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1.
Am J Hum Genet ; 108(2): 337-345, 2021 02 04.
Artigo em Inglês | MEDLINE | ID: mdl-33434492

RESUMO

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is associated with congenital absence of the uterus, cervix, and the upper part of the vagina; it is a sex-limited trait. Disrupted development of the Müllerian ducts (MD)/Wölffian ducts (WD) through multifactorial mechanisms has been proposed to underlie MRKHS. In this study, exome sequencing (ES) was performed on a Chinese discovery cohort (442 affected subjects and 941 female control subjects) and a replication MRKHS cohort (150 affected subjects of mixed ethnicity from North America, South America, and Europe). Phenotypic follow-up of the female reproductive system was performed on an additional cohort of PAX8-associated congenital hypothyroidism (CH) (n = 5, Chinese). By analyzing 19 candidate genes essential for MD/WD development, we identified 12 likely gene-disrupting (LGD) variants in 7 genes: PAX8 (n = 4), BMP4 (n = 2), BMP7 (n = 2), TBX6 (n = 1), HOXA10 (n = 1), EMX2 (n = 1), and WNT9B (n = 1), while LGD variants in these genes were not detected in control samples (p = 1.27E-06). Interestingly, a sex-limited penetrance with paternal inheritance was observed in multiple families. One additional PAX8 LGD variant from the replication cohort and two missense variants from both cohorts were revealed to cause loss-of-function of the protein. From the PAX8-associated CH cohort, we identified one individual presenting a syndromic condition characterized by CH and MRKHS (CH-MRKHS). Our study demonstrates the comprehensive utilization of knowledge from developmental biology toward elucidating genetic perturbations, i.e., rare pathogenic alleles involving the same loci, contributing to human birth defects.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/genética , Anormalidades Congênitas/genética , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/crescimento & desenvolvimento , Mutação , Ductos Mesonéfricos/crescimento & desenvolvimento , Adulto , Proteína Morfogenética Óssea 4/genética , Proteína Morfogenética Óssea 7/genética , Códon sem Sentido , Feminino , Estudos de Associação Genética , Pleiotropia Genética , Proteínas Homeobox A10/genética , Proteínas de Homeodomínio/genética , Humanos , Fator de Transcrição PAX8/genética , Herança Paterna , Penetrância , Proteínas com Domínio T/genética , Fatores de Transcrição/genética , Proteínas Wnt/genética , Ductos Mesonéfricos/anormalidades
2.
J Minim Invasive Gynecol ; 27(7): 1465-1466, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32259653
3.
Artigo em Inglês | MEDLINE | ID: mdl-31517310

RESUMO

Objective: The aim of this study was to use whole genome sequencing (WGS) help detect de novo mutations or pathogenic genes of Mayer-Rokitansky-Küster-Hauser syndrome type 1(MRKH syndrome type 1). Study design: This was a case-parent trios study. Nine unrelated probands, with MRKH syndrome type 1 and their parents were enrolled. The enrollment, sequencing process, establishment of the de novo mutations detecting procedure and experiment part were performed over a 2-year period. Results: we detected 632 de novo single nucleotide variants (SNVs), 267 de novo small insertions/deletions (indels), 39 de novo structural variations (SVs) and 28 de novo copy number alterations (CNAs). Three novel damaging coding de novo SNVs with three damaging coding de novo genes (PIK3CD, SLC4A10 and TNK2) were revealed. Two SNVs were annotated of the promoter region of gene NBPF10 and 3'UTR of NOTCH2NL, potentially contributing to the pathogenesis of MRKH. Conclusion: We identified five de novo mutations in BAZ2B, KLHL18, PIK3CD, SLC4A10 and TNK2 by performing WGS, the functional involvement of all deleterious mutations in MRKH candidate genes of the trios warrant further study. WGS may complement conventional array to capture the complete landscape of the genome in MRKH.

