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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

For 3 years a 75-year-old man with type II diabetes had been suffering from paroxysmal pruritus in a circumscribed area of brown discoloration of the skin over the left should blade. This condition, also known as notalgia paraesthetica, is a rare, but perhaps underdiagnosed, neurocutaneous entity, a largely sensory neuropathy due to a muscular compression phenomenon. In this case histological examination of a biopsy specimen showed focal acanthosis, obvious basal hyperpigmentation and discrete perivascular lymphocytic infiltration with numerous melanophages. At first, glucocorticoids were administered unsuccessfully against the distressing pruritus. Purely symptomatic local capsaicin treatment decreased the pruritus temporarily. Endocrinological diagnosis failed to demonstrate multiple endocrine neoplasia (MEN) syndrome 2A, which has been frequently described in association with notalgia paraesthetica.
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PMID:[The interdisciplinary aspects of notalgia paraesthetica]. 792 28

The pancreatic somatostatinoma belongs to the type of rare endocrine tumors of the pancreas. We report the observation of a 54 year old woman. Previously she was suffering from diabetes mellitus. An abdominal ultrasonography revealed an endocrine tumor of the pancreatic tail. There was no specific symptomatology, with the exception for the hyperglycaemia. The diagnosis of somatostatinoma was certified post operatively by the immunocytochemistry of the tumor. Then, the patient developed a hypercalcaemia associated with an increase of parathyroid hormone. The surgery of the neck revealed three hyperplastic parathyroids, inducing this association as a multiple endocrine neoplasia type 1 (MEN 1). The patient did not develop pituitary tumor. Afterwards, scintigraphy with 111 Indium- octreotide showed a residual tumor at the head of pancreas. Basal levels of somatostatine and calcium, pentagastrine test, computed tomography scan, arteriography were negative. The presence of a second somatostatinoma was confirmed by surgery and immunohistology. One year after the surgery, the patient remains clinically well. The pancreatic localization of the somatostatinoma in a MEN 1 is poorly documented. Its malignant nature can only be assured by the presence of metastases. The genetic detection of the MEN 1 becomes possible. Above all, the treatment is based on surgery and/or chemotherapy (Fluoro-Uracile; Streptozotocine). In our case, 111 In-octreotide scintigraphy was the only method demonstrating a residual focus, suggesting it could be an element of reference for the diagnosis and survey of somatostatinoma the watch of patients having a treatment for somatostatinoma.
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PMID:[Pancreatic somatostatinoma and MEN 1. Apropos of a case. Review of the literature]. 873 92

THE AUTHORS PRESENT DATA FROM 361, 662 MEN ages 35 to 57, screened from 1973 to 1976 for possible participation in the Multiple Risk Factor Intervention Trial (MRFIT). Volunteers identified themselves as "white," "black," "Oriental," "Spanish American," "American Indian," or "other." They also noted if they were taking medication for diabetes. A trained technician measured blood pressure after participants had rested for 5 minutes, using the fifth Korotkoff sound to define diastolic pressure and averaging the second and third of three readings. Differences among the groups included the following: blacks had consistently higher systolic and diastolic blood pressure (SBP and DBP) than other groups; Orientals had slightly lower pressure than other nonblack groups; American Indians had somewhat higher pressure than other nonblack groups at ages 35 to 44 but lower at ages 45 to 54; Hispanics in Miami and Davis, California, had significantly higher SBP and DBP than whites in the same area; Orientals in California had significantly higher DBP (but not SBP) than whites in California.
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PMID:Blood pressure in minorities screened for the Multiple Risk Factor Intervention Trial (MRFIT). 889 81

