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

We describe a pulsatile aneurysm in the skin of 16-year-old boy that was found to be a sign of a systemic vascular disease, that is, arterial fibromuscular dysplasia. The patient had aneurysms in the renal, cerebral, coronary, and other arteries; he developed renovascular hypertension and had a cerebrovascular accident and acute myocardial infarction at 17 years of age. This disease has not been previously reported in the dermatologic literature.
 ...
PMID:Aneurysm in the skin: arterial fibromuscular dysplasia. 146 51

Histology records from 63 nephrectomies were reviewed; 22 patients had unilateral totally dysplastic kidneys and 5 had polar or segmental dysplasia. A clinicopathological study of these cases was undertaken. In the first group, there was a slight male preponderance and 75% of the patients presented were under two years of age. Urinary tract infection was the most common complaint. 4 patients were diagnosed in utero by ultrasound and 5 infants presented an abdominal mass. Hypertension was documented in a newborn baby. Ipsilateral lower urinary tract anomalies were found in 12 patients and those of the contralateral kidney in 2 children. There were 3 cases of extrarenal anomalies. Histological examination revealed 13 cases of multicystic dysplasia and 9 of solid dysplasia. Metaplastic cartilage was found in 1 case. In the group of segmental dysplasia, age ranging from 27 weeks' gestation to 8 years, at the time of the diagnosis. They all had duplex kidneys and 4 had ureterocele. Histological study in these cases was similar to the one found in the previous series, although superimposed inflammatory changes were more pronounced. Some of the theories regarding the pathogenesis of this disorder are reviewed and the importance of its diagnosis is emphasised.
 ...
PMID:[Renal dysplasia: clinico-pathologic review]. 150 40

The anatomical location and appearance of retinal hemorrhages in the infant provide important clues in the diagnosis of underlying disorders. While neonatal retinal hemorrhages related to birth trauma are common, benign, and self-limited, other retinal hemorrhages in infancy may signify intracranial aneurysms, accidental or non-accidental injury, and a variety of ocular (e.g., Coats' disease, PHPV, ROP, retinal dysplasia, hypertension, myopia) or systemic disease (e.g., hematologic or cardiovascular disorders, infection, protein C deficiency). In this review, retinal hemorrhages are illustrated and classified according to location, appearance, and etiology. Prompt diagnosis of retinal hemorrhages in infants is crucial, because treatment may be required to prevent early deprivation amblyopia and blindness. Ophthalmological findings may also be a valuable contribution to the overall medical evaluation of the infant.
 ...
PMID:Fundus hemorrhages in infancy. 150 54

Renal artery fibromuscular dysplasia is a well-known cause of hypertension whose presence is confirmed by its typical arteriographic appearance. The functional significance of these lesions is often difficult to determine, particularly when both renal arteries are involved. Duplex scanning has been shown to be accurate for the detection of renal artery stenosis and estimation of the degree of narrowing. To test whether duplex scanning results after intervention correlate with clinical outcome, we reviewed the studies on nine patients with renal artery fibromuscular dysplasia who had been treated by either angioplasty or surgery. A total of 18 arteries were treated. Fourteen of the 18 treatments were successful as measured by a reduction in blood pressure and antihypertensive drugs. In four instances treatment was unsuccessful. For those patients who had clinical improvement, the hemodynamic parameters from the renal artery also improved. In the patients for whom treatment failed, the velocities recorded from the site of narrowing did not improve after intervention. Thus it appears that duplex scanning along with the clinical results may be used to document the basis for failing to improve after treatment be it angioplasty or operation.
 ...
PMID:A preliminary study of the role of duplex scanning in defining the adequacy of treatment of patients with renal artery fibromuscular dysplasia. 153 41

Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had severe hypertension requiring multiple drug treatment. Diminished peripheral pulses were not helpful in the diagnosis, which is made on aortography. Associated clinical findings were Williams' syndrome (n = 3) and appreciable eosinophilia (n = 3). The differential diagnosis includes Takayasu's arteritis, fibromuscular dysplasia, and neurofibromatosis. Blood pressure was adequately controlled by medical treatment in six patients. Surgical angioplasty was performed in two. One patient remained normotensive without drug treatment 21 months after operation; the other died of sepsis and uncontrollable haemorrhage in the postoperative period. Medical treatment is satisfactory in most cases: surgery should be reserved for those in whom blood pressure cannot be controlled without unacceptable side effects of drug treatment. Although rare, the middle aortic syndrome should be considered in the differential diagnosis of hypertension when commoner causes have been excluded. Aortography is necessary for diagnosis.
 ...
PMID:Middle aortic syndrome: clinical and radiological findings. 158 Jun 80

Reflux nephropathy is characterized by the presence at radiological examination of one or several segmental scars in the renal parenchyma, associated with vesico-ureteral reflux. Histology displays a variety of lesions, among which chronic and atrophic pyelonephritis, segmental hypoplasia and renal dysplasia can be individualized. Most of these renal lesions can be prevented by early detection of the reflux which encourages the development and recurrence of urinary tract infection and its diffusion to the upper urinary tract and the kidneys. The finding of a vesico-ureteral reflux with renal lesions, even after spontaneous or surgical regression of this reflux, requires prolonged surveillance in view of the long-term risk of arterial hypertension and renal failure.
 ...
PMID:[Reflux nephropathy]. 160 64

