Mayer-Rokitansky-Küster-Hauser syndrome of Müllerian agenesis [corrected

Müllerian agenesis, a congenital malformation of the genital tract is the second most common cause of primary amenorrhoea. Its etiology is poorly understood. It may be associated with renal, skeletal or other abnormalities. The diagnosis is often made radiologically or laparoscopically. Three-dimensional ultrasound is a useful diagnostic tool. The hormonal profile and karyotype in these patients are normal. The management varies, but the treatment of choice is non surgical aimed at creating a neovagina. These patients require psychological support due to the implications for reproduction. Here, we present the case of a 24-year-old married woman with primary amenorrhoea with this malformation.     

Müllerian adenosarcoma with sarcomatous overgrowth of the cervix: unusual large polypoid mass

Müllerian adenosarcoma (MS) is a rare neoplasm of uterine cervix composed of benign epithelial and malignant stromal components. An aggressive variant of adenosarcoma, müllerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare. The difference between MS and MASO is the pure high grade sarcoma features in MASO. In this report we present a MASO case, derived from uterine cervix of a 60 year-old-female patient presenting as a cervical polypoid mass, to our knowledge the second case of the English literature. In spite of sarcomatous overgrowth, high mitotic activity and huge tumor size of 12,5 cms, it displayed no myometrial invasion, vascular invasion and heterologous elements. The patient has been clinically free of disease for 14 months of follow up after total abdominal hysterectomy and bilateralsalpingo-oopherectomy. The difficulties in diagnosis and treatment of this entity will be evaluated in this report.     

Primary omental malignant mixed Müllerian tumor in a 67-year-old woman

背景：大网膜原发苗勒氏管混合瘤很少见,迄今为止仅报道了2例,具有其特殊临床特征。病例：女性,67岁,G6P5,主因腹痛、腹胀15天就诊于北京大学人民医院妇科。体格检查发现一直径12厘米腹部包块,活动度差。行肿瘤及大网膜大部分切除,全子宫加双附件切除术。术后病理示大网膜原发恶性苗勒氏管混合瘤。术后顺铂、吡喃阿霉素、足叶已甙化疗一疗程,因严重骨髓抑制,未继续化疗,术后8个月发现肿瘤肝转移。结论：原发性恶性苗勒氏管混合瘤预后差,现今手术仍是最有效的治疗手段,化疗及放疗效果不确切。     

Catheter ablation of atrial fibrillation in a patient with dextrocardia: what is the challenge?

Catheter ablation has been an established strategy for treating paroxysmal atrial fibrillation （AF）.Pulmonaryvein isolation is the predominant approach of catheter ablation. This procedure is characterized as transseptal catheterization and point-by-point ablation around the ipsilateral pulmonary veins （PVs）. Although catheter ablation can be safely performed in a heart with normal structures, it may be challenging to be performed in a dextrocardia.     

Increased procoagulant activity of red blood cells in the presence of cisplatin

Background Cisplatin based chemotherapy is a well recognized risk factor for coagulation disorders and thrombosis. The pathophysiological mechanisms by which cisplatin promote thrombosis are not well understood. Methods Red blood cells （RBCs） were separated from peripheral blood of patients with breast cancer （n=10） and healthy adults （n=6） and treated with cisplatin. Coagulation time of RBCs was assessed by one step recalcification time and the productions of thrombin, intrinsic and extrinsic factor Xa were measured in the presence or absence of various concentrations of lactadherin. Exposed phosphatidylserine was stained with lactadherin and observed by confocal microscopy and flow cytometry. Results Neither fresh RBCs nor RBCs treated without cisplatin had potent procoagulant activity. Cisplatin treatment increased procoagulant activity of RBCs in a cell number- and concentration-dependent manner. Exposed phosphatidylserine was stained with lactadherin and after cisplatin treatment, strong fluorescence was revealed by confocal microscopy. Lactadherin bound RBCs from patients with breast cancer increased from （1.9＋0.5）% on control RBCs to （68.0±3.5）% on RBCs treated with 10umol/L cisplatin for 24 hours. Conclusions Cisplatin treatment increases procoagulant activity of RBCs, which have a strong association with exposure of phosphatidylserine. The increased procoagulant activity may contribute to the pathogenesis of thrombophilia during cisplatin based chemotherapy in breast cancer patients.     

