[Shwachman's syndrome: pancreatic exocrine insufficiency retarded growth, metaphyseal dysostosis and neutropenia (author's transl)].

A boy of 5 8/12 years of age affected with Shwachman's Syndrome is presented. He showed frequent respiratory infections that can be in order with a low account in T-lymphocytes found on immunological study. A cytoplasmic degenerative process was found after microscopical and ultrastructural vision of osteoid tissue, which is compatible with biochemical alterations.