Exploring the unusual: a testosterone-secreting ovarian tumor

ABSTRACT Ovarian steroid cell tumors are rare, representing less than 0.1% of all ovarian neoplasms. Among the myriad causes of hirsutism, ovarian tumors account for 1% of the reported cases. We present the case of a 49-year-old parous postmenopausal woman who sought medical attention for hirsutism for 2 years. This case illustrates the unusual and interesting connection between rare ovarian pathology and the clinical manifestation of hirsutism in a postmenopausal patient. Her ultrasonography and MRI showed a right adnexal mass of solid-cystic consistency with thin septations. Her laboratory workup revealed high levels of total testosterone of 256 ng/ml (8.4-48.1ng/ml) and free testosterone of 7.36 pg/ml (0.2-4.1 pg/ml), while DHEAS - 234 µg/dl (35.4-256 µg/dl) and CA125 - 15.8U/L (0.0-35 U/L) were in the normal range. She underwent exploratory laparotomy with a total abdominal hysterectomy and oophorectomy. Histopathological examination and immunohistochemistry conclusively established the presence of a steroid cell tumor, specifically classified as "Not Otherwise Specified"(NOS), in the right ovary.

ADULT ACNE: ITS CLINICOEPIDEMIOLOGICAL FEATURES

Although acne is principally a disorder of adolescence, the prevalence of adult acne is increasing. Adult acne has been defined as the presence of acne beyond the age of 25 years. Acne in adult women may have different clinical features i.e., more involvement of lower face, association with hair loss, premenstrual flare, signs and symptoms of insulin resistance, dyslipidaemias. Various studies have shown that a significant percentage of adult women with acne failed to respond to treatment with systemic antibiotics and isotretinoin which indicates a need for treatment alternatives with improved effectiveness and acceptable side effects for resistant acne. This study aims to study the different clinicoepidemiological features of adult acne.

Awareness of Polycystic Ovarian Syndrome among College Going Females in Gurgaon: A Cross-Sectional Study

Objectives:?Women not only take care of family members, they are also source of power and symbol of progress in a society. At the same time, physical and mental well-being of women relies on healthy lifestyle and adequate reproductive health knowledge. With growing incidence of polycystic ovarian syndrome (PCOS), it is crucial to increase awareness about the disease among women at an early age in life. The present survey investigates awareness level of college going females about PCOS. Materials and Methods?428 females were recruited from 3 colleges in district Gurgaon, Haryana, India, based on convenience sampling. The respondents filled a self-completion questionnaire containing sociodemographic details, menstrual cycle details, and questions related to PCOS. Statistical Analysis?Descriptive statistics was used to calculate frequency and percentage of variables. Pearson's chi-square test of independence was used to identify factors associated with awareness of PCOS. A p-value of?<?0.05 was considered to be statistically significant. Results?The mean ±SD age of respondents was 19.9?±?1.7 years (range?=?18–24 years). Only 78 females (18.22%) had heard about PCOS. Being knowledgeable was significantly associated with mother's education (p?=?0.001), length of menstrual cycle (p?=?0.022), and family history of PCOS (p?<?0.001). Conclusion?The present study indicates awareness of PCOS among college going females was very poor. There is an urgent need of increasing awareness about PCOS among young adult college going females not only for prevention, early diagnosis, and treatment of the disease but also to prevent its long-term consequences.

Sex cord stromal tumor of ovary masquerading as polycystic ovarian syndrome

Virilization is a portentous sign that suggests the possibility of an ovarian or adrenal neoplasm. Diagnosis may be delayed in some patients due to nonspecific symptoms and overlapping symptoms with that of polycystic ovarian syndrome (PCOS). However, it must be remembered that PCOS usually causes mild to moderate elevation of serum testosterone with hirsutism whereas serum testosterone levels are many times elevated in cases of androgen secreting tumors and virilization is a norm. So high testosterone level with new onset virilization rule out PCOS. Authors are reporting two cases of Sertoli Leydig cell tumor despite their similar histopathology and equivalent levels of serum testosterone had a varied clinical spectrum of virilization.

