4例暴发型肝豆状核变性患者的临床特点及其治疗

我们采用注射激素、驱铜、血浆置换等方法综合治疗4例儿童暴发型肝豆状核变性(FWD),3例黄疸消退,肝功能基本恢复正常,现报道如下.     

Observations on the immunocytes and macrophages in megacolon

An immunoperoxidase staining method was used to study specific immunoglobulin-containing cells in the intestinal mucosa of children who presented with features suggestive of Hirschsprung's disease. No evidence was found to substantiate the hypothesis of an immunologic component in the etiology of Hirschsprung's disease. The megacolon in this condition had an increased proportion of IgG-containing cells and a reduced proportion of IgA-containing cells when compared with normal and unobstructed colon; this may be a reflection of an abnormal mucosal immune defense leading to susceptibility to the enterocolitis found in Hirschsprung's disease. Numerous lipofuscin-bearing macrophages were seen in the colonic mucosa of children with anal stenosis who were given anthraquinone laxatives for varying periods. The possibility that this represents an early stage of melanosis coli, as well as the likelihood of anthraquinones contributing to the pathogenesis of acquired megacolon, are considered. S. S. Wijesinha was on study leave from the Department of Health, Republic of Sri Lanka, and was supported by a Scholarship awarded by the Association of Commonwealth Universities.     

The impact of short-term hyperglycemia and obesity on biventricular and biatrial myocardial function assessed by speckle tracking echocardiography in a population of women with gestational diabetes mellitus

Background and aimsTo compare biventricular and biatrial myocardial strain indices assessed by two-dimensional speckle tracking echocardiography (2D-STE) in women with gestational diabetes mellitus (GDM) and those with uncomplicated pregnancy at the third trimester of pregnancy and in post-partum.Methods and results30 consecutive GDM women and 30 age-, ethnicity- and gestational week-matched controls without any comorbidity were examined in this prospective case–control study. All women underwent obstetric visit, blood tests and transthoracic echocardiography (TTE) implemented with 2D-STE analysis of all cardiac chambers at 36–38 weeks’ gestation. TTE and 2D-STE were repeated at 6–10 weeks after delivery.At 36–38 weeks’ gestation, GDM women, compared to controls, had significantly higher body mass index (BMI), blood pressure values and inflammatory markers. TTE showed increased left ventricular (LV) mass and impaired LV diastolic function in GDM women, whereas there was no significant difference between the groups in ejection fraction. 2D-STE revealed that biventricular global longitudinal strain (GLS) and biatrial reservoir strain indices were significantly lower in GDM women than controls. Third trimester BMI was inversely correlated with LV-GLS (r = ?0.86) and was independently associated with reduced LV-GLS (less negative than ?20%) in GDM women in post-partum (OR 1.81, 95%CI 1.14–2.89). A BMI value ≥ 30 kg/m² had 100% sensitivity and 99.5% specificity for identifying GDM women with impaired LV-GLS in post-partum (AUC = 0.97).ConclusionWomen with GDM, compared to women with uncomplicated pregnancy, have significantly lower biventricular and biatrial myocardial deformation indices. These abnormalities may be persistent in post-partum in GDM women with obesity.     

Blood micronutrient, oxidative stress, and viral load in patients with chronic hepatitis C

AIM: To assess the extent of micronutrient and oxidative stress in blood and to examine their linkages with viral loads in chronic hepatitis C patients. METHODS: Hepatitis C virus (HCV)-RNA levels were quantified in the serum from 37 previously untreated patients with chronic hepatitis C. The plasma and erythrocyte micronutrients (zinc, selenium, copper, and iron) were estimated, and malondialdehyde (MDA) contents were determined as a marker to detect oxidative stress. Antioxidant enzymes, superoxide dismutase (SOD), glutathione peroxidase (GPX) and glutathione reductase (GR) activities in blood were also measured. The control group contained 31 healthy volunteers. RESULTS: The contents of zinc (Zn), and selenium (Se) in plasma and erythrocytes were significantly lower in hepatitis C patients than in the controls. On the contrary, copper (Cu) levels were significantly higher. Furthermore, plasma and erythrocyte MDA levels, and the SOD and GR activities in erythrocytes significantly increased in hepatitis C patients compared to the controls. However, the plasma GPX activity in patients was markedly lower. Plasma Se (R=-0.730, P<0.05), Cu (r=0.635), and GPX (r= -0.675) demonstrated correlations with HCV-RNA loads. Significant correlation coefficients were also observed between HCV-RNA levels and erythrocyte Zn (r=-0.403), Se (r = -0.544), Cu (r= 0.701) and MDA (P=0.629) and GR (r =0.441). CONCLUSION: The levels of Zn, Se, Cu, and oxidative stress (MDA), as well as related anti-oxidative enzymes (GR and GPX) in blood have important impact on the viral factors in chronic hepatitis C. The distribution of these parameters might be significant biomarkers for HCV.     

