Research Articles

Anez-Bustillos L, Dao DT, Baker MA, Fell GL, Puder M, Gura KM. Intravenous Fat Emulsion Formulations for the Adult and Pediatric Patient: Understanding the Differences. Nutr Clin Pract. 2016 Oct;31(5):596-609. doi: 10.1177/0884533616662996. Epub 2016 Aug 16. Review. PubMed PMID: 27533942; PubMed Central PMCID: PMC5438313.
 
Chang MI, Carlson SJ, Nandivada P, O'Loughlin AA, Potemkin AK, Cowan E, Mitchell PD, Gura KM, Puder M. Challenging the 48-Hour Rule-Out for Central Line-Associated Bloodstream Infections in the Pediatric Intestinal Failure Population: A Retrospective Pilot Study. JPEN J Parenter Enteral Nutr. 2016 May; 40(4):567-73. doi: 10.1177/0148607114567897. Epub 2015 Jan 7. PubMed PMID: 25567785.
 
Chou J, Massaad MJ, Cangemi B, Bainter W, Platt C, Badran YR, Raphael BP, Kamin DS, Goldsmith JD, Pai SY, Al-Herz W, Geha RS. A novel mutation in ICOS presenting as hypogammaglobulinemia with susceptibility to opportunistic pathogens. J Allergy Clin Immunol. 2015 Sep;136(3):794-797.e1. doi:
10.1016/j.jaci.2014.12.1940. Epub 2015 Feb 10. PubMed PMID: 25678089.
 
Coffin SE, Klieger SB, Duggan C, Huskins WC, Milstone AM, Potter-Bynoe G, Raphael B, Sandora TJ, Song X, Zerr DM, Lee GM; Pediatric Prevention EpiCenter Consortium. Central line-associated bloodstream infections in neonates with gastrointestinal conditions: developing a candidate definition for mucosal barrier injury bloodstream infections. Infect Control Hosp Epidemiol. 2014 Nov;35(11):1391-9. doi: 10.1086/678410. Epub 2014 Oct 2. PubMed PMID: 25333434; PubMed Central PMCID: PMC4551075.
 
de Meijer VE, Gura KM, Meisel JA, Le HD, Puder M. Parenteral fish oil monotherapy in the management of patients with parenteral nutrition-associated liver disease. Arch Surg. 2010 Jun;145(6):547-51. doi: 10.1001/archsurg.2010.80.  Review. PubMed PMID: 20566974.
 
de Meijer VE, Le HD, Meisel JA, Gura KM, Puder M. Parenteral fish oil as monotherapy prevents essential fatty acid deficiency in parenteral nutrition-dependent patients. J Pediatr Gastroenterol Nutr. 2010 Feb;50(2):212-8. doi: 10.1097/MPG.0b013e3181bbf51e. PubMed PMID: 20038849; PubMed Central PMCID: PMC3365554.
 
Díaz JJ, Gura KM, Roda J, Perez-Atayde AR, Duggan C, Jaksic T, Lo CW. Aspartate aminotransferase to platelet ratio index correlates with hepatic cirrhosis but not with fibrosis in pediatric patients with intestinal failure. J  Pediatr Gastroenterol Nutr. 2013 Sep;57(3):367-71. doi: 10.1097/MPG.0b013e318299fdbd. PubMed PMID: 23666459; PubMed Central PMCID: PMC3758378.
 
Forchielli ML, Lo CW, Richardson D, Gura K, Walker WA, Tonelli E. Central venous line related bacteremia during total parenteral nutrition and/or chemotherapy infusions in children. Ann Ig. 1997 Jan-Feb;9(1):35-40. PubMed PMID: 9284587.
 
Forchielli ML, Paolucci G, Lo CW. Total parenteral nutrition and home parenteral nutrition: an effective combination to sustain malnourished children with cancer. Nutr Rev. 1999 Jan;57(1):15-20. PubMed PMID: 10047701.
 
 
Fuchs J, Fallon EM, Gura KM, Puder M. Use of an omega-3 fatty acid-based emulsion in the treatment of parenteral nutrition-induced cholestasis in patients with microvillous inclusion disease. J Pediatr Surg. 2011 Dec;46(12):2376-82. doi: 10.1016/j.jpedsurg.2011.09.061. Review. PubMed PMID: 22152886.
 
