Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia

Betalli, Pietro; Cheli, Maurizio; Colusso, M.; Casotti, Valeria; Alberti, Daniele; Ferrari, Alberto; Starita, Giusy; Lucianetti, A.; Pinelli, D.; Colledan, M.; D’Antiga, Lorenzo

doi:10.1016/j.jpedsurg.2021.12.030

Cited by 4 publications

(3 citation statements)

References 29 publications

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“…One element that was not addressed in our survey was the prevention of complications evident at the time of transplantation (i.e., reducing post-operative intra-abdominal adhesion). It has been shown [ 56 , 57 ] that duration of total hepatectomy, bleeding, and prognosis of liver transplantation were adversely impacted when KPE was performed in a centre that does not perform liver transplantation. Operative details which may reduce adhesions include avoidance of unnecessary exposure of the intestines outside the abdomen, avoidance of abdominal drains, and possibly the use of anti-adhesion adjuncts such as hyaluronic acid (Seprafilm™, Baxter Inc., Deerfield, IL, USA) and hydrogels (CoSeal™, Baxter Inc., Deerfeield, IL, USA) around the liver.…”

Section: Discussionmentioning

confidence: 99%

Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper

Davenport

Madadi-Sanjani

Chardot

et al. 2022

JCM

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Biliary atresia, a fibro-obliterative disease of the newborn, is usually initially treated by Kasai portoenterostomy, although there are many variations in technique and different options for post-operative adjuvant medical therapy. A questionnaire on such topics (e.g., open vs. laparoscopic; the need for liver mobilisation; use of post-operative steroids; use of post-operative anti-viral therapy, etc.) was circulated to delegates (n = 43) of an international webinar (Biliary Atresia and Related Diseases—BARD) held in June 2021. Respondents were mostly European, but included some from North America, and represented 18 different countries overall. The results of this survey are presented here, together with a commentary and review from an expert panel convened for the meeting on current trends in practice.

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Section: Discussionmentioning

confidence: 99%

Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper

Davenport

Madadi-Sanjani

Chardot

et al. 2022

JCM

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“…A volume-outcome relationship (based upon morbidity and mortality) is well established for complex colorectal procedures in adulthood 6,7 as well as for the treatment of biliary atresia in pediatric surgery. 8 A minimum caseload has been promoted by the European reference network for the corrective surgery for ARM and other complex congenital anomalies. 9,10 These publications highlight the difficulty of measuring outcomes in rare congenital diseases with associated comorbidities, and the necessity for long-term follow-ups.…”

Section: Introductionmentioning

confidence: 99%

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021

Wilms,

Jenetzky,

Märzheuser

et al. 2024

Eur J Pediatr Surg

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Background: Anorectal malformations (ARM) are complex congenital anomalies. The corrective operation is demanding and schedulable. Based on complete national data, patterns of care have not been analyzed in Germany yet. Methods: All cases with ARM were analyzed 1) at the time of birth and 2) during the hospital stay for the corrective operation, based on national hospital discharge data (DRG statistics). Patient’s comorbidities, treatment characteristics, hospital structures, as well as the outcome of corrective operations were analyzed with respect to the hospitals’ caseload. Results: From 2016-2021 1,726 newborns with ARM were treated at the time of birth in 388 hospitals. Of these hospitals, 19% had neither a pediatric nor a pediatric surgical department. At least one additional congenital anomaly was present in 49% of cases and 7% of the newborns had a birthweight below 1,500 grams. 2,060 corrective operations for ARM were performed in 113 hospitals in the same time period. In 24.5% of cases at least one major complication was documented. One third of the operations were performed in 56 hospitals, one third in 20 hospitals and one third in 10 hospitals with median annual case numbers of 2 vs. 5 vs. 10, respectively. Hospitals with the highest caseload operated cloacal defects more often, than hospitals with the lowest caseload (7% vs. 2%) and had more early complications than hospitals with the lowest caseload (30% vs. 21%). This difference was statistically not significant after risk-adjustment. Conclusions: Children with ARM are multimorbid. Early complications after corrective surgery are common. Considering the large number of hospitals with a very low caseload, centralization of care for the complex and elective corrective surgery for ARM remains a key issue for quality of care.

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“…Currently, its treatment of choice is Kasai portoenterostomy (KPE). For infants with failed KPE and progressive biliary cirrhosis, the only viable option to ensure patient survival is liver transplantation (LT) [3].…”

Section: Introductionmentioning

confidence: 99%

A novel model based on immune-related genes for differentiating biliary atresia from other cholestatic diseases

Zheng

Zhang

et al. 2022

Pediatr Surg Int

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Based on a public gene expression database, this study established the immune-related genetic model that distinguished BA from other cholestasis diseases (DC) for the rst time. We explored the molecular mechanism of BA based on the gene model. MethodsThe BA microarray dataset GSE46960, containing BA, other cause of intrahepatic cholestasis than biliary atresia and normal liver gene expression data, was downloaded from the Gene Expression Omnibus (GEO) database. We performed a comprehensive bioinformatics analysis to establish and validate an immune-related gene model and subsequently identi ed hub genes as biomarkers associated with the molecular mechanisms of BA. To assess the model's performance for separating BA from other cholestasis diseases, we used receiver operating characteristic (ROC) curves and the area under the curve (AUC) of the ROC. Independent datasets GSE69948 and GSE122340 were used for the validation process. ResultsThe model was built using eight immune-related genes, including EDN1, HAMP, SAA1, SPP1, ANKRD1, MMP7, TACSTD2, and UCA1. In the GSE46960 and validation group, it presented excellent results, and the prediction accuracy of BA in comparison to other cholestasis diseases was good. Functional enrichment analysis revealed signi cant immunological differences between BA and other cholestatic diseases.Finally, we found that the TNFα-NF-κB pathway is associated with EDN1 gene expression and may explain brosis progression, which may become a new therapeutic target. ConclusionIn summary, we have successfully constructed an immune-related gene model that can distinguish BA from other cholestatic diseases, while identifying the hub gene. Our exploration of immune genes provides new clues for the early diagnosis, molecular mechanism, and clinical treatment of biliary atresia.

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Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia

Cited by 4 publications

References 29 publications

Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper

Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021

A novel model based on immune-related genes for differentiating biliary atresia from other cholestatic diseases

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