O. Pamukcu, S. Sunkak, A. Tuncay, G. Erkan, A. Baykan, N. Narin
Devices may be used for special purposes different than their production purpose. For instance, Amplatzer Ductal Occluder is actually designed for duct closure and its usage for closing defects other than ductus is named as off-label. The aim of this study is to emphasize off-label use of device: not only for PDA and VSD but also for other various defects. This study is designed retrospectively, performed by the evaluation catheterization records of patients in whom ADO II and ADO II-AS devices were used in Erciyes University Medical Faculty Children Hospital, Pediatric Cardiology Department between 2011 and 2018. Patients’ demographic criteria: age, weight at the time of procedure was gathered. The diagnosis, size of device, follow-up period and complications were also noted. From April 2011 to March 2018, a total of 121 patients underwent transcatheter closure by ADO II and 66 patients by ADO II AS. The number of PDA closure with ADO II was 48; with ADO II AS were 62. Rest of the procedures were all off-label. Types of off-label procedures performed were: VSD closure, residual mitral cleft closure, Aorta-Right atrium tunnel closure, pulmonary arteriovenous fistula occlusion, aorta-pulmonary window closure, and occlusion of the artery feeding accessory lobe in scimitar syndrome, Gerbode defect occlusion. Up to our knowledge; this study includes the largest pediatric case series with various different congenital heart defects which were closed with ADO-II. Also our ADO-II occluded VSD case series is one of the largest series in the literature with almost 6 years’ follow-up. We believe in that ADO-II device may be an alternative in percutaneous closure of various rare heart defects. It is used successfully for non-ductal defects with low complication and high compliance rates.
设备可以用于不同于其生产目的的特殊用途。例如，Amplatzer Ductal Occluder实际上是为管道关闭而设计的，它用于关闭管道以外的缺陷被命名为标签外。本研究的目的是强调设备的标签外使用:不仅用于PDA和VSD，还用于其他各种缺陷。本研究采用回顾性设计，通过评估2011年至2018年在埃尔西耶斯大学医学院儿童医院儿童心脏科使用ADO II和ADO II- as装置的患者的置管记录进行研究。收集患者的人口统计标准:手术时的年龄、体重。同时记录诊断、器械大小、随访时间及并发症。2011年4月至2018年3月，共有121例患者采用ADO II关闭导管，66例患者采用ADO II AS关闭导管。用ADO II闭合PDA的病例为48例;ADOⅱAS为62例。其余的程序都是标签外的。执行的非适应症手术类型包括:室间隔缺损关闭、残留二尖瓣裂关闭、主动脉-右心房隧道关闭、肺动静脉瘘关闭、主动脉-肺窗关闭、刀状综合征副叶供血动脉闭塞、Gerbode缺损闭塞。据我们所知;本研究包括了用ADO-II缝合的各种不同先天性心脏缺陷的最大儿科病例系列。此外，我们的ADO-II闭塞性室间隔病例系列是文献中最大的系列之一，随访时间近6年。我们相信，ADO-II装置可能是经皮缝合各种罕见心脏缺损的替代方法。它成功地用于非导管缺陷，并发症低，顺应率高。
{"title":"Off-label Use of ADO II® in the Closure of Various Congenital Heart Defects","authors":"O. Pamukcu, S. Sunkak, A. Tuncay, G. Erkan, A. Baykan, N. Narin","doi":"10.51271/jpea-2022-188","DOIUrl":"https://doi.org/10.51271/jpea-2022-188","url":null,"abstract":"Devices may be used for special purposes different than their production purpose. For instance, Amplatzer Ductal Occluder is actually designed for duct closure and its usage for closing defects other than ductus is named as off-label. The aim of this study is to emphasize off-label use of device: not only for PDA and VSD but also for other various defects. This study is designed retrospectively, performed by the evaluation catheterization records of patients in whom ADO II and ADO II-AS devices were used in Erciyes University Medical Faculty Children Hospital, Pediatric Cardiology Department