Revolutionizing Neonatal Care: A Comprehensive Assessment of Neuromotor Development in At-Risk Infants Using the Novel Neonatal Infant Motor Assessment Scale (NIMAS) Test Battery.

We developed a new neonatal neuromotor test battery, the Neonatal Infant Motor Assessment Scale (NIMAS), to perform a detailed neuromotor and holistic assessment of at-risk infants in the neonatal period. METHODS: A total of 68 infants (28-41 Gestational weeks) hospitalised in the Neonatal Intensive Care Unit were included in the study. The NIMAS is a scale consisting of Automatic Motor Area, Functional Motor Area and sociodemographic form. The Dubowitz Neurological Examination and the Amiel-Tison Neurological Assessment Tests were also applied to evaluate the construct validity of the test. RESULTS: The mean gestational age at birth was 34.62 ± 3.07 weeks and birth weight was 2305.66 ± 738.95. Fifty-one (75%) of the babies were premature and 17 (25%) were term babies. The KMO value to test the adequacy of the distribution for factor analysis was found to be at a very good level. Barlett's test result was 2198.389 (p < 0.05). The amount of variance obtained as 44.76% in the study was at a sufficient level. The factor loads of the questions in the automatic motor domain dimension varied between 0.523 and 0.694 and the factor loads of the questions in the functional motor domain dimension varied between 0.619 and 0.772. Since Cronbach's alpha was above 0.70, the reliability was adequate. Inter-rater scale agreement in the automatic motor domain was 81.1%; scale agreement in the functional motor domFain was 92.9%; and the NIMAS total score agreement was 93.4%. These agreements were statistically significant (p < 0.05). Total correlation above 0.20 indicates that the item is important for the question. According to the results obtained, total correlation values were between 0.258 and 0.720. CONCLUSIONS: The NIMAS is the first test battery to assess the "Functional Motor Area" and this questionnaire, based on the results of the analyses, is a valid, reliable and clinically usable measurement tool for the infant at-risk at the neonatal period.

Epidemiology and Outcomes of Neonatal Meningitis: Results of the Turkish Neo-Meningitis Nationwide Study.

OBJECTIVE: To investigate the incidence and etiology of neonatal meningitis and to assess the associated risk factors, complications and outcomes in a nationwide multicenter retrospective descriptive study. METHOD: Twenty-seven centers from 7 geographical regions participated in the study. Newborns with a positive cerebrospinal fluid culture and/or cerebrospinal fluid polymerase chain reaction were included in the study. Demographic characteristics, clinical, laboratory and neuroimaging findings and mortality characteristics were analyzed. RESULTS: A total of 634 confirmed cases of neonatal meningitis were included in the final analysis. The incidence was 2.51 per 1000 intensive care unit hospitalizations and mortality was observed in 149 (23.5%). Gram-positive bacteria were the predominant pathogens (54.5%), with coagulase-negative Staphylococci accounting for 45.3% of the cases, followed by Gram-negative organisms (37.3%). Viral and fungal organisms were isolated in 3.2% and 1.7% of the infants, respectively. Gram-negative culture growth was more common in infants who died (51% vs. 34.6%; P < 0.001). In the multivariable model, the odds of mortality was higher in those with respiratory distress requiring invasive ventilatory support [odds ratio (OR): 10.3; 95% confidence interval (CI): 4.9-21.7; P < 0.01], hypotension requiring inotropes (OR: 4.4; 95% CI: 2.7-7.1; P < 0.001), low birth weight status (OR: 2.5; 95% CI: 1.4-4.6; P = 0.002), lack of exposure to antenatal steroids (OR: 2.4; 95% CI: 1.3-4.4; P = 0.005) and the presence of concomitant sepsis (OR: 1.9; 95% CI: 1.1-3.2; P = 0.017). CONCLUSIONS: In this nationwide study, neonatal meningitis was found to be associated with high mortality. Coagulase-negative Staphylococci was the most common causative microorganism followed by Gram-negative bacteria. Severe clinical presentation with invasive mechanical ventilation and inotrope requirement, as well as concomitant sepsis, low birth weight status and lack of exposure to antenatal steroids, were found to be independent risk factors for mortality.

