1/18. Post-traumatic anterior pituitary insufficiency developed in a patient with partial lipodystrophy.A case of partial lipodystrophy developing anterior pituitary insufficiency, chronic glomerulonephritis and pulmonary fibrosis was reported. The patient died of respiratory failure secondary to pituitary crisis during the hospital course. From the clinical course in recent several years and the postmortem examination the head injury following car accident in the past history was considered to be the most plausible cause of hypopituitarism. The etiology of pulmonary fibrosis remained unresolved.- - - - - - - - - - ranking = 1keywords = glomerulonephritis (Clic here for more details about this article) |
2/18. Partial lipodystrophy and renal disease.Partial lipodystrophy is a rare disorder with both autosomal recessive and familial forms. The cutaneous findings, which are often subtle, consist of a gradual loss of subcutaneous fat from the face and upper body. Low levels of C3, occasionally low C5 and the presence of the nephritic factor help to identify these patients. Associated systemic abnormalities include the development of mesangiocapillary glomerulonephritis and an increased incidence of autoimmune diseases. Recognition of this unusual disorder is essential for diagnosis and treatment of underlying potentially life- threatening disease.- - - - - - - - - - ranking = 573.3243855639keywords = mesangiocapillary glomerulonephritis, mesangiocapillary, glomerulonephritis (Clic here for more details about this article) |
3/18. Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins.The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.- - - - - - - - - - ranking = 13.078924584562keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis (Clic here for more details about this article) |
4/18. Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature.We describe clinical features, body fat distribution, and prevalence of metabolic abnormalities in 35 patients with acquired partial lipodystrophy (APL) seen by us over 8 years, and also review 220 cases of APL described in the literature. Based on the review and our experience, we propose that the essential diagnostic criterion for APL is the gradual onset of bilaterally symmetrical loss of subcutaneous fat from the face, neck, upper extremities, thorax, and abdomen, in the "cephalocaudal" sequence, sparing the lower extremities. Analysis of the pooled data revealed that female patients were affected approximately 4 times more often than males. The median age of the onset of lipodystrophy was 7 years. Several autoimmune diseases, in particular systemic lupus erythematosus and dermatomyositis, were associated with APL. The prevalence rates of diabetes mellitus and impaired glucose tolerance were 6.7% and 8.9%, respectively. Approximately 83% of APL patients had low complement (C) 3 levels and the presence of polyclonal immunoglobulin C3 nephritic factor. Twenty-two percent of patients developed membranoproliferative glomerulonephritis (MPGN) after a median of approximately 8 years following the onset of lipodystrophy. Compared with patients without renal disease, those with MPGN had earlier age of onset of lipodystrophy (12.6 /- 10.3 yr vs 7.7 /- 4.4 yr, respectively; p < 0.001) and a higher prevalence of C3 hypocomplementemia (78% vs 95%, respectively; p = 0.02). The pathogenesis of fat loss and MPGN in patients with APL remains unclear, but activation of an alternate complement pathway has been implicated. Treating the cosmetic disfigurement by surgical procedures has yielded inconsistent results. The use of thiazolidinediones to treat fat loss in patients with APL remains anecdotal. prognosis is mainly determined by renal insufficiency due to MPGN.- - - - - - - - - - ranking = 8.0789245845622keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis (Clic here for more details about this article) |
5/18. Mesangiocapillary glomerulonephritis type 2 associated with familial partial lipodystrophy (Dunnigan-Kobberling syndrome).The lipodystrophies are a heterogeneous group of disorders of adipose tissue associated with insulin resistance. The sporadic form of partial lipodystrophy, characterised by fat loss from the face and upper body, is associated with complement abnormalities and mesangiocapillary glomerulonephritis type 2 (MCGN II) and the conditions are thought to have a shared autoimmune aetiology. We present the first case of the rare familial form of partial lipodystrophy, caused by a mutation in the LMNA gene, associated with MCGN II. This suggests that partial lipodystrophy of both the sporadic and familial subtypes may predispose to this condition and that the observed renal and complement abnormalities may be secondary to other factors associated with lipodystrophy.- - - - - - - - - - ranking = 577.3243855639keywords = mesangiocapillary glomerulonephritis, mesangiocapillary, glomerulonephritis (Clic here for more details about this article) |
