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Abstract Objective The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. Methods Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. Results Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. Conclusions SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.
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OBJECTIVE: The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. METHODS: Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. RESULTS: Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. CONCLUSIONS: SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.
Assuntos
Vasculite por IgA , Sindecana-1 , Biomarcadores , Criança , Estudos Transversais , Humanos , Imunoglobulina ARESUMO
Abstract Introduction Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. Methods Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated. Results Of 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040). Conclusion Cyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.
Resumo Introdução A nefrite da púrpura de Henoch-Schönlein (NPHS) é definida como púrpura de Henoch-Schönlein com envolvimento renal, incluindo hematúria e/ou proteinúria. O objetivo deste estudo foi avaliar os dados de pacientes com NPHS que foram submetidos à biópsia renal e comparar os principais parâmetros clínicos e laboratoriais que podem afetar os achados da biópsia renal, os protocolos de tratamento e o desfecho de curto e longo prazo desses pacientes. Métodos Foram avaliadas retrospectivamente biópsias realizadas em 72 pacientes com NPHS entre Janeiro de 2007 e Janeiro de 2017. Eles foram divididos em dois grupos de acordo com a classificação de biópsia renal do Estudo Internacional de Doenças Renais em Crianças. O desfecho renal, parâmetros clínicos e laboratoriais, protocolos de tratamento e desfechos foram comparados entre os grupos. Foi avaliado o acompanhamento de pacientes de curto e longo prazo. Resultados De 72 pacientes, 47 eram homens (65,3%) e 44 (61,1%) tinham ≤10 anos de idade. A razão neutrófilo-linfócito foi encontrada mais alta em pacientes com envolvimento escrotal (p=0,042). O desfecho desfavorável de curto prazo foi significativamente maior em pacientes com envolvimento escrotal (p=0,038). Constatou-se que pacientes com hipertensão e diminuição da depuração de creatinina apresentaram desfechos mais desfavoráveis no acompanhamento de longo prazo (p=0,029, p=0,040). Conclusão A ciclosporina-A e a ciclofosfamida podem ser eficazes em pacientes com NPHS não responsivos a esteroides. Pacientes com envolvimento escrotal, diminuição da depuração de creatinina e hipertensão devem ser monitorados de perto para sequelas de NPHS.
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Abstract Introduction: Henoch Schönlein purpura (HSP) is the most common type of vasculitis in childhood. HSP affects small blood vessels, and it rarely leads to serious complications such as bullous small vessel vasculitis, as it occurred in the case presented here. Case presentation: 5-year-old male who was brought to a primary healthcare center due to having arthralgia and purple skin lesions on his lower limbs. After the patient was diagnosed with HSP, he developed bullous lesions, so he was hospitalized and analgesic and topical management was started. During his hospital stay, the patient's renal function was monitored, and since he did not experience other complications, he was discharged. Conclusion: The available literature on HSP suggests that its cutaneous bullous manifestation rarely occurs in pediatric population and that, unlike normal HSP cases, it is not always associated with renal and/or gastrointestinal involvement. However, regardless of the dermatological severity of this type of vasculitis, the function of the gastrointestinal and renal systems must be always monitored in these patients.
Resumen Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación. Presentación del caso. Paciente masculino de cinco años que fue traído a un centro de atención primaria con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta. Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.
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Resumen La púrpura de Shönlein-Henoch es una vasculitis de la infancia caracterizada por una púrpura palpable no trombocitopénica y manifestaciones sistémicas, acompañadas de dolor abdominal, sangrado gastrointestinal, glomerulonefritis, artritis y artralgias. Está relacionada con patologías desencadenantes como las infecciones y las picaduras de insectos. Se presenta el caso de un niño de 4 años de edad, que presentaba cuadros alérgicos de rinitis y urticaria. Se le había diagnosticado una trombocitopenia inmune primaria de evolución crónica. A pesar del empleo de diferentes alternativas de tratamiento, recurrió con trombocitopenia muy severa, asociada a cuadro de púrpura y lesiones maculo-papulares y urticarianas. Los complementarios realizados, a excepción del conteo de plaquetas, estuvieron en rangos normales. La evolución fue satisfactoria en un periodo de tres semanas. Se presenta el caso porque es una forma atípica de esta enfermedad.
Abstract Shönlein-Henoch purpura is a common vasculatis in childhood, characterized by a non-trombocytopenic palpable purpura and systemic manifestations, accompanied by abdominal pain, gastro-intestinal bleeding, glomerulonephritis, arthritis and arthralgias. It is the most common of diseases due to disturbances in the vascular component in children. It is related to triggering pathologies as infections and insect bites. A case of a 4 year old boy is presented who presented allergic episodes of rhinitis and urticaria. He had been diagnosed with an chronic primary inmmune thrombocytopenia. In spite of the different treatment approaches, the patient had a severe thrombocytopenia, associated to purpura and macolu-papular lesions and uticaria. Lab tests, with the exception of platelet count, were within the normal limits. The progress was satisfactory in a three week period.
