National Survey Of The Aetiological Assessment Service Of Permanent Childhood Hearing Loss In Ireland

Aim The Universal Neonatal Hearing Screening Programme (UNHS) was implemented nationally in 2014. All infants identified with permanent childhood hearing loss (PCHL) should have a paediatric assessment performed. This survey aimed to assess available paediatric services and to inform service development. Methods All paediatricians involved in assessment of infants with PCHL were identified. A questionnaire was developed using the BAAP standards. Results were collated on excel. Results Thirty-three paediatricians assess children with PCHL, only 18% (6/33) had received specific training. Waiting time for assessment was beyond the recommended timeframe in the majority of cases (mean 14.4 weeks, range 2-52). Timely access to services such as MRI, genetics and ophthalmology was limited. Conclusion The survey highlights significant deficits in the paediatric component of the UNHS. A model of regionalisation with recommendations to improve the paediatric skill set, resources and supporting services is suggested.

The effect of purse-string approximation versus linear approximation of ileostomy reversal wounds on morbidity rates and patient satisfaction: the 'STOMA' trial.

BACKGROUND: Ileostomy reversal is associated with surgical site infection (SSI) rates as high as 37%. Recent literature suggests that employing a purse-string approximation (PSA) of the reversal wound reduces this rate of SSI. Thus we wished to perform a randomised controlled trial to compare SSI rates in purse-string versus linear closure (PLC) wounds following ileostomy reversal. METHODS: A randomised, controlled trial was conducted at University Hospital Limerick. Sixty-one patients undergoing ileostomy reversal were included. Thirty-four patients were randomised to PSA and 27 patients to linear closure. The primary endpoint was incidence of SSI and secondary endpoints measured were quality of life and satisfaction with cosmesis. Statistical analysis was performed on a per protocol basis using SPSS version 22.0. RESULTS: Three patients in the PSA group developed an SSI compared to 8 in the PLC group at 30 days (8 vs 30%, p = 0.03). The mean time to SSI diagnosis was faster in the PSA group (3 vs 12.3 days, p = 0.08). Patients who developed SSI experienced a longer mean length of stay (6.8 vs 11.4 days, p = 0.012). On multivariate analysis, PLC was the only predictive factor of SSI formation (p < 0.001). There was no difference in patient satisfaction between the two study groups (p = 0.14). CONCLUSIONS: PSA of wounds following ileostomy reversal significantly reduces SSI formation compared to linear approximation without any effect on patient satisfaction.

Neoadjuvant therapy does not affect lymph node ratio in rectal cancer.

AIMS: Recently, lymph-node ratio (LNR) has emerged as a prognostic tool in staging rectal cancer. Studies to date have demonstrated threshold values above and below which survival is differentially altered. Neoadjuvant therapy significantly reduces the number of lymph node retrieved. The aim of the present study was to determine the effect of neoadjuvant therapy on LNR and its prognostic properties. METHODS: Consecutive patients who underwent curative rectal cancer resections in a single institution from 2007 to 2010 were reviewed. LNR was stratified into five subgroups of 0, 0.01-0.17, 0.18-0.41, 0.42-0.69 and 0.7-1.0 based on a previous study. The effect of neoadjuvant therapy on lymph node retrieval, LNR, locoregional (LR) and systemic recurrence (SR), disease-free (DFS) and overall survival (OS) was compared between patients who did (Neoadjuvant) and did not (Surgery Alone) receive neoadjuvant therapy. RESULTS: Neoadjuvant and Surgery Alone groups were comparable in gender, age and tumour stage. The number of lymph nodes retrieved were significantly lower in the Neoadjuvant group (p < 0.01). However, LNR remained similar in both groups (p = 0.36). There was no statistical difference in the DFS and OS between the Neoadjuvant and Surgery Alone groups at the various LNR cut off values in patients with AJCC Stage 3 tumours. CONCLUSIONS: LNR ratio remains unaltered despite reduced lymph node retrieval after neoadjuvant therapy in rectal cancer. LNR may therefore be a more reliable prognostic indicator in this subgroup of patients.

