The aim of this study was to assess the oxidative stress (OS) levels and dynamic thiol-disulfide balance in preterm newborns with bronchopulmonary dysplasia (BPD). This prospective study included newborns separated into 2 groups, those with BPD (case) or without BPD (control). The 2 groups were compared by clinical and laboratory findings. The OS parameters total oxidant status (TOS), total antioxidant status (TAS), OS index (OSI), native thiol (NT), and total thiol were measured within the first day after birth. Oxygen requirements were measured using the fraction of inspired oxygen (FIO₂) recorded in the first hour after birth/admission and the average FIO₂ within 28 days of the birth. Infants diagnosed with BPD had a significantly lower gestational age and birth weight and a lower 5-min Apgar score (P < .05). Infants with BPD also had a higher rate of respiratory distress syndrome, rate of use of surfactant therapy, duration of ventilation therapy, and duration of hospital stay compared with control (P = .001, P = .001, P = .001, and P = .001, respectively). Plasma TAS and NT levels of newborns with BPD were significantly lower than newborns without BPD (P < .05). In the BPD group, plasma TOS and OSI levels were significantly higher than in the control group. We found that OS was increased in newborns with BPD. The clinical significance of this study will provide the clinician with a different perspective on BPD by determining the dynamic thiol disulfide balance.

Systemic pseudohypoaldosteronism (PHA) is a rare, salt-wasting syndrome that is caused by inactivating variants in genes encoding epithelial sodium channel subunits. Hyponatremia, hyperkalemia, metabolic acidosis, increased aldosterone and renin levels are expected findings in PHA. Clinical management is challenging due to high dose oral replacement therapy. Furthermore, patients with systemic PHA require life-long therapy. Here we report a patient with systemic PHA due to SCNN1B variant whose hyponatremia and hyperkalemia was detected at the 24th hour of life. Hyperkalemia did not improve with conventional treatments and dialysis was required. He also developed myocarditis and hypertension in follow-up. Challenges for diagnosis and treatment in this patient are discussed herein. In addition, published evidence concerning common features of patients with SCNN1B variant are reviewed.

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.

The aim of this study is to evaluate both dynamic thiol-disulfide homeostasis and oxidative stress (OS) levels in patients with retinopathy of prematurity (ROP). A total of 129 infants of <34 weeks gestational age were enrolled in the present study. The thiol-disulfide homeostasis was determined by using the new, cost-effective and fully automated colorimetric method. Total antioxidant status (TAS), Total oxidant status (TOS) and Oxidative stress index (OSI) levels were evaluated. We found serum TAS levels were lower while serum TOS and OSI levels were significantly higher in patients with ROP compare to the without ROP group (p < .05). However, native, total and disulfide values were not statistically significant between the groups (p > .05). In addition, we also evaluated the native, total and disulfide levels in patients with ROP according to grades and no statistically significant results were found (p > .05). Low birth weight (p = .001), gestational age (p = .001) and 5-min Apgar score were significantly lower in the ROP group. This study revealed that dynamic thiol-disulfide homeostasis was changed in patients with ROP. Increased TOS and decreased TAS levels may be associated with functional reduction of the antioxidant system due to increased OS. This indicate that ROP patients are highly sensitive to OS. The dynamic thiol-disulfide homeostasis may conduce to the pathophysiological mechanism and disease follow-up in patients with ROP. The results of this study show that ROP patients are highly sensitive to oxidative stress.

Background: Transient tachypnea of the newborn (TTN), which is the most common respiratory disease in the neonatal period, increases respiratory workload in newborns. We purposed to evaluate the oxidative stress (OS) status and thiol disulfide hemostasis in late preterm and term newborns with TTN in this study. Methods: The study was carried out in a single-centre neonatal intensive care unit to investigate the effect of continuous airway positive pressure (CPAP) on the oxidative system in newborns with TTN. Thiol (native and total) and disulfide levels, total antioxidant and oxidant status (TAS/TOS) and Oxidative stress index (OSI) levels were measured. Results: Total thiol levels measured before treatment was 429.5 (369.5–487) µmol/L in the late preterm group and 425 (370–475) µmol/L in the term group (p = 0.741). We found significant changes in TOS, OSI and TAS levels after CPAP treatment in the late preterm group (p < 0.001, p < 0.001, p = 0.012 respectively). It was also found that the disulfide level, which was 26.2 (19.2–31.7) before the treatment, decreased to 19.5 (15.5–28.75) after the treatment (p = 0.001) in late preterms. Conclusion: CPAP treatment reduced the OS status burden associated with TTN in neonates. The late preterm newborns with TTN are more affected by OS and increased OS levels decrease with CPAP treatment.

Background/aim: Most inborn metabolic diseases are diagnosed during the neonatal period. The accumulation of toxic metabolites may cause acute metabolic crisis with long-term neurological dysfunction and death. Renal replacement therapy (RRT) modalities allow the efficient removal of toxic metabolites. In this study, we reviewed our experience with continuous venovenous hemodiafiltration (CVVHDF) as RRT for newborns with an inborn metabolic disease. Materials and methods: Patients diagnosed with an inborn metabolic disease who received CVVHDF treatment at our neonatal intensive care unit between January 2014 and December 2017 were included in this study. Their demographic and clinical data were collected, and the efficacy and safety of CVVHDF was evaluated. Results: Nine continuous RRT (CRRT) sessions as CVVHDF were performed in eight newborns with a diagnosis of urea cycle defect (n= 5), maple syrup urine disease (n= 2), or methylmalonic acidemia (n= 1). The mean age at admission was 10 ± 8.6 days (range: 3–28 days). The mean plasma levels of ammonium were 1120 ± 512.6 mg/dL and 227.5 ± 141.6 mg/dL before and at the end of treatment, respectively. Plasma levels of leucine were 2053.5 ± 1282 µmol/L and 473.5 ± 7.8 µmol/L before and at the end of treatment, respectively. The CVVHDF duration was 32.3 ± 11.1 h (median: 37 h; range: 16–44 h), and the mean length of hospitalization was 14.6 ± 12.9 days. The mean duration of CVVHDF was 32.3 ± 11.1 h (range: 16–44 h). Circuit clotting was the most common observed complication (37.5%) and the survival rate was 50%. Among surviving patients, two developed severe and two developed mild mental and motor retardation. Conclusion: CVVHDF is a CRRT modality that can be used to treat newborns with an inborn metabolic disease. Early diagnosis, and commencement of specific medical therapy, diet, and extracorporeal support, if needed, are likely to result in improved short- and long-term outcomes. Key words: Inborn metabolic diseases, renal replacement therapy, continuous venovenous hemodiafiltration, newborn, survival