Role of Systemic Inflammatory Indices in the Prediction of Moderate to Severe Bronchopulmonary Dysplasia in Preterm Infants

Introduction: The role of systemic inflammatory indices in the diagnosis of bronchopulmonary dysplasia (BPD) is unknown. The aim of the study was to determine the possible clinical utility of systemic inflammatory indices in the prediction of moderate to severe BPD. Methods: Premature infants<32 weeks of gestational age were included in the study. Neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR), systemic immune-inflammation index (SII), pan-immune-inflammation value (PIV), and systemic inflammation response index (SIRI) were calculated at birth and at the time of diagnosis of BPD (at 36th weeks of postmenstrual age). The patients were divided into two groups as no or mild BPD and moderate or severe BPD. Results: A total of 1146 infants were included in the study, 957 in Group 1 and 189 in Group 2. The SIRI value was significantly higher in moderate or severe BPD both at birth and at the 36th week of postmenstrual age (p<0.001 and p<0.001, respectively). The AUC value of SIRI was 0.809 and the cut-off value was>0.98 in the predictivity of BPD at birth. The AUC value of SIRI was 0.842 and the cut-off value was>1.33 for the diagnosis of BPD at 36th week of postmenstrual age. After multiple logistic regression analysis, SIRI was shown to be a significant parameter for the diagnosis of BPD (OR 2.847, 95% CI 1.557–4.875). Conclusions: SIRI may be a useful biomarker for predicting moderate to severe BPD and a marker of clinical importance in the follow-up of infants with BPD. (AU)

Hemoglobin/Red Cell Distribution width Ratio (HRR): A Novel and Promising Red Cell Parameter in Ductal Closure. / Proporção de Largura de Distribuição de Hemoglobina/Glóbulos Vermelhos (HRR): Um Novo e Promissor Parâmetro de Glóbulos Vermelhos no Fechamento Ductal.

BACKGROUND: It is still unclear how effective hematological parameters are in the closure of patent ductus arteriosus (PDA). OBJECTIVES: The primary aim of our study is to investigate the effect of hemoglobin (HB)-to-red cell distribution width (RDW) ratio (HRR) on the closure of PDA. METHODS: Premature babies with very low birth weight (VLBW: <1500 g) and <32 gestational weeks were included in the study, and all data were recorded retrospectively. Demographic characteristics, clinical results, red cell parameters, and HRR and their ratios were compared between hemodynamically significant PDA (hsPDA) and non-hsPDA groups. All results were statically analyzed, and P<0.05 was considered statistically significant. RESULTS: A total of 677 premature babies, 269 in the hsPDA group and 408 in the non-hsPDA group, were included in the study. Hemoglobin (HB), hematocrit (HCT), mean cell volume (MCV), red blood cell (RBC), red cell distribution width (RDW), mean platelet volume (MPV), MCV/RBC ratio, HB/RBC ratio, RDW/RBC ratio, and RDW/MPV ratio were found to be similar between hsPDA and non-hsPDA groups, (p>0.05). HRR was found to be significantly lower in the hsPDA group [median (Quartile 1 (Q1) - Q3) (Q1 - Q3): 0.93 (0.8-1.0)] compared to non-hsPDA [median ( Q1 - Q3): 1.07 (1.0-1.2)] (p<0.001). The AUC for the diagnostic value of HRR in hsPDA was 0.816, and the cutoff value was ≤0.98 (p<0.001, 95% [CI]: 0.785-0.845, sensitivity: 90%, specificity: 92%). CONCLUSIONS: HRR value was found to be both an effective and powerful parameter in diagnosing hsPDA.

