Proteinuria and Lipodystrophy

Hereditary proteinuria syndromes and mechanisms of proteinuria.

N Engl J Med 2006;354:1387-401. Copyright © 2006 Massachusetts Medical Society. The inherited forms of proteinuria comprise a heterogeneous group of rare renal diseases in which glomerular dysfunction and proteinuria are prominent. Despite the rarity of hereditary proteinuria syndromes, genetic, biochemical, and structural studies of these diseases have made important contributions to our knowl...

Partial lipodystrophy and dermatomyositis.

In 1978, an 11-year-old boy began to notice proximal weakness in his limbs together with facial erythema; palpebral violaceous edema; and erythematous papules on his elbows, knees, and over the metacarpophalangeal joints. A diagnosis of polymyositis/dermatomyositis was made. He was treated with oral prednisone, which caused the symptoms to disappear promptly. In 1980, proximal weakness, dysphag...

Lipodystrophy, pancreatitis, and eosinophilia.

Two patients suffering from partial lipodystrophy, pancreatitis, and recurrent eosinophilia are described. In one patient the duodenum and the terminal ileum were narrowed, the appearances suggesting eosinophilic gastroenteritis: bilateral hydronephrosis was also present without ureteric obstruction. An association between lipodystrophy and renal disease is recognized; it is possible that there...

Lipodystrophy progressiva and pregnancy.

Generalized Lipodystrophy.