S Babour, A Ourrai, A Radi, R Abilkassem, A Hassani, M Kmari, A Agadr
{"title":"Infantile onset form of Canavan disease: 2 case reports","authors":"S Babour, A Ourrai, A Radi, R Abilkassem, A Hassani, M Kmari, A Agadr","doi":"10.33545/26648350.2023.v5.i2b.52","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2b.52","url":null,"abstract":"Canavan disease or N-acetyl-aspartic acid (NAA) is a rare and severe, autosomal-recessive metabolic leukodystrophy that causes spongy degeneration of the white matter by brain accumulation of NAA acid due to aspartoacylase (ASPA) deficiency. We report a 13 month-old boy case presented with macrocephaly, abnormal tonus, psychomotor delay and blindness. Brain magnetic resonance imaging and chromatography of urinary organic acids allowed to make the diagnosis. Furthermore, we also report the observation of a 12-year-old girl with healthy psychomotor development which revealed that she was experiencing facial myoclonus at a frequency of 5 seizures per day. The cerebral MRI findings indicated the presence of leukodystrophy, and MRI spectroscopy validated the diagnosis of Canavan disease by revealing an elevated N-acetyl aspartate peak. Our aim is to describe clinical, radiological, and biological presentations in the light recent literature.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135448413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohammad Naim Iqbal Mollah, Shahjadi Nasreen Sultana, Md. Tariqul Islam, Saida Binte Rahman, Begum Sharifun Naher, Mohammad Masudur Rahaman Khan
{"title":"Ultrasonographic evaluation of brain of newborn with hypoxic- ischaemic encephalopathy treated with anticonvulsant drug","authors":"Mohammad Naim Iqbal Mollah, Shahjadi Nasreen Sultana, Md. Tariqul Islam, Saida Binte Rahman, Begum Sharifun Naher, Mohammad Masudur Rahaman Khan","doi":"10.33545/26648350.2023.v5.i2b.53","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2b.53","url":null,"abstract":"Introduction: The most serious consequence of perinatal asphyxia is hypoxic-ischemic encephalopathy (HIE) which causes permanent damage to CNS tissues that may result in neonatal death or manifest later as cerebral palsy or developmental delay. Despite major advances in fetal and neonatal care, hypoxic ischemic encephalopathy (HIE), sometimes called perinatal encephalopathy, continues to cause a significant number of deaths and bong-term disabilities in newborns. Cranial ultrasonography (CUS) in neonates is safe and radiation free. CUS can be initiated even immediately after birth and hence suitable for screening. This investigation is chosen as a tool to detect any early changes that might be a significant predictor of future neurological outcome. Objective: This study was done to identify the ultrasonographic findings of brain of newborn with hypoxic- ischaemic encephalopathy. Methods: A cross sectional study was conducted for ultrasonographic evaluation of brain of newborn with hypoxic ischemic encephalopathy treated with anticonvulsant drug. In this cross sectional study, total 100 neonates were selected according to selection criteria. Parents were interviewed with a specific pre-designed and pre-tested questionnaire and some information was gathered by reviewing the document and observation. Collected data were cleaned, edited and analyzed with the help of software SPSS 17. Results: Among the study subjects majority were delivered within 40 weeks (55.0%) More than one third was delivered within ≥40 weeks (45.0%) More than half of the study subjects were male (63.0%). Average age was 13.6±2.6 (SD) and average birth weight was 2632±94.56 (SD) More than two third of the study subjects had delayed cry after birth (88.0%) and no cry less than one third (12.0%). Respiratory distress was found (66.0%) cases. Half of the subjects had bluish coloration after birth (50.0%). All the patients had convulsion after birth. Majority of the study subjects were lethargic (43.0%). More than one third was in coma (35.0%). Twenty two percent were hyper alert (22.0%). Nearly one third of the study subjects had good activity (30.0%) and good reflexes (31.0%). Majority of the study subjects were hypotonic (70.0%). Hypertonic were 20.0%. Only 10.0% were normal. Less than one third of the study subjects had no HIE (30.0%). Majority of the study subjects were in stage II HIE (67.0%). Stage III and Stage I HIE were found 20.0% and 13.0% cases respectively. Among the study subjects, normal finding in cranial USG was found for 65 (65.0%). Dilatation of the ventricle was found in (15.0%) cases. Periventricular leukomalacia and Intraventricular haemorrhage were 12.0% and 8.0% respectively. No cerebral oedema was detected. Conclusion: Cranial ultrasonography is the most widely used technique for evaluating brain morphology and cerebral lesions in neonates. USG of brain can detect several abnormalities like dilatation of the ventricles, Intraventricular haemorrhage and peri","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"58 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135449843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Supraventricular tachyarrhythmia associated with left ventricular dysfunction in neonates & infants: Experience from a Tertiary Care Children’s Hospital in Dhaka, Bangladesh","authors":"Dr. Rezoana Rima","doi":"10.33545/26648350.2023.v5.i2b.54","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2b.54","url":null,"abstract":"Introduction: Supraventricular Tachyarrhythmia (SVT) in neonates & infants may results in left ventricular (LV) dysfunction if persists for long time. Sometimes it may result into cardiomyopathy & symptomatic heart failure. Left ventricular systolic function improves or normalizes and symptoms resolve if rhythm is corrected or rate controlled is achieved. The purpose of the study is to observe clinical features, management & outcome of various SVT associated with left ventricular dysfunction admitted at cardiac centre of Bangladesh Shishu Hospital & Institute. Methodology: This prospective cross-sectional study was conducted in all the neonates & infants admitted with confirmed SVT with LV dysfunction. SVT was confirmed by ECG criteria & LV dysfunction was confirmed by Echocardiography. All the patient underwent treatment according to unit SVT management protocol for different types of SVT. Presenting complaints, ECG findings, Echo findings, Antiarrhythmic medicine with which the SVT is reverted to sinus rhythm, duration required for reversion, any complication during management was recorded. These patient was followed up at centre’s arrhythmia clinic 1 month, 3 months, 6 months and 1 year after discharge from hospital. During follow up the ongoing antiarrhythmic medicine dose, duration & discontinuation, any side effects, ECG & ECHO findings was recorded. Result: Total 18 neonates & infants were admitted with confirmed SVT with LV dysfunction during the study period. Median age was 43 days & median ventricular rate was 250 BPM. One third of patient presented with symptoms & signs of heart failure, 22% presented with circulatory shock, 44% presented without heart failure or shock. The most frequent SVT was Atrio-ventricular re-entry tachycardia (AVRT) 39%(7), Atrial ectopic tachycardia (AET) focal & multifocal constitute 33.3% (6), Two patient (11.1) each diagnosed as Congenital junctional ectopic tachycardia(JET) & Permanent junctional reciprocating tachycardia (PJRT). Only one infant diagnosed as Atrial flutter. Five patient (27.8%) has severe left ventricular (LV) dysfunction with ejection fraction of less than 35%. Median duration for reversion to sinus rhythm was 26 hours. Median duration of maintenance therapy was 7 months. Recurrence of SVT happened in 8 patients (44.4%) during maintenance therapy & median duration of antiarrhythmic medicine free period was 36 months. Conclusion: Supraventricular tachycardia with LV dysfunction is an under appreciated, but treatable condition in pediatric patients with structurally normal hearts. It shows complete normalization of cardiac function and generally has an excellent prognosis once the arrhythmia is controlled.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135610794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Paediatric visage paralysis: A comprehensive examination and perspectives on treatment strategies","authors":"Dr. Mayyar Soheil Alkawadri","doi":"10.33545/26648350.2023.v5.i2a.51","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.51","url":null,"abstract":"The objective of this article is to present a comprehensive examination of paediatric facial paralysis, encompassing its causes, prevalence, evaluation, diagnostic procedures, and potential treatment modalities. Facial paralysis refers to the condition when an individual has an inability to initiate the contraction of facial muscles, hence resulting in the inability to exhibit facial expressions. In general, the annual incidence rates of this phenomenon are 2.7 per 100,000 children under the age of 10 and 10.1 per 100,000 children aged 10 and above. Facial paralysis can be attributed to several etiological factors, leading to diverse results and necessitating varying treatment approaches. Corticosteroids and face treatment are commonly employed in medical care. However, in cases where facial palsy continues, the consideration of facial deformity surgery arises as a potential intervention to enhance facial symmetry, safeguard vision, and restore dynamic mobility.