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Recognition of the importance of prostate-specific membrane antigen (PSMA) PET/CT in the diagnosis of prostate cancer has steadily increased following the publication of extensive data on its diagnostic accuracy and impact on patient management over the past decade. Several recent clinical trials and investigations regarding PSMA PET/CT have been ongoing in our country, and this examination is expected to become increasingly widespread in the future. This review explains the characteristics of PSMA PET/CT, its diagnostic capabilities and superiority over other modalities, the three proposed PSMA PET/CT interpretation criteria (the European Association of Nuclear Medicine [EANM], the Prostate Cancer Molecular Imaging Standardized Evaluation [PROMISE], and the PSMA Reporting and Data System [PSMA-RADS]), and the application of PSMA PET/CT to prostate cancer treatment (improvement of local control, irradiation of oligometastases, and salvage radiotherapy), incorporating actual clinical images and the latest findings.

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Glycogen storage diseases (GSDs) comprise a group of inherited metabolic disorders characterized by defects in glycogen metabolism, leading to abnormal glycogen accumulation in multiple tissues, most notably affecting the liver, skeletal muscle, and heart. Recent findings have uncovered the importance of glycogen metabolism in the brain, sustaining a myriad of physiological functions and linking its perturbation to central nervous system (CNS) pathology. This link resulted in classification of neurological-GSDs (n-GSDs), a group of diseases with shared deficits in neurological glycogen metabolism. The n-GSD patients exhibit a spectrum of clinical presentations with common etiology while requiring tailored therapeutic approaches from the traditional GSDs. Recent research has elucidated the genetic and biochemical mechanisms and pathophysiological basis underlying different n-GSDs. Further, the last decade has witnessed some promising developments in novel therapeutic approaches, including enzyme replacement therapy (ERT), substrate reduction therapy (SRT), small molecule drugs, and gene therapy targeting key aspects of glycogen metabolism in specific n-GSDs. This preclinical progress has generated noticeable success in potentially modifying disease course and improving clinical outcomes in patients. Herein, we provide an overview of current perspectives on n-GSDs, emphasizing recent advances in understanding their molecular basis, therapeutic developments, underscore key challenges and the need to deepen our understanding of n-GSDs pathogenesis to develop better therapeutic strategies that could offer improved treatment and sustainable benefits to the patients.

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Introduction This study aimed to evaluate the setup accuracy of the new shim mask with mouth bite compared to the standard full brain mask in stereotactic radiosurgery (SRS) and radiotherapy (SRT) treatments for brain metastases or tumors. Method A combined retrospective and prospective design was employed, involving 40 patients treated at our center. Patients previously treated using standard head masks formed the retrospective cohort, while those treated with the Shim mask and mouth bite formed the prospective cohort. Daily cone-beam computed tomography (CBCT) scans were obtained before each treatment session to ensure patient setup accuracy. Key metrics included absolute shifts in translational and rotational directions, the number of repeat CBCTs, and the time interval between CBCTs. Results The Shim mask significantly reduced the mean setup errors in the lateral translation (p=0.022) from 0.17 cm (SD=0.10) to 0.10 cm (SD=0.10), and in X-axis rotation (p=0.030) from 0.79° (SD=0.43) to 0.47° (SD=0.47). By considering cutoff points of 1 mm in translational and 1° in rotational directions, the Shim mask was significantly more accurate in the lateral direction (p=0.004). Moreover, while 70% of patients in the standard group required repeat CBCT scans, none in the Shim group did, resulting in an average time saving of 10.4 minutes per patient. Conclusion The Shim mask with mouth bite offers enhanced immobilization accuracy in SRT/SRS treatments, leading to time and potential cost savings by reducing the need for repeat CBCT scans. This underscores the importance of adopting innovative immobilization techniques to optimize patient outcomes.

