Perceptions and behaviors of learner engagement with virtual educational platforms.

BACKGROUND: The COVID-19 pandemic has increased utilization of educational technology for surgical education. Our aim was to determine attitudes and behaviors of surgical education champions towards virtual educational platforms and learner engagement. METHODS: An electronic survey was distributed to all Association of Surgical Education members addressing i) methods of engagement in virtual learning ii) ways to improve engagement and iii) what influences engagement. Stratified analysis was used to evaluate differences in responses by age, gender, level of training and specialty. RESULTS: 154 ASE members completed the survey (13% response rate). 88% respondents accessed virtual learning events at home. Most (87%) had joined a virtual learning event and then participated in another activity. 1 in 5 who did this did so "always" or "often". Female respondents were more likely than males to join audio and then participate in another activity (62.3% v 37.7%, p = 0.04). CONCLUSIONS: Virtual platforms do not automatically translate into increased learner engagement. Careful design of educational strategies is essential to increase and maintain learner engagement when utilizing virtual surgical education.

Assessing learner engagement with virtual educational events: Development of the Virtual In-Class Engagement Measure (VIEM).

BACKGROUND: The COVID-19 pandemic has necessitated virtual education, but effects on learner engagement are unknown. We developed a virtual in-class engagement measure (VIEM) to assess learner engagement in online surgical education events. METHODS: Using the STROBE, an observer collected tool to document student engagement, as a template an ASE committee workgroup developed the VIEM. The VIEM had two parts: observer assessment and learner self-assessment of engagement. Trained observers collected engagement data from two institutions using the VIEM. Surgical attendings, fellows and residents were observed during virtual learning events. Educator attitudes towards online teaching were also assessed via survey. RESULTS: 22 events with 839 learners were observed. VIEM distinguished between sessions with low and high engagement. 20% of learners pretended to participate. Half of instructors were comfortable with virtual teaching, but only 1/3 believed was as effective as in-person. 2/3 of teachers believed video learners were more engaged than audio learners. CONCLUSIONS: Virtual platforms do not automatically translate into increased engagement. Standard tools such as VIEM may help with assessment of engagement during virtual education.

Computed Tomography of the Mandibles of a Stranded Offshore Killer Whale (Orcinus orca).

A mature, adult female, offshore killer whale (Orcinus orca) was stranded deceased in Portage Bay, Alaska, in October 2015. Full necropsy examination with histopathology was performed. Consistent with previous studies of offshore killer whales, and thought to be a result of their unique elasmobranch diet, all the teeth were significantly abraded and almost flush with the gingival margin. Age was estimated at 30-35 years based on annuli and growth arrest lines in a remaining tooth. The dentate portion of the mandibles were excised en bloc and frozen until imaging could be completed. Radiography and computed tomography revealed lesions consistent with severe abrasion, pulp exposure and evidence of endodontic and/or periodontal disease in nine of the 15 mandibular teeth present (60.0%). Only five (33.3%) teeth were suspected to have been vital at the time of death based on imaging. Lesions were more severe rostrally, with the caudal teeth less affected. Autolysis precluded gingival histopathology and no teeth were analyzed histologically. Necropsy examination revealed a likely multifactorial cause of death, with most significant lesions including the severe chronic periodontal/endodontic disease with abrasion, inanition and emaciation with possible cardiovascular disease. This case highlights the importance of imaging in evaluating periodontal and endodontic status, especially post mortem when other tissues are no longer available, and demonstrates that periodontal and endodontic disease occur naturally in this species and can be a significant cause of morbidity in mature free-ranging killer whales of the offshore ecotype.

Dental and Temporomandibular Joint Pathology of the Grey Wolf (Canis lupus).

