Clinical value of split domino donor auxiliary liver transplantation / 中华消化外科杂志

Objective:To investigate the clinical value of split domino donor auxiliary liver transplantation.Methods:The retrospective and descriptive study was conducted. The clinco-pathological data of 3 liver transplantation recipients who were admitted to Nanjing Drum Tower Hospital affiliated to Nanjing University Medical School and 1 liver transplantation recipient who was admitted to external hospital in September 2018 were collected. The first case was male, aged 22 years, who was diagnosed as type II citrullinemia (CTLN2). The second case undergoing liver transplantation in external hospital was male, aged 59 years, who was diagnosed as decompensated alcoholic cirrhosis. The third case was female, aged 52 years, who was diagnosed as hepatocellular carcinoma of right lobe of liver. The fourth case was female, aged 51 years, who was diagnosed as hepatocellular carcinoma of right lobe of liver. The donor liver from a brain and cardiac death donor was split in vitro into the left liver and the right liver, in which the right liver without middle hepatic vein, and the modified piggyback liver transplantation using the left liver and the classical orthotropic liver transplantation using the right liver was conducted on the first and the second case, respectively. The original liver of the first case was split in vivo into the left liver and the right liver, and the piggyback auxiliary liver transplantation using the left liver and the piggyback auxiliary liver transplantation using the right liver was conducted on the third and the fourth case who underwent extended right hemihepatectomy, respectively. Observation indicators: (1) intraoperative situations; (2) follow-up. Follow-up was conducted using outpatient examination and telephone interview to detect liver function, liver imaging, complication and survival of recipients up to October 2021.Results:(1) Intraoperative situations. Liver transplantation was conducted successfully on the first, third and fourth case, with the operation time, the volume of intraoperative blood loss, the donor liver cold ischemia time, the graft-to-recipient weight ratio were 400 minutes, 370 minutes, 390 minutes, 600 mL, 1 300 mL, 1 600 mL, 230 minutes, 152 minutes, 135 minutes, 1.2%, 0.8%, 1.1%. (2) Follow-up. B-ultrasound examination of the first, third and fourth case after liver transplantation showed that the blood flow was normal, and all the 3 cases discharged and were followed up at postoperative 1, 6 and 12 month. The liver function, the level of blood ammonia and citrulline were normal of the first, third and fourth case at postoperative 1 week. Imaging examina-tion showed normal liver morphology of the first and third case, and a transplanted liver atrophy caused by portal vein steal of the fourth case. ① The level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBil), direct bilirubin (DBil) of the first case before liver transplantation, at postoperative 1 day, 2 day, 3 day, 7 day, 10 day, 6 month and 1 year were 22.8 U/L, 404.1 U/L, 355.5 U/L, 289.6 U/L, 31.0 U/L, 23.1 U/L, 42.1 U/L and 25.8 U/L, 31.5 U/L, 517.7 U/L, 327.6 U/L, 172.9 U/L, 15.9 U/L, 21.4 U/L, 47.5 U/L and 29.7 U/L, 3.8 μmol/L, 92.1 μmol/L, 87.4 μmol/L, 79.7 μmol/L, 90.1 μmol/L, 130.6 μmol/L, 33.8 μmol/L and 25.4 μmol/L, 2.3 μmol/L, 47.0 μmol/L, 44.1 μmol/L, 47.1 μmol/L, 57.4 μmol/L, 70.9 μmol/L, 24.7 μmol/L and 9.7 μmol/L, respectively. The level of citrulline and blood ammonia of the first case before and after liver transplantation were 999.0 μmol/L, 196.0 μmol/L and 14.6 μmol/L, 9.0 μmol/L, respectively. The first case was followed up for 3 years and survived without any liver transplantation related complication. ② The level of ALT, AST, TBil, DBil of the third case before liver transplantation, at postoperative 1 day, 2 day, 3 day, 7 day, 10 day, 6 month and 1 year were 21.3 U/L, 143.9 U/L, 182.0 U/L, 132.0 U/L, 17.2 U/L, 10.1 U/L, 17.6 U/L and 16.8 U/L,20.0 U/L, 291.0 U/L, 227.5 U/L, 106.4 U/L, 15.8 U/L, 10.8 U/L, 17.1 U/L and 19.4 U/L, 6.8 μmol/L, 50.9 μmol/L, 45.0 μmol/L, 34.0 μmol/L, 32.4 μmol/L, 22.3 μmol/L, 12.8 μmol/L and 14.9 μmol/L, 2.5 μmol/L, 18.4 μmol/L, 17.2 μmol/L, 14.9 μmol/L, 14.8 μmol/L, 12.1 μmol/L, 3.6 μmol/L and 4.4 μmol/L. The level of citrulline and blood ammonia of the third case after liver transplantation were 24.9 μmol/L and 16.0 μmol/L. The third case was followed up for 3 years and survived without any liver transplantation related complication. ③ The level of ALT, AST, TBil, DBil of the fourth case before liver transplantation, at postoperative 1 day, 2 day, 3 day, 7 day, 10 day, 6 month and 1 year were 35.0 U/L, 268.7 U/L, 682.0 U/L, 425.8 U/L, 57.5 U/L, 34.0 U/L, 29.4 U/L and 18.1 U/L, 37.0 U/L, 419.1 U/L, 436.2 U/L, 139.5 U/L, 35.2 U/L, 32.4 U/L, 54.7 U/L and 32.8 U/L, 7.1 μmol/L, 64.2 μmol/L, 41.4 μmol/L, 17.6 μmol/L, 34.2 μmol/L, 48.7 μmol/L, 14.1 μmol/L and 21.8 μmol/L, 2.8 μmol/L, 18.9 μmol/L, 16.1 μmol/L, 6.0 μmol/L, 14.6 μmol/L, 26.7 μmol/L, 3.9 μmol/L, 11.8 μmol/L. The level of citrulline and blood ammonia of the fourth case after liver transplantation were 8.4 μmol/L and 47.0 μmol/L. One week after surgery, the transplanted right liver of the fourth case occurred atrophy due to blood stealing from the right branch of the portal vein. B-ultrasound examination showed that the reflux of the hepatic artery and hepatic vein was unobstructed. Immunosuppressants were discontinued 3 months after operation on the fourth case and there was no complication such as rejection, bile leakage, biliary stricture, thrombosis and vascular stricture during follow-up. The fourth case died of lung metastasis 19 months after operation.Conclusion:Split domino donor auxiliary liver transplantation can be used for the treatment of metabolic liver disease and advanced hepatocellular carcinoma.

