Early Detection Of Biliary Atresia Research Articles

Predictive modeling for early detection of biliary atresia in infants with cholestasis: Insights from a machine learning study

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.

Primary Healthcare Providers' Knowledge on the Early Detection of Biliary Atresia

Primary Healthcare Providers' Knowledge on the Early Detection of Biliary Atresia

Stool color card use for early detection of biliary atresia

Stool color card use for early detection of biliary atresia

Early detection of biliary atresia in primary health care: still a problem

Background: Biliary atresia is the leading cause of liver transplantation in children. Early detection of biliary atresia is crucial for diagnosis and disease progression. The purpose of this study was to analyze knowledge about biliary atresia and the effectiveness of health education in increasing the knowledge of primary health care providers. Methods: A quasi-experimental study with pretest and posttest designs was carried out in Sidoarjo, East Java using a self-administered questionnaire. The intervention using health education was delivered by pediatrician and consultant of pediatric gastro hepatology. There were 13 questions on the questionnaire, question numbers 1 to 6 were about normal and abnormal neonatal jaundice, question numbers 7 to 13 were about biliary atresia. Results: A total of 252 participants were involved, the mean age of the participants was 40.7 ± 9.4 years old. Most of the participants were midwives (61.9%) and 77.8% of participants have years of service in primary health care > 5 years. A total of 40.5% participants stated that newborns may have physiological jaundice, which was characterized by icteric sclera, pale stools, and dark urine. A total of 27,4% and 24,2% participants said that all jaundice in newborn will always improve on their own and newborn with prolonged jaundice does not need further examination, respectively. There was an increase in the median value in the pretest and posttest knowledge scores after interventional health education (p < 0,05). Conclusions: The primary health care provider understands about biliary atresia, however, the initial knowledge about early detection of biliary atresia is not evenly distributed in all primary health care providers. These findings suggest that improving knowledge to early detection of biliary atresia is needed. Health education can be used effectively in increasing knowledge about biliary atresia.

Gamma-glutamyl transpeptidase has different efficacy on biliary atresia diagnosis in different hospital patient groups: an application of machine learning approach.

Gamma-glutamyl transpeptidase (GGT) is the most widely used biomarker in the early diagnosis of biliary atresia (BA), but its diagnostic efficacy is questionable in different sub-populations. We aim to identify subgroups that are defined by specific variables with cut-offs and can significantly affect the diagnostic efficacy of GGT for detecting BA. Clinical data from 1273 infants with neonatal obstructive jaundice (NOJ) diagnosed between January 2012 and December 2017 at the Children's Hospital of Fudan University were enrolled, reviewed, and analyzed. Random forest-based Virtual Twins method was used to identify potential subgroups. Hemoglobin (HGB) and fasting gallbladder filling were selected as defining variables. The diagnostic efficacy of GGT was significantly better (AUC = 0.855) for patients with hemoglobin (HGB) ≤ 105g/L and a gallbladder that was not or poorly filled. Diagnostic efficacy was worst in the subgroup defined by HGB > 105g/L (AUC = 0.722). The inclusion of interaction terms between GGT and the subgroups in a logistic regression model significantly improved (p = 0.002) prediction performance. This study provides evidence that the diagnostic efficacy of GGT can differ significantly across different subgroups. Therefore, a GGT diagnostic result should be interpreted cautiously when patients belong to subgroups with low diagnostic efficacy. The development of a prediction model and/or clinical diagnostic pathway for early detection of BA should also account for the heterogeneous diagnostic efficacy of GGT.

Diagnostic Tools for Early Detection of Biliary Atresia: Is a Newborn Screen Attainable?

