1987 Volume 25 Issue 1 Pages 174-183
We experienced a very rare case of constitutional erythroid hypoplasia (Josephs-Blackfan-Diamond type) with immunodeficiency. The patient was a girl,8 years old at the time of the first visit. She had been given adrenocortical hormones since she was 3 months of age. Her physical and mental growth was very noticeably retarded for her age.
A blood analysis revealed that she had already recovered from the anemia (hemoglobin 13.6g/dl, hematocrit 41%), but measurable amount of the immunoglobulins could not be found in every class. In an analysis of the surface antigens of the lymphocytes using the monoclonal antibody, the ratios were as follows.: OKT3 90%, OKT4 35.4%, and OKT8 52.2%.
The chief complaint was a spontaneous pain of the right lower first molar. An oral examination revealed seven deep cavities. Oral hygiene was poor, but her gingiva and oral mucosa were not so unhealthy.
Because of lack of cooperation concerning the dental treatment procedures on the part of the patient, due to her mental retardation, we performed intensive dental treatment under general anesthesia.
Since then, her dental health has been satisfactorily maintained under the periodical dental check-up system of our hospital.
At 9 years 8 months of age (1 year and 10 months after the first visit), a lateral roentgenographic cephalogram analysis was performed. The denture pattern was almost within normal limits, but hypoplasia of the maxilla and the mandible was recognized. A radiograph of the wrist revealed a remarkable retardation of the bone maturational stage. A model analysis showed significantly small values on the upper and lower dental arch width.
These growth retardations seemed due to the side effect of the long term use of the adrenocortical hormones.
