European Journal of Endocrinology最新文献

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Histopathological evaluation based on CYP11B2 staining predicts outcomes in unilateral primary aldosteronism. 基于CYP11B2染色的组织病理学评估可预测单侧原发性醛固酮增多症的预后。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-05-30 DOI: 10.1093/ejendo/lvaf118
Tatiana S Goldbaum, Felipe L Ledesma, Augusto G Guimaraes, Jessica Okubo, Eduardo Z Kawahara, Vinicius F Calsavara, Luiz A Bortolotto, Jose L Chambo, Maria Candida B V Fragoso, Maria Adelaide A Pereira, Andrea Pio-Abreu, Giovanio V Silva, João V Silveira, Fernanda M Consolim-Colombo, Luciano F Drager, William C Nahas, Ana Claudia Latronico, Berenice B Mendonca, Madson Q Almeida, Maria Claudia N Zerbini
{"title":"Histopathological evaluation based on CYP11B2 staining predicts outcomes in unilateral primary aldosteronism.","authors":"Tatiana S Goldbaum, Felipe L Ledesma, Augusto G Guimaraes, Jessica Okubo, Eduardo Z Kawahara, Vinicius F Calsavara, Luiz A Bortolotto, Jose L Chambo, Maria Candida B V Fragoso, Maria Adelaide A Pereira, Andrea Pio-Abreu, Giovanio V Silva, João V Silveira, Fernanda M Consolim-Colombo, Luciano F Drager, William C Nahas, Ana Claudia Latronico, Berenice B Mendonca, Madson Q Almeida, Maria Claudia N Zerbini","doi":"10.1093/ejendo/lvaf118","DOIUrl":"https://doi.org/10.1093/ejendo/lvaf118","url":null,"abstract":"<p><strong>Background: </strong>The utility of histopathological classification based on aldosterone synthase (CYP11B2) immunostaining in unilateral primary aldosteronism (PA) for predicting clinical and biochemical outcomes after adrenalectomy remains controversial.</p><p><strong>Methods: </strong>We conducted a cohort study involving 131 consecutive patients with unilateral PA who underwent unilateral adrenalectomy. Aldosterone-producing adrenal lesions were classified according to the HISTALDO criteria. Biochemical and clinical outcomes were assessed using the PASO criteria.</p><p><strong>Results: </strong>Among the 131 adrenal lesions, classical and non-classical histology were identified in 101 (77.09%) and 30 (22.91%) cases, respectively. In the classical group, 89 cases were classified as aldosterone-producing adenoma (APA), and 12 as aldosterone-producing nodule (APN). Within the non-classical group, 27 cases (90%) had multiple aldosterone-producing micronodules, and 3 cases (30%) had multiple APNs. Patients with classical histology were younger (P = .028) and predominantly female (P = .028) compared to those with non-classical histology. Classical histology was associated with higher rates of complete biochemical success (97.03% vs 68.97%, P < .001) and complete hypertension remission (34.34% vs 10.71%, P < .001) compared to non-classical histology. Although clinical and biochemical outcomes were similar between APA and APN, their immunohistological characteristics differed (fewer clear cells and stronger CYP11B2 staining in APN). In multivariable analysis, classical histology remained independently associated with complete biochemical (P < .001) and clinical (P = .037) success.</p><p><strong>Conclusion: </strong>Classical histology was an independent variable associated with more severe PA, complete biochemical and hypertension remission in surgically treated patients with unilateral PA. Moreover, the distinction between APA and APN did not differentiate outcome.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 6","pages":"763-775"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144332668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Adolescent PCOS in South-East Asia: (epi)genetic origins of a hotspot. 东南亚青少年多囊卵巢综合征(PCOS):一个热点的遗传起源。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-05-30 DOI: 10.1093/ejendo/lvaf107
Francis de Zegher, Lourdes Ibáñez
{"title":"Adolescent PCOS in South-East Asia: (epi)genetic origins of a hotspot.","authors":"Francis de Zegher, Lourdes Ibáñez","doi":"10.1093/ejendo/lvaf107","DOIUrl":"10.1093/ejendo/lvaf107","url":null,"abstract":"","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"L23-L25"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144126504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Prevalence and clinical associations of USP8 variants in corticotroph tumours: a systematic review and aggregate data meta-analysis of 2171 cases. 皮质性肿瘤中USP8变异的患病率和临床相关性:2171例病例的系统回顾和汇总数据荟萃分析
