Lung ultrasound in the evaluation of pulmonary edema in newborns with critical congenital heart disease.

BACKGROUND: Newborns with critical congenital heart disease (CCHD) with increased pulmonary blood flow (PBF) are at high risk for congestive heart failure. In this study, we aimed to evaluate the presence and degree of pulmonary edema in newborns with CCHD using lung ultrasound (LUS) during the perioperative period. METHODS: Prospective clinical trial, 44 newborn patients with CCHD were evaluated in this prospective clinical trial. LUS was repeatedly performed to determine the course of pulmonary edema during the perioperative period. LUS was performed simultaneously with chest radiography (CXR), which was the main part of patient management. The primary outcome of this study was to identify whether a correlation existed between LUS and CXR findings. The secondary outcomes were to determine the relationship between LUS and the need for respiratory support, diuretic use, vasoactive inotropic score (VIS), and pro-B-type natriuretic peptide (pro-BNP) levels during the perioperative period. RESULTS: The mean gestational age of the patients was 38.3 ± 1.7 weeks, with a mean birth weight of 3026 ± 432 g. In the preoperative period, both LUS and CXR images were consistent with clinical signs of pulmonary edema. On the first postoperative day, pulmonary edema increased compared to the preoperative period but gradually decreased by the 6th day of surgery (p < 0.05). Positive correlations were observed between the LUS and CXR findings at all study points (p < 0.05). The LUS findings exhibited trends parallel to those of VIS, serum pro-BNP levels, need for respiratory support, and diuretic requirements. As expected, these trends were more pronounced in CCHDs where PBF increased. CONCLUSION: In CCHD, serial lung ultrasound (LUS) assessments, particularly in cases with increased PBF, can provide valuable guidance for managing patients during the perioperative period.

Renal artery flow alterations in neonates with hypoxic ischemic encephalopathy.

BACKGROUND: To compare kidney blood flow and kidney function tests in infants with hypoxic ischemic encephalopathy (HIE), and the effects of therapeutic hypothermia (TH) during the first 7 days of life. METHODS: Fifty-nine infants with HIE were prospectively evaluated. Infants with moderate-severe HIE who required TH were classified as group 1 (n = 36), infants with mild HIE were classified as group 2 (n = 23), and healthy infants were classified as group 3 (n = 60). Kidney function tests were evaluated on the sixth hour, third and seventh days of life in Group 1 and Group 2, and on the sixth hour and third day of life in group 3. Renal artery (RA) Doppler ultrasonography (dUS) was performed in all infants on the first, third, and seventh days of life. RESULTS: Systolic and end diastolic blood flow in RA tended to increase and RA resistive index (RI) tended to decrease with time in group 1 (p = 0.0001). While end diastolic blood flow rates in RA on the third day were similar in patients with severe HIE and mild HIE, it was lower in patients with mild-moderate-severe HIE than healthy newborns. On the seventh day, all three groups had similar values (p > 0.05). Serum blood urea nitrogen (BUN), creatinine, uric acid, and cystatin C levels gradually decreased and glomerular filtration rate (GFR) gradually increased during TH in group 1 (p = 0.0001). Serum creatinine levels gradually decreased while GFR gradually increased during the study period in group 2. CONCLUSIONS: Therapeutic hypothermia seems to help restore renal blood flow and kidney functions during the neonatal adaptive period with its neuroprotective properties.

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.

The Effects of Thyroid Hormone Levels on Patent Ductus Arteriosus Closure in Newborns.

