• Title/Summary/Keyword: 골연화증(骨軟化症)

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Osteomalacia and Hypophosphatemia Caused by Phosphaturic Mesenchymal Tumor Mixed Connective Tissue Variant (PMTMCT) -A Case Report- (골연화증과 저인산혈증을 유발한 인산뇨성 간엽성 종양 -증례 보고-)

  • Jeong, Jae-Yun;Kim, June-Hyuk;Lee, Sang-Hoon;Kim, Han-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.10 no.2
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    • pp.124-129
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    • 2004
  • The phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) is an extremely rare disease, and is frequently associated with oncogenic osteomalacia showing an paraneoplastic syndrome, which is characterized by phosphaturia, hypophosphatemia, normocalcemia and decreased levels of 1,25-dihydroxyvitamin D3 associated with a tumor. We experienced a 45-year-old female who had a soft tissue tumor on her right buttock causing oncogenic osteomalacia, which was satisfactorily treated by surgical excision of the mass.

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TUMOR INDUCED OSTEOMALACIA : ASSOCIATED WITH GIANT CELL GRANULOMA ON THE GINGIVA (치은부에 발생한 거대세포육아종에 의한 골연화증)

  • Kim, Yeo-Gab;Ryu, Dong-Mok;Lee, Sang Chull
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.13 no.2
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    • pp.185-190
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    • 1991
  • Thr rickets or osteomalacia, that was induced by nonendocrine osseous or soft tissue tumor, is extremely rare disease and fourteen patients has been reported since 1947. The real nature of this disease is unknown, but postulated that unknown phosphaturic subtance which was elaborated from the tumor affect the renal tubule and produce hypophosphatemia and failure of calcification of osseous tissue. This case presented is that of 41-year-old man who suffered from severe generalized aching pain, severe muscular dystrophy, and shortening of the stature 4 years prior hospitalization. The causal coexisting tumor is walnut sized peripheral giant cell granuloma on the upper gingiva. After surgical removal of the tumor, patient's biochemical findings of the serum and urine were returned to the normal limits 12 days later, and clinical symptoms were marked relieved at 6 weeks later. The dental radiograms which were obtained 4 months later revealed remarkable bone regeneration and newly formed alveolar lamina dura.

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TUMOR-INDUCED HYPOPHOSPHATEMIC OSTEOMALACIA : Report of Three Cases (종양유발성 골연화증의 3예)

  • Kim Soh-Hyun;Oh Bong-Hyun;Hang Eui-Hwan;Lee Sang-Rae
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.25 no.1
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    • pp.141-150
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    • 1995
  • 종양유발성 골연화증은 종양에 의하여 생성된 활동성 펩티드호르몬과 유사한 물질이 골이나 위장관에 작용하여 칼슘대사와 인대사의 변화를 일으키거나, 정상적인 비타민 D대사에 장애를 일으킴으로써 2차적으로 발생되는 대사성 질환으로 알려져 있다. 이의 발생빈도는 매우 낮으며, 임상적으로는 체중과 신장의 감소, 전신적인 골격의 동통 및 근무력증과 병적 골절이 동반되어 나타난다. 방사선사진에서는 골소주의 전반적인 소실, 피질골의 비박, 위골절, 치조백선의 소실 등을 보이고, 실험실 소견에서는 혈청인의 농도와 신세뇨관에서 인의 재흡수율의 감소 및 혈청 알칼리성 인산화효소 농도의 상승이 관찰된다. 본질환은 종양이 제거되면 별도의 부가적인 치료 없이도 임상적, 방사선학적 소견 및 실험실 소견의 뚜렷한 개선을 보이므로 이의 치료에 있어서 정확한 진단이 매우 중요하다. 저자들은 전신적인 동통과 근무력증을 주소로 내원한 환자들에게서 발생된 종양유발성 골연화증 3예를 경험하였기에 문헌고찰과 함께 이를 보고하는 바이다.

