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Elevated circulating catecholamine level

MedGen UID:
871156
Concept ID:
C4025629
Finding
HPO: HP:0003334

Definition

An abnormal increase in catecholamine concentration in the blood. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated circulating catecholamine level

Conditions with this feature

Paragangliomas 3
MedGen UID:
340200
Concept ID:
C1854336
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck paragangliomas [HNPGLs]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, extra-adrenal sympathetic paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCCs result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas. Additional tumors reported in individuals with hereditary PGL/PCC syndromes include gastrointestinal stromal tumors (GISTs), pulmonary chondromas, and clear cell renal cell carcinoma.
Paragangliomas 1
MedGen UID:
488134
Concept ID:
C3494181
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck paragangliomas [HNPGLs]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, extra-adrenal sympathetic paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCCs result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas. Additional tumors reported in individuals with hereditary PGL/PCC syndromes include gastrointestinal stromal tumors (GISTs), pulmonary chondromas, and clear cell renal cell carcinoma.
Paragangliomas 6
MedGen UID:
1681559
Concept ID:
C5193112
Disease or Syndrome
Pheochromocytoma/paraganglioma syndrome-6 (PPGL6) is an autosomal dominant adult-onset tumor predisposition syndrome in which affected individuals develop neuroendocrine neoplasms, known as paragangliomas. Many tumors arise in the abdomen, although some may arise in other regions, including the head and neck. Some of the tumors may secrete biologically active normetanephrines, resulting in secondary hypertension. Tumors may be benign or malignant, and some may metastasize (summary by Buffet et al., 2018). For a discussion of genetic heterogeneity of pheochromocytoma/paraganglioma syndrome, see PPGL1 (168000).

Professional guidelines

PubMed

Gupta G, Pacak K; AACE Adrenal Scientific Committee
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Recent clinical studies

Etiology

Ryabov V, Dil S, Vyshlov E, Mochula O, Kercheva M, Baev A, Gergert E, Maslov L
Am J Cardiol 2024 Sep 1;226:118-127. Epub 2024 Jul 25 doi: 10.1016/j.amjcard.2024.07.011. PMID: 39025195
Nef HM, Möllmann H, Akashi YJ, Hamm CW
Nat Rev Cardiol 2010 Apr;7(4):187-93. Epub 2010 Mar 2 doi: 10.1038/nrcardio.2010.16. PMID: 20195267
MacGowan GA, Mann DL, Kormos RL, Feldman AM, Murali S
Am J Cardiol 1997 Apr 15;79(8):1128-31. doi: 10.1016/s0002-9149(96)00063-x. PMID: 9114781
Leslie JB
Acta Anaesthesiol Scand Suppl 1993;99:5-9. doi: 10.1111/j.1399-6576.1993.tb03816.x. PMID: 8480508
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Diagnosis

Gao L, Zhao Y, Wu H, Lin X, Guo F, Li J, Long Y, Zhou B, She J, Zhang C, Sheng J, Jin L, Wu Y, Huang H
Circulation 2023 Dec 12;148(24):1958-1973. Epub 2023 Nov 8 doi: 10.1161/CIRCULATIONAHA.123.065827. PMID: 37937441Free PMC Article
Mamilla D, Gonzales MK, Esler MD, Pacak K
Endocrinol Metab Clin North Am 2019 Dec;48(4):751-764. Epub 2019 Sep 18 doi: 10.1016/j.ecl.2019.08.004. PMID: 31655774Free PMC Article
Gamcrlidze MM, Intskirveli NA, Vardosanidze KD, Chikhladze KhE, Goliadze LSh, Ratiani LR
Georgian Med News 2015 Feb;(239):56-62. PMID: 25802451
Lindner W, Behnisch W, Kunz U, Debatin KM, Pohlandt F
Eur J Pediatr 2001 Jul;160(7):436-8. doi: 10.1007/s004310100772. PMID: 11475582
Sowers JR, Mohanty PK
J Hypertens Suppl 1988 Nov;6(1):S49-54. PMID: 3063794