FUNDAMENTO: Ainda não está clara a eficácia dos parâmetros hematológicos no fechamento da persistência do canal arterial (PCA). OBJETIVOS: O objetivo principal do nosso estudo é investigar o efeito da proporção (HRR) de largura de distribuição de hemoglobina (HB) para glóbulos vermelhos (RDW) no fechamento do PCA. MÉTODOS: Bebês prematuros com muito baixo peso ao nascer (MBPN: <1.500 g) e <32 semanas gestacionais foram incluídos no estudo, e todos os dados foram registrados retrospectivamente. Características demográficas, resultados clínicos, parâmetros de hemácias e HRR e suas proporções foram comparados entre grupos de PCA hemodinamicamente significativa (hsPDA) e não-hsPDA. Todos os resultados foram analisados estatisticamente, e p<0,05 foi considerado estatisticamente significativo. RESULTADOS: Um total de 677 bebês prematuros, 269 no grupo hsPDA e 408 no grupo não-hsPDA, foram incluídos no estudo. Hemoglobina (HB), hematócrito (HCT), volume celular médio (VCM), glóbulos vermelhos (RBC), largura de distribuição dos glóbulos vermelhos (RDW), volume plaquetário médio (VPM), relação VCM/RBC, relação HB/RBC, RDW A razão /RBC e a razão RDW/VPM foram semelhantes entre os grupos hsPDA e não hsPDA, (p>0,05). HRR foi significativamente menor no grupo hsPDA [mediana (Quartil 1 (Q1) - Q3) (Q1 - Q3): 0,93 (0,8-1,0)] em comparação com não-hsPDA [mediana (Q1 - Q3): 1,07 ( 1,0-1,2)] (p<0,001). A AUC para o valor diagnóstico de HRR em hsPDA foi de 0,816 e o valor de corte foi ≤0,98 (p<0,001, 95% [IC]: 0,785-0,845, sensibilidade: 90%, especificidade: 92%). CONCLUSÕES: O valor de HRR foi considerado um parâmetro eficaz e poderoso no diagnóstico de hsPDA.

Evaluation of the Effectiveness of Systemic Inflammatory Indices in the Diagnosis of Respiratory Distress Syndrome in Preterm with Gestational Age of ≤32 Weeks.

OBJECTIVE: It is not yet known whether systemic inflammatory indices affect the development of respiratory distress syndrome (RDS) in premature infants. We aimed to evaluate the relationship between systemic inflammatory indices obtained on the first day of life and the development of RDS in premature infants. STUDY DESIGN: Premature infants with gestational age of ≤32 weeks were included in the study. Six systemic inflammatory indices involving neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR), systemic immune-inflammation index (SII), pan-immune-inflammation value (PIV), and systemic inflammation response index (SIRI) were measured in the first 1 hour after birth and compared in premature infants with and without RDS. RESULTS: A total of 931 premature infants, 579 infants in the RDS group and 352 infants in the non-RDS group, were included in the study. MLR, PLR, and SIRI values were similar between the groups (p > 0.05 for all parameters). NLR, PIV, and SII values in the RDS group were significantly higher than those in the non-RDS group (p = 0.005, p = 0.011, and p < 0.001, respectively). In the predictivity of RDS, the AUC value of SII was 0.842 and the cut-off value was ≥78.200. Multiple logistic analysis showed that a higher level of SII (≥78.2) was independently associated with RDS (odds ratio: 3.03, 95% confidence interval: 1.761-5.301). CONCLUSION: Our results demonstrated that a higher SII level (≥78.2) may be a predictor for the development of RDS in premature infants with gestational age of ≤32 weeks. KEY POINTS: · It is not yet known whether systemic inflammatory indices affect the development of RDS.. · Our results demonstrated high SII levels may be a predictor for the development of RDS.. · SII may provide an advantage as a low-cost, easy-to-detect, useful and powerful parameter in RDS..

Role of Systemic Inflammatory Indices in the Prediction of Moderate to Severe Bronchopulmonary Dysplasia in Preterm Infants.

INTRODUCTION: The role of systemic inflammatory indices in the diagnosis of bronchopulmonary dysplasia (BPD) is unknown. The aim of the study was to determine the possible clinical utility of systemic inflammatory indices in the prediction of moderate to severe BPD. METHODS: Premature infants<32 weeks of gestational age were included in the study. Neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR), systemic immune-inflammation index (SII), pan-immune-inflammation value (PIV), and systemic inflammation response index (SIRI) were calculated at birth and at the time of diagnosis of BPD (at 36th weeks of postmenstrual age). The patients were divided into two groups as no or mild BPD and moderate or severe BPD. RESULTS: A total of 1146 infants were included in the study, 957 in Group 1 and 189 in Group 2. The SIRI value was significantly higher in moderate or severe BPD both at birth and at the 36th week of postmenstrual age (p<0.001 and p<0.001, respectively). The AUC value of SIRI was 0.809 and the cut-off value was>0.98 in the predictivity of BPD at birth. The AUC value of SIRI was 0.842 and the cut-off value was>1.33 for the diagnosis of BPD at 36th week of postmenstrual age. After multiple logistic regression analysis, SIRI was shown to be a significant parameter for the diagnosis of BPD (OR 2.847, 95% CI 1.557-4.875). CONCLUSIONS: SIRI may be a useful biomarker for predicting moderate to severe BPD and a marker of clinical importance in the follow-up of infants with BPD.

