CONTEXT: Impaired glucose homeostasis is a common finding in pheochromocytoma (PHEO), especially with adrenergic phenotype. The possible contribution of incretin dysfunction to dysglycemia in PHEO patients has not been studied. OBJECTIVE: To compare changes in pancreatic endocrine function and gut hormones' production during a liquid meal test before and 1 year after adrenalectomy. METHODS: In a prospective study, we included 18 patients with PHEO (13 females) with adrenergic biochemical phenotype. A liquid meal test with predefined isocaloric enteral nutrition was performed to evaluate dynamic changes in pancreatic hormones and incretins. RESULTS: During the meal test, insulin levels were significantly lower before adrenalectomy only in the early phase of insulin secretion, but changes in area under the curve (AUC) did not reach statistical significance (AUC = 0.07). Plasma glucagon (AUC < 0.01) and pancreatic polypeptide levels (AUC < 0.01) were suppressed in comparison with the postoperative state. Impaired response to the meal was found preoperatively for glucagon-like peptide-1 (GLP-1; AUC P < 0.05), but not glucose-dependent insulinotropic polypepide (GIP; AUC P = 0.21). No significant changes in insulin resistance indices were found, except for the homeostatic model assessment-beta index, an indicator of the function of islet β cells, which negatively correlated with plasma metanephrine (R = -0.66, P < 0.01). CONCLUSIONS: Our study shows suppression of pancreatic α and β cell function and impaired GLP-1 secretion during a dynamic meal test in patients with PHEO, which is improved after its surgical treatment. These data demonstrate a novel and potentially significant interconnection between excessive catecholamine production and the secretion of glucoregulatory hormones.

• Klíčová slova
• glucagon, incretins, insulin, meal test, pheochromocytoma, secondary diabetes mellitus,
• MeSH
• adrenergní látky škodlivé účinky   MeSH
• biologické markery metabolismus   MeSH
• dospělí MeSH
• fenotyp MeSH
• feochromocytom komplikace   MeSH
• gastrointestinální hormony metabolismus   MeSH
• glukagonu podobný peptid 1 metabolismus   MeSH
• inkretiny škodlivé účinky   MeSH
• jídla MeSH
• krevní glukóza analýza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory nadledvin komplikace   MeSH
• následné studie MeSH
• pankreatické hormony metabolismus   MeSH
• porucha glukózové tolerance etiologie   metabolismus   patologie   MeSH
• prognóza MeSH
• prospektivní studie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• adrenergní látky MeSH
• biologické markery MeSH
• gastrointestinální hormony MeSH
• glukagonu podobný peptid 1 MeSH
• inkretiny MeSH
• krevní glukóza MeSH
• pankreatické hormony MeSH

CONTEXT: Impaired diurnal blood pressure (BP) variability is related to higher cardiovascular risk. OBJECTIVE: To assess diurnal variability of BP and its relation to target organ damage (TOD) and catecholamine phenotype in a consecutive sample of pheochromocytoma/paraganglioma (PPGL). DESIGN: We included 179 patients with PPGL All patients underwent 24 hours of ambulatory BP monitoring to determine dipping status. Differences in plasma metanephrine or urine adrenaline were used to distinguish catecholamine biochemical phenotype. To evaluate TOD, renal functions, presence of left ventricle hypertrophy (LVH), and the subgroup (n = 111) carotid-femoral pulse wave velocity (PWV) were assessed. Structural equation modeling was used to find the relationship among nocturnal dipping, catecholamine phenotype, and TOD parameters. RESULTS: According to the nocturnal dipping, patients were divided into the three groups: dippers (28%), nondippers (40%), and reverse dippers (32%). Reverse dippers were older (P < 0.05), with a higher proportion of noradrenergic (NA) phenotype (P < 0.05), a higher prevalence of diabetes mellitus (P < 0.05), and sustained arterial hypertension (P < 0.01) and its duration (P < 0.05), as opposed to the other groups. All parameters of TOD were more pronounced only in reverse dippers compared with nondippers and dippers. The presence of NA phenotype (=absence of adrenaline production) was associated with reverse dipping and TOD (LVH and PWV). CONCLUSIONS: Patients with reverse dipping had more substantial TOD compared with other groups. The NA phenotype plays an important role, not only in impaired diurnal BP variability but also independently from dipping status in more pronounced TOD of heart and vessels.

