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BACKGROUND: Asymptomatic pulmonary nodules may appear at any point after lung transplantation. The differential diagnosis is broad and includes serious life-threatening disease entities. METHODS: A retrospective case report of a single patient who developed a pulmonary nodule after lung transplantation. RESULTS: At 2 years post-transplant, an 11-year-old with cystic fibrosis was asymptomatic and had normal lung function. A single nodule was noted on surveillance chest CT scan. Initial evaluation was negative, but subsequently, he was diagnosed with cryptococcal osteomyelitis in a thoracic rib. He responded well to an extended course of antifungal therapy without loss of allograft function or infectious complications. CONCLUSION: Pulmonary nodules after lung transplantation may be a harbinger of serious complications. A systematic approach to evaluation and follow-up is recommended.
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Criptococosis/diagnóstico por imagen , Trasplante de Pulmón , Osteomielitis/diagnóstico por imagen , Osteomielitis/microbiología , Costillas/diagnóstico por imagen , Costillas/microbiología , Tomografía Computarizada por Rayos X , Adolescente , Antifúngicos/uso terapéutico , Criptococosis/tratamiento farmacológico , Diagnóstico Diferencial , Humanos , Masculino , Osteomielitis/tratamiento farmacológicoRESUMEN
Lung transplantation has become an accepted therapeutic option for a select group of children with end-stage lung disease. We evaluated the impact of early extubation in a pediatric lung transplant population and its post-operative outcomes. Single-center retrospective study. PICU within a tertiary academic pediatric hospital. Patients <22 years after pulmonary transplant between January 2011 and December 2016. A total of 74 patients underwent lung transplantation. The primary pretransplantation diagnoses included cystic fibrosis (58%), pulmonary fibrosis (9%), and surfactant dysfunction disorders (10%). Of 60 patients, 36 (60%) were extubated within 24 hours and 24 patients after 24 hours (40%). A total of seven patients (11.6%) required reintubation within 24 hours. Median length of stay for the early extubation group was shorter at 3 days ([(IQR) 2.2-4.7]) compared to 5 days (IQR, 3-7) (P = .02) in the late extubation group. Median costs were lower for the early extubation group with 13,833 US dollars (IQR, 9980-22,822) vs 23 671 US dollars (IQR, 16 673-39 267) (P = .043). Fourteen patients were in the PICU prior to their transplantation; this did not affect their early extubation success. Neither did the fact of requiring invasive or non-invasive mechanical ventilation before transplantation. Early extubation appears to be safe in a pediatric population after lung transplantation and is associated with a shorter LOS and decreased hospital costs. It may prevent known complications associated with mechanical ventilation.
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Extubación Traqueal/métodos , Trasplante de Pulmón , Cuidados Posoperatorios/métodos , Adolescente , Extubación Traqueal/economía , Niño , Preescolar , Femenino , Costos de Hospital/estadística & datos numéricos , Humanos , Lactante , Recién Nacido , Tiempo de Internación/economía , Tiempo de Internación/estadística & datos numéricos , Trasplante de Pulmón/economía , Masculino , Evaluación de Resultado en la Atención de Salud , Cuidados Posoperatorios/economía , Estudios Retrospectivos , Texas , Adulto JovenRESUMEN
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies. OBJECTIVE: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis. MATERIALS AND METHODS: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018. Two pediatric radiologists evaluated the chest imaging studies for the presence or absence of published radiologic findings of PPFE in adults, including platythorax, pneumothorax, upper lobe predominant pleural and septal thickening, and bronchiectasis. Platythorax indices were calculated from the normal chest CT exams of eight age- and gender-matched individuals obtained via the radiology search engine. RESULTS: The mean presentation age was 12.9 years (range: 7-16 years). Seven of the eight had a history of chemotherapy and radiation therapy for cancer. Three of the eight had undergone bone marrow transplantation and none had undergone lung transplantation. The mean time between chemotherapy, radiation therapy, and/or bone marrow transplantation and the presentation of PPFE was 8.4 years (range: 5.6-12.1 years). Most of the patients presented with dyspnea (63%), cough (50%) and/or pneumothorax (38%). The mean percentage of predicted FEV1 (forced expiratory volume in one second) was 14.1 (range: 7.7-27.5). All eight patients demonstrated platythorax, bronchiectasis, pleural and septal thickening (upper lobes in four, upper and lower lobes in four) and six had pneumothorax. Five underwent lung biopsies, four of whom developed pneumothoraces. CONCLUSION: Clinical and radiologic findings of pediatric PPFE are similar to those in adults, although a majority of the former have a history of treated cancer. Clinical presentation of restrictive lung disease, dyspnea, cough or spontaneous pneumothorax years after treatment for childhood cancer combined with platythorax, upper lobe pleural and septal thickening and traction bronchiectasis on chest CT establishes a presumptive diagnosis of PPFE.
