Hormone Research in Paediatrics最新文献

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Differential Methylation of CYP11B1 in Girls with High DHEAS Levels and Correlation with 11-Oxyandrogen Levels: A Pilot Study. 高 DHEAS 水平女孩体内 CYP11B1 的甲基化差异及与 11-氧基雄激素水平的相关性:一项试点研究。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-12-13 DOI: 10.1159/000542963
Fernando Rodríguez, Diana Ponce, José Patricio Miranda, José L Santos, Gordon B Cutler, Ana Pereira, Esteban Barnafi, Germán Iñiguez, Verónica Mericq
{"title":"Differential Methylation of CYP11B1 in Girls with High DHEAS Levels and Correlation with 11-Oxyandrogen Levels: A Pilot Study.","authors":"Fernando Rodríguez, Diana Ponce, José Patricio Miranda, José L Santos, Gordon B Cutler, Ana Pereira, Esteban Barnafi, Germán Iñiguez, Verónica Mericq","doi":"10.1159/000542963","DOIUrl":"10.1159/000542963","url":null,"abstract":"<p><strong>Introduction: </strong>Premature adrenarche in girls is defined biochemically by an increase in adrenal androgen (DHEAS) levels above the age-specific reference range before age 8 years. Recently, increased levels of 11-oxyandrogens have also been reported in girls with premature adrenarche. Epigenetic modifications, specifically CpG methylation, may affect gene expression and/or activity of steroidogenic enzymes during developmental changes in adrenal androgen secretion.</p><p><strong>Objective: </strong>The aim of the study was to determine whether circulating 11-oxyandrogen levels in post-menarcheal girls are associated with the methylation status of genes involved in 11-oxyandrogen steroidogenesis.</p><p><strong>Methods: </strong>Ninety-seven healthy girls followed since the age of 3 years were classified, according to DHEAS serum concentration at age 6-7 years, as normal DHEAS (<42 μg/dL [75th percentile for population]) or high DHEAS (≥42 μg/dL). At Tanner stage 2, the methylation status of CpG sites located in CYP11B1 and HSD11B2 genes was analyzed in genomic DNA from peripheral blood leukocytes by the melting curve analysis methylation assay. Eleven-oxyandrogen concentrations were assessed at 4 years post menarche.</p><p><strong>Results: </strong>Significantly lower methylation levels were detected in the CYP11B1 gene in girls with high versus normal serum DHEAS levels, with no differences found in HSD11B2 gene. Additionally, CYP11B1 methylation status correlated inversely with 11β-hydroxy-androstenedione and 11-ketotestosterone levels. Furthermore, CYP11B1 methylation in the full cohort correlated inversely with insulin concentration at Tanner 1 and with body mass index at Tanner stage 1 and 2.</p><p><strong>Conclusion: </strong>This pilot study proposes the hypothesis that a lower methylation of CYP11B1 may be a mechanism contributing to increased concentrations of 11-oxyandrogens in premature adrenarche and its associated metabolic risk.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-9"},"PeriodicalIF":2.6,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828306","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Regional Deprivation and Diabetic Ketoacidosis at Type 1 Diabetes Diagnosis in Children and Adolescents: International Comparison among 6 Countries. 地区贫困与儿童和青少年确诊 1 型糖尿病时的糖尿病酮症酸中毒:6 个国家的国际比较。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-12-12 DOI: 10.1159/000543139
G Todd Alonso, Christina Reinauer, Georgina M Williams, Rosaria Gesuita, Craig Jefferies, Klemen Dovc, Julia M Grimsmann, Taylor M Triolo, Ambika Shetty, Reinhard W Holl, Valentino Cherubini
{"title":"Regional Deprivation and Diabetic Ketoacidosis at Type 1 Diabetes Diagnosis in Children and Adolescents: International Comparison among 6 Countries.","authors":"G Todd Alonso, Christina Reinauer, Georgina M Williams, Rosaria Gesuita, Craig Jefferies, Klemen Dovc, Julia M Grimsmann, Taylor M Triolo, Ambika Shetty, Reinhard W Holl, Valentino Cherubini","doi":"10.1159/000543139","DOIUrl":"10.1159/000543139","url":null,"abstract":"<p><strong>Introduction: </strong>The incidence of diabetic ketoacidosis (DKA) at the time of diagnosis of type 1 diabetes in youth varies between countries and is influenced by socioeconomic factors. We investigated the relationship between regional deprivation indices and DKA at the diagnosis of type 1 diabetes in six countries.</p><p><strong>Methods: </strong>We analyzed children 0.5-17.0 years old diagnosed with type 1 diabetes between 2019 and 2022 across six diabetes registries. Regional deprivation index within each country was standardized to compare each individual's socioeconomic status internationally. Log-binomial regression models assessed the association between the standardized deprivation index and DKA, with sex, age group, and year as covariates.