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1.
World J Surg ; 48(4): 967-977, 2024 04.
Artículo en Inglés | MEDLINE | ID: mdl-38491818

RESUMEN

BACKGROUND: Choledochal cysts are rare congenital anomalies of the biliary tree that may lead to obstruction, chronic inflammation, infection, and malignancy. There is wide variation in the timing of resection, operative approach, and reconstructive techniques. Outcomes have rarely been compared on a national level. METHODS: We queried the Pediatric National Surgical Quality Improvement Program (NSQIP) to identify patients who underwent choledochal cyst excision from 2015 to 2020. Patients were stratified by hepaticoduodenostomy (HD) versus Roux-en-Y hepaticojejunostomy (RNYHJ), use of minimally invasive surgery (MIS), and age at surgery. We collected several outcomes, including length of stay (LOS), reoperation, complications, blood transfusions, and readmission rate. We compared outcomes between cohorts using nonparametric tests and multivariate regression. RESULTS: Altogether, 407 patients met the study criteria, 150 (36.8%) underwent RNYHJ reconstruction, 100 (24.6%) underwent MIS only, and 111 (27.3%) were less than one year old. Patients who underwent open surgery were younger (median age 2.31 vs. 4.25 years, p = 0.002) and more likely underwent RNYHJ reconstruction (42.7% vs. 19%, p = 0.001). On adjusted analysis, the outcomes of LOS, reoperation, transfusion, and complications were similar between the type of reconstruction, operative approach, and age. Patients undergoing RNYHJ had lower rates of readmission than patients undergoing HD (4.0% vs. 10.5%, OR 0.34, CI [0.12, 0.79], p = 0.02). CONCLUSIONS: In children with choledochal cysts, most short-term outcomes were similar between reconstructive techniques, operative approach, and age at resection, although HD reconstruction was associated with a higher readmission rate in this study. Clinical decision-making should be driven by long-term and biliary-specific outcomes.


Asunto(s)
Quiste del Colédoco , Laparoscopía , Niño , Humanos , Preescolar , Lactante , Quiste del Colédoco/cirugía , Mejoramiento de la Calidad , Anastomosis en-Y de Roux/métodos , Laparoscopía/métodos , Resultado del Tratamiento , Estudios Retrospectivos
2.
Clin Transplant ; 37(7): e14954, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-36892182

RESUMEN

INTRODUCTION: Living donor liver transplantation (LDLT) is a promising option for mitigating the deceased donor organ shortage and reducing waitlist mortality. Despite excellent outcomes and data supporting expanding candidate indications for LDLT, broader uptake throughout the United States has yet to occur. METHODS: In response to this, the American Society of Transplantation hosted a virtual consensus conference (October 18-19, 2021), bringing together relevant experts with the aim of identifying barriers to broader implementation and making recommendations regarding strategies to address these barriers. In this report, we summarize the findings relevant to the selection and engagement of both the LDLT candidate and living donor. Utilizing a modified Delphi approach, barrier and strategy statements were developed, refined, and voted on for overall barrier importance and potential impact and feasibility of the strategy to address said barrier. RESULTS: Barriers identified fell into three general categories: 1) awareness, acceptance, and engagement across patients (potential candidates and donors), providers, and institutions, 2) data gaps and lack of standardization in candidate and donor selection, and 3) data gaps regarding post-living liver donation outcomes and resource needs. CONCLUSIONS: Strategies to address barriers included efforts toward education and engagement across populations, rigorous and collaborative research, and institutional commitment and resources.


Asunto(s)
Trasplante de Hígado , Obtención de Tejidos y Órganos , Humanos , Consenso , Selección de Donante , Donadores Vivos/educación , Estados Unidos
3.
Pediatr Transplant ; 27(7): e14572, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37462281

