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Evaluation of the effectiveness of treatment with prednisone and azathioprine of autoimmune hepatitis in children.

Pniewska A, Sobolewska-Pilarczyk M, Pawłowska M - Prz Gastroenterol (2015)

Bottom Line: In 13/17 cases of exacerbations they were associated with a reduction in the dose of immunosuppressive drugs.Steroidside effects occurred in 14/15 patients.However, significant side effects of treatment indicate the need for further exploration of effective and safe therapy, especially in the paediatric population.

View Article: PubMed Central - PubMed

Affiliation: T. Browicz Provincial Infectious Diseases Hospital, Bydgoszcz, Poland.

ABSTRACT

Introduction: Autoimmune hepatitis is rarely diagnosed in children, but the course of the disease is often aggresive. Combination therapy with prednisone and azathioprine improves the prognosis of patients.

Aim: To evaluate the effectiveness of combination therapy with prednisone and azathioprine of autoimmune hepatitis (AIH) in children.

Material and methods: There was a retrospective analysis of the medical records of 15 patients with AIH, diagnosed before18 years of age, treated in the Provincial Infectious Diseases Hospital in Bydgoszcz in the years 2002 to 2013. We analysed the results of laboratory tests, ultrasound examination, endoscopy, and morphological liver pictures, as well as periods of exacerbation of inflammation and side effects of therapy.

Results: Biochemical remission of the disease was achieved on average after 36 days of treatment. Histopathological regression in the control liver biopsy was found in 7/15 patients and progression in 2/15 patients. In the study group 10/15 patients experienced exacerbation of the disease from 1 to 3 times during observation, with an increase of ALT activity to greater than 3 norm, and the remaining 5/15 patients had no increase of ALT activity. In total, 10 patients in the study group experienced 17 exacerbations. In 13/17 cases of exacerbations they were associated with a reduction in the dose of immunosuppressive drugs. There was no correlation between the biochemical exacerbation and changes in the histopathological image. Steroidside effects occurred in 14/15 patients.

Conclusions: The treatment allows for biochemical remission of the disease and significantly improves the prognosis of most patients. However, significant side effects of treatment indicate the need for further exploration of effective and safe therapy, especially in the paediatric population.

No MeSH data available.


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A, B. Liver biopsy results
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Figure 0001: A, B. Liver biopsy results

Mentions: Autoimmune hepatitis was diagnosed in 15 children treated in the Provincial Infectious Diseases Hospital in Bydgoszcz in the years 2002–2013. They were the only female patients, diagnosed at the age between 8 to 17 years (average age: 13 years and 9 months). The time of observation of patients ranged from 9 months to 11 years (mean follow up was 4 years and 8 months). In the cases of 5/15 patients the diagnosis was accidental (the reason for the diagnosis was the accidental discovery increased ALT activity, in the absence of clinical signs), in other cases the most common symptoms were weakness, drowsiness, headache, and abdominal pain. On physical examination yellowish discoloration of the skin and the sclera was found in 8/15 patients, hepatosplenomegaly in 7/15, peripheral oedema in 3/15, and ascites in 1 case. Laboratory abnormalities were found in all the girls (Table I). Positive autoantibody titres were found in 12/15 patients. SMA was found in 7/15 of the girls, ANA in 3/15, and anti-LKM1 in 7/15. Type 1 of the disease was diagnosed in 5/12 patients and type 2 in 3/12; 4/12 patients revealed the presence of antibodies specific to both 1 and 2 type of the disease. Infection with hepatitis A, B, and C virus, as well as cytomegalovirus, Epstein-Barr virus, and HIV were excluded in all patients. Ultrasound image of the liver was normal in 6/15 cases, non-specific lesions (hepatomegaly, increased echogenicity of the liver tissue) were found in 3/15, and features of advanced cirrhotic remodelling in 6/15. Features of portal hypertension in endoscopy or ultrasound Doppler were found in 5/15 patients. Oesophageal varices stage 1 without any signs of threatening of bleeding were described in endoscopy in 3 patients. In all patients a liver biopsy was performed and inflammatory activity and fibrosis were assessed by a modified Scheuer's scale [6]. Inflammatory activity was estimated at stage 1 to 2 in 7/15 patients, and at stage 3 to 4 in the remaining 8/15. The advancement of fibrosis in 6/15 patients was assessed at 0–2 and in the remaining 9/15 at 3–4. In 4/15 patients liver cirrhosis was diagnosed (Figure 1).


