Isavuconazole use and TDM in real-world pediatric practice.

Isavuconazole (ISA) is approved for treating invasive aspergillosis and mucormycosis in adults, but its use in children remains off-label. We report on the use of ISA in real-world pediatric practice with 15 patients receiving ISA for treatment of invasive fungal infections. Therapeutic drug monitoring (TDM) was performed in all patients, with 52/111 (46.8%) Ctrough determinations out of range, thus supporting the need for TDM in children, especially those receiving extracorporeal membrane oxygenation (ECMO).

CMV hyperimmune globulin as salvage therapy for recurrent or refractory CMV infection in children undergoing hematopoietic stem cell transplantation.

Cytomegalovirus (CMV) is a major cause of allogeneic hematopoietic stem cell transplant (HSCT)-related morbidity and mortality. Treatment failure continues to be a major issue in patients with CMV infection due to both drug resistance and intolerance. This single-center brief retrospective analysis of a case series aims to investigate the safety and efficacy of CMV-hyperimmune globulin as salvage therapy for CMV infection in children undergoing HSCT. Fifteen pediatric patients received human CMV-specific immunoglobulin (CMVIG) between July 2018 and December 2021 as a salvage therapy for refractory or recurrent CMV infection. At the time of CMVIG prescription, eight children presented with recurrent CMV infection and seven with refractory CMV infection. The overall response rate was 67% at 50 days from the CMVIG administration [95% confidence interval (CI): 44-88]. Overall survival (OS) from CMVIG administration at 100 days was 87% (95% CI: 56-96), and OS from HSCT at 1 year was 80% (95% CI: 50-93). Four patients died, three unrelated to CMV infection and one due to CMV pneumonia. CMVIG as salvage therapy was well tolerated, and no infusion-related adverse events were observed.

Consensus document on the management of febrile neutropenia in paediatric haematology and oncology patients of the Spanish Society of Pediatric Infectious Diseases (SEIP) and the Spanish Society of Pediatric Hematology and Oncology (SEHOP).

Febrile neutropenia is one of the main infectious complications experienced by paediatric patients with blood or solid tumours, which, despite the advances in diagnosis and treatment, are still associated with a significant morbidity and mortality. These patients have several risk factors for infection, chief of which are chemotherapy-induced neutropenia, the disruption of cutaneous and mucosal barriers and the use of intravascular devices. Early diagnosis and treatment of febrile neutropenia episodes based on the patient's characteristics is essential in patients with blood and solid tumours to improve their outcomes. Therefore, it is important to develop protocols in order to optimise and standardise its management. In addition, the rational use of antibiotics, with careful adjustment of the duration of treatment and antimicrobial spectrum, is crucial to address the increase in antimicrobial drug resistance. The aim of this document, developed jointly by the Spanish Society of Pediatric Infectious Diseases and the Spanish Society of Pediatric Hematology and Oncology, is to provide consensus recommendations for the management of febrile neutropenia in paediatric oncology and haematology patients, including the initial evaluation, the stepwise approach to its treatment, supportive care and invasive fungal infection, which each facility then needs to adapt to the characteristics of its patients and local epidemiological trends.

Documento de consenso de manejo de neutropenia febril en el paciente pediátrico oncohematológico de la Sociedad Española de Infectología Pediátrica (SEIP) y la Sociedad Española de Hematología y Oncología Pediátrica (SEHOP) / Consensus document on the management of febrile neutropenia in paediatric haematology and oncology patients of the Spanish Society of Pediatric Infectious Diseases (SEIP) and the Spanish Society of Pediatric Hematology and Oncology (SEHOP)

