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1.
PLoS One ; 19(8): e0307837, 2024.
Article in English | MEDLINE | ID: mdl-39197055

ABSTRACT

BACKGROUND: Newborn screening for biliary atresia (BA) may facilitate earlier diagnosis and intervention for improved clinical outcomes. METHODS: We systematically reviewed the accuracy of population-based screening strategies for BA in the newborn using PRISMA-DTA guidelines. We included cohort or cross-sectional studies. The screening (index) tests included stool color card (SCC) and direct/conjugated bilirubin (DB/CB) and the reference standard was intraoperative cholangiogram. Meta-analysis was performed using random-effects logistic regression models. RESULTS: We included 15 studies (1,816,722 participants) that assessed 5 different population-based screening strategies. QUADAS-2 assessment revealed high risk of bias for patient selection in one study and uncertain risks for reference standard in multiple studies. High certainty evidence suggests that DB/CB assessed after birth had a summary sensitivity of 100% (95% CI 100,100) and specificity of 98.8% (98.8,98.9) (5 studies, 662141 participants). Moderate certainty evidence suggests that SCC screening at a month of age had summary sensitivity of 79.6% (95% CI 70.6, 86.4) and specificity of 99.9% (95% CI 99.9, 99.9) (7 studies, 996262 participants). CONCLUSIONS: DB/CB in the first few days of life has the best diagnostic accuracy for population screening for biliary atresia in the newborn. Future research should focus on cost-effectiveness and combinations of screening strategies.


Subject(s)
Biliary Atresia , Neonatal Screening , Biliary Atresia/diagnosis , Biliary Atresia/surgery , Humans , Infant, Newborn , Neonatal Screening/methods , Sensitivity and Specificity , Bilirubin/blood
2.
Pediatr Surg Int ; 40(1): 219, 2024 Aug 08.
Article in English | MEDLINE | ID: mdl-39115726

ABSTRACT

PURPOSE: We aimed to investigate whether prediction of liver fibrosis using two-dimensional shear wave elastography (2D-SWE) and vascular tree grading using superb microvascular imaging (SMI) are useful for postoperative follow-up in patients with biliary atresia (BA). METHODS: We retrospectively collected data from medical records of 134 patients who underwent ultrasound examination with 2D-SWE or SMI, including 13 postoperative patients with BA and 121 non-BA patients. We investigated the distribution of liver stiffness values with SWE and vascular tree grading with SMI and evaluated correlations between these findings and biochemical indices of liver fibrosis in postoperative BA patients. RESULTS: The SWE values of the BA group were not significantly different from that of any other disease groups in non-BA patients. In postoperative BA patients, SWE values correlated significantly with aspartate aminotransferase to platelet ratio index (Spearman rank correlation coefficient [rs] = 0.6380, p = 0.0256) and with the Fib-4 index (rs = 0.6526, p = 0.0214). SMI vascular tree grading of the BA group was significantly higher than that of the choledochal cyst group (p = 0.0008) and other hepatobiliary disorder group (p = 0.0030). In postoperative BA patients, SMI vascular tree grading was not positively correlated with any biochemical marker of fibrosis. CONCLUSION: 2D-SWE appears to be useful for follow-up in postoperative BA patients.


Subject(s)
Biliary Atresia , Elasticity Imaging Techniques , Liver Cirrhosis , Humans , Biliary Atresia/surgery , Biliary Atresia/diagnostic imaging , Elasticity Imaging Techniques/methods , Male , Retrospective Studies , Female , Liver Cirrhosis/diagnostic imaging , Infant , Microvessels/diagnostic imaging , Liver/diagnostic imaging , Liver/blood supply , Child, Preschool , Postoperative Period , Follow-Up Studies , Child , Postoperative Complications/diagnostic imaging
3.
Pediatr Surg Int ; 40(1): 218, 2024 Aug 08.
Article in English | MEDLINE | ID: mdl-39115750

