REPORT OF SIX CASES OF CHEDIAK–HIGASHI SYNDROME WITH REGARD TO CLINICAL AND LABORATORY FINDINGS

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FARHOUDI A.; CHAVOSHZADEH Z.; POURPAK Z.; IZADYAR MINA; GHARAGOZLOU M.; MOVAHEDI M.; MIRSAEID GHAZI B.; REZAEI N.; MOEIN M.; AGHAMOHAMMADI A.

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Journal: Iranian Journal of Allergy, Asthma and Immunology Year:2003 | Volume:2 | Issue:4 Page(s): 189-192

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Title

REPORT OF SIX CASES OF CHEDIAK–HIGASHI SYNDROME WITH REGARD TO CLINICAL AND LABORATORY FINDINGS

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Keywords

CHEDIAK – HIGASHI SYNDROME

PRIMARY IMMUNODEFICIENCY

Abstract

CHEDIAK – HIGASHI SYNDROME (CHS) is a rare, PRIMARY IMMUNODEFICIENCY disorder with an autosomal recessive (AR) inheritance and characterized by recurrent infection, partial occulocutaneous albinism and an accelerated phase.In this report we describe clinical and laboratory findings from 6 CHS patients. Clinical and laboratory information of six patients who were referred to our center during the last 20 years (from 1983 – 2003) were reviewed. Onset age of disease was between 3 months to 10 years. All patients had history of consanguineous parents and two patients were siblings. All patients had oculocutaneous albinism, nystagmus, recurrent infections which included upper and lower respiratory tract (U&LRT) infections, stomatitis, thrush, and skin abscesses and hepatitis. In laboratory findings, all patients had neutropenia and normal immunoglobulins and normal CD3, CD4, CD8 and CD19 lymphocyte by flowcytometry and three of the four patients had chemotatic defect. Five patients certainly had giant granule in bone marrow neutrophil and in one patient it was equiovocal. Three patients had an accelerated phase, and for one patient bone marrow transplantation was done that was tolerated well and had been well after 7 years.We emphasize the need for early diagnosis on basis of characteristic facies and diagnostic laboratory examinations and early bone marrow transplantation (BMT) in patients.

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APA: Copy

FARHOUDI, A., CHAVOSHZADEH, Z., POURPAK, Z., IZADYAR, MINA, GHARAGOZLOU, M., MOVAHEDI, M., MIRSAEID GHAZI, B., REZAEI, N., MOEIN, M., & AGHAMOHAMMADI, A.. (2003). REPORT OF SIX CASES OF CHEDIAK–HIGASHI SYNDROME WITH REGARD TO CLINICAL AND LABORATORY FINDINGS. IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI), 2(4), 189-192. SID. https://sid.ir/paper/291050/en

Vancouver: Copy

FARHOUDI A., CHAVOSHZADEH Z., POURPAK Z., IZADYAR MINA, GHARAGOZLOU M., MOVAHEDI M., MIRSAEID GHAZI B., REZAEI N., MOEIN M., AGHAMOHAMMADI A.. REPORT OF SIX CASES OF CHEDIAK–HIGASHI SYNDROME WITH REGARD TO CLINICAL AND LABORATORY FINDINGS. IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI)[Internet]. 2003;2(4):189-192. Available from: https://sid.ir/paper/291050/en

IEEE: Copy

A. FARHOUDI, Z. CHAVOSHZADEH, Z. POURPAK, MINA IZADYAR, M. GHARAGOZLOU, M. MOVAHEDI, B. MIRSAEID GHAZI, N. REZAEI, M. MOEIN, and A. AGHAMOHAMMADI, “REPORT OF SIX CASES OF CHEDIAK–HIGASHI SYNDROME WITH REGARD TO CLINICAL AND LABORATORY FINDINGS,” IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI), vol. 2, no. 4, pp. 189–192, 2003, [Online]. Available: https://sid.ir/paper/291050/en

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