• Klíčová slova
• PHEOCHROMOCYTOMA/diagnosis *,
• MeSH
• feochromocytom diagnóza   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Pheochromocytoma (pheo) is adrenal or less frequently extraadrenal tumour of chromafine tissue. Pheos are rare, but cardiovascular and metabolic abnormalities are common. Unrecognised pheo may lead to fatal hypertensive crisis during anesthesia or other stresses. Proper diagnosis of pheo is thus of utmost importance. 24-h blood pressure (BP) monitoring may contribute to the diagnosis of pheo due to increased BP variability and absence of night BP decline. Pheo contains large amount of enzyme catechol-O-methyl transpherase (COMT) with subsequent excessive production of COMT metabolites like metanephrines. Measurement of plasma free metanephrines or urinary fraccionated metanephrines has usually higher sensivitivity and specificity compared with plasma or urinary catecholamines. Morphological diagnosis of adrenal/extraadrenal pheo is based on CT/MR visualisation and 123I-metaiodobenzylguanidin (MIBG) or PET 18F-fluorodeoxyglucose scan. Genetic analysis should be performed in all confirmed pheo cases, especially in younger subjects below 50 years of age in order to detect mutations of following genes: von Hippel-Lindau (VHL), RET- protooncogen, genes encoding B, C and D subunit of mitochondrial sukcinat dehydrogenaze (SDHB, SDHC, SDHD) and neurofibromatosis type I gene. Pharmacological treatment is based on alpha blockers with subsequent (after 24-48 hours) administration of beta-blockers/especially in patients with tendency to tachycardia/. Following this therapy normalisation of BP is common and laparoscopic excision of pheo tumour can be realised. Malignant pheos are difficult to treat due to early occurrence of metastasis and lack of response to chemotherapy or iradiation in most cases.

• MeSH
• diferenciální diagnóza MeSH
• feochromocytom diagnóza   terapie   MeSH
• lidé MeSH
• nádory nadledvin diagnóza   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH

Pheochromocytoma may present with various clinical signs, symptoms due to continuous and/or paroxysmal release of catecholamines. Arterial hypertension may be sustained and/or paroxysmal and palpitations are mainly due to sinus tachycardia. In some cases, even as the first manifestation of pheochromocytoma, may occur severe cardiovascular complications such as hypertensive emergency, myocardial ischemia, cardiomyopathy and heart failure, multisystem crisis or shock. Catecholamine release may be also associated with arrhythmias - tachycardias (supraventricular or ventricular) or less frequently bradycardias (AV blocks and junctional). The effect of catecholamines is not restricted to myocardium, but may also lead to cerebrovascular impairment such as transient ischemic attack or stroke. As many of these complications may be life-threatening, the only prevention is early diagnosis of pheochromocytoma and proper treatment, in particular in specialized centers.

• MeSH
• časná diagnóza * MeSH
• diferenciální diagnóza MeSH
• feochromocytom diagnóza   MeSH
• lidé MeSH
• nádory nadledvin diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

• Klíčová slova
• ABORTION, THERAPEUTIC *, DIAGNOSIS, DIFFERENTIAL *, MANDELIC ACID *, PHEOCHROMOCYTOMA *, PREGNANCY *, PREGNANCY COMPLICATIONS *, URINE *,
• MeSH
• diferenciální diagnóza * MeSH
• feochromocytom * MeSH
• komplikace těhotenství * MeSH
• kyseliny mandlové * MeSH
• lidé MeSH
• moč * MeSH
• potrat léčebný * MeSH
• těhotenství MeSH
• Check Tag
• lidé MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• 3-methoxy-4-hydroxymandelic acid MeSH Prohlížeč
• 4-hydroxymandelic acid MeSH Prohlížeč
• kyseliny mandlové * MeSH
• mandelic acid MeSH Prohlížeč

• Klíčová slova
• DIAGNOSIS *, HYPERTENSION *, HYPNOTICS AND SEDATIVES *, PHENTOLAMINE *, PHEOCHROMOCYTOMA *, RESERPINE *,
• MeSH
• diagnóza * MeSH
• fentolamin * MeSH
• feochromocytom * MeSH
• hypertenze * MeSH
• hypnotika a sedativa * MeSH
• lidé MeSH
• reserpin * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• fentolamin * MeSH
• hypnotika a sedativa * MeSH
• reserpin * MeSH

