg four times a day for 3 days
If no improvement after 48 hrs ->
Nitrofurantoin if not used as first choice
Amoxicillin (only if culture results available and susceptible, 125 mg three times a day for 3 days)
Cefalexin (125 mg twice a day for 3 days, if over 1yo dose is three times a day)
UTI preventative care & follow up
Good perineal hygiene
High fluid intake
Ensuring complete bladder emptying by encouraging children to try a second time
Regular voiding
Lactobacilllus acidophilus - probiotic that reduces pathogen organism
Abx prophylaxis if under 2 and has congenital abnormality of kidneys (trimethoprim 2mg/kg at night)
Followup for children with reflux, recurrent UTIs or scarring:
Urine culture for every non-specific illness
Circumcision in boys can be considered
Annual BP checks if defects are present
Regular assessment of renal growth and function if bilateral defects.
Nephrotic syndrome mx
Steroid sensitive:
60mg/m2 /day of prednisolone (o). After 4 weeks, reduce to 40mg/m2 on alternate days for 4 weeks and then top. Urine should be protein free by ~11 days.
If steroid resistant, refer to paediatric nephrologist and manage oedema with diuretic therpay, salt restriction, ACE inhibitors.
AKI Ix and Mx
Overall, care is symptomatic and dependent on the cause
- USS to identify obstructive cause, see if it is chronic (small kidneys) or acute (large, bright kidneys with loss of corticomedullary differentiation).
- Use fluid balance charts to monitor intake
- If there is circulatory overload, restrict fluids and challenge with diuretic.
- Metabolic abnormality management:
1. HyperK-> SABA, Ca gluconate if ECG changes, Glucose
and insulin, dietry restriction
2. HyperP -> Calcium carbonate, dietary restriction
3. Met acidosis -> Sodium bicarbonate
4. HUS -> Anti-hypertensives, 50% of kids will need
dialysis
- Dialysis criteria: FISH. Failure (of conservative mx or multisystem failure); Increased bp or pul oedema; Severe (acidosis, hypo/hypernatraemia), Hyperkalaemia
- Post renal failure -> obstruction relief by nephrostomy or bladder catheterisation, surgery can be performed once stable
- If cause not known -> renal biopsy to rule out rapidly proressive glomerulonephritis (if +ve treat immediately with immunosuppressants)
Bacterial meningitis Mx
NICE:
<3 months with ?bacterial meningitis using IV cefotaxime plus amoxicillin or ampicillin
>3 mnths ^^^^ IV ceftriaxone
Treat ?meningococcal disease using IV ceftriaxone.
If has travel hx or prolonged/ multiple exposure to abx (within 3 mnth) with additional VANCOMYCIN.
Give DEXAMETHOSONE (0.15 mg/kg to a max dose of 10 mg, four times daily for 4 days) if LP reveals: purulent CSF, WCC > 1000/microlitre, raised WCC with protein concentration greater than 1 g/litre or bacteria on Gram stain. To prevent long term Cx such as deafness
meningitis & encephalitis causative organisms
BACTERIAL
< 3 months - GBS, Ecoli (and other coliforms), Listeria monocytogens
1 month - 6 years - Haemoph & Neisseria & Strep pneumoniae
> 6 years - Neisseria & Strep pneumoniae
VIRAL:
Enteroviruses, EBV, adenovirus and mumps
ENCEPHALITIS:
Enteroviruses, respiratory viruses and herpes viruses in UK, (worldwide mycoplasma, lyme disease).
Bacterial meningitis, confirmed organism mx
< 3 months:
GBS IV cefotaxime for at least 14 days. If the clinical course is complicated consider extending
L monocytogenes - IV amoxicillin or ampicillin for 21 d in, plus gentamicin for at least the first 7 days.
Gram-ve (Ecoli) bacilli with IV cefotaxime for at least 21 d
> 3 months, unless directed otherwise by abx sensitivities:
H influenzae type b - IV ceftriaxone for 10 days
S pneumoniae - IV ceftriaxone for 14 days
Contraindications to LP
Increased ICP (bradycardia, papilloedema, coma, high BP) Thrombocytopenia Coagulopathy Cardioresp instability Focal neuro signs Local infection at LP site
What can strep pneumoniae cause?
