Living donor liver transplantation in Alagille syndrome—Single center experience from south Asia

Abstract

To analyze the clinical characteristics and the outcomes of living donor liver transplantation in children with Alagille syndrome (AGS). Clinical data of children with AGS who underwent liver transplantation between July 2009 and May 2019 in our unit were retrospectively analyzed. Primary end‐points were patient and graft survival. Ten children with AGS underwent living donor liver transplantation at a median age of 28 months (range, 12‐84 months). Jaundice was the most common initial symptom and was noted after a median duration of 20 days after birth (range, 7‐60 days). Two patients had undergone Kasai porto‐enterostomy for misdiagnosis of biliary atresia. The most common indication for transplantation was severe pruritus with poor quality of life. Explant livers in three children showed cirrhosis with early well‐differentiated hepatocellular carcinoma. We have 100% patient and graft survival at a mean follow‐up of 32 months (range 3‐72 months). The median z‐score for weight and height at liver transplantation was −2.66 (range: −6.44 to −0.9) and −3.6 (range: −7.96 to −0.93) while at follow‐up was −1.7 (range: −3.4 to −0.35) and −2.1 (range: −3.9 to −1.4), respectively. The estimated glomerular filtration rate was normal pretransplant and follow‐up. This is the first series of LDLT for Alagille syndrome in the Indian sub‐continent. We report excellent post‐transplant outcomes in contrast to outcomes reported from Western literature.

Liver Transplantation for Pediatric Hepatocellular Carcinoma: A Systematic Review

“…After screening titles and abstracts, 563 articles were retrieved for full-text evaluation. Ultimately, 67 non-overlapping studies [ 11 , 12 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 ,…”

Multidisciplinary Management of Alagille Syndrome

“…On a median post-LT follow-up of 32 months, there has been 100% graft and patient survival. 20 It is to be noted that the disease does not recur in the graft. Celiac artery stenosis and a hypoplastic hepatic artery are commonly noted in ALGS, and hence an extra-anatomical arterial inflow may be required.…”

“…Using this protocol, our recipients have retained a normal estimated glomerular filtration rate (eGFR) in the post-LT period. 20 There are isolated reports of combined liver–kidney transplantation in patients with ALGS.…”

“…The presence of any deviation indicates a pre-LT liver biopsy to rule out PIBD. 20 If no stigmata are found, there is no contraindication for phenotypically normal first-degree relatives to be LDLT donors. Moreover, since there is no specific genotype–phenotype correlation in patients with ALGS, the role of genetic studies from the perspective of organ donation is not well defined.…”

“…Those with structural renal anomalies require antibiotic prophylaxis followed by definite corrective procedures. As mentioned above, patients in the post-LT period should be commenced on renal sparing immunosuppression even if they have a normal eGFR prior to LT. 20 In case of end-stage renal disease (ESRD), renal transplantation may be indicated, occasionally combined with a LT.…”