Nonprogressive course of non-A, non-B chronic hepatitis in multitransfused hemophiliacs

Eleven hemophiliacs with chronic liver disease were studied prospectively for 6 yr, with liver function tests and liver biopsies carried out at intervals of 3 yr. The second series of biopsies, compared with the first series, showed continuation of chronic persistent hepatitis in four patients, change to chronic lobular hepatitis in two, and spontaneous improvement of the disease in the four cases who had had chronic active hepatitis characterized by moderate piecemeal necrosis. One patient with active cirrhosis died of liver failure during the follow-up period. Study of the serum and intrahepatic markers for hepatitis B and delta viruses suggests that chronic liver disease is nonprogressive in hemophiliacs who have no intrahepatic viral marker.     

Silent infections with human immunodeficiency virus type 1 are highly unlikely in multitransfused seronegative hemophiliacs

We used the polymerase chain reaction (PCR) to determine the frequency of silent human immunodeficiency virus type 1 (HIV-1) infections in seronegative high-risk individuals with hemophilia who had been exposed to contaminated blood products more than 3 years previously. In a cross-sectional study of a cohort of 57 prospectively followed seronegative hemophiliacs who received multiple transfusions before 1986, HIV-1 proviral DNA was found transiently in only one patient. These data suggest that the rate of HIV infection among high-risk antibody negative individuals with hemophilia is very low to absent, in the range of 0% to 2%. These findings should provide considerable reassurance to seronegative persons with hemophilia and their sexual partners.     

Liver biopsies in adult hemophiliacs with hepatitis C: a United States center's experience

We report a case series of 13 adult hemophiliacs with serological evidence of hepatitis C who underwent percutaneous liver biopsies without major complications. We also briefly review the recent literature on safety of liver biopsies in this population, and conclude that these patients may be safely biopsied using appropriate precautions regardless of the severity of hemophilia.     

Liver dysfunction and parenteral nutritional therapies

Hepatobiliary dysfunction associated with the use of total parenteral nutrition is a commonly recognized phenomenon occurring in up to 90% of patients on long-term therapy. Reasons for these abnormalities, both supported by research as well as theoretical possibilities are explored. Practical guidelines considered useful in documenting, preventing and treating serious hepatic consequences of total parenteral nutrition are discussed. The role of combined liver and small bowel transplantation as treatment for select patients is also reviewed.     

Liver dysfunction in steady state sickle cell disease

The liver is one of the organs involved in the multiorgan failure that occurs in sickle cell disease, the pathophysiology of liver disease in this condition is complex because of the interrelated multifactorial causes. Liver dysfunction was assessed in both paediatric and adult sickle cell disease patients in the steady state. The transaminases and alkaline phosphatase were analysed by automation while coagulation studies were done manually. The mean (range) of Alanine transaminase (ALT), Aspartate transaminase (AST) and alkaline phosphatase (ALP) were 23.0 (2-77) IU, 48.5 (15-120) IU, 227.5 (37-1200) IU respectively. ALT and AST levels were less than 100 IU in over 95% of the patients. The gender or age of the patients did not significantly affect the level of these three enzymes. There was close association between the liver size and elevation of the liver enzymes except for alkaline phosphatase (ALT=.017, AST=.009, ALP=.056). Twenty-five percent of the patients had normal enzymes while 13% had derangement of the three enzymes, 19%, 50% and 74% had abnormal ALT, AST and ALP respectively. Only 22% and 5% had deranged PT and APTT respectively. In conclusion minimal elevation of the tramsaminases which is not gender or age dependent were observed in steady state sickle cell disease, higher levels of alkaline phosphatase may be due to associated vasoocclussive crises involving the bones rather than a pathology of the liver.     

