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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixty-five abdominal computed tomography (CT) scans of 54 systemic lupus erythrematosus (SLE) patients were retrospectively evaluated together with their clinical records. This was to assess the spectrum of CT findings and to determine the value of abdominal CT in this group of patients. Over a 3.5-year period, abdominal CT scans had been requested for suspected renal vein or inferior vena cava thrombosis (n = 52, 80%), sepsis, mesenteric ischaemia, Conn's syndrome, evaluation of hepatosplenomegaly, portal hypertension and hydronephrosis. The most frequent indication for CT was suspected renal vein thrombosis (RVT). An SLE patient with previously stable renal function who rapidly develops nephrotic syndrome with deteriorating renal function has an increased risk of thromboembolic phenomenon. Also, renal vein thrombosis is difficult to diagnose clinically and prompt anticoagulation can help preserve remaining renal function. Of these with suspected RVT, two had RVT only and five had thrombosis in both renal veins and inferior vena cava. Two patients had CT features strongly suggestive of mesenteric ischaemia, one had bilateral hydronephrosis thought to be secondary to lupus cystitis and CT confirmed two abdominal abscesses. Other incidental CT findings were: subscapular renal haematoma, overall enlargement or diminution of renal size, serositis, bowel wall thickening, splenic, hepatic and pancreatic enlargement and mild para-aortic lymphadenopathy. Abdominal CT revealed many diverse findings and aided the management of these SLE patients.
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PMID:Abdominal computed tomography in systemic lupus erythematosus. 911 46

A 34-year-old man was found to have leukocytosis and thrombocytosis in 1983. In 1988, his leukocyte count was 10,400/microliter, Hb 16.5g and a platelet was 73 x 10(4)/microliter. A bone marrow examination showed megakaryocyte hyperplasia. Essential thrombocythemia (ET) was diagnosed but no treatment was given. In February 1993, anemia and hepatosplenomegaly developed and cytogenetic study of the peripheral blood demonstrated t(1;7) (q10;p10). Myelofibrosis was diagnosed as by bone marrow biopsy. The patient was treated with blood transfusion, oxymetholone and prednisolone, but without effect. In 1995, acute myeloid leukemia developed, and he died in December 1995 due to septicemia. We report here a case of the ET developed myelofibrosis with t(1;7) (q10;p10) anomaly and acute leukemia.
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PMID:[Essential thrombocythemia in transformation to acute leukemia (FAB-M0) as a natural history from myelofibrosis with t(1;7)]. 919 91

Approximately 0.5-1% of all newborns are born infected with cytomegalovirus (CMV), but of these only one out of ten show symptoms at birth, most often with hepatosplenomegaly, thrombocytopenia, and/or brain affection. Of the remaining nine, one may later develop sequelae with hearing loss and/or mental retardation. CMV infection may also be acquired perinatally or in the newborn period, and may cause pneumonia and/or sepsis, possibly also gastrointestinal symptoms like blood in the stool, and poor weight-gain. We have diagnosed CMV infection in ten neonates and infants, and describe these patients in terms of symptoms, diagnosis and treatment. Ganciclovir is being tested in clinical trials as a treatment for congenital CMV infection, and was given to two of our patients with apparently good results.
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PMID:[Cytomegalovirus infection in neonates. Diagnosis and therapeutic experiences]. 919 23

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.
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PMID:[Budd-Chiari syndrome]. 933 72

Eleven patients (10 boys, one girl) with Evans' syndrome with a median follow up time of 8.0 years were evaluated retrospectively. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. In five patients, an increase in lymph node and/or spleen size was observed during the exacerbations of cytopenias. Seven patients had quantitative serum immunoglobulin abnormalities at the time of presentation. There were associated systemic manifestations in nine patients. Various forms of treatment were used with mixed results. Four patients died from sepsis and haemorrhage; four had complete recovery--two after splenectomy. These findings show that Evans' syndrome is a heterogeneous disorder with significant morbidity and mortality. High incidence of quantitative serum immunoglobulin abnormalities, lymphoid hyperplasia, and associated systemic manifestations suggest that Evans' syndrome may represent a stage of a more broad spectrum, generalised immune dysregulation.
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PMID:The spectrum of Evans' syndrome. 962 17

A 52-year-old male presented with a prolonged fever, anemia, weight loss, hepatosplenomegaly, and elevated serum level of alkaline phosphatase. The patient developed septicemia, metabolic acidosis, and died 3 days after admission. Liver necropsy, tissue showed group C, peripheral T-cell proliferative disease. Epstein-Barr virus genomes were demonstrated in the nuclei of these abnormal T-cell lymphocytes. Granulomatous reaction was observed in the liver and bone marrow. Stains and cultures for mycobacteria and fungi gave negative results. Granulomatous reaction is believed to be induced by cytokines which were released from the T-cell lymphocytes that infiltrated in the liver and bone marrow.
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PMID:Granulomatous reaction in peripheral T-cell proliferative disease: a case report. 947 Mar 34