Neonatal intrahepatic cholestasis caused by Citrin deficiency / 中华实用儿科临床杂志

Neonatal intrahepatic cholestasis caused by Citrin deficiency(NICCD) is one of phenotypes of Citrin deficiency.It's an autosomal recessive disorder which was mainly seen in East Asia,including China.Case of NICCD was reported firstly by Japanese in 2001.In south area of China,the morbidity of NICCD is higher than that in north area of China.Most of the patients with NICCD has benign prognosis.Symptoms resolve within the first year of life,thus making a diagnosis difficult after this time.But few of patients will develop liver failure,even be fatal to life.Early diagnosis,regular follow-up and proper management may improve the prognosis.

Overview of Citrin Deficiency:SLC25A13 Mutations and the Frequency / 实用儿科临床杂志

Citrin deficiency, autosomal recessive disorder, caused by mutation of SLC25A13 gene on chromosome 7q21.3 has two major phenotypes : neonatal intrahepatic chnlestatic hepatitis(N1CCD) and adult-onset type Ⅱ citrullinemia(CTLN2).So far, we have identified 52 SLC25A13 mutations and diagnosed the patients not only in Japan(166 CTLN2 and 238 NICCD) but also in other countries.We have detected 76 Chinese, 13 Korean and 15 Vietnamese patients with the same mutations as Japanese, and 13 patients(from Israel, UK, USA or Czech)with mutations different from those found in Japanese,indicating a wide distribution of citrin deficiency.DNA diagnoses of 13 known SLG25A13 mutations revealed that the carrier frequency was high in East Asian populations:Chinese(73/4 600=1/63) ,Japanese(21/1372=1/65) and Korean(25/2 690=1/108), suggesting that near by 100 000 East Asians are liomozygotes.It is important to find out patients with citrin deficiency,to treat them,and to prevent onset of severe CTLN2.