Endocrinology and Metabolism

Review Article

Calcium & bone metabolism
Acquired Forms of Fibroblast Growth Factor 23-Related Hypophosphatemic Osteomalacia: Nobuaki Ito, Naoko Hidaka, Hajime Kato; Endocrinol Metab. 2024;39(2):255-261. Published online March 11, 2024; DOI: https://doi.org/10.3803/EnM.2023.1908

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Fibroblast growth factor 23 (FGF23) is a pivotal humoral factor for the regulation of serum phosphate levels and was first identified in patients with autosomal dominant hypophosphatemic rickets and tumor-induced osteomalacia (TIO), the most common form of acquired FGF23-related hypophosphatemic rickets/osteomalacia (FGF23rHR). After the identification of FGF23, many other inherited and acquired forms of FGF23rHR were reported. In this review article, the detailed features of each acquired FGF23rHR are discussed, including TIO, ectopic FGF23 syndrome with malignancy, fibrous dysplasia/McCune-Albright syndrome, Schimmelpenning-Feuerstein-Mims syndrome/cutaneous skeletal hypophosphatemia syndrome, intravenous iron preparation-induced FGF23rHR, alcohol consumption-induced FGF23rHR, and post-kidney transplantation hypophosphatemia. Then, an approach for the differential diagnosis and therapeutic options for each disorder are concisely introduced. Currently, the majority of endocrinologists might only consider TIO when encountering patients with acquired FGF23rHR; an adequate differential diagnosis can reduce medical costs and invasive procedures such as positron emission tomography/computed tomography and venous sampling to identify FGF23-producing tumors. Furthermore, some acquired FGF23rHRs, such as intravenous iron preparation/alcohol consumption-induced FGF23rHR, require only cessation of drugs or alcohol to achieve full recovery from osteomalacia.

Citations

Citations to this article as recorded by

Paraneoplastic endocrine syndromes: a contemporary overview
Juan Eduardo Quiroz-Aldave, Jacsel Suarez-Rojas, Elman Rolando Gamarra-Osorio, Katia Rivera-Fabián, María Del Carmen Durand-Vásquez, Luis Alberto Concepción-Urteaga, José Paz-Ibarra, Marcio José Concepción-Zavaleta
Expert Review of Endocrinology & Metabolism.2025; : 1. CrossRef
Acquired hypophosphatemic osteomalacia: case series from a Peruvian referral center (1999–2023)
José Paz-Ibarra, Sofía Sáenz-Bustamante, Manuel Inostroza-Fernández, Paola Sifuentes Hermenegildo, Liliana Ancajima Lescano, Marcio Concepción-Zavaleta, Alejandro Román-González, Alfredo Adolfo Reza-Albarrán
Archives of Osteoporosis.2024;[Epub] CrossRef

Original Article

Sporadic Nonfamilial Hypophosphatemic Osteomalacia.: Young Kee Shong, Joong Yeol Park, Ghi Su Kim, You Sook Cho, Goo Yeong Cho, Sang Wook Kim, Jung Sik Park, Ki Up Lee; J Korean Endocr Soc. 1994;9(1):25-31. Published online November 6, 2019

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Abstract PDF: Chronic hypophosphatemia caused by decreased intestinal absorption or increased renal clearance, may lead to rickets or osteomalacia independently of other predisposing abnormalities. The conditions commonly associated with increased renal clearance of phosphate are X-linked hypophosphatemic rickets, tumor associated rickets/osteomalacia, RTA and Fanconi syndrome. Recently we experienced 3 men with adult-onset, histologically proven osteomalacia associated with increased renal clearance of phosphate. None of them had a family history of bone disease, tumors or other tubular defects. All of these had remarkable biochemical and clinical improvement with medical treatment such as 1, 25-dihydroxyvitamin D and phosphate supplementation. Although we did not find tumors yet, we could not rule out the possibility of tumor-associated osteomalcia since it often takes several years to make a diagnosis because of small size, benign nature and unusual location of tumors. Thus, careful long-term follow up for tumor occurrence will be maintained in these patients with sporadic nonfamilial hypophosphatemic osteomalacia.

