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2.
J Clin Exp Hepatol ; 14(5): 101397, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38595988

RESUMO

Introduction: Diagnosis of wall-thickening type gallbladder cancer (GBC) is challenging. Computed tomography (CT) and magnetic resonance imaging (MRI) are commonly utilized to evaluate gallbladder wall thickening. However, there is a lack of data comparing the performance of CT and MRI for the detection of wall-thickening type GBC. Aim: We aim to compare the diagnostic accuracy of CT and MRI in diagnosis of wall-thickening type GBC. Materials and methods: This prospective study comprised consecutive patients suspected of wall-thickening type GBC who underwent preoperative contrast-enhanced CT and MRI. The final diagnosis was based on the histopathology of the resected gallbladder lesion. Two radiologists independently reviewed the characteristics of gallbladder wall thickening at CT and MRI. The association of CT and MRI findings with histological diagnosis and the interobserver agreement of CT and MRI findings were assessed. Results: Thirty-three patients (malignancy, 13 and benign, 20) were included. None of the CT findings were significantly associated with GBC. However, at MRI, heterogeneous enhancement, indistinct interface with the liver, and diffusion restriction were significantly associated with malignancy (P = 0.006, <0.001, and 0.005, respectively), and intramural cysts were significantly associated with benign lesions (P = 0.012). For all MRI findings, the interobserver agreement was substantial to perfect (kappa = 0.697-1.000). At CT, the interobserver agreement was substantial to perfect (k = 0.631-1.000). Conclusion: These findings suggest that MRI may be preferred over CT in patients with suspected wall thickening type GBC. However, larger multicenter studies must confirm our findings.

3.
Nucl Med Mol Imaging ; 58(3): 104-112, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38633288

RESUMO

Purpose: Incidental gallbladder carcinoma (IGBC) is diagnosed in post-cholecystectomy specimens for benign indications, where the role of 2-fluro-2-deoxyglucose positron emission tomography/computed tomography(FDG-PET/CT) is not clearly defined. The present study aimed to assess the benefits of staging and prognosticating with FDG-PET/CT in IGBC. Materials and Methods: A retrospective observational study from a tertiary-care center from January 2010 to July 2020 was performed. The demographic, clinical, histopathological, and treatment-related histories were collected. FDG-PET/CT-image findings were compared with survival outcomes through telephonic follow-up. The chi-square test was used for comparing frequencies. The univariate and multivariate survival estimates were analyzed using the Kaplan-Meier analysis and the Cox-proportional hazard model, respectively. Log-rank test was used to compare the Kaplan-Meier curves. Results: The study included 280 postcholecystectomy participants (mean age: 52 ± 11 years; women: 227) of whom 52.1% had open surgery(146/280). Residual disease in the gallbladder fossa (54.8% vs. 36.6%, p = 0.002) and liver infiltration (32.9% vs. 22.4%, p = 0.05) were seen more frequently in open surgery compared to laparoscopic surgery, while anterior abdominal wall deposits were more common in laparoscopy(35.1% vs. 24%,p = 0.041). FDG-PET/CT changed the management in 10% (n = 28) of patients compared to contrast-enhanced CT. The median survival was 14 months (95%CI-10.3-17.7). A higher stage of the disease on the FDG-PET/CT (loco-regional disease-HR 4.86, p = 0.006; metastatic disease-HR 7.53, p < 0.001) and the presence of liver infiltration (HR-1.92, p = 0.003) were independent predictors of poor survival outcomes. Conclusion: FDG-PET/CT detects residual and metastatic disease in patients with IGBC, enabling the institution of appropriate management and acting as a tool for prognostication of survival.

4.
Hematology ; 29(1): 2329027, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38526239

RESUMO

This retrospective report presents the outcomes and adverse events (AEs) observed in 73 patients aged 60 years or older diagnosed with Philadelphia Chromosome-negative Acute Lymphoblastic Leukemia (Ph-negative ALL) treated with a pediatric-inspired protocol incorporating either Pegylated (PEG-ASP) or Native Asparaginase (EC-ASP). Notably, 61% of patients experienced AEs of Grade III-IV severity. The most prevalent AEs included thrombosis (35.6%), febrile neutropenia (38.4%), and transaminitis (34.2%). AEs did not translate into significant differences concerning overall survival, leukemia-free survival, or early mortality. Furthermore, we observed a reduction in early mortality rates (11% vs. 20%) and an increase in median overall survival (54 vs. 48 months) compared to our previous data. These findings suggest that the utilization of a pediatric-inspired chemotherapy protocol, with ASP, is an effective and well-tolerated therapeutic option for older patients with Ph-negative ALL. However, it emphasizes the importance of diligent monitoring and close follow-up throughout treatment.


