Heterogeneous Group Research Articles

The Nematodes Menace: A Review of Parasitic Species, Transmission Dynamics and Preventive Measures

This review offers a detailed examination of nematodes, directing specifically on gastrointestinal (GI) nematodes and their consequences for human health. Nematodes, commonly known as roundworms, demonstrate a highly heterogenous groups of multicellular organisms and are responsible for various diseases in humans, particularly in resource-limited settings. The paper explores different types of GI nematodes, their characteristics, symptoms, and transmission pathways, highlighting the significant impacts these parasites can have, such as malnutrition, anemia, and cognitive impairments. Additionally, the manuscript emphasizes the importance of prevention and control strategies, including proper sanitation, personal hygiene, and public awareness, in minimizing the spread of these infections. It discusses the role of anthelmintic medications as a vital part of treatment, while also advocating for a comprehensive approach that incorporates improved living conditions, access to clean water, and health education for effective long-term management of GI nematode infections. Ultimately, this work highlights the necessity for coordinated public health initiatives and ongoing research to alleviate the burdens of GI nematodes and improve health outcomes in at-risk populations.

Capabilities for Using Telemonitoring in Physiotherapy Treatment: Exploratory Qualitative Study.

Telemonitoring (TM), as part of telehealth, allows physiotherapists to monitor and coach their patients using remotely collected data. The use of TM requires a different approach compared with face-to-face treatment. Although a telehealth capability framework exists for health care professionals, it remains unclear what specific capabilities are required to use TM during physiotherapy treatments. This study aims to identify the capabilities required to use TM in physiotherapy treatment. An exploratory qualitative study was conducted following a constructivist semistructured grounded theory approach. Three heterogeneous focus groups were conducted with 15 lecturers of the School of Physiotherapy (Bachelor of Science Physiotherapy program) from the Amsterdam University of Applied Sciences. Focus group discussions were audiotaped and transcribed verbatim. Capabilities for using TM in physiotherapy treatment were identified during an iterative process of data collection and analysis, based on an existing framework with 4 different domains. Team discussions supported further conceptualization of the findings. Sixteen capabilities for the use of TM in physiotherapy treatment were found addressing 3 different domains. Four capabilities were identified in the "digital health technologies, systems, and policies" domain, 7 capabilities in the "clinical practice and application" domain, and 5 capabilities in the "data analysis and knowledge creation" domain. No capabilities were identified in the "system and technology implementation" domain. The use of TM in physiotherapy treatment requires specific skills from physiotherapists. To best use TM in physiotherapy treatment, it is important to integrate these capabilities into the education of current and future physiotherapists.

Toward Process-Oriented, Dimensional Approaches for Diagnosis and Treatment of Speech Sound Disorders in Children: Position Statement and Future Perspectives.

Children with speech sound disorders (SSD) form a heterogeneous group, with respect to severity, etiology, proximal causes, speech error characteristics, and response to treatment. Infants develop speech and language in interaction with neurological maturation and general perceptual, motoric, and cognitive skills in a social-emotional context. After a brief introduction into psycholinguistic models of speech production and levels of causation, in this review article, we present an in-depth overview of mechanisms and processes, and the dynamics thereof, which are crucial in typical speech development. These basic mechanisms and processes are: (a) neurophysiological motor refinement, that is, the maturational articulatory mechanisms that drive babbling and the more differentiated production of larger speech patterns; (b) sensorimotor integration, which forms the steering function from phonetics to phonology; and (c) motor hierarchy and articulatory phonology describing the gestural organization of syllables, which underlie fluent speech production. These dynamics have consequences for the diagnosis and further analysis of SSD in children. We argue that current diagnostic classification systems do not do justice to the multilevel, multifactorial, and interactive character of the underlying mechanisms and processes. This is illustrated by a recent Dutch study yielding distinct performance profiles among children with SSD, which allows for a dimensional interpretation of underlying processing deficits. Analyses of mainstream treatments with respect to the treatment goals and the speech mechanisms addressed show that treatment programs are quite transparent in their aims and approach and how they contribute to remediating specific deficits or mechanisms. Recent studies into clinical reasoning reveal that the clinical challenge for speech-language pathologists is how to select the most appropriate treatment at the most appropriate time for each individual child with SSD. We argue that a process-oriented approach has merits as compared to categorical diagnostics as a toolbox to aid in the interpretation of the speech profile in terms of underlying deficits and to connect these to a specific intervention approach and treatment target.

