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2022
Méndez-Sánchez, N., E. Bugianesi, R. G. Gish, F. Lammert, H. Tilg, M. H. Nguyen, S. K. Sarin, N. Fabrellas, S. Zelber-Sagi, J. - G. Fan, et al., "Global multi-stakeholder endorsement of the MAFLD definition.", The lancet. Gastroenterology & hepatology, vol. 7, issue 5, pp. 388-390, 2022.
El-Koofy, N. M., H. M. El-karaksy, M. M. Aboelsnoon, M. A. Ibrahim, N. E. Badawi, and H. M. Fouad, "Growth and Final Height in Children with Autoimmune Hepatitis; A long term observation ", International journal of pediatrics , vol. 10, issue 4, pp. 15695-15709., 2022.
El-Shabrawi, M., I. Memon, D. Attia, and N. M. El-Koofy, "The International Society of Tropical Paediatrics (ISTP) endorses the redefinition of fatty liver disease.", Journal of hepatology, vol. 76, issue 3, pp. 738-739, 2022.
El-Shabrawi, M., I. Memon, D. Attia, and N. M. El-Koofy, "The International Society of Tropical Paediatrics (ISTP) endorses the redefinition of fatty liver disease.", Journal of hepatology, vol. 76, issue 3, pp. 738-739, 2022.
El-Shabrawi, M., I. Memon, D. Attia, and N. M. El-Koofy, "The International Society of Tropical Paediatrics (ISTP) endorses the redefinition of fatty liver disease.", Journal of hepatology, vol. 76, issue 3, pp. 738-739, 2022.
El-Shabrawi, M., I. Memon, D. Attia, and N. M. El-Koofy, "The International Society of Tropical Paediatrics (ISTP) endorses the redefinition of fatty liver disease.", Journal of hepatology, vol. 76, issue 3, pp. 738-739, 2022.
2019
Koofy, N. E., E. M. I. Moawad, M. Fahmy, M. A. Mohamed, H. F. A. Mohamed, E. M. Eid, M. E. Zaki, and R. M. El-Sayed, "Anthropometric, biochemical and clinical assessment of malnutrition among Egyptian children with chronic liver diseases: a single institutional cross-sectional study.", BMC gastroenterology, vol. 19, issue 1, pp. 223, 2019. Abstract

BACKGROUND: Malnutrition is a common problem among children with chronic liver diseases (CLD). We aimed to assess the nutritional status of children with CLD and to correlate the anthropometric indices with the severity of liver disease, liver function tests, insulin growth factor-1 (IGF-1) and 25-hydroxy vitamin D (25- OH D).

METHODS: A total of 69 patients with CLD and 50 healthy controls (6 months - 6 years) were included in the study. Nutritional status was assessed by anthropometric indices expressed in standard deviation score (Z score), biochemical, hematological and clinical parameters.

RESULTS: We found 52.2% of CLD patients underweight by weight for age (W/A); 50.2% were stunted by height for age/ length for age (HAZ or LAZ); and 39% exhibited wasting by weight/height or (length) for age (W/HZ or W/LZ) z scores analysis. The mean values of z scores for all anthropometric parameters were significantly correlated with unconjugated and conjugated bilirubin and INR (p < 0.05), except HAZ or LAZ. Also, a significant correlation to albumin was found, except for W/HZ or (W/LZ) (p = 0.157). The z scores < - 2 SD based on W/ H versus arm indicators showed significant differences in MUAC, UAA and AMA (p < 0.001). We found no correlation between anthropometric z-scores and the mean IGF-1 and (25- OH D) values (p > 0.05). Malnutrition was directly correlated with the severity of hepatic dysfunction, particularly, Child-Pugh C cases. The mean IGF-1 and (25- OH D) values were significantly correlated with the severity of liver disease (p < 0.001).

CONCLUSIONS: Our results identified anthropometric arm indicators and MUAC/A measurements as an effective applied methods for assessing nutritional status in CLD children. Moreover, Integrating comprehensive clinical assessment, anthropometric measurements and objective biochemical analyses is essential for evaluation, follow-up and management of CLD children with variable degree of malnutrition.

