Postnatal surgery for myelomeningocele in neonates: neurodevelopmental outcomes.

BACKGROUND: The study aims to evaluate the neurodevelopmental outcomes of neonates with myelomeningocele (MMC) operated in the postnatal period. METHODS: This is a prospective follow-up study in a tertiary neonatal intensive care unit. Neurodevelopmental outcomes of term neonates operated for MMC and healthy term newborns were compared with the Bayley Scales of Infant and Toddler Development -Third Edition (BSID III) at 12-18 months. RESULTS: A total of 57 cases were included in the study (patient group = 27; control group = 30). Demographic data between the groups were similar. Cognitive, linguistic, and motor composite scores of the patient group were lower than those of the control group (p < 0.001). In the patient group, those who underwent ventriculoperitoneal shunt had lower cognitive, language and motor scores than those without shunt (p < 0.05). The cognitive, linguistic, and motor composite scores in the patient group who underwent surgery before 72 h were better than those who underwent surgery after 72 h. DISCUSSION: In our study, it was found that the neurodevelopmental prognosis of MMC cases requiring ventriculoperitoneal shunt in the postnatal period was significantly worse than those without shunt. It is the first study in which the neurodevelopment of patients with MMC who were operated in the postnatal period was evaluated with BSID III evaluated and delays in all areas were shown in cases with MMC compared to normal cases. Better neurodevelopmental outcomes in patients operated in the first 72 h suggest that early surgery will improve neurodevelopmental outcomes in patients with MMC.

The role of vitamin D receptor gene polymorphism in the development of necrotizing enterocolitis.

BACKGROUND: Vitamin D and its receptor (VDR) effects on the gastrointestinal system are among its most critical multisystemic effects. METHODS: This study aimed to reveal that VDR gene polymorphisms may constitute a risk factor for necrotizing enterocolitis (NEC). VDR Fok1-Bsm1-Apa single-nucleotide polymorphisms were analyzed in the NEC group (n = 74) and the control group (n = 147). Among 1112 babies at and below 36 weeks of gestational age who were hospitalized between January 2013 and December 2016 with a diagnosis of prematurity, 74 of a total of 148 patients who developed NEC during follow-up (NEC group) were included in the study. When NEC was diagnosed according to clinical and radiological findings and staged using Modified Bell criteria, 9 (12.1%) of 74 babies were stage 1A, 13 (17.5%) stage 1B, and 5 (6.7%) stage 2A, 33 (44.5%) stage 2B, 7 (9.4%) stage 3A, 7 (9.4%) stage 3B. Of 964 babies who did not develop NEC during follow-up, 147 were included as the control group in the study. Genotyping of VDR polymorphisms was assayed by real-time PCR. From 221 premature babies in the NEC and control groups, 2 ml peripheral blood was taken appropriately and meticulously into an EDTA tube. DNA was isolated from these blood samples. DNA amplification was performed using a thermal cycler (Applied Biosystems GeneAmp PCR System 9600). RESULTS: When the two groups were compared in terms of the prevalence of VDR Fok1 C/T genotype, it was found that TT genotype increased the risk of NEC by 2.697 times, and there was a significant relationship between TT genotype and the risk of NEC (p = 0.041). Multivariable logistic regression analysis was performed in terms of gestational age, birth weight, VDR gene polymorphism data between NEC and the control group. According to the analysis results, TT polymorphism, increased the risk of disease 4.5 times (p = 0.033). CONCLUSION: Fok 1 C > T polymorphism in the VDR gene plays a role in the development of NEC. Identifying the risk groups by detecting gene polymorphisms that cause increased susceptibility to NEC may assist in the follow-up of these patients and in making early treatment decisions for them. IMPACT: In this study examining the non-bone effects of the genetic differences in vitamin D metabolism in premature babies, Fok 1 polymorphism has been observed to be an essential risk factor for NEC. This is the first study in our country that has investigated the relationship between VDR gene polymorphism and necrotizing enterocolitis among the Turkish population. Identifying the risk groups by detecting gene polymorphisms that cause increased susceptibility to NEC may assist in the monitoring of these patients and in making early treatment decisions for them.

Arrhythmias in children undergoing orthotopic heart transplantation.

Introduction: Heart transplantation (HT) is the only treatment option in children with heart failure secondary to cardiomyopathies and non-reparable congenital heart diseases. Methods: We performed a retrospective clinical data review of all consecutive pediatric patients (aged 2-18 years) who underwent orthotopic HT for advanced heart failure at our institution between January 2007 and January 2023. Clinical, procedural, and follow-up data were collected and comprehensively analyzed. Results: We identified 27 children (66.7% males) with a median age of 15 years (IQR: 7-16) and a median weight of 45 kg (IQR: 22-66) at the time of the intervention. 24 patients (88.8%) were diagnosed with dilated cardiomyopathy, 2 (7.4%) with restrictive cardiomyopathy, and 1 (3.7%) with hypertrophic cardiomyopathy. On a median follow-up of 35.07 months (IQR: 13.13-111.87), arrhythmias were detected in 9 (33%) patients. Three patients developed symptomatic sinus node dysfunction at 18, 25, and 38 days and received permanent pacemakers. One patient developed a complete AV block during acute rejection at 76 months and received a temporary pacemaker. Two patients developed chronic sinus tachycardia at 4 and 16 months and were treated with Beta-blockers after eliminating all causes of sinus tachycardia. One patient developed a complete right bundle branch block at 12 months. One patient developed ventricular extrasystole at 10 months and was found to have grade 2 rejection. An Atrial extrasystole was detected in one patient at 96 months. We did not identify significant risk factors for arrhythmias post-HT. Discussion: After pediatric HT, early-onset rhythm disturbances, often attributed to surgery-related issues such as sinus node dysfunction, may necessitate invasive treatments like permanent pacemaker therapy. Close monitoring post-transplantation is crucial, and routine follow-up with Holter ECG is necessary to identify potential rhythm disorders even in the absence of symptoms. Rhythm disturbances that develop during follow-up can serve as early indicators of graft rejection and should be carefully evaluated.

