A 14-year-old boy with persistent proteinuria (1.6–4.0 g/day), microscopic haematuria, moderate hypertension, macrothrombocytopenia (giant platelets, platelet number 30×109/l) and a familial sensorineural hearing loss (the father and the brother were also affected) was studied. Kidney biopsy revealed a diffuse mesangial proliferation, and a focal thickening of the glomerular basement membrane was seen on electron microscopy. A normal number of megakaryocytes was observed in bone marrow aspirates. The aggregation response of the platelets to collage, epinephrine and adenosine diphosphate (ADP) was decreased. The platelet number was slightly diminished, platelets were of normal size in both parents and the brother, and showed a decreased aggregability in response to collagen, epinephrine and ADP in the brother and mother. No functional abnormality of the platelets was observed in the father. Urinalysis and kidney function were normal in the family members. This boy with nephritis, platelet disorders and hearing loss corresponds to Epstein's syndrome.

Hereditary nephritis, platelet disorders and deafness - Epstein's syndrome

Fundamentals.- Nephron- and Collecting Duct Structure in the Kidney, Rat.- Cellular Mechanisms of Renal Calcium Transport.- Factors Influencing Renal Calcium Excretion.- Ca2+ Antagonists - Mode of Action and Pharmacodynamics.- Nephrocalcinosis and Calcium Antagonists.- Pathological Anatomy, Etiology, and Pathogenesis of Nephrocalcinosis.- Clinical Aspects of Nephrocalcinosis.- Nephrocalcinosis in the Kidney of the Rat on Atherogenic Diet and the Effect of Calcium Antagonists (Nifedipine).- Influence of Calcium Antagonists (Nifedipine) on the Excretion of Calcium and Other Substances Relevant for Stone Formation in Rats with Nephrocalcinosis (Induced by Atherogenic Diet).- The Unique Chemical Composition of Nephrocalcinosis in Experimental Renal Insufficiency, Disturbances of Cellular Calcium Metabolism, and Protective Effect of Verapamil Against Nephrocalcinosis.- Calcium Antagonists in Acute Renal Failure and in Hypertension. Calcium Antagonists in Ischemic Acute Renal Failure (Improvement of Function and Morphologie Damage by Nisoldipine).- Effect of Calcium Antagonist Diltiazem on Acute Renal Failure: Experiments on Animals and Clinical Studies.- Renal Function in Hypertensive Patients and Calcium Entry Blockers.

Nephrocalcinosis Calcium Antagonists and Kidney

Since the first description of nephrocalcinosis (NC) by Virchow in 1855, not much attention has been paid to this disease from a morphological point of view; only a few fundamental light and electron microscopic studies are available (Randall and Melvin 1937; Jaccottet 1957; Caulfield and Schrag 1964; Anderson 1982), but the physiology and pathophysiology of the renal handling of calcium have evoked increasing interest.

Pathological Anatomy, Etiology, and Pathogenesis of Nephrocalcinosis

Glomerular Spherical Electron Dense Deposits in a Patient Undergoing Long-Term Regional Heparinization

Concretions in renal basement membranes.

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Concretions in renal basement membranes.