Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 15-year-old female with short intestine syndrome due to chronic intestinal pseudo-obstruction associated with kidney failure underwent a multivisceral (stomach-duodenum-jejunum-ileum-pancreas-liver) and kidney transplant. She had required parenteral nutrition for the last 5 years, with numerous complications such as sepsis from the central catheter, deep venous thrombosis, severe liver dysfunction, pancytopenia due to bone marrow failure, and severe malnutrition. Surgery lasted 15 hours and was free of complications other than hypothermia, which worsened after revascularization of the grafts. Replacement of 6 units of blood products and crystalloids was required. Biochemical and hemodynamic variables were stable, apart from the development of hypernatremia, hyperglycemia, and lactic acidosis. The anesthetic approach included preoperative assessment of problems related to chronic parenteral nutrition (liver dysfunction, coagulopathy, and restricted venous access), the prevention of hypothermia, correction of electrolyte imbalance and the acid-base status, treatment of reperfusion syndrome, and the replacement of fluids and blood products to maintain circulatory homeostasis and assure sufficient splanchnic perfusion.
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PMID:[Anesthesia for a pediatric multivisceral transplant]. 1507 2

We report the case of a 24-year-old woman with a history of radiotherapy for a cerebellar medulloblastoma 2 years prior to detection of a lymph node metastasis of the former disease and a pancytopenia in the peripheral blood. On bone marrow (BM) examination promyelocyte leukemia vs. a reactive 'promyelocyte arrest' were discussed. The translocation t(15;17) was found in some nuclei and there was a PML-RARalpha gene rearrangement detectable by RT-PCR. Furthermore, there was BM infiltration by the primary cancer. All these results led to the diagnosis of a relapse of the medulloblastoma and of a beginning promyelocyte leukemia. As the patient was pregnant, she had to be parted with the baby to facilitate intensive chemotherapy. She did not respond to a therapeutic regimen specific for promyelocytic leukemia but achieved complete remission of the medulloblastoma as well as the leukemia after the administration of polychemotherapy specific for medulloblastoma. One year later, she suffered from a relapse of her leukemia. Now nearly all cells showed a t(15;17) aberration. Immunophenotype analyses showed a shift to a more undifferentiated blast phenotype that was, however, still HLA-DR negative. The patient again received chemotherapy for leukemia but developed a sepsis 3 months later and died of pancytopenia ensuing her leukemia. There was no clinical evidence for recurrence of the medulloblastoma.
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PMID:Genetic analyses permit the differentiation between reactive malfunctions ('promyelocyte arrest') and arising promyelocyte leukemia in a pregnant patient with a history of a medulloblastoma. 1522 53

Low dose methotrexate [MTX] is now frequently used for various inflammatory diseases. This is a case study of a fatal outcome in a patient with rheumatoid arthritis [RA] treated for a short period with low dose MTX. The patient developed severe pancytopenia followed by bacterial and monilial sepsis upon the co-administration of trimethoprim-sulphamethoxazole [TMP-SMX] for an intercurrent infection. The differential diagnosis of pancytopenia and the mechanisms underlying the increase in plasma free MTX by MTX-SMX in the patient are discussed. It should be noted that this fatal case highlights the risk of severe drug interactions in patients with multiple risk factors treated with low dose MTX for a short period of time.
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PMID:[Fatal pancytopenia and methotrexate-trimethoprim-sulfamethoxazole interaction]. 1552 92

The aim of the study was to the clinical features and long term follow up after treatment with Cladarbine in a tertiary care hospital. Seven patients with hairy cell leukemia were diagnosed between January 1990 till December 2003. Diagnosis in all the patients was established by bone marrow aspirates and trephine biopsy along with TRAP. In two patients the diagnosis was supplemented by flowcytometry and in another two patients by splenectomy. Six patients were male while one was female. Mean age was 47.7 years (range 36-64). Most common presenting features were pallor and weakness (n=5). All patients had splenomegaly. Blood count at presentation revealed that one patient had bicytopenia, two had isolated thrombocytopenia, and three had pancytopenia. Treatment responses were evaluable in seven patients. Complete response was seen in six patients (85.7%). One patient died after two months due to sepsis while 3 (50%) patients relapsed. Those who relapsed received another course of CDA and have maintained remission with a median duration of response of 48 months (20-48). From this small series we can conclude that CDA is an effective treatment for HCL and even it works very well in relapsed cases.
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PMID:Hairy cell leukemia: clinical presentation and long term follow up after treatment with 2-chlorodeoxyadenosine (2-CdA). 1596 Feb 89

A 51-year-old man with poliomyelitis was admitted to emergency because of a severe decubitus ulcer on his right hip that was associated with infection. His general condition deteriorated and he was malnourished and dehydrated. Despite adequate hyperalimentation and antibiotic administration, laboratory data indicated pancytopenia 4 days later. He was diagnosed as having secondary hemophagocytosis (HPS) associated with methicillin-sensitive Staphylococcus aureus sepsis due to decubitus inflammation based on bone marrow aspiration and a blood culture. Although granulocyte colony stimulating factor, packed red blood cell transfusions, platelet transfusions, and antibiotics gradually improved the pancytopenia, the patient died of massive gastrointestinal tract bleeding.
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PMID:Secondary hemophagocytic syndrome in a patient with methicillin-sensitive Staphylococcus Aureus bacteremia due to severe decubitus ulcer. 1659 99

