Clinical and Imaging Features of Cystic Fibrosis in Korean Children.

Cystic fibrosis (CF) is a fatal hereditary disorder that primarily affects Caucasians and is rare in Asian populations, including Koreans. Diagnosing CF is often challenging and delayed owing to its rarity and its overlapping features with non-CF diseases, ultimately affecting the patient prognosis. Radiologists can provide initial clues for clinically unsuspected cases and play a crucial role in establishing an early childhood diagnosis. This pictorial essay reviews the clinical and imaging features of genetically confirmed CF in Korean children and increases awareness of this rare disease, thereby facilitating early diagnosis.

Prognostic significance of body mass index in small-cell lung cancer: Exploring the relationship with skeletal muscle status.

BACKGROUND: We investigated the prognostic significance of body mass index in small-cell lung cancer and explored whether skeletal muscle status affects the body mass index-survival relationship. METHODS: This retrospective study evaluated data from patients who underwent platinum-etoposide chemotherapy for small-cell lung cancer between March 2010 and December 2021. Skeletal muscle status was assessed using non-contrast computed tomography images of baseline positron-emission tomography-computed tomography, with the skeletal muscle index defined as the cross-sectional area of skeletal muscle divided by height squared, and the average attenuation values of skeletal muscle. Cox proportional hazards regression analysis was used to determine the correlations of body mass index, skeletal muscle metrics, and overall survival. RESULTS: We analysed the data of 1146 Asian patients (1006 men and 140 women, with a median age of 67 years [interquartile range: 61-72 years]), including 507 and 639 patients with limited and extensive disease, respectively. Being underweight, defined as a body mass index <18.5 kg/m2 , was associated with shorter overall survival, independent of clinical covariates in both the limited-disease (hazard ratio, 1.77; 95% confidence interval, 1.01-3.09) and extensive-disease (hazard ratio, 1.71; 95% confidence interval, 1.18-2.48) groups. The prognostic value of being underweight remained significant after additional adjustment for skeletal muscle index and attenuation in both limited-disease (hazard ratio, 1.96; 95% confidence interval, 1.09-3.51) and extensive-disease (hazard ratio, 1.75; 95% confidence interval, 1.17-2.61) groups. CONCLUSIONS: Being underweight is an independent poor prognostic factor for shorter overall survival in Asian patients with small-cell lung cancer, regardless of skeletal muscle status.

Redo pull-through for postoperative complications following pull-through in Hirschsprung disease: a single center experience.

Purpose: Although surgical management of Hirschsprung disease (HD) is effective in most patients, some patients experience long-term postoperative complications, and require redo pull-through (PT). The present study evaluated clinical outcomes of redo PT in HD patients at a single center. Methods: Patients with HD who underwent redo PT procedures between 2003 and 2019 were retrospectively reviewed. Results: Thirteen patients were included. Five (38.5%) had undergone initial PT surgery at our center and 8 (61.5%) at other centers. Redo PT procedures were transanal endorectal PT in 12 patients (92.3%) and the posterior sagittal approach in 1 patient (7.7%). Indications for redo PT included pathologic misdiagnosis in 8 patients (61.5%); stricture in 2 (15.4%); and rectal stenosis, obstructing Duhamel pouch and remnant septum in 1 each (7.7%). At a median follow-up of 68 months (range, 3-227 months) after redo PT, 8 patients (61.5%) had normal bowel function, 2 (15.4%) had incontinence, and 1 (7.7%) had constipation. Conclusion: Redo PT procedures could be an effective approach for improving obstructive symptoms in HD patients with anatomic or pathologic reasons following primary PT. Careful selection of patients and discreet indications for redo PT are crucial.

E2F regulation of the Phosphoglycerate kinase gene is functionally important in Drosophila development.

