ADULT HYPOPHOSPHATASIA TREATED WITH TERIPARATIDE: REPORT OF 2 PATIENTS AND REVIEW OF THE LITERATURE.

OBJECTIVE: Hypophosphatasia (HPP) is a rare inherited metabolic bone disease from deficient activity of the tissue-nonspecific isoenzyme of alkaline phosphatase (TNSALP). Reportedly, teriparatide (parathyroid hormone 1-34) can benefit the adult form of HPP, including fracture healing. We studied 2 women with adult HPP given teriparatide and reviewed the reports of 6 additional patients. METHODS: A 68-year-old black woman (patient 1) described low-trauma fractures and had subnormal serum alkaline phosphatase (ALP) activity. Biochemical findings were consistent with HPP. Mutation analysis revealed a heterozygous defect in exon 10 of TNSALP (ALPL). Teriparatide was injected daily for 2 years. Four years later, she fractured her right hip. Treatment was resumed for 8 months without further fractures. A 53-year-old white woman (patient 2) reported low-trauma fractures and had subnormal serum ALP. Mutation analysis revealed a heterozygous defect in exon 8 of TNSALP. She injected teriparatide daily for 2 years. One year later, bone mineral density (BMD) declined and treatment was resumed for 3 months. When she sustained a sacral fracture, teriparatide was administered for a further 18 months. RESULTS: Patient 1's serum ALP increased while receiving teriparatide and returned to baseline after its discontinuation. BMD remained unchanged, but no fractures were sustained. Patient 2's serum ALP increased, but the improvement was not sustained. Femoral neck BMD increased significantly during the first cycle, declined significantly afterwards, and was regained during a second course of teriparatide. CONCLUSION: Teriparatide shows some benefit for adult HPP. ABBREVIATIONS: ALP = alkaline phosphatase BMD = bone mineral density BSAP = bone-specific alkaline phosphatase CTX = C-telopeptide DXA = dual-energy X-ray absorptiometry FN = femoral neck HPP = hypophosphatasia LS = lumbar spine PEA = phosphoethanolamine PLP = pyridoxal 5'-phosphate PTH = parathyroid hormone SQ = subcutaneous TNSALP = tissue-nonspecific isoenzyme of alkaline phosphatase TPTD = teriparatide.

SELECTIVE HYPOALDOSTERONISM: A REVIEW.

OBJECTIVE: Selective hypoaldosteronism (SH) is a condition manifested by hyperkalemia due to low aldosterone secretion with normal cortisol. One of the obstacles in diagnosis is the awareness of the condition itself. The objective of this review is to highlight what is known about the epidemiology, pathophysiology, etiology, presentation, diagnosis, and treatment of SH. METHODS: Literature search was performed on PubMed and Ovid Medline for articles which contained hypoaldosteronism as a major topic. RESULTS: The recent literature on this topic is surprisingly limited. Few recent review articles were found, none of which were in English and less than 5 years old. Case reports and genetic literature were also included in this review, as they contain the most recent reports of SH in the literature. CONCLUSION: Awareness about SH will hopefully help physicians to identify patients at risk as well as decide on treatment if any therapy is required.

Calcium use in the management of osteoporosis: continuing questions and controversies.

Calcium is a vital element in the health and maintenance of growing and mature bone. The amount of calcium recommended for ingestion varies by age, and these requirements can be met by dietary sources or calcium supplementation. This article reviews the role of calcium in the body and the benefits and risks to calcium supplementation. The effects of calcium on fracture risk reduction, bone density, and bone turnover markers as well as the conflicting data on cardiovascular events and increased risk of nephrolithiasis associated with supplementation are discussed.

Prevalence of Hypogonadism in Low-Risk Prostate Cancer Survivors.

Increasing evidence, albeit small, suggests that testosterone replacement therapy can be cautiously considered in selected hypogonadal men treated with curative intent for low-risk prostate cancer and without evidence of active disease.

Treating a transgender patient: overview of the guidelines.

Transgenderism and gender dysphoria are becoming more prevalent diagnoses and the patient population is increasing. Primary care practitioners, endocrinologists, and mental health professionals are all part of the medical care team that treats these patients, but family and internal medicine physicians continue to deliver the care in the long term. Transgender medicine is not a strong part of the medical curriculum, and recent studies have shown there is anxiety in new physicians in taking care of these patients. There are many aspects to transgender care that involve different specialities, including, but not limited to, mental health, primary care, endocrinology, surgery, and obstetrics and gynecology. This article gives an overview of the current guidelines for standards of care of transgender patients as delineated by the World Professional Association for Transgender Health and the Endocrine Society.