Five years of scanning electrochemical cell microscopy (SECCM): new insights and innovations.

Scanning electrochemical cell microscopy (SECCM) is a nanopipette-based technique which enables measurement of localised electrochemistry. SECCM has found use in a wide range of electrochemical applications, and due to the wider uptake of this technique in recent years, new applications and techniques have been developed. This minireview has collected all SECCM research articles published in the last 5 years, to demonstrate and celebrate the recent advances, and to make it easier for SECCM researchers to remain well-informed. The wide range of SECCM applications is demonstrated, which are categorised here into electrocatalysis, electroanalysis, photoelectrochemistry, biological materials, energy storage materials, corrosion, electrosynthesis, and instrumental development. In the collection of this library of SECCM studies, a few key trends emerge. (1) The range of materials and processes explored with SECCM has grown, with new applications emerging constantly. (2) The instrumental capabilities of SECCM have grown, with creative techniques being developed from research groups worldwide. (3) The SECCM research community has grown significantly, with adoption of the SECCM technique becoming more prominent.

Optimizing Amorphous Molybdenum Sulfide Thin Film Electrocatalysts: Trade-Off between Specific Activity and Microscopic Porosity.

Amorphous molybdenum sulfide (a-MoSx) is a promising candidate to replace noble metals as electrocatalysts for the hydrogen evolution reaction (HER) in electrochemical water splitting. So far, understanding of the activity of a-MoSx in relation to its physical (e.g., porosity) and chemical (e.g., Mo/S bonding environments) properties has mostly been derived from bulk electrochemical measurements, which provide limited information about electrode materials that possess microscopic structural heterogeneities. To overcome this limitation, herein, scanning electrochemical cell microscopy (SECCM) has been deployed to characterize the microscopic electrochemical activity of a-MoSx thin films (ca. 200 nm thickness), which possess a significant three-dimensional structure (i.e., intrinsic porosity) when produced by electrodeposition. A novel two-step SECCM protocol is designed to quantitatively determine spatially resolved electrochemical activity and electrochemical surface area (ECSA) within a single, high-throughput measurement. It is shown for the first time that although the highest surface area (e.g., most porous) regions of the a-MoSx film possess the highest total activity (measured by the electrochemical current), they do not possess the highest specific activity (measured by the ECSA-normalized current density). Instead, the areas of highest specific activity are localized at/around circular structures, coined "pockmarks", which are tens to hundreds of micrometers in size and ubiquitous to a-MoSx films produced by electrodeposition. By coupling this technique with structural and elemental composition analysis techniques (scanning electron microscopy, energy-dispersive X-ray spectroscopy, and X-ray photoelectron spectroscopy) and correlating ECSA with activity and specific activity across SECCM scans, this work furthers the understanding of structure-activity relations in a-MoSx and highlights the importance of local measurements for the systematic and rational design of thin film catalyst materials.

Automated Breast Density Assessment in MRI Using Deep Learning and Radiomics: Strategies for Reducing Inter-Observer Variability.

BACKGROUND: Accurate breast density evaluation allows for more precise risk estimation but suffers from high inter-observer variability. PURPOSE: To evaluate the feasibility of reducing inter-observer variability of breast density assessment through artificial intelligence (AI) assisted interpretation. STUDY TYPE: Retrospective. POPULATION: Six hundred and twenty-one patients without breast prosthesis or reconstructions were randomly divided into training (N = 377), validation (N = 98), and independent test (N = 146) datasets. FIELD STRENGTH/SEQUENCE: 1.5 T and 3.0 T; T1-weighted spectral attenuated inversion recovery. ASSESSMENT: Five radiologists independently assessed each scan in the independent test set to establish the inter-observer variability baseline and to reach a reference standard. Deep learning and three radiomics models were developed for three classification tasks: (i) four Breast Imaging-Reporting and Data System (BI-RADS) breast composition categories (A-D), (ii) dense (categories C, D) vs. non-dense (categories A, B), and (iii) extremely dense (category D) vs. moderately dense (categories A-C). The models were tested against the reference standard on the independent test set. AI-assisted interpretation was performed by majority voting between the models and each radiologist's assessment. STATISTICAL TESTS: Inter-observer variability was assessed using linear-weighted kappa (κ) statistics. Kappa statistics, accuracy, and area under the receiver operating characteristic curve (AUC) were used to assess models against reference standard. RESULTS: In the independent test set, five readers showed an overall substantial agreement on tasks (i) and (ii), but moderate agreement for task (iii). The best-performing model showed substantial agreement with reference standard for tasks (i) and (ii), but moderate agreement for task (iii). With the assistance of the AI models, almost perfect inter-observer variability was obtained for tasks (i) (mean κ = 0.86), (ii) (mean κ = 0.94), and (iii) (mean κ = 0.94). DATA CONCLUSION: Deep learning and radiomics models have the potential to help reduce inter-observer variability of breast density assessment. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY: Stage 1.

