Lung ultrasound scores within the first 3 days of life to predict respiratory outcomes.

BACKROUND: Lung ultrasound (LUS) is a rapid and simple method to evaluate preterm babies with respiratory distress. Lately, LUS has also been reported as an accurate predictor for bronchopulmonary dysplasia (BPD). OBJECTIVES: The aim of the study was to investigate the relationship between the LUS scores within the first 3 days of life and respiratory outcomes including the need and the duration of invasive mechanical ventilation, and development of BPD. METHODS: It was a retrospective observational study. Preterm infants younger than 32 weeks were included at an academic tertiary Neonatal Intensive Care Unit between 2018 and 2023. LUS was performed within the first 3 days. Each lung was divided into three regions and defined as a score of 0 to 3 points; the total score was obtained by adding the six regional scores. LUS scores were noted in two groups as the highest and lowest scores. Statistical analyses were done to predict respiratory outcomes. RESULTS: Total 218 patients were enrolled; 40, 17, and 18 infants had mild, moderate, and severe BPD, respectively. BPD did not develop in 143 patients. Within the first 3 days, the highest and lowest LUS scores significantly predicted moderate-to-severe BPD (p < .001) (area under receiver operating characteristic [ROC] curve, 0.684-0.913; area under ROC curve 0.647-0.902; respectively). High LUS scores were also related with the need of mechanical ventilation (p < .001). There was not a significant correlation between the duration of mechanical ventilation and the LUS scores. Regression analysis revealed that the highest LUS scores within the first 3 days of life, sepsis, and the presence of hemodynamically significant patent ductus arteriosus (hsPDA) were significantly associated with the severity of BPD. CONCLUSIONS: In preterm babies, the LUS scores were useful to predict BPD and the need of invasive ventilation in long term. However, it was not related with the length of invasive ventilation.

Glomerulonephritis with crescents in childhood; etiologies and significance of M2 macrophages.

BACKGROUND: Crescentic glomerulonephritis (CGN) is a rapidly progressive and rare cause of glomerulonephritis in childhood. The aim of this study is to evaluate demographic data of children with crescentic glomerulonephritis, to classify the etiologies and to investigate the correlation between the severity of kidney disease and the expression of CD163+ macrophages. METHODS: Between the years 2000 and 2016 in a single center, patients under 18 years of age with kidney biopsies containing crescents were included in the study. A total of 88 children were enrolled. The expression of CD163 in kidney tissues was detected by immunohistochemistry in 61 patients. Clinical features and outcome were collected from their medical records. RESULTS: The most common etiology was Henoch-Schönlein purpura (HSP) nephritis/Immunglobulin A vasculitis (26.1%), followed by lupus nephritis (22.7%) and idiopathic crescentic glomerulonephritis (18.2%). CD163 positive cell counts in patients with GFR levels less and more than 60 ml/min/1.73 m2 at their last visit were 7.6±6.6 cells vs. 2.0±3.0 cells (p=0.057) per one glomerulus and 52.2±18.2 cells/hpf vs. 33.3±10.0 cells/hpf (p < 0.05) in tubulointerstitium, respectively. Tubulointerstitital CD163+ cells were also found to be higher in patients with end stage kidney disease than complete and partial responders (68 cells/hpf vs 39 cells/hpf, p < 0.05). CONCLUSIONS: CD163 positive cell counts, particularly in tubulointerstitial areas, have been associated with poor prognosis of CGN.

A very rare case of a newborn with tetrasomy 9p and literature review.

BACKGROUND: Tetrasomy 9p is a rare genetic condition which usually results from a supernumerary isochromosome derived from the short arm of chromosome 9. Phenotypic findings include multiple congenital anomalies, facial dysmorphism, growth and developmental delays, and also vary according to the presence and degree of mosaicism. CASE: We report on a newborn with tetrasomy 9p who deceased in the newborn period. She had facial features including low-set and anteverted ears, hypertelorism, prominent nasal bridge, and microretrognathia. Bilateral ventriculomegaly, vermian hypoplasia and corpus callosum agenesis were detected on magnetic resonance imaging and double outlet right ventricle (tetralogy of Fallot type), secundum atrial septal defect, and persistent left superior vena cava were displayed by echocardiography. Microarray analysis revealed 38,584 kb tetrasomic region at 9p24.3p13.1. We also present a review of the literature suggesting that there is a recognizable phenotype for this condition and an assessment of cardiac manifestations based on the size and the localization of the breakpoints. CONCLUSIONS: We conclude that cardiac manifestations do not differ according to the localization of the breakpoint. Persistent left superior vena cava seems to be consistent with breakpoints distal to q12, but the present case is different from them by breakpoint p13.1.

