Hyperammonemia in ornithine transcarbamylase-deficient recipients following living donor liver transplantation from heterozygous carrier donors.

Ornithine transcarbamylase deficiency (OTCD) is a urea cycle disorder of X-linked inheritance, affecting the detoxification of excess nitrogen and leading to hyperammonemia (hyper-NH3 ). Living donor liver transplantation (LDLT) has been applied for the treatment of OTCD. This case series retrospectively reviewed two OTCD patients who experienced hyper-NH3 following LDLT. The first case was a 5-year-old girl who had onset of OTCD at 2 years of age. Ornithine transcarbamylase (OTC) enzyme activity was 62% for the donor and 15% for the recipient. The patient suffered from recurrence of hyper-NH3 within 2 months following LDLT. The second case was a 5-year-old girl who had onset of OTCD at 3 years of age. OTC enzyme activity was 42.6% for the donor and 9.7% for the recipient. The patient suffered hyper-NH3 for 12 days starting on the date of surgery. Both of the patients transiently required continuous veno-venous hemodialysis; however, they are currently doing well without intensive medical treatment. The use of asymptomatic OTCD heterozygous donors in LDLT has been accepted with careful examination. However, an OTCD heterozygous carrier donor should be avoided if there is another donor candidate, due to the potentially fatal condition of hyper-NH3 following LDLT.

Chemotherapy-induced B-cell depletion in hepatoblastoma patients undergoing ABO-incompatible living donor liver transplantation.

LT from ABO-I donors requires preconditioning regimens to prevent postoperative catastrophic AMR. NAC for HBL is known to cause myelosuppression leading to a reduction in the number and function of lymphocytes. We investigated this chemotherapy-induced myelosuppression in HBL patients listed for LT from ABO-I donors with reference to the kinetics of B, T cells, and anti-ABO blood type isoagglutinin titers. Between 2005 and 2015, of the 319 patients who underwent LDLT at our institute, 12 were indicated for unresectable HBL. Three patients with unresectable HBL who underwent LDLT from ABO-I donors are included in this study. Immunosuppression consisted of a standard regime of tacrolimus and low-dose steroids as in ABO compatible/identical LDLT. No additional preoperative therapies for B-cell depletion were used. Absolute lymphocyte counts, lymphocyte subsets (including CD20+ B cells, CD3+CD4+ T cells and CD3+CD8+ T cells), and anti-ABO blood type isoagglutinin titers were measured before LDLT and postoperatively. The median age at diagnosis was 19 months (range, 3-31 months). The median follow-up was seven months (range, 6-15 months). The median interval from the last NAC to LDLT was 33 days (range, 25-52 days). The median interval from LDLT to adjuvant chemotherapy was 28 days (range, 22-36 days). The counts of CD20+ B cells before LDLT were depleted to median 5 cells/mm(3) (range, 0-6 cells/mm(3)). There was a transient rebound in the CD20+ B cell counts on day seven (maximum of 82 cells/mm(3)) followed by a decline starting at 14 days after LDLT that was sustained for the duration of adjuvant chemotherapy. Anti-ABO blood type isoagglutinin titers were lowered to between 1:1 and 1:16 before LDLT and remained low for the duration of follow-up in this study. All of the three patients remained in good health without either acute cellular or AMR after LDLT. The B-cell depletion that occurs after cisplatin-based chemotherapy for HBL may help accomplish safe ABO-I LDLT in children without the use of additional conditioning regimens for prevention of AMR.

Living donor liver transplantation on pediatric patient with Budd-Chiari syndrome: Case report and literature review.

INTRODUCTION: The management of Budd-Chiari syndrome is determined on the basis of the severity of the disease. There are no standard guidelines regarding the management of Budd-Chiari syndrome in children, particularly in cases of liver transplantation. Therefore, we present a case of a pediatric patient with Budd-Chiari syndrome treated with liver transplantation. CASE PRESENTATION: A female patient aged 1 year and 8 months presented to the hospital with an enlarged stomach in the last 1.5 months before admission. The patient was moderately ill, malnourished, and jaundiced. Liver biopsy revealed fibrosis in the portal area and confluent necrosis caused by vascular disorders. Magnetic resonance imaging (MRI) revealed a nutmeg liver and ascites due to the stenosis of the inferior vena cava at the level of the liver and the middle and left hepatic veins. The patient underwent living-donor liver transplantation. The occlusion of the proximal right hepatic vein due to the presence of a membrane was identified as the cause of Budd-Chiari syndrome in this case. Postoperatively, the patient's condition was stable and there was no sepsis or any other complications. CLINICAL DISCUSSION: Prothrombotic factors are often the underlying cause of more than 80 % of Budd-Chiari syndrome cases. Protein C deficiency is suspected to be a prothrombotic factor that triggers Budd-Chiari syndrome in patients. Liver transplantation is the treatment of choice for patients with Budd-Chiari syndrome who were not treated with anticoagulation therapy, angioplasty, and TIPS. CONCLUSION: Living-donor liver transplantation has a good outcome in the management of pediatric patients with Budd-Chiari syndrome.

