Extra-hepatic portal vein thrombosis in children: Single center experience.

Aim of the study: We aimed to discuss our experience in management of children with extra-hepatic portal vein thrombosis (EHPVT). Material and methods: This retrospective cohort study included 62 children with EHPVT. All patients' records were reviewed. The patients' socio-demographic data, post-natal history, disease presentation and clinical examination were collected. Data from laboratory investigations - complete blood count, liver function tests, renal function tests, abdominal ultrasound/Doppler studies, upper endoscopic findings and treatment regimens - were collected whenever available. Results: Of the 62 patients, 62.9% were male and 37.1% were female. The mean age at disease presentation was 3.5 ±2.7 years. The main initial clinical presentation of the disease was hematemesis and/or melena (30 cases; 48.4%). History of umbilical catheterization (UVC) was present in 60% of cases. The thrombophilia profile was assessed in 17 patients, of whom 12 (70.6%) were found to have a coagulation disorder. Splenomegaly was present in 91.7% of the patients. Hematological abnormalities in the form of cytopenias were present in most cases. Ultrasound revealed the presence of collaterals in 76.2%. Upper endoscopy showed the presence of varices in 45 cases, all of which needed endoscopic intervention, while in 11 cases the varices were either low grade or absent and thus were subjected only to medical treatment with propranolol and 6 cases were lost to follow-up. Splenectomy was done in only one case and 2 cases underwent the Rex operation. Conclusions: Variceal bleeding is the most common clinical presentation of EHPVT in children. UVC is still the main etiological factor of EHPVT in our cohort especially with presence of thrombophilic disorder.

COVID-19-induced multisystem inflammatory syndrome in a child with Wilson disease: a case report.

Background: Infection with coronavirus disease 2019 (COVID-19) can progress to the multisystem inflammatory syndrome in children (MIS-C). Patients with liver cirrhosis are at increased risk of complications. Case presentation: We report on a 13-year-old Wilson's disease patient who was referred for liver transplantation because of rapid deterioration in his hepatic condition. After admission, he developed fever, respiratory distress, coronary arteries dilatation on echocardiography, laboratory evidence of inflammation, and positive severe acute respiratory syndrome coronavirus (SARS-CoV-2) PCR. SARS-CoV-2-induced MIS-C was diagnosed. Inspite of aggressive management of MIS-C, progressive deterioration of the respiratory, liver, kidney, and cardiac functions occurred and he passed away. Conclusion: MIS-C is a serious possible complication leading to multiorgan failure and higher death rate especially in cirrhotic children. So, early diagnosis and management with higher level of care by a multidisciplinary team are warranted.

Histopathological expression of Yes-associated protein in neonatal cholestasis.

BACKGROUND: Biliary atresia (BA) is a common cause of persistent neonatal cholestasis and liver transplantation in the pediatric population. Yes-associated protein (YAP) has also been shown to be necessary for development of bile ducts and adaptive responses within the gastrointestinal tract. We aimed to evaluate the YAP expression in liver tissues of infants with neonatal cholestasis as well as its diagnostic potential in the differential diagnosis of BA. PATIENTS AND METHODS: This prospective study included 100 infants with neonatal cholestasis. After full history taking, thorough clinical examination, routine investigations, and histopathological assessment, the patients were allocated as BA and non-BA; fifty patients in each group. Ten liver biopsies from 10 donors of liver transplant recipients served as controls. Diagnosis of BA was confirmed by operative cholangiography. Hepatic expression of YAP was assessed by immunohistochemical staining. RESULTS: Presence of clay stool, elevated GGT and absence of gall bladder contractility were the main preliminary signs alarming for the possibility of BA. Bile ductular and interlobular biliary epithelium and hepatic lobule expression of YAP in patients with BA was significantly higher than that in Non-BA group (P<0.05). There was no or weak positive YAP expression in normal liver of transplant donors. Positive YAP immunohistochemical had a sensitivity of 80% and a specificity of 94% with accuracy 87% in discrimination between BA and non-BA group (P-value<0.0001). CONCLUSION: Hepatic expression of YAP was significantly higher in BA than in non-BA group and could discriminate BA from other causes of cholestasis.

Glutathione peroxidase and malondialdehyde in children with chronic hepatitis C.

