Asian Journal of Pediatric Research最新文献

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The Outcome of a Single Stage Repair for Proximal Hypospadias in Otherwise Healthy Child at Mymensingh Medical College Hospital 迈门辛医学院附属医院对其他健康儿童尿道下裂近端一期修复术的效果
Asian Journal of Pediatric Research Pub Date : 2024-07-15 DOI: 10.9734/ajpr/2024/v14i7373
Md. Sakhawat Hossain, Sangul Islam, Mohammad Billal Hossain, Asish Saha Roy, Zia Uddin Ahmed
{"title":"The Outcome of a Single Stage Repair for Proximal Hypospadias in Otherwise Healthy Child at Mymensingh Medical College Hospital","authors":"Md. Sakhawat Hossain, Sangul Islam, Mohammad Billal Hossain, Asish Saha Roy, Zia Uddin Ahmed","doi":"10.9734/ajpr/2024/v14i7373","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7373","url":null,"abstract":"Hypospadias is a birth defect that involves the development of a penile urethra that opens on the ventral side of the penis. Proximal hypospadias is one of the most severe forms of hypospadias that is generally associated with chordee. Hypospadias surgery, especially in the proximal location, is challenging, and many operations have been proposed, including one-stage and two-stage operations. \u0000Objectives: The aim of the study was to evaluate the success rate and safety of a single-stage surgery in children with proximal hypospadias and to determine the complications. \u0000Materials and Methods: This interventional study was conducted on forty patients, aged between one and twelve years, who had proximal hypospadias and were admitted to Mymensingh Medical College Hospital between January 2014 and April 2015. A one-step operation with tabularized incised plate urethroplasty (TIPU) was done. Patients’ demographic data, intraoperative details, and postoperative outcomes were recorded within 3 months after the operation. \u0000Results: The common age at surgery was 2-4 years, and they were 40% of the total patients. There was Haematoma in 5% of cases, a devitalized skin flap in 7.5% of cases, and stent blockage in 10% of cases during the immediate postoperative period. In our 3-month follow-up, all the patients had conical glans and cylindrical penises. Meatal stenosis and narrow stream were noted in 5 percent of patients but were relieved with meatal dilatation. Urethrocutaneous fistulas remained patients in 7.5% of cases. \u0000Conclusion: TIPU repair of proximal hypospadias is safe, effective, yields good results, and has low risks of complications in otherwise healthy children. It is a less risky and more viable method that helps to exclude extra steps in the process.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"18 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141648571","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}
引用次数: 0
Ohdo-Madokoro-Sonoda Syndrome with a De Novo MED12 Mutation 伴有新 MED12 基因突变的 Ohdo-Madokoro-Sonoda 综合征
Asian Journal of Pediatric Research Pub Date : 2024-07-13 DOI: 10.9734/ajpr/2024/v14i7372
H. Baidi, A. Radi, A. Ourrai, A. Hassani, R. Abilkassem
{"title":"Ohdo-Madokoro-Sonoda Syndrome with a De Novo MED12 Mutation","authors":"H. Baidi, A. Radi, A. Ourrai, A. Hassani, R. Abilkassem","doi":"10.9734/ajpr/2024/v14i7372","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7372","url":null,"abstract":"Ohdo syndrome is extremely rare and comprises a heterogeneous group of disorders characterized by intellectual disability (ID) and typical facial features, including blepharophimosis, ptosis, dental hypoplasia, hearing impairment and intellectual disability. So far, fewer than 30 patients have been reported with Ohdo syndrome, with a prevalence of 1/1 000 000. Most reported cases are sporadic, except the original cases of ohdo who described two affected sisters and a first cousin, suggesting autosomal recessive inheritance. Autosomal dominant, X-linked- and mithochondrial inheritance have also been suggested.  \u0000We report the case of a 5-year-old girl, born to healthy, non-consanguineous parents, with no other family members known to be affected by a similar disorder. She exhibited significant dysmorphic facial features, including blepharophimosis, dental hypoplasia, hypertélorism, rétrognatism, microcephaly, trigonocéphaly, microphthalmia, nasolabial furrow, badly hemmed ear, and a pointed palate.  