Outcomes of oesophageal atresia and tracheo‐oesophageal fistula repair

Oesophageal atresia and tracheo‐oesophageal fistula are congenital anomalies of the oesophagus requiring surgical repair in infancy, either by open or thoracoscopic approach. Although mortality rates associated with this procedure are low, children may go on to have complications throughout childhood and into adulthood, most commonly related to ongoing gastrointestinal and respiratory symptoms. This review outlines the early, mid and long‐term outcomes for these children in terms of quality of life and incidence of symptoms.     

Congenital anomalies in patients with choanal atresia: CHARGE-association

Six patients with both choanal atresia (ChA) and additional malformations are described and another 110 cases with this combination reviewed from the literature. Our study of these cases supports the existence of the CHARGE-association (Coloboma, Heart Disease, Atresia of choanae, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness). Our findings suggest the inclusion of orofacial clefts and oesophageal atresia among the main symptoms of this association. A certain degree of facial dysmorphism (low set, dysplastic ears, retrogenia, antimongoloid slant of palpebral fissures and anteverted nares) was observed in each of our cases. Infants with the bilateral type of ChA plus cardiac defects and those with ChA plus renal malformations have a high mortality rate. The aetiology of the association is not clear. The recurrence risk may be low.Abbreviations ChA Choanal atresia - MCA Multiple congenital abnormalities - OFC Occipito-frontal head circumference     

Antenatal care in developing countries: the need for a tailored model

In developed countries, 98% of all women receive prenatal care and 94% give birth under the supervision of skilled healthcare practitioners with timely access to appropriate emergency treatment if complications arise. In contrast, large numbers of pregnant women in Africa and Asia do not receive adequate prenatal care and lack skilled attendance at birth. In developing countries quality of prenatal care is often scarce: models of care adopted in the western world and exported to the developing world have not been monitored early enough to discover their weak points promptly. This blind attitude has transformed antenatal care into an empty and useless ritual, and explains why antenatal care programmes continue to be unsuccessful, being inappropriate to the specific situation. A mix of educational and cultural factors together with persistent lack of resources in a global critical situation all contribute to the poor results of antenatal care programmes. Antenatal care services should be free of charge, planned and implemented within the community, cost-effective, and should yield evidence-based quality care. They should also include information for the patient and family members, provide affordable treatment of existing conditions, and warrant referral for complications.     

Delayed umbilical cord clamping for reducing anaemia in low birthweight infants: implications for developing countries

BACKGROUND: Cheap and effective interventions are needed to reduce the risk of infant anaemia in developing countries. Delayed cord clamping (DCC) has been shown to be a simple, safe and cost-free delivery procedure that augments red cell mass in appropriate-for-gestational-age term and preterm infants. It is not known, however, whether DCC is similarly safe and effective in small-for-gestational-age (SGA) infants. We analysed the available evidence to generate a balanced inference on the use of DCC in developing countries. OBJECTIVES: To examine the short- and long-term effects in SGA infants of DCC compared with immediate clamping, and to assess the relationship between time of clamping and the potential postnatal haematological complications of DCC in SGA infants. SEARCH STRATEGY: PubMed (1966 to January 2006), EMBASE (1988 to January 2006) and The Cochrane Library (Issue 1, 2006) were searched. Reference lists of published trials were examined and major journals of perinatal and tropical medicine were hand-searched. SELECTION CRITERIA: Randomised and quasi-randomised trials comparing delayed with immediate cord clamping in infants born between 30 and 42 completed weeks of gestation and which included a proportion of SGA infants. DATA COLLECTION AND ANALYSIS: Three reviewers assessed eligibility and trial quality. MAIN RESULTS: To date, no trials have specifically reported the effects of DCC in SGA infants. Three trials were included, of 190 term and 40 preterm infants, a proportion of whom were SGA. DCC was associated with higher haemoglobin levels in term infants at follow-up [two trials, 127 infants, weighted mean difference (WMD) 9.17 g/L, 95% confidence interval (CI) 5.94-12.40]. In preterm infants, the proportion who required a blood transfusion in the 1st 6 weeks after birth was lower after DCC (one trial, 38 infants, RR 0.56, 95% CI 0.34-0.94). It was not possible to infer from the available data whether SGA infants were at greater risk of adverse effects in the early neonatal period. CONCLUSIONS: DCC in a group that contains both AGA and SGA infants was associated with higher haemoglobin levels at 2-3 months of age in term infants and a reduction in the number of blood transfusions needed in the 1st 4- 6 weeks of life in preterm infants. No reliable conclusions could be drawn about the potential adverse effects of DCC. The paucity of information on DCC in SGA infants justifies further research, especially in developing countries where the baseline risk for polycythaemia-hyperviscosity syndrome is likely to be lower than in industrialised countries.     

