Neonatal Resuscitation Practices in Turkey: A Survey of the Turkish Neonatal Society and the Union of European Neonatal and Perinatal Societies.

OBJECTIVE: Optimal care in the delivery room is important to decrease neonatal morbidity and mortality. We aimed to evaluate neonatal resuscitation practices in Turkish centers. MATERIALS AND METHODS: A cross-sectional survey consisted of a 91-item questionnaire focused on delivery room practices in neonatal resuscitation and was sent to 50 Turkish centers. Hospitals with <2500 and those with ≥2500 births/year were compared. RESULTS: In 2018, approximately 240 000 births occurred at participating hospitals with a median of 2630 births/year. Participating hospitals were able to provide nasal continuous-positiveairway-pressure/high-flow nasal cannula, mechanical ventilation, high-frequency oscillatory ventilation, inhaled nitric oxide, and therapeutic hypothermia similarly. Antenatal counseling was routinely performed on parents at 56% of all centers. A resuscitation team was present at 72% of deliveries. Umbilical cord management for both term and preterm infants was similar between centers. The rate of delayed cord clamping was approximately 60% in term and late preterm infants. Thermal management for preterm infants (<32 weeks) was similar. Hospitals had appropriate equipment with similar rates of interventions and management, except conti nuous-positive-airway-pressure and positive-end-expiratory-pressure levels (cmH2O) used in preterm infants (P = .021, and P = .032). Ethical and educational aspects were also similar. CONCLUSIONS: This survey provided information on neonatal resuscitation practices in a sample of hospitals from all regions of Turkey and allowed us to see weaknesses in some fields. Although adherence to the guidelines was high among centers, further implementations are required in the areas of antenatal counseling, cord management, and circulation assessment in the delivery room.

Glutathione synthetase deficiency: a novel mutation with femur agenesis.

Introduction: Glutathione synthetase (GSS) deficiency is an autosomal recessive disorder (frequency < 1/1,000,000) with different varyingly severe clinical manifestations that include metabolic acidosis, hemolytic anemia, hyperbilirubinemia, neurological disorders and sepsis. Case report: This infant was small for gestational age, had hemolytic anemia, metabolic acidosis, bilateral subependymal pseudocysts and increased echogenicity of the basal ganglia. GSS deficiency was confirmed by genetic analysis. The patient also had unilateral right femur agenesis. Conclusion: By using next generation sequencing analysis, we identified a novel homozygous variant c.800G > A, p.Arg267Gln in the GSS gene of this patient. Femur agenesis had not previously been associated with GSS.

Fetal Bowel Dilatation due to Intestinal Neuronal Dysplasia: A Rarity.

Intestinal neuronal dysplasia (IND) type B is characterized by malformation of parasympathetic plexus and manifests at more than 6 month of age with progressive severe constipation. We report a case of IND type B presented with bowel dilatation on antenatal scan and neonatal intestinal obstruction which is unusual with this type of IND.

Extralobar pulmonary sequestration as a cause of recurrent pulmonary hemorrhage in a neonate.

Pulmonary sequestration is acystic or solid congenital lung malformation comprised of non functional lung tissue that does not communicate with the normal tracheobronchial tree and has a systemic arterial blood supply. There are two forms of sequestration: intralobar and extralobar. Its treatment is surgical resection. Here we presented a case of premature neonate with extralobar pulmonary sequestration who had respiratory failure and recurrent pulmonary hemorrhage. Following surgery, the patient showed significant clinical improvement.

El secuestro pulmonar es una malformación pulmonar congenita, quística o sólida, compuesta de tejido pulmonar no funcional sin conexión con el árbol traqueobronquial y que recibe sangre arterial de la circulación sistemica. Existen dos formas de secuestro: intralobular y extralobular. El tratamiento se realiza mediante resección quirúrgica. Se describe el caso de un recién nacido prematuro con secuestro pulmonar extralobular que presentó insuficiencia respiratoria y hemorragia pulmonar recurrente. Luego de la cirugía, el paciente mejoró en forma clínicamente significativa.

