Hard to Swallow Results.

The American Neurogastroenterology and Motility Society (ANMS) proposed quality measures (QMs) for performance and interpretation of esophageal manometry (EM). We implemented a quality improvement (QI) study at a large community hospital to assess and improve procedural adherence and interpretation of EM studies based on the ANMS QMs using the Chicago Classification 3.0 (CC) Guidelines. For pre-intervention, three motility independent reviewers reinterpreted 60 EM studies conducted by community gastroenterologists without Tier II-III motility training from October to December 2018 for compliance with pre-procedural, procedural, and data interpretation ANMS QMs. In December 2018, we developed a pre-procedural form, educated nurses on EM procedural compliance, and provided preliminary pre-intervention results to gastroenterologists along with literature utilizing the CC 3.0 Guidelines. For post-intervention, we reinterpreted 54 EM studies from January to August 2019 and investigated whether they met QMs for data interpretation with respect to the CC Guidelines and resulted in appropriate treatment. We found a statistically significant improvement in procedural compliance among nursing staff for 30 s of swallows (76% post-intervention versus 12% pre-intervention, p < 0.001) and 7 evaluable swallows (94% post-intervention versus 53% pre-intervention, p < 0.001). However, quality metrics within data interpretation by physicians post-intervention showed mixed results. An incorrect diagnosis was made in 50% (n = 27)) of studies with 72% (n = 39) having at least one missing item based on the CC. The most missed diagnosis was fragmented peristalsis (30%, n = 29). Among the 39% (n = 21) of surgery referrals, 24% (n = 5) were incorrectly referred. Our study shows poor data interpretation by community gastroenterologists without formal motility training despite adequate performance by nursing staff. This further supports the need for a national ANMS certification process for formal HRM education.

Comparing the epidemiology of hospital-acquired methicillin-resistant Staphylococcus aureus clone groups in Alberta, Canada.

Patients with methicillin-resistant Staphylococcus aureus (MRSA) clones, which were traditionally seen in the community setting (USA400/CMRSA7 and USA300/CMRSA10), are often identified as hospital-acquired (HA) infections using Infection Prevention and Control (IPC) surveillance definitions. This study examined the demographics and healthcare risk factors of patients with HA-MRSA to help understand if community MRSA clones are from a source internal or external to the hospital setting. Despite USA300/CMRSA10 being the predominant clone in Alberta, hospital clones (USA100/CMRSA2) still dominated in the acute care setting. In the Alberta hospitalized population, patients with USA400/CMRSA7 and USA300/CMRSA10 clones were significantly younger, had fewer comorbidities, and a greater proportion had none or ambulatory care-only healthcare exposure. These findings suggest that there are two distinct populations of HA-MRSA patients, and the patients with USA400/CMRSA7 and USA300/CMRSA10 clones identified in hospital more greatly resemble patients affected by those clones in the community. It is possible that epidemiological assessment overidentifies HA acquisition of MRSA in patients unscreened for MRSA on admission to acute care.

International survey of cochlear implant candidacy.

BACKGROUND: The goal of this work was to determine international differences in candidacy based on audiometric and speech perception measures, and to evaluate the information in light of the funding structure and access to implants within different countries. METHOD: An online questionnaire was circulated to professionals in 25 countries. There were 28 respondents, representing the candidacy practice in 17 countries. RESULTS: Results showed differences in the funding model between countries. Unilateral implants for both adults and children and bilateral implants for children were covered by national funding in approximately 60% of countries (30% used medical insurance, and 10% self-funding). Fewer countries provided bilateral implants routinely for adults: national funding was available in only 22% (37% used medical insurance and 41% self-funding). Main evolving candidacy areas are asymmetric losses, auditory neuropathy spectrum disorders and electro-acoustic stimulation. For countries using speech-based adult candidacy assessments, the majority (40%) used word tests, 24% used sentence tests, and 36% used a mixture of both. For countries using audiometry for candidacy (70-80% of countries), the majority used levels of 75-85 dB HL at frequencies above 1 kHz. The United Kingdom and Belgium had the most conservative audiometric criteria, and countries such as Australia, Germany, and Italy were the most lenient. Countries with a purely self-funding model had greater flexibility in candidacy requirements.

A case of linear epidermal naevus presenting as genital warts--a cautionary tale.

Linear epidermal naevus (LEN) in the genital area is quite rare. It may present at birth or appear later on in life, in infancy or childhood and occasionally for the first time in adult life. There are several variants of epidermal naevi (EN), which, to the less experienced, can be mistaken for warts. When extensive, it can be associated with abnormalities in other organ systems (epidermal naevus syndrome). The definitive treatment of LEN is surgical ablation with excision of underlying dermis, but this frequently leads to scarring. Laser therapy is an alternative treatment modality and good results have been shown. We report an unusual case of LEN in the genital area in a 60-year-old man presenting as genital warts.

