Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

We evaluated the contribution of hepatitis C virus infection to liver disease in patients with sickle cell anemia. Antibody to hepatitis C virus (anti-HCV) by commercial enzyme immunoassay and a second confirmatory assay were assayed in 121 consecutive patients with sickle cell anemia. Anti-HCV was detected in 25 of 121 patients (20.7%). Of patients transfused > 10 units of blood products, 30.3% were anti-HCV seropositive, whereas 8.6% of those patients who transfused < 10 units were seropositive. In 11 of the 121 patients, serum alanine aminotransferase levels were repeatedly elevated. Nine of these 11 patients were anti-HCV seropositive, one was positive for hepatitis B surface antigen, and one was negative for all viral markers. In contrast, of 110 patients with normal serum alanine aminotransferase levels, only 14% were anti-HCV seropositive. In patients with sickle cell anemia, exposure to hepatitis C is common, related to the number of previous transfusions, and the most likely cause of persistently elevated aminotransferase levels.
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PMID:Hepatitis C in sickle cell anemia. 803 15

In order for hepatitis B immunization programmes to be cost effective and clinically beneficial, vaccinated persons should maintain an immunity threshold titre of antibodies to hepatitis B surface antigen greater than 10 IU/l. Those who fall below this level should be boosted in order to be covered against the risk for which the vaccine was administered. Persons with sickle cell disease are included in the group for whom hepatitis B immunization is routinely prescribed. Antibody to hepatitis B surface antigen was measured in paired sera of thirty patients with sickle cell disease compared with a control group of healthy medical staff, five years post vaccination. There was no significant difference between patients with sickle cell disease and normal controls in the levels of antibody maintained or numbers that required booster vaccination. Recommendations for the maintenance of protection via revaccination should be the same for persons with sickle cell disease as for healthy persons.
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PMID:Immunization status of thirty patients with sickle cell disease five years post hepatitis B vaccination. A comparison with normal controls. 1199 25

The archives of the blood bank of the hospital of Dr. Louis Manual Morillo King, in the city of La Vega, Dominican Republic, were reviewed to identify all children who had been given blood transfusion during the period of July 1983 to July 1987 in order to identify HIV and the surface antigen of hepatitis B (HBsAg). Those who were released were visited in their homes for administration of HIV and hepatitis tests. Positive tests were confirmed by another test (AUSYME MONOCLONAL and Western Blot). Mothers were also tested to detect vertical transmission. 256 patients had been transfused, of whom 61 died. 80 of the 195 remaining patients could not be located. Of the 115 patients located, 52 had died in their homes after release from the hospital. Thus, the sample comprised 63 patients: 36 were 0-3 years old, 21 were 4-7 years old, and 6 were 8-11 years old. 50 lived in rural and 13 in urban areas. 56 patients had one transfusion and 4 had two transfusions. 28 patients had transfusion for anemia, 19 for malnutrition, 7 for sepsis, 6 for various reasons (meningitis, pleuritis, pneumonia), and 3 for sickle cell disease. 47 patients had been transfused at the hospital using the blood bank, 13 used blood from relatives, and 3 received blood from friends. Out of the 63 samples processed, 2 patients presented seropositivity for hepatitis B, while none were seropositive for HIV. Among the 2 patients who were seropositive for hepatitis B, the mother of one of them was also seropositive.
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PMID:[Human immunodeficiency virus and hepatitis B virus in children transfused in the Dr. Luis Manuel Morillo King Hospital]. 1234 60

Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH), Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty-six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18) were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East). Their mean age at presentation was 4.3 +/- 3.0 years (range 1-12 years). The mean serum albumin at presentation was 15.6 +/- 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 +/- 45.6 micromol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA). The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS) in 39% (n=14), IgM nephro-pathy in 28% (n=10), mesengioproliferative glomerulonephritis (MesPGN) in 17% (n=6), mini-mal change disease (MCD) and C1q nephropathy (C1qNP) in 8% each (n=3 + 3) and IgA nephro-pathy in 3% (n=1). Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some children.
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PMID:Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: a single center study. 1973 91