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1.
Wiad Lek ; 77(3): 577-584, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38691803

RESUMEN

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.


Asunto(s)
Atresia Biliar , Trasplante de Hígado , Humanos , Atresia Biliar/cirugía , Atresia Biliar/diagnóstico , Recién Nacido , Lactante , Niño
2.
J Proteome Res ; 23(5): 1744-1756, 2024 May 03.
Artículo en Inglés | MEDLINE | ID: mdl-38569191

RESUMEN

Early diagnosis of biliary atresia (BA) is crucial for improving the chances of survival and preserving the liver function of pediatric patients with BA. Herein, we performed proteomics analysis using data-independent acquisition (DIA) and parallel reaction monitoring (PRM) to explore potential biomarkers for the early diagnosis of BA compared to other non-BA jaundice cases. Consequently, we detected and validated differential protein expression in the plasma of patients with BA compared to the plasma of patients with intrahepatic cholestasis. Bioinformatics analysis revealed the enriched biological processes characteristic of BA by identifying the differential expression of specific proteins. Signaling pathway analysis revealed changes in the expression levels of proteins associated with an alteration in immunoglobulin levels, which is indicative of immune dysfunction in BA. The combination of polymeric immunoglobulin receptor expression and immunoglobulin lambda variable chain (IGL c2225_light_IGLV1-47_IGLJ2), as revealed via machine learning, provided a useful early diagnostic model for BA, with a sensitivity of 0.8, specificity of 1, accuracy of 0.89, and area under the curve value of 0.944. Thus, our study identified a possible effective plasma biomarker for the early diagnosis of BA and could help elucidate the underlying mechanisms of BA.


Asunto(s)
Atresia Biliar , Biomarcadores , Diagnóstico Precoz , Proteómica , Atresia Biliar/diagnóstico , Atresia Biliar/sangre , Humanos , Biomarcadores/sangre , Proteómica/métodos , Femenino , Lactante , Masculino , Biología Computacional/métodos , Aprendizaje Automático , Sensibilidad y Especificidad
3.
Comput Biol Med ; 174: 108439, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38643596

RESUMEN

Cholestasis, characterized by the obstruction of bile flow, poses a significant concern in neonates and infants. It can result in jaundice, inadequate weight gain, and liver dysfunction. However, distinguishing between biliary atresia (BA) and non-biliary atresia in these young patients presenting with cholestasis poses a formidable challenge, given the similarity in their clinical manifestations. To this end, our study endeavors to construct a screening model aimed at prognosticating outcomes in cases of BA. Within this study, we introduce a wrapper feature selection model denoted as bWFMVO-SVM-FS, which amalgamates the water flow-based multi-verse optimizer (WFMVO) and support vector machine (SVM) technology. Initially, WFMVO is benchmarked against eleven state-of-the-art algorithms, with its efficiency in searching for optimized feature subsets within the model validated on IEEE CEC 2017 and IEEE CEC 2022 benchmark functions. Subsequently, the developed bWFMVO-SVM-FS model is employed to analyze a cohort of 870 consecutively registered cases of neonates and infants with cholestasis (diagnosed as either BA or non-BA) from Xinhua Hospital and Shanghai Children's Hospital, both affiliated with Shanghai Jiao Tong University. The results underscore the remarkable predictive capacity of the model, achieving an accuracy of 92.639 % and specificity of 88.865 %. Gamma-glutamyl transferase, triangular cord sign, weight, abnormal gallbladder, and stool color emerge as highly correlated with early symptoms in BA infants. Furthermore, leveraging these five significant features enhances the interpretability of the machine learning model's performance outcomes for medical professionals, thereby facilitating more effective clinical decision-making.


Asunto(s)
Atresia Biliar , Colestasis , Máquina de Vectores de Soporte , Humanos , Atresia Biliar/diagnóstico , Lactante , Recién Nacido , Masculino , Femenino , Aprendizaje Automático , Diagnóstico Precoz
4.
World J Gastroenterol ; 30(9): 1043-1072, 2024 Mar 07.
Artículo en Inglés | MEDLINE | ID: mdl-38577180

RESUMEN

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.