These features were associated with psychomotor and growth retardation of less than 2 Standard deviations. Using whole exome sequencing (WES), we discovered the variant NM_005120.3 (MED12):c.6352C>T(p.Gin2118Ter)  in the heterozygous state  and its absence in the parents, confirming the de novo nature of this variant, which is compatible with the diagnosis of ohdo syndrome due to a heterozygous mutation of the MED 12.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"45 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141652353","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}
引用次数: 0
Low-grade Gliomas in Children 儿童低级别胶质瘤
Asian Journal of Pediatric Research Pub Date : 2024-07-12 DOI: 10.9734/ajpr/2024/v14i7370
Stefan Bittmann
{"title":"Low-grade Gliomas in Children","authors":"Stefan Bittmann","doi":"10.9734/ajpr/2024/v14i7370","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7370","url":null,"abstract":"Low-grade gliomas (LGG) are primary tumors of the central nervous system, originating from malignant transformation of cells in the brain or spinal cord. They are distinct from metastatic cancers that spread to the CNS from other parts of the body. While low-grade gliomas can occur throughout the nervous system, they are most commonly found in the cerebellum and central regions of the cerebrum, including the optic pathway and hypothalamic-pituitary axis. The growth of low-grade gliomas varies, with some tumors growing slowly and remaining localized, while others may progress rapidly and aggressively. Due to the limited space within the skull, the growth of a tumor can potentially damage critical areas of the brain, posing a risk to the patient's life. Although the risk of metastasis through the cerebrospinal fluid is generally low, children diagnosed with optic pathway gliomas at a young age may have a slightly increased risk of developing metastases. Research focus on new targets to treat this rare group of brain cancer in childhood.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"48 22","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141654611","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}
引用次数: 0
Transgenerational Effects of Maternal Health 孕产妇健康的跨代影响
Asian Journal of Pediatric Research Pub Date : 2024-07-12 DOI: 10.9734/ajpr/2024/v14i7371
Imomoemi Nwinam Thompson, Adetomi Bademosi, G. Ezeh, Sandra Ujunwa Ekesiobi
{"title":"Transgenerational Effects of Maternal Health","authors":"Imomoemi Nwinam Thompson, Adetomi Bademosi, G. Ezeh, Sandra Ujunwa Ekesiobi","doi":"10.9734/ajpr/2024/v14i7371","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7371","url":null,"abstract":"This study highlights the profound impact of maternal health on offspring well-being through transgenerational effects, extending beyond direct genetic inheritance. It emphasizes epigenetic modifications, intrauterine environment, and maternal lifestyle factors, influencing fetal programming and health trajectories across generations. Understanding these mechanisms is crucial for developing targeted interventions to prevent intergenerational disease transmission and promote lifelong health. The seminar explores these complexities, examining mechanisms, implications for offspring health, and the role of environmental and socioeconomic factors. Current research underscores the importance of maternal health in shaping offspring outcomes, revealing associations with chronic diseases, neurodevelopmental disorders, and metabolic disorders. Epidemiological evidence supports these findings, emphasizing the need for interventions addressing health disparities and promoting health equity. Future research priorities include elucidating underlying mechanisms, integrating multi-omics approaches, and addressing societal and environmental determinants. Early intervention strategies, preventive measures, and policy recommendations are vital for mitigating adverse effects and improving maternal and offspring health outcomes, thereby breaking the cycle of intergenerational disease transmission and promoting health equity across generations.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"11 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141655434","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}