Hyperparathyroidism (HPT) can be caused by solitary parathyroid adenomas and carcinomas, and primary and secondary multiglandular parathyroid disease. Primary HPT is also a feature of several hereditary diseases e.g. multiple endocrine neoplasia type 1 and type 2A (MEN1 and 2A), familial hypocalciuric hyperparathyroidism (FHH), the HPT-jaw tumor syndrome (HPT-JT), and familial isolated HPT. Summarizing data from the literature and our own observations, various genetic abnormalities are observed in the pathogenesis of HPT. These include chromosomal deletions of the MEN1 locus on 11q in sporadic and MEN1 associated primary HPT, of RB1 on 13q in carcinomas, and of the FHH gene located on 3q in sporadic primary and secondary HPT. Genetic material is also lost from chromosomes 1p, 6q, 15q and X suggesting loss of yet unidentified tumor suppressor genes in these regions. In addition the HRPT2 gene on 1q, as well as the proto-oncogenes RET on 10q and PRAD1 on 11q are associated with a subset of parathyroid tumors.
Exp Clin Endocrinol Diabetes 1996
PMID:Molecular genetics of primary and secondary hyperparathyroidism. 898 Oct 14

In 34 families out of 35 with hereditary medullary thyroid cancer a mutation in the RET proto-ongene could be identified. In 84 family members gene carrier state could be proven, in 75 family members gene carrier state could be excluded. The majority of gene carriers (67 out of 84) were symptomatic while 17 gene carriers were diagnosed in a presymptomatic state. 9 of the 17 presymptomatic gene carriers had prophylactic thyroidectomy. On histological examination C-cell hyperplasia or multifocal microcarcinomas could be proven. In one of our families with familial MTC no germline mutation in the RET gene could be detected. In this family pentagastrintests and indirect genotyping are necessary. Four family members of MEN 2A families have had thyroidectomy on the basis of pentagastrin testing and now proved not to be gene carriers. The application of genetic testing in families with hereditary thyroid carcinoma has further improved management in these families - it is the first step in the evaluation of family members at risk.
Exp Clin Endocrinol Diabetes 1996
PMID:Application of genetic screening in families with hereditary medullary thyroid carcinoma. 898 Oct 15

Multiple endocrine neoplasia type 1 (MEN1) is tightly linked to the muscle-type glycogen phosphorylase (PYGM) gene in 11q13. This region of the human genome contains additional disease-related loci implicated in the development of insulin-dependent diabetes mellitus, familial paraganglioma type 2, spinocerebellar ataxia type 5, Bardet-Biedl syndrome and translocation t(11;17) described in B-cell non-Hodgkin's lymphoma. We approached cloning of candidate disease genes from 11q13 by large-scale genomic sequencing. We obtained > 106 kb of sequence around the PYGM gene and established a transcriptional map that includes: (i) two genes previously localized to 11q13, PYGM and a zinc-finger protein (ZFM1) gene; (ii) the germinal center kinase (GCK, human B-lymphocyte serine/threonine protein kinase) gene; (iii) a novel human CDC25-like (HCDC25L) gene; (iv) a dystrophia myotonica protein kinase-like (DMPKL) gene; and (v) a novel ubiquitously expressed gene of unknown function (germinal center kinase- neighboring gene, GCKNG).
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PMID:The germinal center kinase gene and a novel CDC25-like gene are located in the vicinity of the PYGM gene on 11q13. 934 81

We experienced a case of MEN type 2a with bilateral and large pheochromocytomas. A 39-year-old man was admitted to the previous hospital with complaints of paroxysmal headache. hypertension and diabetes mellitus. Radiographic imagings showed thyroid tumors in both lobes and bilateral adrenal tumors. Thyroid tumors were histologically proved to be medullary thyroid carcinoma by needle biopsy and systemic investigations revealed an excessive secretion of plasma and urinary cathecholamines which suggested the presence of pheochromocytoma. The patient was diagnosed as MEN type 2a. He was admitted to our hospital for the treatment of bilateral adrenal tumors for which we performed one-stage bilateral adrenalectomy by thoracoabdmonal approach. Both adrenal tumors were histologically confirmed as pheochromocytoma. The patient's postoperative course was uneventful. He underwent uneventful total thyroidectomy approximately 2 months after bilateral adrenalectomy. Even in bilateral and large pheochromocytomas, one-stage bilateral adrenarectomyenables safe postoperative managements. We concluded that the thoracoabdominal approach is feasible in the patients with huge and cranially spreading adrenal tumor, which gives us a wide operative field for easy vascular control.
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PMID:[One-stage-bilateral adrenalectomy by thoracoabdominal approach for bilateral large pheochromocytomas in men type IIa. A case report]. 938 71

