Comparative Outcomes of Levetiracetam and Phenobarbital Usage in the Treatment of Neonatal Seizures: A Retrospective Analysis.

OBJECTIVES AND AIM: The primary aim of this study was to conduct a comparative analysis of the safety and efficacy of levetiracetam (LEV) and phenobarbital (PB) as first-line treatments for neonatal seizure management. This study was designed to measure and compare the incidence of adverse effects and to determine the discharge and mortality rates associated with the use of these antiseizure medications (ASMs). Through this comparison, this research sought to provide insights to optimise care for neonates experiencing seizures. MATERIALS AND METHODS: This retrospective cohort study evaluated 104 neonates treated for seizures at Zeynep Kamil Hospital from 2015 to 2020 after excluding those on non-PB/LEV antiseizure medications. Seizures were characterised using electroencephalogram (EEG) and categorised according to aetiology and frequency. Treatment efficacy was gauged by seizure cessation, as confirmed using EEG. Adverse effects and demographic data were recorded. Statistical analyses were conducted using SPSS, employing the Shapiro-Wilk, independent t-test, Mann-Whitney U test, and chi-square test, with a significance threshold of p < 0.05. RESULTS: Overall, 104 neonates treated with first-line ASM were evaluated for efficacy; PB was administered in 68.26% of the cases, while LEV was utilised in 31.74%. The total complete response rate was 40.38%, with no significant difference between the PB and LEV groups (p = 0.309). The incidence rate ratios (IRRs) demonstrated that seizure frequency profoundly influenced treatment effectiveness, with IRRs of 2.09 for rare seizures, 3.25 for frequent seizures, and 4.01 for status epilepticus, indicating a higher treatment response rate with increasing seizure frequency. For second-line treatment, among a subset of 62 patients, PB had a slight, non-significant advantage over LEV, with an odds ratio of 1.09, suggesting a marginally better response to LEV. Adverse events were significantly more frequent in the PB group, affecting 19 of 67 neonates (28.36%), compared to only 2 of 71 neonates (2.82%) in the LEV group (p < 0.001). No significant difference was observed in the discharge rates between the two groups (PB, 67.61%; LEV, 75.76%; p = 0.674). Interestingly, the mortality rate was significantly higher in the LEV group (45.45%) than that in the PB group (22.54%; p = 0.045). CONCLUSION: This study underscores LEV's superior safety profile over PB in neonatal seizure management, evidenced by a significantly lower rate of adverse events. PB seems to be more effective in the second-line treatment of neonatal seizures. Despite the lack of significant differences in the discharge rates, the higher mortality rate associated with LEV warrants further investigation. These findings advocate the cautious selection of antiepileptic drugs in neonatal care, with a preference for LEV based on its safety profile.

Acute Kidney Injury in Neonatal Hypoxic-Ischemic Encephalopathy Patients Treated with Therapeutic Hypothermia: Incidence and Risk Factors.

Studies in infants with hypoxic-ischemic encephalopathy (HIE) due to perinatal asphyxia have generally focused on neurological outcomes. Although acute kidney injury (AKI) rate decreased in advent of therapeutic hypothermia (TH), it is still a common and important entity. In this retrospective study, we aimed to investigate the risk factors for AKI in HIE patients treated with hypothermia. Infants treated with TH due to HIE were reviewed retrospectively and infants who developed AKI and not were compared. Ninety-six patients were enrolled in the study. AKI developed in 27 (28%) patients and 4 (14.8%) of them were stage III AKI. In the AKI group, gestational age of the patients was significantly higher (p = 0.035), the 1st minute Apgar score was significantly lower (p = 0.042), and convulsions (p = 0.002), amplitude-integrated electroencephalography disorders (p = 0.025), sepsis (p = 0.017), need for inotropic therapy (p = 0.001), need of invasive mechanical ventilation (p = 0.03), and systolic dysfunction in echocardiography (p = 0.022) were significantly higher. In logistic regression tests, Apgar score at the 1st minute was found to be independent risk factor for developing AKI. AKI has the potential to worsen the neurological damage and correlates with morbidities of perinatal asphyxia. It is important to determine the incidence and risk factors for developing AKI in this delicate group of patients to prevent further renal damage.

