Pub Date : 2023-01-01DOI: 10.24953/turkjped.2020.3365
Noor Mohammad Noori, Maryam Nakhaei-Moghadam, Alireza Teimouri
Background: Aortic elasticity is a predictor and recognized factor for future cardiovascular events in children. The aim of the study was to evaluate the aortic stiffness in obese and overweight children compared to healthy ones.
Methods: The study evaluated 98 sex matched children aged 4 to 16 years that were equally distributed in asymptomatic obese or overweight and healthy children groups. All the participants were free of any heart diseases. Arterial stiffness indices were determined using two-dimensional echocardiography.
Results: The mean ages in the obese and healthy children were 10.40±2.50 years and 10.06±1.53 years, respectively. Aortic strain was significantly higher in obese children (20.70±5.04%), compared to healthy (7.06±3.77%) and overweight children (18.59±8.08%, p < 0.001). Aortic distensibility (AD) was significantly higher in obese children (0.010±0.005 cm < sup > 2 < /sup > dyn-1x10-6), compared to healthy (0.0036±0.004 cm < sup > 2 < /sup > dyn-1x10 < sup > -6 < /sup > ) and overweight children (0.009±0.005 cm < sup > 2 < /sup > dyn-1x10 < sup > -6 < /sup > , p < 0.001). Aortic strain beta (ASβ) index, was significantly higher in healthy children (9.26±6.17). Pressure-strain elastic modulus (PSEM) was significantly higher in healthy children (7.52±4.76 kPa). Systolic blood pressure increased with body mass index (BMI) significantly (p < 0.001) but diastolic blood pressure did not change (p=0.143). BMI had significant effect on arterial stiffness (AS) (β=0.732, p < 0.001), AD (β=0.636, p < 0.001), ASβ index (β=-0.573, p < 0.001) and PSEM (β=-0.578, p < 0.001). Age had significant effect on systolic diameter of the aorta (β=0.340, p < 0.001) and diastolic diameter of the aorta (β=0.407, p < 0.001).
Conclusions: We concluded that aortic strain and aortic distensibility increased in obese children when aortic strain beta index and PSEM decreased. This result suggests that, as atrial stiffness is a predictor for future heart diseases, dietary treatment for children with overweight or obese status is important.
背景:主动脉弹性是儿童未来心血管事件的预测因子和公认因素。这项研究的目的是评估肥胖和超重儿童与健康儿童相比的主动脉僵硬程度。方法:研究评估了98名性别匹配的4 ~ 16岁儿童,这些儿童平均分布在无症状肥胖或超重儿童组和健康儿童组。所有的参与者都没有心脏疾病。采用二维超声心动图测定动脉僵硬指数。结果:肥胖儿童和健康儿童的平均年龄分别为10.40±2.50岁和10.06±1.53岁。肥胖儿童主动脉压力(20.70±5.04%)高于健康儿童(7.06±3.77%)和超重儿童(18.59±8.08%,p < 0.001)。肥胖儿童的主动脉膨胀性(AD)(0.010±0.005 cm < sup > 2 < /sup > dyn-1x10-6)显著高于健康儿童(0.0036±0.004 cm < sup > 2 < /sup > dyn-1x10 < sup > -6 < /sup >)和超重儿童(0.009±0.005 cm < sup > 2 < /sup > dyn-1x10 < sup > -6, p < 0.001)。正常儿童主动脉应变β (ASβ)指数(9.26±6.17)明显高于正常儿童。正常儿童压力-应变弹性模量(PSEM)显著高于正常儿童(7.52±4.76 kPa)。收缩压随体重指数(BMI)显著升高(p < 0.001),舒张压无变化(p=0.143)。BMI对动脉硬度(AS) (β=0.732, p < 0.001)、AD (β=0.636, p < 0.001)、ASβ指数(β=-0.573, p < 0.001)和PSEM (β=-0.578, p < 0.001)均有显著影响。年龄对主动脉收缩期内径(β=0.340, p < 0.001)和舒张期内径(β=0.407, p < 0.001)有显著影响。结论:肥胖儿童主动脉应变β指数和主动脉PSEM降低时,主动脉应变和主动脉扩张性增加。这一结果表明,由于心房僵硬是未来心脏病的一个预测指标,对于超重或肥胖儿童的饮食治疗是重要的。
{"title":"Assessment of aortic elasticity parameters in obese and overweight children.","authors":"Noor Mohammad Noori, Maryam Nakhaei-Moghadam, Alireza Teimouri","doi":"10.24953/turkjped.2020.3365","DOIUrl":"https://doi.org/10.24953/turkjped.2020.3365","url":null,"abstract":"<p><strong>Background: </strong>Aortic elasticity is a predictor and recognized factor for future cardiovascular events in children. The aim of the study was to evaluate the aortic stiffness in obese and overweight children compared to healthy ones.</p><p><strong>Methods: </strong>The study evaluated 98 sex matched children aged 4 to 16 years that were equally distributed in asymptomatic obese or overweight and healthy children groups. All the participants were free of any heart diseases. Arterial stiffness indices were determined using two-dimensional echocardiography.</p><p><strong>Results: </strong>The mean ages in the obese and healthy children were 10.40±2.50 years and 10.06±1.53 years, respectively. Aortic strain was significantly higher in obese children (20.70±5.04%), compared to healthy (7.06±3.77%) and overweight children (18.59±8.08%, p < 0.001). Aortic distensibility (AD) was significantly higher in obese children (0.010±0.005 cm < sup > 2 < /sup > dyn-1x10-6), compared to healthy (0.0036±0.004 cm < sup > 2 < /sup > dyn-1x10 < sup > -6 < /sup > ) and overweight children (0.009±0.005 cm < sup > 2 < /sup > dyn-1x10 < sup > -6 < /sup > , p < 0.001). Aortic strain beta (ASβ) index, was significantly higher in healthy children (9.26±6.17). Pressure-strain elastic modulus (PSEM) was significantly higher in healthy children (7.52±4.76 kPa). Systolic blood pressure increased with body mass index (BMI) significantly (p < 0.001) but diastolic blood pressure did not change (p=0.143). BMI had significant effect on arterial stiffness (AS) (β=0.732, p < 0.001), AD (β=0.636, p < 0.001), ASβ index (β=-0.573, p < 0.001) and PSEM (β=-0.578, p < 0.001). Age had significant effect on systolic diameter of the aorta (β=0.340, p < 0.001) and diastolic diameter of the aorta (β=0.407, p < 0.001).</p><p><strong>Conclusions: </strong>We concluded that aortic strain and aortic distensibility increased in obese children when aortic strain beta index and PSEM decreased. This result suggests that, as atrial stiffness is a predictor for future heart diseases, dietary treatment for children with overweight or obese status is important.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 1","pages":"46-53"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9084057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2022.304
