Potential components and mechanism of Liangxue Tuezi Mixture in treating Henoch-Schönlein purpura based on network pharmacology and metabolomics / 中国中药杂志

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.

Efficacy and safety of mycophenolate mofetil versus cyclophosphamide in the treatment of Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria in children: a prospective randomized controlled trial / 中国当代儿科杂志

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.

Clinical effect and mechanism of total glucosides of paeony in the adjuvant therapy for children with Henoch-Schönlein purpura nephritis: a prospective randomized controlled study / 中国当代儿科杂志

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.

Clinical effect of alfacalcidol in children with Henoch-Schönlein purpura: a prospective randomized controlled trial / 中国当代儿科杂志

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

Púrpura de Schönlein-Henoch en pediatría: Diez años de experiencia en un consultorio de moderado riesgo en un hospital general / Henoch-Schönlein purpura in pediatrics: Ten years of experience at a moderate risk office of a general hospital

Introducción. La púrpura de Schönlein-Henoch (PSH) es la vasculitis leucocitoclástica de pequeños vasos más común en la infancia. Se caracteriza por púrpura palpable y/o compromiso articular, abdominal o renal. Objetivo: Describir las características epidemiológicas, clínicas, de laboratorio y evolutivas en pacientes con PSH. Población y métodos. Estudio observacional, descriptivo, retrospectivo. Se incluyeron pacientes menores de 15 años durante el período 1/1/2008 al 31/12/2017 que cumplían con los criterios de Ankara para el diagnóstico de PSH. Resultados. Se incluyeron 339 pacientes con PSH; la distribución fue similar en ambos sexos; edad promedio de presentación: 7,02 años (± 3,02). El 78 % presentó forma típica y el 22 %, atípica, con compromiso articular previo a la aparición de la púrpura. Manifestaciones articulares: el 82 %; compromiso abdominal: el 54 %; manifestaciones renales: el 19 %. El 68 % de los pacientes que tuvieron compromiso renal lo manifestaron dentro del primer mes desde el debut. El síntoma más frecuente fue la hematuria aislada y el 22 % de los pacientes con compromiso renal presentó formas moderadas-graves de nefritis. El 15 % de los pacientes presentó recurrencia/s. Conclusión. Las manifestaciones articulares fueron más frecuentes de lo descrito en otras series y el compromiso renal fue menor. Si bien este compromiso suele manifestarse en los primeros meses de evolución, se destaca la necesidad del seguimiento de estos pacientes con controles urinarios y de tensión arterial en la evaluación pediátrica.

Introduction. Henoch-Schönlein purpura (HSP) is the most common small vessel leukocytoclastic vasculitis during childhood. It is characterized by palpable purpura and/or joint, abdominal or renal involvement. Objective. To describe the epidemiological, clinical, laboratory, and evolutionary characteristics of patients with HSP. Population and methods. Observational, descriptive, and retrospective study. Patients younger than 15 years who met the Ankara criteria for HSP diagnosis were included in the period between 1/1/2008 and 12/31/2017.Results. A total of 339 patients with HSP were included; the male/female distribution was similar; their average age at onset was 7.02 years (± 3.02). The typical form was observed in 78 % and the atypical form, in 22 %, with joint involvement prior to purpura onset. Joint manifestations: 82 %; abdominal involvement: 54 %; renal manifestations: 19 %. Among patients with renal involvement, 68 % experienced it in the first month after onset. The most common symptom was isolated hematuria, and 22 % of patients with renal involvement had moderate to severe nephritis. Recurrences were observed in 15 % of patients. Conclusion. Joint manifestations were more common than what has been described in other series, whereas renal involvement was less common. Although it usually occurs in the first months of disease onset, it is worth noting that these patients need follow-up with urinary and blood pressure controls as part of their pediatric assessment.

Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

A 19-year old man with IgA vasculitis after vaccination

ABSTRACT A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72 h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.

