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OBJECTIVES: To study the correlation of anti-C1q antibodies with active systemic lupus erythematosus (SLE) and lupus nephritis (LN) in children, as well as their diagnostic value for active SLE and LN. METHODS: A retrospective selection of 90 hospitalized children with SLE at the Children's Medical Center of Second Xiangya Hospital, Central South University from January 2016 to March 2019 as the SLE group, all of whom were tested for anti-C1q antibodies. A control group was formed by collecting 70 hospitalized children with other autoimmune diseases (OAD) during the same period. The differences in anti-C1q antibody levels were compared between two groups.The correlation of anti-C1q antibodies with various indicators of SLE and LN was analyzed, and the diagnostic value of anti-C1q in SLE and LN was evaluated. RESULTS: The serum levels of anti-C1q antibodies in the SLE group were higher than those in the OAD group (P<0.05). The SLE disease activity index score was positively correlated with anti-C1q antibodies (rs=0.371, P<0.001) and positively correlated with anti-double-stranded DNA antibodies (rs=0.370, P<0.001). The sensitivity and specificity of anti-C1q antibodies for diagnosing active SLE were 89.90% and 53.90%, respectively, with an area under the curve of 0.720 (P<0.05) and a critical value of 5.45 U/mL. The sensitivity and specificity of anti-C1q antibody levels for diagnosing active LN were 58.50% and 85.00%, respectively, with an area under the curve of 0.675 (P<0.05) and a critical value of 22.05 U/mL. CONCLUSIONS: Anti-C1q antibodies can serve as non-invasive biomarkers for evaluating the activity of SLE or predicting the activity of LN in children.
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Complemento C1q , Lupus Eritematoso Sistémico , Nefritis Lúpica , Humanos , Complemento C1q/inmunología , Nefritis Lúpica/inmunología , Nefritis Lúpica/sangre , Femenino , Niño , Masculino , Lupus Eritematoso Sistémico/inmunología , Estudios Retrospectivos , Adolescente , Autoanticuerpos/sangre , Preescolar , Anticuerpos Antinucleares/sangre , Anticuerpos Antinucleares/inmunologíaRESUMEN
OBJECTIVES: To study the clinical characteristics of children with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: A retrospective analysis was conducted on the clinical data of 25 children diagnosed with AAV at the Second Xiangya Hospital of Central South University from January 2010 to June 2022. RESULTS: Among the AAV children, there were 5 males and 20 females, with a median age of onset of 11.0 years. Involvement of the urinary system was seen in 18 cases (72%); respiratory system involvement in 10 cases (40%); skin involvement in 6 cases (24%); eye, ear, and nose involvement in 5 cases (20%); joint involvement in 4 cases (16%); digestive system involvement in 2 cases (8%). Eleven cases underwent kidney biopsy, with 5 cases (46%) showing focal type, 2 cases (18%) showing crescentic type, 2 cases (18%) showing mixed type, and 2 cases (18%) showing sclerotic type. Immune complex deposits were present in 5 cases (45%). Seven cases reached chronic kidney disease (CKD) stage V, with 2 cases resulting in death. Two cases underwent kidney transplantation. At the end of the follow-up period, 2 cases were at CKD stage II, and 1 case was at CKD stage III. Of the 16 cases of microscopic polyangiitis (MPA) group, 13 (81%) involved the urinary system. Of the 9 cases of granulomatosis with polyangiitis (GPA), 6 cases (66%) had sinusitis. Serum creatinine and uric acid levels were higher in the MPA group than in the GPA group (P<0.05), while red blood cell count and glomerular filtration rate were lower in the MPA group (P<0.05). CONCLUSIONS: AAV is more common in school-age female children, with MPA being the most common clinical subtype. The onset of AAV in children is mainly characterized by renal involvement, followed by respiratory system involvement. The renal pathology often presents as focal type with possible immune complex deposits. Children with MPA often have renal involvement, while those with GPA commonly have sinusitis. The prognosis of children with AAV is poor, often accompanied by renal insufficiency.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Humanos , Femenino , Masculino , Niño , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Estudios Retrospectivos , Adolescente , Preescolar , Insuficiencia Renal Crónica/etiologíaRESUMEN
OBJECTIVE: To examine the transfection of Homeobox A13 (HOXA13) on epithelial-mesenchymal transition (EMT) and the expression of bone morphogenetic protein-7 (BMP-7) induced by albumin-overload in human kidney tubular epithelial cells (HKCs). METHODS: The cultured HKCs were treated with 20 mg/mL human serum albumin (HSA) for 48 hours. Protein expression of cytokeratin (CK), vimentin and HOXA13 in the HKCs was assessed by Western blot. Protein expression of CK, vimentin, and BMP-7 was also detected in HKCs transfected with lipofectamine contained HOXA13 DNA. RESULTS: HSA induced EMT in HKCs, presented by decreased CK expression (P<0.01) and increased vimentin expression (P<0.01). The up-regulated expression of HOXA13 transfected by lipofectamine inhibited the level of EMT induced by HSA in HKCs (P<0.05). The decreased rate of BMP-7 protein expression induced by HSA was inhibited by over-expressed HOXA13 in HKCs (P<0.05). CONCLUSIONS: Transfection of HOXA13 in HKCs could inhibit the degree of EMT induced by albumin-overload, possibly by increasing BMP-7 expression.
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Proteína Morfogenética Ósea 7/genética , Transición Epitelial-Mesenquimal , Proteínas de Homeodominio/fisiología , Túbulos Renales/metabolismo , Células Cultivadas , Células Epiteliales/metabolismo , Humanos , Queratinas/genética , Transfección , Vimentina/genéticaRESUMEN
OBJECTIVE: To investigate the urinary neutrophil gelatinase-associated lipocalin (NGAL) concentration in children with idiopathic nephrotic syndrome (INS) and its clinical significance. METHODS: Thirty-four children newly diagnosed with INS received oral prednisone for 4 weeks. Patients whose urinary protein did not become negative were classified as steroid-resistant nephrotic syndrome (SRNS) group, while those whose urinary protein did become negative were classified as steroid-sensitive nephrotic syndrome (SSNS) group. Morning midstream urine specimens were collected from all patients before use of prednisone and after 1, 2, 3, and 4 weeks of treatment with prednisone. Enzyme-linked immunosorbent assay was used to measure the urinary NGAL concentration. Meanwhile, urinary creatinine (Cr) concentration was measured, and urinary NGAL concentration in a single urine collection was adjusted according to the urinary Cr excretion. The two groups were compared in terms of urinary NGAL/Cr ratio. RESULTS: Compared with the SRNS group, the SSNS group had significantly decreased urinary NGAL/Cr ratios after 3 and 4 weeks of prednisone treatment (P < 0.05). Compared with the SRNS group, the SSNS group had a significantly decreased urinary ß2-MG/Cr ratio after 4 weeks of prednisone treatment (P < 0.05). In both groups, urinary NGAL/Cr ratio was positively correlated with urinary protein/Cr ratio (r = 0.510, P < 0.01). The results of ROC curve analysis showed when diagnostic cut-off point of urinary NGAL/Cr was 0.043 by 3 weeks after treatment, sensitivity and specificity achieved 100% and 79.2% respectively. CONCLUSIONS: Urinary NGAL/Cr ratio remains high in children with SRNS, while this ratio decreases gradually during prednisone treatment in children with SSNS, and it falls ahead of urinary ß2-MG/Cr ratio. These results suggest that dynamic monitoring of urinary NGAL/Cr ratio is useful for early judgment of response to prednisone in patients with INS.
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Proteínas de Fase Aguda/orina , Lipocalinas/orina , Síndrome Nefrótico/orina , Proteínas Proto-Oncogénicas/orina , Niño , Preescolar , Creatinina/orina , Femenino , Humanos , Lipocalina 2 , Masculino , Síndrome Nefrótico/tratamiento farmacológico , Prednisona/uso terapéutico , Microglobulina beta-2/orinaRESUMEN
BACKGROUND: Pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a life-threatening systemic vasculitis featured by liability to renal involvement. However, there are few studies on the risk factors and predictive models for renal outcomes of AAV in children. METHODS: Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively. The risk factors and predictive model of end-stage renal disease (ESRD) in AAV were explored. RESULTS: Renal involvement was the most typical manifestation (95.5%), and the crescent was the predominant pathological lesion (84.9%). The estimated glomerular filtration rate (eGFR) was evaluated in 114 patients, of whom 59.6% developed ESRD, and the median time to ESRD was 3.20 months. The eGFR [P = 0.006, odds ratio (OR) = 0.955, 95% confidence interval (CI) = 0.924-0.987] and the percentages of global glomerulosclerosis (pGGS; P = 0.018, OR = 1.060, 95% CI = 1.010-1.112) were independent risk factors for ESRD of renal biopsy. Based on the pGGS and eGFR at renal biopsy, we developed three risk grades of ESRD and one predictive model. The KaplanâMeier curve indicated that renal outcomes were significantly different in different risk grades (P < 0.001). Compared with serum creatinine at baseline, the predictive model had higher accuracy (0.86 versus 0.58, P < 0.001) and a lower coefficient of variation (0.07 versus 0.92) in external validation. CONCLUSIONS: Renal involvement is the most common manifestation of pediatric AAV in China, of which more than half deteriorates into ESRD. The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children. Supplementary file 2 (MP4 18937 KB).
