Helping babies
breathe
— Alan H. Jobe, MD, PhD
S
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 tech-
nology such as NAVA may allow for synchronization of noninvasive ventilation.
Article page 786<
Growth hormone
treatment of
skeletal dysplasia
— Thomas R. Welch, MD
I
t seems that recombinant human growth hormone (rhGH) therapy can lead to in-
creased growth in just about any child with (or without) chronic disease. An excep-
tion 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 phenotypi-
cally 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 hypochondropla-
sia. 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 propor-
tion 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 hypochondro-
plasia 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<
Rheumatic fever:
Down but not gone
— Sarah S. Long, MD
A
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<
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