[Research progress in treatment of chronic prostatitis/chronic pelvic pain syndrome with traditional Chinese medicine].

Chronic prostatitis/chronic pelvic pain syndrome(CP/CPPS) is a common urological disease with complex etiology. The treatment effect of western medicine is not satisfactory, and the course of the disease is protracted, which brings great trouble to patients. Traditional Chinese medicine(TCM) has a variety of treatment methods based on syndrome differentiation and treatment, including internal treatment with TCM, acupuncture and massage, and other external treatment methods for comprehensive treatment, with significant effect. This study summarized the etiology and pathogenesis of CP/CPPS and found that western medicine cannot fully explain the etiology and pathogenesis of CP/CPPS. It was believed that CP/CPPS was mainly related to many factors such as special pathogen infection, voiding dysfunction, mental and psychological abnormalities, neuroendocrine abnormalities, immune abnormalities, excessive oxidative stress, pelvic diseases, and heredity. TCM believed that CP/CPPS was caused by damp heat, blood stasis, Qi stagnation, and poisoning and was closely related to the organs of the liver, spleen, kidney, lung, stomach, bladder, and meridians of Chong and Ren channels and three yin channels of the foot. In the treatment of TCM, multiple comprehensive treatment plans are currently used, including internal treatment with TCM(decoction, proprietary Chinese medicine, and unique therapies of famous doctors), acupuncture and massage treatment, and other external treatment methods(rectal administration, topical application of TCM, and ear acupoint pressure). Comprehensive regulation has significant clinical efficacy and prominent characteristics of TCM, and it is worth clinical promotion. This study aims to provide a reference for clinical prevention and treatment of CP/CPPS and points out potential directions for future research in this field.

[Role of NLRP3 inflammasome in prevention and treatment of cognitive impairment-related diseases and traditional Chinese medicine intervention: a review].

Alzheimer's disease(AD), vascular dementia(VD), and traumatic brain injury(TBI) are more common cognitive impairment diseases characterized by high disability and mortality rates, imposing a heavy burden on individuals and their families. Although AD, VD, and TBI have different specific mechanisms, their pathogenesis is closely related to the nucleotide-binding oligome-rization domain-like receptor protein 3(NLRP3). The NLRP3 inflammasome is involved in neuroinflammatory responses, mediating microglial polarization, regulating the reduction of amyloid ß-protein(Aß) deposition, neurofibrillary tangles(NFTs) formation, autophagy regulation, and maintaining brain homeostasis, and synaptic stability, thereby contributing to the development of AD, VD, and TBI. Previous studies have shown that traditional Chinese medicine(TCM) can alleviate neuroinflammation, promote microglial polarization towards the M2 phenotype, reduce Aß deposition and NFTs formation, regulate autophagy, and maintain brain homeostasis by intervening in NLRP3 inflammasome, hence exerting a role in preventing and treating cognitive impairment-related diseases, reducing psychological and economic pressure on patients, and improving their quality of life. Therefore, this article elucidated the role of NLRP3 inflammasome in AD, VS, and TBI, and provided a detailed summary of the latest research results on TCM intervention in NLRP3 inflammasome for the prevention and treatment of these diseases, aiming to inherit the essence of TCM and provide references and foundations for clinical prevention and treatment of cognitive impairment-related diseases with TCM. Meanwhile, this also offers insights and directions for further research in TCM for the prevention and treatment of cognitive impairment-related diseases.

[Prediction of Coagulation Factor â § Level in Chinese Hemophilia A Patients by the Pharmacokinetic Management Tool myPKFiT].