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"121 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135007096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A comparative approach of medical professionals towards pattern of drug prescription for acute diarrhoea in children","authors":"Dr. Zaheeruddin Mohammed, Dr. Rafique Memon","doi":"10.33545/26648350.2023.v5.i2a.47","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.47","url":null,"abstract":"The interactions between 1334 children with diarrhoea and medical professionals (32 paediatricians and 68 family physicians) were studied. More over half (51%) of GPs and 67% of paediatricians recommended oral rehydration salt (ORS) in encounters involving more than 500 patients. More than 40% of primary care physician visits and 42% of paediatricians visits involved the prescription of an antibiotic, 65% of primary care physician visits involved the prescription of an antidiarrheal medication, and 42% of primary care physician visits and 36% of paediatricians visits involved the prescription of an antiamoebic medication. When we analysed all of the visits, we found that general practitioners (GPs) and paediatricians (paediatricians) prescribed ORS in 55 and 53% of visits, antibiotics in 44 and 39%, antidiarrheals in 47 and 32%, and antiamoebics in 29 and 24%. Cotrimoxazole was among the most commonly recommended antimicrobial drug by both primary care physicians and specialists. In general practise, antidiarrheals were prescribed more frequently than in Paediatric practise. Seventy-seven percent of the time, primary care physicians used \"mixtures,\" or home-made medication preparations. Patient visits with paediatricians lasted an average of 8 +/- 3 minutes, while visits with general practitioners lasted an average of 4 +/- 1 minutes. These findings point to an issue with under-prescribing ORS and over-prescribing antibiotics, anti-amoeba drugs and anti-diarrhea drugs. Prescription habits of these populations require careful planning of intervention techniques.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"39 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135446513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A quasi experimental study to assess the effectiveness of Structured Teaching Programme (STP) on knowledge regarding prevention of neonatal hypothermia among mothers of newborn in (MP)","authors":"Vandana Kushwaha, Dr. Ratna Chhaya Singh","doi":"10.33545/26648350.2023.v5.i2a.50","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.50","url":null,"abstract":"A quasi experimental study with pre and posttest without control group design was undertaken on 50 mothers of newborn at Gandhi Memorial hospital Rewa (M.P.) and the samples were selected by purposive sampling technique. Data were collected by the use of structured closed ended questionnaire and analyzed by using descriptive & inferential statistics. Findings revealed that the overall mean score in the pretest was (7.82+2.77) which is 19.55% of the total score revealing that the mothers had poor knowledge regarding care of newborn on prevention of hypothermia whereas the overall posttest knowledge score was (35.12+2.01) which is 87.8% of the total score revealing excellent knowledge score. Highly significant difference was found between pre and posttest knowledge scores. No Significant association was found between posttest knowledge scores with their selected demographic variables. Statistical analysis of data revealed that STP was effective in improving knowledge of the mother regarding care of newborn to prevent hypothermia.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"123 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135008509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Children with congenital heart disease: Case of appearance of bronchiolitis","authors":"Dr. Zaheeruddin Mohammed, Dr. Rafique Memon","doi":"10.33545/26648350.2023.v5.i2a.46","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.46","url":null,"abstract":"Usually harmless in infants less than a year, the viral respiratory infection known as bronchiolitis can be life-threatening in youngsters with preexisting cardiac conditions. Our goal was to explain the connection between bronchiolitis and cardiovascular disease from every angle possible: Epidemiological, clinical, therapeutic, and evolutionary. Over the course of 5 years, from 2018 to 2023. There were 87 cases and 165 controls in all. One percent of all hospitalizations were due to bronchiolitis caused by cardiac defects. Isolated septal defect of the ventricular