TY - JOUR
T1 - Congenital 17α-hydroxylase deficiency
T2 - A clinicopathologic study
AU - Sasano, Hironobu
AU - Masuda, Takayuki
AU - Ojima, Motoko
AU - Fukuchi, Soitsu
AU - Sasano, Nobuaki
PY - 1987/10
Y1 - 1987/10
N2 - The histopathologic features of the adrenal glands in three cases of congenital 17α-hydroxylase deficiency are described in relation to clinical and endocrine findings. Diffuse or nodular adrenocortical hyperplasia, particularly in the zonae fasciculata and reticularis, was observed in all cases examined. The hyperplastic adrenal cortices were, composed of cells with morphologic features of hypercorticism and hyperstimulation. Myelolipomatous lesions were detected in two cases. These morphologic findings were consistent with excessive adrenocorticotropic hormone secretion in this disorder. In all the cases examined, the plasma aldosterone concentration was within normal limits, and plasma renin activity was suppressed prior to dexamethasone treatment. Morphologically, however, hyperplasia of the cells with abundant mitochondria and smooth endoplasmic reticulum seemed to involve the zona glomerulosa. Nonencapsulated nests of hypertrophied cortical cells in periadrenal tissue were remarkable in one case. From these morphologic findings, we postulated hyperfunction of the zona glomerulosa as well as involvement of corticosteroids from the zona, glomerulosa in the pathophysiology of this disorder.
AB - The histopathologic features of the adrenal glands in three cases of congenital 17α-hydroxylase deficiency are described in relation to clinical and endocrine findings. Diffuse or nodular adrenocortical hyperplasia, particularly in the zonae fasciculata and reticularis, was observed in all cases examined. The hyperplastic adrenal cortices were, composed of cells with morphologic features of hypercorticism and hyperstimulation. Myelolipomatous lesions were detected in two cases. These morphologic findings were consistent with excessive adrenocorticotropic hormone secretion in this disorder. In all the cases examined, the plasma aldosterone concentration was within normal limits, and plasma renin activity was suppressed prior to dexamethasone treatment. Morphologically, however, hyperplasia of the cells with abundant mitochondria and smooth endoplasmic reticulum seemed to involve the zona glomerulosa. Nonencapsulated nests of hypertrophied cortical cells in periadrenal tissue were remarkable in one case. From these morphologic findings, we postulated hyperfunction of the zona glomerulosa as well as involvement of corticosteroids from the zona, glomerulosa in the pathophysiology of this disorder.
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U2 - 10.1016/S0046-8177(87)80216-2
DO - 10.1016/S0046-8177(87)80216-2
M3 - Article
C2 - 3653875
AN - SCOPUS:0023276475
VL - 18
SP - 1002
EP - 1007
JO - Human Pathology
JF - Human Pathology
SN - 0046-8177
IS - 10
ER -