Overall survival is improved in mucinous adenocarcinoma of the colon.

INTRODUCTION: Debate persists regarding the relationship between mucin expression and outcome in colon cancer. This arises due to discrepancy in the definition of mucinous adenocarcinoma and the combination of both colon and rectal cancers in analyses. This study examines the relationship between increased mucin production and outcomes in colon cancer. METHODS: Cases were classified according to the World Health Organization classification of mucinous adenocarcinoma of the colon. Accordingly, tumors were categorized as either (a) mucinous adenocarcinoma of the colon (greater than 50% of the extracellular matrix occupied by mucin) or (b) non-mucinous adenocarcinoma of the colon. Overall survival and disease-free survival were calculated. A stepwise Cox proportional hazards regression model was employed to determine the risk of death/disease recurrence. Kaplan-Meier estimates of overall survival and disease-free survival were plotted for each group and compared using a log-rank test. RESULTS: On univariate analysis, mucinous adenocarcinoma was associated with reduced risk of death (P = 0.01). On multivariate analysis, mucinous adenocarcinoma was also associated with reduced risk of death (hazard ratio (HR) 0.33, 95% confidence interval (CI) 0.14-0.79, P = 0.01). Kaplan-Meier estimates confirmed improved rate of survival in the mucinous vs. non-mucinous group (P = 0.01). Mucinous adenocarcinoma did not affect disease-free survival (HR 0.75, 95% CI 0.46-1.21, P = 0.22). A comparison of Kaplan-Meier estimates for systemic recurrence demonstrated significant increases in systemic recurrence in the group with no mucin production (P = 0.04) but not for locoregional recurrence (P = 0.24). CONCLUSIONS: Histopathological evidence of mucinous adenocarcinoma in colon cancer is associated with improved outcomes.

Vertical rectus abdominis myocutaneous flap and quality of life following abdominoperineal excision for rectal cancer: a multi-institutional study.

BACKGROUND: To obtain a clear surgical margin, abdominoperineal excision (APE) for rectal cancer frequently leaves a large perineal defect surrounded by irradiated tissue. A vertical rectus abdominis myocutaneous (VRAM) flap may facilitate healing of this wound. The current study aims to determine the effect of VRAM flap perineal reconstruction following APE on patient quality of life (QOL). METHODS: This is a retrospective cohort study from a prospectively collected database. Data on QOL were assessed via telephone questionnaire using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ)-C30, EORTC QLQ-C29 and the Cleveland Clinic QOL questionnaires. RESULTS: Twenty-seven patients underwent primary perineal closure, and 12 patients underwent a VRAM flap perineal reconstruction. The mean duration of follow-up was 16.8 months. Overall, there was no significant difference in the Cleveland Clinic QOL score between groups (VRAM vs. no VRAM: 0.7 ± 0.2 vs. 0.7 ± 0.2, p 0.735). Patients in the VRAM group had lower levels of fatigue (5.5 ± 9.9 vs. 23.6 ± 19.2, p 0.004). Patients in the VRAM group had reduced sore skin scores around the stoma site (11.0 ± 16.2 vs. 31.8 ± 31.1, p 0.036). VRAM flap was associated with an increased incidence of abdominal wall hernia (VRAM vs. no VRAM: 25 % vs. 0 %, p 0.024). CONCLUSIONS: This study is limited by its non-randomized retrospective design and relatively small sample size. A significant difference in patient QOL was not demonstrated between VRAM flap and primary perineal closure after APE for rectal cancer. Further studies in this area are warranted.

Beware the contracted gallbladder - Ultrasonic predictors of conversion.

