A bleeding disorder due to deficiency of alpha 2-antiplasmin

A deficiency of alpha 2-antiplasmin has been identified in a female patient with severe and frequent bleeding episodes. Routine coagulation and platelet assays of the patient's plasma were within normal limits. However, abnormally rapid whole blood or dilute plasma clot lysis times and an abnormal FXIII test in which clots were lysed in the presence of urea or saline suggested an abnormal fibrinolytic system. Analysis of alpha 2-antiplasmin levels by radioimmunoassay revealed less than 1.0 microgram/ml alpha 2-antiplasmin. Functional assays indicated an alpha 2-antiplasmin level less than or equal to 10% of normal. Addition of purified alpha 2-antiplasmin to the patient's plasma restored its ability to inhibit plasmin in in vitro assays, and mixtures of patient plasma with normal plasma did not interfere with the antiplasmin activity of the normal plasma. Whereas normal platelets contain 68 ng alpha 2-antiplasmin/10(9) platelets, platelets from the patient contained 30% of the normal level of antigen. Analysis of alpha 2- antiplasmin functional and antigenic levels in the plasma of both parents and four siblings of the propositus provided evidence consistent with an autosomal mechanism of inheritance of alpha 2- antiplasmin deficiency. One sibling appeared to be homozygous and three siblings and the parents were heterozygous for the deficiency. Two heterozygotes had positive bleeding histories. The association of a bleeding disorder with a deficiency of alpha 2-antiplasmin emphasizes that lack of regulation of the fibrinolytic system can result in a hemostatic dysfunction.     

Hepatic artery pseudoaneurysm: diagnosis with real-time and pulsed Doppler US

Duplex ultrasonography (US), consisting of real-time and pulsed Doppler US, was used to identify a pseudoaneurysm in a patient with recurrent bleeding and chronic pancreatitis. The authors present a case that illustrates the limitations of real-time US in evaluating sonolucent masses in pancreatitis and the need for using pulsed Doppler technique.     

Factors predicting severe radiation pneumonitis in patients receiving definitive chemoradiation for lung cancer

PURPOSE: To identify factors that may predict for severe radiation pneumonitis or pneumonopathy (RP), we reviewed a set of simple, commonly available characteristics. METHODS AND MATERIALS: Medical records of 148 lung cancer patients with good performance status (ECOG 0-1) treated definitively with chemoradiation from 6/92-6/98 at the University of Pennsylvania were reviewed. Actuarial survival and the crude rate of severe radiation pneumonitis were determined as a function of several variables. Potential predictive factors examined included age, gender, histology, stage, pulmonary function, performance status (0 vs. 1), weight loss, tumor location, radiation dose, initial radiation field size, chemotherapy regimen, and timing of chemotherapy. Univariate analysis (log-rank test) was performed for each variable. Multivariate analysis was performed using linear regression. RESULTS: Median survival for the entire cohort was 14.7 months. Four patients were inevaluable for pneumonitis due to early death from progressive disease. Of the remaining 144 evaluable patients, 12 (8.3%) experienced severe RP. The most significant factor predicting for severe RP was performance status (p < 0.003). The risk of severe RP was 16% for PS-1 patients vs. 2% for PS-0 patients. Women were significantly more likely to develop severe RP than men (p = 0.01). Among 67 patients for whom pre-radiation therapy pulmonary function data were available, forced expiratory volume of the lung in 1 second (FEV(1)) was also significant (p = 0. 03). No patient suffering severe RP had a pretreatment FEV(1) > 2.0 liters. The median radiation dose was 59.2 Gy and median initial radiation field size was 228 cm(2). Neither radiotherapy factor predicted for RP. Other factors studied, including chemotherapy drugs, and schedule, also were not significant predictors of severe RP. CONCLUSIONS: Pretreatment performance status, gender, and FEV(1) are significant predictors of severe radiation pneumonopathy, at least when using conventional radiation fields and doses. Complex radiation dose-volume algorithms that attempt to predict lung complication probabilities should probably incorporate these simply obtained clinical parameters.     

Psychotic syndromes in epilepsy

Claims have been made that epilepsy is associated with both brief psychotic episodes and persistent psychotic states. The existence of brief, nonconfusional psychotic episodes in epilepsy has received some formal support; these may be a function of temporal lobe subictal activity. Persistent psychosis is found in about 7% of patients with epilepsy; independent evidence indicates this to be a significant association. Temporal lobe seizure activity, again probably acting subictally, may be involved in the pathogenesis of these states, perhaps in interaction with other factors. Occurrence of psychotic syndromes in epilepsy may be relevant to kindling, atypical psychoses unrelated to epilepsy, and the psychotropic effect of carbamazepine.     

Sensitivity to cuteness in baby faces is not influenced by pregnancy

BackgroundThe ability to discriminate cuteness may aid caregivers in prioritising care to the neediest child. This biologically important ability has been indirectly linked to higher levels of female reproductive hormones via studies of hormonal contraception and menopausal status. Pregnancy provides an opportunity to further investigate the role of reproductive hormones in cuteness discrimination since it is a time of substantial natural hormonal fluctuation.MethodsPregnant (n=23) and matched non-pregnant women (n=11) were assessed four times over 8 months (at 20 weeks of gestation, 32 weeks of gestation, 2 weeks postpartum, 12 weeks postpartum). At each visit, cuteness sensitivity, cuteness intensity ratings, and basic visuospatial perception were assessed. Cuteness sensitivity was assessed by presenting two versions of the same face side by side, with one subtly altered by graphics software to be more or less cute than the other; women were asked to select the cuter face. Cuteness intensity was rated on a seven-point Likert scale. Results were analysed with repeated measures ANOVA.FindingsThere was no difference between pregnant/postpartum mothers and control women in cuteness sensitivity, cuteness intensity ratings, or basic visuospatial perception. There was no change in these abilities across time.InterpretationThis result is not what we hypothesised. It seems that the link between female reproductive hormones and cuteness sensitivity is more indirect and complex than initially thought. Possibly female reproductive hormones other than those elevated in pregnancy are important in determining cuteness sensitivity.FundingWellcome Trust.