Frequency of ABO and rhesus blood groups among neonates born at a private hospital in Istanbul.

In this study we used gel centrifugation to determine the frequency and sex distribution of ABO and Rh blood group antigens among 4,656 neonates (2,317 males, 2,339 females) born at a private hospital in Istanbul. Group A Rh+ was the most frequent blood type and AB Rh- was the least frequent. Female neonates had a higher frequency of Rh+ blood than male neonates. Group A blood was detected most frequently followed by group O, group B, and group AB. Sex was not significantly associated with blood type. These results fall within the range of previously reported blood type frequencies for Turkey. Our data allow the creation of a blood bank database for the hospital.

Electronic media use by children in families of high socioeconomic level and familial factors.

The aim of this research was to describe electronic media access and use among children aged 6 months to 15 years and to identify familial factors. Data was collected with a questionnaire from 724 parents of children seen in the pediatric outpatient clinic of a private hospital while they awaited examination. Questions included electronic media ownership at home or in the child's bedroom, household habits of electronic media use, educational and occupational status of parents, smoking and alcohol consumption at home, and family income. The top three electronic media devices present at home were television, computer and DVD. On a typical day, 32% of children watched television for approximately less than 1 hour, 36% for 2 hours and 22% for 3 hours. Mean television viewing time was 1.89 +/- 0.76 hours. Nearly 12% of parents spent less than 30 minutes with their children, whereas 28% spent 1 hour, and 59% more than 1 hour. Older children spent more time watching television than the younger ones. Forty-six percent of children used computer and the internet at home on a typical day. Almost 20% of children also had a television in their bedroom and spent more time watching television than those without a television in their bedroom, and the ratio increased by age (one-fifth of 3-5-year olds, one-fourth of 6-10-year-olds and half of 11-15-year-olds, on average). The present study provides data for the education and counselling of parents about the use of electronic media by children and it will contribute to increasing the awareness and sensitivity of the population by drawing attention to the subject.

Serum pro-hepcidin levels in term and preterm newborns with sepsis.

BACKGROUND: An iron regulatory peptide hormone, hepcidin, is also part of the innate immune system and is strongly induced during infections and inflammation. The aim of the present study was to determine serum levels of the 60 aa pro-hormone form of hepcidin (pro-hepcidin) in full-term and preterm newborns with sepsis and to determine the possible relationships between pro-hepcidin levels and serum iron and complete blood count parameters. METHODS: Fifteen preterm newborns with sepsis, 17 healthy preterm, six full-term newborns with sepsis and 16 healthy full-term newborns were included the study. Blood samples were collected from patients with sepsis at the time of clinical diagnosis. Each blood sample was analyzed for complete blood count, serum iron and ferritin concentrations, iron-binding capacity, and pro-hepcidin level. RESULTS: The mean serum pro-hepcidin level (mean +/- SD) in preterm neonates with sepsis and in healthy preterm newborns was 565.4 +/- 519.5 ng/mL and 279.8 +/- 227.6 ng/mL, respectively (P < 0.05). The mean serum pro-hepcidin level in full-term newborns with sepsis and in healthy full-term neonates was 981.4 +/- 415.4 ng/mL and 482 +/- 371.9 ng/mL, respectively (P < 0.05). Although the mean serum ferritin levels in the two groups with sepsis were higher when compared with the healthy groups, the difference was not statistically significant in full-term newborns. No statistically significant correlations were found between serum pro-hepcidin levels and any other parameters in each group. CONCLUSIONS: Serum pro-hepcidin levels were higher in newborns with sepsis (either premature or full-term) than they were in healthy newborns at the time of clinical diagnosis.

Relationship between thyroid hormone levels and transient tachypnea of the newborn in late-preterm, early-term, and term infants.

PURPOSE: We aimed to investigate the association between thyroid hormone levels and transient tachypnea of the newborn (TTN) among late-preterm, early-term, and term infants admitted to neonatal intensive care unit (NICU). MATERIALS AND METHOD: In the current retrospective study, neonates admitted to the NICU due to TTN were assigned to the TTN group (n = 404). Healthy neonates who were followed up in the well-baby nursery comprised the control group (n = 7335). Infants were grouped by gestational age into late-preterm (34-366 weeks), early-term (37-386 weeks), and term subgroups (39-416 weeks). Serum levels of thyroid-stimulating hormone (TSH) and thyroxin (T4) were determined from venipuncture samples taken at least 48 hours after birth. The relationship between thyroid hormone levels and the need for NICU admission for TTN was compared between groups. RESULTS: Compared to control infants, term neonates with TTN had significantly higher TSH levels, whereas late-preterm and early-term neonates with TTN had significantly lower T4 levels. Birth weight and mode of delivery had no effect on NICU admission for TTN. CONCLUSIONS: Infants admitted to NICU due to TTN had significantly different thyroid hormone levels with differences depending on gestational age.

