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Objective:To investigate the clinical features of ocular involvement and the application of Rose criteria in patients with relapsing polychondritis (RP).Methods:The data from RP patients with ocular involvement were collected and analyzed. Patients included must have at least one major criteria of Michet criteria and the application Rose criteria was also investigated. Demographic data of these patients was presented as percentages. The difference between types of disease onset was tested by Mann-Whitney U and comparison among groups was tested by False Discovery Rate. Results:A total of 192 patients were enrolled 98 males and 94 females. The mean age of disease onset was (42±14) (0.5-79) years old, the median disease duration (DD) was 13(0.5, 600) months. The median RP disease activity index (RPDAI) was 39(9-74) and the median RP organ damage index (RPODI) was 2.5(0.1, 108). The median RP damage index (RPDAM) was 3(1-6). The statistical significant difference was identified in median DD between groups of nose and pinna ( Z=10.775, P<0.01), nose and OEH ( Z=9.277, P<0.01), in RPODI between groups of nose and pinna ( Z=7.999, P=0.031), nose and and extra-cranial organs ( Z=8.115, P=0.030) and eye and airway involvement of RPDAM could be seen between groups ( Z=7.683, P=0.037) respectively. Ocular involvement(50.0%), auricular chondritis(21.4%) and airway chondritis(13.5%) were the top three most common symptoms at disease onset. The ocular involvement(100%), airway chondritis (75.0%) and inner ear involvement(69.3%) were the top three most frequent affected organs. All parts of eye could be involved in RP ocular damage. Single-organ involvement was 59.9%; and multi-organ involvement could be seen in 40.1% patients. Diagnostic strength was enhanced by application of Rose criteria in 171 cases fulfilled Michet criteria and 21(10.9%) cases partially fulfilled Michet criteria fulfilled Rose criteria. Active screening for organ (especially inner ear and airway) involvement would improve the rate of early diagnosis. The pinna and airway involvement suggested nose and middle-ear might be involved. Conclusion:Ocular involvement in RP can involve all parts of the eye ball. Examining the inner ear and airway may help to confirm the diagnosis. It is worthy to apply this to clinical practice.
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Objective To investigate clinical and imaging characteristics of patients with relapsing polychondritis (RP) with insidious airway involvement.Methods Data collected prospectively and consecutively for patients with the diagnosis of RP and their disease activity evaluation was analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups forquantitative datain normal distri-bution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results Two hundred and sixteen patients with complete data from Dec 1,2007 to Jul 31,2016 were enrolled with a M:F ratio of 1:1.Mean age of disease onset was (44±16)(8～86) years.The median disease duration was 12 (0.3～480) month.The median relapsing polychondritis disease activity index (RPDAI) was 35 (8～67),the median RPODI was 2.4 (0.1～84).The top three initial presentation were auricular chondritis (28.7％),airway chondritis (24.1％) and ocular involvement (22.2％) respectively.RPODI was significantly higher in auricular chondritis (4.4) (Z=-2.084,P＜0.05) and lower in nose chondritis (0.6) (Z=-2.425,P＜0.05).Up to 81.5％ of the patients were found with airway damage and 52.3％ of them were asymptomatic.Airway damage was mostly located in trachea (79.0％ 139/176) and common features on CT scan werecharacterized by airway wail thickening (72.7％,128/176).Airway narrowing was mostly seen in symptomatic patients while calcification was seen more in asymptomatic patients.Insidious hearing-loss and nose chondritis were found not related to airway damage while obvious auricular chondritis (x2=15.580,P＜0.01),ocular involvement (x2=8.105,P＜0.01) were found to be more in patients with asymptomatic airway damage.All diagnosis before RP was organ-driven.Conclusion Airway involvement in RP is one of the three most common disease initial presentation-sand half of them are asymptomatic.RPODI is a reasonable marker for disease evaluation.Routine follow-up of airway damage (wall thickness,calcification and lumen narrowing) is essential for early RP recognition.
