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Serum cortisol mainly binds to the cortisol-binding globulin (CBG). Children with biliary atresia (BA) may have low serum CBG levels; thus, low serum total cortisol (TC) levels and adrenal insufficiency (AI) may be overdiagnosed. This study aimed to assess adrenal function in children with BA. All the patients underwent adrenocorticotropic hormone (ACTH) stimulation tests. Plasma ACTH, serum TC, and CBG levels were measured at baseline, with additional TC measurements at 30 and 60 min during testing. Free cortisol (FC) index (FCI) and calculated FC (cFC) were also calculated. AI was defined as peak TC <500 nmol/L (<18 µg/dL), peak FCI <12 nmol/mg, or peak cFC <33 nmol/L (<1.2 µg/dL). This study enrolled 71 children with BA. The Median (IQR) age of the patients was 5.5 (1.7-11.4) years. Twenty-five (35%) patients were diagnosed with AI based on the peak TC. In the AI group, the median serum CBG level was significantly lower than that in the non-AI group (481 vs. 533 nmol/L, p = 0.03). Only eight patients (11%) met all three AI criteria (six secondary AI and two primary AI). In conclusion, low serum CBG levels contribute to reduced peak TC and, consequently, overdiagnosing AI. Peak FCI and cFC could help reduce the overdiagnosis of AI. [ABSTRACT FROM AUTHOR]

The impact of the coronavirus disease 2019 (COVID-19) pandemic has been immense, and it continues to have lasting repercussions. While the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus primarily infects the respiratory system, other organ systems are affected, including the liver. Scientific knowledge on the role of SARS-CoV-2 infection and liver injury has evolved rapidly, with recent data suggesting specific hepatotropism of SARS-CoV-2. Moreover, additional concerns have been raised in regard to long-term liver damage, related to emerging cases of post-COVID-19 cholangiopathy and chronic cholestasis. Great effort has also been focused on studying how specific subpopulations with chronic medical conditions might be disproportionately impacted by COVID-19. One such population includes individuals with chronic liver disease (CLD) and cirrhosis, with an expanding body of research indicating these patients being particularly susceptible to adverse outcomes. In this review, we provide an updated summary on the current pathogenesis and mechanism of liver injury in the setting of SARS-CoV-2 infection, the association between health outcomes and SARS-CoV-2 infection in patients with CLD, and the unique consequences of the COVID-19 pandemic on the routine care of patients with CLD. [ABSTRACT FROM AUTHOR]

Background: Liver cirrhosis is an end stage liver damage. Biliary atresia (BA) and inherited syndromes of intrahepatic cholestasis are the most frequent causes of liver cirrhosis in children especially in the first year of life. Objective: To compare the prognostic value of albumin-bilirubin grade (ALBI), an alternative predictor of mortality in cirrhotic adults, with Child-Pugh and Pediatric End-Stage Liver Disease (PELD) scores in cirrhotic children with variceal bleeding. Methods: We analyzed the patients' data using MedCalc software, and calculated area under the curves (AUC) of receiving-operator characteristic (ROC). Results: All scores were higher in expired compared to survived patients (p<0.001). The AUC values for predicting mortality in all patients were 0.879±0.04 for Child-Pugh and PELD scores and 0.733±0.06 for ALBI. The prognostic values in two subgroups of BA and those with other underlying diseases (non-BA) showed that in BA group, ALBI score with AUC of 0.607±0.102, p<0.29, had no significant difference between survived and non-survived patients. ALBI in non-BA group, with AUC of 0.863±0.063 showed closer value to other scores (0.954), and was differed between survived and non-survived groups (p<0.001). Conclusion: Our results suggested that ALBI might be useful in predicting mortality in non-BA patients. [ABSTRACT FROM AUTHOR]

Purpose: There is no consensus regarding adjuvant therapies following Kasai portoenterostomy (KP) for biliary atresia (BA). This study aimed to analyze the effect of extended perioperative intravenous antibiotics (PI-Abx) and adjuvant corticosteroid on cholangitis and jaundice clearance rates in the 3 years post-KP in children with BA. Methods: Data of patients who underwent KP between 1999-2018 at a single center were retrospectively analyzed. Group A (1999-2010) received PI-Abx for 5 days, Group B (2010-2012) received PI-Abx for 5 days plus low-dose prednisolone (2 mg/kg), and Group C (2012-2017) received PI-Abx for 14 days plus high-dose prednisolone (5 mg/kg). Results: Fifty-four patients were included with groups A, B, and C comprising 25, 9, and 20 patients, respectively. The number of episodes of cholangitis was 1.0, 1.6, and 1.3 per patient (p=NS) within the first year and 1.8, 2.3, and 1.7 (p=NS) over 3 years in Groups A, B, and C, respectively. The jaundice clearance rate at 6 months was 52%, 78%, and 50% (p=NS), and the 3-year native liver survival (NLS) rate was 76%, 100%, and 80% (p=NS) in Groups A, B, and C, respectively. A near-significant association was observed between the incidence of cholangitis within the first year and decompensated liver cirrhosis/death at 3 years post KP (p=0.09). Persistence of jaundice at 6 months was significantly associated with decompensated cirrhosis/death at 3 years (p<0.001). Conclusion: The extended duration of PI-Abx and adjuvant corticosteroids was not associated with improved rates of cholangitis, jaundice clearance, or NLS in patients with BA. [ABSTRACT FROM AUTHOR]

