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Disrupted harmony of the skull skeleton: Middle ear resonance in acromegaly 颅骨骨架的和谐被破坏:肢端肥大症的中耳共鸣
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.ghir.2024.101594
Elif Kaya Çelik , Serap Er , Kemal Keseroğlu , Fatih Öner , Hakan Düğer , Bekir Uçan , Muhammed Kızılgül , Şirin Çetin , Güleser Saylam

Objectives

This study aims to investigate whether the middle ear resonance frequency (RF) is affected in acromegaly, which causes growth in the skull bone.

Methods

Thirty acromegaly patients and 38 volunteers were included in the study. Pure tone average scores and middle ear RF values of the groups that underwent pure tone audiometry, tympanometry, and multifrequency tympanometry tests were compared.

Results

The pure tone mean was 14.95 ± 12.13 in acromegaly patients and 5.70 ± 8.52 in the control group (p:0.18). Sensorineural hearing loss(SNHL) was observed in 16.6% of the patients. The average middle ear RF was calculated as 815 ± 179.05 Hz in patients with acromegaly and 773 ± 127.15 in the control group. (p = 0.0001).

Conclusion

This study is the first to evaluate middle-ear RF in acromegaly patients. Acromegaly-induced changes in soft tissues and bone structures impact middle ear functions. In this patient group, we found an increase in middle ear RF without conductive-type hearing loss and a 16.6% rate of SNHL.

目的 本研究旨在探讨肢端肥大症患者的中耳共振频率(RF)是否会受到影响,因为肢端肥大症会导致颅骨增生。结果肢端肥大症患者的纯音平均值为(14.95 ± 12.13),对照组为(5.70 ± 8.52)(P:0.18)。16.6%的患者出现感音神经性听力损失(SNHL)。经计算,肢端肥大症患者的平均中耳射频为 815 ± 179.05 Hz,对照组为 773 ± 127.15 Hz。(结论本研究首次评估了肢端肥大症患者的中耳射频。肢端肥大症引起的软组织和骨结构变化会影响中耳功能。在这组患者中,我们发现中耳射频增加,但无传导型听力损失,SNHL 发生率为 16.6%。
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引用次数: 0
Acromegaly in humans and cats: Pathophysiological, clinical and management resemblances and differences 人和猫的肢端肥大症:病理生理学、临床和管理方面的相似之处和不同之处
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-05-24 DOI: 10.1016/j.ghir.2024.101595
Mariana Lopes-Pinto , Patrícia Lunet Marques , Ema Lacerda-Nobre , Diego Miceli , Rodolfo Oliveira Leal , Pedro Marques

Objective

Acromegaly is a disorder associated with excessive levels of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). In general, GH/IGF-1 excess leads to morphologic craniofacial and acral changes as well as cardiometabolic complications, but the phenotypic changes and clinical presentation of acromegaly differ across species. Here, we review the pathophysiology, clinical presentation and management of acromegaly in humans and cats, and we provide a systematic comparison between this disease across these different species.

Design

A comprehensive literature review of pathophysiology, clinical features, diagnosis and management of acromegaly in humans and in cats was performed.

Results

Acromegaly is associated with prominent craniofacial changes in both species: frontal bossing, enlarged nose, ears and lips, and protuberant cheekbones are typically encountered in humans, whereas increased width of the head and skull enlargement are commonly found in cats. Malocclusion, prognathism, dental diastema and upper airway obstruction by soft tissue enlargement are reported in both species, as well as continuous growth and widening of extremities resulting in osteoarticular compromise. Increase of articular joint cartilage thickness, vertebral fractures and spine malalignment is more evident in humans, while arthropathy and spondylosis deformans may also occur in cats. Generalized organomegaly is equally observed in both species. Other similarities between humans and cats with acromegaly include heart failure, ventricular hypertrophy, diabetes mellitus, and an overall increased cardiometabolic risk. In GH-secreting pituitary tumours, local compressive effects and behavioral changes are mostly observed in humans, but also present in cats. Cutis verticis gyrata and skin tags are exclusively found in humans, while palmigrade/plantigrade stance may occur in some acromegalic cats.

Serum IGF-1 is used for acromegaly diagnosis in both species, but an oral glucose tolerance test with GH measurement is only useful in humans, as glucose load does not inhibit GH secretion in cats. Imaging studies are regularly performed in both species after biochemical diagnosis of acromegaly. Hypophysectomy is the first line treatment for humans and cats, although not always available in veterinary medicine.

Conclusion

Acromegaly in humans and cats has substantial similarities, as a result of common pathophysiological mechanisms, however species-specific features may be found.