Asymptomatic in majority of children. Pharyngitis, conjunctivitis, sinusitis, otitis media, invasive infection (pneumonia , sepsis and meningitis).
CMV disease
CMV disease may be treated with ganciclovir or
foscarnet, but both have serious side-effects.
Kawasaki causes
Unknown but likely as a result of hyperactivity to variety of riggers in a genetically suscpetible host e.g. a polymorphism in the ITPKC gene- a negative regulatory of T cell-activation on chromosome 19 is strongly associated with susceptibility to the disease.
Kawasaki management
IVIG within first 10 days reduces coronary artery aneurysms, aspirin reduces the risk of thrombosis, given at high anti-inflammatory dose until fever subsides and inflammatory markers return to normal. Then it is continued at allow anti-platelet dose until echo at 6 weeks reveals absence of aneurysms. If there is persistent inflammation ans fever, may require second IVIG or treatment with infliximab (anti-TNF alpha antibody), steroids or ciclosporin.
Down’s
Trisomy 21
- 95% meiotic-nondisjunction, 5% transloc, 1% mosacism
- most common inherited cause of LD
- 1.5 per 1,000 births
- Symptoms: Hypotonia, single palmar crease, low set ears, flat nasal bridge, sandal gap, incurved 5th finger
- 50% live over 50 yo
- Cx: Congenital Heart disease (atrioventricular canal defect)
Ix: Picked up on antenatal scanning. Biochemical markers can indicate higher risk. USS increased nuchal thickness is a risk factor. If positive, amniocentesis for karyotyping can be carried out. Rapid FISH can also be done 24-48hr (can be done on amniotic fluid if urgent)
Edward’s
Trisomy 18
- can be diagnosed by USS in 2nd trimester
- Sx: cardiac and renal malformations, small chin/mouth, LBW, short sternum
- 1 in 8,000
- most die in infancy
Patau’s
Trisomy 13
- can be diagnosed by USS in 2nd trimester
- Sx: C+ R malformations, scalp defect, small eyes, structural brain defect
- 1 in 14,000
- most die in infancy
Turner’s
!! 45 X !!
- > 95% early miscarriage and picked up by fetal neck oedema, hands, feet or cystic hygroma
- Symptoms really vary, can have short stature only or also webbed fingers, lymphoedema in hands/feet of neonate, spoon-shaped nails, widely spaced nipples
- GH therapy or oestrogen therapy for secondary sexual characteristics
Kleinfelter’s
XXY
- Infertility
- Hypogonadism
- Pubertal development may appear normal
- Tall stature
- Gynaecomastia in adolescence
- Although they can face problems during adolescence, often emotional and behavioral, and difficulties at school, most of them can achieve full independence from their families in adulthood & lead a normal, healthy life
AR diseases
Thalassemia Tay-Sachs disease, Ocultunaeous albinism, Phenylketonuria, Friedrich Ataxia, CF, CAH, SCD, SMA-11 (Werdnig-Hoffman), Hurler Syndrome, Poly, Galactosaemia, Glycogen disorder,
AD diseases
Polyposis Choli, Achondroplasia, Tuberous Sclerosis, Ehlers Danlos, Hypercholesterolaemia (familial) Huntington, NeurofibromaTOSIS, Noonan Syndrome, marfan’s, Myotonic dystrophy, Osteogenic Imperfecta, Osteosclerosis
DKA mx acute
To restore circulating volume if systolic blood pressure is below 90 mmHg (adjusted for age, sex, and medication as appropriate), give 500 mL sodium chloride 0.9% by intravenous infusion over 10–15 minutes; repeat if blood pressure remains below 90 mmHg and seek senior medical advice.
When blood pressure is over 90 mmHg, sodium chloride 0.9% should be given by intravenous infusion at a rate that replaces deficit and provides maintenance; see guideline or suggested regimen.
