Choudhry Muhammad Saad, Zaidi Syed Muhammad Hussain, Mohiuddin Osama, Khan Anosh Aslam, Hanif Amber
General Surgery, Civil Hospital Karachi, Dow University of Health Sciences, Karachi, PAK.
Internal Medicine, Civil Hospital Karachi, Dow University of Health Sciences, Karachi, PAK.
Cureus. 2020 Oct 21;12(10):e11077. doi: 10.7759/cureus.11077.
Budd-Chiari associated with celiac disease is a rare phenomenon in the medical literature with annual incidence of less than five per million. The majority of the cases are reported from the North African region. Our patient presented in the out-patient department with symptoms of progressive abdominal distension, diffuse abdominal pain and shortness of breath for one year. She was a known case of celiac disease for the last three years. The clinical examination revealed ascites, jaundice, decreased air entry in basal segments bilaterally, and multiple hemangiomas all over the body. Haematological and biochemical investigations, including levels of pro-thrombotic factors and homocysteine level, turned out to be normal. However, computed tomography (CT) revealed hepatic vein obstruction. Hence, a diagnosis of Budd-Chiari syndrome was confirmed. The patient was managed with anticoagulants, diuretics and gluten-free diet. Within a month, the patient showed marked improvement with a significant reduction in ascites. To the best of our knowledge, this rare association is the first case to be reported from Pakistan and third from the region of South Asia.
布加综合征合并乳糜泻在医学文献中是一种罕见现象，年发病率低于百万分之五。大多数病例来自北非地区。我们的患者因进行性腹胀、弥漫性腹痛和气短症状在门诊就诊，症状持续一年。她在过去三年中一直是已知的乳糜泻患者。临床检查发现有腹水、黄疸、双侧肺底部呼吸音减弱以及全身多处血管瘤。血液学和生化检查，包括促血栓形成因子水平和同型半胱氨酸水平，结果均正常。然而，计算机断层扫描（CT）显示肝静脉阻塞。因此，布加综合征的诊断得以证实。患者接受了抗凝剂、利尿剂和无麸质饮食治疗。一个月内，患者病情显著改善，腹水明显减少。据我们所知，这种罕见的关联是巴基斯坦报告的首例，也是南亚地区报告的第三例。
