Pubic Hair Distribution Research Articles

Mayer-Rokitansky-Küster-Hauser syndrome

Mayer–Rokitansky–Küster–Hauser syndrome (MRKH) is a syndrome characterized by uterine, cervix, and the two third of upper vagina aplasia which is the cause of incomplete development of the Müllerian duct. Reported a case of woman 28 years old, patient has not menstruated until now. The phenotype of the patient appears to be female, and with normal stature. Breast, axilla dan pubic hair distribution, fatty in buttocks and thigh developed normally. Fallopian tubes, uterine and 2/3 upper part of vagina were not formed. On gynecological clinical examination, found vaginal introitus with a vaginal sonde was 2 cm. On abdominal ultrasound examination, the uterus was seen as a line, right ovary measuring 2.93 x 2.59 cm and left ovary measuring 2.52 x 2.28 cm. The patient then underwent a diagnostic laparoscopic procedure, and found both right and left ovaries within normal limits, intact tubes, but no uterus was visible. Patient are planned for vaginoplasty.

Herlyn-Werner-Wunderlich (HWW) syndrome: a case report

Herlyn-Werner-Wunderlich (HWW) syndrome OHVIRA syndrome (Obstructed HemiVagina Ipsilateral Renal Agenesis) is rare type of Mullerian duct anomaly, with uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Usually presenting in post-pubertal adolescent girls presenting as pain abdomen or pelvic masses on the side of the obstructed Hemivagina. Here we represent a case report of a 14-year-old girl who presented to our outpatient department complaining lower abdominal pain for 2 months with previous normal menstrual cycles. On General Physical examination well-developed breasts and normal axillary and pubic hair distribution were noted. The Baseline laboratory analysis and hormonal profile were reported normal. Magnetic Resonance Imaging (MRI) provided a fruitful help in correct diagnosis of this rare anomaly. The treatment modality being the surgery to relieve obstruction and its associated complications.

Novel compound heterozygous LoF mutations in SRD5A2 may result in disorders of sex development

Objective: To investigate the novel genetic cause associated with hypospadias and the strategy for preventing offspring genetic defects in these patients. Methods: In March 2019, a patient with gonadal dysplasia (hypospadias associated with cryptorchidism) was referred to Shanghai General Hospital. His secondary sex characters, level of sex hormones and the development of male reproductive system was assessed through physical examination, sex hormone examination, male reproductive system B-ultrasound and computed tomography (CT). Whole-exome sequencing (WES) was preformed to investigate the pathogenic genetic variations associated with hypospadias and cryptorchidism. Also, Sanger sequencing was conducted to verify the WES results in the pedigree. Semen analysis was used to assess the fertility of the proband and the SRD5A2 gene analysis of his spouse was performed to assess the risk of genetic defects in the offspring. Results: The patient suffered from gonadal dysplasia (hypospadias associated with cryptorchidism). Physical examination showed an inverted triangular distribution of pubic hair, small penis and the volume of the testis was 8 ml. Sex hormone examination revealed the level of FSH, LH, Pituitary prolactin (PRL), estrogen (E(2)), testosterone (T), and sex hormone-binding globulin (SHBG) was 25.81 U/L, 10.84 U/L, 21.09 μg/L, 153 pmol/L, 16.95 nmol/L, and 36.15 nmol/L respectively. B-ultrasound and computed tomography (CT) showed left inguinal testis. Also, semen analysis illustrated that the volume was 0.05 ml and sperm concentration<2×10(6)/ml, suggesting oligospermia in this case. WES sequencing and Sanger sequencing showed compound heterozygous LoF mutations in SRD5A2 [NM_000348.3:C.679C>T(p.Arg227Ter) and NM_000348.3:C.16C>T(p.Gln6Ter)] in this patient. And there were no pathogenic genetic variations of SRD5A2 in the spouse. Conclusion: Novel compound heterozygous LoF mutations in SRD5A2[NM_000348.3:C.679C>T(p.Arg227Ter) and NM_000348.3:C.16C>T(p.Gln6Ter)] may be the primary cause of disorders of sex development.