was the most common kind of cardiac disease seen during a bronchiolitis episode. About 54% of the cases were really serious. The presence of cardiopathy was linked to a number of factors, including the degree of seriousness of bronchiolitis with odds ratio 2.72, the use of oxygen therapy (odds ratio 24.28), the length of time spent in the hospital (odds ratio 7.1), the presence of a progressive course, and the severity of the cardiopathy itself (odds ratio 26.72). The death rate was 15.2 percent. Bronchiolitis is prevalent among kids who have congenital heart disease. Therefore, consider the likelihood of congenital heart disease when dealing with an episode of bronchiolitis, especially in the severe forms.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135446514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dr. Paramesh Sreekumar, Dr. Maryam Yusuf Alheddi, Dr. Minoosh Nasef, Dr. Emad Shatla
{"title":"Extreme preterm survival and short term outcomes study","authors":"Dr. Paramesh Sreekumar, Dr. Maryam Yusuf Alheddi, Dr. Minoosh Nasef, Dr. Emad Shatla","doi":"10.33545/26648350.2023.v5.i2a.48","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.48","url":null,"abstract":"Background: Advances in perinatal care have aided in the steady rise in survival rate among extreme preterm babies. Babies in periviable age often pose a serious challenge to medical practitioners and parents alike. Aim: To examine the trend of survival and short term morbidities among the extreme preterm babies over the study period Methods: A retrospective study in which data was collected for a period of last five years. Detailed evaluation of medical records of babies admitted to the Neonatal Intensive Care Unit was done. Results: There were 107 infants included in the study. Survival rates of preterm term during the study period was stable. Infants with a higher gestation had higher survival rates (p = 0.009). More number of survivors were noted among preterm with higher birth weight categories (p = 0.001). BPD and sepsis were most prevalent morbidity seen among the preterm babies. More cases of BPD were noted among infants less than 26 weeks of age (0.001). Conclusion: A systematic approach by a multidisciplinary team with active participation of the parents will help in making judicious decisions pertaining to ideal medical management of extreme preterm babies.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"23 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135497032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Anti-TNF therapy in pediatric inflammatory bowel disease: Systematic review","authors":"Duta Putra Sundana","doi":"10.33545/26648350.2023.v5.i2a.49","DOIUrl":"https://doi.org/10.33545/26648350.2023.v5.i2a.49","url":null,"abstract":"Introduction: Anti-TNF drugs have transformed IBD treatment since their introduction over two decades ago. Anti-TNF drugs like infliximab (IFX) and adalimumab (ADA) can elicit and maintain corticosteroid-free remission in adults and adolescents with Crohn's disease (CD) and ulcerative colitis. For decades, corticosteroid (CS) has been the main treatment for IBD, despite its substantial side effects, especially on growth and development. The aim: This article demonstrated study on anti-TNF therapy in pediatric IBD (inflammatory bowel disease). Methods: This study demonstrated that it met all of the requirements by comparing itself to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020 guidelines. As a result, the specialists were able to ensure that the study was as up to date as possible. Publications published between 2013 and 2023 were considered for this search strategy. This was accomplished using a variety of online reference sources, including Pubmed and SagePub. It was chosen not to include review pieces, previously published works, or works that were just partially completed. Result: In the PubMed database, the results of our search brought up 46 articles and SagePub 52 articles. The results of the search conducted for the last year of 2013 yielded a total 11 articles for PubMed and 9 articles from SagePub. In the end, we compiled a total of four papers. Conclusion: Early anti-TNF treatment is becoming more common in juvenile inflammatory bowel disease, notably Crohn's. Targeting higher dosages (> 5 µg/mL) with proactive TDM has been proven to increase disease remission rates and anti-TNF biologic durability in real-world and clinical investigations in children.","PeriodicalId":492247,"journal":{"name":"International Journal of Pediatrics and Neonatology","volume":"84 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136209151","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}