Assessment of CD200R Activation in Combination with Doxycycline in a Model of Melioidosis.

Antimicrobial resistance continues to be a global issue. Pathogens, such as Burkholderia pseudomallei, have evolved mechanisms to efflux certain antibiotics and manipulate the host response. New treatment strategies are therefore required, such as a layered defense approach. Here, we demonstrate, using biosafety level 2 (BSL-2) and BSL-3 in vivo murine models, that combining the antibiotic doxycycline with an immunomodulatory drug that targets the CD200 axis is superior to antibiotic treatment in combination with an isotype control. CD200-Fc treatment alone significantly reduces bacterial burden in lung tissue in both the BSL-2 and BSL-3 models. When CD200-Fc treatment is combined with doxycycline to treat the acute BSL-3 model of melioidosis, there is a 50% increase in survival compared with relevant controls. This benefit is not due to increasing the area under the concentration-time curve (AUC) of the antibiotic, suggesting the immunomodulatory nature of CD200-Fc treatment is playing an important role by potentially controlling the overactive immune response seen with many lethal bacterial infections. IMPORTANCE Traditional treatments for infectious disease have focused on the use of antimicrobial compounds (e.g. antibiotics) that target the infecting organism. However, timely diagnosis and administration of antibiotics remain crucial to ensure efficacy of these treatments especially for the highly virulent biothreat organisms. The need for early antibiotic treatment, combined with the increasing emergence of antibiotic resistant bacteria, means that new therapeutic strategies are required for organisms that cause rapid, acute infections. Here, we show that a layered defense approach, where an immunomodulatory compound is combined with an antibiotic, is better than an antibiotic combined with a relevant isotype control following infection with the biothreat agent Burkholderia pseudomallei. This approach has the potential to be truly broad spectrum and since the strategy includes manipulation of the host response it's application could be used in the treatment of a wide range of diseases.

A Cross-Sectional Survey Reporting on the Value of Patient-Centered Ostomy Programs: A Smooth Transition After Ostomy Surgery.

PURPOSE: The purpose of the study was to evaluate a postsurgical ostomy patient support program in regard to postsurgical experience, education, skin care, pouching system and accessory use, insurance issues, supplier assistance, hospital readmission status, and the benefit of multiple interactions. DESIGN: Cross-sectional design. SUBJECTS AND SETTING: Potential respondents were randomly selected from a group of adults 18 years or older who underwent ostomy surgery within 6 months prior to survey completion. Nine hundred seventy-one individuals met inclusion criteria, and 297 were selected for analysis, based on having 1 or more program interactions. Data were collected between the second and third quarters of 2020. All participants were residents of the United States. METHODS: Participants were contacted by e-mail containing an invitation to participate in a survey; the e-mail also described the intent of the survey. Survey distribution was conducted by the sponsor and linked to a third-party survey management organization for compilation. The survey questionnaire, developed specifically for the purpose of this study, comprised 73 items that queried demographic and pertinent clinical data, participation in an industry-sponsored patient support program, and their post-hospital discharge experiences including unplanned health care provider visits, emergency department visits, and hospital readmissions. RESULTS: Eighty-three percent (n = 237) of respondents did not have postsurgical ostomy-related emergency department visits, 75% (n = 223) did not have related unplanned physician visits, and 90% (n = 268) did not have related hospitalizations. Participants with 2 or more interactions were more likely to contact a program representative for issues of stoma care, leakage and skin care, ostomy products/accessories, and supplier issues than their single-interaction counterparts. CONCLUSIONS: Study findings suggest that patients with new ostomies benefited from engagement in an industry-sponsored patient support program. The benefit appears to arise from personal interactions and respondents; 2 or more interactions were shown to have greater benefit than a single interaction.

