Background Continuous kidney replacement therapies (CKRT) have been reported to be an effective approach to removing toxic metabolites in inborn errors of metabolism (IEM). The present study evaluates efficiency and complications of CKRT in children with IEM. Methods Patients diagnosed with IEM who underwent CKRT in pediatric and neonatal intensive care units were analyzed. CKRT were initiated in patients with persistently high blood ammonia levels (≥ 500 μmol/L), blood ammonia levels > 250 μmol/L in the presence of moderate encephalopathy, high blood leucine levels (≥ 1500 μmol/L), and blood leucine levels < 1500 μmol/L in the presence of deteriorating neurological status or persistent metabolic acidosis. Results Of 22 patients enrolled, nine (40.9%) Maple syrup urine disease (MSUD), eight (36.4%) urea cycle disorders (UCD), and five (22.7%) organic acidemias (OA). Median age was 72.3 [9.9–1040.8] days. In total, 28 dialysis sessions were analyzed [16 (57.1%) continuous venovenous hemodialysis, and 12 (42.9%) continuous venovenous hemodiafiltration]. A significant decrease was noted in leucine levels (from 1608.4 ± 885.3 to 314.6 ± 109.9 µmol/L) of patients with MSUD, while ammonia levels were significantly decreased in patients with UCD and OA (from 1279.9 ± 612.1 to 85.1 ± 21.6 µmol/L). The most frequent complications of CKRT were thrombocytopenia (60.7%), hypotension (53.6%), and hypocalcemia (42.9%). Median age of patients with hypotension treated with vasoactive medications was significantly lower than median age of those with normal blood pressure. Conclusion CKRT is a reliable approach for effective and rapid removal of toxic metabolites in children with IEM, and CKRT modalities can be safely used and are well-tolerated in infants.

Objective: To study the linked result of a complete course of antenatal corticosteroids (ANS) on mortality and short-term morbidity rates among preterm infants in our population. Study design: This single-center retrospective study included the infants born before 32 weeks’ gestation and admitted to neonatal intensive care unit (NICU) between January 1, 2018 and December 31, 2020. The following data of gestational age, birth weight, sex, the etiology of labor, type of delivery, need for intubation in delivery room, APGAR scores (1st and 5th min), the rates of respiratuvary distress syndrome (RDS), surfactant administration, patent ductus arteriosus (PDA), retinopathy of prematurity (ROP), necrotizing enterocolitis (NEC), intraventricular hemorrhage (IVH), and bronchopulmonary dysplasia (BPD) were collected from medical records. Results: The study included 210 infants with a median gestational age of 28.6 weeks (24-31.6), a birth weight of 1065 g (445-2165) and with an ANS use rate of 80%. The mortality rate was lower (p=0.001) with a longer hospital stay (p=0.029), but the rate of BPD was higher (p=0.014) in male infants who received ANS than who did not. According to sex distribution, there was a significant decrease in mortality rate in male infants compared with female ones in ANS received group (11% vs. 23%, p=0.038) with a higher BPD rate (p=0.005). Conclusion: ANS is related with less mortality in male infants born before 32 weeks’ gestation. Further research is currently needed to evaluate advantage of antenatal steroids in different populations.