Melek Büyükeren
Background: To investigate the relationship between neonatal urine bisphenol A (BPA) levels and the prevalence and prognosis of transient tachypnea of the newborn (TTN).
Methods: This prospective study was conducted between January and April 2020 in the Neonatal Intensive Care Unit (NICU) of Gaziantep Cengiz Gökçek Obstetrics and Pediatric Hospital. The study group consisted of patients diagnosed with TTN and the control group was made up of healthy neonates housed together with their mothers. Urine samples were collected from the neonates within the first 6 hours postnatally.
Results: Urine BPA levels and urine BPA/creatinine levels were statistically higher in the TTN group (P < 0.005). The receiver operating characteristic (ROC) curve analysis determined the cut-off value of urine BPA for TTN to be 1.18 µg/L (95% confidence interval [CI]: 0.667-0.889, sensitivity: 78.1%, and specificity: 51.5%) and the cut-off value of urine BPA/creatinine to be 2.65 µg/g (95% CI: 0.727-0.930, sensitivity: 84.4%, and specificity: 66.7%). Furthermore, the ROC analysis indicated that the cut-off value of BPA for neonates requiring invasive respiratory support was 15.64 µg/L (95% CI: 0.568-1.000, sensitivity: 83.3%, and specificity: 96.2%) and the cut-off value for BPA/creatinine was 19.10 µg/g (95% CI: 0.777-1.000, sensitivity: 83.3%, and specificity: 84.6%) among the TTN patients.
Conclusions: BPA and BPA/creatinine values were higher in the urine of newborns diagnosed with TTN which is a fairly common cause of NICU hospitalization, in samples collected within the first 6 hours after birth, which may be a reflection of intrauterine factors.
{"title":"Comparison of urine bisphenol A levels in transient tachypnea of the newborn and healthy newborns.","authors":"Melek Büyükeren","doi":"10.24953/turkjped.2022.304","DOIUrl":"https://doi.org/10.24953/turkjped.2022.304","url":null,"abstract":"<p><strong>Background: </strong>To investigate the relationship between neonatal urine bisphenol A (BPA) levels and the prevalence and prognosis of transient tachypnea of the newborn (TTN).</p><p><strong>Methods: </strong>This prospective study was conducted between January and April 2020 in the Neonatal Intensive Care Unit (NICU) of Gaziantep Cengiz Gökçek Obstetrics and Pediatric Hospital. The study group consisted of patients diagnosed with TTN and the control group was made up of healthy neonates housed together with their mothers. Urine samples were collected from the neonates within the first 6 hours postnatally.</p><p><strong>Results: </strong>Urine BPA levels and urine BPA/creatinine levels were statistically higher in the TTN group (P < 0.005). The receiver operating characteristic (ROC) curve analysis determined the cut-off value of urine BPA for TTN to be 1.18 µg/L (95% confidence interval [CI]: 0.667-0.889, sensitivity: 78.1%, and specificity: 51.5%) and the cut-off value of urine BPA/creatinine to be 2.65 µg/g (95% CI: 0.727-0.930, sensitivity: 84.4%, and specificity: 66.7%). Furthermore, the ROC analysis indicated that the cut-off value of BPA for neonates requiring invasive respiratory support was 15.64 µg/L (95% CI: 0.568-1.000, sensitivity: 83.3%, and specificity: 96.2%) and the cut-off value for BPA/creatinine was 19.10 µg/g (95% CI: 0.777-1.000, sensitivity: 83.3%, and specificity: 84.6%) among the TTN patients.</p><p><strong>Conclusions: </strong>BPA and BPA/creatinine values were higher in the urine of newborns diagnosed with TTN which is a fairly common cause of NICU hospitalization, in samples collected within the first 6 hours after birth, which may be a reflection of intrauterine factors.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 1","pages":"35-45"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9084058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2021.5103
Rahmayani Rahmayani, Fernando Rumapea, Rodman Tarigan, Eddy Fadlyana, Meita Dhamayanti, Kusnandi Rusmil
Background: Height is an anthropometric measurement that serves as the most constant indicator of growth. In certain circumstances, arm span can be used as an alternative to height measurements. This study aims to analyze the correlation between anthropometric measurements of height and arm span in children aged 7-12 years.
