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Published in: Journal of Medical Case Reports 1/2021

Open Access 01-12-2021 | Hormonal Replacement Therapy | Case report

Menarche in primary ovarian insufficiency after a month of hormone replacement therapy: a case report

Author: Biwen Cheng

Published in: Journal of Medical Case Reports | Issue 1/2021

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Abstract

Background

Gynecologic anomalies, including uterine agenesis and ovarian dysgenesis, are some of the several differential diagnoses in adolescent females with primary amenorrhea and delayed puberty. Primary ovarian insufficiency is reported in the clinical practice of reproductive endocrinology can be determined by conducting sex hormone tests to evaluate the hypothalamic-pituitary-ovarian axis. However, confirmation of Mullerian agenesis by image modalities can be extremely challenging. Once the diagnosis is established, breakthrough bleeding usually occurs 2 to 3 years after hormonal replacement therapy.

Case presentation

We report a case of a seventeen year old Taiwanese female, 46 XX karyotype, with ovarian dysgenesis and an initial tentative diagnosis of uterine agenesis who experienced a breakthrough bleeding after a month of hormonal replacement therapy.

Conclusions

The breakthrough bleeding after a month of estrogen therapy in primary ovarian insufficiency is uncommon, and the diagnosis of the absent uterus can have an extensive psychological impact on patients and their families.
Literature
1.
go back to reference Nelson LM. Clinical practice. Primary ovarian insufficiency. N Engl J Med. 2009;360:606–14.CrossRef Nelson LM. Clinical practice. Primary ovarian insufficiency. N Engl J Med. 2009;360:606–14.CrossRef
2.
go back to reference Sullivan SD, Sarrel PM, Nelson LM. Hormone replacement therapy in young women with primary ovarian insufficiency and early menopause. Fertil Steril. 2016;106:1588–99.CrossRef Sullivan SD, Sarrel PM, Nelson LM. Hormone replacement therapy in young women with primary ovarian insufficiency and early menopause. Fertil Steril. 2016;106:1588–99.CrossRef
6.
go back to reference Gordon CM, Kanaoka T, Nelson LM. Update on primary ovarian insufficiency in adolescents. Curr Opin Pediatr. 2015;27:511–9.CrossRef Gordon CM, Kanaoka T, Nelson LM. Update on primary ovarian insufficiency in adolescents. Curr Opin Pediatr. 2015;27:511–9.CrossRef
7.
go back to reference Nelson LM, Covington SN, Rebar RW. An update: spontaneous premature ovarian failure is not an early menopause. Fertil Steril. 2005;83:1327–32.CrossRef Nelson LM, Covington SN, Rebar RW. An update: spontaneous premature ovarian failure is not an early menopause. Fertil Steril. 2005;83:1327–32.CrossRef
8.
go back to reference Michala L, Aslam N, Conway GS, Creighton SM. The clandestine uterus: or how the uterus escapes detection prior to puberty. BJOG. 2010;117:212–5.CrossRef Michala L, Aslam N, Conway GS, Creighton SM. The clandestine uterus: or how the uterus escapes detection prior to puberty. BJOG. 2010;117:212–5.CrossRef
9.
go back to reference Salardi S, Orsini LF, Cacciari E, Bovicell L, Tassoni P, Reggiani A. Pelvic ultrasonography in premenarcheal girls: relation to puberty and sex hormone concentrations. Arch Dis Child. 1985;60:120–5.CrossRef Salardi S, Orsini LF, Cacciari E, Bovicell L, Tassoni P, Reggiani A. Pelvic ultrasonography in premenarcheal girls: relation to puberty and sex hormone concentrations. Arch Dis Child. 1985;60:120–5.CrossRef
10.
go back to reference Yollin E, Jonard S, Reyss AC, Cortet-Rudelli C, Dewailly D. Delayed puberty with extreme uterine hypotrophy: do not conclude too early to the absence of the uterus. Gynecol Obstet Fertil. 2006;34:1029–35.CrossRef Yollin E, Jonard S, Reyss AC, Cortet-Rudelli C, Dewailly D. Delayed puberty with extreme uterine hypotrophy: do not conclude too early to the absence of the uterus. Gynecol Obstet Fertil. 2006;34:1029–35.CrossRef
