Patrick J McKiernan
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Explore the profile of Patrick J McKiernan including associated specialties, affiliations and a list of published articles.
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65
Citations
904
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Recent Articles
1.
Felzen A, van Wessel D, Gonzales E, Thompson R, Jankowska I, Shneider B, et al.
JHEP Rep
. 2023 Jan;
5(2):100626.
PMID: 36687469
Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are...
2.
Squires J, McKiernan P, Squires R
Pediatrics
. 2021 Dec;
149(1 Suppl 1):S59-S65.
PMID: 34970684
Context: Develop evidence-based criteria for individual organ dysfunction. Objectives: Evaluate current evidence and develop contemporary consensus criteria for acute liver dysfunction with associated outcomes in critically ill children. Data Sources:...
3.
Bembea M, Agus M, Akcan-Arikan A, Alexander P, Basu R, Bennett T, et al.
Pediatrics
. 2021 Dec;
149(1 Suppl 1):S1-S12.
PMID: 34970673
Prior criteria for organ dysfunction in critically ill children were based mainly on expert opinion. We convened the Pediatric Organ Dysfunction Information Update Mandate (PODIUM) expert panel to summarize data...
4.
Henkel S, Salgado C, Reyes-Mugica M, Soltys K, Strauss K, Mazariegos G, et al.
Pediatr Transplant
. 2021 Aug;
25(8):e14108.
PMID: 34339082
Background: Progressive familial intrahepatic cholestasis type 1 (PFIC1) arises from biallelic variants in the ATP8B1 gene that annul FIC1 activity, resulting in progressive liver disease. Liver transplant (LT) is indicated...
5.
van Wessel D, Thompson R, Gonzales E, Jankowska I, Shneider B, Sokal E, et al.
Hepatology
. 2021 Mar;
74(2):892-906.
PMID: 33666275
Background And Aims: Mutations in ATPase phospholipid transporting 8B1 (ATP8B1) can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive familial intrahepatic cholestasis type 1. The rarity of...
6.
Raghu V, Squires J, Mogul D, Squires R, McKiernan P, Mazariegos G, et al.
Liver Transpl
. 2021 Jan;
27(5):711-718.
PMID: 33460529
Biliary atresia (BA) is the leading indication to perform a pediatric liver transplantation (LT). Timely hepatoportoenterostomy (HPE) attempts to interrupt the natural history and allow for enteric bile flow; however,...
7.
Sood V, Squires J, Mazariegos G, Vockley J, McKiernan P
J Pediatr Gastroenterol Nutr
. 2020 Sep;
72(1):11-17.
PMID: 32969959
Metabolic liver diseases (MLDs) are a heterogeneous group of inherited conditions for which liver transplantation can provide definitive treatment. The limited availability of deceased donor organs means some who could...
8.
McKiernan P
J Pediatr Gastroenterol Nutr
. 2020 Sep;
72(1):3-4.
PMID: 32960826
No abstract available.
9.
McKiernan P, Squires J, Squires R, Vockley J, Mazariegos G, Soltys K, et al.
Clin Transplant
. 2020 Sep;
34(11):e14090.
PMID: 32955727
Liver transplantation is a successful option for inherited metabolic disease yet little is published on the outcome among siblings. We report outcomes of siblings who have undergone liver transplantation for...
10.
Sreekantam S, Rizvi H, Brown R, Santra S, Raiman J, Vijay S, et al.
JIMD Rep
. 2020 Jul;
54(1):22-24.
PMID: 32685346
Our subject presented at 11 months of age, following a varicella zoster infection, with acute on chronic liver disease and was found to have raised serum chitotriosidase. White cell enzyme...