Coexistence of a Graft With the Preserved Native Liver in Auxiliary Partial Orthotopic Liver Transplantation From a Living Donor for Ornithine Transcarbamylase Deficiency1

Üemoto, Shinji; Yabe, S.; Inomata, Yukihiro; Nishizawa, Hideaki; Asonuma, Katsuhiro; Egawa, Hiroto; Kiuchi, Tetsuya; Okajima, Hideaki; Yamaoka, Yoshio; Yamabe, Hirohoko; Inui, Ayano; Fujisawa, Tomoo; Tanaka, Kōichi

doi:10.1097/00007890-199704150-00021

Cited by 48 publications

(26 citation statements)

References 10 publications

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“…Patients transplanted after 1 year of age generally have poor neurological outcomes even with aggressive treatment and dialysis (17). APOLT has been reported as an alternative to OLT in patients with noncirrhotic liver-based metabolic disorders and has been successfully performed in cases of OTC deficiency (9,18,19). APOLT corrects the enzyme deficiency without complete removal of the native liver.…”

Section: Discussionmentioning

confidence: 99%

Hepatocyte Transplantation Followed by Auxiliary Liver Transplantation—a Novel Treatment for Ornithine Transcarbamylase Deficiency

Puppi

Tan

Mitry

et al. 2008

American Journal of Transplantation

103

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† These authors contributed equally to this work.We report the first successful use of hepatocyte transplantation as a bridge to subsequent auxiliary partial orthotopic liver transplantation (APOLT) in a child antenatally diagnosed with severe ornithine transcarbamylase (OTC) deficiency. A total of 1.74 × 10 9 fresh and cryopreserved hepatocytes were administered intraportally into the liver over a period of 6 months. Immunosuppression was with tacrolimus and prednisolone. A sustained decrease in ammonia levels and a gradual increase in serum urea were observed except during episodes of sepsis in the first 6 months of life. The patient was able to tolerate a normal protein intake and presented a normal growth and neurological development. APOLT was successfully performed at 7 months of age. We conclude that hepatocyte transplantation can be used in conjunction with APOLT as an effective treatment for severe OTC-deficient patients, improving neurodevelopmental outcomes.

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Section: Discussionmentioning

confidence: 99%

Hepatocyte Transplantation Followed by Auxiliary Liver Transplantation—a Novel Treatment for Ornithine Transcarbamylase Deficiency

Puppi

Tan

Mitry

et al. 2008

American Journal of Transplantation

103

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“…This carries a poor prognosis with a mortality rate of more than 50% in most series (1). Auxiliary partial orthotopic liver transplantation (APOLT) has recently been performed in patients with acute liver failure and who developed neurologic complications such as fulminant hepatitis accompanied by hepatic coma (2-7) and in patients with noncirrhotic metabolic diseases such as ornithine carbamoyltransferase deficiency (8). In the former, especially in young patients, recovery of liver function and full regeneration of liver cells of the native liver, as well as the eventual withdrawal of immunosuppression, can be expected (6).…”

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confidence: 99%

Sequential Observation of Liver Cell Regeneration after Massive Hepatic Necrosis in Auxiliary Partial Orthotopic Liver Transplantation

et al. 2000

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The morphogenesis of hepatocytes after massive hepatic necrosis to recovery through liver cell regeneration has not been fully understood. Sequential biopsies were performed on the native liver of a 22-year-old man who underwent auxiliary partial orthotopic liver transplantation 1 month after fulminant hepatitis. Auxiliary partial orthotopic liver transplantation was successful, and the biopsy samples permitted us to examine the regenerating process of hepatocytes after massive necrosis. At the time of auxiliary partial orthotopic liver transplantation (postoperative day 0), 95% of hepatocytes were lost and a few ductules were found in the portal areas. The ductules stained with cytokeratin 19. At postoperative day 7, the ductules began to increase in size and number and became dilated over a period of 1 month, when individual hepatocytes with clear cytoplasm appeared from the ductules. As the differentiation of hepatocytes increased, the expression of cytokeratin 19 was found to decrease. From 2 to 3 months, all of the ductules were transformed into hepatocytes, and they began to form round cell clusters. From 3 to 6 months, the round cell clusters became organized into trabecula with fibrosis. From 6 to 12 months, a lobular architecture was established, and by 14 months, the necrotic liver was fully recovered to normal. This study by examination of sequential biopsies demonstrates the progression of the regenerating process from total hepatic necrosis to complete recovery.