11.
go back to reference Akierman SV, Skappak CD, Girgis R, Ho J. Turner syndrome and apparent absent uterus: a case report and review of the literature. J Pediatr Endocrinol Metab. 2013;26:587–9.CrossRef Akierman SV, Skappak CD, Girgis R, Ho J. Turner syndrome and apparent absent uterus: a case report and review of the literature. J Pediatr Endocrinol Metab. 2013;26:587–9.CrossRef
12.
go back to reference Schmidt PJ, Luff JA, Haq NA, Vanderhoof VH, Koziol DE, Calis KA, Rubinow DR, Nelson LM. Depression in women with spontaneous 46, XX primary ovarian insufficiency. J Clin Endocrinol Metab. 2011;96:E278-287.CrossRef Schmidt PJ, Luff JA, Haq NA, Vanderhoof VH, Koziol DE, Calis KA, Rubinow DR, Nelson LM. Depression in women with spontaneous 46, XX primary ovarian insufficiency. J Clin Endocrinol Metab. 2011;96:E278-287.CrossRef
13.
go back to reference Orshan SA, Ventura JL, Covington SN, Vanderhoof VH, Troendle JF, Nelson LM. Women with spontaneous 46, XX primary ovarian insufficiency (hypergonadotropic hypogonadism) have lower perceived social support than control women. Fertil Steril. 2009;92:688–93.CrossRef Orshan SA, Ventura JL, Covington SN, Vanderhoof VH, Troendle JF, Nelson LM. Women with spontaneous 46, XX primary ovarian insufficiency (hypergonadotropic hypogonadism) have lower perceived social support than control women. Fertil Steril. 2009;92:688–93.CrossRef
14.
go back to reference Alzubaidi NH, Chapin HL, Vanderhoof VH, Calis KA, Nelson LM. Meeting the needs of young women with secondary amenorrhea and spontaneous premature ovarian failure. Obstet Gynecol. 2002;99:720–5.PubMed Alzubaidi NH, Chapin HL, Vanderhoof VH, Calis KA, Nelson LM. Meeting the needs of young women with secondary amenorrhea and spontaneous premature ovarian failure. Obstet Gynecol. 2002;99:720–5.PubMed
15.
go back to reference Schmidt PJ, Cardoso GM, Ross JL, Haq N, Rubinow DR, Bondy CA. Shyness, social anxiety, and impaired self-esteem in Turner syndrome and premature ovarian failure. JAMA. 2006;295:1374–6.CrossRef Schmidt PJ, Cardoso GM, Ross JL, Haq N, Rubinow DR, Bondy CA. Shyness, social anxiety, and impaired self-esteem in Turner syndrome and premature ovarian failure. JAMA. 2006;295:1374–6.CrossRef
16.
go back to reference Rafique S, Sterling EW, Nelson LM. A new approach to primary ovarian insufficiency. Obstet Gynecol Clin North Am. 2012;39:567–86.CrossRef Rafique S, Sterling EW, Nelson LM. A new approach to primary ovarian insufficiency. Obstet Gynecol Clin North Am. 2012;39:567–86.CrossRef
17.
go back to reference Carel JC, Elie C, Ecosse E, Tauber M, Leger J, Cabrol S, Nicolino M, Brauner R, Chaussain JL, Coste J. Self-esteem and social adjustment in young women with Turner syndrome–influence of pubertal management and sexuality: population-based cohort study. J Clin Endocrinol Metab. 2006;91:2972–9.CrossRef Carel JC, Elie C, Ecosse E, Tauber M, Leger J, Cabrol S, Nicolino M, Brauner R, Chaussain JL, Coste J. Self-esteem and social adjustment in young women with Turner syndrome–influence of pubertal management and sexuality: population-based cohort study. J Clin Endocrinol Metab. 2006;91:2972–9.CrossRef
18.
go back to reference Bondy CA, Turner syndrome study G. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab. 2007;92:10–25.CrossRef Bondy CA, Turner syndrome study G. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab. 2007;92:10–25.CrossRef
19.
go back to reference Donaldson MD, Gault EJ, Tan KW, Dunger DB. Optimising management in Turner syndrome: from infancy to adult transfer. Arch Dis Child. 2006;91:513–20.CrossRef Donaldson MD, Gault EJ, Tan KW, Dunger DB. Optimising management in Turner syndrome: from infancy to adult transfer. Arch Dis Child. 2006;91:513–20.CrossRef
20.
