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At least 19 recordsLinked to original sources

Management of maple syrup urine disease in Canada. Committee for improvement of Hereditary Disease Management.

Nine patients with classic maple syrup urine disease (MSUD) and four with variant forms are under care at five treatment centres in the network affiliated with the National Food Distribution Centre for the Treatment of Hereditary Metabolic Diseases (the "Food Bank"). Diagnosis was made by clinicians and not from mass screening programs. MSUD requires complex emergency treatment to prevent severe neurologic damage, but effective management is compatible with normal growth and development. Long-term treatment requires continuous monitoring of the response to diets restricted in branched-chain amino acids; semisynthetic diet products free of branched-chain amino acids, provided by the Food Bank, are essential. Centralized treatment programs reduce the cost of treatment and maximize the potential benefits. The leucine requirement for adequate somatic growth during infancy in MSUD was found to be 200 to 600 mg/d; this range is lower than that estimated for infants with an intact leucine catabolic outflow pathway. The requirements for isoleucine and valine in infancy were also found to be lower than published values for normal infants.

Canada

Genetic Diversity and Pathogenicity of Thielaviopsis paradoxa Isolates and Implications for Coconut Palm Disease Management.

Thielaviopsis paradoxa is an important soilborne pathogen causing bleeding disease and stem rot of coconut and other palm species, posing a serious threat to coconut production in Hainan Province, China. This study investigated the biological characteristics, pathogenic variability, and genetic diversity of T. paradoxa isolates collected from coconut palms in this region, where population-level data remain limited. Isolates exhibited variability in mycelial growth and sporulation under different temperature, pH, and nutrient conditions, indicating physiological differentiation among strains. Pathogenicity assays across multiple coconut varieties revealed four pathogenicity types, with some isolates consistently showing greater aggressiveness. Genetic analysis based on ISSR markers revealed a high level of genetic diversity (82.96% polymorphism). Genetic clustering showed partial associations with geographic origin, host source, and pathogenicity patterns; however, these relationships are correlative and do not imply causality. Overall, this study provides the first region-specific synthesis of physiological traits, pathogenic variability, and ISSR-based genetic diversity of T. paradoxa in Hainan, contributing baseline information for future population genomic studies and supporting the development of improved disease management strategies for coconut palms.[Formula: see text] Copyright © 2026 The Author(s). This is an open access article distributed under the CC BY 4.0 International license.

China

Tobamoviruses: Advances in Molecular Biology, Host Interactions and Integrated Disease Management.

Tobamoviruses (viruses in the genus Tobamovirus, family Virgaviridae) lead to major yield losses in economically important crops around the world. In this review, we go beyond the canonical gene expression framework by integrating recent discoveries of reverse open reading frames (rORFs) on the negative-strand RNA. These rORFs have only been experimentally validated in cucumber green mottle mosaic virus (CGMMV), with predicted sequence-conserved homologs across a subset of the genus, including TMV, ToBRFV, and PMMoV. However, they are not universally present in all tobamoviruses. We systematically dissect the infection cycle-from disassembly and replication to cell-to-cell and systemic movement-with an emphasis on the host factors hijacked at each stage. We synthesize current understanding of plant antiviral immunity, focusing on RNA silencing and NLR receptor-mediated resistance as two pillars of defense, along with the transcription factors and microRNAs that orchestrate these responses. We critically evaluate the experimental evidence for both plant defenses and viral counter-strategies, noting that many mechanistic models derive from limited model systems. We further characterize host genetic resistance and susceptibility factors applicable to crop breeding. These resources include dominant NLR and non-NLR resistance, as well as recessive resistance derived from modified host susceptibility genes. We address how viral mutations, recombination and fitness trade-offs undermine resistance durability. We then evaluate their practical deployment through conventional breeding, the exploitation of quantitative resistance, and genome editing, and outline associated agronomic drawbacks and regulatory constraints. Using ToBRFV as a case study, we analyze its epidemiological traits and assess the current arsenal of surveillance tools, from field diagnostics to remote sensing. Finally, we survey management strategies across a spectrum of maturity. Some approaches, including sanitation protocols and conventionally bred resistant cultivars, have proven effective under field conditions. The first dsRNA-based biopesticide has recently been registered in China, while other biological control agents and low-risk chemical approaches remain largely at the experimental stage. We also discuss the bottlenecks that impede lab-to-field transition and highlight promising solutions such as precision breeding and evolution-oriented cultivar deployment. By bridging molecular virology, epidemiology, and integrated disease management, this review provides a critical, bench-to-field framework for the sustainable control of tobamoviruses.

