Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fat emulsion has been widely accepted a parenteral nutrient for the prevention of essential fatty acid deficiency and the supply of a high energy source. The following problems associated with the administration of fat emulsion are, however, well recognized: 1) The soy protein detected slightly in soy bean oil may induce allergic reactions. 2) Although administered in small quantities emulsified fat particles over 5 microns in diameter can cause fat emboli, including as pulmonary, splenic, placental, and cerebral emboli. 3) Hyperlipidemia may cause pulmonary hypertension and hemophagocytosis when triglycerides contained in the fat emulsion are intravenously administered at a rate greater than 0.1g/kg/hour. 4) Fat emulsion may have immunosuppressive effects, because emulsified fat particles phagocytized by the reticuloendothelial system reduce its functions. Long-chain triglyceride of the omega-6 series can suppress the functions of lymphocytes and neutrophils through prostaglandins. 5) Fungal infections, especially with Malassezia furfur which requires fat for growth, are sometimes associated with intravenous fat emulsion infused through a central venous catheter.
 ...
PMID:[Adverse effects of intravenous fat emulsion administration]. 961 97

The number of women with congenital cardiac disease, who mature into adulthood is increasing. Unfortunately, there are no prospective data published about the relative risk of different forms of contraception for these patients. Most women with congenital cardiac disease can safely use oral contraceptives, especially low-estrogen combination or progestin-only preparations, with the exception of those, who are at particular risk because of thromboembolic complications (especially in cyanosis, pulmonary hypertension, Eisenmenger reaction, rhythm disturbances), fluid retention (especially in reduced ventricular function and congestive heart failure), arterial hypertension (important in coarctation), infectious complications (endocarditis) or hyperlipidemia. Oral contraceptives should be avoided in patients at increased risk for thromboembolic events. Intrauterine devices are very effective, have no metabolic side effects and merely carry a small risk of endocarditis. Newer devices containing progesterone only may put the patients at a still smaller risk. Contraceptive subdermal implants (e.g. levonorgestrel) are used with good results in the United States for patients with contraindications to estrogen-containing oral contraceptives and may well become more widely accepted in patients in Germany in the coming years. Barrier methods can be used, but have a higher failure rate, which may be unacceptable in patients at risk (e.g. Eisenmenger's). Especially in Eisenmenger's, permanent sterilisation should be advised.
 ...
PMID:[Contraception in patients with congenital heart defects]. 1095 86

Lung transplantation has become an accepted treatment modality for end stage lung disease including emphysema, fibrosing alveolitis, cystic fibrosis, pulmonary hypertension and bronchiectasis. Despite the use of potent immunosuppressive drugs, acute rejection occurs frequently, especially in the first few weeks and months after transplantation. Bacterial, viral and fungal infections frequently occur in lung transplant recipients. Rapid diagnosis and adequate treatment of infections is needed. The side effects with the use of long term immunosuppressive agents includes renal toxicity, hypertension, neurotoxicity, hyperlipidemia, leucopoenia, hyperglycaemia, weight gain, osteoporosis and malignancy. However, obliterative bronchiolitis (OB) which is regarded as a chronic rejection process remains the dominant cause of morbidity and mortality in the long-term survivors of lung transplantation. This article focuses on the postoperative and long term management of lung transplant recipients.
 ...
PMID:Lung transplantation: management and complications. 1184 31

Traditional health evaluations are performed while the patient is at rest. Stress echocardiography extends these examinations by providing data in a physiologic setting more closely mimicking the typically active state of children. The test represents a fusion of the fields of two-dimensional echocardiography and cardiovascular stress testing and can be used to assess myocardial perfusion in patients with suspected coronary artery pathology or to evaluate cardiac gradients or functional reserve in patients with noncoronary artery pathology. Testing should be performed with a trained sonographer and attending physician and in collaboration with adult cardiology colleagues. Stress can be administered to the patient through either exercise or pharmacologic agents. Echocardiography is used to assess regional wall motion abnormalities when evaluating myocardial perfusion or gradients and/or function when assessing the patient without coronary artery issues. Conditions with potential coronary artery pathology for which stress echocardiography is appropriate include Kawasaki disease, transplant graft vasculopathy, arterial switch operation for transposition of the great arteries, anomalous coronary artery origins or courses, pulmonary atresia with intact ventricular septum, hyperlipidemia, insulin-dependent diabetes mellitus, and supravalvar aortic stenosis. Stress echocardiography can also be helpful in determining the behavior during activity of gradients in conditions such as hypertrophic cardiomyopathy or aortic and pulmonic stenosis, of cardiac pressures in pulmonary hypertension and of ventricular function in conditions such as dilated cardiomyopathy or mitral and aortic regurgitation.
 ...
PMID:Pediatric stress echocardiography. 1197 81