Lung ultrasonography decreases radiation exposure in newborns with respiratory distress: a retrospective cohort study.

Chest X-ray (CXR) is commonly used as a first-line imaging method to determine the cause of respiratory distress in NICUs. The aim of the study was to retrospectively assess the decrease in the number of CXRs performed due to the use of lung ultrasonography on the first day of life for newborns with respiratory distress. Infants who were admitted to the NICU on the first day of life due to respiratory distress were enrolled in this study (ClinicalTrials.gov identifier NCT04722016) and divided into two groups: the study group (n = 104) included patients born between January 2019 and June 2020, and the historical control group (n = 73) included patients born between June 2017 and December 2018. As a first-line technique for lung imaging, only CXR had been used in the historical control group, whereas ultrasound had been preferred in the study group. The radiation dose to the newborns and the number of CXRs performed in the first day of life were compared between the two groups. Significant reductions in the number of CXRs performed and radiation exposure were observed in the study group. The radiation dose decreased from 5.54 to 4.47 µGy per baby when LUS was routinely used. The proportion of patients who underwent CXR decreased from 100 to 71.2%.Conclusion: We observed that using lung ultrasonography as a first-line evaluation method in neonates with respiratory distress decreased both the number of CXRs performed and radiation exposure. What is Known: • Chest X-ray is commonly used as a first line imaging method to diagnose the reason of respiratory distress in NICUs. • Lung ultrasound is a new diagnostic tool for lung imaging. What is New: • With the use of lung ultrasonography, radiation exposure of both newborns and healthcare workers can be reduced. • This retrospective study revealed that most of the babies with respiratory distress were treated without CXR.

Impact of Prematurity on the Buccal Epithelial Cells of the Neonates via Wnt/Beta-Catenin Signaling Pathway and Apoptosis.

OBJECTIVE: Understanding the reflections of prematurity is necessary for the management of neonatal complications. We focused on the impact of prematurity and related "maternal risk factors/obstetric complications" on buccal cells of the neonates via evaluation of the Wnt/ß-catenin signaling pathway and apoptosis. STUDY DESIGN: This study consisted of "early preterm neonates (EPN) (≤34th gestational week [gw]) (n = 36)," "late preterm neonates (LPN) (34th- < 37th gw) (n = 46)," and "term neonates (control) (≥37th gw) (n = 56)." Cohort was also subclassified according to the presence of maternal risk factors, obstetric complications, and neonatal complications. Wnt/ß-catenin signaling and caspase-3 activation pathways were studied immunocytochemically. RESULTS: Wnt/ß-catenin signaling positivity was statistically more frequent at buccal smears of the EPN and LPN groups compared with controls (p < 0.001). The cutoff for gestational age at delivery in receiver operating characteristic curve with the best balance of sensitivity (67.4%) and specificity (67.3%) was 35.8th gw for determining the reduction of Wnt/ß-catenin signaling positivity (p < 0.001). The study demonstrated that obstetric complications significantly affected the activity of signaling, while maternal risk factors do not have any effect on Wnt/ß-catenin signaling pathway (p = 0.003 and p = 0.828, respectively). This study also demonstrated a significant relationship between Wnt/ß-catenin signaling pathway and the presence of neonatal complications (p = 0.015). CONCLUSION: Dynamic characteristics of buccal cells are influenced by prematurity and related obstetric and neonatal problems. Buccal smear is a good tool to investigate the impact of prematurity and obstetric problems on perinatal outcome. KEY POINTS: · Neonatal buccal cells are affected by prematurity and related obstetric/neonatal problems.. · 35.8th gw is critical for determining the reduction of Wnt/ß-catenin signaling positivity.. · Obstetric and neonatal complications significantly related to Wnt/ß-catenin signaling activity..

Impact of preterm birth on the cellular characteristics of neonatal buccal cells.

OBJECTIVE: To demonstrate the impact of preterm birth on the cytological, cytomorphometrical, and nuclear parameters of neonatal buccal smears. METHODS: This study consisted of Early Preterm Neonates (EPN; ≤34th gestational week [gw]; n = 36), Late Preterm Neonates (LPN; 34th to <37th gw; n = 46), and Term Neonates (control; ≥37th gw; n = 56). Cytological evaluation and buccal cytome assay were performed using Papanicolaou and Feulgen methods, respectively. RESULTS: Cytological evaluation demonstrated that smear background was cleaner (P < .05) and there were less macrophages in the control group (P < .001). Cyto-morphometric analysis showed that the measurements of nuclear diameter, nuclear area, and nucleus-to-cytoplasm ratio were higher in the preterm (EPN and LPN) versus the control groups (P = .016, P < .001, and P < .001, respectively). We also demonstrated that staining intensity of the nucleus and cytoplasm were less intense in the EPN and LPN groups (P < .001). There was no statistically significant difference between the EPN and LPN groups for any parameters (P > .05). Buccal cytome assay showed that nuclear buds were more prevalent in term newborns compared to preterm neonates (P < .001). CONCLUSIONS: Morphological and cytological properties of neonatal buccal cells are influenced by preterm birth status, and buccal smears may be used as a tool to detect biological markers of neonatal health problems.

