Changes in the spectrum of biopsy-proven renal diseases over 11 years: a single-center study in China.

BACKGROUND: There have been some shifts in the frequency and distribution of biopsy-proven renal diseases in China over recent years. The aim of the study was to investigate the changing spectrum of renal diseases from the view of kidney biopsy data in a single center of China. METHODS AND RESULTS: A total of 10,996 cases of native renal biopsies from patients aged ≥15 years old in Huashan Hospital, Fudan University, between 2008 and 2018 were analyzed retrospectively. The results showed that primary glomerular nephropathy (PGN) remained the most common biopsy-proven renal disease (69.42% of total), with IgA nephropathy (IgAN) accounting for 44.40% of PGN, membranous nephropathy (MN) for 28.55%, minimal change disease (MCD) for 13.26% and focal segmental glomerulosclerosis (FSGS) for 8.00%. During the study period, the proportion of MN in PGN appeared an increasing tendency, while that of IgAN and MCD remained stable and that of FSGS showed a decline. Secondary glomerular nephropathy (SGN) constituted 21.54% of total cases, among which the leading two diseases were lupus nephritis (LN) and Henoch-Schonlein purpura nephritis (HSN) which accounted for 41.08% and 19.11% respectively. CONCLUSIONS: The 11-year retrospective study revealed that PGN was the predominant histologic diagnosis among patients undergoing renal biopsy and the most frequent type of PGN remained to be IgAN, followed by MN which increased dramatically.

Clinical characteristics and risk factors for kidney involvement in children with immunoglobulin A vasculitis.

BACKGROUND: Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is associated with a long-term unfavorable outcome and can lead to significant morbidity. This study was conducted to describe the clinical and laboratory characteristics of childhood IgAV with kidney involvement and to identify risk factors associated with IgAV nephritis (IgAVN). METHODS: This was an ambidirectional descriptive study of 77 children with IgAV. All demographic data, clinical features, and laboratory tests were collected from electronic medical records from January 2010 to December 2022. Risk factors for kidney involvement in IgAV were assessed using multivariate logistic regression. Kaplan-Meier survival analysis was used to calculate the time to commencement of kidney involvement. RESULTS: Twenty-five children (32.4% of the IgAV patients) developed IgAVN. The common findings in IgAV with kidney involvement were microscopic hematuria (100%), nephrotic range proteinuria (44%), and non-nephrotic range proteinuria (40%). Multivariate logistic regression showed that age greater than 10 years (adjusted hazard ratio, AHR 4.66; 95% confidence interval, CI, 1.91-11.41; p = 0.001), obesity (body mass index, BMI, z-score ≥ +2 standard deviations, SDs) (AHR 3.59; 95% CI 1.41-9.17; p = 0.007), and hypertension at onset (AHR 4.78; 95% CI 1.76-12.95; p = 0.002) were associated significantly with kidney involvement. During follow up, most IgAV patients developed nephritis within the first 9 months. CONCLUSION: Age greater than 10 years, obesity, and hypertension at presentation were predictive factors for IgAVN. Our study emphasized that IgAV patients with risk factors should be closely monitored for at least 1 year after the onset of the disease.

Common Seasonal Pathogens and Epidemiology of Henoch-Schönlein Purpura Among Children.