2018
N, E. - K., F. HM, F. ME, and E. - K. H. M. M. N. Helmy H, Shaker O, Copper concentrations in Egyptian infants with cholestasis: A single center study, , 2018.
2016
Tawfik, S., N. E. Koofy, and E. M. I. Moawad, "Patterns of Nutrition and Dietary Supplements Use in Young Egyptian Athletes: A Community-Based Cross-Sectional Survey.", PloS one, vol. 11, issue 8, pp. e0161252, 2016. Abstract

The aim of this study was to investigate the pattern of basic and sport nutrition as well as perspectives of young Egyptian athletes. Structured interview survey measuring knowledge, attitudes, beliefs and behaviors about basic and sport nutrition was administered to adolescent athletes recruited from 4 sport clubs and 2 fitness centers in Greater Cairo governorate. A total of 358 participants aged 13-18 years completed questionnaires. Basic nutrition knowledge was reasonable in almost all domains except fast food. Fixed breakfast (78.5%), home meals (lunch, 70.7%), and healthy snacks (55.8%) were the most positive features of the basic dietary pattern. More than 70% perceived themselves as knowledgeable about sport nutrition. The prevalence rate of sport supplement intake was (48.9%, n = 175), predominantly sport drinks (66.9%) and creatine (54.3%). Coaches were the primary source of sport nutrition information. Forty-four percent of participants (n = 77/175) reported supplement consumption during competition seasons only. Better physical appearance and enhancement of athletic performance were the major motivations for supplement intake. These findings indicate the necessity of a comprehensive nutrition education program targeting not only athletes and parents, but also coaching staff, health trainers and all sport team officials.

2015
El-karaksy, H. M., N. El-Koofy, N. Mohsen, H. Helmy, N. Nabil, and M. El-Shabrawi, "Extrahepatic portal vein obstruction in Egyptian children.", Journal of pediatric gastroenterology and nutrition, vol. 60, issue 1, pp. 105-9, 2015 Jan. Abstract

BACKGROUND AND AIM: Extrahepatic portal vein obstruction (EHPVO) is an important cause of portal hypertension in children. The aim of this study was to describe the clinical presentation, possible risk factors, upper gastrointestinal endoscopic findings, and treatment modalities of children with EHPVO.

METHODS: After ethical approval of our study protocol by our institution review board, we analyzed available data from medical records of patients with EHPVO presenting to the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt, for a period of 15 years from January 1996 to December 2010.

RESULTS: The study included 169 patients. Their ages at presentation ranged from 1 month to 12 years (median 2.5 years, interquartile range 5); 101 were boys. Hematemesis was a presenting symptom in 58%, splenomegaly was present in 87%, esophageal varices were present in 94%, and fundal varices were present in 23%. Possible risk factors, in the form of umbilical catheterization, umbilical sepsis, and exchange transfusion, were elicited in 18%. Propranolol was associated with reduction in bleeding episodes (P < 0.001), but was associated with increased chest symptoms (P < 0.01). Both injection sclerotherapy and band ligation were effective in the management of bleeding varices and for primary and secondary prophylaxis; however, injection sclerotherapy was associated with the development of secondary gastric varices (P = 0.03).

CONCLUSIONS: This large study of children with EHPVO demonstrates the efficacy of propranolol in the reduction of gastrointestinal bleeding in children with EHPVO. Both injection sclerotherapy and band ligation were effective in the management of esophageal varices, although the former was associated with the development of secondary gastric varices. Randomized clinical trials to choose the best modalities for the management of portal hypertension in children are still lacking.

El-karaksy, H. M., M. S. El-Raziky, H. M. Fouad, G. M. Anwar, F. M. El-Mougy, N. M. El-Koofy, and A. M. El-Hennawy, "The value of different insulin resistance indices in assessment of non-alcoholic fatty liver disease in overweight/obese children.", Diabetes & metabolic syndrome, vol. 9, issue 2, pp. 114-9, 2015 Apr-Jun. Abstract

OBJECTIVE: The aim of the present study was to determine the association between insulin resistance (IR) and both non-alcoholic fatty liver disease (NAFLD) and metabolic syndrome (MetS) in a group of Egyptian overweight/obese children and adolescents and to evaluate different IR indices in detection of NAFLD.