Functional pulmonary atresia in a patient with neonatal Marfan syndrome caused by a c.3602G>A mutation in exon 29 of the FBN1 gene.

Neonatal Marfan syndrome is a severe form of the syndrome mostly caused by de-novo mutations in the fibrillin-1 gene. We report a newborn with neonatal Marfan syndrome and functional pulmonary atresia who died from congestive heart failure on postnatal day 22 despite treatment. He had a mutation in exon 29 of the fibrillin-1 gene at position c.3602G>A. Functional pulmonary atresia may be a life-threatening cardiovascular manifestation of neonatal Marfan syndrome.

Hepatic involvement in perinatal asphyxia.

BACKGROUND: The pathogenetic mechanisms of hepatic injury in perinatal asphyxia (PNA) are similar to those in ischemic hepatitis, yet liver involvement is currently not considered a component of multi-organ failure in PNA. METHODS: A retrospective study was done on 56 newborns with PNA. Hepatocyte injury was diagnosed based on elevated serum alanine transaminase level (>100 U/L, twice upper normal) with subsequent normalization. RESULTS AND CONCLUSIONS: Twenty-two of the patients had hepatocyte injury. Fetal distress, thrombocytopenia, convulsions, pathologic findings on imaging of the central nervous system, and a high rate of intrauterine growth retardation were the factors significantly associated with hepatocyte injury. This damage was also associated with high mortality.

Invasive Candida albicans infection mimicking leukemia in a neonate.

Congenital leukemia is rare, and is frequently diagnosed as a form of acute myeloid leukemia at, or immediately after birth. Some infections, viral in particular, can mimic clinical signs and/or laboratory findings of congenital leukemia. This is the first documented case of candidemia resembling leukemia.

Thrombocytosis in the neonatal intensive care unit: Experience at a single center.

Although thrombocytosis is frequently detected in newborns, few reports have focused on its risk factors. This report documents the characteristics of 89 neonates with thrombocytosis followed up in a neonatal intensive care unit (NICU). We reviewed the patients' medical and laboratory records retrospectively to determine the associated conditions and risk factors for neonatal thrombocytosis, and complications related to thrombocytosis. We also discussed the differences of neonatal thrombocytosis from that of childhood in the light of literature. The mean platelet count of these newborns was 579.7 ± 111.5 (451-936) x 109/L. Associated conditions included anemia (73.7%), high- isk pregnancies (56. %), prematurity (51.7%), infections (37.1%), antenatal drug use (22.7%), indirect hyperbilirubinemia (20.2%), cardiac disorders (14.6%), respiratory distress syndrome (14.6%), history of hypoxia (13.5%), surgery (12.4%), and hemorrhage (3.4%). In most cases, more than one risk factor for thrombocytosis existed in the same newborn. No hemorrhagic complications related to thrombocytosis were observed; however, 1 newborn had portal vein thrombosis associated with intestinal malrotation. In summary, risk factors and associated conditions for thrombocytosis in newborns differed from those in children. In light of associated disorders, the mechanism of thrombocytosis in newborns may differ from that of childhood thrombocytosis.

Heterozygous methylenetetrahydrofolate reductase 677C-T gene mutation with mild hyperhomocysteinemia associated with intrauterine iliofemoral artery thrombosis.

Neonatal thrombosis is a serious event that can cause mortality or severe morbidity. Newborn-related factors, including genetic prothrombotic risk factors, may affect the occurrence of neonatal thrombosis. In this report, a case of intrauterine iliofemoral arterial thrombosis associated with mild hyperhomocysteinemia caused by methylenetetrahydrofolate reductase 677C-T gene mutation is presented. We suggest that methylenetetrahydrofolate reductase gene mutation might be investigated in neonates and their families presenting with thromboembolic disease.

Risk factors in the development of mild and severe retinopathy of prematurity.

