El-Karaksy, H. - M., M. - I. El-Hawary, N. - M. El-Koofy, R. El-Sayed, M. - A. - S. El-Raziky, S. - A. Mansour, G. - M. Taha, and F. El-Mougy, "Safety and efficacy of hepatitis A vaccine in children with chronic liver disease.", World journal of gastroenterology : WJG, vol. 12, issue 45, pp. 7337-40, 2006 Dec 7. Abstract

AIM: To study the safety and efficacy of hepatitis A vaccine (HAV) in children with chronic liver disease of various etiologies.

METHODS: Eleven children with chronic liver disease and thirteen age- and sex-matched controls negative for HAV antibodies were vaccinated against hepatitis A after they gave their informed consent. Children with uncontrolled coagulopathy or signs of hepatic decompensation were excluded. The vaccine (Havrix: 720 ELISA units in 0.5 mL, from GlaxoSmithKline Biologicals) was given intramuscularly in the deltoid in 2 doses 6 mo apart. Children were tested for HAV antibodies one and six months after the 1st dose and one month after the 2nd dose. Total serum bilirubin, alanine aminotransferase (ALT), and aspartate aminotransferase (AST) were determined immediately before and after one month of the 1st dose of the vaccine.

RESULTS: Only 7 out of the 11 patients were positive for HAV antibodies after the 1st dose of the vaccine, as compared to 100% of the controls. One month after the 2nd dose, all patients tested were positive for HAV antibodies. No deterioration in liver functions of patients was noted after vaccination. No adverse events, immediate or late, were reported by the mothers after each dose of the vaccine.

CONCLUSION: Hepatitis A vaccine is both safe and effective in this small studied group of children with chronic liver disease. Given the high seroconversion rate, post-vaccination testing for HAV antibodies is not needed.

El-Sayed, R., and H. El-Karaksy, "Acute pancreatitis complicating acute hepatitis A virus infection.", Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology, vol. 13, issue 4, pp. 184-5, 2012 Dec. Abstract

Acute pancreatitis complicating acute hepatitis A is very rare especially in children. We report here an 11 year old female patient with picture of acute hepatitis proved to be caused by hepatitis A. One week later patient's condition worsened, she was jaundiced, with persistent vomiting and looked acutely ill and uncomfortable with severe steady abdominal pain mainly in the epigastrium and upper quadrants. Acute pancreatitis was suspected and proved by a clinical picture associated with elevated serum amylase and serum lipase and by MRCP. The patient was managed conservatively with gradual clinical and laboratory improvement, and she was discharged after one week in a good clinical condition.

El-Karaksy, H., M. Rashed, R. El-Sayed, M. El-Raziky, N. El-Koofy, M. El-Hawary, and O. Al-Dirbashi, "Clinical practice. NTBC therapy for tyrosinemia type 1: how much is enough?", European journal of pediatrics, vol. 169, issue 6, pp. 689-93, 2010 Jun. Abstract

Four patients with tyrosinemia type 1 (ages 6-32 months) were treated with 2-(2-nitro-4-trifluoro-methylbenzoyl)-1,3-cyclohexandion (NTBC) at Cairo University Children's Hospital, Egypt and followed up for 12-27 months. The recommended average dose of NTBC is 1 mg/kg/day. They were started on the following doses: 0.8, 0.58, 0.5, and 0.625 mg/kg/day, respectively. Two months after start of therapy, succinylacetone was undetectable in patients 1, 2, and 4, while in case 3, it was 5.4 microM. Her NTBC dose was increased from 0.5 to 0.65 mg/kg/day, and succinylacetone was undetectable 1 month later. They were kept on NTBC doses ranging from 0.55 to 0.65 mg/kg/day. These doses allowed catch up growth, normalization of synthetic liver functions, steep drop in serum alpha fetoprotein, reduction in phosphate loss in urine, normalization of serum calcium, phosphate, and alkaline phosphatase, and healing of active rickets. Succinylacetone was undetectable in urine on these doses. In conclusion: Doses of NTBC, lower than recommended, may be helpful in treatment of tyrosinemia, on condition that succinylacetone production is suppressed, and AFP is maintained normal or showing a progressive decrease. This cost-effective dose may allow treatment of affected children from economically underprivileged countries, but longer follow up periods are needed.

