Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis.

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long-term outcomes, highlighting their individual differences and management strategies. METHODS: Prospectively collected data over a decade (2011-2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1-4) were compared with regard to early and long-term outcomes including attainment of catch-up growth. RESULTS: Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post-LT chronic diarrhea was observed in 6 (46.1%) children with PFIC-I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1-year follow-up, their graft steatosis resolved and they attained catch-up growth. Catch-up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p < .001). Overall 1- and 5-year patient survival of the four PFIC groups (1-4) were 69.2%, 81.8%, 96.8%, 100% & 69.2%, 81.8%, 96.8%, 100%, respectively. CONCLUSION: Ours is the largest to-date series of LT for PFIC illustrating their short- and long-term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch-up growth, graft steatosis, and post-LT diarrhea, which can be optimized by the addition of TIBD during LT.

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.

Presepsin as a promising biomarker for early detection of post-operative infection in children.

Background: Post-operative systemic inflammation response syndrome (SIRS) is an event that results from surgical trauma, white blood cells contact activation, and intra-surgical bacterial translocation, which is difficult to distinguish from sepsis. Presepsin is a novel biomarker that is increased since the early stages of bacterial infection and can be used to confirm the diagnosis of post-operative infectious complications. This study aimed to investigate the diagnostic performance of presepsin for post-operative infectious complications compared to other well-known biomarkers. Method: This cross-sectional study included 100 post-operative patients admitted to Cipto Mangunkusumo National Hospital and Bunda Hospital in Jakarta, Indonesia. The objective was to identify the optimal cutoff and trend of plasma presepsin concentration on the first and third day after surgery and to compare them with other biomarkers. Result: Plasma presepsin level was higher in the infection group compared to the non-infection group (median 806.5 pg/ml vs. 717 pg/ml and 980 pg/ml vs. 516 pg/ml on the first and third day, respectively). Presepsin levels tended to increase on the third post-operative day (median + 252 pg/ml) in children with infection. The opposite trend was observed in the non-infection group from the first to the third day (median -222.5 pg/ml). Presepsin delta, a three-day difference between the first and third post-operative day, had the best diagnostic performance compared to other biomarkers (Area Under the Curve 0.825). The optimal cutoff for presepsin delta to diagnose post-operative infection was +90.5 pg/ml. Conclusion: Serial assessments of presepsin levels on the first and third days post-surgery and their trends are helpful diagnostic markers for clinicians to detect post-operative infectious complications in children.

Emergency separation of conjoined twins in a tertiary hospital in Indonesia: three case reports.

BACKGROUND: Emergency separation of conjoined twins is performed when one twin is already dead or dying and threatens the survival of the other. The particular decision to perform an emergency separation of conjoined twins provides an ethical dilemma that needs special attention. Adding to the complexity of surgical and postsurgical management in emergency separation, ethical and sociocultural aspects further complicate decision-making. CASE PRESENTATION: From 1987 to 2022, 18 conjoined twin separations were performed in our centre. This paper describes three conjoined twin emergency separations. In the first case of thoracoomphalopagus babies at nine days of age, one baby was diagnosed with necrotizing enterocolitis with frequent desaturation and seizures, and the other baby was healthy. Emergency separation was performed on the twelfth day of age; unfortunately, neither baby survived the surgery. In the second case, emergency separation was performed on the 110th day of life due to sepsis in one baby. The nonseptic twin passed away six hours after surgery, while the septic twin died 12 days after surgery due to wound dehiscence and abdominal sepsis. The third case was of an omphalopagus conjoined twin with a parasitic twin. The healthy baby was deemed nonviable but found to be healthy upon birth. Immediate emergency separation was performed at 2 h of age. The living baby survived the surgery but passed away two months later. CONCLUSIONS: When separation is deemed necessary to save one twin, it becomes difficult to apply standard ethical medical reasoning. The decision to separate results in most cases in very high-risk surgeries with poor outcomes during surgery and postsurgery. Compounded by the complexity of the case, sociocultural and religious aspects further add to the dynamics of decision-making. A multidisciplinary team must work together with a health ethics committee and navigate through this ethical conundrum with the patient and family at its decision-making centre to decide on the best plan of care.