A Case Report of Multidisciplinary Diagnosis and Treatment of a Patient with Tuberous Sclerosis Complex and Multi-Organ Involvement / 罕见病研究

Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

A Case Report of Multidisciplinary Diagnosis and Treatment of a Patient with Tuberous Sclerosis Complex and Multi-Organ Involvement / 罕见病研究

Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

Expression and significance of immune checkpoint B7-homolog 4 in endometrial cancer / 中华妇产科杂志

Objective:To investigate the expression of B7 homolog 4 (B7-H4) and its clinical significance in endometrial cancer.Methods:A total of 833 patients with endometrial cancer admitted to Peking Union Medical College Hospital, Chinese Academy of Medical Sciences from 2009 to 2019, were enrolled. The expression of B7-H4, mismatch repair (MMR), p53, programmed cell death ligand 1 (PD-L1) protein, and CD 8+ T lymphocyte density in endometrial cancer tissues were detected by the EnVision two-step method of immunohistochemical staining. First-generation sequencing (Sanger method) was used to determine molecular subtyping of endometrial cancer. The χ2 test was used to compare the differences in positive expression rate of B7-H4 protein in endometrial cancer tissues with different clinicopathological features and molecular subtyping, PD-L1 protein expression, and CD 8+ T lymphocyte density. Survival analyses [including recurrence-free survival (RFS) and disease-specific survival (DSS)] were performed for 664 patients with follow-up time≥3 months, with a median follow-up time of 31 months (range: 4-121 months), and the Cox proportional hazards regression model was used to analyze the relevant factors affecting the prognosis of patients with endometrial cancer. Results:(1) The median age of 833 patients was 58 years (range: 25-88 years); pathological type: 595 with endometrioid carcinoma, 238 with non-endometrioid carcinoma; surgical-pathological staging: 542 cases at stage Ⅰ, 38 cases at stage Ⅱ, 173 cases at stage Ⅲ, and 45 cases at stage Ⅳ. Molecular subtyping was performed in 590 patients, including 50 with POLE mutation, 163 with mismatch repair defect (MMR-d) type, 246 with nospecific molecular change (NSMP) type, and 131 with p53 mutation subtype. (2) B7-H4 protein was expressed with brownish-yellow stainind in the cell membrane and cytoplasm of endometrial carcinoma, and the positivity rate of B7-H4 protein was 71.5% (596/833). The positivity rates of B7-H4 protein among patients with different age, surgical-pathological stage, tumor grade, pathological type, depth of muscular invasion, presence or absence of lymphovascular space invasion, and molecular subtype were significantly different (all P<0.05). The positivity rates of B7-H4 protein among patients with different PD-L1 protein expression and CD 8+ T lymphocyte density were not significantly different ( P>0.05). The 5-year RFS (83.9%) and DSS (87.3%) of B7-H4 protein-positive patients had an increasing trend compared with the 5-year RFS (77.2%) and DSS (78.1%) of B7-H4 protein-negative patients, but there were not statistically significant differences ( P=0.053, P=0.083). (3) Univariate analysis showed that the 5-year RFS and DSS of patients with different age, tumor grade, surgical-pathological stage, pathological type, depth of muscular invasion, lymphovascular space invasion, and molecular subtype were significantly different (all P<0.01). There were no significant differences in 5-year RFS ( P=0.184, P=0.113) and DSS ( P=0.549, P=0.247) among patients with different CD 8+ T lymphocyte density and PD-L1 protein expression. Further analysis according to molecular subtype, the results of CD 8+ T lymphocyte density and PD-L1 protein expression showed that the 5-year RFS and DSS of B7-H4 protein-positive patients were higher than those of B7-H4 protein-negative patients with NSMP subtype, low density of CD 8+ T lymphocyte and PD-L1 protein-negative endometrial carcinoma (all P<0.05), however, there was no significant difference in 5-year DSS between B7-H4 protein-positive patients and B7-H4 protein-negative patients with PD-L1 protein-negative endometrial cancer ( P=0.060). Multivariate analysis showed that positive expression of B7-H4 protein was an independent factor for 5-year RFS ( HR=0.27, 95% CI: 0.09-0.78, P=0.016) and DSS ( HR=0.16, 95% CI: 0.05-0.58, P=0.005) in patients with NSMP subtype endometrial carcinoma. In patients with low-density CD 8+ T lymphocytes endometrial cancer, positive expression of B7-H4 protein was an independent factor for 5-year RFS ( HR=0.45, 95% CI: 0.26-0.80, P=0.006), but it was not an independent factor for 5-year DSS. In patients with PD-L1 protein-negative endometrial cancer, B7-H4 protein was not an independent factor for 5-year RFS. Conclusion:B7-H4 protein expressed highly in endometrial carcinoma tissues, and its high expression is closely related to clinicopathological features, molecular subtype of p53 mutant and NSMP, and the favorable prognosis of patients with low density of CD 8+ T lymphocyte immunophenotype endometrial carcinoma.

