Clinical significance of glucocorticoid receptor expression in premature infants with respiratory distress syndrome.

OBJECTIVE: Previous studies have shown the relationship between lung development and glucocorticoids, but no studies have been conducted to investigate if a relationship exists between respiratory distress syndrome (RDS) and glucocorticoid receptor (GR) expression in preterm babies. We intended to investigate whether low GR expression is a risk factor for RDS. METHODS: Forty-one preterm babies, 24-35 weeks of gestation, were included in the study following informed consent from the parents. The relative gene expression of GRalpha and GRbeta was measured in the peripheral mononuclear cells form cord blood samples. The demographic characteristics of the babies and the diagnosis of RDS were recorded. RESULTS: RDS was more frequent in the group with low GRalpha expression: 12 (60%) in the GRalpha-I group and 6 (28%) in the GRalpha-II group (p = 0.043). Oxygen use with a hood, time to reach full enteral feeds and the duration of neonatal intensive care unit stay was shorter, and nosocomial sepsis episodes and number of erythrocyte transfusions were less in the GRbeta-I group. Higher hospital costs were found in the GRbeta-II group. CONCLUSIONS: Less RDS development, and better clinical follow-up was observed in premature babies with higher GR expression.

Maternal and neonatal outcomes in advanced maternal age: a retrospective cohort study.

OBJECTIVES: To describe the effect of extremely advanced maternal age (EAMA) on maternal/neonatal outcomes. METHODS: This was a case-control study in which 127 women ≥40 years at the time of delivery out of 2853 singleton hospital deliveries in Ondokuz Mayis University between 1 January 2008 and 31 August 2010 constituted the study group. One hundred and twenty-seven else were chosen randomly out of 2412, 21-35 years old women, via a computer system as controls. Demographic features of 254 mothers and infants as well as maternal and neonatal complications were recorded. RESULTS: Mean maternal age was 41.5 ± 1.9 (40-49) years in EAMA group and 28.9 ± 4.2 (21-35) years in controls. Primigravidity was 19.6% in the EAMA group, whereas 37.8% in controls (p = 0.003). No difference was found between groups according to route of delivery, stillbirth, preterm birth, congenital abnormalities, gender of babies, NICU admission and respiratory problems (for all p > 0.05). A 5th min Apgar score <7 was more frequent in babies born to EAMA mothers compared to controls (9.8% versus 4.9%, p = 0.004). CONCLUSION: The present study shows that EAMA mothers and their offsprings have similar peri and neonatal risks compared to younger mothers, except lower 5th minute Apgar scores. We conclude that with good perinatal care, EAMA women and their babies can pass through the perinatal period with similar risks of younger women.

Assessment of different folic acid supplementation doses for low-birth-weight infants.

AIM: The adequacy of 50 mcg folic acid supplementation given to low-birth-weight babies was investigated. The folate levels of the mothers and infants, and breastmilk, and the optimum dose for folic acid supplementation were also investigated. MATERIAL AND METHODS: After obtaining blood from 141 low-birth-weight infants on the 1st day of life for serum and red cell folate levels, the infants were randomly allocated into three groups according to the folic acid supplement dose. Forty-six infants were given 25 µg/d folic acid, 39 were given 50 µg/d folic acid, and 44 were given 75 µg/d folic acid. Folic acid could not be given to 12 infants. Follow-up blood samples were obtained at the end of folic acid supplementation. Maternal samples for red cell and serum folate levels and breast milk folate levels were obtained within the first 48 hours and the samples for measuring breastmilk folate level were obtained on the 3rd day postnatally. The feeding modes of the infants, maternal folic acid intake, and details of neonate intensive care unit course were recorded. RESULTS: The mean birth weight and gestational age of the infants were found as 1788.2±478.4 g and 33.5±2.9 weeks, respectively. The mean serum and red cell folate levels on admission were found as 21.2±12.2 ng/mL and 922.7±460.7 ng/mL, respectively. The mean maternal serum and red cell folate levels and the mean breast milk folate levels were found as 12.3±7.5 ng/mL, 845.5±301.4 ng/mL, and 30.6±33.0 ng/m, respectively. The breast milk folate levels of mothers who were supplemented with folic acid during pregnancy were significantly higher compared with mothers who were not supplemented with folic acid (p<0.001). Infants who were supplemented with folic acid had higher follow-up serum folate levels compared with the basal level in all groups, but there was no statistically significant difference between the groups. CONCLUSION: This study showed that the folic acid doses of 25, 50, and 75 µcg/d affected serum folate levels similarly. We can conclude that the dose of 25 µcg/d is adequate for low-birth-weight infants.