An observational, multicenter, registry-based cohort study of Turkish Neonatal Society in neonates with Hypoxic ischemic encephalopathy.

BACKGROUND: Hypoxic ischemic encephalopathy (HIE) is a significant cause of mortality and short- and long-term morbidities. Therapeutic hypothermia (TH) has been shown to be the standard care for HIE of infants ≥36 weeks gestational age (GA), as it has been demonstrated to reduce the rates of mortality, and adverse neurodevelopmental outcomes. This study aims to determine the incidence of HIE in our country, to assess the TH management in infants with HIE, and present short-term outcomes of these infants. METHODS: The Turkish Hypoxic Ischemic Encephalopathy Online Registry database was established for this multicenter, prospective, observational, nationally-based cohort study to evaluate the data of infants born at ≥34 weeks GA who displayed evidence of neonatal encephalopathy (NE) between March, 2020 and April 2022. RESULTS: The incidence of HIE among infants born at ≥36 weeks GA (n = 965) was 2.13 per 1000 live births (517:242440), and accounting for 1.55% (965:62062) of all neonatal intensive care unit admissions. The rates of mild, moderate and severe HIE were 25.5% (n = 246), 58.9% (n = 568), and 15.6% (n = 151), respectively. Infants with severe HIE had higher rates of abnormal magnetic resonance imaging (MRI) findings, and mortality (p<0.001). No significant difference in mortality and abnormal MRI results was found according to the time of TH initiation (<3 h, 3-6 h and >6 h) (p>0.05). TH was administered to 85 (34.5%) infants with mild HIE, and of those born of 34-35 weeks of GA, 67.4% (n = 31) received TH. A total of 58 (6%) deaths were reported with a higher mortality rate in infants born at 34-35 weeks of GA (OR 3.941, 95% Cl 1.446-10.7422, p = 0.007). CONCLUSION: The incidence of HIE remained similar over time with a reduction in mortality rate. The timing of TH initiation, whether <3 or 3-6 h, did not result in lower occurrences of brain lesions on MRI or mortality. An increasing number of infants with mild HIE and late preterm infants with HIE are receiving TH; however, the indications for TH require further clarification. Longer follow-up studies are necessary for this vulnerable population.

Hypothermia is not therapeutic in a neonatal piglet model of inflammation-sensitized hypoxia-ischemia.

BACKGROUND: Perinatal inflammation combined with hypoxia-ischemia (HI) exacerbates injury in the developing brain. Therapeutic hypothermia (HT) is standard care for neonatal encephalopathy; however, its benefit in inflammation-sensitized HI (IS-HI) is unknown. METHODS: Twelve newborn piglets received a 2 µg/kg bolus and 1 µg/kg/h infusion over 52 h of Escherichia coli lipopolysaccharide (LPS). HI was induced 4 h after LPS bolus. After HI, piglets were randomized to HT (33.5 °C 1-25 h after HI, n = 6) or normothermia (NT, n = 6). Amplitude-integrated electroencephalogram (aEEG) was recorded and magnetic resonance spectroscopy (MRS) was acquired at 24 and 48 h. At 48 h, terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL)-positive brain cell death, microglial activation/proliferation, astrogliosis, and cleaved caspase-3 (CC3) were quantified. Hematology and plasma cytokines were serially measured. RESULTS: Two HT piglets died. aEEG recovery, thalamic and white matter MRS lactate/N-acetylaspartate, and TUNEL-positive cell death were similar between groups. HT increased microglial activation in the caudate, but had no other effect on glial activation/proliferation. HT reduced CC3 overall. HT suppressed platelet count and attenuated leukocytosis. Cytokine profile was unchanged by HT. CONCLUSIONS: We did not observe protection with HT in this piglet IS-HI model based on aEEG, MRS, and immunohistochemistry. Immunosuppressive effects of HT and countering neuroinflammation by LPS may contribute to the observed lack of HT efficacy. Other immunomodulatory strategies may be more effective in IS-HI. IMPACT: Acute infection/inflammation is known to exacerbate perinatal brain injury and can worsen the outcomes in neonatal encephalopathy. Therapeutic HT is the current standard of care for all infants with NE, but the benefit in infants with coinfection/inflammation is unknown. In a piglet model of inflammation (LPS)-sensitized HI, we observed no evidence of neuroprotection with cooling for 24 h, based on our primary outcome measures: aEEG, MRS Lac/NAA, and histological brain cell death. Additional neuroprotective agents, with beneficial immunomodulatory effects, require exploration in IS-HI models.