Objective: Although the role of thyroid hormones in functional and anatomical closure of patent ductus arteriosus (PDA) is well known, their effects on the medical or surgical closure of PDA in newborns remain unclear. This study aimed to assess the correlation between thyroid function tests and PDA closure through medical or surgical interventions in newborns. Methods: This retrospective study was conducted on 65 newborns diagnosed with hemodynamically significant PDA (hs-PDA), with a premature rate of 81.5% (n=53). The subjects were divided into two groups according to the nature of the ductal closure as medically responsive "MR-PDA" or surgically treated "ST-PDA". The groups were compared in terms of thyroid hormone levels and other clinical parameters. Results: Thirty-three (51%) of all 65 patients had PDA and responded to medical treatment. Gestational week, birth weight, and mode of delivery were similar between the medical and surgical treatment groups (p>0.05). Free thyroxine levels were significantly lower in the MR-PDA group than in the ST-PDA group (p=0.01). Conclusions: Because hs-PDA is associated with increased morbidity and mortality in the neonatal period, especially in premature infants, we hypothesize that thyroid hormone levels may play a role in the closure of hs-PDA.

Demographic and clinical characteristics of newborn pneumothorax patients and their effects on respiratory function tests during childhood.

PURPOSE: Pneumothorax is defined as the presence of air between the parietal and visceral leaves of the pleura, resulting in lung collapse. The aim of this study was to evaluate the respiratory functions of these patients when they reach school age and to reveal whether they cause permanent respiratory pathology. METHODS: The files of 229 patients who were hospitalised in a neonatal intensive care clinic had received a diagnosis of pneumothorax and had undergone tube thoracostomy were included in a retrospective cohort review. The respiratory functions of participants in the control and patient groups were evaluated using spirometry in a prospective cross-sectional study design. RESULTS: The study found the rates of pneumothorax to be higher in males, term infants and after caesarean delivery, mortality was 31%. Among patients who underwent spirometry, those with a history of pneumothorax had lower forced expiratory volume at timed intervals of 0.5 to 1.0 (FEV1), forced vital capacity (FVC), FEV1/FVC, peak expiratory flow (PEF) and forced expiratory flow 25-75% (MEF25-75). FEV1/FVC ratio was significantly lower (p < 0.05). CONCLUSION: Patients treated for pneumothorax in the neonatal period should be evaluated for obstructive pulmonary diseases during childhood using respiratory function tests.

Ultrasonographic evaluation of endotracheal tube position in newborns with CHD.

Many studies have been conducted to determine the most reliable technique for evaluating the position of the endotracheal tube in patients receiving mechanical ventilation support. In this study, we aimed to determine the endotracheal tube position by ultrasonography in intubated patients with a diagnosis of critical CHD followed in the neonatal ICU. METHODS: In this prospective observational clinical study, we performed point-of-care ultrasound for endotracheal tube localisation in 65 intubated newborns with critical CHD. After routine radiography, each patient underwent point-of-care ultrasound examination with a portable ultrasonography device for endotracheal tube end-carina measurement. Endotracheal tube end-carina measurements on chest radiographs were compared with ultrasound images. RESULTS: The mean gestational age and birth weight were 37.8 ± 2.19 weeks and 2888 ± 595 g, respectively. Ultrasound images were obtained after an average of 2.08 ± 1.6 hours from the radiographs. The average ultrasound time allocated to each patient was 5 minutes. The mean endotracheal tube tip-to-carina distance on chest X-ray and ultrasound were optimally 1.33 ± 0.64 cm and 1.43 ± 0.67 cm, respectively. There was no significant difference between chest X-ray and ultrasound measurements in endotracheal tube end-carina distance values evaluated by the Bland-Altman method (mean difference 0.10 cm, p = 0.068). There was a linear correlation between the endotracheal tube tip-carina distance in ultrasound and radiography evaluation (r2 = 0.60, p < 0.001). CONCLUSION: It has been concluded that critical CHDs are frequently accompanied by vascular anomalies, and the endotracheal tube tip-carina distance measurement can be used by determining the carina section as a guide point in the ultrasonographic evaluation of the endotracheal tube location in this patient population.

Accidental ingestion of 3% hydrogen peroxide in the neonatal period.

