Pontefract cakes can be bad for you: refractory hypertension and liquorice excess.

when ultrasound and CT scanning of the adrenals failed to demonstrate any abnormality. Moreover, blood pressure was clearly volume-sensitive and plasma Introduction aldosterone concentration and plasma renin activity were found to be low (recumbent aldosterone 58, NR Hypertension is a common problem which is usually 100–500 pmol/l; ambulatory aldosterone 170, NR straightforward to manage. Occasionally, rare clinical 600–1200 pmol/l ). Thus, differential diagnoses of syndromes occur which challenge both the clinician Liddle’s syndrome (pseudohypoaldosteronism type 1) and the chemical pathologist to come up with a dia[1,2], or a form of apparent mineralocorticoid excess gnosis. Here we present the case of a woman with (AME) [3] were considered. The strong family history refractory hypertension secondary to liquorice intoxof hypertension was compatible with the former and ication, who serves as a good example of the importhe possibility of liquorice intoxication was thought to tance of taking a detailed medical and social history have been excluded in the history. (and being persistent when in doubt), whilst demonA urinary steroid profile revealed elevated 11bstrating that laboratory investigations need to be hydroxyandrosterone, and ratio of cortisol metabolites assessed in the context of the clinical findings. to tetrahydrocortisone (Table 1). The total urinary excretion of cortisol metabolites was not raised and individual metabolites measured were within the Case report normal range. Both urinary free cortisol and plasma cortisol were within the normal range. The increase in A 49-year-old woman was referred to the Middlesex the ratio of cortisol to cortisone metabolites, was Hospital in March 1997 for investigation of persistent suggestive of a mild impairment of cortisol metabolism hypertension and hypokalaemia. She suffered a cerebdue to reduced 11b-hydroxysteroid dehydrogenase rovascular accident (CVA) 4 months previously, caus(11b-OHSD) activity [3–5]. The ratio of tetrahying a left hemiparesis from which she had made a drocortisol (THF) plus allo tetrahydrocortisol (aTHF) complete recovery. She had no significant past medical to tetrahydrocortisone (THE) was 1.39–1.65, typical history and up until her CVA, she had been cycling of values observed after liquorice ingestion [6 ], several miles to and from work every day. She had a although again, this was thought to have been excluded strong family history of hypertension, her mother in the history. This ratio may also be affected by having died 8 years previously of a sub-arachnoid diuretics such as frusemide [7], which can also inhibit haemorrhage, and her father and a maternal aunt and 11b-OHSD activity. uncle were all being treated for hypertension. The patient was started on a cocktail of antiInitial biochemical investigations were unremarkable hypertensives which had little impact on her blood apart from a plasma potassium of 2.6 mmol/l (NR pressure. Her plasma potassium concentration did not 3.3–4.8) and a plasma bicarbonate of 32 mmol/l increase on amiloride, which is usually an effective (20–30). She had a normal renal ultrasound scan, mild anti-hypertensive in Liddle’s syndrome. The possibility insignificant stenosis of the origin of the left renal artery, of a primary deficiency of 11b-OHSD (which more and concentric left ventricular hypertrophy on echocartypically presents in childhood with fatal hypertension, diography, with no evidence of coarctation of the aorta. though has been described in adults [8]) was considered, but this was excluded by the absence Correspondence and offprint requests to: Professor Robert Unwin, of a significant decrease in excretion of cortisone Centre for Nephrology, Royal Free and University College London Medical School, 48 Riding House Street, London W1P 7PN, UK. metabolites.