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Development of the Stool Color Card for Early Detection of Biliary Atresia

Development of the Stool Color Card for Early Detection of Biliary Atresia

DIFFERENTIATING BILIARY ATRESIA FROM OTHER CAUSES OF CHOLESTASIS JAUNDICE IN INFANTS

Aim: The aim of this study is to find out the cholestatic etiologies in infants and differences of clinical features, laboratory investigations between biliary atresia and other causes of cholestasis at Vietnam Children Hospital. Background: Cholestasis is defined as reduced bile formation or biliary flow. It results of varied causes. Early detection of biliary atresia is to intervene in time and have the best outcome. Patient and methods: In this retrospective study, 305 infants under 12 months of age with cholestasis were studied in Vietnam Children Hospital during 1/2017-7/2018. Demographic data, duration of jaundice, signs and symptoms as well as laboratory, imaging, liver biopsy and the causes of cholestasis were recorded, divided into 2 group BA and Non-BA. Results: 305 infants (194 boys, 111 girls) with cholestasis and mean age of 83,22±72,10 days were included in the study. The most common causes of cholestasis were idiopathic neonatal hepatitis (33,8%), biliary atresia (25,9%), cytomegalovirus infection (21,6%). In BA group, pale stool (100%), Hepatomegaly (98,7%); increasing less AST, ALT, more GGT level than Non-BA. Find out GGT cutoff > 212,05 UI/l in diagnosing BA. Conclusion: Biliary atresia and idiopathic neonatal hepatitis are the most common causes of infantile cholestasis. Pale stool, hepatomegaly and GGT elevation > 212,05 UI/l are the most reliable tests for diagnosing BA.

Development of the Stool Color Card for Early Detection of Biliary Atresia using Multispectral Image

The stool color card for early detection of biliary atresia have been developed using multispectral images of stools of newborns. Color and its spectra were measured and analyzed spectrally first in the world and used to design the stool color card. Representative texture was selected by medical specialists from captured multispectral images and spectral information of the images was replaced and edited according to the result of spectral and chromaticity analysis. The stool color card was placed within the Maternal and Child Health Handbook that was distributed to all pregnant women by their respective local government according to the Maternal and Child Health Law in Japan.

Stool Color Card Screening for Early Detection of Biliary Atresia

Stool Color Card Screening for Early Detection of Biliary Atresia

GC/MS based metabonomie study of neonatal hepatitis syndrome

Objective Early detection is the most effective way to improve the clinical outcome of biliary atresia(BA). Emerging metabolomics provides a powerful platform for discovering novel biomarkers and biochemical pathways to improve early diagnosis.The aim of this study is to find the potential biomarkers to distinguish BA from neonatal hepatitis syndrome(NHS)by using a metabolomics method. Methods We comprehensively analyzed the serum metabolites in a total of 26 blood samples from patients with BA or neonatal hepatitis syndrome(NHS)and from normal individuals using advanced metabolomic approaches. Results The levels of propanoic acid, hexadecanoic acid, eicosanoic acid, octadecenoic acid and cholesterol significantly increased in the BA group. Conclusion The levels of L-Tyrosine(Tyr)were reduced in the BA group compared to those in the NHS group, but still higher than the normal controls.The levels of L-Proline(Pro)in the NHS group were significantly elevated compared to those in the BA group.And at the same time, we find 5 patients with cirin deficiency.This study demonstrates the possibility of metabolomics as non-invasive biomarkers for the early detection of BA and also provides new insight into pathophysiologic mechanisms for BA. Key words: Neonatal hepatitis syndrome; Biliary atresia; GC-MS; Citrin deficiency; Fatty acid

Modified stool color card with digital images was efficient and feasible for early detection of biliary atresia-a pilot study in Beijing, China.

The aim of this pilot study in Beijing, China, was to validate a screening system for early detection of biliary atresia (BA) by using a modified version of the stool color card (SCC). From 2013 to 2014, a total of 29 799 live born infants were screened. SCC was distributed in maternal facilities. Guardians were asked to check their infants' stool colors daily using SCC up until four months after birth. The screening results among 92.5% of participants were reported. Cases deemed as high risk were referred to a surgical department immediately. Based on the results reported by the guardians, 24 infants showed pale-pigmented stools, of which two males without obvious signs of jaundice were diagnosed with BA at 52 and 55 days of age, respectively. The sensitivity was 100% and specificity was 99.9%. Four infants were confirmed as having other diseases. Two female patients failed to be screened by the SCC because they had severe jaundice and were referred to the Neonatal Intensive Care Unit after birth. They were diagnosed as BA at 14 and 17 days after birth, respectively. The overall prevalence of BA in this study was 1.3 in 10 000 live births. The modified SCC was effective and feasible for early detection of BA, especially for patients with no apparent jaundice.