Neglected congenital adrenal hyperplasia presenting as recurrent abdominal pain

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder with the incidence of the classic type being 1:15,000 births worldwide. It results from defective synthesis of steroid hormones because of deficiency of one of the five enzymes required for synthesis of cortisol. Cases of severe forms of CAH are often missed in developing countries where there is no program for newborn screening with many of them dying in neonatal periods. Management of the mild forms are also hindered or delayed because of superstitions, ignorance and poverty. We present an 11years old girl who presented with abnormal genital since birth and recurrent monthly lower abdominal pain of eight month duration. She had Tanner stage 4 breasts, normal female pubic hair distribution, clitoromegaly, fused scrotalized labia with urogenital sinus and absent vaginal opening. Abdominal ultrasound showed normal female reproductive organs; she had advanced bone age of 16years and elevated serum 17-hydroxyprogesterone level. She’s being worked up for possible genitoplasty.

Testicular atrophy and its clinical manifestations in a patient released from leprosy treatment

Leprosy is a disease that results in various organ impairments, including testicular atrophy. Testicular damage in leprosy causes gynaecomastia, decreased libido, erectile dysfunction (impotence), altered distribution of pubic hair, and infertility. Until now, testicular screening in leprosy patients has not been given special attention by health workers, even by specialists. The aim of this report is to emphasize the importance of testicular screening in leprosy patients.

Effect of the surgical treatment on the keloid of mons pubis with postoperative radiotherapy

Objective To explore an effective surgical treatment to the keloid of mons pubis. Methods From March 2008 to January 2015, local flap and adjacent flap (including ilioinguinal flap and rectus abdominal musculocutaneous flap) were performed, and used to repair the defect after surgical removal of the keloid in 25 patients, and all the patients were received postoperative local radiotherapy, and then chi square test was applied to evaluate the effect in the six aspects, including perineal form, skin color, pubic hair distribution, satisfactory effect, skin texture and sense. Results The local flap group was better than the adjacent flap group in perineal form, skin color, pubic hair distribution, satisfactory effect, but there was no significant difference in skin texture and sense. Conclusions For the keloid of mons pubis, the first choice is local flap graft, which can maintain the normal mons pubis formation as most as possible, while the wound is so large that the local flap cannot work, and therefore the vascular pedicle axial flap should be used. Key words: Flap; Flap graft; Keloid; Mons pubis; Radiotherapy

A young male adolescent with feminine appearance: diagnosis of 46, XX syndrome neglected for 4 years with gynaecomastia presentation

Gynaecomastia is common in infancy and adolescent boys, but other inciting causes should be kept in mind and necessitate further evaluation should be conducted to determine any underlying conditions. A 22-year-old unmarried male adolescent visited our endocrinology clinic for feminine appearance despite operations for bilateral gynaecomastia 4 years ago. Physical examination showed inverted triangular distribution of pubic hair, sparse beard, small-sized testes, flaccid short penis and surgical scar of the chest wall. Serum hormones study revealed primary hypergonadotropic hypogonadism, and cytogenetic study disclosed female complement (46, XX). The authors recommend that sexual chromosome abnormality should be considered in patients with hypogonadism to avert androgen deficiency-related complications early and that long-term team care should be provided to improve the patient's health-related quality of life.

Effect of hair autotransplantation with intact follicle on congenital loss of pubic hair