Skulls from 392 grey wolves (Canis lupus) were examined macroscopically according to predefined criteria. Two hundred and seven skulls were included in this study, comprised of 124 young adults (59.9%) and 83 adults (40.1%); of these, 65 (31.4%) specimens were from male wolves and 104 (50.3%) were from females, with 38 (18.4%) of unknown sex. Out of 8,694 possible teeth, 8,339 (95.9%) were present for evaluation. Fifty-five teeth (15.5%) were absent congenitally, 30 (8.5%) were lost during life and 270 (76.1%) were lost artefactually post mortem. Skeletal or dental malocclusion was present in 37 specimens (17.9%), with level bite being the most commonly encountered malocclusion. Enamel hypoplasia was present in five skulls (2.4%), affecting eight teeth (0.1%) in total. An abnormal number of roots was found on 23 teeth (0.3%) on 13 skulls (6.3%). Persistent deciduous teeth occurred in two (1.0%) specimens, affecting one (0.01%) tooth each. Fenestration or dehiscence was found associated with 203 teeth (2.4%) in 72 skulls (34.8%). Periodontitis was noted on 115 skulls (55.6%) and 1,000 teeth (11.5%), affecting significantly more adults (n = 63, 75.0%) than young adults (n = 52, 41.9%; P <0.0001). One hundred and sixty-one skulls (77.8%) showed signs of endodontal disease, including attrition or abrasion on 144 skulls (69.6%) and 2,522 teeth (30.2%) and 424 fractured teeth (5.1%) on 103 skulls (49.8%). Both lesions affected significantly more adults than young adults. Overt periapical disease was associated with six teeth (0.1%) distributed across five skulls (2.4%). A carious lesion was present on one tooth (0.01%) of one specimen (0.5%). Lesions consistent with temporomandibular joint (TMJ) osteoarthritis were found in 24 specimens (11.6%), affecting 38 joints (9.2%). Trauma to the skull, such as bite marks, bullet holes or blunt trauma, was noted in 44 skulls (21.2%). The grey wolf and the domestic dog (Canis lupus familiaris) share common dental diseases; however, the proportion and severity may vary. Although the clinical significance of dental and TMJ pathology in the grey wolf remains unknown, based on the impact of these disorders on the domestic dog, the occurrence and severity of these lesions are likely to play an important role in the morbidity and mortality of this wild canid species.

Dental and Temporomandibular Joint Pathology of the North American Brown Bear (Ursus arctos horribilis, Ursus arctos middendorffi and Ursus arctos sitkensis).

Museum specimens (maxillae and mandibles) from 393 North American brown bears (Ursus arctos) from Alaska were examined macroscopically according to predefined criteria and 204 specimens were included in this study. The specimens were acquired between 1905 and 2012. There were 99 specimens (48.5%) from male animals, 87 (42.7%) from female animals and 18 (8.8%) from animals of unknown sex. The ages of the animals ranged from neonate to adult, with 92 adults (45.1%) and 112 young adults (54.9%) included and neonates/juveniles excluded from the study. The number of teeth available for examination was 6,525 (76.2%); 8.6% of teeth were absent artefactually, 0.8% were deemed absent due to acquired tooth loss and 14.5% were absent congenitally. None of the brown bears had supernumerary teeth, persistent deciduous teeth or abnormally formed crowns. Only four of the specimens in the present population were affected by enamel hypoplasia and one specimen contained two mandibular fourth premolar teeth with one root instead of two. All 204 specimens displayed at least some degree of attrition/abrasion, affecting 63% of all teeth, ranging from mild wear of the enamel to deep abrasion associated with pulp exposure. Ten-times more adult than young adult specimens had abrasion causing pulp exposure, while more young adults showed mild attrition/abrasion. Dental fractures were noted in one-third of brown bears and in 3.0% of the total number of present teeth. More adult brown bears had dental fractures than young adults. There were 11 specimens (5.4%) that displayed overt periapical disease, affecting a total of 20 dental alveoli (0.22%), with adults significantly more affected than young adults. Some degree of periodontitis was seen in 145 specimens (71.1%), affecting 13.6% of all dental alveoli. Nearly one-third (29.9%) of skulls displayed skeletal and/or dental malocclusion, most commonly a level bite. Lesions consistent with temporomandibular joint (TMJ) osteoarthritis were found in 27 specimens (13.2%). Caries lesions were discovered in four specimens (2.0%), affecting eight teeth in total. Although the clinical significance of dental and TMJ pathology in the brown bear remains elusive, the occurrence and severity of some of these lesions may play an important role in the morbidity of this species.