肢端肥大症是一种与生长激素(GH)和胰岛素样生长因子-1(IGF-1)水平过高有关的疾病。一般来说,GH/IGF-1过量会导致颅面和口角的形态变化以及心脏代谢并发症,但不同物种肢端肥大症的表型变化和临床表现各不相同。在此,我们综述了人类和猫科动物肢端肥大症的病理生理学、临床表现和治疗方法,并对这些不同物种的肢端肥大症进行了系统性比较。结果 在这两种动物中,肢端肥大症都与突出的颅面变化有关:人类通常会出现额凸、鼻子、耳朵和嘴唇肥大以及颧骨突出等症状,而猫则常见头部宽度增加和头骨增大。据报道,这两种动物都会因软组织增大而出现咬合不正、前牙反颌、牙齿间隙和上呼吸道阻塞,四肢也会因持续增长和增宽而导致骨关节受损。关节软骨厚度增加、脊椎骨折和脊椎错位在人类中更为明显,而关节病和脊椎变形症也可能发生在猫身上。在这两种疾病中同样可以观察到全身器官肿大。患有肢端肥大症的人和猫的其他相似之处还包括心力衰竭、心室肥大、糖尿病和整体心脏代谢风险增加。在分泌 GH 的垂体瘤中,局部压迫效应和行为变化主要出现在人类身上,但也出现在猫身上。血清 IGF-1 可用于两种动物的肢端肥大症诊断,但口服葡萄糖耐量试验和 GH 测量只对人类有用,因为葡萄糖负荷不会抑制猫的 GH 分泌。生化确诊肢端肥大症后,两种动物都要定期进行影像学检查。猫下腹切除术是人类和猫的一线治疗方法,但在兽医领域并不总是可行。 结论:由于共同的病理生理机制,人类和猫的肢端肥大症具有很大的相似性,但也可能存在物种特异性。
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引用次数: 0
Prostate-specific antigen (PSA) levels in men with Prader-Willi syndrome 患有普拉德-威利综合征的男性的前列腺特异性抗原(PSA)水平
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-04-21 DOI: 10.1016/j.ghir.2024.101593
Anna Oskarsson , Charlotte Höybye

Prader-Willi syndrome (PWS) is a rare genetic disorder typically characterized by body composition abnormalities, hyperphagia, behavioral challenges, cognitive dysfunction, and hormone deficiencies. Hypogonadism is common but knowledge on potential side effects of testosterone replacement is limited, in particular, the long-term effects on behavior and PSA.

Patients and methods

Retrospective case studies of seven men, median age 46 years, with genetically verified PWS, testosterone treated hypogonadism and available PSA values were included. Long-term follow-up of PSA was accessible in four patients. Medical records were reviewed for adverse effects.

Results

Five men were treated with intramuscular testosterone undecanoate, two had no hypogonadism. Median PSA was 0.68 μg/L (0.23–1.3), median testosterone 15 nmol/L. After a median time of 17 years of testosterone replacement median PSA was 0.75 μg/L (range 0.46–1.4). Testosterone replacement was well tolerated, and no major behavioral changes were reported. Five were treated with growth hormone for >20 years.

Conclusion

Levels of PSA were low. Long-term treatment with testosterone was working well and did not result in any clinically meaningful increase in PSA. Our results indicate that testosterone replacement is neither associated with serious adverse events regarding changes in behavior or effect on PSA. However, larger studies are needed to confirm our results.

普拉德-威利综合征(Prader-Willi Syndrome,PWS)是一种罕见的遗传性疾病,其典型特征是身体成分异常、多食、行为障碍、认知功能障碍和激素缺乏。性腺功能减退症很常见,但有关睾酮替代潜在副作用的知识却很有限,尤其是对行为和 PSA 的长期影响。患者和方法回顾性病例研究纳入了七名男性患者,中位年龄 46 岁,经基因验证患有 PWS,接受过睾酮治疗性腺功能减退症,并有 PSA 值。对四名患者进行了 PSA 长期随访。对医疗记录进行了审查,以了解不良反应。结果五名男性接受了肌肉注射十一酸睾酮治疗,其中两人没有性腺功能减退症。中位 PSA 为 0.68 μg/L (0.23-1.3),中位睾酮为 15 nmol/L。中位睾酮替代时间为 17 年后,中位 PSA 为 0.75 μg/L(0.46-1.4)。患者对睾酮替代治疗的耐受性良好,未报告有重大行为改变。结论 PSA 水平较低。睾酮的长期治疗效果良好,没有导致任何有临床意义的 PSA 增高。我们的研究结果表明,睾酮替代既不会引起行为改变方面的严重不良事件,也不会影响 PSA。不过,还需要更大规模的研究来证实我们的结果。
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引用次数: 0
Pediatric growth hormone deficiency: Understanding the patient and caregiver perspectives 小儿生长激素缺乏症:了解患者和护理人员的观点
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-04-16 DOI: 10.1016/j.ghir.2024.101592
Bradley S. Miller , Nicky Kelepouris , Harlan Tenenbaum , Sonia Di Carlo , Philippe Backeljauw

