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December 1976, Vol 130, No. 12 >
Article | December 1976

Partial DiGeorge Syndrome or Branchial Dysembryogenesis?-Reply FREE

HENRY F. PABST, MD; E. RICHARD STIEHM, MD
Am J Dis Child. 1976;130(12):1377. doi:10.1001/archpedi.1976.02120130082024.
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In Reply.—The point made by Miller underlines our plea for early immunologic assessment and periodic monitoring of the immune response in infants suspected of or diagnosed as having the anomalad of branchial dysembryogenesis. The simplest and quickest screening test to be applied is E-rosetting of the patient's peripheral lymphocytes with sheep red blood cells,1.2 a test not available when we studied our two patients. Reconstitution with fetal thymus is still the preferred mode of therapy if T-cell function is found to be diminished. However, gestational age3 of the thymic graft is critical to minimize graft-vs-host reactions.3 Thus, full immunologic evaluation is essential before reconstitution is considered4 in babies with T-cell deficiency in whom even transfusions have been known to precipitate severe graft-vs-host disease.5

REFERENCES

1 +
Wybran J, Carr MC, Fudenberg HH:  The human rosette-forming cell as a marker of a population of thymus-derived cells . J Clin Invest 51:2537-2543, 1972;.
2 +
Jonsson V:  Technical aspects of the rosette technique for detecting human circulating B and T lymphocytes: Normal values and some remarks on null lymphocytes . Scand JHematol 13:361-369, 1974;.
3 +
Ammann AJ, Wara EW, Doyle NE, et al:  Thymus transplantation in patients with thymic hypoplasia and abnormal immunoglobulin synthesis . Transplantation 20:457-466, 1975;.
4 +
Pabst H, Chung GSH, Segal D, et al:  Mild combined immunodeficiency in identical twins . J Pediatr , to be published.
5 +
Hong R, Gatti RA, Good RA:  Hazards and potential benefits of blood transfusion in immunological deficiency . Lancet 2:388-389, 1968;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Wybran J, Carr MC, Fudenberg HH:  The human rosette-forming cell as a marker of a population of thymus-derived cells . J Clin Invest 51:2537-2543, 1972;.
2 +
Jonsson V:  Technical aspects of the rosette technique for detecting human circulating B and T lymphocytes: Normal values and some remarks on null lymphocytes . Scand JHematol 13:361-369, 1974;.
3 +
Ammann AJ, Wara EW, Doyle NE, et al:  Thymus transplantation in patients with thymic hypoplasia and abnormal immunoglobulin synthesis . Transplantation 20:457-466, 1975;.
4 +
Pabst H, Chung GSH, Segal D, et al:  Mild combined immunodeficiency in identical twins . J Pediatr , to be published.
5 +
Hong R, Gatti RA, Good RA:  Hazards and potential benefits of blood transfusion in immunological deficiency . Lancet 2:388-389, 1968;.

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