Development Of Secondary Sexual Characteristics Research Articles

Late Presentation of Mayer-Rokitansky-Kuster-Hauser Syndrome in the Tropics-Northern Nigeria

Mayer-Rokitansky-Kuster-Hauser syndrome is a congenital malformation characterized by failure of the mullerian duct to develop, resulting in missing of the uterus and upper part of the vagina in women showing normal development of secondary sexual characteristics and a 46, XX karyotype. It is the second most common cause of primary amenorrhea after Turner’s syndrome. A 30 year old nulliparous lady who has been married for 13years presented with history of primary amenorrhoea. Examination revealed a young lady, with normal female pubic and axillary hair distribution, and breast was at tanner stage 4. Pelvic examination showed a normal sized clitoris, labia majora and minora, normal urethra, and blind ended vagina extending to about 7cm. Abdomino-pelvic USS revealed both kidneys normal in position and size, hypoplastic uterus measuring 1.05cm in anteroposterior diameter, left adnexium and pouch of Douglas were normal. Buccal and peripheral blood smear show Barr bodies and drumstick appendages suggested karyotype is XX. She subsequently had diagnostic laparoscopy which revealed a streak of thickened ligament at the position of the uterus. Both ovaries and their ligaments were visualised with a follicular cyst on the right ovary. We Case Report Yakasai et al.; BJMMR, 5(8): 1077-1082, 2015; Article no.BJMMR.2015.12

A rare association of schizophrenia and Mayer-Rokitansky-Küster-Hauser syndrome.

Sir, Schizophrenia is known to be associated with the various congenital disorders such as craniofacial anomalies, DiGeorge syndrome but its association with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome has rarely been reported.[12] MRKH syndrome is characterized by congenital aplasia of the uterus and the upper part. (2/3) of the vagina in women showing the normal development of secondary sexual characteristics and a normal 46, XX karyotype.[3] To the best of our knowledge, till date, there has only been one published case report of the coexistence of these two disorders.[4] We report another case of a 24-year-old unmarried woman Ms. A, with MRKH syndrome and schizophrenia for its interesting phenomenology and management. She presented with worries related to primary amenorrhea and infertility since adolescence, though detailed evaluation had not been done before. This was followed after 3 years by suspiciousness, the delusion of control and impulsive behavior. She believed that a certain unseen man staying abroad was controlling her mind and wants to marry her. She would hear voices of that man and converse with him. She would experience sensations in her vaginal area that she would attribute to sexual intercourse with that man, possible through some scientific technology devised by him. In addition, she would get distressed due to hearing voices of some other unknown persons, discussing about her and commanding her to do certain activities. Those unknown people would know her thoughts and control her actions through a transmitter device fitted in her ear. Ms. A would become irritable, aggressive, and violent toward family members whenever confronted regarding her beliefs. On psychiatric assessment, the psychopathology was confirmed. She had hallucinatory behavior, fearful affect, thought broadcast, the delusion of control, second and third person auditory hallucinations, a haptic hallucination of sexual content, impaired judgment with absent insight on mental status examination. She received a diagnosis of schizophrenia, paranoid type with MRKH syndrome. Diagnosis of MRKH was established by the presence of primary amenorrhea, normal development of secondary sexual characteristics, external genitalia, and ultrasonography finding of absence of the uterine structure between the bladder and rectum. The patient was started on oral olanzapine 10 mg/day, which was later, changed to oral quetiapine up to 600 mg/day with a satisfactory response. She was counseled for the vaginal atresia and vaginoplasty was recommended just before marriage after the consultation with the gynecologist. Future treatment options such as in vitro fertilization and embryo transfer was also discussed with the patient and family members. MRKH syndrome is associated with a number of neuropsychiatric manifestations such as distortions of body image, anxiety, depression, suicide, inter-personal sensitivity, and high psychological distress.[3567] Recent genetic research shows the deletion 17q12 is a recurrent,. pathogenic copy number variants that confers a very high risk for schizophrenia,[8] similarly 17q12 complex micro deletion is also found to be associated with the MRKH syndrome[910] suggestive of common genetic link between the two disorders. Both, psychiatrists and gynecologists need to be aware of this association and more research is required to test the link between these two disorders. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Acknowledgement We acknowledge the help of the department of obstetrics and gynecology in helping us to manage the patient.

