Biliary Atresia Research Papers - Academia.edu (original) (raw)

2025, Brazilian Journal of Medical and Biological Research

Biliary atresia, the most common cause of liver transplantation in children, remains a challenge for clinicians and investigators. The development of new therapeutic options, besides the typical hepatoportoenterostomy, depends on a greater understanding of its pathogenesis and how it relates to the clinical phenotypes at diagnosis and the rate of disease progression. In this review, we present a perspective of how recent research has advanced the understanding of the disease and has improved clinical care protocols. Molecular and morphological analyses at diagnosis point to the potential contributions of polymorphism in the CFC1 and VEGF genes, to the pathogenesis of the disease, and to an association between the degree of bile duct proliferation and long-term outcome. In experimental models, cholangiocytes do not appear to have antigen-presenting properties despite a substantial innate and adaptive immune response that targets the biliary epithelium and produces duct obstruction. Initial clinical trials assessing the efficacy of corticosteroids in decreasing the inflammation and improving outcome do not show a superior effect of corticosteroids as an adjuvant treatment following hepatoportoenterostomy. The best outcome still remains linked to an early diagnosis and surgical treatment. In this regard, the Yellow Alert campaign by the Sociedade Brasileira de Pediatria and the inclusion of the Stool Color Card in the health booklet given to every neonate in Brazil have the potential to decrease the age of diagnosis, shorten the time between diagnosis and surgical treatment, and improve the long-term outcome of children with this devastating disease.

2025, Surgical Case Reports

Background: Biliary atresia (BA) cases are generally not associated with congenital abnormalities. However, accurate diagnosis of BA is often challenging because the histopathological features of BA overlap with those of other pediatric liver diseases and rarely overlap with those of other genetic disorders. We experienced a rare case of BA with the histopathological finding of bile duct paucity, a gene mutation in KDM6A, and KS-like phenotypes. Case presentation: A male baby was diagnosed with biliary atresia by intraoperative cholangiography at 4 days of age, and histological examination following a liver biopsy revealed a paucity of bile ducts and several typical clinical findings of Alagille syndrome. However, Alagille syndrome was ruled out after neither JAG1 nor NOTCH2 gene mutations were identified. Whole-exome sequencing on DNA from his parents was additionally performed to examine other possible syndromic disorders, and a mutation was identified in KDM6A. However, Kabuki syndrome was not diagnosed as a result. The histological finding of interlobular bile duct paucity and the genetic mutation in KDM6A, as well as several clinical findings consistent with Alagille syndrome or Kabuki syndrome, made it difficult to confirm the diagnosis of BA. Conclusions: Based on the interesting findings of the present case, we hypothesized that KDM6A is associated with hepatic malformations via a connection with the Notch signaling pathway.

2025, Annals of Pediatric Surgery

Background It is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. This is a rare association. Case reports A 2-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and a preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy). Discussion It is important to carefully look into the relationship between the preduodenal portal vein and biliary atresia because the patient is at risk of injury from this aberrant vein during operative intervention. Conclusion The relationship between biliary atresia and other congenital anomalies like preduodenal portal vein, intestinal malrotation, and situs inversus must be taken into consideration to avoid other diverse effects during surgical intervention.

2025, Journal of Clinical and Translational Hepatology

Biliary atresia (BA) is a congenital cholestatic disease that can seriously damage children's liver function. It is one of the main reasons for liver transplantation in children. Early diagnosis of BA is crucial to the prognosis of patients, but there is still a lack of reliable non-invasive diagnostic methods. Additionally, as some children are in urgent need of liver transplantation, evaluating the stage of liver fibrosis and postoperative native liver survival in children with BA using a straightforward, efficient, and less traumatic method is a major focus of doctors. In recent years, an increasing number of BA-related biomarkers have been identified and have shown great potential in the following three aspects of clinical practice: diagnosis, evaluation of the stage of liver fibrosis, and prediction of native liver survival. This review focuses on the pathophysiological function and clinical application of three novel BA-related biomarkers, namely MMP-7, FGF-19, and M2BPGi. Furthermore, progress in well-known biomarkers of BA such as gamma-glutamyltransferase, circulating cytokines, and other potential biomarkers is discussed, aiming to provide a reference for clinical practice.

2025, Digestive and Liver Disease

2025, Journal of Pediatric Surgery

The aim of the study was to present early outcomes of the laparoscopic technique for biliary atresia with some technical modifications. Materials and Methods: We reviewed charts of all patients with biliary atresia who underwent laparoscopic portoenterostomy from July to December 2008. There were 11 patients with biliary atresia, including 5 boys and 6 girls. The operation was carried out using 4 trocars. The liver was elevated by 2 transcutaneous stay sutures: one on the round ligament and the other on the gallbladder remnant. The left and right hepatic arteries and portal veins were dissected and retracted laterally by 2 transcutaneous sutures to expose the liver hilum. A stay suture was placed on fibrotic tissue at the liver hilum to facilitate its maximal removal. A jejunal end-to-side anastomosis was constructed extracorporeally. Portoenterostomy was carried out laparoscopically. Results: Mean operative time was 245 ± 31 minutes. No patient required conversion. There were no operative deaths. Blood loss during operation was minimal. One patient died on day 65 after operation because of intractable hepatic liver. Follow-up after discharge from 10 to 16 months revealed that 6 patients still survived and 4 patients died. One patient died because of milk aspiration at 12 months of age. Three patients died because of repeated cholangitis and liver failure at 10, 10, and 14 months, respectively. Conclusion: With a modified laparoscopic technique, good early outcomes of laparoscopic surgery for biliary atresia were achieved.