Include potassium chloride in the fluids unless anuria is suspected; adjust according to plasma-potassium concentration (measure at 60 minutes, 2 hours, and 2 hourly thereafter; measure hourly if outside the normal range).
Start an intravenous insulin infusion: soluble insulin should be diluted (and mixed thoroughly) with sodium chloride 0.9% intravenous infusion to a concentration of 1 unit/mL; infuse at a fixed rate of 0.1 units/kg/hour.
Established subcutaneous therapy with long-acting insulin analogues (insulin detemir or insulin glargine) should be continued during treatment of diabetic ketoacidosis.
Monitor blood-ketone and blood-glucose concentrations hourly and adjust the insulin infusion rate accordingly. Blood-ketone concentration should fall by at least 0.5 mmol/litre/hour and blood-glucose concentration should fall by at least 3 mmol/litre/hour.
Once blood-glucose concentration falls below 14 mmol/litre, glucose 10% should be given by intravenous infusion (into a large vein through a large-gauge needle) at a rate of 125 mL/hour, in addition to the sodium chloride 0.9% infusion.
Continue insulin infusion until blood-ketone concentration is below 0.3 mmol/litre, blood pH is above 7.3 and the patient is able to eat and drink; ideally give subcutaneous fast-acting insulin and a meal, and stop the insulin infusion 1 hour later.
Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions
Patau syndrome (trisomy 13)
Learning difficulties Macrocephaly Long face Large ears Macro-orchidism
Fragile X
Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers
Edwards (trisomy 18)
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Noonan
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Pierre -Robin Syndrom
Hypotonia
Hypogonadism
Obesity
PW syndrome
larynx and neurological problems Feeding difficulties and poor weight gain Learning difficulties Microcephaly and micrognathism Hypertelorism
Cri du chat
Characteristic cry (hence the name) due to the larynx/neuro issues
Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis
William’s Syndrome
When do we give kids PPI for gord?
NICE do not recommend a proton pump inhibitor (PPI) or H2 receptor antagonists (H2RA), to treat overt regurgitation in infants and children occurring as an isolated symptom. A trial of one of these agents should be considered if 1 or more of the following apply:
unexplained feeding difficulties (for example, refusing feeds, gagging or choking)
distressed behaviour
faltering growth
False positives for the sweat test for CF test?
malnutrition adrenal insufficiency glycogen storage diseases nephrogenic diabetes insipidus hypothyroidism, hypoparathyroidism G6PD ectodermal dysplasia
Differential for stridor in an infant
- Laryngomalacia
- Laryngeal cyst, haemangioma or web
- Laryngeal stenosis
- Vocal cord paralysis
- Vascular ring
- Gastro-oesophageal reflux
- Hypocalcaemia (laryngeal tetany)
- Respiratory papillomatosis
- Subglottic stenosis
Impetigo Mx NICE
Localised:
Hydrogen peroxide 1% cream (2-3x times daily for 5 days) for people who are not systemically unwell or at a high risk of complications.
If not 3x a day for 5d of a topical abx fusidic acid 2% (if resistance suspected use mupirocin 2%)
Widespread:
Offer cream as above. If not offer oral flucloxacillin for 5 days (62.5–125 mg 4x daily for children aged 1 month to 1 year; 125–250 for aged 2–9 years; 250–500 mg for children aged 10-17 years) (double after 1 year and 10y)
Status Epilepticus Mx
ABC and DEFG (if glucose under <3mmo/L give glucose IV and recheck blood glucose).
If we don’t vascular access, give a diazepam (PR) or midazolam (buccal) 0.5mg/kg as a trial and aim to get IV access and follow below protoco (only give lorazepam once)l. If you can’t obtain IV access, skip to the PR step.
