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1.
BMC Gastroenterol ; 19(1): 223, 2019 Dec 21.
Article in English | MEDLINE | ID: mdl-31864303

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.


Subject(s)
Liver Diseases/complications , Malnutrition/diagnosis , Nutrition Assessment , Age Factors , Arm/anatomy & histology , Body Height , Body Weight , Carrier Proteins/blood , Case-Control Studies , Child , Child, Preschool , Chronic Disease , Cross-Sectional Studies , Egypt , Female , Growth Disorders/blood , Growth Disorders/diagnosis , Head/anatomy & histology , Humans , Infant , Insulin-Like Growth Factor I/analysis , Liver Diseases/blood , Liver Function Tests , Male , Malnutrition/blood , Malnutrition/etiology , Serum Albumin/analysis , Severity of Illness Index , Skinfold Thickness , Thinness/blood , Thinness/diagnosis , Vitamin D/analogs & derivatives , Vitamin D/blood , Wasting Syndrome/blood , Wasting Syndrome/diagnosis
2.
Minerva Pediatr ; 70(1): 35-45, 2018 Feb.
Article in English | MEDLINE | ID: mdl-25926159

ABSTRACT

BACKGROUND: Hepatic focal lesions in the pediatric age group are diverse and can be broadly classified into congenital, neoplastic and infective. The aim of this paper was to describe the frequency, nature and clinical presentation of focal hepatic lesions from a pediatric hepatologist perspective. METHODS: Data were retrieved from files of all cases with focal hepatic lesions presenting to the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, from January 2006 to December 2013, after the study protocol was approved by the department research committee and the institution ethical committee. RESULTS: Over an 8-year period, 38 cases had focal hepatic lesions. They constituted less than 1% of the 4475 new cases presenting to the unit over this period. The commonest lesion was hepatic hemangioma(s) (34%). Two-thirds were neoplastic lesions whether benign or malignant. Eighty percent were benign focal lesions. Infectious causes (fascioliasis and pyogenic liver abscess) accounted for 29% of cases. Hepatocellular carcinoma was the commonest malignant neoplasm; it occurred in 5 cases (13.2%) on top of a chronic liver disease. Hepatoblastoma was less common. CONCLUSIONS: From the hepatologist perspective, pediatric focal hepatic lesions are more likely to be benign. Hepatic hemangiomas are the commonest. Infectious causes are common in a developing country like Egypt. Hepatocellular carcinoma is the commoner malignant neoplasm and usually develops on a diseased liver. Screening infants and children with chronic liver disease for development of hepatocellular carcinoma is mandatory. Hepatoblastoma is less likely to present to the pediatric hepatologist as it is referred immediately to the oncologist or onco-surgeon.


Subject(s)
Carcinoma, Hepatocellular/diagnosis , Liver Diseases/diagnosis , Liver Neoplasms/diagnosis , Liver/pathology , Carcinoma, Hepatocellular/epidemiology , Carcinoma, Hepatocellular/pathology , Child , Child, Preschool , Chronic Disease , Egypt/epidemiology , Female , Gastroenterologists , Hemangioma/diagnosis , Hemangioma/epidemiology , Hemangioma/pathology , Hepatoblastoma/diagnosis , Hepatoblastoma/epidemiology , Hepatoblastoma/pathology , Hospitals, Pediatric , Hospitals, University , Humans , Infant , Infant, Newborn , Liver Diseases/epidemiology , Liver Diseases/pathology , Liver Neoplasms/epidemiology , Liver Neoplasms/pathology , Male , Mass Screening/methods
3.
Arab J Gastroenterol ; 17(4): 168-175, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27914885

ABSTRACT

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 12months. RESULTS: Patients were 102 cases; 62 were males, with age range 18days to 12years. 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.


