Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases.

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.

Imperforate hymen with hematocolpos.

Presented are two cases of imperforate hymen with hematocolpos seen in a pediatric emergency department (ED) during a seven-month period. The first case presented with abdominal pain, urinary obstruction, and constipation on initial visit. The diagnosis was not made on the initial presentation. The patient was seen a second time, and final diagnosis was not made until a third visit to the pediatric outpatient clinic. The second case presented with syncope and bilateral lower abdominal pain. Ultrasound and subsequent surgery confirmed the physical findings in the ED of imperforate hymen and hematocolpos. Both patients underwent hymenectomy, and they have experienced no further symptoms.