RESUMO
BACKGROUND: Lymphomatoid granulomatosis (LG) is an Epstein-Barr virus-associated, multisystemic disease that combines a granulomatous inflammatory process with lymphoproliferative potential. It is a rare disorder with a variable clinical presentation ranging from an indolent process to an aggressive B-cell lymphoma. Outcome is unpredictable, and a standard treatment has not yet been established. Cases treated with rituximab, an anti-CD20 monoclonal antibody, have been reported with variable results. OBSERVATION: We report on 2 children with LG treated with rituximab and review the literature. The first patient had good response but the second did not. CONCLUSIONS: Rituximab is a treatment option for LG.
Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Antineoplásicos/uso terapêutico , Granulomatose Linfomatoide/tratamento farmacológico , Pré-Escolar , Humanos , Masculino , RituximabRESUMO
Antiepileptic hypersensitivity syndrome (AHS) is a potentially life-threatening syndrome in pediatric cases. It is associated with aromatic anticonvulsivant drugs and others antibiotics with or without associated reactivation of virus. The pathogenesis of the process is not clear. Clinical feature of this syndrome include cutaneous reactions, fever and lymphadenopathies, but different organs can be involved. In laboratory analyses we can find leucocytosis, eosinophilia, and increase in transaminase levels. Significant elevation of procalcitonin (PCT) has been described in two adults patients. This study we report two cases of a 14-year-old male and a 13-year-old female with a AHS and a high level of the PCT. These are the first cases described in pediatric age. Establishing a diagnosis of AHS is important to avoid the use of the involved drug. The treatment is based on the withdrawal of the drugs that are supposed and if there is no improvement or a deterioration of the patient, the use of systemic corticosteroids is often useful.
Assuntos
Anticonvulsivantes/efeitos adversos , Calcitonina/sangue , Hipersensibilidade a Drogas/diagnóstico , Precursores de Proteínas/sangue , Adolescente , Peptídeo Relacionado com Gene de Calcitonina , Carbamazepina/efeitos adversos , Toxidermias , Hipersensibilidade a Drogas/sangue , Hipersensibilidade a Drogas/complicações , Epilepsia do Lobo Frontal/complicações , Epilepsia do Lobo Frontal/tratamento farmacológico , Infecções por Vírus Epstein-Barr/complicações , Feminino , Humanos , Masculino , Pentobarbital/efeitos adversos , Fenitoína/efeitos adversos , Transaminases/sangueRESUMO
OBJECTIVE AND IMPORTANCE: Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician. CLINICAL PRESENTATION: We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage. INTERVENTION: Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased. CONCLUSION: Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.
