Split-liver transplantation in the setting of hepatic herniation due to a congenital diaphragmatic hernia in a down syndrome patient

Casaccia, M.; Barabino, Gabriele; Andorno, Enzo; Nicorelli, M.; Porcile, Elisa; Gelli, Maximiliano; Diviacco, P.; Valente, Umberto

doi:10.1002/lt.21597

Cited by 2 publications

(2 citation statements)

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“…The literature on outcomes of abdominal transplantation and specific genetic diseases is sparse. We identified one case report of a 30‐year‐old patient with Down syndrome and cirrhosis who underwent a liver transplant and who was alive with good graft function at 30 months 20 . A single‐center review of kidney transplant patients from 1968 to 1996 identified one recipient with Down Syndrome and one recipient with DiGeorge Syndrome, both of whom underwent successful transplantation and had functional grafts at least 6 months post‐transplantation 21 .…”

Section: Discussionmentioning

confidence: 99%

Genetic disease and intellectual disability as contraindications to transplant listing in the United States: A survey of heart, kidney, liver, and lung transplant programs

Wall

Lee

Maldonado

et al. 2020

Pediatric Transplantation

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Discrimination based on disability is prohibited in organ transplantation, yet studies suggest it continues in listing practices for intellectual disability and genetic diseases. It is not known if this differs between adult and pediatric programs, or by organ type. We performed an online, forced-choice survey of psychosocial listing criteria for adult and pediatric heart, kidney, liver, and lung transplant programs in the United States. Of 650 programs contacted, 343 (52.8%) submitted complete. A minority of programs had formal listing guidelines for any condition considered (Down Syndrome, Duchenne Muscular Dystrophy, Becker Muscular Dystrophy, DiGeorge Syndrome, and Wolf Hirschhorn Syndrome; and mild [IQ < 70] and severe [IQ < 35] intellectual disability), although a majority had encountered most. Pediatric programs were significantly (P < .02) more lenient in the level of contraindication to listing for all genetic conditions considered except Duchenne Muscular Dystrophy, and for mild and severe intellectual disability. Level of contraindication differed significantly by organ type (heart, lung, liver, and kidney) for Duchenne Muscular dystrophy (P = <.001), Becker Muscular Dystrophy (P < .001), DiGeorge Syndrome (P < .001), Wolf-Hirschhorn syndrome (P = .0012), and severe intellectual disability (P < .001). There is significant variation among transplant programs in availability of guidelines for as well as listing practices regarding genetic diseases and intellectual disability, differing by both adult vs pediatric program, and organ type. Programs with absolute contraindications to listing for specific genetic diseases or intellectual disability should reframe their approach, ensuring individualized assessments and avoiding elimination of patients based on membership in a particular group.

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Section: Discussionmentioning

confidence: 99%

Genetic disease and intellectual disability as contraindications to transplant listing in the United States: A survey of heart, kidney, liver, and lung transplant programs

Wall

Lee

Maldonado

et al. 2020

Pediatric Transplantation

View full text Add to dashboard Cite

show abstract

“…Although liver transplantation is routinely feasible in newborns, reports in the setting of Down syndrome are limited to a few cases described worldwide. 6 Because of cardiopulmonary comorbidities and impaired immune status, liver transplantation was not considered reasonable in this case. In due course, the child suffered from progressive intestinal malabsorption, frequently being present in Down syndrome, 7 but potentially being aggravated by increasing portal hypertension caused by the recurrence of arterial inflow to the hepatic malformation.…”

Section: Discussionmentioning

confidence: 98%