between 2011 and 2018. Patients’ demographic criteria: age, weight at the time of procedure was gathered. The diagnosis, size of device, follow-up period and complications were also noted. From April 2011 to March 2018, a total of 121 patients underwent transcatheter closure by ADO II and 66 patients by ADO II AS. The number of PDA closure with ADO II was 48; with ADO II AS were 62. Rest of the procedures were all off-label. Types of off-label procedures performed were: VSD closure, residual mitral cleft closure, Aorta-Right atrium tunnel closure, pulmonary arteriovenous fistula occlusion, aorta-pulmonary window closure, and occlusion of the artery feeding accessory lobe in scimitar syndrome, Gerbode defect occlusion. Up to our knowledge; this study includes the largest pediatric case series with various different congenital heart defects which were closed with ADO-II. Also our ADO-II occluded VSD case series is one of the largest series in the literature with almost 6 years’ follow-up. We believe in that ADO-II device may be an alternative in percutaneous closure of various rare heart defects. It is used successfully for non-ductal defects with low complication and high compliance rates.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115844412","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}
S. Asadova, A. Kısaarslan, Sümeyra Özdemir Çiçek, Nihal Şahin, Sema Nur Taşkın, Şeyda Doğantan, M. Poyrazoğlu
Introduction:It is essential to evaluate the activation and the articular and extra-articular damage during the Juvenile Idiopathic Arthritis(JIA) disease course. Objectives:This study aimed to evaluate the damage status and affecting factors in JIA patients. Methods:Juvenile Arthritis Damage Index articular(JADI-A) and extra-articular(E) were evaluated in 204 JIA patients who had been followed up for two years andmore. JADI-A and E affecting factors were assessed by univariate and multivariate logistic regression analysis. Results: In this study,127(62.6%) of the patients were female. The median age was 13(IQR: 11-16), and the age at diagnosis was 7(IQR: 4-10) years. The median follow-up time was 5(IQR: 4-8) years. Ninety-two(45.3%) patients had comorbid diseases. JADI-A were median:0(min-max: 0-24), JADI-E were median:0(min-max:0-4).The annual attacks number [OR:1,759 (CI:1,300-2,379],p:<0,001),annual eritrocyte sedimantation rate (ESR) [OR:1,072(CI:1,021-1,125),p:0.005] were effective on JADI-A scores. The CRP at the first admission [OR:1.007(CI: 1,000-1,014), p:0.037], the annual ESR[OR:1,051(CI:1,008-1,095),p:0.019] were found to be effective on the JADI-E. The ideal cut-off point of the attacks number and ESR affecting JADI-A scores were 1.38[AUC:0.734(0.641-0.828),p:0.001] and 14.32[AUC:0.617(0.514-0.721),p:0.027], respectively. The ideal cut-off point of the CRP and ESR affecting JADI-E scores were 13,25[AUC:0,662(0,541-0,782),p:0,009],and15,10[AUC:0.674(0.567-0.780),p:0.002], respectively. Steroid related complications such as, obesity in 12 (5.9%), hirsutism in 3 (1.5%), transient adrenal suppression in 14 (6.9%), 8 (3.9%), and osteoporosis were detectedin 7 (3.4%) patients. Conclusion: We have shown that parameters used routinely can be helpful to predict damage. We also think that new criteria should be added to the scoring.
