Labor and delivery in a patient with hemophilia B.

Hemophilia B is a rare X-linked disorder that may cause dramatic bleeding. Women account for only 3.2% of those clinically affected. The X-linked inheritance frequently delays the diagnosis in women and may expose the patient to an increased risk of adverse events. There is limited experience with these patients during labor and delivery. A 28-year-old primiparous woman with hemophilia B (bleeding phenotype) delivered a male infant by an unplanned cesarean delivery under general anesthesia following treatment with factor IX and normalization of her coagulation parameters, guided by thromboelastography. Postpartum vaginal bleeding required transfusion of two units of packed red blood cells. Factor IX supplementation continued for one week. Once diagnosed with hemophilia B, a multidisciplinary approach and advanced antenatal planning can increase the likelihood of a safe delivery. Neuraxial approaches and cesarean delivery are recommended only after normalization of the coagulation profile. The male fetus of a hemophilia A or B patient requires special attention. Operative vaginal delivery and invasive fetal monitoring should be avoided. Thromboelastography is an excellent technique to assess parturients with bleeding disorders or peripartum hemorrhage and may be underused.

GM-CSF versus G-CSF: engraftment characteristics, resource utilization, and cost following autologous PBSC transplantation.

BACKGROUND: G-CSF and GM-CSF have both been shown to decrease the time to hematopoietic recovery when administered after autologous BM or peripheral stem cell re-infusion. However, few studies have compared G-CSF and GM-CSF to determine which is the preferred myeloid growth factor. METHODS: This study compares a prospectively accrued cohort of 22 patients receiving GM-CSF with a historical cohort of patients who received G-CSF commencing Day + 6 after autologous PBSC transplantation. Patients were matched based on disease type and stage, CD34(+) cell dose/kg, conditioning regimen, and prior treatment. Time to myeloid engraftment, growth factor utilization, antibiotic utilization, fever incidence, and cost were compared. RESULTS: The median time to neutrophil and platelet engraftment was similar in the two groups (ANC > 500 /mm(3), GM-CSF 12 versus G-CSF 11, P = 0.69). There was a trend towards more days of temperature > 38.0 masculine C (six versus three, P = 0.05) and febrile neutropenia (three versus two, P = 0.06) in the GM-CSF arm. There was a trend towards increased use of i.v. antibiotics in the GM-CSF cohort (7.6 days versus 5.5 days, P = 0.06). More chest X-rays (1.5 versus 1.0, P = 0.03) were ordered, and more blood cultures drawn (4.2 versus 2.7, P = 0.05) as part of fever evaluation in the group treated with GM-CSF. Resource utilization based on actual wholesale pricing (AWP) favored the G-CSF cohort. Applying a sensitivity analysis, GM-CSF became cost-effective when priced below $94 per 250 micro g, despite greater resource utilization. DISCUSSION: This study suggests that engraftment characteristics are similar with GM-CSF and G-CSF following PBSC transplantation. Resource utilization for fever treatment and evaluation may be greater with GM-CSF. Determination of which agent is more cost-effective depends on institutional acquisition costs.

Cesarean section combined with splenectomy in a parturient with immune thrombocytopenic purpura.

The case of a 25-year-old parturient with immune thrombocytopenic purpura is discussed. The patient was first seen at 20 weeks' gestation because of a history of thrombocytopenia after delivery of a child six years previously. The patient was not compliant with prednisone therapy or return follow-up visits; a combined cesarean section with splenectomy was planned for this patient. The report discusses the multidisciplinary management of this patient during her pregnancy and delivery.

Autoantibody formation in the alloimmunized red blood cell recipient: clinical and laboratory implications.

Alloimmunization to erythrocyte antigens is a well-characterized complication in heavily transfused patients. Less well recognized, however, is the frequency of autoantibody formation in these previously alloimmunized patients. The autoantibodies are heterogeneous and of variable clinical significance. We describe the clinical history, laboratory evaluation, diagnosis, and treatment in 4 patients who developed autoantibodies in temporal association with alloantibody formation. In one case, the autoantibody found on routine screening had no clinical significance. In another case, the autoantibody made accurate blood typing and subsequent transfusion exceedingly difficult. Two patients experienced hemolysis as a consequence of the autoantibody. The management of both patients included supportive measures, while one patient required glucocorticosteroids and red blood cell transfusion. We review the published literature concerning autoimmunization in the transfused alloimmunized host. The spectrum of clinical consequences is important for the general practitioner to recognize, as these complications may occur during routine blood transfusions.

Management of a patient with a mechanical aortic valve and antibodies to both thrombin and factor V after repeat exposure to fibrin sealant.

We describe a patient who developed a markedly prolonged PT, PTT, and thrombin time 13 days after repeat exposure to fibrin sealant during coronary artery bypass grafting and aortic valve replacement. Evaluation revealed an inhibitor to bovine thrombin that cross-reacted with human thrombin. In addition an inhibitor to human coagulation factor V was identified. Despite coagulation abnormalities there was no evidence of bleeding. Nevertheless, effective anticoagulation was required to minimize the thrombotic complications associated with the patient's prosthetic valve. We elected to take a conservative approach and not utilize pharmacologic anticoagulation until there was diminution in the effect of the acquired inhibitors. We report on our patient's course and review the available literature addressing the management of patients demonstrating inhibitors to blood coagulation factors after repeat exposure to fibrin sealants.

Inclusive education in Finland: present and future perspectives.

The movement to integrate special education students into normal school classes started to develop in Finland in the 1960s. At the same time, the number of students labeled "special" in the Finnish comprehensive school system exploded from 2% to 17% of all school children. Presently, 84% of all special education placements are part-time placements. Special schools and special classes comprise 15% of all special education placements, while full inclusion is only 1% of all special education placements. Some factors affecting the current integration of special students and the development of integration are discussed.

Afferent modulation of warmth sensation and heat pain in the human hand.

The hands of 14 normal humans were used to determine the somatotopic organization of the modulation of warmth sensation and heat pain by different forms of cutaneous stimuli. Test stimuli were 5-sec heat pulses ranging from 36 degrees to 51 degrees C, delivered to the fingerpads of digits 1, 2, 4, and 5 with a contact thermode. Conditioning stimuli (15 sec) bracketed the test stimuli and included vibration, noxious and innocuous heat, cold, and electrical pulses delivered to the fingerpads of digits that were adjacent or nonadjacent to the tested digits. Noxious (48 degrees +/- 1.3 degrees C), but not innocuous (43 degrees C), heat stimuli increased the perceived magnitude estimation of innocuous test stimuli (36-43 degrees C) by 20-37% when delivered to adjacent, but not to nonadjacent, digits. No other conditioning stimuli had any effect on the intensity of warmth perception. In contrast, both noxious and innocuous heat or electrical conditioning reduced the magnitude estimation of noxious (50-51 degrees C), but not innocuous, test pulses by 12-22% when delivered to adjacent digits. Conditioning of nonadjacent digits was significantly less effective. The analgesic effects of noxious and innocuous conditioning were approximately equal. Vibratory (120 Hz, 3.5 microns) and cold (15 degrees C) conditioning stimuli were ineffective. The results are consistent with a dermatomal somatotopic organization of tactile and heat modulatory influences on warmth sensation and heat pain. The results further suggest that the neural mechanism subserving warmth mediate a negative feedback influence on heat pain intensity.