TMV

Asymptomatic coronary artery disease managed by myocardial revascularization: results at 5 years.

A consecutive series of 17 patients surgically treated for asymptomatic coronary artery disease was surveyed for survival, clinical status, and graft patency after a mean follow-up of 75 months. Documented myocardial infarction (MI), 14 transmural and 3 subendocardial, was the major indication for catheterization. All patients were asymptomatic for at least 4 months prior to surgery. Six patients had greater than 70% obstruction in one vessel, seven in two vessels, and four in three vessels. The indication for revascularization was critical stenoses in vessels supplying large areas of viable myocardium. All patients received left internal mammary artery (LIMA) grafts to the left anterior descending coronary artery; seven patients also received saphenous vein grafts (SVG) to other vessels. There was no operative or late mortality. In 12 patients, postoperative angiography (mean interval, 12 1/2 months) showed 11/12 LIMA and all SVG patent. One incompletely revascularized patient with an occluded LIMA is symptomatic. All others are fully active without symptoms or interval MI after a minimum follow-up of 5 1/2 years. The safety and long-term results of myocardial revascularization warrant its consideration for selected asymptomatic postinfarction patients.

Adult

End stage polycystic kidney disease: management by renal transplantation and selective use of preliminary nephrectomy.

The results have been reviewed of 35 renal transplants performed on 31 patients with end stage polycystic renal disease. Patient survival is 81 per cent and 71 per cent of the patients have functioning grafts at an average followup of 3.1 years. The need for pre-transplant nephrectomy was evaluated early in the series and since then the operation has been practiced selectively. Twenty-two patients have received transplants with both polycystic kidneys in situ, while 2 patients have undergone transplantation after unilateral nephrectomy. In the absence of a history of renal infection or significant hematuria it has proved safe and desirable to leave the polycystic kidneys in situ. During the post-transplant period in such cases there has been no difficulty attributed to the in situ polycystic kidneys after more than 450 patient months of immunosuppressive therapy. The size of the polycystic kidneys has not been an indication of nephrectomy in our series and no significant technical difficulties have been encountered with large polycystic kidneys remaining in situ. Hypertension associated with end stage polycystic kidney disease has been controlled easily and has not proved an indication for pre-transplant nephrectomy.

Adult

Thermal injury in von willebrand disease. Management of burn wound bleeding.

A 51-year-old woman sustained a 40% third-degree burn. After five grafting procedures that were unsuccessful due to uncontrolled bleeding, the diagnosis of von Willebrand disease was established. Skin grafting was subsequently accomplished without difficulty using cryoprecipitate as a source of antihemophilic factor (factor VIII). Serial clinical and laboratory investigations demonstrated that using the level of factor VIII procoagulant activity alone as an in vitro index of hemostasis was insufficient as a guide to the control of pathologic burn wound bleeding in this patient.

Blood Transfusion

Clinical impact of 16S rRNA RC-PCR NGS on infectious disease management.