Methods: A cross-sectional study was carried out from September to December 2019 in six elementary schools in Bandung. Children aged 7-12 years were recruited with a multistage cluster random sampling method. Children with scoliosis, contractures, and stunting were excluded from the study. Height and arm span were measured by two pediatricians.
Results: A total of 1,114 children, comprising 596 boys and 518 girls, fulfilled the inclusion criteria. The ratio of height to arm span was 0.98-1.01. The regression equation used to predict height through measurement of arm span in male subjects was Height = 21.8623 + 0.7634 x Arm span (cm) + 0.0791 x age (month); R2 = 94%; standard error of estimate (SEE): 2.66 and that in female subjects was Height = 21.2395 + 0.7779 x Arm span (cm) + 0.0701 x age (month); R2 = 95.4%; SEE: 2.39. The predicted height and the average actual height were not significantly different. There is a strong correlation between height and arm span in children aged 7-12 years.
Conclusions: Arm span can be used to predict the actual height of children aged 7-12 years and as an alternative measurement for growth.
{"title":"Correlation between anthropometric measurements of height and arm span in Indonesian children aged 7-12 years: a cross-sectional study.","authors":"Rahmayani Rahmayani, Fernando Rumapea, Rodman Tarigan, Eddy Fadlyana, Meita Dhamayanti, Kusnandi Rusmil","doi":"10.24953/turkjped.2021.5103","DOIUrl":"https://doi.org/10.24953/turkjped.2021.5103","url":null,"abstract":"<p><strong>Background: </strong>Height is an anthropometric measurement that serves as the most constant indicator of growth. In certain circumstances, arm span can be used as an alternative to height measurements. This study aims to analyze the correlation between anthropometric measurements of height and arm span in children aged 7-12 years.</p><p><strong>Methods: </strong>A cross-sectional study was carried out from September to December 2019 in six elementary schools in Bandung. Children aged 7-12 years were recruited with a multistage cluster random sampling method. Children with scoliosis, contractures, and stunting were excluded from the study. Height and arm span were measured by two pediatricians.</p><p><strong>Results: </strong>A total of 1,114 children, comprising 596 boys and 518 girls, fulfilled the inclusion criteria. The ratio of height to arm span was 0.98-1.01. The regression equation used to predict height through measurement of arm span in male subjects was Height = 21.8623 + 0.7634 x Arm span (cm) + 0.0791 x age (month); R2 = 94%; standard error of estimate (SEE): 2.66 and that in female subjects was Height = 21.2395 + 0.7779 x Arm span (cm) + 0.0701 x age (month); R2 = 95.4%; SEE: 2.39. The predicted height and the average actual height were not significantly different. There is a strong correlation between height and arm span in children aged 7-12 years.</p><p><strong>Conclusions: </strong>Arm span can be used to predict the actual height of children aged 7-12 years and as an alternative measurement for growth.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 1","pages":"13-23"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9084059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2021.4574
Keun Soo Lee, Bo Lyun Lee
Background: This study evaluated the efficacy of a single dose of phenytoin/fosphenytoin (PHT) to control repetitive seizures in children with benign convulsions with mild gastroenteritis (CwG).
Methods: Children aged between 3 months and 5 years with CwG were retrospectively enrolled. Convulsions with mild gastroenteritis were defined as (a) seizures with acute gastroenteritis without fever or dehydration; (b) normal blood laboratory results; and (c) normal electroencephalography and brain imaging findings. Patients were divided into two groups according to whether or not intravenous PHT (10 mg/kg of phenytoin or phenytoin equivalents) was administered. Clinical manifestations and treatment efficacy were evaluated and compared.
Results: Ten of 41 children eligible for inclusion received PHT. Compared to children in the non-PHT group, those in the PHT group had a higher number of seizures (5.2 ± 2.3 vs. 1.6 ± 1.0, P < 0.001) and a lower serum sodium level (133.5 ± 3.2 mmol/L vs. 137.2 ± 2.6 mmol/L, P = 0.001). Initial serum sodium levels were negatively correlated with seizure frequency (r = -0.438, P = 0.004). In all patients, seizures were completely resolved with a single dose of PHT. There were no significant adverse effects from PHT.
Conclusions: A single dose of PHT can effectively treat CwG with repetitive seizures. The serum sodium channel may play a role in seizure severity.
