Project description:Twelve cases of intracardiac tumours operated during the period 1975 to 1994 are presented. There were 8 males and 4 females in the age range of 18 years to 64 years. Tumours were located in the left atrium in 10 patients and in the right atrium in 2 patients. Standard cardiopulmonary techniques were followed. The right atrial tumours were approached through a right atrial incision while the left atrial tumours were approached through a bi-atrial trans-septal approach. One patient underwent a perineal urethrolithotomy for an impacted urethral calculus at the same sitting. Eleven of the tumour masses excised were myxomas confirmed on histopathology. The last patient, operated for synovial sarcoma of the left knee 2 years before, presented with a metastatic tumour mass in the left atrium. No deaths occurred in the series.
Project description:BackgroundTo what extent hospital use and medical resources are used on hemophilia care in China's health care system is unknown.ObjectivesThis study was based on a single center in China and was conducted to comprehensively assess the resource use for hospitalization of people with hemophilia.MethodsWe analyzed clinical characteristics, diagnosis, inhibitor status, reasons, length of stay, and hospital costs of 323 hospitalizations in which hemophilia must be considered as the main factor for hospitalization from January 2009 to December 2020 at the Institute of Hematology and Blood Diseases Hospital in Tianjin, China.ResultsThere were 265 hospitalizations for people with hemophilia A (HA) and 58 with hemophilia B (HB). Seventy-eight hospitalizations (24%) were for patients with inhibitor (INH+). Minor bleeding (eg, hemarthrosis, hematuria) was the most common reason for hospitalization. The cost of clotting factor concentrates was the major burden of inpatients with hemophilia. Total cost in a single hospitalization of a person with HA (median, 21,281 Chinese yuan [¥]) was about twice that for HB (median, ¥11,060). Expenditure of drugs in HA (median ¥14,157) was two to three times more than that in HB (median, ¥5707). Total cost and drug cost in hospitalizations of people with inhibitors were about two times more than these without (INH-) (median cost in INH+ hospitalizations: total cost, ¥27,303; drug cost, ¥20,445. Median cost in INH- hospitalizations: total cost, ¥17,743; drug cost, ¥11,973.).ConclusionsFor hemophilia, the most dominant cost during hospitalization was on clotting factor concentrates. Diagnosed HA and inhibitor positivity increased the global cost.
Project description:BackgroundGastrointestinal stromal tumor (GIST) is known for its wide variability in biological behaviors and it is difficult to predict its malignant potential. The aim of this study is to explore the characteristics and prognostic factors of GIST.MethodsClinical and pathological data of 497 GIST patients in our center between 1997 and 2012 were reviewed.ResultsPatients were categorized into very low-, low-, intermediate- and high-risk groups according to modified National Institutes of Health (NIH) consensus classification system. Among the 401 patients untreated with imatinib mesylate (IM), 5-year overall survival (OS) in very low-, low-, intermediate- and high-risk groups was 100%, 100%, 89.6% and 65.9%; and 5-year relapse-free survival (RFS) was 100%, 98.1%, 90.9% and 44.5%, respectively. Univariate analysis revealed that sex, tumor size, mitotic rate, risk grade, CD34 expression, and adjacent involvement were predictors of OS or RFS. COX hazard proportional model (Forward LR) showed that large tumor size, high mitotic rate, and high risk grade were independent risk factors to OS, whereas high mitotic rate, high risk grade and adjacent organ involvement were independent risk factors to RFS. The intermediate-high risk patients who received IM adjuvant therapy (n = 87) had better 5-year OS and RFS than those who did not (n = 188) (94.9% vs. 72.1; 82.3% vs. 56.3%, respectively). Similarly, advanced GIST patients underwent IM therapy (n = 45) had better 3-year OS and 1-year progression-free survival (PFS) than those who didn't (n = 42) (75.6% vs. 6.8%; 87.6% vs. 12.4%, respectively).ConclusionsVery low- and low-risk GISTs can be treated with surgery alone. Large tumor size, high mitotic rate, high risk grade, and adjacent organ involvement contribute to the poor outcome. IM therapy significantly improves the survival of intermediate-high risk or advanced GIST patients.
Project description:ObjectiveProtein-losing enteropathy (PLE) is a complication in some systemic lupus erythematosus (SLE) patients that is often misdiagnosed. With this study, we provide insight into clinical characteristics, laboratory characteristics, diagnostic tests, risk factors, treatment, and prognosis of the disease.MethodsA retrospective, case-control study was performed in 44 patients with SLE-related PLE (PLE group) and 88 patients with active SLE (control group) admitted to our care from January 2000-January 2012. Risk factors for SLE-related PLE were examined, and we analyzed the accuracy of single and combined laboratory characteristics in discriminating SLE-related PLE from active SLE. Serum albumin and C3 levels were measured as outcome during and after treatment with corticosteroids and immunosuppressive agents.ResultsThe PLE group had lower mean serum albumin and 24-hour urine protein levels, higher mean total plasma cholesterol levels, and greater frequencies of anti-SSA and SSB seropositivity compared with the control group. Anti-SSA seropositivity, hypoalbuminemia, and hypercholesterolemia were independent risk factors for SLE-related PLE. The simultaneous presence of serum albumin (<22 g/l) and 24-hour urine protein (<0.8 g/24 h) had high specificity, positive predictive value, negative predictive value, and positive likelihood ratio, a low negative likelihood ratio and no significant reduction in sensitivity. High dosage of glucocorticosteroid combined with cyclophosphomide were mostly prescribed for SLE-related PLE.ConclusionSLE-related PLE should be considered when an SLE patient presents with generalized edema, anti-SSA antibody seropositivity, hypercholesterolemia, severe hypoalbuminemia, and low 24-hour urine protein levels. Aggressive treatment for lupus might improve prognosis.
