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1.
Lipids Health Dis ; 22(1): 136, 2023 Aug 25.
Artículo en Inglés | MEDLINE | ID: mdl-37626321

RESUMEN

OBJECTIVE: The purpose of this study was to comprehensively evaluate the lipid profiles in patients with juvenile idiopathic arthritis (JIA). METHODS: The literature and relevant reviews were searched for published clinical studies on the relationship between JIA and blood lipid levels. The Newcastle-Ottawa scale (NOS) was applied to evaluate the risk and methodological value of the included case‒control and cohort studies. Standardized mean differences (SMDs) and 95% confidence intervals were derived for all variables with adequate unprocessed data. This meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. RESULTS: In total, 16 studies were incorporated through screening. The analysis findings revealed that the levels of very low-density lipoprotein cholesterol [SMD=-0.411, 95% CI (-0.774~-0.048), P = 0.026], high-density lipoprotein cholesterol [SMD=-0.528, 95% CI (-0.976~-0.079), P = 0.021], and apolipoprotein A1 [SMD=-1.050, 95% CI (-1.452~-0.647), P = 0.000] in JIA patients were statistically lower than those observed in healthy controls. The level of low-density lipoprotein cholesterol [SMD = 0.202, 95% CI (0.003 ~ 0.400), P = 0.046] was significantly higher in JIA patients than in healthy controls. In JIA patients, body mass index [SMD=-0.189, 95% CI (-0.690 ~ 0.311), P = 0.459], high-density lipoprotein [SMD =-1.235, 95% CI (-2.845 ~ 0.374), P = 0.133), low-density lipoprotein [SMD = 0.616, 95% CI (-0.813 ~ 2.046), P = 0.398), triglycerides (SMD = 0.278, 95% CI (-0.182 ~ 0.738), P = 0.236], total cholesterol [SMD=-0.073, 95% CI (-0.438 ~ 0.293), P = 0.696] and apolipoprotein B levels [SMD = 0.226, 95% CI (-0.133 ~ 0.585), P = 0.217] were not significantly different from those in healthy controls. CONCLUSIONS: The outcomes of this meta-analysis suggest that dyslipidemia is common in JIA patients compared to healthy controls. Patients with JIA have a significantly increased risk of atherosclerosis and cardiovascular disease later in life.


Asunto(s)
Artritis Juvenil , Humanos , Apolipoproteínas B , HDL-Colesterol , LDL-Colesterol , Lipoproteínas HDL
2.
Zhongguo Zhong Yao Za Zhi ; 46(14): 3455-3464, 2021 Jul.
Artículo en Zh | MEDLINE | ID: mdl-34402267

RESUMEN

Chinese materia medica( CMM) serves as an important cornerstone for the development of traditional Chinese medicine( TCM) culture and industry due to its unique ecological,medical,economic,scientific and technological,and cultural values. The supply shortage and unstable quality of some CMM resources have hindered the development of TCM. Ensuring the sustainable use of CMM resources has become essential for the development of TCM in China. Enriching CMM resources is the key to ensuring the sustainable utilization of TCM resources in China,which can be achieved via expanding the medicinal parts,developing the substitutes,seeking for analogues,exploring the ethnic and folk medicines,or introducing foreign medicinal materials. CMM efficacy or function positioning plays a very important role in the transformation of new CMM resources. The strategies and methods for efficacy or function positioning of new CMM resources,including analogy,plant genetic relationship exploration,medicinal property deduction,ethnobotanical investigation,text mining,network pharmacology,and structure-activity relationship exploration,were systematically proposed in this study based on CMM theory,textual research,and modern methodologies. This paper is expected to provide a theoretical reference for the continuous enrichment and development of CMM resources and the high-quality development of TCM culture and industry.