Secuestro pulmonar extralobular como causa de hemorragia pulmonar recurrente en un recién nacido / Extralobar pulmonary sequestration as a cause of recurrent pulmonary hemorrhage in a neonate

El secuestro pulmonar es una malformación pulmonar congenita, quística o sólida, compuesta de tejido pulmonar no funcional sin conexión con el árbol traqueobronquial y que recibe sangre arterial de la circulación sistemica. Existen dos formas de secuestro: intralobular y extralobular. El tratamiento se realiza mediante resección quirúrgica. Se describe el caso de un recién nacido prematuro con secuestro pulmonar extralobular que presentó insuficiencia respiratoria y hemorragia pulmonar recurrente. Luego de la cirugía, el paciente mejoró en forma clínicamente significativa.

Pulmonary sequestration is acystic or solid congenital lung malformation comprised of non functional lung tissue that does not communicate with the normal tracheobronchial tree and has a systemic arterial blood supply. There aretwo forms of sequestration: intralobar and extralobar. Its treatment is surgical resection. Here we presented a case of premature neonate with extralobar pulmonary sequestration who had respiratory failure and recurrent pulmonary hemorrhage. Following surgery, the patient showed significant clinical improvement.

Early screening for critical congenital heart defects in asymptomatic newborns in Bursa province.

OBJECTIVE: In this study, pulse oximetry screening results in the early diagnosis of critical congenital heart diseases (CCHD) will be evaluated. METHODS: Eight-thousand two-hundred and eight of 10,200 newborns born between January 2014 and December 2014 were screened using pulse oximetry for the diagnosis of CCHD. Screening test was considered to be positive in the newborns whose saturation after 24 h from the birth with pulse oximetry was ≤ 95% and/or in the newborns who had a difference of ≥ 3% between the lower and right upper extremity. RESULTS: Incidence of CCHD was 1 per 1000 live births. Coarctation of the aorta was the most commonly determined CCHD. Sensitivity, specificity, false negative rate and false positive rate of pulse oximetry in the diagnosis of CCHD were 60%, 99.8%, 40% and 0.12%, respectively. Seventy-five percent of the newborns who had a false negative diagnosis with pulse oximetry had coarctation of the aorta. Coarctation of the aorta was determined at a rate of 20% using CCHD screening. CONCLUSIONS: The diagnosis of coarctation of the aorta is missed in the newborns screened with pulse oximetry in the first 24-48 h after birth. Screening with pulse oximetry should be repeated for early diagnosis of coarctation of the aorta.

The propre-save study: effects of probiotics and prebiotics alone or combined on necrotizing enterocolitis in very low birth weight infants.

OBJECTIVE: To test the efficacy of probiotic and prebiotic, alone or combined (synbiotic), on the prevention of necrotizing enterocolitis (NEC) in very low birth weight (VLBW) infants. STUDY DESIGN: A prospective, randomized, controlled trial was conducted at 5 neonatal intensive care units in Turkey. VLBW infants (n = 400) were assigned to a control group and 3 study groups that were given probiotic (Bifidobacterium lactis), prebiotic (inulin), or synbiotic (Bifidobacterium lactis plus inulin) added to breastmilk or formula for a maximum of 8 weeks before discharge or death. The primary outcome was NEC (Bell stage ≥2). RESULTS: The rate of NEC was lower in probiotic (2.0%) and synbiotic (4.0%) groups compared with prebiotic (12.0%) and placebo (18.0%) groups (P < .001). The times to reach full enteral feeding were faster (P < .001), the rates of clinical nosocomial sepsis were lower (P = .004), stays in the neonatal intensive care unit were shorter, (P = .002), and mortality rates were lower (P = .003) for infants receiving probiotics, prebiotics, or synbiotic than controls. The use of antenatal steroid (OR 0.5, 95% CI 0.3-0.9) and postnatal probiotic (alone or in synbiotic) (OR 0.5, 95% CI 0.2-0.8) decreased the risk of NEC, and maternal antibiotic exposure increased this risk (OR 1.9, 95% CI 1.1-3.6). CONCLUSIONS: In VLBW infants, probiotic (Bifidobacterium lactis) and synbiotic (Bifidobacterium lactis plus inulin) but not prebiotic (inulin) alone decrease NEC.