Importance: Henoch-Schönlein purpura (HSP) is the most common type of vasculitis in children. The factors that trigger the disease are poorly understood. Although several viruses and seasonal bacterial infections have been associated with HSP, differentiating the specific associations of these pathogens with the onset of HSP remains a challenge due to their overlapping seasonal patterns. Objective: To analyze the role of seasonal pathogens in the epidemiology of HSP. Design, Setting, and Participants: This cohort study comprised an interrupted time-series analysis of patient records from a comprehensive national hospital-based surveillance system. Children younger than 18 years hospitalized for HSP in France between January 1, 2015, and March 31, 2023, were included. Exposure: Implementation and relaxation of nonpharmaceutical interventions (NPIs) for the COVID-19 pandemic, such as social distancing and mask wearing. Main Outcomes and Measures: The main outcomes were the monthly incidence of HSP per 100 000 children, analyzed via a quasi-Poisson regression model, and the estimated percentage of HSP incidence potentially associated with 14 selected common seasonal pathogens over the same period. Results: The study included 9790 children with HSP (median age, 5 years [IQR, 4-8 years]; 5538 boys [56.4%]) and 757 110 children with the infectious diseases included in the study (median age, 0.7 years [IQR, 0.2-2 years]; 393 697 boys [52.0%]). The incidence of HSP decreased significantly after implementation of NPIs in March 2020 (-53.6%; 95% CI, -66.6% to -40.6%; P < .001) and increased significantly after the relaxation of NPIs in April 2021 (37.2%; 95% CI, 28.0%-46.3%; P < .001). The percentage of HSP incidence potentially associated with Streptococcus pneumoniae was 37.3% (95% CI, 22.3%-52.3%; P < .001), the percentage of cases associated with Streptococcus pyogenes was 25.6% (95% CI, 16.7%-34.4%; P < .001), and the percentage of cases associated with human rhino enterovirus was 17.1% (95% CI, 3.8%-30.4%; P = .01). Three sensitivity analyses found similar results. Conclusions and Relevance: This study found that significant changes in the incidence of HSP simultaneously with major shifts in circulating pathogens after NPIs for the COVID-19 pandemic indicated that approximately 60% of HSP incidence was potentially associated with pneumococcus and group A streptococcus. This finding suggests that preventive measures against these pathogens could reduce the incidence of pediatric HSP.

Epidemiology and clinical characteristics of biopsy-confirmed adult-onset IgA vasculitis in southern Sweden.

OBJECTIVE: Immunoglobulin A vasculitis (IgAV) is the most prevalent primary childhood vasculitis in Sweden, but is considerably rarer in adults. This study aims to describe the epidemiology, clinical characteristics and renal outcome of adult-onset IgAV in Skåne, Sweden. METHODS: The study area consisted of Skåne, the southernmost region of Sweden, with a population ≥18 years of 990 464 on 31 December 2010. Adult patients assigned the International Classification of Diseases-10 code for IgAV (D69.0) from 2000 through 2019 were retrospectively identified in a population-based database. Medical records were reviewed to validate the diagnosis of IgAV and extract data. Only patients with clinical manifestations of IgAV and biopsy-confirmed disease were included. The annual incidence and point prevalence of biopsy-confirmed IgAV were estimated. RESULTS: Fifty-nine patients (19 women) were classified as having adult-onset IgAV. The incidence was 3 per 1 000 000 and was higher among men than women (4 vs 2/1 000 000, p=0.004). Ninety-seven per cent of patients presented with non-thrombocytopenic purpura, 78% with renal involvement, 59% with arthritis/arthralgia and 39% with gastrointestinal symptoms. Fifteen per cent developed chronic kidney disease stage ≥G3 a and one patient progressed to end-stage kidney disease during follow-up. CONCLUSION: Adult-onset IgAV is rare in southern Sweden with the incidence higher in men than in women. IgAV frequently affects the kidneys and leads to chronic kidney disease in adults, although the long-term renal outcome appears favourable compared with other small-vessel vasculitides affecting the kidneys.

IgA vasculitis in adults, pediatrics and non-vasculitic IgA nephropathy, retrospective analysis from 2 centers.

Renal involvement represents the major long-term morbidity associated with IgA vasculitis (IgAV). Our aim was to evaluate clinical characteristics and long-term renal outcomes of IgAV in pediatrics and adults comparing to IgA nephropathy (IgAN). Our retrospective study included children and adults with IgAV and IgAN patients, admitted in a 13-year period (2007-2019) to rheumatology clinics and in hospital pediatric and internal medicine departments. We compared frequencies of clinical manifestations, laboratory findings, treatments, long-term outcomes at 1 year follow-up, including all-cause mortality and dialysis until the end of follow-up time. A total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were evaluated. Adult IgAV patients were significantly older than IgAN patients (53.1 ±â€…17.4 years vs 45.1 ±â€…15.7 years respectively, P = .02) and had significantly higher rates of cardiovascular comorbidities. The risk and time to dialysis were similar among IgAN and adult IgAV groups. Yet, overall mortality at long term follow up was higher in IgAV adult group compared to IgAN. No dialysis or renal transplantation were reported in pediatric IgAV patients. IgAV and IgAN adult patients were comparable regarding risk of end stage renal disease. Of note, high mortality rates were observed among adult IgAV group.

