PEG reimplantation after Buried Bumper Syndrome: a case report

Percutaneous endoscopic gastrostomy (PEG) is the method of choice to provide long-term enteral nutrition for patients with impossibility to be fed orally. Although it is considered a routine and safe procedure, potential complications exist, which are generally classified into three major categories: endoscopic technical difficulties, PEG procedure-related complications and late complications associated with PEG tube use, such as buried bumper syndrome (BBS). BBS is a potentially life-threatening complication, occurring in 0.3% to 2.5% of cases. Additional complications related to BBS may present, such as wound infection, peritonitis, and necrotizing fasciitis. Once resolved the acute complication, an adequate feeding method should be prompted for the patient, among whom PEG remains of choice. After tissue inflammation, fibrosis may prevent a standard endoscopic procedure for the new implantation, therefore endoscopists should modulate procedures to obtain successful and safe results. A combined surgical-and endoscopic strategy could resolve implantation difficulties ensuring a safe and simple procedure. We present here a case of BBS complicated with abdominal wall cellulitis in a paraplegic 35-year-old-man who was admitted to our hospital. (www.actabiomedica.it)

Case report of a familial triple: a syndrome and review of the literature

RATIONALE: Triple-A syndrome, or Allgrove syndrome (AS), is a rare autosomal recessive disorder characterized by the alacrimia, achalasia, and adrenal insufficiency triad. Alacrimia usually starts at early infancy, while achalasia and adrenal insufficiency appear later during childhood or adulthood. Some patients may also present with the so-called Double-A syndrome (i.e., alacrimia and achalasia, or alacrimia and adrenal insufficiency); adrenal insufficiency usually represents a life-threatening event due to severe hypoglycemia. Many patients may also present other associated manifestations, such as neurological disorders. We describe, here, 2 sisters of non-consanguineous parents. PATIENT CONCERNS: An 8-year-old girl was admitted to the Pediatric Care Unit of Parma after an episode characterized by seizure with loss of consciousness and generalized hypertonia lasting a few minutes. Her sister, a 6-year-old girl, presented with recurrent episodes of vomiting and failure to thrive. DIAGNOSES: Both children were investigated by laboratory tests, esophagogastroduodenoscopy, and imaging. The first patient had the complete triad of AS (alacrimia, achalasia, adrenal insufficiency), while the second one presented only alacrimia and achalasia. Both resulted from a mutation in the achalasia, addisonianism, alacrimia syndrome gene. INTERVENTIONS: Both patients were treated with oral hydrocortisone for Addison disease, and with artificial tears in the first case. After many pneumatic endoscopic dilations and therapy with nifedipine, both patients underwent surgical Heller myotomy for achalasia. OUTCOMES: A rapid and favorable recovery to normal diet and with improvement of growth parameters was obtained. These cases are also compared with the literature data, reported in a brief review. LESSONS: AS is a rare multisystemic disorder. The longer diagnosis is delayed, the greater extent to which this syndrome may be life-threatening, mainly because of hypoglycemia due to adrenal insufficiency. In AS, the red-flag symptom of alacrimia should instigate investigation for achalasia, Addison disease, and achalasia, addisonianism, alacrimia syndrome gene mutation

Relationship between Secondary Metabolites, Antiradical Activities, and Colour Characteristics of Cochlospermum Tinctorium A. Rich. (Bixaceae) Root

Cochlospermum tinctorium A. Rich. (Bixaceae) is a widespread herbaceous plant in the African sahelian and soudanian zones. Its root is used as food and medicinal plant. Studies of secondary metabolites, functional activities and trichromatic parameters were realized according to standard methods of laboratory. At a threshold of α = 0.05, significant correlations (p < 0.05) were found between secondary metabolites contents and functional activities such as radical scavenging activity of antioxidant component or hydroxyl radical scavenging activity. The value of DPPHH was 95.56 ± 0.94% for CT inner Matrix with variation of 0.12% for CT Soaked inner Matrix and 1.63% for CT Peeler. But HSRA was 37.14 ± 5.71 for CT inner Matrix with variation of -38.46% for CT Soaked inner Matrix and -71.79% for CT Peeler. Cochlospermum tinctorium root has exhibited efficiency to heal many diseases thanks to multiple bioactive compounds. In addition, the colour of powders depended upon chemical compounds. Finally, Cochlospermum tinctorium root was found to neutralize organic radicals and reactive oxygen species

Epithelial to mesenchymal transition: A challenging playground for translational research. current models and focus on TWIST1 relevance and gastrointestinal cancers

