Emery-Dreiffus muscular dystrophy: MR imaging and spectroscopy in the brain and skeletal muscle

Emery-Dreifuss muscular dystrophy is a rare disorder characterized by childhood onset of contractures, humeroperoneal muscle atrophy, and cardiac conduction abnormalities. This report presents the cases of two brothers with this dystrophy in whom bilateral hypomyelination of the deep periatrial white matter was noted. In the hypomyelinated regions, a prominent peak centered at 1.5 parts per million was present on short-TE MR spectra likely representing prominence of proteolipids in the macromolecular region. Major peaks (N-acetyl-aspartate, creatine, choline, and myoinositol) were normal. With respect to muscle changes, atrophy of the medial head of the gastrocnemius muscle was noted at MR imaging, and phosphorus spectroscopy of this muscle revealed decreased phosphocreatine and inorganic phosphate peaks.     

Exercise and allergic diseases

The aim of this study was to compare exercise-induced bronchial reaction between healthy control subjects and subjects with allergic rhinitis (AR) and allergic asthma (AA). It included 16 controls, 16 subjects with AR and 19 subjects with AA. A skin prick test, pulmonary function test, histamine challenge test and exercise challenge test (ECT) were performed in all subjects. Bronchial reaction to exercise was expressed as the fall index FEV1 (%), AUC(0-30) (min x %), and fall index FEF(25-75) (%). After ECT, subjects with AA had a significantly greater bronchial reaction to exercise than subjects with AR and controls (respective fall index FEV1 8.4, 2.9, and 2.4%, P=0.0083; AUC(0-30) 127.7, 29.6, and 33.1 min x %, P=0.025; and fall index FEF(25-75) 14.6, 0.06, and 1.9%, P<0.001). No difference was found between subjects with AR and controls. In conclusion, ECT induced a significantly greater bronchial reaction in patients with AA and bronchial hyperreactivity to histamine than in patients with AR and bronchial normoreactivity to histamine and controls. This difference was not found between subjects with AR and controls.     

Non-occupational risk factors for bladder cancer: a case-control study

AIMS: The aim of this study was to determine non-occupational risk factors for bladder cancer in Serbia. METHODS AND DESIGN: A hospital-based, case-control study included 130 newly diagnosed bladder cancer patients and the same number of individually matched controls with respect to sex, age (+/- 2 years) and type of residence (rural or urban), from the Clinical Center of Serbia in Belgrade and from the Clinical Center in Kragujevac in central Serbia. The study took place from June 1997 to March 1999. RESULTS: According to multivariate logistic regression analysis, there was an association between: frequency of daily urination (OR = 0.18; 95% CI = 0.08-0.39); consumption of liver (OR = 13.81; 95% CI = 2.49-76.69), canned meat (OR = 8.38; 95% CI = 1.74-40.36), fruit juices (OR = 0.08; 95% CI = 0.01-0.56); the highest tertile of pork (OR = 4.55; 95% CI = 1.30-15.93), cabbage (OR = 0.25; 95% CI = 0.06-1.01) and vinegar (OR = 4.41; 95% CI = 1.18-16.50) intake and risk for bladder cancer. CONCLUSIONS: Consumption of liver, canned meat, pork (h vs l tertile) and vinegar (m vs l tertile) was indicated as a risk factor for bladder cancer, whereas frequent daily urination, consumption of fruit juices and cabbage (h vs l tertile) were indicated as protective factors.     

Interphalangeal joint cartilage: high-spatial-resolution in vivo MR T2 mapping--a feasibility study

The purpose of this study was to evaluate feasibility of magnetic resonance (MR) T2 mapping of the proximal interphalangeal joint of the index finger. Cartilage T2 maps with an in-plane resolution of 39 microm were obtained from five asymptomatic subjects-four men and one woman, aged 24-45 years-by using a 3.0-T MR imager. Image acquisition time was 9.6 minutes. All cartilage T2 maps demonstrated spatial variation similar to that reported previously for knee cartilage, with T2 values increasing toward the articular surface. These results demonstrate the feasibility of acquiring cartilage T2 maps of small joints in the hand. Application of T2 mapping techniques to non-weight-bearing joints may provide a means for differentiation of local biomechanical and systemic factors that can affect cartilage T2 values.     