{"title":"Assesment of Damage in Juvenile Idiopathic Arthritis: Single Center Experience","authors":"S. Asadova, A. Kısaarslan, Sümeyra Özdemir Çiçek, Nihal Şahin, Sema Nur Taşkın, Şeyda Doğantan, M. Poyrazoğlu","doi":"10.51271/jpea-2022-169","DOIUrl":"https://doi.org/10.51271/jpea-2022-169","url":null,"abstract":"Introduction:It is essential to evaluate the activation and the articular and extra-articular damage during the Juvenile Idiopathic Arthritis(JIA) disease course. \u0000Objectives:This study aimed to evaluate the damage status and affecting factors in JIA patients. \u0000Methods:Juvenile Arthritis Damage Index articular(JADI-A) and extra-articular(E) were evaluated in 204 JIA patients who had been followed up for two years andmore. JADI-A and E affecting factors were assessed by univariate and multivariate logistic regression analysis. \u0000Results: In this study,127(62.6%) of the patients were female. The median age was 13(IQR: 11-16), and the age at diagnosis was 7(IQR: 4-10) years. The median follow-up time was 5(IQR: 4-8) years. Ninety-two(45.3%) patients had comorbid diseases. JADI-A were median:0(min-max: 0-24), JADI-E were median:0(min-max:0-4).The annual attacks number [OR:1,759 (CI:1,300-2,379],p:<0,001),annual eritrocyte sedimantation rate (ESR) [OR:1,072(CI:1,021-1,125),p:0.005] were effective on JADI-A scores. The CRP at the first admission [OR:1.007(CI: 1,000-1,014), p:0.037], the annual ESR[OR:1,051(CI:1,008-1,095),p:0.019] were found to be effective on the JADI-E. The ideal cut-off point of the attacks number and ESR affecting JADI-A scores were 1.38[AUC:0.734(0.641-0.828),p:0.001] and 14.32[AUC:0.617(0.514-0.721),p:0.027], respectively. The ideal cut-off point of the CRP and ESR affecting JADI-E scores were 13,25[AUC:0,662(0,541-0,782),p:0,009],and15,10[AUC:0.674(0.567-0.780),p:0.002], respectively. Steroid related complications such as, obesity in 12 (5.9%), hirsutism in 3 (1.5%), transient adrenal suppression in 14 (6.9%), 8 (3.9%), and osteoporosis were detectedin 7 (3.4%) patients. \u0000Conclusion: We have shown that parameters used routinely can be helpful to predict damage. We also think that new criteria should be added to the scoring.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"86 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121082023","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}
Madelung's deformity (MD) occurs as a result of premature closure of the medial and volar aspects of the distal radial physis.1 It is more frequent and severe in girls, and usually develops in middle/late childhood.2 MD is one of the most characteristic features of he short-stature homeobox gene (SHOX) deficiency, which causes short stature3. Radial bowing is one of the well-known radiological futures. On the other hand, there are three typical radiological sign of the hand radiograph for SHOX deficiency; triangularization, pyramidalization of the os lunatum, and radiolucency at the distal radius4. In the evaluation of a 9-year-old girl who was investigated for precocious puberty, her height measurement was 18th percentile. On the wrist X-ray taken for the determination of the bone age of the patient, there was an appearance compatible with MD (Figure 1). In the genetic studies of the patient with MD, normal female karyotyping (46, XX) was demonstrated by Trypsin G banding Technique. Heterozygous SHOX deletion was detected by Fluorescence In Situ Hybridization technique using a probe specific to the SHOX gene region (Xp22.33). Interpreting the direct X-ray is important in recognizing the MD. Thus, it will be easier to detect SHOX gene deletion in the etiology of short stature patients with this deformity.