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-05-30 DOI: 10.1093/ejendo/lvaf097
Luis G Perez-Rivas, Vivian von Selzam, Prajina Sharma, Martin Reincke, Marily Theodoropoulou
{"title":"Prevalence and clinical associations of USP8 variants in corticotroph tumours: a systematic review and aggregate data meta-analysis of 2171 cases.","authors":"Luis G Perez-Rivas, Vivian von Selzam, Prajina Sharma, Martin Reincke, Marily Theodoropoulou","doi":"10.1093/ejendo/lvaf097","DOIUrl":"10.1093/ejendo/lvaf097","url":null,"abstract":"<p><strong>Objective: </strong>Somatic USP8 variants are common in corticotroph tumours, but their reported prevalence and association with clinical characteristics vary widely among publications.</p><p><strong>Aim: </strong>To determine the prevalence and clinical relevance of USP8 variants based on published evidence.</p><p><strong>Design and methods: </strong>We conducted a systematic review and meta-analysis of existing literature. We used PubMed, Embase, and Web of Science databases. The inclusion criteria were original studies including ≥5 patients with Cushing's disease reporting genetic USP8 status. The exclusion criteria were no human research, unclear USP8 information, and case reports (<5 patients). A random-effects model meta-analysis and meta-regression were conducted. Studies reporting functional corticotroph tumours and also silent/non-functioning tumours were not excluded.</p><p><strong>Results: </strong>From 6782 extracted records, 44 studies summarizing 51 records were included in our meta-analysis (total n = 2171 cases, 692 with USP8 variants). Pooled prevalence was 31.1% (95% CI, 26.5%-36.0%) and was higher in cases with functional tumours (34.1%; 95% CI, 29.4%-39.1%). Patients with USP8 variants were mostly female (odds ratios [OR] 4.52, 95% CI, 3.39-6.02) and in average 4.47 years younger at diagnosis (95% CI, 2.28-6.65 years younger). USP8 status was associated with higher odds for postoperative remission (OR 1.76, 95% CI, 1.18-2.63) and recurrence (OR 2.38, 95% CI, 1.03-5.48). There was no clear evidence of association with any other clinical or tumour variable included in our analysis, mostly due to heterogeneity among studies. Meta-regression analysis showed that the variability in the prevalence of USP8 variants among studies was related to female/male ratio (adjusted R2 = 0.301), but not to other variables, such as tumour size or invasion.</p><p><strong>Conclusions: </strong>The present meta-analysis shows that patients with USP8 variant tumours are mostly female, diagnosed at younger age, more likely to achieve postoperative remission, but at a higher risk of recurrence than those with tumours carrying the reference allele.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"S41-S52"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144110123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Strengthening endocrinology training: integrating non-clinical competencies, patient and trainee perspectives for better implementation strategies. 加强内分泌学培训:整合非临床能力,患者和学员的观点,以更好地实施策略。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-05-30 DOI: 10.1093/ejendo/lvaf110
Amynta Arshad, Kashish Malhotra, Punith Kempegowda
{"title":"Strengthening endocrinology training: integrating non-clinical competencies, patient and trainee perspectives for better implementation strategies.","authors":"Amynta Arshad, Kashish Malhotra, Punith Kempegowda","doi":"10.1093/ejendo/lvaf110","DOIUrl":"10.1093/ejendo/lvaf110","url":null,"abstract":"","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"L26-L27"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144136059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Survival probabilities in patients with ectopic Cushing's syndrome-a systematic review and a single-arm meta-analysis. 异位库欣综合征患者的生存概率——一项系统综述和单臂荟萃分析。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-05-30 DOI: 10.1093/ejendo/lvaf114
Marta Piasecka, Eleni Papakokkinou, Adam Piasecki, Henrik Falhammar, Oskar Ragnarsson