2025, Journal of Comprehensive Pediatrics

We aimed to evaluate the role of aspartate aminotransferase to platelet ratio in the diagnosis of liver fibrosis and its prognosis after surgery in infants with biliary atresia. Methods: This retrospective study was conducted using the medical records of patients diagnosed with biliary atresia who underwent surgery and liver biopsy in Mofid Children's Hospital from 2008 to 2016. The sampling method was complete enumeration and all patients admitted to the hospital in these 8 years were selected as the sample. In this study, an author -made checklist was used. According to data available in the archives of Mofid Children's Hospital, a total of 100 patients with preoperative diagnosis of biliary atresia entered the study. The obtained data were analyzed using descriptive statistics and inferential statistics. The significance level in this study was considered to be 0.05. Results: A total of 100 patients were enrolled in this study, with 64 male patients and 36 female. The patients gave birth at a minimum of 29 weeks and a maximum of 40 weeks. The gestational age was significantly related to the final diagnosis of the disease among children (p = 0.011). With a cut off value of aspartate aminotransferase to platelet ratio of 0.95, 25 patients (50%) with biliary atresia were in the group of significant fibrosis (F4, cirrhosis) and the others were in the non -significant fibrosis group (p value = 0.021). In our study, aspartate aminotransferase to platelet ratio was calculated and showed significant relationship to the final diagnosis based on liver biopsy categorizations among infants with Biliary Atresia after Portoenterostomy.

2025, Journal of Comprehensive Pediatrics

2025, International Journal of Surgery Case Reports

Introduction and importance: Primary biliary cirrhosis (PBC) is a chronic and progressive autoimmune liver disease with no known etiology. This disease is mainly characterized by granulomatous destruction of intrahepatic biliary ducts, severe peri-portal inflammation, and ultimate progress to liver fibrosis and cirrhosis. Here, we report a five-year-old girl diagnosed with PBC, presented to us with end-stage liver disease for liver transplantation. Our patient successfully underwent liver transplantation with an uneventful recovery. This case highlights the need for awareness to report further PBC cases in the pediatric age group. Case presentation: A five-year old female child presented with a 6 months history of progressive jaundice. She had multiple admissions for hepatic encephalopathy and this time she was admitted for hepatic transplantation. On examination, she was icteric and had hepatomegaly. After thorough workup, she underwent successful hepatic transplantation and was alright post-operatively. At 6 months follow up, she is doing well. Conclusion: PBC is rare in childhood. The natural history and exact incidence of PBC in childhood are not known. Hence, there is a need for awareness to report further PBC cases in the pediatric age group.

2025, Nursing & Care Open Access Journal

Purpose: To describes the characteristics of the daily lives of adolescents with biliary atresia surviving with their native liver, and those of their parents, as well as the patient-parent relationship. The participants were adolescents with biliary atresia surviving with their native liver, and their parents. Data were collected through semi-structured interviews individually with patients and parents. And qualitative content analysis was used. The theme of the daily lives of the patients and their parents during the adolescent period involved a change process and be bewildered from "parent and child respectively" to "parent and child together." This theme was composed of three categories; Beginning of anguish of parent and child, Friction between parent and child, and Transition to life and looking ahead to the future. The patients with biliary atresia surviving with their native liver, and their parents, were bewildered because of the ambiguous nature of biliary atresia, the possibility of LDLT, and characteristics of the adolescent developmental stage. Nurses should become the compensating factor to reconstruct patient-parent relationships and support patients in acquiring understanding of their illness and experiencing suitable developmental stages in school or society.

2025, Journal of Pediatric Nursing

BACKGROUND This study examines how the parents of adolescents and young adults with biliary atresia, surviving with their native livers, transfer medical treatment responsibility to their children, and how becoming a living liver transplant donor affects this process. The basis for the study was that becoming a living liver transplant donor may influence the transfer of responsibility. METHODS A qualitative, descriptive design was employed using a modified grounded theory approach. Data were collected from eight parents from November 2016 to August 2017 through semi-structured interviews conducted in two Japanese hospitals. Interpretive analysis from the viewpoint of the analytical theme and analytical focus person was conducted, resulting in a diagram and storyline explaining the process. FINDINGS Five categories were generated from one father and seven mothers (aged 40-60 years) of outpatients (aged 17-25 years): parental ability to cherish the child's "now" (live in the moment); put the brakes on their child's life; learn as they go; leave the child's life to the child; and openly express reservations about becoming a living liver transplant donor for their child. DISCUSSION Parental transfer of responsibility for treatment may be influenced by close-knit parent-child relationships, perception of the child's growth, and parental concern about becoming a living donor. APPLICATION TO PRACTICE Care providers should understand parent-child relationships and the parents' concerns about becoming living donors, thereby enabling them to recognize that medical treatment control must be relinquished to foster children's autonomy as adults.