Management of Biliary Atresia: A Review

Biliary atresia (BA) is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. It is the most common cause of neonatal cholestasis and most common indication for liver transplantation in children. Biliary Atresia clinically presents with jaundice, pale stool, dark urine and hepatomegaly. The treatment of biliary atresia is surgical and is recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (Kasai operation) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/ or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated. Age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. So early detection of biliary atresia is necessary for optimum management and overall survival of the patient.Bangladesh J Child Health 2015; VOL 39 (1) :38-45

Recent Trends in the Diagnosis and Management of Biliary Atresia in Developing Countries.

Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease in developing countries. Articles were retrieved from the PubMed database using the terms biliary atresia, Kasai portoenterostomy and pediatric liver transplantation. Contents of the article are also based on personal experience of the authors. A national screening program using stool color cards as part of standard care in the neonatal period will greatly improve early detection of biliary atresia. Outcomes will improve if it is diagnosed at the earliest after birth, the child is referred to an experienced pediatric hepatobiliary unit for evaluation, and undergoes an early Kasai procedure. If an early Kasai portoenterostomy is performed, nearly half of all children survive into adolescence, and about one-third are likely to have a long-term, symptom-free life with normal liver biochemistry. Sequential treatment combining Kasai as first line and liver transplantation as second line results in 90% survival for children with biliary atresia.

Early diagnosis of biliary atresia

Biliary atresia is the most common indication for liver transplantation in the pediatric population and the most frequent liver-related cause of death in early childhood. However, in some cases, long-term survival with the native liver can be achieved by surgical reestablishment of bile flow. The procedure of choice involves removal of the fibrotic, atretic bile duct segment and construction of a drainage route via hepatoportoenterostomy (the Kasai procedure). The outcome following hepatoportoenterostomy is closely related to the patient's age at the time of the procedure. It is universally acknowledged that the Kasai procedure performed before 60 days of age can improve the likelihood of survival with the native liver and avoid liver transplant. The problem that we continually witness in our clinical practice is late referral of patients with biliary atresia, emphasizing the importance of screening for this potentially devastating disease. The lack of bile flow into the intestine leads to acholic stools—a reliable clue that obstruction is likely. Pale, nonpigmented stools appear within the first month after birth in patients with biliary atresia. Thus, the concept of using a stool color card for screening and parent education regarding the disease was initially introduced to the local population in Japan (Tochigi Prefecture) by Matsui et al in 1993 (Screening 1993;2:201-9). Their preliminary results documented the success in ensuring a prompt diagnosis and early Kasai procedure. The concept of stool color card based mass screening has subsequently been utilized in several other countries, also resulting in earlier referral of patients with biliary atresia. In this issue of The Journal, Gu et al now report the cumulative impact of the stool color card program over the 19 years that it has been used for mass screening of biliary atresia in Tochigi Prefecture. Beginning in 1994, the stool color card was distributed to all pregnant women prior to or during the postnatal one-month health check-up and the mothers returned the completed card to the attending physician. All suspected cases of biliary atresia were referred for further examination, where the diagnosis was confirmed and patients were followed from the date of Kasai procedure until liver transplant or death up to 2013. A total of 313 230 live born infants were screened; 34 patients were confirmed to have biliary atresia. The sensitivity and specificity of stool color card screening at the one-month check-up was 76.5% and 99.9%, respectively. Mean age at the time of performance of the Kasai procedure was 59.7 days. This was associated with high rates of long-term native liver survival (88%, 77%, and 49% at 5, 10, and 15 years, respectively). These data are convincing evidence that this simple, patient-empowered screening for biliary atresia should be universally adopted to improve the outcome of affected children. Article page 897▶ Stool Color Card Screening for Early Detection of Biliary Atresia and Long-Term Native Liver Survival: A 19-Year Cohort Study in JapanThe Journal of PediatricsVol. 166Issue 4PreviewTo 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. Full-Text PDF

Stool Color Card Screening for Early Detection of Biliary Atresia and Long-Term Native Liver Survival: A 19-Year Cohort Study in Japan

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 specificity of stool color card screening at the 1-month check-up was 76.5% (95% CI 62.2-90.7) and 99.9% (95% CI 99.9-100.0), respectively. Mean age at the time of Kasai procedure was 59.7 days. According to Kaplan-Meier analysis, the native liver survival probability at 5, 10, and 15 years was 87.6%, 76.9%, and 48.5%, respectively. The sensitivity and specificity of the stool color card have been demonstrated by our 19-year cohort study. We found that the timing of Kasai procedure and long-term native liver survival probabilities were improved, suggesting the beneficial effect of stool color card screening.