4.
Sci Rep ; 9(1): 8482, 2019 06 11.
Artigo em Inglês | MEDLINE | ID: mdl-31186444

RESUMO

To investigate the artificial vaginal microecological features in patients who underwent laparoscopic peritoneal vaginoplasty. 54 cases of patients with artificial vagina after laparoscopic peritoneal vaginoplasty were included in this study. Microecosystem evaluation was performed. Artificial vaginal functional tests and biopsy from vaginal walls were performed. After laparoscopic peritoneal vaginoplasty, the artificial vaginal flora intensity was level II∼III (88.9%); the vaginal flora diversity was level II∼III (72.2%); the predominant vaginal bacteria were gram-positive macrobacillus (27.8%); approximately 57.4% of the patients had vaginal pH ≤ 4.5; there was no pathogenic bateria or other pathogens; dysbiosis accounted for 53.7% of the patients (64.5% of the patients who had the vaginoplasty operation less than 2 years ago exhibited dysbiosis; 39.1% of the patients who had the operation at least 2 years ago exhibited dysbiosis). Vaginal dysbiosis is common after laparoscopic peritoneal vaginoplasty. However, as time goes by, the artificial vaginal microecological condition gradually becomes normal. Evaluation of vaginal microenvironment after laparoscopic peritoneal vaginoplasty might play an important role in reproductive tract infection prevention and neovagina health care.


Assuntos
Procedimentos Cirúrgicos em Ginecologia , Laparoscopia , Peritônio/cirurgia , Vagina/microbiologia , Adolescente , Adulto , Disbiose/etiologia , Feminino , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Humanos , Concentração de Íons de Hidrogênio , Laparoscopia/efeitos adversos , Período Pós-Operatório , Adulto Jovem
5.
Eur J Obstet Gynecol Reprod Biol ; 228: 313-318, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30075412

RESUMO

OBJECTIVE: The aim of this study was to examine if the advanced tool of 3D MRI model provides more precise information on the anatomy of pelvic organs than MRI alone, and compare to clinical and operative finding of patients with vagina in Müllerian duct anomalies. STUDY DESIGN: One hundred and ten patients with clinically and operatively proven Müllerian duct anomalies were included. The consistency of diagnosis of Mullerian tract anomalies by MRI and 3D MRI compared to clinically and surgically proven anomalies defined according to ASRM classification. RESULTS: We successfully reconstructed retrospectively all 110 patients' three-dimensional models from the simple MRI scans. Eighty-six of the patients (78.2%) showed an agenesis of the uterus, four patients presented with uterine didephys (3.6%). Septate uterus was detectable in 8 of 110 cases (7.3%). Arcuate uterus was detectable in 3 of 110 cases (2.7%). The remaining patients presented with either unilateral (n = 4; 3.6%) or bilateral (n = 5; 4.5%) uterine horns. Reviewed by radiologists, comparing the agreement rate between 3D- MRI reconstruction models and simple MRI, there was a trend towards but not significant difference (P = 0.064). Reviewed by trained surgeons, the agreement between three-dimensional MRI reconstruction models and MRI, there was significant difference (P = 0.003). CONCLUSION: 3D MRI model is a non-expensive add-on software tool that enhance the ability of expert surgeon to reach a more precise diagnosis of the pelvic anatomy structures, compared to MRI alone. Getting a more precise description of the pelvic anatomy allows a better planning of the corrective procedure needed and preoperative assessment of the expected prognosis.


