Chediak-Higashi syndrome

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an inherited disease in humans, cattle, Aleutian mink, white tigers and killer whales. There is dilution of color in the hair and ciliary processes of the eye, with large granules, believed to be lysosomes, in all cell types and particularly noticeable in circulating neutrophils and eosinophils. Leukocytes are defective in chemotaxis and intracellular killing. Affected individuals suffer from an increased susceptibility to infection and bleeding tendencies caused by a platelet storage defect.

Chediak-Higashi syndrome

Chediak-Higashi-Steinbrink syndrome, hereditary leukomelanopathy Molecular medicine A rare AR condition characterized by giant lysosomes and ↑ susceptibility to infections Clinical Partial albinism, lymphadenopathy, hepatosplenomegaly, debilitating neuropathy, photophobia, purulent infections; without BMT most Pts die by age 3 Lab Giant granules in WBCs and platelets, ↓ chemotaxis, bactericidal activity Management BMT, steroids Prognosis Death by age 10

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immune immune complex immune complex disease immune hemolysis immune reaction immune response immune surveillance immune system pseudoalbinism syndrome tapetal			HLH may also occur as a complication of Chediak-Higashi syndrome (35) or after EBV infection in patients with X-linked lymphoproliferative syndrome (36). Hemophagocytic Syndromes and Infection by Fisman, David N. / Emerging Infectious Diseases However, the defect has also been observed in patients with other congenital abnormalities, including Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, Wiskott-Aldrich syndrome, and the syndrome of thrombocytopenia with absent radius. Platelet Storage Pool Deficiency of [Alpha] and [Delta] Granules by Biddle, David A.; Neto, Tony G.; Nguyen, Andy N. D. / Archives of Pathology & Laboratory Medicine Chediak-Higashi Syndrome (CHS) CHS is a rare autosomal recessive disorder characterized by partial ocular and cutaneous albinism, a mild bleeding diathesis, and peripheral sensorimotor neuropathy. Comparative Genomics and Host Resistance against Infectious Diseases by Malo, Danielle / Emerging Infectious Diseases			Chauffeur Fracture chauffeur's fracture Chaussier's areola Chayes' attachment ChB CHC CHD ChE Cheating Check check key check ligament check ligament of eyeball check rein check sample check-bite check-list method checkbite checkerboard Checkerboard Assay Checkerboard Pattern checking Checking Compulsion checkpoint checkup Chediak-Higashi syndrome Chédiak-Higashi syndrome Chediak-Steinbrinck-Higashi syndrome cheek Cheek Augmentation cheek muscle cheekbone CHEESE cheese worker's lung cheese-washer's lung cheeseweed cheesewood cheesy Cheesy Abscess Cheesy Bronchitis cheesy gland cheesy necrosis cheetah cheil(o)- cheil- cheilalgia Cheilanthes cheilectomy cheilectropion cheilion cheilitis			Cheddar (town) Cheddar (town) cheddar cheese cheddar cheese cheddar pink cheddar pink Cheddar Vally Advanced Motorcyclists Cheddarhead Pack of Houston Cheddaring Cheddaring Cheddars Cheddars Chedder Chedder Cheddi Jagan Cheddi Jagan International Airport Cheddite Cheddly and Cloud's Crazy Adventure Cheder Cheder cheders cheders Chediak Higashi disease Chediak-Higashi anomaly Chédiak-Higashi anomaly Chédiak-Higashi anomaly Chédiak-Higashi anomaly Chédiak-Higashi anomaly Chédiak-Higashi anomaly Chediak-Higashi disease Chediak-Higashi syndrome Chédiak-Higashi syndrome Chédiak-Higashi syndrome Chédiak-Higashi syndrome Chediak-Higashi Syndrome 1 Chediak-Steinbrinck-Higashi syndrome Chedoke Minor Hockey League Chedorlaomer CHEDRO-VII CHEDS Chedugudu Chedugudu Chee Chee Soon Juan chee-chee Cheeba Cheebah Cheebah Cheebah Cheebah CHEEC Cheech and chong CHEEF cheek cheek cheek cheek Cheek augmentation cheek biting cheek biting cheek biting

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