Alport Syndrome

Category: generalpediatrics-Nephrology

Findings: sensorineural hearing loss, vision loss, renal disease, hematuria

Notes: X-linked dominant. Males have more issues other than renal compared to females.

Autosomal Dominant Polycystic Kidney Disease

Category: generalpediatrics-Nephrology

Findings: most common inherited primary renal disease, Berry aneurysms

Benign Orthostatic Proteinuria

Category: generalpediatrics-Nephrology

Findings: proteinuria in adolescent without hypertension, no family history of renal disease

Notes: Proteinuria seen when upright and not when supine

Fanconi Syndrome

Category: generalpediatrics-Nephrology

Findings: polyuria, polydipsia, failure to thrive, hyponatremia, disorder of proximal renal tubules resulting in loss of many electrolytes

Fibromuscular Dysplasia

Category: generalpediatrics-Nephrology

Findings: new-onset hypertension in adolescent female, renal artery stenosis, abdominal bruit, headaches

Hemolytic Uremic Syndrome (HUS)

Category: generalpediatrics-Nephrology

Findings: fever, hemolytic anemia, schistocytes, thrombocytopenia, neurologic changes, renal dysfunction, uremia, hematuria, normal complement levels

Notes: Do not treat with antibiotics as this may worsen the disease by releasing more bacterial toxins

Question: What fits these findings?

IgA Nephropathy

Category: generalpediatrics-Nephrology

Findings: hematuria, RBC casts, occurring after recent viral infection, normal complement levels, IgA and IgG deposits

IgA Nephropathy

Category: generalpediatrics-Nephrology

Findings: hematuria, hypertension, concurrent viral illness

Notes: IgA nephropathy occurs concurrently or days after illness while PSGN occurs weeks earlier.

Question: What fits these findings?

Liddle Syndrome

Category: generalpediatrics-Nephrology

Findings: hypertension, metabolic alkalosis, hypokalemia, low aldosterone, runs in families

Notes: Due to epithelial sodium channel (ENaC) abnormality. Resistant to medication but may be treated with low salt diet. Bartter and Gitelman syndrome also presents with metabolic alkalosis and hypokalemia, but patients will have low to normal blood pressure.

Question: Which disease is associated with these findings?

Membranoproliferative Glomerulonephritis (MPGN)

Category: generalpediatrics-Nephrology

Findings: hematuria, proteinuria, hypertension, tram track lesions on biopsy, sometimes associated with systemic lupus erythematosus

Posterior Urethral Valves

Category: generalpediatrics-Nephrology

Findings: bilateral hydronephrosis in a boy, weak urinary stream, associated with Prune Belly syndrome

Poststreptococcal Glomerulonephritis (PSGN)

Category: generalpediatrics-Nephrology

Findings: proteinuria, hematuria, swelling, hypertension, history of skin or throat infection, low C3, normal C4

PPSV23

Category: generalpediatrics-Nephrology

Findings: nephrotic syndrome

Question: What additional vaccine should people with the following condition receive?

Thin Basement Membrane Nephropathy

Category: generalpediatrics-Nephrology

Findings: multiple family members with hematuria without RBC casts, no history of kidney failure, autosomal dominant, usually benign

Notes: Also known as benign familial hematuria. Usually benign but continue to monitor as it may progress to chronic kidney disease.

Thrombotic Thrombocytopenic Purpura (TTP)

Category: generalpediatrics-Nephrology

Findings: fever, hemolytic anemia, renal failure, severe neurologic changes, thrombocytopenia, decreased ADAMTS13, thrombi in brain, presentation in teenager

Ureteropelvic Junction (UPJ) Obstruction

Category: generalpediatrics-Nephrology

Findings: hydronephrosis, dilated renal calyces, oligohydramnios, most common cause of urinary obstruction