Transfeminine Gender Confirmation Surgery with Penile Inversion Vaginoplasty: An Initial Experience.

To detail the early experience with and results of a transfeminine (TF) genital reconstruction at an established plastic surgery practice in Western New York. METHODS: Between June 2016 and June 2019, 30 patients underwent penile inversion vaginoplasty for TF gender reassignment. All patients fulfilled World Professional Association for Transgender Health and NY State criteria for reassignment surgery. All surgeries were carried out at a large, government-owned tertiary care center. RESULTS: There were 30 patients in this retrospective study, with a mean age of 37 years (SD 5.4) and a mean body mass index of 27.3 kg/m2 (SD 3.2 kg/m2). Nineteen patients never smoked, 4 were former smokers, and 7 were current smokers. Primary surgery was an orchiectomy and modified single-stage penile inversion vaginoplasty. Mean operative time was 6.0 hours. Mean initial hospital stay was 8.2 days. Three of the 30 (10%) patients required transfusion. There were 6 (20%) complications. Three complications (10%) required reoperation: 1 patient for wound dehiscence on postoperative day 7, 1 for rectal perforation identified on postoperative day 10, and 1 for urethrovaginal fistula. All complications were addressed without sequalae. Twenty of the 30 (66%) patients have undergone revision surgery. Indications for revision were prolapse correction/deepening, labiaplasty, clitoral hood construction/revision, meatal asymmetry, urinary fistula repair, and posterior vaginal flap revision. Twenty-one of 28 (75%) revisions were outpatient surgeries. There were no complications from these procedures. Overall satisfaction via survey was 92% (24 respondents). CONCLUSIONS: TF gender reassignment is a novel, challenging set of procedures for the specialty of plastic surgery. With appropriate consideration and technique, penile inversion vaginoplasty is a safe, effective means of achieving this goal.

Presurgical induction chemotherapy for squamous cell carcinoma of the tonsil.

OBJECTIVES/HYPOTHESIS: The indications for and efficacy of induction chemotherapy in the management of squamous cell carcinoma of the head and neck is controversial. With the advent of human papillomavirus (HPV)-related cancers, survival has improved significantly. Here we present a group of patients with tonsil cancer treated with induction chemotherapy followed by surgery. STUDY DESIGN: Retrospective cohort study. METHODS: Thirty-eight patients with tonsil cancer were treated with induction chemotherapy, consisting of cisplatin and docetaxel, followed by neck dissection and radical tonsillectomy. Twenty-six patients were HPV+, 28 were nonsmokers or long-term former smokers, and 28 were T1/T2. Fourteen patients required postoperative chemoradiotherapy (CRT). Median follow-up time was 4.1 years. RESULTS: A complete response to induction chemotherapy was achieved in 45% (17) of patients. In total, 76% (29/38) of patients were successfully treated: 53% (20/38) with chemotherapy and surgery alone, and 24% (9/38) required postoperative CRT. Almost 90% (23/26) of HPV+ and half (6/12) of HPV- patients are no evidence of disease (NED). HPV status is a significant prognostic factor (P = .02). Only 38% (5/13) of current smokers were NED compared to 96% (24/25) of nonsmokers (P = .0002). All HPV+ nonsmokers (20/20) were NED at last follow-up. CONCLUSIONS: In this study, the primary driver of prognosis was smoking status. HPV status and T stage were also important. The prognosis for HPV+ nonsmokers is extremely good; most likely regardless of treatment. Treatment failures have a poor chance of salvage, irrespective of treatment type. With the major exception of HPV- smokers, induction chemotherapy followed by surgery with selective CRT is a viable treatment option for tonsil cancer. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1206-1211, 2020.

Sentinel lymph node biopsy for management of the N0 neck in oral cavity squamous cell carcinoma.

