ABSTRACT
OBJECTS: Cushing's disease (CD) is the most common cause of ACTH-dependent hypercortisolism in children age ≥ 7. The utility of bilateral inferior petrosal sinus sampling (BIPSS), an important test in adults, is less defined in children. We present a case series of children with ACTH-dependent hypercortisolemia and review the literature to assess the utility of BIPSS in the diagnosis and localization of CD. METHODS: We performed an IRB-approved chart review of patients aged ≤ 18 with ACTH-dependent hypercortisolism at MGH between 2000 and 2019 and collected clinical, laboratory, radiographic, BIPSS, surgical, and outcomes data. RESULTS: In our cohort (n = 21), BIPSS had a sensitivity of 93% and specificity of 100% for diagnosis of CD. Compared to surgery, successful BIPSS correctly predicted adenoma laterality in 69% of cases vs. 70% by MRI. Among patients with lesions ≥ 4 mm (n = 9), BIPSS correctly lateralized in 50% vs. 100% by MRI. In patients with subtle lesions (< 4 mm, n = 7), BIPSS correctly lateralized in 80% vs. 71% by MRI. In patients (n = 4) with CD and negative MRIs, BIPSS correctly lateralized in 75% cases. Surgical cure was achieved in 90% of patients and 95% of patients had long-term disease control. CONCLUSIONS: In our cohort (n = 21; n = 20 CD, n = 1 ectopic ACTH secretion), BIPSS was sensitive and specific for the diagnosis of CD. Compared to MRI, BIPSS was not additionally helpful for lateralization in patients with lesions ≥ 4 mm on MRI. BIPSS was helpful in guiding surgical exploration and achieving immediate postoperative remission among patients with subtle and negative MRI findings.
Subject(s)
Adrenocorticotropic Hormone/blood , Hypophysectomy/methods , Petrosal Sinus Sampling/methods , Pituitary ACTH Hypersecretion , Pituitary Neoplasms , Adolescent , Body Mass Index , Female , Humans , Hydrocortisone/urine , Magnetic Resonance Imaging/methods , Male , Pituitary ACTH Hypersecretion/blood , Pituitary ACTH Hypersecretion/diagnosis , Pituitary ACTH Hypersecretion/pathology , Pituitary ACTH Hypersecretion/surgery , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/pathology , Pituitary Neoplasms/surgery , Remission Induction/methods , Reproducibility of Results , Time , Treatment OutcomeABSTRACT
As medical-surgical supply distributors begin to establish partnerships with hospitals, thus sharing financial risk, materiel executives must understand all potential contractual ramifications. This article will discuss the types and features of supply capitated contracts and the subsequent need to correctly assess new categories of contractual risk.
