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Published on: May 29, 2020
A case of severe recurrent hepatitis with common variable immunodeficiency.
Koji Fukushima1, Yoshiyuki Ueno, Hirokazu Kanegane
1Department of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan.
A 16-year-old boy with severe hepatitis was diagnosed with common variable immunodeficiency (CVID). Combination therapy with glycyrrhizin and ursodeoxycholic acid improved liver histology and normalized liver enzymes without severe infections.
Area of Science:
- Hepatology
- Immunology
- Pediatrics
Background:
- Severe hepatitis with unclear etiology presents a diagnostic challenge.
- Histological and clinical features initially suggested autoimmune hepatitis.
- The patient exhibited declining immunoglobulin levels and impaired B cell function.
Purpose of the Study:
- To investigate the etiology of severe hepatitis in a young patient.
- To evaluate the efficacy of a combination therapy for hepatitis in the context of immunodeficiency.
- To establish diagnostic and therapeutic guidelines for persistent hepatitis in patients with common variable immunodeficiency (CVID).
Main Methods:
- A 16-year-old boy with severe hepatitis received intravenous glycyrrhizin and oral ursodeoxycholic acid.
- Disease activity was monitored through liver histology and serum alanine aminotransferase levels.
- Immunoglobulin levels and B cell function were assessed to diagnose common variable immunodeficiency (CVID).
Main Results:
- Combination therapy led to normalization of serum alanine aminotransferase levels.
- The patient's liver histology improved significantly after 27 months.
- The diagnosis of common variable immunodeficiency (CVID) was established due to impaired immunoglobulin production.
- The patient tolerated the therapy well, avoiding severe infections and over-immunosuppression.
Conclusions:
- Persistent hepatitis in patients with common variable immunodeficiency (CVID) can be effectively managed with a combination of glycyrrhizin and ursodeoxycholic acid.
- This therapeutic approach improves liver histology and biochemical markers while preventing severe infections.
- Further research is needed to establish definitive diagnostic criteria and therapeutic strategies for hepatitis associated with CVID.
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