Blind percutaneous liver biopsy in infants and children: comparison of safety and efficacy of percussion technique and ultrasound assisted technique

Background and study aims: Liver biopsy remains the most reliable method to diagnose various hepatic disorders in children. We aimed to assess the technical success and complication rate of ultrasound [US] assisted percutaneous liver biopsy versus transthoracic percussion guided technique in paediatrics

Patients and methods: This randomized controlled study included all cases performing liver biopsy at Paediatric Hepatology Unit, Cairo University Paediatric Hospital over 12 months

Results: Patients were 102 cases; 62 were males, with age range 18 days to 12 years. Fifty seven procedures were done using the percussion guided technique and 45 cases were US assisted. The total number of complicated biopsies was 14 [13.7%], with more serious complications occurring in the percussion group. Complications were more frequent with younger age, lower platelet count, number of passes and occurrence of hypotension

Conclusion: US assisted percutaneous liver biopsy, although more costly, but may be safer to perform particularly in younger age

Glycogen storage disease type III in Egyptian children: a single centre clinico-laboratory study

Glycogen storage disease type III [GSD III] is an autosomal recessive disorder caused by deficiency of glycogen debrancher enzyme and is characterised by clinical variability. We herein describe the clinical and laboratory findings in 31 Egyptian patients with GSD III presenting to the Paediatric Hepatology Unit, Cairo University, Egypt. Eighteen patients [58%] were males. Their ages ranged between 6 months to 12 years. The main presenting complaint was progressive abdominal distention in 55%. Twelve patients [38.7%] had a history of recurrent attacks of convulsions; four had an erroneous diagnosis of hypocalcaemia and epilepsy. Dolllike facies was noted in 90%. Abdominal examination of all cases revealed abdominal distention and soft hepatomegaly which had bright echogenicity by ultrasound. Hypertriglyceridaemia was present in 93.6%, hyperlactacidaemia in 51.6% and hyperuricaemia in 19.4%. Liver biopsy showed markedly distended hepatocytes with well distinct cytoplasmic boundaries and 32% had macrovesicular fatty changes. Serum creatine kinase was elevated in 64.6% of patients and correlated positively and significantly with age [r = 0.7 and P = <0.001], while serum triglycerides correlated negatively with age [r = -0.4 and P = 0.05]. Blood glucose assessment and search for hepatomegaly in an infant with recurrent seizures may prevent delay in the diagnosis. A huge soft liver reaching the left midclavicular line that appears echogenic on ultrasonography is characteristic of GSD III. A distended hepatocyte with rarified cytoplasm is pathognomonic but not diagnostic. Hypertriglyceridaemia correlates negatively with age, in contrary to CK level

Factors associated with fibrosis progression in HCV infected children in Egypt

This study aimed at identification of factors that could be associated with development of hepatic fibrosis in children with HCV infection. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt. Liver biopsies were obtained from 43 children with HCV infection after having informed consent from their parents in the period "1995-2002". Their mean age at liver biopsy was and 8.67 +/- 4.3 years. Boys: girls ratio was 1.3:1. The results proved that, by examining the 43 patients' biopsies, 12 were having no fibrosis, 20 were having mild fibrosis and 11 were having moderate to severe fibrosis. The median time for development of fibrosis was estimated to be 5.5 years. Developing fibrosis was significantly associated with shorter duration from first detected ALT elevation to biopsy [P =0.015] and having higher levels of direct serum bilirubin [P Value=0.048]. Unexpectedly, development of fibrosis was slower in the group with co- morbid conditions compared to the group with no co-morbid conditions [P =0.04]. The development of hepatic fibrosis in children with HCV infection was associated with shorter duration of first detected ALT elevation to biopsy and higher direct serum bilirubin levels and it was progressing more slowly in the group having co-morbid conditions

Safety and efficacy of rifampicin in children with cholestatic pruritus

Objective: The present study aimed at verifying the safety and efficacy of rifampicin in ameliorating pruritus in pediatric patients suffering from persistent cholestasis

Methods: Twenty-three patients attending the Pediatric Hepatology Unit at Cairo University Children's Hospital, Egypt, were included in the present study. They were suffering from intractable pruritus secondary to persistent cholestasis from various etiologies. They were 14 males [60.87%] and 9 females [39.13%]. The mean duration of itch was 19 +/- 27.5 months. Rifampicin was started at a dose of 10 mg/kg/day in two divided doses. Liver function tests were followed up weekly to detect any deterioration that may be attributed to the drug

Results: Seventeen patients [74%] showed improvement of pruritus with rifampicin. Fourteen out of the seventeen [61%] improved at a dose of 10 mg/kg/day in 2 divided doses. The remaining 3 patients [13%] needed gradual dose increase by increments of 2 mg/kg/day every 2 weeks [maximum dose 20 mg/kg/day] until clinical improvement was observed. None of the patients showed any deterioration in liver functions, even though, a significant improvement in total serum bilirubin, ALT and AST was noticed following therapy

Conclusions: Rifampicin in a dose of 10-20 mg/kg/day is safe and effective in ameliorating uncontrollable pruritus in pediatric patients suffering from persistent cholestasis. No hepatoxicity was noted on close follow up in the studied children