Schimke-immuno-osseous dysplasia (SIOD) is a multisystem disorder caused by a mutation of the chromatin remodeling protein. The main clinical findings are spondyloepiphyseal dysplasia with disproportional growth deficiency, nephrotic syndrome with focal and segmental glomerulosclerosis, and defective cellular immunity. Transitory ischemic attacks due to vaso-occlusive processes are still an untreatable and life-limiting complication in patients with SIOD. The underlying pathophysiology of vaso-occlusive processes in SIOD is unclear. We report the clinical and pathological findings of the eldest published patient with the severe form of SIOD, who died at the age of 23 years due to pulmonary hypertension with subsequent right heart failure. The autopsy revealed a severe generalized atherosclerosis including the brain, heart, and pulmonary arteries. However, the kidney that was transplanted at the age of 5 years showed a good graft function without glomerular sclerosis and with only minimal nephrosclerosis on histology. Thus, the absence of severe vaso-occlusive processes in the transplanted organ and in the severely atherosclerotic host may indicate that the vaso-occlusive processes in SIOD are not caused by post-transplant cardiovascular morbidity such as arterial hypertension and hyperlipidemia. Instead, vascular factors of the host such as endothelial dysfunction may explain the pathophysiology of atherosclerosis in SIOD.
 ...
PMID:Generalized atherosclerosis sparing the transplanted kidney in Schimke disease. 1505 43

Cardiovascular manifestations of HIV vary according to disease stage, treatment regimen and geographical location. Common cardiac complications of HIV disease in patients off highly active antiretroviral therapy (HAART) include dilated cardiomyopathy, myocarditis, pericardial effusion, endocarditis, pulmonary hypertension and non-antiretroviral drug-related cardiotoxicity. However, with the introduction of HAART that has substantially modified the course of HIV disease by lengthening survival, additional cardiovascular consequences are a result of the metabolic syndrome with a propensity toward hyperlipidaemia and atherosclerotic heart disease. Because most of the world's HIV-infected patients have not been treated with HAART, the principal HIV-associated cardiovascular manifestations of patients off HAART are reviewed and new knowledge about the prevalence, pathogenesis and treatment in the HAART era are emphasised in this review. Exercise, a nonpharmacological approach to treating HAART-associated metabolic syndrome, is also discussed.
 ...
PMID:Cardiovascular disease and toxicities related to HIV infection and its therapies. 1625 61

The survival of patients with HIV infection who have access to highly active antiretroviral therapy has dramatically increased. In HIV-infected persons, cardiovascular disease can be associated with HIV infection, opportunistic infections or neoplasias, use of antiretroviral drugs or treatment of opportunistic complications, mode of HIV acquisition (such as intravenous drug use), or with the classic non-HIV-related cardiovascular risk factors (such as smoking or age). Diseases of the heart associated with HIV infection or its opportunistic complications include pericarditis and myocarditis. Pericarditis may lead to pericardial effusion rarely causing tamponade. Cardiomyopathy is often clinically silent with asymptomatic left ventricular systolic dysfunction. Endocarditis is mainly the consequence of intravenous drug abuse, possibly leading to life-threatening valvular insufficiency with the need for cardiac surgery. A further serious condition associated with HIV infection is pulmonary hypertension potentially leading to right heart failure. The cardiovascular complications of HIV infection such as cardiomyopathy and pericarditis have been reduced by highly active antiretroviral therapy, but premature coronary atherosclerosis is now a growing problem because antiretroviral drugs can lead to serious metabolic disturbances resembling those in the metabolic syndrome. Lipodystrophy, a clinical syndrome of peripheral fat wasting, central adiposity, dyslipidemia, and insulin resistance, is most prevalent among patients treated with protease inhibitors. These patients should thus be screened for hyperlipidemia, hyperglycemia, and hypertension, and they may be candidates for lipid-lowering therapies. When initiating lipid-lowering therapy, interactions between statins and HIV protease inhibitors affecting cytochrome P450 function must be considered. Restenosis rate after percutaneous coronary intervention may be unexpectedly high.
 ...
PMID:Cardiovascular disease in HIV infection. 1678 Dec 13