La neutropenia febril es una de las principales complicaciones infecciosas que sufren los pacientes pediátricos oncohematológicos, y a pesar los avances en diagnóstico y tratamiento, siguen condicionando una mortalidad y morbilidad significativa. Estos pacientes agrupan una serie de factores de riesgo de infección, donde destaca la neutropenia asociada a quimioterapia, la disrupción de barreras cutáneo-mucosas y el uso de dispositivos intravasculares. El abordaje diagnóstico y terapéutico precoz de los episodios de neutropenia febril en los pacientes oncohematológicos, ajustado a las características individuales de cada paciente, es fundamental para mejorar su pronóstico. Por ello, diseñar protocolos de abordaje, que sistematicen su atención, permite optimizar y homogeneizar su abordaje. Además, racionalizar el uso de los antimicrobianos, ajustando la duración y el espectro de los mismos, es crucial para hacer frente al incremento de resistencias a antimicrobianos. El objetivo de este documento, elaborado entre la Sociedad Española de Infectología Pediátrica y la Sociedad Española de Hematología y Oncología Pediátrica, es dar recomendaciones de consenso sobre el manejo de la neutropenia febril en el paciente oncohematológico, respecto al abordaje inicial, terapia secuencial y de soporte e infección fúngica invasiva, que cada centro debe adaptar a las características de sus pacientes y epidemiología local. (AU)

Febrile neutropenia is one of the main infectious complications experienced by paediatric patients with blood or solid tumours, which, despite the advances in diagnosis and treatment, are still associated with a significant morbidity and mortality. These patients have several risk factors for infection, chief of which are chemotherapy-induced neutropenia, the disruption of cutaneous and mucosal barriers and the use of intravascular devices. Early diagnosis and treatment of febrile neutropenia episodes based on the patient's characteristics is essential in patients with blood and solid tumours to improve their outcomes. Therefore, it is important to develop protocols in order to optimise and standardise its management. In addition, the rational use of antibiotics, with careful adjustment of the duration of treatment and antimicrobial spectrum, is crucial to address the increase in antimicrobial drug resistance. The aim of this document, developed jointly by the Spanish Society of Pediatric Infectious Diseases and the Spanish Society of Pediatric Hematology and Oncology, is to provide consensus recommendations for the management of febrile neutropenia in paediatric oncology and haematology patients, including the initial evaluation, the stepwise approach to its treatment, supportive care and invasive fungal infection, which each facility then needs to adapt to the characteristics of its patients and local epidemiological trends. (AU)

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis.

BACKGROUND: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin-tazobactam for this purpose. OBJECTIVE: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. MATERIALS AND METHODS: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010-2019). RESULTS: 113 procedures in 37 patients, median age 4 years (IQR 1-8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. CONCLUSIONS: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis / Complicaciones infecciosas de la manipulación no quirúrgica de las vías biliares en pacientes pediátricos. Función de la profilaxis antibiótica

Background: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin–tazobactam for this purpose. Objective: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. Materials and methods: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010–2019). Results: 113 procedures in 37 patients, median age 4 years (IQR 1–8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. Conclusions: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.(AU)

Antecedentes: Las infecciones relacionadas con la manipulación no quirúrgica de las vías biliares (MNQVB) son acontecimientos frecuentes, a pesar de la profilaxis antibiótica periprocedimiento (PAP). Desde junio de 2017, nuestro protocolo local indica una pauta de 24 h de piperacilina/tazobactam por vía intravenosa para este fin. Objetivo: El objetivo era describir la incidencia y las características de las infecciones pediátricas relacionadas con la MNQVB, definir los factores de riesgo para su desarrollo y analizar el cumplimiento de nuestro protocolo de PAP. Materiales y métodos: Se recogieron datos epidemiológicos, clínicos y microbiológicos en procedimientos consecutivos de MNQVB realizados en pacientes pediátricos (< 18 años) en nuestro centro (2010-2019). Resultados: Se incluyeron 113 procedimientos en 37 pacientes, con una mediana de edad de 4 años (RIC 1-8). Las principales enfermedades subyacentes fueron atresia biliar (32%) y cáncer (14%). El 68% se había sometido a un trasplante de hígado y el 70% a una hepaticoyeyunostomía. En 44 procedimientos (39%), la intervención se realizó durante el transcurso de la infección y se mantuvo el tratamiento antibiótico recetado previamente. En los otros 69, la PAP estaba indicada específicamente para la MNQVB; la eficacia de los antibióticos aumentó del 35 al 100% después de junio de 2017. En total, se produjeron 32 infecciones relacionadas con la MNQVB (28%), principalmente en las primeras 24 h posteriores al procedimiento (72%); no se produjo ninguna muerte. Los patógenos causantes fueron bacilos gramnegativos (64%), cocos grampositivos (28%) y Candida spp. (8%). Los principales factores de riesgo relacionados fueron la hepaticoyeyunostomía, la obstrucción biliar y el trasplante de hígado.(AU)

Influenza-Associated Disseminated Aspergillosis in a 9-Year-Old Girl Requiring ECMO Support.