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“…[6][7][8][9] Auxiliary orthotopic liver transplantation has recently been performed in patients with fulminant liver failure. [10][11][12][13][14][15][16] Although the experience with auxiliary heterotopic liver transplantation was satisfactory, it appears that auxiliary partial orthotopic liver transplantation (APOLT) had some advantages, including improved portal and systemic vein blood flow and abdominal tissue perfusion. 17,18 One also should note that although rather limited, experience with bioartificial liver systems also showed a regenerative potential of not only hepatocytes, but also of nonparenchymal cells, including the stellate cells.…”

Section: Commentsmentioning

confidence: 99%

Vitamin D receptor polymorphism and posttransplantation bone loss

Hay

2001

Liver Transplantation

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CommentsGenetic factors have an important role in bone metabolism. Epidemiological data, including twin studies, leave little doubt that bone mass and osteoporosis are predominantly influenced by genetic factors, with approximately 80% of the variance in bone density genetically determined. 1 The need for prevention of osteoporosis has logically led to an active search for its genetic predictors. The normal allelic variants of several genes are being investigated for their role in the regulation of bone mass. 2 With the importance of vitamin D in calcium homeostasis, the vitamin D receptor (VDR) gene is an obvious candidate gene, and its 3 common polymorphisms have been the most extensively investigated.Osteoporotic fracturing in the first year after liver transplantation is a major cause of morbidity, occurring in up to 40% of cholestatic patients. 3 It is caused by a combination of 2 effects. First, chronic liver disease may cause pretransplantation osteopenia, which is a major determinant for posttransplantation fracturing. The second skeletal insult is provided by the well-recognized phenomenon of accelerated bone loss in the first months after liver transplantation. The degree of pretransplantation osteopenia and early posttransplantation bone loss are both variable; their underlying causes are unknown, and predictors in the individual patient are poorly defined. The identification of genetic factors influencing hepatic osteopenia and bone loss after liver transplantation would provide clinically useful predictors for posttransplantation fracturing.The study of Guardiola et al investigates the influence of VDR genotypes on bone loss after liver transplantation. They showed that 16 men with the bb genotype (b allele is defined by the presence of the polymorphic Bsm1 restriction endonuclease site) lost less vertebral bone mass at 3 months after transplantation than 20 men with Bb or BB genotypes. This difference apparently persisted up to 24 months, although only 18 patients were followed up to this time. Although these results are very interesting, I believe they have to be assessed with caution. Patient numbers are small, and no fracture data are given. The patients are all men with predominantly viral or alcoholic disease; immunosuppression was cyclosporine based, with initial quadruple therapy; and information is not available to assess the possible beneficial mechanism of the favorable genotype. Such results, even if confirmed by a larger study in this population, may not be applicable to the general posttransplantation population.The importance of the VDR gene in the regulation of bone mass in the general and postmenopausal population is still highly controversial and adds more caution to interpretation of this study. The results of studies with much larger numbers of healthy patients have been extremely variable, 3 with some studies showing a positive correlation between bone mass and VDR alleles [5][6][7][8] and some studies showing no correlation. 2,9-12 These major discrepancies remain u...

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Coexistence of a Graft With the Preserved Native Liver in Auxiliary Partial Orthotopic Liver Transplantation From a Living Donor for Ornithine Transcarbamylase Deficiency1

Abstract: APOLT seems to be most useful for the treatment of noncirrhotic metabolic liver diseases.

Cited by 48 publications

References 10 publications

Hepatocyte Transplantation Followed by Auxiliary Liver Transplantation—a Novel Treatment for Ornithine Transcarbamylase Deficiency

Hepatocyte Transplantation Followed by Auxiliary Liver Transplantation—a Novel Treatment for Ornithine Transcarbamylase Deficiency

Sequential Observation of Liver Cell Regeneration after Massive Hepatic Necrosis in Auxiliary Partial Orthotopic Liver Transplantation

Vitamin D receptor polymorphism and posttransplantation bone loss

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