CONGENITAL AFIBRINOGENAEMIA REPORT OF A CASE WITH A REVIEW OF THE LITERATURE

1. A case of congenital afibrinogenaemia, which is a rare causeof the haemorrhagic diathesis, is reported in a boy aged 11years.
2. Six other cases which have been recorded in the literaturearereviewed.
3. The principal clinical features of congenitalafibrinogenaemiaare its hereditary character, a high incidenceof consanguinityin the parents, the susceptibility of bothsexes, a total absenceof fibrinogen in the blood, completeincoagulability of theblood, a bleeding time which is usuallyprolonged, a great reductionof capillary resistance, a lowblood sedimentation rate, andintermittent thrombocytopenia.
4. Treatment of the severe haemorrhage is by intravenous transfusionwith whole blood or non-processed plasma.
5. Absence of fibrinogenis regarded as the principal cause ofthe haemorrhagic diathesisin congenital afibrinogenaemia, butdiminished capillary resistancemay be a. contributory factor.
6. Four cases of congenital hypofibrinogenaemiawhich have beenrecorded in the literature are also reviewed.
7. The principal distinctions between congenital afibrinogenaemiaand congenital hypofibrinogenaemia are discussed. The clottingtime shows the greatest divergence; no clotting occurs in afibrinogenaemia,whereas it is normal in hypofibrinogenaemia.
8. The aetiologyof both the congenital and acquired forms of afibrinogenaemiaand of hypofibrinogenaemia is discussed.