Search

SummaryA new case of dysfibrinogenemia is described, the proband had low fibrinogen level and a moderate hyperfibrinolysis. Attention is drawn to the florid hemorrhagic picture and its early presentation.The family history showed a moderate hyperfibrinolysis.It appeared that the cause of the dysfibrinogenemia was due to a deficient polymerization of the α chains of the fibrinogen.It is not yet known if this is caused by a defective liberation of fibrinopeptides. Due to the peculiar characteristic of this case and in accordance with the international norms, we propose to call this dysfibrinogenemia “Fibrinogen Valencia.”

The renal vein thrombosis represents an etiologic and therapeutic problem in neonatal pathology. In our milieu RVT is more frequent than is reported in current bibliography. This high incidence is accompanied by a high mortality and by the development of chronic renal failure among survivors.This fact led us to try a thrombolytic treatment that could be effective with respect to life and renal function.In our study we describe seven newborn patients with radiologically checked RVT. All cases were secondary to hypernatremic dehydration syndrome due to hyperconcentrated feeding. Five patients were submitted to thrombolytic treatment with UK, one patient was treated with heparin and one patient died before any treatment could be tried.Among the five patients treated with UK, four showed a complete recanalization of the RVT, with recovery of their renal function without sequelae. One of the patients, in whom an intracraneal haemorrhage was clinically suspected, died during treatment.The dosage of UK during treatment was 1000 U/Kg/hour.The duration of treatment was individualized according to renal evolution.The description of treated cases will be done individually.