{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:53Z","timestamp":1784557073938,"version":"3.55.0"},"reference-count":0,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T00:00:00Z","timestamp":1622505600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,6,1]]},"abstract":"\n Summary<\/jats:title>\n Phaeochromocytoma is a rare catecholamine-producing tumour. We present the case of phaeochromocytoma in a young man with a background history of a double-lung transplant for cystic fibrosis (CF). Clinical case: A 25-year-old man, with a background history of CF, CF-related diabetes (CFRD) and a double-lung transplant in 2012 was presented to the emergency department with crampy abdominal pain, nausea and vomiting. He was diagnosed with distal intestinal obstructions syndrome (DIOS). Contrast-enhanced CT imaging of the abdomen and pelvis showed a 3.4 cm right adrenal lesion. This was confirmed by a subsequent MRI of adrenal glands that demonstrated moderate FDG uptake, suggestive of a diagnosis of phaeochromocytoma. The patient was noted to be hypertensive with a blood pressure averaging 170\/90 mm\/Hg despite treatment with three different anti-hypertensive medications \u2013 amlodipine, telmisartan and doxazosin. He had hypertension for the last 3 years and had noted increasingly frequent sweating episodes recently, without palpitations or headache. Laboratory analysis showed elevated plasma normetanephrines (NMN) of 3167 pmol\/L (182\u2013867) as well as elevated metanephrines (MN) of 793 pmol\/L (61\u2013377) and a high 3-MT of 257 pmol\/L (<185). Once cathecholamine excess was identified biochemically, we proceeded to functional imaging to further investigate. MIBG scan showed a mild increase in the uptake of tracer to the right adrenal gland compared to the left. The case was discussed at a multidisciplinary (MDT) meeting at which the diagnosis of phaeochromocytoma was made. Following a challenging period of 4 weeks to control the patient\u2019s blood pressure with an alpha-blocker and beta-blocker, the patient had an elective right adrenalectomy, with normalisation of his blood pressure post-surgery. The histopathology of the excised adrenal gland was consistent with a 3 cm phaeochromocytoma with no adverse features associated with malignant potential.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Five to ten per cent of patients have a secondary cause for hypertension. Phaeochromocytomas are rare tumours, originating in chromaffin cells and they represent 0.1\u20131.0% of all secondary hypertension cases.<\/jats:p>\n <\/jats:list-item>\n \n Secondary causes should be investigated in cases where:<\/jats:p>\n <\/jats:list-item>\n \n Patient is presenting <20 years of age or >50 years of age,<\/jats:p>\n <\/jats:list-item>\n \n There is refractory hypertension, or<\/jats:p>\n <\/jats:list-item>\n \n There is serious end-organ damage present.<\/jats:p>\n <\/jats:list-item>\n \n Patients may present with the triad of headache, sweating and palpitations or more vague, non-specific symptoms.<\/jats:p>\n <\/jats:list-item>\n \n Patients with suspected phaeochromocytoma should have 24-h urinary catecholamines measured and if available, plasma metanephrines measured. Those with abnormal biochemical tests should be further investigated with imaging to locate the tumour.<\/jats:p>\n <\/jats:list-item>\n \n Medical treatment involves alpha- and beta-blockade for at least 2 to 3 weeks before surgery as well as rehydration.<\/jats:p>\n <\/jats:list-item>\n \n There is a possibility of relapse so high-risk patients require life-long follow-up.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-21-0026","type":"journal-article","created":{"date-parts":[[2021,6,7]],"date-time":"2021-06-07T08:05:45Z","timestamp":1623053145000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["Treatment-resistant hypertension in a post-transplant patient with cystic fibrosis: a rare case of phaeochromocytoma"],"prefix":"10.1530","volume":"2021","author":[{"given":"David Joseph","family":"Tansey","sequence":"first","affiliation":[{"name":"1Department of EndocrinologyMater Misericordiae University Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jim John","family":"Egan","sequence":"additional","affiliation":[{"name":"2Department of Respiratory Medicine and Lung & Heart TransplantationMater Misericordiae University Hospital, Dublin, Ireland"},{"name":"5UCD School of MedicineUniversity College Dublin, Belfield, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Murray","sequence":"additional","affiliation":[{"name":"2Department of Respiratory Medicine and Lung & Heart TransplantationMater Misericordiae University Hospital, Dublin, Ireland"},{"name":"5UCD School of MedicineUniversity College Dublin, Belfield, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie","family":"Padfield","sequence":"additional","affiliation":[{"name":"3Department of AnesthesiaMater Misericordiae University Hospital, Dublin, Ireland"},{"name":"5UCD School of MedicineUniversity College Dublin, Belfield, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Conneely","sequence":"additional","affiliation":[{"name":"4Department of General SurgeryMater Misericordiae University Hospital, Dublin, Ireland"},{"name":"5UCD School of MedicineUniversity College Dublin, Belfield, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mensud","family":"Hatunic","sequence":"additional","affiliation":[{"name":"1Department of EndocrinologyMater Misericordiae University Hospital, Dublin, Ireland"},{"name":"5UCD School of MedicineUniversity College Dublin, Belfield, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","container-title":["Endocrinology, Diabetes & Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2021\/1\/EDM21-0026.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2021\/1\/EDM21-0026.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:47:38Z","timestamp":1784555258000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/3765"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,1]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1530\/edm-21-0026","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,1]]}}}