Format
Sort by
Items per page

Send to

Choose Destination

Search results

Items: 1 to 50 of 220

1.

Technique and outcome of domino liver transplantation from patients with maple syrup urine disease: Expanding the donor pool for live donor liver transplantation.

Celik N, Kelly B, Soltys K, Squires JE, Vockley J, Shellmer DA, Strauss K, McKiernan P, Ganoza A, Sindhi R, Bond G, Mazariegos G, Khanna A.

Clin Transplant. 2019 Sep 25:e13721. doi: 10.1111/ctr.13721. [Epub ahead of print]

PMID:
31556146
2.

Crigler-Najjar syndrome type 1: pathophysiology, natural history, and therapeutic frontier.

Strauss KA, Ahlfors CE, Soltys K, Mazareigos GV, Young M, Bowser LE, Fox MD, Squires JE, McKiernan P, Brigatti KW, Puffenberger EG, Carson VJ, Vreman HJ.

Hepatology. 2019 Sep 25. doi: 10.1002/hep.30959. [Epub ahead of print]

PMID:
31553814
3.

Disease burden of Crigler-Najjar syndrome: Systematic review and future perspectives.

Dhawan A, Lawlor MW, Mazariegos GV, McKiernan P, Squires JE, Strauss KA, Gupta D, James E, Prasad S.

J Gastroenterol Hepatol. 2019 Sep 8. doi: 10.1111/jgh.14853. [Epub ahead of print]

PMID:
31495946
4.

Domino liver transplantation for select metabolic disorders: Expanding the living donor pool.

Celik N, Squires JE, Soltys K, Vockley J, Shellmer DA, Chang W, Strauss K, McKiernan P, Ganoza A, Sindhi R, Bond G, Mazariegos G, Khanna A.

JIMD Rep. 2019 Jun 19;48(1):83-89. doi: 10.1002/jmd2.12053. eCollection 2019 Jul.

5.

The Estrogen-Induced miR-19 Downregulates Secretory Leucoprotease Inhibitor Expression in Monocytes.

McKiernan PJ, Smith SGJ, Durham AL, Adcock IM, McElvaney NG, Greene CM.

J Innate Immun. 2019 Jul 2:1-13. doi: 10.1159/000500419. [Epub ahead of print]

6.

A novel mutation in VPS33B gene causing a milder ARC syndrome phenotype with prolonged survival.

Del Brío Castillo R, Squires JE, McKiernan PJ.

JIMD Rep. 2019 Mar 22;47(1):4-8. doi: 10.1002/jmd2.12027. eCollection 2019 May.

7.

Expanding etiology of progressive familial intrahepatic cholestasis.

Henkel SA, Squires JH, Ayers M, Ganoza A, Mckiernan P, Squires JE.

World J Hepatol. 2019 May 27;11(5):450-463. doi: 10.4254/wjh.v11.i5.450.

8.

Phase I/II Trial of Liver-derived Mesenchymal Stem Cells in Pediatric Liver-based Metabolic Disorders: A Prospective, Open Label, Multicenter, Partially Randomized, Safety Study of One Cycle of Heterologous Human Adult Liver-derived Progenitor Cells (HepaStem) in Urea Cycle Disorders and Crigler-Najjar Syndrome Patients.

Smets F, Dobbelaere D, McKiernan P, Dionisi-Vici C, Broué P, Jacquemin E, Lopes AI, Gonçalves I, Mandel H, Pawlowska J, Kamińska D, Shteyer E, Torre G, Shapiro R, Eyskens F, Clapuyt P, Gissen P, Pariente D, Grunewald S, Yudkoff M, Binda MM, Najimi M, Belmonte N, de Vos B, Thonnard J, Sokal E.

Transplantation. 2019 Sep;103(9):1903-1915. doi: 10.1097/TP.0000000000002605.

PMID:
30801523
9.

Evolving Trends in Liver Transplant for Metabolic Liver Disease in the United States.

McKiernan PJ, Ganoza A, Squires JE, Squires RH, Vockley J, Mazariegos G, Soltys K, Sun Q, Sindhi R.

Liver Transpl. 2019 Jun;25(6):911-921. doi: 10.1002/lt.25433. Epub 2019 Apr 15.

