Scholars@Duke
Blythe Hazlehurst Devlin

Blythe Hazlehurst Devlin

Associate Professor Emeritus
Pediatrics, Allergy and Immunology
devli002@mc.duke.edu
Box 3352, Durham, NC 27710
2200 W. Main Street, Wing B, Suite 220, Box 104013, Durham, NC 27705

Scholarly Works - Journal articles

Thymus transplantation for complete DiGeorge syndrome: European experience.

Journal article J Allergy Clin Immunol · December 2017 BACKGROUND: Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). METHODS: Twelve patients with cDGS underwent transplantation with allogeneic cultured thymus. OBJECTIVE: We sought to confirm and ext ... Full text Link to item Cite

Quantification of total T-cell receptor diversity by flow cytometry and spectratyping.

Journal article BMC Immunol · August 6, 2013 BACKGROUND: T-cell receptor diversity correlates with immune competency and is of particular interest in patients undergoing immune reconstitution. Spectratyping generates data about T-cell receptor CDR3 length distribution for each BV gene but is technica ... Full text Link to item Cite

Diagnosis of 22q11.2 deletion syndrome and artemis deficiency in two children with T-B-NK+ immunodeficiency.

Journal article J Clin Immunol · October 2012 Two infants are described who presented with 22q11.2 deletion and a T(-)B(-)NK(+) immune phenotype. For both infants, the initial diagnosis was athymia secondary to complete DiGeorge anomaly. The first infant underwent thymus transplantation but 6 months a ... Full text Link to item Cite

Human FOXN1-deficiency is associated with αβ double-negative and FoxP3+ T-cell expansions that are distinctly modulated upon thymic transplantation.

Journal article PLoS One · 2012 Forkhead box N1 (FOXN1) is a transcription factor crucial for thymic epithelium development and prevention of its involution. Investigation of a patient with a rare homozygous FOXN1 mutation (R255X), leading to alopecia universalis and thymus aplasia, unex ... Full text Link to item Cite

Thymic microenvironment reconstitution after postnatal human thymus transplantation.

Journal article Clin Immunol · September 2011 A functional thymus develops after cultured thymus tissue is transplanted into subjects with complete DiGeorge anomaly. To gain insight into how the process occurs, 7 post-transplantation thymus biopsy tissues were evaluated. In 5 of 7 biopsies, the thymus ... Full text Link to item Cite

First use of thymus transplantation therapy for FOXN1 deficiency (nude/SCID): a report of 2 cases.

Journal article Blood · January 13, 2011 FOXN1 deficiency is a primary immunodeficiency characterized by athymia, alopecia totalis, and nail dystrophy. Two infants with FOXN1 deficiency were transplanted with cultured postnatal thymus tissue. Subject 1 presented with disseminated Bacillus Calmett ... Full text Link to item Cite

Mechanisms of tolerance to parental parathyroid tissue when combined with human allogeneic thymus transplantation.

Journal article J Allergy Clin Immunol · October 2010 BACKGROUND: The induction of tolerance toward third-party solid organ grafts with allogeneic thymus tissue transplantation has not been previously demonstrated in human subjects. OBJECTIVE: Infants with complete DiGeorge anomaly (having neither thymus nor ... Full text Link to item Cite

Thymus transplantation.

Journal article Clin Immunol · May 2010 Thymus transplantation is a promising investigational therapy for infants born with no thymus. Because of the athymia, these infants lack T cell development and have a severe primary immunodeficiency. Although thymic hypoplasia or aplasia is characteristic ... Full text Link to item Cite

The dynamics of T-cell receptor repertoire diversity following thymus transplantation for DiGeorge anomaly.

Journal article PLoS Comput Biol · June 2009 Featured Publication T cell populations are regulated both by signals specific to the T-cell receptor (TCR) and by signals and resources, such as cytokines and space, that act independently of TCR specificity. Although it has been demonstrated that disruption of either of thes ... Full text Link to item Cite

Thymus transplantation in complete DiGeorge anomaly.