Project description:Background Bacterial meningitis is a serious central nervous system infection associated with high morbidity and mortality during the neonatal period, while the pathogen distribution was rarely reported on a large scale in China. This study aimed to investigate the distribution and change trends of neonatal bacterial meningitis pathogens in Children’s Hospital of Fudan University over the past 12 years. Methods This retrospective study included all cases diagnosed with neonatal bacterial meningitis and admitted to our hospital from 2009 to 2020. Results Totally 231 cases were enrolled, including 128 (55.4%) for male, 72 (31.2%) for premature infants, 48 (20.8%) for early-onset meningitis. The most common pathogens were Escherichia coli (E. coli) (39.0%) and Group B Streptococcus (GBS) (22.1%). Gram-negative bacteria were more common in preterm infants than in full-term infants (P=0.005). GBS was more common in term infants (P=0.000); Klebsiella pneumoniae (P=0.000) and Enterobacter cloacae (P=0.034) were more common in preterm infants. Gram-positive bacteria were more frequent in early-onset meningitis than in late-onset meningitis (P=0.002). Both E. coli (46.3% vs. 30.9%, P=0.017) and GBS (29.8% vs. 13.6%, P=0.003) increased, and Enterococcus (3.3% vs. 12.7%, P=0.008) decreased significantly in the epoch from 2015 to 2020 compared with the epoch from 2009 to 2014. Conclusions GBS and E. coli are the most common pathogens of neonatal bacterial meningitis in our hospital, and both have shown an upward trend over the past 12 years.
Project description:Background Renal cell carcinoma (RCC) with tumor thrombus extension into the right atrium (level IV) is a rare life-threatening clinical condition that can only be managed by means of a combined urological and cardiac surgical approach. The early and late outcomes of this radical treatment were analyzed in a large single-institution series over a period of 30 years. Methods In 37 patients with RCC and intracardiac tumor thrombus extension, nephrectomy was performed followed by the extraction of the intracaval and intracardiac tumor thrombus under direct visual control during deep hypothermic circulatory arrest (DHCA). Recently, in 13 patients, selective aortic arch perfusion (SAAP) was instituted during DHCA. Results In all patients, precise removal of the tumor thrombus was accomplished in a bloodless field. The mean duration of isolated DHCA was 15 ± 6 min, and 31.5 ± 10.2 min in the case of DHCA + SAAP, at a mean hypothermia of 22.7 ± 4°C. In-hospital mortality was 7.9% (3 patients). In Kaplan–Meier analysis, the estimated median survival was 26.4 months whereas the 5-year cancer-related survival rate was 51%. Conclusions Despite its complexity, this extensive procedure can be performed safely with a generally uneventful postoperative course. The use of cardiopulmonary bypass with DHCA, with the advantage of SAAP, allows for a safe, precise, and complete extirpation of intracaval and intracardiac tumor mass. Late outcomes after radical surgical treatment in patients with RCC and tumor thrombus reaching up in the right atrium in our series justify this extensive procedure.
Project description:BackgroundBlue rubber bleb nevus syndrome is a rare congenital disease characterized by multiple venous malformations in skin and gastrointestinal tract, not all patients have typical cutaneous lesions, refractory anemia may be the only clinical symptom, it is easy to miss diagnosis.MethodsA retrospective single center study was conducted on 8 patients with blue rubber bleb nevus syndrome from 2009 to 2021. Data were analyzed including clinical feature, diagnostic workup and results, gene detection, treatment and follow-up.ResultsFive children (62.5%) developed the disease in infancy, which initial symptoms were all cutaneous venous malformations. All children had chronic refractory anemia and gastrointestinal bleeding. Cutaneous lesions were observed in 87.5%, 1 child had multiorgan involvement. Gastrointestinal venous malformations were observed in 100%, lesions were more common in small intestine than in stomach or colon. No somatic mutation in TEK was found in our children. Diagnostic interval was on average 4.7 years. Eighty-seven-point-five percent children received at least one endoscopic or surgical intervention, however, those methods could not eradicate all the lesions and prevent relapse. Two children treated with sirolimus for more than 8 years, only 1 have satisfactory therapeutic effect. Besides, I child has growth retardation and emotional problems during follow-up.ConclusionsBlue rubber bleb nevus syndrome needs to be considered when find bluish nodular cutaneous lesions, chronic anemia or gastrointestinal bleeding of unknown origin. Capsule endoscopy is the most sensitive in diagnosing of this disease. Oral sirolimus at a relatively low dosage is effective, further comprehensively studies are required to evaluation of its efficacy, safety and the optimal dosage about the children.