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Objective To analyze the clinical features of chronic rhino-sinusitis (CRS) in patients with immunoglobulin G4-related disease (IgG4-RD).Methods A retrospective analysis of the data from March 2013 to July 2015 in our Department of Rheumatology was conducted in patients who were diagnosed as IgG4-RD and were follow-up for over half a year.The clinical features and the results of laboratory tests were compared between of the case group and the control group.The t test,Mann-Whitney U test and Fisher'Exact Test (n ＜40) were used to analyze the data.Results Twenty-four cases in 44 cases of IgG4-RD were complicated with CRS (54％).The mean age was (49±13) years old,with the ratio of male:female was 2∶1;With the longer disease duration [4.0 (1.0,6.3) year vs 0.5 (0.2,4.3) year,U=-2.182,P=0.041],the more the number of organs involved [4.0 (3.8,5.3) vs 3.0 (1.0,4.0),U=-2.827,P=0.005],the higher the ratio of ocular involvement (89％ vs 42％,P=0.013).The higher the percentage of eosinophils in peripheral blood [8.5％(4.0,13.8) vs 3.3％(0.8,8.5),P=0.043],the more common the allergic manifestations (61％ vs 20％,P=0.026),the higher the operation times [1.5(1.0,3.0) vs1.0(0,10),U=2.096,P=0.048] before making the definitive diagnosis than the control group A.The level of ESR/CRP (56％ vs 0,P=0.004) and the number of IgG4 positive plasma cells [57.5(50.0,66.3)/HP vs 10.0(1.8,20.0)/HP,U=4.358,P＜0.01] and the percentage of IgG4/IgG positive plasma cell (40％ vs 10％,P＜0.01) in the nasal mucosal tissues of the IgG4-related chronic rhino-sinusitis were higher than patients with ordinary CRS (the control group B),but there was no difference in the severity of sinusitis manifestations between patients with IgG4-related chronic rhino-sinusitis and the regular CRS.Conclusion IgG4-related chronic rhino-sinusitis is different from regular CRS,and is closely associated with IgG4-related ocular lesion.IgG4-related chronic rhino-sinusitis has some clinical features which are different from other phenotypes of IgG4-related diseases.
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Objective To investigate the clinical and audiological characteristics of non-elder patients with relapsing polychondritis (RP).Methods Clinical and audiological data of patients with RP under 60 years old were collected consecutively and analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups in quantitative data in normal distribution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results One hundred and seventy four patients with complete data who fulfilled the Michet criteria were enrolled with a M∶F=1∶1.1.The mean age of disease onset was (39±13) (8-60) years;the median time of disease duration was 12 (1-480) months;the median relapsing polychondritis disease activity index (RPDAI) was 38(10-77) and the median RPODI was 2.4(0.1-56).Auricular chondritis (32.8％,57/174),ocular involvement (24.7％,43/174) and airway chonchritis (21.3％,37/174) were the top three onset-pattern.All parts of external,middle and inner ear were involved in RP.Inner-ear damage was the most common (95.4％,166/174) with insidious cochlea and vestibule equally distributed.Auricular chondritis was predominant in external ear involvement (55.2％,96/174);ET dysfunction was included in eardrum abnormalities of neglected middle-ear involvement (29.9％,52/174).Positive HL by active detection was 71.8％(125/174) with 14.3(25/174) HL fulfilled world health organization (WHO)-2006 criteria,including 52.0％(13/25) disabling HL.Sensorineural hearing loss (SNHL) was predominant in RP after 21 years old while 60.0％(6/10) was seen in childhood RP with non-SNHL.At least two parts of ear involvement were seen in almost all patients with heavy overlap.Conclusion All parts of ear are involved in non-elder RP with age related clinical characteristics.Active detection is a key to find insidious middle-and inner-ear involvement for early RP recognition.RPODI is a potential marker for RP evaluation.