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Neumonías Intersticiales Idiopáticas/diagnóstico por imagen , Neumonías Intersticiales Idiopáticas/etiología , Tomografía Computarizada por Rayos X , Adolescente , Trasplante de Médula Ósea , Niño , Preescolar , Femenino , Humanos , Neumonías Intersticiales Idiopáticas/fisiopatología , Masculino , Neoplasias/tratamiento farmacológico , Neoplasias/radioterapia , Pruebas de Función RespiratoriaRESUMEN
OBJECTIVE: To assess the rate of and risk factors for 30-day hospital readmission in children with pulmonary hypertension. STUDY DESIGN: The Pediatric Health Information System database was analyzed for patients ≤18 years old with pulmonary hypertension (International Classification of Diseases, Ninth Revision, diagnosis codes of 416.0, 416.1, 416.8, or 416.9) admitted from 2005 through 2014. A generalized hierarchical regression model was used to determine significant ORs and 95% CIs associated with 30-day readmission. RESULTS: A total of 13580 patients met inclusion criteria (median age 1.7 years [IQR 0.3-8.7], 45.3% with congenital heart disease). Admissions increased 4-fold throughout the study period. Associated hospital charges increased from $119 million in 2004 to $929 million in 2014. During initial admission, 57.4% of patients required admission to the intensive care unit, and 48.2% required mechanical ventilation. The 30-day readmission rate was 26.3%. Mortality during readmission was 4.2%. Factors increasing odds of readmission included a lower hospital volume of pulmonary hypertension admissions (1.41 [1.23-1.57], P < .001) and having public insurance (1.26 [1.16-1.38], P < .001). Decreased odds of readmission were associated with older age and the presence of congenital heart disease (0.86 [0.79-0.93], P < .001). CONCLUSIONS: The pediatric pulmonary hypertension population carries significant morbidity, as reflected by a high use of intensive care unit resources and a high 30-day readmission rate. Younger patients and those with public insurance represent particularly at-risk groups.
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Hipertensión Pulmonar/epidemiología , Readmisión del Paciente/estadística & datos numéricos , Adolescente , Niño , Preescolar , Femenino , Humanos , Hipertensión Pulmonar/terapia , Lactante , Recién Nacido , Masculino , Análisis de Regresión , Estudios Retrospectivos , Factores de RiesgoRESUMEN
OBJECTIVE: To describe a series of patients with pathogenic variants in FLNA and progressive lung disease necessitating lung transplantation. STUDY DESIGN: We conducted a retrospective chart review of 6 female infants with heterozygous presumed loss-of-function pathogenic variants in FLNA whose initial presentation was early and progressive respiratory failure. RESULTS: Each patient received lung transplantation at an average age of 11 months (range, 5-15 months). All patients had pulmonary arterial hypertension and chronic respiratory failure requiring tracheostomy and escalating levels of ventilator support before transplantation. All 6 patients survived initial lung transplantation; however, 1 patient died after a subsequent heart-lung transplant. The remaining 5 patients are living unrestricted lives on chronic immunosuppression at most recent follow-up (range, 19 months to 11.3 years post-transplantation). However, in all patients, severe ascending aortic dilation has been observed with aortic regurgitation. CONCLUSIONS: Respiratory failure secondary to progressive obstructive lung disease during infancy may be the presenting phenotype of FLNA-associated periventricular nodular heterotopia. We describe a cohort of patients with progressive respiratory failure related to a pathogenic variant in FLNA and present lung transplantation as a viable therapeutic option for this group of patients.