Subject(s)
Biopsy/adverse effects , Biopsy/methods , Hematoma/etiology , Liver Diseases/pathology , Liver/pathology , Ultrasonography, Interventional , Age Factors , Ascites/etiology , Biopsy/mortality , Blood Transfusion , Child , Child, Preschool , Female , Humans , Hypotension/complications , Infant , Infant, Newborn , Liver Diseases/diagnosis , Male , Pain, Postoperative/etiology , Percussion , Platelet Count
4.
Arab J Gastroenterol ; 17(2): 105-9, 2016 Jun.
Article in English | MEDLINE | ID: mdl-27397412

ABSTRACT

BACKGROUND AND STUDY AIM: Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome with liver involvement varying from mild dysfunction to severe fulminant failure. The aim of this study was to present a case series of four HLH patients presenting with acute liver failure (ALF) in the neonatal period. PATIENTS AND METHODS: All four patients were neonates at the onset of symptoms. They presented to Cairo University Pediatric Hospital with ALF; they underwent prompt investigations including determination of ferritin, fibrinogen, and triglyceride levels as part of our ALF workup. Further investigations were tailored according to the associated clinical features and the results of preliminary investigations. RESULTS: HLH was diagnosed according to HLH-2004 criteria. Three patients fulfilled at least five out of eight criteria. Fever, splenomegaly, elevated ferritin levels, and low fibrinogen levels were present in all patients. The fourth patient had a serum ferritin level >10,000ng/ml, favouring the diagnosis of HLH, despite fulfilling only four out of eight criteria. For three patients, positive consanguinity and previous sibling death were reported, suggesting a genetic aetiology of HLH. CONCLUSION: ALF can be the presenting feature of HLH; thus, a high index of suspicion is necessary. Fever is a hallmark, especially in neonates. Diagnosis is important for this potentially treatable condition.


Subject(s)
Liver Failure, Acute/etiology , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/diagnosis , Consanguinity , Fatal Outcome , Female , Ferritins/blood , Fever/etiology , Humans , Infant , Infant, Newborn , Lymphohistiocytosis, Hemophagocytic/genetics , Male , Splenomegaly/etiology
5.
Arab J Gastroenterol ; 16(3-4): 142-4, 2015.
Article in English | MEDLINE | ID: mdl-26520282

ABSTRACT

UNLABELLED: Chanarin-Dorfman syndrome, a "neutral lipid storage disease with ichthyosis," is a multisystem inherited metabolic disorder associated with congenital ichthyosis and accumulation of lipid droplets in various types of cells. CASE REPORT: A 3-year-old male presented to the Pediatric Hepatology Unit, Cairo University Children's Hospital, Cairo, Egypt, with accidentally discovered hepatomegaly. He had generalised ichthyosis with dark skin pigmentation and bilateral ectropion. Abdominal examination revealed generalised abdominal distention with firm nontender hepatomegaly. His liver functions were deranged. Blood film showed many vacuolated neutrophils. Serum triglyceride and creatine kinase levels were elevated. Abdominal ultrasound showed a moderately enlarged liver with a bright echo pattern. Liver biopsy revealed marked diffuse macrovesicular fatty changes. The diagnosis of Chanarin-Dorfman Syndrome was made based on the dermatological, haematological, and liver biopsy findings.


Subject(s)
Ichthyosiform Erythroderma, Congenital/diagnosis , Lipid Metabolism, Inborn Errors/diagnosis , Muscular Diseases/diagnosis , Child, Preschool , Creatine Kinase/blood , Hepatomegaly/etiology , Humans , Liver Function Tests , Male , Neutrophils/pathology , Triglycerides/blood , Vacuoles
6.
Hepatogastroenterology ; 61(132): 1090-3, 2014 Jun.
Article in English | MEDLINE | ID: mdl-26158170

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.


Subject(s)
End Stage Liver Disease/surgery , Liver Transplantation/methods , Living Donors , Transplant Recipients , Adolescent , Age Factors , Body Size , Child , Child, Preschool , Comorbidity , Egypt/epidemiology , End Stage Liver Disease/diagnosis , Female , Health Knowledge, Attitudes, Practice , Health Services Accessibility , Hospitals, Pediatric , Hospitals, University , Humans , Infant , Liver Transplantation/adverse effects , Living Donors/psychology , Male , Risk Factors , Time Factors , Treatment Outcome
7.
Blood Coagul Fibrinolysis ; 24(2): 113-7, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23314384

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 ±â€Š3.4 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.


