Clinical and Genetic Characteristics and Outcome in Patients with Neonatal Diabetes Mellitus from a Low Middle-Income Country.

Neonatal Diabetes Mellitus (NDM) is a disorder characterized by persistent, severe hyperglycemia presenting during the first 6 months of life. These disorders are rare and the incidence is approximately 1 in 90,000 live births. To describe the clinical presentation, molecular genetics and outcome of patients with NDM from a single paediatric endocrine center from a low middle income country. A retrospective study was conducted on patients diagnosed with NDM. Medical records were reviewed for demographic data and data on clinical, biochemical and genetic analysis. 96% of patients who underwent mutation analysis had pathogenic genetic mutations on Sanger sequencing. Permanent NDM (PNDM) was diagnosed in 19 patients with 3 of them having a syndromic diagnosis. The commonest mutation was found in KCNJ11 gene. Majority of the PNDM (63%) presented with severe diabetic ketoacidosis. All patients with Transient NDM (TNDM) remitted by 6 months of age. 47% of the cases with PNDM made a switch to sulfonylurea therapy with good glycemic control (glycosylated Haemoglobin A1C 6-7.5). Data from the Sri Lankan cohort is comparable with other populations. The majority of cases are due to KCNJ11 mutations resulting in PNDM.

Survey of rectal cancer MRI technique and reporting tumour descriptors in the UK: a multi-centre British Society of Gastrointestinal and Abdominal Radiology (BSGAR) audit.

AIM: To evaluate variation in magnetic resonance imaging (MRI) technique and reporting of rectal cancer staging examinations across the UK. MATERIALS AND METHODS: A retrospective, multi-centre audit was undertaken of imaging protocols and information documented within consecutive MRI rectal cancer reports between March 2020 and August 2021, which were compared against American and European guidelines. Inclusion criteria included histologically proven rectal adenocarcinoma and baseline staging MRI rectum only. RESULTS: Fully anonymised data from 924 MRI reports by 78 radiologists at 24 centres were evaluated. Thirty-two per cent of radiologists used template reporting, but these reports offered superior documentation of 13 out of 18 key tumour features compared to free-text reports including T-stage, relation to peritoneal reflection and mesorectal fascia (MRF), nodal status, and presence of extramural venous invasion (EMVI; p<0.027 in each). There was no significant differences in the remaining five features. Across all tumour locations, the tumour relationship to the MRF, the presence of EMVI, and the presence of tumour deposits were reported in 79.5%, 85.6%, and 44% of cases, respectively, and tumour, nodal, and distant metastatic stage documented in 94.4%, 97.7%, and 78.3%. In low rectal tumours, the relationship to the anal sphincter complex was reported in only 54.6%. CONCLUSION: Considerable variation exists in rectal cancer MRI acquisition and reporting in this sample of UK centres. Inclusion of key radiological features in reports must be improved for risk stratification and treatment decisions. Template reporting is superior to free-text reporting. Routine adoption of standardised radiology practices should now be considered to improve standards to facilitate personalised precision treatment for patients to improve outcomes.

CTC service heterogeneity and design of a workforce calculator.

AIM: To determine the level of heterogeneity in delivery of computed tomography (CT) colonography services and develop a workforce calculator that accommodates the variation identified. MATERIALS AND METHODS: A national survey, based on the "WHO workforce indicators of staffing need", established activity standards for essential tasks in delivery of the service. From these data a workforce calculator was designed to guide the required staffing and equipment resource by service size. RESULTS: Activity standards were established as mode responses >70%. Service homogeneity was greater in areas where professional standards and guidance were available. The mean service size was 1,101. Did not attend (DNA) rates were lower where direct booking was available (p<0.0001). Service sizes were larger where radiographer reporting was embedded in reporting paradigms (p<0.024). CONCLUSION: The survey identified benefits of radiographer-led direct booking and reporting. The workforce calculator derived from the survey provides a framework to guide the resourcing of expansion while maintaining standards.

What is the impact of CT colonography interpretation by advanced practitioner radiographers on delivery of the 28 day colorectal cancer target?

