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1.
Clin Cosmet Investig Dermatol ; 17: 457-461, 2024.
Article in English | MEDLINE | ID: mdl-38404476

ABSTRACT

Background: Multiple eruptive dermatofibroma (MEDF) is a rare presentation of dermatofibroma which is frequently associated with underlying diseases such as human immunodeficiency virus infection or systemic lupus erythematosus. It generally presents a characteristic histology with hyperplasia of the epidermis, prominent bundles of collagen and a diffuse proliferation of fibrocytes. Case Summary: We report a case of MEDF in a 30-year-old man who presented with a large number of dark brownish red maculopapules distributed over the trunk and extremities for more than 10 years. According to the pathology, the patient was diagnosed with MEDF. Infections and autoimmune diseases were ruled out. As he had no clinical symptoms, and presented with lesions widely distributed over the body, we gave no special treatment, but suggested a regular examination. Conclusion: Patients with MEDF usually have no pain and pruritus. If human immunodeficiency virus infection and systemic lupus erythematosus and other causes are ruled out, and lesions are widely distributed over the body, regular check-up is recommended without specific treatment.

2.
Toxicology ; 489: 153497, 2023 05 01.
Article in English | MEDLINE | ID: mdl-37011868

ABSTRACT

Beta-cypermethrin (ß-CYP) is a universally used pyrethroid pesticide with adverse effects on human health. ß-CYP may impair endometrial remodeling in mice; however, the mechanism remains largely unknown. Endometrial remodeling plays a vital role in embryonic development and the maintenance of pregnancy. Therefore, we explored the mechanism by which peri-implantation ß-CYP administration reduces uterine remodeling in pregnant mice. The C57BL/6 J pregnant mice were administered a dose of 20 mg/kg.bw. d ß-CYP via oral gavage once daily from day 1 of gestation (GD1) to GD7. Molecular markers of endometrial remodeling, stromal cell proliferation, cell cycle regulation, and the PI3K/Akt/mTOR signaling pathway were evaluated in the decidual tissue of the uterus on GD7. An in vivo pseudopregnancy mouse model, a pregnant mouse model treated with an mTOR activator and an mTOR inhibitor and an in vitro decidualization model of mouse endometrial stromal cells were used to confirm ß-CYP-induced defective endometrial remodeling and the key molecules expression of PI3K/Akt/mTOR signaling pathway. The results showed that ß-CYP decreased the expression of the endometrial remodeling markers MMP9 and LIF in the uterine decidua. Peri-implantation ß-CYP treatment markedly downregulated the expression of endometrial proliferation markers PCNA and Ki67 and decreased decidua thickness. Correspondingly, peri-implantation ß-CYP exposure upregulated the expression of FOXO1, P57 and p-4E-BP1 in the decidua. Further experiments showed ß-CYP significantly inhibited key molecules in the PI3K/Akt/mTOR pathway: PI3K, p-Akt/Akt, p-mTOR, and p-P70S6K in the uterine decidua. Additional experiments showed that aberrant endometrial remodeling induced by ß-CYP was aggravated by rapamycin (an mTOR inhibitor) and partially reversed by MHY1485 (an mTOR agonist). In summary, our results indicated that a reduction in the PI3K/Akt/mTOR pathway may enhance defective endometrial remodeling by downregulating the proliferation and differentiation of endometrial stromal cells in early pregnant mice exposed to ß-CYP. Our study elucidates the mechanism of defective endometrial remodeling induced by peri-implantation ß-CYP exposure.


Subject(s)
Pesticides , Pyrethrins , Pregnancy , Female , Mice , Humans , Animals , Decidua/metabolism , Proto-Oncogene Proteins c-akt/metabolism , Pesticides/metabolism , Phosphatidylinositol 3-Kinases/metabolism , Mice, Inbred C57BL , Endometrium , Embryo Implantation , TOR Serine-Threonine Kinases/metabolism , Pyrethrins/toxicity , Stromal Cells
3.
Headache ; 63(1): 104-113, 2023 01.
Article in English | MEDLINE | ID: mdl-36651572

ABSTRACT

OBJECTIVE: To explore gamma-aminobutyric acid (GABA) and glutamate/glutamine (Glx) levels in the right thalamus of patients with episodic migraine (EM) and chronic migraine (CM) and their effects on the chronification of migraine. BACKGROUND: Migraine affects approximately 1 billion people worldwide, with 2.5%-3% of people with EM progressing to CM each year. Magnetic resonance spectroscopy studies have revealed altered GABA and Glx levels in the thalamus of patients with migraine without aura, but these neurometabolic concentrations are underexplored in the thalamus of patients with CM. METHODS: In this cross-sectional study, patients with EM and CM were recruited. Mescher-Garwood point resolved spectroscopy sequence was used to acquire neurotransmitter concentrations in the right thalamus of patients with EM and CM and matched healthy controls (HCs). RESULTS: A total of 26 patients (EM, n = 11; CM, n = 15) and 16 age- and sex-matched HCs were included in the analysis. There were significantly lower GABA+/Water levels in the right thalamus of the CM group (mean ± standard deviation: 2.27 ± 0.4 [institutional units]) than that of the HC group (2.74 ± 0.4) (p = 0.026; mean difference [MD] = -0.5 [i.u.]), and lower Glx/Cr levels in the EM group (mean ± SD: 0.11 ± < 0.1) than in the HCs (0.13 ± < 0.1) and CM group (0.13 ± < 0.1) (p = 0.023, MD < -0.1, and p = 0.034, MD < -0.1, respectively). The GABA+/Glx ratio was lower in the CM group (mean ± SD: 0.38 ± 0.1) compared to the EM group (0.47 ± 0.1) (p = 0.024; MD = -0.1). The area under the curve for GABA+/Water levels in differentiating patients with CM from HCs was 0.83 (95% confidence interval 0.68, 0.98; p = 0.004). Correlation analyses within the migraine group revealed no significant correlation between metabolite concentration levels and headache characteristics after Bonferroni correction. CONCLUSION: Reduced GABA+/Water levels and imbalance of excitation/inhibition in the right thalamus may contribute to migraine chronification.