背景:本研究评估了单剂量苯妥英/磷妥英(PHT)对轻度胃肠炎(CwG)患儿良性惊厥反复发作的控制效果。方法:对年龄在3个月至5岁的CwG患儿进行回顾性研究。痉挛伴轻度肠胃炎定义为(a)癫痫伴急性肠胃炎,无发热或脱水;(b)血液化验结果正常;(c)正常脑电图和脑成像结果。根据是否静脉注射PHT (10 mg/kg苯妥英或苯妥英当量)将患者分为两组。评价和比较两组患者的临床表现和治疗效果。结果:41名符合纳入条件的儿童中有10名接受了PHT治疗。与非PHT组相比,PHT组患儿癫痫发作次数较高(5.2±2.3次vs. 1.6±1.0次,P < 0.001),血清钠水平较低(133.5±3.2 mmol/L vs. 137.2±2.6 mmol/L, P = 0.001)。初始血清钠水平与癫痫发作频率呈负相关(r = -0.438, P = 0.004)。在所有患者中,单剂量PHT完全消除了癫痫发作。PHT没有明显的不良反应。结论:单剂量PHT可有效治疗CwG反复发作。血清钠通道可能在癫痫发作的严重程度中起作用。
{"title":"Efficacy of single dose of phenytoin/fosphenytoin in benign convulsions with mild gastroenteritis.","authors":"Keun Soo Lee, Bo Lyun Lee","doi":"10.24953/turkjped.2021.4574","DOIUrl":"https://doi.org/10.24953/turkjped.2021.4574","url":null,"abstract":"<p><strong>Background: </strong>This study evaluated the efficacy of a single dose of phenytoin/fosphenytoin (PHT) to control repetitive seizures in children with benign convulsions with mild gastroenteritis (CwG).</p><p><strong>Methods: </strong>Children aged between 3 months and 5 years with CwG were retrospectively enrolled. Convulsions with mild gastroenteritis were defined as (a) seizures with acute gastroenteritis without fever or dehydration; (b) normal blood laboratory results; and (c) normal electroencephalography and brain imaging findings. Patients were divided into two groups according to whether or not intravenous PHT (10 mg/kg of phenytoin or phenytoin equivalents) was administered. Clinical manifestations and treatment efficacy were evaluated and compared.</p><p><strong>Results: </strong>Ten of 41 children eligible for inclusion received PHT. Compared to children in the non-PHT group, those in the PHT group had a higher number of seizures (5.2 ± 2.3 vs. 1.6 ± 1.0, P < 0.001) and a lower serum sodium level (133.5 ± 3.2 mmol/L vs. 137.2 ± 2.6 mmol/L, P = 0.001). Initial serum sodium levels were negatively correlated with seizure frequency (r = -0.438, P = 0.004). In all patients, seizures were completely resolved with a single dose of PHT. There were no significant adverse effects from PHT.</p><p><strong>Conclusions: </strong>A single dose of PHT can effectively treat CwG with repetitive seizures. The serum sodium channel may play a role in seizure severity.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 1","pages":"109-117"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9084063","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2022.735
Gülşah Özdemir, Bora Gülhan, Eda Didem Kurt-Şükür, Emine Atayar, Raziye Atan, İsmail Dursun, Zeynep Birsin Özçakar, Seha Saygılı, Alper Soylu, Oğuz Söylemezoğlu, Alev Yılmaz, Aysun Karabay Bayazıt, Fehime Kara Eroğlu, Belde Kasap Demir, Selçuk Yüksel, Yılmaz Tabel, Ayşe Ağbaş, Ali Düzova, Mutlu Hayran, Fatih Özaltın, Rezan Topaloğlu
Background: Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition delays chronic kidney disease (CKD) while the effectiveness of immunosuppressive (IS) therapy in AS is still uncertain. In this study, we aimed to analyze the outcomes of pediatric patients with X-linked AS (XLAS) who received RAAS inhibitors and IS therapy.
Methods: Seventy-four children with XLAS were included in this multicenter study. Demographic features, clinical and laboratory data, treatments, histopathological examinations, and genetic analyses were analyzed retrospectively.
Results: Among 74 children, 52 (70.2%) received RAAS inhibitors, 11 (14.9%) received RAAS inhibitors and IS, and 11 (14.9%) were followed up without treatment. During follow-up, glomerular filtration rate (GFR) decreased < 60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (M/F=6/1). In male patients with XLAS, kidney survival was not different between RAAS and RAAS+IS groups (p=0.42). The rate of progression to CKD was significantly higher in patients with nephrotic range proteinuria and nephrotic syndrome (NS), respectively (p=0.006, p=0.05). The median age at the onset of RAAS inhibitors was significantly higher in male patients who progressed to CKD (13.9 vs 8.1 years, p=0.003).
Conclusions: RAAS inhibitors have beneficial effects on proteinuria and early initiation of therapy may delay the progression to CKD in children with XLAS. There was no significant difference between the RAAS and RAAS+IS groups in kidney survival. AS patients presenting with NS or nephrotic range proteinuria should be followed up more carefully considering the risk of early progression to CKD.