2025, International Journal of Nursing Practice

The purpose of the current study was to describe the following: maternal coping with the prospect of becoming the living‐donor liver transplant for their child; the daily lives of school‐age children surviving biliary atresia with their native liver; and to explore the relationship between these individuals. Semistructured interviews were conducted with 6 school‐age children surviving biliary atresia with their native liver and their mothers. The interviews were conducted from June to August 2014, and a qualitative content analysis was used. Results showed that mothers realized a possible need for transplantation in the future, which contributes to emotional and practical uncertainties. The mothers coexisted with this uncertainty and preferred to use a buffering strategy. In contrast, the children did not consider their illness and future and did not adhere to a therapeutic regimen. It is suggested that living with uncertainty about the health and survival of their children is advan...

2025, The Journal of Pediatrics

Background allelic variants in normal hemopoietic cells and Bloom's syndrome erythrocytes and the possible implication of somatic crossingover.

2025, Journal of Pediatric Surgery

2025, International journal of research publications

Biliary atresia is an obstructive disease affecting the biliary tract. Patients with biliary atresia often appear similar to normal infants, leading to delayed detection in many cases. There are several clinical symptoms and typical laboratory findings that typically manifest in patients with biliary atresia. As the age of the patient advances, the manifestations worsen, potentially leading to the development of cirrhosis, liver failure by the age of 1, and other complications. The aim of this review is to understand the clinical finding of biliary atresia patients, facilitating early detection and intervention to reduce the likelihood of the disease progressing to a more severe stage. Delay in the diagnosis of biliary atresia can result in a poor prognosis.

2025, Journal of Pediatric Gastroenterology & Nutrition

Objectives: Research is lacking into the emotional effects on families of serious chronic illness in infants. We examined the effect of the diagnosis of serious liver disease in infants upon parent psychological symptoms and family functioning. We hypothesized that parent psychological symptoms, family functioning, and father engagement will predict infant emotional outcomes. Methods: Parents of infants recently diagnosed with serious liver disease completed validated questionnaires about parent stress, family function, impact of the illness on the family, and father engagement. The measures were repeated after 1 year, with the addition of the Child Behavior Checklist (CBCL). Results: Parents of 37 infants participated. Parent stress and family functioning scores were not elevated. Parent psychological symptoms, family function, and father engagement did not predict infant outcome. For mothers, infant diagnosis other than biliary atresia, number of outpatient visits, and impact of t...

2025

Background: One of the most important post-operation problems encountered with Hepaticojejunostomy (Kasai operation) in patients with biliary atresia is cholangitis. The aim of this study was to evaluate the efficiency of anti-reflux valve in decreasing the incidence of cholangitis after hepaticojejunostomy. Methods: Ten dogs were randomly divided into two groups of equal size. In group A Roux-en-Y cholecystojejunostomy was performer and in group B antireflux valve was also added. Three months later, liver biopsy was taken to evaluate histopatholic changes. The incidence of cholangitis was significantly reduced in dogs in which anti-reflux valve procedure was performed. Conclusions: Anti-reflux valve procedure effectively decreased post-Roux-en-Y cholecystojejunostomy cholangitis in dogs. Therefore it is plausible and might be beneficial for patients being treated for biliary atresia.

2025, Anuradhapura Medical Journal

Niemann Pick type C [NP-C] disease is a rare neurodegenerative lysosomal storage disorder marked by an accumulation of unesterified cholesterol in the lysosomal system. It has autosomal recessive inheritance caused by mutations in NPC1 and NPC2 genes. The broad clinical spectrum ranges from a prenatal severe manifestation to an adult-onset chronic neurodegenerative disease. The manifestations in the perinatal period and infancy are predominantly visceral, with hepatosplenomegaly, jaundice, and pulmonary infiltrates (in some instances). Patients with NP-C usually do not show neurological manifestations during the neonatal period. Herein we present a case of neonatal cholestasis with hepatosplenomegaly and anaemia, which was diagnosed as Niemann Pick disease type C2. Thus, NP-C is an important differential diagnosis of neonatal cholestasis in the presence of visceromegaly. .

