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Page 1: Congenital Anomalies of the Female Reproductive Tract in a Patient with Goltz Syndrome: First Case Report

exclusion. Since obesity is also linked to oligomenor-rhea and altered sensitivity to insulin, body compo-sition was measured by total body dual x-rayabsorptiometry and trunk fat mass recorded as a surro-gate measure for central adiposity. Fitness levels wereevaluated by measuring oxygen consumption duringa treadmill exercise test. Serum LH, FSH, testosterone,progesterone, and estradiol were obtained. Progester-one levels were consistent with the follicular phasein all participants. Statistical analysis was performedusing the SAS 9.1 software.

Results: No significant difference in BMI, bonedensity, and fitness were found when the two groupswere compared, but trunk fat mass was higher in cy-clic girls. Cardiovascular fitness in both groups waswithin the normal range for age. Testosterone levelswere significantly higher in participants with irregularmenstrual cycles.

Conclusion: Girls with oligomenorrhea were leanerthan their cyclic counterparts. Despite the fact thatandrogenicity increases with obesity during adoles-cence, lean active girls with oligomenorrhea had par-adoxically higher testosterone levels. These elevatedandrogen levels may be linked to ovarian dysfunctionand altered sensitivity to insulin that masquerade asexercise-induced oligomenorrhea.

doi:10.1016/j.jpag.2008.01.029

The Timing of HPVVaccination: Do ACIPRecommendations Apply toAll Populations?

Tricia Huguelet, MD, Stephen Scott, MD,and Jeanelle Sheeder, MSPHDept. of Obstetrics and Gynecology, University of ColoradoHealth Sciences Center, Denver CO

Background: The Gardasil vaccine was approvedby the FDA in 2001 for women ages 9e26, andsupported by the ACIP for use in women ages11e12, with catch-up administration for women ages13e26. These recommendations were based partiallyon results from vaccine trials conducted by Merck,which demonstrated that 73% of women were nega-tive for all 4 HPV types (6, 11, 16, and 18) upon ad-mission. Among the 27% positive, the majority wereonly positive for one HPV type.

The objective of this study was to compare demo-graphic and social characteristics between the

Regular menses Irregular menses P value

BMI 24.3 21.3 0.19Gynecological Age 5.8 5.5 0.74Age 18.3 18.7 0.40BMD 1.17 1.16 0.70% Body Fat 33.6 26.1 0.12Trunk Fat Mass 10244 6430.5 0.039*VO2 Max 37.8 41.2 0.32Estradiol 38.0 37.2 0.95Testosterone 0.32 0.65 0.0018**Progesterone 1.1 0.9 0.65

*!0.05; **!0.001.

77NASPAG 22nd Annual Clinical Meeting

Congenital Anomalies of theFemale Reproductive Tractin a Patient with GoltzSyndrome: First Case Report

Jhansi Reddy, MD, and Marc R. Laufer, MDDivision of Gynecology, Children’s Hospital Boston; Harvard

Medical School, Boston, Massachusetts

Background: Goltz Syndrome or focal dermal hypo-plasia is a rare congenital mesoectodermal syndrome. It

is primarily characterized by extensive cutaneous andmusculoskeletal defects. Although the exact causehas yet to be fully elucidated, its predominance inwomen suggests a dominant X-linked inheritance.

Case: We report a 17-year-old menarchal girl diag-nosed with a severe form of Goltz Syndrome who wasreferred to the Division of Gynecology at the Children’sHospital Boston for the evaluation and management ofpossible vaginal stenosis. A complete evaluation re-vealed a septate hymen, a normal vagina with a singlecervix, a bicornuate uterus and a unilateral kidney. Toour knowledge, there have been no documented casesof congenital anomalies of the female reproductivetract in patients with Goltz Syndrome.

Comments: The normal development of the femalereproductive tract is the result of a complex series ofintegrated steps involving all three embryonic celllayers. Structural anomalies become clinically ap-parent at varying times, making the diagnosis andtreatment challenging. Given the improvement indiagnostic imaging, advances in surgical techniquesand rapidly growing field of reproductive medicine,accurate and early diagnosis may have a profound im-pact on the future fertility of these patients. Femalepatients with multiple abnormalities of ectodermaland mesodermal origin warrant a complete evaluationof their reproductive tracts.

doi:10.1016/j.jpag.2008.01.030

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