Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The renal biopsy of a 3-year old boy with complete Fanconi syndrome showed the association of a membranous glomerulonephritis with severe tubulointerstial changes. Immunofluorescence microscopy disclosed linear and granular deposits of Ig along tubular basement membranes. The presence of anti-tubular basement membrane antibodies in the patient's serum was demonstrated by indirect immunofluorescence and radioimmunoassay. The child also developed pulmonary involvement associated with episodes of acute anemia. Anti-alveolar basement membrane antibodies were detected by indirect immunofluorescence. The present case is the first reported example of auto-immune disease characterized by the presence of anti-tubular and alveolar basement membrane antibodies associated with an immune complex glomerulonephritis.
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PMID:Membranous glomerulonephritis associated with anti-tubular and anti-alveolar basement membrane antibodies. 36 19

We describe an 8-year-old boy who was diagnosed as having diabetes mellitus at the age of 3 months. During the follow-up the diabetes was uncontrolled, and he presented nephrotic syndrome with renal function impairment, a renal biopsy showing a membranous nephropathy. Subsequently he had episodes of anemia and dyspnea, due to alveolar hemorrhage, and he also developed Fanconi's syndrome. A later renal biopsy showed membranous glomerulonephritis and interstitial nephritis. The presence of antitubular basement membrane antibodies was noted but antialveolar basement membrane antibodies were not detected. We do not believe that this unusual clinical picture was a coincidence, and we speculate about a possible explanation.
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PMID:Membranous nephropathy, antitubular basement membrane antibodies and alveolar hemorrhage in a diabetic child. 228 22

The unique association of idiopathic diffuse membranous nephropathy and micro-angiopathic haemolytic anaemia and thrombocytopenia is described. A 7 year old Vietnamese boy with a 1-month history of anaemia resistant to oral iron supplements presented with acute onset of nephrotic syndrome. Investigations revealed a micro-angiopathic haemolytic anaemia and thrombocytopenia. There was no associated oliguria or uraemia. Diffuse membranous nephropathy was diagnosed by renal biopsy. Apart from a fourfold rise in enterovirus titres, no underlying cause for the haematological or glomerular abnormalities was found. There was an apparent, partial haematological response to fresh frozen plasma infusions, but not to Vitamin E.
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PMID:Micro-angiopathic haemolysis, thrombocytopenia and nephrotic syndrome associated with membranous nephropathy in a Vietnamese boy. 322

In a 10-year retrospective study, we evaluated the clinicopathologic features and renal immunofluorescence patterns of glomerulonephritis in 41 dogs. On the basis of results of histologic examinations, the dogs were segregated into 3 groups, including membranous (n = 12), mesangioproliferative (n = 15), or membranoproliferative glomerulonephritis (n = 14). No significant differences existed among groups in regard to age or duration of illness. Most dogs had been ill for one month or longer. The proportion of dogs with azotemia, anemia, and hyperphosphatemia were not different among the disease groups. Proportion of dogs with hypoalbuminemia and the severity of hypoalbuminemia were not different among groups. Highest urine protein losses and 24-hour urine protein/creatinine ratios developed in dogs with membranous glomerulonephritis. Although hypoalbuminemia and hypercholesterolemia were common (49%), the formation of edema or ascites was not (15%) and, therefore, few dogs had all of the classic features of the nephrotic syndrome. Few dogs suffered thromboembolic complications. Antinuclear antibody titers developed in 11 dogs, the highest titers developing in dogs with polyarthritis and systemic lupus erythematosis. Cellulose acetate electrophoresis detected alpha 2 and beta 1 globulin spikes in most dogs (87%). Results of renal immunofluorescence testing were positive in 36 dogs, using polyvalent antisera for immunoglobulins (Ig)G, IgA, IgM, and/or antisera for complement factor C3. When monovalent antisera for IgG, IgA, and IgM, and fibrinogen were used, immunofluorescence was not observed as often. The major fluorescent pattern was discrete multifocal segmental granular glomerular fluorescence, consistent with immune-complex deposition. Two dogs had linear glomerular staining patterns; however, antibodies directed against normal glomerular basement membrane were not found via elution studies. A high prevalence of glucocorticoid excess (treatment with glucocorticoids and spontaneous hyperadrenocorticism) (34%), chronic inflammatory skin disease (27%), neoplasia (17%), polyarthritis (12%), and systemic lupus erythematosis (7%) were observed as clinical problems concurrent with glomerulonephritis. In 5 dogs, treatment of glomerulonephritis with prednisolone (0.5 to 1.1 mg/kg) did not result in beneficial effects and in fact appeared to be detrimental, leading to azotemia and worsening proteinuria and physical condition in some of the dogs.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Clinicopathologic, renal immunofluorescent, and light microscopic features of glomerulonephritis in the dog: 41 cases (1975-1985). 354 34

