Pg 36 Flashcards
What are the most common congenital heart lesions?
- Left-to-right: Breathless / Asymptomatic (VSD)
- Right-to-left: Blue (TOF)
- Mixed: Blue and breathless (AVSD)
- Asymptomatic outflow obstruction (Pulmonary stenosis)
- Outflow obstruction and shock (Coarctation of aorta)
What are the causes of CHD?
Maternal disease or drugs
Congenital infections
Chromosomal
How does CHD present?
- Heart failure
- Cyanosis
- Shock
- Antenatal DX
- Murmur
hallmarks of innocent murmur?
- Positional
- Asymptomatic
- Soft
- Systolic
- LLSB
heart failure causes:
o Neonates: Coarctation
o Infants: Left-right shunt
o Older: Eisenmenger (RHF only), RF, Cardiomyopathy
heart failure sx
o Breathlessness (esp. on feeding)
o Sweating
o Poor feeding
o Recurrent chest infections
heart failure signs:
o FTT o Tachypnea o Tachycardia o Murmur and Gallop rhythm o Enlarged liver, large heart o Cold peripheries o RHF (rare): Oedema, ascites.
causes of peripheral cyanosis?
Cold
unwell
polycythaemia
causes of central cyanosis?
=> check SATS (should be 94 and above)
If baby is well: CHD
Tachypnea (>60) (CHD, Resp, infection)
Small VSD
o Asymptomatic
o Loud murmur
o Closes spontaneously
Large VSD
o HF
o Soft/ no murmur
o Diuretics/ Captopril/ additional calories.
o Surgery at 3-6 months
• DX: XCR, ECG, ECHO
TOF 4 defects
o Large VSD
o Overriding aorta
o Pulmonary stenosis
o RVH
TOF Features:
o Murmur: Loud systolic at ULSB
o Hyper cyanotic spells: Cyanosis, inconsolable crying
o Later: Clubbing
TOF Mgt:
Surgery by 6 months. May use temporary B-T shunt.
Hyper cyanotic spells lasting > 15 mins => Morphine,
muscle paralysis and mechanical ventilation, volume support, Bicarbonate, propranolol
COA
Neonatal collapse at day 2 (duct dependent circulation)
RF delay or absent femoral pulses
Mgt?
ABC
PG infusion
NEURAL type of syncope
Mal-adaptive drop in BP
CARDIAC syncope
investigations
SX on exercise (dangerous)
Palpitations
FHX of sudden death
BP Femoral pulses Marfan ECG corrected QT
JONES criteria for Rheumatic fever?
2 major OR 1 major + 2 minor + Evidence of GAS infection
Major: o Migratory polyarthritis o Carditis o Subcutaneous nodules o Erythema marginatum o Sydenham’s chorea
Minor:
Raised inflammatory markers Fever
Polyathralgia
infective endocarditis
sx:
CE:
fever
anaemia
haematuria
arthralgia
splinter haemorrhage lately clubbing splenomegaly changing cardiac signs Necrotic skin lesions neurological signs retinal infarcts.
infective endocarditis INVX
3 culture samples before antibiotics, acute phase reactants
(ASO/ GAS antigen), cardiac ECHO
infective endocarditis TX:
High dose IV penicillin + gentamycin for 6 weeks + penicillin prophylaxis before dental procedures or any surgery, dental hygiene
most common cardiomyopathy in children?
What causes it?
How is it managed?
Dilated: Mostly viral.
TX: ACEI, B-Blockers, diuretics.
pulmonary hypertension types
o Arterial: Idiopathic/ familial or L-R cardiac shunt o Venous: Left heart disease o Secondary to Lung disease o Thromboembolic events o Vasculitis
pulmonary hypertension TX:
o Heart-lung transplant is definitive
o Drugs to delay transplant (Bosentan, NO2, iloprost)
o Prevent by correcting L-R shunts by 6 months.
What are the main methods of Kidney + UT assessment?
Labs: Plasma U&C, eGFR, PCR
Radio: US, MCUG (reflux) or MAG3, DMSA (Scars)
UTI Factors:
Infrequent/ incomplete voiding, 50% have structural abnormality, Reflux, constipation
UTI Features:
Frequency, urgency, dysuria, Haematuria, pain, fever, rigors, seizures, anorexia, V&V. Septicaemia in infants
UTI INVX:
o Urine dipstick (clean catch/ MSU):
Nitrates more specific: Always treat if present
Leukocyte esterase: Only treat if UTI symptoms
o M&C:
Always unless N and L negative
Diagnostic if 100000 CFU
o US
o MCUG (If abnormal US or anyone < 3 years)
o DMSA (3 M after UTI, for those < 1 year)
UTI Mgt:
Pyelonephritis: IV C-amoxiclav for 4 days=> 1 week of
Trimethoprim.
UTI: PO Trimethoprim for 3 days.
What is VUR?
o Backflow of urine from the bladder into the ureters (mild) or kidneys
(Severe)
o Primary: Short ureter and defective VUJ
o Secondary: Blockage (Posterior urethral valve, recurrent UTI,
Neuropathic bladder)
Presentation: Recurrent UTIs, Pyelonephritis, Hypertension, and CKD.
