Uncertain Prognosis Research Articles

"Finding my way in a maze while the clock is ticking": The daily life challenges of adolescents and young adults with an uncertain or poor cancer prognosis.

Increasingly more adolescent and young adult (AYA, aged 18-39 years) patients with an uncertain and/or poor cancer prognosis (UPCP) are gaining life-years because of novel treatments or refinement of established therapies, and sometimes even face the prospect of long-term disease control. This study aims to examine the challenges of AYAs with a UPCP in daily life to inform the development of AYA care programs. Semi-structured in-depth interviews were conducted among AYAs with a UPCP. Since we expected differences in experiences between three AYA subgroups, we interviewed patients of these subgroups (1): traditional survivors (2), low-grade glioma survivors, and (3) new survivors. Interviews were analyzed using elements of grounded theory. AYA patients were actively involved as research partners. In total 46 AYAs with UPCP participated and shared their challenges in daily life. They were on average 33.4 years old (age range 23-44) and most of them were women (63%). The most common tumor types were low-grade gliomas (16), sarcomas (7), breast cancers (6), and lung cancers (6). We identified five primary themes: (1) feeling inferior to previous self and others (e.g. feeling useless, who wants me in a relationship), (2) feeling of being alone (e.g. lonely thoughts, nobody really gets me), (3) ongoing confrontation (e.g. it is always there, own decline), (4) grief about life (e.g. grief about life I did not get, grief about old life), and (5) loss of control over the future (e.g. not able to make future plans, waiting for growth). Although all of the challenges were identified in the three AYA subgroups, the perceived intensity of the challenges differed slightly between the subgroups. AYAs living with a UPCP experience challenges associated to their sense of altered identity, their position in the social network, and the future uncertainties. This study highlights the importance to recognize and acknowledge the unique challenges of this group. To provide age-specific care, it is important to embed acceptance and commitment therapy and AYA peer support within the healthcare system and other care programs to support AYAs to live well with their disease.

Gentle Correctness Verification of the Theory of Uncertain Event Prognosis to Compute Failure Time Probability

Prognosis of the time of first occurrence of events, particularly failures, is a problem that has been studied and analyzed from different perspectives, being referred to as the “first-hitting time” or “first-passage time” problem, among other names. Within the Prognostics and Health Management community, as well as in other disciplines, the probabilistic benchmark for computing the Time-of-Failure consists of Monte Carlo simulations where the failure event is defined as the moment when the health of the monitored system, typically described by a health indicator, crosses a predetermined threshold that denotes a region known as hazard zone. More recently, this problem was formalized through the introduction of new probability measures based on the concept of uncertain event, which correct widely used expressions for these purposes, and extend the typical threshold criterion for declaring a failure (or other event) to a broader notion of uncertain event likelihood. Following a step-by-step approach supported by code programmed in Python language, this paper verifies the correctness of the aforementioned probability measures, illustrating with a simple example how exactly the same results are obtained using either these new probability measures or the universally accepted benchmark.

3D Polycaprolactone/Gelatin-Oriented Electrospun Scaffolds Promote Periodontal Regeneration.

Periodontitis is a worldwide chronic inflammatory disease, where surgical treatment still shows an uncertain prognosis. To break through the dilemma of periodontal treatment, we fabricated a three-dimensional (3D) multilayered scaffold by stacking and fixing electrospun polycaprolactone/gelatin (PCL/Gel) fibrous membranes. The biomaterial displayed good hydrophilic and mechanical properties. Besides, we found human periodontal ligament stem cell (hPDLSC) adhesion and proliferation on it. The following scanning electron microscopy (SEM) and cytoskeleton staining results proved the guiding function of fibers to hPDLSCs. Then, we further analyzed periodontal regeneration-related proteins and mRNA expression between groups. In vivo results in a rat acute periodontal defect model confirmed that the topographic cues of materials could directly guide cellular orientation and might provide the prerequisite for further differentiation. In the aligned scaffold group, besides new bone regeneration, we also observed that angular concentrated fiber regeneration in the root surface of the defect is similar to the normal periodontal tissue. To sum up, we have constructed electrospun membrane-based 3D biological scaffolds, which provided a new treatment strategy for patients undergoing periodontal surgery.

