OBJECTIVETo study the association between the single nucleotide polymorphisms (SNPs) of the ninth exon Val279Phe of platelet-activating factor acetylhydrolase (PAF-AH) gene and gastrointestinal bleeding in children with Henoch-Schönlein purpura (HSP).

METHODSA total 516 children with HSP were enrolled, among whom 182 had gastrointestinal bleeding and 334 had no gastrointestinal bleeding. PCR was used to investigate the distribution of genotypes and alleles in the SNPs of Val97Phe. The plasma PAF-AH activity was measured, as well as the levels of platelet-activating factor (PAF), granular membrane protein-140 (GMP-140), β-thromboglobulin (β-TG), and platelet factor 4 (PF4).

RESULTSThe Val279Phe genotype and allele frequencies were in Hardy-Weinberg equilibrium, and the homozygous genotype TT and heterozygotes accounted for 0.97% and 6.05% respectively. The gastrointestinal bleeding group had a significantly higher allele frequency than the control group (5.22% vs 3.33%; P<0.01). The HSP patients with GG genotype in the gastrointestinal bleeding group had significantly higher levels of plasma PAF and GMP-140 than those in the non-gastrointestinal bleeding group (P<0.05), while the non-gastrointestinal bleeding group had a significantly higher PAF-AH activity than the gastrointestinal bleeding group (P<0.05). There were no significant differences in β-TG and PF4 between the two groups (P>0.05).

CONCLUSIONSVal279Phe gene polymorphisms in PAF-AH are associated with PAF-AH activity and PAF and GMP-140 levels and may be a risk factor for HSP with gastrointestinal bleeding.

1-Alkyl-2-acetylglycerophosphocholine Esterase ; genetics ; Adolescent ; Child ; Child, Preschool ; Female ; Gastrointestinal Hemorrhage ; etiology ; Genotype ; Humans ; Infant ; Male ; P-Selectin ; blood ; Platelet Activating Factor ; analysis ; Polymorphism, Single Nucleotide ; Purpura, Schoenlein-Henoch ; blood ; complications

OBJECTIVETo investigate the association of serum vitamin D [25-(OH)D] level with the severity and treatment in children with Henoch-Schönlein purpura (HSP).

METHODSA total of 50 children with newly-diagnosed HSP between January and December, 2015 were enrolled as HSP group, and 49 healthy children were enrolled as control group. Fasting serum samples were collected, and ELISA was used to measure serum 25-(OH)Dlevel. According to the serum 25-(OH)Dlevel, the HSP group were further divided into normal group (>20 ng/mL) (n=9), insufficiency group (15-20 ng/mL) (n=15), deficiency group (≤15 ng/mL) (n=25), and severe deficiency group (≤5 ng/mL) (n=1). The general data, clinical manifestations, hormone therapy, course of disease before admission, and length of hospital stay were compared between groups.

RESULTSThe HSP group had a significantly lower serum 25-(OH)Dlevel than the control group (16±6 ng/mL vs 29±5 ng/mL; P<0.01). Compared with the normal and insufficiency groups, the deficiency and severe deficiency groups had significant increases in the incidence rate of renal involvement, rate of hormone application, and median length of hospital stay (P<0.05), while there was no significant difference in course of disease before admission (P>0.05).

CONCLUSIONSChildren with HSP have a low serum 25-(OH)Dlevel, and such children may have a high risk of renal involvement, a high rate of hormone application, and a prolonged length of hospital stay. However, further studies are needed to investigate whether vitamin D supplementation is helpful to the treatment of HSP and can shorten the course of disease in children with HSP.

Child ; Female ; Humans ; Length of Stay ; Male ; Purpura, Schoenlein-Henoch ; blood ; complications ; drug therapy ; Severity of Illness Index ; Vitamin D ; analogs & derivatives ; blood

No abstract available.
Biopsy ; DNA Mutational Analysis ; *Diagnostic Errors ; Enzyme Replacement Therapy ; Fabry Disease/complications/*diagnosis/enzymology/genetics ; Genetic Predisposition to Disease ; Glomerulonephritis, IGA/diagnosis/etiology ; Humans ; Male ; Middle Aged ; Mutation ; Phenotype ; Predictive Value of Tests ; Purpura, Schoenlein-Henoch/*diagnosis ; alpha-Galactosidase/genetics/therapeutic use

Humans ; Nephritis ; etiology ; therapy ; Prognosis ; Purpura, Schoenlein-Henoch ; complications ; therapy ; Sjogren's Syndrome ; etiology ; therapy

No abstract available.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Echocardiography, Doppler, Color ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/complications/diagnosis/drug therapy/*microbiology ; Fluorescent Antibody Technique ; Heart Septal Defects, Ventricular/*complications/diagnosis/surgery ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Valve Stenosis/*complications/diagnosis ; Purpura, Schoenlein-Henoch/diagnosis/drug therapy/*etiology ; Risk Factors

OBJECTIVETo explore the clinical characteristics of Henoch-Schönlein purpura (HSP) in children.

