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Albright syndrome

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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Pseudohypoparathyroidism Ia with Evans syndrome (J Pediatr Hematol Oncol. 2008 Aug;30(8):628-30.). Authors are Pavone P Praticò AD Castellano-Chiodo D et al., from the Department of Paediatrics, University of Catania, Catania, Italy. Pseudohypoparathyroidism Ia (Albright hereditary osteodystrophy or Albright syndrome) is a rare disease, caused by the resistance to the action of the parathyroid hormone in target tissues, such as the bone, kidney, and intestine, with consequent hypocalcemia and hyperphosphatemia and increased levels of parathyroid hormone. The phenotype of Albright syndrome includes 5 common features: brachydactyly, obesity, short stature, a round face, and mental retardation. We report on a child with a classic form of pseudohypoparathyroidism and associated Albright syndrome who developed Evans syndrome (ie, the cooccurrence of severe autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura). To access the full abstract of the article, click here.

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