Asunto(s)
Enfermedades de los Conductos Biliares , Atresia Biliar , Quiste del Colédoco , Lactante , Niño , Recién Nacido , Humanos , Conductos Biliares/diagnóstico por imagen , Conductos Biliares/cirugía , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Quiste del Colédoco/diagnóstico , Quiste del Colédoco/diagnóstico por imagen , Enfermedades de los Conductos Biliares/diagnóstico , Enfermedades de los Conductos Biliares/etiología , Enfermedades de los Conductos Biliares/terapia , Colangiografía
5.
J Pediatr Gastroenterol Nutr ; 78(5): 1038-1046, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38567627

RESUMEN

OBJECTIVES: To identify and distinguish between racial and socioeconomic disparities in age at hepatology care, diagnosis, access to surgical therapy, and liver transplant-free survival in patients with biliary atresia (BA). METHODS: Single-center retrospective cohort study of 69 BA patients from 2010 to 2021. Patients were grouped into White and non-White cohorts. The socioeconomic milieu was analyzed utilizing neighborhood deprivation index, a census tract-based calculation of six socioeconomic variables. The primary outcomes of this study were timing of the first hepatology encounter, surgical treatment with hepatic portoenterostomy (HPE), and survival with native liver (SNL) at 2 years. RESULTS: Patients were 55% male and 72% White. White patients were referred at a median of 34 days (interquartile range [IQR]: 17-65) vs. 67 days (IQR: 42-133; p = 0.001) in non-White patients. White infants were more likely to undergo HPE (42/50 patients; 84%) compared to non-White (10/19; 53%), odds ratio (OR) 4.73 (95% confidence interval: 1.46-15.31; p = 0.01). Independent of race, patients exposed to increased neighborhood-level deprivation were less likely to receive HPE (OR: 0.49, p = 0.04) and achieve SNL (OR: 0.54, p = 0.02). CONCLUSIONS: Racial and socioeconomic disparities are independently associated with timely BA diagnosis, access to surgical treatment, and transplant-free survival. Public health approaches to improve screening for pathologic jaundice in infants of diverse racial backgrounds and to test and implement interventions for socioeconomically at-risk families are needed.


Asunto(s)
Atresia Biliar , Disparidades en Atención de Salud , Portoenterostomía Hepática , Factores Socioeconómicos , Humanos , Atresia Biliar/cirugía , Atresia Biliar/diagnóstico , Atresia Biliar/etnología , Atresia Biliar/mortalidad , Masculino , Estudios Retrospectivos , Femenino , Lactante , Disparidades en Atención de Salud/estadística & datos numéricos , Disparidades en Atención de Salud/etnología , Trasplante de Hígado/estadística & datos numéricos , Recién Nacido , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Población Blanca/estadística & datos numéricos , Disparidades Socioeconómicas en Salud
6.
JAMA Netw Open ; 7(1): e2350044, 2024 Jan 02.
Artículo en Inglés | MEDLINE | ID: mdl-38170523

RESUMEN

Importance: Investigations into the association of antepartum maternal infections with the pathogenesis of biliary atresia (BA) in human offspring are insufficient. Objective: To examine the association between prenatal infections in mothers and the development of BA in their offspring. Design, Setting, and Participants: This population-based case-control study obtained administrative data from the Taiwan National Health Insurance Research Database with linkage to the Taiwan Maternal and Child Health Database, capturing demographic and medical information on nearly all 23 million of the Taiwan population. The cohort comprised 2 905 978 singleton live births among mother-infant dyads between January 1, 2004, and December 31, 2020, in Taiwan. The case group of infants with BA was identified from use of International Classification of Diseases diagnostic codes for BA and subsequent Kasai procedure or liver transplant. The control group was randomly selected from infants without BA, representing approximately 1 in 1000 study population. Data analyses were performed from May 1 to October 31, 2023. Exposure: Prenatal maternal infections, including intestinal infection, influenza, upper airway infection, pneumonia, soft-tissue infection, and genitourinary tract infection. Main Outcomes and Measures: The main outcome was exposure to prenatal maternal infections. Inverse probability weighting analysis was performed by building a logistic regression model to estimate the probability of the exposure observed for a particular infant and using the estimated probability as a weight in subsequent analyses. The weighted odds ratio (OR) estimated by logistic regressions was then used to assess the risk of BA in offspring after prenatal maternal infections. Results: Among the mother-infant dyads included, 447 infants with BA were cases (232 females [51.9%]) and 2912 infants without BA were controls (1514 males [52.0%]). The mean (SD) maternal age at childbirth was 30.7 (4.9) years. Offspring exposed to prenatal intestinal infection (weighted OR, 1.46; 95% CI, 1.17-1.82) and genitourinary tract infection (weighted OR, 1.22; 95% CI, 1.05-1.41) in mothers exhibited a significantly higher risk of BA. Furthermore, maternal intestinal infection (weighted OR, 6.05; 95% CI, 3.80-9.63) and genitourinary tract infection (weighted OR, 1.55; 95% CI, 1.13-2.11) that occurred during the third trimester were associated with an increased risk of BA in offspring. Conclusions and Relevance: Results of this case-control study indicate an association between prenatal intestinal infection and genitourinary tract infection in mothers and BA occurrence in their offspring. Further studies are warranted to explore the underlying mechanisms of this association.


