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At least 739 records · Page 41Linked to original sources
PubMed1987

Urorectal septum malformation sequence. Report of six cases and embryological analysis.

We encountered six female infants with a specific pattern of developmental abnormalities of the urogenital and lower intestinal tracts. The anomalies included ambiguous genitalia, lack of perineal openings, and müllerian and urinary tract anomalies. Each patient had normal female chromosomes and normal adrenal gland function. We believe that this combination of anomalies represents a recognizable and specific sequence that is due to a failure of migration to and/or fusion of the urorectal septum with the cloacal membrane. This, in turn, we postulate, leads to persistence of the cloaca and cloacal membrane and failure of normal differentiation of the external genitalia. Persistence of the cloacal membrane results in absence of the urethral and vaginal openings and an imperforate anus. We propose calling this entity the urorectal septum malformation sequence.

Anus, Imperforate↗
PubMed2006

Molecular genetic cascades for external genitalia formation: an emerging organogenesis program.

External genitalia are anatomical structures located at the posterior embryonic region as part of several urogenital/reproductive organs. The embryonic anlage of the external genitalia, the genital tubercle (GT) develops as a bud-shaped structure with an initial urethral plate and later urethra. Embryonic external genitalia are considered to be one of the appendages. Recent experiments suggest that essential regulatory genes possess similar functions for the outgrowth regulation of the GT and limb appendages. The transient embryonic epithelia located in the distal GT are called the distal urethral epithelium (DUE) regulating, at least in part, the (distal) GT development. This review covers the available data about early patterning of GT and discusses the molecular developmental similarities and points of divergence between the different appendages. Development of the male and female external genitalia is also reviewed.

Animals↗
PubMed2003

Serial MR imaging with MS-325 for evaluating female sexual arousal response: determination of intrasubject reproducibility.

PURPOSE: To determine if a similar sexual arousal response in normal, healthy women could be obtained and monitored by serial magnetic resonance (MR) imaging at two separate sessions. MATERIALS AND METHODS: Serial imaging of the external genitalia was performed on nine healthy, sexually functional women at two separate MR sessions after administration of the contrast agent, MS-325. Images were obtained every three minutes during a 45-minute study period during each MR session. The second MR session began approximately 45 minutes after the end of the first MR session. While undergoing imaging, subjects viewed videotapes that contained neutral and sexually-explicit material through an audiovisual system. Analysis performed at each time point consisted of visual evaluation of the images, clitoral and femoral vein signal intensity measurements, relative regional blood volume calculations, and clitoral volume measurements. Statistical analysis of the results consisted of calculating correlation coefficients of the two MR sessions by using the least square fit method. RESULTS: All nine subjects reported sexual arousal on subjective questionnaires at each MR session. Post-contrast MS-325 MR images showed strong enhancement of the external genitalia at each session. There was excellent correlation between the two sessions for the clitoral volume measurements of all nine subjects. The correlation coefficient, r(2), was 0.95. CONCLUSION: The sexual arousal response in normal, healthy women can be monitored by serial imaging combined with the use of the contrast agent, MS-325, and similar results can be reproduced at two different MR sessions. This method holds promise for future studies of women with female sexual arousal dysfunction.

Adult↗
PubMed2003

Conditional stimulation of the dorsal penile/clitoral nerve may increase cystometric capacity in patients with spinal cord injury.

AIMS: To investigate the feasibility of conditional short duration electrical stimulation of the penile/clitoral nerve as treatment for detrusor hyperreflexia, the present study was initiated. METHODS: Ten patients with spinal cord injury, 4 women and 6 men, with lesions at different levels above the sacral micturition center had a standard cystometry performed. During a subsequent cystometry, conditional short duration electrical stimulation of the penile/clitoral nerve was performed as treatment for one or more detrusor hyperreflexic contractions. RESULTS: In all patients, at least one contraction (mean, 7.8; range, 1-16 contractions) was inhibited by the stimulations. The mean cystometric capacity was increased significantly by conditional electrical stimulation, from 210 mL in the control cystometries to 349 mL in the stimulation cystometries (P=0.016). The maximal detrusor pressure during the first contraction in the control cystometries was mean 51 cm H(2)O, whereas the maximal pressure of the first contraction in the stimulation cystometries was reduced to mean 33 cm H(2)O (P=0.045). CONCLUSIONS: The authors conclude that repeated conditional short duration electrical stimulation significantly increased cystometric capacity in patients with spinal cord injury. The increase was caused mainly by an inhibition of detrusor contractions. The need for a reliable technique for chronic bladder activity monitoring is emphasized, as it is a prerequisite for clinical application of this treatment modality.

Adult↗
PubMed2003

Validation of the clinical bulbocavernosus reflex.

AIMS: To validate the clinically obtained bulbocavernosus reflex (BCR) by comparing it to the results of clitoroanal reflex (CAR) electrodiagnostic testing. MATERIALS AND METHODS: Thirty three women with detrusor overactivity underwent clinical examination and electrodiagnostic testing including evaluation of CAR latency using a Nicolet Viking IIe electrodiagnostic instrument, stimulating the paraclitoral area on each side in turn with paired stimuli while recording anal sphincter compound muscle action potentials bilaterally using surface patch electrodes. The results of clinical and electrodiagnostic testing were compared. RESULTS: The BCR was clinically present on at least one side in 26 (79%) women, and thought to be clinically bilaterally absent in 7 (21%). Electrophysiologic testing revealed CARs to be at least unilaterally present in 30 (91%) patients. When the BCR was clinically present, a CAR was recorded in 92% of patients. Of the seven women with clinically absent BCR, just one had absent CARs. CONCLUSIONS: In this sample of women with detrusor overactivity, the clinical BCR did not have strong correlation with the electrically obtained reflex.

Action Potentials↗
PubMed2006

Event driven electrical stimulation of the dorsal penile/clitoral nerve for management of neurogenic detrusor overactivity in multiple sclerosis.

AIMS: The aim of this study was to evaluate the effect of automatic event driven electrical stimulation on the dorsal penile/clitoral nerve for management of neurogenic detrusor overactivity in patients suffering from Multiple Sclerosis. METHODS: A total of 10 patients participated in the study. Detrusor pressure was recorded during physiological filling of the bladder and electrical stimulation was applied with surface electrodes whenever the detrusor pressure exceeded 10 cm H(2)O. RESULTS: In seven of the eight patients, where neurogenic detrusor overactivity was observed an average of 12 detrusor contractions could be inhibited by stimulation. In one patient, however, stimulation failed to inhibit the detrusor contractions. The average increase in bladder volume from first suppressed detrusor contraction until leakage was 94% (range: 22-366%). On average, the time from first suppressed contraction until leakage was 15 min and 50 sec (range: 4 min 58 sec-32 min 5 sec) with an average physiological filling rate of 8 ml/min. Urgency was effectively suppressed at the onset of stimulation. CONCLUSIONS: The results indicate that involuntary detrusor contractions in patients with multiple sclerosis can effectively be inhibited with event driven stimulation, hereby improving bladder capacity and reducing the number of incontinence episodes. However, the used method for detecting detrusor contractions is not suitable in a chronic setting and alternative techniques needs to be investigated if stimulation should be applied automatically.

Adult↗
PubMed1984

Direct fetal injections of diethylstilbestrol and 17 beta-estradiol: a method for investigating their teratogenicity.

The synthetic estrogen, diethylstilbestrol (DES), causes urogenital malformations in humans, primates, and rodents. This study was designed to determine whether these effects of DES are related to its estrogenicity. Therefore, DES (0.1, 1, and 10 micrograms) or the natural estrogen, estradiol (E2) (10 and 100 micrograms) was injected directly into day 19 rat fetuses. In the 6- to 7-week-old female offspring exposed to DES, a dose-related incidence of cleft phallus, hypospadias, and incomplete coiling of oviducts was observed. The single fetal injection of E2 elicited similar urogenital malformations, but was approximately 100-fold less potent than DES. A single subcutaneous dose of either DES (0.025, 0.25, or 2.5 mg/kg) or E2 (2.5 or 25 mg/kg) to dams on day 19 of pregnancy induced a spectrum of malformations similar to that following fetal injection. The offspring of treated dams, but not those injected directly as fetuses, had nonfunctioning ovaries (no corpora lutea) yet vaginal signs of estrous were present. It is concluded that DES can act directly in the fetus and its teratogenicity does not require maternal mediation. Since a high dose of E2 produced similar malformations when given to fetuses, it appears that excess estrogen during prenatal life is teratogenic. Thus, at least those endpoints of the teratogenicity of DES that were measured are accounted for by its estrogenic activity.

Animals↗
PubMed1998

Sildenafil inhibits phosphodiesterase type 5 in human clitoral corpus cavernosum smooth muscle.

Phosphodiesterases play an important physiological role by regulating the intracellular levels of cyclic nucleotides. In this study, we investigated the kinetic parameters of inhibition of phosphodiesterase (PDE) type 5 (EC 3.1.4.35, 3',5'-cyclic GMP phosphodiesterase) by a novel, high-affinity, selective PDE type 5 inhibitor, sildenafil, in intact cells and in soluble extracts of human clitoral corpus cavernosum smooth muscle cells. Sildenafil inhibited cGMP hydrolysis in the crude extract (Ki = 7.2 +/- 2.7) and in partially purified preparations (Ki = 9 nM) in a competitive manner, as determined by Dixon plots. Sildenafil was a more effective PDE type 5 inhibitor than zaprinast (Ki = 400.0 +/- 76.4 nM, crude extracts; 250 nM, partially purified). Stimulation of intracellular cGMP synthesis by the nitric oxide donor sodium nitroprusside resulted in a 3.3- and 2.9-fold increase in cGMP concentration in the presence of sildenafil or zaprinast, respectively, compared to sodium nitroprusside treatment alone in intact cells at physiological temperatures. These observations suggest that human clitoral corpus cavernosum smooth muscle tone may be regulated by the synthesis and release of nitric oxide and that this pathway is dependent on PDE type 5 activity.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed1993

Metastatic clear-cell hidradenocarcinoma of the vulva.

Clear-cell hidradenocarcinoma is a malignant tumor of sweat gland origin. It is most often found on the trunk, head, and extremities. This case report describes a rare occurrence of this tumor on the vulva of a young woman. The discovery of metastatic disease reflects the potentially aggressive nature of this tumor.

Adenocarcinoma↗