Læknablaðið - 01.03.2023, Page 29

L ÆKNABL AÐIÐ 2023/109 145
R A N N S Ó K N
Heimildir
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75.
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pheochromocytoma. J Am Coll Surg 2009; 209: 727-32.
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syndrome: no longer the 10% tumor. J Surg Oncol 2005; 89: 193-201.
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Syndromes. 2008 May 21. In: Adam MP, Everman DB, Mirzaa GM, et al, ritstj.
GeneReviews®. University of Washington, Seattle 1993-2023.
6. Hajjar I, Kotchen TA. Trends in prevalence, awareness, treatment, and control of hyper-
tension in the United States, 1988-2000. JAMA 2003; 290: 199-206.
7. Calhoun DA, Jones D, Textor S, et al. Resistant hypertension: diagnosis, evaluation,
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117: e510-e526.
Greinin barst til blaðsins 5. desember 2022,
samþykkt til birtingar 10. mars 2023.
E N G L I S H S U M M A R Y
Resistant hypertension – pheochromocytoma
We report a case of a man with a 30-year history of treatment-resistant hypertension,
hydropoiesis, tachycardic spells and dysgeusia. Despite repeated visits to the
emergency department and work-up in an out-patient clinic, the diagnosis was
unknown. Three years prior to remittance to an endocrinologist, the hypertension
worsened, and he developed diabetes type-II. Further work-up revealed a 3 cm extra-
adrenal pheochromocytoma, a paraganglioma. After surgical removal of the tumor, he
is without medication and symptom free.
Pheochromocytoma and paraganglioma are rare causes of hypertension, estimated
to explain 0.1-0.6% of all cases, but nonetheless an important diagnosis to make, due
to serious side effects.
doi 10.17992/lbl.2023.03.735
Krister Blær Jónsson1
Eiríkur Orri Guðmundsson2
Margrét Sigurðardóttir3
Jón Jóhannes Jónsson1,4
Helga Ágústa Sigurjónsdóttir1,5
1Faculty of Medicine, University of Iceland, 2department of urology,
Landspítali National University Hospital of Iceland, 3department of
pathology, Landspítali National University Hospital of Iceland, 4department
of genetics, Landspítali National University Hospital of Iceland,
5department of internal medicine, Landspítali National University Hospital
of Iceland.
Correspondence: Krister Blær Jónsson, kristerb@landspitali.is
Key words: pheochromocytoma, paraganglioma, resistant hypertension,
neuroendocrine tumor, metanephrine.
8. Manger WM, Gifford RW. Pheochromocytoma. J Clin Hypertens 2002; 4: 62-72.
9. Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an
endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99: 1915-42.
10. O'Riordan JA. Pheochromocytomas and anesthesia. Int Anesthesiol Clin 1997; 35: 99-127.
11. Quezado ZN, Keiser HR, Parker MM. Reversible myocardial depression after massive
catecholamine release from a pheochromocytoma. Crit Care Med 1997; 20: 549-51.
12. Tauzin-Fin P, Hilbert G, Krol-Houdek M, et al. Mydriasis and acute pulmonary oedema
complicating laparoscopic removal of phaechromocytoma. Anaesth Intensive Care
1999; 27: 646-9.
13. Roizen MF, Schneider BD, Hassan SZ Anaesthesia for patients with
pheochromocytoma. Anaesthesiol Clin North Am 1987; 5: 269-75.
14. Augoustides JG, Abrams M, Berkowitz D, et al. Vasopressin for hemodynamic rescue in
catecholamine-resistant vasoplegic shock after resection of massive pheochromocytoma.
Anesthesiology 2004; 101: 1022-4.
Langtímahorfur eftir aðgerð eru mjög góðar en um 50% sjúk-
linga eru taldir vera með háþrýsting eftir aðgerð. Endurmeta á
sermisgildi katekólamína 14-28 dögum eftir aðgerð og er endur-
komutíðni æxlisins 17%. Enn hefur ekki tekist að meta endur-
komulíkur með fullnægjandi hætti, þrátt fyrir framfarir í erfða-
og meinafræði. Mælt er því með því að sjúklingar haldi áfram í
langtíma eftirfylgd hjá innkirtlalækni.9
Hér er lýst sjúkratilfelli með hjáhnoðuæxli og einkennandi
sögu einkenna til allt að 30 ára. Upphaflega var til staðar lyfja-
þolinn háþrýstingur, hraðtaktarköst og svitamyndun. Athyglis-
vert er að í uppvinnslu á þessum meðferðarþolna háþrýstingi
höfðu ekki verið gerðar tilraunir til að nálgast undirliggjandi
orsök með til dæmis hormónamælingum. Þá þyrfti einnig að
hafa í huga orsakir eins og aldósterónofgnótt og nýrnaslagæða-
þrengsli. Læknar þurfa að vera vakandi fyrir sjaldgæfum orsök-
um háþrýstings, ekki síst þegar um dæmigerða einkennamynd
er að ræða. Eftirsóknarvert er að finna læknanlegar orsakir há-
þrýstings. Með markvissari og nákvæmari greiningaraðferðum
er nú mun auðveldara að greina sjaldgæfar orsakir háþrýstings
eins og KogH.