Off-label use of muscular VSD device for closure of a rare congenital portosystemic shunt.

BACKGROUND: Congenital portosystemic shunt (CPSS) is a vascular malformation in which portal blood drains toward the systemic circulation, leading to pulmonary hypertension. CASE PRESENTATION: A 10-year-old patient was brought for evaluation because of dyspnea on exertion. Echocardiography revealed a pulmonary hypertension of 75 mmHg, and multi-slice CT angiography revealed the presence of a CPSS. Closure was finally implemented using a muscular ventricular septal defect device. Follow-up of the patient revealed a gradual decline in pulmonary hypertension. CONCLUSIONS: CPSS is an overlooked cause of reversible pulmonary hypertension (PH). Closure of such lesions and reversal pulmonary hypertension are possible via catheterization. The preferred device type depends largely on the intervening team. Plugs are the first choice for interventional radiologists, while ventricular and atrial septal occluder devices and duct occluders are preferred by pediatric cardiologists.

Effect of medium chain triglycerides enriched formula on growth of biliary atresia patients after Kasai portoenterostomy.

BACKGROUND AND STUDY AIMS: Biliary atresia (BA) is the most common cause of neonatal cholestasis, negatively affecting nutritional status, growth, and development. It is the most frequent paediatric indication for liver transplantation. The Kasai portoenterostomy (KPE) operation is an effective procedure with favourable outcomes when performed before two months of age. The present study aimed to assess the nutritional status of patients with biliary atresia who underwent the Kasai operation and to evaluate the effectiveness of nutritional counselling using medium-chain triglyceride (MCT) formulas and proper supplementation on their nutritional status, growth, and vitamin D levels. PATIENTS AND METHODS: This prospective observational study included 36 infants with biliary atresia who underwent Kasai portoenterostomy. All patients underwent clinical assessment, anthropometric evaluation, nutritional counselling, and an evaluation of vitamin D levels. Only compliant patients (22/36) were followed up after 3 and 6 months of nutritional counselling. RESULTS: Z-scores for weight, triceps skinfold thickness, and mid-upper arm circumference improved significantly after three months, and the height velocity Z-score improved after six months of nutritional counselling using an MCT-containing formula and supplementations. Patients who showed an improvement in cholestasis had better responses. The initial assessment revealed low serum levels of 25-hydroxyvitamin D in 77.8 %, which increased significantly (p = 0.012). CONCLUSION: Dietary intervention and supplementation with MCT and micronutrients can improve the nutritional status of children with BA following KPE.

Mitochondrial 3-hydroxymethylglutaryl-CoA synthase-2 (HMGCS2) deficiency: a rare case with bicytopenia and coagulopathy.

Mitochondrial 3-hydroxymethylglutaryl-CoA synthase-2 (HMGCS2) is the main enzyme involved in ketogenesis. It is an essential enzyme for the catalysis of ß-oxidation-derived-acetyl-CoA and acetoacetyl Co-A to produce ß-hydroxy-ß-methylglutaryl-CoA (HMG-CoA) and free coenzyme A.The deficiency of this enzyme (3-hydoxy-3-methylglutaryl-CoA synthase) is a very rare metabolic disorder with limited cases described in the literature. The manifestations of this disease include hypoketotic hypoglycaemia, metabolic acidosis, lethargy, hepatomegaly with fatty liver and encephalopathy.We report a middle childhood male who presented with hepatosplenomegaly, lymphadenopathy and bicytopenia. The case was diagnosed by the whole exome sequencing which revealed a homozygous missense variant of uncertain significance in HMGCS2 gene.

Clinical experience with hepatorenal tyrosinemia from a single Egyptian center.