Journal article Immunol Res · 2009 Featured Publication Complete DiGeorge anomaly is characterized by athymia, congenital heart disease, and hypoparathyroidism. This congenital disease is fatal by age 2 years unless immune reconstitution is successful. There are multiple underlying syndromes associated with com ... Full text Link to item Cite

Characterization of cultured thymus tissue used for transplantation with emphasis on promiscuous expression of thyroid tissue-specific genes.

Journal article Immunol Res · 2009 Featured Publication Autoimmune thyroid disease occurs in some complete DiGeorge anomaly patients after thymus transplantation. This study was designed to assess the effect of culture of thymus tissue on the expression of genes involved in the development of autoimmunity. The ... Full text Link to item Cite

Thymus transplantation

Journal article · December 1, 2008 Thymus transplantation was first attempted in the 1960s and 1970s using fetal thymus tissue [1, 2]. The results overall were disappointing [3-6]. In part the poor outcomes related to the lack of reagents needed to characterize and identify the patients int ... Full text Cite

Thymic reconstitution

Journal article · December 1, 2008 Full text Cite

Immunological reconstitution following thymic transplantation of a nude/SCID patient

Journal article CLINICAL AND EXPERIMENTAL IMMUNOLOGY · November 1, 2008 Link to item Cite

Factors affecting success of thymus transplantation for complete DiGeorge anomaly.

Journal article Am J Transplant · August 2008 Featured Publication Thymus transplantation shows promise for the treatment of athymia in complete DiGeorge anomaly. This report reviews the effects of dose of thymus tissue, ABO compatibility, HLA matching, culture conditions, age of donor and immunosuppression of recipient o ... Full text Link to item Cite

Use of allograft biopsies to assess thymopoiesis after thymus transplantation.

Journal article J Immunol · May 1, 2008 Featured Publication Thymus allograft biopsies were performed in athymic infants with complete DiGeorge anomaly after thymus transplantation to assess whether the thymus allograft tissue was able to support thymopoiesis. Forty-four consecutive infants were treated with postnat ... Full text Link to item Cite

Long-term tolerance to allogeneic thymus transplants in complete DiGeorge anomaly.

Journal article Clin Immunol · March 2008 Featured Publication Thymus transplantation in subjects with complete DiGeorge anomaly using postnatal allogeneic HLA-nonmatched cultured thymus tissue provides immunoreconstitution. Tolerance of the newly developed T cells toward the donor thymus has not previously been studi ... Full text Link to item Cite

Human T cell reconstitution in DiGeorge syndrome and HIV-1 infection.

Journal article Semin Immunol · October 2007 Featured Publication The thymus is essential for proper development and maintenance of a broad T cell repertoire capable of recognizing a wide-range of foreign antigens. Recent advances in multicolor flow cytometry, non-invasive imaging techniques, and molecular assessments of ... Full text Link to item Cite

Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants.

Journal article Blood · May 15, 2007 Featured Publication The purpose of this study was to characterize a large group of infants with complete DiGeorge anomaly and to evaluate the ability of thymus transplantation to reconstitute immune function in these infants. DiGeorge anomaly is characterized by varying defec ... Full text Link to item Cite

SpA: web-accessible spectratype analysis: data management, statistical analysis and visualization.

Journal article Bioinformatics · September 15, 2005 Featured Publication SUMMARY: SpA is a web-accessible system for the management, visualization and statistical analysis of T-cell receptor spectratype data. Users upload data from their spectratype analyzers to SpA, which saves the raw data and user-defined supplementary covar ... Full text Link to item Cite

Statistical analysis of antigen receptor spectratype data.

Journal article Bioinformatics · August 15, 2005 Featured Publication MOTIVATION: The effectiveness of vertebrate adaptive immunity depends crucially on the establishment and maintenance of extreme diversity in the antigen receptor repertoire. Spectratype analysis is a method used in clinical and basic immunological settings ... Full text Link to item Cite

Postnatal thymus transplantation with immunosuppression as treatment for DiGeorge syndrome.