2025, Liver Transplantation

2025, Hepatology

Current American Association for the Study of Liver Diseases (AASLD) liver transplant evaluation guidelines include both adult and pediatric patients. 1 While pediatric liver transplants account for 7.8% of all liver transplants in the United States, sufficient differences between pediatric and adult patients seeking liver transplantation (LT) now require independent, yet complementary documents. This document will focus on pediatric issues at each level of the evaluation process. Disease categories suitable for referral to a pediatric LT program are similar to adults: acute liver failure, autoimmune, cholestasis, metabolic or genetic, oncologic, vascular, and infectious. However, specific etiologies and outcomes differ widely from adult patients, justifying independent pediatric guidelines. Data supporting our recommendations are based on a Medline search of the English language literature from 1997 to the present. Intended for use by physicians, these recommendations suggest preferred approaches to the diagnostic, therapeutic, and preventive aspects of care. They are intended to be flexible, in contrast to standards of care, which are inflexible policies to be followed in every case. Specific recommendations are based on relevant published information. To more fully characterize the available evidence supporting the recommendations, the AASLD Practice Guidelines Committee has adopted the classification used by the Grading of Recommendation Assessment, Development, and Evaluation (GRADE) workgroup with minor modifications (Table ). The classifications and recommendations are based on three categories: the source of evidence in levels I through III; the

2025, Arquivos De Gastroenterologia

• Jaundice is an important clinical sign that motivates referral to a specialist, but, in this study, it did not compromise survival with native liver in patients with alpha 1-antitrypsin deficiency. • Patients with alpha 1-antitrypsin deficiency who develop portal hypertension had higher AST and APRI scores on admission. • Patients with alpha 1-antitrypsin deficiency who develop portal hypertension have significant impairment of survival with native liver.

2025, Revista Espanola De Enfermedades Digestivas

When cholestatic liver disease is present, liver ultrasound should be performed to ascertain if cholestasis is extrahepatic or intrahepatiic. If bile ducts appear dilated and the probability of interventional treatment is high, endoscopic retrograde cholagiopancreatography (ERCP) or trans-hepatic cholangiography (THC) should be the next step. If the probability of interventional therapeutics is low, cholangio-MRI should be performed. Once bile duct dilation and space occupying lesions are excluded, a work up for intrahepatic cholestasis should be started. Some especific clinical situations may be helpful in the diagnostic strategy. If cholestasis occurs in the elderly, drug-induced cholestatic disease should be suspected, whereas if it occurs in young people with risk factors, cholestatic viral hepatitis is the most likely diagnosis. During the first trimester of pregnancy cholestasis may occur in hiperemesis gravidorum, and in the third trimester of gestation cholestasis of pregnancy should be suspected. A familial history of recurrent cholestasis points to benign recurrent intrahepatic cholestasis. The occurrence of intrahepatic cholestasis in a middle-aged woman is a frequent presentation of primary biliary cirrhosis, whereas primary sclerosing cholangitis should be suspected in young males with inflammatory bowel disease. The presence of vascular spider nevi, ascites, and a history of alcohol abuse should point to alcoholic hepatitis. Neonatal cholestasis syndromes include CMV, toxoplasma and rubinfections or metabolic defects such as cystic fibrosis, α 1 -antitripsin deficiency, bile acid synthesis defects, or biliary atresia. The treatment of cholestasis should include a management of complications such as pruritus, osteopenia and correction of fat soluble vitamin deficiencies. When hepatocellular failure or portal hypertension-related complications occur, liver transplantation should be considered.

2025, Pesquisa Veterinária Brasileira

São descritos os achados macroscópicos e histopatológicos de cistos peribiliares em uma fêmea adulta de leão-africano (Panthera leo). Macroscopicamente, todos os lobos hepáticos continham numerosos cistos preenchidos por líquido translúcido. Histologicamente, os cistos estavam localizados no hilo hepático e nos tratos portais. Os cistos eram revestidos internamente por epitélio simples, constituído por células colunares ou cuboidais e não mucinosas, de acordo com avaliação para ácido periódico de Schiff/azul alciano. Na imuno-histiquímica, todas as células desse epitélio foram positivas para citoceratina. O leão morreu de complicações relacionadas a um carcinoma pulmonar e não foram observadas alterações clínicas associadas à lesão hepática, comprovando que cistos peribiliares em leões também ocorrem não associados a sinais clínicos.

2025, Pediatric Radiology

2025, Pediatric Transplantation

2025, The Turkish journal of gastroenterology

2025, European journal of paediatric dentistry : official journal of European Academy of Paediatric Dentistry

Green stain is an uncommon clinical condition associated with deposition of bilirubin in the dental hard tissues. The staining of the teeth is due to hyperbilirubinemia caused by systemic conditions. The purpose of this report is to present a case of green teeth of the primary dentition in a 17-month old girl associated with hydrocephalus and Hirschprung's disease. The clinical characteristics of the teeth may help in the diagnosis of current or past systemic diseases.

2025, The Journal of pediatrics

To evaluate the sensitivity and specificity of a stool color card used for a mass screening of biliary atresia conducted over 19 years. In addition, the age at Kasai procedure and the long-term probabilities of native liver survival were investigated. From 1994 to 2011, the stool color card was distributed to all pregnant women in Tochigi Prefecture, Japan. Before or during the postnatal 1-month health checkup, the mothers returned the completed stool color card to the attending pediatrician or obstetrician. All suspected cases of biliary atresia were referred for further examination. Diagnosis was confirmed by laparotomy or operative cholangiography for high-risk cases before the Kasai procedure. Patients with biliary atresia were followed from the date of their Kasai procedure until liver transplantation, death, or October 31, 2013, whichever comes sooner. A total of 313 230 live born infants were screened; 34 patients with biliary atresia were diagnosed. The sensitivity and speci...

