Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a boy with neurofibromatosis type 1 (NF-1), hypertension, septic infection of an aneurysm in the deltoid muscle, bowel infarction, multiple arterial aneurysms and venous thrombosis occurred within a period of 6 weeks. Histologically, vascular neurofibromatosis of the small vessels of the gut was found. This unusual occurrence of a multitude of clinical features within a few weeks was caused by vascular neurofibromatosis. Awareness of this condition in patients with NF-1 may help the paediatrician to avoid unnecessary diagnostic procedures and to initiate appropriate symptomatic therapy.
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PMID:Neurofibromatosis presenting as a severe systemic vasculopathy. 815 15

A study of headache in a homogeneously ascertained population of 181 subjects suffering from neurofibromatosis type 1 is described. All subjects underwent a diagnostic protocol including imaging studies (for subjects over 5 years old up until 1992). Headache data were collected by means of a questionnaire. Headache was present in 55 of 181 subjects (25 males). Overall headache frequency was 30%, which is not significantly different from the frequency of headache reported in the general population. Headache was primary in 52 cases (5 migraine and 47 tension-type) and secondary to obstructive hydrocephalus with brain tumor-induced intracranial hypertension in 3 with a tension-type pattern. It was concluded that headache is not a specific feature of neurofibromatosis type 1, it is not significantly related to central nervous system abnormalities, and in itself, it is not an indication for neuroradiological examination.
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PMID:Headache in patients with neurofibromatosis type 1. 866 29

A 48-year-old man with neurofibromatosis type 1 presented with chest pain, paroxysmal hypertension, tachycardia, and progressive respiratory insufficiency. Clinical investigation displayed calcified tumors in the anterior mediastinum and pararenal region. Histological examination at autopsy revealed composite tumors consisting of pheochromocytoma and malignant peripheral nerve sheath tumor (MPNST) at two sites: the left adrenal gland and the region surrounding the inferior vena cava, probably corresponding to the right adrenal gland. The MPNST component showed a varied histological appearance, including hyalinized bands with polygonal cells, a cartilaginous and myxoid stroma, a hemangiopericytomatous architecture, and a fibrosarcomatous structure, which suggested osteosarcoma, chondrosarcoma, angiosarcoma, and fibrosarcoma, respectively. In addition, based on the ultrastructural findings, the gastrointestinal tract was involved with mesenchymal tumors showing neurogenic differentiation. These lesions suggest the divergent cellular differentiation of neural crest-derived cells to mesenchymal elements as well as neuroectodermal neoplasms.
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PMID:A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors. 866 38

Neurofibromatosis type 1 is the most common autosomal dominant inheritable disease, which is often associated with secondary forms of hypertension and with tumors of neuroectodermal origin. The authors present the results of evaluation of 60 members of 3 families. Of the 60 family members, 13 subjects had symptoms of neurofibromatosis type 1 disease, of which 7 subjects were evaluated. The case histories of patients are discussed: (1) An incidentally discovered adrenal tumor was proved to be a pheochromocytoma. (2) Because of complaints similar to thyrotoxicosis, thyrostatic drugs were administered for years without effect and, finally, an adrenal phaeochromocytoma was diagnosed after the presence of neurofibromatosis was established. (3) Preeclamptic pregnancy of a young primigravida complicated with severe HELLP syndrome (hemolysis-elevated liver enzymes-low platelet count) led to thorough evaluation which revealed renal artery stenosis. In this patient, percutaneous renal artery angioplasty resulted in a complete cessation of hypertension. (4) Glucocortocoid replacement therapy in a patient with neurofibromatosis type 1 resulted in a complete normalization of both secondary adrenal insufficiency and a previously unexplained iron-refractor iron-deficient anemia. The case histories of the patients demonstrate a lack of in-depth knowledge of neurofibromatosis in clinical practice. A regular follow-up of neurofibromatosis patients is suggested in specialized health centers.
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PMID:[Endocrinologic complications of neurofibromatosis type 1]. 899 16

A 4 year old girl with neurofibromatosis type 1 (NF1) was referred for hypertension. An aortogram showed narrowing of the left main renal artery. An angiogram three and a half years later showed coarctation of the abdominal aorta. She underwent aortoplasty but the stenosis recurred. Vascular involvement in NF1 may be progressive and requires long term follow up.
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PMID:Hypertension secondary to progressive vascular neurofibromatosis. 919 66

