Day :
Session Introduction
Marta Celmina
University of Latvia, Latvia
Title: The importance of diagnosing hereditary epilepsy: EAST/SeSAME syndrome
Biography:
Marta Celmina finished Riga Stradins University in 2014. She is a Pediatric Resident of University of Latvia and also works in the Center of Epilepsy and Sleep Medicine (Clinical University Hospital in Riga, Latvia). She has three publications in reputed journals and is currently working on several research projects.
Abstract:
EAST (epilepsy, ataxia, sensorineural deafness, tubulopathy) or SeSAME (seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance) syndrome is a rare autosomal recessive syndrome first described in 2009 independently by Bockenhauer and Scholl. It is caused by mutations in KCNJ10, which encodes Kir4.1, an inwardly rectifying K+ channel found in the brain, inner ear, kidney and eye. To date, 16 mutations and at least 28 patients (plus four new Latvian patients) have been reported. Seizures are generally the initial presentation and start in infancy. Ataxia is a prominent feature evident from, when the affected individuals learn to walk. Although hearing loss in the majority of cases has been reported as stable, all our patients demonstrate advancement of hearing loss. This is especially important considering that deafness might delay language development and mimic intellectual disability. From a renal aspect, EAST/SeSAME syndrome is similar to Gitelman and Bartter syndromes with hypokalemia, metabolic alkalosis, hypomagnesemia, hyponatremia, hypochloremia, elevated plasma renin and aldosterone. The treatment is based on antiepileptic drugs, electrolyte replacement, hearing aids and mobility devices. Future research should concentrate on recognizing the lesions in the central nervous system to evaluate new potential diagnostic criteria and on formally evaluating intellectual disability. A possible genetic cause for epilepsy (as the presenting symptom), especially in combination with other symptoms, should always be considered. Early recognition of this multisymptomatic syndrome may improve the outcome in terms of electrolyte imbalance, hearing impairment and therefore, overall development.
Nada Al Theyab
Minstry of Health, Saudi Arabia
Title: Fat trajectory after birth in very preterm infants mimics healthy term infants
Biography:
Nada Al Theyab has completed her PhD in School of Medicine and specialized in Public Health at the University of Queensland in Australia. She was awarded at the 24th Royal Brisbane Women Hospital Symposium for her innovation in predicting body fat in infants from anthropometric measurements. She is interested in perinatal research along with women’s health.
Abstract:
Background: Infants born very preterm experience poor postnatal growth relative to intrauterine growth, but at term equivalent age, they have increased percentage body fat compared with infants born at term.
Aim: The aim of this study was to assess body composition in very preterm infants born before 32 weeks postmenstrual age and to compare this with infants born at 32-36 weeks of gestation.
Materials & Methods: Percentage fat, fat mass and fat-free mass were measured in 87 very preterm infants born <32 weeks of gestation and studied at 32-36 weeks and in 88 control infants born at 32-36 weeks of gestation and measured on days 2-5 postnatally.
Results: At 32-36 weeks, very preterm infants were lighter and shorter, had signiï¬cantly greater percentage fat and absolute fat mass and had a signiï¬cantly lower absolute fat-free mass than the control group. The trajectory in percentage fat over increasing postnatal age in very preterm infants was closely aligned to that in term infants.
Conclusions: Infants born very preterm accumulate fat rapidly after birth and have a deï¬cit in fat-free mass. Fat accumulation may be triggered by birth or associated events. If this rapid fat accretion is not taken into account, assessment of growth based on weight alone will underestimate the deï¬cit in fat-free mass.
Masato Kantake
Juntendo University Shizuoka Hospital, Japan
Title: Postnatal relative adrenal insufficiency results in methylation of the glucocorticoid receptor gene in preterm infants
Biography:
Masato Kantake has completed his PhD from Chiba University. He is the Director of Neonatal Medical Center of Juntendo University Shizuoka Hospital. He is Senior Associate Professor of Pediatrics, Juntendo University, School of Medicine. He has published two papers related to epigenetics in neonatal period.