ABSTRACT

PURPOSE: We aimed to identify factors predicting the need for future liver transplantation (LT) at 18 years of age in patients with biliary atresia (BA). METHODS: BA patients with native liver survival at > 18 years of age were retrospectively reviewed. The clinical characteristics, outcomes, hepatobiliary function, and liver fibrosis markers of native liver survivors (NLS group) were compared with patients who subsequently underwent LT (LT group). RESULTS: The study population included 48 patients (NLS, n = 34; LT, n = 14). The male-to-female ratio, age at Kasai procedure, and type of BA in the two groups did not differ to a statistically significant extent. There was no significant difference in the MELD scores between the groups at 18 years of age. The aspartate aminotransferase-to-platelet ratio index (APRI), albumin-bilirubin (ALBI), and BA liver fibrosis (BALF) scores at 18 years of age were significantly higher in the LT group. The AUCs for APRI, ALBI, and BALF were 0.91, 0.79, and 0.85, respectively. CONCLUSION: Adult BA patients have limited options for LT owing to the lack of donor candidates and the low prevalence of deceased donors. The elucidation of prognostic factors for LT in adulthood is important. APRI was the most useful marker in this study.


Subject(s)
Biliary Atresia , Liver Transplantation , Humans , Biliary Atresia/surgery , Male , Female , Retrospective Studies , Adolescent , Prognosis , Young Adult , Adult , Follow-Up Studies
4.
Pediatr Surg Int ; 40(1): 239, 2024 Aug 21.
Article in English | MEDLINE | ID: mdl-39167137

ABSTRACT

PURPOSE: Biliary atresia (BA) poses a persistent challenge characterized by ongoing liver inflammation and subsequent fibrosis even after the clearance of jaundice (COJ). This study aimed to evaluate the therapeutic potential of eicosapentaenoic acid (EPA) in alleviating liver inflammation and limiting fibrosis during the post-COJ phase of BA. METHODS: Among the BA patients undergoing laparoscopic Kasai portoenterostomy (lapKP) between December 2016 and October 2021, EPA (20-40 mg/kg/day) was administered orally to those whose parents consented. The study included patients from January 2014 to October 2021, classifying them into two groups: EPA-treated (Group E) and untreated (Group N). Their liver fibrosis and clinical course at 1 and 2 years post-lapKP were compared. RESULTS: Group E consisted of 25 patients, while Group N comprised 32 patients. Twenty-one patients in Group E and 25 patients in Group N achieved COJ (p = 0.74). Among jaundice-free patients at 1 and 2 years post-lapKP, Group E exhibited significantly lower M2BPGi levels and platelet counts, and Group E showed a significant reduction in Aminotransferase-to-Platelet Ratio Index (APRI) at 2 years post-lapKP. CONCLUSION: Although EPA administration did not improve COJ, it attenuated the progression of liver fibrosis during the 2 years following lapKP in jaundice-free patients. (200/200Words).


Subject(s)
Biliary Atresia , Disease Progression , Eicosapentaenoic Acid , Liver Cirrhosis , Portoenterostomy, Hepatic , Humans , Portoenterostomy, Hepatic/methods , Eicosapentaenoic Acid/therapeutic use , Eicosapentaenoic Acid/administration & dosage , Male , Female , Biliary Atresia/surgery , Infant , Laparoscopy/methods , Postoperative Complications/prevention & control , Retrospective Studies , Treatment Outcome , Child, Preschool
5.
Pediatr Surg Int ; 40(1): 244, 2024 Aug 27.
Article in English | MEDLINE | ID: mdl-39191932

ABSTRACT

PURPOSE: Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA. METHODS: We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6 cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2). RESULTS: 73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%. CONCLUSION: The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations.