先天性食管闭锁合并简单先天性心脏病新生儿预后危险因素分析

目的探讨先天性食管闭锁(CEA)合并简单先天性心脏病(CHD)新生儿预后不良的危险因素。方法回顾性分析1998年—2013年收治并手术的CEA合并简单CHD患儿的临床资料,包括一般情况、实验室检查、术后并发症等,并比较存活和死亡患儿的临床资料。结果 75例患儿纳入最终研究,存活67例,死亡8例,病死率10.67%。死亡及存活患儿的出生胎龄,出生体质量,入院日龄,手术日龄,手术持续时间,手术前、手术当天、痊愈出院/死亡前最近的一次血常规、肝肾功能、电解质、血气分析的差异均无统计学意义(P均0.05);死亡患儿的呼吸衰竭和心力衰竭的发生比例均高于存活患儿,差异有统计学意义(75%、9.0%,P=0.000;50%、1.5%,P=0.000)。结论 CEA合并简单CDH患儿死亡可能与并发呼吸衰竭和心力衰竭有关。     

Combined protocol for severe and moderate acute malnutrition in emergencies: Stakeholder perspectives in four countries

Each year, acute malnutrition affects an estimated 52 million children under 5 years of age. Current global treatment protocols divide treatment of severe acute malnutrition (SAM) and moderate acute malnutrition (MAM) despite malnutrition being a spectrum disease. A proposed Combined Protocol provides for (a) treatment of MAM and SAM at the same location; (b) diagnosis using middle‐upper‐arm circumference (MUAC) and oedema only; (c) treatment using a single product, ready‐to‐use‐therapeutic food (RUTF), and (d) a simplified dosage schedule for RUTF. This study examines stakeholders' knowledge of and opinions on the Combined Protocol in Niger, Nigeria, Somalia, and South Sudan. Data collection included a document review followed by in‐depth interviews with 50 respondents from government, implementing partners, and multilateral agencies, plus 11 global and regional stakeholders. Data were analysed iteratively using thematic content analysis. We find that acute malnutrition protocols in these countries have not been substantially modified to include components of the Combined Protocol, although aspects were accepted for use in emergencies. Respondents generally agreed that MAM and SAM treatment should be provided in the same location, however they said MUAC and oedema‐only diagnosis, although more field‐ready than other diagnostic measures, did not necessarily catch all malnourished children and may not be appropriate for “tall and slim” morphologies. Similarly, using only RUTF presented inherent logistical advantages, but respondents worried about pipeline issues. Respondents did not express strong opinions about simplified dosage schedules. Stakeholders interviewed indicated more evidence is needed on the operational implications and effectiveness of the Combined Protocol in different contexts.     

Laparoscopic management of pediatric choledochal cysts in developing countries: review of ten cases

We report laparoscopic management of choledochal cysts (CDC) in 10 children. We dissect the CDC using conventional mono- and bi-polar diathermy up to the lower end, ligate or clip it at the lowest possible level and divide it. The proximal end is divided after leaving a sufficient cuff for anastomosis. In the first three cases, we formally opened to complete the biliary-enteric anastomosis. However, in the subsequent seven cases, we made a small midline incision to develop a Roux-en Y loop, and the anastomosis was then completed by intra-corporeal suturing after re-creating the pneumo-peritoneum. Apart from biliary leak in one case, we did not encounter any major complications. The mean operative time was 4.2 h. We have discussed the technical points in the study. We conclude that CDC is eminently suitable for laparoscopic correction; it requires advanced skills and expertise for precise dissection and meticulous suturing in restricted spaces. If the case selection is good and if the team is experienced, CDC can be effectively managed using laparoscopy even without a sophisticated equipment. The wound- and scar-related morbidity is minimized.     