Although very recently, in Egypt, sick newborn screening has included screening for hepatorenal tyrosinemia, yet, it is not yet included in nationwide neonatal screening and hence diagnosis may be delayed. The aim of this study was to analyze data of all cases presenting with hepatorenal tyrosinemia to the Pediatric Hepatology Unit, Cairo University, Egypt from 2006 to 2019. Data were retrieved from patients' files including age of onset of symptoms, clinical signs, blood counts, liver functions, serum phosphorous, alpha-fetoprotein, succinylacetone and abdominal ultrasound. During this period, 76 patients were diagnosed with hepatorenal tyrosinemia if succinylacetone in dry blood spot was elevated above 1 µmol/L. These 76 cases came from 70 families; consanguinity was reported in 61 families. In our cohort we reported 30 affected siblings with a similar clinical presentation, who died undiagnosed. Presentation was acute in 26%, subacute in 30% and chronic in 43%. Abdominal distention was the commonest presenting symptom (52.6%). Coagulopathy was the commonest derangement in liver functions; hyperbilirubinemia and raised transaminases were less common. Ultrasound findings included hepatic focal lesions in 47% and enlarged echogenic kidneys in 39% and 45.3% respectively. Only 20 children were treated with Nitisinone because of unavailability and high costs; seven out of them underwent liver transplantation. In conclusion, although hepatorenal tyrosinemia is a rare inborn error of metabolism, in a large population country with high rate of consanguinity; this disease is not uncommonly diagnosed. The current treatment is not readily available because of the costs in a resource-limited country. Neonatal screening and subsidization of the costly medication need to be considered.

Autoimmune hepatitis in Egyptian children: A single center experience.

BACKGROUND AND AIM: Autoimmune hepatitis (AIH) has variable clinical manifestations and should be considered in the diagnostic work-up of any patient with cryptogenic liver disease. The aim of the study was to determine the clinical, biochemical, histopathological characteristics and treatment outcome of AIH in Egyptian children. PATIENTS AND METHODS: This observational study was conducted at the Pediatric Hepatology Unit at Cairo University Pediatric Hospital, Egypt. All children (<18 years of age) presenting from 2009 to 2016 with established diagnosis of AIH were included. Medical history, clinical examination, and results of investigations were retrieved from patients' files. The main outcome measures included the rate of remission, relapses, and mortality. RESULTS: The study included 34 children with AIH. Twenty patients (58%) presented with chronic liver disease. There was a history of concomitant autoimmune diseases in 5 patients. Transaminases were elevated in all patients. There was synthetic dysfunction in 58%. Twenty-four patients (70.5%) had AIH-1, while nine patients (26.4%) had AIH-2 and one patient (2.9%) had autoantibody negative AIH. Piecemeal necrosis was observed in the liver biopsy of 79% of our cohort. Approximately 80% achieved biochemical remission (88% received combined therapy of prednisolone and azathioprine). About half of the patients developed relapses. One patient died of liver cell failure. CONCLUSION: In children with liver disease, a diagnosis of AIH should be considered. In those patients, AIH-1 is more common than AIH-2. Prednisolone monotherapy or combined with azathioprine could achieve remission, but relapse is still common. Treatment non-adherence is the main risk factor for relapse.

Covid-19 pandemic in Egyptian children with liver diseases: Incidence and impact on health care service delivery in a low/middle income country.

BACKGROUND AND STUDY AIMS: The coronavirus disease 2019 (COVID-19) pandemic has had considerable effects on health care services given the need for re-allocation of resources and interruption of medical care. COVID-19 poses a challenge to patients with liver disease who are at risk of infection and more severe disease course. The current study aimed to assess the incidence of COVID-19 in children with liver diseases and evaluate the extent to which health care delivery was affected during lockdown. PATIENTS AND METHODS: This cross-sectional analytical study conducted at the Pediatric Hepatology Unit, Cairo University Children's Hospital utilized a questionnaire to determine the incidence of COVID-19 in patients with liver diseases and the impact of COVID-19 on the patients' liver condition and health care service delivery. A presumed score was implemented to identify patients with probable COVID-19. RESULTS: Data from 349 children with liver diseases were analyzed. The overall incidence of COVID-19 was 8%. Patients with documented and probable COVID-19 were compared to improbable COVID-19 cases. Notably, COVID-19 cases were younger and had higher incidence rates of cholestatic liver diseases. COVID-19 patients experienced significantly higher rates of hepatic complications (43%) and had significantly greater need for medical services during the lockdown. All COVID-19 patients recovered after a median (IQR) duration of 3 (4) days, except for one patient who succumbed to COVID-19 and hepatic complications. CONCLUSIONS: COVID-19 affected the younger hepatic patients with cholestatic disorders of infancy. Hepatic complications were more common among COVID-19 infected children. Alternative ways of communication require development to prioritize patients who needs a hospital visit and monitoring. Clinical scores may help diagnosis of COVID-19 in low/middle income countries like Egypt to compensate for the deficient laboratory diagnostic facilities.

