Ralph P M, Troutman K C
Bronx Lebanon Hospital, New York, USA.
Pediatr Dent. 1996 Nov-Dec;18(7):461-4.
Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport, and concomitant problems of hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, and peripheral edema. Patients develop diarrhea, steatorrhea, and hypocalcemia secondary to fat-soluble vitamin malabsorption. Treatment is a restrictive diet of low fat, medium chain triglycerides. Oral manifestations are gingivitis due to poor PMN function and enamel defects due to poor calcium absorption. A case of a 14-year-old boy with both gingival and enamel problems secondary to intestinal lymphangiectasia is reported.
肠淋巴管扩张症是一种罕见的常染色体显性遗传病或后天性疾病，可导致淋巴阻塞、乳糜运输不良，以及随之而来的低蛋白血症、淋巴细胞减少、低丙种球蛋白血症和外周水肿。患者会因脂溶性维生素吸收不良而出现腹泻、脂肪泻和低钙血症。治疗方法是采用低脂、中链甘油三酯的限制饮食。口腔表现为由于中性粒细胞功能不良导致的牙龈炎，以及由于钙吸收不良导致的牙釉质缺陷。本文报道了一例14岁男孩因肠淋巴管扩张症继发牙龈和牙釉质问题的病例。
