摘要:

One hundred and four patients affected by hereditary angioedema belonging to 31 families have been studied. Twenty-two percent had the variant form related to the deficiency of the functional activity of serum C1 esterase inhibitor. The remaining 78% of patients had the predominant form, characterized by low antigenic levels and low functional activity of serum C1 esterase inhibitor. Attacks of swelling affected the subcutaneous tissue in 86% of patients; the upper airways in 76% of patients, and the bowel mucose in 75% of patients. Before treatment was available the mortality rate was 56%. One or more attacks a month were present in 46% of cases. The infusion of C1 inhibitor concentrate promptly reversed 14 severe attacks without any side effect. Twenty-nine patients were given long term prophylactic treatment with androgen derivatives with full success. Tranexamic acid reduced the frequency of swelling of 70% of the patients.

ISSN:0002-9629    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

The finding of focal glomerulonephritis in a patient with Behcet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. None of the patients had symptoms of renal disease. Protein uria was found in five, two of whom had associated leukocyturia. By light microscopy mesangial and extramembranous glomerular deposits were observed in eight patients. Arterioles in ten patients showed subendothelial and medial hyaline deposits. A granular pattern of fluorescent staining identified the presence of the third component of complement in these deposits. Circulating immune deposits were sought and found in six out of seven patients. The finding of circulating immune complexes and deposition of complement in glomerular and arteriolar tissues supports an immune complex mediated nephropathy and is consistent with the hypothesis of an immunological pathogenesis in Behcet's syndrome.

ISSN:0002-9629    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

We previously reported a hamster model for cholesterol gallstone formation and prophylaxis. The aim of this study was to validate a model for dissolution of cholesterol gallstones by testing bile acids used in patients. Sixty hamsters were allocated to six groups of ten; Group I received the standard diet (.8mg cholesterol/gram food) and Groups II-VI received the lithogenic regime (2.4mg cholesterol/gram food and 15 μg ethinyl estradiol) for twelve weeks. During the next eight weeks. Group I remained on the standard diet, Group II on the lithogenic regime, while Group III switched to the standard diet. Groups IV-VI remained on the lithogenic regime, and received 20mg/kg/d of CDC (Group IV), UDC (Group V) or cholic acid (Group VI). Cholesterol gallstones were found in 90% of hamsters on the lithogenic regime, even after return to the standard diet, in 80% of those receiving cholic acid, and in none receiving the standard diet, CDC or UDC. CDC and UDC but not cholic acid inhibited hepatic HMG-CoA reductase activity (p < 0.01) and desaturated bile (p < 0.01). The highest HMG-CoA reductase (p < 0.02) occurred after return from the lithogenic regime to the standard diet. Conclusions: 1) A new model for cholesterol gallstone dissolution has been validated; 2) CDC and UDC, in contrast to cholic acid, decreased HMG-CoA reductase, desaturated bile and dissolved gallstones as in patients; and 3) Return from the lithogenic regime to the standard diet did not desaturate bile or dissolve gallstones, but did increase HMG-CoA reductase as found in gallstone patients.

ISSN:0002-9629    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

A 32-year-old woman presented five months postparturm with weight loss, dehydration, hypotension, exhaustion and hypercalcemia (serum calcium of 15.8mg/dl). Her work-up demonstrated glucocorticoid insufficiency due to isolated ACTH deficiency'. Over the five years since diagnosis no additional pituitary deficits have emerged. Though isolated ACTH deficiency as a cause of adrenal insufficiency is extrememly rare, this case underscores the need to specifically consider glucocorticoid insufficiency in the patient presenting with hypercalcemia.

ISSN:0002-9629    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Carcinoma of the cervix is the fourth most common neoplasm in women. The mortality from this tumor has dropped with the advent of Papanicolaou smears and routine periodic screening, particularly in high risk populations. Diagnosis and staging includes a careful physical examination, the use of colposcopy, directed biopsy, intravenous urogran and cystoscopy. Computed tomography and lymph-angiography may be helpful for detection of iliac or paraaortic lymph nodes.Early, noninvasive stages of this disease (CIN) may be treated with cryosurgery or laser vaporization. Carcinoma in situ (CIS) and microinvasive carcinoma is usually treated with simple hysterectomy for cure. More advanced invasion localized to the cervix may be treated with radical hysterectomy or radiation therapy with 90% of patients surviving 5 years. More advanced tumors are treated with external and intracavitary radiation therapy. For patients with paraaortic lymph node involvement or recurrent tumor, 5-year survival is less than 10%. Chemotherapy may provide some palliation to patients with recurrent tumors but does not increase long term survivorship.

ISSN:0002-9629    

出版商:OVID    

年代:1982

数据来源: OVID