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)

Thirty-two patients with the Felty syndrome, defined by the presence of rheumatoid arthritis, splenomegaly, and neutropenia, have been studied in comparison with 32 patients with rheumatoid arthritis matched for age, sex, and disease duration, and 9 patients with rheumatoid arthritis and idiopathic neutropenia. Patients with the Felty syndrome had severe destructive arthritis, which progressed during follow-up despite little evidence of objective synovitis, and a higher frequency of extra-articular manifestations, including vasculitis. Bacterial infection tended to occur in patients with the lowest neutrophil count but continued to occur in some despite normalization of the WBC. Prognosis was poor and 8 deaths occurred, predominantly from sepsis. Serologic features were prominent. High titers of IgG rheumatoid factor and circulating immune complexes characterized patients with persistent neutropenia. A family history of rheumatoid arthritis was more common in patients with the Felty syndrome. The association with HLA DR4 was very strong; in addition there was an increased frequency of the DQw3 variant, 3b, suggesting that HLA Class II genes in linkage with DR4 may contribute to disease expression.
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PMID:The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. 196 4

We report clinical and pathologic findings in 22 patients with Wegener's granulomatosis collected from 1966 to 1989. Ten cases were analyzed retrospectively. Organs affected included the lungs (n = 18), upper airways (16), kidneys (15), musculo-skeletal system (10), eyes (8), skin (7) and ear (5). Clinical manifestations of airway involvement included nasal obstruction, dysphonia and epistaxis. Lung involvement was evident in chest X-rays in 18 patients, 14 with a nodular aspects. Histologic study in 14 of these patients showed necrotizing and granulomatous vasculitis. Clinical evidence of nephropathy was evident in 15 patients and led to rapidly progressive renal failure in 8. Biopsy in this group (n = 14) revealed focal glomerulonephritis in 6 and diffuse disease in 8. Overall, 13 patients died: 5 without diagnosis, 4 from renal failure, 2 from sepsis, 1 from a lymphoma developing 3 years after immune suppressive therapy and 1 from unknown causes. Among survivors, one received a renal transplant and one remains in chronic dialysis. The diagnosis of Wegener's granulomatosis is therefore based on clinical findings including rhino pharyngeal, pulmonary and renal manifestations.
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PMID:[Wegener's granulomatosis: clinical and pathological report of 22 cases]. 213 49

We report a patient with the syndrome of large granular lymphocytes in whom the initial clinical features were polyarthritis, hepatosplenomegaly and neutropenia. Relative lymphocytosis was also demonstrated at the expense of a subpopulation with morphology and surface markers characteristic of large granular lymphocytes (CD2+, CD8+, CD16+ and HNK-1+). After 6 months of asymptomatic course, without changes in clinical or laboratory data, the patient died from an acute abdomen with mesenteric ischemia of different likely causes as suggested by necropsy data (multivisceral diffuse infiltrate by large granular lymphocytes, systemic vasculitis and Clostridium sepsis). The association between this syndrome and systemic vasculitis is discussed.
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PMID:[Vasculitis associated with proliferation of large granular lymphocytes]. 225 May 16

Fifteen patients suffering from severe systemic diseases were treated with monthly pulses administration of cyclophosphamide (0.7 g/m2 of body surface): 8 acute systemic lupus erythematosus, 2 Wegener's granulomatous, 1 polyarteritis nodosa, 1 rheumatoid vasculitis, 1 progressive systemic sclerosis, 1 relapsing uveitis and 1 dermatopolymyositis. The indications for cyclophosphamide were: glomerulonephritis (6 cases), resistance to previous treatments (7 cases) and undesirable side effects of corticosteroid therapy (2 cases). After 3 pulses, the disease was controlled in 12 patients (80%) and corticosteroids could be decreased in all 12 cases without an evolutive relapse of the disease. Five patients developed infections (2 septicemia, 1 zona, 1 herpes gingival stomatitis and 1 viral meningitis) which were treated without sequelae. One patient developed cystitis with hematuria after the 3rd pulse; association of mesna, a urinary tract protective agent, enabled the continuation of treatment without a cystitis relapse. At the end of our retrospective study, the efficacy of pulse cyclophosphamide administration seems to be satisfactory but the risk of undesirable side effects should limit its use to severe systemic diseases or those resistant to conventional therapies.
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PMID:[Treatment of systemic diseases with pulse cyclophosphamide: 15 cases]. 225 84

Pathologic changes of brain in five burned children with septicemia and epileptiform convulsion are reported in this article. The important findings are cerebral vasculitis, focal malacia, focal hemorrhage and cerebral edema. Author considers that these lesions result from the effect of bacterial toxin, particular pyocyaneus toxin and are the direct causes for epileptiform convulsion and death.
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PMID:[Pathologic changes in the brain in burned children with septicemia and epileptiform convulsion--report of 5 autopsies]. 235 32

