Differences in Possible Risk Factors, Treatment Strategies, and Outcomes of Neonatal Pneumothorax in Preterm and Term Infants.

OBJECTIVE: The study aimed to compare the risk factors, treatment strategies, and early outcomes of symptomatic neonatal pneumothorax (NP) between preterm and term newborns. MATERIALS AND METHODS: This retrospective cross-sectional study was conducted in a neonatal intensive care unit between 2015 and 2022, consisting of hospitalized neonates with symptomatic NP. The cases were divided into three groups according to their gestational ages: <340/7 (group 1), 340/7-366/7 (group 2), and ≥370/7 weeks (group 3). Risk factors, treatment strategies, and mortality rates of the study groups were compared using Kruskal-Wallis analysis. RESULTS: Fifty-nine infants with a diagnosis of symptomatic NP were included in the study. The number of participants was as follows: 25 (42.3%) in group 1, 18 (30.5%) in group 2, and 16 (27.1%) in group 3. The need of delivery room (DR) resuscitation was significantly higher in group 1 (40%, P = .003). The surfactant administration rate was significantly higher in group 1 when compared to group 2 and group 3 (68% vs. 22% and 19%, respectively), P < .001. Similarly, the invasive mechanical ventilation percentage was significantly higher in group 1 than group 2 and group 3, P = .014. However, compared to group 3 (63%), the percentage of chest drain insertion (CDI) need was significantly higher in group 1 (96%) and group 2 (89%) (P = .014). CONCLUSION: Exposure to DR resuscitation and the need for surfactant are the most common risk factors for NP in preterm infants. Although oxygen and/or needle aspiration treatments are less invasive in symptomatic NP, the improvement rate without CDI is very low in preterm infants born before 34 weeks of gestational age. Cite this article as: Tandircioglu U, Koral Ü, Güzoglu N, Alan S, Aliefendioglu D. Differences in possible risk factors, treatment strategies, and outcomes of neonatal pneumothorax in preterm and term infants. Turk Arch Pediatr. 2024;59(1):87-92.

The diagnostic value of pulmonary near-infrared spectroscopy in the early distinction of neonatal pneumonia from transient tachypnea of the newborn.

AIM: Pulmonary near-infrared spectroscopy (NIRS) is a new and promising tool for diagnosis of neonatal respiratory diseases (RD). The study aimed to determine the role of pulmonary regional oxygen saturation (pRSO2 ) values obtained by NIRS in the early distinction of neonatal pneumonia (NP) from transient tachypnea of the newborn (TTN). METHODS: This prospective, observational, double-blind study was conducted in neonatal intensive care unit (NICU) between 2020 and 2021. Late preterm and term newborns hospitalized in the NICU due to the diagnosis of TTN and NP were included. Cerebral RSO2 and pRSO2 values were measured during the 1st, 24th, 48th, and 72nd hours of hospitalization, using NIRS. RESULTS: Of the eligible 40 infants, 65% (n: 26) were diagnosed as TTN and 35% (n: 16) as NP. The pRSO2 values were significantly higher in the TTN group than the NP group for both apexes (75.3 ± 8.7 vs. 69 ± 5.4, p: .018, respectively) and lateral lung (77.8 ± 6 vs. 72.7 ± 6.2, p: .016, respectively) in the 1st hour of hospitalization. There were significant differences in pRSO2 apex and pRSO2 lateral values between the 1st and 24th hours of hospitalization and the 24th and 48th hours in the NP group (p2 : .001 for both). The optimal pRSO2 apex cut-off value was >72% to predict the diagnosis of NP with a sensitivity of 78.6% and a specificity of 69.2%. CONCLUSION: Pulmonary NIRS may be considered as a feasible and promising diagnostic tool in late preterm and term infants with RD. It may also be helpful for the early differentiation of NP from TTN and the courses of these diseases.

The diagnostic value of n-terminal probrain natriuretic peptides to differentiate neonatal pneumoniae and transient tachypnea of the newborn.