Subject(s)
Biliary Atresia , Humans , Biliary Atresia/diagnosis , Biliary Atresia/surgery , Biliary Atresia/complications , Prospective Studies , Cross-Sectional Studies , Female , Male , Infant, Newborn , Jaundice, Obstructive/etiology , Jaundice, Obstructive/diagnosis , Infant , Cholangiography/methods , Sensitivity and Specificity , gamma-Glutamyltransferase/blood , Early Diagnosis
6.
Pediatr Surg Int ; 40(1): 197, 2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39017936

ABSTRACT

PURPOSE: The prognostic factors of subsequent liver transplantation (LT) in patients with biliary atresia (BA) who presented with jaundice-free native liver survival were investigated. METHODS: This study retrospectively reviewed patients who underwent portoenterostomy (PE) for BA. Patients with jaundice-free native liver survival at 1 year postoperatively were divided into the autologous liver survivor and liver transplant recipient groups. Peri- and postoperative data were compared between the two groups. RESULTS: Among 97 patients with BA, 29 who received LT within 1 year after PE were excluded from the analysis. Further, 48 patients currently living with native liver and 20 who received LT after 1 year postoperatively were compared. Bile lake (BL) was the strongest risk factor of LT. The risk score was 2.38 ∗ B L s c o r e + 0.00466 ∗ T B A , and the area under the receiver operating characteristic curve was 0.83. Patients with BL and those without significantly differed in terms of the native liver survival rate. Patients with BL who presented with not only cholangitis but also gastrointestinal hemorrhage and hepatopulmonary syndrome received LT. CONCLUSION: BL can cause different pathologies. Moreover, it is an evident risk factor of subsequent LT in patients with BA who are living with native liver at 1 year after PE.


Subject(s)
Biliary Atresia , Liver Transplantation , Portoenterostomy, Hepatic , Humans , Biliary Atresia/surgery , Biliary Atresia/complications , Biliary Atresia/mortality , Retrospective Studies , Female , Male , Infant , Risk Factors , Portoenterostomy, Hepatic/methods , Survival Rate/trends , Bile , Prognosis , Child, Preschool , Jaundice/etiology , Liver
7.
Pediatr Surg Int ; 40(1): 196, 2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39017953

ABSTRACT

PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.


Subject(s)
Biliary Atresia , Cholestasis , Hypertension, Portal , Portoenterostomy, Hepatic , Humans , Biliary Atresia/surgery , Biliary Atresia/complications , Portoenterostomy, Hepatic/methods , Male , Female , Hypertension, Portal/etiology , Infant , Cholestasis/etiology , Postoperative Complications/epidemiology , Prospective Studies , Follow-Up Studies , Survivors/statistics & numerical data , Infant, Newborn , Child, Preschool
8.
J Ultrasound ; 27(3): 739-743, 2024 Sep.
Article in English | MEDLINE | ID: mdl-39026132

ABSTRACT

Biliary atresia (BA) is a congenital disease that occurs when extrahepatic bile ducts are either absent or deficient, resulting in liver fibrosis, portal hypertension, and eventually cirrhosis. It is the most common cause of persistent obstructive jaundice in newborns lasting more than two weeks is this condition. Abdominal ultrasound (US) is the primary imaging technique used to diagnose BA, while computed tomography (CT) is reserved for more complex cases. The gold standard for diagnosing BA is still intraoperative cholangiogram with liver biopsy. Treatment for BA usually involves Kasai hepatoportoenterostomy, but some patients still require liver transplantation due to diagnostic delays and advanced disease. In this study, the authors present the case of a 3-month-old infant with biliary atresia and its ultrasound characteristics, who underwent liver transplantation due to advanced disease. The primary objective of imaging is to provide a prompt diagnosis, given the crucial significance of timely surgical intervention.


Subject(s)
Biliary Atresia , Liver Transplantation , Ultrasonography , Humans , Biliary Atresia/diagnostic imaging , Biliary Atresia/surgery , Biliary Atresia/complications , Infant , Ultrasonography/methods , Male , Liver/diagnostic imaging , Liver/pathology , Female
9.
Pediatr Surg Int ; 40(1): 190, 2024 Jul 16.
Article in English | MEDLINE | ID: mdl-39012391

ABSTRACT

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.


