Characteristics and Risk Factors for Pediatric Sepsis.

OBJECTIVE: Sepsis is considered a major cause of health loss in children and had high mortality and morbidity. Currently, there is no reliable model for predicting the prognosis of pediatric patients with sepsis. This study aimed to analyze the clinical characteristics of sepsis in children and assess the risk factors associated with poor prognosis in pediatric sepsis patients to identify timely interventions and improve their outcomes. METHODS: This study analyzed the clinical indicators and laboratory results of septic patients hospitalized in the Pediatric Intensive Care Unit of Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, China, from January 1, 2019, to December 31, 2021. Risk factors for sepsis were identified by logistic regression analyses. RESULTS: A total of 355 children with sepsis were enrolled, with 333 children (93.8%) in the good prognosis group, and 22 children (6.2%) in the poor prognosis group. Among them, there were 255 patients (71.8%) in the sepsis group, and 100 patients (28.2%) in the severe sepsis group. The length of hospital stay in the poor prognosis group was longer than that in the good prognosis group (P<0.01). The levels of interleukin 1ß (IL-1ß) in the poor prognosis group were higher than those in the good prognosis group (P>0.05), and the platelet (PLT), albumin (ALB), and hemoglobin (Hb) levels were lower in the poor prognosis group (P<0.01). The IL-8 levels in the severe sepsis group were higher than those in the sepsis group (P<0.05). Multiple logistic regression analysis suggested that lower Hb levels, ALB levels, peak PLT counts, and higher IL-1ß levels were independent risk factors for poor prognosis in children with sepsis. CONCLUSION: Lower Hb, ALB, and PLT counts and elevated IL-1ß are independent risk factors for poor prognosis in children with sepsis.

Decreased circulating levels of free triiodothyronine in Sepsis children and correlation analysis.

BACKGROUND: Intensive physical stress in sepsis can induce the disorder of endocrine function and impact the clinical course and prognosis. Low T3 syndrome has been verified to be the predictive indicator of poor prognosis in several researches. Reports on the influence factors of thyroid hormonal levels in children with severe sepsis are rare. We aim to investigate the thyroid hormonal variations in the course of sepsis and analyze that how to be affected by clinical data and inflammatory biomarkers. METHODS: In the case-control study, 184 children with sepsis and 323 controls were included in Tongji Hospital, Wuhan, China, in 2019. Data on clinical and inflammatory parameters were collected from all participants. Circulating FT3(Free Triiodothyronine) levels were measured by Electrochemiluminescence immunoassay. Finally, we investigated the correlation between FT3 and related variables with linear regression analysis. RESULTS: Serum FT3 was lower in the sepsis group than in control group(2.59 + 1.17 vs 2.83 + 1.01 pg/mL, p < 0.05). Significant moderately negative correlations(|r| > 0.3) of FT3 levels with ferritin, PCT, duration of symptoms, SOFA score, and mortality were revealed. Moreover, we observed that FT3 had the positive correlation with albumin, as well as white blood cell count. CONCLUSIONS: Concentrations of serum FT3 are dramatically declined in sepsis children than in control children. Our results demonstrate that recognizing the potential abnormality of thyroid hormones in sepsis patients and examine timely through abnormal common clinical data and inflammatory biomarkers is a fine option.

Rifaximin protects against circadian rhythm disruption-induced cognitive impairment through preventing gut barrier damage and neuroinflammation.

Circadian rhythm disruption (CRD) is a potential risk factor for developing Alzheimer's disease (AD). However, the mechanistic link between CRD and AD is still not fully understood. CRD may lead to intestinal barrier impairment. Several studies in animals and humans suggest a connection between gut microbiota disturbance, intestinal barrier damage and neurodegenerative diseases. In this study, we investigated the effect of CRD on cognition in mice and explored the role of intestinal barrier and inflammatory responses in this process. CRD modulates the composition of gut microbiota, impairs intestinal barrier integrity, and induces both peripheral and central inflammation and cognitive impairment in mice. Rifaximin, a non-absorbable antibiotic which modulates the gut microbial composition and increases intestinal barrier integrity, effectively suppresses inflammatory responses, and rescues cognitive impairment induced by CRD. Furthermore, the impairment in hippocampal neurogenesis, tau hyperphosphorylation, and loss in synaptic proteins in CRD mice is also reversed by Rifaximin. These data identify that the impaired intestinal barrier integrity related to gut microbiota disturbance plays a key role in CRD-induced inflammatory responses and cognitive impairments in mice, and Rifaximin is effective in preventing CRD-induced cognitive deficit through protecting the gut barrier and ameliorating neuroinflammation.

Interferon-γ Stimulates Interleukin-27 Derived from Dendritic Cells to Regulate Th9 Differentiation through STAT1/3 Pathway.

The initiation and progression of allergic asthma (AA) are associated with complex interactions between inflammation and immune response. Herein, we report the specific mechanisms underlying the molecular action of interferon (IFN)-γ in AA regulation. We speculated that IFN-γ inhibits Th9 differentiation by regulating the secretion of interleukin (IL)-27 from dendritic cells (DCs), thereby suppressing airway inflammation in asthma. We constructed a mouse model of ovalbumin-induced AA and overexpressed IFN-γ to evaluate the effect on the IL-27/Th9 axis via the in vitro effect of IFN-γ on IL-27 secretion by DCs and their influence on Th9 differentiation and asthmatic inflammation. IFN-γ overexpression reduced the proportion of Th9 cells and DCs and altered lung morphology and cytokine production in AA-induced mice, thus suppressing the AA phenotype. In addition, exogenous IFN-γ stimulation promoted the secretion of IL-27 and suppressed Th9 differentiation of CD4+ T cells via signal transducer and activator of transcription 1/3 (STAT1/3) signaling in a time-dependent manner. This study aimed to clarify the regulatory effect and mechanism of the IFN-γ/DCs/IL-27/Th9 axis on AA and provide novel insights for effective targeted treatment of asthma.

IL-27 overexpression alleviates inflammatory response in allergic asthma by inhibiting Th9 differentiation and regulating Th1/Th2 balance.

OBJECTIVE: To investigate the effect of IL-27 on Th9 differentiation and Th1/Th2 balance. METHODS: C57BL/6 (B6) mice were treated with ovalbumin to establish an allergic asthma (AA) model and subjected to IL-27 overexpression (OV) and empty vector (EV). Hematoxylin-eosin (HE) staining was performed to observe lung tissue inflammation. Flow cytometry was carried out to evaluate the percentage of Th9, Th1, and Th2 cells. The expression of IL-27, IL-27R, IL-9, T-bet, IFN-γ, and IgE was evaluated by quantitative reverse transcription polymerase chain reaction (qRT-PCR) and enzyme-linked immunosorbent assay (ELISA). Western blot was conducted to observe the expression of pSTAT-1 and pSTAT-3. RESULTS: Compared with the Model group, the number of Th1 cells in the Model + OV group increased significantly (p < .05), while those of Th9 and Th2 cells decreased significantly (p < .05). The expression of IL-27, IL-27R, and IFN-γ in blood serum was increased (p < .05), and that of IL-9 and IgE was significantly decreased in the Model + OV group compared to the Model (p < .05). Western blot revealed that Model + OV exhibited lower expression of pSTAT-3 than that in the Model and Model + EV groups (p < .05), while pSTAT-1 expression was significantly increased (p < .05). Inflammatory infiltration in the Model + OV group was significantly reduced, and there was no significant difference between the Model and Model + EV groups. CONCLUSIONS: IL-27 OV inhibits Th9 differentiation and regulates the imbalance of Th1/Th2, thereby alleviating inflammatory response in AA. The findings suggest that IL-27 OV may be a potential strategy for clinical treatment of AA.

Clinical and Renal Histology Findings and Different Responses to Induction Treatment Affecting the Long-Term Renal Outcomes of Children With ANCA-Associated Vasculitis: a Single-Center Cohort Analysis.