Asunto(s)
Medicamentos Herbarios Chinos , Materia Medica , China , Minería de Datos , Medicamentos Herbarios Chinos/farmacología , Humanos , Medicina Tradicional China
3.
Zhongguo Zhong Yao Za Zhi ; 46(14): 3465-3477, 2021 Jul.
Artículo en Zh | MEDLINE | ID: mdl-34402268

RESUMEN

High fat diet induced hyperlipidemia hamster model was used to explore the anti-hyperlipidemia effect of water extract of Moringa oleifera leaves( WEMOL). On this basis,the possible action mechanism was predicted by network pharmacology. Golden hamsters were randomly divided into normal diet group( NFD),high-fat diet group( HFD),simvastatin group,high dose group of WEMOL( HIWEMOL) and low dose group of WEMOL( LOWEMOL). The model was administered simultaneously for 66 days,during which the body weight changes of hamsters were recorded. At the end of the experiment,serum lipid level and serum transaminase level of golden hamsters in each group were detected,and the pathological changes of liver were observed by hematoxylin-eosin( HE) staining. The results showed that WEMOL could significantly decrease the serum total cholesterol( TC),total triglyceride( TG),low density lipoprotein cholesterol( LDL-c) levels,and reduce the lipid deposition in liver tissue,thus improving the hyperlipidemia of golden hamsters. According to the prediction of network pharmacology,219 targets of potential active components of M.oleifera leaves and 185 targets of water-soluble potential active components of M. oleifera leaves for the treatment of hyperlipidemia were obtained separately. The MCODE analysis was performed on the PPI network of 219 targets and 185 targets obtained above and got five and four clusters respectively. The signaling pathway analysis of clusters showed that among the common pathways,nonalcoholic fatty liver,insulin resistance,MAPK signaling pathway,estrogen signaling pathway,cell apoptosis and HIF-1 signaling pathway were associated with hyperlipidemia. In addition,the potential active components of M. oleifera leaves could also inhibit the metabolic inflammation of hyperlipidemia by modulating complement and coagulation cascades signaling pathway,and GSK3 B,F2,AKT1,RELA,SERPINE1 might be the key targets. The water-soluble potential active components of M. oliefera leaves could modulate lipid metabolism by modulating AMPK signaling pathway and JAK-STAT signaling pathway,with PIK3 CB,PIK3 CA,CASP3,AKT1 and BCL2 as the key targets. These results suggested that WEMOL had anti hyperlipidemia effect,and its mechanism might be related to the protein expression regulation of lipid metabolism,nonalcoholic fatty liver disease and atherosclerosis related signaling pathways.


Asunto(s)
Hiperlipidemias , Moringa oleifera , Animales , Cricetinae , Dieta Alta en Grasa , Glucógeno Sintasa Quinasa 3 , Hiperlipidemias/tratamiento farmacológico , Hígado , Hojas de la Planta
4.
Zhongguo Zhong Yao Za Zhi ; 46(4): 865-876, 2021 Feb.
Artículo en Zh | MEDLINE | ID: mdl-33645091

RESUMEN

The network pharmacology and molecular docking methods were used to explore the mechanism of Jinweitai Capsules in the treatment of acute and chronic gastritis, gastric and duodenal ulcers, and chronic colitis. The chemical components of herbs in Jinweitai Capsules were collected through TCMSP, CNKI and PubMed. Target prediction was performed through PubChem and SwissTargetPrediction databases; genes relating to acute and chronic gastritis, gastric and duodenal ulcers, chronic colitis were collected from OMIM database; potential targets of Jinweitai Capsules for relevant gastrointestinal diseases were obtained by Venny analysis; DAVID database was used to perform GO and KEGG enrichment analysis; protein interactions were obtained by STRING database and visua-lized by Cytoscape; AutoDockVina was used for molecular docking of AKT1, EGFR, PTPN11 and its reverse-selected chemical components. Potential mechanisms of Jinweitai Capsules in treating relevant gastrointestinal diseases were clarified according to the results of the docking. The results showed 86 potential active ingredients of Jinweitai Capsules and 268 potential targets for treatment of acute and chronic gastritis, gastric and duodenal ulcers, and chronic colitis. KEGG pathway enrichment analysis showed that 20 pathways relating to acute and chronic gastritis, gastric and duodenal ulcers, and chronic colitis mainly involved calcium signaling pathway and chemokine signaling pathway. Molecular docking showed a good binding activity between AKT1, EGFR, PTPN11 and its reverse screening chemical components. Jinweitai Capsules may exert an effect in the treatment of acute and chronic gastritis, gastric and duodenal ulcers, and chronic colitis by acting on AKT1, EGFR, PTPN11 and other targets in 15 signal pathways relating to cell inflammation and immunity, cell proliferation and apoptosis, Helicobacter pylori infection, and gastrointestinal tract.