PMID:
30753750
10.

Immune Status in Children Before Liver Transplantation-A Cross-Sectional Analysis Within the ChilsSFree Multicentre Cohort Study.

Möhring T, Karch A, Falk CS, Laue T, D'Antiga L, Debray D, Hierro L, Kelly D, McLin V, McKiernan P, Pawlowska J, Czubkowski P, Mikolajczyk RT, Baumann U, Goldschmidt I.

Front Immunol. 2019 Jan 25;10:52. doi: 10.3389/fimmu.2019.00052. eCollection 2019.

11.

Hepatic Lesions Associated With McCune Albright Syndrome.

Johansen L, Haller W, Thyagarajan M, Kelly D, McKiernan P.

J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):e54-e57. doi: 10.1097/MPG.0000000000002266.

PMID:
30628989
12.

Knockdown of Gene Expression in Macrophages by microRNA Mimic-Containing Poly (Lactic-co-glycolic Acid) Microparticles.

McKiernan PJ, Lynch P, Ramsey JM, Cryan SA, Greene CM.

Medicines (Basel). 2018 Dec 15;5(4). pii: E133. doi: 10.3390/medicines5040133.

13.

Ruptured intracranial aneurysm in a patient with autosomal recessive polycystic kidney disease.

Perez JL, McDowell MM, Zussman B, Jadhav AP, Miyashita Y, McKiernan P, Greene S.

J Neurosurg Pediatr. 2018 Oct 12;23(1):75-79. doi: 10.3171/2018.8.PEDS18286. Review.

PMID:
30497224
14.

Molecular Mechanisms in Pediatric Cholestasis.

Squires JE, McKiernan P.

Gastroenterol Clin North Am. 2018 Dec;47(4):921-937. doi: 10.1016/j.gtc.2018.07.014. Epub 2018 Sep 28. Review.

PMID:
30337041
15.

Acute Liver Failure: An Update.

Squires JE, McKiernan P, Squires RH.

Clin Liver Dis. 2018 Nov;22(4):773-805. doi: 10.1016/j.cld.2018.06.009. Epub 2018 Aug 22. Review.

PMID:
30266162
16.

Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes.

Critelli K, McKiernan P, Vockley J, Mazariegos G, Squires RH, Soltys K, Squires JE.

Liver Transpl. 2018 Sep;24(9):1260-1270. doi: 10.1002/lt.25304.

17.

Hepatopulmonary Syndrome in Children: A 20-Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome.

Warner S, McKiernan PJ, Hartley J, Ong E, van Mourik ID, Gupte G, Abdel-Hady M, Muiesan P, Perera T, Mirza D, Sharif K, Kelly DA, Beath SV.

Liver Transpl. 2018 Sep;24(9):1271-1279. doi: 10.1002/lt.25296.

18.

The Experiences of Young Liver Patients Transferring From Children's to Adult Services and Their Support Needs for a Successful Transition.

Toft A, Taylor R, Claridge L, Clowes C, Ferguson J, Hind J, Jones R, McClean P, McKiernan P, Samyn M, Coad J.

Prog Transplant. 2018 Sep;28(3):244-249. doi: 10.1177/1526924818781567. Epub 2018 Jun 13.

PMID:
29898638
19.

Immune monitoring after pediatric liver transplantation - the prospective ChilSFree cohort study.

Goldschmidt I, Karch A, Mikolajczyk R, Mutschler F, Junge N, Pfister ED, Möhring T, d'Antiga L, McKiernan P, Kelly D, Debray D, McLin V, Pawlowska J, Hierro L, Daemen K, Keil J, Falk C, Baumann U.

BMC Gastroenterol. 2018 May 16;18(1):63. doi: 10.1186/s12876-018-0795-x.

20.

Clinical Hepatocyte Transplantation: What Is Next?

Squires JE, Soltys KA, McKiernan P, Squires RH, Strom SC, Fox IJ, Soto-Gutierrez A.

Curr Transplant Rep. 2017 Dec;4(4):280-289. doi: 10.1007/s40472-017-0165-6. Epub 2017 Oct 14.

21.

Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

Johansen LC, McKiernan PJ, Sharif K, McGuirk SP.

J Pediatr Gastroenterol Nutr. 2018 Aug;67(2):173-179. doi: 10.1097/MPG.0000000000002006.

PMID:
29668568
22.

The Health Care Transition of Youth With Liver Disease Into the Adult Health System: Position Paper From ESPGHAN and EASL.

Vajro P, Fischler B, Burra P, Debray D, Dezsofi A, Guercio Nuzio S, Hadzic N, Hierro L, Jahnel J, Lamireau T, McKiernan P, McLin V, Nobili V, Socha P, Smets F, Baumann U, Verkade HJ.

J Pediatr Gastroenterol Nutr. 2018 Jun;66(6):976-990. doi: 10.1097/MPG.0000000000001965.

PMID:
29570559
23.

Healthcare resource use and costs of managing children and adults with lysosomal acid lipase deficiency at a tertiary referral centre in the United Kingdom.

Guest JF, Ingram A, Ayoub N, Hendriksz CJ, Murphy E, Rahman Y, McKiernan P, Mundy H, Deegan P.

PLoS One. 2018 Feb 2;13(2):e0191945. doi: 10.1371/journal.pone.0191945. eCollection 2018.

24.

Survival of children after liver transplantation for hepatocellular carcinoma.

Baumann U, Adam R, Duvoux C, Mikolajczyk R, Karam V, D'Antiga L, Chardot C, Coker A, Colledan M, Ericzon BG, Line PD, Hadzic N, Isoniemi H, Klempnauer JL, Reding R, McKiernan PJ, McLin V, Paul A, Salizzoni M, Furtado ESB, Schneeberger S, Karch A; European Liver and Intestine Transplant Association.

Liver Transpl. 2018 Feb;24(2):246-255. doi: 10.1002/lt.24994.

25.

Hepatic Parenchymal Injury in Crigler-Najjar Type I.

Mitchell E, Ranganathan S, McKiernan P, Squires RH, Strauss K, Soltys K, Mazariegos G, Squires JE.

J Pediatr Gastroenterol Nutr. 2018 Apr;66(4):588-594. doi: 10.1097/MPG.0000000000001843.

PMID:
29176474
26.

Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day.

Kienstra NS, van Reemst HE, van Ginkel WG, Daly A, van Dam E, MacDonald A, Burgerhof JGM, de Blaauw P, McKiernan PJ, Heiner-Fokkema MR, van Spronsen FJ.

J Inherit Metab Dis. 2018 Mar;41(2):181-186. doi: 10.1007/s10545-017-0112-9. Epub 2017 Nov 23.

27.

Portal hypertension and its management in children.

Grammatikopoulos T, McKiernan PJ, Dhawan A.

Arch Dis Child. 2018 Feb;103(2):186-191. doi: 10.1136/archdischild-2015-310022. Epub 2017 Aug 16. Review.

PMID:
28814423
28.

X Chromosome-encoded MicroRNAs Are Functionally Increased in Cystic Fibrosis Monocytes.

McKiernan PJ, Molloy KP, Cryan SA, McElvaney NG, Greene CM.

Am J Respir Crit Care Med. 2018 Mar 1;197(5):668-670. doi: 10.1164/rccm.201707-1417LE. No abstract available.

PMID:
28796536
29.

Liver Transplantation for Hereditary Tyrosinaemia Type 1 in the United Kingdom.

McKiernan P.

Adv Exp Med Biol. 2017;959:85-91. doi: 10.1007/978-3-319-55780-9_7.

PMID:
28755186
30.

Sequencing of FIC1, BSEP and MDR3 in a large cohort of patients with cholestasis revealed a high number of different genetic variants.

Dröge C, Bonus M, Baumann U, Klindt C, Lainka E, Kathemann S, Brinkert F, Grabhorn E, Pfister ED, Wenning D, Fichtner A, Gotthardt DN, Weiss KH, McKiernan P, Puri RD, Verma IC, Kluge S, Gohlke H, Schmitt L, Kubitz R, Häussinger D, Keitel V.

J Hepatol. 2017 Dec;67(6):1253-1264. doi: 10.1016/j.jhep.2017.07.004. Epub 2017 Jul 19.