Subject(s)
Blood Coagulation Factors/analysis , Blood Coagulation/physiology , Fibrinolysis/physiology , Liver Cirrhosis/blood , Liver Diseases/blood , Liver Function Tests/methods , Blood Coagulation Factors/metabolism , Case-Control Studies , Child , Chronic Disease , Female , Humans , Liver Cirrhosis/diagnosis , Liver Diseases/diagnosis , Male , Prospective Studies
8.
Eur J Radiol ; 81(11): 3120-3, 2012 Nov.
Article in English | MEDLINE | ID: mdl-22817846

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.


Subject(s)
Fatty Liver/diagnostic imaging , Fatty Liver/epidemiology , Overweight/diagnostic imaging , Overweight/epidemiology , Ultrasonography/statistics & numerical data , Adolescent , Child , Child, Preschool , Comorbidity , Egypt/epidemiology , Female , Humans , Male , Non-alcoholic Fatty Liver Disease , Prevalence , Reproducibility of Results , Sensitivity and Specificity
9.
Arab J Gastroenterol ; 13(4): 184-5, 2012 Dec.
Article in English | MEDLINE | ID: mdl-23432988

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.


Subject(s)
Hepatitis A/complications , Pancreatitis/diagnosis , Abdominal Pain/diagnostic imaging , Abdominal Pain/etiology , Acute Disease , Child , Cholangiopancreatography, Magnetic Resonance , Female , Hepatitis A/diagnosis , Humans , Pancreatitis/etiology , Ultrasonography
10.
Liver Int ; 32(3): 449-56, 2012 Mar.
Article in English | MEDLINE | ID: mdl-22098096

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.


Subject(s)
Hepatitis C/epidemiology , Hepatitis C/transmission , Age Factors , Child , Child, Preschool , Egypt/epidemiology , Female , Hepatitis C/genetics , Hepatitis C/immunology , Hepatitis C Antibodies/blood , Humans , Infant , Logistic Models , Male , Prevalence , RNA, Viral/genetics , Reverse Transcriptase Polymerase Chain Reaction , Risk Factors , Seroepidemiologic Studies
11.
Arab J Gastroenterol ; 12(3): 125-30, 2011 Sep.
Article in English | MEDLINE | ID: mdl-22055589

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.


Subject(s)
Ceruloplasmin/metabolism , Copper/urine , Hepatolenticular Degeneration/diagnosis , Liver Transplantation/methods , Penicillamine/therapeutic use , Adolescent , Biomarkers/blood , Biomarkers/urine , Chelating Agents/therapeutic use , Child , Child, Preschool , Diagnosis, Differential , Egypt , Female , Follow-Up Studies , Hepatolenticular Degeneration/metabolism , Hepatolenticular Degeneration/therapy , Humans , Liver/metabolism , Liver/pathology , Male , Retrospective Studies
12.
J Pediatr Hematol Oncol ; 33(7): e267-70, 2011 Oct.
Article in English | MEDLINE | ID: mdl-21941130

ABSTRACT

INTRODUCTION: In patients with portal hypertension, thrombocytopenia in cirrhotics and noncirrhotics is thought to be caused by sequestration and destruction of platelets within a large spleen, suppression of platelet production in the bone marrow, and decreased activity of the hematopoietic growth factor thrombopoietin (TPO). AIM: Determining the level of TPO in cirrhotic thrombocytopenic patients and correlate it to the degree of disease severity and platelet count. METHODS: A cross-sectional study conducted on 62 children; 25 cases with cirrhosis, 20 patients with extrahepatic portal vein obstruction (EHPVO), and 17 healthy age-matched and sex-matched controls. The severity of liver cirrhosis was graded according to the Child-Pugh classification. TPO was measured using the quantitative human TPO by enzyme-linked immunosorbant assay. RESULTS: Serum TPO levels were significantly lower in the cirrhotic group compared with both EHPVO patients and healthy controls (P=0.01 for each). Both of the Child-Pugh B and C cirrhotic cases had significantly lower TPO levels compared with Child A cases (P=0.003). We found a significant positive correlation between platelet count and serum TPO level (r=0.56, P=0.004) in the cirrhotic group but not in the EHPVO group (r=0.1, P>0.05). CONCLUSIONS: TPO underproduction contributes to thrombocytopenia in children with cirrhosis; whereas in children with EHPVO, TPO production is unaffected and thrombocytopenia is secondary to hypersplenism. TPO receptor agonists may be useful to improve platelet counts in the former group.