Analysis of clinical characteristics of Henoch-Schonlein purpura patients from different altitudes in plateau areas / 北京大学学报(医学版)

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.

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.

Prevalencia y manifestaciones clínicas más frecuentes de la Púrpura de Henoch Schõnlein en la población pediátrica de la Clínica Universitaria Colombia y Clínica Reina Sofía entre los años 2009-2015 / Prevalence and most frequent clinical manifestations of the Henoch-Schõnlein Purpura in the pediatric population of the Clínica Universitaria Colombia and Clinica Reina Sofia between the years 2009-2015

Objetivo: estimar la prevalencia y determinar las manifestaciones clínicas más frecuentes de la Púrpura de Henoch-Schõnlein teniendo en cuenta los criterios EULAR-PRINTO-PReS en la población pediátrica que ingresó a la Clínica Universitaria Colombia y Clínica Reina Sofía a los servicios de urgencias y hospitalización entre los años 2009 al 2015. Método: estudio descriptivo, cohorte histórica, que se realizó en la ciudad de Bogotá, se incluyeron 109 pacientes menores de 18 años con diagnóstico de Púrpura de Henoch-Schõnlein. Resultados: Se encontró una prevalencia en el ámbito hospitalario de 2,07 casos por cada 1.000 hospitalizaciones y en el escenario de urgencias 0,2 por cada 1.000 consultas, respecto a las manifestaciones clínicas se comportó así: Purpura palpable (100 por ciento), artritis/artralgia (80 por ciento), Dolor abdominal (33 por ciento), nefropatía (6 por ciento), Depósitos IgA (0 por ciento). Conclusiones: En nuestro estudio, esta enfermedad fue mucho más prevalente en niñas, respecto a las manifestaciones clínicas de acuerdo a los criterios diagnósticos EULAR/PRINTO/Pres se comportó de manera similar a otros estudios.

Objective: estimate the prevalence and determine the most frequent clinical manifestations of Henoch-Schõnlein Purpura, taking into account the EULAR-PRINTO-PReS criteria in the pediatric population that entered the emergency and Hospitalization services in the Clinica Universitaria Colombia and Clínica Reina Sofía between the years 2009 and 2015. Method: descriptive study, historical cohort, carried out in the city of Bogotá, included 109 patients under the age of 18 years with diagnosis of Henoch-Schõnlein Purpura. Results: A prevalence of 2.07 cases per 1000 hospitalizations was found in the hospital setting and in the emergency setting 0.2 per 1000 visits, in relation to the clinical manifestations, it was: Palpable purpura (100percent), arthritis / arthralgia 80 percent, Abdominal pain (33 percent), nephropathy (6 percent), IgA deposits (0 percent). Conclusions: In our study, this disease was much more prevalent in girls, on the other hand in regard to the clinical manifestations according to diagnostic criteria of EULAR / PRINTO / Pres, our population behaved in a similar way to other studies.

Púrpura de Henoch-Schõnlein: epidemiología, clínica y seguimiento por cinco años en pacientes hospitalizados en el Instituto Especializado de Salud del Niño, 1995-1999 / Henoch-Schõnlein Purpura: epidemiology, clinical and follow-up for five years in patients hospitalized in Specialized Institute of Child Health, 1995-1999

