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Updated: Nov 5, 2025
Isolation and Transplantation of Different Aged Murine Thymic Grafts.
Published on: May 13, 2015
Care of Children with DiGeorge Before and After Cultured Thymus Tissue Implantation
Stephanie E Gupton1, Elizabeth A McCarthy2, M Louise Markert2,3
1Department of Pediatrics, Division of Allergy and Immunology, Duke University Medical Center, Durham, NC, USA. stephanie.gupton@duke.edu.
Insights
Caring for children with complete DiGeorge anomaly (cDGA) requires a multidisciplinary team approach. This strategy optimizes patient condition before and after cultured thymus tissue implantation (CTTI) for improved outcomes.
Area of Science:
- Pediatric immunology
- Developmental biology
- Genetics
Background:
- Complete DiGeorge anomaly (cDGA) presents as congenital athymia with diverse clinical conditions, including 22q11.2DS and CHARGE syndrome.
- Over 100 children with congenital athymia have undergone cultured thymus tissue implantation (CTTI) since 1993.
- Patients often have significant comorbidities like heart disease, hypoparathyroidism, and recurrent infections.
Purpose of the Study:
- To provide guidance for multidisciplinary teams caring for children with cDGA.
- To outline best practices for patient management before and after CTTI.
Main Methods:
- A multidisciplinary team of 31 specialists shared their clinical experience.
- Focus on care strategies at Duke University Health System.
Main Results:
- A multidisciplinary approach aims to optimize children's condition for CTTI and post-implantation care.
- Protection of cultured thymus tissue (CTT) from high-dose steroids is crucial.
- Minimizing adverse effects of immunosuppression on organs is essential.
Conclusions:
- Establishing a multidisciplinary team is vital for optimal outcomes in children with cDGA.
- A detailed care plan is important for managing complex pediatric cases.
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.
