Prepregnancy Assessment of Liver Function to Predict Perinatal and Postpregnancy Outcomes in Biliary Atresia Patients with Native Liver

Considering that some biliary atresia (BA) survivors with native liver have reached reproductive age and face long-lasting complications, specific attention needs to be paid to pregnant cases. This study aimed to investigate the relationship between liver function, perinatal outcomes, and prognosis. A database review was conducted to identify pregnant BA cases with native liver and perinatal data, and clinical information on BA-related complications was analyzed. Perinatal serum cholinesterase (ChE) levels, model for end-stage liver-disease (MELD) score, and platelet trends were analyzed, and the association between these indicators and perinatal outcomes was investigated. Patients were categorized into three groups according to the perinatal clinical outcomes: favorable (term babies with or without several episodes of cholangitis; n = 3), borderline (term baby and following liver dysfunction; n = 1), and unfavorable (premature delivery with subsequent liver failure; n = 1). Lower serum ChE levels, lower platelet counts, and higher MELD scores were observed in the unfavorable category. Borderline and unfavorable patients displayed a continuous increase in MELD score, with one eventually needing a liver transplantation. Pregnancy in patients with BA requires special attention. Serum ChE levels, platelet counts, and MELD scores are all important markers for predicting perinatal prognosis.

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To Study the Correlation of Serum Cholinesterase Level With Different Scoring Systems in Cirrhosis of the Liver

International Journal of Innovative Research in Medical Science ◽

10.23958/ijirms/vol06-i02/1073 ◽

2021 ◽

Vol 6 (02) ◽

pp. 162-166

Author(s):

Jagadeesh B S ◽

Ravi K ◽

Avinash H R ◽

Nitish Ashok Gurav

Keyword(s):

Liver Disease ◽

Liver Function ◽

Prothrombin Time ◽

Liver Dysfunction ◽

Scoring Systems ◽

Meld Score ◽

Cirrhosis Of The Liver ◽

Decompensated Cirrhosis ◽

Serum Cholinesterase ◽

Cirrhosis Of Liver

Background: Chronic liver disease in the clinical context is a disease process of the liver that involves a process of progressive destruction and regeneration of the liver parenchyma leading to fibrosis and cirrhosis1. The serum cholinesterase is mainly synthesized in the liver and it is released into the blood, which is reduced in liver dysfunction due to reduced synthesis, marked reduction of cholinesterase in liver dysfunction and restoration of synthesis with hepatocyte recovery suggest serum cholinesterase activity might be a more specific marker of liver dysfunction than traditional liver function tests. Objectives: To estimate the level of serum cholinesterase in patients with cirrhosis of the liver. To correlate the level of serum cholinesterase with different scoring systems of cirrhosis of the liver and assess the utility of serum cholinesterase levels in prognostification. Materials and Methods: A cross-sectional, hospital-based, time-bound study conducted on 200 patients with cirrhosis of liver attending medicine OPD and getting admitted in hospitals attached to BMCRI from November 1st2016 to August 30th2018. All cirrhosis of liver patients were included and patients with Pregnancy, Acute infection, Chronic infection like tuberculosis and Oral contraceptive use were excluded. Serum cholinesterase, ultrasound abdomen, prothrombin Time, International Normalized Ratio, liver function test, Child-Pugh score, MELD score were measured. Results: In the study, the majority of the study subjects belonged to the age group 41 – 50 years (38.5%), followed by 31-40 years (21.5%), 51 – 60 years (18.5%). Sex distribution male 70% and female 30%. Serum Cholinesterase was positively correlated with Albumin and Prothrombin time and negatively correlated with MELD, Creatinine and Child-Pugh Score. The mean S. cholinesterase values found in study subjects belonged to Child-Pugh Score A, B and C were 4235.17 + 341.260, 3226.26 + 707.206 and 1764.09 + 808.797. The ANOVA results showed that there was a significant association found between child-pugh scores and S. cholinesterase (p – 0.001). Conclusion: The Study has demonstrated that the level of cholinesterase is correlated with the severity of the liver disease. Serum cholinesterase shows a good correlation with serum albumin, PT INR, Child-pugh score, MELD score. Compared to the above parameters serum cholinesterase is less complex and not easily affected by treatments for decompensated cirrhosis.