{"title":"Wrist radiography for hand bone age tells a lot; a girl with SHOX deficiency","authors":"Hüsnü Maraşlı, N. Çelik, Yeşim Sidar Duman","doi":"10.51271/jpea-2022-194","DOIUrl":"https://doi.org/10.51271/jpea-2022-194","url":null,"abstract":"Madelung's deformity (MD) occurs as a result of premature closure of the medial and volar aspects of the distal radial physis.1 It is more frequent and severe in girls, and usually develops in middle/late childhood.2 MD is one of the most characteristic features of he short-stature homeobox gene (SHOX) deficiency, which causes short stature3. Radial bowing is one of the well-known radiological futures. On the other hand, there are three typical radiological sign of the hand radiograph for SHOX deficiency; triangularization, pyramidalization of the os lunatum, and radiolucency at the distal radius4. \u0000 In the evaluation of a 9-year-old girl who was investigated for precocious puberty, her height measurement was 18th percentile. On the wrist X-ray taken for the determination of the bone age of the patient, there was an appearance compatible with MD (Figure 1). In the genetic studies of the patient with MD, normal female karyotyping (46, XX) was demonstrated by Trypsin G banding Technique. Heterozygous SHOX deletion was detected by Fluorescence In Situ Hybridization technique using a probe specific to the SHOX gene region (Xp22.33). \u0000 Interpreting the direct X-ray is important in recognizing the MD. Thus, it will be easier to detect SHOX gene deletion in the etiology of short stature patients with this deformity.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"68 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121327666","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}
The most common type of nephrotic syndrome in children is minimal change disease (MCD), which is usually responsive to steroid therapy. Steroid dependency is one of the handicaps in the management of these children. Thus, the early prediction of the disease course may improve treatment strategy. Demographic characteristics and laboratory parameters of 35 patients at the time of MCD diagnosis were retrospectively obtained from the hospital records. There were 23 (65%) patients with steroid sensitive (SSNS) and 12 (35%) with steroid dependent nephrotic syndrome (SDNS). There was a significant difference between the patients with SSNS and SDNS in terms of age at diagnosis, remission time, and mean values of platelet volume, low density lipoprotein cholesterol, uric acid, urine protein-to-creatinine ratio, total cholesterol and creatinine (p = 0.003, p < 0.001, p = 0.013, p = 0.006, p = 0.036, p = 0.02, p = 0.003, and p = 0.034, respectively). The prediction of early markers of steroid dependency can reduce the side effects of steroids and facilitate the use of appropriate drugs.
{"title":"Predictive Parameters of Steroid Dependency in Minimal Change Disease","authors":"Esra Nagehan Akyol Önder, P. Ertan","doi":"10.51271/jpea-2022-185","DOIUrl":"https://doi.org/10.51271/jpea-2022-185","url":null,"abstract":"The most common type of nephrotic syndrome in children is minimal change disease (MCD), which is usually responsive to steroid therapy. Steroid dependency is one of the handicaps in the management of these children. Thus, the early prediction of the disease course may improve treatment strategy. Demographic characteristics and laboratory parameters of 35 patients at the time of MCD diagnosis were retrospectively obtained from the hospital records. There were 23 (65%) patients with steroid sensitive (SSNS) and 12 (35%) with steroid dependent nephrotic syndrome (SDNS). There was a significant difference between the patients with SSNS and SDNS in terms of age at diagnosis, remission time, and mean values of platelet volume, low density lipoprotein cholesterol, uric acid, urine protein-to-creatinine ratio, total cholesterol and creatinine (p = 0.003, p < 0.001, p = 0.013, p = 0.006, p = 0.036, p = 0.02, p = 0.003, and p = 0.034, respectively). The prediction of early markers of steroid dependency can reduce the side effects of steroids and facilitate the use of appropriate drugs.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122301600","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}
M. Tekin, R. Düşünsel, Nihal Şahin, I. Dursun, B. Sözeri, S. Çiçek, A. Kısaarslan, M. Poyrazoğlu
Abstract Introduction and Objectives: Juvenile idiopathic arthritis (JIA) is a frequently seen chronic rheumatoid disease in childhood, which may cause disability and severely affect quality of life (QoL). The aim of present study was to assess relationships between disease activation and socio-cultural status of family, QoL, anxiety level, and depression level in patients with JIA and their parents. Methods: The study included 100 patients with JIA. The socio-demographic data were obtained from all patients. Child- and parent-reported PedsQL, Beck depression inventory (BDI), Kovacs' Child Depression Inventory (CDI), SCARED child version, CHAQ discomfort and disability scales were applied and JADAS-27 score was calculated in a cross-sectional manner. Then, we compared the characteristics of patients with the scales’ results. Results: JADAS-27, BDI, and CHAQ discomfort scores were higher and child- and parent-reported PedsQL scores were lower in patients with active disease than patients on remission (p<0.05). The SCARED score was higher in girls than boys. The CHAQ disability score was high in children aged 8-12 years (p<0.05). JADAS-27 and CHAQ disability scores were significantly low in patients with better compliance to treatment. Parental statements about changes in mental health after diagnosis were consistent with results of depression and anxiety scales of children. Conclusions: Quality of life is adversely affected in children with JIA, which may result in depression and anxiety. In management of JIA, one of our goals should be maintaining QoL. Further comprehensive studies in relationships between QoL and depression, anxiety, socio-demographic parameters, disease activation and social circle of patient are needed.
