Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Vitamin D Deficiency Prevalence in Late Neonatal Hypocalcemia: A Multicenter Study

Objective: Late neonatal hypocalcemia (LNH) is a common metabolic problem associated with hypoparathyroidism, high phosphate intake and vitamin D deficiency, often presenting with seizures. In this cross-sectional study, we aimed to evaluate the role of vitamin D deficiency in LNH in Turkey and to describe the characteristics of affected newborns. Methods: Conducted with a cross-sectional design and with the participation of 61 neonatal centers from December 2015 to December 2016, the study included term neonates with LNH (n=96) and their mothers (n=93). Data were registered on the FAVOR Web Registry System. Serum samples of newborns and mothers were analyzed for calcium, phosphate, magnesium, albumin, alkaline phosphatase, intact parathyroid hormone (iPTH) and 25 hydroxyvitamin D [25(OH)D] levels. Results: The median (range) onset time of hypocalcemia was 5.0 (4.0-8.0) days of age, with a male preponderance (60.4%). The median (range) serum 25(OH)D levels of the neonates and their mothers were 6.3 (4.1-9.05) and 5.2 (4.7-8.8) ng/mL, respectively. The prevalence of vitamin D deficiency (<12 ng/mL) was high in both the neonates (86.5%) and mothers (93%). Serum 25(OH)D levels of the infants and mothers showed a strong correlation (p<0.001). While the majority (93.7%) of the neonates had normal/high phosphorus levels, iPTH levels were low or inappropriately normal in 54.2% of the patients. Conclusion: Vitamin D deficiency prevalence was found to be high in LNH. Efforts to provide vitamin D supplementation during pregnancy should be encouraged. Evaluation of vitamin D status should be included in the workup of LNH.

Effect of breast milk and sucrose on pain and perfusion index during examination for retinopathy of prematurity.

OBJECTIVE: The objective of this study is to investigate the effect of breast milk and sucrose on pain scores and perfusion index (PI) and to evaluate the alteration in pain and PI during retinopathy of prematurity (ROP) examination. METHODS: This prospective randomized controlled study was conducted with preterm infants who were born in our hospital, hospitalized in the neonatal intensive care unit and whose gestational week was <32 weeks and birth weight was <1500 g. The preterm infants who would undergo ROP examination were allocated to three groups according to simple randomization method as follows: group 1: only local anesthetic eye drops, proparacaine HCl ophthalmic solution 0.5%, group 2: proparacaine HCl ophthalmic solution 0.5% plus breast milk, and group 3: proparacaine HCl ophthalmic solution 0.5% plus sucrose 24%. Postductal PI, transcutaneous oxygen saturation and heart rate (HR) values were measured before the eye examination (0), at the 30th, 60th, and 90th seconds (s) of the eye examination and 30 s after lasting of the examination in all infants. Pain was evaluated using Neonatal Infant Pain Scale (NIPS) during the examination. RESULTS: Fifty-one preterm neonates were prospectively enrolled into the study. The HR was higher during and after the examination in all infants according to before the examination (p < .001). Transcutaneous oxygen saturation values significantly decreased during the examination in breast milk and sucrose groups (p = .001 and <.001, respectively). While PI was found to be lower at the 60th s compared to the 30th s of the examination in the proparacaine HCl group, no difference was found between the values before and after the examination. Perfusion index was found to significantly decrease during and after the examination compared to the values before the examination in the breast milk group. Perfusion index values were determined to significantly decrease at the 30th and 60th s of the examination in the sucrose group. The NIPS scores during the examination were determined to be higher compared to the NIPS scores before the examination in all groups (p< .001). In the intergroup comparisons, the NIPS scores were found to be higher in the sucrose group compared to the proparacaine HCl group at the 60th s of the examination and higher than that in the breast milk group at the 90th s of the examination (p = .02 and p = .01, respectively). CONCLUSIONS: The present study indicates that alterations may be seen in PI during the ROP examination; in other words, peripheral tissue perfusion could be affected. We consider that eye examination is a very painful procedure, and administering breast milk, sucrose or local anesthetic is not sufficient for reducing pain.

Hemolytic anemia caused by non-D minor blood incompatibilities in a newborn.