INTRODUCTION: CT Colonography (CTC) is an indicated test to assess the colon and rectum for evidence of polyps and neoplasms. The advanced practitioner radiographer (APR) is increasingly involved with the entirety of the radiology pathway including procedural modification, preliminary clinical evaluation (PCE) and multi-disciplinary team (MDT) meeting notification of high risk colonic pathologies. METHODS: A retrospective audit of the Radiology Information System (RIS) was undertaken at a large secondary care centre, 12 months of data of 119 consecutive patients who had undergone CTC with summary coded reports of high risk pathology were included for analysis. Analysis of accuracy of procedural modification, PCE and impact of hypothesised earlier full radiological staging data being available for MDT discussions were measured and evaluated. RESULTS: For high risk C4b studies, just 16.67% of colonic pathology was observed during the CTC study, rising to 79% during radiographer PCE. For likely colonic neoplasm C5a studies 86% of colonic pathology was observed during the CTC study, rising to 93% during radiographer PCE. Where subsequent CT chest staging was deemed necessary following CTC by the referring team, patients had a median wait of 34 days for completion CT chest scan staging. CONCLUSION: This study supports the integration of the advanced practitioner radiographer into the entire radiological processes of a CTC, with time advantages apparent for both diagnostics, but also the decision to treat. IMPLICATIONS FOR PRACTICE: Appropriately trained radiographers are able to support CTC services to ensure delivery of an effective two-week wait diagnostic service with direct MDT liaison.

A portable microfluidic device-based Fe3O4-urease nanoprobe-enhanced colorimetric sensor for the detection of heavy metals in fish tissue.

A portable microfluidic device with highly sensitive enzyme nanoprobe (Fe3O4 MNPs-urease, average size 34.6 nm) was demonstrated for the analysis of heavy metals ions (Hg2+, Cd2+ and Pb2+) in fish gill and muscle tissue. The immobilized urease nanoprobe (Km = 0.05 mM) exhibited twofold sensitivity over the free enzyme assay (apparent Km = 0.1 mM). The nanoprobe was characterized using SEM, EDAX, PSA and FT-IR. The inhibition measurements were carried out for individual as well as the mixture of metal ions (CRM standards of 9 elements (CRMmix-9)). The lower limit of quantification (LOQ) (0.5, 0.1, and 0.1 ng L-1 for Hg2+, Cd2+, and Pb2+) and lower limit of detection (LOD) was achieved at 0.1 ng L-1 with sensitivity 8-14% per decade for Hg2+, Cd2+, and Pb2+ ions. A visual result can be observed by the naked eye through the microfluidic device as well as with 96 transparent microwell plates. The order of relative inhibition was found to be CRMmix-9 > (Hg2+ + Cd2+ + Pb2+) > (Cd2+ + Pb2+) > (Pb2+ + Hg2+) > (Hg2+ + Cd2+) > Pb2+ > Cd2+ > Hg2+, respectively. The recovery % in fish tissues were found to be 88-98% for Hg2+, Cd2+ and Pb2+ ions.

A national audit of radiology practice in cancer multidisciplinary team meetings.

AIM: A national audit reviewing compliance of imaging departments with the Royal College of Radiologist (RCR) standards for cancer multidisciplinary team meetings (MDTMs). MATERIALS AND METHODS: The audit consisted of a generic and subspecialty component completed for breast, colorectal, and lung cancer MDTMs. RESULTS: The study achieved the highest response from a RCR national audit with 145/191 (76%) hospitals responding. Compliance with the RCR standards was suboptimal, particularly relating to MDTM attendance, documentation, and reviewing MDTM imaging. Comprehensive radiology MDTM attendance occurred in 52-65%, a supplementary report denoting staging/treatment plans happened in 15-26%, and late additions were discussed frequently without prior review of imaging (44-77%). Contributing factors maybe 13% of radiologists had no programmed activity for MDTMs in their job plan and a perceived negative impact of increasing MDTM referrals (51%). Adjuncts to improve MDTM workload, such as treatment pathways/algorithms (breast/colorectal 54%) and pro-forma (43-50%), were poorly implemented. Discrepancies with the original imaging report highlighted at MDTMs were well documented (92-94%) and frequently presented at discrepancy meetings (70-81%). Learning from involvement in MDTM was well communicated with 76-84% providing peer feedback. CONCLUSIONS: Radiologists are unable to comprehensively attain the RCR MDTM standards on providing and documenting a specialist opinion on the imaging. Increasing referrals to the MDTM appears the predominant factor and differentiating complex cases that benefit from MDTM discussion from those that can be managed via treatment pathways is required. Improved utilisation of pre-MDTMs/pro-forma and information technology in MDTMs may further aid radiologists to provide consistent high-quality contribution towards MDTMs.

