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1.
Rev. colomb. reumatol ; 30(1)mar. 2023.
Article in English | LILACS | ID: biblio-1536234

ABSTRACT

Schoenlein-Henoch purpura is a systemic small vessel vasculitis mediated by IgA-1 deposition in organs such as the skin, kidney, and gastrointestinal tract; it has been mainly described in children where it has a favourable prognosis. Although much rarer in adulthood it is associated with an increased risk of severe kidney involvement, gastrointestinal com-plications, and prolonged hospital stay. The therapeutic options are wide and vary according to the degree of involvement of the patient and the organ mainly affected.


La púrpura de Schönlein-Henoch es una vasculitis sistêmica de pequeno vaso mediada por depósito de IgA en órganos como la piel, el riñón y el tracto gastrointestinal. Se ha descrito principalmente en niños, grupo de población en el que tiene un pronóstico favorable. Si bien en la edad adulta es mucho menos frecuente, se asocia con un mayor riesgo de compromiso renal severo, complicaciones gastrointestinales y estancia hospitalaria prolongada. Las opciones terapêuticas son amplias y varían según el grado de compromiso del paciente y el órgano más afectado.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , IgA Vasculitis , Vascular Diseases , Vasculitis , Immunoglobulin A , Cardiovascular Diseases , Proteins , Amino Acids, Peptides, and Proteins
2.
Chinese Journal of Contemporary Pediatrics ; (12): 1287-1292, 2023.
Article in Chinese | WPRIM | ID: wpr-1009883

ABSTRACT

Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura, has complex etiology and pathogenesis which have not been fully clarified. The latest research shows that SARS-CoV-2 and related vaccines, human papilloma vaccine, and certain biological agents can also induce IgAV. Most studies believe that the formation of galactose-deficient IgA1 (Gd-IgA1) and Gd-IgA1-containing immune complex plays a crucial role in the pathogenesis of IgAV. It is hypothesized that the pathogenesis of IgAV is associated with the binding of IgA1 to anti-endothelial cell antibodies. In addition, genetics also constitutes a major focus of IgAV research. This article reviews the new advances in the etiology of IgAV and summarizes the role of Gd-IgA1, Gd-IgA1-containing immune complex, anti-endothelial antibody, IgA1 conjugates, T lymphocyte immunity, and genetic factors in the pathogenesis of IgAV.


Subject(s)
Humans , IgA Vasculitis , Antigen-Antibody Complex , Immunoglobulin A/genetics
3.
Chinese Journal of Contemporary Pediatrics ; (12): 1137-1142, 2023.
Article in Chinese | WPRIM | ID: wpr-1009860

ABSTRACT

OBJECTIVES@#To study the effect of vaccination on the short-term risk of immunoglobulin A vasculitis (IgAV) in children.@*METHODS@#A retrospective analysis was conducted on the general data and the vaccination history within one year prior to onset in children with IgAV hospitalized in the Children's Hospital Affiliated to Zhengzhou University from November 2021 to January 2023. Vaccine exposure rates in the risk period (3 months prior to IgAV onset) and the control period were compared by autocontrol-case crossover analysis, and the odds ratio and 95% confidence interval (95%CI) were calculated. A sensitivity analysis for the one-month and two-month risk periods was conducted.@*RESULTS@#A total of 193 children with IgAV were included, with a median age of 7.0 years. Among the 193 children, 36 (18.7%) received at least one dose of the vaccine within 1 year prior to IgAV onset, and 14 (7.3%) received at least one dose of the vaccine during the 3-month risk period. Compared to the unvaccinated IgAV group, the vaccinated IgAV group had a significantly younger age of onset (P<0.05). There were no significant differences in the proportions of children with gastrointestinal involvement, renal involvement, and joint involvement between the two groups (P>0.05). The odds ratio for developing IgAV after receiving any type of vaccine within 3 months prior to IgAV onset was 2.08 (95%CI: 0.82-5.27, P>0.05). Further sensitivity analysis for the 1-month and 2-month risk periods demonstrated that the odds ratios for developing IgAV after receiving any type of vaccine were 2.74 (95%CI: 0.72-10.48, P>0.05) and 2.72 (95%CI: 0.95-7.77, P>0.05), respectively.@*CONCLUSIONS@#Vaccination dose not increase the risk of IgAV, nor does it exacerbate clinical symptoms in children with IgAV.