Turkish Neonatal Society Guideline on the Diagnosis and Management of Germinal Matrix Hemorrhage-Intraventricular Hemorrhage and Related Complications.

Germinal matrix hemorrhage-intraventricular hemorrhage (GMH-IVH) remains an important cause of brain injury in preterm infants, and is associated with high rates of mortality and adverse neurodevelopmental outcomes, despite the recent advances in perinatal care. Close neuroimaging is recommended for both the detection of GMH-IVH and for the follow-up of serious complications, such as post-hemorrhagic ventricular dilatation (PHVD). Although the question when best to treat PHVD remains a matter of debate, recent literature on this topic shows that later timing of interventions predicted higher rates of neurodevelopmental impairment, emphasizing the importance of a well-structured neuroimaging protocol and timely interventions. In this guideline, pathophysiologic mechanisms, preventive measures, and clinical presentations of GMH-IVH and PHVD will be presented, and a neuroimaging protocol as well as an optimal treatment approach will be proposed in light of the recent literature.

A Multicentered Study on Epidemiologic and Clinical Characteristics of 37 Neonates With Community-acquired COVID-19.

BACKGROUND: Coronavirus disease 2019 (COVID-19) primarily affects adults and spares children, whereas very little is known about neonates. We tried to define the clinical characteristics, risk factors, laboratory, and imagining results of neonates with community-acquired COVID-19. METHODS: This prospective multicentered cohort study included 24 neonatal intensive care units around Turkey, wherein outpatient neonates with COVID-19 were registered in an online national database. Full-term and premature neonates diagnosed with COVID-19 were included in the study, whether hospitalized or followed up as ambulatory patients. Neonates without severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) via reverse transcriptase-polymerase chain reaction testing or whose mothers had been diagnosed with COVID-19 during pregnancy were excluded. RESULTS: Thirty-seven symptomatic neonates were included. The most frequent findings were fever, hypoxemia, and cough (49%, 41%, 27%, respectively). Oxygen administration (41%) and noninvasive ventilation (16%) were frequently required; however, mechanical ventilation (3%) was rarely needed. Median hospitalization was 11 days (1-35 days). One patient with Down syndrome and congenital cardiovascular disorders died in the study period. C-reactive protein (CRP) and prothrombin time (PT) levels were found to be higher in patients who needed supplemental oxygen (0.9 [0.1-8.6] vs. 5.8 [0.3-69.2] p = 0.002, 11.9 [10.1-17.2] vs. 15.2 [11.7-18.0] p = 0.01, respectively) or who were severe/critical (1.0 [0.01-8.6] vs. 4.5 [0.1-69.2] p = 0.01, 11.7 [10.1-13.9] vs. 15.0 [11.7-18.0] p = 0.001, respectively). CONCLUSIONS: Symptomatic neonates with COVID-19 had high rates of respiratory support requirements. High CRP levels or a greater PT should alert the physician to more severe disease.

A case of laryngeal atresia accompanied by persistent pharyngotracheal ductus.