A study of exons 14, 15, and 24 of the ABCB11 gene in Egyptian children with normal GGT cholestasis.

BACKGROUND AND STUDY AIMS: Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare inherited disorder caused by mutation in the ATP-binding cassette subfamily B member 11 gene (ABCB11) that encodes the bile salt export pump (BSEP), which is the main transporter of bile acids from hepatocytes to the canalicular lumen. Defects in BSEP synthesis and/or function lead to reduced bile salt secretion followed by accumulation of bile salts in hepatocytes and hepatocellular damage. This study aimed to detect variations in exons 14, 15, and 24 of the ABCB11 gene in patients with suspected PFIC2 among a group of Egyptian infants and children with normal gamma-glutamyl transpeptidase (GGT) cholestasis. PATIENTS AND METHODS: This observational case-control study was conducted on 13 children with suspected PFIC2 and 13 healthy subjects as controls. Genotyping of the ABCB11 gene was performed via DNA extraction followed by PCR amplification, purification, and then sequencing analysis of exons 14, 15, and 24 of the ABCB11 gene. RESULTS: The study detected two single nucleotide variations, c.1638+ 32T > C (rs2241340) in exon 14 and c.3084A > G (p.Ala1028 = ) (rs497692) in exon 24 of the ABCB11 gene. No variations were identified in exon 15. CONCLUSION: The study revealed two benign variants involving exons 14 and 24 of the ABCB11 gene. Exons 14, 15, and 24 are not hot spots for common mutations in Egyptian PFIC2 patients. Further study of other exons of the ABCB11 gene is necessary to confirm the diagnosis of PFIC2.

Resuming post living donor liver transplantation in the COVID-19 pandemic: real-life experience, single-center experience.

BACKGROUND: Solid organ transplantation (SOT) service has been disrupted during the current coronavirus disease 2019 (COVID-19) pandemic, which deferred the service in most centers worldwide. As the pandemic persists, there will be an urgency to identify the best and safest practices for resuming activities as areas re-open. Resuming activity is a difficult issue, in particular, the decision of reopening after a period of slowing down or complete cessation of activities. OBJECTIVES: To share our experience in resuming living donor liver transplantation (LDLT) in the context of the COVID-19 pandemic in the Liver Transplantation Unit of El-Manial Specialized Hospital, Cairo University, Egypt, and to review the obstacles that we have faced. MATERIAL AND METHODS: This study is a single-center study. We resumed LDLT by the 26th of August 2020 after a period of closure from the 1st of March 2020. We have taken a lot of steps in order to prevent COVID-19 transmission among transplant patients and healthcare workers (HCWs). RESULTS: In our study, we reported three LDLT recipients, once resuming the transplantation till now. All our recipients and donors tested negative for SARS-CoV-2 by nasopharyngeal RT-PCR a day before the transplantation. Unfortunately, one of them developed COVID-19 infection. We managed rapidly to isolate him in a single room, restricting one team of HCWs to deal with him with strict personal protective measures. Finally, the patient improved and was discharged in a good condition. The second patient ran a smooth course apart from FK neurotoxicity which improved with proper management. The third patient experienced a sharp rise in bilirubin and transaminases on day 14 that was attributed to drug toxicity vs. rejection and managed by discontinuing the offending drugs and pulse steroids. In addition, one of our head nurses tested positive for SARS-CoV-2 that was manageable with self-isolation. CONCLUSION: Careful patient, donor, personnel screening is mandatory. Adequate supply of personal protective equipments, effective infection control policies, and appropriate administrative modifications are needed for a safe return of LDLT practice.

Improvement in Liver Stiffness in Pediatric Patients with Hepatitis C Virus after Treatment with Direct Acting Antivirals.

