Letters to the Editor

PEDIATRICS ◽  
1973 ◽  
Vol 52 (1) ◽  
pp. 150-150
Author(s):  
Gertrude Costin ◽  
Maurice D. Kogut
Keyword(s):  
Thyroid Hormone ◽  
Clinical Data ◽  
Pubic Hair ◽  
Breast Development ◽  
Letters To The Editor ◽  
Female Patients ◽  
Secondary Sexual Characteristics ◽  
Adequate Therapy ◽  
Sexual Characteristics ◽  
Axillary Hair

Drs. Costin and Kogut comment as follows: We were pleased to read Dr. Comas' letter in which he describes a 12-4/12-year-old girl whose clinical data suggest that she may be an example of the syndrome that we reported. The evidence for this was the occurrence of menstruation, pubic hair, and breast development when the patient was hypothyroid and disappearance of menstruation following treatment with thyroid hormone. It is not clear why Dr. Comas' patient had an increase in pubic hair and appearance of axillary hair following treatment at a time when her menstrual periods ceased; in all the reported female patients regression of the secondary sexual characteristics was noted following adequate therapy.

Prophylactic gonadectomy in 46 XY females; why, where and when?

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Nurul Iftida Basri ◽  
Chong Hong Soon ◽  
Anizah Ali ◽  
Nur Azurah Abdul Ghani ◽  
Ani Amelia Zainuddin
Keyword(s):  
Histopathological Examination ◽  
Early Adulthood ◽  
Breast Development ◽  
Case Presentation ◽  
Female Patients ◽  
Primary Amenorrhoea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Operative Assessment ◽  
Male Karyotype

Abstract Objectives We compared cases of phenotypic female patients who presented with male karyotype and underwent prophylactic gonadectomy. Case presentation Five patients with female phenotypes presented in early adulthood with primary amenorrhoea with varying degrees of puberty. One was tall with breast development. Another was very short with clitoromegaly and multiple co-morbidities. The other three had no secondary sexual characteristics. They were examined, after which hormonal profile, karyotyping, ultrasound examination and magnetic resonance imaging were done to assess the site of gonads. Gonadectomy was performed once their 46 XY karyotype was confirmed. Results of histopathological examination of their gonads ranged from dysgenetic gonads to having testicular tissues and malignancy. Conclusion Female patients with 46 XY karyotypes require prophylactic gonadectomy performed at different timings depending on diagnosis due to the malignancy risk. Pre-operative assessment is essential to locate the gonads prior to surgery.

REPLACEMENT ORAL ETHINYLOESTRADIOL THERAPY FOR GONADAL DYSGENESIS: GROWTH AND ADRENAL ANDROGEN STUDIES

1979 ◽  
Vol 91 (3) ◽  
pp. 519-528 ◽  
Author(s):  
Anne W. Lucky ◽  
Samuel P. Marynick ◽  
Robert W. Rebar ◽  
Gordon B. Cutler ◽  
Michael Glen ◽  
...  
Keyword(s):  
Gonadal Dysgenesis ◽  
Bone Age ◽  
Pubic Hair ◽  
Height Velocity ◽  
First Year ◽  
Adrenal Androgen ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Second Year ◽  
Pre Treatment

ABSTRACT We have studied growth and adrenal dehydroepiandrosterone (DHA) responses to iv synthetic adrenocorticotrophic hormone (ACTH, Cortrosyn) in 6 girls with gonadal dysgenesis before and during treatment with lowdose ethinyloestradiol (EOe2). In all patients there was a statisfactory induction of secondary sexual characteristics including increase in breasts and pubic hair and onset of withdrawal bleeding within 6 months of therapy. Height velocity increased from 2.8 ± 0.9 cm/year pre-treatment to 5.3 ± 1.5 cm/year (P < 0.02) in the first year. There was deceleration to 1.9 ± 1.1 cm/year in the second year. There was no disproportionate advancement in bone age and thus, presumably, no loss of ultimate height. We could demonstrate no change in basal or ACTH-stimulated levels of DHA, a specific adrenal androgen, to account for the increased pubic hair and growth in these patients.

scholarly journals Turner's Syndrome: Approach to Diagnosis and Treatment

2018 ◽  
Vol 13 (3) ◽  
pp. 61-62
Author(s):  
Sadhana Sah ◽  
Ganesh Dangal ◽  
Aruna Karki ◽  
Hema Pradhan ◽  
Ranjana Shrestha ◽  
...  
Keyword(s):  
Short Stature ◽  
Estrogen Therapy ◽  
Tanner Stage ◽  
Breast Development ◽  
Primary Amenorrhea ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Secondary Sexual Characteristics ◽  
Karyotypic Abnormality ◽  
Sexual Characteristics

