JOURNAL ARTICLE
Add like
Add dislike
Add to saved papers

Single-center experience with pediatric Cushing's disease.

OBJECT: Despite ongoing advances in surgical and radiotherapeutic techniques, pediatric Cushing's disease remains a diagnostic and therapeutic challenge. The authors report on the results of a single-center retrospective review of 33 pediatric patients with Cushing's disease, providing details with respect to clinical presentation, diagnostic evaluation, therapeutic course, complications, and outcomes.

METHODS: There were 17 female and 16 male patients whose mean age was 13 years (range 5-19 years) in whom a diagnosis of Cushing's disease was based on clinical and biochemical criteria. Typical symptoms included weight gain (91%), prepubertal growth delay (83%), round facies (61%), hirsutism (58%), headache (45%), abdominal striae (42%), acne (33%), amenorrhea (24%), and hypertension (24%). In 67% of the cases, preoperative magnetic resonance images revealed a pituitary lesion and in 82% of the cases the imaging studies effectively predicted lateralization. Inferior petrosal sinus sampling was performed in seven patients (21%), and in all of these cases lateralization was 100% reliable. Fifty-five percent underwent selective adenomectomies and 45% underwent subtotal hypophysectomies. Complications included one case of diabetes insipidus, one of persistent hypocortisolemia necessitating prolonged glucocorticoid replacement therapy, and one minor vascular injury that did not necessitate postoperative management modification or cause sequelae. There were no surgery-related deaths and no cases of postoperative cerebrospinal fluid leakage or meningitis. During a mean follow-up period of 44 months, clinical remission was ultimately achieved in 91% of patients: 76% after transsphenoidal surgery alone and an additional 15% after adjuvant radiosurgery and/or adrenalectomy following surgical failure. Three patients (12%) experienced disease recurrence and underwent a second surgical procedure at 18, 81, and 92 months, respectively; based on clinical and biochemical criteria a second remission was achieved in all. Three patients (9%) remain with persistent disease.

CONCLUSIONS: Pediatric Cushing's disease is a rare condition, often requiring a multidisciplinary diagnostic and a multimodal therapeutic approach for successful long-term remission.

Full text links

For the best experience, use the Read mobile app

Group 7SearchHeart failure treatmentPapersTopicsCollectionsEffects of Sodium-Glucose Cotransporter 2 Inhibitors for the Treatment of Patients With Heart Failure Importance: Only 1 class of glucose-lowering agents-sodium-glucose cotransporter 2 (SGLT2) inhibitors-has been reported to decrease the risk of cardiovascular events primarily by reducingSeptember 1, 2017: JAMA CardiologyAssociations of albuminuria in patients with chronic heart failure: findings in the ALiskiren Observation of heart Failure Treatment study.CONCLUSIONS: Increased UACR is common in patients with heart failure, including non-diabetics. Urinary albumin creatininineJul, 2011: European Journal of Heart FailureRandomized Controlled TrialEffects of Liraglutide on Clinical Stability Among Patients With Advanced Heart Failure and Reduced Ejection Fraction: A Randomized Clinical Trial.Review

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

Read by QxMD is copyright © 2021 QxMD Software Inc. All rights reserved. By using this service, you agree to our terms of use and privacy policy.

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app