Hydrogen peroxide (H2O2) is an oxidizing agent. High concentrations of H2O2 are used in the chemical industry, and 3% concentrations are used in household disinfectants. Severe H2O2 toxicity occurs with a 35% concentration. After poisoning with H2O2, corrosive damage occurs. We describe a 3-day-old male who ingested H2O2 accidentally and was treated with supportive care. Hydrogen peroxide intoxication usually occurs in adults accidentally. This is the first report of a newborn case of H2O2 ingestion.

Evaluation of Newborns Diagnosed with COVID-19: A Single-Center Experience.

OBJECTIVE: The causative agent of the severe acute respiratory syndrome is a new type of coronavirus infection called coronavirus disease-2019 (COVID-19) which has spread around the world. COVID-19 is thought to rarely affect infants, so pandemic planning focuses on adults. This study aimed to share our 1-year experience with COVID-19-positive newborns in a tertiary neonatal intensive care unit (NICU). STUDY DESIGN: Hospitalized newborns after a reverse transcription polymerase chain reaction (RT-PCR) for COVID-19 were evaluated in this retrospective, cohort study between March 2020 and March 2021. The clinical course, laboratory tests, imaging studies, and outcomes of affected newborns were collected from medical records. RESULTS: Eleven COVID-19-diagnosed newborns were analyzed in our study. Four (36.6%) patients had contact with a COVID-19-positive individual in their families. The main symptoms were fever (81.8%), hypoxemia (63.6%), and tachypnea (63.6%). Bacterial coinfection was identified in two newborns. Aortic coarctation was detected as an accompanied disease by a newborn who was referred to our hospital with a suspicion of congenital heart disease and two patients had a bacterial coinfection. We did not detect any cases of neonatal multisystem inflammatory syndrome. All patients were discharged in good health. None of the newborns had any complications and repeated infection with another variant during the first-year follow-up. CONCLUSION: Contrary to popular belief, newborns are a sensitive age group for COVID-19 and need a high rate of hospitalization. Congenital heart diseases and coinfections should not be overlooked in COVID-19-infected newborns. The acquired immunity due to COVID-19 infection protects newborns from recurrent COVID-19 infections in their first year of life. The absence of COVID-19-positive individual in the family in most cases suggests that PCR-negative adults may also play a role in the transmission of the disease. KEY POINTS: · COVID-19 is still a pandemic all over the world.. · COVID-19 is dangerous for newborns.. · PCR-negative adults may also play a role in the transmission of the disease to the newborn..

Efficiency of Lung Ultrasonography in the Diagnosis and Follow-up of Viral Pneumonia in Newborn.

OBJECTIVE: Lung ultrasonography (LUS) is a useful method for diagnosis of lung diseases such as respiratory distress syndrome, transient tachypnea of the newborn, pneumonia, and pneumothorax in the neonatal period. LUS has become an important tool in the diagnosis and follow-up of lung diseases. LUS is easy to apply at the bedside and is a practical and low-cost method for diagnosing pneumonia. STUDY DESIGN: This study was conducted in neonatal intensive care unit of Dr. Sami Ulus Obstetrics, Children's Health and Diseases Training and Research Hospital. From September 2019 to April 2020, 50 patients who were diagnosed with viral pneumonia were included in the study. Also, 24 patients with sepsis-related respiratory failure were included in the study as a control group. LUS was performed at the bedside three times, by a single expert, once each before treatment for diagnosis, on discharge, and after discharge in outpatient clinic control. RESULTS: Before treatment, LUS findings were lung consolidation with air bronchograms (50/50), pleural line abnormalities (35/50), B-pattern (25/50), disappearance of lung sliding (21/50), lung pulse (5/50), and pleural effusion (9/50). During discharge, we found significant changes: lung consolidation with air bronchograms (6/50), pleural line abnormalities (7/50), B-pattern (12/50), and pleural effusion (1/50) (p < 0.05). Outpatient clinic control LUS findings were lung consolidation with air bronchograms (0/50), pleural line abnormalities (0/50), B-pattern (0/50), disappearance of lung sliding (0/50), and pleural effusion (0/50) (p < 0.05). Also, B-pattern image, disappearance of lung sliding, and pleural line abnormalities were higher in control group (p < 0.05). CONCLUSION: Ultrasound gives no hazard, and the application of bedside ultrasonography is comfortable for the patients. Pneumonia is a serious infection in the neonatal period. Repeated chest radiography may be required depending on the clinical condition of the patient with pneumonia. This study focuses on adequacy of LUS in neonatal pneumonia. KEY POINTS: · Lung ultrasound is a practical and low-cost method in diagnosing pneumonia.. · Neonatal pneumonia is a very important cause of morbidity and mortality in NICU.. · We can evaluate neonatal pneumonia with combination of clinical presentations and LUS findings..

