在沙特1例1型糖尿病和甲状腺功能减退的年轻女孩中发现了罕见的Mauriac综合征和Van-Wyk Grumbach综合征的关联:1例报告和简短的文献回顾

Aida Al Jabri, Aeshah Al Johar, M. Yacoubi
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摘要

1型糖尿病(T1DM)是一种以胰腺β细胞胰岛素分泌不足为特征的自身免疫性疾病。T1DM患者患其他自身免疫性疾病的风险更高,如乳糜泻和甲状腺疾病。甲状腺功能减退症是指甲状腺不能分泌足量的甲状腺素,而甲状腺素是身体生长、大脑发育和细胞代谢所必需的。大多数研究报道,T1DM儿童甲状腺功能减退的发生率高于正常儿童,9.6%的儿童甲状腺功能减退,19%的儿童抗tpo抗体阳性。甲状腺功能减退会加重T1DM患儿的病情,反之亦然。长期不受控制的糖尿病可能由于下丘脑-垂体-甲状腺轴的完全抑制而增加胰岛素抵抗。控制不良的T1DM的一个罕见并发症是Mauriac综合征,其特征是肝酶升高、高脂血症、库欣样特征、生长迟缓和糖原性肝病引起的肝肿大。Van Wyk-Grumbach综合征也是长期未经治疗的甲状腺功能减退症的罕见并发症,表现为乳房发育、多囊卵巢、阴毛和腋毛生长不足相关的子宫出血以及骨龄延迟。在这里,我们报告了一个患有甲状腺功能减退和T1DM控制不良的儿童的两种罕见并发症Mauriac综合征和Van Wyk-Grumbach综合征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare association of Mauriac syndrome and Van-Wyk Grumbach syndrome found in a young Saudi girl with poorly controlled type 1 Diabetes and Hypothyroidism: A Case Report and Brief literature review
Type 1 diabetes mellitus (T1DM) is an autoimmune disease characterized by insufficient insulin production of the pancreatic beta-cells. Patients with T1DM will have a higher risk of other autoimmune disorders like celiac and thyroid diseases. Hypothyroidism is the failure of the thyroid gland to secrete an adequate amount of thyroxine, which is required for physical growth, brain development, and cellular metabolism. Most studies reported that children with T1DM have a higher incidence of hypothyroidism than normal children, with 9.6% having hypothyroidism and 19% having positive anti-TPO antibodies. Hypothyroidism will aggravate the condition in a child with T1DM and vice versa. Uncontrolled diabetes for a long time might increase insulin resistance due to complete depression of the hypothalamus-pituitary thyroid axis. A rare complication of poorly controlled T1DM is Mauriac syndrome, characterized by elevated liver enzymes, hyperlipidemia, cushingoid features, growth retardation, and hepatomegaly due to glycogenic hepatopathy. Van Wyk–Grumbach syndrome is also a rare complication of long-standing, untreated hypothyroidism, manifested by breast development, multicystic ovary, uterine bleeding associated with lack of pubic and axillary hair growth, and delayed bone age. Here, we report a case with two rare complications of Mauriac syndrome and Van Wyk–Grumbach syndrome in a child with hypothyroidism and poorly controlled T1DM.
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