Subject(s)
Humans , Child , Retrospective Studies , Immunoglobulin A , IgA Vasculitis , Vaccination , Vaccines
4.
Chinese Journal of Contemporary Pediatrics ; (12): 1113-1117, 2023.
Article in Chinese | WPRIM | ID: wpr-1009856

ABSTRACT

OBJECTIVES@#To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups.@*METHODS@#A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions.@*RESULTS@#There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05).@*CONCLUSIONS@#The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.


Subject(s)
Child , Humans , Mycophenolic Acid/adverse effects , IgA Vasculitis/drug therapy , Retrospective Studies , Cyclophosphamide/adverse effects , Immunosuppressive Agents/adverse effects , Vasculitis/drug therapy , Nephritis/complications
5.
Chinese Journal of Contemporary Pediatrics ; (12): 837-842, 2023.
Article in Chinese | WPRIM | ID: wpr-1009829

ABSTRACT

OBJECTIVES@#To investigate the clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Schönlein purpura nephritis (DEP-HSPN).@*METHODS@#A retrospective analysis was performed on the clinical, pathological, and prognosis data of 44 children with DEP-HSPN and 765 children without DEP-HSPN. The children with DEP-HSPN were diagnosed by renal biopsy in Jiangxi Provincial Children's Hospital from January 2006 to December 2021.@*RESULTS@#Among the 809 children with purpura nephritis, 44 (5.4%) had DEP-HSPN, with a mean age of (8±3) years, and there were 29 boys (65.9%) and 15 girls (34.1%). Compared with the non-DEP-HSPN group, the DEP-HSPN group had a significantly shorter time from onset to renal biopsy and a significantly higher proportion of children with respiratory infection or gross hematuria, and most children had nephrotic syndrome. The DEP-HSPN group had significantly higher levels of 24-hour urinary protein, urinary protein grading, microscopic hematuria grading, serum creatinine, and blood urea nitrogen and significantly lower levels of serum albumin and complement C3 (P<0.05). The DEP-HSPN group had a higher pathological grading, with predominant deposition of IgA in the mesangial area and capillary loops, and higher activity scores in the modified semi-quantitative scoring system (P<0.05). The Kaplan-Meier survival analysis showed that there was no significant difference in the renal complete remission rate between the two groups (P>0.05).@*CONCLUSIONS@#Children with DEP-HSPN have a rapid onset, severe clinical manifestations and pathological grading, and high activity scores in the modified semi-quantitative scoring system. However, most of the children with DEP-HSPN have a good prognosis, with a comparable renal complete remission rate to the children without DEP-HSPN.


Subject(s)
Male , Female , Humans , Child , Child, Preschool , Hematuria , IgA Vasculitis , Retrospective Studies , Prognosis , Nephritis
6.
China Journal of Chinese Materia Medica ; (24): 5915-5931, 2023.
Article in Chinese | WPRIM | ID: wpr-1008790

ABSTRACT

This study used UPLC-TQ-MS technology to replicate a Henoch-Schonlein purpura(HSP) model in rats by administering warm drugs by gavage and injecting ovalbumin with Freund's complete adjuvant emulsion. The distribution differences and characteristics of eight major components(ferulic acid, caffeic acid, neochlorogenic acid, cryptochlorogenic acid, benzoyl oxypaeoniflorin, tracheloside, loganin, and paeoniflorin) in rat liver, lung, heart, spleen, and kidney tissues were determined after oral administration of the Liangxue Tuizi Mixture at a dose of 42 g·kg~(-1) in both normal physiological and HSP states at 0.5, 1, 2, 6, and 12 hours. The results showed that the distribution patterns of the eight components of Liangxue Tuizi Mixture in the tissues of normal and HSP model rats were different. The main component, paeoniflorin, in Moutan Cortex and Paeoniae Radix Alba had higher content in all tissues. The eight components were predominantly distributed in the liver, lung, and kidney tissues, followed by spleen and heart tissues.