Esmat, G., M. Hashem, M. El-Raziky, W. El-Akel, S. El-Naghy, N. El-Koofy, R. El-Sayed, R. Ahmed, M. Atta-Allah, M. A. Hamid, et al., "Risk factors for hepatitis C virus acquisition and predictors of persistence among Egyptian children.", Liver international : official journal of the International Association for the Study of the Liver, vol. 32, issue 3, pp. 449-56, 2012 Mar. Abstract

BACKGROUND: Hepatitis C virus (HCV) has a lower prevalence in children and knowledge is limited regarding the natural outcome of HCV infection in children.

AIM: To study the risk factors of HCV acquisition and predictors of persistence in Egyptian children.

METHODS: Children, 1-9 years of age, were evaluated for acquisition of HCV (anti-HCV positive regardless of viraemia) and persistence of HCV (anti-HCV and HCV-RNA positive) at two paediatric hepatology clinics in Cairo at enrollment and at 3 monthly intervals. Spontaneous clearance of HCV was defined as ≥ two positive anti-HCV antibody tests with negative HCV-RNA at least 6 months apart.

RESULTS: Over a 33-month-period a total of 226 children <9 years of age were screened for HCV antibodies. Of those, 146 (65%) were anti-HCV positive of which 87 (60%) were HCV-RNA positive. The HCV acquisition was more likely to occur in older children (P = 0.003) with comorbid conditions (P < 0.01) compared to anti-HCV negative children. In a multivariate logistic regression analysis, the highest risk factors for HCV acquisition were surgical interventions [odds ratio (OR): 4.7] and blood transfusions (OR: 2.3). The highest risk factor for HCV persistence was dental treatment (OR: 16.9) and male gender (OR: 7.5). HCV persistence was also strongly associated with elevated baseline alanine aminotransaminase (ALT) levels (OR: 4.9) and fluctuating aspartate aminotransferase (AST) levels (OR: 8.1).

CONCLUSION: Although surgical interventions and blood transfusion are significant risk factors for HCV acquisition in Egyptian children, dental treatment remains the highest risk factor for HCV chronic persistence in children.

El-Karaksy, H., S. Mansour, R. El-Sayed, M. El-Raziky, N. El-Koofy, and G. Taha, "Safety and efficacy of rifampicin in children with cholestatic pruritus.", Indian journal of pediatrics, vol. 74, issue 3, pp. 279-81, 2007 Mar. Abstract

OBJECTIVE: The present study aimed at verifying the safety and efficacy of rifampicin in ameliorating pruritus in cholestatic children.

METHODS: Twenty-three Egyptian children (14 boys and 9 girls), suffering from intractable pruritus of cholestasis, were included. Rifampicin was started at a dose of 10 mg/Kg/day in two divided doses and increased gradually to a maximum of 20 mg/Kg/day if there was no response. Liver function tests were followed up weekly.

RESULTS: Seventeen patients (74%) showed improvement of pruritus with rifampicin. None of the patients showed any deterioration in liver functions.

CONCLUSION: Rifampicin in a dose of 10-20 mg/Kg/day is safe and effective in ameliorating uncontrollable pruritus in children with persistent cholestasis.

El-Karaksy, H., M. Fahmy, M. El-Raziky, N. El-Koofy, R. El-Sayed, M. S. Rashed, H. El-Kiki, A. El-Hennawy, and N. Mohsen, "Hereditary tyrosinemia type 1 from a single center in Egypt: clinical study of 22 cases.", World journal of pediatrics : WJP, vol. 7, issue 3, pp. 224-31, 2011 Aug. Abstract

BACKGROUND: Hereditary tyrosinemia type 1 (HT1) is an increasingly recognized inborn error of metabolism among Egyptian children. This study was undertaken to define the presenting clinical, biochemical and imaging features and outcome of 2-(2-motrp-4-trifluoromethylbenzoyl)-1, 3-cyclohexanedione (NTBC) therapy and liver transplantation in a cohort of Egyptian children diagnosed with HT1.