Role of imaging in Mayer-Rokitansky-Kuster-Hauser syndrome

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital absence of the uterus and upper 2/3 rd of vagina with a normal 46, XX karyotype and normally developed secondary sexual characteristics. It affects 1:4000 women. It may be isolated (Type I) or frequently associated with renal, vertebral anomalies and less frequently associated with auditory, cardiac defects (Type II). It presents with primary amenorrhea in young women with normal external genitalia and normal development of secondary sexual characteristics with normal functioning ovaries and karyotype 46, XX without visible chromosomal anomaly. It was considered as sporadic anomaly but an increasing number of familial cases is suggestive of genetic cause in whom it is transmitted as autosomal dominant trait with incomplete penetrance and variable expressibility. This is suggestive of involvement of mutation in a major development gene or a limited chromosomal imbalance. Etiology of MRKH syndrome is still unclear. In order to allow sexual intercourse, treatment is aimed at creation of neovagina. Patients and their families must attend counseling before and throughout treatment as psychological distress is very important in young women with MRKH. We hereby report a rare case of MRKH syndrome in a 19-year-old married female patient presenting with primary amenorrhea, coital difficulty and no other clinical disorder.

The Role of MRI in the Management of Cases of Mayer-Rokitansky- Kuster-Hauser (MRKH) Syndrome

Mayer- Rokitansky- Kuster- Hauser (MRKH) syndrome is a rare entity characterized by congenital aplasia of the uterus and the upper part of 2/3rd of vagina in normal karyotype females with normal development of secondary sexual characteristics. The incidence rate is about 1 in every 4500 females. We report a case series of three different patients who presented with different clinical picture to various outpatient departments of our hospital. Magnetic Resonance Imaging of the pelvis was done in all these patients.

Mutational Analysis of the LHX1 Gene in a French National Cohort of Patients with Mayer-Rokitansky-Küster-Hauser Syndrome

Mutational Analysis of the LHX1 Gene in a French National Cohort of Patients with Mayer-Rokitansky-Kuster-Hauser Syndrome Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH syndrome) is characterized by congenital aplasia of the uterus and the upper part of the vagina in women showing normal secondary sexual characteristics development and a normal 46, XX karyotype. MRKH syndrome is a cause of primary amenorrhea with an incidence of approximately 1 in 5,000 newborn girls. Most cases of MRKH syndrome are sporadic, but some familial cases have been reported, suggesting a genetic cause. The genetic basis of MRKH syndrome remains largely unknown and seems heterogeneous. The LHX1 gene has been suggested to be a strong candidate, because (i) inactivation of the ortholog gene (Lim1) in the mouse model causes a Mullerian aplasia, and (ii) it is located in the chromosomal region 17q12, where deletions including this gene have been identified in approximately 5% of MRKH patients. Recently, three relevant heterozygous mutations of the LHX1 gene have been reported in association with the syndrome.

Effect of food on seasonal response in Indian weaver bird (Ploceus philippinus)

We investigated the effect of normal (adlib) and supplementary food on body mass, testes growth, moult and plumage color in Indian weaver birds. Birds intially were kept under natural day length (NDL) and then exposed to short day lengths (8 h light: 16 h darkness) for at least 2 weeks before being subjected to an experimental regime. They were divided into five groups (n=4 each), groups 1 and 2 were held under SDL (Short day length -8L: 16D), groups 3 and 4 were exposed to LDL (Long daylength-16L: 8D) and simultaneously group 5 held under natural day length, served as control. One group of SDL (Group 2) and LDL (group 3) as well as group 5 (NDL) were fed on grain with supplement proteinaceous food and groups 2 and 4 were fed on adlib food (grain) only. Observations on body mass, testis growth, bodymolt and plumage color were recorded bi weekly. A significant change in the body mass under both photoperiodic groups (SDL; group 2 and LDL; group 4), but the testicular growth was noticed on long day lengths (LDL: 16L: 8D) and control (NDL) group. However the supplement food modulated the testes growth, body molt, beak pigmentation and body plumage regeneration. Early feather regeneration was found in supplementary food group under long day length. It is suggested that the supplementary food, affects the timing of the development of secondary sexual characteristics.

Factor structure and factorial invariance of the State-Trait Anxiety Inventory for Chinese children and adolescents.