Experience of a tertiary care center on 100 newborns with neural tube defects.

The aim of this study was to analyze the sociodemographic features, postoperative complications, long-term problems, and cost of care of patients followed in the neonatal intensive care unit (NICU) with a diagnosis of neural tube defects (NTDs). Babies with NTD followed in the Neonatology Unit of Ondokuz Mayis University Faculty of Medicine between January 2003 and December 2011 were analyzed retrospectively. One hundred (1.2%) of 8408 babies admitted to the NICU were diagnosed as NTD during the study period. Of the cases with NTD, 74% of mothers were graduates of primary school/illiterate, and none had used folic acid (FA) preconceptionally. Prenatal diagnosis was made in 72%, but parents had chosen not to terminate the pregnancy. The most frequent type and site of NTD was meningomyelocele (82%) of the lumbosacral region (36%). In 80% of the babies, the NTD sac was closed with in the first 72 hours of life. The most frequently observed postoperative complications were wound infection and septicemia. The mortality rate of babies with NTD during the follow-up period was 7%, and all deaths occurred in the first year of life. Sixty-two percent of the patients had neurologic deficits on follow-up. Patients were rehospitalized during the follow-up for an average of 2.9 times. Neural tube defect (NTD) is a disabling problem, with operations, rehospitalizations and other costly treatments. Maternal education regarding preconceptional FA use/fortification of food with FA and appropriate guidance to the family with prenatal diagnosis will decrease the incidence and burden of the disease.

Retinopathy of prematurity in extremely low birth weight infants in Turkey.

PURPOSE: This study was designed to determine the incidence rate of retinopathy of prematurity (ROP) in extremely low birth weight (ELBW) infants in the middle Black Sea region of Turkey. METHODS: The charts of 1,719 preterm infants were reviewed between January 2003 and September 2011. Of them, 225 (13.1%) infants with birth weights of less than 1,000 g were enrolled in the study. ROP was classified according to the international classification of retinopathy. RESULTS: Of the 225 ELBW infants, 47.6% had advanced ROP, 23.1% had mild ROP, and 29.3% did not have ROP; 30.2% infants required treatment. ROP was detected in 75.6% of multiple births versus 69.6% of singleton births; there were no significant differences between the two groups (P = .388). CONCLUSION: Extremely premature infants with low gestational ages had high incidence rates of advanced ROP. Most ELBW infants require ROP treatment. In addition, increased survival of ELBW infants owing to advancements in neonatal care warrants the specialization of ophthalmologists in regard to improved diagnosis and treatment of ROP.

Urinary neutrophil gelatinase-associated lipocalin in septic preterm babies: a preliminary study.

OBJECTIVE: This study was conducted to evaluate the predictive value of urinary neutrophil gelatinase-associated lipocalin (uNGAL) for acute kidney injury (AKI) among septic preterm infants. METHODS: Twenty-six very low-birth-weight (VLBW) babies were separated into three groups: group I, healthy preterms; group II, preterms with sepsis but without AKI; group III, preterms with sepsis and AKI. Demographic, clinical, and laboratory data of the babies were recorded. uNGAL and creatinine values were obtained on days 1, 3, and 7 of life. RESULTS: uNGAL levels differed statistically among three groups for all 3 days. Levels in group I (days 1, 3, and 7) were significant lower than levels in both groups II and III [median (interquartile range): 4.5 (10.8) µ/L, 8.7 (18.5) µ/L, and 4.3 (1.1) µ/L, respectively]. In group III, uNGAL levels on days 1 and 3 were significantly higher than levels in group II (p = 0.001, 0.016, respectively). CONCLUSION: First-day uNGAL levels were higher in VLBW preterm infants who later developed sepsis; whether the baby had AKI or not; but uNGAL levels were higher in septic babies with AKI compared with the infants without AKI. uNGAL is a promising early biomarker of AKI in VLBW infants with sepsis.

Is vitamin d deficiency a risk factor for respiratory distress syndrome?