Asunto(s)
Atresia Biliar , Adulto , Femenino , Humanos , Masculino , Embarazo , Atresia Biliar/epidemiología , Atresia Biliar/etiología , Atresia Biliar/diagnóstico , Estudios de Casos y Controles , Modelos Logísticos , Tercer Trimestre del Embarazo , Recién Nacido
7.
Pediatr Surg Int ; 40(1): 42, 2024 Jan 30.
Artículo en Inglés | MEDLINE | ID: mdl-38289412

RESUMEN

Biliary atresia is an occlusive biliary disease involving intrahepatic and extrahepatic bile ducts. Its etiology and pathogenesis are unclear. There are many manifestations of bile duct involvement in biliary atresia, but little is known about its occurrence and development. In addition, different classification methods have been proposed in different periods of biliary atresia, each with its advantages and disadvantages. The combined application of biliary atresia classification will help to improve the survival rate of patients with native liver. Therefore, this article reviews the development, pathological features, and classification of intrahepatic and extrahepatic bile ducts in biliary atresia, to provide a reference for the study of the pathogenesis and the choice of treatment methods.


Asunto(s)
Conductos Biliares Extrahepáticos , Atresia Biliar , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Hígado
8.
Hepatol Commun ; 8(1)2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-38180987

RESUMEN

BACKGROUND: Mitochondrial hepatopathies (MHs) are primary mitochondrial genetic disorders that can present as childhood liver disease. No recognized biomarkers discriminate MH from other childhood liver diseases. The protein biomarkers growth differentiation factor 15 (GDF15) and fibroblast growth factor 21 (FGF21) differentiate mitochondrial myopathies from other myopathies. We evaluated these biomarkers to determine if they discriminate MH from other liver diseases in children. METHODS: Serum biomarkers were measured in 36 children with MH (17 had a genetic diagnosis); 38 each with biliary atresia, α1-antitrypsin deficiency, and Alagille syndrome; 20 with NASH; and 186 controls. RESULTS: GDF15 levels compared to controls were mildly elevated in patients with α1-antitrypsin deficiency, Alagille syndrome, and biliary atresia-young subgroup, but markedly elevated in MH (p<0.001). FGF21 levels were mildly elevated in NASH and markedly elevated in MH (p<0.001). Both biomarkers were higher in patients with MH with a known genetic cause but were similar in acute and chronic presentations. Both markers had a strong performance to identify MH with a molecular diagnosis with the AUC for GDF15 0.93±0.04 and for FGF21 0.90±0.06. Simultaneous elevation of both markers >98th percentile of controls identified genetically confirmed MH with a sensitivity of 88% and specificity of 96%. In MH, independent predictors of survival without requiring liver transplantation were international normalized ratio and either GDF15 or FGF21 levels, with levels <2000 ng/L predicting survival without liver transplantation (p<0.01). CONCLUSIONS: GDF15 and FGF21 are significantly higher in children with MH compared to other childhood liver diseases and controls and, when combined, were predictive of MH and had prognostic implications.


