Liver transplantation in a case of hypoproteinemia and coagulopathy

A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly. hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore. LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely ‘congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia’.     

重组人生长激素治疗肝硬化低清蛋白血症

目的:观察重组人生长激素(rhGH)治疗肝硬化引起的低清(白)蛋白血症的临床疗效。方法:选择血清清蛋白低于30g·L-1的肝硬化病人46例,男性39例,女性7例,分为治疗组、对照组各23例。治疗组在常规保肝及对症治疗基础上,给予rhGH,4IU,皮下注射,每日1次,连续14d;对照组仅给予常规保肝及对症治疗,14d为一个疗程。检测2组病人治疗前及治疗结束d1和4wk后血清清蛋白及其他肝功能指标变化,并进行临床观察。结果:治疗组治疗前血清清蛋白(25±s3)g·L-1,治疗结束d1血清清蛋白(31±3)g·L-1(P<0.01),4wk后血清清蛋白(33±3)g·L-1(P<0.01);对照组治疗前血清清蛋白(25±3)g·L-1,治疗结束d1血清清蛋白(25±3)g·L-1,4wk后血清清蛋白(24.4±2.6)g·L-1(P>0.05),2组治疗结束d1及4wk后血清清蛋白相比,差异有非常显著意义(P<0.01)。治疗结束d1及4wk内治疗组比对照组病人精神状态、食欲、尿量、腹腔积液、肝功能等均明显改善且持续稳定,有显著差异(P<0.05)。无明显不良反应。结论:rhGH可以增加肝硬化病人的血清清蛋白的水平,改善营养状况,无明显不良反应。     

发热患者中γ干扰素释放实验QuantiFERON-TBGold In Tube不确定结果及影响因素

目的:探讨发热患者中γ干扰素释放实验Quanti FERON-TB Gold In-Tube(QFT-GIT)不确定结果及影响因素。方法:选择感染科因发热收治入院的病例,统计患者一般状况、实验室检查包括γ干扰素释放实验QFT-GIT,血常规、血清白蛋白、补体、降钙素原(PCT)、超敏C反应蛋白(hs-CRP)、乳酸脱氢酶(LDH)、血清铁蛋白(SF)等炎症指标,并进行统计学分析。结果:215例患者中,QFT试验阳性83例,阴性66例,不确定结果 66例。QFT-QIT不确定组患者年龄、SF及LDH水平显著高于QFT确定组(P 0. 01或P 0. 05),淋巴细胞计数及血清白蛋白、补体C3、C4水平显著低于确定结果组(P 0. 01或P 0. 05)。多因素回归分析显示发热患者中出现QFT不确定结果的独立危险因素有低蛋白血症(OR=1. 156,95%CI 1. 028～1. 299)、淋巴细胞减少(OR=6. 009,95%CI 2. 147～16. 817)、高白细胞计数(OR=0. 800,95%CI 0. 680～0. 941)。结论:发热患者中QFT-GIT不确定结果出现比例较高,尤其是在低蛋白血症、低淋巴细胞血症及炎症反应强烈的患者。     

胶体治疗在门脉高压症患者门奇断流术后的应用分析

目的探讨胶体治疗在乙肝肝硬化门脉高压症患者脾切除门奇断流术后应用的安全性和有效性。方法选择2009年1月~2013年12月在本院普外科住院,接受脾切除门奇静脉断流的乙肝肝硬化门脉高压症患者159例,随机分为治疗组87例及对照组72例。对照组采用传统的围术期治疗方法,治疗组额外采用0.6%羟乙基淀粉130/0.4氯化钠500 ml/d常规输注;采集不同组间患者围手术期并发症发生、住院时间、输液总量、白蛋白及血浆输入总量、首次排气及排便时间、低蛋白血症纠正时间、住院经费等指标,统计分析两组间术后并发症发生及相关指标差异,以评估羟乙基淀粉在乙肝肝硬化门脉高压症患者脾切除门奇断流术围手术期诊疗中应用的安全性和有效性。结果治疗组切口并发症、胃肠道功能障碍、水电解质平衡紊乱、胸腹水及总计并发症发生均较低,较对照组差异显著,有统计学意义(P0.05)。与对照组比较,治疗组白蛋白输注量、补液总量及住院费用显著减少,出入院体重下降值增加,差异具有统计学意义(P0.05)。结论在乙肝肝硬化门脉高压症患者脾切除门奇断流术后实施胶体治疗是安全、有效的,可减少术后并发症出现、降低白蛋白输注总量及住院费用,对患者术后治疗具有积极意义。     