A Simple Assessing Method for Screening Prevalence of Polycystic Ovary Syndrome (The Thief of Womanhood) and Emotional Distress in Female Students at Northern Border University (NBU) in Arar, Kingdom of Saudi Arabia (KSA)

Background: Polycystic ovary syndrome (PCOS) is an endocrine condition which is caused due to disturbance in hormonal balances. There are more than one influencing factors that can lead to cause of PCOS. Yet, there are few reports that have fully elucidated the connection of conventional factors linked with PCOS so far. Aim and objectives: 1. To study the prevalence of risk factors associated with PCOS among female students of NBU, Arar, KSA. 2. To study the correlation between emotional distress and associated risk factors of PCOS among female students of NBU. 3. To create the awareness of PCOS and its associated risk factors among female students of NBU. Material and methods: The Northern Border University female students those were willing to participate were selected to enroll in our study. A pre-validated self-structured questionnaire was used as a tool for the collection of the data. Students were grouped into two viz PCOS-group and non-PCOS-group founded on criteria’s given by NIH (National Institutes of Health Criteria). As per this criteria diagnosis of PCOS is established founded on incidence of both chronic anovulation (ANOVU) characterized by occurrence of vaginal bleeding for more than 35 days gaps or 8 cycles/year to understand absence of menstruation and clinical hyperandrogenism (CH). CH is defined as the occurrence of acne, androgenic alopecia, acanthosis and hirsutism. Conclusion: Students recognized with linked risk factors for PCOS were guided and counseled to have complete investigations done including radiological and hormonal assays for confirming diagnosis and starting treatment.

Presentación de un caso de síndrome de Cooke-Apert-Gallais / Presentation of a case of Cooke-Apert-Gallais syndrome

Se estudió una paciente de 47 años de edad con antecedentes de buena salud, sin antecedentes atópicos personales ni familiares, que acude a la consulta de endocrinología por presentar aumento del vello corporal con dos años de evolución. Al examen físico se le constató rubicundez facial, escudo pubiano masculino, predominio de la cintura escapular sobre la cintura abdominal e hipertrofia de 3,5 cm del clítoris. Referente a los exámenes complementarios presentó un marcado aumento de la testosterona en sangre así que se le practicó una histerectomía con doble anisectomía y los resultados de la biopsia fueron: un tumor benigno de células de Leydig. Se concluyó que se trataba de un Síndrome de Cooke-Apert-Gallais por un tumor productor de testosterona en el ovario(AU)

A study was conducted of a female 47-year-old patient with a history of good health and no personal or family atopic antecedents, who attends endocrinology consultation due to increased growth of body hair of two years' evolution. Physical examination revealed facial reddishness, a male pubic shield, predominance of the scapular waist over the abdominal waist, and 3.5 cm hypertrophy of the clitoris. Complementary tests found a marked increase in blood testosterone, which led to the performance of hysterectomy with double adnexectomy. The result of the biopsy was benign Leydig cell tumor. The final diagnosis was Cooke-Apert-Gallais syndrome due to a testosterone-producing tumor in the ovary(AU)

Androgenic alopecia in post-menopausal female: a clinical presentation

Androgenic alopecia is a patterned hair loss occurring due to systemic androgens and genetic factors. It is the most common cause of hair loss in both genders. The appearance of this condition is the cause of significant stress and psychological problems, making appropriate management important. A 68-year-old postmenopausal female presented with complaints of increased hair loss from scalp, excessive hair growth at undesired sites and hirsutism not corrected with medications. On thorough investigations, CT scan whole abdomen and endocrinological workup, a clinical diagnosis of alopecia and hirsutism due to hyperandrogenemia secondary to ovarian tumor made. Abdominal hysterectomy with B/L salpingo-oophorectomy was done. Histopathological examination revealed an encapsulated tumor in right ovary-sex cord stromal tumor consistent with Leydig cell tumor in right ovary, no evidence of malignancy. Left ovary was normal. Patient showed significant regression of clinical signs and symptoms on follow up after 1 month. All women with severe hirsutism or androgenic alopecia needs further work up to locate the source of androgen over production.

