Objective:To report a case of hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis (POIKTMP), and analyze the genotype-phenotype correlation through a literature review.Methods:The clinical manifestations and genetic testing results of a Chinese Han child with POIKTMP were reported. Relevant literature was searched in databases using ′FAM111B gene′, ′hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis′ or ′POIKTMP′ as keywords, and the clinical manifestations, mutation sites of the FAM111B gene, and the correlation between them were statistically analyzed.Results:A 6.5-year-old girl developed POIKTMP at 6 months of age. Dermatological examination showed irregular brown patches and dotted hypopigmentation on the face and neck, mainly on the forehead and around the mouth, telangiectasia on the cheeks and nose, pigmentation and hypopigmentation on the limbs and trunk, as well as sparse, pale eyebrows. A total of 39 cases of POIKTMP were retrieved, including this case, all of which had clinical data and were definitively diagnosed. Fourteen variants of the FAM111B gene had been reported, including 1 in-frame deletion variant and 13 missense variants. Among the 39 cases, the incidence of poikiloderma/photosensitivity/facial erythema/telangiectasia was 100% (39/39), alopecia was 87.2% (34/39), and that of hypohidrosis/heat intolerance was 82.1% (32/39). The incidence of extracutaneous manifestations was as follows: tendon contractures/digital sclerosis, 69.2% (27/39) ; elevated liver transaminases, 46.2% (18/39) ; muscle pain/weakness/amyotrophy, 43.6% (17/39). The incidence of eczema-like lesions, bullous lesions, and elevated liver transaminases was significantly higher in the young versus the adult group ( P < 0.05) . Conclusions:This case of POIKTMP was characterized by brown patches, hypopigmentation, and sparse eyebrows. POIKTMP is a progressive multisystem disorder with age-related clinical manifestations. Early genetic testing is crucial for evaluating potential complications and providing genetic counseling.

Objective:To report a case of hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis (POIKTMP), and analyze the genotype-phenotype correlation through a literature review.Methods:The clinical manifestations and genetic testing results of a Chinese Han child with POIKTMP were reported. Relevant literature was searched in databases using ′FAM111B gene′, ′hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis′ or ′POIKTMP′ as keywords, and the clinical manifestations, mutation sites of the FAM111B gene, and the correlation between them were statistically analyzed.Results:A 6.5-year-old girl developed POIKTMP at 6 months of age. Dermatological examination showed irregular brown patches and dotted hypopigmentation on the face and neck, mainly on the forehead and around the mouth, telangiectasia on the cheeks and nose, pigmentation and hypopigmentation on the limbs and trunk, as well as sparse, pale eyebrows. A total of 39 cases of POIKTMP were retrieved, including this case, all of which had clinical data and were definitively diagnosed. Fourteen variants of the FAM111B gene had been reported, including 1 in-frame deletion variant and 13 missense variants. Among the 39 cases, the incidence of poikiloderma/photosensitivity/facial erythema/telangiectasia was 100% (39/39), alopecia was 87.2% (34/39), and that of hypohidrosis/heat intolerance was 82.1% (32/39). The incidence of extracutaneous manifestations was as follows: tendon contractures/digital sclerosis, 69.2% (27/39) ; elevated liver transaminases, 46.2% (18/39) ; muscle pain/weakness/amyotrophy, 43.6% (17/39). The incidence of eczema-like lesions, bullous lesions, and elevated liver transaminases was significantly higher in the young versus the adult group ( P < 0.05) . Conclusions:This case of POIKTMP was characterized by brown patches, hypopigmentation, and sparse eyebrows. POIKTMP is a progressive multisystem disorder with age-related clinical manifestations. Early genetic testing is crucial for evaluating potential complications and providing genetic counseling.