BACKGROUND: The primary objective of the study was to determine the diagnostic value of serum N-terminal probrain natriuretic peptide (NT-proBNP) levels to differentiate neonatal pneumonia (NP) and transient tachypnea of the newborn (TTN). The secondary objective was to investigate the prognostic role of NT-proBNP levels in neonates with severe respiratory distress (RD). METHODS: A prospective, observational, single-blinded study involving 58 late preterm and term newborns who were diagnosed with TTN or NP was conducted between June 2020 and June 2021 at a level-3 neonatal intensive care unit in Kirikkale University Faculty of Medicine. TTN and NP groups were compared for serum NT-proBNP levels measured at the 1st and 24th hours of life. Optimal cut-off NT-proBNP value was determined by Youden index to predict the diagnosis of NP. Lung ultrasound was used to support the diagnosis of TTN and NP. In addition, lung ultrasound score (LUS) was used to determine severe RD. RESULTS: The median of NT-proBNP level was significantly higher at the 24th hour of life in the NP group than in the TTN group, respectively 7263.5 pg/mL (1643-35,000) and 3308 pg/mL (69-19,746), p = 0.004. At a cut-off value of 5515.5 pg/mL, NT-proBNP had a sensitivity of 75% and specificity of 73.8% to predict NP [AUC= 0.749 (95% CI: 0.602-0.895; p = 0.004)]. The study population was divided into two groups as high score group (n: 23, LUS ≥ 7) and low score group (n: 35, LUS < 7) according to the LUS at the 6th hour of life. NT-proBNP values at 24th hour of life were 6320 pg/mL (69-35,000) in high score group and 3500 pg/mL (570-15,948) in low score group, p = 0.044. Duration of oxygen support (p = 0.006), noninvasive ventilation (p = 0.008) and NICU stay (p = 0.004) were higher in high-score group. DISCUSSION: NT-proBNP values at 24th hour of life can be used as a relatively early predictor in the differentiation between NP and TTN in late preterm and term neonates. In addition, elevated NT-proBNP values are related to the higher LUS which reflects the severity of RD regardless of diagnosis.

Evaluation of Patients with Neonatal Thrombosis.

The aim of the study is to characterize acquired and genetic risk factors and to give an account of the hereditary thrombophilia panel in neonatal thrombosis. All newborns diagnosed with neonatal thrombosis in a level III NICU were included in this retrospective cohort study. A total of 1850 patients were admitted to the NICU during the 5-y period; and 11 patients were diagnosed with thrombosis (0.58%). The most common risk factors were central venous catheter placement, hypoxia and prematurity and related complications, and sepsis. Four patients were investigated regarding the inherited risk factors for thrombosis. In these 4 patients, homozygous A1298C alleles of MTHFR and heterozygous FXIIIV34L mutations; homozygous PAI-SERPINE1 and heterozygous MTHFRA1298C mutations; compound heterozygous mutations of MTHFRC677T and MTHFRA1298C; and compound heterozygous mutations of MTHFRC677T, MTHFRA1298C, and PAISERPINE1 were detected respectively. In conclusion, neonatal thrombosis is multifactorial; newborns with acquired risk factors may also have hereditary risk factors. TRIAL INFORMATION: ClinicalTrials.govIdentifier: NCT05367466.

Life-threatening massive upper gastrointestinal bleeding in a term and healthy baby.

Upper gastrointestinal (UGI) bleeding due to gastric ulcer and gastritis can be seen in severely ill or premature infants but is rarely reported in healthy term newborns. UGI endoscopy is crucial for the etiological evaluation and appropriate treatment of UGI hemorrhages. This report discusses the differential diagnosis and treatment approach in a previously healthy infant who was admitted to the neonatal intensive care unit due to life-threatening severe UGI bleeding causing hemodynamic instability.

A Case of Early Infantile Epileptic Encephalopathy Due to KCNQ2 Gene Mutation Presenting With Episodes of Hiccups.

Neonatal epilepsy syndromes are responsible for only 15% of the cases of neonatal seizure. An underlying genetic disorder can be detected in approximately 42% of this subgroup. KCNQ2 gene-associated epilepsies are very rare and more common presentations are self-limited familial neonatal epilepsy (SLFNE) and early infantile epileptic encephalopathies (EIEE). The most common initial seizure semiologies are tonic seizures with or without autonomic symptoms in EIEE resulting from KCNQ2 gene mutation. It is characterized by early neonatal onset seizures with suppression burst pattern on electroencephalogram and typically results in severe developmental delay. Therapeutic options for infants with KCNQ2-related EIEE are limited and there is no consensus about it in the literature. Herein, the neonate with EIEE with unexpected episodes of hiccups due to novel mutation of the KCNQ2 gene, which was reported second time, was presented and antiepileptic treatment strategies were discussed in the light of current literature.

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.

Influence of Intensive Care Unit Enlightenment on Premature Infants on Functional Brain Maturation Assessed by Amplitude-Integrated Electroencephalograph.

