Severe idiopathic nephrotic syndrome with XXXX (report of nine pediatric cases).

@entity1 @entity8 @entity2002 @entity82 @entity417 @entity177 @entity134 @entity520 @entity10200 @entity1985 @entity715 @entity4434 @entity1371
The association of @entity1985 ( @entity1985 ) with @entity4434 is uncommon. The authors report on 9 pediatric cases (6 @entity1 and 3 @entity1 ). All the @entity1 (age 12 to 48 months) presented initially with a pure @entity1985 . The syndrome was clinically severe and either initially or secondarily resistant to @entity134 and immunosuppressive therapy. In 6 cases initial renal biopsies showed @entity8 and in one case, segmental and focal hyalinization was observed. Symptoms of tubular insufficiency appeared progressively and included: proximal and distal @entity520 (9/9 cases), renal @entity10200 (9/9 cases), @entity1371 with hyperkaluria (7/9 cases), hypohposphatemia (4/9 cases), global hyperaminoaciduria (4/9 cases), tubular proteinura (2/9 cases). Control of water and electrolyte balance was difficult. The urinary concentration of @entity2002 breakdown products was very high throughout the course of the disease. In all cases, @entity417 preceded the inevitable alteration of glomerular filtration which resulted in four cases of @entity177 after an average evolution of 2 to 3 years. Renal biopsies during the later stages of evolution demonstrated five cases of @entity8 with @entity82 associated in 3 cases with histiocytic infiltration. Immunologic studies were negative. The authors discuss the physiopathologic aspects of the disease including the possibility of @entity715 of intrarenal veins and compare their observations with those of Royer et al. [1963].