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A Literature Study of The Osteomalacia (골연화증(骨軟化症)에 대(對)한 동서의학적(東西醫學的) 문헌고찰(文獻考察))

  • Park, Jong-Hyuck;Hwang, Young-Geun;Jeong, Ji-Gheon
    • The Journal of Dong Guk Oriental Medicine
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    • v.8 no.1
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    • pp.159-169
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    • 1999
  • Osteomalacia is syndrome of diverse etiology. characterized pathophysiologically by a failure of normal mineralization of bone and epiphyseal cartilage. This study was performed to investigate causes of disease, pathogenic mechanisms, symptoms, therapies and precriptions through the successive medical literatures. recent chinese medical literatures and chinese medical journals. It is similar to atrophic debility of bones, bone leaning, bone exhaustion, rheumatism involving the bone, osteodynia and cold and heat of bone etc. of oriental medicine. The most principal cause of this is deficiency of kidney. similar to hypophosphatemia caused by increased renal clearance and deficiency of vitamin D, and the rest are senility, deficiency of spleen, deficiency of qi and deficiency of blood. There are nourishing the kidney and spleen, nourishing the qi and blood, warming and passing the muscle and mac, passing an articulation an invigorating the muscle and bone, in principal therapy. And in medical herbs are rehmanniae radix preparat, corni fructus, discoreae rhizoma, cuscutae semen, tigridis os, juglandis semen, hominis placenta, drynariae rhizoma, eucommiae cortex, cynomorii herba, cervi cornus colla, cervi pantotrichum cornu, moutan cortex, polygoni multiflori radix, angelicae gigantis radix, achyranthis bidentatae radix, cibotii rhizoma, hirudo, eupolyphaga, spatholobi caulis, salviae miltiorrhizae radix, draconis resina, curcumae longae rhizoma. In care there are a sun-bath, exercise, high protein diet and taking vitamin D. And they reduce smoking, coffee, drinking etc.

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DENTAL RADIOGRAPHIC STUDY ON THE DILANTIN INDUCED OSTEOMALACIA (Dilantin에 의한 골연화증에 관한 치과방사선학적 연구)

  • Kim Yung Gul;Yu Dong Soo
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.13 no.1
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    • pp.87-96
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    • 1983
  • Radiographic measurements on the width of mandibular cortical plate and the lamina dura and on the root length were done in 42 patients who were in long-term Dilantin medication. Osteoporosis and root abnormalities were also investigated. The obtained results were as follows: 1. The number of male patients was greater than that of female patients. 2. The width of mandibular cortical plate was thinner in patient group than in control group. 3. There was no significant change in the width of lamina dura between the patient group and control group. 4. The root length of patient group was generally shorter than that of control group. 5. There were evidences of generalized mandibular osteoporosis and alteration in mandibular canal wall in 8 patients. (19%) 6. In Dilantin induced osteomalacia, the radiographic changes of mandibular canal wall and mandibular cortical plate were prominent, but that of lamina dura was not significant.

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PATHOLOGICAL STUDY OF CADMIUM POISONING IN OLFACTORY BULB OF RATS (카드뮴 중독이 흰쥐의 후구에 미치는 영향)

  • 여상원;김병우
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1991.06a
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    • pp.17-17
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    • 1991
  • 산업의 발전과 함께 최근 카드뮴 중독이 사회적 문제가 되고 있다. 카드뮴 중독은 특히 건전지 제조공장, 카드뮴 광산 등에서 잘 발생하며 카드뮴 분진의 흡입, 음식물내 카드뮴 복합물의 섭취 등에 의해 유발된다. 중독증상으로는 폐부종 및 섬유화, 신부전증, 폐부전증, 고혈압, 생식선 위축, 골연화증, 무취증, itai-itati 질환등을 일으키는 것으로 알려져 있으나 무취증에 대한 정확한 발생기전은 밝혀져 있지 않다. 이에 저자들은 카드뮴 중독을 유발시키기 위하여 흰쥐에 $CaCl_2$ 11.2 mg/kg을 3주일간 매일 피하주사한 후 3주 후에 두개골을 제거하고 후구를 절취한 후 투과전자현미경으로 관찰하여 다음과 같은 결과를 얻었다. 1. 사구체 주위 부위에 있는 사구체 주위 세포의 세포돌기와 신경축삭에 퇴행성 변화가 발생하였다. 2. 외상망 부위와 승모세포, 과랍세포 등에서는 뚜렷한 변화를 발견할 수 없었다.