Pheochromocytomas are catecholamine-producing tumors, representing one of the most important causes of secondary hypertension. The classification of these tumors considers both sporadic and familial forms, intra- and extraadrenal localization as well as the dignity. Familial pheochromocytomas are primarily seen under the conditions of multiple endocrine neoplasia, von Hippel-Lindau disease or neurofibromatosis type 1. The list of clinical symptoms includes hypertension, which can be both continuous or intermittent, headache, tachycardia and sweating. It is most important to standardize the pre-analytical procedures, i.e. control for sampling conditions and adequate choice of parameters in plasma or urine. For screening sensitive methods will be employed (free catecholamines in 24h-urine) and for confirmation of the diagnosis, specific procedures are performed (Clonidine test, MIBG-scintigraphy). The endocrinological and biochemical procedures are completed by molecular genetic techniques in familial pheochromocytoma.
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PMID:[Clinical and endocrine diagnosis of pheochromocytoma]. 933 11

The authors detected a case of neurofibromatosis type 1 complicated with preeclampsia and the syndrome of hemolysis, elevated liver enzymes, and low platelets, which developed during pregnancy. When the cause of the patient's hypertension was investigated after birth, renal stenosis of the right artery was detected, which was then successfully treated with percutaneous transluminal angioplasty.
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PMID:Neurofibromatosis type 1 with pregnancy-associated renovascular hypertension and the syndrome of hemolysis, elevated liver enzymes, and low platelets. 970 4

Neurofibromatosis Type 1 produces a broad spectrum of clinical manifestations as a result of widespread dysplasia of mesodermal and neuroectodermal tissues. One of the most serious aspects of the disease relates to the arterial involvement that may occur. We report a case of severe stenosis of the internal carotid artery and intracerebral hemorrhage associated with neurofibromatosis Type 1. A 49-year-old female was admitted to our hospital after she had suddenly become comatose. On admission, she demonstrated a decerebrate posture in response to painful stimuli, and was assessed as grade 200 according to the Japan Coma Scale. Physical examination disclosed widespread cutaneous neurofibromas and cafe-au-lait spots. Computed tomography of the head revealed a right putaminal extensive hematoma, with a maximum diameter of 7 cm. The hematoma was removed. After this surgical treatment, cerebral angiography was performed. It showed severe stenosis of the terminal portion of the right internal carotid artery associated with a fine telangiectatic network, indicating the presence of moyamoya vessels in the basal ganglia. Although intracranial hemorrhage associated with neurofibromatosis type 1 is a rare condition, fine telangiectatic collateral vessels caused by occlusive cerebrovascular disease, intracranial aneurysms, brain tumors, or hypertension caused by pheochromocytoma or stenosis of the renal artery should be considered as the cause of hemorrhage.
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PMID:[Severe stenosis of the internal carotid artery and intracerebral hematoma associated with neurofibromatosis type 1: a case report]. 1002 86

Neurofibromatosis type 1 is a phacomatosis. Neurofibromas are the most common tumours associated with the disease, and along with other tumours, make neurofibromatosis type 1 the most common tumour predisposing syndrome in humans. Hypertension may be coincidental, but at least two specific neurofibromatosis related causes must be considered, namely neurofibromatous involvement of the renal artery and pheochromocytoma. We have described the first known case of a patient with neurofibromatosis type 1 who developed hypertension due to an aldosterone-producing adenoma of the adrenal. The question of whether this association was coincidental or due to the tumour predisposition of neurofibromatosis type 1 was debated.
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PMID:A case of neurofibromatosis type 1 with an aldosterone-producing adenoma of the adrenal. 1058 21

The neurofibromatoses are autosomal dominant diseases that have widespread effects on ectodermal and mesodermal tissue. The commonest member of the group is neurofibromatosis type 1 (NF1) which varies in severity but which can affect all physiological systems. Neurofibromas are the characteristic lesions of the condition and not only occur in the neuraxis but may also be found in the oropharnyx and larynx; these may produce difficulties with laryngoscopy and tracheal intubation. Pulmonary pathology includes pulmonary fibrosis and cystic lung disease. The cardiovascular manifestations of NF1 include hypertension, which may be associated with phaeochromocytoma or renal artery stenosis. Neurofibromas may also affect the gastrointestinal tract and carcinoid tumours may be found in the duodenum. This review documents the aetiology and clinical manifestations of the neurofibromatoses and discusses their relevance to the anaesthetist.
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PMID:Neurofibromatosis: clinical presentations and anaesthetic implications. 1157 32


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