Abstract:
Early life experiences impact on the neurobiological regulation of stress responsiveness, mood, and anxiety disorders. Maternal care, familial functioning, and childhood adversity all contribute to neurobiological regulation through epigenetic modification of DNA, which is thought to be highly stable across a lifespan. Hippocampal glucocorticoid receptor (GR) plays a central role in modulating hypothalamus pituitary adrenal axis activity. We have investigated the relationship between early life stress in NICU such as invasive treatment and the methylation rates in GR gene promoter 1F region which is known to be important for developing neurodevelopmental disabilities in later life. Cell-free DNA was extracted from preterm birth infants at birth and at one and two months after birth. Through bisulfite amplicon sequencing using an Illumina Miseq system and Bismarck-0.15.0 software, we identified the rate of cytosine methylation. Postnatal glucocorticoid administration to treat circulatory collapse was the most-associated independent variable with a positive regression coefficient for a change in methylation rate at nine of the 39 analyzed CpG sites. It also influenced the methylation ratio at 22 of the 39 CpG sites at two months of age. The standard deviation score of body weight at birth was extracted as an independent variable, with a negative regression coefficient at nine of the 22 CpG sites together with glucocorticoid administration. The results of this study indicate that a prenatal environment that results in intrauterine growth restriction and postnatal relative adrenal insufficiency requiring glucocorticoid administration leads to GR gene methylation. That, in turn, may result in neurodevelopmental disabilities.
Sarabon Tahura
Bangladesh Institute of Child Health, Bangladesh
Title: Therapeutic Bronchoscopy in ventilated neonates by Thin Fibreoptic Flexible Bronchoscope (FFB)
Biography:
Sarabon Tahura is a Medical graduate (MBBS) from Mymensingh Medical College under Dhaka University and obtained her professional degree FCPS (Paediatrics) from Bangladesh College of Physicians and Surgeon (BCPS). Afterwards, she has completed her fellowship in Paediatric Intervention Pulmonology from Qilu Children Hospital under Shandong University, China and training from All India Institute of Medical Science (AIIMS). She is the 1st Paediatric Interventional Pulmonologist in Bangladesh and experienced in teaching and conducting Post-graduation examinations. Many articles have been published under her name in different national and international journals and have been serving as an Editorial Board Member of repute.
Abstract:
Background & Aim: Though flexible bronchoscopy has been well established for diagnostic and therapeutic purposes, it is rarely used in neonates. Now-a-days the availability of thin and ultrathin fibre optic flexible bronchoscope (FFB) allows the therapeutic bronchoscopy in ventilated neonates. This study aimed to outlines the clinical evidence of the utility of flexible bronchoscopy in ventilated neonates by thin flexible bronchoscope for management of atelectasis in neonate.
Materials & Methods: A total of six neonates underwent bronchoscopy by thin (Diameter 2.8 mm with 1.2 mm action Channel) fibre optic flexible bronchoscopes that were in ventilator and diagnosed as atelectasis clinically and radiologically in neonatal care unit (NICU) in Square Hospitals Ltd., from July to September 2018. FFB was introduced through endotracheal tube. X-ray was done in every neonate just after bronchoscopy.
Result: The results indicated that thick mucus plug was found in five neonates and blood clot was detected in one case in different parts of the airway. Mucus plug was removed by normal saline wash and blood clot was removed by Dormia basket. Radiological and clinical improvement of atelectasis was detected just after therapeutic bronchoscopy and all neonates were weaned from ventilator within 24 hours (Mean 14 hours). Death or severe complications during or after bronchoscopy did not occur to any neonate.
Conclusions: This study documented the therapeutic advantages offered by thin flexible bronchoscopy in ventilated neonates who developed atelectasis and hypoxaemia. Immediate results of therapeutic procedures on radiographs and clinical improvement were very encouraging.
Boris M. Petrikovsky
American College of Obstetrics and Gynecology, USA
Title: Fetal Cardiac Pacemaker
Biography:
Boris Petrikovsky completed his postgraduate training at SUNY Downstate/Maimonides Medical Center (Brooklyn, NY) and his fellowship in Maternal-Fetal Medicine at the University of Connecticut in 1988. That same year, he was a visiting researcher at Kings College School of Medicine (London, UK), specializing in Fetal Medicine and Surgery. From 1992-1999, he served as a Chief of Maternal-Fetal Medicine at North Shore University Hospital. He serves as a member of the editorial board of journals of neonatal intensive care, ultrasound diagnosis in obstetrics and pediatrics. He also serves as a reviewer for the American Journal of Obstetrics and Gynecology and the Journal of Clinical Ultrasound and Obstetrics and Gynecology. His major research interests are fetal medicine, prenatal diagnosis, fetoscopy, fetal cardiology, Ob/Gyn sonography and invasive Ob/Gyn procedures. He is board certified in Obstetrics and Gynecology and Maternal-Fetal Medicine. He is a Professor of Obstetrics and Gynecology at NYU School of Medicine.