A 78-year-old man was diagnosed as leukocytosis in February 1994. Physical examination revealed marked hepatosplenomegaly. A peripheral blood examination disclosed 95,090/microliter leukocytes without hiatus leukemicus, 6.5 g/dl Hb, and 15.0 x 10(4)/microliter platelets. The neutrophil alkaline phosphatase score was 27, and serum VB12 was above 1,600pg/ml. IgG was identified as monoclonal immunoglobulin of type lambda. Bone marrow specimens demonstrated marked granulocytic hyperplasia. Neither the Philadelphia chromosome (Ph1) nor BCR gene rearrangement was detected; hence, the diagnosis of Ph1 (-) chronic myeloid leukemia (CML) was made. The patient was treated with hydroxyurea and low-dose VP-16 with no improvement, and died of pneumonia and sepsis in June 1995. This case was considered to be consistent with atypical CML (aCML) according to the FAB classification because monocytosis was not observed. It seems likely and interesting that the coexistent monoclonal gammopathy and aCML might have arisen from common abnormal hematopoietic stem cells.
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PMID:[Atypical chronic myeloid leukemia presenting with trilineage dysplasia and IgG (lambda) type monoclonal gammopathy]. 1019 7

Less than 2% of all lymphoproliferative diseases are indolent or small T-cell disorders, and include T-cell chronic lymphocytic leukemia (CLL)/prolymphocytic leukemia (PLL), large granular lymphocyte (LGL) leukemia, and mycosis fungoides (MF). T-PLL has an aggressive clinical course characterized by high lymphocyte counts, marked hepatosplenomegaly, anemia, thrombocytopenia, and median survival times less than 1 year. The majority of cases are associated with abnormalities of chromosome 14. T-CLL probably represents a small cell variant of T-PLL with a similar aggressive course and similar cytogenetics. T-LGL leukemia is a clonal disorder of CD3+, cytotoxic T lymphocytes. Common clinical features include neutropenia, anemia, splenomegaly, and recurrent bacterial infections. The prognosis is dictated by the severity of the neutropenia, with 10-year actuarial survival rates greater than 80%, and most deaths related to sepsis. A small subset of LGL leukemias have a natural killer (NK) phenotype, are refractory to treatment, and result in multiorgan failure and death in a few months. Mycosis fungoides (MF), the most common of the small T-cell disorders, is a cutaneous T-cell lymphoma with a chronic course, often extending over decades, with most patients eventually succumbing to infection. The small T-lymphocyte disorders represent a rare, diverse group of diseases, which generally have an indolent course, but are not curable.
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PMID:T-small lymphocyte disorders. 1031 85

Two patients with hemophagocytic lymphohistiocytosis who presented with acute liver failure are reported. Both presented with fever, hepatosplenomegaly, markedly elevated liver function tests, abnormal coagulation profiles, and an increase in serum ferritin. Both infants were diagnosed with neonatal hemochromatosis based on a clinical picture of hepatic insufficiency with hyperferritinemia and were referred for liver transplantation. The first patient died of liver failure and septicemia before transplantation. Review of autopsy material revealed a hepatitis-like pattern and extensive infiltration of liver and other organs including bone marrow by histiocytes, some of which were hemophagocytic. The second patient underwent liver transplantation but died 44 days thereafter from progressive hemophagocytic lymphohistiocytosis. Examination of the resected liver demonstrated a hepatitis-like pattern, proliferation of histiocytes, and hemophagocytosis, and the bone marrow revealed hemophagocytic histiocytosis. Hemophagocytosis recurred in the allograft. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. Elevated serum ferritin can make the distinction from neonatal hemochromatosis and other forms of neonatal liver failure difficult. Hemophagocytic lymphohistiocytosis should be considered in the differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.
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PMID:Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis. 1034 80

A patient with AIDS and asymptomatic Chagas's disease and positive xenodiagnosis was taking ketoconazole in order to suppress parasitemia and prevent reactivation of Chagas's disease. Ketoconazole was unplanned suspended after 6 months, and the patient was admitted with fever, headache, vomiting, tachycardia, postural hypotension, hepatosplenomegaly, and positive xenodiagnosis one month later. Treatment with benzonidazole was begun leading to suppression of parasitemia. The patient had probability a neurotoxoplasmosis associated and progressed to coma and death with sepsis. No parasite was found in autopsy.
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PMID:[Reactivation of Trypanosoma cruzi infection in patients with acquired immunodeficiency syndrome]. 1038 May 69


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