Beyond Men, Women, or Both: A Comprehensive, LGBTQ-Inclusive, Implicit-Bias-Aware, Standardized-Patient-Based Sexual History Taking Curriculum.

Introduction: This standardized-patient-based module prepares medical students to take inclusive, comprehensive sexual histories from patients of all sexual orientations and gender identities. Health disparities faced by lesbian, gay, bisexual, transgender, and queer (LGBTQ) people are at least partially the result of inadequate access to health care and insufficient provider training. This module incorporates implicit bias activities to emphasize the important role providers can play in mitigating these disparities through compassionate, competent care. Furthermore, two of the three included cases highlight the negative impact sexual dysfunction can have on emotional well-being. Methods: Over 3 hours, students participate in a 30-minute large-group lecture and three 40-minute small-group standardized patient encounters with debrief. Prework consists of a short video on sexual history taking, assigned readings, and an implicit bias activity. These materials are included in this resource, along with lecture slides, facilitator guide, and standardized patient cases. Though the cases are adaptable to all levels of medical education, this module is designed for second-year and early third-year medical students. Results: Qualitative student evaluations were positive, and postparticipation surveys revealed statistically significant improvement in comfort with their ability to take a sexual history in general, and take one from patients with a differing sexual orientation. Deployed in the second year of our Doctoring curriculum, this module continues to receive positive evaluations. Discussion: Introducing these skills begins to address the curricular deficiencies seen across medical education and lays the foundation for a more competent health care workforce to address the needs of LGBTQ patients.

Dental and Temporomandibular Joint Pathology of the American Black Bear (Ursus americanus).

Museum specimens (maxillae and/or mandibles) from 371 American black bears (Ursus americanus) acquired between 1889 and 2006 were examined macroscopically according to predefined criteria, and 348 were included in this study. Of the 348 specimens, 126 (36.2%) were from male animals, 106 (30.5%) were from female animals and 116 (33.3%) were from animals of unknown sex. Specimen ages ranged from young adult (n = 63, 18.1%) to adult (n = 285, 81.9%), with juveniles excluded from the study. The number of teeth available for examination was 12,019 (82.2%); 7.0% of teeth were absent artefactually, 0.4% were deemed absent due to acquired tooth loss and 9.7% were absent congenitally. In 43 specimens (12.3%), 82 teeth (0.68%) were small vestigial structures with crowns that were flush with the level of surrounding alveolar bone. The remaining teeth (99.3%) were of normal morphology. Only three supernumerary teeth and three instances of enamel hypoplasia were encountered. Persistent deciduous teeth or teeth with an aberrant number of roots were not encountered in any of the specimens. Approximately one-third of the teeth examined (4,543, 37.8%) displayed attrition/abrasion, affecting nearly all of the specimens (n = 338, 97.1%). Incisor and molar teeth accounted for 52.5% and 34.3% of the affected teeth, respectively, with significantly more adults affected than young adults. Dental fractures were noted in 63 bears, affecting 18.1% of specimens and 1.0% of the total number of present teeth. The canine teeth were most often fractured, with adults having significantly more complicated crown fractures of these teeth than young adults. There were 11 specimens (3.2%) that displayed periapical lesions, affecting 12 (0.1%) dental alveoli. There were 179 specimens (51.4%) displaying bony changes indicative of periodontitis, affecting 816 (6.8%) dental alveoli. The proportion of adult bears affected by periodontitis (57.9%) was significantly greater than that of young adults (22.2%). Exactly half of the specimens (n = 174) possessed lesions consistent with mild temporomandibular joint osteoarthritis. The occurrence and severity of the dental pathology encountered in this study may play an important role in the morbidity and mortality of the American black bear.

Dental Pathology of the Hoary Marmot (Marmota caligata), Groundhog (Marmota monax) and Alaska Marmot (Marmota broweri).