A previously healthy 9-year-old girl developed fulminant myocarditis due to severe influenza A infection complicated with methicillin-resistant Staphylococcus aureus pneumonia, requiring extracorporeal membrane oxygenation (ECMO) support. Twelve days after admission, Aspergillus fumigatus was isolated in tracheal aspirate, and 12 h later she suddenly developed anisocoria. Computed tomography (CT) of the head showed fungal brain lesions. Urgent decompressive craniectomy with lesion drainage was performed; histopathology found hyphae in surgical samples, culture-positive for Aspergillus fumigatus (susceptible to azoles, echinocandins, and amphotericin B). Extension workup showed disseminated aspergillosis. After multiple surgeries and combined antifungal therapy (isavuconazole plus liposomal amphotericin B), her clinical course was favorable. Isavuconazole therapeutic drug monitoring was performed weekly. Extensive immunological study ruled out primary immunodeficiencies. Fluorine-18 fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) follow-up showed a gradual decrease in fungal lesions. Influenza-associated pulmonary aspergillosis is well-recognized in critically ill adult patients, but pediatric data are scant. Clinical features described in adults concur with those of our case. Isavuconazole, an off-label drug in children, was chosen because our patient had severe renal failure. To conclude, influenza-associated pulmonary aspergillosis is uncommon in children admitted to intensive care for severe influenza, but pediatricians should be highly aware of this condition to enable prompt diagnosis and treatment.

Voriconazole Use in Children: Therapeutic Drug Monitoring and Control of Inflammation as Key Points for Optimal Treatment.

Voriconazole plasma concentrations (PC) are highly variable, particularly in children. Dose recommendations in 2-12-year-old patients changed in 2012. Little data on therapeutic drug monitoring (TDM) after these new recommendations are available. We aimed to evaluate voriconazole monitoring in children with invasive fungal infection (IFI) after implementation of new dosages and its relationship with safety and effectiveness. A prospective, observational study, including children aged 2-12 years, was conducted. TDM was performed weekly and doses were changed according to an in-house protocol. Effectiveness, adverse events, and factors influencing PC were analysed. A total of 229 PC from 28 IFI episodes were obtained. New dosing led to a higher rate of adequate PC compared to previous studies; still, 35.8% were outside the therapeutic range. In patients aged < 8 years, doses to achieve therapeutic levels were higher than recommended. Severe hypoalbuminemia and markedly elevated C-reactive protein were related to inadequate PC. Therapeutic PC were associated with drug effectiveness and safety. Higher doses in younger patients and a dose adjustment protocol based on TDM should be considered. Voriconazole PC variability has decreased with current updated recommendations, but it remains high and is influenced by inflammatory status. Additional efforts to control inflammation in children with IFI should be encouraged.

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis.

BACKGROUND: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin-tazobactam for this purpose. OBJECTIVE: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. MATERIALS AND METHODS: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010-2019). RESULTS: 113 procedures in 37 patients, median age 4 years (IQR 1-8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. CONCLUSIONS: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.

