Lung ultrasound score in the decision of patent ductus arteriosus closure in neonates.

PURPOSE: We aimed to investigate the role of lung ultrasound (LUS) score in the closure of hemodynamically insignificant patent ductus arteriosus (PDA) and the clinical findings of the patients before and after closure. METHODS: The study groups (107 preterm neonates under 34 gestational weeks) were classified as hemodynamically significant PDA (group 1), hemodynamically insignificant PDA with closure therapy (group 2), hemodynamically insignificant PDA without closure therapy (group 3), and no PDA group (group 4) based on the echocardiography. 6- and 10-region LUS scores were compared for each group. RESULTS: There was a significant difference between groups 1 and 3 on first, third, and seventh days. In contrast, groups 1 and 2 had similar LUS scores on the first, third, and seventh days. There was a negative correlation between LUS scores on the first and third days and gestational age, birth weight, the first- and fifth-minute APGAR scores, and there was a positive correlation between aortic root to left atrium ratio, and PDA diameter/weight ratio. CONCLUSION: We observed that LUS scores in patients with hemodynamically insignificant PDA treated with closure therapy were similar to in patients with hemodynamically significant PDA. Thus, LUS score can have role in PDA closure in preterm neonates. However, more comprehensive studies are needed.

Factors predicting kidney replacement therapy in pediatric earthquake victims with crush syndrome in the first week following rescue.

Crush syndrome due to traumatic rhabdomyolysis is one of the most significant problems to occur following earthquakes. On February 6, 2023, millions of people in Turkey were affected by two consecutive Kahramanmaras earthquakes. The present study reports the analysis of clinical and laboratory findings of crush syndrome in pediatric earthquake victims admitted to our hospital from our region where the earthquake had a devastating effect. Clinical and laboratory findings concerning earthquake victims with crush syndrome were analyzed within the first week to determine what factors are predictive of kidney replacement therapy (KRT). The data of patients were retrospectively collected from medical records. A total of 310 children were admitted as earthquake victims to the pediatric emergency department. Ninety-seven (31%) of these patients had crush syndrome. Fifty-three (55%) of those with crush syndrome were female. The mean age was 10.9 ± 4.7 years, and the mean time under the rubble was 30.6 ± 23.8 h. Twenty-two patients (23%) required KRT. Hemodialysis was applied to 16 (73%) of them, and hemodiafiltration was applied to the other six (27%) in the pediatric intensive care unit. Regarding creatine kinase (CK) levels, the area under the receiver operating characteristic (ROC) curve (AUC) for predicting KRT was 0.905 (95% confidence interval [CI] 0.848-0.963; p < 0.001). The optimal cut-off value was 40,000 U/L with a sensitivity of 86% and a specificity of 83%. In terms of the percentage of body area crushed, the AUC for predicting KRT was 0.907 (95% CI 0.838-0.976; p < 0.001). The optimal cut-off value was 30% with a sensitivity of 86% and a specificity of 88%. Multiple logistic regression analysis showed that each 10% increase in body area crushed (OR 4.16, 95% CI 1.58-10.93, p = 0.004) and 1 mg/dl increase in the serum phosphorus level (OR 4.19, 95% CI 1.71-10.28, p = 0.002) were significant risk factors for dialysis treatment. CONCLUSIONS: Crush syndrome and kidney problems are common following disasters like earthquakes. Clinical and laboratory findings at admission can predict dialysis requirement in earthquake victims. While CK elevation, body area crushed percentage, and increased phosphorus level were predictive of dialysis treatment, time under the rubble was not. Even if the patients were under the rubble for a short time, acute kidney injury (AKI) may develop as a result of severe hypovolemia due to crush injuries, and patients may need KRT. WHAT IS KNOWN: •Crush syndrome after earthquakes needs to be treated carefully in victims and can cause AKI and mortality when not treated timely and appropriately. WHAT IS NEW: •CK level elevation, body area crushed percentage, and increased phosphorus level are predictive of dialysis treatment. •The time under the rubble may not be predictive of dialysis requirement.

A homozygous MED11 C-terminal variant causes a lethal neurodegenerative disease.

