Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lecithin-cholesterol acyltransferase is responsible for the formation of most cholesteryl esters in plasma. Absence of this enzyme can result in a rare syndrome that includes diffuse corneal opacities, normocytic normochromic anemia, proteinuria, renal failure, and premature arteriosclerosis. The deficiency can be inherited in an autosomal recessive manner, or it can be acquired through liver disease. Diagnosis requires a high index of suspicion and documentation of impairment of enzyme mass or activity (or both). This article includes a case report of the first United States citizen known to have lecithin-cholesterol acyltransferase deficiency. The authors review the literature related to this disease.
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PMID:Lecithin-cholesterol acyltransferase deficiency: first report of case in a United States citizen. 802 2

The upper limits of normal blood pressure have been considered to be 139 mmHg systolic and 89 mmHg diastolic for adults, but these values are not necessarily applicable to the elderly. This report presents blood pressure values of healthy persons aged 65 to 94 and estimates the upper limits of normal blood pressure in the elderly based on follow-up studies. The Blood Pressure Subgroup of the Study on Reference Values of Laboratory Tests in Elderly Subjects defined inclusion criteria for the healthy elderly as follows: (1) persons aged 65 to 94, (2) persons not complicated with cardiovascular diseases, (3) persons capable of living and walking freely, (4) persons without dementia, (5) persons without anemia, liver disease, renal failure, diabetes mellitus on drug treatment, lung disease, valvular disease or marked arrhythmias, (6) persons without neuromotor disease. The subgroup collected 2008 persons who fulfilled the criteria. Of the 2008 persons, 663 were not taking antihypertensive drugs, had body weight within an average Body Mass Index +/- standard deviation and had no abnormalities on ECG. The 663 persons were considered to be a group of most the normal elderly. Blood pressure values in this group were 133.3 +/- 18.9/77.0 +/- 10.6 mmHg for males (N = 318) and 134.3 +/- 18.7/75.7 +/- 10.2 mmHg for females (N = 345). Follow-up studies carried out by some members of the Blood Pressure Subgroup suggested that the upper limits of the normal blood pressure were 140 to 159 mmHg systolic and 80 to 89 mmHg diastolic for the elderly.
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PMID:[Reference values of laboratory tests in elderly subjects--blood pressure]. 804 Oct 19

We measured serum erythropoietin (EPO) immunoenzymatically in 245 subjects (151 male, 94 female) to investigate the pathophysiology of its liberation in patients with liver disease. Twelve patients had acute hepatitis, 60 mild chronic liver disease (CLD), 50 cirrhosis (CIR), 43 hepatocellular carcinoma (HCC), 16 malignant extrahepatic disease, 32 benign extrahepatic disease (BEN); 32 subjects served as healthy controls. Higher EPO levels were found in all groups of patients as compared with controls (Bonferroni's test, P < 0.01); CIR and HCC had higher values than CLD and BEN (P < 0.01). By multiple regression analysis, EPO correlated with haematocrit, cholinesterase and C-reactive protein (F = 18.63, P < 0.0001). Thus, circulating EPO increases in patients with liver disease, particularly in its more advanced forms. Besides anaemia, both impairment of liver function (possibly via decreased EPO metabolism) and inflammation seem to play contributory roles in elevating serum EPO.
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PMID:Evidence for a multifactorial control of serum erythropoietin concentration in liver disease. 755 88