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As a significant structure in activated sludge, extracellular polymeric substances (EPS) hold considerable value regarding resource recovery and applications. The present study aimed to elucidate the relationship between the microbial community and the composition and properties of EPS. A biological nutrient removal (BNR) reactor was set up in the laboratory and controlled under different solid retention times (SRT), altering microbial species within the system. Then EPS was extracted from activated and analyzed by chemical and spectroscopic methods. High-throughput sequencing and metagenomic approaches were employed to investigate bacterial community and metabolic pathways. The results showed that lower SRT with a higher abundance of the family-level Proteobacteria (27.7%-53.5%) favored EPS synthesis, while another dominant group Bacteroidetes (20.0%-32.6%) may not significantly affect EPS synthesis. Furthermore, the abundance of alginates-producing bacteria including Pseudomonas spp. and Azotobacter vinelandii was only 2.53%-6.76% and 1.98%-6.34%, respectively. The alginate synthesis pathway genes Alg8 and Alg44 were also present at very low levels (0.05‱-0.11‱, 0.01‱-0.02‱, respectively). Another important gene related to alginates operons, AlgK, was absent across all the SRT-operated reactors. These findings suggest an impossible and incomplete alginate synthesis pathway within sludge. In light of these results, it can be concluded that EPS does not necessarily contain alginate components.

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The spline reconstruction technique (SRT) is a fast algorithm based on a novel numerical implementation of an analytic representation of the inverse Radon transform. The purpose of this study was to compare the SRT, filtered back-projection (FBP), and the Tera-Tomo 3D algorithm for various iteration numbers, using small-animal dynamic PET data obtained from a Mediso nanoScan® PET/CT scanner. For this purpose, Patlak graphical kinetic analysis was employed to noninvasively quantify the myocardial metabolic rate of glucose (MRGlu) in seven male C57BL/6 mice (n=7). All analytic reconstructions were performed via software for tomographic image reconstruction. The analysis of all PET-reconstructed images was conducted with PMOD software (version 3.506, PMOD Technologies LLC, Fällanden, Switzerland) using the inferior vena cava as the image-derived input function. Statistical significance was determined by employing the one-way analysis of variance test. The results revealed that the differences between the values of MRGlu obtained via SRT versus FBP, and the variants of he Tera-Tomo 3D algorithm were not statistically significant (p > 0.05). Overall, the SRT appears to perform similarly to the other algorithms investigated, providing a valid alternative analytic method for preclinical dynamic PET studies.

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PURPOSE: SRS MapCHECK (SMC) is a commercially available patient-specific quality assurance (PSQA) tool for stereotactic radiosurgery (SRS) applications. This study investigates the effects of degree of modulation, location off-axis, and low dose threshold (LDT) selection on gamma pass rates (GPRs) between SMC and treatment planning system, Analytical Anisotropic Algorithm (AAA), or Vancouver Island Monte Carlo (VMC++ algorithm) system calculated dose distributions. METHODS: Volumetric-modulated arc therapy (VMAT) plans with modulation factors (MFs) ranging from 2.7 to 10.2 MU/cGy were delivered to SMC at isocenter and 6 cm off-axis. SMC measured dose distributions were compared against AAA and VMC++ via gamma analysis (3%/1 mm) with LDT of 10% to 80% using SNC Patient software. RESULTS: Comparing on-axis SMC dose against AAA and VMC++ with LDT of 10%, all AAA-calculated plans met the acceptance criteria of GPR ≥ 90%, and only one VMC++ calculated plan was marginally outside the acceptance criteria with pass rate of 89.1%. Using LDT of 80% revealed decreasing GPR with increasing MF. For AAA, GPRs reduced from 100% at MF of 2.7 MU/cGy to 57% at MF of 10.2 MU/cGy, and for VMC++ calculated plans, the GPRs reduced from 89% to 60% in the same MF range. Comparison of SMC dose off-axis against AAA and VMC++ showed more pronounced reduction of GPR with increasing MF. For LDT of 10%, AAA GPRs reduced from 100% to 83% in the MF range of 2.7 to 9.8 MU/cGy, and VMC++ GPR reduced from 100% to 91% in the same range. With 80% LDT, GPRs dropped from 100% to 42% for both algorithms. CONCLUSIONS: MF, dose calculation algorithm, and LDT selections are vital in VMAT-based SRT PSQA. LDT of 80% enhances sensitivity of gamma analysis for detecting dose differences compared to 10% LDT. To achieve better agreement between calculated and SMC dose, it is recommended to limit the MF to 4.6 MU/cGy on-axis and 3.6 MU/cGy off-axis.

