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Archivos argentinos de pediatría
versão impressa ISSN 0325-0075versão On-line ISSN 1668-3501
Resumo
BUJAN, María M; CERVINI, Andrea B; FANO, Virginia e PIERINI, Adrián M. Albright´s hereditary osteodystrophy: report of three cases. Arch. argent. pediatr. [online]. 2010, vol.108, n.2. ISSN 0325-0075.
Albright's hereditary osteodystrophy includes an heterogeneous group of genetic diseases, including the pseudohypoparathyroidism type Ia and pseudo-pseudohypoparathyroidism. Features of this disease are: round faces, over-weight, subcutaneous calcifications and brachydactylia. Pseudohypoparathyroidism type Ia is associated with laboratory abnormalities such as hypocalcemia, and hyperphosphatemia due to parathormone resistance. Furthermore, these patients may present peripheral resistance to other hormones, such as gonadotrophins and thyroids. Pseudo-pseudohypoparathyroidism has no peripheral resistance to these hormones. We present three patients evaluated in our hospital with clinical manifestations of Albright ´s syndrome.
Palavras-chave : Hereditary osteodystrophy; Albright´s syndrome; Children.