背景:Alport综合征(AS)以进行性肾脏疾病为特征。越来越多的证据表明肾素-血管紧张素-醛固酮系统(RAAS)抑制延缓慢性肾脏疾病(CKD),而免疫抑制(is)治疗AS的有效性仍不确定。在这项研究中,我们旨在分析儿童x连锁AS (XLAS)患者接受RAAS抑制剂和IS治疗的结果。方法:74例XLAS患儿纳入本多中心研究。回顾性分析患者的人口学特征、临床和实验室资料、治疗方法、组织病理学检查和遗传分析。结果:74例患儿中,52例(70.2%)接受RAAS抑制剂治疗,11例(14.9%)同时接受RAAS抑制剂和IS治疗,11例(14.9%)未接受治疗。随访期间,74例患者中有7例(9.5%)肾小球滤过率(GFR)降至< 60 ml/min/1.73 m2 (M/F=6/1)。在男性XLAS患者中,RAAS组和RAAS+IS组肾脏存活率无差异(p=0.42)。肾病范围蛋白尿和肾病综合征(NS)患者进展为CKD的比率分别显著高于前者(p=0.006, p=0.05)。在进展为CKD的男性患者中,RAAS抑制剂发病的中位年龄明显更高(13.9 vs 8.1岁,p=0.003)。结论:RAAS抑制剂对蛋白尿有有益作用,早期开始治疗可以延缓XLAS患儿CKD的进展。RAAS组与RAAS+IS组肾脏存活率无显著差异。考虑到早期发展为CKD的风险,伴有NS或肾病范围蛋白尿的AS患者应更仔细地随访。
{"title":"The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome.","authors":"Gülşah Özdemir, Bora Gülhan, Eda Didem Kurt-Şükür, Emine Atayar, Raziye Atan, İsmail Dursun, Zeynep Birsin Özçakar, Seha Saygılı, Alper Soylu, Oğuz Söylemezoğlu, Alev Yılmaz, Aysun Karabay Bayazıt, Fehime Kara Eroğlu, Belde Kasap Demir, Selçuk Yüksel, Yılmaz Tabel, Ayşe Ağbaş, Ali Düzova, Mutlu Hayran, Fatih Özaltın, Rezan Topaloğlu","doi":"10.24953/turkjped.2022.735","DOIUrl":"https://doi.org/10.24953/turkjped.2022.735","url":null,"abstract":"<p><strong>Background: </strong>Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition delays chronic kidney disease (CKD) while the effectiveness of immunosuppressive (IS) therapy in AS is still uncertain. In this study, we aimed to analyze the outcomes of pediatric patients with X-linked AS (XLAS) who received RAAS inhibitors and IS therapy.</p><p><strong>Methods: </strong>Seventy-four children with XLAS were included in this multicenter study. Demographic features, clinical and laboratory data, treatments, histopathological examinations, and genetic analyses were analyzed retrospectively.</p><p><strong>Results: </strong>Among 74 children, 52 (70.2%) received RAAS inhibitors, 11 (14.9%) received RAAS inhibitors and IS, and 11 (14.9%) were followed up without treatment. During follow-up, glomerular filtration rate (GFR) decreased < 60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (M/F=6/1). In male patients with XLAS, kidney survival was not different between RAAS and RAAS+IS groups (p=0.42). The rate of progression to CKD was significantly higher in patients with nephrotic range proteinuria and nephrotic syndrome (NS), respectively (p=0.006, p=0.05). The median age at the onset of RAAS inhibitors was significantly higher in male patients who progressed to CKD (13.9 vs 8.1 years, p=0.003).</p><p><strong>Conclusions: </strong>RAAS inhibitors have beneficial effects on proteinuria and early initiation of therapy may delay the progression to CKD in children with XLAS. There was no significant difference between the RAAS and RAAS+IS groups in kidney survival. AS patients presenting with NS or nephrotic range proteinuria should be followed up more carefully considering the risk of early progression to CKD.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 3","pages":"456-468"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10109680","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2023.122
Devrim Can Saraç, Serkan Metin, Belde Kasap Demir, Özge Altuğ Gücenmez, İsrafil İnanç, İdil Akar, Cennet Hanım Karakurt, Sena Kongur, Ece Pastutmaz, Deniz Bayraktar
Background: Fatigue is a common problem in pediatric rheumatic diseases and is associated with poor quality of life. However, no validated methods are available to measure fatigue in adolescents with familial Mediterranean fever (FMF). The aim of the study was to establish validity and reliability for the child self-report PedsQL Multidimensional Fatigue Scale (PedsQL-MFS) and to investigate the effects of physical characteristics, diseaserelated characteristics, sleep quality/duration, and the amount of physical activity on fatigue in adolescents with FMF.
Methods: Seventy-one adolescents with FMF (13-18 years) were included. Children were examined regarding physical- and disease-related characteristics and completed patient-reported outcome measures (PROMs) regarding sleep quality/duration, physical activity levels, and fatigue. PedsQL-MFS was re-completed within the following 7-14 days.
Results: PedsQL-MFS demonstrated excellent test-retest reliability (ICC in 95% CI: 0.877-0.958) and internal consistency (Cronbach`s α: 0.928). All items contributed to the total score (item-total correlation > 0.3). PedsQLMFS scores were significantly correlated to fatigue (r: -0.666, p < 0.001), physical activity (r: 0.373, p < 0.001), sleep quality (rs: 0.678, p < 0.001), and sleep duration (r: 0.473, p < 0.001). Being female, having attacks in the last six months, a sleep duration of less than seven hours, and engaging in less physical activity resulted in higher fatigue.