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Darier's disease (DD) is an autosomal dominant genodermatosis characterized by hyperkeratotic papules, often accompanied by scaling and crusting. Managing DD presents significant challenges due to the absence of an effective cure, with only symptom targeting treatments currently available. This study presents a case of refractory DD that showed poor response to established pharmacological treatments but demonstrated improvement with low-dose superficial X-ray radiotherapy (SRT). The radiation was delivered as a single 200 cGy treatment, which visibly improved the condition. Considering the different degrees of side effects, sequelae, and risk of developing radiation-induced cancer after exposure to moderate levels of radiation, it may be considered that we attempt to treat recalcitrant DD initially by applying a low dose of radiation in order to mitigate these undesired side effects. If larger doses or additional courses are necessary due to inadequate response, the risks and benefits must be carefully evaluated and discussed with patients.

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Hearing loss is frequently associated with Gaucher disease (GD). Gaucher cells are enlarged reticuloendothelial cells containing glucocerebroside in the lysosomes due to deficiency of the glucocerebrosidase. Gaucheromas consist of accumulated Gaucher cells. Gaucher cells accumulate in variable tissues including the liver, spleen, bone marrow, and the middle ear and the mastoid causing conductive hearing loss. Neurons and astrocytes in the central nervous system are affected in neuronopathic GD leading to sensorineural hearing loss. Gaucheromas can develop even in patients treated with enzyme replacement therapy (ERT). We report a 19-year-old female patient with GD type 3 who developed profound bilateral hearing loss associated with intracranial Gaucheroma. Combination therapy of ERT with imiglucerase and substrate reduction therapy (SRT) with eliglustat significantly decreased the size of Gaucher cells and cleared the characteristic microtubular structures in the lysosomes in Gaucher cells. Early implementation of SRT may prevent at least conductive hearing impairment in GD although it may not prevent sensorineural hearing loss due to inner hair cell dysfunction which is also known to be associated with neuronopathic GD.

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Updating of the current Urban Waste Water Treatment Directive (91/271/EEC) will demand stricter regulations for nutrients removal. In this frame, wastewater treatment plants (WWTPs) of small-to-medium potential will face new challenges for achieving process intensification. Integrating intermittent aeration (IA) and integrated fixed-film activated sludge (IFAS) technologies could be a promising solution to meet such requirements. This study analyzed how IA cycles affected nutrients removal in IFAS reactors with different biofilm carriers (e.g., plastic and sponge media). The plants responses to different carbon/nitrogen/phosphorous (C/N/P) ratios were evaluated while operating under low sludge retention time (SRT) to simulate overloaded conditions. A short IA cycle (1 h) with an aeration/not aeration ratio of 2:1 enabled high organic carbon and nitrification performances when operating at high C/N/P (11.8/1/1), whereas low denitrification and phosphorous removal yields were obtained because of the short not-aerated phase. Decreasing C/N ratio (8.8/1/1) without changing the IA cycle resulted in nitrification worsening because of the reduced metabolic kinetics of biofilm. Under such load conditions, a higher IA cycle (2 h) was necessary to improve process performance. A longer not-aerated phase was also positive for denitrification and phosphorous removal because of the establishment of anoxic and anaerobic environments within the bulk and inner biofilm layers. Besides, results suggested that sponge carriers offered advantages over plastic ones, enabling a higher biofilm retention capacity, better nutrient removal, as well as robustness and resilience to operating condition changes. This would result in simpler management systems for implementing the IA process, thus reducing process complexity and costs.

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Evidence-based medicine drives patient care decision-making; thus, accurate and complete reporting in scientific publications is paramount. A checklist for complete reporting of veterinary radiation therapy was proposed in 2012 using the recommendations of the International Commission of Radiation Units and Measurements (ICRU). The purpose of this study is to review the 2012 checklist and propose updates based on changes in technology. Significant technology advancements have gained traction in veterinary medicine, including intensity-modulated radiation therapy (IMRT) and stereotactic radiation therapy (SRT), both of which have related modality-specific ICRU reporting recommendations. The 2012 checklist and proposed 2024 checklist are then used to assess the completeness of reporting in veterinary radiation oncology publications between 2015 and 2022, of which one hundred and eight publications met the inclusion criteria. Prior to the publication of the 2012 checklist (2005-2010), only nine checklist items showed a good level of completeness in reporting, and from 2015 to 2022, this increased to 16 items. Encouraging and/or requiring the use of reporting checklists at the time of manuscript submission may be responsible for this improvement in reporting. Using the 2024 checklist, which is more relevant to publications discussing IMRT and SRT treatments, only 14 of the analyzed checklist items (34%) show a good level of completeness in reporting, suggesting there is a need for updated guidelines to capture the nuances of advanced techniques. This study proposes a 2024 checklist that can be used as a guideline for future reporting of radiation therapy in veterinary medicine.