Assuntos
Asparaginase , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Idoso , Asparaginase/efeitos adversos , Estudos Retrospectivos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Polietilenoglicóis/efeitos adversos
5.
Cureus ; 16(2): e54150, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38496104

RESUMO

BACKGROUND: Chronic suppurative otitis media (CSOM) is a prevalent and persistent middle ear condition that not only affects auditory health but also potentially influences various aspects of an individual's life. This study explores the correlation between CSOM, depression, anxiety, and stress, using the 21-item Depression, Anxiety, and Stress Scale (DASS 21), also assessing quality of life (QoL) using the Chronic Ear Survey (CES) questionnaire. The primary objective of this study was to gather prospective audiological data along with information on both disease-specific quality of life and psychological well-being, utilizing validated measurement instruments. METHODS: This cross-sectional study was conducted at a tertiary care center in Central India, involving 182 patients with CSOM. The study included individuals aged 18 years and above diagnosed with CSOM in at least one ear. Patients with a history of psychological disorders, head injury, and those with comorbidities such as diabetes, hypertension, and chronic heart diseases were excluded. Pure tone audiometry was employed for hearing evaluation, while the assessment of psychological well-being utilized the DASS 21 questionnaire. Furthermore, the quality of life was evaluated using the CES tool. RESULTS: From the initial cohort of 182 patients diagnosed with CSOM, 32 were excluded based on predefined criteria, resulting in a final sample of 150 patients. The cohort, with a mean age of 34.3 years, exhibited a predominantly female population (63.3%). Psychological assessments using DASS 21 revealed depression in 22 (14.7%) patients and anxiety in 23 (15.3%) patients. Among those with depression, majority of the participants had mild depression. Similarly, among those with anxiety, the majority were found to be experiencing mild anxiety. Bilateral CSOM demonstrated a higher prevalence of anxiety and depression, establishing a significant association. QoL parameters, assessed by the Chronic Ear Survey, indicated a more adverse impact in bilateral cases across all categories except symptoms. Correlation analysis between psychological well-being, quality of life, and hearing loss severity yielded statistically significant results. CONCLUSION: CSOM with the symptom of hearing loss can lead to reduced QoL and psychological well-being in the affected individuals. This study highlights the psychological impact of CSOM, particularly in bilateral cases and severe hearing loss. Integrating psychological support into treatment plans is crucial for comprehensive patient care. Regular assessments are essential for guiding timely interventions, ensuring a holistic approach to enhance both quality of life and psychological well-being in individuals affected by CSOM.

6.
Cureus ; 16(2): e54142, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38496131

RESUMO

Tracheostomy is a life-saving procedure in which an opening is created in the anterior wall of the trachea. Different skin incision types are administered in tracheostomy procedures, predominantly vertical and horizontal. Various literature on the skin incision types in tracheostomy had contradictory findings, with different studies observing that one skin incision type had better outcomes than its counterpart. Hence the objective of this study was to compare the outcomes associated with vertical and horizontal skin incisions in patients undergoing tracheostomy. Method The present study assessed the outcome measures between the two incision types (vertical and horizontal) in tracheostomy. A prospective longitudinal study was done based on an academic tertiary hospital in Bhopal, Madhya Pradesh. Participants above 18 years who underwent tracheostomy were enrolled in the study and followed up over six months during intraoperative, immediate, within seven days, and long-term periods. Result In intraoperative complications, bleeding was most common (n = 15, 16.7%), followed by passage of tube into false tract (n = 6, 6.7%) and saturation drop (n = 2, 2.2%). Immediate complications comprised T-tube blockage (n = 4, 4.4%) and bleeding (n = 1, 1.1%). Complications within seven days occurred only in the horizontal group in which stomal site ulceration (n = 4, 6.7%) and delayed bleeding (n = 2, 3.3%) was seen, and one participant had unintended decannulation. In the long term, complications observed were stomal granulation (n = 9, 19.1%), dysphagia (n = 7, 14.9%), and unintended decannulation (n = 4, 8.5%). Conclusion In the current study, the most common intraoperative complication was bleeding, the immediate complication was tube dislodgement, and tracheostomy site ulcer was the most common complication within seven days, similar to the literature findings.