</p><p><strong>Results: </strong>Among 21,020 children (mean age 9.08 [SD 4.19] years), the DKA rate of 36.5%. Cohorts were Germany (n = 13,561, DKA 32.5%), Italy (4,659, 42.5%), Colorado, USA (1,318, 54.9%), Wales (769, 35.2%), New Zealand (407, 43.7%), and Slovenia (306, 37.6%). Deprivation was associated with the increased risk of DKA in children 0.5-<6 (OR 1.16 [95% CI: 1.10-1.23], p < 0.0001) and 6-<12 years of age (1.05 [1.05-1.11], p = 0.02). Female sex increased risk of DKA (1.06 [1.00-1.13], p = 0.04). The proportion of DKA was lower in 2019 than in 2020, 2021, and 2022 (each p < 0.0001).</p><p><strong>Conclusion: </strong>Deprivation was significantly associated with the risk of DKA at the diagnosis of type 1 diabetes, and DKA was more common during the pandemic years 2020-2022 than in 2019. Younger children appear to be more vulnerable to deprivation than older patients. Understanding and reducing local and demographic-specific disparities are essential for effective intervention.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-8"},"PeriodicalIF":2.6,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817914","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Long-Term Treatment for Laron Syndrome with IGF-1 Injection over 22 Years in Saudi Arabia: A Cohort Study. 在沙特注射IGF-1长期治疗Laron综合征超过22年:一项队列研究
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-12-10 DOI: 10.1159/000543047
Abdullah Ali Alashwal, Areej Al-Fattani, Khushnooda Ramzan, Faiqa Imtiaz, Amal Binladen
{"title":"Long-Term Treatment for Laron Syndrome with IGF-1 Injection over 22 Years in Saudi Arabia: A Cohort Study.","authors":"Abdullah Ali Alashwal, Areej Al-Fattani, Khushnooda Ramzan, Faiqa Imtiaz, Amal Binladen","doi":"10.1159/000543047","DOIUrl":"10.1159/000543047","url":null,"abstract":"<p><strong>Introduction: </strong>Laron syndrome (LS) is a rare autosomal recessive disorder caused by mutations in the growth hormone (GH) receptor gene, resulting in GH resistance and reduced levels of insulin-like growth factor 1 (IGF-1). Patients with LS exhibit severe growth retardation, low IGF-1 levels, elevated basal GH, and poor response to GH stimulation. Recombinant IGF-1 is the only approved treatment and has been shown to improve linear growth. This study evaluates the long-term efficacy and safety of IGF-1 therapy in a large cohort of LS patients treated at King Faisal Specialist Hospital and Research Center (KFSH &amp; RC), Riyadh, Saudi Arabia over 22 years.</p><p><strong>Methods: </strong>We conducted a retrospective review of medical records for 28 patients with growth hormone insensitivity syndrome, including 12 males and 16 females, treated with IGF-1 from 1998 to 2020. Patients were selected based on criteria including age over 2 years, height standard deviation score (SDS) ≤-2.8, normal or elevated GH secretion (>2.5 ng/mL), IGF-1 levels <50 ng/mL, and insensitivity to exogenous GH. IGF-1 was administered initially at 40 μg/kg/dose subcutaneously twice daily, escalating to a maximum of 120 μg/kg/dose as tolerated. Dosage was adjusted to minimize hypoglycemia risk, with blood glucose monitored frequently during hospitalization. In addition, molecular genetic results were reviewed for each patient in the cohort.</p><p><strong>Results: </strong>IGF-1 treatment significantly increased height velocity (HV) from a baseline of 3.4 cm/year to 6.5 cm/year in the first year (mean difference of 3.1 cm/year, p < 0.0001). In the second year, HV remained elevated at 5 cm/year (mean difference of 1.6 cm/year, p = 0.0015). Long-term follow-up over 10 years demonstrated sustained improvements in HV compared to baseline, with the most substantial gains occurring within the initial 5 years. Weight SDSs also showed significant improvement. Age at the start of therapy did not notably affect growth outcomes, though longer treatment durations were associated with greater growth. Ten disease-causing variants in the GHR gene were identified in 24 of the 28 LS patients.</p><p><strong>Conclusion: </strong>IGF-1 therapy significantly enhanced linear growth in children with Laron syndrome and was generally well tolerated. Although many patients did not reach normal adult height, the growth achieved with IGF-1 treatment was markedly better than expected without therapy. This study underscores the effectiveness of IGF-1 in improving growth outcomes and highlights the need for continued longitudinal studies to optimize treatment strategies and manage potential complications.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-11"},"PeriodicalIF":2.6,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Use of Automated Insulin Delivery around Physical Activity and Exercise in Type 1 Diabetes: A Position Statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD). 1型糖尿病患者在体育活动和运动中使用自动胰岛素输送:欧洲糖尿病研究协会(EASD)和国际儿科和青少年糖尿病学会(ISPAD)的立场声明
