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Compounded with hemoglobin Port Phillip and -α4.2 or --SEA deletions were identified in Chinese population.
Du, Li; Bao, Xiuqin; Qin, Danqing; Wang, Jicheng; Yao, Cuize; Liang, Jie; Chen, Jianhong; Yin, Aihua.
Afiliación
  • Du L; Medical Genetic Center, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Bao X; Maternal and Children Metabolic-Genetic Key Laboratory, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Qin D; Thalassemia Diagnosis Center, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Wang J; Guangdong Birth Defect Prevention and Management Center, Guangzhou, Guangdong, China.
  • Yao C; Medical Genetic Center, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Liang J; Maternal and Children Metabolic-Genetic Key Laboratory, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Chen J; Thalassemia Diagnosis Center, Guangdong Women and Children Hospital, Guangzhou, Guangdong, China.
  • Yin A; Guangdong Birth Defect Prevention and Management Center, Guangzhou, Guangdong, China.
Mol Genet Genomic Med ; 9(9): e1699, 2021 09.
Article en En | MEDLINE | ID: mdl-34398528
INTRODUCTION: Although over 1000 hemoglobin (Hb) variants were identified so far, Hb Port Phillip compound with α-thalassemia deletion had no reported before. METHODS: Two patients and the associated families from Guangdong province in China were recruited. Hematological parameters were determined by blood routine examination and hemoglobin electrophoresis. Genotyping was performed by Gap-PCR and Sanger sequencing. RESULTS: One patient was diagnosed as Hb Port Phillip, while her daughter was compounded with -α4.2 deletion, with normal Hb level (150 g/L), mean corpuscular volume (MCV) 108.4 fl and mean corpuscular hemoglobin (MCH) (30.5 pg). Another patient was diagnosed as compound Hb Port Phillip and --SEA deletion. This proband presented with more severe α-thalassemia trait than the patient compounded with -α4.2 deletion, with hemoglobin 80 g/L, MCV 61.7 fl, and MCH 18.7 pg. CONCLUSION: Here we first time identified two patients compound with Hb Port Phillip and -α4.2 and --SEA deletions, respectively, which had never been reported. Our study widens the genotypes of hemoglobinopathy and provides reference for genetic counselling and prenatal diagnosis in this population.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fragmentos de Péptidos / Talasemia / Hemoglobinas Tipo de estudio: Prognostic_studies Límite: Adult / Female / Humans / Male Idioma: En Revista: Mol Genet Genomic Med Año: 2021 Tipo del documento: Article País de afiliación: China Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fragmentos de Péptidos / Talasemia / Hemoglobinas Tipo de estudio: Prognostic_studies Límite: Adult / Female / Humans / Male Idioma: En Revista: Mol Genet Genomic Med Año: 2021 Tipo del documento: Article País de afiliación: China Pais de publicación: Estados Unidos