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Background: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO 2  < 50 mm Hg and those with PaO 2  ≥ 50 mm Hg., Methods: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO 2  ≥ 50 mm Hg, 11 patients, and G2-PaO 2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups., Results: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22)., Conclusion: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O 2 weaning time than those with mild, moderate, or severe HPS., (© 2021 Wiley Periodicals LLC.)

Advancements in critical care management have led to improvement in pediatric LT outcomes. However, there are no specific guidelines for many aspects of immediate post-LT care. This survey examines practice variations in the immediate postoperative care of pediatric LT patients at a large number of active US centers. This study is a cross-sectional survey of medical directors at PALISI-affiliated PICU in the United States. Centers performing pediatric LT were analyzed. Study measures included PICU practices regarding staffing, composition of the multidisciplinary team, early post-LT graft and patient monitoring, and anticoagulation. Of the thirty-five responding centers, twenty-five had a LT program which accounted for one-half of all US pediatric LTs. For analysis, centers were categorized by volume: high (7), medium (11), and low (7). The majority of PICU teams included an intensivist (80%) and hepatologist (84%). High-volume centers were less likely to have 24-hour in-house attending coverage (29%, compared to 64% (medium) and 100% (low)). High-volume centers were most likely to have pre-printed orders, but least likely to have written PICU management protocols. Most centers utilize routine daily liver ultrasound. Routine prophylactic anticoagulation, and the agent of choice, was variable. There is marked inconsistency in post-LT practice across PALISI centers in regards to team composition and immediate post-LT management. A national US consensus for post-LT PICU practices would facilitate outcomes research and would establish a platform for multicenter studies., (© 2021 Wiley Periodicals LLC.)

Background: Sleep-disordered breathing is commonly found in adults with heart failure both before and after HTx. Untreated sleep-disordered breathing post-transplant has been linked to late graft dysfunction, reduced quality of life, and increased morbidity. Sleep-disordered breathing has not been investigated in pediatric HTx recipients., Methods: We conducted retrospective review of patients <21yo who underwent primary HTx at our center from 2009 to 2019 to describe clinical characteristics, cardiac history, and PSG results., Results: One hundred and fifty patients were included; 60% had congenital heart disease, and 40% had cardiomyopathy. Fifty patients had PSG performed at median age of 6.1 years. Forty-one were referred for symptoms of sleep-disordered breathing. Obstructive sleep apnea was diagnosed in 45 patients and central sleep apnea in 3 patients. Of those with first PSG post-transplant (n = 36), median AHI was 9.1/h, and 19 (53%) were diagnosed with moderate or severe sleep apnea. Patients diagnosed with obstructive sleep apnea on PSG had more post-transplant ventilator days (median 3 vs 2 days, P < .05) and longer post-transplant lengths of stay (median 28 vs 22 days, P < .05)., Conclusions: In this single-center cohort of pediatric HTx recipients, sleep-disordered breathing was common and associated with longer peri-transplant respiratory support and length of stay. Given the high incidence of moderate and severe OSA detected in this population, clinicians should regularly screen for SDB and consider PSG testing more frequently in children who have undergone HTx. Further study into the long-term impact of sleep-disordered breathing in pediatric HTx recipients is needed., (© 2020 Wiley Periodicals LLC.)

Pneumocystis jirovecii pneumonia (PJP) prophylaxis after pediatric solid organ transplant (SOT) is routinely recommended, but practice varies. Online survey was sent in 2018 to 707 members of the International Pediatric Transplant Association. A total of 105 responded, representing 47 institutions in 18 countries consisting of transplant physicians (66%), transplant surgeons (19%), nurse practitioners (6%), infectious disease physicians (5%), or pharmacists (4%). PJP prophylaxis was reported by 88%, while 12% did not routinely give prophylaxis. The majority not using PJP prophylaxis performed renal transplants (67%) citing low incidence of PJP (62%). Trimethoprim/sulfamethoxazole was first-line agent (95%). PJP prophylaxis for 4-6 months was the most frequent duration following kidney (48%, 27/56), liver (42%, 13/31), and heart (40%, 10/25) transplant. Abdominal multivisceral providers equally gave 10-12 months (47%) or lifelong (47%); most lung transplant providers gave lifelong prophylaxis (85%). Across all organs, 21% provided lifetime prophylaxis. After completion of prophylaxis, 32% do not restart for any reason; majority of the rest would restart for treatment of acute graft rejection. 83% reported no PJP cases in the prior 12 months; 14% reporting 1-5 infections. Only 3% reported a case of PJP infection on prophylaxis; none in SOT. PJP prophylaxis is routinely provided to pediatric SOT patients though practice and duration vary by center and organ type. Durations of 4-6 months were most common for renal, liver, and heart transplant recipients, while 10-12 months or lifelong prophylaxis were commonly reported for abdominal multivisceral recipients and most lung transplant recipients are given lifelong prophylaxis., (© 2019 Wiley Periodicals, Inc.)

