Children with protein-losing enteropathy after the Fontan operation are at risk for abnormal bone mineral density.

2012

1264-8

2012 Dec

1432-1971

<p>Protein-losing enteropathy (PLE) is a rare but potentially devastating complication of single-ventricle physiology after the Fontan operation. Although abnormal bone mineral density (BMD) is a known complication of chronic disease and congenital heart disease, no reports have described BMD in patients with PLE. This study investigated a cross-sectional sample of children and young adults with a confirmed diagnosis of PLE. Serum levels of 25(OH)D, calcium, total protein, and albumin were recorded from the first outpatient encounter with each subject. Corrected calcium (cCa) was calculated from the serum calcium and albumin levels. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD, and z-scores were generated using appropriate software. DXA results were available for 12 patients (eight males and four females). The age at DXA ranged from 7.2 to 25.2 years. The mean z-score was -1.73 standard deviation (SD) for the entire cohort, with 42 % z-scores below -2 SDs. Serum 25(OH)D levels were abnormal in 58 % of the patients. There was a positive correlation between cCa and DXA z-score and a negative correlation between total protein and DXA z-score. Patients receiving corticosteroid therapy had a significantly lower DXA z-score than those not receiving corticosteroids (-3.15 vs. -0.31; p = 0.02). Children with PLE are at risk for abnormal BMD compared with age- and sex-matched control subjects. In the study cohort, corticosteroid exposure, a marker of disease severity, appeared to be associated with decreased BMD. Routine bone health screening is warranted for children with PLE, particularly those receiving corticosteroid therapy.</p>

10.1007/s00246-012-0290-z

Pediatr Cardiol

22434509

Live Virus Vaccination Following Pediatric Liver Transplantation: Outcomes from Two Academic Children's Hospital.

2021

2021 Dec 29

1600-6143

<p>Pediatric liver transplant recipients are often transplanted at a young age, precluding them from receiving live virus vaccinations (LVV) such as varicella (VZV) vaccine and measles, mumps and rubella (MMR). This places them at profound risk for vaccine preventable illness. We sought to detail safety of vaccination. This was a retrospective cohort study of pediatric liver transplant recipients at two children's hospitals.&nbsp;Among 204 liver transplant recipients included in the study, 97 received at least one LVV after liver transplant. Six patients who did not receive LVV after transplant had evidence of vaccine-preventable infection following vaccination (1 disseminated VZV disease, 5 VZV-related rash), while one patient who received LVV after transplant developed a diffuse VZV-related rash. Rejection rates were the same between those that did and did not receive a live virus vaccine post-transplant. There were no serious adverse events caused by vaccination post-transplant.&nbsp;Live virus vaccination following liver transplant was safe at our two institutions, although there exist limitations in our study due to its retrospective study design. Larger scale studies should be performed to evaluate the effectiveness of LVV in relation to immunosuppression.</p>

10.1111/ajt.16937

Am J Transplant

34967134

Center Variability in Acute Rejection and Biliary Complications after Pediatric Liver Transplantation.

2021

2021 Aug 08

1527-6473

<p>Transplant center performance and practice variation for pediatric post-liver transplantation (LT) outcomes other than survival are understudied. This was a retrospective cohort study of pediatric LT recipients between 1/1/2006-5/31/2017 using United Network for Organ Sharing (UNOS) data that was merged with the Pediatric Health Information System database. Center effects at 1 year post-LT for acute rejection (AR1) using UNOS coding and biliary complications (BC1) using inpatient biling claims data were estimated by center-specific rescaled odds ratios that accounted for potential differences in recipient and donor characteristics. There were 2,216 pediatric LT recipients at 24 free-standing children's hospitals in the US during the study period. The median unadjusted center rate of AR1 was 36.92% (IQR: 22.36-44.52%), while that of BC1 was 32.29% (IQR: 26.14-40.44%). Accounting for recipient case-mix and donor factors, 5/24 centers performed better-than-expected with regards to AR1, while 3/24 centers performed worse-than-expected. There was less heterogeneity across the center effects for BC1 than for AR1. There was no relationship observed between center effects for AR1 or BC1 and center volume. CONCLUSION: Beyond recipient and allograft factors, differences in transplant center management are an important driver of center AR1 performance, and less so of BC1 performance. Further research is needed to identify the sources of variability so as to implement the most effective solutions to broadly enhance outcomes for pediatric LT recipients.</p>

10.1002/lt.26259

Liver Transpl

34365719

Early Detection of SARS-CoV-2 and other Infections in Solid Organ Transplant Recipients and Household Members using Wearable Devices.