Subject(s)
Glutamine , Migraine Disorders , Humans , Glutamine/analysis , Glutamine/metabolism , Proton Magnetic Resonance Spectroscopy/methods , Glutamic Acid , Cross-Sectional Studies , Migraine Disorders/diagnostic imaging , Migraine Disorders/metabolism , gamma-Aminobutyric Acid/analysis , gamma-Aminobutyric Acid/metabolism , Thalamus/diagnostic imaging , Thalamus/metabolism
4.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-906145

ABSTRACT

Objective:To investigate the protective effect and molecular mechanism of Angelicae Sinensis Radix-Chuanxiong Rhizoma medicated serum (ASRCRS) against oxidative damage of PC12 cells induced by H<sub>2</sub>O<sub>2</sub>. Method:Oxidative damage of PC12 cells was induced by H<sub>2</sub>O<sub>2</sub><italic> in vitro</italic>, and intervention was performed in the low-, medium-, and high-dose ASRCRS groups with a final volume fraction of 15%. The cell viability was determined by methyl thiazolyl tetrazolium (MTT) assay. Cell morphology was observed by an inverted fluorescence microscope. The content of lactate dehydrogenase (LDH) and malondialdehyde (MDA), the activity of superoxide dismutase (SOD), and the distribution of reactive oxygen species (ROS) in the cell supernatant were detected by the kits. Cell apoptosis was detected by Annexin V-FITC/PI double staining. The protein expression levels of nuclear factor E<sub>2</sub>-related factor 2 (Nrf2), Kelch-like epichlorohydrin associated protein-1 (Keap1), heme oxygenase-1 (HO-1), and SOD1 were detected by Western blot. Result:Oxidative damage was induced by 300 μmol·L<sup>-1</sup> H<sub>2</sub>O<sub>2</sub> for 24 hours. Compared with the normal group, the model group showed abnormal cell morphology, reduced cell viability (<italic>P</italic><0.01), increased LDH and MDA (<italic>P</italic><0.01), blunted SOD activity, elevated intracellular distribution of ROS, down-regulated protein expression of Nrf2, HO-1, and SOD1 (<italic>P</italic><0.05, <italic>P</italic><0.05), and up-regulated protein expression of Keap1 (<italic>P</italic><0.01). Compared with the model group, ASRCRS groups displayed improved cell morphology, increased cell viability, inhibited cell apoptosis, potentiated SOD activity (<italic>P</italic><0.01), suppressed release of LDH (<italic>P</italic><0.01) and generation of ROS, decreased content of MDA (<italic>P</italic><0.01), up-regulated protein expression of Nrf2, HO-1 and SOD1 (<italic>P</italic><0.05, <italic>P</italic><0.01), and down-regulated protein expression of Keap1 (<italic>P</italic><0.01). Conclusion:ASRCRS could protect PC12 cells from oxidative damage induced by H<sub>2</sub>O<sub>2</sub> by up-regulating the expression of Nrf2 to activate the Nrf2/antioxidant response element (ARE) signaling pathway, enhancing the ability to resist oxidative damage, and inhibiting cell apoptosis.

5.
Journal of Experimental Hematology ; (6): 1251-1256, 2021.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-888547