PURPOSE: To evaluate the risk factors in the development of mild and severe retinopathy of prematurity (ROP). METHODS: All infants (n = 88) screened for ROP at a neonatal intensive care unit over a 2-year period with gestational age

Extreme hyperbilirubinemia in newborn infants.

This study was undertaken to determine the frequency and investigate the etiology of extreme hyperbilirubinemia (total serum bilirubin [TSB]>or=25 mg/dL [428 micromol/L]) in newborns admitted to a neonatal intensive care unit in southern Turkey. The charts of 93 term and near-term infants admitted with TSB levels of 25 mg/dL (428 micromol/L) or greater in the first 30 days after birth were retrospectively reviewed. During the 4.5-year study period, 774 infants were admitted to our unit with neonatal jaundice. Ninety-three (12%) of these infants had TSB levels of 25 mg/dL (428 micromol/L) or greater. The mean TSB level in the 93 cases was 30.1+/-5.7 mg/dL (514.7+/-97.5 micromol/L), and the peak levels ranged from 25.0 to 57.4 mg/dL (428-981.5 micromol/L). Thirty-three (35.5%) of the 93 babies had TSB levels of 30 mg/dL (513 micromol/L) or greater. Eighty-nine of 93 infants were being exclusively breast-fed. Nineteen babies were isoimmunized, 7 were bacteremic, 2 of the 39 babies tested for glucose-6-phosphate dehydrogenase had this enzyme deficiency, and 1 of the 71 infants tested for thyroid function had hypothyroidism. No cause for extreme hyperbilirubinemia was found in 61 (65.6%) cases.

Serum pro-hepcidin levels and relationships with iron parameters in healthy preterm and term newborns.

A recently isolated peptide hormone, hepcidin, is thought to be the principal regulator of iron homeostasis. Hepcidin acts by limiting intestinal iron absorption and promoting iron retention in reticuloendothelial cells. Its precursor peptide form is called pro-hepcidin. The aims of this study were to determine serum pro-hepcidin levels in healthy preterm and term newborns, and to assess possible relationships between pro-hepcidin and serum iron, serum ferritin, and transferrin. A serum sample was collected from each of 26 healthy preterm (gestational age < 37 weeks) and 16 healthy, full-term, appropriate-for-gestational age babies. The preterm babies were also divided into 2 subgroups based on gestational age. Samples were analyzed for complete blood count, serum iron and ferritin concentrations, iron-binding capacity, and transferrin and pro-hepcidin levels. Group findings were compared and correlations between pro-hepcidin and the iron parameters were tested. The respective serum pro-hepcidin levels (mean +/- SD) in the 16 healthy term and 26 healthy preterm newborns were 482 +/- 371.9 ng/mL and 496.7 +/- 443.5 ng/mL. Analysis revealed no significant correlations between serum pro-hepcidin level and serum iron, serum ferritin, or transferrin in the preterm or term newborns. Pro-hepcidin levels were not correlated with gestational age in the preterm group. The results indicate that healthy preterm and term newborns have high pro-hepcidin levels.

Fatal course of ABO hemolytic disease associated with hydrops in a twin pregnancy.

Hydrops fetalis associated with ABO incompatibility is an extremely rare condition. We report twin infants both afflicted with significant ABO hemolytic disease but showing different degrees of clinical severity, in which fatal hydrops developed in one of the twins. Hemolysis due to ABO incompatibility is usually difficult to diagnose. All causes of non-immune hydrops should be ruled out in order to identify hydrops due to ABO incompatibility.

Evaluation of coagulation tests before newborn circumcision: is it necessary?

Evaluation of coagulation parameters prior to newborn circumcision is routinely performed in many countries. However, the value of this screening in predicting the bleeding risk is unknown. The aim of this study was to evaluate the correlation between the preoperative prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT) and excessive bleeding after the circumcision in term, healthy newborns without family history of coagulopathy. The medical records of healthy, full term newborns born at VKV American Hospital, in Istanbul, Turkey, between 2009 and 2012 who were circumcised within the first week of life, were reviewed retrospectively. The data for family history of coagulopathy, clinical sign(s) of bleeding during and/or after delivery, preoperative PT, aPTT levels and the amount of bleeding after circumcision were gathered. The most recent medical records of the patients' were also reviewed for any possible, lately diagnosed bleeding disorder. A total of 450 newborns met the above criteria. None had a family history of bleeding disorder or clinical bleeding. A total of 158 (35%) newborns had an aPTT result greater than 54.5 s, 269 (59%) had PT result greater than 15.9 s and 72 (16%) had international normalized ratio result greater than 1.62. Neither of the patients with prolonged PT and/or aPTT had prolonged or excessive bleeding. The evaluation of PT and aPTT before elective newborn circumcision is not necessary in the absence of clinical bleeding or a family history of bleeding disorder. It is rather a habit in general practice and possibly a result of defensive medicine.