Neonatal Lymphatic Flow Disorder.

OBJECTIVE: To examine and discuss patients diagnosed with acquired and congenital chylothorax in the neonatal period in the light of the literature. METHODS: The files of newborns followed-up in the neonatal intensive care unit (NICU) and diagnosed with congenital and acquired chylothorax were reviewed retrospectively. Patients with isolated chylothorax were classified as Group 1 and those with multiple lymphatic flow disorders were classified as Group 2. Antenatal and clinical features were recorded and compared between the groups. RESULTS: Thirteen infants were diagnosed with chylothorax; 92.3% (n = 12) of the patients were congenital. The rate of antenatal diagnosis was 61.5% (n = 8). Eight patients (61.5%) were diagnosed with hydrops fetalis. Among the cases in Group 1 and Group 2, receiving ocreotide and the incidence of sepsis (p = 0.05) were partially significant. Seven of the patients (66.6%) responded to medium chain triglycerides (MCT), and complete resolution was seen in 6 (85.7%) of the responders. Complete resolution of chylothorax fluid was observed in 7 (77.7%) of nine patients who responded to ocreotide treatment. CONCLUSIONS: In neonatal chylothorax, the postnatal period includes a multidisciplinary approach that requires drug therapy, dietary modifications, drainage of pleural fluid, and rarely, surgery.

Vasoactive inotropic score as a predictor of mortality in neonatal septic shock.

BACKGROUND: Although many improvements in neonatal care have been achieved, mortality rates for sepsis and septic shock in newborns are still high. The vasoactive inotropic score (VIS) was designed and studied to predict mortality in different settings. There are currently no data on the predictive ability of the VIS for mortality in newborn patients with septic shock. METHODS: Patients with late-onset neonatal sepsis who required inotropes because of fluid-refractory septic shock during the study period were included in the study. Four distinct VIS values were calculated for each septic shock episode after inotropic treatment had begun, that is, at the initiation of inotropic treatment and at 24 and 48 h after inotropic treatment had begun, and the highest VIS (VISmax) at any time after initiation of inotropic agents. RESULTS: The 98 episodes studied were divided into two groups according to the outcomes of their sepsis episodes as survivors (n = 39) or nonsurvivors (n = 59). The areas under the curve of the VIS values for the prediction of mortality were the VISmax (0.819, p < 0.001), followed by the VIS48 (0.802, p < 0.001), VIS24 (0.762, p = 0.001) and VIS0 (0.699, p = 0.015). Patients with a VISmax of greater than 20 had significantly higher odds of mortality (p < 0.001, ß = 14.7, 95% confidence interval [4.7-45.9]). CONCLUSION: We found that the VISmax was an easy-to-use and helpful tool for predicting a poor outcome in neonatal sepsis. Physicians should be aware that the prognosis is poor for any newborn with a VIS of 20 or greater at any point after the onset of sepsis.

Neonatal sepsis is still one of the most important causes of mortality and morbidity in the neonatal period, and it is also a significant public health problem. Researchers have been looking for reliable biomarkers and scoring systems to detect neonatal sepsis and predict outcomes. The vasoactive inotropic score has been validated and found to be useful for predicting mortality in septic shock in adults and children and newborns who underwent cardiac surgeries. However, there are no neonatal sepsis data. In this retrospective study, we showed that a maximal vasoactive inotropic score of 20 or greater is an easy, noninvasive and useful tool to determine the poor outcome.

Early-Onset Neonatal Sepsis in Turkey: A Single-Center 7-Year Experience in Etiology and Antibiotic Susceptibility.

