Effect of Moshen Decoction for the patients with idiopathic membranous nephropathy and spleen-kidney qi deficiency syndrome / 国际中医中药杂志

Objective:To evaluate the curative effect of Moshen Decoction combined with routine western medicine on idiopathic membranous nephropathy (IMN) of spleen-kidney qi deficiency syndrome and explore its influences on renal function, C1q, PLA2R and E-cadherin levels.Methods:A total of 62 patients with IMN meeting inclusion criteria in the hospital were enrolled between January 2018 and January 2021. According to simple random grouping method, they were divided into control group (hormones combined with cyclophosphamide) and observation group (Moshen Decoction on basis of control group), 31 in each group. Both groups were treated continuously for 6 months. Before and after treatment, TCM syndromes were scored. The levels of blood urea nitrogen (BUN), serum creatinine (SCr), cystatin C (Cys-C), anti-phospholipase A2 receptor (PLA2R) and E-cadherin (EC) were detected by ELISA. The level of serum complement C1q was detected by immunoturbidimetry. The 24 h urine was collected for quantitative determination by full-automatic biochemical analyzer. The adverse events during treatment were observed and recorded. And clinical curative effect was evaluated.Results:The differences in total response rate between observation group and control group were statistically significant [93.55% (29/31) vs. 74.19% (23/31); χ2=4.29, P=0.038]. After treatment, scores of TCM syndromes (edema of lower limbs, fatigue and poor appetite, lusterless complexion) in observation group were significantly lower than those in the control group ( t=10.07, 10.80, 4.34, 4.57, P<0.001). After treatment, levels of serum Cys-C [(0.51±0.05) mg/L vs. (0.55±0.06) mg/L, t=2.85], 24 h urine protein quantification [(0.95±0.19) g vs. (1.38±0.23) g, t=13.32] in observation group were lower than those in the control group ( P<0.01), and levels of serum PLA2R [(17.53±1.84) Ru/ml vs. (19.62±2.05) Ru/ml, t=4.22], EC [(2.74±0.26) μg/L vs. (3.05±0.37) μg/L, t=3.82] and complement C1q [(152.34±15.62) mg/L vs. (169.33±16.77) mg/L, t=4.13] in observation group were significantly lower than those in the control group ( P<0.01). During treatment, there was no significant difference in incidence of adverse events between observation group and control group [12.90% (4/31) vs. 16.13% (5/31); χ2=0.13, P=0.781]. Conclusion:Moshen Decoction combined with routine western medicine can improve renal function and clinical curative effect in patients with IMN of spleen-kidney qi deficiency syndrome. Its mechanism of action may be related to reducing urine protein, complement C1q, PLA2R and EC.

Changes of Serum Complement C1q in Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To analyze the change of serum C1q in the course of multiple myeloma (MM) and its correlation with clinical characteristics.@*METHODS@#A total of 138 newly diagnosed MM patients in Zhongnan Hospital of Wuhan University from June 2016 to December 2019 were selected as research objects, during the same period 50 age-matched anemia patients, 50 lymphoma patients, 50 leukemia patients, and 50 myelodysplastic syndrome (MDS) patients were selected as control groups. All the patients met WHO disease classification, and were definitely diagnosed by pathology or bone marrow smear/biopsy. The changes of C1q between MM patients and control group, as well as in different therapeutic responses of MM patients before and after treatment were compared, also the difference of clinical characteristics among MM patients with different C1q level, so as to analyze risk factors which led to C1q decline.@*RESULTS@#The average value of C1q in MM patients was (128.18±51.24) mg/L, which was significantly lower than control group (P<0.01). The levels of white blood cell, platelet (PLT), hemoglobin (Hb), serum calcium, albumin, lactate dehydrogenase (LDH) in newly diagnosed high C1q group were significantly higher than those in low C1q group (P<0.05). Logistic analysis showed that the levels of PLT, Hb, albumin, and LDH in newly diagnosed high C1q group were higher than those in low C1q group (r=0.248, r=0.394, r=0.405, r=0.295). After treatment, the levels of C1q in MM patients with complete remission and very good partial remission were significantly higher than before treatment (P<0.05), while those with partial remission and stable disease also increased but not significantly (P>0.05).@*CONCLUSION@#The C1q level in MM patients is significantly lower than that in patients with other hematologic system diseases, and it increases with the remission of the disease after treatment.