Prognostic role of cardiac magnetic resonance in left ventricular non compaction

Abstract Introduction Left ventricular non compaction (LVNC) is a heterogeneous entity with uncertain prognosis. Cardiac magnetic resonance (CMR) is widely used in the diagnosis of LVNC. However, its role in risk stratification has not been well established. Purpose Therefore, the aim of our study was to identify prognostic CMR variables in LVNC. Methods We conducted a retrospective longitudinal multicentre cohort study of consecutive patients fulfilling CMR LVNC criteria. The endpoints were heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause mortality. Biventricular volumes, ejection fraction (LVEF and RVEF) as well as late gadolinium enhancement (LGE) were analysed. Results A total of 310 patients were included: age was 44.4±19 and 43% female. LVEF was 47% ± 15%, RVEF was 48±12 and 28 patients (9%) presented LGE. After a median follow-up of 3.8 2.5 years, 40 patients (13%) presented HF, 31 (10%) had VA, SE occurred in 6 (2%) and 3 patients (1%) died. Baseline characteristics of patients with and without HF and VA are described in Table 1. In univariate analysis, LVEF, LV volumes, LGE, and RVEF were associated with both HF and VA risk. In multivariate analysis, LVEF was the only variable independently associated with HF (HR 0.932, CI 95% 0.88–0.97, p 0.003). Patients with an LVEF >35% were at very low risk of HF (Figure 1A). With regards to VA, LGE was the only independent predictor (HR 2.64, IC 95% 1.059–6.61, p 0.003) (Figure 1B). In LGE negative patients, the arrhythmic risk was higher among those with an LVEF <35% (HR 2.81, 95% CI 1.02–8.12, p 0.047). Conclusions In a large multicentre retrospective LVNC study, left ventricular ejection fraction and late gadolinium enhancement were the main predictors of cardiovascular events. Patients with an LVEF <35% and with LGE were at markedly increased risk. Therefore, we suggest that these variables should be combined to enhance risk stratification in LVNC. Funding Acknowledgement Type of funding sources: None.

ADVANCED-CARE PLANNING IN PULMONARY HYPERTENSION AND INTERSTITIAL LUNG DISEASE: NEEDS ASSESSMENT AND DEVELOPMENT OF A QUALITY IMPROVEMENT PROCESS

ADVANCED-CARE PLANNING IN PULMONARY HYPERTENSION AND INTERSTITIAL LUNG DISEASE: NEEDS ASSESSMENT AND DEVELOPMENT OF A QUALITY IMPROVEMENT PROCESS

Application of the Intensity Normalization Indicators method for predicting occupational morbidity in leading industries

Introduction. Identification of risk factors for the occurrence of occupational diseases by comparing various prognostic criteria is one of the most important tasks of hygiene and occupational health. The purpose of the study is to develop a prognostic matrix of occupational morbidity (OM) in the leading industries of the Irkutsk region for a set of main predictor factors using the method of intensity normalization indicators (INI). Materials and methods. There was used an information array of long-term OM in the leading industries of the Irkutsk region. The method of calculating weight indices was used to assess the strength of the influence of OM predictor factors and the INI method was used to calculate the prognostic significance of the factors. Results. The risk of occupational disease (OD) has been established to be associated with belonging to a certain occupation (OR=61.8), combined exposure to harmful factors in the working environment (OR=23.8), due to the imperfection of the technological process and equipment. The work experience with a harmful occupational factor, the age of employee, the industry are also significant, but the degree of their influence on the occurrence of OD is much lower. Based on the predictor factors, the risk of OD was calculated, its possible range (0.019-0.412 conventional units) with three subranges (favourable, uncertain and unfavourable prognosis) was determined. The risks of OD in persons with a work experience of 1-4 years, under the age of 40, exposed to the combined effects of physical factors, working as excavator driver and an assembler-riveter, were 0.269 and 0.226 (uncertain forecast). The risk values of OD in workers of these occupations increase by 24.4% and 29.1%, respectively, with an increase in length of service and age, reaching a maximum at the age of 50-59 years with an work experience of 30 or more years (0.334 and 0.292, unfavourable prognosis). The limitations of this INI model include the non-inclusion of clinical, functional, biochemical and socio-psychological indicators of workers among the predictor factors. Limitations. An analysis of one thousand eight hundred sixty two cases of newly diagnosed occupational diseases over a 10-year period, 11 main predictor factors, which is a sufficient reference sample, was made to study occupational morbidity in the leading industries of the Irkutsk region and developing a prognostic matrix. Conclusion. The use of INI makes it possible to give an integrated risk assessment of the OD both for individual factors and for their complex, and to determine risk groups.