OBJECTIVE: Amplitude-integrated electroencephalograph (aEEG) presents a valuable tool for functional brain maturation of preterm infants. However, the effect of enlightenment on functional brain maturation of premature infants has not been investigated. We aimed to do this with aEEG. STUDY DESIGN: A total of 32 infants, 30 to 35 gestational weeks, were involved in the study. They were randomly distributed into three groups in which different lighting protocols were applied. In group 1, the infants' incubators were covered for 24 hours. In group 2, the infants' incubators were open for 24 hours. In group 3, the infants' incubators were covered for 12 hours and open for another 12 hours. The infants are evaluated with aEEG recordings done on the 3rd (first measurement) and 10th days (second measurement) along with the Burdjalov scoring. Analysis of aEEG recordings was performed, based on sleep-wake cycles (SWCs), upper and lower margin amplitudes, narrowband and broadband of SWC, and bandwidth of SWC. RESULTS: At first, the narrowband lower amplitudes in group 1 were higher than those of the other groups (p = 0.042), but the difference was not significant in the second measurement (p = 0.110). The Burdjalov scores were higher in group 1 and group 3 on 10th day, though not statistically significant (p = 0.871). When the infants were re-evaluated according to the gestational weeks, the Burdjalov scores of the two groups less than 34 weeks (30-31 and 32-33 weeks) were similar, whereas 34 to 35 weeks were higher when compared with those of the two groups. CONCLUSION: The difference observed between groups in terms of narrowband lower amplitude in the first measurement may reflect the effect of intrauterine environment rather than enlightenment at the same gestational age because it was made on the third day. However, the fact that all groups have similar results on day 10 suggests that other factors in the intensive care setting may diminish the effect of enlightenment. Burdjalov scores are associated with maturation, and high scores found in the 34- to 35-week group suggest that the 34-week maturation might be a threshold for SWC and development in our group sample.

Management of hypoglycemia in newborn: Turkish Neonatal and Pediatric Endocrinology and Diabetes Societies consensus report.

Hypoglycemia is one of the most important and most common metabolic problems of the newborn because it poses a risk of neurological injury, if it is prolonged and recurs. Therefore, newborns who carry a risk of hypoglycemia should be fed immediately after delivery and the blood glucose level should be measured with intervals of 2-3 hours from the 30th minute after feeding. The threshold value for hypoglycemia is 40 mg/dL for the first 24 hours in symptomatic babies. In asymptomatic babies, this value is considered 25 mg/dL for 0-4 hours, 35 mg/dl for 4-24 hours, 50 mg/dL after 24 hours and 60 mg/dL after 48 hours. Screening should be performed with bed-side test sticks. When values near the limit value are obtained, confirmation with laboratory method should be done and treatment should be initiated, if necessary. The level targeted with treatment is considered 50 mg/dL in the postnatal first 48 hours before feeding, 60 mg/dL after 48 hours in babies with high risk and above 70 mg/dL in babies with permanent hypoglycemia. In cases in which the blood glucose level is below the threshold value and can not be increased by feeding, a glucose infusion of 6-8 mg/kg/min should be initiated. If symptoms accompany, a mini bolus of 10% dextrose (2 ml/kg/min) should accompany. Incements (2 mg/kg/min) should be performed, if the target level can not be achieved and decrements (2 ml/kg/ min) should be performed, if nutrition and stabilization is provided. The infusion should be discontinued, if the infusion rate decreases to 3-5 mg/ kg/min. If necessary, blood samples should be obtained during hypoglycemia in terms of differential diagnosis and the investigation should be performed following a 6-hour fasting period in babies fed enterally and at any time when the plasma glucose is <50 mg/dL in babies receiving parenteral infusion. The hypoglycemic babies in the risk group whose infusions have been terminated can be discharged, if the plasma glucose level is found to be at the target level for two times before feeding and babies with permanent, severe or resistant hypoglycemia can be discharged, if the plasma glucose level is >60 mg/dL following a 6-hour fast.

Management of hyperbilirubinemia in preterm infants in Turkey.

BACKGROUND/AIM: Prematurity is a significant risk factor for developing unconjugated hyperbilirubinemia. This study investigated the current approach to managing hyperbilirubinemia in preterm newborns in Turkey. MATERIALS AND METHODS: A study-specific questionnaire on the management of jaundice in preterm infants was sent to 100 level III neonatal intensive care units in Turkey. RESULTS: Responses were obtained from 84 centers from all regions of Turkey. Of the centers, 75.3% used the Turkish Neonatology Society guidelines for deciding to start phototherapy, and 24.7% used different guidelines. The monitoring of bilirubin varied among the participants. Of the participants, 53.6% believed that prophylactic phototherapy was necessary if the infant's birth weight was below 1000 g. The participants reported 6 cases of kernicterus in preterm infants in recent years. CONCLUSION: There is no single standard approach for managing neonatal hyperbilirubinemia in preterm infants in Turkey. Prophylactic phototherapy for extremely low birth-weight infants might be added to the guidelines for Turkey.