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Filaminas/genética , Hipertensión Pulmonar/cirugía , Enfermedades Pulmonares/genética , Enfermedades Pulmonares/cirugía , Trasplante de Pulmón , Insuficiencia Respiratoria/cirugía , Niño , Preescolar , Femenino , Humanos , Hipertensión Pulmonar/etiología , Lactante , Insuficiencia Respiratoria/etiología , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Transplant volume represents lung transplant (LTx) expertise and predicts outcomes, so we sought to determine outcomes related to center volumes in cystic fibrosis (CF). United Network for Organ Sharing data were queried for patients with CF in the United States (US) receiving bilateral LTx from 2005 to 2015. Multivariable Cox regression was used to model survival to 1 year and long-term (>1 year) survival, conditional on surviving at least 1 year. A total of 2025 patients and 67 centers were included in the analysis. The median annual LTx volumes were three in CF [interquartile range (IQR): 2, 6] and 17 in non-CF (IQR: 8, 33). Multivariable Cox regression in cases with complete data and surviving at least 1 year (n = 1510) demonstrated that greater annual CF LTx volume (HR per 10 LTx = 0.66; 95% CI: 0.49, 0.89; P = 0.006) but not greater non-CF LTx volume (HR = 1.00; 95% CI: 0.96, 1.05; P = 0.844) was associated with improved long-term survival in LTx recipients with CF. A Wald interaction test confirmed that CF LTx volume was more strongly associated with long-term outcomes than non-CF LTx volume (P = 0.012). In a US cohort, center volume was not associated with 1-year survival. CF-specific expertise predicted improved long-term outcomes of LTx for CF, whereas general LTx expertise was unassociated with CF patients' survival.
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Fibrosis Quística/cirugía , Hospitales/estadística & datos numéricos , Trasplante de Pulmón , Adolescente , Adulto , Niño , Femenino , Humanos , Pulmón/cirugía , Masculino , Persona de Mediana Edad , Análisis Multivariante , Modelos de Riesgos Proporcionales , Sistema de Registros , Estudios Retrospectivos , Factores de Riesgo , Análisis de Supervivencia , Trasplantes , Resultado del Tratamiento , Estados Unidos , Adulto JovenRESUMEN
BACKGROUND: Premature infants surviving beyond a postmenstrual age (PMA) of 36 weeks with severe or grade 3 bronchopulmonary dysplasia (sBPD) have significant predischarge mortality. The in-hospital mortality for BPD supported by invasive mechanical ventilation beyond 36 weeks PMA is not well described. The role of lung transplantation in treating severe BPD is uncertain. We studied our experience over 20 years to better define the predischarge mortality of infants with progressive grade 3 BPD and whether lung transplant is a feasible intervention. METHODS: Data were obtained from a retrospective review of medical records from Children's Minnesota over a 20-year period (1997-2016). Inclusion criteria included prematurity <32 weeks PMA, BPD, tracheostomy for chronic respiratory failure, and survival beyond 36 weeks PMA. Collected data included perinatal demographics, in-hospital medications and interventions, level of respiratory support, and outcomes. RESULTS: In all, 2374 infants were identified who survived beyond 36 weeks PMA with a diagnosis of <32 weeks gestation prematurity and BPD. Of these, 143/2374 (6.0%) survived beyond 36 weeks PMA and required invasive mechanical ventilation with subsequent tracheostomy for management. Among these patients, discharge to home with tracheostomy occurred in 127/143 (88.8%), and predischarge death or lung transplantation occurred in 16/143 (11.2%). Deteriorating cardiopulmonary status was associated with pulmonary hypertension, prolonged hypoxemic episodes and the need for deep sedation or neuromuscular relaxation. Three of four patients referred for lung transplantation had >5-year survival, chronic allograft rejection, and mild to moderate developmental delays. CONCLUSIONS: Chronic respiratory failure requiring invasive mechanical ventilation for grade 3 BPD is associated with significant morbidity and mortality. For selected patients and their families, timely referral for lung transplantation is a viable option for end-stage grade 3 BPD. As in other infants receiving solid organ transplants, long-term issues with co-morbidities and special needs persist into childhood.