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Objective To investigate the clinical characteristics of granulomatosis with polyangiitis (GPA) patients with scleritis.Methods The completed clinical data of 102 GPA patients from March 2012 to July 2016 in Capital Medical University, Tong Ren Hospital were collected. Altogether 43 GPA patients with scleritis were enrolled, and the clinical data were analyzed respectively. The t test, Mann-Whitney U test and x2testwere used to analyze the data. Results The most common ocular manifestation in GPA was scleritis. The incidence of renal damage(37.2% vs 11.9%,x2=9.148,P=0.002),disease activity(BVAS/GPA score 8.9 vs 5.1,Z=7.847, P=0.000),DEI score (7 vs 4,Z=5.919, P=0.000),inflammation index(56 mm/1 h vs 36 mm/1 h, Z=2.456, P=0.016; CRP 51 mg/L vs 8 mg/L, Z=3.748, P=0.000), neutrophil percent (70% vs 61%, t=3.993, P=0.000),complement 3(1.2 g/L vs 1.0 g/L, t=2.652, P=0.009),plasma fibrinogen(3.5 g/L vs 3.3 g/L, t=3.947, P=0.000), D-dimmer (0.5 mg/L FEU vs 0.3 mg/L FEU, Z=2.371, P=0.018) and ANCA positive rate (90.7% vs 57.6%,x2=13.369,P=0.000)were higher in patients with scleritis,as well as a poor prognosis(2009-FFS 0.4 vs 0.1, Z=2.600, P=0.009). However, a lower rate of subglottic involvement was detected in scleritis group (4.7% vs 20.3%, x2=5.169, P=0.023). There were no significant differences between GPA patients with scleritis and episcleritis in clinical feature, disease activity, inflammatory index and prognosis. Aggressive immunosuppres-sive treatment was appropriate for GPA with scleritis. Tumor necrosis factor (TNF)-аinhibitor was an effective treatment. Conclusion GPA with scleritis usually suffered from higher disease activity index,broader DEI,as well as poorer prognosis. Aggressive treatment should be initiated in order to achieve disease remission. Subglottic involvement is less common in patients with scleritis. The phenotype of scleritis is not associated with disease severity.
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Objective To investigate the impact of AG490 on the blood-brain barrier (BBB ) permeability and the expression of interleukin-6 (IL-6 )and tumor necrosis factor-α(TNF-α)after traumatic brain injury (TBI)in rats. Methods A total of 144 healthy male SD rats were randomly divided into a control group,a trauma group,and an AG490 intervention group (n=48 in each group). The rats in each group were redivided into four subgroups (4 h,1 d,3 d,and 7 d subgroups)according to the time points after cerebral injury (n=12 in each subgroup). A brain trauma models were induced by hydraulic shock method. Evans blue was used to determine the changes of the BBB permeability after cerebral injury in each group. Real-time fluorescence quantitative PCR was to detect the expression levels of TNF-αand IL-6 mRNA in rat brain tissue. Immunohistochemistry was used to detect the expression of human phospho tyrosine kinase (P-JAK2). Results (1)The permeability of BBB:The permeability of BBB increased at 4 h,1 d,3 d and 7 d after brain injury in the trauma group (Evans blue permeation:10. 4 ± 1. 2,16. 0 ± 1. 4,22. 3 ± 2. 0,and 8. 4 ± 0. 9μg/g,respectively). Compared with the control group, there were significant differences (all P<0. 01). The Evans blue permeation of the AG490 intervention group were 9. 1 ± 1. 0,12. 8 ± 1. 1,17. 5 ± 1. 4 and 7. 1 ± 0. 8μg/g,respectively at each time point,and they were all significantly lower than those of the trauma group (all P<0. 01). (2)The expression of IL-6 and TNF-α mRNA:The expression levels of IL-6 mRNA and TNF-α mRNA at 4 h,1 d,3 d and 7 d after traumatic brain injury in the trauma group were 2. 31 ± 0. 35,2. 73 ± 0. 35,3. 32 ± 0. 29,2. 14 ± 0. 24 and 7. 46 ± 1. 18,9. 42 ± 1. 54,13. 76 ± 1. 89,and 6. 28 ± 1. 00,respectively,they were all significantly higher than those of the control group (all P<0. 01). The expression levels of IL-6 mRNA and TNF-α mRNA of the AG490 intervention group were 1. 14 ± 0. 22,1. 54 ± 0. 23,1. 94 ± 0. 32,1. 26 ± 0. 21 and 5. 57 ± 0. 88, 7. 78 ± 1. 02,11. 51 ± 1. 29,and 5. 05 ± 0. 97,respectively,they were all lower than those of the trauma group,but they still higher than the control group. There were significant differences (all P<0. 01). (3 )The expression of P-JAK2:The expression levels of P-JAK2-positive cells at each time point after traumatic brain injury in the trauma group were significantly higher than the control group (all P<0. 01),they were 17. 4 ± 2. 7,56. 2 ± 6. 7,26. 1 ± 5. 4,and 15. 3 ± 2. 5,respectively;those of the AG490 intervention group were 12. 2 ± 1. 4,41. 5 ± 4. 6,19. 4 ± 4. 1,and 9. 6 ± 2. 0,respectively,they were all lower than those of the trauma group,but still higher than the control group. There were significant differences (all P<0. 01). Conclusion During the acute phase after TBI,AG490 may activate the factor signaling pathways by inhibiting the non-receptor tyrosine kinase/signal transduction and transcription,significantly inhibit the expression of brain tissue inflammatory cytokines IL-6 IL-6 and TNF-α,reduce the BBB damage,and help to reduce secondary brain injury.