Museum specimens (maxillae and mandibles) of the three marmot species occurring in Alaska (Marmota caligata [n = 108 specimens], Marmota monax [n = 30] and Marmota broweri [n = 24]) were examined macroscopically according to predefined criteria. There were 71 specimens (43.8%) from female animals, 69 (42.6%) from male animals and 22 (13.6%) from animals of unknown sex. The ages of animals ranged from neonatal to adult, with 121 young adults (74.4%) and 41 adults (25.3%) included, and 168 excluded from study due to neonatal/juvenile age or incompleteness of specimens (missing part of the dentition). None of the teeth were missing, so 3,564 teeth were available for examination. All teeth were normal in morphology and none was affected by enamel hypoplasia. Two specimens displayed two supernumerary teeth each. One-third of specimens displayed attrition/abrasion and in 38.9% of these the change involved all premolar and molar teeth. The proportion of adult specimens affected by attrition/abrasion was three times as high as young adult specimens. Dental fractures were rare, noted in only two specimens, affecting 0.08% of teeth (n = 3). Periapical disease was also rare, with a striking lesion in one young adult female specimen. Some degree of periodontitis was seen in 26 specimens (16.1%), affecting 4.6% of premolar and molar teeth. The proportion of adults with periodontitis was over three times as high as that for young adults. Five specimens displayed mild malocclusion. Although the clinical significance of dental pathology in the marmot species of Alaska remains elusive, the occurrence and severity of some dental lesions may play an important role in their morbidity and mortality.

Dental and Temporomandibular Joint Pathology of the Polar Bear (Ursus maritimus).

Museum specimens (maxillae and/or mandibles) from 317 polar bears (Ursus maritimus) were examined macroscopically according to predefined criteria and 249 specimens were included in this study. The specimens were acquired between 1906 and 2011. There were 126 specimens (50.6%) from male animals, 93 (37.3%) from female animals and 30 (12.1%) from animals of unknown sex. The ages of the animals ranged from neonate to adult, with 125 adults (50.2%) and 124 young adults (49.8%) included and neonates/juveniles excluded from the study. The number of teeth available for examination was 7,638 (73.5%); 12.3% of teeth were absent artefactually, 0.8% were deemed absent due to acquired tooth loss and 13.4% were absent congenitally. With respect to tooth morphology, 20 teeth (0.26% of available teeth) in 18 specimens (7.2% of available specimens) were small vestigial structures with crowns that were flush with the level of surrounding alveolar bone. One supernumerary tooth and one tooth with enamel hypoplasia were encountered. Persistent deciduous teeth and teeth with an aberrant number of roots were not found. Relatively few teeth (3.7%) displayed attrition/abrasion, 90% of which were the maxillary and mandibular incisor teeth, in 41 polar bears (16.5%). Nearly twice as many adult specimens exhibited attrition/abrasion as those from young adults; significantly more males were affected than females. Dental fractures were noted in 52 polar bears, affecting 20.9% of specimens and 1.3% of the total number of teeth present. More adult polar bears had dental fractures than young adults. There were 21 specimens (8.4%) that displayed overt periapical disease, affecting a total of 24 dental alveoli (0.23%). Some degree of periodontitis was seen in 199 specimens (79.9%); however, only 12.6% of dental alveoli had bony changes indicative of periodontitis. Lesions consistent with temporomandibular joint osteoarthritis (TMJ-OA) were found in 23 specimens (9.2%). TMJ-OA was significantly more common in adults than in young adults and in males than in females. Although the clinical significance of dental and TMJ pathology in the polar bear remains elusive, the occurrence and severity of these lesions may play an important role in the morbidity and mortality of this species.

Dental and Temporomandibular Joint Pathology of the Walrus (Odobenus rosmarus).

