Congenital Heart Defects in Hirschsprung’s Disease: A Survey in Iranian Population Abstract Hirschsprung’s disease (HSCR) may be accompanied by other anomalies including congenital heart disease (CHD) resulting in additional complications. This study was performed to evaluate the prevalence and type of concomitant CHD in hospitalized children with Hirschsprung’s disease. Materials and Methods: All HSCR patients (129 children) admitted to Mofid children’s hospital-Tehran, from April 2016 to August 2019 were studied in a descriptive cross-sectional study. Two-dimensional, M-mode and pulsed, continuous, and color-Doppler provided echocardiography were applied to evaluate cardiac structure and function. Results: Congenital heart disease (CHD) was observed in 48 (37.2%) children. The most common anomalies were ASD in 20(15.5%), VSD in 1(0.8%), PDA in 2(1.6%), Tetralogy of Fallot (TF) in 3(2.3%), ASD and pulmonary stenosis (PS) in 2(1.6%), ASD and PDA in7 (5.4%), ASD and VSD in 3(2.3%), VSD and PDA in 2 (1.6%) of the patients. Conclusion: Cardiac anomalies are relatively prevalent in our Iranian Hirschsprung’s disease population. We recommend early echocardiographic evaluation in the setting of HSCR.

Introduction: Asphyxia may lead to serious complications among them acute kidney injury (AKI) is the most common. Early diagnosis of AKI can help prevent impaired acid-base, fluid and electrolytes balance that may lead to life-threatening complications. The current study has aimed to assess efficacy of KIM-1 and Cystatin-C in early diagnosis of AKI among asphyxiate neonates. Methods: This is a case-control study conducted on 45 asphyxiate neonates, which 24 were in the control group and 23 in the case group.Creatinine, KIM-1 and Cystatine-C were measured for participants within 8- and 4 days after birth and compared between cases and controls. Results: The mean level of Cr-Standardized KIM1 8 hours and 4 days after birth was significantly higher in case group compared to the control (p-value 0.67 ng/ml allows to predict kidney failure in newborns with 57.1‌% sensitivity and 86.4‌% specificity. Conclusion: Our study showed that high-specificity Kim-1 is a good biomarker for early detection of acute renal failure in asphyxiated infants, but similar expectations of cystatin cannot be expected for at least the first 8 hours after birth.

Primary craniosynostosis is a from of premature fusion of the cranial sutures, an event which usually occurs prenatally. Both syndromic and nonsyndromic forms exist. Most cases are of unknown etiology; genetic syndromes account for 10%–20% of cases, of which Apert, Crouzon and Pfeiffer syndromes are the most common. Scaphocephaly occurs from premature closer of the sagittal suture and is the most common form of craniosynostosis. Frontal plagiocephaly is the next most common form and results from premature fusion of a coronal and sphenofrontal suture. Palpation of the suture at birth often reveals a bony ridge. Skull radiograph or head CT may be considered. Certain genetic forms of craniosynostosis are caused by mutations in TWIST, FGFR1, FGFR2, or FGFR3. Apert syndrome is a rare congenital disorder characterized by craniosynostosis (such as cone-shaped calvarium) and severe symmetrical syndactyly of the hands and feet. We have aimed to present a newborn male patient having all the features of classical Apert syndrome.

Objective: To evaluate the outcome of radiofrequency ablation of the umbilical cord in the reduction of Twin Reversed Arterial Perfusion (TRAP) sequence or acardiac twin in monochorionic diamniotic pregnancies. Material and methods: In this prospective study, 25 cases of monochorionic diamniotic pregnancy with one acardiac twin underwent selected reduction using radiofrequency ablation (RFA) in the second trimester of gestational age, from 2018 to 2019 in a tertiary referral hospital affiliated to Tehran University of Medical Sciences. Results: The overall survival rate was 84%. The mean±SD of gestational age at the time of the procedure and delivery were 20.0±3.8 and 35w±6.0 days respectively. Nine neonates were admitted to the neonatal intensive care unit (NICU) and 4 miscarriages were recorded in less than 24 weeks. The procedures in all cases were technically successful in achieving selective termination. Conclusion: Radiofrequency ablation for fetal reduction in acardiac twins in monochorionic pregnancies appears to be a safe and reasonable option.