ObjectiveNon-invasive respiratory support strategies are known to reduce the complications of invasive mechanical ventilation in preterm infants. Nasal continuous positive airway pressure (NCPAP) and nasal intermittent positive pressure ventilation (NIPPV) are commonly used ones. The recent meta-analyses indicated that early NIPPV did appear to be superior to NCPAP for decreasing respiratory failure and the need for intubation among preterm infants with respiratory distress syndrome (RDS). The aim of the study was to compare the short-term outcomes of extremely preterm infants who received NCPAP or NIPPV as an initial treatment of RDS.MethodsThis retrospective study included infants born before 29 weeks' gestation between 1 January 2018 and 31 December 2021 who received non-invasive respiratory support with NCPAP or NIPPV. For every infant included in the cohort, only the first episode of NCPAP or NIPPV as initial treatment was evaluated. The primary outcome was the need for intubation within 72 h, and the secondary outcomes were the need for intubation within 7 days, administration of surfactant, prematurity-related morbidities, mortality, and death or bronchopulmonary dysplasia (BPD).ResultsDuring the study period, there were 116 inborn admissions of preterm infants born &lt;29 weeks' gestation and 60 of them met the inclusion criteria. Of these, 31 (52%) infants received NCPAP while 29 (48%) infants received NIPPV at the first hours after birth. There were no differences in the baseline demographics between the groups (p &gt; 0.05). Blood gas parameters (pH, pCO2, HCO3, and lactate) at admission were not different. The need for intubation within 72 h as the primary outcome was similar between NCPAP and NIPPV groups (35.5 vs. 34.5%, p = 0.935). The rates of surfactant requirement, need for intubation within 7 days, prematurity-related morbidities, mortality, and death/BPD were similar among the groups (p &gt; 0.05).ConclusionNasal intermittent positive pressure ventilation is non-inferior to NCPAP as an initial treatment in extremely preterm infants with RDS. Although the rate of intubation in the first week, mortality, and BPD did not differ between groups, additional studies are needed and the synchronization of NIPPV should be evaluated.

We aimed to compare the definitions of National Institute of Child Health and Human Development (NICHD) for bronchopulmonary dysplasia (BPD) for determining the incidences, and predicting late death and respiratory outcome. This retrospective cohort study included infants born at<32 weeks’ gestation who survived up to 36 weeks’ postmenstrual age (PMA). Infants were classified as having BPD or no BPD per thedefinitions of NICHD 2001 and 2018. The incidences of BPD were 49 and 32% according to the 2001 and 2018 NICHD definitions. Gestational age, birth weight and intubation after birth were associated with BPD by both definitions. The NICHD 2018 definition displayed similar sensitivity (100%) and negative predictive value (100%), and higher specificity (70 vs. 52%) for predicting death after 36 weeks’ PMA; a higher specificity (72 vs. 53%), comparable negative predictive value (77 vs.76%), but lower sensitivity for predicting adverse respiratory outcome within 12 months corrected age compared with the NICHD 2001 definition. The NICHD 2018 definition is as powerful as the 2001 definition for predicting late death and seems to be a better indicator for long-term respiratory outcome. The use of supplemental oxygen or oxygen plus respiratory support should be considered while predicting both late death and long-term respiratory outcome.

Background Nonimmune hydrops fetalis (NIHF) can be caused by different types of etiologies. Some rare intrathoracic lesions are associated with NIHF. Combination of mediastinal teratoma, NIHF, and chylothorax is extremely rare. Mediastinal teratomas which are located in midline should be difficult to be detected. Thoracic imaging should be performed with unknown etiology for hydrops, and in case of chylothorax, the presence of a mass compressing the ductus thoracicus should be considered primarily. Case presentation An infant was born with a diagnosis of NIHF. Bilateral chest tubes were inserted cause of bilateral pleural effusions. After enteral feeding, the previously clear pleural fluid became chylous. Medium-chain triglyceride infant formula and somatostatin analog octreotide were initiated. A mass was appeared on her neck with the disappearance of skin edema. Magnetic resonance imaging confirmed a large, heterogeneous mass which was suggesting immature teratoma originating from thyroid gland. Complete surgical excision of the mass was performed. Histology confirmed high-grade immature teratoma. The neonate made an uneventful recovery. Following complete cessation of pleural fluid drainage, octreotide was stopped. She was discharged home on exclusive breast milk on day 34 of life. Conclusions Mediastinal teratomas are rare masses that cause hydrops fetalis. Although the association of NIHF and mediastinal teratoma is rare, thoracic imaging can be performed if an etiology cannot be found despite basic evaluations for hydrops. In case of chylothorax, the presence of a mass compressing the ductus thoracicus should be considered primarily, and thoracic imaging should be performed.