RESUMO
The perception of facilitators and barriers to referral to pediatric palliative care (PPC) is a widely studied phenomenon, with scarce information in Latin America. OBJECTIVE: to adapt a survey on the perception of facilitators and barriers to PPC referral and evaluation. SUBJECTS AND METHOD: electronic survey with cultural adaptation with translation-retrotranslation in pediatricians and pediatric subspecialists of 3 tertiary centers in Santiago. The survey consisted of 4 sections and 51 questions, corresponding to sociodemographic data (including self-perception of spirituality and religiosity), training and clinical practice of the respondent in PPC, and facilitators and barriers to referral to PPC. RESULTS: 146 pediatricians were invited and 78 surveys were obtained (response rate: 53.4%). The median age was 42 years and 11.5 years of professional practice. The majority corresponded to pediatricians without subspecialty training (n = 34; 43.6%). Twenty-three (29.5%) of the participants received PPC training, which was perceived as insufficient in 17 (74%) cases; there were also no differences in PPC training when comparing pediatricians with or without subspecialty training. Ninety-five percent of the respondents agreed with the perceived benefit of early referral to PPC for patients with life-threatening pathologies, regardless of their diagnosis, although only 47.7% stated that they had made a referral to a PPC team. The emotional relationship with the patients and their families was perceived as a barrier by pediatricians in those subspecialists susceptible to PPC compared with those who were not (20% vs. 50%; p = 0.03). CONCLUSION: A significant deficit in PPC training was detected. There were no differences in facilitators and barriers between pediatricians and subspecialties susceptible to PPC. Specialties not accustomed to PPC-susceptible patients may be affected by emotional factors in their decisions.
Assuntos
Cuidados Paliativos , Encaminhamento e Consulta , Humanos , Criança , Adulto , PediatrasRESUMO
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a severe syndrome, potentially lethal, with a pathological activation of the immune system and an extreme hyperinflammatory response. The etiology is classified in primary HLH (familiar or genetic) and secondary (infectious, oncological, and rheumatological diseases). AIM: To analyze clinical and laboratory characteristics, treatment, and follow-up rates in pediatric patients with HLH. METHODS: A pediatric cohort of patients with HLH diagnosis attending in a tertiary hospital between January 2000 to February 2019 was analysed. RESULTS: 23 hospitalized patients were recruited with a median of 36 months of age. The most frequent clinical and laboratory findings were fever, cytopenias, and hyperferritinemia. The most frequent aetiologies were infectious (Epstein Barr virus and citomegalovirus) and rheumatological diseases. The global mortality was 35%, there was no significant difference between etiologies. DISCUSSION: Considering the high mortality of HLH it is very important to have a high grade of suspicion that allows treating at an early stage. It would be important to determine clinical and laboratory predictors in multicentric studies.
Assuntos
Infecções por Vírus Epstein-Barr , Linfo-Histiocitose Hemofagocítica , Criança , Seguimentos , Herpesvirus Humano 4 , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/etiologia , Centros de Atenção TerciáriaRESUMO
INTRODUCCIÓN: La linfohistiocitosis hemofagocítica (HLH en inglés) es un síndrome clínico grave, potencialmente fatal, caracterizado por una activación patológica del sistema inmune y una respuesta hiperinflamatoria extrema. Según su etiología se clasifica en primario (genético o familiar) y secundario (gatillado por causas infecciosas, oncológicas o reumatológicas). OBJETIVOS: Describir y analizar las características clínicas y laboratorio, tratamiento recibido y seguimiento en pacientes pediátricos con diagnóstico de HLH. PACIENTES Y MÉTODOS: Se describió una cohorte pediátrica en pacientes hospitalizados con diagnóstico de HLH en un centro terciario universitario entre enero de 2000 y febrero de 2019. RESULTADOS: Se reclutaron 23 pacientes pediátricos con una mediana de edad de 36 meses. Los hallazgos clínicos y de laboratorio más frecuentes fueron fiebre, citopenias e hiperferritinemia. La etiología más frecuente fue infecciosa (virus Epstein Barr/citomegalovirus) e inmunológica/reumatológica. La mortalidad global fue de 35%, sin diferencias significativas según etiología. DISCUSIÓN: Dada su alta mortalidad es relevante un alto índice de sospecha que permita instaurar terapia de forma precoz. Son necesarios estudios multicéntricos para determinar predictores clínicos y de laboratorio con valor pronóstico.
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a severe syndrome, potentially lethal, with a pathological activation of the immune system and an extreme hyperinflammatory response. The etiology is classified in primary HLH (familiar or genetic) and secondary (infectious, oncological, and rheumatological diseases). AIM: To analyze clinical and laboratory characteristics, treatment, and follow-up rates in pediatric patients with HLH. METHODS: A pediatric cohort of patients with HLH diagnosis attending in a tertiary hospital between January 2000 to February 2019 was analysed. RESULTS: 23 hospitalized patients were recruited with a median of 36 months of age. The most frequent clinical and laboratory findings were fever, cytopenias, and hyperferritinemia. The most frequent aetiologies were infectious (Epstein Barr virus and citomegalovirus) and rheumatological diseases. The global mortality was 35%, there was no significant difference between etiologies. DISCUSSION: Considering the high mortality of HLH it is very important to have a high grade of suspicion that allows treating at an early stage. It would be important to determine clinical and laboratory predictors in multicentric studies.