{"title":"Survival probabilities in patients with ectopic Cushing's syndrome-a systematic review and a single-arm meta-analysis.","authors":"Marta Piasecka, Eleni Papakokkinou, Adam Piasecki, Henrik Falhammar, Oskar Ragnarsson","doi":"10.1093/ejendo/lvaf114","DOIUrl":"10.1093/ejendo/lvaf114","url":null,"abstract":"<p><strong>Objective: </strong>We aimed to estimate 1- and 5-year survival probabilities in patients with different forms of ectopic Cushing's syndrome (ECS) and identify factors influencing survival.</p><p><strong>Methods: </strong>In this systematic review and meta-analysis, we searched the online databases PubMed, Scopus, and Web of Science up to October 18, 2023, for studies reporting survival in patients with ECS. Data extraction and risk of bias assessment were performed by 3 independent investigators. Primary outcome was survival in patients with ECS and secondary outcome was factors influencing survival.</p><p><strong>Results: </strong>We included 40 studies with a total of 1148 patients. The pooled mean 1-year survival probability for ECS of mixed etiologies was 78% while the mean pooled 5-year survival probability was 47%. The 5-year survival probabilities for patients with pulmonary neuroendocrine neoplasm (NEN) was 81%, occult ECS 66%, thymic NEN 50%, and pancreatic NEN 40%. Only 8 studies reported factors influencing survival, where total resection of the primary tumor was associated with better overall survival, and unresectable tumors, metastatic disease at diagnosis, severe hypercortisolism, hypokalemia, and new-onset diabetes mellitus were associated with worse prognosis.</p><p><strong>Conclusion: </strong>Survival in ECS varies considerably, mainly due to the underlying origin of the tumor, tumor stage, and severity of the hypercortisolism. Further studies analyzing the importance of factors affecting survival are needed.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"S53-S65"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144208049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Hyperandrogenaemia, polycystic ovary syndrome, and physical fitness in women-a Northern Finland birth cohort study. 高雄激素血症、多囊卵巢综合征和女性体质——芬兰北部出生队列研究
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-04-30 DOI: 10.1093/ejendo/lvaf080
Katri Tuorila, Emilia Pesonen, Meri-Maija Ollila, Elisa Hurskainen, Marjukka Nurkkala, Raija Korpelainen, Maisa Niemelä, Laure Morin-Papunen, Terhi T Piltonen
{"title":"Hyperandrogenaemia, polycystic ovary syndrome, and physical fitness in women-a Northern Finland birth cohort study.","authors":"Katri Tuorila, Emilia Pesonen, Meri-Maija Ollila, Elisa Hurskainen, Marjukka Nurkkala, Raija Korpelainen, Maisa Niemelä, Laure Morin-Papunen, Terhi T Piltonen","doi":"10.1093/ejendo/lvaf080","DOIUrl":"https://doi.org/10.1093/ejendo/lvaf080","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the independent associations of hyperandrogenaemia (HA) and polycystic ovary syndrome (PCOS) with physical fitness in women among the general population.</p><p><strong>Design: </strong>A population-based birth cohort study including 5889 women.</p><p><strong>Methods: </strong>Longitudinal associations of serum testosterone (T), free androgen index (FAI), and PCOS with cardiorespiratory fitness (CRF) (measured by heart rate after a submaximal exercise test) and grip strength over the 31 to 46 years of age timespan were examined using multivariable linear mixed models adjusted for time, body mass index, homeostatic model assessment of insulin resistance, physical activity and smoking. The results are reported as regression coefficients (β) with corresponding 95% confidence intervals [95% CI].</p><p><strong>Results: </strong>The third and fourth T and FAI quartiles were associated positively with higher heart rate after the submaximal exercise test in multivariable models indicating poorer CRF compared with women in Q1 of T and FAI (Q3: β of T = 1.58 [95% CI: 0.21 to 2.96], β of FAI = 1.97 [0.54 to 3.39]; Q4: β of T = 1.88 [0.46 to 3.30], β of FAI = 2.70 [1.15 to 4.25]). The second, third, and fourth quartiles of FAI were associated with higher grip strength in multivariable models compared with women in Q1 (Q2: β = 0.59 [0.04 to 1.14], Q3: β = 0.74 [0.16 to 1.30], Q4: β = 0.68 [0.06 to 1.27]). Excluding women with PCOS did not alter these results, while PCOS itself was not associated with CRF or grip strength.</p><p><strong>Conclusion: </strong>Hyperandrogenaemia in premenopausal women was associated with poorer CRF but better grip strength, independently of PCOS, which suggests that HA, rather than PCOS, has an independent and complex association with physical fitness in premenopausal women.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 5","pages":"519-528"},"PeriodicalIF":5.3,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Genetic predisposition to adiposity, and type 2 diabetes: the role of lifestyle and phenotypic adiposity. 肥胖和2型糖尿病的遗传易感性:生活方式和表型肥胖的作用。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-04-30 DOI: 10.1093/ejendo/lvaf084