Gluchowski NL, Chitraju C, Picoraro JA, Mejhert N, Pinto S, Xin W, Kamin DS, Winter HS, Chung WK, Walther TC, Farese RV Jr. Identification and characterization of a novel DGAT1 missense mutation associated with congenital diarrhea. J Lipid Res. 2017 Jun;58(6):1230-1237. doi: 10.1194/jlr.P075119. Epub 2017 Apr 3. PubMed PMID: 28373485; PubMed Central PMCID: PMC5454518.
 
Gura KM. Aluminum contamination in products used in parenteral nutrition: has anything changed? Nutrition. 2010 Jun;26(6):585-94. doi: 10.1016/j.nut.2009.10.015. Epub 2010 Apr 3. Review. PubMed PMID: 20363591.
 
Gura KM, Lee S, Valim C, Zhou J, Kim S, Modi BP, Arsenault DA, Strijbosch RA, Lopes S, Duggan C, Puder M. Safety and efficacy of a fish-oil-based fat emulsion  in the treatment of parenteral nutrition-associated liver disease. Pediatrics. 2008 Mar;121(3):e678-86. doi: 10.1542/peds.2007-2248. PubMed PMID: 18310188.
 
Gura KM, Mulberg AE, Mitchell PD, Yap J, Kim CY, Chen M, Potemkin A, Puder M.  Pediatric Intestinal Failure-Associated Liver Disease: Challenges in Identifying  Clinically Relevant Biomarkers. JPEN J Parenter Enteral Nutr. 2016 Sep 28. pii: 0148607116671781. [Epub ahead of print] PubMed PMID: 27683392.
 
Gura KM, Puder M. Rapid infusion of fish oil-based emulsion in infants does not appear to be associated with fat overload syndrome. Nutr Clin Pract. 2010 Aug;25(4):399-402. doi: 10.1177/0884533610373770. PubMed PMID: 20702846.
 
Hudgins JD, Goldberg V, Fell GL, Puder M, Eisenberg MA. Reducing Time to Antibiotics in Children With Intestinal Failure, Central Venous Line, and Fever.  Pediatrics. 2017 Nov;140(5). pii: e20171201. doi: 10.1542/peds.2017-1201. PubMed  PMID: 29066581.
 
Jones BA, Hull MA, Richardson DS, Zurakowski D, Gura K, Fitzgibbons SC, Duro  D, Lo CW, Duggan C, Jaksic T. Efficacy of ethanol locks in reducing central venous catheter infections in pediatric patients with intestinal failure. J Pediatr Surg. 2010 Jun;45(6):1287-93. doi: 10.1016/j.jpedsurg.2010.02.099. PubMed
PMID: 20620333; PubMed Central PMCID: PMC4547776.
 
Khan FA, Squires RH, Litman HJ, Balint J, Carter BA, Fisher JG, Horslen SP, Jaksic T, Kocoshis S, Martinez JA, Mercer D, Rhee S, Rudolph JA, Soden J, Sudan D, Superina RA, Teitelbaum DH, Venick R, Wales PW, Duggan C; Pediatric Intestinal Failure Consortium. Predictors of Enteral Autonomy in Children with Intestinal Failure: A Multicenter Cohort Study. J Pediatr. 2015 Jul;167(1):29-34.e1. doi:
10.1016/j.jpeds.2015.03.040. Epub 2015 Apr 25. PubMed PMID: 25917765; PubMed
Central PMCID: PMC4485931.
 
Le HD, de Meijer VE, Robinson EM, Zurakowski D, Potemkin AK, Arsenault DA, Fallon EM, Malkan A, Bistrian BR, Gura KM, Puder M. Parenteral fish-oil-based lipid emulsion improves fatty acid profiles and lipids in parenteral nutrition-dependent children. Am J Clin Nutr. 2011 Sep;94(3):749-58. doi: 10.3945/ajcn.110.008557. Epub 2011 Jul 20. PubMed PMID: 21775562.
 
Le HD, de Meijer VE, Zurakowski D, Meisel JA, Gura KM, Puder M. Parenteral fish oil as monotherapy improves lipid profiles in children with parenteral nutrition-associated liver disease. JPEN J Parenter Enteral Nutr. 2010 Sep-Oct;34(5):477-84. doi: 10.1177/0148607110371806. PubMed PMID: 20852175.
 
