Biopsy-driven diagnosis in infants with cholestatic jaundice in Iran

Talachian, Elham; Bidari, Ali; Mehrazma, Mitra; Nick-khah, Nahid

doi:10.3748/wjg.v20.i4.1048

Cited by 7 publications

(7 citation statements)

References 22 publications

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“…Biliary atresia accounts for more than half of all cases of prolonged neonatal cholestasis [1] and is managed differently from other causes of neonatal/infantile liver disease, such as neonatal hepatitis (of which there are many etiologies) and Alagille syndrome (congenital paucity of bile ducts). In the setting of biliary atresia, it is extremely important to make a prompt diagnosis because affected children rapidly develop end-stage liver disease (cirrhosis, liver failure and eventual death) in the first year of life if it is not addressed surgically with a Kasai portoenterostomy or liver transplantation [2].…”

Section: Introductionmentioning

confidence: 99%

Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases

et al. 2014

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Section: Introductionmentioning

confidence: 99%

Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases

et al. 2014

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“…Considerable delay in the referral of patients with biliary atresia was documented in present study. Also, a significant delay in presentation of cholestasis and liver biopsy in a research from Iran was found which may have caused irreversible liver damage in conditions such as BA (12). Such a late referral would affect not only the success rate of surgery, but also its outcomes.…”

Section: Discussionmentioning

confidence: 99%

Frequency of Different Causes of Infant Cholestasis in a Tertiary Referral Center in South-East Iran

2018

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Objectives: This study reports frequency of different causes of infant cholestasis diagnosed by clinical and biochemical parameters as well as liver biopsy. Methods: Cholestasis persisting beyond two-weeks of age in 46 infants less than 6 months old was evaluated. A checklist including signs and symptoms, as well as laboratory, imaging and liver biopsy results was prepared. According to diagnosis, patients were classified into two groups: biliary atresia (BA) and non-biliary atresia (Non-BA) and results were compared between two groups. P value < 0.05 was considered significant. Results: Forty six infants (25 girls, 21 boys) with cholestasis and mean age of 65 ± 99 days were included in the study. Most of the infants (42%) were referred at the age of > 91 days. The most common symptoms and signs were jaundice in 100% followed by hepatomegaly (78%), splenomegaly (52%), acholic stool (35%) and dark urine (37%). There were no significant differences between the two groups for age, gender, onset of jaundice and organomegaly. Patients in BA group experienced more acholic stool, abnormal AST, ALT and bilirubin (P < 0.05). The most common underlying causes of liver disease in study group were BA (30.4%) and idiopathic neonatal hepatitis (INH) in 30.4% followed by metabolic disorders. Conclusions: Sixty one percent of infants with cholestasis in present study had BA and INH. A systemic approach is the key for rapid diagnosis to rule out BA and other treatable disorders for a better outcome.

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“…Persistent coloured stool rules out BA, but intermittently acholic stool can be seen in both conditions. 1,11 In Taiwan, a universal screening system using an infant stool colour card was established to promote the early diagnosis for BA, and this enhanced early referral, timely performance of Kasai operation and better outcome. 12 The initial assessment should confirm rapidly that cholestasis is present, provide a baseline assessment of severity of liver dysfunction, and exclude potentially treatable infectious and metabolic disorders.…”

Section: Resultsmentioning

confidence: 99%

“…BA is the leading cause of end-stage liver disease and the most common indication of liver transplantation in the childhood. 11,22,23 In a study from Turkey which evaluated 27 patients with BA, delay of the diagnosis resulted in death about half of the cases in the postoperative period, and 30% of the cases underwent liver transplantation. 24 However the Netherlands Study Group on Biliary Atresia reported performance of surgery before two months of age and clearance of jaundice associated independently with 4 year transplantfree survival.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

Gökçebay¹,

Gülerman²,

Arda³

et al. 2015

Turkiye Klinikleri J Med Sci

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ABS TRACT Objective: Neonatal cholestasis can be the initial evidence of a heterogeneous group of diseases of different etiologies. The aim of the study is to evaluate the patients with neonatal cholestasis and analyse the etiologic factors, diagnostic tools and outcome. Material and Methods: Seventy-five patients (65% males, 45% females) between 0-6 months of age with neonatal cholestasis were retrospectively evaluated; analysed for the clinical, laboratory, radiological, scintigraphic data and histopathological findings from liver biopsies. Results: Eighty per cent of the infants' admitted to our hospital because of prolonged jaundice. The onset of the jaundice was in the first week of life in 64% of the cases, but admission time of the referral center was median two months. Biliary atresia (BA) was determined in 21 (28%), neonatal hepatitis (NH) in 54 (72%) of the patients. Biliary atresia group had significantly more frequent acholic stool, relatively lower aspartate aminotransferase, but higher gamma-glutamyl transpeptidase, and serum protein levels. Liver biopsy and hepatobiliary scintigraphy were the most sensitive methods for differentiation BA from NH (p<0.05). During the follow-up period, 13 of the patients (18%) died, whereas cholestasis improved in 14 of the patients with NH (27%) within median 6 months. Survival rate at one year was 45.5% for the patients with BA, and 87% for the patients with NH. So patients with NH had better prognosis (p<0.05). Conclusion: It has been evident that early diagnosis and intervention of treatable causes of neonatal cholestasis have a vital importance.Key Words: Jaundice, neonatal; cholestasis; biliary atresia ÖZET Amaç: Neonatal kolestaz farklı nedenlerle oluşan heterojen bir grup hastalığın ilk bulgusu olabilir. Bu çalışmanın amacı neonatal kolestazlı hastaların değerlendirilmesi ve etyolojik faktör-ler, tanı yöntemleri ve sonuçlarının incelenmesidir. Gereç ve Yöntemler: Neonatal kolestazı olan, yaşları 0-6 ay arasında değişen 75 hasta (%65'i erkek, %45'i kız) geriye dönük olarak değerlen-dirildi; klinik, laboratuvar, radyolojik, sintigrafik verileri ve karaciğer biyopsilerindeki histopatolojik bulguları incelendi. Bulgular: Bebeklerin %80'i uzamış sarılık nedeniyle hastanemize başvurmuştu. Olguların %64'ünde sarılık yaşamın ilk haftasında başlamasına rağmen ortanca başvuru zamanı iki aydı. Hastaların 21'inde (%28) bilier atrezi (BA), 54'ünde (%72) neonatal hepatit (NH) saptandı. Bilier atrezi grubunda akolik dışkı daha sık, aspartat aminotransferaz görece düşük, ancak gama-glutamil transpeptidaz ve serum protein düzeyleri yüksek saptandı. Karaciğer biyopsisi ve hepatobilier sintigrafi BA'nın NH'den ayrımında en duyarlı yöntem-lerdi (p<0,05). İzlem sırasında 13 hasta (%18) kaybedildi, ancak NH'li 14 hastada (%27) kolestaz ortanca 6 ay içinde düzeldi. Bilier atrezili hastaların bir yıllık yaşam oranı %45,5 iken, NH tanılı hastaların %87 idi. Neonatal hepatitli hastaların prognozları daha iyiydi (p<0,05). Sonuç: Erken tanı ile neonatal kolestazın tedavi edilebilir ned...

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Biopsy-driven diagnosis in infants with cholestatic jaundice in Iran

Abstract: A significant delay was found between IC presentation and liver biopsy, which is detrimental in conditions that can cause irreversible liver damage, such as BA.

Cited by 7 publications

References 22 publications

Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases

Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases

Frequency of Different Causes of Infant Cholestasis in a Tertiary Referral Center in South-East Iran

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

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