ResumoObjetivos: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schönlein.Métodos: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schönlein 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%). A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95% 1,18-2,21), sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95% 1,10-2,26) e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95% 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% 1,234-5,452). Conclusões Results: Evidence of nephritis was detected in 70 patients (49.3%).The univariate analysis revealed that severe abdominal pain (p = 0.0049; OR = 1.6; 95%CI 1.18-2.21), gastrointestinal bleeding (p = 0.004; OR = 1.6; 95%CI 1.10-2.26) and corticosteroid use (p = 0.0012; OR = 1.7; 95%CI 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%CI 1.234-5.452). Conclusions:Severe abdominal pain was a significant predictor of nephritis in Henoch-Schönlein purpura. Consequently, pediatric patients exhibiting this clinical manifestation should be rigorously monitored, due to the increased risk of renal involvement. J Pediatr (Rio J). 2007;83(3):259-266:Henoch-Schönlein purpura, nephritis, children, kidney disease, prognosis.
Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.
Artigo submetido em 12.03.03, aceito em 28.05.03. ResumoObjetivo: Relatar três casos de osteomielite crônica multifocal recorrente de mandíbula. A osteomielite crônica multifocal recorrente é uma doença inflamatória, com acometimento de um ou mais ossos, sem patógenos isolados nas áreas afetadas, sendo o envolvimento da mandíbula raramente descrito na literatura.Descrição: Caso 1 -paciente feminina, com 13 anos, após tratamento dentário evoluiu com dor mandibular e febre. A paciente foi tratada com antibióticos por osteomielite, evoluindo com aumento progressivo da mandíbula e pustulose palmoplantar. A cintilografia óssea mostrou hipercaptação difusa da mandíbula. A redução da tumoração mandibular foi evidenciada com a indometacina e sessões de câmara hiperbárica. Caso 2 -paciente feminina, com 9 anos, apresentou dor e edema em região de mandíbula direita recorrente por três anos. O diagnóstico de osteomielite foi realizado e introduzido amoxicilina. Após três meses, a tomografia computadorizada mostrou osteólise mandibular difusa. Indometacina e câmara hiperbárica foram introduzidas, porém a paciente apresentou recidiva e foi tratada com prednisona, rofecoxib e metotrexato. Caso 3 -paciente masculino, com 10 anos, apresentou pustulose palmoplantar e aumento difuso da mandíbula recorrente. A tomografia computadorizada de mandíbula evidenciou osteólise mandibular, e a cintilografia óssea, hipercaptação difusa. O paciente foi tratado com prednisona. O rofecoxib foi substituído após duas recidivas.Comentários: A osteomielite crônica multifocal recorrente da mandíbula pode apresentar um curso doloroso prolongado, com intervalos de atividade e remissão do processo inflamatório. O seu reconhecimento é importante para evitar uma antibioticoterapia prolongada e procedimentos invasivos desnecessários. J Pediatr (Rio J) 2003;79(5):467-70:Osteomielite crônica, mandíbula. AbstractObjective: To report three cases of chronic recurrent multifocal osteomyelitis of the mandible, an inflammatory disease affecting one or more bones with absence of isolated microorganisms in affected areas. Description:The first case is a 13 year-old female presenting with pain and fever after dental treatment. The patient received antibiotic treatment for osteomyelitis, but developed progressive enlargement of the mandible and palmoplantar pustulosis. Bone scintigraphy showed intense and diffuse uptake in the mandible. The swelling decreased after indomethacin and hyperbaric oxygen therapy. Case 2 is a 9 year-old female patient with recurrent pain and edema of the right mandible for three years. The diagnosis of osteomyelitis was established and amoxacillin introduced. After three months, tomography showed diffuse mandible osteolysis. Indomethacin and hyperbaric oxygen therapy were introduced, however the patient presented a relapse and was treated with prednisone, rofecoxib and methotrexate. Patient 3, a 10 year-old male, had palmoplantar pustulosis and recurrent enlargement of the mandible. Tomography showed diffuse mandible osteolysis and scint...
Chronic recurrent multifocal osteomyelitis of the mandible is often associated with prolonged pain periods and periods of activity and remission of the inflammatory process. Its recognition is important to prevent the patient from being submitted to prolonged antibiotic therapy and unnecessary invasive procedures.
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