Article (Scientific journals)
Cushing's disease in childhood: presentation, investigation, treatment and long-term outcome.
Savage, M. O.; Lienhardt, A.; LEBRETHON, Marie-Christine et al.
2001In Hormone Research, 55 Suppl 1, p. 24-30
Peer Reviewed verified by ORBi
 

Files


Full Text
Cushing's Disease in Childhood Presentation, Investigation, Treatment and Long-Term Outcome.pdf
Publisher postprint (392.31 kB)
Request a copy

All documents in ORBi are protected by a user license.

Send to



Details



Keywords :
Adolescent; Child; Cushing Syndrome/complications/diagnosis/physiopathology/therapy; Dexamethasone/diagnostic use; Female; Follow-Up Studies; Glucocorticoids/diagnostic use; Growth; Humans; Magnetic Resonance Imaging; Male; Pituitary Gland/radiation effects/surgery; Postoperative Period; Radiotherapy; Tomography, X-Ray Computed; Treatment Outcome
Abstract :
[en] Seventeen patients with Cushing's disease (CD) were treated from 1978 to 2000. There were 11 males and 6 females aged 6.8-18.8 years (mean age 13.0 +/- 5.9 years). Presenting features were: weight gain (100%); growth failure (71%); hirsutism (53%); striae (53%); hypertension (47%). Mean age of patients with striae was 15.2 +/- 2.3 years, without striae 10.3 +/- 3.3 years. Median height SDS was -1.81 (range -0.28 to -4.17), 53% having height SDS < -1.8. The height velocity in 6 subjects was subnormal (0.9-3.8 cm/year). Median BMI SDS was 2.29 (range 1.72-5.06). Cushing's disease was confirmed by detectable serum ACTH, median 28 ng/l (range 12-99, NR <10-50) (n = 15); loss of cortisol circadian rhythm values at midnight ranging from 216 to 1,080 nmol/l (NR <50) (n = 15); lack of cortisol suppression (NV < 50 nmol/l) during low-dose dexamethasone suppression test (LDDST) (0.5 mg 6-hourly x 8) (n = 14); and >50% suppression of cortisol compared with the basal value during high-dose dexamethasone suppression test (HDDST) (2 mg 6-hourly x 8) (n = 14). A CRH test (1 microg/kg i.v.) showed an increase of cortisol from 12 to 217% (median 73.5%) (n = 16). Pituitary imaging (CT/MRI) showed an image consistent with microadenoma in 6/17 patients, but there was concordance between pituitary imaging and surgical findings in 1/11 patients (9%). Inferior petrosal sinus sampling (IPSS) for ACTH after CRH was performed in 11 subjects (age 10.7-18.8 years). Central to peripheral ACTH ratios were >2 (2.5-157.2) in 10/11 patients. The inter-petrosal sinus ACTH gradient was >1.4 in 10 patients (2.1-20.8), indicating lateralization of ACTH secretion. In 10 patients (91%), the side of the tumour on IPSS was predictive of findings at surgery. Therapy consisted of transsphenoidal microadenomectomy (TSS) in 16 patients and bilateral adrenalectomy (1978) in 1. Following TSS alone, 7 patients were cured (cortisol <50 nmol/l) and 2 were in remission (cortisol <300 nmol/l), i.e. 56%. Seven had persisting hypercortisolaemia and underwent pituitary irradiation (4,500 cGy). Therapeutic outcome for a median of 8 years (0.5-24 years) resulted in cure of CD in 14/17 patients (82%) and remission in 1. Linear growth after TSS +/- pituitary irradiation in 10 subjects showed no short-term catch-up growth, with peak growth hormone (GH) 0.5-20.9 mU/l to insulin tolerance test (ITT)/glucagon. Eight patients were treated with human growth hormone (hGH) (14 U/m(2)/week) combined in 3 with GnRH analogue. The mean final (n = 6) or latest (n = 4) height SDS was -1.36. The difference between final/latest height SDS and target height SDS was 0.93 +/- 1.13, i.e. less (p = 0.005) than the difference between height SDS and target height SDS at presentation, i.e. 1.72 +/- 1.26, indicating long-term catch-up growth.
Disciplines :
Pediatrics
Endocrinology, metabolism & nutrition
Author, co-author :
Savage, M. O.
Lienhardt, A.
LEBRETHON, Marie-Christine ;  Centre Hospitalier Universitaire de Liège - CHU > Pédiatrie
Johnston, L. B.
Huebner, A.
Grossman, A. B.
Afshar, F.
Plowman, P. N.
Besser, G. M.
Language :
English
Title :
Cushing's disease in childhood: presentation, investigation, treatment and long-term outcome.
Publication date :
2001
Journal title :
Hormone Research
ISSN :
0301-0163
eISSN :
1423-0046
Publisher :
Karger, Basel, Switzerland
Volume :
55 Suppl 1
Pages :
24-30
Peer reviewed :
Peer Reviewed verified by ORBi
Commentary :
Copyright 2001 S. Karger AG, Basel.
Available on ORBi :
since 16 April 2015

Statistics


Number of views
33 (0 by ULiège)
Number of downloads
0 (0 by ULiège)

Scopus citations®
 
59
Scopus citations®
without self-citations
43
OpenCitations
 
30

Bibliography


Similar publications



Contact ORBi