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

Structural cardiac defects such as peripheral pulmonary stenosis are well-described in Alagille syndrome (AS), which is transmitted in an autosomal dominant inheritance. The genetic defect, with incomplete penetrance and variable expression, is localized to the short arm of chromosome 20. Abdominal coarctation is an uncommon congenital anomaly, with a spectrum of symptoms that may range from hypertension, intermittent claudication to abdominal pain. The association of abdominal coarctation with AS is rarely described. We report such a patient who also had aberrations of the visceral vascular supply involving the celiac, splenic, and superior mesenteric arteries. The indications to treat the coarctation, and in the context of a patient with AS, in whom liver transplantation may be contemplated at some stage, merit discussion.
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PMID:Abdominal coarctation and Alagille syndrome. 1087 78

We report here a 9-year-old girl with fibromuscular dysplasia of many muscular arteries including both renal and internal carotid arteries, the celiac artery, superior mesenteric artery, and one external carotid artery. She suffered from severe renovascular hypertension with beginning secondary cardiac decompensation, typical angina abdominalis, and neurological signs, including severe headaches and hemianopsia. Surgery was performed for all major vessels and the outcome is good 2.5 years after the operation. The clinical presentation, differential diagnosis, and treatment options of fibromuscular dysplasia in childhood are discussed and the literature is reviewed.
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PMID:Multivisceral fibromuscular dysplasia in childhood: case report and review of the literature. 1099 May 61

Coral reef aorta is a rare calcifying disease of the juxtarenal and suprarenal aorta. We report here our surgical experience in treating 21 patients, with a mean follow-up of 4 years and 7 months. Both genders were equally affected. Ten male (48%) and 11 female (52%) patients with a mean age of 54.6 years (range 42-76 years) underwent surgery. The main symptoms were limb claudication (n = 11, 52%), renovascular stenosis (n = 9, 43%) with concurrent renovascular hypertension (n = 5, 24%), and angina abdominalis (n = 7, 33%). Most patients had multiorgan vascular disease such as iliofemoral arterial occlusive disease (n = 14, 66%), coronary artery obstruction (n = 8, 38%), or obstruction of the carotid artery (n = 6, 28%). Risk factors did not differ between coral reef patients and those with other occlusive vascular diseases. All patients were treated through vascular operations, including open thromboendarterectomy of the suprarenal (n = 9, 43%), infrarenal (n = 4, 19%), or supra- and infrarenal aorta (n = 8, 38%), and thromboendarterectomy of the following vessels: celiac artery (n = 7, 33%), superior mesenteric artery (n = 12, 57%), inferior mesenteric artery (n = 3, 14%), unilateral renal artery (n = 3, 14%), or bilateral renal artery (n = 9, 43%). Bypass reconstructions were performed in 39% (n = 8). A thoracoabdominal approach was used in 14 patients (67%) and a median laparotomy in 7 (33%). Our results show that coral reef aorta is not confined to either gender. It appears most frequently in the context of general atherosclerotic disease and patients benefit from timely diagnosis and operation before onset of severe, life-threatening visceral and renal complications.
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PMID:Coral reef aorta: a long-term study of 21 patients. 1112 58

We report on a 15-year-old boy with stenosis and occlusion of multiple cranial, renal, and celiac arteries, aneurysm of the basilar artery, bilateral cutaneous syndactyly between fingers IV-V, partial cutaneous syndactyly between fingers III-IV on the right hand, brachydactyly, and borderline mental retardation. The clinical course was characterized by recurrent abdominal pain, gastritis, and high blood pressure. The pattern of the clinical and radiological findings is different from fibromuscular dysplasia (FMD) and Moyamoya disease, and highly suggestive of a syndrome described by Grange in four siblings (MIM#602531) Grange et al. [1998: Am J Med Genet 75: 469-480].
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PMID:Severe arterial occlusive disorder and brachysyndactyly in a boy: a further case of Grange syndrome? 1124 88