Early detection of neonatal cholestasis by stool color card screening.

The objective of this study was to identify, by early screening, one-month old infants with acholic or hypocholic stools for the early detection of biliary atresia and other causes of neonatal cholestasis. The study was essentially exploratory (observational, prospective, statistically underpowered and with no clinical intervention) and used a color card screening of all newborns attending the first month of life checkup from 1999 to 2002 in a public hospital in Argentina. Of 12 484 newborn infants, 4239 were examined at their first month of life checkup visit with the stool color card. Eighteen infants were identified with hypocholic stools, of whom only four were proven to have cholestasis. Although no case of biliary atresia was detected, screening by stool color cards proved useful for the detection of other causes of neonatal cholestasis.

Early detection of biliary atresia: past, present & future

The age threshold at the time of Kasai hepatic portoenterostomy associated with a prognosis of biliary atresia (BA) is becoming clearer as 10-year native liver survival data become more frequent in the recent literature, whereas the age at diagnosis has not dramatically decreased during the last 3 decades. A stool color card screening implemented in 1994 in Japan is now expanding worldwide. However, the contribution of this modality will probably be limited because of the nature of this disease, for example, ‘progressive obliterative cholangiopathy’. A cholic stool was actually observed only in 50% before diagnosis according to the Japanese BA Registry data. Thus, color card screening does not appear to be instrumental in detecting patients with BA early enough before 1 month of age. A highly sensitive, adequately specific, noninvasive and quantitative method may be expensive, but the overall cost would be lower than that of liver transplant.

Early Detection of Biliary Atresia Raises Questions About Etiology and Screening

Biliary atresia (BA) is the most significant life-threatening hepatobiliary disorder in children and is the most common indication for liver transplantation in the pediatric population.1 It occurs in ∼1 in 10 000 infants.2 BA is the result of an obliteration of all or part of the biliary ductal system. If BA is left untreated, the prognosis is poor: it leads to either liver transplantation or death from complications of liver failure.3 The commonly accepted treatment for BA is the restitution of the bile flow via the hepatoportoenterostomy operation (Kasai procedure), which is achieved by the excision of the extrahepatic biliary remnants and anastomosis to a jejunal loop. A successful hepatoportoenterostomy can delay or decrease the need for a liver transplant. A number of research studies have found that the most favorable outcomes of the hepatoportoenterostomy are related to the appropriate timing of the procedure; better results are … Address correspondence to Ezequiel Neimark, MD, Hasbro Children's Hospital, 593 Eddy St, MPH-131, Providence, RI 02903. E-mail: eneimark{at}lifespan.org

Urinary sulfated bile acid analysis for the early detection of biliary atresia in infants

Measurement of urinary sulfated bile acid (USBA) is a non-invasive method to detect bile congestion. Our aim was to evaluate the feasibility of USBA analysis for the early detection of biliary atresia (BA). We determined the USBA-to-creatinine ratio (USBA/cr) in 1148 infants at 10-40 days after birth. All infants were followed until the 3- to 4-month postnatal routine health check. The cutoff value for USBA/cr was 55.0 µmol/g creatinine. Among the infants tested, 47 (4.10%) had USBA/cr ratios that exceeded the cutoff value. Two of these 47 infants had liver disease; one was diagnosed with neonatal hepatitis syndrome, and the other was diagnosed with BA. The BA patient underwent USBA analysis for the first time on day 18 after birth and hepatoportoenterostomy on day 49. No other infants were diagnosed with hepatobiliary disease during the follow-up period. This USBA analysis provided the correct assessment without fail and identified a case of BA. This approach could be used for the screening and early detection of BA when the false-positive rate is decreased by improving the methods for sample collection and urine storage.