Subject(s)
Hypertension, Portal/blood , Liver Cirrhosis/blood , Portal Vein/pathology , Thrombocytopenia/blood , Thrombopoietin/blood , Adolescent , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Hypertension, Portal/diagnosis , Infant , Liver Cirrhosis/diagnosis , Male , Platelet Count , Severity of Illness Index , Thrombocytopenia/diagnosis
13.
World J Pediatr ; 7(3): 224-31, 2011 Aug.
Article in English | MEDLINE | ID: mdl-21633861

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.


Subject(s)
Tyrosinemias , Child, Preschool , Cyclohexanones/therapeutic use , Egypt , Enzyme Inhibitors/therapeutic use , Female , Humans , Infant , Liver Transplantation , Male , Nitrobenzoates/therapeutic use , Tyrosinemias/diagnosis , Tyrosinemias/therapy
14.
Trop Gastroenterol ; 32(4): 267-72, 2011.
Article in English | MEDLINE | ID: mdl-22696906

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.


Subject(s)
Aspartate Aminotransferases/blood , Biopsy, Needle , Hepatitis C, Chronic/diagnosis , Liver/pathology , Platelet Count , Adult , Child , Clinical Enzyme Tests , Female , Hepatitis C, Chronic/complications , Humans , Liver Cirrhosis/complications , Liver Cirrhosis/diagnosis , Male
15.
Eur J Pediatr ; 169(6): 689-93, 2010 Jun.
Article in English | MEDLINE | ID: mdl-19882170

ABSTRACT

UNLABELLED: 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.


Subject(s)
Cyclohexanones/administration & dosage , Enzyme Inhibitors/administration & dosage , Nitrobenzoates/administration & dosage , Tyrosinemias/drug therapy , Child, Preschool , Cost-Benefit Analysis , Cyclohexanones/economics , Dose-Response Relationship, Drug , Drug Costs , Egypt , Enzyme Inhibitors/economics , Female , Heptanoates/blood , Humans , Infant , Nitrobenzoates/economics
16.
World J Gastroenterol ; 13(20): 2846-51, 2007 May 28.
Article in English | MEDLINE | ID: mdl-17569121

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.


Subject(s)
Hepatitis C/complications , Liver Cirrhosis/virology , Liver/pathology , Adolescent , Alanine Transaminase/metabolism , Bilirubin/blood , Biopsy , Child , Child, Preschool , Cross-Sectional Studies , Disease Progression , Egypt , Female , Hepacivirus/pathogenicity , Hepatitis C/ethnology , Hepatitis C/pathology , Humans , Incidence , Infant , Liver/metabolism , Liver/virology , Liver Cirrhosis/ethnology , Liver Cirrhosis/metabolism , Male , Risk Factors
17.
Indian J Pediatr ; 74(3): 279-81, 2007 Mar.
Article in English | MEDLINE | ID: mdl-17401268

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.


Subject(s)
Cholestasis/complications , Pruritus/drug therapy , Pruritus/etiology , Rifampin/therapeutic use , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Rifampin/adverse effects
18.
World J Gastroenterol ; 12(45): 7337-40, 2006 Dec 07.
Article in English | MEDLINE | ID: mdl-17143952

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.


Subject(s)
Hepatitis A Vaccines/standards , Hepatitis A Vaccines/therapeutic use , Liver Diseases/immunology , Adolescent , Alanine Transaminase/blood , Antibody Formation , Child , Child, Preschool , Female , Hepatitis A/immunology , Hepatitis A Antibodies/blood , Humans , Liver Function Tests , Male , Patient Selection , Safety
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