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Blood Pressure in Pediatric Patients with Cushing Syndrome

Section on Endocrinology Genetics (M.B.L., D.L.B., M.K., J.S., J.M., S.V., C.A.S.), Program on Developmental Endocrinology Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Pediatric Endocrinology Inter-Institute Training Program (M.B.L., C.A.S.), Biostatistics and Clinical Epidemiology Service (N.S.), National Institutes of Health Clinical Center, and Department of Diagnostic Radiology (N.P.), Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892; and Division of Endocrinology, Diabetes, and Metabolism (J.P.), Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center Pittsburgh, Pennsylvania 15213

Address all correspondence and requests for reprints to: Maya B. Lodish, M.D., Section on Endocrinology Genetics, Program on Developmental Endocrinology Genetics, and Pediatric Endocrinology Program, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC (East Laboratories), Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892. E-mail: lodishma{at}mail.nih.gov.

Context: Hypertension (HTN) has been reported in up to 60% of children with Cushing syndrome (CS), but its course, side effects, and potential differences among various causes of CS have not been adequately studied.

Objective: The objective of the study was to measure blood pressure in pediatric patients with CS before and after transphenoidal surgery or adrenalectomy and identify side effects and rates of residual HTN.

Design: Data from 86 children with corticotropinomas [Cushing disease (CD)] and 27 children with ACTH-independent CS (AICS) were analyzed.

Results: Patients with CD and AICS had significant HTN before surgery; more patients with AICS had systolic HTN (SHTN) than with CD (74 vs. 44%, P = 0.0077), but the rate of diastolic HTN (DHTN) was similar. Both groups experienced significant decreases in SHTN immediately after transphenoidal surgery and adrenalectomy. One year postoperatively, both SHTN and DHTN were lower than the preoperative values in all patients, but as many as 16 and 4% of the patients with CD and 21 and 5% of the patients with AICS still had SHTN and DHTN, respectively. Higher blood pressure preoperatively correlated with cortisol levels. Two patients suffered serious side effects: one with multiple infarcts and another with hypertensive encephalopathy.

Conclusions: Children with CS are at risk for residual HTN despite a significant improvement after surgical cure. HTN appears to correlate with the degree of hypercortisolemia. Serious HTN-related side effects, although rare, may occur during the perioperative period.