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Neurofibromatosis type 1 (NF1) is associated with vascular lesions, such as renal artery stenosis, and secondary hypertension. The real prevalence is largely unknown, particularly in children. We observed 27 patients with NF1, mean age 12.8 years (range 4.2-24 years), for 2-10 years to assess the association of NF1 with vascular abnormalities and secondary hypertension. Patients were studied with angiography, 24-h blood pressure monitoring, a captopril test, and Doppler ultrasonography of aorta and renal arteries. The prevalence of hypertension was 18.5%; 61.5% of patients studied with angiography had vascular lesions, half of whom were apparently normotensive. However, they had abnormal 24-h blood pressure monitoring, which was a first sign of poor blood pressure control. Those patients with severe hypertension (11.1%) were successfully treated with percutaneous transluminal angioplasty (PTA); stenosis recurred in 2 of 3 patients after a 2-year follow-up period, and was responsive to drugs. We conclude that hypertension is a frequent complication of NF1 in pediatric patients, it is usually secondary to typical vascular lesions, and requires careful follow-up. Ambulatory blood pressure monitoring (24-h) is a sensitive method for detecting initial alterations of the blood pressure pattern. PTA may be an effective treatment in this condition.
online pharmacy ref source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10955932&dopt=Abstract
mfi.ku.dk
The role for ANG II in renal blood flow (RBF) autoregulation is unsettled. The present study was designed to test the effect of clamping plasma ANG II concentrations ([ANG II]) by simultaneous infusion of the angiotensin-converting enzyme inhibitor captopril and ANG II on RBF autoregulation in halothane-anesthetized Sprague-Dawley rats. Autoregulation was defined as the RBF response to acute changes in renal perfusion pressure (RPP). Regulation was defined as changes in RBF during long-lasting changes in RPP. The results showed that a prolonged reduction of RPP reset the lower limit of autoregulation from 85 +/- 1 to 73 +/- 2 mmHg (P < 0.05) and regulated RBF to a lower level. Reduction of RPP to just above the lower limit of autoregulation (88 mmHg) induced regulation of RBF to a lower level within 10 min. Clamping [ANG II] per se reset the lower limit of autoregulation to 62 +/- 5 mmHg. In this case, reduction in RPP to 50 mmHg did not induce a downregulation of RBF. We conclude that ANG II plays an important role in the resetting of the autoregulation limits. The ability to regulate RBF to a new level as a response to changes in RPP also depends on changes in [ANG II].
online pharmacy ref source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10956261&dopt=Abstract
J Thorac Cardiovasc Surg. 2000 Sep;120(3):573-80.
Inhibition of angiotensin-converting enzyme by captopril: a novel approach to reduce ischemia-reperfusion injury after lung transplantation.
Fischer S, Maclean AA, Liu M, Kalirai B, Keshavjee S.
Thoracic Surgery Research Laboratory, Division of Thoracic Surgery, Toronto General Hospital Research Institute, University of Toronto, Toronto, Ontario, Canada.
OBJECTIVES: Ischemia-reperfusion injury after lung transplantation involves the generation of free radicals. Captopril has been shown to be protective in models of ischemia-reperfusion injury in other organs by acting as a free radical scavenger. The purpose of this study was to assess the protective effects of captopril against ischemia-reperfusion injury and to evaluate the ability of captopril to scavenge free radicals and inhibit neutrophil activation in an experimental model of lung transplantation. METHODS: A rat single-lung transplant model was used. Donor lungs were flushed and preserved in low-potassium dextran-glucose solution with (n = 5) and without captopril (500 micromol/L; n = 5) for 18 hours at 4 degrees C and then transplanted and reperfused for 2 hours. At the conclusion of the 2-hour reperfusion period, arterial blood gases, blood pressure, and peak airway pressure were measured. Lung tissue biopsy specimens were obtained for assessment of wet/dry weight ratios, histology, and neutrophil sequestration (myeloperoxidase activity). Lipid peroxidation (F(2)-isoprostane assay) was analyzed from plasma samples and tissue lysates. RESULTS: The addition of captopril to the lung preservation solution significantly improved postreperfusion PO (2) (312 +/- 63.3 mm Hg vs 202 +/- 21.1 mm Hg; P =.006), peak airway pressure (11.4 +/- 1.1 cm H(2)O vs 15.6 +/- 1.5 cm H(2)O; P =.001), and wet/dry weight ratio (4.9 +/- 0.4 vs 15.8 +/- 10.9; P =.008). Blood pressures did not differ significantly between groups. No significant differences were seen in myeloperoxidase activity or F(2)-isoprostane levels. CONCLUSIONS: The use of captopril in the preservation solution ameliorates ischemia-reperfusion injury in transplanted lungs after an extended cold preservation period. The mechanisms by which captopril is protective remain elusive but do not appear to include inhibition of neutrophil sequestration or lipid peroxidation. This novel approach to ischemia-reperfusion injury may lead to improved lung function after transplantation and provide further insight into the pathogenesis of acute lung injury.
online pharmacy ref source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10962421&dopt=Abstract
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