scholarly journals SUN-004 Case Study: A 17-year-old Girl with HAIR-AN Syndrome, T2DM and Primary Amenorrhea

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Abdulghani Habib Alsaeed ◽  
Hayat Al Harthi

Abstract Introduction: HAIR-AN syndrome is a sub-type of polycystic ovarian syndrome which consists of virilization, insulin resistance and acanthosis nigricans. It may be associated with menstrual irregularity, and hyperandrogenic features such as hirsutism and masculinization. Aim: We report an adolescent girl with HAIR-AN syndrome, T2DM, a very high testosterone level (male range) and primary amenorrhea Case report: A 17-year-old female who had been investigated by pediatric endocrinology since the age of 13 because of hyperpigmentation which was proven to be acanthosis nigricans on skin biopsy. The patient was found to have insulin resistance with initially normal glucose level. She has hirsutism and hypertrichosis. There is no similar condition in her family, she has three siblings all are well. She developed T2DM with at the age of 14. She was started on Metformin 2 gram daily and them pioglitazone 30 mg was added when she was 16 years. She never had menarche. The clinical examination revealed an adolescent girl with normal BP 106/68 mmHg, and BMI 19.6kg/m2. She scored 24 onFerriman-Gallwey hirsutism scoring system. She had severe acanthosis nigricans on both axillae. She also had back and upper limbs hyperpigmentation.Lab tests revealed normal thyroid function tests, prolactin, cortisol, DHEA-S, and 17 hydroxy progesterone. Fasting glucose 7.2, insulin 123 μU/ml (2.6-24.9), c-peptide 964, HbA1c 8.2%. Total testosterone 24.61 nmol/L (0.069-2.715), SHBG 184.9 nmol/L, and Free testosterone index 13.31 (0.51-6.53). Her LH 8.9 and FSH 4.7.Radiological investigations revealed polycystic ovaries on pelvic ultrasound. MRI abdomen showed normal adrenals, and mildly enlarged ovaries with peripherally located follicles consistent with polycystic ovarian syndrome. The patient was started in Diane-35 (cyproterone acetate and ethinyl estradiol) oral pills. She started to have menarche three months after using Diane-35. Her Total testosterone had dropped from 24.61 to 1.69 nmol/L (0.069-2.715), SHBG 579 nmol/L, and Free testosterone index 0.29 (0.51-6.53). She reported that the hirsutism is getting less than before starting the treatment.Conclusion: Primary amenorrhea might be a manifestation of in HAIR-AN syndrome due to sever hyperandrogenism. The management of such condition is challenging. In addition to controlling the metabolic parameters, combined oral pills with antiandrogen effect might be effective.

Author(s):  
Sukhleen Kaur ◽  
Sunil K. Gupta ◽  
Sunil K. Juneja ◽  
Sukhjot Kaur ◽  
Monika Rani

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.


Author(s):  
Jin Hui Ho ◽  
Ana Vetriana Abd Wahab ◽  
Yin Khet Fung ◽  
Serena Sert Kim Khoo

Summary Polycystic ovarian syndrome (PCOS) is associated with menstrual irregularities, ovulatory dysfunction, hirsutism, insulin resistance, obesity and metabolic syndrome but is rarely associated with severe hyperandrogenaemia and virilisation resulting in male pattern baldness and clitoromegaly. Total serum testosterone greater than twice the upper limit of the reference range or free androgen index of over five-fold elevated suggests a diagnosis other than PCOS. We reported a case of a 15 years old obese girl presented with secondary amenorrhoea, virilising signs: frontal baldness, clitoromegaly and prominent signs of insulin resistance and marked acanthosis nigricans. Her total testosterone level was markedly elevated at 9.4 nmol/L (0.5–1.7 nmol/L) and MRI pelvis revealed a right ovarian mass with fat and cystic component and a left polycystic ovary. The patient underwent laparoscopic right ovarian cystectomy and histologically confirmed mature cystic teratoma. Post-operatively, her testosterone level declined but did not normalise, menses resumed but remained irregular. Her fasting insulin was elevated 85.2 mIU/L (3–25 mIU/L) and HOMA-IR was high at 13.1 (>2) with persistent acanthosis nigricans suggesting co-existing HAIR-AN syndrome, an extreme phenotype of polycystic ovarian syndrome. Learning points Rapid onset of hyperandrogenic symptoms, especially if associated with signs of virilisation must raise the suspicion of an androgen-secreting tumour. Total serum testosterone greater than twofold the upper limit of the reference range or free androgen indices over fivefold suggest a diagnosis other than polycystic ovarian syndrome (PCOS). High levels of testosterone with normal levels of the DHEA-S suggest an ovarian source. Ovarian androgen-secreting tumour and HAIR-AN syndrome, an extreme spectrum of PCOS can co-exist.


QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
M R Halawa ◽  
R S Abdelbaky ◽  
Y M Eid ◽  
M S Nasr ◽  
L M Hendawy ◽  
...  

