Short‐term results in infants with multiple left heart obstructive lesions

Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructivelesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aorticor mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV)and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology andpresence of a ventricular septal defect (VSD) and coarctation were assessed. Clinicaloutcomes included successful 2V repair, complications, and repeat interventions orsurgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death.
Results: For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients wereselected. Initial surgical procedures of the 2V group were simple coarctation repair(5), complex coarctation/arch reconstruction +/− septal defect closure (6), hybridstage 1 (2), and none (1). Three of the 2V patients required reintervention in the first90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratioof <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aorticvalve z score were significantly larger in the 2V group compared to the 1V group.All patients in the 1V group had a nonapex forming LV. There was no mortality withfollow‐up to three years of age.
Conclusions: This study showed excellent short‐term and midterm surgical results inthe 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in therisk stratification of a successful 2V repair even in cases with a small MV.     

Growth evaluation in infants with neonatal cholestasis

BACKGROUND: [corrected] Chronic liver diseases in childhood often cause undernutrition and growth failure. To our knowledge, growth parameters in infants with neonatal cholestasis are not available AIM: To evaluate the nutritional status and growth pattern in infants with intrahepatic cholestasis and extrahepatic cholestasis. PATIENTS AND METHODS: One hundred forty-four patients with neonatal cholestasis were followed up at the Pediatric Gastroenterology Service of the Teaching Hospital, State University of Campinas, Campinas, SP, Brazil, in a 23-year period, from 1980 to 2003. The records of these patients were reviewed and patients were classified into two groups, according to their anatomical diagnosis: patients with intrahepatic cholestasis - group 1, and patients with extrahepatic cholestasis - group 2. Records of weight and height measurements were collected at 4 age stages of growth, in the first year of life: 1) from the time of the first medical visit to the age of 4 months (T1); 2) from the 5th to the 7th month (T2); 3) from the 8th to the 10th month (T3); and 4) from the 11th to the 13th month (T4). The weight-by-age and height-by-age Z-scores were calculated for each patient at each stage. In order for the patient to be included in the study it was necessary to have the weight and/or height measurements at the 4 stages. Analyses of variance and Tukey's tests were used for statistical analysis. Repeated measurement analyses of variance of the weight-by-age Z-score were performed in a 60-patient sample, including 29 patients from group 1 and 31 patients from group 2. The height-by-age data of 33 patients were recorded, 15 from group 1 and 18 from group 2 RESULTS: The mean weight-by-age Z-scores of group 1 patients at the 4 age stages were: T1=-1.54; T2=-1.40; T3=-0.94; T4=-0.78. There was a significant difference between T2 X T3 and T1 X T4. The weight-by-age Z-scores for group 2 patients were :T1=-1.04; T2=-1.67; T3=-1.93 and T4=-1.77, with a significant difference between T1 X T2 and T1 X T4. The mean weight-by-age Z-scores also showed a significant difference between group 1 and group 2 at stages T3 and T4. The mean height-by-age Z-scores at the four stages in group 1 were: T1=-1.27; T2=-1.16; T3=-0.92 and T4=-0.22, with a significant difference between T3XT4 and T1XT4. The scores for group 2 patients were: T1=-0.93; T2=-1.89; T3=-2.26 and T4=-2.03, with a significant difference between T1XT2 and T1XT4. The mean height-by-age Z-scores also showed a significant difference between group 1 and group 2 at T3 and T4 CONCLUSION: The weight and height differences between the groups became significant from the 3rd measurement onward, with the most substantial deficit found in the extrahepatic group. In this group, there is evidence that the onset of weight and height deficit occurs between the first and second evaluation stages.     