Calidad de prescripción de la antibioterapia prolongada en pediatría. Impacto de las intervenciones programas PROA / Prescription quality of prolonged antibiotherapy in pediatrics. Impact of ASP program interventions

Introducción: Adecuar la duración del tratamiento antibiótico es uno de los puntos claves de los programas de optimización del uso de antimicrobianos (PROA) dada la relación entre el riesgo de aparición de resistencias con los días de exposición a antimicrobianos. Métodos: Monitorización de tratamientos antibióticos por vía intravenosa de > 7 días de duración en el Hospital Infantil Vall d’Hebron, Barcelona, mediante cortes transversales semanales durante 24 semanas, con recomendaciones posteriores a los prescriptores para adecuar su uso. Resultados: Se realizaron 190 revisiones de 146 tratamientos antibióticos prolongados, prescritos en 81 pacientes. El 78,7% de prescripciones fueron adecuadas. Se realizaron 36 intervenciones sobre las prescripciones inadecuadas, con 52,7% de adaptación a la recomendación. Se optimizaron 19 tratamientos (14 suspendidos, 5 desescalados) reduciendo su duración un 8,75%. Conclusiones: La intervención activa del grupo PROA-NEN permite mejorar la adecuación antibiótica, reduciendo los tratamientos innecesariamente prolongados, especialmente en ámbitos con mayor margen de mejora.(AU)

Introduction: the duration adequacy of antibiotic regimens is one of the key points of Antimicrobial Stewardship Programs (ASP) given the relationship between the risk of resistance and days of exposure to antimicrobials. Methods: monitoring activities of intravenous antibiotics longer than 7 days at Hospital Infantil Vall d’Hebron, Barcelona, by reviewing data over a 34-weeks period from weekly cross-sectional analysis, followed by recommendations to prescribers to adapt their use. Results: a total of 81 patients with 146 prolonged intravenous antibiotic treatments (78.8% of prescriptions were adequate) were reviewed. A total of 190 revisions were performed. 36 interventions on inappropriate prescriptions were carried out (52.7% of adherence to recommendation). Nineteen treatments were optimized (14 suspended, 5 de-escalated) reducing their duration by 8.75%. Conclusions: active intervention of ASP group is an effective tool to improve antibiotic optimization, reducing unnecessarily prolonged treatments, mainly on these areas with a greater range of improvement.(AU)

Nen de 9 anys trasplantat cardíac I renal amb lesió dolorosa a l'engonal / No disponible

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Antifungal stewardship in a tertiary care paediatric hospital: the PROAFUNGI study.

BACKGROUND: The increasing use of antifungal drugs (AF) in children and the concern for related adverse events and costs has led to the development of specific AF stewardship programmes (AFS). Studies in adult patients have shown improvements in AF prescription and usage after implementation, but paediatric data are scant. The aim of this PROAFUNGI study was to describe the use and appropriateness of AF in a high complexity paediatric centre. METHODS: Observational, prospective, single-centre, modified point-prevalence study (11 surveys, July-October 2018), including paediatric (< 18 years) patients receiving at least one systemic AF. Prescriptions were evaluated by the AFS team. RESULTS: The study included 119 prescriptions in 55 patients (53% males, median age 8.7 years [IQR 2.4-13.8]). The main underlying condition was cancer (45.5% of patients; HSCT in 60% of them); and the first indication for AF was prophylaxis (75 prescriptions, 63.2%). Liposomal amphotericin B was used most commonly (46% prescriptions), mainly as prophylaxis (75%). Among the 219 evaluations, 195 (89%) were considered optimal. The reason for non-optimal prescriptions was mostly lack of indication (14/24), especially in critical patients with ventricular assist devices. The use of AF without paediatric approval accounted for 8/24 inappropriate prescriptions. CONCLUSIONS: A high rate of AF appropriateness was found for the children's hospital as a whole, in relation with a well-established AFS. Nonetheless, the identification of specific areas of improvement should guide future actions of the AFS team, which will focus mainly on prophylaxis in critically ill patients receiving circulatory assistance and the use of non-approved drugs in children.

Prescription quality of prolonged antibiotherapy in pediatrics. Impact of ASP program interventions. / Calidad de prescripción de la antibioterapia prolongada en pediatría. Impacto de las intervenciones programas PROA.

