Objective To study the clinical and pathologic features,histological criteria and pathologic factors contributing to diagnosis of mixed epithelial and mesenchymal tumors(mixed m?llerian tumors,MMT)of the uterus.Methods A retrospective study of 102 cases of MMT of the uterus (74 adenofibromas including 9 recurrent cases,3 atypical polypoid adenomyomas,2 carcinofibromas,10 adenosareomas and 13 carcinosarcomas)was undertaken.Clinical records,gross features and tissue slices were reviewed.The follow-up data were analysed.Results The most common symptom was vaginal bleeding.Clinical signs included pelvic mass,uterine polyps,and enlarged uterus.Benign MMT usually presented as exophytic polypoid masses extending into the uterine cavity or protruding through the external os,often broad-based,lobulated and papillary.It was hard to distinguish low-grade malignant MMT from the benign ones by gross appearance.High-grade malignant MMT had the common gross features of carcinoma and sarcoma.Histologically,MMT showed a biphasic differentiation of mesenchymal and epithelial components.MMT were classified according to whether these elements were benign or malignant.Nine cases of adenofibroma without unique features for the diagnosis of adenosarcoma recurred at postoperative intervals of 3 to 96 months.Recurrent tumors were almost always confined to the original site.Conclusions Uterine MMT tumors according to WHO diagnostic criteria are not rare.The differential diagnosis depends on a multifactorial analysis.The recurrent adenofibromas may be a kind of borderline tumors with benign appearances and malignant behavior.

This study was aimed to investigate the genetic characteristics, identification method and transfusion strategy of rare blood type B(A). The rare blood group B(A) was typed by serological technique, PCR-SSP genotyping and sequencing of exon 6, 7 of ABO blood group. The genetic characteristics and molecular mechanism of B(A) blood group were also analyzed. Blood group compatibility test was conducted between blood donors of B(A) and recipients by clinical transfusion. The results showed that both forward and reverse grouping did not match the 3 cases of serological result in their family survey, while all of the 3 cases were grouped as AB blood group by forward grouping, B blood group by reverse grouping with serological result and B(A)04/001 group were genotyped by ABO genotyping. The patient of B blood group was transfused by 1 bag of washed red blood cells of donor of B(A) under closely monitoring, the patient's condition changed, and a mild adverse transfusion reaction was appeared. Washed red blood cell of O blood group was transfused into B(A) patient without blood transfusion reaction. It is concluded that the forward ABO serological grouping and reverse ABO serological grouping are not compatible, that may be verified by family survey and molecular biological methods. If in some cases transfusion therapy was applied, and group B(A) can not be transfused to the patient with group B or AB. Thus, transfusion compatibility or autologous transfusion can be adopted to transfuse to the patient from group B(A).
ABO Blood-Group System ; genetics ; immunology ; Adult ; Base Sequence ; Blood Grouping and Crossmatching ; Genotype ; Humans ; Male ; Transfusion Reaction

OBJECTIVETo study the clinicopathologic features, immunophenotype, differential diagnosis and prognosis of uterine epithelioid trophoblastic tumor(ETT).

METHODSFrom 2000 to 2007, 5 ETTs cases were diagnosed in the affiliated Women's Hospital, School of Medicine, Zhejiang University. The pathologic characteristics and immunophenotype of the tumors were analyzed by histological examination and immunohistochemistry of CK18, p63, inhibin-alpha, HCG, HPL, PLAP and Ki-67. The clinical prognostic factors were evaluated based on a following-up data with a period of 11 - 50 months.

RESULTSThe overall prevalence of ETT was 0.48% among all the gestational trophoblastic diseases patients received in the same period. Five ETT patients were in the reproductive ages with a median of 33 years. Histologically, the tumor showed an invasive, nodular growth consisting of uniform mononuclear trophoblastic cells. There were zones of hyaline material in the tumour nests. Necrosis was commonly seen with a characteristic geographic pattern. Immunohistochemically, all cases displayed a diffuse CK18 and p63 positivity, to be either positive focally or negative for HCG, HPL and PLAP staining. Inhibin-alpha staining was positive or negative either in the 5 cases. Two patients died of the tumour relapse: one died after 1 year with the tumor having a high mitotic activity (averagely 15 mitotic figures per 10 high-power fields), and the other died of lung metastasis 2 years after the diagnosis.

CONCLUSIONSETT is a rare trophoblastic disease with distinct clinicopathological features and immunostaining patterns. A high mitotic index and lung metastasis are indicators for an unfavorable prognosis.