Mutaciones en el gen MEFV y evolución clínica en pacientes pediátricos con púrpura de Schonlein-Henoch / MEFV gene mutations and clinical course in pediatric patients with Henoch-Schonlein purpura

Objetivo. Determinar la frecuencia de mutaciones del gen MEFV en niños con diagnóstico de púrpura de Schonlein-Henoch y evaluar el efecto que tienen en el pronóstico. Materiales y métodos. Estudio transversal que incluyeron pacientes pediátricos de entre 2 y 11 años, con diagnóstico de púrpura de Schonlein-Henoch. Se estudiaron para detectar 6 mutaciones en el gen MEFV (M694V, M680I, A744S, R202Q, K695R y E148Q). Resultados. Se incluyeron ochenta pacientes, de los cuales el 55% eran de sexo masculino (n= 44). La media de edad fue 6,44 ± 2,52 años. Durante el seguimiento, 9 pacientes presentaron recurrencia de la enfermedad, 5 sufrieron invaginación intestinal y 1 paciente tuvo convulsiones. Aproximadamente la mitad de los pacientes recibió corticoides. En 44 pacientes (55%) no se detectaron mutaciones en el gen MEFV. En 19 pacientes (22%) hubo una mutación heterocigota. Se encontró E148Q en 8 pacientes, M694V en 5 pacientes, A744S en 4 pacientes y la mutación heterocigota R202Q en 2 pacientes. En 1 paciente se detectó la mutación heterocigota M608I y en otro paciente se encontró la mutación homocigota M694V. En 15 pacientes se encontraron mutaciones heterocigotas compuestas en el gen MEFV. Las mutaciones en el gen MEFV no se correlacionaban con la frecuencia de compromiso renal y gastrointestinal ni con el pronóstico, desarrollo de complicaciones y uso de corticoides. Conclusiones. Las mutaciones en el gen MEFV no se correlacionan con la evolución clínica ni con las complicaciones en pacientes pediátricos con púrpura de Schonlein-Henoch en Turquía.

Objective. To determine the frequency of the MEFV gene mutations in pediatric patients diagnosed with HSP and to assess the effect of the MEFV gene mutations on their prognosis. Material and Methods. Ccross-sectional study; pediatric patients between 2-11 years diagnosed with HSP were included. These cases were investigated for 6 MEFV gene mutations (M694V, M680I, A744S, R202Q, K695R, E148Q). Results. Eighty cases were included in the study of which 55% were male (n= 44). The mean age was 6.44 ± 2.52 years. Disease recurrence occurred in 9 patients, invagination in 5 patients and convulsion in 1 patient during follow-up. Approximately half of the patients received steroids. The MEFV gene mutations was not detected in 44 (55%) of the patients. There was a heterozygous mutation in 19 (22%). E148Q was found in 8 patients, M694V in 5 patients, A744S in 4 patients, and the R202Q heterozygous mutation in 2 patients. The M608I homozygous mutation was detected in 1 patient and the M694V homozygous mutation in 1 patient. The compound heterozygous MEFV gene mutations was found in 15 patients. The presence of the MEFV gene mutations was not correlated with the frequency of renal and gastrointestinal involvement and prognosis, the development of complications and the use of steroids. Conclusion. The presence of the MEFV gene mutations does not correlate with the clinical course and complication in Turkish pediatric patients with HSP.

Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis in children

OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.

Púrpura Schõnlein Henoch con compromiso renal: reporte de un caso / Henoch Schõnlein purpura with renal involvement: case report

INTRODUCCIÓN: El Púrpura Schõnlein Henoch es una vasculitis sistémica que afecta principalmente a la piel, articulaciones, sistema gastrointestinal y renal. Es una de las vasculitis más comunes en la infancia. El compromiso renal se da en aproximadamente el40 por ciento de los casos, y en la mayoría se presenta con hematuria, sin embargo, también puede manifestarse como proteinuria, síndrome nefrótico y nefrítico. PRESENTACIÓN DEL CASO: Se presenta el caso de una paciente de 6 años que consulta en Servicio de Urgencia por dolor en el tarso del pie izquierdo de 4 días de evolución, sin antecedentes de trauma, acompañándose de dolor en rodilla izquierda con limitación a la movilización, y lesiones purpúricas en extremidades inferiores. Durante la hospitalización, la paciente presentó al inicio presiones arteriales altas y oliguria, manejándose con antihipertensivos, además del inicio del tratamiento con corticoides. Luego de la mejoría del compromiso articular y cutáneo, se evidenció compromiso renal con proteinuria, que al persistir se aumentó las dosis de corticoides, teniendo favorable respuesta al disminuir sus niveles. Al encontrarse en mejores condiciones, se decidió manejo ambulatorio manteniendo tratamiento corticoidal y antihipertensivo, controlándose en Nefrología y Reumatología Infantil. DISCUSIÓN: Se concluye de este trabajo que la corticoterapia y el manejo de la presión arterial fueron pilares fundamentales para el tratamiento en este caso, mejorando su evolución clínica y principalmente la disminución de la proteinuria con el uso de los corticoides, evitando un compromiso mayor a pesar de que no esté comprobado su beneficio en la literatura.