Abstract Background study of chemerin level in polycystic ovarian syndrome (PCO) patients and its relation to insulin resistance (IR). Upon chemerin on adipose tissue and glucose metabolism, serum chemerin has been recently studied in (PCO) women Aim We aimed to study the level of serum chemerin in PCO patients and its relation to insulin resistance. Methods The current study included 45 subjects with PCO syndrome and 45 healthy subjects as a control group. PCO subjects were divided into 27 obese PCO and 18 lean PCO. Control women were divided into 25 obese women and 20 lean women. Measurement of serum chemerin levels, fasting blood glucose (FBG),fasting insulin (FIN), total testosterone and pelvic ultrasonography Results Serum chemerin was significantly higher in the obese PCOS group (99.65 ± 13.72 ng/mL) compared with lean PCOS (87.99 ± 5,64 ng/mL) and the obese (76.82 ± 2.39 ng/mL) and non-obese (69.19 ± 8.40 ng/mL) control groups. In PCOS women, serum chemerin levels were positively correlated with Body mass index (BMI) (r = 0.835, P &lt; 0.001), Fasting blood glucose (FBG) (r = 0.493, P &lt; 0.005), Fasting insulin (FIN) (r = 0.913, P &lt; 0.001), Homeostasis model assessment of insulin resistance (HOMA-IR) (r = 0.9181, P &lt; 0.001). Conclusion There is an increase in serum chemerin level in PCOS patients with even more significant increase in patients with obese PCOS.


Author(s):  
Madhusmita S. Nayak ◽  
Deepa D. Kala ◽  
Amit Agarwal

Background: Polycystic ovarian syndrome (PCOS) is a common endocrine disorder treated with combined oral contraceptives (COCs). The androgenic potential of any type of COC is in part determined by the progestin present. A newer form of progestin, drospirenone, has been recently introduced, and is available in combination with ethinyl estradiol.Methods: The study was conducted on 60 patients fulfilling inclusion criteria, in the department of obstetrics and gynecology, Terna Medical College and Hospital. Patients were diagnosed as PCOS according to Rotterdam criteria. After initial evaluation, blood samples were sent for levels of serum luteinizing hormone (LH), follicle stimulating hormone (FSH), dehydroepiandrosterone-sulphate (DHEA-S), free testosterone, sex hormone binding globulin (SHBG). Each patient was advised to take a combination of EE (30 mcg) + drosperinone (3 mg), 1 tablet daily from the second day of her menstruation for 21 days then a 7 days gap and again for 21 days and so on cyclically for 3 cycles, then to repeat the tests as done at the beginning.  Hirsutism was assessed clinically using the Ferriman-Gallwey scale at initial visit and three months later.Results: We found a significant improvement in the Serum LH, FSH levels and a significant fall in free testosterone levels accompanied by a rise in SHBG levels. There is also overall improvement in the hirsutism scores.Conclusions: Drosperinone based COCs are a good and effective means of treatment of PCOS. Treatment duration of three months was found to be effective in our study.


2021 ◽  
Vol 8 (4) ◽  
pp. 231-236
Author(s):  
Didem Dereli Akdeniz ◽  
Candeğer Yılmaz

Objective: Polycystic ovarian syndrome (PCOS) was thought to be a gynecologic disorder and then accepted as a general endocrine and metabolic syndrome. The genetic component of PCOS seems to be very important in its etiology. Because of this reason there should be a male PCOS equivalent. Early androgenetic alopecia (EAGA) is a specific pattern of hair loss and it should start before age 30 years and it is claimed to be a male equivalent of PCOS in women. Materials and Methods: In this study we aimed to investigate the hormonal and metabolic parameters of men with EAGA and compare them with healthy age-matched controls. Thirty men with EAGA and 30 controls were screened for free testosterone, DHEAS, gonadotropins, 17OH progesterone, ACTH, fasting glucose, fasting insulin, homocysteine and metabolic profile. Homeostasis model assessment (HOMA) results were used for the marker of insulin sensitivity. Alopecia classification was made by using the scale of Hamilton with Norwood modification. Results: Patients with EAGA had higher free testosterone (25,12±3,05 vs 21,3±1,77), DHEAS (634,90±27,09 vs 578±17,82), LH (9,16±0,28 vs 5,13±0,40). The EAGA group had insulin resistance but the control group did not (HOMA results were 3,34±0,47 vs 1,43±0,3). The homocysteine levels of EAGA group were higher than controls (12,37±1,31 vs 9,33±2,12) which is another cardiovascular risk factor. The correlations that we found in our study among HOMA, serum androgen levels, homocysteine and alopecia scores were positive in EAGA patients. We didn’t find any correlations among those parameters in control group. Because of these findings men with EAGA can be considered as male synonym to PCOS syndrome. These young men should be followed for the same long time risk profile like PCOS women. Insulin resistance and its results like metabolic syndrome, diabetes and cardiovascular diseases are real risks but there may be even a risk for infertility. Conclusion: We aimed to investigate whether EAGA can be accepted as the male phenotype of PCOS and if they have elevated risk factors for chronic complications than their age and sex matched controls.