The Effect of Surface Treatments on the Shear Bond Strength of Acrylic Resin Denture Base With Different Repair Acrylic Resin: An In Vitro Study.

BACKGROUND: Fracture of the denture base is a common problem associated with dental prostheses. Fractured denture base surfaces treated with chemical agents and mechanical features have the potential for improved bond strength. AIM: The aim of this study was to evaluate the effect of surface treatment on the shear bond strength of heat-cured denture base with different repair acrylic resins. MATERIALS AND METHODS: A total of 100 circular specimens (2-cm diameter × 3.3-mm thickness) were fabricated from heat-cured denture resins (DPI) according to the manufacturer's instructions. The heat-cured denture base acrylic resin specimens were divided into two groups: In group 1, auto-polymerizing acrylic resin (DPI) was used as a repair resin, and in group 2, light-cured acrylic resin (VLC) was used as the repair resin. Further, the heat-cured denture base acrylic resin specimens were subdivided into five subgroups. The shear bond strength (in megapascal) was measured in a universal testing machine at a crosshead speed of 1 mm/min. The results were subjected for statistical analysis. RESULT: Comparison of mean and standard deviation of shear bond strength between DPI and VLC group using one-way analysis of variance showed that the mean shear bond strength of DPI group is higher than that of VLC group. CONCLUSION: From the study, heat-cured denture base specimens repaired with auto-polymerizing repair resin showed higher mean shear bond strength than the visible light cure resin material.

Quantitative and qualitative assessment of a coding system for reporting CT colonography.

AIM: To validate a coding system implemented to summarise computed tomography colonography (CTC) findings for the detection of suspected colorectal cancer (CRC) by assessing interobserver variability and also to evaluate any weaknesses through qualitative analysis. MATERIALS AND METHODS: All CTC investigations over a 6-month period (01/07/2016 to 31/12/2016) were analysed retrospectively. Each study was read initially by an advanced practitioner radiographer with a final report issued by a consultant gastrointestinal radiologist. Rates of interobserver agreement, using the kappa statistic, provided a quantitative assessment of levels of agreement. Areas of poor interobserver agreement were identified for further qualitative assessment. RESULTS: The present study included 1,321 CTC procedures and the mean age of patients was 68.4 years (range 28-96 years). Percentage agreement for colonic coding was 90% and for extra-colonic coding 47%. This corresponds to kappa scores of 0.69 (substantial agreement) and 0.22 (fair agreement), respectively. Reasons and examples of disagreement in the colonic coding are highlighted. CONCLUSIONS: High interobserver agreement was observed for C coding, suggesting it is a reproducible method of classifying intra-colonic CTC findings. Some of the difference in classifying extra-colonic findings is the perceived importance of incidental findings between readers, as well as differences in skill set; however, some themes recurred in areas of disagreement and recommendations for refining and improving the coding system are provided.

Experimental investigation into the impact of density wave oscillations on flow boiling system dynamic behavior and stability.

