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BackgroundThis study aimed to evaluate the indications, complications, and outcomes of peritoneal dialysis (PD) in term neonates from a state hospital.MethodsThe demographic, clinical, and laboratory data of 67 newborn term infants who underwent PD within the first 4 weeks of life between June 2014 and June 2019 were retrospectively analyzed.ResultsTwenty-five patients (37.3%) were male, 42 (63.7%) were female. The mean gestational age was 38.3 ± 0.8 (range: 37–40) weeks and mean birthweight 3,100 ± 504.9 g (range: 1,800–5,000 g). The mean age of patients at the start of dialysis was 7.97 ± 8.34 days (range:1–44 days) and the mean duration for dialysis was 3 ± 5.42 days (range 1–40 days). The majority of patients who underwent PD had inborn error of metabolism (59.7%). The most common complication was dialysate leakage. Thirty-three neonates (49.2%) died during PD process because of underlying disease, 17 patients (25.4%) were referred to other centers, and 17 patients (25.4%) were discharged. There were seven acute kidney injury patients associated with hypernatremic dehydration. There were statistically significant differences between non-survivors and survivors in terms of dialysis duration, birthweight, weight at admission, requirements for mechanical ventilation, and inotrop agent, and also the renal angina index.ConclusionsIn our region, hypernatremic dehydration is still a one of the major cause for acute kidney injury (AKI) in newborns. In state hospitals, pediatric specialists must be present in neonatal intensive care units in order not to refer unstable patients to other centers and to provide high-quality patient care.

We aimed to evaluate the demographic, clinical, and laboratory findings and the management of newborns with hypernatremic dehydration (HDH). A total of 85 term newborns with serum sodium (Na) levels higher than 145 mEq/L who admitted to our hospital between January 2011 and December 2018 were included in this study. Among all cases, 54.1% were female infants with the mean birth weight, weight loss ratio, and median age at diagnosis of 3095 ± 540 g, 13.6 ± 10%, and 8 (2-24) days, respectively. The most common presenting complaints were breastfeeding difficulties (90.5%), fever (63.5%), decreased urination (43.5%), jaundice (22.3%), and convulsion (15.3%). The mean sodium and potassium, and median blood urea and creatinine levels on admission were 167.9 ± 13.4 mEq/L, 5.4 ± 2.8 mmol/L, 213 mg/dL (11-476 mg/dL), and 2.4 mg/dL (0.52-9.96 mg/dL), respectively. There was metabolic acidosis in 67% and acute renal failure in 74.4% of patients, while peritoneal dialysis was performed in 12 of them. There was a positive correlation between weight loss ratio and admission age, serum urea, and creatinine levels; there was a negative correlation between weight loss and blood pH. Eight patients died (9.4%). In our study, serum urea, creatinine, potassium, metabolic acidosis levels, convulsion, and dialysis requirements at the time of admission of the newborns with HDH were found to be higher in those who died compared to those who survived. Convulsion was a presenting complaint, and it was also observed during the treatment.

Congenital syphilis is a severe disease that arises from the vertical transmission of Treponema pallidum. Clinical findings are related to the pregnancy stage, fetal gestational week, maternal treatment and fetal immunologic response. Prematurity, low birth weight, nonimmune hydrops fetalis, necrotizing enterecolitis, hepatomegaly, skin eruptions, thrombocytopenia, hemolytic anemia and fever can be detected in the symptomatic newborn. Postnatal respiratory insufficiency, hepatomegaly, anemia and thrombocytopenia were detected in a baby who was born at the 29 week of gestation, weighing 1.160 g and followed due to intestinal hyperechogenicity from the second trimester. Her and her mother's Venereal Disease Research Laboratory titers were positive, confirming test Treponema pallidum hemagglutination was reactive. After penicillin was administered for 10 days, anemia, and thrombocytopenia were regressed. In the 15 day of life, findings of perforated necrotising enterocolitis (NEC) suddenly appeared. The operation was performed due to NEC for three times but nonresponsive laboratory and clinical findings and died in the 54 day of life. We assumed that syphilis is the cause of both bowel hyperechogenicity and necrotising enterocolitis.

Sirolimus has been reported to be effective in the treatment of the diffuse form of congenital hyperinsulinism (CHI), unresponsive to diazoxide and octreotide, without causing severe side effects. Two newborns with CHI due to homozygous ABCC8 gene mutations were started on sirolimus aged 21 and 17 days, due to lack of response to medical treatment. A good response to sirolimus was observed. At follow-up after ten and two months of treatment, liver enzymes were found to be increased [serum sirolimus level 1.4 ng/mL (normal range: 5-15), aspartate aminotransferase (AST): 298U/L, alanine aminotransferase (ALT): 302U/L and serum sirolimus level: 9.9 ng/mL, AST: 261U/L, ALT: 275U/L, respectively]. In Case 1, discontinuation of the drug resulted in normalization of liver enzymes within three days. Two days after normalization, sirolimus was restarted at a lower dose, which resulted in a repeated increase in transferases. In Case 2, a reduction of sirolimus dose caused normalization of liver enzymes within ten days. When the dose was increased, enzymes increased within three days. Sirolimus was discontinued in both cases. The rapid normalization of liver enzyme levels after sirolimus withdrawal or dose reduction; elevation of transaminases after restart or dose increase and rapid normalization after sirolimus withdrawal were findings strongly suggestive of sirolimus-induced hepatitis. To the best of our knowledge, this is the first report of sirolimus-induced hepatitis in CHI. Sirolimus is a promising drug for CHI patients who are unresponsive to medical treatment, but physicians should be vigilant for adverse effects on liver function.