The red-cell mass is continuously adjusted to the optimal size for its function as an oxygen carrier by messages transmitted to the bone marrow from an oxygen sensor in the kidney. These messages are mediated by the hormone erythropoietin. Erythropoietin is a glycoprotein growth factor synthesized by cells adjacent to the proximal renal tubule in response to signals from a renal oxygen-sensing device, probably a heme protein (1). In the bone marrow, erythropoietin binds to and activates specific receptors on the erythroid progenitor cells (2). In the presence of this erythropoietin-receptor complex the progenitor cells continue their predestined development into mature erythrocytes. Erythropoietin was the first hemopoietic growth factor to be molecularly cloned in 1985 (3). Our understanding of the biology and physiology of erythropoietin has been considerably improved with the advent of recombinant human erythropoietin (rHuEpo). During the past 7 years, rHuEpo has undergone extensive testing in clinical trials. It has been approved for treatment of the anemia of chronic renal failure, both in progressive renal failure and endstage renal failure (ESRD). In these instances, the administration of rHuEpo has been used in effect as a substitutive therapy, since patients' erythropoietin levels are very low despite severe anemia, due to the failure of affected kidneys to produce adequate amounts of the hormone. However, the application of rHuEpo has now moved largely from the primitive indication of renal diseases, and the hormone is currently under study in a number of anemic states of different etiologies, even with relatively high serum erythropoietin levels. Among these, some of the best documented indications are the anemia associated with malignancies, either due to neoplastic bone marrow infiltration or to chemotherapy-related myelosuppression, the anemia of myelodysplastic syndromes and AIDS, the anemia of chronic inflammatory diseases, prematurity, and bone marrow transplantation (4). The purpose of this review is to provide a summary of our present knowledge regarding rHuEpo therapy for the anemia of renal failure. We provide some clues for the correct use of rHuEpo in the treatment of the anemia of chronic inflammatory diseases. In addition, we address a series of new issues in the attempt to better understand the relationship between erythropoietin and liver disease.
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PMID:Erythropoietin and the anemia of chronic diseases. 840 91

Lidocaine-induced seizures have been reported after topical administration. A 30-year-old, 48-kg women with acquired immunodeficiency syndrome, chronic end-stage renal failure, anemia, congestive heart failure (CHF), cardiomyopathy, and increased liver function tests was admitted to the hospital with fever, chills, and dry cough. Bronchoscopy was performed to rule out Pneumocystis carinii pneumonitis; the patient experienced seizure activity after administration of a total dose of topical lidocaine 300 mg. Plasma drug concentration measured shortly after seizure, and at 4 and 22 hours after seizure were 12.0, 7.6, and 1.4 mg/L, respectively. A direct correlation exists between clinical symptoms and blood level of lidocaine; as the level increases to 8-12 mg/L the probability of seizure increases. The extent of absorption and bioavailability after airway administration depends on tissue vascularity, sites and techniques of application, patient's disease state, and, most important, the dose/unit body weight. The lidocaine dose should be titrated slowly and patients monitored for altered mental status. The dose often has to be decreased empirically in patients with liver disease or CHF. Efforts should be made to deliver minimum amounts of the drug to the lower respiratory tract, since its pharmacokinetics at that site are similar to those with intravenous administration.
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PMID:Seizure after lidocaine for bronchoscopy: case report and review of the use of lidocaine in airway anesthesia. 843 71

We report a case of Zieve's Syndrome that developed after an important alcohol consumption in a 32-yr-old female patient. She was admitted to the hospital with anorexia, asthenia and jaundice. Physical examination showed liver stigmata and hepatomegaly. Laboratory tests demonstrated increased aminotransferase levels, hyperbilirubinemia, hyperlipidemia and normocytic and normochromic anemia with dianocytes in peripheral blood smear. Ultrasonography showed a hyperechoic liver and a liver biopsy showed acute and chronic alcoholic liver disease. Clinical evolution was satisfactory and the therapy consisted of blood transfusion, parenteral fluids, B-complex vitamin and a fatty free diet. Jaundice, hyperlipidemia and haemolytic anemia define Zieve's Syndrome (Z.S.) There is a pathogenetic relationship among the clinical and biological phenomena in this syndrome, whose starter is an acute alcohol intake. Haemolysis is the distinctive feature with respect to the classical acute alcoholic hepatitis, and it is due to erythrocyte's metabolic and osmotic instability in relation to lipids abnormalities. Its clinical resolution precedes the normalization of serum lipids levels. Therapy is similar to that for acute alcoholic hepatitis although sometimes the anemia requires blood transfusion.
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PMID:[Zieve's syndrome. A case report]. 864 20

