Afibrinogenemia

Abstract

These and subsequent cases have proved to us that several definite facts are known and that certain standard procedures are warranted in all hospitals for the proper diagnosis and handling of such a condition.As defibrination occurs and increases, the platelets then are reduced by lytic action. Defibrination may occur without lysis. Prolonged labor, infection, and other states will produce lysis. Afibrinogenemia and lower nephron nephrosis, while two separate and distinct entities, appear to have at times a common pathogenesis. Page21 states that the renal-cortical syndrome is the cause of one-third of all the deaths. Decreased fibrinogen and lytic activity together are serious conditions. It is not actually a bilateral cortical syndrome but rather it is an acute tubular necrosis and caused mainly by shock.In 2 of our 3 cases there was pre-eclampsia along with the abruptio placentae and afibrinogenemia. The autopsy findings in the kidney of the patient who died showed extensive epithelial damage in the tubules, which is in agreement with Page's findings. The autopsy on our first patient showed little or no hemorrhage into the uterus. This substantiates the conclusion of observers in this field that hysterectomy in such cases os not indicated. Removal of the uterus simply shifts the site of bleeding to other areas. Two of these patients had transfusion reactions. One of them, who did not have toxemia, showed blood and casts in the postoperative urine. These patients have had a derangement of the clotting mechanism of the blood; it has been corrected and the blood volume replaced. Apparently they are more vulnerable to anaphylactoid reactions than usual. It is therefore suggested that if the patient is doing well with a hemoglobin of 8 Gm. she should be given iron and the hemoglobin built up slowly.A fibrinogen index should be obtained routinely in all cases of premature separation of the placenta. It should be remembered that afibrinogenemia may also occur as a result of amniotic fluid infusion and in instances of long-standing fetal death. The easiest, best, and most used test is the clot retraction time determination. This should be followed by a quantitative test. Ten grams of fibrinogen should be maintained in the hospital at all times. Afibrinogenemia should be corrected preoperatively and blood volume replaced with fresh blood. Usually 1 Gm. of fibrinogen is needed for every pint of blood (averaging approximately 5 to 6 Gm. in treatment). If the case is a missed abortion and the fetus is still present, giving fibronogen is a waste of the fibrinogen. Some authors have recently been using Apresoline because it relieves spasm of the afferent arterioles and does not affect hypotension already present. They even advocate giving it as routine medication in all acute cases. Blutene is reported as having good antilytic action. It stops lysis. But remember it is of no value in preventing defibrination.Patients with premature separation of the placenta should be promptly delivered by the route indicated by the usual obstetric criteria. The use of Pitocin to stimulate labor in such instances may well be questioned if prompt and easy delivery is not anticipated. Its use may precipitate or increase the severity of afibrinogenemia or lower nephron nephrosis.