Polycystic ovarian syndrome and insulin resistance: a South Indian study

Background: Polycystic ovary syndrome (PCOS) is a common endocrine disorder in women of reproductive age. Some studies have characterized different aspects of women presenting with PCOS. In this study we characterise the association of insulin resistance (IR) in patients with PCOS in the southern Indian state of Andhra Pradesh.Methods: A total of 50 women diagnosed to have PCOS according to Rotterdam criteria were studied. IR was estimated using Homeostatic model assessment - insulin resistance (HOMA-IR) and clinical characteristics were recorded.Results: The prevalence of IR among the study population was 36%. All PCOS patients with IR were overweight or obese, and had impaired glycaemic status, 75% of PCOS patients with IR also had features of hirsutism.Conclusions: Considering the prevalence of IR, obesity and impaired fasting glucose in women with PCOS, early institution of treatment by lifestyle changes or medication would lead to improvement in reproductive and metabolic abnormalities.

Study of cutaneous manifestations of polycystic ovarian syndrome

Background: Polycystic ovarian syndrome (PCOS) is one of the most frequently encountered endocrine disorders that occurs in as many as 4 to 10% of women of reproductive age group. It presents with a series of skin changes including acne, hirsutism, seborrhea, androgenetic alopecia (AGA) and acanthosis nigricans. Aim of the study was to determine the prevalence and frequency of different cutaneous manifestations in PCOS patients and to correlate them with the degree of hormonal abnormalities.Methods: A total 100 patients with features of PCOS who presented to department of dermatology, gynecology (January 2018-December 2019) with cutaneous manifestations were recorded and diagnosis of PCOS was made using Rotterdam’s criteria. Pregnant women and diagnosed cases of any other endocrine disorder were excluded. Hirsutism was assessed using Ferriman-Gallwey score and AGA according to Ludwig’s classification. Serum hormonal profile including FSH, LH, prolactin, testosterone (free), DHEAS, TSH, FBS, fasting insulin were done. Insulin resistance was determined by calculating HOMA-IR score.Results: Among cutaneous manifestations of PCOS, hirsutism (85%) was the most common finding followed by acne (73%), seborrhea (50%), AGA (36%), acanthosis nigricans (29%) and acrochordons (9%). The most common hormonal abnormality was insulin resistance in 53% patients, followed by raised free testosterone in 19% and serum prolactin in 18% patients. A statistically significant association was present between AGA and insulin resistance, hirsutism and raised prolactin levels, seborrhea and raised body mass index (p < 0.05).Conclusions: Dermatological manifestations of PCOS play a significant role in making the diagnosis and constitute a substantial portion of the symptoms experienced by women with this syndrome.

A Study on the Clinical and Biochemical Profile of Polycystic Ovary Syndrome Patients Attending a Tertiary Care Hospital in Bihar

Background: Polycystic ovarian syndrome (PCOS) is one of the most common reproductive endocrinological disorders with a broad spectrum of clinical manifestations affecting women of reproductive years. It is a common diagnosis in women presenting with infertility. All the dimensions of PCOS have not been completely explored. Many studies have tried to characterize the exact presentation of the disease. The main objective of this study was to find out the clinical and biochemical profile of patients with polycystic ovary syndrome (PCOS) in patient population attending the tertiary care hospital in Gaya, Bihar. Methods: A cross sectional study was done among 300 PCOS patients in a span of one year. Pregnant patients, patients with symptomatic disease and those of age less than 15 years and more than 35 years were excluded. PCOS patients were diagnosed using Rotterdam’s criteria. A detailed history and examination was obtained. Transvaginal USG was undertaken to detect PCOS. Anthropometric measurements were also done. Biochemical and hormonal test were done by standard methods. Results: The mean age group of patients was 28.11± 4.9 SD with mean BMI 26.12± 5.12. Maximum (42.7%) number of patients was in the age group 26-30 years. Out of 245 fertile patients, 68.6% were nulliparous. Among them, 82.7% had primary infertility. According to duration of infertility, maximum (45.2%) were of 6-10 years duration. About 93.7% were detected to have PCOS in ultrasonography. Androgenic features like acne was present in 22.3% of the patients, acanthosis nigricans in 18% of the patients and androgenic alopecia was present in 7.7%. The prevalence of metabolic syndrome was 21.7% while 20.3% had hypothyroidism. Elevated total testosterone was elevated in 41.3% of the patients. Conclusion: Early diagnosis and intervention will reduce the long term health complications associated with PCOS. A complete clinical, biochemical and radiological workup is necessary for appropriate management.