INTRODUCTION: Pre-operative ultrasound is the gold standard pre-operative investigation for patients undergoing a cholecystectomy. Ultrasound provides a sensitive approach for analysing characteristics of the gallbladder. Thus, we aimed to examine the importance of ultrasonic gallbladder characteristics on laparoscopic cholecystectomy conversion rates and then sought to devise a pre-operative predictive score for conversion based on our findings. METHODS: A retrospective analysis of patients undergoing a laparoscopic cholecystectomy was performed between January 2000 and December 2006. Patient demographic data and pre-operative imaging results from abdominal ultrasounds were analysed. We then devised a pre-operative predictive score for conversion based on independent variables derived from multivariate analysis. RESULTS: A total of 1061 patients underwent a laparoscopic cholecystectomy. Conversion to an open procedure was required in 58 cases. The overall conversion rate was 5.4%. Univariate analysis revealed male gender (p < 0.0001), gallbladder wall thickness >4 mm (p = 0.0024), a contracted gallbladder (p = 0.005) and a dilated CBD (p = 0.0416) as being significantly associated with conversion. These variables were then evaluated using multivariate analysis and three variables, namely, male gender, a contracted gallbladder and a thickened gallbladder wall were identified as independent predictors. A pre-operative predictive score for conversion was devised from a training cohort (n = 761) and tested on a sub-cohort (n = 300). Patients with a score of 2 or more had a 19.2% risk of conversion (p < 0.001). CONCLUSION: Conversion to an open cholecystectomy shows a strong associated with gallbladder ultrasonic characteristics which are available pre-operatively. The likelihood of conversion can be accurately predicted using a pre-operative scoring system.

Effect of fat content and homogenization under conventional or ultra-high-pressure conditions on interactions between proteins in rennet curds.

The objective of this study was to investigate the influence of conventional and ultra-high-pressure homogenization on interactions between proteins within drained rennet curds. The effect of fat content of milk (0.0, 1.8, or 3.6%) and homogenization treatment on dissociation of proteins by different chemical agents was thus studied. Increasing the fat content of raw milk increased levels of unbound whey proteins and calcium-bonded caseins in curds; in contrast, hydrophobic interactions and hydrogen bonds were inhibited. Both homogenization treatments triggered the incorporation of unbound whey proteins in the curd, and of caseins through ionic bonds involving calcium salts. Conventional homogenization-pasteurization enhanced interactions between caseins through hydrogen bonds and hydrophobic interactions. In contrast, ultra-high-pressure homogenization impaired hydrogen bonding, led to the incorporation of both whey proteins and caseins through hydrophobic interactions and increased the amount of unbound caseins. Thus, both homogenization treatments provoked changes in the protein interactions within rennet curds; however, the nature of the changes depended on the homogenization conditions.

Rectum-conserving surgery in the era of chemoradiotherapy.

BACKGROUND: A complete pathological response occurs in 10-30 per cent of patients with locally advanced rectal cancer undergoing neoadjuvant chemoradiotherapy (CRT). The standard of care has been radical surgery with high morbidity risks and the challenges of stomata despite the favourable prognosis. This review assessed minimalist approaches (transanal excision or observation alone) to tumours with a response to CRT. METHODS: A systematic review was performed using PubMed and Embase databases. Keywords included: 'rectal', 'cancer', 'transanal', 'conservative', 'complete pathological response', 'radiotherapy' and 'neoadjuvant'. Original articles from all relevant listings were sourced. These were hand searched for further articles of relevance. Main outcome measures assessed were rates of local recurrence and overall survival, and equivalence to radical surgery. RESULTS: Purely conservative 'watch and wait' strategies after CRT are still controversial. Originally used for elderly patients or those who refused surgery, the data support transanal excision of rectal tumours showing a good response to CRT. A complete pathological response in the T stage (ypT0) indicates < 5 per cent risk of nodal metastases. CONCLUSION: Rectal tumours showing an excellent response to CRT may be suitable for local excision, with equivalent outcomes to radical surgery. This approach should be the subject of prospective clinical trials in specialist centres.

Anterior translation of the tibia in relation to femur in mucoid degeneration of ACL - An observational study.