METHODSThe clinical data of 325 hospitalized children who were diagnosed with HSP between June 2012 and June 2014 were analyzed retrospectively.

RESULTSIn the 325 children with HSP, the incidence of HSP was higher in winter and spring, with 33.9% and 27.4%, respectively. Infection was the major factor to induce HSP (57.2%). The incidence of renal damage in children with purpura accompanied by abdominal symptoms and children with purpura accompanied by abdominal and joint symptoms was 60.3% and 48.9%, respectively, with statistically significant differences compared with children with purpura alone (P<0.05). In 32 children with purpura nephritis, the pathological grades of IIIa and IIIb were more common, accounting for 28% and 31%, respectively. In 325 children, an increased serum D-dimer level was observed in 260 children (80.0%), an increased peripheral IgA content in 101 children (46.3%), and a decreased CD4+ cell percentage in 62 children (56.4%).

CONCLUSIONSA high incidence of HSP is often seen in spring and winter. HSP is often induced by upper respiratory tract infection. Renal damage is more likely to occur in children with digestive tract symptoms, with IIIa and IIIb as the common pathological grades of renal damage.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Incidence ; Infant ; Kidney ; pathology ; Male ; Purpura, Schoenlein-Henoch ; complications ; epidemiology ; pathology ; Retrospective Studies ; Seasons

OBJECTIVETo investigate the effect of Helicobacter pylori (Hp) eradication therapy on prognosis in children with Henoch-Schonlein purpura (HSP).

METHODSA total of 153 children with HSP were divided into Hp infection treatment group (n=22), Hp infection control group (n=21), and Hp infection-negative group (n=110). The Hp infection treatment group received one-week triple therapy for Hp eradication in addition to conventional treatment, while the Hp infection control group and Hp infection-negative group received conventional treatment. All patients were followed up for prognostic evaluation.

RESULTSThe response rates of the Hp infection treatment, control, and negative groups were 86% (19/22), 90% (19/21) and 85% (94/110), respectively (P>0.05). The recurrence rates of HSP in the Hp infection treatment, control, and negative groups were 14% (3/22), 24% (5/21) and 31% (34/110), respectively (P>0.05). The incidence of Henoch-Schonlein purpura nephritis (HSPN) in the Hp infection-negative group (36%, 40/110) and control group (33%, 7/21) was significantly higher than that in the Hp infection treatment group (5%, 1/22) (P<0.05 for both), but no significant difference in the incidence of HSPN was found between the control and negative groups (P>0.05).

CONCLUSIONSOne-week triple therapy for Hp eradication may be useful to reduce the incidence of HSPN in children with HSP infected with Hp.

Child ; Child, Preschool ; Female ; Helicobacter Infections ; complications ; drug therapy ; Helicobacter pylori ; Humans ; Incidence ; Male ; Prognosis ; Purpura, Schoenlein-Henoch ; epidemiology ; etiology ; Recurrence

OBJECTIVETo investigate the levels and functions of CD4(+)CD25(+) regulatory T cells and specific transcription factor Foxp3 and Th17 cells related cytokine in peripheral blood mononuclear cells (PBMC) and renal tissues, and explore their roles in pathogenesis of Henoch-Schonlein purpura nephropathy (HSPN) in children.

METHODFrom March, 2011 to March, 2013, 30 cases of HSPN children underwent renal biopsy and were treated in Guiyang Children's Hospital were enrolled into this study. Ten healthy children who underwent health check up were enrolled as blood sample control group. The normal kidney tissue specimens were taken from 5 children who underwent surgery for urologic disorders were used as renal sample control group. The circulating proportions of CD4(+)CD25(+) regulatory T cells in PBMC of 30 cases of HSPN children and 10 cases of control group were determined by flow cytometry, respectively.Reverse transcription-polymerase chain reaction (RT-PCR) were used to analyze the mRNA expressions of IL-17, IL-1β and Foxp3 in PBMC. The expression of IL-17 and IL-1β in renal tissue of HSPN and control group were measured by immunohistochemistry. CD4(+)CD25(+) regulatory T cells, Foxp3, IL-17, IL-1β expression were analyzed and compared in HSPN group and control groups respectively.

RESULTThirty cases of HSPN pathological classification were as follows: type I was found in 0 case; type II in 9 cases; type III in 16 cases; type IV in 5 cases; type V in 0 case. The circulating proportions of CD4(+)CD25(+)/CD4(+)T cells and the CD4(+)CD25(+)Foxp3(+)Treg/CD4(+)T cells level were (5.84 ± 0.78)%, (1.01 ± 0.46) % in HSPN groups were substantially lower than those in control group. All these two differences had statistical significance (t = 27.200, 33.260, P < 0.05). The mRNA levels of IL-17, IL-1β in HSPN groups (0.86 ± 0.01,0.71 ± 0.01) were higher than those in control group (t = 25.000, 31.840, all P < 0.05). Foxp3 mRNA expression in HSPN groups (0.24 ± 0.02) were significantly lower than those in control group (t = 21.690, P < 0.05). Protein expression of IL-17 and IL-1β in renal tissues of HSPN children (13.31 ± 0.54, 11.56 ± 0.28) were significantly stronger than those in the control group (t = 27.6, 14.0, all P < 0.01). The highest level of protein expression of IL-17 and IL-1β in renal biopsy of HSPN was in type IV (IV>III>II, F = 545.800, 262.500, all P < 0.01).

CONCLUSIONThe disorder of quantity and function of CD4(+)CD25(+) regulatory T cells, and increase in levels of IL-17, IL-1β (cytokine related to Th17 cells) may play important roles in pathogenesis of HSPN in children; increased protein expression of IL-17, IL-1β in renal tissue may contribute to the development of renal pathological damage in HSPN children.

Case-Control Studies ; Child ; Child, Preschool ; Female ; Flow Cytometry ; Forkhead Transcription Factors ; genetics ; metabolism ; Humans ; Interleukin-17 ; genetics ; metabolism ; Interleukin-1beta ; genetics ; metabolism ; Kidney ; metabolism ; pathology ; Male ; Nephritis ; etiology ; immunology ; pathology ; Purpura, Schoenlein-Henoch ; complications ; immunology ; pathology ; RNA, Messenger ; genetics ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Severity of Illness Index ; T-Lymphocytes, Regulatory ; immunology ; Th17 Cells ; immunology

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.
Adult ; Aged ; Aged, 80 and over ; Arthralgia/epidemiology/etiology ; C-Reactive Protein/analysis ; Child ; Child, Preschool ; Diarrhea/epidemiology/etiology ; Extremities/pathology ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin A/blood ; Immunosuppressive Agents/therapeutic use ; Infant ; Middle Aged ; Odds Ratio ; Prognosis ; Purpura, Schoenlein-Henoch/complications/*diagnosis/drug therapy/*pathology ; Renal Insufficiency/epidemiology/etiology ; Retrospective Studies ; Young Adult

Nineteen comprehensive hospital information system (HIS) data are collected. Cleaning up the database, then analysis the patients' information. Using the basic description method and association rules to analysis the data. Analysis the data come from HIS database, we found that in 2 110 patients, main five complications are Henoch Schonlein purpura nephritis, upper respiratory tract infections, renal insufficiency, virus infection and glomerulonephritis (chronic), treatment of chemical drugs with sugar cortical hormone and anti infection drugs, such as intravenous input cephalosporin drugs. Traditional Chinese medicine with blood-activating and stasis-resolving medicine, heat clearing and detoxifying medicine. Combination scheme with blood-activating and stasis-resolving medicine joint glucocorticoid, followed by blood-activating and stasis-resolving medicine combined antiallergic drugs, heat-clearing and detoxifying medicine combined antiallergic drugs. Promoting blood circulation and removing blood stasis is an important part of traditional Chinese medicine treatment in patients with Henoch Schonlein purpura.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Anti-Allergic Agents ; therapeutic use ; Child ; Child, Preschool ; Female ; Glucocorticoids ; therapeutic use ; Hospitals ; Humans ; Infant ; Infant, Newborn ; Male ; Medicine, Chinese Traditional ; methods ; Middle Aged ; Purpura, Schoenlein-Henoch ; complications ; diagnosis ; drug therapy ; Young Adult