Article
Veterinary Sciences
Elber A. Soler Arias, Victor A. Castillo, Estelle Louiset, Herve Lefebvre
Summary: This case report describes a 13-year-old Labrador retriever diagnosed with Cushing's syndrome caused by primary bilateral nodular adrenocortical hyperplasia with adrenocorticotropic hormone (ACTH) expression. The study found that a disease similar to primary bilateral macronodular adrenocortical hyperplasia in humans also occurs in dogs, with intra-adrenocortical expression of ACTH, glucocorticoids excess, and clinical signs of CS.
JOURNAL OF VETERINARY INTERNAL MEDICINE
(2022)
Article
Endocrinology & Metabolism
Andreea Liliana Serban, Lorenzo Rosso, Paolo Mendogni, Arianna Cremaschi, Rita Indirli, Beatrice Mantovani, Mariagrazia Rumi, Massimo Castellani, Arturo Chiti, Giorgio Alberto Croci, Giovanna Mantovani, Mario Nosotti, Emanuele Ferrante, Maura Arosio
Summary: This report describes a case of severe hypercortisolism due to EAS which was not detected by functional imaging methods, with the localization of ACTH ectopic origin being puzzled by a concomitant metastatic rectal carcinoma. A multidisciplinary approach was crucial for the management of this rare disease.
FRONTIERS IN ENDOCRINOLOGY
(2021)
Article
Medicine, General & Internal
Hironori Abe, Hirotsugu Suwanai, Nodoka Kambara, Koji Sano, Junpei Shikuma, Hiroaki Akaoka, Akira Kanazawa, Hideaki Hirai, Takashi Miwa, Toshitaka Nagao, Masato Odawara
Summary: A 40-year-old woman with recurrent olfactory neuroblastoma developed ectopic ACTH syndrome, characterized by morbidly elevated levels of ACTH and cortisol. Treatment with metyrapone to suppress cortisol production and resection of ACTH-producing tumor led to alleviation of Cushing's syndrome symptoms.
Article
Medicine, General & Internal
Marta Piasecka, Martin Larsson, Eleni Papakokkinou, Lena Olsson, Oskar Ragnarsson
Summary: The study aimed to determine whether ECS is underdiagnosed in patients with SCLC. The results showed that out of 213 SCLC patients, one had confirmed ECS, two had probable ECS, and twenty-three had possibly ECS. Patients with possibly or probable ECS had significantly shorter survival.
FRONTIERS IN MEDICINE
(2022)
Article
Oncology
Xiao Zhang, Liangliang Meng, Yueyong Xiao, Zenan Chen
Summary: This study reports a case of suspected pulmonary carcinoid tumor with difficulty in surgical resection and poor response to drug therapy. The patient was successfully treated with radiofrequency ablation combined with intraoperative biopsy of the lesion, leading to improvement of high cortisol symptoms.
FRONTIERS IN ONCOLOGY
(2022)
Review
Endocrinology & Metabolism
Fernando Guerrero-Perez, Inmaculada Peiro, Agustina Pia Marengo, Alex Teule, Jose Carlos Ruffinelli, Roger Llatjos, Teresa Serrano, Ivan Macia, Nuria Vilarrasa, Pedro Iglesias, Carles Villabona
Summary: Ectopic Cushing's syndrome caused by thymic neuroendocrine tumors presents as aggressive hypercortisolism in middle-aged populations. The disease is commonly diagnosed in advanced stages, with persistent or recurrent tumors and a high short-term mortality rate after surgery.
REVIEWS IN ENDOCRINE & METABOLIC DISORDERS
(2021)
Article
Biology
Xuebin Zhang, Penghu Lian, Mingming Su, Zhigang Ji, Jianhua Deng, Guoyang Zheng, Wenda Wang, Xinyu Ren, Taijiao Jiang, Peng Zhang, Hanzhong Li
Summary: In this study, single-cell RNA sequencing was used to analyze a rare case of ectopic ACTH&CRH-secreting pheochromocytoma, leading to the identification of a novel multi-functionally chromaffin-like cell type. The molecular mechanism behind Cushing's syndrome in this rare case was hypothesized to involve this newly identified tumor cell type. Immunohistochemistry results confirmed the unique properties of this cell type, along with the heterogeneous endothelial and immune microenvironment in different adrenal tumor subtypes.
Article
Veterinary Sciences
Carlos Melian, Beatriz Blanco, Pedro J. Ginel, Laura Perez-Lopez
Summary: This study proposed a reference interval for cortisol values 1-hour after administration of a low-dose of depot ACTH in healthy dogs and evaluated the sensitivity of this test to detect Cushing's Syndrome and differentiate among different types of Cushing's Syndrome based on ultrasound findings.