ABSTRACT

OBJECTIVE@#To study the expression of B lymphocyte-induced mature protein-1 (BLIMP-1) in regulatory T cells (Tregs) of children with aplastic anemia (AA), and analyze its correlation with the number of Tregs and the levels of inhibitory cytokines interleukin (IL)-10 and transforming growth factor (TGF)-β in plasma.@*METHODS@#The peripheral blood samples of 10 newly diagnosed AA children and 10 healthy children were collected for experiment. qPCR was used to detect FOXP3 and PRDM1 mRNA expression levels. Flow cytometry was used to detect the proportion of Tregs, the expression of BLIMP-1 in Tregs, and the levels of cytokines such as IL-2, IL-17A, IL-6, interferon (IFN)-γ, IL-10 and TGF-β in plasma. Pearson correlation model was used to evaluate the relationship between the expression of BLIMP-1 in Treg and the number of Tregs, as well as the levels of IL-10 and TGF-β in plasma.@*RESULTS@#Compared with control group, the proportion of Tregs in peripheral blood of AA children was decreased significantly (P<0.001); The plasma levels of proinflammatory cytokines IL-2, IL-6 and IFN-γ in AA children were increased significantly (P=0.033, P=0.031, P=0.006), and IL-17A also was increased but the difference was not statistically significant (P=0.052), while anti-inflammatory cytokines IL-10 and TGF-β were significantly reduced (P=0.048, P=0.002). The relative expressions level of FOXP3 and PRDM1 mRNA in AA children were significantly lower than those in control group (P=0.037, P=0.016). The expression of BLIMP-1 protein in Tregs of AA children was significantly lower than that in control group (P<0.001). The expression level of BLIMP-1 protein in Tregs was positively correlated with the percentage of Tregs in lymphocytes (r=0.671, P=0.001), and was also positively correlated with the levels of IL-10 and TGF-β in plasma (r=0.500, P=0.029; r=0.486, P=0.030).@*CONCLUSION@#The expression of BLIMP-1 in Tregs of AA children is impaired, and the low expression of BLIMP-1 is related to the decrease of the number in Tregs and IL-10 and TGF-β expressions.


Subject(s)
Child , Humans , Anemia, Aplastic , Cytokines , Flow Cytometry , Forkhead Transcription Factors , Positive Regulatory Domain I-Binding Factor 1 , T-Lymphocytes, Regulatory , Transforming Growth Factor beta
6.
Langmuir ; 36(49): 14924-14932, 2020 Dec 15.
Article in English | MEDLINE | ID: mdl-33271018

ABSTRACT

Good control of the morphology, particle size, and wettability of silica nanoparticles is of increasing importance to their use in a variety of fields. Here, we propose a strategy to tune the surface wettability of nanosilica by changing the dosage of a chemical modifier. A series of measurements, including scanning electron microscopy (SEM), laser scatting technique, Fourier transform infrared (FTIR) spectroscopy, thermogravimetry, and surface hydroxyl number and water contact angle measurement, were conducted to verify the surface chemistry and wettability of these nanoparticles. Through controlled chemical modification, the contact angle of the treated nanoparticles increases from 34.7 to 155° with increasing amount of dichlorodimethylsilane (DCDMS) within a molar ratio (MR) between DCDMS and nanoparticles of 5.17. The number of hydroxyl groups covered on the particle surface decreases gradually from 1.79 to 0.47, and the surface grafting rate could reach 73.7%. As the addition of dichlorodimethylsilane equals MR 5.17, the contact angle reaches the maximum value of 155°, which displays excellent superhydrophobicity. After surpassing the point of MR 5.17, the contact angle does not increase but starts to decrease, ultimately remaining stable at 135°. It can be concluded that the surface wettability of nano-SiO2 particles can be precisely modulated by varying the amounts of the modifier. Furthermore, the modulating mechanism of the process occurring on the surface of SiO2 particles has been investigated at the molecular level.

7.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-802270

ABSTRACT

Objective:To investigate the protective effect of ferulic acid on PC12 cells injured by H2O2 and the molecular mechanisms. Method:The oxidative stress model was established by treating PC12 cells with H2O2, and then different dosages of ferulic acid (1, 10, 100 μmol·L-1) were used for intervention. Methyl thiazolyl tetrazolium (MTT) assay was used to evaluate the cell viability,lactate dehydrogenase (LDH) and malondialdelyde (MDA) in cell supernatant, and superoxidedismutase (SOD) in cells was tested by biochemical method respectively. Insulin-like growth factors-1 (IGF-1) mRNA and protein expressions were detected by Real-time fluorescence quantitative polymerase chain reaction(Real-time PCR) and Western blot. Result:The 24 h intervention with different dosages of ferulic acid (1, 10, 100 μmol·L-1) could significantly improve the oxidative damage of PC12 cells induced by H2O2, compared with the model group, ferulic acid at 1,10,100 μmol·L-1 significantly increased PC12 cells viability,significantly decreased LDH and MDA content in cell supernatant (PPPPPConclusion:Ferulic acid exerts a protective effect on H2O2-inducing PC12 cells injury,which might be related to insulin signaling pathways.

