1/18. T-cell lineage acute lymphoblastic leukemia with chromosome 5 abnormality in a patient with Crohn's disease and lipoid nephrosis.We describe a 17-year-old patient with a documented history of Crohn's disease (CD) and of minimal-change nephrotic syndrome (MCNS) in whom a diagnosis of T-cell acute lymphoblastic leukemia (ALL) was made. The diagnosis of ALL was established by morphological examination of bone-marrow aspirates and confirmed by means of immunophenotypic analysis showing the involvement of T-cell lineage leukemic cells. The lymphoid clone showed a karyotypic abnormality involving the long arm of chromosome 5 in a translocation (5;6). Few cases of CD complicated by ALL have been previously reported. The present one is the first case combining CD and ALL in a patient with a past history of MCNS. This raises the possibility of a relationship among those diseases. The possible mechanisms for such a relationship are discussed here.- - - - - - - - - - ranking = 1keywords = nephrosis (Clic here for more details about this article) |
2/18. nephrotic syndrome in Hodgkin's disease. Evidence for pathogenesis alternative to immune complex deposition.The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration of renal vein thrombosis. Three patients are presented with Hodgkin's disease and the nephrotic syndrome whose renal biopsy specimens studied with light, immunofluorescence and electron microscopy were compatible with "lipoid nephrosis" (minimal change disease). A review of the literature reveals 35 patients with Hodgkin's disease and the nephrotic syndrome. Renal tissue was available for examination in only 27 patients. The majority of patients apparently had glomerular alterations consistent with lipoid nephrosis. The nephrotic syndrome in most of these patients remitted with a variety of methods of therapy (including excision, irradiation, prednisone and cyclophosphamide) and tended to relapse with a recurrence of Hodgkin's disease. In three-fourths of the patients with Hodgkin's disease and the nephrotic syndrome, the Hodgkin's disease was of a mixed cellularity type. The etiology of lipoid nephrosis, although unclear, may be a consequence of altered lymphocyte function. Hodgkin's disease is a malignancy involving T lymphocytes, and the nephrotic syndrome occurring in the course of Hodgkin's disease may be a result of an adverse effect of glomeruli by products of tumor lymphocytes rather than of glomerular deposition of immune complexes.- - - - - - - - - - ranking = 0.75keywords = nephrosis (Clic here for more details about this article) |
3/18. Minimal change nephrotic syndrome revealing solid tumors.In 2 patients with the nephrotic syndrome, unsuspected solid tumors were found. One was a small cell lung carcinoma, accompanied with the syndrome of inappropriate ADH secretion. The other was a cancer of the breast with lymph node and bone metastases. In both, renal biopsy showed minimal change disease without immune complex deposits. There are only 14 other reported cases of paraneoplastic lipoid nephrosis complicating solid tumors. Such cases lead to the discussion on the respective roles of tumor cell gene product(s) inducing proteinuria and of lymphokine secretion by lymphocytes directed against the tumor itself. Cancer should be considered as a possible etiology of the minimal change nephrotic syndrome in the adult.- - - - - - - - - - ranking = 0.25keywords = nephrosis (Clic here for more details about this article) |
4/18. Frequent relapser minimal change nephrosis: an unrecognized X-linked disorder?We report three brothers who developed nephrosis between the age of 3-10 years. The parents were nonconsanguineous and of Arab descent. The mother's sister had a son with a similar condition. patients were steroid responders and frequent relapsers. Renal biopsies in the three brothers showed findings of minimal change nephrosis. This family may suggest the existence of an X-linked recessive nephrosis which provides further evidence for genetic heterogeneity of familial nephrosis.- - - - - - - - - - ranking = 2keywords = nephrosis (Clic here for more details about this article) |
5/18. ibuprofen-associated lipoid nephrosis without interstitial nephritis.A report of ibuprofen-associated lipoid nephrosis without interstitial nephritis is presented. Previous reported cases had incomplete biopsy descriptions or concurrent interstitial disease. The patient responded to withdrawal of ibuprofen.- - - - - - - - - - ranking = 1.25keywords = nephrosis (Clic here for more details about this article) |
6/18. Minimal-change nephropathy associated with pancreatic carcinoma.A 67-year-old man presented with nephrotic syndrome and polymyalgia rheumatica. A renal biopsy revealed minimal-change nephropathy. The proteinuria and rheumatologic findings responded to prednisone therapy. The patient presented three months later with biliary tract obstruction secondary to pancreatic adenocarcinoma metastatic to the liver. The glomerulopathy and polymyalgia rheumatica in this case seemed to be components of the paraneoplastic syndrome. The response of both entities to prednisone therapy supports the hypothesis that they are caused by derangements in cell-mediated immunity. The fact that the tumor progressed despite resolution of the nephrosis and polymyalgia rheumatica suggests that cell-mediated immunity in general is altered by the tumor and not that the carcinoma liberates a factor that directly damages the kidney.- - - - - - - - - - ranking = 0.25keywords = nephrosis (Clic here for more details about this article) |
7/18. Steroid-sensitive nephrotic syndrome with mesangial IgA deposits: a separate entity? observation of two cases.In minimal-change steroid-sensitive nephrotic syndrome with selective proteinuria, mesangial IgA deposition at immunofluorescence is a very rare finding which has been previously considered a pure coincidence. Two patients, aged 6 and 14 years, respectively, with a steroid-sensitive but frequently relapsing nephrotic syndrome and highly selective proteinuria, exhibited minor glomerular alterations at light microscopy and an immunofluorescence deposition of predominant and diffuse mesangial IgA, confirmed by electron microscopy as dense deposits. The observed syndrome, that is surprisingly identical to sporadic literature reports, can be considered a separate entity or subgroup belonging either to IgA nephropathy or to lipoid nephrosis. In the latter case mesangial IgA could be the marker of an easy relapsing course.- - - - - - - - - - ranking = 0.25keywords = nephrosis (Clic here for more details about this article) |
8/18. nephrotic syndrome in two patients with cured Hodgkin's disease.Two cases of lipoid nephrosis (minimal change glomerulonephritis) in patients cured of Hodgkin's disease are reported and the literature is reviewed. Cases reported to date have shown a close temporal relationship between this renal lesion and the presence of Hodgkin's disease. The patients reported are 11 and 9 years without evidence of active malignancy after successful treatment for Hodgkin's disease. Each had abnormal immunologic parameters, depressed T4 (helper) cells and increased T8 (suppressor) cells, which may predispose to the development of the nephrotic syndrome. However, the advent of this complication is not necessarily a harbinger of recurrent lymphoma.- - - - - - - - - - ranking = 0.25keywords = nephrosis (Clic here for more details about this article) |
9/18. Mesangial IgA deposits with steroid responsive nephrotic syndrome: probable minimal lesion nephrosis.Two children presented with nephrotic syndrome. Renal biopsy demonstrated minimal lesion nephrosis with mesangial IgA deposits. Response to prednisone therapy was prompt in both cases. The clinical and histologic features of these and other reported cases suggested a diagnosis of minimal lesion nephrosis and not IgA nephropathy (Berger's disease).- - - - - - - - - - ranking = 1.5keywords = nephrosis (Clic here for more details about this article) |
10/18. Congenital lipoid nephrosis with left renal vein thrombosis and Chiari's syndrome.An infant with primary congenital lipoid nephrosis then developed left renal vein thrombosis and secondary hepatic vein obstruction. This was shown by inferior venacavography. The thrombus detached subsequently, and the child died from massive pulmonary embolism.- - - - - - - - - - ranking = 1.25keywords = nephrosis (Clic here for more details about this article) |
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