In May 2002, a 59-year-old male was admitted to our hospital because of extreme fatigue during the last week and elevated levels of urea and S-creatinine on biochemical tests. His past history included mild proteinuria since 5 years (last measurement 1,35 gr/day), cardiomyopathy since 7 years (he received verapamil treatment), chronic obstructive pulmonary disease (on inhalation therapy) since 5 years and partial deafness with tinnitus since 10 years. His family history was positive for renal disease: his mother had suffered from cardiomyopathy, deafness and chronic kidney disease (CKD) of unknown origin. On admission he did not report abnormalities of diuresis or fever.
Physical examination was regular except for reddish macular-papular skin lesions in the abdomen (which were reported since the age of 30 years) and blood pressure of 160/80 mmHg.
Laboratory examinations showed serum creatinine of 4,4 mg/dl (please provide SI units), BUN of 80.9 mg/ (please provide SI units),and moderate anaemia. In the urinary sediment 20-25 RBC/hmf (please explain) were found. 24-hour urinary protein excretion was 800 mg. A renal ultrasound revealed small, shrunken kidneys and a cardiac echo a thickening of the interventricular septum of 1,99 cm and a left ventricular outflow gradient of 67 mmHg. Ophthalmologic examination revealed tortuosity and ''sausage-like'' anomalies of the conjunctival and retinal vessels.