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Displasia Broncopulmonar , Trasplante de Pulmón , Insuficiencia Respiratoria , Recién Nacido , Lactante , Niño , Humanos , Displasia Broncopulmonar/cirugía , Traqueostomía , Ventiladores Mecánicos , Insuficiencia Respiratoria/etiología , Insuficiencia Respiratoria/terapiaRESUMEN
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.
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Trasplante de Pulmón , Donantes de Tejidos , Obtención de Tejidos y Órganos , Listas de Espera , Adolescente , Adulto , Niño , Humanos , Lactante , Pulmón , Trasplante de Pulmón/métodos , Trasplante de Pulmón/normas , Trasplante de Órganos , Obtención de Tejidos y Órganos/organización & administración , Obtención de Tejidos y Órganos/normas , Donantes de Tejidos/provisión & distribución , Listas de Espera/mortalidadRESUMEN
Rationale: Since its inception, older children and adolescents have predominated in pediatric lung transplantation. Most pediatric lung transplant programs around the world have transplanted few infants and young children. Early mortality after lung transplantation and inadequate donor organs have been perceived as limitations for success in lung transplantation at this age. Objectives: Our aim was to describe our experience in a large pediatric lung transplant program with respect to lung transplantation in infants and young children, focusing on diagnosis, waitlist, and mortality. Methods: We performed a retrospective review of infants and young children under 3 years of age at the time of transplant in our program from 2002 through 2020. Results: The patient cohort represented a severely morbid recipient group, with the majority hospitalized in the intensive care unit on mechanical ventilation just before transplantation. There was a marked heterogeneity of diagnoses distinct from diagnoses in an older cohort. Waitlist time was shorter than in older age cohorts. There was a decrease in early mortality, lower incidence of allograft rejection, and satisfactory long-term survival in this age group compared with the older cohort and published experience. Severe viral infection was an important cause of early mortality after transplant. Nonetheless, survival is comparable to older patients, with better enduring survival in those who survive the early transplant period in more recent years. Conclusions: Carefully selected infants and young children with end-stage lung and pulmonary vascular disease are appropriate candidates for lung transplantation and are likely underserved by current clinical practice.
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Enfermedades Pulmonares , Trasplante de Pulmón , Enfermedades Vasculares , Adolescente , Niño , Humanos , Lactante , Preescolar , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
Heritable pulmonary artery hypertension (HPAH) is an increasingly recognized type of pulmonary arterial hypertension, in both pediatric and adult population. Intrinsic to hereditary disease, screening for genetic mutations within families is an important component of diagnosis and understanding burden of disease. Recently, consensus guidelines are published for genetic screening in PAH. These guidelines include recommendations for screening at diagnosis, noting individuals with presumed PAH due to familial, or idiopathic etiologies. Cascade genetic testing is specifically recommended as a testing paradigm to screen relatives for detection of mutation carriers, who may be asymptomatic. Without targeted genetic testing, familial mutation carriers may only come to attention when pulmonary vascular disease burden is high enough to cause symptoms, suggesting more advanced disease. Here, we present our collective experience with HPAH in five distinct families, specifically to report on the clinical courses of patients who were diagnosed with genetic mutation at diagnosis versus those who were offered genetic screening. In three families, asymptomatic mutation carriers were identified and monitored for clinical worsening. In two families, screening was not done and affected family members presented with advanced disease.
