中国儿童心脏移植操作规范(2019版)

儿童心脏移植是治疗年龄18岁终末期心力衰竭患者的有效手段。1967年美国Adrian Katrowitz实施第一例儿童心脏移植,近五年全球80家单位每年开展500例左右。中国儿童心脏移植起步晚、发展慢。自1995年开展第一例儿童心脏移植以来,目前国内已登记病例超过130例。中华医学会器官移植学分会组织心脏移植专家,总结国内外相关研究最新进展,结合国际指南和临床实践,针对儿童心脏移植受者选择及常用术式的操作要点、程序和方法,以及各类复杂先天性心脏病心脏移植的特殊操作,制订《中国儿童心脏移植适操作规范(2019版)》。     

Neurofibromatosis Type 1 Diagnosed in a Child Based on Multiple Juvenile Xanthogranulomas and Juvenile Myelomonocytic Leukemia

An association between juvenile xanthogranuloma (JXG), neurofibromatosis type 1 (NF1), and juvenile myelomonocytic leukemia (JMML) has been described in the literature but has only been documented in approximately 20 cases. We diagnosed a patient with NF1 at 25 months of age, before any cutaneous stigmata of this disease had appeared, because we decided to screen for the NF1 gene mutation because of his presentation with multiple JXGs and moderate macrocephaly (2.5 standard deviations) at 9 months of age and JMML diagnosed at 20 months of age. The child is well today after treatment with chemotherapy and allogenic bone marrow transplantation. With increased awareness, patients with JXG and NF1 who develop symptoms possibly related to JMML, such as paleness, skin bleeding, cough, unexplained fever, and hepatosplenomegaly, should be further evaluated. We also emphasize that multiple JXG lesions can be an early marker of NF1.     

Effect of obstructive sleep apnoea on retinal microvascular function: a randomised controlled trial

Graefe's Archive for Clinical and Experimental Ophthalmology - Retinal microvascular endothelial dysfunction is thought to be of importance in the development of ocular vascular diseases....