Endocrine Connections最新文献

{"title":"An internet-based, multicenter, large-sample survey: knowledge, attitudes, and practices and pathway mechanisms among caregivers of children with central precocious puberty.","authors":"Shuxia Ding, Wenli Lu, Xiao Yu, Jing Chen, Shuangxi Cheng, Wei Gu, Qiao Wang, Gaohui Zhu, Pingping Zhang, Xiaoyu Ma, Caihong Liu, Yanyan Lin, Zhuannan Jiang, Yeqing Yuan, Jinglu Jin, Yu Pan, Yan Sun, Xiaoping Luo","doi":"10.1530/EC-26-0351","DOIUrl":"10.1530/EC-26-0351","url":null,"abstract":"<p><p>Central precocious puberty (CPP) requires long-term treatment and follow-up, during which caregivers play a central role in treatment adherence and family decision-making. This multicenter, cross-sectional, internet-based survey aimed to characterize knowledge, attitudes, and practices (KAP) among caregivers of children with CPP and to examine determinants and pathway relationships among these domains. Caregivers of children aged 2-16 years with CPP were recruited from eight pediatric endocrinology centers in China. An anonymous online questionnaire based on the disease-specific PRO-CaregiverCPP scale was administered. Knowledge scores, positive attitudes, and good practices were assessed using predefined criteria. Internal consistency was evaluated using Cronbach's alpha. Multivariable linear and logistic regression models were used to identify factors associated with knowledge scores, positive attitudes, and good practices. Observed-variable path analysis was performed to estimate direct and indirect associations among KAP domains. A total of 1,471 caregivers were included. The mean knowledge score was 6.31 ± 2.25; 72.5 and 58.8% of caregivers demonstrated positive attitudes and good practices, respectively. Higher knowledge scores were independently associated with greater odds of positive attitudes and good practices. Among caregivers of treated children, 3-monthly injections were associated with higher knowledge scores and higher proportions of positive attitudes and good practices than monthly injections. Path analysis showed that knowledge was directly associated with practices and indirectly associated with practices through positive attitudes. These findings indicate a structured KAP pattern in CPP caregiving and support physician-led education and follow-up strategies to improve caregiver engagement and long-term disease management.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012070","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}

{"title":"The EuRREB Core Endocrine Registry and its potential for studying transition of care in Endo-ERN centres.","authors":"Ana Luisa Priego Zurita, Mariya Cherenko, Jacqueline van der Blom-de Gunst, Jillian Bryce, Christopher Smythe, S Faisal Ahmed, Natasha M Appelman-Dijkstra","doi":"10.1530/EC-26-0333","DOIUrl":"10.1530/EC-26-0333","url":null,"abstract":"<p><strong>Abstract: </strong>Natural history registries can address knowledge gaps and support the development of best practice guidelines to improve outcomes in rare conditions. The European Registries for Rare Endocrine and Bone conditions (EuRREB) Core Registry is a standardised, multi-national platform designed to collect harmonised clinical data across rare endocrine disorders and facilitate longitudinal real-world analyses. The aim of this study was to describe data collected in the Core Registry across eight endocrine main thematic groups (MTGs) during the first six years following its launch in 2019, with a particular focus on the transition period, defined as 14-25 years of age. Forty-eight centres from 20 European and non-European countries contributed data, of which 33 (69%) were members of the European Reference Network on rare endocrine conditions (Endo-ERN). A total of 3,225 unique patients were registered, with a median age of 37 years. Overall, the hypothalamic and pituitary MTG accounted for the largest number of records (n = 1,548). The highest proportion of patients in the transition age group was observed in the sex development and maturation MTG (66%, 445/677), followed by thyroid (33%, 86/260) and growth and genetic obesity (32%, 42/130). A total of 303 patients had an active platform account, and 85 completed patient-reported outcome measures. Clinicians completed a total of 2,390 condition-specific outcomes. These findings demonstrate the capacity of the Core Registry to generate real-world insights across rare endocrine conditions and age groups. The observed transition-age representation highlights an opportunity to strengthen structured pathways and support continuity of care across the paediatric-adult interface.