Turner's syndrome is the most common karyotypic abnormality causing gonadal failure and primary amenorrhea. It is characterized by short stature and absence of secondary sexual characteristics. It is diagnosed by increased plasma FSH and LH level with low level of estrogen i.e. hypergonadotrophic hypogonadism. Ultrasound abdomen reveals streak ovaries and atrophic uterus. Karyotype confirms the diagnosis of Turner's syndrome (45XO). We present here a 15 years girl who presented with primary amenorrhea with short stature with breast development corresponds to Tanner stage I. Her FSH was raised. Ultrasound abdomen showed uterine agenesis and streak ovaries. Karyotype showed 45XO which confirmed the diagnosis of Turner's syndrome. She is now on estrogen therapy and her height has increased and breast development corresponds to Tanner stage II. Keywords: hypergonadotrophic hypogonadism, primary amenorrhea, Turner's syndrome

Testosterone Therapy for Pubertal delay

1992 ◽  
Vol 13 (1) ◽  
pp. 5-39
Keyword(s):  
Anabolic Steroids ◽  
Pubic Hair ◽  
Height Velocity ◽  
Delayed Puberty ◽  
Secondary Sexual Characteristics ◽  
Self Image ◽  
Pubertal Delay ◽  
Constitutional Delay ◽  
Sexual Characteristics ◽  
Age Appropriate

Despite considerable variation in the age at which puberty normally begins, a male adolescent who has demonstrated neither testicular nor pubic hair growth by 14 years of age is considered to have delayed puberty. Although such delay may indicate hypopituitarism or isolated gonadotropin deficiency, most commonly it represents a normal variation, termed "constitutional delay in growth and maturation." Such patients eventually will enter puberty and achieve normal sexual maturation and adult height. However, during adolescence, such delays may be accompanied by impaired self-image and social isolation. Under these conditions, many pediatric endocrinologists advocate short-term use of anabolic steroids. Such therapy is aimed at accelerating height velocity during the ensuing year, accompanied by the development of age-appropriate secondary sexual characteristics.

PLASMA TESTOSTERONE IN MALE PUBERTY

1974 ◽  
Vol 75 (1) ◽  
pp. 181-194 ◽  
Author(s):  
D. Knorr ◽  
F. Bidlingmaier ◽  
O. Butenandt ◽  
H. Fendel ◽  
R. Ehrt-Wehle
Keyword(s):  
Bone Age ◽  
Pubic Hair ◽  
Cross Sectional Study ◽  
Growth Spurt ◽  
Plasma Testosterone ◽  
Gas Liquid Chromatography ◽  
Cross Sectional ◽  
Sexual Characteristics ◽  
Testicular Size ◽  
Axillary Hair

ABSTRACT Plasma testosterone was investigated by gas-liquid chromatography with an electron capture detector in a cross-sectional study in childhood and during puberty in 214 boys. The testosterone values were correlated with sexual characteristics such as axillary hair, pubic hair, testicular size and bone age. In 22 individuals during puberty a longitudinal study was started, indicating a very steep increase of plasma testosterone between 40 and 240 ng/100 ml. This stage is mostly passed through very rapidly within 10 months. Plasma testosterone, growth velocity and weight gain were correlated in two boys during puberty. The peak of growth spurt occurs between 50 and 170 ng of plasma testosterone.

Zespół policystycznych jajników jako problem w ginekologii i medycynie rodzinnej, z uwzględnieniem pacjentek w wieku rozwojowym

2018 ◽  
Vol 21 (4) ◽  
Author(s):  
Grażyna Jarząbek-Bielecka ◽  
Justyna Opydo-Szymaczek ◽  
Małgorzata Mizgier ◽  
Katarzyna Bojanowska ◽  
Magdalena Pisarska-Krawczyk ◽  
...  
Keyword(s):  
Polycystic Ovary Syndrome ◽  
Polycystic Ovary ◽  
Clinical Signs ◽  
Developmental Changes ◽  
Breast Development ◽  
Ovary Syndrome ◽  
Secondary Sexual Characteristics ◽  
Common Causes ◽  
Sexual Characteristics ◽  
Stimulation Of

Symptoms of female developmental changes associated with puberty appear at the age of 9 to 14 years. These include the formation of secondary sexual characteristics that begin with breast development followed by rapid growth and, finally, the first menstruation (menarche), after which fertility is achieved. There is no fixed age limit for puberty in girls. It may start early or late. It may be also disturbed by endocrinopathies. Polycystic ovary syndrome (PCOS), which develops at a late stage of puberty, is one of them. The syndrome is associated with hyperandrogenism and overweight. At the same time, it is one of the most common causes of hyperandrogenism in women. Polycystic ovary syndrome is manifested by the lack of menstruation, hirsutism, obesity and significantly enlarged cystic ovaries. Girls with clinical signs of polycystic ovary syndrome show a different pattern of GnRH secretion during early adolescence, which may lead to abnormal stimulation of the maturing ovary. Therefore, polycystic ovary syndrome is not only a problem in adult gynaecology, but also in adolescent gynaecology.

