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 37-yr-old woman with RF-positive RA developed cholestatic hepatitis after 10 days of D-penicillamine therapy. This was discontinued immediately. The cholestasis persisted for the remaining 14 months of her life. Severe hypercholesterolemia developed with xanthelasmata and eventually pancytopenia, which was caused by a massive infiltration of the bone marrow by lipid-containing foam cells. The patient died of sepsis. Review of the literature shows intrahepatic cholestasis to be a rare and idiosyncratic complication of D-penicillamine therapy. To our knowledge, ours is the first documented case of persistent cholestatic icterus.
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PMID:Fatal cholestatic hepatitis caused by D-penicillamine. 805 7

Neutropenic colitis is a complication of the treatment of hematologic malignancies and, less commonly, of other disease entities. The septic, inflammatory process has a predilection for the terminal ileum and right colon. While the pathogenesis is not clear, mucosal injury caused by several different mechanisms and local opportunistic infection play significant roles. An association has been recognized between neutropenic colitis and sepsis caused by C. septicum. Patients present with fever, diarrhea, and acute abdominal pain and tenderness often localized in the right lower quadrant. Sonography and CT are helpful in demonstrating colonic wall thickening and pericolic fluid. Peritoneal lavage has been used to exclude perforation in these critically ill patients. Although there has been debate about whether medical or operative management is best, the optimal initial therapy includes supportive care with gastric decompression, fluid and blood product replacement, and broad-spectrum antibiotics. The indications for surgery include continued intestinal bleeding despite correction of coagulopathy and pancytopenia, free intraperitoneal air, and uncontrolled sepsis. At operation, a right colectomy with ileostomy and mucous fistula or, in selected patients, primary anastomosis is the procedure of choice. Timely return of functioning neutrophils and the eventual prognosis of the primary disease are crucial to the overall success or failure of treatment of neutropenic colitis.
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PMID:Collagenous colitis, eosinophilic colitis, and neutropenic colitis. 837 36

A 57-year-old male presented with palpitations and dyspnea on exertion. Examination of the peripheral blood and bone marrow showed pancytopenia with marked red cell aplasia. Hypogammaglobulinemia was also recognized. Chest X-ray and CT showed a mass in the anterior mediastinum. A biopsy showed thymoma. Two months after admission, the patient died of sepsis secondary to worsening pancytopenia and hypogammaglobulinemia. Autopsy showed non-invasive spindle cell type thymoma and a marked decrease of hematogenous cells. Review of the literature indicates that pancytopenia associated with thymoma is resistant to all forms of treatment and its prognosis is poor.
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PMID:[Autopsy case of non-invasive thymoma associated with pancytopenia and hypogammaglobulinemia]. 851 9

The familial occurrence of duodenal atresia is uncommon. This study evaluated the inheritance patterns, the nature and associations, and the presence of immunologic deficits in duodenal atresia recurring in at least three siblings each in two nonrelated families. In the first family, an association with Fanconi's anemia was observed in three of seven pregnancies (2 boys, 1 girl) suggesting an autosomal recessive mode of transmission. Patients died as a result of overwhelming (fungal) septicemia in association with pancytopenia. In a second family, identical multiple atresias occurred in two female siblings born 18 months apart and a third child with a duodenal stenosis. Overwhelming sepsis and a T-cell dysfunction was seen in the postoperative period, which had partially corrected by follow-up at 5 months. A history of family occurrence of duodenal atresia should alert the physician to the possibility of associated pathology including immune deficiency states.
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PMID:Immune deficiency in familial duodenal atresia. 898 5

Two children with acute lymphoblastic leukemia presenting with cellulitis and sepsis are described. Both presented as having hemophagocytic syndrome with the manifestations of prolonged fever, jaundice, pancytopenia, coagulopathy and histiocytic proliferation with hemophagocytosis in their bone marrows. It is similar to the virus-associated hemophagocytic syndrome (VAHS), except for the absences of lymphadenopathy, skin rash and hepatosplenomegaly. Concomitant virus infections were excluded in these two cases. Both patients' conditions improved after appropriate antibiotics and intravenous immunoglobulin therapy. The prognosis seemed better in bacteria-associated hemophagocytic syndrome (BAHS) than in VAHS even in immunocompromised patients.
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PMID:Bacteria-associated hemophagocytic syndrome in childhood acute lymphoblastic leukemia: report of two cases. 907 88

