RESUMO
OBJECTIVE: To analyze the long-term outcomes in pediatric liver transplant recipients after they have transferred to an adult provider and assess for racial disparities in health outcomes. STUDY DESIGN: This is a single-center, retrospective review of pediatric patients who underwent liver transplantation between July 1990 and August 2015 at a tertiary healthcare system with a large transplant center. Patient mortality and retransplantation were assessed after transfer to adult care. RESULTS: There were 120 patients who were transferred, of whom 19 did not meet the inclusion criteria. Of the remaining 101 patients, 64 (63%) transferred care to a nearby affiliated tertiary adult facility, 29 (29%) were followed by other healthcare systems, and 8 (8%) were lost to follow-up. Of the patients followed at our affiliated adult center, 18 of the 64 (28%) died. Of those 18 deaths, 4 (22%) occurred within the first 2 years after transfer, and 10 (55%) within 5 years of transfer. Four patients were retransplanted by an adult provider, of whom 2 eventually received a third transplant. African Americans had higher rates of death after transfer than patients of other races (44% mortality vs 16%, representing 67% of all cases of death; P = .032), with nearly 50% mortality at 20 years from time of transplantation. CONCLUSIONS: Death is common in pediatric liver transplant recipients after transfer to adult care, with African Americans having disproportionately higher mortality. This period of transition of care is a vulnerable time, and measures must be taken to ensure the safe transfer of young adults with chronic health care needs.
Assuntos
Negro ou Afro-Americano , Hepatopatias/mortalidade , Transplante de Fígado , Transição para Assistência do Adulto , Transplantados , Adolescente , Adulto , Criança , Estudos de Coortes , Feminino , Rejeição de Enxerto/epidemiologia , Rejeição de Enxerto/cirurgia , Humanos , Hepatopatias/cirurgia , Masculino , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Estados Unidos/epidemiologia , Adulto JovemRESUMO
OBJECTIVE: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool. STUDY DESIGN: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage. Test scores were translated to age- and sex-dependent z scores, generating a maximum frailty score of 10. RESULTS: The median frailty score of the cohort was 4 (IQR 3, 5). Subjects with ESLD had significantly higher frailty scores (median 5; IQR 4, 7) than subjects with CCLD (median 3; IQR 2, 4); (P < .0001). Area under the curve receiver operating characteristic for frailty scores to discriminate between ESLD and CCLD was 0.83 (95% CI 0.73, 0.93). Forty-six percent of children with ESLD were frail and there was no correlation between pediatric frailty scores and physician's global assessments (r = -0.24, 95% CI -0.53, 0.10). CONCLUSIONS: A novel frailty tool assessed additional dimensions of health, not captured by standard laboratory measures and identified the sickest individuals among a cohort of children with chronic liver disease. This tool may have applicability to other children with chronic disease.
Assuntos
Fragilidade/diagnóstico , Hepatopatias/complicações , Adolescente , Composição Corporal , Criança , Pré-Escolar , Doença Crônica , Estudos Transversais , Feminino , Fragilidade/etiologia , Marcha , Força da Mão , Humanos , Hepatopatias/fisiopatologia , Masculino , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
OBJECTIVE: To determine whether abdominal magnetic resonance imaging (MRI) detects hepatic abnormalities before clinical or biochemical perturbations in patients after the Fontan procedure. STUDY DESIGN: Thirty-nine children and adolescents who underwent the Fontan procedure and were referred to a pediatric hepatologist by cardiology services between 2011 and 2012 were reviewed retrospectively. Physical examination findings, routine laboratory tests of liver function, evaluation for chronic liver disease, and abdominal MRI findings were recorded. MRI findings were evaluated relative to time elapsed since surgery by 2 radiologists (blinded). RESULTS: Assessment for coexisting chronic liver disease was negative in all patients. All patients had a normal serum albumin level and International Normalized Ratio. Twenty-six of the 39 patients (67%) underwent abdominal MRI, 4 had MRI-incompatible hardware, and 9 did not undergo MRI because of insurance denial. All MRI scans demonstrated morphologic liver changes with varying degrees of reticular contrast enhancement compatible with fibrosis and congestion. Reticular contrast enhancement was often nonuniform, and 9 patients (35%) had multifocal arterially enhancing lesions. CONCLUSION: MRI can identify hepatic abnormalities in patients after Fontan surgery that go undetected by standard clinical and laboratory assessments. These abnormalities are not uniformly distributed throughout the liver, and thus assessment by liver biopsy analysis is subject to sampling error.