Hindshaker, a novel myelin mutant showing hypomyelination preferentially affecting the spinal cord

Animals with spontaneous mutations affecting myelin formation have provided useful information about the genetic and cellular mechanisms regulating normal and abnormal myelination. In this paper we describe a novel murine mutation termed hindshaker (hsh), which is inherited in an autosomal recessive manner. Affected mice are characterised by a variable tremor of the hind end which commences at about 2 weeks of age and largely disappears in animals older than 6 weeks. There is hypomyelination affecting predominantly the spinal cord, although the optic nerves and brain are involved to a much lesser degree. The defect of thinly myelinated and naked axons is maximal at 20 days of age and largely resolves with time so that in the adult most axons are myelinated. The myelin structure appears normal and immunostains for the major proteins. Although the distribution of oligodendrocytes in the spinal cord is similar to normal during the period of hypomyelination, there are fewer mature cells. The hsh mutation appears to delay the maturation of oligodendrocytes, particularly in the spinal cord. Additionally, there is a considerable variation in phenotypic expression and in penetrance when the mutation is expressed on different genetic backgrounds, suggesting the hsh locus is subject to the influence of modifying gene(s). Identification of the hsh gene should identify a factor important in the development of oligodendrocytes, particularly those in the spinal cord.     

Electronic thermography for the assessment of acute temporomandibular joint pain

Electronic thermography (ET) has the potential to be a nonionizing, noninvasive, low-cost diagnostic alternative for evaluating temporomandibular joint (TMJ) disorders. This study was designed to evaluate the use of ET as a diagnostic aid in the assessment of patients with acute TMJ pain. Computer measurements made using facial thermography were able to distinguish normal patient populations from symptomatic patients with acute TMJ pain. Additional studies are needed before thermographic diagnosis of TMJ disorders will be clinically accepted.     

Rheumatic heart disease in India

A cross-sectional study was carried out in rural areas of Jawan Block, District Aligarh, Uttar Pradesh, India, covering a total population of 3760 drawn from 11 villages. The total number of confirmed cases of rheumatic heart disease was 24 with a prevalence rate of 6.4 per 1000 of the general rural population. The prevalence of rheumatic heart disease increased with age until the age of 25 years. Females were more prone to rheumatic heart disease compared to males. Socio-economic class had a direct impact on the occurrence of rheumatic heart disease.     

Keratin 17 gene expression during the murine hair cycle

Treatment of rats with phenytoin (DPH), an anti-epileptic drug, results in lower tissue thyroid hormone (TH) levels and interferes with the metabolic pathway of TH. To test the hypothesis that DPH affects the enterohepatic cycle of TH and, thus, the kinetics of TH turnover, we performed a kinetic experiment (three-compartment analysis) and a steady-state, double-isotope equilibrium experiment in rats treated for 3 weeks with DPH (50 mg/kg body weight per day) and in untreated controls. This included measurements of TH and TH metabolite levels, as well as the activities of enzymes involved in the TH metabolic pathway. DPH treatment resulted in a decrease in the production of thyroxine (T4) (by 25%) and tri-iodothyronine (T3) (by 37%), a decrease in the T3 concentration in all three pools, and a redistribution of T4 from the fast to the slow pool. The amount of T4 increased in intestinal contents and feces by 66% and 71% respectively. Expressed as a fraction of daily TH disposal, fecal loss of T4 was enhanced from 10 to 23% and that of T3 from 16 to 21%. An increase in T4 and T3 UDP-glucuronyltransferase activities was observed, suggesting that the increased fecal loss of T4 and T3 is secondary to an increased biliary output of their glucuronides. The reduced secretion of TH and increased fecal clearance during DPH treatment can lead in the long run to depletion of TH stores.     

Weighing the risks and benefits of an oral cancer screening

The study into the causes underlying nonspecific lymphadenitis (NSL) and adenophlegmons of the maxillofacial region and the neck (AF) revealed a great variety of etiological factors and primary infectious foci in these diseases. Of 204 children admitted to hospital of the city of Derbent, odontogenic genesis of the disease was determined in 27.45% of cases; dermatogenic, stomatogenic in 23.04 and 12.74% of cases, respectively. ENT and systemic diseases were responsible for NSL and AF in 13.23 and 3.43% of cases. The cause remained unclear in 20.09% of patients. NSL and AF occurred most frequently in the coldest (January, February) and the hottest (July, August) months of the year.     

A 6-year clinical assessment of electronic facial thermography

The authors briefly report their experience regarding the opportunities offered by the use of current ultrasound methods in carotid surgery. They describe: a system for the quantification of athcromasic plaque used to monitor non-operated patients over time; ultrasound methods used to analyse the carotid wall to establish whether it can be utilised as an index of vascular aggression in hypertension, diabetes and atherosclerosis; the use of transcranial Doppler; criteria for the definition of high risk plaque; the applications of eco-color Doppler. The paper also illustrates a new pathology identified by the authors, defined as primary intimal fibrous hyperplasia, and the evolution of the carotid wall after endarterectomy. The structural characteristics of primary hyperplasia can only be shown using ultrasound given that arteriography cannot distinguish it from atheromatic stenosis. After endarterectomy the carotid wall is subject to hematic and hemodynamic stimuli which determine the type of evolution of the wall itself. The authors therefore examine the myointimal reaction, myointimal hyperplasia, early restenosis and late restenosis as different facets of the same phenomenon.     

Visual, cognitive, and neurodevelopmental outcome at 51/2 years in children with perinatal haemorrhagic-ischaemic brain lesions

To determine predictive values of early visual and neurocognitive assessment in children with perinatally acquired haemorrhagic or ischaemic brain lesions selected on the basis of ultrasound, 63 children (37 boys, 26 girls), who had been followed and examined until the age of 18 months, were reexamined at 5 1/2 years. Good correlations between visual and neurodevelopmental assessments at 18 months and at 5 1/2 years were found. When ultrasound abnormalities were combined with early visual and neurocognitive assessment data, good predictive values, especially for the group of children who had grade 2 to 4 leukomalacia, were found for visual acuity and neurodevelopment.     

Bioethics for clinicians: 16. Dealing with demands for inappropriate treatment

Demands by Patients or their Families for treatment thought to be inappropriate by health care providers constitute an important set of moral problems in clinical practice. A variety of approaches to such cases have been described in the literature, including medical futility, standard of care and negotiation. Medical futility fails because it confounds morally distinct cases: demand for an ineffective treatment and demand for an effective treatment that supports a controversial end (e.g., permanent unconsciousness). Medical futility is not necessary in the first case and is harmful in the second. Ineffective treatment falls outside the standard of care, and thus health care workers have no obligation to provide it. Demands for treatment that supports controversial ends are difficult cases best addressed through open communication, negotiation and the use of conflict-resolution techniques. Institutions should ensure that fair and unambiguous procedures for dealing with such cases are laid out in policy statements.