Cargando…
Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation
BACKGROUND: Children with complete DiGeorge anomaly (cDGA) have congenital athymia plus a myriad of other challenging clinical conditions. The term cDGA encompasses children with congenital athymia secondary to 22q11.2DS, CHARGE syndrome (coloboma, heart defects, choanal atresia, growth or mental re...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8249267/ https://www.ncbi.nlm.nih.gov/pubmed/34003433 http://dx.doi.org/10.1007/s10875-021-01044-0 |
_version_ | 1783716878336655360 |
---|---|
author | Gupton, Stephanie E. McCarthy, Elizabeth A. Markert, M. Louise |
author_facet | Gupton, Stephanie E. McCarthy, Elizabeth A. Markert, M. Louise |
author_sort | Gupton, Stephanie E. |
collection | PubMed |
description | BACKGROUND: Children with complete DiGeorge anomaly (cDGA) have congenital athymia plus a myriad of other challenging clinical conditions. The term cDGA encompasses children with congenital athymia secondary to 22q11.2DS, CHARGE syndrome (coloboma, heart defects, choanal atresia, growth or mental retardation, genital abnormalities, and ear abnormalities and/or deafness), and other genetic abnormalities. Some children have no known genetic defects. Since 1993, more than 100 children with congenital athymia have been treated with cultured thymus tissue implantation (CTTI). Naïve T cells develop approximately 6 to 12 months after CTTI. Most of the children had significant comorbidities such as heart disease, hypoparathyroidism, and infections requiring complex clinical care post cultured thymus tissue implantation (CTTI). OBJECTIVE: The purpose of this guidance is to assist multidisciplinary teams in caring for children with cDGA both before and after CTTI. METHODS: Thirty-one specialists, in addition to the authors, were asked to share their experience in caring for children with cDGA at Duke University Health System, before and after CTTI. These specialists included physicians, nurses, dentists, therapists, and dieticians. RESULTS: The goal of a multidisciplinary approach is to have children in the best possible condition for receiving CTTI and provide optimal care post CTTI through development of naïve T cells and beyond. The CTT (cultured thymus tissue) must be protected from high doses of steroids which can damage CTT. Organs must be protected from adverse effects of immunosuppression. CONCLUSION: Creating a multidisciplinary team and a detailed plan of care for children with cDGA is important for optimal outcomes. |
format | Online Article Text |
id | pubmed-8249267 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Springer US |
record_format | MEDLINE/PubMed |
spelling | pubmed-82492672021-07-20 Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation Gupton, Stephanie E. McCarthy, Elizabeth A. Markert, M. Louise J Clin Immunol How I Manage BACKGROUND: Children with complete DiGeorge anomaly (cDGA) have congenital athymia plus a myriad of other challenging clinical conditions. The term cDGA encompasses children with congenital athymia secondary to 22q11.2DS, CHARGE syndrome (coloboma, heart defects, choanal atresia, growth or mental retardation, genital abnormalities, and ear abnormalities and/or deafness), and other genetic abnormalities. Some children have no known genetic defects. Since 1993, more than 100 children with congenital athymia have been treated with cultured thymus tissue implantation (CTTI). Naïve T cells develop approximately 6 to 12 months after CTTI. Most of the children had significant comorbidities such as heart disease, hypoparathyroidism, and infections requiring complex clinical care post cultured thymus tissue implantation (CTTI). OBJECTIVE: The purpose of this guidance is to assist multidisciplinary teams in caring for children with cDGA both before and after CTTI. METHODS: Thirty-one specialists, in addition to the authors, were asked to share their experience in caring for children with cDGA at Duke University Health System, before and after CTTI. These specialists included physicians, nurses, dentists, therapists, and dieticians. RESULTS: The goal of a multidisciplinary approach is to have children in the best possible condition for receiving CTTI and provide optimal care post CTTI through development of naïve T cells and beyond. The CTT (cultured thymus tissue) must be protected from high doses of steroids which can damage CTT. Organs must be protected from adverse effects of immunosuppression. CONCLUSION: Creating a multidisciplinary team and a detailed plan of care for children with cDGA is important for optimal outcomes. Springer US 2021-05-18 2021 /pmc/articles/PMC8249267/ /pubmed/34003433 http://dx.doi.org/10.1007/s10875-021-01044-0 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | How I Manage Gupton, Stephanie E. McCarthy, Elizabeth A. Markert, M. Louise Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title | Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title_full | Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title_fullStr | Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title_full_unstemmed | Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title_short | Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation |
title_sort | care of children with digeorge before and after cultured thymus tissue implantation |
topic | How I Manage |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8249267/ https://www.ncbi.nlm.nih.gov/pubmed/34003433 http://dx.doi.org/10.1007/s10875-021-01044-0 |
work_keys_str_mv | AT guptonstephaniee careofchildrenwithdigeorgebeforeandafterculturedthymustissueimplantation AT mccarthyelizabetha careofchildrenwithdigeorgebeforeandafterculturedthymustissueimplantation AT markertmlouise careofchildrenwithdigeorgebeforeandafterculturedthymustissueimplantation |