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to induction treatment associated with the long-term renal outcomes in children with AAV in a single center. Methods: All pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD). Results: In total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden's classification. In total, 34 patients (70.8%) showed eGFR <60 ml/min/1.73 m2 at the time of diagnosis. Complete and partial remission was achieved in 8 patients (16.7%) and 19 patients (39.6%), respectively, following 6-month induction treatment. Half of the patients eventually progressed to ESRD at a mean time of (13.04 ± 15.83) months after diagnosis. The independent predictors of nonremission following induction treatment and progression to ESRD were baseline eGFR <60 ml/min/1.73 m2 and hypertension at diagnosis. Renal survival significantly decreased over time in patients with renal sclerotic subtypes or those with nonremission following induction treatment by Kaplan-Meier curve estimation. Conclusions: Our study demonstrates that women, MPA, and crescentic subtypes are predominant in pediatric AAV in China. Initial renal failure (eGFR <60 ml/min/1.73 m2), hypertension, sclerotic pathological subtype, and nonremission following induction treatment are predictive of long-term renal outcomes.

Recommendations for the Diagnosis, Prevention, and Control of Coronavirus Disease-19 in Children-The Chinese Perspectives.

Ever since SARS-CoV-2 began infecting people by the end of 2019, of whom some developed severe pneumonia (about 5%), which could be fatal (case fatality ~3.5%), the extent and speed of the COVID-19 outbreak has been phenomenal. Within 2.5 months (by March 18, 2020) over 191,127 COVID-19 patients have been identified in 161 countries. By then, over 700 pediatric patients were confirmed to have COVID-19 in China, with only about 58 diagnosed elsewhere. By now, there are thousands of children and adolescents infected. Chinese pediatricians would like to share their experience on how these patients were managed in China and the key recommendations that had guided them in meeting the evolving challenges. A group of experts were summoned by the Chinese Pediatric Society and Editorial Board of Chinese Journal of Pediatrics to extract informative data from a survey on confirmed COVID-19 pediatric patients in China. Consensus on diagnosis, management, and prevention of pediatric COVID-19 were drawn up based on the analysis of such data plus insights gained from the past SARS and MERS coronavirus outbreaks. Relevant cumulating experiences from physicians managing adult patients, expedited reports on clinical and scientific COVID-19 and SARS-CoV-2 data, and the National Health Committee guidelines on COVID-19 management were integrated into this proposal.

[Etiology and risk factors for childhood unintentional injuries: a retrospective analysis of data at a pediatric intensive care unit].

OBJECTIVE: To investigate the etiology and risk factors for unintentional injuries in children admitted to the pediatric intensive care unit (PICU), and to provide a basis for preventing these injuries and decreasing the mortality rate. METHODS: A retrospective analysis was performed on the clinical data of children with unintentional injuries admitted to the PICU from December 2012 to December 2017. RESULTS: A total of 102 children with unintentional injuries were admitted to the PICU, which accounted for 3.30% (102/3 087) of the overall PICU patients. The top three causes of unintentional injuries were food or drug poisoning, drowning, and foreign body ingestion and aspiration. The proportion of unintentional injuries in boys was significantly higher than in girls (P<0.05). The younger children had a significantly higher proportion of unintentional injuries (P<0.05). The cause of unintentional injuries was also related to age, and the common causes of unintentional injuries varied between different age groups. The proportion of unintentional injuries was not significantly different between children from urban and rural areas (P>0.05). The logistic regression analysis showed that the number of organs with dysfunction after unintentional injuries, especially respiratory, cardiac, neurological, renal and hematological involvement, was closely associated with the mortality rate of children with unintentional injuries (P<0.05); however, it is not an independent risk factor (P>0.05). CONCLUSIONS: Prevention is the key to decreasing the incidence of childhood unintentional injuries. Preventive measures should be taken based on patient's sex and age and the cause of unintentional injuries. The spread of first aid knowledge, improvement in emergency transportation, and more attention to organ protection may be useful for decreasing the mortality rate of children with unintentional injuries.

Identification of hub genes and potential molecular mechanisms in gastric cancer by integrated bioinformatics analysis.

