Dataset Information

Juvenile erythrocytosis in children after liver transplantation: prevalence, risk factors and outcome.

ABSTRACT: Most reports of post-transplant erythrocytosis have involved kidney recipients and, so far, there have been no large studies of onset of erythrocytosis after orthotopic liver transplantation (OLT) in children. We present a long-term survey of pediatric liver recipients, evaluating prevalence, outcome and the main potential causes of erythrocytosis, including a comprehensive mutational analysis of commonly related genes (mutations of HBB and HBA, JAK2, EPOR, VHL, EPAS1 and EGLN1). Between 2000 and 2015, 90 pediatric OLT recipients were observed for a median period of 8.7 years (range 1-20.4 [IQR 4.9-13.6] years). Five percent of the study population (4 males and 1 female) developed erythrocytosis at 8.5 years post OLT (range 4.1-14.9 [IQR 4.7-14.7]) at a median age of 16.6 years (range 8.2-18.8 [IQR 11.7-17.7]). Erythrocytosis-free survival after OLT was 98.6% at 5 years, 95% at 10 years, and 85% at 15 years, with an incidence rate of 6/1000 person-years. No cardiovascular events or thrombosis were reported. No germinal mutation could be clearly related to the development of erythrocytosis. One patient, with high erythropoietin levels and acquired multiple bilateral renal cysts, developed clinical hyper-viscosity symptoms, and was treated with serial phlebotomies. In conclusion, this prospective longitudinal study showed that erythrocytosis is a rare complication occurring several years after OLT, typically during adolescence. Erythrocytosis was non-progressive and manageable. Its pathogenesis is still not completely understood, although male gender, pubertal age, and renal cysts probably play a role.

SUBMITTER: Casale M

PROVIDER: S-EPMC7298026 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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Juvenile erythrocytosis in children after liver transplantation: prevalence, risk factors and outcome.

Casale Maddalena M Roberti Domenico D Mandato Claudia C Iorio Raffaele R Caropreso Maria M Scianguetta Saverio S Picariello Stefania S Perrotta Silverio S Vajro Pietro P

Scientific reports 20200616 1

Most reports of post-transplant erythrocytosis have involved kidney recipients and, so far, there have been no large studies of onset of erythrocytosis after orthotopic liver transplantation (OLT) in children. We present a long-term survey of pediatric liver recipients, evaluating prevalence, outcome and the main potential causes of erythrocytosis, including a comprehensive mutational analysis of commonly related genes (mutations of HBB and HBA, JAK2, EPOR, VHL, EPAS1 and EGLN1). Between 2000 an ...[more]

PMID: 32546701

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Project description:New-onset systolic heart failure (HF) after liver transplantation (LT) is a significant cause of morbidity and mortality; however, its characteristics are still insufficiently delineated. HF may involve the left ventricle (LV), right ventricle (RV), or both ventricles. We explored the incidence, characteristics, etiologies, risks, involved cardiac chambers, and outcomes of HF after LT.MethodsThis study included 528 adult patients with preoperative LV ejection fraction ≥ 55% who underwent LT between 2016 and 2020. The primary outcome was new-onset systolic HF, defined by the presence of clinical signs, symptoms, and echocardiographic evidence of reduced LVejection fraction <50% and RV dysfunction within the first year after LT.ResultsThirty-one patients (6%) developed systolic HF within a median of 9 d (1-364). Of those, 23% of patients had ischemic HF, whereas 77% had nonischemic HF. Nonischemic HF was caused by stress (11), sepsis (8), or other factors (5). Nonischemic HF was secondary to isolated LV failure in 58% of patients or RV ± LV failure in 42% of patients. Recursive partitioning identified subgroups with varying risks and uncovered interaction between variables. HF risk increased from 4.2% to 13% when epinephrine and/or norepinephrine drips were used intraoperatively (P < 0.01). When no epinephrine and/or norepinephrine were used, HF risk increased from 3.1% to 38.5% if baseline hemoglobin was <7.2 g/dL (P < 0.01). When baseline hemoglobin was ≥7.2 g/dL, HF risk increased from 0% to 5.2% when ≥3500 mL crystalloid was used intraoperatively (P < 0.01). Posttransplant first-year survival and reversibility of HF depended on the etiology (stress, sepsis, ischemia, etc) and cardiac chamber involvement (isolated LV or RV ± LV). RV dysfunction was associated with inferior recovery of cardiac function and poorer survival than nonischemic isolated LV dysfunction (50% versus 70%, respectively).ConclusionsPosttransplant new-onset HF is mostly nonischemic in nature and is associated with increased morbidity and mortality.

Dataset Information

Juvenile erythrocytosis in children after liver transplantation: prevalence, risk factors and outcome.

Publications

Juvenile erythrocytosis in children after liver transplantation: prevalence, risk factors and outcome.

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