Kleint Austin, Dussan Monica, Chandran Arjun, Salameh Mohammed
Pediatrics, Baylor College of Medicine, San Antonio, USA.
Pediatric Endocrinology, Baylor College of Medicine, San Antonio, USA.
Cureus. 2024 Jul 17;16(7):e64748. doi: 10.7759/cureus.64748. eCollection 2024 Jul.
Mauriac syndrome is a rare complication of longstanding, poorly controlled type 1 diabetes in pediatric patients. Mauriac syndrome is characterized by hepatomegaly and growth retardation. This case report discusses a 14-year-old girl with persistent, poorly controlled type 1 diabetes mellitus (T1DM) admitted to the pediatric intensive care unit (PICU), where she was ultimately diagnosed with Mauriac syndrome. The patient presented with severe hypoglycemia and a history of multiple admissions for diabetes ketoacidosis (DKA) despite insulin therapy. The patient had a history of poor glycemic control and growth retardation, and on physical exam, she was found to have hepatomegaly. Based on clinical presentation, history of poorly controlled diabetes hepatomegaly, imaging, and laboratory findings, the diagnosis of Mauriac syndrome was made. Management of the patient included diabetes education, optimizing insulin therapy, nutritional support, and closely monitoring labs in a multi-disciplinary approach. The patient responded well to insulin therapy and was started on closed-loop insulin administration. Liver enzymes and hepatomegaly normalized, and growth parameters improved over the subsequent months. This case emphasizes the importance of early recognition, monitoring, and management of an extremely rare syndrome that is crucial in preventing the short-term complications of lactic acidosis and rapidly progressing retinopathy and the long-term complications of hepatic dysfunction and growth impairment.
莫里亚克综合征是小儿患者长期未得到良好控制的1型糖尿病的一种罕见并发症。莫里亚克综合征的特征是肝肿大和生长发育迟缓。本病例报告讨论了一名14岁患有持续性、控制不佳的1型糖尿病（T1DM）的女孩，她被收入儿科重症监护病房（PICU），最终在那里被诊断为莫里亚克综合征。尽管接受了胰岛素治疗，该患者仍出现严重低血糖，并有多次因糖尿病酮症酸中毒（DKA）入院的病史。该患者有血糖控制不佳和生长发育迟缓的病史，体格检查发现她有肝肿大。根据临床表现、糖尿病控制不佳病史、肝肿大、影像学和实验室检查结果，做出了莫里亚克综合征的诊断。对该患者的治疗包括糖尿病教育、优化胰岛素治疗、营养支持，并采用多学科方法密切监测实验室检查结果。该患者对胰岛素治疗反应良好，并开始接受闭环胰岛素给药。肝酶和肝肿大恢复正常，生长参数在随后几个月有所改善。本病例强调了早期识别、监测和管理这种极其罕见综合征的重要性，这对于预防乳酸酸中毒和快速进展性视网膜病变的短期并发症以及肝功能障碍和生长发育受损的长期并发症至关重要。