Objetivo: Determinar las características epidemiológicas, clínicas y el pronóstico de la púrpura de Henoch-Schönlein (PHS) en pacientes hospitalizados en el Instituto Especializado de Salud del Niño (IESN), durante el periodo 1995-1999.Material y métodos: Estudio transversal retrospectivo. Se seleccionaron las historias de pacientes entre 2 y 17 años, hospitalizados con diagnóstico de PHS, que cumplieran con los criterios. Se excluía a todo paciente que hubiera presentado patologías previas con vasculitis o durante el curso de su enfermedad. Los datos obtenidos fueron procesados por SPSS versión 13 y Microsoft Excel 2003. Resultados: Se estudiaron 49 niños, 71% mujeres y 29% varones, el rango de edades fue de 2 a 16 años (media 6.2±3.3), el grupo etario más afectado fue el escolar (63%), seguido del preescolar (33%). Se identificó como posible factor desencadenante la infección de vías respiratorias altas en 14 pacientes (29%), de los cuales sólo uno registró ingesta previa de fármacos. El número de casos de PHS fue mayor en invierno con un 43%. Se observó compromiso cutáneo (100%), articular (63%), digestivo (53%) y renal (20%). El signo de púrpura palpable se encontróen todos los pacientes y los miembros inferiores fueron los más afectados (98%). El patrón articular más frecuente fue el oligoarticular. A nivel gastrointestinal el dolor abdominal fue el signo de mayor presentación (49%), y la afección renal se presentó en un 20%, más frecuentemente con microhematuria. No existió uniformidad en la solicitud de los exámenes auxiliares de laboratorio. Se realizó biopsia de piel en nueve pacientes, encontrándose vasculitis leucocitoclástica en seis de ellos. La inmunofluorescencia directa, realizada en cinco pacientes, fue negativa. Se registraron controles posteriores al alta en solo el 31% de pacientes, de los cuales el 8% registró recurrencia...

Objectives: To determine the epidemiological and clinical characteristics as well as the prognosis of Henoch-Schönlein Purpura (HSP) inhospitalized patients at the Specialized Institute of Child Health (IESN) during the period 1995-1999. Material and Methods: A retrospective cross study. We selected clinical records from patients aged 2 to 17 years, hospitalized al the IESN with a diagnosis of HSP, who met the criteria. All patients who had vasculitis diseases prior or during the course of their illness were excluded. The data was processed by SPSS 13th version and Microsoft Excel 2003. Results: We studied 49 children, 71% women and 29% male, age range was from 2 to 16 years (average 6.2±3.3 years), the age group most affected was the schoolar (63%), followed by preschoolar (33%). The upper respiratory tract infection was identified as a possible trigger in 14 patients (29%), of which only 1 recorded previous ingestion of drugs. The number of cases of HSP was higher in winter with 43%. It is characterized by the association of skin(100%), joint (63%), gastrointestinal (53%) and renal (20%) manifestations. The sign of palpable purpura was found in allpatients and lower limbs were the most affected (98%). The most frequent pattern was the oligoarticular. Abdominal pain was the greatest sign ofsubmission (49%) between the gastrointestinal manifestations, and renal disease was presented by 20%, more often with microscopic hematuria.There was no uniformity in the application of laboratory tests. Skin biopsy was performed in 9 patients, found leukocytoclastic vasculitis in sixof them. The direct immunofluorescence, in 5 patients, was negative. Only 31% of patients were follow up, of whom 8% recorded recurrence. Conclusions: The HSP is characterized by skin, joints, gastrointestinal and renal manifestations. The palpable purpura is the most important sing and constitute the basis of clinical sine qua non to suspect or establish the diagnosis...

Púrpura de Schönlein-Henoch. Estudio de factores asociados con el desarrollo y evolución de la enfermedad / Henoch-Schönlein purpura. Study of factors associated with the development and course of the disease

Introducción: La púrpura de Schönlein-Henoch (PSH) es la vasculitis más frecuente en la infancia. El objetivo fue investigar posibles factores asociados con el desarrollo y evolución de la enfermedad. Pacientes y métodos: Se realizó un estudio caso-control. Los casos fueron los pacientes con PSH vistos en Reumatología y Nefrología Pediátrica de un hospital terciario durante 2 años y los controles los atendidos en Reumatología Pediátrica por problemas mecánicos o funcionales. Se recogieron antecedentes infecciosos, ingesta de medicación previa y se investigó la seroprevalencia de diferentes virus. Se realizó frotis faríngeo y se cuantificó la cifra de antiestreptolisina O. Posteriormente los pacientes fueron seguidos de forma prospectiva y se compararon las diversas manifestaciones de la enfermedad con los factores epidemiológicos referidos. Resultados: El número de pacientes fue 70 y el de los controles 58. Sólo el antecedente de infección del tracto respiratorio superior (ITRS) y la ingesta de antibióticos se asociaron de forma independiente con el desarrollo de PSH. El 100 % de los pacientes presentó púrpura palpable. Las manifestaciones digestivas fueron las más frecuentes (63 %), seguidas de las articulares (50 %) y de las renales (18,6 %). Las artralgias fueron más frecuentes en las niñas y el tiempo de duración de la púrpura fue más prolongado cuando la enfermedad empezó en primavera o verano. Ninguno de los otros factores epidemiológicos estudiados se asoció con el desarrollo de la enfermedad ni con parámetros de peor evolución. Conclusiones: El antecedente de ITRS y la ingesta de antibióticos se asociaron con el desarrollo de PSH. Otros factores epidemiológicos estudiados no se encontraron asociados con el desarrollo ni la evolución de la enfermedad

