A hemodialysis patient with congenital solitary kidney and various anomalies.

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A hemodialysis patient with congenital solitary kidney and various anomalies is presented. This 51-year-old woman was admitted to our institution because of hypertension and proteinuria which were noticed for the first time 9 years ago. Family history was significant, in which her father died of kidney disease and four brothers had deafness. Physical examination on admission revealed slight disturbance of growth, mental retardation, teleangiectasia of face, left strabismus divergens, atrophy of retina, perceptive deafness and auditory hallucination. Blood pressure was 160/110 mmHg. Abnormal laboratory findings were as follows : proteiuria 1 g/day, BUN 65 mg/dl Creatinine 5.6 mg/dl, plasma renin activity 0.2 ng/ml/h, plasma aldosterone concentration (PAC) 53.7 ng/dl. The left kidney was not visualized on both renoscintigraphy and abdominal computed tomography. The adrenal glands were normal in size on I-131-adosterol scintigraphy. Proline was not high in the plasma aminogram. Aortography showed hypoplastic right renal artery and non-visualized left renal artery. The left ureteral orifice was missing on cystoscopy. In summary, congenital solitary kidney with various other anomalies is the most likely diagnosis in this case, although familial nephritis can not be completely ruled out. High blood pressure might be related to high PAC. No case such as this has ever been reported in the literature.
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