Journal article Blood · October 15, 2004 Featured Publication Complete DiGeorge syndrome is a fatal congenital disorder characterized by athymia, hypoparathyroidism, and heart defects. Less than half of patients are 22q11 hemizygous. The goal of this study was to assess if immune suppression followed by postnatal thy ... Full text Link to item Cite

Complete DiGeorge syndrome: development of rash, lymphadenopathy, and oligoclonal T cells in 5 cases.

Journal article J Allergy Clin Immunol · April 2004 Featured Publication BACKGROUND: Five patients with DiGeorge syndrome presented with infections, skin rashes, and lymphadenopathy after the newborn period. T-cell counts and function varied greatly in each patient. Initial laboratory testing did not suggest athymia in these pa ... Full text Link to item Cite

Comparison of PCR, culture, and histopathology for diagnosis of tuberculous pericarditis.

Journal article J Clin Microbiol · December 1997 Nucleic acid amplification techniques for the diagnosis of tuberculosis (TB) are rapidly being developed. Scant work, however, has focused on pericardial TB. Using cryopreserved specimens from a prior study of pericarditis, we compared PCR to culture and h ... Full text Link to item Cite

Myotropic Th1 T helper cell lymphoma presenting as granulomatous inflammatory masses in synovium and muscle

Journal article Journal of Investigative Medicine · January 1, 1996 Human T helper (Th) lymphocyte subsets can be differentiated by cytokine production; Thl cells produce inteferon-gamma and interleukin-2, while Th2 cells produce interleukin-4 and interleukin-5. Imbalance of Th subsets leads to disordered immunity and a my ... Cite

Inclusion of a sensitivity control to add a quality-control parameter and improve reproducibility in BCR, immunoglobulin, and T-cell receptor gene-rearrangement studies.

Journal article Diagn Mol Pathol · December 1994 Molecular analyses to determine clonality of T and B cells in malignant lymphoma and leukemia and to detect the (9;22) translocation in chronic myelogenous leukemia are commonly used in clinical molecular biology laboratories. We describe the inclusion of ... Full text Link to item Cite

A novel beta-globin mutation, beta Durham-NC [beta 114 Leu-->Pro], produces a dominant thalassemia-like phenotype.

Journal article Blood · February 15, 1994 Mutations within exon 3 of the beta-globin gene are relatively uncommon, and many of these mutations produce a dominant thalassemia-like phenotype. We describe a novel thalassemic hemoglobinopathy caused by a single nucleotide substitution (CTG-->CCG) at c ... Link to item Cite

Genotypic divergence precedes clinical dissemination in a case of synchronous bilateral B-cell malignant lymphoma of the testes.

Journal article Hum Pathol · June 1993 Malignant lymphoma of the testis occurs bilaterally more often than any other tumor type. We report the case of a 62-year-old man who presented with synchronous, bilateral, testicular malignant lymphomas without clinical or radiologic evidence of extratest ... Full text Link to item Cite

Functional heterogeneity of mammalian TATA-box sequences revealed by interaction with a cell-specific enhancer.

Journal article Nature · March 15, 1990 A regulatory element upstream of the human myoglobin gene functions as a muscle-specific enhancer (MSE) in conjunction with core promoter elements of the myoglobin gene, but not in combination with the simian virus 40 (SV40) early promoter. These two promo ... Full text Link to item Cite

Identification of a muscle-specific enhancer within the 5'-flanking region of the human myoglobin gene.

Journal article J Biol Chem · August 15, 1989 A 2-kilobase fragment from the 5'-flanking region of the human myoglobin gene extending from -2038 to +7 relative to the cap site regulates expression of a heterologous reporter gene in a cell-specific and developmentally regulated manner. Functional analy ... Link to item Cite

Reentry into the cell cycle of differentiated skeletal myocytes.

Journal article Dev Biol · January 1983 The activation of muscle-specific myosin synthesis and its relationship to withdrawal from the cell cycle have been examined in cycle-synchronized myoblasts under growth-restrictive, fusion-impermissive (low Ca2+) culture conditions. Under these conditions ... Full text Link to item Cite