RESUMO
Congenital plasminogen deficiency is a rare autosomal recessive disorder, characterized by chronic mucosal membranous lesions. Although the most common clinical manifestation is eye involvement as ligneous conjunctivitis, extra-ocular lesions affecting other mucosal surfaces indicates a systemic disease. In this report we describe two cases with atypical extra-ocular involvement that includes pericarditis and recurrent hematocolpos, and one with paradoxical correlation between ocular lesions and plasminogen levels. In ligneous conjunctivitis, although different treatment strategies have been tried with mild success, the only effective therapy is topical or systemic plasminogen concentrates that are not commercially available. Unfortunately there is not either effective management for cases with multisystemic disease. Hence, treatment for plasminogen deficiency is still a challenge and the variability of the clinical spectrum in this pathology makes necessary a multidisciplinary approach.
Assuntos
Transtornos Herdados da Coagulação Sanguínea , Plasminogênio/administração & dosagem , Plasminogênio/deficiência , Transtornos Herdados da Coagulação Sanguínea/sangue , Transtornos Herdados da Coagulação Sanguínea/tratamento farmacológico , Transtornos Herdados da Coagulação Sanguínea/genética , Transtornos Herdados da Coagulação Sanguínea/patologia , Pré-Escolar , Conjuntivite/sangue , Conjuntivite/tratamento farmacológico , Conjuntivite/genética , Conjuntivite/patologia , Feminino , Hematocolpia/sangue , Hematocolpia/tratamento farmacológico , Hematocolpia/genética , Hematocolpia/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Pericardite/sangue , Pericardite/tratamento farmacológico , Pericardite/genética , Pericardite/patologiaRESUMO
The prevalence of nalidixic acid-resistant Salmonella Typhi (NARST) infection is increasing worldwide. We are reporting an unusual case of infected haematocolpos presenting as urinary obstruction in a patient with fever of unknown origin (FUO). This case report highlights the importance of quinolone-resistant typhoid fever in the differential diagnosis of any acute febrile illness in countries, like India, where Salmonella infection is endemic.