Macrophage activation syndrome is a rare and potentially fatal complication of many childhood pathological settings, most frequently reported in systemic onset-juvenile idiopathic arthritis. The disruption of the macrophage-lymphocyte interaction leads to uncontrolled proliferation of highly activated macrophages and T lymphocytes. The syndrome comprises a heterogeneous group of disorders featuring sepsis-like characteristics typically combined with impaired function of natural killer cells and cytotoxic T-cells, haemophagocytosis and hypercytokinemia, often resulting in fatal multiple organ failure. The clinical picture shows high grade fever, hepatosplenomegaly, pancytopenia, lymphoadenopathy, central nervous system involvement and consumptive coagulopathy. Macrophage activation syndrome is associated with high mortality: even though diagnostic criteria have been proposed, definite diagnosis can be a challenge for clinicians, especially in early phases. There is no standardized therapeutic protocol for macrophage activation syndrome, but it is widely recognized that aggressive treatment strategies might strongly influence prognosis. First line-therapy is usually represented by parenteral administration of high dose-corticosteroids, whilst cyclosporine is added in the steroid-resistant cases. In this paper we provide clinical clues and summarize the most recent studies about pathophysiology and management suggestions for macrophage activation syndrome.
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PMID:The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age. 1670 49

We report a rare case of acute graft-vs-host disease (aGVHD) after a lung transplant. The patient presented with advanced disease manifested by skin rash, fever, diarrhea, liver dysfunction and severe pancytopenia. He went on to die of sepsis and multi-organ failure. aGVHD is a disease often confused with drug reactions or viral infection; therefore, it is important to have a high index of suspicion and to confirm the diagnosis early with tests for donor cell chimerism. Effective treatment is elusive but we are learning a great deal about the underlying mechanisms of this disease and hope to develop better treatment.
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PMID:Graft-vs-host disease as a complication of lung transplantation. 1696 84

We report on a 66-year-old patient originating from Greece and living in Germany with a prosthetic mitral valve because of a combined vitium following juvenile rheumatic fever. The patient fell ill with acute fever, splenomegaly, and pancytopenia. After unsuccessful antibiotic therapy because of presumed endocarditis or sepsis with unknown focus, visceral leishmaniasis was suspected because of recent travel to Greece. Subsequently, this diagnosis was confirmed by serology. Considering thrombocytopenia and concurrent anticoagulation after prosthetic mitral valve replacement, we avoided a bone marrow biopsy usually required for definite proof of leishmania infection. Instead, infection with Leishmania infantum was diagnosed by PCR of a peripheral blood sample. After treatment with liposomal amphotericin B the patient recovered fully.
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PMID:[Acute febrile disease with splenomegaly and pancytopenia. A 66-year-old Greek patient with a prosthetic mitral valve]. 1754 31

Primary thymic epithelial neoplasms (PTENs) are uncommon tumors of anterior mediastinum with a broad range of biological characteristics. We retrospectively reviewed 58 consecutive patients with a diagnosis of PTENs that were confirmed pathologically during 28 yr. There were 58 patients, 31 males (53.4%) and 27 females (46.6%), with a mean age of 43.6 +/-13.8 yr (range, 17-73 yr). Twenty-one (36.2%) patients presented at the Masaoka stage I, 13 (22.4%) patient at stage II, 18 (31.0%) patient at stage III, and 6 (10.4%) patients at stage IV. Forty-five (77.7%) patients had myasthenia gravis, 1 (1.7%) immune deficiency, 1 (1.7%) pancytopenia, and 1 (1.7%) nephrotic syndrome. No paraneoplastic syndrome was associated in 10 (17.2%) patients. Complete resection was accomplished in 41 (70.7%) patients, while incomplete resection was performed in 8 (13.8%) patients. In nine (15.5%) patients only biopsy was carried out. Radiotherapy was administered to 19 (32.8%) patients. Eleven (19.0%) out of 58 who presented at advanced stages (at least III) received chemotherapy. Median follow-up period was 59 mo (range, 1-278 mo). During the follow-up period, 17 deaths occurred. Five patients (29.4%) died of tumor-related causes, and the remaining 12 patients died of other causes (cardiovascular diseases [n = 1, 5.9%], sepsis [n = 4, 23.5%], and MG-related respiratory insufficiency [n = 7, 41.2%]). The overall survival rates at 5 yr and 10 yr were 63.9% and 54.2%, respectively. Tumor-related survival rates at 5 yr and 10 yr were 89.0% and 83.2%, respectively. In our series, disease stage, presence or absence of myasthenia gravis, and tumor size did not affect survival (p> 0.05), either. Complete resection of the tumor seems to be the best predictive factor for long-term survival.
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PMID:Thymic epithelial neoplasia: a study of 58 cases. 1784 44

We report a 53 years old Saudi man a known diabetic for more than 15 years. He presented with lower abdominal pain, diarrhea and vomiting. He had symptoms and signs of sepsis. He had pancytopenia, renal failure, and his blood culture grew E.Coli. He remained febrile despite antibiotics administration for one week and developed crepitation over both thighs. Radiologically, plain-x ray, ultrasound and CT scan of the abdomen confirmed the presence of air in the left kidney involving the renal parenchyma and the collecting system and extensive gas in subcutaneous tissue of the thighs with abscesses. Repeated surgical drainage of the renal and the extra renal abscesses helped the antibiotic that was continued for several weeks to control the infection. Emphysematous pyelonephritis is a rare but life threatening condition that can be difficult to treat especially if the gas forming organism extends outside the kidney.
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PMID:Emphysematous pyelonephritis complicated with extrarenal abscesses. 1820 69


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