BACKGROUND: Previous studies have shown the relationship between in utero lung development and vitamin D [25(OH)D], but there have been no studies to investigate whether vitamin D deficiency is a risk factor for respiratory distress syndrome (RDS) in preterm babies. OBJECTIVES: In this study, we investigated if 25(OH)D deficiency is a risk factor for RDS. METHODS: One hundred fifty-two preterm newborns, born at 29 - 35 weeks gestational age, were included in the study following informed consent from the parents. Peripheral blood samples were collected within the first 24 hours of life and 25(OH)D levels were measured by liquid chromatography-tandem mass spectrometry. Demographic characteristics of the babies and the diagnosis of RDS were recorded. RESULTS: In 64 % of preterm infants, 25(OH)D levels were compatible with severe deficiency (≤ 10 ng/mL), 33 % with moderate deficiency (10 - 20 ng/mL), and 3 % with mild deficiency (20 - 30 ng/mL). In none of the babies was a normal 25(OH)D level observed. Serum 25(OH)D levels were not correlated with gestational age. Respiratory distress syndrome was more common in preterm babies with severe (28 %) compared to mild-moderate 25(OH)D deficiency (14 %) (p < 0.05). CONCLUSIONS: None of the preterm infants in this study had normal vitamin D level, which underlined the burden of vitamin D deficiency in pregnant women and their offspring. RDS was more common in severely vitamin D-deficient preterms. Determination of vitamin D status of the mothers and appropriate supplementation might be a valuable strategy to reduce RDS, in addition to antenatal steroids. Besides, since vitamin D is a regulatory factor in many organs during fetal development, long-term effects of in utero vitamin D deficiency warrant further studies.

Kidney injury molecule-1 as a promising biomarker for acute kidney injury in premature babies.

OBJECTIVE: To evaluate the role of urinary kidney injury molecule-1 (uKIM-1) in early determination of renal injury in premature infants with respiratory distress syndrome (RDS). STUDY DESIGN: Forty-eight premature babies hospitalized in the neonatal intensive care unit were included in the study and divided into three groups: group I, healthy premature infants; group II, preterm infants with RDS without acute kidney injury (AKI); group III, preterm infants with RDS and AKI. uKIM-1 and creatinine along with serum creatinine levels were measured with enzyme-linked immunosorbent assay on days 1, 3, and 7 of life. RESULTS: On day 1, uKIM-1 levels in babies with RDS and AKI were higher than the other two groups. In this group, a significant increase in uKIM-1 levels were detected on day 3 (p = 0.015). The sensitivity and specificity of uKIM-1 were calculated as 73.3% and 76.9%, respectively, along with the increase of 0.5 ng per milligram of creatinine of uKIM-1 in day 3, when compared with values on day 1. Elevated uKIM-1 on day 7 was found to increase the risk of death by 7.3 times. CONCLUSION: Serial uKIM-1 measurements can be used as a noninvasive indicator of kidney injury and uKIM-1 can be an ideal biomarker in premature infants.

Screening for retinopathy of prematurity (ROP) in the middle Black Sea region of Turkey.

This study was designed to determine the frequency of retinopathy of prematurity (ROP) and the effectiveness of the screening protocol in preterm infants for our country. With these objectives, the charts of 1000 preterm infants were reviewed in Ondokuz Mayis University, Department of Ophthalmology. ROP frequency, the effect of gestational age (GA) and birth weight (BW) and the effectiveness of the screening protocol were evaluated. In this study, ROP was observed in 30.8% of infants and not observed in 69.2% of infants. Threshold ROP was detected in 7.0%. The frequency of threshold ROP was 43.5%, 20.0%, 12.6%, and 8.8% in the infants with GA of < or = 26, 26-28, 29-30, and 31-32 weeks, respectively. Threshold ROP was not observed in babies born after 34 weeks. Treatment was required for 11% of the infants. ROP treatment requirement (11%) was limited to babies with GA of < 34 weeks of gestation. Incidence of ROP was inversely proportional with GA and BW. Treatment was not required when GA was > 34 weeks. A new ROP screening protocol is proposed for Turkey, which is: screening of preterm babies with GA of < 34 weeks and BW of < 1800 g.

Temporary peritoneal dialysis in newborns and children: a single-center experience over five years.

