Cushings Disease With A Spontaneous Remission
Pediatrics 50(3): Sept 1972; 477-481
View Original Source →Abstract
Since its description in 1932, Cushing’s disease has become a therapist’s disease. With the discovery of its existence came the natural reaction of destroying the cause or supposed cause as quickly as possible lest the more dangerous complications of the syndrome manifest themselves. As a result of therapy, the natural history of the disease is unknown in children. Also apparent from the complications and secondary effects of surgery and or irradiation is the fact that the ideal treatment is yet to be found. Our experience with the patient described herein establishes that a spontaneous remission is possible. Therefore, we propose that no treatment be given to patients with mild Cushing’s disease.
Case Details
Personal Characteristics
The propositus presented to the endocrine clinic in december 1965, at the age of 11 years, 10 months, with the single complaint of short stature. At age 5, eugenes height had been in the 85th percentile and had fallen from the 50th percentile at age 8 years to the 3rd percentile when he presented. At 8 years of age his appearance had been that of a normal prepubertal boy; however, in the ensuing three years he had grown only 3.1 centimeters but gained 21 kilograms. His parents depicted him as a pleasant, happy individual of bright intellect. Eyesight had not deteriorated, and no history of headaches could be obtained.
Clinical Characteristics
Physical examination revealed marked obesity, mild acne, moon facies, buffalo hump, supraclavicular fat pad and pubic hair despite small prepubertal testes. The elevated urinary 17-hydroxycorticosteroids (17- ohcs) in december 1965 and elevated cortisol secretory rate in march 1966, clearly established the diagnosis of cushings disease.
Remission Characteristics
When the urinary 17-hydroxycorticosteroids became normal in 1967, we suspected that eugene was in biochemical remission and confirmed this repeatedly over the next four years. In 1970 his tolerance to orally administered glucose was normal (fasting blood sugar: 76 mg/ 100 ml; 1 hour: 149 mg/100 ml; 3 hours: 98 mg/100 ml). Also, plasma cortisol levels showed the usual circadian rhythm. Eugene had grown from 55 inches in 1967 to 62 inches in october 1971, a growth rate of approximately 1 3/4 inches per year, a marked improvement to the earlier rate of 3/8 inch per year. He has lost the stigmata of cushings disease and matured sexually.
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Non-Clinical Treatment
Because of the absence of the dangerous problems, neither subtotal adrenalectomy, hypophysectomy, nor irradiation of the pituitary was performed. In addition, since all available therapy was potentially destructive and his disease was mild, it was felt that treatment might be more detrimental to the patients health than the disease itself, if the latter did not progress.
Additional Notes
Our experience with the patient described herein establishes that a spontaneous remission is possible. Therefore, we propose that no treatment be given to patients with mild Cushings disease.