Supraventricular tachycardia after respiratory syncytial virus infection in a newborn.

Respiratory syncytial virus (RSV) is a common cause of lower respiratory tract infections in newborn infants. RSV can cause cardiac arrhythmias. We present a case of a term newborn with supraventricular tachycardia that developed during RSV infection and was successfully treated with amiodarone. RSV infection increases the risk of cardiac arrhythmia due to its course and the agents used in the treatment; therefore, close cardiac monitoring is required.

Intravenous Immunoglobulin Use in Hemolytic Disease Due to ABO Incompatibility to Prevent Exchange Transfusion.

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion. Materials and Methods: This study evaluated the data of infants with ABO HDN in the Turkish Neonatal Jaundice Online Registry. The infants with ABO HDN who met the total serum bilirubin level inclusion criteria (within 2-3 mg/dL of exchange transfusion or even above exchange transfusion level) were included in the study according to the guidelines from the American Academy of Pediatrics and the Turkish Neonatal Society. All patients were managed according to the unit protocols recommended by these guidelines and received light-emitting diode (LED) phototherapy. Infants who only received LED phototherapy, and who received one dose of IVIG with LED phototherapy were compared. Results: During the study period, 531 term infants were included in the study according to inclusion criteria. There were 408 cases in the phototherapy-only group, and 123 cases in the IVIG group. The demographic findings and the mean bilirubin and reticulocyte levels at admission were similar between the groups (p > 0.05), whereas the mean hemoglobin level was slightly lower in the IVIG group (p = 0.037). The mean age at admission was earlier, the need for exchange transfusion was higher, and the duration of phototherapy was longer in the IVIG group (p < 0.001, p = 0.001, and p < 0.001, respectively). The rate of re-hospitalization and acute bilirubin encephalopathy (ABE) was higher in the IVIG group (p < 0.001 and p = 0.01, respectively). Conclusion: In this study, we determined that one dose of IVIG did not prevent an exchange transfusion nor decrease the duration of phototherapy in infants, who had bilirubin levels near or at exchange transfusion level, with hemolytic disease due to ABO incompatibility.

Role of systemic immune-inflammatory index in early diagnosis of sepsis in newborns with CHD.

OBJECTIVE: Congenital heart diseases (CHD) are the most common causes of birth defects that have increased the risk of infections. Neonatal sepsis is a life-threatening condition and early diagnosis can be life-saving. We aimed to evaluate the potential role of the systemic immune-inflammatory index in the early diagnosis of neonatal sepsis. METHODS: A retrospective cohort study was conducted on 166 newborns with a diagnosis of neonatal sepsis who were admitted to our hospital with CHD between January 2017 and June 2021. Haematological indices including neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and systemic immune-inflammatory index were calculated for all patients at the time of diagnosis of neonatal sepsis (sepsis). The sepsis values of these indices were compared with the admission values (pre-sepsis) of the patients. RESULTS: The mean gestational age and birth weight of the patients were 38.36 ± 1.42 weeks and 3057.75 ± 484.68 g. It was found that absolute neutrophil count, systemic immune-inflammatory index, neutrophil/lymphocyte ratio, but not platelet/lymphocyte ratio were significantly increased at the time of sepsis. The receiver operating characteristic curve showed that systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count have predictive ability to define neonatal sepsis among newborns with CHD. The systemic immune-inflammatory index produced an area under the curve receiver operating characteristic curve of 0.76 (70% sensitivity, 70.5% specificity). To discriminate neonatal sepsis, the cut-off values of systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count were 517.19, 2.62, and 9210/mm3, respectively. CONCLUSION: As an easily accessible and reliable indicator, systemic immune-inflammatory index may be used in combination with the other parameters in the early diagnosis of neonatal sepsis.