INTRODUCTION: the duration adequacy of antibiotic regimens is one of the key points of Antimicrobial Stewardship Programs (ASP) given the relationship between the risk of resistance and days of exposure to antimicrobials. METHODS: monitoring activities of intravenous antibiotics longer than 7 days at Hospital Infantil Vall d'Hebron, Barcelona, by reviewing data over a 34-weeks period from weekly cross-sectional analysis, followed by recommendations to prescribers to adapt their use. RESULTS: a total of 81 patients with 146 prolonged intravenous antibiotic treatments (78.8% of prescriptions were adequate) were reviewed. A total of 190 revisions were performed. 36 interventions on inappropriate prescriptions were carried out (52.7% of adherence to recommendation). Nineteen treatments were optimized (14 suspended, 5 de-escalated) reducing their duration by 8.75%. CONCLUSIONS: active intervention of ASP group is an effective tool to improve antibiotic optimization, reducing unnecessarily prolonged treatments, mainly on these areas with a greater range of improvement.

Adverse Events Associated With New Injectable-Free Multidrug-Resistant Tuberculosis Drug Regimens / Efectos adversos asociados a los nuevos tratamientos farmacológicos sin inyectables contra la tuberculosis multirresistente

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Low-dose liposomal amphotericin B for antifungal prophylaxis in paediatric allogeneic haematopoietic stem cell transplantation.

BACKGROUND: Primary antifungal prophylaxis in paediatric allogeneic HSCT recipients is mainly based on azoles, which can have related toxicity and drug interactions. Low-dose liposomal amphotericin B (L-AmB) is an attractive intravenous alternative because of its low toxicity and lower risk of interactions. OBJECTIVES: To evaluate the effectiveness and safety of L-AmB (1 mg/kg/day) for primary antifungal prophylaxis in pre-engraftment paediatric HSCT patients. PATIENTS AND METHODS: Retrospective, observational study including all consecutive patients aged ≤18 years who underwent HSCT and received antifungal prophylaxis with intravenous L-AmB (1 mg/kg/day, from day -1 to 48 h before discharge) between January 2012 and December 2016. RESULTS: In total, 125 HSCT procedures in 118 patients were included, median age 7.2 years (IQR 4.2-11.5). Haematological malignancies were the main underlying condition (63.6%), and 109 (87.2%) were considered at high risk for invasive fungal infection (IFI). Ten patients (7.7%), all high risk, developed breakthrough IFI (three Candida spp., seven invasive mould infections) and tended to have higher overall mortality. The only statistically significant risk factor for IFI was cytomegalovirus co-infection. Adverse events, all grade I, occurred in 25 (20%), requiring L-AmB withdrawal in one case. Overall survival at 30 days was 99.2%. At study completion, one patient had died of IFI. CONCLUSIONS: The incidence of breakthrough IFI was comparable to that of previous reports, with a very low rate of significant toxicity. Thus, prophylactic L-AmB may be a safe, effective option for antifungal prophylaxis in the pre-engraftment phase for children undergoing HSCT, even those at high risk.

Health-Related Quality of Life and Multidimensional Fatigue Scale in Children with Primary Immunodeficiencies.

PURPOSE: Patients with primary immunodeficiency disease (PID) have an increased risk of experiencing physical activity limitations, social difficulties, and psychological problems due to their chronic condition. Evaluation of their health-related quality of life (HRQOL) and fatigue is crucial in these patients to help understand their complex disease and provide adequate medical care. METHODS: In this study, we evaluated HRQOL and fatigue in pediatric and young adult patients with PID attending our center. Participants completed the Pediatric Quality of Life Inventory (PedsQL), version 4.0, and the PedsQL multidimensional fatigue module, standard version. RESULTS: Fifty-three PID patients were recruited (age range: 2-23 years). The mean HRQOL score obtained was 66.61 (SD: 18.73) out of 100, and the emotional and work/school dimensions were the ones most highly affected. There were no significant differences in reported quality of life between patients and their caregivers. The mean patient-reported fatigue value was 68.81 (SD: 17.80) out of 100, and the rest-related dimension was the one most highly affected. In the caregivers' assessment, general fatigue was the most highly affected dimension. CONCLUSIONS: The results of this study show that quality of life is poor and fatigue measures are considerably increased in our young adult and pediatric patients with PIDs. These findings can indicate areas requiring more intensive interventions, and they will serve as a basis for comparison of future results.