PMID:
28733223
31.

Using Mobile Phone Technology to Support Young Liver Transplant Recipients Moving to Adult Services.

Coad J, Toft A, Claridge L, Ferguson J, Hind J, Jones R, McClean P, McKiernan P, Samyn M, Taylor R.

Prog Transplant. 2017 Jun;27(2):207-218. doi: 10.1177/1526924817699958. Epub 2017 Mar 22.

PMID:
28617171
32.

Long-term outcomes of de novo autoimmune hepatitis in pediatric liver transplant recipients.

Ekong UD, McKiernan P, Martinez M, Lobritto S, Kelly D, Ng VL, Alonso EM, Avitzur Y.

Pediatr Transplant. 2017 Sep;21(6). doi: 10.1111/petr.12945. Epub 2017 May 29.

33.

Long-term challenges and perspectives of pre-adolescent liver disease.

Hadžić N, Baumann U, McKiernan P, McLin V, Nobili V.

Lancet Gastroenterol Hepatol. 2017 Jun;2(6):435-445. doi: 10.1016/S2468-1253(16)30160-1. Review.

PMID:
28497759
34.

Early and Late Factors Impacting Patient and Graft Outcome in Pediatric Liver Transplantation: Summary of an ESPGHAN Monothematic Conference.

McLin VA, Allen U, Boyer O, Bucuvalas J, Colledan M, Cuturi MC, d'Antiga L, Debray D, Dezsofi A, Goyet JV, Dhawan A, Durmaz O, Falk C, Feng S, Fischler B, Franchi-Abella S, Frauca E, Ganschow R, Gottschalk S, Hadzic N, Hierro L, Horslen S, Hubscher S, Karam V, Kelly D, Maecker-Kolhoff B, Mazariegos G, McKiernan P, Melk A, Nobili V, Ozgenç F, Reding R, Sciveres M, Sharif K, Socha P, Toso C, Vajro P, Verma A, Wildhaber BE, Baumann U.

J Pediatr Gastroenterol Nutr. 2017 Sep;65(3):e53-e59. doi: 10.1097/MPG.0000000000001564.

PMID:
28319600
35.

Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European Survey.

Jahnel J, Zöhrer E, Fischler B, D'Antiga L, Debray D, Dezsofi A, Haas D, Hadzic N, Jacquemin E, Lamireau T, Maggiore G, McKiernan PJ, Calvo PL, Verkade HJ, Hierro L, McLin V, Baumann U, Gonzales E.

J Pediatr Gastroenterol Nutr. 2017 Jun;64(6):864-868. doi: 10.1097/MPG.0000000000001546.

PMID:
28267072
36.

UK guideline on transition of adolescent and young persons with chronic digestive diseases from paediatric to adult care.

Brooks AJ, Smith PJ, Cohen R, Collins P, Douds A, Forbes V, Gaya DR, Johnston BT, McKiernan PJ, Murray CD, Sebastian S, Smith M, Whitley L, Williams L, Russell RK, McCartney SA, Lindsay JO.

Gut. 2017 Jun;66(6):988-1000. doi: 10.1136/gutjnl-2016-313000. Epub 2017 Feb 21. Review.

37.

What Is the Best Blood Sampling Time for Metabolic Control of Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients?

van Dam E, Daly A, Venema-Liefaard G, van Rijn M, Derks TGJ, McKiernan PJ, Rebecca Heiner-Fokkema M, MacDonald A, van Spronsen FJ.

JIMD Rep. 2017;36:49-57. doi: 10.1007/8904_2016_37. Epub 2017 Jan 25.

38.

Expression of X-linked Toll-like receptor 4 signaling genes in female vs. male neonates.

O'Driscoll DN, De Santi C, McKiernan PJ, McEneaney V, Molloy EJ, Greene CM.

Pediatr Res. 2017 May;81(5):831-837. doi: 10.1038/pr.2017.2. Epub 2017 Jan 6.

PMID:
28060792
39.

Reply.

Kelly D, Hartleif S, Verkade HJ, Rajanayagam J, McKiernan P, Mazariegos G, Hübscher S.

Liver Transpl. 2017 Mar;23(3):405-406. doi: 10.1002/lt.24705. No abstract available.