The Serum Levels of Asymmetric Dimethylarginine, Vascular Endothelial Growth Factor, and Insulin-Like Growth Factor-1 in Preterms with Retinopathy of Prematurity.

Objective:To determine the serum levels of asymmetric dimethylarginine (ADMA), vascular endothelial growth factor (VEGF), and insulin-like growth factor-1 (IGF-1) in preterms with retinopathy of prematurity (ROP). Materials and Methods: We included 37 preterm infants. The first blood samples were obtained within the first 5 days of life and repeated at the time of the first ophthalmologic examination for ROP. The levels of ADMA, IGF-1, and VEGF were measured in all samples. Results: ROP was detected in 12 of the subjects (32.4%). We categorized the subjects as non-ROP (Group 1; n = 25), untreated ROP (Group 2; n = 7), and treated ROP (Group 3; n = 5) according to the eye findings. There were no significant differences among the groups for serum levels of ADMA, VEGF, and IGF-1 at the first sampling. Conclusion: We did not find any differences in terms of serum ADMA, IGF-1, and VEGF levels in preterm infants with or without ROP.

Thiol-Disulfide Homeostasis in Neonatal Patients with Urinary Tract Infection.

OBJECTIVE: Urinary tract infection (UTI) is a disease that can cause significant complications in the neonatal period. The thiol-disulfide homeostasis is one of the important antioxidant defense mechanisms. The purpose of this study is to show the relationship between UTI and thiol-disulfide homeostasis in newborns. STUDY DESIGN: In this prospective study, 40 newborns with UTI and 40 healthy controls were included. Thiol-disulfide tests (disulfide, native thiol, and total thiol levels) and septic screening tests were performed before and after antibiotherapy in UTI group. The control group was selected from healthy newborns who applied to the outpatient clinic. RESULTS: The C-reactive protein and interleukin-6 levels were higher, while native thiol and native thiol/total thiol ratio were significantly lower in pretreatment group compared with posttreatment and control group. Also, the levels of disulfide, ischemia modified albumin, disulfide/native thiol ratio, and disulfide/total thiol ratio were higher in pretreatment group compared with posttreatment group. CONCLUSION: The thiol-disulfide homeostasis is an important indicator of oxidative stress during infections. It is valuable to be detected with small amounts of serum in newborns. These molecules can be used to support the diagnosis of UTI in the newborn. Further studies are needed to define the role of thiol-disulfide homeostasis in the UTI of newborn. KEY POINTS: · The thiol-disulfide homeostasis can be an important indicator of oxidative stress during infections such as UTI.. · The thiol-disulfide homeostasis of newborn is valuable to be detected with small amounts of serum in neonatal period.. · Laboratory tests such as white blood cell count, erythrocyte sedimentation rate, and C-reactive protein are not significantly different in UTIs..

