chediak - higashi syndrome (chs) is a rare, primary immunodeficiency disorder with an autosomal recessive (ar) inheritance and characterized by re¬current infection, partial occulocutaneous albinism and an accelerated phase. in this report we describe clinical and laboratory findings from 6 chs pa¬tients. clinical and laboratory information of six patients who were referred to our center during...

Phagocytic, antimicrobial, and metabolic functions were studied in leukocytes obtained from three patients with the Chediak-Higashi syndrome (CHS) and compared to normals, individuals, heterozygous for Chediak-Higashi syndrome, and two subjects with chronic granulomatous disease of childhood (CGD). Chediak-Higashi syndrome leukocytes showed normal ingestion of a variety of bacteria, Candida alb...

chédiak-higashi syndrome is a rare autosomal recessive congenital immunodeficiency mainly characterized by a condition called oculo-cutaneous albinism. the affected subjects have light-colored hair, vision problems, blood clotting (coagulation) abnormalities and in adulthood varying neurologic disorders. recurrent infections, particularly viral infection with other disorders in childhood are us...

Chediak Higashi syndrome, is a rare autosomal recessive disorder characterised by oculocutaneus albinism, recurrent respiratory system infections and other pyogenic infections. Hemophagocytic lymphohistiocytosis can develop in any time of the life in patients with Chediak Higashi syndrome. A 14-month-old girl patient was diagnosed as hemophagocytic lymphohistiocytosis with the laboratory findin...

Validation of a method of blood pressure measurement for a study of hypertension in a black African population. Cahiers d'études recherches francophones/santé vasculaire chez l'adulte en milieu rural à Thiadiaye. The prevalence of hyperten-sion and its relationship with obesity: results from a national blood pressure survey in Eritrea. Faciès de l'hypertension artérielle en milieu professionnel...

Studies of granulocyte chemotaxiswere Higashi mink generated normal performed in mink with the Chediakamounts of chemotactic factors. The Higashi syndrome. In vivo migration cellular defect in leukocyte chemotaxis of leukocytes to an inflammatory site in mink is comparable to that observed was reduced in the affected animals. in humans with the Chediak-Higashi In vitro studies documented a cons...

primary immunodeficiencies (pid) are a group of disorders, characterized by an unusual susceptibility to infections. delay in diagnosis results in increased morbidity and mortality in affected patients. the purpose of this study was to determine the mortality rate of iranian immunodeficient patients referred to children medical center hospital affiliated to tehran university of medical sciences...

Impaired vision Photophobia Albinism of the OCA2 type, giving a lighter complexion than unaffected family members [6] Silvery sheen to hair which may be fair in colour Frequent infections (skin, mucous membranes, respiratory) Epilepsy Mental retardation Enlarged liver and spleen Jaundice Ataxia causing incoordination and a typical ataxic gait Tremor Epilepsy Peripheral neuropathy causing motor ...