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Four pediatric patients with mucopolysaccharidoses and an associated carpal tunnel syndrome are presented. Findings in these cases were typical of the adult form of median nerve compression at the wrist, but the patients had minimal symptoms in view of these findings. The importance of careful clinical eximination and early surgical decompression is emphasized.

Two siblings born to consanguineous Bedouin parents and grandparents are reported with the phenotypic features of Ehlers‐Danlos Syndrome (EDS), type VI. In addition, the affected individuals have a polyneuropathy as confirmed by nerve conduction velocity, electromyographic and muscle biopsy studies. We propose that this clinical combination represents a distinct type of EDS.

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The clinical problems associated with the simultaneous and critical presentation of acute hyperparathyroidism and perforated jejunal ulceration in multiple endocrine adenopathy (MEA type I) require a multidisciplinary approach to management. In the patient described here, the use of cimetidine, a histamine-2 receptor blocker, provided sufficient time after surgical closure of the ulcer to control the hypercalcemic crisis medically and surgically, until the persistent hypergastrinemia could be corrected by sequential excisions of the stomach and pancreaticoduodenal apudomas and hyperplasia. The serum gastrin levels, basal and stimulated (by secretin), provide necessary indications for surgical management of otherwise undetected islet cell pathology. The simultaneously associated pituitary hyperproiactinemia in this patient required both surgical and radiologic therapy.Certain endocrinopathies occur in combination sufficiently frequently in predictable associations to be designated as multiple endocrine adenopathy (MEA) syndromes. 123[2], with medullary carcinoma of the thyroid, adrenal medullary pheochromocytoma(s), and hyperparathyroidism; and MEA type lib [3], with medullary carcinoma of the thyroid, adrenal medullary pheochromocytoma, and mucosal neuromas of the alimentary tract. The endocrinopathies within each of these syndromes usually occur metachronously but, when they occur synchronously, the management of such patients requires multidisciplinary attention to diagnosis and treatment. An example of such a critical clinical condition in a young patient is presented with a critique of the sequential management through the multiple and synchronous endocrinopathies. IncidenceIn MEA type I, the relative incidence of the endocrine components is reported to be hyperparathyroidism in about 90% of cases; pancreatic hypergastrinism, hyperinsulinism, and hyperglucagonism in about 50%; and pituitary abnormalities in about 30% [4]. However, in all probability, the incidence of each is higher. When the endocrinopathies occur metachronously, the first to elucidate symptoms is usually parathyroid in origin, while the pituitary disease is the latest to present clinically. When pancreatic islet abnormalities pre-0364-2313/80/0004-0123 $01.40