Welcome to the ACH PIG Challenge! To answer the questions below please post a comment (which will be sent to us but not published) or email ACHPIGChallenge@gmail.com . We will post answers in May, when the new case is published. You will be placed in a drawing to win an Atlas of Pediatric Physical Diagnosis by Zitelli and Davis every time you participate!!
Jimmy is a 5 yo little boy who is brought to the ER by his mother because he “looks puffy” and has been tired and short of breath lately. About 1 week ago, he had cough, rhinorrhea, sore throat, and fever, which have since resolved. Being an astute medical student, you suggest a renal function panel (electrolytes, BUN, Cr, Albumin, Phos) and a urinalysis as initial work-up. See the 2 scenarios below:
Scenario A- Physical exam is notable for peri-orbital edema, abdominal distension, lower extremity edema, and scrotal edema, mildly diminished breath sounds at bilateral bases; Vital signs: HR 100, RR 22, T 36.5, BP 104/68, Wt 20 kg; Lab: Na 135, K 4, Cl 105, HCO3 24, BUN 11, Cr 0.5, Glucose 85, Albumin 1.7, Phos 5; UA is unremarkable except for protein of 3+
1. What additional labs would you like?
spot urine protein to creatinine ratio (preferrably first morning void) or 24 hr urine collection to quantitatively measure protein excretion (though it is difficult to obtain accurately timed collections in young children); cholesterol, triglycerides, C3, C4
2. After appropriate diagnostic work-up, Jimmy’s urine protein is found to be 2 grams/day (100mg/kg/day). What is your diagnosis? What is the most common form?
– nephrotic syndrome- Nephrotic syndrome is diagnosed by fulfilling two defining characteristics: urine protein excretion (nephrotic range proteinuria) >50 mg/kg per day, and hypoalbuminemia defined as serum albumin <3 g/dL (30 g/L).
– in children, the most common form is minimal change disease(idiopathic nephrotic syndrome)
3. What is your treatment plan?
glucocorticoid treatment (prednisone)- children with idiopathic nephrotic syndrome will further be classified as glucocorticoid-sensitive (the majority) or glucocorticoid resistant (about 20% will not respond to glucocorticoid and have a worse prognosis)
Scenario B- Physical exam notable for generalized edema and mildly diminished breath sounds at bilateral bases; Vital signs: HR 100, RR 22, T 36.5, BP 136/92, Wt 20kg; Lab: Na 135, K 4, Cl 105, HCO3 24, BUN 28, Cr 1.7, Glucose 85, Albumin 3.5, Phos 5; UA is remarkable for 1+ protein, 3+ blood (urine is coca-cola/tea colored), Small amount of RBC casts, WBC 35
1. What additional lab tests would you like?
streptozyme measures the following 5 antibodies: Anti-streptolysin (ASO), Anti-hyaluronidase (AHase), Anti-streptokinase (ASKase), Anti-nicotinamide-adenine dinucleotidase (anti-NAD),Anti-DNAse B antibodies- or you can get these tests individually.
C2, C3, C4, CH50, throat culture for strep (only + in 25% of cases b/c after antecedent strep pharyngitis infxn), skin culture in pt’s with impetigo, may also get ANA (if >10yo or clinical symptoms suspicious of lupus)
2. What is your diagnosis? What is the most common etiology/form?
– nephritic syndrome- The clinical findings of acute nephritis include hematuria with or without red blood cell casts, variable degrees of proteinuria, edema, and hypertension;
– most common etiology = post-strep glomerulonephritis (C3, CH50 are usually low in Post-strep GN, but in lupus nephritis C3 and C4 are low))
3. What is your treatment plan? Assuming that he does have the most common etiology, what should you tell mom and dad about prognosis?
supportive care; focused on manifestations of disease, particularly complicatioms from volume overload i.e. hypertension and pulmonary edema- diuretics (lasix (furosemide)) for edema (generalized and pulmonary); usually self-limited disease- diuresis begins within 1 week and serum creatinine returns to nml within 3-4 weeks; hematuria can last 3-6 months, increased protein excretion may last years. Most patients, particularly children, have complete clinical recovery/excellent prognosis, and resolution of their disease process begins within the first two weeks. However, there is a small subset of patients who have late renal complications (ie, hypertension, increasing proteinuria, and renal insufficiency).
