Mariam M Ismail, Olivia A Al-Hassan, Ghassan Mohamadsalih, Mohamed A Abdullah
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引用次数: 0
Abstract
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.
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.