Correlation of serum complement C1q level with metabolic syndrome / 中华检验医学杂志

Objectives To analyze the changes of serum complement C1q level in patients with metabolic syndrome (MS) and investigate whether it is associated with lipid metabolism and glycometabolism. Methods In a cross-sectional study, the subjects were selected as the patients and healthy people who went to the second xiangya hospital of central south university from July 2017 to June 2018. A total of 152 MS patients were enrolled and another 90 healthy subjects were enrolled as control group. Anthropometry parameters such as body mass index (BMI), systolic blood pressure (SBP), diastolic blood pressure (DBP) were measured. Serum concentrations of C1q and other biochemical indexes including blood glucose (GLU), triglycerides (TG), total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C) and low-density lipoprotein cholesterol (LDL-C) were measured in all groups. The correlations between C1q and these parameters were analyzed by spearman's rho test and the clinical value of C1q in predicting MS was further evaluated by stepwise multiple linear regression analysis. Results MS group had higher serum C1q levels (244.34±62.66) mg/L compared with the control group (202.37±35.92) mg/L (t=-6.250, P=0.000). C1q levels (244.34±62.66) mg/L were positively associated with TG levels [2.34(1.89, 3.62)] mmol/L (r=0.245, P=0.001), TC levels (4.91±1.26) mmol/L (r=0.398, P=0.000), LDL-C levels (3.23±1.03) mmol/L (r=0.325, P=0.000) in MS group, While C1q levels (258.92±69.59)mg/L were positively associated with SBP (144.76 ± 22.94) mmHg (r=0.388, P=0.018), TG levels [2.65(1.87, 3.82)] mmol / L (r=0.482, P=0.003), TC levels (5.18±1.31) mmol/L (r=0.529,P=0.001) in MS patients with obesity. The stepwise multiple regression analysis showed that TG levels were independently correlated with serum C1q levels both in MS patients (β=0.302, P=0.000) and in MS patients with obesity (β=0.653, P=0.000) after adjusting for age, gender and other biochemical markers. Conclusions MS patients had higher C1q levels than healthy subjects and serum C1q levels were closely positive related to harmful lipid profiles. Serum TG level was an independent influencing factor of serum C1q in MS patients.

Diagnostic value of pathological features of atypical membranous nephropathy / 中华肾脏病杂志

Objective To evaluate the diagnostic value of pathological features of atypical membranous nephropathy (AMN). Methods Ninety - one patients with AMN diagnosed by renal biopsy during 2011 and 2017 were enrolled in this study. On the basis of M - type phospholipase A2 receptor (PLA2R) and thrombospondin type - 1 domain - containing 7A protein (THSD7A) by immunohistochemistry, patients were divided into AMN group (25 cases without PLA2R and THSD7A) and idiopathic membranous nephropathy (IMN) group (66 cases with positive PLA2R or THSD7A). The results of immunofluorescence (IF), light microscopy (LM) and electron microscopy (EM) of these two groups were compared, and the parameters with statistical difference were screened out in order to assess their value in the diagnosis of AMN in fourfold table. Results IF results showed that in AMN group the proportions of IgG deposition on capillary wall and mesangial area as well as positive otherIgG subclasses and complement C1q but negative IgG4 were significantly higher than those in IMN group (respectively, 56.0％ vs 12.1％ , 44.0％ vs 0, both P＜0.05). Their diagnostic specificities for AMN were 87.9％ and 100.0％, respectively. However, the positive rates of IgG accompanied with IgA and/or IgM, predominant IgG4 with other IgG subclasses and complement C1q in two groups were not significantly different (all P＞0.05). LM results showed that the proportions of false double track sign on basement membrane and fuchsinophilic proteins under epithelium, endothelium, basement membrane and mesangial region in AMN group were significantly higher than those in IMN group (respectively, 36.0％ vs 0, 44.0％ vs 1.5％, both P＜0.05). Their diagnostic specificities for AMN were 100.0％ and 98.5％ , respectively. However, the scores of mesangial cell proliferation of these two groups showed no significantly difference (P＞0.05). EM results showed that the rate of endothelial electron dense deposits in AMN group was significantly higher than that in IMN group (36.0％ vs 1.5％, P＜0.05), and its diagnostic specificity for AMN was 98.5％. Conclusions IgG deposition on both capillary wall and mesangial area, positive other IgG subclasses and C1q with negative IgG4, false -double contour sign, multi - site fuchsinophilic deposits and endothelial electron dense deposits may help for the AMN diagnosis in the absence of PLA2R and THSD7A related data.