Childhood growth hormone deficiency (GHD) is a rare disorder associated with significant burden on both patients and caregivers. Although previous reports have detailed aspects of the burden experienced by patients and their caregivers, there is a paucity of first-hand information on the patient and caregiver journeys from their respective voices. To address this need, an advisory board meeting was conducted on September 30, 2022, with 4 pediatric patients with GHD and their caregivers to discuss their experiences prior to GHD diagnosis, during the diagnostic process, and during ongoing treatment with recombinant growth hormone. Feedback from patients and caregivers was reviewed by pediatric endocrinologists, who provided their own perspectives on the patient and caregiver journeys based on the information reported. Despite the small sample size, important insights were obtained: patients and caregivers reported remarkable growth benefits achieved with treatment, which provided strong motivation to remain adherent to daily injection regimens. Patient and caregiver accounts reflected wide variability between families in time from suspicion to diagnosis and in treatment challenges faced, ranging from practical issues such as handling and administration of medication to broader concerns about treatment access and continuity, as well as key knowledge gaps among patients, caregivers, and clinicians. Recommendations are provided to enhance the patient and caregiver journeys, including increasing development and availability of educational materials, providing opportunities for patient advocacy by clinicians and health care providers, and encouraging institutional improvements to ensure that patients continue to receive uninterrupted treatment during their critical period of growth.

儿童生长激素缺乏症(GHD)是一种罕见疾病,给患者和照护者都带来了沉重的负担。虽然以往的报告详细介绍了患者及其护理人员所承受的各方面负担,但从患者和护理人员各自的角度出发,了解他们心路历程的第一手资料却非常缺乏。为了满足这一需求,我们于 2022 年 9 月 30 日召开了一次咨询委员会会议,与 4 名儿科 GHD 患者及其护理人员讨论了他们在 GHD 诊断前、诊断过程中以及正在接受重组生长激素治疗期间的经历。儿科内分泌专家对患者和护理人员的反馈意见进行了审查,并根据所报告的信息对患者和护理人员的心路历程提出了自己的看法。尽管样本量较小,但我们还是获得了重要的启示:患者和护理人员报告了治疗对生长的显著益处,这为坚持每日注射提供了强大的动力。患者和照护者的陈述反映出不同家庭从怀疑到确诊的时间以及所面临的治疗挑战存在很大差异,这些挑战既包括处理和用药等实际问题,也包括对治疗机会和持续性的更广泛关注,还包括患者、照护者和临床医生之间的主要知识差距。本报告提出了一些建议,以改善患者和护理人员的治疗过程,包括增加教育材料的开发和供应,为临床医生和医疗服务提供者提供为患者宣传的机会,以及鼓励机构改进以确保患者在其成长的关键时期继续接受不间断的治疗。
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引用次数: 0
Growth hormone treatment in aged patients with comorbidities: A systematic review 合并症老年患者的生长激素治疗:系统综述
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-03-09 DOI: 10.1016/j.ghir.2024.101584
Olivia Tausendfreund, Martin Bidlingmaier, Sebastian Martini, Katharina Müller, Michaela Rippl, Katharina Schilbach, Ralf Schmidmaier, Michael Drey

Objective

Hormonal substitution with growth hormone in aged patients remains a debated research topic and is rarely initiated in clinical practice. This reluctance may originate from concerns about adverse effects and the uncritical use as an anti-aging agent. Nevertheless, beneficial effects for selected patients suffering from certain acute and chronic illnesses could justify its use at an advanced age. This systematic review analyzes randomized controlled studies of GH interventions in older patients with different comorbidities to assess both, beneficial and harmful effects.

Design

A systematic search strategy was implemented to identify relevant studies from PubMed, MEDLINE, and The Cochrane Library. Inclusion criteria: participants aged over 65 years, randomized controlled trials involving human growth hormone (GH) and presence of at least one additional comorbidity independent of a flawed somatotropic axis.

Results

The eight eligible studies encompassed various comorbidities including osteoporosis, frailty, chronic heart failure, hip fracture, amyotrophic lateral sclerosis and hemodialysis. Outcomes varied, including changes in body composition, physical performance, strength, bone mineral density, cardiovascular parameters, quality of life and housing situation. Study protocols differed greatly in GH application frequency (daily, 2nd day or 3×/week), doses (0.41 mg-2.6 mg; mean 1.3 mg per 60 kg patient) and duration (1-12 months; mean 7 months). Mild dose-related side effects were reported, alongside noticeable positive impacts particularly on body composition, functionality, and quality of life.

Conclusion

Despite limited evidence, GH treatment might offer diverse benefits with few adverse effects. Further research with IGF-I dependent indication and clear outcomes, incorporating IGF-I dependent GH titration in older adults is warranted.