RESEARCH IN VETERINARY SCIENCE
(2022)
Article
Endocrinology & Metabolism
Henrik Elenius, Raven McGlotten, Lynnette K. Nieman
Summary: Determining the etiology of ACTH-dependent Cushing's syndrome can be difficult. The corticotropin-releasing hormone stimulation test and the high-dose dexamethasone suppression test have good performance in distinguishing Cushing's disease from ectopic ACTH syndrome. The combination of these tests can accurately diagnose approximately 64% of patients with Cushing's disease, obviating the need for the expensive and less widely available inferior petrosal sinus sampling (IPSS).
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2023)
Article
Medical Laboratory Technology
Jacquelien J. Hillebrand, Li Zhou, Marilee A. Marcinkus, Maria Datwyler, Susan H. Gawel, Frans Martens, Gerard J. Davis, Annemieke C. Heijboer
Summary: This study investigated the long-term storage stability of corticotropin in plasma. The results showed that corticotropin levels remained stable when stored at -20 degrees C for one and a half years, but a significant reduction was observed in patient samples stored for up to 6 years at the same temperature.
CLINICAL CHEMISTRY AND LABORATORY MEDICINE
(2022)
Review
Oncology
Shinobu Takayasu, Kazunori Kageyama, Makoto Daimon
Summary: Cushing's disease is caused by autonomous secretion of adrenocorticotropic hormone (ACTH) from corticotroph pituitary neuroendocrine tumors, leading to excess cortisol production. It presents with a variety of clinical features, from overt to subtle, caused by hormonal activities. Understanding the molecular and genetic causes of Cushing's disease is essential for its treatment. This review discusses recent advances in molecular insights and targeted therapy for Cushing's disease.
Article
Endocrinology & Metabolism
Rosario Pivonello, Pamela N. Munster, Massimo Terzolo, Rosario Ferrigno, Chiara Simeoli, Soraya Puglisi, Utsav Bali, Andreas G. Moraitis
Summary: Somatostatin inhibits pituitary hormone secretion, and glucocorticoids can downregulate SSTR2 expression, resulting in the ineffectiveness of traditional SSTR2-targeting analogs in ACTH-secreting NETs. However, GR modulation with relacorilant can reverse the glucocorticoid-induced downregulation of SSTR2, leading to increased SSTR2 expression.
FRONTIERS IN ENDOCRINOLOGY
(2022)
Article
Endocrinology & Metabolism
Tobias Skrebsky de Almeida, Ticiana da Costa Rodrigues, Fabiola Costenaro, Leandro Armani Scaffaro, Mauricio Farenzena, Fernando Gastaldo, Mauro Antonio Czepielewski
Summary: This retrospective cohort study aimed to assess the accuracy of the inferior petrosal sinus to peripheral ACTH gradient (IPS:P) before and after desmopressin stimulation for the differential diagnosis of ACTH-dependent Cushing's syndrome (CS). The results showed that the application of desmopressin stimulation improved the diagnostic accuracy of IPS:P, making it a valuable tool for investigating the differential diagnosis of ACTH-dependent CS.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Biotechnology & Applied Microbiology
Yan Zhang, Danrong Wu, Ruoqiu Wang, Min Luo, Dong Wang, Kaiyue Wang, Yi Ai, Li Zheng, Qiao Zhang, Lixin Shi
Summary: This article reports a rare case of ectopic ACTH-secreting pituitary adenoma in the clival region. In addition to the more common organs, special attention should be paid to ectopic ACTH-secreting pituitary adenomas in the clivus region when diagnosing ectopic ACTH syndrome.
ONCOTARGETS AND THERAPY
(2022)
Article
Biochemistry & Molecular Biology
Dongxu Hu, Jiaqin Li, Yuan Zhuang, Xiaoyan Mao
Summary: ACTH has been used in the treatment of nephrotic syndrome, especially in refractory cases, but there is currently a lack of research evidence supporting its effectiveness.
Article
Genetics & Heredity
F. M. van Haalen, M. Kaya, I. C. M. Pelsma, O. M. Dekkers, N. R. Biermasz, S. C. Cannegieter, M. V. Huisman, B. J. M. van Vlijmen, R. A. Feelders, F. A. Klok, A. M. Pereira
Summary: There is a large practice variation regarding thromboprophylaxis management in patients with Cushing's syndrome, even in Endo-ERN reference centers.
ORPHANET JOURNAL OF RARE DISEASES
(2022)
Article
Endocrinology & Metabolism
Leontine E. H. Bakker, Marco J. T. Verstegen, Eidrees Ghariq, Berit M. Verbist, Pieter J. Schutte, Waiel A. Bashari, Mark C. Kruit, Alberto M. Pereira, Mark Gurnell, Nienke R. Biermasz, Wouter R. van Furth, Lenka M. Pereira Arias Bouda
Summary: Met-PET/MRICR can provide additional information to guide multidisciplinary preoperative and intraoperative decision making in selected cases of difficult to treat prolactinoma.