AIM: To evaluate the indications, complications, and outcomes of temporary peritoneal dialysis (TPD) in children with acute renal failure (ARF). PATIENTS AND METHODS: All patients undergoing TPD between February 2006 and January 2011 in a children's hospital were included in the study. Patient characteristics, indications, complications, and duration of TPD (DPD), requirement of re-operation, length of stay, presence of sepsis, and outcome were recorded. RESULTS: There were 21 newborns (14 prematures), 9 infants, and 9 children. The main nephrotoxic agents were gentamicin (n = 7), netilmisin (n = 5), vancomycin (n = 3), and ibuprophen (n = 3). Patients with multiorgan failure (n = 9) had significantly higher blood urea nitrogen (BUN) and creatinine levels than those without multiorgan failure (n = 30) [BUN: 94 ± 27.3 vs. 34.3 ± 4.9) and creatinine: 4.1 ± 0.8 vs. 1.9 ± 0.2)]. The mean DPD was longer in mature patients than in prematures (newborn: 3.7; children: 7.1). Nine complications were observed (23%) (leakage in three and poor drainage in six patients). Twenty-five patients (64.1%) responded to TPD treatment and were discharged, and 14 patients (10 newborns and 7 of them were premature) died (35.9%). Mortality rate was higher in prematures (n = 7) and patients with a history of nephrotoxic agent (n = 10). CONCLUSION: TPD is effective especially in neonates with ARF and it is a reliable alternative to the hemodialysis or other continuous renal replacement therapies but it is not free of complications. It has limited effects, particularly in patients with multiorgan failure.

Simplified gyral pattern with cerebellar hypoplasia in Sedaghatian type spondylometaphyseal dysplasia: a clinical report and review of the literature.

We report on a patient with Sedaghatian type spondylometaphyseal dysplasia (SSMD) who presented with metaphyseal dysplasia, congenital atrioventricular block, simplified gyral pattern, hypogenesis of corpus callosum, and severe cerebellar hypoplasia. We want to emphasize that in this rare congenital lethal skeletal dysplasia with unknown etiology, central nervous system malformations might be a major component of the disorder and should be evaluated in detail to possibly uncover the underlying pathophysiology.

Two cases of fetus in fetu.

Fetus in fetu (FIF) is a rare cause of abdominal mass in children. One of the malformed monozygotic diamniotic twins is located in the body of other twin. It is differentiated from teratoma by the presence of vertebral organization with limb buds and other organ systems. Diagnosis is based on radiologic findings. Surgical excision is the treatment of choice, leading to the complete removal of the mass. To our knowledge, less than 200 cases have been described in the literature. Herein, we report 2 cases of FIF, a newborn who was diagnosed antenatally and a three-and-half-year- old boy diagnosed with mediastinal FIF after admission for recurrent respiratory tract infections.

Neonatal morbidity and mortality of late-preterm babies.

OBJECTIVE: To analyze neonatal morbidity and mortality rates of late-preterms and to compare them with their term counterparts in a tertiary care unit in Turkey. STUDY DESIGN: The study included 252 late-preterm newborns (34 0/7--36 6/7 weeks' gestational age), admitted to Neonatal Intensive Care Unit in the first 24 h of life between January 2005 and June 2007 and 252 newborns born in the same hospital in the same period of time. Babies with major congenital and/or chromosomal abnormalities were excluded. RESULTS: The mortality rate was 2.3% in late-preterms. None of the term newborns died. Compared to terms, late-preterms were 11 times more likely to develop respiratory distress, 14 times more likely to have feeding problems, 11 times more likely to exhibit hypoglycemia, 3 times more likely to be readmitted and 2.5 times more likely to be rehospitalized. Late-prematurity, being large for gestational age, male gender, and cesarean delivery were significant risk factors for respiratory distress. CONCLUSION: Late-preterms have significantly higher risk of morbidity and mortality compared with term newborns. Greater concern and attention is required for the care of this ignored, at-risk population.

Tear secretion and ferning patterns among premature and full-term newborns.

In this study, we aimed to assess the quality and quantity of tears among premature and term newborns. Tear ferning and Schirmer's tests were conducted over the first 10 days of life. Correlations between tear ferning patterns, Schirmer's scores, post-conceptional age, and birth weight were evaluated. Forty-six newborns (23 preterm, 23 term) were enrolled, with a mean post-conceptional age of 36.6 weeks and a mean weight of 2598.2 g. Mean total, basal and reflex Schirmer's test results were 13.5 mm, 6.7 mm and 6.8 mm, on the right and 14.0 mm, 7.1 mm and 6.9 mm on the left eye, respectively. Median ferning score was 2 (range 1-3) bilaterally. Schirmer scores were correlated with ferning capacity and post-conceptional age. Our study shows that newborns secrete moderate quantity, good quality tears. Ferning capacity, Schirmer scores and post-conceptional age are correlated.

Diffuse multicystic encephalomalacia in a preterm baby due to homozygous methylenetetrahydrofolate reductase 677 C-->T mutation.