Normal puberty and its disorders

2020 ◽  
pp. 2428-2435
Author(s):  
Fiona Ryan ◽  
Sejal Patel
Keyword(s):  
Clinical Assessment ◽  
Significant Variation ◽  
Reproductive Capacity ◽  
Breast Development ◽  
Sequence Of Events ◽  
Clinical Presentations ◽  
Sexual Characteristics ◽  
Hair Development ◽  
Axillary Hair ◽  
Development Proceeds

Puberty is the physiological sequence of events when secondary sexual characteristics develop, reproductive capacity is achieved, and final adult stature reached. The outward signs usually develop over 3 to 5 years, with significant variation both in the age that puberty starts and the pace at which development proceeds. The events that lead to the triggering of puberty remain uncertain, but clinical presentations may arise because the process is abnormally early (precocious puberty) or abnormally late (delayed or absent puberty). Several variants of the normal processes may also present for clinical assessment, for example, premature isolated thelarche (breast development) or adrenarche (pubic and axillary hair development), which do not require treatment.

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

scholarly journals Long-Term Follow-Up and Treatment of a Female With Complete Estrogen Insensitivity

2020 ◽  
Vol 105 (5) ◽  
pp. 1478-1488 ◽  
Author(s):  
Soumia Brakta ◽  
Lynn P Chorich ◽  
Hyung-Goo Kim ◽  
Laurel A Coons ◽  
John A Katzenellenbogen ◽  
...  
Keyword(s):  
Medical Center ◽  
Academic Medical Center ◽  
Lower Abdominal Pain ◽  
Breast Development ◽  
White Female ◽  
Vaginal Smear ◽  
Primary Amenorrhea ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Secondary Sexual

Abstract Context We previously reported the first female with a causative ESR1 gene variant, who exhibited absent puberty and high estrogens. At age 15 years, she presented with lower abdominal pain, absent breast development, primary amenorrhea, and multicystic ovaries. The natural history of complete estrogen insensitivity (CEI) in women is unknown. Objective The purpose of this report is to present the neuroendocrine phenotype of CEI, identify potential ligands, and determine the effect of targeted treatment. Design We have characterized gonadotropin pulsatility and followed this patient’s endocrine profile and bone density over 8 years. Seventy-five different compounds were tested for transactivation of the variant receptor. A personalized medicine approach was tailored to our patient. Setting Academic medical center. Patient or Other Participants A 24-year-old adopted white female with CEI. Intervention(s) The patient was treated with diethylstilbestrol (DES) for approximately 2.5 years. Main Outcome Measure(s) Induction of secondary sexual characteristics. Results Luteinizing hormone (LH) pulse studies demonstrated normal pulsatile LH secretion, elevated mean LH, and mildly elevated mean follicle-stimulating hormone (FSH) in the presence of markedly increased estrogens. DES transactivated the variant ESR1 in vitro. However, DES treatment did not induce secondary sexual characteristics in our patient. Conclusions Treatment with DES was not successful in our patient. She remains hypoestrogenic despite the presence of ovarian cysts with a hypoestrogenic vaginal smear, absent breast development, and low bone mineral mass. Findings suggest additional receptor mechanistic actions are required to elicit clinical hormone responses.

scholarly journals Central Precocious Puberty in a Three-Year-Old Girl

2021 ◽  
Vol 27 (3) ◽  
pp. 341
Author(s):  
Suryani Jamal ◽  
Liong Boy Kurniawan ◽  
Suci Aprianti ◽  
Ratna Dewi Artati ◽  
Ruland DN Pakasi ◽  
...  
Keyword(s):  
Physical Examination ◽  
Precocious Puberty ◽  
Tanner Stage ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Breast Development ◽  
Hypothalamic Hamartoma ◽  
Secondary Sexual Characteristics ◽  
Pelvic Ultrasonography ◽  
Sexual Characteristics

Precocious puberty is defined as the onset of secondary sexual characteristics before 8 years of age in girls and 9 years in boys. Central Precocious Puberty (CPP) is caused by early activation of the hypothalamic-pituitary-gonadal axis. Laboratory test of LH, FSH, and Estradiol is recommended for monitoring suppressive effects from GnRHa therapy in the early three months and every six months. This study aimed to report a case of CPP in a 3-year and 3-month-old girl. A 3-year and 3-month-old girl went to the hospital with vaginal bleeding (menstruation), breast development, and pubic and axilla hair for 7-month-old. Physical examination found moderately ill with obesity, body weight 20 kg, height 98 cm. Tanner stage was A2M3P2, café au lait was found in the left forehead with size 7x3.5 cm. In March 2015 before GnRHa therapy, LH, FSH and Estradiol level increased with levels of 4.32 mlU/mL, 6.01 mlU/mL, and 67 pg/mL, and after 3 months of the treatment was 0.87 mlU/mL, 2.51 mlU/mL and <20 pg/mL. Pelvic ultrasonography showed suggestive precocious puberty, bone age 5-year and 9-month (Greulich and Pyle), CT-Scan of the brain showed hypothalamic tumor suspected hypothalamic hamartoma. This patient was treated with a GnRHa injection every 4 weeks. Leuprorelin is a synthetic non-peptide analogue of natural GnRH. The diagnosis was based on medical history, physical examination, laboratory, and radiological findings. The prognosis of the patient was good.

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