1/28. osteomalacia in a patient with severe anorexia nervosa.A 27-year-old woman with anorexia nervosa since adolescence was referred to our unit for generalized bone pain most severe at the pelvis and an inability to stand. She reported a pelvic fracture diagnosed one year earlier, which had failed to heal. Laboratory tests showed low serum phosphate, normal total serum calcium corrected for serum albumin, and very low urinary calcium excretion. Serum bone alkaline phosphatase and parathyroid hormone levels were elevated, whereas 25-hydroxy-vitamin D was severely decreased. Multiple vertebral and rib fractures were seen on plain radiographs. Radiographic images consistent with osteomalacia were pseudofractures of the left inferior pubic ramus, a bilateral complete fracture of the superior pubic ramus, and a characteristic pseudofracture (Looser zone) in the lateral margin of the right scapula. Vitamin D-deficient osteomalacia with secondary hyperparathyroidism was strongly suspected at this point, but it was decided not to confirm this diagnosis by bone biopsy with histomorphometry and osteoid labeling because of the emotional instability of the patient. Dual-energy X-ray absorptiometry disclosed severe demineralization. After two months on calcium and vitamin D supplements, the bone pain had abated and the patient was able to stand. Serum calcium had increased; serum phosphate, 25-hydroxy-vitamin D, and parathyroid hormone had returned to normal, and the pseudofractures showed evidence of healing. osteoporosis is a well-known complication of anorexia nervosa. This case shows that osteomalacia can also occur. Vitamin D status should be assessed in patients with long-standing severe anorexia nervosa.- - - - - - - - - - ranking = 1keywords = vitamin (Clic here for more details about this article) |
2/28. Gonadotropin-dependent sexual precocity in a boy affected by pseudohypoparathyroidism.pseudohypoparathyroidism (PHP) is characterized by hypocalcemia and hyperphosphatemia due to PTH resistance. PHP type Ia is due to diminished G(s)alpha activity in several tissues, causing resistance to hormones whose action is mediated by cAMP. Only two cases of males with PTH type Ia who paradoxically showed sexual precocity have been described in the literature. We describe an 11.5 year-old boy affected by PHP without AHO but with associated true precocious puberty, who came to the I.C.U. for tetanic seizures and drowsiness due to severe hypocalcemia. hyperphosphatemia, increased PTH levels and normal 25-OH-vitamin D values were present. Skeletal X-ray showed mild osteopenia. brain MRI revealed symmetric calcifications in basal ganglia and in frontal areas. Thyroid and thyreotropinic function were normal. testosterone levels were in the adult range, as well as basal and stimulated gonadotropin levels. Tanner stage P4, G4; testicular volume 12-15 mi. Molecular cytogenetics studies are now underway to further elucidate the etiology of this form of PHP.- - - - - - - - - - ranking = 0.33333333333333keywords = vitamin (Clic here for more details about this article) |
3/28. Severe osteopenia in a young boy with Kostmann's congenital neutropenia treated with granulocyte colony-stimulating factor: suggested therapeutic approach.Kostmann's syndrome is a congenital disorder that causes an impairment of myeloid differentiation in the bone marrow characterized by severe neutropenia, which can be treated with recombinant human granulocyte colony-stimulating factor (G-CSF). We present the case of a 13-year-old boy with Kostmann's syndrome who was treated with recombinant human G-CSF from age 3.5 years. His growth and development was normal, although complicated by intermittent infections. Bone mineral density (BMD) measurement revealed severe osteopenia at the spine and hips (lumbar spine BMD 0.486 g/cm(2); Z score -3.6), and he was referred to the Endocrine Service. Relevant laboratory evaluation showed a pretreatment ionized calcium level at the upper limit of normal (1.28 mmol/L; range: 1.13-1.32 mmol/L), suppressed intact parathyroid hormone (iPTH) level (12 pg/mL; range: 10-65 pg/mL), and a low 1,25-dihydroxy vitamin D level (21 pg/mL; range: 24-65 pg/mL). He had evidence of increased bone turnover evidenced by elevated urinary deoxypyridinoline (DPD) cross-links (46.9 nmol/mmol creatinine; range: 2-34 nmol/mmol creatinine) and a simultaneous increase in markers of bone formation with elevated osteocalcin level (200 ng/mL; normal: 20-80 ng/mL) and alkaline phosphatase level (236 IU/mL; normal: 38-126 IU/mL). Because of clinical concern for his skeletal health, bisphosphonate therapy with intravenous pamidronate was initiated. One month after treatment, the iPTH and DPD cross-links were in the normal range (54 pg/mL and 17.7 nmol/mmol creatinine, respectively) and the 1,25-dihydroxy