Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085580 (essential hypertension)
 14,686 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hypertension in neurofibromatosis is mostly a consequence of a stenosis of the renal artery or is due to phaeochromocytoma. Riccardi pointed out primary hypertension in patients with several cervical neurofibromas in the absence of phaeochromocytoma and he noticed that the elevation of BP was often already present in children. Nine (15.8%) of 57 neurofibromatosis patients (age from 1.5 to 23 years) examined, presented BP levels above the 95th percentile on several occasions and three in particular had severe hypertension with compromised target organs. Two of them had a stenosis of the renal artery, in the third an organic origin of hypertension was not demonstrated, but there was an asymptomatic glioma of the hypothalamus. The other six children had a labile or borderline hypertension and two of them had, respectively, a glioma of the thalamus and of the optical chiasm. Elevation of the catecholamine metabolites or other causes of hypertension were not found in any of these patients. These preliminary data show a high incidence of hypertension in neurofibromatosis, primary or due to organic causes and overall they point out a possible correlation between hypertension and cerebral neoplasia.
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PMID:Hypertension in children with neurofibromatosis. 806 89

Sporadic pheochromocytoma is a rare tumor that should be taken into account in patients with hypertensive crisis, arrhythmias, and panic disorder. Familial pheochromocytoma is frequently found in subjects with von Hippel-Lindau disease, multiple endocrine neoplasia type II, neurofibromatosis, and SDHD gene mutations. The prevalence of sporadic pheochromocytoma is very low, approximately 0.05% among subjects with essential hypertension and even less in the general population. However, aggressive diagnostic intervention is recommended whenever a pheochromocytoma is suspected because the uncontrolled catecholamine release from the tumor can lead to serious and potentially lethal complications. Plasma free metanephrines have been shown to have high sensitivity and specificity in the biochemical diagnosis of sporadic and familial pheochromocytoma. Measurement of 24-hour urinary fractionated metanephrines may be an acceptable alternative in many patients. The current approach to the diagnostic localization of pheochromocytoma relies on computed tomography (CT), magnetic resonance imaging (MRI) and [123-I] and [131-I] MIBG scintigraphy. CT and MRI have very high sensitivity but low specificity, whereas MIBG scintigraphy has good specificity but its sensitivity is less than optimal, especially for the detection of metastases. In difficult cases, PET imaging appears to be promising.
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PMID:[The sympathetic system and neuroendocrine hypertension]. 1835 May