[13C]Methionine breath test to assess intestinal failure-associated liver disease.
Oxidation of L[1-C]methionine ([C]-Met) in liver mitochondria can be quantified by measuring exhaled CO2. We hypothesized that CO2 recovery after i.v. administered [C]-Met would provide a noninvasive measure of liver function in pediatric intestinal failure-associated liver disease (IFALD). After Institutional Review Board (IRB) approval, 27 patients underwent L[1-C]-Met breath tests ([C]-MBTs), five of whom underwent repeat testing after clinical changes in liver function. Sterile, pyrogen-free [C]-Met was given i.v. Six breath samples collected during 120 min were analyzed for CO2 enrichment using isotope ratio mass spectrometry. Pediatric end-stage liver disease (PELD) scores were recorded, and total carbon dioxide (CO2) production was measured by indirect calorimetry. Twenty-seven patients (median age = 5.3 mo) underwent a total of 34 [C]-MBTs without adverse events. Fourteen patients had documented liver biopsies (five with cirrhosis and nine with cholestasis or fibrosis). The [C]-MBT differentiated patients with and without cirrhosis (medians 210 and 350, respectively, p = 0.04). Serial [C]-MBTs in five patients reflected changing PELD scores. i.v. administering the stable isotope [C]-Met with serial breath sampling provides a useful, safe, and potentially clinically relevant evaluation of hepatic function in pediatric IFALD. The [C]-MBT may also help quantify progression or improvement of IFALD.
Duro
Debora
D
Division of Gastroenterology and Nutrition, Children Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Fitzgibbons
Shimae
S
Valim
Clarissa
C
Yang
Chi-Fu Jeffrey
CF
Zurakowski
David
D
Dolan
Melanie
M
Bechard
Lori
L
Yu
Yong Ming
YM
Duggan
Christopher
C
Jaksic
Tom
T
D001706:Biopsy; D001900:Boston; D001944:Breath Tests; D002153:Calorimetry, Indirect; D002245:Carbon Dioxide / Q000378:metabolism*; D002247:Carbon Isotopes / Q000008:administration & dosage; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007223:Infant; D007275:Injections, Intravenous; D007410:Intestinal Diseases / Q000150:complications*; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D008111:Liver Function Tests; D008297:Male; D013058:Mass Spectrometry; D008715:Methionine / Q000008:administration & dosage; D008930:Mitochondria, Liver / Q000378:metabolism; D011237:Predictive Value of Tests; D013997:Time Factors
D002247:Carbon Isotopes; D002245:Carbon Dioxide; D008715:Methionine