Effect of sarcopenia on skeletal muscle and cardiac function in elderly patients with chronic heart failure / 中华全科医师杂志

Objective To investigate the effect of sarcopenia on the skeletal muscle and cardiac function in elderly patients with chronic heart failure (CHF).Methods Sixty patients with CHF and sarcopenia and 60 sex and age-matched CHF patients without sarcopenia were enrolled from September 2014 to December 2015.The skeletal mass was evaluated by fat-free mass index (FFMI) and muscle function was evaluated by gait speed (GS),hand strength (HS) and the simple physical performance battery (SPPB).The cardiac function was accessed by a 6-min walk distance (6-MWD) and left ventricular ejection fraction (LVEF).Furthermore,the serum inflammation cytokines IL-6,TNF-α,and skeletal muscle biomarker C 1q were measured.Results The CHF patients with sarcopenia had lower values for skeletal muscle mass:FFMI [(17.68±0.74) vs.(18.34±0.54)kg/m2,F=33.696,P＜0.05] and lower muscle function:HS [(17.26±4.20)vs.(28.85±6.43)kg,F=136.54,P＜0.05],GS [(0.65±0.11) vs.(0.90±0.10)m/s,F=-12.922,P＜0.05],SPPB [(6.45±2.07) vs.(7.65± 1.76),t=-3.452,P＜0.05].And the cardiac function decreased significantly in patients with sarcopenia:6-MWD [(253.76 ± 72.62) vs.(340.91 ± 55.78)m,F=54.350,P＜0.05],LVEF [(39.12 ± 7.02)vs.(43.83±5.81)％,t=16.060,P＜0.05].Serum IL-6/TNF-α/C1q levels were significantly elevated:IL-6[(14.12± 1.40) vs.(13.46±1.06) ng/L,F=8.513,P＜0.05],TNF-α [(443.43±28.06) vs.(299.37±21.53)ng/L,t=31.556,P＜0.05],C1q[(578.92±23.63) vs.(504.1 1±41.77)ng/L,F=145.78,P＜0.05].Conclusion The CHF patients with sarcopenia present less skeletal muscle mass,poorer skeletal function and reduced cardiac function,and higher inflammation levels.

Correlation between serum complement C1q and microalbuminuria in patients with type 2 diabetes mellitus / 中国医师进修杂志

Objective To explore the correlation between serum complement C1q and microalbuminuria (MAU) in patients with type 2 diabetes mellitus (T2DM). Methods Two hundred and thirty patients with T2DM from January 2017 to July 2018 were selected. The patients were divided into 3 groups according to the MAU: group A (100 patients with MAU≤30 mg/L), group B (80 patients with 30 mg/L ＜ MAU ≤ 300 mg/L) and group C (50 patients with MAU ＞300 mg/L). The clinical data were recorded and serum complement C1q was measured by immunoturbidimetry. The homeostatic model assessment C-peptide resistance index (HOMA-CR) was calculated. The correlation was analyzed by Spearman correlation analysis, and multivariate Logistic regression analysis was performed with MAU ＞300 mg/L as dependent variable. Results The complement C1q in group C was significantly higher than that in group A and group B: (198.72 ± 43.25) mg/L vs. (173.42 ± 29.36) and (181.57 ± 35.79) mg/L, and there was statistical difference (P＜0.05). Spearman correlation analysis result showed that serum complement C1q had positive correlation with body mass index (BMI), fasting C-peptide (FCP), HOMA-CR and MAU in patients with T2DM (r = 0.22, 0.26, 0.31 and 0.38; P＜0.05). Multivariate Logistic regression analysis result showed that course of disease, glycosylated hemoglobin A1c (HbA1c) and complement C1q were the independent risk factor of MAU＞300 mg/L in patients with T2DM (P＜0.01). Conclusions In patients with T2DM, serum complement C1q has positive correlation with MAU, and it is the independent risk factor of MAU＞300 mg/L. The serum complement C1q may be one of the indicators of large amount of MAU.