Objective To evaluate the performance of myPKFiT,a tool guiding the dosing of antihemophilic factor (recombinant) plasma/albumin-free method (rAHF-PFM),in maintaining the coagulation factor Ⅷ (FⅧ) level above a target threshold at the steady state and estimating the pharmacokinetics (PK) parameters in hemophilia A patients in China. Methods The data of 9 patients with severe hemophilia A in a trial (CTR20140434) assessing the safety and efficacy of rAHF-PFM in the Chinese patients with hemophilia A were analyzed.The myPKFiT was used to predict the adequate dose to maintain a patient's FⅧ level above target threshold at the steady state.Furthermore,the performance of myPKFiT in estimating the pharmacokinetics parameters of individuals was evaluated. Results Twelve combinations of two dosing intervals and six sparse sampling schedules were investigated,and 57%-88% of the patients remained the FⅧ level above the target threshold of 1 U/dl (1%) for at least 80% of the dosing interval.The clearance and time to FⅧ level of 1% obtained from sparse sampling by myPKFiT were similar to those obtained from extensive sampling. Conclusions The myPKFiT can provide adequate dose estimates to maintain the FⅧ level above the target threshold at the steady state in Chinese patients with severe hemophilia A.Moreover,it demonstrates good performance for estimating key pharmacokinetics parameters,including clearance and time to FⅧ level of 1%.

Development and evaluation of a direct TaqMan qPCR assay for the rapid detection of diverse carnivore amdoparvoviruses.

Amdoparvoviruses infect carnivore species, including mink, raccoon dog, fox, skunk, and red panda. Amdoparvovirus infection is a major cause of morbidity and mortality in farmed minks. Here, we developed a direct TaqMan qPCR assay for detection and quantification of carnivore amdoparvoviruses by using three primers and one probe based on the conserved VP2 gene. The detection limit for Aleutian mink disease virus (AMDV) and Raccoon dog and arctic fox amdoparvovirus (RFAV) were 4.06 × 101 copies/µl and 2.93 × 101 copies/µl, respectively. Both intra- and inter-assay variability were less than 2%. Among 74 carnivore samples, the positive rates for amdoparvoviruses were 62.2% (46/74) by direct TaqMan qPCR, while only 40.5% (30/74) by SYBR Green I qPCR. This result suggests that the direct TaqMan qPCR was more sensitive than the SYBR Green I qPCR. Additionally, the direct TaqMan qPCR is a rapid and sensitive method for liquid samples at microliter level as the assay employed the direct alkaline lysis method to obtain viral DNA and, therefore, eliminated the cumbersome steps in extracting DNA. Overall, the direct TaqMan qPCR assay possessed high specificity, sensitivity, and reproducibility, indicating that it can be used as a powerful tool for detection and quantification of various carnivore amdoparvoviruses in epidemiological and pathogenesis studies.

Analysis of the function of KIF3A and KIF3B in the spermatogenesis in Boleophthalmus pectinirostris.

Spermatogenesis represents one of the most complicated morphological transformation procedures. During this process, the assembly and maintenance of the flagella and intracellular transport of membrane-bound organelles required KIF3A and KIF3B. Our main goal was to test KIF3A and KIF3B location during spermatogenesis of Boleophthalmus pectinirostris. We cloned complete cDNA of KIF3A/3B from the testis of B. pectinirostris by PCR and rapid amplification of cDNA ends (RACE). The predicted secondary and tertiary structures of B. pectinirostris KIF3A/3B contained three domains: (a) the head region, (b) the stalk region, and (c) the tail region. Real-time quantitative PCR (qPCR) results revealed that KIF3A and KIF3B mRNA were presented in all the tissues examined, with the highest expression seen in the testis. In situ hybridization (ISH) showed that KIF3A and KIF3B were distributed in the periphery of the nuclear in the spermatocyte and the early spermatid. In the late spermatid and mature sperm, the KIF3A and KIF3B mRNA were gradually gathered to one side where the flagella formed. Immunofluorescence (IF) showed that KIF3A, tubulin, and mitochondria were co-localized in different stages during spermiogenesis in B. pectinirostris. The temporal and spatial expression dynamics of KIF3A/3B indicate that KIF3A and KIF3B might be involved in flagellar assembly and maintenance at the mRNA and protein levels. Moreover, these proteins may transport the mitochondria resulting in flagellum formation in B. pectinirostris.

Good outcome of severe lupus patients with high-dose immunosuppressive therapy and autologous peripheral blood stem cell transplantation: a 10-year follow-up study.

