Clinical manifestations and cerebral angiographic findings of moyamoya disease

Objective To study the clinical features and angiographic findings of moyamoya disease (MMD) as well as their relationship. Methods A total of 22 MMD patients received routine digital substraction angiography (DSA). The clinical manifestations and angiographic findings were analyzed. Results Clinical manifestations varied and each patient often had multiple symptoms,including cerebral infarction in 9 patients with an average age of 23.6 (13-39 years) and cerebral hemorrhage in 7 patients with an average age of 31.2 (28-46 years). Angiographic examination found that all the diseased sides showed MMD blood vessels. The patients who received encephalo-myo-arterio-synangiosis (EMAS) had better prognosis than those without receiving the treatment. Conclusion Cerebral infarction is frequent in children and adolescents with MMD,whereas cerebral hemorrhage is common in adults. DSA is a golden criterion for diagnosing MMD. Surgical treatment,EMAS blood supply reconstruction in particular,should be prescribed.

Clinicopathologic Characteristics and Survival of Patients with Rare Malignant Ovarian Yolk Sac Tumors:A Population-based Analysis

Yolk sac tumors (YSTs) are rare malignant germ cell tumors that usually affect youngfemales. To date, there have been few studies on YSTs. We evaluated the relationship betweenclinicopathologic characteristics of patients with ovarian YSTs and disease outcome based onSurveillance, Epidemiology, and End Results data. The K aplan-Meier method and log-rank testwere used to evaluate differences in survival rates. Data for 269 patients were analyzed. Theincidence of YSTs among ovarian germ cell tumors (OGCTs) cases was 0.4%;median patient agewas 22.0 years, and most tumors were unilateral. Patients presented with distant metastasis (37.5%),localized disease (49. 1%), and regional spread (8.9%). American Joint Committee on Cancer stagewas available for 13 patients (stage IA, n=2;stage IC, n=1;stage IA, n=1;stage IB, n=3;stageIlC, n=2;and stage IV, n=4). Survival rates at 1, 3, and 5 years were 91.0%, 84.0%, and 83.2%,respectively, for overall survival (OS) and 92.0%, 85.4%, and 84.5%, respectively, for disease-specific survival (DSS). The 5-year OS and DSS of patients with ovary tumors were 91.5% and92.9%, respectively, compared to 74.8% and 77.2%, respectively, for those with extra-ovarianspread (P<0.001 for both OS and DSS). Age >50 years was associated with shorter OS and DSS(both P<0.001), whereas no associatios of OS and DSS were observed with pathologic grade (P=0.49for OS and 0.52 for DSS). In summary, YSTs are typically unilateral, of a high grade, and localizedto the ovary;extra-ovarian spread has a poor outcome, and postmenopausal women have worseprognosis than premenopausal women.

Venous thromboembolism in children with acute lymphoblastic leukemia in China:a report from the Chinese Children’s Cancer Group-ALL-2015

Venous thromboembolism(VTE)is a complication in children with acute lymphoblastic leukemia(ALL).The Chinese Children’s Cancer Group-ALL-2015 protocol was carried out in China,and epidemiology,clinical characteristics,and risk factors associated with VTE were analyzed.We collected data on VTE in a multiinstitutional clinical study of 7640 patients with ALL diagnosed in 20 hospitals from January 2015 to December 2019.First,VTE occurred in 159(2.08%)patients,including 90(56.6%)during induction therapy and 108(67.92%)in the upper extremities.T-ALL had a 1.74-fold increased risk of VTE(95%CI 1.08–2.8,P=0.022).Septicemia,as an adverse event of ALL treatment,can significantly promote the occurrence of VTE(P<0.001).Catheter-related thrombosis(CRT)accounted for 75.47%(n=120);and,symptomatic VTE,58.49%(n=93),which was more common in patients aged 12–18 years(P=0.023),non-CRT patients(P<0.001),or patients with cerebral thrombosis(P<0.001).Of the patients with VTE treated with anticoagulation therapy(n=147),4.08%(n=6)had bleeding.The VTE recurrence rate was 5.03%(n=8).Patients with VTE treated by non-ultrasoundguided venous cannulation(P=0.02),with residual thrombus(P=0.006),or with short anticoagulation period(P=0.026)had high recurrence rates.Thus,preventing repeated venous puncture and appropriately prolonged anticoagulation time can reduce the risk of VTE recurrence.