老年功能性便秘与脂质过氧化的关系

检测60例老年功能性便秘患者和48例健康老年对照者血浆过氧化脂质(P—LPO)、红细胞过氧化脂质(E—LPO)含量的结果表明,患者组的P—LPO、E—LPO平均含量皆显著高于对照组(P<0.005～0.001),患者排便周期与P—LPO、E—LPO含量之间均呈直线正相关(r=0.697,P<0.001;r=0.780,P<0.001)。提示老年功能性便秘患者体内的脂质过氧化反应呈现病理性加剧。     

Acute changes in plasma non-esterified fatty acid concentration do not change hepatic glucose production in people with type 2 diabetes

The effect of changes in plasma non-esterified fatty acid concentration (NEFA) on plasma glucose concentration, hepatic glucose production (Ra), and glucose disposal (Rd) rates was determined in 14 patients with Type 2 diabetes. Seven patients had relatively mild fasting hyperglycaemia (less than 10.0 mmol l-1), whereas the remaining seven had relatively severe fasting hyperglycaemia (greater than 14.0 mmol l-1). Each patient was infused from 2000 to 0800 h with 3-3H-glucose on two occasions, with or without neutral fat emulsion and heparin (mild hyperglycaemia group), or with or without nicotinic acid (severe hyperglycaemia group). Plasma NEFA concentration increased from 0.33 +/- 0.06 (+/- SE) to 4.78 +/- 0.42 mmol l-1 in response to the lipid and heparin infusion, but plasma glucose concentration (7.8 +/- 0.7 vs 7.4 +/- 0.8 mmol l-1), Ra (0.44 +/- 0.02 vs 0.46 +/- 0.02 mmol m-2 min-1), and Rd (0.42 +/- 0.02 vs 0.46 +/- 0.02 mmol m-2 min-1) were unchanged. Nicotinic acid decreased plasma NEFA concentration from 0.54 +/- 0.15 to 0.23 +/- 0.08 mmol l-1, but plasma glucose (15.0 +/- 1.0 vs 15.5 +/- 1.4 mmol l-1), Ra (0.74 +/- 0.07 vs 0.68 +/- 0.07 mmol m-2 min-1), and Rd (0.73 +/- 0.07 vs 0.68 +/- 0.07 mmol m-2 min-1) were unchanged. The results indicate that acute changes in plasma NEFA concentration did not lead to any change in overnight glucose production or disposal rates.     

Glycaemia,arterial pressure and micro-albuminuria in Type 1 (insulin-dependent) diabetes mellitus

Summary Plasma glucose control and arterial pressure were assessed in 28 Type 1 (insulin-dependent) diabetic patients with different degrees of micro-albuminuria. They were divided into two groups according to their urinary albumin excretion rate: a low micro-albuminuria group (n= 16) with albumin excretion ranging between 12.1 and 28.9 g/min and a high micro-albuminuria group (n= 12) with albumin excretion between 32.4 and 91.3 g/min. The groups were matched for age, sex and duration of diabetes with the same number of normo-albuminuric (2.0–10.4 g/min) diabetic control subjects. Both the low and high micro-albuminuria groups had significantly higher glycosylated haemoglobin levels and mean plasma glucose concentrations during a 24-h profile than their respective normo-albuminuric control subjects. A correlation between glycosylated haemoglobin level and urinary albumin excretion rate was found in the whole study group (r= 0.48; p< 0.001). Arterial pressure (both systolic and diastolic) was significantly higher in the high micro-albuminuria group than in either the control group or the low microalbuminuria group. A significant correlation was found between arterial pressure and albumin excretion rate in the whole study population (r= 0.49; p< 0.001) as well as in the pooled micro-albuminuria groups (r= 0.43; p< 0.05). Multiple regression analysis showed that glycosylated haemoglobin and arterial pressure levels were independently correlated with albumin excretion rates. Diabetic patients with micro-albuminuria of any degree have worse glycaemic control than normo-albuminuric patients. Higher levels of arterial pressure, though often sub-hypertensive, are associated with levels of micro-albuminuria predictive of later development of clinical proteinuria. Thus high plasma glucose and high arterial pressure, or both, characterise those diabetic patients at increased risk of nephropathy. These indices of risk are potentially reversible.     