As previous research utilizing Spielberger's State-Trait Anxiety Inventory (Form Y; STAI-Y) has mostly involved adults or clinical groups, there have been relatively few reports assessing adolescents. This study is the first using data for children and adolescents in mainland China, from a large-scale cross-sectional survey in Beijing (Sample 1) and a longitudinal survey from the Wenchuan 512 earthquake (Sample 2), to clarify the factor structure and factorial invariance of the STAI-Y, Mandarin Chinese version. As a result, only in Sample 1 did a comparison of 11 confirmatory factor analysis models indicate the best goodness-of-fit indices shown by a two-factor structure for both state and trait anxiety, with both models reaching the selected cutoff criteria. These two optimal models were used in a subsequent simultaneous confirmatory factor analysis to test four conditions of factorial invariance, using eight participant groups divided on the basis of sex and school grade (fourth, fifth, sixth, and seventh to ninth). The two-factor structure state and trait anxiety models achieved the cutoff criteria for factorial invariance, with the exception of male fourth graders. Further, it was clearly shown that in comparison with the early stage of puberty, as puberty advanced the absence of state and trait anxiety gradually decreased, while scores for the presence of anxiety gradually increased. At the same time, in the case of Sample 2, which had experienced a traumatic event, as the goodness-of-fit indices for none of the 11 models reached the cutoff criteria, the factor scores showed arbitrariness and a lack of objectivity. The authors conclude that cognitive structure with regard to the STAI-Y may change with traumatic experience or the development of secondary sex characteristics at the onset of or in the stage of puberty. Also, computing the scores according to the STAI-Y manual is problematic.

Efficacy risks of the minimal-invasive plastic and reconstructive neovagina Vecchietti technique in Mayer-Rokitansky-Küster-Hauser syndrome.

Introduction: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder clinically defined by primary amenorrhea and infertility, congenital aplasia of the uterus and upper vagina. The patients with MRKH-syndrome have a female karyotype (46, XX), normally functioning ovaries and regular development of secondary sexual characters. Generally, the initial clinical sign of the syndrome is the failure to begin the menstrual cycle. Even though the psychological impact of the disease may be overwhelming, its physiological disorder can be successfully treated, after the surgical intervention the patients being able to have a normal sexual function. Reproduction may also be possible with assisted techniques. Objective: The aim of the study was to evaluate the efficacy risks of the traditional laparoscopic Vecchietti method in the plastic and reconstructive treatment of vaginal agenesis, documenting complications, as well as anatomical and functional results by means of a prospective, single center, single arm study. Material and methods: Between 2003 and 2011, 53 patients with MRKH-syndrome were treated in the gynecological clinic of the Kochi’s Lakshmi Hospital, Kerala, India. All the patients suffered from primary amenorrhea and the karyotype was (46, XX). The clinical suspicion was confirmed sonographically and intraoperatively, showing an absent uterus or the presence of rudimentary uterine horns, with normal ovaries. All the patients received a plastic laparoscopic neovagina construction, in order to achieve a satisfactory sexual function. We adopted a combined strategy: the patients were prepared by a psychological counselling, followed by the surgical intervention and a postoperative follow-up. The surgical procedure used in this series was a laparoscopic modified Vecchietti’s technique. All the patients were dismissed with the obligation to have intercourse and/or vaginal dilation 3 times a week for 6 months. Results: The patients’ ages varied between 13 and 40 years, with a mean age of 25 years. The mean operation time was 42.3 minutes, with a range between 36 and 63 minutes. In 2 cases, an intraoperative bladder injury occurred. A good qualitative vaginal tissue was clinically achieved in all the patients. The mean follow up period of the patients was 4 years ranging from 1 to 5 years. The anatomic success was observed in 94% of the cases and functional success in 79%. Conclusion: The anatomical failures in 6% were in women not having regular intercourse or not performing vaginal dilatation postoperatively. The psychological impact of the disease in combination with the performed surgery seems responsible for the difference of 15% of the cases where the anatomical result was good, but no regular intercourse could be achieved. The minimal-invasive plastic and reconstructive neovagina Vecchietti technique in MRKH-syndrome has a high success rate. In case of an intraoperative urinary bladder injury, the repair can be performed laparoscopically during the same session. Qualitatively good vaginal tissue and anatomical reconstruction can easily be achieved. In one patient desiring assisted reproductive medicine, a transvaginal oocyte retrieval was achieved.

Physical growth, puberty and hormones in adolescents with Nodding Syndrome; a pilot study.