OBJECTIVES: To assess the degree of liver stiffness using transient elastography in Egyptian children infected with hepatitis C virus (HCV) at baseline and 1 year after achievement of sustained virologic response (SVR) with direct acting antivirals. STUDY DESIGN: This prospective study included children infected with HCV who received treatment with sofosbuvir/ledipasvir and achieved SVR. At baseline and 1 year after achievement of SVR, the extent of hepatic fibrosis was assessed by transient elastography using FibroScan to measure liver stiffness, in addition to noninvasive markers including aspartate aminotransferase/platelet ratio index (APRI) and fibrosis-4 (FIB-4) index. RESULTS: The study included 23 cases that had variable degrees of fibrosis at baseline; their ages ranged between 10 and 18 years. At baseline, 13 patients had F1; 3 patients had F1-F2; 1 patient had F2; 3 patients had F3; 2 had F3-F4; and 2 patients with F4. One year after achievement of SVR, there was a statistically significant improvement in liver stiffness, APRI, and FIB-4 index (P = .03, <.001, .02, respectively). In 13 patients (56.5%), the liver stiffness improved; in 7 patients, it was stationary; and the remaining 3 patients showed mild increase in liver stiffness that was, however, associated with improvement in APRI and FIB-4 index. Comorbid conditions and previous treatment with interferon were not associated with increased liver stiffness 1 year after SVR. CONCLUSIONS: Egyptian children infected with HCV genotype 4 achieved significant regression in liver stiffness after treatment with direct acting antivirals.

Predictors of Short-Term Outcome of Kasai Portoenterostomy for Biliary Atresia in Infants: a Single-Center Study.

PURPOSE: The outcome predictors of Kasai portoenterostomy (KPE) for biliary atresia (BA) are controversial. This study aimed to identify possible short-term outcome predictors of KPE for BA in infants. METHODS: This retrospective study included infants with BA who underwent KPE between January 2015 and December 2017 and were followed up for at least 6 months after surgery at the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt. The short-term outcome was jaundice clearance within 6 months following surgery. All data were compared between the jaundice free group and those with persistent jaundice to identify the predictors of jaundice clearance. RESULTS: The study included 75 infants. The mean age at the time of surgery was 82.43±22.77 days (range, 37-150 days), and 28 (37.3%) infants cleared their jaundice within 6 months postoperative. Age at surgery did not significantly affect the outcome (p=0.518). Infants with persistent jaundice had significantly higher pre-operative levels of aspartate aminotransferase (AST) than those who were jaundice free (p=0.041). Receiver operating characteristic curve analysis showed that preoperative AST ≤180 IU/L was predictive of a successful KPE, with sensitivity 74.5% and specificity 60.7%. Infants with bile plugs in liver biopsy had a 6-fold higher risk of persistent jaundice than those without bile plugs (95% confidence interval: 1.59-20.75, p=0.008). CONCLUSION: Jaundice clearance after KPE for BA can be predicted using preoperative AST and presence of bile plugs in liver biopsy.

Secondary hepatic dysfunction in pediatric intensive care unit: Risk factors and outcome.

BACKGROUND: Hepatic dysfunction has a significant role in intensive care unit patients' morbidity and mortality. AIM: To study the frequency, risk factors and outcome of secondary hepatic dysfunction in children admitted to the pediatric intensive care unit. METHODS: Secondary hepatic dysfunction was defined as the development of abnormal liver functions in a patient without a previous liver disease during intensive care unit stay. The following data were collected: age, gender, indication of admission, type of organ dysfunction, presence of sepsis, shock, need for inotropic support or mechanical ventilation, administered medications and mortality scores. Liver function tests were done on admission and at 7-day intervals. RESULTS: One hundred and fifty-one patients were included. Forty-three (28.5%) acquired secondary hepatic dysfunction. Several risk factors were significantly associated with secondary hepatic dysfunction: sepsis (p<0.001), cardiovascular events (p<0.001), hypoxia (p<0.001), number of administered antibiotics (P = 0.001), use of inotropes (p<0.001) and mechanical ventilation (p = 0.001). Secondary hepatic dysfunction was significantly associated with mortality and prolonged length of stay (P=<0.001). CONCLUSION: Secondary hepatic dysfunction is a common finding in the pediatric intensive care unit. Sepsis, cardiovascular events and hypoxia, are the main risk factors for secondary hepatic dysfunction. Mortality and prolonged length of stay are strongly related to secondary hepatic dysfunction.