Conclusions: PedsQL-MFS seems to be feasible for assessing fatigue in adolescents with FMF. Sex, recent attacks, sleep, and physical activity should be taken into consideration in the fatigue management of patients with FMF.
背景:疲劳是儿童风湿性疾病的常见问题,并与生活质量差有关。然而,尚无有效的方法来测量患有家族性地中海热(FMF)的青少年的疲劳。本研究的目的是建立儿童自我报告PedsQL多维疲劳量表(PedsQL- mfs)的效度和信度,并探讨身体特征、疾病相关特征、睡眠质量/持续时间和身体活动量对FMF青少年疲劳的影响。方法:选取青少年FMF患者71例(13 ~ 18岁)。检查儿童的身体和疾病相关特征,并完成患者报告的关于睡眠质量/持续时间、身体活动水平和疲劳的结果测量(PROMs)。在接下来的7-14天内重新完成PedsQL-MFS。结果:PedsQL-MFS具有良好的重测信度(95% CI: 0.877 ~ 0.958)和内部一致性(Cronbach’s α: 0.928)。所有项目对总分都有贡献(项目-总相关性> 0.3)。PedsQLMFS评分与疲劳(r: -0.666, p < 0.001)、体力活动(r: 0.373, p < 0.001)、睡眠质量(r: 0.678, p < 0.001)、睡眠时间(r: 0.473, p < 0.001)显著相关。作为女性,在过去的六个月里有过发作,睡眠时间少于7小时,体育活动较少会导致更严重的疲劳。结论:PedsQL-MFS对于评估青少年FMF患者的疲劳似乎是可行的。在FMF患者的疲劳管理中,应考虑到性别、近期发作、睡眠和身体活动。
{"title":"Assessing fatigue and related factors in adolescents with familial Mediterranean fever (FMF): psychometric properties of the PedsQL Multidimensional Fatigue Scale.","authors":"Devrim Can Saraç, Serkan Metin, Belde Kasap Demir, Özge Altuğ Gücenmez, İsrafil İnanç, İdil Akar, Cennet Hanım Karakurt, Sena Kongur, Ece Pastutmaz, Deniz Bayraktar","doi":"10.24953/turkjped.2023.122","DOIUrl":"https://doi.org/10.24953/turkjped.2023.122","url":null,"abstract":"<p><strong>Background: </strong>Fatigue is a common problem in pediatric rheumatic diseases and is associated with poor quality of life. However, no validated methods are available to measure fatigue in adolescents with familial Mediterranean fever (FMF). The aim of the study was to establish validity and reliability for the child self-report PedsQL Multidimensional Fatigue Scale (PedsQL-MFS) and to investigate the effects of physical characteristics, diseaserelated characteristics, sleep quality/duration, and the amount of physical activity on fatigue in adolescents with FMF.</p><p><strong>Methods: </strong>Seventy-one adolescents with FMF (13-18 years) were included. Children were examined regarding physical- and disease-related characteristics and completed patient-reported outcome measures (PROMs) regarding sleep quality/duration, physical activity levels, and fatigue. PedsQL-MFS was re-completed within the following 7-14 days.</p><p><strong>Results: </strong>PedsQL-MFS demonstrated excellent test-retest reliability (ICC in 95% CI: 0.877-0.958) and internal consistency (Cronbach`s α: 0.928). All items contributed to the total score (item-total correlation > 0.3). PedsQLMFS scores were significantly correlated to fatigue (r: -0.666, p < 0.001), physical activity (r: 0.373, p < 0.001), sleep quality (rs: 0.678, p < 0.001), and sleep duration (r: 0.473, p < 0.001). Being female, having attacks in the last six months, a sleep duration of less than seven hours, and engaging in less physical activity resulted in higher fatigue.</p><p><strong>Conclusions: </strong>PedsQL-MFS seems to be feasible for assessing fatigue in adolescents with FMF. Sex, recent attacks, sleep, and physical activity should be taken into consideration in the fatigue management of patients with FMF.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 4","pages":"630-639"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10182120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2023.185
Ümit Ayşe Tandırcıoğlu, Özge Doğan, R Önder Günaydın, Şule Yiğit, H Tolga Çelik
Background: Babies with severe bronchopulmonary dysplasia (BPD) are discharged with the support of a home-type mechanical ventilator, after opening a tracheostomy. In addition, although rare, tracheostomy is required in the neonatal period in congenital airway malformations. Early tracheostomy is appropriate to prevent complications due to prolonged intubation. We aimed to find the appropriate time for tracheostomy by examining the tracheostomy opening and closing times, complications and demographic characteristics of the patients, who were hospitalized and underwent tracheostomy in our neonatal intensive care unit.
Methods: This retrospective study involved infants admitted to the neonatal intensive care unit between January 2014 and 2019 and discharged following tracheostomy. Information acquired from hospital data was enrolled. The protocol was registered with ClinicalTrials.gov identifier NCT04497740.