OBJECTIVES: This study aimed to examine the long-term efficacy, remission and survival of patients with severe systemic lupus erythematosus (SLE) after the combination treatment with high-dose immunosuppressive therapy (HDIT) and autologous peripheral blood stem cell transplantation (APBSCT). METHODS: Chinese patients with severe SLE receiving combination therapy with HDIT and APBSCT in Peking Union Medical College Hospital were enrolled from July 1999 to October 2005. Disease activity, treatment, and adverse effects of these patients were evaluated. The 10-year overall survival and 10-year remission survival were also analysed. RESULTS: Among the 27 patients, one patient failed to collect enough CD34+ cells and data was missing for two patients. In the end, 24 patients were included in the final analysis. After APBSCT, one patient died, two patients achieved partial remission and 21 (87.5%) achieved remission at 6 months. The median follow-up duration of the 23 patients was 120 months. Fourteen patients had completed a ten-year follow-up. The median proteinuria level of the 14 patients with LN with ten years of follow-up significantly decreased from 4.00 g/24 hours at pre-treatment to 0.00g/24 hours at year 5 and 0.00 g/24 hours at year 10 (both p=0.001). The 10-year overall survival rate and 10-year remission survival rate were both 86.0% (95% CI: 71.1-100.9%). After a median follow-up for 120 months, 16 patients (66.7%) remained in remission, 4 patients were lost to follow-up, 2 patients died and 1 patient remained active. CONCLUSIONS: The combination of HDIT and APBSCT may be an option to improve the survival of severe lupus patients.

Characterization of mitochondrial prohibitin from Boleophthalmus pectinirostris and evaluation of its possible role in spermatogenesis.

Prohibitin (PHB) is an evolutionarily conserved mitochondrial membrane protein. It plays a vital role in cell proteolysis, senescence, and apoptosis and is associated with spermatogenesis and sperm quality control in mammals. To study the characteristics of the PHB gene and its potential roles during spermatogenesis in Boleophthalmus pectinirostris, we cloned a 1153-bp full-length cDNA from the testis of B. pectinirostris with an open reading frame of 816 bp, which encodes 272 amino acid residues. Real-time quantitative PCR (qPCR) analysis revealed the presence of phb mRNA in all the tissues examined, with higher expression levels found in the testis, kidney, intestine, and muscle tissues. We examined the localization of phb mRNA during spermatogenesis by in situ hybridization (ISH), showing that phb mRNA was distributed in the periphery of the nucleus in primary and secondary spermatocytes. In spermatid and mature sperm, the phb mRNA gradually moved toward one side, where the flagellum is formed. Immunofluorescence (IF) results showed co-localization of the PHB and mitochondria at different stages during spermatogenesis of B. pectinirostris. The signals obtained for PHB decreased as spermatogenesis proceeded; the strongest detection signal was found in secondary spermatocytes, with lower levels of staining in other stages. Additionally, in the mature germ cells, the PHB signals were weak and aggregate in the midpiece of the flagellum.

The C-terminal kinesin motor KIFC1 may participate in nuclear reshaping and flagellum formation during spermiogenesis of Larimichthys crocea.

