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Vasculite por IgA , Nefrite , Humanos , Vasculite por IgA/diagnóstico , Imunoglobulina A , PrognósticoRESUMO
The prognosis of IgA vasculitis (also known as Henoch-Schönlein purpura) is determined by renal or other organ involvement. We conducted a retrospective study to identify the initial features of 106 children with IgA vasculitis and their association with unfavorable outcomes. Location of purpura above the waist and an altered urinalysis at diagnosis predicted a more aggressive course of disease.
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Vasculite por IgA , Criança , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Rim , Prognóstico , Estudos RetrospectivosRESUMO
INTRODUCTION: there are few case reports available that describe compartment syndrome as a complication of Henoch-Schönlein purpura. CASE REPORT: we report the case of a 17-year-old patient with bilateral compartment syndrome of the foot as an atypical presentation of Henoch-Schönlein purpura. A case like this has not been reported before. CONCLUSION: although the patient had an extremely rare clinical presentation, the viability and functionality of the limbs was preserved even after six months of follow-up thanks to an early diagnosis and surgical treatment.
INTRODUCCIÓN: en la literatura existen escasos reportes de caso del desarrollo de síndrome compartimental como una potencial complicación de la púrpura de Henoch-Schönlein. CASO CLÍNICO: se presenta el caso clínico de una paciente de 17 años con un cuadro de síndrome compartimental bilateral en pies como presentación atípica de la púrpura de Henoch-Schönlein, nunca antes descrita en la literatura. CONCLUSIÓN: con una rápida sospecha diagnóstica y un tratamiento quirúrgico con fasciotomías, se consiguió preservar la viabilidad de las extremidades y su funcionalidad a los seis meses de seguimiento, a pesar de tratarse de una presentación sumamente atípica de la patología en cuestión.
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Síndromes Compartimentais , Vasculite por IgA , Humanos , Adolescente , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Síndromes Compartimentais/diagnóstico , Síndromes Compartimentais/etiologia , PéRESUMO
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.
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Vasculite por IgA , Nefrite , Biópsia , Criança , Humanos , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Rim/patologia , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. CASE REPORT: A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. CONCLUSIONS: We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.
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Glomerulonefrite por IGA , Glomerulonefrite , Vasculite por IgA , Psoríase , Adulto , Criança , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Imunoglobulina A , Masculino , Psoríase/complicaçõesRESUMO
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.