PATIENTS AND METHODS: The study included 76 overweight/obese children aged 2-15 years; 52.6% were males. Laboratory analysis included fasting blood glucose, serum insulin, lipid profile, liver biochemical profile, and liver ultrasound. IR was calculated using the following indices; the homeostasis model assessment method (HOMA-IR), the quantitative insulin-sensitivity check index (QUICKI) and hepatic insulin sensitivity. The National Cholesterol Education Program Adult Treatment Panel III criteria were used to estimate prevalence of MetS. Liver biopsy was done when medically indicated and accepted by parents.

RESULTS: IR was detected in 43.4% and 34.2% by using QUICKI and HOMA, respectively. MetS was detected in 36.8% and NAFLD was detected in 45.5% among those performing liver biopsy. Cases with NAFLD had more frequent IR than children with normal histology. QUICKI showed significant difference between normal subjects and both steatosis and non-alcoholic steatohepatitis; while HOMA-IR was sensitive in cases with NASH only. MetS was present in 100% of patients with NASH and in 75% of those with steatosis and they were all obese. Patients with NASH had significantly higher ALT than those with normal histology.

CONCLUSION: IR was significantly associated with NAFLD. QUICKI is considered more sensitive than HOMA-IR in differentiating simple steatosis from normal liver histology.

2014
El-Karaksy, H., N. El-Koofy, R. El-Sayed, M. El-Raziky, F. Rabah, M. El-Shabrawi, E. Salama, T. El-Baz, and M. El-Shazly, "Limitations of Living Donor Liver Transplantation in Egyptian Children.", Hepato-gastroenterology, vol. 61, issue 132, pp. 1090-3, 2014 Jun. Abstract

BACKGROUND/AIMS: In Egypt, the liver transplantation (LTx) program that became available since 2001 is a living donor program. We aimed to assess the obstacles to pediatric LTx.

METHODS: Over a six-month-period, 41 pediatric patients were indicated for LTx; their ages ranged between 1.5 months to 17 years. Patients and potential donors were evaluated according to the program protocol.

RESULTS: The obstacles for performing LTx were classified into recipient, donor and program obstacles or limitations. Each patient may have more than one limitation. Late presentation and co-morbid conditions were on the top of the recipient list of obstacles. Refusal of potential donors to donate was the commonest limitation on the donor side (33%). The commonest program limitations were young age and small size of the recipient.

CONCLUSIONS: Limitations in recipient characteristics as well as donor shortage are still the main obstacles for living donor liver transplantation (LDLT) in our pediatric liver disease patients. Small weight and young age of potential LDLT candidates are the principle causes for delaying this life saving procedure. Increasing community awareness about living organ donation and nutritional support for end stage liver disease (ESLD) babies is pivotal, given our limitation to a living donor program.

2013
El-Sayed, R., H. El-Karaksy, M. El-Raziky, M. El-Hawary, N. E. Koofy, H. Helmy, and M. Fahmy, "Assessment of coagulation and fibrinolysis in children with chronic liver disease.", Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis, vol. 24, issue 2, pp. 113-7, 2013 Mar. Abstract

We aimed at assessing the coagulation profile and detecting early evidence of fibrinolysis in pediatric patients with chronic liver disease. Seventy-six patients (40 boys) with a mean age of 9.8 ± 3.4 years suffering from chronic liver disease were enrolled in this study. They were followed up in the Pediatric Hepatology Unit, Cairo University Children's Hospital. Thirty healthy children were included as controls. Patients were classified etiologically into four groups: chronic viral hepatitis, autoimmune hepatitis, miscellaneous and cryptogenic groups. Investigations to detect coagulopathy were done for all patients and controls: prothrombin time (PT), activated partial thromboplastin time, fibrinogen, fibrinogen degradation products, and D-dimer and complete blood count. Liver functions were done for all patient groups. A significantly lower platelet count, prolonged prothrombin time, with prolonged aPTT time was detected in all patients compared with controls (P < 0.001). The fibrinogen level showed no significant difference between patients and controls. D-dimer level was significantly higher in the miscellaneous and cryptogenic groups when compared to other patient groups and control group (P < 0.001). Significantly higher D-dimer levels were detected in patients with liver cirrhosis of child class A and B compared with noncirrhotic and control groups (P < 0.001). D-dimer correlated positively with PT (r = 0.290, P = 0.003), and negatively with platelet count (r = -0.324, P = 0.001) and prothrombin concentration (r = -0.270, P = 0.018). Fibrinolytic activity, as evidenced by high D-dimer, was detected in pediatric patients with chronic liver disease particularly if cirrhotic.

