Global Summit on
Advances in Pediatrics and Pediatric Neurology

Biography:

Alexey N. Biyanov, pediatric cardiologist, doctor of higher category, PhD of medical Sciences, associate professor of Pediatrics Department of Wagner Perm State Medical University. He graduated from the medical University in 2003. In the year of 2009 defended his thesis "Sick sinus syndrome in children". An author of 27 scientific papers and one patent for invention. Currently, works as a pediatric cardiologist at the “Federal Center for Cardiovascular Surgery n. a. S.G. Sukhanov” of Healthcare Ministry of Russian Federation, Perm. Deals with the problems of diagnosis and treatment of children with congenital heart disorders. Research interests are patients with univentricular hemodynamics and mathematical modeling of systemic pulmonary anastomoses. Also specializes in the neonatal cardiology arena, makes examinations and follow ups of premature infants with cardiovascular diseases. Conducts skills training for pediatric cardiologists and neonatologists.

Abstract:

Statement of the problem: Recent medical technologies help to raise the survival rate of infants with extremely low-birth-weight who are more likely to develop critical conditions. One of complications of the neonatal period in premature infants (gestation age less than 28 weeks) is hemodynamically significant patent ductus arteriosus (hsPDA). Echocardiography (ECHO) is the current gold standard for the diagnosis of PDA –its hemodynamic significance, anatomy and post-treatment follow-up. Obviously, researcher’s experience as well as technical facility capability is important. At present extra markers, are being searched and they would help the practitioner to define the treatment of hsPDA patients. There are researches evidencing the diagnostic significance of natriuretic peptide in preterm infants with hsPDA what allow to optimize the therapeutic approach for very low-birth-weight (VLBW) and extremely low-birth-weight (ELBW) infants. The purpose of this research is to study natriuretic peptide to determine the PDA hemodynamic significance in preterm infants with ELBW. Methods and results: we examined 37 newborn babies with a body weight less than 1500gr on 3^rd day after birth using echocardiography, defined the standard criteria of PDA hemodynamic significance, and compared them with the value of natriuretic peptide. It was evaluated with the level NT-pro BNP in blood serum defined by Chemi Luminescent Immuno Assay (CLIA), test system VITROS NT-proBNP, Ortho-Clinical Diagnostics, Inc. In compliance with echocardiographic measurements, hemodynamically significance of the PDA was determined in 54.0 % (20/37) of newborn. There was median value NT-proBNP 9180 pcg/ml [IQR 3505]; 21300).  We established that echocardiographic measurements of hemodynamic significance correlates with level NT-pro BNP (r=0. 63). We used regression analysis and obtained a regression equation y=-1.85+0.00016x, where x- value of NT-pro BNP (pcg/ml). Through ROC-analysis defined the cut-off value of NT-pro BNP= 18000 pcg/ml (sensitivity Se=0.50, specificity Sp= 0.91). Positive Predictive Value (+VP) for BNP=18000 pcg/ml compounds 0.91 (Contour Interval 0.82-1.0). Likelihood ratio of LR (-) =0.54. Thus research revealed that hsPDA will be presented 5.5 times more often in VLBW infants (<1500gr) and the level NT-proBNP=18000 pcg/ml (determined with the test system VITROS NT-pro BNP). Conclusions: Natriuretic peptide pro BNP with high specificity and probability can be used as a predictive marker of hemodynamic significance of PDA in infants with very low-birth-weight.

Biography:

Kanij Fatema is a Associate Professor in Bangabandhu Sheikh Mujib Medical University. she has Published her research work in many reputed journals.

Abstract:

Inborn errors of metabolism (IEM) constitute a diverse group of genetic disorders characterized by defects in biochemical pathways. Many have a predominantly neurological phenotype reflecting accumulation of neurotoxic metabolites and/or a deficiency of substrates critical for normal neurological development and function.

In this presentation, the pattern of neurometalbolic disorders found in Bangladesh would be discussed. There is very limited study regarding metabolic disorders affecting nervous system till date particularly in developing country. Thus this presentation would highlight the scenario of neurometabolic diseases in Bangladesh. Here along with the discussion of the neurometabolic diseases, some cases would be discussed with clinical features, investigation profile, neuroimaging , treatment and outcome. In this regard, we would like to share our experience with some interesting cases like methylmalonic academia, nonketotic hyperglycemia, urea cycle disorder, molybodynum cofactor deficiency, isovaleric academia, leigh disease etc. Moreover, a study to observe the incidence of inborn error of metabolism in 50 sick newborn in a tertiary care hospital in Bangladesh would be discussed .

.Conclusion: Although the burden of infectious disease is high in developing country like Bangladesh, neurometabolic disorders are not uncommon which should be recognized and treated in spite of limited resources. This presentation highlights the reorganization and management of those cases.

Biography:

Kanij Fatema is a Associate Professor in Bangabandhu Sheikh Mujib Medical University. she has Published her research work in many reputed journals.