Latex agglutination: an appropriate technology for the diagnosis of bacterial meningitis in developing countries

We evaluated prospectively the utility of a latex agglutination technique for the diagnosis of Haemophilus influenzae type b meningitis in a paediatric ward in India. Eight of 44 children had H. influenzae grown from cerebrospinal fluid. These proven cases plus four additional cases of H. influenzae meningitis were detected by the latex agglutination test. There were no cross reactions with other organisms. The high degree of sensitivity and specificity, combined with the speed and simplicity of this technique make it an appropriate method for developing countries.     

Barriers to neonatal care in developing countries: Parents' and providers' perceptions

Aim: Hospital care and advanced medical technologies for sick neonates are increasingly available, but not always readily accessible, in many countries. We characterised parents' and providers' perceptions of barriers to neonatal care in developing countries. Methods: We interviewed parents whose infant was hospitalised within the first month of life in Cambodia, Malaysia, Laos and Vietnam, asking about perceived barriers to obtaining newborn care. We also surveyed health-care providers about perceived barriers to providing care. Results: We interviewed 198 parents and 212 newborn care providers (physicians, nurses, midwives, paediatric and nursing trainees). Most families paid all costs of newborn care, which they reported as a hardship. Although newborn care is accessible, 39% reported that hospitals are too distant; almost 20% did not know where to obtain care. Parents cited lack of cleanliness (46%), poor availability of medications (42%) or services (36%), staff friendliness (42%), poor infant outcome (45%), poor communications with staff (44%) and costs of care (34%) as significant problems during prior newborn care. Providers cited lack of equipment (74%), lack of staff training (61%) and poor infrastructure (51%) as barriers to providing neonatal care. Providers identified distance to hospital, lack of transportation, care costs and low parental education as barriers for families. Conclusions: Improving cleanliness, staff friendliness and communication with parents may diminish some barriers to neonatal care in developing countries. Costs of newborn care, hospital infrastructure, distance to hospital, staffing shortages, limited staff training and limited access to medications pose more difficult barriers to remedy.     

Fetal and post-natal diagnosis of major congenital heart disease: implications for medical and psychological care in the current era

Aim: The fetal or post‐natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences. Methods: We reviewed infants who underwent cardiac surgery in the first year of life at the Heart Centre for Children, The Children's Hospital at Westmead during 2009. The aims of this study were to: (i) examine the key features of cardiac diagnosis and clinical outcome, and (ii) consider how these data can inform priorities for the delivery of clinical services. Results: Over the 12‐month study period, a first cardiac surgical procedure was performed on 195 infants, with 85 infants (44%) diagnosed in the antenatal period. Of the total sample, a subset of 90 babies (46%) underwent their first procedure in the neonatal period, with 62% having had a fetal diagnosis. Major intracardiac lesions including truncus arteriosus (100%), single ventricular lesions (83%), pulmonary atresia with ventricular septal defect (78%) and transposition of the great arteries (53%) were diagnosed prior to birth. Improved haemodynamic stability at initial presentation was found in those with a fetal diagnosis. The overall mortality rate for all patients was 6.1% at 12 months, with a higher mortality in infants with single ventricle. Conclusions: The contemporary paradigm of care for infants with major congenital heart disease requires a multidisciplinary approach to care, with improvements in clinician–clinician and clinician–family communication, and psychological support and education for families. Changes in the allocation of resources are required to meet this model of best practice.     

Transgenic Mice in the Search for Development-related Genes: Application for the Studies of Congenital Anomalies*

Transgenic mice technology in conjunction with other molecular biological and embryo manipulation techniques has made it possible to manipulate the mouse genome to elucidate the molecular mechanisms of normal and abnormal embryonic development. This genetic technology also permits the isolation of novel genes involved in developmental events. It has and certainly will greatly contribute to the better understanding of the pathogenesis of congenital anomalies. However, the majority of congenital anomalies in humans occur from multifactorial causes including not only genetic but also various environmental factors. Thus, transgenic mice can be utilized as an excellent genetic tool on which all the available conventional teratological techniques and informations are concentrated to elucidate molecular mechanisms and the holistic picture of congenital anomaly formation.     