Basal Serum Anti-Müllerian Hormone Level as a Predictor of Clinical Outcomes in Freezing-all Embryo Transfer Program

As a novel biomarker, there is inconsistent evidence regarding the association between anti-Müllerian hormone (AMH) and live birth rate in freezing-all embryo transfer cycles. We aim to assess the prognostic effect of baseline AMH on clinical outcomes, especially live birth rate in freezing-all embryo transfer cycles. A total of 828 non-polycystic ovary patients that underwent their first frozen-thawed embryo transfers in our center between January 2010 and January 2015 were recruited in this retrospective analysis. Patients were stratified into three groups based on their baseline AMH concentration: low AMH group (<1.4 ng/mL), middle AMH group (1.4–5.8 ng/mL) and high AMH group (>5.8 ng/mL). The results showed that low AMH level was associated with adverse clinical outcomes. The differences in implantation rate (21.9% vs. 43.2% vs. 58.8%, P<0.001), clinical pregnancy rate (32.0% vs. 55.2% vs. 65.7%, P<0.001), live birth delivery rate (21.8% vs. 43.6% vs. 52.7%, P<0.001) and miscarriage rate (31.8% vs. 17.5% vs. 15.4%, P=0.014) among the three groups were statistically significant. After adjusting confounders (i.e. age, baseline FSH level, AFC, endometrium thickness, endometrium preparation protocols, number of embryos transferred, etiologies of infertility), differences in live birth rate, clinical pregnancy rate and implantation rate between groups remained significant. The further age subgroup analysis demonstrated that low AMH concentration was significantly associated with poor outcomes both in young and advanced patients. The area under the curve for serum AMH, age, AFC and FSH were 0.635, 0.634, 0.615 and 0.543 respectively, for predicting live birth. In conclusion, baseline AMH was an independent prognostic factor of live birth rate of freezing-all embryo transfers, but its predictive value on live birth rate was of limited clinical value.     

Evaluation of non-invasive diagnostic tests in detecting cancer of the pancreas.

Monoclonal antibody defined antigen (CA19-9), carcinoembryonic antigen (CEA), ultrasonography (US) and computerized tomography (CT) were carried out as the tests in 113 patients suspected to have pancreatic cancer. Of 98 patients who underwent operation, 55 were found to have pancreatic cancer, 25 benign pancreatic disease, and 18 cancer of the biliary tract. Follow-up study was carried out for more than 1 year in 15 other patients suffering from pancreatitis without surgical intervention. The accuracy of CA19-9, US, CEA and CT was 87%, 69%, 61% and 56% respectively. The sensitivity of each test was CA19-9, 98%; US, 89%; CT, 80%; and CEA, 38%. The sensitivity of parallel test combined with CA19-9 and CEA was 100%. It may be regarded as the first step to detect pancreatic cancer in suspected population. The specificity of each test was CEA, 82%; CA19-9, 76%; US, 50% and CT, 40%. And the specificity of serial tests combined with CA19-9 and US was 90%. When these two tests were both positive, pancreatic cancer was highly suspected.

     

Primary choriocarcinoma in the anterior mediastinum in a man: a case report and review of the literatures

Primarymediastinalchoriocarcinomainmenisexceedinglyrareandisthoughttobeunresponsivetotreatment Wereviewtheliteraturesandreportacaseofmaleprimarychoriocarcinomaintheanteriormediastinum CASEREPORTA 19 year oldmanpresentedwithafever,coughandchestpainoverthepreviousweek HehadbeentreatedinalocalhospitalasStaphylococcusaureusinfectionwithoutimprovement ChestCT (Fig 1)showedalargemassintheanteriormediastinumaccompaniedbymultiplenodulesinbothlungfields On physicalexamination ,gynecomastiaanddecr…     

IFN-γ release assay: a diagnostic assistance tool of tuberculin skin test in pediatric tuberculosis in China

Background Prompt diagnosis of Mycobacterium tuberculosis （MTB） infection is an essential step in tuberculosis control and elimination. However, it is often difficult to accurately diagnose pediatric tuberculosis （TB）. The tuberculin test （TST） may have a low specificity because of cross-reactivity with antigens present in Mycobacterium bovis bacillus Calmette-Guerin （BCG） and other mycobacteria, especially in China with a predominantly BCG-vaccinated population.Early-secreted antigenic target 6-kDa protein （ESAT-6） and culture filtrate protein 10 （CFP-10）, stand out as suitable antigens that induce an interferon-gamma （IFN-γ） secreting, T-cell-mediated immune response to infection. While,considered the higher costs and complexity of the IFN-γ release assay （TSPOT）, we aimed to evaluate the TSPOT and TST test in the clinical diagnosis of pediatric tuberculosis and to establish a diagnostic process suitable for China.Methods The sensitivity and specificity of the assay were evaluated in total seventy four children with active tuberculosis and fifty one nontuberculous children with other disease, and then the results were compared with TST.Logistic regression models were used to identify variables that were associated with positive results for each assay. The independent variables included sex, age, birth place, vaccination history, close contract with an active TB patient.Results The sensitivity of TSPOT was higher than TST in active TB children with or without BCG vaccination, as well as in children with culture-confirmed TB. But the difference was not significant statistically. Combining results of the TSPOT and TST improved the sensitivity to 94.6%. Agreement of the TST and TSPOT was low （77.0%, k=0.203） in active TB patients. The difference in specificity between TSPOT and TST test was statistically significant （94.1% vs.70.6%, P=0.006）. Specificity of the two tests in patients without prior BCG vaccination history was similar （80.0% vs.60.0%）. The concordance between the two tests results in BCG vaccinated subjects was low （71.7%, k=0.063）. For TSPOT, none of the included risk factors was significantly associated with positive results. For TST, BCG vaccination （OR：1.78; 95% CI： 1.30-2.00） was significantly associated with positive results.Conclusions Although IFN-γ release assay had relatively high sensitivity and specificity, we also should consider the higher costs and complexity of this test. Therefore, TSPOT could be used as the complementary tool of TST in circumstances when a suspected patient with negative TST results, or to exclude a positive TST result caused by BCG vaccination.     

