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Treatment of Cholestasis in Infants and Young Children
Heinz, Nicole; Vittorio, Jennifer
PURPOSE OF REVIEW/OBJECTIVE:Cholestasis is characterized by a conjugated hyperbilirubinemia secondary to impaired bile synthesis, transport, or excretion from the liver. It is always pathologic and can be indicative of an underlying hepatobiliary, genetic, or metabolic disorder, several of which require timely diagnosis to ensure proper management and optimal outcomes. This review provides an overview of the evaluation of cholestasis with a focus on current and emerging treatment strategies. RECENT FINDINGS/RESULTS:Increased accessibility of next generation sequencing (NGS) allows for utilization of genetic testing early in the diagnostic process. This may alter the clinical algorithm for diagnosis of cholestatic disorders. An enhanced understanding of the underlying pathophysiology may help guide future development of targeted therapies, such as ileal bile acid transporter (IBAT) inhibitors. These were recently approved for treatment of cholestatic pruritus in patients with Alagille syndrome and Progressive Familial Intrahepatic Cholestasis. Current management of cholestasis is aimed at the biochemical consequences of impaired bile flow, including malnutrition, pruritus, and progressive fibrosis. NGS has led to an enhanced understanding of biliary pathology and may guide development of future treatment modalities based on specific gene mutations. Rapid discernment of the underlying etiology is essential as new treatment modalities emerge.
PMID: 37651067
ISSN: 1534-312x
CID: 5609262
Current approach to health care transition and integration into adult care for pediatric liver transplant recipients: A call for partnership
King, Lindsay Yount; Kosmach-Park, Beverly; Parish, Alice; Niedzwiecki, Donna; Jackson, Whitney Erika; Vittorio, Jennifer Mary
Despite the increased risk of non-adherence, allograft rejection, and mortality following transfer from pediatric to adult care in liver transplantation (LT), there is no standardized approach to health care transition (HCT). Two electronic national surveys were developed and distributed to members of the Society for Pediatric Liver Transplantation and all adult LT programs in the United States to examine current HCT practices. Responses were received from 40 pediatric and 79 adult centers. Pediatric centers were more likely to focus on HCT noting the presence of a transition/transfer policy (60.2% vs. 39.2%), transition clinic (51.6% vs. 16.5%), and the routine use of transition readiness assessment tools (54.8% vs. 10.2%). Perceived barriers to HCT were similar among pediatric and adult respondents and included patient willingness to transfer and participate in care, failure to show for appointments, and lack of sufficient time and staffing. These results highlight the need for an increased awareness of HCT at both pediatric and adult LT centers. The path to improvement requires a partnership between pediatric and adult providers. Recognizing the importance of a comprehensive HCT program initiated in pediatrics and continued throughout young adulthood with ongoing support by the adult team is essential.
PMID: 37105553
ISSN: 1399-0012
CID: 5502882
Health Care Transition for Adolescents and Young Adults With Pediatric-Onset Liver Disease and Transplantation: A Position Paper by the North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition
Vittorio, Jennifer; Kosmach-Park, Beverly; King, Lindsay Y; Fischer, Ryan; Fredericks, Emily M; Ng, Vicky L; Narang, Amrita; Rasmussen, Sara; Bucuvalas, John
Advances in medical therapies and liver transplantation have resulted in a greater number of pediatric patients reaching young adulthood. However, there is an increased risk for medical complications and morbidity surrounding transfer from pediatric to adult hepatology and transplant services. Health care transition (HCT) is the process of moving from a child/family-centered model of care to an adult or patient-centered model of health care. Successful HCT requires a partnership between pediatric and adult providers across all disciplines resulting in a transition process that does not end at the time of transfer but continues throughout early adulthood. Joint consensus guidelines in collaboration with the American Society of Transplantation are presented to facilitate the adoption of a structured, multidisciplinary approach to transition planning utilizing The Six Core Elements of Health Care Transition TM for use by both pediatric and adult specialists. This paper provides guidance and seeks support for the implementation of an HCT program which spans across both pediatric and adult hepatology and transplant centers.
PMID: 35830731
ISSN: 1536-4801
CID: 5397352
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
Analysis of quality of life, hepatic biochemical markers, and sleep in patients with progressive familial intrahepatic cholestasis who had a pruritus response with odevixibat treatment [Meeting Abstract]
Gupte, Girish; Thompson, Richard J.; D\Antiga, Lorenzo; Grammatikopoulos, Tassos; Gonzales, Emmanuel; Lacaille, Florence; Lachaux, Alain; Roquelaure, Bertrand; Baumann, Ulrich; Lainka, Elke; Sturm, Ekkehard; Shteyer, Eyal; Czubkowski, Piotr; Artan, Reha; Dalgic, Buket; Ozen, Hasan; Loomes, Kathleen M.; Vittorio, Jennifer M.; Karpen, Saul J.; McKiernan, Patrick; Mack, Cara L.; Di Giorgio, Angelo; Yu, Qifeng; Kjems, Lise; Horn, Patrick
ISI:000826275102360
ISSN: 0168-8278
CID: 5397482
Changes in hepatic parameters, growth, sleep, and biochemical markers with odevixibat treatment across patients with various types of progressive familial intrahepatic cholestasis [Meeting Abstract]
D\Antiga, Lorenzo; Gupte, Girish; Thompson, Richard J.; Sturm, Ekkehard; Calvo, Pier Luigi; Shagrani, Mohammad Ali; Stoll, Janis M.; Artan, Reha; Dalgic, Buket; Ozen, Hasan; Loomes, Kathleen M.; Vittorio, Jennifer M.; Karpen, Saul J.; Di Giorgio, Angelo; Ni, Quanhong; Kjems, Lise; Horn, Patrick
ISI:000826275102359
ISSN: 0168-8278
CID: 5397472
RELATIONSHIPS BETWEEN DECREASES IN SERUM BILE ACIDS, PRURITUS, AND SLEEP DISTURBANCE SCORES WITH UP TO 72 WEEKS OF ODEVIXIBAT TREATMENT IN PATIENTS WITH PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS [Meeting Abstract]
Dalgic, Buket; Verkade, Henkjan J.; Lachaux, Alain; Baumann, Ulrich; D\Antiga, Lorenzo; Czubkowski, Piotr; Artan, Reha; Vittorio, Jennifer; Ozen, Hasan; Gupte, Girish; Grammatikopoulos, Tassos; Ni, Quanhong; Kjems, Lise; Horn, Patrick
ISI:000826446205139
ISSN: 0016-5085
CID: 5397492
BARRIERS TO IDENTIFYING AND INTERVENING ON SOCIAL NEEDS IN PEDIATRIC LIVER TRANSPLANTATION: QUALITATIVE RESULTS FROM THE MULTI-CENTER SOCIAL & CONTEXTUAL IMPACT ON CHILDREN UNDERGOING LIVER TRANSPLANTATION (SOCIAL-TX) STUDY [Meeting Abstract]
Wadhwani, Sharad; Alvarado, Alejandra; Shifman, Holly; Squires, James E.; Campbell, Kathleen M.; Ebel, Noelle; Hsu, Evelyn K.; Vittorio, Jennifer M.; Zielsdorf, Shannon; Desai, Dev M.; Bucuvalas, John C.; Gottlieb, Laura; Kotagal, Uma; Lyles, Courtney; Lai, Jennifer Cindy
ISI:000870796604420
ISSN: 0270-9139
CID: 5397502
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