Hyperbilirubinemia is one of the most widely seen cause of neonatal morbidity. Besides ABO and Rh isoimmunization, minor blood incompatibilities have been also been identified as the other causes of severe newborn jaundice. We report a newborn with indirect hyperbilirubinemia caused by minor blood group incompatibilities (P1, M, N, s and Duffy) whose hemolysis was successfully managed with intravenous immunoglobulin therapy. A thirty-two gestational weeks of preterm male baby became severely icteric on postnatal day 11, with a total bilirubin level of 14.66 mg/dl. Antibody screening tests revealed incompatibility on different minor groups (P1, M, N, s and Duffy (Fya ve Fyb)). On postnatal day thirteen, the level of bilirubin increased to 20.66 mg/dl although baby was under intensive phototherapy. After the administration of intravenous immunoglobulin and red blood cell transfusion, hemoglobin and total bilirubin levels became stabilised. Minor blood incompatibilities should be kept in mind during differential diagnosis of hemolytic anemia of the newborn. They share the same treatment algorithm with the other types hemolytic anemia. New studies revealed that intravenous immunoglobulin treatment in hemolytic anemia have some attractive and glamorous results. It should be seriously taken into consideration for treatment of minor blood incompatibilities.

Neonatal cerebral sinovenous thrombosis: Two cases, two different gene polymorphisms and risk factors.

Turan Ö, Anuk-Ince D, Olcay L, Sezer T, Gülleroglu K, Yilmaz-Çelik Z, Ecevit A. Neonatal cerebral sinovenous thrombosis: Two cases, two different gene polymorphisms and risk factors. Turk J Pediatr 2017; 59: 71-75. Cerebral sinovenous thrombosis (CSVT) is a rare disease in the neonatal period and also the greatest risk of neonatal mortality and morbidity. In this report, we presented two cases with CSVT and different risk factors. One of these cases had methylenetetrahydrofolate reductase (MTHFR) C677T homozygous polymorphism and the other case had both MTHFR A1298C homozygous polymorphism, plasminogen activator inhibitor-1 (PAI-1) 4G/ 5G polymorphism and elevated lipoprotein a. Early diagnosis and prompt initiation of therapy of neonatal CSVT may prevent neonatal mortality and poor long-term neurodevelopmental outcomes.

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.

Comparing the fatty acid levels of preterm and term breast milk in Turkish women.

AIM: Lipids are the main source of calories and considered very important in infant growth. We aimed to compare fatty acid composition of term and preterm breast milk. This is the first study that compares the fatty acid levels of preterm and term breast milk in Turkish women. MATERIALS AND METHODS: Breast milk samples were obtained from mothers of term (n = 15) and preterm (n = 15) infants on postnatal days 3, 7, and 28. Fatty acid composition of human breast milk was determined longitudinally by gas-chromatography/mass spectrometry. RESULTS: There Were 31 fatty acids measured in the milk samples. In the first month, 17 fatty acid levels had significant differences. In group comparison, some fatty acids (C14:0, C16:0, C18:1 and C20:5) had significantly increased in the preterm group (P = 0.041, P = 0.046, P = 0.027, P = 0.033, respectively), whereas myristoleic acid (C14:1) and eicosanoic acid (C20:0) had significantly increased in the term group (P = 0.015, P = 0.048, respectively). CONCLUSION: Term and preterm milk have different compositions of fatty acids. Breast milk composition changes over time. As a general conclusion, breast milk provides the lipid requirements of infants.

Congenital hairy polyp and autoamputation in an infant with acute otitis media.

Hairy polyp is a rare, benign tumor that frequently localizes in the nasopharynx and oropharynx. The embryogenesis of hairy polyp is not known precisely. The clinical profile can vary depending on the size and location of the tumor. In this report, we present a case with hairy polyp who was admitted with acute otitis media and completely recovered after spontaneous autoamputation.

The prediction of later neurodevelopmental status of preterm infants at ages 7 to 10 years using the Bayley Infant Neurodevelopmental Screener.