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Assessment of native liver fibrosis using ultrasound elastography and serological fibrosis indices in children with biliary atresia after the Kasai procedure

Acta Radiologica ◽

10.1177/0284185120948489 ◽

2020 ◽

pp. 028418512094848

Author(s):

Jisun Hwang ◽

Hee Mang Yoon ◽

Kyung Mo Kim ◽

Seak Hee Oh ◽

Jung-Man Namgoong ◽

...

Keyword(s):

Liver Cirrhosis ◽

Liver Fibrosis ◽

Liver Function ◽

Diagnostic Accuracy ◽

Biliary Atresia ◽

Non Invasive ◽

Kasai Procedure ◽

Serologic Markers ◽

Native Liver ◽

Us Elastography

Background Validated non-invasive examinations are necessary to monitor liver fibrosis in children with biliary atresia (BA) after the Kasai procedure. Purpose To evaluate the diagnostic accuracy of two-dimensional shear wave elastography (2D-SWE), transient elastography (TE), and the serologic biomarkers of aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for evaluating native liver fibrosis in children with BA. Material and Methods We retrospectively reviewed same-day 2D-SWE and TE liver stiffness (LS) measurements of 63 patients with BA who underwent the Kasai procedure. The APRI and FIB-4 score were computed. Hepatic fibrosis was categorized into three clinical categories based on the ultrasound (US) hepatic morphology and clinical manifestations of liver cirrhosis: I, pre-cirrhotic liver state (n = 15); II, US and/or clinical signs of liver cirrhosis with compensated liver function (n = 27); and III, liver cirrhosis with decompensated liver function (n = 21). We compared area under the receiver operating characteristic curve (AUC) data among 2D-SWE, TE, APRI, and FIB-4 score. Combined evaluation of serologic fibrosis indices and US elastography was conducted and AUCs of combinations were analyzed. Results 2D-SWE, TE, APRI, and FIB-4 score showed good to excellent diagnostic accuracy for differentiating clinical categories (AUCs 0.779–0.955). AUC values were significantly increased after adding TE to FIB-4 score for detecting liver cirrhosis ( P = 0.02). Conclusion 2D-SWE, TE, APRI, and FIB-4 score are accurate non-invasive markers for monitoring native liver fibrosis in patients with BA. Combined use of serologic markers and US elastography could yield more accurate diagnoses of liver fibrosis than serologic markers alone.

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Liver transplantation for deterioration in native liver function after portoenterostomy for biliary atresia in Japan: Short- versus long-term survivors

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2017.11.016 ◽

2018 ◽

Vol 53 (2) ◽

pp. 277-280

Author(s):

Takanori Ochi ◽

Hiroki Nakamura ◽

Momoko Wada ◽

Tsuyoshi Tamura ◽

Hiroyuki Koga ◽

...

Keyword(s):

Liver Transplantation ◽

Liver Function ◽

Biliary Atresia ◽

Native Liver ◽

Long Term Survivors

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LIVER FUNCTION TESTS IN INFANTS WITH BILIARY ATRESIA

PEDIATRICS ◽

10.1542/peds.26.4.630 ◽

1960 ◽

Vol 26 (4) ◽

pp. 630-640

Author(s):

Jesus Kumate ◽

Francisco Beltrán ◽

Lázaro Benavides ◽

Marí Antonieta Flores

Keyword(s):

Liver Function ◽

Biliary Atresia ◽

Half Life ◽

Older Patients ◽

Serum Bilirubin ◽

Exploratory Laparotomy ◽

Liver Function Tests ◽

Nutritional Condition ◽

Serum Cholinesterase ◽

Total Ratio

Simultaneous changes of serum bilirubin (direct/total ratio), flocculation tests (thymol and cephalin-cholesterol), transaminases (GOT and GPT), serum cholinesterase and serial clearance of BSP were studied in 30 cases of biliary atresia confirmed by exploratory laparotomy or necropsy. Serum bilirubin generally showed a tendency to increase as age advanced (more evident after the fourth month) although its correlation was poor (r = 0.31). In over 80% of the cases, the direct/total ratio was 0.50 or greater. Flocculation tests showed abnormal values in over 35% of the cases and were more marked as age and degree of malnutrition increased. Transaminase activities in the serum showed slight increases (about 100 units/ml), predominantly of GOT and without relation to age, nutritional condition or flocculation tests. Subnormal activity of cholinesterase was observed in three-fourths of the cases; it was more evident in older patients and no relation was found to percentage of the patient's theoretic weight. Serial study of BSP clearance showed increase of retention at 45 minutes (about 30%) and exponential elimination (with a half-life of 2.4 days) and levels above 6% by the fifth day following administration of the dye.