2025, Outcome of chronic pancreatitis

Background: Chronic pancreatitis is characterized by progressive and irreversible damage of exocrine as well as endocrine functions of the gland that eventually results in significant impairment. Objective: To study the outcome of optimum surgical management of chronic pancreatitis Methods: A hospital based observational follow up study was undertaken among the patients attending surgical units of a tertiary care institute in Assam, who were diagnosed as chronic pancreatitis. Results: Total 50 patients presenting with symptoms of chronic pancreatitis, confirmed by radiological and laboratory investigation were included in the study. Most of the cases were 41-50 years (32%) of age, with a male, female ratio of 7:3. Pain abdomen was the main complaint of each patient (100%), followed by nausea and vomiting in 66% patients. Majority of the patients had epigastric tenderness (38%) followed by tenderness on left hypochondrium. Out of 50 patients, 29 patients treated conservatively and where 6 patients had aggravated symptoms after initial recovery. So, 27 patients required surgical interventions. A systematic follow up of all patients done upto 12 months. It was found that all patients undergoing surgery recovered from abdominal pain, irrespective of the type of surgical procedure. Conclusion: An integrated approach for correct diagnosis and planning early intervention is required for chronic pancreatitis.

2025

2025, Vaccine

Biliary atresia is a neonatal cholangiopathy of unknown etiology that results in obliteration of bile ducts and is the primary indication for liver transplant in children. A murine model of BA, which involves infecting newborn mice with rhesus rotavirus (RRV) and leads to development of an obstructive cholangiopathy, has provided a model to assess measures to prevent and treat BA. We used this mouse model of RRV-induced BA to determine if passive immunization of pups using maternal immunization [injection of dams with non-replicating rotavirus (RV) virus-like particles (VLPs) or live RRV] or injection of pups with RV immune serum would protect these RRVinfected neonates from developing BA (measured using cholestasis). Parenteral immunization of mouse dams with two formulations of VLPs (containing viral proteins 2/6 or 2/6/7) resulted in a significant increase in serum RV antibody, and pups born to these immunized dams were protected from developing cholestasis following neonatal infection with RRV. Serum RV-specific antibody with titers of ≥ 400-800 in dams significantly protected pups from developing cholestasis, and a significant trend of increasing protection with high titers was observed (p>0.0001). Cholestatic pups had lower levels of RV serum antibody and higher serum (p>0.01) and liver (p>0.05) RV antigen compared to healthy pups. Passive transfer of low-titer (1600; p>0.05) or high-titer (25,600; p>0.01) RV immune serum to neonatal pups prior to RRV infection also protected them from developing cholestasis. Together, these findings indicate that passively acquired, neutralizing or non-neutralizing RV serum antibody attenuates viral replication and protects pups against disease in the RRV BA model. Early reduction of viral load by clearance with RV-specific antibody is likely a critical determinant of disease in this model.

2025

This manuscript can be downloaded from the webpage: http://tipdergisi.erciyes.edu.tr/download/2007;29(2):168-171.pdf Cholestatic hepatitis like syndrome and congenital hypothyroidism Konjenital hipotiroidizm ve kolestatik hepatit benzeri sendrom Özet Konjenital hipotiroidinin klinik bulgularý yenidoðan döneminde non-spesifik olup tipik bulgularýnýn geliþmesi oldukça yavaþtýr. Erken tanýsý prognoz açýsýndan çok önemlidir. Kolestaz ise yenidoðan döneminde tanýsý zor ve zaman alýcý bir hastalýktýr. Safra kesesinde çamurlaþma prenatal hayatta görülebilen bir bulgu olup kendiliðinden düzelebilir. Doðumdan sonra safra çamurunun en sýk nedeni parenteral beslenmedir. Geliþinde kolestatik hepatit tablosu tespit edilip, konjenital hipotiroidi tanýsý alan ve bütün bulgularý levotiron tedavisiyle düzelen bir yenidoðan vakasý takdim ettik. Konjenital hipotiroidinin vakamýzda kolestatik hepatit, karýnda þiþme ve safra çamurlaþmasý ile baþvurmasý ilginç bulunarak sunuldu. Anahtar kelimeler: Doðums...

2025, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine

The purpose of this study was to investigate the potential utility of shear wave elastography (SWE) for diagnosis of biliary atresia and for differentiating biliary atresia from infantile hepatitis syndrome by measuring liver stiffness. Thirty-eight patients with biliary atresia and 17 patients with infantile hepatitis syndrome were included, along with 31 healthy control infants. The 3 groups underwent SWE. The hepatic tissue of each patient with biliary atresia had been surgically biopsied. Statistical analyses for mean values of the 3 groups were performed. Optimum cutoff values using SWE for differentiation between the biliary atresia and control groups were calculated by a receiver operating characteristic (ROC) analysis. The mean SWE values ± SD for the 3 groups were as follows: biliary atresia group, 20.46 ± 10.19 kPa; infantile hepatitis syndrome group, 6.29 ± 0.99 kPa; and control group, 6.41 ± 1.08 kPa. The mean SWE value for the biliary atresia group was higher than the v...