Results: Twenty-six neonates with median 27.5 weeks gestational age and birth weight 885 gr were enrolled in the study. The mean opening time for tracheostomy was 54 ± 24 days, and the postmenstrual age (PMA) was 36 ± 3 weeks. The mean time to closure of tracheostomy in newborns with a tracheostomy was 387 ± 164 days. The duration of accidental decannulation developed as an early complication in 8 patients was mean 11 ± 8 days. Aspiration pneumonia in 2, subglottic stenosis in 5, accidental decannulation in 2, suprastomal collapse in 7, tracheocutaneous fistula in 8 and granulation tissue in 2 patients were found to be late complications, which occurred within median 90 days.
Conclusions: If there is no evidence that breathing has improved and the patient is still using a mechanical ventilator at high pressures and high oxygen concentration, a tracheostomy placement should be considered within two months.
{"title":"Outcomes of newborns with tracheostomy: single center experience.","authors":"Ümit Ayşe Tandırcıoğlu, Özge Doğan, R Önder Günaydın, Şule Yiğit, H Tolga Çelik","doi":"10.24953/turkjped.2023.185","DOIUrl":"https://doi.org/10.24953/turkjped.2023.185","url":null,"abstract":"<p><strong>Background: </strong>Babies with severe bronchopulmonary dysplasia (BPD) are discharged with the support of a home-type mechanical ventilator, after opening a tracheostomy. In addition, although rare, tracheostomy is required in the neonatal period in congenital airway malformations. Early tracheostomy is appropriate to prevent complications due to prolonged intubation. We aimed to find the appropriate time for tracheostomy by examining the tracheostomy opening and closing times, complications and demographic characteristics of the patients, who were hospitalized and underwent tracheostomy in our neonatal intensive care unit.</p><p><strong>Methods: </strong>This retrospective study involved infants admitted to the neonatal intensive care unit between January 2014 and 2019 and discharged following tracheostomy. Information acquired from hospital data was enrolled. The protocol was registered with ClinicalTrials.gov identifier NCT04497740.</p><p><strong>Results: </strong>Twenty-six neonates with median 27.5 weeks gestational age and birth weight 885 gr were enrolled in the study. The mean opening time for tracheostomy was 54 ± 24 days, and the postmenstrual age (PMA) was 36 ± 3 weeks. The mean time to closure of tracheostomy in newborns with a tracheostomy was 387 ± 164 days. The duration of accidental decannulation developed as an early complication in 8 patients was mean 11 ± 8 days. Aspiration pneumonia in 2, subglottic stenosis in 5, accidental decannulation in 2, suprastomal collapse in 7, tracheocutaneous fistula in 8 and granulation tissue in 2 patients were found to be late complications, which occurred within median 90 days.</p><p><strong>Conclusions: </strong>If there is no evidence that breathing has improved and the patient is still using a mechanical ventilator at high pressures and high oxygen concentration, a tracheostomy placement should be considered within two months.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 4","pages":"555-561"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10182123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2022.1128
Penghong Xu, Jinsu Zhou, Yue Xiao, Hongjun Miao
Background: We aimed to analyze the levels and associations of SH2 domain-containing protein 1A (SH2D1A), immunoglobulins and T lymphocyte (TL) subsets in children with Epstein-Barr virus (EBV) infections.
Methods: Sixty children with EBV infections admitted from January 2019 to January 2022 were selected, including 29 cases of infectious mononucleosis (IM group) and 31 cases of chronic active EBV infections (CAEBV group). Another 42 healthy children undergoing physical examination in the same period were selected as a control group. Their changes in SH2D1A, immunoglobulins and TL subsets (CD3+, CD4+ and CD8+) were compared.
Results: The levels of CD3+, CD4+ and CD8+ in the IM group were higher than those of the control group (P < 0.05), while they were lower in the CAEBV group than those of the control and IM groups (P < 0.05). The levels of SH2D1A, signaling lymphocyte activation molecule (SLAM) and SLAM-associated protein (SAP) were significantly higher in the IM group than those in the control and CAEBV groups (P < 0.05). The CAEBV group had decreased protein expressions of SLAM and SAP compared with those of the IM group. SH2D1A was positively correlated with immunoglobulin A, immunoglobulin G and TL subsets (CD3+, CD4+ and CD8+) (P < 0.05).
Conclusions: Detecting SH2D1A, immunoglobulins and TLs contributes to the diagnosis and differentiation of IM and CAEBV.
