Laboratory characteristics of chronic granulomatous disease patients in vietnam national Children’s Hospital
Main Article Content
Abstract
at leads to Chronic Granulomatous Disease (CGD). A cross - sectional study was performed on 13 CGD patients in Vietnam National Children’s hospital from 6th, 2020 to 10th, 2021 to evaluate the laboratory t characteristics of these patients. The result showed that the patient group's median Stimulated Index (SI) of DHR test was 1.6. Both cell count and the ratio of neutrophils are elevated in all patients. Lymphocyte and subtype of lymphocyte are also elevated. Almost all patients had microcytic hypochromic anemia with low MCV and normal red blood cell count. The antibody levels were within reference value or increased, in which almost there is an increased of IgG.
Article Details
Keywords
Chronic granulomatous disease; DHR test, Neutrophil oxidative burst function, Vietnam National Children’s Hospital.
References
2. Drink Roos, Steven M.Holland, Taco W.Kuijpes (2013). Chronic granulomatous disease. Primary Immunodeficiency Diseases: A Molecular and Genetic Approach. 3rd ed. Oxford University Press. 689.
3. Meda Spaccamela V, Valencia RG, Pastukhov O, et al (2019). High Levels of IL - 18 and IFN - γ in Chronically Inflamed Tissue in Chronic Granulomatous Disease. Front Immunol. 10:2236.
4. Feld JJ, Hussain N, Wright EC, et al (2008). Hepatic involvement and portal hypertension predict mortality in chronic granulomatous disease. Gastroenterology. 134(7): 1917 - 1926.
5. Cấn Thị Bich Ngọc, Vũ Chí Dũng (2021). Giải trình tự toàn bộ vùng gen biểu hiện phát hiện đột biến gen CYBB gây u hạt mạn tính. Tạp chí Nghiên cứu Y học, 137(2), 19 - 27.
6. Steven M. Holland. Neutropenia and Neutrophil Defects. Sci - hub 10.1128/9781555818722. chapter 78: 767 - 774.
7. Winkelstein JA, Marino MC, Johnston RB, et al (2000). Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine (Baltimore).79(3):155 - 169.
8. Wang S, Wang T, Xiang Q, et al (2019). Clinical and Molecular Features of Chronic Granulomatous Disease in Mainland China and a XL - CGD Female Infant Patient After Prenatal Diagnosis. J Clin Immunol. 39(8):762 - 775.
9. Kwon WK, Choi S, Kim HJ, et al (2020). Flow Cytometry for the Diagnosis of Primary Immunodeficiency Diseases: A Single Center Experience. Allergy Asthma Immunol Res. 12(2):292 - 305.
10. Vowells SJ, Fleisher TA, Sekhsaria S, et al (1996). Genotype - dependent
variability in flow cytometric evaluation of reduced nicotinamide adenine dinucleotide phosphate oxidase function in patients with chronic granulomatous disease. J Pediatr.128:104 - 7.
11. Wolach B, Gavrieli R, de Boer M, et al (2017). Chronic granulomatous disease: Clinical, functional, molecular, and genetic studies. The Israeli experience with 84 patients. Am J Hematol. 92(1):28 - 36.
12. Köker MY, Camcıoğlu Y, van Leeuwen K, et al (2013). Clinical, functional, and genetic characterization of chronic granulomatous disease in 89 Turkish patients. J Allergy Clin Immunol. 132(5):1156 - 1163.e5.
13. Vowells SJ, Sekhsaria S, Malech HL, et al (1995). Flow cytometric analysis of the granulocyte respiratory burst: a comparison study of fluorescent probes. J Immunol Methods. 178(1):89 - 97.
14. Wu J, Wang WF, Zhang YD, Chen TX (2017). Clinical Features and Genetic Analysis of 48 Patients with Chronic Granulomatous Disease in a Single Center Study from Shanghai, China: New Studies and a Literature Review. J Immunol Res. 8745254.
15. Noh LM, Latiff AHA, Ismail IH, et al (2021). Clinical and demographic pattern of chronic granulomatous disease (CGD) from a multicenter perspective: Malaysia’s experience over 26 years. Allergy Asthma Clin Immunol. 17(1):50.