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Objectives: Infants and young children awaiting lung transplantation present challenges that often preclude successful extracorporeal membrane oxygenation support as a bridge to transplantation. Instability of neck cannulas often results in the need for intubation, mechanical ventilation, and muscle relaxation creating a worse transplant candidate. With the use of Berlin Heart EXCOR cannulas (Berlin Heart, Inc) in both venoarterial and venovenous central cannulation configurations, 5 pediatric patients were successfully bridged to lung transplant. Methods: We performed a single-center retrospective case review of central extracorporeal membrane oxygenation cannulation used as a bridge to lung transplantation cases performed at Texas Children's Hospital between 2019 and 2021. Results: Six patients, 2 with pulmonary veno-occlusive disease (15-month-old male and 8-month-old male), 1 with ABCA3 mutation (2-month-old female), 1 with surfactant protein B deficiency (2-month-old female), 1 with pulmonary arterial hypertension in the setting of D-transposition of the great arteries after repair as a neonate (13-year-old male), and 1 with cystic fibrosis and end-stage lung disease, were supported for a median of 56.3 days on extracorporeal membrane oxygenation while awaiting transplantation. All patients were extubated after initiation of extracorporeal membrane oxygenation, participating in rehabilitation until transplant. No complications due to central cannulation and use of the Berlin Heart EXCOR cannulas were observed. One patient with cystic fibrosis developed fungal mediastinitis and osteomyelitis resulting in discontinuation of mechanical support and death. Conclusions: Novel use of Berlin Heart EXCOR cannulas for central cannulation eliminates the problem of cannula instability allowing extubation, rehabilitation, and bridge to lung transplant for infants and young children.
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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.
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Fibrosis Quística , Trasplante de Pulmón , Staphylococcus aureus Resistente a Meticilina , Adolescente , Niño , Fibrosis Quística/complicaciones , Fibrosis Quística/epidemiología , Fibrosis Quística/cirugía , Humanos , Pulmón , Trasplante de Pulmón/efectos adversos , Estudios Retrospectivos , Factores de Riesgo , Receptores de Trasplantes , Estados Unidos/epidemiologíaRESUMEN
Diffuse lung disease in early childhood due to mutations in the filamin A gene has been recently reported. Clinical outcomes vary among individuals indicating variability in phenotype but a substantial proportion of reported cases in early life have ended up in death or lung transplantation. We recently encountered a school-aged child in whom the diagnosis of a filamin A mutation was delayed and the natural history of emphysematous lung disease was altered by serial lung volume reduction surgeries. She eventually underwent a bilateral lung transplant and we report the natural history of her disease and treatments applied herein.
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Enfermedades Pulmonares , Neumonectomía , Niño , Femenino , Filaminas/genética , Humanos , Enfermedades Pulmonares/genética , Enfermedades Pulmonares/cirugía , Mutación , FenotipoRESUMEN
Lung transplant referral guidelines for individuals with cystic fibrosis (CF) were published recently. Most of the recommendations focus on the specific indications and barriers to transplantation in adults with CF. Although the number of children with CF and end-stage lung disease continues to decrease, the specific issues related to pediatric patients merit further elucidation. We address each recommendation from the recent publication with a pediatric perspective. Furthermore, we note some significant differences between the practice and policy related to lung transplantation between Canada and the United States.
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Trasplante de Pulmón , Derivación y Consulta/estadística & datos numéricos , Canadá , Niño , Consenso , Fibrosis Quística/terapia , Fundaciones , Humanos , Guías de Práctica Clínica como Asunto , Estados UnidosRESUMEN
Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a clinical picture that could be confusing to clinicians. We describe three subsets of PGA, including its association with the histologic marker of pulmonary interstitial glycogenosis, and its common association with pulmonary hypertension. We propose a new approach to what we consider an increasingly broad array of different disease entities.