引用次数: 0
Relationship among Socio-economic Determinants and Non-compliance with Vaccination in Children under 6 Years of Age in Guanajuato, México: A Cross-sectional Study 墨西哥瓜纳华托州 6 岁以下儿童的社会经济决定因素与不遵守疫苗接种规定之间的关系:横断面研究
Asian Journal of Pediatric Research Pub Date : 2024-07-04 DOI: 10.9734/ajpr/2024/v14i7369
Edith Fernanda Villanueva-Méndez, Michelle Arias-Morales, Luis Ignacio Pérez-Velázquez, Gilberto Flores-Vargas, M. D. J. Gallardo-Luna, E. Navarro-Olivos, Ligia Gricelda Arce-Padilla, Ma. Dolores Mabel Salgado-Hernández, Laura Elena Escalera-Morales, Erick Olav Duran-Arredondo, Juan Carlos González-Araiza, Nicolas Padilla-Raygoza
{"title":"Relationship among Socio-economic Determinants and Non-compliance with Vaccination in Children under 6 Years of Age in Guanajuato, México: A Cross-sectional Study","authors":"Edith Fernanda Villanueva-Méndez, Michelle Arias-Morales, Luis Ignacio Pérez-Velázquez, Gilberto Flores-Vargas, M. D. J. Gallardo-Luna, E. Navarro-Olivos, Ligia Gricelda Arce-Padilla, Ma. Dolores Mabel Salgado-Hernández, Laura Elena Escalera-Morales, Erick Olav Duran-Arredondo, Juan Carlos González-Araiza, Nicolas Padilla-Raygoza","doi":"10.9734/ajpr/2024/v14i7369","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7369","url":null,"abstract":"Vaccination is a quick and effective way to protect the population against harmful diseases before coming into contact with pathogens in the external environment. One-third of deaths worldwide are caused by infectious diseases caused by viruses, bacteria, or parasites. Over the years, the planet has experienced different epidemics and pandemics such as the Black Death, smallpox, measles, cholera, influenza, and the most recent SARS-CoV-2; these diseases have claimed the lives of many people. \u0000Aims: Determine the social and economic factors that influence the non-compliance with the vaccination schedule at a primary health center in Guanajuato, Mexico. \u0000Study Design:  It is a cross-sectional, analytical, quantitative and observational study. \u0000Place and Duration of Study: CAISES Pardo Guanajuato from March to May 2024 \u0000Methodology: The sample were 200 surveys of parents of pediatric patients under 6 years of age at CAISES El Pardo in Guanajuato, Mexico. \u0000200 vaccination cards of children under 6 years old were reviewed to determine whether the vaccination schedule was complete or incomplete. Subsequently, a survey was conducted with the guardians or parents of the children to observe their stance on vaccines, their knowledge, and interest in vaccination. \u0000The survey had a construct validity and an intra-observer reliability of Kappa 0.87, and th e inter-observer Kappa was 0.81. \u0000Results: Mothers in free union, as marital status do have the highest rate of non-compliance with their children's vaccination schedule. Although the marital status of the mother did prove to be an important factor in compliance with the vaccination schedule, various other factors affecting the child's vaccination schedule were identified. \u0000Conclusion: This research shows the main causes why mothers do not vaccinate their children in a timely manner, is availability of vaccination center and distance from home yo vaccination center. The socio-economic determinants did not show effect in incomplete vaccination card.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":" 21","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141679194","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}
引用次数: 0
Ebstein Anomaly in an Adolescent: A ‘Miranda Warning’ against Blaming Sickle Cell Cardiomyopathy: A Case-Based Scholarly Update 青少年艾布斯坦异常:禁止指责镰状细胞心肌病的 "米兰达警告":基于病例的学术更新
Asian Journal of Pediatric Research Pub Date : 2024-06-13 DOI: 10.9734/ajpr/2024/v14i7362
Wala Si, Abubakar Ig, Ibrahim Ba, Jelani Ni, Farouk AG.