Laryngeal atresia is generally a fatal congenital anomaly with an incidence of 1: 50,000 births. This congenital anomaly is a condition of multifactorial inheritance, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphragm, fetal hydrops, and ascites. Diagnosis is usually made when there is failure to perform endotracheal intubation in a neonate with severe respiratory distress and absence of audible cry. Here, we present a very rare case of a newborn with laryngeal atresia who had respiratory distress and was sustained for the first few minutes of life using partial ventilation via a persistent pharyngotracheal duct. We would like to draw the attention of all physicians to this issue by reporting a rare fatal case of a newborn with a congenital presentation.

Laringeal atrezi, 50.000 dogumda bir görülen ve üst hava yolu tikanikligi ile giden ölümcül bir dogustan anomalidir. Çok etmenli kalitilir. Fetal ultrasonografide trakeada genisleme, akcigerlerde genisleme ve hiperekojenite, diyafragmada düzlesme ya da tersine dönme, hidrops ve asit saptanir. Dogumda agir solunum sikintisi olan yenidoganlarda endotrakeal entübasyonun basarilamamasi ve aglama çabasina ragmen ses duyulmamasi ile tani konulur. Bu yazida dogumdan sonra solunum sikintisi gelisen, ancak entübe edilemeyen, yasamin ilk dakikalarinda persistan faringotrakeal kanal yardimiyla kismi solunum yaparak hayatta kalabilen laringeal atrezili bir preterm olgu klinisyenlerin dikkatine sunuldu.

Early Retinal Findings Following Cooling in Neonatal Encephalopathy.

BACKGROUND AND AIM: Perinatal HI (hypoxia-ischemia)-related visual defects including blindness are known to be associated with ischemic lesions in intracerebral visual pathways and ischemic retinal damage (IRD). Intraocular hemorrhages (IOH) such as retinal hemorrhage (RH), which may result from perinatal HI, can cause IRD by various mechanisms. We aimed to evaluate the early retinal findings in neonates with moderate-to-severe neonatal encephalopathy (NE) who underwent TH and its relationship between coagulation status, amplitude-integrated electroencephalography (aEEG) patterns, and magnetic resonance imaging-magnetic resonance spectroscopy (MRI-MRS) findings. METHOD AND PATIENTS: A total of 31 newborn infants who underwent moderate-to-severe NE and TH included in the study. Coagulation parameters were taken immediately before starting TH, and daily during TH period. aEEG records were obtained during TH and rewarming period.Binocular indirect ophthalmoscopic examination (BIOE) and MRI-MRS scanning were performed when TH protocol completed. RESULTS: Total 13 (41.9%) patients had abnormal BIOE findings. Ten of them were (77%) IOH, other findings are as follows: RH (n = 7), optic disc hemorrhage (n = 2), and vitreous hemorrhage (n = 1). Initial coagulation status was not related to IOH. Worsened aEEG and MRI-MRS results were not related to BIOE findings. CONCLUSION: Frequency of IOH is high in newborns with NE who underwent TH being independent from severity of MRS-MRI findings, aEEG pattern, and disturbed coagulation status.

Short-term Refractive Effects of Propranolol Hydrochloride Prophylaxis on Retinopathy of Prematurity in Very Preterm Newborns.

PURPOSE: Retinopathy of prematurity (ROP) is one of the major problems of surviving premature infants with several ophthalmic morbidities such as increased risk of refractive errors, strabismus, and cortical visual impairment. Use of propranolol hydrochloride (PH) for the prevention of ROP is a new promising treatment modality. However, long-term effects are still to be defined. In our study, we aimed to investigate the short-term refractive effects of PH used for ROP prophylaxis in very preterm newborns. METHODS: This is a prospective, randomized, double-blind, placebo-controlled study. Very preterm newborns with a birthweight less than or equal to 1500 g and/or born prior to 32 gestational weeks were included in the study. The subjects were randomly divided into two groups: control group (CG, n = 37) given placebo and PH group (PHG, n = 34) given PH starting from 4 weeks after birth (27.1 ± 2.1 day). PHG patients received PH therapy for about 1 month (25.7 ± 7.8 day). Anthropometric measurements including weight, length, and head circumference were recorded before PH treatment (at birth) and during eye control (at corrected age). Cycloplegic refraction values were measured by retinoscopy at corrected age (CG: 10.3 ± 4.3 months, PHG: 11.4 ± 4.8 months). RESULTS: Anthropometric measurements including gestational age, weight, length, and head circumference were similar at birth and corrected age in both groups. The mean level of spherical refraction was significantly less hyperopic in the PHG than in the CG (CG: 1.37 ± 1.40 D, PHG: 0.37 ± 1.44 D) (p = 0.005). CONCLUSION: PH may lead to myopic shift by affecting the beta-adrenergic receptors in the choroid or ciliary body of the developing eye. Long-term refractive follow-up is required in order to elucidate the effects of PH on emmetropization process of these very preterm infants.

