{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:18:31Z","timestamp":1747185511113,"version":"3.40.5"},"reference-count":25,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2022,11,19]],"date-time":"2022-11-19T00:00:00Z","timestamp":1668816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Phaeochromocytomas (PCC) and paragangliomas (PGL; together PPGL) are rare tumours of the adrenal medulla or extra\u2010adrenal paraganglia. They may secrete catecholamines with significant cardiovascular effects. Management of PPGL is predominantly surgical, despite the anaesthetic risks related to potential haemodynamic instability. Meticulous pre\u2010treatment and intra\u2010operative management are required to improve cardiovascular outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>There are limited local data regarding the incidence of PPGL and the clinical characteristics of individuals diagnosed with these tumours in New Zealand. We undertook a retrospective study investigating the local practice and patient characteristics with an additional focus on intra\u2010operative haemodynamic stability and post\u2010operative outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Electronic patient records were searched for individuals with a diagnosis of PPGL. Clinical records and electronic databases were interrogated for pre\u2010operative, intra\u2010operative and post\u2010operative data points. Particular attention was paid to rates and types of germline mutations, intra\u2010operative haemodynamic stability and post\u2010operative renal and cardiovascular outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 49 individuals with PPGL, of whom 34 were from the local area. This gave a local incidence of PPGL of around five cases per million people per year. M\u0101ori were significantly over\u2010represented in our cohort, with this being in part due to high rates of the <jats:italic>SDHB<\/jats:italic> R46Q mutation.<\/jats:p><jats:p>Over 95% of our cohort met pre\u2010specified pre\u2010operative blood pressure parameters. Intra\u2010operative monitoring revealed a tendency to hypotension, but this did not translate into adverse post\u2010operative outcomes, which were infrequent.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>M\u0101ori were over\u2010represented due to high rates of germline <jats:italic>SDHB<\/jats:italic> R46Q mutations. There were few post\u2010operative adverse outcomes in this contemporary cohort.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.15965","type":"journal-article","created":{"date-parts":[[2022,11,8]],"date-time":"2022-11-08T14:41:31Z","timestamp":1667918491000},"page":"1883-1889","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["High rates of the <scp>SDHB p.Arg46Gln<\/scp> pathogenic variant predisposes New Zealand M\u0101ori to phaeochromocytoma\/paraganglioma"],"prefix":"10.1111","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8444-4201","authenticated-orcid":false,"given":"James A. 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