6/18. Proteinuric nephropathy in acquired and congenital generalized lipodystrophy: baseline characteristics and course during recombinant leptin therapy.Generalized lipodystrophy is characterized by adipose tissue absence, hypoleptinemia, hypertriglyceridemia, insulin resistance, diabetes, hepatomegaly, and nonalcoholic steatohepatitis. In the course of recruiting patients for treatment with recombinant leptin, we were struck by the frequency and severity of proteinuria. We evaluated 25 patients with generalized lipodystrophy. Eighteen were treated with recombinant leptin, and we have followed 15 on leptin for 4-36 months. We followed renal parameters at baseline and during follow-up visits. Renal biopsies were performed as clinically indicated. At baseline, 22 of 25 patients (88%) had elevated urine albumin excretion (>30 mg/24 h), 15 (60%) had macroalbuminuria (>300 mg/24 h), and five (20%) had nephrotic-range proteinuria (>3500 mg/24 h). Twenty-three (92%) had elevated creatinine clearance (>125 ml/min.1.73 m(2)). Eleven of 15 patients (73%) treated with recombinant leptin exhibited reduction in proteinuria, associated with reduction of hyperfiltration. Four patients who did not improve are discussed individually. Renal biopsy findings were remarkable for focal segmental glomerulosclerosis in four patients, membranoproliferative glomerulonephritis in two patients, and diabetic nephropathy in one patient. In conclusion, generalized lipodystrophy is associated with proteinuria and unique renal pathologies, including focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis. The majority treated with recombinant leptin demonstrated reduction in proteinuria and hyperfiltration.- - - - - - - - - - ranking = 16.157849169124keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis (Clic here for more details about this article) |
7/18. Systemic lupus erythematosus in a patient with partial lipodystrophy.Systemic lupus erythematosus developed in a 35-year-old woman who had partial lipodystrophy since she was 7 years old. To our knowledge, this association has been reported only once. She also had hypocomplementemia, mesangiocapillary glomerulonephritis type II, and a serum assay with positive results for C3 nephritic factor. The association of partial lipodystrophy with other autoimmune disorders suggests an immunologic pathogenesis for this disease.- - - - - - - - - - ranking = 573.3243855639keywords = mesangiocapillary glomerulonephritis, mesangiocapillary, glomerulonephritis (Clic here for more details about this article) |
8/18. Familial incidence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonephritis.C3 nephritic factor is an IgG autoantibody that causes complement activation by stabilizing the alternative pathway C3 convertase. It is associated with partial lipodystrophy and membrano-proliferative glomerulonephritis. The occurrence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonephritis, whether singly or in any combination, is usually sporadic. We describe the coexistence of all three of these conditions in members spanning two generations of a single family. This suggests that the pathogenesis of these conditions may be linked and that genetically determined factors may, in some circumstances, contribute to disease susceptibility.- - - - - - - - - - ranking = 41.394622922811keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis (Clic here for more details about this article) |
9/18. Production of C3 nephritic factor by cultured lymphocytes derived from a patient with partial lipodystrophy.C3 nephritic factor (C3 NeF) has been found mainly in the sera of patients with membranoproliferative glomerulonephritis (MPGN) and partial lipodystrophy (PLD). We examined whether peripheral blood mononuclear cells (PBMC) from a patient with PLD could produce C3 NeF. We investigated the in vitro immunoglobulin synthesis of PBMC with mitogen. We further studied the C3bBb stabilizing activity and undertook agglutination assays of the IgG obtained from the culture supernatants. The patient IgG was able to agglutinate only EAC4b3bBb cells and none of the other intermediate cells. We this demonstrated that C3 NeF could be produced in vitro by PBMC derived from a patient with PLD.- - - - - - - - - - ranking = 8.0789245845622keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis (Clic here for more details about this article) |
10/18. C3 nephritic factor in an individual with recurrent viral infection and lipodystrophy.A patient with nephritic factor in the serum following an attack of disseminated herpes is described. In the majority of cases, the factor is associated with mesangio-capillary glomerulonephritis, with or without partial lipodystrophy. It has, however, been described in cases of partial lipodystrophy alone, in one patient with recurrent pyogenic infections, and in one healthy individual. It has not previously been described in an individual with disseminated viral infection.- - - - - - - - - - ranking = 1keywords = glomerulonephritis (Clic here for more details about this article) |
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