Abstract:

Inborn errors of metabolism (IEM) constitute a diverse group of genetic disorders characterized by defects in biochemical pathways. Many have a predominantly neurological phenotype reflecting accumulation of neurotoxic metabolites and/or a deficiency of substrates critical for normal neurological development and function.

In this presentation, the pattern of neurometalbolic disorders found in Bangladesh would be discussed. There is very limited study regarding metabolic disorders affecting nervous system till date particularly in developing country. Thus this presentation would highlight the scenario of neurometabolic diseases in Bangladesh. Here along with the discussion of the neurometabolic diseases, some cases would be discussed with clinical features, investigation profile, neuroimaging , treatment and outcome. In this regard, we would like to share our experience with some interesting cases like methylmalonic academia, nonketotic hyperglycemia, urea cycle disorder, molybodynum cofactor deficiency, isovaleric academia, leigh disease etc. Moreover, a study to observe the incidence of inborn error of metabolism in 50 sick newborn in a tertiary care hospital in Bangladesh would be discussed .

.Conclusion: Although the burden of infectious disease is high in developing country like Bangladesh, neurometabolic disorders are not uncommon which should be recognized and treated in spite of limited resources. This presentation highlights the reorganization and management of those cases.

Biography:

Kanij Fatema is a Associate Professor in Bangabandhu Sheikh Mujib Medical University. she has Published her research work in many reputed journals.

Abstract:

Inborn errors of metabolism (IEM) constitute a diverse group of genetic disorders characterized by defects in biochemical pathways. Many have a predominantly neurological phenotype reflecting accumulation of neurotoxic metabolites and/or a deficiency of substrates critical for normal neurological development and function.

In this presentation, the pattern of neurometalbolic disorders found in Bangladesh would be discussed. There is very limited study regarding metabolic disorders affecting nervous system till date particularly in developing country. Thus this presentation would highlight the scenario of neurometabolic diseases in Bangladesh. Here along with the discussion of the neurometabolic diseases, some cases would be discussed with clinical features, investigation profile, neuroimaging , treatment and outcome. In this regard, we would like to share our experience with some interesting cases like methylmalonic academia, nonketotic hyperglycemia, urea cycle disorder, molybodynum cofactor deficiency, isovaleric academia, leigh disease etc. Moreover, a study to observe the incidence of inborn error of metabolism in 50 sick newborn in a tertiary care hospital in Bangladesh would be discussed .

.Conclusion: Although the burden of infectious disease is high in developing country like Bangladesh, neurometabolic disorders are not uncommon which should be recognized and treated in spite of limited resources. This presentation highlights the reorganization and management of those cases.

Biography:

Degree in Medicine and Surgery, Federico II Faculty of Medicine, University of Naples, Italy; Postgraduate Diploma in Child Neuropsychiatry, Federico II Faculty of Medicine, University of Naples and in Pediatrics, Faculty of Medicine of Catanzaro, University of Reggio Calabria, Italy; Academic degree: Aggregate Professor of Child Neuropsychiatry, Department of Medical and Surgical Sciences, University "Magna Graecia" of Catanzaro, Italy; Member appointed by the Dean School of Medicine and Surgery in the University Committee for Disability, University “Magna Graecia of Catanzaro. Health degree: Head of Child Neuropsychiatry Service, U.O.C. of Pediatrics, "Pugliese-Ciaccio” Hospital, Catanzaro.

Dr Marotta has her expertise in:1) Clinical-behavioral assessment of individuals with Autism Spectrum Disorder, 2) Evaluation of the evolutionary pathway of subjects affected by neurodevelopmental disorders, with particular reference to intellectual disability and epilepsy.

Abstract:

Introduction: Close-loop VNS neurostimulator is able to detect episode of ictal tachycardia and identify it as pre-critical episode, therefore linked to onset of a crisis. In addition to standard VNS open-loop stimulation, this responsive device provides automatic stimulation produced in response to sudden increase in heart rate of at least 20% of the basic one. We wanted to show our experience with use of responsive Aspire SR 106 system in drug resistant pediatric epilepsy. Material and method: We selected 6 patients, 3 males and 3 females, 2 with Post Traumatic Epilepsy, 1 with Postanossic, 2 with Criptogenetic Epilepsy. Age at implantation was 13 + _ 4.4 years, duration of disease was 9 + -2 years. All patients underwent to implant of responsive Aspire SR 106: surgical procedure was not different from implantation of standard VNS system but position of generator was more medially. Stimulation started 15 days after implant and effective amplitude was achieved with increments of 0.50 mA every two weeks. Follow-up varied from 6 months to 12 months. Results: 1 patient presented complete resolution of seizures with control of both partial and generalized seizures, 3 patients reduction of seizures frequency > 50%, 1 patient reduction <50%, while 1 patient did not present substantial improvement. In responder patients was also detected significant reduction in seizures duration and thy declared themselves satisfied with the result. In one case there were side effects consisting of cough and dysphonia that limited the increase of stimulation amplitude. VNS had effects on QoL with reduction in anxiety, mood improvement, enhanced verbal recognition memory. Conclusions: Responsive VNS therapy may be considered in pediatric patients with medically refractory epilepsy who are not favorable candidates for resection. It is safe procedure with low morbidity. VNS decreases seizure frequency but also reduce their intensity and has favourable effect on patient’s mood.

