Thyroid C cells in the DiGeorge anomaly: a quantitative study.
The developmental field defect in the DiGeorge anomaly (DGA) principally affects derivatives of the third and fourth branchial pouches (areas containing a large population of cephalic neural crest cells), including the ultimobranchial body (UB), the reputed source of thyroid calcitonin-producing cells (C cells) in humans. To evaluate the content of C cells in children with DGA, sections of the thyroid in 16 cases of DGA (group I) (8 incomplete and 8 complete forms) and 16 age-matched controls (group II) were stained by immunoperoxidase for calcitonin and chromogranin. Eleven of 16 (69%) cases in group I [4 of 8 (50%) of the complete and 7 of 8 (88%) of of the incomplete cases] and 14 of 16 (88%) of group II exhibited positive-staining cells for both markers, either individually or in small clusters within the follicular epithelial basement membrane. The average number of C cells per high-power field (HPF) (400x) for group I was 1.6 +/- 0.9 and for group II 4.9 +/- 1.7 (P < .005). Although the percentage of positive incomplete DGA cases was the same as that of the control cases, the average number of C cells/HPF was 2.0 +/- 1.1 and similar to that of complete DGA cases (1.2 +/- 0.6). These results demonstrated that C cells are present in the thyroid of patients with DGA more frequently than expected, although in deficient numbers when compared quantitatively to age-matched controls showing a normal infantile pattern of thyroid C cell distribution. Although this observation confirms that there is deficiency of thyroid C cell development in DGA and is in keeping with the assumption that the cells are of neural crest origin, our data raise the possibility of an additional source of C cells, perhaps from thyroid endoderm, in a manner analogous to the endocrine cells of the gut and respiratory tract.
Pueblitz
S
S
Department of Pathology, Children's Medical Center of Dallas, Texas.
Weinberg
A G
AG
Albores-Saavedra
J
J
D002116:Calcitonin / Q000032:analysis*; D002452:Cell Count; D002864:Chromogranins / Q000032:analysis; D004062:DiGeorge Syndrome / Q000150:complications / Q000473:pathology*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D013961:Thyroid Gland / Q000737:chemistry* / Q000473:pathology
D002864:Chromogranins; D002116:Calcitonin