Pediatric Nephrology: Paulson Flashcards
What counts as pediatric CKD?((According to KDIGO)
Must meet one of the following:
- GFR <60 ml/min for >3 months
- GFR >60 ml/min + evidence for structural damage (like albuminuria, proteinuria, pathologic abnormalities on histology or imaging)
Stages of Chronic Kidney disease
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Pediatric CKD: causes
-list 2
- Congenital disease: 60% of cases
- Glomerular disorders: 2nd most common cause
Pediatric CKD: describe Congenital disease
Obstructive uropathy Renal hypoplasia Renal dysplasia Reflux nephropathy PKD
Pediatric CKD: describe Glomerular Disorders
-More common in kids >12 years
FSGS
Membranoproliferative glomerulonephritis
Minimal change disease
Presenting S/S of Pediatric (nonglomerular) CKD
Polyuria
Elevation in serum creatinine
Poor growth
Presenting S/S of Pediatric glomerular CKD
Tea-colored or cola-colored urine–>Look for hematuria, RBC casts
Edema
Elevation in serum creatinine
Elevated BP for age
Systemic findings indicative of a concurrent systemic disease that can affect kidney function (ie: SLE)
Things to look for on exam
Growth parameters Hypertension Hypervolemia Pericardial rub Pallor (anemia) Deformities of extremities from CKD-caused bone-mineral disorders
Diagnostics: best studies to use
-**Ultrasound-Most widely used—>Measure size of kidneys against normal values for age. *Look for deformities
Serum creatinine
UA may be helpful
Serum calcium, phosphorous, 25-hydroxyvitamin D, and PTH helpful if you suspect abnormalities in bone and mineral metabolism
General Management of Pediatric CKD
Treat reversible kidney dysfunction
Prevent or slow progression
Treat complications of CKD
Identify and prepare kids/families if RRT will be needed
Reversible Causes of CKD
-Decreased perfusion to kidneys:
Hypotension
Volume depletion
Medications that ↓ kidney perfusion
-Nephrotoxic drugs:
NSAIDs, contrast materials, aminoglycosides, as a few examples
How to Slow CKD Progression
-BP control:
ACEI/ARB preferred for kids with HTN + proteinuria
- Differences from adults:
- -Don’t limit protein intake
- No data to support lipid lowering therapy or anemia correction in kids
Symptoms for CKD in peds, usually start around CKD stage ___
-list other Sx
Start around CKD stage 3 Anorexia Fatigue N/V Pericarditis Bone and mineral disease ↓ neurocognitive function
Management of MBD(mineral bone disease):
- Sx/clinical manifestations: list Ex’s
- Tx: diet and ____
Mineral/Bone Disease:
- Growth failure
- Avascular necrosis
- Skeletal fractures/deformities/pain
- Vascular calcification
- Control PO4, Ca, PTH, 25D
Tx: diet, binders (Sevalemer, Calcium, Iron), Vitamin D2/D3, Vitamin D analogs
Renal Replacement Therapy (RRT):
-initiated once GFR is less than ___
-Once GFR <30, start preparing the child/family
- **Kidney transplantation is the preferred treatment for best survival and growth outcomes
2nd: PD (peritoneal dialysis)
3rd: HD (hemodialysis) - RRT often started earlier than GFR 10-15 because:
- Poor calorie intake –> FTT (failure to thrive)
- Symptomatic uremia
- Delay in psychomotor development
CKD prognosis:
- Higher morbidity & mortality
- Increased hospitalizations
- Increased depression
- Worse QOL for patients, their parents, and their siblings
- More likely to be unemployed
- Leading causes of death are CV disease and infection
Obstructive Uropathy:
- causes?
- tx?
- Stricture
- Stenosis
- Stones
- Posterior urethral valves
- ->Refer to urology
Reflux Nephropathy (Vesicoureteral reflux)(VUR)=
=Retrograde passage of urine from bladder–> upper urinary tract
-these kids are at higher risk for UTIs–> leads to renal scarring and..
Please refer to Brian Miller’s powerpoint for a review of this topic
Renal dysplasia=
- kidneys may be variable in ____
- Unilateral or bilateral?
=Malformed kidneys
- Kidneys may be variable in size, but most are smaller than normal
- May be unilateral or bilateral
Renal dysplasia:
-on a microscopic level:
- Disorganized nephron elements
- Decreased number of nephrons
- Maldifferentiation of mesenchymal & epithelial elements
- Transformation of tissue to cartilage and bone
Multicystic dysplasia=
a nonfunctioning dysplastic kidney with multiple cysts
Renal hypoplasia=
=Low number of structurally normal nephrons –>= a small kidney
- ->Reduction of renal size by 2 SD for the mean size by age –and-
- ->Exclusion of renal scarring (done by DMSA radionucline scan)
Renal hypoplasia: etiology
- Thought to be genetic
- No damage or malformations
Renal agenesis=
Congential absence of renal parenchymal tissue
Renal agenesis:
- demographic?
- Sx?
- Males»_space; females
- Most asymptomatic: Solitary kidney is usually an incidental finding on an ultrasound–> Antenatally or as part of UTI eval
Renal agenesis:
-Associated nonrenal anomalies (list)
Malformations of heart, genitals, bones, GI tract, respiratory tract
Renal agenesis:
-Presentation?
