SM 217a - Pediatric Nephrology Flashcards
What are the 4 most common CAKUT diagnoses?
- Hydronephrosis (most common)
- +/- obstruction, vesicoureteral reflux (VUR)
- Renal cysts
- Renal dysplasia
- +/- hydronephrosis and/or cysts
- Renal agenesis
How do the adult CVD mortality causes in CKD differ from the pediatric CVD mortality causes in CKD?
- Adults
- CAD + CHF
- Intimal calcifications, atherosclerosis
- Pediatric
- Arrhythmias
- Valve diseases
- Cardiomyopathy
- Cardiac arrest
-
Medial calcification
- Diffuse, non-occlusive stiffening of vessels
- Less likely to have preceding warning symptoms before a fatal event
What factors contribute to growth failure in CKD?
- Age at CKD onset
- Primary renal disease
- -> Metabolic acidosis
- Malnutrition
- Oral aversion
- Catabolic state of uremia/acidosis
- Feeding intolerance is common
- Anemia
- Kidneys cannot produce erythropoietin
- Iron homeostasis problems
- Renal osteodystrophy
- Growth hormone/IGF axis perturbation
What are the cognitive and psychosocial impacts on pediatric CKD patients?
- Negative effects on neurocognitive ability
- Decreased quality of life measures
- Anxiety and depression
- Short stature
- Associated wtih low physical, social, and QOL measures
- GH treatment is associated with improvement in these measures
How are mineral bone disorders in pediatric patients treated?
- Dietary phosphorous restriction
- Phosphorous binders
- With every meal/snack
- Calcitriol to treat secondary hyperparathyroidism
- Be careful to avoid hypercalcemia, worsening hyperphosphatemia
What causes aplasia?
Failure of the ureteric bud to induce the metanephric mesenchyme
Why do children require higher doses of rHuEPO than adults for the same effect?
Kids have more non-hematopoietic EPO bidning sites
Which complication of CKD is unique to the pediatric population?
- CKD-MBD (mineral bone disorder)
- Anemia
- Hypertension
- Growth Impairment
- Psychosocial impairment
d. Growth Impairment
How is growth failure in CKD treated?
- Correct metabolic abnormalities
- Intesive nutritional support
- Enough sodium intake is important!
- Low threshold for tube feed/G-Tube placement
- Growth hormone therapy
- After addressing the above
How do the recommendations for sodium intake differ in pediatric and adult CKD patients?
Adults: low sodium diet recommended
Kids: Positive Na+ balance is important for growth, so adequate sodium intake is recommended
What is vesicoureteral reflux (VUR)?
- Retrograde flow of urine from the bladder to the ureters
- Normally prevented by a functional valve at the ureterovesicular junction
- Primary VUR
- Congenital deficiency of insertion site/angle into the bladder wall
- Secondary VUR
- Associted with bladder outlet obstruction, neurogenic bladder, chronic bladder inflammation/infection, or trauma
What are the possible consequences of reflux?
- UTI
- Renal scars
- Hypertension
- CKD (UTI is a symptom of worse problems, not necessarily a cause of CKD)
When in gestation do the first glomeruli form?
9-10 weeks
What is the cut-off for a normal protein-to-creatinine spot ratio in the urine?
0.2
How do the causes of CKD differ between adult and pediatric patients?
- Pediatric
- 36% of ESKD is from CAKUT
- Adults
- DM and HTN are common causes
Why is CVD so common in pediatric CKD patients?
- High prevalence of CVD risk factors
- Dyslipidemia
- Abnormal glucose metabolism
- BMI > 95th percentile (15% of pediatrick CKD pts)
- Increased obesity prevalence 1 year after kidney transplant
- CKD-related risk factors for CVD
- Increased Calcium-phosphorous product
- Anemia
- Hyperparathyroidism
Why is important to treat mineral bone disorders in pediatric CKD patients?