BACKGROUND AND OBJECTIVES: The management of the clinically N0 (cN0) neck is controversial for early stage squamous cell carcinoma of the oral cavity (OSCC). This paper represents a single institution series analyzing the efficacy of sentinel lymph node biopsy (SNB) for early stage oral cavity cancers. METHODS: From 2005 to 2017, 108 patients with cN0 OSCC were treated with primary resection and SNB. Patients with positive biopsy results proceeded to neck dissection with or without adjuvant chemoradiotherapy. Mean follow-up for the entire cohort was 50.8 months (range: 8-147 months). Clinically, 56 patients were T1N0, 49 patients were T2N0, and three patients were T3N0 or greater. RESULTS: Disease-specific survival was 93% within the entire cohort. Sentinel lymph nodes were identified in 95.4% of patients. Twenty one patients had a positive biopsy. There were seven false-negative biopsies. The overall rate of nodal disease was 26%. Accuracy of node biopsy was 93%, with sensitivity of 75%, and negative predictive value of 91%. Recurrence rate was 19% (20/108), with an overall survival of 60% in this subgroup. CONCLUSION: SNB is a safe, effective, and well tolerated method for staging cN0 OSCC.

The Burden of Peristomal Skin Complications on an Ostomy Population as Assessed by Health Utility and the Physical Component Summary of the SF-36v2®.

BACKGROUND: Body-altering surgery may affect perceptions of one's self. For those with abdominal stoma surgeries, altered perceptions amplified by peristomal skin condition can increase health burdens. OBJECTIVES: To assess health utility and health-related quality of life in an adult US ostomy sample in the presence of three levels of peristomal skin condition: intact, moderately compromised, and severely compromised. METHODS: The short form 36 health survey version 2, a generic health survey incorporating the six-dimensional health state short form preference-based utility index, was chosen to assess the sample. Analysis of covariance adjusted for age and time from surgery was used. RESULTS: The six-dimensional health state short form utilities for those with intact skin and physical component summary (PCS) levels indicating no physical limitations varied significantly from those with severely compromised skin and indicating the greatest degree of physical limitation (0.833 vs. 0.527). Peristomal skin condition decreases were associated with health utility decreases across all levels of the PCS. Because peristomal skin conditions are intermittent, the analysis presents quality-adjusted life-days (QALDs) per month. Ostomates with intact skin and PCS levels indicating no physical limitations demonstrated significant differences from those with severe skin condition and indicating the greatest degree of physical limitations (26.5 d/mo vs. 15.8 d/mo). As peristomal skin condition worsened, QALDs decreased across all levels of the PCS. A minimally important expected value of health was estimated to be an increase of 2.18 QALDs/mo. CONCLUSIONS: Successful treatment from a clinical perspective is more than the elimination of conditions-it is also a return of quality time to an individual.

Torsade de pointes arrhythmias arise at the site of maximal heterogeneity of repolarization in the chronic complete atrioventricular block dog.

AIMS: The chronic complete atrioventricular block (CAVB) dog is highly sensitive for drug-induced torsade de pointes (TdP) arrhythmias. Focal mechanisms have been suggested as trigger for TdP onset; however, its exact mechanism remains unclear. In this study, detailed mapping of the ventricles was performed to assess intraventricular heterogeneity of repolarization in relation to the initiation of TdP. METHODS AND RESULTS: In 8 CAVB animals, 56 needles, each containing 4 electrodes, were inserted in the ventricles. During right ventricular apex pacing (cycle length: 1000-1500 ms), local unipolar electrograms were recorded before and after administration of dofetilide to determine activation and repolarization times (RTs). Maximal RT differences were calculated in the left ventricle (LV) within adjacent electrodes in different orientations (transmural, vertical, and horizontal) and within a square of four needles (cubic dispersion). Dofetilide induced TdP in five out of eight animals. Right ventricle-LV was similar between inducible and non-inducible dogs at baseline (327 ± 30 vs. 345 ± 17 ms) and after dofetilide administration (525 ± 95 vs. 508 ± 15 ms). All measurements of intraventricular dispersion were not different at baseline, but this changed for horizontal (206 ± 20 vs. 142 ± 34 ms) and cubic dispersion (272 ± 29 vs. 176 ± 48 ms) after dofetilide: significantly higher values in inducible animals. Single ectopic beats and the first TdP beat arose consistently from a subendocardially located electrode terminal with the shortest RT in the region with largest RT differences. CONCLUSION: Chronic complete atrioventricular block dogs susceptible for TdP demonstrate higher RT differences. Torsade de pointes arises from a region with maximal heterogeneity of repolarization suggesting that a minimal gradient is required in order to initiate TdP.