Pulmonary lipid accumulation associated with intralipid infusion has been observed in autopsies of premature babies and a few adults. We report a 6-year-old boy with radiographically identifiable bilateral pulmonary nodules while he had Staphylococcus epidermidis sepsis. At the time, the child was receiving intralipid as a part of total parenteral nutritional support and was undergoing chemotherapy and radiotherapy for abdominal rhabdomyosarcoma. Pathological studies of the biopsied nodules revealed lipid deposits and foreign body type granulomatous vasculitis. Possible mechanisms for intralipid nodules in the lungs are discussed. Pulmonary intralipid accumulation can occur antemortem and should be included in the differential diagnosis of pulmonary nodules in patients with malignant tumors who are receiving intralipid infusion.
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PMID:Pulmonary lipid nodules after intralipid infusion in a child with rhabdomyosarcoma and Staphylococcus epidermidis sepsis. 237 18

A 31-year-old Hispanic man presented in the pancytopenic phase of acute myelocytic leukemia and was treated with the chemotherapeutic agents mitoxantrone and cytarabine. After 5 days, an erythematous, blanching, papular, crusted eruption developed on his forehead, chest, and legs. Some lesions showed confluence and all were at the same developmental stage. Clinical diagnoses included necrotizing vasculitis and sepsis. A biopsy specimen revealed widespread noninflammatory syringometaplasia of eccrine ducts. Well-developed intercellular bridges and eosinophilic cytoplasm were seen within the metaplastic cells; apoptoses and occasional mitoses were present. This process is distinct and probably occurred secondary to direct toxic injury from the chemotherapeutic drugs. Because similar changes have occurred in patients with neutrophilic eccrine hidradenitis, we believe our patient represents an example of the noninflammatory end of the spectrum of chemotherapeutic eccrine gland reactions.
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PMID:Eccrine squamous syringometaplasia. A cutaneous sweat gland reaction in the histologic spectrum of 'chemotherapy-associated eccrine hidradenitis' and 'neutrophilic eccrine hidradenitis'. 240 65

Clinical and biopsy findings in six patients (aged 29-64 years), suspected of having Wegener's granulomatosis, were not diagnostic. Cardinal signs were skin necroses, isolated deficits of cranial nerves, sudden amaurosis, renal failure with shunt sepsis and lung opacities suspicious of tumour. Only positive tests for anti-cytoplasmatic antibodies (ACPA) in serum made the diagnosis and led to appropriate treatment. This simple yet highly specific immunofluorescence test should be performed in every case of vasculitis of uncertain cause, even if typical clinical signs of Wegener's granulomatosis are at first absent.
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PMID:[ACPA reaction in atypical Wegener's disease. Diagnostic test]. 268 29

Using an enzyme-linked immunosorbent assay, we measured the concentration of fibronectin containing an extra type III domain (ED1) in the circulation of humans. Plasma levels of ED1 + fibronectin averaged 2.8 +/- 1.0 micrograms/ml in healthy individuals and did not differ substantially according to age or sex. In comparison with those from normal subjects, plasma samples obtained from patients with collagen vascular disorders contained increased average levels of ED1 + fibronectin. Among this group, levels of ED1 + fibronectin were significantly greater in samples taken from individuals with clinical evidence of vasculitis. Although levels of total (ED1 + plus ED1 -) fibronectin were also elevated in plasma samples from patients with vasculitis, only the concentration of the ED1 + variant correlated with severity of disease in two patients examined serially. Elevations in plasma content of ED1 + fibronectin, but not total fibronectin, were also noted in patients with acute vascular tissue injury associated with major trauma or sepsis syndrome. Western blot examination revealed the presence of intact dimeric ED1 + fibronectin in the circulation of all patients studied, although fragments bearing the ED1 were also detected. Human plasma normally contains small quantities of soluble ED1 + ("cellular") fibronectin, and these levels are increased in disorders involving vascular injury.
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PMID:Elevated plasma levels of ED1+ ("cellular") fibronectin in patients with vascular injury. 271 81

Critical conditions had been established in 21 (23.1%) of 91 patients with systemic connective tissue diseases for a 12 year period: renal failure (most often), sepsis, pericarditis with cardiac tamponade, hemorrhagic diathesis, terminal arteritis with gangrene, gastrointestinal perforations with peritonitis, etc. The corticosteroids applied in high doses and predominantly parenterally and the immunosuppressors are the main drugs used in the treatment of these conditions. Plasmapheresis when possible is a useful supplement. The prognosis of the acute critical conditions depends mainly on the affected organ (more favorable in pericarditis with tamponade and unfavorable in renal failure and gastrointestinal perforations with peritonitis (and on the basic disease) more optimistic in systemic lupus erythematodes and very pessimistic in nodal periarteritis and other allergic vasculitis).
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PMID:[The problems of treating acute critical states in diffuse connective tissue diseases]. 321 40


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