Objective To explore the effect of autotransplantation of hair with intact follicle on pubic hair reconstruction in patients with congenital loss of pubic hair. Methods The scalps strips of 12 female patients were harvested from the back of the head, close to the hairline. Under the microscope, the strip was then divided into a series of follicular-unit micrografts. The hair was transplanted to defective pubic area with the needle of syringe (0.9 mm × 38. 0 mm). Four hundred to 700 follicle units (U) were transplanted for small areas with the shape of inverted triangle or diamond. The density of transplanted hair was 20 U/cm2 in the area of mons pubis. The further from the area of mons pubis the distance was, the lower the density was. But it was not less than 10 U/cm2. Results The patients were followed-up for nine months to two years. The hairs transplanted grew well. The appearance was close to the normal distribution of pubic hair. All of the patients were treated by this one-session operation, and satisfied with the results. The incision scar of donor area on the back of the head was inconspicuous. Conclusions The above-mentioned technique is a simple, safe and effective method for pubic hair reconstruction. It might be an ideal method for pubic hair reconstruction with the appearance much closer to a normal pubic hair. Key words: Hair; Transplantation; Pubic hair; Hair follicle

Index of Suspicion

Index of Suspicion

Relationship of pubertal gynecomastia with varicocele and various parameters of growth: a seven year prospective study

Gynecomastia is a complex process resulting from many hormonal changes. It is related to either actual or relative decrease in testosterone-to-estrogen ratio. Varicocele is known to be associated with a progressive decline in testicular function. Hence we tried to study their relationship in otherwise clinically healthy males. Furthermore, we analyzed the relationship of gynecomastia with various physical changes occurring during pubertal development of boys so as to explore its pathophysiology and predict its onset or disappearance. Prospective population based study. 131 clinically healthy boys aged 6–8 were followed for 7 years by visiting 3 schools once every year and twice a year as they near their puberty. Children were examined for the presence of gynecomastia (≥1 cm of palpable button of firm subareolar breast tissue) and varicocele (by examining the boys in standing position with help of Valsalva maneuver when appropriate). Remaining clinical examination included height (Siber Hegner anthropometer), weight (beam balance), testicular volumes (Prader orchidometer), and Tanner stage according to pubic hair distribution. Logistic regression analysis was used to determine the risk for developing gynecomastia in those who had varicocele as compared to those without it. Prevalence of gynecomastia and varicocele in our study population was 15.2% (20 of 131) and 22.1% (29 of 131) respectively. Bilateral gynecomastia (13 of 131) was more common than either right (5 of 131) or left sided (4 of 131). In 2 boys, unilateral gynecomastia subsequently became bilateral. Gynecomastia was more frequent in Tanner stages 3 and 4. In 13 boys, gynecomastia subsided on follow up. Mean ± standard deviation of age (years), height (cm), weight (kg), left and right testicular volumes (mL) at which gynecomastia was seen to appear were 12.27 ± 0.86, 157.6 ± 7.3, 46.0 ± 6.7, 8.1 ± 2.9 and 9.3 ± 4.0 respectively. Corresponding values for disappearance of gynecomastia were 13.0 ± 0.86, 164.5 ± 5.6, 48.62 ± 5.8, 12.5 ± 4.2, 13.5 ± 4.0 respectively. In 11 year olds, when gynecomastia was seen for the first time, it was positively correlated with varicocele (odds ratio = 3.039, 95% confidence interval = 1.215 – 7.598). However no significant correlation was seen in subsequent years which may be due to the transient nature of gynecomastia. Adolescent gynecomastia is a transient mid-puberty event. Gynecomastia is more likely to develop in young boys having varicocele.

Anthropometry and body composition do not predict bioavailable androgen or progesterone concentration in adolescent girls

Our objective was to test the hypothesis that anthropometry and body composition were independently associated with sex-steroid concentration in adolescent girls. Premenarcheal (age, mean +/- SD = 10.9 +/- 0.6, N = 51), perimenarcheal (age = 13.7 +/- 0.6, N = 74), and postmenarcheal (age = 16.7 +/- 0.6, N = 44) cohorts provided saliva at morning recess during the schoolday over 6 weeks. Estradiol, testosterone, progesterone, and dehydroepiandrosterone (DHEA) were quantified. Age, developmental cohort, self-reported Tanner stage of breast development and pubic-hair distribution, gynecological age, height, weight, waist/hip ratio, and percent body fat (by impedance) were assessed. Repeated measures were reduced to a mean. Data were hierarchically standardized for sexual maturation status, and then used to predict anthropometry/percent body fat. After intensive repeated sampling, individuals still varied widely in testosterone, progesterone, and DHEA concentrations within a developmental cohort (35.0 < or = coefficient of variation < or = 66.5%). Individual hormone concentration was repeatable across 2 years (0.51 < or = r(2) < or = 0.73). In spite of the variability within a cohort, there was no evidence for an association between the standardized hormone and body mass index, percent body fat, or waist/hip ratio, whether or not measures were age-adjusted (21 univariate tests; 0.19 < or = P < or = 0.96). Stratification by developmental cohort also did not reveal associations. Low estradiol assay precision precluded analyses. In conclusion, despite considerable population variation in hormone concentration after repeated sampling, no evidence supported an association between anthropometry or pecent body fat and testosterone, progesterone, or DHEA in adolescent girls.

