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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the clinical and pathological findings of the hemolytic uremic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolytic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both patients. None of these children presented with bloody diarrhea, evidence of circulating antibody response to Escherichia coli O157 lipopolysaccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma infusion and renal replacement therapy. Their clinical course was characterized by non-oliguria and lack of significant hypertension throughout the acute phase of the disease. Despite these favorable clinical parameters, both patients developed end-stage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unknown and the role of HIV infection in its pathogenesis, although possible, is unclear.
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PMID:A typical hemolytic uremic syndrome in human immunodeficiency virus-1-infected children. 909 Jun 54

This report describes a child who presented with classic hemolytic uremic syndrome (HUS) and 4 months later developed a life-threatening but reversible cardiomyopathy with global cardiac dysfunction and a left ventricular ejection fraction of 14%. There was no evidence of electrolyte abnormalities, anemia, hypertension, severe fluid overload, or viral infection. Endomyocardial biopsies were consistent with a dilated cardiomyopathy. This paper highlights the importance of considering the diagnosis of associated cardiomyopathy when presenting with late-onset edema following HUS.
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PMID:Cardiomyopathy: a late complication of hemolytic uremic syndrome. 909 Jun 71

We report a case of renal crisis in a patient with elderly onset systemic scleroderma (SSc). A sixty-one-year-old woman was diagnosed as having SSc with rapidly advancing generalized skin sclerosis. After experiencing an upper respiratory infection, she suddenly developed renal failure, hemolytic anemia and malignant hypertension. Laboratory examination revealed uremia with a significantly high plasma renin level. Ophthalmologic study revealed Keith-Wagner's retinopathy Grade IV. Combination therapy including captopril, systemic corticosteroid and prostaglandin E1 venous infusion for the hemolytic uremic syndrome was effective and saved her from the renal crisis. However, her renal function deteriorated and she needs permanent hemodialysis. Rapidly progressive skin sclerosis in SSc, especially in elderly onset cases, suggests a high risk for renal crisis and indicates the need for careful consideration of hypertension and renal function.
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PMID:Scleroderma renal crisis complicated by hemolytic uremic syndrome in a case of elderly onset systemic sclerosis. 911 17

Hemolytic uremic syndrome spontaneously arises in a few patients with advanced cancer, but it is more commonly related to the use of certain chemotherapeutic agents. Mitomycin-C is, etiologically, the most common causative agent inducing hemolytic uremic syndrome, in a dose dependent manner. We report this syndrome, attributable to mitomycin-C at a cumulative dose of 40 mg/m2, in a gastric cancer patient. A 42-year-old female with stage III gastric cancer underwent radical gastrectomy and was given mitomycin-C at 10 mg/m2 intravenously every four weeks as adjuvant therapy. Hemolytic uremic syndrome was diagnosed three months after the last dose of mitomycin-C administration. The most prominent symptoms included pallor, hypertension and anasarca, with laboratory evidence of microangiopathic hemolytic anemia, azotemia and hyperkalemia. Her disease was progressive, but fortunately stabilized after staphylococcus column A dialysis. Her disease remained in remission for 24 months from the time of diagnosis, and then relapsed in the form of peritoneal carcinomatosis with partial intestinal obstruction.
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PMID:Mitomycin-C induced hemolytic uremic syndrome: a case report and literature review. 915 2

Hemolytic uremic syndrome (HUS) is a rare condition which most frequently follows gastrointestinal or respiratory infection episodes in young children, but it can also occur in other settings such as the postpartum period and during use of drugs such as oral contraconceptives, immunosuppressors, and antineoplastics. In early pregnancy, however, its frequency is thought to be very low. The authors report a case of a 30-year-old woman who developed HUS early in her first pregnancy. She had persistent aqueous diarrhea from the beginning of the pregnancy. At the 21st week she developed hypertension which in 2 weeks was followed by seizures, oliguria, and acute pulmonary edema despite intensive medical efforts to control her blood pressure. Surgical intervention for fetal delivery was performed. The patient was initially kept on continuous hemodialysis (CVVHD) followed by an alternate-day conventional hemodialysis schedule. A peripheral blood analysis showed a microangiopathic hemolytic anemia with thrombocytopenia; blood coagulation tests were completely normal. A brain CT scan and an abdominal MRI showed no major abnormalities. HUS was confirmed by a percutaneal kidney biopsy, performed at the 21st day of anuria. Techniques for identification of verotoxin-producing E. coli were not available. Renal function did not recover and the patient has been undergoing regular maintenance hemodialysis for a year.
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PMID:Early gestational hemolytic uremic syndrome: case report and review of literature. 915 64