Expression and clinical significance of serum CysC and CTRP9 levels in patients with essential hypertension with different blood pressure grades / 国际检验医学杂志

Objective To explore the expression and clinical significance of serum cystatin C (CysC) and complement-C1q/TNF-related protein 9 (CTRP9) levels in patients with essential hypertension with with different blood pressure grades.Methods 80patients with essential hypertension who were treated in our hospital were selected as the observation group.According to the difference of blood pressure level, the patients in the observation group were divided into 1grade hypertension group (24cases) , 2grade hypertension group (36cases) and 3grade hypertension group (20cases) .In addition, 50healthy volunteers who were received physical examination in our hospital were selected as the control group.The levels of CysC, CTRP9, ankle brachial pulse wave velocity (baPWV) , carotid intima media thickness (cIMT) , mean arterial pressure (MAP) , high-density lipoprotein cholesterol (HDL-C) and low density lipoprotein cholesterol (LDL-C) were detected in all subjects.The correlation between CysC, CTRP9and baPWV, cIMT, MAP, HDL-C and LDL-C in patients with essential hypertension was analyzed.Results The level of CysC was positively correlated with baPWV, cIMT and MAP (r=0.556, 0.488, 0.369, P<0.05) , and the level of CTRP9was negatively correlated with baPWV, cIMT, MAP and LDL-C (r=-0.437, -0.397, -0.296, -0.203, P<0.05) .Conclusion The expression level of CysC and CTRP9in the serum of patients with essential hypertension is related to blood pressure classification and atherosclerosis related indexes, serum levels of CysC and CTRP9can be used to assess blood pressure and atherosclerosis.

The role of complement signaling pathway in the pathogenesis of neuromyelitis optica / 中华眼底病杂志

Neuromyelitis optica (NMO) is a kind of demyelinating disorder that preferentially affects the optic nerves and spinal cord and results in permanent vision loss.NMO pathogenesis is thought to involve binding of anti-aquaporin-4 (AQP4) auto-antibodies to astrocytes,which causes complement-dependent cytotoxicity (CDC) and downstream inflammation leading to oligodendrocyte and neuronal injury.Vasculocentric deposition of activated complement is a prominent feature of NMO pathology.In recent years,a number of groups have found complements play an important role in the pathogenesis of NMO,and basic researches in NMO therapy due to its specificity and uniformity.Its inhibition would protect against proteins in the classical complement pathway so that cure the disease.This review will expound the the role of complement signaling pathway in the pathogenesis of NMO,and provide reference for a more in-depth understanding and clinical treatments of NMO.

Lipoprotein-associated phospholipase A2, C-reactive protein, complement component C1q and homocysteine levels in patients with Alzheimer's disease / 中华老年医学杂志

Objective To investigate serum levels of lipoprotein-associated phospholipase A2 (Lp-PLA2),high-sensitivity C-reactive protein (hs-CRP),complement component C1q (C1q) and homocysteine(HCY) in patients with Alzheimer's disease(AD),in order to provide a basis for establishing laboratory markers in AD patients.Methods One hundred AD patients and one hundred healthy controls from Beijing Hospital were selected.Serum levels of Lp-PLA2,C1q,hs-CRP and HCY were determined using a biochemistry analyzer.Serum levels of amyloid β-protein 40(Aβ40)and Aβ42 were measured using enzyme-linked immunosorbent assays.Results Serum levels of Aβ40,Aβ42,Lp-PLA2,hs-CRP,C1 q and HCY were higher in AD patients than in the control group[(66.0±14.0) pmol/L vs.(7.1±8.2) pmol/L,(7.2±1.4) pmol/L vs.(1.9±1.7) pmol/L,(510.6±140.1)U/L vs.(419.0±91.8) U/L,(2.8±3.4) mg/L vs.(1.2±0.7) mg/L,(218.0±58.4) mg/L vs.(194.8 ± 27.7) mg/L and (18.8 ± 9.3) μmol/L vs.(14.9 ± 5.2) μmol/L,all P ＜ 0.01],and the differences were greater in female subjects than in male subjectss.Conclusions High serum levels of Lp-PLA2,C1q,hs-CRP and HCY may be associated with Alzheimer's disease,while the exact relationships need to be further investigated.