Introduction: Henoch-Schönlein purpura (HSP) is the most common form of pediatric vasculitis. The objective of this study was to determine the factors associated with the development and course of this disease. Patients and methods: A case-control study was performed. The case group included patients with HSP followed-up at the pediatric rheumatology and nephrology units of a tertiary university hospital over a 2-year period. The control group included children followed-up at the pediatric rheumatology unit for mechanical or non-inflammatory conditions. A medical history including data on infectious conditions and previous medication was taken. A throat culture was performed and antistreptolysin 0 levels were quantified. The seroprevalence of different viruses was investigated. Subsequently, the patients were prospectively followed-up and disease manifestations were compared with reported epidemiological factors. Results: Seventy patients and 58 controls were studied. A history of a recent upper respiratory infection (URI) and antibiotic intake were independently associated with development of HSP. Palpable purpura was present in 100 % of the patients. Gastrointestinal manifestations were recorded in 63 %, articular in 50 %, and renal in 18.6 %. Arthralgias were more frequent in girls and purpura duration was longer when disease onset occurred in spring or summer. Other factors studied were not associated with disease development or with a worse clinical course. Conclusions: Factors associated with the development of HSP were a history of URI and antibiotic administration. Other epidemiological factors studied were not associated with either the development or the course of the disease

Henoch-Schõnlein purpura: recurrence and chronicity

OBJETIVOS: Descrever um grupo de pacientes com púrpura de Henoch-Schõnlein seguido em nosso serviço, com ênfase nos casos recorrentes e crônicos, e comparar pacientes com doença monocíclica ou recorrente segundo características clínicas e demográficas. MÉTODOS: Foram avaliados dados de 67 pacientes atendidos desde o início da doença. Doze pacientes foram excluídos, pois abandonaram o seguimento antes de 3 meses de acompanhamento, sendo incluídas 55 crianças no estudo. Recorrência foi definida pela presença de novo quadro após um período de 3 meses sem sintomas, e cronicidade como persistência de manifestações cutâneas, abdominais e renais por um período ≥ 12 meses. RESULTADOS: Foi observada recorrência em 8/55 pacientes (14,4 por cento) e cronicidade em quatro (7,2 por cento). Em 29/55 pacientes (52,7 por cento), foi constatada infecção como fator desencadeante. Curso monocíclico esteve presente em 43 pacientes (26 meninas, média de idade de 5,4 anos). Acometimento gastrointestinal e renal foi observado em 55,8 e 20,9 por cento dos pacientes, respectivamente. Dentre os 12 pacientes com púrpura de Henoch-Schõnlein recorrente ou crônica, três apresentaram artrite, quatro tiveram sinais e sintomas de comprometimento abdominal e sete de doença renal: hematúria microscópica em cinco, hematúria macroscópica em um e hematúria com proteinúria em um paciente. Idade de início tardia foi a única variável relacionada com a presença de recorrência (p < 0,05). CONCLUSÕES: Como observado na literatura médica, o curso monocíclico é mais freqüente nas crianças com início da doença em idade mais precoce. Pacientes com púrpura de Henoch-Schõnlein devem ser seguidos a longo prazo, uma vez que recorrências e/ou cronicidade são observadas em mais de 20 por cento dos casos.

