Lysinuric protein intolerance
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop in Atlas of Inherited Metabolic Diseases, 2020
IV sodium benzoate or phenylacetate, or both, may be employed as adjunctive therapy [57]. Lysine depletion may be improved with supplemental L-lysine-HCl (0.05–0.5 mmol/kg, three times per day) [58], but this is limited by malabsorption and intestinal tolerance. ɛ-N-Acetyllysine has been shown to increase plasma concentrations of lysine [59]. Increase may also be accomplished by the IV administration of lysine [60]. Pulmonary disease may be effectively treated with high-dose regimens of prednisolone [43], but some patients have not responded. Repeated whole-lung lavages are a successful approach for pulmonary alveolar proteinosis [61]. Heart-lung transplantation has not been recommended, because recurrence of disease in transplanted lungs has been reported [62]. Effective treatment of renal complications has not yet been reported. Elevated levels of cholesterol and triglyceride were documented in 39 Finnish patients [17] whose fat intake was no higher than the general population. Successful lowering was obtained with statin therapy. The authors recommended atorvastatin over others because of more effective reduction of both cholesterol and triglyceride.
Commonly prescribed drugs
Alistair Burns, Michael A Horan, John E Clague, Gillian McLean in Geriatric Medicine for Old-Age Psychiatrists, 2005
AmiodaroneIndicationsThis is used for the treatment of supraventricular and ventricular arrhyth- mias when other drugs are ineffective or contraindicated. Side-effects/cautionsSinus brachycardia and heart block are side-effects. It should only be initi- ated under specialist hospital provisi�n. It has a very long half-life and only needs to be given once a day. Liver function and thyroid function tests should be carried out before treatment and every 6 months. Dosage 200 mg three times a day for a week, reducing to 200 mg twice a day for a further week, with the maintenance dose being 200 mg a day. StatinsThe statins (atorvastatin, fluvastatin, pravastatin, rosuvastatin and simvas- tatin) inhibit one of the enzymes involved in cholesterol synthesis in the liver and are the most effective class of drugs in reducing low-density lipoproteins (LDLs) and cholesterol. There is good evidence that taking statins reduces myocardial infarction, cardiovascular episodes and mortality. They are now very widely used for the primary and secondary prevention of all cerebrovascular and cardiovascular events. Their main use is obviously in the reduction of cholesterol, but while the risk of coronary events is not particularly accurately predicted from the level of cholesterol alone, it is still a reasonable target. Side-effects/cautionsLiver disease or high alcohol intake should prompt the statins to be used with caution. Liver function tests should be done before and within 3 months of starting treatment and repeated every 6 months. Treatment should be discontinued if serum transaminase concentration is three times normal. Reversible myositis is a significant (but rare) side-effect of the statins, and treatment should be discontinued if creatine kinase is more than five times normal. Myalgia, myositis and myopathy have all been reported, and rhabdomyolysis associated with acute renal failure has been reported. Dosage The level of cholesterol which should trigger prescription of a statin is said to be when the total concentration is 5 mmol/1 or greater, and there is a coronary heart risk of 30% or greater over 10 years.
Cardiovascular Drugs
Radhwan Nidal Al-Zidan in Drugs in Pregnancy, 2020
FDA Category: XRisk Summary : The use of Atorvastatin is contraindicated during pregnancy due to the fact that Cholesterol and products synthesized by cholesterol are essential during fetal development. Moreover, the discontinuation of Atorvastatin during pregnancy should have no influence on the long-term treatment of hyperlipidemia.
Effects of Atorvastatin on Systemic and Renal Nitric Oxide in Healthy Man
Published in Clinical and Experimental Hypertension, 2013
Frank Holden Mose, Thomas Larsen, Jesper Noergaard Bech, Erling Bjerregaard Pedersen
Statin treatment improves endothelial function but the effects of statins on renal nitric oxide have not been clarified. In this crossover study, 26 healthy subjects received atorvastatin 80 mg per day or placebo for 5 days. After 5 days of treatment, L-NG-monomethyl arginine caused a similar increase in blood pressure and decrease in urine output and glomerular filtration rate. The decrease in fractional excretion of sodium to L-NG-monomethyl arginine was more pronounced after atorvastatin treatment. Atorvastatin did not change the response to several vasoactive hormones. The results indicate that atorvastatin increase renal nitric oxide, which may explain a part of the pleiotropic effects of statins.
Atorvastatin: safety and tolerability
Published in Expert Opinion on Drug Safety, 2010
Vasilios G Athyros, Konstantinos Tziomalos, Asterios Karagiannis, Dimitri P Mikhailidis
Importance of the field: Atorvastatin is the most widely used statin administered in a variety of settings, including primary and secondary prevention of cardiovascular events, in the elderly, in patients with chronic kidney disease and in diabetic patients. Therefore, the safety and tolerability of atorvastatin is of paramount importance. Areas covered in this review: We searched MEDLINE for literature published between 1997 and 2010 on the safety and tolerability of atorvastatin. We retrieved data from randomized controlled trials, meta-analyses, post-marketing studies, reports to regulatory bodies and case reports of rare adverse events. What the reader will gain: The reader will gain insight into the incidence, severity, prevention and management of the major adverse effects of atorvastatin (i.e., liver function abnormalities and muscle-related side effects) overall and in special populations. Take home message: The existing data suggest that atorvastatin is generally well tolerated across the range of its therapeutic dosage (10 – 80 mg/day).
Atorvastatin and cardiovascular protection: a review and comparison of recent clinical trials
Published in Expert Opinion on Pharmacotherapy, 2005
Kausik K Ray, Christopher P Cannon
Until recently, atorvastatin was known only as a new but more potent statin (‘me too’ drug) for lowering low-density lipoprotein cholesterol. In the last 2 years, data has become available on nearly 32,000 patients, in clinical settings ranging from primary prevention to acute coronary syndromes. These trials show the remarkably consistent clinical benefit of atorvastatin in patients with hypertension, diabetes, acute coronary syndromes, stable coronary disease with reductions in death, myocardial infarction and stroke, as well as in prevention of atherosclerosis progression. In addition, new data are also emerging to suggest that intensive therapy with high dose atorvastatin 80 mg is associated with a greater reduction in inflammatory markers, such as C-reactive protein, compared with other statin regimens. This suggests that intensive therapy with atorvastatin is associated with a potent pleiotropic effect. This review aims to summarise the recently concluded and ongoing clinical trials and to stimulate further reading.
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