8.
Sichuan Da Xue Xue Bao Yi Xue Ban ; 49(4): 599-603, 2018 Jul.
Article in Chinese | MEDLINE | ID: mdl-30378317

ABSTRACT

OBJECTIVE: To determine the reliability and validity of the abridged Chinese version of the autism spectrum quotient (AQ) -child form. METHODS: A total of 86 children with autism spectrum disorder (ASD) were recruited from the West China Hospital from July 2014 to December 2016, along with 6 896 children recruited from three schools in Chengdu. The participants completed the AQ scale under instructions from a trained interviewer. Then 170 school children were selected and repeated the AQ scale within one month. RESULTS: All subscale scores were correlated with the scale score, but with weak inter-subscale correlations. The total AQ score of the control group was continuously distributed in the population, which was similar to the normal distribution. The skewness was -0.127 and the kurtosis was -0.124, indicating that the total AQ score was negatively skewed and slightly flat in the population.There were differences in AQ scores between different genders in community children (P<0.01), with male group (42.09±9.92) higher than female group (40.07±9.94).There was no gender difference in the ASD individuals. There was a correlation between age and AQ score (R=0.06).The autistic children had a higher AQ score (54.49±14.16) than the school children (41.12±9.98)(P<0.01). Similar results were found in the subscale scores, except for"attention to detail". The AQ scale had a Cronbach α coefficient of 0.71: ranging from 0.21 to 0.69 for the subscales. The test-retest reliability was good for the scale and the subscales (all P>0.05) . The sensitivity and specificity of AQ for screening ASD was both 0. 71. CONCLUSION: The abridged Chinese version of the AQ-child scale has good psychometrics properties and may be a valid and reliable instrument for ASD screening with a cut-off score of 48.


Subject(s)
Autism Spectrum Disorder/diagnosis , Autistic Disorder/diagnosis , Psychometrics/instrumentation , Child , China , Female , Humans , Male , Reproducibility of Results
9.
Journal of Experimental Hematology ; (6): 1447-1452, 2018.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-689915

ABSTRACT

<p><b>OBJECTIVE</b>To study the relationship of Blimp-1 hypoexpression with abnormality of Treg level and pathogenesis of aplastic anemia (AA).</p><p><b>METHODS</b>The mouse model with AA was established by adminis tration of IFN-γ combined with busulfan. The samples were collected at different day establishing AA model, and the spleen Treg number was detected, the Treg cells were sorted and expression level of prdm-1 was detected.</p><p><b>RESULTS</b>The number of Tregs in mice with AA was lower than that in control mice, moreover, the level of Treg decrease positively correlated with the AA severity (r=0.805), the higher the expression level of prdm-1, the higher the ratio of Treg/lymphocytes, showing positive correlation between them (r=0.548).</p><p><b>CONCLUSION</b>Blimp-1 expression may promote the proliferation and differentiation of Treg. The hypoexpression of Blimp-1 mediates the pathogenesis of AA and promotes progression of AA through reducing the proliferation of Treg, and decreacing the number of Treg.</p>

10.
Pancreas ; 46(10): 1327-1335, 2017.
Article in English | MEDLINE | ID: mdl-28930866

ABSTRACT

OBJECTIVES: The aim of this study was to investigate the role of P2X7R (purinergic 2X7 receptor) and NLRP3 (NACHT, LRR, and PYD domains-containing protein 3) inflammasome activation in the process of pancreatic fibrosis in a mouse model of chronic pancreatitis (CP). METHODS: Chronic pancreatitis was induced by repeated intraperitoneal injections of 50 µg/kg cerulein for 6 weeks in mice. P2X7R antagonist oxidized ATP (OxATP) or brilliant blue G (BBG) was administered after the last cerulein injection for 2 weeks. Pancreatic chronic inflammation and fibrosis were evaluated by histological score, Sirius red staining, and alpha-smooth muscle actin immunohistochemical staining. We further determined pancreatic P2X7R, NLRP3, and caspase-1 expressions in gene and protein levels and the pancreatic concentrations of caspase-1, interleukin 1ß (IL-1ß), and IL-18. RESULTS: The pancreatic P2X7R, NLRP3, and caspase-1 expressions in gene and protein levels and the pancreatic concentrations of caspase-1, IL-1ß, and IL-18 were all reduced significantly in both the OxATP and BBG groups (P < 0.05). The pancreatic chronic inflammation and the fibrosis indices were all remarkably attenuated (P < 0.05). CONCLUSIONS: P2X7R antagonist OxATP and BBG significantly decreased pancreatic chronic inflammation and fibrosis in a mouse CP model and suggested that blockade of P2X7R-NLRP3 inflammasome signaling pathway may represent a novel therapeutic strategy for CP and its fibrotic process.


Subject(s)
Disease Models, Animal , Inflammasomes/genetics , NLR Family, Pyrin Domain-Containing 3 Protein/genetics , Pancreas/metabolism , Pancreatitis, Chronic/genetics , Receptors, Purinergic P2/genetics , Animals , Caspase 1/genetics , Caspase 1/metabolism , Ceruletide , Cytokines/metabolism , Fibrosis , Gene Expression/drug effects , Humans , Inflammasomes/metabolism , Male , Mice, Inbred C57BL , NLR Family, Pyrin Domain-Containing 3 Protein/metabolism , Pancreas/drug effects , Pancreas/pathology , Pancreatitis, Chronic/chemically induced , Pancreatitis, Chronic/metabolism , Receptors, Purinergic P2/metabolism , Rosaniline Dyes/pharmacology
11.
Chinese Pharmacological Bulletin ; (12): 171-174, 2017.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-508272

ABSTRACT

Aim To investigate whether the pain modi-fication by group I metabotropic glutamate receptors (mGluRs)required the involvement of Src homology-2 domain-containing phosphatase-2 (SHP-2 ).Methods Co-immunoprecipitation was performed to examine the possible interaction between SHP-2 and group I mGluRs in spinal cord dorsal horn of mice.By measur-ing the paw withdrawal thresholds,the effects of SHP-2 inhibitor NSC-87877 or its catalytically inactive SHP-2 (C459S ) mutant on allodynia induced by group I mGluRs agonist DHPG (50 nmol)were observed.Re-sults Anti-mGluR5 antibody was able to co-immuno-precipitate SHP-2 from spinal dorsal horn of mice, while no SHP-2 was precipitated by anti-mGluR1 anti-body.Inactivation of SHP-2 by NSC-87877 (6 nmol) or SHP-2 (C459S ) effectively attenuated allodynia caused by DHPG.Conclusion SHP-2 can physically interact with mGluR5.The activation of SHP-2 may be necessary for group I mGluRs to process the nocicep-tive information.