Decision making and memory: a critique of Juslin and Olsson's (1997) sampling model of sensory discrimination.

P. Juslin and H. Olsson's (1997) distinction between Thurstonian and Brunswikian uncertainty is examined and their sampling model of sensory discrimination analyzed as a representative of the class of memoryless decision processes. The separate characteristics and combined behavior of 4 main components of the model are explored: (a) the basic decision process, (b) the assumption of deadline responding, (c) the moving window model of memory, and (d) the hypothesized basis for confidence. It is argued that grafting a moving window memory onto a memoryless decision process has several undesirable consequences. Moreover, the suggested basis for confidence leads to predictions that are counterintuitive and unsupported by empirical evidence. It is concluded that the window-sampling model is a maladapted combination of inappropriate elements, which is implausible as a model of decision making, memory, or confidence, in sensory discrimination.

A technique for measuring regional bone mineral density in human lumbar vertebral bodies.

A method for measuring the regional bone mineral density (rBMD) in human lumbar vertebral bodies using a series of contiguous computed tomography images, each 1 mm thick, is fully described. The technique has a sample volume of 0.004 cm3, a sample spacing of 0.8 X 0.8 X 1.0 mm, and results in a bone marrow radiation dose of 1.59 to 2.75 rads (0.016-0.028 Gy). The use of physical density (mg/cm3) is introduced and the measurement noise (0.7-1.3%), accuracy (2.7%), and serial precision (0.2%) have been evaluated in vitro using appropriate phantoms. The corresponding percentage errors for accuracy and precision relative to K2HPO4 concentration were 6.9% and 2.0%, respectively. A multiple region density measurement is described and evaluated.

The relative role of beats and combination tones in determining the shapes of masking patterns at 2 kHz: I. Normal-hearing listeners.

Masking patterns for a 2-kHz sinusoidal masker at 45, 65 or 85 dB SPL were measured for three normal-hearing subjects, using a 3-AFC method with feedback (condition 1). The patterns showed distinct irregularities, particularly at the highest masker level. In condition 2, a lowpass noise was added to mask combination tones. The noise increased thresholds mainly for the 85 dB masker, for signal frequencies of 2.3-3.0 kHz. In condition 3, a pair of high-frequency tones ('modulation detection interference (MDI) tones') was used to introduce beats at the same rate as produced by the interaction of the masker and signal. Thresholds were higher than for condition 1, particularly for signal frequencies adjacent to the masker frequency. In condition 4, the lowpass noise was presented simultaneously with the MDI tones. Thresholds were well predicted as a combination of the effects of the lowpass noise and the MDI tones. In condition 5, a pair of low-frequency MDI tones was added to the masker. The thresholds had the same overall pattern as in condition 4. We conclude that the shapes of masking patterns measured using a 2-kHz masker are influenced by the detection of beats for masker-signal frequency separations up to at least 300 Hz and by the detection of combination tones for separations between 300 and 1000 Hz.

Effect of 24 months of recombinant growth hormone on height and body proportions in SHOX haploinsufficiency.

Leri-Weill syndrome (LWS) is a skeletal dysplasia with mesomelic short stature, bilateral Madelung deformity (BMD) and SHOX (short stature homeobox-containing gene) haploinsufficiency. The effect of 24 months of recombinant human growth hormone (rhGH) therapy on the stature and BMD of two females with SHOX haploinsufficiency (demonstrated by fluorescence in situ hybridisation) and LWS was evaluated. Both patients demonstrated an increase in height standard deviation score (SDS) and height velocity SDS over the 24 months of therapy. Patient 1 demonstrated a relative increase in arm-span and upper segment measurements with rhGH while patient 2 demonstrated a relative increase in lower limb length. There was appropriate advancement of bone age, no adverse events and no significant deterioration in BMD. In this study, 24 months of rhGH was a safe and effective therapy for the disproportionate short stature of SHOX haploinsufficiency, with no clinical deterioration of BMD.

Familial growth and skeletal features associated with SHOX haploinsufficiency.

This study was designed to determine the intrafamilial effect of SHOX haploinsufficiency on stature, by comparing the growth and phenotype of 26 SHOX haploinsufficient individuals with 45 relatives and population standards. It confirmed that SHOX haploinsufficiency leads to growth restriction from birth to final height. Compared to unaffected siblings, the SHOX haploinsufficient cohort was 2.14 SDS (3.8 cm) shorter at birth and 2.1 SDS shorter through childhood. At final height females were 2.4 SDS (14.4 cm) shorter and males 0.8 SDS (5.3 cm) shorter than normal siblings. The family height analysis suggests that the effect of SHOX haploinsufficiency on growth may have been previously underestimated at birth and overestimated in males at final height. SHOX haploinsufficiency leads to short arms in 92%, bilateral Madelung deformity in 73% and short stature in 54%. Females were more severely affected than males. We conclude that SHOX is a major growth gene and that mutations are associated with a broad range of phenotype.

