Rheumatic fever: Down but not gone

Helping babies breathe — Alan H. Jobe, MD, PhD

Growth hormone treatment of skeletal dysplasia — Thomas R. Welch, MD

Rheumatic fever: Down but not gone — Sarah S. Long, MD

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ince the introduction of ventilators designed for infants in the 1970s, there have been multiple innovations to better deliver mechanical breaths. Ventilators that synchronize with spontaneous breathing and deliver operator-selected tidal volumes are now routinely used. Flow sensors are used to synchronize breaths and deliver tidal volumes. A new wrinkle is the recent development of an esophageal catheter that can detect the amount of neural signaling to the diaphragm. This vagal nerve signal can be used to synchronize mechanical breaths with spontaneous breaths of the infant and vary volume/pressure with the size of the neural signal. In this issue of The Journal, Stein et al report that the use of neutrally adjusted ventilatory assistance (NAVA) seems to provide better ventilatory assistance than conventional ventilators for intubated infants with respiratory distress syndrome or bronchopulmonary dysplasia. Many clinicians are now using various devices to provide noninvasive (no endotracheal tube) ventilation via nasal devices. Synchronization of these ventilatory assists using flow sensors is most difficult because of the large and variable leaks. Adaptation of technology such as NAVA may allow for synchronization of noninvasive ventilation. Article page 786<

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t seems that recombinant human growth hormone (rhGH) therapy can lead to increased growth in just about any child with (or without) chronic disease. An exception to this has been one of the more common skeletal dysplasias achondroplasia. Attempts at achieving enhanced growth in children with this disorder have not been successful. Achondroplasia is caused by a mutation in the FDFR3 gene. There is a phenotypically very similar condition, hypochrondroplasia, which is actually associated with a different mutation in this same gene. In this issue of The Journal, Rothenbuhler et al provide an interim report of a trial of rhGH in 6 children with hypochondroplasia. This is an important report for a couple of reasons. First of all, the authors used a dosing regimen for the rhGH that was titrated to achieve preset goal levels of insulin-like growth factor 1 in each patient. Unlike the situation with achondroplasia, all of these children had improved statural growth and improvement in body proportion during this first period of treatment. The second important take-home message from this report is the potential need for mutation analysis in children with suspected achondroplasia. Because the disorder may not be differentiated from hypochondroplasia clinically, and the two disorders appear to differ in their responsiveness to growth hormone, making the correct diagnosis may be extremely important for the child’s ultimate outcome. Article page 849<

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uthors from the well-industrialized area of Abruzzo, in Central Italy document the population-based incidence of acute rheumatic fever and prevalence of rheumatic heart disease in children from 2000 through 2009. The observed rates are lower than those in developing countries but higher than those in most of the United States. Of the 88 cases identified, 75% were 5-14 years of age (with 14% younger than 5 years), 59% had arthritis, and 49% had carditis. The study reminds us that rheumatic fever is down but not gone. The Jones criteria for diagnosis still are relevant, with consideration of stand-alone echocardiographic criteria ongoing. Primary prophylaxis (eg, diagnosis and treatment of streptococcal pharyngitis) and secondary prophylaxis (continuous penicillin to prevent recurrent infection) are still our best weapons against this ongoing threat to children’s lives and well being. Article page 832<

Vol. 160, No. 5