Left ventricular function in normal newborn infants and asymptomatic infants with neonatal polycythemia

Digitized M-mode echocardiography was used to evaluate left ventricular (LV) function in normal neonates and to document subtle abnormalities of LV function in 19 asymptomatic newborn infants with polycythemia and hyperviscosity. Echocardiograms were performed before and after partial exchange transfusion and at 48 hours of age. Results were compared to those obtained from analysis of echocardiograms from 18 normal infants with normal hematocrits at 12 and 48 hours of age. In the normal group there were no differences between the digitized indices of LV function at 12 hours and those at 48 hours of age. In the polycythemic group, prior to hemodilution, the peak rate of LV emptying was low compared to normal values (-3.1 +/- 0.7 sec-1 vs -3.6 +/- 1.0 sec-1, p less than 0.05) and normalized following the procedure to -3.7 +/- 1.3 sec-1. The peak rate of left ventricular posterior wall (LVPW) thickening also increased following partial exchange transfusion (3.2 +/- 0.6 sec-1 to 3.7 +/- 1.0 sec-1, p less than 0.05). At 48 hours of age peak rates of change for the left ventricle (-2.9 +/- 0.8) and LVPW (3.0 +/- 0.7) were abnormally low in the polycythemic infants, all of whom had undergone partial exchange transfusion. Also, at 48 hours, the LVPW time to peak rate of thickening and time to the end of thickening were prolonged. In addition, the peak rate of LV filling in diastole was low in the polycythemic group (3.0 +/- 0.9 sec-1 vs 3.9 +/- 1.3 sec-1, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

Nutritional status of infants with neonatal cholestasis

Purpose We constructed a study to determine the association of anthropometric measurements, biochemical parameters and bone mineral content with nutritional status in infants with neonatal cholestasis. Methods The study included 38 children with neonatal cholestasis. Nutritional status was assessed by Waterlow criteria, and anthropometric measurements, biochemical parameters and bone mineral content were correlated with the nutritional status at initial admission and at the end of 2 months after nutritional support. Results We found that the prevalence of acute and chronic malnutrition was 34.2% and 39.4% in infants with neonatal cholestasis, respectively. No significant difference was found in triceps skinfold thickness (TST), scapular skinfold thickness (SST) and suprailiac skinfold thickness (SuST), arm fat area (AFA) and arm area (AA) among the groups. Mid-arm circumference (MAC), arm muscle circumference (AMC) and arm muscle area (AMA) were significantly lower in patients with chronic malnutrition than both acute malnutrition and patients without malnutrition. MAC was the most reliable marker for the assessment of malnutrition and had the highest positive predictive value (PPV) (80.6%), sensitivity (89.2%) and negative predictive value (NPV) (57.1%). Prealbumin levels were significantly lower in patients with chronic malnutrition than the patients without malnutrition. Increment in MAC and AMC were significantly high in the three groups after 2 months. Conclusion MAC is a good indicator of malnutrition in neonatal cholestasis and may also be used for the monitoring of nutritional support; prealbumin may be used for assessment of the severity of malnutrition.     

肝性脑病大鼠脑超微结构的改变

目的：通过观察肝性脑病大鼠脑部分区域及氨对体外培养大鼠神经元超微结构的改变，讨论其病理发生机制。方法选用健康雄性SD大鼠12只，随机分为肝性脑病模型组和正常对照组两组，每组6只。用电子透射显微镜观察硫代乙酰胺诱导的肝性脑病大鼠和体外氨中毒大鼠皮质神经元的超微结构。结果肝性脑病大鼠神经元细胞数量减少；神经元线粒体肿胀，尼氏体数量明显减少；可见凋亡各期表现。神经胶质细胞细胞器减少，黑质的超微结构改变程度较基底核略重。体外培养氨中毒神经元变化：神经元细胞数量明显减少；细胞明显水肿，线粒体明显肿胀，尼氏体显著减少；可见不同时期的凋亡表现。结论肝性脑病大鼠脑超微结构改变明显，其主要机制可能与氨中毒引起的神经元凋亡有关。     

新生儿缺血缺氧性脑病的超声诊断价值

目的研究头颅彩色多普勒血流动力学和颅内结构改变在诊断新生儿缺血缺氧性脑病(HIE)中的应用价值。方法应用彩色多普勒及二维超声检测58例HIE患儿及20例正常出生1～2d内新生儿的大脑中动脉、大脑前动脉参数及脑组织结构变化情况。结果出生1～2d内窒息的早产儿及足月儿大脑中动脉和大脑前动脉收缩期峰值流速(Vs)及舒张末期流速(Vd)较对照组下降,阻力指数较对照组升高,与对照组比较差异有统计学意义(P0.05),双侧或单侧侧脑室冠状面前角变小,甚至不显示,侧脑室周边脑组织回声增强,呈放射状或脑实质内广泛均匀分布的轻度回声增强。结论彩色多普勒及二维超声对诊断HIE及指导治疗有着重要意义。     

新生儿缺氧缺血性脑病患者血浆GMP-140水平变化及意义

目的：研究新生儿缺氧缺血性脑病（ｈｙｐｏｘｉｃ－ｉｓｃｈｅｍｉｃ ｅｎｃｅｐｈａｌｐｏｐａｔｈｙ,ＨＩＥ）患者血浆α颗粒膜蛋白－１４０（ｇｒａｎｕｌｅ ｍｅｍｂｒａｎｅ ｐｒｏｔｅｉｎ－１４０,ＧＭＰ－１４０）水平变化,探讨其临床意义。方法：４３例ＨＩＥ患儿分为轻、中、重３级,并以正常新生儿作为对照。血浆ＧＭＰ－１４０采用ＥＬＩＳＡ双抗体夹心法测定。结果：起病初期,血浆ＧＭＰ－１４０水平显著升高,     

Mechanisms of gastro-oesophageal reflux in preterm and term infants with reflux disease

BACKGROUND AND AIMS: Invoked peristaltic contractions and movement of solid content have not been attempted in normal canine colon. The purpose of this study was to determine if movement of solid content through the colon could be produced by microprocessor controlled sequential stimulation. METHODS: The study was performed on six anaesthetised dogs. At laparotomy, a 15 cm segment of descending colon was selected, the proximal end closed with a purse string suture, and the distal end opened into a collecting container. Four sets of subserosal stimulating electrodes were implanted at 3 cm intervals. The segment of bowel was filled with a mixture of dog food and 50 plastic pellets before each of 2-5 random sessions of non-stimulated or stimulated emptying. Propagated contractions were generated using microprocessor controlled bipolar trains of 50 Hz rectangular voltage having 20 V (peak to peak) amplitude, 18 second stimulus duration, and a nine second phase lag between stimulation trains in sequential electrode sets. RESULTS: Electrical stimulation using the above mentioned parameters resulted in powerful phasic contractions that closed the lumen. By phase locking the stimulation voltage between adjacent sets of electrodes, propagated contractions could be produced in an aboral or orad direction. The number of evacuated pellets during the stimulation sessions was significantly higher than during the non-stimulated sessions (p<0.01). CONCLUSIONS: Microprocessor controlled electrical stimulation accelerated movement of colonic content suggesting the possibility of future implantable colonic stimulators.     

Synchronous skin lesions in mother and baby with neonatal lupus erythematosus

Clinical Rheumatology -     

Microglial proliferation in the brain of chronic alcoholics with hepatic encephalopathy

Hepatic encephalopathy (HE) is a common complication of chronic alcoholism and patients show neurological symptoms ranging from mild cognitive dysfunction to coma and death. The HE brain is characterized by glial changes, including microglial activation, but the exact pathogenesis of HE is poorly understood. During a study investigating cell proliferation in the subventricular zone of chronic alcoholics, a single case with widespread proliferation throughout their adjacent grey and white matter was noted. This case also had concomitant HE raising the possibility that glial proliferation might be a pathological feature of the disease. In order to explore this possibility fixed postmortem human brain tissue from chronic alcoholics with cirrhosis and HE (n?=?9), alcoholics without HE (n?=?4) and controls (n?=?4) were examined using immunohistochemistry and cytokine assays. In total, 4/9 HE cases had PCNA- and a second proliferative marker, Ki-67-positive cells throughout their brain and these cells co-stained with the microglial marker, Iba1. These cases were termed ‘proliferative HE’ (pHE). The microglia in pHEs displayed an activated morphology with hypertrophied cell bodies and short, thickened processes. In contrast, the microglia in white matter regions of the non-proliferative HE cases were less activated and appeared dystrophic. pHEs were also characterized by higher interleukin-6 levels and a slightly higher neuronal density . These findings suggest that microglial proliferation may form part of an early neuroprotective response in HE that ultimately fails to halt the course of the disease because underlying etiological factors such as high cerebral ammonia and systemic inflammation remain.     

Human immunodeficiency virus in brain biopsies of patients with AIDS and progressive encephalopathy

Human immunodeficiency virus (HIV) has been identified in patients with AIDS in their circulating and tissue lymphocytes and in their monocyte-macrophages, an arm of the cell-mediated immune system. We report our findings of HIV in brain biopsies. Virus was detected in oligodendroglial and astroglial cells. Virus was also present in the brain capillaries, both in lumens and at the endothelial gaps. Virus was also found in extracellular spaces. Budding forms of the developing virus and bar-shaped nucleoids of mature virus, the diagnostic hallmark of the HIV retrovirus, were identified in brain tissue of five of seven patients with AIDS studied. We believe that HIV may enter the the brain via the vascular capillaries (through the endothelial gaps), bind to brain cells containing T4 receptors, enter the cells, and replicate. There follows a reactive hyperplasia and degeneration of oligodendroglial and astroglial cells. The oligodendroglial cells are the myelin-forming cells, and thus, the result is myelin degeneration.     

晚期早产儿及足月儿急性胆红素脑病92例临床特点分析

［摘要］　目的　分析晚期早产儿及足月儿急性胆红素脑病（ABE）的临床特点。方法　回顾性分析2015-01～2017-12河南省4家医院胎龄≥35周的新生儿胆红素脑病患儿92例的临床资料。结果　92例患儿中入院日龄的中位数为5.0 d,黄疸出现日龄的中位数为2.1 d,诊断为胆红素脑病时间的中位数为4.0 d。原发疾病中溶血性疾病53例（57.6%）,败血症或感染36例(39.1%),出生相关的创伤性疾病2例(2.2%),1例未找到原发疾病,考虑与喂养不当相关。总胆红素（TBiL）峰值平均为（522.63±120.64）μmol/L,血总胆红素/血白蛋白（B/A）平均为（9.21±2.24）mg/g,白蛋白平均为（33.73±4.29）g/L。完成脑干听觉诱发电位（BAEP）检查71例,正常22例,轻-中度异常8例,重度异常以上41例,异常率为69.0%。完成头颅MRI检查69例,发现ABE典型改变32例（46.4%）。接受机械通气21例,光疗92例,换血84例,静脉应用丙种免疫球蛋白57例,静脉输注白蛋白76例,酶诱导剂36例。死亡21例。存活71例中遗留听力及脑瘫等严重神经功能障碍39例（54.9%）。死亡组TBiL峰值平均为（568.04±103.63）μmol/L,与存活组(511.28±123.36）μmol/L比较差异无统计学意义（P>0.05）。结论　新生儿ABE易遗留听力损伤。光疗无论是否和换血同时进行,持续时间和强度都是必要的。     

Seroepidemiology of toxoplasmosis in pregnant women and neonatal infants

A serological screening among pregnant females for toxoplasmosis in the Belgorod Region has shown a rise in the incidence of this infection in them during seven years (1996-2002). There is also a more than 2-fold increase in the rate of Toxoplasma infection by the second trimester. This increases a risk for fetal congenital malformations and the severity of neonatal congenital malformations. Different awareness levels are shown in fertile-age females from different administrative areas of the region.