Asunto(s)
Síndrome de Alagille , Atresia Biliar , Factor 15 de Diferenciación de Crecimiento , Enfermedad del Hígado Graso no Alcohólico , Niño , Humanos , Síndrome de Alagille/diagnóstico , Atresia Biliar/diagnóstico , Biomarcadores , Factor 15 de Diferenciación de Crecimiento/sangre , Factor 15 de Diferenciación de Crecimiento/química , Enfermedades Mitocondriales/diagnóstico
9.
Saudi J Gastroenterol ; 30(2): 89-95, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37706420

RESUMEN

BACKGROUND: We utilized the data from the Saudi national biliary atresia (BA) study (2000-2018) to describe the clinical, biochemical, imaging, and histopathological features of BA and the perioperative clinical practices among local pediatric gastroenterologists. METHODS: This is a retrospective, multicenter, nationwide study that included 10 tertiary care governmental hospitals including the four liver transplant (LT) centers in different regions across Saudi Arabia. RESULTS: BA was diagnosed in 204 infants (106 females; 10% preterm). The median age at referral was 65 days. Congenital anomalies were present in 68 patients (33%); 22 were splenic malformation (10.8%). The medians of laboratory investigations were total bilirubin (189 µmol/l), direct bilirubin (139 µmol/l), ALT (164 u/l), and GGT (472 u/l). The level of serum GGT was normal in 26 cases (12.7%). The ultrasound findings included hypoplastic or atrophic gall bladder (GB) (65%), normal GB (30%), and cord sign (5%). A HIDA scan was performed in 99 cases (48.52%). Magnetic resonance cholangiopancreatography (MRCP) was performed in 27 cases (13%). A total of 179 liver biopsies (88%) were obtained. The most common histopathologic findings were bile duct proliferation (92%), canalicular cholestasis (96%), bile plugs (84%), and portal fibrosis (95%). Cholangiography was performed in 139 cases (68%): operative in 122 (60%) and percutaneous in 17 (8%). A total of 143 children (70%) underwent Kasai portoenterostomy (KPE) at a median age of 70 days. After KPE, steroid was used in 37% of the cases and 100 cases (70%) were prescribed prophylactic antibiotics for variable duration (ranging between 3 and 12 months). CONCLUSION: Our data show marked variation in the diagnostic evaluation and perioperative management of BA cases among the different tertiary centers. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize pre- and postoperative clinical practices. Additionally, normal serum GGT level, normal GB size on ultrasound, and being a premature baby should not preclude the diagnostic workup for BA.


Asunto(s)
Enfermedades de los Conductos Biliares , Atresia Biliar , Lactante , Niño , Femenino , Recién Nacido , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Estudios Retrospectivos , Arabia Saudita/epidemiología , Bilirrubina
10.
Pediatr Res ; 95(1): 302-307, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37726543

RESUMEN

BACKGROUND: Acute cholangitis is an ominous complication in biliary atresia (BA) patients. We investigated the prevalence of small intestine bacterial overgrowth (SIBO) in BA patients and its role in predicting acute cholangitis. METHODS: There are 69 BA patients with native liver recruited into this study prospectively. They received hydrogen and methane-based breath testing (HMBT) to detect SIBO after recruitment and were followed prospectively in our institute. RESULTS: There are 16 (23.19%) subjects detected to have SIBO by HMBT. BA subjects with SIBO were noted to have higher serum alanine aminotransferase levels than others without SIBO (P = 0.03). The risk of acute cholangitis is significantly higher in BA patients with SIBO than in others without SIBO (62.50% vs. 15.09%, P < 0.001). The logistic regression analysis demonstrated that BA subjects with SIBO have a higher risk of acute cholangitis than others without SIBO (odds ratio = 9.38, P = 0.001). Cox's proportional hazard analysis further confirmed the phenomena in survival analysis (hazard ratio = 6.43, P < 0.001). CONCLUSIONS: The prevalence of SIBO in BA patients is 23.19% in this study. The presence of SIBO is associated with the occurrence of acute cholangitis in BA patients. IMPACT: What is the key message of your article? Acute cholangitis is common in BA, and is associated with SIBO after hepatoportoenterostomy in this study. What does it add to the existing literature? This study demonstrated that SIBO is common in BA after hepatoportoenterostomy, and is predictive of acute cholangitis and elevated serum ALT levels in BA. What is the impact? This prospective cohort study provides data regarding the significance of SIBO on the risk of acute cholangitis in BA patients.