RESUMEN

BACKGROUND: Previous publications identified a gap in standard education on topics related to advanced hepatology and liver transplantation for pediatric transplant hepatology trainees. The Society of Pediatric Liver Transplantation (SPLIT) Education Committee designed a Zoom-based lectureship series for all advanced pediatric transplant hepatology trainees. We aim to describe the educational series and feedback from fellow participants. METHODS: Pediatric transplant hepatology trainees from across the United States and Canada were invited to attend 25 Zoom-based lectures on a broad list of topics pertaining to pediatric transplant hepatology. At the completion of the lectureship, a 53-item REDcap survey using single-answer, Likert-scale, and open-ended questions was distributed via email to all participants. RESULTS: A total of 16 fellows from broad geographic areas responded to the survey. Nineteen percent (n = 3/16) of fellows attended all 25 lectures and 31% (n = 5/16) attended 16-20 lectures. Majority of fellows (88%, n = 14/16) reported the lecture series increased knowledge of liver disease, increased confidence in managing children with liver disease, and aided with board preparation. Additionally, over half of the fellows (81%, n = 13/16) reported the series served as a platform for networking and mentoring from peers and experts in the field. All fellows recommended the lecture series for future fellows. CONCLUSION: The SPLIT educational lectureship for advanced pediatric transplant hepatology trainees provided a national education curriculum that not only led to increased knowledge and confidence in the diagnosis and management of common conditions encountered in pediatric transplant hepatology but also provided a unique networking and mentorship environment.

4.
Am J Med Genet A ; 188(11): 3364-3368, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35972026

RESUMEN

There is a broad differential diagnosis of infantile hepatosplenomegaly, with some etiologies being debilitating and treatable. A structured approach to history, examination, and laboratory and radiographic findings is important in diagnosis. Herein, we present a case of Wolman disease presenting as hepatosplenomegaly in an infant. This case details important learning points to help distinguish the diagnosis of Wolman disease from other conditions with overlapping clinical features, such as hemophagocytic lymphohistiocytosis (HLH). The advent of enzyme replacement therapy has dramatically changed the natural history of Wolman disease, and this child showed remarkable improvement with treatment. This child was later found to have extensive adenopathy with retroperitoneal lymph node biopsy demonstrating diffuse infiltration by lipid-laden macrophages, fatty deposits, cholesterol crystals, and calcifications. Similar to the collection of characteristic cells in other lysosomal storage disorders, we postulate that this is characteristic of underlying Wolman disease. We conclude with a summary of learning points from this presentation on infantile hepatosplenomegaly, pertinent to the geneticist, pediatrician, and pediatric subspecialists.


Asunto(s)
Linfohistiocitosis Hemofagocítica , Enfermedad de Wolman , Niño , Colesterol , Hepatomegalia/diagnóstico , Humanos , Lactante , Lípidos , Linfohistiocitosis Hemofagocítica/diagnóstico , Esplenomegalia/complicaciones , Esplenomegalia/diagnóstico , Enfermedad de Wolman/diagnóstico , Enfermedad de Wolman/tratamiento farmacológico , Enfermedad de Wolman/genética
5.
Clin Transplant ; 35(7): e14324, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-34046945

RESUMEN

This is a descriptive study reviewing the outcomes of mammalian target of rapamycin inhibitors (mTORs) in intestinal (IT) and multivisceral transplantation (MVT). This study included 22 patients, 20 adults, and two children, and an overall mean age of 46 years old at the time of transplantation. Twelve patients (54.5%) received IT, and the remainder (45.5%) MVT. The mean time between transplantation and mTORs initiation was 24 months. The indication was worsening renal function in 13 patients (59%), with 9/13 (69.2%) noted to have an increase in glomerular filtration rate of at least 10 ml/min/1.73m2 . The indication for four patients (18.2%) was a history of neuroendocrine tumor. After mTOR initiation, 50% of patients were reduced or weaned off tacrolimus and 13.7% off prednisone. mTORs were discontinued in 11/22 patients. Six patients (54.5%) stopped due to side effects, two (18.1%) for surgery, and one (9%) for acute cellular rejection. Side effects were edema (33.3%), headaches (33.3%), diarrhea (16.7%), and oral ulcers (16.7%). The average duration of mTORs prior to discontinuation due to side effects was 7 months. mTORs may function in their own niche of patients due to the potential renal safety profile, but use is most limited by tolerance to side effects.