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Successful Outcome and Biliary Drainage in an Infant with Concurrent Alpha-1-Antitrypsin Deficiency and Biliary Atresia

Case Reports in Surgery ◽

10.1155/2017/9348461 ◽

2017 ◽

Vol 2017 ◽

pp. 1-5

Author(s):

Andrew W. Wang ◽

Kimberly Newton ◽

Karen Kling

Keyword(s):

Liver Function ◽

Biliary Atresia ◽

Successful Outcome ◽

Dual Pathology ◽

Native Liver ◽

Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin ◽

Prompt Diagnosis ◽

Rare Instance

We describe the rare instance of concomitant biliary atresia and alpha-1-antitrypsin deficiency and the first documented successful portoenterostomy in this scenario. The potential for dual pathology must be recognized and underscores that prompt diagnosis of biliary atresia, despite concomitant alpha-1-antitrypsin deficiency, is essential to afford potential longstanding native liver function.

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EXISTENCE OF BACTERIA IN NATIVE LIVER OF BILIARY ATRESIA PATIENTS IS ASSOCIATED WITH SUBSEQUENT BACTERIAL INFECTIONS POST TRANSPLANTATION

10.26226/morressier.58fa1768d462b80290b50baa ◽

2017 ◽

Author(s):

Kazuhiro Uda

Keyword(s):

Biliary Atresia ◽

Bacterial Infections ◽

Post Transplantation ◽

Native Liver

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Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

European Journal of Pediatric Surgery ◽

10.1055/s-0041-1723994 ◽

2021 ◽

Author(s):

Omid Madadi-Sanjani ◽

David Fortmann ◽

Udo Rolle ◽

Burkhard Rodeck ◽

Ekkehard Sturm ◽

...

Keyword(s):

Biliary Atresia ◽

Data Transfer ◽

Previous Analysis ◽

Outcome Data ◽

Free Survival ◽

Change In Practice ◽

Native Liver ◽

Survival With Native Liver ◽

Pediatric Liver Transplant

Abstract Introduction The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. Materials and Methods In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. Result Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001–2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001–2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001–2005 [p = 0.5]). Compared with the 2001–2005 analysis, all criteria showed improvement but the differences are statistically not significant. Conclusion Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

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Viability Assessment in Liver Transplantation—What Is the Impact of Dynamic Organ Preservation?

Biomedicines ◽

10.3390/biomedicines9020161 ◽

2021 ◽

Vol 9 (2) ◽

pp. 161

Author(s):

Rebecca Panconesi ◽

Mauricio Flores Carvalho ◽

Matteo Mueller ◽

David Meierhofer ◽

Philipp Dutkowski ◽

...

Keyword(s):

Liver Function ◽

Organ Preservation ◽

Ex Situ ◽

Current Status ◽

Intensive Care Treatment ◽

Viability Assessment ◽

Great Opportunity ◽

Continuous Increase ◽

Diagnostic Time ◽

The Impact

Based on the continuous increase of donor risk, with a majority of organs classified as marginal, quality assessment and prediction of liver function is of utmost importance. This is also caused by the notoriously lack of effective replacement of a failing liver by a device or intensive care treatment. While various parameters of liver function and injury are well-known from clinical practice, the majority of specific tests require prolonged diagnostic time and are more difficult to assess ex situ. In addition, viability assessment of procured organs needs time, because the development of the full picture of cellular injury and the initiation of repair processes depends on metabolic active tissue and reoxygenation with full blood over several hours or days. Measuring injury during cold storage preservation is therefore unlikely to predict the viability after transplantation. In contrast, dynamic organ preservation strategies offer a great opportunity to assess organs before implantation through analysis of recirculating perfusates, bile and perfused liver tissue. Accordingly, several parameters targeting hepatocyte or cholangiocyte function or metabolism have been recently suggested as potential viability tests before organ transplantation. We summarize here a current status of respective machine perfusion tests, and report their clinical relevance.

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