Hormone Research in Paediatrics最新文献

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Erratum. 勘误。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-06-13 DOI: 10.1159/000539579
{"title":"Erratum.","authors":"","doi":"10.1159/000539579","DOIUrl":"10.1159/000539579","url":null,"abstract":"","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"118"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11495235/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141319521","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}
引用次数: 0
Pigmented Hypertrichosis with Insulin-Dependent Diabetes Mellitus Syndrome: A Case Series. 色素性多毛症伴胰岛素依赖型糖尿病综合征:病例系列。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-01-01 DOI: 10.1159/000536019
An Jacobs, Paramita Cifelli, Daniel Delbeck, Nancy Elbarbary, Evelien Gevers, Zdenek Sumnik, Shenali Anne Amaratunga, Auste Pundziute Lyckå, Kristina Casteels
{"title":"Pigmented Hypertrichosis with Insulin-Dependent Diabetes Mellitus Syndrome: A Case Series.","authors":"An Jacobs, Paramita Cifelli, Daniel Delbeck, Nancy Elbarbary, Evelien Gevers, Zdenek Sumnik, Shenali Anne Amaratunga, Auste Pundziute Lyckå, Kristina Casteels","doi":"10.1159/000536019","DOIUrl":"10.1159/000536019","url":null,"abstract":"<p><strong>Introduction: </strong>Pigmented hypertrichosis with insulin-dependent diabetes mellitus (PHID) syndrome is a rare disease, and part of the cluster histiocytosis-lymphadenopathy plus syndrome (H syndrome), which is associated with mutations in the SLC29A3 gene. Patients with PHID show clinical features of H syndrome but also have insulin-dependent diabetes mellitus. The PHID has previously been described as predominantly in absence of pancreatic autoantibodies. Case Series Presentation: Through an open call in two international diabetes registers, clinical and genetic characteristics of 7 PHID patients in 6 treatment centres were collected after informed consent. All of them had consanguinity in their families, and their origins were located in North-African and Middle Eastern regions. Four out of 7 patients had at least one positive pancreatic autoantibody.</p><p><strong>Discussion and conclusion: </strong>Our case series reveals that PHID exhibits a wide range of clinical symptoms and signs. When consanguinity is present in a patient with newly diagnosed diabetes, and/or if other atypical symptoms such as dysmorphic features, skin lesions, haematological abnormalities, and developmental delay are present, threshold for genetic analysis should be low. Moreover, the presence of autoantibodies should not withhold genetic testing as our case series contradicts the previous observation of predominant autoantibody absence in PHID.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"84-88"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139073870","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-Related Adverse Conditions in Pediatric Patients Treated with Immune Checkpoint Inhibition for Malignancies. 接受免疫检查点抑制剂治疗的儿科恶性肿瘤患者出现的内分泌相关不良症状。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-02-23 DOI: 10.1159/000537969
Shlomit Shalitin
{"title":"Endocrine-Related Adverse Conditions in Pediatric Patients Treated with Immune Checkpoint Inhibition for Malignancies.","authors":"Shlomit Shalitin","doi":"10.1159/000537969","DOIUrl":"10.1159/000537969","url":null,"abstract":"<p><strong>Background: </strong>In recent years, remarkable advances in cancer immunotherapy have been introduced in the field of oncology. Since the discovery of immune checkpoint inhibitors (ICIs), these groups of medications have become a crucial treatment for several types of adult cancer.</p><p><strong>Summary: </strong>To date, pediatric experience with this group of medications is limited. Nevertheless, as clinicians, we have to be aware of the possible immune-related adverse events including immune-related endocrinopathies (thyroid dysfunction, diabetes mellitus, adrenal insufficiency, and pituitary insufficiency) that have been reported regarding these medications. These adverse events probably result from uncontrolled activation of the immune system.</p><p><strong>Key message: </strong>Early diagnosis, monitoring, and treatment of immune-related endocrinopathies associated with ICIs treatment are also essential for the best supportive care and administration of ICIs in pediatric patients. This review presents the current data on the immune-related endocrinopathies associated with the ICIs treatment, with suggestions for management.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"124-135"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139971711","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