Assuntos
Ductos Paramesonéfricos/anormalidades , Anormalidades Urogenitais/diagnóstico por imagem , Útero/anormalidades , Adolescente , Adulto , Criança , Feminino , Humanos , Imageamento Tridimensional , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Estudos Retrospectivos , Útero/diagnóstico por imagem , Adulto Jovem
6.
Rev Sci Instrum ; 89(7): 073501, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30068087

RESUMO

The diagnosis of the fuel retention and impurity deposition on the plasma facing components (PFCs) is very important for monitoring plasma-wall interactions and improving the performance of long-pulse operation for tokamak devices. In this study, a remote in situ laser-induced breakdown spectroscopic (RIS-LIBS) system has been developed to be an effective and routine method for the diagnosis of the composition of the PFCs on Experimental Advanced Superconducting Tokamak (EAST). The RIS-LIBS system can be operated between EAST discharges via a remote network control system. This allows a flexible diagnosis for the PFCs at a specific EAST discharge operation or under planned plasma scenarios according to the experimental requirement. Measurements on the fuel retention and impurity deposition of the PFCs have been performed for the test of the RIS-LIBS system, and the depth resolution and the lateral resolution of the RIS-LIBS system have been achieved to be ∼100 nm and ∼3.0 mm, respectively. For the test of detectable elements, the fuel (deuterium) and impurities have been detected and identified clearly. In addition, the measurement of fuel abundance on the first wall as a function of the days of EAST deuterium plasma discharges has been carried out for the first time. These results well manifest a significant prospect of the RIS-LIBS for the diagnosis of the PFCs in the upcoming fusion devices like China Fusion Engineering Test Reactor (CFETR) and ITER.

7.
Rev Sci Instrum ; 88(11): 113508, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29195382

RESUMO

Plasma Facing Components (PFCs) in a magnetically confined fusion plasma device will be exposed to high heat load and particle fluxes, and it would cause PFCs' surface morphology to change due to material erosion and redeposition from plasma wall interactions. The state of PFCs' surface condition will seriously affect the performance of long-pulse or steady state plasma discharge in a tokamak; it will even constitute an enormous threat to the operation and the safety of fusion plasma devices. The PFCs' surface morphology evolution measurement could provide important information about PFCs' real-time status or damage situation and it would help to a better understanding of the plasma wall interaction process and mechanism. Meanwhile through monitoring the distribution of dust deposition in a tokamak and providing an upper limit on the amount of loose dust, the PFCs' surface morphology measurement could indirectly contribute to keep fusion operational limits and fusion device safety. Aiming at in situ dynamic monitoring PFCs' surface morphology evolution, a laboratory experimental platform DUT-SIEP (Dalian University of Technology-speckle interferometry experimental platform) based on the speckle interferometry technique has been constructed at Dalian University of Technology (DUT) in China. With directional specific designing and focusing on the real detection condition of EAST (Experimental Advanced Superconducting Tokamak), the DUT-SIEP could realize a variable measurement range, widely increased from 0.1 µm to 300 µm, with high spatial resolution (<1 mm) and ultra-high time resolution (<2 s for EAST measuring conditions). Three main components of the DUT-SIEP are all integrated and synchronized by a time schedule control and data acquisition terminal and coupled with a three-dimensional phase unwrapping algorithm, the surface morphology information of target samples can be obtained and reconstructed in real-time. A local surface morphology of the real divertor tiles adopted from EAST has been measured, and the feasibility and reliability of this new experimental platform have been demonstrated.