Proporção de Largura de Distribuição de Hemoglobina/Glóbulos Vermelhos (HRR): Um Novo e Promissor Parâmetro de Glóbulos Vermelhos no Fechamento Ductal / Hemoglobin/Red Cell Distribution width Ratio (HRR): A Novel and Promising Red Cell Parameter in Ductal Closure

Resumo Fundamento Ainda não está clara a eficácia dos parâmetros hematológicos no fechamento da persistência do canal arterial (PCA). Objetivos O objetivo principal do nosso estudo é investigar o efeito da proporção (HRR) de largura de distribuição de hemoglobina (HB) para glóbulos vermelhos (RDW) no fechamento do PCA. Métodos Bebês prematuros com muito baixo peso ao nascer (MBPN: <1.500 g) e <32 semanas gestacionais foram incluídos no estudo, e todos os dados foram registrados retrospectivamente. Características demográficas, resultados clínicos, parâmetros de hemácias e HRR e suas proporções foram comparados entre grupos de PCA hemodinamicamente significativa (hsPDA) e não-hsPDA. Todos os resultados foram analisados estatisticamente, e p<0,05 foi considerado estatisticamente significativo. Resultados Um total de 677 bebês prematuros, 269 no grupo hsPDA e 408 no grupo não-hsPDA, foram incluídos no estudo. Hemoglobina (HB), hematócrito (HCT), volume celular médio (VCM), glóbulos vermelhos (RBC), largura de distribuição dos glóbulos vermelhos (RDW), volume plaquetário médio (VPM), relação VCM/RBC, relação HB/RBC, RDW A razão /RBC e a razão RDW/VPM foram semelhantes entre os grupos hsPDA e não hsPDA, (p>0,05). HRR foi significativamente menor no grupo hsPDA [mediana (Quartil 1 (Q1) - Q3) (Q1 - Q3): 0,93 (0,8-1,0)] em comparação com não-hsPDA [mediana (Q1 - Q3): 1,07 ( 1,0-1,2)] (p<0,001). A AUC para o valor diagnóstico de HRR em hsPDA foi de 0,816 e o valor de corte foi ≤0,98 (p<0,001, 95% [IC]: 0,785-0,845, sensibilidade: 90%, especificidade: 92%). Conclusões O valor de HRR foi considerado um parâmetro eficaz e poderoso no diagnóstico de hsPDA.

Abstract Background It is still unclear how effective hematological parameters are in the closure of patent ductus arteriosus (PDA). Objectives The primary aim of our study is to investigate the effect of hemoglobin (HB)-to-red cell distribution width (RDW) ratio (HRR) on the closure of PDA. Methods Premature babies with very low birth weight (VLBW: <1500 g) and <32 gestational weeks were included in the study, and all data were recorded retrospectively. Demographic characteristics, clinical results, red cell parameters, and HRR and their ratios were compared between hemodynamically significant PDA (hsPDA) and non-hsPDA groups. All results were statically analyzed, and P<0.05 was considered statistically significant. Results A total of 677 premature babies, 269 in the hsPDA group and 408 in the non-hsPDA group, were included in the study. Hemoglobin (HB), hematocrit (HCT), mean cell volume (MCV), red blood cell (RBC), red cell distribution width (RDW), mean platelet volume (MPV), MCV/RBC ratio, HB/RBC ratio, RDW/RBC ratio, and RDW/MPV ratio were found to be similar between hsPDA and non-hsPDA groups, (p>0.05). HRR was found to be significantly lower in the hsPDA group [median (Quartile 1 (Q1) - Q3) (Q1 - Q3): 0.93 (0.8-1.0)] compared to non-hsPDA [median ( Q1 - Q3): 1.07 (1.0-1.2)] (p<0.001). The AUC for the diagnostic value of HRR in hsPDA was 0.816, and the cutoff value was ≤0.98 (p<0.001, 95% [CI]: 0.785-0.845, sensitivity: 90%, specificity: 92%). Conclusions HRR value was found to be both an effective and powerful parameter in diagnosing hsPDA.