Measured Resting Energy Expenditure by Indirect Calorimetry and Energy Intake in Long-Term Growth Hormone-Treated Children with PWS. 通过间接热量计测量长期接受生长激素治疗的 PWS 患儿的静息能量消耗和能量摄入。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-02-16 DOI: 10.1159/000536466
Demi J Trueba-Timmermans, Lionne N Grootjen, Alicia F Juriaans, Gerthe F Kerkhof, Edmond H H M Rings, Anita C S Hokken-Koelega
{"title":"Measured Resting Energy Expenditure by Indirect Calorimetry and Energy Intake in Long-Term Growth Hormone-Treated Children with PWS.","authors":"Demi J Trueba-Timmermans, Lionne N Grootjen, Alicia F Juriaans, Gerthe F Kerkhof, Edmond H H M Rings, Anita C S Hokken-Koelega","doi":"10.1159/000536466","DOIUrl":"10.1159/000536466","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Severe obesity can develop in children with PWS when food intake is not controlled. Maintenance of body weight requires an energy balance, of which energy intake and energy expenditure are important components. Previous studies described a decreased resting energy expenditure (REE) in growth hormone (GH)-untreated children with PWS. In short-term studies, no difference in REE was found between GH-treated and untreated children with PWS. However, there are limited data on REE in children with PWS who were GH-treated for a long period.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This study describes measured REE (mREE), energy intake, and body composition during long-term GH treatment in children with PWS. Patients were treated with 1.0 mg GH/m2/day (∼0.035 mg/kg/day). REE was determined by indirect calorimetry; dietary energy intake was calculated using a 3-day dietary record. Body composition by dual-energy X-ray absorptiometry (DXA) scans.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;We included 52 GH-treated children with PWS with a mean (SD) age of 8.53 (4.35) years and a median (IQR) GH-treatment duration of 7 (4-11) years. mREE increased with age, but was not associated with GH-treatment duration. A higher LBM was associated with higher mREE. Mean energy intake was significantly lower compared to daily energy requirements (DER) for age- and sex-matched healthy children (p &lt; 0.001), ranging from 23 to 36% less intake in children aged 3.5-12 years to 49% less intake in children aged 12-18 years. Fifty percent of children had a normal REE, 17.3% a decreased REE, and 32.7% an elevated REE, according to predicted REE based on measured REE in a large group of healthy children.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;In children with PWS, mREE increases with age. GH-treatment duration is not associated, whereas LBM is an important determinant of mREE. Children with PWS have a low to very low energy intake compared to DER for age- and sex-matched children, with a declining intake when becoming older.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Severe obesity can develop in children with PWS when food intake is not controlled. Maintenance of body weight requires an energy balance, of which energy intake and energy expenditure are important components. Previous studies described a decreased resting energy expenditure (REE) in growth hormone (GH)-untreated children with PWS. In short-term studies, no difference in REE was found between GH-treated and untreated children with PWS. However, there are limited data on REE in children with PWS who were GH-treated for a long period.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This study describes measured REE (mREE), energy intake, and body composition during long-term GH treatment in children with PWS. Patients were treated with 1.0 mg GH/m2/day (∼0.035 mg/kg/day). REE was determined by indirect calorimetry; dietary energy intake was calculated using a 3-day dietary record. Body composition by dual-energy X-ray absorptiome","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"164-173"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11965815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139899711","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}
引用次数: 0
Safety and Efficacy of Bilateral Epiphysiodesis Surgery to Reduce Final Height in Extremely Tall Adolescents: A Follow-Up Study. 通过双侧骨骺成形术降低极高青少年最终身高的安全性和有效性 - 一项随访研究。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-02-23 DOI: 10.1159/000538016
Tim R J Aeppli, Emelie Benyi, Henrik Wehtje, Dionisios Chrysis, Lars Sävendahl
