Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, characterized clinically by rapid progression of renal insufficiency, a male and African-American racial predominance, and pathologically by segmental glomerular collapse, visceral epithelial cell hypertrophy and hyperplasia, and the absence of endothelial tubuloreticular inclusions. Pathologically similar lesions have been reported in adult and pediatric patients with human immunodeficiency virus (HIV) infection and/or intravenous (IV) drug abuse. Most patients with ICG who have been reported in the literature are adults. Six children with ICG were retrospectively identified (two from East Carolina University, four from University of North Carolina-Chapel Hill). Clinical data and renal biopsy findings were reviewed for all patients. All six patients were male; five African-American and one Hispanic. Ages ranged from 2 to 17 years (mean 12 years). Steroid-resistant nephrotic syndrome was the presenting clinical finding. Average 24-h urine protein excretion was 6.3 g (range 3.2-15 g). Five patients were serologically negative for HIV infection (one patient not tested) and none had a history of IV drug abuse or known HIV risk factors. Progression to end-stage renal insufficiency in two patients within 1 year of biopsy required renal transplantation, and within 1 month of biopsy one patient required dialysis. We report a series of pediatric patients with ICG, an aggressive variant of focal segmental glomerulosclerosis. ICG in children is similar clinically and pathologically to this disease in adult patients.
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PMID:Idiopathic collapsing glomerulopathy in children. 1068 63

The pathogenesis of human immunodeficiency virus (HIV)-associated focal segmental glomerulosclerosis (FSGS) has remained obscure. It has been proposed that renal parenchymal cells may be infected with HIV-1. If such infection occurs, the target cells would be expected to express viral proteins and thus could be targets for cytotoxic T lymphocytes. We previously described mice transgenic for a gag-pol-deleted HIV-1 genome that developed FSGS. In the present study, we tested the requirement for functional T cells in the evolution of renal disease in this model. We bred the HIV-transgenic mice (T26) with athymic nude mice to produce athymic T26 mice. We confirmed by flow cytometry of peripheral blood, thymus, lymph node, and spleen that the athymic T26 mice lacked mature T cells. The athymic T26 mice developed renal disease characterized by FSGS, tubular atrophy and dilatation, and interstitial infiltrate that was qualitatively identical to that seen in the parental T26 mice. Quantitative assessment of the athymic T26 mouse kidneys showed that glomerulosclerosis, tubular injury, and interstitial infiltrate were less severe compared with the parental T26 mouse kidneys. Although T26 mouse kidneys had a mixed cellular infiltrate composed of CD4 cells, CD8 cells, and macrophages, interstitial infiltrates within the athymic T26 mouse kidneys included macrophages but lacked both CD4 and CD8 cells. The renal expression of the HIV transgene was 1. 7-fold greater in T26 mice compared with athymic T26 mice. We conclude that mature T cells are not absolutely required for the development of HIV-associated nephropathy in transgenic mice but that, in their absence, renal disease is significantly milder. These data suggest that T-cell-mediated cytotoxicity directed against renal cells expressing virally encoded proteins is not an essential feature of renal pathogenesis in this model.
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PMID:Role of T lymphocytes in renal disease in HIV-transgenic mice. 1069 66

Previous studies have reported that approximately 10% of the patients with human immunodeficiency virus (HIV) infection develop HIV-associated nephropathy (HIVAN). However, over the last decade, morbidity and mortality as a result of HIV-1 infection has remarkably decreased with the availability of potent new antiretroviral drugs. We therefore determined the prevalence of HIVAN from autopsy data of HIV-infected patients in more recent years (1992 to 1997). Autopsy reports of 389 patients were reviewed. In reports suggestive of possible HIVAN, slides of renal tissue were retrieved and reviewed again to ensure appropriate classification. The criteria for the diagnosis of HIVAN were focal segmental glomerulosclerosis with collapse of the glomerular tuft in some glomeruli, extensive tubular ectasia, and significant tubulointerstitial disease. Of 389 autopsy reports, 54% of the patients were black, 35% were white, and 11% were Hispanic. Thirty-three percent of the patients had a history of intravenous drug abuse. The mean CD4 count of the patients was 54 +/- 91/microL (mean +/- SD). In 27 cases, typical features of HIVAN were found based on the criteria used, accounting for an overall HIVAN prevalence of 6.9% (27 of 389 autopsies). Because the overwhelming majority of these patients were black (93%), the prevalence in blacks was 12% (25 of 209 autopsies). We conclude that although mortality and morbidity from HIV infection is decreasing, HIVAN remains an important complication of HIV infection in blacks, even in recent years.
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PMID:Prevalence of HIV-associated nephropathy in autopsies of HIV-infected patients. 1079 23