Clinical characteristics and risk factors for mortality in Candida auris infections

Background:Candida auris infections pose a threat to public health,necessitating increased awareness in China.This study aimed to analyze the strains of C.auris,assess the infection status,and investigate clinical characteristics and risk factors for mortality.Methods:A retrospective analysis was conducted on 18 patients with Candida auris infection.We focused on evaluating basic characteristics,strain sources,and antibacterial susceptibility test results.Statistical methods were used to determine clinical features and identify risk factors for death.Results:The strain type,composition ratio,and specimen source of C.auris were not associated with mortality.Neither the infection index nor the length of hospitalization showed an association with the prognosis.However,significant risk factors for mortality included cerebral infarction,cardiac disease,renal dysfunction,hypoproteinemia,and anemia(all p<0.05).Conclusions:Cerebral infarction,cardiac disease,renal dysfunction,hypoproteinemia,and anemia are significant risk factors for death in C.auris infections.These findings indicate the importance of recognizing and addressing these factors in the clinical management of C.auris infection.

Update on Mayer—Rokitansky—Küster—Hauser syndrome

This review presents an update of Mayer—Rokitansky—Küster—Hauser(MRKH)syndrome on its etiologic,clinical,diagnostic,psychological,therapeutic,and reproductive aspects.The etiology of MRKH syndrome remains unclear due to its intrinsic heterogeneity.Nongenetic and genetic causes that may interact during the embryonic development have been proposed with no definitive etiopathogenesis identified.The proportion of concomitant extragenital malformations varies in different studies,and the discrepancies may be explained by ethnic differences.In addition to physical examination and pelvic ultrasound,the performance of pelvic magnetic resonance imaging is crucial in detecting the presence of rudimentary uterine endometrium.MRKH syndrome has long-lasting psychological effects on patients,resulting in low esteem,poor coping strategies,depression,and anxiety symptoms.Providing psychological counseling and peer support to diagnosed patients is recommended.Proper and timely psychological intervention could significantly improve a patient’s outcome.Various nonsurgical and surgical methods have been suggested for treatment of MRKH syndrome.Due to the high success rate and minimal risk of complications,vaginal dilation has been proven to be the first-line therapy.Vaginoplasty is the second-line option for patients experiencing dilation failure.Uterine transplantation and gestational surrogacy are options for women with MRKH syndrome to achieve biological motherhood.

Differences in the prognostic value of tumor extent of resection among the molecular subgroups of medulloblastoma: A single centre study of 113 cases

Medulloblastoma(MB) is one of the most common pediatrics malignant tumors of the central nervous system. Studies in America and Europe have demonstrated significant differences in the prognostic value of medulloblastoma extent among molecular subgroups of medulloblastoma. However, studies focusing on the Chinese population are still lacking. A total of 113 patients with medulloblastoma who underwent surgical resection in Huashan Hospital between January 2002 and December 2013 were included in this study. Histological diagnoses were confirmed by 2 or more pathologists. Immunohistochemistry and CTNNB1 exon 3 mutation analysis were used to determine the different subgroups. Complete or incomplete resection was defined based on surgeons' reports and confirmed by postoperative computer tomography(CT). In this study, we included 113 patients with medulloblastoma(13 with WNT subgroup, 18 with SHH subgroup, and 82 with non-SHH/WNT subgroups) to assess their event-free and overall survival. We identified event-free survival and overall survival benefit for complete resection over incomplete resection. We found that for patients with NON-SHH/WNT medulloblastoma, incomplete resection was significantly associated with progression and overall survival compared with complete resection. To our best knowledge, the present study is the first to demonstrate the prognostic value of tumor extent of resection among the molecular subgroups of 113 medulloblastoma in the Chinese population.The prognostic benefit of the increased extent of resection for patients with medulloblastoma is attenuated after the molecular subgroups are taken into account. We still need further study to assess the benefit of surgical resection of small residual portions.