PURPOSE: The mediator (MED) multisubunit-complex modulates the activity of the transcriptional machinery, and genetic defects in different MED subunits (17, 20, 27) have been implicated in neurologic diseases. In this study, we identified a recurrent homozygous variant in MED11 (c.325C>T; p.Arg109Ter) in 7 affected individuals from 5 unrelated families. METHODS: To investigate the genetic cause of the disease, exome or genome sequencing were performed in 5 unrelated families identified via different research networks and Matchmaker Exchange. Deep clinical and brain imaging evaluations were performed by clinical pediatric neurologists and neuroradiologists. The functional effect of the candidate variant on both MED11 RNA and protein was assessed using reverse transcriptase polymerase chain reaction and western blotting using fibroblast cell lines derived from 1 affected individual and controls and through computational approaches. Knockouts in zebrafish were generated using clustered regularly interspaced short palindromic repeats/Cas9. RESULTS: The disease was characterized by microcephaly, profound neurodevelopmental impairment, exaggerated startle response, myoclonic seizures, progressive widespread neurodegeneration, and premature death. Functional studies on patient-derived fibroblasts did not show a loss of protein function but rather disruption of the C-terminal of MED11, likely impairing binding to other MED subunits. A zebrafish knockout model recapitulates key clinical phenotypes. CONCLUSION: Loss of the C-terminal of MED subunit 11 may affect its binding efficiency to other MED subunits, thus implicating the MED-complex stability in brain development and neurodegeneration.

Effect of Short-Term Morbidities, Risk Factors and Rate of Growth Failure in Very Low Birth Weight Preterms at Discharge.

BACKGROUND: Very low birth weight (VLBW) infants often demonstrate postnatal growth failure (PGF). We aimed to analyze incidence and risk factors for PGF in surviving VLBW infants hospitalized more than 28 days. MATERIALS AND METHODS: Fenton growth chart (2013) was used for Z-scores for birth weight (BW) and discharge weight. Infants with a decrease in their Z-scores at discharge >1 were considered as 'PGF group' and with a decrease >2 were considered as 'severe PGF group'. RESULTS: One hundred and forty-one of 148 (95.3%) infants had PGF, 88 of 141 (62.4%) had severe PGF. There were significant differences in gestational age, birth and discharge weight, and days to regain BW, age of first and full enteral feeding, duration of parenteral nutrition, lipid emulsions, intubation and hospitalization between groups (p < 0.05). Vasopressor treatment, nosocomial infection, patent ductus arteriosus and bronchopulmonary dysplasia rates were significantly higher in severe PGF group (p < 0.05). CONCLUSION: PGF remains a serious problem in our unit. All VLBW preterm infants should be followed for PGF.

Pseudomonas aeruginosa infections due to electronic faucets in a neonatal intensive care unit.

AIM: To evaluate the role of electronic faucets in a newborn intensive care unit during a Pseudomonas aeruginosa outbreak. METHODS: After three patients had P. aeruginosa bacteremia, environmental cultures including those from patient rooms, incubator, ventilators, total parenteral nutrition solutions, disinfection solutions, electronic and hand-operated faucet filters/water samples after removing filters and staff hands were taken. RESULTS: Only filters of electronic faucets and water samples after removing filters and one liquid hand soap showed P. aeruginosa (3-7 × 106 cfu/mL). We have removed the electronic faucets and new elbow-operated faucets were installed. Pulsed-field gel electrophoresis analysis of outbreak-blood culture isolates from two patients and isolates from electronic water faucets/one liquid hand soap indicated the presence of 90.7% genetically related subtype, probably from the same clone. Water cultures from new faucets were all clean after installation and after 7 months. CONCLUSION: We suggest that electronic faucets may be considered a potential risk for P. aeruginosa in hospitals, especially in high-risk units.

Two newborns of heroin-addicted mothers suffering neonatal withdrawal syndrome.

Neonatal withdrawal syndrome is characterized by non-specific signs and symptoms that occur in infants following in-utero drug exposure. The incidence of neonatal withdrawal syndrome is 16-90% in infants of mothers abusing heroin. Clinical signs of withdrawal syndrome usually occur within the first 48-72 hours after birth. Central nervous system and gastrointestinal system symptoms are the main symptoms. In this case report, two newborns born to the mothers addicted to heroin who suffered neonatal withdrawal syndrome are presented. They were successfully treated with phenobarbital and morphine infusion.

Birth weight, head circumference, and length of newborns are unaffected by maternal levels of vitamin B12 and folate.