Mengrong Zhang, Joey Ward, Rona J Strawbridge, Jana J Anderson, Carlos Celis-Morales, Jill P Pell, Frederick K Ho, Donald M Lyall
{"title":"Genetic predisposition to adiposity, and type 2 diabetes: the role of lifestyle and phenotypic adiposity.","authors":"Mengrong Zhang, Joey Ward, Rona J Strawbridge, Jana J Anderson, Carlos Celis-Morales, Jill P Pell, Frederick K Ho, Donald M Lyall","doi":"10.1093/ejendo/lvaf084","DOIUrl":"https://doi.org/10.1093/ejendo/lvaf084","url":null,"abstract":"<p><strong>Aims: </strong>Genetic predisposition to adiposity is associated with type 2 diabetes (T2D), even in the absence of phenotypic adiposity (obesity and central obesity). We aimed to quantify the overall contribution of obesity and modifiable lifestyle factors to the association between genetic predisposition to adiposity and the development of T2D.</p><p><strong>Methods: </strong>This prospective cohort study involved 220 703 White British participants from the UK Biobank. It examined the associations between genetic predisposition to adiposity [body mass index polygenic risk (BMI-PRS) and waist-hip ratio polygenic risk (WHR-PRS)] and incident T2D, as well as interactions and mediation via lifestyle factors (diet quality, physical activity levels, total energy intake, sleep duration, and smoking and alcohol intake) and phenotypic adiposity.</p><p><strong>Results: </strong>People with high phenotypic adiposity and high adiposity PRS values (>1 SD above the mean) had the highest risk of incident T2D (versus non-obese/central obese and non-high PRS). This was the case for BMI-PRS [hazard ratio (HR) = 3.72] and WHR-PRS (HR = 4.17). Lifestyle factors explained 30.5% of the BMI-PRS/T2D association (2.0% mediation; 28.5% effect modification), and lifestyle and obesity together explained 92.1% (78.8% mediation; 13.3% effect modification). Lifestyle factors explained 20.4% of the WHR-PRS/T2D association (3.4% mediation; 17.0% effect modification), and lifestyle and central obesity together explained 72.8% (41.1% mediation; 31.7% effect modification).</p><p><strong>Conclusions: </strong>Whilst phenotypic adiposity explains a large proportion of the association between BMI-PRS/WHR-PRS and T2D, modifiable lifestyle factors also make contributions. Promoting healthy lifestyles among people prone to adiposity is important in reducing the global burden of T2D.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 5","pages":"549-557"},"PeriodicalIF":5.3,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056655/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143976170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sexual dimorphism in benign adrenocortical tumours. 良性肾上腺皮质肿瘤中的两性异形现象。
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-04-30 DOI: 10.1093/ejendo/lvaf088
Onnicha Suntornlohanakul, Cristina L Ronchi, Wiebke Arlt, Alessandro Prete
{"title":"Sexual dimorphism in benign adrenocortical tumours.","authors":"Onnicha Suntornlohanakul, Cristina L Ronchi, Wiebke Arlt, Alessandro Prete","doi":"10.1093/ejendo/lvaf088","DOIUrl":"10.1093/ejendo/lvaf088","url":null,"abstract":"<p><p>Benign adrenocortical tumours are the most common adrenal neoplasms. Evidence over the past few decades has highlighted sex differences in their prevalence, clinical characteristics, and treatment outcomes. Cortisol-producing adenomas causing either Cushing's syndrome, particularly those with PRKACA or GNAS somatic mutations associated with a more severe phenotype, or mild autonomous cortisol secretion (MACS) are more commonly observed in women. The mechanisms underpinning this sexual dimorphism remain to be fully elucidated. Studies in mice