Omitting routine gastric residual checks may help to accelerate enteral feeds and postnatal growth in stable preterm infants.

BACKGROUND: The prefeed gastric residual check (GRC) when increasing the amounts of feeds given via orogastric and nasogastric tubes as a precaution for necrotizing enterocolitis (NEC) and intestinal intolerance is a routine procedure. However, it is mostly misleading, and recently, there has been a tendency not to check prefeed residuals. METHODS: We changed our nutrition protocol at the end of 2018 to start minimal enteral feeds (MEFs) and increase feeds without GRCs. We investigated the effects on the incidence of NEC, complications, time to reach full feeds, and growth parameters RESULTS: We compared the results of 60 preterm infants at ≤35 weeks' gestational age (group 1: 2016-2017, cared for with GRC) and 77 preterm infants (group 2: 2019, without routine GRCs). No differences in incidence of NEC and complications were observed. Group 2 started enteral feeds 3 days earlier, reached total feeds 6 days earlier (P < 0.01), and had higher weight (P < 0.01) and head circumference gain (P < 0.01). Extrauterine growth restriction was significantly less for head circumference and also insignificantly less for weight and height. CONCLUSION: We conclude that starting MEFs earlier and omitting routine GRCs in clinically stable preterm infants accelerate enteral feeds and growth parameters.

Can urinary biomarkers predict acute kidney injury in newborns with critical congenital heart disease?

BACKGROUND: Congenital heart disease (CHD) is the most common congenital malformation group and is the leading cause of newborn mortality in developed countries. Most of the infants with CHD develop preoperative or postoperative acute kidney injury (AKI). Acute kidney injury may develop before the serum creatinine rise and oliguria. Urinary biomarkers such as kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL), interleukin (IL)-18, and cystatin C may predict AKI in patients with critical CHD (CCHD) before the serum creatinine rise. In this study, we aimed to determine the AKI incidence among newborn patients with CCHD and investigate the predictivity of urinary biomarkers for AKI. METHODS: Newborns with a gestational age >34 weeks and birth weight >1500 g with a diagnosis of CCHD were enrolled in the study. Blood and urine samples were collected at birth, during the first 24-48 h, and in the preoperative and postoperative periods. RESULTS: A total of 53 CCHD patients requiring surgery during the neonatal period were enrolled in the study. The 24-48 h KIM-1 levels of the cases with exitus were higher (P = 0.007). The 24-48 h cystatin C and preoperative NGAL levels were higher in patients with postoperative AKI (P = 0.02). DISCUSSION: In newborns with CCHD, high KIM-1 levels may predict mortality, whereas high cystatin C and preoperative NGAL levels may be indicative of AKI. These biomarkers deserve further investigation in larger study populations.

Experience of pandemic influenza with H1N1 in children with leukemia.

It is not exactly known the risks from infection with pandemic influenza (H1N1) 2009 in children with leukemia. Here the authors present their experience in 5 children with leukemia. Pandemic influenza (H1N1) 2009 was detected in 5 patients (F/M: 3/2) at their institution. The ages of these patients were between 2 and 16 years. Four had acute lymphoblastic leukemia (ALL) and 1 acute myeloblastic leukemia (AML). Three of the ALL patients had the diagnosis of pandemic influenza (H1N1) 2009 at the same time as they were diagnosed with ALL. The remaining 2 patients were receiving intensive chemotherapy. All patients had fever, rhinorrhea, and cough. Although bronchopneumonia was seen in 3 patients, only 1 revealed respiratory distress. Stomach ache and diarrhea was seen in the patient who had no pneumonia. All treated as inpatients, but none of them required hospitalization in intensive care unit. One to 3 days after the symptoms of influenza appeared, oseltamivir (Tamiflu) was given to all patients in combination with broad-spectrum antibiotics. Fever declined to normal ranges in 1 to 3 days after treatment was started. The patients received oseltamivir for 5 to 7 days. Cell culture tests were found to be positive for influenza A and polymerase chain reaction (PCR) revealed H1N1 for all 5 patients. Although this is a very small case series, pandemic influenza (H1N1) 2009 did not seem to be very dangerous for children with leukemia if the oseltamivir treatment was given early when symptoms of influenza appeared.