Maxillae and/or mandibles from 76 walruses (Odobenus rosmarus) were examined macroscopically according to predefined criteria. The museum specimens were acquired between 1932 and 2014. Forty-five specimens (59.2%) were from male animals, 29 (38.2%) from female animals and two (2.6%) from animals of unknown sex, with 58 adults (76.3%) and 18 young adults (23.7%) included in this study. The number of teeth available for examination was 830 (33.6%); 18.5% of teeth were absent artefactually, 3.3% were deemed to be absent due to acquired tooth loss and 44.5% were absent congenitally. The theoretical complete dental formula was confirmed to be I 3/3, C 1/1, P 4/3, M 2/2, while the most probable dental formula is I 1/0, C 1/1, P 3/3, M 0/0; none of the specimens in this study possessed a full complement of theoretically possible teeth. The majority of teeth were normal in morphology; only five teeth (0.6% of available teeth) were malformed. Only one tooth had an aberrant number of roots and only one supernumerary tooth was encountered. No persistent deciduous teeth were found in any of the young adult or adult specimens, nor were any specimens affected by enamel hypoplasia. The majority of teeth (85.5%) displayed attrition/abrasion. Of the adult and young adult specimens, 90.8% showed some degree of attrition/abrasion on at least one tooth. Tooth fractures were noted in eight walruses, affecting 10.5% of specimens and 1.3% of the total number of teeth, nearly three-quarters of which were maxillary canine teeth (tusks). Three specimens (3.9%), all adult males, displayed overt periapical disease. The majority (99.2%) of dental alveoli did not have bony changes indicative of periodontitis, with only five specimens (6.6%) affected by periodontitis. Lesions consistent with temporomandibular joint osteoarthritis (TMJ-OA) were found in 46 specimens (60.5%) and TMJ-OA was significantly more common in adults than young adults and males than females. Although the clinical significance of dental and TMJ pathology in the walrus remains unknown, the occurrence and severity of these lesions may play an important role in the morbidity and mortality of this species.

Hyponatraemia in Guillain-Barré syndrome revisited.

OBJECTIVES: The objective of this study was to determine the relevance of hyponatraemia in the prognosis of Guillain-Barré syndrome (GBS). MATERIALS AND METHODS: We retrospectively analysed records of 48 consecutive patients with GBS and performed a systematic literature review on frequency/correlates of hyponatraemia in GBS. RESULTS: Hyponatraemia <133 mmol/l was detected in 18/48 of our patients with GBS (37.5%). In 10/18 (55.5%), hyponatraemia occurred post-immunoglobulin therapy. Hyponatraemia correlated with age >50 years (P = 0.011), concurrent malignancy (P = 0.039), diuretic use (P < 0.001), preceding diarrhoea (P = 0.042) and Medical Research Council (MRC) sum score at discharge (MRCSSD) (P = 0.026). Only concurrent malignancy (P < 0.001) and diuretic use (P < 0.001) were independently associated with hyponatraemia. MRCSSD also correlated with MRC sum score on admission (MRCSSA) (P < 0.001), length of hospital stay (P < 0.001), summated compound muscle action potential (P = 0.034) and lowest forced vital capacity (P = 0.001). Only MRCSSA (P = 0.004) and length of hospital stay (P < 0.001) independently predicted MRCSSD. Combining our findings with previous literature indicates comparable frequencies of hyponatraemia in GBS in four of five studies and association with mortality in three of four studies, with an independent link in one. Independent association of hyponatraemia with muscle strength is not demonstrated. CONCLUSION: Hyponatraemia appears of comparable frequency in GBS to that in other diseased cohorts suggesting it is common but non-specific. Hyponatraemia has otherwise been shown to be an independent predictor of death in other disorders and available data indicate the same is also likely in GBS, although this may vary in patient subgroups. Hyponatraemia is, however, not an independent prognostic indicator of neuromuscular weakness severity in GBS.

An update in guillain-barré syndrome.

Guillain-Barré syndrome (GBS) was first described in 1916 (Guillain G, 1916) and is approaching its 100th anniversary. Our knowledge of the syndrome has hugely expanded since that time. Once originally considered to be only demyelinating in pathology we now recognise both axonal and demyelinating subtypes. Numerous triggering or antecedent events including infections are recognised and GBS is considered an immunological response to these. GBS is now considered to be a clinical syndrome of an acute inflammatory neuropathy encompassing a number of subtypes with evidence of different immunological mechanisms. Some of these are clearly understood while others remain to be fully elucidated. Complement fixing antibodies against peripheral nerve gangliosides alone and in combination are increasingly recognised as an important mechanism of nerve damage. New antibodies against other nerve antigens such as neurofascin have been recently described. Research databases have been set up to look at factors associated with prognosis and the influence of intravenous immunoglobulin (IvIg) pharmacokinetics in therapy. Exciting new studies are in progress to examine a possible role for complement inhibition in the treatment of the syndrome.