This work discusses the clinical performance of deconjugated metanephrine (MN), normetanephrine (NMN) and 3-methoxytyramine (3MT) determined in the basal first morning urine using a chromatographic method with electrochemical detection for the clinical diagnosis of pheochromocytoma (PHEO) and paraganglioma (PGL). Urine samples were collected from 44 patients (36 with PHEO, 8 with PGL) aged 54+/-17 (20-78) years (22 females, 22 males). A sampling of biological materials was performed preoperatively and about one week, six months and one year after adrenal gland surgery. The control group consisted of 34 PHEO/PGL patients more than 4 months after adrenal gland surgery. All subjects in the control group were without a diagnosis of PHEO or PGL. Clinical sensitivity was 55 % for MN, 64 % for NMN, 80 % for combination of both MN and NMN, and only 23 % for 3TM. Clinical specificity calculated from the control group was 93 % for MN, 95 % for NMN, 95 % for the combination MN and NMN, and 97 % for 3TM. Cut-off values for deconjugated metanephrines in the basal urine were 310 (MN), 690 (NMN) and 250 microg/l (3MT). Chromatographic determination of deconjugated urinary metanephrines, which is simple without the necessity of special laboratory material, can serve for the screening of PHEO or PGL patients. Urine NMN and 3MT exerts an association to malignity, and all markers are associated with tumor mass. However, the principal laboratory diagnosis of PHEO or PGL must be based on plasma-free metanephrines and plasma chromogranin A with better performance in the laboratory diagnosis of PHEO or PGL.

• MeSH
• biologické markery moč   MeSH
• dopamin analogy a deriváty   moč   MeSH
• dospělí MeSH
• feochromocytom diagnóza   moč   MeSH
• klinické laboratorní techniky metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• metanefrin moč   MeSH
• mladý dospělý MeSH
• nádory nadledvin diagnóza   moč   MeSH
• normetanefrin moč   MeSH
• paragangliom diagnóza   moč   MeSH
• senioři MeSH
• vysokoúčinná kapalinová chromatografie metody   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• 3-methoxytyramine MeSH Prohlížeč
• biologické markery MeSH
• dopamin MeSH
• metanefrin MeSH
• normetanefrin MeSH

Pheochromocytoma (PHEO) is considered to be a rare cause of hypertension. However, if left untreated, PHEOs may lead to fatal hypertensive crises during anesthesia and other stresses. The diagnosis of PHEO is therefore extremely important. A 24-hour blood pressure (BP) pattern per se might be of some diagnostic value due to frequently observed higher BP variability as well as an attenuated night-time BP decrease. So far, germline mutations in five genes have been identified to be responsible for familial PHEOs: the von Hippel-Lindau gene, which causes von Hippel-Lindau syndrome, the RET gene leading to multiple endocrine neoplasia type 2, the neurofibromatosis type 1 gene, which is associated with von Recklinghausen's disease and the genes encoding the B and D subunits of mitochondrial succinate dehydrogenase (SDHB, SDHD), which are associated with familial paragangliomas and PHEOs. Genetic analysis should be offered to those patients with confirmed PHEO who are 50 years old or younger. Plasma-free metanephrines or urinary fractionated metanephrines seem to have higher diagnostic values compared to plasma or urinary catecholamines for the biochemical diagnosis of PHEO. Imaging with (123)I-metaiodobenzylguanidine or (18)F-fluorodopamine PET, if available, are in addition to CT/MRI useful for the detection of multifocal/extra-adrenal forms. Appropriate pharmacologic treatment with subsequent laparoscopic extirpation of PHEO is usually successful in benign forms. There is, however, no convincingly effective mode of treatment in malignant PHEOs.