BACKGROUND: The pathogen distribution and antibiotic susceptibility of the pathogens in early-onset sepsis (EOS) differ between countries. The epidemiological data from a limited number of studies about EOS in Turkey are insufficient. In this study, we aimed to evaluate the culture-proven EOS cases, causative microorganisms, antibiotic susceptibility patterns, and risk factors for mortality in EOS. METHODS: This is a retrospective, single-center study over a 7-year period, from 2013 to 2020, at Zeynep Kamil Maternity and Children's Hospital, Istanbul, Turkey. RESULTS: During the study period, 8229 newborns were admitted to our neonatal intensive care unit. Culture-proven EOS was detected in 101 patients (0.12%). Out of these, 56 (55.4%) were Gram-positive, and 45 (44.5%) were Gram-negative sepsis. The most common isolated organism was E. coli (28.7%, n = 29), followed by GBS (16.8%, n = 17) and S. aureus (15.8%, n = 16). An ampicillin and gentamicin combination had antimicrobial coverage in 92.6% of cases. Seventeen patients (16.8%) died because of EOS. Severe neutropenia was found to be an independent risk factor for mortality in EOS (p = 0.001, OR = 14.4, CI 95%: 2.8-74). CONCLUSIONS: Although the majority of causative agents were Gram-positive (55.4%), the most common isolated organism was E. coli. An empirical antibiotic regimen of ampicillin and gentamicin continues to have an adequate coverage for EOS in our population.

G-ROP criteria for predicting retinopathy of prematurity among neonates with different birth weight percentiles.

BACKGROUND: The Postnatal Growth and Retinopathy of Prematurity (G-ROP) Study showed that adding postnatal weight gain to birth weight and gestational age detected 100% of cases with type 1 retinopathy of prematurity (ROP) while reducing the ROP examinations by 30%. The purpose of this study was to investigate whether being small for gestational age (SGA) affects the sensitivity and specificity of the G-ROP model. METHODS: We applied the G-ROP criteria for premature infants. The infants were classified as three subgroups: SGA, appropriate for gestational age (AGA), and large for gestational age (LGA). The performance of G-ROP criteria was assessed for each group for ROP. RESULTS: There were 41 (10.5%) SGA, 312 (80%) AGA, and 37 (9.5%) LGA neonates. Twenty-six (6.7%) neonates were treated for ROP, and the G-ROP model identified all of them. The sensitivity of the model for treatment-requiring ROP (TR-ROP) was found to be 100% in the whole patient group and for each subgroup. The specificity for TR-ROP was 46.4% for the whole group, 50% for SGA, 44% for AGA, and 63.6% for LGA. By applying the G-ROP model, the number of ROP examinations could be reduced by 25% for the whole group, 27% for SGA, 24% for AGA, and 31% for LGA, without missing TR-ROP. CONCLUSIONS: The sensitivity and specificity of the G-ROP model for TR-ROP in SGA infants were similar to the whole group. The model did not miss any cases of TR-ROP.

Factors Associated with the Transition Time to Full Enteral Feeding in Newborns with Hypoxic Ischemic Encephalopathy.

BACKGROUND: We aimed to assess the factors associated with the transition time to full enteral feeding (FEF) in newborns with hypoxic ischemic encephalopathy (HIE) undergoing therapeutic hypothermia. METHODS: We obtained data retrospectively from medical records of the neonates diagnosed with HIE and treated by therapeutic hypothermia to evaluate the factors associated with transition time to FEF. RESULTS: Sixty-one neonates were included in the study. The median gestational age (GA) and birth weight were 39 (37-40) weeks and 3245 (2715-3575) grams, respectively. APGAR scores at the first and fifth minutes were 3 (1-5) and 6 (4-7), respectively. Fifty-seven (93.4%) of the newborns were diagnosed as having moderate HIE, and 4 (6.6%) of them had severe HIE. Transition time to FEF was found to be negatively correlated with gestational week (r, P: -0.280, 0.029) and birth weight (r, P: -0.315, 0.013); and positively correlated with lactate (r, P: 0.295, 0.044), BUN (r, P: 0.285, 0.026) and creatinine levels (r,P: 0.345, 0.007); duration of invasive (r, P: 0.565, 0.0001) and non-invasive mechanical ventilation (r, P: 0.261, 0.042), use of antibiotics (r, P: 0.556, 0.0001) and inotropic agents (r, P: 0.524, 0.0001) and hospitalization (r, P: 0.654, 0.0001). CONCLUSION: Clinicians should be more careful while starting to feed babies undergoing therapeutic hypothermia with higher lactate levels and impaired renal functions, and should be encouraged to feed clinically stable neonates with HIE as soon as possible, as the transition time to FEF could be related with better clinical outcomes.