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-12-10 DOI: 10.1159/000542287
Othmar Moser, Dessi Zaharieva, Peter Adolfsson, Tadej Battelino, Richard M Bracken, Bruce A Buckingham, Thomas Danne, Elizabeth A Davis, Klemen Dovc, Gregory P Forlenza, Pieter Gillard, Sabine E Hofer, Roman Hovorka, Peter J Jacobs, Julia K Mader, Chantal Mathieu, Kirsten Nørgaard, Nick S Oliver, David N O'Neal, John Pemberton, Remi Rabasa-Lhoret, Jennifer L Sherr, Harald Sourij, Martin Tauschmann, Jane E Yardley, Michael C Riddell
{"title":"The Use of Automated Insulin Delivery around Physical Activity and Exercise in Type 1 Diabetes: A Position Statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD).","authors":"Othmar Moser, Dessi Zaharieva, Peter Adolfsson, Tadej Battelino, Richard M Bracken, Bruce A Buckingham, Thomas Danne, Elizabeth A Davis, Klemen Dovc, Gregory P Forlenza, Pieter Gillard, Sabine E Hofer, Roman Hovorka, Peter J Jacobs, Julia K Mader, Chantal Mathieu, Kirsten Nørgaard, Nick S Oliver, David N O'Neal, John Pemberton, Remi Rabasa-Lhoret, Jennifer L Sherr, Harald Sourij, Martin Tauschmann, Jane E Yardley, Michael C Riddell","doi":"10.1159/000542287","DOIUrl":"https://doi.org/10.1159/000542287","url":null,"abstract":"<p><p>Regular physical activity and exercise (PA) are cornerstones of diabetes care for individuals with type 1 diabetes. In recent years, the availability of automated insulin delivery (AID) systems has improved the ability of people with type 1 diabetes to achieve the recommended glucose target ranges. PA provides additional health benefits but can cause glucose fluctuations, which challenges current AID systems. While an increasing number of clinical trials and reviews are being published on different AID systems and PA, it seems prudent at this time to collate this information and develop a position statement on the topic. This joint European Association for the Study of Diabetes (EASD)/International Society for Pediatric and Adolescent Diabetes (ISPAD) position statement reviews current evidence on AID systems and provides detailed clinical practice points for managing PA in children, adolescents and adults with type 1 diabetes using AID technology. It discusses each commercially available AID system individually and provides guidance on its use in PA. Additionally, it addresses different glucose responses to PA and provides stratified therapy options to maintain glucose levels within the target ranges for these age groups.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-28"},"PeriodicalIF":2.6,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Endocrine Management of Transgender and Gender-Diverse Adolescents: Expert Opinion of the ESPE Working Group on Gender Incongruence and the Endo-ERN Main Thematic Group on Sexual Development and Maturation. 跨性别和性别多样化青少年的内分泌管理:ESPE性别不一致工作组和Endo-ERN性发展和成熟专题小组的专家意见。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-12-02 DOI: 10.1159/000542904
Sabine E Hannema, Kanetee Busiah, Gary Butler, Hedi L Claahsen-van de Grinten, Martine Cools, Aneta M Gawlik-Starzyk, Daniel Klink, Katharina M Main, Laetitia Martinerie, Annette Richter-Unruh, Nicos Skordis, Martine C de Vries
{"title":"Endocrine Management of Transgender and Gender-Diverse Adolescents: Expert Opinion of the ESPE Working Group on Gender Incongruence and the Endo-ERN Main Thematic Group on Sexual Development and Maturation.","authors":"Sabine E Hannema, Kanetee Busiah, Gary Butler, Hedi L Claahsen-van de Grinten, Martine Cools, Aneta M Gawlik-Starzyk, Daniel Klink, Katharina M Main, Laetitia Martinerie, Annette Richter-Unruh, Nicos Skordis, Martine C de Vries","doi":"10.1159/000542904","DOIUrl":"10.1159/000542904","url":null,"abstract":"","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-27"},"PeriodicalIF":2.6,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142768428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Contribution of Sex Steroids in Management of Tall Stature: Is It Effective or Not? 性类固醇在高个子管理中的作用:是否有效?