Asunto(s)
Infecciones Bacterianas , Atresia Biliar , Colangitis , Humanos , Prevalencia , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico , Atresia Biliar/epidemiología , Estudios Prospectivos , Intestino Delgado/microbiología , Infecciones Bacterianas/complicaciones , Infecciones Bacterianas/diagnóstico , Infecciones Bacterianas/epidemiología , Pruebas Respiratorias , Colangitis/epidemiología
11.
Virchows Arch ; 484(1): 93-102, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38008855

RESUMEN

A liver biopsy is essential for the diagnostic workup of persistent neonatal cholestasis (NC). The differential diagnosis of NC is broad, including obstructive and non-obstructive causes. In addition, histologic features of certain disorders may be non-specific in the early course of the disease. To evaluate liver biopsies using a practical histopathologic approach for NC and to define a simple scoring system for biliary atresia (BA) for routine clinical practice. From June 2006 to December 2021, liver biopsy specimens from infants with persistent NC were examined by two independent pathologists. The cases diagnosed as BA were correlated with clinical, radiologic, and laboratory data to calculate the final score. Four hundred and fifty-nine cases were enrolled in the study. They had a mean age of 63.94 ± 20.62 days and were followed for a median time of 58 (1-191) months. They included 162 (35.3%) cases of BA. On multivariate analysis, portal edema, ductular proliferation, cholangiolitis, and bile duct/ductular plugs were the histopathologic predictors of BA. A liver biopsy did perform well with a 95.1% sensitivity, 91.6% specificity, 86% PPV, and 97.1% NPV. At a cutoff of 5 of the scoring system, diagnosis of BA could be done with a sensitivity of 95.1% and a specificity of 100%. We have shown detailed histopathologic features of BA with more depth to infants aged ≤ 6 weeks. We have developed a simple scoring system using a combination of liver biopsy with non-invasive methods to increase the diagnostic accuracy of BA.


Asunto(s)
Atresia Biliar , Colestasis , Hepatopatías , Lactante , Recién Nacido , Humanos , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Atresia Biliar/diagnóstico , Atresia Biliar/complicaciones , Atresia Biliar/patología , Hígado/patología , Sensibilidad y Especificidad , Colestasis/diagnóstico , Hepatopatías/patología , Biopsia , Diagnóstico Diferencial
12.
J Pediatr Surg ; 59(4): 648-652, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38145921

RESUMEN

PURPOSE: Serum matrix metalloproteinase-7 (MMP-7) levels can precisely differentiate biliary atresia (BA) from non-BA cholestasis. However, serum MMP-7 levels of some BA patients were within normal range or slightly elevated. This study aimed to investigate the clinical characteristics and prognosis of biliary atresia with low serum MMP-7 levels. METHOD: This is a retrospective cohort study. Cases of BA from July 2020 to December 2022 were consecutively enrolled. They were divided into low-MMP-7 group (MMP-7 ≤ 25 ng/ml) and high-MMP-7 group (MMP-7 > 25 ng/ml) according to serum MMP-7 levels preoperatively. The perioperative clinical characteristics, the 3-month and 6-month jaundice clearance rate post-Kasai procedure, and the native liver survival were compared between the two groups. RESULTS: A total of 329 cases were included in this study, 40 of which were divided into the low-MMP-7 group. Preoperative GGT and direct bilirubin levels in the low-MMP-7 group were significantly lower than those in the high-MMP-7 group (258.6 U/L, interquartile range [IQR]: 160.4411.6 vs. 406.8 IU/L, IQR: 215,655.0, P = 0.0076; 103.8 µmol/L, IQR: 79.0,121.4 vs. 115.3 µmol/L, IQR: 94,138.8, P = 0.0071), while the gender, the day at surgery and preoperative ALT, AST, TBA, total bilirubin levels showed no significant differences (P > 0.05). The 3-month and 6-month jaundice clearance rate post-Kasai procedure in the low-MMP-7 group were lower than those in the high-MMP-7 group (29.73% vs. 53.09%, P = 0.049; 32.14% vs. 54.73%, P = 0.023). The 1-year native liver survival rate was 29.63% for the low-MMP-7 group and 53.02% for the high-MMP-7 group (P = 0.022). CONCLUSION: Preoperative clinical characteristics were similar between low-MMP-7 group and high-MMP-7 group, while patients with low serum MMP-7 levels showed worse prognosis, indicating that this might be listed as a new clinical subtype of BA which could contribute to designing new treatment strategies for BA in the future. STUDY TYPE: Cohort Study. LEVEL OF EVIDENCE: Level II.