Acute Peritoneal Dialysis in Premature Infants.

OBJECTIVE: This study aimed to investigate the underlying causes and outcomes of less than 1500 g birth weight infants who underwent acute peritoneal dialysis (PD). METHODS: Case records of infants with birthweight less than 1500 g from January 2015 to June 2018 were reviewed. RESULTS: The median (range) birth weight and gestational age of the patients were 720 g (555-1055) and 26 weeks (23-27.5), respectively. Underlying factors for the development of acute kidney injury (AKI) were patent ductus arteriosus (PDA) (15 patients), necrotizing enterocolitis (NEC) (10 patients), sepsis (7 patients), asphyxia (2 patients) and hydrops fetalis (2 patients). Multifunctional 10 F flexible catheter was used for the procedure. Median PD onset time was 7 days (4.5-13.5) and median PD duration was 3 days (1.5-3.5). Overall mortality rate was 81 % (n=17). CONCLUSIONS: Despite high overall mortality, PD is technically feasible in very low birthweight (VLBW) and extremely low birthweight (ELBW) neonates using a multifunctional catheter.

Is the First Postnatal Platelet Mass as an Indicator of Patent Ductus Arteriosus?

BACKGROUND: The aim of this study is to evaluate whether there is an association between the platelet mass and patent ductus arteriosus (PDA) closure in premature newborns. METHODS: Preterm infants (gestational age ≤33 weeks) with hemodynamically significant PDA (group 1, n = 178) and a control group of preterm infants without PDA (group 2, n = 211) were retrospectively evaluated between August 1, 2013 and July 30, 2015 in the neonatal intensive care unit (NICU). Platelet counts and platelet indices including mean platelet volume (MPV), and platelet mass (platelet count x mean platelet volume) in the first 24 hours of life, demographic findings and morbidities were recorded. RESULTS: No differences were observed in demographic findings between the study groups in terms of birth weight, gestational age, gender and maternal risk factors. The mean platelet count in the first postnatal hemogram in group 1 and group 2 were 189.43 ± 72.14 (X103 /mm3) and 206.86 ± 70.11(X103/mm3), respectively (P < 0.05). The MPV were similar in both groups (P > 0.05). Platelet mass values were 1443.70 ± 572.40 fL/nL in Group 1 and 1669.49 ± 1200.42 fL/nL in group 2. There was a statistically significant difference in platelet mass values between the two groups (P = 0.011). Multivariable analysis including presence of thrombocytopenia, MPV and platelet mass showed that hemodynamically significant PDA was not independently associated with platelet count <150 000 (OR = 1.001, 95% CI 0.980-1.023; P = 0.921), MPV (OR = 0.967, 95% CI 0.587-1.596; P = 0.897) or platelet mass (OR = 0.999, 95% CI 0.997-1.002; P = 0.681). The optimal cut-off value of platelet mass for patients with PDA was ≤1530.8 fL/nL (area under the curve [AUC]: 0.580), with sensitivity of 58% and specificity of 56.2% (P = 0.008). CONCLUSION: Our data suggest that platelet count, MPV, and platelet mass do not contribute to closure of PDA in premature newborns.

Efficacy of levetiracetam in premature infants: our experience and review of the literature.

Objective: To evaluate levetiracetam (LEV) efficacy in preterm infants admitted in NICU.Study design: Clinical characteristics of 26 preterm infants treated with LEV were evaluated retrospectively. The results were compared with those of 44 preterm infants from the literature who were given LEV.Result: The mean gestational week of the infants receiving LEV was found as 26.7 ± 3.3 weeks, mean birth weight as 938 ± 561 g and mean dose of LEV as 17 ± 9.23 mg/kg. Overall seizure control rate with LEV was found as 65%, while seizure control was achieved by 11.5% when it was used as the first drug, 35% as the second drug and 15.3% as the third drug. The incidence of sepsis and intraventricular hemorrhage in seizure etiology was 73% in infants who received LEV. There was no side effect observed during LEV treatment.Conclusions: Seizure control was better achieved with LEV given as the 2nd antiepileptic in premature infants. Further studies with randomization of LEV and other antiepileptics in seizure control are needed.

