Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long term outcome

Concurrent extrahepatic autoimmune disease (CEHAID) associated with autoimmune hepatitis (AIH) have been incorporated into the diagnostic criteria stipulated by the International Autoimmune Hepatitis Group (IAIHG). Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty AIM: To systematically assess features and clinical impact of CEHAID on AIH METHODS: Clinical records of 562 patients with AIH from two tertiary centres in the United Kingdom were retrospectively reviewed RESULTS: Prevalence of CEHAID in patients with AIH were 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH-2 than AIH-1 (21.9% vs.7%, p=0.009). Personal history of CEHAID was more commonly found in AIH patients with than without first degree family history of CEHAID [(48/86, 55.8% vs 169/446, 37.9%), p=0.002]. AIH patients with CEHAID were more often female [201/236 (85.2%), p=0.008], had higher post-treatment IAIHG score (22 vs. 20, p<0.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, p<0.001), more likely to have mild fibrosis at diagnosis (20.9% vs. 6.5%, p<0.001), less often had ascites (6.3% vs. 13.6%, p=0.008) and coagulopathy (1.18 vs. 1.27, p=0.013) at presentation. Presence of CEHAID, however, did not significantly affect disease progression, prognosis and survival in AIH CONCLUSIONS: Our study confirms the strong association of CEHAID with AIH. Association between personal and familial extrahepatic autoimmunity especially among first degree relatives was evident. Presence of CEHAID may influence clinical phenotype of AIH at presentation but without notable impact on the long term clinical outcome

Human Leukocyte Antigen Profile Predicts Severity of Autoimmune Liver Disease in Children of European Ancestry

Background and Aims Genetic predisposition to autoimmune hepatitis (AIH) in adults is associated with possession of human leukocyte antigen (HLA) class I (A*01, B*08) and class II (DRB1*03, ‐04, ‐07, or ‐13) alleles, depending on geographic region. Juvenile autoimmune liver disease (AILD) comprises AIH‐1, AIH‐2, and autoimmune sclerosing cholangitis (ASC), which are phenotypically different from their adult counterparts. We aimed to define the relationship between HLA profile and disease course, severity, and outcome in juvenile AILD. Approach and Results We studied 236 children of European ancestry (152 female [64%], median age 11.15 years, range 0.8‐17), including 100 with AIH‐1, 59 with AIH‐2, and 77 with ASC. The follow‐up period was from 1977 to June 2019 (median 14.5 years). Class I and II HLA genotyping was performed using PCR/sequence‐specific primers. HLA B*08, ‐DRB1*03, and the A1‐B8‐DR3 haplotype impart predisposition to all three forms of AILD. Homozygosity for DRB1*03 represented the strongest risk factor (8.8). HLA DRB1*04, which independently confers susceptibility to AIH in adults, was infrequent in AIH‐1 and ASC, suggesting protection; and DRB1*15 (DR15) was protective against all forms of AILD. Distinct HLA class II alleles predispose to the different subgroups of juvenile AILD: DRB1*03 to AIH‐1, DRB1*13 to ASC, and DRB1*07 to AIH‐2. Possession of homozygous DRB1*03 or of DRB1*13 is associated with fibrosis at disease onset, and possession of these two genes in addition to DRB1*07 is associated with a more severe disease in all three subgroups. Conclusions Unique HLA profiles are seen in each subgroup of juvenile AILD. HLA genotype might be useful in predicting responsiveness to immunosuppressive treatment and course

Eosinophilic gastroenteritis: Clinical profiles and treatment outcomes, a retrospective study of 18 adult patients in a Singapore Tertiary Hospital

Results: Eighteen patients with EG were identified (mean age 52 years; male/female: 11/7). Fifteen patients (83%) had peripheral blood eosinophilia. Seven patients (39%) had atopic conditions. Most common symptoms were diarrhoea and abdominal pain. Small intestine was the most common site involved. Endoscopic finding was non-specific. Ten patients were treated with corticosteroids (nine prednisolone, one budesonide): eight patients (89%) responded clinically to prednisolone but four patients (50%) relapsed following tapering-off of prednisolone and required maintenance dose. One patient each responded to diet elimination and montelukast respectively. Half of the remaining six patients who were treated with proton-pump inhibitors, antispasmodic or antidiarrheal agents still remained symptomatic. Conclusion: Prednisolone is an effective treatment though relapses are common. Small intestine is most commonly involved. EG should be considered in the evaluation of unexplained chronic recurrent GI symptoms

Evidence of viral replication in HBsAg positive patients with hepatocellular carcinoma: measurement of serum hepatitis B virus deoxyribonucleic acid (HBV-DNA).

Annals of the Academy of Medicine Singapore1818-1

HLA association in Singapore children with Grave's disease

Metabolism: Clinical and Experimental376518-519META

Lung water detection using acoustic techniques

The presence of an excessive amount of water in lung is a sign of pulmonary edema which can be caused by heart failure. The current solutions for lung water detection involve the use of X-ray, CT scan or serum biomarkers, which require bulky and expensive instruments as well as long measurement duration. This paper reports on a study conducted on the use of a different sensing modality to detect the presence of water in lung. The main contributions of the paper are twofold: 1) we propose to employ acoustic (or sound) based techniques for lung water detection. The design is simple and can be implemented on a portable or wearable system; 2) we establish the feasibility of sound-based techniques for lung water detection, by carrying out experimental studies using four feature extraction methods combined with two classification methods. The findings of this study will be beneficial to the design of portable devices for rapid and accurate lung water detection