Morphologic improvements of trachea of children with congenital tracheal stenosis after slide tracheoplasty / 中华胸心血管外科杂志

Objective:To study the changes of stenotic segment trachea diameter, trachea length and carina angle before and after slide tracheoplasty.Methods:From January 2010 to January 2018, 77 children with congenital tracheal stenosis(female=36) with complete clinical data accepted slide tracheoplasty in Shanghai Children's Medical Center were selected as the observation group. The diameter of stenosis segment, the length of stenosis segment, the full length of trachea and carina angle were measured by CT image, and the ratio of stenosis segment length to full trachea length was calculated. A group of children with normal trachea morphology and the same sample number, and they had no significant difference in age, height and weight were selected as the control group.Results:The median operative age of 77 children in the observation group was 1.3(1.1, 1.9)years old, the height was 76.0(72.0, 83.0)cm, and the weight was 9.0(7.9, 10.8)kg. The age, height and weight of 77 children in the control group were 1.5(1.0, 2.0) years old and 78.0(71.0, 85.0) cm, 9.2(7.9, 11.0) kg respectively. After slide tracheoplasty, the diameter of trachea in the stenotic segment of children increased from 2.7 mm to 4.4 mm, which was increased by 63.0%( P<0.001), but still smaller than that of children in the control group(6.1 mm). The average length of trachea was shortened by 0.7 cm( P<0.001), and there was no significant difference between the total length of trachea(5.4 cm) and the total length of trachea(5.5 cm). The postoperative carina angle decreased by 27.9°( P<0.001), there was no significant difference in postoperative carina angle between the observation group(93.0°) and the control group(90.7°). Conclusion:The diameter of reconstructed trachea in children with CTS is significantly increased, the length and carina angle of trachea become smaller after slide tracheoplasty. The morphology of trachea is more similar to children in the control group.

Analysis of early death and long-term reoperation after 11-year single-center arterial switch operation / 中华胸心血管外科杂志

Objective:To analyze the early postoperative mortality and long-term reoperation of ASO and to explore the surgical risk and the reasons of long-term reoperation.Methods:The clinical data and follow-up data of ASO children undergoing surgery in Shanghai Children Medical Center from January 2010 to December 2020 were analyzed retrospectively. Four groups were divided into transposition of the great vessels(TGA/IVS) , transposition of the great vessels with ventricular septal defect(TGA/VSD) , Taussig-bing anomaly(TBA) , and two stage ASO(Ⅱ-ASO) groups. χ2 test was used to analyze the early mortality and long-term reoperation rates of ASO in different groups. Results:A total of 861 ASO patients were included in this study and 108 died early(12.5%, 108/861) . Seven hundred and fifty three cases were followed up and 102 cases were lost(13.5%, 102/753) . The median follow-up time was 7.23 years and the quartile interval was 4.74-9.37 years old. Sixty six patients(10.1%, 66/651) underwent long-term reoperation. Four patients(6%, 4/66) died after reoperation. In 241 cases of TGA/IVS, 24 cases(10%) were performed reoperation. In 256 cases of TGA/VSD, 23 cases(9%) had reoperation. In 126 cases of TBA, 18 cases(14.3%) for reoperation. And in 28 cases of Ⅱ-ASO only 1 case(3.6%) had reoperation. Among all the reoperation cases, there were 36 cases(2 deaths) for pulmonary angioplasty, 2 cases for pulmonary stenting, 10 cases for right ventricular outflow tract obstruction(RVOTO) repair, 2 cases for aortic valvularplasty, 5 cases for aortic valve replacement(2 deaths), 4 cases for aortic anastomotic stenosis repair, 3 cases for left ventricular outflow tract obstruction(LVOTO) repair, 2 cases for VSD residual shunt repair and 2 cases for coarctation of the aorta(CoA) correction.Conclusion:The early mortality rate of ASO surgery is still higher than that of developed countries. Long-term follow-up after ASO surgery should focus on right ventricular outflow tract stenosis and aortic valve insufficiency.