Review Article

Vitamin D: A D-Lightful Vitamin for Health.: Michael F Holick; Endocrinol Metab. 2012;27(4):255-267. Published online December 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.4.255

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Abstract PDF

Vitamin D is a sunshine vitamin that has been produced on this earth for more than 500 million years. Because foods contain so little vitamin D most humans have always depended on sun exposure for their vitamin D requirement. Vitamin D deficiency has been defined as a serum 25-hydroxyvitamin D concentration < 20 ng/mL (50 nmol/L); vitamin D insufficiency as a serum 25-hydroxyvitamin D of 21-29 ng/mL and vitamin D sufficiency as a serum 25-hydroxyvitamin D of 30-100 ng/mL whereas toxicity is usually not seen until blood levels are above 150 ng/mL. Vitamin D deficiency is a global health problem that increases risk for metabolic bone diseases in children and adults as well as many chronic illnesses including autoimmune diseases, type 2 diabetes, cardiovascular disease, infectious disease, and cancer. The major causes of vitamin D deficiency are lack of adequate sensible exposure to sunlight, inadequate dietary intake and obesity. The United States Endocrine Society recommended that to prevent vitamin D deficiency in those at risk, children 1 year and older require 600-1,000 international unit (IU) of vitamin D daily and adults require 1,500-2,000 IU of vitamin D daily. Obese patients require 2-3 times more vitamin D to both treat and prevent vitamin D deficiency.

Citations

Citations to this article as recorded by

Higher intakes of dietary caffeine are associated with 25-hydroxyvitamin D deficiency
Qiwei Chen, Hamed Kord-Varkaneh, Heitor O. Santos, Rafael Genario, Minyan Dang
International Journal for Vitamin and Nutrition Research.2022; 92(2): 85. CrossRef
WITHDRAWN: Higher intakes of dietary caffeine are associated with 25-hydroxyvitamin D deficiency: a study from the NHANES
Fang Yang, Ning Wang
Nutrition.2021; : 111380. CrossRef
Association of metabolic syndrome and 25‐hydroxyvitamin D with cognitive impairment among elderly Koreans
Eun Young Lee, Su Jin Lee, Kyoung Min Kim, Young Mi Yun, Bo Mi Song, Jong Eun Kim, Hyeon Chang Kim, Yumie Rhee, Yoosik Youm, Chang Oh Kim
Geriatrics & Gerontology International.2017; 17(7): 1069. CrossRef
Korean Society for Bone and Mineral Research Task Force Report: Perspectives on Intermittent High-dose Vitamin D Supplementation
Han Seok Choi, Yong-Ki Min, Dong Won Byun, Myung Hoon Hahn, Kyoung Min Kim, Beom Jun Kim, Ki-Won Oh
Journal of Bone Metabolism.2017; 24(3): 141. CrossRef
Efficacy and safety of vitamin D3 B.O.N intramuscular injection in Korean adults with vitamin D deficiency
Han Seok Choi, Yoon-Sok Chung, Yong Jun Choi, Da Hea Seo, Sung-Kil Lim
Osteoporosis and Sarcopenia.2016; 2(4): 228. CrossRef
Endocrine Risk Factors for Cognitive Impairment
Jae Hoon Moon
Endocrinology and Metabolism.2016; 31(2): 185. CrossRef
The effect of thyroid stimulating hormone suppressive therapy on bone geometry in the hip area of patients with differentiated thyroid carcinoma
Jae Hoon Moon, Kyong Yeun Jung, Kyoung Min Kim, Sung Hee Choi, Soo Lim, Young Joo Park, Do Joon Park, Hak Chul Jang
Bone.2016; 83: 104. CrossRef
Serum 25‐hydroxyvitamin D level and the risk of mild cognitive impairment and dementia: the Korean Longitudinal Study on Health and Aging (KLoSHA)
J.H. Moon, S. Lim, J.W. Han, K.M. Kim, S.H. Choi, K.W. Kim, H.C. Jang
Clinical Endocrinology.2015; 83(1): 36. CrossRef
Vitamin D Status in Korea
Han Seok Choi
Endocrinology and Metabolism.2013; 28(1): 12. CrossRef

Case Reports

1-34 PTH Could Reverse Impaired Bone Mineralization Induced By the Overdose of Bisphosphonate.: Kyeong Hye Park, Kwang Joon Kim, Han Seok Choi, Kyoung Min Kim, Eun Young Lee, Seonhui Han, Hyun Sil Kim, Daham Kim, Hannah Seok, Eun Yeong Choe, Yumie Rhee, Sung Kil Lim; Endocrinol Metab. 2012;27(3):247-250. Published online September 19, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.3.247

