Successful Treatment of Kasabach-Merritt Syndrome with Prednisone and Epsilon-Aminocaproic Acid

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.     

Patients with schizophrenia previously stabilized on conventional depot antipsychotics experience significant clinical improvements following treatment with long-acting risperidone.

BACKGROUND: Conventional depot antipsychotics can provide constant pharmacologic treatment, eliminating partial compliance and reducing relapse risk. Atypical antipsychotics, have improved clinical profiles but require daily dosing, compromising their overall effectiveness. As oral risperidone provides safety and efficacy benefits over oral haloperidol, improvements may be realized by replacing conventional with atypical agents in long-acting therapy. This report examines 50-weeks of long-acting risperidone therapy in patients previously stabilized with conventional depot antipsychotics. METHODS: A multi-center, open-label study enrolled 725 patients with schizophrenia or schizoaffective disorder, judged clinically stable and maintained on stable antipsychotic doses for > or =4 weeks. Assignment by clinician judgment to receive 25-75 mg of long-acting risperidone every 2 weeks for 50 weeks followed, with performance of standard safety and efficacy assessments. Data are presented on patients receiving conventional depot antipsychotic monotherapy at study entry. RESULTS: In the 188 (25.9%) patients receiving conventional depot antipsychotic monotherapy at entry, mild-to-moderate mean (+/-S.D.) Positive and Negative Syndrome Scale (PANSS)-total scores improved significantly after receiving long-acting risperidone (64.2 +/- 18.9 to 58.2 +/- 20.3; P < 0.001). Clinical improvement of > or =20%, 40%, or 60% reduction in PANSS-total score, occurred in 52%, 34%, and 16% of patients, respectively. ESRS subjective ratings and objective physician ratings (Parkinsonism) decreased significantly (P < 0.001). CONCLUSION: Stable patients with mild, residual symptomatology treated with conventional depot antipsychotics experienced significant improvement in psychiatric and movement disorder symptomatology following 1-year of treatment with long-acting risperidone.     

Comparison of self-reports and biological measures for alcohol, tobacco, and illicit drugs consumption in psychiatric inpatients

BACKGROUND: Asking psychiatric in-patients about their drug consumption is unlikely to yield reliable results, particularly where alcohol and illicit drug use is involved. The main aim of this study was to compare spontaneous self-reports of drug use in hospitalized psychiatric patients to biological measures of same. A secondary aim was to determine which personal factors were associated with the use of tobacco, alcohol, and illicit drugs as indicated by these biological measures. METHODS: The consumption of substances was investigated using biological measures (urine cotinine, cannabis, opiates, cocaine, amphetamines and barbiturates; blood carbohydrate-deficient transferrin [CDT] and gamma-glutamyl transferase [GGT]) in 486 consecutively admitted psychiatric patients, one day following their hospitalization. Patients' self-reports of alcohol, tobacco and illicit drugs consumption were recorded. Socio-professional and familial data were also recorded. RESULTS: The results show a low correlation between biological measures and self-reported consumption of alcohol and illicit drugs. Fifty-two percent of the patients under-reported their consumption of illicit drugs (kappa=.47). Patients with schizophrenia and personality disorders were more likely to disclose their illicit drug consumption relative to patients suffering from mood disorders and alcohol dependence. Fifty-six percent of patients underreported alcohol use, as evaluated by CDT (kappa=.2), and 37% underreported when using the CDT+GGT measure as an indicator. Smoking appeared to be reported adequately. In the study we observed a strong negative correlation between cannabis use and age, a strong correlation between tobacco and cannabis use, and correlations between tobacco, cannabis and alcohol consumption. CONCLUSION: This study is the first to compare self-reports and biological measures of alcohol, tobacco and illicit drug uses in a large sample of inpatients suffering from various categories of psychiatric illnesses, allowing for cross-diagnosis comparisons.     

Expression and biological effects of high levels of serum IgE in epsilon heavy chain transgenic mice

We have generated and examined transgenic mice carrying a rearranged immunoglobulin transgene coding for the heavy chain of an IgE antibody. These mice produce the secreted form of the recombinant epsilon heavy chain. Serum IgE levels were increased at least 100-fold over control values. Transgenic epsilon mRNA was detected in spleen and thymus, not in liver and heart. Transgenic epsilon production in vitro was slightly up-regulated by T cells, but not affected by interleukin 4 in vitro or Nippostrongylus infestation in vivo. The B cell and T cell compartments and antigen-specific IgE, IgG1 and IgM responses as well as the increase in endogenous IgE after Nippostrongylus infestation in transgenic mice were normal. These data indicate that the presence of high levels of transgenic IgE did not induce class-specific suppressive mechanisms. Transgenic IgE bound to Fc epsilon receptor type I and Fc epsilon receptor type II and mediated histamine release from mast cells in vitro and an allergic skin reaction in vivo. It inhibited an ovalbumin-specific skin reaction in ovalbumin-immunized transgenic mice only during the initial phases of the immune response. This result has a bearing on the feasibility of immune therapy of allergic diseases with substances that block binding of IgE to its receptors.     

Quantitative SPECT leads to improved performance in discrimination tasks related to prodromal Alzheimer's disease.