Intravenous Immunoglobulin Use in Hemolytic Disease Due to ABO Incompatibility to Prevent Exchange Transfusion.

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion. Materials and Methods: This study evaluated the data of infants with ABO HDN in the Turkish Neonatal Jaundice Online Registry. The infants with ABO HDN who met the total serum bilirubin level inclusion criteria (within 2-3 mg/dL of exchange transfusion or even above exchange transfusion level) were included in the study according to the guidelines from the American Academy of Pediatrics and the Turkish Neonatal Society. All patients were managed according to the unit protocols recommended by these guidelines and received light-emitting diode (LED) phototherapy. Infants who only received LED phototherapy, and who received one dose of IVIG with LED phototherapy were compared. Results: During the study period, 531 term infants were included in the study according to inclusion criteria. There were 408 cases in the phototherapy-only group, and 123 cases in the IVIG group. The demographic findings and the mean bilirubin and reticulocyte levels at admission were similar between the groups (p > 0.05), whereas the mean hemoglobin level was slightly lower in the IVIG group (p = 0.037). The mean age at admission was earlier, the need for exchange transfusion was higher, and the duration of phototherapy was longer in the IVIG group (p < 0.001, p = 0.001, and p < 0.001, respectively). The rate of re-hospitalization and acute bilirubin encephalopathy (ABE) was higher in the IVIG group (p < 0.001 and p = 0.01, respectively). Conclusion: In this study, we determined that one dose of IVIG did not prevent an exchange transfusion nor decrease the duration of phototherapy in infants, who had bilirubin levels near or at exchange transfusion level, with hemolytic disease due to ABO incompatibility.

Clinical, biochemical, and echocardiographic evaluation of neonates with vitamin D deficiency due to maternal vitamin D deficiency.

OBJECTIVE: There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency. PATIENTS AND METHODS: In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Following remission of biochemical features of vitamin D deficiency, control echocardiography was performed. Biochemical and echocardiographic characteristics of the present cohort were compared with those of 27 previously published cases with dilated cardiomyopathy due to vitamin D deficiency. RESULTS: The study included 148 cases (95 males). In the echocardiographic evaluation, none of the patients had dilated cardiomyopathy. All of the mothers were also vitamin D deficient and treated accordingly. Comparison of patients with normocalcaemia and hypocalcaemia at presentation revealed no statistically significant difference between the ejection fraction and shortening fraction, while left ventricle end-diastolic diameter and left ventricle end-systolic diameter were higher in patients with hypocalcaemia. Previously published historical cases were older and had more severe biochemical features of vitamin D deficiency. CONCLUSION: To the best of our knowledge, in this first and largest cohort of neonates with vitamin D deficiency, we did not detect dilated cardiomyopathy. Early recognition and detection before developing actual rickets and preventing prolonged hypocalcaemia are critically important to alleviate cardiac complications.

Effect of diazepam, sertraline and melatonin on the stress-induced reproductive disorders and intrauterine growth restriction in female rats.

OBJECTIVE: Psychological stress is an important factor triggering depression and anxiety. Infertility is known to cause stress; however, it is not clearly known whether stress causes infertility as well. In addition, there are different opinions accounting for the relation of stress-induced oxidative stress to infertility and intrauterine growth restriction. The aim of the study is to examine the effect of sertraline, diazepam and melatonin on the infertility, intrauterine growth restriction and oxidative stress that can be caused by forced immobilization stress management (FISM) in female rats. MATERIALS AND METHODS: Wistar rats were grouped as healthy rats (HG) applied distilled water, stress treated control group (SC), and 20 mg/kg sertraline + stress (SS), 2 mg/kg diazepam + stress (DS) and 10 mg/kg melatonin + stress (MS) treated rats. The medicines were administered orally once a day for 30 days. At the end of this period, oxidant/antioxidant parameters were measured through the blood samples collected from the tail veins of all rats. Then the rats were kept in a suitable environment for 2 months for breeding. RESULTS: FISM caused oxidative stress in blood serum of animals, infertility and intrauterine growth restriction (decrease in birth weight of the baby). Best medicines to suppress FISM-related oxidative stress are melatonin > diazepam > sertraline respectively, while sertraline > diazepam > melatonin were most successful in terms of preventing infertility. The best medicines preventing the FISM-caused intrauterine growth restriction were found to be melatonin > diazepam > sertraline, respectively. CONCLUSION: FISM causes oxidative stress in animals. Oxidative stress is understood to affect the intrauterine growth negatively although it is not a major component in the pathogenesis of infertility. While melatonin is only effective in preventing the oxidative stress-related intrauterine growth restriction, antidepressants and anxiolytic treatment were found to be helpful in preventing both infertility and intrauterine growth restriction.

