PubMed HealthSearch

Biomedical subjects

M Kiehn

Publications and source records attributed to M Kiehn.

4 recordsLinked to original sources

Pancreatic glucagonoma with and without syndrome. Immunocytochemical study of 5 tumour cases and review of the literature.

In five patients single or multiple glucagonomas were characterized by immunocytochemistry. Two large single glucagonomas were associated with the glucagonoma syndrome, which completely dissappeared after removal of the tumours. The morphologic findings in these patients are compared with 48 others collected from literature. In the other three patients, the glucagonomas were not associated with a clinical syndrome and were detected by chance (one accompanying an insulinoma; the other in pancreases of patients suffering from multiple endocrine neoplasia I; MEN I). These tumours appeared by their histological, immunocytochemical and ultrastructural features better organized than the glucagonomas with syndrome. Glucagonomas not producing a syndrome can be classified into (a) solitary, often malignant endocrine pancreatic tumours, (b) glucagonomas associated with insulinomas and other tumours, (c) multiple glucagonomas in MEN I and (d) single microglucagonomas in elderly patients. It is emphasized that only immunohistology allows clear identification of these tumours as glucagonomas.

Adenoma, Islet Cell

Multiple basal cell carcinomas and keratocysts - the Gorlin and Goltz syndrome.

This paper is based on our experience with the Gorlin-Goltz syndrome and on data from 14 patients of the Nordwestdeutsche Kieferklinik in whom this disorder was detected, treated and followed up. A clinical concept has been produced, with a diagnostic check list including a genetic and a dermatological routine work up as well as a radiological survey of the jaws and skeleton. Whenever multiple basal cell carcinomas plus the typical jaw lesions are found in a patient, the diagnosis is easy. A minimum diagnostic criterion is the combination of either the skin tumours or multiple odontogenic keratocysts plus a positive family history for this disorder, bifid ribs, lamellar calcification of the falx cerebri or any one of the skeletal abnormalities typical of this syndrome. All those in whom this disorder is diagnosed or suspected should be followed up for the rest of their lives. The family should be examined and genetic counselling should be offered.

Adolescent