Platelet mass index can be a reliable marker in predicting the prognosis of retinopathy of prematurity in very preterm infants.

BACKGROUND: Retinopathy of prematurity (ROP) is a proliferative vitreoretinopathy resulting from vascular defect of the retina. The present study evaluates platelets, which are involved in VEGF storage, transport and release, and their functions with regard to the prognosis of the disease. The objective was to suggest a simple minimal invasive method that will facilitate the management of the disease and help clinicians in predicting the prognosis. METHODS: In this single center, retrospective, case-control study, we included a control group consisting of very preterm newborns (n = 83) at risk of ROP and a laser photocoagulation group including infants (n = 63) who received laser therapy during their follow-up examinations. The employed assessments included platelet counts and platelet mass index (PMI) which provide guidance in understanding platelet activity. In doing so, consideration was given to the first and second phases of ROP. The accuracy of prognostication was assessed with receiver operating characteristic analyses. RESULTS: The study groups did not differ statistically significantly by platelet count during the first and second phases of ROP (p > 0.05) nor were the PMI measurements statistically significantly different between the study groups during the first phase of the disease (p > 0.05). PMI values of the study groups, however, differed significantly in the second phase of ROP (p < 0.05). CONCLUSION: The present study found a significant difference between the two groups in PMI measurements which reflect increased VEGF levels during the neovascularization phase, which underlies the disease. This conclusion demonstrated that monitoring the PMI values in newborns at risk of ROP can be considered to be a minimally invasive method that by changing the retinal examination procedure in use today which is rather troublesome for both the physician and the newborn, can provide facilities in monitoring the disease for both the physician and the newborn.

Evaluation of aortic intima-media thickness in newborns with Down syndrome.

BACKGROUND: Health care programs for children with Down syndrome (DS) help improve the overall outcome and quality of life of these children. It is therefore very important to focus on the most common and serious problems of these patients, such as congenital heart defects and cardiac problems, and to keep medical guidelines updated with regard to these problems. OBJECTIVES: The aim of this study was to evaluate aortic intima-media thickness (aIMT), lipid profiles and blood pressure in DS patients in comparison with a control group of ageand gender-matched neonates without DS. MATERIAL AND METHODS: Serum concentrations of lipids (total cholesterol, LDL-cholesterol, HDL-cholesterol, and triglyceride), blood pressure and abdominal aIMT were measured in newborns with DS, and compared with the measurements from ageand gender-matched newborns without DS. RESULTS: No statistically significant differences between the 2 groups of newborns were detected with respect to aIMT, lipid levels or blood pressure. CONCLUSIONS: This study represents the first investigation of aIMT - one of the most important indicators of atherosclerosis - in DS patients, but neither a significantly increased aIMT, nor any significant changes in lipid profiles or blood pressure were detected in this group of patients. Whether aIMT differs according to the type of congenital heart defect (such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and atrioventricular septal defect) among DS patients remains to be determined in future studies.

The Efficacy of Propranolol in Retinopathy of Prematurity and its Correlation with the Platelet Mass Index.

