A case series of bilateral inferior petrosal sinus sampling using desmopressin for evaluation of ACTH-dependent cushing's syndrome in pediatric patients: insights from Iran.
{"title":"A case series of bilateral inferior petrosal sinus sampling using desmopressin for evaluation of ACTH-dependent cushing's syndrome in pediatric patients: insights from Iran.","authors":"Majid Valizadeh, Fatemeh Rahmani, Mohammad Nikoohemmat, Arash Khameneh Bagheri, Behnaz Abiri","doi":"10.1007/s40200-025-01634-4","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Pediatric Cushing Syndrome (CS) is rare and difficult to diagnose, especially when distinguishing ACTH-dependent subtypes. Bilateral inferior petrosal sinus sampling (BIPSS) is an essential but technically challenging procedure for this purpose. Because corticotropin-releasing hormone (CRH), the standard stimulant, has limitations, desmopressin is being explored as an alternative. This study assesses desmopressin-stimulated BIPSS for its diagnostic accuracy and tumor localization in pediatric CS within an Iranian cohort, addressing a gap in pediatric-specific diagnostic strategies and offering insights into the applicability of desmopressin in this context.</p><p><strong>Methods: </strong>Four pediatric patients with inconclusive pituitary imaging and suspected Cushing's disease (CD) underwent BIPSS with desmopressin at Taleghani Hospital, Tehran, Iran, between August 2015 and March 2019. Sensitivity of BIPSS for CD diagnosis was assessed, and tumor localization accuracy was evaluated during surgery.</p><p><strong>Results: </strong>Bilateral IPSS demonstrated a sensitivity of 100% for diagnosing CD in pediatric patients. However, accuracy for tumor lateralization was moderate, with only 50% concordance between BIPSS lateralization and surgical findings. Specifically, two out of four patients had correct lateralization confirmed during surgery, while one patient with left lateralization was consistent with hypophysectomy findings. These discrepancies highlight challenges such as anatomical and drainage variations that can lead to mislocalization.</p><p><strong>Conclusion: </strong>Desmopressin enhances the sensitivity of BIPSS for diagnosing pediatric CD, presenting as a viable alternative to CRH stimulation. Despite high sensitivity, caution is advised when interpreting BIPSS results for tumor localization. Further research is needed to optimize diagnostic strategies for pediatric CS management.</p>","PeriodicalId":15635,"journal":{"name":"Journal of Diabetes and Metabolic Disorders","volume":"24 1","pages":"125"},"PeriodicalIF":1.8000,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12098220/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Diabetes and Metabolic Disorders","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s40200-025-01634-4","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/6/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"ENDOCRINOLOGY & METABOLISM","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Pediatric Cushing Syndrome (CS) is rare and difficult to diagnose, especially when distinguishing ACTH-dependent subtypes. Bilateral inferior petrosal sinus sampling (BIPSS) is an essential but technically challenging procedure for this purpose. Because corticotropin-releasing hormone (CRH), the standard stimulant, has limitations, desmopressin is being explored as an alternative. This study assesses desmopressin-stimulated BIPSS for its diagnostic accuracy and tumor localization in pediatric CS within an Iranian cohort, addressing a gap in pediatric-specific diagnostic strategies and offering insights into the applicability of desmopressin in this context.
Methods: Four pediatric patients with inconclusive pituitary imaging and suspected Cushing's disease (CD) underwent BIPSS with desmopressin at Taleghani Hospital, Tehran, Iran, between August 2015 and March 2019. Sensitivity of BIPSS for CD diagnosis was assessed, and tumor localization accuracy was evaluated during surgery.
Results: Bilateral IPSS demonstrated a sensitivity of 100% for diagnosing CD in pediatric patients. However, accuracy for tumor lateralization was moderate, with only 50% concordance between BIPSS lateralization and surgical findings. Specifically, two out of four patients had correct lateralization confirmed during surgery, while one patient with left lateralization was consistent with hypophysectomy findings. These discrepancies highlight challenges such as anatomical and drainage variations that can lead to mislocalization.
Conclusion: Desmopressin enhances the sensitivity of BIPSS for diagnosing pediatric CD, presenting as a viable alternative to CRH stimulation. Despite high sensitivity, caution is advised when interpreting BIPSS results for tumor localization. Further research is needed to optimize diagnostic strategies for pediatric CS management.
期刊介绍：
Journal of Diabetes & Metabolic Disorders is a peer reviewed journal which publishes original clinical and translational articles and reviews in the field of endocrinology and provides a forum of debate of the highest quality on these issues. Topics of interest include, but are not limited to, diabetes, lipid disorders, metabolic disorders, osteoporosis, interdisciplinary practices in endocrinology, cardiovascular and metabolic risk, aging research, obesity, traditional medicine, pychosomatic research, behavioral medicine, ethics and evidence-based practices.As of Jan 2018 the journal is published by Springer as a hybrid journal with no article processing charges. All articles published before 2018 are available free of charge on springerlink.Unofficial 2017 2-year Impact Factor: 1.816.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