Although hemolytic-uremic syndrome (HUS) is a clinico-pathological entity, renal biopsies are usually not indicated for diagnosis, and therefore, studies concerning the histological aspects of the syndrome are few. This study mainly describes the morphological characteristics of 15 tissue-diagnosed sporadic cases of HUS. The ages of the patients ranged between 10 mos. to 15 yrs., with five being under two. The male/female ratio was 2:3. The prodromal phase was present in 10 patients (67%) with gastrointestinal symptoms in four patients (27%) with neurological symptoms, and in three patients (20%) with upper respiratory infections. Five patients had HUS associated with diarrhea (D+) (three infants and two children), while the remaining ten patients (two infants and eight children) had no diarrhea (D-). E. coli was identified in the stool of four of the D+ cases, one of which was also associated with Shigella. The shortest clinical course was 14 days and the longest 55 days in 13 patients. The disease recurred after three months in one patient, and on three occasions in 15 months after onset of HUS in the other. Fourteen patients died and one biopsy-diagnosed case recovered after the acute phase. All patients had anemia (Hb 3.4-10 g/dl) and acute renal failure. Seven cases demonstrated Burr cells, eight cases had thrombocytopenia and six cases oliguria/anuria. Microscopic hematuria was detected in four cases and gross hematuria in two cases. All patients revealed proteinuria and azotemia (40-200 mg/dl). Five/five (100%) cases had decreased creatinine clearance, 12/14 (86%) cases had increased uric acid levels, 9/14 (64%) cases had an electrolyte imbalance. Light microscopy revealed microangiopathic type involvement of the glomeruli in all cases. According to additional findings, the cases were classed into three histological groups: type 1 showing cortical necrosis (3 cases), type 2 predominant glomerular and arteriolar involvement (11 cases) and type 3 predominant arterial involvement (1 case). All cases were considered primary HUS except for one which was associated with membranous glomerulonephritis. (D+) HUS cases were predominantly of the microangiopathic type, similar to the (D-) group; the latter being contrary to the literature. Hypertension was present in 67% of cases and there was no correlation found between the clinical duration of HUS and the histological type. All five patients studied immunohistologically revealed a nonspecific type fibrinogen deposition. Extra-renal microangiopathy was demonstrated in the adrenals, stomach, pancreas, liver and skin in two necropsies studied.
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PMID:Hemolytic-uremic syndrome (HUS): a clinicopathological study of 15 cases. 823 14

An unusual case of a patient with Goodpasture's disease presenting with hemoptysis, severe iron deficiency anemia and microscopic hematuria and proteinuria is described. Both circulating and tissue anti-glomerular basement membrane (GBM) antibodies were present, and renal function remained normal throughout. Immunosuppressive therapy was given for subclinical pulmonary hemorrhage with successful resolution of anemia and disappearance of the circulating anti-GBM antibody. Nine months after presentation he developed nephrotic range proteinuria and a repeat renal biopsy revealed membranous glomerulonephritis with no evidence of his original disease. Both the Goodpasture's associated HLA-DR2 and the membranous associated HLA-DR3 class II antigens were present. The association of antibody mediated and immune complex glomerulonephritis is discussed. The simultaneous presence of HLA-DR2 and HLA-DR3 may predispose to this association.
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PMID:Progression from Goodpasture's disease to membranous glomerulonephritis. 853 89

To assess the potential contribution of elementary decision analysis to evidence-based medicine in nephrology, the treatment of primary membranous nephropathy with nephrotic syndrome is taken as an example involving quantity and quality of life, and the treatment of uremic anemia in hemodialysis patients as an example involving economic constraints of interventions. Technical details on decision tree calculation procedures, outcome quantitative assessment, and probability assignment to branches are outlined. Indications strictly based on significance tests of individual outcomes of randomized controlled trials can either miss clinical advantages or disregard economic constraints that can be easily established by decision analysis and that may have a major impact in practice.
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PMID:Elementary clinical decision analysis in evidence-based nephrology. 1113 58

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. Prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
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PMID:Glomerular involvement in myelodysplastic syndromes. 1179 99

A 59-year-old man diagnosed as having Hashimoto's thyroditis, primary biliary cirrhosis (PBC) and membranous nephropathy (MN) showed consciousness disturbance, convulsions of the upper part of his body, and rapid progression of anemia, which seemed to be derived from subdural and retroperitoneal hemorrhage, respectively. He had been diagnosed as having eosinophilia about 6 weeks before the attack. Coagulation tests revealed a prolonged activated partial thromboplastin time and prothrombin time, which could not be normalized by mixing with normal plasma. Factor V (FV) activity was severely decreased and the purified immunoglobulin G of the patient inhibited normal plasma FV activity in a dose-dependent manner, suggesting the presence of antibody-mediated circulating inhibitors specific for FV. Treatment with steroids and azathioprine as well as plasmapheresis led to improvement of his clinical symptoms, normalization of the coagulation tests, and disappearance of eosinophilia. However, the inhibitor reappeared about 7 months later in association with eosinophilia, which was also improved by steroid therapy. To our knowledge, this is the first report of the co-existence of these three kinds of immune-mediated disorders, and the first report concerning the association between acquired FV inhibitors and PBC with MN. A new unknown immune mechanism, which causes eosinophilia, may be involved in the development of the FV inhibitor in this patient.
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PMID:Acquired factor V inhibitor complicated by Hashimoto's thyroditis, primary biliary cirrhosis and membranous nephropathy. 1254 35

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated with steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented also hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementamia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient haematological improvement occurred. Relapse subsequently occurred that was manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease but haematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, two years after the onset of MDS. Patient 2, who had refractory anaemia with clonal monosomy 19, manifested bowel disease, hepatosplenomegaly, anaemia and non-organic specific autoantibodies. Prednisone led to both clinical and haematological remission. Haematologic disease relapsed 12 months later, when nephrotic-range proteinuria, haematuria and mild azotaemia were also found. Corticosteroid treatment led to long-lasting renal and haematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with either acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis and AL amyloidosis, were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS; (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
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PMID:[Corticoid-sensitive nephrotic syndrome in children with myelodysplastic syndromes]. 1257 74


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