40.

MiRNA Expression in Cystic Fibrosis Bronchial Epithelial Cells.

Oglesby IK, McKiernan PJ.

Methods Mol Biol. 2017;1509:57-69.

PMID:
27826918
41.
42.

Late graft hepatitis and fibrosis in pediatric liver allograft recipients: Current concepts and future developments.

Kelly D, Verkade HJ, Rajanayagam J, McKiernan P, Mazariegos G, Hübscher S.

Liver Transpl. 2016 Nov;22(11):1593-1602. doi: 10.1002/lt.24616. Review. Erratum in: Liver Transpl. 2017 Feb;23 (2):270.

43.

Incidence of Primary Mitochondrial Disease in Children Younger Than 2 Years Presenting With Acute Liver Failure.

McKiernan P, Ball S, Santra S, Foster K, Fratter C, Poulton J, Craig K, McFarland R, Rahman S, Hargreaves I, Gupte G, Sharif K, Taylor RW.

J Pediatr Gastroenterol Nutr. 2016 Dec;63(6):592-597.

44.

Successful long-term outcome of liver transplantation in late-onset lysosomal acid lipase deficiency.

Sreekantam S, Nicklaus-Wollenteit I, Orr J, Sharif K, Vijay S, McKiernan PJ, Santra S.

Pediatr Transplant. 2016 Sep;20(6):851-4. doi: 10.1111/petr.12748. Epub 2016 Jul 9.

PMID:
27392817
45.

Neurocognitive outcome in tyrosinemia type 1 patients compared to healthy controls.

van Ginkel WG, Jahja R, Huijbregts SC, Daly A, MacDonald A, De Laet C, Cassiman D, Eyskens F, Körver-Keularts IM, Goyens PJ, McKiernan PJ, van Spronsen FJ.

Orphanet J Rare Dis. 2016 Jun 29;11(1):87. doi: 10.1186/s13023-016-0472-5.

46.

Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome.

Sentner CP, Hoogeveen IJ, Weinstein DA, Santer R, Murphy E, McKiernan PJ, Steuerwald U, Beauchamp NJ, Taybert J, Laforêt P, Petit FM, Hubert A, Labrune P, Smit GPA, Derks TGJ.

J Inherit Metab Dis. 2016 Sep;39(5):697-704. doi: 10.1007/s10545-016-9932-2. Epub 2016 Apr 22.

47.

Combined liver and kidney transplantation in children: analysis of renal graft outcome.

Ranawaka R, Lloyd C, McKiernan PJ, Hulton SA, Sharif K, Milford DV.

Pediatr Nephrol. 2016 Sep;31(9):1539-43. doi: 10.1007/s00467-016-3396-3. Epub 2016 Apr 22.

PMID:
27105881
48.

High-throughput profiling for discovery of non-coding RNA biomarkers of lung disease.

McKiernan PJ, Greene CM.

Expert Rev Mol Diagn. 2016;16(2):173-85. doi: 10.1586/14737159.2016.1122526. Epub 2015 Dec 10. Review.

PMID:
26581119
49.

Recurrent acute liver failure due to NBAS deficiency: phenotypic spectrum, disease mechanisms, and therapeutic concepts.

Staufner C, Haack TB, Köpke MG, Straub BK, Kölker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Lenz D, Kühr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Ozolek JA, Zschocke J, Kuster A, Dick A, Das AM, Wieland T, Terrile C, Strom TM, Meitinger T, Prokisch H, Hoffmann GF.

J Inherit Metab Dis. 2016 Jan;39(1):3-16. doi: 10.1007/s10545-015-9896-7. Epub 2015 Nov 5.

PMID:
26541327
50.

Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium.

Shneider BL, de Ville de Goyet J, Leung DH, Srivastava A, Ling SC, Duché M, McKiernan P, Superina R, Squires RH, Bosch J, Groszmann R, Sarin SK, de Franchis R, Mazariegos GV.

Hepatology. 2016 Apr;63(4):1368-80. doi: 10.1002/hep.28153. Epub 2015 Oct 23. Review.

PMID:
26358549

Supplemental Content

Loading ...
Support Center