BackgroundNodding syndrome is an epidemic symptomatic generalized epilepsy syndrome of unknown cause in Eastern Africa. Some patients have extreme short stature. We hypothesized that growth failure in nodding syndrome is associated with specific endocrine dysfunctions. In this pilot study, we examined the relationship between serum hormone levels and stature, bone age and sexual development.ResultsWe recruited ten consecutive children, 13 years or older, with World Health Organization defined nodding syndrome and assessed physical growth, bone age, development of secondary sexual characteristics and serum hormone levels. Two children with incomplete results were excluded. Of the eight remaining, two had severe stunting (height for age Z [HAZ] score < -3) and three had moderate stunting (HAZ score between-3 and -2). The bone age was delayed by a median 3(range 0-4) years. Serum growth hormone levels were normal in all eight but the two patients with severe stunting and one with moderate stunting had low levels of Somatomedin C (Insulin like Growth Factor [IGF1]) and/or IGF binding protein 3 (IGFBP3), mediators of growth hormone function. A linear relationship was observed between serum IGF1 level and HAZ score. With the exception of one child, all were either pre-pubertal or in early puberty (Tanner stages 1 and 2) and in the seven, levels of the gonadotrophins (luteinising and follicle stimulating hormone) and the sex hormones (testosterone/oestrogen) were all within pre-pubertal ranges or ranges of early puberty. Thyroid function, prolactin, adrenal, and parathyroid hormone levels were all normal.ConclusionsPatients with nodding syndrome may have dysfunctions in the pituitary growth hormone and pituitary gonadal axes that manifest as stunted growth, delayed bone age and puberty. Studies are required to determine if such endocrine dysfunction is a primary manifestation of the disease or a secondary consequence of chronic ill health and malnutrition and if so, whether targeted interventions can improve outcome.

Neovaginoplasty Using Labial and Vestibular Advancement Flap in Patients with Müllerian Agenesis

Müllerian agenesis is a rare congenital anomaly of the female genital tract with an incidence of 1 in 4000 female births and accounts for approximately 10% of primary amenorrhea. Affected individuals have absence of the vagina and uterus with normal 46XX female karyotype, functional ovaries and normal development of secondary sexual characteristics. Various surgical techniques have been suggested for neovagina formation in patients with müllerian agenesis. The most widely used surgical techniques include McIndoe operation, laparoscopic Davydov procedure, and laparoscopic Vechietti procedure.

Distribution and male reproductive system of the Bridled Mabuya, Trachylepis vittata (Olivier, 1804) (Reptilia: Scincidae), in Lebanon

The distribution of the Bridled Mabuya, Trachylepis vittata, in Lebanon is described and the male reproductive cycle was studied in a population living at 2000 m a.s.l. on Mount Sannine. Males emerge from hibernation in March, one month earlier than females. They show a short seasonal testicular cycle in which spermiogenesis occurs in spring, followed by testicular regression and subsequent recrudescence in summer. Hibernation starts in October and lasts 5 months. The development of secondary sexual characteristics was in synchrony with the testicular cycle. There was a significant difference between the reproductive stages and the testicular volume. The species is assigned to an associated reproductive cycle with short vernal spermiogenesis.

Swyers Syndrome: primary amenorrohea with gonadal dysgenesis a rare cause

Swyer syndrome is an uncommon form of gonadal dysgenesis, characterised by a 46, XY karyotype (1). In spite of presence of Y chromosome the affected individual has a female phenotype as the dysgenetic gonad produces neither AMH nor androgens. As a result of failure of masculinisation of both internal and external genitalia is that of a female. These cases generally present at puberty with primary amenorrhoea and lack to development of secondary sexual characteristics. With this case report, we aimed to underline the necessity of considering 46, XY complete pure gonadal dysgenesis in the differential diagnosis in every adolescent female patient with primary amenorrhoea and the importance of early gonadectomy in order to avoid the risk for gonadal tumour development.

46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma.

Sex reversal syndrome is a rare congenital condition of complete or disordered gonadal development leading to discordance between the genetic, gonadal and phenotypic sexes, including 46,XX and 46,XY. The gonadoblastoma on the Y-chromosome (GBY) region is associated with an increased risk of developing type II germ cell tumors/cancer. The present study reports a unique case of a phenotypically normal female (age 17 years), presenting with primary amenorrhea and later diagnosed with 46,XY female sex reversal syndrome. Following bilateral gonadectomy, bilateral gonadoblastoma and dysgerminoma were diagnosed. Thus, estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation, and to prevent osteoporosis. A four year follow-up showed no tumor recurrence and a regular menstrual cycle in this patient.

Associations between early alcohol and tobacco use and prolonged time to puberty in boys.