Subject(s)
Biliary Atresia , Cholangitis , Liver Transplantation , Postoperative Complications , Humans , Biliary Atresia/surgery , Biliary Atresia/complications , Cholangitis/blood , Male , Female , Infant , Prognosis , Retrospective Studies , Child, Preschool , Inflammation/blood , Biomarkers/blood , Living Donors
10.
Medicine (Baltimore) ; 103(28): e38757, 2024 Jul 12.
Article in English | MEDLINE | ID: mdl-38996159

ABSTRACT

To evaluate the efficacy and safety of indocyanine green (ICG)-guided near-infrared fluorescence (NIRF) imaging during surgery to diagnose the cause of neonatal cholestasis (NC). Data on NC patients who underwent both NIRF with ICG and conventional laparoscopic bile duct exploration (the gold standard) at our institute from January 2022 to December 2022 were retrospectively analyzed. The patients' baseline characteristics and liver function outcomes were collected and analyzed, and the diagnostic consistency was compared between the 2 methods. In total, 16 NC patients were included in the study, comprising 8 (50%) male and 8 (50%) female patients, ranging in age from 42 to 93 days, with a median age of 54.4 ±â€…21 days. During surgery, all the patients underwent NIRF with ICG, followed by conventional laparoscopic bile duct exploration. Finally, 15 of the patients were diagnosed with biliary atresia (BA) (1 with type-I BA, and 14 with type-II BA). The other patient was diagnosed with cholestasis. The diagnostic results from fluorescence imaging with ICG were consistent with those from conventional laparoscopic bile duct exploration. ICG-guided NIRF is associated with an easy operation, less trauma, and good safety. Also, its diagnostic accuracy is similar to conventional laparoscopic bile duct exploration.


Subject(s)
Cholestasis , Indocyanine Green , Optical Imaging , Humans , Indocyanine Green/administration & dosage , Female , Male , Retrospective Studies , Cholestasis/diagnostic imaging , Cholestasis/etiology , Optical Imaging/methods , Infant , Infant, Newborn , Biliary Atresia/surgery , Biliary Atresia/diagnostic imaging , Laparoscopy/methods , Coloring Agents/administration & dosage , Spectroscopy, Near-Infrared/methods
11.
S Afr Med J ; 114(3b): e1190, 2024 Apr 24.
Article in English | MEDLINE | ID: mdl-39041452

ABSTRACT

BACKGROUND: The Wits Transplant Unit performed its first paediatric liver transplant in 2005. Initial experiences from the unit were published in 2012 and 2014. Since then, significant progress has been made in capacity-building the unit, improving outcomes and enhancing service delivery. This paper presents a broad overview and update of the unit's 17-year experience.   Methods: We conducted a retrospective review of all paediatric liver transplants performed in Johannesburg from 1 January 2005 to 31 December 2021 with a minimum one-year follow-up. Data were accessed from the Wits Donald Gordon Medical Centre Paediatric Liver Transplant Research Database (University of the Witwatersrand Human Research Ethics approval: M190749). The following data were collected: donor and recipient sociodemographic and clinical characteristics, details of transplant procedures, donor grafts and recipient outcomes (post-operative complications, graft and recipient survival).   Results: A total of 270 transplants were performed during the review period. Two thirds of recipients (n=180, 67%) were younger than 5 years at time of transplant and half (n=135, 50%) received a living donor graft. The most common indication for liver transplant was biliary atresia, followed by acute liver failure. Unadjusted recipient survival was 80% (95% CI: 75-85%) at one year, and 68% (95% CI: 59-75%) at five years. Waiting list mortality decreased from 27.3% in 2017 to 5.9% in 2021. One hundred and fifty-four (57.0%) recipients experienced at least one type of intervention requiring surgical complication - the most common being biliary in nature (n = 91; 33.7%).   Conclusion: Over last seventeen years, a sustainable paediatric liver transplantation service has been established in Johannesburg. Living donor, split and ABO incompatible liver transplants have been incorporated in response to the severe organ shortage in South Africa. However, our outcomes can be improved. Additionally, a national transplant initiative to coordinate timeous referrals and expand access to liver transplantation for children with severe acute and chronic liver failure is advised.