2012
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-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.

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.

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-Koofy, N. M., G. M. Anwar, M. S. El-Raziky, A. M. El-Hennawy, F. M. El-Mougy, H. M. El-karaksy, F. M. Hassanin, and H. M. Helmy, "The association of metabolic syndrome, insulin resistance and non-alcoholic fatty liver disease in overweight/obese children.", Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association, vol. 18, issue 1, pp. 44-9, 2012 Jan-Feb. Abstract

BACKGROUND/AIM: To study the prevalence of metabolic syndrome (MS), insulin resistance (IR) and non-alcoholic fatty liver disease (NAFLD) in overweight/obese children with clinical hepatomegaly and/or raised alanine aminotransferase (ALT).

PATIENTS AND METHODS: Thirty-three overweight and obese children, aged 2-13 years, presenting with hepatomegaly and/or raised ALT, were studied for the prevalence of MS, IR and NAFLD. Laboratory analysis included fasting blood glucose, serum insulin, serum triglycerides (TG), total cholesterol, high-density lipoprotein cholesterol (HDL-c), low-density lipoprotein cholesterol (LDL-c) and liver biochemical profile, in addition to liver ultrasound and liver biopsy.

RESULTS: Twenty patients (60.6%) were labeled with MS. IR was present in 16 (48.4%). Fifteen (44%) patients had biopsy-proven NAFLD. Patients with MS were more likely to have NAFLD by biopsy (P=0.001). Children with NAFLD had significantly higher body mass index, waist circumference, ALT, total cholesterol, LDL-c, TG, fasting insulin, and lower HDL-c compared to patients with normal liver histology (P< 0.05) and fitted more with the criteria of MS (80% vs. 44%). IR was significantly more common among NAFLD patients (73% vs. 28%).

CONCLUSION: There is a close association between obesity, MS, IR and NAFLD. Obese children with clinical or biochemical hepatic abnormalities are prone to suffer from MS, IR and NAFLD.

El-Koofy, N. M., G. M. Anwar, M. S. El-Raziky, A. M. El-Hennawy, F. M. El-Mougy, H. M. El-karaksy, F. M. Hassanin, and H. M. Helmy, "The association of metabolic syndrome, insulin resistance and non-alcoholic fatty liver disease in overweight/obese children.", Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association, vol. 18, issue 1, pp. 44-9, 2012 Jan-Feb. Abstract

BACKGROUND/AIM: To study the prevalence of metabolic syndrome (MS), insulin resistance (IR) and non-alcoholic fatty liver disease (NAFLD) in overweight/obese children with clinical hepatomegaly and/or raised alanine aminotransferase (ALT).

PATIENTS AND METHODS: Thirty-three overweight and obese children, aged 2-13 years, presenting with hepatomegaly and/or raised ALT, were studied for the prevalence of MS, IR and NAFLD. Laboratory analysis included fasting blood glucose, serum insulin, serum triglycerides (TG), total cholesterol, high-density lipoprotein cholesterol (HDL-c), low-density lipoprotein cholesterol (LDL-c) and liver biochemical profile, in addition to liver ultrasound and liver biopsy.