Le HD, Gura KM, Arsenault DA, Fallon EM, Potemkin AK, Puder M. Assessing portal fibrosis in parenteral nutrition-dependent patients treated with omega-3 fatty acid lipid emulsion. J Pediatr. 2010 Sep;157(3):517; author reply 518. doi: 10.1016/j.jpeds.2010.05.021. Epub 2010 Jul 2. PubMed PMID: 20584532.
 
Mehta NM, Raphael B, Guteirrez IM, Quinn N, Mitchell PD, Litman HJ, Jaksic T,  Duggan CP. Comparison of body composition assessment methods in pediatric intestinal failure. J Pediatr Gastroenterol Nutr. 2014 Jul;59(1):99-105. doi: 10.1097/MPG.0000000000000364. PubMed PMID: 24614122; PubMed Central PMCID: PMC4409423.
 
Merritt RJ, Cohran V, Raphael BP, Sentongo T, Volpert D, Warner BW, Goday PS;  Nutrition Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Intestinal Rehabilitation Programs in the Management of Pediatric Intestinal Failure and Short Bowel Syndrome. J Pediatr Gastroenterol Nutr. 2017 Nov;65(5):588-596. doi: 10.1097/MPG.0000000000001722. PubMed PMID: 28837507.
 
Miller J, Dalton MK, Duggan C, Lam S, Iglesias J, Jaksic T, Gura KM. Going with the flow or swimming against the tide: should children with central venous catheters swim? Nutr Clin Pract. 2014 Feb;29(1):97-109. doi: 10.1177/0884533613515931. Epub 2013 Dec 17. Review. PubMed PMID: 24347530; PubMed
Central PMCID: PMC4632199.
 
Murphy MK, Gura KM, Tascione C, Carey AN, Duggan CP, Raphael BP. Home Parenteral Nutrition and Intravenous Fluid Errors Discovered Through Novel Clinical Practice of Reconciling Compounding Records: A Case Series. Nutr Clin Pract. 2017 Dec;32(6):820-825. doi: 10.1177/0884533617722759. Epub 2017 Aug 29. PubMed PMID: 28850287.
 
Nandivada P, Anez-Bustillos L, O'Loughlin AA, Mitchell PD, Baker MA, Dao DT, Fell GL, Potemkin AK, Gura KM, Neufeld EJ, Puder M. Risk of post-procedural bleeding in children on intravenous fish oil. Am J Surg. 2017 Oct;214(4):733-737. doi: 10.1016/j.amjsurg.2016.10.026. Epub 2016 Dec 1. PubMed PMID: 27979360; PubMed Central PMCID: PMC5464992.
 
Nandivada P, Baker MA, Mitchell PD, O'Loughlin AA, Potemkin AK, Anez-Bustillos L, Carlson SJ, Dao DT, Fell GL, Gura KM, Puder M. Predictors of failure of fish-oil therapy for intestinal failure-associated liver disease in children. Am  J Clin Nutr. 2016 Sep;104(3):663-70. doi: 10.3945/ajcn.116.137083. Epub 2016 Aug 10. PubMed PMID: 27510535; PubMed Central PMCID: PMC4997303.
 
Nandivada P, Fell GL, Gura KM, Puder M. Lipid emulsions in the treatment and prevention of parenteral nutrition-associated liver disease in infants and children. Am J Clin Nutr. 2016 Feb;103(2):629S-34S. doi: 10.3945/ajcn.114.103986. Epub 2016 Jan 20. Review. PubMed PMID: 26791189; PubMed Central PMCID: PMC4733251.
 
Nandivada P, Fell GL, Mitchell PD, Potemkin AK, O'Loughlin AA, Gura KM, Puder  M. Long-Term Fish Oil Lipid Emulsion Use in Children With Intestinal Failure-Associated Liver Disease [Formula: see text]. JPEN J Parenter Enteral Nutr. 2017 Aug;41(6):930-937. doi: 10.1177/0148607116633796. Epub 2016 Mar 9.
PubMed PMID: 26962059.
 
Nandivada P, Potemkin AK, Carlson SJ, Chang MI, Cowan E, O'Loughlin AA, Gura KM, Puder M. Elevated Alkaline Phosphatase in Infants With Parenteral Nutrition-Associated Liver Disease Reflects Bone Rather Than Liver Disease. JPEN  J Parenter Enteral Nutr. 2015 Nov;39(8):973-6. doi: 10.1177/0148607114545995. Epub 2014 Aug 8. PubMed PMID: 25106918.
 