BACKGROUND: Mucoid degeneration (MD) of the anterior cruciate ligament (ACL) are a well-known pathological entity.We have encountered several patients with MD of the ACL, found to have a anterior translation of tibia a exceeding 5 mm with an intact ACL. We studied this cohort and investigated the likely cause of this. METHODS: A retrospective search of our department's radiology system to identify all patients referred from the knee orthopaedic clinic for MR imaging over a span of 10 years. All patients had MD within the substance of the ACL and an intact ACL. We evaluated the degree of anterior translation of the tibia (ATT) in relation to the femur in mucoid degeneration of ACL. RESULTS: We identified 464 consecutive cases. The mean age was 52 years. There was a male predominance of 261 to 203 female. The average PTF measurement was 2.4 mm with a range of 0-20mm. Of the 464 cases, 3 397 patients had an insignificant ATT of < 5 mm. (0mm. 67. 67 67 patients had a ATT >5 mm. Of them, 32.8% had a ATT of 6 mm, 53.7% had a ATT range between 7 and 9 mm, with the remaining 13.4% above 9 mm in length. CONCLUSION: It is essential to look for other secondary signs of ACL tears and not only focus on ATT as well as correlate this with clinical findings.

Proxy assessment of quality of life in patients with prostate cancer: how accurate are partners and urologists?

OBJECTIVES: To assess the ability of partners and clinicians to make proxy judgements on behalf of patients with prostate cancer relating to selection of life priorities and quality of life (QoL). DESIGN: 47 consecutive patients with histologically proven adenocarcinoma, and their partners, were recruited. The partners were asked to assess, by proxy, the QoL of the patient by completion of a series of interview-led questionnaires assessing global QoL (SEIQoL-DW), health-related QoL (FACT-P) and overall QoL (visual analogue score [VAS]). The patients' clinicians were asked to complete the SEIQoL-DW and VAS by proxy as soon as possible after a consultation with the patient. SETTING: Patients with histologically proven adenocarcinoma, their partners and their clinicians. MAIN OUTCOME MEASURES: Proxy scores for SEIQoL-DW, FACT-P and VAS, as provided by partners and clinicians. RESULTS: 25 partners made a proxy assessment of the patients. The results showed that partners were able to select similar QoL cues to those of the patients (Spearman-Rank correlation 0.89). Comparison of the QoL scores obtained from patients and partners in proxy using the questionnaires showed no statistically significant difference (paired t-test). Urologists were poor predictors of areas of life (cues) that were important to their patients. The doctors overemphasized the importance of survival, postoperative complications, urinary symptoms, sexual ability, activities of daily living and finance, but underestimated the importance of wife, family, home and religion. Comparison of the QoL scores obtained from patients and urologists by proxy showed a significantly lower score when assessed by urologists using the SEIQoL-DW questionnaire. CONCLUSIONS: Partners are able to accurately assess, by proxy, the areas of life that are of importance to patients. Clinicians, however, who are charged with making decisions on behalf of patients, are very poor judges of their patients' life priorities and QoL. This illustrates that conventional views held by most doctors regarding the priorities patients set themselves when planning treatment should be called into question and consequently suggests that the way in which doctors and patients arrive at treatment decisions must be reviewed.

Goats singly heterozygous for PRNP S146 or K222 orally inoculated with classical scrapie at birth show no disease at ages well beyond 6 years.

