Jornal de pediatria
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Jornal de pediatria · May 2012
Case ReportsAnti-N-methyl D-aspartate receptor encephalitis in childhood.
To discuss the differential diagnosis of encephalitis beyond that of infectious etiology and to inform pediatricians about the possibility of anti-N-methyl-D-aspartate receptor (NMDAr) encephalitis in children by highlighting its most important clinical features. ⋯ Clinical signs of anti-NMDAr encephalitis in children are similar to those previously described in adults. Tumors are not usually detected by this age. The diagnosis of anti-NMDAr encephalitis must be addressed only after the exclusion of infectious and other recognizable causes of encephalitis. Pediatricians should be aware of this treatable autoimmune condition.
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Jornal de pediatria · Jan 2012
Case ReportsModerately progressive Ullrich congenital muscular dystrophy.
To describe genetic and clinical features of Ullrich congenital muscular dystrophy (UCMD), and to report the case of a patient diagnosed with UCMD after an exhaustive investigation, which included collagen VI immunohistochemical and genomic analyses. ⋯ A better knowledge of congenital muscular dystrophies will improve the number of correct diagnoses and open new horizons for the treatment of such diseases. Genetic evaluation of UCMD patients has relevant implications for prognosis and genetic counseling of the family. The dissemination of this disorder in the pediatric community is advisable, because of the early onset of clinical manifestations and the fact that it is frequently misdiagnosed or not diagnosed at all.
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Jornal de pediatria · Jan 2012
Reliability of the Pediatric Quality of Life Inventory - Healthcare Satisfaction Generic Module 3.0 version for the assessment of the quality of care of children with chronic diseases.
To translate to Brazilian Portuguese and to evaluate the reliability of the Healthcare Satisfaction (HS) Generic Module version 3.0 of the PedsQL inventory for the Brazilian culture. ⋯ The Brazilian version of the HS-PedsQL is a valid and reliable tool to measure the overall satisfaction of parents and caregivers with the treatment of their children.
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Jornal de pediatria · Nov 2011
Staff workload and adverse events during mechanical ventilation in neonatal intensive care units.
To investigate a possible association between the intensity of staff workload and intermediate adverse events, such as accidental extubation, obstruction of the endotracheal tube, and accidental disconnection of the ventilator circuit, during neonatal mechanical ventilation in high-risk neonatal units. ⋯ The workload of NICU professionals seems to interfere with the intermediate results of neonatal care and thus should be taken into consideration when evaluating NICU outcomes.