Oesophageal atresia: what has changed in the last 3 decades?

The aim of the study was to analyse the outcomes of children born with oesophageal atresia over the last 3 decades. The records of 104 patients born between 1973 and 1999 were reviewed retrospectively. To evaluate changes over time, the analysis was done for three consecutive time periods: 1973–79, 1980–89, and 1990–99. Mean birth weight was 2553 g (SD 640), and mean gestational age was 39 weeks (SD 4). Forty-two newborns (40%) had one or more associated congenital malformations, and 30% had associated cardiac malformations. There was no change in incidence of associated anomalies over the three time periods studied. Mortality of patients decreased from 33% to 14% ( p =0.048). There was a significant association between the presence of a major cardiac malformation and survival (survival: 88% vs. 57%, p =0.004). Analysing the three different time periods separately reveals that cardiac disease was not a significant risk factor in the first period but became significant in the period from 1980–99 (relative risk: 6.76, 95% CI 1.44–31.77). Birth weight was significantly higher in infants who survived (2626 g, SD 642) compared with those who died (2290, SD 570, p =0.028). This effect, however, is mainly based on the difference during the first period and is lost later. Early and late postoperative complications occurred in 44/102 patients. Strictures developed in 33/91 patients who survived the first month of life (33%). The rate of symptomatic strictures decreased significantly over the three time periods, from 50% to 23% ( p =0.022). In summary, this study shows no significant change in patient characteristics over the last 3 decades, but mortality and postoperative complication rates decreased, and associated cardiac anomalies became the far most important risk factor for mortality.     

Polymorphisms of surfactant protein B encoding gene: modifiers of the course of neonatal respiratory distress syndrome?

Surfactant protein B (SP-B) is a lipophilic protein and plays a major role in lung mechanics. Polymorphisms of surfactant protein A, another component of the surfactant system, have been previously described to be a risk factor for respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD) in preterms. The aim of this prospective study was to determine whether polymorphisms within intron 4 of the SP-B gene are related to the incidence, severity and complications of RDS in Caucasian newborns. In order to identify SP-B intron 4 polymorphisms, we analysed genomic DNA by means of polymerase chain reaction, fragment length and sequence analysis in 140 preterms and 58 healthy term neonates. The frequency of intron 4 variations did not differ between preterms and terms. A total of 111 preterms with the intron 4 wild type (group 1) and 29 preterms carrying the genetic variations (group 2) did not differ in gestational age, gender distribution and birth weight. Compared to group 1, the overall incidence of RDS (75.7% versus 93.1%, P < 0.05), the frequency of severe RDS (28.4% versus 55.2%, P < 0.01) and BPD (21.6% versus 48.3%, P < 0.01) were all higher in group 2. The median duration of oxygen dependency (4 days versus 17 days, P < 0.05) and the need for surfactant administration were also higher in group 2 than in group 1 (43.2% versus 72.4%, P < 0.01). Duration of mechanical ventilation and rate of chronic lung disease at 36 weeks were comparable in both groups. CONCLUSION: we suggest that polymorphisms in intron 4 of the surfactant protein B gene independently modify the course of neonatal respiratory distress syndrome.     

Efficacy of volume-targeted ventilation versus high-frequency oscillatory ventilation in the treatment of neonatal respiratory distress syndrome北大核心CSCD

Objective To study the clinical efficacy of volume-targeted ventilation (VTV) versus high-frequency oscillatory ventilation (HFOV) in the treatment of neonatal respiratory distress syndrome (NRDS). Methods A retrospective cohort analysis was performed on the medical data of 140 neonates with severe NRDS who were admitted from September 2016 to February 2022, with 55 neonates in the VTV group and 85 in the HFOV group. The neonates in the VTV group received conventional mechanical ventilation and target tidal volume, and those in the HFOV group received HFOV. Arterial blood gas parameters were collected at 48 hours after admission, and related indices during hospitalization were recorded, including mortality rate, duration of invasive mechanical ventilation, duration of oxygen therapy, and the incidence rates of complications. Results Compared with the VTV group, the HFOV group had significantly lower incidence rates of grade Ⅲ-Ⅳ periventricular-intraventricular hemorrhage and neonatal necrotizing enterocolitis (P<0.05), and there were no significant differences between the two groups in the duration of invasive mechanical ventilation, the duration of oxygen therapy, mortality rate, and the incidence rates of bronchopulmonary dysplasia, hypocapnia, hypercapnia, periventricular leukomalacia, and retinopathy of prematurity (P>0.05). Conclusions HFOV has a better clinical efficacy than VTV in the treatment of NRDS. © 2022 Xiangya Hospital of CSU. All rights reserved.     

