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Background: Successful early extubation (EE) after liver transplant (LT) has been shown to reduce intensive care unit (ICU) and hospital length of stay and infectious, vascular, and sedation-related complications in adults. EE may not always be feasible in children, and many may require prolonged mechanical ventilation. Limited data exists regarding the candidacy of EE, risk factors, consequences, and hospital costs of delayed extubation (DE) in pediatric LT., Methods: We conducted a retrospective review to investigate predictive factors and associated costs of EE and DE in infants and children after orthotopic LT at our institution between 2011 and 2021., Results: Of 338 LT (median age 39 months, 54% females), 246 (73%) had EE (within 24 h of LT), while 27% had DE. Age < 1 year (p = 0.0019), diagnosis of biliary atresia (0.02), abnormal pre-LT echocardiogram (0.02), and patients with ongoing hospital admission before LT (0.0001) were independently associated with DE. Hospital costs were significantly (∼3-fold) higher (p < 0.0001) in the DE group. In addition, factors associated with increased total hospital costs were age < 1 year and hospitalization before LT., Conclusion: EE post-LT is feasible and merits a trial. The prevalence of DE though modest is associated with increased resource utilization and hospital costs. Children who can be extubated early and those at risk for DE can be identified pre-operatively for optimal planning and allocation of resources., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction: The American College of Surgeons Children's Surgery Verification (CSV) status recognizes hospitals that deliver high-quality pediatric surgical care. Texas has 5 CSV centers in three cities, which may limit equitable access to care. We explored the characteristics and outcomes of infants admitted in Texas as a function of facility CSV status., Materials and Methods: We retrospectively reviewed a state-wide hospital discharge database (2013-2021). All patients <1 y of age were included. Patients transferred to an outside hospital were excluded to avoid double counting. Descriptive statistics and chi-square analysis were performed., Results: We analyzed 3,617,173 admissions, with 211,278 (6%) treated at CSV centers. CSV admissions were less likely to be inborn (46% versus 93%) and more likely to be transfers (16% versus 1%). CSV centers also had sicker patients (32% versus 13% extreme illness severity) with higher mortality rates (1% versus 0%), longer length of stay (9 ± 22 versus 4 ± 9), and higher operative rates (33% versus 20%). However, mortality was lower at CSV centers when matched for illness severity. Proportionately more patients from rural counties (9% versus 4%) and counties along the United States-Mexico border (13% versus 1%), as well as patients of Hispanic ethnicity (39% versus 33%), were treated at non-CSV centers. Meanwhile, proportionately more African Americans (21% versus 11%) were treated at CSV centers., Conclusions: CSV centers are associated with improved outcomes among patients with high illness severity. Population differences among patients treated at CSV centers compared to non-CSV centers may represent disparities in access to care and warrant further evaluation., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Understanding the economics of pediatric liver transplantation (LT) is central to high-value care initiatives. We examined cost and resource utilization in pediatric LT nationally to identify drivers of cost and hospital factors associated with greater total cost of care. We reviewed 3295 children (<21 y) receiving an LT from 2010 to 2020 in the Pediatric Health Information System to study cost, both per LT and service line, and associated mortality, complications, and resource utilization. To facilitate comparisons, patients were stratified into high-cost, intermediate-cost, or low-cost tertiles based on LT cost. The median cost per LT was $150,836 [IQR $104,481-$250,129], with marked variance in cost within and between hospital tertiles. High-cost hospitals (HCHs) cared for more patients with the highest severity of illness and mortality risk levels (67% and 29%, respectively), compared to intermediate-cost (60%, 21%; p <0.001) and low-cost (51%, 16%; p <0.001) hospitals. Patients at HCHs experienced a higher prevalence of mechanical ventilation, total parental nutrition use, renal comorbidities, and surgical complications than other tertiles. Clinical (27.5%), laboratory (15.1%), and pharmacy (11.9%) service lines contributed most to the total cost. Renal comorbidities ($69,563) and total parental nutrition use ($33,192) were large, independent contributors to total cost, irrespective of the cost tertile ( p <0.001). There exists a significant variation in pediatric LT cost, with HCHs caring for more patients with higher illness acuity and resource needs. Studies are needed to examine drivers of cost and associated outcomes more granularly, with the goal of defining value and standardizing care. Such efforts may uniquely benefit the sicker patients requiring the strategic resources located within HCHs to achieve the best outcomes., (Copyright © 2024 American Association for the Study of Liver Diseases.)