Adult ; Alkaline Phosphatase ; metabolism ; Chemotherapy, Adjuvant ; Chorionic Gonadotropin ; metabolism ; Epithelioid Cells ; pathology ; Female ; Follow-Up Studies ; GPI-Linked Proteins ; metabolism ; Humans ; Hysterectomy ; Inhibins ; metabolism ; Isoenzymes ; metabolism ; Keratin-18 ; metabolism ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; secondary ; Membrane Proteins ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Placental Lactogen ; metabolism ; Pregnancy ; Trophoblastic Neoplasms ; drug therapy ; metabolism ; pathology ; secondary ; surgery ; Uterine Neoplasms ; drug therapy ; metabolism ; pathology ; surgery

Hennekam syndrome (HS) is a rare autosomal recessive syndrome characterized by defective lymphatic development. A 34-month-old boy with HS and who had unexplained developmental retardation and hypoalbuminemia as main clinical manifestations is reported here. He had a history of generalized edema and poor feeding. He was not thriving well. He manifested as facial anomalies (hypertelorism, flat nasal bridge and flat face), fracture of teeth, and superficial lymph nodes enlargement. He had low serum total protein, low serum albumin, and low serum immunoglobulin levels. Duodenal bulb biopsy revealed lymphangiectasia. Color Doppler ultrasound, magnetic resonance imaging and CT scan showed multi-site lymphangioma, and HS was thus confirmed. Mutations in CCBE1 and FAT4 have been found responsible for the syndrome in a part of patients. Diagnosis of the disease depends on the familial history, clinical signs, pathological findings and genetic tests.
Child, Preschool ; Craniofacial Abnormalities ; diagnosis ; etiology ; therapy ; Genital Diseases, Male ; diagnosis ; etiology ; therapy ; Humans ; Lymphangiectasis, Intestinal ; diagnosis ; etiology ; therapy ; Lymphedema ; diagnosis ; etiology ; therapy ; Male ; Syndrome

Autism spectrum disorder (ASD) is a complex neurodevelopmental disorder, with multiple genetic and environmental risk factors. The interplay between genetic and environmental factors has become the subject of intensified research in the last several years. Vitamin D deficiency has recently been proposed as a possible environmental risk factor for ASD. Vitamin D has a unique role in brain homeostasis, embryogenesis and neurodevelopment, immunological modulation (including the brain's immune system), antioxidation, antiapoptosis, neural differentiation and gene regulation. Children with ASD had significantly lower serum levels of 25-hydroxy vitamin D than healthy children.Therefore vitamin D deficiency during pregnancy and early childhood may be an environmental trigger for ASD.
Child Development Disorders, Pervasive ; etiology ; genetics ; Embryonic Development ; Homeostasis ; Humans ; Vitamin D ; physiology ; Vitamin D Deficiency ; complications

Objective To examine the effects of gross tumor volume (GTV) and radiation dose on the prognosis of hepatocellular carcinoma (HCC) patients treated with whole body gamma knife.Methods The clinical data of 69 HCC patients who underwent body gamma knife treatment from January 2012 to June 2015 in the Radiotherapy Center of the PLA General Hospital were retrospectively reviewed.Based on a 50% or 60% isodose coverage of the planning target volume (PTV), patients were treated with a radiation dose of 4-5 Gy per fraction, and a total marginal dose of 36-50 Gy (median dose 45 Gy).Short-term efficacy, overall survival (OS), and the adverse effect of the treatment were evaluated.The optimal cut-off tumor volume was identified using the receiver operating characteristic curve, and survival was determined by the Kaplan-Meier method.Univariate and multivariate analyses were performed using the log-rank test and Cox proportional hazards regression model, respectively.Results The overall short-term response rate of the 69 patients was 67%.The 1-and 2-year OS rates were 62% and 40%, respectively, with a median survival of 18.6 months.The multivariate analysis showed that gross tumor volume (GTV)<93 cm3(P=0.013) and short-term efficacy of radiotherapy (P=0.000) were significant independent prognostic factors for survival.When GTV was<93 cm3, prognosis was significantly better in patients treated with a dose of ≥45 Gy than in those with<45 Gy (P=0.019).In contrast, radiation dose had no significant effect on survival among patients with GTV>93 cm3(P=0.665).Conclusions GTV is an independent prognostic factor for overall survival of HCC patients.Although high-dose radiotherapy provides survival benefits to patients with small GTV, it is not necessarily suitable for patients with large GTV.