Diagnosis of myelodysplastic syndromes: the classic and the novel.

The Myelodysplastic syndromes (MDS) are a heterogenous group of clonal bone marrow (BM) stem cell myeloid neoplasms, characterized by bone marrow (BM) dysplasia, macrocytic anemia or cytopenia with a tendency for leukemic transformation. The suspicion of MDS is raised by a typical but not specific clinical picture and routine labs, but the gold standard for MDS diagnosis is still BM examination with the presence of uni-or multi-lineage dysplasia and blast percentage, together with exclusion of other reasons. Cytogenetics is also a part of the diagnostic process. Flow cytometry and genetics are helpful but are not always mandatory for MDS diagnosis. This review summarizes the current steps in the diagnostic approach for a patient suspected of having MDS. We also describe new concepts that use non-invasive diagnostic technologies, especially digital methods as well as peripheral blood genetics. The hope is that one day these will mature, be introduced into clinical practice, and perhaps in many cases even replace the invasive BM biopsy.

Voltage-Gated Ion Channel Compensatory Effect in DEE: Implications for Future Therapies

Developmental and Epileptic Encephalopathies (DEEs) represent a clinically and genetically heterogeneous group of rare and severe epilepsies. DEEs commonly begin early in infancy with frequent seizures of various types associated with intellectual disability and leading to a neurodevelopmental delay or regression. Disease-causing genomic variants have been identified in numerous genes and are implicated in over 100 types of DEEs. In this context, genes encoding voltage-gated ion channels (VGCs) play a significant role, and part of the large phenotypic variability observed in DEE patients carrying VGC mutations could be explained by the presence of genetic modifier alleles that can compensate for these mutations. This review will focus on the current knowledge of the compensatory effect of DEE-associated voltage-gated ion channels and their therapeutic implications in DEE. We will enter into detailed considerations regarding the sodium channels SCN1A, SCN2A, and SCN8A; the potassium channels KCNA1, KCNQ2, and KCNT1; and the calcium channels CACNA1A and CACNA1G.

Identifying diagnostic biomarkers for Erythemato-Squamous diseases using explainable machine learning

Erythemato-squamous diseases (ESD) are a heterogeneous group encompassing six clinically and histopathologically overlapping subtypes, representing a substantial diagnostic challenge within dermatology. The existing body of research reveals a notable void in detailed examinations that deconvolute the distinct features endemic to each ESD variant. To bridge this knowledge gap, our study applied Explainable Artificial Intelligence (XAI) techniques to systematically elucidate the intricate diagnostic biomarker profiles unique to each ESD category. Methodological rigor was fortified through the employment of stratified cross-validation, bolstering the robustness and generalizability of our diagnostic model. The CatBoost classifier emerged as a preeminent algorithm within our analytical framework, manifesting exemplary classification prowess with an accuracy of 99.07%, precision of 99.12%, recall of 98.89%, and an F1 score of 98.97%. Central to our inquiry was the deployment of Shapley Additive exPlanations (SHAP) values, which afforded granular insight into the contributory weight of individual diagnostic biomarkers for each ESD subtype. Our findings delineated pivotal diagnostic biomarkers including saw-tooth appearance of retes (STAR), melanin incontinence (MI), vacuolisation and damage of basal layer (VDBL), polygonal papules (PP), and band-like infiltrate (BLI) as instrumental in the identification of seborrheic dermatitis, while Psoriasis was characterized by fibrosis of the papillary dermis (FPD), thinning of the suprapapillary epidermis (TSE), elongation of the rete ridges (ERR), clubbing of the rete ridges (CRR), and notable psoriatic spongiosis. This integrative approach, leveraging the analytical acumen of Random Forest coupled with the interpretability afforded by SHAP, signifies a significant advancement in the nuanced diagnostic landscape of ESD.