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Abstract PDF: Bisphosphonates are the mainstay of osteoporosis treatment. Despite the fact that bisphosphonates have a relatively good safety record and are tolerated well by the majority of patients, serious adverse events have been associated with their use. A 41-year-old man had been diagnosed with osteoporosis and had taken etidronate 200 mg/day daily for 2 years due to the judgmental error. He was referred for the management of refractory bone pain and generalized muscle ache. Serum calcium, phosphate, 25-hydroxy-vitamin D (25(OH)D), and immunoreactive parathyroid hormone (iPTH) were within normal range. Plain X-ray showed multiple fractures. Whole body bone scan confirmed multiple sites of increased bone uptakes. Tetracycline-labeled bone biopsy showed typical findings of osteomalacia. He was diagnosed with iatrogenic, etidronate-induced osteomalacia. The patient received daily parathyroid hormone (PTH) injection for 18 months. PTH effectively reverses impaired bone mineralization caused by etidronate misuse. Currently, he is doing well without bone pain. Bone mineral density significantly increased, and the increased bone uptake was almost normalized after 18 months. This case seems to suggest that human PTH (1-34) therapy, possibly in association with calcium and vitamin D, is associated with important clinical improvements in patients with impaired bone mineralization due to the side effect of bisphosphonate.

A Case of Osteomalacia with Multiple Fractures and Hypocalcemia Associated with Phenytoin Therapy.: Eun Kyung Kim, Min Suk Lee, Yoon Sok Chung, Kyu Sung Kwack, Ji Man Hong, Ye Yeon Won; J Korean Endocr Soc. 2009;24(3):212-216. Published online September 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.3.212

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Abstract PDF: Many studies have shown that patients taking antiepileptic drugs are at an increased risk for metabolic bone disease and low bone mineral density. Traditionally, this has been attributed to alterations in vitamin D metabolism by antiepileptic drugs which induce hepatic microsomal cytochrome P450 enzyme. However, there appear to be multiple mechanisms for antiepileptic drug-induced bone loss including lack of physical activity, reduced sunlight exposure, increased propensity for falling, and fractures associated with seizures or loss of consciousness. We experienced a case of antiepileptic drug-induced osteomalacia in a 63-year-old woman who had been on phenytoin for 8 years and was admitted with hypocalcemic seizures and multiple pathological fractures. This patient also had other risk factors for osteomalacia including reduced sunlight exposure, prolonged immobilization, and decreased dietary vitamin D intake. We discontinued phenytoin, and started calcium and vitamin D replacement. The patient's serum calcium and vitamin D level were normalized after treatment. Metabolic bone disease including osteomalacia should be considered in patients who are taking antiepileptic drugs especially those who are exposed to other risk factors.

A Case of Tumor-induced Osteomalacia with Elevated Fibroblast Growth Factor-23.: Hae Sung Kim, Hyun Seung Jung, Hee Jung Kim, Sung Yeon Kim, Sang Wan Kim, Chan Soo Shin, Chong Jai Kim, Seong Yeon Kim; J Korean Endocr Soc. 2007;22(2):142-148. Published online April 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.2.142

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Abstract PDF

Tumor-induced osteomalacia (TIO), a paraneoplastic disease, is characterized by hypophosphatemia, and caused by renal phosphate wasting inappropriately, normal or decreased 1, 25(OH)2D3 production, and defective calcification of cartilage and bone. Because the removal of the responsible tumor normalizes phosphate metabolism, unidentified humoral phosphaturic factors (phosphatonin) are believed to be responsible for this syndrome. These factors include fibroblast growth factor (FGF)-23, secreted frizzled-related protein-4 and matrix extracellular phosphoglycoprotein. However, no case of TIO producing FGF-23 has been clearly reported in Korea. Herein, a case of TIO producing FGF-23 in a 45-year-old woman is reported. The patient presented with a large tumor on her buttock, with severe bone and muscle pain. A histological examination of the tumor revealed a mixed connective tissue tumor, consisting of deposition of calcified materials and surrounding primitive spindle cells, with prominent vascularity. Whether FGF-23 is a secreted factor, as well as its levels of expression in tumors were investigated. An immunohistochemical study showed the tumor cells to be FGF-23 positive. Furthermore, the levels of serum FGF-23 were extremely high and an RT-PCR analysis, using total RNA from the tumor, revealed the abundant expression of FGF-23 mRNA. After removal of the tumor, all the biochemical and hormonal abnormalities disappeared, with marked symptomatic improvement.