PURPOSE: Retinopathy of Prematurity (ROP) is a proliferative vitreoretinopathy which is one of the most frequent causes of blindness in children. In an attempt to find a solution to this important problem in preterm children, the search for new, effective treatment modalities with fewer side effects is underway. In our study, which was planned for this reason, we aimed to investigate the effects of propranolol treatment applied to cases of ROP in various stages during the second phase (known as the neovascularization-hypoxia phase) and to determine the correlation of these effects with the platelet mass index (PMI). METHOD: A total of 171 preterm infants at risk of ROP were selected randomly for inclusion in the study. All of the patients were classified according to their stage of ROP and were divided into control and treatment groups. While the cases in the control group were administered physiological saline solution, those in the treatment group were administered propranolol in the period that corresponded to the second stage of the disease. The thrombocyte and PMI values in the first and second stages of each study group were recorded. RESULTS: A significant difference was found between the control and treatment groups of the stage 2 ROP study subjects. In the stage 2 ROP study group, no significant difference was detected between the control and treatment cases in terms of platelet counts in phase 1 or in the PMI values and the thrombolytic counts in phase 2. On the other hand, in phase 2 of the stage 2 ROP study subjects significant differences were detected between the control and treatment group in terms of PMI values. CONCLUSION: In the study, it was found in the stage 2 ROP study group that propranolol reduced the need for laser photocoagulation significantly. Also, in parallel to the efficacy of propranolol in this study group, a decrease was observed in PMI values.

Effect of parenterally L-arginine supplementation on the respiratory distress syndrome in preterm newborns.

L-Arginine (L-Arg) is the precursor of nitric oxide which plays an important role on pulmonary circulation and pulmonary vascular tone. Earlier studies suggested that L-Arg levels in preterm newborns with respiratory distress syndrome (RDS) were low due to its consumption and L-Arg supplementation may reduce the severity of RDS. Our aim was detect the effect of the parenterally L-Arg supplementation on RDS severity. The subjects were chosen between preterm newborns (gestational age <34 weeks) (n = 30). Twenty of the subjects were diagnosed with permaturity and RDS, and 10 of the subjects were healthy preterm newborns. Ten of the subjects was taken L-Arg (1.5 mmol/kg/d) in addition to routine RDS treatment and assumed as "Group 1". In this group, daily L-Arg supplementation was started end of the first day, and continued at end of fifth day. The others of the subjects diagnosed with RDS was take routine RDS treatment and assumed as "Group 2". Healthy preterm newborns assumed as "Group 3". Blood collections for L-Arg levels via tandem mass spectrometry were made in first day and repeated on the seventh days. Oxygenation index was used to determine severity of RDS. L-Arg consentrations in Group 1 were 8.7 ± 4.1 µM/L and 11.9 ± 5.0 µM/L in first and seventh day, respectively. L-Arg consentrations were 12.6 ± 4.5 µM/Land 10.9 ± 5.4 µM/L in Group 2 and 8.6 ± 5.1 µM/L and 9.4 ± 4.1 µM/L in Group 3. There is no correlation between L-Arg concentrations and OI also duration of the mechanical ventilation of the subjects in patient groups (Group 1 and 2).

Pentraxin 3 concentrations of the mothers with preterm premature rupture of membranes and their neonates, and early neonatal outcome.