Asunto(s)
Inmunosupresores , Sirolimus , Adulto , Niño , Rechazo de Injerto/tratamiento farmacológico , Rechazo de Injerto/etiología , Rechazo de Injerto/prevención & control , Humanos , Inmunosupresores/uso terapéutico , Persona de Mediana Edad , Sirolimus/efectos adversos , Serina-Treonina Quinasas TOR , Tacrolimus
6.
Genet Med ; 21(12): 2686-2694, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31263214

RESUMEN

PURPOSE: In glycogen storage disease type III (GSD III), liver aminotransferases tend to normalize with age giving an impression that hepatic manifestations improve with age. However, despite dietary treatment, long-term liver complications emerge. We present a GSD III liver natural history study in children to better understand changes in hepatic parameters with age. METHODS: We reviewed clinical, biochemical, histological, and radiological data in pediatric patients with GSD III, and performed a literature review of GSD III hepatic findings. RESULTS: Twenty-six patients (median age 12.5 years, range 2-22) with GSD IIIa (n = 23) and IIIb (n = 3) were enrolled in the study. Six of seven pediatric patients showed severe fibrosis on liver biopsy (median [range] age: 1.25 [0.75-7] years). Markers of liver injury (aminotransferases), dysfunction (cholesterol, triglycerides), and glycogen storage (glucose tetrasaccharide, Glc4) were elevated at an early age, and decreased significantly thereafter (p < 0.001). Creatine phosphokinase was also elevated with no significant correlation with age (p = 0.4). CONCLUSION: Liver fibrosis can occur at an early age, and may explain the decrease in aminotransferases and Glc4 with age. Our data outlines the need for systematic follow-up and specific biochemical and radiological tools to monitor the silent course of the liver disease process.


Asunto(s)
Enfermedad del Almacenamiento de Glucógeno Tipo III/patología , Cirrosis Hepática/patología , Adolescente , Biomarcadores , Niño , Preescolar , Colesterol/análisis , Colesterol/metabolismo , Femenino , Glucógeno , Enfermedad del Almacenamiento de Glucógeno/patología , Enfermedad del Almacenamiento de Glucógeno Tipo I/patología , Enfermedad del Almacenamiento de Glucógeno Tipo III/metabolismo , Humanos , Hígado/patología , Cirrosis Hepática/metabolismo , Hepatopatías , Masculino , Oligosacáridos/análisis , Oligosacáridos/metabolismo , Transaminasas/análisis , Transaminasas/metabolismo , Triglicéridos/análisis , Triglicéridos/metabolismo , Adulto Joven
7.
Pediatr Transplant ; 22(8): e13305, 2018 12.
Artículo en Inglés | MEDLINE | ID: mdl-30341782

RESUMEN

PURPOSE: Improvement in outcomes of LT for pediatric HB and HCC has been reported in small series. We analyzed national outcomes and changes in donor, recipient, and perioperative factors over time that may contribute to survival differences. METHODS: The UNOS database was queried for patients age <21 years that underwent LT for a primary diagnosis of HB or HCC (1987-2017). Subjects were divided into historic (transplant before 2010) and contemporary (transplant after 2010) cohorts. Baseline characteristics were compiled and examined. Survival was estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: In total, 599 children with HB received LT (320 historic vs 279 contemporary). Concurrently, 141 children with HCC received LT (92 historic vs 49 contemporary). For both tumors, waitlist time decreased (HB 56.2 days historic vs 33.2 days contemporary, P = 0.017; HCC 189.3 days historic vs 71.7 days contemporary, P = 0.012). In the historic cohorts, patients with HB had a 1-year and 5-year OS of 84.6% and 75.1%, respectively. Survival for HCC was 84.4% and 59.9%, respectively. Outcomes improved in the contemporary era to 89.1% and 82.6% for HB, and 94.7% and 80.8% for HCC, respectively (both log-rank test P < 0.0001). CONCLUSION: Outcomes of LT have improved significantly, with contemporary survival now equivalent between these tumors and exceeding 80% 5-year OS. Future studies are needed to explore whether offering LT in patients that are resectable is justifiable.


