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

Renal involvement in the tuberous sclerosis complex includes angiomyolipomas, cysts, and carcinomas. Angiomyolipomas, despite a sometimes frightening histopathologic appearance, are benign. Cystic disease, apparently resulting from tubular epithelial hyperplasia, causes hypertension and renal insufficiency, progressing to end-stage renal disease. The same epithelial hyperplasia predisposes to renal carcinoma.
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PMID:The renal lesions of tuberous sclerosis. 361 20

Tuberous sclerosis (TS) represents a relatively frequent inherited disorder of the skin and neurological tissues. Defects of other organs may also be present, but subjects differ significantly in their individual involvement. Usually, white leaf - shaped macules, even though most subtle, are the first precocious sign of the disease in young patients. Other signs tend to appear when the patient grows older. Visceral disorders include renal angiomyolipomata; clinical behavior of these solid tumors is almost always benign. Sometimes the renal lesions present themselves as polycystic kidneys, and may be the earliest sign and the only manifestation of TS, such as the case here described. This unusual form of renal involvement may be a severe potential complication of TS for the possible blood hypertension, recurrent urinary sepsis and chronic renal failure. It is of the utmost importance to search for the classical stigmata of TS in any patient who has cystic renal enlargement as only apparent abnormality. Careful inquiry into the family history cannot be overemphasized. The authors believe that, failing availability of adequate therapy for TS, the role in genetic counseling is to provide as much informations as possible to enable the involved family to make an intelligent decision about future children.
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PMID:[Clinical aspects (especially nephropathologic) and genetic counseling in tuberous sclerosis. Presentation of a case with polycystic kidney]. 367 Nov 35

In this paper we give a summarizing review of the pathology of apoplexy. Apoplexy is the clinical term for sudden loss of consciousness, followed by paralysis resulting from a cerebral haemorrhage, occlusion of a cerebral artery, thrombosis, or embolism, with loss of cerebral function of the affected brain region. Apoplexy is a very important disease in gerontology and geriatric practice and clinics, since atherosclerosis with cerebral sclerosis, hypertension, and hypertensive angiopathy occur very frequently in the elderly. Besides these main causes of apoplexy aneurysm and other causes of apoplexy are also discussed (i.e. angiomas, primary and secondary "apoplectiform" brain tumors). In this paper only the most important morphological and clinical findings in apoplexy are discussed for general and clinical physicians, giving an introduction to the following contributions in this journal.
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PMID:[Pathology of stroke (= apoplexy)]. 405 3

Two cases of pheochromocytomas in multiple locations are described. In the first case, a 5-year-old girl had surgery for a pheochromocytoma that was nonsecreting but had obstructed the renal artery. Ten years later, hypertension recurred and 2 adrenal pheochromocytomas, one on the left and one on the right side, were discovered. In addition, a papillary angioma was present in the fundus of the right eye, which indicated a phacomatosis. The child was cured. In the second case, another 5-year-old girl had a secreting pheochromocytoma in the upper thoracic region. Six months after extirpation of the tumour, a second pheochromocytoma was found in the right adrenal gland. These findings indicate 4 points of interest: 1. The thoracic localization. This prompted a review of all the available literature with the conclusion that this form of tumour is extremely rare. 2. The association of a pheochromocytoma and renal arterial stenosis. This has been observed by a number of other authors as well. 3. The coincidence of a pheochromocytoma as part of a phacomatosis and pheochromocytomas associated with Sipple's syndrome. 4. The multiple tumour sites. The necessity of generalized examinations in patients with pheochromocytomas to determine the presence of a phacomatosis or Sipple's syndrome is apparent.
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PMID:Rare forms of pheochromocytoma in children. 613 68

Among neurocutaneous disorders, coarctation of the abdominal aorta and renal artery stenosis have traditionally been associated with neurofibromatosis. We report a 5-year-old girl who, during the evaluation of asymptomatic hypertension, was discovered to have bilateral renal artery stenosis, coarctation of the abdominal aorta, renal cysts and typical skin lesions of tuberous sclerosis. Renal vascular hypertension has not been reported previously in tuberous sclerosis. We conclude that the tuberous sclerosis complex should be expanded to include vascular malformations and that hypertension should not be assumed to be secondary to renal hamartomata or cysts in patients with tuberous sclerosis.
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PMID:Coarctation of the aorta and renal artery stenosis in tuberous sclerosis. 647 22

