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)

Fourteen patients with Wegener's granulomatosis (WG) and severe renal and extrarenal involvement were studied (serum creatinine on admission 5.8 +/- 3.4 mg/dl). Renal histology showed a necrotizing, crescentic glomerulonephritis in all patients. Despite advanced renal disease on admission cyclophosphamide, steroids (in 13 patients) and plasma exchange (in 9 patients) caused a rapid and sustained improvement of renal function. Four patients required intermittent hemodialysis over a period of one week. After 2 weeks of treatment serum creatinine values below 2 mg/dl (n = 4) indicated a nearly complete recovery of renal function in the long-term follow up (mean serum creatinine achieved after 12 months therapy: 1.1 +/- 0.1 mg/dl (n = 4). Therefore serum creatinine values observed after 2 weeks of therapy, appear to be of prognostic value with regard to renal outcome. No relapse of active WG or progressive renal deterioration was observed during follow-up (22 +/- 13 months) except in one patient with persisting renal impairment. Three patients died (staphylococcus sepsis, intracerebral hemorrhage during hypertensive crisis, pulmonary embolism) during the first two months of therapy. The decline of serum creatinine seemed to be a better indicator of successful therapy than the decrease of anticytoplasmatic antibody (ANCA), erythrocyte sedimentation rate (ESR) and hematuria. On admission ANCA titer neither correlated with serum creatinine, the degree of renal involvement, nor was it of prognostic value. ANCA, serum creatinine and hematuria normalized within 2 to 8 months, whereas ESR and proteinuria remained elevated. Our data indicate a good prognosis of WG even with advanced renal involvement and generalized vasculitis provided aggressive treatment is performed early.
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PMID:Crescentic glomerulonephritis in Wegener's granulomatosis: morphology, therapy, outcome. 187 37

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

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

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

Seventeen patients, who presented with unhealing ulcers or destructive lesions of the upper aero-digestive tract at Ramathibodi hospital from 1977 to 1985 were reported. Lesions caused by infection, Wegener's granulomatosis or non-hematopoietic malignancy were excluded. A spectrum of histopathologic findings were evident in our patients, ranging from acute and chronic inflammatory changes with or without necrosis, polymorphic reticulosis or lymphamatoid granulomatosis, and malignant lymphoma of the non-Hodgkin's type (NHL). Although some initial histopathologic findings were non-specific, evidence of lymphoproliferative disorders finally emerged. These malignant lymphoid cells had a predilection for the GI tract and skin. Lymphoma staging should thus be done. Bleeding from the lesion, treatment-induced leucopenia, and sepsis were common in these patients. Early aggressive treatment including adequate antibiotic coverage for superimposed infection, improved nutritional status, and early radiation to the primary lesion are suggested for those diseases.
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PMID:Lethal midline granuloma and lymphoproliferative disorders. 276 18

The granuloma gangraenescens in the oral, maxillary and facial region is a rare disease. It is a destroying process in the region of palate, nose, paranasal sinuses, cheeks and orbit with characteristic signs of granulomatosis, infection and malignancy. The disease shows often a lethal development with cachexia or sepsis. Besides local inflammations and tumors, the granulomatosis of Wegener, and the so-called necrotizing sialometaplasia are above all to be excluded by differential diagnosis. Five cases are presented in order to describe the diagnostic and therapeutic problems. In literature, the greatest efficacy is attributed to radiotherapy, however, a detailed definition of the most efficient irradiation conditions cannot be given yet because of the small number of cases. Good long-term results or recoveries can be achieved in 75 to 80% of cases by a relatively high radiation dose of 40 to 50 Gy administered within four to five weeks. During the observation time of two to six years, no one of the five patients treated here only by megavoltage therapy showed a recurrence.
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PMID:[The role of radiotherapy in the treatment of granuloma gangraenescens (lethal midline granuloma]. 642 81

Wegener's granulomatosis was diagnosed in a 42-year-old female 16 years ago. From the outset there was upper respiratory, pulmonary, and renal involvement. Over the years she received varying combinations of glucocorticoids and azathioprine. Renal function deteriorated, and she was treated by hemodialysis and living related donor transplantation 9 and 10 years, respectively, after the onset of the disease. 3 months prior to transplantation, the patient had a right pneumonectomy for bronchial stenosis, bronchiectasis and repeated sepsis. 5 years' posttransplantation, she is well and has no signs of activity of original disease.
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PMID:Wegener's granulomatosis: prolonged patient survival after pneumonectomy and renal transplantation. 676 94

An extremely rare observation of gangrenizing granuloma of the nose in a woman of 29 with a fatal outcome is presented. The disease is characterized by rapidly developing changes in the nasal mucosa and sinuses, skin, soft tissues of the face, destruction of cartilage and bone tissues of the nose, upper jaw with perforation of the hard palate and loss of teeth. Wegener's granulomatosis was diagnosed in the hospital. The disease was complicated by multiple errosive bleedings and sepsis. The lack of involvement of the internal organs and granulomatous reaction distinguished the present observation from the classic Wegener's granulomatosis. The presence of marked vascular changes with fibrinoid necrosis of vessel walls and thrombosis, large necrotic areas and accumulation of plasma cells should be regarded as manifestations of hyperergic reaction of the immediate type. The abundance of microbes and polymorphonuclear leukocytes indicates the occurrence of a secondary infection and purulent inflammation.
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PMID:[Granuloma gangraenescens of the nose]. 711 35

Inflammatory conditions of the aorta may present with non-specific clinical features, including unexplained fever. Indium-111 labelled leucocyte imaging may be performed in such patients to look for the presence of occult sepsis or to assess the activity of a known vasculitis. Of approximately 1100 patients to undergo leucocyte scintigraphy for these indications over a 5 year period, three had focal leucocyte uptake in the aorta. The final diagnoses were: (1) periaortitis in Wegener's granulomatosis; (2) aortic dissection in giant cell arteritis; and (3) streptococcal aortitis with impending rupture. In all three cases the uptake was initially not thought to be in the aorta, but in bowel, a paravertebral abscess and in the lumbar spine respectively. Further imaging with CT and MRI led to the correct diagnoses. As the aorta is a rare site of focal leucocyte uptake, errors in image interpretation are likely. The rapid diagnosis of inflammatory conditions of the aorta is essential, however, as they may be life-threatening if unrecognized; therefore awareness of the aorta as a potential site of uptake is important. Urgent referral for further imaging is imperative in these cases as a false or delayed diagnosis may lead to avoidable morbidity and mortality.
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PMID:Indium-111 labelled leucocyte uptake in aortitis. 782 92

There is presented an atypical case Wegener's granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA assays have very good sensitivity and specificity for Wegener's granulomatosis.
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PMID:[An atypical case of Wegener's granulomatosis complicated by sepsis and coxitis]. 816 59


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