Kinh nghiệm trong chẩn đoán và điều trị bệnh lí giãn mạch bạch huyết ruột tiên phát tại Bệnh viện Nhi đồng 2

Abstract

Intestinal lymphangiectasia is a rare but most popular abnormality of the lymphatic system causing protein-losing gastroenteropathy. We present 6 specific clinical cases with a definitive diagnosis of primary lymphangiectasia by gastrointestinal endoscopy, intestinal biopsy or trial therapy. All 6 cases have clinical manifestations starting in infancy, presenting at the hospital with diarrhea and systemic edema, and hypoalbuminemia. We proceeded to approach patients step by step from persistent diarrhea with low albumin in the blood to obtain a definitive diagnosis. When diagnosis was confirmed, all patients were treated with LCTs elimination dietary adjustments, supplemented with MCTs, and sandostatin with doses ranging from 4-15 µg/kg/day. All of them experienced clinical improvement: diarrhea and edema resolved and blood albumin gradually increased and stabilized. We found that 4 patients were also allergic to cow milk protein.