Pediatric Nephrology Flashcards
When does a pediatric patient count as CKD?
GFR less than 60 for more than 3 months
GFR greater than 60 + evidence of structural damage like (albuminuria, proteinuria, patholgoic abnormalities on histology or imaging)
Pediatric CKD most common cause? 2nd?
Congenital disease MC
Glomerular disorders 2nd most common.
Obstructive uropathy, renal hypoplasia, renal dysplasia, reflux nephropathy, and PKD are examples of?
Congenital kidney disease
FSGS, membranoproliferative glomerulonephritis, and minimal change disease are examples of?
Glomerular disorders.
What are the presenting s/s of pediatric (nonglomerular) CKD? (3)
Polyuria
Elevation in serum creatinine
Poor growth
Tea-colored or cola colored urine, edema, elevation in serum creatinine, elevated BP for age, systemic findings indicative of concurrent systemic disease that can affect kidney function like SLE.
These are presenting s/s of?
Pediatric glomerular CKD
What are diagnostic tests for pediatric CKD?
Ultrasound is most widely used - measures size of kidneys against normal values for age, looks for deformities - size shape etc.
What lab values can you use for diagnosis of pediatric CKD?
Serum creatinine
UA
Serum calcium, phosphorus, 25-hydroxyvitamin D, PTH. - if you suspect abnormalities in bone and mineral metabolism.
What is the general management of pediatric CKD?
Treat reversible kidney dysfunction
Prevent or slow progression
Treat complications
Identify and prepare kids/families if RRT will be needed.
What are reversible causes of kidney disease?
Decreased perfusion to kidneys (hypotension, volume depletion, medications that decrease kidney perfusion)
Nephrotoxic drugs - NSAIDs, contrast materials, aminoglycosides
How do you slow CKD progression in kids? How is this different from adults
BP control in kids - ACEi/ARB preferred for kids with HTN and proteinuria.
Difference is don’t limit protein intake and no data to support lipid lowering therapy or anemia correction in kids.
What are symptoms in pediatric CKD? When do they appear?
Appear in CKD stage 3
Anorexia, fatigue, n.v, pericarditis, bone and mineral disease, decrease in neurocognitive function.
What is the management of mineral bone disease in pediatric CKD?
Control phosphate, calcium, PTH and vit D levels
Treat with diet, binders (sevalemer, calcium, iron) vitmain D2/3, Vitamin D analogs.
People with pediatric CKD and mineral bone disease are likely to suffer from?
Growth failure, avascular necrosis, skeletal fractures/deformities/pain, vascular calcification.
When do you begin RRT in pediatric CKD?
Generally earlier than 10-15, start preparing family/kid at GFR<30
Start earlier due to poor calorie intake –> FTT, symptomatic uremia, delay in psychomotor development.
What is the preferred treatment for best survival and growth outcomes in pediatric CKD?
Kidney transplant
2nd is peritoneal dialysis followed by hemodialysis.
Leading cause of death in pediatric CKD?
CV disease and infection.
Reflux Nephropathy (vesicuretral reflux) is descibed as?
Retrograde passage of urine from the bladder to the upper urinary tract.
Renal dysplasia =
Malformed kidneys
Microscopic level findings of disorganized nephron elements, decreased number of nephrons, maldifferentitation of mesenchymal and epithelial elements, and transformation of tissue to cartilage and bone is described as?
Renal dysplasia.
In Renal dysplasia what are the size of kidneys?
Variable in size, but most are smaller than normal.
Multicystic dysplasia, a nonfunctioning dysplastic kidney with multiple cysts is what kind of kidney disease?
Renal dysplasia.
What describes a low number of structurally normal nephrons leading to a small kidney? This is a genetic disorder with no actual damage or malformations.
Renal hypoplasia
Renal hypoplasia can lead to a reduction in kidney size of?
2 standard deviations for the mean size by age
Exlcusion of renal scarring is necessary for what diagnosis?
Renal hypoplasia
What is a congenital absence of renal parenchymal tissue?
Renal agenesis
Males>>>Female
A kid with a UTI had his kidneys ultrasound to find a solitary kidney. This is an incidental finding of?
Renal agenesis.
Renal agenesis is associated with what non-renal anomalies?