The canonical role of the transcription factor E2F is to control the expression of cell cycle genes by binding to the E2F sites in their promoters. However, the list of putative E2F target genes is extensive and includes many metabolic genes, yet the significance of E2F in controlling the expression of these genes remains largely unknown. Here, we used the CRISPR/Cas9 technology to introduce point mutations in the E2F sites upstream of five endogenous metabolic genes in Drosophila melanogaster. We found that the impact of these mutations on both the recruitment of E2F and the expression of the target genes varied, with the glycolytic gene, Phosphoglycerate kinase (Pgk), being mostly affected. The loss of E2F regulation on the Pgk gene led to a decrease in glycolytic flux, tricarboxylic acid cycle intermediates levels, adenosine triphosphate (ATP) content, and an abnormal mitochondrial morphology. Remarkably, chromatin accessibility was significantly reduced at multiple genomic regions in PgkΔE2F mutants. These regions contained hundreds of genes, including metabolic genes that were downregulated in PgkΔE2F mutants. Moreover, PgkΔE2F animals had shortened life span and exhibited defects in high-energy consuming organs, such as ovaries and muscles. Collectively, our results illustrate how the pleiotropic effects on metabolism, gene expression, and development in the PgkΔE2F animals underscore the importance of E2F regulation on a single E2F target, Pgk.

Dietary Restriction Impacts Peripheral Circadian Clock Output Important for Longevity in Drosophila.

Circadian clocks may mediate lifespan extension by caloric or dietary restriction (DR). We find that the core clock transcription factor Clock is crucial for a robust longevity and fecundity response to DR in Drosophila. To identify clock-controlled mediators, we performed RNA-sequencing from abdominal fat bodies across the 24 h day after just 5 days under control or DR diets. In contrast to more chronic DR regimens, we did not detect significant changes in the rhythmic expression of core clock genes. Yet we discovered that DR induced de novo rhythmicity or increased expression of rhythmic clock output genes. Network analysis revealed that DR increased network connectivity in one module comprised of genes encoding proteasome subunits. Adult, fat body specific RNAi knockdown demonstrated that proteasome subunits contribute to DR-mediated lifespan extension. Thus, clock control of output links DR-mediated changes in rhythmic transcription to lifespan extension.

Comparison of splenic vessel preserving distal pancreatectomy and the Warshaw technique for solid pseudopapillary neoplasm in children.

PURPOSE: Spleen-preserving distal pancreatectomy (SPDP) such as splenic vessel preservation (SVP) for solid pseudopapillary neoplasms (SPNs) in the body or tail of the pancreas in children prevents post-splenectomy infection and hematologic disorders. However, SVP could be technically challenging, and the Warshaw technique (WT) could be an alternative technique that has been reported to be safe and effective in adults. We, therefore, compared the perioperative outcomes of SVP and WT in pediatric patients. METHODS: We retrospectively reviewed the medical records of pediatric patients with SPN who underwent SPDP using SVP or WT between November 2002 and November 2018 at a large-sized tertiary referral center. RESULTS: Twenty-eight patients were included. Sixteen (57.1%) patients underwent SVP and 12 (42.9%) patients underwent WT. There were no significant differences in the baseline characteristics between the two groups. Postoperative complications occurred in 8 patients each in the SVP (50%), and the WT (66.7%) groups. Two (12.5%) in the SVP group and 1 (8.3%) in the WT group required additional intervention. During a median follow-up duration of 49 months, there were no significant differences in the incidence of splenic infarctions or perigastric varices between the two groups. CONCLUSIONS: There were no significant differences in the surgical outcomes between WT and SVP in pediatric patients with SPN. WT could be a safe and feasible alternative technique for SVP in challenging cases.

Heart-Sparing Capability and Positional Reproducibility of Continuous Positive Airway Pressure in Left-Sided Breast Radiation Therapy.

PURPOSE: Our purpose was to investigate the interfraction and intrafraction reproducibility and practical applicability of continuous positive airway pressure (CPAP) for left breast volumetric modulated arc therapy (VMAT). METHODS AND MATERIALS: Interfraction reproducibility of the position of the heart was evaluated by measuring the heart-to-target distance on 20 planning computed tomography (CT) and 300 daily cone beam CT of 20 patients with left breast cancer treated with a 15-fraction VMAT. The dosimetric metrics of the whole heart and its substructures were compared between CPAP and free-breathing based VMAT plans. Intrafraction reproducibility was evaluated by measuring the motions of the breast target and diaphragm in 4-dimensional CT of 20 female patients with nonbreast cancer. Lastly, we analyzed the CPAP compliance data of 237 consecutive patients with left-sided breast cancer with and without internal mammary node irradiation (IMNI). RESULTS: The heart position was reproducible as evidenced by an absolute average heart-to-target distance error of 2.0 ± 2.0 mm. Compared with free-breathing, CPAP significantly reduced the mean heart dose and the dose to the left ventricle and left anterior descending artery. The average intrafraction position variation of the breast target was 0.5 ± 0.5, 2.5 ± 2.0, and 1.8 ± 1.4 mm in the mediolateral, craniocaudal, and anteroposterior directions, respectively. CPAP was successfully applied in 221 patients (93%), with a mean heart dose of 1.6 ± 0.7 Gy (IMNI: 2.0 Gy and no IMNI: 1.1 Gy). CONCLUSIONS: CPAP has adequate heart-sparing capability and sufficient reproducibility in VMAT for left-sided breast cancer treatment, with a high compliance rate. Thus, CPAP is applicable in routine practice for left-sided breast cancer radiation therapy.