The pathophysiology of chronic rejection of renal allografts is poorly understood and specific morphologic markers are being sought for its diagnosis. Ultrastructural splitting and reduplication of the basal lamina of the intertubular capillaries (ITCs) have been shown to be consistently associated with transplant glomerulopathy (TG) in renal allografts and have been used as a marker of chronic allograft rejection. Although the presence of ITC abnormalities is extremely helpful diagnostically and has been considered a surrogate for the diagnosis of TG when glomeruli are not available for examination, their specificity has not been tested. This study examined 135 biopsy specimens from renal allografts and native kidneys and categorized the ITC basal lamina alterations into 5 patterns. The results showed that although marked ITC basal lamina abnormalities are characteristically seen in association with TG, lesser degrees of these changes may also be found in native kidneys and in transplants with other types of glomerulopathies. In native kidneys, splitting and reduplication of the ITC basal lamina were observed in cases of active lupus nephritis, membranoproliferative glomerulonephritis type I, crescentic glomerulonephritis, cryoglobulinemia, and hypertension. In allografts, ITC changes were seen in postinfectious proliferative glomerulonephritis, acute cyclosporin toxicity, and hemolytic uremic syndrome, in addition to cases with TG. The histopathologic diagnosis in renal diseases relies heavily on clinical, immunofluorescence, and ultrastructural findings. Therefore, in the transplantation setting, with other less common pathological processes ruled out, the presence of abnormalities of the ITC basal lamina is highly indicative of TG. This association is particularly true for cases with severe ITC abnormalities.
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PMID:Specificity of intertubular capillary changes: comparative ultrastructural studies in renal allografts and native kidneys. 918 23

Atypical, non-diarrhoea associated haemolytic uraemic syndrome (D-HUS) is a heterogeneous disorder with a generally poor outcome, although this view has now been questioned. The clinical and laboratory features of 23 children with D-HUS, representing a third of all patients with HUS seen during the last 26 years, were examined. The median age was 4.9 years (range 3 days-13.8 years). Twenty one children (91%) survived the initial phase. All patients except six infants aged < 18 months required dialysis (74%). Hypertension (43%), cardiomyopathy (43%), and cerebral convulsions (48%) were common. Nineteen (83%) children were followed up for a median period of 5.5 years (range 0.5-23.4). Only five (26%) patients, among them four infants, recovered completely. Six (32%) patients had one to 10 recurrences, including two siblings with neonatal onset, and eight (42%) developed end stage renal failure. Five children underwent cadaveric renal transplantation, with recurrence and subsequent graft failure in two. Four children died, resulting in an overall mortality of 26%. Atypical HUS is heterogeneous with regard to epidemiology, pathophysiology, and outcome. Children with a recurrent, familial, or neonatal course have worse outcomes; in contrast, infants not requiring dialysis in the acute phase have a better prognosis.
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PMID:Heterogeneity of atypical haemolytic uraemic syndromes. 924 50

A 23-year old male patient had eight distinct episodes of hemolytic uremic syndrome (HUS) between 8.5 and 15 years of age, five of them accompanied by hypocomplementemia. In the further course, severe hypertension, renal insufficiency as well as protein-losing enteropathy due to intestinal lymphangiectasia developed, whilst hypocomplementemia persisted. The association of recurrent HUS with hypocomplementemia and intestinal lymphangiectasia may represent a new association within a subgroup of the atypical HUS.
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PMID:Recurrent hemolytic uremic syndrome with hypocomplementemia and intestinal lymphangiectasia. 927 48

Infection with verotoxigenic Escherichia coli, mainly strain O157:H7, has been incriminated in the cause of the hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Both syndromes, which cause serious problems in children and elderly patients, are not readily treated. Conventional treatment with antibiotics has been reported to not only stimulate bacteria to increase toxin production, but also to enhance its release from the cell. This in turn increases the severity of the disease. An alternative approach to the control of the disease would involve treatment with the amino acid L-arginine, in high amounts through total parenteral nutrition. L-arginine will generate high amounts of nitric oxide which will decrease platelet aggregation and increase vasodilation. Both effects will decrease the development of the tubular occlusion and the accompanied hypertension. In summary, we hypothesize that L-arginine can be used to control two diseases caused by E. coli O157:H7.
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PMID:L-arginine as a therapeutic approach for the verotoxigenic Escherichia coli-induced hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. 929 73

Of 193 children who underwent hematopoietic stem cell transplantation (HSCT) for various malignancies, 10 developed hemolytic uremic syndrome (HUS) 1 1/2-5 months later. All 10 had microangiopathic hemolytic anemia, thrombocytopenia and impaired renal function. Six of 10 presented with pericardial effusion, while three presented with hypertension. No child required plasma exchange, and all patients have survived without life-threatening long-term sequelae. By univariate analysis, the underlying diagnosis of neuroblastoma and a history of cisplatin (CDDP) administration were significantly associated with the development of HUS (P < 0.0001). By multivariate analysis using logistic regression, neuroblastoma and use of total body irradiation (TBI) as a conditioning regimen were significant contributing factors for HUS (P = 0.0001 and 0.036, respectively). Although CDDP administration could not be evaluated because of its strong correlation with the underlying diagnosis, we speculate that CDDP may enhance the nephrotoxicity of TBI, leading to a high incidence of HUS in patients with neuroblastoma.
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PMID:Hemolytic uremic syndrome after allogeneic or autologous hematopoietic stem cell transplantation for childhood malignancies. 948 51


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