scholarly journals Primary amenorrhoea secondary to two different syndromes: a case study

2019 ◽  
Vol 12 (3) ◽  
pp. e228148
Author(s):  
Zareen Kiran ◽  
Tayyaba Jamil
Keyword(s):  
Turner Syndrome ◽  
Bone Growth ◽  
Single Patient ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Müllerian Agenesis ◽  
Sexual Characteristic ◽  
Secondary Sexual

Turner syndrome is a relatively common chromosomal abnormality presenting as primary amenorrhoea in gynaecological and endocrine clinics, caused by complete or partial X monosomy in some or all cells. Mayer-Rokitansky-Kuster-Hauser syndrome is another common cause of primary amenorrhoea characterised by Mullerian agenesis of varying degrees. We report a case of an 18-year-old girl, who presented with primary amenorrhoea, absence of secondary sexual characteristics and short stature. Hormonal profile confirms hypergonadotrophic hypogonadism. Karyotyping was consistent with Turner syndrome (45,XO). In addition, radiological imaging of the pelvis showed the absence of both ovaries as well as the uterus, cervix and vagina. This patient had therefore presented with two different syndromes as the cause of her primary amenorrhoea, which is extremely rare in a single patient. Moreover, oestrogen replacement therapy will trigger the development of secondary sexual characteristic and promote bone growth, but induction of menstruation and fertility is impossible.

Two cases of 17α-hydroxylase deficiency - one combined with complete gonadal agenesis

1981 ◽  
Vol 98 (2) ◽  
pp. 267-273 ◽  
Author(s):  
Erling Tvedegaard ◽  
Viggo Frederiksen ◽  
Klaus Ølgaard ◽  
Meta Damkjær Nielsen ◽  
Jørgen Starup
Keyword(s):  
Plasma Renin ◽  
Sequential Therapy ◽  
Plasma Corticosterone ◽  
Hydroxylase Deficiency ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Male Genotype ◽  
Gonadal Agenesis ◽  
Secondary Sexual

Abstract. Two cases of 17α-hydroxylase deficiency are described. Both patients had primary amenorrhoea, total lack of female secondary sexual characteristics, slight hypertension and hypokalaemia. One patient was of male genotype (male pseudohermaphrodite), and in addition this patient had complete gonadal agenesis. The other patient was of female genotype. In both patients the level of plasma corticosterone was markedly increased, whereas the concentration of plasma cortisol was very low and plasma aldosterone low within the normal range. Furthermore, the plasma ACTH level was significantly increased and the plasma renin activity around the lower normal limit. The urinary excretion of corticosterone metabolites was markedly increased, whereas the excretion of both cortisol metabolites and tetrahydroaldosterone was decreased. The patients had no symptoms of glucocorticoid deficiency. Treatment with dexamethasone 0.5 mg daily completely suppressed the abnormal corticosterone production and normalized both blood pressure and serum potassium. In addition, the patient of male genotype has received sequential therapy with oestrogen and gestagen for 3 years, but so far no development of the secondary sexual characteristics has occurred.

scholarly journals Adolescent Girls with Pure Gonadal Dysgenesis: A Rare Disease

2018 ◽  
Vol 36 (4) ◽  
pp. 170-174
Author(s):  
Joysree Saha ◽  
Kohinoor Begum ◽  
Kamil Ara Khanom ◽  
Indrajit Prasad ◽  
Sumaya Akter
Keyword(s):  
Gonadal Dysgenesis ◽  
Ovarian Function ◽  
Sensorineural Deafness ◽  
Pubic Hair ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Patient Will ◽  
History Of ◽  
Secondary Sexual