Scrapie is a transmissible spongiform encephalopathy of sheep and goats, and scrapie eradication programs in many parts of the world rely on strong genetic resistance to classical scrapie in sheep. However, the utility of putative resistance alleles in goats has been a focus of research because goats can transmit scrapie to sheep and may serve as a scrapie reservoir. Prior work showed that disease-free survival time was significantly extended in orally inoculated goats singly heterozygous for prion amino acid substitutions S146 or K222, but average durations were only around 3 years post-inoculation. The aim of this study was to investigate whether extended survival would exceed 6 years, which represents the productive lifetimes of most commercial goats. While all control homozygotes were clinically affected by an average of <2 years, none of the NS146 or QK222 goats developed clinical scrapie or had PrPSc-positive rectal biopsies. Several NS146 and QK222 goats developed other conditions unrelated to scrapie, but tissue accumulation of PrPSc was not detected in any of these animals. The NS146 heterozygotes have remained disease-free for an average of 2734days (approximately 7.5 years), the longest duration of any classical scrapie challenge experiment with any genotype to date. The QK222 heterozygotes have remained disease-free for an average of 2450days (approximately 6.7 years), the longest reported average duration for QK222 goats challenged with classical scrapie. This research is ongoing, but the current results demonstrate S146 and K222 confer strong resistance to classical scrapie in goats.

Retroperitoneal hemangiosarcoma causing chronic hindlimb lameness in a dog.

The clinical, radiographic, ultrasonographic, computed tomographic, surgical and histopathological findings in a Boxer dog with retroperitoneal hemangiosarcoma are described in this study. A seven-year-old, male, castrated Boxer dog was referred for evaluation of chronic hindlimb lameness. The physical examination revealed muscle atrophy and sciatic nerve deficits. Radiography and ultrasonography revealed a caudodorsal abdominal mass. Computed tomography revealed that the mass involved the left margin of the L7 vertebra, lumbosacral canal, and lumbosacral plexus. At surgery, a large retroperitoneal haematoma was removed. Histopathology of amorphous tissue found near the haematoma was consistent with haemangiosarcoma. The owner declined any further treatment. Ten weeks after discharge, the dog was euthanatized due to collapse and haemo-abdomen.

Development of polymer-based sensors for integration into a wireless data acquisition system suitable for monitoring environmental and physiological processes.

In this work, the pressure sensing properties of polyethylene (PE) and polyvinylidene fluoride (PVDF) polymer films were evaluated by integrating them with a wireless data acquisition system. Each device was connected to an integrated interface circuit, which includes a capacitance to frequency converter (C/F) and an internal voltage regulator to suppress supply voltage fluctuations on the transponder side. The system was tested under hydrostatic pressures ranging from 0 to 17 kPa. Results show PE to be the more sensitive to pressure changes, indicating that it is useful for the accurate measurement of pressure over a small range. On the other hand PVDF devices could be used for measurement over a wider range and should be considered due to the low hysteresis and good repeatability displayed during testing. It is thought that this arrangement could form the basis of a cost-effective wireless monitoring system for the evaluation of environmental or physiological processes.

A review of digestible microsystems for gastrointestinal tract diagnostic applications.

Present methods for investigation of the GI tract are invasive, distressing for the patient, and give a low diagnostic yield. Wireless, radio telemetry capsules, capable of monitoring physiological changes or visualizing the GI tract are noninvasive and could realize a faster time to diagnoses along with improved treatment of both organic diseases and functional disorders. Consequently, the patient's quality of life would improve. In this paper, early radiotelemetry capsules and the motivating factors for their development are discussed. Following this, prototype and commercially available digestible microsystems making use of microelectronics and microelectromechanical systems are presented. It is shown that these capsules have the potential to combine the functions of their predecessors and furthermore can be used for visualizing the gastrointestinal tract. These systems have the potential to improve diagnostic yield and, in the future, treatment of disease using these capsules should become a reality.

Early onset of colonic adenocarcinoma associated with cystic fibrosis--a case report.

We report the case of the onset of colorectal adenocarcinoma occurring in an 18 year-old patient, suffering from Cystic Fibrosis. This represents a presentation of colon cancer at an earlier stage than previously reported. Despite this, there is an established link between the two diseases although the pathogenesis is, as yet, unknown. This early onset emphasizes yet another association with the disease and encourages clinicians to be aware of potentially disastrous complications.

Quality-of-life measurement in advanced cancer: assessing the individual.

