Pregnancy in women with Hereditary Angioedema due to C1-inhibitor deficiency: Results from the ITACA cohort study on outcome of mothers and children with in utero exposure to plasma-derived C1-inhibitor.

Background: In women with Hereditary Angioedema (HAE) due to C1-inhibitor (C1INH) deficiency (C1INH-HAE), pregnancy counseling and treatment can be challenging. Despite the evidence of the immediate favorable outcome and safety of plasma-derived (pd)C1INH concentrate, there are no data regarding any difference among women who underwent or not pdC1INH during pregnancy or on children with in utero exposure to pdC1INH. The present interview study aimed at analyzing outcome of C1INH-HAE mothers and children according to pdC1INH-exposure during pregnancies. Methods: C1INH-HAE women who experienced at least 1 pregnancy were included from seven centers of the Italian Network for Hereditary and Acquired Angioedema (ITACA). The interview study retrospectively analyzed pregnancies who underwent (group 1) or not (group 2) pdC1INH. The overall goals of the study included immediate and long-term outcomes, in terms of outcomes in the time interval between pregnancy and survey. Results: A total of 168 pregnancies from 87 included women were analyzed. At term delivery (>37 gestation-week, GW) has been registered in 73.8% of cases, while spontaneous abortion (SA) occurred in 14.2% of cases with a mean GW 7 ± 2. The group 1 including pdC1INH-treated pregnancies comprised a third of the cohort (51/168, time interval 1.5 ± 10.4 yrs), while the group 2 represented 69.6% (117/168, time interval 32.8 ± 14 yrs). The same prevalence of SA occurred when comparing group 1 (11.7%) with group 2 (15.4%) with a similar GW at SA. The group 1 was older at the pregnancy time and younger at the interview than the group 2 (P < 0.01 for both); moreover, the group 1 showed a higher prevalence of cesarean delivery (P < 0.0001). The overall prevalence of obstetrical syndromes was similar between two groups: however, gestational diabetes was described only in pdC1INH-untreated pregnancies. In utero pdC1INH-exposed children (n = 45) did not show differences compared with unexposed ones (n = 99) in neonatal short-term outcomes. Conclusion: Through appropriate management and counseling, most of C1INH-HAE women undergo successful pregnancy and delivery. For pregnant C1INH-HAE women being treated with pdC1INH, our findings are reassuring and might lead to an improvement of both the knowledge about treatments and the experience of HAE itself.

Clinical aspects of hymenoptera venom allergy and venom immunotherapy.

Summary: Hymenoptera venom allergy (HVA) is the most frequent cause of anaphylaxis in Europe, accounting for most of the severe reactions occurring in adults, and being the second cause of anaphylaxis in children. Prevention of further episodes in patients who developed a systemic reaction (SR) is based on the correct management of the allergic emergency, the referral to an allergist for a correct diagnosis, prescription of adrenaline auto-injectors (AAI) and specific venom immunotherapy (VIT), if recommended. Diagnosis is based on the classification of the type of reaction, confirmation of an IgE-mediated pathogenesis and the identification of the offending insect. The use of component resolved diagnostics may be helpful in case of poly-sensitization or negative allergy tests with a proven history of previous SRs. When a severe SR occurs, baseline serum tryptase levels should always be assessed. The prescription of AAI is recommended or suggested for untreated patients, patients undergoing VIT and after discontinuation of treatment, according to multiple evidence. VIT is the most effective treatment available for HVA patients, as confirmed by recent European guidelines. VIT has an early, sustained and persistent protective effect and modifies the natural course of the disease. Moreover, VIT proved to be safe and well tolerated. According to a recent systematic review, no treatment-related fatalities were recorded to date. Compared to AAI, VIT significantly improves the quality of life of HVA patients by reducing the anxiety and limitations in daily activities caused by the fear of stinging insects. The memory of a life-threatening experience is the most likely reason why adherence to VIT is higher compared to immunotherapy with inhalant allergens. Several risk factors in HVA have been identified that can influence not only the severity of sting reactions in untreated patents, but also the occurrence of side effects, treatment effectiveness and the risk of relapse after discontinuation of VIT. Patient and treatment-related risk factors must be considered while selecting the best candidates for VIT, the type and duration of treatment. In this paper we address the most important issues related to HVA and VIT that may have an impact on daily clinical practice.

Arterial embolization is a rapid and effective technique for controlling pelvic fracture hemorrhage.

OBJECTIVE: To review the success rate of embolization in stopping hemorrhage for unstable patients with severe pelvic fractures, to calculate the time to achieve embolization, and to determine the yield from angiography. DESIGN: Retrospective review of patients admitted to a Level I trauma center with pelvic fractures during a 5-year period. MATERIALS AND METHODS: Charts were reviewed for Injury Severity Score, age, blood pressure, prothrombin time/partial thromboplastin time, pelvic fracture type, mortality, time to reach the angiography suite, time to achieve embolization, and mechanism of injury. MEASUREMENTS AND MAIN RESULTS: Of 806 patients admitted with pelvic fractures, 35 underwent pelvic angiography, and 15 (1.9%) required embolization. Embolization was successful for all patients. No deaths resulted from ongoing hemorrhage. Angiography yield in initially unstable patients was 64%. The mean age and initial hemodynamic instability were significantly greater in nonsurvivors. The time from arrival in the trauma bay to arrival in the angiography suite ranged from 50 to 1,140 minutes, and the time spent in the angiography suite performing embolization ranged from 50 to 140 minutes, with an average time of 90 minutes. Patients who were embolized within 3 hours of arrival had a significantly greater survival rate. CONCLUSION: Only a small percentage of patients with pelvic fractures require embolization, but when it is used, embolization can be 100% effective. Age, time to achieve embolization, and initial hemodynamic instability appear to be important factors in survival.

Splenic infarction caused by a large thoracic aortic thrombus.

We report on a patient with left upper quadrant pain as a result of splenic infarction; the patient was subsequently found to have a thoracoabdominal aortic thrombus extending through the celiac axis. The patient was successfully treated with an aortic thrombectomy guided by intraoperative transesophageal echocardiography.