peds nephro Flashcards
causes of nephrotic syndrome in first year of life
primary
1. congenital syndrome of the finnish type
2. mesangial sclerosis
3. associated with other genetic syndromes
4. minimal change
secondary
- congenital infections - syph, CMV
features of congenital syndrome of the finnish type
- thrombosis
- malnutrition - peeing protein
causes of nephrotic syndrome after the first year of life
main one is minimal change diease
patho of minimal change disease
normal under light microscope
- some fusion of foot process under electron microscope
features that suggest a diagnosis other than idopathic minimal change (require biopsy)
- age 12 years
- pos. fam Hx
- extrarenal disease
- chronic disease
- Sx due to intravasc. volume expansion
- renal failure
- RBC casts
Tx of minimla change
high dose prednisone - 95% respond in 4 weeks - control edema - low salt diet repeat with relapses, which are common
possible complications
- infections
- hypercoag
- fluid and lyte changes
- mech prblems due to edema
- complications of steroids
outcome for steroid responsive diseasae
very good
- no chronic kidney disease
what to do when not resnosive to steroids
- biopsy of kidney
- poor outcome - CKD
3 signs of nephritic syndrome
- hematuia
- HT
- renal dysfunction
RBC casts
2 main causes of nephritic in children
- hypocomplementemic (low c3)
2. normocomplement
3 primairy and 1 systemic cause of hypocomplementic
primary 1. post-infectous 2. membranoproliferative 3. C3 nephropathy systemic 1. SLE
2 primary and 3 systemic causes of normocomplementemic
primary 1. IgA nephropathy 2. ANCA systemic 1. ANCA+disease 2. goodpastures - anti GMB 3. henoch schonlein
what is post-infectious glom nephritis
clinical diagnosis
- previous strep infection 10-14 days after
- impetego - 3 weeks following
- nephritic syndrome
- low C3
Tx of post strep
conservative Tx - fluid restriction anti-hypertensives diuretics NOT steroids
outcome in post-strep
back to normal in 6-8 weeks
95% spont. resolve.
what is hemolytic uremic syndrome
disease of younger children presenting with acute kidney injury
triad of HUS
- microangiopathic hemolytic anemia
- thrombocytopenia
- AKI
2 major forms of HUS
- typical or diarrhea positive
- E.coli - food poisoning - atypical
- genetic abnromality
HUS presentation
- prodrome of vomiting abd bloody diarrhea
- later simultaneous microangiopathic hemolytic anemia
HUS prevention
prevent food poisoning and clean water
- nothing to do once infected
what are outcomes from triad of HUS
- microangiopathic hemolytic anemia
- can be severe anemia and need transfusion - thrombocytopenia
- can have very low counts and risk of bleeds - AKI
- variable
up to 50% will need dyalisis
what is mech of HUS
damage to the endothelial cells by the TOXIN
- arteriolar and cap. microthrombi
HUS tx
symptomatic
- AKI
- blood transfusions for severe anemia
- platelet transfusions
- treat other organs
HUS outcomes
mort - 5% in acute phase
morb -
5%severe renal sequelae
5-25% have some renal sequelae