Asunto(s)
Medicamentos Herbarios Chinos , Enfermedades Gastrointestinales , Infecciones por Helicobacter , Helicobacter pylori , Medicina , Cápsulas , Enfermedades Gastrointestinales/tratamiento farmacológico , Humanos , Simulación del Acoplamiento Molecular
6.
Zhongguo Zhong Yao Za Zhi ; 43(23): 4608-4621, 2018 Dec.
Artículo en Zh | MEDLINE | ID: mdl-30717549

RESUMEN

The genus Orobanche, Cistanche and Boschniakia are taxonomically classified as members of Orobanchaceae. The medicinal plants of these three genera are closely related, and their traditional curative effects are similar. As representative compounds, phenethyl glycosides are predominantly dominant type both in amount and in chemical structural varieties, which are considered to be the important bioactive material basis of these genera. In this paper, phenethyl glycosides and their pharmacological activities are described in a single list. In addition, the other compounds were also reviewed in order to better compare the difference of the bioactive substances. These findings have important reference value for effective development and rational utilization of resources of medicinal plants in the family Orobanchaceae.


Asunto(s)
Cistanche , Orobanchaceae , Orobanche , Plantas Medicinales , Glicósidos
7.
Zhongguo Zhong Yao Za Zhi ; 40(21): 4218-22, 2015 Nov.
Artículo en Zh | MEDLINE | ID: mdl-27071260

RESUMEN

Orobanche caerulescens is an important medicinal resource in Orobanchaceae. The present study aims to establish methods for determination of acteoside, crenatoside, and total phenylethanoid glycosides in O. caerulescens, and determine the content in 15 samples to evaluate the resource utilization of this medicinal plant. The content of acteoside and crenatoside were quantitatively determined by HPLC, while total phenylpropanoid glycosides was estimated by UV-VIS spectrophotometry. According to the results, the content of acteoside was the highest in O. caerulescens, followed by crenatoside. The contents of acteoside, crenatoside, and total phenylethanoid glycosides were between 1.15% - 15.60%, 0.83% - 4.47%, and 6.78% - 27.43%, respectively, which had significant differences. The acquisition time has great influence on the content of main components of O. caerulescens. The content of phenylethanoid glycosides is higher in the samples which were collected at the flowering stage. The two determination methods were proved to be simple, accurate and reliable, and can be used to evaluate the quality and resource utilization of O. caerulescens.


Asunto(s)
Cromatografía Líquida de Alta Presión/métodos , Medicamentos Herbarios Chinos/análisis , Glicósidos/análisis , Orobanche/química
8.
Front Pediatr ; 12: 1374150, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39035464

RESUMEN

Degos disease also known as malignant atrophic papulosis (MAP), is an autoinflammatory disease that mainly affects small- to medium-sized arteries. Gastrointestinal and nervous system are most commonly affected systems. Herein, we reported a case of Degos disease with disease onset during infantile and had severe neurological involvement.