To examine the clinical manifestations, intensity of oral anticoagulation and outcomes in the prevention of recurrent thromboses in patients with antiphospholipid syndrome (APS) in a tertiary rheumatology centre in Singapore. Retrospective case review of consecutive patients with APS attending a rheumatology clinic from 1st January 2004 to 31st December 2005. There were 59 (44%) patients with definite APS and 75 (56%) with probable APS. Systemic lupus erythematosus (SLE) was the most common cause of secondary APS. Hypertension and hyperlipidaemia were the most common cardiovascular comorbidities. The most common manifestations were haematological (thrombocytopaenia and haemolytic anaemia), neurological (seizure, headache) and pulmonary hypertension. Among those with definite APS, there were similar proportions with arterial and venous thromboses. Recurrent thromboses occurred in 14 (23.7%) patient with definite APS receiving warfarin, comprising 14 (73.7%) episodes of arterial and 5 (26.3%) episodes of venous thromboses. Recurrent arterial thromboses occurred at international normalized ratio (INR) of <2 in 5 (35.7%), INR 2-3 in 6 (42.9%), INR > 3 in 3 (21.4%) episodes, respectively. Recurrent venous thromboses occurred at INR < 2 in 4 (80.0%) and INR > 3 in 1 (20.0%) episode, respectively. Twenty-eight episodes of bleeding occurred in 21 (35.6%) patients, the majority (78.6%) being minor bleeding. Two-thirds of all major bleeds occurred at INR >/= 3. Venous and arterial thromboses were equally common in our patients with definite APS, although recurrent thromboses were more common in the arterial circulation. Target INR > 3 was associated with lower rates of recurrent arterial thromboses but higher rates of major and recurrent bleeding. Target INR >/= 2 appeared to be sufficient to prevent recurrent venous thromboses.
 ...
PMID:Clinical manifestations and outcomes of antithrombotic treatment of the Tan Tock Seng Hospital Singapore antiphospholipid syndrome cohort. 1950 74

Type 1a glycogen storage disease (GSD 1a), or von Gierke disease, is a rare, autosomal-recessive disease caused by a deficiency of glucose-6-phosphatase, which leads to glycogen accumulation in the liver, kidney, and intestinal mucosa. Clinical manifestations include hypoglycemia, growth retardation, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Long-term complications include renal disease, gout, osteoporosis, pulmonary hypertension, short stature, and hepatocellular adenomas, which may undergo malignant transformation. Herein we have described the management and the clinical course of a GSD1a patient who underwent simultaneous preemptive liver- kidney transplantation (SPLKT), which solved the liver and renal disease. We confirmed the rapid normalization of glucose metabolism, and correction of hyperlipemia after liver transplantation. In our opinion uremic patients with GSD 1a with or without adenomas must be considered for SPLKT. To our knowledge this is the fifth case of SPLKT and the first preemptive one to be described in the literature.
 ...
PMID:Preemptive liver-kidney transplantation in von Gierke disease: a case report. 2162 87

Mitochondrial disorders (MIDs) may occasionaly go along with dysmorphism but hand deformities, as in the following case, have been only rarely reported. A 72 year old female with ptosis, hypoacusis, tremor, myopathy, diabetes mellitus, arterial hypertension, severe cardiac disease, pulmonary hypertension, gastric carcinoid, hepatopathy, generalised atherosclerosis, anemia, polyarthrosis, and hyperlipidemia, additionally presented with brachydactylia. Upon neurological work-up a MID was suspected. The family history was positive for diabetes but negative for brachydactylia or other features of a MID. MIDs may be associated with brachydactylia. Skeletal deformities may be a phenotypic manifestation of MIDs.
 ...
PMID:Brachydactylia as a phenotypic feature of mitochondrial disorder. 2345 27


1 2 Next >>