Background: Although identifying cases in large administrative databases may aid future research studies, previous reports demonstrated that the use of the International Classification of Diseases, Tenth Revision (ICD-10) code alone for diagnosis leads to disease misclassification. Purpose: We aimed to assess the value of the ICD-10 diagnostic code for identifying potential children with biliary atresia. Methods: Patients aged <18 years assigned the ICD-10 code of biliary atresia (Q44.2) between January 1996 and December 2016 at a quaternary care teaching hospital were identified. We also reviewed patients with other diagnoses of code-defined cirrhosis to identify more potential cases of biliary atresia. A proposed diagnostic algorithm was used to define ICD-10 code accuracy, sensitivity, and specificity. Results: We reviewed the medical records of 155 patients with ICD-10 code Q44.2 and 69 patients with other codes for biliary cirrhosis (K74.4, K74.5, K74.6). The accuracy for identifying definite/probable/possible biliary atresia cases was 80%, while the sensitivity was 88% (95% confidence interval [CI], 82%-93%). Three independent predictors were associated with algorithm-defined definite/probable/possible cases of biliary atresia: ICD-10 code Q44.2 (odds ratio [OR], 2.90; 95% CI, 1.09-7.71), history of pale stool (OR, 2.78; 95% CI, 1.18-6.60), and a presumed diagnosis of biliary atresia prior to referral to our hospital (OR, 17.49; 95% CI, 7.01-43.64). A significant interaction was noted between ICD-10 code Q44.2 and a history of pale stool (P<0.05). The area under the curve was 0.87 (95% CI, 0.84-0.89). Conclusion: ICD-10 code Q44.2 has an acceptable value for diagnosing biliary atresia. Incorporating clinical data improves the case identification. The use of this proposed diagnostic algorithm to examine data from administrative databases may facilitate appropriate health care allocation and aid future research investigations. [ABSTRACT FROM AUTHOR]

Aim: To determine factors associated with severe hospitalized Respiratory syncytial virus (RSV)‐associated LRTI and to describe management in tertiary care center. Methods: Retrospective medical record review was conducted among children under 5 years old hospitalized with RSV‐associated LRTI at King Chulalongkorn Memorial Hospital. Severe RSV‐associated LRTI was defined as death, mechanical ventilator, or positive pressure ventilation use, prolonged hospitalization >7 days. Factors associated with severe RSV were analyzed using univariate and multivariate logistic regression. Results: From January 2011 to December 2016, 427 children were hospitalized. Median age was 10 months (IQR 4.2‐23.0). One hundred seventy‐four (41%) patients had severe RSV (11 deaths, 56 mechanical ventilators, 19 positive pressure ventilation, and 88 prolonged hospitalization). Factors associated with severe RSV were chronic lung disease (aOR 15.16 [4.26‐53.91]), cirrhosis/biliary atresia (aOR 15.01 [3.21‐70.32]), congenital heart disease (aOR 5.11 [1.97‐13.23]), chemotherapy (aOR 4.7 [1.34‐16.56]), and pre‐term (aOR 2.03 [1.13‐3.67]). Oxygen therapy was mainly low flow oxygen delivery. 88% of cases received bronchodilator. Parenteral antibiotics were prescribed in 37.9% of cases. Conclusions: Children with co‐morbidities have higher risk of severe RSV‐associated LRTI. More than two‐third of patients received bronchodilator, of which was not recommended by American Academy of Pediatrics. The specific treatment and prevention for RSV are urgently needed. [ABSTRACT FROM AUTHOR]

