Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 16 years. Patients were followed up for 1 month to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2 1/2, 6 1/2, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2 1/2 years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome.
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PMID:Role of splenectomy in homozygous sickle cell disease in childhood. 614 Apr 33

Patients with end-stage renal disease who develop hypersplenism, patients with mild neutropenia, and those patients whose WBC fails to increase in response to cortisol administration will develop significant neutropenia following transplantation with routine doses of azathioprine. This "intolerance" of azathioprine mandates a reduction in the dose of azathioprine often resulting in allograft rejection. Splenectomy will prevent azathioprine-induced neutropenia, but the hazards of splenectomy in these immunosuppressed patients have led to attempts to salvage at least part of the spleen. Partial splenic ablation by embolization has been utilized in adults prior to transplantation to prevent azathioprine-induced neutropenia while preserving the spleen's protective mechanisms against infection. Eight children in our series of transplant candidates required a reduction of splenic function to prevent azathioprine induced neutropenia. One child had a functioning renal allograft but had recurrent neutropenia limiting the azathioprine dose. Partial splenic embolization was attempted in four children and was initially successful in two. Both patients later developed recurrent neutropenia and needed partial splenectomy. The two patients in whom partial splenic embolization was unsuccessful and five further patients in whom embolization was not attempted also underwent partial splenectomy. Approximately 75% to 80% of the spleen was resected. Six children have since undergone renal transplantation and one child had a transplant with chronic rejection at the time of partial splenectomy. Routine doses of azathioprine have been used in these children with no episodes of neutropenia or sepsis observed. We recommend partial splenectomy in those children requiring renal transplantation who are at risk for development of azathioprine induced neutropenia.
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PMID:Partial splenic ablation in preparation for renal transplantation in children. 636 68

Immunosuppressive therapy is necessary in the treatment and prevention of rejection in renal transplant recipients. Unfortunately, these patients may become intolerant to this therapy when it is complicated by hypersplenism with leukopenia and/or thrombocytopenia. The therapy must then be either decreased or stopped, thus preventing adequate treatment or prevention or rejection. Splenectomy has been used to treat the hypersplenism to break this cycle. It requires operative intervention with general anesthesia and prolonged hospitalization, and has been associated with fulminant bacterial septicemia. For these reasons, partial splenic embolization was offered to our patients as an alternative to splenectomy in the treatment of their immunosuppressive therapy intolerance due to hypersplenism. Six patients with acute rejection episodes and one patient on dialysis awaiting transplantation underwent partial splenic embolization. It corrected the hypersplenism in each case without significant complications allowing control of the rejection episode with adequate immunosuppressive therapy in six patients. Strict aseptic technique, pain control, and antibiotic prophylaxis are advised to prevent the complications of splenic abscess, rupture, or septicemia when this technique is used.
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PMID:Partial splenic embolization for hypersplenism in renal transplantation. 703 69

Because of the risk of overwhelming sepsis following splenectomy, and since it is possible to reduce the splenic function while preserving the spleen, the treatment of various hematologic disorders by splenic artery ligation has been attempted in 11 children, among whom 3 under 5 years of age. 3 patients had thalassemia, 4 had hereditary spherocytosis and 4 others suffered from secondary hypersplenism. One patient died of overlooked splenic necrosis. The other patients are well, with an average follow-up of 19 mo. The aim of this study is to find out whether the desarterization produces the expected results and is well tolerated and whether the desarterialized spleen still preserves its ability to protect the host against infection. The satisfactory treatment of hereditary spherocytosis and secondary hypersplenism would indicate that splenic artery ligation offers an alternative to splenectomy, especially in children under 5 year old.
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PMID:[Treatment of hematologic disorders in children by splenic artery ligation (author's transl)]. 707 16

The records of eleven patients who underwent splenectomy for Gaucher's disease at The Mount Sinai Hospital from 1967 to 1981 have been reviewed. The major indications for operation were hypersplenism, pain, and mechanical problems associated with a massively enlarged spleen. There were no operative mortalities and the hematologic picture returned to normal in all cases. Because of the danger of overwhelming sepsis following splenectomy, partial splenectomy should be considered as a therapeutic modality in these patients.
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PMID:The surgical management of Gaucher's disease. 712 40

Transcatheter embolization of the spleen is gaining popularity as a non-surgical method of treatment for hypersplenism. While early reports documented frequent serious complications, a more recent study noted good results using a fractionated approach with only partial embolization of the periphery of the spleen. This technique was recently used on three patients with hypersplenism associated with severe liver disease. All had grave complications, including sepsis, pneumonia, abscess formation, and progressive liver failure, and all died within six weeks of the angiographic procedure in spite of good haematological responses. Since it is frequently this category of patient in whom the procedure is attempted, definitive surgical splenectomy is suggested following the embolization as soon as the clotting parameters return to normal.
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PMID:Severe complications from partial splenic embolization in patients with liver failure. 723 27

