Fat luck: three siblings requiring liver transplantation for nonalcoholic steatohepatitis

Bonatti, Hugo; Falkensammer, Juergen; Sawyer, Robert G.; Goegele, Hannes; Aranda-Michel, Jaime; Hinder, Ronald A.; Dickson, Rolland C.; Scolapio, James S.; Nguyen, Justin H.

doi:10.1111/j.1432-2277.2007.00586.x

Cited by 3 publications

(6 citation statements)

References 20 publications

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“…There are multiple case reports of adults who have undergone liver transplantation for cirrhosis secondary to NASH (11). It has also been proposed that a large proportion of cryptogenic cirrhosis may actually have been caused by NASH (6,12).…”

Section: Discussionmentioning

confidence: 99%

Rapid Progression of NASH in Childhood

Kohli

Boyd

Lake

et al. 2010

J. pediatr. gastroenterol. nutr.

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N onalcoholic steatohepatitis (NASH) and nonalcoholic fatty liver disease (NAFLD) have been recognized entities in adult and pediatric medicine for more than 2 decades (1,2). Longterm follow-up studies of adults with NASH have confirmed that fibrosis can progress to cirrhosis (3). Individuals with NAFLD who have hepatic fibrosis and/or inflammation at presentation can progress to more severe fibrosis or cirrhosis and have a higher mortality when compared with individuals with steatosis alone (4-6).Nonalcoholic fatty liver disease is recognized as the most common cause of elevated liver enzymes in children. A recent autopsy series demonstrated that the estimated prevalence of NASH was 2.96% (22/742) in children. Furthermore, 23% of children with NAFLD in this study had NASH, whereas bridging fibrosis or cirrhosis was seen in 9% of children with NASH in this study (7).Long-term natural history data of pediatric NAFLD or NASH are not available. Certain components of routine laboratory tests may be predictive of NAFLD pattern and advanced fibrosis, but these serologic markers do not at present have adequate sensitivity or specificity to replace liver biopsy data to distinguish NASH from NAFLD (8). Serum alanine aminotransferase (ALT) and aspartate animotransferase (AST), for example, are not reliable measures of histological improvement in NASH (9).Thus, liver tissue histology becomes a critical requirement for accurate diagnosis and evaluation of fibrosis progression in patients with NAFLD or NASH. The invasive nature and inherent risk of percutaneous liver biopsy, coupled with the debatable benefit of liver biopsies in guiding therapy or changing clinical outcomes, have prevented gaining a full understanding of the natural history and progression rates of NASH in both children and adults. This report presents 2 children with NASH whose serial liver biopsies demonstrate progression of hepatic fibrosis in a short period of time. This report attempts to highlight that worsening of hepatic fibrosis can occur within childhood in patients with NASH.

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Section: Discussionmentioning

confidence: 99%

Rapid Progression of NASH in Childhood

Kohli

Boyd

Lake

et al. 2010

J. pediatr. gastroenterol. nutr.

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“…2,7,8 Other indications were related to altered cholesterol metabolism. 6 In our series, the indications for orthotopic liver transplant in pediatric cases were biliary cirrhosis, Wilson disease, hepatocellular Abbreviations: ALT, alanine aminotransferase; AST, aspartate aminotransferase; CRP, C-reactive protein; FFP, fresh frozen plasma; INR, international normalized ratio; PRBC, packed red blood cell; RRT, renal replacement therapy carcinoma, and hyperoxalosis; in adult cases, the indications were viral hepatitis and biliary cirrhosis. One previous study in Finland collected data on 6 pairs of siblings with liver transplant, which were selected from 886 individuals who received transplants from 1982 to 2013.…”

Section: Discussionmentioning

confidence: 70%

“…Previous literature experience of liver or kidney transplant in siblings has disseminated mainly through case reports. [2][3][4][5][6][7][8] In pediatric cases, the indications for liver transplant were type IV glycogen storage disease, 4 familial hypercholesterolemia, 5 and Crigler-Najjar syndrome. 3 Adult sibling liver transplant recipients generally had amyloid metabolic disturbances.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Ersoy

