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22. Corr PB, Creer MH, Yamada KA, Saffitz JE, Sobel BE. Prophylaxis ofearly ventricular fibrillation by inhibition of acylcarnitine accumulation. J ClinInvest 1989;83:927–36.23. Bergmann SR, Herrero P, Sciacca R, Hartman JJ, Rubin PJ, Hickey KT,et al. Characterization of altered myocardial fatty acid metabolism in patientswith inherited cardiomyopathy. J Inherit Metab Disord 2001;24:657–74.
24. Tyni T, Palotie A, Viinikka L, Valanne L, Salo MK, von Dobeln U, et al.Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency with theG1528C mutation: clinical presentation of thirteen patients. J Pediatr1997;130:67–76.25. Shortland GJ, Schmidt M, Losty H, Leonard JV. LCHAD deficiencytreated with creatine. J Inherit Metab Disord 2001;24:71,139.
50 Years Ago in The Journal of PediatricsTHE USE OF BANTHINE BROMIDE IN INFANTILE PYLOROSPASM. REPORT OF 9 CASES WITHX-RAY STUDY
Levy H, Zweifler B. J Pediatr 1953;42:673-9
The authors studied the effect of banthine bromide, an anticholinergic medication, on vomiting in young infants. Nine ba-bies, 3 weeks to 4 months old, underwent upper gastrointestinal contrast radiography with barium, which demonstrated de-layed passage of contrast from the stomach. In the absence of obstruction, the delayed emptying was attributed to pylorospasm.Films were obtained every half hour for two hours to reveal the amount of residual barium in the stomach semiquantitatively.Improvement was demonstrated in four of the infants whose parents consented to repeat contrast studies after treatment withthe banthine bromide. Clinically, vomiting resolved in all the infants.
The use of an anticholinergic to improve gastric emptying remains counterintuitive. Inducing gastric hypomotility mightwell limit vomiting by interfering with gastric contractions, but the impact on the pylorus should mimic the effect of vagotomy(ie, failed relaxation and gastric outlet obstruction). As such, one might predict that, under the influence of anticholinergicdrugs, gastric emptying would be prolonged further if cholinergic neurons were primarily responsible for all phases of gastricmotor function. We now recognize that other neurohormonal effects, such as those due to motilin, play a substantial role ingastric emptying.
Gastric emptying of inert substances such as barium fails to provide a full picture of gastric emptying under more physiolog-ic circumstances. Many variables affect gastric emptying, including the caloric density of the meal and emotion, such as anxi-ety. In addition, the rates of solid and liquid emptying are different. As such, crude measures of gastric emptying such as thatseen with a barium meal fails to account for the details of what is otherwise a tightly controlled physiologic event.
The authors commented that pylorospasm accounted for most nonobstructive vomiting in infancy. The contrast studies didshow slow emptying of the barium, but the contribution of the pylorus itself to that phenomenon is only assumed. We nowrecognize that vomiting in infancy has a number of causes ranging from simple gastroesophageal reflux to formula protein in-tolerance to obstruction that can be diagnosed specifically and treated directly. Establishing the absence of obstruction due topyloric stenosis, antral web, duodenal web, or malrotation by upper GI series remains an important first step in the evaluationof the vomiting young infant. In the absence of obstruction, however, the role of the upper GI in establishing an etiology forthe symptom remains limited.
Philip E. Putnam, MDDivision of Pediatric Gastroenterology, Hepatology, and Nutrition
Cincinnati Children’s Hospital Medical CenterCincinnati OH, 45229
YMPD25710.1067/mpd.2003.257
Mitochondrial Trifunctional Protein Deficiency: A Severe Fatty AcidOxidation Disorder With Cardiac and Neurologic Involvement 689