Pattern of acute poisonings in childhood in Ankara: what has changed in twenty years?

Poisoning represents one of the most common medical emergencies in childhood, and epidemiological properties differ from country to country. Thus, special epidemiological surveillance for each country is necessary to determine the problem according to which preventive measures can be taken. The purpose of this study was to clarify the characteristics of acute poisoning cases admitted to a pediatric referral hospital. All poisoned patients under 17 years of age, except for cases food poisoning, presenting to the Emergency Department (ED) from January 1995 to December 2000 were determined. The information about each case was recorded on standardized forms and a retrospective chart review survey was done. Complete epidemiological and clinical data were obtained for 489 patients. The mean age of all poisoned patients (mean +/- standard deviation) was 5.96 +/- 4.87 years, and the age range was 0.01 to 17 years. Three hundred and thirty-one children, forming 63.6% of all patients, were under five years of age. Slightly more boys (52.3%) than girls were intoxicated at ages less than 10 years, after which more girls (79%) than boys were involved. The majority of all cases were due to accidental poisoning (78.1% of all poisonings) which occurred mostly in children under five years of age (73.3%). While accidental poisonings (97.1%) were the most common mode of poisoning between 1-5 years, self-poisonings (67.3%) had the highest ratio in cases over 10 years of age. In patients younger than one year of age, 74.2% of all poisonings were due to therapeutical error. Drugs were the most frequent offending agent (57.7%), followed by ingestion of a caustic/corrosive substance (16.8%) and carbon monoxide (CO) intoxication (9.4%). Analgesics were the most common agents, forming 23.7% of all poisonings due to drugs, followed by ingestion of multiple drugs and tricyclic antidepressants at ratios of 21.6% and 9.6%, respectively. The most common route of poisoning was ingestion of the poison (437/489 patients, 89.4%) and most were ingested inside the house (93.3%). About half of all poisoned patients (50.9%) were admitted to the ED within the first two hours of ingestion, and gastric lavage was performed on about half of the poisoned children (48.7%). In most of the cases, hospital treatment was non-specific, including general measures of decontamination and supportive-symptomatic therapy. During the six-year study period, two patients were lost due to acute poisoning, yielding an overall mortality rate of 0.4%. While most of the poisonings were due to accidental ingestions in infancy and primary school ages without sex predilection, the incidence of self-poisonings, especially in girls, was found to be increased. Analgesics, tricyclic antidepressant drugs (which seemed to form a new and dangerous group) and caustic/corrosive substances were the most commonly ingested agents. The early awareness of poisoning and appropriate therapeutic measures taken seemed to be efficacious with a very low mortality rate. The epidemiological and preventive properties of childhood poisonings should be further searched by prospectively designed multicentered studies throughout our country.     

Does smoking in pregnancy modify the impact of antenatal steroids on neonatal respiratory distress syndrome? Results of the Epipage study

OBJECTIVES: To assess the relation between cigarette smoking during pregnancy and neonatal respiratory distress syndrome (RDS) in very preterm birth, and to analyse the differential effect of antenatal steroids on RDS among smokers and non-smokers. DESIGN: A population based cohort study (the French Epipage study). SETTING: Regionally defined births in France. METHODS: A total of 858 very preterm liveborn singletons (27-32 completed weeks of gestation) of the French Epipage study were included in this analysis. The odds ratio for RDS in relation to smoking in pregnancy was estimated using a logistic regression to control for gestational age. The odds ratio for RDS in relation to antenatal steroids was estimated taking into account an interaction between antenatal steroids and cigarette smoking, using multiple logistic regression to control for gestational age, birthweight ratio, main causes of preterm birth, mode of delivery, and sex. RESULTS: The odds ratio for RDS in relation to smoking in pregnancy adjusted for gestational age (aOR) was 0.59 (95% confidence interval (CI) 0.44 to 0.79). The aOR for RDS in relation to antenatal steroids was 0.31 (95% CI 0.19 to 0.49) in babies born to non-smokers and 0.63 (95% CI 0.38 to 1.05) in those born to smokers; the difference was significant (p = 0.04). CONCLUSIONS: Cigarette smoking during pregnancy is associated with a decrease in the risk of RDS in very preterm babies. Although antenatal steroids reduce the risk of RDS in babies born to both smokers and non-smokers, the reduction is smaller in those born to smokers.     

Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia?

OBJECTIVES: To determine the impact of surfactant replacement on survival, need for extracorporeal membrane oxygenation (ECMO), and chronic lung disease in term infants with prenatally diagnosed congenital diaphragmatic hernia (CDH). STUDY DESIGN: Prenatally diagnosed infants born at > or =37 weeks' gestation with immediate distress at delivery and no other major congenital anomalies, who were enrolled in the CDH Registry, were analyzed. For univariate analysis, chi 2 tests were used for categoric variables and unpaired t tests for nominal variables. Multiple logistic regression was used to calculate adjusted odds ratios. RESULTS: Eligible infants (n = 522) were identified. Demographic variables were similar between the surfactant-treated (n = 192) and nonsurfactant-treated (n = 330) groups, with the exception of race (white, 88.0% vs 71.2%; P =.0007). The use of ECMO and incidence of chronic lung disease were higher (59.8 vs 50.6, P =.04; 59.9 vs 47.6, P =.0066) and survival lower in the surfactant-treated cohort (57.3 vs 70.0, P =.0033). Adjusted logistic regression for use of ECMO, survival, and chronic lung disease resulted in odds ratios inconsistent with an improved outcome associated with surfactant use. CONCLUSIONS: This analysis shows no benefit associated with surfactant therapy for term infants with a prenatal diagnosis of isolated CDH.     

Specific reading disability (dyslexia): what have we learned in the past four decades?

We summarize some of the most important findings from research evaluating the hypothesized causes of specific reading disability ('dyslexia') over the past four decades. After outlining components of reading ability, we discuss manifest causes of reading difficulties, in terms of deficiencies in component reading skills that might lead to such difficulties. The evidence suggests that inadequate facility in word identification due, in most cases, to more basic deficits in alphabetic coding is the basic cause of difficulties in learning to read. We next discuss hypothesized deficiencies in reading-related cognitive abilities as underlying causes of deficiencies in component reading skills. The evidence in these areas suggests that, in most cases, phonological skills deficiencies associated with phonological coding deficits are the probable causes of the disorder rather than visual, semantic, or syntactic deficits, although reading difficulties in some children may be associated with general language deficits. Hypothesized deficits in general learning abilities (e.g., attention, association learning, cross-modal transfer etc.) and low-level sensory deficits have weak validity as causal factors in specific reading disability. These inferences are, by and large, supported by research evaluating the biological foundations of dyslexia. Finally, evidence is presented in support of the idea that many poor readers are impaired because of inadequate instruction or other experiential factors. This does not mean that biological factors are not relevant, because the brain and environment interact to produce the neural networks that support reading acquisition. We conclude with a discussion of the clinical implications of the research findings, focusing on the need for enhanced instruction.     

The current status of palliative care in Cyprus: has it improved in the last years?

The oncology and palliative care services in Cyprus were reviewed looking especially on changes that occurred recently. Although there are encouraging signs in the management of palliative care patients, there is still room for improvement. Especially efforts should be made to improve communication between hospitals and palliative care organizations. The Ministry of Health should also take the initiative to get all palliative care organizations to work together under an official body and integrate palliative care into mainstream health service provision and education.     

Spinal muscular atrophy type 1: what are the ethics and practicality of respiratory support?

Spinal Muscular Atrophy Type I (SMA I) is the most severe form of SMA. It presents in infancy and without treatment death occurs by 2 years. Treatments in use address respiratory and nutritional issues but even with aggressive treatment death is still likely in childhood. Thus their use is not obligatory. However, pediatric respirologists must be willing and comfortable at presenting all treatment options, including the option of palliative care, to families and then supporting the family's choice. Whatever the chosen treatment regimen, decision making is difficult for families. Support and help must be provided from the time of presentation till death by a knowledgeable and compassionate team.