Cases reported "Acute Disease"

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1/60. Fatal virus-associated hemophagocytic syndrome associated with coexistent chronic active hepatitis b and acute hepatitis c virus infection.

    A 28-year-old man was admitted to our department with intermittent fever, hepatosplenomegaly and pancytopenia. Liver parameters and serum ferritin were markedly elevated. Bone marrow biopsy showed hypocellularity, histiocytic hyperplasia, and hemophagocytosis consistent with a virus-associated hemophagocytic syndrome (VAHS). There was serological evidence of chronic active hepatitis b and acute hepatitis c virus infection. The patient died despite aggressive immunosuppressive and supportive treatment. autopsy revealed signs of acute viral hepatitis with cholestasis. histiocytes engaged in hemophagocytosis were observed in bone marrow and spleen. The condition was interpreted as VAHS associated with chronic active hepatitis b and acute hepatitis c virus infection. To our knowledge this is the first report of a hemophagocytic syndrome in that setting.
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2/60. Functional, life-threatening disorders and splenectomy following liver transplantation.

    splenectomy (SPL) in cirrhotic patients undergoing liver transplantation (LTx) may resolve specific problems related to the procedure itself, in case of functional and life-threatening clinical situations often occurring as a result of liver cirrhosis and portal hypertension. METHOD: A single-center experience of ten splenectomies in a series of 180 consecutive adult liver transplant patients over a period of 6 yr is reported. The mean patient age was 46.8 /- 9.5 yr (range 25 57 yr). Indications for SPL were post-operative massive ascitic fluid loss (n = 3), severe thrombocytopenia (n = 3), acute intra-abdominal hemorrhage (n = 2), infarction of the spleen (n = 1), and multiple splenic artery aneurysms (n = 1). RESULTS: Extreme ascites production due to functional graft congestion disappeared post-SPL, with an improvement of the hepatic and renal functions. SPL was also effective in cases of thrombocytopenia persistence post-LTx, leading to an increase in the platelet count after about 1 wk. Bleeding episodes related to left-sided portal hypertension or trauma were also resolved. The rejection rate during hospitalization was 0%, and no other episodes were recorded in the course of the long-term follow-up. However, sepsis with a fatal outcome occurred in 4 patients, i.e. between 2 and 3 wk post-SPL in three cases and 1 yr after the procedure as a result of pneumococcal infection in the last case. Fatal traumatic cranial injury occurred 3 yr post-LTx in another case. Five patients (50%) are still alive and asymptomatic after a median follow-up period of 36 months. CONCLUSION: The lowering of the portal flow appears to resolve unexplained post-operative ascitic fluid loss as a result of functional graft congestion following LTx. However, because of the enhanced risk of SPL-related sepsis, a partial splenic embolization (PSE) or a spleno-renal shunt could be used as an alternative procedure because it allows us to preserve the immunological function of the spleen. SPL is indicated in case of post-transplant bleeding due to left-sided portal hypertension and trauma, spleen infarction, and to enable prevention of hemorrhage in liver transplant patients with multiple splenic artery aneurysms. Severe and persistent thrombocytopenia could be treated with PSE. Because the occurrence of fatal sepsis post-SPL is a major complication in LTx, functional disorders, such as ascites and thrombocytopenia, should be treated with a more conservative approach.
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3/60. Laparoscopic splenectomy for splenic sequestration crisis.

    Medical and surgical advances have improved the treatment of splenic sequestration crisis in pediatric patients with sickle cell disease (SCD). Rapid enlargement of the spleen can result from sickled blood cels being trapped in the spleen, which can be life threatening. The laparoscopic splenectomy procedure using the lateral approach has been adapted successfully for the pediatric patient since 1993. Children with SCD who have a history of a splenic sequestration crisis usually are scheduled for an elective splenectomy procedure after the first documented crisis to reduce the risk of death. Some key benefits of this new surgical approach include a shortened hospital stay, decreased postoperative pain, and faster recovery and return to normal activities for the child with SCD.
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4/60. Pneumococcal vaccine in patients with absent or dysfunctional spleen.

    Four patients (3 long-term hodgkin disease survivors and 1 recipient of an allogeneic bone marrow transplant) developed severe infections with streptococcus pneumoniae after staging splenectomy or due to functional hyposplenism after total body irradiation and bone marrow transplantation. Current guidelines for prevention of infection recommend pneumococcal immunization for patients with hodgkin disease treated with splenectomy and others with functional hyposplenism. Booster vaccination after 5 years is also advised. Hospital- and community-based vaccination initiatives may help identify at-risk patients.
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5/60. Splenic abscess as a complication of acute pancreatitis.

    Though there is close anatomical proximity between pancreas and the spleen, the involvement of latter is extremely uncommon in acute inflammation of the pancreas. In this report, we present a case of splenic abscess as a complication of acute pancreatitis. The aspirate from the abscess yielded the organism, klebsiella. We were able to successfully treat this case with percutaneous drainage along with parenteral antibiotics, and we did not have a resort to splenectomy.
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6/60. Spontaneous rupture of malarial spleen: two case reports and review of literature.

