肝癌患者因食欲降低、进食减少导致不同程度的营养不良,引发厌食症,甚至恶病质,严重影响患者生存质量。食欲评估是降低营养风险的基础,医护人员可通过食欲症状的评估调整患者饮食及营养指导措施,以改善患者营养状态,提高生存质量。该文对食欲的评估工具、方法及干预在肝癌营养不良患者中的应用现状进行阐述,为肝癌患者的食欲干预提供科学依据。

Hepatocellular carcinoma patients suffer from different degrees of malnutrition due to decreased appetite and eating,which triggers anorexia and even cachexia,seriously affecting the quality of patient survival．Appetite assessment is the basis for reducing nutritional risk,and healthcare professionals can adjust patients’ diet and nutritional guidance measures through the assessment of appetite symptoms in order to improve patients’ nutritional status and survival quality．In this paper,the current status of the assessment tools,methods and interventions of appetite in malnutrition patients with hepatocellular carcinoma are described to provide a scientific basis for appetite interventions．

目的 观察脑性瘫痪（CP）伴营养不良患儿进行系统性饮食调整的效果。方法 2015年1月—2022年1月我院收治以CP伴营养不良患儿80例,以随机数字表法分为对照组和研究组各40例,常规护理指导用于对照组,系统性饮食调整护理用于研究组。比较2组患儿在干预前、后的生化指标（白蛋白、血红蛋白、肌酐、低密度脂蛋白水平）、身体指标（体质量、身高、体质指数、腹部皮下脂肪厚度）、肠内营养混悬液使用剂量及饮食行为能力评分和患儿监护人对干预的满意率。结果 干预后2组的生化指标可见明显变化,其中研究组与对照组相比,白蛋白、血红蛋白、肌酐指标提升更多更平稳,而低密度脂蛋白研究组较对照组相比,降低更多更平稳,（P<0.01）。在干预后两组间的身体指标可见明显变化,其中研究组与对照组相比,体质量、体质指数、腹部皮下脂肪厚度较干预前增加更多更平稳,而低密度脂蛋白研究组较对照组相比,降低更多更平稳,（P<0.01）。在干预后2组的肠内营养混悬液使用剂量及饮食行为能力评分较干预前可见明显变化,其中研究组与对照组相比,肠内营养混悬液使用剂量及饮食行为能力降低更多更平稳（均P<0.05）。研究组监护人对干预的满意率高于对照组（P<0.05）。结论 在CP伴营养不良患儿中,开展有效的系统性饮食调整干预,可有效改善患儿的生化指标,提升各项身体指标,减少肠内营养混悬液的使用剂量,改善饮食行为能力,获得患儿监护人的认可,效果理想。

Objective To observe the effect of systematic diet adjustment in children with cerebral palsy（CP）and malnutrition．Methods A total of 80 CP children with malnutrition treated in our hospital（from January 2015 to January 2022）were divided into two groups by random number table method．Control group received routine nursing guidance and study group received systematic diet adjustment nursing．The biochemical indexes（albumin,hemoglobin,creatinine,low-density lipoprotein levels）,physical indexes（weight,height,body mass index,abdominal subcutaneous fat thickness）,the dosage of enteral nutritional suspension and the score of dietary behavior ability were compared between the two groups before and after the intervention．The satisfaction rate of the children's families with the intervention was evaluated．Results After the intervention,the biochemical indexes of the two groups showed significant changes．Compared with the control group,the indexes of albumin,hemoglobin and creatinine in the study group increased more and more stably,while the index of low-density lipoprotein in the study group decreased more and more stably（P<0.01）．After the intervention,the physical indexes of the two groups showed significant changes．Compared with the control group,the weight,body mass index and abdominal subcutaneous fat thickness of the study group increased more and more stably than before the intervention,while the low-density lipoprotein study group decreased more and more stably（P<0.01）．After the intervention,the dosage of enteral nutritional suspension and the score of dietary behavior ability in the two groups showed significant changes compared with those before the intervention．Compared with the control group,the immune indexes in the study group increased more and more stably,while the dosage of enteral nutritional suspension and the score of dietary behavior ability in the study group decreased more and more stably（P<0.05）．The satisfaction rate of family members in the study group was higher than that in the control group（P<0.05）．Conclusions In children with CP and malnutrition,carrying out effective systematic diet adjustment intervention can significantly improve the biochemical indexes of children,improve various physical indexes,improve immune ability,reduce the dosage of enteral nutritional suspension,improve dietary behavior,and obtain the recognition of children's family members,which has ideal effects．

目的 总结一个以心脏损害为首发症状的假肥大肌营养不良症家系的遗传学及临床特征。方法 对先证者和家系成员进行临床观察、并收集其血清酶、胸片、心电图、 心脏彩色超声、肌肉组织活检及抗肌萎缩蛋白基因突变检测等结果。结果 先证者及家系成员患者符合假肥大肌营养不良症诊断,但以心脏扩大为首发症状,表现为心肌酶谱异常,心电图异常,心彩超提示扩张型心肌病,同时骨骼肌受累不明显,基因检测提示先证者及家系成员患者携带抗肌萎缩蛋白基因外显子40的无义突变[c.5632C>T, p(Gln1878^*)]。结论 该家系成员患者符合X连锁扩张型心肌病诊断,患者存在新发的抗肌萎缩蛋白基因无义突变。

Objective To summarize the genetic and clinical characteristics of a family with Duchenne muscular dystrophy (DMD) with cardiac damage as the first symptom. Methods The results of clinical observations, laboratory tests, chest radiographs, electromyography, echocardiography, muscle tissue biopsies, and DMD gene mutations were collected. Results Patients with probands and family members met the diagnosis of DMD. However, cardiac enlargement was the first symptom, presenting as abnormal myocardial enzyme spectrum, abnormal electrocardiogram, and dilated cardiomyopathy in echocardiography, while skeletal muscle involvement was not obvious. One nonsense pathogenic mutation c.5632C>T, p(Gln1878^*) of exon 40 in the DMD gene was identified in the patients with probands and family members. Conclusion The patients of this family met the diagnosis of x-linked dilated cardiomyopathy, and a new nonsense pathogenic mutation of DMD gene was identified in this family.