Over 45 years ago, Montreal Platelet Syndrome was first described as a rare inherited platelet disorder characterised by macrothrombocytopenia with spontaneous platelet clumping, abnormal platelet shape change upon stimulation and a defect in platelet calpain. This syndrome has now been reclassified as type 2B von Willebrand disease with the V1316M VWF mutation in the only kindred ever reported. We herein revisit the historical platelet characteristics originally described in Montreal Platelet Syndrome in light of the new diagnosis. This paper will review the 45-year saga of Montreal Platelet Syndrome, a story that highlights the value of revisiting a rare diagnosis to look for a more common explanation.
OBJECTIVES: HIV/AIDS surveillance methods are under revision as the diversity of HIV epidemics is becoming more apparent. The so called "2nd generation surveillance (SGS) systems" aim to enhance surveillance by broadening the range of indicators to prevalence, behaviors and correlates, for a better understanding and a more complete and timely awareness of evolving epidemics. METHODS: Concepts of HIV SGS are reviewed with a special focus on injecting drug users, a major at-risk and hard to reach group in Europe, a region with mainly low or concentrated epidemics. RESULTS: The scope of HIV/AIDS surveillance needs to be broadened following principles of SGS. Specifically for IDUs we propose including hepatitis C data as indicator for injecting risk in routine systems like those monitoring sexually transmitted infections and information on knowledge and attitudes as potential major determinants of risk behavior. CONCLUSIONS: The suggested approach should lead to more complete and timely information for public health interventions, however there is a clear need for comparative validation studies to assess the validity, reliability and cost-effectiveness of traditional and enhanced HIV/AIDS surveillance systems.
The results of screening more than 23,000 serum samples from persons belonging to risk groups, as well as those not belonging to such groups, in Moscow, Vilnius and Klaipeda are presented. Screening was carried out with the use of an assay system manufactured by the Scientific and Industrial Amalgamation "Antigen" (USSR). In this screening 3 HIV carriers were detected; of these, 2 were foreign students from two African countries.
Three cases of visceral leishmaniasis (kala-azar) are presented. One of these was in a 43-year-old patient with AIDS who was infected in Southern Spain. Another was in a man aged 25 years infected in West Africa. These cases are the first two adults to be reported in Denmark. The third case was an 18 month old previously healthy boy, infected in Southern Spain. The symptomtology, diagnosis and treatment of the disease are discussed and it is stressed that serological diagnostic tests have limited value in HIV positive patients.
OBJECTIVE: To follow the clinical course of patients with the mitochondrial DNA mutation 3243A>G for 3 years. METHODS: Thirty-three adult patients with the 3243A>G mutation entered a 3-year follow-up study. They were clinically evaluated annually, audiometry was performed, and samples were drawn for the analysis of blood chemistry and mutation heteroplasmy in leukocytes. Holter recording was performed three times during the follow-up and echocardiography, neuropsychological assessment, and quantitative EEG and brain imaging conducted at entry and after 3 years. RESULTS: The incidence of new neurologic events was low during the 3-year follow-up. Sensorineural hearing impairment (SNHI) progressed, left ventricular wall thickness increased, mean alpha frequency in the occipital and parietal regions decreased, and the severity of disease index (modified Rankin score) progressed significantly. The rate of SNHI progression correlated with mutation heteroplasmy in muscle. The increase in left ventricular wall thickness was seen almost exclusively in diabetic patients. Seven patients died during the follow-up, and they were generally more severely affected than those who survived. CONCLUSIONS: Significant changes in the severity of disease, sensorineural hearing impairment, left ventricular hypertrophy, and quantitative EEG were seen in adult patients with 3243A>G during the 3-year follow-up.
Comment In: Neurology. 2007 Jan 9;68(2):163-417210904
OBJECTIVE: Schizophrenia is associated with a shortened life expectancy and increased somatic comorbidity with, e.g., cardiovascular disorders. One major risk factor for these disorders is the metabolic syndrome, which has been reported to have a higher frequency in schizophrenic patients. Our objective was to study the prevalence of metabolic syndrome in a population-based birth cohort. METHOD: The study sample consisted of 5613 members of the Northern Finland 1966 Birth Cohort who participated in the field study from 1997 to 1998. Subjects were divided into 4 diagnostic categories (DSM-III-R): (1) schizophrenia (N = 31), (2) other functional psychoses (N = 22), (3) nonpsychotic disorders (N = 105), and (4) no psychiatric hospital treatment (N = 5455, comparison group). Subjects were assessed for the presence of metabolic syndrome according to the criteria of the National Cholesterol Education Program. RESULTS: The prevalence of metabolic syndrome was higher in subjects with schizophrenia compared with the comparison group (19% vs. 6%, p = .010). The prevalence of metabolic syndrome in subjects with other psychoses was 5%. After controlling for sex, the results of logistic regression analysis showed that the risk of metabolic syndrome in schizophrenia was 3.7 (95% CI = 1.5 to 9.0). CONCLUSIONS: The high prevalence of metabolic syndrome in schizophrenia even at such a relatively young age underscores the need to select antipsychotic medications with no or little capability to induce metabolic side effects. Also, developing comprehensive efforts directed at controlling weight and diet and improving physical activity are needed.
Polyalthia longifolia var. pendula is used as an antipyretic agent in indigenous systems of medicine. Microglia-mediated inflammation plays an important role in the pathway leading to neuronal cell death in a number of neurodegenerative diseases. The aim of this study was to investigate the effects of 6-hydroxycleroda-3,13-dien-15,16-olide (PL3) extracted from Polyalthia longifolia var. pendula on lipopolysaccharide(LPS)-induced inflammation in microglia-like HAPI cells and primary microglia cultures. In microglia-neuron co-cultures, LPS decreased the cell viability of neuroblastoma SH-SY5Y cells. LPS-induced cell death was attenuated by the NOS inhibitor, L-NAME, the COX-2 inhibitor, NS-398 or the NADPH oxidase inhibitor, DPI, respectively. In LPS-treated microglia cells, PL3 decreased the expression of iNOS, COX-2, gp91 (phox), and NF- kappaBp65, the degradation of I kappaB alpha, and the production of NO, PGE (2), iROS, and TNF- alpha. PL3 also enhanced the expression of HO-1, a cytoprotective and anti-inflammatory enzyme. Moreover, PL3 reduced LPS-activated microglia-induced cell death. The present results suggest that PL3 inhibits microglia-mediated inflammation and inflammation-related neuronal cell death. Therefore, PL3 has potential use for the treatment of inflammation-related neurodegenerative diseases.