Osteoarthritis of the temporomandibular joint in southern sea otters (Enhydra lutris nereis).

Museum skull specimens (n = 1,008) of southern sea otters (Enhydra lutris nereis) were examined macroscopically according to defined criteria for the presence, severity and characteristics of temporomandibular joint osteoarthritis (TMJ-OA). The specimens were from stranded young adult to adult animals. Overall, 4.1% of the specimens had findings consistent with TMJ-OA. Of these, 61.0% were from females and 39.0% were from males. In addition, 85.4% of the affected specimens were from adults and 14.6% were from young adults. However, there was no significant association between age and sex with the presence or severity of TMJ-OA. Lesion severity was mild in 41.5%, moderate in 19.5% and severe in 39.0% of affected specimens. The most prominent changes were the presence of osteophytes and subchondral bone defects and porosity. The mandibular condylar process and fossa were affected equally. The lengths of the right and left mandibular heads were significantly associated with age (P = 0.002 and P = 0.003, respectively) and sex (P = 0.0009 and P = 0.001, respectively), but not with the presence of TMJ-OA. The significance of this disease in sea otters remains elusive, but this condition may play an important role in survival of these animals.

The dental pathology of southern sea otters (Enhydra lutris nereis).

Skulls (n = 1,205) of southern sea otters were examined macroscopically according to defined criteria. The museum specimens, acquired from strandings, varied in age from juvenile to adult, with an equal sex distribution. The results from all young adult and adult specimens were pooled according to tooth type. Ninety-two percent of teeth were available for examination, with 6.5% artifactually absent, 0.6% deemed absent due to acquired tooth loss and 0.03% deemed congenitally absent. All teeth were normal in morphology, except for three pairs of fused teeth, including two instances of fused maxillary first incisor teeth. Supernumerary teeth were associated with 97 normal teeth (most commonly maxillary canine teeth) in 68 specimens. At least one persistent deciduous tooth was present in six skulls, two of which were from adults. The majority (94.6%) of alveoli, either with or without teeth, were not associated with bony changes consistent with periodontitis; however, the majority (74.4%) of specimens did have at least one tooth associated with mild periodontitis. The mesial root of the mandibular third premolar tooth was the most common location at which periodontal hard tissue lesions were observed (56.6%). Ten sea otters had lesions consistent with focal enamel hypoplasia. Approximately half of the teeth (52.0%) were abraded; almost all adult specimens (98.1%) contained at least one abraded tooth, while fewer young adults were affected (76.4%). Tooth fractures were uncommon, affecting 1,343 teeth (4.5%). Periapical lesions were associated with 409 teeth (1.3%) in a total of 176 specimens, and these would likely have caused considerable morbidity while the animals were alive.

Predicting the survival of experimental ischaemic small bowel using intraoperative near-infrared fluorescence angiography.

BACKGROUND: Predicting the long-term viability of ischaemic bowel during surgery is challenging. The aim was to determine whether intraoperative near-infrared angiography (NIR-AG) of ischaemic bowel might provide metrics that were predictive of long-term outcome. METHODS: NIR-AG using indocyanine green was performed on 24 pigs before, and after inducing bowel ischaemia to determine the feasibility of NIR-AG for detecting compromised perfusion. Contrast-to-background ratio (CBR) over time was measured in regions of interest throughout the bowel, and various metrics of the CBR-time curve were developed. Sixty rat small bowels, with or without strangulation, were imaged during surgery and on day 3 after operation. CBR metrics and clinical findings were assessed quantitatively for their ability to predict animal survival, histological grade of ischaemic injury and visible necrosis on day 3. RESULTS: In ischaemic pig small bowel, various qualitative and quantitative CBR metrics appeared to correlate with bowel injury as a function of distance from normal bowel. In rats, intraoperative clinical assessment showed high specificity but low sensitivity for predicting outcome on day 3 after operation. Qualitative patterns of the CBR-time curve, such as absence of an arterial inflow peak and presence of a NIR filling defect, resulted in better prediction of survival (90 per cent), histological grade (85 per cent) and visible necrosis on day 3 (92 per cent). CONCLUSION: Survival of ischaemic bowel was predicted by intraoperative NIR-AG with greater accuracy than clinical evaluation alone.

