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A Case of Prader-Willi Syndrome With Long-term Mazindol Treatment

Akio Inui, MD, PhD; Masaharu Uemoto, MD, PhD; Shizuo Takamiya, MD; Yuhei Shibuya, MD, PhD; Shigeaki Baba, MD, PhD; Masato Kasuga, MD, PhD
Arch Intern Med. 1997;157(4):464. doi:10.1001/archinte.1997.00440250124020.
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Prader-Willi syndrome is a disorder characterized by obesity, hypogonadism, hypotonia, and mental retardation, which is genetically caused by a deletion of a small part of chromosome 15 in some, but not all, patients.1-4 Obesity is associated with diabetes mellitus of the maturity-onset type. The hyperphagia associated with Prader-Willi syndrome is severe and chronic and is responsible for the early mortality of people with the condition. The hyperphagia, together with the behavioral difficulties, makes Prader-Willi syndrome one of the most serious and pervasive conditions with respect to its impact on the family unit.

Report of a Case.  We treated a male patient with Prader-Willi syndrome and overt diabetes mellitus for which he took insulin; we found that the addition of a small dose of mazindol to the insulin therapy was effective in preventing hyperglycemic coma. At the time of the initial mazindol administration, the patient was 23 years old and


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