Neonatology oxidative status in preterm infants with premature preterm rupture of membranes and fetal inflammatuar response syndrome.

BACKGROUND: The aim of this study, to determine an index of oxidative stress index in preterm infants less than 34 weeks gestational age with premature preterm rupture of membrane (PPROM) and fetal inflammatory response syndrome (FIRS). METHODS: This study was designed as a prospective study. Fifty-one premature infants less than 35 weeks of gestational age were included in the study. The umbilical cord blood concentrations of IL-6, TAC (total antioxidant capacity) and PON-1 (paraoxonase-1) levels and TOS (total oxidative stress) were studied. The oxidative stress index (OSI = TAC/TOS) was calculated in all of prematüre infants. PPROM was defined as rupture of membranes at least 24 hours before the onset of labor. FIRS was defined by an umbilical cord IL-6 level greater than 11 pg/mL. Premature infants included in the study were divided into 4 groups. Group 1 included preterm infants without FIRS and with PPROM (n = 16), while Group 2 included preterm infants without PPROM and with FIRS (n = 9), Group 3 consisted of premature infants with PPROM and FIRS (n = 21) and Group 4 included premature infants without PPROM or FIRS (n = 5). RESULTS: Umbilical cord TOS level was found to be higher in the preterm infants without FIRS and with PPROM (36.1 µmol H2O2 Equiv./L) compared to the preterm infants without PPROM or FIRS (11.9 µmol H2O2 Equiv./L) (p = 0.03). Umbilical cord PON-1 level was found to be lower in the preterms without FIRS and with PPROM (32 U/L), preterms without PPROM and with FIRS (30. 3 U/L) and the preterm infants with both PPROM and FIRS (48.6 U/L) compared to the preterm infants having no PPROM or FIRS (85.6 U/L) (p = 0.001). CONCLUSION: High pro-oxidant capacity was found in PPROM and low antioxidant capacity in PPROM and FIRS.

Serum lactate levels and perfusion index: are these prognostic factors on mortality and morbidity in very low-birth weight infants?

AIM: Early hemodynamic assessment of global parameters in critically ill newborns fails and requires mostly invasive measurements in neonatal intensive care unit. Clinical signs are frequently used for assessment of peripheral perfusion. Perfusion index (PI) is a new noninvasive numerical value of peripheral perfusion. Serum lactate levels and PI are the indicators that are important in determining prognosis of preterm infants. In this study, we aimed to investigate the relationship of serum lactate levels and PI with mortality and morbidity in very low-birth weight infants (VLBW). STUDY DESIGN: This study was conducted between July 2014 and July 2015 in a Level III NICU. The study enrolled preterm infants with a gestational age ≤ 32 weeks, birth weight ≤ 1500 g. Serum lactate levels from blood gases and PI, SpO2 measurements were recorded at 1st, 12th and 24th hours by using a new generation pulse-oximeter. Morbidities and mortalities were documented. RESULTS: A total of 60 VLBW infants were enrolled the study. Mean birth weight and gestational age were 991 ± 288 g and 27.5 ± 2.5 w, respectively. Retinopathy of prematurity (ROP) was significantly higher in the patients with high lactate levels (>4 mg/dl) at 1st hour and low-PI levels (<0.5) at 12th hour of life (p = 0.042, p = 0.015), respectively. Bronchopulmonary displasia (BPD) was significantly higher in the patients with low PI (< 0.5) at 1st hour. Lactate and PI values were not significantly correlated with necrotizing enterocolitis, intraventricular hemorrhage, patent ductus arteriosus, sepsis and mortality. CONCLUSION: High lactate levels (> 4 mg/dl) and low PI (< 0.5) could be used as early parameters for prediction of ROP and BPD. This data suggests that in VLBW infants lactate levels and PI parameters during the first 24 h will be effective in determining the prognosis of the disease. We believe that larger, randomized controlled clinical trials are likely to establish the true benefit.

Does platelet mass influence the effectiveness of ibuprofen treatment for patent ductus arteriosus in preterm infants?