Subject(s)
Liver Transplantation , Humans , South Africa , Retrospective Studies , Child , Child, Preschool , Male , Female , Adolescent , Infant , Graft Survival , Living Donors , Postoperative Complications/epidemiology , Biliary Atresia/surgery
12.
Transplant Proc ; 56(5): 1148-1152, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38825401

ABSTRACT

Alanine aminotransferase (ALT) is an enzyme that catalyzes the transfer of amino groups from alanine to ketoglutaric acid. ALT is an established marker of liver diseases. Occasionally, ALT levels may be abnormally low due to various factors, making accurate assessment difficult. To date, no studies have documented ALT alterations following Living donor liver transplantation (LDLT) in patients with low ALT levels. Here, we present a case of abnormally low ALT levels that were ameliorated by LDLT. A 27-year-old woman underwent LDLT for refractory cholangitis with biliary atresia. The patient's preoperative ALT level was 1 IU/L. Following graft reperfusion, ALT levels increased (peak value, 456 IU/L), primarily attributed to the donor liver. After LDLT, ALT levels consistently surpassed the lower limit. The differential diagnosis of abnormally low ALT levels suggested a genetic mutation as the most probable underlying cause. Even after LDLT, ALT levels in organs other than the transplanted liver would remain abnormally low. Therefore, to prevent underestimating liver damage, the standard ALT range for such cases should be set lower than the typical range.


Subject(s)
Alanine Transaminase , Liver Transplantation , Living Donors , Humans , Female , Adult , Alanine Transaminase/blood , Biliary Atresia/surgery , Cholangitis/surgery
13.
J Pediatr Gastroenterol Nutr ; 79(2): 222-228, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38828708

ABSTRACT

OBJECTIVE: Biliary atresia (BA) is the leading cause of liver cirrhosis and chronic liver insufficiency in children in the world. Gastroesophageal varices bleeding is an ominous complication of cirrhosis in BA patients and is associated with high morbidity and mortality. In this study, we aimed to investigate the utility of noninvasive Baveno VI and Baveno VII criteria for the screening of varices need treatment (VNT) and the need for liver transplantation in BA patients. METHODS: This study enrolled 48 BA patients (23 females and 25 males) who underwent an esophagogastroduodenoscopy (EGD) and transient elastography at a mean age of 11.18 ± 1.48 years; the clinical data were surveyed in a retrospective design. RESULTS: The sensitivity and negative predictive value of Baveno VI and Baveno VII criteria for the prediction of VNT in BA patients are both 100% and 100%, respectively. The VNT missing rate of Baveno VI and Baveno VII criteria are both 0% in our cohort. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are also predictive of the need for liver transplantation in our cohort (OR = 10.33, 4.24, and 21.33; p = 0.009, 0.03, and 0.007, respectively). CONCLUSION: The Baveno VI and Baveno VII criteria are useful for the screening of VNT and minimize non-necessary invasive EGD in BA patients with low VNT missing rates. The Baveno VI, expanded Baveno VI, and Baveno VII criteria are associated with the need for liver transplantation.


Subject(s)
Biliary Atresia , Esophageal and Gastric Varices , Liver Transplantation , Humans , Biliary Atresia/complications , Biliary Atresia/surgery , Esophageal and Gastric Varices/etiology , Esophageal and Gastric Varices/diagnosis , Female , Male , Retrospective Studies , Child , Endoscopy, Digestive System/methods , Elasticity Imaging Techniques , Adolescent , Predictive Value of Tests , Gastrointestinal Hemorrhage/etiology , Liver Cirrhosis/complications , Sensitivity and Specificity , Mass Screening/methods
14.
Pediatr Surg Int ; 40(1): 153, 2024 Jun 08.
Article in English | MEDLINE | ID: mdl-38850286

