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Adhesive small-bowel obstruction as a challenging complication of familial Mediterranean fever: A case-based review
Batuhan Küçükali1, Deniz Gezgin Yıldırım1, Pelin Esmeray Şenol1
1Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.
Abstract:
Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self-limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis. Physicians are generally aware of other complications associated with FMF but reports on peritoneal adhesions and intestinal obstruction in English-language literature are inadequate to increase clinicians' awareness. Therefore, it is crucial to meticulously evaluate FMF patients presenting with abdominal pain and ileus because these symptoms could be due to adhesive small-bowel obstruction (ASBO). Furthermore, patients presenting with ASBO without a history of abdominal surgery should also be thoroughly evaluated, especially as it could be an initial presentation for an autoinflammatory disease. Herein, we present a pediatric case of FMF with the M694V homozygous mutation, complicated by ASBO while under colchicine treatment. Additionally, we provide a comprehensive review of the available literature on ASBO in FMF.
Insights
Familial Mediterranean fever (FMF), an autoinflammatory disorder, can cause bowel obstruction due to peritoneal adhesions. Early evaluation of abdominal pain in FMF patients is crucial to prevent severe complications like intestinal necrosis.
Area of Science:
- Genetics and Immunology
- Gastroenterology
Background:
- Familial Mediterranean fever (FMF) is a common inherited autoinflammatory disorder causing recurrent fever and serosal inflammation.
- Peritoneal adhesions, a rare complication of FMF, can lead to mechanical bowel obstruction, mimicking FMF attacks and causing misdiagnosis.
Approach:
- Presents a pediatric case of FMF with M694V homozygous mutation experiencing adhesive small-bowel obstruction (ASBO) despite colchicine treatment.
- Reviews English-language literature on ASBO in FMF patients to enhance clinical awareness.
Key Points:
- Abdominal pain and ileus in FMF patients warrant thorough evaluation for ASBO.
- ASBO in patients without prior abdominal surgery may indicate an underlying autoinflammatory disease like FMF.
- Accurate diagnosis and management of ASBO in FMF are critical to prevent morbidity, including strangulation and intestinal necrosis.
Conclusions:
- Physicians must meticulously evaluate FMF patients with abdominal symptoms for ASBO.
- Increased awareness of ASBO as a complication of FMF is needed in the medical community.
- This case highlights the importance of considering ASBO in FMF and vice versa.
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