Diagnostic performance and inter-observer variability to differentiate between T1- and T2-stage gallbladder cancers using multi-detector row CT.

PURPOSE: To evaluate the diagnostic performance and inter-observer variability of differentiating T1 and T2 gallbladder (GB) cancers using multi-detector row CT (MDCT). METHODS: This retrospective study included 151 patients with surgically confirmed T1 (n = 49)- or T2 (n = 102)-stage GB cancer who underwent contrast-enhanced MDCT from 2016 to 2020. Five radiologists (two experienced and three less experienced) evaluated the T-stage with a confidence level calculated using a six-point scale. GB cancers were morphologically classified into three types: polypoid, polypoid with wall thickening, and wall thickening. The diagnostic performance of T-staging was assessed using receiver operating characteristic (ROC) curve analysis. Sensitivity, specificity, accuracy, positive predictive value, and negative predictive value were calculated based on a binary scale (T1 = positive). Inter-observer agreement was assessed using Fleiss κ statistics. RESULTS: The area under the receiver operating characteristic (ROC) curve of each reviewer for T-staging ranged from 0.69 to 0.80 (median 0.77). The overall accuracy of the five radiologists was 78% (95% confidence interval [CI] 71-84%). Sensitivity was higher and specificity was lower in experienced radiologists than in less experienced radiologists (P < 0.001). The overall inter-observer agreement was fair (κ = 0.36; 95% CI 0.31, 0.41). The overall accuracy for T-stage was 63% (95% CI 48-76), 78% (95% CI 63-88), and 87% (95% CI 77-93) for polypoid, polypoid with wall thickening, and wall thickening type, respectively. CONCLUSION: The accuracy of MDCT for differentiating T1 and T2 GB cancer is limited, and there is considerable inter-observer variability.

Funneling venoplasty for anomalous graft left hepatic vein in living donor liver transplantation using a split left lateral section graft for an infant patient.

The left lateral section (LLS) can have an unusual variant left hepatic vein (LHV) anatomy. We present a case of customized funneling venoplasty of the graft LHV in a 22-month-old girl diagnosed with ornithine transcarbamylase deficiency undergoing deceased donor liver transplantation (LT) using a split LLS graft. The split LLS graft weighed 350 g, yielding a graft-to-recipient weight ratio of 3.2%. Notably, the graft LHV opening was located at the graft liver cut surface, which was only 1 cm in size and 2 cm away from the cephalad apex of the LLS graft. Since such a variant location of the small LHV opening was unsuitable for direct anastomosis, we performed a funneling venoplasty using an inferior vena cava fragment homograft obtained from the same donor. The graft implantation was performed according to standard procedures of infant split LT. Follow-up imaging studies showed no vascular complications. The patient recovered uneventfully from the LT operation. She had normal blood test findings, including normal ammonia level. She has been doing well for 6 months after the transplantation. In conclusion, our surgical technique using a funneling venoplasty enabled successful reconstruction of the anomalous graft LHV. Our results suggest that individualized reconstruction techniques should be applied to infant patients undergoing LT using a LLS graft with variant types of graft LHV anatomy.

Dextroplantation of a reduced left lateral section graft in an infant undergoing living donor liver transplantation.

Graft size matching is essential for successful liver transplantation in infant recipients. We present our technique of graft dextroplantation used in an infant who underwent living donor liver transplantation (LDLT) using a reduced left lateral section (LLS) graft. The patient was an 11-month-old female infant weighing 7.8 kg with hepatoblastoma. She was partially responsive to systemic chemotherapy. Thus, LDLT was performed to treat the tumor. The living donor was a 34-year-old mother of the patient. After non-anatomical size reduction, the weight of the reduced LLS graft was 235 g, with a graft-to-recipient weight ratio of 3.0%. Recipient hepatectomy was performed according to the standard procedures of pediatric LDLT. At the beginning of graft implantation, the graft was temporarily placed at the abdomen to determine the implantation location. The graft portal vein was anastomosed with an interposed external iliac vein homograft. As the liver graft was not too large and it was partially accommodated in the right subphrenic fossa, thus the abdominal wall wound was primarily closed. The patient recovered uneventfully. An imaging study revealed deep accommodation of the graft within the right subphrenic fossa. The patient has been doing well for six months without any vascular complications. This case suggests that dextroplantation of a reduced LLS graft can be a useful technical option for LDLT in infant patients.