OBJECTIVE: The aim of this study is to evaluate whether the platelet mass in the first 24 h of life is effective on closure of patent ductus arteriosus (PDA) or not. STUDY DESIGN: Preterm infants with a gestational age of < 32 weeks, hospitalized at a tertiary neonatal intensive care unit (NICU) and requiring medical treatment (intravenous or oral ibuprofen) for hemodinamically significant PDA (hsPDA) were enrolled in this study. The patients were divided into two groups after first course of pharmacologic treatment according to closure of PDA (Group 1: PDA closure, Group 2: PDA without closure). Groups were compared in terms of demographics findings, morbidities, platelet measurements like counts, mean platelet volume (MPV) and platelet mass (platelet count × mean platelet volume). RESULTS: The study included 77 preterm newborns in Group 1, and 30 preterms in Group 2. There were no differences in birth weight, gestational age, gender and maternal risk factors between the study groups. The mean platelet count in the first postnatal blood count was in Group 1: 211.3 ± 89.2 × 10(3)/mm(3) and in Group 2: 216.5 ± 26 × 10(3)/mm(3), respectively (p = 0.783). The mean platelet volumes (MPV) were similar in both groups (p = 0.535). No statistically significant difference between platelet mass values was detected (Group 1: 1811 ± 884 fl/nl, Group 2: 1868 ± 717 fl/nl) (p = 0.753). CONCLUSION: Our data suggest that platelet count, MPV and platelet mass did not affect the closure of hsPDA with ibuprofen.

Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease.

Prostaglandin E1 is crucial for keeping the patent ductus arteriosus in critical congenital heart disease for the survival and palliation of particularly prematurely born babies until a cardiosurgical intervention is available. In this study, the side effects of prostaglandin E1 in newborns with critical congenital heart disease and clinical outcomes were evaluated. Thirty-five newborns diagnosed with critical congenital heart disease were treated with prostaglandin E1 between January 2012 and September 2014 at our hospital. Patient charts were examined for prostaglandin E1 side effects (metabolic, gastric outlet obstruction, apnea), clinical status, and prognosis. Acquired data were analyzed in the SPSS 20.0 program. Patients with birth weight under 2500 g needed more days of prostaglandin E1 infusion than ones with birthweight over 2500 g (P = 0.016). The ratio of patients with birth weight under 2500 g who received prostaglandin E1 longer than 7 days was higher than the patients with birth weight over 2500 g (P = 0.02). Eighteen side effects were encountered in 11 of 35 patients (31%). Of these side effects, 1 patient had 4, 4 patients had 2, and 6 patients had only 1 side effect. Discontinuation of the therapy was never needed. Prostaglandin E1 is an accepted therapy modality for survival and outcome in critical congenital heart disease in particularly low-birth-weight babies until a surgical intervention is available. Side effects are not less encountered but are almost always manageable, and discontinuation is not needed.

Morbidity in preterm infants with fetal inflammatory response syndrome.

BACKGROUND: The aim of this study was to evaluate the relationship between umbilical cord blood interleukin (IL)-6 concentration and preterm morbidity and mortality in premature infants born with fetal inflammatory response syndrome (FIRS). METHODS: This prospective, observational study included 84 preterm infants with a gestational age of 24-36 weeks who had been admitted to the neonatal intensive care unit (NICU). FIRS was defined as umbilical cord blood IL-6 > 11 pg/mL. In premature infants with FIRS, morbidities (multiple organ failure [MOF], respiratory distress syndrome [RDS], patent ductus arteriosus, intraventricular hemorrhage, bronchopulmonary dysplasia, retinopathy of prematurity) and death were evaluated. Critical umbilical cord blood IL-6 concentrations for the development of RDS, death, and for MOF were determined in premature infants with FIRS. RESULTS: Fifty-two infants with IL-6 concentration > 11 pg/mL constituted the FIRS group. Thirty-two infants without FIRS served as a control group. RDS, MOF, and mortality were significantly higher in the FIRS group (P = 0.001, P = 0.001, and P = 0.005, respectively). Umbilical cord blood IL-6 concentration > 26.7 pg/mL in the FIRS group was found to be predictive of RDS, with 70% sensitivity and 85% specificity. Umbilical cord blood IL-6 concentration > 37.7 pg/mL was found to be predictive of death, with 78.6% sensitivity and 60% specificity. The predictive value of IL-6 for the development of MOF was 17.5 pg/mL, with 91% sensitivity and 66% specificity. CONCLUSIONS: Umbilical cord blood IL-6 concentration > 26.7, 37.7, and 17.5 pg/mL in premature infants with FIRS was found to be predictive for RDS, death, and MOF, respectively.