Asunto(s)
Carcinoma Hepatocelular/cirugía , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado , Adolescente , Carcinoma Hepatocelular/mortalidad , Niño , Preescolar , Femenino , Hepatoblastoma/mortalidad , Humanos , Estimación de Kaplan-Meier , Neoplasias Hepáticas/mortalidad , Donadores Vivos , Masculino , Sistema de Registros , Estudios Retrospectivos , Donantes de Tejidos , Resultado del Tratamiento , Estados Unidos , Listas de Espera
9.
Hepatology ; 73(4): 1625, 2021 04.
Artículo en Inglés | MEDLINE | ID: mdl-33040372
10.
Pediatr Res ; 78(2): 181-9, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25897540

RESUMEN

BACKGROUND: Phytosterols in soybean oil (SO) lipids likely contribute to parenteral nutrition-associated liver disease (PNALD) in infants. No characterization of phytosterol metabolism has been done in infants receiving SO lipids. METHODS: In a prospective cohort study, 45 neonates (36 SO lipid vs. 9 control) underwent serial blood sample measurements of sitosterol, campesterol, and stigmasterol. Mathematical modeling was used to determine pharmacokinetic parameters of phytosterol metabolism and phytosterol exposure. RESULTS: Compared to controls, SO lipid-exposed infants had significantly higher levels of sitosterol and campesterol (P < 0.01). During SO lipid infusion, sitosterol and campesterol reached half of steady-state plasma levels within 1.5 and 0.8 d, respectively. Steady-state level was highest for sitosterol (1.68 mg/dl), followed by campesterol (0.98 mg/dl), and lowest for stigmasterol (0.01 mg/dl). Infants born < 28 wk gestational age had higher sitosterol steady-state levels (P = 0.03) and higher area under the curve for sitosterol (P = 0.03) during the first 5 d of SO lipid (AUC5) than infants born ≥ 28 wk gestational age. CONCLUSION: Phytosterols in SO lipid accumulate rapidly in neonates. Very preterm infants receiving SO lipid have higher sitosterol exposure, and may have poorly developed mechanisms of eliminating phytosterols that may contribute to their vulnerability to PNALD.


Asunto(s)
Nutrición Parenteral , Fitosteroles/farmacocinética , Femenino , Semivida , Humanos , Recién Nacido , Masculino , Estudios Prospectivos
11.
J Pediatr Gastroenterol Nutr ; 61(4): 408-10, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25885878

RESUMEN

The aims of the study were to describe infliximab adherence in a pediatric inflammatory bowel disease cohort, to identify demographic and disease factors associated with adherence, and to examine differences in acute care use among adherent and nonadherent patients. Charts of patients who received infliximab at the Children's Hospital of Wisconsin (CHW) between October 2010 and October 2012 were retrospectively reviewed. A total of 151 patients met the inclusion criteria; 91.4% of the patients were adherent. Nonadherent patients had more emergency room visits and hospitalizations than adherent patients. The study is the first to show high adherence rates to infliximab in a pediatric cohort.


Asunto(s)
Antiinflamatorios no Esteroideos/uso terapéutico , Colitis Ulcerosa/tratamiento farmacológico , Enfermedad de Crohn/tratamiento farmacológico , Fármacos Gastrointestinales/uso terapéutico , Infliximab/uso terapéutico , Quimioterapia de Mantención , Cumplimiento de la Medicación , Adolescente , Niño , Preescolar , Estudios de Cohortes , Colitis Ulcerosa/fisiopatología , Colitis Ulcerosa/terapia , Enfermedad de Crohn/fisiopatología , Enfermedad de Crohn/terapia , Servicio de Urgencia en Hospital , Femenino , Fármacos Gastrointestinales/administración & dosificación , Hospitalización , Hospitales Pediátricos , Humanos , Infliximab/administración & dosificación , Infusiones Intravenosas , Masculino , Registros Médicos , Estudios Retrospectivos , Brote de los Síntomas , Wisconsin
12.
Prog Transplant ; 25(2): 139-46, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26107274