</p><p><strong>Plain language summary: </strong>The European Registries for Rare Endocrine and Bone conditions (EuRREB), supported by the Endo-ERN, collects information on rare diseases to improve care. This study shares what we learned over six years, including how young people move from paediatric to adult care.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037077","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}

{"title":"Efficacy and safety of the CAPTEM regimen in patients with advanced adrenocortical carcinoma.","authors":"Barbara Ziółkowska, Agnieszka Kotecka-Blicharz, Daria Handkiewicz-Junak, Kornelia Hasse-Lazar, Tomasz Gawlik, Adam Idasiak, Aleksandra Krzywon, Rafał Suwiński","doi":"10.1530/EC-26-0136","DOIUrl":"10.1530/EC-26-0136","url":null,"abstract":"<p><strong>Purpose: </strong>The purpose of this study was to evaluate the activity and tolerability of the CAPTEM regimen (capecitabine + temozolomide) in patients with advanced, heavily pretreated adrenocortical carcinoma (ACC).</p><p><strong>Methods: </strong>This is a retrospective analysis of 15 patients with metastatic ACC who received at least two lines of systemic therapy, including the CAPTEM regimen, between 2017 and 2022. Clinical characteristics, treatment outcomes (PFS and OS), and toxicities were collected and analysed. Treatment response was assessed using the RECIST 1.1 criteria. Survival outcomes were estimated using the Kaplan-Meier method.</p><p><strong>Results: </strong>Among the 15 patients, CAPTEM was administered in the first-line therapy in three patients (20%), in the second-line therapy in eight patients (53.3%), and in further lines in the remaining cases. The overall disease control rate was 26.6% (all stable disease). The median progression-free survival (PFS) was 3 months (95% CI: 2-6), and the median overall survival (OS) was 8 months (95% CI: 6-13). Patients who achieved disease stabilization had improved outcomes (mPFS: 9.5 months; mOS: 16.5 months) compared with those with progression (mPFS: 2 months; mOS: 7 months). Treatment-related adverse events occurred in 86.7% of patients, mostly mild; grade 3 toxicity was observed in one patient.</p><p><strong>Conclusion: </strong>The CAPTEM regimen demonstrated modest activity and acceptable toxicity in a heavily pretreated ACC population. While not recommended as a standard treatment, CAPTEM may be considered in select patients, particularly those who achieve disease stabilization. Further prospective studies are needed to better define its role and identify predictive biomarkers.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149024303","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}

{"title":"Epidemiology and management of neuroendocrine neoplasms of unknown primary in Switzerland.","authors":"Lena K Rhonheimer, Julia Beck, Alexander R Siebenhüner, Damian Wild, Emanuel R Christ, Julie Refardt","doi":"10.1530/EC-26-0498","DOIUrl":"10.1530/EC-26-0498","url":null,"abstract":"<p><strong>Background: </strong>Neuroendocrine neoplasms (NENs) are mainly located in the gastrointestinal tract or the lungs. In up to 14% of cases, the primary tumor remains unidentified, classified as cancer of unknown primary (CUP). Due to their rarity and heterogeneity, CUPs are poorly studied.</p><p><strong>Methods: </strong>This study conducts retrospective data analyses using the data provided by the SwissNET registry from 2008 to 2022, including patients diagnosed with CUPs or metastasized NENs with known primary (mNENs). The main objective was to assess the demographics, tumor characteristics, and the diagnosis and treatment of CUPs in comparison with mNENs. The secondary objective was to evaluate the overall survival of CUPs in general and compare with mNENs using propensity score matching.