Etiologic Aspects of Hirsutism in Kashmiri Women Presenting to a District Hospital in Northern State of India

To assess the etiological aspects of hirsutism in Kashmiri women presenting to a district hospital in the northern state of India. Methods: Design: Prospective evaluation of hirsute patients referred to our endocrinology clinic. Setting: District-level primary care hospital. Patient(s): 150 consecutive women referred for hirsutism. Intervention(s): Assessment of body hair as per the Ferriman and Gallwey scoring system and an investi-gative protocol including detailed clinical assessment with endocrinologic workup including estimations of gonadotropins, PRL, T, and 17- hydroxyprogesterone and abdominopelvic ultrasound. Main Outcome Measure: Cause of hirsutism. Results: The etiology of hirsutism revealed idiopathic hirsutism in 35%, polycystic ovary syndrome (PCOS) in 35%, postmenopausal state in 12%, adrenal and ovarian tumors in 5 %, congenital adrenal hyperplasia in 2 %, and drug-induced hirsutism in 6 %. The cause remained undetermined in 5 % of patients for whom the available information was not adequate. Conclusion: Hirsutism is as common a problem in the Kashmir Valley (India) as elsewhere in the world. Idiopathic hirsutism (35 %), PCOS (35 %), and postmenopausal state ( 12 %) are common causes of hirsutism. Late-onset congenital adrenal hyperplasia is a relatively uncommon cause of hirsutism in the Kashmir Valley.

Evaluation of patients with polycystic ovarian syndrome at a tertiary care center

Background: It has been estimated that prevalence of PCOS ranges from 5-10% in reproductive females. Thus, it becomes most common endocrinopathy in this age group. Lack of ovulation and androgen over activity are key features of PCOS. The objective of this study was to patients with polycystic ovarian syndrome at a tertiary care center.Methods: Present study was following up study with 200 cases of PCOS. Rotterdam criteria were used for the diagnosis of the PCOS. This criterion includes three things. First is woman having in last year <6 periods of menstruation. Second is testosterone value >0.6 ng/ml and third is each ovary having >12 follicles with increased size and volume. Those cases with hypothyroidism, adrenal tumors, congenital adrenal hyperplasia etc were excluded from the present study. Those on steroid therapy were also excluded. Detailed history and clinical examination were carried out for included cases as well as required investigations.Results: Overall incidence of infertility was found as 27.9%. Out of these 200 cases, 44 (22%) had PCOS. Majority cases belonged to 20-24 years of age. Hirsutism was most common findings in clinical hyperandrogenism. Risk of insulin resistance syndrome was 4.79 times higher in obesity compared to not obese. Age, BMI, duration of infertility, ovarian volume, and menstrual pattern had effect on ovulation rate following laparoscopic ovarian drilling. Increasing duration of infertility and BMI had lower ovulation rate. Ovulation rate was highest when treated with clomiphene citrate alone than the combination therapy.Conclusions: Women with obesity have more chances of having PCOS as seen from the present study.

Study of prevalence and determinants of polycystic ovarian syndrome among adolescent girls in rural area: a prospective study

Background: Polycystic ovarian syndrome (PCOS) is the most frequent endocrinological disorder affecting 5-10% of women in the reproductive age. This prevalence ranging from 2.2% to 26% in adult women from 18-45 year. In a recent study the prevalence of a confirmed diagnosis of PCOS in adolescents aged 10 to 19 years was 5-15%, which increased to 10-22%. When undiagnosed cases with documented symptoms qualifying for PCOS according to NIH (National institute of Health) criteria were included. PCOS is a complex endocrine disorder which is most common in women of reproductive age. PCOS may first present in adolescence, but the incidence of PCOS in adolescence is not known, as diagnostic criteria for PCOS in the adolescent age-group is still not defined, PCOS symptoms tend to overlap with normal pubertal changes making the diagnosis even more challenging. The objective is to study prevalence and symptomatology of polycystic ovary syndrome (PCOS) in adolescent girls.Methods: Prospective Cross sectional study between April 2018 and March 2019. 150 adolescent girls aged 10 to 19 years attending OPD with oligomenorrhea, irregular menstrual cycle, obesity and/or hirsutism were advised for biochemical, hormonal, and ultrasonographic evaluation for diagnosis of PCOS on the basis of Rotterdam's criteria at the Department of Obstetrics and Gynaecology, Krishna Institute of Medical Sciences, Deemed University, Karad, Maharashtra, India.Results: Prevalence of PCOS in the study was 17.33% in the study group.Conclusions: PCOS is increasingly encountered during adolescence, although the overall prevalence is low and evaluation of PCOS in adolescents is challenging. At this age, lifestyle modification is imperative to prevent long-term metabolic and reproductive complications.

