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.
In 1958 Durand published an account of an infant with vomiting and diarrhoea since birth, and he demonstrated the presence of lactose in the urine. Since that time more widespread attention has been paid to sugar intolerance in infants, and an increasing number of reports on such cases have been published.A survey of the available published material suggests that two forms of this condition exist, which from a clinical and biochemical standpoint show certain distinguishing features.In the first, diarrhoea is the presenting symptom and the occurrence of vomiting is not stressed: in these cases mellituria occurs but rarely, or not at all; where such studies have been carried out, tolerance curves utilizing the offending sugar have been flat, suggesting impairment of absorption from the intestinal lumen. The diarrhoea in these cases is deemed to be caused by the presence of large quantities of unhydrolysed and unabsorbed sugars in the intestine, and is mediated by osmotic factors or by bacterial action. Characteristically the stools are frothy, strongly acid, and have a high lactic acid content. Such cases have been described by
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