Cystinuria (NORD)

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High Yield Notes

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Flashcards

Cystinuria (NORD)

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Questions

USMLE® Step 1 style questions USMLE

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A 13-year-old girl comes to the emergency room with abdominal pain. The pain was sudden in onset and localized to the right lower abdomen.  She has had blood in her urine and 2 episodes of vomiting since this morning. She is not sexually active and does not smoke, use illicit drugs or consume alcohol. Temperature is 37.0°C (98.6°F), pulse is 96/min, respirations are 20/min, and blood pressure is 125/85 mmHg. Physical examination reveals nodulocystic acne. Abdominal examination is notable for tenderness in the right flank. Routine urinalysis is positive for blood and 5-10 erythrocytes/hpf. A noncontrast CT detects a staghorn calculus in the right proximal ureter with mild dilation of the pelvic calyxes. Urine cyanide nitroprusside test is found to be positive. Which of the following crystals are likely to be found on urinalysis?

External References

First Aid

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Arginine

cystinuria p. 83

Cystinuria p. 83

Lysine

in cystinuria p. 83

Ornithine

cystinuria p. 83

Summary

Cystinuria is an inherited metabolic disorder that causes the body to accumulate large amounts of cysteine. Too much cysteine in urine can precipitate into crystals and renal stones, leading to associated symptoms like renal colic (intense pain in the lower back flanks and iliac fossae), hematuria, urinary tract obstruction which increases the risk of infections, and possible damage to the kidneys due to obstruction of the urinary tract.

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