Therapy

Ryabov V, Dil S, Vyshlov E, Mochula O, Kercheva M, Baev A, Gergert E, Maslov L
Am J Cardiol 2024 Sep 1;226:118-127. Epub 2024 Jul 25 doi: 10.1016/j.amjcard.2024.07.011. PMID: 39025195
Ahlskog JE
Parkinsonism Relat Disord 2023 Apr;109:105357. Epub 2023 Mar 6 doi: 10.1016/j.parkreldis.2023.105357. PMID: 36922273
Nybo L, Nielsen B, Blomstrand E, Moller K, Secher N
J Appl Physiol (1985) 2003 Sep;95(3):1125-31. Epub 2003 May 16 doi: 10.1152/japplphysiol.00241.2003. PMID: 12754171
Packer M, Lee WH, Kessler PD, Gottlieb SS, Bernstein JL, Kukin ML
Circulation 1987 May;75(5 Pt 2):IV80-92. PMID: 2882867
Cassidy JP, Phero JC, Grau WH
Anesth Prog 1986 Nov-Dec;33(6):289-97. PMID: 3544965Free PMC Article

Prognosis

Sobotka PA, Krum H, Böhm M, Francis DP, Schlaich MP
Curr Cardiol Rep 2012 Jun;14(3):285-92. doi: 10.1007/s11886-012-0258-x. PMID: 22392370
Petrov IS, Tokmakova MP, Marchov DN, Kichukov KN
Folia Med (Plovdiv) 2011 Apr-Jun;53(2):5-12. doi: 10.2478/v10153-010-0031-0. PMID: 21797101
Salem RM, Cadman PE, Chen Y, Rao F, Wen G, Hamilton BA, Rana BK, Smith DW, Stridsberg M, Ward HJ, Mahata M, Mahata SK, Bowden DW, Hicks PJ, Freedman BI, Schork NJ, O'Connor DT
J Am Soc Nephrol 2008 Mar;19(3):600-14. Epub 2008 Jan 30 doi: 10.1681/ASN.2007070754. PMID: 18235090Free PMC Article
Lindner W, Behnisch W, Kunz U, Debatin KM, Pohlandt F
Eur J Pediatr 2001 Jul;160(7):436-8. doi: 10.1007/s004310100772. PMID: 11475582
Leslie JB
Acta Anaesthesiol Scand Suppl 1993;99:5-9. doi: 10.1111/j.1399-6576.1993.tb03816.x. PMID: 8480508

Clinical prediction guides

Gao L, Zhao Y, Wu H, Lin X, Guo F, Li J, Long Y, Zhou B, She J, Zhang C, Sheng J, Jin L, Wu Y, Huang H
Circulation 2023 Dec 12;148(24):1958-1973. Epub 2023 Nov 8 doi: 10.1161/CIRCULATIONAHA.123.065827. PMID: 37937441Free PMC Article
Graham J, Hobson D, Ponnampalam A
Med Hypotheses 2014 Dec;83(6):819-21. Epub 2014 Oct 31 doi: 10.1016/j.mehy.2014.10.016. PMID: 25468785
MacGowan GA, Mann DL, Kormos RL, Feldman AM, Murali S
Am J Cardiol 1997 Apr 15;79(8):1128-31. doi: 10.1016/s0002-9149(96)00063-x. PMID: 9114781
Leslie JB
Acta Anaesthesiol Scand Suppl 1993;99:5-9. doi: 10.1111/j.1399-6576.1993.tb03816.x. PMID: 8480508
Vaughan GM, Pelham RW, Pang SF, Loughlin LL, Wilson KM, Sandock KL, Vaughan MK, Koslow SH, Reiter RJ
J Clin Endocrinol Metab 1976 Apr;42(4):752-64. doi: 10.1210/jcem-42-4-752. PMID: 1262447

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