{"title":"Assessment of Factor Affecting the Quality of Life in Children with Juvenile Idiopathic Arthritis","authors":"M. Tekin, R. Düşünsel, Nihal Şahin, I. Dursun, B. Sözeri, S. Çiçek, A. Kısaarslan, M. Poyrazoğlu","doi":"10.51271/jpea-2022-163","DOIUrl":"https://doi.org/10.51271/jpea-2022-163","url":null,"abstract":"Abstract \u0000Introduction and Objectives: Juvenile idiopathic arthritis (JIA) is a frequently seen chronic rheumatoid disease in childhood, which may cause disability and severely affect quality of life (QoL). The aim of present study was to assess relationships between disease activation and socio-cultural status of family, QoL, anxiety level, and depression level in patients with JIA and their parents. \u0000Methods: The study included 100 patients with JIA. The socio-demographic data were obtained from all patients. Child- and parent-reported PedsQL, Beck depression inventory (BDI), Kovacs' Child Depression Inventory (CDI), SCARED child version, CHAQ discomfort and disability scales were applied and JADAS-27 score was calculated in a cross-sectional manner. Then, we compared the characteristics of patients with the scales’ results. \u0000Results: JADAS-27, BDI, and CHAQ discomfort scores were higher and child- and parent-reported PedsQL scores were lower in patients with active disease than patients on remission (p<0.05). The SCARED score was higher in girls than boys. The CHAQ disability score was high in children aged 8-12 years (p<0.05). JADAS-27 and CHAQ disability scores were significantly low in patients with better compliance to treatment. Parental statements about changes in mental health after diagnosis were consistent with results of depression and anxiety scales of children. \u0000Conclusions: Quality of life is adversely affected in children with JIA, which may result in depression and anxiety. In management of JIA, one of our goals should be maintaining QoL. Further comprehensive studies in relationships between QoL and depression, anxiety, socio-demographic parameters, disease activation and social circle of patient are needed.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"57 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127051839","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}
Hypoxic ischemic encephalopathy (HIE) is an important cause of mortality and morbidity in newborns. Our study aimed to determine the neurodevelopmental outcomes, risk factors, and the relationship between risk factors and prognosis of cases followed up with HIE diagnosis and who were treated with hypothermia treatment. Medical records of 23 patients who were followed up with HIE diagnosis in the pediatric neurology outpatient clinic between January 1, 2018, and December 31, 2021, and treated with therapeutic hypothermia in the newborn period were retrospectively reviewed. Denver Developmental Screening Test II was used in the developmental evaluation. Neurodevelopment was normal in 12 (52.2%) of 23 cases and retarded for age in 11 (47.8%) cases. Variables such as Sarnat encephalopathy score in the neonatal period, need for prolonged ventilation, presence of severe acidosis at birth, Apgar scores at the 1st and 5th minutes, and abnormal cranial magnetic resonance (Mrg) findings were determined to be major factors in determining neurodevelopmental prognosis in asphyxic cases. The male gender was found to be riskier in terms of prognosis. Of the patients with HIE, retardation was determined for fine motor skills in 52.2%, speaking in 39.1%, gross motor skills in 26.1%, and personal development in 21.7%. Epilepsy, cerebral palsy, and speech disorders were the most common chronic period problems in patients with HIE. Despite therapeutic hypothermia treatment, neurodevelopmental problems are still observed in newborns with HIE. We found that male gender, low Apgar score, severe acidosis, and abnormal cranial Mrg findings in the neonatal period are risk factors in determining the neurodevelopmental prognosis of newborns with HIE. Monitoring and supporting the development of HIE cases with risk factors from the first months of life and intervening in developmental problems promptly are crucial in order to improve long-term outcomes.
