Relationship Between Pituitary Siderosis and Endocrinological Disorders in Pediatric Patients with Beta-Thalassemia

Yılmaz, Kamil; Kan, Ahmet; Çetinçakmak, Mehmet Güli; Üzel, Veysiye Hülya; Yılmaz, Deniz; Deniz, Muhammed Akif; Hattapoğlu, Salih

doi:10.7759/cureus.12877

Cited by 3 publications

(4 citation statements)

References 27 publications

(38 reference statements)

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“…GHD aetiology might be due to chronic iron overload affecting hypothalamic and pituitary structures, even if literature data are still not conclusive [27]. Compared to nGHD patients, the GHD group presented significantly lower basal and peak GH levels, confirming GH secretion impairment.…”

Section: Discussionmentioning

confidence: 86%

GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study

Gagliardi

Mungari

Gamberini³

et al. 2022

J Endocrinol Invest

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Purpose GH deficit (GHD) could represent an endocrine issue in ß-Thalassemia Major (ßTM) patients. GH/IGF-1 axis has not been extensively explored in ßTM adults, so far. We aim to assess GHD and IGF-1 deficiency prevalence in ßTM adult population, focusing on the relationship with liver disease. Methods Cross-sectional multi-centre study conducted on 81 adult ßTM patients (44 males, mean age 41 ± 8 years) on transfusion and chelation therapy. GHD was investigated by GHRH + arginine test. IGF-1 levels, routine biochemical exams, Fibroscan, Hepatic Magnetic Resonance Imaging (MRI) and pituitary MRI were collected. Results Eighteen patients were affected by GHD and 63 were not (nGHD) according to GHRH + arginine test, while basal GH levels did not differ. GHD was associated with a higher BMI and a worse lipid profile (p < 0.05). No significant differences were observed regarding liver function between the two groups. Pituitary MRI scan was normal except for one case of empty sella. The 94.4% and 93.6% of GHD and nGHD, respectively, presented lower IGF-1 levels than the reference range, and mean IGF-1 SDS was significantly lower in GHD patients. Conclusion GHD is frequent in adult ßTM patients and is associated with higher BMI and worse lipid profile. nGHD patients present lower IGF-1 levels as well. There was no relationship between IGF-1 levels and liver disease. Further, multicentric studies with larger cohorts and standardized diagnostic protocols are needed.

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Section: Discussionmentioning

confidence: 86%

GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study

Gagliardi

Mungari

Gamberini³

et al. 2022

J Endocrinol Invest

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“…[27] The widespread hepatic iron overload and the associated decline in quality of life emphasize the critical need to address socioeconomic obstacles to enhance patient outcomes. [28] This exploration of MRI T2* values and their impact on the quality of life in pediatric patients with severe β-TM yields vital insights into the condition's comprehensive effects. The findings highlight the necessity for holistic therapeutic strategies to navigate the intricate challenges faced by these patients, offering valuable perspectives on the broader implications of β-TM on patient health and well-being.…”

Section: Discussionmentioning

confidence: 99%

“…[27] The widespread hepatic iron overload and the associated decline in quality of life emphasize the critical need to address socioeconomic obstacles to enhance patient outcomes. [28]…”

Section: Discussionmentioning

confidence: 99%

Cross-sectional study on the impact of cardiac and hepatic iron overload, as measured by MRI T2*, on the quality of life in children with severe beta-thalassemia major

Lan,

Ye,

et al. 2024

Medicine

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A cross-sectional study to explore the correlation between cardiac and hepatic iron overload and its impact on the quality of life in children diagnosed with severe beta-thalassemia major (β-TM). A cohort of 55 pediatric patients with β-TM, diagnosed via genetic testing at the Affiliated Hospital of Guangdong Medical University from January 2015 to January 2022, was included in this study. The assessment of cardiac and hepatic iron overload was conducted using the magnetic resonance imaging T2* technique. The Chinese version of the Pediatric Quality of Life Inventory (PedsQL) 4.0. Pearson correlation analysis was utilized to assess the relationships between the cardiac and hepatic T2* values and between these T2* values and the total scores of PedsQL 4.0. Analysis showed no significant correlation between cardiac and hepatic T2* values. However, a significant relationship was observed between cardiac T2* values and PedsQL 4.0 total scores (r = 0.313, P < .05), indicating that cardiac, but not hepatic, iron overload is associated with the quality of life. This study highlights the absence of correlation between cardiac and hepatic iron overload levels and demonstrates a significant impact of cardiac iron overload on the quality of life in children with β-TM. These findings suggest the need for a focused approach to cardiac health in managing β-TM.

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“…This study shown that thalassemia trait prevalence was 1.92%, which is less than a study conducted in Turkey which found to be 2.6 % by Keskin et al in 2000 (19) . Among the genetic diseases, the most common disorders are hemoglobinopathies (20) . Β-thalassemia is prevalent in Mediterranean countries (21) .…”

Section: Discussionmentioning

confidence: 99%

Premarital Screening Program in Al-Nuaman Teaching Hospital

Hanoon¹,

Khalf²,

Khalaf³

2021

IraqiJMS

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Background: Premarital blood screening helps couples to identify potential health problems and risks for themselves and their offspring, considered a primary preventive approach for couples planning for conception and an important step towards protecting society from spread of diseases especially thalassemia, and allowing people to enjoy life. Objective: To determine the prevalence of minor β-thalassemia, human immunodeficiency virus (HIV), hepatitis B virus (HBV), hepatitis C virus (HCV) and venereal disease research laboratory (VDRL) referred to Al-Nuaman Teaching Hospital. Methods: The cross-sectional study as part of premarital screening in Al-Nuaman Teaching Hospital was conducted over six months from May 2017 to the end of October 2017. Total individual of 3027 underwent routine mandatory tests. Results: Regarding HIV and VDRL cases were zero, regarding HBV was no case in August, only one case in May and June, two cases in September and October, and four cases in July, regarding HCV there was no cases in May, June and Aug., two cases in September, and October, and three cases in July, Regarding β-thalassemia minor, one couple in August and another couple in September, those two couples both male and female were thalassemia minor , while the rest either the male or female were thalassemia minor. Conclusion: Premarital screening program is an important project in detecting asymptomatic carriers of hepatitis viral infection and thalassemia. This study showed that incidence of hepatitis B infections 0.33% were more frequent than Hepatitis C infections 0.23%, there was no cases of HIV infection detected so far, and β-thalassemia trait prevalence was 1.92%. Keywords: Premarital, β-thalassemia, virology, screening Citation: Hanoon BD, Sudad A. Khalf SA, Khalaf IA. Premarital screening program in Al-Nuaman Teaching Hospital. Iraqi JMS. 2021; 19(2): 147-151. doi: 10.22578/IJMS.19.2.3

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Relationship Between Pituitary Siderosis and Endocrinological Disorders in Pediatric Patients with Beta-Thalassemia

Cited by 3 publications

References 27 publications

GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study

GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study

Cross-sectional study on the impact of cardiac and hepatic iron overload, as measured by MRI T2*, on the quality of life in children with severe beta-thalassemia major

Premarital Screening Program in Al-Nuaman Teaching Hospital

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