have revealed a protective role of androgens in males, leading to a decelerated growth rate of adrenocortical cells. Furthermore, evidence from human adrenal tumour tissue suggests that oestrogen, progesterone, and luteinising hormone/choriogonadotropin signalling in the adrenal cortex may play a role in adrenal tumourigenesis and steroid production. Clinically, this is supported by the increased incidence of cortisol-producing adrenocortical adenomas or nodular hyperplasia during puberty, pregnancy, and menopause. Notably, women with MACS seem to be more vulnerable to the harmful effects of cortisol excess and carry a higher mortality risk than men. Women with aldosterone-producing adenomas have a higher prevalence of somatic KCNJ5 mutations than men, and patients harbouring these mutations are likely to have more favourable clinical outcomes after adrenalectomy. In this review, we summarise the possible mechanisms behind the sexual dimorphism of benign adrenocortical tumours and provide an up-to-date overview of the sex-specific differences in their prevalence, clinical presentation, and outcomes, focusing on cortisol and aldosterone excess. Considering sexual dimorphism is crucial to guide diagnosis and management, and to counsel these patients for optimised care.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 5","pages":"R1-R12"},"PeriodicalIF":5.3,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12068951/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143990543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Increased insulin-like growth factor-1 concentrations in paediatric suprasellar low-grade glioma: an international multicentre study. 儿童鞍上低级别胶质瘤中胰岛素样生长因子-1浓度升高:一项国际多中心研究
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-04-30 DOI: 10.1093/ejendo/lvaf095
Ichelle M A A van Roessel, Boudewijn Bakker, Antoinette Y N Schouten-van Meeteren, Wim J E Tissing, Hoong-Wei Gan, Hanneke M van Santen
{"title":"Increased insulin-like growth factor-1 concentrations in paediatric suprasellar low-grade glioma: an international multicentre study.","authors":"Ichelle M A A van Roessel, Boudewijn Bakker, Antoinette Y N Schouten-van Meeteren, Wim J E Tissing, Hoong-Wei Gan, Hanneke M van Santen","doi":"10.1093/ejendo/lvaf095","DOIUrl":"https://doi.org/10.1093/ejendo/lvaf095","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study was to assess the prevalence of elevated insulin-like growth factor (IGF)-1 in children with suprasellar low-grade glioma (LGG) and explore the course of IGF-1 over time and its association with anthropometrics, hypothalamic syndrome, tumour characteristics, and tumour behaviour.</p><p><strong>Design: </strong>This retrospective study included children from the Netherlands and the United Kingdom diagnosed with a suprasellar LGG under the age of 18 between 2003 and 2023, with a minimum 1-year follow-up. Elevated IGF-1 was defined as IGF-1 standard deviation score (SDS) >+2.0 for age and biological sex, without growth hormone use.</p><p><strong>Results: </strong>We included 235 patients with a median age at brain tumour diagnosis of 3.8 years (IQR 1.7-7.1). Elevated IGF-1 was observed in 73 patients (31.1%) at any time point. At tumour diagnosis, 15.2% of the 138 children tested showed elevated IGF-1. Elevated IGF-1 was associated with younger age at tumour diagnosis (P = .004), neurofibromatosis type 1 (NF1; P = .028), and diencephalic syndrome (P = .047). In 55 of the 73 patients with elevated IGF-1 (75.3%), IGF-1 normalized spontaneously over time. Final height SDS corrected for target height SDS was not associated with having had an elevated IGF-1 (P = .113). No difference was found in the number of tumour progressions.</p><p><strong>Conclusions: </strong>Increased IGF-1 concentrations are commonly observed in children with suprasellar LGG, especially in younger children, those with an NF1 mutation, or during underweight. Elevation of IGF-1 can resolve over time, and the absence of an effect on final height or on tumour progressions seems reassuring.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 5","pages":"641-650"},"PeriodicalIF":5.3,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144119021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Blood pressure and its associations in 554 children and young people with congenital adrenal hyperplasia. 554例儿童和青少年CAH的血压及其相关性分析