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-11-29 DOI: 10.1159/000542553
Nese Akcan, Federica Verdecchia, Peter C Hindmarsh, Mehul Dattani
{"title":"Contribution of Sex Steroids in Management of Tall Stature: Is It Effective or Not?","authors":"Nese Akcan, Federica Verdecchia, Peter C Hindmarsh, Mehul Dattani","doi":"10.1159/000542553","DOIUrl":"10.1159/000542553","url":null,"abstract":"<p><strong>Introduction: </strong>Reduction in adult height by high-dose sex steroids was introduced decades ago. Here, we present the impact of lower doses of sex steroids on the predicted adult height (PAH) in children with tall stature.</p><p><strong>Methods: </strong>This single-center retrospective observational study included 22 tall children treated with low-dose sex steroids. Patients with familial tall stature, constitutional advance of growth, or Marfan syndrome were included. Anthropometric measurements at the commencement of treatment, six-monthly intervals on treatment, cessation of treatment, and at final assessment, were evaluated. Bone age (BA) determination, and PAH were made using both the tables of Bayley-Pinneau (BP) and Tanner-Whitehouse (TW) mark II methods.</p><p><strong>Results: </strong>The final height was significantly lower than the predicted height in girls whereas it was not significantly lower than predicted height in boys. In patients with Marfan syndrome, the final height was only lower than the prediction of TW rather than BP. Non-Marfan cases had significantly lower final height than both the predicted heights. Conversely, although there was a decrease in height SDS over time, this difference was not statistically significant in the study cohort. Starting treatment at early BA (<10 years) did not affect the last height SDS or the difference between predicted height and final height.</p><p><strong>Conclusion: </strong>Sex difference, sex steroid dosage, differences in treatment duration and differences in BA measurement method, PAH method, BA and chronological age at the start of treatment may all influence the therapy response. Shortcomings about these influences can be overcome in future prospective studies with a larger sample size.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-11"},"PeriodicalIF":2.6,"publicationDate":"2024-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142768387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Co-Occurrence of Two Rare Diseases: A Child with Phenylketonuria and WNT1 Osteoporosis. 两种罕见疾病的并发症:一名患有苯丙酮尿症和 WNT1 骨质疏松症的儿童。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-11-27 DOI: 10.1159/000541378
Artemis Doulgeraki, Fan Wang, Anastasia Skouma, Eleana Petropoulou, Symeon Tournis, Alice Costantini, Outi Mäkitie
{"title":"Co-Occurrence of Two Rare Diseases: A Child with Phenylketonuria and WNT1 Osteoporosis.","authors":"Artemis Doulgeraki, Fan Wang, Anastasia Skouma, Eleana Petropoulou, Symeon Tournis, Alice Costantini, Outi Mäkitie","doi":"10.1159/000541378","DOIUrl":"10.1159/000541378","url":null,"abstract":"<p><strong>Introduction: </strong>Phenylketonuria (PKU), an inborn error of metabolism, when inadequately treated, may lead to nutritional deficits, which could affect bone health. This remains a controversial issue, given that in the majority of PKU cases, bone mineral density is within normal limits. On the other hand, WNT1 mutations are detrimental for bone, as they lead to primary osteoporosis.</p><p><strong>Case presentation: </strong>We present an eleven-year-old girl under a very strict diet for PKU (i.e., with low phenylalanine levels) and severe osteoporosis, signified by the presence of multiple vertebral fractures, which could not be attributed to her inborn error of metabolism. Family screening, including bone densitometry, revealed unexplained osteoporosis in her father and brother. Further genetic workup revealed a new WNT1, disease-causing mutation. The patient's dietary plan was modified, in order to achieve better metabolic control, and she was given vitamin D and calcium supplements. These measures led to great clinical and radiological improvement, without the use of bisphosphonates.