vitamin D level was elevated (111 pg/mL). Four months after treatment, there was a striking increase in BMD at the lumbar spine ( 30.86%), femoral necks (left, 20.02%; right, 17.98%), and total hips (left, 18.40%; right, 15.94%). Seven months after bisphosphonate therapy, his biochemical parameters showed a return toward pretreatment levels with increasing urinary DPD cross-links (28.7 nmol/mmol creatinine) and decreasing iPTH (26 pg/mL). However, the BMD continued to increase (8 months posttreatment), but the magnitude of the increment was attenuated (lumbar-spine, 4.8%; left total hip, 1.2% and right total hip 2.4%), relative to BMD at 4 months. Eight months after the initial treatment, his iPTH was suppressed at 14 pg/mL and he again received pamidronate (at a lower dose); 3 months later, he had an additional increase in BMD (lumbar spine 7.4%, left total hip 3.9%, right total hip 2.7%), relative to the previous study. We hypothesize that prolonged administration of G-CSF as treatment for Kostmann's syndrome is associated with increased bone resorption, mediated by osteoclast activation and leading to bone loss. In children, the resulting osteopenia can be successfully managed with antisreorptive bisphosphonate therapy with significant improvement in bone density. Measurements of biochemical parameters of bone turnover can be used to monitor the magnitude and duration of the therapeutic response and the need for BMD reassessment and, perhaps, retreatment.- - - - - - - - - - ranking = 0.66666666666667keywords = vitamin (Clic here for more details about this article) |
4/28. The pathophysiology and management of renal bone disease in dialysis patients.As renal function declines, changes in mineral metabolism occur including phosphate retention, calcitriol deficiency, and the development of secondary hyperparathyroidism. Renal bone disease related to disordered mineral metabolism may result in increased patient morbidity and mortality. Uncontrolled parathyroid hormone (PTH) secretion will result in osteitis fibrosa, a high turnover bone disease. The use of calcium and aluminum-based phosphate binders and vitamin D sterols may contribute to the development of low turnover bone diseases such as osteomalacia and aplastic bone disease. Prevention and control of renal bone disease is an important goal for the interdisciplinary health care team. This paper discusses disordered mineral metabolism and its relationship to renal bone disease. Case studies illustrate the development and treatment of renal bone disease related to secondary hyperparathyroidism and aluminum intoxication.- - - - - - - - - - ranking = 0.33333333333333keywords = vitamin (Clic here for more details about this article) |
5/28. Severe metabolic bone disease as a long-term complication of obesity surgery.BACKGROUND: Metabolic bone disease is a well-documented long-term complication of obesity surgery. It is often undiagnosed, or misdiagnosed, because of lack of physician and patient awareness. Abnormalities in calcium and vitamin D metabolism begin shortly after gastrointestinal bypass operations; however, clinical and biochemical evidence of metabolic bone disease may not be detected until many years later. CASE REPORT: A 57-year-old woman presented with severe hypocalcemia, vitamin d deficiency, and radiographic evidence of osteomalacia, 17 years after vertical banded gastroplasty and Roux-en-Y gastric bypass. Following these operations, she was diagnosed with a variety of medical disorders based on symptoms that, in retrospect, could have been attributed to metabolic bone disease. Additionally, she had serum metabolic abnormalities that were consistent with metabolic bone disease years before this presentation. Radiographic evidence of osteomalacia at the time of presentation suggests that her condition was advanced, and went undiagnosed for many years. These symptoms and laboratory and radiographic abnormalities most likely were a result of the long-term malabsorptive effects of gastric bypass, food intake restriction, or a combination of the two. CONCLUSION: This case illustrates not only the importance of informed consent in patients undergoing obesity operations, but also the importance of adequate follow-up for patients who have undergone these procedures. A thorough history and physical examination, a high index of clinical suspicion, and careful long-term follow-up, with specific laboratory testing, are needed to detect early metabolic bone disease in these patients.- - - - - - - - - - ranking = 0.66666666666667keywords = vitamin (Clic here for more details about this article) |