IF 5.3 1区 医学
European Journal of Endocrinology Pub Date : 2025-04-30 DOI: 10.1093/ejendo/lvaf060
Neil R Lawrence, Irina Bacila, Joseph Tonge, Jeremy Dawson, Gary S Collins, Zi-Qiang Lang, Jillian Bryce, Malika Alimussina, Minglu Chen, Salma Rashid Ali, Safwaan Adam, Erica L T van den Akker, Tânia Aparecida Sartori Sanchez Bachega, Federico Baronio, Niels Holtum Birkebæk, Walter Bonfig, Hedi Claahsen-van der Grinten, Martine Cools, Eduardo Correa Costa, Miguel Debono, Liat de Vries, Christa E Flück, Gabriella Gazdagh, Ayla Güven, Sabine E Hannema, Violeta Iotova, Hetty J van der Kamp, Ruth Krone, Sofia Leka-Emiri, María Clemente-León, Corina Raducanu Lichiardopol, Renata L Markosyan, Tatjana Milenkovic, Mirela Costa de Miranda, Uta Neumann, John Newell-Price, Şükran Poyrazoğlu, Ursina Probst-Scheidegger, Gianni Russo, Luisa De Sanctis, Sumudu Nimali Seneviratne, Marianna Rita Stancampiano, Rieko Tadokoro-Cuccaro, Ajay Thankamony, Ana Vieites, Malgorzata Wasniewska, Diego Yeste, Jeremy Tomlinson, S Faisal Ahmed, Nils Krone
{"title":"Blood pressure and its associations in 554 children and young people with congenital adrenal hyperplasia.","authors":"Neil R Lawrence, Irina Bacila, Joseph Tonge, Jeremy Dawson, Gary S Collins, Zi-Qiang Lang, Jillian Bryce, Malika Alimussina, Minglu Chen, Salma Rashid Ali, Safwaan Adam, Erica L T van den Akker, Tânia Aparecida Sartori Sanchez Bachega, Federico Baronio, Niels Holtum Birkebæk, Walter Bonfig, Hedi Claahsen-van der Grinten, Martine Cools, Eduardo Correa Costa, Miguel Debono, Liat de Vries, Christa E Flück, Gabriella Gazdagh, Ayla Güven, Sabine E Hannema, Violeta Iotova, Hetty J van der Kamp, Ruth Krone, Sofia Leka-Emiri, María Clemente-León, Corina Raducanu Lichiardopol, Renata L Markosyan, Tatjana Milenkovic, Mirela Costa de Miranda, Uta Neumann, John Newell-Price, Şükran Poyrazoğlu, Ursina Probst-Scheidegger, Gianni Russo, Luisa De Sanctis, Sumudu Nimali Seneviratne, Marianna Rita Stancampiano, Rieko Tadokoro-Cuccaro, Ajay Thankamony, Ana Vieites, Malgorzata Wasniewska, Diego Yeste, Jeremy Tomlinson, S Faisal Ahmed, Nils Krone","doi":"10.1093/ejendo/lvaf060","DOIUrl":"10.1093/ejendo/lvaf060","url":null,"abstract":"<p><strong>Background: </strong>Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) affects approximately 1 in 15 000 individuals. We leveraged the power of multicentre registry data to assess the trend and predictors of blood pressure (BP) within children and young persons with 21OHD to inform monitoring strategies.</p><p><strong>Method: </strong>Data from the International CAH Registry in patients younger than 20 years was compared to normative values. Values of BP were modeled to create reference curves, multiple change point analysis applied to quantify the difference with normative data. Covariate adjustment was informed by a directed acyclic graph, prior to joint outcome regression modeling to accurately assess predictors of BP.</p><p><strong>Results: </strong>A total of 6436 visits within 554 patients (52.5% females) showed BP-Standard deviation scores (SDS) were higher at younger ages. Patients under five years had systolic BP-SDS of 1.6 (Q1:0.6-Q3:2.7) decreasing to 1.0 (Q1:0.2-Q3:1.8) over 5 years, equating to 31.0% over the 95th centile decreasing to 15.0%. Higher doses of fludrocortisone were associated with a small increase in systolic BP equivalent to 1.2 mmHg with every 100 µg extra fludrocortisone. Renin of 100µU/mL was associated with 4.6 mmHg lower systolic BP than a renin of 1µU/mL, higher 17OH-progesterone and androstenedione also predicted lower systolic and diastolic BP (P < .05).</p><p><strong>Conclusion: </strong>Higher BP in children with 21OHD is common and particularly pronounced at a younger age, but may not be attributable to excessive mineralocorticoid replacement. There is a need to improve our understanding of the determinants of this raised BP as well as its long-term effects.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"529-539"},"PeriodicalIF":5.3,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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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