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Displasia Broncopulmonar/diagnóstico por imagen , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Pulmón/anomalías , Mutación , Niño , Preescolar , Femenino , Filaminas/genética , Enfermedad del Almacenamiento de Glucógeno/complicaciones , Humanos , Hipertensión Pulmonar/complicaciones , Lactante , Recién Nacido , Pulmón/patología , Enfermedades Pulmonares Intersticiales/patología , Masculino , Alveolos Pulmonares/patología , Anomalías del Sistema Respiratorio/complicaciones , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Despite successes in lung transplantation, with infection as the leading cause of death in the first year following lung transplantation, there remains a lag in survival compared with other solid organ transplants. Infections that occur early after transplantation may impact short- and long-term outcomes in pediatric lung transplant recipients (LTRs). METHODS: We performed a retrospective review of pediatric LTRs at a large quaternary-care hospital from January 2009 to March 2016 to evaluate both epidemiologic features of infection in the first 30 days post-transplantation and mortality outcomes. The 30 days were divided into early (0-7 days) and late (8-30 days) periods. RESULTS: Among the 98 LTRs, there were 51 episodes of infections. Cystic fibrosis (CF) was associated with early bacterial infections (P = .004) while non-CF was associated with late viral (P = .02) infections. Infection after transplantation was associated with worse survival by Kaplan-Meier analysis (P value log rank test = .007). Viral infection in the late period was significantly associated with 3-year mortality after multivariable analysis (P = .02). CONCLUSIONS: Infections in pediatric LTRs were frequent in the first 30 days after transplant, despite perioperative antimicrobial coverage. The association of 3-year mortality with late viral infections suggests a possible important role in post-transplant lung physiology and graft function. Understanding the epidemiology of early post-lung transplant infections can help guide post-operative management and interventions to reduce their incidence and the early- and long-term impact in this population.
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Infecciones Bacterianas , Fibrosis Quística , Trasplante de Pulmón , Niño , Humanos , Incidencia , Trasplante de Pulmón/efectos adversos , Estudios RetrospectivosRESUMEN
BACKGROUND: The global COVID-19 pandemic was particularly concerning for the pediatric pulmonary hypertension (PH) population due to immature immune systems and developmental comorbidities. This study aims to describe a single-center experience of pediatric PH patients diagnosed with COVID-19 disease. METHODS: A retrospective cohort study of all pediatric patients followed by the PH Center at Texas Children's Hospital diagnosed with COVID-19 infection from April 2020 to February 2021. RESULTS: We identified 23 patients with a median age of 58 months (interquartile range [IQR]: 25-75th, 21-132 months), 48% being Hispanics. Eight patients (35%) required hospitalization; median length of stay was 6 days (IQR: 25-75th, 5-8 days). Only three of these eight patients required increased respiratory support. Targeted PH therapy was escalated in four patients (two in dual and two in triple therapy). There was one mortality in a patient with failing Fontan physiology. Ninety-one percent of patients have had post-COVID outpatient follow-up, median of 101 days (IQR: 25-75th, 50-159 days) from diagnosis. Of the five patients with 6 min walk test (6MWT) data, three (60%) children walked less distance, median of -12 m (IQR: 25-75th, -12 to +49 m) compared to pre-COVID testing. Postinfection pulmonary function testing (PFT) was notable for decrease in predicted forced vital capacity (FVC; median -6%, range -11% to +6%) and forced expiratory volume in one second (FEV1; median -14%, range -12% to -18%) in 75% of the patients with PFT data. CONCLUSION: In our institution, COVID-19 was found more frequently in Hispanics and associated with low mortality.
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COVID-19 , Hipertensión Pulmonar , Prueba de COVID-19 , Niño , Preescolar , Humanos , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/etiología , Pandemias , Estudios Retrospectivos , SARS-CoV-2RESUMEN
KL-6 is a glycoprotein expressed by pulmonary epithelial cells, and its serum level has been used as a marker of disease activity in a variety of respiratory illnesses. Previously, we showed that KL-6 was elevated in lung transplant recipients diagnosed with BOS. In this study, we followed serum KL-6 levels and lung functions prospectively in lung transplant recipients who were within the first five-yr post-transplant and had no evidence of BOS at the time of study entry. Mean peak KL-6 levels were 596.16 ± 309.32 U/mL in the nine recipients who developed BOS compared to 352.41 ± 140.68 in 36 recipients who did not (p = 0.05). Six of the nine patients with BOS had an absolute rise in KL-6 above baseline level >200 U/mL compared to two of the 37 who had the same increase in KL-6 but did not develop BOS. Using the 200 U/mL elevation of KL-6 from baseline as a threshold for a positive test would produce a sensitivity of 67%, specificity of 95%, PPV of 75%, and a NPV of 92%. In addition, mean KL-6 levels of patients during acute rejection were not significantly elevated compared to the prerejection mean KL-6 levels (p = 0.71). We conclude that serum KL-6 is a relatively specific marker of BOS in lung transplant recipients.