Biliary Atresia/Neonatal Cholestasis

2021 ◽  
Vol 68 (6) ◽  
pp. 1333-1341
Author(s):  
Sara E. Yerina ◽  
Udeme D. Ekong
PEDIATRICS ◽  
1992 ◽  
Vol 89 (2) ◽  
pp. 329-330
Author(s):  
PIETRO VAJRO ◽  
ANGIOLA FONTANELLA ◽  
EMILIA VUTTARIELLO ◽  
ANNA DE VINCENZO ◽  
GIULIANA FORTUNATO

For the practicing pediatrician, the most commonly encountered causes of neonatal cholestasis are idiopathic neonatal hepatitis (INH) and extrahepatic biliary atresia (EHBA). However, despite extensive research, little improvement in our understanding of the etiology of INH and EHBA has occurred.1 It has been proposed that both of them may be part of a continuum of hepatobiliary disease caused by a common insult with a different predominant injury site.2 In this respect, it is interesting that, up to the present, several cases of discordance for EHBA in twins of various zygosity have been reported,3-8 whereas no data are available about either concordance or discordance for INH both in monozygotic and dizygotic twins.


Author(s):  
Judith A. O'Connor ◽  
Ronald J. Sokol

2017 ◽  
Vol 5 ◽  
pp. 2050313X1769599 ◽  
Author(s):  
Noella Maria Delia Pereira ◽  
Ira Shah

Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection.


2008 ◽  
Vol 3 (1) ◽  
pp. 10 ◽  
Author(s):  
Fatemeh E Mahjoub ◽  
Reza Khairkhah ◽  
Mehri Sani ◽  
Guiti Irvanloo ◽  
Maryam Monajemzadeh

2020 ◽  
Vol 60 (2) ◽  
pp. 66-70
Author(s):  
Md. Benzamin ◽  
Mukesh Khadga ◽  
Fahmida Begum ◽  
Md. Rukunuzzaman ◽  
Md. Wahiduzzaman Mazumder ◽  
...  

Background Neonatal cholestasis is an important etiology of chronic liver disease in young children. It has a varied etiology. There is considerable delay in presentation and diagnosis of neonatal cholestasis in Bangladesh. Lack of awareness and knowledge among the pediatricians regarding etiological diagnosis and outcome of neonatal cholestasis is the reasons for poor outcome in major portion of cases in Bangladesh. Objective To evaluate the etiological spectrum of neonatal cholestasis. Methods This retrospective study was conducted at the Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. We reviewed medical records of children who were diagnosed with neonatal cholestasis. Complete diagnostic profiles of every cases with age of presentation, male-female ratio and final diagnosis were analyzed. Results A total of 114 children with neonatal cholestasis were evaluated. Subjects’ male-female ratio was 1.92: 1.0, and mean age at hospitalization was 92.7 (SD 39.5) days. Biliary atresia was the most common etiology (47.4%), followed by idiopathic neonatal hepatitis/INH (21.9%). Other identified etiologies were, toxoplasmosis, others (syphilis, varicella-zoster, parvovirus b19), rubella, cytomegalovirus (CMV), and herpes/TORCH infection (8.61%), progressive familial intrahepatic cholestasis/PFIC (4.4%), galactosemia (4.4%), choledochal cyst (3.5%),  sepsis (1.8%), urinary tract infection/UTI (1.8%), hypothyroidism (1.8%), lipid storage disease/Niemann-Pick disease (0.9%), non-syndromic paucity of interlobular bile ducts (2.67%), and Caroli’s disease (0.9%). Conclusion  In Bangladesh, neonatal cholestasis cases are most often due to obstructive causes, particularly biliary atresia. Idiopathic (INH), infectious (primarily TORCH), metabolic, and endocrine causes followed in terms of frequency.


2020 ◽  
Vol 27 (1) ◽  
pp. 37-41 ◽  
Author(s):  
Mehmet Agin ◽  
◽  
Gokhan Tumgor ◽  
Murat Alkan ◽  
Onder Ozden ◽  
...  

2017 ◽  
Vol 28 (03) ◽  
pp. 261-267 ◽  
Author(s):  
Ahmed Negm ◽  
Claus Petersen ◽  
Andrea Markowski ◽  
Birgit Luettig ◽  
Kristina Ringe ◽  
...  

Introduction Biliary atresia (BA) is a rare destructive inflammatory obliterative cholangiopathy of neonates. Early diagnosis is important in disease management. The aim was to evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing BA in a large cohort. In addition, we evaluated whether parameters such as bile trace, GGT, bilirubin, and laboratory values in combination can be used to develop a risk score that could indicate the referral to specialized centers. Materials and Methods All infants with neonatal cholestasis (2000–2014) who presented to our endoscopy unit for suspected BA were included. Demographics, laboratory parameters, ultrasound findings, liver biopsy results, ERCP diagnosis, and surgical outcome were collected. Value and safety of ERCP and risk factors for BA were retrospectively analyzed. Results We included 251 infants in our cohort (55% males, median age: 53 days). BA was intraoperatively diagnosed in 155 (83.4%) patients and was excluded in 30 (16.2%). Fifty-six cases were not operated due to the ERCP findings. ERCP was successful in 224/251 patients (89.2%) with no procedure-related complications. The operative and endoscopic diagnosis matched in 96.6% of the patients (positive predictive value: 92.2%, negative predictive value: 97.1%). In comparison to cases with excluded BA, the ones with this disease were significantly associated with absence of duodenal bile traces (98.4 vs. 1.6%, p < 0.001), higher bilirubin (p < 0.001, cutoff 7.3 mg/dL), and higher GGT (p < 0.001, cutoff 250 U/L). Conclusion ERCP is safe and accurate in the hands of experts in diagnosing BA if the cause of cholestasis is unclear. While evaluating the role of ERCP for diagnosing this disease, we found that the secondary parameters GGT > 250 U/L, bilirubin > 7.3 mg/dL (125 μmol/L), and the absence of bile traces are risk factors.


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