OBJECTIVES: To describe a group of patients treated at our service for Henoch-Schõnlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. METHODS: Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return for follow-up consultations after less than 3 months, leaving a total of 55 children in the study sample. Recurrence was defined as the presence of a fresh episode after a period of at least 3 months without symptoms, and cases were defined as chronic when cutaneous, abdominal and renal manifestations persisted for a period of 12 months or more. RESULTS: Recurrence was observed in 8/55 patients (14.4 percent) and four cases were chronic (7.2 percent). In 29/55 patients (52.7 percent), infection was identified as the trigger factor. A monocyclic clinical course was observed in 43 patients (26 of whom were girls, with a mean age of 5.4 years). Gastrointestinal and renal involvement was observed in 55.8 and 20.9 percent of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schõnlein purpura, three had arthritis, four exhibited signs and symptoms of abdominal involvement and seven of kidney disease: microscopic hematuria in five, macroscopic hematuria in one and hematuria with proteinuria in one other. Late onset was the only variable related to recurrence (p < 0.05). CONCLUSIONS: As is observed in medical literature, monocyclic cases are more common among children with early onset disease. Patients with Henoch-Schõnlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20 percent of the total.

Invaginación intestinal en la púrpura de Schonlein-Henoch / Intestinal intussusception in Henoch-Schonlein purpura

Introducción: La invaginación intestinal es la complicación abdominal más frecuente en el paciente con púrpura de Schonlein-Henoch (PSH). y presenta una clínica superponible a la del paciente con afectación intestinal sin complicación, lo que puede retrasar el diagnóstico. Caso clínico: Paciente de 3 años que reingresa dos días después de ser dado de alta por un brote agudo de PSH. A su ingreso presentaba proteinuria, hematuria y dolor abdominal. En el ingreso previo, se descartó la invaginación mediante ecografía. La sintomatología abdominal fue aumentando de intensidad, asociándose vómitos incoercibles. A las 72 horas del ingreso y con ecografía compatible con invaginación, fue remitido al Hospital General de Castellón, donde se le practicó una laparotomía exploradora y una resección intestinal por invaginación íleo-ileal evolucionada. Conclusión: La alta incidencia de sintomatología abdominal en el paciente con PSH, así como la importante morbilidad derivada del retraso diagnóstico en la invaginación intestinal, obliga a un estrecho seguimiento de estos pacientes, debiéndose efectuar estudios ecográficos seriados con el objetivo de descartar la invaginación o conseguir un diagnóstico precoz. En casos seleccionados y si se dispone de un equipo quirúrgico, puede considerarse el tratamiento conservador, puesto que se han descrito resoluciones espontáneas

Introduction. Intestinal intussusception is the most common abdominal complication in patients with Henoch-Schonlein purpura (HSP). The clinical features resemble those of intestinal involvement without complications, a circumstance that can lead to diagnostic delays. Case reporto A three-year-old boy was readmitted three days after he had been discharged after hospitalization for an acute HSP episode. He presented with abdominal pain, proteinuria and hematuria. Abdominal symptoms had also been detected during his first hospital stay, but complications had been ruled out by means of ultrasonography. Abdominal pain became increasingly severe and uncontrollable vomiting appeared. Seventy-two hours later he was transferred to our center with abdominal ultrasound findings compatible with intestinal intussusception. He underwent exploratory laparotomy, which revealed ileo-ileal intussusception. Although it was reduced, segmental resection was necessary beca use of intestinal necrosls. Conclusion. Morbidity derived from missed intussusception, together with a high incidence of abdominal symptoms in HSP patients, makes close follow-up of these patients indispensable. Serial ultrasonographic studies are indicated in order to rule out intussusception and establish an accurate early diagnosis. In selected cases, and provided that a surgical team is available, conservative treatment can be considered, as spontaneous reductions have been reported

Púrpura trombopénica inmunológica en la infancia / Immunological thromhopenic purpura in childhood

La púrpura trombopénica idiomática es una enfermedad común en la edad pediátrica con tendencia a resolverse espontáneamente en la mayor parte de los casos. Sin embargo, en cerca de un 15% evoluciona a formas crónicas rebeldes a todo tratamiento que afectan significativamente a la calidad de vida de los pacientes y de sus familias. La falta de evidencia en la literatura actual hace que su manejo se base a menudo en la opinión y las prácticas locales. Presentamos 5 casos que ejemplifican los diferentes cursos clínicos y respuestas al tratamiento, así como una puesta al día de lo publicado hasta la fecha (AU)