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Eun Jung Lee, Ji Hoon Kim, Hye Jin Hwang, Min Ki Kim, Min Seok Kang, Kyung-Su Kim
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Dietary assessment according to frequency of food consumed away from home among children and adolescents: Based on the 2010~2012 Korea National Health and Nutrition Examination Survey
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Pediatric Gastroenterology, Hepatology & Nutrition.2014; 17(2): 85. CrossRef
Length and Volume of Morphologically Normal Kidneys in Korean Children: Ultrasound Measurement and Estimation Using Body Size
Jun-Hwee Kim, Myung-Joon Kim, Sok Hwan Lim, Jieun Kim, Mi-Jung Lee
Korean Journal of Radiology.2013; 14(4): 677. CrossRef
Factors Influencing Obesity among Adolescent: Analysis of 2011 Korean Youth Risk Behavior Survey
Young-Ju Jee, Young-Hae Kim
The Korean Journal of Obesity.2013; 22(1): 39. CrossRef
A Case of Nutritional Osteomalacia in Young Adult Male
Choong-Kyun Noh, Min-Jeong Lee, Bu Kyung Kim, Yoon-Sok Chung
Journal of Bone Metabolism.2013; 20(1): 51. CrossRef
The Relationship between High Energy/Low Nutrient Food Consumption and Obesity among Korean Children and Adolescents
Gyu Jin Heo, So-Young Nam, Soo-Kyung Lee, Sang-Jin Chung, Ji hyun Yoon
Korean Journal of Community Nutrition.2012; 17(2): 226. CrossRef
Changes in Prevalence of Obesity and Underweight among Korean Children and Adolescents: 1998-2008
Ki Eun Kim, Shin Hye Kim, Sangshin Park, Young-Ho Khang, Mi Jung Park
The Korean Journal of Obesity.2012; 21(4): 228. CrossRef
A new age-based formula for estimating weight of Korean children
Jungho Park, Young Ho Kwak, Do Kyun Kim, Jae Yun Jung, Jin Hee Lee, Hye Young Jang, Hahn Bom Kim, Ki Jeong Hong
Resuscitation.2012; 83(9): 1129. CrossRef
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Shin Hye Kim, Byung Chul Ahn, Hyojee Joung, Mi Jung Park
Endocrinology and Metabolism.2012; 27(3): 208. CrossRef
Trends in obesity among Korean children using four different criteria
Young-Ho Khang, Mi Jung Park
International Journal of Pediatric Obesity.2011; 6(3-4): 206. CrossRef
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Joo Hyun Gil, Mi Na Lee, Hye Ah Lee, Hyesook Park, Jeong Wan Seo
Korean Journal of Pediatric Gastroenterology and Nutrition.2010; 13(2): 180. CrossRef

A Case of Osteomalacia Caused by Severe Vitamin D Deficiency.: Seok Yeon Kim, Sang Youl Rhee, Soo Young Moon, Suk Chon, In Kyung Jeong, Seungjoon Oh, Kyu Jeung Ahn, Deog Yoon Kim, Ho Yeon Chung, Sung Woon Kim, Jin Woo Kim, Young Seol Kim, Jeong Taek Woo; J Korean Endocr Soc. 2007;22(1):55-61. Published online February 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.1.55

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Abstract PDF

Vitamin D is a kind of hormone that can be synthesized in the skin or it is supplied in the diet it is involved in a central role in bone and calcium homeostasis in vivo. Metabolic bone diseases such as rickets in children and osteomalacia in adults can also be caused by deficiency or metabolic defects in the vitamin D hormone related system. However, there has been a lack of generalized epidemiologic studies about the vitamin D deficiency status in Koreans. We recently experienced a case of osteomalacia caused by severe vitamin D deficiency. These kinds of case have not been reported elsewhere in Korea for the last twenty years.

Citations

Citations to this article as recorded by

A Case of Nutritional Osteomalacia in Young Adult Male
Choong-Kyun Noh, Min-Jeong Lee, Bu Kyung Kim, Yoon-Sok Chung
Journal of Bone Metabolism.2013; 20(1): 51. CrossRef

A Case of Sporadic Nonfamilial Hypophosphatemic Osteomalacia.: Jun Goo Kang, Dong Sun Kim, Chan Bum Choi, Tae Jong Kim, Jong Pyo Kim, Chang Beom Lee, Yong Soo Park, You Hern Ahn, Tae Wha Kim, Sang Cheol Bae, Chan Gum Park; J Korean Endocr Soc. 2002;17(4):610-616. Published online August 1, 2002

