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

Thrombocytopenia is characteristically associated with septicemia and hemolytic uremic syndrome (HUS), a subset of which has been shown to be associated with endotoxemia and shigellosis. An experimental model that closely resembles these clinical conditions is the generalized Shwartzman reaction modified with a continuous intravenous infusion of endotoxin for 5 hr in rabbits. In addition to exhibiting the triad of HUS (thrombocytopenia, hemolytic anemia, and azotemia), these animals also had circulating platelet aggregates, leukocytosis, lipidemia, hemoglobinemia, hyperfibrinogenemia, and prolonged partial thromboplastin time. Platelets that remained in circulation were chemically exhausted in serotonin content and functionally impaired in aggregation activities. Plasma from animals during thrombocytopenia and platelet functional deficiency had no effect of the aggregation responses of normal platelets. Although the single triggering event of endotoxin infusion was stopped at hour 5, recovery from abnormalities was only partial on day 2 and within normal limits by day 3. In vitro studies supported platelet exhaustion as a mechanism for decreased platelet function after endotoxin infusion. The presence of circulating platelet aggregates and exhausted platelets suggested that the process of platelet activation took place at as long as 24 hr after the cessation of LPS infusion. Endotoxin and other mechanism(s) are likely to be operative in the pathogenesis leading to platelet activation. Further studies to reveal the mechanism of platelet exhaustion in the experimental model may help our understanding of corresponding events in clinical endotoxic injury and HUS associated with endotoxemia.
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PMID:Impaired and exhausted platelets in modified generalized Shwartzman reaction: an analogue of hemolytic uremic syndrome associated with endotoxemia. 664 55

Two sisters, 2 and 5 years of age, suffered from acute haemolytic anaemia occurring after gastroenteritis with Escherichia coli O157. One patient developed clinical signs of severe and acute intravascular haemolysis and sepsis. She received transfusion and antibiotic therapy. The second patient presented with mild to moderate haemolytic symptoms only. None of them developed renal impairment. In serum of both children, elevated titres of short-lived agglutinins were demonstrated in the indirect haemagglutination assay consisting of sheep erythrocytes coated with lipopolysaccharide from E. coli O157. By immunoblot analysis IgM antibodies against the O157 lipopolysaccharide were demonstrated in the acute phase sera but not in follow up sera taken 2 months after disease. On erythrocyte membranes, adsorption of microbial antigens was detected by use of a pool-immunoglobulin fluorescence test. The immunological status of both patients was normal. Complete recovery from haemolytic disease was observed without further therapy. Microbial antigens attached to the cell surface were assumed to be the pathophysiological cause of E. coli O157 associated haemolytic anaemia in two siblings.
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PMID:Haemolytic anaemia in association with Escherichia coli O157 infection in two sisters. 795 24

Acquired autoimmune hemolytic anemia frequently occurs in an individual with a deranged immune system. This impaired immune system can also predispose the patient to infections with a wide range of organisms. Also, it is known that certain infectious organisms can induce immune hemolytic anemia in normal hosts by diverse mechanisms. When autoimmune hemolytic anemia presents concomitantly with infection, it is extremely difficult to establish the etiology of the condition. We present a case of severe autoimmune hemolytic anemia of the warm antibody type associated with pneumococcal sepsis in which both the infectious process and the hemolysis are probably secondary to an altered immune system.
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PMID:Severe autoimmune hemolytic anemia associated with pneumococcal bacteremia. 806 Apr 37

Between 1982 and 1992, 18 cases of pregnancy-related acute renal failure (PR-ARF) were observed (9% of the total number of ARF). Mean age of the women was 32 years (22-40 years). Uterine hemorrhage and preeclampsia/eclampsia were the major causes of ARF, accounting for 61% of the cases. Patchy renal cortical necrosis was suspected in 2 cases whereas signs of disseminated intravascular coagulation (DIC) or microangiopathic hemolytic anemia were present in 6 (33%) and 9 (50%) cases, respectively. Ten women required hemodialysis; and 6 of them, additional plasma exchange sessions. Five patients (28%) died during the acute phase of the illness, mainly due to brain damage, hepatic failure, and sepsis. Among the survivors, a complete (61.5%) or partial recovery (23.1%) was usually seen, but irreversible renal failure was recorded in 2 cases with postpartum hemolytic uremic syndrome (HUS). Short-lasting oligoanuria (< 3 days) represents a good prognostic index. However, the presence of vascular injury (cortical necrosis, HUS) seems to carry a poor prognosis. In conclusion, PR-ARF is still a critical occurrence, associated with serious prognosis for both women and kidneys. So far, the most effective measures remain the careful prevention and the aggressive management of the obstetric complications.
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PMID:Acute renal failure in pregnancy. 829 Jul 7