{"title":"Ebstein Anomaly in an Adolescent: A ‘Miranda Warning’ against Blaming Sickle Cell Cardiomyopathy: A Case-Based Scholarly Update","authors":"Wala Si, Abubakar Ig, Ibrahim Ba, Jelani Ni, Farouk AG.","doi":"10.9734/ajpr/2024/v14i7362","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7362","url":null,"abstract":"Background: Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve associated with atrialisation of the right ventricle. The defect arises from failure of the normal process by which the tricuspid valve is separated from the right ventricular myocardium. Most cases are diagnosed in childhood, but asymptomatic ones may remain undiagnosed until adulthood. \u0000Case Summary: We present a rare case of EA diagnosed for the first time in a 13-year-old female sickle cell anaemia patient when she developed biventricular heart failure with severe tricuspid regurgitation and biventricular thrombi which was managed medically; however, patient died 17 days into admission. \u0000Conclusion: The Ebstein anomaly can presents in adolescents for the first time and is usually associated with pericardial effusion and ventricular thrombi. Due to its similarity in presentation to sickle cell cardiomyopathy, it can be missed in sickle cell anaemia patients. Echocardiography can help unravel this diagnostic dilemma.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"48 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141349583","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}
引用次数: 0
Comparative Outcomes of Propranolol Monotherapy and Combined Therapy with Intralesional Triamcinolone in Infantile Hemangioma 婴儿血管瘤中普萘洛尔单药治疗与曲安奈德内注射联合治疗的疗效比较
Asian Journal of Pediatric Research Pub Date : 2024-06-10 DOI: 10.9734/ajpr/2024/v14i7361
Shabnam Rashedi, Md. Tawhidul Islam, Manash Talukdar, Emon Kanti Moharer, Dibendu Kumar Das
{"title":"Comparative Outcomes of Propranolol Monotherapy and Combined Therapy with Intralesional Triamcinolone in Infantile Hemangioma","authors":"Shabnam Rashedi, Md. Tawhidul Islam, Manash Talukdar, Emon Kanti Moharer, Dibendu Kumar Das","doi":"10.9734/ajpr/2024/v14i7361","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7361","url":null,"abstract":"Background: Infantile Hemangiomas (IHs) are the most common vascular tumors of infancy. Oral propranolol has achieved great success in treating IHs since 2008. Recently combined oral propranolol with intralesional injection of Triamcinolone acetonide is the effective method of treatment for infantile Hemangioma with minimal adverse effects. \u0000Objectives: To observe the outcome of Propranolol monotherapy and combined therapy with intralesional Triamcinolone in Infantile Hemangioma. \u0000Materials and Methods: This prospective study was carried out in the Department of Pediatric Surgery, Sylhet MAG Osmani Medical College Hospital, Sylhet, during the period from January 2018 to December 2019. It included 42 infantile hemangioma patients divided into two groups: Group A (21) treated with oral propranolol, and Group B (21) treated with oral propranolol plus intralesional triamcinolone acetonide. Patients were followed up at regular intervals for 6 months. \u0000Results: The data of two groups of patients with IH were compared in the study. The demographic variables, including median age, sex, size, type, and site of IH, were comparable between the two groups. This showed that pre-treatment complications were slightly higher in Group B but not significantly different. Superior size reduction was observed in Group B (71.4% vs. 38.1%), though the difference was not significant statistically. Group B had excellent color regression, which was significantly higher compared to the other group (90.5% vs. 9.5%, p < 0.001). The mean treatment cost was higher in Group B, which was 187.86. taka compared to 124.76. taka in Group A (P value was <0.001). In summary, Group B had better color regression of the pinprick but at a higher cost of treatment as compared to Group A, and other parameters were almost equal in both groups. \u0000Conclusion: Combined propranolol and intralesional Triamcinolone is more effective compared to propranolol alone in the treatment of IHs.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":" 53","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141365085","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}
引用次数: 0
Selective Phosphodiesterase 4D (PDE4D) Allosteric Inhibitors for the Treatment of FMR-1 Gene Defects and other Pediatric Syndromal Disorders Associated with Cognitive Impairment 选择性磷酸二酯酶 4D (PDE4D) 异构体抑制剂用于治疗 FMR-1 基因缺陷和其他与认知障碍相关的儿科综合症
