苏丹儿童毛里亚克综合症:在资源有限的国家仍然存在的老综合症。

IF 5.6 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM
Pediatric Diabetes Pub Date : 2025-08-26 eCollection Date: 2025-01-01 DOI:10.1155/pedi/7047312
Mariam M Ismail, Olivia A Al-Hassan, Ghassan Mohamadsalih, Mohamed A Abdullah
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引用次数: 0

摘要

目的:Mauriac综合征(MS)是一种与1型糖尿病(T1DM)患者血糖控制不足相关的罕见疾病,新生儿糖尿病患者也很少报道。MS表现为生长衰竭、青春期延迟、库欣样特征和肝肿大。这种情况可能与血脂异常、视网膜病变和肾病等并发症有关。本研究的主要目的是描述苏丹儿童和青少年由于糖尿病控制不足而患多发性硬化症的严重程度、临床特征、管理和结局。研究设计和方法:这是一项以医院为基础的横断面研究。回顾了所有MS患者的医疗记录。获得的数据包括人口统计学、临床特征、调查、管理和结果。对患者进行再教育,加强管理并随访。结果:37例MS患者入组,男性占59.5%。5.4%的患者被诊断为新生儿糖尿病,而其他患者则患有T1DM。诊断为多发性硬化症的中位年龄为12岁。诊断仅基于临床表现,包括长期血糖控制不佳、身材矮小和肝肿大的病史。结果方面,失访8例(21.6%),死亡1例(2.7%),病情静止7例(18.9%),好转21例(56.8%)。改善的迹象是肝脏大小减小,生长速度增加或不增加。肾病是最常见的相关并发症;在我们的队列中,有33.3%的人出现了这种情况。有些人在很小的时候就染上了。结论:尽管我们已经做了很多努力来更好地控制T1DM儿童的血糖,但多发性硬化症在我们的环境中仍然存在。虽然肝活检是诊断的金标准,但它是侵入性的,可以根据临床特征和治疗反应保守诊断。这种情况可以通过良好的代谢控制来逆转。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Mauriac Syndrome in Sudanese Children: An Old Syndrome Still Existing in Resource-Limited Countries.

Mauriac Syndrome in Sudanese Children: An Old Syndrome Still Existing in Resource-Limited Countries.

Mauriac Syndrome in Sudanese Children: An Old Syndrome Still Existing in Resource-Limited Countries.

Objective: Mauriac syndrome (MS) is a rare condition linked to inadequate glycemic control in type 1 diabetes mellitus (T1DM) and has also rarely been reported in patients with neonatal diabetes. MS manifests as growth failure, delayed puberty, cushingoid features, and hepatomegaly. The condition can be associated with complications like dyslipidemia, retinopathy, and nephropathy. The main objective of this study was to describe the magnitude of the condition, clinical features, management, and outcome of Sudanese children and adolescents with MS due to inadequate control of diabetes in our center. Study Design and Methods: This is a cross-sectional hospital-based study. All medical records of patients with MS were reviewed. Data, including demographics, clinical features, investigations, management, and outcome, were obtained. Patients were re-educated and management intensified then followed up. Results: Thirty-seven MS patients were enrolled in this study, with a male predominance of 59.5%. Neonatal diabetes was diagnosed in 5.4% of the patients, while others had T1DM. The median age at diagnosis of MS was 12 years. The diagnosis was based solely on clinical findings, including a history of prolonged unsatisfactory glycemic control, short stature, and hepatomegaly. Regarding the outcome, eight children (21.6%) were lost to follow-up, one patient died (2.7%), seven (18.9%) had a static condition, and those who showed improvement were 21 (56.8%). Signs of improvement were a decrease in liver size with or without an increase in growth velocity. Nephropathy was the most common associated complication; it was seen in 33.3% of our cohort. Some got it at a very young age. Conclusions: Despite many efforts that have been made to achieve better glycemic control in children with T1DM, MS still exists in our setting. Though liver biopsy is the gold standard for diagnosis, being invasive, the diagnosis could be made conservatively, based on clinical features and response to treatment. The condition can be reversed with good metabolic control.

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来源期刊
Pediatric Diabetes
Pediatric Diabetes 医学-内分泌学与代谢
CiteScore
6.60
自引率
14.70%
发文量
141
审稿时长
4-8 weeks
期刊介绍: Pediatric Diabetes is a bi-monthly journal devoted to disseminating new knowledge relating to the epidemiology, etiology, pathogenesis, management, complications and prevention of diabetes in childhood and adolescence. The aim of the journal is to become the leading vehicle for international dissemination of research and practice relating to diabetes in youth. Papers are considered for publication based on the rigor of scientific approach, novelty, and importance for understanding mechanisms involved in the epidemiology and etiology of this disease, especially its molecular, biochemical and physiological aspects. Work relating to the clinical presentation, course, management and outcome of diabetes, including its physical and emotional sequelae, is considered. In vitro studies using animal or human tissues, whole animal and clinical studies in humans are also considered. The journal reviews full-length papers, preliminary communications with important new information, clinical reports, and reviews of major topics. Invited editorials, commentaries, and perspectives are a regular feature. The editors, based in the USA, Europe, and Australasia, maintain regular communications to assure rapid turnaround time of submitted manuscripts.
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