Clinicopathological and molecular characterization of high-grade endometrial carcinoma with POLE mutation: a single center study / 부인종양

Objective@#The molecular classification system of endometrial carcinoma (EC) in ‘The Cancer Genome Atlas’ is widely acknowledged for its prognostic utility. Subsequently, more simplified classification system that incorporate DNA polymerase epsilon (POLE) exonuclease domain mutations, mismatch repair deficiencies (MMRd), and abnormal p53 (P53abn) has also demonstrated its clinical utility. These classifications helped identifying a ‘POLE ultramutated’ (POLEmut) category of patients, most of whom show excellent prognoses despite having high-grade ECs. We aimed to investigate the clinicopathological and molecular characteristics of high-grade ECs with POLEmut. @*Methods@#We investigated 414 patients with high-grade ECs (including endometrioid carcinomas grade 3, serous carcinomas, clear cell carcinomas, mixed carcinomas, undifferentiated and dedifferentiated carcinomas, and carcinosarcomas) by sequencing and immunohistochemical staining. @*Results@#Forty-three tumors (10.4%) were classified as POLEmut, including 2 with new, possibly pathogenic POLE mutations at P286C and L424V. These patients had very good prognoses except for 1 with stage IV disease and residual tumor. Eleven patients in this group also had P53abn and 4 had MMRd; molecular analysis revealed that patients with synchronous POLE pathogenic mutation and other mutations had a POLEmut or MMRd phenotype; survival analysis found no difference in prognosis between these patient categories. The prognoses of patients in the POLEmut EC group were not significantly influenced by treatment or risk category. @*Conclusions@#Patients with high-grade EC exhibiting POLEmut have very good clinical outcomes, and should be identified urgently in daily work owing to their conflicting morphology. Our findings also provide guidance on subclassifying ECs with poor histological appearance.

Necessity and feasibility of mid - and long-term pulmonary valve reconstruction after tetralogy of fallot repair implemented in childhood / 中华胸心血管外科杂志

Pulmonary valve regurgitation is a common complication after radical surgery in children with tetralogy of fallot. Long-term presence of pulmonary valve regurgitation can lead to increased right heart load, and then increase the risk of arrhythmia, right heart failure, movement limitation and even sudden death. This review summarize and analyze the mid- and long-term pulmonary valve regurgitation, right heart function, and reconstruction effect after radical surgery in patients with tetralogy of fallot, in order to clarify the necessity and feasibility of long-term pulmonary valve reconstruction of children with tetralogy of fallot after radical surgery, and to briefly discuss the operation timing and indications.