The aim of this study was to see whether the scores of the Bayley Infant Neurodevelopmental Screener of 45 high-risk preterm infants (gestational age 26-37 wk) between the ages of 3 and 24 months predicted neurodevelopmental status at 7 to 10 years of age. Neurodevelopmental status of 45/122 preterm infants, grouped according to their gestational ages of 26 to 29, 30 to 32, and 33 to 37 weeks, were previously evaluated by Bayley Infant Neurodevelopmental Screener. The scores were categorized as low or high-moderate. Verbal and performance scores of Wechsler Intelligence Scale for Children-Revised (WISC-R) of those patients were assessed between 7 and 10 years. The patients with high-moderate-risk scores of Bayley Infant Neurodevelopmental Screener at all times, regardless of their gestational age, had lower performance, verbal, and total scores of WISC-R than those of who had low Bayley Infant Neurodevelopmental Screener risk scores. High-moderate risk score of Bayley Infant Neurodevelopmental Screener at 7 to 10, and 16 to 20 months, of all patients especially showed good prediction for identifying lower verbal and performance scales. For 7 to 10 months, verbal scale: positive predictive value = 92.3%, negative predictive value = 44.4%, sensitivity = 70.58%, and specificity = 80%; performance scale: positive predictive value = 100%, negative predictive value = 30%, sensitivity = 68.18%, and specificity = 100%. For 16 to 20 months, verbal scale: positive predictive value = 90%, negative predictive value = 37.5%, sensitivity = 64.3%, and specificity = 80%; performance scale: positive predictive value = 90%, negative predictive value = 12.5%, sensitivity = 56.3%, and specificity = 50%. Bayley Infant Neurodevelopmental Screener shows good prediction of later verbal and performance scores of Wechsler Intelligence Scale-Revised for Children as early as 7 to 10 months, which gives us the opportunity to start early intervention.

Intravenous Immunoglobulin G Treatment in ABO Hemolytic Disease of the Newborn, is it Myth or Real?

Intravenous Immunoglobulin G (IVIG) therapy has been used as a component of the treatment of hemolytic disease of the newborn. There is still no consensus on its use in ABO hemolytic disease of the newborn routinely. The aim of this study is to determine whether administration of IVIG to newborns with ABO incompatibility is necessary. One hundred and seventeen patients with ABO hemolytic disease and positive Coombs test were enrolled into the study. The subjects were healthy except jaundice. Infants were divided into two groups: Group I (n = 71) received one dose of IVIG (1 g/kg) and LED phototherapy whereas Group II (n = 46) received only LED phototherapy. One patient received erythrocyte transfusion in Group I, no exchange transfusion was performed in both groups. Mean duration of phototherapy was 3.1 ± 1.3 days in Group I and 2.27 ± 0.7 days in Group II (p < 0.05). Mean duration of hospital stay was 5.34 ± 2.2 days in Group I and 3.53 ± 1.3 days in Group II (p < 0.05). Mean duration of phototherapy was 4.0 ± 1.5 days and 2.73 ± 1.1 days in double and single doses of IVIG respectively, and this was statistically significant (p < 0.05). IVIG therapy didn't decrease neither phototherapy nor hospitalization duration in infants with ABO hemolytic disease. Meticulus follow-up of infants with ABO hemolytic disease and LED phototherapy decreases morbidity. IVIG failed to show preventing hemolysis in ABO hemolytic disease.

Cerebral hemodynamic changes and pain perception during venipuncture: is glucose really effective?

Newborns are exposed to a considerable number of painful stimuli. This study is aimed to investigate the effects of 30% glucose solution and nonnutritive sucking on pain perception during venipuncture. Twenty-five term infants were randomized as receiving 30% dextrose (group 1) or sterile water (group 2). Neonatal Infant Pain Scale scores, skin conductance algesimeter recordings, and near-infrared spectroscopy measurements were recorded during the procedure. Neonatal Infant Pain Scale and skin conductance algesimeter results were decreased in both groups from that during venipuncture to after the procedure. Group 1 had lower Neonatal Infant Pain Scale scores compared with group 2 after venipuncture, different from the skin conductance algesimeter, where no difference was observed between groups. In group 1, cerebral blood volume increased after venipuncture. Glucose does not attenuate the Neonatal Infant Pain Scale score and skin conductance algesimeter index during venipuncture, but it leads to a lower Neonatal Infant Pain Scale score after venipuncture unlike the skin conductance algesimeter index, which was not lowered.

Short- and long-term effects of individualized enteral protein supplementation in preterm newborns.