Paraurethral cyst in a newborn.

Paraurethral cysts are rare and occur with obstruction of the Skene duct. In this case, we aimed to present a paraurethral cyst in a baby girl. A 4-day-old newborn was consulted for pediatric urology because of an interlabial mass. In genital examination, a noninvasive mass measuring 2 × 1.5 cm was observed in the interlabial region, and the introitus was completely closed. Ultrasonography showed a cystic lesion localized on the right side of the urethra with a smoothly contoured, thin-walled, hypoechoic structure. The patient underwent puncture under sterile conditions. The content of the mass was mucoid and clear fluid. Paraurethral cysts are often asymptomatic and benign. There is no definitive consensus on treatment.

A Comparative Trial of the Effectiveness of Nasal Interfaces Used to Deliver Continuous Positive Airway Pressure for a Brief Period in Infants With Transient Tachypnea of the Newborn / Estudio comparativo sobre la eficacia de las interfaces nasales para la administración de presión positiva continua de la vía aérea durante un periodo breve de tiempo en neonatos con taquipnea transitoria del recién nacido

INTRODUCTION: There is insufficient data on the effectiveness of the interfaces used for nasal continuous airway pressure (nCPAP) in newborn infants. Transpulmonary pressure (PTP) calculated from a measured esophageal pressure (Pes) could be used as a surrogate for the pressure transmitted to the distal airways during nCPAP. We aimed to compare the effectiveness of two nasal interfaces, the nasal mask and bi-nasal short prongs, during a relatively brief period of respiratory support by calculated PTP (cPTP) in infants with transient tachypnea of the newborn (TTN). METHODS: Newborns with TTN who needed respiratory assistance with nCPAP were randomized to use either bi-nasal short prongs or a nasal mask. Esophageal pressure measurements were done in order to calculate PTP with either interface. The primary outcome was the cPTP transmitted with each nasal interface. Esophageal pressure measurements were recorded and PTP values were calculated from Pes measurements at the 1st, 6th, 12th and 24th hours in each patient as long as the respiratory support lasted. RESULTS: Sixty-two newborns with TTN and on nCPAP were randomized into two groups: Group 1 to use bi-nasal short prongs (n: 31) and Group 2 to use a nasal mask (n: 31). Inspiratory and expiratory Pes and cPTP values at the 1st, 6 th, 12 th and 24 th hours were similar with the two interfaces (P < .05). CONCLUSIONS: A nasal mask is similarly effective and safe as bi-nasal short prongs during a brief period of non-invasive respiratory support with nCPAP in late preterm and term neonates with TTN

