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Progressive Renal Failure in a 24-Year-Old Man

  • A detailed family history obtained at presentation revealed moderate hearing loss in several relatives, including first-degree cousins, but none reported in his immediate family—mother, father, and 2 sisters.

    Of note, the patient was held back from sports at age 14 years because he had had hypertension and mild proteinuria, although it was never quantitated. He had required corrective lenses since age 5 years because of myopia.

    A 24-hour urinalysis showed a total volume of 350 ml/24 hrs, 3226 mg protein/24 hrs, and creatinine clearance of 4 mL/min. Results of an echocardiography scan showed normal left ventricular systolic function (50%-55%), concentric left ventricular heart function, and mild to moderate mitral regurgitation.

    There was no bloodwork conducted prior to this ED visit. The last urinalysis was conducted at age 14 years, results of which showed 1+ protein and 1+ red blood cells. Results of a renal ultrasonography scan showed atrophic kidneys, with the left at 6.3 cm in length (reference range, 10-12 cm), the right at 6.9 cm in length, and thinned cortices. No hydronephrosis was noted.


    Answer and discussion on next page.