Homozygous familial hypercholesterolemia: specific indication for domino liver transplantation

Popescu, Irinel; Simionescu, Maya; Tulbure, D; Sima, Anca V.; Catana, Cosmin; Niculescu, Loredan S.; Hâncu, Nicolae; Gheorghe, Liana; Mihaila, Mariana; Ciurea, S; Vidu, Viorica

doi:10.1097/01.tp.0000093996.96158.44

Cited by 31 publications

(29 citation statements)

References 23 publications

(19 reference statements)

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“…When hepatic inadequacy occurred because of liver cancer and chronic liver disease, form, esterification and evacuation of cholesterol were blocked, which causes changes of plasma cholesterol level (Cooper et al, 1996;Motta et al, 2001;Ooi et al, 2005;Heeren et al, 2004;Kanel et al, 1983;Popescu et al, 2003); and it was also discovered that serum cholesterol level was negative correlated with occurrence and mortality of some cancers by epidemiologic study (Law and Thompson, 1991;Williams et al, 1981). Study of the correlation between total serum cholesterol and tumor showed that the cholesterol level in patients who died of cancer was significantly lower than that in those who survived (Cambien et al, 1980).…”

Section: Influence Of Liver Cancer On Choles-terol Metabolismmentioning

confidence: 99%

Lipids changes in liver cancer

Jiang

Zhang

et al. 2007

J. Zhejiang Univ. - Sci. B

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Abstract:Liver is one of the most important organs in energy metabolism. Most plasma apolipoproteins and endogenous lipids and lipoproteins are synthesized in the liver. It depends on the integrity of liver cellular function, which ensures homeostasis of lipid and lipoprotein metabolism. When liver cancer occurs, these processes are impaired and the plasma lipid and lipoprotein patterns may be changed. Liver cancer is the fifth common malignant tumor worldwide, and is closely related to the infections of hepatitis B virus (HBV) and hepatitis C virus (HCV). HBV and HCV infections are quite common in China and other Southeast Asian countries. In addition, liver cancer is often followed by a procession of chronic hepatitis or cirrhosis, so that hepatic function is damaged obviously on these bases, which may significantly influence lipid and lipoprotein metabolism in vivo. In this review we summarize the clinical significance of lipid and lipoprotein metabolism under liver cancer.

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Section: Influence Of Liver Cancer On Choles-terol Metabolismmentioning

confidence: 99%

Lipids changes in liver cancer

Jiang

Zhang

et al. 2007

J. Zhejiang Univ. - Sci. B

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“…Since then, more than 540 cases of DLTx have been performed using FAP domino grafts, mostly in Europe (2). Three other kinds of domino liver grafts for metabolic disorders have also been reported, including primary hyperoxaluria (3), HFH (4) and MSUD (5). This report is to describe a domino liver transplantation using an HFH liver as a domino graft with successful results.…”

mentioning

confidence: 99%

Domino liver graft from a patient with homozygous familial hypercholesterolemia

Liu

Niu

Loong

et al. 2010

Pediatric Transplantation

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HFH is a metabolic disease caused by a defect in the gene that encodes the synthesis of the cellular receptor for LDL-Rs. A high plasma level of cholesterol is present from birth and leads to severe atherosclerosis in childhood, and death from myocardial infarction usually occurs before the age of 20 yr. The liver contains approximately 50-75% of the total body LDL-Rs; therefore, liver transplantation has been carried out to treat this metabolic disorder effectively. The rationale for using an HFH liver for a domino graft is that the absence of functional LDL-Rs in the liver may be compensated for by the extra-hepatic LDL-Rs. Therefore, an HFH liver can possibly be used as a domino graft for a recipient with a normal plasma cholesterol level before transplantation. We herein report a domino liver transplantation using an HFH liver as a domino graft with successful results. To expand the donor pool, especially in Asian countries, domino grafts from HFH should be encouraged by careful selection of the domino recipient.

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“…In some cases, the recipient of the domino graft will express serious consequences of the disease afflicting the domino donor, as in the case of hyperoxaluria. 17 In other cases, the recipient of the domino transplant seems to have enough of a delay in onset of symptoms, as in the case of familial amyloid polyneuropathy, or sufficiently attenuated symptoms, as in the case of familial hypercholesterolemia, 18 to make domino transplantation worth considering in cases where the survival of the final recipient is questionable in the time frame for organ procurement through routine channels.…”

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confidence: 99%

Domino liver transplantation in maple syrup urine disease

et al. 2006

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Liver transplantation has been reported in a few cases of maple syrup urine disease (MSUD), but is controversial. Many patients with approved indications for liver transplantation die before grafts are available. A 25-yr-old man with MSUD underwent liver transplantation, and his liver was used as a domino graft for a 53-yr-old man with hepatocellular carcinoma who had low priority on the liver transplant waiting list and was unlikely to survive until routine organ procurement. Both transplants were performed as "piggy back" procedures, reconstructing the domino graft with caval segments from the cadaveric donor. Neither required veno-venous bypass. Whole body leucine oxidation was estimated by 13 CO 2 in breath after oral boluses of L-[1-13 C]-leucine, before and after transplantation in both patients and a control subject. The surgical outcome was successful. The patient with MSUD had marked decreases in plasma branched-chain amino acids (BCAAs) and alloisoleucine (from 255 Ϯ 66 to 16 Ϯ 7 mol/L), despite advancement of dietary protein from 6 to Ͼ40 gm/day. The domino recipient maintained near-normal levels of plasma amino acids with no detectable alloisoleucine on unrestricted diet. Leucine oxidation increased in the patient with MSUD (from 2.2 to 5.6% recovered in 4 hours) and decreased in the recipient (from 9.7 to 6.2%). Neither patient demonstrated any apparent symptoms of MSUD over more than 7 months. In conclusion, liver transplantation substantially corrects whole body BCAA metabolism in MSUD and greatly attenuates the disease. Livers from patients with MSUD may be considered as domino grafts for patients who might otherwise not survive until transplantation. Liver Transpl 12: 876 -882, 2006.

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Homozygous familial hypercholesterolemia: specific indication for domino liver transplantation

Abstract: Domino transplantation can be efficiently used in a patient with marginal indications for transplantation using a liver from a patient with FHC. The slightly elevated serum cholesterol level in the recipient may be explained by the normal function of extrahepatic LDL-R.

Cited by 31 publications

References 23 publications

Lipids changes in liver cancer

Lipids changes in liver cancer

Domino liver graft from a patient with homozygous familial hypercholesterolemia

Domino liver transplantation in maple syrup urine disease

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