16S rRNA metagenomics provides a culture-independent method for diagnosing infections with fastidious or uncultivable organisms, guiding targeted therapy, and detecting polymicrobial communities. This study utilizes reverse complement (RC)-PCR next-generation sequencing (NGS) to accurately identify bacterial pathogens from clinical specimens and assess its impact on clinical decision-making, setting it apart from conventional 16S sequencing approaches. A retrospective analysis of an ISO 15189 accredited 16S RC-PCR NGS diagnostic workflow targeting the V1-6 and V9 regions of the 16S rRNA gene was conducted over a 2-year period, including 390 clinical specimens from 316 patients. 16S RC-PCR NGS results were discussed in a multidisciplinary consultation and subsequently reported to the clinic. In total, 1,283 RC-PCR results were analyzed, of which 517 were from clinical specimens, 284 were negative controls, 66 were positive controls, and 416 were from wet lab and bioinformatic pipeline validation. 16S RC-PCR NGS assay detected bacterial taxa in 179/390 (45.9%) of clinical specimens, while 201/390 (51.5%) were negative, and 10/390 (2.6%) yielded uninterpretable results. The specimen types pus, pleural fluid, and heart valves exhibited the highest positivity rate (68% to 70%). Overall, 16S RC-PCR NGS influenced diagnostic decision making in 145/282 (51.4%) clinical cases and guided therapeutic management in 77/282 (27.3%) cases. Results providing definite evidence for either the presence or absence of bacterial infection were considered clinically valuable. Integration of 16S RC-PCR NGS pathogen detection with multidisciplinary consultation markedly improved clinical management, directly impacting diagnosis and treatment of complex clinical cases in a tertiary care setting. The effect was most pronounced in brain abscess patients, where RC-PCR results guided treatment decisions in 9/13 (69.2%) of cases.IMPORTANCETimely and accurate diagnosis is essential for managing serious infections, yet clinicians often face situations where routine laboratory tests do not provide clear answers. This study demonstrates that next-generation sequencing (NGS) of the bacterial 16S rRNA gene can decisively resolve these uncertainties. By revealing whether bacteria are present in clinical specimens, this approach influenced clinical reasoning and supported treatment decisions across a variety of challenging cases. 16S reverse-complement PCR was especially powerful for brain abscesses and infections where the causative microorganism was unclear, providing clarity that directly improved patient care. These findings show that integrating advanced sequencing with expert clinical interpretation can enhance the management of complex infections and support more confident, evidence-based therapy.

Humans

[Comparative psychological studies on disease management and attitude to life in patients with hemophilia with special reference to their motor disabilities].

The motor skills of 39 haemofiliacs are examined under the question to what extent they are permanently disabled and how this disability is subjectively experienced. Besides the question is followed how far the restriction of the motorial functions in relation to other psychological stress within this illness reduces and neurotizises their attitude to life. The results are discussed unter depth-psychological points of view and with regard to the Psychology of Neurosis.

Adult

Leveraging traveller genomics for LMIC diarrhoeal disease management.

Diarrhoeal pathogens impose a substantial global health burden, disproportionately affecting low- and middle-income countries (LMICs). However, in these settings, health-seeking behaviours, suboptimal microbiological capacity, and challenges in establishing genomics capacity constrain effective surveillance, including surveillance of antimicrobial resistance (AMR). In contrast, high-income countries routinely generate and share large volumes of diarrhoeal pathogen genomes through established systems, with a significant proportion originating from travellers returning from LMICs. These data reveal strong geographical structuring of lineages and clinically relevant AMR patterns, demonstrating untapped potential to support improvements in geographically granulated surveillance to support antimicrobial treatment recommendations. In this opinion article, we outline the potential to integrate traveller-derived microbial genomic data into LMIC public health decision-making and highlight the scientific, ethical, practical, and governance considerations for implementation.

antimicrobial resistance

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease). Management of epistaxis in nine patients using systemic hormone therapy.

The combination of a progestogen and an estrogen (Enovid, Enovid E) has been used successfully in the management of nine patients with hereditary hemorrhagic telangiectasia (HHT) and severe epistaxis: Such therapy was instituted when epistaxis was severe and only after other therapeutic measures had failed. Seven of our patients required the 5-mg tablet (Enovid) to achieve a satisfactory clinical result; two patients received the 2.5-mg preparation (Enovid E). The eight women received cyclic therapy; the man received therapy without interruption. Because of the possibility of serious adverse effects from the drugs, it is required that each patient receive a complete medical evaluation prior to therapy and at frequent intervals while receiving therapy. Enovid therapy for HHT is not recommended for the patient with mild bleeding that can be controlled by conventional therapy.

Adult

Infectious disease management with oral antibiotics.

This paper has reviewed the bacterial etiologies and therapeis for commonly seen infections in the out-patient clinic or physician's office. The use of oral antibiotics for the treatment of pharyngitis, otitis media, sinusitis, bronchitis, certain pneumonias, cellulitis, urinary tract infections and as follow-up therapy to systemic administration is discussed. Emphasis on the decreasing bacterial spectra of the tetracyclines is noted as well as a discussion of therapy of infections due to beta-lactamase-producing Staphylococcus aureus and Haemophilus influenzae.

Administration, Oral