OBJECTIVE: Gastric cancer (GC) is the fourth most common cause of cancer-related deaths in the world. In the current study, we aim to identify the hub genes and uncover the molecular mechanisms of GC. METHODS: The expression profiles of the genes and the miRNAs were extracted from the Gene Expression Omnibus database. The identification of the differentially expressed genes (DEGs), including miRNAs, was performed by the GEO2R. Database for Annotation, Visualization and Integrated Discovery was used to perform GO and KEGG pathway enrichment analysis. The protein-protein interaction (PPI) network and miRNA-gene network were constructed using Cytoscape software. The hub genes were identified by the Molecular Complex Detection (MCODE) plugin, the CytoHubba plugin and miRNA-gene network. Then, the identified genes were verified by Kaplan-Meier plotter database and quantitative real-time PCR (qRT-PCR) in GC tissue samples. RESULTS: A total of three mRNA expression profiles (GSE13911, GSE79973 and GSE19826) were downloaded from the Gene Expression Omnibus (GEO) database, including 69, 20 and 27cases separately. A total of 120 overlapped upregulated genes and 246 downregulated genes were identified. The majority of the DEGs were enriched in extracellular matrix organization, collagen catabolic process, collagen fibril organization and cell adhesion. In addition, three KEGG pathways were significantly enriched, including ECM-receptor interaction, protein digestion and absorption, and the focal adhesion pathways. In the PPI network, five significant modules were detected, while the genes in the modules were mainly involved in the ECM-receptor interaction and focal adhesion pathways. By combining the results of MCODE, CytoHubba and miRNA-gene network, a total of six hub genes including COL1A2, COL1A1, COL4A1, COL5A2, THBS2 and ITGA5 were chosen. The Kaplan-Meier plotter database confirmed that higher expression levels of these genes were related to lower overall survival, except for COL5A2. Experimental validation showed that the rest of the five genes had the same expression trend as predicted. CONCLUSION: In conclusion, COL1A2, COL1A1, COL4A1, THBS2 and ITGA5 may be potential biomarkers and therapeutic targets for GC. Moreover, ECM-receptor interaction and focal adhesion pathways play significant roles in the progression of GC.

Twenty-three-year review of disease patterns from renal biopsies: an experience from a pediatric renal center.

BACKGROUND: The aim of this study was to investigate the clinical and pathological characteristics as well as their associations with trends for diseases in 1,579 pediatric renal biopsies from 1989 to 2012. METHODS: The clinical and pathological data were retrospectively analyzed for children undergoing renal biopsy from 1989 to 2012 in our hospital. RESULTS: Primary glomerulonephritis (PGN) accounted for 60.1% of total cases, followed by secondary glomerulonephritis (SGN) (31.2%) and hereditary nephropathy (8.3%). The major clinical patterns of PGN and SGN were nephritic syndrome (NS) and Henoch-Schönlein purpura nephritis (HSPN), respectively. Minimal change disease/mild disease (MCD/ML), IgAN and mesangial proliferative glomerulonephritis (MsPGN) were the most common pathological patterns of PGN. Male patients were most likely to suffer from NS, HBV-associated glomerulonephritis (HBVGN) or Alport syndrome, while females were most likely to suffer from isolated hematuria, rapidly progressive glomerulonephritis (RPGN), lupus nephritis (LN), ANCA-associated glomerulonephritis or thin basement membrane disease. The proportions of NS, isolated hematuria, acute nephritic syndrome, chronic nephritic syndrome, HBVGN, LN and hemolytic uremic syndrome changed significantly with aging. The clinical patterns of PGN were significantly correlated with the distribution of pathological types: MCD/ML and IgMN presented most often as NS; MCD/ML and IgAN presented most often as isolated hematuria; IgAN and MsPGN presented most often as hematuria with proteinuria. The spectrum of NS, HSPN, HBVGN and IgAN changed during the 23 years, and the percentage of repeated renal biopsies was low (1.2%) in pediatric cases with kidney disease. CONCLUSIONS: Glomerular diseases in children are closely related to age and sex of patient. The spectrum of kidney diseases from our center has changed significantly over the last 23 years.

Is surgical treatment of cancer of the gastric cardia or esophagus associated with a concurrent major pulmonary operation feasible? One center's experience.