Two half-brothers with tuberous sclerosis (TS) presented with polycystic kidneys in early childhood, before the classical stigmata became apparent. Their father shows no evidence of the disease. The older boys subsequently developed adenoma sebaceum at nine years and the younger boy developed infantile spasms. Hypertension occurred in both cases but neither showed evidence of renal failure. Extensive renal cyst formation in TS is rare, but when it does occur it differs from both infantile and adult-type polycystic disease. TS should be considered in the differential diagnosis of renal enlargement, haematuria and hypertension in childhood.
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PMID:Two half-siblings with tuberous sclerosis, polycystic kidneys and hypertension. 685 88

We report on a case of tuberous sclerosis in a 36-year-old female with chronic renal failure, who was treated by hemodialysis and transplantation. The diagnosis of tuberous sclerosis was ascertained by the association of typical cutaneous retinal and osseous lesions. On the contrary, the renal localisations were unusual. Early hypertension was exacerbated in the course of 2 pregnancies but was never severe. It preceded the late onset of renal failure which progressed slowly. The kidneys were small, with a peculiar angiographic aspect: the renal tissue was not invaded by hamartomas but the whole arterial bed was diffusely modified, with a cortical perfusion defect. Both kidneys were studied after bilateral nephrectomy. The lesions were far more complex than a simple replacement of the renal tissue by angiomyolipomas: they involved the vessels, the interstitium (with lipids inclusions surrounded by a macrophagic reaction) and the glomeruli (with focal and segmental sclerosis). This observation (which is the 11th case in the literature of tuberous sclerosis with chronic renal failure and the 3rd treated by transplantation) documents a particular variety of congenital and familial nephropathy with delayed revelation.
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PMID:[Tuberous sclerosis with chronic renal failure treated by hemodialysis and transplantation (author's transl)]. 702 34

We report the ultrastructure of bilateral renal angiomyolipomas in a case of tuberous sclerosis. The patient also had adult-type polycystic renal disease (Potter type 3) with systemic hypertension. Smooth muscle differentiation was supported by immunofluorescence localization of contractile protein using both smooth muscle and platelet-specific antibodies against myosin. There were cells with ultrastructural features intermediate between mature smooth muscle and fat suggesting origin from progenitor pericytic cells closely related to vascular endothelium. There was also evidence of secretory or synthetic capacity by some mesenchymal cells raising the possibility that hypertension in part may have been hormonally mediated by tumor. A unifying theory of histogenesis is proposed with special reference to the study of Wassermann regarding embryonal lipogenesis.
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PMID:Angiomyolipomas and polycystic renal disease in tuberous sclerosis. Ultrastructural observations. 724 58

A characteristic cystic renal lesion is described in three infants with tuberous sclerosis. Large cystic kidneys were present at birth in one child. The other two patients were discovered to have cystic kidneys and hypertension at 3 months of age. Development delay and seizures were ultimately present in two infants; typical cutaneous lesions of tuberous sclerosis developed in only one child. The roentgenographic appearance of the kidneys in the two older children was similar to that of adult-type polycystic kidney disease. Cysts were lined by a distinctive, hyperplastic epithelium in each patient. The diagnosis of tuberous sclerosis may be determined by renal biopsy in infants presenting with cystic kidneys.
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PMID:The cystic renal lesion in tuberous sclerosis. 742 Feb 20

There are two groups of diabetics under report, group I comprising 70 cases of retinopathia simplex, group II 100 cases of retinopathia proliferans in the centre. Retinopathy, showing as first sign of angiopathy, offers a comparatively good prognosis for the overall case of diabetes since, normally, neither retinopathia proliferans nor glomerulosclerosis are apt to develop. The same, in a higher degree, applies to cases of simple retinopathy fully devoloped and verified through ten years, at least: Retinopathy then shows stationary, in fact regressive development, the attending nephropathy generally expressing itself by the benign form of pyelonephritis and arteriolosclerosis renum rather than by glomerulosclerosis. All this in sharp contrast to proliferating retinopathy. Coronary sclerosis, peripheral and cerebral sclerosis and, to a limited extent, arterial hypertension stand independent of that; they attract attention by noteworthy independence.
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PMID:[The type of the diabetic retinopathy and the clinical picture of diabetes mellitus (author's transl)]. 744 11


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