Forty-nine members of 6 families with multiple endocrine neoplasia type 1 (MEN 1) were investigated with a standardized meal stimulation test to detect the presence of pancreatic endocrine tumors. Fifteen age-matched subjects and 4 patients with primary hyperparathyroidism also were studied. Serum pancreatic polypeptide (PP), gastrin, and insulin as well as plasma glucagon and somatostatin concentrations were determined before and during the test meal. Patients with demonstrable pancreatic endocrine tumors had significantly increased mean basal and peak serum PP (P less than 0.001) and gastrin (P less than 0.001) responses to the meal compared with healthy family members and normal subjects. Seven of 12 MEN 1 patients with parathyroid and pituitary disease but no demonstrable pancreatic endocrine tumors had exaggerated PP and/or gastrin responses to the meal; 4 of them developed pancreatic endocrine tumors, detected by abdominal computerized tomography, 0.5-4 yr later. None of the healthy members of the MEN 1 families or the patients with primary hyperparathyroidism had responses different from those of the normal subjects. Our experience with the meal stimulation test indicates that an elevated basal or exaggerated serum PP and/or gastrin response is an earlier sign of pancreatic involvement in the MEN 1 trait than is abdominal computerized tomography.
Thirty‐nine patients with hyperparathyroidism (HPT) in association with the multiple endocrine neoplasia type I (MEN‐I) syndrome underwent parathyroid surgery from 1961 to 1985. Twenty‐one patients underwent resection of 1–21/2 glands, 6 had 3–31/2 glands removed, and 18 (9 of whom had previously been subjected to parathyroid surgery) underwent total parathyroidectomy with autotransplantation to the forearm. Resection of 1–21/2 glands resulted in persistent or recurrent HPT in 18 (88%) of the 21 cases. Two (33%) of the 6 patients who underwent 3–31/2‐gland resection had recurrent disease. After total parathyroidectomy with autotransplantation to the forearm, no patient had persistent or recurrent HPT. Five (26%) of 18 patients had permanent hypocalcemia requiring supplemental therapy. After a change in the operative technique, however, only 1 of 10 was permanently hypocalcemic. It is concluded that primary HPT in the MEN‐I syndrome always should be treated with radical surgery. Total parathyroidectomy with autotransplantation seems more favorable than 3–31/2‐gland resection owing to the absence of recurrent HPT in this material and the limited number of patients with long‐standing postoperative hypocalcemia, provided that an optimal technique for autotransplantation was performed.
A patient is presented in whom the amyloid component of an intracerebral 'amyloidoma' has been purified and characterized by amino acid sequence analysis. The material originated from an autopsy of a 76-year-old man who 15 years earlier had been operated for an intracerebral 'amyloid tumour'. The tumour had recurred and grown to an almost walnut-sized mass in the right cerebral hemisphere. It was located in the parietal lobe close to the lateral ventricle and had a close connection to the choroid plexus. Histological examination showed large masses of amyloid surrounded by some plasma cells and a few macrophages of the foreign body type. Amino acid sequence analysis of a major fibril subunit protein showed homology with the variable region of a monoclonal lambda immunoglobulin light chain, subgroup III or IV. This shows that the amyloid in the 'tumour' was of AL type and presumably derived from local synthesis by plasma cells.
A constant EDTA infusion of 24 mg/kg/h during 60\p=n-\120 min was given to 26 patients with primary hyperparathyroidism (HPT), 8 patients with hypercalcaemia of other origin and 10 healthy control subjects. PTH and ionized calcium concentrations were measured at 5\p=n-\10 min intervals. In all three groups the infusion caused a linear decrease in plasma ionized calcium. In both the HPT patients and the healthy subjects there was a prompt increase in the serum levels of parathyroid hormone (PTH) until a plateau was reached. The PTH response of the HPT patients appeared already within the hypercalcaemic range and the plateau value was attained at higher levels of PTH and ionized calcium than in the healthy subjects. The enhanced response distinguished half of the HPT patients with basal PTH values within the reference range from the healthy controls. The patients with nonhyperparathyroid hypercalcaemia displayed no increase in PTH values until the ionized calcium concentration was reduced far into or below the reference range. Thus the EDTA infusion permitted a complete differentiation between HPT and other causes of hypercalcaemia. In most cases an infusion over 30 min was sufficient for this purpose.
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