{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,3]],"date-time":"2025-06-03T04:25:06Z","timestamp":1748924706504,"version":"3.38.0"},"reference-count":35,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2002,9,1]],"date-time":"2002-09-01T00:00:00Z","timestamp":1030838400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2002,9]]},"abstract":"<jats:p> Apoptosis, along with cellular proliferation, plays a major role in normal developmental processes and tissue homeostasis. We hypothesized that altered apoptosis-related pathways and\/or reduced cell proliferation might play a role in the thymic hypoplasia or aplasia in Di-George syndrome (DG). We used immunohistochemistry to evaluate the apoptosis-related antigens Fas (CD95), bcl-2, and p53, as well as Mib-1 proliferation index in the thymuses from six patients with DG. The results were compared with those from the thymuses from six patients with non-DG congenital heart disease. All DG patients (age 32 weeks GA to 4 months) had hypoplastic thymuses ranging from microscopic foci to 2.7 g in weight (expected for age, 4.7 \u00b1 3.6 g to 10 \u00b1 6 g). The thymic weights from the patients with non-DG congenital heart disease (age 37 weeks GA to 1 month) ranged from 3 to 5.6 g and were at the lower range of expected weight by age (expected for age, 8.4 \u00b1 5.6 g to 12 \u00b1 7 g). All thymuses showed histologic features of stress-induced involution. In both groups, a \u2265 50% Mib-1 proliferation index was found in the cortical thymocytes, whereas &lt; 5% Mib-1 labeling was seen in the medullary thymocytes; Fas stained medullary epithelial cells (3+) and cortical epithelial cells (1 +); bcl-2 stained medullary thymocytes (3+) and cortical thymocytes (1+); p53 stained less than 1% of nuclei in all sections. No significantly altered Mib-1 proliferation index or expression of Fas, bcl-2, and p53 was observed in the hypoplastic thymuses in DG, compared to these same measures in non-DG. These results suggest that thymic hypoplasia in DG may be mediated by mechanisms other than reduced cellular proliferation and\/or altered Fas, bcl-2, and p53 apoptotic pathways. <\/jats:p>","DOI":"10.1007\/s10024-002-2004-7","type":"journal-article","created":{"date-parts":[[2002,11,20]],"date-time":"2002-11-20T20:29:14Z","timestamp":1037824154000},"page":"465-471","source":"Crossref","is-referenced-by-count":3,"title":["Expression of Apoptosis-related Antigens, Fas, bcl-2, p53, and Mib-1 Proliferation Index in the Hypoplastic Thymus of DiGeorge Syndrome"],"prefix":"10.1177","volume":"5","author":[{"given":"Holly","family":"Zhou","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Utah, School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sherrie L.","family":"Perkins","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah, School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheryl","family":"Tripp","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah, School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jerry W.","family":"Hussong","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah, School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheryl M.","family":"Coffin","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah, School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2002,9,1]]},"reference":[{"key":"bibr1-s10024-002-2004-7","unstructured":"Online Mendelian Inheritance in Man, OMIM (TM). 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