Storage at -80°C preserves the antioxidant capacity of preterm human milk.

BACKGROUND AND OBJECTIVE: It is essential to establish optimum parameters for maintaining the quality of stored milk until the moment of consumption with minimal deterioration of its properties. The aim of the study was to evaluate total antioxidant capacity (TAC) and total oxidation status (TOS) of fresh and freeze-stored samples (at -80°C) of preterm human milk (HM). METHODS: Samples of colostrum were collected from 98 healthy women within the first 4 days after delivery. The total milk volume collected (6 ml) was divided in two aliquot parts: 3 ml for the fresh analysis which was done immediately after the extraction and 3 ml for storage under freezing conditions at -80°C for three months. The antioxidant status and oxidative stress of the fresh and stored breast milk were assessed via determination of TAC and TOS levels. RESULTS: The mean gestational age and the birth weight of the infants were 31.26 ± 2.93 weeks and 1620 ± 581.91 g; respectively. There were no significant correlations between maternal age, route of delivery and milk oxidative stress. There was no significant difference between the levels of TAC, TOS and the oxidative stress index in fresh and freeze-stored samples of colostrum in preterm HM (p > 0.05). CONCLUSION: Freeze storage of preterm HM at -80°C for three months preserves the antioxidant capacity without changing oxidative status of HM, which could be noteworthy for the preterm infant nutrition.

Role of pentoxifylline and/or IgM-enriched intravenous immunoglobulin in the management of neonatal sepsis.

OBJECTIVE: To investigate the effectivity of pentoxifylline (PTX) and immunoglobulin M (IgM)-enriched intravenous immunoglobulin (IVIG) therapy in the treatment of neonatal sepsis (NS), alone or in combination. STUDY DESIGN: This was a prospective, double-blind, controlled study. Newborns with suspicion of sepsis were enrolled in the study. The patients were separated into four groups according to treatment protocol: Group 1 = placebo, Group 2 = pentoxifylline, Group 3 = IgM-enriched IVIG, and Group 4 = pentoxifylline + IgM-enriched IVIG. Blood samples were taken for C-reactive protein, interleukin-6, neutrophil CD64 expression, and tumor necrosis factor-alfa measurements immediately before treatment (1st day), and measurements were repeated on the 2nd and 4th days of the therapy. RESULTS: A total of 204 patients, 51 in each group, were recruited into the study. There were no significant differences for symptoms of sepsis among groups, except lethargy. No significant differences were observed among the groups according to laboratory data. Overall mortality rate was 8.8%. The rates of morbidities and mortality among study groups were similar. CONCLUSION: PTX and IgM-enriched IVIG therapies, either alone or in combination, did not reduce the rates of morbidities and mortality in NS.

Antioxidant capacity of fresh and stored breast milk: is -80°C optimal temperature for freeze storage?

OBJECTIVE: To determine total antioxidant capacity and total oxidation status in fresh and freeze stored (at -80°C) breast milk during the stages of lactation. METHODS: Samples of colostrum, transitional and mature milk were collected from 44 healthy women at 3, 8 and 30 days after birth. The total milk volume collected (6 ml) was divided in two aliquot parts: 3 ml for the fresh analysis which was done immediately after the extraction and 3 ml for storage under freezing conditions at -80°C for two months. The antioxidant status and oxidative stress of the fresh and stored breast milk were assessed via determination of total antioxidant capacity and total oxidation status. RESULTS: Antioxidant capacity of transitional and mature milk decreased (p = 0.0001, p = 0.028, respectively); however, antioxidant capacity of colostrum did not change by storage at -80°C (p > 0.05). Total antioxidant capacity of fresh and stored breast milk significantly decreased during the stages of lactation (p < 0.0001, p = 0.028, respectively). Total oxidation status showed no significant difference in fresh and stored breast milk during the stages of lactation (p > 0.05). CONCLUSION: Freeze storage of breast milk at -80°C for two months seems not to be the optimal condition to preserve the antioxidant capacity of breast milk.

