• Title/Summary/Keyword: Osteopetrosis

Search Result 16, Processing Time 0.022 seconds

OSTEOPETROSIS;Report of a case and review of the literature (골석화증에 대한 문헌고찰)

  • Rim, Jae-Suk;Kim, Sung-Moon;Kim, Onn;Kim, Hoi-Jong
    • Maxillofacial Plastic and Reconstructive Surgery
    • /
    • v.11 no.2
    • /
    • pp.61-66
    • /
    • 1989
  • Osteopetrosis (Albers-Schonberg disease) is a rare disease characterized by generaized sclerosis of bones, hepatoslenomegaly, pancytopenia, multiple fracture, blindness. The disease shows characteristic radiographic feature and two rather well-defined pattern have been recognized. We experienced a case of osteopetrosis in a 31 year old man, who has had pus discharge and fetid odor after extraction of upper maxillary molar. We made a brief review of literature.

  • PDF

A Case of Osteopetrosis Including Mandible (하악골 침범을 포함한 골화석증 1예)

  • Shin, Keum-Back;Kim, Do-Yun;Oh, Hee-Myung
    • Journal of Oral Medicine and Pain
    • /
    • v.24 no.1
    • /
    • pp.1-8
    • /
    • 1999
  • The authors experienced a diagnosis of a benign form of osteopetrosis based on the comprehensive data obtained from (1) clinical examination, (2) radiological and nuclear medicinal findings of the thickening of cortex of mandible, femur, clavicle, and the increased bone density of mandible, maxilla, cranial base, calvarium, lumbar, femur, and additionally (3) histopathological findings of sclerotic bony tissue in a Korean male of 38-year-old.

  • PDF

OSTEOPETROSIS WITH MAXILLARY OSTEOMYELITIS (상악골 골수염을 수반한 골화석증)

  • Lee Won-Shik;You Dong-Soo;Park Tae-Won;Choi Soon-Chul
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
    • /
    • v.21 no.2
    • /
    • pp.405-413
    • /
    • 1991
  • The authors observed a 33-year-old male patient who had suffered from osteopetrosis with maxillary osteomyelitis. The obtained results were as follows: 1. Physical examination revealed hepatosplenomegaly and bowing legs. 2. Nearly all bones of the entire skeleton showed unusual increased radiodensity and the paranasal sinuses were markedly obliterated. 3. There were seveal old fractured sites in the femur and hip bones and subluxation of hip joint. 4. The radiodensity of both jaws were prominently increased and there were many malformed teeth and impacted teeth. 5. Bone scan revealed multiple, irregularly increased uptakes in large joints and long bones.

  • PDF

The dental management of infantile osteopetrosis: case report (유아형 골화석증 환아의 구강관리: 증례보고)

  • Lee, Hyo-Seol;Song, Je-Seon;Lee, Jae-Ho
    • The Journal of Korea Assosiation for Disability and Oral Health
    • /
    • v.4 no.2
    • /
    • pp.77-81
    • /
    • 2008
  • 골화석증(Osteopetrosis)은 전신의 뼈가 대리석 모양으로 골 경화를 일으키며 골수강의 폐쇄 및 골의 취약성을 보이는 드문 질환이다. 성인형(Adult form)은 우성 유전되며 경미한 증상을 나타낸다. 반면에 유아형(Infantile form)은 열성 유전되면서 심각한 증상을 나타내고, 조기에 사망에 이를 수도 있다. 골화석증은 골격계, 조혈계, 신경계에서 다양한 증상을 유발한다. 본 증례의 목적은 드물게 발생하는 유아형 골화석증을 가진 2 명의 환아를 치료한 후, 그 질환과 치과적 관리에 대해 고찰하고자 함이다. 첫 번째 증례는 골화석증으로 진단된 5세 8개월 남환이었다. 임상 및 방사선 검진 결과 악골 및 치아의 변형을 나타내었으나, 특별한 병적 소견이 없어 예방 치료 및 주기적인 검진 시행하였다. 두 번째 증례는 중증도의 치아우식증을 가진 환아로 의료진과의 협조 하에 수혈한 후 발치하였다. 유아형 골화석증은 다양한 구강 합병증을 유발하며, 질환의 심각성에 의해 치료 자체도 어렵다. 치과의사로서 골화석증 환아를 관리할 때에는 건전한 구강 상태를 유지하기 위한 지속적인 관심과 예방 치료가 필요하며, 아무리 간단한 발치나 치과 술식이더라도 의료진과 협조를 해야 한다.

  • PDF

UNERUPTED PRIMARY MOLAR (미맹출 유구치에 관한 증례)

  • Han, Yeon-Sun;Choi, Byung-Jai;Kim, Seong-Oh;Lee, Chong-Gap
    • Journal of the korean academy of Pediatric Dentistry
    • /
    • v.29 no.3
    • /
    • pp.444-449
    • /
    • 2002
  • The term 'impaction' is used to designate a tooth which remains unerupted in the jaw beyond the time at which it should normally be erupted. The main causal factors are local (lack of space, ectopic positions of teeth, supernumerary teeth, cyst, the occurrence of infectious process in the eruption path, traumatic facial injury etc.). Systemic and genetic disorders, however, may have primary failure of eruption and retarded eruption as additional symptoms (cleidocranial dysplasia, osteopetrosis etc.). Most cases of impacted teeth reported in the literature are of permanent teeth. The absence of primary teeth occur rarely whereas impaction of second primary molars is more numerous than all other impactions. Impaction due to primary failure of eruption must be distinguished from the secondary infraocclusion. The etiology of impaction of primary teeth is probably related to early ankylosis of primary teeth, but it is not clear. Failure of eruption of primary teeth may cause a number of complications, such as interference with development and eruption of succedaneous teeth, formation of cyst, and damage to adjacent teeth. This study is to report cases of primary failure of eruption in the primary dentition.

  • PDF

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
    • /
    • v.30 no.11
    • /
    • pp.983-989
    • /
    • 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.