Perinatal stroke (a case report)

Perinatal stroke and, in particular, intracranial hemorrhage in fetus refer to topical issues of modern perinatology and perinatal neurology because of diagnostic challenges and uncertain prognosis. The severity of outcome in intracranial hemorrhage in fetus depends on its localization and affected area. We report a case of fetal stroke in а newborn. In the patient, initial minimal clinical signs were accompanied by pathological changes detected by visual diagnostic methods and electroencephalography. This makes prognosis more challenging and requires the vigilance of neonatologists and pediatric neurologists.

Experiences of resuming life after immunotherapy and associated survivorship care needs: a qualitative study among patients with metastatic melanoma.

BackgroundImmune checkpoint inhibitors (ICIs) have significantly improved the overall survival of patients with metastatic melanoma. It is unclear how the growing group of metastatic melanoma survivors resume their lives after treatment, and which needs they have regarding survivorship care (SSC).ObjectivesTo gain an in‐depth understanding of metastatic melanoma survivors’ experiences of resuming life after ICIs and their associated SSC needs.MethodsA qualitative study was conducted among 20 patients with metastatic melanoma in whom ICIs had been discontinued after ongoing tumour response. One focus group (n = 9) was held, which was complemented by 11 individual interviews. Purposive sampling was used to select a variable sample in terms of sex, age, time since discontinuation of ICIs, and perceived impact of the disease. A topic guide was used to structure the (group) interviews, which were transcribed verbatim and analysed in a thematic content analysis, using several phases of coding.ResultsIn resuming life after ICIs, the prognosis switch often caused mixed feelings among patients, mainly because of the uncertainty about the future. Demands and expectations from self and others, persistent complaints and new problems in different life domains often make it challenging to proceed with life as it was prior to metastatic cancer. Patients indicated they needed to find a new balance, which included learning to cope with uncertainty and a changed perspective on life and close relationships. In terms of SSC needs, patients particularly stressed the need for more tailored patient information, available at one location. In addition, they emphasized the need to know who to turn to in case of questions and indicated the need for psychosocial support, also for their close relatives.ConclusionsMetastatic melanoma survivors face various challenges in resuming life after ICIs and are left with several unmet SSC needs. Efforts should be focused on offering psychosocial supportive care in addition to medical care, from diagnosis onwards, taking into account the patient’s close relatives. A single point of contact and personalized survivorship care plan (SCP) could be of added value in guiding them through the patient journey, which is, given its multidisciplinary nature, particularly important in melanoma care. What is already known about this topic? Since the introduction of immune checkpoint inhibitors (ICIs) the overall survival of patients with metastatic melanoma has improved significantly, leading to a growing group of melanoma survivors.Melanoma survivors may face various problems and challenges in resuming life after treatment, which may be associated with unmet survivorship care (SSC) needs.An in‐depth understanding of their experiences with resuming life and the associated SSC needs is currently lacking. What does this study add? Metastatic melanoma survivors experience various challenges after immunotherapy, from the uncertain prognosis switch to the struggle of finding a new balance in life.Besides negative aspects, such as complaints in different life domains, the patient journey is often accompanied by positive outcomes, for example a changed perspective on life.They stress the need for tailored patient information and broader supportive care, also for their close relatives. What are the clinical implications of this work? In addition to medical care, efforts should be focused on offering psychosocial supportive care, including return‐to‐work issues, from diagnosis onwards, ideally taking into account the patient’s close relatives.To guide them through the patient journey, a single point of contact and a personalized survivorship care plan (SCP) could be of added value.The latter is particularly important in melanoma care, given its multidisciplinary nature.

