The dilemma of inherited dysfibrinogenemia during pregnancy.

Inherited dysfibrinogenemia is a rare disorder caused by mutations in the fibrinogen gene, described in approximately 400 families to date. We present the case of a 20-year-old woman at 7 weeks of pregnancy with a history of two first-trimester spontaneous abortions and a family history of thrombotic events. Her testing revealed evidence of dysfibrinogenemia, necessitating multidisciplinary management planning including Hematology, OB-GYN, Maternal-Fetal Medicine, Blood Bank Services and Anesthesia. Antenatal care included a combination of intravenous fibrinogen infusions to maintain fibrinogen levels above 100 mg/dl and anticoagulation with low molecular weight heparin. She had an uneventful full-term delivery and continued fibrinogen infusions and thromboprophylaxis for 6 weeks postpartum. The combination of fibrinogen infusions and anticoagulation maintained the balance between bleeding and clotting in our patient during pregnancy. We recommend a multidisciplinary team approach for the management of dysfibrinogenemia during pregnancy to provide successful pregnancy outcomes.

Successful management of a Jehovah's Witness with thrombotic thrombocytopenic purpura unwilling to be treated with therapeutic plasma exchange.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease treated successfully with plasma exchange. Jehovah's Witnesses whose religious beliefs preclude them from accepting plasma exchange may require alternative forms of therapy. We report a case of one such patient who presented with TTP, whom we successfully managed with vincristine and responded favorably without the need for plasma exchange.

Visual prognosis in patients with traumatic choroidal rupture.

BACKGROUND: There is a paucity of work in the literature examining the long-term visual prognosis of patients with choroidal ruptures. We performed a study to get a better assessment of long-term visual recovery in these patients as well as to identify prognostic indicators. METHODS: We reviewed the charts of 32 patients who experienced traumatic choroidal rupture following blunt ocular trauma. All charts contained International Classification of Diseases code 363.63 (choroidal rupture). For inclusion in the study, the chart had to contain detailed drawings or photographic evidence that could confirm the presence and location (foveal, juxtafoveal or extrafoveal) of the choroidal rupture. In addition, demographic data and visual acuity on follow-up examinations had to be present. The mechanism and location of the injury, initial and final visual acuity, associated ocular findings and length of follow-up were recorded. RESULTS: Of the 32 eyes 31 had indirect choroidal ruptures and I had a direct rupture. The mean final visual acuity values in the foveal, juxtafoveal and extrafoveal subgroups were 20/68, 20/35 and 20/60 respectively after a mean duration of follow-up of 4.5, 3.5 and 4.4 years respectively. There was no statistically significant difference in mean final visual acuity or mean length of follow-up between the three groups. The eight patients with multiple choroidal ruptures had a mean final vision of 20/31 after a mean follow-up period of 3.8 years. The 24 patients with a single choroidal rupture achieved a mean final vision of 20/47 over a mean duration of follow-up of 4.1 years. There was no difference in final vision or in length of follow-up between the two groups. The six patients under 15 years of age attained a mean final vision of 20/34 over a mean follow-up period of 4.5 years, whereas the adult group achieved a mean final vision of 20/44 over a mean follow-up period of 3.8 years. Again, there was no difference in final vision or in length of follow-up between the two groups. INTERPRETATION: Traumatic choroidal rupture involving the fovea has been thought to have a poor visual prognosis. Our findings show that eyes with foveal choroidal ruptures may regain good central vision over longer follow-up. Furthermore, multiple choroidal ruptures do not necessarily predict a poor visual outcome. Children with choroidal ruptures attained good final visual outcomes.

Platelet function testing to assess effectiveness of platelet transfusion therapy.

BACKGROUND: Posttransfusion corrected count increments (CCI) following administration of platelets is the standard method for assessing effectiveness of platelet transfusion therapy. However, improvement in platelet count following transfusion may not necessarily indicate improvement in platelet function or restoration of primary hemostatic capacity. To address this possibility, we investigated the effectiveness of platelet transfusion based on results of the Platelet Function Analyzer (PFA-100) and post-transfusion CCI. INVESTIGATION DESIGN AND METHODS: Platelet transfusion requests with different indications received at the blood bank were evaluated for inclusion in the investigation. Pre-transfusion, the following laboratory tests were performed: (1) PFA-100 assays (blood collected in 3.2% buffered sodium citrate) performed with CEPI and CADP test cartridges; (2) complete blood count (in EDTA) and platelet count; and (3) routine coagulation profile including PT, PTT, fibrinogen and D-Dimer. Only patients with normal coagulation profiles were included. The same set of tests were performed on a new blood sample collected 10-60 min post-transfusion. Chart review and clinical evaluation for response to platelet therapy were performed on each occasion of transfusion. RESULTS: Thirty-one patients, five of whom were transfused on more than one occasion were evaluated. Thirty-five transfusion incidents were included. Posttransfusion outcomes were divided into two groups--those that resulted in shortening (>40 s) or normalization of the closure time (Group A) and those that had no change or greater prolongation of the closure time (Group B) when compared to the pre-transfusion value. Seventeen and eighteen transfusion episodes were categorized as Groups A and B, respectively. In Group A with improved PFA testing, nine patients had bleeding as indication for transfusion and six of these had concomitant improvement in their clinical picture as confirmed by control of hemorrhage. In contrast in Group B with no improvement in PFA testing, seven patients had bleeding as indication for transfusion and none showed cessation of hemorrhagic symptoms. These findings were statistically significant (p=0.0114). Similar evaluation using the post-transfusion CCI showed no correlation to bleeding symptoms in these patients (p-=0.500). CONCLUSIONS: In this evaluation, platelet function testing using the PFA-100 provided a better indication of transfusion outcome than did the post-transfusion CCI. Using this approach, PFA-100 may be an effective aid for supporting platelet transfusion decisions and may further aid in improving management of the hospital blood bank platelet inventory.