Asian Journal of Pediatric Research Pub Date : 2024-06-07 DOI: 10.9734/ajpr/2024/v14i7359
S. Ma
{"title":"Selective Phosphodiesterase 4D (PDE4D) Allosteric Inhibitors for the Treatment of FMR-1 Gene Defects and other Pediatric Syndromal Disorders Associated with Cognitive Impairment","authors":"S. Ma","doi":"10.9734/ajpr/2024/v14i7359","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i7359","url":null,"abstract":"The selective phosphodiesterase 4 D enzymes play a crucial role in regulating signaling through the cyclic adenosinmonophosphate second messenger system by hydrolyzing cyclic nucleotides. The PDE4 gene family includes four subtypes, PDE4A-D, distinguished by the presence of conserved regions called upstream conserved regions UCR1 and UCR2. These enzymes can exist as dimers or monomers, with UCR1 facilitating dimerization and UCR2 controlling enzyme activity by modulating access to cAMP. Dimeric isoforms exhibit increased activity in response to cAMP signaling through PKA-mediated phosphorylation of UCR1. Mutations in the PDE4D gene have been linked to the rare neurodevelopmental disorder acrodysostosis-2 (ACRDYS2), characterized by intellectual disability and brachydactyly. These mutations, predominantly missense mutations on the protein surface, disrupt protein kinase A phosphorylation sites or alter interactions between UCR2 and the catalytic domain, affecting enzyme activity. Some mutations at the dimerization site increase basal enzyme activity. Genetic variations in PDE4D also influence human cognitive abilities, as evidenced by GWAS studies linking allelic variation in the gene's 5' exons encoding dimeric forms to cognitive function. This highlights the significance of dimeric PDE4D isoforms in normal brain function, both in rare disorders like ACRDYS2 and in common genetic variants associated with cognitive abilities. Blocking PDE4D can boost signaling through the cAMP-PKA-SIRT1-Akt-Bcl-2/Bax pathway, potentially offering therapeutic advantages in neurocognitive disorders. We focus on the role of selective phosphodiesterase 4D (PDE4D) allosteric inhibitors for the treatment of fragile X mental retardation protein (FMR-1) gene defects and other brain disorders in childhood with focus on spinal cord injury in childhood, Down syndrome, Angelman syndrome, Rett syndrome and Prader Willi syndrome.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":" 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141370864","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}
引用次数: 0
To Determine the Accuracy and Reliability of Broselow Tape in Comparison with Actual Weight and Advance Pediatric Life Support Formula Weight Assessment among the Pediatric Population Presenting to Emergency Department of a Low Income Setting 在低收入地区急诊科就诊的儿科患儿中,确定布罗塞洛胶带与实际体重和高级儿科生命支持公式体重评估的准确性和可靠性比较
Asian Journal of Pediatric Research Pub Date : 2024-05-24 DOI: 10.9734/ajpr/2024/v14i6356
Emad Uddin Siddiqui, Ghazala Irfan Kazi, Mehreen Thayani, Ahmed Raheem, Tooba Siddiqui
{"title":"To Determine the Accuracy and Reliability of Broselow Tape in Comparison with Actual Weight and Advance Pediatric Life Support Formula Weight Assessment among the Pediatric Population Presenting to Emergency Department of a Low Income Setting","authors":"Emad Uddin Siddiqui, Ghazala Irfan Kazi, Mehreen Thayani, Ahmed Raheem, Tooba Siddiqui","doi":"10.9734/ajpr/2024/v14i6356","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i6356","url":null,"abstract":"Background: Near precise weight assessment among sick pediatric patients remains a dilemma and conventionally accepted weight assessment methods, in busy ED might be inaccurate or unreliable. Children have different weights at different ages, and accurate/precise weight measurement is of utmost importance for weight-related dose calculation of drugs/fluids, equipment sizes, an accurate dose of electrical currents during cardiac shock, etc. \u0000Several weight estimation methods are available and are dependent on the child's age, length, or both. However, length or length-and-age-based methods may have greater accuracy than merely age-based, still precise weight measurement while children are recumbent (length) has its own challenges. \u0000Objective: We determined the accuracy and reliability of BT by comparing it with actual weight and advanced pediatric life support formula (APLS) among the pediatric population presenting to the emergency