- May be discovered on routine antenatal screening –or-
- Postnatally in a dysmorphic kid
- May have:
- -Impaired renal function at birth & progressive renal failure
- -Associated urologic abnormalities of renal pelvis, calyces (congential hydronephrosis), and ureters (stenosis, VUR, megaureter)
- –Can lead to symptomatic presentation from complications of the above: UTI, hematuria, fever, abdominal pain
Renal Agenesis:
-Dx?
- Renal ultrasound
- Frequent association of dysplasia with a collecting system anomaly–> consider voiding cystourethrography as well
Renal Agenesis: tx?
- Monitoring for progression
- Transplant/RRT
Glomerular disorders: (list 3)
- FSGS
- Membranoproliferative glomerulonephritis
- Minimal change disease
Focal Segmental Glomerulosclerosis=
Sclerosis in parts (segmental) of at least 1 glomerulus (focal) in the entire kidney biopsy specimen when examined histologically
Focal Segmental Glomerulosclerosis:
-etiology?
Can be from:
Genetics
Injury to podocytes and/or direct toxicity from drugs or viral infections
Secondary causes: obesity, HTN, HIV, chronic urinary reflux, analgesic or bisphosphonate exposure
Focal Segmental Glomerulosclerosis:
-MC presentation?
Most commonly: acute onset of nephrotic syndrome
- Peripheral edema
- Hypoalbuminemia
- Proteinuria (usually >3.5 g/day)
FSGS:
____ GFR in about half of patients
decreased
FSGS:
What do these Pts usually develop by 6-8 yrs?
ESRD
FSGS:
-diagnosis made by**?
**Diagnosed by renal biopsy
May do genetic testing
FSGS Treatment
Diuretics for edema
ACEI/ARB for HTN/proteinuria
Statin/niacin for HLD
Prednisone or immunosuppressants (ie: cyclosporine, tacrolimus)
Plasma exchange helpful prior to renal transplant to lower risk of graft loss
Also helpful in those who appear like they are about to relapse
Membranoproliferative Glomerulonephritis= pattern of ____
glomerular injury on renal biopsy with characteristic changes on light microscopy
–>Relatively rare
Membranoproliferative Glomerulonephritis: Variety of causes, 2 major causes are:
- Immune-complex mediated
- Complement-mediated
Membranoproliferative Glomerulonephritis:
Clinical Presentation
Anywhere on the spectrum of nephritidies:
Asymptomatic glomerular hematuria to
Gross hematuria to
Rapidly progressive glomerulonephritis (RPGN)
Membranoproliferative Glomerulonephritis: dx?
Hematuria, often with dysmorphic red cells and red cell casts
Variable amount of proteinuria
Creatinine may be normal or elevated
Low complement levels
*Diagnosis by renal biopsy
Membranoproliferative Glomerulonephritis: tx of mild diseases?
ACEI/ARB
Membranoproliferative Glomerulonephritis: tx of severe diseases?
Cyclophosphamide or MMF + steroids –or- rituximab
- Despite therapy, ESRD will develop in most
- May do kidney transplant…but may recur afterwards
Minimal Change Disease (MCD)= MC cause of _____
nephrotic syndrome in kids
Can be: Idiopathic After a viral URI Associated with neoplasms (ie: Hodgkin disease) From meds (lithium) Hypersensitivity reactions
Minimal Change Disease: Clinical Findings
-Nephrotic syndrome symptoms
Even though there’s an increase in extracellular fluid volume, some kids will present with s/s decreased effective circulating volume:
Tachycardia, peripheral vasoconstriction, oliguria, decreased GFR, elevation of plasma renin, aldosterone, and norepinephrine
More susceptible to infection
Have a tendency toward thromboembolic events
Develop severe hyperlipidemia
- or question on horse shoe kidney
Ex: pance question: child with nephrotic syndrome (or proteinuria and edema etc) what is the cause of this? MCD
Minimal change disease: Dx?
- Clinical diagnosis:Biopsy rarely done–No changes seen on light microscopy
- -On electron microscopy, effacement of podocyte foot processes is seen
Minimal change disease: tx?
Treated with:
- Prednisone–>Can take up to 4 months to respond
- Continue for several weeks after proteinuria is completely resolved
- Cyclophosphamide or rituximab –>If resistant to steroids or for relapse
- Progression to ESRD is rare
Horseshoe kidney= MC kidney _____
- fusion anomaly
- One pole of each kidney fuses to the other–> Usually between weeks 5-9 of gestation
Horseshoe Kidney:
-up to half of these PTs have ?
Up to half have another congenital anomaly:
- -Urological and genital anomalies
- -Syndromes: Feature of Turner syndrome, and Trisomy 13, 18, and 21
Horseshoe kidney:
-these Pts are at increased risk for ____ tumor
*Wilms (Please reference oncology slides)
Horseshoe Kidney: Sx?
-Most patients are asymptomatic & diagnosed incidentally
-Some have pain and/or hematuria from obstruction or infection
80% get hydronephrosis
20% with renal calculi
Increased infection risk (from ↑urinary stasis) and impaired drainage
Horseshoe Kidney: Labs?
Evaluate with:
Creatinine
Ultrasound
Voiding cystourethrogram
Horseshoe Kidney: tx/prognosis?
- Most have excellent prognosis without any intervention
- If they have VUR (vesicoureto reflux) –> consider prophylactic antibiotics for prevention of UTI
- If obstruction present –> refer to urology
