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OBJECTIVES: The objective of this article is to review current information about fever without source in children less than 36 months of age and to discuss the practical aspects of diagnosis and management.METHODS: Articles from the specific area were reviewed and presented in a practical form with recommendations from the authors.RESULTS: Febrile children comprise a substantial proportion of ambulatory pediatric visits. The majority of children are less than 3 years old. At first examination, there may be considerable overlap in the clinical appearance of children with fever due to viral illness, occult bacteremia and serious bacterial infection. The authors present the recommendations and protocols cited in the literature to assist physicians in managing infants and children with fever without source.CONCLUSION: Although many approaches to the evaluation and management of the febrile infant exist, no diagnostic protocol or therapeutic scheme is optimal for all patients. Pediatricians may individualize their management of these patients, depending on their experience, or interpretation of the recommendations presented in the literature.
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We report here the treatment and poor outcome of a case of Maple Syrup Urine Disease with late diagnosis and retrieval (2 and 5 months, respectively). As the proband had quite high levels of plasmatic leucine (1956 micromol/L for a normal upper limit of 77), we started immediately with a gluco-insulin therapy to produce anabolism in the infant. When leucine has fallen to 275.3 micromol/L, we instituted feeding with branched chain amino acid-free protein and high energy from carbohydrates. After reviewing briefly the clinical, biochemical and therapeutic aspects of this disorder, we comment on the great difficulties of making early diagnosis and of obtaining the specific dietetic formulas to Maple Syrup Urine Disease, in Brazil.