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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Practice Guideline
Clinical report—Guidelines for the determination of brain death in infants and children: an update of the 1987 task force recommendations.
To review and revise the 1987 pediatric brain death guidelines. ⋯ (1) Determination of brain death in term newborns, infants and children is a clinical diagnosis based on the absence of neurologic function with a known irreversible cause of coma. Because of insufficient data in the literature, recommendations for preterm infants less than 37 weeks gestational age are not included in this guideline. (2) Hypotension, hypothermia, and metabolic disturbances should be treated and corrected and medications that can interfere with the neurologic examination and apnea testing should be discontinued allowing for adequate clearance before proceeding with these evaluations. (3) Two examinations including apnea testing with each examination separated by an observation period are required. Examinations should be performed by different attending physicians. Apnea testing may be performed by the same physician. An observation period of 24 hours for term newborns (37 weeks gestational age) to 30 days of age, and 12 hours for infants and chi (> 30 days to 18 years) is recommended. The first examination determines the child has met the accepted neurologic examination criteria for brain death. The second examination confirms brain death based on an unchanged and irreversible condition. Assessment of neurologic function following cardiopulmonary resuscitation or other severe acute brain injuries should be deferred for 24 hours or longer if there are concerns or inconsistencies in the examination. (4) Apnea testing to support the diagnosis of brain death must be performed safely and requires documentation of an arterial Paco(2) 20 mm Hg above the baseline and ≥ 60 mm Hg with no respiratory effort during the testing period. If the apnea test cannot be safely completed, an ancillary study should be performed. (5) Ancillary studies (electroencephalogram and radionuclide cerebral blood flow) are not required to establish brain death and are not a substitute for the neurologic examination. Ancillary studies may be us d to assist the clinician in making the diagnosis of brain death (i) when components of the examination or apnea testing cannot be completed safely due to the underlying medical condition of the patient; (ii) if there is uncertainty about the results of the neurologic examination; (iii) if a medication effect may be present; or (iv) to reduce the inter-examination observation period. When ancillary studies are used, a second clinical examination and apnea test should be performed and components that can be completed must remain consistent with brain death. In this instance the observation interval may be shortened and the second neurologic examination and apnea test (or all components that are able to be completed safely) can be performed at any time thereafter. (6) Death is declared when the above criteria are fulfilled.
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The effects of in utero tobacco smoke exposure on childhood respiratory health have been investigated, and outcomes have been inconsistent. ⋯ Exposure to tobacco smoke in utero was significantly associated with persistent asthma among Mexican, Puerto Rican, and black children compared with those with intermittent asthma. These results suggest that smoking cessation during pregnancy may lead to a decrease in the incidence of persistent asthma in these populations.
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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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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.