RESUMEN

CONTEXT: Solid-organ transplant is the treatment of choice for end-stage organ failure and requires a transition from management of a life-threatening condition to a chronic illness. Despite research focusing on quality of life after transplant, there is a gap addressing the role of managing a chronic illness focusing on vulnerability and impact on family. OBJECTIVE: Identify patient and family patterns of adaptation among kidney and liver transplant recipients in regard to (1) vulnerability, (2) impact of illness on the family, (3) family functioning, and (4) quality of life (parent and child report). DESIGN: Cross-sectional study enrolling children 5 to 18 years old and their parent at a single time point after kidney or liver transplant. Validated self-report tools were completed. RESULTS: In all, 47 participants (24 kidney and 23 liver) were recruited. Mean age at transplant was 4.0 (kidney) and 2.1 (liver) years. Mean age at report was 12.1 (kidney) and 7.1 (liver) years. Child vulnerability correlated negatively with (1) family impact in the kidney (P < .05) and liver (P < .05) transplant groups, (2) PedsQL subscales including Parent Emotional (P< .05), Parent Social (P< .01), Parent Psychosocial (P < .01), Parent Physical (P < .05), Parent School (P < .05), and Child Social (P < .01) in the kidney transplant group, (3) PedsQL Parent Emotional subscale (P< .01) in the liver transplant group, and (4) Functional status (P < .01) in the liver transplant group. CONCLUSIONS: Child vulnerability provides insight into quality of life and the impact of illness on the family and family functioning.


Asunto(s)
Enfermedad Crónica/psicología , Familia/psicología , Trasplante de Riñón/psicología , Trasplante de Hígado/psicología , Padres/psicología , Calidad de Vida , Receptores de Trasplantes/psicología , Adaptación Psicológica , Adolescente , Adulto , Niño , Preescolar , Enfermedad Crónica/enfermería , Estudios Transversales , Femenino , Humanos , Trasplante de Riñón/enfermería , Trasplante de Hígado/enfermería , Masculino , Persona de Mediana Edad , Estrés Psicológico , Wisconsin
13.
Transplant Proc ; 56(1): 161-168, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38195284

RESUMEN

BACKGROUND: This study aims to evaluate patient outcomes of simultaneous triple organ transplants, which may provide insight into optimal donor allocation while maximizing recipient benefit. METHODS: Triple organ transplants and their corollary dual organ transplants were identified using the United Network for Organ Sharing database. Triple organ transplants evaluated included heart-lung-kidney (n = 12) and heart-liver-kidney (n = 37). Heart-lung-kidney recipients were compared with heart-lung (n = 325), lung-kidney (n = 91), and heart-kidney (n = 2022) groups. Heart-liver-kidney recipients were compared with heart-liver (n = 451), liver-kidney (n = 10422), and heart-kidney (n = 2517) recipients. Patient survival outcomes were calculated using the Kaplan-Meier method and compared using log-rank tests. RESULTS: Patients undergoing triple organ transplants showed similar 10-year survival as their corresponding dual organ transplant cohorts. Patient survival estimate at 10 years for the heart-lung-kidney group was 45%, with no statistically significant difference in survival when compared with dual organ groups (P = .16). Survival estimates at 10 years for the heart-liver-kidney group was 49%, with no statistically significant difference in survival when compared with dual organ groups (P = .06). CONCLUSION: Despite the surgical burden of adding a third organ transplant, heart-liver-kidney and heart-lung-kidney have similar survival outcomes to dual organ equivalents and represent a reasonable allocation option in well-selected patients.


Asunto(s)
Trasplante de Corazón , Trasplante de Órganos , Obtención de Tejidos y Órganos , Humanos , Estados Unidos , Trasplante de Corazón/efectos adversos , Incidencia , Estudios Retrospectivos , Trasplante de Órganos/efectos adversos , Riñón , Donantes de Tejidos , Supervivencia de Injerto
14.
Prog Transplant ; 23(3): 253-7, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23996945

RESUMEN

Respiratory syncytial virus (RSV) is a ubiquitous virus responsible for acute infections of the respiratory tract in patients of all ages. RSV presents significant health risks to immunocompromised patients. Two patients, 1 before a liver transplant and 1 after a liver transplant, died of a severe RSV infection. Because of the high risk of death, we recommend expanding the criteria for palivizumab prophylaxis to 2 types of patients: (1) patients with chronic liver disease or who have received a liver transplant and are 24 months old or less and (2) transplant recipients with underlying pulmonary conditions who are less than 36 months old. Further research is indicated in pediatric solid-organ transplant centers to evaluate the effective management of RSV infection to prevent morbidity.