</p><p><strong>Conclusion: </strong>In a patient with a chronic disorder known to affect the skeleton, the presence of disproportionally severe osteoporosis should prompt further diagnostic workup, in order to explain the severe bone phenotype, thus enabling more efficient and targeted therapeutic interventions.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-11"},"PeriodicalIF":2.6,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142739483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Similarities and Differences in Diurnal Salivary Adrenal Hormones in Monozygotic Twins with Discordant Birthweight. 出生体重不一致的单卵双胞胎昼间唾液肾上腺激素的异同。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-11-27 DOI: 10.1159/000542549
Thea Roedig, Nora Eberhardt, Peter Bartmann, Felix Schreiner, Paul-Martin Holterhus, Alexandra E Kulle, Sandra Schulte, Bettina Gohlke
{"title":"Similarities and Differences in Diurnal Salivary Adrenal Hormones in Monozygotic Twins with Discordant Birthweight.","authors":"Thea Roedig, Nora Eberhardt, Peter Bartmann, Felix Schreiner, Paul-Martin Holterhus, Alexandra E Kulle, Sandra Schulte, Bettina Gohlke","doi":"10.1159/000542549","DOIUrl":"10.1159/000542549","url":null,"abstract":"<p><strong>Introduction: </strong>Dehydroepiandrosterone sulphate is increased in formerly small-for-gestational age singletons and in smaller twins compared to their normal-weight co-twin. Less is known concerning other adrenal hormones. We compared diurnal salivary profiles in monozygotic twins with intra-twin birthweight (bw) differences to analyse the long-term impact of bw and persistent intra-twin auxological differences on various adrenal hormones.</p><p><strong>Methods: </strong>Forty-six monozygotic pre-pubertal twin pairs with bw difference of <1 standard deviation score (SDS) (concordant; n = 29, 13 female) and ≥1 SDS (discordant; n = 17, 6 female) were recruited. At a mean age of 6.9 years, saliva samples were collected (7:00 a.m./waking, 1:00 p.m., 6:00 p.m., and 9:00 p.m.) and analysed with liquid chromatography-tandem mass spectrometry.</p><p><strong>Results: </strong>Diurnal salivary concentrations showed significant intra-twin correlations in all twin pairs for nearly all time points: progesterone and androstenedione (4/4 time points), 17-hydroxyprogesterone (2/4). However, in the discordant group, mean progesterone differed significantly (p = 0.018) between the smaller (3.27 nmol/L, SD 3.87) and the larger twin (0.72 nmol/L, SD 0.42) at 7:00 a.m., and intra-twin differences were observed at 3/4 time points. Regression analyses of intra-twin differences confirmed that actual hormonal parameters were explained mainly by the co-twin parameter. However, at 6:00 p.m., progesterone concentration of the smaller twin was explained by bw (Β -1.102; SE 0.563; p = 0.05) and actual weight (Β 0.799; SE 0.327; p = 0.019).</p><p><strong>Conclusion: </strong>The significant correlations within monozygotic twin pairs in all measured hormones at nearly all time points suggest a strong genetic influence on the adrenal axis. Among the discordant twin pairs, we found significant variations in progesterone, suggesting additional long-lasting influence from intrauterine conditions on adrenal hormones with possible metabolic consequences.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-13"},"PeriodicalIF":2.6,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142739485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Feasibility of Using Continuous Glucose Monitoring to Detect Glycemic Abnormalities in Children with Cystic Fibrosis. 使用连续葡萄糖监测仪检测囊性纤维化患儿血糖异常的可行性。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-11-27 DOI: 10.1159/000542786
Amanda Leonard, Isabel R Judware, Lori L Vanscoy, Shruti M Paranjape, Donna Peeler, Malinda Wu, Scott M Blackman, Peter J Mogayzel