Results of the treatment of 71 patients with postbulbar ulcer of the duodenum are presented. Stomach resection by Bilrot-II was performed in 22 (30.9%) patients, by Bilrot-I--in 2 (2.8%), by Roux with plastic reconstruction of duodenal stump with intestinal tube--in 16 (22.5%) patients. Precardial proximal vagotomy with drainage operation was performed in 31 (43.6%) patients with massive periulcerous infiltration. Acute postoperative pancreatitis (APP) was seen in 29 (40.8%) patients chiefly after resection of stomach, 6 (8.4%) of them had pancreonecrosis. Postoperative lethality in the group of patients with APP was 12.6%. Ultrasonic examination of the pancreas and its vascular structures is the main method for diagnosis of postoperative pancreatitis. The cause of APP is the mechanic deformation and spasm of major duodenal papilla (MDP) that may be associated with cholinergic denervation and edema of MDP after resection and trauma of tissues. The removal of MDP spasm is an effective prophylactic measure for elimination of pancreatic hypertension. One of the methods of removal of MDP spasm is periarterial sympathectomy of gastroduodenal artery and prolonged drug blockade of celiac plexus in addition to stomach resection.
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PMID:[Acute pancreatitis after resection of stomach for low duodenal ulcer]. 1140 Apr 59

Takayasu's arteritis is a non-specific form of vasculitis involving the aorta, its main branches and pulmonary arteries. It is a rare disease in our country, contrasting with the high prevalence in Southeast Asia, Africa and South America. We discuss the course of the disease in our patient who was a young women who developed Takayasu's arteritis associated with autoimmune thyroiditis and malabsorption syndrome due to celiac sprue. Long-term immunosuppressive therapy contributed to stabilizing the associated diseases, but did not stop the progression of the vascular lesions. The main adverse outcome in our patient was the development of severe renovascular hypertension. Bilateral renal artery stenosis was treated by angioplasty with stent implantation. Based on literature reports, the association of Takayasu's disease with multiple autoimmune disorders is a rare event. However, it would appear that the arteritis was the limiting disease for prognosis in our patient.
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PMID:[Takayasu's disease associated with autoimmune thyroiditis and celiac disease. Clinical course and limitations of treatment]. 1143 24

We report a nine-year-old boy referred for evaluation of suspected coarctation of the aorta. He presented with hypertension and absent femoral pulses. A 50 mmHg gradient was detected in the abdominal aorta by color flow doppler echocardiography but the level of coarctation was impossible to locate. The diagnosis was made by magnetic resonance imaging and aortography, showing segmentary hypoplasia in the upper abdominal segment of the descending aorta, immediately below the celiac axis and above the renal arteries. Treatment consisted in placement of a stent Palmaz, which was effective in significantly decreasing the gradient. Some clinical aspects and different therapeutic strategies for this unusual case are discussed.
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PMID:[Coarctation of the abdominal aorta. Treatment by stent placement]. 1170 45

This study focuses on certain aspects of the renal structure of the giraffe, with some implications as to its function. About 4,000 collecting ducts open at the truncated end of a curved crest that juts into the renal pelvis as the inner medulla (IM). Extensions of the pelvis pass between the medullary (MP) and vascular (VP) processes almost to the corticomedullary border. The MPs contain an IM and an outer medulla (OM) containing clusters of capillaries (vascular bundles). The VPs contain the interlobar arteries and veins. All of the IM and almost all of the OM, with its vascular bundles, are bathed with pelvic urine. The cortex comprises 63% of the parenchyma. The OM has nine times the mass of the IM. The IM comprises 4% of the parenchyma. The ratio of mass of the adult cortex to the medulla is 1.7:1.0, and the number of glomeruli per kidney is 6.6 x 10(6). Glomerular mass is 6.2-6.7% of renal mass in the adult and 5.2% in the 6-month-old calf. The dimensions of the glomerular capsules are the same across the thickness of the cortex. Every terminal collecting duct drains an estimated 1,650 nephrons. In the adult giraffe the ratio of thickness of the muscularis of the main renal artery (RA) to its diameter is 0.117 (right RA) and 0.132 (left RA). These ratios are close to those in rhinoceros and ox but greater than in man. The visceral arteries (celiac, anterior mesenteric, and renal) have about the same muscularis : diameter ratio. Giraffes have arterial hypertension, but atherosclerosis is apparently absent and serum lipid fractions are low.
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PMID:Kidney of giraffes. 1199 78