Objective:To investigate the role of microRNA (miRNA)-101a in the liver fibrosis induced by activated hepatic stellate cell (HSC), through upregulating IRE1α signaling pathway.Methods:Carbon tetrachloride (CCl 4) induced liver fibrosis model of mice was established. RT-PCR was used to measure the mRNA level of miRNA-101a in liver tissue of mice. Protein level of α smooth muscle actin(αSMA), collagen I and IRE1α were investigated by Western blot. It was divide into Vehicle, TGFβ1, TGFβ1+ miRNA-NC and TGFβ1+ miRNA-M groups. TGFβ1+ miRNA-NC and TGFβ1+ miRNA-M group were transfected with miRNA-101a mimic negative control and miRNA-101a mimic, respectively. After the corresponding treatments, mRNA level of miRNA-101a was detected by RT-PCR, the protein level of αSMA、collagen I and IRE1α were measured by Western blot. Results:Compared to normal mice, the fibrotic deposition in liver tissue of CCl 4 group was increased significantly [(0.17±0.06) vs. (2.09±0.39), P<0.001)]. Protein level of αSMA, collagen I and IRE1α was increased significantly in the model group [(1.00±0.23) vs. (4.09±0.80), (1.00±0.21) vs. (4.98±1.19), (1.00±0.24) vs. (3.27±0.65), all P<0.001)]. While the mRNA level of miRNA-101a was decreased (1.00±0.05) vs. (0.43±0.05), P<0.001). In vitro study, we found that TGFβ1 could inhibit the mRNA expression of miRNA-101a, induced HSC-T6 activation and then up-regulated protein expression of αSMA, collagen I and IRE1α. Compared to TGFβ1+ miRNA-NC group, the expression of miRNA-101a in TGFβ1+ miRNA-M group increased significantly [(0.59±0.19) vs. (1.89±0.20), P<0.001)]. The protein levels of αSMA, collagen I were reduced by over expression of miRNA-101a [(2.65±0.69) vs. (0.84±0.13), (3.15±0.59) vs. (1.31±0.25), all P<0.05)], and the protein content of IRE1α was down-regulated [ (2.63±0.47) vs. (1.03±0.15), P<0.001)]. Conclusion:miRNA-101a may play a critical role in the inhibition of HSC activation and liver fibrogenesis by blocking IRE1α signaling pathway.

Objective:To explore the mediating effect of expectations regarding aging between social support and frailty in elderly patients with chronic obstructive pulmonary disease (COPD), so as to promote better health education among medical staff and improve patients′ ability to cope with frailty.Methods:This was a cross-sectional study, the convenience sampling method was used to select 258 elderly COPD patients hospitalized in the Department of Respiratory Medicine and Geriatrics of Xinjiang Uygur Autonomous Region People′s Hospital from November 2021 to April 2022. They were investigated by the general information questionnaire, Fried Scale, Social Support Scale and Expectations Regarding Aging-21. Analyzed the correlation among social support, expectations regarding aging and frailty. AMOS 26.0 was used to investigate the mediating effect of expectations regarding aging between social support and frailty in elderly COPD patients.Results:The score of social support, expectations regarding aging and frailty in elderly patients with COPD was (30.45 ± 5.57), (35.25 ± 6.28) and (2.34 ± 1.71)points, respectively. There was negative correlation between social support and frailty in elderly COPD patients ( r=-0.541, P<0.01), while positive correlation between social support and expectations regarding aging ( r=0.477, P<0.01). The negative correlation between social support and expectations regarding aging ( r=-0.536, P<0.01). Expectations regarding aging played a partial mediating role between social support and frailty, with mediating effect value of -0.229, accounting for 35.39% of the total effect. Conclusions:Expectations regarding aging is a mediator variable between social support and frailty in elderly COPD patients. Health care providers can alleviate or even reverse patients' debilitating condition by improving their expectations regarding aging and social support.

Objective To evaluate the accuracy of Hadeco ES-1000spm hand-held doppler during the anterolateral thigh(ALT)flap harvest.Methods Twenty-five patients(26 sides)with ALT flaps for head and neck reconstruction between May 2005 and May 2010 received preoperative Doppler examination for the location of the cutaneous perforators of ALT flaps.The Doppler signals and body mass index(BMI)were recorded preoperatively according to ABC system.The locations of Doppler signals and of the actual cutaueous perforators at surgery were plotted and compared.The diameter of perforators was measured.Results One to three cutaneous perforators of the ALT flap were consistently found at specific locations.They were named perforators A,B,C from proximal to distal.Perforators A,B and C were present in 15(58%),24(92%)and 20(77%)cases and the diameter(>0.5 mm)of A,B and C were 11/15,22(92%)and 8(40%)respectively.The Doppler signal was within 0.5 cm of the actual perforator location in 85% flaps.The accuracy of Doppler decreased with increase of BMI.Conclusions Preoperative assessment by hand-held Doppler is useful in predicting the perforator vessels'locations and diameter although it's accuracy is limited.

BACKGROUNDInterventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome, but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava (IVC) and discusses potential surgical errors and postoperative complications.

METHODSOf the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium.

RESULTSExcept 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse.

CONCLUSIONSRadical correction is a beneficial therapy in the treatment of Budd-Chiari syndrome.

Adolescent ; Adult ; Budd-Chiari Syndrome ; pathology ; surgery ; Child ; Female ; Humans ; Male ; Middle Aged ; Treatment Outcome ; Vascular Surgical Procedures ; methods ; Vena Cava, Inferior ; surgery