We investigated the impact of the quantitation and reconstruction protocol on clinical tasks. The performance of standard clinical reconstruction procedures in discrimination tasks related to the diagnosis of prodromal Alzheimer's disease (AD) was compared with the performance of a quantitative approach incorporating improved corrections for scatter, attenuation, intrinsic spatial resolution, and distance-dependent spatial resolution. METHODS: Seventeen normal controls (normal group), 56 subjects who did not have dementia, who did have memory problems, but who did not develop AD within 5 y of follow-up (questionable group), and 27 subjects who did not have dementia, who did have memory problems, and who did develop AD over the follow-up period (converter group) were considered in this study. (99m)Tc-hexamethylpropyleneamine oxime SPECT and MRI studies were performed for each subject at baseline. The standard quantitation protocol (STD), routinely used in our clinic, consisted of Compton window scatter correction followed by filtered backprojection with attenuation correction using a uniform attenuation map. In the improved quantitative approach (QUAN), projections were corrected for scatter by use of a general spectral method and reconstructed by use of ordered-subset(s) expectation maximization, incorporating corrections for collimator response and attenuation using both a uniform attenuation map (QUANunif) and a nonuniform attenuation map (QUANnonunif). Mean SPECT activity concentration and MRI volume were estimated for 7 structures: rostral anterior cingulate gyrus, caudal anterior cingulate gyrus, posterior cingulate gyrus, hippocampus, basal forebrain, amygdala, and the banks of the superior temporal sulcus. Data were analyzed by pairwise discriminant analysis, and performance in binary group discrimination was measured by correlated receiver-operating-characteristic analysis. RESULTS: The use of QUANnonunif yielded a small but systematic improvement in discrimination accuracy for normal versus converter groups (accuracy or area under the receiver-operating-characteristic curve [Az], 0.965), normal versus questionable groups (Az, 0.973), and questionable versus converter groups (Az, 0.881) compared with the results obtained with QUANunif (Az, 0.955, 0.962, and 0.866, respectively). Discrimination performance was significantly lower (P < 0.05) with STD than with QUAN in all 3 tasks (Az with STD, 0.906, 0.878, and 0.768, respectively). MRI volume estimation led to a lower overall performance in all 3 tasks than did QUANnonunif (Az with MRI, 0.947, 0.917, and 0.872, respectively). CONCLUSION: Improved quantitative image reconstruction with accurate compensation for scatter, attenuation, and variable collimator response led to significantly better performance in discrimination tasks related to the diagnosis of prodromal AD than did standard clinical reconstruction procedures. The use of a nonuniform brain attenuation map yields a small improvement in discrimination accuracy.     

Tuberculosis in healthcare workers caring for a congenitally infected infant.

OBJECTIVE: To assess the extent of nosocomial transmission of tuberculosis among infants, family members, and healthcare workers (HCWs) who were exposed to a 29-week-old premature infant with congenital tuberculosis, diagnosed at 102 days of age. DESIGN: A prospective exposure investigation using tuberculin skin test (IST conversion was conducted. Contacts underwent two skin tests 10 to 12 weeks apart. Clinical examination and chest radiographs were performed to rule out disease. Isoniazid prophylaxis was administered to exposed infants at higher risk. SETTING: A neonatal intensive care unit in an urban hospital in Brussels, Belgium. PARTICIPANTS: Ninety-seven infants, 139 HCWs, and 180 visitors. RESULTS: Newly positive TST results occurred in HCWs who had been in close contact with the infant. Six (19%) of 32 primary care nurses and physicians had TST conversions and received treatment. Among the 97 exposed infants, 85 were screened and 34 were identified as at higher risk of infection. Of these, 27 received preventive isoniazid. None of the infants and none of the 93 other infants' family members evaluated were infected. CONCLUSIONS: Congenital tuberculosis in an infant poses a risk for nosocomial transmission to HCWs. Delayed diagnosis of this rare disease and close proximity are the most important factors related to transmission.     

Can patients with severe left ventricular dysfunction be treated by coronary artery bypass surgery?]

Forty three men and 3 women, with an average age of 59 years (13 to 78 years) underwent aorto-coronary bypass surgery despite severe left ventricular dysfunction (ejection fraction < 35%); 96% of the patients had previous infarction; 60% (N = 28) had unstable angina, 52% (N = 24) had had pulmonary oedema or an episode of congestive cardiac failure. The average ejection fraction was 29 +/- 4%, range 17 to 35%. Thirteen patients had ventricular aneurysms, 4 had grade 3 or 4 mitral regurgitation. The coronary lesions were usually multivessel left main coronary (6), triple vessel disease (27), double vessel disease (12), single vessel disease (1). The average number of bypass grafts per patient was 2.3. The average aorting clamping time was 63 minutes (range 26 to 133 minutes). There were 4 mitral valve replacements, 4 resections of ventricular aneurysms and 1 double procedure (aneurysmectomy and valve replacement). The operative mortality was 2.1% (1 death). During an average follow-up period of 27 months (range 3 to 90 months), there were: 2 recurrent infarctions, 13 episodes of cardiac failure and 8 cardiac deaths (cardiac failure: 5, sudden death: 2, recurrent infarction: 1). Two patients underwent cardiac transplantation. The regression of angina (90% of operated patients were asymptomatic) and the low operative risk, justify aortocoronary bypass surgery despite left ventricular dysfunction in patients with severe symptoms (unstable angina, chronic, invalidating angina). The medium-term results indicate a high risk of cardiac failure which is partially responsible for the secondary mortality rate of 17% at 2 years.