Mesh : Child, Preschool Male Child Young Adult Humans Adult Chitosan Phytosterols / adverse effects Xanthomatosis Coronary Artery Bypass / adverse effects Atherosclerosis Collagen Hypercholesterolemia Intestinal Diseases Lipid Metabolism, Inborn Errors

来  源:   DOI:10.1097/WON.0000000000001069

Abstract:
BACKGROUND: Sitosterolemia, also known as phytosterolemia, is a rare recessive genetic disorder characterized by accumulation of sitosterol from vegetable oils, nuts, and other plant-based foods in the body. In those with sitosterolemia, there is an increase of fatty deposits in the arteries (atherosclerosis), which may occur in early childhood, impeding blood flow and increasing the risk of a heart attack, stroke, or sudden death at a very early age. Visual signs of sitosterolemia may include small yellowish xanthomas beginning in early childhood. Xanthomas are accumulated lipids that develop in the heels, elbows, and buttocks.
METHODS: A clinical case study of a patient with sitosterolemia with slow healing surgical wound from coronary artery bypass surgery is described. Treatment of sitosterolemia is aimed at lowering plasma plant sterol levels with dietary restriction intake of both animal- and plant-based sterols. However, plant-based products (collagen, chitosan, etc) are also used for wound dressings, so alternative wound dressings were selected to decrease the possibility of systemic absorption.
CONCLUSIONS: This case study describes a young adult male with sitosterolemia who presented with a slow healing surgical incision following coronary artery bypass surgery. Sitosterolemia is often characterized by atherosclerosis of the coronary arteries that occurs in children and early adulthood, especially affecting men. Treatment is aimed at lowering plasma sterol levels with the restriction of animal and plant sterols. There is considerable interest today in natural versus synthetic wound care products. Dressings containing chitosan, cellulose, collagen, etc, to be avoided to decrease the chance of systemic absorption.
摘要:
背景:谷甾醇血症,也被称为植物甾醇血症,是一种罕见的隐性遗传疾病,其特征是植物油中谷甾醇的积累,坚果,和其他植物性食物在体内。在那些患有谷甾醇血症的人中,动脉中脂肪沉积增加(动脉粥样硬化),这可能发生在儿童早期,阻碍血液流动并增加心脏病发作的风险,中风,或者在很小的时候突然死亡。谷甾醇血症的视觉体征可能包括在儿童早期开始的黄色小黄瘤。黄色瘤是在脚跟中积累的脂质,肘部,还有臀部.
方法:描述了一例因冠状动脉搭桥手术导致的手术伤口愈合缓慢的谷甾醇血症患者的临床病例研究。谷甾醇血症的治疗旨在通过饮食限制摄入基于动物和植物的甾醇来降低血浆植物甾醇水平。然而,植物性产品(胶原蛋白,壳聚糖,等)也用于伤口敷料,因此选择替代伤口敷料以降低全身吸收的可能性。
结论:本病例研究描述了一名患有谷甾醇血症的年轻成年男性,在冠状动脉搭桥手术后出现手术切口缓慢愈合。谷甾醇血症通常以儿童和成年早期发生的冠状动脉粥样硬化为特征。尤其是影响男人。治疗的目的是在限制动物和植物甾醇的情况下降低血浆甾醇水平。目前对天然与合成的伤口护理产品有相当大的兴趣。含壳聚糖的敷料,纤维素,胶原蛋白,etc,要避免减少全身吸收的机会。
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