Vitamin B12, folate, and other micronutrients are essential for healthy growth. We hypothesized that there is a high prevalence of vitamin B12 deficiency in mothers and their newborns, and that blood serum vitamin B12 and folate levels may affect anthropometric measurements at birth. A total of 204 newborn babies and their 196 mothers were included. Blood samples of newborns and mothers were obtained for vitamin B12 (<200 pg/mL) and folate (<3 ng/mL) deficiencies. Additionally, iron and ferritin levels were measured. The mean gestational age and birth weight were 37.2 ± 2.6 (22.3-41) weeks and 3045 ± 770 (505-4525) g, respectively. All micronutrient levels in cord blood were higher than maternal levels (P = .001). A total of 96.3% of mothers and 64.5% of babies had vitamin B12 deficiency; 4% of mothers and none of the infants had folate deficiency. In total, 38.2% of mothers and 10.6% of infants had ferritin deficiency and 38.7% of mothers and 41.4% of newborns had iron deficiency. There was a negative correlation between cord vitamin B12 level and birth weight and head circumference (r = -0.21, P = .004 and r = -0.16, P = .036, respectively), whereas no correlation was found between maternal micronutrient status and anthropometric measurements of newborns. In conclusion, anthropometric measurements were unaffected by maternal levels, but vitamin B12 deficiency is very common in pregnant women and newborn babies. Mothers and their infants may benefit from early diagnosis and treatment. Awareness of vitamin B12 deficiency in pregnant women and newborns should be increased in Turkey.

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.

Healthcare-associated infections in a neonatal intensive care unit in Turkey in 2008: incidence and risk factors, a prospective study.

In this study, we have prospectively recorded healthcare-associated infections (HAIs) in NICU and found incidence density as 18 infections per 1000 patient days. Of the infections, 51.3% was bacteriemia (BSI), and 45.1% was ventilator-associated pneumonia (VAP). Gram-negative microorganisms were predominant in VAP and Staphylococcus epidermidis was the leading microorganism (53.0% of BSIs) in BSIs. Multivariate logistic regression analysis showed the importance of hood O(2) use in days (RR: 1.3) and total parenteral nutrition use in days (RR: 1.09) for BSIs. Umbilical arterial catheterization in days (RR: 1.94), ventilator use in days (RR: 1.05), chest tube (RR: 12.55), orogastric feeding (RR: 3.32) and total parenteral nutrition in days (RR: 1.05) were found to be significantly associated with VAP. In conclusion, incidence density in our unit is high and Gram-negative rods are predominant similar to developing countries. These results strongly suggest improving measures of prevention and control of HAIs in the unit.

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.

A 6-year prospective surveillance of healthcare-associated infections in a neonatal intensive care unit from southern part of Turkey.

AIM: To report the incidence of healthcare-associated infections (HAIs), site of infection and bacterial epidemiology in the Neonatal Intensive Care Unit in a university hospital in Adana, Turkey, between 2001 and 2006. METHODS: During these years, HAIs were collected by an active surveillance system. RESULTS: Five hundred one of 2832 infants hospitalised more than 72 h had 1124 HAI. The HAI incidence and incidence density ranged between 14.1 and 29.7 infections/100 patients, and 10.9-17.3 infections/1000 patient days within the study period; 61.5% of HAIs were ventilator-associated infections; 26.2% were bloodstream infections; 3.5% were urinary tract infections; 3.5% were necrotising enterocolitis (Stages II and III) and 1.4% was meningitis. The most frequent pathogens were gram-negative pathogens (75.6% of all infections) followed by gram-positive micro-organisms (21.4%) and Candida species (3.0%). Birthweight, gestational age and Apgar scores were lower and overall mortality rate (32.9% vs. 19.7%) and number of inpatient days were higher in patients with HAIs (for all P<0.001) when compared with those who did not have HAIs. Furthermore, HAI rate was inversely related to birthweight (P<0.001). CONCLUSION: In this study, the overall infection rate is high compared with developed countries and predominant micro-organisms are gram-negative enteric rods. These results strongly suggest the need for improving measures for prevention and control of HAIs in this hospital.

The Turkish Neonatal Society proposal for the management of COVID-19 in the neonatal intensive care unit.

