目的：探讨尿毒症维持性血透(MHD)患者营养不良、炎症与脂质代谢紊乱三者间的相关关系，以及对心血管并发症的预测。方法:测51例尿毒症维持性血透患者血清总胆固醇(TC)、甘油三酯(TG)、高密度脂蛋白(HDL)、低密度脂蛋白(LDL)、载脂蛋白(Apo)A1、ApoB、脂蛋白（a）LP(a)、白蛋白(ALB)、C反应蛋白(CRP)、血清铁蛋白（SF），与30例正常人进行对照研究。结果：MHD患者TG、LDL、ApoB、LP(a)、CRP、SF显著增高,HDL、ApoA1、ALB显著降低(P＜0.05)；随着透析时间的延长，各项检测指标变化进一步加重；且心血管事件发生者血清CRP和LP(a)明显增高，ALB明显降低，血脂紊乱更显著。相关性分析：ALB与HDL、ApoA1正相关，与CRP、LP(a)、TC、TG、ApoB负相关；CRP与LP(a)、TC、TG、ApoB、LDL、SF正相关，与HDL、ApoA1负相关，P均＜0.05。结论：MHD患者存在明显的脂质代谢紊乱、炎症和营养不良，三者的相互促进和影响可能是心血管事件发生的危险因素。
Objective To investigate the effect of myoblast transplantation on duchenne muscular dystrophy (DMD) and to explore the method and feasibil ity of applying gene therapy to DMD. Methods Myoblast of C57/BL10 mice were cultured using multiple-step enzyme digestion method and differential velocity adherent technique. The morphology of the cells was observed with inverted phase contrast microscope. The cells at passage 4 were labeled with 5-BrdU. Twenty-four DMDmodel mice (mdx mice: aged 4-6 weeks, male, 13.8-24.6 g) were randomly divided into two groups (n=12 per group): group A, 1 × 106/mL labeled myoblast were injected via ven caudal is twice at an interval of 2 weeks; group B: 1 mL DMEM/F12 was injected in the same manner serving as a control group. The mice were killed 4 weeks after operation and the motor abil ity of the mice was detected by one-time exhaustive swimming before their death. HE staining and immunohistochemistry staining observation for 5-BrdU, desmin, and dystrophin (Dys) were preformed, and the imaging analysis was conducted. Results The primary myoblast could be sub-cultured 5-7 days after culture, providing stable passage and sufficient cells. The time of onetime exhaustive swimming was (60.72 ± 5.76) minutes in group A and (47.77 ± 5.40) minutes in group B, there was significant significance between two groups (P lt; 0.01). At 4 weeks after injection, HE staining showed that in group A, there were round and transparent-stained myocytes and the percentage of centrally nucleated fibers (CNF) was 67%; while in group B, there were uneven muscle fiber with such pathological changes as hypertrophia, atrophia, degeneration, and necrosis, and the percentage of CNF was above 80%. Immunohistochemistry staining revealed that the expression of 5-BrdU, desmin, and Dys was positive in group A; while in group B, those expressions were l ittle or negative. Image analysis result displayed that integral absorbency (IA) value of desmin was 489.70 ± 451.83 in group A and 71.15 ± 61.14 in group B (P lt; 0.05) and the ratio of positive area to thetotal vision area was 0.314 3 ± 0.197 3 in group A and 0.102 8 ± 0.062 8 in group B (P lt; 0.05); the Dys IA value was 5 424.64 ± 2 658.01 in group A and 902.12 ± 593.51 in group B (P gt; 0.05) and the ratio of positive area to the total vision area was 0.323 7 ± 0.117 7 in group A and 0.035 2 ± 0.032 9 in group B (P lt; 0.05). Conclusion Myoblast transplantation has certain therapeutic effect on DMD of mice.
Objective To review and summarize the latest development of the therapy for the Duchenne muscular dystrophy (DMD). Methods Therecentlypublished articles related to the therapies for DMD were extensively reviewed and briefly summarized. Results The therapeutic approaches for DMD included the gene therapy, the cell therapy, and the pharmacological therapy. The gene therapy and the cell therapy were focused on the treatment for the cause of DMD by the delivery of the missing gene, the modification of the mutated gene, and the transfer of the normal cells including the stem cells, while the pharmacological therapy dealt with the downstream events caused by the dystrophin gene defect, slowed down the pathologic progress of DMD, and improved the DMD patient’s life quality and life span, by medication and other factor treatments. Conclusion There is still no cure for DMD because of various difficulties in replacing or repairing thedefected gene and of the multifaceted nature of the severe symptoms. Therefore,it is imperative for us to find out a more effective treatment that can solve these problems.
Objective To observe the efficacy of photodynamic therapy for vitelliform macular dystrophy(VMD) with choroidal neovascularization(CNV). Methods The clinical data of 7 patients (7 eyes) of VMD with CNV who had undergone photodynamic therapy (PDT) were retrospectively analyzed. The patients were 4 males and 3 females, aged from 20 to 54 years. The patients received the examinations of best corrected visual acuity (BCVA), slitlamp microscopy, fundus photography, fluorescein angiography (FFA), indocyanine green angiography (ICGA), spectral domain OCT(SD-OCT), electrooculogram（EOG）and electroretinogram (ERG)before and after PDT. The BCVA ranged from finger counting to 0.6. Retinal edema and the subretinal fluid were observed. The mean thickness of central retina was (506.00plusmn;30.71) mu;m. PDT was performed according to the standard treatment. The follow-up period ranged from 2 to 11 months with the mean of 6.3 months. The changes of BCVA, CNV and side effects were observed after treatment. Results BCVA improved in all patients ranging from 0.12 to 1.0. The regression of the CNV and resolution of the subretinal fluid were observed by FFA, ICGA and SD-OCT after PDT. The mean thickness of central retina was reduced to (401.00plusmn;52.22) mu;m. There was no PDTassociated ocular or systemic side effect. Conclusions PDT is an effective and safe treatment for VMD with CNV. It may improve or stabilize the visual acuity.