Haemorrhagic pneumonia caused by Stenotrophomonas maltophilia in two newborns.

Invasive procedures and antibiotic treatment increase the risk of nosocomial infections in neonatal intensive care units. Early identification and appropriate treatment is important. Herein we report two cases of massive hemorrhagic pneumonia caused by Stenotrophomonas maltophilia. The first case was diagnosed with congenital pneumonia; a chest tube was inserted because of pneumothorax on the third day of life. The second case had been referred with respiratory distress syndrome, and bilateral pneumothorax was present on admission. Upon follow up, the cases' clinical condition worsened; acute respiratory distress syndrome and massive pulmonary haemorrhage developed. After Stenotrophomonas maltophilia was isolated in blood cultures, the cases were treated successfully using a combination of trimethoprim/sulfamethoxazole and fluoroquinolone.

Phenotypic and genotypic spectrum of Turkish patients with isovaleric acidemia.

We aim to investigate the genetic basis of isovaleryl-CoA dehydrogenase (IVD) gene mutations and genotype-phenotype correlations in Turkish patients. Accordingly, bi-directional sequencing was performed to screen 26 patients with isovaleric acidemia (IVA). Nine novels (c.145delC, c.234 + 3G > C, c.506_507insT, p.Glu85Gln, p.Met147Val, p.Ala268Val, p.Ile287Met, p.Gly346Asp and p.Arg382Trp) and six previously reported (c.456 + 2T > C, p.Arg21His, p.Arg21Pro, p.Arg363Cys, p.Arg363His p.Glu379Lys) pathogenic mutations were identified. Pathogenicity of the novel mutations was supported using computational programs. No clear genotype-phenotype correlation could be determined. One of the cases with the novel c.234 + 3G > C mutation has portoseptal liver fibrosis, the clinical condition that was first reported for IVA. This study is the first comprehensive report from Turkey related to IVA genetics that provides information about the high number of disease-causing novel mutations.

Anesthesia for a child with Walker-Warburg syndrome.

BACKGROUND AND OBJECTIVES: Walker-Warburg Syndrome is a rare, autosomal recessive congenital muscular dystrophy manifested by central nervous system, eye malformations and possible multisystem involvement. The diagnosis is established by the presence of four criteria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinal malformation. Most of the syndromic children die in the first three years of life because of respiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation. CASE REPORT: The anesthetic management of a two-months-old male child listed for elective ventriculo-peritoneal shunt operation was discussed. CONCLUSIONS: A careful anesthetic management is necessary due to the multisystem involvement. We reported anesthetic management of a two-months-old male child with Walker-Warburg Syndrome who was listed for elective ventriculo-peritoneal shunt operation.

Anestesia em criança com síndrome de Walker-Warburg / Anesthesia for a child with Walker-Warburg syndrome / Anestesia en niño con el síndrome de Walker-Warburg

Justificativa e objetivos: A síndrome de Walker-Warburg é uma distrofia muscular autossômica recessiva congênita rara, manifestada pelo sistema nervoso central com malformações oculares e possível envolvimento de vários sistemas. O diagnóstico é estabelecido pela presença de quatro critérios: distrofia muscular congênita, lisencefalia tipo II, malformação cerebelar e malformação da retina. A maioria das crianças com a síndrome morre nos primeiros três anos de vida por causa de insuficiência respiratória, pneumonia, convulsões, hipertermia e fibrilação ventricular. Relato de caso: É discutida a conduta anestésica em uma criança do sexo masculino, de dois meses, programada para cirurgia eletiva de derivação ventrículo-peritoneal. Conclusões: Uma abordagem anestésica cuidadosa é necessária por causa do envolvimento de vários sistemas. Relatamos a conduta anestésica em uma criança do sexo masculino de dois meses com síndrome de Walker-Warburg, que foi programada para cirurgia eletiva de derivação ventrículo-peritoneal. .

Background and objectives: Walker-Warburg Syndrome is a rare, autosomal recessive congenital muscular dystrophy manifested by central nervous system, eye malformations and possible multisystem involvement. The diagnosis is established by the presence of four criteria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinal malformation. Most of the syndromic children die in the first three years of life because of respiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation. Case report: The anesthetic management of a two-months-old male child listed for elective ventriculo-peritoneal shunt operation was discussed. Conclusions: A careful anesthetic management is necessary due to the multisystem involvement. We reported anesthetic management of a two-months-old male child with Walker -Warburg Syndrome who was listed for elective ventriculo-peritoneal shunt operation. .