{"title":"Evaluation and Neurodevelopmental Outcomes of Infants with Hypoxic Ischemic Encephalopathy Treated with Therapeutic Hypothermia: A Single Center Experience","authors":"Berna Saygın Hekimoğlu, Beril Dilber, Filiz Aktürk Acar, Handan Bezirganoğlu, İ. Eyüboğlu, Gülnur Esenülkü","doi":"10.51271/jpea-2022-183","DOIUrl":"https://doi.org/10.51271/jpea-2022-183","url":null,"abstract":"Hypoxic ischemic encephalopathy (HIE) is an important cause of mortality and morbidity in newborns. Our study aimed to determine the neurodevelopmental outcomes, risk factors, and the relationship between risk factors and prognosis of cases followed up with HIE diagnosis and who were treated with hypothermia treatment. Medical records of 23 patients who were followed up with HIE diagnosis in the pediatric neurology outpatient clinic between January 1, 2018, and December 31, 2021, and treated with therapeutic hypothermia in the newborn period were retrospectively reviewed. Denver Developmental Screening Test II was used in the developmental evaluation. Neurodevelopment was normal in 12 (52.2%) of 23 cases and retarded for age in 11 (47.8%) cases. Variables such as Sarnat encephalopathy score in the neonatal period, need for prolonged ventilation, presence of severe acidosis at birth, Apgar scores at the 1st and 5th minutes, and abnormal cranial magnetic resonance (Mrg) findings were determined to be major factors in determining neurodevelopmental prognosis in asphyxic cases. The male gender was found to be riskier in terms of prognosis. Of the patients with HIE, retardation was determined for fine motor skills in 52.2%, speaking in 39.1%, gross motor skills in 26.1%, and personal development in 21.7%. Epilepsy, cerebral palsy, and speech disorders were the most common chronic period problems in patients with HIE. Despite therapeutic hypothermia treatment, neurodevelopmental problems are still observed in newborns with HIE. We found that male gender, low Apgar score, severe acidosis, and abnormal cranial Mrg findings in the neonatal period are risk factors in determining the neurodevelopmental prognosis of newborns with HIE. Monitoring and supporting the development of HIE cases with risk factors from the first months of life and intervening in developmental problems promptly are crucial in order to improve long-term outcomes.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"49 1-2","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"120920027","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}
Abstract Hereditary angioedema (HAE) is a rare, mostly inherited disorder however 25% of patients have de novo mutations. Although it’s rarity, it can be life threatening due to laryngeal involvement. Along with understanding the basis of swelling, several new treatment options aside from C1-inhibitory protein (C1-INH) replacement have been developed and are avaible on the markets. However the avaibility of approved drugs for attacks of HAE varies world wide. Treatment management requires angioedema attacks treatment, pre-procedural treatment and long term prophylaxis (LTP). C1-INH which was firstly developed and approved for on-demand treatment, pre-procedural treatment and LTP by iv route, nowadays for LTP, other developed and approved options are used by orally and sc route. Despite the new developing medications, permanent treatment such as gene therapy is needed.
