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  Vol. 152 No. 10, October 1998 TABLE OF CONTENTS
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Picture of the Month

Hüseyin Özbey, MD; Nese Özbey, MD; Walter W. Tunnessen, Jr, MD
From the Departments of Pediatric Surgery (Dr H. Özbey) and Endocrinology, Nutrition and Metabolism (Dr N. Özbey), University of Istanbul, Istanbul Medical Faculty, Istanbul, Turkey; and the American Board of Pediatrics, Chapel Hill, NC (Dr Tunnessen).

Arch Pediatr Adolesc Med. 1998;152:1031-1032.

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

THIS 5-MONTH-OLD infant, the product of a consanguineous marriage, now weighs 3500 g. He has a lifelong history of poor growth with high blood glucose levels unresponsive to insulin. Recurrent rectal prolapse has resulted in chronic mechanical intestinal obstruction (Figure 1).


Figure.


Denouement and Discussion: Leprechaunism

Figure 1, left, The infant shows the typical "elfinlike" facies of leprechaunism with hirsutism, large, low-set ears, broad nasal tip and flared nares, and thick lips. Figure 1, right, Other prominent features present in this infant include reduced subcutaneous fat, prominent nipples, a distended abdomen, large external genitalia, and rectal prolapse.

Donohue1 and Donohue and Uchida2 were the first to describe this rare syndrome featuring dysmorphic facies, failure to thrive, hirsutism, and multiple endocrine abnormalities.2 Leprechaunism was the euphemistic name used to describe the "elfinlike" facies and poor growth characteristic of the syndrome. . . . [Full Text of this Article]







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