2021

2021 Mar 18

1432-2277

<p>The increasing global prevalence of SARS-CoV-2 and the resulting COVID-19 disease pandemic pose significant concerns for clinical management of solid organ transplant recipients (SOTR). Wearable devices that can measure physiologic changes in biometrics including heart rate, heart rate variability, body temperature, respiratory, activity (such as steps taken per day) and sleep patterns and blood oxygen saturation, show utility for the early detection of infection before clinical presentation of symptoms. Recent algorithms developed using preliminary wearable datasets show that SARS-CoV-2 is detectable before clinical symptoms in &gt;80% of adults. Early detection of SARS-CoV-2, influenza, and other pathogens in SOTR, and their household members, could facilitate early interventions such as self-isolation and early clinical management of relevant infection(s). Ongoing studies testing the utility of wearable devices such as smartwatches for early detection of SARS-CoV-2 and other infections in the general population are reviewed here, along with the practical challenges to implementing these processes at scale in pediatric and adult SOTR, and their household members. The resources and logistics, including transplant specific analyses pipelines to account for confounders such as polypharmacy and comorbidities, required in studies of pediatric and adult SOTR for the robust early detection of SARS-CoV-2 and other infections are also reviewed.</p>

10.1111/tri.13860

Transpl Int

33735480

Defining the role of liver biopsy in the assessment of liver fibrosis in patients with Fontan circulation-reply.

2017

2017 Jun 08

1532-8392

10.1016/j.humpath.2017.04.029

Hum. Pathol.

28603067

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study.

2017

2017 Apr 26

2047-9980

<p><strong>BACKGROUND: </strong>Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking.</p>

<p><strong>METHODS AND RESULTS: </strong>Liver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10&nbsp;years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5&nbsp;years and mean time from Fontan of 14.9±4.5&nbsp;years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (r=0.33, P&lt;0.01). Serum liver enzymes and platelet count did not correlate with %CD. The median inferior vena cava pressure was 13&nbsp;mm&nbsp;Hg (range 6-24&nbsp;mm&nbsp;Hg) and did not correlate with %CD. There was no difference in %CD based on ventricular morphology or severity of atrioventricular valve insufficiency.</p>

<p><strong>CONCLUSIONS: </strong>In this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.</p>

10.1161/JAHA.116.004809

J Am Heart Assoc

28446492

Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation.

2016

2016 Jul 27

1532-8392

<p>The Fontan operation is a widely used palliative procedure in patients with single ventricle anatomy that results in liver injury. As timely identification of liver fibrosis may result in management changes to Fontan patients, the aim of our study was to identify clinically meaningful semi-quantitative/quantitative pathologic parameters for biopsy assessment. We performed a retrospective review of 74 liver needle biopsies from Fontan patients. Fibrosis was assessed using quantitative % collagen deposition (%CD) by Sirius red image analysis, METAVIR, congestive hepatic fibrosis score (CHFS), sinusoidal fibrosis (SF) score, and sinusoidal dilation score. Contemporaneous laboratory, hemodynamic, and ultrasound data were collected. Centrilobular and peri-sinusoidal fibrosis was observed in all cases, with 39.2% high grade. Portal fibrosis was observed in 93.2%, with 36.2% high grade (METAVIR F3-F4). Cirrhosis was observed in 5.4%. %CD was increased over control tissue (p&lt;0.001) and correlated with time from Fontan (r=0.3, p=0.009) and prothrombin time (PT) (r=0.25, p=0.034). Mildly elevated PT/INR was the only measure of liver function consistently associated with multiple high-grade fibrosis scores (METAVIR p=0.046, SF p=0.018). Abnormal liver echotexture on ultrasound was associated with high grade CHFS (p=0.03). Pathologic gradings and %CD correlated with each other (r=0.48-0.8, p&lt;0.001). Hepatic fibrosis in Fontan patients in our study is universally present, appears to be time dependent, and correlates with few laboratory measurements of liver function. Careful assessment of needle liver biopsies lends a more meaningful measure of liver fibrosis in the Fontan patient than clinical and laboratory data, allowing for appropriate changes to patient management.</p>

10.1016/j.humpath.2016.07.006

Hum. Pathol.

27476041