Background: Kangaroo mother care is essential improves outcomes of premature and low birth weight infants. Even though kangaroo mother care is now recognized by global experts as an integral part of essential newborn care, the adoption and implementation of the kangaroo mother care is still challenging. Aim of this study to explore perceived enablers and barriers of kangaroo mother care among mothers and nurses in neonatal intensive care unit.Methods: Descriptive Phenomenological study design was conducted in Tikur Anbessa Specialized referral Hospital at Addis Ababa with 13 mothers and 7 nurses from 10th May -15th July, 2020. In-depth interview used with semi-structured questionnaire and data was collected till saturation of information. Thematic analysis was done with ATLAS.Ti software version 7.5.16 .Results: Major enablers and barriers of practicing kangaroo mother care among mothers and nurses reported that lack of understanding of KMC, family responsibility and workload, lack of awareness of KMC by community, social practice and traditional adaptation were the barriers to practice of KMC. Poor supervision and follow-up, limited resource especially sanitation resource are the major barriers related to health staff and setting. Nurses reported that scale- up of kangaroo mother care was influenced by absence of training, poor attention given by managers and administrative, shortage of rooms and facilities, workload and time shortage.Conclusion: A complex array of barriers and enablers determine a mother’s and nurses ability to provide KMC. Improve the mothers' to practice KMC and to promote the health of preterm infants, supports such as family, community and health professional support needed. Nurses needed in-service education, proper administration and less workload to promote KMC practice.

Cantrell's pentalogy (CP) is an orphan congenital disease resulting from embryological alterations of the mesoderm, characterized by a defect in the lower sternal portion, supraumbilical abdominal wall, anterior diaphragm as well as the diaphragmatic pericardium, and cardiac alterations. We report the case of a male newborn with a prenatal diagnosis of abdominal wall defect, in whom a class 1 or complete phenotype of Cantrell's Pentalogy was diagnosed and early measures were taken to prevent adverse outcomes related to this disease. It is known that congenital syndromic disease, such as CP, may be timely addressed with primary prevention strategies and adequate prenatal controls, also, early diagnosis will permit an effective clinical and surgical management of the patient thus leading to a positive prognosis. Finally, it has been established that in this population, proper decision-taking of therapeutic possibilities during the patient’s early years may improve their quality of life and their lifespan.

Background This study aimed to evaluate the competence of the score for neonatal acute physiology (SNAP-II) as a tool to anticipate morbidity and mortality of neonates with early or late sepsis in neonatal intensive care units (NICUs).Methods This prospective cohort study was conducted on all neonates of > 32 weeks with sepsis in tertiary NICUs at Cairo University Children Hospital and El Galaa Hospital For Armed Forces Officers Families, Cairo, Egypt, within May-October 2019. The eligible samples consisted of 100 neonates with septicemia who met inclusion and exclusion criteria and were enrolled. the score for neonatal acute physiology-II was calculated within 24 h of sepsis onset and followed up for 2 weeks for mortality and organ dysfunction (OD). The collected data were analyzed in SPSS software (version 25).Results It was revealed thatSNAP-II was significantly higher in neonates who passed away, compared to the survived neonates (46±17 vs. 12±10, respectively; P<0.001). Moreover, SNAP-II was significantly higher in neonates who developed OD within 14 days of sepsis onset, compared to those without OD (37±17 vs. 9±7, respectively; P<0.001). The score for neonatal acute physiology-II at 14.5 was considered the best cut-off point in predicting OD with a sensitivity of 100%, positive predictive value of 70.4%, specificity of 81.2%, and negative predictive value of 100%. In addition, SNAP-II at 23.5 was considered the best cut-off point in predicting overall mortality with 100% sensitivity, 58.6% PPV, 85.5% specificity, and 100% NPV.Conclusion Higher SNAP-II within 24 h of the early- or late-onset neonatal sepsis was a reliable predictor of OD and death.