INTRODUCTION: The Henoch Schõnlein Purpura is a systemic vasculitis that mainly affects the skin, joints, gastrointestinal system and renal system. It is one of the most common vasculitis in childhood. Renal involvement occurs in approximately40 percent of cases, and in most cases it occurs with hematuria. However, it may also manifest as proteinuria, nephrotic and nephritic syndrome. CASE REPORT: We report the case of a 6-year old patient who consulting in the emergency service for 4 days with pain in left foot tarsal, without a history of trauma, accompanied by pain in left knee with limited mobilization, and purpuric lesions in lower extremities. During hospitalization, the patient presented at the beginning high blood pressure and oliguria, handling itself with antihypertensive, in addition to the initiation of treatment with corticosteroids. After improvement of cutaneus and articular involvement, renal involvement was evident with proteinuria, at persist was increased doses of corticosteroids, with favorable response to decrease levels. Being in better conditions was decided ambulatory management keeping antihypertensive and corticosteroid treatment, controlling in Nephrology and Child Rheumatology. DISCUSSION: It can be concluded from this study that the steroids and blood pressure management were fundamental pillars for the treatment in this case, improving their clinical course and mainly the proteinuria decreased with the use of corticosteroids, preventing a greater commitment although is not verified in the literature their advantage.

Uso de corticoesteroides en el tratamiento de la púrpura de Henoch Schonlein en pacientes hospitalizados en el Instituto Nacional de Salud del Niño, periodo 2005-2010 / Use of corticosteroids in the treatment of Henoch Schonlein purpura in hospitalized patients at the National Institute of Child Health, period 2005-2010

INTRODUCCION: La púrpura de Henoch Schonlein es una vasculitis sistémica no trombocitopénica común en la infancia, caracterizada por la afectación de piel, tracto gastrointestinal y renal, que tiene como sustrato histopatológico una vasculitis leucocitoclástica de pequeños vasos. A pesar de muchos intentos terapéuticos, no existe un consenso internacional para su tratamiento. OBJETIVO: Describir las características clínicas de la Púrpura de Henoch Schonlein en pacientes que reciben corticoides hospitalizados en el Instituto Nacional de Salud del Niño. METODOLOGIA: Es un estudio descriptivo, transversal, retrospectivo. La muestra estuvo conformada por todos los pacientes con diagnóstico de Púrpura de Henoch Schonlein hospitalizados en el Instituto Nacional de Salud del Niño durante el periodo 2005-2010 que cumplieron con los criterios de inclusión. RESULTADOS: Se revisaron las historias clínicas de 118 niños, observándose que la media en la edad de presentación fue de 6 años, con una distribución similar en ambos sexos. La presentación fue mayor durante los meses de invierno. La media de duración de la enfermedad fue de 15.3 días. Se encontró purpura palpable en el 100 por ciento de los pacientes, dolor abdominal en 82 pacientes (70 por ciento) y presencia de sangre en heces en 20 pacientes (17 por ciento). El promedio de duración de las manifestaciones digestivas fue de 3.7 días. Se observó hematuria en 35 pacientes (30 por ciento). La media en días de la duración de las manifestaciones renales fue de 13.72 días. Se tomó biopsia cutánea a 13 pacientes, encontrándose Vasculitis leucocitoclástica como principal diagnóstico. Se administró corticoides al 81.7 por ciento de los pacientes que presentaron dolor abdominal y al 88.6 por ciento de los pacientes que presentaron hematuria. CONCLUSIONES: El dolor abdominal fue encontrado en un alto porcentaje, representando el 70 por ciento de los pacientes con diagnóstico de Púrpura de Henoch Schonlein. La administración...