A comparative study of combination of Myo-inositol and D-chiro-inositol versus Metformin in the management of polycystic ovary syndrome in obese women with infertility

Background: Polycystic ovary syndrome (PCOS) is a symptom complex associated with increased amounts of circulating androgens in females, increased insulin resistance and obesity. The drugs, Myo-inositol, D-chiro-inositol and Metformin, which are insulin sensitizers, are very helpful in taking care of one of the key components of PCOS that is insulin resistance. This study was done to compare the effects of combination of Myo-inositol and D-chiro-inositol with the use of metformin on clinical and biochemical profile in PCOS.Methods: A prospective, randomized, comparative study was conducted on 200 patients. The patients were randomly assigned into the two groups of 100 each. Group A receiving Tab. Myoinositol 550mg twice daily and Tab. D-chiro-inositol 13.8mg twice daily and Group B receiving Tab. Metformin 500mg thrice daily. The patients were assessed by menstrual cycle regulation, hirsutism score (Ferriman Gallwey), fasting and post prandial glucose and insulin levels, serum DHEA levels, serum free testosterone levels and fasting day 3 serum LH and FSH ratio.Results: In both the groups there was significant improvement in all the above mentioned parameters, however the group with Combination of Myo-inositol and D-chiro-inositol had statistically significant improvement over the Metformin group.Conclusions: Combination of Myo-inositol and D-chiro-inositol and use of metformin, significantly improved insulin sensitivity in PCOS women. But combination of Myo-inositol and D-chiro-inositol was effective in controlling the hormonal profiles (LH/FSH ration and free testosterone) when compared to Metformin.

A Case of True Hermaphroditism, 46, XY DSD

A 26 year old student reared as a female, presented with inability to menstruate and increased facial hair growth. On examination , patient had hyperandrogenic features including hirsutism, low pitched voice, microphallus with hypospadias. Investigations revealed a 46 XY karyotype with increased testosterone and imaging revealed both ovaries and testes with a hypoplastic uterus. The patient was managed with bilateral testicular gonadectomy, feminising genitoplasty and hormonal therapy.

A prospective analysis of polycystic ovarian syndrome in infertile women

Background: Polycystic ovarian syndrome (PCOS) is a frequently occurring metabolic and reproductive endocrinopathy. Young women with PCOS mainly present with reproductive problems such as hyperandrogenism, menstrual irregularities, infertility and chronic anovulation. Despite its high prevalence and implications on reproductive health, PCOS is underdiagnosed. The objective was to study the prevalence of PCO in women with infertility, their symptomatology, endocrine profiles and coexisting factors of infertility in women with PCOS.Methods: A prospective study was performed over a period of 6 months. Women presenting with infertility were subjected to detailed history taking, general and gynecological examination. Women with features of PCOS on ultrasound were identified and advised investigations such as serum FSH, LH, prolactin and laparoscopy when indicated.Results: During the study period, 102 patients consulted the infertility clinic, out of which 56 were diagnosed with PCOS. The prevalence of PCOS was found to be 54.9% among infertile women. Menstrual irregularity was found in 33 (59%) women, however, 23 (41%) had regular menstrual cycles. 4 among the 33 patients complained of dysmenorrhea, 5 had heavy menstrual bleeding. On examination, 19 (33.9%) were found hirsute, 8 (14.28%) had thyroid enlargement and 2 (3.57%) had galactorrhea. Investigations revealed mean FSH and LH levels of 8.7±2SD and 13.7±2SD respectively and mean LH/FSH ratio of 1.57±2SD.Conclusions: PCOS is a heterogenous disorder and with its high prevalence in infertile women, proper diagnosis and management is essential as it has many potential metabolic and cardiovascular risks if not managed appropriately.