Rare cavernous lymphangioma of the bladder in an adult female：treatment and review of the literature

Objective： Lymphangioma is a benign tumor representing a congenital malformation of the lymphatic channels. The cavernous lymphangioma of the bladder did not report before. Here we report 1 case of cavernous lymphangioma of the bladder in an adult female and review the literature in order to accumulate the experience of diagnosis and treatment for these diseases. Methods： In our case a 50-year-old woman presented with irritative voiding symptoms and had painless macroscopic hematuria at times. Cystoscopy showed a non-papillary tumor at apex vesicae and mucous membrane hyperaemia between the two orifices within the trigone. The pathologic diagnosis was cystitis glandularis with hyperplasia of urothelium and cavernous lymphangioma in lamina propria. Transurethral resection was performed and then bleomycin A5 was injected into lesion multipoint applying ureteral catheter with puncture needle. Results： Two months after operation the irritative voiding symptoms improved and the urine analysis was normal. Cystoscopy showed no residual tumor. Bleomycin A5 was injected into the lesion area again just like the operation before. All symptoms disappeared completely when the patient was examined a month later. There was no side effect after injection and no recurrence during the follow-up of 1 year. Conclusion： Lymphangioma of the bladder can be diagnosed exactly by cystoscopy and pathological examination. Surgery may be the best treatment. The bleomycin A5 intralesional sclerosant is also an effective therapy for the disease after surgical removal.     

Serum CA125 changes in one case with malignant transformation from benign endometriosis to Müllerian adenosarcoma

To the editor： We here present a rare case of mullerian adenosarcoma arising in pelvic endometriosis and the changes ofserum CA125 level during the course of malignant changes. Serum CA125 was fobund being persistently abnormal during the course of endometriosis progression; and the fluctuation of CA125 seemed be relative to disease condition and could reflect the response to treatment.     

Serum CA125 changes in one case with malignant transformation from benign endometriosis to Müllerian adenosarcoma

To the editor:We here present a rare case of müllerian adenosarcoma arising in pelvic endometriosis and the changes of serum CA125 level during the course of malignant changes.Serum CA125 was found being persistently abnormal during the course of endometriosis progression;and the fluctuation of CA125 seemed be relative to disease condition and could reflect the response to treatment.     

Autopsy study of febrile deaths during monsoon at a tertiary care institute in India: Is malaria still a challenge?

Background:

To utilise an autopsy-based approach to study the febrile deaths and deaths due to malaria during monsoon period of three years at a tertiary care teaching hospital in Mumbai, India.

Materials and Methods:

All autopsies done at the hospital during monsoon period from 2005 to 2007 when fever was the main presenting symptom were included in the study. Monsoon period was defined from June to September. A study on the duration of hospital stay of malaria deaths was also attempted.

Results:

There were 202 autopsies of febrile illness during the study period. Malaria resulted in 20.8% of the deaths besides other causes. A majority of deaths had intrapulmonary haemorrhages as the only pathological finding. Incidence of malaria deaths was more during monsoon period than the non-monsoon period. Plasmodium falciparum was the most common species responsible for malaria deaths while cerebral malaria was the most common mode of death. In 27% of the cases, post-mortem examination helped to arrive at the correct final diagnosis. In 88.1% of the cases, malaria deaths occurred within the first 24 hours of admission to the hospital.

Conclusion:

The study reiterates the fact that malaria remains a preventable but major cause of death in India, predominantly during the monsoon period. The study also emphasises the importance of developing treatment protocols for malaria during such crucial times besides reinforcing the existing preventive measures.     

Urodynamic changes in a female case of obstructive sleep apnea syndrome with enuresis: 7 years’ follow-up

Obstructive sleep apnea syndrome （OSAS） is characterized by repetitive upper airway occlusion resulting in apnea lasting 10 seconds or more. Clinical manifestations include snoring, daytime somnolence, intellectual deficiency, sexual impotence, and nocturnal polyuria. Enuresis associated with OSAS is suggested to be more common in children than in adults） In children, removal of upper airway obstruction by surgical intervention （tonsillectomy and/or adenoidectomy） leads to a significant decrease in or complete cure of enuresis. Although a great deal has been written concerning aduk enuresis,