Gonadal dysgenesis is a rare cause of primary amenorrhoea ,which is a relatively common problem among teenage girls.Primary amenorrhoea occurs in patient with gonadal dysgenesis because of absence or limited ovarian function due to inappropriate development.Streak gonads are unable to produce estrogens and/or androgens,resulting in minimal to no development of secondary sexual characteristics.Adrenal androgens may induce production of pubic hair,but patient will have minimal breast development.These patients may have a family history of infertility, short stature,sensorineural deafness,ataxia,mild mental retardation or gonadoblastoma. Here two cases of primary amenorrhoea due to pure gonadal dysgenesis are presented. 1 st  one was a 18yr old girl whose mother consulted with a gynaecologist at the age of 16yr because of her worries about absence of menarche of her daughter and secondone was a 14yr old girl whose mother consulted with a gynaecologist at the age of 16yr because of absence of secondary sexual characteristics as well as menarche of her daughter. In both cases, blood test showed very high levels of follicle stimulating hormone (FSH) & luteinizing hormone (LH), low levels of oestradiol& very low level of AMH. USG findings of both cases showed a bit hypoplastic uterus and volume of ovaries were smaller than normal. A diagnostic laparoscopy with biopsy of both gonads of one case was performed.Another case did not give consent for laparoscopy.Hormonal replacement therapy was applied on them for establishment of normal menstruation and menstruation was established in both cases.  An early diagnosis is extremely important to prevent long term consequences of Gonadal dysgenesis.J Bangladesh Coll Phys Surg 2018; 36(4): 170-174

Amenorrhea

2021 ◽  
pp. 175573802110316
Author(s):  
Pooja Munjal ◽  
Manju Nair
Keyword(s):  
Common Type ◽  
Reproductive Age ◽  
Primary Amenorrhea ◽  
Women Of Reproductive Age ◽  
Secondary Amenorrhea ◽  
Secondary Amenorrhoea ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Secondary Sexual

Amenorrhoea is defined as absence of menstruation in women of reproductive age. Primary amenorrhoea is a failure to start menstruation by the age of 13 years without secondary sexual characteristics or by the age of 15 years with normal secondary sexual characteristics. Secondary amenorrhoea is the absence of menstruation for 6 months in a woman with normal prior menstruation. Secondary amenorrhea is more common type, with a prevalence of between 3 and 4%. This compares with a prevalence of 0.3% for those with primary amenorrhea.

scholarly journals Clinical and Radiological Findings in Mayer-Rokitansky-Küster-Hauser Syndrome type 2

2021 ◽  
Author(s):  
Samih Abed Odhaib ◽  
Miaad Jassim Mohammed ◽  
Ahmed Jaafar Hindi Al-Ali ◽  
Abbas Ali Mansour
Keyword(s):  
Iliac Fossa ◽  
Unknown Etiology ◽  
Primary Amenorrhea ◽  
Radiological Findings ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Müllerian Agenesis ◽  
The Right ◽  
Secondary Sexual

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) or (Müllerian Agenesis) represents uterovaginal aplasia or hypoplasia of unknown etiology in young women with usual 46, XX karyotype, and normal secondary sexual characteristics. We report a 15-year-old adolescent woman with primary amenorrhea, and normal pubertal secondary sexual characteristics, normal hormonal workup, and clinical examination. Abdominopelvic magnetic resonance imaging (MRI) revealed cervical and uterine agenesis, with the absence of the proximal thirds of the vagina. Both kidneys are fused in the right iliac fossa with oval lobulated appearance, picture of crossed fused ectopia. The ovaries are normal and located bilaterally. The diagnosis of MRKHS type 2 was confirmed based on clinical, biochemical, and radiological findings. The correct clinical and radiological diagnosis of MRKHS by MRI is crucial for the next steps in long-term management.   Keywords: Müllerian Agenesis, Uterovaginal malformations.

scholarly journals Secondary Sexual Characteristics in the Galerucinae (Sensu Stricto) (Coleoptera: Chrysomelidae)

ISRN Zoology ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-60 ◽  
Author(s):  
M. S. Mohamedsaid ◽  
D. G. Furth
Keyword(s):  
Sensu Stricto ◽  
The Body ◽  
Line Drawings ◽  
Secondary Sexual Characteristics ◽  
Secondary Sexual Characteristic ◽  
The Family ◽  
Sexual Characteristics ◽  
Species Specific ◽  
Sexual Characteristic ◽  
Secondary Sexual