7.
Leuk Lymphoma ; : 1-13, 2024 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-38501751

RESUMO

A key hallmark of myelofibrosis is anemia, which ranges from mild to severe based on hemoglobin levels. To more clearly define outcomes with the Janus kinase (JAK) 1/JAK2/activin A receptor type 1 inhibitor momelotinib by anemia severity, we performed a descriptive post hoc exploratory analysis of the double-blind, randomized, phase 3 SIMPLIFY-1 study (NCT01969838; N = 432, JAK inhibitor naive, momelotinib vs. ruxolitinib); subgroups were defined by baseline hemoglobin: <10 (moderate/severe), ≥10 to <12 (mild), or ≥12 g/dL (nonanemic). Spleen and symptom results were generally consistent with those previously reported for the intent-to-treat population. In anemic subgroups, momelotinib was associated with higher rates of transfusion independence and reduced/stable transfusion intensity vs. ruxolitinib. No new or unexpected safety signals were identified. Overall, momelotinib provides spleen, symptom, and anemia benefits to JAK inhibitor-naive patients with myelofibrosis regardless of baseline hemoglobin level, and greater anemia-related benefits vs. ruxolitinib in patients with hemoglobin <12 g/dL.

8.
J Clin Exp Hepatol ; 14(4): 101393, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38550799

RESUMO

Objective: This article aims to evaluate the intrareader and interreader agreement of ultrasound (US) gallbladder reporting and data system (GB-RADS) and validate the risk of malignancy in each GB-RADS category. Materials and methods: This retrospective study comprised consecutive patients with nonacute gallbladder wall thickening who underwent US evaluation between January 2019 and December 2022. Three radiologists independently read the static US images and cine-loops for GB-RADS findings and assigned GB-RADS categories. The intraobserver (static images) and interobserver (static images and cine-loops) agreement was calculated using kappa statistics and Krippendorff's alpha. Another radiologist assigned a consensus GB-RADS category. The percentage of malignancy in each GB-RADS category was calculated. Results: Static US images of 414 patients (median age, 56 years; 288 women, benign = 45.6% and malignant = 54.4%) and cine-loops of 50 patients were read. There was weak to moderate intrareader agreement for most GB-RADS findings and moderate intrareader agreement for the GB-RADS category for all readers. On static images, the interreader agreement was acceptable for GB-RADS categories. On cine-loops, the interreader agreement for GB-RADS findings and categories was better than static images. The percentage of malignancy was 1.2%, 37%, 71.1%, and 89.1% in GB-RADS 2, 3, 4, and 5 categories. Conclusion: GB-RADS has moderate intrareader for GB-RADS categories. As originally proposed, the risk of malignancy is negligible in GB-RADS 2 category and highest in GB-RADS 5 category. However, the discriminatory performance of GB-RADS 3 and 4 categories is low. Larger multicenter studies with more readers must assess the reader agreement and validate the GB-RADS systems for wider clinical utilization.

9.
Int J Infect Dis ; : 107023, 2024 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-38555060

RESUMO

OBJECTIVES: To evaluate the clinical and economic outcomes in adults hospitalized with invasive pneumococcal disease (IPD) and non-invasive all-cause pneumonia (ACP) overall and by antimicrobial resistance (AMR) status. METHODS: Hospitalized adults from the BD Insights Research Database with an ICD10 code for IPD, non-invasive ACP or a positive Streptococcus pneumoniae culture/urine antigen test were included. Descriptive statistics and multivariable analyses were used to evaluate outcomes (in-hospital mortality, length of stay [LOS], cost per admission, and hospital margin [costs - payments]). RESULTS: The study included 88,182 adult patients at 90 US hospitals (October 2015-February 2020). Most (98.6%) had non-invasive ACP and 40.2% were <65 years old. Of 1,450 culture-positive patients, 37.7% had an isolate resistant to ≥1 antibiotic class. Observed mortality, median LOS, cost per admission, and hospital margins were 8.3%, 6 days, $9,791, and $11, respectively. Risk factors for mortality included ≥50 years of age, higher risk of pneumococcal disease (based on chronic or immunocompromising conditions), and intensive care unit admission. Patients with IPD had similar mortality rates and hospital margins compared with non-invasive ACP, but greater costs per admission and LOS. CONCLUSION: IPD and non-invasive ACP are associated with substantial clinical and economic burden across all adult age groups. Expanded pneumococcal vaccination programs may help reduce disease burden and decrease hospital costs.