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Abstract PDF: Acquired hypophosphatemic rickets, or osteomalacia, requires the recognition of the typical clinical and radiological features of osteomalacia in association with hypophosphatemia, which is caused by the decrease in intestinal absorption or impaired renal tubular phosphate reabsorption. The latter form may either be hereditary or acquired. Acquired hypophosphatemic osteomalacia includes oncogenic osteomalacia, neurofibromatosis, fibrous dysplasia, renal tubular acidosis and sporadic nonfamilial hypophosphatemic osteomalacia. A 33-year-old man presented with bone pain, progressive severe muscle weakness and a height loss of more than 10 cm over a 5 year period. The familiy history was negative for bone disease or other renal tubular defects. He was found to have hypophosphatemia, impaired phosphate reabsorption, normocalcemia, normal vitamin D metabolite levels, normal PTH and elevated alkaline phophatase. A bone biopsy showed thickened unmineralized osteoid compared to pelvic bone in control cases. Clinical symptoms, such as bone pain and muscle weakness, were improved after supplementation of oral phosphorus and calcitriol, although the serum phosphorus level did not normalize.

Development of Tertiary Hyperparathyroidism during the Treatment of Hypophosphatemic Osteomalacia.: W S Shinn, S Lim, Y M Cho, T Y Kim, M K Moon, D J Park, K S Park, S Y Kim, B Y Cho, H K Lee, Y K Youn, S K Oh; J Korean Endocr Soc. 2001;16(4-5):520-527. Published online October 1, 2001

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Abstract PDF: Osteomalacia is characterized by the accumulation of an increased amount of unmineralized bone matrix. Of the several possible causes, hypophosphatemia is one of the major and mainly results from intestinal malabsorption of phosphate or from renal tubular phosphate loss. Oral phosphate supplementation is the mainstay of therapy for hypophosphatemic osteomalacia of diverse causes, but secondary hyperparathyroidism may be induced by such treatment. Although concomitant therapy with vitamin D is recommended for the prevention of this complication, it can nevertheless occur and sometimes results in tertiary hyperparathyroidism. We report the development of tertiary hyperparathyroidism in a patient with sporadic non-familial hypophosphatemic osteomalacia who had been treated with long-term phosphate therapy despite undergoing concomitant vitamin D therapy.

A Case of Adult Fanconi's Syndrome with Glomerular Podocyte Foot Process Effacement and osteomalacia Induced by k Light Chain Disease.: Jae Hyun Nam, Gyu Hyun Choi, Ki Hyun Park, Chul Woo Ahn, Young Duk Song, Sung Kil Lim, Hyun Chul Lee, Kap Bum Huh; J Korean Endocr Soc. 2000;15(4-5):627-633. Published online January 1, 2001

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Abstract PDF: The Fanconi's syndrome is characterized by generalized disturbance of proximal tubular function. It leads to excessive losses of amino acids, glucose, phosphate, bicarbonate, and other substrates handled by the proximal tubules. The metabolic consequences are acidosis, hypophosphatemia, hypocalcemia, osteomalacia, osteoporosis, and growth retardation. Adult Fanconi's syndrome is mostly secondary form caused by multiple myeloma, primary amyloidosis, light chain nephropathy, and heavy metal poisoning. We experienced 50-year-old woman with kappa light chain disease whose chief complaints were weakness of both lower extremities and multiple bone pain. This patient had renal glycosuria, hypercalciuria, normal anion gap type metabolic acidosis, osteomalacia and normal distal tubule acidification. Her bone marrow biopsy showed inappropriate proliferation of plasma cell. The patient underwent percutaneous renal biopsy in which was exceptionally observed focal effacement of podocyte foot process. So we report a case of osteomalacia caused by adult Fanconi's syndrome and foot process effacement by kappa -light chain disease.

Original Article

A Case of Osteomalacia with Long Term Carbamazepine Therapy.: Hee Soo Kim, Dong Sun Kim, Nak Won Choi, Sang Hyun Baik, Sung Hoon Kim, Chang Beom Lee, Yong Soo Park, Woong Hwan Choi, You Hern Ahn, Tae Hwa Kim; J Korean Endocr Soc. 2000;15(2):286-290. Published online January 1, 2001

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Abstract PDF: Anticonvusant therapy with any of several agents, especially phenytoin, phenobarbital, and primidone causes disturbances in bone mineral metabolism. Anticonvulsants stimulate the hepatic microsomal mixed-oxidase enzymes and hence increase the rate of clearance of vitamin D and its metabolism. The severity of clinical manifestations in any given individual appears to be a function of the combined effects of variety of factors including drug type and total drug dose, dietary vitamin D intake, sunlight exposure, and physical activity level. We report a case of osteomalacia associated with long term carbamazepine therapy in a 21-year-old male with less exposure to sunlight.

