Paediatric Nephrology Flashcards
What are features of congenital glomerulopathy?
- rare
- podocyte cytoskeletal integrity
- proteins- podocin (AR), nephrin (AR)
- basement membrane proteins
- alport syndrome (XL)
- thin basement membrance disease (AD)
- endothelial/microvascular integrity
- complement regulatory proteins (MPGN)
What are the features of minimal change disease (nephrotic syndrome)?
- 2-5 yrs
- normal BP
- resolving microscopic haematuria
- normal renal function
- only consider renal biopsy when:
- suggestions of autoimmune disease
- abnormal renal function
- steroid resistance
What are side effects of high dose glucocorticoids?
- varicella status
- pneumococcal vaccination
- behaviour
- Cushing’s syndrome
What is the usual outcome of idiopathic nephrotic syndrome in childhood?
- remission
- 95% in 2-4 weeks
- relapse
- 80%
- long term remission
- 80%
- steroid toxicity + steroid resistance
- steroid dependant relapses
What are features of steroid resistant nephrotic syndrome?
Aquired:
- focal segmental glomerular sclerosis (FSGS)
- podocyte loss
- progressive inflammation + sclerosis
Congenital:
- infant presentations
- NPHS1- nephrin
- NPHS2- podocin
- podocyte loss
What are features of acute post-infectious glomerulonephritis?
- usually group A strep
- beta haemolytic
- throat 7-10 days
- skin 2-4 weeks
- bacterial culture
- ASOT +ve
- low C3
What is the treatment for acute post-infective glomerulonephritis?
- antibiotics
- support renal function
- diuretics
What are some common causes of CKD in children?
- congenital abnormalities of the kidney and urinary tract (CAKUT)
- 55% of paediatric CKD
- reflux nephropathy
- dysplasia
- obstructive uropathy
- hereditary conditions
- 17%
- cystic kidney disease
- cystinosis
- glomerulonephritis
- 10%
What are the signs of UTI in neonates?
- fever
- vomiting
- fever
- lethargy
- poor feeding
- failure to thrive
- abdominal pain
- jaudice
- haematuria
- offensive urine
What are the signs of UTI in children (pre-verbal)?
- fever
- abdominal/loin pain/tenderness
- vomiting
- poor feeding
- lethary
- irritability
- haematuria
- offensive urine
- failure to thrive
What are the signs of UTI in children (verbal)?
- frequency
- dysuria
- dysfunctional voiding
- changes to continence
- abdominal/loin pain/tenderness
- fever
- malaise
- vomiting
- haematuria
- offensive/cloudy urine
How are urine specimens obtained?
- clean catch urine, MSSU
- collectio. pads
- urine bags
- catheter samples
- suprapubic aspiration
What is vesico-ureteric reflux?
- wrong direction of flow of urine
- grades 1-5
What is the treatment for UTIs in children?
Lower tract:
- antibiotics, 3 days
Upper tract/pyelonephritis:
- antibiotics, 7-10 days
What are features of developmental multicystic dysplastic kidney (DMDC)?
- 1 in 2000/4000
- usually sporadic
- non-functioning kidney
- ureteric atresia
- hypertrophy of normal contralateral kidney
- usually unilateral 75%
What are features of ARPKD and Potter’s sequence?
- antenatally large bright kidneys
- oligohydramnios
- severe respiratory distress
- pulmonary hypoplasia
- nephromegaly mass effect
- severe end of spectrum
- Potter’s sequence
- dec. amniotic fluid
- pulmonary hypoplasia
- fetal compression (faces, contracture)
- bilateral renal agenesis (absent ureteric bud)
- AR polycystic kidney disease (truncating mutation)
What are the target organs affected by different renal cystic diseases?
- ARPKD
- renal collecting duct, hepatic ductal plate
- ADPKD
- all nephron segments, liver, pancreas, brain
- nephronophthisis
- tubular (medullary) cysts, retina, liver, brain, bone
What are features of ciliopathy syndromes?
- renal cysts on US
- cystic tubular dilation on renal biopsy
- retinal pigment inc.
- cerebellar vermis aplasia, molar tooth sign
What are features of renal cysts and diabetes (RCAD)?
- autosomal dominant
- US- cortical cysts
- early onset diabetes mellitus (MODY)
- genetic heterogeneity
- HNF1- B mutations
What are features of Alport syndrome?
- GBM disease
- collagen 4 abnormalities
- X-linked dominant inheritance
- COL4A5 gene on X chromosome
- less common AR and AD inheritance
- haematuria
- proteinuria
- hypertension
- deafness- high tone sensori-neural loss
- renal failure in early adult life (20-30 yrs)
- lenticonus, macular changed in retina
