Internal Medicine

0521779407-C04 CUNY1086/Karliner 0 521 77940 7 June 14, 2007 20:37

458 Cystinuria Cystitis and Pyelonephritis

Cystinuria..........................................

MICHEL BAUM, MD

history & physical

■History of severe flank pain, may have history of gross hematuria

■Recurrent renal stones – usually start in second and third decade

tests

■Urinalysis with hexagonal cystine crystals, hematuria

■Urinary cystine >250 mg/g creatinine

differential diagnosis

■Autosomal recessive disorder due to defect in dibasic amino acid

transporter resulting in increased urinary excretion of lysine, argi-

nine, ornithine, and cystine

■Distinguish from other causes of nephrolithiasis.

management

n/a

specific therapy

■Fluid intake

■Alkalinize urine to pH 6.5–7.0

■Chelation with Thiola or penicillamine

follow-up

■To assess response to cystine excretion and complications of therapy

■To assess renal function

■Sonograms looking for new stones

complications and prognosis

Renal insufficiency results from recurrent stone formation and repeated

interventions.

Cystitis and Pyelonephritis.............................

GARY SINCLAIR, MD

history & physical

■It is essential to distinguish between lower urinary tract infections

(cystitis) and upper urinary tract infections (pyelonephritis).