The impact of therapeutic hypothermia on peripheral blood cell in newborns with hypoxic ischemic encephalopathy

Abstract The effect of hypothermia treatment on white blood cell (WBC), neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR) and platelet-to-lymphocyte ratio (PLR) values as an indicator of inflammation was evaluated in newborns with hypoxic ischemic encephalopathy (HIE). The study was performed that the before-therapeutic hypothermia (TH) and after-TH WBC, lymphocytes, neutrophils, monocytes and NLR, LMR and PLR values of the complete blood cell count were retrospectively evaluated. The results of the patient group were compared with the results of healthy newborns. A total of 78 patients who underwent TH were evaluated in our study. Mean values before and after TH were NLR3.8/2.7, LMR 5.6/8.6, and PLR 60.3/67.1 respectively. A statistical significance was present for NLR values before and after TH in those with seizure in our study (4.15±2.95/3.01±2.54) but no statistical significance was found for LMR or PLR. In neonates with HIE, effect of TH on complete blood cell count and inflammatory mechanisms (mediated neutrophil and lymphocyte) may be minimal.

Exchange transfusion for neonatal hyperbilirubinemia: A multicenter, prospective study of Turkish Neonatal Society.

Objective: The frequency of neonatal exchange transfusion has declined in recent years, but is still performed in many countries. The procedure is associated with complications. The aim of the study was to determine the clinical features and etiologies of infants with hyperbilirubinemia who underwent exchange transfusion and evaluate the adverse events and clinical outcomes. Material and Methods: We performed a secondary analysis of the multicenter Turkish Neonatal Jaundice Online Registry data. Otherwise healthy newborns born ≥35 weeks of gestation who were hospitalized for jaundice and underwent exchange transfusion were included. Results: One-hundred thirty-two patients with a mean serum bilirubin level on admission of 24.9±9.1 mg/dL were enrolled in the study. The most common cause for exchange transfusion was hemolytic jaundice (63.6%), followed by lack of proper feeding (12.9%). It was found that the infants with lack of proper feeding were discharged earlier from the maternity ward (p=0.02), but they were admitted to hospital later (p<0.001) with a higher bilirubin level (p=0.001), and geater weight loss (p=0.04). The reported rate of adverse events associated with exchange transfusion was 11.4%. The most common complication was thrombocytopenia (40%). None of the infants died during the procedure. Acute bilirubin encephalopathy was reported in 13 (9.8%) patients. Conclusion: Severe hyperbilirubinemia requiring exchange transfusion and acute bilirubin encephalopathy are still challenging problems in neonatal periodin our country. The policies including blood group analysis of pregnant women, programs informing parents about breastfeeding and jaundice, and monitoring bilirubin levels of high-risk newborns should be developed to reduce the necessitating for exchange transfusion and to avoid related complications.

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.

Genotypic and phenotypic features in Turkish patients with classic nonketotic hyperglycinemia.

Nonketotic hyperglycinemia is an autosomal recessive inborn error of glycine metabolism, characterized by deficient activity of the glycine cleavage enzyme system. Classic nonketotic hyperglycinemia is caused by mutations or genomic changes in genes that encode the protein components of the glycine cleavage enzyme system. We aimed to investigate clinical, biochemical, radiological findings and molecular genetic data in ten Turkish patients with classic nonketotic hyperglycinemia. Ten Turkish patients who were diagnosed with classic nonketotic hyperglycinemia in a single center from 2013 to 2019 were included in this study. Their clinical, radiological, electrophysiological and laboratory data were collected retrospectively. Sixty percent of the patients were in neonatal group, while 40 % of the patients were infantile. There were no late-onset patients. 90 % of the patients had the severe form. All patients had developmental delay and seizures. Mortality ratio was 30 % in all groups and 50 % in the neonatal group, while no mortality was seen in infantile group. Median (range) values of cerebrospinal fluid (CSF) glycine levels, plasma glycine levels and CSF/plasma glycine ratios were 148 (15-320) µmol/L, 896 (87-1910) µmol/L, 0.17 (0.09-0.21) respectively. Diffuse hypomyelination and corpus callosum anomaly were the most common cranial MRI findings and multifocal epileptic activity and burst supression pattern were the most common electroencephalographic findings. Six patients had variants in GLDC gene and four in AMT gene; five novel variants including AMT gene deletion were detected. Prognosis was poor and treatment was not effective, especially in the severe form. Classic nonketotic hyperglycinemia causes high morbidity and mortality. Neonatal-onset disease was more common and severe than infantile-onset disease. The ratio of AMT gene variants might be higher in Turkey than other countries. AMT gene deletion also plays a role in the etiology of classic nonketotic hyperglycinemia.