Ehlers-Danlos syndrome VIII with novel C1R variant accompanying white matter changes

Ehlers-Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intractable periodontal inflammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identified in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodontitis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without definite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inflammation processes such as changes in brain white matter.

The clinical research of donor specific antibody in liver transplantation / 中华器官移植杂志

Objective To analyze the donor specific antibody (DSA) in liver transplantation,and discuss the therapeutic schemes.Methods We retrospectively analyzed prospectively collected samples from 139 cases of liver transplantation from September 1,2013 to July 1,2015.Luminex assays were applied to determine human leukocyte antigen,panel reactive antibody (PRA).For PRA positive cases,DSA,C1q and C4d were detected,and liver biopsy was done.Results Of 139 cases enrolled,there were 12 cases positive for DSAs,including 2 cases of PreDSA:1 case of Ⅰ DSA (HLA-A mismatch),and 1 case of Ⅱ DSA (HLA-DQ mismatch).Ten cases of de novo DSA (including 1 case of PreDSA) all were HLA-DQ mismatch.The liver biopsy on 5 cases showed hepatic fibrosis,early rejection and intrahepatic cholestasis,and only 2 cases showed positive C4d.Of 6 cases of DSA,5 cases showed positive C1q.In the patients positive for DSA,tacrolimus dose was adjusted postoperatively,adding mycophenolatemofetil or increasing its dose,or methylprednisolone and immunoglobulin given.Conclusion DSAs are important indicators of sensitized recipients in liver transplantation,associated with trends toward worse outcomes in patients or allografts.The monitoring of DSA is requisite in order to adjust the immunosuppressant.

Male patient with C1q nephropathy and rheumatoid arthritis: A case report / Hombre con nefropatía C1q y artritis reumatoide: reporte de un caso

Abstract C1q nephropathy was first described in 1985 as a process of glomerulonephritis with mesangial C1q deposit. The histology is similar to lupus nephritis, and was initially described as being seronegative renal lupus. However, these two entities are now considered different pathological processes. Its association with rheumatoid arthritis is unusual, and there are no cases with a similar presentation reported in the literature. In this article, the case is presented of a man who developed both these conditions.

Resumen La nefropatía C1q, se describió por primera vez en 1985, como un proceso de glomerulonefritis con depósito mesangial de C1q, histológicamente similar a la nefritis lúpica, siendo inicialmente descrita como lupus renal seronegativo, sin embargo, estas dos entidades se consideran actualmente como procesos patológicos diferentes. Su asociación con artritis reumatoide es inusual y la literatura no reporta casos con presentación similar. A continuación, presentamos el caso de un hombre que desarrolla estas dos entidades.

Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

Abstract: Background: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. Objective: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. Results: Five members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. Study limitations: Small sample size of participants. Conclusion: Patients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

Serum CTRP1 level and its relationship with serum adiponectin level in elderly male metabolic syndrome patients / 上海交通大学学报（医学版）

Objective · To observe the change of serum complement C1q tumor necrosis factor-related protein1 (CTRP1) level and explore its relationship with serum adiponectin (APN) level in elderly male metabolic syndrome (MS) patients. Methods · Clinical data of 279 male objects (60-90 years old) were analyzed retrospectively, serum CTRP1 and APN levels of all objects were tested by enzyme-linked immuno sorbent assay (ELISA). The patients were divided into three groups, i.e. 105 MS patients (MS group), 90 MS patients combined with hypertension (HMS group), and 84 non-MS patients (control group). All general information, including height, body weight, systolic blood pressure (SBP), diastolic blood pressure, fasting blood glucose, fasting insulin and serum three acyl glycerin (TAG) were recorded, in order to calculate body mass index (BMI) and homeostasis model assessment of insulin resistance (HOMA-IR). Results · Compared with the control group, serum CTRP1 levels of patients in MS group and HMS group both increased, and the latter was more obviously. Serum APN levels of patients decreased obviously in HMS group and MS group. The level of serum CTRP1 was negatively related with the level of serum APN. Conversely, serum CTRP1 level was positively related with blood glucose, BMI, SBP, TAG and HOMA-IR. Conclusion · The level of serum CTRP1 is elevated, while the level of serum APN declines in elderly male MS patients. The serum level of CTRP1 is even higher in HMS patients. Serum CTRP1 level is related to many risk factors of atherosclerosis.