In order to better understand and quantify the effect of instabilities in systems utilizing flow boiling heat transfer, the present study explores dynamic results for pressure drop, mass velocity, thermodynamic equilibrium quality, and heated wall temperature to ascertain and analyze the dominant modes in which they oscillate. Flow boiling experiments are conducted for a range of mass velocities with both subcooled and saturated inlet conditions in vertical upflow, vertical downflow, and horizontal flow orientations. High frequency pressure measurements are used to investigate the influence of individual flow loop components (flow boiling module, pump, pre-heater, condenser, etc.) on dynamic behavior of the fluid, with fast Fourier transforms of the same used to provide critical frequency domain information. Conclusions from this analysis are used to isolate instabilities present within the system due to physical interplay between thermodynamic and hydrodynamic effects. Parametric analysis is undertaken to better understand the conditions under which these instabilities form and their impact on system performance. Several prior stability maps are presented, with new stability maps provided to better address contextual trends discovered in the present study.

Flow condensation pressure oscillations at different orientations.

Investigation of two-phase flow dynamic behavior and instabilities has traditionally centered on phenomena present in boiling flows due to the safety critical nature of boiling in a variety of cooling applications. Analysis of pressure signals in condensing systems reveal the presence of relevant oscillatory phenomena during flow condensation as well, which may impact performance in applications concerned with precise system control. Towards this end, the present study presents results for oscillatory behavior observed in pressure measurements during flow condensation of FC-72 in a smooth circular tube in vertical upflow, vertical downflow, and horizontal flow orientations. Dynamic behavior observed within the test section is determined to be independent of other components within the flow loop, allowing it to be isolated and interpreted as resulting from physical aspects of two-phase flow with condensation. The presence of a peak oscillatory mode (one of significantly larger amplitude than any others present) is seen for 72% of vertical upflow test cases, 61% of vertical downflow, and 54% of horizontal flow. Relative intensities of this peak oscillatory mode are evaluated through calculation of Q Factor for the corresponding frequency response peak. Frequency and amplitude of peak oscillatory modes are also evaluated. Overall, vertical upflow is seen to exhibit the most significant oscillatory behavior, although in its maximum case amplitude is only seen to be 7.9% of time-averaged module inlet pressure, indicating there is little safety risk posed by oscillations under current operating conditions. Flow visualization image sequences for each orientation are also presented and used to draw parallels between physical characteristics of condensate film behavior under different operating conditions and trends in oscillatory behavior detected in pressure signals.

Experimental investigation of frequency and amplitude of density wave oscillations in vertical upflow boiling.

Historically, study of two-phase flow instabilities has been arguably one of the most challenging endeavors in heat transfer literature due to the wide range of instabilities systems can manifest depending on differences in operating conditions and flow geometry. This study utilizes experimental results for vertical upflow boiling of FC-72 in a rectangular channel with finite inlet quality to investigate Density Wave Oscillations (DWOs) and assess their potential impact on design of two-phase systems for future space missions. High-speed flow visualization image sequences are presented and used to directly relate the cyclical passage of High and Low Density Fronts (HDFs and LDFs) to dominant low-frequency oscillations present in transient pressure signals commonly attributed to DWOs. A methodology is presented to determine frequency and amplitude of DWO induced pressure oscillations, which are then plotted for a wide range of relevant operating conditions. Mass velocity (flow inertia) is seen to be the dominant parameter influencing frequency and amplitude of DWOs. Amplitude of pressure oscillations is at most 7% of the time-averaged pressure level for current operating conditions, meaning there is little risk to space missions. Reconstruction of experimental pressure signals using a waveform defined by frequency and amplitude of DWO induced pressure fluctuations is seen to have only moderate agreement with the original signal due to the oversimplifications of treating DWO induced fluctuations as perfectly sinusoidal in nature, assuming they occur at a constant frequency value, and neglecting other transient flow features. This approach is nonetheless determined to have potential value for use as a boundary condition to introduce DWOs in two-phase flow simulations should a model be capable of accurately predicting frequency and amplitude of oscillation.

Quality Matters - Hematopoietic Stem Cell Transplantation versus Transfusion and Chelation in Thalassemia Major.

