Clinical and genetic risk factors for cystic fibrosis-related liver disease in Egyptian CF children: A single-center experience.

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple organ systems, including the liver, leading to CF-related liver disease (CFLD). It was noted that CFLD in Egyptian children with CF is more common than in non-Egyptian people with CF (pwCF). This study aimed to determine the incidence of CFLD and the potential risk factors for developing CFLD in Egyptian children. The correlation between CFLD and the various genotypes prevalent in Egyptian CF children will be discussed. In addition, comparison of CFLD in Egyptian and non-Egyptian CF patients will be presented. METHODS: This cross-sectional study included 50 pwCF from Ain Sham University's Pediatric Pulmonology Clinic in Children's Hospital, Cairo, Egypt. The sweat chloride test and genetic studies were done at the time of diagnosis. Additionally, all subjects underwent detailed history taking, laboratory investigations, clinical assessment, and pelvic abdominal ultrasound for evaluation of hepatic involvement. RESULTS: One-third of the Egyptian children with CF were found to have liver disease. The following independent risk factors for developing CFLD were identified as: male sex, severe genetic mutation (class I and II), long duration of CF disease, early onset of the CF, pancreatic insufficiency, as well as history of meconium ileus. In addition, diabetes mellitus and severe lung disease were proven to significantly increase the risk of developing CFLD. CONCLUSION: CFLD is common in Egyptian pwCF. CFLD's risk factors are similar to other reported research from other countries in the region.

Serum IL-6 and IL-8 in infants with biliary atresia in comparison to intrahepatic cholestasis.

BACKGROUND: Biliary atresia (BA) is an idiopathic progressive inflammation and fibrosclerosis of the extra and intrahepatic bile ducts. AIM: To measure the serum levels of IL-6 and IL-8 in biliary atresia and correlate with clinical outcome. METHODS: Sixty children were included, 20 with BA (group 1), 20 with intrahepatic cholestasis (group 2) and 20 normal children as controls. Complete blood count (CBC), liver function tests, serum IL-6 and IL-8 were analyzed in all the three groups. RESULTS: Mean serum IL-6 and IL-8 levels were significantly higher in group 1 (394.7 +/- 40.2 and 111.2 +/- 9.7 pg/dl, respectively) than group 2 (175.5 +/- 33.1 and 53.5 +/- 8.2 pg/dl, respectively) (p=0.0 for both). Both IL-6 and IL-8 were significantly higher in both the disease groups compared to controls (30.4 +/- 8.8 and 13.7 +/- 4.1 pg/dl, respectively). IL-8 showed a significant positive correlation with lymphocytes and significant negative correlation with albumin in group 1. Higher IL-6 was found in patients with BA with severe fibrosis than in patients with mild fibrosis while higher IL-8 was found in children with persistent jaundice than those free from jaundice in group 1. CONCLUSION: Serum IL-6 and IL8 are high in patients with BA indicating ongoing inflammation. They may serve to determine disease severity and may predict the progression to liver fibrosis in these cases.

Intestinal parasitic infection among Egyptian children with chronic liver diseases.

Patients with chronic liver diseases (CLD) are often highly susceptible to parasitic infection due to a depressed immune system. The objective of this study was to detect the most commonly intestinal parasites found among Egyptian children with CLD. The present study was conducted on 50 children with CLD of different etiology (25 were having different intestinal symptoms, 25 without intestinal symptoms) and 50 non-CLD children with gastrointestinal complaints served as controls. All cases were subjected to stool examination and investigated by liver function tests. Also, anthropometric measurements were taken for all children including weight and height. It was found that the most commonly intestinal protozoa identified in the patients with CLD in order of frequency were: Entamoeba histolytica/Entamoeba dispar (16 %), Giardia lamblia (14 %), Blastocystis hominis (14 %), Cryptosporidium parvum (10 %), E. histolytica and G. lamblia (2 %), E. histolytica and B. hominis (2 %), G. lamblia and B. hominis (2 %), B. hominis and Entamoeba coli (2 %), Microsporidium (2 %) and no cases were found infected with Strongyloides stercoralis. As compared to the controls, the observed incidence of these organisms in CLD patients was significantly higher (p < 0.045) as regards stool examination by unstained techniques while, there was no significant difference between both groups as regards stool examination by stained techniques (p < 0.478). In addition, this study showed that the weight and height of studied patients were affected by parasitic infection while, there was no significant correlation between parasitic infection and liver function tests. In conclusion, chronic liver diseases affect the immunity of the patients as shown in significant increase in the incidence of intestinal parasites in cases compared to controls.

Transfusion transmitted hepatitis: where do we stand now? A one center study in upper egypt.

BACKGROUND: Despite progress made in the prevention of transfusion-transmitted infections (TTI) over the last few years, they continue to be a problem in many parts of the world, particularly in multitransfused patients. OBJECTIVES: The aim of this study was to estimate the prevalence of hepatitis B virus (HBV), hepatitis C virus (HCV), and to evaluate the screening and vaccination program among our cohort of multitransfused children from Qena, Upper Egypt. PATIENTS AND METHODS: One-hundred children suffering from diseases requiring repeated blood transfusions were included in the study. They were classified into group 1, which included 67 children with thalassemia, and group 2, which included 33 children with hemophilia. Screening for hepatitis B surface antigen, hepatitis B surface antibody, hepatitis B core antibody and antibody to HCV was done using a second-generation enzyme-linked immunosorbent assay technique. RESULTS: Only 12% of all patients were either acutely or chronically infected with HBV. 46% were immune due to previous vaccination, whereas 39% of patients were not protected from HBV infection. HCV antibodies were positive in 45% of cases. Seventy-eight patients had a complete hepatitis B vaccination in the form of three doses as documented by birth certificate. Thirty-six patients mentioned history suggestive of hepatitis. The prevalence of the studied hepatitis markers was similar in both the thalassemia and hemophilia groups of children. CONCLUSIONS: Transfusion-transmitted hepatitis is still a major problem for multitransfused children in Egypt. More effort is required to reduce the infection rate through proper screening of blood and blood products, strict emphasis on receiving the vaccine, regular follow-up for those children with a hepatitis B antibody titer, and providing booster doses for those in need.