Value of Oxford classification and ISKDC classification in the prognosis of children with Henoch-Schönlein purpura nephritis / 中华肾脏病杂志

Objective@#To analyze the Oxford classification (MESTC) and the International Study of Kidney Disease in Children (ISKDC) classification for evaluating the clinical manifestations, histological lesion and short-term prognosis of children with Henoch-Schönlein purpura nephritis (HSPN).@*Methods@#According to the Oxford classification and ISKDC classification, the histological lesions of children with HSPN diagnosed by renal biopsy from Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to December 2018 were re-evaluated. The renal biopsy specimens of the selected subjects were scored according to the Oxford classification and the ISKDC classification. According to whether the first symptom was combined with renal performance, MESTC score and ISKDC classification, children were grouped. The differences in clinicopathological manifestations between the groups were compared. Correlation between MESTC and ISKDC grades was analyzed by nonparametric test rank correlation. Kaplan-Meier survival curve and Log-rank test were used to compare the difference of proteinuria remission rate between the two groups. Univariate and multivariate Cox regression equations were used to analyze the influencing factors of the proteinuria remission rate.@*Results@#A total of 78 children with HSPN were enrolled. There were 37 male patients (47.4%) with age of (10.4±2.9) years. When the patients were divided according to MESTC scores and ISKDC classification, the results showed that the proportion of children with nephrotic-range proteinuria in the group of endocapillary hypercellularity (E1, P=0.008), segmental glomerulosclerosis (S1, P=0.015) and ISKDCⅢ(P=0.041) was higher than that of E0, S0 and ISKDCⅡ groups. The proportion of children with E1 (P=0.015), crescents (C1&C2, P=0.025) or ISKDCⅢ(P=0.017) that had been treated with high-dose methylprednisolone was higher. The result of Kaplan-Meier survival curve showed more difficult for proteinuria remission in children with C2 are than C0&C1 group (P=0.026), while no difference were found when children were grouped by M, E, S, T and ISKDC. Multivariate Cox regression analysis showed that the C2 (HR=0.143, 95%CI 0.020-1.046, P=0.055) might be a risk factor for proteinuria remission, while the P value was close to 0.05.@*Conclusions@#Children with HSPN scored as ISKDCⅢ, E1 and S1 are more likely to show nephrotic-range proteinuria. C2 may indicate that patients are more difficult to achieve proteinuria remission.

Changes of coagulation and fibrinolysis system in children with Henoch-Schonlein purpura nephritis and its clinical significance / 中国基层医药