Asunto(s)
Trasplante de Hígado , Infecciones por Virus Sincitial Respiratorio/complicaciones , Atresia Biliar/cirugía , Preescolar , Resultado Fatal , Humanos , Huésped Inmunocomprometido , Lactante , Masculino
15.
Hepatol Commun ; 7(10)2023 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-37755881

RESUMEN

BACKGROUND: Tobacco smoking during pregnancy is associated with metabolic dysfunction in children, but mechanistic insights remain limited. Hypomethylation of cg05575921 in the aryl hydrocarbon receptor repressor (AHRR) gene is associated with in utero tobacco smoke exposure. In this study, we evaluated whether AHRR hypomethylation mediates the association between maternal smoking and metabolic dysfunction in children. METHODS: We assessed metabolic dysfunction using liver fat content (LFC), serum, and clinical data in children aged 7-12 years (n=78) followed since birth. Maternal smoking was self-reported at 12 weeks gestation. Methylation was measured by means of pyrosequencing at 3 sequential CpG sites, including cg05575921, at birth and at ages 7-12. Regression models were used to evaluate whether AHRR methylation mediated the association between maternal smoking and child metabolic dysfunction. RESULTS: Average AHRR methylation at birth was significantly higher among children of nonsmoking mothers compared with children of mothers who smoked (69.8% ± 4.4% vs. 63.5% ± 5.5, p=0.0006). AHRR hypomethylation at birth was associated with higher liver fat content (p=0.01), triglycerides (p=0.01), and alanine aminotransferase levels (p=0.03), and lower HDL cholesterol (p=0.01) in childhood. AHRR hypomethylation significantly mediated associations between maternal smoking and liver fat content (indirect effect=0.213, p=0.018), triglycerides (indirect effect=0.297, p=0.044), and HDL cholesterol (indirect effect = -0.413, p=0.007). AHRR methylation in childhood (n=78) was no longer significantly associated with prenatal smoke exposure or child metabolic parameters (p>0.05). CONCLUSIONS: AHRR hypomethylation significantly mediates the association between prenatal tobacco smoke exposure and features of childhood metabolic dysfunction, despite the lack of persistent hypomethylation of AHRR into childhood. Further studies are needed to replicate these findings and to explore their causal and long-term significance.


Asunto(s)
Contaminación por Humo de Tabaco , Recién Nacido , Femenino , Embarazo , Niño , Humanos , HDL-Colesterol , Contaminación por Humo de Tabaco/efectos adversos , Fumar/efectos adversos , Fumar Tabaco , Metaboloma , Proteínas Represoras/genética , Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/genética
16.
JAMA Netw Open ; 6(10): e2337602, 2023 10 02.
Artículo en Inglés | MEDLINE | ID: mdl-37824141

RESUMEN

Importance: Live vaccines (measles-mumps-rubella [MMR] and varicella-zoster virus [VZV]) have not been recommended after solid organ transplant due to concern for inciting vaccine strain infection in an immunocompromised host. However, the rates of measles, mumps, and varicella are rising nationally and internationally, leaving susceptible immunocompromised children at risk for life-threating conditions. Objective: To determine the safety and immunogenicity of live vaccines in pediatric liver and kidney transplant recipients. Design, Setting, and Participants: This cohort study included select pediatric liver and kidney transplant recipients who had not completed their primary MMR and VZV vaccine series and/or who displayed nonprotective serum antibody levels at enrollment between January 1, 2002, and February 28, 2023. Eligibility for live vaccine was determined by individual US pediatric solid organ transplant center protocols. Exposures: Exposure was defined as receipt of a posttransplant live vaccine. Transplant recipients received 1 to 3 doses of MMR vaccine and/or 1 to 3 doses of VZV vaccine. Main Outcome and Measure: Safety data were collected following each vaccination, and antibody levels were obtained at 0 to 3 months and 1 year following vaccination. Comparisons were performed using Mann-Whitney U test, and factors associated with development of postvaccination protective antibodies were explored using univariate analysis. Results: The cohort included 281 children (270 [96%] liver, 9 [3%] kidney, 2 [1%] liver-kidney recipients) from 18 centers. The median time from transplant to enrollment was 6.3 years (IQR, 3.4-11.1 years). The median age at first posttransplant vaccine was 8.9 years (IQR, 4.7-13.8 years). A total of 202 of 275 (73%) children were receiving low-level monotherapy immunosuppression at the time of vaccination. The majority of children developed protective antibodies following vaccination (107 of 149 [72%] varicella, 130 of 152 [86%] measles, 100 of 120 [83%] mumps, and 124 of 125 [99%] rubella). One year post vaccination, the majority of children who initially mounted protective antibodies maintained this protection (34 of 44 [77%] varicella, 45 of 49 [92%] measles, 35 of 42 [83%] mumps, 51 of 54 [94%] rubella). Five children developed clinical varicella, all of which resolved within 1 week. There were no cases of measles or rubella and no episodes of graft rejection within 1 month of vaccination. There was no association between antibody response and immunosuppression level at the time of vaccination. Conclusions and Relevance: The findings suggest that live vaccinations may be safe and immunogenic after solid organ transplant in select pediatric recipients and can offer protection against circulating measles, mumps, and varicella.


