Autoimmune Liver Diseases in Children

Mataya, Leslie; Patel, Namrata; Azzam, Ruba

doi:10.3928/19382359-20181022-02

Cited by 8 publications

(8 citation statements)

References 29 publications

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“…We found the AIH type-1 (positive anti ANA) as a commonest cause of CLD, was diagnosed by combination of clinical picture, laboratory work up and typical liver histology in the absence of a particular cause. 5 The study conducted by Hanif et al 3 also showed that in their group of children of CLD, viral hepatitis B followed by Wilson disease were the commonest diseases. Another study performed in Pakistan revealed the hepatitis C and glycogen storage disease were the top two diseases in the list of their group of children.…”

Section: Discussionmentioning

confidence: 96%

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Seerat

Khan

Atique³

et al. 2021

Pak J Med Sci

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Background and Objectives: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. Methods: This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined. Results: Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing. Conclusion: Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality. doi: https://doi.org/10.12669/pjms.37.3.3862 How to cite this:Seerat I, Khan ED, Atique M, Aujla UI. Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study. Pak J Med Sci. 2021;37(3):---------. doi: https://doi.org/10.12669/pjms.37.3.3862 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Section: Discussionmentioning

confidence: 96%

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Seerat

Khan

Atique³

et al. 2021

Pak J Med Sci

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“…AIH is characterized biochemically by high level of transaminase, histologically by the presence of interface hepatitis, and serologically by a high level of immunoglobulin G (IgG) [1][2][3][4]. AIH is further subdivided into type I and type II, depending on the autoantibody profile: AIH type I is characte-rized by positivity for the anti-nuclear antibody (ANA) and/or anti-smooth muscle antibody (ASMA), and AIH type II is characterized by the presence of anti-liver kidney microsomal antibody type I (anti-LKM1) and/or anti-liver cytosol type 1 [5][6][7][8][9]. Overlap syndrome describes variant forms of AIH, which includes features of a combination of AIH, primary sclerosing cholangitis, and primary biliary cirrhosis.…”

Section: Introductionmentioning

confidence: 99%

“…procedure with risk of complications [3][4][5][6][7]. Limited studies discuss the role of shear-wave elastography, magnetic resonance (MR) elastography, and MR spectroscopy in the diagnosis and monitoring of patients with AIH, but these results are preliminary and overlapping [10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

“…interface hepatitis and rosette formation, and clinically ranging from asymptomatic to having liver failure. AIH is characterized by positive autoantibodies including ANA, ASMA, and anti-LKM1 and increased immunoglobulin G (IgG) [2][3][4][5][6][7].…”

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confidence: 99%

“…This may be due to the presence of interface hepatitis at the portal-parenchymal interface, with dense plasma cell-rich lymphoplasmacytic, infiltrates, hepatocellular regeneration with rosette formation, emperipolesis (active penetration by one cell into and through a larger cell), and hepatocyte swelling and/or pycnotic necrosis piecemeal necrosis with periportal/periseptal lymphocyte infiltrating the portal plasma cells. Fibrosis is present in all children with AIH apart from those with the mildest forms of the disease [2][3][4][5][6][7].…”

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confidence: 99%

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The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

et al. 2021

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Purpose:To evaluate the role of diffusion tensor imaging (DTI) of the liver in children with autoimmune hepatitis (AIH). Material and methods:A prospective study was done on 42 children with AIH (30 girls and 12 boys, with a mean age of 13 years) and 20 age-and sex-matched healthy control children. They underwent DTI of the liver and laboratory tests. Liver biopsy was done for the patients. The mean diffusivity (MD) and fractional anisotropy (FA) of the liver were calculated and correlated with the pathological results. Results:The mean MD and FA of the liver in children with AIH were 1.42 ± 0.06 × 10 -3 mm²/s and 0.37 ± 0.11; and in the control children they were 1.55 ± 0.07 × 10 -3 mm²/s and 0.25 ± 0.03, respectively. The MD and FA were significantly different in the children with AIH compared to the control children (p = 0.001). The cutoff MD and FA used to differentiate patients from controls were 1.50 × 10 -3 mm²/s, 0.31 with AUC of 0.919 and 0.813, sensitivity of 97.6% and 66.7%, a specificity of 80% and 70%, an accuracy of 94.2% and 67.3%, PPV of 95.3 and 90.3, and NPV of 88.9 and 33.3, respectively. There was significantly lower MD and higher FA of the liver in children with AIH type I (n = 31) than type II (n = 11) (p = 0.001), and patients with (n = 9) and without (n = 33) overlap syndrome (p = 0.005). Conclusions:We concluded that DTI parameters can help to diagnose AIH, detect its phenotyping, and give clues as to the presence of associated overlap syndrome.

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