METHODS: The study was carried out at the Pediatric Hepatology Unit at Cairo University Children's Hospital. HT1 was diagnosed by quantification of succinylacetone (SA) in dry blood spots.

RESULTS: Twenty-two patients were diagnosed with HT1 in a period of 3 years from August 2006 to July 2009. Infants with focal hepatic lesions and hepatomegaly (n=13) were younger at diagnosis than those with rickets (n=5) (median age: 3.25 vs. 10 months; P=0.05). Alpha fetoprotein was highly elevated in all children. Seven children died within a few weeks of diagnosis before therapy was initiated. Ten children were treated with NTBC. The response to NTBC treatment was apparent by a steep drop in serum alpha fetoprotein (AFP) and undetectable SA in urine within 2 months. Three children underwent living donor liver transplantation after treatment with NTBC for 10, 18 and 22 months respectively, despite adequate response to therapy because of financial issues. The explanted livers were all cirrhotic with no dysplasia or malignant transformation.

CONCLUSIONS: Focal hepatic lesions are the commonest presentation of HT1 patients and they present at an earlier age than rickets. NTBC is effective but very expensive. Liver transplantation is still considered in HT1 patients.

El-Karaksy, H., G. H Anwar, M. S. El-Raziky, M. El-Hawary, M. Hashem, R. El-Sayed, M. El-Shabrawi, N. Mohsen, H. Fouad, and G. Esmat, "Anti-HCV prevalence among diabetic and non-diabetic Egyptian children.", Current diabetes reviews, vol. 6, issue 6, pp. 388-92, 2010 Nov. Abstract

Our aim was to determine the prevalence of the HCV infection among children with type 1 DM as compared to a group of non-diabetic children attending the general outpatient clinics of the same hospital and investigate the possible risk factors. The study was carried out on 692 children with type 1 DM attending the Pediatric Diabetes Unit at Cairo University Pediatric Hospital, Egypt, and 1042 non-diabetic children attending the general outpatient clinics of the same hospital. They were screened for HCV antibodies using third generation ELISA. Anti-HCV antibody prevalence in diabetic children below 9 years of age was comparable to that of non diabetic children (2.5% vs. 1.4%; p=0.25). Diabetic children had higher exposure to medical care (p=0.04); all diabetics were exposed to daily insulin injections and daily blood glucose monitoring. Non-diabetics had higher exposure to razors used by others (p=0.05) and higher rate of traditional hair cutting (p=0.05). To conclude, the prevalence of anti-HCV in diabetic children below 9 years of age was comparable to non diabetic children of the same age group. Application of standard precautions for infection control could successfully limit spread of HCV infection in our Pediatric Diabetes Unit, in a country with high HCV load as Egypt.

El-Karaksy, H., M. Fahmy, M. S. El-Raziky, M. El-Hawary, R. El-Sayed, N. El-Koofy, F. El-Mougy, A. El-Hennawy, and M. El-Shabrawi, "A clinical study of Wilson's disease: The experience of a single Egyptian Paediatric Hepatology Unit.", Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology, vol. 12, issue 3, pp. 125-30, 2011 Sep. Abstract

BACKGROUND AND STUDY AIMS: Most paediatric patients with Wilson's disease (WD) present with hepatic manifestations, but some may have neurologic or psychiatric features. Our aim was to define the clinical, biochemical features and the outcome of therapy of a group of Egyptian children diagnosed with WD.

PATIENTS AND METHODS: The study was carried out at the Paediatric Hepatology Unit at Cairo University Children's Hospital, Egypt; 54 patients were diagnosed with WD from 1996 to 2009. The diagnosis was based on low serum ceruloplasmin levels, increased urinary copper concentrations before or after D-penicillamine challenge and/or the presence of Kayser-Fleischer (K-F) rings.

RESULTS: The clinical presentation was as follows: hepatic presentation in 33 patients (61%), hepato-neurologic 3 (5.5%), neurologic 5 (9.3%) and presymptomatic 13 (24%). Twelve couples had more than one affected sib. Increased urinary copper concentrations before or after D-penicillamine challenge was found in all patients, low serum ceruloplasmin in 97% and K-F rings in 31.5%. All patients were treated with penicillamine and zinc sulphate except one presymptomatic case who was treated with zinc sulphate only. Three patients underwent liver transplantation and eight patients died after a median duration of treatment of 6 months (1-36). The hepatic symptoms improved with treatment but the neurological symptoms remained stationary.