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Objective To investigate the clinical features of child-onset relapsing polychondritis (CORP). Methods The clinical manifestations of 11 CORP cases were analyzed retrospectively. Results Eleven cases of CORP were collected with Male to Female ratio of 5 ∶6; the mean age of disease onset was (11±4)(3-15) years; and the mean age of final diagnosis was(16±5)(6-22) years-old. The mean time of delay of diagnosis was(55±46)(2-144) month. Nine cases were simple CORP and 2 cases were concurred with SLE and pSS respectively. Laryngotracheal symptoms as initial presentation was seen in 7/11(64%) of cases, followed by rhinitis, eye involvement and arthritis. Laryngotracheal involvement occurred in 10/11 (91%) cases and 6/11(55%) underwent trachectomy when the diagnosis was made, followed by eye and nose involvement (saddle nose)(46% each), involvement of auricular cartilage,vestibule disorder and arthritis (36% each). Elevation of ESR or CRP(36%) were observed and positive autoantibody(18%) were detected in serology study; 9/11 (82%) were treated with steroid combined immunosuppressant. All cases were improvedor stable except 2 cases with exacerbation after upper respiratory infection during the follow-up of mean(27±15)(4-48) month. Conclusion Laryngopharyngeal involvement is the most common phenomenon in CORP and is associated with poor prognosis due to diagnosis delay. Imaging investigation may provide useful evidence for early diagnosis. Aggressive treatment is helpful in improving outcome.
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This study was aimed to explore the curative effect of Zhuo-Du-Qing (ZDQ) particles on diabetic cystopa-thy (DCP) based on the theory of turbid toxin. A total of 100 DCP patients were randomly divided into the control group with 47 cases and the treatment group with 53 cases. Mecobalamin tablets was given in the control group. And ZDQ particles were given to the treatment group. The treatment course was 3 months. The observation was made on the bladder residual urine (BRU), clinical manifestation integral, hemodynamic indexes, HOMA-IR and HbA1c before and after the treatment. The results showed that compared with pretreatment, the BRU, clinical manifestation integral, hemodynamic indexes, and HOMA-IR were significantly reduced (P < 0.01). Compared with the control group, the BRU, clinical manifestation integral, hemodynamic indexes, and HOMA-IR were also significantly reduced (P <0.01). The effective rate in the treatment group was 81.1%, which was significantly higher than 59.6% in the control group (P< 0.05). There were no obvious changes on the blood routine examination, urine routine examination, stool routine examination, liver function, renal function, electrocardiogram and so on before and after treatment. It was con-cluded that the treatment of DCP with ZDQ particles was safe and effective. To remove toxin and descend turbid may be another effective treatment method for DCP. The occurrence and development of DCP were closely related to the turbid toxin.
