Renal biopsy, the introduction of immunohistologic methods and electron microscopy have allowed the differentiation of clinicopathologic entities associated to nephrotic syndrome. Two main categories must be differentiated: in the first, diffuse lesions of the glomerulus, including those secondary to specific diseases the same as those that are apparently primary, are responsible for the increased permeability of glomerular capillaries. Any one of the following clinical signs suggests this category: acute onset with nephritic syndrome, moderate nephritic syndrome, moderate nephrotic syndrome, gross hematuria, persistent hypertension and/or renal failure, poor selectivity of proteinuria and drop in complement serum levels (C3). In the second category, known as idiopathic nephrotic syndrome, the mechanism of disorder of the glomerular capillary is unknown and the nephrotic syndrome is more marked. In most cases with idiopathic nephrotic syndrome, minimal glomerular lesions (MGL) are present. The clinicopathologic correlation among these three types of lesions shows that the type with MGL is characterized by selective proteinuria, absence of hematuria, good response to corticosteroids and good outlook; whereas in types with diffuse mesangial proliferation (DMP) and segmentary sclerosis, proteinuria is frequently non selective, hematuria shows in 50 to 75% of the patients; prognosis is poor. However, MGL, DMP and focal segmentary glomerular sclerosis are not different entities, but represent variants of the same disease. Considering that corticosensitive nephrosis to this moment is the most common cause of the nephrotic syndrome, especially in children under 8 years, renal biopsy should be done only under two circumstances: a) when the clinical symptoms suggest diffuse glomerular lesions and b), when resistance to corticosteroids becomes evident.

Adrenal Cortex Hormones↗

PubMedDate not supplied

[Partial lipodystrophy, hypocomplementemia and glomerulonephritis].

Renal involvement is found in 20 to 50% of cases of Partial lipodystrophy (PLD). We report 8 cases of PLD of which 6 had a glomerular nephropathy and 2 had no renal disease but all had persistent hypocomplementemia and 5 had circulating nephritic factor (C3NeF). The analysis of these cases and of all the cases reported in the literature shows the specificity of the glomerular involvement. Membranoproliferative glomerulonephritis (MPGN) with dense intramembranous deposits is a constant finding in PLD with renal involvement. This variety of MPGN is well known for being associated with persistent hypocomplementemia. However, the presence of hypocomplementemia and C3NeF in patients with PLD but without nephritis raises the question of the interrelationship between alternative pathway complement activation and the development of MPGN with or without lipodystrophy. There is no valid explanation in the present state of knowledge for the association of partial lipodystrophy hypocomplementemia, and MPGN. From the answer to this problem should emerge a better understanding of the role of complement in renal disease and in particular in the unusual form of glomerular injury seen in MPGN.

Adolescent↗