The Effect of Intrahepatic and Intrasplenic Administration of Mesenchymal Stem Cell to Liver Function and Degree of Liver Fibrosis in Common Bile Duct Ligation Model in Rabbit.

BACKGROUND: Mesenchymal stem cells (MSC) is a promising alternative method in liver cirrhosis management. Several administration routes of MSC have been studied, but few studies compared one to another. The purpose of this study is to compare the intrahepatic and intrasplenic route of MSC administration in terms of liver function and degree of liver fibrosis in the bile duct ligation model in rabbits. METHOD: Experimental study was conducted using rabbits (Oryctolagus cuniculus) model undergoing bile duct ligation (BDL). The subjects were randomized into 4 groups: sham surgery; bile duct ligation; bile duct ligation followed by intrahepatic route of MSC (BDL + IH MSC), and bile duct ligation followed by intrasplenic route of MSC (BDL + IS MSC). Umbilical cord mesenchymal stem cell (UC MSC) was administered on the fifth day after bile duct ligation, and the subjects were observed until the fourteenth day after bile duct ligation. The liver function was evaluated with alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total and direct bilirubin. The degree of fibrosis was evaluated with Laennec score, fibrosis area fraction, the number of viable and necrosis hepatocytes, and the number of hepatic progenitor cells. RESULT: The subjects were randomized into 4 groups: 2 in sham surgery group, and 7 in each of the following groups: BDL, BDL + IH MSC and BDL + IS MSC groups. The mortality rate in BDL group was 57.1 %, while mortality in BDL + IH MSC and BDL + IS MSC groups were 14.3 % and 28.6 % respectively. No significant difference was found regarding liver function in each group, such as AST, ALT, total, and direct bilirubin. Histopathology examination in almost every subject undergone bile duct ligation (regardless of MSC administration) showed degree of fibrosis of Laennec 4B. Fibrosis area fraction, the number of viable and necrotic hepatocytes, and progenitor cells were analyzed; no significant difference was found between BDL + IH MSC and BDL + IS MSC groups, but the groups administered with MSC showed a larger number of viable hepatocytes compared to BDL group. No difference was found between BDL + IH MSC and BDL + IS MSC groups in terms of liver function and histologic parameters. CONCLUSION: Administration of MSC increases the number of viable hepatocytes, but no difference was found in terms of liver function and degree of liver fibrosis between the intrahepatic route and intrasplenic route of administration. TYPE OF STUDY: Animal Research, Randomized Controlled Study. LEVEL OF EVIDENCE: Level I? (animal research is not indicated in the levels of evidence table in the journal website).

Management of prenatally diagnosed multiple giant cystic lymphangioma with combination of surgery and intralesional bleomycin injection: A case report.

INTRODUCTION AND IMPORTANCE: Giant cystic lymphangioma was a congenital lymphatic system malformation. Although it is benign, it can cause some complications requiring prompt treatment. The selection of therapy was challenging because none of them is perfect. In this report, we presented a case of multiple giant cystic lymphangiomas in an infant treated with stepwise surgeries combined with intralesional bleomycin injection. CASE PRESENTATION: A two-day-old infant presented with multiple, soft, skin-colored tumors on the right side of the neck, thoracoabdominal region, axilla, and upper extremity which on pre-natal MRI appeared as heterogeneous masses with septate cystic components infiltrating upper chest cavity and compressing subclavian and carotid arteries. The patient was treated successfully with stepwise surgeries followed by intralesional bleomycin injections. CLINICAL DISCUSSION: Cystic lymphangioma is a benign malformation of lymphatic vessels with the neck and axillar region being the most common site. There are several options for treatment modalities. Surgery has been the mainstay of treatment, but other treatment modes are getting more popular. A combination of surgery and sclerotherapy has shown satisfying results. CONCLUSION: In large and infiltrative lymphangioma cases, staged surgery followed by intralesional bleomycin injection can be a treatment approach to minimize morbidity and mortality.