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Abstract: The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.
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Humanos , Masculino , Adulto Jovem , Vasculite por IgA/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/análise , Técnica Direta de Fluorescência para AnticorpoRESUMO
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.
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Humanos , Feminino , Criança , Nefropatias/etiologia , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Glucocorticoides/uso terapêutico , Prednisona/uso terapêutico , Proteinúria/etiologiaRESUMO
Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children. In the absence of significant renal disease it has an excellent prognosis. In the case described, HSP initially presented together with orchitis. This infrequent event required the exclusion of testicular torsion.
A Púrpura de Henoch-Schönlein é a vasculite mais comum na idade pediátrica, tendo um prognóstico excelente na ausência de doença renal significativa. No caso descrito, a apresentação inicial cursou com orquite, o que não é frequente, obrigando a exclusão de torção testicular.
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Pré-Escolar , Humanos , Masculino , Orquite/diagnóstico , Vasculite por IgA/diagnóstico , PrognósticoRESUMO
O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.
Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.
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Humanos , Lactente , Masculino , Edema/patologia , Hemorragia/patologia , Vasculite por IgA/patologia , Vasculite Leucocitoclástica Cutânea/patologia , Doença Aguda , Diagnóstico Diferencial , Vasculite Leucocitoclástica Cutânea/etiologiaRESUMO
PURPOSE: To report a 16-year long-term follow-up of a patient with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome). A 21-year old male was seen in 1994 with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome), first in the left eye, and later in the right eye. He was treated with retinal photocoagulation in areas of retinal ischemia and oral steroids, followed by sequential annual fundus examination and photography for 16 years. Vision improved to 20/25 in both eyes after retinal ischemic areas photocoagulation and oral steroids, and his vision has been maintained for 16 years. Photocoagulation of retinal ischemia and oral steroids are effective for the treatment of acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome).
Relato de caso com acompanhamento por 16 anos de um paciente com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz). Um paciente de 21 anos de idade foi diagnosticado em 1994 com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz), primeiro no olho esquerdo e depois no olho direito. Foi tratado com fotocoagulação retiniana nas áreas retinianas isquêmicas e corticosteroide oral e seguido por exames complementares da retina por 16 anos. A visão melhorou para 20/25 em ambos os olhos após a fotocoagulação retiniana nas áreas isquêmicas da retina e corticosteroide oral permanecendo assim até o momento por 16 anos. A fotocoagulação retiniana nas áreas isquêmicas e o uso de corticosteróide oral são tratamentos efetivos para a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz).
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Adulto , Humanos , Masculino , Adulto Jovem , Hemorragia Retiniana/diagnóstico , Vasculite Retiniana/diagnóstico , Doença Aguda , Aciclovir/uso terapêutico , Angiofluoresceinografia , Seguimentos , Fotocoagulação/métodos , Prednisona/uso terapêutico , Hemorragia Retiniana/terapia , Vasculite Retiniana/terapia , Síndrome , Esteroides/uso terapêuticoRESUMO
La púrpura de Schönlein-Henoch es una vasculitis leucocitoclástica, la más común en pediatría, que afecta a pequeños vasos, su etología no es conocida, es una enfermedad multisistémica que afecta piel, articulaciones, tracto gastrointestinal y riñones. Se presenta caso clínico de escolar de 11 años de sexo femenino la cual cursó con púrpuras palpables en miembro inferiores asociado a dolor abdominal al ingreso de su internación como antecedente dos semanas antes presentó infección en tracto respiratorio alto que evoluciónó favorablemente. En el tratamiento de la púrpura se le administró corticoide con lo cual mejoró el cuadro clínico abdominal favorablemente ya que en un momento se pensó que éste dolor abdominal podría complicarse con proceso quirúrgico como invaginación intestinal.
Schonlein-Henoch Purpura is a leukocytoclastic vasculitis, most common in children which affects small vessels; its etiology remains unknown, it is a multisystemical disease that affects skin, joints, gastrointestinal tract and kidneys. The clinical case is presented in a 11 years old female children, with a palpable purpura associated to abdominal pain, in the moment of the hospitalization, having presented two weeks before a history of upper respiratory tract infection that evolved favorably; with a corticosteroid administration, the Purpura this improved clinically and the abdominal pain decreased, instead of increasing, in which case it was thought that it may be complicated with a surgical process as an intestinal instussusception.