9.
Zhonghua Yi Xue Za Zhi ; 93(13): 984-6, 2013 Apr 02.
Artículo en Zh | MEDLINE | ID: mdl-23886260

RESUMEN

OBJECTIVE: To explore the association of HLA-DM gene with childhood systemic lupus erythematosus (SLE). METHODS: DMA and DMB genes were genotyped by sequence-based typing(SBT)in 79 SLE patients at our hospital from 2003 to 2006 and 57 normal controls. RESULTS: The frequency of DMB*0101/0102 was lower significantly in SLE patients than that in controls (5.1% vs 21.1%). And the frequency of DMB*0102/0102 in SLE patients with renal involvement was higher than that in controls (17.6% vs 1.8%, P < 0.05). The frequency of all alleles and other genotypes had no significance in SLE patients, SLE patients with different organ involvements and controls. CONCLUSION: As a protective gene, DMB*0102/0102 may be a susceptible allele for SLE patients with renal involvement.


Asunto(s)
Antígenos HLA-D/genética , Lupus Eritematoso Sistémico/genética , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Genotipo , Humanos , Masculino
10.
Zhonghua Yi Xue Za Zhi ; 93(31): 2487-9, 2013 Aug 20.
Artículo en Zh | MEDLINE | ID: mdl-24300271

RESUMEN

OBJECTIVE: To explore the correlation of serum ferritin (SF) and systemic onset juvenile idiopathic arthritis (SOJIA) so as to determine the prognostic values of SF for SOJIA. METHODS: All samples were collected from 92 juvenile idiopathic arthritis (JIA) patients at Beijing Children's Hospital between February 2005 to September 2012. Their age range was 2-15 years. There were macrophage activation syndrome (MAS, n = 25), polyarticular JIA (n = 33) and oligoarticular JIA (n = 30). And 47 healthy children and another 30 with acute infective diseases were selected as control groups respectively. Blood samples were collected and SF was measured in different disease phases.Other parameters include leucocyte, hemoglobin, platelet, C-reactive protein and erythrocyte sedimentation rate.Statistics of SF level at different groups as well as at different disease phases were performed. RESULTS: The SF level of active phase SOJIA patients was significantly higher (P < 0.01) than that in Other groups. And its level in the active phase of SOJIA was significantly higher than that in patients during the recovery phase. The SF level in patients with MAS was significantly higher than that in those without MAS. CONCLUSION: Correlated with the course of SOJIA, the level of SF may judge the disease activity and predict the outcomes of SOJIA.


Asunto(s)
Artritis Juvenil/sangre , Artritis Juvenil/diagnóstico , Ferritinas/sangre , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Masculino , Pronóstico
11.
Zhongguo Dang Dai Er Ke Za Zhi ; 15(8): 666-70, 2013 Aug.
Artículo en Zh | MEDLINE | ID: mdl-23965882

RESUMEN

OBJECTIVE: To evaluate the clinical efficacy of mycophenolate mofetil (MMF) in the treatment of systemic-onset juvenile idiopathic arthritis (SoJIA). METHODS: Thirty-five patients with a confirmed diagnosis of SoJIA who had received initial treatment were randomly divided into control (n=15), MMF1 (n=7) and MMF2 groups (n=13). The control group received conventional treatment, the MMF1 group received MMF after 2 weeks of conventional treatment that had not led to remission, and the MMF2 group received combination therapy with non-steroidal anti-inflammatory drugs, prednisone and MMF. Symptoms, signs, laboratory indices, and adverse events were observed after 2, 4, and 12 weeks of treatment, and follow-up was performed for 3-6 months. RESULTS: Before treatment, the MMF2 group had a significantly longer disease course than the control group (P<0.05). After 2 weeks of treatment, the MMF1 and MMF2 groups had a significantly lower prednisone dose and erythrocyte sedimentation rate (ESR) than the control group (P<0.05). The MMF1 group had significantly higher body temperature than the other two groups (P<0.05). After 4 weeks of treatment, the MMF1 group had a significantly lower prednisone dose and ESR than the control group (P<0.05). The MMF2 group had a significantly lower prednisone dose, body temperature (recovery to normal), white blood cell count, ESR and serum ferritin concentration than the control group (P<0.05). Body temperature was significantly lower in the MMF2 group than in the MMF1 group (P<0.05). No adverse events were observed in either the MMF1 or MMF2 groups during treatment. CONCLUSIONS: Combination therapy with MMF can lead to better control of the patient's condition, more rapid relief of clinical symptoms and reduced glucocorticoid dose. The therapy with MMF is safe in children.