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Calcium supplement is needed for Chinese people.Calcium antagonists are also used in China.Authors suggest that calcium owns dual direction effects like a double-edged sword.Based on the knowledge of calcium metabolism,the adverse drugs reactions of excessive calcium supplement and the harmful effects of cellular calcium overload are introduced.Scientific and efficient application of calcium agents and calcium antagonists is expected.
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Objective To investigate the incidence,pathogenesis,clinical features,principle in treatment and prognosis of pulmonary hypertension (PHT) secondary to systemic lupus erythematosus (SLE).Methods The clinical features,management and outcome of 6 patients with PHT secondary to SLE were reported and literature was reviewed.Results Six cases of PHT related to SLE,mean age 39 5 year,mean period of SLE 3 2 year,mean pulmonary artery pressure 61 8 mm Hg,3 were severe PHT.Organ involvements were seen in all cases:6 with cardial involvement,of which 2 with heart failure;5 with renal impairment;3 with pulmonary lesions other than PHT,2 with hematological involvement;2 with thrombosis and 2 with Raynaund′s phenomenon (RP).Remarkable decrease of complement C3 was seen in all cases,anti Scl 70 and antiphospholipid antibody (ACL) positive were seen in 2 cases as well.No neurological involvement was found.After early comprehensive and intensive treatment,normalized PHT was seen in all but one case,concomitant with decrease in SLE activity.After 9 months′ follow up,1 case died from dysrhythmia,the rest kept stable,except RP.Conclusion PHT is a severe complication of SLE,often accompanied by multi organ involvement and poor outcome;Intensive and comprehensive treatment should be made early,especially for primary affection and its cause.Vasodialator (usually calcium channel blocker) and anticoagulant is a principle medication and prostacyclin should be considered for refractory cases.Better outcome can be achieved for those who are responsive to the treatment.
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The recently successful cloning of extracellular calcium sensing receptor (CaR) has revealed its vital role in adjusting and maintaining the homeostasis calcium or even other mineral ion in the body. CaR belongs to the big family of G-protein coupled receptors, and has 1079 amino acids in its structure. CaR is broadly distributed in a variety of tissues, including parathyroid, thyroid C cells, kidney, intestine and bone. So far, three disorders of extracellular calcium homeostasis have been linked with abnormalities of CaR function. Agonists and antagonists of CaR may be proved useful in the treatment of the diseases due to calcium metabolic disorders.
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The total aralosides extracted from Aralia elata (Miq) Seem. were studied in mice ( 50 mg/kg/d for 7 d ) to investigate the pharmacological mechanisms on the molecular level.The results showed that aralosides can obviously stimulate the production of PGE2、PGF2, and cAMP but reduce the cGMP level and nave no effect on histamine release.It is suggested that the changes in the level of cyclic nucleotide might be due to the alternations of PGs, which is now been suspected as the "Second Messenger" . These findings could explain in part the pharmacological activities of aralosides on the molecular level.
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Calcium ion is involved in many processes of cellular living activities. It is critically important for maintaining normal functions of human body. The review will discuss intracellular calcium regulation, distribution changes of calcium in ischemic cerebravascular and cardiovascular diseases, and intracellular intervention of calcium by specific drugs.
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In order to explore the mechanism of cardiac damage by PTH, the release of PGI2 from heart cells was measured. The results showed that bPTH ( 1-34) increased the release of 6-keto-PGFla from heart cells in a dose dependent manner. Calcium inophone A23187 also increased the 6-keto-PGF1? release, while EDTA and verapamil reduced it. These suggest that the PGI2 synthesis in heart cells was affected by intra and extracellular calcium. The significance of bPTH( 1-34) induced increase of PGI2 synthesis might be associated with interference of energy metabolism, and then, cell damage.
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To date, pancreatitis lacks of specific pharmacological therapy. Recently, it has been found that cytosolic Ca2+ overload causes pancreatitis. This article summarized the earliest events of Ca2+ transport in pancreatic acinar cell during pancreatitis and the influence of bile, alcohol, and hyperlipidaemia.Abnormal Ca2+ signal can be inhibited by caffeine. Based on these results, potential therapeutic targets are suggested.
