Cases reported "Hemophilia A"

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1/54. Transient hypoplastic anemia caused by primary human parvovirus B19 infection in a previously untreated patient with hemophilia transfused with a plasma-derived, monoclonal antibody-purified factor viii concentrate.

    BACKGROUND: Modern plasma-derived clotting factor concentrates are produced using various virus-inactivation protocols and are assumed to be safer than they were previously with regard to the risk for transmitting viral infections such as human immunodeficiency virus, hepatitis b, and hepatitis c. The risks from viruses that are relatively resistant to the current inactivation procedures remain uncertain. PATIENT: A 7-year-old with mild hemophilia a who had not been previously infused with any blood products was treated with a plasma-derived, monoclonal antibody-purified factor viii concentrate to cover orthopedic surgery after traumatic fracture of his left arm. RESULTS: A typical primary human parvovirus (HPV)-B19 infection was observed associated with transient hypoplastic anemia. retrospective studies including serologic examination and polymerase chain reaction analysis confirmed that the HPV-B19 infection was transmitted by the factor viii concentrate. CONCLUSIONS: Clotting factor concentrates for the treatment of hemophilia retain a risk for HPV-B19 contamination. HPV-B19 viral infection might induce hypoplastic anemia in these patients, particularly during enhanced hemopoiesis after acute blood loss.
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ranking = 1
keywords = hepatitis
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2/54. Acquired factor viii inhibitor in a non-hemophilic patient with chronic hepatitis c viral infection.

    Production of coagulation factor viii inhibitor is rarely encountered in non-hemophilic patients. A 63-year-old Japanese male suffered from severe bleeding tendency caused by this inhibitor. Although he did not have malignancy or collagen disease, he had chronic hepatitis c virus (HCV) infection. Although HCV is known to induce production of various autoimmune antibodies, this may be the first report of a case with both acquired factor viii inhibitor and HCV infection.
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ranking = 2.5
keywords = hepatitis
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3/54. Management of liver failure in a haemophilic patient co-infected with human immunodeficiency and hepatitis c viruses.

    We present a case of liver failure in a haemophilic patient coinfected with transfusion acquired human immunodeficiency (hiv) and hepatitis c (HCV) viruses. The case illustrates the interaction of multiple viruses with accelerated progression to end stage liver disease and ultimately death. We report the impact on the patient management of two liver biopsies, which diagnosed an initial drug induced hepatitis and subsequently an atypical HCV related hepatitis.
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ranking = 3.5
keywords = hepatitis
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4/54. Orthotopic liver transplantation in a patient with severe haemophilia A and with advanced liver cirrhosis.

    A patient with severe haemophilia A underwent orthotopic liver transplantation because of changes correlated to end-stage liver cirrhosis due to hepatitis b, C and D infection. Replacement therapy was carried out for 4 days and the clinical course was uneventful. At the time of reporting the patient has a normal working life. FVIII plasma concentration is normal. The indirect hyperbilirubinaemia may be related to the Gilbert's anomaly of the donor.
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ranking = 0.5
keywords = hepatitis
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5/54. prostatectomy in hemophilia.

    The advent of factor viii concentrates has made it possible for true hemophiliac patients to survive to an age when prostatic obstruction is common. Formerly, operative procedures in these patients were limited to life-threatening situations but now prostatic operations should not be feared as long as adequate serum levels of factor viii can be achieved and maintained throughout the wound healing period. Allergic reactions, hepatitis and cost are significant factors to consider when the concentrates are administered but hemophiliac patients are able to undergo treatment without a significantly increased risk to their lives.
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ranking = 0.5
keywords = hepatitis
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6/54. Clinical relevance of serum cryoglobulins in hemophilic patients with hepatitis c virus infection.

    Twenty-four hemophilic patients positive for hepatitis c virus were evaluated for the presence of serum cryoglobulins and associated symptoms. Eight (33.3%) of them had serum cryoglobulins (type II mixed cryoglobulinemia in all cases). The average time lapse between infection and appearance of serum cryoglobulins was 14.4 years (range 7-22 years). Two (25%) out of the 8 patients presented symptoms: one had systemic vasculitis, the other mild purpura. Hemophiliacs are a particularly suitable model for studying cryoglobulinemia in patients with hepatitis c virus infection since the onset of the infection is known and there is a long-term follow-up period.
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ranking = 3
keywords = hepatitis
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7/54. Bell's palsy during interferon therapy for chronic hepatitis c infection in patients with haemorrhagic disorders.

    Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-alpha2b therapy for chronic hepatitis c infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-alpha2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis b or C infection, or interferon-alpha2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of safety in medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-alpha2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect.
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ranking = 3
keywords = hepatitis
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8/54. Interaction between nelfinavir and tacrolimus after orthoptic liver transplantation in a patient coinfected with hiv and hepatitis c virus (HCV).

