This is a cross-sectional study that was conducted over 1 year, from December 2018 to December 2019, on pediatric and adolescent patients diagnosed with portal hypertension collected from hepatology clinic, Children Hospital, ain Shams University.
We included 47 portal hypertensive pediatric cases (diagnosis was based on clinical, laboratory, and radiological investigations) who were divided according to endoscopic findings into two groups: (varices group: which included 37 cases with EVs, and non-varices group: which included 10 cases without EVs).
Patients older than 18 years, and those with disorders of VWF as heart failure, renal failure, acute Infection on time of sampling, diabetes mellitus., hypertension, hyperlipidemia, malignancy, and patients on anticoagulant or antiplatelet therapy therapy, patients with active bleeding were excluded from the study. Written informed consent from the care giver of the participants was attained before being engaged in the study after getting approval from the Research Ethics Committee at the Faculty of Medicine.
Demographic data of the patients were recorded including age and sex, they were examined for clinical signs of liver disease and portal hypertension, size of liver, spleen, and presence of ascites. Patients were subjected to routine laboratory investigations including complete blood count, liver enzyme: alanine transaminase (ALT), aspartate aminotransferase (AST), serum alkaline phosphatase, albumin, bilirubin, international normalized ratio (INR)). Child-Pugh classification was used to classify the severity of liver disease .
Abdominal ultrasonography was done to detect size of liver and spleen, presence of cirrhosis, portal vein diameter [for children younger than 10 years, the normal diameter was 8.5 mm (± 2.7). For those whose age between 10 and 20 years, the normal diameter was 10 mm (± 2)], and presence of collaterals . Moderate splenomegaly was considered if the largest dimension was 11–20 cm and marked splenomegaly if the largest dimension was more than 20 cm .
Upper GIT endoscopy was done using disinfected upper gastrointestinal video scope (OLYMPUS model) after good preparation of the patient. Patients were advised to fast for at least 6 h before the upper endoscopy. Complete evaluation of the esophagus, stomach and the duodenum down to the second part of the duodenum. Upper GIT endoscopy was performed in all cases to detect and grade the presence of EVs, they were graded according to the Japanese Research for Portal Hypertension Classification System as follows: grade (Gr) I: small EVs, Gr-II: moderated sized varices with slight obscuring of the gastroesophageal junction, Gr-III: large varices displaying luminal prolapse markedly obscuring the gastroesophageal junction and Gr IV: very large EVs, entirely obscuring the gastroesophageal junction and do not flattens on insufflation . Portal hypertensive gastropathy was detected and categorized according to the classification proposed by Tanoue and his associates  into mild, moderate, and severe.
VWF-Ag measurement using VWF-Ag ELISA:vWF: Ag detection is a sandwich ELISA through subsequent processes including dilution, incubation, washing, and quantification. It runs on the automated VIDAS® immunoanalyzers (VIDAS, Biomerieux, France). Patient vWF Ag in comparative percent intensity is settled alongside a curve based on the reference plasma provided with the kit.
The collected data were coded, and analyzed using the SPSS (Statistical Package for Social Sciences) version 22 for Windows® (IBM SPSS Inc., Chicago, IL, USA). Qualitative data were signified as frequencies and relative percentages. The chi-square test (χ2) was utilized to determine the difference between qualitative variables as indicated. Continuous data were expressed as mean ± SD or median (min-max).
. Independent samples t test was used to compare between two independent groups of normally distributed variables while Mann-Whitney U test was used for non-normally distributed data. Comparison between three or more groups with normally distributed quantitative data was performed using the one-way ANOVA test. Pearson’s correlation was used to test the correlation between two variables with parametric quantitative data. The receiver operator characteristic (ROC) curve was tested to calculate the diagnostic ability of quantitative variable (Von Willebrand factor) in the prediction of categorical outcome (varices). For all the above-mentioned tests, the level of significance was expressed as the probability of (p value) and the results were explained as following: non-significant if the p value is > 0.05, significant if the p value is ≤ 0.05, highly significant if the p value < 0.001.