Epidermolysis bullosa and gastrointestinal anomalies

Epidermolysis bullosa and gastrointestinal anomalies

EDITORIAL C O R R E S P O N D E N C E Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics o ...

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EDITORIAL C O R R E S P O N D E N C E

Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics o f current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.

Epidermolysis bullosa and gastrointestinal anomalies To the Editor: We have read with great interest the recent reports in THE JOURNAL~-3 and elsewherC on the association of epidermolysis bull0sa and pyloric atresia. We have recently evaluated and treated a 5-year-old white girl with a dominantly inherited, nonscarring form of epidermolysis buUosa. She presented with episodes of abdominal pain that sometimes were associated with intractable vomiting. Investigation had not revealed a cause, suggesting a psychosomatic etiology. An electroencePhalogram was abnormal, suggesting that she might have psychomotor epilepsy. However, after several episodes of intractable vomiting necessitated treatment with fluids intravenously, an upper gastrointestinal series revealed a dilated stomach and duodenum. There was considerable narrowing at the juncture of the duodenum and jejunum, with partial malrotation of the jejunum. Some contrast medium did pass, giving the Classical corkscrew appearance. At operation, she was found to have classical malrotation with nonattachment of the small bowel mesentery. Ladd bands stretched across the cecum to the posterior parietal peritoneum at the level of the duodenum. There was a volvulus with 280 degree clockwise rotation at the level of the third portion of the duodenum. The appendix and cecum were lying in the area Of the midtransverse colon. The volvulus was derotated, Ladd bands were lysed, the duodenum and jejunum were allowed to lie in a straight fine on the right pericolic gutter, and the large intestine was allowed to lie freely on the left side. She has had rio further difficulty with vomiting. Six cases o f clinically diagnosed, dystrophic epidermolysis bullosa have been reported in association with pyloric atresi~t. These patients plus the association of malrotation o f the midgut and epidermolysis bnllosa in our patient may indicate that epidermolysis buliosa is associated with various types of congenital gut abnormalities. Bernard N. Robinowitz, M.D. James G. Coldwell, M.D. Subramania Jegathesan, M.D. Department of Pediatrics Children's Medical Center 5300 E. Skelly Dr. Tulsa, O K 74135 488

TheJournalofPEDIATRICS

REFERENCES

1. Korber JS. and Glasson M J: Pyloric atresia associated with epidermolysis bullosa, J PEDIATR 90:600. 1977. 2. Pedersen PV. and Hertel J: Pyloric atresia and epidermolysis bullosa. J PEDIATR 91:852. 1977, 3. DeGroot WG. Postuma R, and Hunter AGW: Familial pyloric atresia associated with epidermolysis bullosa. J PEDIATR 92:429. 1978. 4. Arrabal TC et al: Atresia de piloro eu un recien nacido con epidermolisis ampollosa, Boletin de la Catedra de Pediatria-Madrid, 16:47. 1972.

More on Karo syrup in infant formulas To the Editor: Karo syrup has been used effectively as an integral part of the evaporated milk infant feeding formula for over 50 years. Many pediatricians also prescribe up to 3 teaspoons of Karo syrup daily to relieve occasional constipation in infants receiving commercial formulas. Two recent case reports (J PEDIATR93:883. 1978 and J PEDIArR 94:1012, 1979) describe edema in 1- and 2-month-old premature infants whose mothers misunderstood the pediatrician's directions and apparently gave-these infants approximately 15 teaspoons (5 tablespoons) of Karo corn syrup daily rather than the prescribed 3 teaspoons: As is stated on the label of the bottle, one tablespoon of Karo dark corn syrup provides 60 kcal and Contains 30 mg sodium, determined by routine analyses. Thus. these infants received each day, unintentionally, about 240 kcal and 120 mg (approximately 5 mEq) sodium in addition to that in the prescribed commercial formula and the 3 teaspoons of Karo syrup. The cause(s) for the observed edema are not clear. Urinary sodium excretion and seium electrolyte levels were within normal limits for infants of this age. It may be significant that the two infants were premature. Both extra calorie and sodium intake may have been involved. The edema disappeared after the infants' feedings were restricted to thecommercial formula. To our knowledge, these are the first reports of infant edema linked to an overdose of Karo syrup. It seems essential that pediatricians and dietitians make sure that mothers understand instructions for the addition of Karo syrup to commercial