The vascular endothelium maintains a relatively vasodilated state via the release of nitric oxide (NO), a process that could be disrupted by hyperhomocysteinaemia. Since endothelial dysfunction is associated with increased systemic vascular resistance that is the hallmark of sustained arterial hypertension, we hypothesised that in patients with both hypertension and coeliac disease with hyperhomocysteinaemia (via malabsorption of essential cofactors), treatment of the latter disease could improve blood pressure (BP) control. A single patient with proven sustained hypertension and newly-diagnosed coeliac disease had baseline and post-treatment BP and endothelial function assessed by ambulatory BP monitoring (ABPM) and brachial artery forearm occlusion plethysmography respectively. This 49 year-old woman had uncomplicated sustained hypertension proven on repeated ABPM carried out 6 weeks apart (daytime mean 151/92 mm Hg and 155/95 mm Hg), and sub-clinical coeliac disease (gluten-sensitive enteropathy). Initial assessments revealed raised homocysteine levels with low normal vitamin B(12) level. It was likely that she had impaired absorption of essential cofactors for normal homocysteine metabolism. She adhered to a gluten-free diet and was give oral iron, folate and B(6) supplementations as well as B(12) injections for 3 months. Her BP had improved by 6 months and normalised by 15 months (daytime ABPM mean 128/80 mm Hg). There was parallel restoration of normal endothelial function with normalisation of her homocysteine levels. These observations suggest that sub-clinical coeliac disease related hyperhomocysteinaemia might cause endothelial dysfunction, potentially giving rise to a reversible form of hypertension. In addition, this case study supports the notion that irrespective of aetiology, endothelial dysfunction may be the precursor of hypertension. This highlights the need to resolve co-existing vascular risk factors in patients with hypertension.
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PMID:Reversible hypertension following coeliac disease treatment: the role of moderate hyperhomocysteinaemia and vascular endothelial dysfunction. 1203 96

We report a case of systemic sclerosis complicated by Takayasu's arteritis. A 68-year-old woman had been treated for hypertension since 1984. She also had Raynaud's phenomenon and noticed vertigo and dizziness. In January 1992, she was diagnosed with back bruit. In April 1994, she was complicated by vertigo and diagnosed with asymmetrical blood pressure. In October 1998, she consulted our hospital because of neck bruit, abdominal bruit, back bruit and stenosis of descending aorta bifurcation on chest computed tomography. Her blood pressure was asymmetrical, being recorded as 190/101 on the right and 140/90 on the left. Scleroderma was observed from the finger to forehand, precordia, and face. Telangiectasia was observed on the precordia. Laboratory studies revealed the presence of anti-nuclear antibody (x 1280, discrete speckled.), anti-centromere antibody but anti-topoisomerasel antibody was negative. Skin biopsy from the left forehand detected proliferation of collgen fibers and perivascular inflammatory cell infiltration. A diagnosis of systemic sclerosis was made according to the American Rheumatism Association criteria. We suspected complication by Takayasu's arteritis because of asymmetrical blood pressure and bruit. Chest-abdominal angiography detected stenosis of the right brachiocephalic trunk, celiac artery, and left renal artery. We diagnosed Takayasu's arteritis. This is the second case report of a patient with systemic sclerosis complicated by Takayasu's arteritis.
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PMID:[A case of systemic sclerosis complicated by Takayasu's arteritis]. 1216 15


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