Spray-dried carbamazepine-loaded chitosan and HPMC microspheres: preparation and characterisation

In this study, the potential of the spray-drying technique for preparing microspheres able to modify the release profile of carbamazepine was investigated. Low-, medium- and high-molecular-weight chitosan and hydroxypropyl methylcellulose (HPMC) in different drug-polymer ratios were used for the preparation of microspheres. The microspheres, characterized by X-ray powder diffractometry (XRD) and differential scanning calorimetry (DSC), were also studied with respect to particle size distribution, drug content and drug release. The results indicated that the entrapment efficiency (EE), as well as carbamazepine release profile, depended on polymeric composition and drug-polymer ratios of the microspheres prepared. The best entrapment efficiencies were obtained when chitosan of low-molecular-weight (CL) or HPMC were used for the microencapsulation. For all types of polymer used, the microspheres with low carbamazepine loading (6.3% w/w) showed better control of drug release than the microspheres with higher drug loadings. The HPMC microspheres showed the slowest carbamazepine release profile with no initial burst effect. Carbamazepine release profiles from ternary systems, carbamazepine-CL-HPMC microspheres, depended mostly on HPMC content and showed similar carbamazepine release profile as CL microspheres when HPMC content was low (9:1 CL-HPMC ratio, w/w). Otherwise, the carbamazepine release from CL-HPMC microspheres was remarkably faster than from either chitosan or HPMC microspheres. The release profile of carbamazepine from the microspheres was highly correlated with the crystalline changes occurring in the matrix.     

Cdk5 in the adult non-demented brain

Cyclin-dependent kinase 5 (Cdk5), a Ser/Thr kinase, regulates the phosphorylation of neuronal proteins and thereby influences neuronal morphology, migration and axon growth. Tightly coordinated interactions between Cdk5 and its activator proteins p35 and p39 are critical for the developmental processes of post-mitotic neurons as well as functioning of the adult CNS. Excessive up-regulation of Cdk5 activity leading to hyperphosphorylation of cytoskeletal proteins has been linked to neurodegenerative disorders, such as Alzheimer's disease (AD). On this basis it was proposed that Cdk5 might be a promising drug target. The physiologic role of Cdk5 in the adult CNS has been addressed recently. It was demonstrated that Cdk5 is involved in striatal and hippocampal neuronal plasticity and long-term behavioral changes associated with these processes. On the basis of the newly identified role of Cdk5 in synaptic plasticity, learning and memory the view that Cdk5 represents a good drug target in AD accompanied by cognitive dysfunctions may have to be revisited. Alternatively, targeting the mechanisms up-stream of Cdk5 leading to deregulation of Cdk5 activity, such as proteolytic cleavage of its activating subunits may prove to be more beneficial as a therapeutical approach.     

Influence of the wars in Croatia and Bosnia and Herzegovina on the incidence and outcome of singleton premature births in the Split University Hospital

To investigate the influence of the wars in Croatia and Bosnia and Herzegovina on incidence and perinatal outcome of singleton preterm births at the Department of Gynecology and Obstetrics in the Split University Hospital. Data were collected by reviewing patients’ files at the Department of Gynecology and Obstetrics from three periods: the three years before the war (1988–1990), during the war (1992–1994), and after the war (1996–1998). A total of 2,358 patients’ files of singleton preterm delivery were analyzed. Singleton preterm delivery rate decreased during the war (5.02%) and post-war period (4.74%) compared to the pre-war period (6.19%). Stillbirth and early neonatal mortality rates of singleton premature babies significantly increased during the war to 226%, compared to 193% before the war and 134% after the war. Early neonatal mortality rate was 215% during the war, 209% in the pre-war period, and 156% after the war. Despite the continuous decrease in singleton preterm birth rate throughout the observed periods, the increase in stillbirth rate and early neonatal mortality rate during the war might have been caused by the war. This may be due to primary gynecological care being inadequate for many pregnant women.     

The prevalence of Parkinson’s disease among Vilnius inhabitants

In a hospital-based study we investigated the prevalence of Parkinson’s disease among inhabitants of the Vilnius city, the capital of Lithuania. The study group was selected from patients who were diagnosed with Parkinson’s disease during the time frame of 1978-2005. Patients’ time of diagnosis were based on the data of dispensary cards, registration journals and/or other documentation. A questionnaire and Mini Mental State Examination provided data for analysis on the conditions of the patients. The prevalence of Parkinson’s disease in Vilnius is 1.32/1000 inhabitants and is higher in men than in women (p < 0.05). The age of Parkinson’s disease onset in men and women is the same (63.77 ± 0.70 years). The rigidity-tremor form of Parkinson’s disease is the most frequent (76.8% of all cases). The PD prevalence rate in Vilnius inhabitants are close to the mean levels observed in studies made in Finland, Austria, Germany. The prevailing form of Parkinson’s disease is rigidity-tremor.