Asunto(s)
Artritis Juvenil/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Ácido Micofenólico/análogos & derivados , Artritis Juvenil/sangre , Sedimentación Sanguínea , Preescolar , Femenino , Humanos , Masculino , Ácido Micofenólico/uso terapéutico , Prednisona/uso terapéutico
12.
Int J Rheum Dis ; 26(9): 1826-1829, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37166030

RESUMEN

Paraneoplastic pemphigus (PNP) is a rare autoimmune skin disease closely related to tumors, characterized by a maculopapular rash with mucosal pain, bronchiole occlusion, and respiratory failure may occur over time, even resulting in death. We report a rare case of a child with autoimmune PNP misdiagnosed as juvenile dermatomyositis (JDM), and summarize the key points of differentiation of clinical manifestations and auxiliary examinations of PNP and JDM. When the diagnosis is not clear because the patient has features not typical of JDM, then skin biopsy and other diagnostic studies should be considered prior to any immunosuppressive therapy, as this could potentially obscure and delay the diagnosis of malignancy.


Asunto(s)
Enfermedades Autoinmunes , Dermatomiositis , Síndromes Paraneoplásicos , Pénfigo , Niño , Humanos , Pénfigo/diagnóstico , Pénfigo/tratamiento farmacológico , Dermatomiositis/diagnóstico , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/complicaciones , Enfermedades Autoinmunes/complicaciones , Piel/patología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/etiología , Errores Diagnósticos/efectos adversos
13.
Rheumatol Ther ; 10(3): 507-522, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-36906693

RESUMEN

Juvenile idiopathic Arthritis (JIA) is a common rheumatic disorder in children that can cause multiple systems to be affected simultaneously, leading to severe clinical symptoms and a high mortality rate in those with pulmonary involvement. Pleurisy is the most common manifestation of pulmonary involvement. At the same time, other conditions, such as pneumonia, interstitial lung disease, occlusive bronchiectasis, and alveolar protein deposition, have been increasingly reported in recent years. This review aims to provide an overview of the clinical manifestations of JIA lung damage and the current treatment options to assist in identifying and treating JIA lung involvement.

14.
Zookeys ; 1180: 333-354, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38312323

RESUMEN

Soft scale insects (Hemiptera, Coccidae) are important pests of various agricultural and horticultural crops and ornamental plants. They have negative impacts on agriculture and forestry. The tribe Coccini represents one of the most ancient evolutionary lineages of soft scale insects. However, no complete Coccini mitochondrial genome (mitogenome) is available in public databases. Here, we described the complete mitogenome of Coccushesperidum L., 1758. The 15,566 bp mitogenome of C.hesperidum had a high A+T content (83.4%) and contained a typical set of 37 genes, with 13 protein-coding genes (PCGs), 22 transfer RNA genes (tRNAs) and two ribosomal RNA genes (rRNAs). Only seven tRNAs had the typical clover-leaf secondary structure and the remaining tRNAs lacked the DHU arm, TψC arm or both. Moreover, a comparative analysis of all reported scale insect mitogenomes from GenBank database was performed. The mitogenomes of scale insects showed high similarities in base composition and A+T content. Additionally, our phylogenetic analysis confirmed the monophyly of Coccomorpha and revealed that the archaeococcoids were the most basal lineage within Coccomorpha, while Ericeruspela and Didesmococcuskoreanus, belonging to Coccidae, were often mixed with Aclerdidae, making Coccidae a paraphyletic group. These findings expand the mitogenome database of scale insects and provide new insights on mitogenome evolution for future studies across different insect groups.