Clinical assessment of the treatment for pulmonary artery sling combined with anomalous bronchial branching and congenital tracheal stenosis / 中华胸心血管外科杂志

Objective:To summarize the morphological characteristics and clinical experience of pulmonary artery sling(PAS) combined with anomalous bronchial branching, and provide new considerations for surgically treating PAS.Methods:A retrospective review of surgical experience of all patients with anomalous bronchial branching from January 2012 to December 2018 at Shanghai Children's Medical Center was conducted. There are 4 types according to the morphology of anomalous bronchial branching: tracheal bronchus, bronchial trifurcation, bridging bronchus, tracheal bronchus combined with bronchial trifurcation. The proportion of patients with anomalous bronchial branching combined with PAS was counted. The rate of tracheoplasty for four types was determined. We measured the interbronchial and subcarinal/subpseudocarinal angles before and after tracheoplasty.Results:140 patients were included in our study. The tracheal diameter of 11(21%, 11/53) patients with tracheal bronchus and 4(9%, 4/44) patients with bronchial trifurcation was normal. Among these patients, 125 patients had congenital tracheal stenosis(CTS). 115 patients underwent tracheoplasty. The rate of tracheoplasty of bridging bronchus was higher than tracheal bronchus(100% vs. 60%, P=0.000). One-stage surgery was performed in 76(61%) patients who had PAS. The interbronchial and subcarinal/subpseudocarinal angles were significantly reduced postoperatively( P=0.000). Conclusion:It is difficult to explain merely with vascular compression in patients with PAS with CTS and anomalous bronchial branching. One-stage slide tracheoplasty is currently the optimal option.

Advancein surgicaltreatment of congenital tracheal stenosis in children / 中华胸心血管外科杂志

Congenital tracheal stenosis is a rarebut life-threatening disorder. In recent years, the improvement of surgical skills has significantly reduced the incidence of postoperative complications and mortality in children. Through researching the relevant published articles and books, this review introduces the pathology, types, commonsurgical methods of congenital tracheal stenosis, summarizes the surgical indications for tracheoplasty, and prospects for providing some suggestions for thetreatment of congenital tracheal stenosis.

Advancein surgicaltreatment of congenital tracheal stenosis in children / 中华胸心血管外科杂志

Congenital tracheal stenosis is a rarebut life-threatening disorder. In recent years, the improvement of surgical skills has significantly reduced the incidence of postoperative complications and mortality in children. Through researching the relevant published articles and books, this review introduces the pathology, types, commonsurgical methods of congenital tracheal stenosis, summarizes the surgical indications for tracheoplasty, and prospects for providing some suggestions for thetreatment of congenital tracheal stenosis.

Construction of patient-level prediction model for in-hospital mortality in congenital heart disease surgery: regression and machine learning analysis / 中华胸心血管外科杂志

Objective:Explore a predictive model for predicting postoperative hospital mortality in children with congenital heart disease.Methods:We retrospectively analyzed the characteristics of all children with congenital heart disease from January 1, 2006 to December 31, 2017 at Shanghai Children's Medical Center. Each procedure was assigned a complexity score based on Aristotle Score. In-hospital death prediction models including a procedure complexity score and patient-level risk factors were constructed using logistic regression analysis and machine learning methods. The predictive values of the models were tested by C-index. Results:A total of 24 693 patients underwent CHD operations were include in the study, there were 585 (2.4%) in-hospital deaths. In-hospital mortality for each procedure varies between 0 to 77.8%, with 32 procedures with 0 death record. The prediction model constructed using logistic regression found that in addition to the complexity score, other risk factors included age, height, operation history, echocardiography characteristics as well as certain laboratory test results (mainly coagulation factors) were significantly correlated with in-hospital death. Receiver operating curve analysis showed that prediction with only the complexity score resulted in an AUC of 0.654 (95% CI: 0.628-0, 681, P<0.01) while model containing patient-level risk factors had significant higher prediction value with AUC of 0.886 (95% CI: 0.868-0.904, P<0.01). Training with machine learning method resulted in a final prediction model with high prediction value ( AUC 0.889, with a sensitivity value for death prediction of 0.817). The key risk factors in machine learning model are in general agree with the logistic regression model however with subtle differences. Conclusion:Through combination of procedure complexity score with pre-operative patient-level factors, predictive model constructed using regression or machine learning method had high accuracy in in-hospital mortality prediction.