A clinical decision support system optimising adjuvant chemotherapy for colorectal cancers by integrating deep learning and pathological staging markers: a development and validation study

The DoMore-v1-CRC marker was recently developed using deep learning and conventional haematoxylin and eosin-stained tissue sections, and was observed to outperform established molecular and morphological markers of patient outcome after primary colorectal cancer resection. The aim of the present study was to develop a clinical decision support system based on DoMore-v1-CRC and pathological staging markers to facilitate individualised selection of adjuvant treatment. We estimated cancer-specific survival in subgroups formed by pathological tumour stage (pT<4 or pT4), pathological nodal stage (pN0, pN1, or pN2), number of lymph nodes sampled (≤12 or >12) if not pN2, and DoMore-v1-CRC classification (good, uncertain, or poor prognosis) in 997 patients with stage II or III colorectal cancer considered to have no residual tumour (R0) from two community-based cohorts in Norway and the UK, and used these data to define three risk groups. An external cohort of 1075 patients with stage II or III R0 colorectal cancer from the QUASAR 2 trial was used for validation; these patients were treated with single-agent capecitabine. The proposed risk stratification system was evaluated using Cox regression analysis. We similarly evaluated a risk stratification system intended to reflect current guidelines and clinical practice. The primary outcome was cancer-specific survival. The new risk stratification system provided a hazard ratio of 10·71 (95% CI 6·39-17·93; p<0·0001) for high-risk versus low-risk patients and 3·06 (1·73-5·42; p=0·0001) for intermediate versus low risk in the primary analysis of the validation cohort. Estimated 3-year cancer-specific survival was 97·2% (95% CI 95·1-98·4; n=445 [41%]) for the low-risk group, 94·8% (91·7-96·7; n=339 [32%]) for the intermediate-risk group, and 77·6% (72·1-82·1; n=291 [27%]) for the high-risk group. The guideline-based risk grouping was observed to be less prognostic and informative (the low-risk group comprised only 142 [13%] of the 1075 patients). Integrating DoMore-v1-CRC and pathological staging markers provided a clinical decision support system that risk stratifies more accurately than its constituent elements, and identifies substantially more patients with stage II and III colorectal cancer with similarly good prognosis as the low-risk group in current guidelines. Avoiding adjuvant chemotherapy in these patients might be safe, and could reduce morbidity, mortality, and treatment costs. The Research Council of Norway.

Large Vertex Encephaloceles: Management and Outcomes.

Complex vertex and posterior encephaloceles containing brain tissue have uncertain prognosis and high operative risk. Patients may not be offered operative intervention depending on local and regional specialist expertise. The authors present their experience treating 5 such pediatric patients. This is a retrospective review of the surgical assessment, planning, and technique of cranial repairs, as well as surgical outcomes and developmental follow-up regarding adaptive functioning for patients presenting for second opinion for encephalocele of the cranial vertex after having been deemed too high risk at another institution. Five consecutive patients presented between January 2014 and June 2016. One patient was not offered repair. Of 4 patients who underwent reconstruction, average age at time of repair was 2.7 months (range, 0.9-6.7). One presented with ruptured encephalocele, whereas the remaining 3 underwent drainage of the encephalocele (average volume of 1200mL) at time of surgical resection. Operative time averaged 3.7 hours (range, 2.2-5.3). There were no deaths. One patient had a single seizure postoperatively. Two patients required placement of permanent shunt for hydrocephalus. Two patients completed developmental evaluations, both of whom exhibited delays in adaptive functioning relative to same-aged peers. Patients with large, complex encephalocele warrant evaluation by an experienced high-volume tertiary care pediatric craniofacial center. The decision to proceed with surgical management should include an interdisciplinary team of surgeons, anesthesiologists, neurologists, and social work. Further study of developmental outcomes in both operated and unoperated patients is necessary to better understand risks and benefits of reconstruction.

