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

In a young man and a pregnant young woman presenting since childhood of tuberous sclerosis without epilepsy or mental disorders severe intracranial hypertension developped secondary to obstructive hydrocephalus. Ventriculo-peritoneal shunting unilateral in the first case, bilateral in the second, releaved the patients of all their symptoms. Angiographic studies in both cases showed the existence of extracerebral visceral lesions. After a short review of similar published cases and the proposed treatments the authors suggest as only treatment a shunting procedure without attempt of removal of intracerebral nodules.
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PMID:[Tumoral forms of tuberous sclerosis. The clinical and therapeutic importance of concomitant hydrocephalus (author's transl)]. 30 1

Pathological type complications associated with 46 cases of neurofibromatosis in children under 12 are reported. It is noted that in 65.2% of the cases there are mental retardation, usually serious. More than 50% (24 cases) had some type of tumoration. All were benign with the exception of a suprarenal neuroblastoma that caused arterial hypertension and histological characteristics of malignancy. Fifteen tumors were located in the optica ways, one in the mediastinum, one in the abdomen, one in the paravertebral area, one which was a craneal plexiform tumor and four of the moluscum pendulum type on the eyelids or in neighbouring regions. Twelve children suffered from some type of seizures (Salaam's spasms, tonic-clonic, myoclonic, atonic and versive). Radiological abnormalities were very frequent in the simple X rays as well as in those in which contrast medium was used. In four cases malformations of the midline were observed, three of which were non-communicating cysts of the septum pellucidum, the other agenesis of the corpus callosum. Neurofibromatosis was further seen associated iwth Bourneville's syndrome, Morquio's syndrome, Batten's type of lipofuscinosis, facial or generalized hemihypertrophia and stenosis of the aqueduct. Heredity was dominant autosomic in 16 cases, the rest being due to possible recent mutations.
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PMID:[Pathological complications in 46 cases of neurofibromatosis in children (author's transl)]. 82 74

The authors present a case of pheochromocytoma observed in a 49 year old man suffering from the cutaneous form of von Recklinghausen's disease. In this patient, they noted the constant absence of the inferior abdominal cutaneous reflex on the right side, contrasting with the briskness of the other abdominal cutaneous relexes. From this they concluded that the chromaffin tumor was very probably situated in the right adrenal region. This hypothesis was confirmed by retro-pneumoperitoneum, selective arteriography and finally surgical intervention. After having reviewed the essential medical data from the literature concerning the conditions associated with pheochromocytoma and especially phacomatosis, and having looked at the problem of hypertension related to neurofibromatosis, they make a critical study of the behaviour of the abdominal reflexes in case of pheochromocytoma and emphasize the part that it is possible to draw from this in view of its' topographic diagnosis. The present case is the fourth in which one of the authors has noted the abolition of the inferior abdominal reflex on the side corresponding to the site of the pheochromocytoma.
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PMID:[Pheochromocytoma and Recklinghausen's cutaneous neurofibromatosis. Localizing value of abdominal reflexes in a case]. 82 54

On the basis of a clinical case, the authors review the principal signs of Bourneville tuberose sclerosis and show the renal manifestations which may be encountered. The most common is the presence in both kidneys of tumours which are hamartomas. In addition, this patient suffered from hypertension and renal insufficiency. Their frequency and pathogenesis are discussed in the context of this disease.
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PMID:[A rare association of hamartomas of the kidney, renal insufficiency, Bourneville disease and arterial hypertension (author's transl)]. 85 54

Strokes may occur with a large number of genetic disorders. Natowicz and Kelley have reviewed the single gene disorders capable of causing familial haemorrhagic strokes. These may be classified into four groups: a) hereditary disorders affecting clotting factors or platelets; b) hereditary disease producing vascular anomaly; c) hereditary disease causing hypertension and d) miscellaneous group including neurofibromatosis, sickle cell disease and tuberous sclerosis.
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PMID:Familial intracranial haemorrhage due to factor V deficiency. 156 89

Angiomyolipoma is an uncommon benign tumor of the kidney. The tumor is composed of fat, smooth muscle, and blood vessels. The same pathological entity can appear in two clinically different forms, with or without tuberous sclerosis. We present two cases of renal angiomyolipomas with unusual manifestations. One was associated with tuberous sclerosis and both had the presentation of acute abdomen. Case one presented with epilepsy, angiofibroma, subungual fibroma, periventricular calcification, and bilateral renal angiomyolipomas. Tuberous sclerosis is characterized by these findings. Both cases had spontaneous hemorrhage with hypovolemic shock. Massive hemorrhage resulting in shock is uncommon and the incidence has been estimated to be about 10 per cent. In fact, many angiomyolipomas are clinically occult. The size of the tumor correlates well with the presence or absence of symptoms which include microhematuria, flank pain, hypertension and urinary tract infection. Abdominal CT is the preferred modality for diagnosis of angiomyolipoma. The most important finding is the presence of an intrarenal tumor with fat component which is recognized as a relative low density on CT. Our patients were hospitalized under the impression of angiomyolipoma after the CT study. In addition, the CT defined either the size of the tumor or the extension of the hemorrhage. Although many believe that renal angiography is not sufficient by itself to establish the diagnosis of angiomyolipoma, occasionally it is mandatory in the management of the tumor. The management is decided by two factors, the size of tumor and the clinical presentation. The attitude of management should include conservative treatment with regular follow-up, selective arterial embolization, enucleation, and partial or total nephrectomy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Unusual presentations of angiomyolipoma]. 179 71

