"C" trigonocephaly syndrome: clinical variability and possibility of surgical treatment.

We report on 3 new cases of C trigonocephaly syndrome. In addition to the findings characteristic of this condition, one of the patients also had a large omphalocele. This patient was referred from a suburban hospital with a diagnosis of Down syndrome, stressing the fact that C syndrome is still under-recognized and underdiagnosed. Another patient was diagnosed at birth and immediately submitted to craniosynostectomy. A second operation was performed 7 months later resulting in normal brain growth and close to normal psychomotor development at 3 years, in contrast to the third patient, who was not treated surgically and was severely retarded at 4 years.

The fibrinolytic system in the newborn: role of histidine-rich glycoprotein.

The fibrinolytic system controls fibrin deposition and its clearance. The efficacy of this system can be evaluated by plasminogen concentration determinations and by the behavior of factors such as histidine-rich glycoprotein (HRG) which controls plasminogen activation and alpha 2-antiplasmin which controls plasmin activity. Circulating plasminogen levels are decreased in the neonatal period. We studied factors affecting fibrinolysis in neonates and observed that an important reduction in HRG accompanied the reduced circulating plasminogen levels, with the result that 85% of circulating plasminogen was not bound to HRG and was thus free for binding to fibrin and for activation to plasmin. This condition is consistent with the increased fibrinolytic activity secondary to the "clotting activation' observed in the neonatal period particularly on the 1st day of life.