Autoimmune hepatitis in children: experiences in a tertiary center

Autoimmune hepatitis [AIH] is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH. The medical records of 87 children [56 girls and 31 boy] diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome. The mean age of the patients was 10.1 +/- 4.5 years [64.4% females]. The most common clinical findings were jaundice [70.1%], splenomegaly [67.8%], and hepatomegaly [51.7%]. Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively [36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases]. The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 [74.7%] patients. The complete response was seen in 52 [59.8%] patients and 24 [27.6%] patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients. AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice

Idiopathic nephrotic syndrome in Iranian children.

Of 231 children with nephrotic syndrome, 87% were steroid sensitive and 13% steroid resistant. Of patients with steroid sensitive nephrotic syndrome, 38.8% were non-relapsers, 34.8% frequent relapsers and 26.4% infrequent relapsers. Among those with steroid resistant nephrotic syndrome, 37.5% had focal segmental glomerulosclerosis with a high mortality rate of 44%

Idiopathic hypercalciuria in Iranian children

The aim of this study was to determine the prevalence of idiopathic hypercalciuria [IH] in school children in Ahvaz, a city with different ethnic groups located in the Southwest of Iran. In a descriptive cross sectional study from October to December 2006, we determined urinary calcium [UCa] and urinary creatinine [UCr] in the morning urine samples of 500 primary school children. The levels of 24-hour UCa and UCr were measured in these children. Level of 24-hour UCa exceeding 4 mg/kg/day was considered as hypercalciuria, and UCa/UCr ratio exceeding 0.21 [mg/kg] was considered abnormal. Children who had hypercalciuria with a normal concentration of serum Ca were categorized as idiopathic hypercalciuric. Of 500 children aged 6-12 years, 231 were males and 269 females. In the first screening, 64 [12.8%] children [45 males, 19 females] had an abnormal UCa/UCr ratio. But in the end only 15 had the criteria of IH, i.e. the prevalence of IH was 3% [1.8-4.8%, confidence interval of 95%]. The prevalence in females and males was 0.74% and 5.6%, respectively [P= 0.003]. Of these children 10 had hematuria [including 2 cases of gross hematuria], 8 children gave a history of recurrent abdominal pain, 5 children suffered from dysuria and 3 persons had a history of personal or familial urolithiasis The study showed that 3% of primary school children in Ahvaz had IH predisposing to short-term and long-term complications of the disease. IH was significantly more common in boys than in girls