Drug-Drug Interactions in Children and Adolescents Receiving Ledipasvir/Sofosbuvir for the Treatment of Hepatitis C Virus Infection.

BACKGROUND AND OBJECTIVE: Drug-drug interactions need to be considered to optimize the pharmacotherapeutic outcome of direct-acting antivirals. The aim of this study was to report on possible drug-drug interactions between ledipasvir/sofosbuvir and other medications received by children and adolescents with hepatitis C virus, in addition to suggested management for these drug-drug interactions. METHODS: Hepatitis C virus-infected children and adolescents, 12-17 years of age and/or weighing ≥ 35 kg, who presented to the Pediatric Hepatology Unit at Cairo University Pediatric Hospitals for ledipasvir/sofosbuvir treatment were included. Medication history was taken including long-term medications for chronic conditions and on-demand medications for inter-current illnesses. Medications were reviewed by the Kasr Alainy Drug Information Center to identify possible drug-drug interactions with prescribed ledipasvir/sofosbuvir and their management. HEP Drug Interactions provided by the University of Liverpool, Lexicomp®, and Medscape were the utilized references. Each drug-drug interaction was assigned a risk rating of A, B, C, D, or X. RESULTS: Sixty hepatitis C virus-infected children and adolescents assigned to receive ledipasvir/sofosbuvir were enrolled. Thirty percent of patients had associated chronic co-morbid conditions. The overall number of medications received was 48; 39 were prescribed as long-term medications with a median of 3 (interquartile range 4.24) medications per patient. Proton pump inhibitors, antacids, histamine H2 receptor antagonists, sodium bicarbonate, and colchicine were reported to be associated with a drug-drug interaction risk D necessitating therapy modification, which occurred prior to administration. CONCLUSIONS: Early identification and prompt response to drug-drug interactions with the aid of pharmacists optimize the pharmacotherapeutic outcome and eliminate possible morbidities when using direct-acting antivirals in children and adolescents with hepatitis C virus.

Urinary abnormalities in children and adolescents with Wilson disease before and during treatment with d-penicillamine.

BACKGROUND AND AIM: Renal abnormalities can occur at any time point during the course of Wilson disease (WD). We aimed to fill a literature gap in this respect by studying urinary abnormalities in children and adolescents with WD. METHODS: This study included 60 children with WD presenting to the Pediatric Hepatology Unit, Cairo University. The following data were retrieved from the patients' files including age, sex, liver function tests, serum ceruloplasmin, 24-h urinary copper, serum creatinine, blood urea nitrogen, urinalysis, urinary albumin/creatinine ratio, urinary calcium/creatinine ratio, urinary ß2-microglobulin, liver and renal biopsy results when available. RESULTS: All studied cases had no symptoms related to renal involvement. Microscopic hematuria was detected in 11% and 12% at baseline and within 5 years of therapy, respectively. Moderate microalbuminuria was detected in 34%, 50%, and 33% at baseline, within 5 years and > 5 years after therapy, respectively. Hypercalciuria was detected in 23% at baseline, 34% in those patients treated for up to 5 years and 37.5% > 5 years of therapy. Age and international normalized ratio were significantly higher in patients with high calcium/creatinine ratio compared with those with normal values at initial evaluation. Frequency of elevated urinary ß2-microglobulin was 36%, 36%, and 37% in patients at baseline, up to 5 years and > 5 years of therapy, respectively. CONCLUSION: Asymptomatic urinary abnormalities are present in patients with WD at any time point of the disease and during treatment with d-penicillamine. They have to be searched for, as early intervention may prevent progression to renal insufficiency.

Is sofosbuvir/ledipasvir safe for the hearts of children with hepatitis C virus?