Subject(s)
Rats , Animals , IgA Vasculitis/drug therapy , Monoterpenes , Administration, Oral , Liquid Chromatography-Mass Spectrometry
7.
China Journal of Chinese Materia Medica ; (24): 3327-3344, 2023.
Article in Chinese | WPRIM | ID: wpr-981470

ABSTRACT

Ultra-performance liquid chromatography-quadrupole time of fight/mass spectrometry(UPLC-Q-TOF-MS) and UNIFI were employed to rapidly determine the content of the components in Liangxue Tuizi Mixture. The targets of the active components and Henoch-Schönlein purpura(HSP) were obtained from SwissTargetPrediction, Online Mendelian Inheritance in Man(OMIM), and GeneCards. A "component-target-disease" network and a protein-protein interaction(PPI) network were constructed. Gene Ontology(GO) functional annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment analysis were performed for the targets by Omishare. The interactions between the potential active components and the core targets were verified by molecular docking. Furthermore, rats were randomly assigned into a normal group, a model group, and low-, medium-, and high-dose Liangxue Tuizi Mixture groups. Non-targeted metabolomics was employed to screen the differential metabolites in the serum, analyze possible metabolic pathways, and construct the "component-target-differential metabolite" network. A total of 45 components of Liangxue Tuizi Mixture were identified, and 145 potential targets for the treatment of HSP were predicted. The main signaling pathways enriched included resistance to epidermal growth factor receptor tyrosine kinase inhibitors, phosphatidylinositol 3-kinase/protein kinase B(PI3K-AKT), and T cell receptor. The results of molecular docking showed that the active components in Liangxue Tuizi Mixture had strong binding ability with the key target proteins. A total of 13 differential metabolites in the serum were screened out, which shared 27 common targets with active components. The progression of HSP was related to metabolic abnormalities of glycerophospholipid and sphingolipid. The results indicate that the components in Liangxue Tuizi Mixture mainly treats HSP by regulating inflammation and immunity, providing a scientific basis for rational drug use in clinical practice.


Subject(s)
Animals , Rats , IgA Vasculitis/drug therapy , Network Pharmacology , Molecular Docking Simulation , Phosphatidylinositol 3-Kinases , Metabolomics
8.
Journal of Experimental Hematology ; (6): 837-842, 2023.
Article in Chinese | WPRIM | ID: wpr-982138

ABSTRACT

OBJECTIVE@#To investigate the predictive value of complete blood count (CBC) and inflammation marker on the recurrence risk in children with Henoch-Schönlein purpura (HSP).@*METHODS@#One hundred and thirty-three children with HSP admitted to Cangzhou Central Hospital from February 2017 to March 2019 were enrolled. The clinical data of the children were collected, at the time of admission CBC and C-reactive protein (CRP) were detected. After discharge, the children were followed up for 1 year, the clinical data of children with and without recurrence were compared, and multivariate logistic regression was used to analyze the risk factors affecting HSP recurrence. Receiver operating characteristic (ROC) curve should be drawn and the predictive value of CBC and CRP on HSP recurrence should be analyzed.@*RESULTS@#In the follow-up of 133 children, 8 cases were lost and 39 cases recurred, with a recurrence rate of 31.20% (39/125). The age, skin rash duration, proportion of renal damage at the initial onset, percentage of neutrophils, percentage of lymphocytes, platelet count (PLT), mean platelet volume (MPV) and neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), MPV/PLT ratio (MPR), and CRP level of patients with recurrence were statistically different from those without recurrence (P <0.05). Multivariate logistic regression analysis showed that long skin rash duration, renal damage at the initial onset, increased PLR, high PLT, increased MPV and elevated CRP level were independent risk factors for recurrence in children with HSP (P <0.05). The ROC curve analysis showed that the area under the curve (AUC) of the combination of the four blood and inflammation marker (PLT, MPV, PLR and CPR) in the early prediction of HSP recurrence was 0.898, which was higher than the initial renal damage (AUC=0.687) and persistent skin rash time (AUC=0.708), with a sensitivity of 84.62% and a specificity of 83.72%.@*CONCLUSION@#Observation of CBC and CPR can predict the risk of HSP recurrence early and guide early clinical intervention.


