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Download Citation on ResearchGate | [Osteogenesis imperfecta (Lobstein’s disease) and its surgical treatment] | Osteogenesis imperfecta, of which the authors. remodelado ventricular, Lisofosfatidilcolina, Lobstein, Jean, 2 Losartàn , de &bypass» de arteria coronaria, Probabilidad de enfermedad, LVIII. Color Amarillo Depende, de la impregnación de bilirrubina (ictericia) En la enfermedad de Lobstein y Eddoves, las escleróticas son de color.

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Willem Vrolik did work on the condition in the s. Additional information Further information on this disease Classification s 3 Gene s 0 Other website s 0. This often involves hydrotherapy and the use of support cushions to improve posture. Sodium fluoride has been reported to slow the progression of otosclerosis lesions, and the consequent sensorineural hearing loss.

Osteogenesis imperfecta

Type II can be further subclassified into groups A, B, C, which are distinguished by radiographic evaluation of the long bones and ribs.

This condition is mainly observed in otosclerosis, but is also found in chronic otitis media with tympanosclerosis, and other rare bone diseases such as Paget’s disease and osteogenesis imperfecta Lobstein disease. Retrieved 20 May Abnormalities of dermal fibrous and elastic tissue Skeletal disorders Collagen disease. Continuing to use this site, you agree with this. People with OI are born with defective connective tissue, or without the ability to make it, usually because of a deficiency of Type-I collagen.

In all the above etiologies, the progression of the hearing loss is generally slow. Further characterized by the “V Triad” consisting of a radio-opaque band adjacent to growth plates, b hypertrophic calluses at fracture sites, and c calcification of the radio – ulnar interosseous membrane. Frequency is approximately the same across groups, but for unknown reasons the Shona and Ndebele of Zimbabwe seem to have a higher proportion of Type III to Type I than other groups.


According to recent results, extended periods of pamidrinate, i. Look at other dictionaries: Some children are under a study of pamidronate.

The current four type system began with Sillence in Jean Lobstein dealt with it in adults in The prevalence of symptomatic otosclerosis is estimated as 0. Among some of the most common alternatives are Ekman-Lobstein syndromeVrolik syndromeand the colloquial glass-bone disease. His treatment proved extremely useful in the rehabilitation and prevention of fractures; it was adopted throughout the world and still forms lobsein basis for orthopedic treatment of OI.

The name osteogenesis imperfecta dates to at least [ 12 ] and has been the usual medical term in the 20th century to present.

The Norse king Ivar the Boneless may have had this condition as well.

Check this box if you wish to receive a copy of your message. Food and Drug Administration FDA will not approve Fosamax as a lobstwin for OI because long term effects of the drug have not been fully researched yet, although it is often used lobsrein preteens, instead of Pamidronate. Osteogenesis imperfecta Osteogenesis imperfecta Classification and external resources The classic blue sclerae of a person with osteogenesis imperfecta ICD- 10 Q Diagnostic methods Otoscopy is normal in otosclerosis, Paget’s disease, and osteogenesis imperfecta.

Otosclerosis appears to be a multifactorial disease.

Orphanet: Sordera progresiva debida a anquilosis de huesecillos

Miller, MD, Sofield wrote a seminal article describing enffrmedad solution that seemed radical at the time: Type IIC demonstrates thin and longer long bones with thin and beaded ribs. At the present time, the cause for Type V is unknown, though doctors have determined that it is inherited. The classic blue sclerae of a person with osteogenesis imperfecta. Only comments seeking to improve the quality and accuracy of enfermmedad on the Orphanet website are accepted.


For all other comments, please send your remarks via contact us. In case of otosclerosis, an audiometry with stapedial reflex assessment should be recommended for the parents, siblings, and children above 20 years of age of the patient. Bisphosphonates BPsparticularly those containing nitrogenare being increasingly administered to increase bone mass and reduce the incidence of fracture. Differential diagnosis should include minor ossicular malformations presenting with a normal otoscopy, a conductive hearing loss, and abolished stapedial reflexes.

Type IIB demonstrates broad and short long bones with thin ribs that have little or no beading. This can make physiotherapy difficult to administer to young children. He described the condition in his doctoral thesis and mentioned cases of it going back to In the past several years, there has been the identification of autosomal recessive forms.

In these varied cases the total number of OIs of all four types was roughly the same as any other ethnicity.

OI Type V leads to calcification of the membrane between the two forearm bones, making it difficult to turn the wrist. Otoscopy is normal in otosclerosis, Paget’s disease, and osteogenesis imperfecta.