Resembling the development of cancer by multistep carcinogenesis, the evolution towards metastasis involves several passages, from local invasion and intravasation, encompassing surviving anoikis into the circulation, landing at distant sites and therein establishing colonization, possibly followed by the outgrowth of macroscopic lesions. Within this cascade, epithelial to mesenchymal transition (EMT) works as a pleiotropic program enabling cancer cells to overcome local, systemic, and distant barriers against diffusion by replacing traits and functions of the epithelial signature with mesenchymal‐like ones. Along the transition, a full‐blown mesenchymal phenotype may not be accomplished. Rather, the plasticity of the program and its dependency on heterotopic signals implies a pendulum with oscillations towards its reversal, that is mesenchymal to epithelial transition. Cells in intermixed E⇔M states can also display stemness, enabling their replication together with the epithelial reversion next to successful distant colonization. If we aim to include the EMT among the hallmarks of cancer that could modify clinical practice, the gap between the results pursued in basic research by animal models and those achieved in translational research by surrogate biomarkers needs to be filled. We review the knowledge on EMT, derived from models and mechanistic studies as well as from translational studies, with an emphasis on gastrointestinal cancers (GI)

Anisotropia ed orientamento cristallografico preferenziale di leghe di titanio alfa per impieghi a caldo

Attualmente, le leghe di titanio per impieghi ad alta temperaturautilizzano come leganti caratteristici elementiquali alluminio, silicio, niobio e ferro. L’aggiunta di questielementi ha come obiettivo principale quello di incrementarela resistenza all’ossidazione e le caratteristiche meccanichedelle leghe e di renderle molto più performanti deigradi di titanio commercialmente puro, come il Grado 2,che costituiscono una vasta fetta del mercato mondiale diquesta famiglia di materiali.Comprendere il meccanismo attraverso il quale avvienela deformazione plastica a freddo di questi materiali è digrande importanza se si vuole raggiungere l’obiettivo di ottenereun processo produttivo stabile e caratteristiche sulprodotto finito note e controllabili.Come molti materiali metallici che presentano strutturaIn virtù delle elevate caratteristiche meccaniche, della bassa densità e dell’elevata resistenza all’ossidazione,il titanio e le sue leghe di tipo alpha trovano un largo impiego in svariati settori industriali. Tuttavia, spessol’utilizzo di questa famiglia di materiali risulta limitato a causa della ridotta formabilità a freddo di questeleghe: Nel presente lavoro, i parametri di anisotropia di due differenti leghe di titanio alpha per impieghi adalta temperatura sono stati determinati e successivamente una analisi dell’orientamento cristallograficopreferenziale (tessitura) è stato eseguito al fine di poter determinare la sua influenza sulle caratteristichemeccaniche macroscopiche del materiale

A 3-Year Multicentric Study on Switching from Ustekinumab to Guselkumab in Partial Responders with Psoriasis—IL PSO (Italian Landscape Psoriasis)

Introduction: Guselkumab, a human monoclonal antibody targeting the p19 subunit of interleukin-23 (IL-23), has shown efficacy in psoriasis and psoriatic arthritis. However, long-term real-world data on its effectiveness in patients with inadequate response to ustekinumab are limited. This study investigates guselkumab's long-term effectiveness and safety in patients with psoriasis with partial response to ustekinumab. Methods: We performed a retrospective multicentric study analyzing data of patients with psoriasis from seven Italian hospitals between January 2021 and May 2024. The study included 169 patients who switched from ustekinumab to guselkumab. Primary endpoints were Psoriasis Area and Severity Index (PASI) 75, PASI 90, PASI 100, and absolute PASI ≤ 2. Site-specific Physician Global Assessment (PGA) scores were also collected for difficult-to-treat areas. Results: The study included 169 patients. After 3 years of treatment, PASI 75, PASI 90 and PASI 100 were achieved by 88.4%, 55.8%, and 32.6% of patients, respectively. Site-specific PGA showed significant improvements, especially in the scalp and genital areas. After 3 years of treatment, no significant impact of higher body mass index (BMI) or cardiometabolic comorbidities on guselkumab effectiveness was detected. No severe adverse events were reported during the study period. Conclusions: In our study, guselkumab provided significant long-term effectiveness and safety in patients partially responsive to ustekinumab, improving both PASI score and site-specific PGA and confirming its potential use for patients with psoriasis switching from ustekinumab

Real-life effectiveness and safety of guselkumab in patients with psoriasis who have an inadequate response to ustekinumab: A 104-week multicenter retrospective study – IL PSO (ITALIAN LANDSCAPE PSORIASIS)