2025, Journal of Pediatric Surgery

Background: Hepatic fibrosis and cirrhosis develop progressively in extrahepatic biliary atresia (EHBA) despite timely surgical intervention. Purpose: The aim of the study was to define CD4 + helper T lymphocytes, cytotoxic CD8 + T lymphocytes, and CD68 + (macrophages) infiltration of portal tracts and lobules and hepatic fibrosis as possible predictive measures of outcome of infants having EHBA. Methods: The outcome of 32 infants with EHBA was correlated to their percutaneous biopsy and postportoenterostomy core liver tissue infiltration by CD4 + , CD68 + , and CD8 + cells and to the degree of detected fibrosis. Results: Portoenterostomy cores were heavily infiltrated by CD4 + , CD8 + , and CD68 + , compared with the preoperative liver biopsy ( P = .008, .004, and .017, respectively). Infants having favorable outcome had more macrophage infiltration in portoenterostomy core compared with those having an unfavorable outcome (25.66 F 29.77 per HPF compared with 11.62 F 4.58, P = .000). Mean CD4 + /CD8 + ratio was 1.54 F 1.37 in those who died within 18 months postoperatively and 0.733 F 0.48 in others ( P = .021). Conclusion: Immune-mediated destruction of portal tracts is an integral part of pathogenesis of EHBA.

2025, Journal of Pediatric Gastroenterology & Nutrition

Objectives: Biliary atresia (BA) is a rare and progressive idiopathic disease affecting the biliary tract that can lead to end-stage liver disease. The main treatment is Kasai portoenterostomy (KP). The use of adjuvant therapy (AT; prophylactic antibiotics and steroids) after KP aims to prevent cholangitis and reduce the need for liver transplantation (LT), but there is a lack of evidence on their effectiveness. We investigated the impact of significant changes in the post-KP protocol on the overall outcomes of BA. Methods: We enrolled 43 consecutive infants undergoing KP at Bambino Gesù Children's Hospital between July 2012 and October 2018. We compared AT (AT group; n=25) against no treatment (AT-free group; n = 18). Results: No significant differences in anthropometric and laboratory parameters were shown between the 2 groups at baseline and every study evaluation (1, 3, and 6 months). The incidences of clinical complications of liver disease were similar. Six months post-KP,...

2025, Gastroenterology

Biliary atresia (BA) is a rare disease of infants with unknown mechanisms of pathogenesis. It is characterized by hepatobiliary inflammatory, progressive destruction of the biliary system leading to liver fibrosis and deterioration of liver function. Interleukin 17A (IL17A) promotes inflammatory and autoimmune processes. We studied the role of IL17A and cells that produce this cytokine in a mouse model of BA and in hepatic biopsy samples from infants with BA. We obtained peripheral blood and liver tissue specimens from 20 patients with BA, collected at time of Kasai porto-enterostomy, along with liver biopsies from infants without BA (controls). The tissue samples were analyzed by reverse transcription (RT)-quantitative PCR, in situ PCR, and flow-cytometry analyses. BA was induced in balb/cAnNCrl mice by rhesus rotavirus infection (RRV); uninfected mice were used as controls. Liver tissues were collected from mice and analyzed histologically and by RT-PCR; leukocytes were isolated, ...

2025, Japanese Journal of Gastroenterology and hepatology

1.1. Background: Fecal calprotectin (FC) is increased in gastrointestinal inflammation due to increased permeability of the gastrointestinal mucosa. It could be used to differentiate between different gastrointestinal disorders. 1.2. Purpose: The aim of the present study was to examine the relationship between fecal calprotectin and different pediatric gastrointestinal diseases and to investigate if it could be helpful in differentiation between different GI diseases. 1.3. Methods: 180 patients in addition to 30 normal children as control, divided into groups according to the final diagnosis, 30 patients with Inflammatory bowel disease (IBD), 20 patients with eosinophilic colitis, 30 patients with helicobacter pylori infection, 40 patients with Functional constipation, 30 patients cow milk allergy, 30 patients with Celiac disease. All children were subjected to investigations that confirm their diagnosis in addition to measure fecal calprotectin according to the manufacturer's instructions. 1.4. Results: Fecal calprotectin in patients with inflammatory bowel disease had mean FC 4640 ± 850 µg/g, In Helicobacter pylori infection was 78.9 ± 25.1 µg/g. Celiac disease patients had mean FC 456 ± 123 µg/g. Eosinophilic esophagitis had mean FC4.2 ± 2.9 µg/g. Functional constipation patients had mean FC 23.6 ± 21.8 µg/g. while normal control children had mean fecal calprotectin 4.1± 6.9 µg/g. Conclusion: Fecal Calprotectin could be helpful in differentiating different pediatric gastrointestinal diseases.