Spermatogenesis is a highly ordered process in the differentiation of male germ cells. Nuclear morphogenesis is one of the most fundamental cellular transformations to take place during spermatogenesis. These striking transformations from spermatogonia to spermatozoa are a result of phase-specific adaption of the cytoskeleton and its association with molecular motor proteins. KIFC1 is a C-terminal kinesin motor protein that plays an essential role in acrosome formation and nuclear reshaping during spermiogenesis in mammals. To explore its functions during the same process in Larimichthys crocea, we cloned and characterized the cDNA of a mammalian KIFC1 homolog (termed lc-KIFC1) from the total RNA of the testis. The 2481 bp complete lc-KIFC1 cDNA contained a 53 bp 5' untranslated region, a 535 bp 3' untranslated region, and a 1893 bp open reading frame that encoded a special protein of 630 amino acids. The predicted lc-KIFC1 protein possesses a divergent tail region, stalk region, and conserved carboxyl motor region. Protein alignment demonstrated that lc-KIFC1 had 73.2, 49.8, 49.3, 54.6, 56.5, 53.1, and 52.1% identity with its homologs in Danio rerio, Eriocheir sinensis, Octopus tankahkeei, Gallus gallus, Xenopus laevis, Mus musculus, and Homo sapiens, respectively. Tissue expression analysis revealed that lc-kifc1 mRNA was mainly expressed in the testis. The trend of lc-kifc1 mRNA expression at different growth stages of the testis showed that the expression increased first and then decreased, in the stage IV of testis, its expression quantity achieved the highest level. In situ hybridization and immunofluorescence results showed that KIFC1 was localized around the nucleus in early spermatids. As spermatid development progressed, the signals increased substantially. These signals peaked and were concentrated at one end of the nucleus when the spermatids began to undergo dramatic changes. In the mature sperm, the signal for KIFC1 gradually became weak and was mainly localized in the tail. In summary, evaluation of the expression pattern for lc-KIFC1 at specific stages of spermiogenesis has shed light on the potential functions of this motor protein in major cytological transformations. In addition, this study may provide a model for researching the molecular mechanisms involved in spermatogenesis in other teleost species, which will lead to a better understanding of the teleost fertilization process.

[Low-dose Tertiary Prophylactic Therapy for Severe Haemophilia A Adults in A Single Medical Center in Beijing Area].

OBJECTIVE: To investigate the benefit of low-dose tertiary prophylaxis in adults with severe haemophilia A(SHA). METHODS: Twenty-two SHA patients aged 18 to 60 years from the Haemophilia Centre of Peking Union Medical College Hospital, Beijing, China, were retrospectively observed from their one year on-demand treatment to one year tertiary prophylaxis using plasma derived factor 8 concentrates at 5-10 IU/kg 2-3x per week. All the patients had already developed arthropathy. Gilbert and the functional independence scores in hemophilia were used to assess the joint status and the ability in the activities of daily living of the patients. RESULTS: Comparing with on-demand therapy,the annual bleeding frequency during low-dose tertiary prophylaxis decreased significantly by 72.7%,from 39.9 ± 21.5 to 11.1 ± 7.2 (P<0.0001),the total Gilbert score decreased from 50.5±32.1 to 45.2±29.6(P<0.05),and the total functional independence score in hemophilia score increased from 18.6 ± 5.2 to 21.7 ± 4.1 (P<0.05). CONCLUSION Low-dose tertiary prophylaxis in adults with SHA is beneficial by reducing bleeding frequency, improving the health status of joints, and improving the activities of daily living, thus raising the quality of life.

Glutathione peroxidase-1 is required for self-renewal of murine embryonic stem cells.

Embryonic stem (ES) cells are pluripotent cells that are capable of giving rise to any type of cells in the body and possess unlimited self-renewal potential. However, the exact regulatory mechanisms that govern the self-renewal ability of ES cells remain elusive. To understand the immediate early events during ES cell differentiation, we performed a proteomics study and analyzed the proteomic difference in murine ES cells before and after a 6-h spontaneous differentiation. We found that the expression level of glutathione peroxidase-1 (GPx-1), an antioxidant enzyme, is dramatically decreased upon the differentiation. Both knockdown of GPx-1 expression with shRNA and inhibiting GPx-1 activity by inhibitor led to the differentiation of ES cells. Furthermore, we showed that during early differentiation, the quick degradation of GPx-1 was mediated by proteasome. Thus, our data indicated that GPx-1 is a key regulator of self-renewal of murine embryonic stem cells.

[Prevalence and features of pathogenic bacteria in the department of hematology without bone marrow transplantation in Peking Union Medical College Hospital from 2010 to 2012].