{"title":"Hereditary Angioedema: Diagnosis, Management, Current State of Art and Advances","authors":"Esra Karabiber, Elif Karakoç Aydıner","doi":"10.51271/jpea-2022-164","DOIUrl":"https://doi.org/10.51271/jpea-2022-164","url":null,"abstract":"Abstract \u0000Hereditary angioedema (HAE) is a rare, mostly inherited disorder however 25% of patients have de novo mutations. Although it’s rarity, it can be life threatening due to laryngeal involvement. Along with understanding the basis of swelling, several new treatment options aside from C1-inhibitory protein (C1-INH) replacement have been developed and are avaible on the markets. However the avaibility of approved drugs for attacks of HAE varies world wide. Treatment management requires angioedema attacks treatment, pre-procedural treatment and long term prophylaxis (LTP). C1-INH which was firstly developed and approved for on-demand treatment, pre-procedural treatment and LTP by iv route, nowadays for LTP, other developed and approved options are used by orally and sc route. Despite the new developing medications, permanent treatment such as gene therapy is needed.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128565917","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}
Z. Ekici Tekin, M. Kaplan, M. C. Polat, E. Çelikel, Vildan Güngörer, T. Kurt, Nilüfer Tekgöz, Müge Sezer, Cüneyt Karagöl, Serkan Coşkun, Nimet Öner, Banu Çelikel Acae
Methotrexate that has a safe and inexpensive profile is a first choice of juvenile idiopathic arthritis treatment. Nevertheless, gastrointestinal symptoms are not serious but common side effects of methotrexate. Sometimes patients may have nausea by thinking methotrexate and even refuse methotrexate use. In this study, we aimed to define prevalence of methotrexate side effects in juvenile idiopathic arthritis and the possible risk factors for methotrexate intolerance. Methotrexate intolerance severity score showed severity of gastrointestinal symptoms. Eighty-seven patients accepted to join study and answered the questions. The prevalence of adverse events of methotrexate was 64.4% and the rate of gastrointestinal symptoms was 55.2%. The nausea (27.6%) was the most common gastrointestinal symptom. The median of methotrexate intolerance severity score was 14.5 (interquartile range: 10-18). However, there was no significant difference in main parameters between tolerant and intolerant groups and no risk factor was observed for methotrexate intolerance. As a conclusion, methotrexate is an important actor for juvenile idiopathic arthritis treatment as a first and safe drug and, prospective studies in larger cohorts is necessary to understand and prevent occurrence of gastrointestinal symptoms.
{"title":"Prevalence of methotrexate intolerance in juvenile idiopathic arthritis and possible risk factors: a tertiary center experience","authors":"Z. Ekici Tekin, M. Kaplan, M. C. Polat, E. Çelikel, Vildan Güngörer, T. Kurt, Nilüfer Tekgöz, Müge Sezer, Cüneyt Karagöl, Serkan Coşkun, Nimet Öner, Banu Çelikel Acae","doi":"10.51271/jpea-2022-184","DOIUrl":"https://doi.org/10.51271/jpea-2022-184","url":null,"abstract":"Methotrexate that has a safe and inexpensive profile is a first choice of juvenile idiopathic arthritis treatment. Nevertheless, gastrointestinal symptoms are not serious but common side effects of methotrexate. Sometimes patients may have nausea by thinking methotrexate and even refuse methotrexate use. In this study, we aimed to define prevalence of methotrexate side effects in juvenile idiopathic arthritis and the possible risk factors for methotrexate intolerance. Methotrexate intolerance severity score showed severity of gastrointestinal symptoms. Eighty-seven patients accepted to join study and answered the questions. The prevalence of adverse events of methotrexate was 64.4% and the rate of gastrointestinal symptoms was 55.2%. The nausea (27.6%) was the most common gastrointestinal symptom. The median of methotrexate intolerance severity score was 14.5 (interquartile range: 10-18). However, there was no significant difference in main parameters between tolerant and intolerant groups and no risk factor was observed for methotrexate intolerance. As a conclusion, methotrexate is an important actor for juvenile idiopathic arthritis treatment as a first and safe drug and, prospective studies in larger cohorts is necessary to understand and prevent occurrence of gastrointestinal symptoms.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"61 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126175192","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":"Pollakiuria due to Constipation in a Girl: LUTS or PEDUF?","authors":"S. Kurtoğlu","doi":"10.51271/jpea-2022-191","DOIUrl":"https://doi.org/10.51271/jpea-2022-191","url":null,"abstract":"<jats:p>N/A</jats:p>","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"96 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123066328","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}