Gonadal determination and adrenal development are regulated by the orphan nuclear receptor steroidogenic factor-1, in a dose-dependent manner.

The orphan nuclear receptor steroidogenic factor-1 (SF-1, NR5A1) regulates the transcription of multiple genes involved in steroidogenesis, reproduction, and male sexual differentiation. A heterozygous loss-of-function SF-1 mutation (G35E) has been described in a patient with adrenal failure and complete 46XY sex-reversal, indicating that haploinsufficiency of this factor is sufficient to cause a severe clinical phenotype. This mutation in the P-box region of the DNA-binding domain markedly impairs SF-1 binding to most response elements. In an infant with a similar clinical phenotype, we identified an SF-1 mutation (R92Q) in a highly conserved residue of the A-box, a region that functions as a secondary DNA-binding domain. Strikingly, the affected infant was homozygous for the R92Q mutation, but three relatives (parents, sister) were phenotypically normal despite being heterozygous for the mutation. In functional assays, the R92Q mutant exhibited partial loss of DNA binding and transcriptional activity when compared with the G35E P-box change, consistent with its phenotypic expression only when transmitted as a homozygous trait. Taken together, these two naturally-occurring SF-1 mutations reveal the relative functional importance of the P-box and A-box regions for monomeric binding by nuclear receptors. In addition, these patients reveal the exquisite sensitivity of SF-1-dependent developmental pathways to gene dosage and function in humans.

Persistent pulmonary interstitial emphysema in an unventilated neonate.

Persistent pulmonary interstitial emphysema (PPIE) is a chronic form of pulmonary interstitial emphysema. The disease is histologically distinguished by large cysts and giant cells. Our patient was a female twin who was born at 31 weeks of gestation with a birth weight of 1,450 g. A chest X-ray at 2 hr after delivery was normal. At 12 hr, respiratory distress developed, and nasal continuous positive airway pressure (CPAP) was initiated. A chest film revealed left-sided pneumothorax. A chest tube was inserted, and the baby continued on nasal CPAP for 5 days. Her chest X-ray on postnatal day 4 showed diffuse cystic changes in the left lung. Thoracic computed tomography revealed multiple thick-walled cysts, the largest measuring 3 cm in diameter. Our case confirms that localized PIE may occur in preterm infants who have been treated with nasal CPAP only. Since this method is being used increasingly to avoid mechanical ventilation and in the postextubation period, it is very important that clinicians be aware of its complications.

Factors influencing sleeping pattern of infants.

Many families apply to pediatricians with complaints of sleep problems of their infants. It is very important to inform families about the sleep pattern of infants and factors influencing it, and to answer their questions about infantile sleep. A questionnaire was given to 165 families to elucidate the factors influencing continuous sleep pattern of the baby. Our study demonstrated that 50% of four-month-old and 96% of nine-month-old infants acquired continuous sleep pattern. Our figures were compatible and even higher than those in the literature. None of the factors studied has an independent effect on infantile continuous sleep pattern in multifactorial analysis. Early weaning does not facilitate acquisition of continuous sleep pattern by the baby. This will help to support prolonged breast-feeding. It may be advisable not to keep children beside their mother during sleep for a long period of time. Care of the children by the maternal grandmother may facilitate their acquisition of continuous sleep pattern.

Factors influencing breastfeeding for working mothers.

In order to evaluate the relation between breastfeeding and working conditions a descriptive study was conducted on 301 working mothers. Seventy-seven percent of mothers breastfed their infants four months and longer, and the mean breastfeeding period was 6.2 +/- 3.4 months. Forty-one percent of mothers started weaning before four months of age. The multifactorial analysis of independent factors significantly influencing breastfeeding time were, in decreasing order of significance, breastfeeding conditions at work, maternal leave period, mother's smoking habit and the use of breastpump. For weaning period, these factors were the use of breastpump, breastfeeding leave at work and maternal leave period. In conclusion, in order to support breastfeeding at work, maternal leave period must be prolonged, and breastfeeding conditions at work must be improved.