Ectopic CRF production is biochemically indistinguishable from ectopic ACTH secretion, except that IPSS mimics pituitary Cushing's disease and cortisol dynamics may normalize rapidly postadrenalectomy. CRF secretion can be inferred through tumour immunohistochemistry, even if no CRF assay is available. Unrecognized phaeochromocytoma ACTH secretion may underpin some cases of cardiovascular collapse postadrenalectomy through acute hypocortisolaemia. Despite advances in phaeochromocytoma genetics since previous reports, we were unable to identify somatic DNA defects associated with either ACTH or CRF secretion.
This case describes the longest reported clinical course of diabetic hepatosclerosis, spanning 11 years, in which time the patient did not develop evidence of cirrhosis or portal hypertension. It is difficult to estimate the clinical relevance of this condition because little is known regarding its clinical course and effect on morbidity and mortality. Identified patients should undergo low-intensity, long-term follow-up to improve understanding of its clinical sequelae and relevance.
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