Efficacy and safety of autologous adipose tissue-derived stem cell therapy for children with refractory Crohn's complex fistula: a Phase IV clinical study.

PURPOSE: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn's fistulae. METHODS: Patients with a refractory Crohn's fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn's fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm2). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months. RESULTS: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months. None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration. CONCLUSION: ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn's complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.

Third retransplantation using a whole liver graft for late graft failure from hepatic vein stent stenosis in a pediatric patient who underwent split liver retransplantation.

We present a case of third retransplantation using a whole liver graft in a 13-year-old girl who suffered graft failure and hepatopulmonary syndrome following split liver retransplantation with endovascular stenting of the hepatic and portal veins as an infant. She was diagnosed with biliary atresia-polysplenia syndrome, and thus underwent living donor liver transplantation from her mother at 9 months of age. The first liver graft failed due to stenosis of the portal vein. She underwent the second liver transplantation with a split left lateral section graft. Endovascular stenting was performed to the portal vein stenosis 2 months and hepatic vein stenosis 9 months after transplantation. During the next 9 years, 11 sessions of balloon angioplasty for hepatic vein stent stenosis were performed. Ten years after the second transplantation, she underwent third transplantation using a whole liver graft recovered from a 12-year-old-girl. The double inferior vena cava technique was used for outflow vein reconstruction. The graft portal vein was anastomosed with the stent-containing portal vein stump because it was not possible to remove the stent and the inner diameter of the portal vein stent was large enough. An aorto-hepatic jump graft was used for arterial reconstruction. The patient recovered slowly and is doing well for 6 months posttransplant. In conclusion, because stenting of the hepatic vein or portal vein can induce graft failure leading to late retransplantation, we emphasize secure vascular reconstruction to prevent endovascular stenting during LT in infants.

Effects of sirolimus in the treatment of unresectable infantile hemangioma and vascular malformations in children: A single-center experience.

OBJECTIVE: Recently, sirolimus has emerged as a safe and effective treatment modality for unresectable vascular lesions. In the present study, we investigated the effectiveness and safety of sirolimus from our early experience with patients with unresectable vascular anomalies. METHODS: The medical records and radiologic images of all patients with unresectable vascular anomalies treated with sirolimus at our center from January 2018 to November 2019 were retrospectively reviewed. All patients were administered oral doses of sirolimus 0.8 mg/m2 every 12 hours as the initial dose, followed by maintenance of a target serum concentration (5-10 ng/mL) with therapeutic drug monitoring. RESULTS: Six patients with unresectable vascular anomalies were treated with sirolimus for ≥10 months. Their median age at the initiation of sirolimus treatment was 17 months (range, 8-67 months). The median duration of treatment was 13 months (range, 10-16 months). One patient had a good response, four had an intermediate response, and one had no response to sirolimus therapy. None of the patients had discontinued sirolimus therapy because of adverse effects. CONCLUSIONS: Sirolimus can be used effectively and safely for patients with unresectable vascular anomalies. However, further prospective studies are warranted to evaluate the long-term effects of sirolimus and clarify the indications for early intervention.

The Advantages of Indocyanine Green Fluorescence Imaging in Detecting and Treating Pediatric Hepatoblastoma: A Preliminary Experience.

Background: Currently, indocyanine green (ICG) fluorescence imaging enables radical surgical resection in hepatoblastoma (HB) and has beneficial uses; however, its usage in pediatric patients is still limited. Methods: From 2015 to 2019, 17 hepatoblastoma patients underwent 22 fluorescence-guided surgery using ICG. ICG (0.3 mg/kg) was intravenously injected 24-48 h before the operation. With ICG/NIR camera, intraoperative identification of biological structures and demarcation of mass were conducted. Results: ICG fluorescence-guided surgery was performed for hepatoblastoma in 22 cases: 16, 1, and 2 cases underwent anatomic resection, partial hepatectomy, and liver transplantation, respectively. Six patients accompanied lung metastasis at the time of surgery, and two patients underwent lung surgery using ICG. The median interval from ICG injection to surgery was 38.3 h (range, 20.5-50.3 h). The median tumor size was 36.5 mm (range, 2-132 mm). According to the pathologic finding, the median safety margin was secured for 6 mm (range, 0-11 mm) and there was no residual finding at the liver at the follow-up computed tomography (CT). Conclusions: ICG fluorescence imaging in children with HB was feasible and safe for tumor demarcation and enhancing the accuracy of radical tumor resection.