In patients with end-stage liver disease complicated with hypersplenism, neutropenia and thrombocytopenia are risk factors for systemic sepsis and spontaneous bleeding. Granulocyte-macrophage colony-stimulating factor is a naturally occurring cytokine that promotes proliferation and differentiation of granulocyte and monocyte progeny cells. In addition, it is reported to promote the proliferation of megakaryocytes. Its use as an intravenous infusion is Federal Drug Authority (USA) approved for the enhancement of myeloid recovery following autologous bone-marrow transplantation. The present study was initiated to determine whether granulocyte-macrophage colony-stimulating factor could be used to increase the white blood cell and platelet count in patients with cirrhosis and hypersplenism and to determine whether the more convenient subcutaneous route can be used with the same efficacy as the recommended intravenous route. Nine patients with cirrhosis and hypersplenism manifested by a reduced absolute neutrophil count (mean value of 1300 +/- 200/mm3) were studied. In eight patients, Indium white blood cell splenic sequestration scans were obtained before and after the administration of granulocyte-macrophage colony-stimulating factor intravenous infusion or subcutaneously for 7 days. One patient had to discontinue the therapy due to a reaction to granulocyte-macrophage colony-stimulating factor. Following intravenous infusion of granulocyte-macrophage colony-stimulating factor, the mean absolute neutrophil count increased to 2600 +/- 1100/mm3. Following subcutaneous administration, the mean absolute neutrophil count increased to 4100 +/- 200/mm3. No significant change in platelet count occurred with either route of administration. Indium scans obtained before and after the treatment period revealed no significant difference in the splenic uptake.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The use of granulocyte-macrophage colony-stimulating factor to enhance hematologic parameters of patients with cirrhosis and hypersplenism. 781 5

We assessed the long-term efficacy of transjugular intrahepatic portasystemic stent-shunt (TIPSS) in 64 patients. Insertion was successful in 56 patients (87.5%). The reasons for its use were: variceal bleeding (49); ascites (6); portal hypertensive gastropathy (6); hypersplenism (2); and embolization of a spontaneous shunt (1). Fourteen patients were Childs A, 20 Childs B and 28 Childs C cirrhotics. Two patients were non-cirrhotic; one with amyloidosis and one with non-cirrhotic portal fibrosis. Patients were followed clinically and radiologically (Doppler ultrasonography and routine portography at 6 months). During 33 patient-years of follow-up, 22 died, 12 during index admission (two were procedure-related) and nine were transplanted. Twenty-five patients are alive, with a mean survival of 7.1 (SD 7) months. Variceal rebleeding occurred in 10 patients (22.7%), one of whom died, and was always associated with shunt insufficiency (shunt thrombosis 2, hepatic vein stenosis (HVS) 1, intimal hyperplasia (IH) 4, dislocated stent 1, inadequate stent 2). Clinical encephalopathy was induced in seven patients (17.1%) following TIPSS. All responded to medical therapy, but two required reduction in shunt size. Ascites improved after TIPSS in 36 patients (87.8%), but reaccumulated in seven (17.5%), associated with shunt dysfunction in five (SBP 2, IH 3, HVS 2). Fatal sepsis occurred in two patients, and 14 other episodes of infection required antibiotics. TIPSS is a useful treatment for variceal bleeding, resistant ascites and portal hypertensive gastropathy. Shunt dysfunction and sepsis occur frequently, and regular surveillance is necessary.
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PMID:Transjugular intrahepatic portosystemic stent-shunt (TIPSS): long-term follow-up. 795 6

Hypersplenism is defined as the association of anemia, leukopenia, or thrombocytopenia with bone marrow hyperplasia and splenomegaly. Hypersplenism is common in liver cirrhosis and frequent in patients with portal hypertension. The effects of portacaval shunt are variable; hypersplenism hardly ever improves but rarely develops after surgery. Since the spleen is a major component of the mononuclear phagocyte system, splenectomy reduces antibody synthesis. Although splenectomy abolishes hypersplenism, it may lead to sepsis. Recently, partial splenic embolization, using gelform injected directly into the splenic artery, has been performed in patients with cirrhosis. Partial splenic embolization induces an increase in the number of circulating blood cells. In addition, the levels of albumin, hepaplastintest, cholesterol and cholinesterase are increased significantly after treatment. Partial splenic embolization rarely causes problems and may actually be beneficial.
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PMID:[Hypersplenism in liver cirrhosis]. 811 16

Liver transplantation is one of the most extensive operations in visceral surgery. Preexisting cardiopulmonary abnormalities, disturbances of glucose, hormone, and electrolyte metabolism and cirrhosis-associated diseases, including hypersplenism with thrombopenia and severe coagulopathy, require advanced surgical skills. Optimal perioperative intensive care management is necessary because of the patient's immunosuppressed condition. In principle, patient management after liver transplantation is similar to that performed after major visceral surgery. However, special attention should be paid to initial liver perfusion and function. Like for sepsis in visceral surgery, in liver transplantation monitoring of cytokines and other mediators is important. New approaches for bioartificial liver support in patients with acute liver failure have primarily been developed as a bridging system to liver transplantation, but they may also be of value for patients with septic liver failure or liver failure after major liver resections.
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PMID:[Liver transplantation as school for visceral surgery--experiences for perioperative management]. 864 19


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