scholarly journals Our Experience in Diagnosis and Treatment of Children with Biliary Atresia

2016 ◽  
Vol 23 (3) ◽  
Author(s):  
O. Kulyk ◽  
H. Kurylo ◽  
O. Nykyforuk ◽  
D. Hrytsak
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Surgical Treatment ◽  
Biliary Atresia ◽  
Neurological Status ◽  
Postoperative Treatment ◽  
Biliary Cirrhosis ◽  
Surgical Department ◽  
Kasai Procedure

Biliary atresia is a congenital disease that occurs with lesions of the bile ducts leading to the development of cholestasis and manifests in the neonatal period. Without timely surgical treatment, patients with this pathology die during the first year of liver failure, esophagus bleeding or infections.The objective of the research was to analyze the results of examinations and treatment of 21 children with biliary atresia who were treated at the surgical department of conformational abnormalities in children in Lviv City Children’s Clinical Hospital since 2008 to 2015.Methods of the research included follow-up, laboratory ones, duodenal intubation, ultrasound, scintigraphy, MRI, diagnostic laparoscopy, liver paracentesis, determination of hepatitis B and C markers, DNA of CMV virus.Results of the research. Among the examined children biliary atresia was diagnosed in 18 patients at the age under 2 months. All children were operated timely. 6 patients needed liver transplantation. Diagnosis was made in 3 children under the age of 3 months. The Kasai onoperat was conducted in 2 children. All 3 children needed liver transplantation. Children with satisfactory quality of life after liver transplantation are on permanent immunosuppressive therapy. They have signs of biliary cirrhosis and undergo periodically inpatient treatment of an ascending cholangitis.Maintenance of normal nutritional (food) status, biliary tract patency and prevention of cholangitis and infections are the primary task in the course of postoperative treatment (The Kasai procedure).Conclusions. Early diagnosis of biliary atresia and timely conducted surgical treatment (under 2 months of age) makes it possible to improve the prognosis, neurological status, quality of life and to prevent the necessity of liver transplantation at an early age.

The Quality of Life of Long Term Survivors of Patients with Biliary Atresia

2021 ◽  
Author(s):  
Melanie Le ◽  
Konrad Reinshagen ◽  
Christian Tomuschat
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
Long Term Results ◽  
Cochrane Library ◽  
Healthy Controls ◽  
Term Care ◽  
Associated Malformations

Abstract Purpose: Advances in surgical techniques and perioperative care have improved patients' short-and mid-term postoperative outcomes with Biliary Atresia (BA). However, the long-term results of these patients have not been thoroughly investigated. This systematic review aims to determine the long-term outcomes and the patients' health-related Quality of life (HrQoL) with their native livers or liver transplantation. Methods: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to August 2021. Original studies reporting on BA, Hepatoportoenterostomie, portoenterostomy, Kasai, Liver transplantation, Quality of life, or HrQoL were included. Pooled prevalence has been calculated for cholangitis, secondary liver transplantation, or associated malformations using MetaXL (version 5.3). Subgroup analysis on HrQoL followed surgical treatment after BA was calculated by using RevMan (version 5.4).Results: 12 articles were considered for data synthesis. Nine studies compared biliary atresia patients to an age-matched healthy reference group. 4/9 (n = 338) of these studies indicated lower scores for biliary atresia patients; 5/9 (n = 127) stated similar health status. A Forest plot analysis including all studies with total HrQoL showed a tendency of higher scores towards healthy controls (MD -0.79, 95% CI: -6.00-4.41). Comparing patients after Kasai Hepatoportoenterostomy with healthy controls demonstrated favorable outcomes for the control group (MD -3.22, 95% CI: -7.20-0.75) with no statistical significance (p = 0.11). The pooled estimation of the prevalence of cholangitis, secondary liver transplantation and associated malformations are 0.33 (95% CI: 0.06–0.66), 0.59 (95% CI: 0,42–0.75) and 0.13 (95% CI: 0,01–0.33).Conclusion: Biliary atresia patients have an overall high prevalence of progressive liver-related complications and risk of lower HrQoL compared to their healthy peers. Furthermore, those patients who received liver transplantation appear to have the same Quality of life as those living with their native livers. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research das focuses on the attributes and predictors of the long-term prognosis of patients with biliary atresia are necessary.

scholarly journals Diagnosis and Management of Cirrhosis-Related Osteoporosis

2016 ◽  
Vol 2016 ◽  
pp. 1-12 ◽  
Author(s):  
Lívia Alves Amaral Santos ◽  
Fernando Gomes Romeiro
Keyword(s):  
Quality Of Life ◽  
Risk Factors ◽  
Liver Transplantation ◽  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Immunosuppressant Therapy ◽  
Treatment Of Osteoporosis ◽  
Screening Strategies ◽  
Cirrhotic Patients

Management of cirrhosis complications has greatly improved, increasing survival and quality of life of the patients. Despite that, some of these complications are still overlooked and scarcely treated, particularly those that are not related to the liver. This is the case of osteoporosis, the only cirrhosis complication that is not solved after liver transplantation, because bone loss often increases after immunosuppressant therapy. In this review, the definitions of bone conditions in cirrhotic patients are analyzed, focusing on the more common ones and on those that have the largest impact on this population. Risk factors, physiopathology, diagnosis, screening strategies, and treatment of osteoporosis in cirrhotic patients are discussed, presenting the more striking data on this issue. Therapies used for particular conditions, such as primary biliary cirrhosis and liver transplantation, are also presented.

Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort

2015 ◽  
Vol 26 (04) ◽  
pp. 349-356
Author(s):  
Yuk-Kueng Sze ◽  
Alida van der Steeg ◽  
Egbert Sieders ◽  
Robert Porte ◽  
Henkjan Verkade ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
National Cohort

Assessment of Quality of Life and Related Stressors following Liver Transplantation

1998 ◽  
Vol 8 (2) ◽  
pp. 113-118 ◽  
Author(s):  
Linda Gubby
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Rating Scale ◽  
Social Activity ◽  
Biliary Cirrhosis ◽  
General Effect ◽  
Health Related ◽  
Group A ◽  
Group B

The aim of this descriptive study was to identify and compare transplant-associated stressors among 30 adult liver transplant recipients following orthotopic transplantation for primary biliary cirrhosis. Recipients were divided in three groups according to length of time following transplantation: 12 months (group A), 1 to 5 years (group B), and 5 or more years (group C). With the use of a recipient stressor scale and a rating scale assessing health-related quality of life, patients were asked to indicate a degree of concern for 25 items. In addition, participants were asked to define quality of life before and after liver transplantation and to attribute a degree of positivity or negativity to 33 items in the subscales of physical health, physical activity, social activity, and general effect. Results from the stressor analysis identified health-related items, in particular the “possibility of rejection,” as causing the greatest concern to respondents. Social and family-related items were considered least stressful. Comparative statistical analysis identified group B as significantly more stressed than either group A or group C.

Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort

2015 ◽  
Vol 26 (04) ◽  
pp. e1-e1
Author(s):  
Yuk-Kueng Sze ◽  
Alida van der Steeg ◽  
Egbert Sieders ◽  
Robert Porte ◽  
Henkjan Verkade ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
National Cohort
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