Chédiak-Higashi syndrome: a case report on an African infant.

An African Kenyan female infant was born with very light skin and ashen grey, scanty hair. At 18 months she presented with a bluish skin pigmentation, hepatosplenomegaly, generalised lymphadenopathy and non-responsive fever. A bone marrow aspirate and peripheral blood examination done revealed characteristic features of the Chédiak-Higashi Syndrome. This is a rare disorder, to our knowledge not previously described in Africans. The case is presented with a brief review of the literature.

Neurological complications associated with sickle cell anaemia: an experience at the Aga Khan Hospital, Nairobi.

Over a period of 18 months, six patients with sickle cell anaemia and neurological complications were seen. Four had acute hemiplegia, one had epilepsy and mental changes and the sixth had cerebral haemorrhage and died in the acute phase. Two patients with hemiplegia had recurrences and three were associated with convulsions and/or aphasia. Three of the patients had fever at presentation while the other three were well at the time of the neurological complication. Two patients had not been diagnosed as sicklers previously. A brief review of the literature on neurological disease in sickle cell anaemia is included.