go back to reference Popat VB, Calis KA, Kalantaridou SN, Vanderhoof VH, Koziol D, Troendle JF, Reynolds JC, Nelson LM. Bone mineral density in young women with primary ovarian insufficiency: results of a three-year randomized controlled trial of physiological transdermal estradiol and testosterone replacement. J Clin Endocrinol Metab. 2014;99:3418–26.CrossRef Popat VB, Calis KA, Kalantaridou SN, Vanderhoof VH, Koziol D, Troendle JF, Reynolds JC, Nelson LM. Bone mineral density in young women with primary ovarian insufficiency: results of a three-year randomized controlled trial of physiological transdermal estradiol and testosterone replacement. J Clin Endocrinol Metab. 2014;99:3418–26.CrossRef
21.
go back to reference Anasti JN, Kalantaridou SN, Kimzey LM, Defensor RA, Nelson LM. Bone loss in young women with karyotypically normal spontaneous premature ovarian failure. Obstet Gynecol. 1998;91:12–5.CrossRef Anasti JN, Kalantaridou SN, Kimzey LM, Defensor RA, Nelson LM. Bone loss in young women with karyotypically normal spontaneous premature ovarian failure. Obstet Gynecol. 1998;91:12–5.CrossRef
22.
go back to reference Ucar A, Abaci A, Pirgon O, Dundar B, Tutunculer F, Catli G, Anik A, Kilinc Ugurlu A, Buyukgebiz A. A synopsis of current practice in the diagnosis and management of patients with Turner Syndrome in Turkey: a survey of 18 pediatric endocrinology centers. J Clin Res Pediatr Endocrinol. 2018;10:230–8.CrossRef Ucar A, Abaci A, Pirgon O, Dundar B, Tutunculer F, Catli G, Anik A, Kilinc Ugurlu A, Buyukgebiz A. A synopsis of current practice in the diagnosis and management of patients with Turner Syndrome in Turkey: a survey of 18 pediatric endocrinology centers. J Clin Res Pediatr Endocrinol. 2018;10:230–8.CrossRef
23.
go back to reference Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, Lin AE, Mauras N, Quigley CA, Rubin K, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017;177:G1–70.CrossRef Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, Lin AE, Mauras N, Quigley CA, Rubin K, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017;177:G1–70.CrossRef
24.
go back to reference Hewitt SC, Li L, Grimm SA, Chen Y, Liu L, Li Y, Bushel PR, Fargo D, Korach KS. Research resource: whole-genome estrogen receptor alpha binding in mouse uterine tissue revealed by ChIP-seq. Mol Endocrinol. 2012;26:887–98.CrossRef Hewitt SC, Li L, Grimm SA, Chen Y, Liu L, Li Y, Bushel PR, Fargo D, Korach KS. Research resource: whole-genome estrogen receptor alpha binding in mouse uterine tissue revealed by ChIP-seq. Mol Endocrinol. 2012;26:887–98.CrossRef
25.
go back to reference Hamilton KJ, Hewitt SC, Arao Y, Korach KS. Estrogen hormone biology. Curr Top Dev Biol. 2017;125:109–46.CrossRef Hamilton KJ, Hewitt SC, Arao Y, Korach KS. Estrogen hormone biology. Curr Top Dev Biol. 2017;125:109–46.CrossRef
26.
go back to reference Taylor AE, Adams JM, Mulder JE, Martin KA, Sluss PM, Crowley WF Jr. A randomized, controlled trial of estradiol replacement therapy in women with hypergonadotropic amenorrhea. J Clin Endocrinol Metab. 1996;81:3615–21.PubMed Taylor AE, Adams JM, Mulder JE, Martin KA, Sluss PM, Crowley WF Jr. A randomized, controlled trial of estradiol replacement therapy in women with hypergonadotropic amenorrhea. J Clin Endocrinol Metab. 1996;81:3615–21.PubMed
27.
go back to reference Rebar RW, Erickson GF, Yen SS. Idiopathic premature ovarian failure: clinical and endocrine characteristics. Fertil Steril. 1982;37:35–41.CrossRef Rebar RW, Erickson GF, Yen SS. Idiopathic premature ovarian failure: clinical and endocrine characteristics. Fertil Steril. 1982;37:35–41.CrossRef
28.
go back to reference Rebar RW, Connolly HV. Clinical features of young women with hypergonadotropic amenorrhea. Fertil Steril. 1990;53:804–10.CrossRef Rebar RW, Connolly HV. Clinical features of young women with hypergonadotropic amenorrhea. Fertil Steril. 1990;53:804–10.CrossRef
29.
