Progeria (Hutchinson-Gilford Syndrome). Presented by DR. ADOLPH ROSTENBERG and (by invitation) DR. IRA ROSENTHAL.
Medical history: This child was of normal birth weight. Her parents noted poor weight gain by the age of 4 months. Her hands were noted to be abnormal at birth. A diagnosis of progeria (Hutchinson-Gilford syndrome) was made at the age of 4 months. Weight gain and growth have been poor. The child now weighs 10 lb. She is in the hospital at present for treatment of a corneal ulcer. Child is believed to be of low-normal intelligence.
Description: The child shows marked paucity of subcutaneous tissue. There is almost complete alopecia. There are flexion contractures of the hands, knees, and ankles. The ears are deformed, and the ear lobes are missing. The splanchnicranium is poorly developed. Fontanels are still open.
Laboratory data: Routine tests all normal. Slight amino-aciduria.
Treatment: None.
Discussion
Dr. Ira
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