Heterogeneity of metabolic phenotypes in adolescent boys with obesity due to excess calories

The aim of the study was to determine the heterogeneity of metabolic parameters in teenage boys diagnosed with “obesity due to excess calories” using cluster analysis. Materials and methods. One hundred and twenty-three randomly selected patients aged 14 to 17 years diagnosed with “obesity due to excess calories” were examined. The statistical analysis included descriptive and analytical methods, in particular aimed to identify clusters of biochemical indicators, including serum fasting glucose level, insulin level, total cholesterol level, low density lipoproteins (LDLP), very low-density lipoproteins (VLDL), high density lipoproteins (HDLP) and triglycerides. Results. Two clusters of observations have been identified. The first cluster consisted of 72 patients (58.5%) having fasting glucose levels slightly below average, insulin levels above average, total cholesterol, triglycerides, LDLP and VLDL above average, and HDLP levels below average (“metabolically unhealthy” obesity). The second cluster included 51 teenagers (41.5%) who presented with opposite trends in metabolic characteristics (“metabolically healthy” obesity). The adolescents from the first cluster were slightly older, had higher body mass, body mass index (BMI) and z-BMI scores, and higher HOMA-IR scores. Arterial hypertension and non-alcoholic fatty liver disease were diagnosed statistically significantly more frequently in adolescents from this cluster. Conclusion. Teenage boys with obesity are a heterogeneous group in terms of the values of major metabolic indicators.

Combined immunohistochemical profile CD10/LMO2/MYC is a useful tool to screen MYC rearrangements in aggressive large B-cell lymphomas.

Aggressive large B-cell lymphomas (LBCL) are a heterogeneous group of lymphomas with variable biological characteristics, for which the identification of MYC rearrangements (MYCr) is a defining and prognostic feature. Both the International Consensus Classification and the 5th edition of the World Health Organization Classification of Hematolymphoid Tumors recommend performing cytogenetic studies in all aggressive LBCL to detect MYCr. Since MYCr incidence is low, cost-effective screening tools are necessary. We asked whether the immunohistochemical combined profile of CD10, LMO2, and MYC could be a useful tool to screen for MYCr. For this purpose, we used two strategies: first, a scoring system assigning 0 points each for CD10 - , LMO2 + , and MYC - and 1 point for CD10 + , LMO2 - , and MYC + , adding the results, and second, an algorithm that selected tumors with CD10 + /LMO2 - profile and/or MYC overexpression. All analyses were performed in a training series including 482 cases from a single center and a validation series of 124 patients from two centers. The resulting system classified cases in scores from 0 to 3. Scores 0 and 1 had low MYCr (0/92 and 7/224, 3%, respectively), being higher for scores 2 (40/98, 41%) and 3 (61/68, 90%) (P < 0.001) in the training cohort. The incidence of MYCr in the validation series was as follows: score 0, 0/29 cases; score 1, 3/64 (5%); score 2, 10/23 (43.5%); score 3, 8/8 (P < 0.001). Sensitivity and negative predictive values were respectively 93.5% and 97.8% for the training and 85.7% and 96.8% for the validation cohorts. The algorithm rescued 2 and 1 MYCr cases included in score 1 from both series. In conclusion, we suggest that both approaches combining the interpretation of CD10/LMO2/MYC by immunohistochemistry are useful to screen for MYCr.

Intragenic duplication disrupting the reading frame of MFSD8 in Small Swiss Hounds with neuronal ceroid lipofuscinosis.

Neuronal ceroid lipofuscinosis (NCL) represents a heterogenous group of lysosomal storage diseases resulting in progressive neurodegeneration. We investigated two Small Swiss Hound littermates that showed progressive ataxia and loss of cognitive functions and vision starting around the age of 12 months. Both dogs had to be euthanized a few months after the onset of disease owing to the severity of their clinical signs. Pathological investigation of one affected dog revealed cerebral and cerebellar atrophy with cytoplasmic accumulation of autofluorescent material in degenerating neurons. The clinical signs in combination with the characteristic histopathology led to a tentative diagnosis of NCL. In the subsequent genetic investigation, the genome of one affected dog was sequenced. This revealed a duplication of 18 819 bp within the MFSD8 gene. The duplication breakpoints were located in intron 3 and exon 12 of the gene and were predicted to disrupt the reading frame. Both affected dogs carried the duplication in a homozygous state and there was perfect cosegregation of the genotypes with the phenotype in a large pedigree, consistent with autosomal recessive inheritance. MFSD8 loss-of-function variants are a known cause of NCL7 in human patients, dogs and other mammalian species. The existing knowledge on MFSD8 together with the experimental data strongly suggests that the identified intragenic MFSD8 duplication caused the disease in the Small Swiss Hounds. These results allow their diagnosis to be refined to NCL7 and enable genetic testing in the breed to avoid further unintentional carrier × carrier matings.

Syndromic obesity in children (using the example of clinical cases)

Obesity is a heterogeneous group of hereditary and acquired diseases associated with excessive accumulation of adipose tissue in the body. One example of syndromic obesity in children is Bardet-Biedl syndrome. This is a rare autosomal recessive disease from the group of ciliopathies, characterized by retinal dystrophy, obesity, polydactyly, mental retardation, hypogonadism, and renal dysfunction. The article presents two clinical cases of Bardet-Biedl syndrome. Diagnostic criteria for the disease are given, and the need for molecular genetic research methods in the early stages of the diagnostic search is shown. Promising directions in the treatment of the syndrome are considered.

The Evolving Landscape of Small Fiber Neuropathy.

Small fiber neuropathy (SFN) belongs to a heterogeneous group of disorders in which thinly myelinated Aδ and unmyelinated C-fibers are primarily affected, leading to neuropathic pain and autonomic symptoms. SFN can be associated with systemic conditions such as diabetes, autoimmune diseases, exposure to drugs and toxins, and infection, with the list of associated diseases continuing to expand. Variants in the SCN9A, SCN10A, and SCN11A genes encoding Nav 1.7, Nav 1.8, and Nav 1.9 sodium channel subunits, as well as in the TRPA1 gene, have been found in SFN patients, expanding the spectrum of underlying conditions and enhancing our understanding of pathophysiological mechanisms. There is also growing interest in immune-mediated forms that could help identify potentially treatable subgroups. According to international criteria, diagnosis is established through clinical examination, the assessment of intraepidermal nerve fiber density, and/or quantitative sensory testing. Autonomic functional tests allow for a better characterization of dysautonomia in SFN, which can be subclinical. Other tests can support the diagnosis. Currently, the management of SFN prioritizes treating the underlying condition, if identified, within a multidisciplinary approach that combines symptomatic pain therapy, lifestyle changes, and biopsychological interventions. Emerging insights from the molecular characterization of SFN channelopathies hold promise for improving diagnosis, potentially leading to the discovery of new drugs and refining trial designs in the future. This article reviews the clinical presentation, diagnostic workup, and advancing knowledge of associated conditions and interventional management of SFN.

Assessment of genes involved in lysosomal diseases using the ClinGen Clinical Validity framework.