If you have vascular access, you give lorazepam 0.1mg/kg IV. Repeat in 10 min if no reponse. If there is still no response in 10 in we give Paraldehyde 0.4ml/kg PR. If there is still no response in 10 min you call for senior help and give phenytoin 18mg/kg IV/IO over 20 min (unless the pt is on oral phenytoin in which case we give phenobarbital 15mg/kg).Call anaesthetist if there is no response in 20min and transfer to PICU for rapid sequence induction with thipoental.
Anaphylaxis Mx
Call for help + put pt in supine position with legs raised
Adrenaline 1:1000 dose IM.
<6 years - 150micrograms (0.15ml)
6-12 years 300 micrograms (0.3ml)
>12 years 500mcg (0.5ml)
Establish airway and give high flow oxygen
IV Fluids (20ml/kg crystalloids)
Chlorpheniramine (IM or slow IV0
Hydrocortisone (IM or slow IV)
Monitor: pulse ox, ecg and BP
CHOAF - chlorpheniramine, hydrocortisin, o2, adrenaline and fluids
Headache Mx
Rescue Treatments
Analgesia, NSAIDs
Anti emetics -> Metoclopromide and Procholperazine
5-HT1 agonists e.g. Sumatriptan (a nasal prep is licenced for use in children 12+)
Conservative
Therapy, CBT, headache diaries
Prophylaxis (only if v. severe)
Pizotifen (5-HT antagonist) can cause weight gain and sleepiness
Beta blockers - propanolol, contraindicated in asthma
Na channel blockers (valproate)
For cluster: verapamil if >12, subcut sumatriptan if <12
Acute Liver failure Ix and Mx
Ix: Transaminases are greatly elevated (10-100x n), ALP raised, coagulation abnormal, ammonia is elevated. ABG and blood glucose monitoring is crucial. EEG will show the encephalopathy. CT might show cerebral oedema.
Mx: Maintain blood glucose >4mmol/L using IV dextrose, IV Vit K/FFP to prevent haemorrhage, fluid restrict if cerebral oedema. Without transplantation, 70% of children who progress to coma will die.
Cx: Cerebral oedema, haemorrhage from gastritis or coagulopathy, sepsis and pancreatitis
Autoimmune hepatitis Mx
check for other autoimmune signs e.g. rash, AIHA, arthritis.
Presents 7-10 years, girls > boys.
Requires close monitoring.
Ix: Diagnosis is based on hypergammaglobulinaemia (IgG>20g/L), positive antibodies and low C4 serum.
Mx: Prednisolone and azathioprine
Idiopathic intracranial HTN management
Medical Management: acetazolamide
- 2nd line: furosemide, topiramate
Analgesia (e.g. amitriptyline or naproxen) can be offered for persistent headaches
CSF shunting (ventriculoperitoneal shunt) may be used for intractable headache
Paeds Resus
SAFE approach (Shout for help, Approach with care, Free from danger, Evaluate ABC)
Airway & B - Assess (look for obstruction and chest movement, listen for breaths, feel for air movement)
Open airway (head tilt, chin lift gently)
Reassess airway for max 10 seconds
BREATH- 5 x RESCUE BREATHS: pinch nose, mouth to mouse (unless infant in which case mouth over infant’s mouth and nose). Chest should rise.
Reassess - for max 10 seconds, check breathing or pulses (if less than 1 year old check femoral/brachial) if more than 1 year carotid, femoral. Pulse more than 60/min? -> STOP
COMPRESS CHEST - 15 chest compressions + 2 breaths. Infant - two thumbs, Small child - one hand. Large child - two hands.
Tricuspid Atresia Mx
Only the left ventricle is effective, the right being small and non-functional. Presentation depends on whether cyanosis or HF i s more predominant.