Objective To study the clinical characteristics of myotonic dystrophy. Method Patient records in West China Hospital, Sichuan University and China Biological Medicine Database (CBM-disc 1980-1999) were searched. Demographic data, clinical manifestations, laboratory findings of patients with myotonic dystrophy were analyzed. Results Of the total 97 patients, 64 cases were male, and 33 were female. Mean age was 28.5 years old. Ninety percent of patients had a family history. The frequency of symptoms in turn was myotonia (99%), muscle weakness (97%), muscle atrophy (85%), cataract (63%), hair losing or bald (57%) and gonadal atrophy (37%), sexuality disfunction (33%), heart damage (11%), intelligence impairment (11%), hypothyroid or disfunction of adrenal gland (8%), mental state disorders (8%). Conclusions In this group of patients, myotonia, muscle weakness and muscle atrophy were most common symptoms. In addition, some other systemic symptoms were common, such as cataract, hair losing, bald and gonadal atrophy. The clinical manifestations of myotonic dystrophy were complex.
目的 探索微型营养评估简易法（MNA-SF）能否客观、正确地评估老年住院患者的营养状况以及对临床预后进行预测。 方法 选择2012年3月－4月四川大学华西医院新入院老年患者407例，平均年龄（75.4 ± 7.10）岁，以MNA-SF评估其营养状态并根据营养状态分为两组，随访至出院或住院第30 d，分析其营养状况与住院时间，住院费用，全身炎症反应综合征（SIRS），死亡等临床结局的相关性。 结果 营养不良者22.6%（92例），营养不良风险者31.9%（130例），营养状态良好者45.5%（185例）。其中34例营养不良者，18例营养不良风险者和4例营养良好者发生SIRS；另有11例营养不良者，7例营养不良风险者和1例营养良好者死亡。MNA-SF分值在0～7分的患者无论在发生SIRS还是临床不良结局方面显著高于分值为8～14分的患者（P＜0.05）。营养不良者住院时间为（14.6 ± 8.30）d，营养不良风险或营养良好者为（12.1 ± 7.99）d，差异具有统计学意义（P＜0.05）。 结论 MNA-SF是一可靠，简便易行，可以预测老年住院患者临床预后的营养评估工具。
【摘要】 目的 探索持续非卧床腹膜透析（CAPD）患者年龄与营养状况的关系，为营养教育提供指导。 方法 对2007年8月-2010年1月腹膜透析177例患者的临床资料进行回顾性分析，包括血红蛋白（HGB）、血清白蛋白（ALB）、年龄、体重指数（BMI）、腹膜平衡试验（PET）、尿素清除指数（Kt/V）、24 h尿蛋白、腹透液漏出蛋白、标准化每日蛋白质分解率（nPCR）等，分析年龄与营养状况的关系。 结果 老年组（≥60岁）和中青年组（lt;60岁）ALB分别为（31.54±5.91）、（33.24±4.75）g/L，老年组ALB较低（Plt;0.05）；老年组Kt/V值1.95±0.63和HGB水平（95.25±22.89） g/L均高于青年组（Plt;0.05）。两组患者营养不良与BMI、PET、每日蛋白漏出总量、nPCR无关。 结论 CAPD老年患者（≥60岁）更容易发生营养不良，且老年组低蛋白血症与透析充分性无明显关系。故应加强老年CAPD患者的饮食营养管理指导，设计合理的饮食。【Abstract】 Objective To investigate the relationship between age and nutrition status of the patients undergoing continuous ambulatory peritoneal dialysis (CAPD), and provide instructions for nutrition education. Methods We retrospectively analyzed the clinical data of 177 patients having undergone CAPD in the Department of Nephrology of West China Hospital between April 2007 and January 2010. Clinical parameters collected included hemoglobin (HGB), serum albumin (ALB), age, body mass index (BMI), results of the peritoneal equilibrium test (PET), urea clearance index (Kt/V), 24-hour urinary protein, protein loss in the peritoneal dialysate, normalized protein catabolic rate (nPCR). Relationship between age and nutrition status in these patients were analyzed. Results Plasma albumin was (31.54±5.91) g/L and (33.24±4.75) g/L respectively in patients of or older than 60 years old (the elderly group) and in patients younger than 60 years old (the younger group). Compared with the younger group, patients in the elderly group had lower ALB (Plt;0.05), but higher Kt/V 1.95±0.63 and hemoglobin level (95.25±22.89) g/L (Plt;0.05). In addition, malnutrition in both groups was not associated with BMI, PET results, daily protein loss in the peritoneal dialysate or nPCR. Conclusions Patients of or older than 60 years old undergoing CAPD are more likely to develop malnutrition, indicating that hypoalbuminemia is not associated with dialysis inadequacy. We advocate enhancing diet nutrition education in elderly CAPD patients and providing them with balanced diet regime.