Malformations of heart, genitals, ones, GI tract, respiratory tract.
How does Renal agenesis present?
May be discovered on routine antenatal screening or postnatally in a dysmorphic kid.
May have impaired renal funciton and progressive kidney failure. Abnormal urologic findings leading to symptomatic presentation of UTI, hematuria, fever, abdominal pain.
How do you diagnose Renal agenesis?
Renal ultrasound - frequent assocaited of dysplasia with a collecting system anomaly
Should consider getting a voiding cystourethrography as well.
How do you treat renal agenesis?
Monitor for progression, transplant/RRT
Sclerosis in parts (segmental) of at least 1 glomerulus (focal) in the entire kidney biopsy specimen when examined histologically is?
Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis can be caused by?
Genetics
Injury to podocytes -drugs or viral infections.
Secondary causes - HTN, obesity, HIV etc.
FSGS most commonly has an acute onset of?
Nephrotic syndrome
Proteinuria, edema, hypoalbuminemia.
In FSGS about half of patients have a decreased GFR with ESRD that develops in _______ years.
6-8 years.
Treatment for FSGS?
Diuretics for edema,
ACEi ARB for HTN/proteinuria
Statin/niacin for HLD
Prednisone or immunosuppressants (cyclosporine, tacrolimus)
Plasma exchagne helpful prior to renal transplant to lower risk of graft loss.
What is a pattern of glomerular injury on renal biopsy with characteristic changes on light microscopy?
Membranoproliferative glomerulonephritis.
Membranoproliferative glomerulonephritis presents by?
Anywhere on spectrum of nephritidies
Asymptomatic glomerular hematuria to gross hematuria, to rapid progressive glomeruloneprhitis (RPGN)
How do you diagnose membranoproliferative glomerulonephritis?
Diagnosis by renal biopsy.
Hematuria, often with dysmorphic red cells and red cell casts.
Variable amount of proteinuria
Creatinine may be normal or elevated.
Low complement levels.
How do you treat mild membranoproliferative glomerulonephritis?
ACEi/ARB
How do you treat severe membranoproliferative glomerulonephritis?
Cyclophosphamide or MMF + steroids or rituximab.
Despite therapy, ESRD will develop in most. May recur after kidney transplant.
What is the most common cause of nephrotic syndrome in kids?
Minimal chagne disease
What causes minimal change disease?
Idiopathic
Viral URI
Associated neoplasms
Meds - Lithium
Hypersensitivity reactions.
What are the clinical findings of minimal change disease?
Nephrotic syndrome symptoms
Even though there’s an increase in extracellular fluid volume, some kids will present with s/s of decreased effective circulating volume: (tachycardia, peripheral vasoconstriction, oliguira, decreased GFR)
T/F kids with minimal change disease are more likely to have thrombotic events, more susceptible to infection, and develop severe hyperlipidemia.
True
How do you diagnose minimal change disease?
Biopsy - rarely done - so its a clinical diagnosis
Under biopsy there is no changes on light microscopy - (minimal change)
On electron microscopy - effacement of podocyte foot process is seen.
How do you treat minimal change disease?
Immunosuppression - prednisone - can take 4 months to respond. Continue for several weeks after proteinuria has resolved
Cyclophosphamide or tixuimab - if resistant to steroids for replapse.
What is the most common kidney fusion anomaly?
Horseshoe kidney
What occurs in horseshoe kidney?
One pole of each kidney fuses together in week 5-9 of gestation.
What syndromes are associated with horseshoe kidney?
Turner syndrome, Trisomy 13 18 21
Kids with horseshoe kidney are at increased risk for what tumor?
Wilms tumor.
How do you diagnose horseshoe kidney?
Most are asymptomatic and diagnosed incidentally
Some have pain and/or hematuria from obstruction or infection
80% of people with what malformation get hydronephrosis. 20% get renal calculi.
Horseshoe kidney
How do you evaluate horseshoe kidney?
Creatinine
Ultrasound
Voiding cystourethrogram
What is treatment for horseshoe kidney?
Most have excellent prognosis without intervention
If they have VUR - consider prophylactic antibiotics as they are at increased risk for infection
If obstruction is present –> refer to urology