12.
Journal of Experimental Hematology ; (6): 1605-1610, 2017.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-301680

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the clinical efficacy of Fu Fan Huang Dai Pian(RIF) and arsenic trioxide (ATO) regimens for treatment of children with acute promyelocytic leukemia (APL) and to explore the risk factors affecting the prognosis of patients.</p><p><b>METHODS</b>The clinical data of 45 newly diagnosed APL children admitted in our hospital from January 2004 to May 2017 were analyzed retrospectively. Among 45 APL children, 25 children were treated by chemotherapetic regimen including RIF (RIF group), another 20 children were treated by chemotherapeutic regimen including ATO (ATO group). The follow-up was performed in all APL children. The prognosis and incidence of side reactions from drugs in 2 groups were compared, and the high risk factors affecting the prognosis of patients were analyzed.</p><p><b>RESULTS</b>The median follow-up time was 49.8% months. In RIF group, no early death occured in 25 APL children; 5 cases did not achieve complete remission (CR) after induction therapy, CR rate was 88%. Out of 25 cases 2 caes relapsed, 3 cases died, 20 cases maintained contined CR (CCR), 2 cases failed to be followed-up. In ATO group, 2 cases suffered from early death, 5 cases did not achieve CR after induction therapy, CR rate was 90%, 2 caese relapsed and died, 15 cases maintained CCR, the follow-up failed in 1 caes. The 5 year- OS and EFS rate in all the patients were predicted as (82.2±6.2)% and (76.4±6.6)% respectively. The OS and EFS rate in RIF group were (86.1±7.4)% and (78.4±8.6)% respectively, which were significantly different from OS and EFS rate (76.4%±10.6%) and (74.0%±10.1%) respectively in ATO group (all P>0.05). As for the side reaction from drug, except for the cardiac damage (P<0.05), incidence of other side reactions was not significantly different between 2 groups (P>0.05). In addition, the 5 year-OS and EFS rates in APL children with CNSL were significantly lower than those in APL children without CNSL (all P<0.05), the 5 year OS and EFS rate in APL children did not reache M1 and with high risk were significantly lower than those in APL children reached M1 after induction therapy and with low and standerd risk (P<0.05 and P<0.05); the 5 year-OS and EFS rates did not correlate with age and sex.</p><p><b>CONCLUSION</b>The Fu Fang Huang Dai Pian shows the therapeutic efficacy on APL children same as ATO, moreover, no obvious enhancement in incidence of side reactions is observed, therefore, the Fu Fang Huang Dai Pian is effective and safe for treatment of APL children. The CNSL, poor respond to treatment, high risk in clinical stratification are high risk factors affecting prognosis of patients.</p>

13.
Journal of Experimental Hematology ; (6): 1825-1828, 2017.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-278735

ABSTRACT

<p><b>OBJECTIVE</b>To analyze the clinical features and pathogenetic gene mutation in a fetus at his third trimester with familial haemophagocytic lymphohistiocytosis (FHL).</p><p><b>METHODS</b>Target region sequencing and high-throughput sequencing were used to detect pathogenetic gene mutations for familial haemophagocytic lymphohistiocytosis in a late onset HLH fetus. Pathogenetic gene mutations of the patient and his parents were verified by Sanger dideoxy sequencing.</p><p><b>RESULTS</b>A male neonate, who had right pleural effusion, hepatomegaly and splenomegaly previously revealed by fetus ultrasound, was delivered at full-term by cesarean section. His clinical presentation showed recurrent fever, tachypenea, decreased breath sounds on right side, hepatosplenomegaly etc., which were gradually aggravating Lab.tests results were as follows: WBC 9.88×10/L, Hb 91 g/L, Plt 13×10/L, ALT 18 U/L,AST 69 U/L,TBIL 207.2 µmol/L, DBIL 183.5 µmol/L, TG 3.05 mmol/L, Fib 0.88 g/L, Serum ferritin 3 120 ng/ml and sIL-2R 57 420 U/ml. FCM showed that CD3CD16CD56cells reached to 3.60% in the pripheral blood. Haemophagocytes were occasionally found in the bone marrow. NK/NKT stimulation test showed a severe damage of degranulation of NK cells. Sequence analysis of genomic DNA from his peripheral blood demonstrated the compound heterozygous mutations of UNC13D gene: c.2448-13 G>A in exon26 and c.1055+1 G>A in exon12, both were pathogenetic mutations. In detailed family survey, it was confirmed that the mutation c.2448-13 G>A in exon26 was inherited from his mother and c.1055+1 G>A in exon12 from his father.</p><p><b>CONCLUSION</b>A rare case of familial haemophagocytic lymphohistiocytosis type 3 (FHL3) with late fetus onset who carried pathogenetic compound heterozygous mutations of UNC13D gene. Those neonates with recurrent fever, serous effusions and multiple organ failure should be screened for FHL. Identifying the pathogenic gene mutations laid the foundation of conceiving disease-free newborns.</p>