Lung ultrasound (LUS) and surfactant treatment: looking for the best predictive moment.

OBJECTIVE: Assess the earliest time of LUS to guide surfactant therapy. STUDY DESIGN: In this observational study (ClinicalTrials.gov Identifier NCT04544514), LUS was performed within 30 min and repeated at 1, 2, 4, and 6 h on preterm babies. White lung appearance was defined as type 1 group, whereas prevalence of lines B as type 2 and lines A as type 3. Ultrasound and radiographic findings were also compared to determine surfactant need. RESULTS: Among 71 patients, 41 received surfactant therapy. In the first evaluation, 37 of them have been defined as type 1, whereas 4 of them have been as type 2 group. Type 3 group did not receive surfactant. Type 1 findings were superior to predict surfactant need and the predictive value was 100% at 2 h. CONCLUSION: Even early LUS assessment at the first 20-30 min  was more significant to predict surfactant need than x-ray. Presence of white lung appearance for 2 h indicates an absolute surfactant need.

Transplacental hemophilia A and prophylactic treatment with intravenous immunoglobulin and recombinant factor VIIa in the newborn period: a case report.

Acquired hemophilia due to inhibitor antibodies to factor VIII (FVIII) is a very rare entity in neonatal period. Maternal IgG antibodies may cross the placenta and can cause life-threatening hemorrhages in newborns. Here, we represent a newborn who diagnosed as a transplacental acquired hemophilia A. A very high titer of inhibitor level (320 Nijmegen-Bethesda unit) was detected in plasma due to transplasental transfer in this case. According to the best of our knowledge the baby had the highest inhibitor level in neonatal period in the literature. Bleeding complications including intracranial hemorrhage secondary to this condition were reported before. Therefore, to prevent possible life complications, prophylactic recombinant FVIIa was administered in the presenting case and any bleeding event was not observed during follow-up. In conclusion, using prophylactic recombinant FVIIa in newborns is a safe choice for transplacental acquired hemophilia A.

Antenatal management and outcomes of pregnancies with congenital diaphragmatic hernia.

BACKGROUND: The objective of this study is to evaluate the obstetric outcomes of pregnancies with congenital diaphragmatic hernia (CDH). METHODS: Fifty one pregnancies prenatally diagnosed with CDH at our institution between January 1, 2002 and August 31, 2018 were retrospectively evaluated. The pregnancies were divided into two groups according to neonatal survival. Demographic features, clinical characteristics and prognostic factors were compared between the neonatal survival (n = 16) and non-survival (n = 28) groups. Cut-off values of fetal lung area to head circumference ratio (LHR), observed/expected LHR (o/e LHR) and observed/expected total fetal lung volume (o/e TFLV) for neonatal survival were calculated. RESULTS: Thirty six (70.6%) and fifteen (29.4%) fetuses had left and right sided CDH respectively. Seven patients chose termination of their pregnancies (13.7%). Statistically significant differences were found between survival and non-survival groups in terms of parity, median gestational week at diagnosis, polyhydroamniosis rate, CDH type, stomach position, liver position, median LHR, o/e LHR, o/e TFLV, median 5th minute Apgar score and neonatal operation rate values (p values were 0.03,<0.001, 0.02, 0.006,<0.001, 0.006,<0.001,<0.001,<0.001, 0.04 and <0.001 respectively). According to ROC curve analysis, 1.05 (82% sensitivity, 74% specificity) for LHR, 22.5 (78.6% sensitivity, 73.9% specifity) for o/e LHR and 23.5 (85.7% sensitivity,74.2% specificity) for o/e TFLV were determined to be cut-offs for neonatal survival, respectively, with highest sensitivity and specificity. CONCLUSION: Earlier gestational week at diagnosis, right sided CDH, presence of liver herniation, supradiaphragmatic stomach position, lower LHR, o/e LHR and o/e TFLV were associated with decreased rates of neonatal survival.

Non-immune hydrops fetalis: A retrospective analysis of 151 autopsies performed at a single center.