Incidence of ocular injuries in motor vehicle crash victims with concomitant air bag deployment.

OBJECTIVE: To report the incidence of ocular and nonocular trauma in patients admitted to the Henry Ford Hospital via the emergency room since 1994 after a motor vehicle crash (MVC) with and without air bag deployment. DESIGN: A retrospective cohort study. METHODS: The Henry Ford Hospital Trauma Registry database was reviewed for patients involved in MVCs with and without air bag deployment since 1994. RESULTS: From 1994 to 1999, there were only seven air bag-related ocular injuries, representing 4.4% of all MVC-related ocular injuries. From 1997 to 1999, MVC-related ocular injuries with and without air bags represented 5.0% and 12.7%, respectively, of all MVC-related injuries. For that same period, the death rate and average Injury Severity Score for MVCs with air bag deployment were 3.4% and 10.75, compared with 8% and 14.5, respectively, for MVCs without air bag deployment. CONCLUSIONS: MVC-related ocular injuries associated with air bag deployment are rare, and the incidence of ocular injuries associated with MVCs was lower when air bags were deployed.

Hemostatic alterations associated with supraceliac aortic cross-clamping.

PURPOSE: The causative role of consumptive coagulopathy in the development of bleeding complications after supraceliac (SC) aortic cross-clamping (AXC) has been challenged by recent reports that ascribe this coagulopathy to primary fibrinolysis. This theory is made on the basis of evidence that tissue plasminogen activator (t-PA) antigen (Ag) levels increase after SC AXC. However, t-PA Ag levels reflect both active and inactive (bound to serum t-PA inhibitors) forms of serum t-PA, and elevations confirm the presence of fibrinolysis only in conjunction with an increase in t-PA activity. METHODS: To investigate the etiology of this coagulopathy, we submitted eight pigs to SC AXC and six pigs to infrarenal (IR) AXC for 30 minutes. Blood was drawn from the portal vein, the hepatic vein, and the carotid artery before AXC, just before unclamping, and 5, 30, and 60 minutes after unclamping. Prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen (FBG), platelets (PLT), thrombin-antithrombin complexes (TAT), t-PA Ag, t-PA activity, plasminogen activator inhibitor-1 (PAI-1), and alpha2-antiplasmin (AP) activities were measured. Statistical analysis was performed by using repeated measures analysis of variance and t tests RESULTS: The PT did not differ between the two groups at any point. After unclamping, in the SC group there was a drop in PLT levels (P =.005), a decrease in FBG levels (P <.001), and a trend toward PTT prolongation (P =.06) compared with baseline. In contrast, there were no changes in PTT, PLT levels, or FBG levels in the IR group. TAT, a serum marker of thrombin generation, increased with SC AXC (P =.04), remained elevated 5 minutes after unclamping (P =.08), and returned to normal 30 minutes after unclamping. In contrast, TAT levels did not change in the IR control group. In the SC AXC group, the TAT levels did not differ between the three test sites at any time. SC AXC was associated with an increase in t-PA Ag just before unclamping (P <.001) and 5 minutes after unclamping (P =.002), but IR AXC was not. t-PA activity levels decreased in both experimental groups 30 and 60 minutes after unclamping. Levels of alpha2-AP activity decreased to a similar degree in both groups after unclamping when compared with baseline CONCLUSION: Thirty minutes of SC AXC results in intravascular thrombosis that cannot be localized to the ischemic visceral circulation. This intravascular thrombosis is associated with consumption of clotting factors. Thirty minutes of SC AXC causes an activation of fibrinolytic pathways that does not result in a hyperfibrinolytic state. An increase in t-PA Ag without a rise in t-PA activity does not represent true fibrinolysis, but rather an increase in the bound, inactive forms of serum t-PA. Both IR and SC AXC result in decreased fibrinolytic activity ("fibrinolytic shutdown") after release of the aortic clamp.