ABSTRACT

BACKGROUND: Systemic Immune-Inflammation Index (SII), known as an easy, economical and useful marker, correlates with the balance of inflammation and immune response. However, the usefulness of SII in biliary atresia (BA) remains unclear. Therefore, we evaluated the relationship of SII level and postoperative clinical outcomes of BA. METHODS: Retrospective review of 168 patients with BA was conducted with assessments of demographic information, histological findings, laboratory parameters, and clinical outcomes. The LASSO logistic regression analysis was established using the "glmnet" software package to explore the influencing factors related to native liver survival time. Numerical variables were dichotomized based on the receiver operating characteristic (ROC) curve and Youden index yielding the best performance of prediction. R software was used for statistical analysis. RESULTS: Overall, the 24 month native liver survival rate was 43.5% (73 cases) after Kasai portoenterostomy. LASSO logistic regression analysis show that preoperative malnutrition (OR = 0.032, 95%CI 0.001-0.424), gamma-glutamyltransferase (GGT, OR = 0.994, 95%CI 0.987-0.998), lymphocyte count (LY, OR = 2.426, 95%CI 1.467-4.604), SII (OR = 0.977, 95%CI 0.960-0.989), and liver fibrosis grading (LFG, reference: Grade 1, Grade 3, OR = 0.076, 95%CI 0.007-0.614) were the independent influencing factors for 24 month native liver survival. ROC curve analysis showed that the area under the curve of SII level (0.919) was larger than that of preoperative malnutrition (0.690), LFG (0.759), GGT (0.747), and Ly (0.773). A SII < 140.09 was found to be a significant marker in the prediction of 24-month native liver survival, with 90.41% sensitivity and 93.68% specificity. Furthermore, the rates of 24-month native liver survival (33.1% vs. 72.7%), jaundice clearance (46.8% vs. 75.0%), and good liver function recovery (46.8% vs. 65.9%) were lower in the SII ≥ 140.09 group than that in the SII < 140.09 group (all P < 0.05), but there was no difference in the occurrence of cholangitis (P > 0.05). CONCLUSION: Preoperative malnutrition, GGT, Ly, SII, and LFG were independent influencing factors for postoperative 24-month native liver survival of BA. The SII level, as a routine haematological marker, has better universality and simplicity and is related to clinical outcomes after Kasai portoenterostomy.


Subject(s)
Biliary Atresia , Inflammation , Portoenterostomy, Hepatic , Humans , Retrospective Studies , Male , Female , Portoenterostomy, Hepatic/methods , Biliary Atresia/surgery , Infant , Inflammation/immunology , Treatment Outcome , Survival Rate , Biomarkers/blood , ROC Curve
15.
BMC Pediatr ; 24(1): 380, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38824506

ABSTRACT

BACKGROUND: The survival rate of children with biliary atresia (BA) after liver transplantation (LT) is significantly improved, and their quality of life has attracted much attention.This study aimed to investigate the cognition and its influencing factors in children with BA after primary living donor LT (BA-pLDLT) during infancy. METHODS: Children with BA were recruited 6 months after pLDLT at Children's Hospital of Chongqing Medical University (2018-2022). Demographic and clinical data were collected from the health information system. Cognition was assessed using the Chinese version of the Griffiths Mental Development scale (GMDS-C). Multivariate linear regression were used to analyze the influencing factors of their cognitive function. RESULTS: In total, 57 children with BA-pLDLT, aged 5.00(3.90-9.30) months at transplantation and 25.00(14.00-60.80) months at evaluation were included. The general developmental quotient (89.02 ± 12.07) and motor, language, eye-hand coordination, performance, and practical reasoning quotients of these children were significantly lower than the normative mean values of GMDS-C(P < 0.05). Of the 57 children, 16 (28.07%) had borderline developmental delay (DQ between 70 and 84), 3 (5.26%) had developmental delay (DQ < 70), and 11(19.29%) had language delay. Reoperation for biliary or vascular complications after pLDLT was a risk factor for decreased general development quotient and motor quotient and lower ZW at assessment was associated with decline motor quotient. CONCLUSION: Children with BA-pLDLT have varying degrees of developmental delays in early life. Reoperation and nutritional deficiencies had adverse effects on cognitive development.