department. \u0000Methods: This was a single-center, cross-sectional study design. This study was conducted at pediatric emergency of an urban tertiary care hospital after ethics committee approval and written consent from parents/caregivers during July 2021- June 22. \u0000Pediatric patients aged 1 month to 12 years, weight 3-36 kg, and height 46.5-142.5 cm on BT were included. Actual weight was measured on a standard weighing machine. We use Broselow pediatric emergency tape (2017 edition), APLS formula was also used to measure the estimated weight by using the age provided by parents. \u0000Descriptive analysis, mean and standard deviation were calculated, frequencies and percentages were calculated for categorical variables. Cronbach’s alpha and Passing-Bablok regression analysis was applied to assess the reliability and identify systematic biases between actual body weights with estimated BT. Bland–Altman analysis was also performed to measure the precision, accuracy, and bias. \u0000Results: 250 children were included with equal gender distribution and were divided in to three categories as per the weight estimation by BT in to <10 kg (n=58, 23.2%), 10-18 kg (n=151, 60.4%) and >18 kg (n=41, 16.4%). The mean age was 5.26 (±2.37) years, majority of children were below 5 years of age (n=144, 58%). \u0000Positive agreement between BT weight with actual weight and other formulas in weight category of <10 kg, however as weight increases from 10 kg, onward results are not significant. \u0000Passing and Bablok Regression analysis showed a positive correlation between the estimated and actual (AW) body weight (r=0.9280, p<0.001) and accuracy (r2=0.929), and the accuracy of BT weight decreases with the increasing weight of children.  \u0000Similarly, 95% agreement limit and mean biased was 0.465 to 1.113 and 0.789±2.602 between BT and APLS, BT with AW was -0.50 to -0.28 and -0.39±0.885. Comparing and correlating weight assessment of APLS formula vs LF and TF didn't show significance with a p-value of 0.041 and 0.034 respectively. \u0000Bland-Altman plot between ","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"89 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141101004","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}
引用次数: 0
Current Pathophysiological and Therapeutic Options for Children with Hutchinson Gilford Syndrome 哈钦森-吉尔福特综合征患儿目前的病理生理学和治疗方案
Asian Journal of Pediatric Research Pub Date : 2024-05-24 DOI: 10.9734/ajpr/2024/v14i6355
Stefan Bittmann
{"title":"Current Pathophysiological and Therapeutic Options for Children with Hutchinson Gilford Syndrome","authors":"Stefan Bittmann","doi":"10.9734/ajpr/2024/v14i6355","DOIUrl":"https://doi.org/10.9734/ajpr/2024/v14i6355","url":null,"abstract":"Hutchinson-Gilford-Progeria syndrome (HGPS) cannot, to date, be treated causally. Therapy for affected children focus on alleviating the symptoms, treating secondary diseases and preventing complications such as strokes or heart attacks. Various medications and physiotherapeutic methods are primarily available for this extremely rare pediatric genetic disease. Lonafarnib, a farnesyltransferase inhibitor, has been used for the treatment of progeria in children since 2022, which can extend the life of children with HGPS up to 4 years. Farnesyltransferase inhibitors are able to block an enzyme that is involved in progerin processing. Progerin is the altered protein that occurs in HGPS due to the spelling mistake in the lamin A gene and accumulates within the cell nucleus envelope. As a result, the envelope is weakened and the cell nucleus becomes deformed. The spectrum of therapies includes progerin-targeting strategies on one hand and on therapies to alleviate the tremendous effects by progerin. Research focus on different new targets in the management of HGPS-like the farnesyltransferase inhibitor lonafarnib, Acetyltransferase NAT10-inhibitors, KAT 6a/b and -7 inhibitors, paclitaxel, small molecule ICMT-inhibitors, exportin CRM-1 Inhibitors, progerin-lamin A binding inhibitors (Progerinin), Ghrelin, micro-RNA inhibitors, doxycycline and the regulation of rapamycin complex 1 (mTORC1). This manuscript analyses these new therapeutic targets and pathophysiological aspects in a review manuscript.","PeriodicalId":393364,"journal":{"name":"Asian Journal of Pediatric Research","volume":"10 31","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141098808","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}
引用次数: 0
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