Five-Year Evaluation of the PROA-NEN Pediatric Antimicrobial Stewardship Program in a Spanish Tertiary Hospital.

Introduction: Actions to reduce and optimize antimicrobial use are crucial in the management of infectious diseases to counteract the emergence of short- and long-term resistance. This is particularly important for pediatric patients due to the increasing incidence of serious infections caused by resistant bacteria in this population. The aim of this study was to evaluate the impact of a pediatric antimicrobial stewardship program (PROA-NEN) implemented in a Spanish tertiary hospital by assessing the use of systemic antimicrobials, clinical indicators, antimicrobial resistance, and costs. Methods: In this quasi-experimental, single-center study, we included pediatric patients (0-18 years) admitted to specialized pediatric medical and surgical units, as well as pediatric and neonatal intensive care units, from January 2015 to December 2019. The impact of the PROA-NEN program was assessed using process (consumption trends and prescription quality) and outcome indicators (clinical and microbiological). Antibiotic prescription quality was determined using quarterly point prevalence cross-sectional analyses. Results: Total antimicrobial consumption decreased during the initial three years of the PROA-NEN program, followed by a slight rebound in 2019. This decrease was particularly evident in intensive care and surgical units. Antibiotic use, according to the WHO Access, Watch and Reserve (AWaRe) classification, remained stable during the study period. The overall rate of appropriate prescription was 83.2%, with a significant increase over the study period. Clinical indicators did not substantially change over the study period. Direct antimicrobial expenses decreased by 27.3% from 2015 to 2019. Conclusions: The PROA-NEN program was associated with reduced antimicrobial consumption, improved appropriate use, and decreased costs without compromising clinical and/or microbiological outcomes in patients.

Antifungal Drug Usage in European Neonatal Units: A Multicenter Weekly Point Prevalence Study.

BACKGROUND: Data on antifungal prescribing in neonatal patients are limited to either single-center or single-country studies or to 1-day recording. Therefore, we assessed antifungal longitudinal usage in neonatal units (NUs) within Europe. METHODS: CALYPSO, a prospective weekly point prevalence study on antifungal drug usage in NUs in 18 hospitals (8 European countries), was conducted in 2020 during a 12-week period. All patients receiving systemic antifungals were included. Ward demographics were collected at the beginning; ward and patient data including indication, risk factors and antifungal regimen were weekly collected prospectively. RESULTS: Among 27 participating NUs, 15 (56%) practiced antifungal prophylaxis for neonates with birth weight <1000 g or <1500 g and additional risk factors. In total, 174 patients received antifungals with a median frequency per week of 10.5% ranging from 6.9% to 12.6%. Indication for antifungal prescribing was prophylaxis in 135/174 (78%) courses and treatment in 22% [39 courses (69% empirical, 10% preemptive, 21% targeted)]. Fluconazole was the most frequent systemic agent used both for prophylaxis (133/135) and treatment (15/39, 39%). Among neonates receiving prophylaxis, the most common risk factors were prematurity (119/135, 88%), mechanical ventilation (109/135, 81%) and central vascular catheters (89/135, 66%). However, gestational age <28 weeks was only recorded in 55/135 (41%) courses and birth weight <1000 g in 48/135 (35%). Most common reason for empirical treatment was late-onset sepsis; all 8 targeted courses were prescribed for invasive candidiasis. CONCLUSION: Antifungal usage in European NUs is driven by prophylaxis and empirical treatment with fluconazole being the most prescribed agent for both indications.

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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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.