12.
J Family Med Prim Care ; 13(1): 169-174, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38482275

RESUMO

Background: Despite efforts to improve healthcare infrastructure and service delivery, significant disparities in healthcare utilization persist, leading to suboptimal health outcomes and hindering progress toward achieving universal health coverage. This research article aims to conduct a qualitative exploration of the under-foot fall in utilization of health services, shedding light on the barriers and challenges faced by individuals in accessing and utilizing health care to inform targeted interventions and improve health service utilization. Methods: This qualitative study employed free listing, pile sorting, and focus group discussions (FGDs) as data collection methods. Representatives from various stakeholders involved in the primary healthcare delivery system were selected based on their vocalness, knowledge, willingness to participate, and heterogeneity of responses. Subsequently, FGDs and key informant interviews (KIIs) were conducted to further explore the identified barriers. The collected transcripts underwent manual thematic analysis using coding rules and theme generation procedures. Results: A total of 30 participants, including healthcare providers, community leaders, and individuals from the local community, took part in the qualitative exploration. The themes encompassed limited awareness and knowledge, geographical and infrastructural barriers, socioeconomic constraints, trust and perceptions of the healthcare system, and cultural and social factors. These findings provide valuable insights into the multifaceted barriers hindering healthcare utilization and can guide the development of targeted interventions and policies to improve healthcare access and delivery in the study area. Conclusion: The identified barriers, including limited awareness and knowledge, geographical and infrastructural challenges, socioeconomic constraints, trust and perceptions of the healthcare system, cultural and social factors, and gender disparities, are consistent with the existing literature.

13.
Clin Infect Dis ; 2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38483935

RESUMO

BACKGROUND: Growing evidence indicates antimicrobial resistance disproportionately affects individuals living in socially vulnerable areas. This study evaluated the association between Streptococcus pneumoniae (SP) antimicrobial resistance (AMR) and the CDC/ATSDR Social Vulnerability Index (SVI) in the United States. METHODS: Adult patients ≥ 18 years with 30-day nonduplicate SP isolates from ambulatory/hospital settings from January 2011-December 2022 with zip codes of residence were evaluated across 177 facilities in the BD Insights Research Database. Isolates were identified as SP AMR if they were non-susceptible to ≥ 1 antibiotic class (macrolide, tetracycline, extended-spectrum cephalosporins, or penicillin). Associations between SP AMR and SVI score (overall and themes) were evaluated using generalized estimating equations with repeated measurements within county to account for within-cluster correlations. RESULTS: Of 8,008 unique SP isolates from 574 US counties across 39 states, the overall proportion of AMR was 49.9%. A significant association between socioeconomic status (SES) theme and SP AMR was detected with higher SES theme SVI score (indicating greater social vulnerability) associated with greater risk of AMR. On average, a decile increase of SES, indicating greater vulnerability, was associated with a 1.28% increased risk of AMR (95% confidence interval [CI], 0.61%, 1.95%; P=0.0002). A decile increase of household characteristic score was associated with a 0.81% increased risk in SP AMR (95% CI,0.13%, 1.49%; P=0.0197). There was no association between racial/ethnic minority status, housing type and transportation theme, or overall SVI score and SP AMR. CONCLUSIONS: SES and household characteristics were the SVI themes most associated with SP AMR.

14.
EJHaem ; 5(1): 105-116, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38406514

RESUMO

Bone marrow fibrosis (BMF) is a pathological feature of myelofibrosis, with higher grades associated with poor prognosis. Limited data exist on the association between outcomes and BMF changes. We present BMF data from Janus kinase (JAK) inhibitor-naive patients from SIMPLIFY-1 (NCT01969838), a double-blind, randomized, phase 3 study of momelotinib vs ruxolitinib. Baseline and week 24 bone marrow biopsies were graded from 0 to 3 as per World Health Organization criteria. Other assessments included Total Symptom Score, spleen volume, transfusion independence status, and hemoglobin levels. Paired samples were available from 144 and 160 patients randomized to momelotinib and ruxolitinib. With momelotinib and ruxolitinib, transfusion independence was achieved by 87% and 44% of patients with BMF improvement of ≥1 grade and 76% and 56% of those with stable/worsening BMF; there was no association between BMF changes and transfusion independence for either arm (momelotinib, p = .350; ruxolitinib, p = .096). Regardless of BMF changes, hemoglobin levels also generally increased on momelotinib but decreased on ruxolitinib. In addition, no associations between BMF changes and spleen (momelotinib, p = .126; ruxolitinib, p = .407)/symptom (momelotinib, p = .617; ruxolitinib, p = .833) outcomes were noted, and no improvement in overall survival was observed with ≥1-grade BMF improvement (momelotinib, p = .395; ruxolitinib, p = .407). These data suggest that the anemia benefit of momelotinib is not linked to BMF changes, and question the use of BMF assessment as a surrogate marker for clinical benefit with JAK inhibitors.

