Kyphectomy in neonates with meningomyelocele.

PURPOSE: Kyphosis is the most severe spinal deformity associated with meningomyelocele (MMC) and is seen in approximately 15% of neonates. Our purpose is to present our clinical experience, to discuss the technique and deformity correction in kyphectomy in neonates with MMC, and to assess its long-term outcomes. METHOD: In this prospective study, the authors reviewed eight cases submitted to surgery between 2013 and 2015. We evaluated clinical characteristics that were analyzed, as were the operative technique employed, and angle range of the kyphosis deformity postcorrection follow-up. RESULTS: Neonatal kyphectomy was performed of six females and two males. The mean birth weight was 2780 g, and the mean age at the time of surgery was 5.6 days. There were S-shaped type deformity in lumbar region in all neonates. In the correction of the kyphotic deformity, a total vertebrae were removed from four patient, whereas a partial vertebrectomy was done in four. The mean operative time was 116 min. No patients did not require the blood transfusion. There were no serious complications, and wound closure was successful in all patients. The mean follow-up period was 4 years and 3 months (range 36-61 months), except one patient who died 1 week after discharge. The mean preoperative kyphosis of 75.6° (range, 50°-90°) improved at last follow-up to 35° (range 15°-55°). All patients had surgical procedures for hydrocephalus. Three patients had surgery for Chiari type II malformation. The mean hospital stay was 27.7 days. CONCLUSION: Kyphectomy performed at the time of dural sac closure in the neonate is a safe procedure with excellent correction.

Comparison of neonatal transport scoring systems and transport-related mortality score for predicting neonatal mortality risk.

OBJECTIVES: To predict the risk of mortality of neonates, birth weight and gestational age were previously used. However, these criteria were considered inadequate; therefore, various scoring systems have been developed in the recent years. The aim of the study was to evaluate the performance of predicting mortality by Mortality Index for Neonatal Transportation (MINT), Score for Neonatal Acute Physiology-Perinatal Extension II (SNAP-PE-II), and Transport Related Mortality Score (TREMS). METHODS: All infants transferred to the neonatal intensive care unit between January 1 and December 31, 2011, were included. The scores of SNAP-PE-II, MINT, and TREMS of the all cases were calculated. TREMS is our proposed scoring system and it consists of 5 variables (hypoglycemia, hypoxia, hypercarbia, hypotension, and hypothermia). The scoring systems, SNAP-PE-II, MINT, and TREMS, were compared in terms of mortality risk. RESULTS: A total of 306 newborn infants constituted the study population. The mean gestational age was 33.1 ± 5 weeks and the mean birth weight was 2031.2 ± 1018 g, and 183 (59%) babies were male. The sensitivity of MINT score for predicting mortality was higher than SNAP-PE-II and TREMS. However, specificity was higher in TREMS score. The negative predictive value was highest in MINT score, whereas TREMS has the highest positive predictive value. CONCLUSIONS: The TREMS scoring system is a simple scoring system with a high specificity for predicting mortality. Further studies with larger sample size including more centers and newborn infants with diverse clinical problems are needed to assess the validity and reliability of the TREMS scoring system.

Effects of synchronized intermittent mandatory ventilation versus pressure support plus volume guarantee ventilation in the weaning phase of preterm infants*.

OBJECTIVE: To compare the effects and short-term outcomes of pressure support ventilation with volume guarantee versus synchronized intermittent mandatory ventilation in the weaning phase of very low-birth weight infants with respiratory distress syndrome. DESIGN: Randomized controlled prospective study. SETTING: Tertiary care neonatal unit. PATIENTS: A total of 60 premature infants who were less than 33 weeks' gestation and/or less than 1,500 g birth weight and received mechanical ventilation because of respiratory distress syndrome were studied. INTERVENTIONS: All infants were ventilated from the time of admission with synchronized intermittent positive pressure ventilation mode after surfactant treatment for respiratory distress syndrome and then switched to pressure support ventilation with volume guarantee or synchronized intermittent mandatory ventilation mode in the weaning phase. The ventilatory variables and neonatal outcomes were recorded in each group. MEASUREMENTS AND MAIN RESULTS: The mean peak inflation pressure was higher in synchronized intermittent mandatory ventilation group (p < 0.001) and the mean airway pressure was higher in pressure support ventilation with volume guarantee group (p = 0.03), whereas mean tidal volume and respiratory rates were similar in both groups. The prevalence of postextubation atelectasis was higher in synchronized intermittent mandatory ventilation group, but the difference was not statistically significant (p = 0.08). No differences were found in the prevalence of reintubation, patent ductus arteriosus, intraventricular hemorrhage, retinopathy of prematurity, bronchopulmonary dysplasia, and pneumothorax between the groups. CONCLUSIONS: Pressure support ventilation with volume guarantee mode may be a safe and feasible mode during the weaning phase of very low-birth weight infants on mechanical ventilation support for respiratory distress syndrome with respect to reducing the frequency of postextubation atelectasis and using less peak inflation pressure.

Cranial sonography in extremely preterm infants.

Survival rates of extremely preterm infants have risen dramatically, paralleling improvements in prenatal and neonatal care. Cranial sonography is the primary imaging technique for the evaluation of brain injury in these patients. Extremely preterm infants have some unique features related to ongoing maturation, and associated findings on cranial sonography should be considered for accurate and early diagnosis. The aim of this pictorial essay is to illustrate normal anatomy, normal variants, and pitfalls that lead to misinterpretation of cranial sonography in extremely preterm infants.

Reference ranges for sonographic renal dimensions in preterm infants.