• MeSH
• dospělí MeSH
• fenotyp MeSH
• feochromocytom komplikace   metabolismus   patofyziologie   MeSH
• katecholaminy analýza   metabolismus   MeSH
• krevní tlak * MeSH
• lidé středního věku MeSH
• lidé MeSH
• měření krevního tlaku MeSH
• nádory nadledvin komplikace   metabolismus   patofyziologie   MeSH
• paragangliom komplikace   metabolismus   patofyziologie   MeSH
• retrospektivní studie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• katecholaminy MeSH

BACKGROUND: Neurofibromatosis type-1 (NF1), also called von Recklinghausen disease, is a rare genetic disease which can lead to the development of benign or even malignant tumors. NF1 is mostly diagnosed in children or early adolescents who present with clinical symptoms. A curative therapy is still missing and the management of NF1 is based on careful surveillance. Concerning tumors which affect the gastrointestinal tract in patients with NF1, the most common is a gastrointestinal stromal tumor (GIST). CASE PRESENTATION: We present a case of a 58-year-old adult patient with dyspeptic symptoms who was incidentally diagnosed with triple malignancy (pheochromocytoma, multiple GISTs of small intestine and an ampullary NET) as a first manifestation of NF1. The patient underwent surgical treatment (adrenalectomy and pancreaticoduodenectomy) with no complications and after 2 years remains in oncological remission. CONCLUSION: NF1 is a rare genetic disease which can cause various benign or malignant tumors. The coincidence of GIST and NET is almost pathognomonic for NF1 and should raise a suspicion of this rare disorder in clinical practice.

• Klíčová slova
• Gastrointestinal stromal tumor, Neuroendocrine tumor, Neurofibromatosis type-1, Pheochromocytoma, von Recklinghausen disease,
• MeSH
• feochromocytom komplikace   diagnostické zobrazování   genetika   patologie   MeSH
• gastrointestinální stromální tumory komplikace   diagnostické zobrazování   genetika   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• neuroendokrinní nádory komplikace   dietoterapie   genetika   patologie   MeSH
• neurofibromatóza 1 komplikace   diagnostické zobrazování   genetika   patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

AIM: The aim of this article is to provide an overview of the most frequent clinically significant adrenal diseases and to describe the latest trends in their diagnostics, particularly by means of imaging techniques. METHODS: The authors reviewed standard textbooks and subsequently conducted a search using the PubMed (Public/Publisher MEDLINE) electronic database by the year 2013 with the following search terms: adrenal masses, adrenal adenoma, phaeochromocytoma, adrenocortical carcinoma, metastases, incidentalomas, hypercortisolism, hyperaldosteronism. RESULTS: If adrenal disease is clinically suspected, hormone tests are performed to detect adrenal hyperfunction and imaging studies are used to assess the nature of adrenal lesion. The most frequent syndromes include hypercortisolism, primary hyperaldosteronism, and phaeochromocytoma. The clinically most significant pathologies of the adrenal glands are adenomas and adrenal hyperplasia, adrenocortical carcinomas, phaeochromocytomas, and metastases. Given the availability and improved quality of imaging techniques, adrenal incidentalomas are detected increasingly often. In these cases, it is necessary to rule out hormonal activity and malignancy. Incidentalomas can be associated with clinical syndromes of adrenal hormone overproduction. In most cases, they are clinically silent. In some cases, the definitive diagnosis can be determined as early as during the initial examination with an imaging technique (most frequently, a CT scan). If the finding is inconsistent, other imaging techniques can be used: CT contrast washout analysis, MRI, SPECT or PET/CT. CONCLUSION: In the case of adrenal gland disorders, correct interpretation of the results of laboratory tests and imaging studies is essential for further management of these patients.