The application of quantitative immune fecal occult blood test for the screening of colorectal cancer in health check?up participants / 中华健康管理学杂志

Objective To evaluate the application value of quantitative immune fecal occult blood test (FOBT) in colonoscopy for the screening of colorectal cancer in health check-up participants. Methods The subjects were selected from July 2017 to June 2018 in the Health Management Center of the Second Affiliated Hospital of Suzhou University. The subjects were the healthy individuals who chose quantitative immune FOBT or chemical method plus immunogold double-method FOBT (referred to as"double-method FOBT"), excluding those who had interfering factors. Individuals with a positive result in primary screening were selected and conducted with colorectal cancer by colonoscopy. If the polyploidy lesions were observed during colonoscopy, the biopsy or excision was performed, and the pathological diagnosis was performed. The positive rate of primary screening, compliance rate of colonoscopy and pathological results of colonoscopy were compared between the two methods. Quantitative immunoassay FOBT was analyzed in different gender, age group, physical examination nature, positive rate of primary screening, compliance rate of colonoscopy and pathological results of colonoscopy. Results 18 728 people chose quantitative immunoassay FOBT and 6 212 people chose double-method FOBT at the same time. There was no significant difference in gender and age between the two groups (all P>0.05), which was comparable. The detection rate of quantitative immune FOBT was higher than double-method FOBT (74.62% vs 32.23%, P<0.001). The positive rate of quantitative immune FOBT in primary screening was lower than double-method FOBT (4.11% vs 5.34%, P=0.003). The colonoscopy screening rate in positive population by quantitative immune FOBT was higher than double-method FOBT (27.83% vs 13.08%, P=0.001). These differences were statistically significant. The detection rate of total lesions by colonoscopy was 71.88% in positive population by quantitative immune FOBT. It was 42.86% in double-method FOBT. There was no statistical difference between the two methods (P=0.05). The detection rates of quantitative immune FOBT were significantly different among different genders, ages and physical properties (all P<0.001). The detection rate was higher in males than in females (79.14% vs 68.75%). The detection rate was highest in the group between 40 and 59 years old (79.96%). The individual detection rate was higher than the group (90.08% vs 66.07%). The positive rates in primary screening were significantly different among different ages (P=0.001).It was highest in the group aged 60 or above (5.59%). The colonoscopy screening rate in positive population by quantitative immune FOBT was highest in the group aged 50 or above (36.96%). The detection rate of inflammatory lesions were significantly different among different ages (P<0.001). The detection rate of colorectal cancer in males was higher than in females (11.11% vs 0.00%, P=0.009). In addition, with the increasing of fecal occult blood value, the detection rate of cancer was increased (P=0.041). Conclusion The quantitative immune FOBT is an ideal non-invasive examination for early screening of colorectal cancer. It has important application values.

Extrauterine adenomyoma of the liver with a focally cellular smooth muscle component occurring in a patient with a history of myomectomy: case report and review of the literature.

Since first reported in 1986, 14 cases of extrauterine adenomyoma have been reported in the English literature, most often occurring in the ovaries. In this report, we present the first case of extrauterine adenomyoma involving the liver in a 29-year-old woman who presented with a 2-year history of low back pain with recent worsening and a history of laparoscopic myomectomy 5 years previously. Gross inspection of the specimen revealed a subcapsular mass that had a well-circumscribed margin with the adjacent liver tissue. By histopathologic examination, the multilobular mass was composed of a smooth muscle component and benign endometrioid glands and stroma. The smooth muscle component was focally cellular, and the endometrioid glands had secretory features. Both the smooth muscle component and endometrioid tissue were positive for ER and PR. The smooth muscle component was also positive for desmin and SMA, while the endometrioid stroma was positive for CD10. Other extrauterine lesions composed of a mixture of smooth muscle tissue and heterotopic endometrioid tissue, including endometriosis with a smooth muscle component, leiomyomatosis/leiomyomas associated with endometriosis and uterus-like masses, should be included in differential diagnoses. The patient was free from recurrence 5 months after liver tumor resection. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1327125766102291.