AIM OF THE STUDY: We aimed to assess oxidative stress factors, glutathione peroxidase (GPX) and malondialdehyde (MDA) in children with chronic hepatitis C (CHC) and their relation to treatment response. MATERIAL AND METHODS: The study included 50 children with chronic hepatitis C virus (HCV) before treatment (naïve HCV), 25 children responders to HCV treatment, 25 children non-responders to HCV treatment and 25 healthy controls. All patients and controls were subjected to GPX and MDA measurement by enzyme-linked immunosorbent assay. RESULTS: The average GPX activity in erythrocytes of naïve CHC patients was 29.2 ±10.3 mU/ml. It was statistically significantly lower than the average activity of GPX in erythrocytes of the healthy control group (47.3 ±5.2 mU/ml) (p < 0.05). The average GPX activity in erythrocytes of the responder group was 34.93 ±3.17 mU/ml. It was statistically significantly higher than the average activity of GPX in erythrocytes of the non-responder group (11.7 ±4.2 mU/ml) (p < 0.05). Plasma MDA was significantly higher in naïve CHC patients than in healthy controls (9.7 ±3.7 nmol/ml vs. 3 ±1.1 nmol/ml, p < 0.0001). Furthermore, plasma MDA concentration was significantly decreased in the responder group (5.36 ±0.7 nmol/ml) and elevated in the non-responder group (16.05 ±2.9 nmol/ml). CONCLUSIONS: Lower pretreatment levels of GPX and higher MDA level might be markers of oxidative stress occurring in HCV patients. Reversal of changes of these levels with completion of the treatment may indicate a correlation between oxidative stress and the viral pathogenesis.

Expression of vascular endothelial growth factor A in liver tissues of infants with biliary atresia.

AIM OF THE STUDY: Assessment of hepatic expression of vascular endothelial growth factor A (VEGF-A) in liver tissues of infants with biliary atresia (BA). MATERIAL AND METHODS: This retrospective study included 35 infants with BA (BA group), and 38 infants with cholestasis due to causes other than BA (non-BA group). All patients had undergone full history taking, through clinical examination, routine investigations and immunostaining of liver tissue for VEGF-A and cytokeratin 7 (CK7). The diagnosis of BA was confirmed by intraoperative cholangiography. In the non-BA group, other specific laboratory tests according to the expected etiology were done. RESULTS: Most of the BA group showed positive VEGF-A expression with variable degrees in both bile ducts (BDs; 80%), and arterial walls (AWs; 77.2%), while most of the non-BA group showed negative staining of VEGF in both BDs and AWs (89.5% and 86.8% respectively) (p < 0.0001). Positive VEGF expression in the portal structures in both BDs and AWs had 84.9% and 82.19% accuracy; respectively. The majority of BA group showed either grade II of positive cytokeratin-7 expression in liver tissues (45.7%) or grade III (34.3%), while most of the non-BA group showed grade I (71.1%) (p < 0.0001). Positive CK7 expression in > 25% of the liver tissues had 80.8% accuracy in discriminating between BA and non-BA. CONCLUSIONS: VEGF-A expression in the portal structures in liver tissues in both BDs and AWs had very good accuracy in discriminating between BA and non-BA patients.

Diagnosis of spontaneous bacterial peritonitis in children using leukocyte esterase reagent strips and granulocyte elastase immunoassay.

AIM OF THE STUDY: We aimed to assess the utility and rapidity of granulocyte elastase (GE) latex immunoassay and leukocyte esterase (LE) reagent strips for the diagnosis of spontaneous bacterial peritonitis (SBP) in hepatic children with ascites. MATERIAL AND METHODS: This study included 80 ascitic fluid (AF) samples from 45 patients with chronic liver diseases. They were divided into 2 groups (SBP and non-SBP groups). White blood cells > 500 cell/mm3 or polymorphonuclear leukocytes > 250 cell/mm3 were considered as a positive result for the diagnosis of SBP. The AF obtained at bedside was immediately tested with an LE enzyme reagent strip, and another sample was aliquoted for measurement of serum GE. RESULTS: On doing LE dipstick strips, all the non-SBP group gave no coloration with LE strips while 62% of the SBP group gave coloration. LE strips had accuracy of 86.25% in differentiating SBP and non-SBP at a cut-off value of 1 (color grade 1). The diagnostic performance of GE in differentiating SBP and non-SBP was assessed and showed accuracy of 70% for a cutoff value of 123.5 ng/ml. CONCLUSIONS: Application of LE reagent strips is a bedside, rapid, easy-to-use, and inexpensive method for diagnosis of SBP. It has an accuracy of 86.25% in differentiating SBP and non-SBP, which is higher than more complicated and delayed methods such as GE latex immunoassay, which has an accuracy of 70%.