{"title":"Safety and Efficacy of Bilateral Epiphysiodesis Surgery to Reduce Final Height in Extremely Tall Adolescents: A Follow-Up Study.","authors":"Tim R J Aeppli, Emelie Benyi, Henrik Wehtje, Dionisios Chrysis, Lars Sävendahl","doi":"10.1159/000538016","DOIUrl":"10.1159/000538016","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Treatment options in patients with extreme tall stature are limited. Bilateral epiphysiodesis has emerged as a possible treatment method aiming to reduce final height. However, there is still insufficient data on long-term safety and final height outcome. Therefore, the aim of this study was to assess the efficacy and safety of bilateral epiphysiodesis to reduce final adult height in tall adolescents.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;The study population consisted of 72 patients with extreme tall stature who were followed at the Pediatric Endocrine Clinic at the Karolinska University Hospital, Stockholm (Sweden), and subsequently underwent bilateral epiphysiodesis around the knees (girls n = 45, boys n = 27).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;When compared to the final height prediction at time of surgery, the procedure significantly reduced the achieved final height by a mean of 3.6 cm ± 0.4 cm in girls (p &lt; 0.001; 26.0 ± 2.9% reduction) and 8.6 ± 0.9 cm in boys (p &lt; 0.001; 40.5 ± 3.0% reduction). Furthermore, a negative correlation was observed between the absolute height reduction and the bone age at time of surgery, which was stronger in boys (r = -0.63, p &lt; 0.001) than in girls (r = -0.44, p &lt; 0.001). Besides reducing final height, body proportions were affected in all patients subjected to bilateral epiphysiodesis. However, as tall individuals typically have relatively long legs, body proportions were rather normalized after the surgery. There were no serious complications reported.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;This study suggests that bilateral epiphysiodesis is an efficient and safe method to reduce final height in extremely tall adolescent girls and boys. The achieved height reduction was higher in boys and when performed at an earlier bone age. Importantly, no serious side effects were reported. However, a continued follow-up is still warranted to detect any potential rare complications.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Treatment options in patients with extreme tall stature are limited. Bilateral epiphysiodesis has emerged as a possible treatment method aiming to reduce final height. However, there is still insufficient data on long-term safety and final height outcome. Therefore, the aim of this study was to assess the efficacy and safety of bilateral epiphysiodesis to reduce final adult height in tall adolescents.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;The study population consisted of 72 patients with extreme tall stature who were followed at the Pediatric Endocrine Clinic at the Karolinska University Hospital, Stockholm (Sweden), and subsequently underwent bilateral epiphysiodesis around the knees (girls n = 45, boys n = 27).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;When compared to the final height prediction at time of surgery, the procedure significantly reduced the achieved final height by a mean of 3.6 cm ± 0.4 cm in girls (p &lt; 0.001; 26.0 ± 2.9% reduction) and 8.6 ± 0.9 cm in boys (p &lt; 0.001; 40.5 ± 3","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"185-192"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11965831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139971712","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}
引用次数: 0
Post-Menarcheal Growth Patterns in a Contemporary Cohort of Latino Girls. 当代拉丁裔女孩月经初潮后的生长模式。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-01-31 DOI: 10.1159/000536506
Ximena Gaete, Pedro Ferrer-Rosende, Ana Pereira, Verónica Mericq
{"title":"Post-Menarcheal Growth Patterns in a Contemporary Cohort of Latino Girls.","authors":"Ximena Gaete, Pedro Ferrer-Rosende, Ana Pereira, Verónica Mericq","doi":"10.1159/000536506","DOIUrl":"10.1159/000536506","url":null,"abstract":"<p><strong>Introduction: </strong>Menarche is the last stage of pubertal development, which coincides with the completion of longitudinal growth. As a consequence of the lack of national and up-to-date data related to post-menarcheal (PM) growth, the aim of our work was to evaluate PM growth in a group of contemporary healthy Chilean girls followed, prospectively, until 4 years PM.</p><p><strong>Methods: </strong>This study was nested within the GOCS cohort, in a prospective fashion. The girls were followed yearly after menarche for at least for 4 years. We modeled each girl's growth using a SuperImposition by Translation and Rotation (SITAR) model.