Pediatrics
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Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) falls within a group of pediatric disorders with both respiratory control and autonomic nervous system dysregulation. Children with ROHHAD typically present after 1.5 years of age with rapid weight gain as the initial sign. Subsequently, they develop alveolar hypoventilation, autonomic nervous system dysregulation, and, if untreated, cardiorespiratory arrest. ⋯ The unaffected twin demonstrated rapid weight gain later in age but not development of signs/symptoms consistent with ROHHAD. This discordant twin pair demonstrates key features of ROHHAD including the importance of early recognition (especially hypoventilation), complexity of signs/symptoms and clinical course, and importance of initiating comprehensive, multispecialty care. These cases confound the hypothesis of a monogenic etiology for ROHHAD and indicate alternative etiologies including autoimmune or epigenetic phenomenon or a combination of genetic predisposition and acquired precipitant.
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To develop a set of quality-of-care indicators for the management of children with sickle cell disease (SCD) who are cared for in a variety of settings by addressing the broad spectrum of complications relevant to their illness. ⋯ Children with SCD are at risk for serious morbidities and early mortality, yet efforts to assess and improve the quality of their care have been limited compared with other chronic childhood conditions. This set of 41 indicators can be used to assess quality of care and provide a starting point for quality-improvement efforts.
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Two varicella-containing vaccines are licensed for use in the United States: monovalent varicella vaccine (Varivax [Merck & Co, Inc, West Point, PA]) and quadrivalent measles-mumps-rubella-varicella vaccine (MMRV) (ProQuad [Merck & Co, Inc]). It is estimated from postlicensure data that after vaccination at 12 through 23 months of age, 7 to 9 febrile seizures occur per 10,000 children who receive the MMRV, and 3 to 4 febrile seizures occur per 10,000 children who receive the measles-mumps-rubella (MMR) and varicella vaccines administered concurrently but at separate sites. Thus, 1 additional febrile seizure is expected to occur per approximately 2300 to 2600 children 12 to 23 months old vaccinated with the MMRV, when compared with separate MMR and varicella vaccine administration. ⋯ Febrile seizures do not predispose to epilepsy or neurodevelopmental delays later in life and are not associated with long-term health impairment. The American Academy of Pediatrics recommends that either MMR and varicella vaccines separately or the MMRV be used for the first dose of measles, mumps, rubella, and varicella vaccines administered at 12 through 47 months of age. For the first dose of measles, mumps, rubella, and varicella vaccines administered at ages 48 months and older, and for dose 2 at any age (15 months to 12 years), use of MMRV generally is preferred over separate injections of MMR and varicella vaccines.
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Acellular pertussis vaccine is safe and effective in adults. An explicit recommendation for pertussis booster vaccination in pediatric health care workers is based on the importance of health care workers as a potential source of infection for patients. However, limited information is available on the economic attractiveness of this intervention. We sought to evaluate the health-economic attractiveness of a diphtheria-tetanus-acellular pertussis booster vaccination program for health care workers in a pediatric intensive care setting. ⋯ The implementation of a hospital-based and funded diphtheria-tetanus-acellular pertussis vaccine program administered through an occupational health program is cost-effective or cost-saving in the context of pediatric health care facilities in which many of the patients are at risk of serious morbidity and mortality should they acquire pertussis while hospitalized.