Early screening for critical congenital heart defects in asymptomatic newborns in Bursa province.

OBJECTIVE: In this study, pulse oximetry screening results in the early diagnosis of critical congenital heart diseases (CCHD) will be evaluated. METHODS: Eight-thousand two-hundred and eight of 10,200 newborns born between January 2014 and December 2014 were screened using pulse oximetry for the diagnosis of CCHD. Screening test was considered to be positive in the newborns whose saturation after 24 h from the birth with pulse oximetry was ≤ 95% and/or in the newborns who had a difference of ≥ 3% between the lower and right upper extremity. RESULTS: Incidence of CCHD was 1 per 1000 live births. Coarctation of the aorta was the most commonly determined CCHD. Sensitivity, specificity, false negative rate and false positive rate of pulse oximetry in the diagnosis of CCHD were 60%, 99.8%, 40% and 0.12%, respectively. Seventy-five percent of the newborns who had a false negative diagnosis with pulse oximetry had coarctation of the aorta. Coarctation of the aorta was determined at a rate of 20% using CCHD screening. CONCLUSIONS: The diagnosis of coarctation of the aorta is missed in the newborns screened with pulse oximetry in the first 24-48 h after birth. Screening with pulse oximetry should be repeated for early diagnosis of coarctation of the aorta.

Familial Mediterranean fever gene mutation frequencies and genotype-phenotype correlations in the Aegean region of Turkey.

Familial Mediterranean fever (FMF) is a disease characterized by recurrent, self-limiting fever and serositis and caused by altered pyrin due to mutated MEFV gene. The aim of this study was to investigate clinical manifestations and MEFV mutations among patients with FMF and healthy controls in the Aegean region of Turkey. This study included 308 patients and 164 healthy controls. Patients were divided into three groups according to Tel-Hashomer criteria; definitive, probable, and suspicious. Among the patients, 146 were women (47.4%) and 162 were men (52.6%). The mean age (±SD) of the patients at the diagnosis was 9.6±3.95 (range 0.5-18). The mean age (±SD) at onset of the symptom was 6.2±3.95 (range 1-18). Symptoms were seen earlier onset in definitive group than the suspicious group in our cohort (4.7±3.9 years, 6.6±3.9 years, respectively; P=0.001). Clinical features were abdominal pain (83.1%), fever (55%), arthritis (17.1%), myalgia (4.5%), pleuritis (10%), and erysipelas-like erythema (7.7%). Fever, arthralgia, arthritis, chest pain, and amyloidosis were found statistically significant more in definitive group than suspicious group (P<0.001, P<0.001, P<0.001, P<0.05, and P<0.001, respectively). MEFV gene mutations were identified in 199 patients (64.6%). The most commonly encountered MEFV mutation among the patients was M694V homozygote (25%). M694V homozygous mutation was found most frequently in definitive FMF group than other groups (49, 9, 8.9%, respectively). To our knowledge that FMF should be suspected in the case of non-specific but recurrent attacks of serositis and high fever, and molecular analysis should be performed in order to make diagnosis of FMF.

Kluyvera cryocrescens sepsis in a preterm infant.

Kluyvera cryocrescens, formerly accepted as a benign saprophytic microorganism, is an opportunistic pathogen and its infection is very rare in humans. This report describes a preterm infant born at 30 weeks of gestational age and successfully treated for K. cryocrescens sepsis in the 3rd week of life. To our knowledge, this is the first case of K. cryocrescens sepsis in a newborn. The potential of K. cryocrescens as a serious pathogen should be recognized especially in patients such as preterm infants in whom the prognosis may be compromised without appropriate treatment.