6/28. Fractures in biliary atresia misinterpreted as child abuse.Bone fractures in children without a history of injury are highly suspicious for child abuse. biliary atresia is a disorder associated with metabolic bone disease, and there are numerous reports of osteopenia, rickets, and/or fractures in this population. We report 3 cases of children with biliary atresia who had bony fractures as well as osteopenia whose caretakers were investigated for child abuse. Pediatricians should be aware of an increased incidence of fractures and overall prevalence of bone disease in this population.- - - - - - - - - - ranking = 5.1564867427521keywords = rickets (Clic here for more details about this article) |
7/28. Primary osteopenia in a female military flight crewmember.This report describes a case of primary severe osteopenia in a 35-yr-old female crewmember of a USAF transport aircraft. This previously healthy individual presented with musculoskeletal symptoms that led to the incidental finding of severe osteopenia on plain films of the pelvis. The subsequent evaluation, including dual energy X-ray absorptiometry (DEXA) showed a T-score of 1-2 SD below sex-adjusted expected levels for age. work-up revealed no secondary causes and the patient was placed on alendronate sodium, vitamin D, and calcium supplementation. Within 2 yr, her bone studies were normal. The crewmember was returned to normal flight status and has had no aeromedical problems. Osteopenia and osteoporosis represent points on a continuum of bone disease. Recognizing and addressing risk factors for osteoporosis earlier may reduce the chances for fractures later in life. Aeromedical specialists must be cognizant of the increased risk for bone disease in female crewmembers.- - - - - - - - - - ranking = 0.33333333333333keywords = vitamin (Clic here for more details about this article) |
8/28. Inherited vitamin k deficiency: case report and review of literature.Vitamin K is the cofactor for the hepatic carboxylation of glutamic acid residues in a number of proteins including the procoagulants factors II, VII, IX, and X. The role of vitamin K in normal bone function is not fully understood. Inherited deficiency of vitamin K dependent coagulation factors is a rare bleeding disorder reported only in a few patients. Here we present an 18-month old child who presented with osteopeni due to inherited vitamin k deficiency. While the patient had high bone specific alkaline phosphatase and parathyroid hormone levels and low osteocalcin and bone mineral density values, with the regular supplementation of vitamin K all the mentioned parameters returned to normal values.- - - - - - - - - - ranking = 2.6666666666667keywords = vitamin (Clic here for more details about this article) |
9/28. Hungry bone syndrome in a child following parathyroid surgery.The rare hungry bone syndrome was encountered in a 15-year-old child after the removal of a parathyroid adenoma. Contrary to the hypocalcemias caused by the removal of all parathyroid glands or transient ischemia after parathyroid surgery, in which the serum inorganic phosphorus level is usually normal, both serum calcium and inorganic phosphorus levels are decreased in hungry bone syndrome in the early postoperative period. Vigorous calcium supplementation and vitamin D are required for prolonged periods.- - - - - - - - - - ranking = 0.33333333333333keywords = vitamin (Clic here for more details about this article) |
10/28. 7: Treatment of osteoporosis: why, whom, when and how to treat. The single most important consideration is the individual's absolute risk of fracture.All women and men with a history of fragility fractures should be considered for treatment of osteoporosis to reduce their risk of future fracture. There is high-level evidence for the anti-fracture efficacy of treatment in women with osteoporosis, particularly if there is prevalent fracture; the evidence is less compelling for women with osteopenia, with or without a fracture, and for men. The rigorously investigated drugs reported to reduce vertebral fractures are the bisphosphonates alendronate and risedronate, the selective oestrogen-receptor modulator raloxifene, the anabolic agent parathyroid hormone and, most recently, strontium ranelate. Only the two bisphosphonates and hormone replacement therapy (HRT) have been reported to reduce hip fractures in community-dwelling women, and calcium plus vitamin D and hip protectors have been reported to reduce these fractures in elderly people in institutions. HRT is not recommended in women for fracture risk reduction alone. Evidence for the anti-fracture efficacy of calcitonin, fluoride, anabolic steroids and active vitamin D metabolites is insufficient to justify their use; lifestyle changes, while not shown to reduce fracture risk, may have a role in maintaining bone strength throughout life.- - - - - - - - - - ranking = 0.66666666666667keywords = vitamin (Clic here for more details about this article) |
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