Biography:

Professor in Early Childhood Division in the Faculty of Education at the Hacettepe University. Her research includes early childhood special education, quality of inclusive classrooms, professional development of teachers and parent training.

Abstract:

The early years are valuable for all children and being in an inclusive environment with same-age, typically developing children is essential to the development of young children with developmental disabilities. The children with disabilities may have various levels of difficulties in social, cognitive, and emotional skills, and problems in physical well-being. However, the results of the studies investigating the effects of preschool inclusion appear to be promising regarding the developmental outcomes of children with disabilities. In this presentation, I will share the results of the Preschool Inclusion Project aiming to determine both the developmental outcomes of inclusive preschools and predictors of developmental gains of the children with disabilities.

In Turkey, inclusion is accepted as being a service model for children with developmental disabilities. It is mandatory that all needs of young CWD must be met in public and private preschools, through the provision of the necessary support services. Although research focusing on the aspects of inclusive practices accelerated in the last decades; there is currently a lack of research addressing what extent inclusive preschools in Turkey support the development of children with disabilities and typically developing children. Therefore, to analyze the effects of inclusive preschools in the development of children with disabilities and their typically developing peers, The Preschools Inclusion Project, a longitudinal study, was started in 2015.

Data addressing the social skills, problem behaviors, school adjustment, student-teacher relationship, home environment and the developmental functions of children with and without disabilities were collected from the mothers and teachers, while trained assistants assessed the children's development. Analyses indicated that although all children made developmental gains; psychomotor, language and socioemotional developmental gains were greater for children with disabilities than those of the typically developing children. Social skills and school adjustment were found to be significant predictors of developmental gains of children with disabilities. Implications of these findings will be discussed in terms of the importance of a rich environment for the development of children with disabilities, in that there were children with skills at different levels

Biography:

Hatice BakkaloÄŸlu is an associate professor of Special Education at the Ankara University. Her research focuses on early interventions for children with disabilities, inclusive preschools and preschool and special education teacher training.

Abstract:

The early years are valuable for all children and being in an inclusive environment with same-age, typically developing children is essential to the development of young children with developmental disabilities. The children with disabilities may have various levels of difficulties in social, cognitive, and emotional skills, and problems in physical well-being. However, the results of the studies investigating the effects of preschool inclusion appear to be promising regarding the developmental outcomes of children with disabilities. In this presentation, I will share the results of the Preschool Inclusion Project aiming to determine both the developmental outcomes of inclusive preschools and predictors of developmental gains of the children with disabilities.

In Turkey, inclusion is accepted as being a service model for children with developmental disabilities. It is mandatory that all needs of young CWD must be met in public and private preschools, through the provision of the necessary support services. Although research focusing on the aspects of inclusive practices accelerated in the last decades; there is currently a lack of research addressing what extent inclusive preschools in Turkey support the development of children with disabilities and typically developing children. Therefore, to analyze the effects of inclusive preschools in the development of children with disabilities and their typically developing peers, The Preschools Inclusion Project, a longitudinal study, was started in 2015.

Data addressing the social skills, problem behaviors, school adjustment, student-teacher relationship, home environment and the developmental functions of children with and without disabilities were collected from the mothers and teachers, while trained assistants assessed the children's development. Analyses indicated that although all children made developmental gains; psychomotor, language and socioemotional developmental gains were greater for children with disabilities than those of the typically developing children. Social skills and school adjustment were found to be significant predictors of developmental gains of children with disabilities. Implications of these findings will be discussed in terms of the importance of a rich environment for the development of children with disabilities, in that there were children with skills at different levels

Biography:

Dr Amirhossein Hosseini has his expertise in field of pediatric gastroenterology and hepatology.

Abstract:

  Statement of the Problem: Wilson's disease (WD) is a genetic disorder   with various clinical presentations due to excessive accumulation of copper in the liver and other organs. It can present as acute/chronic hepatitis, liver failure, extrahepatic and neuromuscular manifestations. Autoimmune hepatitis (AIH) is a necro-inflammatory disease of the liver, which affects a lot of people particularly the children population. AIH has a broad clinical presentation that is similar to WD. Coexistence of WD with elevated creatinine phosphokinase (CPK) and AIH, may be a diagnostic dilemma

Case: We presented a 6 years old boy with dysarthria, aggressive behavior, weak attention, concentration and weight loss with abnormal physical examination. Laboratory, histochemical, genomic studies, muscle/liver biopsy and atomic absorption test confirmed the diagnosis of both WD and AIH in the boy.

Conclusion & Significance: Although CPK and liver enzyme elevation is a rare presentation of chronic hepatitis with dominant feature of WD and AIH; however, simultaneous therapy with immunosuppressive drugs and Penicillamine may have superior benefit with a significant response.