Objective@#To investigate the changes of coagulation and fibrinolysis system in children with Henoch-Schonlein purpura nephritis and its clinical significance.@*Methods@#From January 2013 to December 2016, 73 children with Henoch-Schonlein purpura inthe First People's Hospital of Huzhouwere selected as the Henoch-Schonlein purpura group, 73 children with Henoch-Schonlein purpura nephritis were selected as the Henoch-Schonlein purpura nephritis group, and 73 healthy children were selected as the control group.Antithrombin III activity (AT III), fibrinogen degradation products (FDP), plasma D-dimer (D-D), prothrombin activator inhibitor-1 (PAI-1), tissue plasminogen activator (t-PA), fibrinogen (FIB) levels and 24-hour urinary protein excretion were measured in three groups.@*Results@#The levels of FIB, AT III%, FDP, D-D, PAI-1 and t-PA in the Henoch-Schonlein purpura group and the Henoch-Schonlein purpura nephritis group[(2.89±0.76)g/L, (3.51±0.81)g/L; (145.72±8.46)%, (163.24±9.05)%; (1.31±0.67)mg/L, (1.54±0.72)mg/L; (0.87±0.52)mg/L, (1.18±0.67)mg/L; (66.47±2.58)ng/L, (91.02±3.24)ng/L; (16.34±0.98)μg/L, (12.35±1.06)μg/L]were higher than those in the control group[(1.88±0.54)g/L, (119.48±8.92)%, (0.92±0.33)mg/L, (0.32±0.18)mg/L, (31.25±3.02)ng/L, (6.82±0.75)μg/L](t=9.256, 18.236, 4.462, 8.540, 75.760, 65.912; 14.306, 29.423, 6.688, 10.591, 115.297, 36.387, all P<0.05). The levels of FIB, AT III%, FDP, D-D and PAI-1 in theHenoch-Schonlein purpura nephritis group were higher than those in the Henoch-Schonlein purpura group (t=4.769, 12.083, 1.998, 3.123, 50.644, all P<0.05), and t-PA in the Henoch-Schonlein purpura nephritis group was lower than that in Henoch-Schonlein purpura group (t=23.165, P<0.05). The 24-hour urinary protein excretion in theHenoch-Schonlein purpura nephritis group[(1.48±0.89)g/24h]was higher than that in the Henoch-Schonlein purpura group[(0.11±0.02)g/24h] and control group[(0.10±0.05)g/24h](t=13.149, 13.227, all P<0.05). There was no statistically significant difference between Henoch-Schonlein purpura group and control group (t=1.587, P>0.05). The 24-hour urinary protein excretion was positively correlated with AT III%, FDP, D-D and IL-33 in patients with Henoch-Schonlein purpura nephritis (r=0.502, 0.546, 0.483, all P<0.05), but not correlated with FIB, PAI-1 and t-PA (r=0.189, 0.213, -0.175, all P>0.05).@*Conclusion@#Patients with purpuric nephritis are in a state of hypercoagulability and hyperfibrinolysis, and coagulation and fibrinolysis disorders are closely related to renal damage in patients.

The clinical diagnosis significance of EGF and PAF-AH on henoch-schonlein purpura renal damage / 重庆医学

Objective To explore the correlation and clinical diagnostic significance between EGF ,PAF‐AH on henoch‐schonlein purpura (HSP) renal damage in children ,in order to provide a new way of thinking for early diagnosis and clinical prog ‐nosis for HSP nephritis(HSPN) .Methods Totally 86 cases of children with HSP from September 2013 to January 2014 in hospital were collected .According to the presence of renal damage ,the patients were divided into non‐HSPN group and HSPN group ,50 ca‐ses of healthy children as control group ,and 30 casea were diagnosied as bronchial pneumonia .Using PCR technique to detect the change of blood EGF and PAF‐AH ,and using ELISA to detect the change of blood EGF ,PAF‐AH and urine EGF .Results Detec‐tion of blood PAF‐AH mRNA ,EGF mRNA in case group obviously higher than control group ,and detection of blood PAF‐AH mRNA in HSPN group were also hgher than non‐HSPN group(P 0 .05) .Detection of serum PAF‐AH ,EGF and urine EGF in case group obviously higher than control group and the case group significantly lower recovery than acute stage (P<0 .05) .Conclusion Blood EGF ,PAF‐AH and urine EGF process involved in the pathogenesis of HSP ,and is related to renal dam‐age .

Differences in Clinical Manifestations and Outcomes between Adult and Child Patients with Henoch-Schonlein Purpura

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schonlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.

Analysis of the incidence of hospitalized children with Henoch-Sch(o)nlein purpura and Henoch-Sch(o)nlein purpura nephritis / 中华风湿病学杂志

