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Mortality in Pediatric Palliative Care in a Tertiary Center

Introduction: Complex chronic diseases have been responsible for about a third of all pediatric deaths in Portugal in recent years. Early referral and implantation of an advance care plan are strategies that result in greater preparation on the part of families for dealing with the course of illness of these children. This study aimed to characterize the mortality data of patients followed by the pediatric pall...

PEDOPSIQUIATRIA EM CUIDADOS PALIATIVOS PEDIÁTRICOS

Segundo a definição da Organização Mundial de Saúde, os Cuidados Paliativos Pediátricos (CPP) destinam-se a crianças com doenças ameaçadoras de vida e abrangem elementos físicos, emocionais, sociais e espirituais. As crianças e adolescentes a quem é diagnosticada uma doença potencialmente ameaçadora de vida, apresentam um risco acrescido de desenvolver uma perturbação mental ou de um agravamento da psicopatolog...

Coagulation Reference Values and Indications for the Use of Plasma Derivatives ...

Normal levels of coagulation factors in neonates are generally lower, which lead to prolonged clotting times, namely prothrombin time and activated partial thromboplastin time. These tests are frequently requested in neonatal intensive care units, and altered values are commonly found. However, these coagulation factor deficiencies are physiologic. Developmental hemostasis is a process that leads to the progres...

Fanconi Anemia: How to Recognize It Before Bone Marrow Failure?

Fanconi anemia is the most common of the rare inherited bone marrow failure syndromes. It is caused by genetic mutations that lead to genomic instability, the hallmark of the disorder. Therefore, patients are extremely vulnerable to bone marrow failure, leukemia and neoplasms. Several physical anomalies have been associated, affecting multiple organ systems. We present three cases in whom the diagnosis was made...

Operationalizing dignity therapy for adolescents

Diamond-Blackfan Anemia: Case Series in a Portuguese Tertiary Level Hospital

Introduction: Diamond-Blackfan anemia (DBA) is a rare congenital erythroid aplasia associated with congenital abnormalities and evolving to bone marrow failure. It should be considered when the evolution differs from transient erythroblastopenia of childhood. Methods: Retrospective and descriptive study including the DBA cases followed in a Unit of Pediatric Hematology in a Portuguese tertiary level hospital, o...

An Atypical Presentation of Rhabdomyosarcoma

Thromboembolism is infrequent in children, nonetheless, whenever malignancy is present, there is an increased risk, around 7% to 25%. Rhabdomyosarcomas are typically asymptomatic masses, but patients can become symptomatic due to mass overgrowth and compression of surrounding structures, therefore presentation might be as a thromboembolic event. Sarcomas have recognized risk factors including large tumour masse...

Operationalizing dignity therapy for adolescents

Dignity Therapy (DT) is a brief, individualized intervention, which provides terminally ill patients with an opportunity to convey memories and essential disclosures that culminate in a legacy document. DT often broaches psychosocial and existential issues, hence bolstering a sense of meaning and purpose (Chochinov et al., 2005). During DT, trained therapists guide a psychotherapeutic session based on a framewo...

Trombose Venosa Profunda em Adolescente - Caso Clínico

A trombose venosa profunda (TVP) é uma entidade rara na idade pediátrica mas quando presente é causa de morbilidade e mortalidade importantes. Em 95% dos casos, a TVP tem uma causa subjacente, hereditária ou adquirida que deve ser investigada. Os autores apresentam o caso clínico de uma adolescente com TVP extensa do membro inferior em que a investigação etiológica revelou a existência de vários factores protro...

Trombose Venosa Profunda em Pediatria

A trombose venosa profunda em pediatria é uma entidade rara, habitualmente de etiologia secundária e pode ser causa de morbilidade e mortalidade importantes. Neste grupo etário, a prevenção e o tratamento do tromboembolismo diferem pela fisiopatologia e resposta aos antitrombóticos. Os autores revêem a epidemiologia, diagnóstico e conceitos terapêuticos novos desta entidade.