</p><p><strong>Results: </strong>A total of 207 CUP and 437 mNEN patients were included. Prevalence was slightly higher in men (54.6 and 57.9%, respectively). Median (IQR) age at diagnosis was higher for patients with CUPs (67.7 years (59.0, 77.2)) compared with mNENs (62.9 years (53.5, 71.5), P < 0.001). Grading in CUP patients was higher compared with mNENs, with tumors classified as G1 (20.8 vs 38.0%), G2 (30.4 vs 43.5%), and G3 (32.9 vs 9.6%; P < 0.001). Main CUP treatment involved somatostatin analogs (42%), followed by chemotherapy (32%) and PRRT (30%). Median survival time was significantly shorter for CUP patients compared with mNEN patients (23.3 months (95% CI: 15.5, 31.1) vs 151.5 months (95% CI: 122.8, 180.1; P < 0.001)). This difference persisted after propensity score matching (P = 0.007).</p><p><strong>Conclusion: </strong>The overall survival time of patients with CUPs was shorter compared with mNENs, possibly due to the higher-grade tumors at diagnosis.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149105903","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}

{"title":"Short and Long time Impact of SARS-CoV-2 on clinical characters of Graves' Disease Patients: A Retrospective Cohort Study.","authors":"Bingxin Li, Shiqi Song, Yuwei Gong, Xiaoyun Feng, Yanfang Hou, Jiaying Yang, Fang Liu, Yufan Wang, Yongde Peng, Yihan Zhang, Huanbai Xu, Ruihua Chen","doi":"10.1530/EC-26-0616","DOIUrl":"https://doi.org/10.1530/EC-26-0616","url":null,"abstract":"<p><strong>Background: </strong>To comprehensively evaluate the effects of SARS-CoV-2 infection on treated Graves' disease (GD) patients.</p><p><strong>Methods: </strong>This single-center retrospective cohort study included 220 patients with GD receiving stable antithyroid drug treatment. Among 179 questionnaire respondents, 107 had confirmed SARS-CoV-2 infection and 72 were uninfected. Paired thyroid-function analyses were performed in 65 infected patients, and long COVID was assessed in 72 participants.</p><p><strong>Results: </strong>Among the cohort, 107 patients (77.1% female; 69.7% aged <60 years) were infected with SARS-CoV-2. COVID-19 vaccination was less frequent among infected than uninfected participants (70.1% vs 84.7%, P = 0.025). At 12 weeks post-infection, TT3 increased from 1.76 to 1.97 nmol/L (P = 0.032), whereas TSH, FT3, FT4, TT4, and TRAb remained unchanged. Total cholesterol and HDL-C decreased after infection. TT3 changes differed by vaccination status (P for interaction=0.019), although vaccination was not associated with biochemical thyroid-function deterioration (OR 0.88, 95% CI 0.25-3.12). Among 72 participants completing long COVID assessment, 37 (51.4%) met the operational definition; increased hair loss was the most common symptom. Smoking was associated with higher odds of long COVID (OR 5.37, 95% CI 1.05-27.36).</p><p><strong>Conclusions: </strong>SARS-CoV-2 infection in treated GD patients was associated with modest short-term thyroid and metabolic changes in treated GD patients, while most thyroid indices remained stable. These exploratory findings highlight the need for further prospective studies.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963183","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}

{"title":"Immune checkpoint inhibitor-associated severe hyponatremia: evidence mapping of SIADH-like phenotypes and endocrine immune-related adverse events.","authors":"Fangfei Guo, Songchen Han, Zhihui Xue, Wenlong Chen, Yunjiu Gou","doi":"10.1530/EC-26-0417","DOIUrl":"10.1530/EC-26-0417","url":null,"abstract":"<p><strong>Objective: </strong>Severe or symptomatic hyponatremia during immune checkpoint inhibitor (ICI) therapy may reflect syndrome of inappropriate antidiuresis (SIADH), endocrine immune-related adverse events (irAEs), cancer-related factors, or mixed mechanisms. We mapped the evidence and reclassified diagnostically extractable cases to distinguish SIADH-like phenotypes from endocrine irAEs.</p><p><strong>Methods: </strong>PubMed, Embase, Web of Science Core Collection, Scopus, and the Cochrane Library were searched through May 24, 2026. Reports were assigned to A1 case-level, A2 population-level, or A3 diagnostic-framework layers. A1 summaries used patient/case denominators after a report-versus-patient audit. Diagnostic categories were reviewer-derived. Reporting completeness was described using six prespecified domains. We performed source-label and full-length-only sensitivity analyses.