{"title":"Feasibility of Using Continuous Glucose Monitoring to Detect Glycemic Abnormalities in Children with Cystic Fibrosis.","authors":"Amanda Leonard, Isabel R Judware, Lori L Vanscoy, Shruti M Paranjape, Donna Peeler, Malinda Wu, Scott M Blackman, Peter J Mogayzel","doi":"10.1159/000542786","DOIUrl":"10.1159/000542786","url":null,"abstract":"<p><strong>Introduction: </strong>Cystic fibrosis-related diabetes (CFRD) is linked to worsening pulmonary function and increased mortality in people with cystic fibrosis (CF). Because of this correlation, early recognition of CFRD is important. Current recommendations for detecting glucose abnormalities using an oral glucose tolerance test (OGTT) can be difficult to achieve in a busy clinical setting.</p><p><strong>Methods: </strong>We trialed a 10-day continuous glucose monitoring (CGM) (Dexcom G6Pro) session in patients seen in our pediatric CF clinic that could not do an OGTT (reasons include emesis with OGTT or fear of needles) or that had an abnormal OGTT (to gather additional data to make treatment decisions).</p><p><strong>Results: </strong>Of the 36 sensors placed, 34 (94%) were returned. Devices were worn for a median of 10 days (range 4-10 days). Of the 34 CGMs returned, 20 (59%) met the criterion for referral to a pediatric endocrinologist.</p><p><strong>Conclusion: </strong>CGM placement is feasible in a busy CF clinic to detect glucose abnormalities in children.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-6"},"PeriodicalIF":2.6,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142739484","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Adult Height in Girls with Central Precocious Puberty with Onset after 6 Years: Effects of Gonadotropin-Releasing Hormone Analog Therapy. 6岁后发病的中枢性性早熟女孩的成年身高:促性腺激素释放激素类似物疗法的效果。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2024-11-21 DOI: 10.1159/000542038
Reiko Saito, Kayo Ozaki, Yoshiro Baba, Kento Ikegawa, Keisuke Nagasaki, Akie Nakamura, Takashi Hamajima, Shinji Higuchi, Yukihiro Hasegawa
{"title":"Adult Height in Girls with Central Precocious Puberty with Onset after 6 Years: Effects of Gonadotropin-Releasing Hormone Analog Therapy.","authors":"Reiko Saito, Kayo Ozaki, Yoshiro Baba, Kento Ikegawa, Keisuke Nagasaki, Akie Nakamura, Takashi Hamajima, Shinji Higuchi, Yukihiro Hasegawa","doi":"10.1159/000542038","DOIUrl":"10.1159/000542038","url":null,"abstract":"<p><strong>Introduction: </strong>Precocious puberty (PP), which is sometimes divided into gonadotropin-dependent or gonadotropin-independent PP, is a pathological condition characterized by premature secretion of gonadal steroids resulting in the early development of secondary sexual characteristics. Girls younger than 6 years with idiopathic gonadotropin-dependent PP (referred to as central PP or CPP) who receive gonadotropin-releasing hormone analog (GnRHa) therapy experience an increase in their adult height (AH) in contrast to girls who are aged 6 years or more, who show no consistent pattern of increase even with GnRHa therapy.</p><p><strong>Methods: </strong>In total, 133 girls aged 6 years or more who visited any one of the seven study centers between April 2000 and March 2020 and who met the diagnostic criteria for PP in Japan were retrospectively examined. The participants were divided into a treatment (n = 56) and no-treatment group (n = 77). The AH and target height (TH) were compared between the groups, and the factors influencing the AH were examined.</p><p><strong>Results: </strong>The patients receiving GnRHa therapy achieved significantly greater increase in their AH, AH - TH, and predicted AH at the age of 6, 7, and 8 years (6 ≤ years < 9) than those without the treatment. The TH and height at the start and end of treatment influenced the AH of the former group.</p><p><strong>Conclusion: </strong>GnRHa therapy was effective in improving the AH in girls with CPP onset at the age of 6, 7, or 8 years. The TH was a strong determinant of the AH.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"1-8"},"PeriodicalIF":2.6,"publicationDate":"2024-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142686874","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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