EVALUATION OF A THERAPEUTIC REGIMEN IN CUSHING'S DISEASE

1977 ◽  
Vol 86 (1) ◽  
pp. 146-155 ◽  
Author(s):  
S. W. J. Lamberts ◽  
F. H. de Jong ◽  
J. C. Birkenhäger
Keyword(s):  
Cushing’S Disease ◽  
Plasma Cortisol ◽  
Sella Turcica ◽  
Therapeutic Regimen ◽  
Cushing's Disease ◽  
Unilateral Adrenalectomy ◽  
Lysine Vasopressin ◽  
Pituitary Irradiation ◽  
Pituitary Adrenal Axis

ABSTRACT Eighteen unselected patients with Cushing's disease were treated by unilateral adrenalectomy followed by conventional external pituitary irradiation (4500 rads). In 9 of these patients (50 %) a remission has been reached as judged by the clinical picture and the cortisol secretion rate (CSR) up to an average of 5 years after operation (follow-up varying between 1 and 7.5 years). The 9 other patients showed a relapse making necessary a second adrenalectomy after an average of 15 months (5 to 57 months). Four of these patients had a slightly enlarged (two cases) or asymmetrical sella turcica (two cases). The gain of this therapeutic regimen was that no permanent adrenal insufficiency is induced in 50 % of the patients, that no pituitary tumours developed and that no loss of pituitary function occurred. Before treatment the group of 9 patients, who did not improve or relapsed after treatment, showed a greater rise of plasma cortisol in response to lysine vasopressin, a (greater) decrease of plasma cortisol in response to a single oral dose of 1 mg dexamethasone and an earlier decrease of the urinary 17-OGS in response to 2 mg dexamethasone four times daily orally. It is concluded that in the patients whose hypothalamo-pituitary-adrenal axis could be manipulated less this therapeutic regimen had a better result.

Results of external pituitary irradiation after unsuccessful transsphenoidal surgery in Cushing's disease

1991 ◽  
Vol 125 (4) ◽  
pp. 470-474 ◽  
Author(s):  
A. Vicente ◽  
J. Estrada ◽  
C. de la Cuerda ◽  
B. Astigarraga ◽  
M. Marazuela ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
High Efficiency ◽  
Cushing's Disease ◽  
Anterior Pituitary Function ◽  
Low Incidence ◽  
Pituitary Irradiation ◽  
Age Range ◽  
Pituitary Adrenal Axis ◽  
Tsh Deficiency

Abstract. Fourteen adult patients (10 females and 4 males; age range 20-60 years) with persistent Cushing's disease after transsphenoidal microsurgery were treated with pituitary irradiation. Supervoltage multiportal administration was employed at a total dose of 50 Gy (±0.65 sd). The interval between microsurgery and pituitary irradiation was less than 6 months in 6 patients and more than 6 months in 8. The pituitary-adrenal axis was evaluated postsurgically, before irradiation and every 6 months thereafter. The remaining anterior pituitary function was simultaneously tested. Remission rates at 12 months and 24 months after radiotherapy were 61 and 70%, respectively. Two patients developed TSH deficiency and another gonadotropin deficiency during the follow-up after radiation. We conclude that pituitary irradiation is the treatment of choice for persistent Cushing's disease after unsuccessful surgery because of its high efficiency and low incidence of adverse reactions when compared with other forms of treatment.

The recovery of the hypothalamo-pituitary-ad renal axis after transsphenoidal operation in three patients with Cushing's disease. The effect of prior external pituitary irradiation

1981 ◽  
Vol 98 (4) ◽  
pp. 580-585 ◽  
Author(s):  
Steven W.J. Lamberts ◽  
Jan G. M. Klijn ◽  
Frank H. de Jong ◽  
Jan C. Birkenhäger
Keyword(s):  
Growth Hormone ◽  
Cushing’S Disease ◽  
Plasma Cortisol ◽  
Hormone Secretion ◽  
Growth Hormone Secretion ◽  
Cushing's Disease ◽  
Plasma Acth ◽  
Basal Plasma ◽  
Pituitary Irradiation ◽  
Pituitary Adrenal Axis