OBJECTIVE: To investigate the incidence, pathogens, and clinical features of infection in consecutive cases from 2010 to 2012 in Peking Union Medical College Hospital. METHOD: The incidence, pathogen, treatment, and outcomes of patients with hematological diseases who had positive findings of bacterium in their samples from 2010 to 2012 were retrospectively analyzed. RESULTS: There were 449 positive samples (5.8%) from 4 890 patients during this period, among which 388 were proved to be with pathogenic bacteria. Samples separated from patients with community-aquired infections accounted for 8.4% of all positive samples. Most community-aquired infections were caused by Gram-negative bacteria (75%), although no multidrug-resistant bacteria was observed. Samples separated from patients with nosocomial infections accounted for 91.6% of all positive samples. Respiratory tract (49.4%) and peripheral blood (32.6%) were the most common samples with positive results. Skin soft tissues (10.4%), and urine (3.7%) were less common samples. Most of the pathogenic bacteria of the nosocomial infections were Gram-negative (66.9%). The most common Gram-negative bacteria included Escherichia coli (13.8%), Pseudomonas aeruginosa (12.1%), and Klebsiella pneumonia (12.1%), while Staphylococcus aureus (10.4%), Enterococcus faecium (7.0%), and Staphylococcus epidermidis (5.1%) were the most common Gram-positive bacteria. Gram-negative bacteria consisted of most of sputum samples and peripheral blood samples. Samples from the surface of skin wound and anal swab were composed largely by Gram-positive bacteria (63.8%). The detection rates of extended-spectrum beta-lactamase-producing Klebsiella pneumonia/Klebsiella oxytoca, Escherichia coli, and Proteus mirabilis were 24.0%, 87.9% and 38.4%, respectively. The resistance to Acinetobacter baumannii was serious. Multidrug-resistant, extensive drug resistant and pan drug resistant A. baumannii acountted for 74% of all A. Baumannii infections. Stenotrophomonas maltophilia showed low resistance to sulfamethoxazole/trimethoprim, levofloxacin and minocycline. Also, 22 methicillin-resistant Staphylococcus aureus and 9 methicillin-resistant Staphylococcus Epidermidis were detected, which were only sensitive to vancomycin, teicoplanin, and linezolid. All patients were treated in the haematology wards and most of them were under agranulocytosis or immunosuppression. Finally, 22 patients reached clinical recovery through anti-infective therapy, whereas 49 patients died. Among those deaths, 42 patients attributed to severe infections and infection-associated complications. Fourteen of all the deaths might be infected with drug-resistance bacteria. There were 61 samples proved to be bacteria colonization. Nonfermenters such as Acinetobacter baumannii and Stenotrophomonas maltophilia made up for a large amount of bacteria colonization. CONCLUSIONS: The pathogens of nosocomial infections in the hematology ward are mainly Gram-negative bacteria. The incidences and pathogens vary from different infection sites. Nosocomial infection still has a higher mortality rate. Once nonfermenters are detected positive, the pathogenic or colonial bacteria should be distinguished.

Application of temporarily functional antibiotic-containing bone cement prosthesis in revision hip arthroplasty.

PURPOSE: To investigate the clinical outcome of two-stage revision total hip arthroplasty for infected hip arthroplasty using antibiotic-impregnated cement prosthesis. MATERIALS AND METHODS: Forty-one patients, who suffered from an infection after hip replacement or internal fixation of femoral neck and trochanteric fractures, were treated with a two-stage revision hip arthroplasty and followed up for an average of 37 months. All the patients were implanted with antibiotic-impregnated cement prosthesis as one-stage treatment and were then managed with two-stage revision hip arthroplasty after 12-24 weeks. During the follow-up, Merle d'Aubigné hip score and Harris score were employed for assessment of hip function, and infection recurrence was observed. RESULTS: According to Merle d'Aubigné hip score, 16 patients (39.2 %) were excellent, 19 (46.3 %) were good, 6 (14.6 %) were moderate, and no bad result and the average score was 15.42. Mean Harris score of preoperation, interval period, and postoperation was 46.7, 66.5, and 92.3, respectively. There was no infection recurrence. CONCLUSION: Two-stage revision total hip arthroplasty for infected hip arthroplasty using antibiotic-impregnated cement prosthesis has a satisfying clinical outcome.

