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37


CHILDREN WITH ACUTE SEVERE HEPATITIS OF UNDETERMINED ETIOLOGY HAVE A SYSTEMIC T CELL ACTIVATION AND INTERFERON GAMMA (IFN-G) ACTIVITY REMINISCENT OF HEMOPHAGOCYTIC HISTIOCYTOSIS: A POTENTIAL ROLE FOR T CELL AND IFN-G DIRECTED THERAPIES [Meeting Abstract]

Nguyen, Thinh H.; Satwani, Prakash; Kumar, Deepak; Prince, Chengyu; Remotti, Helen; Goldner, Dana; Vittorio, Jennifer M.; Romero, Rene; Chandrakasan, Shanmuganathan
ISI:000870796604460
ISSN: 0270-9139
CID: 5397512

Risk Factors for 30-Day Unplanned Readmission After Hepatectomy: Analysis of 438 Pediatric Patients from the ACS-NSQIP-P Database

Kang, Elise; Shin, John Inho; Griesemer, Adam D; Lobritto, Steven; Goldner, Dana; Vittorio, Jennifer M; Stylianos, Steven; Martinez, Mercedes
BACKGROUND:Hepatic resections are uncommon in children. Most studies reporting complications of these procedures and risk factors associated with unplanned readmissions are limited to retrospective data from single centers. We investigated risk factors for 30-day unplanned readmission after hepatectomy in children using the American College of Surgeons National Surgical Quality Improvement-Pediatric database. METHODS:The database was queried for patients aged 0-18 years who underwent hepatectomy for the treatment of liver lesions from 2012 to 2018. Chi-squared tests were performed to evaluate for potential risk factors for unplanned readmissions. A multivariate regression analysis was performed to identify independent predictors for unplanned 30-day readmissions. RESULTS:Among 438 children undergoing hepatectomy, 64 (14.6%) had unplanned readmissions. The median age of the hepatectomy cohort was 1 year (0-17); 55.5% were male. Patients readmitted had significantly higher rates of esophageal/gastric/intestinal disease (26.56% vs. 14.97%; p=0.022), current cancer (85.94% vs. 75.67%; p=0.012), and enteral and parenteral nutritional support (31.25% vs. 17.65%; p=0.011). Readmitted patients had significantly higher rates of perioperative blood transfusion (67.19% vs. 52.41%; p=0.028), organ/space surgical site infection (10.94% vs. 1.07%; p<.001), sepsis (15.63% vs. 3.74%; p<.001), and total parenteral nutrition at discharge (9.09% vs. 2.66%; p=0.041). Organ/space surgical site infection was an independent risk factor for unplanned readmission (OR=9.598, CI [2.070-44.513], p=0.004) by multivariable analysis. CONCLUSION:Unplanned readmissions after liver resection are frequent in pediatric patients. Organ/space surgical site infections may identify patients at increased risk for unplanned readmission. Strategies to reduce these complications may decrease morbidity and costs associated with unplanned readmissions.
PMID: 33825121
ISSN: 1873-4626
CID: 5151322

Bone Fractures in Children With Cholestatic Liver Disease May Mimic Those Seen in Child Abuse

Goldner, Dana; Vittorio, Jennifer; Barrios, Dulce M; McGuire, Jillian; Brodlie, Susan; Brown, Jocelyn; Lobritto, Steven; Martinez, Mercedes
Certain fractures in children are highly specific for child abuse. Metabolic bone disease frequently develops in patients with cholestatic liver disease (CLD); this can result in weakened bones and a predisposition to pathologic fractures. Fractures that occur in patients with rickets and osteopenia may mimic a bone response to inflicted injury, which in children raise the concern of child abuse. Here we report a series of 15 patients with CLD who developed pathologic fractures in the setting of metabolic bone disease. During initial evaluation, the caretakers of 5 of these 15 patients were reported to child protective services and investigated for child abuse. Pediatricians should be aware that children with CLD have an increased incidence of pathologic fractures, even after the cholestasis has resolved.
PMID: 30672906
ISSN: 1535-1815
CID: 5397302

Embolization of a rare case of focal nodular hyperplasia in an adolescent boy

Shanmugasundaram, Srinidhi; Gioioso, Valeria; Martinez, Mercedes; Lobritto, Steven; Vittorio, Jennifer; Goldner, Dana; Griesemer, Adam; Tulin-Silver, Sheryl
ISI:000600790400032
ISSN: 2213-5766
CID: 5161252

Substantial clinical benefits with odevixibat treatment across progressive familial intrahepatic cholestasis genetic deficiencies: subgroup analysis of serum bile acids, pruritus, and safety using pooled data from the PEDFIC 1 and 2 studies [Meeting Abstract]

Thompson, Richard; Horn, Patrick; Houwen, Roderick H. J.; Lacaille, Florence; Ni, Quanhong; Stein, Philip; Tessier, Mary Elizabeth; Thompson, Carrie; Vittorio, Jennifer; Kjems, Lise
ISI:000667753801336
ISSN: 0168-8278
CID: 5397432

A HIGH DIAGNOSTIC RATE FOR MENDELIAN LIVER DISORDERS: LESSONS FROM EXOME SEQUENCING IN 10,804 INDIVIDUALS WITH OR WITHOUT LIVER DISEASES [Meeting Abstract]