目的对老年患者使用生长激素进行激素替代仍是一个备受争议的研究课题,在临床实践中也很少使用。这种不情愿可能源于对不良反应的担忧,以及将生长激素作为抗衰老药物使用的不严谨态度。然而,对某些急性和慢性疾病患者的有益作用可以证明高龄患者使用激素是合理的。本系统性综述分析了对患有不同合并症的老年患者进行 GH 干预的随机对照研究,以评估其有益和有害影响。设计 采用系统性检索策略,从 PubMed、MEDLINE 和 Cochrane 图书馆中查找相关研究。纳入标准:参与者年龄超过 65 岁,随机对照试验涉及人生长激素 (GH),且至少有一种额外的合并症与有缺陷的体液轴无关。结果八项符合条件的研究涵盖了各种合并症,包括骨质疏松症、虚弱、慢性心力衰竭、髋部骨折、肌萎缩性脊髓侧索硬化症和血液透析。研究结果各不相同,包括身体成分、体能、力量、骨质密度、心血管参数、生活质量和住房情况的变化。研究方案在使用 GH 的频率(每天、第 2 天或每周 3 次)、剂量(0.41 毫克-2.6 毫克;平均每 60 公斤患者 1.3 毫克)和持续时间(1-12 个月;平均 7 个月)方面存在很大差异。尽管证据有限,但 GH 治疗可带来多种益处,且不良反应较少。有必要进一步研究 IGF-I 依赖性适应症和明确的结果,并在老年人中采用 IGF-I 依赖性 GH 滴定法。
{"title":"Growth hormone treatment in aged patients with comorbidities: A systematic review","authors":"Olivia Tausendfreund,&nbsp;Martin Bidlingmaier,&nbsp;Sebastian Martini,&nbsp;Katharina Müller,&nbsp;Michaela Rippl,&nbsp;Katharina Schilbach,&nbsp;Ralf Schmidmaier,&nbsp;Michael Drey","doi":"10.1016/j.ghir.2024.101584","DOIUrl":"https://doi.org/10.1016/j.ghir.2024.101584","url":null,"abstract":"<div><h3>Objective</h3><p>Hormonal substitution with growth hormone in aged patients remains a debated research topic and is rarely initiated in clinical practice. This reluctance may originate from concerns about adverse effects and the uncritical use as an anti-aging agent. Nevertheless, beneficial effects for selected patients suffering from certain acute and chronic illnesses could justify its use at an advanced age. This systematic review analyzes randomized controlled studies of GH interventions in older patients with different comorbidities to assess both, beneficial and harmful effects.</p></div><div><h3>Design</h3><p>A systematic search strategy was implemented to identify relevant studies from PubMed, MEDLINE, and The Cochrane Library. Inclusion criteria: participants aged over 65 years, randomized controlled trials involving human growth hormone (GH) and presence of at least one additional comorbidity independent of a flawed somatotropic axis.</p></div><div><h3>Results</h3><p>The eight eligible studies encompassed various comorbidities including osteoporosis, frailty, chronic heart failure, hip fracture, amyotrophic lateral sclerosis and hemodialysis. Outcomes varied, including changes in body composition, physical performance, strength, bone mineral density, cardiovascular parameters, quality of life and housing situation. Study protocols differed greatly in GH application frequency (daily, 2nd day or 3×/week), doses (0.41 mg-2.6 mg; mean 1.3 mg per 60 kg patient) and duration (1-12 months; mean 7 months). Mild dose-related side effects were reported, alongside noticeable positive impacts particularly on body composition, functionality, and quality of life.</p></div><div><h3>Conclusion</h3><p>Despite limited evidence, GH treatment might offer diverse benefits with few adverse effects. Further research with IGF-I dependent indication and clear outcomes, incorporating IGF-I dependent GH titration in older adults is warranted.</p></div>","PeriodicalId":12803,"journal":{"name":"Growth Hormone & Igf Research","volume":"75 ","pages":"Article 101584"},"PeriodicalIF":1.4,"publicationDate":"2024-03-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140134209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Duration-specific association between plasma IGFBP7 levels and diabetic complications in patients with type 2 diabetes mellitus 2 型糖尿病患者血浆 IGFBP7 水平与糖尿病并发症持续时间的关系
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-02-21 DOI: 10.1016/j.ghir.2024.101574
Xiaoyan Zhu , Fei Liang , Jieyun Yin , Xiaoliang Li , Lai Jiang , Yan Gao , Yan Lu , Yihe Hu , Ningbin Dai , Jian Su , Zhuoqiao Yang , Mengxin Yao , Yue Xiao , Wenxin Ge , Yue Zhang , Yi Zhong , Jun Zhang , Ming Wu

Objective

Insulin-like growth factor binding protein 7 (IGFBP7) has a strong affinity to insulin. This study aimed to evaluate the relationship between IGFBP7 and complications among type 2 diabetes mellitus (T2DM) patients.

Design

A total of 1449 T2DM patients were selected from a cross-sectional study for disease management registered in the National Basic Public Health Service in Changshu, China, and further tested for their plasma IGFBP7 levels. Logistic regressions and Spearman's rank correlation analyses were used to explore the associations of IGFBP7 with diabetic complications and clinical characteristics, respectively.