9.
Rev Sci Instrum ; 88(4): 043502, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28456259

RESUMO

To facilitate long-pulse high power operation, an ITER-like actively cooled tungsten (W) divertor was installed in Experimental Advanced Superconducting Tokamak (EAST) to replace the original upper graphite divertor in 2014. A dedicated multichannel visible spectroscopic diagnostic system has been accordingly developed for the characterization of the plasma and impurities in the W divertor. An array of 22 lines-of-sight (LOSs) provides a profile measurement of the light emitted from the plasma along upper outer divertor, and the other 17 vertical LOSs view the upper inner divertor, achieving a 13 mm poloidal resolution in both regions. The light emitted from the plasma is collected by a specially designed optical lens assembly and then transferred to a Czerny-Turner spectrometer via 40 m quartz fibers. At the end, the spectra dispersed by the spectrometer are recorded with an Electron-Multiplying Charge Coupled Device (EMCCD). The optical throughput and quantum efficiency of the system are optimized in the wavelength range 350-700 nm. The spectral resolution/coverage can be adjusted from 0.01 nm/3 nm to 0.41 nm/140 nm by switching the grating with suitable groove density. The frame rate depends on the setting of LOS number in EMCCD and can reach nearly 2 kHz for single LOS detection. The light collected by the front optical lens can also be divided and partly transferred to a photomultiplier tube array with specified bandpass filter, which can provide faster sampling rates by up to 200 kHz. The spectroscopic diagnostic is routinely operated in EAST discharges with absolute optical calibrations applied before and after each campaign, monitoring photon fluxes from impurities and H recycling in the upper divertor. This paper presents the technical details of the diagnostic and typical measurements during EAST discharges.

10.
Rev Sci Instrum ; 88(12): 123501, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29289185

RESUMO

Fiber Bragg Gratings (FBGs) have been widely used in the sensor field to monitor temperature and strain. However, the weak mechanical property of optical fibers and insufficient heat-resistant property of general optic-fiber sensors have prevented it from being widely used, such as in some extreme engineering situations. In this work, a bare FBG sensor system had been introduced to measure thermal strain of an Experimental Advanced Superconducting Tokamak tungsten divertor component under baking condition. This strain measurement system had withstood as high temperature as 210 °C and finished the measurement experiment successfully. Meaningful measurement results had been obtained and analyzed, which showed the applicability of such a bare fiber grating sensor system and as well contributed to studying on tungsten divertor's thermal strain conditions.

11.
Sci Rep ; 6: 32678, 2016 09 06.
Artigo em Inglês | MEDLINE | ID: mdl-27597314

RESUMO

Dense W and W-Zr composites reinforced with Sc2O3 particles were produced through powder metallurgy and subsequent spark plasma sintering (SPS) at 1700 °C and 58 MPa. Results showed that the W-1vol.%Zr/2vol.%Sc2O3 composites exhibited optimal performance with the best relative density of up to 98.93% and high Vickers microhardness of approximately 583 Hv. The thermal conductivity of W-Zr/Sc2O3 composites decreased initially and then increased as the Zr content increased. The moderate Zr alloying element could combine well with Sc2O3 particles and W grains and form a solid solution. However, excess Zr element leads to agglomeration in the grain boundaries. W-1vol.%Zr/2vol.%Sc2O3 composite had a good deuterium irradiation resistance very closing to pure tungsten compared with the other Zr element contents of composites. Under 500 K, D2 retention and release of them were similar to those of commercial tungsten, even lower between 400 K to 450 K. Pre-irradiation with 5 keV-He(+) ions to a fluence of 1 × 10(21) He(+)/m(2) resulted in an increase in deuterium retention (deuterium was implanted after He(+) irradiation), thereby shifting the desorption peak to a high temperature from 550 K to 650 K for the W-1vol.%Zr/2vol.%Sc2O3 composite.

