Hepatic abnormalities in youth with Turner syndrome.
Hepatic abnormalities in youth with Turner syndrome.
复制标题
DOI：
10.1111/liv.15358
复制
发表时间：
2022-10
影响因子：
6.7
通讯作者：
Davis, Shanlee M.
中科院分区：
文献类型：
--
作者：
Singh, Isani;Noel, Gillian;Barker, Jennifer M.;Chatfield, Kathryn C.;Furniss, Anna;Khanna, Amber D.;Nokoff, Natalie J.;Patel, Sonali;Pyle, Laura;Nahata, Leena;Cole, Francis S.;Ikomi, Chijioke;Bamba, Vaneeta;Fechner, Patricia Y.;Davis, Shanlee M.
特纳综合征（TS）儿童的肝脏疾病与相关的生长、心脏和生殖并发症的关系尚不清楚。这项研究旨在更好地描述一个大型国家青年TS队列中的肝脏异常。使用PEDSnet机构的电子健康记录数据，将2，145名TS女性与8，580名无TS女性在8个人口统计学变量上进行匹配。结果包括肝酶（AST和ALT）分层为正常，高于正常上限（ULN）1-2倍，2-3倍ULN和>3倍ULN，以及特定的肝病诊断。58%患有TS的年轻人肝酶升高。TS患者的酶1-2倍ULN（OR：1.7，95% CI：1.4-1.9）、2-3倍ULN（OR：2.7，95% CI：1.7-3.3）和>3倍ULN（OR：1.7，95% CI：1.3-2.2）的几率较高。他们也有更高的几率任何肝脏诊断（OR：2.4，95% CI：1.7-3.3），脂肪肝（OR：1.9，95% CI：1.1-3.2），肝炎（OR：3.7，95%CI：1.9-7.1）、肝硬化/纤维化（OR：5.8，95%CI：1.3-25.0）和肝肿瘤/恶性肿瘤（OR：4.8，95%CI：1.4-17.0）。在多项模型中，年龄、BMI和心血管疾病或糖尿病的存在显著增加了TS女孩肝酶升高的几率。与匹配的对照组相比，患有TS的年轻人有更高的肝酶升高和临床显著肝病的几率。这些结果强调了临床筛查和额外研究TS肝病病因和治疗的必要性。特纳综合征是一种染色体疾病，其中女性缺失第二性染色体，通常与身材矮小，不孕症和心脏并发症有关。肝脏异常在文献中描述较少。在这项研究中，近60%的TS青少年肝酶升高。此外，TS患者比无TS的患者更常诊断为肝病。我们的研究结果支持早期和一致的肝功能筛查的重要性，以及额外的研究，以确定破坏儿童TS女性肝功能的机制。
Liver disease in children with Turner Syndrome (TS) is poorly understood relative to associated growth, cardiac, and reproductive complications. This study sought to better characterize hepatic abnormalities in a large national cohort of youth with TS. Using electronic health record data from PEDSnet institutions, 2,145 females with TS were matched to 8,580 females without TS on 8 demographic variables. Outcomes included liver enzymes (AST and ALT) stratified as normal, 1–2 times above the upper limit of normal (ULN), 2–3 times ULN, and >3 times ULN, as well as specific liver disease diagnoses. Fifty-eight percent of youth with TS had elevated liver enzymes. Patients with TS had higher odds of enzymes 1–2 times ULN (OR: 1.7, 95% CI: 1.4–1.9), 2–3 times ULN (OR: 2.7, 95% CI: 1.7–3.3), and >3 times ULN (OR: 1.7, 95% CI: 1.3–2.2). They also had higher odds of any liver diagnosis (OR: 2.4, 95% CI: 1.7–3.3), fatty liver disease (OR: 1.9, 95% CI: 1.1–3.2), hepatitis (OR: 3.7, 95% CI: 1.9–7.1), cirrhosis/fibrosis (OR: 5.8, 95% CI: 1.3–25.0), and liver tumor/malignancy (OR: 4.8, 95% CI: 1.4–17.0). In a multinomial model, age, BMI, and presence of cardiovascular disease or diabetes significantly increased the odds of elevated liver enzymes in girls with TS. Youth with TS have higher odds for elevated liver enzymes and clinically significant liver disease compared with matched controls. These results emphasize the need for clinical screening and additional research into the etiology and treatment of liver disease in TS. Turner Syndrome, a chromosomal condition in which females are missing the second sex chromosome, is often associated with short stature, infertility, and cardiac complications. Liver abnormalities are less well described in the literature. In this study, nearly 60% of youth with TS have elevated liver enzymes. Furthermore, patients with TS had a diagnosis of liver disease more often than patients without TS. Our results support the importance of early and consistent liver function screening and of additional research to define mechanisms that disrupt liver function in pediatric TS females.
登录
查看更多内容
影响因子：
3.7
作者：
Dong MH;Bettencourt R;Barrett-Connor E;Loomba R
通讯作者：
Loomba R
影响因子：
12.8
作者：
Bakalov VK;Gutin L;Cheng CM;Zhou J;Sheth P;Shah K;Arepalli S;Vanderhoof V;Nelson LM;Bondy CA
通讯作者：
Bondy CA
影响因子：
12.6
作者：
Abdo, Ayman;Meddings, Jonathan;Swain, Mark
通讯作者：
Swain, Mark
影响因子：
13.5
作者：
Roulot, D;Degott, C;Valla, D
通讯作者：
Valla, D
影响因子：
3.2
作者：
Koulouri, Olympia;Ostberg, Julia;Conway, Gerard S.
通讯作者：
Conway, Gerard S.