Adolescent Varicocele: Association with Somatometric Parameters

Introduction: The developmental changes that occur as a result of puberty have been hypothesized to be important causes of varicocele. Various somatometric parameters were known to affect the occurrence of varicocele during the growth period. We conducted this study in order to examine these relationships and to determine the incidence of varicocele in adolescent males. Patients and Methods: We evaluated 1,200 healthy males aged 0–19 years for varicocele and correlated it with the following somatometric parameters: age, height, body mass index (BMI), pubic hair distribution, penile length and testicular volume. Results: Adolescent varicocele was found in 5.6% of the participants. The 13- to 19-year age-group had the highest incidence of varicocele (10.5%). Logistic regression analysis showed that the incidence was positively correlated with age, height and penile length (odds ratio 1.61, 1.04 and 1.37, respectively) and negatively correlated with left testicular volume, BMI and pubic hair distribution (odds ratio 0.87, 0.87 and 0.47, respectively).Conclusion: Varicocele was more prevalent in tall boys with a lower BMI, who had quickly progressed through puberty. Our observations suggest that varicocele is associated with various somatometric parameters.

A new technique for repair of exstrophy-epispadias complex.

We report a new method of repair of exstrophy-epispadias complex (EEC) at a second stage by using flaps from the skin between the penis and scrotum (the clitoris and labia majora in females) used in seven patients. The non-pigmented skin between the penis and scrotum in males or a band of skin between the clitoris and labia majora in female is mobilized in two flaps, which are rotated superiorly. Five patients were treated primarily by this method and two patients at the time of secondary reconstruction. There were six boys and one girl. Two patients were post-pubertal, one male and one female. Follow-up (6-12 months) revealed good cosmetic and functional results. If EEC is repaired in a single stage, the entire pelvic diaphragm is visualized from inside and the sphincters can be reconstructed around the bladder neck. This procedure gives better exposure of attachments of the corporal bodies to the pubic rami, allowing easier mobilization. The bulbospongiosus muscle can be reconstructed. A normal penoscrotal relation is achieved in male patients. The root of the scrotum, which is splayed out and wide, narrows. Rotation of the flaps superiorly normalizes the symphyseal area. In female patients the appearance of the mons pubis is satisfactory. In post-pubertal children the pubic hair distribution appears normal.

A Case of 46,XX African American male

We report on a 16-year-old African American male with 46, XX karyotype. He was referred for evaluation because of gynecomastia. A pathology report from a surgically removed undescended testis at 2 years of age showed a left ovotestis. When we saw him, he had a tall thin habitus, female distribution of pubic hair, gynecomastia, and penile abnormality. Molecular methods using primers specific to YP11 locus failed to detect the presence of the SRY gene. Furthermore, using primers specific for the Y-linked and X-linked amelogenin loci showed the presence of only the X-linked amelogenin locus but not he Y-linked amelogenin locus in his genome Fluorescence in situ hybridization (FISH) indicated the presence of Y material on one of his X-chromosomes. The presence of SRY sequences and other Y chromosome genes in this patient's cell lines of specific tissues might probably be the reason for his phenotype. The findings in this patient suggest the need to use both DNA analysis and FISH methods in studying patients with sex-chromosome abnormalities presenting with unusual phenotypes.