Asunto(s)
Atresia Biliar , Ictericia , Humanos , Lactante , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Metaloproteinasa 7 de la Matriz , Estudios Retrospectivos , Estudios de Cohortes , Pronóstico , Portoenterostomía Hepática , Ictericia/cirugía , Bilirrubina
13.
Indian J Pathol Microbiol ; 66(4): 790-798, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38084534

RESUMEN

Background: Cholestatic disorders are a significant cause of morbidity and mortality in infants. Characterization of these disorders and differentiating biliary atresia (BA) from other causes of intrahepatic cholestasis is an age-old problem. Objectives: To study the spectrum of different infantile cholestatic disorders in our population, to differentiate BA from other causes of neonatal cholestasis (NC) on a liver biopsy, and validation of the available scoring system for the characterization of these disorders. Materials and Methods: This is an observational cross-sectional study performed over a period of 3 years between 2018 and 2021, done on neonates and infants presenting with cholestatic jaundice. The changes on liver biopsy were evaluated by different histological parameters and available scoring systems to differentiate BA from non-BA causes. Correlation with clinical, biochemical, and imaging findings was done in all cases. Results: This study included 87 cases of NC, of which BA comprised 28 cases (32%), whereas idiopathic neonatal hepatitis (INH) comprised only 12 cases (14%). Portal neutrophilic inflammation (P = 0.000053), ductal cholestasis (P < 0.001), neoductular bile plugs (P < 0.001) and bile ductular proliferation (P < 0.0001) were significantly more in BA, whereas lobular lymphocytic inflammation (P = 0.001) and giant cell transformation of hepatocytes (P = 0.0024) were more frequent in the non-BA group. Using the Lee and Looi scoring system, a histologic score ≥7 was helpful in identifying BA with 85.7% sensitivity, 92.6% specificity, and 90.6% accuracy. Conclusion: BA is the commonest cause of NC in neonates, whereas the frequency of INH is declining. Detailed histomorphologic analysis of liver biopsy, aided with IHC, is the cornerstone for the diagnosis of these disorders.


Asunto(s)
Atresia Biliar , Colestasis Intrahepática , Colestasis , Lactante , Recién Nacido , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/complicaciones , Atresia Biliar/patología , Hígado/patología , Estudios Transversales , Sensibilidad y Especificidad , Colestasis/diagnóstico , Colestasis/etiología , Colestasis/patología , Biopsia , Colestasis Intrahepática/diagnóstico , Inflamación/patología , Diagnóstico Diferencial
14.
Hepatol Commun ; 7(12)2023 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-38051554

RESUMEN

BACKGROUND: We aimed to quantify ductular reaction (DR) in biliary atresia using a neural network in relation to underlying pathophysiology and prognosis. METHODS: Image-processing neural network model was applied to 259 cytokeratin-7-stained native liver biopsies of patients with biliary atresia and 43 controls. The model quantified total proportional DR (DR%) composed of portal biliary epithelium (BE%) and parenchymal intermediate hepatocytes (PIH%). The results were related to clinical data, Sirius Red-quantified liver fibrosis, serum biomarkers, and bile acids. RESULTS: In total, 2 biliary atresia biopsies were obtained preoperatively, 116 at Kasai portoenterostomy (KPE) and 141 during post-KPE follow-up. DR% (8.3% vs. 5.9%, p=0.045) and PIH% (1.3% vs. 0.6%, p=0.004) were increased at KPE in patients remaining cholestatic postoperatively. After KPE, patients with subsequent liver transplantation or death showed an increase in DR% (7.9%-9.9%, p = 0.04) and PIH% (1.6%-2.4%, p = 0.009), whereas patients with native liver survival (NLS) showed decreasing BE% (5.5%-3.0%, p = 0.03) and persistently low PIH% (0.9% vs. 1.3%, p = 0.11). In Cox regression, high DR predicted inferior NLS both at KPE [DR% (HR = 1.05, p = 0.01), BE% (HR = 1.05, p = 0.03), and PIH% (HR = 1.13, p = 0.005)] and during follow-up [DR% (HR = 1.08, p<0.0001), BE% (HR = 1.58, p = 0.001), and PIH% (HR = 1.04, p = 0.008)]. DR% correlated with Sirius red-quantified liver fibrosis at KPE (R = 0.47, p<0.0001) and follow-up (R = 0.27, p = 0.004). A close association between DR% and serum bile acids was observed at follow-up (R = 0.61, p<0.001). Liver fibrosis was not prognostic for NLS at KPE (HR = 1.00, p = 0.96) or follow-up (HR = 1.01, p = 0.29). CONCLUSIONS: DR predicted NLS in different disease stages before transplantation while associating with serum bile acids after KPE.