2021 ◽  
Vol 10 (16) ◽  
pp. 1140-1144
Author(s):  
Nandhini Logaprabhu ◽  
Sarmishta Murugesan

BACKGROUND We wanted to analyse the clinical profile of polycystic ovarian syndrome (PCOS) women with history, examination and ultrasonogram and correlate hirsutism with biochemical markers as free testosterone, dehydro-epiandrosterone sulphate (DHEAS), sex hormone binding globulin (SHBG), free testosterone, DHEAS, and SHBG. METHODS This study is a prospective observational study conducted from 2011 to 2013 in the Department of Obstetrics and Gynaecology, Shree Balaji Medical College and Hospital, Chrompet in patients attending Gynaecology OPD. 100 women visiting the OPD were taken as control and 100 women were taken for PCOS study. RESULTS Hyperandrogenism was studied and all the biochemical markers were significantly higher in polycystic ovarian syndrome patients than in controls (P < 0.0001). The highest AUC-ROC was found for bioavailable testosterone (0.852) followed by free androgen index (0.847) and free testosterone (0.837). Lower AUC-ROC was found for androstenedione, total testosterone and SHBG (0.706, 0.799 and 0.76, respectively). When free androgen index of 4.97 was taken as a cut off value, sensitivity was 71.4 % and specificity was 85.2 %. A cut off of 0.78 nmol / L for bioavailable testosterone had even higher sensitivity of 75.9 %, but slightly lower specificity of 83.3 %. Bioavailable testosterone and free androgen index correlated significantly (all P < 0.05) with DHEAS, LH / FSH ratio, androstenedione and total testosterone. In addition, bioavailable testosterone, free androgen index, and free testosterone correlated significantly with follicle count, ovarian volume, and hirsutism scores. CONCLUSIONS White women have about 20 % of excess of dehydro-epiandrosterone sulphate (DHEAS) and black women have 30 % excess of dehydro-epiandrosterone sulphate (DHEAS) in those having poly cystic ovaries patients. There is an age-associated decline in DHEAS levels which is similar in both control and poly cystic ovaries women, regardless of the race which was seen in this study. KEY WORDS Free Testosterone, Dehydro–Epiandrosterone Sulphate (DHEAS), Sex Hormone Binding Globulin (SHBG)


Author(s):  
Hiba Al-Zubeidi ◽  
Karen O. Klein

AbstractPolycystic ovarian syndrome (PCOS) is characterized by irregular menses, elevated androgens, and insulin resistance. Little information is published about the treatment of adolescent PCOS.The aim of this study was to evaluate metformin versus oral contraceptive pills (OCP) in treating adolescent PCOS. Twenty-two girls were randomized to either treatment for 6 months. The outcomes variables included body mass index (BMI) and free testosterone (FT).BMI decreased in all patients (metformin p=0.004, OCP p=0.045). FT decreased significantly only with OCP. Insulin resistance measures decreased in all patients but did not reach significance. The only significant difference in any of the variables between the two groups was number of menses. BMI and FT remained less than baseline for 3 months off treatment.Metformin and OCP have a positive effect on BMI, which persists after treatment is discontinued. FT decreased with both treatments, but only reached significance with OCP.


2020 ◽  
Vol 6 (4) ◽  
Author(s):  
Huda Basim Al-Lami ◽  
Fadhil Jawad Al-Tu’ma ◽  
Wasan Ghazi Al-Safi

Objectives: The current study aimed to investigate the role of of Anti-Müllerian hormone and other hormonal biomarkers and insulin resistance in pathogenesis of polycystic ovarian syndrome of Iraqi women with various ages and BMI. Method: The sublects include 50 cases of polycystic ovarian syndrome obtained from gynecological and obstetric teaching hospital, Kerbala health directorate / Kerbala – Iraq and another 50 apparently healthy women as a control group. with age ranged between (18-37) years during the period from Dec. 2019 to June, 2020 which was divided into two groups depending upon their age; first group with age (18-27) years and the second with age (28-37) years, also they divided into three subgroups depending upon the body mass index, normal weight (18.5-24.9 kg/m2), overweight (25-29.9 kg/m2), obesity (30-34.9 kg/m2) and  measurement of various hormonal levels were performed including LH, FSH, insulin, insulin resistance, total testosterone and anti-müllerian hormone. Results: A significant elevated levels of each of Anti-Müllerian hormone (AMH), insulin, LH, total testosterone, prolactin, fasting blood glucose, FBG and insulin resistance while, a non-significant differences was found in FSH levels in women with PCOS as compared with control group. Conclusion: A significant high levels of the all hormonal parameters including anti-müllerian hormone, LH, prolactin and total testosterone  was found in women with PCOS as compared with the control, except the FSH values.


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