12.
Fertil Steril ; 106(5): 1190-1194, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27349924

RESUMO

OBJECTIVE: To analyze the phenotypic and clinical aspects of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. DESIGN: Cross-sectional study. SETTING: University hospital. PATIENT(S): Five hundred and ninety-four patients with MRKH syndrome. INTERVENTION(S): Clinical examination, abdominal or perineal/rectal ultrasound, magnetic resonance imaging, hormonal profile, karyotype, and laparoscopy. MAIN OUTCOME MEASURE(S): Clinicopathologic data, VCUAM (vagina cervix uterus adnex-associated malformation) classification, types with cycle phase, and karyotype. RESULT(S): We identified associated malformations in 43 out of 594 (7.2%) cases of MRKH. The 594 patients could be grouped into hormone phases: 53.7% follicular, 35.2% luteal, and 11.1% ovulatory. The major karyotype of MRKH patients was 46,XX; abnormal karyotypes were found in two cases. CONCLUSION(S): A lower proportion of associated malformations were found when compared with those provided in the current literature. Renal anomalies were the most frequent associated malformations, and most of the patients presented with a normal karyotype. Given the large cohort of this study, the lower malformation rates might be related to geographic or referral patterns, so further investigation is warranted.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Anexos Uterinos/anormalidades , Colo do Útero/anormalidades , Anormalidades Congênitas/diagnóstico , Ductos Paramesonéfricos/anormalidades , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/sangue , Transtornos 46, XX do Desenvolvimento Sexual/genética , Transtornos 46, XX do Desenvolvimento Sexual/fisiopatologia , Anexos Uterinos/diagnóstico por imagem , Adolescente , Adulto , Biomarcadores/sangue , Colo do Útero/diagnóstico por imagem , Colo do Útero/fisiopatologia , Criança , China , Cromossomos Humanos X , Anormalidades Congênitas/sangue , Anormalidades Congênitas/genética , Anormalidades Congênitas/fisiopatologia , Estudos Transversais , Feminino , Predisposição Genética para Doença , Hormônios/sangue , Humanos , Cariótipo , Cariotipagem , Laparoscopia , Imagem por Ressonância Magnética , Ciclo Menstrual/sangue , Ductos Paramesonéfricos/fisiopatologia , Fenótipo , Ultrassonografia , Vagina/diagnóstico por imagem , Adulto Jovem
13.
Int J Gynaecol Obstet ; 133(3): 320-4, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27087418

RESUMO

OBJECTIVE: To evaluate the outcomes of laparoscope-assisted peritoneal vaginoplasty for the treatment of congenital vaginal atresia. METHODS: A retrospective study enrolled patients diagnosed with congenital vaginal atresia who were treated with one of two different laparoscope-assisted peritoneal vaginoplasty techniques (named Luohu-one and Luohu-two) between October 31, 2001 and December 31, 2014. Operative time, intraoperative bleeding volume, surgical difficulty, complications, and post-procedure sexual satisfaction were reported. RESULTS: Data were collected for 620 patients. The Luohu-one procedure was used in the treatment of 145 patients, while 475 patients were treated with the Luohu-two procedure. In 5 (0.8%) patients, it was necessary to perform a sigmoid colon vaginoplasty. During surgery, 16 patients experienced a rectal injury, among whom, 9 patients experienced a rectal-vaginal fistula. Follow-up data extending to 7years were available for 285 patients. Of these 285 patients, 231 agreed to report details of their sexual experiences. In total, 222 (96.1%) patients reported being very satisfied with their vaginal conditions and sex life. The Luohu-two procedure demonstrated shorter operative and recovery time, and reduced intraoperative bleeding. However, both procedures demonstrated satisfactory results. CONCLUSION: Laparoscope-assisted peritoneal vaginoplasty demonstrated good safety and effectiveness in the treatment of patients with congenital vaginal atresia.


Assuntos
Anormalidades Congênitas/cirurgia , Laparoscopia/métodos , Complicações Pós-Operatórias/epidemiologia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Vagina/anormalidades , Adolescente , Adulto , China , Colo Sigmoide/cirurgia , Feminino , Seguimentos , Humanos , Laparoscopia/efeitos adversos , Duração da Cirurgia , Orgasmo , Períneo/cirurgia , Peritônio/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Fístula Retovaginal/cirurgia , Estudos Retrospectivos , Vagina/cirurgia , Adulto Jovem
14.
Eur J Med Res ; 20: 69, 2015 Aug 22.
Artigo em Inglês | MEDLINE | ID: mdl-26297245

