Valizadeh Majid, Rahmani Fatemeh, Nikoohemmat Mohammad, Bagheri Arash Khameneh, Abiri Behnaz
Obesity Research Center, Research Institute for Metabolic and Obesity Disorders, Research Institute for Endocrine Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Clinical Research and Development Center, Shahid Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
J Diabetes Metab Disord. 2025 May 22;24(1):125. doi: 10.1007/s40200-025-01634-4. eCollection 2025 Jun.
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.
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.
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.
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.
儿童库欣综合征（CS）较为罕见且诊断困难，尤其是在区分促肾上腺皮质激素（ACTH）依赖性亚型时。双侧岩下窦采血（BIPSS）是实现这一目的的关键检查，但技术上具有挑战性。由于标准刺激物促肾上腺皮质激素释放激素（CRH）存在局限性，目前正在探索去氨加压素作为替代方案。本研究评估了去氨加压素刺激下的BIPSS在伊朗儿童CS队列中的诊断准确性和肿瘤定位情况，填补了儿童特异性诊断策略的空白，并为去氨加压素在此背景下的适用性提供了见解。
2015年8月至2019年3月期间，4例垂体成像结果不明确且疑似库欣病（CD）的儿科患者在伊朗德黑兰塔莱哈尼医院接受了去氨加压素刺激下的BIPSS检查。评估了BIPSS对CD诊断的敏感性，并在手术期间评估了肿瘤定位的准确性。
双侧岩下窦采血对儿科患者CD诊断的敏感性为100%。然而，肿瘤侧别定位的准确性一般，BIPSS侧别定位与手术结果之间仅有50%的一致性。具体而言，4例患者中有2例在手术中证实侧别定位正确，1例左侧定位与垂体切除术结果一致。这些差异凸显了解剖结构和引流变异等可能导致定位错误的挑战。
去氨加压素提高了BIPSS对儿科CD诊断的敏感性，是CRH刺激的可行替代方案。尽管敏感性较高，但在解读BIPSS肿瘤定位结果时仍需谨慎。需要进一步研究以优化儿童CS管理的诊断策略。