Integrated 4D label-free proteomics and data mining to elucidate the effects of thermal processing on crisp grass carp protein profiles.

The crisp grass carp (CGC; Ctenopharyngodon idellus C. et V.), known for its unique texture and flavour, is a culinary delicacy whose quality is significantly influenced by thermal processing. This study employed 4D label-free proteomics and data mining techniques to investigate the proteomic changes in CGC muscle tissue induced by various heating temperatures. CGC samples were subjected to a series of heat treatments at increasing temperatures from 20 °C to 90 °C. Proteins were extracted, digested, and analysed using high-resolution mass spectrometry. The proteomic data were then subjected to extensive bioinformatics analysis, including GO and KEGG pathway enrichment. We identified a total of 1085 proteins, 516 of which were shared across all the temperature treatments, indicating a core proteome responsible for CGC textural properties. Differential expression analysis revealed temperature-dependent changes, with significant alterations observed at 90 °C, suggesting denaturation or aggregation of proteins at higher temperatures. Functional enrichment analysis indicated that proteins involved in amino acid metabolism, glutathione metabolism, and nucleotide metabolism were particularly affected by heat. Textural analysis correlated these proteomic changes with alterations in CGC quality attributes, pinpointing 70 °C as the optimum temperature for maintaining the desired texture. A strong positive correlation between specific upregulated proteins was identified, such as the tubulin alpha chain and collagen alpha-1(IV) chain, and the improved textural properties of CGC during thermal processing, suggesting their potential as the potential biomarkers. This study offers a comprehensive proteomic view of the thermal stability and functionality of CGC proteins, delivering invaluable insights for both the culinary processing and scientific management of CGC. Our findings not only deepen the understanding of the molecular mechanisms underpinning the textural alterations in CGC during thermal processing but also furnish practical insights for the aquaculture industry. These insights could be leveraged to optimize cooking techniques, thereby enhancing the quality and consumer appeal of CGC products.

Transcriptome sequencing of garlic reveals key genes related to the heat stress response.

With global warming, heat stress has become an important factor that seriously affects crop yield and quality. Therefore, understanding plant responses to heat stress is important for agricultural practice, but the molecular mechanism of high-temperature tolerance in garlic remains unclear. In this study, 'Xusuan No. 6' was used as the experimental material. After heat stress for 0 (CK), 2 and 24 h, transcriptome sequencing was used to screen metabolic pathways and differentially expressed genes (DEGs) closely related to heat stress and was further verified by quantitative real-time polymerase chain reaction (qRT-PCR). A total of 86,110 unigenes obtained from the raw transcriptome sequencing data were spliced. After 2 h of heat treatment, the expression levels of 8898 genes increased, and 3829 genes were decreased in leaves. After 24 h, the expression levels of 7167 genes were upregulated, and 3176 genes were downregulated. Gene Ontology enrichment analysis showed that DEGs were mainly enriched in seven categories: cellular processes, metabolic processes, binging, catalytic activity, cellular anatomical entity and protein-containing complex response to stimulus. Kyoto Encyclopedia of Genes and Genomes pathway enrichment showed that DEGs are involved in protein processing in the endoplasmic reticulum, plant hormone signal transduction, phenylpropanoid biosynthesis, and photosynthetic antenna proteins. Six genes were selected and further verified by qRT-PCR. In this study, the full-length transcriptome of garlic was constructed, and the regulatory genes related to the heat resistance of garlic were studied. Taken together, these findings can provide a theoretical basis for the cloning of heat resistance genes in garlic and for the analysis of heat resistance mechanisms.

[The incidence of heparin-induced thrombocytopenia and positivity of antibody in patients treated with heparin preparations].