A 4 month old girl developed a severe hemolytic uremic syndrome (HUS) following pneumococcal sepsis and meningitis. As a result of the hemolytic anemia and thrombocytopenia with associated gastrointestinal bleeding several red blood cell and thrombocyte transfusions became necessary. No plasma was transfused to the patient and all cellular blood components to be transfused were washed thoroughly in order to avoid the administration of eventually dangerous donor anti-T antibodies. Continuous peritoneal dialysis was performed for 23 days until sufficient spontaneous urine production was resumed. From the start of increased hemolysis T-transformation of the patient's red blood cells could be shown; bacterial neuraminidase was proven in the patient's serum, which could be neutralized in vitro by a commercial intravenous IgG preparation. The direct Coombs-Test was negative and no significant amounts of anti-T antibodies were detectable in the patient's serum at any stage of the disease. Our observation suggest that the T-transformation itself has caused the increased hemolysis and not an antigen-antibody (T-anti-T) reaction. In cases of HUS und proven T-transformation, intravenous IgG preparations should be tried therapeutically to inhibit the bacterial neuraminidase.
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PMID:[Hemolytic-uremic syndrome in pneumococcal meningitis and infection. Importance of T-transformation]. 847 69

A 27-year-old male with acute lymphoblastic leukemia (L2) received allogeneic bone marrow transplantation on June, 7 1990. He was conditioned with cyclophosphamide, Ara-C and total body irradiation. GVHD prophylaxis consisted of cyclosporin and short term methotrexate. He was diagnosed as having hemolytic uremic syndrome (HUS) on the basis of microangiopathic hemolytic anemia, thrombocytopenia and renal dysfunction on day 224. Cyclosporin was discontinued and FFP was transfused and plasma exchange was performed. He died of heart failure and sepsis on day 582. Autopsy confirmed the findings of HUS.
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PMID:[An autopsy case report of hemolytic uremic syndrome after allogeneic bone marrow transplantation]. 849 23

Since the beginning of the decade we can remark an increasing of publications about laparoscopic approach in splenic surgery. We report about 17 unselected own cases (8 males, 9 females). Main indications were 12 hematologic diseases: 9 idiopathic thrombopenic purpuras, 1 hemolytic anemia, 1 Hodgkin's disease and 1 recurrent splenic infarction with hypersplenism and extramedulary hemapoiesis, further 1 unclear sepsis with focal lesions and splenomegaly, 1 dysontogenetic splenic cyst, 1 splenic co-affection of Wegener's disease, and 2 traumatic lesions. We performed in 13 cases asplenectomy, under these 1 time combined with a laparoscopic hernioplasty and 1 time with a complete staging, further in 1 case a partial splenic resection, in 1 case a diagnostic excision and in 2 cases a hemostyptic management without splenectomy. We had to convert in no case, the lethality rate was also 0. As a complication we noted one postoperative bleeding, we could managing also laparoscopically. Our mean operating time of 137 min was evident shorter than the published ones by other authors. Because of our good results and the excellent tolerance by patients we can recommend this proceeding. Nevertheless, it should be limited for surgeons with a large experience at laparoscopic centers.
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PMID:[Laparoscopic splenic surgery--report of 17 personal cases]. 922 40

Approximately 2,000 infants with sickle cell disease are born each year in the United States. Sickle cell disease is an inherited disorder of red blood cell hemoglobin. Sickle cells increase adhesion and cause blockage in the small blood vessels, resulting in tissue damage. The cells' production of hemoglobin S results in two major pathophysiologic features of sickle cell disorders: chronic hemolytic anemia and vaso-occlusion. These disorders cause ischemic tissue damage and acute and chronic organ failure. Potential complications for children with sickle cell disease include vaso-occlusive events, splenic sequestration, bacterial septicemia from splenic hypofunction, aplastic crisis, pulmonary compromise including acute chest syndrome, renal tubular dysfunction and renal failure, priapism, aseptic necrosis, gallstones, delayed growth and development, leg ulcers, stroke and premature death. Three major sickle cell complications during the first years of life are dactylitis, splenic hypofunction and splenic sequestration. The risk for pneumococcal meningitis is 36 times greater in children with sickle cell anemia than for black children without the disease, and 314 times greater than for white children.
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PMID:The young child with sickle cell disease. 970 52

