Horm Res

Evaluation of glucose tolerance and insulin secretion in cystic fibrosis patients.

Peraldo M. Fasulo A. Chiappini E. Milio C. Marianelli L.
Department of Pediatrics, Cystic Fibrosis Center, A. Meyer Children's Hospital, University of Florence, Firenze, Italy.
Blood glucose profile and insulin levels during the oral glucose tolerance test were retrospectively analyzed in 52 cystic fibrosis (CF) patients aged 10-33 years (mean 18 years). Thirty-seven subjects (71.1%) had normal glucose tolerance; 6 patients (11.5%) had impaired glucose tolerance and 9 patients (17.3%), with fasting blood glucose levels below 7.2 mmol/l were classified as diabetic, according to WHO diagnostic criteria. No significant differences in the total amount of secreted insulin (expressed as area under the curve) were detected in CF patients with or without glucose intolerance, but a significant delay in the insulin peak time appeared to be related to a compromised nutritional status. Our data indicate a significantly increased risk of glucose metabolism impairment related to poor nutritional condition (RR 5.40; 95% CI: 1.5-19.7) and worse clinical status (RR 4.27; 95% CI: 1.05-17.2). In particular, glucose tolerance abnormalities were found in 50% of CF patients with an unsatisfactory nutritional condition versus 15.6% of CF patients with good nutritional status. Conclusions: Since CF-related diabetes is often underdiagnosed and associated with deterioration in patients' overall clinical status, it is very important to identify subjects at risk of developing diabetes by strict monitoring of glucose metabolism when deterioration in nutritional and clinical conditions is seen which cannot be otherwise explained.

Three cases of malignant pheochromocytoma treated with cyclophosphamide, vincristine, and dacarbazine combination chemotherapy and alpha-methyl-p-tyrosine to control hypercatecholaminemia.

Year 1998
Tada K. Okuda Y. Yamashita K.
Department of Endocrinology and Metabolism, Institute of Clinical Medicine, University of Tsukuba, Japan.
We describe 3 cases of malignant pheochromocytoma with multiple metastases which were treated with cyclophosphamide, vincristine and dacarbazine (CVD) combination chemotherapy and alpha-methyl-p-tyrosine. Case 1 was operated on first but hypercatecholaminemia could not be completely controlled. Cases 2 and 3 received chemotherapy, CVD combination chemotherapy and/ or alpha-methyltyrosine and good control of hypercatecholaminemia was attained. In all cases it was possible to control hypercatecholaminemia during most of the time they were administered a-methyltyrosine despite the presence of tumors. None of the patients suffered hypertension crises. In our patients treatment with CVD plus a-methyl-p-tyrosine proved to be safe and ameliorated the clinical course.