A. Yilmaz, Pembe Soylu Üstünkoyuncu, I. Dursun, Z. Gunduz
The purpose of the study was to determine the effect of maternal glucocorticoids on experimental growth retardedrats and the effect of maternal undernutrition in different gestation periods for function of the kidney. This studyhad two sections. In the first section, 5 groups were formed. 10g/d diet was given in the 1st, 2nd, and 3rd trimesterand throughout the pregnancy period. The control group was fed a normal diet. In the second section, 3 groupsformed and all the rats take 10 g/d diet throughout pregnancy period. To determine the effect of endogenousglucocorticoids first group was treated with metyrapone, second group metyrapone+dexamethasone and theplacebo injected saline until 14 days of pregnancy. The offsprings body and kidney weights were detected inthe 0, 3rd and 20th weeks of age. Renal extraction functions and blood pressures from tail detected in the 20thweek of age. Urinary excretion and glomerular filtration rate were low in rats that had dietary restriction in thelast trimester. The glomerular filtration rates were found to be low in the group that had diet restriction duringthe whole pregnancy. Blood pressure values were found to be lower in the group that had diet restriction duringtheir pregnancy compared to the control group. Kidney weights were similar in all groups in the first phase. Itwas observed that renal excretion functions were preserved in the group receiving metyrapone treatment, butthere was no statistically significant difference between the results. Low blood pressures were normalized withmetyrapone treatment. The kidney sizes at the 20th week of the rats which receiving metyrapone treatment werefound to be smaller than those receiving physiological saline solution. Food restriction destroys renal functionsbut no effects with high blood pressure in adulthood. Glucocortiod exposure in pregnancy may reduce renaldevelopment.
{"title":"Evaluation of The Renal Function in The Intrauterine Growth Restricted Rats and The Effect of Maternal Glucocorticoids","authors":"A. Yilmaz, Pembe Soylu Üstünkoyuncu, I. Dursun, Z. Gunduz","doi":"10.51271/jpea-2022-155","DOIUrl":"https://doi.org/10.51271/jpea-2022-155","url":null,"abstract":"The purpose of the study was to determine the effect of maternal glucocorticoids on experimental growth retardedrats and the effect of maternal undernutrition in different gestation periods for function of the kidney. This studyhad two sections. In the first section, 5 groups were formed. 10g/d diet was given in the 1st, 2nd, and 3rd trimesterand throughout the pregnancy period. The control group was fed a normal diet. In the second section, 3 groupsformed and all the rats take 10 g/d diet throughout pregnancy period. To determine the effect of endogenousglucocorticoids first group was treated with metyrapone, second group metyrapone+dexamethasone and theplacebo injected saline until 14 days of pregnancy. The offsprings body and kidney weights were detected inthe 0, 3rd and 20th weeks of age. Renal extraction functions and blood pressures from tail detected in the 20thweek of age. Urinary excretion and glomerular filtration rate were low in rats that had dietary restriction in thelast trimester. The glomerular filtration rates were found to be low in the group that had diet restriction duringthe whole pregnancy. Blood pressure values were found to be lower in the group that had diet restriction duringtheir pregnancy compared to the control group. Kidney weights were similar in all groups in the first phase. Itwas observed that renal excretion functions were preserved in the group receiving metyrapone treatment, butthere was no statistically significant difference between the results. Low blood pressures were normalized withmetyrapone treatment. The kidney sizes at the 20th week of the rats which receiving metyrapone treatment werefound to be smaller than those receiving physiological saline solution. Food restriction destroys renal functionsbut no effects with high blood pressure in adulthood. Glucocortiod exposure in pregnancy may reduce renaldevelopment.","PeriodicalId":118905,"journal":{"name":"The Journal of Pediatric Academy","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114423307","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}
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