Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the combined occurrence of parathyroid, pancreatic islet and anterior pituitary tumours. To facilitate a screening programme for MEN1, we investigated 709 people (364 males and 345 females, age range 1-84 years) from 62 MEN1 families, and 36 non-familial MEN1 patients. Of those investigated, 220 (95 males and 125 females, age range 8-79 years) suffered from MEN1. Parathyroid, pancreatic and pituitary tumours occurred in 95%, 41% and 30% of the patients, respectively. Parathyroid tumours were the first manifestation of MEN1 in 87% of patients, and amongst the pituitary and pancreatic tumours, somatotrophinomas and gastrinomas were more common in patients above the age of 40 years, whilst insulinomas occurred more frequently in patients below the age of 40 years. Biochemical screening indicated that the penetrance of MEN1 by the ages of 20, 35 and 50 years was 43%, 85% and 94%, respectively, and that the development of MEN1 was confined to first-degree relatives in 91% of patients and to second-degree relatives in 9% of patients. These findings have helped to define a proposed screening programme for MEN1.
To compare interpretations of growth hormone (GH) provocative tests in laboratories using six different GH immunoassays (one enzymeimmunometric assay (EIMA, assay 1), one immunoradiometric assay (IRMA, assay 5), one time-resolved fluorimmunometric assay (TRFIA, assay 3) and three radioimmunoassays (RIAs, assays 2, 4 and 6)), aliquots of peak samples from GH provocative tests were distributed between the four participating laboratories, quantified in the respective immunoassays and interpreted according to the cut-off values for provocative tests defined for each assay method. There was a high degree of relative correlation between the different assays, but absolute GH estimates differed. Assays 2, 4, 5 and 6 yielded almost identical GH levels. Assays 1 and 3 yielded serum GH levels approximately 39% and 70%, respectively, of those of assays 2, 4, 5 and 6. Although the absolute GH level measured in the various assay methods varied, there was good agreement between the interpretation of a given sample among the participating laboratories. This indicates that the differences in estimates of serum GH concentration by different immunoassay systems can be compensated for when cut-off values are defined for each method.
Summary:Papillary carcinoma of the thyroid is the most common type of thyroid cancer and is associated with a good prognosis. Complications of treatment with surgery and radioiodine are uncommon. We report the case of a 13 year old boy who developed testicular damage following treatment with radioactive iodine 350 mCi for a papillary carcinoma of the thyroid. Four years after radioiodine treatment there has been no suggestion of recovery of spernatogenesis. Detailed follow-up studies of similarly treated young patients are required to define the incidence of this complication and to determine its reversibility.
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