Review
Endocrinology & Metabolism
Amir H. Zamanipoor Najafabadi, Merel van der Meulen, Ana Luisa Priego Zurita, S. Faisal Ahmed, Wouter R. van Furth, Evangelia Charmandari, Olaf Hiort, Alberto M. Pereira, Mehul Dattani, Diana Vitali, Johan P. de Graaf, Nienke R. Biermasz
Summary: The European Reference Network on Rare Endocrine Conditions (Endo-ERN) aims to improve healthcare for pituitary adenoma patients in Europe. This study reviewed surgical outcomes of pituitary adenoma patients within Endo-ERN MTG6 reference centers between 2010 and 2019. The study found that the remission rates varied for different types of adenomas, and a majority of centers used endoscopic surgery with the collaboration of ENT surgeons.
ENDOCRINE CONNECTIONS
(2023)
Article
Cardiac & Cardiovascular Systems
Yoshitaka Kimura, Marta de Riva, Micaela Ebert, Claire Glashan, Adrianus P. Wijnmaalen, Sebastiaan R. Piers, Olaf M. Dekkers, Serge A. Trines, Katja Zeppenfeld
Summary: In dilated cardiomyopathy (DCM), pleomorphic VT (PL-VT) is not associated with mortality but predicts VT recurrence independent from LVEF, indicating a specific arrhythmic substrate difficult to control by current ablation techniques. In ischemic heart disease (IHD), PL-VT is associated with low LVEF and mortality.
CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY
(2023)
Article
Endocrinology & Metabolism
Eva M. G. Viho, Jan Kroon, Richard A. Feelders, Rene Houtman, Elisabeth S. R. van den Dungen, Alberto M. Pereira, Hazel J. Hunt, Leo J. Hofland, Onno C. Meijer
Summary: Glucocorticoid stress hormones are essential for physiology and exert numerous actions via binding to the glucocorticoid receptor (GR). Relacorilant is a selective GR antagonist being evaluated for the treatment of endogenous Cushing's syndrome. This study compared the effects of relacorilant and the non-selective antagonist mifepristone, showing that relacorilant has potent antagonistic effects on GR signaling and prevents anti-inflammatory effects of glucocorticoids.
JOURNAL OF ENDOCRINOLOGY
(2023)
Article
Public, Environmental & Occupational Health
Jungyeon Choi, Olaf M. Dekkers, Saskia le Cessie
Summary: Ill-defined research questions can cause problems in epidemiological studies where measurements are subject to fluctuations due to events like medication use. Failing to specify how medication use should be handled during analysis can lead to arbitrary decisions and a disconnect between the intended question and analysis, resulting in different or meaningless interpretations of effects. This study discusses the formulation of well-defined research questions when medication use is involved, distinguishing between scenarios where exposure or outcome is affected by medication use and addressing methodological considerations under each question.
PHARMACOEPIDEMIOLOGY AND DRUG SAFETY
(2023)
Article
Endocrinology & Metabolism
Hanna F. Nowotny, Jillian Bryce, Salma R. Ali, Roberta Giordano, Federico Baronio, Irina Chifu, Lea Tschaidse, Martine Cools, Erica L. T. van den Akker, Henrik Falhammar, Natasha M. Appelman-Dijkstra, Luca Persani, Guglielmo Beccuti, Ian L. Ross, Simona Grozinsky-Glasberg, Alberto M. Pereira, Eystein S. Husebye, Stefanie Hahner, S. Faisal Ahmed, Nicole Reisch
Summary: This study is the first to collect data on the outcome of COVID-19 infection in patients with adrenal gland disorders, suggesting good clinical outcomes with appropriate dose adjustments and emphasizing the importance of patient education on sick day rules.
ENDOCRINE CONNECTIONS
(2023)
Article
Endocrinology & Metabolism
Savi R. Shishkov, Luigi Tuccillo, Violeta M. Iotova, Rosario Pivonello, Iris C. M. Pelsma, Alberto M. Pereira, Nienke R. Biermasz
Summary: Rare endocrine conditions pose challenges for healthcare providers, particularly in the transition of care, where there is a lack of consensus and guidelines. This study examined the current practices and unmet needs in transition of care for patients with pituitary disease in European reference centers. The study found that transition is commonly practiced, but with variations in criteria, assessment methods, and protocols, highlighting the need for a structured approach and EU-wide guidelines.