Quality of Life in Persons Living With an Ostomy Assessed Using the SF36v2: Mental Component Summary: Vitality, Social Function, Role-Emotional, and Mental Health.

PURPOSE: The purpose of this study was to assess the Mental Health Component of health-related quality of life (HRQOL) in community-dwelling persons with ostomies residing in the United States. DESIGN: Cross-sectional descriptive study. SUBJECTS AND SETTING: Two thousand three hundred twenty-nine participants completed the survey for a response rate of 14.9% and a margin of error of 2.03%. Study respondents were geographically distributed throughout the United States, representing all 50 states. Fifty-three percent of study respondents were male. Respondents had a median age of 65 years. Forty percent have colostomies, 44% are living with ileostomies, and 13% have urostomies. The remaining 3% are living with multiple stomas or they indicated that they were uncertain as to the type of stoma. INSTRUMENT: The SF36v2 was used to assess HRQOL. This instrument was selected because it has the ability to measure HRQOL in a target population and it allows comparison with the general population. METHODS: Potential participants were randomly selected from an electronic database of 15,591 persons with ostomies. They were contacted by e-mails and provided with an electronic nontransferable link to the survey. This is a secondary analysis of findings from the Mental Component Summary (MCS) of the SF36v2. RESULTS: Persons who have undergone ostomy surgery did not score as well as the general population when components of the MCS were compared. While overall differences were identified, they differed based on age and cumulative MCS score levels. Analysis of individuals found to have significant impairment in MCS scores (cumulative soccer <40) tended to report less negative response than persons with comparable scores in the general population. Analysis also revealed that respondents aged 55 years or older had mental component domain scores that remained with the normal range of the general population. In contrast, participants who were younger than 55 years had lower scores that the general population. CONCLUSIONS: The overall MCS score of community-dwelling persons living with ostomies as lower than scores generated from the general population. However, these findings varied based on age and cumulative MCS score.

Quality of Life in US Residents With Ostomies Assessed via the SF36v2: Role-Physical, Bodily Pain, and General Health Domain.

PURPOSE: The purpose of this study was to investigate the physical-role, bodily pain, and general health domains of health-related quality of life in the adult with ostomies living in the United States and to compare findings to population-based norms established using the Short Form 36 version 2 (SF36v2). SUBJECTS AND SETTING: Study participants were identified by randomly sampling from proprietary databases. Within the United States, 2,329 completed the survey for a response rate of 14.9% and a margin of error of 2.03%. METHODS: Secondary analysis of data from a propietary database. RESULTS: A greater proportion of persons living with an ostomy experienced limitations in the physical component summary levels of the SF36v2, indicating greater impairment than the general population. In contrast, no differences in pain interference with work or other activities reductions were found between the 2 groups. Persons with ostomies reported similar levels of general health, but they were more likely to indicate they "got sick a little easier than others." CONCLUSIONS: Persons with ostomies report more limitations in physical activities than do individuals within the general population. In contrast, they were no more likely to report bodily pain than the general population of the United States, and no more likely to indicate pain interferes with physical activity than the general population. Additional research is needed to elucidate the nature of these limitations and potential interventions to ameliorate these effects of ostomy creation.

Two Cases of Pneumatosis Intestinalis during Cetuximab Therapy for Advanced Head and Neck Cancer.

Pneumatosis intestinalis is a rare but known potential complication of treatment with cetuximab. Here we present two cases of pneumatosis intestinalis occurring in patients who were receiving cetuximab as treatment for advanced head and neck cancer. In both cases, cetuximab was discontinued after discovery of the pneumatosis intestinalis.

Health-Related Quality of Life in Community-Dwelling Persons With Ostomies: The Physical Functioning Domain.