The Modified Ferriman-Gallwey Score and Hirsutism among Filipino Women

BACKGROUND: The modified Ferriman-Gallwey (mFG) score is the gold standard for the clinical evaluation of hirsutism. However, racial variations in terminal hair growth limit this tool. This study aimed to determine the mFG cut-off score among Filipino women and its association with biochemical hyperandrogenism.METHODS: A total of 128 Filipino women were included in this prospective cross-sectional study and were divided into two groups: a polycystic ovary syndrome (PCOS) group (n=28) and a non-PCOS group (n=100). The participants underwent mFG score determination, ovarian ultrasound conducted by a single sonographer, and hormone testing. The mFG cut-off score was determined based on the 95th percentile of the non-PCOS group. Logistic regression was used to analyze the relationship between mFG score and biochemical hyperandrogenism.RESULTS: Although the mFG score was generally low in both the PCOS and non-PCOS groups, the former exhibited a higher mean score than the latter (4.3±3.0 vs. 2.0±2.2, P<0.001). Normal values for the total mFG score ranged from 0 to 7. Using a cut-off score of 7, a higher proportion of hirsute women (mFG score ≥7) was observed in the PCOS group versus the non-PCOS group (17.9% vs. 5.0%, P=0.025). Elevated calculated free testosterone (FT) was also found to be significantly associated with hirsutism (odds ratio, 6.2; 95% confidence interval, 1.2 to 32.4 pmol/L; P=0.030).CONCLUSION: A score of 7 and above constitutes hirsutism in this population of Filipino women. Hirsute women are more likely than non-hirsute women to have elevated calculated FT.

Metabolic and hormonal assessment of adolescent and young adult women with prior premature adrenarche

OBJECTIVE: Follow-up studies of girls with premature adrenarche have reported the development of polycystic ovary syndrome, insulin resistance, and dyslipidemia and a propensity to cardiovascular disease. The aim of this study was to analyze the presence of these conditions in patients previously treated at the Universidade Federal do Triângulo Mineiro. METHODS: A total of 130 medical records reported premature adrenarche. One hundred and twenty-two patients were invited to participate, of whom 54 accepted; 34 patients were selected, as they had reached their final height. Anthropometric, blood glucose, insulin, and lipid and hormonal profile (LH, FSH, estradiol, 17α-OH-progesterone, androstenedione, dehydroepiandrosterone sulfate, testosterone) data were obtained, the HOMA-IR index was calculated, and pelvic ultrasonography was performed. To characterize polycystic ovary syndrome and metabolic syndrome, the Rotterdam and International Diabetes Federation criteria, respectively, were used. Data were analyzed according to measures of dispersion, frequency and correlations of interest. RESULTS: The age of the participants ranged from 15.2 to 28.2 years/months; 23.5% of the patients were overweight, 11.8% were obese, 29.4% had a large waist circumference, and 8.8% were hypertensive. None of the patients had altered glucose levels, and insulin levels and HOMA-IR were elevated in 29.4% and 38.2% of the participants, respectively; 14.7% of the patients exhibited acanthosis nigricans. The lipid profiles of the participants were variable, and one patient (2.9%) had metabolic syndrome. Polycystic ovary syndrome was found in 41.2% of patients. CONCLUSION: The percentage of patients with polycystic ovary syndrome who also had overweight, obesity and insulin resistance corroborates the literature data about the need for follow-up aiming at interventions, especially for conditions associated with cardiometabolic risk.

