[Show abstract][Hide abstract] ABSTRACT: The classical human leukocyte antigen (HLA)-DRB1*03:01 and HLA-DRB1*04:01 alleles are established AIH risk alleles. To study the immune modifying effect of these alleles, we imputed the genotypes from genome-wide association data in 649 Dutch autoimmune hepatitis (AIH) type 1 patients. We therefore compared the international AIH group (IAIHG) diagnostic scores as well as the underlying clinical characteristics between patients positive and negative for these HLA alleles. Seventy-five per cent of the AIH patients were HLA-DRB1*03:01/HLA-DRB1*04:01 positive. HLA-DRB1*03:01/HLA-DRB1*04:01 positive patients had a higher median IAIHG score than HLA-DRB1*03:01/HLA-DRB1*04:01 negative patients (P<0.001). We did not observe associations between HLA alleles and alanine transaminase (ALT) levels (HLA-DRB1*03:01: P=0.2; HLA-DRB1*04:01; P=0.5), however HLA-DRB1*03:01 was independently associated with higher IgG levels (P=0.04). The HLA-DRB1*04:01 allele was independently associated with presentation at older age (P=0.03) and a female predominance (P=0.04). HLA-DRB1*03:01 positive patients were received immunosuppressive medication and liver transplantation. In conclusion, the HLA-DRB1*03:01 and HLA-DRB1*04:01 alleles are both independently associated with the aggregate diagnostic IAIHG score in type-1 AIH patients, but are not essential for AIH development. HLA-DRB1*03:01 is the strongest genetic modifier of disease severity in AIH.
Genes and Immunity 01/2015; DOI:10.1038/gene.2014.82 · 3.79 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background
Cyst infection is a severe complication of hepatic cystic disease. However, an evidence-based treatment strategy is not available.AimTo assess the available treatment strategies and provide a treatment advice for de novo hepatic cyst infection.Methods
We systematically searched PubMed (1948–2014), EMBASE (1974–2014), and the Cochrane Library (until 2014) for studies involving humans (≥18 years) treated for a hepatic cyst infection. We extracted data on patient characteristics, treatment and follow-up.ResultsWe identified 41 articles; all were case series or case reports, implicating a high risk of bias. We included 54 hepatic cyst infection cases (male 39%; mean age 63 ± 12 years; diabetes 6%; dialysis 19%; transplant recipients 30%). Initial therapy consisted of antimicrobial (56%), percutaneous (31%) or surgical treatment (13%). We identified 42 antimicrobial regimens consisting of 23 different combinations. Most used antibiotic classes were quinolones (34%) and cephalosporins (34%). Antimicrobials failed in 70% of cases, eventually requiring percutaneous or surgical treatment in, respectively, 37% and 27%. Recurrent hepatic cyst infection was frequent (20%). Median time to recurrence was 8 weeks (IQR 3–24 weeks). In 46%, recurrence occurred in renal transplant recipients. Cyst infection related deaths occurred in 9%, of whom 40% were on dialysis.Conclusions
The literature shows that treatment of hepatic cyst infection is highly heterogeneous. We recommend first line treatment with oral ciprofloxacin. In case of failure, percutaneous cyst drainage needs to be considered.
[Show abstract][Hide abstract] ABSTRACT: Mass-related symptoms caused by hepatic cysts are effectively treated by aspiration sclerotherapy (AS). This minimal-invasive intervention is regarded as a safe procedure. Hence, occurrence of complications is low. Transient fever is commonly reported as a side effect. However, documentation on a post-procedural hepatic cyst infection as a complication of AS is limited. We present five cases in which a tentative diagnosis of post-procedural hepatic cyst infection was made. Patients typically presented with abdominal pain and fever, had to be admitted to our hospital, and were treated with long term antibiotics. Ultimately, the cyst infection successfully resolved with ciprofloxacin in all cases.
Journal of gastrointestinal and liver diseases: JGLD 12/2014; 23(4):441-4. · 1.85 Impact Factor