Results

Among the 1449 included T2DM patients, 403 (27.81%) had complications. In patients with shorter duration (less than five years), the base 10 logarithms of IGFBP7 concentration were associated with T2DM complications, with an adjusted odds ratio (OR) of 2.41 [95% confidence interval (95%CI) = 1.06–5.48]; while in patients with longer duration (more than five years), plasma IGFBP7 levels were not associated with T2DM complications. Furthermore, in T2DM patients with shorter duration, those with two or more types of complications were more likely to have higher levels of IGFBP7.

Conclusion

IGFBP7 is positively associated with the risk of complication in T2DM patients with shorter duration.

目的胰岛素样生长因子结合蛋白7(IGFBP7)与胰岛素有很强的亲和力。设计从常熟市国家基本公共卫生服务项目疾病管理横断面研究中选取 1449 例 T2DM 患者,进一步检测其血浆 IGFBP7 水平。结果 在纳入的1449例T2DM患者中,403例(27.81%)有并发症。在病程较短(少于五年)的患者中,IGFBP7浓度的基10对数与T2DM并发症有关,调整后的比值比(OR)为2.41[95%置信区间(95%CI)=1.06-5.48];而在病程较长(五年以上)的患者中,血浆IGFBP7水平与T2DM并发症无关。结论IGFBP7与病程较短的T2DM患者的并发症风险呈正相关。
{"title":"Duration-specific association between plasma IGFBP7 levels and diabetic complications in patients with type 2 diabetes mellitus","authors":"Xiaoyan Zhu ,&nbsp;Fei Liang ,&nbsp;Jieyun Yin ,&nbsp;Xiaoliang Li ,&nbsp;Lai Jiang ,&nbsp;Yan Gao ,&nbsp;Yan Lu ,&nbsp;Yihe Hu ,&nbsp;Ningbin Dai ,&nbsp;Jian Su ,&nbsp;Zhuoqiao Yang ,&nbsp;Mengxin Yao ,&nbsp;Yue Xiao ,&nbsp;Wenxin Ge ,&nbsp;Yue Zhang ,&nbsp;Yi Zhong ,&nbsp;Jun Zhang ,&nbsp;Ming Wu","doi":"10.1016/j.ghir.2024.101574","DOIUrl":"10.1016/j.ghir.2024.101574","url":null,"abstract":"<div><h3>Objective</h3><p>Insulin-like growth factor binding protein 7 (IGFBP7) has a strong affinity to insulin. This study aimed to evaluate the relationship between IGFBP7 and complications among type 2 diabetes mellitus (T2DM) patients.</p></div><div><h3>Design</h3><p>A total of 1449 T2DM patients were selected from a cross-sectional study for disease management registered in the National Basic Public Health Service in Changshu, China, and further tested for their plasma IGFBP7 levels. Logistic regressions and Spearman's rank correlation analyses were used to explore the associations of IGFBP7 with diabetic complications and clinical characteristics, respectively.</p></div><div><h3>Results</h3><p>Among the 1449 included T2DM patients, 403 (27.81%) had complications. In patients with shorter duration (less than five years), the base 10 logarithms of IGFBP7 concentration were associated with T2DM complications, with an adjusted odds ratio (OR) of 2.41 [95% confidence interval (95%CI) = 1.06–5.48]; while in patients with longer duration (more than five years), plasma IGFBP7 levels were not associated with T2DM complications. Furthermore, in T2DM patients with shorter duration, those with two or more types of complications were more likely to have higher levels of IGFBP7.</p></div><div><h3>Conclusion</h3><p>IGFBP7 is positively associated with the risk of complication in T2DM patients with shorter duration.</p></div>","PeriodicalId":12803,"journal":{"name":"Growth Hormone & Igf Research","volume":"75 ","pages":"Article 101574"},"PeriodicalIF":1.4,"publicationDate":"2024-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139921618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Evaluation of growth hormone deficiency in women with unexplained infertility 评估不明原因不孕妇女的生长激素缺乏症
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-02-01 DOI: 10.1016/j.ghir.2024.101571
Mehmet Serif Ortac , Aysa Hacioglu , Semih Zeki Uludag , Zuleyha Karaca , Kursad Unluhizarci , Fahrettin Kelestimur , Fatih Tanriverdi

Purpose

Growth hormone (GH) has been recognized to play a regulatory role in female reproduction. It has been reported that infertile GH deficient patients regained fertility after GH replacement. The frequency of GH deficiency is not established in patients diagnosed with unexplained infertility. Here, we aim to present the prevalence of GH deficieny in this patient group.

Methods

We included patients diagnosed with unexplained infertility throughout 18 months. Insulin tolerance test (ITT) and glucagon stimulation tests (GST) were performed and insufficient response to both tests was required for the diagnosis of GH deficiency.

