Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3331940 | Hematology/Oncology Clinics of North America | 2009 | 16 Pages |
Abstract
Shwachman-Diamond syndrome is a rare autosomal-recessive, multisystem disease characterized by exocrine pancreatic insufficiency, impaired hematopoiesis, and leukemia predisposition. Other clinical features include skeletal, immunologic, hepatic, and cardiac disorders. This article focuses on the clinical presentation, diagnostic work-up, clinical management, and treatment of patients with Shwachman-Diamond syndrome.
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Authors
Lauri MD, Ann MD, Akiko MD, PhD,