Homeostasis of complement system and the severity of coronary artery disease / 中华检验医学杂志

Objective To investigate if the relative ratio between C1q and C3a, C5a had a relationship with the extent of coronary artery disease ( CAD) which had never been evaluated in humans.Methods Fifty-three patients scheduled for elective percutaneous coronary intervention ( PCI ) from February, 2016 to April, 2016 at Fuwai hospital were prospectively enrolled.According to the clinical and angiographic characters patients were divided into two groups:acute coronary syndrome ( ACS) group ( n=24), and control group (n=29, 19 patients with stable angina and 10 patients without CAD confirmed by angiography).In all individuals, fasting venous blood was collected by EDTA tubes after admission and strictly before PCI.The plasma level of C1q was measured by immune turbidimetric analysis, C3a and C5a were measured by ELISA tests.Differences between groups were assessed using t test, Mann-Whitney Utests, chi-squared test or Fisher exact test depending on the type of data respectively.Multivariate logistic regression analyses were conducted to evaluate the adjusted effect of C1q, C3a, C5a, C1q/C3a and C1q/C5a on ACS.Results Compared with control group, ACS group has an elevated circulation level of C3a (4 531.14 μg/L vs.4 179.95 μg/L, t=1.381,P=0.173) and C5a (6.44 μg/L vs.4.42 μg/L, t=0.133, P=0.108) but a decreased level of C1q (176.98 μg/ml vs.200.60 μg/ml, t=-2.022, P=0.048).The relative ratio of C1q/C3a was significantly decreased in ACS patients(4.05 ×10 -2 vs.4.97 × 10 -2 , t=-2.484, P=0.016).According to the multiple logistic regression analysis, lower relative ratio of C1q/C3a level proved to be independently associated with ACS ( OR=0.937, P=0.047, 95% CI:0.879-0.998).Conclusions The decreased relative ratio of C1q/C3a level proved to be independently associated with ACS.C1q/C3a ratio could be used as an important index reflecting the complement system homeostasis status which might have potential clinical value in evaluating the prognosis of patients with CAD.

Correlation between CTRP-3 level and hypertension / 心血管康复医学杂志

Objective:To study expression of complement C1q tumor necrosis factor-related protein 3 (CTRP-3)in patients with hypertension and its significance.Methods:A total of 562 patients with hypertension were enrolled as hypertension group,and 570 normal subjects undergoing physical examination were regarded as normal control group. General data,plasma levels of adiponectin (APN),leptin and CTRP-3 etc. were measured and compared be- tween two groups. Correlation among CTRP-3 level and related factors of hypertension were analyzed. Results:Compared with normal control group,there were significant reductions in plasma levels of APN [(12.1±0.4)μg/ml vs. (7.3±0.5)μg/ml],leptin [(10.1±0.4)ng/ml vs. (6.2±0.8)ng/ml]and CTRP-3 [(429±15)ng/ml vs. (314±13)ng/ml]in hypertension group,P<0.05 all. Spearman correlation analysis indicated that after correcting age and gender,plasma CTRP-3 level was significant inversely correlated with body mass index,systolic blood pres- sure,diastolic blood pressure,waist circumference,waist hip ratio,levels of total cholesterol,low density lipopro- tein cholesterol (LDL-C),triglyceride,fasting blood glucose,insulin,homeostasis model-insulin resistance index (HOMA-IR),glycosylated hemoglobin,high sensitivity C reactive protein (hsCRP)(r=-0.852~-0.011,P<0.05 or <0.01),and significant positively correlated with levels of high density lipoprotein cholesterol (HDL-C), APN and leptin (r=0.654~0.962,P<0.05 all). Multi-factor regression analysis indicated that compared with high tertile CTRP-3 group,the OR=14.17 (95%CI:3.62~28.34),P=0.001 in low tertile CTRP-3 group,sug- gesting that low plasma CTRP-3 level was independent risk factor for hypertension.Conclusion:Complement C1q tumor necrosis factor-related protein 3 level significantly correlated with hypertension,and it's an independent risk factor for hypertension.

