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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.
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PMID:Benign course of extreme hyperbilirubinemia in sickle cell anemia: analysis of six cases. 87 60

The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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PMID:Sickle cell anemia- clinical manifestations in 100 patients and review of the literature. 113 Apr 36

Maternal mortality is examined from June 1980 to December 1986 at Mulago, Nsambyo, Old Kampala, Rubaga, and Mengo Hospitals in Kampala, Uganda. Clinical or immediate causes, direct and indirect, were recorded from case summary forms based on ICD9 definitions of obstetric complications. The nonabortion maternal mortality rate (NAMMR) was 2.65/1000 deliveries (580 deaths); the abortion-related maternal mortality rate (ARMMR) was 3.58/1000 abortions. The hospital maternal mortality rate was 2.0/1000 deliveries. 75% of maternal deaths of women of 28 weeks' gestation or more had delivered outside the hospital. NAMMR doubled between 1980-86, a statistically significant increase. ARMMR increases were almost significant. 75% were direct obstetric and 21% were indirect obstetric causes. 38% had clinical anemia, 29% had some sepsis, 18% had substantial bleeding, and 14% had obstructed labor. Other contributing conditions were pneumonia, ruptured uterus, laparotomy, evacuations and curettage, malaria, preeclampsia, sickle cell anemia, pulmonary embolism, malnutrition, tetanus, meningitis, prolonged labor, and hepatitis. At admission, 48% were in poor condition, 30% in good condition, and 22% in fair condition. 27% had sickle cell anemia, high blood pressure, multiple pregnancy, or malaria at admission. 64% were admitted within 24 hours after delivery, 67% 1-7 days after delivery, and 92% 7-42 days after delivery. Those in good condition were all admitted 7 days postdelivery. 41% of deaths were due to lack of drugs, 7% lack of fluids, 20% with theater problems, 14% with doctor-related factors, and 3% with midwife-related factors. Better information is needed on mortality before delivery, mortality in hospitals vs. outside, and mortality from abortion, and ectopic and hydatidiform molar pregnancies. An explanation given for the increase in maternal mortality is the decline in economic conditions. Abortion complications may be due to the concealment practiced. Causes are consistent with trends from the 1950s, 1960s, and 1970s in Uganda and developing countries in general. Availability and accessibility of gynecological and obstetric services needs great improvement. Training traditional birth attendants and obtaining rural ambulance services are also needed. Health workers lack creativity and imagination for developing country conditions; scarce resources are not the only problem.
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PMID:Incidence and causes of maternal mortality in five Kampala hospitals, 1980-1986. 176 15

There has been significant decrease in maternal morbidity and mortality of sickle cell disease patients during pregnancy due to better understanding of the pathophysiology of the disease and physiologic changes during pregnancy. Prophylactic blood transfusion does not appear to reduce complications in patients with sickle cell anemia. Patients with sickle hemoglobin C disease and with S beta thalassemia+ have fewer complications but still need close monitoring. Blood transfusion therapy should be made available for medical and obstetrical complications to include increasing hypoxemia, progressive anemia, acute chest syndrome, twin pregnancy, splenic sequestration syndrome, preeclampsia, septicemia, or prior to general anesthesia and surgery. Blood transfusion therapy is associated with hepatitis, allergic reaction, alloimmunization, AIDS, and iron overload states. These aspects should be considered prior to using blood transfusion therapy. Excellent prenatal monitoring and aggressive intervention should be instituted when problems arise for the successful management of the pregnant patient with sickle cell disease. Prenatal diagnosis and cord blood screening should be made available for the infant. Appropriate pediatric referral and prophylactic penicillin is recommended for the infant with sickle cell disease.
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PMID:Management of sickle cell anemia and pregnancy. 181 45