A number of chimeric simian-human immunodeficiency virus (SHIV) viruses containing tat, rev, vpu, and env from HIV-1 (strain HXBc2) in a genetic background of simian immunodeficiency virus (SIV(mac)239) have been derived from the parental nonpathogenic SHIV-4 virus. In this article we examine the renal pathology associated with the derivation of these pathogenic SHIV strains. The first of the pathogenic SHIVs, SHIV(KU-1), is associated with rapid CD4(+) T cell loss and opportunistic infections associated with AIDS, but only one of four infected pigtail macaques examined has developed significant renal pathology. The renal pathology in this macaque consists of a diffuse increase in matrix in the core of each lobule with collapsed glomerular capillries, which is similar to the renal changes reported in HIVAN. Passage of this virus into rhesus macaques yielded SHIV(KU-2), which results in renal pathology in three of four inoculated rhesus macaques in which <10% of the glomeruli are involved. A molecular clone of SHIV(KU-2) was derived (SHIV(KU-2MC4)) that causes neurologic and renal pathology with more than 60% of the glomeruli involved and results in uremic level BUN concentrations. These results indicate that SHIV(KU-2MC4) causes severe significant glomerular pathology and should permit a detailed analysis of the molecular determinants associated with the development of SHIV-associated glomerulosclerosis in rhesus macaques.
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PMID:Simian-human immunodeficiency virus-associated nephropathy in macaques. 1095 26

For various ethnic and socioeconomic reasons the pattern of renal disease in the inner city displays distinctive features. Hypertension is frequent, often intractable, and generally conditioned by salt sensitivity and a high sodium intake. Chronic hypertensive nephrosclerosis, found predominantly in African Americans, comprises marked cardiomegaly, renal shrinkage, and hypertensive retinopathy. It has been overdiagnosed in the past, but actually accounts for less than 20% of end-stage renal disease (ESRD) in African Americans. Malignant hypertension, less frequent nowadays, may cause renal shutdown, which is reversible in a few cases; the heart and kidneys are often of normal size. Idiopathic focal segmental glomerulosclerosis is the most common cause of the primary nephrotic syndrome in blacks, but its incidence has also been rising in whites and Hispanics; it does not respond well to treatment, and almost one half of the patients develop ESRD within 10 years. Systemic lupus erythematosus is also more common in African Americans, in whom the severe proliferative forms of lupus nephritis pursue a more virulent course: one half of such patients develop ESRD in 5 years. Cocaine, the use of which has assumed epidemic proportions, may cause accelerated hypertension, acute renal failure from rhabdomyolysis, and progression of preexisting renal disease. Heroin nephropathy has all but disappeared and has been replaced by human immunodeficiency virus (HIV) nephropathy. The prognosis of HIV-infected patients maintained by dialysis has greatly improved. Sickle glomerulopathy, consisting of mesangial expansion, basement membrane duplication, and the absence of immune deposits, may cause the nephrotic syndrome in 4% of patients with severe sickle cell anemia, heralding death within 2 years in one half of patients and ESRD in two thirds; survival has not improved with dialysis. Diabetes is now the most common cause of ESRD. Familial aggregation of ESRD is frequently encountered. Interventions useful in the general population, such as vascular bypass procedures, should be undertaken with great caution and restraint in dialysis patients.
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PMID:Renal disease in the inner city. 1145 21

Human immunodeficiency virus-associated nephropathy (HIVAN) is a distinct clinico-pathological syndrome that occurs almost exclusively in black patients with an AIDS defining diagnosis. It is characterized by rapidly progressive renal failure with a severe nephrotic syndrome. The renal biopsy typically shows a collapsing glomerular sclerosis and variable tubulo-interstitial nephritis. The pathogenesis most likely involves infection of renal tubular and epithelial cells with HIV. The use of ACE-inhibitors and steroids may slow down the progression to end-stage renal failure. With the introduction of highly active anti-retroviral therapy, HIVAN may now be treated effectively although clinical data are so far limited to case-reports.
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PMID:Diagnosis and treatment of HIV-associated nephropathy. 1158 26

Human immunodeficiency virus-associated nephropathy (HIVAN) is a clinicopathological entity characterised by proteinuria, rapidly developing azotemia and histologically by collapsig variant of focal and segmental glomerulosclerosis with acute tubular necrosis and mild interstitial inflammation. Untreated, it may result in end stage renal disease (ESRD) in as little as four months. The incidence of HIVAN continues to increase and is the single most common cause of chronic renal disease in HIV-1 seropositive patients. It affects predominantly black individuals. Exact pathogenesis is still not clear but a great deal of progress has been made in the recent past by studies on transgenic mouse model, renal cell cultures and from study of human biopsy material. Current considerations revolve around the role of HIV or protein in renal epithelium and the effects of cytokines, including transforming growth factor-beta and basic fibroblast growth factor on renal structures. Different modalities of treatment with corticosteroids, zidovudine or angiotensin converting enzyme inhibitors have been tried with modest success.
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PMID:Human immunodeficiency virus-associated nephropathy. 1183 70