PURPOSE: The purpose of this study was to explore health-related quality of life in persons living with ostomies and to compare these findings with a community-dwelling population residing in the United States. This detailed analysis reports the magnitude of physical limitations for each domain within the Physical Component Summary. DESIGN: Cross-sectional design. SUBJECTS AND SETTING: Potential respondents were identified by randomly sampling from proprietary databases containing e-mail contact information provided by persons living with a fecal or urinary ostomy. The sample comprised 2329 persons with ostomies; most were male (53.4%), and living with a single fecal ostomy (82.7%) or urostomy (21%). Health-related quality of life was measured using the Medical Outcomes Study Short Form-36 version 2. RESULTS: Persons with an ostomy are more likely to experience limitations in vigorous activity than individuals in the general population (odds ratio [OR]: 3.46, 95% confidence interval [CI]: 3.03-3.96) and activities requiring moderate exertion (OR: 2.72, 95% CI: 2.45-3.03). The prevalence of respondents reporting limitations was highest for those with colostomies. CONCLUSIONS: Persons with ostomies are more likely to report significant limitations in physical health than age-matched persons in the general population. Additional research is needed to determine whether these limitations are caused by the ostomy itself or a combination of factors including the underlying disease that led to creation of a stoma.

Interdigitating dendritic cell sarcoma.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare dendritic cell tumor with slightly more than 100 cases reported in the English literature. This report discusses a case of localized IDCS involving cervical lymph nodes and provides a literature review of clinicopathologic aspects and treatment outcomes.

Quality of life in US residents with ostomies as assessed using the SF36v2.

PURPOSE: The purpose of this study was to assess the quality of life of the adult US ostomy population (18 years of age or older). DESIGN: Cross-sectional descriptive study. SUBJECTS AND SETTING: Potential participants were randomly selected from an electronic database of 15,591 persons with ostomies. Potential respondents were contacted by e-mail and provided with an electronic nontransferable secured link to the survey. Two thousand three hundred twenty-nine participants completed the survey for a response rate of 14.9% and a margin of error of 2.03%. Study respondents were geographically distributed throughout the United States, representing all 50 states. Males comprised 53% of the study respondents; the median age of participants was 65 years, with 67% of the study respondents between the ages of 50 and 74 years. Forty percent had a colostomy, 44% had an ileostomy, 13% had a urostomy, 1% had multiple stomas, and 1% were unsure of their stoma type. INSTRUMENT: Health-related quality of life was evaluated using the SF36v2 survey instrument. This instrument was selected because of its ability to measure health-related quality of life in a sample of community-dwelling people with stomas and reference the data against a general population. RESULTS: The data presented a broad profile of the adult ostomy population in the United States. The average physical component summary score for the ostomy sample fell below the average for the US general population, while the average mental component summary (MCS) score fell within the average range of the US population. Analysis revealed that physical component summary scores decrease as age categories increase; however, MCS scores increased proportionally with age, suggesting that the health burden is primarily associated with physical health status, and not mental health status. CONCLUSION: Study findings suggest that the ostomy population demonstrates successful aging similar to the US general population. This is most noticeable at age categories greater than 55 years where increasing MCS scores for males and females fell in the range equivalent to the average of the US general population.

Dietary practices in pyridoxine non-responsive homocystinuria: a European survey.

BACKGROUND: Within Europe, the management of pyridoxine (B6) non-responsive homocystinuria (HCU) may vary but there is limited knowledge about treatment practice. AIM: A comparison of dietetic management practices of patients with B6 non-responsive HCU in European centres. METHODS: A cross-sectional audit by questionnaire was completed by 29 inherited metabolic disorder (IMD) centres: (14 UK, 5 Germany, 3 Netherlands, 2 Switzerland, 2 Portugal, 1 France, 1 Norway, 1 Belgium). RESULTS: 181 patients (73% >16 years of age) with HCU were identified. The majority (66%; n=119) were on dietary treatment (1-10 years, 90%; 11-16 years, 82%; and >16 years, 58%) with or without betaine and 34% (n=62) were on betaine alone. The median natural protein intake (g/day) on diet only was, by age: 1-10 years, 12 g; 11-16 years, 11 g; and >16 years, 45 g. With diet and betaine, median natural protein intake (g/day) by age was: 1-10 years, 13 g; 11-16 years, 20 g; and >16 years, 38 g. Fifty-two percent (n=15) of centres allocated natural protein by calculating methionine rather than a protein exchange system. A methionine-free l-amino acid supplement was prescribed for 86% of diet treated patients. Fifty-two percent of centres recommended cystine supplements for low plasma concentrations. Target treatment concentrations for homocystine/homocysteine (free/total) and frequency of biochemical monitoring varied. CONCLUSION: In B6 non-responsive HCU the prescription of dietary restriction by IMD centres declined with age, potentially associated with poor adherence in older patients. Inconsistencies in biochemical monitoring and treatment indicate the need for international consensus guidelines.