Analysis of individual dose monitoring results among radiation workers in a first-class hospital at Grade 3 from 2010 to 2017 / 中华劳动卫生职业病杂志

Objective@#To understand the occupational external exposure dose among radiation workers in a first-class hospital at Grade 3 of Suzhou, and to provide reference for radiological protection.@*Methods@#The individual dose of 1156 radiation workers in the hospital from 2010 to 2017 were detected, the annual collective effective dose and per capita annual effective dose were analyzed for different years, different occupations (diagnostic radiology, radiotherapy, nuclear medicine, interventional radiology) , gender, and age.@*Results@#From 2010 to 2017, the total annual collective effective dose was 351.40 person·mSv, the per capita annual dose was 0.30 mSv/a, and radiation workers whose annual effective dose was less than 1 mSv accounted for 94.98%. There were 5 interventional radiology workers and 1 nuclear medicine worker with annual effective dose between 2 and 4 mSv. There was no worker with annual effective dose over 4 mSv. The per capita annual effective dose of nuclear medicine workers was the highest (0.40 mSv/a) . The per capita annual effective dose was not significantly different between radiation workers with different genders and ages (P>0.05) .@*Conclusion@#Most of radiation workers have low individual dose level in the hospital. It is important to focus on nuclear medicine workers and interventional radiology workers.

Lymphomatoid gastropathy: one case report and literatures review / 中华血液学杂志

Objective@#To report the first case of lymphomatoid gastropathy in China, and to demonstrate the clinical characteristics, diagnostic approach, treatment and prognosis in this kind of patients.@*Methods@#One patient was diagnosed as lymphomatoid gastropathy at Peking Union Medical College Hospital, and her clinical characteristics, lab data, treatment and follow-up outcomes were reviewed.@*Results@#A case of a 51-year-old female was presented, who underwent esophagogastroduodenoscopy (EGD) due to slight epigastric discomfort. EGD revealed multiple ulcers and erosions. Biopsies showed atypical lymphocytes infiltration with CD3(+), CD56(+), CD20(-), CD8(-), TIA(+), Granzyme B(-) and Ki-67 (75%). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Four months later, repeated EGD examination showed regression of the lesions without specific treatment.@*Conclusion@#Lymphomatoid gastropathy was a unique disease entity mimicking NK/T-cell lymphomas in pathology, with the quite different profile of treatment and prognosis. It’s important to consider this issue during the differential diagnosis to avoid any excessive treatment.

Clinicopathologic features of three cases of erythropoietic protoporphyria with liver involvement / 中华病理学杂志

Objective@#To investigate the clinicopathologic features of the erythropoietic protoporphyria (EPP) with liver involvement.@*Methods@#The clinical findings and hepatic biopsy of 3 cases of EPP diagnosed between July, 2011 to August, 2014 with liver involvement were reviewed, with relevant literature review.@*Results@#All patients presented with persistent and refractory abdominal pain, with obvious jaundice and deranged liver function. Imaging showed homogeneous hepatomegaly in all patients. Histologically, the hepatocytes were edematous, and contained numerous cytoplasmic globular brown pigments and bile pigments, which were also found in Kupffer cells, in the bile canaliculi and in some of dilated sinusoid. The pigments were of different sizes and showed uneven distribution. Some pigments showed bright red or yellow birefringence with a distinctive central maltese cross configuration on polarizing microscopy. Furthermore, some hepatocytes showed piecemeal necrosis and steatosis, the portal tracts were usually infiltrated by lymphocytes, with fibroplasia and biliary ductular reaction. There was no dilatation of intrahepatic bile ducts.@*Conclusion@#Full understanding of the clinical and pathological features of EPP with liver involvement can help to recognize this small group of patients, and to offer proper effective treatments.