Previous research has demonstrated a relationship between prepubertal alcohol and tobacco use and delayed pubertal characteristics in girls. Although, laboratory research indicates that alcohol and tobacco use inhibits sexual maturation in male rats, human research in this area is lacking. To address this question among boys, we conducted a study to explore the association between early use of alcohol and tobacco and time to development of secondary sexual characteristics. The study population included 3199 boys interviewed between the ages of 11 and 21. Participants reported the ages at which they first experienced body hair growth, deepening of the voice and facial hair growth. Early alcohol and tobacco use were defined as first use preceding the age of pubertal development among those reporting regular consumption patterns. Hazard ratios (HR) and 95% confidence intervals (CI) were calculated using Cox proportional hazard models. Early alcohol use was associated with longer time to body hair growth (HR 0.77; 95% CI 0.69-0.87), voice changes (HR 0.72; 95% CI 0.64-0.82) and facial hair growth (HR 0.77; 95% CI 0.68-0.86), after adjusting for tobacco use and age at interview. Tobacco use was not independently associated with the puberty indicators after controlling for alcohol use and age at interview. Our findings are consistent with the hypothesis that alcohol may inhibit puberty onset in boys, an association that has been previously observed among young girls. Thus, alcohol may be an exposure deserving more scrutiny as a disruptor to normal pubertal development.

Relationship between Bone Age, Chronological Age, Anthropometric Parameters, and Diagnosed Pulse Rate on secondary sexual character development of child-Adolescence

*Two authors contributed equally to this article. Aijoa Korean Medical Clinic Suwon, Dept. of Acupuncture and Moxibustion, College of Korean Medicine, Kyung Hee University, Aijoa Korean Medical Clinic Busan, Aijoa Korean Medical Clinic Pyungtaek, Suwon Jaseng Hospital of Korean Eastern Medicine, Gangnam Inuri Korean Medical Clinic, Dept. of Korean Medicine Classics, College of Korean Medicine, Kyung Hee University, Dept. of Herbology, College of Korean Medicine, Kyung Hee University

Advances in the etiology, diagnosis and treatment of central precocious puberty

The onset of puberty is first detected as an increase in the amplitude and frequency of pulses of gonadotropin-releasing hormone (GnRH) after a quiescent period during childhood. The reemergence of pulsatile GnRH secretion leads to increases in the secretion of the gonadotropins, luteinizing hormone (LH), and follicle-stimulating hormone (FSH) by the pituitary gland, and the consequent activation of gonadal function. Early activation of the hypothalamic-pituitary-gonadal axis results in gonadotropin-dependent precocious puberty, also known as central precocious puberty (CPP), which is clinically defined by the development of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys. Pubertal timing is influenced by complex interactions among genetic, nutritional, environmental, and socioeconomic factors. CPP is diagnosed on the basis of clinical signs of progressive pubertal development before the age of 8 years in girls and 9 years in boys, pubertal basal and/or GnRH-stimulated LH levels, and advanced bone age. Magnetic resonance imaging of the central nervous system is essential for establishing the CPP form as organic or idiopathic. Depot GnRH-analogues represent the first-line of therapy in CPP. Very recently, the genetic component of CPP was demonstrated by the evidence that the deficiency of the MKRN3 gene, located on long arm of chromosome 15, causes familial CPP in humans. In this current review, clinical and therapeutic aspects of the CPP will be discussed, contributing to adequate diagnosis and criterious approach of this relevant condition of pediatric endocrinology.

The impact of increased physical exertion on the state of adrenal cortex and pubertal development in boys

The study of the functional state of the adrenal cortex (AC) in young athletes aged 11 to 15 years, which was assessed by daily free (Cf) and bound (Cb) cortisol excretion levels, and the comparison of these levels with those of the boys from the control group made it possible to conclude that the increased physical exertion in the form of regular athletic training had a dominating effect on the age-related changes in the AC and pubertal development in the young athletes. It was found that the 12- to 14-year-old athletes had stably high Cf excretion levels, which were significantly decreased by the age of 15 years on the background of high Cb levels, as compared to the nonathletes, whose urinary cortisol levels were significantly lower (p < 0.05) and were progressively increased (p < 0.05) from 13 to 15 years of age. It was found that pubertal development (the development of secondary sex characteristics) was relatively delayed in the athletes, and pubertal changes in the glucocorticoid function of the AC reflected mainly its adaptive responses providing the increased resistance of the child’s body to increased physical exertion.

Pubertal development profile in patients with Turner syndrome.

