Selma A. Myhre

Biography

• Born Selma Ann Myhre on October 27, 1937 in Tacoma, Washington, USA
• Graduated medicine from the University of Utah Medical School
• Pediatric residency, University of Washington
• 1970 – Married Paul William Hageman, MD (1939-2008)
• Western State Hospital, Steilacoom
• Rainier School in Buckley, Washington
• 1999 – Retired
• Died November 23, 2012, Puyallup, Washington, USA

Medical Eponyms

Myhre syndrome (1981)

Myhre syndrome (MYHRS) is caused by heterozygous mutation in the SMAD4 gene on chromosome 18q21. Rare sporadic disorder characterized by mental retardation, dysmorphic facial features, including microcephaly, midface hypoplasia, prognathism, and blepharophimosis, typical skeletal anomalies, including short stature, square body shape, broad ribs, iliac hypoplasia, brachydactyly, flattened vertebrae, and thickened calvaria, and cardiovascular defects with a striking fibroproliferative response to surgical intervention. [OMIM #139210]

[aka Laryngotracheal stenosis, arthropathy, prognathism, and short stature; LAPS syndrome; Growth mental deficiency syndrome of Myhre]

The purpose of this report is to describe two unrelated male subjects with similar dysmorphic features, which we believe represent a syndrome not previously recognized. The cardinal clinical features, summarized in Table 1, were mental retardation, growth deficiency, unusual facies, hearing loss, generalized muscular hypertrophy, joint limitations and skeletal findings…Older paternal age has been documented in these two cases.

Myhre 1981

Major Publications

• Myhre SA, Ruvalcaba RH, Johnson HR, Thuline HC, Kelley VC. The effects of testosterone treatment in Klinefelter’s syndrome. J Pediatr. 1970;76(2):267-276.
• Johnson HR, Myhre SA, Ruvalcaba RH, Thuline HC, Kelley VC. Effects of testosterone on body image and behavior in Klinefelter’s syndrome: a pilot study. Dev Med Child Neurol. 1970;12(4):454-460
• Ruvalcaba RH, Churesigaew S, Myhre SA, Kelley VC, Martin GM. Children who age rapidly–progeroid syndromes: case report of a new variant. Clin Pediatr (Phila). 1977;16(3):248-252.
• Ruvalcaba RH, Myhre SA, Roosen-Runge EC, Beckwith JB. X-linked mental deficiency megalotestes syndrome. JAMA. 1977;238(15):1646-1650.
• Myhre SA, Williams R. Teratogenic effects associated with maternal primidone therapy. J Pediatr. 1981;99(1):160-162.
• Myhre SA, Ruvalcaba RH, Graham CB. A new growth deficiency syndrome. Clin Genet. 1981;20(1):1-5. [Myhre syndrome]
• Myhre SA, Ruvalcaba RH, Kelley VC. Congenital deafness and hypogonadism: a new X-linked recessive disorder. Clin Genet. 1982;22(6):299-307.
• Shepard TH, Wener MH, Myhre SA, Hickok DE. Lowered plasma albumin concentration in fetal Turner syndrome. J Pediatr. 1986;108(1):114-116.

References

• Selma Ann Hageman, MD. Legacy.com
• Myhre syndrome; MYHRS. OMIM #139210