A 58 year old man presented with skin manifestations of scleroderma without systemic involvement. Within few weeks of oral corticosteroids and penicillamine therapy, rapidly progressive systemic sclerosis developed. The deterioration manifested by skin lesions, diffuse interstitial restrictive lung disease, acute renal failure with normal blood pressure values, and microangiopathic hemolytic anemia compatible with hemolytic uremic syndrome. Chronic renal failure developed and was treated by dialysis, but the patient died due to sepsis. The course of renal involvement was normotensive; antihypertensive therapy was not prescribed even once. The association of scleroderma renal crisis with normal blood pressure is probably a rare and ominous combination.
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PMID:Normotensive scleroderma renal crisis: case report and review of the literature. 976 42

This was a phase I, multi-center study of 13 pediatric patients (median age, 11 years) to evaluate toxicity, hematopoietic recovery, and graft-versus-host disease (GVHD) after allogeneic transplantation of enriched blood CD34(+) cells obtained from genotypically haploidentical but partially HLA-mismatched related donors (8 parents and 5 siblings). With regard to rejection, donor HLA disparity was 1 (5), 2 (6), or 3 loci (2). With regard to GVHD, recipient HLA disparity was 0 (1), 1 (3), 2 (8), or 3 (1). The patients suffered from acute myelogenous leukemia (6), chronic myelogenous leukemia (4), acute lymphoblastic leukemia (2), or hemolytic anemia plus immunodeficiency disorder (1). To reduce the risk of graft failure through the infusion of a large amount of stem cells, peripheral blood cells (PBC) were mobilized by recombinant granulocyte colony-stimulating factor (G-CSF; lenograstim, 10 microgram/kg/d for 5 days) and collected by 2 to 5 aphereses. To both enhance engraftment and reduce GVHD, CD34(+) cells were enriched using immunomagnetic procedures with the Baxter ISOLEX 300 system (Baxter Healthcare Corp, Irvine, CA) and cryopreserved. After variable cytoreductive regimens, a median of 7.7 (range, 2.2 to 14) x 10(6)/kg of CD34(+) cells and 1.03 (0.05 to 2.09) x 10(5)/kg CD3(+) cells were infused. Using Center-specific posttransplant supportive care and immunosuppressive GVHD prophylaxis, two patients experienced early death; one from veno-occlusive disease at day 17 and one from sepsis at day 18. Nine of 11 patients showed signs of engraftment; however, subsequent rejection was seen in 4 patients, 2 of whom had autologous recovery. Eight patients were evaluated in the early phase of marrow recovery. The median number of days to achieve an absolute granulocyte count of 0.5 x 10(9)/L was 14 (range, 9 to 20) and that to achieve a platelet count of 20 x 10(9)/L was 17.5 (range, 12 to 23). Donor chimerism persisted in five patients until death or current survival. All of the surviving patients with functioning-donor-type hematopoiesis were given total body irradiation. De novo acute GVHD (grades II and IV) was observed in two of the eight evaluated patients. Scheduled donor lymphocyte infusion (DLI), using the CD34(-) fraction, was administered to four patients, free of de novo acute GVHD, beginning between 28 to 43 days after transplant. Three of these patients developed acute GVHD (grades I, II, and IV). Cytomegalovirus infection was a major infectious complication but was successfully managed with gamma-globulin and gancyclovir treatment with or without additional DLI. Five patients are currently surviving, free of disease, with a follow-up ranging from 476 to 937 days. Each survivor has functioning hematopoiesis, three of donor origin and two of autologous origin. In conclusion, our results show that enriched blood CD34(+) cells from a mismatched haploidentical donor are a feasible alternative source of stem cells, but do not appear to ensure engraftment. Because none of the patients who were administered DLI survived, the therapeutic efficacy and safety of periodic DLI, as an integrated part of such transplants, needs to be clarified in further studies.
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PMID:Partially mismatched pediatric transplants with allogeneic CD34(+) blood cells from a related donor. 978 47


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