RESULTS: Twenty patients (60.6%) were labeled with MS. IR was present in 16 (48.4%). Fifteen (44%) patients had biopsy-proven NAFLD. Patients with MS were more likely to have NAFLD by biopsy (P=0.001). Children with NAFLD had significantly higher body mass index, waist circumference, ALT, total cholesterol, LDL-c, TG, fasting insulin, and lower HDL-c compared to patients with normal liver histology (P< 0.05) and fitted more with the criteria of MS (80% vs. 44%). IR was significantly more common among NAFLD patients (73% vs. 28%).

CONCLUSION: There is a close association between obesity, MS, IR and NAFLD. Obese children with clinical or biochemical hepatic abnormalities are prone to suffer from MS, IR and NAFLD.

2011
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-Sayed, R., M. Fahmy, N. E. Koofy, M. El-Raziky, M. El-Hawary, H. Helmy, W. El-Akel, A. El-Hennawy, and H. El-Karaksy, "Can aspartate aminotransferase to platelet ratio index replace liver biopsy in chronic hepatitis C?", Tropical gastroenterology : official journal of the Digestive Diseases Foundation, vol. 32, issue 4, pp. 267-72, 2011 Oct-Dec. Abstract

BACKGROUND AND AIM: We aimed to evaluate the accuracy of readily available laboratory tests (ALT, AST, platelet count, AST to platelet ratio index: APRI) in predicting liver fibrosis in chronic hepatitis C, in comparison to the predictive accuracy obtained by liver biopsy. Pediatrics,

METHODS: One hundred and thirteen patients suffering from chronic hepatitis C (CHC) were included in this study. They included 76 children enrolled from the Pediatric Hepatology Unit and 37 adults enrolled from the Hepatology Unit of Tropical Medicine Department, Cairo University, Egypt. Fibrosis results obtained from liver biopsy were assigned a score from 0 to 4 score as per Metavir scoring. Results of serum ALT and AST levels were expressed as ratio of the upper limit of normal (ULN).

RESULTS: Of the pediatric patients, 28 (36.8%) showed no evidence of fibrosis on liver biopsy, 26 (34.2%) showed grade 1 fibrosis, and 22 (29%) had grade 2 fibrosis. Among the adult patients, 12 (32.4%) had grade 2 fibrosis and 25 patients (67.6%) had grades 3 to 4 fibrosis. There was a lack of correlation between the degree of fibrosis and AST levels, AST/ALT ratio, platelet count and APRI. The AUROC curve for predicting significant fibrosis was 0.5 for AST levels, 0.37 for AST/ALT ratio and 0.49 for APRI, in pediatric patients (p > 0.05). In adult patients the AUROC curve for predicting significant fibrosis was 0.59 for AST levels, 0.76 for AST/ALT ratio and 0.63 for APRI (p > 0.05).

CONCLUSION: Liver biopsy remains the gold standard to assess the extent of hepatic fibrosis in patients with CHC.

El-Koofy, N. M., R. El-Mahdy, M. E. Fahmy, A. El-Hennawy, M. Y. Farag, and H. M. El-karaksy, "Alagille syndrome: clinical and ocular pathognomonic features.", European journal of ophthalmology, vol. 21, issue 2, pp. 199-206, 2011 Mar-Apr. Abstract

PURPOSE: This study was carried out to determine the frequency of clinical diagnostic criteria in patients with Alagille syndrome (AGS) in comparison to a group of children with cholestasis and histologically proven neonatal hepatitis (NH). The type and frequency of ocular manifestations are highlighted.

METHODS: According to histologic findings on liver biopsy, the 32 patients included in the study were classified into 2 groups: group 1 had paucity of interlobular bile ducts (PILBD) (n=13) and at least 3 of 5 characteristics of AGS and group 2 had NH on liver biopsy (n=19).

RESULTS: The mean age of patients with AGS was higher than in group 2 (3.9 vs 1.6 years) (p<0.05). Pruritus was a significant symptom in the AGS group (p<0.05). Characteristic facies was detected in 61.5% of the AGS group. None of our patients with AGS had vertebral anomalies. Although cardiac murmurs were heard in 5 patients with AGS, cardiac anomalies were detected in only 3 by echocardiography: pulmonary stenosis in 2 patients and patent foramen ovale in 1. Posterior embryotoxon (PE) was detected in 69.2% of patients with AGS as compared to 10.5% of the NH group. Optic nerve drusen was detected in 91% of patients with AGS as compared to 5.3% of patients with NH.