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. 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. 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. 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 objectives of the study were to assess folate deficiency in patients with classic galactosemia, and to determine whether folic acid supplementation has an effect on galactose-1-phosphate uridyltransferase enzyme activity. Sixty-one newborn infants diagnosed with classic galactosemia between 2010 and 2017 were retrospectively evaluated. Within this group, 48 patients with Q188R homozygous mutation alone were enrolled into the study. Serum folate concentration was studied using chemiluminescence; and in folate deficient patients, galactose-1-phosphate uridyltransferase measurements before and after folic acid supplementation (100 mg/day folic acid for 30 days) were performed using an enzymatic calorimetric measurement technique based on kinetics. The serum folate level was low ( < 4 ng/ml) in 12 patients (25%). The galactose-1-phosphate uridyltransferase enzyme activity after folic acid supplementation was significantly higher than the values before folic acid supplementation (1.00±0.19 U/g Hb vs. 0.74±0.23 U/g Hb, p < 0.05); but was still less than the normal levels. Folate deficiency, most likely due to poor dietary intake, may develop in pediatric patients with classical galactosemia, and folic acid should be supplemented. Folic acid supplementation appears to have a low, but statistically significant, effect on galactose-1-phosphate uridyltransferase enzyme activity, but comprehensive research is needed to clarify whether there is any clinical significance.

This study aimed to evaluate the efficacy, cost, and effects of anti-D immunoglobulin (anti-D Ig), methylprednisolone, or intravenous immunoglobulin (IVIG) therapy on the development of chronic disease in children who are Rh-positive with diagnosed immune thrombocytopenic purpura (ITP). Children with newly diagnosed ITP and platelet count <20,000/mm 3 were prospectively randomized to treatment with anti-D Ig (50 μg/kg), methylprednisolone (2 mg/kg/day), or IVIG (0.4 g/kg/day, 5 days). Sixty children with a mean age of 6.7 years were divided into three equal groups. No difference was observed between platelet counts before treatment and on day 3 of treatment. However, platelet counts at day 7 were lower in the methylprednisolone group than in the IVIG group ( P  = 0.03). In the anti-D Ig group, hemoglobin and hematocrit levels were significantly lower at the end of treatment ( P  < 0.05). Chronic ITP developed in 30 % of the anti-D Ig group, 35 % of the methylprednisolone group, and 25 % of the IVIG group, but no significant difference was noted among the groups. The cost analysis revealed that the mean cost of IVIG was 7.4 times higher than anti-D Ig and 10.9 times higher than methylprednisolone. In the treatment of ITP in childhood, one 50 μg/kg dose of anti-D Ig has similar effects to IVIG and methylprednisolone. Among patients who were treated with anti-D Ig, serious anemia was not observed, and the cost of treatment was less than that of IVIG treatment.

Hypoxic-ischemic encephalopathy (HIE) continues to be a predominant cause of morbidity and mortality in neonates. Therapeutic hypothermia (TH) is the only method with proven neuroprotective effects, and the aim of this study was to evaluate the short-term results of patients treated with TH. Demographic, clinical characteristics, laboratory and aEEG results of patients who received TH treatment with a diagnosis of Stage II or Stage III HIE according to modified Sarnat staging in the Neonatal Intensive Care Unit were analyzed retrospectively. A total of 101 patients were included in the study. The mean gestational age of the patients was 38.8±1.5 weeks, the mean birth weight was 3215±499.5 g, and 40.6% were female. According to the modified Sarnat staging, 50.5% of the patients were evaluated as Stage II, and the others as Stage III HIE. The most common peripartum risk factors were meconium delivery (25.7%) and prolonged or difficult labor (20.7%). Mortality rates in patients with Stage II and Stage III HIE were 5.9% and 26%, respectively. In one of the patients who died, the 6th-hour aEEG background activity was moderately abnormal, and in 15 patients there was a severely abnormal voltage pattern. Acute kidney injury was found to be the most effective factor in mortality. In our study, it was concluded that the mortality rate of newborns diagnosed with Stage III HIE was higher, the biggest impact factor on mortality was acute kidney injury, and 6th-hour voltage activity in aEEG monitoring was useful in predicting prognosis.