Background: Preliminary studies have shown that diffusion tensor imaging (DTI) is helpful in evaluating liver disorders. However, there is no published literature on the use of DTI in the diagnosis of biliary atresia (BA). This study aimed to investigate the diagnostic value of the liver average apparent diffusion coefficient (ADC) and fractional anisotropy (FA) measured using DTI for BA in neonates and infants. Methods: Fifty-nine patients with infant jaundice were included in this study. DTI was performed with b factors of 0 and 1000 s/mm2. Liver fibrosis in the BA group was determined and graded (F0, F1, F2, F3, F4) based on the pathological findings. Statistical analyses were performed to determine the diagnostic accuracy of DTI for BA. Results: The ADC value was significantly lower in the BA group [(1.262±0.127)×10−3 mm2/s] than in the non-BA group [(1.430±0.149)×10−3 mm2/s, (P<0.001)]. The area under the receiver operating characteristic curve was 0.805±0.058 (P<0.001) for ADC. With a cut-off value of 1.317×10−3 mm2/s, ADC achieved a sensitivity of 75% and a specificity of 81.5% for the differential diagnosis of BA and non-BA. In the BA group, the ADC value was significantly correlated with fibrotic stage. Further analysis showed that the ADC value of stage F0 was significantly higher than that of stages F1, F2, F3 and F4, whereas there were no significant differences among stages F1, F2, F3 and F4. Conclusion: Hepatic ADC measured with DTI can be used as an adjunct to other noninvasive imaging methods in the differential diagnosis of BA and non-BA. ADC was helpful in detecting liver fibrosis but not in differentiating the fibrotic grades. [ABSTRACT FROM AUTHOR]

Purpose: To compare preoperative CT findings before liver transplantation between patients with Alagille syndrome (AGS) and those with end-stage biliary atresia (BA). Materials and Methods: The institutional review board approved this retrospective study. Eleven children with AGS (median age, 19.0 ± 13.0 months; male to female ratio, 3:8) and 109 children with end-stage BA (median age, 17.9 ± 25.8 months; male to female ratio, 37:72) who underwent abdomen CT as candidates for liver transplant were included. CT images were reviewed focusing on hepatic parenchymal changes, vascular changes, presence of focal lesions, and signs of portal hypertension. Results: Hepatic parenchymal changes were present in 27% (3/11) of AGS patients and 100% (109/109) of end-stage BA patients (P < .001). The hepatic artery diameter was significantly smaller (1.9 mm versus 3.6 mm, P = 008), whereas portal vein diameter was larger (6.8 mm versus 5.0 mm, P < .001) in patients with AGS compared with patients with end-stage BA. No focal lesion was seen in patients with AGS, whereas 44% (48/109) of patients with end-stage BA had intrahepatic biliary cysts (39%, 43/109) and hepatic tumors (8%, 9/109) (P = .008). Splenomegaly was commonly seen in both groups (P = .082), and ascites (9% [1/11] versus 50% [54/109], P = .010) and gastroesophageal varix (0% [0/11] versus 80% [87/109], P < .001) were less common in patients with AGS than in patients with end-stage BA. Conclusion: Fibrotic or cirrhotic changes of the liver, presence of focal lesions, and relevant portal hypertension were less common in patients with AGS than in patients with end-stage BA. [ABSTRACT FROM AUTHOR]

Background/Aims Variceal bleeding (VB) is seen both with childhood cholestatic as well as hepatocellular disorders. There is limited data on morbidity related to VB in infants and children. We aimed to study the organ failures and outcome of cirrhotic children with VB necessitating liver transplantation (LT). Methods We retrospectively collected and analyzed data of all infants and children with presence of cirrhosis and variceal bleed who presented between December 2010 and March 2019. Severity scores, organ failures and outcome were analysed. Results We identified 101 variceal bleeding episodes in 79 children (30.4% females)-median one episode (range, 1 to 8) and >/=3 VB happened in 14 children. The commonest etiologies were biliary atresia (35.4%), autoimmune hepatitis (21.5%), hepatic venous outflow tract obstruction (11.4%) and other genetic and cholestatic conditions (25.3%). Sixteen of these episodes were tolerated well without development of second decompensation. One or more organ failure was present in 98 episodes-median Pediatric CLIF-SOFA score was 6 (interquartile range, 4 to 10). Seven children developed advance (grade 3-4) hepatic encephalopathy while stage 3-4 acute kidney injury was seen in 11 children. By 90 days, 12 children received living donor LT, while 11 died. On univariate analysis, mortality at day-90 was associated with presence of Hepatopulmonary syndrome, hepatic encephalopathy, pneumonia and spontaneous bacterial peritonitis and with high bilirubin, creatinine, international normalized ratio, PELD/Model for End Stage Liver Disease, Child-Pugh and pediatric CLIF-SOFA scores and low sodium. On multivariate analysis, pediatric CLIF-SOFA (Wald, 10.593; p=0.001) and Hepatopulmonary syndrome (Wald, 4.079; p=0.043) were associated with poor outcome. There was recurrence of VB in 31 children over a median duration of 36 months. Conclusions Variceal bleeding in children is associated with organ failures which affect short term prognosis. Hepatopulmonary syndrome also relates to poor outcome in these children. [ABSTRACT FROM AUTHOR]