Justificativa y objetivos: el síndrome de Walker-Warburg es una distrofia muscular autosómica recesiva congénita rara, manifestada por el sistema nervioso central con malformaciones oculares y la posible participación de varios sistemas. El diagnóstico se establece por la presencia de 4 criterios: distrofia muscular congénita, lisencefalia tipo II, malformación cerebelar y malformación de la retina. La mayoría de los niños con el síndrome se muere a los primeros 3 años de vida debido a la insuficiencia respiratoria, neumonía, convulsiones, hipertermia y fibrilación ventricular. Relato de caso: se discute aquí la conducta anestésica en un niño del sexo masculino, de 2 meses de edad, programado para la cirugía electiva de derivación ventrículo-peritoneal. Conclusiones: un cuidadoso abordaje anestésico se hace necesario debido a la involucración de varios sistemas. Relatamos la conducta anestésica en un niño del sexo masculino de 2 meses de edad, con el síndrome de Walker-Warburg, que fue programado para la cirugía electiva de derivación ventrículo-peritoneal .

Can resistin be a new indicator of neonatal sepsis?

BACKGROUND: Sepsis is an important cause of neonatal death and perinatal brain damage, particularly in preterm infants. It is thought that activation of the inflammatory cascade triggered by cytokine might play a role in the pathogenesis of sepsis. Recent evidence supports a role for resistin in inflammation. There are no data in the literature on resistin levels of premature newborns with sepsis, which can also cause inflammatory response. The objective of this study was to evaluate whether resistin can be used as an indicator in neonatal sepsis of preterm babies. MATERIALS AND METHODS: Forty-three premature newborns considered to have sepsis were included in the study. Forty-three gestational and postnatal age- and sex-matched premature newborns without premature prolonged rupture of membrane or sepsis served as controls. RESULTS: The median resistin and interleukin-6 (IL-6) levels of the premature babies with sepsis were 85.9 ng/mL and 342.7 pg/mL, respectively, and were higher than those of the control group (29.9 ng/mL and 17.7 pg/mL, respectively). The sensitivity, specificity, positive, and negative predictive values for resistin were 73.7%, 45.8%, 68.3%, and 52.4%, respectively. CONCLUSION: Resistin levels were higher in premature newborns with sepsis and correlated with IL-6 levels, which is an indicator of neonatal sepsis. This suggests that resistin may also be used in the diagnosis of neonatal sepsis. However, it has limited value when compared with the other inflammatory markers including C-reactive protein, procalcitonin, and IL-6.

Novel Mutations in the PC Gene in Patients with Type B Pyruvate Carboxylase Deficiency.

We have investigated seven patients with the type B form of pyruvate carboxylase (PC) deficiency. Mutation analysis revealed eight mutations, all novel. In a patient with exon skipping on cDNA analysis, we identified a homozygous mutation located in a potential branch point sequence, the first possible branch point mutation in PC. Two patients were homozygous for missense mutations (with normal protein amounts on western blot analysis), and two patients were homozygous for nonsense mutations. In addition, a duplication of one base pair was found in a patient who also harboured a splice site mutation. Another splice site mutation led to the activation of a cryptic splice site, shown by cDNA analysis.All patients reported until now with at least one missense mutation have had the milder type A form of PC deficiency. We thus report for the first time two patients with homozygous missense mutations with the severe type B deficiency, clinically indistinguishable from other patients with type B form of PC deficiency.The mutations found here are novel; it is noteworthy that four Turkish patients did not have any mutations in common, despite the rarity of PC deficiency. There is thus no evidence for recurrent mutations in the Turkish or other populations.

Microarray based mutational analysis of patients with methylmalonic acidemia: identification of 10 novel mutations.

Methylmalonic acidemia is an autosomal recessive metabolic disorder affecting the propionate oxidation pathway in the catabolism of several amino acids, odd-chain fatty acids, and cholesterol. Methylmalonic acidemia is characterized by elevated levels of methylmalonic acid in the blood and urine. Mutations in the MUT gene, encoding methylmalonyl-CoA mutase carries out isomerization of L-methylmalonyl-CoA to succinyl-CoA, cause methylmalonic acidemia. In this study, 30 Turkish patients diagnosed with mut methylmalonic acidemia were screened for mutations using custom designed sequencing microarrays. The study resulted in detection of 22 different mutations, 10 of which were novel: p.Q132*, p.A137G, c.753+1T, p.T387I, p.Q514E, p.P615L, p.D625V, c.1962_1963delTC, p.L674F, and c.2115_2116insA. The most common, p.P615T, was identified in 28.0% of patients. These results suggest that microarray based sequencing is a useful tool for the detection of mutations in MUT in patients with mut methylmalonic acidemia.