Subject(s)
Humans , Child , IgA Vasculitis , Blood Cell Count , Inflammation , C-Reactive Protein , Lymphocytes , Neutrophils , Exanthema , Retrospective Studies
9.
Journal of Peking University(Health Sciences) ; (6): 923-928, 2023.
Article in Chinese | WPRIM | ID: wpr-1010150

ABSTRACT

OBJECTIVE@#To investigate the risk factors of different types of Henoch-Schönlein purpura (HSP) in Tibetan patients at high altitude, as to provide reference for correctly identifying high-risk patients.@*METHODS@#A retrospective study was used to analyze the 304 HSP patients admitted to Tibet Autonomous Region People's Hospital from April 2014 to March 2022. The gender, age, allergic history, family history, clinical type, laboratory indexes (hemoglobin, platelet count, eosinophil, C-reactive protein (CRP), albumin, immunoglobulin G, immunoglobulin A, complement C3 and C4) were analyzed retrospectively. Univariate and multivariate Logistic regression analysis to screen for risk factors affecting different types of HSP.@*RESULTS@#Renal HSP patients showed higher IgA [(9.2±1.7) g/L vs. (6.4±2.4) g/L, P=0.015], lower complement C3 [(203.3±21.6) mg/dL vs. (301.1±19.5) mg/dL, P=0.043], and complement C4 [(33.5±2.3) mg/dL vs. (53.0±7.2) mg/dL, P=0.032]. The patients with abdominal HSP showed lower levels of hemoglobin [(119.6±19.6) g/L vs. (146.6±47.3) g/L, P=0.038] and plasma albumin [24.8 (22.1, 33.9) g/L vs. 32.6 (24.6, 35.1) g/L, P=0.045]. The patients with articular HSP exhibited higher CRP [13.5 (0.2, 20.6) g/L vs. 7.5 (0.1, 15.2) g/L, P=0.036] and erythrocyte sedimentation rate (ESR) [24 (5, 40) mm/h vs. 15 (4, 30) mm/h, P=0.049]. Elevated IgA and decreased complement C4 were risk factors for renal HSP, anemia and decreased plasma albumin were risk factors for abdominal HSP, and elevated CRP was a risk factor for articular HSP.@*CONCLUSION@#The clinical characteristics of different types of HSP in plateau areas were different. Patients with high IgA, low complement C4, anemia, hypoalbuminemia, and significantly elevated CRP should be highly vigilant. Early and effective intervention can improve the clinical efficacy, avoid severe development, and improve the prognosis.


Subject(s)
Humans , Retrospective Studies , Tibet/epidemiology , Complement C3/analysis , IgA Vasculitis/complications , Altitude , Complement C4 , C-Reactive Protein/analysis , Immunoglobulin A , Risk Factors , Anemia , Hemoglobins/analysis , Serum Albumin/analysis
10.
Rev. argent. reumatolg. (En línea) ; 33(2): 90-96, abr. - jun. 2022. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1393400

ABSTRACT

La vasculitis por inmunoglobulina A, anteriormente llamada púrpura Schönlein Henoch (VIgA/PSH), es la vasculitis sistémica más frecuente en la infancia. El desencadenante más común es una infección previa del tracto respiratorio superior. Se caracteriza por púrpura palpable no trombocitopénica con artralgias y/o artritis, afectación gastrointestinal y compromiso renal. SARS-CoV-2 es un virus ARN que causa la enfermedad COVID-19. Afecta frecuentemente el sistema respiratorio con presentaciones que varían desde una rinitis hasta condiciones severas como síndrome de distress respiratorio, shock séptico o síndrome de inflamación multisistémica (multi-system inflammation syndrome, MIS). Se describe el caso de un niño de 5 años de edad con clínica de VIgA/PSH como forma inicial de presentación y diagnóstico posterior de infección por SARS-CoV-2, derivado al hospital de mayor complejidad, con encefalopatía hipertensiva que presentó evolución favorable y restitución completa del cuadro clínico


Immunoglobulin A vasculitis, previously called Henoch Schonlein purpura (IgAV/ HSP), is the most common systemic vasculitis in childhood. The most common trigger is a previous upper respiratory infection. It is characterized by palpable non-thrombocytopenic purpura with arthralgia and/or arthritis, gastrointestinal and kidney involvement. SARS-CoV-2 is an RNA virus that causes COVID-19 disease. It frequently affects the respiratory system with presentations ranging from rhinitis to severe conditions such as respiratory distress syndrome, septic shock, or multi-system inflammation syndrome (MIS). We describe the case of a 5-year-old boy with symptoms of IgAV/HSP as the initial form of presentation and subsequent diagnosis of SARS-CoV-2 infection, being referred to a more complex hospital with hypertensive encephalopathy, presenting a favorable evolution and complete restoration of the clinical picture.


