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Lichen sclerosus: early diagnosis is the key to treatment.

Lichen sclerosus of the vulvar is an often misdiagnosed and chronic gynecologic disease. Women of any age can be affected, but it is most common in the perimenopausal and postmenopausal years. The treatment for lichen sclerosus is limited with no cure. The current treatment is a lifelong procedure and may not prevent the progression of the disease. The primary treatment for lichen sclerosus is testosterone ointment, which may cause unwanted side effects. Complications arising from vulvar lichen sclerosus include loss of vulvar structure and fusing of labia which causes covering of the clitoris. Vulvar lichen sclerosus may also cause painful intercourse and defecation. Due to the intense pruritus, excoriation of the area occurs. This information can help the clinician to detect the disease early and start treatment before serious complications begin. Diagnostic and treatment information is provided in this article to help the clinician understand the nature of this disease.

Biopsy↗

[The clinical picture of the fetal alcohol syndrome].

Six new cases of alcoholic embryopathy on newborn children are described. We found out that the mother suffers from chronicle alcoholism. The diagnosis fetal alcohol syndrome is made because there are the minimal diagnostically syndromes: prenatalis hypotrophia, microcephalia, mental retardation, face dysmorphism, cardinal anomalies. We have observed an unfolded clinical picture with added hypertrophia on the clitoris and anomalies of the kidneys on one of the children. With the description of the cases we direct obstetrician's and neonatologist's attention to this current in this moment medical problem.

Female↗

Genital mutilation of young girls traditionally practiced in militarily significant regions of the world.

Very few physicians practicing in the United States have experience in treating female patients who have undergone mutilation of the external genitalia, incorrectly termed female circumcision. This procedure, known as infibulation, consists of removing the clitoris, prepuce, and portions of the labia of young girls, usually younger than 7 years of age. Infibulation has been practiced by lay midwives for centuries in the Horn of Africa and in other African and Middle Eastern countries. This paper discusses infibulation, the techniques, and the recommended medical and obstetric management of patients subjected to genital mutilation. With increased immigration to the United States by Africans and Middle Easterners, and with readily increasing military medical deployments, primary care physicians and specialists can expect to be confronted with patients who have undergone this disfiguring procedure during their youth.

Adolescent↗

[Surgical correction of genital virilization in girls with congenital adrenal hyperplasia].

Based on an analysis of 10 patients after a feminizing operation of a virilized genital in female pseudohermaphroditism caused by congenital adrenal hyperplasia on account of 21-hydroxylase deficiency the authors emphasize in conjunction with multidisciplinary collaboration the necessity of early modification and surgical repair with adjustment of the external genital before the age of two years. An excellent anatomical and cosmetic result of reconstruction with clitoroplasty emphasizes the importance of preserving the clitoris and erectile tissue to ensure adequate social, psychic and sexual development of the patient.

Adrenal Hyperplasia, Congenital↗

[Failure of milk ejection reflex in primiparous cows from the differential diagnostic and therapeutic aspects].

A particular failure of the milk-ejection reflex in dairy cows is described on the base of own experiences in farms and reports from literature. Four cases are reported more in detail. Quantity and fat content of cisternal, alveolar and residual milk fractions, oxytocin content in blood serum, milk-ejection after manual teat stimulation, after massage of the clitoris or rectal stimulation of the vagina and cervix were investigated. This failure of the milk-ejection is observed mostly in primiparous cows and always at the begin of the lactation period. It is complete and permanent unless successfully treated, and seems to be based on an central inhibition of the oxytocin release. Treatment consists of thorough udder stimulation and milking followed by an i.v. application of oxytocin in order to remove the residual milk portion. The condition has a good prognosis, if the treatment is carried out regularly for some days and even weeks. Acute Mastitis is the main differential diagnosis.

Animals↗

Lichen sclerosus. Therapy with clobetasol propionate.

OBJECTIVE: To assess the efficacy of treating lichen sclerosus with clobetasol propionate. STUDY DESIGN: A retrospective chart review of 81 consecutive symptomatic private practice and clinic patients with biopsy-proven lichen sclerosus were included. All subjects' punch biopsies, baseline histories and physical examinations were reviewed by the same examiner. Each subject's symptomatology and responses to previous treatment modalities were recorded. A standard regimen of 0.05% clobetasol propionate cream was initiated. Subjects were reevaluated at three months and asked to rate the improvement of symptoms. Follow-up examinations were conducted 6-12 months later on 36 subjects. Descriptive statistics and chi 2 analyses were performed. RESULTS: The mean age of subjects was 54 +/- 15.5 years (range, 15-86), and the average duration of treatment prior to clobetasol use was 6 +/- 6.9 years (range, 0.5-29). Twenty-seven subjects did not complete the study or were lost to follow-up. The average subject had tried 2.25 treatment modalities (range, 1-13). The most common symptoms were pruritus (98%) and irritation (61%), with complaints of burning and dyspareunia. Most subjects (76%) had labial involvement, with concomitant involvement of the clitoris (70%), perineum (68%) and perianus (32%). The majority (88%) of subjects had a primary lesion of white and crinkled tissue. With clobetasol, 77% of subjects had complete remission of symptoms, 18% had partial remission and 5% reported no change. A change in clinical appearance was noted for the complete-remission (32%) and partial-remission groups (46%). Twenty-two percent revealed no change. CONCLUSION: Clobetasol propionate cream is recommended for treatment of lichen sclerosus, with a 77% chance of complete remission of symptoms and a 47% chance of improvement in the clinical appearance of the vulva. Women may have to continue to use clobetasol as needed after finishing a base treatment course.