Objective To study the effects of Danggui-Chuanxiong herb pair medicine on vasoactive substances,adhesion factors,inflammatory factors,and VEGF-PI3K-AKT/NF-κB signaling pathways,in order to elucidate the mechanism of Danggui-Chuanxiong herb pair on the treatment of ischemic stroke(IS).Methods The oxygen glucose deprivation/reoxygenation(OGD/R)model of human umbilical vein endothelial cells(HUVEC)was constructed,and the cell viability was detected by cell proliferation kit(CCK-8 method)to explore the optimal modeling time of seven components;The release of lactate dehydrogenase(LDH)was detected by cytotoxicity kit;The expression of related cytokines was detected by enzyme-linked immunosorbent assay(ELISA);The mRNA expression of key proteins in the signaling pathway was detected by reverse transcription-polymerase chain reaction(RT-PCR).Results Reoxygenation after 6 h of oxygen-glucose deprivation of HUVEC is the best modeling time.High-dose chlorogenic acid group,ferulic acid group,senkyunolide H,low-dose and medium-dose butylidenephthalide group,medium-dose and high-dose senkyunolide A and ligustilide groups significantly decreased LDH leakage rate(P<0.05,P<0.01);The expression of IL-6 in the cells of the partial dose group of chlorogenic acid,caffeic acid,butenylphthalide,senkyunolide H and senkyunolide A was significantly increased,the expression of IL-1 in the cells of the partial dose group of chlorogenic acid,ferulic acid and senkyunolide A was significantly decreased,the expression of VEGF,ICAM-1 and VCAM-1 in the cells of the partial dose group of chlorogenic acid,ferulic acid and senkyunolide H was significantly decreased,the expression of NF-κB in the cells of the partial dose group of chlorogenic acid,ferulic acid,senkyunolide H and ligustilide was significantly decreased,the expression of PAI-1 in the cells of ferulic acid and senkyunolide H partial dose group decreased significantly(P<0.05,P<0.01);The mRNA relative expression levels of ERK,VEGF,NF-κB,VEGFR2 and MMP9 were significantly down-regulated in the cells of chlorogenic acid,ferulic acid,caffeic acid,butylidenephthalide and senkyunolide A partial dose group,while the mRNA relative expression levels of AKT were significantly up-regulated in the cells of senkyunolide H and senkyunolide A partial dose groups(P<0.05,P<0.01).Conclusion The medicinal components of Danggui-Chuanxiong herb pair may play a role in IS by inhibiting the mRNA expression of adhesion factor,inflammatory factor and key protein of VEGF-PI3K-AKT/NF-κB signaling pathway in HUVEC.

Abstract: Objective To analyze the influence of meteorological factors on Aedes larvae density in Haikou City, Hainan Province, and to study its lagged effect, providing a basis for monitoring and controlling dengue vectors. Methods The Aedes larvae monitoring data and meteorological factor data were collected from 2017 to 2022. The X11 model was employed to decompose and examine the basic characteristics of seasonality and trend in the Aedes aegypti larval density time series. The correlation between Aedes aegypti larval density and meteorological factors was assessed using the Spearman correlation coefficient test. A distributed lag non-linear model was employed to examine the lag non-linear impact of meteorological factors on Aedes larval density. The significance test of relative risk at the longest lag time and the principle of minimizing Akaike's information criterion were utilized to ascertain the optimal parameters of the model. Results The time-series analysis of Aedes larval density revealed distinct seasonality and a decreasing trend. The distribution lag non-linear modeling results indicated that, compared to the median precipitation of 120 mm, with the increase in monthly average precipitation, the relative risk of Aedes larvae density first increased and then decreased, showing an inverted "U" shaped non-linear relationship. The longest lag time of the average precipitation was 1 month, with a lag effect on Aedes larvae density when the precipitation ranged from 119.89 mm to 562.14 mm (P<0.05), peaking at 397.98 mm (RR=1.57, 95%CI: 1.11-2.20). Compared to a median temperature of 25.35 ℃, the relative risk to Aedes larvae density increased with the increase of monthly average temperature in a “J” shape. The longest lag time of average temperature was 1 month, with a lag effect on Aedes larvae density between 25.35 ℃ and 30.10 ℃ (P<0.05), peaking at 30.10 ℃ (RR=2.24, 95%CI: 1.24-4.05). Conclusions High precipitation and high temperature both have a risk effect on Aedes larvae density, with a 1-month lag effect. Timely knowledge of the effects of meteorological factors on Aedes larvae density can provide a basis for vector prevention and control and early warning of dengue fever.