Tracheotomy During Pandemic COVID-19 Outbreak. Experience At University Clinical Center Tuzla

Objective: The aim of the study was to present experience of performed tracheotomies during the Covid19 pandemic and to outline the adjustments made to the procedure for security reasons. Materials and Methods: In the retrospective study for the period March 2020 to April 2022 we analyzed the disease history data and surgical findings from all patients in UCC Tuzla who underwent surgical tracheotomy during the COVID-19 pandemic. Results: 52 patients who underwent open surgical tracheotomy after an invasive mechanical ventilation were analyzed in our study. Group A were 32 COVID-19 consecutive patients (22 male, mean age±13.54 years, range 23-76). The tracheotomy was performed approximately on day 12.4 of the intubation (range 4-28). Group B consisted of 22 patients who had not suffered from COVID-19, and their PCR test was negative for SARSCov-2 (12 male, mean age 59.4±20.40 years, range 19-87). The tracheotomy was performed approximately on day 10.1 of the intubation (range 2-20). There was a statistically significant difference in mortalities when both groups were compared. The most common complication was diffuse bleeding from soft tissue of the neck in the early post tracheotomy period and local infection in the later period. The most common comorbidities were arterial hypertension and diabetes mellitus. Conclusion: According to our study results, COVID-19 elderly patients who are on Invasive Mechanical Ventilation (IMV) have an uncertain prognosis. Correct timing of the tracheotomy is necessary so as not to further traumatize the patients.

Apocrine adenocarcinoma of the breast: A rare special subtype with divergent theranostic markers

ABSTRACT Carcinoma of the breast with apocrine differentiation also known as apocrine adenocarcinomas is special histological subtypes comprising approximately 1% of breast cancers. They are estrogen receptor/progesterone receptor-negative and androgen receptor-positive tumors having more than 90% population of tumor cells with apocrine morphology. We present a 49-year-old woman with a breast lump in the right upper outer quadrant clinically and radiologically diagnosed as malignancy and histologically proven as apocrine adenocarcinoma of the breast owing to the morphology comprising tumor cells having abundant granular cytoplasm, central to the eccentric nucleus and prominent nucleoli. On immunohistochemistry, it was a triple-negative tumor with AR positivity. As apocrine adenocarcinoma of the breast has an uncertain prognosis, variable HER2/neu overexpression, debatable responses to neoadjuvant therapy, and probable response to androgen therapy, the onus of diagnosing and reporting these tumors accurately lies with the pathologist. Moreover, as the presentation of these tumors is similar to invasive breast carcinoma, no special type but with potentially different and useful theranostic markers, an emphasis on specifying this histological subtype is becoming increasingly essential.

A qualitative study on the involvement of adolescents and young adults (AYAs) with cancer during multiple research phases: “plan, structure, and discuss”

BackgroundIncluding the lived experience of patients in research is important to improve the quality and outcomes of cancer studies. It is challenging to include adolescents and young adults (AYAs) cancer patients in studies and this accounts even more for AYAs with an uncertain and/or poor prognosis (UPCP). Little is known about involving these AYAs in scientific research. However, by including their lived experiences during multiple phases of research, the quality of the study improves and therefore also the healthcare and quality of life of this unique patient group. We first aimed to document experiences of AYAs and researchers with AYA involvement initiatives using the Involvement Matrix and the nine phases of the research cycle. Second, we aimed to map the (expected) challenges and recommendations, according to patients and researchers, for AYA involvement in each research phase.MethodsThirteen semi-structured qualitative interviews were conducted with AYAs and researchers from February 2020 to May 2020. A thematic analysis codebook with a critical realistic framework was used to analyze the data.ResultsAYAs and researchers were predominantly positive about AYA involvement within six of the nine phases of research: identify and prioritize topics, develop study design, disseminate information, implement, and evaluate findings. Not all respondents were positive about AYA involvement in the following three phases: formulate research questions, conduct research, and analysis and interpretation. However, few respondents had experience with AYA-researcher collaborations in multiple phases of the research cycle. Last, the results indicate the importance of adding a role (practical support) and two phases (grant application and recruitment) to the Involvement Matrix.ConclusionOur results show the added value of AYA (with a UPCP) involvement within scientific research projects. We recommend researchers to actively think about the level and phase of collaboration prior to each research project, by involving and brainstorming with AYAs at the conception and throughout research projects. Besides, to enhance fruitful participation, we suggest thoroughly discussing the pros and cons of collaboration for each phase together with AYAs via the proposed Involvement Matrix to support transparency. We recommend to report experiences, choices, and results of AYA involvement.

Meningosarcoma in The Infratemporal Fossa: An Unusual Presentation

Meningosarcoma represent a rare disease with an uncertain prognosis. Moreover, localization of tumours in the infratemporal fossa is rare. Lack of associated symptomatology of infratemporal fossa's lesions could result in a diagnosis and treatment delay. Also, surgical management of infratemporal fossa neoplasm remain challenging.