Asunto(s)
Atresia Biliar , Aprendizaje Profundo , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Pronóstico , Portoenterostomía Hepática/métodos , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/cirugía , Cirrosis Hepática/complicaciones , Ácidos y Sales Biliares
16.
Acta Biochim Pol ; 70(3): 475-480, 2023 Sep 17.
Artículo en Inglés | MEDLINE | ID: mdl-37717264

RESUMEN

The aim of this review is to specify new potential reliable and non-invasive methods for the diagnosis of biliary atresia (BA) that could shorten the way to diagnose BA, and finally the surgical treatment. Apart from the biomarkers that have been proven helpful and are used nowadays in neonatal wards, there are several new potential biomarkers that researchers have found to be helpful in the diagnosis of biliary atresia. Circulating microRNAs, matrix metalloproteinase-7, stool proteins, interleukin-33, Th17-associated cytokines, urinary metabolomics, anti-smooth muscle antibodies, heat shock proteins 90 and positive biliary epithelial cells CD56 are among those presented in this summary. These markers may play a new significant role in BA diagnosis. The described methods include Nomogram, Circulating microRNAs (miRNAs), Matrix metalloproteinase-7 (MMP-7), Stool proteins, Interleukin-33 (IL-33), Th17-associated cytokines, Alpha-aminoadipic acid and N-acetyl-d-mannosamine in urine, Anti-smooth muscle antibodies (ASMA), Heat shock proteins 90 (HSP90), Positive biliary epithelial cells CD56.


Asunto(s)
Atresia Biliar , MicroARNs , Recién Nacido , Humanos , Atresia Biliar/diagnóstico , Proteínas HSP90 de Choque Térmico , Interleucina-33 , Metaloproteinasa 7 de la Matriz , Autoanticuerpos , Biomarcadores , Citocinas
17.
J Pediatr Gastroenterol Nutr ; 77(6): e93-e98, 2023 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-37697468

RESUMEN

PURPOSE: Recent studies demonstrate the success of Kasai portoenterostomy for biliary atresia (BA) is linearly related to infant age at time of Kasai. We sought to review the feasibility and safety of laparoscopic needle micropuncture cholangiogram with concurrent core liver biopsy (if needed) for expedited exclusion of BA in patients with direct conjugated hyperbilirubinemia. METHODS: Expedited laparoscopic cholangiogram and liver biopsy were instituted at our facility for infants with direct hyperbilirubinemia for whom clinical exam and laboratory workup failed to diagnose. A retrospective chart review was performed in infants <1 year with hyperbilirubinemia from 2016 to 2021. Demographics, preoperative evaluation, procedure details, and complications were reviewed. RESULTS: Two hundred ninety-seven infants with unspecified jaundice were identified, of which, 86 (29%) required liver biopsy. Forty-seven percutaneous liver biopsies were obtained including 8 (17%) in whom BA could not be excluded. Laparoscopic cholangiogram was attempted in 47 infants following basic workup; BA was diagnosed in 22 infants (47%) of which 3 were <18 days old. Biliary patency was demonstrated laparoscopically in 22 of 25 (88%); 3 (12%) required conversion to open cholangiogram. Infants with percutaneous liver biopsy had an average delay of 3 days (range: 2-36) to cholangiogram. Preoperative studies and liver biopsy alone did not reliably exclude the diagnosis of BA. CONCLUSION: Laparoscopic cholangiogram with liver biopsy is a safe procedure resulting in the confirmation or exclusion of BA in infants. Forty-seven percent of infants who underwent laparoscopic cholangiogram were found to have BA; those who were surgical candidates underwent Kasai during the same operation.


Asunto(s)
Atresia Biliar , Laparoscopía , Humanos , Lactante , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Atresia Biliar/complicaciones , Biopsia/efectos adversos , Hiperbilirrubinemia/diagnóstico , Laparoscopía/métodos , Hígado/patología , Portoenterostomía Hepática/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Estudios de Factibilidad
18.
J Gastroenterol Hepatol ; 38(9): 1610-1617, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37407246

RESUMEN

BACKGROUND AND AIM: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA. METHODS: From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices. RESULTS: A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices. CONCLUSION: Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding.


