Impact of genotype on endocrinal complications of Children with Alpha-thalassemia in China

Luo, Hao; Luo, Qiong; Huang, Fu-Gao; Wang, Chunfang; Wei, Ye‐Sheng

doi:10.1038/s41598-017-03029-9

Cited by 4 publications

(4 citation statements)

References 25 publications

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“…A study involving 57 transfusion-dependent β-TM patients from Sun Yat-sen Memorial Hospital showed that 7% with β-TM was diagnosed with DM, and 24% had impaired fasting glucose 15. A study of 200 children with alpha-thalassemia discovered that DM was identified in 14 patients and 71.4% of them were 6 years old with no significant difference identified between males and females 16. In our previous study of adolescent, TM patients we found that the overall prevalence of diabetes and IFG in thalassaemic children under 18 years was 2% and 30%, respectively 8…”

Section: Discussionmentioning

confidence: 99%

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

Luo¹,

Bajoria²,

Lai³

et al. 2019

DMSO

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Purpose: To determine the prevalence and underlying pathology of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia (NTDT). Patients and methods: In this study, we enrolled 211 patients aged 4–63 years with NTDT, including 79 β thalassemia intermedia patients, 114 Hb H disease patients and 18 Hb E/β thalassemia patients. All had oral glucose tolerance test, serum ferritin (SF), homeostasis model assessment (HOMA) and liver iron concentration (LIC) measurement. One hundred and twenty healthy age-matched controls were also used for the comparative purpose. Iron load was assessed by using SF and hepatic load by LIC using validated MRI techniques. Results: The 211 patients were divided into three groups according to their fasting and 2 hrs postprandial blood glucose levels: hypoglycemic, normal glucose tolerance (NGT) and hyperglycemic groups. In this study, 149 patients had NGT, 33 had hypoglycemia, 4 had diabetes and 25 had impaired glucose tolerance (IGT). None had impaired fasting glucose. There was a significant correlation between 2 hrs postprandial blood glucose levels and age, PINS120, HOMA-IR, alanine aminotransferase and LIC ( P <0.05). Risk factors for IGT in NTDT patients were older age (≥24 years) and SF concentration of ≥2,500 ng/mL. Conclusion: Age ≥24 years and SF ≥2,500 ng/mL of NTDT patients were at a greater risk for impaired glucose tolerance.

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Section: Discussionmentioning

confidence: 99%

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

Luo¹,

Bajoria²,

Lai³

et al. 2019

DMSO

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“…Growth failure in thalassemia may be attributed to economic status 24 and clinical factors such as the degree of chronic hypoxia, iron overload, several micronutrient deficiencies, and parental height. 25 , 26 In this study, the prevalence of growth failure was 15%, consistent with other studies (13%–21%), 10 , 12 with the failure more pronounced among our patients with NDHbH.…”

Section: Discussionmentioning

confidence: 99%

“…23 Health education, food fortification policies, and early detection monitoring are necessary to mitigate the risk of vitamin D deficiency and promote bone health and growth in thalassemia patients. 21 Growth failure in thalassemia may be attributed to economic status 24 and clinical factors such as the degree of chronic hypoxia, iron overload, several micronutrient deficiencies, and parental height. 25,26 In this study, the prevalence of growth failure was 15%, consistent with other studies (13%-21%), 10,12 with the failure more pronounced among our patients with NDHbH.…”

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confidence: 99%

HEMOGLOBIN H DISEASE AND GROWTH: A COMPARATIVE STUDY OF DHbH AND NDHbH PATIENTS

Hunnuan,

SAnpakit,

Lertbannaphong

et al. 2023

Mediterr J Hematol Infect Dis

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Background: Hemoglobin H disease (HbH), a hemoglobinopathy resulting from abnormal alpha globin genes, is classified into two categories: deletional HbH (DHbH) and non-deletional HbH (NDHbH). The alpha-mutation genotypes exhibit a range of clinical anemias, which differentially impact patient growth. Objectives: This retrospective study assessed the growth of HbH patients at Siriraj Hospital, Mahidol University. Methods: Patients diagnosed with HbH between January 2005 and April 2021 were analyzed using growth standard scores of the Thai Society for Pediatric Endocrinology (2022 version) and BMI-for-age Z scores of the World Health Organization. Growth failure was defined as a patient’s height for age exceeding two standard deviations below the mean. Results: Of the 145 HbH patients, 75 (51.7%) had NDHbH, with --SEA/αCSα being the most common genotype (70 patients; 93.3%). The mean baseline hemoglobin level was significantly lower in NDHbH patients than in DHbH patients (8.16 ± 0.93 g/dL vs. 9.51 ± 0.68 g/dL; P < 0.001). Splenomegaly and growth failure prevalences were higher in NDHbH patients (37.3% vs. 0%, with P < 0.001, and 22.7% vs. 8.6%, with P = 0.020, respectively). Multivariable analysis revealed that splenomegaly > 3 cm was associated with growth failure (OR = 4.28; 95% CI, 1.19–15.39; P = 0.026). Conclusions: NDHbH patients exhibited lower hemoglobin levels and more pronounced splenomegaly than DHbH patients. Growth failure can occur in both HbH types but appears more prevalent in NDHbH. Close monitoring of growth velocity is essential, and early treatment interventions may be required to prevent growth failure.

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“…Most complications are caused by increased iron sedimentation in tissues like heart, endocrine glands and these results in heart failure, arrhythmia, hypothyroidism, diabetes mellitus and so on. 2,3 A study has reported that 29.4% children with thalassemia had diabetes, 11.8% had hypothyroidism. 4 A local study by Ibrahim et al, 5 reported 13.8% hypothyroidism and 5.6% diabetes in children with thalassemia.…”

Section: Introductionmentioning

confidence: 99%

Thalassemia ENDOCRINOPATHIES IN THALASSEMIA PATIENTS

Touheed

Maryam

Murtaza

et al. 2021

PAFMJ

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Objective: To determine the common endocrine complications found in children having thalassemia major. Study Design: Cross-sectional study. Place and Duration of Study: Department of Pediatric Medicine, Combined Military Hospital Multan, from May to Nov 2019. Methodology: A total of 160 Children with thalassemia were taken in this study. Once registered, venous blood sample was taken and sent to the laboratory for endocrine profile. SPSS-21 was applied for analysis of collected data. Results: Out of 160 study cases, 100 (62.5%) were boys while 60 (37.5%) were female patients. Mean age of our study cases was 8.58 ± 1.98 years. Mean duration of disease was 5.28 ± 3.29 years. Parental consanguinity was positive in 122 (76.3%) and only 47 (29.4%) were taking chelation therapy. Mean HbA1C level was 6.23 ± 1.18% mg/dl and diabetes was noted in 41 (25.6%) of our study cases. Mean FT4 was 0.98 ± 0.13 ng/dl and hypothyroidism was noted in 24 (15%) of our study cases. Conclusion: Diabetes and hypothyroidism were the common endocrine complications noted in our study among children having thalassemia. All physicians treating such patients should always screen such patients for early diagnosis and timely management in order to reduce burden of related morbidities and enhance quality of life of these patients.

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Impact of genotype on endocrinal complications of Children with Alpha-thalassemia in China

Cited by 4 publications

References 25 publications

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

HEMOGLOBIN H DISEASE AND GROWTH: A COMPARATIVE STUDY OF DHbH AND NDHbH PATIENTS

Thalassemia ENDOCRINOPATHIES IN THALASSEMIA PATIENTS

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