    malaria has long been among the most common diseases in the southeast Anatolia region of turkey. In 1992, 18676 cases were diagnosed in turkey, and Diyarbakir city had the highest incidence (4168 cases), followed by SanliUrfa city (3578 cases). malaria was especially common during 1994 and 1995, with 84345 and 82094 cases being diagnosed in these years, respectively. Spontaneous rupture of malarial spleen is rare. We saw two cases during 1998, which are reported herein. Both patients were male, and were receiving chloroquine treatment for an acute attack of malaria. One of the patients had developed abdominal pain and palpitations, followed by fainting. The other patient had abdominal pain and fever. Explorative laparotomy revealed an enlarged spleen in both patients. splenectomy was performed in both patients. We have identified 15 episodes of spontaneous rupture of the spleen in the English language literature published since 1961. Because of increased travel to endemic areas and resistance to antimalarial drugs, malaria is a major medical problem that is becoming increasingly important to surgeons worldwide. malaria is a particularly important problem in the southeast Anatolia region of turkey. Prophylactic precautions should be taken by tourists who travel to this region, especially during the summer.
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7/60. [diagnosis and treatment of spleen rupture during pancreatitis]

    A 31-year old man was admitted for acute pancreatitis. His condition deteriorated progressively and he developed an acute anemia followed five days after admission by an hemorrhagic shock consecutive to splenic rupture. A 45-year old woman was admitted because of an acute episode of chronic pancreatitis. She improved progressively but developed eleven days after admission an hemorrhagic shock consecutive to the rupture of a subcapsular haematoma of the spleen. splenic rupture, an infrequent complication of acute or chronic pancreatitis, is responsible for anemia and hemorrhagic shock. Abdominal ultrasonography and CT scan are necessary to make the diagnosis of splenic rupture and to look for risk factors of splenic rupture, i.e. necrosis in the spleen hilium, left pancreatic pseudocyst, splenic vein thrombosis, segmental portal hypertension, splenomegaly and intrasplenic collection. When possible, embolization of the splenic artery can stop bleeding. splenectomy with distal pancreatectomy seems to be the appropriate treatment of splenic rupture.
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8/60. CD7 and CD56 myeloid/natural killer cell precursor acute leukemia treated with idarubicin and cytosine arabinoside.

    We describe a 69-year-old Japanese male with acute leukemia with a CD7 and CD56 immunophenotype presenting with multiple lymphadenopathy. He was treated with idarubicin and cytosine arabinoside. Although the leukemia showed partial response, the patient did not achieve complete remission. He died of sepsis due to severe neutropenia after the third course of chemotherapy. His autopsy revealed blast infiltration in the lymph nodes, liver, spleen and vertebral bone marrow. Recently, CD7 and CD56 myeloid/natural killer precursor acute leukemia has been associated with a poor prognosis. Our case illustrates that myeloid/natural killer cell precursor acute leukemia shows some response to intensive chemotherapy for acute myeloid leukemia, but such therapy is insufficient to effect a cure. To overcome the resistance of this disease to chemotherapy, further studies should explore other treatment strategies.
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9/60. Durable response to combination therapy including staphylococcal protein a immunoadsorption in life-threatening refractory autoimmune hemolysis.

    BACKGROUND: Few therapeutic options are available for severe, life-threatening, refractory autoimmune hemolytic anemia. CASE REPORT: A 53-year-old 110-kg man was seen with acute onset of symptomatic severe anemia with syncope, unstable angina, and jaundice. His nadir Hct was 8.3 percent with a peak total bilirubin of 44 mg per dL. The DAT was positive but the IAT was negative. Elution studies demonstrated an IgG pan-agglutinin antibody reactive at 37 degrees C. Treatment with high-dose corticosteroids and IVIG was instituted. An accessory spleen measuring 2 cm was identified and surgically removed, but the patient continued to have intense hemolysis. cyclophosphamide at 200 mg per day was started. Apheresis with a staphylococcal protein a immunoadsorption column (Prosorba, Cypress Bioscience, Inc.) was initiated on Day 18 and was performed twice weekly for a total of six treatments. cyclophosphamide was continued for a total of 14 days. His transfusion requirement ceased by the third immunoadsorption treatment. Forty units of RBCs were required over 23 days in an attempt to maintain a Hct greater than or equal to 15 percent. CONCLUSION: Refractory autoimmune hemolysis can be a life-threatening event. The patient did not achieve a response until after several different therapeutic modalities were instituted, including plasmapheresis with a staphylococcal protein a column (Prosorba). A complete response continues to be durable for more than 1 year after therapy.
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10/60. Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases.

    Acute infectious purpura fulminans (AIPF) is a rare syndrome of hemorrhagic infarction of the skin, extremity loss, and intravascular thrombosis. It progresses rapidly and is accompanied by disseminated intravascular coagulation and vascular collapse. The victims often succumb to the disease. Our objective was to investigate the clinical manifestations, outline the clinical course, and delineate factors related to mortality among the patients with AIPF. patients diagnosed with AIPF over a 15-year period were reviewed retrospectively for patient history, comorbid conditions, progression of clinical course, and medical and surgical management. The vast majority of the patients were under the age of 7; however, the disease process can be seen in adults. The overall mortality rate was 43 per cent. The major predisposing factors were history of recent upper respiratory infection, recent surgery or childbirth, young age, and absence of a spleen. The most common clinical manifestations were skin discoloration, disseminated intravascular coagulation, fever, and septic shock. The most common bacteria cultures were neisseria meningitidis, Hemophilus influenzae, and streptococcus pneumoniae. There appears to be a higher mortality in patients who did not undergo a surgical intervention. Compartment syndrome needs to be evaluated early on in the presentation. Rapid diagnosis, intensive care unit management, and prompt surgical consultation and debridement may decrease the mortality.
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