Histopathological and Immunohistochemical Appearance of Pleomorphic Dermal Sarcoma

Pleomorphic dermal sarcoma is a rare and aggressive entity that originates in the skin’s dermis. This poorly defined tumor usually appears on the sun exposed skin of the elderly and involves the male gender more often. We describe the case of a 54 year old male patient who presented to the Surgery Department with a tumor located on the scalp. Surgery was performed and the sample was sent to the Pathology Department of Mures Clinical County Hospital for further analysis. Histological analysis showed a tumor with solid architecture and nesting phenomenon, composed of pleomorphic epithelioid cells with high atypia. Typical and atypical mitoses were observed. Immunohistochemistry showed positivity for the following markers: vimentin, CD68 and ki67- proliferation value of 90%. The cells were negative to CK AE1/AE3, S100, SOX10, HMB45, MelanA, CD31, Desmin and SMA. In conclusion, pleomorphic dermal sarcoma represents a challenging diagnosis. The case highlights the histological and immunohistochemistry appearance of the neoplasm. Due to the local aggressivity, difficulty in providing negative resection margins and uncertain prognosis, it’s important to take this entity into consideration each time we encounter a cutaneous pleomorphic tumor.

Progress in the treatment of refractory macular hole by human amniotic membrane

The surgery of refractory macular hole is often very tough for its uncertain prognosis. There is no unified definition for refractory macular hole, which usually refers to macular hole with a long duration, large diameter, or failure of initial operation. Treatment success can be achieved in some cases by expanding the range of internal limiting membrane peeling or filling with autologous tissue. Human amniotic membrane is a kind of tissue with strong anti-inflammatory and anti-fibrosis effects, and has been widely used in the treatment of ocular surface diseases. In recent years, amniotic membrane has been used in treating refractory macular hole. Some positive results have been achieved, including improvement of postoperative macular structure and increase of visual function. This review focuses on the mechanism, efficacy and prospect of human amniotic membrane in the treatment of refractory macular hole, providing reference for clinical practice and research.

Rare Carotid Bifurcation Tumor

Introduction: Paraganglioma or chemodectoma of the carotid corpuscle or carotid glomus is a relatively rare neoplasm of slow progression, late diagnosis and uncertain prognosis. This tumor originates from the chemoreceptors of the carotid bifurcation and clinically presents as a laterocervical mass. It represents the majority of paragangliomas of the head and neck (60-70%). Observation: We report the observation of a 56-year-old female patient who consulted for a right laterocervical swelling without any other associated clinical signs. A complete radiological workup (Angioscanner/Angiography) will confirm the vascular nature of the lesion, characterize the size of the mass, its nature, its mapping, and its relationship to adjacent organs. Surgical excision had confirmed the diagnosis on histopathological examination. From this observation, we review the clinical, radiological and histopathological features of this tumor, as well as its uncertain evolutionary mode and the therapeutic modalities. Conclusion: Lateral cervical paragangliomas remain rare and poorly known affections which require an early diagnosis and a multidisciplinary management: ENT surgeon, vascular surgeon, oncologist and radiotherapist according to the evolutive forms.

Risk of Dental Caries and Erosive Tooth Wear in 117 Children and Adolescents' Anorexia Nervosa Population-A Case-Control Study.