The short- and long-term outcome and special problems of 24 children with infantile spasms and tuberous sclerosis (TS) was studied. The diagnosis of TS is frequently missed: white spots on the skin have to be carefully looked for. In the present study, these spots were always found, and calcifications or hypodense areas of the brain were revealed by CT scans before one year of age. In addition to epilepsy, manifestations of TS included: giant cell astrocytoma, iridic heterotopia, cardiac rhabdomyoma, brain cysts, polycystic kidneys with severe arterial hypertension, retinal phakomas, angiofibroma and white spots on the skin. Long-term outcome was poor. None of the children had normal intelligence and all but one had epilepsy, which was often intractable. Behavioural problems were common. The prognosis, in terms of later intelligence, epilepsy and behavioural problems, was worse than in those with 'idiopathic' infantile spasms or in those with simultaneous neurological disorders. An early diagnosis of TS in patients with infantile spasms clarifies the prognosis and avoids some of the hazards of ACTH therapy. TS children have a high relapse rate after ACTH therapy, so prolonged courses may be indicated.
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PMID:Tuberous sclerosis and infantile spasms. 215 40

The cause of hypertension in this patient with tuberous sclerosis appeared to be the result of volume expansion due to renal failure. Renal insufficiency was presumably caused by extensive replacement of renal parenchyma with angiomyolipomas, resulting in compression and distortion of the renal parenchyma. Noninvasive imaging techniques were the most useful for characterizing the extent of the disease. It is clearly important to monitor renal function and blood pressure of patients with tuberous sclerosis as they grow older.
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PMID:Hypertension and renal failure in a patient with tuberous sclerosis. 218 93

In the present study, the mean arterial pressures (MAP) and heart rates (HR) were measured in Wistar-Kyoto (WKY) and spontaneously hypertensive rats (SHR) that had received a 10-day continuous subcutaneous infusion of either saline or the centrally acting alpha-adrenoceptor agonist clonidine (10 micrograms/kg/h). In separate groups of similarly treated rats, the concentrations of neurotensin (NT), vasoactive intestinal polypeptide (VIP), cholecystokinin octapeptide (CCK-8), calcitonin gene-related peptide (CGRP), and neuropeptide Y (NPY) were measured in the cortex (COR), hypothalamus (HYP), medulla oblongata/pons (MO/P), and cervical (CSC) and thoracic (TSC) spinal cord. In comparison to the WKY rats, the SHR had significantly lower neuropeptide concentrations within the COR (NPY, CCK-8), HYP (NT), MO/P (NPY, NT, and CCK-8), CSC (all neuropeptides), and TSC (NPY, NT, CCK-8, and VIP). The infusion of clonidine lowered the MAP of the WKY and SHR rats (-10 and -35 mm Hg, as compared with respective saline controls) and HR in the WKY rats (-45 beats/min). In general, the infusion of clonidine produced decreases in neuropeptide levels within the CNS of the WKY rather than the SHR strain. When there was a strain difference (i.e., SHR less than WKY), it was evident, particularly within the spinal cord, that clonidine reduced the levels of the neuropeptides in the WKY rats to those levels in the SHR. These findings suggest that the reduced neuropeptide concentrations of the SHR (particularly those within the CSC) and TSC may be a result of, rather than a causal factor in, hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Regional brain concentrations of several putative peptide neurotransmitters in normotensive and spontaneously hypertensive rats: effects of continuous (10-day) clonidine infusion. 245 67

After reporting the general features of renal angiomyolipoma (AML), either isolated or associated to other conditions (Bourneville sclerosis, Wunderlich's syndrome), the Authors discuss a case complicated by perirenal spontaneous hematoma, undergone emergency operation (simple nephrectomy). At a 3 year follow-up the patient is in good general and local conditions (negative TC, normal renal function, preexistent hypertension disappeared). Literature is reviewed and some considerations about diagnosis and surgical tactics are reported.
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PMID:[Angiomyolipoma of the kidney: a case of spontaneous rupture]. 251 6


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