• MeSH
• adenom komplikace   diagnóza   MeSH
• asymptomatické nemoci MeSH
• Cushingův syndrom etiologie   MeSH
• cysty diagnóza   MeSH
• diferenciální diagnóza MeSH
• feochromocytom komplikace   diagnóza   MeSH
• hematom diagnóza   MeSH
• hyperaldosteronismus etiologie   MeSH
• karcinom komplikace   diagnóza   MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• nádory nadledvin komplikace   diagnóza   sekundární   MeSH
• náhodný nález MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

Excess of catecholamines in pheochromocytoma is usually accompanied with classical symptoms and signs. In some cases, severe cardiovascular complications (e. g., heart failure, myocardial infarction) may occur. We performed a retrospective analysis focused on the incidence of cardiovascular complications (classified as follows: arrhythmias, myocardial involvement or ischemia and atherosclerosis, cerebrovascular impairment) before the establishment of diagnosis of pheochromocytoma among 145 subjects treated in our hospital. Cardiovascular complications occurred in 28 subjects, but these subjects did not differ significantly from subjects without complications in age, gender, body mass index, paroxysmal symptoms, symptom duration, tumor dimension, catecholamine secretory phenotype, and incidence of hypertension or diabetes mellitus. Arrhythmias occurred in 15 subjects (2 arrhythmia types in 2 subjects): atrial fibrillation in 9 subjects, supraventricular tachycardia in 3 cases, and ventricular tachycardia in 2 patients. Significant bradycardia was noted in 3 cases. Five subjects presented with heart failure with decreased systolic function (takotsubo-like cardiomyopathy found in 2 cases). One subject suffered from hypertrophic obstructive cardiomyopathy. Seven subjects presented with non-ST-segment elevation myocardial infarction, 2 patients with ST-segment myocardial infarction, and 1 subject underwent coronary artery bypass grafting. Two subjects suffered from significant peripheral atherosclerosis. Among cerebrovascular complications, transient ischemic attack was found in 3 cases, 2 subjects suffered from stroke, and subarachnoidal bleeding occurred in 1 patient. One subject suffered from diffuse neurological impairment due to multiple ischemic white matter lesions. These data show relatively high incidence of cardiovascular complications (19.3%) in subjects with pheochromocytoma. Early diagnosis is mandatory to prevent severe complications in pheochromocytoma.

• MeSH
• dospělí MeSH
• feochromocytom komplikace   metabolismus   MeSH
• incidence MeSH
• kardiovaskulární nemoci diagnóza   epidemiologie   etiologie   MeSH
• katecholaminy metabolismus   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory nadledvin komplikace   metabolismus   MeSH
• retrospektivní studie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• katecholaminy MeSH

OBJECTIVE: To investigate the effect of long-term catecholamine excess in pheochromocytoma on leukocyte and platelet count and on proteins of acute-phase response. METHODS: Fifteen subjects with pheochromocytoma, 16 with primary aldosteronism, 18 with essential hypertension and 17 healthy controls were studied. Sixteen subjects with pheochromocytoma were investigated after tumor removal. Leukocyte, neutrophil and platelet count, as well as C-reactive protein were measured in all subjects, while fibrinogen, alpha(1)-antitrypsin, alpha(2)-macroglobulin, orosomucoid, transferrin and prealbumin were only measured in subjects with pheochromocytoma, primary aldosteronism and essential hypertension. RESULTS: Subjects with pheochromocytoma showed significantly higher leukocyte [7.5 +/- 0.9 10(9)/l, p < 0.001 vs. primary aldosteronism (5.4 +/- 0.9 10(9)/l) and healthy controls (5 +/- 0.9 10(9)/l), p = 0.04 vs. essential hypertension (6.3 +/- 1.6 10(9)/l)], neutrophil (p < 0.001 vs. primary aldosteronism and healthy subjects) and platelet counts (p < 0.001 vs. primary aldosteronism; p = 0.01 vs. essential hypertension) compared to the other groups of subjects. Similar results were obtained for positive proteins of acute-phase response in subjects with pheochromocytoma [C-reactive protein: 0.62 +/- 0.52 mg/dl, p < 0.001 vs. healthy subjects (0.08 +/- 0.08 mg/dl), p = 0.001 vs. primary aldosteronism (0.17 +/- 0.19 mg/dl), p = 0.04 vs. essential hypertension (0.31 +/- 0.26 mg/dl); fibrinogen: p = 0.02 vs. primary aldosteronism; orosomucoid: p = 0.005 vs. primary aldosteronism; alpha(2)-macroglobulin: p = 0.009 vs. primary aldosteronism]. No significant differences were found in plasma levels of alpha(1)-antitrypsin, transferrin and prealbumin. Tumor removal led to a significant decrease in leukocyte (p = 0.004), neutrophil (p = 0.007) and platelet count (p = 0.003) and also to a significant decrease in acute-phase proteins (C-reactive protein: p = 0.03, fibrinogen: p = 0.008, alpha(1)-antitrypsin: p = 0.003, orosomucoid: p = 0.04). CONCLUSIONS: Chronic catecholamine excess in pheochromocytoma is accompanied by an increase in inflammation markers which was reversed by the tumor removal.