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OBJECTIVE: To study the effects of huai qi huang, a traditional Chinese medicine, on cytokines Th1, Th2 and Th17 levels and alveolar macrophage phagocytosis in asthmatic rats sensitized by ovalbumin (OVA). METHODS: Forty male Sprague-Dawley rats were randomly divided into five groups: normal control, untreated asthma, budesonide-treated, huai qi huang-treated and budesonide+huai qi huang-treated asthma (n=8 each). Asthma was induced by OVA sensitization and challenge. The levels of IL-4, IFN-γ and IL-17 in plasma and bronchoalveolar lavage fluid (BALF) were measured using ELISA. The phagocytosis of alveolar macrophages which were isolated and purified from BALF was evaluated by the colorimetric assay. RESULTS: The levels of IL-4 and IL-17 increased, in contrast, the IFN-γ level decreased in plasma and BALF in the untreated asthma group compared with those in the normal control group. The IFN-γ level in the huai qi huang-treated asthma group was higher than that in the untreated asthma group. The IFN-γ level increased and the IL-17 level decreased more significantly in the budesonide+huai qi huang-treated asthma group when compared with the budesonide and huai qi huang alone treatment groups. The phagocytosis of alveolar macrophages in the untreated asthma group was lower than that in the normal control group. Huai qi huang alone or combined with budesonide increased the phagocytosis of alveolar macrophages compared with the normal control, untreated asthma and budesonid-treated asthma groups. The levels of IFN-γ in plasma and BALF were positively correlated with the phagocytosis of alveolar macrophages. CONCLUSIONS: The levels of IL-4 and IL-17 increase and the IFN-γ level decreases in plasma and BALF, and the phagocytosis of alveolar macrophages decreases in asthmatic rats. Huai qi huang treatment may increase the IFN-γ expression in plasma and BALF and the phagocytosis of alveolar macrophages in asthmatic rats. There is a synergistic effect between huai qi huang and glucocorticoids.
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Asma/tratamiento farmacológico , Citocinas/biosíntesis , Macrófagos Alveolares/efectos de los fármacos , Medicina Tradicional China , Fagocitosis/efectos de los fármacos , Linfocitos T Colaboradores-Inductores/inmunología , Animales , Asma/inmunología , Macrófagos Alveolares/inmunología , Masculino , Ratas , Ratas Sprague-Dawley , Células TH1/inmunología , Células Th17/inmunología , Células Th2/inmunologíaRESUMEN
PURPOSE: The current study is aimed at investigating whether urinary CD80 is reliable to predict the recurrence of pediatric PNS. MATERIALS AND METHODS: A total of 128 children, 105 males and 23 females, were enrolled in this study. Urinary samples were collected from SSNS and SRNS patients and 25 healthy children as controls. Urinary CD80 was measured by ELISA and adjusted for urinary creatinine excretion. RESULTS: Urinary CD80 in relapse stage of SSNS was significantly higher, and the urinary CD80 of paired relapse and remission stages of each SSNS patient were also significantly different. No significant difference was found between the urinary CD80 in SRNS relapse group, SRNS remission group, and the control group. Similarly, there was no significant difference between frequent SSNS and not frequent SSNS in remission group, as well as the relapse group. There is no correlation between urinary CD80 and 24-hour urinary protein. CONCLUSION: The increase of urinary CD80 was closely associated with the relapse of SSNS but was not related to the frequency of relapse. The urinary CD80 changes of concentration were reliable to predict the recurrence of SSNS. However, it cannot be used to predicate the frequent recurrence of PNS.