Secondary Carnitine Deficiency in Neonates and Infants Requiring Surgery for Intestinal Obstructions-An Underestimated and Undermanaged Problem.

ABSTRACT

This study aims to elucidate the relationship between intestinal obstruction and carnitine deficiency in neonates and infants. We retrospectively reviewed medical records of 330 neonates and infants, younger than six months, who underwent surgery for intestinal obstruction at our institute from January 2009 to April 2022. The analysis focused on clinical symptoms, related signs, complications, and etiology of the intestinal obstruction. Tandem mass spectrometry (MS/MS) or urine organic acid analysis was conducted for 47 patients, revealing carnitine deficiency in 16 patients. The incidence of carnitine deficiency was 34.0% in the suspicious group and 4.8% overall, significantly higher than in the general population in Taiwan. Notably, patients with carnitine deficiency experienced prolonged ileus, with a mean fasting duration of 41.7 days (range 7.8-65.5 days), compared to 10.8 days (range 8.2-13.4 days) in patients without carnitine deficiency. Carnitine replacement therapy was administered to twelve patients at dosages ranging from 32 to 90 mg/kg/day. One patient exhibited a drug allergy with skin rashes. Our findings suggest that carnitine deficiency should be considered in cases of neonatal and infant intestinal obstruction. Replacement therapy is straightforward and can be prognostically beneficial for some patients. Therefore, we recommend generalizing MS/MS and urine organic acid analysis, particularly for patients with prolonged ileus.