14.
Journal of Experimental Hematology ; (6): 1190-1195, 2016.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-246793

ABSTRACT

<p><b>OBJECTIVE</b>To analyze and compare the clinical features, treatment and prognosis of 31 children with Langerhans cell histiocytosis(LCH) treated with modified DAL-HX83/90 or JLSG-96 protocol.</p><p><b>METHODS</b>The clinical features, treatment and prognosis of 31 children with Langerhans cells admitted in our hospital from January 2005 to December 2014 were analyzed retrospectively. The outcome of patients treated with modified DAL-HX83/90 or JLSG-96 protocols were compared by using the Kaplan-Meier survival curve. Among 31 children with LCH, 19 males and 12 females, 12 younger than 2 years old, and 19 older than 2 years old. LCH usually affected skeleton system(77.4%), skin(42.0%), liver(29.0%), spleen(19.4%), hematopoietic system(12.9%). The most common misdiagnoses were upper respiratory tract infection, malignancies, focal infection, and eczema.</p><p><b>RESULTS</b>Response rate at week 6 was 76.9% in modified DAL-HX83/90 group and 94.1% in JLSG-96 group respectively, and no significant differences had been found between 2 groups. The 1-and 3-year overall survival rates of the patients treated with JLSG-96 protocol were 100% and 83.3%±15.2% respectively, while The 1-, 3-,5-year overall survival rates of those patients treated with the modified DAL-HX83/90 protocol were 70%±14.5%. The 1-and 3-year event-free rates of children treated with JLSG-96 protocol were 73.3%±11.4% and 66.7%±12.2%, respectively, while the 1-, 3-, 5-year event-free rates of those treated with modified DAL-HX83/90 protocol were 50%±15.8%, 40%±15.6% and 26.7%±15% respectively. No differences were found between the 2 groups for OS or EFS.</p><p><b>CONCLUSION</b>JLSG-96 protocol shows a better prognosis, and the risk of secondary malignancy caused by etoposide can be avoided. Early diagnosis of refractory LCH, specified grouping strategy and prolonged maintenance therapy may contribute to enhancing the EFS rates and reducing relapses.</p>


Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Histiocytosis, Langerhans-Cell , Kaplan-Meier Estimate , Neoplasm Recurrence, Local , Retrospective Studies , Survival Rate , Treatment Outcome
15.
Zhonghua Yi Shi Za Zhi ; 45(2): 104-9, 2015 Mar.
Article in Chinese | MEDLINE | ID: mdl-26420415

ABSTRACT

Yang's Haiyuan Private Library in Liaocheng City, Shandong Province, is the only extant one among the four famous private libraries of the Qing dynasty in Northern China. Its collection is ample and graceful and especially rich in rare and secret versions, amounting to over 2 000 kinds, 200- thousand volumes. By sorting out 5 kinds of such collections from the Yang's family catalogue of Liaocheng in Shandong, plus 3 kinds of supplemented catalogue by Wang Shaozeng, the Catalogue of Haiyuan Private Library Collected in Shandong Provincial Library, and catalogues of modern scholars, it can be basically identified that the number of medical collections of Yang's Haiyuan Private Library is 87 kinds. Supposing all kinds contain 112 works, then Haiyuan Private Library totally has 1296 volumes according to the catalogues.


Subject(s)
Books/history , Medicine, Chinese Traditional/history , History, Medieval
16.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-264947

ABSTRACT

This study was aimed to explore whether hyperglycemia during chemotherapy influences the prognosis of children with acute lymphocytic leukemia (ALL). The clinical medical records of all newly diagnosed patients with ALL at SUN Yat-Sen Memorial Hospital from June 2008 to May 2012 were analyzed retrospectively. The median time of follow-up for patients was 2.6 years (range 0.08 to 4.9 years). Patients were divided to hyperglycemia and euglycemia groups according to their blood glucose concentrations during chemotherapy which contains L-asp and dexamethasone. The variables between two groups were compared using χ(2) test, the RFS and OS among two groups were compared by use of Kaplan-Meier and Cox-proportional hazard analyses. The results showed that the hyperglycemia correlated with older age (43.33% vs 19.23%, P = 0.008) and high-risk disease at diagnosis (26.62% vs 4.76%, P = 0.017) , but did not associate with sex (P = 0.059). Patients with hyperglycemia had worse OS (94.2 ± 2.9% vs 83.1 ± 6.3%, P = 0.014) and more poor RFS (64.1 ± 8.9% vs 88.6 ± 3.8%, P < 0.001) at 5 years than their counterpart. It is concluded that the incidence rate of hyperglycemia during chemotherapy correlated with older age and high-risk disease in ALL children, and the patients with hyperglycemia during chemotherapy may have poorer prognosis.


Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Antineoplastic Combined Chemotherapy Protocols , Hyperglycemia , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Blood , Diagnosis , Prognosis , Retrospective Studies , Risk Factors
17.
Journal of Experimental Hematology ; (6): 1386-1390, 2014.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-340492

ABSTRACT

The purpose of this study was to investigate the clinical characteristics and the treatments of patients with vinblastine-related hyponatremia which was aggravated by azole antifungal agents in children with acute lymphoblastic leukemia(ALL). A total of 93 children treated with vinblastine in our department during April 2013 to March 2014 were enrolled in this study and were divided into 3 groups:VDLD, VDLD with azoles antifungal, VDLD with non azoles antifungal. The incidence and severity of hyponatremia were statistically analysed. The results showed that (1) the incidence of hyponatremia in VDLD group was 93.1%(67/72),100%(13/13) in VDLD with azoles antifungal group, and 75%(6/8) in VDLD with non-azoles antifungal, there was no statistically difference between these three groups. (2) Incidence of moderate to severe hyponatremia (Na<129 mmol/L) in VDLD with azoles antifungal group was(9/13,69.2%),which was significartly higher than those in VDLD group (22/72, 30.6%) and in VDLD with non azoles antifungal group (1/8, 12.5%). However, the difference between VDLD group and VDLD with non azoles antifungal group were not statistical significant. (3) the lowest serum sodium level in VDLD with azoles antifungal group (124.0 ± 8.6 mmol/L) was significantly lower than that in VDLD group (130.8 ± 3.8 mmol/L)and VDLD+non azoles antifungal group(132.9 ± 4.9 mmol/L). Otherwise, the difference was not statistically significant between VDLD group and VDLD with non azoles antifungal group. (4) four children with severe hyponatremia showed convulsions and coma which all belong to VDLD with azoles antifungal group. The children with hyponatremia were restricted intake of fluid, adjusted the liquid tension, supplied hypertonic sodium and given diuretic, the serum sodium value gradually picked up in these children. In 4-11 months' follow-up, no hyponatremia happened again in these children. It is concluded that the incident of hyponatremia in children treated with vinblastine is high, but most of them seldom showed clinical characteristics. The combination of antifungal azoles with vinblastine can increase the incidence and severity of hyponatremia. Therefore, combined administration of azole antifungals with vinblastine should be avoided.


Subject(s)
Child , Humans , Acute Disease , Antifungal Agents , Therapeutic Uses , Azoles , Therapeutic Uses , Hyponatremia , Incidence , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Vinblastine
18.
Journal of Experimental Hematology ; (6): 1661-1666, 2014.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-340440

ABSTRACT

This study was purposed to compare the efficacy and safety of two different doses of rabbit anti-thymocyte globulin (r-ATG) combined with cyclosporine (CsA) for treating children with severe aplastic anemia (SAA). From January 2005 to July 2010, a total of 95 children with SAA accepted intensive immunosuppressive therapy (IIST) in our department, out of them 55 cases were treated with r-ATG 2.5 mg/(kg·d) for 5 days in combination with CsA (group I) and other 40 cases were treated with r-ATG 3.5 mg/(kg·d) for 5 days in combination with CsA (group II). The responsive rate, adverse reactions, early mortality, relapse and clonal disease were analyzed retrospectively and results between the two groups were compared. Out of 95 patients 43 were boys and 52 were girls, their ages were from 1 to 16 years. The sex, age, severity and course of the disease were comparable between the two groups. The results showed that after treating for 3 and 6 months, the response of patients in group II was higher than that of patients in group I (50% vs 32.1%, P = 0.08 and 65% vs 45.3%, P = 0.059), at 9 and 12 months the response rate of patients in group II and group I did not show significant difference (70.0% vs 71.1%,P = 0.904 and 82.5% vs 80.8%,P = 0.832); at 12 months of treatment, the complete response rate of patients in group II was significantly higher than that of patients in group I (40.0% vs 23.1%,P = 0.08); at 3, 6, 9 months of treatment, the complete response rate of 2 groups showed no obvious difference. The incidence of serum disease, early infection and early mortality did not show statistical difference between two groups. There was no statistical difference in 2 year overall survival rate of two groups. In group I 39 patients were followed-up for more than 2 years, among them 3 patients relapsed, 1 patient died and 1 patient was diagnosed as acute monocytic leukemia (M5). In group II 15 patients were followed up for more than 2 years, there were no relapse, death and clonal disease. It is concluded that the r-ATG combined with CsA is an effective and safe therapeutic regimen for the SAA children. The effect of r-ATG 3.5 mg/(kg·d) is better than the 2.5 mg/(kg·d). The early safety is comparable between the two groups. However, the long-term effect, complications and survival rate need longer follow-up study to evaluate.