BACKGROUND: Pentraxin 3 (PTX3) is an acute phase reactant which has been used to detect intra-amniotic infections (IAI) in pregnancy, but the prognostic value of PTX3 concentrations on neonates has not been studied. We aimed to investigate the relationship between maternal PTX3-neonatal PTX3 concentrations and early neonatal outcome. METHODS: The mothers diagnosed with preterm prelabor rupture of membranes (PPROM) (n = 28) and their preterm infants (n = 28) were included in the study. PTX3 concentrations were studied in plasma in the maternal peripheral blood and umbilical/peripheral vein in the neonates. The relationship between the mPTX3-nPTX3 concentrations and neonatal outcome were investigated using non-parametric tests and binary logistic regression analysis. RESULTS: The mean mPTX3 concentration was 10.35 ± 7.82 µg/L. Ten (35.7%) of all mothers were within the normal range and 18 (64.3%) in high percentile (≥ 97.5 percentile). There was no relation between mPTX3 concentrations and clinical or histologic chorioamnionitis, latency of PPROM, and early neonatal outcome. Mean nPTX3 concentrations was 9.18 ± 7.83 µg/L and high nPTX3 concentrations were detected in five (17.8%) neonates. nPTX3 concentrations were inversely correlated with gestational age and correlated with rate of intraventricular hemorrhage (IVH) and mortality. Neonates with high nPTX3 concentrations also have lowered APGAR scores, increased rate of respiratory distress syndrome, clinical sepsis, IVH, necrotizing enterocolitis and prolonged NICU stay. CONCLUSION: High PTX3 concentrations of the newborns are associated with some worsened early neonatal outcome including lower gestational age at delivery, increased rate of IVH and mortality. Maternal PTX3 concentrations are not an adequate marker in defining clinical or histologic chorioamnionitis and early neonatal outcome.

Investigation on malondialdehyde, S100B, and advanced oxidation protein product levels in significant hyperbilirubinemia and the effect of intensive phototherapy on these parameters.

BACKGROUND: The parameters of oxidative stress [advanced oxidation protein products (AOPPs), malondialdehyde (MDA), and S100B] and the effect of intensive phototherapy (PT) on these parameters have not been studied extensively in newborns with significant hyperbilirubinemia (SH). We aimed to measure the levels of MDA, S100B, and AOPPs in newborns with SH, and to compare newborns with healthy control newborns without hyperbilirubinemia on the basis of these parameters of oxidative stress. In addition, we investigated the effect of intensive PT on these parameters during the treatment of SH and report our findings for the first time in the literature. METHODS: The study was performed in newborns (n = 62) who underwent intensive PT because of SH. Newborns without jaundice constituted the control group (n = 30). Both groups were compared with respect to demographic characteristics and biochemical (laboratory) parameters including MDA, AOPPs, and S100B. MDA, AOPPs, and S100B were also compared before and after intensive PT in the PT group. In the study group, a correlation analysis of demographic characteristics; MDA, AOPP, and S100B values; and changes occurring in MDA, AOPPs, and S100B values due to the effect of intensive PT was performed. RESULTS: Serum total bilirubin, S100B, and MDA levels in the PT group before performing PT were significantly higher than those in the control group. In newborns receiving PT serum total bilirubin, MDA and AOPP levels decreased significantly after intensive PT. In correlation analysis, a statistically significant negative correlation was found only between the amount of bilirubin decrease with PT and AOPP levels after PT in the study group. CONCLUSION: Whether the significant decrease in MDA levels, which was higher prior to PT, is due to the decrease in serum bilirubin levels or due to the effect of intensive PT itself remains to be determined in further studies. The decrease in AOPP levels after PT implies that intensive PT has protective effects on oxidative stress.

Quantitative assessment of hepatic blood flow in the diagnosis and management of necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most important gastrointestinal emergency in the neonatal period and early detection is very important for its management. Bowel ischemia-hypoperfusion is one of the main etiological factors. In the literature, a few studies have focused on arterial Doppler ultrasonography (DUS) features of splanchnic arteries; however, their clinical implications are not clear. OBJECTIVE: In this study, we aimed to quantitatively evaluate the blood flow features in the hepatic portal vein (PV) and hepatic veins (HVs) by using DUS in newborns with NEC. Patient-Method: Enrolled subjects were divided into two groups as patient (suspected/confirmed NEC, n = 24), and control group (n = 25). Daily serial DUS examinations were performed after the onset of the suspicion of NEC and continued until the initial day of the enteral feeding. Portal blood flow (PBF) and "hepatic blood flow ratio" (RHBF) were calculated manually by using DUS findings. Two groups were compared with respect to their PBF and RoHBF values. RESULTS: PBF and RHBF levels were significantly lower in patient group than those in control group. Clinical improvement in patients with NEC was associated with improvement in the PBF and RHBF. Cut-off level of the RHBF for the diagnosis of NEC was 0.66. CONCLUSION: DUS seems to be useful for the diagnosis and follow-up of NEC by providing quantitative information on liver blood flow. Daily measurements of the PBF and RoHBF in newborns with NEC may be beneficial to make the decision of starting enteral feeding.