Raphael BP, Duggan C. Prevention and treatment of intestinal failure-associated liver disease in children. Semin Liver Dis. 2012 Nov;32(4):341-7. doi: 10.1055/s-0032-1329903. Epub 2013 Feb 8. PubMed PMID:
23397535; PubMed Central PMCID: PMC4878432.
 
Raphael BP, Murphy M, Gura KM, Litman H, Dalton MK, Finkelstein JA, Lightdale  JR. Discrepancies Between Prescribed and Actual Pediatric Home Parenteral Nutrition Solutions. Nutr Clin Pract. 2016 Oct;31(5):654-8. doi: 10.1177/0884533616639410. Epub 2016 Apr 18. PubMed PMID: 27091745.
 
Raphael BP. 3.8 Nutritional management in cholestatic liver disease. World Rev Nutr Diet. 2015;113:178-81. doi: 10.1159/000360333. Epub 2015 Apr 13. Review. PubMed PMID: 25906880.
 
Raphael BP, Mitchell PD, Finkton D, Jiang H, Jaksic T, Duggan C. Necrotizing Enterocolitis and Central Line Associated Blood Stream Infection Are Predictors of Growth Outcomes in Infants with Short Bowel Syndrome. J Pediatr. 2015 Jul;167(1):35-40.e1. doi: 10.1016/j.jpeds.2015.02.053. Epub 2015 Apr 1. PubMed PMID: 25841540; PubMed Central PMCID: PMC4794334.
 
Raphael BP, Nurko S, Jiang H, Hart K, Kamin DS, Jaksic T, Duggan C. Cisapride improves enteral tolerance in pediatric short-bowel syndrome with dysmotility. J  Pediatr Gastroenterol Nutr. 2011 May;52(5):590-4. doi: 10.1097/MPG.0b013e3181fe2d7a. PubMed PMID: 21502831; PubMed Central PMCID: PMC3079853.
 
Reimold FR, Balasubramanian S, Doroquez DB, Shmukler BE, Zsengeller ZK, Saslowsky D, Thiagarajah JR, Stillman IE, Lencer WI, Wu BL, Villalpando-Carrion S, Alper SL. Congenital chloride-losing diarrhea in a Mexican child with the novel homozygous SLC26A3 mutation G393W. Front Physiol. 2015 Jun 23;6:179. doi: 10.3389/fphys.2015.00179. eCollection 2015. PubMed PMID: 26157392; PubMed Central
PMCID: PMC4477073.
 
Squires RH, Balint J, Horslen S, Wales PW, Soden J, Duggan C, Li R, Belle SH;  Pediatric Intestinal Failure Consortium. Race affects outcome among infants with  intestinal failure. J Pediatr Gastroenterol Nutr. 2014 Oct;59(4):537-43. doi: 10.1097/MPG.0000000000000456. PubMed PMID: 24918984; PubMed Central PMCID: PMC4176512.
 
Thiagarajah JR, Donowitz M, Verkman AS. Secretory diarrhoea: mechanisms and emerging therapies. Nat Rev Gastroenterol Hepatol. 2015 Aug;12(8):446-57. doi: 10.1038/nrgastro.2015.111. Epub 2015 Jun 30. Review. PubMed PMID: 26122478; PubMed Central PMCID: PMC4786374.
 
Thiagarajah JR, Ko EA, Tradtrantip L, Donowitz M, Verkman AS. Discovery and development of antisecretory drugs for treating diarrheal diseases. Clin Gastroenterol Hepatol. 2014 Feb;12(2):204-9. doi: 10.1016/j.cgh.2013.12.001. Epub 2013 Dec 4. Review. PubMed PMID: 24316107; PubMed Central PMCID: PMC3935719.
 
Thiagarajah JR, Verkman AS. Chloride channel-targeted therapy for secretory diarrheas. Curr Opin Pharmacol. 2013 Dec;13(6):888-94. doi: 10.1016/j.coph.2013.08.005. Epub 2013 Aug 27. Review. PubMed PMID: 23992767; PubMed Central PMCID: PMC3890324.
 
Thiagarajah JR, Yildiz H, Carlson T, Thomas AR, Steiger C, Pieretti A, Zukerberg LR, Carrier RL, Goldstein AM. Altered goblet cell differentiation and surface mucus properties in Hirschsprung disease. PLoS One. 2014 Jun 19;9(6):e99944. doi: 10.1371/journal.pone.0099944. eCollection 2014. PubMed PMID: 24945437; PubMed Central PMCID: PMC4063789.