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SIDS?—Think CAH! As the frequency of SIDS resulting from cardiopulmonary events related to sleeping position and inappropriate bedding decrease, more unusual genetic/metabolic causes of SIDS are being diagnosed by targeted autopsy and metabolic evaluations. Gassner et al report 3 causes of near-miss SIDS events in infants who had a late presentation of severe adrenal crisis at 6 to 8 months of age. Two of these infants had complete deficiencies in steroidogenesis resulting from mutations preventing cholesterol transport into mitochondria. The third case remains undiagnosed as to the genetic cause of the global steroid deficiency. These unusual presentations of atypical congenital adrenal hyperplasia emphasize the importance of complete evaluations of SIDS and near-miss SIDS cases. Alan H. Jobe, MD, PhD Page 178 Postconceptional age and IVH in ECMO patients Although extracorporeal membrane oxygenation (ECMO) is an effective treatment for term infants with severe respiratory failure, this therapy is associated with a high incidence of intra- ventricular hemorrhage (IVH) in preterms. As the number of term infants with only respiratory failure requiring ECMO has strikingly decreased, infants and older children with other and more complex problems are receiving ECMO. One group with a high incidence of primarily respiratory factor is the preterm. Preterm infants in general are at highest risk of IVH for several days after birth only, and that risk interval seems to be independent of gestational age. Hardart et al queried the Extracorporeal Life Support Organization Registry to determine which age is associated with IVH in preterms treated with ECMO: gestational age at birth, postnatal age, or post- conceptional age (gestational age + postnatal age). Postnatal age was not a strong predictor of IVH, but postconceptional age was the best predictor. This information is useful for decisions about which preterm infant might benefit from ECMO. The 24-week preterm who develops severe respiratory failure from RSV at 32 weeks’ postconceptional age probably has an unacceptably high risk of IVH if ECMO is used. Alan H. Jobe, MD, PhD Page 184 ‘‘What’s your dehydration score?’’ For decades, clinicians have assessed dehydrated children and assigned some descriptor—‘‘mild,’’ ‘‘moderate,’’ or ‘‘severe’’ would be typical classifications. Although such systems have the patina of objectivity, in reality they are typically based more on gestalt. In the current issue of The Journal, Friedman et al from Toronto report on a study designed to systematize this classi- fication. They studied a prospectively ascertained cohort of dehydrated children, and used the difference between their admission and ‘‘rehydrated’’ weights as an objective measure of the severity of their dehydration. Each child additionally was assessed for 12 carefully defined clinical signs. The signs that could be shown statistically to be most predictive of hydration status were then combined into a rating scale. General appearance, eyes, mucous membranes, and tears were the items ultimately in- corporated into their scale. This scale may have immediate applicability to the practicing pediatrician; in the same way in which we assign a Glasgow coma score or an Apgar score, we now have a validated dehydration score to use with our hospitalized patients. Perhaps more importantly, we now have a score that can become incorporated into clinical research studies. Thomas R. Welch, MD Page 201 What’s coming up with reflux? Transient lower esophageal sphincter relaxation (TLESR) is the predominant mechanism for gastroesophageal reflux (GER) in infants. Multichannel intraluminal impedance (MII), basically a ‘‘flow meter,’’ is a new technique that is used to assess the velocity and direction transit of liquids and gases in the gut. Omari et al used a novel modified MII technique to help understand the factors associated with GER in healthy preterm infants. They noted that in these infants, GER was liquid in nature and right- side positioning paradoxically triggered TLESR, hence, GER. Although the clinical significance remains to be defined, it is clear that the technique of impedance measurement is providing new insight into GER. William F. Balistreri, MD Page 194 2A August 2004 The Journal of Pediatrics

What's coming up with reflux?

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SIDS?—Think CAH!As the frequency of SIDS resulting from cardiopulmonary

events related to sleeping position and inappropriate beddingdecrease, more unusual genetic/metabolic causes of SIDS arebeing diagnosed by targeted autopsy and metabolic evaluations.Gassner et al report 3 causes of near-miss SIDS events in infantswho had a late presentation of severe adrenal crisis at 6 to 8months of age. Two of these infants had complete deficiencies insteroidogenesis resulting from mutations preventing cholesteroltransport into mitochondria. The third case remains undiagnosedas to the genetic cause of the global steroid deficiency. Theseunusual presentations of atypical congenital adrenal hyperplasiaemphasize the importance of complete evaluations of SIDS andnear-miss SIDS cases.

—Alan H. Jobe, MD, PhDPage 178

Postconceptional age and IVH in ECMOpatients

Although extracorporeal membrane oxygenation (ECMO)is an effective treatment for term infants with severe respiratoryfailure, this therapy is associated with a high incidence of intra-ventricular hemorrhage (IVH) in preterms. As the number ofterm infants with only respiratory failure requiring ECMO hasstrikingly decreased, infants and older children with other andmore complex problems are receiving ECMO. One group witha high incidence of primarily respiratory factor is the preterm.Preterm infants in general are at highest risk of IVH for severaldays after birth only, and that risk interval seems to beindependent of gestational age. Hardart et al queried theExtracorporeal Life Support Organization Registry to determinewhich age is associated with IVH in preterms treated withECMO: gestational age at birth, postnatal age, or post-conceptional age (gestational age + postnatal age). Postnatal agewas not a strong predictor of IVH, but postconceptional age wasthe best predictor. This information is useful for decisions aboutwhich preterm infant might benefit from ECMO. The 24-weekpreterm who develops severe respiratory failure from RSV at 32weeks’ postconceptional age probably has an unacceptably highrisk of IVH if ECMO is used.

—Alan H. Jobe, MD, PhDPage 184

‘‘What’s your dehydration score?’’For decades, clinicians have assessed dehydrated children

and assigned some descriptor—‘‘mild,’’ ‘‘moderate,’’ or ‘‘severe’’would be typical classifications. Although such systems have thepatina of objectivity, in reality they are typically based more ongestalt.

In the current issue of The Journal, Friedman et al fromToronto report on a study designed to systematize this classi-fication. They studied a prospectively ascertained cohort ofdehydrated children, and used the difference between theiradmission and ‘‘rehydrated’’ weights as an objective measure of theseverity of their dehydration. Each child additionally was assessedfor 12 carefully defined clinical signs. The signs that could beshown statistically to be most predictive of hydration status werethen combined into a rating scale. General appearance, eyes,mucous membranes, and tears were the items ultimately in-corporated into their scale.

This scale may have immediate applicability to thepracticing pediatrician; in the same way in which we assigna Glasgow coma score or an Apgar score, we now have a validateddehydration score to use with our hospitalized patients. Perhapsmore importantly, we now have a score that can becomeincorporated into clinical research studies.

—Thomas R. Welch, MDPage 201

2A August 2004

What’s coming up with reflux?Transient lower esophageal sphincter relaxation (TLESR)

is the predominant mechanism for gastroesophageal reflux (GER)in infants. Multichannel intraluminal impedance (MII), basicallya ‘‘flow meter,’’ is a new technique that is used to assess the velocityand direction transit of liquids and gases in the gut. Omari et alused a novel modified MII technique to help understand thefactors associated with GER in healthy preterm infants. Theynoted that in these infants, GER was liquid in nature and right-side positioning paradoxically triggered TLESR, hence, GER.Although the clinical significance remains to be defined, it is clearthat the technique of impedance measurement is providing newinsight into GER.

—William F. Balistreri, MDPage 194

The Journal of Pediatrics