[CONGENITAL ADRENOGENITAL SYNDROME IN INFANTS].
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We assessed the usefulness and reliability of computed tomography (CT scan) in evaluating adrenal hyperplasia in 38 patients, including 14 with Cushing's disease, 17 with idiopathic hyperaldosteronism (IHA), and 7 with the adrenogenital syndrome (AGS). Eighty-two normal subjects were also examined. We analyzed the shape of the adrenal gland and quantitated its thickness, width and length. Visual inspection revealed V-shaped right adrenal glands in 100% of patients with Cushing's disease, 94% of patients with IHA, 100% of patients with AGS and in 41% of the normal subjects. Triangular left adrenal glands were observed in 100% of patients with Cushing's disease, 82% of patients with IHA, 67% of patients with AGS and in 12% of the normal subjects. Quantitative analysis showed that the right adrenal gland was significantly thicker and longer in patients with Cushing's disease, IHA and in those with AGS than in normal subjects. The right adrenal gland was significantly wider in the patients with Cushing's disease and AGS than in control subjects. The left adrenal gland was significantly wider and longer in patients with Cushing's disease and AGS than in the normal controls. Analysis of individual data indicated that the upper limit of normal for thickness of the right adrenal was 7 mm. Therefore, adrenal hyperplasia was strongly suggested when the right adrenal gland was more than 7 mm thick. Our findings suggest that the CT scan is useful and reliable in diagnosing adrenal hyperplasia.
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A number of studies in the history of endocrinology makes reference to two pediatric cases from the early 19th century which are commonly seen as containing the earliest descriptions of the adrenogenital syndrome substantiated by post-mortem findings. Their authors, in addition to being considered the first to furnish an exact description of the symptoms involved, are also occasionally credited with having been aware of the underlying causes of this disorder, i.e. the interaction between the adrenal cortex and the genitalia. The present study demonstrates that this opinion is not founded on facts. Furthermore, a retrospective analysis of the pathological details seemingly irreconcilable with this condition may in fact be explained as resulting from imperfect embryonic development.
Adrenal tumors accidently detected by CT scan are increasingly seen in patients without clinical signs of adrenal diseases. We studied whether enhanced adrenal stimulation is of importance in the development of adrenal tumors. For this purpose 22 patients with adrenogenital syndrome (AGS) were studied by CT scan. One of these patients suffered from C-11 beta-hydroxylase-, one from C-3 beta-hydroxy steroid dehydrogenase-, and 20 from C-21-hydroxylase deficiency. The average adrenal size of these patients was 506 +/- 79 mm2 as compared to 132 +/- 8 mm2 in the controls (P less than 0.001). Only two patients with the late onset form revealed adrenal glands of normal size. There was a significant correlation between adrenal size and patients' age (P less than 0.01). Females with the simple virilizing form revealed adrenal glands larger than those of the late onset form (640 +/- 169 vs 308 +/- 56 mm2). Eighteen patients with AGS exhibited one (n = 11) or several (n = 7) adrenal tumors, the size of which was 5-9 mm in diameter in 9, 10-20 mm in 7, and more than 50 mm in 2 patients. There was a significant correlation between adrenal hyperplasia and tumor diameter (P less than 0.001). No correlation was found between tumor size and plasma concentrations of testosterone or 17-hydroxyprogesterone, patients' age at the time of diagnosis, or clinical signs of androgenization. Again, tumors were larger in females suffering from the simple virilizing form of AGS than in those with the late onset form (14.8 +/- 5.5 vs 7.7 +/- 0.8 mm).(ABSTRACT TRUNCATED AT 250 WORDS)
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A case of extensive transitional cell metaplasia (TCM) and ectopic prostatic tissue was encountered in the uterine cervix and vagina of a 23-year-old woman with the adrenogenital syndrome. A previously described case of ectopic prostatic tissue in the cervix in a patient with female pseudohermaphroditism and our case suggest that prolonged androgen stimulation plays a role in the development of prostatic tissue. Similarly, the occurrence of TCM in the perimenopausal and postmenopausal periods when the ovaries secrete androgens, in female transsexuals treated with androgens, and in our patient with high endogenous androgen levels suggest that androgen has a major role in the histogenesis of TCM. Significantly decreased Ki-67 immunopositivity in TCM compared with the normal cervical mucosa of women in the reproductive age indicates that TCM is a mild form of epithelial atrophy, presumably caused by the neutralizing effect of androgens.
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