</p><p><strong>Results: </strong>A total of 534 girls were evaluated prospectively; 399 girls had height measured 2 years after menarche, 421 after 3 years, and 364 of 534 had height measured at 4 years PM. Expected height gained PM in the complete study group was 6.6 ± 2.5 cm. We observed that the largest gain in height occurred after the first year PM (3.8 ± 1.5 cm). According to the age of menarche, the group with earlier menarche (<11 years old) had a greater height gain in cm after 4 years PM (8.2 ± 3.2 cm) and the smallest gain was among girls with menarche at an age older than 13 years (4.4 ± 1.6) (p < 0.001). Age at menarche was significantly associated with all PM growth patterns (size, timing, and intensity), indicating that girls with older age at menarche grew taller, later, and slower than girls with younger age at menarche. Adjusting the PM growth pattern by BMI maintained all these associations. Applying the SITAR model specifically, girls experiencing menarche after the age of 13 years exhibited slower growth, occurring slightly earlier and with less intensity when adjusted by BMI at menarche.</p><p><strong>Conclusion: </strong>In a national and updated dataset, we observed that girls grew until 4 years PM an average of 6.6 ± 2.5 cm, with greatest gain occurring in the first year PM (3.8 ± 1.5 cm). Age at menarche was associated with menarche growth patterns.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"66-74"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139722342","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
Health-Related Quality of Life of Young Adult Women with a History of Premature Adrenarche. 有过早发情史的年轻成年女性的健康相关生活质量。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-03-12 DOI: 10.1159/000538283
Jussi Tennilä, Harri Sintonen, Pauliina Utriainen, Raimo Voutilainen, Jarmo Jääskeläinen, Jani Liimatta
{"title":"Health-Related Quality of Life of Young Adult Women with a History of Premature Adrenarche.","authors":"Jussi Tennilä, Harri Sintonen, Pauliina Utriainen, Raimo Voutilainen, Jarmo Jääskeläinen, Jani Liimatta","doi":"10.1159/000538283","DOIUrl":"10.1159/000538283","url":null,"abstract":"<p><strong>Introduction: </strong>Children with premature adrenarche (PA) have increased adrenal androgen concentrations and earlier pubertal development than their peers. Early sexual maturation and exposure to androgens have both been associated with an increased risk for neuropsychological adversities in adulthood. Such adversities would presumably influence the experienced health-related quality of life (HRQoL) of those affected.</p><p><strong>Methods: </strong>In a longitudinal case-control cohort study, 30 PA girls and 40 age-matched controls were followed from childhood to young adult age. The main outcome measure was the total 15D HRQoL score. In addition, we assessed specific dimensions of the questionnaire, the subjects' relationship statuses, and living arrangements.</p><p><strong>Results: </strong>There were no differences between the groups in the overall 15D scores (PA, 0.956 [0.052]; control, 0.947 [0.055]; p 0.482) or on any dimension of this instrument.</p><p><strong>Conclusion: </strong>The study suggests that a history of PA does not lead to impaired HRQoL in adult age.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"357-361"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133110/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140109849","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}
引用次数: 0
Neural Correlates of Obesity and Inflammation in Children and Adolescents with Congenital Adrenal Hyperplasia. 先天性肾上腺皮质增生症儿童和青少年肥胖和炎症的神经相关性。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-02-19 DOI: 10.1159/000537847
Mimi S Kim, Trevor A Pickering, Devyn L Cotter, Nicole R Fraga, Shan Luo, Cindy Y Won, Mitchell E Geffner, Megan M Herting
{"title":"Neural Correlates of Obesity and Inflammation in Children and Adolescents with Congenital Adrenal Hyperplasia.","authors":"Mimi S Kim, Trevor A Pickering, Devyn L Cotter, Nicole R Fraga, Shan Luo, Cindy Y Won, Mitchell E Geffner, Megan M Herting","doi":"10.1159/000537847","DOIUrl":"10.1159/000537847","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Patients with classical congenital adrenal hyperplasia (CAH) exhibit an increased prevalence of obesity from childhood including central adiposity and inflammation. There is also an emerging affected brain phenotype in CAH, with decreased cortico-limbic gray matter volumes and white matter abnormalities. We aimed to study the relationship between brain structure, obesity, and inflammation in children and adolescents with CAH compared to controls.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;27 CAH (12.6 ± 3.4 y, 16 females) and 35 control (13.0 ± 2.8 y, 20 females) participants had MRI of gray matter regions of interest (prefrontal cortex [PFC], amygdala, hippocampus) and white matter microstructure (fornix, stria terminalis [ST]). Anthropometric measures and lab analytes were obtained. Relaimpo analyses (relative importance for linear regression; percent variance) identified which brain structures were most different between groups. Subsequent regressions further quantified the magnitude and direction of these relationships. Correlations analyzed relationships between brain structure, obesity, and inflammation in the context of CAH status.