Kayki G, Güçer S, Akçören Z, Orhan D, Talim B, Yurdakök M, Yigit S, Boduroglu OK, Utine GE, Örgül G, Beksac MS. Non-immune hydrops fetalis: A retrospective analysis of 151 autopsies performed at a single center. Turk J Pediatr 2018; 60: 471-477. We retrospectively evaluated autopsies performed on 151 non-immune hydrops fetalis (NIHF) cases to determine the etiology and pathological findings. Further, cases identified between 1980 and 2004 were compared with those identified between 2005 and 2015 to investigate the improvement of diagnostic performance of our institution. The mean gestational age during the fetal autopsy was 25 weeks. There were 30 live-born infants in the study group. The etiology of NIHF could be determined in 91 cases (60.3%), while it remained undefined in remaining 60 cases. The most commonly associated pathological conditions were cardiovascular malformations (11.3%), followed by chromosomal abnormalities (9.3%). Prior to 20th gestation week, genetic anomalies and cystic hygromas were the most common etiological factors, and after 30 weeks of gestation, cardiac abnormalities were found to be the most common causes. With time, the rate of undefined cases decreased from 48.4% to 33.75%. NIHF is a complex medical condition necessitating a multidisciplinary management approach. Progress in molecular genetics and imaging techniques is expected to improve diagnostic performance for rapid and better identification.

Normoblastemia multinuclear en un recién nacido secundaria a hipoxia / Multinucleated normoblastemia in a newborn secondary to hypoxia

Es posible detectar normoblastos en los frotis de sangre periférica de los recién nacidos. En general, la cantidad de normoblastos por cada 100 leucocitos está en el intervalo de 0 a 10. Se observan con más frecuencia de lo usual ante una situación de hipoxia porque la hipoxia intrauterina aumenta la producción de eritrocitos. Sin embargo, no se había informado antes un caso de normoblastos multinucleados en un recién nacido a causa de la hipoxia. Presentamos el caso de un recién nacido con normoblastos multinucleados secundarios a hipoxia intrauterina. Este caso es importante porque es la primera vez que se han detectado normoblastos multinucleados en el frotis de sangre periférica de un recién nacido hipóxico.

Normoblasts may be seen in peripheral blood smear of newborns. The number of normoblasts per 100 white blood cells is generally in the range of 0-10.They can be seen more common than usual in hypoxic condition, because intrauterine hypoxia increases the production of red blood cells. However, multinucleated normoblasts in a newborn caused by hypoxia haven't been reported before. We present a newborn with multinucleated normoblasts secondary to intrauterine hypoxia. This case is important; because it is the first time multinucleated normoblasts in peripheral blood smear of a hypoxic newborn has been detected.

Normoblastemia multinuclear en un recién nacido secundaria a hipoxia. / Multinucleated normoblastemia in a newborn secondary to hipoxia.

Normoblasts may be seen in peripheral blood smear of newborns. The number of normoblasts per 100 white blood cells is generally in the range of 0-10.They can be seen more common than usual in hypoxic condition, because intrauterine hypoxia increases the production of red blood cells. However, multinucleated normoblasts in a newborn caused by hypoxia haven't been reported before. We present a newborn with multinucleated normoblasts secondary to intrauterine hypoxia. This case is important; because it is the first time multinucleated normoblasts in peripheral blood smear of a hypoxic newborn has been detected.

Es posible detectar normoblastos en los frotis de sangre periférica de los recién nacidos. En general, la cantidad de normoblastos por cada 100 leucocitos está en el intervalo de 0 a 10. Se observan con más frecuencia de lo usual ante una situación de hipoxia porque la hipoxia intrauterina aumenta la producción de eritrocitos. Sin embargo, no se había informado antes un caso de normoblastos multinucleados en un recién nacido a causa de la hipoxia. Presentamos el caso de un recién nacido con normoblastos multinucleados secundarios a hipoxia intrauterina. Este caso es importante porque es la primera vez que se han detectado normoblastos multinucleados en el frotis de sangre periférica de un recién nacido hipóxico.

Epidermolysis Bullosa with Pyloric Atresia and Aplasia Cutis in a Newborn Due to Homozygous Mutation in ITGB4.

BACKGROUND: Epidermolysis bullosa with pyloric atresia (EB-PA) is an autosomal recessive disorder due to mutations in ITGA6 and/or ITGB4, resulting in altered expression of α6ß4 integrin. EB-PA can also occur with aplasia cutis. CASE REPORT: We present a newborn with EB-PA and aplasia cutis, born of consanguineous parents, with a homozygous c.3793+1G>A mutation affecting ITGB4, previously described only in the heterozygous state with other mutations. CONCLUSION: The previously unreported homozygous c.3793+1G>A mutation affecting ITGB4 causes a severe form of junctional epidermolysis bullosa with pyloric atresia and aplasia cutis.