There is growing acceptance of transplantation across a positive crossmatch for highly allosensitized pediatric HT candidates. While survival may be similar to patients transplanted across a negative crossmatch, costs are unknown. Among 60 HT recipients at our center from 5/07 to 6/12, we analyzed hospital charges and length of stay from the day of HT to discharge and through the first year after transplant. Median age at HT was 6.2 years (15 days-20.5 years). Charges in the first year post-HT were greater for crossmatch-positive patients ($907 678 vs $549 754; P = .017), with a trend toward higher charges for the HT hospitalization ($537 640 vs $407 374; P = .07). Plasmapheresis was more common in crossmatch-positive patients during the HT hospitalization (80% vs 4%, P < .001). In the first year after HT, crossmatch-positive patients had a greater number of endomyocardial biopsies (10 vs 7.5, P = .03) and episodes of treated rejection (2 vs 0, P = .004). Pediatric HT across a positive crossmatch is associated with higher first-year costs, including increased use of plasmapheresis and care around an increased number of rejections. These novel data will help inform decision and policymaking regarding care practices for the growing population of highly sensitized pediatric HT candidates., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The "GVM" has emerged as an alternative to traditional individualized appointments in the ambulatory care setting. We hypothesized that group visits could successfully be utilized in a PHtx clinic. Seven patients, ages 1-18 yr old, and their families participated in a total of 11 group visits in lieu of individualized appointments. Patients were divided into two groups based on whether they were greater or less than one yr post-transplant. Patient/provider satisfaction, medication adherence, and content retention were ascertained via questionnaires and free-response tests. Total clinic throughput time, including per-patient clinic utilization time, was compared to historical data. Six of seven patients completed the study with one dropout. Overall satisfaction ratings were 3.98 of 4 with all patients reporting that they would "strongly recommend" group visits to others. Health information retention tests demonstrated improvement between pre- and post-tests in eight of nine (89%) of the group visits. Overall clinic utilization decreased by nearly 50% while providing 70 min of face-to-face time with the provider. Medication adherence neared 100% for all patients. The GVM can be successfully applied to the PHtx population with high patient and provider satisfaction, more face-to-face time, excellent content retention, and greatly improved clinic efficiency., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

EMB is widely utilized for graft surveillance after HTx; however, there is significant variation in the frequency of surveillance EMB use during the first year post-HTx. The aim of this study was to assess changes in the utilization of surveillance EMB over time among member institutions of PHTS. A survey of PHTS centers assessing the frequency of surveillance EMB use during the first year post-HTx was conducted in 2006. The same survey was repeated in 2014 to assess changes in practice over time. The number of EMB in infants ranged from 0 to 9 and in adolescents 0 to 16. The number of EMB decreased or remained unchanged in the majority of centers. Fewer EMB are performed in infants compared to adolescents and this practice did not change over time. There was a significant decrease in surveillance EMB use in adolescents (p = 0.012). International centers perform significantly fewer EMB in adolescents when compared to centers within the United States (p = 0.006). There continues to be significant variation in the utilization of surveillance EMB, with a shift toward less reliance on EMB for adolescents in the current era. Further research is necessary to determine the optimal frequency of invasive monitoring that reduces costs without compromising outcomes., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The ABO incompatibility between donor and recipient is not considered a barrier to successful allogeneic HSCT. Nevertheless, conflicting data still exist about the influence of ABO incompatibility on transplant outcome in pediatric patients with thalassemia. Fifty-one children with beta-thalassemia major who underwent allogeneic HSCT were enrolled this study. Twenty-three of them (45%) received an ABO-incompatible transplant [minor ABO mismatch: six (26%), major ABO mismatch: fourteen (61%), and bidirectional mismatch: three (13%)]. In this study, ABO incompatibility did not significantly impair GVHD, VOD, neutrophil and platelet engraftment, TRM, OS and TFS. Particularly in major and bidirectional ABO-mismatched patients, a delayed erythroid recovery was recorded as compared to the group receiving an ABO-compatible graft (median time, 31 and 38 days vs. 19.5 days; p: 0.02 and p: 0.03). Median time to red cell transfusion independence was significantly longer in major ABO-incompatible patients (median time, 87 days vs. 32 days; p: 0.001). Therefore, whenever feasible, major ABO-mismatched donors should be avoided in HSCT recipients, to prevent delayed erythroid recovery with prolonged RBC transfusion needs and impaired quality of life., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)