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Background: Long-term efficiency of attenuated immunosuppressive therapies is not well characterized in pediatric liver transplantation (LT)., Objective: To assess the efficiency of tacrolimus once daily (TAC-OD) and sirolimus once daily (SLR-OD) immunosuppression in pediatric LT., Methods: We retrospectively evaluated 59 children who underwent LT in our center during 2002 to 2016. Those including children who underwent planned decrease in immunosuppressant dose (stable clinical conditions after 2 years of LT), and those who underwent unplanned decrease in immunosuppressant dose (because of complications such as post-transplant lymphoproliferative disorder [PTLD] and renal failure)., Results: 25 of 59 children underwent planned decrease in immunosuppressant dosage (mean±SD duration of 4.5±1.8, range: 3-11 years); 34 had unplanned decrease (mean±SD of 1.3±0.6, range: 0.5-2.6 years). 19 of 25 children with planned conversion received TAC-OD; 6 received SLR-OD (22 with 1 mg/day dose, and 3 with 1 mg every two days). Of 34 children with unplanned conversion, 27 received TAC-OD, 7 SLR-OD (25 children with 1 mg/day, 7 with 1 mg every two days, 1 with 0.5 mg/day TAC, and 1 with 0.5 mg TAC every two days). We found no adverse events including acute or chronic graft rejection, renal insufficiency, infections, PTLDs, or cardiovascular thrombotic events after initiation of the modified immunosuppression in none of the groups., Conclusion: TAC-OD or SLR-OD monotherapies are safe and effective for long-term management of LT children with either stable clinical conditions or those with LT complications.

Background: Wilson's disease (WD) is an autosomal-recessive hereditary liver disease affecting copper metabolism., Objective: To test the diagnostic value of a questionnaire for the diagnosis of WD in pediatrics age group., Methods: 70 children with biopsy-proven diagnosis of WD and 70 without WD were included in the study. A modified questionnaire with 4 items was used for the diagnosis of WD. The results were then compared to the definite diagnosis made by pathology (the gold standard test)., Results: The median (IQR) modified score in those with WD was 4 (4-5), significantly (p<0.001) higher than that calculated for the comparison group, which was 0 (0-1). The most appropriate cut-off value for the score was 2.5, corresponding to a sensitivity and specificity of 100%, and 98.6%, respectively. Using this cut-off value to classify 20 children with and without WD who underwent liver transplantation resulted in an accuracy of 100%., Conclusion: The modified scoring system is a sensitive and specific diagnostic tool for the diagnosis of WD in children. This is especially important in regions with limited access to specific laboratory tests for the diagnosis of WD.

Background: Particular requirements of pediatric and adolescent liver-transplant (LT) recipients necessitate the evaluation of such population from the endocrine viewpoint., Objective: To determine the endocrine disorders among LT recipients., Methods: 129 LT recipients younger than 18 years, and at least 6 months post-LT with no pervious history of endocrine disorders were included in the study. Demographic, anthropometric and biochemical data were collected., Results: 36% of cases had evidence of impaired fasting glucose; the problem, however, was dramatically resolved (decreased to 2.3%) by using of prediabetic diet. Identifying only 1 case of primary hypothyroidism indicated that thyroid dysfunction seems not to be a prevalent finding in the patients. 3 cases of rickets and no case of parathyroid dysfunction were identified. 11% of the study population were hypocalcemic (2 had rickets as well). Pubertal condition in 3 patients and delayed puberty before LT in 6 remained the same; further evaluation revealed they had hypogonadotropic hypogonadism., Conclusion: Regular monitoring for development of diabetes and hypocalcemia is indicated. Evaluation of those with delayed puberty for receiving sexual hormones is also recommended.

Background: Perivalvular and valve involvement are prevalent in patients with end-stage renal disease (ESRD), especially in younger patients compared with normal population. Kidney transplantation improves the prognosis of these patients. Patients with cardiac valvular disease is also be improved following kidney transplantation., Objective: To evaluate the impact of renal transplantation on the severity of mitral regurgitation (MR)., Methods: We studied 95 kidney transplantation candidates in Sina Hospital. The patients underwent echocardiography preoperatively and at the 3 rd , 6 th , and 12 th months post-operatively., Results: Pre-operatively, the average MR fraction was 30%; MR volume 30 mL/beat; mitral valve mean gradient 1.8 mm Hg; mitral valve area 4.6 cm 2 ; and mitral annular size 3 cm. No significant difference was observed among the measurements made at the 3 rd , 6 th , and 12 th months post-operatively., Conclusion: There was no significant association between the variables measured pre- and post-operatively. The reason might be the fact that patients with ESRD in Iran do not have to expect long transplant waiting lists and dialysis cannot affect their heart adversely.

Background: Acute cellular rejection (ACR), a reversible process, can affect the graft survival., Objective: To evaluate the relation between ACR and clinical factors in recipients of allograft liver transplantation., Methods: 47 recipients of liver were consecutively enrolled in a retrospective study. Their information were retrieved from their medical records and analyzed., Results: Of the 47 recipients, 38 (81%) experienced acute rejection during 24 months of the transplantation. None of the studied factors for occurring transplant rejection, i.e ., blood groups, sex, age, familial history of disease, receiving drugs and blood products, type of donor, Child score, and Child class, was not found to be significant., Conclusion: During a limited follow-up period, we did not find any association between ACR and suspected risk factors.