OBJECTIVES: To investigate the incidence of the heparin-induced thrombocytopenia (HIT) and positivity of anti-heparin/platelet factor 4 complex antibody (HIT-antibody) in inpatients treated with heparin preparations. METHODS: A total of 197 consecutive patients, including 120 men and 77 women, who were treated with unfractioned heparin (UFH) or low molecular weight heparin (LMWH), were enrolled in this study. HIT-antibody was detected by enzyme-linked immunosorbent assay (ELISA). All patients were classified into subgroups based on "4Ts" (Pretest Clinical Scoring System) and types of underlying disorders. The incidence of HIT and positivity of HIT-antibody among different groups were analyzed. RESULTS: The overall incidence of HIT was 3.0% (6/197), and the positivity of HIT antibody was 12.2% (24/197). The positivity of HIT-antibody was 10.1% (18/178), 7.7% (1/13) and 83.3% (5/6) in low, moderate and high HIT probability group respectively. There were significant differences of HIT positivity and mean level of HIT-antibody between the high HIT probability group and the low or moderate HIT probability group (P = 0.000). Both the incidence of HIT and positivity of HIT-antibody were higher in surgical patients than in medical patients (5.8% vs 0.9%, P = 0.047 and 19.8% vs 6.3%, P = 0.004). CONCLUSIONS: The incidence of HIT and positivity of HIT-antibody were 3.0% and 12.2% respectively, both of which were related with the types of disorders. Detection of HIT-antibody has a better applicable value in patients with high HIT probability.

[The clinical analysis of 14 cases of systemic lupus erythematosus complicated by thrombotic thrombocytopenic purpura].

OBJECTIVE: To analyze the clinical features, diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE). METHODS: Clinical manifestations, laboratory findings, diagnosis,treatment and prognosis of 14 SLE patients with TTP were retrospectively analyzed. RESULT: Of the 14 patients diagnosed with SLE and TTP, 4 were men and 10 were women. The median age at diagnosis was 23 (17-69) years old. In five patients, the onset of SLE preceded TTP, and in nine patients SLE and TTP occurred simultaneously. All the 14 patients had thrombocytopenia and hemolytic anemia, 12 had fever, 11 had neurologic abnormalities, and 11 had renal dysfunction. Eight patients presented with the classic pentad of symptoms. Six patients were given steroids (alone or in combination with intravenous immunoglobulin and cyclophosphamide), and eight patients were treated with steroids in combination with plasmapheresis, with response rates of 2/6 and 6/8, respectively. Six patients died, with overall mortality rate of 6/14. No patients relapsed during the follow-up period. CONCLUSIONS: SLE and TTP share some similar clinical symptoms. As a result, repeated examinations of peripheral blood smears are very important for early diagnosis. The renal damage in patients of co-existing diseases is more serious than those with TTP alone or SLE alone. Early diagnosis and prompt treatment with plasma exchange and steroids may improve the prognosis in SLE patients with TTP.

[Hennekam syndrome: a case report and review of literature].

OBJECTIVE: To study the clinical characteristics of Hennekam syndrome. METHODS: We described a case of long-term iron deficiency anemia, characteristic facial anomalies, growth retardation, and intestinal lymphangiectasia. To our knowledge, this is the first case of Hennekam syndrome reported in China. Meanwhile, relevant literature was also reviewed. RESULTS: A total of 35 cases of Hennekam syndrome were identified, consisting of 18 males and 17 females (age ranging 0 - 40 years old). Lymphangiectasia, lymphedema, facial anomalies (hypertelorism, flat nasal bridge and flat face) and developmental retardation were the major clinical manifestations of the syndrome. CCBE1 mutation may have played an important role in the pathogenesis of the syndrome. Long-term moderate-to-severe iron deficiency anemia was a distinctive feature of our case. Lymphangiography revealed lymphangiectasia of small intestine and lower limb, as well as thoracic outlet obstruction. Complete elimination of anemia and significant increase of serum albumin level were observed several months after the adhesiolysis procedure of the distal end of thoracic duct. However, anemia and severe hypoalbuminemia relapsed after taking greasy food. CONCLUSIONS: Hennekam syndrome is a rare autosomal recessive syndrome characterized by defective lymphatic development. Congenital lymphangiectasia should be considered in the patients with unexplained developmental retardation and hypoalbuminemia. Moreover, intestinal lymphangiectasia can be a rare cause of gastrointestinal bleeding.