Administration, Topical↗

Sacral reflex latency correlated by age and sex.

This prospective study was undertaken on 190 subjects who had no prior central or peripheral nerve supply pathology and where the value of the sacral reflex latency was compared by age and sex in order to determine precise norms. There were 90 men whose age averaged 56.9 +/- 13 years and 100 women with an average age of 54 +/- 13.1. The reflex was obtained by stimulating the dorsal nerve of the penis and the clitoris and taking a reading in the intermediate part of the external anal sphincter muscle. An averaging technique was used (over 30 stimulation occurrences). The average intensity of the stimulation was 15 mA. The value of the stimulus reaction latency taken at the positive peak was analyzed statistically. The average value was 37.6 +/- 5.36 ms in male subjects and 46.6 +/- 8.08 in female subjects. In men, values varied from 36.6 +/- 4.26 ms at under 35 years of age and reached 42 +/- 3.52 ms at over 75 years. In women, values varied from 37.33 +/- 7.69 ms at under 35 years to 55.6 +/- 4.39 ms at over 75 years of age. In a previous article we discussed ageing features in nerves and muscles. These features are largely responsible for the occurrence of static pelvic problems. The purpose of this study is to compare the sacral reflex latency by sex and age in normal subjects.

Adult↗

Skinning vulvectomy for the treatment of vulvar intraepithelial neoplasia 2-3: a study of 21 cases.

Twenty-one cases of patients with vulvar intraepithelial neoplasia (VIN) 2-3 were reviewed. The mean age at diagnosis was 45.4 years. All of the patients presented with vulvar pruritus. Five of the patients had hypertension, two had coronary heart disease and two had diabetes mellitus as complicating medical illnesses. None of the patients had history or evidence of vaginal intraepithelial neoplasia (VAIN) or cervical intraepithelial neoplasia (CIN), and only one patient had invasive cervical cancer at diagnosis. Provided the histology confirmed VIN, the patients were subjected to a skinning vulvectomy procedure. Of the patients, 15 (71.4%) had VIN 2, and the remaining 6 (28.6%) had VIN 3 at preoperative evaluation. Histologic analysis of skinning vulvectomy specimens revealed no evidence of neoplasia in three patients (14.2%). Multifocality was observed in only three patients (14.2%). The areas involved were the perineum in four patients, labia in 15 and clitoris in two patients. Associated vulvar pathologies were condyloma acuminata in one, squamous vulvar hyperplasia in three and lichen sclerosus with squamous hyperplasia in one patient. The complications of the procedure included febrile morbidity in three patients and minor wound break-down in one patient. None of the patients in this series experienced recurrence. Skinning vulvectomy seems to have a high success rate in treatment of VIN 2-3 with minimal postoperative complications and satisfactory cosmetic results. However, observation of only three patients with multifocal lesions as well as no patient with invasive cancer adds credence to an ablative procedure after appropriate evaluation under colposcopy.

Adult↗

Genitourinary anomalies in the CHARGE association.

PURPOSE: We identified the incidence and types of genital and urinary anomalies, and established a plan for evaluating the urinary system in the CHARGE association. MATERIALS AND METHODS: We retrospectively reviewed the charts of 32 patients in whom the CHARGE association was diagnosed. RESULTS: Of the 32 patients identified 22 (69%) had genitourinary abnormalities. Genital anomalies, including micropenis, penile agenesis, hypospadias, chordee, cryptorchidism, a bifid scrotum, atresia of the uterus, cervix and vagina, and hypoplastic labia majora, labia minora and clitoris, were present in 18 patients (56%). Of the 24 patients who underwent renal ultrasound 10 (42%) were diagnosed with urinary tract anomalies including a solitary kidney, hydronephrosis, renal hypoplasia and duplex kidneys. Further evaluation revealed vesicoureteral reflux, neurogenic bladder secondary to spinal dysraphism, nephrolithiasis, ureteropelvic junction obstruction and a nonfunctioning upper pole in both duplex kidneys. CONCLUSIONS: There is a high incidence of genitourinary anomalies in the CHARGE association. Because of this high incidence of anomalies, patients with this condition should undergo a careful genitourinary evaluation, including renal and bladder ultrasound, and voiding cystourethrography screening.

Abnormalities, Multiple↗