Unusual manifestations of henoch-schonlein purpura

Henoch schonlein syndrome (HSP) represents a diagnostic challenge when gastrointestinal symptoms or other atypical symptoms precede the cutaneous manifestations. We describe two patients whose abdominal symptoms antedated cutaneous manifestations. Six-year-old, black male patient, presented with 1 wk history of vomiting (without diarrhea or urinary symptoms), hypertension (140/90 mmHg), diffuse abdominal pain and hypoactive bowel sounds:. WBC’s 40,000/Cu mm³ with 80 percent hypoproteinemia, (albumin 1.6 G%), high plasma renin and stools positive for occult blood. Renal function and urinalysis was normal. Two weeks later patient developed urticarial purpuric rash over the ankles, gross hematuria and gross edema. Biopsy of the skin lesion was consistent with HSP vasculitis. Second patient 4 yr Brazilian male patient presented with abdominalpain, vomiting, blood in the stools, and pain in the right testis. Three days later, developed purpuric rash over his limbs and trunk. He also had hypoproteinemia, hypoalbuminemia, leukocytosis and normal urine analysis. Abdominal pain, bloody stools, painful testicular swelling and hypoproteinemia abated with steroid therapy. In HSP patients, atypical presentation of abdominal pain, hypertension, hypoproteinemia without proteinuria testicular swelling and leucocytosis in the absence of cutaneous and joint manifestations poses as diagnostic problem. Protein-losing enteropathy should be considered in HSP patients with hypoproteinemia.     

补中益气汤合西药治疗肝硬化低蛋白血症62例

探讨中西医结合对肝硬化低蛋白血症的治疗效果。设治疗组 6 2例 ,以中药补中益气汤合西药治疗肝硬化低蛋白血症 ,西药对照组 (38例 )常规西药对症治疗。疗程 6周。结果 :治疗组总有效率 87 2 5 % ,明显优于对照组 6 3 16 % (P <0 0 5 ) ,复发率明显低于对照组 (P <0 0 5 ) ,治疗组治疗前后白蛋白显著升高 (P <0 0 5 ) ,球蛋白显著降低 (P <0 0 5 ) ,白蛋白与球蛋白之比显著升高 (P <0 0 5 ) ,而对照组治疗前后血清蛋白无显著差异 (P >0 0 5 )。提示中西医结合治疗肝硬化低蛋白血症疗效明显优于西药对照组。     

人工关节置换术后低蛋白血症的临床观察与分析

[目的]探讨人工关节置换术后低蛋白血症有针对性的治疗护理措施,预防并发症的发生.[方法]对所有行关节置换的病人分别于术前、术日、术后1 d、术后3 d和术后7 d进行血液生化检查,对检查结果及时分析和记录,并采取措施进行干预.[结果]人工关节置换术后病人血液白蛋白均明显低于正常,尤以术日为甚.[结论]通过监测病人白蛋白指标,有针对性地在肠外和肠内营养支持方面进行调节,对预防术后并发症的发生具有重要的预防作用.     

重组人生长激素对重症脑血管疾病低蛋白血症患者的治疗作用

目的观察重组人生长激素（rhGH）对重症脑血管疾病低蛋白血症患者的治疗作用。方法40例重症脑血管疾病患者,分成治疗组和对照组。治疗组每天应用rhGH,共10d。使用前、使用后第7天、第14天分别检测丽组患者血清蛋白、转铁蛋白、前白蛋白、氮平衡等。结果治疗组使用rhGH后血清蛋白、前白蛋白、转铁蛋白浓度均明显上升,负氮平衡转为正氮平衡;对照组变化不明显。两组间比较,差异有非常显著性意义（P〈0．01）。结论rhGH能有效地纠正重症脑血管疾病低蛋白血症患者的低蛋白血症,增加免疫功能,改善重症脑血管疾病患者的预后。