Subject(s)
COVID-19 , Pediatrics , IgA Vasculitis , Severe acute respiratory syndrome-related coronavirus
11.
Acta Medica Philippina ; : 77-81, 2022.
Article in English | WPRIM | ID: wpr-988671

ABSTRACT

@#Henoch-Schönlein purpura (HSP) is the most common form of vasculitis in children that is clinically characterized by the classic triad of palpable purpura, joint symptoms, and abdominal pain. A 6-year-old girl, one of fraternal twins, was admitted to the Pediatric Department, Universitas Airlangga with fever, rashes on legs and arms and intermittent mild abdominal pain. She had multiple purpuric rashes on her extremities, abdomen and buttocks. Laboratory investigation revealed immunoglobulin A level of 289.6 mg/dL. The patient was diagnosed as HSP vasculitis according to EULAR criteria and treated with intravenous methylprednisolone. She was discharged after three days with normal physical examination and laboratory findings. Intraoral examination showed dental infection in the upper tooth region. The paediatrician suspected a correlation between HSP and her dental infection. The dental infection and genetic susceptibility may be the stimulant factors for the autoimmune reactions that caused HSP vasculitis. Hence, it might be useful to investigate the presence of dental infection in the etiology of HSP cases.


Subject(s)
IgA Vasculitis , Twins, Dizygotic
12.
Rev. colomb. reumatol ; 28(1): 57-63, ene.-mar. 2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1341361

ABSTRACT

RESUMEN La púrpura de Henoch-Schönlein en el adulto es un reto diagnóstico. Su baja incidencia y su sintomatología poco específica configuran un cuadro clínico que puede pasar desapercibido en diversas ocasiones o solaparse bajo el peso de diferentes sospechas diagnósticas. La púrpura de Henoch-Schönlein no es un cuadro de espectro único. Se considera un grupo de enfermedades de manifestación heterogénea con un eje patogénico común dado por el hallazgo de inflamación de la pared en vasos de pequeño calibre mediada por complejos inmunes. Este es el caso de un paciente de 70 arios quien cursa con un cuadro compatible con púrpura de Henoch-Schönlein, de inicio tardío, caracterizada por su difícil manejo y constantes recaídas. a pesar del uso cuidadoso de las pautas terapéuticas establecidas por los consensos actuales. En este paciente se documentó, de forma concomitante, una infección por citomegalovirus que al recibir tratamiento permitió el control adecuado de síntomas. Adicionalmente, este paciente presentaba una linfocitopenia que parecía ser secundaria a la infección viral.


ABSTRACT Henoch-Schönlein purpura in the adult is a diagnostic challenge. Its low incidence and its unspecific symptomatology in this age group, establish a clinical chart that can be ignored on several occasions. Henoch-Schönlein purpura is considered a group of diseases of heterogeneous manifestation with a common pathogenic axis: the finding of inflammation of the wall of the small calibre vessels, mediated by immune complexes. The case is presented of a 70-year-old patient with a difficult to treat Henoch-Schönlein purpura, with constant relapses despite the use of the therapeutic guidelines established in the current guidelines. In this patient, a concomitant cytomegalovirus infection was documented that, after receiving treatment, allowed adequate control of symptoms. Additionally, this patient also had a lymphocytopenia that was secondary to cytomegalovirus.


Subject(s)
Humans , Male , Aged , IgA Vasculitis , Cytomegalovirus , Diagnosis , Therapeutics , Cytomegalovirus Infections
13.
Medisur ; 19(1): 175-181, graf
Article in Spanish | LILACS | ID: biblio-1180842

ABSTRACT

RESUMEN La vasculitis IgA, también conocida como púrpura de Schönlein-Henoch, es una vasculitis leucocitoclástica que involucra pequeños vasos con depósito de inmunocomplejos IgA. Puede abarcar piel, articulaciones, riñones y tracto gastrointestinal. Su presentación en adultos es rara, y las formas clínicas suelen ser más agresivas. Es objetivo del presente trabajo describir el curso y evolución de vasculitis IgA, en un paciente de 59 años, con púrpuras en miembros inferiores y tronco, hematuria macroscópica, y edema de miembros inferiores. Los complementarios mostraron creatininas elevadas, proteinuria de rango nefrótico, elevación de la IgA y anticuerpos contra el citoplasma de los neutrófilos negativos. Se descartaron causas neoplásicas. El estudio anatomo-patológico del riñón concluyó una vasculitis IgA.