BACKGROUND: Symptomatic bradycardia has been reported in adults treated for chronic hepatitis C using sofosbuvir based regimens. AIM: We studied the cardiac safety of sofosbuvir/ledipasvir in Egyptian children, treated for chronic hepatitis C. METHODS: The study included 40 hepatitis C virus infected children and adolescents 12-17 years old, using the combination of sofosbuvir (400 mg)/ledipasvir (90 mg) in a single oral tablet (Harvoni) taken daily for 12 weeks. All subjects underwent a baseline standard 12-lead surface Electrocardiography that was repeated at 4 and 12 weeks of therapy. Electrocardiography parameters (Heart Rate, RR interval, PR interval, QRS, QT interval, corrected QT interval, QT dispersion, JT interval, corrected JT interval, JT dispersion, Tpeak-Tend interval) were compared at the 3 different time points during antiviral therapy. RESULTS: No symptoms related to the cardiovascular system were reported during treatment. There were no cases of symptomatic bradycardia/syncope. Heart rate was noted to be significantly lower and RR and QT intervals were significantly longer in the baseline electrocardiography. Heart rate was significantly lower and RR interval was significantly longer in patients with higher viral load. CONCLUSION: No adverse cardiovascular events were observed in this group of HCV infected children and adolescents treated with sofosbuvir/ledipasvir. None of the patients developed bradyarrhythmias during treatment.

Paediatric chronic hepatitis B virus infection: are children too tolerant to treat?

AIM: Guidelines for managing the hepatitis B (HB) virus infection in children are still evolving. We aimed to assess the eligibility of children with HB virus infections for treatment based on the current guidelines. METHODS: This observational study took place in 2016 and focused on children with isolated chronic HB infections, who attended the paediatric hepatology units at two centres in Egypt. We recruited all treatment-naïve children aged one year to 18 years who had completed at least 12 months of follow-up. RESULTS: The study comprised 103 children aged between 1.5-18 years. Of these, 51 (50%) had the HB e antigen-positive chronic infection, 28 (27%) had the HB-negative chronic infection, 11 (11%) had the HB e antigen-positive chronic hepatitis and none had the HB e antigen-negative chronic hepatitis. The remaining 13 (12%) children did not fulfil the criteria for chronic HB definitions. Only two of the children were candidates for treatment: both had HB e antigen-positive chronic hepatitis and had undergone liver biopsies. CONCLUSION: Only two of the 103 children with chronic HB were eligible for treatment according to the current guidelines and every measure should be taken to prevent the HB virus infection in children.

Routine analysis of ascitic fluid for evidence of infection in children with chronic liver disease: Is it mandatory?

Ascitic fluid infection is a major cause of morbidity and mortality in cirrhotic patients, requiring early diagnosis and therapy. We aimed to determine predictors of ascitic fluid infection in children with chronic liver disease. The study included 45 children with chronic liver disease and ascites who underwent 66 paracentesis procedures. Full history taking and clinical examination of all patients were obtained including fever, abdominal pain and tenderness and respiratory distress. Investigations included: complete blood count, C-reactive protein, full liver function tests, ascitic fluid biochemical analysis, cell count and culture. Our results showed that patients' ages ranged between 3 months to 12 years. Prevalence of ascitic fluid infection was 33.3%. Gram-positive bacteria were identified in six cases, and Gram-negative bacteria in five. Fever and abdominal pain were significantly more associated with infected ascites (p value = 0.004, 0.006). Patients with ascitic fluid infection had statistically significant elevated absolute neutrophilic count and C-reactive protein. Logistic regression analysis showed that fever, abdominal pain, elevated absolute neutrophilic count and positive C-reactive protein are independent predictors of ascitic fluid infection. Fever, elevated absolute neutrophilic count and positive C-reactive protein raise the probability of ascitic fluid infection by 3.88, 9.15 and 4.48 times respectively. The cut-off value for C-reactive protein for ascitic fluid infection was 7.2 with sensitivity 73% and specificity of 71%. In conclusion, prevalence of ascitic fluid infection in pediatric patients with chronic liver disease and ascites was 33.3%. Fever, abdominal pain, positive C-reactive protein and elevated absolute neutrophilic count are strong predictors of ascitic fluid infection. Therefore an empirical course of first-line antibiotics should be immediately started with presence of any of these predictors after performing ascitic fluid tapping for culture and sensitivity. In absence of these infection parameters, routine ascitic fluid analysis could be spared.

Sustained Viral Response in Genotype 4 Chronic Hepatitis C Virus-infected Children and Adolescents Treated With Sofosbuvir/Ledipasvir.