2025, Journal of neonatal surgery

The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai's portoenterostomy at 52nd day of life, the child succumbed to post-operative sepsis.

2025, Journal of Neonatal Surgery

The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai’s portoenterostomy at 52nd day of life, the child succumbed to post-operative sepsis.

2025, PubMed

Background: Iran's experience with liver transplantation (LT) began more than two decades ago. The purpose of this article is to present the status of LT in Iran, review specific characteristics of the programs, their outcomes, and their growth to become one of the largest LT programs in the world. Methods: A questionnaire, asking for data on the number of transplants performed and specifics of the recipients and type of donors with focus on indications and outcomes was sent to LT programs. Results: During a period of 23 years, 4,485 LTs were performed at 6 centers in the country. Of these, 4106 were from deceased donors and 379 were from living donors. There were 3553 adults and 932 pediatric recipients. Hepatitis B and biliary atresia were the most common etiologies in adult and pediatric patients, respectively. Overall survival rates at 1, 5, and 10 years were 85%, 77%, and 71% for adults and 76%, 67% and 56% for pediatric patients, respectively. Conclusion: Approval of the brain death law in Iran and coordinated efforts by the transplant centers to build comprehensive LT programs has resulted in the ability to procure more than 700 deceased donors per year with acceptable long-term survival.

2025, Journal of Pediatric Surgery

Objective: The Kasai portoenterostomy has been accepted as the primary therapeutic treatment for biliary atresia. However, successful bile drainage does not always promise a long-term native liver survival. Several clinical factors were evaluated to discover associations with the outcomes. Methods: A retrospective chart review was conducted of infants with biliary atresia who underwent the conventional Kasai portoenterostomy at Siriraj Hospital, January 2006-August 2018. The patient demographics, perioperative clinical and laboratory data, adjuvant therapies, complications, and interventions were analyzed in correlation to the short-and long-term outcomes. The short-term outcome evaluated was the resolution of jaundice, while the long-term outcome was remaining jaundice-free with the native liver. Results: The complete medical records of 80 patients were retrospectively reviewed. Their mean age at the time of the Kasai portoenterostomies was 97 days. Overall, 66.3% achieved jaundice clearance. The mean follow-up duration was 50.5 months, with 51.3% of the patients remaining jaundice-free with their native liver. A prolonged duration of a prophylactic antibiotic was significantly related to the jaundice clearance, with a p-value of 0.002. Moreover, a lower number of episodes of cholangitis was significantly related to a good long-term outcome (p-value, 0.024). The prolonged provision of a prophylactic antibiotic as adjuvant therapy after the Kasai portoenterostomy was associated with jaundice clearance. An increased incidence of cholangitis episodes was associated with poor long-term outcomes. Postoperative adjuvant therapy should be standardized and maintained for the care of biliary atresia patients to improve their outcomes.

2025, Cureus

Objectives: To determine the frequency of clinical presentation and laboratory profile in the diagnosis of the etiological spectrum of neonatal cholestasis. Material and methods: In this prospective cross-sectional study, we recruited children who presented with jaundice and direct hyperbilirubinemia with onset in the first three months of life. The study was conducted between April 2019 to March 2021 (24 months) at the Government Lady Reading Hospital of Khyber Pakhtunkhwa province in Pakistan. The diagnosis was based on history and clinical findings that included jaundice, stool color, itching, abdominal distention, and deranged liver function tests and confirmed on liver biopsy and specific diagnostic tests. Data was recorded and analyzed using SPSS version 20 (IBM Corp., Armonk, NY). Results: A total of 90 children were included in the study, out of which 65.6% were male. The average age was recorded as 118.01 days + 118.1 SD. Jaundice, dark urine, and hepatomegaly were found in 85.6% of children while ophthalmologic disorder, congenital heart disease, and itching were the least common symptoms. Laboratory findings of the cholestasis patients showed high bilirubin (mean: 8.88 mg/dL), alanine transaminase (ALT) (mean: 177.48 IU/mL), aspartate transaminase (AST) (mean: 187.11 IU/mL), gammaglutamyl transpeptidase (GGT) (mean: 187.66 IU/mL) and prolonged international normalized ratio (INR) (mean: 2.20) in majority of patients. The genetic and metabolic disorder was the leading cause found in the majority of children, which was 43.8%. The common causes of neonatal cholestasis in this study are galactosemia, idiopathic hepatitis, and biliary atresia. The common presentation includes jaundice, hepatomegaly, direct hyperbilirubinemia, raised liver enzymes, and INR.