[Significance of magnetic resonance imaging in the detection of iron overload].

OBJECTIVE: To evaluate the significance of magnetic resonance imaging (MRI) T2(*) value analysis in patients with iron overload and compare it with other clinical parameters. METHODS: A total of 53 patients with suspected iron overload were recruited from four Beijing hospitals from December 2010 to December 2012. Their liver and heart T2(*) values were calculated and their serum ferritin (SF), transferrin saturation, blood transfusion volume and other clinical parameters were recorded and analyzed. RESULTS: There were 37 males and 16 females with a medium age of 50 years(15-72 years). Their etiologies included myelodysplastic syndromes (MDS, n = 25), aplastic anemia (AA, n = 16), myelofibrosis (n = 5), hemochromatosis (n = 2) and ß thalassaemia (n = 2), and 3 patients with high SF values were found on regular health examinations. Among them, there were transfusion history (n = 45), SF>1000 µg/L (n = 49), sign of iron overload (n = 10), abnormal liver function (n = 38) and hyperglycemia (n = 32). T2(*) value analysis showed that 10 patients had no evidence of iron overload, 43 patients had liver iron overload (14 mild, 22 moderate and 7 severe) and 2 patients had heart iron overload (1 MDS with heavy transfusion history and 1 AA with heart failure). No relations existed between T2(*) value and SF (P = 0.050) , T2(*) value and transfusion volume (P = 0.820) , and liver T2(*) value and heart T2(*) value (P = 0.129) . CONCLUSIONS: MRI T2(*) value is an accurate way of quantitative detection of iron overload. It provides a comprehensive understanding of patients with iron overload in conjunctions with MRI T2(*) value and other clinical parameters.

[Efficacy and safety of rituximab therapy for refractory/relapsing thrombotic thrombocytopenic purpura].

OBJECTIVE: To evaluate the efficacy and safety of rituximab in treating patients with refractory and/or relapsing thrombotic thrombocytopenic purpura (TTP). METHODS: Totally three patients received rituximab as salvage therapy in our hospital. Rituximab was administered at a weekly dose of 375 mg/m(2) for 2 or 4 consecutive weeks. After clinical remission, patients were followed up every 3 months. RESULTS: All three patients achieved complete remission. The median time to platelet count recovery was 7 days (4-12 days) after the first rituximab infusion. During the follow-up (median: 12 months; range: 9-18 months), no patients experienced relapse. No side effect was noted during treatment and follow-up period. CONCLUSION: Therapy with rituximab is effective and well tolerated for patients with refractory or relapsing TTP.

Unsupervised Deep Tensor Network for Hyperspectral-Multispectral Image Fusion.

Fusing low-resolution (LR) hyperspectral images (HSIs) with high-resolution (HR) multispectral images (MSIs) is a significant technology to enhance the resolution of HSIs. Despite the encouraging results from deep learning (DL) in HSI-MSI fusion, there are still some issues. First, the HSI is a multidimensional signal, and the representability of current DL networks for multidimensional features has not been thoroughly investigated. Second, most DL HSI-MSI fusion networks need HR HSI ground truth for training, but it is often unavailable in reality. In this study, we integrate tensor theory with DL and propose an unsupervised deep tensor network (UDTN) for HSI-MSI fusion. We first propose a tensor filtering layer prototype and further build a coupled tensor filtering module. It jointly represents the LR HSI and HR MSI as several features revealing the principal components of spectral and spatial modes and a sharing code tensor describing the interaction among different modes. Specifically, the features on different modes are represented by the learnable filters of tensor filtering layers, the sharing code tensor is learned by a projection module, in which a co-attention is proposed to encode the LR HSI and HR MSI and then project them onto the sharing code tensor. The coupled tensor filtering module and projection module are jointly trained from the LR HSI and HR MSI in an unsupervised and end-to-end way. The latent HR HSI is inferred with the sharing code tensor, the features on spatial modes of HR MSIs, and the spectral mode of LR HSIs. Experiments on simulated and real remote-sensing datasets demonstrate the effectiveness of the proposed method.