TESTICULAR TERATOMA IN A MAN WITH XX/XXY MOSAIC KLINEFELTER'S SYNDROME

No AccessJournal of UrologyClinical Urology: Case Reports1 May 1999TESTICULAR TERATOMA IN A MAN WITH XX/XXY MOSAIC KLINEFELTER'S SYNDROME SHINGO MATSUKI, ISOJI SASAGAWA, HIROSHI KAKIZAKI, YASUHIRO SUZUKI, and TERUHIRO NAKADA SHINGO MATSUKISHINGO MATSUKI More articles by this author , ISOJI SASAGAWAISOJI SASAGAWA More articles by this author , HIROSHI KAKIZAKIHIROSHI KAKIZAKI More articles by this author , YASUHIRO SUZUKIYASUHIRO SUZUKI More articles by this author , and TERUHIRO NAKADATERUHIRO NAKADA More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(05)68964-1AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "TESTICULAR TERATOMA IN A MAN WITH XX/XXY MOSAIC KLINEFELTER'S SYNDROME." The Journal of Urology, 161(5), pp. 1573–1574 References 1 : Choriocarcinoma, thyrotoxicosis, and the Klinefelter syndrome. Cancer Genet. Cytogenet.1983; 9: 1. Google Scholar 2 : Bilateral testicular teratoma in Klinefelter's syndrome. Brit. J. Urol.1993; 72: 384. Google Scholar 3 : Simian papovavirus 40 transformation of cells from cancer patient with XY-XXY mosaic Klinefelter's syndrome. Cancer1970; 30: 1769. Google Scholar From the Department of Urology, Prefectural Nihonkai Hospital and Yamagata University School of Medicine, Yamagata, Japan© 1999 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 161Issue 5May 1999Page: 1573-1574 Advertisement Copyright & Permissions© 1999 by American Urological Association, Inc.MetricsAuthor Information SHINGO MATSUKI More articles by this author ISOJI SASAGAWA More articles by this author HIROSHI KAKIZAKI More articles by this author YASUHIRO SUZUKI More articles by this author TERUHIRO NAKADA More articles by this author Expand All Advertisement PDF downloadLoading ...

A 47, XXY female

Klinefelter syndrome (47,XXY) with male phenotype is the most common sex chromosomal abnormality. It is believed that SRY (sex determining region on Y) is the major gene necessary to induce the undifferentiated bipotential gonadal primordium to develop as testis. Recently, several SRY-boxrelated (SOX) genes have been identified on autosomes. Mutations in the SRY or SOX gene have been implicated in sex reversal. We report an individual having 47,XXY chromosome constitution, normal SRY, SOX9, and ZFY genes and yet with a female phenotype. A 15-year-old phenotypic female has well developed breasts; prominent labioscrotal folds; an enlarged clitoris; feminine pubic hair distribution; urethra in the centre of the vestibule; a urogenital sinus 5 cm deep; a uterus; prepubertal in shape and size, palpable per rectum; and a cervix which could not be palpated. Ultrasonography of the abdomen revealed normal shape of urinary bladder with smooth and regular walls. Uterus was small in size, with normal shape and outline. Myometrium was homologous, cavity was empty, midline echo was normal. Laparoscopic assessment revealed a normal uterus. Right ovary was normal in size and shape with a cyst and a normal Fallopian tube. Left ovary was not seen as it was hidden beneath an epithelial fold. Chromosome analysis showed 47,XXY karyotype in all 100 metaphases analysed. Hormone profiles showed average female range of corticotrophin, T3, T4, thyrotrophin, and testosterone. Progesterone and prolactin were higher when compared with normal female levels. Histology of the right gonad showed features compatible with ovarian stroma. No RESEARCH LETTERS

Sex differences in adult pubic hair distribution in Nigeria

Pubic hair was studied in 520 men and 270 women belonging to the various parts of the Northern Nigeria. An attempt has been made to study the pubic hair pattern in three age groups. The distribution of pubic hair was classified into four types, designated as horizontal, sagittal, acuminate and disperse. The acuminate or "masculine" type is present in approximately half of the men and 10 percent of the women. It is characteristic of 39.8 per cent of 19-20 years old males, and persists in 20.2 per cent of adult males. In the majority the division into "masculine" and "feminine" types is found to be satisfactory. There is definite relationship between the age of an individual and hair pattern. In males the decrease in frequency of the horizontal pattern with age is associated with increase in general hirsutism and is associated by the development of a disperse pubic hair pattern.