Monitoring breastfeeding indicators in high‐income countries: Levels,trends and challenges

Monitoring indicators of breastfeeding practices is important to protect and evaluate the progress of breastfeeding promotion efforts. However, high‐income countries lack standardized methodology to monitor their indicators. We aimed to update and summarize nationally representative annual estimates of breastfeeding indicators in high‐income countries and to describe methodological issues pertaining to the data sources used. A review was conducted through population‐based surveys with nationally representative samples or health reports from nationally representative administrative data of electronic surveys or medical records. Methodological aspects and rates of all breastfeeding indicators available were summarized by country. The median and annual growth of breastfeeding in percentage points within countries with time‐series data were estimated. Data from 51 out of 82 high‐income countries were identified. The data were obtained through surveys (n = 32) or administrative data (n = 19). Seventy‐one percent of countries have updated their indicators since 2015. Ever breastfed was the indicator most frequently reported (n = 46), with a median of 91%. By 6 months of age, the median equals 18% for exclusive and 45% for any breastfeeding. At 12 months, the median of continued breastfeeding decreased to 29%. The annual growth rate for ever breastfed, exclusive and any breastfeeding at 6 months and continued at 12 months varied from 1.5 to −2.0, 3.5 to −3.1, 5.0 to −1.0 and 5.0 to −1.9, respectively, with positive changes for most countries. Stronger interventions are needed to promote breastfeeding in high‐income countries as a whole, and investments are required to monitor trends with standardized methodologies.     

Transition Care: Future Directions in Education,Health Policy,and Outcomes Research

All youth must transition from pediatric to adult-centered medical care. This process is especially difficult for youth with special health care needs. Many youth do not receive the age-appropriate medical care they need and are at risk during this vulnerable time. Previous research has identified barriers that may prevent effective transition, and protocols have been developed to improve the process. Health outcomes related to successful transition have yet to be fully defined.Health care transition can also be influenced by education of providers, but there are gaps in medical education at the undergraduate, graduate, and postgraduate levels. Current changes in federal health policy allow improved health care coverage, provide some new financial incentives, and test new structures for transitional care, including the evolution of accountable care organizations (ACO). Future work must test how these systems changes will affect quality of care. Finally, transition protocols exist in various medical subspecialties; however, national survey results show no improvement in transition readiness, and there are no consistent measures of what constitutes transition success.In order to advance the field of transition, research must be done to integrate transition curricula at the undergraduate, graduate, and postgraduate levels; to provide advance financial incentives and pilot the ACO model in centers providing care to youth during transition; to define outcome measures of importance to transition; and to study the effectiveness of current transition tools on improving these outcomes.     

Patterns in child stunting by age: A cross-sectional study of 94 low- and middle-income countries

Child stunting prevalence is primarily used as an indicator of impeded physical growth due to undernutrition and infections, which also increases the risk of mortality, morbidity and cognitive problems, particularly when occurring during the 1000 days from conception to age 2 years. This paper estimated the relationship between stunting prevalence and age for children 0–59 months old in 94 low- and middle-income countries. The overall stunting prevalence was 32%. We found higher stunting prevalence among older children until around 28 months of age—presumably from longer exposure times and accumulation of adverse exposures to undernutrition and infections. In most countries, the stunting prevalence was lower for older children after around 28 months—presumably mostly due to further adverse exposures being less detrimental for older children, and catch-up growth. The age for which stunting prevalence was the highest was fairly consistent across countries. Stunting prevalence and gradient of the rise in stunting prevalence by age varied across world regions, countries, living standards and sex. Poorer countries and households had a higher prevalence at all ages and a sharper positive age gradient before age 2. Boys had higher stunting prevalence but had peak stunting prevalence at lower ages than girls. Stunting prevalence was similar for boys and girls after around age 45 months. These results suggest that programmes to prevent undernutrition and infections should focus on younger children to optimise impact in reducing stunting prevalence. Importantly, however, since some catch-up growth may be achieved after age 2, screening around this time can be beneficial.     