Kong, Xiao-Fei; Bogyo, Kelsie; Rasouly, Hila Milo; Cocchi, Enrico; Martinez, Mercedes; Vittorio, Jennifer M.; Dove, Lorna M.; Shea, Patrick; Bier, Louise; Worman, Howard J.; Marasa, Maddalena; Wang, Timothy C.; Emond, Jean C.; Verna, Elizabeth C.; Wattacheril, Julia J.; Goldstein, David; Gharavi, Ali G.
ISI:000707188001322
ISSN: 0270-9139
CID: 5397442

CURRENT APPROACH TO HEALTH CARE TRANSITION FOR PEDIATRIC LIVER TRANSPLANT RECIPIENTS: A CALL FOR PARTNERSHIP [Meeting Abstract]

King, Lindsay Y.; Jackson, Whitney E.; Parish, Alice; Niedzwiecki, Donna; Vittorio, Jennifer M.
ISI:000707188005306
ISSN: 0270-9139
CID: 5397452

EFFECTS ON SERUM BILE ACIDS, PRURITUS, AND SAFETY WITH UP TO 72 WEEKS OF ODEVIXIBAT TREATMENT: POOLED DATA FROM THE PEDFIC 1 AND PEDFIC 2 STUDIES IN CHILDREN WITH PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS [Meeting Abstract]

Loomes, Kathleen M.; Verkade, Henkjan J.; Thompson, Richard J.; Kamath, Binita; Hardikar, Winita; Lacaille, Florence; Mozer-Glassberg, Yael; Shteyer, Eyal; Calvo, Pier Luigi; Dalgic, Buket; Grammatikopoulos, Tassos; Rajwal, Sanjay; Vittorio, Jennifer M.; Soufi, Nisreen; McKiernan, Patrick James; Tessier, Mary Elizabeth; Yu, Qifeng; Kjems, Lise; Horn, Patrick
ISI:000707188005309
ISSN: 0270-9139
CID: 5397462

A case of an Infant with SARS-CoV-2 hepatitis early after liver transplantation [Case Report]

Heinz, Nicole; Griesemer, Adam; Kinney, Joanna; Vittorio, Jennifer; Lagana, Stephen M; Goldner, Dana; Velasco, Monica; Kato, Tomoaki; Lobritto, Steven; Martinez, Mercedes
We present a case of a pediatric liver transplant recipient diagnosed with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection four days after receiving a living donor liver allograft from her mother. The recipient was a 6-month-old with end-stage liver disease due to biliary atresia and failed Kasai. The infant had an uncomplicated implantation, excellent graft function and down-trending liver enzymes until developing fevers, diarrhea, and moderate respiratory distress requiring non-invasive respiratory support. SARS-CoV-2 testing (nasal swab Polymerase Chain Reaction) was positive on post-operative day (POD) 4. Liver enzymes peaked ~1000 U/L (5-fold higher than the previous day) on POD 6. Histology demonstrated a mixed picture of moderate acute hepatitis and classical elements of mild to moderate acute cellular rejection. Her hepatitis and respiratory symptoms improved coincident with completing treatment with hydroxychloroquine, reduced immunosuppression, and intravenous gamma globulin (IVIG).
PMCID:7323125
PMID: 32559354
ISSN: 1399-3046
CID: 5151252

Role of Budesonide for the Treatment of Rejection in Pediatric Liver Transplantation

Chen, Justin; Ferreira, Johanna; Martinez, Mercedes; Lobritto, Steven; Goldner, Dana; Vittorio, Jennifer
INTRODUCTION:Corticosteroids are an integral part of liver transplant (LT) immunosuppression regimens but are often accompanied by many adverse effects. Budesonide is an oral corticosteroid with extensive (80%-90%) hepatic first-pass metabolism and minimal systemic absorption. The aim of this study was to examine the safety and efficacy of budesonide for management of acute cellular rejection (ACR) in pediatric LT recipients. METHODS:A retrospective descriptive analysis was performed for all pediatric patients who underwent LT at our center and were prescribed oral budesonide for the treatment of ACR. Alanine aminotransferase (ALT) values and documented adverse effects were reviewed. RESULTS:Twenty-nine patients were prescribed budesonide for the treatment of ACR; 65.5% with biopsy-proven acute rejection and 34.5% with presumed ACR. There was a significant decrease in ALT noted from the time of rejection when compared to values 1 month (P = 0.0011), 3 months (P = 0.0003), and 6 months (P = 0.0001) after treatment with budesonide. There was no difference noted between patient baseline ALT levels before rejection when compared to 1, 3, and 6 months posttreatment values suggesting resolution of rejection. Three patients required conversion from budesonide to systemic steroids. There were no discontinuations of budesonide secondary to adverse effects. CONCLUSION:Oral budesonide may be a promising alternative to systemic corticosteroids for the management of mild/moderate ACR and for empiric treatment of ACR in select pediatric LT recipients. Data from this study may provide the foundation for larger, prospective, multicenter trials to assess the effectiveness of budesonide in the treatment of ACR.
PMID: 32404767
ISSN: 1536-4801
CID: 5397332