BACKGROUND: Pulmonary complications are a major cause of mortality after operation for cancer of the gastric cardia or esophagus. Although the risk involved in gastric cardiectomy or esophagectomy associated with a concurrent major pulmonary operation is expected to be much higher, it has seldom been evaluated on the basis of clinical experience. The aim of this study was to investigate the possibility and feasibility of the gastric cardiectomy or esophagectomy associated with a major pulmonary operation. METHODS: From August 2003 to January 2011, 14 patients underwent concurrent gastric cardiectomy or esophagectomy and a major pulmonary operation in our hospital. This included eight for pulmonary invasion of esophageal carcinoma, and six for synchronous lung tumor. All patients underwent systematic lymph node dissection for cardiac or esophageal cancer. To prevent postoperative complications, the operative approach and dissection procedures for cardiac or esophageal carcinoma were modified according to the associated pulmonary operation and the extent of cancer invasion. All thoracotomies for cardiectomy or esophagectomy were performed on the same side as the major pulmonary operation. RESULTS: All patients underwent a curative operation. There were no deaths or postoperative complications in the six synchronous lung tumor patients. In the eight pulmonary invasion patients, one patient died of respiratory failure 11 days after operation, and postoperative complications developed in four of them, but none was fatal. Six patients were still alive. CONCLUSIONS: Curative gastric cardiectomy or esophagectomy associated with concurrent major pulmonary operation is not contraindicated in patients in good condition. In selected patients, when the operative procedures for cardiectomy or esophagectomy are appropriately modified to minimize the effect of the associated pulmonary operation, the treatment is associated with a low operative morbidity and mortality with an acceptable long-term survival.

[Clinical features and surgical treatment of thoracic Castleman's disease].

OBJECTIVE: To explore the clinical features and surgical treatment of thoracic Castleman's disease. METHODS: The clinical symptoms, pathological, laboratory, CT findings and results of surgery in 32 patients with Castleman's disease from June 1996 to November 2008 were evaluated. Among the 32 patients, there were 14 male and 18 female, aged from 16 to 48 years old with a mean age of 34.2 years old. Thirteen cases had symptoms including short of breath, irritable cough, or chest pain, while 14 cases had no symptoms. Mediastinal or hilar tumors were found by CT examination. RESULTS: Tumor was surgically removed in all the 32 patients except one died with anesthetic accident. Castleman's disease was conformed by pathology. Five cases were diagnosed as with paraneoplastic pemphigus, 3 of them were attacked by bronchiolitis obliterans. All 5 cases were failed by the use of prednisone. The signs of PNP were dissolved after operation, but pulmonary lesions failed to improve. There was no recurrence in all cases. CONCLUSIONS: PNP and lung abnormalities are the rare and severe complications of thoracic Castleman's disease. Surgical resection of the tumor is the first choice for treatment.

Surgical treatment of IIIb-T4 lung cancer invading left atrium and great vessels.

BACKGROUND: IIIb-T(4) non-small cell lung cancer (NSCLC) is commonly considered a contraindication to surgery, although chemo-radiotherapy also achieves a poor survival rate. We reviewed our experience with T(4) NSCLC patients who underwent surgery to explore the indications and prognostic factors of surgical treatment of lung cancer invading the left atrium and great vessels. METHODS: We investigated a cohort of 105 patients, 79 men and 26 women, who underwent surgery from May 1996 to July 2008. Their pathological staging was T(4)N(0)-(2)M(0). The median age was 59 years, ranging from 36 to 75 years. Patients were grouped based on invading sites: tumors invading the left atrium (LA group), tumors invading the superior vena cava (SVC group), and tumors invading the intrapericardial pulmonary artery (PA group). Patients were further characterized based upon the type of operation, complete resection and incomplete resection groups, and on the lymph node pathological status, N(0), N(1) and N(2) groups. We calculated the overall five-year survival rate. RESULTS: All patients received resection of primary lesions, with partial resection of the left atrium in the LA group (n = 25), angioplasty of superior vena cava in the SVC group (n = 23) and intrapericardial ligation of the pulmonary artery in the PA group (n = 57). Complete resection was possible in 77 patients (73.3%). The overall survival rate of the 105 patients was 41.0% at 5 years; 36.0% for the LA group, 34.8% for the SVC group and 45.6% for the PA group. Pathological N status significantly influenced the overall 5-year survival rate; 61.5% for N(0), 51.1% for the N(1) and 11.8% for the N(2) groups (N(2) group versus N(0) group, P < 0.0001, N(2) versus N(1) group, P < 0.0001). Surgical resection also influenced survival; 49.4% for the complete resection group and 17.9% for the incomplete resection group (P < 0.0001). Cox regression analysis demonstrated that pathological N status was a significant independent predictor of prognosis. CONCLUSIONS: Pathological N status is a significant independent predictor for survival of patients with IIIb-T(4) lung cancer invading the left atrium and great vessels. The completeness of resection has a significant influence on the overall 5-year survival rate. Surgery for T(4) lung cancer may be effective in patients without mediastinal lymph node involvement.