Results

Twenty-five patients were included in the study, the mean age was 27.4 ± 4.5 years and the median duration of infertility was 60 months (min:14, max:120). Two patients were GH deficient according to GST and 14 to ITT. Two patients (8%) showed lack of response on both tests and were diagnosed with GH deficiency.

Conclusion

The rate of GH deficiency among women with unexplained infertility was 8% in this preliminary study. There is need for further studies with larger patient groups to verify the results.

目的生长激素(GH)被认为在女性生殖过程中发挥着调节作用。有报道称,缺乏 GH 的不孕症患者在补充 GH 后可恢复生育能力。在诊断为不明原因不孕症的患者中,GH缺乏症的发病率尚未确定。在此,我们旨在介绍 GH 缺乏症在这一患者群体中的发病率。结果25名患者被纳入研究,平均年龄为(27.4±4.5)岁,中位不孕时间为60个月(最小14个月,最大120个月)。根据 GST,2 名患者缺乏 GH,根据 ITT,14 名患者缺乏 GH。结论在这项初步研究中,不明原因不孕症妇女中的 GH 缺乏率为 8%。需要对更大的患者群体进行进一步研究,以验证研究结果。
{"title":"Evaluation of growth hormone deficiency in women with unexplained infertility","authors":"Mehmet Serif Ortac ,&nbsp;Aysa Hacioglu ,&nbsp;Semih Zeki Uludag ,&nbsp;Zuleyha Karaca ,&nbsp;Kursad Unluhizarci ,&nbsp;Fahrettin Kelestimur ,&nbsp;Fatih Tanriverdi","doi":"10.1016/j.ghir.2024.101571","DOIUrl":"10.1016/j.ghir.2024.101571","url":null,"abstract":"<div><h3>Purpose</h3><p><span>Growth hormone (GH) has been recognized to play a regulatory role in female reproduction. It has been reported that infertile GH deficient patients regained fertility after GH replacement. The frequency of GH deficiency is not established </span>in patients diagnosed with unexplained infertility. Here, we aim to present the prevalence of GH deficieny in this patient group.</p></div><div><h3>Methods</h3><p>We included patients diagnosed with unexplained infertility throughout 18 months. Insulin tolerance test<span> (ITT) and glucagon stimulation tests (GST) were performed and insufficient response to both tests was required for the diagnosis of GH deficiency.</span></p></div><div><h3>Results</h3><p>Twenty-five patients were included in the study, the mean age was 27.4 ± 4.5 years and the median duration of infertility was 60 months (min:14, max:120). Two patients were GH deficient according to GST and 14 to ITT. Two patients (8%) showed lack of response on both tests and were diagnosed with GH deficiency.</p></div><div><h3>Conclusion</h3><p>The rate of GH deficiency among women with unexplained infertility was 8% in this preliminary study. There is need for further studies with larger patient groups to verify the results.</p></div>","PeriodicalId":12803,"journal":{"name":"Growth Hormone & Igf Research","volume":"74 ","pages":"Article 101571"},"PeriodicalIF":1.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139562519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Insights from an advisory board: Facilitating transition of care into adulthood in brain cancer survivors with acquired pediatric growth hormone deficiency 咨询委员会的见解:促进后天性小儿生长激素缺乏症脑癌幸存者向成年期的护理过渡
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-02-01 DOI: 10.1016/j.ghir.2024.101573
Craig Alter , Margaret Boguszewski , David Clemmons , Georgiana A. Dobri , Mitchell E. Geffner , Nicky Kelepouris , Bradley S. Miller , Richard Oh , Heidi Shea , Kevin C.J. Yuen

Objective

Children with growth hormone deficiency (GHD) face multiple challenges that can negatively impact the transition from pediatric to adult endocrinology care. For children with GHD resulting from brain cancer or its treatment, the involvement of oncology care providers and possible disease-related comorbidities add further complexity to this transition.

Design

An advisory board of pediatric and adult endocrinologists was convened to help better understand the unique challenges faced by childhood cancer survivors with GHD, and discuss recommendations to optimize continuity of care as these patients proceed to adulthood. Topics included the benefits and risks of growth hormone (GH) therapy in cancer survivors, the importance of initiating GH replacement therapy early in the patient's journey and continuing into adulthood, and the obstacles that can limit an effective transition to adult care for these patients.

Results/conclusions

Some identified obstacles included the need to prioritize cancer treatment over treatment for GHD, a lack of patient and oncologist knowledge about the full range of benefits provided by long-term GH administration, concerns about tumor recurrence risk in cancer survivors receiving GH treatment, and suboptimal communication and coordination (e.g., referrals) between care providers, all of which could potentially result in treatment gaps or even complete loss of follow-up during the care transition. Advisors provided recommendations for increasing education for patients and care providers and improving coordination between treatment team members, both of which are intended to help improve continuity of care to maximize the health benefits of GH administration during the critical period when childhood cancer survivors transition into adulthood.