A list of 1298 species and 172 genera of Chrysomelidae from the subfamily Galerucinae (sensu stricto) with the males having at least one form of secondary sexual characteristic (SSC) is presented. The number of species amounts to 24% of the total Galerucinae presently known from all over the world—a very significant amount. The SSCs comprise various types of modified structures found on all parts of the body—head, thorax, and abdomen. They are not variable but species specific. Illustrations from selected 87 species that include 84 images and 15 line drawings showing various types of SSC are provided. The amazing array of SSCs from the Galerucinae offers a large and taxonomically diverse set of data that are not comparable with other subfamilies in the Chrysomelidae and may be useful in phylogenetic analysis of the family.

scholarly journals Prospective study of primary amenorrhoea and its management in a rural tertiary centre

2019 ◽  
Vol 8 (9) ◽  
pp. 3442
Author(s):  
Pavanaganga A. ◽  
Sai Prathyusha Ivalla ◽  
Nagarathnamma R. ◽  
Sailakshmi M. P. A.
Keyword(s):  
Prospective Study ◽  
Social Life ◽  
Primary Amenorrhea ◽  
Imperforate Hymen ◽  
Her Family ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Tertiary Centre ◽  
Sexual Characteristics ◽  
Secondary Sexual

Background: Amenorrhoea (absence of menstruation) is a symptom of varied causes. It results from dysfunction of hypothalamic-pituitary ovarian axis, uterus and vagina. It is a major concern for pubertal girls and their family members. It has a major impact on the physical, mental, psychological and social life of the girl and her family. The objective of the present study was to evaluate the aetiology and management of primary amenorrhoea in young adolescent girls.Methods: It was a prospective study conducted for a period of 2 years from August 2016 to July 2018 at Rajarajeswari medical college and hospital. Patients presenting with history of amenorrhoea that is, absence of menses by the age of 13 years with no visible development of secondary sexual characteristics or by 15 years of age with the presence of normal secondary sexual characteristics were included in our study. Cases of secondary amenorrhoea were excluded. Detailed history, examination, investigations and management was documented and analysed.Results: A total of 25 patients of primary amenorrhea were studied during the study period. In our study outflow tract anomalies were the commonest cause of amenorrhoea accounting for 84%, of which imperforate hymen (32%) and Mayer Rokitansky Küster Hauser syndrome (MRKH) 36% were the two most common Mullerian anomaly causing primary amenorrhoea. Gonadal dysgenesis accounted for 12% of the cases. Amenorrhoea was the commonest complaint patients presented with accounting to 76%, followed by cyclical pain abdomen accounting for 16% of cases.Conclusions: Primary amenorrhoea is multifactorial and is of major concern among adolescent girls. Early diagnosis and intervention has an impact on the physical and psychological wellbeing of the girl.

Primary Amenorrhoea with Pituitary Dwarfism: A rare case report

2016 ◽  
Vol 2 (2) ◽  
pp. 145-147
Author(s):  
Siva S ◽  
Divya Gopineni ◽  
Shafi P ◽  
Chandra Sekhar
Keyword(s):  
Pituitary Gland ◽  
General Medicine ◽  
Pituitary Hormones ◽  
Thyroid Stimulating Hormone ◽  
Primary Amenorrhea ◽  
Pituitary Dwarfism ◽  
Primary Amenorrhoea ◽  
Sexual Characteristics ◽  
Loss Of Appetite ◽  
Secondary Sexual

Females with pituitary dwarfism and a multiple deficiency of pituitary hormones show ovarian dysfunction due to hypogonadotropism. Primary amenorrhea can be diagnosed if a patient has normal secondary sexual characteristics but no menarche by 16 years of age. A 16 year-old female patient admitted in general medicine department with chief complaints of shortness of breath on exertion since 15 days, swelling of both legs since 10 days, loss of weight since 5 months, loss of appetite since 3 months, history of pain during swallowing. Pelvis scan examination reveals that uterus measures 3.2×0.5×0.5cm; uterus is hypo plastic, ovaries not visualized. Patient parents reveled that from patient birth to 11years of age her growth and other developments were normal, after that her growth is stopped and no changes were observed in development since 5 years. Patient has hypothyroidism so pituitary gland make an important role to maintain hormone levels, pituitary gland produces thyroid stimulating hormone (TSH) which stimulates thyroid gland to produce thyroid hormones. Primary Amenorrhea, short stature and poorly developed secondary sexual characters which could have been contributed and should be subjected for karyotyping. This type of Pituitary Dwarfism is very difficult to manage.

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