1. Colchicine is a highly active alkaloid used in the treatment of gouty arthritis and pseudogout. In overdose colchicine inhibits cell division effecting organs with a high rate of cell turn-over, such as the gastrointestinal tract and bone marrow. Early fatality results from cardiovascular collapse and respiratory failure, however pancytopenia and overwhelming septicaemia can occur later. 2. We describe a case of suicidal ingestion of 25-30 mg of colchicine in a previously healthy 43-year-old woman. Initial symptoms were mainly gastrointestinal. By day 5 she had developed severe pancytopenia and early sepsis, which were successfully treated using granulocyte colony stimulating factor (G-CSF) 600 micrograms s.c. 3. In vitro G-CSF is produced by the haematopoietic system. However, G-CSF can now be produced by recombinant DNA cloning technology and thus is available clinically. 4. There is no recognised antidote for colchicine poisoning and treatment is symptomatic. Fab fragments may have a promising future in eliminating colchicine from the body, but are currently not clinically available. In those patients that survive the initial phase of poisoning, G-CSF offers an effective method of treating the pancytopenia and preventing overwhelming septicaemia. Daily monitoring of the patient's haematological status is strongly recommended.
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PMID:Granulocyte-colony stimulating factor in the treatment of colchicine poisoning. 915 49

The case described concerns a 68-year-old male patient, who received intravesical BCG instillations for non-resectable urothelial carcinoma (stage pT1, G2). After the third instillation, which was complicated by hematuria during catheterization, he had a high temperature, dyspnoea, a weight-loss of 15 kg and critical recurrent hypotension for 3 weeks. On admission to the clinic he presented with high serum liver enzymes and pancytopenia. The suspected diagnosis of BCG sepsis was confirmed by the detection of typical granulomas in liver and bone marrow histology. After initiation of tuberculostatic therapy, the patient's condition improved and laboratory results returned to normal. This case shows the potential of a life-threatening systemic side effect after intravesical BCG instillation.
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PMID:A case of BCG sepsis with bone marrow and liver involvement after intravesical BCG instillation. 950 83

A 59-year-old man, who manifested lower back pain, was admitted with sepsis and disseminated intravascular coagulation (DIC). A computed tomographic scan showed a slight thickening of the abdominal aortic wall. A blood examination revealed pancytopenia. Myelodysplastic syndrome was diagnosed after bone marrow aspiration and a chromosome analysis. Sepsis due to a Staphylococcus aureus infection and DIC subsided after medical treatment; however, an aortobifemoral bypass was performed upon the detection of a localized rupture of a mycotic abdominal aortic aneurysm 1 month later. The patient is still alive 2 years after operation despite the presence of a hematological disorder.
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PMID:Mycotic abdominal aortic aneurysm associated with myelodysplastic syndrome (MDS): report of a case. 959 Jul 13

Twenty-three patients who had myelofibrosis with myeloid metaplasia (MMM) were treated at our institution with 50 courses of splenic irradiation (SI) for symptomatic splenomegaly. The median dose of radiation per course was 277.5 cGy, administered in a median of 7.5 fractions. 8/23 patients received multiple courses of SI. Of 49 evaluable courses of SI, 46 (93.9%) resulted in an objective decrease in spleen size. The median duration of response was 6 months (range 1-41). Reduction in spleen size was associated with symptomatic relief in all patients. Overall median survival after SI was 22 months. Significant cytopenia occurred in 10 (43.5%) patients, or 16 (32%) of the 50 courses of SI. Prolonged, life-threatening pancytopenia after a single course of SI occurred in six patients (26%), resulting in fatal sepsis or haemorrhage in three (13%). Nine patients underwent subsequent splenectomy: the perioperative mortality rate was 11%. One third of patients experienced postoperative intra-abdominal haemorrhage necessitating surgical re-exploration. SI can provide symptomatic relief and a reduction in spleen size in most MMM patients. The increased risk of postoperative bleeding in patients requiring subsequent splenectomy dictates against considering SI as an alternative to splenectomy for patients who are otherwise good surgical candidates.
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PMID:Splenic irradiation for symptomatic splenomegaly associated with myelofibrosis with myeloid metaplasia. 982 26

Two siblings manifested a neuro-haematologic syndrome characterised by low birth weight, failure to thrive, chronic persistent tongue ulceration, severe truncal ataxia and pancytopenia without either telangiectasia or chromosomal instability. One sibling died from sepsis and the cerebellum demonstrated reduced cellularity of the molecular and granular layers with relative preservation of Purkinje cells and minimal gliosis. A surviving sibling has shown haematologic progression to a myelodysplastic disorder. There was no evidence of any chromosomal instability following exposure of fibroblasts and lymphocytes to irradiation. Monosomy-7 was not present in the surviving sibling. We suspect that these two patients represent another example of the rare Hoyeraal-Hreidarsson syndrome and we are currently engaged in very close monitoring of the surviving sibling for evidence of any karyotypic abnormality.
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PMID:Familial cerebellar hypoplasia and pancytopenia without chromosomal breakages. 1002 49


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