Objective: To determine whether balloon dilation of Eustachian tube (BDET) improves postoperative audiology and quality of life scores in children with chronic Eustachian tube dysfunction., Study Design: Retrospective study., Setting: Tertiary care pediatric center., Methods: Eligible participants were patients 8 years or older, with a history of 2 prior tubes placement. Group 1-patients completed pre-and post-Eustachian Tube Dysfunction Quality of Life Survey (ETDQ-7) survey scores, Group 2-patients had available pre- and postdilation tympanogram data (TD), and Group 3-patients had both ETDQ-7 survey and TD. The average time for the first and subsequent follow-ups was 3.8 and 12.9 months, respectively., Results: A total of 43 patients (85 ears) underwent BDET. The mean age was 13.3 years (8-18 years). Twenty-four patients were male (55.8%) and over 80% were Caucasian. The average mean ETDQ-7 score before and after dilation was 3.9 and 2.5, respectively. Ninety-three percent experienced improvement of their postoperative ETDQ-7 scores and 53% had normal postdilation ETDQ-7 score (P < .0001). Thirty-seven ears in Group 2 (60.7%) had improvement in postdilation TD. A greater proportion of ears showed improvement of 62.3% with a 95% confidence interval (CI) [50.1%-74.5%] compared to 37.7% without improvement, 95% CI [25.5%-49.87%]. Ears with type A or B TD were more likely to show improvement than ears with type C, perforated, or with tubes (P < .0001). Eighteen out of 30 ears in Group 3 (60%) experienced an improvement in both ETDQ-7 and tympanogram., Conclusion: BDET is a safe, efficacious alternative to tubes in selected pediatric patients., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Background: Umbilical cord gases are often used to assess the impact of labor and delivery on the fetus. However, no large series exists that reflects contemporary obstetrical practice or that analyzed blood gas ranges by route of delivery. Baseline, prelabor acid-base status in the human fetus is also poorly defined, rendering the assessment of blood gas changes during labor difficult., Objective: This study aimed to define normal umbilical cord gas and lactate values, stratified by mode of delivery, in a large contemporary series in which universal umbilical cord gas evaluation was dictated by protocol., Study Design: This was a retrospective cohort study. We analyzed the umbilical cord gas and lactate data of an unselected population of infants born between March 2012 and April 2022 at a large teaching hospital. These values were then analyzed by mode of delivery and, for cesarean deliveries, by indication for cesarean delivery and type of anesthesia. Umbilical cord gas values from infants delivered by elective cesarean delivey under general anesthesia without labor were considered representative of baseline, prelabor values., Results: Data were available for 45,475 infants. The median arterial pH values and interquartile ranges for vaginal births, elective cesarean deliveries without labor, and cesarean deliveries performed for fetal heart rate concerns were 7.27 (0.09), 7.27 (0.06), and 7.25 (0.09), respectively. Arterial lactate values for these same 3 groups were 4.1 (2.5), 2.5 (1.2), and 4.0 (2.8) mmoles/L, respectively. Because of the very large sample size, most comparisons yielded differences that were statistically significant, but clinically irrelevant. Of all the infants, 14% had an arterial pH <7.20; a pH value of 7.1 represents 2 standard deviations from the mean., Conclusion: This large, population-based study of umbilical cord gas and lactate levels in an unselected population, stratified by delivery mode, represents a previously unavailable benchmark for the evaluation of umbilical cord gases. Arterial umbilical cord pH values for infants delivered by elective caesarean delivery without labor (median pH 7.28) reflect a lower prelabor fetal pH baseline than previously assumed. This finding, coupled with our determination that a 2 standard deviation below normal pH limit of 7.1, instead of the historic arbitrary pH of 7.2 threshold, helps to explain the poor positive predictive value of electronic fetal heart rate monitoring, a test designed to detect arterial pH levels that have fallen from an assumed baseline near pH 7.4 to an assumed potentially injurious pH level of <7.2. Uncomplicated labor, even when prolonged, does not generally lead to a clinically significant cumulative hypoxic stress to the human fetus. These findings, along with our determination that there is no difference in the acid-base status among infants delivered by cesarean delivery for fetal heart rate concerns, help to explain the failure of current approaches in labor and delivery management to reduce the rates of neonatal hypoxic-ischemic encephalopathy and cerebral palsy, conditions that almost always reflect developmental events rather than the effects of