Liza Das1, Anil Bhansali1, Rosario Pivonello2, Pinaki Dutta1, Sanjay Kumar Bhadada1, Chirag Kamal Ahuja3, Ravimohan Mavuduru4, Santosh Kumar4, Arunanshu Behera5, Uma Nahar Saikia6, Sivashanmugam Dhandapani7, Rama Walia8. 1. Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. 2. Dipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Università Federico II di Napoli, 80131, Naples, Italy. 3. Department of Radiology, PGIMER, Chandigarh, India. 4. Department of Urology, PGIMER, Chandigarh, India. 5. Department of General Surgery, PGIMER, Chandigarh, India. 6. Department of Histopathology, PGIMER, Chandigarh, India. 7. Department of Neurosurgery, PGIMER, Chandigarh, India. 8. Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. ramawalia@rediffmail.com.
Abstract
PURPOSE: Nelson's syndrome (NS) is regarded as an aggressive complication of total bilateral adrenalectomy (TBA) for Cushing's disease (CD). This challenge may be addressed by using clinical criteria to guide frequency of neuroimaging to enable timely management of NS and also avoid unnecessary frequent imaging. METHODS: All patients (n = 43) with CD subjected to TBA over 35 years at a tertiary care centre were included. NS was defined as a newly appearing or expanding (> 2 mm) pituitary adenoma with or without ACTH levels exceeding 500 pg/ml. Pre-and post-TBA parameters like clinical symptomatology, cortisol, ACTH and radiology were analysed for the prediction of NS. RESULTS: NS developed in 39.5% (n = 17) patients with a median follow-up of 7 years. Half of them had new appearance, while rest had an expansion of pre-existing pituitary tumour. Majority (90%) had ACTH above 500 pg/ml. On Cox proportional hazards analysis, frequent discriminatory features of protein catabolism (≥ 4) (HR 1.15, CI 0.18, 7.06), proximal myopathy (HR 8.82, CI 1.12, 69.58) and annual ACTH increment of 113 pg/ml (HR 12.56, CI 1.88, 88.76) predicted NS. First post-operative year ACTH indices predicting NS included ACTH rise of 116 pg/ml and absolute ACTH of 142 pg/ml (sensitivity, specificity exceeding 90%). Annual ACTH increment exceeding 113 pg/ml, ≥ 4 discriminatory features and uncontrolled hypertension had the best overall prediction. CONCLUSION: Patients who developed NS had higher rebound rise of ACTH following TBA and a more severe disease phenotype at baseline. Consistent ACTH increment can be used as a marker for predicting the development of NS.
PURPOSE:Nelson's syndrome (NS) is regarded as an aggressive complication of total bilateral adrenalectomy (TBA) for Cushing's disease (CD). This challenge may be addressed by using clinical criteria to guide frequency of neuroimaging to enable timely management of NS and also avoid unnecessary frequent imaging. METHODS: All patients (n = 43) with CD subjected to TBA over 35 years at a tertiary care centre were included. NS was defined as a newly appearing or expanding (> 2 mm) pituitary adenoma with or without ACTH levels exceeding 500 pg/ml. Pre-and post-TBA parameters like clinical symptomatology, cortisol, ACTH and radiology were analysed for the prediction of NS. RESULTS: NS developed in 39.5% (n = 17) patients with a median follow-up of 7 years. Half of them had new appearance, while rest had an expansion of pre-existing pituitary tumour. Majority (90%) had ACTH above 500 pg/ml. On Cox proportional hazards analysis, frequent discriminatory features of protein catabolism (≥ 4) (HR 1.15, CI 0.18, 7.06), proximal myopathy (HR 8.82, CI 1.12, 69.58) and annual ACTH increment of 113 pg/ml (HR 12.56, CI 1.88, 88.76) predicted NS. First post-operative year ACTH indices predicting NS included ACTH rise of 116 pg/ml and absolute ACTH of 142 pg/ml (sensitivity, specificity exceeding 90%). Annual ACTH increment exceeding 113 pg/ml, ≥ 4 discriminatory features and uncontrolled hypertension had the best overall prediction. CONCLUSION:Patients who developed NS had higher rebound rise of ACTH following TBA and a more severe disease phenotype at baseline. Consistent ACTH increment can be used as a marker for predicting the development of NS.
Authors: A Dutta; N Gupta; R Walia; A Bhansali; P Dutta; S K Bhadada; R Pivonello; C K Ahuja; S Dhandapani; A Hajela; C Simeoli; N Sachdeva; U N Saikia Journal: J Endocrinol Invest Date: 2021-01-16 Impact factor: 4.256
Authors: Eleni Papakokkinou; Marta Piasecka; Hanne Krage Carlsen; Dimitrios Chantzichristos; Daniel S Olsson; Per Dahlqvist; Maria Petersson; Katarina Berinder; Sophie Bensing; Charlotte Höybye; Britt Edén Engström; Pia Burman; Cecilia Follin; David Petranek; Eva Marie Erfurth; Jeanette Wahlberg; Bertil Ekman; Anna-Karin Åkerman; Erik Schwarcz; Gudmundur Johannsson; Henrik Falhammar; Oskar Ragnarsson Journal: Pituitary Date: 2021-05-25 Impact factor: 4.107