Abstract:
When function of SN cells is compromised fetal arrhythmia can arise and be detected using, echocardiography Transient fetal arrhythmias are more common than persistent fetal arrhythmias. However, persistent severe bradycardia and sustained tachycardia may cause a congestive heart failure. The progression of congestive heart failure to a hydropic state is related to an elevation of venous pressure resulting in an increased capillary permeability and edema. In the year 2000, perinatal mortality was still 100% in fetuses with heart block and bradycardia at the stage of full-blown hydrops. Fetal cardiac pacing systems were developed to save these fetuses and included pacing catheters and external pulse generators. These generators are located outside the uterus and a pacing catheter passes through the abdominal wall, uterine musculature, across the amniotic fluid directly to the heart of the fetus. However, most efforts to save a fetus so far met with failure due to either dislodging of the catheter or umbilical cord complications. Permanent fetal pacemaker will have the following features: a) to be implanted, the device does not use the umbilical vasculature and b) the cylindrical battery/pulse generator, was made short in length to avoid or decrease the chance of umbilical cord knotting or entanglement. Design for continuous fetal cardiac pacing, this pacemaker contains a pulse generator and a short pacing electrode with a spiral end, which straightens for insertion and widens after being implanted. The spiral end of the electrode maintains stable position once placed in the fetal thoracic cavity. New directions in fetal pacing-wireless pacemaker: Over the years development of cardiac pacing revealed the weakness of the wire component of the pacing system and led to the creation and manufacturing of the wireless or leadless pacemakers. However, despite the clear advantage of the leadless device, there are many technical problems still remain. Loel, et al. proposed a miniature pacemaker with a long electrode and a circuit board powered by a lithium battery. A charging system of this device works by using radio waves. The system is small and minimally interferes with fetal activity. This device is still under development and requires additional experiments to achieve optimal performance.
Aftab Yusuf Raj
Square Hospitals Ltd, Bangladesh
Title: Emerging Multi-drug Resistant Bacterial Strain in Neonatal Intensive Care Unit (NICU)s in a developing country-Bangladesh
Biography:
Aftab Yusuf Raj is a Medical Graduate (MBBS) from Sher-e-Bangla Medical College under Dhaka University and obtained MD (Pediatrics) from Bangladesh Institute of Child Health (BICH) and MD (Neonatology) from Bangabandhu Sheikh Mujib Medical University, Bangladesh. Afterwards, he has completed his training from All India Institute of Medical Science (AIIMS) and Fellowship in Neonatology from Colgery Medical University and Alberta Children Hospital, Canada. He has experience in teaching and conducting post-graduation examinations of Bangladesh and also registered by Canadian Royal College of Physicians and Surgeons. Many articles have been published under his name in different national and international medical journals.
Abstract:
Background: Neonatal sepsis remains a major health problem in any NICU worldwide. As, compared to developed countries, the use of antimicrobials in Bangladesh is less regulated and enough data are not available.
Aim: The aim of the study was to determine the causative bacteria and the level of their resistance to commonly used antibiotics in NICUs of Bangladesh.
Materials & Methods: A total of 1724 blood samples were analyzed (culture and sensitivity tests) from neonates from three major referral NICUs of Bangladesh, who were clinically diagnosed as sepsis. Only culture-positive sepsis was included in the study.
Result: Among total, 363 (21.05%) culture positive sepsis, gram negative and gram positive bacteria were found in 88.15% (n=320) and 11.84% (n=43) respectively. Acinetobacter was the most frequent (40.22%) isolated gram negative organism followed by Klebsiella pneumoniae (32.5%), Escherichia coli (7.3%) and Pseudomonas (3%). Overall, 78% of the isolates were multidrug-resistant (68% of gram-positive bacteria and 85.4% of gram-negative bacteria) against commonly used antibiotics such as ampicillin, amoxicillin, cefotaxime, ceftriaxone, gentamicin, linezolid and vancomycin. This study found new emerging bacteria- Burkholderia cepacia (n=5) and Stenotrophomonas maltophilia (n=4), which were both multidrug-resistant and only sensitive to meropenem and colistin respectively.
Conclusions: Very high percentage of multi drug resistance to the commonly used antibiotics including emerging multi drug resistant Burkholderia cepacia and Stenotrophomonas maltophilia in NICUs in Bangladesh is alarming and challenge to the management of neonatal sepsis. Continuous surveillance for antibiotic susceptibility is needed to ensure proper empirical therapy.