Lysosomal diseases (LDs) are a heterogeneous group of rare genetic disorders that result in impaired lysosomal function, leading to progressive multiorgan system dysfunction. Accurate diagnosis is paramount to initiating targeted therapies early in the disease process in addition to providing prognostic information and appropriate support for families. In recent years, genomic sequencing technologies have become the first-line approach in the diagnosis of LDs. Understanding the clinical validity of the role of a gene in a disease is critical for the development of genomic technologies, such as which genes to include on next generation sequencing panels, and the interpretation of results from exome and genome sequencing. To this aim, the ClinGen Lysosomal Diseases Gene Curation Expert Panel utilized a semi-quantitative framework incorporating genetic and experimental evidence to assess the clinical validity of the 56 LD-associated genes on the Lysosomal Disease Network's list. Here, we describe the results, and the key themes and challenges encountered.

Clinical and molecular epidemiology of malignant salivary gland tumors

Salivary gland carcinomas are a rare and heterogeneous group of malignant tumors, accounting for 3-6% of all malignant tumors in the head and neck region. The 1-, 3- and 5-year survival rates are 83%, 69% and 63% respectively. Due to new molecular pathological and genetic findings, new entities are constantly being defined as part of the recurring WHO classification of salivary gland carcinomas, so that the incidence rates of the entities are subject to constant change. The only certain risk factor for the development of salivary gland carcinomas is ionizing radiation. In addition, large tumors, cervical lymph node involvement and perineural sheath involvement significantly worsen the prognosis. Today, molecular pathology is coming to the fore, with which potential targets have been identified that can offer prognosis-improving treatment options, particularly in recurrent or distant metastatic stages. Entity-specific tyrosine kinase inhibitors such as axitinib in adenoid cystic carcinoma or larotrectinib in secretory carcinoma and cross-entity therapies such as HER2 inhibition and androgen deprivation can prolong median and progression-free survival with a favorable side effect profile.

Identification of Genetic Variants Associated with Hereditary Thoracic Aortic Diseases (HTADs) Using Next Generation Sequencing (NGS) Technology and Genotype-Phenotype Correlations.

Hereditary thoracic aorta diseases (HTADs) are a heterogeneous group of rare disorders whose major manifestation is represented by aneurysm and/or dissection frequently located at the level of the ascending thoracic aorta. The diseases have an insidious evolution and can be encountered as an isolated manifestation or can also be associated with systemic, extra-aortic manifestations (syndromic HTADs). Along with the development of molecular testing technologies, important progress has been made in deciphering the heterogeneous etiology of HTADs. The aim of this study is to identify the genetic variants associated with a group of patients who presented clinical signs suggestive of a syndromic form of HTAD. Genetic testing based on next-generation sequencing (NGS) technology was performed using a gene panel (Illumina TruSight Cardio Sequencing Panel) or whole exome sequencing (WES). In the majority of cases (8/10), de novo mutations in the FBN1 gene were detected and correlated with the Marfan syndrome phenotype. In another case, a known mutation in the TGFBR2 gene associated with Loeys-Dietz syndrome was detected. Two other pathogenic heterozygous variants (one de novo and the other a known mutation) in the SLC2A10 gene (compound heterozygous genotype) were identified in a patient diagnosed with arterial tortuosity syndrome (ATORS). We presented the genotype-phenotype correlations, especially related to the clinical evolution, highlighting the particularities of each patient in a family context. We also emphasized the importance of genetic testing and patient monitoring to avoid acute aortic events.

Signs of hyperpathia in chronic peripheral neuropathic pain.