CONCLUSIONS: Optic nerve drusen was the most common finding in AGS, followed by PE and facial features. Ocular ultrasound needs to be performed in all cholestatic infants with PILBD.

El-karaksy, H. M., M. M. El-Shabrawi, N. A. Mohsen, N. M. El-Koofy, W. A. El-Akel, M. E. Fahmy, and N. A. Yassin, "Study of predictive value of pediatric risk of mortality (PRISM) score in children with end stage liver disease and fulminant hepatic failure.", Indian journal of pediatrics, vol. 78, issue 3, pp. 301-6, 2011 Mar. Abstract

OBJECTIVE: To evaluate the use of the PRISM score as a predictor of outcome in patients with end stage liver disease (ESLD) and fulminant hepatic failure (FHF).

METHODS: The present study included 30 patients with ESLD and FHF, with ages ranging from 2 to 108 months, who were admitted to the Emergency room (ER) and the Pediatric Hepatology Unit at Cairo University Children's Hospital (tertiary referral hospital) over a six-month-period from May through October 2008. Survivors were followed up for 6 months. Two other scores were also calculated, the PELD score and the Child-Pugh score. The outcome was defined as survivors and deceased.

RESULTS: Deceased patients as compared with survivors were significantly younger (median age 7 vs. 24 months, p=0.003). A ROC curve was constructed for the PRISM score, the predicted death rate (PDR) and the PELD score in the 30 patients. PRISM score was significantly associated with mortality (p=0.04). The best cut off value was 9.5 (70.6% sensitive and 61% specific). PDR was also significantly associated with mortality (p=0.011). The best cut off value for PDR was 5.95 (70.6% sensitive, 85% specific). On the other hand, the PELD score was not associated with mortality (p=0.202).

CONCLUSIONS: PRISM score can be applied with an adequate degree of accuracy for severity assessment and mortality prediction to pediatric patients with ESLD or FHF.

El-Koofy, N. M., H. M. El-karaksy, I. M. Mandour, G. M. Anwar, M. S. El-Raziky, and A. M. El-Hennawy, "Genetic polymorphisms in non-alcoholic fatty liver disease in obese Egyptian children.", Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association, vol. 17, issue 4, pp. 265-70, 2011 Jul-Aug. Abstract

BACKGROUND/AIM: Polymorphisms in the promoter of microsomal triglyceride transfer protein (MTP) lead to decreased MTP transcription, less export of triglyceride from hepatocytes, and greater intracellular triglyceride accumulation. Therefore, functional polymorphisms in MTP may be involved in determining susceptibility to nonalcoholic steatohepatitis (NASH). The aim of this study is to examine the effect of some genetic influences among a group of obese Egyptian children.

PATIENTS AND METHODS: A cross-sectional study was conducted on 76 overweight and obese children presenting to the Pediatric Endocrinology Unit, Cairo University Children's Hospital, Egypt, as well as on 20 healthy controls. Anthropometric measurements were taken for all the patients and they underwent clinical examination, ultrasonographic examination of the liver, and liver biopsy when appropriate. Liver functions, blood glucose, serum insulin, C-peptide, and lipid profile were assessed and HOMA-IR calculated. Blood samples from biopsy-proven NASH patients and controls were analyzed by polymerase chain reaction (PCR) and restriction fragment length polymorphism for the -493 G/T polymorphism in the promoter of MTP and the 1183 T/C polymorphism in the mitochondrial targeting sequence of manganese superoxide dismutase (MnSOD).

RESULTS: Eight had biopsy-proven simple steatosis and 7 had NASH. NASH patients had a much higher incidence of the MTP G/G genotype (P = 0.002, CI: 2.9-392) compared with the controls. NASH patients also had a 100% prevalence of the MnSOD T/T genotype.

CONCLUSION: Certain genotypes in MTP and MnSOD are significantly more prevalent among obese children with NASH and may be responsible for such a phenotype.