INTRODUCCIÓN: No hay datos suficientes sobre la eficacia de las interfaces que se utilizan para la administración de presión positiva continua de la vía aérea por vía nasal (CPAPn) en neonatos. La presión transpulmonar (PL), calculada a partir de la medición de la presión esofágica (Pesof), podría utilizarse como alternativa para medir la presión transmitida a la vía aérea distal durante la CPAPn. Nuestro objetivo fue comparar la eficacia de 2 interfaces nasales, la mascarilla nasal y las cánulas binasales cortas, durante un periodo relativamente corto de soporte respiratorio mediante la PL calculada (PLc) en neonatos con taquipnea transitoria del recién nacido (TTRN). MÉTODOS: Los neonatos con TTRN que requirieron ventilación con CPAPn se aleatorizaron para el uso de cánulas binasales cortas o mascarilla nasal. Se realizaron mediciones de la presión esofágica para calcular la PL con cada interfaz. La variable de resultado fue la PLc transmitida con cada interfaz nasal. Las mediciones de presión esofágica se registraron y los valores de PL se calcularon a partir de las mediciones de la Pesof en las 1.ª, 6.a, 12.a y 24.a horas en cada paciente durante el tiempo que durara la ventilación mecánica. RESULTADOS: Se aleatorizaron 62 neonatos con TTRN y tratados con CPAPn en 2 grupos: el grupo 1 usó las cánulas binasales cortas (n = 31) y el grupo 2 usó la mascarilla nasal (n = 31). Los valores inspiratorios y espiratorios de Pesof y PLc en las 1.a, 6.a, 12.a y 24.a horas fueron similares con ambas interfaces (P < 0,05). CONCLUSIONES: La máscara nasal tiene una eficiencia similar a las cánulas binasales cortas durante la administración breve de ventilación mecánica no invasiva mediante CPAPn en neonatos prematuros tardíos y neonatos a término con TTRN

A Comparative Trial of the Effectiveness of Nasal Interfaces Used to Deliver Continuous Positive Airway Pressure for a Brief Period in Infants With Transient Tachypnea of the Newborn.

INTRODUCTION: There is insufficient data on the effectiveness of the interfaces used for nasal continuous airway pressure (nCPAP) in newborn infants. Transpulmonary pressure (PTP) calculated from a measured esophageal pressure (Pes) could be used as a surrogate for the pressure transmitted to the distal airways during nCPAP. We aimed to compare the effectiveness of two nasal interfaces, the nasal mask and bi-nasal short prongs, during a relatively brief period of respiratory support by calculated PTP (cPTP) in infants with transient tachypnea of the newborn (TTN). METHODS: Newborns with TTN who needed respiratory assistance with nCPAP were randomized to use either bi-nasal short prongs or a nasal mask. Esophageal pressure measurements were done in order to calculate PTP with either interface. The primary outcome was the cPTP transmitted with each nasal interface. Esophageal pressure measurements were recorded and PTP values were calculated from Pes measurements at the 1st, 6th, 12th and 24th hours in each patient as long as the respiratory support lasted. RESULTS: Sixty-two newborns with TTN and on nCPAP were randomized into two groups: Group 1 to use bi-nasal short prongs (n: 31) and Group 2 to use a nasal mask (n: 31). Inspiratory and expiratory Pes and cPTP values at the 1st, 6th, 12th and 24th hours were similar with the two interfaces (P<.05). CONCLUSIONS: A nasal mask is similarly effective and safe as bi-nasal short prongs during a brief period of non-invasive respiratory support with nCPAP in late preterm and term neonates with TTN.

Systemic Pseudohypoaldosteronism Type 1 due to 3 Novel Mutations in SCNN1Aand SCNN1BGenes.

OBJECTIVE: The systemic form of pseudohypoaldosteronism type 1 (PHA1) is an autosomal recessive disorder characterized by defective sodium transport in multi-organ systems. Mutations in the genes encoding the amiloride-sensitive epithelial sodium channel, ENaC, account for genetic causes of systemic PHA1. We describe systemic PHA1 due to 4 novel variants detected in SCNN1A and SCNN1B in 3 cases from 3 unrelated consanguineous families. PATIENTS AND METHODS: We evaluated the clinical presentations, biochemical and hormonal characteristics, and molecular genetic analysis results of 3 patients from 3 unrelated consanguineous families and parents from whom samples were available. RESULTS: The ages at presentation were postnatal days 9, 10, and 5. The main presentation symptoms were vomiting, poor feeding, weakness, weight loss, and skin rash. All patients exhibited laboratory characteristics including severe hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma renin, elevated aldosterone, and positive sweat tests, suggesting a diagnosis of systemic PHA1. Molecular genetic analysis revealed 2 novel pathogenic variants [c.87C>A(p.Tyr29*)/IVS9 + 1G>A (c.1346 + 1G>A)] in SCNN1Bin case 1, a novel homozygous pathogenic variant [p.His69Arg(c.206A>G] in SCNN1Ain case 2, and a homozygous one-base duplication, p.A200Gfs*6 (c.598dupG), in SCNN1A in case 3. CONCLUSION: PHA1 should be considered at differential diagnosis in patients presenting with hyponatremia, hyperkalemia, and metabolic acidosis. The cases in this report involving 4 novel variants will add valuable insights into the phenotype-genotype relationship and will expand the mutation database.