Subject(s)
Biliary Atresia , Cognition , Liver Transplantation , Living Donors , Humans , Biliary Atresia/surgery , Liver Transplantation/adverse effects , Male , Female , Infant , Child, Preschool , Developmental Disabilities/etiology
16.
Wiad Lek ; 77(3): 577-584, 2024.
Article in English | MEDLINE | ID: mdl-38691803

ABSTRACT

OBJECTIVE: Aim: to review information resources and analysis of the own experience on this problem for the provision of modern knowledge in the pathogenesis of the pathology, the latest diagnostic and treatment technologies, with consideration of the need to adhere to a single strategy in the management of patients with BA. PATIENTS AND METHODS: Materials and Methods: The analysis of the data regarding the results of existing studies evaluating the clinical benefit and safety of diagnostic and treatment methods in Biliary atresia. CONCLUSION: Conclusions: BA is the leading cause of neonatal cholestasis development. Early diagnostics of BA, based on the complex evaluation of clinical-laboratory, instrumental and morphological signs of the pathology, has a significant meaning. Surgical correction during the first 2 months of life - the Kasai procedure, as well as dynamic post-surgery follow-up significantly prolong the life of children and allow postponing liver transplantation. The highest patient survival both at the first stage of treatment - conduction of the Kasai procedure and the stage of liver transplantation may be achieved by joined work of surgeons and pediatricians, which allows considering the whole row of possible problems.


Subject(s)
Biliary Atresia , Child , Humans , Infant , Infant, Newborn , Biliary Atresia/diagnosis , Biliary Atresia/pathology , Biliary Atresia/surgery , Liver Transplantation
18.
J Pediatric Infect Dis Soc ; 13(5): 282-284, 2024 May 30.
Article in English | MEDLINE | ID: mdl-38588463

ABSTRACT

For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population.


Subject(s)
Anti-Bacterial Agents , Antibiotic Prophylaxis , Biliary Atresia , Portoenterostomy, Hepatic , Humans , Anti-Bacterial Agents/therapeutic use , Biliary Atresia/surgery , Practice Guidelines as Topic
19.
World J Gastroenterol ; 30(9): 1043-1072, 2024 Mar 07.
Article in English | MEDLINE | ID: mdl-38577180

ABSTRACT

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.


Subject(s)
Bile Duct Diseases , Biliary Atresia , Choledochal Cyst , Infant , Child , Infant, Newborn , Humans , Bile Ducts/diagnostic imaging , Bile Ducts/surgery , Biliary Atresia/diagnosis , Biliary Atresia/surgery , Choledochal Cyst/diagnosis , Choledochal Cyst/diagnostic imaging , Bile Duct Diseases/diagnosis , Bile Duct Diseases/etiology , Bile Duct Diseases/therapy , Cholangiography
20.
J Pediatr Surg ; 59(7): 1362-1368, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38614948

ABSTRACT

BACKGROUND: Biliary atresia is the most common cause of obstructive jaundice in infants and conventional cholangiography is the current diagnostic gold standard. Fluorescent cholangiography with indocyanine green can enhance biliary tree visualization during surgery because it is exclusively excreted into the bile ducts and eventually into the intestine. Therefore, we hypothesized that indocyanine green presence in stool could confirm bile duct patency in infants. METHODS: A prospective single center cohort study was performed on infants (age ≤ 12 months) with and without jaundice after obtaining IRB approval. Indocyanine green was administered intravenously (0.1 mg/kg). Soiled diapers collected post-injection were imaged for fluorescence. RESULTS: After indocyanine green administration, fluorescence was detected in soiled diapers for control patients (n = 4, x = 14 h22 m post-injection) and jaundiced patients without biliary atresia (n = 11, x = 13 h28 m post-injection). For biliary atresia patients (n = 7), post-injection soiled diapers before and after Kasai portoenterostomy were collected. Fluorescence was not detected in stool from 6 of 7 biliary atresia patients. As a test, indocyanine green detection in stool was 97% accurate for assessing biliary patency. CONCLUSION: Fluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants. LEVEL OF EVIDENCE: Level III.


Subject(s)
Biliary Atresia , Coloring Agents , Feces , Indocyanine Green , Humans , Biliary Atresia/diagnostic imaging , Biliary Atresia/surgery , Biliary Atresia/complications , Pilot Projects , Infant , Feces/chemistry , Prospective Studies , Male , Female , Coloring Agents/administration & dosage , Cholangiography/methods , Portoenterostomy, Hepatic , Optical Imaging/methods , Jaundice, Obstructive/etiology , Jaundice, Obstructive/diagnostic imaging , Infant, Newborn
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