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BACKGROUND: Enhancing Local Control (LC) of brain metastases is pivotal for improving overall survival, which makes the prediction of local treatment failure a crucial aspect of treatment planning. Understanding the factors that influence LC of brain metastases is imperative for optimizing treatment strategies and subsequently extending overall survival. Machine learning algorithms may help to identify factors that predict outcomes. METHODS: This paper systematically reviews these factors associated with LC to select candidate predictor features for a practical application of predictive modeling. A systematic literature search was conducted to identify studies in which the LC of brain metastases is assessed for adult patients. EMBASE, PubMed, Web-of-Science, and the Cochrane Database were searched up to December 24, 2020. All studies investigating the LC of brain metastases as one of the endpoints were included, regardless of primary tumor type or treatment type. We first grouped studies based on primary tumor types resulting in lung, breast, and melanoma groups. Studies that did not focus on a specific primary cancer type were grouped based on treatment types resulting in surgery, SRT, and whole-brain radiotherapy groups. For each group, significant factors associated with LC were identified and discussed. As a second project, we assessed the practical importance of selected features in predicting LC after Stereotactic Radiotherapy (SRT) with a Random Forest machine learning model. Accuracy and Area Under the Curve (AUC) of the Random Forest model, trained with the list of factors that were found to be associated with LC for the SRT treatment group, were reported. RESULTS: The systematic literature search identified 6270 unique records. After screening titles and abstracts, 410 full texts were considered, and ultimately 159 studies were included for review. Most of the studies focused on the LC of the brain metastases for a specific primary tumor type or after a specific treatment type. Higher SRT radiation dose was found to be associated with better LC in lung cancer, breast cancer, and melanoma groups. Also, a higher dose was associated with better LC in the SRT group, while higher tumor volume was associated with worse LC in this group. The Random Forest model predicted the LC of brain metastases with an accuracy of 80% and an AUC of 0.84. CONCLUSION: This paper thoroughly examines factors associated with LC in brain metastases and highlights the translational value of our findings for selecting variables to predict LC in a sample of patients who underwent SRT. The prediction model holds great promise for clinicians, offering a valuable tool to predict personalized treatment outcomes and foresee the impact of changes in treatment characteristics such as radiation dose.

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AIM: AMPK can be considered as an important target molecule for cancer for its unique ability to directly recognize cellular energy status. The main aim of this study is to explore the role of different AMPK activators in managing cancer cell aggressiveness and to understand the mechanistic details behind the process. MAIN METHODS: First, we explored the AMPK expression pattern and its significance in different subtypes of lung cancer by accessing the TCGA data sets for LUNG, LUAD and LUSC patients and then established the correlation between AMPK expression pattern and overall survival of lung cancer patients using Kaplan-Meire plot. We further carried out several cell-based assays by employing different wet lab techniques including RT-PCR, Western Blot, proliferation, migration and invasion assays to fulfil the aim of the study. KEY FINDINGS: SIGNIFICANCE: This study identifies the importance of AMPK activators as a repurposing agent for combating lung and colon cancer cell aggressiveness. It also suggests SRT-1720 as a potent repurposing agent for cancer treatment especially in NSCLC patients where a point mutation is present in LKB1.

