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The intermediate autosomal recessive form usually becomes clinically significant during the first decade of life. These patients will often suffer pathologic fractures and progressive cranial nerve compression neuropathies but typically live into adulthood. There are two subclassifications of autosomal dominant osteopetrosis, and these patients are often asymptomatic into adulthood. Type I typically does not have increased fracture risk and presents with isolated osteosclerotic thickening of the cranial vault. Patients with type II often
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