Özdemirkan²,

Pirat³

et al. 2015

Exp Clin Transplant

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Objectives: Reasons for chronic liver and kidney failure may vary; sometimes more than 1 family member may be affected, and may require a transplant. The aim of this study was to examine the similarities or differences between the perioperative characteristics of siblings undergoing liver or kidney transplant. Materials and Methods:The medical records of 6 pairs of siblings who underwent liver transplant and 4 pairs of siblings who underwent kidney transplant at Baskent University Hospital between 1989 and 2014 were retrospectively analyzed. Collected data included demographic features; comorbidities; reasons for liver and kidney failure; perioperative laboratory values; intraoperative hemodynamic parameters; use and volume of crystalloids, colloids, blood products, cell saver system, and albumin; duration of anesthesia; urine output; and postoperative follow-up data. Results: The mean age of the 6 sibling pairs who underwent liver transplant was 16.3 ± 12.2 years. All 12 patients had Child-Pugh grade B cirrhosis, with mean disease duration of 7.8 ± 3.9 years. There were no significant differences between siblings with respect to intraoperative blood product transfusion, crystalloid and colloid fluid replacements, hypotension frequency, blood gas analyses, urinary output, duration of anhepatic phase, inotropic agent administration, postoperative laboratory values, need for mechanical ventilation and vasopressors, occurrence of acute renal failure and infections, and duration intensive care unit stay (P > .05). The mean age of the 4 sibling pairs who underwent kidney transplant was 21.3 ± 6.4 years, with mean duration of renal insufficiency of 2.2 ± 1.6 years. There were no significant differences between siblings with respect to intraoperative crystalloid and colloid fluid administration, duration of anesthesia, intraoperative mannitol and furosemide administration, and postoperative laboratory values (P > .05). Conslusions: In conclusion, the 6 sibling pairs who underwent liver transplant and 4 sibling pairs who underwent kidney transplant in our cohort had similar perioperative characteristics.

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“…There have only been two previously published case series describing siblings undergoing LT, along with several case reports of single families with multiple affected siblings. [5][6][7][8][9][10][11] Koljonen at al 5 including non adherence, poor quality of life, and psychological distress. 13 It seems intuitive that these burdens would be increased where multiple siblings are affected, but this has not been formally investigated.…”

Section: Discussionmentioning

confidence: 99%

“…There have only been two previously published case series describing siblings undergoing LT, along with several case reports of single families with multiple affected siblings 5–11 . Koljonen at al 5 reported 14 siblings from six families in the Finnish national transplant center, most of whom were adults at the time of LT.…”

Section: Discussionmentioning

confidence: 99%

Liver transplant for inherited metabolic disease among siblings

McKiernan

Squires

et al. 2020

Clinical Transplantation

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Liver transplantation (LT) is a very successful therapy for many forms of inherited metabolic disease (IMD). 1 Most of these disorders have autosomal recessive inheritance and hence more than one sibling may be affected. This situation is likely to result in unique stresses and challenges for affected families. Despite this, there is little published experience describing outcomes when multiple siblings undergo LT for the same genetic disorder. Indications for LT in IMD fall into two broad groups. Where the defect causes structural liver disease (SLD), LT is utilized to treat complications of end-stage liver disease. In those without SLD, LT is used to correct the metabolic phenotype, hence preventing systemic complications and improving quality of life. The indications for LT in those with SLD are usually clear and consistent, whereas indications to correct the metabolic phenotype are more subjective and still evolving. 1,2 These latter will likely reflect the experience of treating and referring teams, family expectations, and cultural factors. There is no published information as to how families make decisions on timing of LT or order of LT among siblings. It seems likely that decisions on LT for subsequent siblings will be impacted by the experience and outcome of earlier cases. We report our experience from a single pediatric transplant program of siblings who have undergone LT for IMD in order to document indications for LT, if there were differences in LT timing and

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Fat luck: three siblings requiring liver transplantation for nonalcoholic steatohepatitis

Cited by 3 publications

References 20 publications

Rapid Progression of NASH in Childhood

Rapid Progression of NASH in Childhood

Untitled

Liver transplant for inherited metabolic disease among siblings

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