Clinical manifestation

Type 1: blue sclera; in-utero fractures; mild-to-moderate bone fragility; kyphoscoliosis; hearing loss; premature arcus senilis; easy bruising; short stature Type 2: abnormal dentition; blue sclera; small nose; micrognathia; connective tissue fragility; short trunk

Type 3: abnormal dentition; sclera of variable hue; in-utero fractures; limb shortening and progressive deformities; triangular facies with frontal bossing; pulmonary hypertension

Type 4: normal sclera; normal hearing; fractures beginning in infancy; mild angu-lation and shortening of long bones

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