Onset of the release of spermatozoa (spermarche) in boys in relation to age, testicular growth, pubic hair, and height.

The onset of production of spermatozoa (spermarche) is the basis for achievement of reproductive capacity in men. We collected 24-h urine samples every 3 months in a 7-yr longitudinal study of 40 normal boys initially aged 8.6-11.7 yr. After centrifugation, the urine was analyzed for the presence of spermatozoa by microscopic examination, and spermarche was estimated on the basis of age at first observed spermaturia. The results were corrected for the intermittent occurrence of spermatozoa in the urine after first observed spermaturia and the fact that the urine samples were collected quarterly. In addition, physical examination, including determination of testicular size by orchidometer measurement, pubic hair distribution (Tanner stage), and height, was carried out every 6 months. Spermarche occurred at a median age of 13.4 yr (range, 11.7-15.3 yr), at a time when testicular size was 4.7-19.6 ml (median, 11.5 ml), and pubic hair distribution was 1-5 (median, 2.5). In most boys, spermarche preceded the age of peak height velocity (median, 13.8 yr; range, 12.2-15.2 yr); at the time of spermarche, median peak height growth velocity was 9.9 cm/yr (range, 7.5-13.4 cm/yr), and median height was 160.4 cm (range, 151.7-175.9 cm). We conclude that spermarche is an early pubertal event and that a wide variation in testicular size and secondary sex characteristics is found at that time. In particular, spermarche may occur when little or no pubic hair has developed, and the testes have grown only slightly.

MASSIVE PREGNANETRIOL EXCRETION DUE TO VIRILIZING ADRENAL TUMOUR

Urinary 17-oxosteroid values in excess of 100 mg./24 hr. are not uncommonly associated with virilizing tumours of adrenal origin. However, the secretion of pregnanetriol in amounts greater than 50 mg./24 hr. is unusual. The patient reported here exhibited the highest level of pregnanetriol excretion due to adrenal tumour without metastases ever recorded in the English literature. A 47-year-old Negro woman presented with a 10-month history of hirsutism, darkening of the facial skin, periorbital oedema, abdominal swelling, and right flank pain, progressively severe and debilitating. Twenty years previously, total abdominal hysterectomy and bilateral salpingo-oophorectomy had been accomplished for pelvic inflammatory disease. Physical examination revealed a round-faced hirsute Negress in no distress with blood pressure of 150/105 mm. Hg, atrophic breasts, masculine abdominal and pubic hair distribution and patchy hyperpigmentation of the face and arms. The liver was palpable 4 cm. below the right costal margin and was only slightly tender. The

Inhibitory effects of portal cirrhosis of liver on prostatic enlargement.

BENIGN prostatic hypertrophy is a very common disease in men over 50 years of age, 1 but little is known of the precise cause of this disorder. Various theories have been promulgated, but most of them have been untenable. Today most authorities believe that the cause may be hormonal imbalance. There is abundant evidence that cirrhosis of the liver is often accompanied by hormonal abnormalities. Testicular atrophy, loss of body hair, change in pubic hair distribution, loss of libido, and, at times, gynecomastia have been described in cases of hepatic cirrhosis. 2 It is thought by many that these are secondary changes, caused by the inability of the damaged liver to metabolize estrogenic hormones properly. 3 Whether the derangement of hepatic physiology involves other hormones is not clearly established, but various investigators believe that factors such as pituitary, adrenal, and testicular function deserve consideration. 4 No direct evidence has been presented to suggest that