Human parotid proline-rich proteins: correlation of genetic polymorphisms to dental caries

Parotid saliva contains a variety of proline-rich proteins. This study found that, among 306 children between the ages of 5 to 15 years, there is a significant increase in the decayed-missing-filled tooth surface (DMFS) score of the permanent teeth with age in children with the specific proline-rich protein phenotypes Pa and Pr. However, the increase in DMFS score of the permanent teeth of children was significantly greater in children with Pa+ and Pr22 than in those with the other phenotypes (Pa- or Pr11 and Pr12). The previously established close correlation between the Pa and Pr phenotypes and the genetic variants of salivary peroxidase (a powerful antibacterial system in the oral cavity) may provide an explanation for the relationship of certain proline-rich protein phenotypes to dental caries.     

Unique cerebellar phenotype combining granule and Purkinje cell loss: Morphological evidence forweaver*pcd double mutant mice

Summary Weaver (wv/wv) mutant mice lose most granule cells of the cerebellum during the first 2 weeks of postnatal life; Purkinje cell degeneration (pcd/pcd) mutants lose virtually all Purkinje cells between postnatal days 17 and 45. Both these neurological mutations are autosomal recessive. We designed a breeding protocol that, in theory, should result in the production of mice with a doubly mutant,wv/wv*pcd/pcd, genotype. Some of the offspring of such crosses had a novel cerebellar phenotype in which both granule and Purkinje cells underwent degeneration, leading to a highly atrophic cortex. This phenotype is what would be expected inwv/wv*pcd/pcd double mutants, and the proportion of such progeny obtained fits with genetic expectations. We propose that (1)wv/wv*pcd/pcd double mutant mice are viable, and (2) the anatomical phenotype of such mice is a combined expression of the component phenotypes.     

Total but not resting energy expenditure is increased in infants with ventricular septal defects

OBJECTIVE: The purpose of this study was to determine the effect of left-to-right shunting on the resting energy expenditure (REE), total energy expenditure (TEE), and energy intake in a group of 3- to 5-month-old infants with moderate to large unrepaired ventricular septal defects (VSDs) compared with age-matched, healthy infants. METHODS: Eight infants with VSDs and 10 healthy controls between 3 to 5 months of age participated in the study. Indirect calorimetry was used to measure REE and the doubly-labeled water method was used to measure TEE and energy intake. An echocardiogram and anthropometric measurements were performed on all study participants. Daily urine samples were collected at home for 7 days. Samples were analyzed by isotope ratio mass spectrometry. Data were compared using analysis of variance. RESULTS: No significant differences were found in REE (VSD, 42.2 +/- 8.7 kcal/kg/d; control, 43.9 +/- 14.1 kcal/kg/d) or energy intake (VSD, 90.8 +/- 19.9 kcal/kg/d; control, 87.1 +/- 11.7 kcal/kg/d) between the groups. The percent total body water was significantly higher in the VSD infants and the percent fat mass was significantly lower. TEE was 40% higher in the VSD group (VSD, 87.6 +/- 10.8 kcal/kg/d; control, 61.9 +/- 10.3 kcal/kg/d). The difference between TEE and REE, reflecting the energy of activity, was 2.5 times greater in the VSD group. CONCLUSIONS: REE and energy intake are virtually identical between the two groups. Despite this, infants with VSDs have substantially higher TEE than age-matched healthy infants. The large difference between TEE and REE in VSD infants suggests a substantially elevated energy cost of physical activity in these infants. These results demonstrate that, although infants with VSDs may match the energy intake of healthy infants, they are unable to meet their increased energy demands, resulting in growth retardation.     

Intravenous ketamine for treatment of super-refractory convulsive status epilepticus with septic shock: A report of two cases

Refractory and super-refractory status epilepticus is a life-threatening neurological emergency, associated with high morbidity and mortality. Treatment should be aimed to stop seizure and to avoid cerebral damage and another morbidity. Published data about effectiveness, safety and outcome of various therapies and treatment approaches are sparse and are mainly based on small case series and retrospective data. Here we report successful management of two cases of super-refractory status epilepticus refractory to anesthetic therapy with midazolam and complicated by septic shock, managed successfully with ketamine infusion.     

Dysphagia and hemispheric stroke: A transcranial magnetic study

INTRODUCTION: Dysphagia is a common and distressing consequence of hemispheric stroke. STUDY AIM: To verify the usefulness of transcranial magnetic stimulation (TMS) studies of swallowing in healthy subjects and in stroke patients. MATERIAL AND METHODS: TMS studies of the motor cortical projections to the upper esophageal sphincter were performed in 45 patients with acute mono-hemispheric stroke (26 patients with dysphagia) and 20 healthy adult volunteers. RESULTS: TMS of either hemisphere in normal volunteers evoked motor evoked potentials (MEP) in the esophagus. The average point of optimal excitability was slightly more anterior in the right hemisphere; otherwise, MEP amplitudes and latencies were similar from both hemispheres as were the areas of the cortical map. The cortical map area and amplitude of MEPs were significantly smaller and the latencies longer after stimulation of the affected hemisphere compared with the unaffected hemisphere and pooled control data. Twenty-four dysphagic patients (92.3%) had abnormalities of MEP of the affected hemisphere, while only five non-dysphagic patients (26%) had these abnormalities. Dysphagic patients were older and had more disability compared with non-dysphagic patients. MEPs of the affected hemisphere of patients with dysphagia were later and smaller in amplitude than MEPs of non-dysphagic patients. The cortical map area was also smaller. CONCLUSION: The esophagus is represented bilaterally in motor cortex, but the hot spot lies more anterior to Cz in right hemisphere compared to left hemisphere. Both the severity of stroke and neuroplasticity of the unaffected hemisphere have implications in the development of dysphagia.