| pubmed-article:7258987 | pubmed:abstractText | Nine children with silvery hair have been dermatologically studied: eight out nine presented a context of abnormal sensitivity to infections. The study of pigmentation, granulocytes functions and immunity system allows a separation in several types: 1) 4 patients had a characteristic Chediak-Higashi syndrome, this diagnosis being based on pigmentary and immunohematological grounds. 2) Two siblings exhibited functional disturbance of the Chediak-Higashi type, but without its cytological abnormalities. A third patient resembled closely to this picture, but was insufficiently investigated. 3) The eighth patient has been studied by Drs. Griscelli and Pruniéras who had previously described a new disease on immunological, hematological and pigmentary grounds. All these case with defective polymorphonuclear functions have febrile peaks with accelerated aggravation close to Chediak-Higashi syndrome. 4) The ninth patient with silvery hair does not have any immunological abnormalities, nor any accelerated phase at the age of eight but suffers from diffuse epilepsy, cerebellar and chorioretinal atrophy. | lld:pubmed |