Due to immaturity of immune function and the possibility of mother-fetal vertical and aerosol transmissions, neonates are particularly susceptible to the new coronavirus (SARS-CoV-2). Perinatal-neonatal departments should cooperate closely and take integrated approaches, and neonatal intensive care units (NICU) should prepare emergency plans for the coronavirus disease 2019 (COVID-19) as far as possible, so as to ensure the optimal management and treatment of potential victims. During the epidemic of COVID-19, the emergency response plan for the NICU should be based on the actual situation, including diagnosis, isolation, and treatment, as well as available equipment and staffing, and take into account the psychosocial needs of the families and neonatal care staff. In this context of the COVID-19 pandemic, the Turkish Neonatal Society has proposed a protocol with the evidence available at the time of preparation to handle neonates with SARS-CoV-2 infections and outbreaks in NICUs. We hope that this proposal can provide valuable information so medical workers do not have to enter the battlefield alone. At this moment, sharing resources, experiences and lessons, regardless of who you are, is our only chance to win.

A neonate with homozygous protein C deficiency with a homozygous Arg178Trp mutation.

Homozygous protein C deficiency affects approximately 1/400,000 to 1/1,000,000 live births. Homozygous protein C deficiency is associated with catastrophic and fatal purpura fulminans-like or thrombotic complications and disseminated intravascular coagulation. In the present patient, genetic study revealed Arg178Trp, a mutation found widely in European population; but this is the first case of homozygous Arg178Trp mutation who suffered from catastrophic purpura fulminans phenotype.

A newborn infant with intrapericardial rhabdomyosarcoma: a case report.

Cardiac tumors are uncommon in neonates and most of them are histologically benign. The most common cardiac tumor in neonates and infants is rhabdomyoma. Malignant cardiac tumors are considerably rarer, and rhabdomyosarcoma (RMS) is the leading malignancy. To our knowledge, only one case of intrapericardial RMS was reported in the literature, in a seven-month-old baby. Here we present another newborn baby with intrapericardial RMS.

Turkish Neonatal Society guideline on the safe transport of newborn.

The rational presentation form of perinatal care in developed countries is evaluated within the regionalization program. Neonatal transport is the main step for the regionalization of perinatal care. It is a very important factor for reducing neonatal mortality and morbidity, especially in developing countries. Neonatal transport is a system integrity that includes more than one essential matter. Neonatal transport requires special hardware and serious organization because it can be a caused of mortality and morbidity in its own right. This guideline deals with the transport of newborn infants (definitions, personnel, technical equipment, stabilization conditions, special medical conditions, communication), and the current situation in our country and standard approaches. It should not be forgotten that changes in public space will also change the approach and that every baby should be evaluated within himself or special conditions.

Ultrasonographic measurement of subarachnoid space in normal term newborns.

This study was conducted in order to assess the normal range of subarachnoid space width in healthy term newborns. A total of 230 healthy newborns were evaluated within the first 28 days of life. Measurements were correlated with body weight, height and head circumference. Mean measurements for falx-cortex and craniocortical widths and the correlations are given in tables. Subarachnoid space widths increased as weight, height and head circumference increased and the correlation was statistically significant.

A neonate with malignant ectomesenchymoma.

Malignant ectomesenchymoma is a rare tumor reported in head-neck, abdomen and perineal regions. It consists of mesenchymal and neuroectodermal elements. In this tumor group, neoplastic cells are differentiated into neuronal cells. It also has at least one malignant mesenchymal element, generally rhabdomyosarcoma. In this report we present a neonate with ectomesenchymoma.

Sonographic measurements of the liver, spleen and kidney dimensions in the healthy term and preterm newborns.

This study was conducted in order to assess normal liver, spleen and kidney dimensions in premature and term newborns and determine the acceptable range. A total of 253 (99 preterm and 154 term) healthy newborns were evaluated within the first week of life by sonography. Gestational age ranged from 24 to 41 weeks, weight ranged from 638 to 4800 g. Measurements were compared with gestational age, weight and height of the infants. Normal ranges for kidney, liver and spleen measurements according to gestational age and weight were obtained. We found that weight showed the best correlation with any one of the mentioned organ dimensions.

A newborn infant with generalized glutathione synthetase deficiency.

Pyroglutamic aciduria (5-oxoprolinuria) is a rare autosomal recessive disorder caused by either glutathione synthetase deficiency (GSSD) or 5-oxoprolinase deficiency. The severe form of the disease, generalized GSSD, is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. We report a female infant who had a severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia. High level of 5-oxoproline was detected in her urine and a diagnosis of generalized GSSD was made. She died of severe metabolic acidosis and sepsis at the age of six weeks.