Adolescent and pediatric gynecology最新文献

Background: Communicating double uterine anomalies are defined as müllerian defects which involve two hemiuteri with communication between the uterine halves. Nine subcategories of communicating uterine anomalies have been described; only two of these subcategories are associated with hemivaginal obstruction.

Case: An 11-year-old white female was brought to the Emergency Department with fever and acute pelvic pain. This condition was found to be caused by abscess formation behind an obstructed left hemivagina with involvement of a communicating double uterine anomaly.

Conclusion: To our knowledge, this is the first case report involving a communicating double uterine anomaly in which fever was a presenting symptom.

Study Objective: To develop a new technique for labioplasty with the goal of decreased scar formation, decreased vulva pain, and dyspareunia.

Design: We present a simple surgical approach to labial reduction in two young women. We performed a wedge resection of the excess labial tissue after first identifying and preserving the main arterial supply to each labia.

Results: After surgical correction, the patients had excellent cosmetic results with smooth symmetrical contours, minimal scarring, and relief from symptoms.

Conclusions: Hypertrophy of the labia minora is a frequently overlooked problem in young women. In general, gynecologists have been reluctant to surgically remove the excess tissue. We present a new approach to labioplasty that is safe and can be performed easily in symptomatic patients.

Study Objective: To correlate phenotype with genotype based on molecular diagnosis in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Design: Polymerase chain reaction amplification, restriction fragment length polymorphism, and allele specific oligonucleotide hybridization were utilized to ascertain eight previously recognized deleterious mutations in affected individuals and their family members.

Participants/Setting: DNA samples were obtained from 20 individuals with 21-hydroxylase deficiency followed through the Endocrine Clinic of the Children's Hospital of Pittsburgh. Fifteen individuals had salt-losing 21-hydroxylase deficiency and two families had two affected individuals. Four individuals presented prior to 5 years of age without salt loss. One individual presented in adolescence with hirsutism and oligomenorrhea.

Main Outcome Measure: Molecular diagnosis of 21-hydroxylase deficiency.

Results: Five patients have homozygous deletions of the promoter region. Two patients are homozygous for the splicing mutation in the second intron. Individual patients were homozygous for the following three mutations: Arg³⁵⁶→Trp, Ile¹⁷²→ Asn, and Val²⁸¹ → Leu. Ten patients were compound heterozygotes.

Conclusions: In general, the magnitude of enzyme activity correlates with the phenotypic findings. For example, mutations that completely impair enzyme activity, such as homozygous deletions of the promoter region, are associated with salt-losing congenital adrenal hyperplasia. However, the splicing mutation at nucleotide 655 in the second intron is characterized by greater phenotypic heterogeneity than the other mutations in this series.

Androgen insensitivity syndromes are the clinical manifestations of end-organ resistance to androgen actions. In this inborn error of sexual differentiation, the most common cause of end-organ resistance is altered androgen receptor function. The clinical features of androgen insensitivity range from phenotypic females with complete androgen insensitivity to minimally affected phenotypic males with partial androgen insensitivity. The mechanism of action of the androgen receptor involves binding of the ligand which promotes conformational changes to overcome the inhibition to dimerization. The receptor is translocated to the nucleus if it is not already located there. The receptor then binds to a specific region of DNA, the hormone response element, eliciting its actions within the target cell. Post-translation modification in terms of phosphorylation may occur. Hydroxyfluta-mide, an antiandrogen, is able to bind to the androgen receptor, but presumably does not achieve the correct conformation to promote DNA binding.

Analyzing the abnormal receptor gene in affected families has provided much information regarding the structure and function of the androgen receptor. In contrast to cystic fibrosis in which many affected individuals carry a deletion of phenylalanine at codon 508, the diversity of mutations in androgen insensitivity complicates the development of simple molecular screening tests. Nevertheless, for those instances of partial androgen insensitivity and significant genital ambiguity, phenotype/genotype correlations for previously recognized mutations may be helpful in predicting the natural history, i.e., magnitude of androgen resistance.

Study Objective: This study examined the utilization of medroxyprogesterone acetate (Depo-Provera) in adolescent patients to determine selected side effects, degree of satisfaction, and continuation rates.

Design: The design was a retrospective chart review of 50 adolescent patients totaling 384 clinic visits and accumulating 1007 woman-months of experience on DepoProvera.

Setting: The Teenage Clinic at Children's Hospital, Columbus, Ohio, was the setting for this study.

Participants: The mean age was 15.6 (±2.5); racial distribution was 52% African-American and 48% white. The mean number of visits per patient was 7.4 (±7.9), ranging from 2 to 39 visits.

Interventions: Each patient was given Depo-Provera for either a pre-existing medical indication or for contraception.

Main Outcome Measures: Uterine bleeding patterns, blood pressure readings, patient satisfaction, and continuation rates. Results: A strong correlation was found between length of time on Depo-Provera and amenorrhea and spotting (p < 0.01). No new recordings of hypertension occurred. The majority of patients expressed overall satisfaction with Depo-Provera, and most found amenorrhea to be a positive aspect of the drug. Continuation rates exceeded 50% after 2 years and generally fell within ranges reported for adult women. No pregnancies were recorded during the course of the study.

Conclusions: This study indicates that Depo-Provera can be used in an adolescent population with a high degree of acceptability and does not increase patient risk for hypertension. The likelihood of amenorrhea increases greatly with duration of treatment and does not seem to deter the continuation of its use.

Objective: To test the hypothesis that adolescents who experience a decrease in depressive symptomatology during gestation give birth to larger, gestationally older infants than do adolescents who do not experience a decrease in depressive symptomatology during gestation.

Method: We studied 122 poor, black 12- through 19-year-olds enrolled in an adolescent-oriented prenatal program. The subjects completed two psychological evaluations during gestation. Infant birth weight and gestational age were analyzed in relation to change in depressive symptomatology.

Results: We found no relationship between change in depressive symptomatology and maternal age, socioeconomic status, adequacy of prenatal care, living arrangements, gestational weight gain, or substance abuse. At enrollment, subjects who experienced a decrease in depressive symptoms during gestation had significantly higher depression and worry scores and significantly lower social support scores. During gestation these young women reported a more significant decrease in worries and increase in family support than did young women who did not experience a decrease in depressive symptoms during gestation. Change in depressive symptomatology was also significantly related to the duration of gestation (r = 0.26; p = 0.004).

Conclusions: This study suggests that changes in the intensity of maternal psychological distress during gestation may be associated with changes in maternal social support and measurable biologic effects on the fetus.

Uterine torsion in childhood is rare. It seems to occur secondary to other pathology, namely the presence of an ovarian cyst. We discuss a 9-year-old child who presented in an insidious fashion with ultrasound findings compatible with hydrometrosalpinx. In our patient, the uterine torsion resulted in infarction of the right ovary, the tubes bilaterally, and the uterus. We describe our treatment of this child, and we hope that increased awareness might prevent future delays in diagnosis.