Between November 1980 and July 1989, 29 ex vivo reconstructions were performed in 12 women and five men (mean age: 42 years) for dysplastic lesions of renal artery branches. Fifteen patients had hypertension, associated with advanced renal failure in one case. The internal iliac artery was used routinely for reconstruction. The saphenous vein was used on one side where bilateral repairs were necessary. A total of 52 distal anastomoses were performed, an average of 2.6 per revascularized kidney. Mean follow-up was four years and 10 months. No patients were lost to follow-up. There were no early or late deaths. At least one follow-up arteriogram and technetium99 diethylene triamine pentacetic acid scintiscan was obtained for all patients. Forty-five (86%) of 52 anastomoses remained patent. Eleven of 15 hypertensive patients completely recovered while four were improved. Systolic arterial blood pressure decreased by an average of 42 mmHg (p less than 0.001), and results remained stable with time. For fibromuscular dysplasia occurring in young patients whose life expectancy is usually long, this type of surgery provides excellent long-term clinical and anatomical results.
 ...
PMID:Dysplastic lesions of renal artery branches: late results of ex vivo repair. 161 Jun 53

From 1980 to 1990, 48 (4.7%) of 1,002 patients underwent elective aortic reconstruction and simultaneous renal artery reconstruction. Forty-five men and three women (mean age: 66.5 years) had 59 renal artery lesions (51 stenoses, six occlusions, one dysplasia, and one aneurysm) associated with 20 infrarenal aortic aneurysms and 28 aortoiliac occlusive lesions. One nephrectomy and 58 renal artery reconstructions were performed (35 prosthetic bypasses, 11 vein bypasses, six direct reimplantations, five transaortic endarterectomies, and one resection of an intrahilar aneurysm followed by autotransplantation). Operation was always indicated for the aortic lesions. Indication for renal artery repair was hypertension in 33 cases (17 associated with renal insufficiency) and one with isolated renal insufficiency. In the remaining 14 cases, surgery was deemed preventive. One patient died (2%). There were 12 nonfatal complications two of which were kidney failures requiring chronic extrarenal epuration. Routine follow-up arteriograms showed four postoperative renal artery occlusions. Mean follow-up was 35.8 months. Four patients were lost to follow-up; 10 died secondarily. Five year survival was 72.1 +/- 19.1%. Secondary patency of renal artery reconstruction was 89.5 +/- 9.4% at five years. Late results were favorable in 45% of patients with hypertension and in 39% of patients with renal insufficiency. Mortality in simultaneous aortic and renal artery reconstruction is not superior to that of isolated infrarenal aortic surgery.
 ...
PMID:Simultaneous reconstruction of infrarenal abdominal aorta and renal arteries. 161 Jun 54

We report a 29-year-old Japanese female with severe hypertension and unilateral renal dysplasia, which is rarely found in adults. The involved kidney was surgically removed and then the blood pressure fell to borderline levels without any changes in plasma renin activity or plasma aldosterone concentration. Histological examinations revealed typical renal dysplasia without hyperplasia of the juxtaglomerular apparatus. Unilateral renal dysplasia may be a cause of secondary hypertension in adults, though the mechanisms are still unknown.
 ...
PMID:Hypertension in an adult with unilateral renal dysplasia. 163 64

We experienced 41 cases of Cushing's syndrome (12 males and 29 females, 15 years old - 65 years old) during the last 20 years. These included 20 patients with unilateral adrenal adenoma (Cushing's syndrome), 19 patients with bilateral adrenal hyperplasia (Cushing's disease), one patient with adrenal carcinoma and one patient with primary adrenocortical nodular dysplasia (PAND). Moreover, these cases included some special ones, i.e. 5 cases with destructive thyroiditis after treatment, 2 cases with aggravation of arthritis after treatment, a case of Carney's complex with PAND, one case with paradoxical response to dexamethasone, and one case combined with empty sella syndrome. The most specific clinical signs were moon face (95% occurrence), hypertension (95%) and subcutaneous bruising (80%). Other significant signs were eye edema (66%), buffalo hump (68%), subcutaneous purpura (63%) and osteoporosis (49%). Skin striae was not a common sign in our cases (41%). Renal stone was observed in only 20% of our patients but was a significant sign in this syndrome. There was no difference in the occurrence of each clinical sign between Cushing's syndrome and Cushing's disease. The elevation of white blood cell count (WBC) and serum sodium, a decrease of serum potassium, and a decrease of reabsorption of phosphate (%TRP) were observed. Thyroid-stimulating hormone (TSH) and human growth hormone (HGH) were suppressed in patients with Cushing's syndrome and patients with Cushing's disease. These results were consistent with those of previous reports. However, luteinizing hormone (LH), follicle-stimulating hormone (FSH) and prolactin (PRL) were high in those patients with Cushing's syndrome and those with Cushing's disease. Oral glucose tolerance test was carried out in 34 patients before and after treatment. Thirty-one percent of those had diabetes mellitus and 26% had impaired glucose tolerance (IGT). The response of IRI in this test was high in patients with Cushing's syndrome and patients with Cushing's disease, and decreased 4 weeks after treatment in those with Cushing's syndrome but remained high in those with Cushing's disease. Plasma ACTH level and urinary 17-OHCS excretion were significantly higher in Cushing's disease than in Cushing's syndrome. During an 8mg-high-dose dexamethasone suppression test, urinary 17-OHCS excretion in 13 of 14 patients with Cushing's disease (93%) was suppressed by more than 50% of baseline on the second day of testing. However, all of 18 patients with Cushing's syndrome, who had an 8mg-dexamethasone suppression test, failed to suppress urinary 17-OHCS by 50% of baseline.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[Forty-one cases of Cushing's syndrome: a comparison between Cushing's syndrome (adrenal adenoma) and Cushing's disease (adrenal hyperplasia)]. 163 31


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>