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重型β地中海贫血青少年的胰腺功能可预测心脏和肝脏铁负荷:与T2*(T2星)磁共振成像的关系

Pancreatic functions in adolescents with beta thalassemia major could predict cardiac and hepatic iron loading: relation to T2-star (T2*) magnetic resonance imaging.

作者信息

Mokhtar Galila M, Ibrahim Wafaa E, Elbarbary Nancy S, Matter Randa M, Ibrahim Ahmed S, Sayed Safa M

机构信息

Hematology and Oncology Unit, Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Department of Clinical Radiology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

出版信息

J Investig Med. 2016 Mar;64(3):771-81. doi: 10.1136/jim-2015-000031. Epub 2016 Feb 12.

Abstract

The aim of this study is to assess the correlation between cardiac and hepatic T2* MRI findings with the endocrine and exocrine pancreatic functions in known patients with β-thalassaemia major (β-TM). A total of 50 adolescent patients with β-TM and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test and T2* MRI, to assess iron content in the heart and liver. Diabetes was found in 20%, and 40% of patients had impaired fasting glucose (IFG). Cardiac T2* was less than 10 ms in 22% indicating heavy load with iron in cardiac tissues. There was a significant decrease in median serum amylase (63.5 vs 87.5 IU/L, p=0.003) and lipase (63 vs 90 IU/L, p=0.017) among patients in comparison with the control group. Patients with β-TM and diabetes had lower serum amylase (32 vs 68 IU/L), lipase (28 vs 79 IU/L), cardiac and hepatic T2* MRI (7 vs 25.5 ms; 3 vs 6 ms, p<0.001 for all) than those without diabetes. Similar results were found among patients with IFG when compared with others (p<0.001 for all). Cardiac and hepatic T2* were inversely correlated to triglyceride index (r=-0.376, p=0.014 and r=-0.475, p=0.001, respectively) and positively correlated to amylase (r=0.791 and r=0.790) and lipase (r=0.784 and r=0.783; p<0.001 for all). The endocrine and exocrine pancreatic functions might become an equivalent predictor to cardiac and hepatic iron overload, especially in countries where MRI is not available or where it is expensive. The early occurrence of these abnormalities warrants more intensive chelation therapy.

摘要

本研究旨在评估已知重型β地中海贫血(β-TM)患者心脏和肝脏T2磁共振成像(MRI)结果与胰腺内分泌和外分泌功能之间的相关性。通过血清淀粉酶、脂肪酶、甘油三酯指数、口服葡萄糖耐量试验和T2 MRI对50例青少年β-TM患者和44例健康对照者进行了调查,以评估心脏和肝脏中的铁含量。20%的患者患有糖尿病,40%的患者空腹血糖受损(IFG)。22%的患者心脏T2小于10毫秒,表明心脏组织中铁负荷过重。与对照组相比,患者的血清淀粉酶中位数(63.5对87.5 IU/L,p = 0.003)和脂肪酶中位数(63对90 IU/L,p = 0.017)显著降低。患有β-TM和糖尿病的患者血清淀粉酶(32对68 IU/L)、脂肪酶(28对79 IU/L)、心脏和肝脏T2 MRI(7对25.5毫秒;3对6毫秒,所有p<0.001)均低于无糖尿病患者。与其他患者相比,IFG患者也有类似结果(所有p<0.001)。心脏和肝脏T2*与甘油三酯指数呈负相关(r = -0.376,p = 0.014和r = -0.475,p = 0.001),与淀粉酶呈正相关(r = 0.791和r = 0.790),与脂肪酶呈正相关(r = 0.784和r = 0.783;所有p<0.001)。胰腺内分泌和外分泌功能可能成为心脏和肝脏铁过载的等效预测指标,尤其是在无法进行MRI或MRI费用昂贵的国家。这些异常的早期出现需要更强化螯合疗法。

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