Impact and timing of bilateral adrenalectomy for refractory adrenocorticotropic hormone-dependent Cushing’s syndrome.

[cushing syndrome]

In patients with refractory adrenocorticotropic hormone-dependent Cushing ’s syndrome ,we evaluated steroidogenesis inhibition (SI ) and bilateral adrenalectomy (BA ) to predict which patients might benefit most from each treatment modality .Clinical data from patients treated 1970 -2012 were reviewed retrospectively by treatment group (SI or SI +BA ). Validated severity scales were used to calculate metabolic (M ) score (hypokalemia , hyperglycemia , hypertension , proximal muscle weakness ) and adverse events (AE ) score (thrombosis , fracture , infection ).A total of 65 patients (16 pituitary , 49 ectopic ) were treated with SI +BA (n = 21 ,32 %) or SI alone (n = 44 ,68 %). Presenting M scores and source of adrenocorticotropic hormone excess (ectopic versus pituitary ) were similar . Both groups improved metabolically after treatment . Over one -third of AEs in the SI +BA group occurred within 12 months of presentation . Half (n = 24 , 55 %) of the patients treated with SI died (median survival , 24 .0 months ). Steroid excess contributed to 71 % of complications . Six SI +BA patients died (29 %), including all 3 patients with recurrent Cushing ’s syndrome after BA . Minor perioperative complications occurred in 7 patients (33 %).Posttreatment M and AE scores improved for all patients and 70 % of AEs occurred in SI +BA patients within 12 months of presentation , emphasizing the importance of early operative intervention . These data argue for the safety and efficacy of early BA in selected patients with uncontrollable Cushing ’s syndrome .

Diseases
Validation

Diseases presenting "hypertension" symptom

achondroplasia

acute rheumatic fever

adrenal incidentaloma

aniridia

aromatase deficiency

cadasil

child syndrome

cohen syndrome

congenital adrenal hyperplasia

congenital diaphragmatic hernia

cushing syndrome

cystinuria

erdheim-chester disease

erythropoietic protoporphyria

esophageal adenocarcinoma

fabry disease

familial hypocalciuric hypercalcemia

gm1 gangliosidosis

heparin-induced thrombocytopenia

hereditary cerebral hemorrhage with amyloidosis

holt-oram syndrome

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

inclusion body myositis

kallmann syndrome

kindler syndrome

lamellar ichthyosis

lymphangioleiomyomatosis

pendred syndrome

primary effusion lymphoma

scrub typhus

severe combined immunodeficiency

sneddon syndrome

typhoid

von hippel-lindau disease

well-differentiated liposarcoma

werner syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated