Bannayan-Zonana syndrome (BZS) is a genetic disorder with autosomal dominant inheritance characterized by macrocephaly and multiple hamartomas of mesodermal origin. Here we present a patient with BZS manifested by many of the classic features, as well as a high-flow upper extremity arteriovenous malformation (AVM). Although this rare syndrome was initially described in 1971, to our knowledge, this is the first report showing an association of AVM with BZS and the first report of this syndrome in the radiologic literature.

Abnormalities, Multiple↗

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A 15-item short form of the psychological and interpersonal relationship scales.

Erectile dysfunction (ED) is a prevalent condition associated with complex psychological and interpersonal issues. The Psychological and Interpersonal Relationship Scales (PAIRS) is a valid and reliable measure of Sexual Self-Confidence, Spontaneity, and Time Concerns associated with ED and its treatment. This paper describes the development and validation of a 15-item, short form of PAIRS (SF-PAIRS). Domain scores and psychometric properties of SF-PAIRS were assessed using data from the validation studies of the full-length (23-item) PAIRS. Items in the two briefer scales (Spontaneity and Time Concerns) were identified using internal consistency analyses and crossvalidated during two multinational clinical trials and a community sample. The scales in SF-PAIRS showed good internal consistency, convergent and discriminant validity, were stable over a 2-week period, and showed sensitivity to treatment. SF-PAIRS is a valid, brief measure of psychological and interpersonal outcomes associated with ED.

Adult↗

PubMedDate not supplied

The Female Sexual Function Index (FSFI): a multidimensional self-report instrument for the assessment of female sexual function.

This article presents the development of a brief, self-report measure of female sexual function. Initial face validity testing of questionnaire items, identified by an expert panel, was followed by a study aimed at further refining the questionnaire. It was administered to 131 normal controls and 128 age-matched subjects with female sexual arousal disorder (FSAD) at five research centers. Based on clinical interpretations of a principal components analysis, a 6-domain structure was identified, which included desire, subjective arousal, lubrication, orgasm, satisfaction, and pain. Overall test-retest reliability coefficients were high for each of the individual domains (r = 0.79 to 0.86) and a high degree of internal consistency was observed (Cronbach's alpha values of 0.82 and higher) Good construct validity was demonstrated by highly significant mean difference scores between the FSAD and control groups for each of the domains (p < or = 0.001). Additionally, divergent validity with a scale of marital satisfaction was observed. These results support the reliability and psychometric (as well as clinical) validity of the Female Sexual Function Index (FSFI) in the assessment of key dimensions of female sexual function in clinical and nonclinical samples. Our findings also suggest important gender differences in the patterning of female sexual function in comparison with similar questionnaire studies in males.

Adult↗

PubMedDate not supplied

Topical alprostadil in the treatment of Female Sexual Arousal Disorder: a pilot study.

This study evaluated the efficacy and safety of three doses of topical alprostadil USP (prostaglandin E1) cream in 8 patients with Female Sexual Arousal Disorder (FSAD). Each patient was administered a single intravaginal dose of placebo followed by escalating intravaginal doses of the active drug at 2-week intervals. Alprostadil's effectiveness in enhancing subjective and physiological arousal during visual sexual stimulation was supported by patient ratings and physician assessments of vaginal erythema and transudate volume. Photoplethysmography measurement of vaginal pulse amplitude was not able to demonstrate treatment sensitivity in the present study. Adverse events included mild cases of vaginal itching and burning. The data support further investigation of the use of alprostadil for FSAD.

Administration, Topical↗

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Challenges in conducting multicenter clinical trials in female sexual dysfunction: baseline differences between study populations.

Female sexual dysfunction (FSD) presents several unique challenges in the design and interpretation of multicenter clinical trials. This paper discusses the issue of baseline differences between study populations, using the demographic data of a recent publication describing the validation of a new questionnaire for the evaluation of FSD.

Adult↗

PubMedDate not supplied

Physiology of the excretory urogram. 3. A densitometric and subjective assessment of changes in contrast medium concentration.

Explore the source record for details and available documents.

Contrast Media↗

PubMedDate not supplied

Endoscopic ligation of umbilical cord at 19 week's gestation in monoamniotic monochorionic twins discordant for hypoplastic left heart syndrome.

We report the first attempt of reduction of monoamniotic twins, discordant for hypoplastic left heart syndrome, using a new fetoscopic technique. Employing sonographic guidance and endoscopic visualization, cord ligation was accomplished, but significant cord entanglement, not previously appreciated, resulted in the ligation of the umbilical cord of the normal fetus. Cord entanglement may frustrate endoscopic techniques in monoamniotic twins.

Adult↗

PubMedDate not supplied

Microbial sensors of ultraviolet radiation based on recA'::lux fusions.

Escherichia coli strains containing plasmid-borne fusions of the recA promoter-operator region to the Vibrio fischeri lux genes were previously shown to increase their luminescence in the presence of DNA damage hazards, and thus to be useful for genotoxicant detection. The present study expands previous work by demonstrating and investigating the luminescent response of these strains to ultraviolet radiation. Several genetic variants of the basic recA'::lux design were examined, including a tolC modification of membrane efflux capacity, a chromosomal integration of the recA'::lux fusion, a different lux reporter (Photorhabdus luminescens instead of V. fischeri, allowing the assay to be run at 37 degrees C), and a different host bacterium (Salmonella typhimurium instead of E. coli). Generally, two modifications provided the fastest responses: the use of the S. typhimurium host or the P. luminescens lux reporter. Highest sensitivity, however, was demonstrated in an E. coli strain in which a single copy of the V. fischeri lux fusion was integrated into the bacterial chromosome.

Bacterial Proteins↗

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Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature.

We present the management challenge provided by a patient with kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon. A female child presented at 14 months of age with an ecchymotic swelling of her right upper arm and axilla. Subsequently, she developed profound thrombocytopenia and hypofibrinogenemia (Kasabach-Merritt phenomenon). Biopsy of the lesion revealed kaposiform hemangioendothelioma, which has been reported as the predominant pathologic diagnosis associated with Kasabach-Merritt phenomenon. To achieve involution of the lesion and preserve function of the arm, the following interventions were involved: embolization, systemic interferon, cyclophosphamide, epsilon aminocaproic acid, and compression therapy. The clinical management of this patient was formidable until we arrived at the proper combination of therapies. Multimodal intervention may be required to manage fastidious hemangioendotheliomas of childhood, achieve clinical improvement, and prevent further morbidity.

Female↗

PubMedDate not supplied