Brain metastasis is a common and serious complication of breast cancer, which is commonly associated with poor survival and prognosis. In particular, the treatment of brain metastasis from triple-negative breast cancer (BM-TNBC) has to face the distinct therapeutic challenges from tumor heterogeneity, circulating tumor cells (CTCs), blood-brain barrier (BBB) and blood-tumor barrier (BTB), which is in unmet clinical needs. Herein, combining with the advantages of synthetic and natural targeting moieties, we develop a "Y-shaped" peptide pVAP-decorated platelet-hybrid liposome drug delivery system to address the all-stage targeted drug delivery for the whole progression of BM-TNBC. Inherited from the activated platelet, the hybrid liposomes still retain the native affinity toward CTCs. Further, the peptide-mediated targeting to breast cancer cells and transport across BBB/BTB are demonstrated in vitro and in vivo. The resultant delivery platform significantly improves the drug accumulation both in orthotopic breast tumors and brain metastatic lesions, and eventually exhibits an outperformance in the inhibition of BM-TNBC compared with the free drug. Overall, this work provides a promising prospect for the comprehensive treatment of BM-TNBC, which could be generalized to other cell types or used in imaging platforms in the future.

The liver has a complex cellular composition and a remarkable regenerative capacity. The primary cell types in the liver are two parenchymal cell populations, hepatocytes and cholangiocytes, that perform most of the functions of the liver and that are helped through interactions with non-parenchymal cell types comprising stellate cells, endothelia and various hemopoietic cell populations. The regulation of the cells in the liver is mediated by an insoluble complex of proteins and carbohydrates, the extracellular matrix, working synergistically with soluble paracrine and systemic signals. In recent years, with the rapid development of genetic sequencing technologies, research on the liver's cellular composition and its regulatory mechanisms during various conditions has been extensively explored. Meanwhile breakthroughs in strategies for cell transplantation are enabling a future in which there can be a rescue of patients with end-stage liver diseases, offering potential solutions to the chronic shortage of livers and alternatives to liver transplantation. This review will focus on the cellular mechanisms of liver homeostasis and how to select ideal sources of cells to be transplanted to achieve liver regeneration and repair. Recent advances are summarized for promoting the treatment of end-stage liver diseases by forms of cell transplantation that now include grafting strategies.
Humans ; Liver/surgery* ; Hepatocytes/transplantation* ; Stem Cells/metabolism* ; Liver Diseases/surgery*

Objective: To understand the possible transmission route of a family cluster of COVID-19 in Zhengzhou and the potential infectivity of COVID-19 in incubation period, and provide scientific evidence for the timely control of infectious source and curb the spread of the epidemic. Methods: Epidemiological investigation was conducted for a family cluster of COVID-19 (8 cases) with descriptive epidemiological method, and respiratory tract samples of the cases were collected for the nucleic acid detection of 2019-nCoV by RT-PCR. Results: Two primary cases, which occurred on 31 January and 1 February, 2020, respectively, had a common exposure history in Wuhan. The other six family members had onsets on 30 January, 31 January, 1 February (three cases) and 3 February, 2020. Conclusions In this family cluster of COVID-19, six family members were infected through common family exposure to the 2 primary cases. Five secondary cases had onsets earlier than or on the same day as the primary cases, indicating that COVID-19 is contagious in incubation period, and the home isolation in the early phase of the epidemic might lead to the risk of family cluster of COVID-19.

BACKGROUND/AIMS: Solitary hepatocellular carcinoma (HCC) is a subgroup of HCCs. We aimed to establish nomograms for predicting the survival of solitary HCC patients after hepatectomy. METHODS: A total of 538 solitary HCC patients were randomly classified into training and validation sets. A Cox model was used to identify predictors of overall survival (OS) in the training set. A nomogram was generated based on these predictors and was validated using the validation set. RESULTS: Tumor size, microvascular invasion, and major vascular invasion were significantly associated with OS in the training set. Nomograms were developed based on these predictors in the multivariate analysis. The C-index was 0.75 for the OS nomogram and 0.72 for the recurrence-free survival nomogram. Compared to the index of conventional staging systems for predicting survival (0.71 for Barcelona Clinic Liver Cancer, 0.66 for the seventh American Joint Committee on Cancer, 0.68 for Cancer of the Liver Italian Program, and 0.70 for Hong Kong Liver Cancer), the index of the OS nomogram was significantly higher. Moreover, the calibration curve fitted well between the predicted and observed survival rate. Similarly, in the validation set, the nomogram discrimination was superior to those of the four staging systems (p<0.001). CONCLUSIONS: The nomograms demonstrated good discrimination performance in predicting 3- and 5-year survival rates for solitary HCCs after hepatectomy.
Calibration ; Carcinoma, Hepatocellular* ; Discrimination (Psychology) ; Hepatectomy* ; Hong Kong ; Humans ; Joints ; Liver ; Liver Neoplasms ; Multivariate Analysis ; Nomograms* ; Prognosis ; Survival Rate