Objective To analyze the incidence of hospitalized children with Henoch-Sch(o)nle in purpura (HSP) and Henoch-Sch(o)nlein purpura nephritis (HSPN) from 2009 to 2012,and to characterize the epidemiology of HSP and HSPN in Jiangxi province.Methods Inpatients of Jiangxi Children's Hospital with the diagnosis of HSP or HSPN were recruited during 2009 to 2012.The basic messages in the home page of medical records,such as the admission year,sex,age,area coming from were collected.Chi-square test was used for statistical analysis.Results A total of 2516 HSP pediatric patients were included in this study.Of whom,412 cases were diagnosed in 2009,568 cases in 2010,750 cases in 2011,786 cases in 2012.Among them,renal damage as the presenting symptom in 110 cases in 2009,148 cases in 2010,198 cases in 2011,and 196 cases in 2012.The average incidence of HSPN was 25.91％ (652/2516).The morbidity of HSPN were similar in different admission years (x2=0.62,P＞0.05).In HSP patients,the ratio between male and female was 1.59∶1; the peak age was 4 to 9 year-old (65.50％,1648/2516).The morbidity of HSPN was similar in both girls and boy patients group (x2=0.14,P＞0.05).The morbidity of HSPN was 18.76％ (163/869) in patients younger than 6 years old,which was lower than 26.85％(359/1337) in patients between age 6 to 11 years old,and the morbidity of HSPN was 41.94％ (130/310) in patients older than 11years old,the difference was significant (x2=65.24,P＜0.01).The morbidity of HSP in the month of year was different,the peak time was between October and December,which as 40.74％(1025/2516).The morbidity of HSPN was 30.23％(208/688) during January to March,higher than in other period of the year (x2=9.87,P＜0.05).In the last four years,there were 824(32.75％) hospitalized patients of HSP in Nanchang district,1692 cases (67.25％) in other areas in Jiangxi province.The morbidity of HSPN in Nanchang district was 17.35％ (143/824),compared with the average level 25.91％(652/2516),the difference was significant (x2=25.08,P＜0.01).Conclusion The number of children diagnosed with HSP is progressively increased from 2009 to 2012 in Jiangxi province.The peak period for HSP is between October and December,and the peak age is 4 to 9 year-old.The incidence of HSPN between January to March group is higher than in other periodof the year.The morbidity of HSPN is increased with age.

Observation on the effect of the integrative medicine in the treatment of gastrointestinal manifestations of Henoch-Schonlein purpura in children / 中国基层医药

Objective To investigate the clinical effect and safety of integrative medicine in treatment of gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) in children.Methods 90 children with gastrointestinal manifestations of HSP were randomly divided into two groups,45 cases in each group.Control group was given hydrocortisone and prednisone,the treatment group was given self decoction of Chinese medicine on the basis of the control group.Clinical efficacy was observed after two courses.Results Total effective rate of the treatment group was 95.56％,which was higher than that of the control group(77.78％) (P ＜0.05).The disappearance time of HSP of the treatment group [(12.32 ± 3.59) d] was significantly shorter than that of the control group [(18.56 ± 4.18) d](P ＜ 0.05),and the incidence rate of adverse reactions has a significant reduction (4.44％ vs 11.11％,P ＜ 0.05).Conclusion Adjuvant treatment with Chinese medicine on the basis of western medicine in treatment of gastrointestinal manifestations of HSP in children,can significantly improve the clinical efficacy.

Compromiso Duodenal en púrpura de Henoch-Schõnlein / Duodenal involvement in Henoch- Schõnlein purple

La Purpura de Henoch-Schõnlein (PHS) es una vasculitis sistémica de pequeños vasos que afecta piel, articulaciones, tracto gastrointestinal y riñones en diferente grado y variada intensidad. Se presenta con mayor frecuencia en niños por lo que es considerada como la causa mas común de púrpura no trombocitopénica en esa edad, aunque también puede aparecer en edad adulta. Los síntomas así como el compromiso gastrointestinal puede ser variados, aunque se considera que puede ocurrir hasta en el 85% de pacientes. Se describe el caso de un paciente de 12 años de edad con hemorragia digestiva debido a compromiso duodenal asociado a esta afección y evidenciado por endoscopia.

Henoch-Schõnlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin, joints, gastrointestinal tract and kidneys. It is the most common cause of non thrombocytopenic purpura in children, but may also occur in adults. Gastrointestinal symptoms and disease occurs in up to 85% of patients and can be of varying intensity. We report a 12-year-old boy with gastrointestinal bleeding due to duodenal involment associated with this condition evidenced by endoscopy.

Progress in IgA and the pathogenesis of Henoch-Schonlein purpura / 国际儿科学杂志

IgA plays an important role in the course of Henoch-Schonlein purpura.The current study shows that the HSP is characterized by IgA principally IgA1) depositions in the wall of dermal vessel and the renal mesangium, and IgA1 is deficient in galactose. IgA1 with aberrant glycosylation has a tendency to be self-aggregated, not to be efficiently cleared by the hepatic asialoglycoprotein receptor. It also weakens the ability of complement to remove immune complexes, and causes the exposure of new epitope and so on. All these lead to the formation of IgA1 immune complexes and the deposition in the wall of dermal vessel and the renal mesangium. IgA1 immune complexes activate complement principally through the alternative pathway, which causes inflammatory injury. In addition, various IgA autoantibodies, such as anti-endothelial cell antibodies,anticardiolipin antibodies and anti-neutrophil cytoplasmic antibodies, are also involved in the process of tissue damage of Henoch-Schonlein purpura.

Edema agudo hemorrágico de la infancia en una niña mayor; una entidad diferente de la púrpura de Henoch-Shönlein / Infantile acute hemorrhagic edema in a female child: a different entity from Henoch-Shönlein purpura

Introducción. El edema agudo hemorrágico de la infancia (EAHI) es una entidad nosológica que se manifiesta en niños pequeños, es autolimitada y de curso benigno. Usualmente su manifestación es secundaria y existe el antecedente de infección de vías aéreas superiores, administración de antibióticos o aplicación de vacunas. Las dos manifestaciones cutáneas principales son lesiones purpúricas en roseta o cocarda y anulares o en tiro al blanco, que se encuentran de manera primaria en cara y extremidades superiores, además hay edema en cara y extremidades. Caso clínico. Se informa el caso de un preescolar femenino de 4 años y 11 meses de edad con manifestaciones clínicas clásicas de esta entidad, pero que se presentó en edad tardía. Conclusión. Se refuerza el concepto de que la púrpura de Henoch-Schönlein y el EAHI son entidades diferentes.

Introduction. Infantile acute hemorrhagic edema (IAHE) is an entity manifested in young children and has a self-limiting and benign course. It usually appears secondary to a history of upper respiratory illness, course of antibiotics or vaccination. The two primary cutaneous features include large "cockade" or rosette appearance or annular purpuric lesions found primarily on the face and upper extremities along with edema of the limbs and face. Case report. We report the case of a female patient (age 4 years and 11 months) who manifested all the classic clinical characteristics of this entity at an older age. Conclusion. The concept that Henoch-Schönlein purpura and acute hemorrhagic edema of infancy are different entities is reinforced.

Endoscopic Characteristics and Clinical Analysis of Henoch-Schonlein Purpura in Children / 实用儿科临床杂志

Objective To investigate the diagnostic significance of endoscopic findings in Henoch-Schonlein purpura(HSP),especially when abdominal pain preceded the cutaneous lesions.Methods The clinical data and gastroscopic findings in 37 cases of children with HSP were studied and analysed retrospectively in order to detect the pathological changes in the stomach and duodenum mucosa.The biopsy was taken in the pathological changeing place,and the relationship between clinical and endoscopic findings was analyzed.Results Detection rate of the pathological changes in the stomach and duodenum mucosa was 62.2%,31.3% of which experienced only cutaneous lesions,100% of which presented the acute abdominal pain.Three patients were not checked up the pathological changes.Of them,1 had arthritis,2 had Henoch-Schonlein nephritis.Characteristically endoscopic findings in the stomach and duodenum mucosa were found.The endoscopic findings included anabrosis,hyperemia,edema and hemorrhage.Conclusions Detection rate of the pathological changes in the stomach and duodenum mucosa is higher.Endoscopy is very helpful to the early diagnosis of HSP in children,especially abdominal pain presented firstly.

