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W S, boy born 23.10.69 History: Parents were second cousins. Baby had very fair hair and grey-pink irides. Admitted aged 18 days with staphylococcal pneumonia; blood film revealed abnormal cytoplasmic granules in the neutrophils. Repeated respiratory infections over next 18 months responded to antibiotics. Persistent neutropenia. Photophobia and rotatory nystagmus; when exposed to sun his fair skin developed urticaria and prominent venules. Whenever he became ill his blond hair changed to silver-grey.