Abstract. The recovery of the hypothalamo-pituitary-adrenal axis after selective transsphenoidal adenomectomy was studied in 3 patients with Cushing's disease by measuring basal plasma ACTH and cortisol concentrations, cortisol secretion rate, the diurnal rhythm of cortisol, and the reaction of cortisol to lysine vasopressin (LVP), of compound S to metyrapone and of cortisol and growth hormone to an insulin-induced hypoglycaemia. The third patient had been treated previously by external pituitary irradiation. In 2 patients basal plasma ACTH levels returned within normal values before plasma cortisol, but no supra-physiological plasma concentrations of ACTH were seen as has been observed after withdrawal of exogenous glucocorticoids. With regard to the different stimulation tests: at first the normal reaction of plasma cortisol to LVP returned after 3 months, at the same time as the restoration of growth hormone secretion in response to hypoglycaemia. A normalization of the reaction to metyrapone was seen thereafter while finally the reaction of cortisol to an insulin-induced hypoglycaemia and the diurnal rhythm of plasma cortisol returned 15 to 18 months after operation in the first patient and after 12 months in the second patient. Selective adenomectomy had also been carried out in the third patient, as evidenced by normal TSH, LH and FSH secretion. Hypocortisolism, and a deficient ACTH and growth hormone secretion in response to the stimuli mentioned, however, did not normalize up till 22 months after operation. The restoration of the hypothalamo-pituitary-adrenal axis after selective pituitary adenomectomy in Cushing's disease was prevented in this patient by prior external pituitary irradiation.

UNILATERAL ADRENALECTOMY FOLLOWED BY PITUITARY IRRADIATION IN CUSHING'S DISEASE; WITH OBSERVATIONS ON CORTISOL SECRETION AFTER THERAPY

1965 ◽  
Vol 48 (2) ◽  
pp. 253-262 ◽  
Author(s):  
A. P. van Seters ◽  
M. Jenny ◽  
A. Querido
Keyword(s):  
Cushing’S Disease ◽  
Primary Treatment ◽  
Cushing's Disease ◽  
Cortisol Secretion ◽  
Therapeutic Result ◽  
Unilateral Adrenalectomy ◽  
Follow Up Study ◽  
Pituitary Irradiation ◽  
Secretion Rates

ABSTRACT Nineteen out of 38 patients with Cushing's disease were successfully treated by unilateral adrenalectomy and post-operative pituitary irradiation. Follow-up periods ranged from 1 to 11 years, with an average of 5.7 years. Determinations of cortisol secretion were performed in 13 patients as part of the follow-up study for the evaluation of the therapeutic result. In 11 patients, the secretion rates varied between 12.4 and 30.9 mg/24 h, values which are considered normal. Unilateral adrenalectomy followed by pituitary irradiation is advocated as the primary treatment in patients suffering from Cushing's disease without serious complications.

Concomitant falls of plasma cortisol and ACTH levels in a case of Cushing's disease during treatment with trilostane

1984 ◽  
Vol 105 (1) ◽  
pp. 93-98 ◽  
Author(s):  
Kaoru Nomura ◽  
Hiroshi Demura ◽  
Toshihiro Imaki ◽  
Megumi Miyagawa ◽  
Masami Ono ◽  
...  
Keyword(s):  
Urinary Excretion ◽  
Cushing’S Disease ◽  
Plasma Levels ◽  
Plasma Cortisol ◽  
Competitive Inhibitor ◽  
Cushing's Disease ◽  
Plasma Acth ◽  
Lysine Vasopressin ◽  
Hypothalamic Pituitary Axis ◽  
Pituitary Irradiation

Abstract. A 72-year-old man with Cushing's disease was treated with trilostane, a competitive inhibitor of adrenal 3β-hydroxysteroid dehydrogenase (3β-HSDH). The treatment with trilostane successfully lowered urinary excretion of 17-hydroxycorticosteroids (17-OHCS) and plasma levels of cortisol and elevated the plasma level of dehydroepiandrosterone. Unexpectedly, plasma ACTH fell from 109.7 ± 45.0 to 42.7 ± 27.3 pg/ml (P < 0.01) in parallel with plasma cortisol. The hyperresponsiveness of plasma ACTH observed both in the metyrapone test and the lysine-vasopressin test was also ameliorated by treatment with trilostane. Then low dose of pituitary irradiation with cobalt-60 was added and his urinary excretion of 17-OHCS and plasma levels of cortisol decreased further. After treatment with trilostane was finally stopped, the plasma ACTH increased from 45.9 ± 21.9 to 69.6 ± 25.3 pg/ml (P < 0.05) in parallel with plasma cortisol. Since then he has had no recurrence for 12 months. These observations suggest that trilostane, like other adrenal enzyme-inhibiting drugs, may induce unexpected improvement of the abnormality of the hypothalamic-pituitary axis present in Cushing's disease.