    A 49-year old male patient with severe hemophilia a, coinfected with hiv and HCV, who underwent orthoptic liver transplantation because of hepatitis c cirrhosis is presented. We describe a strong interaction between nelfinavir and tacrolimus postoperatively, that caused a reduction of the dose of tacrolimus by a factor 70 compared with normal, to achieve therapeutic blood concentrations and to avoid toxic side effects. We suggest that nelfinavir inhibits the metabolism of tacrolimus because both compounds are well-known substrates for the cytochrome P450 isoenzyme CYP 3A4. The nelfinavir serum concentrations were not affected by the institution of tacrolimus. Although the interaction dramatically changed the tacrolimus dose-concentration relationship, the situation was manageable by frequent monitoring of blood concentrations of tacrolimus.
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ranking = 2.5
keywords = hepatitis
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9/54. immune tolerance in a haemophilia A patient with high inhibitor using locally prepared lyophilized cryoprecipitate.

    The immune tolerance in a 12-year-old haemophilia A patient was carried out at the faculty of medicine, Ramathibodi Hospital, Bangkok in 1998. His inhibitor titres ranged from 10 to 3450 Bethesda units (BU). He suffered from serious bleeding episodes requiring prolonged hospitalization and the disarticulation of the left knee joint. After obtaining informed consent, locally prepared lyophilized cryoprecipitate (LC), heat treated at 60 degrees C for 25 h, was given in a dose of 13 units kg-1 body weight of factor viii three times per week. His inhibitor was increased from 15 to 580 BU within the first 4 weeks of immune tolerance. Finally, it was decreased to 40 BU in the 36th week. The only adverse effect was seroconversion of anti-hepatitis c virus after receiving 108 bottles of LC for 36 weeks. In conclusion, the locally prepared LC was able to control the bleeding episodes in a haemophilia A patient with high inhibitor. To our knowledge, this is the first reported case in thailand.
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ranking = 0.5
keywords = hepatitis
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10/54. peritoneal dialysis is the therapy of choice for end-stage renal disease patients with hereditary clotting disorders.

    Chronic renal failure is an unusual complication of hereditary clotting disorders (HCDs), but this situation could change in the near future. The modality of dialysis for end-stage renal disease (ESRD) in patients with an HCD is a difficult choice. Hemodialysis (HD) may be considered, but intensive treatment with coagulation factors is required for vascular access execution and for each HD procedure. peritoneal dialysis (PD) has been infrequently proposed. However, PD requires coagulation replacement therapy only during peritoneal catheter placement. The aim of this paper is to describe our experience of three patients with ESRD and HCD, successfully treated with chronic PD in the medium term. Case 1 was a 58-year-old man with moderate hemophilia a, type 2 diabetes mellitus, and hepatitis c virus (HCV) infection. His ESRD was secondary to glomerulonephritis. A double-cuff peritoneal catheter was surgically placed with pre-emptive factor viii administration. He began treatment with continuous ambulatory peritoneal dialysis (CAPD). An inguinal hernia was repaired without complications. After eleven months of follow-up, no hemorrhage episodes have been observed and clinical outcome is optimal. Case 2 was a 46-year-old man with severe hemophilia A, type 2 diabetes mellitus, and HCV and human immunodeficiency virus (hiv) infections. He developed a diabetic nephropathy that required renal replacement therapy. A permanent silicone catheter was inserted in the left internal jugular vein, and the patient started HD treatment. Later on, PD therapy was proposed. A peritoneal catheter was implanted with simultaneous factor viii infusion. Minimal bleeding was observed at the subcutaneous tunnel over the following 48 hours. The patient started PD treatment without complications, and two months later, remaining asymptomatic, transferred to another center. Case 3 was a 41-year-old woman diagnosed with von Willebrand disease type 2A, HCV infection, and polycystic kidney disease, who presented with ESRD. An internal arteriovenous fistula was performed under coagulation factor cover. During a fistulography, and despite coagulation factor substitutive treatment, the patient showed an important hematoma. Afterwards, PD was considered. A peritoneal catheter was implanted under coagulation factor cover. The postoperative course was uncomplicated, and the patient started CAPD treatment. During follow up, she suffered two hemoperitoneum episodes that were resolved with cold dialysate. After nine months, she uneventfully continued on PD. In conclusion, PD is the therapy of choice for patients with hereditary clotting disorders and ESRD requiring dialysis. peritoneal dialysis therapy avoids many of the complications related to HD therapy.
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ranking = 0.5
keywords = hepatitis
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