Sclerosing encapsulating peritonitis in a child with X-linked immune dysregulation syndrome, polyendocrinopathy and enteropathy (IPEX)
Abstract
The aim is to focus the attention of surgeons and other specialists on the problem of sclerosing encapsulating peritonitis and to show the effectiveness of the interdisciplinary approach to examination and treatment, as well as the role of hereditary immunity defects in the pathogenesis of this disease.
Clinical case. A 3.5-year-old boy with enteropathy, atopic dermatitis and with a total adhesive process in the abdominal cavity after diagnostic laparoscopy, which caused recurrent intestinal obstruction, was examined and treated. Based on the clinical-histological picture, a diagnosis of sclerosing encapsulating peritonitis was established. A genetic test revealed a mutation of p.S390N in the FOXP3 gene, responsible for the immune dysregulation syndrome, polyendocrinopathy and enteropathy (IPEX). Surgical treatment of intestinal obstruction was impossible. The boy was introduced into the system of home parenteral nutrition, gastrostomy for decompression was formed, methylprednisolone was prescribed as a targeted therapy at a dose of 1 mg/kg per day. Against the background of complex treatment, the child's condition stabilized during 2 years.
Conclusion. The reasons for the development of sclerosing encapsulating peritonitis are not completely clear, but it is possible that immune dysregulation is one of the main triggers. Timely diagnosis of the disease allows to avoid ineffective multiple operations on the abdominal cavity and achieve a good effect through targeted therapy.
Keywords:еncapsulating peritoneal sclerosis, primary enteropathy, primary immunodeficiency, syndrome IPEX, FOXP3, recurrent infections
Clin. Experiment. Surg. Petrovsky J. 2018; 6 (2): 80–6.
doi: 10.24411/2308-1198-2018-12011. Received: 00.00.2018. Accepted: 00.00.2018.
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