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INTRODUCTION: The standard treatment for patients with focal drug-resistant epilepsy (DRE) who are not eligible for open brain surgery is the continuation of anti-seizure medication (ASM) and neuromodulation. This treatment does not cure epilepsy but only decreases severity. The PRECISION trial offers a non-invasive, possibly curative intervention for these patients, which consist of a single stereotactic radiotherapy (SRT) treatment. Previous studies have shown promising results of SRT in this patient population. Nevertheless, this intervention is not yet available and reimbursed in the Netherlands. We hypothesize that: SRT is a superior treatment option compared to palliative standard of care, for patients with focal DRE, not eligible for open surgery, resulting in a higher reduction of seizure frequency (with 50% of the patients reaching a 75% seizure frequency reduction at 2 years follow-up). METHODS: In this waitlist-controlled phase 3 clinical trial, participants are randomly assigned in a 1:1 ratio to either receive SRT as the intervention, while the standard treatments consist of ASM continuation and neuromodulation. After 2-year follow-up, patients randomized for the standard treatment (waitlist-control group) are offered SRT. Patients aged ≥ 18 years with focal DRE and a pretreatment defined epileptogenic zone (EZ) not eligible for open surgery will be included. The intervention is a LINAC-based single fraction (24 Gy) SRT treatment. The target volume is defined as the epileptogenic zone (EZ) on all (non) invasive examinations. The seizure frequency will be monitored on a daily basis using an electronic diary and an automatic seizure detection system during the night. Potential side effects are evaluated using advanced MRI, cognitive evaluation, Common Toxicity Criteria, and patient-reported outcome questionnaires. In addition, the cost-effectiveness of the SRT treatment will be evaluated. DISCUSSION: This is the first randomized trial comparing SRT with standard of care in patients with DRE, non-eligible for open surgery. The primary objective is to determine whether SRT significantly reduces the seizure frequency 2 years after treatment. The results of this trial can influence the current clinical practice and medical cost reimbursement in the Netherlands for patients with focal DRE who are not eligible for open surgery, providing a non-invasive curative treatment option. TRIAL REGISTRATION: Clinicaltrials.gov Identifier: NCT05182437. Registered on September 27, 2021.

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While procedural learning (PL) has been implicated in delayed motor skill observed in developmental coordination disorder (DCD), few studies have considered the impact of co-occurring attentional problems. Furthermore, the neurostructural basis of PL in children remains unclear. We investigated PL in children with DCD while controlling for inattention symptoms, and examined the role of fronto-basal ganglia-cerebellar morphology in PL. Fifty-nine children (6-14 years; nDCD = 19, ncontrol = 40) completed the serial reaction time (SRT) task to measure PL. The Attention-Deficit Hyperactivity Disorder Rating Scale-IV was administered to measure inattention symptoms. Structural T1 images were acquired for a subset of participants (nDCD = 10, ncontrol = 28), and processed using FreeSurfer. Volume was extracted for the cerebellum, basal ganglia, and frontal regions. After controlling for inattention symptoms, the reaction time profile of controls was consistent with learning on the SRT task. This was not the case for those with DCD. SRT task performance was positively correlated with cerebellar cortical volume, and children with DCD trended towards lower cerebellar volume compared to controls. Children with DCD may not engage in PL during the SRT task in the same manner as controls, with this differential performance being associated with atypical cerebellar morphology.

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PURPOSE: Single-isocenter multi-target intracranial stereotactic radiotherapy (SIMT) is an effective treatment for brain metastases with complex treatment plans and delivery optimization necessitating rigorous quality assurance. This work aims to assess five methods for quality assurance of SIMT treatment plans in terms of their suitability and sensitivity to delivery errors. METHODS: Sun Nuclear ArcCHECK and SRS MapCHECK, GafChromic EBT Radiochromic Film, machine log files, and Varian Portal Dosimetry were all used to measure 15 variations of a single SIMT plan. Variations of the original plan were created with Python. They comprised various degrees of systematic MLC offsets per leaf up to 2 mm, random per-leaf variations with differing minimum and maximum magnitudes, simulated collimator, and dose miscalibrations (MU scaling). The erroneous plans were re-imported into Eclipse and plan-quality degradation was assessed by comparing each plan variation to the original clinical plan in terms of the percentage of clinical goals passing relative to the original plan. Each erroneous plan could be then ranked by the plan-quality degradation percentage following recalculation in the TPS so that the effects of each variation could be correlated with γ pass rates and detector suitability. RESULTS & CONCLUSIONS: It was found that 2%/1 mm is a good starting point for the ArcCHECK, Portal Dosimetry, and the SRS MapCHECK methods, respectively, and provides clinically relevant error detection sensitivity. Looser dose criteria of 5%/1 mm or 5%/1.5 mm are suitable for film dosimetry and log-file-based methods. The statistical methods explored can be expanded to other areas of patient-specific QA and detector assessment.

