Biliary drainage as treatment for allograft steatosis following liver transplantation for <scp>PFIC</scp>‐1 disease: A single‐center experience

Alrabadi, Leina; Morotti, Raffaella; Valentino, Pamela L.; Rodríguez-Dávalos, Manuel I.; Ekong, Udeme D.; Emre, Sükrü

doi:10.1111/petr.13184

Cited by 17 publications

(27 citation statements)

References 23 publications

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“…13,14 Owing to the resulting colonic reflux, attempts at reversing the stoma have been met with failures. 15 While total external biliary diversion may be better at achieving complete biliary diversion, TIBD is more acceptable to the patients and is associated with a better quality of life. Mali et al showed that TIBD performed at the time of LT could abrogate the diarrhea and steatosis.…”

Section: Discussionmentioning

confidence: 99%

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Total Internal Biliary Diversion for Post–Liver Transplant PFIC-1–Related Allograft Injury

Shanmugam

Menon

Vij

et al. 2022

Journal of Clinical and Experimental Hepatology

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Section: Discussionmentioning

confidence: 99%

“…To avoid the risk of colonic reflux and cholangitis, the isolated jejunal segment is left long (approximately 30 cm). 15 There, however, remains the risk of malnutrition due to nonabsorption of fat-soluble vitamins due to TIBD. These children will therefore need replacement of these nutrients.…”

Section: Discussionmentioning

confidence: 99%

Total Internal Biliary Diversion for Post–Liver Transplant PFIC-1–Related Allograft Injury

Shanmugam

Menon

Vij

et al. 2022

Journal of Clinical and Experimental Hepatology

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“…Several subsequent reports showed outstanding outcomes of post-transplant biliary diversion, with relief of diarrhea and regression of liver steatosis ( Table 3 ). Some authors described good results of internal biliary diversion used preemptively at the time of LT as a stoma-free procedure to prevent postoperative graft steatosis or as a next step after PEBD [ 9 , 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…Unlike PFIC-2, PFIC-1 is multiorgan disorder due to the broad tissue distribution of FIC1 expression [ 1 , 2 ]. Extrahepatic symptoms, such as diarrhea, pancreatitis, deafness, and poor growth may persist, develop, or aggravate after liver transplantation (LTx) [ 3 – 9 ]. Moreover, the presence of steatohepatitis progressing to cirrhosis and the need for re-transplantation may be observed after LTx [ 3 – 6 ].…”

Section: Introductionmentioning

confidence: 99%

A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant

et al. 2021

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Case series Patients: Male • Male / (siblings) Final Diagnosis: Progressive intrahepatic familial cholestasis type 1 (PFIC-1) Symptoms: Jaundice Medication: — Clinical Procedure: — Specialty: Transplantology Objective: Unusual clinical course Background: Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). The role and timing of LTx in PFIC-1 remains debated. We present 2 case reports of male siblings with PFIC-1 who benefited from different treatments. Case Report: Both siblings harbored a homozygous truncating mutation in ATP8B1 characteristic for PFIC-1 and both underwent PEBD after unsuccessful UDCA treatment at the age of 7 and 5 months, respectively. The older brother, after initial improvement of symptoms, developed severe pruritus, cholestasis, and diarrhea 9 months after PEBD and underwent LTx at the age of 16 months. Chronic diarrhea and abnormal transaminases activity appeared soon after transplantation. A liver biopsy was performed 3 months after LTx and showed severe macrovesicular steatosis (95%). Sixteen months after LTx, total biliary diversion was performed, with rapid relief from diarrhea and significant regression of graft steatosis by <30%. In his brother we observed persistent severe pruritus and cholestasis after PEBD, but we decided to postpone LTx due to lack of a living related donor and risk of graft steatosis. Eight months after PEBD, bilirubin and bile acids significantly decreased and pruritus disappeared completely. Currently, in 5-year follow-up, liver function is stable and he has no pruritus. Conclusions: The good effect of PEBD may be delayed in PFIC-1, even in severe mutation; thus, the decision to perform LTx should be made cautiously. Total biliary diversion is an efficient procedure in case of persistent symptoms after LTx and can reverse graft steatosis in children with PFIC-1.

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“…Total biliary diversion involves diverting all the bile flow from the common bile duct to the exterior through a jejunal loop. It is usually reserved for pruritus refractory to PEBD or to prevent and treat allograft steatosis in post LT PFIC1[ 56 ]. Irrespective of the technique used, biliary diversion surgeries lead to a decrease in the concentration of BA, improvement in pruritus score, growth spurt, histological improvement and even reversal of fibrosis in some cases[ 38 , 47 ].…”

Section: Therapeutic Strategies For Pficmentioning

confidence: 99%

Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies

Alam¹,

Lal²

2022

WJH

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Recent evidence points towards the role of genotype to understand the phenotype, predict the natural course and long term outcome of patients with progressive familial intrahepatic cholestasis (PFIC). Expanded role of the heterozygous transporter defects presenting late needs to be suspected and identified. Treatment of pruritus, nutritional rehabilitation, prevention of fibrosis progression and liver transplantation (LT) in those with end stage liver disease form the crux of the treatment. LT in PFIC has its own unique issues like high rates of intractable diarrhoea, growth failure; steatohepatitis and graft failure in PFIC1 and antibody-mediated bile salt export pump deficiency in PFIC2. Drugs inhibiting apical sodium-dependent bile transporter and adenovirus-associated vector mediated gene therapy hold promise for future.

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Biliary drainage as treatment for allograft steatosis following liver transplantation for PFIC‐1 disease: A single‐center experience

Cited by 17 publications

References 23 publications

Total Internal Biliary Diversion for Post–Liver Transplant PFIC-1–Related Allograft Injury

Total Internal Biliary Diversion for Post–Liver Transplant PFIC-1–Related Allograft Injury

A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant

Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies

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