RESUMO
BACKGROUND AND METHODS: Elevated serum ferritin is a common clinical finding. The etiology of hyperferritinemia in the Asia-Pacific population is less clear due to a low prevalence of known HFE mutations such as C282Y and H63D, as well as an increased prevalence of viral hepatitis and hereditary anemia. A retrospective case review of 80 patients of Asian ethnicity referred to three subspecialists in tertiary care teaching hospitals between January 1997 and March 2005 for assessment of hyperferritinemia was performed. RESULTS: Only four patients (5%) had iron overload on liver biopsy or quantitative phlebotomy. Forty-nine patients (61%) had secondary causes for their hyperferritinemia, of which 26 had liver disease; 16 of those patients also had viral hepatitis. Thirteen patients fulfilled criteria for the insulin resistance syndrome. Other causes included hematological disorders (n=10), malignancy (n=2) and inflammatory arthritis (n=2). Twenty-seven cases (34%) of unexplained hyperferritinemia were found. Of a total of 22 patients who underwent liver biopsy, significant iron deposition was found in one patient. Fifteen patients underwent C282Y and H63D genotyping, with two cases of H63D heterozygosity. Fourteen patients had first-degree relatives with hyperferritinemia. Three families were identified with more than two members affected, which is suggestive of a possible hereditary hyperferritinemia syndrome. CONCLUSION: Secondary causes of elevated ferritin in the Asian population, particularly liver disease, are common, but primary iron overload syndromes appear to be rare. In a significant proportion of patients, the etiology remains unexplained. The genetic basis for hyperferritinemia in Asians is poorly defined and requires further study.
Assuntos
Povo Asiático/estatística & dados numéricos , Ferritinas/sangue , Sobrecarga de Ferro/etnologia , Sobrecarga de Ferro/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Colúmbia Britânica/epidemiologia , Feminino , Genótipo , Hospitais de Ensino , Humanos , Sobrecarga de Ferro/diagnóstico , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos RetrospectivosRESUMO
BACKGROUND: It has been previously reported that British Columbia's (BC's) First Nations (Aboriginal) community has an increased risk of autoimmune diseases, including rheumatological conditions (rheumatoid arthritis, systemic lupus) and primary biliary cirrhosis. The researchers hypothesized that this community may also be at increased risk for autoimmune hepatitis (AIH). METHODS: Independent, retrospective reviews of the databases of two separate tertiary/quaternary British Columbia university-affiliated health care institutions, the Adult Liver Transplant Program of the BC Transplant Society and the Division of Pediatric Gastroenterology, BC Children's Hospital (Vancouver, BC), were performed. All patients referred with a diagnosis of probable or definite AIH who identified themselves as being of First Nations descent from 1988 to 2004 were reviewed. The liver transplant database records all adult patients in the province referred for transplant assessment. The pediatric database records all children referred to the BC Children's Hospital. RESULTS: A total of 68 adult patients with a definite or probable diagnosis of AIH were referred to the liver transplant program. Twelve patients (17.6%) were Aboriginal, 11 of which were female. Similarly, a total of 30 children with probable or definite AIH were identified from the pediatric database. Six of these cases (20%) were identified in Aboriginal children. CONCLUSIONS: The findings suggest an increased prevalence of AIH among BC's First Nations community. A disproportionate First Nations representation was found on independent review of two databases. Future studies are needed to determine the true prevalence of AIH in this community, and to uncover the genetic predisposition and the environmental triggers explaining this phenomenon.
Assuntos
Hepatite Autoimune/etnologia , Hepatite Autoimune/epidemiologia , Indígenas Norte-Americanos , Adolescente , Adulto , Colúmbia Britânica/epidemiologia , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Indígenas Norte-Americanos/etnologia , Lactente , Recém-Nascido , Transplante de Fígado/etnologia , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Fatores de RiscoRESUMO
PURPOSE: To assess sustained viral response rate and adherence to standard interferon alpha-2b and ribavirin therapy in inmates with chronic hepatitis C (HCV) in Canadian penitentiaries in the Pacific region. METHODS: A retrospective chart review of all inmates with chronic HCV who were treated with standard interferon alpha-2b and ribavirin therapy between March 2001 and October 2002. RESULTS: A total of 90 male inmates were treated. The mean age at time of treatment was 40 years. There were 49 inmates with HCV genotype 1, 11 with HCV genotype 2 and 30 with HCV genotype 3. Eight inmates discontinued treatment because of intolerance to side effects. Nine inmates were stopped by the physician because of nonresponse at an average of 27 weeks. All inmates achieved at least 80% adherence of interferon and ribavirin therapy. The overall sustained virological response (SVR) was 55.9%. SVR was 31.6% for genotype 1, 100% for genotype 2 and 71.4% for genotype 3. CONCLUSION: There was excellent SVR and adherence to treatment with interferon and ribavirin. This experience highlights an important opportunity to treat a population with a high prevalence of HCV-positive persons who may otherwise not seek treatment.
Assuntos
Antivirais/uso terapêutico , Hepatite C Crônica/tratamento farmacológico , Interferon-alfa/uso terapêutico , Prisioneiros , Ribavirina/uso terapêutico , Adulto , Canadá , Quimioterapia Combinada , Feminino , Humanos , Interferon alfa-2 , Masculino , Proteínas Recombinantes , Estudos RetrospectivosRESUMO
Classically, a diagnosis of systemic lupus erythematosus (SLE) is dependent on renal, rheumatological, cutaneous and neurological target organ damage with supporting serological markers. A previously healthy 26-year-old Japanese woman whose only manifestation of otherwise occult SLE was severe abdominal pain is reported. A computed tomographic scan of the abdomen revealed thickened loops of small bowel, endoscopic findings were nonspecific and jejunal biopsy revealed a nonspecific enteritis. Laboratory studies revealed lymphopenia, hypocomplementemia, a positive antinuclear antibody, a weakly positive anti-Smith and a strongly positive anti-double stranded DNA. There was a prompt symptomatic recovery with immunosuppressive therapy. The authors' experiences, and a review of the literature suggest that a diagnosis of SLE should be considered in young Asian women who present with significant but clinically enigmatic gastrointestinal illness.