ABSTRACT IgA vasculitis, also known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis that involves small vessels with deposition of IgA immune complexes. It can include skin, joints, kidneys, and gastrointestinal tract. Its presentation in adults is rare, and the clinical forms are usually more aggressive. The objective of this study is to describe the course and evolution of IgA vasculitis, in a 59-years-old patient, with purples in the lower limbs and trunk, macroscopic hematuria, and lower limb edema. The complementary ones showed elevated creatinines, nephrotic range proteinuria, elevated IgA and negative antibodies against the cytoplasm of neutrophils. Neoplastic causes were dismissed. The anatomical-pathological study of the kidney concluded IgA vasculitis.


Subject(s)
Male , Middle Aged , IgA Vasculitis/physiopathology , IgA Vasculitis/diagnostic imaging
14.
Journal of Peking University(Health Sciences) ; (6): 1072-1077, 2021.
Article in Chinese | WPRIM | ID: wpr-942299

ABSTRACT

OBJECTIVE@#To investigate the clinical characteristics of Henoch-Schonlein purpura (HSP) patients from different altitudes in Tibet plateau areas of China.@*METHODS@#A retrospective study was used to analyze the 190 HSP patients admitted to Tibet Autonomous Region People ' s Hospital form April 2014 to May 2021. The subjects were divided into 3 groups according to the altitude of long-term residence before onset and the clinical data at different altitudes were compared and analyzed.@*RESULTS@#There were no significant differences in the age of onset and gender in HSP patients at different altitudes (P>0.05). The HSP patients in high altitude areas were more likely to have digestive symptoms (P < 0.01). The patients were more likely to have kidney or joint involvement at higher altitudes. The platelets [(512.1±55.0)×109 /L] and C reactive protein [11.2 (5.7, 19.4) g/L] in high altitude areas were significantly higher than at medium altitudes [(498.3±76.9)×109 /L and 9.5 (4.6, 13.5) g/L] and lower altitudes [(456.4±81.2)×109/L and 3.7 (0.2, 8.9) g/L] respectively. The effective rate of treatment was 98.9%, while there was no significant difference of outcome from different altitudes (P>0.05). The patients who were repeatedly hospitalized all had kidney involvement and no immunosuppressive agents were added in the initial treatment.@*CONCLUSION@#HSP is common in high altitude areas. There was little difference in age of onset and gender at different altitudes. Abdominal pain was the most common clinical manifestation. Patients in high altitude areas were more likely to have severe abdominal problems. Kidney involvement may be poor prognostic factor. Early application of glucocorticoid combined with immunosuppressive agents can effectively control the disease and reduce the recurrence of HSP.


Subject(s)
Humans , Altitude , China/epidemiology , IgA Vasculitis/epidemiology , Retrospective Studies , Tibet
15.
Chinese Journal of Contemporary Pediatrics ; (12): 338-342, 2021.
Article in Chinese | WPRIM | ID: wpr-879857

ABSTRACT

OBJECTIVE@#To study the efficacy and safety of mycophenolate mofetil (MMF) versus cyclophosphamide (CTX) in the treatment of children with Henoch-Schönlein purpura nephritis (HSPN) and nephrotic-range proteinuria.@*METHODS@#A prospective clinical trial was conducted in 68 pediatric patients who were admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics and who were diagnosed with HSPN and nephrotic-range proteinuria from August 2016 to November 2019. The patients were randomly divided into two groups:MMF treatment (@*RESULTS@#At months 3, 6, and 12 of treatment, there was no significant difference in the complete remission rate and the response rate between the MMF treament and CTX treatment groups (@*CONCLUSIONS@#MMF and CTX have similar efficacy and safety in the treatment of HSPN children with nephrotic-range proteinuria.


Subject(s)
Child , Humans , Cyclophosphamide/adverse effects , Immunosuppressive Agents/adverse effects , Mycophenolic Acid/adverse effects , Nephritis/drug therapy , Prospective Studies , Proteinuria/etiology , IgA Vasculitis/drug therapy , Retrospective Studies
16.
Med. lab ; 25(3): 569-580, 2021. ilus
Article in Spanish | LILACS | ID: biblio-1343146

ABSTRACT

La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad


Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options


Subject(s)
Glomerulonephritis , Purpura , IgA Vasculitis , Steroids , Biopsy , ISCOMs , Glomerulonephritis, IGA , Kidney Failure, Chronic
17.
Chinese Journal of Contemporary Pediatrics ; (12): 111-115, 2021.
Article in Chinese | WPRIM | ID: wpr-879818

