Bmc Pediatr
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Newborns with hypoxic ischemic encephalopathy (HIE) are at risk for coagulopathy due to systemic oxygen deprivation. Additionally, therapeutic hypothermia (TH) slows enzymatic activity of the coagulation cascade, leading to constitutive prolongation of routinely assessed coagulation studies. The level of laboratory abnormality that predicts bleeding is unclear, leading to varying transfusion therapy practices. ⋯ Laboratory evidence of coagulopathy is universal in HIE babies undergoing TH. Transfusion strategies to maintain PLT counts >130 × 109/L, fib level >1.5 g/L, and INR <2 may prevent clinical bleeding in this high risk population.
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Case Reports
Spontaneous pneumothorax as indicator for Birt-Hogg-Dubé syndrome in paediatric patients.
Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited disorder caused by germline mutations in the folliculin (FLCN) gene. Clinical manifestations of BHD include skin fibrofolliculomas, renal cell cancer, lung cysts and (recurrent) spontaneous pneumothorax (SP). All clinical manifestations usually present in adults > 20 years of age. ⋯ Although childhood occurrence of SP in BHD is rare, these two cases illustrate that BHD should be considered as cause of SP in children.
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Consideration of the quality of life in relation to individual health status is crucial for planning and maintaining a system of patient-centered care. Until recently, there have been no suitable instruments to assess health-related quality of life (HRQoL,) of children and adolescents with severe, non-ambulant cerebral palsy (GMFCS functional levels IV and V). The "Caregiver Priorities and Child Health Index of Life with Disabilities Questionnaire" (CPCHILD) was developed in English specifically for this population, and has been validated in Canada. The aim of this study was to translate and adapt the CPCHILD Questionnaire into Brazilian Portuguese, thus permitting researchers in Brazil to access this important tool for measuring HRQoL in this population, as well as the possibility of making comparisons with other studies that use the same questionnaire in other languages. ⋯ The translation and cross-cultural adaptation of the CPCHILD questionnaire provides a Brazilian Portuguese equivalent to measure the HRQoL of children with severe developmental disabilities, with the potential to measure the benefits of various procedures that are indicated for these patients. This adaptation exhibited a satisfactory level of semantic equivalence between the Portuguese target and the original English source versions. The validity of the Brazilian version of the instrument must be established in the future by assessing its psychometric properties on Brazilian epidemiological samples.
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Children with cancer, parents, and clinicians, face difficult decisions when cure is no longer possible. Little is known about decision-making processes, how agreement is reached, or perspectives of different actors. Professionals voice concerns about managing parental expectations and beliefs, which can be contrary to their own and may change over time. We conducted the first systematic review to determine what constitutes best medico-legal practice for children under 19 years as context to exploring the perspectives of actors who make judgements and decisions when cancer treatment is no longer curative. ⋯ There needs to be fresh impetus to more effectively and universally implement the ethics of professionalism into daily clinical practice in order to reinforce humanitarian attitudes. Ethical guidelines and regulations attempt to bring professionals together by articulating shared values. While important, ethics training must be supported by institutions/organizations to assist doctors to maintain good professional standards. Findings will hopefully stimulate further normative and descriptive lines of research in this complex under-researched field. Future research needs to be undertaken through a more deliberative cultural lens that includes children's and multi-disciplinary team members' perspectives to more fully characterize and understand the dynamics of the decision-making process in this specific end-of life context.
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Despite huge public campaigns, there is still overconsumption of antibiotics in children with self-limiting diseases. Possible explanations may be the physicians' and parents' uncertainty about the gravity of the disease and inadequate communication between physicians and parents leading to lack of reassurance for the parents. In this paper we describe the design and methods of a trial aiming to rationalize antibiotic prescribing by decreasing this uncertainty and parental anxiety. ⋯ This is a unique multifaceted intervention, in that it targets both physicians and parents by aiming specifically at their uncertainty and concerns during the consultation. Both interventions are easy to implement without special training. When proven effective, they could offer a feasible way to decrease inappropriate antibiotic prescribing for children in family practice and thus avoid emergence of bacterial resistance, side effects and unnecessary healthcare costs. Moreover, the observational part of the study will increase our insight in the course, management and parent's concern of acute illness in children.