Annular pancreas associated with diffuse chronic pancreatitis.

A patient with longstanding diabetes and renal failure presented with painless vomiting due to duodenal obstruction was found to have an annular pancreas. Initial operative evaluation, later pathologically confirmed, demonstrated involvement of not only the annulus, but also the entire gland by diffuse atrophic chronic pancreatitis. We speculate on the possible influence of the underlying diabetes and renal disease on the pathogenesis of the unusual generalized chronic inflammatory changes and the precipitation of duodenal obstruction in this patient.

Multiple endocrine neoplasia type IIb: a description of several patients and review of the literature.

Our experience exemplifies the varied clinical presentations of patients with MEN IIb. This syndrome may be familial or sporadic, and clinical stigmata may be identifiable in infancy, particularly the characteristic facies and the appearance of ganglioneuromas. First-degree relatives of affected propositi and individuals with other stigmata of the syndrome should be screened carefully and repeatedly for both medullary thyroid carcinoma and pheochromocytoma. The availability of sensitive screening tests may permit detection of C-cell hyperplasia of the thyroid and adrenal medullary hyperplasia before the development of malignancy or hemodynamic consequences of pheochromocytoma. Early detection of these thyroid and adrenal disorders will permit early surgical intervention.

Ethchlorvynol pharmacokinetics during long-term administration in a patient with hyperlipidemia and hypothyroidism.

A 33-year-old obese, hypothyroid, white male with several medical problems was admitted to University Hospital in September 1984 for treatment of drug intoxication. Admitting medications included ethchlorvynol in addition to other central nervous system depressants. Initial serum concentrations were reported at 70 micrograms/ml in this somnolent yet totally conscious adult. Established therapeutic concentrations are 2-8 micrograms/ml, with toxic exceeding 20 micrograms/ml. A tolerance phenomenon seemed evident. Serum ethchlorvynol concentrations were monitored daily during early hospitalization and continued to be substantially greater than reported toxic concentrations. Kinetic values were as follows: total body clearance 9.92 ml/min, volume of distribution 68.0 liters, and half-life 78 hours. These values are unique in that they were calculated from a patient who had not suffered an acute overdose, thereby differing markedly from previously published values. The influence of hypothyroidism and hyperlipidemia on these markedly different values appears to be significant. Ethchlorvynol should probably be added to the list of drugs influenced by thyroid disease.

Adrenal insufficiency following unilateral adrenalectomy: a case report.

We report a case of resection of a unilateral adrenal pseudocyst that precipitated life-threatening adrenal insufficiency. The patient had extremis unresponsive to pressor agents in the immediate postoperative period. Hydrocortisone administration produced temporary resolution and a cosyntropin-stimulation test confirmed adrenal insufficiency. Because of the presence of an adrenal mass, the preoperative evaluation included a formal endocrinological evaluation. Thus, we were able to document the occurrence of acute postoperative primary adrenal insufficiency.

Evaluation of the hypercalcemic patient.

Malignancy is the most common cause of hypercalcemia, which may result from direct involvement of bone or from local or distant production of substances that enhance bone resorption. The recognized incidence of primary hyperparathyroidism has increased greatly since the advent of automated biochemical screening. A single parathyroid adenoma is most frequently the cause. Sarcoidosis commonly results in hypercalciuria but seldom causes sustained hypercalcemia. Increased production of 1,25-dihydroxycholecalciferol leads to hyperabsorption of calcium and enhanced bone resorption.

Immunoheterogeneity of parathyroid hormone in venous effluent serum from hyperfunctioning parathyroid glands.

The immunoreactive parathyroid hormone (iPTH) in the plasma of hyperparathyroid man consists largely of carboxyl (COOH)-terminal fragments of the hormone. Although these fragments have been thought to arise principally or solely from peripheral metabolism of intact human PTH {hPTH(1-84)} secreted from the parathyroid gland, there is disagreement about the source of iPTH fragments in vivo. To reexamine this question, we fractionated peripheral and thyroid or parathyroid venous effluent sera from four patients with primary hyperparathyroidism using a high-resolution gel filtration system (Bio-Gel P-150 columns run by reverse flow). The column effluents were analyzed using two PTH radioimmunoassays, one directed toward the amino(NH(2))-terminal region of the molecule, the other toward the COOH-terminal region. In all four thyroid or parathyroid venous effluent sera studied, iPTH was 9-180 times higher than in peripheral serum from the same patient; after fractionation, hPTH(1-84) accounted for only a portion of the total iPTH (35-55% with the assay directed toward the COOH-terminal region of hPTH, >90% with the NH(2)-terminal directed assay.) The remaining iPTH eluted from Bio-Gel P-150 after hPTH(1-84) as NH(2)-or COOH-terminal hPTH fragments. These results suggest that parathyroid tumors secrete large quantities of hPTH fragments. Based on estimates of their molar concentrations in serum, tumor-secreted COOH-terminal hPTH fragments could account for most of these peptides in peripheral serum if their survival times were, as estimated by several other workers, 5-10 times that of hPTH(1-84). We conclude that, in contrast to published information, secretory products of hyperfunctioning parathyroid tissue are probably a major source of serum PTH immunoheterogeneity.