Complete Remission from C1q Nephropathy with Disappearance of C1q Deposition after Steroid Therapy / 대한내과학회지

C1q nephropathy is a rare glomerular disease, defined by characteristic mesangial C1q immune deposition seen in immunofluorescence microscopy with no serological evidence of systemic lupus erythematosus. C1q nephropathy can be diagnosed with a subsequent biopsy, as with IgA nephropathy. There are some cases with an initial diagnosis of hematuria and proteinuria with minimal disease changes, focal segmental glomerulonephritis, and mesangial proliferative glomerulonephritis, but lacking C1q nephropathy, in which C1q deposition on immunofluorescence subsequently develops. We report a case that was diagnosed as diffuse mesangial proliferative glomerulonephritis, but a subsequent biopsy showed C1q nephropathy, with C1q deposition in both immunohistochemistry and electron microscopy (EM). We treated the C1q nephropathy with methylprednisolone and confirmed the disappearance of C1q depositions by both immunohistochemistry and EM in a follow-up biopsy.

Angioedema hereditario en Medellín, Colombia: evaluación clínica y de la calidad de vida / Hereditary angioedema in Medellín (Colombia): Clinical evaluation and quality of life appraisal

Introducción. El angioedema hereditario es una inmunodeficiencia primaria de carácter autosómico dominante, debida a un déficit en la proteína inhibidora del factor C1 y caracterizada por episodios recurrentes de edema subcutáneo y de las mucosas. Las impredecibles y frecuentes crisis de angioedema afectan la calidad de vida de los individuos que las padecen. Objetivo. Analizar las características clínicas de una familia con un caso índice de angioedema hereditario y determinar el impacto de la enfermedad en la calidad de vida. Materiales y métodos. En el estudio se incluyeron 26 miembros de la familia, a 25 de los cuales se les midieron los niveles sanguíneos del factor C4 del complemento y del inhibidor de C1 antigénico y funcional. Se utilizaron dos instrumentos, el SF-36 para evaluar la salud del adulto y el KIDSCREEN-27 para la calidad de vida de niños y adolescentes. Resultados. El 83 % de los individuos que reportaron síntomas cumplían con los criterios serológicos del angioedema hereditario de tipo I: valores bajos del factor C4 del complemento y del inhibidor de C1 cuantitativo (antigénico) y cualitativo (funcional). Se encontró que la calidad de vida en cuanto al bienestar psicológico y el desempeño emocional de los pacientes, se veía considerablemente afectada por los síntomas de la enfermedad. Conclusión. Este estudio provee información sobre la primera familia caracterizada con angioedema hereditario de tipo 1 en el Valle de Aburrá, Colombia. Aunque para ello se usó un instrumento genérico, se confirmó, además, el efecto negativo de la enfermedad en la calidad de vida de los individuos que la padecen.

Introduction: Hereditary angioedema is an autosomal dominant primary immunodeficiency caused by a deficiency of the C1 inhibitor protein and characterized by recurrent episodes of subcutaneous and mucosal edema. Unpredictable and frequent crisis of angioedema affect the quality of life of individuals suffering this kind of disorder. Objective: To analyze the clinical characteristics of a family with an index case of hereditary angioedema and to determine the impact of this disease on their quality of life. Materials and methods: Twenty six members of the family were included in the trial; 25 of them were analyzed for C4 complement and antigenic and functional C1 inhibitor blood levels. Two instruments (SF-365 and KIDSCREEN-27) were used to evaluate adult health quality and children and teenagers quality of life, respectively. Results: Eighty three percent (83%) of individuals reporting symptoms of the condition exhibited serological criteria of hereditary angioedema type I: low levels of both C4 complement and quantitative (antigenic) and qualitative (functional) C1 inhibitor. In relation to patients' psychological and emotional performance, their quality of life was significantly affected by the symptoms of hereditary angioedema. Conclusion: This study provides evidence of the first family in Valle de Aburrá (Colombia) characterized as having hereditary angioedema type I. Despite the use of a generic instrument, the negative impact on the quality of life of individuals suffering this disease was also confirmed.