</p><p><strong>Results: </strong>All 194 reports sought for retrieval were assessed, and 146 were included: 127 A1 reports describing 144 patients/cases, 16 A2 reports, and 3 A3 reports. Strict classification identified 127/144 (88.2%) confirmed/probable adrenal-axis irAEs, 1/144 (0.7%) confirmed/probable SIADH, 3/144 (2.1%) SIADH-like phenotypes with incomplete endocrine exclusion, 2/144 (1.4%) thyroid-related cases, and 11/144 (7.6%) mixed, confounded, or non-endocrine mechanisms. Ten patients/cases carried an explicit source-level SIADH label; strict review classified six as adrenal-axis irAEs, one as SIADH, and three as SIADH-like with incomplete endocrine exclusion. After exclusion of 81 abstract/database-only A1 reports, 46 full-length reports described 59 patients: 52 adrenal-axis irAEs, 1 SIADH, and 6 mixed/confounded or non-endocrine cases.</p><p><strong>Conclusion: </strong>Published classifiable A1 cases were dominated by adrenal-axis irAEs. Strictly supported SIADH occurred but was rare in this selected evidence base, and the direction of findings persisted in the full-length-only analysis. These proportions are not incidence estimates. The proposed algorithm supports diagnostic sequencing and transparent reporting; it is not a stand-alone practice guideline.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13588285/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148758790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From bench to bedside: primary aldosteronism revisited.","authors":"Jia Wei, Tracy Ann Williams","doi":"10.1530/EC-26-0524","DOIUrl":"10.1530/EC-26-0524","url":null,"abstract":"<p><p>Primary aldosteronism (PA) is a common, under-recognised cause of hypertension carrying cardiovascular and renal risk beyond blood pressure alone. Management requires accurate subtype diagnosis: lateralised disease may be cured by adrenalectomy, whereas bilateral disease usually requires mineralocorticoid receptor antagonist therapy. The PASO criteria standardised postsurgical outcome assessment and showed that biochemical remission is achieved in most patients with adrenal vein sampling-confirmed lateralised disease, whereas clinical remission is variable, reflecting pre-existing hypertensive burden, age and sex. The PAMO criteria extend this framework to medically treated PA and show that complete clinical response is uncommon. At the tissue level, the HISTALDO classification distinguishes classical lateralised disease, typically caused by an aldosterone-producing adenoma, from non-classical disease, dominated by multiple micronodules; the latter carries a higher risk of postsurgical persistent or recurrent aldosteronism. These observations support a broader model in which PA forms a continuous rather than binary spectrum, from subclinical renin-independent aldosteronism and age-related micronodular remodelling to overt bilateral and lateralised disease. Tissue omics studies support a model in which aldosterone-producing lesions progress from zona glomerulosa cells to micronodules and adenomas. KCNJ5-mutated adenomas may follow a direct route, arising without a detectable micronodule stage, possibly because reduced oxidative stress favours early cell survival and expansion. The adenoma is therefore not a static endpoint but a maturing tissue, in which immune remodelling and changes in cell survival mechanisms may shape progression towards a hypersecretory state. Together, these findings link clinical phenotype to adrenal tissue biology and support a dynamic, genotype-modulated model of PA pathogenesis.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13588286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148817867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ERRATUM to: Intracellular FGF1 promotes invasion and migration in thyroid carcinoma via HMGA1 independent of FGF receptors.","authors":"","doi":"10.1530/EC-23-0014e3","DOIUrl":"https://doi.org/10.1530/EC-23-0014e3","url":null,"abstract":"","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":"15 9","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148976104","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}