{"title":"Association of SH2 domain-containing protein 1A, immunoglobulins and T lymphocyte subsets with Epstein- Barr virus infections.","authors":"Penghong Xu, Jinsu Zhou, Yue Xiao, Hongjun Miao","doi":"10.24953/turkjped.2022.1128","DOIUrl":"https://doi.org/10.24953/turkjped.2022.1128","url":null,"abstract":"<p><strong>Background: </strong>We aimed to analyze the levels and associations of SH2 domain-containing protein 1A (SH2D1A), immunoglobulins and T lymphocyte (TL) subsets in children with Epstein-Barr virus (EBV) infections.</p><p><strong>Methods: </strong>Sixty children with EBV infections admitted from January 2019 to January 2022 were selected, including 29 cases of infectious mononucleosis (IM group) and 31 cases of chronic active EBV infections (CAEBV group). Another 42 healthy children undergoing physical examination in the same period were selected as a control group. Their changes in SH2D1A, immunoglobulins and TL subsets (CD3+, CD4+ and CD8+) were compared.</p><p><strong>Results: </strong>The levels of CD3+, CD4+ and CD8+ in the IM group were higher than those of the control group (P < 0.05), while they were lower in the CAEBV group than those of the control and IM groups (P < 0.05). The levels of SH2D1A, signaling lymphocyte activation molecule (SLAM) and SLAM-associated protein (SAP) were significantly higher in the IM group than those in the control and CAEBV groups (P < 0.05). The CAEBV group had decreased protein expressions of SLAM and SAP compared with those of the IM group. SH2D1A was positively correlated with immunoglobulin A, immunoglobulin G and TL subsets (CD3+, CD4+ and CD8+) (P < 0.05).</p><p><strong>Conclusions: </strong>Detecting SH2D1A, immunoglobulins and TLs contributes to the diagnosis and differentiation of IM and CAEBV.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 4","pages":"603-610"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10182125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2023.622
Sinem Akgül, Zeynep Alev Özön
{"title":"Gender affirming care is an evidence-based approach and misinformation harms patients and clinicians.","authors":"Sinem Akgül, Zeynep Alev Özön","doi":"10.24953/turkjped.2023.622","DOIUrl":"https://doi.org/10.24953/turkjped.2023.622","url":null,"abstract":"<jats:p xml:lang=\"tr\" />","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 4","pages":"707-708"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10217618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.24953/turkjped.2022.1049
Alper Kahvecioğlu, Melis Gültekin, Ferah Yıldız
Background: For children with non-rhabdomyosarcoma soft tissue sarcomas, a risk-adapted treatment approach is generally used in order to minimize treatment-related morbidity and mortality in low-risk patients and maximize the benefit in high-risk patients. Our aim in this review is to discuss the prognostic factors, riskadapted treatment options and the details of radiotherapy.
Methods: The publications reached by searching the keywords `pediatric soft tissue sarcoma`, `nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)`, and `radiotherapy` in Pubmed database were evaluated in detail.
Results: Today, based on prospective COG-ARST0332 and EpSSG studies, a risk-adapted multimodal treatment approach has become the standard in pediatric NRSTS. According to them, adjuvant chemotherapy/ radiotherapy can be safely omitted in low-risk patients, while adjuvant chemotherapy/radiotherapy or both are recommended in intermediate and high-risk groups. Recent prospective studies for pediatric patients have reported excellent treatment outcomes with smaller radiotherapy fields and lower doses than adult series. The primary goal of surgery is maximal tumor resection with negative margins. In cases that are initially unresectable, neoadjuvant chemotherapy and radiotherapy should be considered.
Conclusions: A risk-adapted multimodal treatment approach is the standard in pediatric NRSTS . Surgery alone is sufficient in low-risk patients, and adjuvant therapies may safely be omitted. On the contrary, in intermediateand high-risk patients, adjuvant treatments should be applied to reduce recurrence rates. In unresectable patients, the chance of surgery increases with the neoadjuvant treatment approach and thus treatment results may improve. In the future, outcome might be improved with further clarification of molecular features and targeted therapies in such patients.
{"title":"Radiotherapy for pediatric non-rhabdomyosarcoma soft tissue sarcomas: a comprehensive review.","authors":"Alper Kahvecioğlu, Melis Gültekin, Ferah Yıldız","doi":"10.24953/turkjped.2022.1049","DOIUrl":"https://doi.org/10.24953/turkjped.2022.1049","url":null,"abstract":"<p><strong>Background: </strong>For children with non-rhabdomyosarcoma soft tissue sarcomas, a risk-adapted treatment approach is generally used in order to minimize treatment-related morbidity and mortality in low-risk patients and maximize the benefit in high-risk patients. Our aim in this review is to discuss the prognostic factors, riskadapted treatment options and the details of radiotherapy.</p><p><strong>Methods: </strong>The publications reached by searching the keywords `pediatric soft tissue sarcoma`, `nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)`, and `radiotherapy` in Pubmed database were evaluated in detail.</p><p><strong>Results: </strong>Today, based on prospective COG-ARST0332 and EpSSG studies, a risk-adapted multimodal treatment approach has become the standard in pediatric NRSTS. According to them, adjuvant chemotherapy/ radiotherapy can be safely omitted in low-risk patients, while adjuvant chemotherapy/radiotherapy or both are recommended in intermediate and high-risk groups. Recent prospective studies for pediatric patients have reported excellent treatment outcomes with smaller radiotherapy fields and lower doses than adult series. The primary goal of surgery is maximal tumor resection with negative margins. In cases that are initially unresectable, neoadjuvant chemotherapy and radiotherapy should be considered.</p><p><strong>Conclusions: </strong>A risk-adapted multimodal treatment approach is the standard in pediatric NRSTS . Surgery alone is sufficient in low-risk patients, and adjuvant therapies may safely be omitted. On the contrary, in intermediateand high-risk patients, adjuvant treatments should be applied to reduce recurrence rates. In unresectable patients, the chance of surgery increases with the neoadjuvant treatment approach and thus treatment results may improve. In the future, outcome might be improved with further clarification of molecular features and targeted therapies in such patients.</p>","PeriodicalId":49409,"journal":{"name":"Turkish Journal of Pediatrics","volume":"65 3","pages":"362-376"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10127432","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}