The aim of this retrospective study was to assess the need for additional enteral protein supplementation in preterm newborns with gestational age (GA) ≤32 weeks after full enteral feeds with either fortified breast milk (FBM) or preterm formula (PF) were reached, and to determine the effects of additional protein on physical and neurological development. After the standard early total parenteral nutrition (TPN) and reaching full enteral nutrition with 150-160 ml/kg/day, preterms were assessed for the requirement of additional protein based on serum blood urea nitrogen (BUN)/prealbumin levels. Additional enteral protein was given for BUN <5 mg/dl and/or prealbumin ≤8 mg/dl with weekly assessments as per Neonatal Intensive Care Unit (NICU) protocol. Growth in the NICU and neurodevelopmental outcome at 18 months' corrected age (CA) were determined. There were 32 newborns in the non-supplemented group (Group 1) and 33 newborns in the supplemented group (Group 2). All newborns in Group 2 were on FBM. Weight gain and head growth were better and Bayley scores at 18 months' CA were higher in Group 2. Standard preterm nutrition with FBM may not be sufficient for preterms, and additional enteral protein supplementation may improve the physical growth rate in the NICU and result in better neurodevelopmental outcome at 18 months' CA.

Peripheral microcirculation is affected during therapeutic hypothermia in newborns.

BACKGROUND: Hypothermia is becoming a common treatment for newborns with hypoxic ischaemic encephalopathy. Cerebral metabolic effects have been studied extensively. However, acute effects on peripheral microcirculation are unknown. The effects of therapeutic hypothermia on peripheral microcirculation assessed by side-stream dark field (SDF) imaging technique are presented. METHODS: Peripheral microcirculation was assessed in seven newborns undergoing selective head-cooling treatment with SDF imaging video recordings during core temperature 34°C, and then after re-warming at 37°C, and also in seven control patients with rectal temperature 37°C. Microvascular flow index (MFI) and per cent of vessels with sluggish flow were determined by using appropriate software. RESULTS: Sluggish microcirculation was observed during hypothermia compared with controls. MFI and per cent of vessels with sluggish flow returned to normal after re-warming. CONCLUSIONS: The results of this small group of newborns going through therapeutic hypothermia suggests that microcirculation is effected with this treatment. Whether this finding has other clinical impacts requires further research.

Use of amplitude-integrated electroencephalography (aEEG) and near infrared spectroscopy findings in neonates with asphyxia during selective head cooling.

BACKGROUND: Amplitude-integrated electroencephalogram (aEEG) at <6 h is the best single outcome predictor in term infants with perinatal asphyxia at normothermia. Hypothermia treatment has changed the cutoff values for outcome prediction by using time at onset of normal trace and SWC. Cerebral hemodynamics and oxygenation changes detected by near infrared spectroscopy (NIRS) during hypothermia treatment in aphyxiated neonates are not a well known issue. AIM: The aim of this study was to investigate the correlations between brain monitoring (amplitude integrated EEG and NIRS) and outcome in asphyxiated full-term infants with moderate/severe hypoxic-ischemic encephalopathy before, during and after hypothermia treatment. METHOD: Ten neonates were recruited for hypothermia treatment by using the cool cap entry criteria. aEEG and NIRS were applied in 10 and 8 patients, respectively with moderate and severe hypoxic-ischemic encephalopathy before, just after brain cooling and rewarming periods. Patterns and voltages of aEEG backgrounds sleep-wake cycles (SWC) and NIRS values (TOI% and FTOE) were recorded. During the follow up their outcomes were assessed by using the Bayley Scales of Infant Development II. CONCLUSION: Hypothermia changes the predictive value of early aEEG. Normalization of a baby's aEEG and the appearance of SWCs while being cooled occurs later. In our study one patient had normal aEEG background pattern at 80 and imminent SWC at 90 h after birth and still had normal Bayley scores at 24 months. Time to normal aEEG and SWC appearance should be carefully evaluated during the cooling period. NIRS values were different due to the clinical presentations of the patients.

Are the neonatal outcomes similar in large-for-gestational age infants delivered by women with or without gestational diabetes mellitus?