CONCLUSIONS: Clinical and biochemical assays remain the standard for diagnosis of WD. Penicillamine and zinc therapy can effectively treat WD with hepatic symptoms. Liver transplantation remains life saving for those with fulminant and end stage WD. Screening for presymptomatic sibs is of utmost importance.

El-Koofy, N., H. El-Karaksy, W. El-Akel, H. Helmy, G. Anwar, R. El-Sayed, and A. El-Hennawy, "Ultrasonography as a non-invasive tool for detection of nonalcoholic fatty liver disease in overweight/obese Egyptian children.", European journal of radiology, vol. 81, issue 11, pp. 3120-3, 2012 Nov. Abstract

INTRODUCTION: Liver biopsy, although a gold standard in diagnosis of nonalcoholic fatty liver disease (NAFLD), is an invasive and expensive tool.

AIM: To assess the diagnostic accuracy of abdominal ultrasound in detecting NAFLD among a group of overweight/obese children having one or more liver abnormality (clinical hepatomegaly, raised ALT or echogenic liver parenchyma by ultrasound).

METHODS: Seventy-eight overweight/obese children were referred to the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt, for assessment for hepatic abnormalities. Out of the 78 children, 34 had one or more abnormality in the form of clinical hepatomegaly, raised alanine aminotransferase (ALT) and/or echogenic liver parenchyma by ultrasound. All 34 cases underwent liver biopsy for evaluation for NAFLD.

RESULTS: Histological NAFLD was detected in 15 cases; 8 simple steatosis and 7 nonalcoholic steatohepatitis (NASH). Sonographic evaluation of hepatic parenchymal echogenicity revealed: 11 with grade 1 echogenicity, 12 with grade 2 and 9 with grade 3 while only 2 had normal liver echopattern. Ultrasonography was 100% sensitive and 100% specific in detecting histological NAFLD, while the positive predictive value (PPV) was 47% and negative predictive value (NPV) was 11%. After consolidating the included children into 2 groups: the first including normal and grade 1 echogenicity and the second including grades 2 and 3, the sensitivity of ultrasonography in detecting histological NAFLD was still 100%, while negative predictive value increased to 100% with an accuracy of 82%.

CONCLUSION: We conclude that ultrasonography is an important non invasive tool in assessment for NAFLD. Normal or grade 1 hepatic echogenicity can soundly exclude histological NAFLD and obviates the need for liver biopsy.

El-Hawary, M. A., M. S. El-Raziky, G. Esmat, H. Soliman, A. Abouzied, M. El-Raziky, W. El-Akel, R. El-Sayed, F. Shebl, A. - A. Shaheen, et al., "Assessment of hepatic fibrosis in pediatric cases with hepatitis C virus in Egypt.", World journal of gastroenterology : WJG, vol. 13, issue 20, pp. 2846-51, 2007 May 28. Abstract

AIM: To assess hepatic fibrosis and factors associated with its progression in children with HCV infection.

METHODS: At the Hepatology Unit, Cairo University Children's Hospital, a single liver biopsy was performed to 43 children with HCV infection after an informed consent between 1998-2004. Their mean age at liver biopsy was 8.67 +/- 4.3 years.

RESULTS: Among the 43 patients' biopsies, 12 (27.9%) were having no fibrosis, 20 (46.5%) mild fibrosis and 11 (25.6%) 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 (12 mo vs 1.2 mo, P=0.015) and having higher levels of direct serum bilirubin (0.3 mg/dL vs 0.5 mg/dL, P=0.048). No association was found between fibrosis stage and the presence of co-morbid conditions (P=0.33).

CONCLUSION: Hepatic fibrosis was present in 72.1% of children with HCV infection. The development of fibrosis was associated with higher levels of direct serum bilirubin. There was no significant association between fibrosis and age, duration of infection, risk factors, co-morbid conditions and most biochemical parameters.

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