go back to reference Nelson LM, Anasti JN, Kimzey LM, Defensor RA, Lipetz KJ, White BJ, Shawker TH, Merino MJ. Development of luteinized graafian follicles in patients with karyotypically normal spontaneous premature ovarian failure. J Clin Endocrinol Metab. 1994;79:1470–5.PubMed Nelson LM, Anasti JN, Kimzey LM, Defensor RA, Lipetz KJ, White BJ, Shawker TH, Merino MJ. Development of luteinized graafian follicles in patients with karyotypically normal spontaneous premature ovarian failure. J Clin Endocrinol Metab. 1994;79:1470–5.PubMed
30.
go back to reference Conway GS, Kaltsas G, Patel A, Davies MC, Jacobs HS. Characterization of idiopathic premature ovarian failure. Fertil Steril. 1996;65:337–41.CrossRef Conway GS, Kaltsas G, Patel A, Davies MC, Jacobs HS. Characterization of idiopathic premature ovarian failure. Fertil Steril. 1996;65:337–41.CrossRef
32.
go back to reference Griffin IJ, Cole TJ, Duncan KA, Hollman AS, Donaldson MD. Pelvic ultrasound measurements in normal girls. Acta Paediatr. 1995;84:536–43.CrossRef Griffin IJ, Cole TJ, Duncan KA, Hollman AS, Donaldson MD. Pelvic ultrasound measurements in normal girls. Acta Paediatr. 1995;84:536–43.CrossRef
33.
go back to reference Nabhan ZM, Dimeglio LA, Qi R, Perkins SM, Eugster EA. Conjugated oral versus transdermal estrogen replacement in girls with Turner syndrome: a pilot comparative study. J Clin Endocrinol Metab. 2009;94:2009–14.CrossRef Nabhan ZM, Dimeglio LA, Qi R, Perkins SM, Eugster EA. Conjugated oral versus transdermal estrogen replacement in girls with Turner syndrome: a pilot comparative study. J Clin Endocrinol Metab. 2009;94:2009–14.CrossRef
34.
go back to reference Kim HJ, Lee DY, Yoon BK, Choi D. Uterine development after estrogen replacement therapy in women with different etiologies of primary hypogonadism. J Pediatr Adolesc Gynecol. 2016;29:344–7.CrossRef Kim HJ, Lee DY, Yoon BK, Choi D. Uterine development after estrogen replacement therapy in women with different etiologies of primary hypogonadism. J Pediatr Adolesc Gynecol. 2016;29:344–7.CrossRef
35.
go back to reference Snajderova M, Mardesic T, Lebl J, Gerzova H, Teslik L, Zapletalova J, Czech National Study Group for HRTOiP, Adolescent E, Gynaecology. The uterine length in women with Turner syndrome reflects the postmenarcheal daily estrogen dose. Horm Res. 2003;60:198–204.PubMed Snajderova M, Mardesic T, Lebl J, Gerzova H, Teslik L, Zapletalova J, Czech National Study Group for HRTOiP, Adolescent E, Gynaecology. The uterine length in women with Turner syndrome reflects the postmenarcheal daily estrogen dose. Horm Res. 2003;60:198–204.PubMed
36.
go back to reference Burt E, Davies MC, Yasmin E, Cameron-Pimblett A, Mavrelos D, Talaulikar V, Conway GS. Reduced uterine volume after induction of puberty in women with hypogonadism. Clin Endocrinol (Oxf). 2019;91:798–804.CrossRef Burt E, Davies MC, Yasmin E, Cameron-Pimblett A, Mavrelos D, Talaulikar V, Conway GS. Reduced uterine volume after induction of puberty in women with hypogonadism. Clin Endocrinol (Oxf). 2019;91:798–804.CrossRef
37.
go back to reference McDonnell CM, Coleman L, Zacharin MR. A 3-year prospective study to assess uterine growth in girls with Turner’s syndrome by pelvic ultrasound. Clin Endocrinol (Oxf). 2003;58:446–50.CrossRef McDonnell CM, Coleman L, Zacharin MR. A 3-year prospective study to assess uterine growth in girls with Turner’s syndrome by pelvic ultrasound. Clin Endocrinol (Oxf). 2003;58:446–50.CrossRef
38.
go back to reference Paterson WF, Hollman AS, Donaldson MD. Poor uterine development in Turner syndrome with oral oestrogen therapy. Clin Endocrinol (Oxf). 2002;56:359–65.CrossRef Paterson WF, Hollman AS, Donaldson MD. Poor uterine development in Turner syndrome with oral oestrogen therapy. Clin Endocrinol (Oxf). 2002;56:359–65.CrossRef
Metadata
Title
Menarche in primary ovarian insufficiency after a month of hormone replacement therapy: a case report
Author
Biwen Cheng
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2021
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-020-02603-6

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