A 5-cm pheochromocytoma located in segment 7 of the liver was found incidentally in a 45-year-old man with mild diabetes mellitus and hypertension, and resected. The tumor was demonstrated by computed tomography and magnetic resonance imaging to have completely invaginated itself into the liver and to be receiving blood from a dilated right hepatic artery alone. Surgery revealed the hepatic mass to be tightly adherent to the right adrenal gland. The histopathologic diagnosis was pheochromocytoma growing exophytically from the right adrenal gland. There was no association with multiple endocrine neoplasia type 1 and type 2. A postoperative 131I metaiodobenzylguanidine scan revealed no accumulation, and the patient is currently doing well without recurrence or hypertension one year after the operation. A pheochromocytoma deeply invaginating into the liver should be considered in the differential diagnosis of primary hypervascular hepatic tumors.
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PMID:Pheochromocytoma growing exophytically from the right adrenal gland and invaginating into the liver. 939 Feb 15

Thyroid cancer is a rare disease in childhood and adolescence. However, it represents the most frequent cancer type in this age group. Thyroid cancer amounts about 0.5%-1.5% of all malignancies in children and adolescents. In Germany 10-30 cases could be expected in a year. The most common histologic type for this age group is the differentiated thyroid cancer (DTC), i.e. the papillary and follicular subtypes (90%). In 10% of patients medullary thyroid cancer (MTC) will be diagnosed. DTC occur more often in girls than in boys (female/male-ratio 2:1), with a median age of about 12-13 years (yrs). An important aetiological factor of DTC is a former exposition to different kinds of radiation. In childhood and adolescence MTC mostly appears in patients suffering from the syndrome MEN-2, whereas the sporadic form is rare. As in DTC, more girls than boys can be diagnosed for MTC (female/male-ratio 2-3:1), with a median age about 10 yrs. The anaplastic/undifferentiated subtype occurs extremely rare. For all types of thyroid cancer the most important therapeutic approach is the surgical intervention. In most cases of DTC an optimal disease control could be achieved by radioiodine therapy. Prognosis of DTC in children and adolescents is favourable. However, therapeutic strategies and modalities reported in the literature are very different. In contrast, prognosis of MTC is rather poor, that of the anaplastic type is infaust. No other effective therapy option than surgery is available. To evaluate the therapeutic efficacy of a combined modality therapy by both radiotherapy and cytostatic drugs in children and adolescents with poor prognosis types of thyroid cancer, an interdisciplinary multicenter therapy study will now be started in Germany.
Exp Clin Endocrinol Diabetes 1997
PMID:Epidemiology and therapy of thyroid cancer in childhood and adolescence. 943 21

In 11 MEN 2A gene carriers prophylactic thyroidectomy was carried out between the age of 4 to 17 years. All gene carriers had pathological basal and/or pentagastrin stimulated serum calcitonin levels. On histological examination in five patients C-cell hyperplasia (CCH) and in 6 patients medullary microcarcinoma (< 1 cm, mostly multifocally) was shown. No patient had lymph node involvement. There was no recurrent laryngeal nerve damage in these 11 patients and no permanent hypoparathyroidism. After prophylactic thyroidectomy all 11 patients were cured, they showed normal basal and pentagastrin stimulated calcitonin levels. In the follow-up (mean 19 months) under thyroxine substitution therapy in 9 of these 11 patients TSH was at least at one examination out of the normal range. In 2 patients TSH was suppressed, in 1 of these FT3 was slightly elevated. In 7 patients TSH was elevated between 4.9 to 147 microU/ml. The application of genetic testing is save concerning diagnostic accuracy. If prophylactic thyroidectomy is done at early ages cure rate is 100%. Despite this encouraging results thyroxine substitution therapy in the follow-up carries some problems concerning optimal dosage and non-compliance. Therefore TSH has to be estimated in the follow-up every 3 months.
Exp Clin Endocrinol Diabetes 1997
PMID:Results and follow-up in eleven MEN 2A gene carriers after prophylactic thyroidectomy. 943 23


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