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April 1987

Fetal AIDS Syndrome Score: Correlation Between Severity of Dysmorphism and Age at Diagnosis of Immunodeficiency

Author Affiliations

From the Divisions of Immunology (Drs Wiznia and Rubinstein) and Genetics (Drs Marion and Hutcheon), the Department of Pediatrics, and the Departments of Microbiology and Immunology (Dr Rubinstein), Albert Einstein College of Medicine, Bronx, NY.

Am J Dis Child. 1987;141(4):429-431. doi:10.1001/archpedi.1987.04460040087022

• To objectively evaluate the fetal acquired immunodeficiency syndrome, we have developed a scoring system based on the presence of the characteristic features that we have previously reported. Using this scoring system, 37 children seropositive for the human immunodeficiency virus were classified into three groups: (1) dysmorphologically severely affected (12 children); (2) moderately affected (15 children); and (3) mildly affected (ten children). There was a statistically significant correlation between the severity of the dysmorphic features and both the presence of opportunistic infections within the first year of life and the age at onset of symptoms associated with immune dysfunction, with the more severely stigmatized children manifesting symptoms at a younger age. There was no correlation, however, between severity of the dysmorphic features and presence of opportunistic infections at the time of our examination. We conclude that this scoring system may be useful in presymptomatic identification of severely dysmorphic human immunodeficiency virus—infected infants.

(AJDC 1987;141:429-431)

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