ABSTRACT

OBJECTIVE@#To explore the reasonable and effective enteral nutrition regimen for children with abdominal Henoch-Schönlein purpura (HSP).@*METHODS@#A retrospective analysis was performed on the medical data of children with abdominal HSP who were hospitalized from August 2013 to August 2018. According to the starting time of enteral nutrition after abdominal pain relief, the children were divided into three groups: < 24 hours (@*RESULTS@#The retrospective analysis showed that the children who were given extensively hydrolyzed lactoprotein formula for enteral nutrition at 24-48 hours after abdominal pain relief had a lower recurrence rate of clinical symptoms and the highest degree of satisfaction among their family members (@*CONCLUSIONS@#It is reasonable and effective to start the feeding with extensively hydrolyzed lactoprotein formula at 24-48 hours after abdominal pain relief in children with abdominal HSP.


Subject(s)
Child , Humans , Enteral Nutrition , Parenteral Nutrition , Prospective Studies , IgA Vasculitis/therapy , Retrospective Studies
18.
Chinese Journal of Contemporary Pediatrics ; (12): 49-54, 2021.
Article in Chinese | WPRIM | ID: wpr-879808

ABSTRACT

OBJECTIVE@#To study the clinical effect and mechanism of total glucosides of paeony (TGP) in the adjuvant therapy for children with Henoch-Schönlein purpura nephritis (HSPN).@*METHODS@#Sixty-four HSPN children with moderate proteinuria were divided into a TGP treatment group (@*RESULTS@#Compared with the healthy children before treatment, the children with HSPN had higher proportion of Tfh cells and expression levels of IL-21 and IL-4 (@*CONCLUSIONS@#TGP has a marked clinical effect in the treatment of HSPN and can reduce the inflammatory response of the kidney and exert a protective effect on the kidney by inhibiting the proliferation of Tfh cells and downregulating the expression of IL-21 and IL-4 in plasma.


Subject(s)
Child , Humans , Glucosides/therapeutic use , Nephritis , Paeonia , Prospective Studies , IgA Vasculitis/drug therapy
19.
Chinese Journal of Contemporary Pediatrics ; (12): 797-801, 2021.
Article in English | WPRIM | ID: wpr-888484

ABSTRACT

OBJECTIVES@#To study the effects of alfacalcidol on serum 25-(OH)D@*METHODS@#A total of 200 children with HSP were prospectively enrolled from June 2018 to June 2020. According to the random number table method, they were divided into an observation group and a control group (@*RESULTS@#After treatment, the observation group showed a significantly higher serum 25-(OH)D@*CONCLUSIONS@#Alfacalcidol can increase the serum 25-(OH)D


Subject(s)
Child , Humans , Hydroxycholecalciferols , Interleukin-6 , Prospective Studies , IgA Vasculitis/drug therapy
20.
Arch. argent. pediatr ; 118(2): 139-142, abr. 2020. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1100250

ABSTRACT

Antecedentes: La púrpura de Henoch-Schönlein (PHS) es una vasculitis sistémica de vasos pequeños. El objetivo fue evaluar el índice de neutrófilos/linfocitos (INL) en sangre y el volumen plaquetario medio (VPM) en la PHS e investigar la relación con el compromiso renal y gastrointestinal.Métodos: Se incluyeron niños con PHS y controles sanos. Se evaluaron concentración de hemoglobina, recuento de leucocitos, recuento de trombocitos, INL, VPM, velocidad de sedimentación globular y proteína C-reactiva.Resultados: El INL fue significativamente mayor en los pacientes con PHS con hemorragia gastrointestinal (p < 0,001). El valor ideal de corte del INL para predecir la hemorragia gastrointestinal fue 2,05, con 93 % de sensibilidad y 62 % de especificidad. El VPM fue significativamente mayor en los pacientes con PHS con compromiso renal (p = 0,027).Conclusiones: El INL en sangre y el VPM podrían ser útiles para identificar el compromiso renal y gastrointestinal en la PHS


Background: Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement.Methods: Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated.Results: There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027).Conclusions:Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , IgA Vasculitis/blood , Lymphocytes/pathology , Mean Platelet Volume , Neutrophils/pathology , IgA Vasculitis/diagnosis , Retrospective Studies , Lymphocyte Count , Gastrointestinal Hemorrhage , Kidney Diseases
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