Pediatric liver transplantation with hyperreduced left lateral segment graft.

BACKGROUNDS/AIMS: To prevent large-for-size graft-related complications in small infant patients, the size of a left lateral segment (LLS) graft can be reduced to be a hyperreduced LLS (HRLLS) graft. METHODS: This study was intended to describe the detailed techniques for harvesting and implanting HRLLS grafts developed in a high-volume liver transplantation (LT) center. RESULTS: The mean recipient age was 4.0±1.7 months (range: 3-6) and body weight was 5.3±1.4 kg (range: 4.1-6.9). Primary diagnoses of the recipients were progressive familial intrahepatic cholestasis in 2 and biliary atresia in 1. The types of LT were living donor LT in 1 and split deceased donor LT in 2. Non-anatomical size reduction was performed to the transected LLS grafts. The mean weight of the HRLLS grafts was 191.7±62.1 g (range: 120-230) and graft-recipient weight ratio was 3.75±1.57% (range: 2.45-5.49). Widening venoplasty was applied to the graft left hepatic vein outflow orifice. Vein homograft interposition was used in a case with portal vein hypoplasia. Types of the abdomen wound closure were one case of primary repair, one of two-staged closure with a mesh, and one of three-staged repair with a silo and a mesh. All three patients recovered uneventfully from the LT operation and are doing well to date for more than 6 years after transplantation. CONCLUSIONS: Making a HRLLS graft through non-anatomical resection during living donor LT and split deceased donor LT can be a useful option for treating small infant patients.

A pipeline for precise and efficient genome editing by sgRNA-Cas9 RNPs in Drosophila.

Genome editing via homology-directed repair (HDR) has made possible precise and deliberate modifications to gene sequences. CRISPR/Cas9-mediated HDR is the simplest means to carry this out. However, technical challenges remain to improve efficiency and broaden applicability to any genetic background of Drosophila melanogaster as well as to other Drosophila species. To address these issues, we developed a two-stage marker-assisted strategy in which embryos are injected with RNPs and pre-screened using T7EI. Using sgRNA in complex with recombinant Cas9 protein, we assayed each sgRNA for genome-cutting efficiency. We then conducted HDR using sgRNAs that efficiently cut target genes and the application of a transformation marker that generates RNAi against eyes absent. This allows for screening based on eye morphology rather than colour. These new tools can be used to make a single change or a series of allelic substitutions in a region of interest, or to create additional genetic tools such as balancer chromosomes.

Graft outflow vein unification venoplasty with superficial left hepatic vein branch in pediatric living donor liver transplantation using a left lateral section graft.

Orifice size of the left hepatic vein trunk (LHV) in left lateral segment (LLS) grafts is often too small to perform direct anastomosis. A small superficial branch of LHV is encountered in approximately 30% of LLS grafts. Unification venoplasty of the LHV trunk and its superficial vein branch makes the orifice size of LLS outflow vein larger than the original size. We present refined surgical techniques for LHV unification venoplasty with a superficial LHV branch. The patient was a 5-month-old 9 kg-weighing girl with biliary atresia. Her general condition deteriorated, but there was low possibility of deceased donor liver allocation, thus living donor liver transplantation was performed using her mother's LLS. The graft hepatic vein was widened through unification venoplasty of LHV and its superficial branch. Recipient hepatic vein orifice was widened through unification of three hepatic veins. The graft and recipient hepatic vein orifices were well matched in size, and they were anastomosed with 5-0 continuous sutures. The portal vein was reconstructed with interposition of cold-preserved external iliac vein homograft. The graft left hepatic artery was reconstructed using the recipient right hepatic artery and hepaticojejunostomy was performed. This patient recovered uneventfully and is doing well for 3 months to date. The unification venoplasty with LHV trunk and its superficial vein branch makes the size of LLS outflow vein definitely larger than the original size, thus it can be a useful technical option to reduce the risk of hepatic vein outflow obstruction in pediatric liver transplantation using a LLS graft.