- Future bone health
- Impacts final adult height
- Treatment can slow progression of CVD
What is the most common CAKUT diagnosis?
Hydronephrosis
May resolve over time, may not
What is the number of RBCs to be seen on a microscopic urinalysis that can be considered/defined as microscopic hematuria?
>5 RBCs per high powered field (RBC/hpf)
A 16 year old boy with no past medical history presents with clearly chronic, end-stage kidney disease (ESKD) of unknown etiology. Which is the most likely underlying diagnosis?
- ADPKD
- Renal Dysplasia
- ARPKD
- Lupus Nephritis
- Diabetic Nephropathy
b. Renal Dysplasia
Which acute GNs present with low complement levels?
Post-infectious GN
MPGN
Lupus nephritis
Outgrowth of the _____ forms the ureteric bud
Outgrowth of the mesonephric/wolffian duct forms the ureteric bud
What are the most common sites of urinary tract obstruction?
Uretopelvic junction (UPJ)
Uretovesical junction (UVJ)
Posterior urethral valves (PUV)
CAKUT comprises ______% of major birth defects
CAKUT comprises 20-30% of major birth defects
A 7 year old boy presents with brown urine, hematuria and proteinuria on UA, and hypertension. Complement level C3 is low. What is the most likely diagnosis?
- Lupus nephritis
- Post-infectious glomerulonephritis
- Membranoproliferative glomerulonephritis (MPGN)
- IgA nephropathy
- Focal segmental glomerulosclerosis (FSGS)
b. Post-infectious glomerulonephritis
Why is the transition from pediatric to adult care a high-risk period for CKD patients?
- Deficits in risk judement in late teens/early 20s
- High rates of adolescent non-adherence to treatment
- 42% of patients with a functioning graft at age 17 lose their graft by age 24
How does anemia develop in pediatric CKD patients?
CKD ->
- Decreased erythropoietin production
- Shortened RBC lifespan with higher BUN levels
- Iron dysregulation
- CKD = difficult to tolerate oral iron supplements
How does the differential change for painless vs. painful hematuria?
Painless => Kidney or glomerularnephritic cause
Painful => Urologic cause
Above what number is a protein-to-creatinine ratio considered to be in the nephrotic range?
2-3
What are the most common causes of CKD in children?
- Aplasia/hypoplasia/dysplasia =16%
- Obstructive Uropathy = 15%
- Focal Segmental Glomerulosclerosis =12%
- Reflux Nephropathy =5%
- Polycystic Kidney Diseases = 3%
- Other Chronic GN= 3%
For comparison, in adults:
- Diabetic nephropathy
- HTN
- ADPKD (usually does not present in childhood)
What acute GNs present with normal complement levels?
IgA Nephropathy
ACNA-accociated GN (Pauci-immune)
How do mineral and bone disorders develop in patients with CKD?
- Malfunctioning kidney
- -> Increased FGF-23
- -> Decreased 1,25-Hydroxy-D3 (calcitriol) production
- -> Decreased Ca2+ absorption
- -> Increased PTH
- -> Increased bone resorption
- Phosphorous retention
What is the leading cause of death in the pediatric CKD population?
CVD
What is the most common renal vasculitis seen in kids?
Henoch Schonlein Purpura (HSP)
aka IgA Vasculitis
How does the management of febrile UTI differ in a child vs. an adult?
- Adult
- Prescribe antibiotics
- Child
- Prescribe antibiotics + Renal ulrasound + VCUG
- Need to evaluate possible urinary obstruction
- Especially caused by proteus, enterococcus, klebsiella, or coagulase-negative staphylococcus
- Infections caused by e. coli are less likely to cause obstruction
A prenatal ultrasound reveals that the fetus has 2 kidneys that are both measuring large for gestational age and are echogenic. What is the most likely diagnosis?
- Multicystic Dysplastic Kidney
- ADPKD
- ARPKD
- Renal Agenesis
- Hydronephrosis
c. ARPKD