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The choice of medicines is a key point in the treatment of hypertension.There are hundreds of antihypertensive drugs in China,including Traditional Chinese Medicines(TCM),chemical medicines and all kinds of compound preparation.But the rates of treatment and control of hypertension in China are very low,the situation of hypertension management in China is critical.In this article we compared the differences in the drug treatment among Chinese,American and European guidelines for the management of hypertension,and discussed the principles of choosing antihypertensive drugs.Chinese,American and European guidelines all mention that the specific drug classes may differ in some effect or in special groups of patients,so certain compelling indication requires certain antihypertensive drug classes.Three guidelines also emphasize the benefits obtained from the combination therapy and recommend the long-acting drugs or preparations with 24 h efficacy.The main differences among the three guidelines is whether to recommend the firstline drugs.Thiazide-type diuretics are recommended by American guideline(JNC7) in drug treatment for most patients with uncomplicated hypertension.But the European and Chinese guidelines do not recommend the first-line drugs,and the major classes of antihypertensive agents are suitable for the initiation and maintenance of therapy.Traditional Chinese medicines are invaluable resource of China,all kinds of antihypertensive TCM are widely used in the clinical,but owing to the deficiency of the high-quality evidence for TCM,the part of TCM is not included in the 2004 Chinese guideline for the management of hypertension.The choice of antihypertensive drugs is determined by its efficacy and safety.When safety and efficacy are equal,the lowest cost drug should be preferred.For the majority of patients without a compelling indication for another class of drug,a low dose of thiazides diuretic should be considered as the first choice in China.2004 Chinese guideline for the management of hypertension is based on many scientific evidences,and its publication is very important for the prevention and cure of hypertension in China.Nowadays,we must strengthen the dissemination and implementation of guideline,promote the rational use of antihypertensive drugs and improve the control rate of hypertensive in China.
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Store operated calcium channels (SOCCs) are referred to as plasma membrane calcium channels that are opened in response to a depletion of intracellular calcium stores. SOCCs are broadly distributed in excitable and non excitable cells, and may play important roles in conducting intracellular calcium signals, modulations of cell functions and gene expression. Although the current understanding of SOCCs related mechanism is still limited, the physiological significance of SOCCs and their possible links to certain diseases have been suggested. Future study of highly selective SOCCs modulating agents may promote not only related research, but also the development of novel therapeutic drugs.
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In order to obtain some evidences of the role of parathyroid hormone (PTH) in regulating calcium metabolism, and explore the mechanisms by which PTH regulates calcium in the heart, 45Ca was used to study the influence of bovine PTH1-34 fragment (bPTH1-34) on calcium transsacolemma. The results indicate that bPTH1-34(l?I0-7mol ? L-1) increases beating rate of heart cells under normal state and it markedly increases the rapid (5 min) and the slow (120 min) phases of 45Ca influx in heartcells (under normal, KC1 10-5 mol?L-1 high K+depolarised, NA 10-7mol?L-1receptors activated states), but it could also reduce 45Ca efflux from the cultured heart cells. It suggests that the mechanisms caused by bPTH1-34 which enhances the cytosolic calcium concentration and myocardial beating rate may be related with cAMP level elevated in heart cells.
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Lymphokine-osteoclast activating factor(OAF) is a potent mediator of bone resorption. PGs which mediate bone resorption are necessary for OAF. It has been reported that mononuclear is an important factor in bone resorption; and circulating monocytes may be osteoclast precursors,but it is not clear at present. We used CT and PTH which are potent physiological calcium-regulator hormones to stimulated mono-nuclear to study it's role for bone resorption. Mononuclear was isolated from normal human peripheral blood by Ficoll-Hypaque. After cultured in C02 incubator at 37℃ for 24h. 0.05g/L PTH and 100U/L CT were added and incubated in the same condation. After 2.5min, 5min, 10min, and 15min cAMP was measured by RIA. In the sam way PGE was determined. Our experiment exhibited that circulating mononuclear responds to CT and PTH with an increase in cAMP firstly, and enhances PGE production by CT and PTH stimulated mononuclear secondly. The results suggest that mononuclear participated in the regulation of bone resorption by PGE, OAF and other factor.