目的生长激素缺乏症(GHD)患儿面临着多种挑战,这些挑战可能会对从儿科内分泌治疗向成人内分泌治疗的过渡产生负面影响。设计召集了一个由儿科和成人内分泌专家组成的咨询委员会,以帮助更好地了解患有生长激素缺乏症的儿童癌症幸存者所面临的独特挑战,并讨论在这些患者进入成年期时优化护理连续性的建议。讨论的主题包括生长激素 (GH) 治疗对癌症幸存者的益处和风险、在患者早期开始 GH 替代治疗并持续到成年的重要性,以及可能限制这些患者有效过渡到成人护理的障碍。结果/结论已发现的一些障碍包括:需要优先考虑癌症治疗而不是 GHD 治疗;患者和肿瘤学家缺乏对长期服用 GH 所带来的全部益处的了解;担心接受 GH 治疗的癌症幸存者的肿瘤复发风险;以及医疗服务提供者之间的沟通和协调(如转诊)不够理想、转诊),所有这些都有可能导致治疗缺口,甚至在治疗过渡期间完全失去随访。顾问们建议加强对患者和护理提供者的教育,并改善治疗团队成员之间的协调,这两点都旨在帮助改善护理的连续性,以便在儿童癌症幸存者向成年过渡的关键时期最大限度地发挥GH治疗对健康的益处。
{"title":"Insights from an advisory board: Facilitating transition of care into adulthood in brain cancer survivors with acquired pediatric growth hormone deficiency","authors":"Craig Alter ,&nbsp;Margaret Boguszewski ,&nbsp;David Clemmons ,&nbsp;Georgiana A. Dobri ,&nbsp;Mitchell E. Geffner ,&nbsp;Nicky Kelepouris ,&nbsp;Bradley S. Miller ,&nbsp;Richard Oh ,&nbsp;Heidi Shea ,&nbsp;Kevin C.J. Yuen","doi":"10.1016/j.ghir.2024.101573","DOIUrl":"10.1016/j.ghir.2024.101573","url":null,"abstract":"<div><h3>Objective</h3><p>Children with growth hormone deficiency (GHD) face multiple challenges that can negatively impact the transition from pediatric to adult endocrinology care. For children with GHD resulting from brain cancer or its treatment, the involvement of oncology care providers and possible disease-related comorbidities add further complexity to this transition.</p></div><div><h3>Design</h3><p>An advisory board of pediatric and adult endocrinologists was convened to help better understand the unique challenges faced by childhood cancer survivors with GHD, and discuss recommendations to optimize continuity of care as these patients proceed to adulthood. Topics included the benefits and risks of growth hormone (GH) therapy in cancer survivors, the importance of initiating GH replacement therapy early in the patient's journey and continuing into adulthood, and the obstacles that can limit an effective transition to adult care for these patients.</p></div><div><h3>Results/conclusions</h3><p>Some identified obstacles included the need to prioritize cancer treatment over treatment for GHD, a lack of patient and oncologist knowledge about the full range of benefits provided by long-term GH administration, concerns about tumor recurrence risk in cancer survivors receiving GH treatment, and suboptimal communication and coordination (e.g., referrals) between care providers, all of which could potentially result in treatment gaps or even complete loss of follow-up during the care transition. Advisors provided recommendations for increasing education for patients and care providers and improving coordination between treatment team members, both of which are intended to help improve continuity of care to maximize the health benefits of GH administration during the critical period when childhood cancer survivors transition into adulthood.</p></div>","PeriodicalId":12803,"journal":{"name":"Growth Hormone & Igf Research","volume":"74 ","pages":"Article 101573"},"PeriodicalIF":1.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1096637424000030/pdfft?md5=096342c1834a83d56df592c8537ea9c5&pid=1-s2.0-S1096637424000030-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139897152","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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
Growth hormone directly stimulates GATA2 expression 生长激素直接刺激 GATA2 的表达
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2024-02-01 DOI: 10.1016/j.ghir.2024.101572
Mana Mitsutani , Midori Matsushita , Mei Yokoyama , Ayumu Morita , Hiromi Hano , Tomomi Fujikawa , Tetsuya Tagami , Kenji Moriyama

Objective

GATA2 is a key transcription factor involved in the differentiation and determination of thyrotrophs and gonadotrophs in pituitary and hematopoietic development. However, studies on the upstream ligands of the GATA2 signal transduction pathway have been limited. To identify upstream ligands, we examined growth hormone (GH) as a plausible stimulator.

Design

We evaluated GH-induced GATA2 expression in murine TtT/GF thyrotrophic pituitary tumor cells and its direct impact on the GHR/JAK/STAT5 pathway using a combination of a reporter assay, real-time quantitative polymerase chain reaction, and western blotting.