OBJECTIVE: To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion. METHODS: The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale. RESULTS: The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92.8, respectively in transplant group and 83, 82.7 and 83.6, respectively in children in transfusion group. The adult group on transfusion showed overall poorer scores of 74.9, 76 and 73.9, respectively. The average scores in all domains were significantly (P<0.05) lower and drop steeply in second decade in transfusion group. CONCLUSIONS: Allogeneic stem cell transplantation improves quality of life in thalassemia major.

Medicines use in hospitalised children: current status and outcome after an intervention.

INTRODUCTION: Challenges in rational use of medicines (RUM) in children are different from that of adults. In Sri Lanka, data on RUM in children are limited. OBJECTIVE: To assess the current status and to investigate effectiveness of an intervention in improving RUM in children. METHODS: Non-randomised controlled before and after study design was employed. Study settings were one paediatric unit in two Teaching Hospitals one for intervention (IU) and the other as a control (CU) unit. After assessing the current status in both units, a combined intervention (one-time training and distribution of a paediatric formulary) was offered to IU and medicine use was re-assessed in both units three months and one year after intervention. Fourteen indicators (7 WHO and 7 developed by investigators) were employed in the assessment. Any improvement was analysed using percentage changes, Chi-square or t tests as appropriate. RESULTS: A total of 1134 charts, 735 (3197 medicines) in IU and 399 (1539 medicines) in CU were subjected to analysis. At base level, of the 14 indicators, 9 were assessed satisfactory in both units. Four could not be assessed without knowing the clinical setting. The remaining indicator, reason for prescribing was recorded for 48% and 76% of medicines respectively in IU and CU. After intervention, only three indicators, medicines that had the reason for prescription recorded in the patient records, children treated without regular medicines, and children received the recommended doses of paracetamol, showed favourable changes in three months and one year. Percentage of medicines written in abbreviation showed an undesirable increase in IU (6.9, 16.2, 29.6) which was higher than what was observed in CU (3.2, 13.5, 18.4). CONCLUSIONS: Passive interventions appear to be ineffective in improving RUM in children. In addition, general medicine use indicators seem to be insensitive to capture the true challenges in paediatric pharmacotherapy.

Auditory white noise reduces age-related fluctuations in balance.

Fall prevention technologies have the potential to improve the lives of older adults. Because of the multisensory nature of human balance control, sensory therapies, including some involving tactile and auditory noise, are being explored that might reduce increased balance variability due to typical age-related sensory declines. Auditory white noise has previously been shown to reduce postural sway variability in healthy young adults. In the present experiment, we examined this treatment in young adults and typically aging older adults. We measured postural sway of healthy young adults and adults over the age of 65 years during silence and auditory white noise, with and without vision. Our results show reduced postural sway variability in young and older adults with auditory noise, even in the absence of vision. We show that vision and noise can reduce sway variability for both feedback-based and exploratory balance processes. In addition, we show changes with auditory noise in nonlinear patterns of sway in older adults that reflect what is more typical of young adults, and these changes did not interfere with the typical random walk behavior of sway. Our results suggest that auditory noise might be valuable for therapeutic and rehabilitative purposes in older adults with typical age-related balance variability.

Lymphoblastic lymphoma presenting as bilateral renal enlargement diagnosed by percutaneous kidney biopsy: Report of three cases.

Renal involvement by lymphoma can be a diagnostic challenge. Acute kidney injury (AKI) is an unusual manifestation of lymphomatous infiltration in the kidneys. We report three cases of lymphoblastic lymphoma, a very rare form of lymphoma, presenting with AKI and bilateral enlargement of kidneys, diagnosed by percutaneous kidney biopsy. Lymphomatous infiltration should be suspected with such clinical presentation. Kidney biopsy is a valuable diagnostic tool, to establish the correct diagnosis and subtype of lymphoma for timely initiation of therapy for these aggressive hematological malignancies.

A Single-Step Purification of Cauliflower Lysozyme and Its Dual Role Against Bacterial and Fungal Plant Pathogens.