US teenagers have had access to the injectable contraceptive depot medroxyprogesterone acetate (DMPA; Depo-Provera) since the US Food and Drug Administration approved it in 1992. DMPA suppresses follicle stimulating hormone and luteinizing hormone (LH) levels, which in turn prevents the LH surge and thus inhibits ovulation. It also causes a thick cervical mucus (reducing sperm penetration). Since DMPA also changes tubal mobility and creates shallow and atrophic endometrium, implantation is prevented. DMPA must be administered every 3 months to be effective. Its first-year failure rate is 0.3%, which is lower than that of oral contraceptives (3%). Advantages of DMPA are that it: allows for privacy; improves compliance (since action is required every 3 months rather than every day); has no estrogen-related complications (e.g., thrombophlebitis); is effective; is safe for breast feeding teenagers; reduces seizure frequency in teenagers with epilepsy; has a favorable effect on sickle cell disease or coagulopathy; reduces menstrual flow, thus preventing iron-deficiency anemia; reduces menstrual pain and pre-menstrual symptoms; and decreases risk of pelvic inflammatory disease. The leading disadvantages are menstrual irregularities and spotting. Some other possible disadvantages include weight gain (most common reason for discontinuation), delayed return of fertility, headaches, acne, and nervousness. Health providers must perform a complete history of teenagers requesting DMPA. They should determine the presence or absence of absolute and relative contraindications to DMPA. Absolute contraindications are known or suspected pregnancy, undiagnosed or abnormal vaginal bleeding, known or suspected history of breast cancer, acute liver disease or jaundice, thromboembolism, and sensitivity to DMPA. DMPA is administered intramuscularly at a concentration of 150 mg/ml. Health providers need to use a frank, nonjudgmental, empathic, and unhurried approach to facilitate a trusting relationship and rapport with teenagers. Advanced counseling on the pros and cons of DMPA, how DMPA works, and DMPA's inability to protect against sexually transmitted diseases is essential.
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PMID:Use of depo-provera in teens. 892 Mar 51

Growing numbers of patients suffering from many symptoms believe that they have a condition called multiple chemical sensitivity syndrome (MCSS). It has been suggested that this syndrome can be triggered by exposure to any of a large and usually incompletely defined number of natural and synthetic chemical substances. Major medical organizations, including the National Research Council and the American Medical Association, have not recognized MCSS as a clinical syndrome because of a lack of valid, well-controlled studies defining it and establishing pathogenesis or origin. Lately, some have proposed that many patients with MCSS suffer from hereditary coproporphyria. However, this purported association is based chiefly on results from a single reference laboratory of a fundamentally flawed assay for erythrocyte coproporphyrinogen oxidase. Although patients with MCSS may, at times, have modest increases in urinary coproporphyrin excretion, this is a common finding found in many asymptomatic subjects or patients with diverse other conditions (eg, diabetes mellitus, heavy alcohol use, liver disease, and many kinds of anemia). Such secondary coproporphyrinuria does not indicate the existence of coproporphyria. To our knowledge, there is no scientifically valid evidence to support an association between MCSS and coproporphyria, nor is there any unifying hypothesis for rationally linking these 2 disorders.
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PMID:Multiple chemical sensitivity syndrome and porphyria. A note of caution and concern. 904 Feb 94

We report 3 cases of acute hepatitis A infection with haematological manifestations. In the first case, severe aplastic anemia occurred in a 6 year-old child, who underwent 3 bone marrow grafts before responding favourably. In the second and third cases, severe anemia and thrombocytopenia occurred in a 42 year-old man with cholestatic hepatitis, and in a 66 year-old man with fulminant hepatitis; there was a favourable outcome in both cases. These cases demonstrate that haematological manifestations in hepatitis A can be severe, independent of the severity of liver disease. Although these manifestations seem to be rare, we suggest performing systematic haematological evaluations in cases of viral hepatitis A with unusual outcomes.
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PMID:[Hematologic manifestations related to hepatitis A virus. 3 cases]. 920 99

Gastrointestinal hemorrhage in the patient with liver disease is often massive and life threatening. Although varices are the most likely cause of hemorrhage, other sources, such as peptic ulcer disease, Mallory-Weiss tears, and portal hypertensive gastropathy, are common. As liver disease is an important risk factor for intractable bleeding and death in patients with gastrointestinal hemorrhage, outcome in these patients is often poor regardless of the cause of the bleeding. Essential elements of initial therapy include prompt and adequate intravascular volume replacement, correction of severe anemia and coagulopathies, and adequate airway management. After initial resuscitation, urgent endoscopy is required to secure the diagnosis and to deliver endoscopic therapy if possible. The specific form of therapy will differ depending on the lesion encountered. Adjunctive measures, such as the administration of antibiotics and drugs that reduce portal pressure, including octreotide, may also improve outcome. Clinical and endoscopic information can be used to predict first bleeding in patients with liver disease. A large body of data supports the use of beta-blockers in the prevention of first bleeding in patients with known varices.
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PMID:The importance of accurate diagnosis and vigorous care of the patient with liver disease and gastrointestinal hemorrhage. 936 Feb 81


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