Non-A, non-B hepatitis, recently renamed as hepatitis C virus (HCV), accounts for over 90% of hepatitis cases worldwide associated with blood transfusions. Application of a recombinant-based enzyme immunoassay for the detection of antibodies to HCV to a sample of 500 male Saudi blood donors and 260 healthy Saudi pregnant women indicated that HVC is endemic in the Saudi population. Anti-HCV was detected in 28 (5.6%) of the blood donors and 12 (4.6%) of the pregnant women, for an overall frequency of 5.3% in healthy Saudi adults who had never received blood transfusions. This rate is at least 5 times higher than that reported for the US and Western Europe. Also assessed was the HCV rate in subsamples of Saudis considered at risk of this infection. Here, anti-HCV was detected in 22 (78.6%) hemophiliacs, 26 (33.3%) patients with thalassemia and sickle cell disease, 17 (26.1%) hemodialysis patients with renal failure, and 35 (15.9%) individuals with a sexually transmitted disease. The prevalence of anti-HBc ranged from 28% in blood donors to 46% in hemophiliacs. The significantly higher prevalence of HCV in patients with sexually transmitted diseases than in blood donors suggests that this disease is transmitted through heterosexual contact as well as blood transfusions. Given the high baseline level of HCV infection in the Saudi population and the possibility of serious sequelae (e.g., chronic active hepatitis, cirrhosis, and hepatocellular carcinoma), routine anti-HCV screening of blood donations is urged.
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PMID:Hepatitis C virus antibodies in high-risk Saudi groups. 177 46

The aetiological agents of acute icteric hepatitis and fulminant hepatic failure were investigated in 47 children less than 12 years of age presenting at our hospital during the period January to December 1987. Hepatitis A virus was the aetiological agent in 72%, hepatitis B virus in 11%, cytomegalovirus in 2% and non A non B in 15%. These results confirm the endemicity of these viruses in the Saudi population and that they can be contracted in early life and could lead to severe hepatitis. Three of the patients who had hepatitis A virus infection developed fulminant hepatic failure and two of them died. Two of the patients who developed fulminant hepatic failure were also suffering from sickle cell disease. This raises the question of a possible predisposition to hepatic failure in sickle cell disease upon infection with hepatitis A virus.
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PMID:Aetiology of icteric hepatitis and fulminant hepatic failure in children and the possible predisposition to hepatic failure by sickle cell disease. 215 27

Medically compromised children and adults are increasingly likely to seek orthodontic care as improved medical management creates more long-term survivors. For the majority, treatment of orthodontic problems is feasible, but special precautions usually are required. These include medical consultation to establish the patient's prognosis, maintaining a current knowledge of drug therapy, and modifications in office procedures. Patients with a history of multiple transfusions should be presumed to be hepatitis carriers until proved not to be, and special precautions to protect office staff members and other patients should be taken. Decreased resistance to infection is a common complicating factor in medically compromised patients. Dentists must therefore avoid mucosal irritation and carefully monitor periodontal health. The practitioner should be alert to side effects of drug treatment such as xerostomia and depressed immune response, and be aware of the particular features of the underlying disease. Bleeding disorders, which can be managed by replacement of missing clotting factors, do not contraindicate orthognathic surgery. The major contraindication is poor anesthetic risk, which almost always is true for patients having sickle cell anemia because of poor blood oxygen saturation. Because orthodontic treatment can provide positive benefits, it should not be withheld solely because of the presence of a serious medical problem. With appropriate management, successful orthodontic treatment can be done for most patients.
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PMID:Orthodontic care for medically compromised patients: possibilities and limitations. 293 66

Differentiating acute cholestatic jaundice resulting from hepatic vasoocclusive crises and hepatitis in children with sickle cell disease can be difficult. Both conditions result in hyperbilirubinemia, mainly of the conjugated type, and in elevation of serum transaminases. Five children with sickle cell disease, acute severe cholestatic jaundice and negative serology for hepatitis A and B presented in good general condition, with modest elevation of serum transaminases, and had an early uneventful recovery. Five children with sickle cell disease and serologically proved hepatitis A infection were sicker, exhibited a similar elevation of bilirubin concentration with marked elevation of the serum transaminases and recovered more slowly. The clinical course and outcome of hepatitis A in children with sickle cell disease was similar to that of hepatitis A in normal children. Unlike early reports acute cholestatic jaundice in our patients with sickle cell disease, whether caused by hepatitis or by hepatic vasoocclusive crises, was found to be benign with an uneventful recovery.
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PMID:Acute cholestatic jaundice in children with sickle cell disease: hepatic crises or hepatitis? 318 39

The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.
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PMID:Splenic growth rates in cirrhotic and other splenomegalic diseases of childhood. 384 62

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


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