Neuropathic pain is pain due to a disease or lesion of the somatosensory system, and can be either spontaneous, evoked or both. Hyperpathia is a type of evoked pain defined by IASP as 'a painful syndrome characterized by an abnormally painful reaction to a stimulus, especially a repetitive stimulus, as well as an increased threshold'. The literature is sparse, and definitions are unclear and inconsistent. The aim of this study was to examine for the presence of mechanical hyperpathia in a heterogeneous group of patients with peripheral neuropathic pain and correlate signs of hyperpathia with other sensory signs. Patients were examined with graded pinprick stimuli to obtain a stimulus-response curve and repetitive pinprick stimuli to assess increase in pain over time and aftersensations. Then, patients were grouped based on the likelihood of having mechanical hyperpathia with either a steep stimulus-response curve or a steep curve on the repetitive pinprick test and results were correlated to mechanical detection and pain thresholds and other outcomes from a full quantitative sensory testing. We included 124 patients with documented peripheral neuropathic pain. Patients with a steep stimulus-response curve did not overlap with patients with a steep curve on the repetitive pinprick test and both groups more often had decreased rather than increased detection and pain thresholds. Our study questions the concept of hyperpathia and suggests that more studies are needed to identify which symptoms and signs group together and might form a syndrome. Hyperpathia is a syndrome of evoked pain. It is poorly defined and little is known about its clinical presentation. Since it is part of pain symptomatology it is important to have a clear definition and understand the pathophysiology behind. This study explored signs of hyperpathia in a heterogeneous group of patients with peripheral neuropathic pain. We used stimulus-response function and repetitive pinprick stimulation to group patients based on the IASP definition. More studies are needed to understand how symptoms and signs coincide.

Gastrointestinal symptoms in children with mastocytosis

Introduction. Mastocytosis is a heterogeneous group of diseases characterized by the abnormal accumulation of clonal mast cells (MCs) in various tissues and organs, including skin, bone marrow, liver, spleen and lymph nodes. The clinical picture of cutaneous and indolent systemic mastocytosis is formed by a wide range of symptoms associated with activation of mast cells. Single European studies have demonstrated wide variability in the frequency of gastrointestinal symptoms (GI-symptoms)) in children with mastocytosis (from 15 to 50%).Aim. To analyze the frequency of mediator-related GI-symptoms in children with different subtypes and clinical forms of mastocytosis.Materials and methods. A prospective observational study included data from 289 children aged 3 to 17 years who were under observation at the Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology. Symptoms were assessed using the Pediatric grade scale of symptoms of mastocytosis. Clinical manifestations of reactions caused by mast cell degranulation were compared with data from clinical and laboratory studies. The concentrations of tryptase and histamine in the blood serum of patients were determined. The incidence of organomegaly and mesenteric lymphadenitis in children with mediating symptoms and without symptoms was determined using ultrasound.Results. 67 (23.2%) patients had GI symptoms. More than half of the patients (51.6%) indicated abdominal pain, 32.3% reported diarrhea. The severity of pain symptoms correlated with the level of serum tryptase (ρ = 0.564, p &lt; 0.01). The incidence of hepatomegaly and mesadenitis in children with GI- symptoms was 19.4% and 25.4%, respectively. In a comparative analysis in groups of children with and without GI symptoms, the differences in the number of identified cases of organomegaly and mesadenitis were statistically significant (p &lt; 0.001). An increase in histamine levels in the blood was detected in half of the patients with complaints of heartburn and nausea.Conclusion. Our results demonstrated that a study of the level of serum tryptase, ultrasound of the abdominal organs and lymph nodes should be carried out in all children with mediator-related GI-symptoms regardless of the clinical form of mastocytosis.

Efficacy and Safety of Ibrutinib as Monotherapy or Combination Therapy in Relapsed/Refractory Diffuse Large B-cell Lymphoma (R/R DLBCL): A Systematic Review and Meta-analysis.

