Serologic, endoscopic and pathologic findings in pediatric celiac disease: A single center experience in a low/middle income country.

BACKGROUND: Studies in Africa, Asia, and Latin America are needed to provide a comprehensive picture of the global incidence of celiac disease (CD). AIM: To describe the serology, endoscopic and histological findings in typical and atypical presentations of pediatric CD at a tertiary referral hospital in an African low/middle income country (LMIC). METHODS: This observational study was conducted on 199 patients with CD from 2010 to 2019. The patients were divided into typical and atypical groups according to the presenting symptoms including 120 and 79 patients respectively. Serology, upper gastrointestinal endoscopy with duodenal biopsy were performed for patients who had symptoms suggestive of CD. The severity of the intestinal damage was graded according to the histo-pathologic Marsh-Oberhuber classification. RESULTS: Chronic diarrhea was the main intestinal presentation in the typical group. Anemia was the most common extraintestinal symptom in both the typical and atypical group. Marsh-Oberhuber type 3b and 3c was significantly higher in the seropositive patients with a P value of 0.007. A significant correlation was observed between the histological grade of the biopsied duodenal mucosa and the clinical presentation (P < 0.001). Age was significantly higher in the atypical group (P value < 0.001). CONCLUSION: Although typical CD was observed in 120 patients in this study, the clinical variability of the condition was frequently observed. Age only was a significant predictor for the appearance of atypical CD. Therefore, CD presentations in LMIC are not different from industrialized countries.

Hepatobiliary manifestations in children with inflammatory bowel disease: A single-center experience in a low/middle income country.

BACKGROUND: There has been a worldwide increase in the reported incidence of inflammatory bowel disease (IBD) in children over the past 2-3 decades. The hepatobiliary (HB) manifestations of IBD have been well-studied in children in industrialized and developed countries but are infrequently reported in low- and middle-income countries (LMIC) such as Egypt. AIM: To determine the prevalence of the HB manifestations in a cohort of Egyptian children with IBD. METHODS: This cross-sectional observational study was carried out over a period of 6 mo (between June 2013 to December 2013) at the Paediatric Hepatology and Gastroenterology Units of Cairo University Children's Hospital, which is the largest paediatric tertiary care centre in the country. RESULTS: The study included 48 patients with confirmed IBD based upon clinical, laboratory, endoscopic and histopathological features, 29 (60.4%) were male. Twenty-four patients (50%) had ulcerative colitis (UC), 11 (22.9%) had Crohn's disease (CD) and 13 (27.1%) had unclassified-IBD (IBD-U), which was formerly known as indeterminate colitis. The mean age of the patients at the time of presentation was 8.14 (± SD 4.02) years and the mean age at the time of study enrolment was 10.16 (± SD 4.19) years. All patients were screened for HB manifestations by physical examination, liver function tests, imaging and liver biopsy when indicated. HB disorders were confirmed in 13 patients (27.1%). Transaminases were elevated in 3 patients (6.3%). Two patients (4.2%) had elevated biliary enzymes (one was diagnosed as primary sclerosing cholangitis (PSC) and the other was diagnosed with PSC/autoimmune hepatitis overlap syndrome and the third patient had hepatitis C virus infection. Ten patients (20.8%) had bright echogenic liver on ultrasound suggesting fatty infiltration as a sequel of malnutrition or medication toxicity. CONCLUSION: The commonest HB disorders in Egyptian children with IBD were abnormal liver function tests, fatty infiltration and PSC. These HB manifestations in paediatric patients in LMIC may be relatively more common than in industrialized countries. Therefore, IBD patients in LMIC should be meticulously screened for liver disease to allow prompt diagnosis and management.

Effects of dual sofosbuvir/daclatasvir therapy on, chronic hepatitis C infected, survivors of childhood malignancy.