Easy orientation of the cut ends for nerve suture.

A technique for orienting cut nerve ends for suturing is presented. A mirror is used to reflect one end, making its image identical to that of the other end--so that proper orientation is greatly facilitated.

Clinodactyly of the little finger: a simple operative technique for reversal of the growth abnormality.

The traditional approach to clinodactyly has been either to accept the deformity or to perform an osteotomy. As an alternative to osteotomy, a resection of the mid-zone of the continuous epiphysis along with the underlying physis and its replacement by a fat graft (physiolysis) is simpler and allows further growth in the phalanx. Twelve of these operations have been reviewed with a maximum follow-up of six years. Excellent functional and cosmetic results have been obtained. The renewed growth is manifest not only by an increase in length, but also by a decrease in the angular deformity and improvement of the articular surfaces. Osteotomy at maturity has only been necessary in one patient.

Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion.

The majority of cases of Madelung deformity are caused by hereditary dyschondrosteosis at the wrist. The principal lesion in the ulnar zone of the distal radial physis retards growth asymmetrically, especially in late childhood. Resection of this zone and its replacement with autologous fat (Langenskiöld procedure, or physiolysis) restores growth and minimizes deformity. The resection of an abnormal ligament tethering the lunate proximally may assist carpal advancement. A series of 17 patients (24 wrists) treated over a 12-year period is presented, with sufficient follow-up for evaluation of 11 patients (15 wrists). The results of this prophylactic procedure are encouraging, and, if it is performed early, the authors believe that Madelung deformity may be preventable, or at least controllable.

Memory capacity and intelligence: novel techniques for evaluating rival models of a fundamental information-processing mechanism.

Data from a fundamental cognitive task in which participants discriminate the relative frequency of visual or auditory binary stimuli were examined. Accuracy on this task correlates well with psychometric intelligence. The experimental paradigm is highly tractable, lending itself to rigorous analyses of precisely defined simulation models. Numerous models are evaluated, using multiple comparisons between response patterns of individual (and pooled) participants and predictive measures based on simulations for each trial sequence. Implications for theoretical accounts of short-term memory, discrimination, and absolute judgement as well as the measurement of individual differences in cognitive ability, are discussed. The results suggest a reinterpretation of memory capacity and support a new kind of model (with a single estimable parameter) in which discrete, valued units of information are stochastically displaced by further input.

Histopathological analysis of Leri-Weill dyschondrosteosis: disordered growth plate.

Leri-Weill syndrome (LWS) is a dominant (pseudoautosomal) skeletal dysplasia with mesomelic short stature and bilateral Madelung deformity, due to dyschondrosteosis of the distal radius. It results from the loss of one copy of the Short Stature Homeobox Gene (SHOX) from the tip of the short arm of the X or Y chromosome. SHOX molecular testing enabled us to evaluate the histopathology of the radial physis in LWS patients with a documented SHOX abnormality. A widespread disorganisation of physeal anatomy was revealed with disruption of the normal parallel columnar arrangement of chondrocytes. Tandem stacking of maturing chondrocytes within columns was replaced by a side-by-side arrangement. The presence of hypertrophic osteoid with micro-enchondromata in the radial metaphysis suggests abnormal endochondral ossification. The Vickers' ligament was confirmed to blend with the triangular fibrocartilage complex (TFCC). This histopathological study demonstrates that the zone of dyschondrosteosis in LWS is characterised by marked disruption of normal physeal chondrocyte processes and that a generalised physeal abnormality is present.

Cochlear-implanted children from homes where English is an additional language: findings from a recent audit in one London centre.

A 5-year retrospective audit of demographic, audiological, and other records of 147 children implanted at one London centre was conducted. The aim was to detail the number of children implanted, with a specific focus on children from families with English as an additional language (EAL), and to compare these children with children from monolingual English-speaking families on a variety of characteristics known to affect paediatric cochlear implant outcomes. In all, 28% of children were from families where English is an additional language, with 15 different languages recorded. There were no differences between EAL and English-speaking children with respect to age of implantation; bilateral versus unilateral implants or hearing levels in better ear. There were differences between these groups in aetiology, in the occurrence of additional needs, and in educational placements. Information about speech and language outcomes was difficult to gather. Conclusions indicate the need for more detailed record-keeping especially about children's home languages for purposes of planning intervention and for the inclusion of children with EAL in future studies.