Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used.
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PMID:Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. 965 31

The patient was a 23-year-old woman with HTLV-I carrier and was diagnosed as pityriasis rubra pilaris soon after her birth. In November 1990 at her age of 16, she began to have fever and polyarthralgia which were not improved despite the administration of antibiotics. Her laboratory findings showed the positive antinuclear antibody and anti-RNP antibody suggesting one of collagen diseases. A tentative diagnosis as unclassified connective tissue disease (UCTD) was made since her symptoms and laboratory findings were not satisfied with any criteria for rheumatic disorders. The steroid therapy was started in February 1991 and showed a good response. On April 9th, 1996, however, she was admitted to our hospital because of recurrence of high fever and chills. This time, she had sepsis because of the evidence that Enterococcus faecalis was detected in blood culture. Although her condition was improved by antibiotics on June 4th 1996, high fever re-appeared and followed by convulsion and disseminated intravascular coagulation. After the doses of prednisolone per day was increased to 40 mg with antibiotics and anticoagulant, her condition gradually improved. This patient was a rare case of pityriasis rubra pilaris associated with UCTD and sepsis in clinical course.
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PMID:[A case of pityriasis rubra pilaris associated with unclassified connective tissue disease and sepsis in clinical course]. 1115 93