Hydrops Fetalis due to Kell Alloimmunization: A Perinatal Approach to a Rare Case.

OBJECTIVE: While routine administration of rhesus (Rh) immunoglobulin has significantly reduced the incidence of Rh alloimmunization, maternal alloimmunization to other red cell antigens remains a contributor to perinatal morbidity and mortality. Although the Kell antigen is seen on the red cells of only 9% of the general population, attention to Kell antibodies continues to increase. CASE REPORT: A case of fetal hydrops was sonographically detected at 30 weeks of gestation. Antenatal tests to evaluate the fetus's condition clearly showed that the level of hemolytic disease was critical and the baby was delivered prematurely due to fetal distress. The combination of anemia, reticulocytopenia, hydrops fetalis, and a positive indirect Coombs test suggested Kell isoimmunization. The baby was successfully treated with exchange transfusion of Kellnegative packed red cells, and was discharged on postnatal d 30. CONCLUSION: The presented case of hydrops fetalis was due Kell alloimmunization that was detected during the postnatal period, and thus we plan to discuss the perinatal approach to Kell immunization.

A newborn case with perinatal-lethal Gaucher disease due to R463H homozygosity complicated by C677T homozygosity in the MTHFR gene.

Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis, in utero fetal demise and neonatal distress. In some cases without hydrops, neurological signs occur in the first week of life and lead to death within 3 months. Less common signs of the disease are hepatosplenomegaly, ichthyosis, arthrogryposis and facial dysmorphy. We describe a preterm neonate with Gaucher disease homozygous for R463H mutation in GBA gene who showed severe neurologic signs in addition to refractory thrombocytopenia, hepatosplenomagaly, direct hyperbilirubinemia, facial dysmorphy and ichthyosiform skin abnormalities in addition to having thrombosis in portal and splenic veins possibly due to homozygosity for C677T mutation in MTHFR gene. To the best of our knowledge, this is the first case homozygous for the GBA R463H mutation resulting in Gaucher disease with a concomitant homozygous MTHFR C677T mutation.

Does polycythemia affect interleukin-6 response pattern in early postnatal period?

INTRODUCTION: Neonatal polycythemia may result in increased cytokine production. We aimed to investigate whether polycythemia and partial exchange transfusion (PET) affect interleukin-6 (IL-6) response pattern in early neonatal period. METHODS: Ninety-four newborns, 57 with polycythemia (Group 1) and 37 as control group (Group 2) were enrolled in the study. PET was performed at 4-6 hr following birth in the first group. Blood levels of IL-6 were measured at 2-4 hr following birth; measurements were repeated at 6 and 24 hr after PET in newborns with polycythemia and at similar hours in Group 2. In Group 1, two patients (3.5%) who were diagnosed with proven sepsis excluded from the study. RESULTS: Both initial and the last IL-6 levels were higher in Group 1 (21.7; 5.5-190 pg/ml and 18.3; 2.7-92.4 pg/ml) than those of the controls (8.4; 0.2-47.8 pg/ml and 8.6; 2.0-21.0 pg/ml) (P=0.001 for both comparisons). In Group 1, IL-6 levels increased at 6 hr after PET and decreased thereafter. IL-6 showed the same pattern in the control group. IL-6 levels were higher than >70 pg/ml in two (3.6%), seven (12.7%), and two (3.6%) subjects during three evaluation steps, respectively. Neither clinical nor proven sepsis was subsequently detected in any of these subjects. IL-6 levels were within the acceptable values in Group 2. CONCLUSION: IL-6 levels seem to be high in newborns with polycythemia during the first days of life, although they rarely exceed maximum acceptable levels. The pattern of IL-6 response might be taken into account to optimize its use in the diagnosis of early-onset neonatal sepsis.