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Recalcitrant Low Back Pain Diagnosed as Hypophosphatemic Osteomalacia Induced by Antiviral Medication (항바이러스제에 의한 저인산성 골연화증으로 진단된 난치성 요통)

  • Chae, Hyun Jun;Won, Jun Hee;Lee, Won Kyung;Kim, Keewon
    • Clinical Pain
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    • v.20 no.2
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    • pp.131-134
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    • 2021
  • We report a rare case of anti-viral agent induced hypophosphatemic osteomalacia presented with localized and radicular pain. A 51-year-old man, who had been taking adefovir for chronic hepatitis, had experienced low back pain radiating to his right thigh for 2 years. With impression of lumbar disc herniation, he underwent magnetic resonance imaging and found multi-level disc herniation with facet joint synovial cysts. He received transforaminal epidural steroid injections, however, symptoms did not improve. To find other possible causes, additional tests were performed. Blood tests revealed hypophosphatemia and increased serum alkaline phosphatase, and osteoporosis was noted in dual-energy X-ray absorptiometry with multiple hot uptakes in bone scan. After replacement of adefovir to entecavir and supplement of phosphate and vitamin D, phosphate level and the clinical symptoms were improved. This is the first to report the presentation of osteomalacia due to anti-viral agent as radicular low back pain with facet synovial cysts.

A Case with Perinatal Hypophosphatasia Caused by the ALPL Mutations (ALPL 유전자의 돌연변이를 가진 양성 주산기 저인산증 1례)

  • Kim, Joonil;Kang, Eungu;Kim, Yoon-Myung;Lee, Beom Hee;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.141-147
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    • 2016
  • Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

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Inhibitory Effects of Tenebrio molitor Larvae Ethanol Extract on RANKL-Induced Osteoclast Differentiation (갈색거저리 유충 에탄올 추출물이 RANKL에 의해 유도되는 파골세포 분화에 미치는 영향)

  • Seo, Minchul;Baek, Minhee;Lee, Hwa Jeong;Shin, Yong Pyo;Lee, Joon Ha;Kim, In-Woo;Kim, Mi-Ae;Hwang, Jae-Sam
    • Journal of Life Science
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    • v.30 no.11
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    • pp.983-989
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    • 2020
  • The balance between bone-resorbing osteoclasts and bone-forming osteoblasts is key to bone health. An imbalance between osteoclasts and osteoblasts leads to various bone-related disorders, such as osteoporosis, osteomalacia, and osteopetrosis. However, the bone-resorption inhibitor drugs that are currently used may cause side effects. Natural substances have recently received much attention as therapeutic drugs for the treatment of bone health. This study was designed to determine the effect of Tenebrio molitor larvae ethanol extract (TME) on receptor activator of nuclear factor-κB ligand (RANKL)-induced osteoclast differentiation. To measure the effect of TME on osteoclast differentiation, RAW264.7 cells were treated with RANKL with or without TME for 5 days. The tartrate-resistant acid phosphatase (TRAP) activity was significantly inhibited by treatment of TME without cytotoxicity up to 2 mg/ml. In addition, TME effectively suppressed expression of osteoclast differentiation-related marker genes and proteins such as TRAP, NFATc1, and c-Src. TME also significantly inhibited the p38 mitogen-activated protein kinase (MAPK) signaling pathway without affecting ERK and JNK signaling in RANKL-induced RAW264.7 cells. Consequently, we conclude that TME suppresses osteoclast differentiation by inhibiting RANKL-induced osteoclastogenic genes expression through the p38 MAPK signaling pathways. These results suggest that TME and its bioactive components are potential therapeutics for bone-related diseases such as osteoporosis.