Subject(s)
Animals , Child , Female , Humans , Male , Rabbits , Anemia, Aplastic , Drug Therapy , Antilymphocyte Serum , Cyclosporine , Therapeutic Uses , Drug Combinations , Follow-Up Studies , Immunosuppressive Agents , Therapeutic Uses , Leukemia, Monocytic, Acute , Retrospective Studies , Survival Rate , Treatment Outcome
19.
Zhonghua Yu Fang Yi Xue Za Zhi ; 47(3): 238-44, 2013 Mar.
Article in Chinese | MEDLINE | ID: mdl-23866750

ABSTRACT

OBJECTIVE: To explore possible interrelationships among resistance to peer pressure, risky decision-making and health risk behaviors among young adolescents. METHODS: Based on the cluster sampling method, the participants who were recruited from 5 junior middle schools in Guangzhou and 3 junior middle schools in Shenyang city on October, 2010, were administered to complete the questionnaire concerned with their experiences with drinking and smoking during the past 30 days preceding the survey, and the hours using computer daily both in weekdays and in weekend. The level of resistance to peer influence and risky decision-making were assessed by Resistance to peer influence scale (RPIS) and Youth decision-making questionnaire (YDMQ). Logistic regression was used to explore possible interrelationships among resistance to peer influence, risky decision-making and health risk behaviors among young adolescents. RESULTS: A total of 1985 questionnaires were valid, including 1001(50.4%) boys and 984 (49.6%) girls. About 27.1% (537/1985) junior middle school students reported having health risk behaviors, boys' (30.7%, 307/1001) was higher than girls' (23.4%, 230/984) with significant gender difference (P < 0.05). The prevalence of smoking, drinking during the past 30 days before the survey and using computer over 3 hours daily in weekdays and in weekend were 5.1% (102/1985), 14.3% (284/1985), 3.5% (70/1985) and 13.7% (272/1985), respectively. The rate of drinking, using computer over 3 hours daily in weekdays and in weekend were higher in males (16.4% (164/1001), 4.5% (45/1001), 16.2% (162/1001)) than those in females (12.2% (120/984), 2.5% (25/984), 11.2% (110/984)) (P < 0.05). The scores of RPIS and YDMQ of the two cities adolescents were 2.82 ± 0.39 and 1.68 ± 0.62. The students reported smoking, drinking during the past 30 days before the survey and using computer over 3 hours daily in weekend gained lower RPIS scores (2.43 ± 0.40, 2.61 ± 0.41, 2.77 ± 0.40) than their counterparts who didn't report these kind of health risk behaviors (2.84 ± 0.38, 2.85 ± 0.38, 2.82 ± 0.39)(P < 0.05). And those reported smoking, drinking during the past 30 days before the survey and using computer over 3 hours daily in weekdays and in weekend gained higher YDMQ scores (2.38 ± 0.66, 2.06 ± 0.66, 1.97 ± 0.72, 1.84 ± 0.64, respectively) than their counterparts who didn't report these kind of health risk behaviors (1.64 ± 0.38, 1.61 ± 0.58, 1.67 ± 0.61, 1.65 ± 0.61, respectively) (P < 0.05). After adjusting gender, area, parental education degree, self-reported family economic condition, multi-variant logistic regression analysis indicated that the low and middle level of resistance to peer influence (low and middle level vs high level, had odds ratios of 2.97 (1.96 - 4.50) and 1.51 (1.05 - 2.16)), and also the middle and high level of risky decision-making (middle and high level vs low level, had odds ratios of 1.62 (1.19 - 2.22) and 3.43 (2.39 - 4.90)) were all the risk factors of adolescent health risk behaviors. CONCLUSION: Adolescents with poor ability of resistance to peer pressure and high risky decision-making were both the risk factors of adolescent health risk behaviors.


Subject(s)
Health Behavior , Peer Group , Risk-Taking , Adolescent , Child , Cross-Sectional Studies , Female , Humans , Logistic Models , Male , Risk Assessment , Surveys and Questionnaires
20.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-325196

ABSTRACT

This study was purposed to summarize the clinical characteristics and laboratorial data of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in pediatric patients in order to enhance understanding this disease in diagnosis and therapy. A rare case of BPDCN in children was enrolled in this study. The blood routine test, examination of bone marrow cell morphology, histopathology and immunophenotype of the skin lesions were performed and analysed, the single cell suspensions of the biopsied skin mass were detected by flow cytometry. The results showed that tumor cells expressed CD4, CD56, CD43 and CD123, while not expressed CD19, CD20, CD3, CD8, CD13, CD11b and myeloperoxidase (MPO). According to the clinical and laboratorial features and the results from histopathological and immunophenotype examinations, BPDCN was confirmed. It is concluded that BPDCN in children is an extremely rare hematopoietic malignancy with presenting a rapidly and fatally aggressive clinical course. The diagnosis of this disease is mainly based on the clinical presentations, pathologic and immunohistochemical features. BPDCN is a highly aggressive disease, its prognosis is very poor, its pathogenesis remans still unclear. A standard treatment protocol for BPDCN has not yet been established.


Subject(s)
Adolescent , Humans , Male , Dendritic Cells , Hematologic Neoplasms , Skin Neoplasms , Waldenstrom Macroglobulinemia
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