15.
World J Pediatr ; 18(6): 383-397, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35364799

RESUMEN

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. With the gradual expansion of the incidence of JIA in the population, the pathogenesis and treatment of JIA were further explored and analyzed, and JIA has achieved some success in drug therapy. DATA SOURCES: A systemic literature search was conducted on PubMed, Cochrane Library, EMBASE, ISI Web of Science, the US National Institutes of Health Ongoing Trials Register, and the EU Clinical Trials Register. Through the searching of clinical trials of JIA in recent years, we summarized the progress of the clinical treatment of JIA. RESULTS: The main treatment drugs for JIA include non-steroidal anti-inflammatory drugs, glucocorticoids, disease-modifying antirheumatic drugs and biological agents. So far, a variety of biological agents targeting the cytokines and receptors involved in its pathogenesis have been gradually approved for JIA in many countries. The application of biological agents in JIA showed good efficacy and safety, bringing unprecedented experience to children and adolescents with JIA. CONCLUSIONS: The potential and advantages of biologic agents in the treatment of JIA are significant, and the application of biologic agents in the treatment of JIA will be more and more common.


Asunto(s)
Antirreumáticos , Artritis Juvenil , Adolescente , Antirreumáticos/uso terapéutico , Artritis Juvenil/tratamiento farmacológico , Factores Biológicos/uso terapéutico , Niño , Glucocorticoides/uso terapéutico , Humanos , Incidencia , Resultado del Tratamiento
16.
Zhongguo Dang Dai Er Ke Za Zhi ; 13(8): 631-4, 2011 Aug.
Artículo en Zh | MEDLINE | ID: mdl-21849111

RESUMEN

OBJECTIVE: To evaluate the efficacy of thalidomide in the treatment of juvenile idiopathic arthritis (JIA). METHODS: Twelve children with JIA who did not respond to conventional treatment were administered with thalidomide (2 mg/kg daily). The symptoms, signs, and laboratory test results were compared before and after treatment. The thalidomide-related side effects were observed. RESULTS: The average dosage of prednisone was reduced from 1.92 ± 0.16 mg/kg•d to 0.49 ± 0.42 mg/kg•d in the 12 patients 6 months after thalidomide treatment (P<0.01). Four patients did not need prednisone treatment any more. White blood cell count, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and serum ferritin (SF) significantly decreased after treatment in all of 12 patients (P<0.01). Hemoglobin level increased to normal in 8 patients after treatment (P<0.01). The number of affected joints decreased from 5 before treatment to zero to 2 after treatment in patients with polyarticular JIA (P<0.01). Signs of hip involvement and Schober's sign turned negative in enthesitis-related cases. No thalidomide-related side effects were observed. CONCLUSIONS: Thalidomide is effective in the treatment of JIA in children who do not respond to conventional treatment.


Asunto(s)
Artritis Juvenil/tratamiento farmacológico , Talidomida/uso terapéutico , Adolescente , Artritis Juvenil/sangre , Niño , Femenino , Humanos , Masculino , Prednisona/uso terapéutico , Estudios Retrospectivos
17.
Water Res ; 195: 116995, 2021 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-33721675

RESUMEN

Membrane fouling is a prominent problem that hinders the stable and efficient operation of the reverse osmosis (RO) system for wastewater reclamation. Previous studies showed that chlorine disinfection, which was commonly used in industrial RO systems as pretreatment, could lead to significant change in microbial community structure and resulted in serious biofouling. In order to prevent biofouling during wastewater reclamation, the effect of ultraviolet (UV) disinfection on RO membrane fouling was investigated and the mechanism was also revealed in this study. With the disinfection pretreatment by UV of 20, 40 and 80 mJ/cm2, the bacteria in the feed water were inactivated significantly with a log reduction of 1.11, 2.55 and 3.61-log, respectively. However, RO membrane fouling aggravated with higher UV dosage. Especially, in the group with the UV dosage of 80 mJ/cm2, the normalized RO membrane flux decreased by 15% compared with the control group after 19-day operation. The morphology of the fouled RO membranes indicated serious biofouling in all groups. The analysis on the microbial amount of the foulants showed that the heterotrophic plate counts (HPC) and ATP content on the fouled RO membranes with and without UV disinfection were at the same level. However, the total organic carbon content of the foulants with the UV dosage of 40 and 80 mJ/cm2 was significantly higher than the control group, with higher content of proteins and polysaccharides as indicated by EEM and FTIR spectrum. Microbial community structure analysis showed that some typical UV-resistant bacteria were selected and remained on the RO membrane after disinfection with high UV dosage, including. These residual bacteria after disinfection with high UV dosage showed higher extracellular polymeric substances (EPS) secretion compared with those without UV disinfection, and thus aggravated RO membrane fouling. Thicker EPS could decrease the transmission of UV rays, and thus bacteria with higher EPS secretion might be selected after UV disinfection.