Methylenetetrahydrofolate reductase catalyzes the formation of 5-methyltetrahydrofolate from 5,10-methylentetrahydrofolate and produces folate for the methylation of homocysteine to methionine. Due to insufficient conversion of homocysteine to methionine, plasma homocysteine levels increase in methylenetetrahydrofolate reductase deficiency. Homocysteine is an amino acid that contains a neurotoxic sulfur molecule and can induce neuronal apoptosis. Methylenetetrahydrofolate reductase deficiency is 1 of the etiological factors that causes neurological symptoms and signs in the newborn and childhood period. Here, we report a premature baby with prenatal onset diffuse multicystic encephalomalacia and cerebellar atrophy due to homozygous methylenetetrahydrofolate reductase mutation.

Reference values of cord blood transferrin, ceruloplasmin, alpha-1 antitrypsin, prealbumin, and alpha-2 macroglobulin concentrations in healthy term newborns.

Acute phase proteins are sensitive markers of tissue necrosis and inflammatory process. These markers may be especially useful in the neonatal period, in which mortality and morbidity rates are high, because fetus and baby are subjected to numerous metabolic, genetic, physiologic and environmental injuries such as neonatal asphyxia and septicemia. The purpose of the present study was to establish normal cord blood levels of some acute phase proteins in healthy term neonates. Umbilical cord blood was obtained at the time of vaginal delivery in 60 newborn infants (30 girls, 30 boys). Specific protein concentrations were measured by nephelometric assay. Transferrin, ceruloplasmin, alpha-1 antitrypsin, prealbumin, and alpha-2 macroglobulin concentrations [arithmetic mean (+/- SD)] were found to be 199.7 (+/- 34.6) mg/dl, 14.6 (+/- 4.0) mg/dl, 160.2 (+/- 23.6) mg/dl, 11.9 (+/- 2.2) mg/dl, and 284.6 (+/- 44.4) mg/dl, respectively. Prealbumin levels for girls [12.9 (+/- 2.2)] were found to be significantly higher than those of boys [10.9 (+/- 1.8)] (p < 0.001), while there were no significant differences between the other proteins. We conclude that these results may be used as reference values for the diagnosis of pathological conditions in newborns.

An unusual cause of acute renal failure in a newborn: hydrometrocolpos.

Here we report on an unusual cause of acute renal failure in a newborn: hydrometrocolpos due to imperforate hymen. Hymenotomy resolved all of the laboratory and clinical abnormalities of the patient and the baby was sent home healthy.

Timing of umbilical cord separation and neonatal intensive care unit practices.

We investigated the effects of antibiotic and corticosteroid treatment of the mother close to birth and postnatal neonatal intensive care unit (NICU) practices such as phototherapy, parenteral nutrition, and antibiotic treatment of the infant on umbilical cord detachment time. The impact of dry cord care on risk of omphalitis in NICU patients was also investigated. Cord separation time was longer in preterm babies. Antenatal antibiotic and corticosteroid use did not affect cord detachment time. The use of postpartum antibiotics, parenteral nutrition, and phototherapy delayed cord separation. Among these, antibiotic treatment was the most effective factor. Dry cord care did not increase the risk of omphalitis in NICU patients in our series.

Severe hypothyroidism caused by hepatic hemangioendothelioma in an infant of a diabetic mother.

Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than in babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothyroidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L-thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected.

Hypoxic-ischemic encephalopathy: correlation of serial MRI and outcome.

Twenty-four patients with hypoxic-ischemic encephalopathy were examined with serial magnetic resonance imaging up to 4 years of age. Magnetic resonance imaging studies were performed in the neonatal period, at the fourth month and the fourth year of age, and the findings were compared with the patients' neurodevelopmental outcome at the fourth year of age. Periventricular signal alterations and deep gray matter involvement were usually evident in the initial magnetic resonance imaging studies, and encephalomalacia, periventricular leukomalacia, and atrophy were the common findings on follow-up magnetic resonance imaging studies. In the patients with hypoxic-ischemic encephalopathy, some correlation between magnetic resonance imaging findings and neurodevelopmental outcome was recognized. The patients with deep gray matter involvement on the initial magnetic resonance imaging had a poor prognosis, and the ones with normal magnetic resonance imaging findings had a favorable neurodevelopmental outcome. On the follow-up magnetic resonance imaging findings, encephalomalacia and periventricular leukomalacia were associated with poor neurodevelopmental outcome. In predicting the neurologic outcome at 4 years of age, magnetic resonance imaging findings of the neonatal period had the highest negative predictive value, whereas magnetic resonance imaging findings at 4 months of age and 4 years of age had the highest positive predictive value.