Background: Acute cellular rejection (ACR) has a reversible effect on graft and its survival., Objective: To evaluate the relation between ACR and clinical factors in recipients of liver transplant allografts., Methods: 47 consecutive liver recipients were retrospectively studied. Their data were extracted from records and analyzed., Results: 38 (81%) of the 47 recipients experienced ACR during a 24-month follow-up. The rate of rejection was associated with none of the studied factors-recipient's blood group, sex, age, familial history of disease, drugs and blood products received, type of donor, and Child score and class., Conclusion: During a limited follow-up period, we did not find any association between ACR and suspected risk factors., Competing Interests: None declared.

Background: So far numerous post-transplant outcome predictors have been studied to decrease the loss of resources and grafts after organ transplantation. The role of education, as a predictor, in liver transplantation outcome has so far been studied in several articles. However, in most of the studies it was evaluated as a surrogate for socioeconomic status or other variants. The absolute impact of parents' education has rarely been studied. Adult patients are their own caregivers whereas pediatric liver transplantation recipients are mostly cared by their parents., Objective: To evaluate the effect of level of patients' education on the mortality and morbidity of pediatric liver transplant recipients., Methods: We studied a group of 91 children who had undergone liver transplantation in our center from March 21, 2012 to July 21, 2013. In this retrospective study, patients' medical charts and questionnaire were used to collect the necessary data. Post-transplantation mortality and complications were divided into two categories: Early (<6 months after liver transplantation), and late (≥6 months after the transplantation). Parents' educational level was also categorized into 5 groups., Results: Multivariate analysis of all groups showed that paternal education is an independent predictor of the late post-transplantation complications (p=0.024). Educational level of children's mothers had no significant correlation with the late post-transplantation complications (p=0.45). Neither maternal (p=0.59) nor paternal (p=0.607) education had significant effect on the late post-transplantation mortality., Conclusion: Paternal educational level of liver transplanted children is associated with the late post-transplantation complications.

Background: Wound healing disorders are probably the most common post-transplantation surgical complications. It is thought that wound healing disturbance occurs due to antiproliferative effects of immunosuppressive drugs. On the other hand, success of transplantation is dependent on immunosuppressive therapies. Antihuman thymocyte globulin (ATG) has been widely used as induction therapy but the impact of this treatment on wound healing is not fully understood., Objective: To investigate wound healing complications after ATG therapy in renal transplant recipients., Methods: The medical records of 333 kidney transplant recipients were assessed for wound healing disorders. Among these patients, 92 received ATG and 5 doses of 1.5 mg/kg ATG along with the standard protocol of drugs., Results: The mean age of patients was 38.9 years. Of 333 recipients, 92 (23.7%) received ATG; 21 (6.3%) developed wound healing complications. There was a significant relationship between ATG therapy and wound complications (p=0.034). Also, women were more likely to develop wound healing disorders than men (p=0.002). No statistical difference was observed between age and wound healing complication (p=0.28). There was no significant difference between the mean duration of hospitalization between ATG and Non-ATG group (p=0.9)., Conclusion: ATG increases the risk of overall wound complications. It is needed to pay more attention to the patients treated with this immunosuppressant to avoid the risk of re-interventions, lessen the duration of hospitalization and decrease the impairment of graft function.

Background: Liver transplantation (LT) is the standard treatment of end-stage liver diseases (ESLD). Invasive fungal infection is one of the important causes of morbidity and mortality after transplantation., Objective: To determine the incidence of late-onset (after 6 months of LT) Candida infection in recipients., Methods: A retrospective study was conducted to evaluate 50 pediatric patients after LT for 8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. We followed the patients until 6 months post-LT for episodes of Candida infection proven by culture., Results: One recipient (2%) developed late-onset esophageal candidiasis with improvement after intravenous amphotricin therapy but finally expired with a diagnosis of post-transplant lymphoproliferative disorder (PTLD)., Conclusions: The incidence of late-onset Candida infection is not significant in pediatric liver recipient, but it still remains a significant problem. Control of Candida colonization would reduce the risk of invasive fungal infections and possibly more fatal complications.

Background: Liver transplantation (LT) is a life-saving treatment for end-stage liver diseases (ESLD). Cytomegalovirus (CMV) infection is one of the important causes of morbidity after LT., Objective: To evaluate the incidence of late-onset (after 6 months of LT) CMV infection in pediatric recipients., Methods: A retrospective analysis was conducted to evaluate 50 pediatric patients who underwent LT for 8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. We retrospectively investigated episodes of CMV infection after 6 months of LT proven by CMV antigenemia test., Results: Three recipients (6%) developed late-onset CMV infection. These patients finally responded to ganciclovir., Conclusion: CMV infection is one of the most common post-LT viral infections that usually occurs in the first six months of LT. Our study shows that the incidence of late-onset CMV infection is relatively low, but it still remains a significant problem. Therefore, monitoring and management is crucial for improving the survival of children.