Cyclosporine therapy for autoimmune hepatitis in children

The current immunosuppressive treatment of patients with autoimmune hepatitis [AIH] consists of prednisolone and azathioprine. Higli doses of prednisolone used for disease remission are not universally effective and have serious adverse effects. Some authors have provided evidence of AIH therapy in children and adults with cyclosporine [Neoral] should corticosteroid therapy become ineffective. Preliminary results using cyclosporine in a small group of patients have shown that this drug appears to be a good substitute for corticosteroids. We performed this study to assess the efficacy and safety of cyclosporine in induction of remission in children with AIH. This was a case series, interventional clinical trial that involved children with AIH. twelve children with a median age of 9 years, 3 months who were diagnosed according to international criteria as having definite AIH were recruited. Cyclosporine alone was administered at a dosage of 3.5-5 mg/kg in 3 daily doses for 5 months, followed by low dose prednisolone [0.3 mg/kg/d] for one month, then followed by combined low doses of prednisolone and azathioprine [1.5 mg/kg/d in two doses]. Patients discontinued cyclosporine after seven months. Biochemical remission of the disease was established by assessment of serum transaminase activity levels. Growth parameters that included Z-scores for height and weight, and adverse effects of the treatment were recorded, Of the 10 remaining patients, 7 had normalized alanine aminotransferase [ALT] activity levels by 4 months and all patients had normalized ALT levels by 9 months of treatment. Adverse effects of cyclosporine were mild and disappeared during weaning off the medication. Cyclosporine induced biochemical remission of the hepatic inflammatory/necrotic process in children with AIH. There were few, well-tolerated adverse effects. Longer follow-up of patients is necessary to establish possible long-term toxicity of cyclosporine

Triple-A syndrome [Allgrove syndrome] in Iranian children: report of 4 cases

Allgrove syndrome also known as triple-A syndrome is an autosomal recessive disorder characterized by alacremia, achalasia and ACTH-resistant adrenal insufficiency. Although this syndrome is rare, herein we report four cases with different clinical manifestations. They were referred to the gastrointestinal ward during a one year period with complaints of vomiting and dysphagia. The diagnosis of triple-A syndrome was confirmed after careful evaluations for vomiting

[Liver copper storage disease in children with cryptogenic chronic liver disease]

We aimed to determine the frequency of liver copper storage diseases in Iranian children with cryptogenic chronic liver disease [CLD]. One-hundred and twenty children [52 girls, 70 boys; age range: 3 months-14 years] with CLD who attended Children's Medical Center in Tehran, Iran during the years 1997 through 1999, were enrolled. All viral, metabolic and autoimmune causes of CLD were excluded in them, all underwent slit lamp examination for KF ring by an experienced ophthalmologist, and liver biopsy done on all of them. In addition to the routine histopathologic examination, the liver biopsy specimen was checked for its copper content with proton-induced X-ray method [PIX-E]. Eight [6.6%, 4 girls, 4 boys, age: 6 to 14 years] of the 122 children with CLD had no identifiable cause despite extensive evaluation. All 8 had cirrhosis on liver biopsy and elevated hepatic copper content [mean 31 times normal]. None had KF rings and all had normal serum Ceruloplasmin levels. NICC was diagnosed for all by the absence of KF ring, normal serum ceruloplasmin levels and pathognomic histopathologic findings. Mean hepatic Cu level showed a 7-fold increase in children with CLD other than NICC. Mean serum ceruloplasmin level was 45 mg/dl in NICC and 31 mg/dl in CLD patients. Mean 24-hour urinary Cu excretion was 225 mg and 76 mg in NICC and CLD patients, respectively. All patients with NICC [n=8] had the history of using cooking utensils made of copper. Four of these were from one family. According to our data 6.6% of Iranian children with CLD who diagnosed as cryptogenic cirrhosis suffer from NICC, Therefore early diagnosis and appropriate treatment with D-Penicillamin are important for survival in patients with NICC. Hence it is suggested that all children with cryptogenic cirrhosis be assessed for NICC