RESUMO

BACKGROUND: Surgical vaginoplasty is the standard treatment for women suffering from Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. This study compares the advantages and disadvantages of Luohu I technique or its modification, Luohu II technique. METHODS: Women with MRKH syndrome undergoing laparoscopic peritoneal vaginoplasty using either the Luohu I (N = 145) or Luohu II (N = 155) technique were recruited. We compare the effectiveness of the Luohu II and one of Luohu I. Sexual satisfaction was checked by Female Sexual Function Index. RESULTS: There was no significant difference in the mean operation time, volume of intraoperative blood loss, time for the first passage of gas, sexual satisfaction (and hospital stay for patients in either group (P > 0.05). But patients in the Luohu II group had a significantly lower incidence of complications than patients in the Luohu I group. All patients had vaginal depths more than 9 cm over 3 months post-surgery. CONCLUSIONS: Compared with the traditional Luohu I laparoscopic peritoneal vaginoplasty, the Luohu II operation is easier to perform and causes less damage to the bladder and rectum. The physiological and anatomical features of the artificial vagina resemble the normal vagina in both techniques.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Ductos Paramesonéfricos/anormalidades , Peritônio/cirurgia , Vagina/cirurgia , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Cuidados Pós-Operatórios , Resultado do Tratamento
15.
Sci Rep ; 5: 12755, 2015 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-26227480

RESUMO

A wet-chemical method combined with spark plasma sintering was used to prepare a W-Y2O3 alloy. High-temperature tensile tests and nano-indentation microhardness tests were used to characterize the mechanical properties of the alloy. After He-ion irradiation, fuzz and He bubbles were observed on the irradiated surface. The irradiation embrittlement was reflected by the crack indentations formed during the microhardness tests. A phase transformation from α-W to γ-W was investigated by X-ray diffraction (XRD) and transmission electron microscopy (TEM). Polycrystallization and amorphization were also observed in the irradiation damage layer. The W materials tended to exhibit lattice distortion, amorphization, polycrystallization and phase transformation under He-ion irradiation. The transformation mechanism predicted by the atomic lattice model was consistent with the available experimental observations. These findings clarify the mechanism of the structural transition of W under ion irradiation and provide a clue for identifying materials with greater irradiation resistance.

16.
Arch Gynecol Obstet ; 290(4): 691-6, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24838288

RESUMO

PURPOSE: To investigate a better approach for androgen insensitivity syndrome (AIS) treatment using laparoscopic peritoneal or ileal vaginoplasty. METHOD: Improved laparoscopic peritoneal vaginoplasty and gasless laparoscopic ileal vaginoplasty were, respectively, applied to AIS patients in vaginal reconstruction. The operation condition and surgical results were summarized and follow-up was performed from the 5th week after operation, then once a month. The sexual function was scored by Female Sexual Function Index (FSFI), and FSFI score >30 represented a very good sexual function, 25-30 as good, and <25 as bad. RESULT: The operation time (including gonadal resection and genital plastic surgery) of improved laparoscopic peritoneal vaginoplasty was 77.2 ± 18.9 (55-105) min, while of ileal vaginoplasty was 183.3 ± 30.1 (155-215) min, longer than peritoneal vaginoplasty, and the bleeding amount was 7.2 ± 13.0 (5-30) versus 50.0 ± 30.0 (20-80) mL, respectively. The surgery was successful in all 12 cases without complications. During 1 month to 7 years follow-up, the depth of artificial vagina was approximately 8-12 cm after peritoneal vaginoplasty, while 6-11 cm after ileal vaginoplasty. The vaginal mucosa appeared as normal in all cases. Besides, 4/9 cases had normal FSFI score after peritoneal vaginoplasty while only 1/3 case after ileal vaginoplasty. CONCLUSION: The peritoneal vaginoplasty seems better than the ileal vaginoplasty because of the shorter operation time, less bleeding, rapid recovery, less trauma and scar, easy operating and early sexual life without mold. But the treatment should be individualized and comprehensive considering the patient anatomical characteristics and other factors.