The prevalence and diagnostic criteria of health-care associated infections in neonatal intensive care units in Turkey: A multicenter point- prevalence study.

BACKGROUND: Healthcare-acquired infections (HAIs) in the neonatal period cause substantial morbidity, mortality, and healthcare costs. Our purpose was to determine the prevalence of HAIs, antimicrobial susceptibility of causative agents, and the adaptivity of the Centres for Disease Control and Prevention (CDC) criteria in neonatal HAI diagnosis. METHODS: A HAI point prevalence survey was conducted in the neonatal intensive care units (NICUs) of 31 hospitals from different geographic regions in Turkey. RESULTS: The Point HAI prevalence was 7.6%. Ventilator-associated pneumonia (VAP) and central line-associated bloodstream infections (CLABSI) and late onset sepsis were predominant. The point prevalence of VAP was 2.1%, and the point prevalence of CLABSI was 1.2% in our study. The most common causative agents in HAIs were Gram-negative rods (43.0%), and the most common agent was Klebsiella spp (24.6%); 81.2% of these species were extended spectrum beta-lactamase (ESBL) (+). Blood culture positivity was seen in 33.3% of samples taken from the umbilical venous catheter, whereas 0.9% of samples of peripherally inserted central catheters (PICCs) were positive. In our study, 60% of patients who had culture positivity in endotracheal aspirate or who had purulent endotracheal secretions did not have any daily FiO2 change (p = 0.67) and also 80% did not have any increase in positive end-expiratory pressure (PEEP) (p = 0.7). On the other hand, 18.1% of patients who had clinical deterioration compatible with VAP did not have endotracheal culture positivity (p = 0.005). CONCLUSIONS: Neonatal HAIs are frequent adverse events in district and regional hospitals. This at-risk population should be prioritized for HAI surveillance and prevention programs through improved infection prevention practices, and hand hygiene compliance should be conducted. CDC diagnostic criteria are not sufficient for NICUs. Future studies are warranted for the diagnosis of HAIs in NICUs.

The role of thiol-disulfide homeostasis in neonatal sepsis.

BACKGROUND AND AIM: The thiol-disulfide homeostasis is essential for the body to maintain effective antioxidant defense mechanisms. We aimed to show the relationship between sepsis and thiol-disulfide homeostasis in newborns. MATERIALS AND METHODS: In this prospective study, 66 newborns with sepsis (group 1) and 51 healthy controls (group 2) were included. In group 1, 53 newborns were diagnosed as clinical sepsis (group 1a) and 13 as definite sepsis (group 1b). The study has two time points; the day of diagnosis (step 1) and three days after the treatment (step 2). At step 1, group 1 and group 2 were compared for thiol-disulfide homeostasis as well as inflammatory markers. At step 2, the same laboratory tests were repeated only in group 1. RESULTS: At step 1, the levels of C-reactive protein (CRP) and interleukin-6 (IL-6) were higher, while native thiol and total thiol levels were lower in group 1 compared to controls. Serum disulfide/total thiol ratio was also significantly higher in group 1. When analyzed for subgroups of group 1, demonstration of microorganism did not affect the serum thiol levels. Within group 1, at step 2, although CRP and IL-6 levels were significantly lowered compared to step 1, we did not observe significant changes in thiol-disulfide parameters. CONCLUSIONS: The thiol-disulfide homeostasis may have a role in the pathogenesis of sepsis in newborns. The related parameters might be new markers for the diagnosis of sepsis in newborn patients. Further studies are needed to define the role of thiol-disulfide homeostasis in the course of neonatal sepsis.