Objective To investigate the value of early?phase enhancement ratio combined with peripheral vascular diameter in the differential diagnosis of benign and malignant breast lesions using 3.0 T dynamic contrast?enhanced magnetic resonance imaging (DCE?MRI). Methods Sixty seven cases of patients (35 with malignant lesions and 32 with benign lesions in the breasts) were retrospectively analyzed. Their diagnoses were confirmed by surgery and pathology and all the patients underwent breast MRI plain scan and DCE?MRI in the two weeks before surgery. Lesion ROIs were drawn and time?signal intensity curves in the DCE?MRI were generated. Early?phase enhancement rate, time to peak, early?phase enhancement ratio, numbers of tumor vessel within 3 cm of the lesion and diameter of the largest vessel were recorded. Mann?Whitney U test was used to compare the difference of DCE?MRI between benign and malignant lesions, and the ROC curve was used to evaluate the efficiency of early?phase enhancement rate, early?phase enhancement ratio and vascular diameter in differentiating benign and malignant lesions. Results With breast malignant lesions, the medians of time to peak, early?phase enhancement rate, early?phase enhancement ratio, numbers of tumor vessel and vascular diameter were 2.2 s, 176.0%, 100.0%, 4 and 2.96 mm respectively, while with benign lesions of these parameters were 4.7 s, 113.3%, 81.9%, 0 and 0.00 mm respectively, and the differences were statistically significant (all P<0.05).When early?phase enhancement rate was used for differential diagnosis of breast benign and malignant lesions, the area under the ROC curve was 0.702 and the sensitivity and specificity were 82.86%and 56.25%with a threshold of 120.0%. When early?phase enhancement ratio was used, the area under the ROC curve was 0.854 and the sensitivity and specificity were 94.29% and 68.75% with a threshold of 86.0%. When peripheral vascular diameter was used, the area under the ROC curve was 0.896 and the sensitivity and specificity were 74.29%and 84.38% with a threshold of 2.78 mm. When early?phase enhancement ratio was combined with peripheral vascular diameter, the area was 0.925 and the sensitivity and specificity were 97.14% and 62.50%. Conclusion In the differential diagnosis of benign and malignant breast lesions under DCE?MRI, early?phase enhancement ratio combining with peripheral vascular diameter has improved sensitivity.

Objective To explore the clinicopathological characteristics of lupus nephritis (LN) with antinucleosome antibody (AnuA).Methods Data of 481 patients with biopsy-proven LN in the First Affiliated Hospital of Zhengzhou University from 2004 to 2011 were analyzed retrospectively.The patients were divided into two groups:AnuA-positive group (76 patients) and AnuA-negative group (405 patients).The clinical manifestations,laboratory examinations,histopathologic classes of LN,disease activity measured by SLE disease activity index (SLEDAI) of two groups were investigated and compared.Results There were 15 male patients in positive group (15/76,19.74％) with mean age of (27.99±10.88) years and 45 patients in negative group (45/405,11.11％) with mean age of (31.15±12.15) years respectively,which showed that male patients were more common in positive group (P＜0.05).Incidences of oral ulcer,fever,anemia,low complement and positive anti-dsDNA antibody were higher in positive group (P＜0.05).Percentage of diffuse proliferative lupus nephritis (class Ⅳ ) and pathological activity index (AI) in positive group were higher compared to negative group (all P＜0.05),while no significant differences of other pathological types,chronic index (CI) and SLEDAI were found between two groups.Conclusion LN patients with positive AnuA have special clinicopathological characteristics and AnuA may be used as a promising biomarker for the proliferative LN.