Early relaparotomy following pediatric living-donor liver transplantation: experience in an Indonesian national referral hospital.

Background: Living donor liver transplantation (LDLT) remains the only curative treatment for children with end-stage liver disease; however, complications of the procedure are associated with indications for early relaparotomy. Several risk factors associated with early relaparotomy after liver transplantation include pediatric end-stage liver disease (PELD) score, warm ischemia time (WIT), and cold ischemia time (CIT). Our study investigated the incidence and indications of early relaparotomy in postoperative pediatric LDLT recipients and compared the outcomes with patients who did not require relaparotomy. Methods: A retrospective cohort study of pediatric LDLT recipients from Cipto Mangunkusumo Hospital, Jakarta, Indonesia, was collected from 2010 to August 2022. Indications for early relaparotomy were investigated. Factors analyzed in the early relaparotomy group compared with the nonrelaparotomy group included intraoperative blood loss, surgery duration, CIT, WIT, and PELD score. Results: The highest indication for early relaparotomy was biliary leakage. Most patients who underwent early relaparotomy only had one incidence of relaparotomy (60%). The surgery duration in subjects with early relaparotomy was longer by a median of 3 hours compared with those without early relaparotomy (p=0.289). Intraoperative blood loss was greater in early relaparotomy subjects than in subjects without early relaparotomy (95 vs 77 mL/kg, p=0.552). Other factors, such as PELD score, CIT, and WIT, also showed no significant difference between the two groups. Conclusion: Biliary leakage was the most common indication for early relaparotomy in our center. There were no preoperative or intraoperative factors that significantly influenced the incidence of early relaparotomy due to the limited sample size and the early advancement of our liver transplant center.

Vaginal reconstruction using ileum segment and posterior sagittal anorectoplasty in a patient with Mayer-Rokitansky-Küster-Hauser syndrome with anorectal malformations: A case report.

Vaginal agenesis with anorectal malformations is a complex pediatric condition that adversely affects various physiological processes in the body. It may cause disturbances in defecation and urination, abnormalities in the urinary and gastrointestinal tract, dysfunction of the genital and reproductive organs, and sexual function disorders. The complexity in the surgical management of vaginal agenesis includes the selection of a functional reconstruction technique for anal and vaginal formation, timing of the reconstruction, and management of complications in the associated organ system. Herein, we describe a patient with Mayer-Rokitansky-Küster-Hauser syndrome accompanied by a rectovesical fistula. Other abnormalities, such as microcephaly, polydactyly, long urethral abnormalities resembling the male urethra, and complications in the kidney and urinary tract, were observed in the patient. The associated complications included recurrent urinary tract infections, urinary overflow incontinence, vesicoureteric reflux, hydroureter, and left renal hydronephrosis. The patient underwent posterior sagittal anorectoplasty surgery and vaginal reconstruction. The long-term vaginal physiological development of patients with this condition remains unknown.

Omphalopagus conjoined twins separation during coronavirus disease-19 pandemic era: A case report.

INTRODUCTION AND IMPORTANCE: Conjoined twin is a rare congenital anomaly characterized by a fusion of certain anatomical structures. Coronavirus-19 (COVID-19) is a new emerging infectious respiratory disease affecting worldwide and potentially leads to acute respiratory distress (ARDS) in children. COVID-19 has reconstructed the healthcare system, including surgical care and decision-making. CASE PRESENTATION: Herein we describe a surgical separation of 2.5 months old omphalopagus conjoined twins, with one of them (Baby A) presenting COVID-19-associated respiratory distress, as well as the challenges faced during the preparation and the execution of the complex surgical procedure. CLINICAL DISCUSSION: Baby A underwent antiviral therapy, oxygen supplementation, and ventilation in the ICU, while baby B remained stable and confirmed negative for SARS-CoV-2. The separation surgery was conducted after baby A had become clinically stable. Defect closure and reconstruction were accomplished. At one week follow-up, Baby A died of lung infection, while baby B remained well after one year. CONCLUSION: The complexity of surgical separation requires careful planning by a multidisciplinary team. Surgical separation of conjoined twins during the pandemic era has not been reported much in the literature, more reports are required to provide further insight.