A novel lysozyme from cauliflower was purified in a single step, for the first time, using Sephadex G100 column chromatography. The purified lysozyme exhibited a homogenized single band in sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE), and its molecular mass was calculated to be 22.0 kDa. The purified lysozyme showed activity between 30 to 60 °C with 40 °C as the optimum temperature for its maximal activity. Although the purified lysozyme was functional at pH ranges between 3.0 and 9.0, the optimum pH for the enzyme activity was 8.0. By Michaelis-Menten equation, the threshold substrate concentration for the optimal enzyme activity was calculated to be 133.0 µg. The purified lysozyme showed extraordinary activity against plant pathogenic bacteria and fungi. At 10-µg concentrations, it inhibited the growth of plant pathogenic bacteria such as Pseudomonas syringae, Xanthomonas campestris, and Erwinia carotovora exhibiting 4.28, 5.90, and 3.88-fold inhibition, respectively. Further, it also completely inhibited the conidial germination of Archemonium obclavatum and, to a very large extent, other fungal species such as Fusarium solani (79.3 %), Leptosphaeria maculans (88.6 %), Botrytis cinera (73.3 %), Curvularia lunata (68 %), Rhizoctonia solani (79.6 %), and Alternaria alternata (83.6 %).

A national survey of price and affordability of key essential medicines for children in Sri Lanka.

OBJECTIVE: To investigate the price and affordability of key essential medicines for children in Sri Lanka. METHODS: This national survey investigated the prices and affordability of 25 key essential medicines for children in private sector pharmacies using the WHO/Health Action International (HAI) medicine price methodology. Data were collected from a representative sample of 48 private sector pharmacies selected from 8 Provinces using a multistage clustered approach. At each pharmacy prices of originator brand (OB) and lowest priced generics (LPG) of the selected medicines were collected. Medicine prices were compared with international reference prices to obtain a median price ratio (MPR). Income of the lowest paid unskilled government worker was used to establish the affordability. RESULTS: The median MPR of retail prices to patients was 3.7 for OB (range 0.23-20) and 1.35 for LPG (range 0.05-3.75). MPR >5 was observed for OBs of 5 medicines: ceftriaxone injection, diazepam injection, mebendazole syrup, mebendazole chewable tablet and metronidazole tablet whereas MPR >2.5 was observed only for LPGs of 3 medicines: chlorphenamine syrup, clotrimazole topical cream, and paracetamol syrup. Mean percent difference in price between OB and LPG products was 365 % (range -21, 2343). Rational treatment for acute infections such as mild lower respiratory tract infections and acute gastroenteritis seems to be affordable but treatment for chronic illnesses requiring liquid or inhaled dosage forms were largely unaffordable. CONCLUSIONS: Intervention is needed to improve the economic access to key essential medicines for children indicated in the treatment of chronic diseases.

Clinical study of carbapenem sensitive and resistant Gram-negative bacteremia in neutropenic and nonneutropenic patients: The first series from India.

OBJECTIVE: Carbapenem resistance is a growing global concern. There is a lack of published clinical studies on the topic from Indian subcontinent. Aim of this study was to analyze clinical profile of patients with carbapenem sensitive and resistant bacteremia among neutropenic and nonneutropenic patients. MATERIALS AND METHODS: Retrospective analysis of 141 patients who had carbapenem resistant or sensitive Gram-negative bacteremia, identified over a period of 1-year was done by medical records review, in Apollo Specialty Hospital, a 300-bedded tertiary care Oncology, neurosurgical and orthopedic center in South India. RESULTS: Of the total 141 patients with Gram-negative bacteremia, 44 had carbapenem resistant ones. Of these 44 patients, 17 were neutropenics (resistant neutropenic group) and 27 nonneutropenic patients (resistant nonneutropenic group). Of the 97 patients with carbapenem sensitive bacteremia, 43 were neutropenic (sensitive neutropenic group) and 54 nonneutropenics (sensitive nonneutropenic group). The 28 days mortality was significantly higher in carbapenem resistant bacteremic group compared to the sensitive one (P = 0.008). CONCLUSION: This is the first study from India comparing clinical features of patients with carbapenem sensitive and resistant blood stream infections. Patients with carbapenem resistant bacteremia had higher mortality compared to patients with sensitive bacteremia.