Pediatric Renal Disease Flashcards
CAKUT
Congenital Abnormality of the kidneys/urinary tract
-decreased nephron mass at birth: long term risk HTN/CVD
Bilateral Renal Agenesis
Death shortly after birth
Lack of urine output means lungs do not develop (no amniotic fluid)
Kidney Dysplasia
Decreased kidney function: abnormal cells
Increased risk for CKD
Multicystic dysplastic kidney
Non functional, unilateral cyst
Often corrects on its own
Horseshoe Kidney
Kidneys form lower in the pelvis than is normal
Increased occurrence of reflux, UTI, kidney stoneos
Hydronephrosis
Most common**
Uteropelvic junction obstruction/stenosis
Kidney cannot empty (unilateral)
Swollen, distended kidney
Primary vesicourectic reflux
Backflow into kidneys of urine
Nephrocalcinosis
Calcifications in the kidney, typically resolve spontaneously (may need diuretic to help flush)
Renal Vein Thrombosis
Blood cannot get OUT of the kidney
Causes: diabetic mother, birth asphyxia, premie, polycythemia
Sx: wet lung disease, acute renal failure, chronic renal disease
Tx: kidney transplant
Fanconi’s Syndrome
Proximal Tubular reabsorption disease
Cystinosis
Lysosomal storage disease leading to proximal tubulopathy, hypothyroidism, growth failure
Suspect if CORNEAL CRYSTALS
Chronic Kidney Disease
Uncommon, most due to congenital causes
Most common renal mass
Simple cysts
Abnormal ureteral bud leads to
Multicast if dysplasic kidney disease (non functional kidneys)
Tuberous sclerosis
Cause of cystic kidney disease, also has neuro SX and skin changes
Autosomal dominant
Von hipped Lindau disease
Autosomal dominant
Associated with cancers
Genetic basis of adpkd
*PKD 1and 2
Genetic basis arpkd
Phhd1: ciliary function is impaired
Potters sequence
Typical appearance of child with low amniotic fluid: Widely spaced eyes Broad nasal bridge Receding chin *pulmonary hypoplasia
How does ciliary dysfunction cause cysts?
Leads to epithelial proliferation and subsequent fluid accumulation within expansion
Causes of pediatric renal cystic disease?
Genetic: ADPKD, tuberous sclerosis, Von hipped-Lindau disease, ARPKD, nephronophthisis
Acquired: simple, sinus cysts, chronic renal impairment
Developmental: medullary sponge kidney
Features of ARPKD
Large cystic kidneys Microcysts (CCD) Hepatic fibrosis No extra renal cysts HTN
Nephronophthisis
Genetic recessive kidney disease, occurs in Hutterites
Huge urine volume because cannot concentrate urine
Medullary cystic disease
Genetic kidney disease
No extra renal manifestations
May have gout
Clinical features of ADPKD
Cysts in all parts of the nephron
Increased kidney volume
SX in 40-50s HTN concentrating defects, proteinuria, hematuria Stones, infections Pain due to large kidneys
Extra renal manifestations*** berry aneurysms Hepatic, pancreatic, splenic cysts Heart valves abnormal Hernias, diverticula
Therapy ADPKD
Vasopressin receptor antagonists
MTOR inhibitors
Somatostatins
Control HTN (ACEI)