In order to study the functions of simian immunodeficiency virus (SIV) Nef in vivo in a small-animal model, we constructed transgenic (Tg) mice expressing the SIV(mac)239 nef gene in the natural target cells of the virus under the control of the human CD4 gene promoter (CD4C). These CD4C/SHIV-nef(SIV) Tg mice develop a severe AIDS-like disease, with manifestations including premature death, failure to thrive or weight loss, wasting, thymic atrophy, an especially low number of peripheral CD8+ T cells as well as a low number of peripheral CD4+ T cells, diarrhea, splenomegaly, and kidney (interstitial nephritis, segmental glomerulosclerosis), lung (lymphocytic interstitial pneumonitis), and heart disease. In addition, these Tg mice fail to mount a class-switched antibody response after immunization with ovalbumin, they produce anti-DNA autoantibodies, and some of them develop Pneumocystis carinii lung infections. All these results suggest a generalized Nef-induced immunodeficiency. The low numbers of peripheral CD8+ and CD4+ T cells are likely to reflect a thymic defect and may be similar to the DiGeorge-like "thymic defect" immunophenotype described for a subgroup of human immunodeficiency virus type 1-infected children. Therefore, it appears that SIV Nef alone expressed in mice, in appropriate cell types and at sufficient levels, can elicit many of the phenotypes of simian and human AIDS. These Tg mice should be instrumental in studying the pathogenesis of SIV Nef-induced phenotypes.
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PMID:Expression of simian immunodeficiency virus nef in immune cells of transgenic mice leads to a severe AIDS-like disease. 1190 38

Collapsing glomerulopathy (CG), an aggressive variant of focal segmental glomerular sclerosis, is a renal disease with severe proteinuria and rapidly progressive renal failure. The pathogenesis of CG is unknown. It strongly resembles human immunodeficiency virus (HIV)-associated nephropathy, but the patients are HIV negative. The characteristic glomerular lesion is capillary loop collapse with prominent podocytes filling Bowman's space. Interestingly, these glomerular changes are usually associated with severe tubulointerstitial injury, including tubular epithelial degenerative changes, microcystic dilation of several tubules, and interstitial inflammatory cell infiltrate. Recently, it became evident that the morphologic pattern of CG may appear not only in native kidneys, but also de novo in renal allografts, and that the pattern of CG in renal transplants is not always associated with severe proteinuria. Studies describing CG in renal allografts are all based on biopsies. We report 3 allograft nephrectomy specimens that showed a zonal distribution of the characteristic collapsing glomerular changes with associated tubulointerstitial injury. All 3 kidneys had obliterative vascular changes. One nephrectomy specimen had chronic obliterative transplant arteriopathy, 1 had acute vascular rejection, and 1 had thrombotic microangiopathy. None of the patients had severe proteinuria. Our cases suggest that the morphologic pattern of CG in renal allografts may not represent the same disease process as CG in native kidneys and provide further evidence that collapsing glomerular changes do not define the disease entity of CG, but rather represent a pattern of renal injury. Among other factors, hemodynamic disturbance may play a role in the development of the pattern of CG in renal allografts.
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PMID:Zonal distribution of glomerular collapse in renal allografts: possible role of vascular changes. 1205 80

Renal biopsies were evaluated in 422 patients with nephrotic syndrome at the Kenyatta National Hospital between 1982 and 1993. Three hundred and fifty five (84.1%) of the patients were less than 30 years old (range: 7 months to 66 years; mean=SD: 28.4 - 9.2 years). The commonest histological lesions were mesangial proliferative glomerulonephritis (25.1%), minimal change nephropathy (17.5%) and focal segmental glomerulosclerosis (15.2%). Poststreptococcal aetiology was implicated in diffuse proliferative glomerulonephritis while use of skin lightening cosmetics appeared to play a role in the aetiology of minimal change nephrophathy in females. No aetiological role was apparent for hepatitis B virus, human immunodeficiency virus, malarial or schistosomal infection. All patients with minimal change nephropathy, focal segmental glomerulosclerosis and mesangial proliferative glomerulonephritis were treated with steroids and/or cytotoxics with a variable response.
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PMID:Gromerular diseases in Kenya-another look at diseases characterised by nephrotic proteinura. 1215 47


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