Initial medical: Maintain adequate flow through PDA = prostaglandin E1 IV
Cardiorespiratory support = O2 and mechanical ventilation, inotropes, IV fluid
Surgical:
o First stage in neonates:
Early palliation to maintain a secure supply of blood to the lungs at low pressure by Blalock-Taussig shunt insertion (allows mixing) & Pulmonary artery banding operation (reduces pulmonary blood flow + keeps at low pressure).
o Second stage at 3-6m old:
Removal of shunt and direct anastomosis of SVC to Right pulmonary artery = Glenn
o Third stage at 2-5yo:
Direct venous pathway from IVC into pulmonary arteries = Fontan. Needs antibiotic prophylaxis
• Important: complete corrective surgery is not possible in most cases because there is only one functioning ventricle
PPHTN of the newborn
Due to a failure of the pulmonary vascular resistance to fall
PC: Cyanosis soon after birth (grunting etc)
Ix: Urgent echo to establish no CHD
Mx: Mechanical ventilation and circulatory support. Sildenafil (Viagra) is a vasodilator which can be used. ECMO is indicated for severe, reversible cases.
Aetiology: anything that causes respiratory distress e.g. RDS, MAS, pneumonia, also congenital diaphragmatic hernia.
RDS
Surfactant deficiency causes low surface tension, alveolar collapse and inadequate gaseous exchange
PC: develops at birth either immediately or over a few hours while the neonate absorbs the lung fluids with increased resp work, grunting etc
Ix: CXR Ground glass, indistinct heart borders
Mx: CPAP & Pulmonary surfactant (CPAP- give positive pressure to airways continuously as opposed to pushing air in and out). If severe, intubate and give surfactant through endo-trach tube
Cx: Pneumothorax (unequal chest inflation, dip o2 sats, cold light exam -will see a red bit around touch due to space not lung; baby might become bradycardic
NEC
! Pneumostasis Intestinalis - gas cysts in bowel !
Most common life threatening emergency experienced by premature babies
Epi: Occurs in ~15% of LBW prem infants
Path: Essentially, the neonatal gut overreacts to insult and this causes inflammation. In turn, the premature gut reacts unfavourably to the inflammatory mediators and this results in the symptoms. Examples of ‘insult’ are hypoxia and hypoperfusion, enteral feeding with infant formula, and eventually, aberrant colonization of the premature gut, result in injury to the epithelial cells and subsequent intestinal inflammation. (Leaky gut -> slippery slope)
PC: Bell staging criteria can be used to grade severity. Distension, PR bleeding/fresh blood in stool. More severe: R. LQ mass, ascites, absent bs, peritonitis. (Plus all ‘ill’ signs: temperature, lethargy, apnea)
Ix: XR for bowel dilation, ileus, pneumatosis intestinalis (gas cysts in bowel wall). Sometimes air in portal tract / under diaphragm if perforation.
Mx: Stop oral feeding, broad-spec Abx, parentral nutrition, artificial ventilation. S if bowel perforation/necrosis- exploratory laparotomy, bowel resection and osteomy has a fatality of 50%.
Cx: Those who survive are at risk of SHORT BOWEL syndrome (malabsorption due to lack of SI presenting with diarrhoea), parenteral nutrition-associated cholestasis, prolonged neonatal hospitalization, significantly impaired growth, and poor long-term neurodevelopment. As well as failure to thrive
Soft Tissue birth injuries
Caput succedaneum - fluid accumulates subcut, resolves in a few days, due to ‘tourniquet’ effect of cervix and occurs around the presenting part of the scalp, poorly defined margins
Cephalhaematoma - haematoma under the periosteum and so bulge is confined to suture lines. Centre of haematoma feels soft. Resolves over weeks.
Chignon - occurs with ventouse delivery (vacuum device used to deliver baby in second stage of labour if prolonged)
Nerve palsies due to birth injury
Usually ones involving the brachial plexus or facial nerve. May occur at breech deliveries or shoulder dystocia (look for ‘turtle sign’ as an indicator of shoulder dys).
Brachial Plexus - Erb’s palsy, ‘waitiers tip’ C5 and C6 injury, can be accompanied by phrenic nerve palsy resulting in elevated diaphragm
Facial nerve palsy - due to facial nerve compressing again mother’s ischial spine. Unilateral facial weakness e.g. eye remaining open whilst crying
Most palsies resolve completely but should be referred to orthopod or plastic surgeon if not resolved in 2-3months. Most recover by 2 years.
