To analyze clinical efficacy and prognosis of liver transplantation in children with hyperammonemia caused by urea cycle disorders.A retrospective analysis was performed on the occurrence of disease, operation and the follow-up post liver transplantation in 4 patients with urea cycle disorders who underwent liver transplantation during June 2001 to May 2014.Four girls were diagnosed with ornithine carbamoyl transferase deficiency by genetic test. They had the clinical onset at the age of 1.5 to 3.0 years. Liver transplantation had been performed at their age of 53.9 months, 40.6 months, 40.3 months and 22.8 months, respectively. The grafts of case 1 and case 2 were from left lateral lobe of liver of cadaveric donor, the graft of case 3 was from left lateral lobe of liver of a living donor, the graft of case 4 was a whole liver of a dead child. The liver function of 4 patients gradually returned to normal, blood ammonia levels were normal and restored the normal diet, 4 children were discharged on postoperative 25-30 days. Regular follow-up was done, the liver function, biochemical features and growth status have been followed up for 162.2 months, 124.2 months, 12.0 months and 4.8 months after liver transplantation, respectively. Now, all the four cases are healthy and growth is normal.Liver transplantation is an important way to the patients with severe hyperammonemia caused by urea cycle disorders. In this study, the patients with ornithine carbamoyl transferase defect got satisfactory long-term outcome after liver transplantation.
Objective The present study was conducted to screen rice materials pyramiding brown planthopper and rice blast genes for providing reference on breeding new rice germplasms with resistance against brown planthopper (BPH) (Ni-laparvata lugens Sta°l) and rice blast caused by the fungus Magnaporthe grisea. Method By using conventional breeding, molecular marker-assisted breeding and BPH resistance bioassay, BPH resistance genes viz., bph20(t) and bph21(t), and the broad-spectrum and highly resistant blast resistance gene Pi9 were pyramided into the genetic background of an elite main-tainer line BoIIIB. ResultThe molecular markers RM540 and BYL7 for bph20(t), as well as RM5348 and RM222 for bph21(t), could not amplify specific bands and showed no obvious polymorphism among the resistant parents and susceptible parents, so they could not be used in molecular marker-assisted selection of the present breeding population. The dominant molecular marker pB8 and codominant molecular marker PB9-1 for Pi9 could amplify specific bands and showed polymorphism among the resistant parents and susceptible parents, and could be used in the molecular marker assisted selection of the present breeding population. The marker PB9-1 was used for PCR amplification of 22 rice plants in BC 6 F2 population after insect resistant identification, It was found that 10 plants was heterozygote with Pi9 gene, 6 plants was homozygote and 7 plants had no Pi9 gene. By insect resistant identification using rice plants susceptible to brown planthopper and rice blast in field of Nanning, a series of rice plants against to brown planthopper, and 5 accessions had Pi9 gene and resistance to rice blast among 6 accessions with equivalent resistance of parent BPH54. Conclusion By using conventional breeding, molecular detection and insect-disease resistance bioassay, 5 accessions of above resistant rice intermediate materials pyramiding brown planthopper genes and rice blast gene, which could provide germplasm meterials for breeding new double resistant maintainer line and sterile line.
Objective
To analysis the clinical effect and outcomes of 15 case of ABO-incompatible liver transplantation.
Method
The date of 15 cases ABO-incompatible liver transplantation between August 2013 to August 2015 was analyzed retrospectively. The age, primary disease, level of anti-blood group antibodies, preoperative and intraoperative parameters, postoperative complications and prognosis were analyzed. Among the 15 ABO-incompatible liver transplantation cases, 6 cases were children, age of the recipients was from 5.0 months to 39.6 months, and 5 cases were diagnosed as biliary atresia, 1 cases was diagnosed as progressive familial intrahepatic cholestasis type 1 (Byler disease); 9 cases were adults, age from 45.2 years to 69.6 years, among them, 5 patients were Hepatitis B virus (HBV) cirrhosis with HBV Hepatocellular carcinama, 2 patients were alcoholic cirrhosis and HCC, 2 patients were HBV liver cirrhosis.
Result
The antibody titer of IgM was 1∶4~1∶256 in patients before operation, and the antibody titer of IgG was 1∶4~1∶256 before operation. 4 cases underwent splenectomy during operation, 1 case underwent splenectomy 4 years before liver transplantation. 10 cases of patients injected with Rituximab, 375 mg/m2 preoperatively; 5 cases of pediatric patients did not use rituximab. All patients received intravenous immunoglobulin 400 mg·kg-1·d-1 during operation to 7 days. 2 patients died in the perioperative period, 1 patients died of tumor recurrence postoperation, and the remaining 12 patients were alive and the graft function was good.
Conclusion
The ABO-incompatible liver transplantation can obtain relatively satisfactory prognosis by preoperative evaluation and perioperation treatment with Rituximab injection and intravenous immunoglobulin.
Key words:
Liver transplantation; Child; ABO blood-group system; Blood group incompatibility; Living donor; Prognosis
Appendage regeneration relies on the formation of blastema, a heterogeneous cellular structure formed at the injury site. However, little is known about the early injury-activated signaling pathways that trigger blastema formation during appendage regeneration. Here, we provide compelling evidence that the extracellular signal-regulated kinase (ERK)-activated casein kinase 2 (CK-2), which has not been previously implicated in appendage regeneration, triggers blastema formation during leg regeneration in the American cockroach,
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