Project description:(1) Background: The number of adnexal masses detected during pregnancy has increased due to the use of first-trimester screening and increasingly advanced maternal age. Despite their low risk of malignancy, other risks associated with these masses include torsion, rupture and labor obstruction. Correct diagnosis and management are needed to guarantee both maternal and fetal safety. Adnexal masses may be troublesome to classify during pregnancy due to the increased volume of the uterus and pregnancy-related hormonal changes. Management should be based on ultrasound examination to provide the best treatment. The aim of this study was to describe the ultrasound features of ovarian masses detected during pregnancy and to optimize and personalize their management with the expertise of gynecologists, oncologists and sonographers. (2) Methods: Clinical, ultrasound, histological parameters and type of management (surveillance vs. surgery) were retrospectively retrieved. Patient management, perinatal outcomes and follow-up were also evaluated. (3) Results: according to the literature, these masses are most frequently benign, ultrasound follow-up is the best management, and obstetric outcomes are not considerably influenced by the presence of adnexal masses. (4) Conclusions: the management of patients with ovarian masses detected during pregnancy should be based on ultrasound examination, and a centralization in referral centers for ovarian masses should be considered.
Project description:BackgroundAge-standardized mortality rates for metastatic colorectal cancer (mCRC) are highest among elderly patients. In current clinical guidelines, treatment recommendations for this patient population are based on a limited number of clinical trials.Patients and methodsIn this monocentric, retrospective analysis we characterized patients aged ≥70 years undergoing systemic therapy for mCRC and overall survival (OS) was investigated.ResultsWe included 117 unselected, consecutive mCRC patients aged ≥70 years undergoing systemic therapy for mCRC between February 2009 and July 2022. Median OS was 25.6 months (95% CI: 21.8-29.4). The median age was 78 years (range: 70-90) and 21%, 48%, 26% and 5% had an ECOG performance score of 0, 1, 2, and 3, respectively. The median number of systemic therapy lines was 2 (range: 1-5). The choice of first-line chemotherapy backbone (doublet/triplet versus mono) did not impact OS (HR: 0.83, p=0.50) or the probability of receiving subsequent therapy (p=0.697). Metastasectomy and/or local ablative treatment in the liver, lung, peritoneum and/or other organs were applied in 26 patients (22%) with curative intent. First-line anti-EGFR-based therapy showed a trend towards longer OS compared to anti-VEGF-based therapy or chemotherapy alone in left-sided mCRC (anti-EGFR: 39.3 months versus anti-VEGF: 27.3 months versus chemotherapy alone: 13.8 months, p=0.105). In multivariable analysis, metastasectomy and/or local ablative treatment with curative intent (yes versus no, HR: 0.22, p<0.001), the ECOG performance score (2 versus 0, HR: 3.07, p=0.007; 3 versus 0, HR: 3.66, p=0.053) and the presence of liver metastases (yes versus no, HR: 1.79, p=0.049) were independently associated with OS.ConclusionsOur findings corroborate front-line monochemotherapy in combination with targeted therapy as the treatment of choice for elderly mCRC patients with palliative treatment intent. Metastasectomy and/or local ablative treatment with curative intent are feasible and may improve OS in selected elderly mCRC patients.
Project description:Synopsis: Lymphovascular space invasion is an independent risk factor for disease progression and presence of tumor stalk an independent protective factor. Fertility sparing surgery may be acceptable in cases whose tumors present with stalks and without high risk factors. Objectives: The aim of the present study was to investigate the potential prognostic factors of uterine adenosarcoma. Methods: A total of 49 cases of uterine adenosarcoma were retrospectively reviewed at our institution between April 2006 and October 2018. Results: Median follow-up time was 34 months (range: 1-148). Median age was 47.50 years (19-75). Nineteen (38.9%) patients were uterine cervical adenosarcoma and 30 (61.22%) patients were uterine corpus adenosarcoma. Twenty-nine (59.2%) patients were polypoid with a stalk to the uterine cervix or corpus. Twenty-six (38.8%) patients were stage IA. Fifteen (30.6%) patients showed sarcomatous overgrowth. Six (12.2%) patients displayed lymphovascular space invasion (LVSI). Four (8.16%) patients had heterologous elements. In univariate analysis, Disease-free-survival (DFS) was associated with tumor location, presence of tumor stalk, heterologous elements, LVSI. In multivariate analysis, presence of tumor stalk remained an independently protective factor for recurrence (HR = 0.088, P = 0.005), and LVSI a risk factor for recurrence (HR = 11.953, P = 0.002). Fertility-sparing surgery (FSS) was performed in seven stage IA patients. When patients of stage IA analyzed separately, FSS was not significant with the DFS or OS. Conclusions: Presence of tumor stalk remained an independently protective factor for recurrence. Along with adequate counseling, FSS may be acceptable in cases whose tumors present with stalks and without high risk factors.