Asunto(s)
Varicela , Sarampión , Paperas , Rubéola (Sarampión Alemán) , Vacunas Virales , Niño , Humanos , Preescolar , Adolescente , Varicela/prevención & control , Vacuna contra la Varicela/efectos adversos , Vacunas Combinadas , Receptores de Trasplantes , Estudios de Cohortes , Rubéola (Sarampión Alemán)/prevención & control , Sarampión/prevención & control , Vacunas Atenuadas/efectos adversos
17.
JMIR Nurs ; 5(1): e39263, 2022 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-35838761

RESUMEN

BACKGROUND: Around 1800 pediatric transplantations were performed in 2021, which is approximately 5% of the annual rate of solid organ transplantations carried out in the United States. Effective family self-management in the transition from hospital to home-based recovery promotes successful outcomes of transplantation. The use of mHealth to deliver self-management interventions is a strategy that can be used to support family self-management for transplantation recipients and their families. OBJECTIVE: The study aims to evaluate the acceptability of an mHealth intervention (myFAMI) that combined use of a smartphone app with triggered nurse communication with family members of pediatric transplantation recipients. METHODS: This is a secondary analysis of qualitative data from family members who received the myFAMI intervention within a larger randomized controlled trial. Eligible participants used the app in the 30-day time frame after discharge and participated in a 30-day postdischarge telephone interview. Content analysis was used to generate themes. RESULTS: A total of 4 key themes were identified: (1) general acceptance, (2) positive interactions, (3) home management after hospital discharge, and (4) opportunities for improvement. CONCLUSIONS: Acceptability of the intervention was high. Family members rated the smartphone application as easy to use. myFAMI allowed the opportunity for families to feel connected to and engage with the medical team while in their home environment. Family members valued and appreciated ongoing support and education specifically in this first 30 days after their child's hospital discharge and many felt it contributed positively to the management of their child's medical needs at home. Family members provided recommendations for future refinement of the app and some suggested that a longer follow-up period would be beneficial. The development and refinement of mHealth care delivery strategies hold potential for improving outcomes for solid organ transplantation patients and their families and as a model to consider in other chronic illness populations. TRIAL REGISTRATION: ClinicalTrials.gov NCT03533049; https://clinicaltrials.gov/ct2/show/NCT03533049.

18.
Epigenetics ; 17(11): 1446-1461, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35188871

RESUMEN

Non-Alcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Epigenetic alterations, such as through DNA methylation (DNAm), may link adverse childhood exposures and fatty liver and provide non-invasive methods for identifying children at high risk for NAFLD and associated metabolic dysfunction. We investigated the association between differential DNAm and liver fat content (LFC) and liver injury in pre-adolescent children. Leveraging data from the Newborn Epigenetics Study (NEST), we enrolled 90    mother-child dyads and used linear regression to identify CpG sites and differentially methylated regions (DMRs) in peripheral blood associated with LFC and alanine aminotransferase (ALT) levels in 7-12yo children. DNAm was measured using Infinium HumanMethylationEPIC BeadChips (Illumina). LFC and fibrosis were quantified by magnetic resonance imaging proton density fat fraction and elastography. Median LFC was 1.4% (range, 0.3-13.4%) and MRE was 2.5 kPa (range, 1.5-3.6kPa). Three children had LFC ≥ 5%, while six (7.6%) met our definition of NAFLD (LFC ≥ 3.7%). All children with NAFLD were obese and five were Black. LFC was associated with 88 DMRs and 106 CpGs (FDR<5%). The top two CpGs, cg25474373 and cg07264203, mapped to or near RFTN2 and PRICKLE2 genes. These two CpG sites were also significantly associated with a NAFLD diagnosis. As higher LFC associates with an adverse cardiometabolic profile already in childhood, altered DNAm may identify these children early in disease course for targeted intervention. Larger, longitudinal studies are needed to validate these findings and determine mechanistic relevance.


