Nephrology- Peds Flashcards
CKD- pathophysiology
GFR <60ml/min for >3min
GFR >60ml/min + evidence for structural damage
- albuminuria, proteinuria, pathologic abnormalities on histology or imaging
Staging - if below 1 standard deviation - more concerned
Reversible Causes
Dec Perfusion to Kidneys
- Hypotension
- Volume depletion - vomiting, diarrhea
- Meds that dec kidney perfusion - NSAIDs, ACE, ARB
Nephrotoxic drugs - NSAIDs, contrast, aminoglycosides
CKD- cause
Congenital disease - 60%
- obstructive uropathy
- renal hypoplasia
- Renal dysplasia
- Reflux nephropathy
- PKD
Glomerular disorders - 2nd most common
- > 12yo
- FSGS
- membranoproliferative glomerulonephritis
- Minimal change disease
CKD- S/S & PE
Nonglomerular
- Polyuria
- Elevation in serum creatinine
- poor growth
Glomerular
- Tea colored/colar colored urine
- Edema
- Inc serum creatinine
- Inc BP for age
- systemic findings of concurrent systemic disease that affect kidney function - SLE
Stage 3
- anorexia, N/V, Fatigue
- pericarditis
- bone and mineral disease
- dec neuro function
PE: Growth parameters HTN Hypervolemia Pericardial rub Pallor Deformities or extremities - CKD bone-mineral disorder
CKD- diagnosis
U/S - widely used
- measure size of kidneys against nl values for age
- look for deformities
CKD- labs & imaging
Serum Cr UA Serum Ca Sphphorous Vit D PTH - if suspect bone and minilaral issues
CKD- treatment
Treat reversible kidney dysfunction
Prevent or slow progression
- BP control - ACEI/ARB - HTN + Proteinuria
- Don’t limit protein intake
- No data to support lipid lowering or anemia correction
Treat complications
Identify and prepare kids/families if RRT needed
Mineral/Bone Disease
- growth failure, avascular necrosis, skeletal fractures/deformities/pain, vascular calcification
- Control - PO4, Ca, PTH, VitD
- Tx: diet, binders (sevalemer, Ca, Iron), Vit D2/D3, Vit D analogs
Renal Replacement Therapy (RRT)
- GFR <30 - start preparing family/child
- 1st line - Kidney transplant preferred for best survival
- 2nd line - Paranteral Diaylsis
- 3rd line - Hemodyalisis
- Start before GFR 10-15 if - poor calarie intake, Symptomatic uremia, delay in psychomotor development
CKD- prognosis
Inc M&M Inc Hosp Inc Depression Worse QOL for pts, and family \: More likely unemployed Leading cause of death - CV and infection
Obstructive uropathy- pathophysiology
Stricture
Stenosis
Stones
In posterior urethral valves
Obstructive uropathy- treatment
Refer to Urology
Reflux Nephropathy/ Vesicoureteral Reflux (VUR)- pathophysiology
Retrograde passage of urine from bladder -> upper urinary tract
Renal Dysplasia- pathophysiology
Malformed kidneys
Microscopic level:
- disorganized nephron elements
- Dec # of nephrons
- maldifferentiation of mesenchymal and epithelial elements
- Transformation of tissue to cartilage and bone
Renal Dysplasia- cause
Kidney’s variable in size - usually small
Unilateral or bilateral
Multicystic - nonfunctioning dysplastic kidney w/ multiple cysts
Renal Hypoplasia- pathophysiology
Low number of structural nl nephrons -> small kidneys
Reduce renal size by 2 SD of mean size by age
AND
Exclusion of renal scaring
Renal Hypoplasia- cause
Genetic
No damage or malformations
Renal agenesis- pathophysiology
Congenital absence of renal parenchymal tissue - only have 1 kidney
Renal agenesis- epidemiology
M>F
Renal agenesis- S/S & PE
Asymptomatic Incidental finding on U/S - antenatally - during preg - part of UTI eval - postnatally in dysmorphic kid
Impaired renal function at birth & progressive renal failure
Associated urologic abnormalities - jrenal pelvis, calyces (congenital hydronephrosis), and ureters (Stenosis,VUR, megaureter)