• MeSH
• alfa-1-antitrypsin krev   MeSH
• alfa-makroglobuliny analýza   MeSH
• biologické markery krev   MeSH
• C-reaktivní protein analýza   MeSH
• dospělí MeSH
• feochromocytom krev   komplikace   patofyziologie   MeSH
• fibrinogen analýza   MeSH
• hyperaldosteronismus krev   komplikace   patofyziologie   MeSH
• hypertenze krev   etiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory nadledvin krev   komplikace   patofyziologie   MeSH
• neutrofily MeSH
• orosomukoid analýza   MeSH
• počet leukocytů MeSH
• počet trombocytů MeSH
• prealbumin analýza   MeSH
• transferin analýza   MeSH
• zánět krev   komplikace   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Research Support, N.I.H., Intramural MeSH
• Názvy látek
• alfa-1-antitrypsin MeSH
• alfa-makroglobuliny MeSH
• biologické markery MeSH
• C-reaktivní protein MeSH
• fibrinogen MeSH
• orosomukoid MeSH
• prealbumin MeSH
• transferin MeSH

Pheochromacytoma is a relatively rare cause of arterial hypertension. Untreated pheochromacytoma may however lead to a fatal hypertensive crisis during anaesthesia or another form of stress. It is therefore important to correctly diagnose this disease. 24-hour monitoring of blood pressure (BP) can already contribute to the diagnosis of pheochromacytoma based on the frequent occurrence of BP variability and the absence of a night-time fall in BP. 5 gene mutations have so far been identified that may be responsible for the familial form of pheochromacytoma: mutation of the von Hippel-Lindau (VHL) gene, leading to the onset of VHL syndrome, mutation of the RET-proto-oncogene in multiple endocrine adenomatosis type 2, mutation of the type 1 gene for neurofibromatosis, which is associated with von Recklinghausen's disease and finally mutation of the genes encoding the B and D subunits of succinated hydrogenase (SDHB, SDHD), which are associated with familial paragangliomas and pheochromacytoma. Genetic analysis should therefore be carried out for all confirmed cases of pheochromacytoma, especially for young people under 50 years of age. Biochemical diagnostics relies mainly on measurements of free metanephrines in plasma or urine, which usually has greater diagnostic weight than plasma, or catecholamines in urine. The diagnosis of extraadrenal or multiple forms can use not only CT/MR but also imaging using the radiopharmaceutical 123I-Metaiodobenzylguanidine (MIBG) or 18F-fluorodopamine PET (only available in the USA). Pharmacological treatment using alpha or beta receptor blockers with subsequent laparoscopic excision of the tumor is usually successful in benign forms of pheochromocytoma. Unfortunately, there are still no convincingly effective therapeutic procedures available for malign forms.

• MeSH
• feochromocytom komplikace   diagnóza   genetika   terapie   MeSH
• hypertenze etiologie   MeSH
• lidé MeSH
• mnohočetná endokrinní neoplazie typ 2A diagnóza   MeSH
• nádory nadledvin komplikace   diagnóza   genetika   terapie   MeSH
• neurofibromatóza 1 diagnóza   MeSH
• paragangliom diagnóza   MeSH
• protoonkogen Mas MeSH
• von Hippelova-Lindauova nemoc diagnóza   etiologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