INTRODUCTION: Henoch Schonlein Purpura is a systemic vasculitis not thrombocytopenic common in childhood, characterized by the involvement of skin, gastrointestinal tract and kidney, whose substrate histopathological is a leukocytoclastic vasculitis of small vessels. Despite many therapeutic attempts, there is not international consensus for treatment. OBJECTlVE: To describe the clinical characteristics of Henoch Schonlein Purpura in hospitalized patients receiving corticosteroids at the National Institute of Child Health. METHODOLOGY: A cross-sectional study, retrospective. The sample consisted of all patients diagnosed with Henoch Shonlein Purpura hospitalized at the National Institute of Child Hea1th during the period 2005-2010 that met the inclusion criteria. RESULTS: We reviewed the medical records of 118 children, the average age at presentation was 6 years, with a similar distribution in both sexs. The presentation was higher during the winter months. The mean duration of disease was 15.3 days. Palpable purpura was found in 100 per cent of patients, abdominal pain in 82 patients (70 per cent) and presence of blood in feces in 20 patients (17 per cent). The average duration of gastrointestinal manifestations was 3.7 days. Hematuria was observed in 35 patients (30 per cent). The mean days of the duration of the renal manifestations were 13.72 days. Skin biopsy was taken in 13 patients, being leukocytoclastic vasculitis as the main diagnosis. Corticosteroids were administered to 81.7 per cent of patients had abdominal pain and 88.6 per cent of patients had hematuria. CONCLUSIONS: The abdominal pain was found in a high percentage, representing 70 per cent of patients diagnosed with Henoch Shonlein Purpura. Corticosteroid administration was performed at 66 per cent of patients diagnosed with Henoch Shonlein Purpura, gastrointestinal manifestations being the main reason for its use. Received corticosteroid treatment 82 per cent of patients had abdominal...

Compromiso testicular en púrpura de Schõnlein Henoch, utilidad de la ecotomografía doppler / Testicular involvement in Henoch-Schõnlein purpura: the useful of doppler sonography

El púrpura de Schõnlein Henoch (PSH) es la vasculitis más frecuente de la infancia; afecta principalmente la piel, articulaciones y sistema gastrointestinal. El compromiso de los testículos se observa en el 15 por ciento de los pacientes, pero es raro como forma de presentación (1-2 por ciento). El presente caso clínico describe un niño de 4 años de edad sin antecedentes mórbidos que consultó por dolor, eritema, aumento de volumen testicular derecho y púrpura palpable de extremidades inferiores, cuya ecografía testicular mostró testículos normales con flujo Doppler normal y aumento de grosor de escroto y cordón espermático ipsi lateral. Exámenes de laboratorio fueron normales. Se diagnosticó PSH con compromiso testicular y se trató exitosamente con corticoides orales por una semana. Es importante considerar esta patología como diagnóstico diferencial de testículo agudo, para evitar la exploración quirúrgica innecesaria. Las características ecográficas permiten confirmar la sospecha diagnóstica.

Henoch Schõnlein purpura is the most common vasculitis presenting in the childhood, with frequent skin, articular and gastrointestinal compromise. Testicular pain is rare as an onset symptom (1-2 percent), but 15 percent of the cases develop it during the illness course. This case report describes a previously healthy 4 year old boy, who was admitted in theemergency department with a painful, erythematous right scrotal swelling, associated with palpable purpura on both inferior extremities. Testicular Color Doppler US imagingout ruled testicular alterations, but the ipsilateral scrotum and spermatic cord were enlarged. Blood was drawn for serologic and hematologic testing with normal results. Shõnlein Henoch purpura with testicular involvement was diagnosed and successfully treated with oral corticosteroids for one week. It is important to consider Schõnlein Henoch purpura among the differential diagnosis of acute testicle in order to avoid unnecessary surgical exploration. Testicular Doppler Ultrasound imaging is recommended to confirm the diagnosis.

Púrpura de Schõnlein-Henoch: manifestaciones clínicas más frecuentes y actualización del tratamiento esteroidal / Schõnlein-Henoch purpura: clinical manifestations and updates in steroidal treatment

El púrpura de Schõnlein-Enoch corresponde a la vasculitis más frecuente en pediatría, afecta principalmente vasos pequeños de la piel, articulaciones, tracto gastrointestinal y riñones. este artículo revisa la actualización del tratamiento esteroidal.