- can lead to complications - UTI, hematuria, fever, abdominal pain
Non renal anomalies - malformations of heart, genitals, bones, GI tract, respiratory tract
Renal agenesis- diagnosis
Ranal U/S
Frequent association of dysplasia w/ a collecting system anomaly
- get voiding cystourethrography as well
Renal agenesis- treatment
Monitor
Transplant/RRT
Focal Segmental Glomeruloscerosis (FSGS)- pathophysiology
Most common Glomerular disorder
Sclerosis in parts/segments of 1 glomerulus in entired kidney biopsy
Focal Segmental Glomeruloscerosis (FSGS)- cause
Genetic
Injury to podocytes and/or direct toxicity from drugs or viral infections
2nd to - obesity, HTN, HIV, chronic urinary reflux, analgesic or bisphospbonate exposure
Focal Segmental Glomeruloscerosis (FSGS)- S/S & PE
Acute onset - Most common
- Peripheral Edema
- Hypoalbuminemia
- Proteinuria - >3.5 g/day
Dec GFR
ESRD - develop in 6-8 yr
Focal Segmental Glomeruloscerosis (FSGS)- diagnosis
Renal biopsy
Genetic testing
Focal Segmental Glomeruloscerosis (FSGS)- treatment
Diuretics - edema
ACEI/ARB - HTN/proteinuria
Statin/niacin - HLD – controversial
Prednisone
Immunosuppressants - cyclosporine, tacrolimus
Plasma exchange - prior to renal transplant - lower risk of graft loss
- good for those who are about to relapse
Membranoproliferative Glomerulonephritis- pathophysiology
Pattern of glomerular injury on biopsy w/ characteristic changes on light microscopy
Rare
Membranoproliferative Glomerulonephritis- cause
Immune - complex mediated
Complement mediated - less common
Membranoproliferative Glomerulonephritis- S/S & PE
Spectrum of nephritidies:
- asymptomatic glomerular hematuria
- Gross hematuria
- Rapidly progressive glomerulonephritis
Membranoproliferative Glomerulonephritis- diagnosis
Renal biopsy
Membranoproliferative Glomerulonephritis- labs & imaging
Cr - nl or inc
Complement levels - low
Hematuria -dysmorphic red cells and red cell casts
Proteinuria - variable
Membranoproliferative Glomerulonephritis- treatment
Mild - ACEI/ARB
Severe - cyclophosphamide or MMF + steroids or rituximab
ESRD - develop in most even w/ treatment
Kidney transplant - still may recur
Minimal Change Disease- pathophysiology
Most common nephrotic syndrome
Minimal Change Disease- cause
Idiopathic After Viral URI Neoplams - Hodgkin Meds - lithium Hypersensitivity reaction
Minimal Change Disease- S/S & PE
Nephrotic syndrome symptoms
Even though inc fluid vol -> will have s/s of dec effective circulating volume
- tachy, peripheral vasoconstriction, oliguria, dec GFR, inc plasma renin, aldosterone, and norepinephrine
Susceptible to infection - gram +
Tendency toward thromboembolic events
Severe hyperlipidemia
Minimal Change Disease- diagnosis
Clinical
Biopsy - rare
- no changes on light microscopy
- electron microscopy - effacement of podocyte foot processes
Minimal Change Disease- treatment
Prednisone
- 4m to respond
- cont several weeks after proteinuria is resolved
Cyclophosphamide or Retuximab
- resistant to steroids or relapse
Prog to ESRD - rare
Horseshoe Kidney- pathophysiology
Most common kidney fusion anomaly
One pole of each kidney fuses to the other - 5-9w gestation
1/2 have another congenital anomaly
- urological or genital
- syndromes - turner, trisomny 13, 18, 21
Inc risk for Wilms tumor
Horseshoe Kidney- S/S & PE
Asymptomatic
Pain and/or hematuria - obstruction or infection
Hydronephrosis - 80%
Renal calculi - 20%
Inc inf risk - inc urinary stasis and impaired drainage
Horseshoe Kidney- diagnosis
Incidental
Horseshoe Kidney- labs & imaging
Cr
U/S
Voiding cystourethrogram
Horseshoe Kidney- treatment
Good prognosis w/out intervention
VUR - prophylactic abx for prevention of UTI
Obstruction - refer to urology