ENDOCRINE CONNECTIONS
(2023)
Article
Neurosciences
Philippe C. Habets, Konstantinos Kalafatakis, Oleh Dzyubachyk, Steven J. A. van der Werff, Arlin Keo, Jamini Thakrar, Ahmed Mahfouz, Alberto M. Pereira, Georgina M. Russell, Stafford L. Lightman, Onno C. Meijer
Summary: Recent evidence suggests that the ultradian cortisol pulsatility is crucial for normal emotional response in humans. This study investigates the anatomical and cell type signature of brain regions sensitive to a loss of ultradian rhythmicity in emotional processing. The findings highlight the importance of GABAergic function and specific genes in cortical brain areas for emotional processing response.
NEUROBIOLOGY OF STRESS
(2023)
Article
Surgery
Cyril P. Landstra, Michiel F. Nijhoff, Dave L. Roelen, Aiko P. J. de Vries, Eelco J. P. de Koning
Summary: Islet transplantation stabilizes glycemic control in patients with complicated diabetes mellitus, but rapid functional decline could be due to islet allograft rejection. Currently, there is no reliable method for assessing rejection and treatment protocols are lacking. This study aimed to identify diagnostic features of islet allograft rejection and evaluate the effectiveness of high-dose methylprednisolone treatment.
AMERICAN JOURNAL OF TRANSPLANTATION
(2023)
Review
Medicine, General & Internal
Tessa N. A. Slagboom, Dirk Jan Stenvers, Elsmarieke van de Giessen, Stefan D. D. Roosendaal, Maartje M. L. de Win, Joseph C. J. Bot, Eleonora Aronica, Rene Post, Jantien Hoogmoed, Madeleine L. L. Drent, Alberto M. M. Pereira
Summary: The study provides a structured analysis of the diagnostic accuracy and clinical use of molecular imaging in patients with ACTH-dependent Cushing's syndrome, as well as the role of multidisciplinary counseling in decision making. It proposes a complementary diagnostic algorithm for both de novo and recurrent or persistent Cushing's disease. The use of molecular imaging modalities is of added and complementary value in the detection of pituitary microadenoma in ACTH-dependent Cushing's syndrome.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Endocrinology & Metabolism
Stephan Petersenn, Maria Fleseriu, Felipe F. Casanueva, Andrea Giustina, Nienke Biermasz, Beverly M. K. Biller, Marcello Bronstein, Philippe Chanson, Hidenori Fukuoka, Monica Gadelha, Yona Greenman, Mark Gurnell, Ken K. Y. Ho, Juergen Honegger, Adriana G. Ioachimescu, Ursula B. Kaiser, Niki Karavitaki, Laurence Katznelson, Maya Lodish, Dominique Maiter, Hani J. Marcus, Ann Mccormack, Mark Molitch, Christopher A. Muir, Sebastian Neggers, Alberto M. Pereira, Rosario Pivonello, Kalmon Post, Gerald Raverot, Roberto Salvatori, Susan L. Samson, Ilan Shimon, Joanna Spencer-Segal, Greisa Vila, John Wass, Shlomo Melmed
Summary: This Consensus Statement provides evidence-based graded consensus recommendations and key summary points for clinical practice on the diagnosis and management of prolactinomas. It covers various aspects including epidemiology, pathogenesis, clinical presentation, assessment, imaging strategies, complications, and treatment in special situations. The workshop emphasizes the need for additional therapeutic options to address clinical challenges in treating these patients.
NATURE REVIEWS ENDOCRINOLOGY
(2023)
Review
Endocrinology & Metabolism
Violeta Iotova, Camilla Schalin-Jaentti, Charlotte Van Beuzekom, Petra Bruegmann, Manuela Broesamle, Olaf Hiort, Alberto M. Pereira
Summary: The current study assesses the development and impact of the knowledge generation program of the Endo-ERN, specifically focusing on webinars. The study finds that the program has successfully organized a significant number of webinars, engaging a large number of participants and attracting active participation and feedback. The findings highlight the importance and effectiveness of webinars in knowledge sharing and education in the field of rare endocrine conditions.
ENDOCRINE CONNECTIONS
(2023)
Review
Endocrinology & Metabolism
Anne-Sophie C. A. M. Koning, Merel van der Meulen, Daphne Schaap, Djaina D. Satoer, Christiaan H. Vinkers, Elisabeth F. C. van Rossum, Wouter R. van Furth, Alberto M. Pereira, Onno C. Meijer, Olaf M. Dekkers
Summary: The proportion of neuropsychiatric adverse effects in patients using synthetic glucocorticoids is high, with depression and mania being the most significant associations. Awareness of possible psychiatric side effects is essential when starting glucocorticoid treatment.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2023)