Effects of exogenous hormones and glucose on plasma levels and hepatic metabolism of amino acids in the fetus and in the newborn rat

Summary The present study examines the role of insulin, glucagon and cortisol in the regulation of gluconeogenesis from lactate and amino acids in fetal and newborn rats. Injection of glucagon in the fullterm fetal rat caused a rise in glucose (and insulin) and a fall in blood levels of most individual amino acids, stimulated hepatic accumulation of ¹⁴C-amino isobutyric acid and ¹⁴C-cycloleucine and increased the conversion of ¹⁴C lactate, alanine and serine to glucose in vivo and in vitro (liver slices). Such changes were equivalent to the changes seen in 4 h old newborn rats. When glucagon was administered at birth, little difference was observed between control and treated animals in plasma amino acids and a smaller increment in conversion of ¹⁴C substrate to glucose occurred. By contrast, insulin injection at birth caused hypoglycemia, suppression of levels of certain amino acids and inhibition of conversion of ¹⁴C substrates into glucose. Glucose injection at birth caused elevated glycemia and plasma insulin and suppression of most amino acid levels and of conversion of ¹⁴C substrate into glucose. Cortisol injection at birth caused a marked, generalized hyperaminoacidemia, a stimulation of glucagon secretion and of conversion of ¹⁴C substrates into glucose. These observations support the thesis that glucagon plays a major role in the induction of hepatic gluconeogenesis and that insulin acts as an antagonist hormone.     

2013年广西血液净化治疗基础现状调查

目的：了解广西血液净化基础现状。方法对广西14个地市115家具备血液净化准入资质的医院进行血液净化基础现状调查,内容包括机器、医生、护师、技师数量,2013年全年开展血液净化治疗的总例次及血液透析、血液透析滤过、血液灌流、血浆置换、连续性血液净化的例次,不良反应发生例次。结果广西拥有血液净化机器1745台,具备血液净化诊疗资质的医生348名,护士828名,技师110名。2013年开展血液净化治疗的总例次为928155例次,血液透析855154例次、血液透析滤过36959例次、血液灌流24714例次、血浆置换1306例次、连续性血液净化10022例次,不良反应发生11097例次。结论广西血液净化诊疗机器设备与医护比例匹配,治疗模式主要是血液透析、血液透析滤过和血液灌流,需要加强对不良反应的监测。     

Efficacy of plasma exchange in children with severe hemophagocytic syndrome: a prospective randomized controlled trial北大核心CSCD

目的 探讨血浆置换在辅助救治儿童重症噬血细胞综合征（hemophagocytic syndrome,HPS）中的疗效和应用价值。 方法 采用前瞻性随机对照研究方法,选取2018年10月至2020年10月在湖南省儿童医院儿童重症监护室（pediatric intensive care unit,PICU）接受救治的重症HPS患儿40例为研究对象,将患儿随机分为置换组和常规组（n=20）,常规组患儿进行病因和对症常规支持治疗,置换组在常规治疗的基础上加以血浆置换辅助治疗。对两组患儿的基本情况、治疗前后的临床症状体征、主要实验室检查指标、疗效和预后情况进行比较分析。 结果 两组患儿治疗前在性别、年龄、入院前病程、病因构成、小儿危重病例评分、器官或系统功能累及情况等指标上比较差异均无统计学意义（P>0.05）,在实验室指标的比较上差异亦均无统计学意义（P>0.05）。两组患儿治疗7 d后,临床症状体征均有所缓解和改善。治疗后置换组C反应蛋白、降钙素原、血清铁蛋白、丙氨酸氨基转移酶、总胆红素水平均低于常规组（P<0.05）。置换组的PICU住院时间较常规组明显缩短,总有效率较常规组明显提高（P<0.05）,但两组总的住院时间和3个月病死率比较差异无统计学意义（P>0.05）。 结论 血浆置换在辅助治疗儿童重症HPS上的疗效优于常规治疗,能改善患儿的临床症状体征和部分实验室指标,缩短PICU住院时间,可能成为治疗儿童重症HPS的一项有效辅助治疗方法。