Dietary management of urea cycle disorders: European practice.

BACKGROUND: There is no published data comparing dietary management of urea cycle disorders (UCD) in different countries. METHODS: Cross-sectional data from 41 European Inherited Metabolic Disorder (IMD) centres (17 UK, 6 France, 5 Germany, 4 Belgium, 4 Portugal, 2 Netherlands, 1 Denmark, 1 Italy, 1 Sweden) was collected by questionnaire describing management of patients with UCD on prescribed protein restricted diets. RESULTS: Data for 464 patients: N-acetylglutamate synthase (NAGS) deficiency, n=10; carbamoyl phosphate synthetase (CPS1) deficiency, n=29; ornithine transcarbamoylase (OTC) deficiency, n=214; citrullinaemia, n=108; argininosuccinic aciduria (ASA), n=80; arginase deficiency, n=23 was reported. The majority of patients (70%; n=327) were aged 0-16y and 30% (n=137) >16y. Prescribed median protein intake/kg body weight decreased with age with little variation between disorders. The UK tended to give more total protein than other European countries particularly in infancy. Supplements of essential amino acids (EAA) were prescribed for 38% [n=174] of the patients overall, but were given more commonly in arginase deficiency (74%), CPS (48%) and citrullinaemia (46%). Patients in Germany (64%), Portugal (67%) and Sweden (100%) were the most frequent users of EAA. Only 18% [n=84] of patients were prescribed tube feeds, most commonly for CPS (41%); and 21% [n=97] were prescribed oral energy supplements. CONCLUSIONS: Dietary treatment for UCD varies significantly between different conditions, and between and within European IMD centres. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are required.

Human papillomavirus and tobacco use in tongue base cancers.

Human papillomavirus 16 (HPV-16) infection and tobacco use are associated with human oropharyngeal cancers. We conducted a study of the role of HPV and tobacco use in base of the tongue (BOT) cancers. DNA from 34 such cancers was subjected to HPV-16 and HPV-18-specific polymerase chain reaction analysis. Demographic and clinicopathologic data were obtained from each patient's medical record. HPV-16 was detected in 68% of tumors. Tobacco use was the only factor found to be significantly associated with HPV status. Tumors from 100% of patients who had never used tobacco tested positive for HPV, compared with only 56% of those who had ever used tobacco (Fisher exact test, p = 0.024). All tumors were associated with either tobacco use or HPV infection. These findings are consistent with the hypothesis that either tobacco use or HPV infection is necessary to the etiology of BOT tumors, and they suggest that tongue base carcinoma may be prevented by combining HPV vaccination with tobacco avoidance.

Pectoralis major myocutaneous flap for reconstruction of circumferential pharyngeal defects.

BACKGROUND: A 270-degree partially tubed pectoralis major myocutaneous flap (PMMF) is an excellent option for total circumferential pharyngoesophageal defects in patients who are not candidates for more complex reconstructions. METHODS: Patients undergoing circumferential pharyngoesophageal reconstruction with partially tubed PMMF were reviewed. End points were stricture, fistula, resumption of oral intake, perioperative death, and recurrence. RESULTS: Eleven patients underwent 270-degree PMMF for reconstruction: 6 (55%) were men and 5 (45%) were women (mean, 62 years; range, 42-78 years). Three patients (27%) developed fistulas and 2 (18%) developed stenosis. Ten patients (91%) were able to resume adequate nutrition via oral intake. There were no perioperative deaths. CONCLUSIONS: Patients with severe comorbidities, metastatic disease, a lack of donor vessels, or a potentially hostile abdomen may not be ideal candidates for free tissue transfer. For these patients, partially tubed PMMF using the prevertebral fascia provides a reliable alternative for reconstruction with excellent functional results.