Asunto(s)
Atresia Biliar , Várices Esofágicas y Gástricas , Várices , Masculino , Niño , Humanos , Lactante , Várices Esofágicas y Gástricas/diagnóstico , Várices Esofágicas y Gástricas/etiología , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Esplenomegalia/diagnóstico por imagen , Esplenomegalia/etiología , Estudios de Cohortes , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/prevención & control , Endoscopía Gastrointestinal , Cirrosis Hepática
19.
Arch Pediatr ; 30(6): 427-437, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37394364

RESUMEN

Multiple causes of congenital neonatal cholestasis have been identified, and are classified as extrahepatic or intrahepatic. Biliary atresia (BA), Alagille syndrome (AGS), and progressive familial intrahepatic cholestasis (PFIC) are the most common of these. Many factors associated with cholestatic diseases are known to degrade the oral health of these children. What are the oral manifestations associated with these diseases in the pediatric population? The aim of this article was to evaluate the impact of congenital cholestasis on oral health in pediatric patients. A systematic review of case reports and case series was carried out in PubMed, the Cochrane Library, and the Web of Science to identify relevant articles in French and English published up to April 2022. The review included 19 studies, 16 case reports, and three case series. Only studies dealing with BA and AGS were found. These studies showed an impact on jaw morphology, dental structure, and periodontal health. The facial dysmorphism observed in AGS was specific. Exposure to high levels of bilirubin during the period of dental calcification led to particular coloration. Regarding periodontal status, gingival inflammation was common in these patients, probably resulting from the use of certain treatment-associated drugs and poor oral hygiene. Cohort studies are needed to confirm the classification of these children as being at high individual risk of caries. Many major oral manifestations are found in children with AGS and BA, confirming the need to include a dentist in the care team of patients with congenital cholestatic disease as early as possible. It appears necessary to carry out individual prospective studies of each phenotype in order to confirm and better describe the oral impact of these cholestatic diseases and provide adequate medical care.


Asunto(s)
Síndrome de Alagille , Atresia Biliar , Colestasis Intrahepática , Colestasis , Niño , Humanos , Recién Nacido , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico , Estudios Prospectivos , Colestasis/complicaciones , Colestasis/diagnóstico , Colestasis Intrahepática/diagnóstico , Colestasis Intrahepática/complicaciones , Colestasis Intrahepática/congénito , Síndrome de Alagille/complicaciones , Síndrome de Alagille/diagnóstico
20.
Adv Neonatal Care ; 23(5): E107-E113, 2023 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-37442109

RESUMEN

BACKGROUND: Biliary atresia is a rare and serious neonatal disease that affects the quality of life of both infants and parents. There is currently limited literature on the experiences of parents with infants diagnosed with biliary atresia. PURPOSE: To explore the psychological journey and coping styles of parents of infants with biliary atresia in a single center in Shanghai, China. METHODS: A qualitative study design was used. Face-to-face and semistructured interviews were conducted with 10 parents of infants with biliary atresia. Colaizzi's method of data analysis was utilized, using NVivo 11.0 software. RESULTS: The psychological journey and coping styles of parents could be divided into 4 stages. Different themes were extracted at different stages: before diagnosis, parents experienced complex emotions and actively sought treatment; at the diagnosis stage, negative emotions dominated and parents convinced themselves to accept reality; in the postoperative stage, positive emotions, accepting reality, active response, and the need to learn to take care of their infant gradually appeared; and at the discharge stage, parents accepted the coexistence of positive and negative emotions and the variety of needs that emerged. IMPLICATIONS FOR PRACTICE: The findings of the study may help healthcare professionals identify and focus on the psychological needs of parents of infants with biliary atresia, leading them to implement effective coping strategies to increase the caregiving ability of parents. IMPLICATIONS FOR RESEARCH: Future research should explore the effects of supportive interventions for parents of infants with serious chronic illnesses.


Asunto(s)
Atresia Biliar , Recién Nacido , Lactante , Humanos , Atresia Biliar/cirugía , Atresia Biliar/diagnóstico , Calidad de Vida , China , Adaptación Psicológica , Padres/psicología , Investigación Cualitativa
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