Diffuse large B-cell lymphoma (DLBCL) is a highly heterogeneous disease group. Ibrutinib's monotherapy or combination therapy is effective in relapsed/refractory (R/R) DLBCL. However, the treatment response in R/R DLBCL varies from 15% to 90% with different regimens, and the tolerance remains controversial. The efficacy and safety of ibrutinib monotherapy or combination therapy in patients with R/R DLBCL remain uncertain. The PubMed, CBM, MEDLINE, Cochrane Library, and Embase databases were searched from their inception to July 2021. The total complete remission rate (CRR) and overall response rate in R/R DLBCL patients treated with ibrutinib were 26% and 49%, respectively. The CRR of ibrutinib combination therapy was significantly higher than the ibrutinib monotherapy (45% vs. 19%). Moreover, the CRR of patients was 40% in double expressing lymphoma, 35% in central nervous system lymphoma, and 33% in nongerminal center B-cell-like (non-GCB) DLBCL, which was higher than the 8% in those with the GCB subtype. The pooled median PFS and overall survival were 5.57 and 10.17 months, respectively. GCB-DLBCL had the worst overall survival (5.1 months). Nevertheless, we found that combination regimens had no survival advantage compared with monotherapy (P > 0.05), indicating that combination therapy was only a transitional treatment and bridge for chimeric antigen receptor T cells or other treatments. Moreover, 12% of patients on ibrutinib combination therapy had ≥grade 3 adverse events compared with 9% on ibrutinib monotherapy. Ibrutinib monotherapy or combination therapy was safe and effective in treating R/R DLBCL with tolerable adverse reactions.

Modelling household online shopping and home delivery demand using latent class & ordinal generalized extreme value (GEV) models

The surge in e-commerce during the past decade has led to dramatic changes in consumer shopping behaviour. The study applies two Generalized Extreme Value (GEV) family models to investigate households' e-shopping demands. The study proposes a model structure to jointly model ordinal-based choice behaviour with choice-makers' latent class membership. Introducing latent class structure with the OGEV formulation accounts for the relationship between choice-makers heterogeneous preferential groups and their ordinal choice outcomes. Furthermore, the study also applies the Ordered General Extreme Value (OGEV)-Negative Binomial (NB) model, capturing the interplay between consumers' in-store shopping demands and online shopping behaviour. The RUM principle inherited within the OGEV-NB model allows econometric valuation of in-store shopping activity explicitly considering households' e-shopping demands. Both models are empirically estimated using a dataset collected in the Greater Toronto Area (GTA), Canada. The empirical findings and behavioural implications are also discussed.

A hierarchical consensus reaching model based on trust and uncertain degree for heterogeneous large-scale group decision making and application to product design

Nowadays, due to the fierce market competition, companies in many industries needs to constantly introduce new products. How to select an optimal product design has become a widespread problem. Due to complexity and a large number of decision makers (DMs) participating in decision-making, this paper formulates the product design selection as a type of large-scale group decision making (LSGDM). Because of the differences in occupation, purpose, and personality of DMs in LSGDM, they prefer to provide evaluations by heterogeneous representation formats. A hierarchical consensus reaching model is developed based on trust and uncertain degree for heterogeneous LSGDM. Firstly, the heterogeneous evaluations of DMs are transformed into fuzzy preference relations (FPRs) through possibility degrees. According to trust relationships and original evaluations of DMs, the trust and uncertain degrees of DMs are extracted considering the inherent personalities and psychological traits implied in the heterogeneous evaluations. Thereby, this paper (1) constructs an optimization model to determine the weights of DMs and (2) divides the feedback mechanism into four cases. Case study of product design selection is provided to illustrate the applicability of the proposed model. Several comparisons validate the advantages of the proposed model.

Congenital enteropathies in children: An algorithm for differential diagnosis and therapeutic management. Case report

Infant diarrhea may be caused by infectious, allergic, or surgical diseases. A distinct group comprises congenital enteropathies, a heterogeneous group of genetically determined diseases, including defects in the absorption and transport of nutrients and electrolytes, impaired differentiation of enterocytes and enteroendocrine cells, and defects in the modulation of the intestinal immune response. The final diagnosis of enteropathies is based on the results of molecular genetic tests; however, a detailed assessment of clinical and medical history data and the results of laboratory and instrumental studies enables quick diagnostic search direction and choice of the correct therapeutic tactics. The article considers the algorithm for the differential diagnosis of congenital diarrhea, presents a clinical case of congenital osmotic diarrhea due to disaccharidase insufficiency, and considers the difficulties of the diagnostic search and approaches to diet therapy. The prospective of using a fructose-based formula, Galactomin-19, in the diet therapy of glucose-galactose malabsorption, is presented.