2025, Cureus

2025, Children (Basel)

Background: Despite the fact that Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA), liver transplantation (LT) remains the ultimate surgery for two-thirds of these patients. Their true survival rate with the native liver reflects the original KPE and the burden of postoperative complications. We report an original modification of the adhesion-sparing liver eversion (ASLE) technique during KPE that facilitates the total native hepatectomy at time of transplantation. Methods: All consecutive patients with BA who underwent KPE at our department and subsequent LT at Paediatric Liver Transplant Centre at Papa Giovanni XXIII Hospital between 2010-2018 were retrospectively enrolled. All patients underwent ASLE during KPE. Patients' demographic data, type of KPE, total transplant time (TTT), hepatectomy time (HT), intra-operative packed red blood cells and plasma transfusions, intra-and post-operative complications were noted. Results: 44 patients were enrolled. Median TTT and HT were 337 and 57 min, respectively. The median volume of packed red blood cell transfusion was 95 mL. No patients presented bowel perforation during the procedure or in the short post-operative course. No mortality after LT was recorded. Conclusions: In addition to the well-known advantages of the standard liver eversion technique, ASLE reduces the formation of intra-abdominal adhesions, lowering significantly the risk of bowel perforation and bleeding when liver transplantation is performed for failure of KPE.

2025, Journal of Pediatric Surgery

The aim of this study was to investigate whether polyunsaturated phosphatidylcholine. (PPC) pretreatment has any protective effect on gastric mucosal damage induced by cold-restraint stress (CRS) in rats. Methods: Forty swiss albino rats were divided into 3 groups. Group 1 (n ϭ 10) was control, group 2 (n ϭ 15) was stress ulcer, and group 3 (n ϭ 15) was PPC-treated rats with stress ulcer. Stress ulcer was induced by the cold-restraint method for 4 hours at 4°C after a starvation period of 72 hours. In the group 3 rats, PPC treatment was started 10 days before stress at a dose of 100 mg/d by oral route. Rats were terminated, stomachs were excised. Macroscopic ulcer index (UI), gastric tissue malondialdehyde (MDA) and superoxide dismutase (SOD) activities, plasma total nitrite, and erythrocyte catalase (CAT) concentrations were assayed. Results: Histopathologic examination showed a stress ulcer index of 0.12 Ϯ 0.19 mm in the treatment group and 23.6 Ϯ 8.97 mm in the stress ulcer group (P Ͻ .001). Tissue MDA and SOD concentrations were higher in the stress ulcer group than in the treatment group, the differences were statistically significant (P Ͻ .001). Plasma NO 3 Ϫ ϩ NO 2 Ϫ levels were higher (P Ͻ .005) and CAT levels were lower (P Ͻ .001) in the nontreatment group. There were no significant differences with respect to Ul, MDA, and SOD levels among the control and treatment groups (P Ͼ .05). Conclusions: These results suggest that pretreating rats with PPC inhibits cold-restraint stress-induced gastric mucosal injury and might be useful in preventing stress-induced stomach ulcers.

2025, Journal of Hepatology

2025, Archives of Disease in Childhood

consanguineous marriages. Intractable diarrhea was the presenting symptom in 40patients (93%), Biochemical data revealed: Serum potassium (1.3-4.1, mean 2.4Mmol/l), s. chloride (39-95, mean76.2Mmol/l), s.bicarbonate (22-54) meam-37.6 Mmol/). Fecal chloride (134±21.6, mean±SD)(range 90-205). The fecal chloride over fecal sodium plus potassium ratio was 0.6 (1.1±0.3, mean ± SD) (N.=0.2). Associated disorders were: chronic renal failure 7 (16%), congenital anomalies 8 (19%), mental retardation4 (9.3%) seizures 8 (19%), and brain atrophy 4 (9%). Complications were seen mostly among patients with late referral or poor compliance. At diagnosis, 35 (81.4%) cases were below -2SD for weight for-age, 31 (72%) for weight-for-height, and 31 (72%) for height-for-age. Children under five years of age showed improvement in weight for height as compared with older children. Conclusions CCD is a treatable cause of intractable diarrhea in infancy. Awareness, early diagnosis and proper management are essential in preventing irreversible and long-term organ damage and a better outcome compared to those with late referrals. CCD is to be considered in infants with severe persistent diarrhea where a high rate of consanguineous marriage prevails.

2025, Anesthesia & Analgesia

2025, BMC Gastroenterology

Background Investigators from different parts of the world are calling for a re-evaluation of the role of liver biopsy (LB) in the evaluation of infantile cholestasis (IC), especially in the light of emerging non-invasive diagnostic technologies. Therefore, this retrospective single-center study was conducted to determine the impact of LB on the diagnosis and management of IC in a cohort from Arabs. Methods From 2007 until 2019, 533 cases of IC were referred for evaluation. All infants who underwent LB were included in the study. We categorized the yield of LB into: (1) defined specific diagnosis; (2) excluded an important diagnosis. A single pathologist reviewed and made the histology report. Results 122 LB specimens met the inclusion criteria. The main indication for LB was a high suspicion of biliary atresia (BA) [high gamma-glutamyl transferase (GGT) cholestasis and pale stool] in 46 cases (37.8%). Liver biopsy had sensitivity of 86.4%, specificity (66.7%), PPV (70.4%), NPV (84....