Placenta, Secret Witness of Infant Morbidities: The Relationship Between Placental Histology and Outcome of the Premature Infant.

OBJECTIVE: The microscopic and macroscopic features of the placenta can contribute to the clinical understanding of premature delivery. The aim of our study was to figure out the relationship between the histopathological findings of the placentas of premature deliveries and its effects on neonatal morbidity and mortality. MATERIAL AND METHOD: The placentas of 284 singleton preterm infants with < 35 weeks of gestation were examined. Three groups were created as the normal, chorioamnionitis and vasculopathy groups according to the histopathological findings in the placentas of the subjects. RESULTS: The mean gestational age of the infants in the study group was 30.5 ± 3.2 weeks, and the mean birth weight was 1588 ± 581 g. The pathology was normal in ninety-six (33.8%), vasculopathy in 153 (53.9%) and chorioamnionitis in 35 (12.3%). The gestation age of the infants was lower in the chorioamnionitis group. Moreover, retinopathy of prematurity, early onset neonatal sepsis, and duration of respiratory support were found to be higher in the chorioamnionitis group. In the vasculopathy group, preeclampsia and small for gestational age were found to be significantly higher. CONCLUSION: Histopathological findings of the placentas from preterm deliveries provided important data in determining the etiology of preterm delivery and outcomes of infants. Infants delivered by mothers with chorioamnionitis were particularly found to be more preterm, and these preterm infants would have a longer hospital stay, higher respiratory support requirement, and more serious morbidities.

Health care-associated infection surveillance: A prospective study of a tertiary neonatal intensive care unit.

INTRODUCTION: Health care-associated infection (HCAI) is a serious problem of neonatal intensive care units (NICUs) which is related to morbidity, mortality and increased cost of medical care. This study aimed to determine the incidence of HCAI in a tertiary NICU and identify the risk factors. METHODOLOGY: This prospective cohort study was conducted between July 1, 2011 and June 30, 2012. All newborns admitted to the NICU except for those who died or were discharged within 48 hours after admission were included. The definitions of Centers for Disease Control and Prevention (CDC) were used to diagnose specific types of infections. The incidence, causative organisms, risk factors and mortality of HCAIs were evaluated. RESULTS: Among 352 newborns, a total of 60 HCAI episodes were evaluated in 37 (10.5%) of the patients over 5,212 patient-days. The overall incidence of HCAI was 17%, and the rate was 11.5/1,000 patient-days. Blood stream infection (BSI) was the most common HCAI (n = 42, 70%). In a multivariable logistic regression analysis, the presence of a central venous catheter/umbilical catheter (CVC/UC), the presence of a urinary catheter, and gestational age (< 32 weeks of gestation) were identified as significant independent risk factors. Gram-negative pathogens were the most common isolates. The overall mortality rate was 4%. The HCAI-related mortality rate was 10.8%. CONCLUSIONS: Patient care quality can be improved with surveillance of HCAI. The incidence and rate of HCAI in our NICU were found to be higher than international reports with a direct impact on mortality of preterm infants.

Influence of platelet count, platelet mass index, and platelet function on the spontaneous closure of ductus arteriosus in the prematurity.

BACKGROUND: This study aims at evaluating the influence of platelet count, platelet mass index, and platelet function on the spontaneous closure of ductus arteriosus in prematurity. METHODS: All preterm babies were divided into two groups, including Group 1 with "open PDA" and Group 2 with "closed PDA". The variables of platelet count, mean platelet volume, platelet mass index, and platelet function were analyzed and compared between two groups of patients to identify the factors that significantly influenced spontaneous closure of ductus arteriosus. RESULTS: Twenty-four patients were in the "open PDA" group, whereas 36 patients were in the "closed PDA" group. Mean GA and BW were 27.6 ± 1.8 (23.1-30.4) and 28 ± 1.6 (23.4-30.6) weeks and 1009 ± 270 (585-1480) g and 1035 ± 298 (505-1500) g in "open PDA" and "closed PDA" groups, respectively (p > 0.05). The incidence of "Collagen-ADP > 130 s" was significantly higher in the "open PDA" group, and the levels of hemoglobin and hematocrit were significantly lower in the "open PDA" group (p < 0.05). Multivariate logistic regression analysis showed that respiratory distress syndrome (OR: 9, CI: 1.5-51.8) and collagen-ADP > 130 s (OR: 5.7 CI: 1.55-21.3) are two independent factors associated with ductal patency. CONCLUSION: This is the first study in the English literature providing evidence of the influence of platelet dysfunction on the spontaneous closure of ductus arteriosus in prematurity. Longer collagen-ADP duration is identified as a risk factor of ductal closure.

