Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In an effort to clarify the features of hepatic dysfunction in sickle cell disease, we obtained serial tests of liver function in 100 consecutive patients with sickle cell anemia and in 30 consecutive patients with hemoglobinopathy SC during a five-year period. There were 32 patients with chronic abnormalities in tests of liver function. These abnormal tests were explained by a variety of lesions in 30 cases, and the liver disease remained unexplained in only 2 patients who declined liver biopsy. The diagnoses in these 30 patients included hepatitis, chronic passive congestion, common duct obstruction, alcoholic liver disease, pregnancy, collagen-vascular disease, and sarcoidosis. Evidence for hepatitis B infection was present in 19 of those with sickle cell anemia and in 6 of those with hemoglobinopathy SC. The bilirubin levels in sickle cell anemia appeared to have a trimodal distribution, with six patients exhibiting markedly elevated levels of indirect bilirubin suggesting a difference in bilirubin metabolism. There was no evidence of liver disease in 72 patients with sickle cell anemia, nor in 24 patients with hemoglobinopathy SC, as these patients exhibited only mild elevation of their serum indirect bilirubin levels owing to chronic hemolysis. Intrasinusoidal sickling and Kupffer cell erythrophagocytosis were nearly universal findings at liver biopsy, irrespective of the clinical disorder, and were not related to the degree of liver test abnormalities. Liver and biliary tract dysfunction in sickle cell disease have been attributed to anoxia secondary to sinusoidal obstruction by sickled erythrocytes and Kupffer cell erythrophagocytosis. However, some causes of liver disease in sickle cell patients can be explained by clinical disorders other than the hemoglobinopathy alone.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver involvement in sickle cell disease. 403 12

Modern contraceptive methods are discussed, with special emphasis on oral contraceptives, which are regarded as the most effective. They are also regarded as generally safe, although there are contraindications and the drugs should only be prescribed after careful examination. The need for selecting the drug most suitable for the individual patients, mainly on the basis of the characteristics of the menstrual cycle (suggesting a predominance of estrogen or progestin, within safety limits, such as 50 mcg of estrogen), is emphasized. The examinations required include a general clinical, gynecological, and breast examination, cytology tests, evaluation of the menstrual flow pattern, measurements of arterial pressure, weight, glucose, cholesterol and triglyceride levels, and urine tests. They should be repeated at 6-month intervals, or 3-month intervals in the case of high-risk patients (varicose veins, obesity, heavy smokers, high cholesterol and triglyceride levels, history of jaundice, slight heart condition, clinical or potential diabetes, porphyria or predisposition to uterine myoma). Oral contraceptives are contraindicated in cases presenting a history of thromboembolism, phlebitis, cerebral apoplexy; sickle cell anemia, which indicates a predisposition to thromboembolic accidents; serious liver disease or recent hepatitis; serious heart disease; hormone-dependent neoplasia (breast cancer); predisposition to uterine cancer; erythematous lupus; metorrhagia of unknown origin; psychic disorders, especially of a depressive type. They should also be avoided for 3-4 years after puberty, in order to avoid interfering with the development of the hypothalamus and with growth. A carcinogenic effect of the pill and an increase in the risk of giving birth to abnormal children can be ruled out, although the incidence of abortions due to chromosome anomalies after suspending treatment is rather high (due to the previous inhibition of ovulation, a situation similar to repeated pregnancies at short intervals, which involve the same risk).
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PMID:[Current clinical problems of contraception]. 502 53

The prevalence of serum autoantibodies was determined in 50 black adult patients with sickle cell anemia, and in 33 age- and sex-matched healthy black individuals. Twenty-seven of the patients (54%) had at least one type of autoantibody, in contrast to only four (18.2%) of the control subjects (P less than .01). Antismooth muscle, antiskeletal muscle and antinuclear antibodies were the most frequently seen autoantibodies. There was no correlation between the presence of autoantibodies and history of hepatitis, number of blood transfusions or concurrent drug therapy. Sickle cell anemia is associated with the induction of autoimmune phenomena.
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PMID:Serum autoantibodies in patients with sickle cell anemia. 636 65

