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1.
Pediatr Nephrol ; 37(1): 153-161, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-34292379

RESUMO

BACKGROUND: We propose a novel clinically significant finding, de novo lupus-like glomerulonephritis (DNLLGN), in patients with autoantibodies and kidney abnormalities in pediatric liver transplant (LT) and intestinal inclusive transplants (ITx). METHODS: We describe the clinical, serologic, and histopathologic presentation and kidney outcomes in eight patients from our center found to have DNLLGN on kidney biopsy. RESULTS: Pediatric recipients of non-kidney solid organ transplants developed an unusual de novo immune complex glomerulonephritis with morphologic similarity to lupus nephritis. Six had isolated LT (0.9% of all pediatric LT at our center) and two had ITx (2.1% of all ITx). Five (63%) presented with nephrotic syndrome. Five patients had autoantibodies. Patients underwent kidney biopsy at a mean of 11.5 years in LT and 2.8 years in ITx after the index transplant. Biopsies demonstrated changes similar to focal or diffuse active lupus. Follow-up eGFR at a mean of 6 years after biopsy showed a mean decrease of 30 ml/min/1.73 m2 in all patients (p = 0.11). CONCLUSIONS: DNLLGN has not been previously recognized in this clinical setting, yet 8 kidney biopsies from pediatric recipients of LT and ITx at our center in 25 years demonstrated this finding. DNLLGN appears to be an under-reported phenomenon of clinical significance. A higher resolution version of the Graphical abstract is available as Supplementary information.


Assuntos
Glomerulonefrite , Nefrite Lúpica , Transplante de Órgãos , Autoanticorpos/análise , Criança , Glomerulonefrite/imunologia , Humanos , Transplante de Fígado/efeitos adversos , Nefrite Lúpica/imunologia , Transplante de Órgãos/efeitos adversos
2.
Prog Transplant ; 32(1): 41-48, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-34894854

RESUMO

INTRODUCTION: Timely access to kidney transplant is essential to reducing mortality of children with kidney disease. We examined factors affecting providers' decisions to delay waitlisting, compared perceptions of important factors of providers to families, when delaying activation, and describe recommendations to improve support for pediatric patients and families to overcome waitlisting delays. METHODS: Using a mixed-methods design, 20 providers and 20 families of pediatric patients with kidney disease, participated in interviews and surveys. Interviews were analyzed using thematic analysis. Surveys were analyzed with descriptive statistics. RESULTS: Avoiding retransplantation, treatment nonadherence, poor psychological readiness for transplant, poor physical health, and greater need for social support were the key themes affecting providers' decisions to delay waitlisting. At least 70% of both providers and families felt that waitlisting should be delayed until patients and families had reliable access to transportation, mental health support, and caregivers who can better understand medical information. At least 70% of families surveyed felt it was important to delay waitlisting until they had regulated blood pressure and well-managed labs. Ethical concerns emerge that waitlisting practices may contribute to disparities in access to transplant. CONCLUSION: Providers and families agree that stabilizing the family situation and improving adherence to treatment are important reasons to delay waitlisting. However, pediatric patients facing greater disparities need easier access to psychological services, strengthened social support, access to economic resources, and stronger relationships with coordinators. Addressing patient burdens is essential for reaching more equitable listing practices.


Assuntos
Transplante de Rim , Criança , Humanos , Apoio Social , Inquéritos e Questionários , Listas de Espera
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