[Diagnosis and treatment of pulmonary sequestration].

OBJECTIVE: To explore the clinical manifestations and treatment of pulmonary sequestration. METHODS: Thirty-five patients with pulmonary sequestration confirmed by pathological studies were analyzed. RESULTS: Among these patients, 29 were of intralobar type and 6 of extralobar type. All patients of intralobar type had symptoms of cough, expectoration, fever and hemoptysis while 6 cases of extralobar type were asymptomatic. Twenty-three cases were diagnosed preoperatively by enhanced CT scan, CT angiography or selective arteriography. Success of operation had been achieved in all cases. CONCLUSION: The clinical manifestations of pulmonary sequestration are non-specific. Though invasive, selective arteriography plays a decisive role in the diagnosis. Such non-invasive angiographies as enhanced CT scan and CT angiography have become important supplements to it. Once the diagnosis is confirmed, operation should be performed.

[Comparison of VATS and mini-incision thoracotomy for the treatment of benign esophageal diseases].

OBJECTIVE: To compare video-assisted thoracis surgery (VATS) and mini-incision thoracotomy for the treatment of benign esophageal diseases. METHODS: The clinical data of 52 patients with benign esophageal diseases underwent minimally invasive surgical treatment from January 1995 to January 2007 were analyzed retrospectively. VATS were performed in 25 cases and mini-incision thoracotomy were performed in 27 cases. RESULTS: There was no operative death or major perioperative complications in both groups. There were no significant difference between them according to operation time, postoperative thoracic drainage time, postoperative gastric drainage time and postoperative hospital stay time. CONCLUSIONS: For the treatment of benign esophageal diseases, both VATS and mini-incision thoracotomy are safe and effective. VATS can be performed in suitable cases with more minimal incision and decreased operative trauma, mini-incision thoracotomy can be performed in almost all cases, preferring the sophisticated cases.

[Comparison of two surgical approach for cardiac cancer].

OBJECTIVE: To explore the preferable surgical approach for cardiac cancer. METHODS: One hundred and sixty patients with cardiac cancer underwent operation in two surgical approaches (epigastrium and left chest). Analysis was conducted on lymph nodes resected, stump positive rate, radical resectability rate, perioperative mortality, surgical complication rate, postoperative hospital days, survival rate between the two groups. RESULTS: In the epigastrium group and the left chest group the average number of resected lymph nodes was 15.7 and 10.6, respectively, the upper stump positive rate was 5.0% and 1.2%, and there was significant difference between the two groups (P < 0.05). The lower stump positive rate, perioperative mortality, radical resectability rate, surgical complication rate, postoperative hospital days, survival rate of the two groups were 1.2%, 0.0%, 81.2%, 10.0%, 10 d, 53.7% and 1.2%, 1.2%, 83.7%, 11.2%, 12 d, 56.2%, respectively, and there was no significant difference between the two groups (P > 0.05). CONCLUSION: There was no difference on the radical respectability rate and 5-year survival rate between the two groups. Thus the surgical approaches for cardiac cancer should based on the location and extent of lesions and the state of patients.

Childhood Henoch-Schönlein purpura nephritis and IgA nephropathy: one disease entity?--A clinico-pathologically comparative study.

In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schönlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and follow-up study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8% children with IgA nephropathy, but only 10% in HSPN (P < 0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia, compared with only abdominal pain in 3.2% children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5% of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9% of IgA nephropathy and 6.3% of HSPN, but endothelial proliferation in 65.6% of HSPN and 29% of IgA nephropathy (all P < 0.01). Thin basement membrane nephropathy was only found in 6.5% children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2% of HSPN, and overwhelming IgG deposits in 12.5% of HSPN with relatively weak IgA deposits, moreover 6.3% of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9% of HSPN had IgG deposits in glomeruli and only 19.4% of IgA nephropathy showed glomerular IgG deposits (P < 0.01). No IgG deposit was observed in 81.6% of IgA nephropathy, among them most showed IgA and IgM and/or C3 deposits, moreover overwhelming IgG deposits and linear IgG deposits couldn't be found in IgA nephropathy. Mean 20 months follow-up showed complete remission in 72.5% of HSPN, but only 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5% of IgA nephropathy had consistent hematuria and proteinuria and 16.1% had active nephritides (P < 0.05). It was concluded that significant clinico-pathological difference was found between HSPN and IgA nephropathy, which didn't support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.

