Cross-sectional Survey and Influencing Factors for Pubertal Development in Patients with Beta Thalassemia Major
- VernacularTitle:重型β-地中海贫血患者青春发育状况及影响因素
- Author:
Zhuan-nan JIANG
1
,
2
;
Li-na ZHANG
1
;
Zhe MENG
1
;
Hui OU
1
;
Le-le HOU
1
;
Zu-lin LIU
1
;
Si-qi HUANG
1
;
Li-yang LIANG
1
Author Information
1. Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou 510120, China
2. Department of Child Healthcare, Maternal and Child Health Hospital of Dongguan, Dongguan 523000, China
- Publication Type:Journal Article
- Keywords:
β-thalassemia major;
abnormal puberty development;
iron overload
- From:
Journal of Sun Yat-sen University(Medical Sciences)
2020;41(6):967-974
- CountryChina
- Language:Chinese
-
Abstract:
【Objective】 The purpose of this study was to investigate puberty development in β-TM patients and to analyze its clinical characteristics and influencing factors. 【Methods】 A total of 42 β-TM patients aged ≥10 years old were evaluated for their stages of puberty development by reviewing follow-up data(using the REDCAP system, the thalassemia follow-up database), questionnaire, physical examination and laboratory tests. To investigate The correlations between multiple factors, such as age, beginning age of iron chelation, iron overload and so on, and abnormal puberty development in β-TM patients, were investigated. 【Results】 Twenty-four cases of β-TM patients were diagnosed as abnormal puberty development, including 11 girls and 13 boys. The common clinical manifestations of β-TM patients with abnormal puberty development were delayed puberty development and primary amenorrhea for girls and short penis and small testicles for boys. The prevalence rate of abnormal puberty development was significantly higher in β-TM patients who had older beginning age of iron chelation, β0β0 genotype, a history of splenectomy, vitamin D deficiency and diabetes(χ2 = 3.966, 5.196, 5.567, 4.714, P = 0.046, 0.023, 0.018, 0.030). The result of logistic regression analysis indicated that cardiac MRT2* < 20 ms was an independent risk factor for abnormal puberty development in β-TM patients. 【Conclusions】 Abnormal puberty development in β-TM patients is very common. Influencing factors include beginning age of iron chelation, β0β0 genotype, vitamin D deficiency, diabetes and cardiac iron deposition. Moreover, hypogonadotropic hypogonadism may be an important pathogenesis of abnormal puberty development in β-TM patients.
