Previous studies suggest that high airway pressuremaycompromise cardiac output. Weinvestigated theeffect of 3 nasal continuous positive airway pressure levels on cardiac output in preterm infants with evolving chronic lung disease. We found that brief changes in continuous positive airway pressure did not affect cardiac output.

J Pediatr. 2015; Vol. 166 (2): 477–9.

Objective. We performed a randomised trial in very preterm, small for gestational age (SGA) babies to determine if prophylaxis with granulocyte macrophage colony stimulating factor (GM-CSF) improves outcomes (the PROGRAMS trial). GM-CSF was associated with improved neonatal neutrophil counts, but no change in other neonatal or 2-year outcomes. As subtle benefits in outcome may not be ascertainable until school age we performed an outcome study at 5 years.

Patients and methods. 280 babies born at 31 weeks of gestation or less and SGA were entered into the trial. Outcomes were assessed at 5 years to determine neurodevelopmental and general health status and educational attainment.

Results. We found no significant differences in cognitive, general health or educational outcomes between 83 of 106 (78%) surviving children in the GMCSF arm compared with 81 of 110 (74%) in the control arm. Mean mental processing composite (equivalent to IQ) at 5 years were 94 (SD 16) compared with 95 (SD 15), respectively [difference in means -1 (95% CI -6 to 4)], and similar proportions were in receipt of special educational needs support [41% vs 35%; risk ratio 1.2 (95% CI 0.8 to 1.9)]. Performance on Kaufmann-ABC subscales and components of NEPSY were similar. The suggestion of worse respiratory outcomes in the GM-CSF group at 2 years was replicated at 5 years.

Conclusions. The administration of GM-CSF to very preterm SGA babies is not associated with improved or more adverse neurodevelopmental, general health or educational outcomes at 5 years.

Trial registration number ISRCT N 42553489. Arch Dis Child Fetal Neonatal Ed. 2015; 0: F1–F7. doi:10.1136/archdischild-2014-307410

Asphyxiating thoracic dystrophy (Jeune syndrome) is a rare genetically heterogeneous autosomal recessive disease, characterized by the development of generalized osteochondrodysplasia, anomalies of skeletal system with multi-organ involvement. This syndrome is based on mutations of the IFT80, DYNC2H1, WDR19, TTC21B genes, localized in the locus of chromosomes 3q25.33, 11q22.3, 4p14, 2q24.3 resspectively and mutation in the locus of chromosome 15q13 too. These genes play a role in function of cilia in the mammalian body. The article presents modern literature findings on genetics, pathogenesis, its clinical, X-ray presentations and CT patterns, diagnosis and treatment of this rare disease. The article also presents the first Russian case series including 7 patients with this disease. Combinations of such symptoms as narrow bell-shaped thorax, variable limb shortness, respiratory failure, including oxygen dependence, recurrent respiratory infections, motor development delay in observed patients helped to diagnose Jeune syndrome at all cases.

Esophageal atresia (EA) and/or tracheo-esophageal (TEF) fistula are relatively common malformations occurring in approximately 1 in 3500 births. Data from twin and family studies suggest that genetic factors do not play a major role, and yet there are well-defined instances of this malformation where genetic factors clearly are important. This is highlighted by the recent identification of no fewer than three separate genes with a role in the etiology of esophageal atresia: those for Feingold syndrome (N-MYC), anophthalmia-esophageal-genital (AEG) syndrome (SOX2), and CHARGE syndrome (CHD7).

Purpose. Consider EA epidemiological, clinical and genetics characteristics. This paper reviews current knowledge of the genetics and epidemiology of the different esophageal atresia/tracheo-esophageal fistula syndromes and associations.

Results. In this review there were analyzed more than 15 foreign sources, which presents current knowledge of the etiology, epidemiology and phenotypic manifestations of states with esophageal atresia.

Conclusion. The refinement of our knowledge of genetic etiologies in EA/TEF is likely to have implications for future epidemiological studies. One goal of epidemiological research in this area is to try to identify geographical or temporal trends in the birth frequency of EA/TEF in order to gain clues about possible contributing environmental factors.

The aim of this study was to evaluate the informativeness of pulse oximetry as a method of neonatal screening for the presence of critical heart defects in apparently healthy full term newborn infants during the early neonatal period. Total 544 term infants were examined. For all neonates oxygen saturation was assessed at the age of 3–72 hours of life. Echocardiography was performed 226 newborns. Primarily it was carried out with a positive result of the screening of blood oxygen saturation, the second – with a negative test result by random sampling. A critical congenital heart disease (transposition of the great vessels) has been found in one of the newborn. The frequency of positive results of screening of blood oxygen saturation was 5.15%, if the test was performed within the first 24 hours of life, and 0.73% if the test is carried out at 63–72 hours of life. Later carrying out pulse oximetry to detect critical congenital heart diseases can reduce the number of false positive results. A small but statistically significant correlation between the frequency of positive screening test result and hemoglobin level (for SpO2 less than 90%), and ejection fraction (for SpO2 less than 95%) was found.

Objective. To compare the efficacy and safety of a new standardized protocol of progressive feeding advancement vs slow advancement protocol in very low birth weight (VLBW) neonatal intensive care unit (NICU) patients.

Study design. This was a prospective single center study with retrospective historical control (before vs after comparison), performed in Federal State Scientific Center for Obstetrics, Gynecology and Perinatology by V.I.Kulakov, Moscow, Russia between April 2014 and April 2015. 53 patients were included. 24 infants in the progressive feeding group were compared to 29 infants in the slow feeding group. There were no statistically significant differences between two groups at the study entry. Statistical analysis was performed with Statistica 8 software. Multiple outcome measures were evaluated. Fisher’s exact and Mann–Whitney U-tests were used to reveal statistically significant differences.

Results. Infants in progressive advancement enteral feeding group reached the enteral volume of 100 ml/kg 5 days faster than controls. Statistically significant improvements were seen in other outcome measures: duration of central venous catheters inserted were 5 days shorter and duration of peripheral venous acsess was 2-fold less in progressive advancement group. Broad-spectrum antibiotics, reserved for nosocomial infections, were used 1.5 times less and the diagnosis of any stage necrotizing enterocolitis (NEC) was seen 3.3 times rarely in the main study group, than in controls. One case of surgical NEC occurred in the slow advancement group and no surgical cases were registered in the standardized progressive group.

Conclusion. Implementation of a progressive standardized approach to nutrition in VLBW infants reduces the use of parenteral nutrition (PN) thereby reducing cost and PN-related infectious risks. This may contribute in reduction of infant mortality and disability, ralated to infectious and other neonatal complications. No adverse increases in NEC were seen in our study. The results must be cautiously extrapolated on the basic population of ELBW infants, further research is needed.

Objectives. On the basis of available literature data and results of our research to examine the incidence of Candida infection in preterm infants, to determine the efficiency of the drug micafungin in the treatment of Candida infection in children of this group.

Results. On the basis of the analysis of literary data of selected risk factors for fungal process in newborn infants, the indications for targeted antifungal therapy, describes the group of drugs used for therapy of invasive candidiasis in the newborn period.