Asunto(s)
Incrustaciones Biológicas , Purificación del Agua , Desinfección , Membranas Artificiales , Ósmosis , Aguas Residuales
18.
World J Pediatr ; 16(1): 82-88, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31529313

RESUMEN

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a rare and disabling heritable connective tissue disease that is difficult to treat. This study seeks to explore the clinical characteristics, clinical manifestations, treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment. METHODS: Twenty-six children with FOP were retrospectively analyzed in terms of their onset, clinical manifestations, auxiliary examinations and treatment. RESULTS: Among the 26 cases, the youngest age of manifestation of mass was 8 days after birth, and the average age was 3 years and 2 months. The peak age was 2-5 years old. Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease. These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis, such as the neck (22/26, 84.6%), back (20/26, 76.9%), and head (13/26, 50%). Toe-finger deformity mainly manifests as symmetrical great toe deformity, or short and deformed thumb and little finger. The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection. The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs. Although not compliant with the recommended medical management of FOP, in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination. CONCLUSIONS: FOP is a rare autosomal dominant heritable disease. The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity. If the diagnosis and treatment are not performed in a timely manner, serious complications are likely to affect the prognosis. Therefore, early diagnosis and active treatment should be performed.


Asunto(s)
Miositis Osificante/diagnóstico , Miositis Osificante/tratamiento farmacológico , Antiinflamatorios no Esteroideos/uso terapéutico , Niño , Preescolar , China , Diagnóstico por Imagen , Femenino , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Lactante , Recién Nacido , Masculino , Miositis Osificante/genética , Pronóstico , Estudios Retrospectivos
20.
Chin Med J (Engl) ; 130(11): 1276-1282, 2017 Jun 05.
Artículo en Inglés | MEDLINE | ID: mdl-28524825

RESUMEN

BACKGROUND: Approximately 15-20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, neither comparing the differences between cSLE and adult-onset SLE (aSLE). The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group (CSTAR) database. METHODS: We made a prospective study of 225 cSLE patients (aged Results: The mean age of cSLE patients was 12.16 ± 2.92 years, with 187 (83.1%) females. Fever (P < 0.001) as well as mucocutaneous (P < 0.001) and renal (P = 0.006) disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects (P < 0.001). The cSLE patients were found to present more frequently with malar rash (P = 0.001; odds ratio [OR], 0.624; 95% confidence interval [CI], 0.470-0.829) but less frequently with arthritis (P < 0.001; OR, 2.013; 95% CI, 1.512-2.679) and serositis (P = 0.030; OR, 1.629; 95% CI, 1.053-2.520). There was no significant difference in SLE disease activity index scores between cSLE and aSLE groups (P = 0.478). Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients (hazard ratio 0.335 [0.170-0.658], P = 0.001). The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test (0.557, P = 0.455). CONCLUSIONS: cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difference in 5- year survival between cSLE and aSLE groups.


Asunto(s)
Lupus Eritematoso Sistémico/epidemiología , Adolescente , Adulto , Factores de Edad , Edad de Inicio , Niño , China/epidemiología , Femenino , Humanos , Lupus Eritematoso Sistémico/mortalidad , Lupus Eritematoso Sistémico/patología , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Modelos de Riesgos Proporcionales , Estudios Prospectivos , Sistema de Registros , Índice de Severidad de la Enfermedad , Adulto Joven
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