OBJECTIVES: Recently, direct acting antivirals (DAAs), sofosbuvir (SOF) combined with ledipasvir (LED), were approved for treatment of hepatitis C virus (HCV)-infected children 12 years of age and older or weighting at least 35 kg for all HCV genotypes. The aim of this study was to assess the safety and efficacy of SOF/LED in genotype 4 HCV-infected Egyptian children and adolescents. METHODS: This observational study included 40 consecutive HCV-infected children of age 12 to <18 years old or weighing >35 kg, both treatment-naive and treatment-experienced. All of the children were hepatitis B virus-negative and had normal renal functions and heart rate. Patients received oral, fixed-dose combination tablet of SOF/LED (400 mg SOF, 90 mg LED [Harvoni]) once daily for 12 weeks. Potential side effects were recorded at weeks 4, 8, and 12 weeks of treatment. The study primary outcome was sustained virological response 12 weeks (SVR12) after end-of-treatment. RESULTS: The study included 40 children and adolescents, 24 were boys (60%); their age ranged between 11.5 and 17.5 years (mean 13.9 ±â€Š1.5). Baseline viral load ranged between 9630 and 24,600,000 IU/mL. HCV RNA became negative in 39 patients (97.5%) at 4 weeks and in all patients (100%) at weeks 8, 12, and SVR12. Asthenia was the commonest side effect, reported in 52.5% followed by headache in 47.5%. CONCLUSIONS: Treatment with all-oral DAAs (SOF/LED) for 12 weeks was well tolerated in Egyptian children and adolescents infected with genotype 4 HCV, with 100% SVR12 and negligible side effects.

Copper concentrations in Egyptian infants with cholestasis: A single center study.

BACKGROUND AND STUDY AIMS: Hepatobiliary cholestatic disorders produce excess copper (Cu) retention in the liver, which is toxic and may cause hepatitis, fulminant hepatic failure, cirrhosis and death. In this study, we measured hepatic Cu and tested its correlation with serum Cu (S. Cu) and serum ceruloplasmin (S. ceruloplasmin) in cholestatic infants. PATIENTS AND METHODS: 41 cholestatic infants were enrolled as cases and 11 healthy infants as control subjects. S. Cu and S. ceruloplasmin were done for all infants and hepatic Cu was measured in the liver specimen in cases. RESULTS: Cases were 63.5% males with their age ranging between 1 and 7 months, while control subjects were 45.5% males with an age range between 3 and 18 months. Among cases, 41.5% had biliary atresia and 58.5% had intrahepatic cholestasis. Cholestatic infants had significantly higher levels of S. Cu and S. ceruloplasmin than control subjects and their hepatic Cu concentration was significantly higher than literature control. Infants with biliary atresia showed higher levels of Cu indices, with no statistical significance. Serum and hepatic Cu levels positively correlated with each other and with S. ceruloplasmin. Results of ROC curve showed that S. Cu was highly sensitive and specific for predicting hepatic Cu concentration at cut-off 181 µg/dl. CONCLUSION: Serum and hepatic Cu concentrations were markedly elevated in patients with cholestasis and positively correlated with each other and with S. ceruloplasmin. S. Cu level can predict hepatic Cu concentration.

Helicobacter pylori infection in children with portal hypertensive gastropathy.

BACKGROUND: Data about the association of Helicobacter pylori (H. pylori) and portal hypertensive gastropathy (PHG) are scarce in children. The present study aimed to fill the knowledge gap in this area. METHODS: The prevalence of H. pylori infection was studied in a group of infants and children with PHG using rapid urease test and histological demonstration of H. pylori in gastric mucosal biopsy obtained by upper gastrointestinal endoscopy. The results were compared to a control group who underwent endoscopy for other indications mainly hematemesis and/or dyspepsia. RESULTS: H. pylori was equally prevalent in both groups (~60%). Children with PHG were significantly stunted in height, had significantly lower hemoglobin, platelets and serum iron. Severe PHG was associated with higher grade of esophageal varices. Within the group with PHG, H. pylori infection was associated with lower hemoglobin, serum iron and serum ferritin. Moderate to severe PHG was more associated with H. pylori infection. CONCLUSIONS: H. pylori infection was not more commonly associated with PHG, however, it might contribute to the severity of PHG. The synergistic effect of PHG and H. pylori infection might contribute to the retarded growth and iron deficiency status noted in this group.

Focal hepatic lesions in Egyptian infants and children: the pediatric hepatologist perspective.

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.