PURPOSE: Despite the increasing importance of assessing quality of life (QoL) in patients with advanced cancer, relatively little is known about individual patient's perceptions of the issues contributing to their QoL. The Schedule for the Evaluation of Individual Quality of Life (SEIQoL) and the shorter SEIQoL-Direct Weighting (SEIQoL-DW) assess individualized QoL using a semistructured interview technique. Here we report findings from the first administration of the SEIQoL and SEIQoL-DW to patients with advanced incurable cancer. PATIENTS AND METHODS: QoL was assessed on a single occasion using the SEIQoL and SEIQoL-DW in 80 patients with advanced incurable cancer. RESULTS: All patients were able to complete the SEIQoL-DW, and 78% completed the SEIQoL. Of a possible score of 100, the median QoL global score was as follows: SEIQoL, 61 (range, 24 to 94); SEIQoL-DW, 60.5 (range, 6 to 95). Psychometric data for SEIQoL indicated very high levels of internal consistency (median r =.90) and internal validity (median R(2) = 0.88). Patients' judgments of their QoL were unique to the individual. Family concerns were almost universally rated as more important than health, the difference being significant when measured using the SEIQoL-DW (P =.002). CONCLUSION: Patients with advanced incurable cancer were very good judges of their QoL, and many patients rated their QoL as good. Judgments were highly individual, with very high levels of consistency and validity. The primacy given to health in many QoL questionnaires may be questioned in this population. The implications of these findings are discussed with regard to clinical assessment and advance directives.

Review of the potential of a wireless MEMS and TFT microsystems for the measurement of pressure in the GI tract.

Telemetry capsules have existed since the 1950s and were used to measure temperature, pH or pressure inside the gastrointestinal (GI) tract. It was hoped that these capsules would replace invasive techniques in the diagnosis of function disorders in the GI tract. However, problems such as signal loss and uncertainty of the pills position limited their use in a clinical setting. In this paper, a review of the capabilities of MicroElectroMechanical Systems (MEMS) and thick film technology (TFT) for the fabrication of a wireless pressure sensing microsystem is presented. The circuit requirements and methods of data transfer are examined. The available fabrication methods for MEMS sensors are also discussed and examples of wireless sensors are given. Finally the limitations of each technology are examined.

Retrotracheal goiter: a diagnostic and therapeutic problem.

A patient with chronic cough and recent dysphagia was found to have a retrotracheal mass extending into the visceral mediastinum on chest roentgenogram. A computed tomographic scan confirmed a retrotracheal lesion, which was believed to be of lymphatic origin. A thyroid scan demonstrated downward displacement of the left lobe but little uptake in the mass. Histological findings of mediastinal biopsies were inconclusive. A large retrotracheal thyroid adenoma was easily excised through a right thoracotomy. The approach to diagnosis and, in cases of doubt, the safety of surgical access through thoracotomy for thyroid lesions in this unusual site is discussed.

Quality of life issues in palliative medicine.

The assessment of patient's quality of life is assuming increasing importance in medicine and health care. Illnesses, diseases and their treatments can have significant impacts on such areas of functioning as mobility, mood, life satisfaction, sexuality, cognition, and ability to fulfil occupational, social and family roles. The emerging quality of life construct may be viewed as a paradigm shift in outcome measurement since it shifts the focus of attention from symptoms to functioning. This holistic approach more clearly establishes the patient as the centre of attention and subsumes many of the traditional measures of outcome. Quality of life assessment is particularly relevant to patients with progressive conditions, particularly in the later phases of the disease. Despite the fact that current definitions of palliative medicine include quality of life as a central concern, relatively little research has been conducted on the impact of palliative care on patient quality of life. This paper introduces the concept of quality of life and describes the significant difficulties in definition, measurement and interpretation that must be addressed before such measures can be used as reliable and valid indicators of disease impact and treatment outcomes. It is argued that the unique individual perspective of the patient on his or her own quality of life must be incorporated into outcome assessments aimed at improving the quality of health care delivery in progressive diseases.