Living donor liver retransplantation for primary non-function of liver graft following multivisceral transplantation in a pediatric patient.

Multivisceral organ transplantation (MVOT) includes transplantation of three or more abdominal organs, generally including the small bowel, duodenum, stomach, liver, pancreas, and colon. We here presented the detailed procedures of repeat living donor liver transplantation for primary non-function of the first liver graft following MVOT in a pediatric patient. A 6-year-old girl with chronic intestinal pseudo-obstruction underwent MVOT with 5-year-old donor organs. However, the primary non-function of the liver graft developed, and an emergency living donor liver transplantation operation using a left lateral section graft was performed on the third day after MVOT. The donor was the patient's father. Portal flow interruption induced ischemic congestion of the whole small bowel, thus we used a series of porto- caval shunt to reduce the risk of ischemic splanchnic congestion during recipient hepatectomy and graft implantation. Other surgical procedures were the same as the standardized procedures for left liver graft implantation. The graft-recipient weight ratio was 2.15. The patient was managed conservatively for 3 months and discharged in an improved condition at 4 months after MVOT. She finally passed away at 22 months after MVOT. We think that our experience will be helpful for surgeons to cope with portal vein clamping-associated splanchnic congestion during liver transplantation and other abdominal surgeries.

Effect of everolimus rescue therapy for acute cellular rejection following pediatric living donor liver transplantation: Report of one case.

Acute cellular rejection (ACR) after pediatric living donor liver transplantation (LDLT) is often curable with steroid pulse therapy, but a few pediatric patients show steroid-resistant ACR, which is difficult to control. We report the effect of everolimus as a rescue therapy for ACR in a case of pediatric LDLT. The patient was a 11-year-old girl who was admitted due to subacute liver failure of unknown cause. LDLT operation using a modified right liver graft from her mother was performed. The graft-recipient weight ratio was 1.30. The explant liver showed massive hepatic necrosis. The patient recovered uneventfully with immunosuppression using tacrolimus and low-dose steroid. However, at postoperative day (POD) 20, the liver enzyme levels began to increase. The first liver biopsy taken at POD 25 showed moderate ACR with rejection activity index (RAI) score of 7. At that time, steroid pulse therapy was performed, but the patient did not respond and the liver enzyme levels increased further. The second liver biopsy taken at POD 40 showed moderate ACR with RAI score of 7. At this time, everolimus was administered, and soon after that, liver enzyme levels had gradually improved. Currently, the patient is doing well for 44 months to date without any abnormal findings. The maintenance target trough concentrations were tacrolimus 5 ng/ml and everolimus 3 ng/ml. Our case demonstrated the effect of rescue therapy using everolimus for ACR following pediatric LDLT. Further studies are needed to assess the role of everolimus in pediatric liver transplant recipients suffering from ACR.

Living-donor liver transplantation with inferior vena cava replacement in an infant recipient with advanced hepatoblastoma.

Replacement of the inferior vena cava (IVC) after concurrent resection of hepatoblastoma-containing liver and retrohepatic IVC is regarded as a feasible option for pediatric living-donor liver transplantation (LDLT). This technique makes the extent of resection of LDLT comparable to that of deceased-donor liver transplantation (DDLT). We present one case of pediatric LDLT with IVC homograft replacement for advanced hepatoblastoma. The patient was a 10-kg 18-month-old girl suffering from large multiple hepatoblastomas, which were partially regressed by neoadjuvant chemotherapy. Because the tumors had invaded the retrohepatic IVC, there was a high risk of residual tumor cells at the IVC if it was preserved. Thus, we decided to replace the IVC during the LDLT operation. After a cold-stored fresh iliac vein homograft was prepared, we performed LDLT using her mother's left lateral section liver graft. A 4-cm-long common iliac vein homograft was attached to the liver graft at the back table. The left lateral-section graft with IVC attachment was implanted using the standard procedures like those of DDLT. We also did portal vein graft interposition. The patient recovered uneventfully and has been undergoing scheduled adjuvant chemotherapy to date. This is our second case of IVC homograft replacement for pediatric LDLT. In pediatric recipients, various vein homografts, such as iliac vein, IVC, and other large veins, can be used depending on the body size of the recipient and availability of vein homografts.