BACKGROUND: Ultrasonographic measurement of kidney dimensions is important in evaluation of renal disease in preterm infants who have multiple comorbidities that affect renal function. OBJECTIVE: The purpose of this study was to determine the reference ranges of kidney dimensions in preterm newborns and to provide a chart to use easily in daily practice. MATERIALS AND METHODS: We evaluated renal dimensions in 498 preterm infants with a gestational age of <37 weeks using sonography within the first week of life. We statistically analyzed the relationships between all dimensions and gender, gestational age (based on the last menstrual period), height and weight. Minimum and maximum values of dimensions were defined. RESULTS: All dimensions of the kidneys were statistically different in boys and girls (P < 0.05). Both longitudinal and anteroposterior dimensions of the right and left kidneys showed high correlation with gestational age, weight and height in girls and boys. Weight correlated best with dimensions. CONCLUSION: Nomograms from these data can be used to determine an abnormality in kidneys of preterm newborns.

Reference ranges for sonographic dimensions of the liver and spleen in preterm infants.

BACKGROUND: Preterm infants usually have multiple comorbidities that affect spleen and liver. Ultrasonographic measurement of organ sizes is an important and reliable parameter in evaluation of spleen and liver pathology in preterm newborns. OBJECTIVE: The purpose of this study was to determine reference values of ultrasonographic measurements of the liver and spleen in preterm newborns. MATERIALS AND METHODS: We prospectively performed sonography on 498 preterm newborns in the first week of life. We measured spleen and liver dimensions and statistically analyzed relationships between the dimensions and gender, gestational age (based on mother's last menstrual period), height and weight. Reference ranges of dimensions were defined. RESULTS: Longitudinal and anteroposterior dimensions of the liver and spleen were statistically significantly different between the boys and girls (P < 0.05) and showed high correlation with the gestational age, weight and height. Weight was the parameter best correlated with the dimensions. CONCLUSION: Nomograms from these data are useful for sonographic evaluation of the liver and spleen in preterm newborns.

Escherichia coli brain abscess in a twin pair associated with TLR4 gene mutation.

Brain abscesses are uncommon complications of bacterial meningitis or sepsis in neonates and infants. The causative pathogens of brain abscess in newborns are various. Of those, Escherichia coli is rarely seen as a pathogen in brain abscess at this age. Herein we reported brain abscesses in twin infants caused by E. coli sepsis. Interestingly, genetic analysis identified heterozygous Toll-like receptor 4 (TLR4) gene mutation in the twins. Because TLR plays an important role in the natural response to bacterial products and initiates specific immune response against these pathogens, this may explain the development of brain abscess in the present case.

Central nervous system involvement of epstein barr virus associated lymphoproliferative disorder in a child with acute lymphoblastic leukemia: successful treatment with rituximab and interferon-alpha.

UNLABELLED: Central nervous system (CNS) involvement of Epstein-Barr virus (EBV)-associated lymphoproliferative disease is a rare and serious complication in children with leukemia. Although rituximab therapy seems to be promising in these cases, persistent hypogammaglobulinemia may appear after treatment due to complete depletion of normal B lymphocytes in the peripheral blood. Here we report isolated CNS involvement of EBV-associated lymphoproliferative disorder in a 4-year-old boy with acute leukemia. The patient was treated with rituximab and interferon alpha; however, persistent hypogammaglobulinemia developed as a complication. Given the rarity of the complication in children receiving these agents, our experience with such a case may be helpful to others. CONFLICT OF INTEREST: None declared.

Mutation of the proton-coupled folate transporter gene (PCFT-SLC46A1) in Turkish siblings with hereditary folate malabsorption.

Hereditary folate malabsorption (HFM) is a rare autosomal recessive disorder characterized by systemic and central nervous system folate deficiency. Turkish siblings are reported with the clinical syndrome of HFM, homozygous for deletion of 2 bases (c.204_205 delCC) within the first exon of the proton-coupled folate transporter (PCFT) gene, causing a frameshift. Low blood and cerebrospinal fluid folate levels were detected at ages 3.5 and 1 month. Treatment with parenteral 5-formyltetrahydrofolate resulted in normal development now at ages 3 and 1 year. Extending current knowledge on the phenotypic manifestations of HFM and the PCFT mutation spectrum will provide opportunities to define possible genotype-phenotype correlations and clarify the basis for the phenotypic variability that is characteristic of this disorder.

Value of urinary kidney injury molecule-1 levels in predicting acute kidney injury in very low birth weight preterm infants.

OBJECTIVE: This study aimed to evaluate the significance of urinary kidney injury molecule-1 (uKIM-1) levels in predicting acute kidney injury (AKI) and mortality in very low birth weight (VLBW) preterm infants. METHODS: This prospective, observational cohort study was conducted on 39 VLBW preterm infants. Serum creatinine (SCr) and uKIM-1 levels were measured in the first 24 and 48 to 72 hours of life. The estimated glomerular filtration rate (eGFR) was calculated. Levels of uKIM-1 were measured with an enzyme-linked immunosorbent assay. RESULTS: Among 39 VLBW infants, 9 (23%) developed AKI. The mortality rate was 17.9% (n = 7 neonates). There was no significant difference in SCr levels, uKIM-1 levels, or the eGFR obtained in the first 24 hours in the AKI group compared with controls. However, significant differences were found in SCr and uKIM-1 levels, and the eGFR rate at 48 to 72 hours between the groups. Levels of uKIM-1 were significantly higher in non-survivors than in survivors in the first 24 and 48 to 72 hours of life. CONCLUSION: The level of uKIM-1 can be used as a simple noninvasive diagnostic method for predicting AKI and mortality, especially within 48 to 72 hours of life.Clinical trial registration: We do not have a clinical trial registration ID. In Turkey, clinical trial registration is not required for non-drug, noninvasive, clinical studies.