Envolvimento renal na púrpura de Henoch-Schõnlein: uma análise multivariada de fatores prognósticos iniciais / Renal involvement in Henoch-Schõnlein purpura: a multivariate analysis of initial prognostic factors

OBJETIVOS: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schõnlein. MÉTODOS: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schõnlein admitidos em nosso Hospital Universitário foram revistos. Os fatores preditivos iniciais avaliados nos primeiros 3 meses incluíram: dados demográficos, manifestações clínicas (púrpura palpável persistente, artrite, dor abdominal, dor abdominal intensa, sangramento gastrointestinal, orquite, envolvimento do sistema nervoso central e hemorragia pulmonar), exames laboratoriais (níveis séricos de IgA) e tratamento utilizado (corticosteróides, imunoglobulina endovenosa e medicação imunossupressora). Os pacientes foram divididos em dois grupos (com presença ou ausência de nefrite) e avaliados de acordo com a análise univariada e multivariada. RESULTADOS: Nefrite foi evidenciada em 70 pacientes (49,3 por cento). A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95 por cento 1,18-2,21), sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95 por cento 1,10-2,26) e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95 por cento 1,28-2,40) foram associados com uma maior incidência de envolvimento renal. Na análise multivariada, a regressão logística mostrou que a única variável independente na predição da ocorrência de nefrite foi dor abdominal intensa (p < 0,012; OR = 2,593; IC95 por cento 1,234-5,452). CONCLUSÕES: Dor abdominal intensa representou um preditor significativo da nefrite na púrpura de Henoch-Schõnlein. Conseqüentemente, os pacientes pediátricos com esta manifestação clínica devem ser rigorosamente seguidos, devido ao maior risco de acometimento renal.

OBJECTIVES: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schõnlein purpura. METHODS: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schõnlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis, central nervous system involvement and pulmonary hemorrhage), laboratory tests (serum IgA levels) and treatment given (corticosteroids, intravenous immunoglobulin and immunosuppressive drugs). Patients were divided into two groups (presence or absence of nephritis) and assessed by univariate and multivariate analysis. RESULTS: Evidence of nephritis was detected in 70 patients (49.3 percent). The univariate analysis revealed that severe abdominal pain (p = 0.0049; OR = 1.6; 95 percentCI 1.18-2.21), gastrointestinal bleeding (p = 0.004; OR = 1.6; 95 percentCI 1.10-2.26) and corticosteroid use (p = 0.0012; OR = 1.7; 95 percentCI 1.28-2.40) were all associated with increased incidence of renal involvement. In the multivariate analysis, logistic regression demonstrated that the only independent variable that predicted nephritis was intense abdominal pain (p < 0.012; OR = 2.593; 95 percentCI 1.234-5.452). CONCLUSIONS: Severe abdominal pain was a significant predictor of nephritis in Henoch-Schõnlein purpura. Consequently, pediatric patients exhibiting this clinical manifestation should be rigorously monitored, due to the increased risk of renal involvement.

Unusual presentations of Henoch-Schonlein purpura: case report and review of the literature

Henoch-Schonlein purpura, the most common leukocytoclastic vasculitis of childhood, may present with a wide variation of signs and symptoms. Other systems involvements that are usually rare and underestimated are commonly reported as case reports in the literature. Neuromuscular and hepatic involvement is very rare. We present a ten years old boy who had Henoch-Schonlein purpura with neuromuscular and hepatic involvement and had rapid response to therapy

Treatment of acute infectious purpura fulminans with activated protein C.

Infectious purpura fulminans is associated with high mortality and morbidity despite standard antimicrobial therapy. We report satisfactory clinical outcome in two children with sepsis associated purpura fulminans who were treated with activated protein C (APC). There is need for proper evaluation of the efficacy of this extremely expensive therapeutic modality by randomized controlled trials before it is made standard of care in childhood infectious purpura fulminans.

Púrpura de Scholein-Henoch en el adulto / Scholein-Henoch purpura in an adult

La púrpura de Scholein-Henoch (PSH) es una vasculitis autolimitada que afecta a los pequeños vasos. Si bien su etiopatogenia es desconocida, se reconocen algunos factores desencadenantes. Es mediada por inmunocomplejos a predominio de IgA. Clínicamente se caracteriza por la tétrada consistente en púrpura palpable no trombocitopénica, artritis, nefritis y enteropatía. Aparece con mayor frecuencia en la primera y segunda década de la vida y es poco usual en adultos. El tratamiento se basa en medidas de sostén y en ocasiones corticoides, siendo fundamental el control de la función renal a largo plazo. Se presenta un caso y se realiza una revisión de la literatura sobre esta patología