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INTRODUCTION: Purpose of our study was to compare two competing methods of performing bisyllabic word speech audiometry for the detection of the 50% speech reception threshold in noise (SRT50). METHODS: Classic method is performed submitting multiple word lists at a fixed signal-to-noise ratio. A newer Fast method - Italian Fast Speech Reception Threshold 50 (IFastSRT50) - is performed by means of program software with a single list of bisyllabic words and noise intensity shifting. RESULTS: Means comparison between SRT50 Classic and IFastSRT50 shows a slight significant correlation (r = 0.263; p = 0.044) and a wide significant difference: SRT50 Classic = -2.763 dB (SD = 4.1) and IFastSRT50 = -7.803 dB (SD = 2.1) (p < 0.0001). There is a high difference between the test execution time means (SRT50 Classic = 11 min, IFastSRT50 = 2 min; p < 0.0001). The correlation between test results and execution times was higher for SRT50 Classic than IFastSRT50. CONCLUSION: IFastSRT50 test is a reliable method to quickly investigate signal-to-noise ratio needed to obtain 50% of recognition scores with bisyllabic words; it allows less execution time than SRT50 Classic method and can avoid patient fatigue and other limitations of different speech discrimination tests in noise as sentences based ones.

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Gaucher disease (GD), a rare hereditary lysosomal storage disorder, occurs due to a deficiency in the enzyme ß-glucocerebrosidase (GCase). This deficiency leads to the buildup of substrate glucosylceramide (GlcCer) in macrophages, eventually resulting in various complications. Among its three types, GD2 is particularly severe with neurological involvements. Current treatments, such as enzyme replacement therapy (ERT), are not effective for GD2 and GD3 due to their inability to cross the blood-brain barrier (BBB). Other treatment approaches, such as gene or chaperone therapies are still in experimental stages. Additionally, GD treatments are costly and can have certain side effects. The successful use of messenger RNA (mRNA)-based vaccines for COVID-19 in 2020 has sparked interest in nucleic acid-based therapies. Remarkably, mRNA technology also offers a novel approach for protein replacement purposes. Additionally, self-amplifying RNA (saRNA) technology shows promise, potentially producing more protein at lower doses. This review aims to explore the potential of a cost-effective mRNA/saRNA-based approach for GD therapy. The use of GCase-mRNA/saRNA as a protein replacement therapy could offer a new and promising direction for improving the quality of life and extending the lifespan of individuals with GD.

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This study aimed to compare procedural learning skills between Spanish-speaking preschool children (ages 4 years to 4 years, 11 months) with developmental language disorder (DLD) and their chronologically matched typically developing (TD) peers. Using the serial reaction time (SRT) task, participants (30 children with DLD and 30 TD children) responded to visual stimuli in a sequenced manner over four blocks, followed by a random order block. The task assessed reaction time (RT) and accuracy. The results showed a significant interaction between group and block for RT and accuracy, with children with DLD exhibiting longer RTs and accuracy deficits across blocks. In contrast, the TD group showed higher RT efficiency and accuracy in the sequential blocks and, as expected, decreased performance in the random block according to the experimental manipulation. Overall, the results of this investigation suggest that there was no implicit learning in the DLD group, as indicated by the SRT task paradigms of procedural memory. These findings align with some aspects of the procedural deficit hypothesis (PDH), which suggests that linguistic deficits in the DLD population may derive from a deficit in sequential learning from the procedural memory system domain in the Spanish context.

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Gaucher disease is an inherited disorder in which there is a deficiency of the enzyme glucocerebrosidase, which leads to the accumulation of glucosylceramide. Although much scientific evidence is now available, there is still limited data on the impact on the different life stages of women with this disease. Among other alterations, a delay in menarche has been described, although it has not been related to fertility problems. Menorrhagia is relatively frequent, being related to the presence of thrombocytopenia, thrombocytopathies or coagulation disorders. On the other hand, pregnancy planning is an increasingly frequent concern. All patients should undergo genetic counseling, and it is important to monitor the appearance or worsening of organomegaly, bone and hematologic abnormalities to establish clinical and therapeutic recommendations. Management during the puerperium will depend on the evolution of gestation, and, during the lactation period, the potential appearance of bone complications should be assessed. An early onset of menopause, compared to the general population, has also been described, which may accelerate the development of osteopenia. Finally, although the usual screening protocols for neoplasms are currently being performed, it is recommended to watch for early signs of liver or renal neoplasms when examining the results of imaging tests performed during evaluations for this disease.