Tumor ovárico de células de Leydig en una niña de 7 años que se presentó como pubarquia precoz con elevación de 17 hidroxiprogesterona / Ovarian tumor of Leydig cells in a 7-year-old girl who presented as precocious pubarche with elevation of 17 hydroxyprogesterone

Clinical case: a girl of 7 ½ years who consulted for early pubarche without thelark, with a percentile size of 75 for a genetic target size in the 10th percentile, overweight with a 90th percentile BMI, and normal blood pressure. The biochemical study showed high levels of androgens: testosterone: 7.2 ng/dL, androstenedione of 5.1 ng / ml, 17OHP: 15 ng / dL with low normal DHEAS (0.26 ug/ml), Plasma Renin Activity normal low: 0.22 ng/mL/h. Initial imaging study showed a bone age of 10 years 6 months and normal abdominal and pelvic ultrasound. Molecular study showed no pathogenic variants in the CYP21A2 gene (21 Hydroxylase). With a probable diagnosis of non-classical congenital adrenal hyperplasia (HSRNC) and no known mutation, he started treatment with hydrocortisone (12 mg/m2). At 8.7 years, pubertal development begins and braking begins with LHRH analogues, which are administered for 18 months. Despite the treatment, signs of virilization and elevation of androgens (testosterone up to 130 ng/ml) are progressively accentuated, which do not diminish when trying different corticosteroid schemes. MRI of the abdomen and pelvis shows the normal adrenal glands and a solid nodular image of 2.1 x 1.6 cm in the right ovary (Figure 2), later demonstrated with pelvic ultrasound (Figure 2). Right laparoscopic oophorectomy was performed, whose biopsy demonstrated a Leydig cell tumor. One month after surgery, all androgenic levels were normalized, so the gradual suspension of corticosteroids began. Conclusion: Although HSRNC is the most frequent pathological cause of early pubarche, when it is associated with progressive clinical and biochemical hyperandrogenism despite adequate treatment and without pathogenic variants in the CYP21A2 gene, even with high levels of 17OHP, other causes should be considered, specifically, androgen producing tumors.

Caso clínico: niña de 7½ años que consulta por pubarquia precoz sin telarquia, con talla en percentil 75 para una talla objetivo genético en percentil 10, sobrepeso con IMC percentil 90 y presión arterial normal. El estudio bioquímico mostró niveles elevados de andrógenos: testosterona: 7,2 ng/dL, androstenediona de 5,1 ng/ml, 17OHP: 15 ng/dL con DHEAS normal baja (0,26 ug/ml), Actividad de Renina Plasmática normal baja: 0.22 ng/ mL/h. Estudio de imágenes inicial mostró una edad ósea de 10 años 6 meses y ecografía abdominal y pelviana normales. Estudio molecular no mostró variantes patogénicas en el gen CYP21A2 (21 Hidroxilasa). Con diagnosticó probable de hiperplasia suprarrenal congénita no clásica (HSRNC) y sin mutación conocida,inició el tratamiento con hidrocortisona (12 mg/m2). A los 8.7 años comienza desarrollo puberal y se inicia frenación con análogos de LHRH, los cuales se administran por 18 meses. A pesar del tratamiento se acentúan progresivamente los signos de virilización y hayelevación de los andrógenos (testosterona hasta 130 ng/ml), que no disminuyen intentando diferentes esquemas de corticoides. Se realiza RM de abdomen y pelvis que muestra las glándulas suprarrenales normales y una imagen nodular sólida de 2.1 x 1.6 cm en el ovario derecho (Figura 2), demostrada posteriormente con Ecografía pelviana (Figura 2). Se realiza ooforectomía derecha por vía laparoscópica, cuya biopsia demostró un tumor de células de Leydig. Un mes después de la cirugía, se normalizan todos los niveles androgénicos por lo que se inició la suspensión gradual de los corticoides. Conclusión: Aunque la HSRNC es la causa patológica más frecuente de la pubarquia precoz, cuando se asocia con un hiperandrogenismo clínico y bioquímico progresivo a pesar de un tratamiento adecuado y sin variantes patógenicas en el gen CYP21A2, incluso con niveles elevados de 17OHP, otras causas deben ser consideradas, específicamente tumores productores de andrógenos.

Hair manifestations of endocrine diseases: A brief review

Hair disorders are common in clinical practice and depending upon social and ethnic norms, it can cause significant psychosocial distress. Hair growth, cycling and density are regulated by many endogenous factors, mainly circulating hormones. Thus, diseases affecting the endocrine system can cause varied changes in physiological hair growth and cycling. Diagnosis and treatment of these disorders require a multidisciplinary approach involving a dermatologist, gynecologist and an endocrinologist. In this review, we briefly discuss the influence of hormones on the hair cycle and hair changes in various endocrine disorders.