RT Journal Article
SR Electronic
T1 Agammaglobulinemia and Thymic Dysplasia Associated with Ectodermal Dysplasia
JF Pediatrics
JO Pediatrics
FD American Academy of Pediatrics
SP 143
OP 147
VO 47
IS 1
A1 Feigin, Ralph D.
A1 Middelkamp, J. Neal
A1 Kissane, John M.
A1 Warren, Richard J.
YR 1971
UL http://pediatrics.aappublications.org/content/47/1/143.abstract
AB A 5-month-old female with agammaglobulinemia, thymic dysplasia, ectodermal dysplasia, and an erythematous icthyosiform dermatitis has been presented. Death at 7 months of age was the result of pneumocystis carinii pneumonia. The association of a broadly based immunologic deficit with ectodermal defects noted at birth lends support to the concept that a basic genetic abnormality may disturb the development of a number of mesenchymal components. It also suggests that skeletal anomalies are not invariably associated with the immunologic and ectodermal defects.