Aggressive and devastating neuropathy: the consequence of untreated slow-onset type 1 diabetes.

Though type 1 diabetes (T1D) is described to be a disease of acute onset, there is strong evidence for a period of subclinical hyperglycaemia leading up to diagnosis. We describe two clinical cases with a prolonged and insidious onset of T1D, where neurological complications were present at the time of diagnosis. In both, there was an initial rapid and debilitating progression in neurological as well as other microvascular complications, but with a subsequent stabilization in complications over the next few years. These rare and unusual cases illustrate the variable nature of the natural history of T1D as well as its microvascular complications.

Guillain-Barré syndrome: clinical variants and their pathogenesis.

Numerous clinical subtypes of Guillain-Barré syndrome have been described over the century since the original description of the syndrome. These variants of Guillain-Barré syndrome are discussed and their immunological pathogenesis reviewed.

McArdle disease: a clinical review.

METHODS: The clinical phenotype of 45 genetically confirmed McArdle patients is described. RESULTS: In the majority of patients (84%), the onset of symptoms was from early childhood but diagnosis was frequently delayed until after 30 years of age. Not all patients could recognise a second wind although it was always seen with exercise assessment. A history of myoglobinuria was not universal and episodes of acute renal failure had occurred in a minority (11%). The condition does not appear to adversely affect pregnancy and childbirth. Clinical examination was normal in most patients, muscle hypertrophy was present in 24% and mild muscle wasting and weakness were seen only in patients over 40 years of age and was limited to shoulder girdle and axial muscles. The serum creatine kinase was elevated in all but one pregnant patient. Screening for the mutations pArg50X (R50X) and pGly205Ser (G205S) showed at least one mutated allele in 96% of Caucasian British patients, with an allele frequency of 77% for pArg50X in this population. A 12 min walking test to evaluate patients is described. CONCLUSION: The results demonstrated a wide spectrum of severity with the range of distance walked (195-1980 m); the mean distance walked was 512 m, suggesting significant functional impairment in most patients.

Effect of chlorhexidine gluconate on the skin integrity at PICC line sites.

OBJECTIVE: To determine the effects of chlorhexidine gluconate (CHG) on skin inflammation and stratum corneum barrier integrity at peripherally inserted central catheter (PICC) sites among patients in the neonatal intensive care setting. STUDY DESIGN: In a within-subject design, PICC sites with CHG plus semipermeable dressing (PICC) were compared with contralateral dressing sites and untreated controls among 40 neonates (gestational age 32.1+/-4.7) at weekly dressing changes, using quantitative measures of skin erythema, dryness and barrier integrity (transepidermal water loss, TEWL). Data were analyzed using analysis of variance and linear mixed methods. RESULTS: At week 1, all three sites differed for erythema with the highest value indicating poorer skin condition at the PICC site. Dressing-site erythema was higher than the untreated control. Dryness and TEWL were higher, indicating poorer skin integrity, for the PICC site than either the dressing or the control. After 2 weeks, erythema and dryness scores were higher for the PICC site than the dressing and control skin. By week 3, scores were comparable for PICC and dressing sites and both were higher than the control for erythema and dryness. After 3 weeks, PICC skin TEWL was higher than both dressing and control and they did not differ from each other. CONCLUSION: The dressings used to secure PICC lines contribute to the observed skin compromise at CHG-treated skin sites and may affect skin barrier development in similar populations of neonates.