Domino Liver Transplantation: Anesthetic Considerations for a Patient With Maple Syrup Urine Disease and Type I Diabetes Mellitus.

In this report, we describe the case of a patient with concomitant maple syrup urine disease (MSUD) and type I diabetes mellitus (T1DM) who underwent domino liver transplantation (DLT) , and the associated perioperative management. To the best of our knowledge, a DLT in an adult with both MSUD and T1DM has not been previously reported in the literature. Intensive care admission with multidisciplinary oversight is necessary for metabolic preconditioning prior to surgery. The complex interplay between these two disease processes presented with grossly elevated baseline insulin requirements and refractory intraoperative hyperglycemia. Following the successful procedure, the patient maintained excellent glycemic control on a normal diet. Four months post transplant, the patient presented with mild to moderate cellular graft rejection.

Risk Factors Associated With Development of Multiple-Organ Dysfunction Syndrome After Pediatric Drowning.

OBJECTIVE: This study aimed to describe a single-center experience of pediatric drowning and to investigate risk factors associated with the development of pediatric multiple-organ dysfunction syndrome (MODS) after drowning events. METHODS: A single-center retrospective case-control study was performed at a tertiary children's hospital examining patients aged 1 month to 25 years who were admitted to the pediatric intensive care unit after a drowning event. The study period was June 2016 to June 2021. Patients who developed MODS at day 1 of intensive care admission were compared with those who did not. RESULTS: A total of 48 patients with a median age of 2.3 years were included. Twenty-nine (60%) had MODS at 24 hours. Those with MODS at 24 hours were more likely to require cardiopulmonary resuscitation (CPR), required longer duration of CPR, and had longer submersion times; otherwise, there were no differences in baseline characteristics. Those who developed MODS at 24 hours had longer lengths of stays, longer lengths of mechanical ventilation, and higher mortality. Multiple admission parameters were evaluated based on MODS-free survival at 24 hours. On univariable analysis, patients without MODS-free survival at 24 hours had higher rates of CPR, higher blood glucose on admission, higher illness severity scores, higher lactates, and lower Glasgow Coma Scale scores. A multivariable model was constructed using risk factors at presentation that were significant on univariable analysis; blood glucose greater than 200 mg/dL was associated with decreased odds of MODS-free survival at 24 hours after controlling for CPR administration of greater than 5 minutes and body temperature. CONCLUSIONS: Development of MODS in pediatric drowning is associated with worse patient outcomes. Hyperglycemia was identified as a potentially modifiable risk factor for the development of MODS at 24 hours and could serve as a useful prognostic parameter in this unique patient population.