[Clinical and pathological characterization of antineutrophil cytoplasmic antibody associated small vasculitis in children].

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA) associated small vasculitides (ASV) are rare in children and often complicated in clinical manifestations and have very poor prognosis. In order to deepen our understanding of ANCA-associated small vasculitis (ASV) in children, the present study aimed to characterize their clinical manifestations, serum ANCA and renal histopathological findings and outcomes in Chinese children. METHODS: Serum ANCA was qualitatively tested with indirect immunofluorescence microscopy and anti-proteinase 3 (PR(3)) and anti-myeloperoxidase (MPO) activity were quantitated by enzyme-linked immunosorbent assays (ELISA), and renal biopsies were done to investigate the pathological changes. The clinical manifestation, serum ANCA and renal histopathological findings and outcome were characterized in 5 children with ANCA associated small vasculitis. RESULTS: (1) Five children with ANCA associated small vasculitis only accounted for 1.20% of children in whom renal biopsy was performed and 0.25% of hospitalized children with renal diseases during the same period. The age of onset of the 5 children with ASV was between 8 to 12 years with mean age 10.5 years. All ASV children were female. (2) All ASV children were negative for C-ANCA and showed normal anti-proteinase 3 activities, but positive for P-ANCA with high anti-myeloperoxidase activities between 98 to 242 kEU/L. The mean value of MPO-ANCA was 154.5 kEU/L (normal range < 12.7 kEU/L). (3) All ASV in the children was microscopic polyarteritis with wide-spread glomerular crescents formation and capillary tuft fibrinoid necrosis. Variety of complement C3 deposits and weak immunoglobulin deposits were noted in all ASV but one child who showed relatively strong deposits of IgA and IgM. The electronic dense deposits were mainly located in subendothelial space but were also found in the glomerular basement membrane in one child. (4) Three children with ASV died within one year after diagnosis, and two got remission and restored renal function after combined pulse therapy with methylprednisolone and cyclophosphamide (CTX), but remained to have hematuria and small amount of proteinuria after 1 and 5 year follow-up, respectively. CONCLUSION: Childhood ASV was female and P-ANCA predominant, more vulnerable to progress to renal failure and poorer in prognosis than adult cases. Qualitative and quantitative ANCA measurement and renal biopsy were key to the diagnosis of ASV in children.

[Clinical study on treatment of childhood Henoch-Schonlein purpura nephritis with colquhounia root tablet].

OBJECTIVE: To study the effect of Colquhounia root tablet (CRT) in treating childhood Henoch-Schonlein purpura nephritis (HSPN) and compared with tripterygium wilfordii multiglycoside tablet (TWMGT). METHODS: Eighty-two children with HSPN were divided into the CRT group and the control group. To patients of nephritic type, treatment with CRT and TWMGT was applied separately, but to those of nephrotic type, the treatment was given together with prednisone. Therapeutic effect and changes of related indexes, including urinary protein, retinal-binding protein (RBP), N-acetyl-beta-glucosaminidase (NAG), were observed after patients received treatment for 6 months. RESULTS: The complete remission (CR) rate and partial remission (PR) rate in patients of nephritic type in the treated group was 58.8% and 41.2% respectively, while those in the control group was 20.8% and 66.7% respectively, and the ineffective rate being 12.5%. Comparison of the therapeutic effect between the two groups showed significant difference (P < 0.05). Although the CR rate in patients of nephrotic type in the treated group was higher than that in the control group, the difference was insignificant (P > 0.05). The urinary levels of protein, RBP and NAG lowered in both groups, but level of urinary protein in the treated group of nephritic type after 6 months of treatment was significantly lower than that in the control group (P < 0.05). CONCLUSION: CRT shows obvious effect in improving childhood HSPN of nephritic type, nephrotic type and attenuating the tubulointerstitial lesions, compared with TWMGT, CRT is more effective in lowering proteinuria and better in total effective rate.