Liver dysfunction in patients with acute pulmonary embolism

Aim: Although liver injury due to cardiac, chronic respiratory and circulatory failure has been reported, this has yet to be studied in patients with pulmonary embolism (PE). We investigated liver injury in patients with acute PE. Methods: We retrospectively reviewed 107 acute PE patients over a two‐year period. Patients were categorized as having: (1) severe (P_aO₂ < 45 mmHg), moderate (45 mmHg ≤ P_aO₂≤ 60 mmHg) or mild hypoxemia (60 mmHg < P_aO₂ < 80 mmHg) groups; (2) massive and non‐massive PE; (3) absence or presence of pre‐existing cardiopulmonary disease; and (4) absence or presence of right ventricle dysfunction. Serum levels of liver enzymes were compared between groups. Results: Transaminase levels were higher in severe hypoxemia patients compared mild hypoxemia patients (p=0.045 and p=0.036). Albumin and bilirubin levels were lower and higher, respectively, in patients with severe and moderate hypoxemia compared to mild hypoxemia patients (p < 0.05 and p < 0.01). There was a negative correlation between hepatic markers and P_aO₂ and %S_aO₂: r=–0.212, p=0.032 between AST and %S_aO₂; r=–0.243, p=0.013 and r=–0.241, p=0.014 between ALT and P_aO₂ and %S_aO₂; and r=–0.224, p=0.024 and r= –0.283, p=0.004 between direct bilirubin and P_aO₂ and %S_aO₂. AST and ALT levels were higher in massive PE than non‐massive PE patients (p=0.0001). Albumin levels were lower in patients with right ventricle dysfunction than in those without (p=0.02). One (0.9%) had a clinical picture of hypoxic hepatitis. Conclusion: Abnormal LFTs showed a mixed pattern in patients with acute PE.     

EEG topography in HIV-positive hemophiliacs

HIV-central nervous system manifestations, here the AIDS-Dementia complex, are at the moment of special interest owing to its unexpected development. With EEG-topography one can expect to gain more information about possible changes in frequency according to topographical location. We recorded 69 HIV-positive hemophiliacs (23 WR 2, 26 WR 3-5 and 6 patients) plus 50 HIV-seronegative controls with a 12 channel EEG-topography evaluation. During the critical examination we looked for the results of two parameters: a) alpha 1-3/alpha 4 and b) alpha 4-4/9. In comparison to the control group we found a statistical significance (p less than 0.05) for WR 2-6 in alpha 1-3/alpha 4 index. Concerning the alpha 1-4/9 index one can only recognize a statistical significance (p less than 0.05) for WR 3-6 patients in comparison to the controls.     

Platelet and granulocyte alloimmunisation in multitransfused Tunisian patients

We determined the frequency of post-transfusion alloimmunisation against platelet and granulocyte antigens in 51 Tunisian polytransfused patients with haematological diseases. Serum samples were analysed by a standard and an antiglobulin-augmented lymphocytotoxicity technique, a granulocyte agglutination test, a granulocyte immunofluorescence test, a platelet immunofluorescence test and the monoclonal antibody-specific immobilisation of platelet antigens assay. No granulocyte-specific antibodies were detected. HLA antibodies were found in 58.8% of patients. Platelet-specific antibodies were detected in four patients and were directed against human platelet antigen (HPA)-5b, HPA-1b and HPA-3a. The three patients with Glanzmann's thrombasthenia developed anti-GPIIb/IIIa antibodies. This study provides immunogenetic information that could improve the management of transfusion therapy in Tunisia.     

Hepatitis G virus in multitransfused thalassaemics from India

Hepatitis G virus (HGV)/GB virus-C (GBV-C) has been identified as a blood-borne agent with disputed pathogenicity. This virus belongs to the flaviviridae with a distant relationship to hepatitis C virus (HCV). Genetically divergent HGV isolates have been reported from different parts of the world. This study describes the prevalence of HGV in multitransfused thalassaemic children in India and genomic sequence variations in 11 HGV isolates from the same geographical location. Hepatitis G virus RNA was detected in 39.7% multitransfused thalassaemic children. The seroprevalence of hepatitis B virus (HBV) and HCV was 23.8% and 17.1%, respectively, and 11.4% had dual infection. The nucleotide sequence of a 166 bp HGV genomic segment from the putative capsid-envelope region (nucleotide; nt 578–743) from 11 Indian isolates was compared to the sequences available in the nucleotide databases. The isolates from India were 81.3–94.5% homologous to the isolates from other parts of the world. On phylogenetic analysis, it was observed that HGV isolates from India may belong to two genetically divergent types.