How is VUR diagnosed and managed?
DX: US, MCUG, DMSA
TX: o UTI prevention: Frequent double voiding, hydration, Perineal hygiene, and probiotics. o UTI Mgt as above o Long-term low dose antibiotics o Monitor: Check urine when unwell o Annual screening for hypertension, proteinuria, and renal growth o Surgery.
causes of enuresis?
o Psychological upset
o Diabetes (Mellitus or insipidus)
o UTI
Enuresis Hx
o Has X ever been dry?
o Are they dry by day? Does X have to run to the toilet? Is he
constantly damp, dripping (Ectopic ureter)?
o Is X drinking more? Loosing weight? Tired?
o Has X had any head trauma? Does X complain of headache,
vision problems?
o Does X having problems passing urine/ peeing more often?
o Is X a happy child?
nephrotic syndrome
Causes:
Clinical features:
Minimal change disease.
Triad:
Oedema
Proteinuria
Hypoalbuminaemia.
nephrotic syndrome
Complications: HIT:
Hypovolemia: Faint
Infections: Peritonitis, septic arthritis, sepsis
Thrombosis.
Hypercholesterolemia
nephrotic syndrome
Steroid sensitive:
Asian boy
Atopy
Previous resp infection.
Normal: BP, Renal function, complement, no gross haematuria, age 1-10.
nephrotic syndrome INVX:
o Urine Dipstick, M & C:
Heavy proteinuria.
Exclude haematuria and infection
Urinary Na (Low = Hypovolemia)
o Bloods: FBC: Thrombocytosis and increased HCT ESR: Vasculitis? U&E, Cr: Kidney function Serum albumin Exclude nephritis: Compliment C3, C4, ASO titres, Anti- DNA-B antibodies, Throat swap Exclude infections: HB, HC, and Malaria.
nephrotic syndrome Mgt:
Volume support: • Fluids +/albumin infusion • Daily weight Steroids: • 60 mg/m2 for a month • 40 mg/m2 for a month/ alternate day • Taper down • No response after a month => renal biopsy (Steroid resistant?). Vaccinate: Pneumococcus, flu
nephrotic syndrome Prognosis:
1/3rd rule
Relapse frequently (steroid dependent),
relapse infrequently
resolve.
haematuria DDX:
Glomerular nephritis
(with proteinuria, IgA nephropathy, Alport,
Thin basement membrane disease)
Non-glomerular (Stones, tumors, trauma, SCD, clotting).
haematuria INVX:
All patients
- Urine microscopy (with phase contrast) and culture
- Protein and calcium excretion
- Kidney and urinary tract ultrasound
- Plasma urea, electrolytes, Creatinine, calcium, phosphate, albumin
- Full blood count, platelets, coagulation screen, sickle cell screen
haematuria INVX
If suggestive of glomerular hematuria:
- ESR, complement levels, and anti-DNA antibodies
- Throat swab and antistreptolysin O/anti-DNAse B titers
- Hepatitis B and C screen
- Renal biopsy if indicated
- Test mother’s urine for blood (if Alport syndrome suspected)
- Hearing test (if Alport syndrome suspected)
causes, clinical features and Mgt of acute nephritis?
o Post-strep, Vasculitis (HSP), IgA nephropathy, Good Pastures
o HSP
o IgA
o Alport: X-linked: Check mother for hematuria, vision and hearing
o All vasculitidies:
o Renal biopsy
o Steroids, immunosuppressant and plasma exchange (ANCA +)
HSP
Fever, palpable rash on extensor surfaces and buttocks (weeks),
Nephritis, Abdominal (pain, hematemesis, Intussisuption), Joint swelling. F/U for a year to detect persistent hematuria and proteinuria.
IgA
HSP-like pathology (Excess IgA, complement activation => inflammation), but limited to kidneys
causes of HTN?
o Renal (stenosis) o Cardiac (Coarctation) o Endocrine (Cushing’s) o Catecholamine excess o Mostly present as Heart and growth failure in infants.
causes of palpable kidneys?
o Polycystic
o Hydronephrosis
o Wilm’s
types of AKI and how is it managed?
o Prerenal: most common cause in children, from hypovolaemia and
circulatory failure.
o Renal: most often haemolytic uraemic syndrome or multisystem failure.
o Postrenal: from urinary obstruction.
Treat underlying cause, metabolic abnormalities, dialysis
CKD Causes:
congenital (structural malformations and hereditary nephropathies) most common.
CKD Presentation:
abnormal antenatal ultrasound, anorexia and lethargy,
polydipsia and polyuria, faltering growth/growth failure, renal rickets
(osteodystrophy), hypertension, proteinuria, anaemia.
CKD Management:
diet and nasogastric or gastrostomy feeding, phosphate restriction and activated vitamin D to prevent renal osteodystrophy, salt
supplements and free access to water to control salt and water balance, bicarbonate supplements to prevent acidosis, erythropoietin to
prevent anaemia, growth hormone, and dialysis and transplantation
CKD grades
Based on eGFR.
• > 90 is stage 1; normal
• < 15% is stage 5: End-stage
• Mild (symptomatic) => moderate (osteodystrophy) => Severe
(metabolic derangements, prepare transplantation). (down by 30).