The aim of our study was to evaluate potential differences in the concentration of biochemical markers of endothelial dysfunction between essential hypertension, endocrine hypertension (pheochromocytoma, primary hyperaldosteronism) and control healthy group and to assess a potential relationship between these markers of endothelial dysfunction and vasopressor substances overproduced in endocrine hypertension. We have investigated 21 patients with moderate essential hypertension, 29 patients with primary hyperaldosteronism, 24 subjects with pheochromocytoma and 26 healthy volunteers. Following parameters of endothelial dysfunction were measured, von Willebrand factor (vWf), plasminogen activator (t-PA) and E-selectin (E-sel). Clinical blood pressure was measured according to the European Society of Hypertension recommendations. We found significantly higher levels of the von Willebrand factor in patients with essential hypertension in comparison with a control group (114+/-20 IU/dl vs 90+/-47 IU/dl; P=0.04) and patients with primary hyperaldosteronism (114+/-20 IU/dl vs 99+/-11 IU/dl; P=0.01). Patients with endocrine hypertension revealed increased levels of vWF compared to the control group, but these differences did not reach statistical significance. Levels of t-PA were increased in patients with pheochromocytoma in comparison with the control group (4.6+/-1.9 ng/ml vs 3.4+/-0.9 ng/ml; P=0.01) and with primary hyperaldosteronism (4.6+/-1.9 ng/ml vs 3.4+/-1.1 ng/ml; P<0.01). In case of E-selectin we found lower levels in patients with pheochromocytoma in comparison with other groups, but they differed significantly only with primary hyperaldosteronism (40.2+/-15.0 ng/ml vs 51.3+/-23.0 ng/ml; P=0.05). Our study did not reveal any convincing evidence of differences in the levels of biochemical markers of endothelial dysfunction between essential and endocrine hypertension. No correlation between the biochemical markers of endothelial dysfunction and vasopressor substances activated in endocrine hypertension was found.

• MeSH
• biologické markery krev   MeSH
• cévní endotel patofyziologie   MeSH
• E-selektin krev   MeSH
• feochromocytom komplikace   MeSH
• hyperaldosteronismus komplikace   MeSH
• hypertenze krev   etiologie   patofyziologie   MeSH
• krevní tlak MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory nadledvin komplikace   MeSH
• tkáňový aktivátor plazminogenu krev   MeSH
• von Willebrandův faktor metabolismus   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH
• Názvy látek
• biologické markery MeSH
• E-selektin MeSH
• tkáňový aktivátor plazminogenu MeSH
• von Willebrandův faktor MeSH

Endocrine (adrenal) hypertension (primary aldosteronism, hypercortisolism and pheochromocytoma) had been considered for a long time as a rare cause of arterial hypertension. However, recent studies have shown a frequent incidence of primary aldosteronism among hypertensive subjects and therefore it is necessary to exclude primary aldosteronism in all subjects with severe hypertension. On the contrary, pheochromocytoma and hypercortisolism may be often overlooked in patients with incidentally found adrenal tumor and thus they can cause unexpected per- and postoperative complications.

• MeSH
• adrenokortikální hyperfunkce komplikace   MeSH
• feochromocytom komplikace   MeSH
• hyperaldosteronismus komplikace   MeSH
• hypertenze etiologie   MeSH
• lidé MeSH
• nádory nadledvin komplikace   MeSH
• nemoci nadledvin komplikace   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH

The authors present a case of a 37 year old male (proband) with a 13 year history of progressive sight impairment leading to blindness and a 4 year history of a mild hypertension. He was incidentally found to have large adrenal tumors after an ultrasound kidney examination. The tumors were confirmed with CT scan and magnetic resonance imaging. A bilateral pheochromocytoma was biochemically demonstrated and successfully removed. The eye diagnosis of angiomatosis retinae von Hippel-Lindau was ascertained after a search of the patient files in other medical departments, which led to a family screening. Proband's brother, having hypertension and a history of a cerebellar astrocytoma operation, was also diagnosed with CT scan to have a bilateral pheochromocytoma. Unfortunately, at the same time he was found to have a large irremovable neuroendocrine pancreatic carcinoma, which caused complications and his eventual death. Both proband and his brother were affected by the same CGG(Arg167)->CAG(Gln) mutation in the exon 3 of the VHL gene. Other living and examined family members were not affected, which was confirmed by negative genetic testing. One year after the pheochromocytoma operation, proband was diagnosed to have a retroperitoneal tumor left to the aorta, clinically silent, with slightly and non-constantly elevated urine norepinephrine and normetanephrine. Metaiodobenzylguanidine scintigraphy showed that it was a paraganglioma. The old CT and magnetic resonance picture review demonstrated that the tumor had already been present at the time of the operation. It was surgically removed and histologically verified. It is a pity that proband had not been sent by his ophthalmologist for an endocrine examination when the eye diagnosis was determined. Affection of the family would have been discovered earlier, and proband's brother might have possibly been saved.

• MeSH
• dospělí MeSH
• feochromocytom komplikace   genetika   MeSH
• lidé MeSH
• nádory nadledvin komplikace   genetika   MeSH
• von Hippelova-Lindauova nemoc komplikace   genetika   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH