新生儿先天性梅毒诊断准确性的回顾性分析

目的探讨制定统一先天性梅毒(CS)诊断标准的必要性。方法回顾分析2016年1月—2020年2月因母亲梅毒感染而入院的85例新生儿的临床资料,分别采用性传播疾病管理指南(2015)/2015美国儿科学会感染委员会报告(美国指南)、2014年欧洲梅毒管理指南(欧洲指南)和2015年版预防艾滋病、梅毒和乙肝母婴传播工作实施方案(中国方案)进行评估诊断,比较上述三种标准诊断CS的一致性。结果将确诊/高度疑似或疑似病例归入CS,美国指南共诊断32例,欧洲指南诊断40例,中国方案诊断15例,三个标准共诊断40例,其中三个标准均符合者14例。美国指南和中国方案诊断病例均包含于欧洲指南诊断病例,美国指南和中国方案诊断病例重复例数为14例。10例梅毒螺旋体(Tp)试验阳性+长骨X线异常患儿中,7例因母亲孕前或孕期接受规范治疗未被美国指南诊断,其余仅3例因母孕期不规范治疗而被美国指南诊断,10例均未被中国方案诊断。Tp试验阳性+Tp-IgM阳性1例未被美国指南诊断,但均被其他两个标准所诊断。Tp试验阳性+其他指标阴性且母孕期不规范治疗15例未被中国方案诊断,但均被其他两个标准诊断。美国指南与欧洲指南、美国指南和中国方案一致性较强,加权κ值分别为0.611和0.705,而欧洲指南与中国方案一致性一般,加权κ值仅为0.304。结论临床上制定统一的CS诊断标准非常必要。     

新生儿梅毒螺旋体IgM抗体检测的方法研究与临床应用

目的研究新生儿梅毒螺旋体(TP)-IgM抗体的检测方法,及其在先天性梅毒早期诊断中的价值。方法采用甲苯胺红不加热血清试验(TRUST)和TP酶联免疫吸附试验(TP-ELISA)检测梅毒新生儿及其母亲血清TP-IgM抗体,同时检测血清TRUST滴度。以德国欧蒙试剂为对照,评估去除血清中IgG抗体和IgG型类风湿因子(IgG/RF)、酶标抗IgM抗体和酶标TP抗原等方法对IgM抗体检测效能的影响。结果 108例梅毒新生儿及其母亲TP-ELISA阳性率差异无统计学意义(P>0.05),梅毒母亲的TRUST阳性率显著高于其新生儿(P<0.05),而且梅毒母亲TRUST抗体滴度普遍高于新生儿(P<0.05)。与对照方法相比,IgM抗体检测时去除血清中IgG抗体可获得更好的相关性和一致性(Kappa>0.75),而使用酶标抗人IgM抗体(间接法,Kappa=0.956)比使用酶标TP抗原(双抗原夹心法,Kappa=0.817)的检测结果与对照方法更相近。结论 TRUST和TP-ELISA检测对新生儿先天性梅毒的早期诊断意义较小,而TP-IgM抗体检测方法在先天性梅毒的早期诊断中有较好应用前景。     

先天性梅毒骨骼改变的X线表现:附11例报告

为了分析先天性梅毒骨骼改变的X线征象，以利于梅毒的早期诊断，将经血清学检查确诊的11例先天性梅毒，结合临床症状分析其骨骼改变的X线表现。结果：①长骨干骺端致密改变，致密线下方出现横形透亮带，长骨干骺端锯齿状改变，局限性骨质缺损；②骨膜反应；③病理性骨折。提示：X线平片能反映骨骼尤其是长骨的改变（包括干骺端炎、骨膜炎、病理性骨折等），结合临床表现，有助于及时发现先天性梅毒。     

Outcomes of herniotomy in premature infants: Recent 10 year experience

Background: The timing of herniotomy in premature infants is controversial. Methods: Outcomes of herniotomy in 47 premature infants admitted to the neonatal intensive care unit (NICU) were retrospectively reviewed for preoperative clinical features, respiratory interventions, and anesthetic and surgical complications. The data were compared with those of full‐term infants (n= 52). Fourteen of the premature infants underwent herniotomy before NICU discharge and 33 after discharge. The predictive factors for anesthetic and surgical complications were also investigated via multiple regression analysis. Results: Mean post‐conceptional age at surgery in premature infants and full‐term infants was 47 weeks and 50 weeks, respectively. Mean bodyweight at surgery in those infants was 4087 g and 5454 g, respectively. The rate of incarcerated hernia and emergency surgery was lower in premature infants. Delayed extubation of the tracheal tube after surgery was noted in four premature infants, but not in full‐term infants. Two cases of cryptorchidism in premature infants and one recurrence in a full‐term infant that required reoperation were noted. On multiple regression analysis no factor (including respiratory interventions) was found to be capable of predicting complications. Conclusion: Although no predictive factor for complications was identified, there were some anesthetic and surgical complications in premature infants. If there is no risk of incarceration, herniotomy in premature infants should be performed at a time when the risk of anesthetic complications is decreased. If there is a risk of incarcerated hernia, herniotomy should be performed carefully in order to avoid occurrence of anesthetic and surgical complications.     

Incidence of death from congenital toxoplasmosis in 0–4‐year‐old children in Japan

Congenital toxoplasmosis is caused by Toxoplasma gondii. The incidence of death due to congenital toxoplasmosis in Japan from 1974 to 2007 was calculated using the autopsy database of the Japanese Society of Pathology and vital statistics from the Ministry of Health, Labour and Welfare. Two neonatal deaths due to congenital toxoplasmosis were reported during that time. As there were 161 195 neonatal deaths during this period and 32 465 autopsies were performed, the yearly neonatal death from congenital toxoplasmosis was calculated as 2 × 161 195/32 465/34 = 0.29 and the autopsy rate as 32 465/161 195 = 0.2014 (20.14%). The calculated number of annual deaths in infants was 0.82 and in children aged 1–4 years it was 2.09; thus, although few, deaths from congenital toxoplasmosis do still occur in neonates, infants, and young children. Therefore, obstetricians and pediatricians should be aware of the potential for congenital toxoplasmosis, and pregnant women should make every effort to avoid T. gondii infection.     

Outcome of fetal echocardiography: A 17 year single‐institution experience in Japan

Background: The aim of this retrospective study was to evaluate the influence of prenatal diagnosis on perinatal outcomes of congenital heart disease (CHD) over a 17 year period at a single center. Methods: The perinatal outcome of CHD in 146 patients diagnosed on fetal echocardiography between 1994 and 2010 were reviewed. The characteristics of 193 neonatal inpatients with CHD treated at the authors’ department between 2001 and 2010 were also analyzed; among the inpatients, 61 were diagnosed before birth (prenatal group) and 132 were diagnosed after birth (postnatal group). Results: Among the 146 patients prenatally diagnosed with CHD, the prenatal mortality, including abortion and stillbirth, decreased from 1994 to 2010. Among the 193 neonatal inpatients, the prenatal group had lower gestational age and bodyweight than the postnatal group. Further, the prenatal group had lower blood pH at admission, but no patient in that group experienced ductal shock, although six patients in the postnatal group did. The average dose of prostaglandin E1 used in duct‐dependent CHD was significantly lower in the prenatal group than in the postnatal group (3.4 vs. 4.6 ng/kg per min; P = 0.015). Conclusions: Prenatal diagnosis of CHD enables planned labor, prevents ductal shock, and reduces prostaglandin E1 side‐effects and medical expenditure.     

A Retrospective Analysis of the Growth Pattern in Patients with Salt-wasting 21-Hydroxylase Deficiency

The objective of this study was to investigate the growth pattern of children with the salt-wasting form of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency (21-OHD). We reviewed the medical records of 13 patients in whom salt-wasting 21-OHD was diagnosed during the first 2 mo of life at our hospital from 1980 through 2008. Six reached adult height. Growth patterns, bone age, biochemical data, and the hydrocortisone dose at each growth stage were analyzed retrospectively. The mean adult height was 155.1 ± 6.5 cm (mean ± SD) in females and 158.1 ± 7.1 cm in males. Although length at birth was normal or longer than the national mean in almost all patients, the mean height SD score of both boys and girls decreased to below 0 SD during infancy. Subsequently, both boys and girls transiently showed growth acceleration and reached their peak growth velocity at 3–10 yr of age. In conclusion, in addition to suppression of growth during infancy, there was inappropriate growth acceleration during childhood. Especially from 3 mo to 3 yr of age, decreasing the hydrocortisone dose in patients who exhibit slower growth may lead to satisfactory height outcomes. Also, strict adjustment of the hydrocortisone dose to avoid accelerated growth from childhood to adolescence might improve adult height outcomes of patients with 21-OHD.     

Excess of congenital abnormalities in French-Canadian children with neuroblastoma: A case series study from Montreal

Neuroblastoma is one of the most common cancers of childhood. Some studies have shown an excess of congenital abnormalities in children who have been diagnosed with neuroblastoma. In this study we examined the medical records of all children with neuroblastoma seen at St. Justine Children's Hospital between the years 1977 and 1993. A total of 141 children (131 of French-Canadian ancestry) were included in this study. Twelve children (8.5%) had 21 defined congenital abnormalities (1,490 per 10,000 children). This compared with a rate of 444.3 children with abnormalities per 10,000 live births (4.44%) for all congenital abnormalities in the British Columbia Health Surveillance Registry, 1979–1988 (relative risk 1.91, P = 0.03). Six of the 12 children had cardiovascular malformations. These and previous results suggest that there may be a common developmental origin to neuroblastoma and to some congenital malformations. Genes that control development may be worthy of further study in these children. Med. Pediatr. Oncol. 29:272–279, 1997. © 1997 Wiley-Liss, Inc.     

Candida species bloodstream infections in hospitalised children: A 10‐year experience

In a 10‐year retrospective study we assessed the epidemiology of candidemia and the association between the presence and removal of indwelling central venous catheters, antifungal use and clinical outcomes among hospitalised children. Demographic and clinical information were retrieved from the electronic medical records. One hundred six episodes of candidemia were identified in 83 unique patients. Candida parapsilosis was the most prevalent (52%) species, followed by C. albicans (25%). Non‐oncologic children receiving fluconazole within 30 days of developing candidemia were most likely to develop C. parapsilosis infection (40%, P = 0.006), independent of total parenteral nutrition (odds ratio (OR) 2.5, 95% confidence interval (CI): 0.6–11, P = 0.3). Crude mortality rate was 12% and significantly higher for children less than 2 years (OR: 6.7, 95% CI: 1.9–23, P = 0.003), and those infected with C. lusitaniae (OR: 9, 95% CI: 1.6–51, P = 0.02). The aggregate use of antifungal agents decreased overtime (χ²: 16.7, P < 0.0001). Fluconazole remained the most common antifungal agent used during the study.     

Neurodevelopment in 1‐year‐old Japanese infants after congenital heart surgery

Background: The purpose of this study was to determine the prevalence of persistent neurodevelopmental sequelae in 1‐year‐old infants after open heart surgery for congenital heart disease, using the Bayley Scales of Infant Development second edition (BSID‐II). A secondary objective was to confirm the applicability and usefulness of the BSID‐II in Japanese infants. Methods: Thirty‐nine infants who underwent repair of a ventricular septal defect before 6 months of age and 108 normal Japanese infants at 1 year of age were assessed using the BSID‐II. Results: In normal infants, scores on the Mental Development Index and the Psychomotor Development Index components of the BSID‐II ranged from borderline retardation to very superior following a normal distribution similar to those obtained for US controls. No problems were encountered, either in translation or in following the instructions when the BSID‐II was used to evaluate the 1‐year‐old Japanese infants. On the other hand, the mean scores on the Mental Development Index and the Psychomotor Development Index were significantly lower in Japanese patients than in normal Japanese infants, particularly for gross motor development (P < 0.001). Linear regression analysis showed that a longer intensive care unit stay was associated with impaired cognitive development at 1 year of age (P= 0.03). Conclusions: Neurodevelopmental functions in 1‐year‐old infants with congenital heart disease were lower than those in normal infants, especially for gross motor function using the BSID‐II. One risk factor that correlated with neurodevelopmental sequelae was the length of intensive care unit stay.