Transient tachypnea of the newborn: are there bedside clues for predicting the need of ventilation support?

Decision making to transfer a late preterm or term neonate with the diagnosis of transient tachypnea of the newborn (TTN) to an intensive care unit for respiratory support is a challenge for caregivers in level one and two NICUs. The aim of this study was to identify "practical bedside clinical clues" that may help to predict the severity of disease and need for respiratory support in patients with the diagnosis of TTN. Newborns having the diagnosis of TTN were classified into two groups according to the intensity of the respiratory support. Infants receiving only supplemental oxygen and infants requiring nasal continuous positive airway pressure or mechanical ventilation constituted group 1 (mild) and group 2 (severe), respectively. Demographic, clinical and laboratory characteristics were compared between the two groups. Patients in group 2 had lower gestational age, higher Silverman and Richardson scores, longer mean duration of oxygen support and hospitalization. A positive correlation was found between subcostal and xiphoid retractions, asynchrony in chest-abdomen movements, arterial pH < 7.30, ratio of PaO < sub > 2 < /sub > / % inspired O < sub > 2 < /sub > < 1.2 and need of respiratory support (p < 0.05). We suggest that simple scores can help physicians to get a good sense of a given baby's likelihood of deterioration.

Impact of Prophylactic Continuous Positive Airway Pressure on Transient Tachypnea of the Newborn and Neonatal Intensive Care Admission in Newborns Delivered by Elective Cesarean Section.

OBJECTIVE: This study aims to evaluate the effect of the prophylactic continuous positive airway pressure (CPAP) administration in the delivery room to newborns who were delivered by elective cesarean section (CS). STUDY DESIGN: Inborn infants with gestational age between 34(0/7) to 38(6/7) and born by elective CS were prospectively randomized to receive either prophylactic CPAP for 20 minutes via face mask or standardized care without CPAP in the delivery room. Primary outcomes were the incidence of transient tachypnea of the newborn (TTN) and neonatal intensive care unit (NICU) admission due to respiratory distress. RESULTS: A total of 259 infants with a mean gestational age of 37.7 ± 0.8 weeks and birth weight of 3,244 ± 477 g were included. A total of 134 infants received prophylactic CPAP and 125 received control standard care. The rate of NICU admission was significantly lower in prophylactic CPAP group (p = 0.045). Although the rate of TTN was lower in the prophylactic CPAP group, the difference was not statistically significant (p = 0.059). The rate of NICU admission due to respiratory distress was significantly higher in late-preterm cohort than early-term cohort (p < 0.0001). CONCLUSION: Prophylactic CPAP administration decreases the rate of NICU admission without any side effect in late-preterm and early-term infants delivered by elective CS.

Report of a case of neonatal chylothorax that responded to long-term octreotide treatment, and review of the literature.

Chylothorax is a relatively uncommon condition defined as an abnormal collection of lymphatic fluid within the pleural space. Morbidity of congenital chylothorax (CC) is high, and prognosis is very poor if CC is associated with hydrops fetalis. Although the optimal treatment of CC has not been determined, conservative treatment and surgical intervention are employed. However, there is still little experience with the use of octreotide therapy for this condition, and optimal duration of the treatment for response evaluation is not known. We report a newborn with CC who presented with intrauterine bilateral pleural effusion and was resistant to conservative treatments. Octreotide (6 µg/kg/h) infusion was started on the 10th postnatal day due to ongoing pleural drainage. Although the patient improved rapidly with continuous administration of octreotide, we had to continue the drug for 151 days, even subcutaneously on outpatient follow-up. To the best of our knowledge, this patient is unique in receiving octreotide treatment for such a long time, with a successful outcome and a safe profile.