Clinical Features and Treatment of Human Parvovirus B_(19) Infection Associated Henoch-Schonlein Purpura / 实用儿科临床杂志

0.05),but the latter was superior to the former in extinction of exanthem.4.B_(19)-DNA clearance of hormone group was 25.0%,that of gamma globulin group was 81.82%,and there was significant difference between 2 groups(P

Expression and significance of platelet membrane glycoproteins and antiplatelet antibody in patients with anaphylactoid purpura / 中华皮肤科杂志

Objective To investigate the expression and significance of platelet membrane glyco- proteins and antiplatelet antibody in patients with anaphylactoid purpura.Methods Forty-five patients with anaphylactoid purpura were divided into three groups according to their clinical manifestations,15 in dermal purpura group,18 in mixed group and 12 in nephritis group.The expression of CD62P,CD63 and CD41 in these 45 patients was detected by flow cytometry.The level of antiplatetlet antibody was analysed by ELISA with monoclonal antibody as the probe.Results The percentage of CD62P,CD63 and the level of an- tiplatelet antibody in patients in acute phase were significantly higher than those in regressive phase and those in normal control.Neither of the expression of CD62P,CD63 and CD41,nor the level of antiplatetlet antibody in nephritis group was significantly different from that in the mixed group.Compared with dermal purpura group,the percentage of CD62P was significantly higher in the nephritis group and mixed group. There was no significant difference in CD41 expression among all groups.Conclusion Abnormal humoral immunity exists in anaphylactoid purpura.The activation of platelet could be closely related to the progress of anaphylactoid purpura.CD62P and CD63 could be considered as an index for monitoring of symptoms, determination of prognosis and predicting the outcome in anaphylactoid purpura.

Significance of Changes of Serum Levels and Ratios of Matrix Nephritis Metalloproteinase-2,-9 and Tissue Inhibitor of Metalloproteinase-1 in Children with Henoch-Schonlein Purpura Nephristis / 实用儿科临床杂志

Objective To explore the effect of levels and ratios of matrix nephritis metalloproteinase-2(MMP-2), -9 and inhibitor of metalloproeinase-1(TIMP-1) in the pathogenesis of children with Henoch-Schonlein purpura nephritis(HSPN),and the correlation between them and urinary micro albumin(MA).Methods Serum levels of MMP-2, -9 and TIMP-1 were determined by double antibody enzyme linked immunosorbent assay(ELISA),and urine MA was determined by immune rate nephelometry in 36 children with HSPN,16 children with simple purpura and 30 healthy controls.Results Levels of MMP-2, -9,TIMP-1 and ratios of MMP-2/ TIMP-1,MMP-9/TIMP-1 rose in acute phase of HSPN. The levels and ratios in HSPN group were higher than those in simple purpura group,and those in simple purpura group higher than those in controls (P

Protective Effect of Heparin on Kidneys of Children with Henoch-Schonlein Purpura / 实用儿科临床杂志

Objective To explore protective effect of heparin on renal of children with Henoch-Schonlein purpura(HSP).Methods Two hundred and fifteen cases with HSP were divided into heparin-treated group(110 cases,male 64,female 46) and control group (105 cases,male 61,female 44).Serum IL-6 and IL-8 levels were examined by using radioimmunoassay respectively.These patients were followed up for more than 6 months.Results 1.The level of IL-6,IL-8 did not show significant difference between heparin-treated group and control group before treatment.The serum concentrations of IL-6,IL-8 in heparin-treated group were obviously lower than those in control group,after treatment,there was significantly different[IL-6(116.50?19.45)vs(123.88?22.55)ng /L,t=2.573 P=0.011;IL-8(0.161?0.043)vs(0.173?0.048)?g/L,t=2.024 P=0.044].2.During more than 6 months follow-up there were 26 cases suffering from purpura nephritis in heparin-treated group, the morbidity was 25%; the morbidity in control group was 39.8%,the morbidity in treatment group was significant lower than that in control group(?2=5.061 P=0.024).The time of renal injury was significantly delay in treatment group(35.0?13.2)d compared with that of control group (26.0?12.1)d(t=2.659 P=0.010).Conclusions The serum IL-6 and IL-8 concentrations in HSP obviously decrease after heparin treatment.Heparin therapy can decrease the morbidity of renal injury in children with HSP and delay the time of renal injury effectively.