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Decrease in calcitonin-containing cells in truncus arteriosus
C Gamallo1, M García, J Palacios
1Department of Pathology, La Paz Hospital, Madrid, Spain.
Abstract:
Experimental studies in chick embryos have demonstrated that truncus arteriosus (TA), a form of conotruncal cardiac defect, is due to abnormalities in the cranial neural crest. However, no data are available to support this hypothesis in humans with isolated TA. In the present study, the assessment of calcitonin immunoreactive cells (C-cells) has been employed to evaluate whether or not the proportion of thyroid cells derived from the cranial neural crest is normal in patients with isolated TA. Thyroid sections from 15 such patients in which no other extracardiac malformations were neither clinically nor pathologically found, and from 11 control age-matched patients were studied immunohistochemically at autopsy in order to determine the number and distribution of calcitonin-containing cells. The volume density of C-cells (0.888%) and the number of C-cells per follicle (0.991) was significantly lower in patients with TA than in control patients (3.475%, and 2.367, respectively). The decrease of neural crest-derived cells in the thyroid of patients with "isolated" TA documents more extent abnormalities than clinically suspected and supports the hypothesis of neural crest disturbance as the pathogenetic factor responsible for this heart malformation.
Insights
Neural crest abnormalities are implicated in truncus arteriosus (TA), a heart defect. This study found fewer neural crest-derived cells in the thyroids of infants with isolated TA, supporting the neural crest disturbance hypothesis.
Area of Science:
- Developmental Biology
- Cardiology
- Endocrinology
Background:
- Truncus arteriosus (TA) is a conotruncal cardiac defect.
- Experimental studies suggest cranial neural crest abnormalities cause TA.
- Human data for isolated TA and neural crest involvement are lacking.
Purpose of the Study:
- To investigate the role of cranial neural crest in isolated TA in humans.
- To assess calcitonin-immunoreactive cells (C-cells) as a marker for neural crest-derived cells in the thyroid.
- To determine if the proportion of thyroid C-cells is altered in patients with isolated TA.
Main Methods:
- Immunohistochemical analysis of thyroid sections from 15 patients with isolated TA and 11 age-matched controls.
- Quantification of calcitonin-containing cells (C-cells) and their volume density.
- Autopsy-based study to evaluate C-cell number and distribution.
Main Results:
- Significantly lower volume density of C-cells in TA patients (0.888%) compared to controls (3.475%).
- Significantly fewer C-cells per follicle in TA patients (0.991) than in controls (2.367).
- These findings indicate a reduction in neural crest-derived cells in the thyroid of infants with isolated TA.
Conclusions:
- The reduced proportion of neural crest-derived C-cells in the thyroid supports a neural crest origin for isolated TA.
- This suggests more widespread abnormalities than clinically apparent in isolated TA.
- Neural crest cell disturbance is a likely pathogenetic factor in truncus arteriosus.