labor on the fetus., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: Microtia is a congenital condition known to be associated with vertebral anomalies and congenital syndromes, most prominently hemifacial microsomia. There is controversy, however, on whether to screen with spinal imaging. Additionally, microtia ear reconstruction utilizes rib harvesting that could potentially worsen pre-existing vertebral and rib anomalies, specifically scoliosis. We report on the prevalence and characteristics of vertebral anomalies among microtia patients at a tertiary pediatric center., Study Design: Retrospective case review with literature review., Setting: Tertiary pediatric referral center., Methods: A review of 425 children with microtia was conducted, characterized as either syndromic or nonsyndromic. Data included demographics, spinal imaging performed, indications, anomalies detected, and microtia repair., Results: Among 425 microtia patients, 24.5% were syndromic with an average age of 9.7 years. Only 18.4% of all patients had spinal imaging performed (50% syndromic vs 8.1% nonsyndromic). Overall, 10.6% had a vertebral anomaly with a 57.7% detection rate (67.3% syndromic vs 38.5% nonsyndromic). The most common anomaly was scoliosis, with a prevalence of 7.8%. Fusion defects and rib deformities were the next most prominent. Microtia repair, most commonly with an autologous rib graft, was performed in 21.6% of the cohort. However, only 19.2% had spinal imaging and 16.7% with a vertebral anomaly., Conclusion: Children with microtia are at a greater risk of vertebral abnormalities. Scoliosis prevalence in isolated microtia is comparable to the general population (2%-3%) but greatly increased with genetic syndromes. Screening for vertebral anomalies should be considered when planning microtia reconstructions, especially in the syndromic population., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Background: Despite clinical progress over time, a shortage of suitable donor organs continues to limit solid organ transplantation around the world. Lungs are the organs most likely to be assessed as unsuitable during donor management among all transplantable organs. Although the number of lung transplants performed in children is limited, death on the wait list remains a barrier to transplant success for many potential transplant candidates. Optimizing organ donor management can yield additional organs for transplant candidates., Methodology: We accessed the Donor Management Goal (DMG) Registry to evaluate the efficiency and efficacy of donor management in the procurement of lungs for transplantation. Further, we stratified donors by age and compared pediatric age cohorts to adult cohorts with respect to attainment of donor management target goals and successful pathway to transplantation. We utilized recipient data from the Organ Procurement Transplantation Network (OPTN) to put this data into context. The DMG bundle consists of nine physiologic parameters chosen as end-points guiding donor management for potential organ donors. The number of parameters fulfilled has been regarded as an indication of efficacy of donor management., Results: We noted a markedly lower number of organ donors in the pediatric age group compared to adults. On the other hand, the number of donors greatly exceeds the number of infants, children and adolescents who undergo lung transplantation. Organs transplanted per donor peaks in the adolescent age group. At initial donor referral, DMG bundle attainment is lower in all age groups and improves during donor management. With respect to oxygenation, there is less overall improvement in younger donors compared to older donors during donor management. When donors who yield lungs for transplantation are compared to those whose lungs were not transplanted, oxygenation improved more substantially during donor management. Furthermore, improved oxygenation correlated with the total number of organs transplanted per donor., Conclusions: In the face of continued wait list mortality on the pediatric lung transplant wait list, the number of young donors may not be a limiting factor. We believe that this dataset provides evidence that management of young pediatric donors is not as consistent or efficient as the management of older donors, potentially limiting the number of life-saving organs for pediatric lung transplant candidates. Across all ages, optimizing donor lung management may increase the potential to transplant multiple other organs., Competing Interests: Disclosure statement The authors of this publication have no conflicts of interest or financial considerations to disclose. Darren Malinoski, M.D. has provided assistance and advice with respect to the project, the history of the DMG formation and maturation, and the manuscript. Alex Garza, Project Manager in the Research Department of the United Network for Organ Sharing, oversaw the process of contracting with the DMG advisory group and UNOS. Their help is gratefully acknowledged., (Copyright © 2023 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Kidney transplantation in small children is technically challenging. Consideration of whether to use intraperitoneal versus extraperitoneal placement of the graft depends on patient size, clinical history, anatomy, and surgical preference. We report a large single-center experience of intraperitoneal kidney transplantation and their outcomes., Methods: We conducted a retrospective review of pediatric patients who underwent kidney transplantation from April 2011 to March 2018 at a single large volume center. We identified those with intraperitoneal placement and assessed their outcomes, including graft and patient survival, rejection episodes, and surgical or non-surgical complications., Results: Forty-six of 168 pediatric kidney transplants (27%) were placed intraperitoneally in children mean age 5.5 ± 2.3 years (range 1.6-10 years) with median body weight 18.2 ± 5 kg (range 11.4-28.6 kg) during the study period. Two patients (4%) had vascular complications; 10 (22%) had urologic complications requiring intervention; all retained graft function. Thirteen patients (28%) had prolonged post-operative ileus. Eight (17%) patients had rejection episodes ≤6 months post-transplant. Only one case resulted in graft loss and was associated with recurrent focal segmental glomerular sclerosis (FSGS). Two patients (4%) had chronic rejection and subsequent graft loss by 5-year follow-up. At 7-year follow-up, graft survival was 93% and patient survival was 98%., Conclusions: The intraperitoneal approach offers access to the great vessels, which allows greater inflow and outflow and more abdominal capacity for an adult donor kidney, which is beneficial in very small patients. Risk of graft failure and surgical complications were not increased when compared to other published data on pediatric kidney transplants., (© 2022 Wiley Periodicals LLC.)

Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those in the pubertal or postpubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the United States., Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years and compared them to our older CF lung transplant recipients and our larger CF Center population., Results: The transplant population was demographically distinct from our CF center in terms of ethnicity, country of origin, and insurance status. Other notable findings were a high prevalence of methicillin-resistant Staphylococcus aureus, a high prevalence of CF-related diabetes mellitus, and a high prevalence of consolidated lobar or whole lung disease. Posttransplant outcomes were comparable to those older than 10 years of age in our center until 5 years after transplant after which the younger cohort showed a superior enduring survival., Conclusions: In an era of increasingly effective medications modifying the natural history of CF, identification of risk factors for early severe lung disease in CF remains relevant to permit interventions to prevent or postpone the time of future lung transplantation., (© 2022 Wiley Periodicals LLC.)

Cirrhotic cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely because of a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective two-dimensional echocardiographic (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CCM with liver transplant (LT) outcomes in this population. Using receiver operating characteristic (ROC) curve analysis, optimal cut-off values for left ventricular (LV) geometrical parameters that were highly sensitive and specific for the primary outcomes: A composite of serious adverse events (CSAE) and peritransplant death were determined. These results were used to propose a working definition for BA-CCM: (1) LV mass index (LVMI) ≥95 g/m 2.7 or (2) relative wall thickness of LV ≥0.42. Applying these criteria, BA-CCM was found in 34 of 69 (49%) patients with BA listed for LT and was associated with increased multiorgan dysfunction, mechanical and vasopressor support, and longer intensive care unit (ICU) and hospital stays. BA-CCM was present in all 4 waitlist deaths, 7 posttransplant deaths, and 20 patients with a CSAE (P < 0.01). On multivariable regression analysis, BA-CCM remained independently associated with both death and a CSAE (P < 0.01). Utilizing ROC analysis, LVMI was found to be a stronger predictor for adverse outcomes compared with current well-established markers, including Pediatric End-Stage Liver Disease (PELD) score. Conclusion: BA-CCM is highly sensitive and specific for morbidity and mortality in children with BA listed for LT. 2DE screening for BA-CCM may provide pertinent clinical information for prioritization and optimal peritransplant management of these children., (© 2018 by the American Association for the Study of Liver Diseases.)