Asunto(s)
Enfermedad del Hígado Graso no Alcohólico , Adolescente , Humanos , Recién Nacido , Alanina Transaminasa/genética , Alanina Transaminasa/metabolismo , Metilación de ADN , Hígado/metabolismo , Enfermedad del Hígado Graso no Alcohólico/diagnóstico por imagen , Enfermedad del Hígado Graso no Alcohólico/genética
19.
PLoS One ; 17(10): e0269766, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36256635

RESUMEN

BACKGROUND: Pediatric solid organ transplant (SOT) recipients commonly have Epstein-Barr virus (EBV) DNAemia and are at risk of developing post-transplant lymphoproliferative disorder (PTLD). EBV DNAemia has not been analyzed on a continuous scale in this population. METHODS: All children ≤ 18 years of age who underwent SOT at a single center between January 1, 2007 and July 31, 2018 were included in this retrospective study. Transplant episodes in which PTLD occurred were compared to transplant episodes without PTLD. Multivariable logistic regression was used to identify factors associated with the development of EBV DNAemia and maximum height of EBV DNAemia. A Cox proportional hazards model was used to calculate hazard ratios for time to PTLD. RESULTS: Of 275 total transplant recipients and 294 transplant episodes, there were 14 episodes of PTLD. Intestinal and multivisceral transplant were strongly associated with PTLD (p = 0.002). Risk factors for the development of EBV DNAemia include donor and recipient positive EBV serologies (p = 0.001) and older age (p = 0.001). Maximum level of EBV DNAemia was significantly associated with development of PTLD (p<0.0001). Every one log (log10) increase in the maximum level of EBV DNAemia was associated with a more than doubling of the hazard on developing PTLD (HR: 2.18, 95% CI 1.19-3.99). CONCLUSIONS: Transplant type was strongly associated with development of PTLD in pediatric SOT recipients. EBV serologies and age were associated with the development of EBV DNAemia and height of DNAemia. High levels of EBV DNAemia were strongly associated with an increased hazard for PTLD.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Trastornos Linfoproliferativos , Trasplante de Órganos , Niño , Humanos , Herpesvirus Humano 4/genética , Receptores de Trasplantes , Estudios Retrospectivos , ADN Viral/genética , Trastornos Linfoproliferativos/epidemiología , Trastornos Linfoproliferativos/etiología , Trasplante de Órganos/efectos adversos
20.
West J Nurs Res ; 44(10): 955-965, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34154460

RESUMEN

Families of pediatric solid organ transplant recipients need ongoing education and support in the first 30 days following hospital discharge for the transplantation. The purpose of this report is to describe the feasibility, acceptability, and preliminary efficacy of a mHealth family-self management intervention, (myFAMI), designed to improve post-discharge outcomes of coping, family quality of life, self-efficacy, family self-management, and utilization of health care resources. We enrolled 46 primary family members. myFAMI was feasible and acceptable; 81% (n=17/21) of family members completed the app at least 24/30 days (goal 80% completion rate). Family members generated 134 trigger alerts and received a nurse response within the goal timeframe of < 2 h 99% of the time. Although there were no significant differences between groups, primary outcomes were in the expected direction. The intervention was well received and is feasible for future post-discharge interventions for families of children who receive an organ transplant.


Asunto(s)
Automanejo , Telemedicina , Cuidados Posteriores , Niño , Estudios de Factibilidad , Humanos , Alta del Paciente , Calidad de Vida
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