BACKGROUND: Infants are considered large for gestational age (LGA) if their birth weight is greater than the 90th percentile for gestational age and they have an increased risk for adverse perinatal outcomes. Maternal diabetes is one of the factors affecting birthweight. However there are limited data on the perinatal outcomes of infants of gestational diabetic mothers. The aim of the present study was to compare the neonatal outcomes of LGA infants delivered by women with and without gestational diabetes mellitus. METHODS: This was a retrospective study of LGA infants of ≥36 weeks of gestation born at the Gazi University Medical School Hospital during the period of 2006-2009. Neonatal outcomes included hypoglycemia and polycythemia in the early neonatal period and hospital admissions. The Chi-square and Student's t test were used for comparing variables. RESULTS: Seven hundred eligible infant-mother pairs were enrolled in the study. Eighty-seven of them (12.4%) were infants of gestational diabetic mothers and 613 (87.6%) were infants of non-diabetic mothers. The incidence of hypoglycemia at the first hour was higher in infants of diabetic mothers (12.8%) than in infants of non-diabetic mothers (5.3%) (P=0.014). Polycythemia was also more frequently observed in infants of the gestational diabetic mothers (9.3%) than in infants of the non-diabetic mothers (3.0%) (P=0.010). Although overall hospital admission rates were not different between the two groups, infants of diabetic mothers were more likely to be admitted because of resistant hypoglycemia (P=0.045). CONCLUSIONS: The results of this study suggested that LGA infants of mothers with gestational diabetes mellitus were at a greater risk for hypoglycemia and polycythemia in the early neonatal period than LGA infants of nondiabetic mothers.

A rare case of severe lactic acidosis in a preterm infant: lack of thiamine during total parenteral nutrition.

Total parenteral nutrition (TPN) is a revolution in neonatal intensive care unit (NICU) care, but this therapy is not without problems. A 35-week-old, 1300 g female infant was transferred to our NICU because of bilious vomiting and feeding problems. When enteral feeding was started again, a severe condition similar to the previous one developed. On the 24th day, the patient underwent surgery with a diagnosis of Hirschprung's disease. One week before surgery, the parenteral solutions were composed without vitamins because intravenous vitamin supplements suitable for infants were not available. Thereafter, the patient suffered from severe hypoglycaemia, and sepsis started to develop, accompanied by a large anion gap and metabolic acidosis which is severe lactic acidosis refractory to massive doses of bicarbonate. The acidosis improved significantly when the patient was treated with thiamin. Although TPN is life saving in the NICU, meticulous attention must be paid while treating a patient with TPN, and all possible nutrients should be provided. In this report, a case of a preterm newborn requiring a prolonged period of TPN and complicated by serious lactic acidosis is presented and discussed.

Prenatally detected congenital cystic adenomatoid malformation and postnatally diagnosed trisomy 13: case report and review of the literature.

Congenital cystic adenomatoid malformation (CCAM) is a rare bronchopulmonary malformation characterized by loss of the normal pulmonary tissue. CCAM may be frequently associated with cardiac and renal anomalies. Rarely, CCAM may be seen with chromosome abnormalities. This is the first reported neonatal case of prenatally detected CCAM and postnatally diagnosed trisomy 13.

Language assessment of non-handicapped twins at 5 years of age.

BACKGROUND: The aim of the present study was to determine language levels in twins and singletons born after at least 34 weeks gestation and without identifiable neurological abnormality and to evaluate whether in vitro fertilization (IVF) affects language development in twin pregnancies. METHODS: A prospective study of a large cohort of all children born between 1 January 2001 and 31 December 2003 was carried out at Gazi University Hospital. All live-born twin pairs in which both twins survived were identified, and a comparable sample of families with pairs of singletons were chosen. The Stanford-Binet Intelligence Scale Form and the translated Turkish form of the Peabody Picture Vocabulary Test were completed at 60 months. RESULTS: Even after excluding the most premature twins and those with diagnosable neurological damage, twins performed worse than singletons on language development tests. Twin girls had better scores than twin boys. A statistically significant difference was found between the scores of term and preterm twins. No significant difference was noted when compared according to birth order. Appropriate for gestational age (AGA) twins did better than small for gestational age (SGA) twins in the test scores. All twin girls did not differ from singleton girls, but all twin boys performed worse than singleton boys. Term twins had similar results with term singletons, but preterm twins had lower scores than preterm singletons. SGA singletons had better scores than SGA twins, while AGA twins and singletons did not differ. When the children were compared with regard to method of conception, IVF children had significantly lower scores on the tests than those in the spontaneous conception group. CONCLUSION: It is hoped that the present findings could lead to a more precise assessment of children for speech impairment and, above all, to more efficient preventive intervention. Whatever mechanisms are involved, the present results indicate that twins born as a result of IVF, are at a disadvantage in terms of language development in comparison with spontaneously conceived twins.