BackgroundGuselkumab is a humanized monoclonal antibody that binds selectively to the p19 subunit of interleukin-23, which has shown efficacy in patients with previous incomplete response to ustekinumab in the NAVIGATE clinical trial. ObjectivesWe conducted a 104-week multicenter retrospective study to assess the effectiveness and safety of guselkumab in patients affected by plaque psoriasis with an inadequate response to ustekinumab in a real-life setting. MethodsOur retrospective study included 233 adults affected by moderate-to-severe plaque psoriasis, enrolled in 14 different Italian centres, and treated with guselkumab after failing therapy with ustekinumab. Patient characteristics and PASI (Psoriasis Area and Severity Index) score at each visit (baseline, weeks 16, 52 and 104) were recorded. The percentages of patients achieving 75%, 90% and 100% (PASI 75, PASI 90 and PASI 100) improvement in PASI, compared with baseline, were registered. ResultsAt week 52, PASI 75 was reached by 89.88% of patients, PASI 90 by 71.43%, PASI 100 by 58.83% and absolute PASI <= 2 by 90.48%. At week 104, similar effectiveness results were observed. Compared to the NAVIGATE trial, we observed higher rates of PASI 75/90/100. Patients with the involvement of difficult-to-treat areas were significantly less likely to achieve PASI90 and PASI100 at week 16. Obese patients had significantly lower rates of PASI75 and PASI <= 2 at week 52. At week 104, comparable responses were observed among all patients' subgroups, regardless of BMI status, involvement of difficult-to-treat areas, presence of cardiometabolic comorbidities and concomitant psoriatic arthritis. No significant safety findings were reported throughout the study. ConclusionOur data suggest that the efficacy of guselkumab in patients with inadequate response to ustekinumab for plaque psoriasis in 'real-life' clinical practice is comparable with NAVIGATE study with higher percentages of patients achieving PASI90 and PASI100 at weeks 16, 52 and 104

"I have got something positive out of this situation": psychological benefits of caregiving in relatives of young people with muscular dystrophy.

This paper focuses on the psychological benefits of caregiving in key relatives of patients with muscular dystrophies (MD), a group of rare diseases characterized by progressive weakness and restriction of the patient's functional abilities. We describe whether relatives perceived caregiving to be a positive experience and test whether relatives' perceptions vary in relation to their view of the patient as a valued person, the degree of involvement in care, and the level of support provided by social network and professionals. The study sample included 502 key relatives of patients aged 4-25 years, suffering from Duchenne, Becker, or limb-girdle MD, in treatment for at least 6 months to one of the eight participating centers, living with at least one relative aged 18-80 years. Of key relatives, 88 % stated that they had gotten something positive out of the situation, 96 % considered their patients to be sensitive, and 94 % viewed their patients as talented. Positive aspects of caregiving were more recognized by key relatives who were more convinced that the patient was sensitive and who perceived that they received higher level of professional help and psychological social support. These results suggest that most key relatives consider that their caregiving experience has had a positive impact on their lives, despite the practical difficulties of caring for patients with MD. Professionals should help relatives to identify the benefits of caregiving without denying its difficulties. Clinicians themselves should develop positive attitudes towards family involvement in the care of patients with long-term diseases

Maitotoxin-4, a Novel MTX Analog Produced by Gambierdiscus excentricus

Maitotoxins (MTXs) are among the most potent toxins known. These toxins are produced by epi-benthic dinoflagellates of the genera Gambierdiscus and Fukuyoa and may play a role in causing the symptoms associated with Ciguatera Fish Poisoning. A recent survey revealed that, of the species tested, the newly described species from the Canary Islands, G. excentricus, is one of the most maitotoxic. The goal of the present study was to characterize MTX-related compounds produced by this species. Initially, lysates of cells from two Canary Island G. excentricus strains VGO791 and VGO792 were partially purified by (i) liquid-liquid partitioning between dichloromethane and aqueous methanol followed by (ii) size-exclusion chromatography. Fractions from chromatographic separation were screened for MTX toxicity using both the neuroblastoma neuro-2a (N2a) cytotoxicity and Ca2+ flux functional assays. Fractions containing MTX activity were analyzed using liquid chromatography coupled to high-resolution mass spectrometry (LC-HRMS) to pinpoint potential MTX analogs. Subsequent non-targeted HRMS analysis permitted the identification of a novel MTX analog, maitotoxin-4 (MTX4, accurate mono-isotopic mass of 3292.4860 Da, as free acid form) in the most toxic fractions. HRMS/MS spectra of MTX4 as well as of MTX are presented. In addition, crude methanolic extracts of five other strains of G. excentricus and 37 other strains representing one Fukuyoa species and ten species, one ribotype and one undetermined strain/species of Gambierdiscus were screened for the presence of MTXs using low resolution tandem mass spectrometry (LRMS/MS). This targeted analysis indicated the original maitotoxin (MTX) was only present in one strain (G. australes S080911_1). Putative maitotoxin-2 (p-MTX2) and maitotoxin-3 (p-MTX3) were identified in several other species, but confirmation was not possible because of the lack of reference material. Maitotoxin-4 was detected in all seven strains of G. excentricus examined, independently of their origin (Brazil, Canary Islands and Caribbean), and not detected in any other species. MTX4 may therefore serve as a biomarker for the highly toxic G. excentricus in the Atlantic area

Drug survival of IL-12/23, IL-17 and IL-23 inhibitors for moderate-to-severe plaque psoriasis: a retrospective multicenter real-world experience on 5932 treatment courses – IL PSO (Italian landscape psoriasis)

The development of several effective biological drugs for moderate-to-severe plaque psoriasis has dramatically changed the lives of patients. Despite the wide use of interleukin (IL) inhibitors, limited data are available to date regarding long-term treatment persistence