Pneumocystis jirovecii pneumonia in children. A retrospective study in a single center over three decades / Neumonía por Pneumocystis jirovecii en niños. Estudio retrospectivo unicéntrico de tres décadas

INTRODUCTION: Pneumocystis jirovecii pneumonia (PJP) is a life-threatening condition in immunocompromised children. Our aim is to analyze the epidemiologic and clinical characteristics of PJP cases in our setting, describing the prognosis and related risk factors. METHODS: Retrospective study including all pediatric patients (≤ 18 years) with PJP admitted to our hospital (January 1989-December 2016). Case definition: patient with acute pneumonitis and P.jirovecii detection in bronchoalveolar lavage or tracheal aspirate using methenamine silver or direct antibody fluorescence staining, or Real-Time Polymerase Chain Reaction. RESULTS: Twenty-five cases (0.9 cases/year) were identified. Median age was 2.2 years (interquartile range: 0.5-12.3), 64% were male, and 12% were receiving appropriate antimicrobial prophylaxis. Cytomegalovirus coinfection was detected in 26% cases. The most common underlying diseases were primary immunodeficiencies (36%) and 16% were human immunodeficiency virus (HIV)-infected children. Eighteen were admitted to the pediatric intensive care unit (PICU) and overall 30-day mortality was 20% (31.25% in HIV non-infected vs 0% in HIV-infected patients; OR: 0.33, 95% CI: 0.02-7.24, p = 0.55). Clinical outcome was worse in girls and those patients requiring adjuvant steroid therapy. HIV non-infected patients, higher initial LDH, younger age and shorter time elapsed between diagnosis of PJP and the underlying disease were identified as risk factors to be admitted to the PICU (p = 0.05, p = 0.026, p = 0.04 and p = 0.001 respectively). CONCLUSION: Accompanying the widespread use of combined antiretroviral therapy, PJP has been diagnosed almost exclusively in HIV non-infected children at our institution. Moreover, significant higher morbidity rates associated with PJP are seen in this group of patients

INTRODUCCIÓN: La neumonía por Pneumocystis jirovecii (PJP) es una enfermedad potencialmente letal en niños inmunocomprometidos. Nuestro objetivo es analizar las características epidemiológicas y clínicas de la PJP, describiendo el pronóstico y los factores de riesgo. MÉTODOS: Estudio retrospectivo (enero 1989-diciembre 2016) de pacientes pediátricos (≤ 18 años) con PJP. Definición de caso: paciente con neumonitis aguda y detección de P. jirovecii en lavado broncolaveolar o aspirado traqueal usando tinción con plata-metenamina o inmunofluorescencia directa, o reacción en cadena de polimerasa en tiempo real. RESULTADOS: Se identificaron veinticinco casos (0,9 casos/año); edad mediana: 2,2 años (rango intercuartílico: 0,5-12,3), 64% de sexo masculino, y 12% bajo profilaxis anti-PJP. La coinfección por citomegalovirus se demostró en el 26%. Las enfermedades subyacentes más frecuentes fueron las inmunodeficiencias primarias (36%) y el 16% estaban infectados por el VIH. Dieciocho ingresaron en Cuidados Intensivos Pediátricos (UCIP) y la mortalidad global a los 30 días fue del 20% (31,25% en VIH- vs 0% VIH + ; OR: 0,33 95%CI 0,02-7,24 p = 0,55). El pronóstico fue peor en niñas y en aquellos que recibieron tratamiento adyuvante con corticoides. Se identificaron como factores de riesgo para ingreso en UCIP la ausencia de infección por VIH, valores iniciales elevados de LDH, menor edad y un período más corto entre el diagnóstico de PJP y la enfermedad subyacente (p = 0,05, p = 0,026, p = 0,04 y p = 0,001, respectivamente). CONCLUSIONES: Tras la aplicación generalizada de la terapia antirretroviral, la PJP se diagnostica casi exclusivamente en niños no infectados por el VIH en los que, además, se identificó una mayor morbilidad