Maternal morbidity and perinatal outcome in 108 pregnancies complicated by sickle cell anemia (hemoglobin SS disease), sickle cell-hemoglobin C disease (hemoglobin SC disease), or sickle cell-beta-thalassemia disease were analyzed. Women given prophylactic red cell transfusions (1973-1982) during pregnancy were matched with historic controls whose care was almost identical except that blood was not given unless indicated (1955 to 1972). In women with hemoglobin SS disease who received prophylactic red cell transfusions, there was a sevenfold reduction in perinatal mortality and negligible maternal morbidity. In pregnancies complicated by hemoglobin SC disease during which transfusions were given, there were no perinatal losses, whereas there were in 18% of women not given transfusions. Maternal morbidity in women given transfusions was negligible; however, half of those not transfused experienced morbidity and, importantly, pulmonary complications were common. Transfusion-related complications included hepatitis and alloimmunization. From these experiences the authors conclude that prophylactic red cell transfusions reduce maternal morbidity and perinatal mortality appreciably, although perinatal morbidity is not eliminated. Transfusion therapy is justifiably started early in pregnancy for women with hemoglobin SS disease; however, transfusions may be withheld until the end of the second trimester for women with hemoglobin SC or sickle cell-beta-thalassemia disease.
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PMID:Pregnancy and sickle cell hemoglobinopathies: results with and without prophylactic transfusions. 688 18

The prevalence of persistent hepatitis-B surface (HBS) antigenaemia and hepatic functions have been determined in 125 children with sickle cell disease (SCD) as well as in 100 age-matched healthy children. Hepatic functions and the presence of HBS antigenaemia have been followed for 1 year in six children with SCD and 10 normal children following acute hepatitis-B infection. The prevalence of chronic HBS antigenaemia (3 per cent) in children with SCD is not higher than in normal children (11 per cent). The significant elevation of serum alanine transferase (ALT) and bilirubin concentrations in sickle cell children denotes a process of mild hepatocellular dysfunction which is unrelated to hepatitis-B viral antigenaemia. The high incidence of chronic HBS antigenaemia accompanied by elevated serum ALT and bilirubin concentrations in sickle cell children following acute hepatitis-B infection, in addition to the significant impairment of hepatic functions in sicklers with chronic HBS antigenaemia compared to those without the antigenaemia, point out to the high risk of continual parenchymal hepatic damage in these children following acute hepatitis-B infection. Vaccination against hepatitis-B virus should eliminate this risk.
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PMID:Study of hepatic functions and prevalence of hepatitis-B surface antigenaemia in Omani children with sickle cell disease. 763 38

Management of transfusion therapy in sickle cell disease patients with acute complications is often made difficult because of confusing indications, a variety of methods, disparate goals, and varying needs for maintenance transfusion. In priapism, acute chest syndrome, many major surgical procedures, toxemia of pregnancy, and cerebrovascular accidents, the target hemoglobin A level should be made as close to 100% as possible by mechanized red blood cell exchange. If mechanized exchange is unavailable, manual exchange should be instituted. Hemoglobin A should be maintained at greater than 60% to 70% by periodic simple transfusion until patients are fully recovered. Stroke patients should undergo maintenance transfusions for at least 3 years and perhaps 5 to 12 years. Physicians and patients should be aware of the transfusion-related risks of hepatitis and HIV infection. Alloimmunization and iron overload should be minimized in patients requiring frequent transfusions and chelation therapy should be utilized for iron overload.
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PMID:Transfusion therapy in sickle cell disease patients: methods and acute indications. 812 Apr 39

Population statistics of Amsterdam between the 17th and 19th centuries indicate that 20-30% of young married people had been born in foreign lands. At the present time, 6% of the country's population, nearly 1 million people, are direct descendants of foreign parents: 240,000 Surinamese, 210,000 Turks, 170,000 Moroccans, and 80,000 from the Antilles. 40% of foreigners live in the four large cities, and there they make up about 15% of the population; 30-50% of children in these cities have foreign born parents. Among health concerns affecting these people are parasitic diseases, tuberculosis, salmonellosis, and the importation of infections such as viral B hepatitis, which so far has been successfully controlled. About 4% of the foreigners (30,000 people) carry a defective gene, and when two such people marry, in 25% of cases a child can be born with a severe defect as well as thalassemia major (mainly children of Moroccans and Turks) and sickle cell anemia (Surinamese and Antillans). 20-40% of children from tropical or subtropical areas also have lactase enzyme deficiency, which gives them stomach complaints because of incomplete metabolism of milk sugar. In recent years it has been reported that asthma and respiratory infections with longer hospitalizations occur more frequently among foreign children. Infant mortality is also 2-3 times higher among them. Intercultural aspects affecting Turkish and Moroccans immigrants include communication problems, primarily those of the first generation, which should be facilitated by language centers and educational materials. Generation conflicts arise from contrasts between homelife and the outside world as well as from the fact that many of the parents are illiterate. Cultural difference are rooted in Islam, which requires loyalty to the group with traditional role patterns. Other problems pertain to the social isolation of the mother and the lower position of women, and the uncertain legal position of foreigners, which can result in sometimes unwarranted feelings of discrimination.
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PMID:[International and intercultural aspects of pediatrics and adolescent health care]. 812 29

In this report, we describe the case of a 28-yr-old woman with sickle cell anemia who presented with acute hepatic failure manifested by anorexia, malaise, painless jaundice, elevated aminotransferase activities, and severe coagulopathy. Liver biopsy revealed changes consistent with autoimmune hepatitis. Treatment with corticosteroids and azathioprine was followed by improvement in biochemical liver test results. The literature on sickle cell-associated liver diseases is reviewed.
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PMID:Autoimmune hepatitis in a patient with sickle cell disease. 863 41

We report a 6-week-old boy with meperidine neurotoxicity. What distinguished our patient from those previously reported was his minimal exposure to therapeutic doses of meperidine in the setting of normal renal function, and no history of sickle cell anemia, cancer, hepatitis, or cirrhosis. In addition, our patient had no abnormal changes in the electroencephalogram during the event. After only 2 doses of meperidine, he exhibited acute orofacial dyskinesias consisting of tongue thrusting, lip pursing, and facial grimacing combined with prominent flexion of the arms and stiffening of his legs. However, a normal sucking response remained. His symptoms resolved over the next 36 hours and did not respond to naloxone. We believe that this unique presentation of meperidine-induced neurotoxicity may be due to changes in the basal ganglia resulting from perinatal hypoxemia.
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PMID:Acute neurotoxicity of meperidine in an infant. 896 93

Blood transfusion services for civilian populations were poorly established until the mid-1980s in most of sub-Saharan Africa and were unable to provide sufficient supplies of blood with acceptable safety. From 1985 onwards the HIV pandemic was recognized seroepidemiologically. About 10-15% of HIV transmission has been related to blood transfusion. The high-risk groups are children suffering from malaria and anemia, women with pregnancy-related hemorrhage or anemia, victims of trauma, and persons with sickle cell disease. However, hemophiliacs are not a major risk group in comparison. As a result of the HIV/AIDS pandemic, national and international attention has at last been focused on the inadequacies of blood transfusion services. Organizational structures have been developed. There have been concerted efforts to recruit voluntary unremunerated blood donors, chosen from population groups with low seroprevalence. Serological screening for HIV, hepatitis viruses, and syphilis has been introduced or strengthened. Standards for blood group serology, blood storage, and handling have been improved and guidelines for the proper use of blood have been developed and adopted. In addition, there have been many training and retraining programs. Much has yet to be completed, however, using national and international resources, before the blood supply achieves acceptable standards of safety and is adequate in both remote and central areas of Africa.
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PMID:HIV and blood transfusion in sub-Saharan Africa. 1017 81


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