{"title":"Clinical and genetic spectrum of cytochrome P450 oxidoreductase deficiency in Chinese patients.","authors":"TingTing Wang, Chong Li, Chao Han, YanYan Zhao, GuangZhao Qi, LiLi Zheng","doi":"10.1530/EC-26-0247","DOIUrl":"10.1530/EC-26-0247","url":null,"abstract":"<p><strong>Background: </strong>Cytochrome P450 oxidoreductase deficiency (PORD) is a rare autosomal recessive disorder with heterogeneous endocrine, reproductive, DSD-related, and skeletal manifestations. Population-specific data in Chinese patients remain limited.</p><p><strong>Methods: </strong>We described two Chinese probands with genetically confirmed PORD and systematically reviewed published Chinese cases. Clinical features, POR genotypes, skeletal manifestations, adrenal function, DSD phenotypes, and reproductive features were extracted. Carrier frequency and modeled genetic prevalence were estimated using gnomAD and WBBC, with variants stratified by evidence of pathogenicity.</p><p><strong>Results: </strong>The pooled cohort included 40 unique Chinese patients, comprising two newly reported probands and 38 literature-derived cases. One proband carried p.R457H and a novel frameshift variant, p.N178Efs*28; the second carried p.R457H and p.Y607C. The recurrent p.R457H variant accounted for 40 of 80 alleles in the Chinese cohort. Using the expanded exploratory variant set, the modeled genetic prevalence was 2.4 per 1,000,000 individuals in WBBC Chinese and 3.3 per 1,000,000 individuals in gnomAD East Asians.</p><p><strong>Conclusion: </strong>Chinese patients with PORD show broad clinical heterogeneity, and p.R457H is a recurrent East Asian-enriched POR variant. The novel p.N178Efs*28 variant expands the POR mutational spectrum. Population-database estimates should be interpreted as modeled genetic prevalence rather than observed clinical incidence, especially when predicted deleterious variants are included.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13588290/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148789343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Circulating matrix GLA protein during puberty in healthy and central precocious puberty children.","authors":"Maria Bentz Damholt, Stine A Holmboe, Jørgen Holm Petersen, Martin Blomberg Jensen, Anders Juul, Li Juel Mortensen","doi":"10.1530/EC-26-0408","DOIUrl":"10.1530/EC-26-0408","url":null,"abstract":"<p><strong>Objective: </strong>Matrix GLA protein (MGP) is involved in the regulation of extracellular matrix mineralisation, including bone formation. The peripubertal years are critical for linear growth and acquisition of bone mass. Emerging evidence suggests that plasma concentrations of dephosphorylated undercarboxylated MGP (dp-ucMGP) may vary across pubertal stages, potentially reflecting its role in bone remodelling.</p><p><strong>Methods: </strong>This study examines longitudinal changes in circulating plasma dp-ucMGP in two cohorts: 111 healthy Danish children (52% girls) aged 6-15 years from the Copenhagen Puberty Study and 15 female patients with central precocious puberty (CPP) before, during, and after GnRH analogue treatment.</p><p><strong>Results: </strong>Age- and sex-specific reference curves were generated for healthy children and adolescents. Dp-ucMGP increased significantly with age and pubertal progression in both sexes, while it declined from mid-puberty exclusively in boys. In girls only, dp-ucMGP SD scores were associated with body weight and BMI. In girls with CPP, dp-ucMGP concentrations were lower compared with healthy puberty-matched girls (Tanner B2: P = 0.016, Tanner B3: P = 0.0072). Initiation of GnRH agonist in CPP resulted in a significant increase in dp-ucMGP (-1.21 SD (baseline) vs 0.30 SD (3 months), P = 0.0048) but plateaued after 12 months of treatment and further declined towards baseline at treatment cessation.</p><p><strong>Conclusion: </strong>We show that circulating dp-ucMGP concentrations increase with age and pubertal progression in healthy children, are lower in CPP patients, and are suppressed by GnRH agonist. Altogether, our findings support that circulating concentrations of dp-ucMGP reflect its role in bone remodelling and linear growth during puberty.</p><p><strong>Plain language summary: </strong>In this study, we show that matrix GLA protein (MGP), a regulator of bone formation, increases with pubertal progression in healthy children but is suppressed when puberty is pharmacologically blocked in girls with early puberty. Our findings provide new insights into the potential role of MGP in bone growth during puberty.</p>","PeriodicalId":11634,"journal":{"name":"Endocrine Connections","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13641139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}