Background: Pre-transplant nutrition is a key driver of outcomes following liver transplantation in children. Patients with biliary atresia (BA) may have difficulty achieving satisfactory weight gain with enteral nutrition alone, and parenteral nutrition (PN) may be indicated. While PN has been shown to improve anthropometric parameters of children with BA listed for liver transplantation, less is known about the risks, particularly infectious, associated with this therapy among this specific group of patients., Aim: To describe the incidence, microbiology, and risk factors of central line-associated bloodstream infection (CLABSI) among children with BA listed for liver transplantation., Methods: Retrospective review of children aged ≤ 2-years of age with BA who were listed for primary liver transplantation at Texas Children's Hospital from 2008 through 2015 ( n = 96). Patients with a central line for administration of PN ( n = 63) were identified and details of each CLABSI event were abstracted. We compared the group of patients who experienced CLABSI to the group who did not, to determine whether demographic, clinical, or laboratory factors correlated with development of CLABSI., Results: Nineteen of 63 patients (30%, 95%CI: 19, 43) experienced 29 episodes of CLABSI during 4800 line days (6.04 CLABSI per 1000 line days). CLABSI was predominantly associated with Gram-negative organisms (14/29 episodes, 48%) including Klebsiella spp., Enterobacter spp., and Escherichia coli . The sole polymicrobial infection grew Enterobacter cloacae and Klebsiella pneumoniae . Gram-positive organisms (all Staphylococcus spp.) and fungus (all Candida spp.) comprised 9/29 (31%) and 6/29 (21%) episodes, respectively. No demographic, clinical, or laboratory factors were significantly associated with an increased risk for the first CLABSI event in Cox proportional hazards regression analysis., Conclusion: There is substantial risk for CLABSI among children with BA listed for liver transplantation. No clinical, demographic, or laboratory factor we tested emerged as an independent predictor of CLABSI. While our data did not show an impact of CLABSI on the short-term clinical outcome, it would seem prudent to implement CLABSI reduction strategies in this population to the extent that each CLABSI event represents potentially preventable hospitalization, unnecessary healthcare dollar expenditures, and may exact an opportunity cost, in terms of missed allograft offers., Competing Interests: Conflict-of-interest statement: All authors declare no conflicts-of-interest related to this article.

The incidence of death by CA after PHTx is unknown. We aimed to determine the incidence and factors for fatal CA after PHTx, and whether a PM affects survival. Retrospective cohort study utilizing the United Network of Organ Sharing registry of patients transplanted ≤18 years. Multivariable analyses in hazard-function domain and Kaplan-Meier analyses were performed for an outcome of death due to CA. There were 7719 PHTx patients queried. CA was the reported cause of death in 11%. Age ≥13 years at time of transplant, presence of a PM, and depressed EF were identified as significant factors for fatal CA. Death due to CA beyond 10 years post-transplant was associated with depressed EF, CAV, and presence of a PM. Kaplan-Meier analysis demonstrated higher likelihood of fatal CA in patients with CAV and in those with a PM vs those without. In total, 15% of patients with a PM died from CA. CA is a relatively common cause of death after PHTx. The benefit of a PM remains unclear, but its presence does not confer complete protection. Patients with associated factors warrant vigilant surveillance and consideration for retransplantation., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We evaluated patient sera for flow PRA, FCXM, and end-point donor-antigen titer, and we correlated the results with graft survival. You cannot accurately predict a positive or negative FCXM result-not even when the sera have donor-specific antigens-unless you actually perform a crossmatch. Using fluorescence intensity as a surrogate for antibody concentration does not correlate quantitatively with the occurrence of a positive or negative crossmatch. Therefore, it is imperative to give each recipient a chance at being offered a donor organ by performance of a real-time crossmatch and not rely on a virtual evaluation.

The Meyer-Probst questionnaire on typical encephalopathic behavior in children with early brain damage was used with 32 children attending special schools, at intervals during the first four years of school. Over a long-term test, the questionnaire has proved to be a suitable method for the registration of educational and physiotherapeutic results.