The Journal of clinical endocrinology and metabolism
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J. Clin. Endocrinol. Metab. · May 2014
Randomized Controlled TrialTwo years of Denosumab and teriparatide administration in postmenopausal women with osteoporosis (The DATA Extension Study): a randomized controlled trial.
Current osteoporosis medications increase bone mineral density (BMD) modestly and reduce, but do not eliminate, fracture risk. Attempts to improve efficacy by administering anabolic agents and bisphosphonates concomitantly have been unsuccessful. Conversely, 12 months of concomitant denosumab and teriparatide therapy increases BMD more than either drug alone. ⋯ Two years of concomitant teriparatide and denosumab therapy increases BMD more than therapy with either medication alone and more than has been reported with any current therapy. The combination of these agents may prove to be an important treatment option in patients at high risk of fracture.
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J. Clin. Endocrinol. Metab. · May 2014
Case ReportsDetection of metastatic insulinoma by positron emission tomography with [(68)ga]exendin-4-a case report.
Insulinomas are the most common cause of endogenous hyperinsulinemic hypoglycemia in nondiabetic adult patients. They are usually benign, and curative surgery is the "gold standard" treatment if they can be localized. Malignant insulinomas are seen in less than 10% of patients, and their prognosis is poor. The glucagon like peptide-1 receptor (GLP-1R) is markedly up-regulated in insulinomas-especially benign lesions, which are difficult to localize with current imaging techniques. ⋯ The results of the [(68)Ga]Exendin-4-PET/CT examination governed the treatment strategy of this particular patient and demonstrated the potential of this technique for future management of patients with this rare but potentially fatal disease.
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J. Clin. Endocrinol. Metab. · Apr 2014
Case ReportsCentral precocious puberty in a girl and early puberty in her brother caused by a novel mutation in the MKRN3 gene.
Central precocious puberty (CPP), defined as the development of secondary sex characteristics prior to age 8 years in girls and 9 years in boys, results from the premature activation of the hypothalamic-pituitary-gonadal axis. Mutations in the imprinted gene MKRN3 have been recently implicated in familial cases of CPP. ⋯ We report a novel MKRN3 mutation (p.C340G) in a girl with CPP and her brother with early puberty. MKRN3 alterations should be suspected in all cases with familial CPP or early puberty, especially if male patients are also involved or the precocious puberty trend does not follow the usually observed mother-to-daughter inheritance.
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J. Clin. Endocrinol. Metab. · Apr 2014
Evidence for a U-shaped relationship between prehospital vitamin D status and mortality: a cohort study.
The objective of the study was to examine the association between prehospital serum 25-hydroxyvitamin D [25(OH)D]and the risk of mortality after hospital admission. ⋯ Analysis of 24 094 adult patients showed that 25(OH)D levels less than 20 ng/mL and 60 ng/mL or greater before hospitalization were associated with an increased odds of 90-day mortality. Although previous reports have suggested an association between low vitamin D status and mortality, these data raise the issue of potential harm from high serum 25(OH)D levels, provide a rationale for an upper limit to supplementation, and emphasize the need for caution in the use of extremely high doses of vitamin D among patients.
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J. Clin. Endocrinol. Metab. · Apr 2014
Clinical experience in the screening and management of a large kindred with familial isolated pituitary adenoma due to an aryl hydrocarbon receptor interacting protein (AIP) mutation.
Germline AIP mutations usually cause young-onset acromegaly with low penetrance in a subset of familial isolated pituitary adenoma families. We describe our experience with a large family with R304* AIP mutation and discuss some of the diagnostic dilemmas and management issues. ⋯ Families with AIP mutations present particular problems such as the occurrence of large invasive tumors, poor response to medical treatment, difficulties with fertility and management of pregnancy, and the finding of AIP sequence variants of unknown significance. Because disease mostly develops at a younger age and penetrance is low, the timing and duration of the follow-up of carriers without overt disease requires further study. The psychological and financial impact of prolonged clinical screening must be considered. Excellent relationships between the family, endocrinologists, and geneticists are essential, and ideally these families should be managed in centers with specialist expertise.