Evaluation of the effectiveness of treatment with prednisone and azathioprine of autoimmune hepatitis in children.

Pniewska A, Sobolewska-Pilarczyk M, Pawłowska M - Prz Gastroenterol (2015)

A, B. Liver biopsy results
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4814537&req=5

Figure 0001: A, B. Liver biopsy results
Mentions: Autoimmune hepatitis was diagnosed in 15 children treated in the Provincial Infectious Diseases Hospital in Bydgoszcz in the years 2002–2013. They were the only female patients, diagnosed at the age between 8 to 17 years (average age: 13 years and 9 months). The time of observation of patients ranged from 9 months to 11 years (mean follow up was 4 years and 8 months). In the cases of 5/15 patients the diagnosis was accidental (the reason for the diagnosis was the accidental discovery increased ALT activity, in the absence of clinical signs), in other cases the most common symptoms were weakness, drowsiness, headache, and abdominal pain. On physical examination yellowish discoloration of the skin and the sclera was found in 8/15 patients, hepatosplenomegaly in 7/15, peripheral oedema in 3/15, and ascites in 1 case. Laboratory abnormalities were found in all the girls (Table I). Positive autoantibody titres were found in 12/15 patients. SMA was found in 7/15 of the girls, ANA in 3/15, and anti-LKM1 in 7/15. Type 1 of the disease was diagnosed in 5/12 patients and type 2 in 3/12; 4/12 patients revealed the presence of antibodies specific to both 1 and 2 type of the disease. Infection with hepatitis A, B, and C virus, as well as cytomegalovirus, Epstein-Barr virus, and HIV were excluded in all patients. Ultrasound image of the liver was normal in 6/15 cases, non-specific lesions (hepatomegaly, increased echogenicity of the liver tissue) were found in 3/15, and features of advanced cirrhotic remodelling in 6/15. Features of portal hypertension in endoscopy or ultrasound Doppler were found in 5/15 patients. Oesophageal varices stage 1 without any signs of threatening of bleeding were described in endoscopy in 3 patients. In all patients a liver biopsy was performed and inflammatory activity and fibrosis were assessed by a modified Scheuer's scale [6]. Inflammatory activity was estimated at stage 1 to 2 in 7/15 patients, and at stage 3 to 4 in the remaining 8/15. The advancement of fibrosis in 6/15 patients was assessed at 0–2 and in the remaining 9/15 at 3–4. In 4/15 patients liver cirrhosis was diagnosed (Figure 1).

Bottom Line: In 13/17 cases of exacerbations they were associated with a reduction in the dose of immunosuppressive drugs.Steroidside effects occurred in 14/15 patients.However, significant side effects of treatment indicate the need for further exploration of effective and safe therapy, especially in the paediatric population.

View Article: PubMed Central - PubMed

Affiliation: T. Browicz Provincial Infectious Diseases Hospital, Bydgoszcz, Poland.

ABSTRACT

Introduction: Autoimmune hepatitis is rarely diagnosed in children, but the course of the disease is often aggresive. Combination therapy with prednisone and azathioprine improves the prognosis of patients.

Aim: To evaluate the effectiveness of combination therapy with prednisone and azathioprine of autoimmune hepatitis (AIH) in children.

Material and methods: There was a retrospective analysis of the medical records of 15 patients with AIH, diagnosed before18 years of age, treated in the Provincial Infectious Diseases Hospital in Bydgoszcz in the years 2002 to 2013. We analysed the results of laboratory tests, ultrasound examination, endoscopy, and morphological liver pictures, as well as periods of exacerbation of inflammation and side effects of therapy.

Results: Biochemical remission of the disease was achieved on average after 36 days of treatment. Histopathological regression in the control liver biopsy was found in 7/15 patients and progression in 2/15 patients. In the study group 10/15 patients experienced exacerbation of the disease from 1 to 3 times during observation, with an increase of ALT activity to greater than 3 norm, and the remaining 5/15 patients had no increase of ALT activity. In total, 10 patients in the study group experienced 17 exacerbations. In 13/17 cases of exacerbations they were associated with a reduction in the dose of immunosuppressive drugs. There was no correlation between the biochemical exacerbation and changes in the histopathological image. Steroidside effects occurred in 14/15 patients.

Conclusions: The treatment allows for biochemical remission of the disease and significantly improves the prognosis of most patients. However, significant side effects of treatment indicate the need for further exploration of effective and safe therapy, especially in the paediatric population.

No MeSH data available.


Related in: MedlinePlus