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;PFC (13.3% variance) and its superior frontal (SF) subregion (14%) were most different between CAH and controls for gray matter; ST (16%) for white matter. Patients with CAH had lower caudal middle frontal (β = -0.56 [-0.96, -0.15]) and superior frontal (β = -0.58 [-0.92, -0.25]) subregion volumes, increased orientation dispersion index in the fornix (β = 0.56 [0.01, 1.10]) and ST (β = 0.85 [0.34, 1.36]), and decreased fractional anisotropy in the fornix (β = -0.91 [-1.42, -0.42]) and ST (β = -0.83 [-1.34, -0.33]) (all p's &lt; 0.05) indicating axonal disorganization, reduced myelin content, and/or higher microglial density within the affected white matter tracts. For the full cohort, SF was correlated with MCP-1 (r = -0.41), visceral adipose tissue (r = -0.25), and waist-to-height ratio (r = -0.27, all p's &lt; 0.05); ST was correlated with MCP-1 (r = 0.31) and TNF-α (r = 0.29, all p's &lt; 0.05); however, after adjusting for CAH status, almost all correlations were attenuated for significance.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Relationships among key brain structures, body composition, and inflammatory markers in pediatric patients with CAH could be largely driven by having CAH, with implications for obesity and neuroinflammation in this high-risk population.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Patients with classical congenital adrenal hyperplasia (CAH) exhibit an increased prevalence of obesity from childhood including central adiposity and inflammation. There is also an emerging affected brain phenotype in CAH, with decreased cortico-limbic gray matter volumes and white matter abnormalities. We aimed to study the relationship between brain structure, obesity, and inflammation in children and adolescents with CAH compared to controls.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"174-184"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11331025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139905539","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}
引用次数: 0
The Evaluation and Management of Methimazole-Induced Agranulocytosis in the Pediatric Patient: A Case Report and Review of the Literature. 小儿甲巯咪唑诱发粒细胞减少症的评估和处理:病例报告和文献综述。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-01-31 DOI: 10.1159/000536508
Meghan E Fredette, Sabitha Sasidharan Pillai, Osama Ibrahim, Manpreet Kochhar, Travis M Cotton, Tanya O Rogo, Margaret D Scheffler, Andrew J Bauer
{"title":"The Evaluation and Management of Methimazole-Induced Agranulocytosis in the Pediatric Patient: A Case Report and Review of the Literature.","authors":"Meghan E Fredette, Sabitha Sasidharan Pillai, Osama Ibrahim, Manpreet Kochhar, Travis M Cotton, Tanya O Rogo, Margaret D Scheffler, Andrew J Bauer","doi":"10.1159/000536508","DOIUrl":"10.1159/000536508","url":null,"abstract":"<p><strong>Introduction: </strong>Agranulocytosis is a rare but serious complication of methimazole (MMI) use for Graves' disease (GD). Treatment requires discontinuation of MMI, and the use of propylthiouracil (PTU) is also contraindicated. Few reports exist about the optimal alternative treatment regimens for the management of thyrotoxicosis in these medically complex patients in the pediatric population.</p><p><strong>Case report: </strong>We report prolonged saturated solution of potassium iodide (SSKI) use (29 days) in a 17-year-old female with GD and MMI-induced agranulocytosis, who presented with septic shock. Her treatment course also included β-blockade, cholestyramine, and granulocyte colony stimulating factor. We performed a review of the literature on the use of SSKI in the management of thyrotoxicosis, as well as best practices from the view of endocrinology, infectious disease, hematology, surgery, and intensivists, for the evaluation and management of MMI-induced agranulocytosis.</p><p><strong>Discussion: </strong>The management of MMI-induced agranulocytosis and associated sequelae require subspecialty input and intensive evaluation and monitoring. Alternative treatments to manage hyperthyroidism and control symptoms of thyrotoxicosis during agranulocytosis are a bridge to definitive therapy and include β-blockade, SSKI, cholestyramine, steroids, lithium, and plasmapheresis.</p><p><strong>Introduction: </strong>Agranulocytosis is a rare but serious complication of methimazole (MMI) use for Graves' disease (GD). Treatment requires discontinuation of MMI, and the use of propylthiouracil (PTU) is also contraindicated. Few reports exist about the optimal alternative treatment regimens for the management of thyrotoxicosis in these medically complex patients in the pediatric population.