Department of Surgery, Hinchingbrooke Hospital, Huntingdon, PE29 6NT, UK.
Department of Surgery, Hinchingbrooke Hospital, Huntingdon, PE29 6NT, UK.
J Clin Lipidol. 2022 Mar-Apr;16(2):164-166. doi: 10.1016/j.jacl.2021.12.002. Epub 2021 Dec 22.
Mesenteric panniculitis is a rare disease caused by idiopathic inflammation of adipose tissue, most commonly affecting the mesentery of the small bowel. We present a unique case of mesenteric panniculitis in a patient with Tangier disease; a rare genetic disorder caused by mutations in the ABCA1 gene, leading to deficiency of high-density lipoprotein in the blood and accumulation of cholesterol esters within various tissues. The accumulation of cholesterol esters in body tissues in patients with Tangier disease may contribute to the pathogenesis of mesenteric panniculitis; although there is limited evidence to support this hypothesis due to the rarity of concurrent disease.
肠系膜脂膜炎是一种由脂肪组织的特发性炎症引起的罕见疾病，最常影响小肠的肠系膜。我们报告了一例唐吉氏病患者的肠系膜脂膜炎的独特病例；唐吉氏病是一种由 ABCA1 基因突变引起的罕见遗传性疾病，导致血液中高密度脂蛋白缺乏，胆固醇酯在各种组织中蓄积。唐吉氏病患者体内组织中胆固醇酯的蓄积可能导致肠系膜脂膜炎的发病机制；尽管由于疾病的罕见性，支持这一假说的证据有限。
