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Chediak-Higashi Syndrome

A form of phagocyte bactericidal dysfunction characterized by unusual oculocutaneous albinism, high incidence of lymphoreticular neoplasms, and recurrent pyogenic infections. In many cell types, abnormal lysosomes are present leading to defective pigment distribution and abnormal neutrophil functions. The disease is transmitted by autosomal recessive inheritance and a similar disorder occurs in the beige mouse, the Aleutian mink, and albino Hereford cattle.
Also Known As:
Chediak-Steinbrinck-Higashi Syndrome; Oculocutaneous Albinism with Leukocyte Defect; Chediak Higashi Syndrome; Chediak Steinbrinck Higashi Syndrome; Chediak-Steinbrinck-Higashi Syndromes
Networked: 219 relevant articles (5 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Chronic Granulomatous Disease
2. Myeloperoxidase Deficiency
3. Lung Injury
4. Infections
5. Hermanski-Pudlak Syndrome (Hermansky-Pudlak Syndrome)

Experts

1. De Lozanne, Arturo: 6 articles (05/2013 - 01/2002)
2. Gahl, William A: 3 articles (04/2016 - 01/2010)
3. Fischer, Alain: 3 articles (02/2015 - 07/2005)
4. de Saint Basile, Geneviève: 3 articles (02/2015 - 07/2005)
5. Kasai, Hirotake: 3 articles (07/2014 - 07/2007)
6. Tanabe, Fuminori: 3 articles (07/2014 - 07/2007)
7. Introne, Wendy J: 2 articles (04/2016 - 02/2013)
8. Burgess, Agathe: 2 articles (02/2015 - 05/2009)
9. Ménasché, Gaël: 2 articles (02/2015 - 08/2006)
10. Simonsen, Anne: 2 articles (12/2014 - 08/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chediak-Higashi Syndrome:
1. Ascorbic Acid (Vitamin C)FDA LinkGeneric
2. Peroxidase (Myeloperoxidase)IBA
3. SilverIBA
4. Specific Granule DeficiencyIBA
5. Cyclic NucleotidesIBA
6. Cholinergic Agents (Cholinergics)IBA
7. ColchicineFDA LinkGeneric
8. Proteins (Proteins, Gene)FDA Link
9. Serotonin (5 Hydroxytryptamine)IBA
10. EnzymesIBA

Therapies and Procedures

1. Therapeutics
2. Bone Marrow Transplantation (Transplantation, Bone Marrow)
3. Stem Cell Transplantation
03/01/2015 - "Successful stem cell transplantation in Chediak-Higashi syndrome."
05/01/2014 - "Chediak-Higashi syndrome (CHS) is a rare, autosomal, recessive lysosomal disorder with hematological and immunologic abnormalities; however, stem-cell transplantation from a matched or related donor may be curative. "
08/29/2018 - "Neurologic disorders in Chediak-Higashi syndrome are usually late presentations and also may manifest long after bone marrow stem cell transplantation. "
01/01/2009 - "Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. "
02/01/2013 - "Stem cell transplantation was performed for treatment of various diseases such as acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, beta-thalassemia major, sickle- cell thalassemia, sickle- cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combined immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. "
4. Transplantation
5. Splenectomy