Vitamin A for acute respiratory infection in developing countries: a meta-analysis

Aim: To determine the efficacy of intervention with high-dose vitamin A as an adjunct to standard treatment on outcome in acute lower respiratory tract infection in children in developing countries. Methods: A systematic review of double-blinded, randomized, controlled intervention studies of high-dose vitamin A or placebo in children aged between 1 mo and 6 y presenting with acute non-measles lower respiratory tract infection. Results: Five studies fulfilling the criteria were identified and included a total of 2177 children (1067 intervention, 1110 control). The main outcome measures were time to normalization of fever, respiratory rate and oxygen dependence, time to discharge, and mortality. On meta-analysis, there were no significant differences in any of the recovery measures or mortality between the intervention and control groups. Pooled results for recovery times are given showing difference in days to recovery days and 95% confidence intervals. Positive summary measures indicate faster recovery in the vitamin A group and negative in the placebo: fever: 0.03 (-0.10 to 0.17); oxygen requirement: -0.08 (-0.31 to 0.16); raised respiratory rate: -0.09 (-0.38 to 0.19); hospital stay: -0.06 (-0.52 to 0.40). Mortality was below 2% in both groups, with a non-significantly higher risk in the intervention group (odds ratio 1.16, 95% CI: 0.61-2.21).

Conclusion: There is no evidence from this meta-analysis that intervention with high-dose vitamin A improves recovery from pneumonia in children in developing countries aged from 1 mo to 6 y.     

Low levels of high density lipoprotein cholesterol in Turkish children: An important risk factor

In Turkish adults, the incidence of coronary artery disease (CAD) has been found to be high. However, no detailed lipid, or lipoprotein data of children are available from Turkey. The present study was designed to define the borderline lipid and lipoprotein levels of sera in 397 healthy children (aged5–14 years; 206 boys and 191 girls). Mean levels of total cholesterol (TC), triglyceride (TG), high- and low-density lipoprotein cholesterol (HDL-C and LDL-C, respectively) were found to be 150, 79, 46.7, and 87.6 mg/dL, respectively, for boys, and 152, 77.5, 46.3 and 90.5 mg/dL, respectively, for girls. Lipids and lipoproteins did not show any significant correlation with age and body mass index (BMI), except for TG in boys in whom TG levels were positively correlated with age and BMI. There were no significant differences in lipid and lipoprotein levels between boys and girls. As in the Turkish adult population, serum HDL-C levels of Turkish children were profoundly low on international comparison. Twenty-three (53%) of 43 children with low HDL-C level ( 35 mg/dL) had abnormal ratios of TC/HDL-C ( 5) and/or LDL-C/HDL-C ( 4.5), whereas only 13 (3.7%) of the remaining 354 children with a HDL-C level less than 35 mg/dL had abnormal ratios of TC/HDL-C (5) and/or LDL-C/HDL-C ( 4.5). The low levels of HDL-C in Turkish children may be associated with the high incidence of CAD in the Turkish adult population.     

Cystogastrostomy: a valid option for treating pancreatic pseudocysts of children in developing countries

Twelve children with pancreatic pseudocysts were managed for over 10 years at our institute that is a tertiary referral center of our country. A majority of them had posttraumatic pancreatic pseudocysts. Six of them were early referrals and presented within 1–2 weeks of pancreatic injury while the remaining six were referred later than 6 weeks with thick cyst walls. An initial conservative management and observation (with serial ultrasounds) led to a resolution of the pseudocysts in three patients (25% resolution rate). All the remaining subjects were treated using surgical modality (cystogastrostomy). In all the subjects where cystogastrostomy was done, the pseudocysts resolved completely, except in one child, who required the procedure to be repeated. The authors encountered no complications of the pancreatic pseudocyst disease in children i.e. infection, rupture, etc, that have been frequently described for adults. The authors conclude that pancreatic pseudocyst is a comparatively benign entity in children with a better outcome than in adults. Though various sophisticated treatment modalities are in vogue in the developed countries for managing pancreatic pseudocysts in children, cystogastrostomy is still a valid option for this purpose in the developing countries with suboptimal infrastructure and gives good results.