BACKGROUND: Childhood cancer survivors are potentially at a higher risk of infection with hepatitis C virus (HCV). The effects of all-oral direct-acting antiviral therapy (DAA) on both the HCV infection as well as the state of cancer remission have not been well investigated in this population. AIM: To test the effects of dual sofosbuvir/daclatasvir (SOF/DCV) therapy in the treatment of chronic HCV in survivors of hematologic malignancy in pediatric age group. METHODS: We conducted a prospective, uncontrolled, open-label multicenter study. A total of 20 eligible, chronic HCV, genotype-4, infected children who had been in continuous complete remission from hematologic cancer (leukemia/lymphoma) for at least one year were included in the study. All patients were treated with combined SOF/DCV for 12 wk. Patients were monitored throughout the study till 12 wk after end of treatment for safety and efficacy outcomes including the sustained virologic response 12 (SVR12) rate, hematological indices, liver and kidney functions. RESULTS: The intent-to-treat SVR12 rate was 20 of 20 (100%; 95%CI: 84%-100%). All patients showed normalized liver enzymes from week-4. All hematological indices, liver and kidney functions were kept normal throughout the study. No fatalities or treatment-emergent serious or severe adverse events were reported throughout the study. CONCLUSION: SOF/DCV combined therapy could be used safely and effectively in the treatment of chronic HCV genotype-4 infection in leukemia/lymphoma treated children. No relapses were detected during treatment and throughout the follow up period for either the original malignant disease or the HCV infection.

Histopathological changes in the oesophageal mucosa in Egyptian children with corrosive strictures: A single-centre vast experience.

BACKGROUND: The caustic ingestion continues to be a major problem worldwide especially in developing countries. The long-term complications include stricture and increased life time risk of oesophageal carcinoma. Patients suffered from corrosive induced oesophageal strictures have more than a 1000-fold risk of developing carcinoma of the oesophagus. AIM: To determine the possibility of oesophageal mucosal dysplasia after prolonged dilatation in post corrosive stricture. METHODS: This observational study was conducted at the Paediatric Endoscopy Unit in Cairo University Children's Hospital. It included children of both sexes older than 2 years of age who had an established diagnosis of post-corrosive oesophageal stricture and repeated endoscopic dilatation sessions for more than 6 mo. All patients were biopsied at the stricture site after 6 mo of endoscopic dilatation. A histopathological examination of an oesophageal mucosal biopsy was performed for the detection of chronic oesophagitis, inflammatory cellular infiltration and dysplasia. RESULTS: The mean age of the enrolled children was 5.9 ± 2.6 years; 90% of the patients had ingested an alkaline corrosive substance (potash). The total number of endoscopic dilatation sessions were ranging from 16 to 100 with mean number of sessions was 37.2 ± 14.9. Histopathological examination of the specimens showed that 85% of patients had evidence of chronic oesophagitis (group A) in the form of basal cell hyperplasia, hyperkeratosis and subepithelial fibrosis. Thirteen percent of the patients had evidence of reactive atypia (group B) in the form of severe neutrophilic intraepithelial inflammatory cellular infiltration, and 2 patients (2%) had mild squamous dysplasia (group C); we rebiopsied these two patients 6 mo after the initial pathological assessment, guided by chromoendoscopy by Lugol's iodine. CONCLUSION: The histopathology of oesophageal mucosal biopsies in post-corrosive patients demonstrates evidence of chronic oesophagitis, intraepithelial inflammatory cellular infiltration and dysplasia. Dysplasia is one of the complications of post-corrosive oesophageal stricture.

Value of duplex Doppler ultrasonography in non-invasive assessment of children with chronic liver disease.

AIM: to investigate the value of duplex Doppler ultrasonography (US) in the assessment of the hemodynamics of the portal and hepatic veins in a cohort of children with chronic liver disease (CLD) and to detect any relationship between the US changes, etiology and severity (or stage) of CLD. METHODS: we prospectively enrolled 25 children with biopsy-proven CLD. Thirteen had cirrhosis (aged 8.9 ± 2.0 years) and 12 had chronic hepatitis (aged 9.3 ± 2.3 years). Gray scale and color-coded duplex Doppler US were performed for all, as well as 30 healthy age and sex-matched controls. Findings were correlated with clinical, laboratory and histopathological characteristics. RESULTS: prominent caudate lobe was detected in 100% of cirrhotics, but none of the chronic hepatitis or controls. Thickened lesser omentum and loss of the triphasic waveform of the hepatic vein were present in 69.2% and 53.8% of cirrhotics vs 33.3% and 8.3% of chronic hepatitis respectively. Portal vein flow velocity was significantly lower (P < 0.0001) and the congestion index was significantly higher (P < 0.005) in both patient groups compared to controls. Child-Pugh's staging showed a positive correlation with both abnormal hepatic vein waveform and direction of portal blood flow; and a negative correlation with both hepatic and portal vein flow velocities. No correlation with the etiology of CLD could be detected. CONCLUSION: duplex Doppler added to grayscale US can detect significant morphologic and portal hemodynamic changes that correlate with the severity (stage) of CLD, but not with etiology.