Idiopathic thrombopenic purpura is a common disease in the pediatric with tendency to resolves spontaneously in most of the cases. However, it evolves to chronic forms resistant to all treatmentsin approximately 15%. These significantly affect the quality of life of the patients and their families. Because of lack of evidence in the present literature, its management is often based on opinion and local practices. We present 5 cases thet illustrate the different clinical courses and responses to treatment and an up-dating of that published up to now (AU)

Púrpura de Schölein-Henoch:Estudio retrospectivo de los últimos 20 años / Henoch-Schonlein purpura: a 20-year retrospective study

Fundamento. La púrpura de Schónlein-Henoch (PSH) es una vasculitis sistémica por hipersensibilidad, mediada por Ig-A, que afecta casi exclusivamente a la edad pediátrica. Las afectaciones principales son a nivel cutáneo, gastrointestinal, renal y articular. La afectación nefrológica, que se puede presentar desde su inicio o bien días o semanas después, condiciona el pronóstico. Objetivo. Evaluar las características clínicas, epidemiológicas y evolutivas de la enfermedad de los niños con diagnóstico de PSH controlados en consultas externas de nefrología pediátrica. Método. Estudio retrospectivo y descriptivo realizado entre septiembre de 1980 i enero de 2001. Se han revisado las historias clínicas de los pacientes controlados en consulta externa por presentar una PSH. Resultado. Hemos estudiado 77 pacientes con una edad media de 4.7 años (2 -15 años). Encontramos antecedentes de infección aguda en 17 (22 por ciento) de los que 6 son amigdalitis. Todos han presentado afectación cutánea no siendo la forma de debut en dos (1 dolor abdominal, 1 artritis). Encontramos dolor abdominal en 22 (28.5 por ciento), 2 acompañados de hemorragia digestiva i 1 de invaginación que requirió intervención. La afectación articular se observa en 22 (28.5 por ciento). Hemos encontrado afectación renal en 17 (22 por ciento). De éstos, 3 han tenido síndrome nefrótico y a 2 se le ha realizado biopsia renal (2 nefropaties IgA con glomerulonefritis segmentaria y focal y 1 de éstos con 20 por ciento de semilunas). Han presentado rebrote 21 (29 por ciento). Han recibido tratamiento con corticoides 8 (11.2 por ciento). Conclusiones. 1. La afectación renal encontrada es similar a otras series publicadas (20-25 por ciento). 2. La posibilidad de una afectación renal, que ocasionalmente puede ser grave, obliga al seguimiento de los pacientes con PSH. 3. La determinación por tira reactiva de orina de proteinuria y/o hematuria es un buen instrumento para el cribado. (AU)

Púrpura de Henoch-Schönlein na criança e adolescente / Henoch-Schönlein purpura in childhood and adolescence

A púrpura de Henoch-Schönlein (PHS) é a vasculite mais frequente na infância. A manifestação clínica presente em todos os pacientes é a púrpura palpável não plaquetopênica, de localização predominante em membros inferiores e nádegas, podendo ocorrer em face, couro cabeludo, braços e raramente, tronco. Artralgia e/ou artrite ocorre em 60 a 84 por cento dos pacientes. O comprometimento gastrintestinal ocorre em 50 a 85 por cento dos casos e em 19 por cento dos pacientes é a apresentação inicial dos pacientes, geralmente nos primeiros três meses da doença. Doença renal grave ocorre em 1 a 4,5 por cento de todas as crianças com PHS e em 7,5 por cento daquelas com alterações renais persistentes. As lesões cutâneas resolvem-se naturalmente, na maioria das vezes, sem tratamento. As artralgias e artrites têm boa resposta ao paracetamol e/ou naproxeno. Os corticosteróides são indicados no comprometimento intestinal grave, orquite, hemorragia pulmonar e nefrites. Os casos graves particularmente a glomerulonefrite crescêntica, podem requerer terapia com gamaglobulina endovenosa, plasmaférese e/ou imunossupressores. As alterações cutâneas, articulares e gastrintestinais costumam resolver em um a dois meses. O prognóstico da PHS é bom, apesar de recorrências eventuais. A mortalidade na fase aguda está relacionada com complicações e insuficiência renal aguda. A longo prazo a morbimortalidade está relacionada com a insuficiência renal crônica