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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 12-year-old boy with neurofibromatosis, kyphoscoliosis, and hypertension is reported. The association of neuroflibromatosis and kryphoscoliosis is well known. There are no reports on the association of neurofibromatosis and hypertension in the orthopaedic literature. The cause was found to be intrarenal vascular abnormalities which are characteristic of neurofibromatosis. Other possible causes of hypertension in this disease are discussed. This patient was successfully treated by a partial nephrectomy.
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PMID:Neurofibromatosis and hypertension. A case report. 11 95

38 cases of severe hypertension due to a vascular abnormality of the renal pedicle were studied in children under 16 years of age, 18 boys and 20 girls. The most common presentation was at routine clinical examination. The diagnosis of an abnormality of the renal artery was suggested by the appearances of intravenous urography. There were many causes; 4 aneurysms of the renal artery or its branches, 4 fibromuscular dysplasias with one case of bilateral fibromuscular dysplasia, 4 idiopathic stenoses, 2 endarteritis, and 6 thromboses revascularised to variable degrees (2 after umbilical vein catheterisation and one due to DLE). In three cases the hypertension was related to compression of the pedicle by a tumour of haematome, and 14 cases had multiple arterial lesion. In the latter group, 6 cases of neurofibromatosis, 2 cases of William and Beuren's disease, 1 case of generalised Elastorhexia, 2 cases of aortic medio stenosis, probably Takayashu's disease, and 3 unidentified conditions. Surgery was performed on 29 patients, 21 of whom had unilateral lesions and were definitively cured of hypertension. Of the 8 cases with multiple lesions, only 2 were completely corrected with cure of their hypertension.
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PMID:[Arterial hypertension caused by anomaly of the renal artery or its branches in children]. 11 10

Vascular involvement in von Recklinghausen's neurofibromatosis has been known since the work of Reubi (1944, 1946). We found 72 cases of neurofibromatosis with renovascular hypertension reported in the literature. We have observed three female patients, of whom two had unilateral renal artery abnormalities consisting of stensoses and small aneurysms. The third patient, who had a right-sided nephrectomy for probable occlusion of the renal artery, had a coarctation of the abdominal aorta and complete occlusion of the left renal artery and of the coeliac-mesenteric trunk. There were, in addition, large, bilateral aneurysms in the circle of Willis.
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PMID:[Renovascular hypertension in neurofibromatosis. Simultaneous occurrence of multiple abdominal and cerebral vascular abnormalities (author's transl)]. 13 38

Neurofibromas and phaeochromocytomas derive ultimately from ectodermal tissue. It is therefore not surprising that there is an increase in the incidence of phaeochromocytomas in Recklinghausen's neurofibromatosis. When investigating hypertension in patients with neurofibromatosis one must therefore consider the possibility of a phaeochromocytoma as well as vascular involvement by neurofibromatosis. Two patients with neurofibromatosis and phaeochromocytomas are described.
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PMID:[Phaeochromocytomas in neurofibromatosis (author's transl)]. 14 23

Vascular lesions due to neurofibromatosis have been predominantly reported in the renal arteries as cause of arterial hypertension, but they can occur everywhere. The angiography shows a smoothly bordered stenosis at the origin of the artery with an elongated funnel shaped poststenotic deformity. Collateral vessels are often present. Reconstructive arterial surgery is in a high percentage successful. The main differential diagnosis includes fibromuscular dysplasia. Arterial hypertension in a young person with neurofibromatosis should suggest the presence of pheochromocytoma or renovascular disease.
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PMID:[Multiple vascular stenoses in neurofibromatosis (author's transl)]. 40 67

Angiographic studies in a young girl aged 16 years who developed arterial hypertension during the course of Von Recklinghausen's disease, demonstrated the presence of an obliterated renal artery in a horseshoe kidney. The distal arterial segment was revascularized by two homolateral ureteral arteries. A full review of the literature revealed 30 cases of renal artery lesions for which angiography had been carried out in young people with neurofibromatosis. This demonstrates the low incidence of obliterative lesions (4 cases throughout the world) which are always associated with collateral vascularization. It was not possible to apply the usual surgical treatment in our case because of the associated horseshoe kidney.
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PMID:[Arterial hypertension and horseshoe kidney during Von Recklinghausen's disease. Angiographic study and review of the literature (author's transl)]. 41 77

The course of the illness in a six-year-old boy is reported. His recurrent headaches led to the detection of arterial hypertension (170/135 mmHg). Clinical and histological examination revealed neurofibromatosis von Recklinghausen. A bilateral paraumbilical murmur hinted at a renovascular form. Arteriography showed an aneurysm of the right renal artery and multiple bilateral intrarenal stenoses and aneurysmatic dilatations. Under conservative treatment with Propranolol and Dihydralazine blood pressure remained almost normal over two years. 38 paediatric cases of renovascular hypertension in childhood reported in the literature are analyzed with regard to clinical manifestation, morphology and localisation of the renovascular lesions.
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PMID:[Renovascular hypertension in neurofibromatosis von Recklinghausen (author's transl)]. 41 39

A casual relationship between von Recklinghausen's disease, or neurofibromatosis, and arteriolar abnormalities has been reported in the European literature. A patient was seen who had biopsy-proved neurofibromatosis and renovascular hypertension and retroperitoneal bleeding. An arteriographic study showed multiple small aneurysms throughout the coeliac axis, the superior mesenteric artery and in several small intrarenal vessels. Renal vein renin levels were elevated particularly in the right renal vein, supporting the diagnosis of renovascular hypertension. Both the aneurysms seen in angiographic studies and the retroperitoneal hemorrhage are probably vascular manifestations of von Recklinghausen's disease. Support for this conclusion is enhanced by the absence of clinical, laboratory or histologic data supporting the only tenable differential diagnosis, periarteritis nodosa.
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PMID:Vascular manifestations of von Recklinghausen's disease. 80 43

A child with neurofibromatosis and hypertension also demonstrated renal artery stenosis, the most common cause of hypertension in children with neurofibromatosis; abdominal coarctation, which has previously been described; and thoracic coarctation, which to our knowledge, has not been previously reported. Rib notching may appear in patients with uncomplicated nueorfibromatosis, but the possibility of associated thoracic coarctation must also be considered in these patients.
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PMID:Thoracic coarctation associated with neurofibromatosis. 80 24

A case of hypertension in a patient with neurofibromatosis and renal artery stenosis with aneurysm formation in a solitary kidney is described. Studies of plasma renin activity and body sodium content are presented and have been related to findings in patients and experimental animals with renovascular hypertension. A reconstructive operative procedure is described in which the kidney is perfused in situ.
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PMID:Hypertension and renal artery stenosis with aneurysm formation in a solitary kidney in a patient with neurofibromatosis. 80 81


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