Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Surgical exploration identified 40 patients with several enlarged parathyroid glands (two to five) out of a retrospective series of 500 cases of primary hyperthyroidism. Complete clinicopathological correlations were available in 27 cases (19 women and 8 men). Seven patients had a history of Wermer's syndrome (type I, MEN). Final diagnosis was as follows: double adenomas, 11 cases; primary hyperplasia, 16 cases. In two cases, there was a clear-cell hyperplasia, 16 cases. In two cases, there was a clear-cell hyperplasia with massive involvement of four parathyroid glands. The fourteen other cases presented with predominantly chief-cell hyper plasia; the involvement of the parathyroid glands was frequently asymmetrical, mainly or exclusively confined to two enlarged glands in 9 cases. The authors conclude that when a patient presents with two enlarged parathyroid glands, two diagnoses are possible--double adenoma or asymmetrical hyperplasia. Since anatomical study of the two enlarged glands is not always sufficient for accurate classification of the lesions, it is advisable to make a histological examination of a third.
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PMID:[Multiple lesions in primary hyperparathyroidism. Nosologic approach]. 613 40

Six patients with multiple endocrine neoplasia (MEN) types 2a and 2b were investigated to determine the spectrum of pheochromocytoma by scintigraphy. Iodine-131-metaiodobenzylguanidine (131I-MIBG), a new imaging agent which concentrates in adrenergic neurotransmitter vesicles, was administered at 0.5 mCi/1.7m2 and scintiscans were taken at 24 and 48 hours. Two normotensive patients with normal plasma and urinary catecholamines had no adrenal tracer uptake. One patient with a modest and intermittent increase only in urinary catecholamine metabolites showed faint adrenal images. Two other patients with increased plasma and urinary catecholamines showed bilateral adrenal imaging patterns. The sixth patient who had increased norepinephrine and epinephrine secretion showed bilateral asymmetrical adrenal images, findings that were corroborated at operation. Functional as well as anatomic evidence of adrenal medullary abnormalities in patients with MEN-2 syndromes are demonstrated by 131I-MIBG scintigraphy. Therefore, the procedure can be used to define the extent of abnormalities of the adrenal medulla in these patients.
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PMID:Spectrum of pheochromocytoma in multiple endocrine neoplasia. A scintigraphic portrayal using 131I-metaiodobenzylguanidine. 723 18

The primitive hyperparathyroidism (PHPT) constitutes still cause of discussion both from the diagnostic point of view and from the therapeutic one although surgical successes are generally reported. Between the most important problems there is the increase of the HPT asymptomatic or oligosymptomatic patients with the decisional difficulties in the timing of the surgical treatment and the difficult framing of the HLP disease associated with MEA and the relating surgical failures. Besides some authors support an unilateral dissection of the neck in patients with adenoma diseases diagnosed before the intervention against the traditional address of a bilateral exploration. Our experience is based on 31 patients subjected to intervention of parathyroidectomy for primitive HPT: 26 carriers of adenomas, of which 1 double, and 5 of diffused hyperplasia. We have effected 25 simple parathyroidectomy for adenoma, 1 resection of three parathyroid glands for double adenoma, 2 subtotal parathyroidectomy (7/8) for diffused hyperplasia. 2 patients had new surgical treatment for persistent hypercalcemia, and they were respectively carriers: 1 of a second ectopic adenoma and 1 of asymmetrical hyperplasia; 2 patients finally, operated in other hospitals had a second exploration and they were affected from MLP. In 26 patients we had very good results, in 2 persistence of hypercalcemia (patients who had a second look) and 3 hypocalcemia.
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PMID:[The surgical treatment of primary hyperparathyroidism: clinical experience]. 1080 73

The risk for bilateral tumors and long-term outcome after conservative cortical-sparing adrenal surgery was studied in a consecutive single-center series. One hundred fifty-four patients were operated on (1950-2004) for pheochromocytoma (PC=137), or abdominal paraganglioma (PG=17). Twenty had MEN 2 (16 MEN 2A; 4 MEN 2B), 15 von Recklinghausen's disease (VRD), and 1 von Hippel-Lindau (VHL) disease. Twelve patients had, or developed, bilateral adrenal medullary tumors; four with MEN 2A, four with MEN 2B, three with VRD, and one with probably hereditary PC associated with brain tumors/meningioma. Two patients with MEN 2B and one with MEN 2A with had bilateral adrenalectomy (adx). Three VRD patients, two MEN 2B and one MEN 2A patients had cortical-sparing surgery. Two patients were operated on unilaterally, but developed small contralateral tumors; one of these (MEN 2A) had a second asymptomatic PC diagnosed at an older age, so surgery was withheld; the other patient (hereditary PC syndrome) had a small contralateral PC diagnosed at autopsy 9 years later. Only three of nine patients with bilateral operations needed corticosteroid replacement after surgery. Four of six patients died of associated tumors (MTC and meningioma). The mean follow-up was 13 (1-25) years. Twelve MEN 2A patients with unilateral adx have been followed up for 20 (4-36) years without developing a second PC. Cortical-sparing adrenal surgery can safely be performed in the majority of patients with bilateral PC. On the basis of our long-term experience of MEN 2A we perform contralateral adrenal resection only if a second PC is confirmed. Five patients underwent adrenal exploration because of clinical and biochemical findings compatible with PC. Four had asymmetrical positive MIBG scans. They all underwent unilateral adx and diffuse medullary hyperplasia was confirmed (medullary weight estimated morphometrically to 1.0-3.4 g vs. normal weight 0.3-0.5 g in matched controls). These patients have been followed for 19 (5-27) years with normal clinical and biochemical findings. In this rare condition removal of the largest adrenal seems adequate.
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PMID:Treatment of bilateral pheochromocytoma and adrenal medullary hyperplasia. 1710 11

Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day) during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1.
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PMID:Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center. 2258 18