Assuntos
Síndrome de Resistência a Andrógenos/cirurgia , Laparoscopia/métodos , Estruturas Criadas Cirurgicamente , Vagina/anormalidades , Vagina/cirurgia , Adulto , Perda Sanguínea Cirúrgica , Feminino , Seguimentos , Humanos , Íleo/cirurgia , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Peritônio/cirurgia , Estudos Retrospectivos , Comportamento Sexual , Adulto Jovem
17.
Zhonghua Zheng Xing Wai Ke Za Zhi ; 27(5): 343-7, 2011 Sep.
Artigo em Chinês | MEDLINE | ID: mdl-22259983

RESUMO

OBJECTIVE: To investigate the technique and therapeutic effect of laparoscopy-insisted vaginoplasty with peritoneum in patients with androgen insensitivity syndrome. METHODS: From May. in the Fifth People' s Hospital of Shenzhen. The therapeutic effect was retrospectively analyzed. RESULTS: Laparoscopy-insisted vaginoplasty was successfully completely with peritoneum in patients with androgen in 4 cases. Ileumtivity segyndroment was used instead of peritoneum in one case. Open operation was not adopted in any cases. The ectopic testicles were removed during operation in 4 cases. The average operation time and bleeding volume was 60 min and 20 ml, respectively. Rectum, bladder and urethra were not injured in any case. The average vaginal length was 9 cm (range 8-10 cm) 21-28 days after operation. 6 months after operation, the surface of reconstructed vagina was smooth, ruddy and flexible, with satisfactory anatomical and functional results. Normal sexual activity was achieved in 2 cases. CONCLUSIONS: Laparoscopy-insisted vaginoplasty with peritoneum could be used for female patients with androgen insensitivity syndrome. The ectopic testicles should be removed. Estrogen supplement and psychological guide after operation are also important.


Assuntos
Síndrome de Resistência a Andrógenos/cirurgia , Laparoscopia/métodos , Peritônio/cirurgia , Vagina/cirurgia , Adulto , Feminino , Humanos , Masculino , Procedimentos Cirúrgicos Reconstrutivos/métodos , Adulto Jovem
18.
Zhonghua Fu Chan Ke Za Zhi ; 41(7): 464-6, 2006 Jul.
Artigo em Chinês | MEDLINE | ID: mdl-17083812

RESUMO

OBJECTIVE: To investigate the feasibility of complicate myomectomy. METHODS: Six-seven patients with complicated uterine myomas undergoing laparoscopic myomectomy were retrospectively analyzed. The myomectomy was done using ureteral infravision imaging system or/and with self-made myoma segregate-stick. Among the total, there are 29 cases of multiple myomas (the number of myomas > or = 5) and 23 cases of single myoma (the diameter of myoma > or = 7cm, including 19 cases of intramural myoma, 4 cases of subserous myoma), 6 cases of myoma of broad ligament of uterus and 9 cases of cervical myoma. RESULTS: All cases were performed successfully laparoscopically. No intra-operative laparotomy or complications occurred. The average operating time and blood loss were (114 +/- 32) min and (114 +/- 78) ml respectively. The average time of hospital stay was 5.1 d. The average operating time and blood loss in the group (including multiple myoma group, intramural myoma group and cervical myoma) were significantly exceeded the other groups (including myoma of broad ligament of uterus group and subserous myoma group). CONCLUSIONS: Laparoscopic complicate myomectomy can be performed and the operation indication is enlarged using Ureteral Infravision Imaging System. Advancement in surgical instruments and expert operating skills are the key to operation success.


Assuntos
Procedimentos Cirúrgicos em Ginecologia/métodos , Laparoscopia/métodos , Leiomioma/cirurgia , Neoplasias Uterinas/cirurgia , Adulto , Perda Sanguínea Cirúrgica , Estudos de Viabilidade , Feminino , Humanos , Complicações Pós-Operatórias , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
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