Cases reported "Osteopetrosis"

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1/7. osteopetrosis.

    This case study describes the fairly rare entity of osteopetrosis and points out how a seemingly insignificant "anemia" can, on thorough investigation, be found to be a very significant disease with a poor prognosis.
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2/7. Autosomal dominant osteopetrosis type II with "malignant" presentation: further support for heterogeneity?

    The osteopetroses are a heterogeneous group of disorders characterised by generalised bony sclerosis. The autosomal dominant form usually has a "benign" prognosis, in contrast to the "malignant" course of the autosomal recessive variety. In this paper we describe a kindred in which the phenotypic spectrum varied from an asymptomatic condition in adults to a severely affected infant, presenting with anaemia, hepatosplenomegaly, hydrocephalus and blindness. The findings in this family are reported and discussed to elucidate further the possible genetic heterogeneity in autosomal dominant osteopetrosis.
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3/7. Osteomesopyknosis. Report of two new cases.

    Two inherited cases of osteomesopyknosis are reported. This is an autosomal dominant osteosclerosis described by Maroteaux in 1980; its radiological abnormalities consist of increased density of the vertebral plates, pelvis, and sometimes of the upper part of the femur. Osteomesopyknosis, usually discovered incidentally on radiographic examination, is a mild form of familial osteosclerosis and must be distinguished from osteopetrosis which carries a worse prognosis.
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4/7. Oral aspects of osteopetrosis.

    The characteristic feature of osteopetrosis is a lack of osteoclastic activity, leading to a series of somatic problems for afflicted persons. The life span of osteopetrotic patients has increased in recent years, thereby making oral aspects of the disease more evident. Four children with malignant osteopetrosis, born between 1967 and 1975, were examined. In all patients the anterior teeth were of normal shape, and erupted on schedule. Primary molars and all permanent teeth were greatly distorted, and remained totally or partly embedded in basal bone. Vertical growth of alveolar ridge was very limited. Where a fenestration of overlaying mucosa had occurred, a localized progressive osteitis developed, leading to soft tissue inflammation and, in two cases, extraoral mandibular fistulas. Peridontal attachment was very poor, spontaneous exfoliation had occurred in all patients. In two children tooth germs and necrotic bone were surgically removed. No beneficial effect of the treatment was observed. Large doses of antibiotics were needed to control recurring infections. No means of curing progressive osseous destruction of mandibular bone has been found. The general prognosis is poor.
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5/7. osteopetrosis in south africa. The benign, lethal and intermediate forms.

    osteopetrosis is an unusual bone disorder in which the skeleton is radiographically dense. The condition is conventionally subclassified into a benign autosomal dominant adult form and a malignant autosomal recessive variety. Among 14 affected individuals whom we have studied, 4 adults had an intermediate type of osteopetrosis in which serious complications included osteomyelitis, pathological fractures and dyshaemopoiesis. The fundamental biochemical relationship of this disorder with the classic forms of osteopetrosis is uncertain. The osteopetroses must be distinguished from other sclerosing bone conditions which have a different course and prognosis. Sclerosteosis and craniometaphyseal dysplasia, both of which occur in south africa, are of practical importance in this context.
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6/7. Albers-Schonberg disease complicated with periodontal disease. Report of a case with 36-year history.

    A patient had the benign form of Albers-Schonberg disease complicated by periodontitis. review of the patient's 36-year history revealed that the long-term prognosis can be good. Determining factors in the prognosis are the severity of bony involvement and proper periodontal and dental maintenance.
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7/7. Intermediate form of osteopetrosis with recessive inheritance.

    The clinical and radiographic features of the intermediate form of osteopetrosis in two sibs are presented in which the disorder appears to have been inherited as a recessive trait. Although this form of osteopetrosis has been poorly delineated, its recognition is practically important in order to give an accurate prognosis. This paper also presents an unusual complication of bilateral avascular necrosis of the femoral head in the younger sib. Radiographic changes of the femoral heads suggest those of legg-calve-perthes disease, though the possibility of avascular necrosis following unrecognized femoral neck fracture is not completely excluded.
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