RESUMO
BACKGROUND: Data on the efficacy of particular therapeutic protocols of interferon-alpha (IFN-alpha) treatment for chronic hepatitis C in patients on hemodialysis (HD) vary. AIM: To compare the efficacy of two different therapeutic protocols for HD patients. PATIENTS AND METHODS: 15 hepatitis C virus (HCV)-positive patients on chronic HD at two dialysis centers: 8 patients treated with IFN-alpha 3 x 3 MU/week s.c. for 6 months (group A), and 7 patients treated with IFN-alpha 3 x 5 MU/week for 3 months, then 1 x 5 MU/week for another 3 months (group B). End of treatment response (ETR) and sustained virologic response (SVR) were evaluated by HCV-RNA determination. There was no statistically significant difference between the two patient groups according to age, sex, duration of HD and HCV infection. RESULTS: ETR was 87.5% (7/8) in group A and 28.5% (2/7) in group B, being statistically significant (p < 0.05). Although better SVR [50% (4/8) vs. 28.5% (2/7)] and lower drop-out rate [0% (0/8) vs. 28.5% (2/7)] were achieved in group A compared to group B, these differences did not reach statistical significance (p > 0.05). CONCLUSION: Therapy with IFN-alpha 3 x 3 MU/week s.c. for 6 months seems to be more appropriate for treatment of hepatitis C in HD patients, mostly due to better tolerability, i.e. lower drop-out rate. These differences could be attributed to different pharmacokinetic properties of the particular therapy protocol.
Assuntos
Antivirais/administração & dosagem , Hepatite C Crônica/tratamento farmacológico , Interferon-alfa/administração & dosagem , Diálise Renal , Adolescente , Adulto , Protocolos Clínicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
INTRODUCTION: Fabry's disease is a recessive X-linked disorder that results from a deficiency of the lysosomal hydrolase a-galactosidase A (alpha-Gal A). The absence of alpha-Gal A enzyme activity leads to accumulation of glycosphingolipid globotryaosyl ceramide (GL-3) in the lysosomes of a variety of cell types. Subsequently, angiokeratoma and ocular signs develop until, in most cases after the third decade of life, severe renal dysfunction or cardiomyopathy becomes obvious. Corneal opacities (cornea verticillata) occur in 90% conjunctival vascular changes in 60%, retinal vessel tortuosity in 55%, and cataracts in 50% of cases. Recently, enzyme replacement therapy has been shown to be an effective treatment modality that can eliminate glycolipid stores and reverse the disease pathology. CASE REPORT: A male patient born in 1971 was admitted for clinical examination due to proteinuria and erythrocyturia. During further evaluation nephrologist suspected Fabry's disease, because patient had skin changes early referred as petechiae, and acroparesthesias. He had also low heat tolerance and virtually no sweat. Physical status: angiokeratoma on gluteat regions and upper arms. Urine analysis in several occasions 10-15 E in sediment, alb. positive. On ECG, 2-mm depression of ST in precordial region. Heart ultrasound: low mitral regurgitation angio stage 1, left ventricle hypertrophy. Abdomen ultrasound: both kidneys around 12 cm large, parapyelic cysts in both kidneys of 2.5 cm in diameter. Biomicroscopy of both eyes: cornea verticillata. Fundus of both eyes: papillae n. optici with poorly defined edges, but without prominence, very tortuotic retinal blood vessels. In March 2002, very low alpha GAL enzyme activity in blood (alpha-Gal A = 0.5 +/- 0.2 nmol of substrate hydrolyzed hourly per serum mL--normal serum enzyme level 8.5-18.9 nmol/mL/h). In May 2002, enzyme replacement therapy was started with recombinant alpha-Gal A enzyme (Fabrazyme) 1 mg/kg every 14 days. Control evaluation and examination showed good cardiac and renal function. The patient felt better and stronger with improved heat tolerance. CONCLUSION: Fabry's disease occurs in all ethnic groups. It is estimated that one in 200 people is a carrier, and one in 40,000-100,000 has the disease. Today in Croatia, Fabry's disease has been diagnosed in only one patient, and according to the usual prevalence there are still 45-100 unrecognized patients. The ophthalmologists are in excellent position to diagnose Fabry's disease in early stages. Therefore it is very important that the ophthalmologists in Croatia become aware of the importance of ocular findings in Fabry's disease, so they can participate in the identification of unrecognized patients.
Assuntos
Oftalmopatias/complicações , Doença de Fabry/complicações , Adulto , Doenças da Córnea/complicações , Doenças da Córnea/diagnóstico , Oftalmopatias/diagnóstico , Doença de Fabry/diagnóstico , Doença de Fabry/genética , Humanos , Masculino , LinhagemRESUMO
AIM, PATIENTS AND METHODS: The high prevalence of anti-hepatitis C virus antibodies (anti-HCV) in hemodialyzed (HD) patients has been recognized since the early 1990s. Over the last decade, a significant decrease of anti-HCV prevalence among HD patients has been observed in many west European countries. In order to evaluate whether this trend is also present in Dialysis Center of Dubrava University Hospital, Zagreb, we tested HD patients for anti-HCV and HCV RNA in serum. ELISA 3 (Sorin) was used as a screening anti-HCV test, and confirmatory testing relied on western blotting (BioRad). HCV RNA was tested by HCV RNA PCR (Roche) and AMPLICOR, HCV test, version 2.0 (Roche). RESULTS AND DISCUSSION: The low prevalence of HCV infection is a consequence of the screening of blood donors with increasingly sensitive anti-HCV tests, followed by the progressive reduction of blood transfusion due to the availability of erythropoietin and the reinforcement of universral hygienic precautions and strict infection control in our HD unit. A contributing factor was the prevention of nosocomial transmission by the separation of anti-HCV positive from anti-HCV negative patients. Thus, the low prevalence of HCV infection in our HD center contributes-to an improved prognosis in end stage renal disease patients by additionally reducing the risk of nosocomial HCV infection.