Effect of Simvastatin on expressions of myocardial chromogranin A and apoptosis signal-regulating kinase 1 of immature rabbits with chronic heart failure / 中华实用儿科临床杂志

Objective To observe the influence of Simvastatin on immature rabbit model of chronic heart failure(CHF),and to explore the possible protective mechanism of Simvastatin for the rabbits with CHF.Methods Thirty immature male rabbits were divided into 3 groups randomly:control group,heart failure group (HF group)and Simvastatin group(SIM group),10 rabbits in each group.The models of CHF were established by injecting Adrinmycin via the auricular vein of rabbits (1.5 mg/kg,once 1 week,for 12 weeks).The control group were injected the same amount of 9 g/L saline.SIM group were given both injection of Adrinmycin and Simvastatin [1.5 mg/(kg · d)for 12 weeks].The immature rabbit's cardiac function and myocardial morphology changes were evaluated.The expressions of chromogranin A (CgA) and apoptosis signal-regulating kinase 1 (ASK1) were evaluated.Results (1) In control group,the immature rabbits were all alive;in the other 2 groups,most immature rabbits were depressed and sluggish.The survival rate of HF group was 60％,while in SIM group the survival rate was 80％.(2)Compared with the control group,the left ventricular ejection fraction in HF group and SIM group decreased significantly [(40.05 ± 6.74)％,(50.18 ± 5.73) ％ vs.(65.93 ± 5.65) ％,all P ＜ 0.01],while in SIM group was higher than that of HF group,and the difference was significant (P ＜ 0.05);compared with HF group,the left ventricular end diastolic diameter and left ventricular end systolic diameter decreased in SIM group [(13.48 ± 1.24) mm vs.(16.23 ± 2.82) mm;(9.87 ± 0.85) mm vs.(11.13 ± 1.21) mm],and the differences were significant (all P ＜ 0.05).(3) Compared with the control group,the expression of CgA (mean optical density 142.24 ± 17.14,127.93 ± 12.12 vs.78.65 ± 6.78,P ＜ 0.05;integrated optical density 1 422.41 ± 167.34,1 279.37 ± 118.15 vs.786.54 ± 75.84,P ＜ 0.05) and ASK1 (mean optical density 140.32 ± 18.65,115.48 ± 12.30 vs.69.85 ± 6.54,P ＜ 0.05;integrated optical density 1 403.23 ± 165.67,1 158.79 ± 137.81 vs.698.58 ± 64.51,P ＜ 0.05) increased in the HF group and SIM group.While the expression in the SIM group decreased significantly compared with that of the HF group,and the difference was significant (P ＜ 0.05).Conclusions Simvastatin can improve cardiac function of immature rabbits with CHF.The mechanism of SIM may depress the expressions of CgA and ASK1.

An analysis of risk factors of open cardiac surgery in low body weight neonates / 中华胸心血管外科杂志

Objective To explore risk factors of cardiac surgery in neonates with low body weight.Methods Retrospective analysis was made in neonates weighing less than 3 kg at surgery(n =192;group 1) and 3 to 6 kg(n =517;group 2),who had undergone open cardiac repairs from January 2006 to December 2015 at our institution.Patients were grouped according to the primary procedure performed and analyzed according to their weight at the time of surgical intervention.Patients were also analyzed according to preoperative risk scores.Univariate versus multivariate risk analysis was performed.Results Hospital early mortality in group 1 was 25.0％ (n =48) versus 14.5 ％ (n =75) in group 2 (P =0.001).Compared with group 2,neonates in group 1 had a significantly higher mortality for simple arterial switch procedure.Lower body weight remained strongly associated with mortality risk after stratifying the population by preoperative risk scores category levels 2.Within group 1,age,weight at surgery,preoperative risk score and type of procedure were not associated with significant differences in early mortality.Cardio-pulmonary bypass time,aortic cross-clamp time and the presence of a surgical complication were independent risk factors for early mortality in group 1.Conclusion Among neonates weighing less than 3.0kg who underwent open cardiac surgery,perioperative hemodynamic status,reflected by bypass time,cross-clamp time,and surgical complications,strongly influenced early mortality.In contrast,low body weight itself was not associated with early mortality.