Case Reports

A Case of Oncogenic Osteomalacia Caused by Chondromyxoid Fibroma.: Ki Won Oh, Moo II Kang, Won Young Lee, Tae Kyu Lee, Jae Hyuck Chang, Jung Pil Suh, Bong Yun Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Jeong Mi Park, Kyo Young Lee, Seung Koo Rhee, Young Kyun Woo; J Korean Endocr Soc. 1999;14(4):764-770. Published online January 1, 2001

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Abstract PDF: Oncogenic osteomalacia is a rare clinicopathological condition. The syndrome is characterized by hypophosphataemic osteomalacia with hyperphosphaturia, low plasma 1,25-dihydroxyvitamin D and normal plasma calcaemia and parathyroid hormone, associated with a tumor, generally of mesenchymal origin. Complete excision of the tumour results in cure of the whole syndrome. Recently we experienced 56-year-old woman with oncogenic osteomalacia caused by a chondromyxoid fibroma of the left foot. We report this case with the review of literatures.

A Case of Osteosarcoma induced Oncogenic Osteomalacia Detected by MRI.: Sung Kil Lim, Young Duk Song, Hyun Chul Lee, Kap Bum Huh, Ki Hyun Park, Kyung Rae Kim, Soon Won Hong, Jae Hyun Nam, Jong In Yook, Byung Joo Choi, Mi Kyung Kim, Kyu Ho Shin; J Korean Endocr Soc. 1999;14(2):401-409. Published online January 1, 2001

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Abstract PDF: Oncogenic osteomalacia is a syndrome characterized by phosphaturia, hypophosphatemia, decreased 1,25-dihydroxyvitamin D level and specific signs and symptoms of osteomalacia. It is associated with the presence of neoplasm originated from mesenchyme. Until now, less than 100 cases of oncogenic osteomalacia have been reported. The pathophysiology of oncogenic osteomalacia has not been fully understood, but it has been suggested that a certain substance released by tumor may inhibit not only la-hydroxylase activity and reduce 1,25-dihydroxyvitamin D level in part, but directly inhibit reabsorption of phosphate. And then, reduced phosphaturia, hypophosphatemia and eventually osteomalacia develop. We report a case of osteosarcoma induced oncogenic osteomalacia detected by MRI in 59 year old woman.

A Case of Adult Fanconi Syndrome and Osteomalacia associated with x-Light Chain Monoclonal Gammopathy.: Chul Hee Kim, Hong Kyu Kim, Shi Jung Chung, Juog Min Ko, Soo Kil Park, Woo Kun Kim, Ki Soo Kim; J Korean Endocr Soc. 1998;13(1):99-107. Published online January 1, 2001

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Abstract PDF: The Fanconi syndrome is a complex tubulopathy, which is characterized by urinary hyperexcretion of amino acids of all classes, phosphate, glucose, bicarbonate, calkium, potassium, and otherions, and proteins with molecular weights under 50,000 daltons. This metabolic disease leads to hypophospatemia, hypokalemia, growth failure, metabolic acidasis, and rickets/osteomalacia. Fanconi syndrome may be inherited or acqulred. Most cases of adult Fanconi syndrome are acquired, and the acquired syndrome is associated with thermal burns, exposure to heavy metals or drugs, vitmnin D deficiency, renal transplantation, or light chain deposition. The most common cause of adult Fanconi syndrome is multiple myeloma. We ribe here a case of adult Fanconi syndrome and osteomalacia associated with x-light chain monoclonal gammopathy. A 47-year-old woman presented with multiple bane pain and proximal muscle weakness for 2 years. Laboratory findmgs showed hypophosphatemia, mild hypocalcemia, marked elevation of serum alkaline phosphatase, metabolic acidosis, low 25-OH- vitamm D level and secondary hyperparathyroidism. Urinary excretion of protein, uric acid, phosphate, and glucose was mcreased, and tubular reabsorption of phosphate was decreased to 50%. Protein immunofixation electrophoresis of serum and urine showed x-light chain type monoclonal gammopathy. Bone marrow examination was normal except moderate elevation of plasma cell component(8.8%). The skeletal radiography showed fractures of both lower ribs and pseudofracture in right femoral lesser trochanter. We treated the patient with calcium, 1.25-(OH)2-vitamin D, phosphorus, bicarbonate, and potassium, and her clinical symptoms were gradually relieved.

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