• MeSH
• feochromocytom diagnóza   farmakoterapie   genetika   metabolismus   patofyziologie   MeSH
• krevní tlak fyziologie   MeSH
• lidé MeSH
• nádory nadledvin diagnóza   farmakoterapie   genetika   metabolismus   patofyziologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

This work discusses the clinical performance of chromogranin A, free metanephrine and normetanephrine determination in plasma using a radioimmunoanalytical methods for the diagnosis of pheochromocytoma and paraganglioma. Blood samples were collected from 55 patients (46 pheochromocytomas, 9 paragangliomas). A sampling of biological materials was performed preoperatively and about one week, six months and one year after adrenal gland surgery. The comparative group without a diagnosis of pheochromocytoma/paraganglioma consisted of 36 pheochromocytoma/paraganglioma patients more than 4 months after adrenal gland surgery, and of 87 patients, 16 of them with multiple endocrine neoplasia, 9 with medullary and 5 with parafolicullar carcinoma of the thyroid gland. The rest were patients with various adrenal gland disorders. Chromogranin A, metanephrine and normetanephrine were determined in the EDTA-plasma using a radioimmunoassay kits Cisbio Bioassays, France and IBL International GmbH, Germany. Clinical sensitivity was 96 % for the combination of metanephrine and normetanephrine, and 93 % for chromogranin A. Clinical specificity was 100 % for the combination metanephrine and normetanephrine, and 96 % for chromogranin A. Falsely elevated levels of chromogranin A were observed in 1 patient with chronic renal insufficiency and 9 analyses were influenced by the administration of proton pump inhibitors. These results were excluded of CGA specificity. Both the combination of plasma free metanephrine, normetanephrine and chromogranin A as determined by radioimmunoassays, which are simple without the necessity of special laboratory material, are effective markers of pheochromocytoma or paraganglioma. Chromogranin A exerts association to malignity and all markers are associated with tumor mass.

• MeSH
• chromogranin A krev   MeSH
• dospělí MeSH
• feochromocytom krev   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• metanefrin krev   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádorové biomarkery krev   MeSH
• nádory nadledvin krev   diagnóza   MeSH
• normetanefrin krev   MeSH
• radioimunoanalýza metody   MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• chromogranin A MeSH
• metanefrin MeSH
• nádorové biomarkery MeSH
• normetanefrin MeSH

This work discusses the clinical performance of chromogranin A (CGA), a commonly measured marker in neuroendocrine neoplasms, for the diagnosis of pheochromocytoma/paraganglioma (PPGL). Plasma CGA (cut-off value 150 µg/L) was determined by an immunoradiometric assay. Free metanephrine (cut-off value 100 ng/L) and normetanephrine (cut-off value 170 ng/L) were determined by radioimmunoassay. Blood samples were collected from PPGL patients preoperatively, one week, six months, one year and two years after adrenal gland surgery. The control patients not diagnosed with PPGL suffered from adrenal problems or from MEN2 and thyroid carcinoma. The clinical sensitivity in the PPGL group of patients (n = 71) based on CGA is 90% and is below the clinical sensitivity determined by metanephrines (97%). The clinical specificity based on all plasma CGA values after surgery (n = 98) is 99% and is the same for metanephrines assays. The clinical specificity of CGA in the control group (n = 85) was 92% or 99% using metanephrines tests. We can conclude that plasma CGA can serve as an appropriate complement to metanephrines assays in laboratory diagnosis of PPGL patients. CGA is elevated in PPGLs, as well as in other neuroendocrine or non-neuroendocrine neoplasia and under clinical conditions increasing adrenergic activity.

• Klíčová slova
• chromogranin A, metanephrines, paraganglioma, pheochromocytoma,
• Publikační typ
• časopisecké články MeSH

Serious predominantly diastolic treatment-resistant hypertension with unstable blood pressure values draws first contact physician's attention to a possibility of pheochromocytoma, although in some periods of disease blood pressure values of a patient can be normal or only slightly elevated. Clinical picture is sometimes atypical, can be overlooked or lead to a wrong diagnosis. The author brings forward atypical cases where pheochromocytoma emulates different disease or diseases with clinical pictures similar to that of pheochromocytoma.

• MeSH
• diferenciální diagnóza MeSH
• feochromocytom diagnóza   MeSH
• hypertenze etiologie   MeSH
• lidé MeSH
• nádory nadledvin diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH