Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the period of 1978 to 1986, 66 patients (31 men, 35 women) with a mean age of 28.4 years and various sickle cell hemoglobinopathies underwent 82 surgical procedures; 28 were emergencies. Fifty of the 66 patients had HbSS, 13/66 had HbSC, and 3/66 had HbS-thalassemia. All 66 patients received transfusions, although not for all procedures. In 48 patients, transfusion therapy was only administered preoperatively. Simple transfusions (1 to 10 units) were administered in 31 of 48 procedures. Exchange transfusions (1 to 6 units) were performed in nine of 48 procedures. Preoperative hematocrit ranged from 7.0% to 54.2%; of those receiving transfusions the hematocrit ranged from 22.6% to 53.7%. Intraoperative transfusions (1 to 10 units) were performed in 14 of 82 procedures; postoperative transfusions (1 to 6 units) were performed in 13 of 82 procedures. No advantage was noted in preoperative exchange transfusion as measured by a decrease in postoperative complications; a slight increase was seen in atelectasis in this group of patients with preoperative transfusions. An increase was reported in the complication rate of patients with an hematocrit of less than 30%. The type of transfusion (preoperative, intraoperative, or postoperative) administered did not appear to be related to postoperative morbidity rates. The complication rate for simple transfusions was 51.6% and for multiple transfusions, 55.6%. HbSS hemoglobinopathy had the higher complication rate. The hepatitis B surface antigen was demonstrated in four of 66 (6.1%) patients; ten of 66 (15.2%) developed alloantibodies. The benefits of transfusion therapy should be judged according to clinical needs; not all sickle cell patients need exchange or preoperative transfusion.
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PMID:Assessment of the use of transfusion therapy perioperatively in patients with sickle cell hemoglobinopathies. 335 66

Although 50% of Indochinese refugees are under 18 years of age, previous studies have emphasized the prevalence of parasites, anemia, tuberculosis, and hepatitis, with few addressing age-related health care needs. In this study the specific health care needs of 80 Indochinese refugee teenagers, evaluated during a 4-year period, were determined. The Centers for Disease Control's suggested screening measures were used, and it was found that 52% had positive purified protein derivative skin tests, 38% lacked immunizations, 35% had stool specimens positive for parasites (prevalence and number of parasites greatest among Cambodians), 14% had blood tests positive for hepatitis B surface antigen, and 10% were anemic. Additional evaluations showed that 19% had hemoglobinopathies, 14% were in or below the fifth percentile for height and weight, 12% had goiters, 12% had skin disorders, 8% had positive hepatitis B surface antigen, 5% had visual defects, 5% had hearing loss, 5% had psychosomatic illness, and 4% had idiopathic scoliosis. Although suggested Centers for Disease Control screening measures may be adequate for younger Indochinese children, these data suggest that additional studies are necessary for teenagers. For the sexually active adolescent, identification of and counseling for hepatitis antigenemia and hemoglobinopathies are crucial. In addition, early identification of emotional and physical problems during screening may enhance assimilation into a new society and facilitate completion of the psychosocial tasks of adolescence.
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PMID:Health care needs of Indochinese refugee teenagers. 379 58

Most pathologic studies of liver disease in sickle cell anemia and its variants were performed retrospectively on autopsy specimens, and, because of the prominent histologic features of intrasinusoidal sickling and Kupffer cell erythrophagocytosis, hepatic dysfunction was attributed to the intrahepatic sickling of erythrocytes in this hemoglobinopathy. We compared the liver histology from 19 patients who had liver biopsies to the autopsy specimens from 32 patients who succumbed to the complications of the hemoglobinopathy. In the former, nine patients had histological evidence of viral hepatitis. Four of these patients had both serological and immunohistochemical evidence of hepatitis B surface antigen. The features of biliary tree obstruction were found in two cases and alcoholic cirrhosis and sarcoid granuloma in one case each. Only one patient, who had recovered from septic shock, showed ischemic necrosis. In five patients incidentally biopsied during cholecystectomy, no significant lesions were found. Fourteen of the autopsy specimens showed ischemic necrosis, a result which was significantly different from the biopsy group. Ten cases had no significant morphologic changes other than heavy iron deposits. There were two cases with chronic active hepatitis, two with diffuse fibrosis, and one case each of cirrhosis, acute viral hepatitis, cholestasis, and giant cell hepatitis. Intrahepatic sickling and erythrophagocytosis were seen in almost all specimens and did not correlate with liver disease or transaminase elevation. Other than the patient with septic shock, ischemic necrosis was found only in postmortem material. These histological features may represent red cell destruction rather than the etiology of liver disease in these patients.
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PMID:Pathological spectrum of liver diseases in sickle cell disease. 394 29