Results

GATA2 expression increased with activated STAT5B in a dose-dependent manner and was inhibited by a STAT5 specific inhibitor. Moreover, we found functional STAT5B binding site consensus sequences at −359 bp in the GATA2 promoter region.

Conclusion

These findings suggest that GH directly stimulates GATA2 via the GHR/JAK/STAT pathway and participates in various developmental phenomena mediated by GATA2.

目的GATA2是一种关键的转录因子,参与垂体和造血发育过程中甲状腺和促性腺激素的分化和决定。然而,有关 GATA2 信号转导通路上游配体的研究还很有限。为了确定上游配体,我们将生长激素(GH)作为一种可能的刺激物进行了研究。结果GATA2的表达以剂量依赖的方式随活化的STAT5B而增加,并受到STAT5特异性抑制剂的抑制。此外,我们在 GATA2 启动子区域的 -359 bp 处发现了功能性 STAT5B 结合位点共识序列。
{"title":"Growth hormone directly stimulates GATA2 expression","authors":"Mana Mitsutani ,&nbsp;Midori Matsushita ,&nbsp;Mei Yokoyama ,&nbsp;Ayumu Morita ,&nbsp;Hiromi Hano ,&nbsp;Tomomi Fujikawa ,&nbsp;Tetsuya Tagami ,&nbsp;Kenji Moriyama","doi":"10.1016/j.ghir.2024.101572","DOIUrl":"10.1016/j.ghir.2024.101572","url":null,"abstract":"<div><h3>Objective</h3><p><span>GATA2 is a key transcription factor involved in the differentiation and determination of thyrotrophs and </span>gonadotrophs<span> in pituitary and hematopoietic development. However, studies on the upstream ligands of the GATA2 signal transduction pathway have been limited. To identify upstream ligands, we examined growth hormone (GH) as a plausible stimulator.</span></p></div><div><h3>Design</h3><p>We evaluated GH-induced GATA2 expression in murine TtT/GF thyrotrophic pituitary tumor<span> cells and its direct impact on the GHR/JAK/STAT5 pathway using a combination of a reporter assay, real-time quantitative polymerase chain reaction, and western blotting.</span></p></div><div><h3>Results</h3><p>GATA2 expression increased with activated STAT5B<span> in a dose-dependent manner and was inhibited by a STAT5 specific inhibitor. Moreover, we found functional STAT5B binding site consensus sequences at −359 bp in the GATA2 promoter region.</span></p></div><div><h3>Conclusion</h3><p>These findings suggest that GH directly stimulates GATA2 via the GHR/JAK/STAT pathway and participates in various developmental phenomena mediated by GATA2.</p></div>","PeriodicalId":12803,"journal":{"name":"Growth Hormone & Igf Research","volume":"74 ","pages":"Article 101572"},"PeriodicalIF":1.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139562520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Growth hormone therapy for children with Duchenne muscular dystrophy and glucocorticoid induced short stature 生长激素治疗Duchenne肌营养不良和糖皮质激素诱导的矮小儿童
IF 1.4 4区 医学 Q4 CELL BIOLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.ghir.2023.101558
Eran Lavi , Amitay Cohen , Abdulsalam Abu Libdeh , Reuven Tsabari , David Zangen , Talya Dor

Objective

To evaluate the outcome of recombinant human Growth Hormone (rhGH) therapy in patients with Duchene Muscular Dystrophy (DMD) and glucocorticoid treatment with compromised growth.

Design

Four DMD patients on Deflzacort 0.6–0.85 mg/kg/day or prednisolone 0.625 mg/kg/day recieved rhGH (0.24 mg/kg/week) for 6–18 months. Primary outcomes were Growth velocity and Height for age Z-scores (Height SD).

Results

Growth velocity increased from 0 to 3.25 cm/year prior to GH therapy to 3.3–7.8 cm/year over a period of 6–18 months. The typical Height SD decline in DMD was reversed in two patients and blunted in one. No adverse events or deterioration in cardiac or respiratory parameters were associated with the rhGH treatment.

Conclusions

rhGH appears to be safe and efficient in promoting growth of patients with glucocorticoid induced growth failure in DMD.

目的评价重组人生长激素(rhGH)治疗杜氏肌营养不良(DMD)和糖皮质激素治疗生长障碍的疗效。设计4名DMD患者接受0.6–0.85 mg/kg/天的替扎卡或0.625 mg/kg/天泼尼松治疗,接受rhGH(0.24 mg/kg/周)治疗6–18个月。主要结果是生长速度和身高Z评分(Height SD)。结果生长速度从生长激素治疗前的0至3.25 cm/年增加到6至18个月的3.3至7.8 cm/年。DMD的典型身高SD下降在两名患者中逆转,在一名患者中减弱。rhGH治疗无不良事件或心脏或呼吸参数恶化。结论srhGH对糖皮质激素诱导的DMD生长衰竭患者的生长具有安全有效的促进作用。
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Growth Hormone & Igf Research
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