Matches in SemOpenAlex for { <https://semopenalex.org/work/W2893313445> ?p ?o ?g. }
Showing items 1 to 90 of
90
with 100 items per page.
- W2893313445 endingPage "4" @default.
- W2893313445 startingPage "1" @default.
- W2893313445 abstract "Studies on genome-wide transcription patterns have shown that many genetic alterations implicated in pheochromocytoma-paraganglioma (P-PGL) syndromes cluster in a common cellular pathway leading to aberrant activation of molecular response to hypoxia in normoxic conditions (the pseudohypoxia hypothesis). Several cases of P-PGL have been reported in patients with cyanotic congenital heart disease (CCHD). Patients affected with CCHD have an increased likelihood of P-PGL compared to those affected with noncyanotic congenital heart disease. One widely supported hypothesis is that chronic hypoxia represents the determining factor supporting this increased risk. We report the case of a 23-year-old woman affected with congenital tricuspid atresia surgically by the Fontan procedure. The patient was admitted to hospital with hypertensive crisis and dyspnea. Chest computed tomography revealed, incidentally, a 6-cm mass in the left adrenal lodge. Increased levels of noradrenaline (NA) and its metabolites were detected (plasma NA 5003.7 pg/ml, n.v.<480; urinary NA 1059.5 µ g/24 h, n.v.<85.5; urinary metanephrine 489 µ g/24 h, n.v.<320). The patient did not report any additional symptom related to catecholamine excess. The left adrenal tumor showed abnormal accumulation when 131I-metaiodobenzylguanidine scintigraphy was performed. A 18F-fluorodeoxyglucose positron emission tomography showed no significant metabolic activity in the left adrenal gland but intense uptake in the supra- and subdiaphragmatic brown adipose tissue, probably due to noradrenergic-stimulated glucose uptake. The patient underwent left open adrenalectomy after preconditioning with α - and β -blockers and histopathological examination confirmed the diagnosis of pheochromocytoma (Ki-67<5%). Screening for germline mutations did not show any genes mutation (investigated mutations: RET, TMEM127, MAX, SDHD, SDHC, SDHB, SDHAF2, SDHA, and VHL). Clinicians should consider P-PGL when an unexplained clinical deterioration occurs in CCHD patients, even in the absence of typical paroxysmal symptoms." @default.
- W2893313445 created "2018-10-05" @default.
- W2893313445 creator A5019533894 @default.
- W2893313445 creator A5030254744 @default.
- W2893313445 creator A5041580911 @default.
- W2893313445 creator A5055186824 @default.
- W2893313445 creator A5072631239 @default.
- W2893313445 creator A5080104899 @default.
- W2893313445 date "2018-09-25" @default.
- W2893313445 modified "2023-10-16" @default.
- W2893313445 title "Pheochromocytoma in Congenital Cyanotic Heart Disease" @default.
- W2893313445 cites W2012517517 @default.
- W2893313445 cites W2027398510 @default.
- W2893313445 cites W2046289207 @default.
- W2893313445 cites W2058480413 @default.
- W2893313445 cites W2091231785 @default.
- W2893313445 cites W2100213871 @default.
- W2893313445 cites W2148813221 @default.
- W2893313445 cites W2610197218 @default.
- W2893313445 cites W2794798512 @default.
- W2893313445 cites W2795960037 @default.
- W2893313445 cites W2884369510 @default.
- W2893313445 doi "https://doi.org/10.1155/2018/2091257" @default.
- W2893313445 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6176301" @default.
- W2893313445 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30356369" @default.
- W2893313445 hasPublicationYear "2018" @default.
- W2893313445 type Work @default.
- W2893313445 sameAs 2893313445 @default.
- W2893313445 citedByCount "4" @default.
- W2893313445 countsByYear W28933134452019 @default.
- W2893313445 countsByYear W28933134452020 @default.
- W2893313445 countsByYear W28933134452022 @default.
- W2893313445 countsByYear W28933134452023 @default.
- W2893313445 crossrefType "journal-article" @default.
- W2893313445 hasAuthorship W2893313445A5019533894 @default.
- W2893313445 hasAuthorship W2893313445A5030254744 @default.
- W2893313445 hasAuthorship W2893313445A5041580911 @default.
- W2893313445 hasAuthorship W2893313445A5055186824 @default.
- W2893313445 hasAuthorship W2893313445A5072631239 @default.
- W2893313445 hasAuthorship W2893313445A5080104899 @default.
- W2893313445 hasBestOaLocation W28933134451 @default.
- W2893313445 hasConcept C126322002 @default.
- W2893313445 hasConcept C134018914 @default.
- W2893313445 hasConcept C142724271 @default.
- W2893313445 hasConcept C164705383 @default.
- W2893313445 hasConcept C2776734335 @default.
- W2893313445 hasConcept C2776783082 @default.
- W2893313445 hasConcept C2779505177 @default.
- W2893313445 hasConcept C2779512018 @default.
- W2893313445 hasConcept C2780074459 @default.
- W2893313445 hasConcept C2780694816 @default.
- W2893313445 hasConcept C71924100 @default.
- W2893313445 hasConcept C77411442 @default.
- W2893313445 hasConceptScore W2893313445C126322002 @default.
- W2893313445 hasConceptScore W2893313445C134018914 @default.
- W2893313445 hasConceptScore W2893313445C142724271 @default.
- W2893313445 hasConceptScore W2893313445C164705383 @default.
- W2893313445 hasConceptScore W2893313445C2776734335 @default.
- W2893313445 hasConceptScore W2893313445C2776783082 @default.
- W2893313445 hasConceptScore W2893313445C2779505177 @default.
- W2893313445 hasConceptScore W2893313445C2779512018 @default.
- W2893313445 hasConceptScore W2893313445C2780074459 @default.
- W2893313445 hasConceptScore W2893313445C2780694816 @default.
- W2893313445 hasConceptScore W2893313445C71924100 @default.
- W2893313445 hasConceptScore W2893313445C77411442 @default.
- W2893313445 hasLocation W28933134451 @default.
- W2893313445 hasLocation W28933134452 @default.
- W2893313445 hasLocation W28933134453 @default.
- W2893313445 hasLocation W28933134454 @default.
- W2893313445 hasLocation W28933134455 @default.
- W2893313445 hasLocation W28933134456 @default.
- W2893313445 hasOpenAccess W2893313445 @default.
- W2893313445 hasPrimaryLocation W28933134451 @default.
- W2893313445 hasRelatedWork W1420833956 @default.
- W2893313445 hasRelatedWork W1986980947 @default.
- W2893313445 hasRelatedWork W2167706723 @default.
- W2893313445 hasRelatedWork W2416987420 @default.
- W2893313445 hasRelatedWork W2893313445 @default.
- W2893313445 hasRelatedWork W2982609970 @default.
- W2893313445 hasRelatedWork W4229331744 @default.
- W2893313445 hasRelatedWork W4283034524 @default.
- W2893313445 hasRelatedWork W4288884580 @default.
- W2893313445 hasRelatedWork W2102763509 @default.
- W2893313445 hasVolume "2018" @default.
- W2893313445 isParatext "false" @default.
- W2893313445 isRetracted "false" @default.
- W2893313445 magId "2893313445" @default.
- W2893313445 workType "article" @default.