Background: Coronavirus disease 2019 (COVID-19) is an emerging viral disease with a high rate of transmissibility that has spread and become the first pandemic of the century. There are limited data available regarding COVID-19 during pregnancy. An important question is whether pregnant mothers transmit the virus to their fetuses or newborns. Case report: This study was conducted to investigate the vertical transmission of the COVID-19 virus from pregnant mothers to fetuses. This study reported the case of a preterm newborn admitted to the neonatal intensive care unit of Imam Khomeini Hospital Urmia, Iran, from a COVID-19- positive mother with severe respiratory illness. A preterm female newborn with a gestational age of 34 weeks, 1,800 g weight, and 1-minute and 5-minute Apgar scores of 4 and 5, respectively, was born through the emergency cesarean section from a 39-year-old gravid 1 mother. She was positive for COVID-19, in two consecutive reverse transcription polymerase chain reaction tests which were performed 48 h apart from each other. Conclusion: According to the limited available documents of our case, the researchers claimed that vertical transmission of COVID-19 was possible at this time.

Background: Recent surge in the diagnoses of congenital hypothyroidism (CH) has necessitated the measurement of newborn TSH levels and understanding of the way various antenatal and perinatal factors influence its dynamicity. It is a cross-sectional study on newborns delivered or admitted to the All India Institute of Medical Sciences Raipur (AIIMS Raipur), Chhattisgarh, India. Methods: Dried blood spot analysis of newborn thyroid-stimulating hormone (nTSH) was carried out on 1,216 newborns after ethical clearance. The TSH levels were presented in percentage to determine the distribution in the study population. The mean values were compared within the groups categorized under each variable. The newborns' variables in this study included birth weight, Ponderal index, and neonatal complications. The studied maternal variables included antenatal visits, maternal age, gestational age, anemia, and mode of delivery. Results: The mean (standard error of the mean) for nTSH was obtained at 3.37 (0.12) mIU/L and 97% of newborns were below 8 mIU/L. Mean nTSH was significantly high in mothers older than 30 years (P=0.019) and those with anemia during the antenatal period (p <0.001). It was significantly raised in babies with complications (P=0.004). Besides, higher values were also observed in low birth weight babies and those with low Ponderal Index. Higher nTSH was observed among newborns born to mothers with a thyroid disorder, those delivered prematurely and/or by cesarean section, and those with no antenatal visits. Conclusion: It is highly essential to take a precautionary note on the antenatal status of mothers in terms of advanced age, premature delivery, associated maternal diseases, mode of delivery, newborn’s birth weight, and complications which are supposed to influence the dynamicity of thyroid hormones in newborns and result in CH.

Background: Dengue in pregnancy is associated with adverse maternal and fetal outcomes, including perinatal transmission. Case report: We report a case of neonatal dengue in a baby born to a 29-year-old primigravida at 38 weeks of gestation. She developed acute dengue fever 2 days prior to delivery. Her dengue nonstructural protein 1 antigen was reactive. She delivered a term baby girl via spontaneous vaginal delivery. Her fever persisted in the post-partum period which was associated with post-partum hemorrhage, altered coagulation, and liver function. She was clinically diagnosed to have hemophagocytic lymphohistiocytosis complicated with disseminated intravascular coagulation and treated with intravenous (IV) dexamethasone and multiple blood products, including fresh frozen plasma and platelet concentrate. She recovered in over the next 5 days. The baby girl was born with a birth weight of 3040g and developed fever on the third day of life with poor perfusion, associated with mottling and hypotension. The baby was treated with IV fluids, inotropes, and supportive care. The fever subsided after 48 h, along with clinical improvement, but continued with thrombocytopenia. The baby did not have any bleeding. Platelet recovery started on the 11th postnatal day (i.e., the 8th day of illness), and platelet count was normalized at 2 weeks. Dengue serology immunoglobulin M by enzyme-linked immunosorbent assay was positive for both mother and baby. The clinical diagnosis was confirmed by laboratory tests. Conclusion: Dengue fever in mothers very late in pregnancy can cause symptomatic dengue infection in neonates.