Recent advances in research of cofactor C1q related diseases / 中华检验医学杂志

The complement cascade, as a part of innate immune system, plays a major role in phagocytosis, clearance of apoptotic cells, immune response and inflammation.As an initiator of the classical pathway, C1q not only facilitates apoptotic debris removal but also gets involved in the maintenance of vascular endothelial integrity.As a result, deficiency, excessive consumption or dysfunction of C1q leads to the imbalance of such mechanisms and increases the susceptibility of nephropathy, atherosclerosis and central nervous system diseases.Recenlty, C1q was identified as a new biomarker of aging.C1q could be a useful indicator for early diagnosis, therapy and prognosis.

Icatibant, an inhibitor of bradykinin receptor 2, for hereditary angioedema attacks: prospective experimental single-cohort study / Icatibanto, um inibidor de receptor 2 de bradicinina, para ataques de angioedema hereditário: estudo experimental prospectivo de coorte sem grupo controle

CONTEXT AND OBJECTIVE: Hereditary angioedema (HAE) with C1 inhibitor deficiency manifests as recurrent episodes of edema involving the skin, upper respiratory tract and gastrointestinal tract. It can be lethal due to asphyxia. The aim here was to evaluate the response to therapy for these attacks using icatibant, an inhibitor of the bradykinin receptor, which was recently introduced into Brazil. DESIGN AND SETTING: Prospective experimental single-cohort study on the efficacy and safety of icatibant for HAE patients. METHODS: Patients with a confirmed HAE diagnosis were enrolled according to symptoms and regardless of the time since onset of the attack. Icatibant was administered in accordance with the protocol that has been approved in Brazil. Symptom severity was assessed continuously and adverse events were monitored. RESULTS: 24 attacks in 20 HAE patients were treated (female/male 19:1; 19-55 years; median 29 years of age). The symptoms were: subcutaneous edema (22/24); abdominal pain (15/24) and upper airway obstruction (10/24). The time taken until onset of relief was: 5-10 minutes (5/24; 20.8%); 10-20 (5/24; 20.8%); 20-30 (8/24; 33.4%); 30-60 (5/24; 20.8%); and 2 hours (1/24; 4.3%). The time taken for complete resolution of symptoms ranged from 4.3 to 33.4 hours. Adverse effects were only reported at injection sites. Mild to moderate erythema and/or feelings of burning were reported by 15/24 patients, itching by 3 and no adverse effects in 6. CONCLUSION: HAE type I patients who received icatibant responded promptly; most achieved improved symptom severity within 30 minutes. Local adverse events occurred in 75% of the patients. .

CONTEXTO E OBJETIVO: O angioedema hereditário (AEH) com deficiência de inibidor de C1 manifesta-se por episódios recorrentes de edema envolvendo pele, trato respiratório superior e gastrointestinal. Pode ser letal por asfixia. O objetivo foi avaliar a resposta à terapia dos ataques com icatibanto, inibidor do receptor de bradicinina, recentemente introduzido no Brasil. TIPO DE ESTUDO E LOCAL: Estudo experimental prospectivo de coorte, sem grupo controle, da eficácia e segurança do icatibanto em paciente com AEH. MÉTODOS: Pacientes com diagnóstico confirmado de AEH foram incluídos de acordo com os sintomas, independentemente do tempo de início do ataque. Icatibanto foi administrado segundo protocolo aprovado no Brasil. A gravidade do sintoma foi estabelecida continuamente e os eventos adversos foram monitorados. RESULTADOS: 24 ataques em 20 pacientes com AEH foram tratados (19 F:1 M; 19-55 anos; mediana 29 anos). Os sintomas foram: edema subcutâneo (22/24), dor abdominal (15/24) e obstrução de vias aéreas superiores (10/24). O tempo para o início do alívio foi: 5-10 minutos, 5/24 (20,8%); 10-20, 5/24 (20,8%); 20-30, 8/24 (33,4%); 30-60, 5/24 (20,8%) e 2 horas, 1/24 (4,3%). O tempo para a resolução completa variou de 4,3-33,4 horas. Somente efeitos adversos nos locais das injeções foram relatados. Eritema leve a moderado e/ou sensação de ardor foram relatados por 15/24 pacientes, prurido em 3, e 6 não tiveram efeitos adversos. CONCLUSÃO: Pacientes com AEH tipo I receberam icatibanto com pronta resposta; a maioria teve melhora na gravidade dos sintomas em 30 minutos. Eventos adversos locais ocorreram em 75% dos pacientes. .