IntroductionRestrictive type of anorexia nervosa (AN) is still one of the most severe eating disorders worldwide with an uncertain prognosis. Patients affected by AN should be encouraged to undertake psychiatric care and psychotherapy, but whether they should necessarily be included in careful dental care or not may still be questionable. Even though there is a constantly increasing number of AN studies, there are just a few data about the youngest group of AN children and adolescents aged < 18.MethodologyThis case-control study aimed to compare the dental health and gingival inflammation level in female adolescent inpatients affected by severe AN restrictive subtype vs. controls. Based on clinically confirmed 117 AN cases (hospitalized in years 2016–2020 in public Psychiatric Unit, BMI < 15 kg/m2, mean age 14.9 ± 1.8), the dental status has been examined regarding the occurrence of caries lesions using Decay Missing Filling Teeth (DMFT), erosive wear as Basic Erosive Wear Examination (BEWE), gingival condition as Bleeding on Probing (BOP) and plaque deposition as Plaque Control Record (PCR). The results were compared with age-matched 103 female dental patients (BMI 19.8 ± 2.3 kg/m2, age 15.0 ± 1.8, p = 0.746) treated in a public University dental clinic.ResultsAN patients were found to present a higher incidence of oral-related complications according to dental status (DMFT 3.8 ± 4.5 vs. 1.9 ± 2.1, p = 0.005), erosive tooth wear (BEWE 18.9 vs. 2.9%, p < 0.001), less efficient in controlling plaque (PCR 43.8 vs. 13.7%, p < 0.001) and gingival inflammation (BOP 20.0 vs. 3.9%, p < 0.001) compared with female adolescents. In the AN group, a significant correlation between BOP, BEWE, and duration of AN disease (p < 0.05), similarly to the number of decayed teeth D, filled teeth F and PCR were detected (p < 0.05).ConclusionsAlthough the obtained results did not reveal any severe oral status, our findings indicated impaired dental and gingival conditions in young anorexics. Considering AN's potential role in oral health, it is essential to monitor dental treatment needs and oral hygiene levels in their present status to prevent forward complications in the future.

A qualitative study on the challenges health care professionals face when caring for adolescents and young adults with an uncertain and/or poor cancer prognosis

BackgroundAdolescents and young adults (AYAs, aged 18-39 years) with advanced cancer have an increased life expectancy due to improvements and refinements in cancer therapies, resulting in a growing group of AYAs living with an uncertain and/or poor cancer prognosis (UPCP). To date, no studies have examined the difficulties of health care professionals (HCPs) providing care to AYAs with a UPCP. This study aimed to understand the challenges in daily clinical practice experienced by HCPs from different disciplines who provide palliative as well as general care to AYAs with a UPCP.MethodsHCPs from a variety of backgrounds (e.g. clinical nurse specialists, medical oncologists, neurologists psychologists) were invited for a semi-structured interview. The interviews were transcribed verbatim and analysed using reflexive thematic analysis. Two AYA patients were actively involved as research partners to increase the relevance of the study design and to optimise interpretation of results.ResultsForty-nine HCPs were interviewed. Overall, we found that the threat of premature death within this young patient group increased emotional impact on HCPs and evoked a feeling of unfairness, which was an extra motivation for HCPs to provide the most optimal care possible. We generated four key themes: (i) emotional confrontation (e.g. feeling helplessness and experiencing a greater sense of empathy), (ii) questioning own professional attitude and skills, (iii) navigating uncertainty (e.g. discussing prognosis and end of life) and (iv) obstacles in the health care organisation (e.g. lack of knowledge and clarity about responsibilities).ConclusionsHCPs experienced unique emotional and practical challenges when providing care to AYAs with a UPCP. The results from this study highlight the need to develop an education module for HCPs treating AYAs with UPCP to increase their own well-being and optimise the delivery of person- and age-adjusted care.

The diagnosis and management of Gaucher disease in pediatric patients: Where do we go from here?

Gaucher disease (GD) is an autosomal recessive inherited lysosomal storage disease that often presents in early childhood and is associated with damage to multiple organ systems. Many challenges associated with GD diagnosis and management arise from the considerable heterogeneity of disease presentations and natural history. Phenotypic classification has traditionally been based on the absence (in type 1 GD) or presence (in types 2 and 3 GD) of neurological involvement of varying severity. However, patient management and prediction of prognosis may be best served by a dynamic, evolving definition of individual phenotype rather than by a rigid system of classification. Patients may experience considerable delays in diagnosis, which can potentially be reduced by effective screening programs; however, program implementation can involve ethical and practical challenges. Variation in the clinical course of GD and an uncertain prognosis also complicate decisions concerning treatment initiation, with differing stakeholder perspectives around efficacy and acceptable cost/benefit ratio. We review the challenges faced by physicians in the diagnosis and management of GD in pediatric patients. We also consider future directions and goals, including acceleration of accurate diagnosis, improvements in the understanding of disease heterogeneity (natural history, response to treatment, and prognosis), the need for new treatments to address unmet needs for all forms of GD, and refinement of the tools for monitoring disease progression and treatment efficacy, such as specific biomarkers.