A Neonate with CLOVES Syndrome.

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet).

Unusual course of congenital hypothyroidism and route of the L-thyroxine treatment in a preterm newborn.

Congenital hypothyroidism (CH) is the most common endocrine pathology in neonates. Inappropriate treatment of CH is complicated by irreversible brain damage or low IQ score. Hormone replacement therapy with L-thyroxine (L-T4) is sufficient for a very large proportion of patients. However, during treatment, the patient needs to be carefully monitored for presence of factors which might affect the absorption or bio-availability of the drug as well as its dose. Herein, we report a preterm newborn with CH who presented with gastrointestinal problems mimicking necrotizing enterocolitis. The clinical course was also complicated by cholestasis. The L-T4 replacement treatment was switched from oral route to parenteral. After resolution of the cholestasis, L-T4 treatment was continued successfully by the oral route.

Expanding the mutational spectrum of CRLF1 in Crisponi/CISS1 syndrome.

Crisponi syndrome (CS) and cold-induced sweating syndrome type 1 (CISS1) share clinical characteristics, such as dysmorphic features, muscle contractions, scoliosis, and cold-induced sweating, with CS patients showing a severe clinical course in infancy involving hyperthermia associated with death in most cases in the first years of life. To date, 24 distinct CRLF1 mutations have been found either in homozygosity or in compound heterozygosity in CS/CISS1 patients, with the highest prevalence in Sardinia, Turkey, and Spain. By reporting 11 novel CRLF1 mutations, here we expand the mutational spectrum of CRLF1 in the CS/CISS1 syndrome to a total of 35 variants and present an overview of the different molecular and clinical features of all of them. To catalog all the 35 mutations, we created a CRLF1 mutations database, based on the Leiden Open (source) Variation Database (LOVD) system (https://grenada.lumc.nl/LOVD2/mendelian_genes/variants). Overall, the available functional and clinical data support the fact that both syndromes actually represent manifestations of the same autosomal-recessive disorder caused by mutations in the CRLF1 gene. Therefore, we propose to rename the two overlapping entities with the broader term of Crisponi/CISS1 syndrome.

Endocrine abnormalities of patients with cleft lip and/or cleft palate during the neonatal period.

BACKGROUND/AIM: There are close interactions among the developing oral cavity, pituitary gland, and central nervous system (CNS) in early embryonic life. In this study we aimed to screen endocrine abnormalities in patients with orofacial clefts in the neonatal period. MATERIALS AND METHODS: Thirty-one patients with isolated orofacial median clefts wereincluded in the study. Pituitary, thyroid, and adrenal hormones were measured at the first week and remeasured in the third or fourth weeks. Imaging studies were done for detection of CNS anomalies in all patients. RESULTS: Endocrine abnormality was detected in 22 (70.9%) patients. The number of patients with single and multiple endocrine abnormalities were 13 (41.9%) and 9 (29%), respectively. Thyroid hormone-related disorders were detected in 10 (32.3%) patients. Growth hormone deficiency was detected in 4 (12.9%) patients. Adrenocorticotrophic hormone and/or glucocorticoid deficiency was detected in 5 (16.1%) patients. Neonatal hypoglycemia due to endocrinological abnormalities was detected in 6 (19.4%) patients. Defected mini-puberty was seen in 2 (15.4%) patients. There was no relationship between the types of orofacial cleft and endocrine abnormalities. CONCLUSION: Endocrinological evaluation of the patients with orofacial clefts in the neonatal period is a worthwhile endeavor to detect hormone deficiencies regardless of the type of the cleft.