Latent hypoparathyroidism in an osteoporotic patient with multiple endocrinopathies and secondary hemochromatosis due to multiple blood transfusions, unmasked by alendronate and glucocorticoid at adrenal crisis
ABSTRACT A 30-year-old normocalcemic man with hypopituitarism, hypogonadism, diabetes mellitus, and secondary hemochromatosis due to multiple blood transfusions was admitted because of adrenal crisis. After intravenous administration of saline and cortisol, the corrected serum level of calcium decreased to 7.3 mg/dl. This osteoporotic patient had been prescribed alendronate for radial bone fracture. Since the increase in intact PTH (68 pg/ml) was impaired compared to that seen in hypocalcemic patients with secondary hyperparathyroidism, we presume that the patient has had latent hypoparathyroidism, which was unmasked by the administration of glucocorticoid and bisphosphonate. With a supplemented dose of 1alpha-OHD3, the patient has been eucalcemic.
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ABSTRACT: BACKGROUND: Transfusional iron overload primarily results in reticuloendothelial iron accumulation, which is considered to be less harmful than parenchymal iron accumulation. However, systematic and comprehensive data on endocrine function in transfusion-associated hemochromatosis are limited. METHODS: We examined 25 aplastic anemia patients (11 men and 14 women) diagnosed with transfusion-associated hemochromatosis at a single institution. Pituitary function was determined with a combined pituitary function test. On a different day, a 75 g oral glucose tolerance test was performed. The bone mineral density (BMD) of the lumbar spine and total hip was assessed with dual-energy X-ray absorptiometry. RESULTS: Twenty-two (88%) of these 25 patients had at least one endocrine abnormality, and 12 had more than one abnormality. The most common pituitary hormonal deficiency involved the pituitary-gonadal axis; 54% of the total subjects had hypogonadotropic hypogonadism. Two patients had an insufficient cortisol response to corticotropin-releasing hormone stimulation. No patient had a deficiency of growth hormone or thyroid-stimulating hormone. Twelve (48%) had diabetes mellitus and these patients tended to have higher concentrations of ferritin, alanine aminotransferase, and γ-glutamyl transferase. Osteoporosis (T-score < -2.5 SD) was observed in 48% of patients. The reduction in BMD was more pronounced in the lumbar spine than in the total hip. The patients with osteoporosis were accompanied by hypogonadism, which predominantly affected the trabecular bone. CONCLUSIONS: Our observations suggest that endocrinopathies are common in transfusion-associated hemochromatosis. © 2012 Blackwell Publishing Ltd.Clinical Endocrinology 07/2012; DOI:10.1111/j.1365-2265.2012.04495.x · 3.35 Impact Factor
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ABSTRACT: Context:Normocalcemic primary hyperparathyroidism is typically identified after referral to a specialty clinic. At diagnosis, patients demonstrate features seen in hypercalcemic primary hyperparathyroidism. Normocalcemic hypoparathyroidism has been discovered following hypocalcemia unmasked after bisphosphonate administration.Objective: We hypothesized that screening unselected, non-referral populations, such as The Osteoporotic Fractures in Men (MrOS) study and Dallas Heart Study (DHS), would identify asymptomatic subjects with normocalcemic hyperparathyroidism and hypoparathyroidism.Methods:Normocalcemic hyperparathyroidism was defined as serum PTH greater than the upper reference range with normal albumin-adjusted serum calcium, excluding common secondary causes [renal failure (estimated glomerular filtration rate <60 mL/min), 25-hydroxyvitamin D <20 ng/mL, thiazide use], and normocalcemic hypoparathyroidism as PTH below the reference range with normocalcemia. Cross-sectional data was obtained from MrOS, and longitudinal data (baseline and 8 years) from DHS.Results:In 2364 men from MrOS, we identified 9 with normocalcemic hyperparathyroidism (prevalence 0.4%) and 26 with normocalcemic hypoparathyroidism (1.1%). In 3450 men and women from DHS, we identified 108 with normocalcemic hyperparathyroidism (3.1%) and 68 with normocalcemic hypoparathyroidism (1.9%). Of the 108 normocalcemic hyperparathyroid subjects, 64 had follow-up data. Hypercalcemic primary hyperparathyroidism developed in 1 subject while 13 (0.6% of the follow-up cohort) showed persistently elevated PTH levels with normocalcemia. Of the 26 normocalcemic hypoparathyroid subjects with follow-up data, none developed overt hypoparathyroidism and 2 (0.09%) had persistent evidence of normocalcemic hypoparathyroidism.Conclusions:This study documents normocalcemic primary hyperparathyroidism and hypoparathyroidism identified among community-dwelling individuals. Larger studies are needed to determine the true prevalence and natural history of these parathyroid disorders.The Journal of Clinical Endocrinology and Metabolism 05/2013; 98(7). DOI:10.1210/jc.2013-1300 · 6.31 Impact Factor
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ABSTRACT: Bisphosphonates (BPs) are the first-line therapy for osteoporosis. In recent years, atypical femoral fractures (AFF) have been described in patients on BPs therapy. However, the relationship between BPs and AFF remains to be clarified. We evaluated clinical and hormonal characteristics of AFF patients, in order to determine AFF risk factors. We studied 11 females with AFF and 58 females with typical femoral fractures (TFF), admitted to our Department for surgical repair between January 2008 and December 2011. All AFF patients received BPs therapy for 6 to 13 yrs, whereas 36.2% (p<0.0001) of TFF patients received BPs for shorter period (TFF, 6.1±1.8 yr vs. AFF, 8.6±1.9 yr, p<0.0001). A higher prevalence of hypocalcemia was observed in AFF patients compared with TFF (p<0.02), with significantly (p<0.05) lower corrected calcium levels in AFF patients. By contrast a reduced prevalence of elevated PTH levels (p<0.05) was found in AFF patients. No significant difference in prevalence of vitamin D defect was observed between the two groups. Younger age (p<0.004), higher BMI (>30 kg/m(2), p<0.03) and early menopausal age (p<0.05) were observed in AFF patients. At time of fracture, prevalence of osteopenia/osteoporosis and levels of bone turnover markers were significantly (p<0.01) lower in AFF compared with TFF patients. By multivariate analysis hypocalcemia, obesity, and younger age (<70 yr) were confirmed to be independent predictors of AFF; elevated PTH level was the predominant independent protective factor (p<0.004). In conclusion, our data indicate that clinical characteristics and metabolic factors may favour the development of AFF in BP treated patients. We identified hypocalcemia due to latent hypoparathyroidism as primary risk factor for AFF; age, obesity, early menopause, and BMD may also influence the development of AFF. An adequate clinical and metabolic assessment is suggested to prevent the development of AFF in BP treated patients.Bone 07/2013; 56(2). DOI:10.1016/j.bone.2013.07.010 · 4.46 Impact Factor