</p><p><strong>Case report: </strong>We report prolonged saturated solution of potassium iodide (SSKI) use (29 days) in a 17-year-old female with GD and MMI-induced agranulocytosis, who presented with septic shock. Her treatment course also included β-blockade, cholestyramine, and granulocyte colony stimulating factor. We performed a review of the literature on the use of SSKI in the management of thyrotoxicosis, as well as best practices from the view of endocrinology, infectious disease, hematology, surgery, and intensivists, for the evaluation and management of MMI-induced agranulocytosis.</p><p><strong>Discussion: </strong>The management of MMI-induced agranulocytosis and associated sequelae require subspecialty input and intensive evaluation and monitoring. Alternative treatments to manage hyperthyroidism and control symptoms of thyrotoxicosis during agranulocytosis are a bridge to definitive therapy and include β-blockade, SSKI, cholestyramine, steroids, lithium, and plasmapheresis.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"156-163"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11965825/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139722343","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}
引用次数: 0
ERRγ1 and Aromatase Expression in Human Placental Tissues from Term Deliveries of Large for Gestational Age Newborns. ERRγ1和芳香化酶在足月分娩的大妊娠期(LGA)新生儿的人类胎盘组织中的表达。
IF 2.6 3区 医学
Hormone Research in Paediatrics Pub Date : 2025-01-01 Epub Date: 2024-03-26 DOI: 10.1159/000538284
Marcos Abdul Palligas, Cristina Patricia Nemer, Claudia Monica Cannizzaro, Maria Sonia Baquedano, Alicia Belgorosky, Nora Saraco
{"title":"ERRγ1 and Aromatase Expression in Human Placental Tissues from Term Deliveries of Large for Gestational Age Newborns.","authors":"Marcos Abdul Palligas, Cristina Patricia Nemer, Claudia Monica Cannizzaro, Maria Sonia Baquedano, Alicia Belgorosky, Nora Saraco","doi":"10.1159/000538284","DOIUrl":"10.1159/000538284","url":null,"abstract":"<p><strong>Introduction: </strong>Being born either large for gestational age (LGA) or small for gestational age has been associated with an increased risk of developing metabolic syndrome in adulthood. However, the mechanism underlying this early programming remained unclear. Estrogen-related receptor gamma (ERRγ) is an orphan nuclear receptor with a high expression in the human placenta, particularly ERRγ1. ERRγ has been proposed to play a central role in controlling genes involved in energy metabolism. In the placenta, ERRγ1 acts as an oxygen-responsive transcription factor, regulating aromatase expression during trophoblast differentiation. Aromatase is an enzyme that catalyzes the synthesis of estrogens from androgens and is located in the syncytiotrophoblast. An adequate estrogen-androgen balance is required for normal pregnancy progression. Our aim was to analyze ERRγ1 and aromatase mRNA in human placenta from term LGA newborns. We propose that ERRγ1 and CYP19A1 expressions in the human placenta of LGA newborns are impaired, which would modify the fetal programming of LGA newborns since an imbalance in the intrauterine estrogen-androgen ratio would occur.</p><p><strong>Methods: </strong>Total RNA was obtained from the placental tissues of LGA (GA: 39-41 weeks, n = 8) and adequate for gestational age (AGA; 39-40 weeks, n = 10) newborns. ERRγ1 and aromatase mRNA variants were analyzed by RT2-PCR. Primers for aromatase analysis were specific for total aromatase (TotalAro) binding in exons 2-3 and for active aromatase (ActAro) in exons 9-10. Aromatase protein was analyzed by Western blot.</p><p><strong>Results: </strong>ERRγ1 mRNA was significantly higher in LGA compared to AGA. TotalAro mRNA was significantly lower in LGA in comparison with AGA control. Similar results were observed with aromatase protein. In contrast, ActAro/TotalAro ratio was higher in LGA compared to the AGA control.</p><p><strong>Conclusions: </strong>High expression of ERRγ1 as well as ActAro/TotalAro ratio in LGA suggests that ERRγ1 is involved in ActAro variant expression and hence disrupted estrogen-androgen balance in the intrauterine environment. We propose that dysregulation of ERRγ1 in the placenta might modify the estrogen-androgen balance in the intrauterine environment in LGA newborns, possibly representing one of the key factors in the regulation of fetal programming.</p>","PeriodicalId":13025,"journal":{"name":"Hormone Research in Paediatrics","volume":" ","pages":"307-315"},"PeriodicalIF":2.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140293397","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
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