Intraductal papillary mucinous neoplasm of pancreas: analysis of the clinicopathologic features and prognosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic and immunohistochemical features, and the prognosis of intraductal papillary mucinous neoplasms (IPMN) of the pancreas.</p><p><b>METHODS</b>The clinical findings, morphologic features, immunophenotype and prognosis were investigated in 61 cases of IPMN.</p><p><b>RESULTS</b>Of these 61 cases, 33 were in the pancreatic head and 14 were in the body and tail, and 14 in the entire pancreas. The average patients' age was 61.8 years. The initial symptom was abdominal pain in 37 cases, and the tumors were detected at routine checkup in 14 cases. The imaging examination showed dilated ducts and/or cystic and solid masses. Grossly, 32 cases were multi-loculated cystic masses containing mucin and papillary areas; 13 cases were solid. Microscopically, the IPMN showed four patterns, including gastric-type (16 cases), intestinal-type (21 cases), pancreatobiliary-type (21 cases) and eosinophilic-type (3 cases). The IPMN cohort included 13, 13 and 6 IPMN with low, intermediate and high-grade dysplasia respectively, and 29 IPMN associated with invasive carcinoma. The IPMN associated carcinomas were mainly ductal adenocarcinoma (23/29, 79.3%), followed by colloid carcinoma (4/29, 13.8%) and undifferentiated carcinoma (2/29, 6.9%). Immunohistochemically, IPMN expressed MUC5AC (51/57, 89.4%), MUC2 (21/57, 36.8%), and MUC1 (13/46, 28.3%). The mean postoperative follow-up period was 32 months (range 12-112 months). Six of 61 patients were lost to follow-up. Overall 5-year survival rate was 76%. The 5-year survival rate of IPMN with low, intermediate or high-grade dysplasia was 100%, and recurrence was local in 3 patients. The 3-year survival rate of IPMN associated with invasive carcinoma was 55%. 12 of 13 patients died within 2 years after operation.</p><p><b>CONCLUSIONS</b>IPMN is a common cystic neoplasm of the pancreas located in the ducts. The pathologic types and classifications are clearly defined. MUC stains are helpful for the diagnosis and papillary typing. IPMN with invasive carcinoma was associated with significantly worse survival than IPMN with dysplasia.</p>

Clinical Value of Captopril Test for Primary Aldosteronism Diagnosis / 中国循环杂志

Objective: To evaluate the clinical value of Captopril test for diagnosing primary aldosteronism (PA) and to calculate the best cut-off point for PA diagnosis. Methods: We retrospectively analyzed 96 PA patients with conifrmed diagnosis by clinical situation, laboratory test and auxiliary examination in our hospital from 1994-06 to 2012-05, and meanwhile, studied 45 highly suspicious PA patients with final exclusion by confirmed diagnosis of primary hypertension (PH). All patients received the in-hospital Captopril test, the area under the curve of receiver operating characteristic (AUCROC) was applied to evaluate plasma aldosterone level and the ratio of aldosterone/renin after Captopril test and to obtain the best cut-off point with the corresponding sensitivity and speciifcity for PA diagnosis. Results: At 1h and 2h after Captopril test, AUCROC for plasma levels of aldosterone were 0.831 and 0.818, the ratios of aldosterone/rennin were 0.909 and 0.922 respectively. At 1h after Captopril test, the cut-off point of aldosterone level was 544.95 pmol/L and the diagnostic sensitivity was 70%, speciifcity was 90.7%; at 2h after Captopril test, the cut-off point of aldosterone level was 466.8 pmol/L and the diagnostic sensitivity was 69.8%, speciifcity was 70.5%. At 1h after Captopril test, the ratio of aldosterone/rennin was 34.6 [ng/dl: μg/(ml·h)] with the sensitivity at 78.3% and speciifcity at 88.4%. At 2h after Captopril test, the maximum AUCROC for the ratio of aldosterone/rennin was obtained, when cut-off point of aldosterone level was 42.2[ng/dl: μg/(ml·h)] , the diagnostic sensitivity was 76.7%, speciifcity was 95.3%. Conclusion: At 1h and 2h after Captopril test, plasma aldosterone level and the ratio of aldosterone/rennin had been valuable for PA diagnosis, the maximum diagnostic value could be obtained at 2h after Captopril test.