Puberty can be divided into two independent events: adrenarche and gonadarche. In healthy children, adrenarche is followed by gonadarche, but in patients with gonadal dysgenesis there is partial or complete dissociation between these two events. To evaluate the age and chronology of the development of secondary sexual characteristics and occurrence of these events and their relationship to the induction of puberty in patients with Turner syndrome (TS). A descriptive analysis with historical records of the patients with clinical and cytogenetic TS was conducted. The following variables were recorded: karyotype; age of thelarche, pubarche, and menarche; occurrence of spontaneous puberty; maintenance of puberty or secondary failure; and the onset of hormone replacement therapy (HRT) with estrogen. We evaluated 123 medical charts. Seven (5.7%) patients were prepubertal, 10 (8.1%) had only pubarche, and 5 (4%) had only thelarche. Forty-seven (38.2%) patients entered puberty spontaneously. Among these, 35 (28.5%) remained in puberty, and 12 (9.8%) required subsequent HRT; 54 (44%) had puberty induced. Sixty-six (56.9%) patients had pubarche started before thelarche. Menarche occurred in 67 patients, spontaneously in 19. Pubarche spontaneously presented in 91 (78.4%) patients, and in 25 (21.5%) after HRT introduction. Spontaneous puberty occurred in approximately one-third of the patients. Pubarche was the first feature in most patients and about 20% showed pubarche only after estrogen therapy.

Study of Age of Appearance and Development of Secondary Sexual Characteristics amongst Adolescents of the Bikaner Region, Rajasthan

Estimation of the age of living persons is required in many civil and criminal cases and in questionable cases. Registration of birth certificate is the most reliable document as an evidence of real age, but for many reasons, births are not registered in many cases in India, especially in the rural areas. This requires various radiological investigations to be carried out to estimate the age. In rural settings, these facilities are not available and, if available, are limited to the use for patients who are in more need of such medical facilities. Thus, physical examination involving secondary sexual characteristics promises to be an easy and cost-effective means for the same purpose. The objectives of this study were: (1) to find out the age of appearance of various secondary sexual characteristics in adolescent males and females; (2) to note the ages of development of various stages of such characteristics; and (3) to find the maximum age after which a specific characteristic is universally present. The study involved the examination of adolescents of both the sexes, aged between 12 and 20 years, coming to the Out patient department (OPD) of the Forensic Medicine Department of the PBM Hospital and from the population residing around the campus. A total of 165 subjects (109 males and 56 females) were examined to study the appearance and development of various secondary sexual characteristics. Breast development started before the age of 12 years and most girls aged between 12 and 13 years exhibited stage A development. All girls aged above 17 years showed stage D development. Pubic hairs were in stage A in 90% of the girls aged between 12 and 13 years and 100% girls beyond 17 years exhibited stage D development. Axillary hairs were present in 100% subjects aged above 16 years. In males, pubic hairs appeared later than in females, but were present in all cases above 14 years and were in stage D in boys aged above 17 years. Axillary hairs appeared between 14 and 15 years of age in all the cases. Adam's apple became prominent between 14 and 16 years of age in all the cases and facial hairs appeared between 15 and 16 years of age. Secondary sexual characteristics could be utilised as a definite procedure for age estimation within a narrow age range in maximum number of cases, thus saving time, energy and resources. Remaining doubtful cases could be solved with more sophisticated methods, like radiological examination and dental analysis, requiring special diagnostic tools and specialised personnel. Though some regional variations were seen in secondary sexual characteristics, as were seen in other methods too, these could be easily worked out by undertaking regional research and setting the standards.

Development of secondary sexual characters in a case of idiopathic hypogonadotropic hypogonadism

We report a rare case of idiopathic hypogonadotropic hypogonadism in a 15 year old adolescent girl, who presented in gynecology outpatient clinic with complaint of primary amenorrhea and poor development of secondary sexual characters. Her clinical examination revealed small, underdeveloped breasts (Tanner stage II), absent axillary hair and sparse pubic hair (Tanner stage II). Ultrasound pelvis showed uterus of 4.76 x 2.29 x 1.25 centimeter, small and hypoplastic with very thin endometrial lining, bilateral ovaries were small with 1-2 small follicles. Magnetic Resonance Imaging (MRI) of the hypothalamic-pituitary unit showed no abnormality. Further workup was done to detect other causes of hypogonadism, which was normal, hence we concluded the patient to have idiopathic hypogonadotropic hypogonadism.