scholarly journals The Complete Normalization of the Adrenocortical Function as the Criterion of Cure after Transsphenoidal Surgery for Cushing’s Disease

2001 ◽  
Vol 86 (12) ◽  
pp. 5695-5699 ◽  
Author(s):  
Javier Estrada ◽  
José García-Uría ◽  
Cristina Lamas ◽  
José Alfaro ◽  
Tomás Lucas ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Plasma Cortisol ◽  
Cushing's Disease ◽  
Adrenocortical Function ◽  
Urinary Cortisol ◽  
Group Iii ◽  
Group I ◽  
Group Ii

Transsphenoidal microsurgery is the standard treatment for patients with Cushing’s disease. However, there is general lack of agreement regarding the definition of cure. We studied 58 patients with corrected hypercortisolism after transsphenoidal surgery for Cushing’s disease. Plasma and urinary cortisol levels were measured after surgery. After the postsurgical hypocortisolism stage (or periodically in patients without hypocortisolism), urinary free cortisol, plasma cortisol at 0800 h and 2300 h, morning cortisol after 1 mg dexamethasone, and cortisol response to insulin-induced hypoglycemia were performed. Patients were classified in 3 groups: group I, patients with transient hypocortisolism and normal hypothalamus-pituitary-adrenal axis afterwards; group II, patients with transient hypocortisolism and abnormalities in the circadian rhythm or the stress response afterwards; and group III, patients without postoperative hypocortisolism. Thirty-three patients were included in group I, 8 in group II, and 17 in group III. Groups I and II were similar in postsurgical plasma cortisol (46.9 ± 30.3 vs. 60.7 ± 38.6 nm) and mean follow-up (69.8 vs. 68.8 months) but were significantly different in their recurrence rate (3.4% vs. 50%, P &lt; 0.001). Patients in group III had normal postsurgical plasma and urinary cortisol but persistent abnormalities in circadian rhythm and stress response. After a mean follow-up of 39.1 months, their recurrence rate was similar to that of group II (64.7% vs. 50%). The complete normalization of the adrenocortical function, which is always preceded by postsurgical hypocortisolism, is associated with a very low recurrence risk and should be considered, in our opinion, the main criterion of surgical cure in Cushing’s disease.

MEGAVOLTAGE PITUITARY IRRADIATION IN THE MANAGEMENT OF CUSHING'S DISEASE AND NELSON'S SYNDROME: LONG-TERM FOLLOW-UP

1989 ◽  
Vol 31 (3) ◽  
pp. 309-323 ◽  
Author(s):  
T. A. HOWLETT ◽  
P. N. PLOWMAN ◽  
J. A. H. WASS ◽  
L. H. REES ◽  
A. E. JONES ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Long Term Follow Up ◽  
Pituitary Irradiation

Long Term Follow-up of Cushing's Disease Treated with Reserpine and Pituitary Irradiation Followed by Subtotal Adrenalectomy.

1994 ◽  
Vol 172 (2) ◽  
pp. 97-109
Author(s):  
MASANORI MURAYAMA ◽  
KEIGO YASUDA ◽  
YOSHIAKI MINAMORI ◽  
LEILANI B. MERCADO-ASIS ◽  
NORIYOSHI YAMAKITA ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Subtotal Adrenalectomy ◽  
Long Term Follow Up ◽  
Pituitary Irradiation

LONG-TERM FOLLOW-UP OF LOW-DOSE EXTERNAL PITUITARY IRRADIATION FOR CUSHING'S DISEASE

1990 ◽  
Vol 33 (4) ◽  
pp. 445-455 ◽  
Author(s):  
M. D. LITTLEY ◽  
S. M. SHALET ◽  
C. G. BEARDWELL ◽  
S. R. AHMED ◽  
M. L. SUTTON
Keyword(s):  
Cushing’S Disease ◽  
Low Dose ◽  
Cushing's Disease ◽  
Long Term Follow Up ◽  
Pituitary Irradiation
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