15.
Antimicrob Resist Infect Control ; 13(1): 21, 2024 02 14.
Artigo em Inglês | MEDLINE | ID: mdl-38355621

RESUMO

BACKGROUND: Antimicrobial resistance research in uncomplicated urinary tract infection typically focuses on the main causative pathogen, Escherichia coli; however, little is known about the antimicrobial resistance burden of Klebsiella species, which can also cause uncomplicated urinary tract infections. This retrospective cohort study assessed the prevalence and geographic distribution of antimicrobial resistance among Klebsiella species and antimicrobial resistance trends for K. pneumoniae in the United States (2011-2019). METHODS: K. pneumoniae and K. oxytoca urine isolates (30-day, non-duplicate) among female outpatients (aged ≥ 12 years) with presumed uUTI at 304 centers in the United States were classified by resistance phenotype(s): not susceptible to nitrofurantoin, trimethoprim/sulfamethoxazole, or fluoroquinolone, extended-spectrum ß-lactamase-positive/not susceptible; and multidrug-resistant based on ≥ 2 and ≥ 3 resistance phenotypes. Antimicrobial resistance prevalence by census division and age, as well as antimicrobial resistance trends over time for Klebsiella species, were assessed using generalized estimating equations. RESULTS: 270,552 Klebsiella species isolates were evaluated (250,719 K. pneumoniae; 19,833 K. oxytoca). The most frequent resistance phenotypes in 2019 were nitrofurantoin not susceptible (Klebsiella species: 54.0%; K. pneumoniae: 57.3%; K. oxytoca: 15.1%) and trimethoprim/sulfamethoxazole not susceptible (Klebsiella species: 10.4%; K. pneumoniae: 10.6%; K. oxytoca: 8.6%). Extended-spectrum ß-lactamase-positive/not susceptible prevalence was 5.4%, 5.3%, and 6.8%, respectively. K. pneumoniae resistance phenotype prevalence varied (p < 0.0001) geographically and by age, and increased over time (except for the nitrofurantoin not susceptible phenotype, which was stable and > 50% throughout). CONCLUSIONS: There is a high antimicrobial resistance prevalence and increasing antimicrobial resistance trends among K. pneumoniae isolates from female outpatients in the United States with presumed uncomplicated urinary tract infection. Awareness of K. pneumoniae antimicrobial resistance helps to optimize empiric uncomplicated urinary tract infection treatment.


Assuntos
Klebsiella , Infecções Urinárias , Feminino , Humanos , Antibacterianos/farmacologia , beta-Lactamases/genética , Farmacorresistência Bacteriana , Escherichia coli , Klebsiella pneumoniae , Nitrofurantoína/farmacologia , Pacientes Ambulatoriais , Prevalência , Estudos Retrospectivos , Combinação Trimetoprima e Sulfametoxazol , Estados Unidos/epidemiologia , Infecções Urinárias/epidemiologia , Infecções Urinárias/tratamento farmacológico
16.
Curr Probl Diagn Radiol ; 53(3): 359-368, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38302304

RESUMO

This retrospective study aimed to reveal discrepancies between planned (Tplan) and actual (Tact) slot lengths of abdomen MRI exams, and to improve Tplan by predicting slot lengths via a machine learning algorithm. Tplan and Tact were retrieved from RIS and modality logfiles, respectively, covering 3038 MRI exams of 17 protocols performed at an abdomen department. Comparisons showed that 30% of exams exceeded planned slot lengths. On the other hand, exams completed within planning failed to manifest good adherence to schedule, as many of them were assigned with an unnecessarily long slot. While adjusting the planned exam duration by a fixed amount of time for each protocol could move Tplan closer to the mean or median Tact, the large spread of Tact would still be unaffected. This is why this study goes one step further, introducing a method to predict the required slot length not only per protocol, but for each individual exam. A Random Forest Regression model was trained on historic data to predict individual slot lengths (Tpred) based on patient and exam context. The correlation between Tpred and Tact was found to be better than that of Tplan and Tact, with Pearson correlation factors of 0.66 and 0.50, respectively. The overall adherence to schedule was also improved by the prediction, as seen by a reduction of both the root mean squared error (-28%) and the standard deviation (-16%) of the differences between planned/predicted slot times and Tact. To provide further insights into the discrepancies between planning and execution of MRI exams, nineteen exams from the Liver protocol with verified clinical information were selected. This case study showed that patient conditions, diagnostic purposes and the selection of sequences during exams could explain some variations of exam durations, but the potential for improving the exam time prediction by including this additional context is limited.


Assuntos
Fígado , Imageamento por Ressonância Magnética , Humanos , Estudos Retrospectivos , Imageamento por Ressonância Magnética/métodos
17.
Leukemia ; 38(3): 570-578, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38321107

RESUMO

Myeloproliferative neoplasms (MPNs) are a group of chronic hematologic malignancies that lead to morbidity and early mortality due to thrombotic complications and progression to acute leukemia. Clinical and mutational risk factors have been demonstrated to predict outcomes in patients with MPNs and are used commonly to guide therapeutic decisions, including allogenic stem cell transplant, in myelofibrosis. Adolescents and young adults (AYA, age ≤45 years) comprise less than 10% of all MPN patients and have unique clinical and therapeutic considerations. The prevalence and clinical impact of somatic mutations implicated in myeloid disease has not been extensively examined in this population. We conducted a retrospective review of patients evaluated at eight Canadian centers for MPN patients diagnosed at ≤45 years of age. In total, 609 patients were included in the study, with median overall survival of 36.8 years. Diagnosis of prefibrotic or overt PMF is associated with the lowest OS and highest risk of AP/BP transformation. Thrombotic complications (24%), including splanchnic circulation thrombosis (9%), were frequent in the cohort. Mutations in addition to those in JAK2/MPL/CALR are uncommon in the initial disease phase in our AYA population (12%); but our data indicate they may be predictive of transformation to post-ET/PV myelofibrosis.


Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Mielofibrose Primária , Trombocitemia Essencial , Trombose , Humanos , Adulto Jovem , Adolescente , Pessoa de Meia-Idade , Mielofibrose Primária/genética , Mielofibrose Primária/terapia , Policitemia Vera/genética , Trombocitemia Essencial/genética , Canadá/epidemiologia , Transtornos Mieloproliferativos/complicações , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/terapia , Trombose/genética , Janus Quinase 2/genética , Mutação , Calreticulina/genética
18.
Cureus ; 16(1): e52074, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38344535

RESUMO

INTRODUCTION: Malignancy of the nose and paranasal sinuses is a highly heterogeneous tumor group that arises from various cell types commonly seen in the fifth to sixth decades of life, with twice as much commonness in males. Patients present with varied clinical presentations like nasal obstruction, facial swelling, orbital complications, etc. Squamous cell carcinoma and adenocarcinoma are the most common variant. Surgery followed by adjuvant chemo or radiotherapy is the treatment of choice. METHODS: The study was undertaken in the Department of Otorhinolaryngology All India Institute of Medical Sciences, Bhopal, India, from 2021 to 2023. It was a retrospective study in which patients diagnosed and underwent treatment in the last 2 years were enrolled. Data were retrieved from the medical record department and surgical registry. Twenty-eight patients were recruited for the study. Detailed history, clinical examination, imaging findings, surgical plans, postoperative adjuvant therapy details, and histopathological findings were recorded. RESULTS: There were 18 (64.2%) males and 10 (35.8%) females, with a male-to-female ratio of 1.8: 1. The mean age of patients was 50.5 years. Facial swelling was the most frequent symptom (n=15, 54%). Twenty-one (75%) patients use chewable tobacco, while sixteen (57%) are smokers. All our patients belong to the lower socioeconomic group. Endoscopic resection was done in 15 (62.5%) patients, and combined open and endoscopic approaches were used in 9 (37.5%) patients. The most common histological variant was squamous cell carcinoma (n=8, 28%). CONCLUSION: Malignancy of the nose and paranasal sinus is very rare. They presented with varied masked clinical presentations of benign diseases. Early identification and high clinical suspicion, along with imaging studies, are pivotal in managing malignancy of the nose and paranasal sinuses.

19.
J Clin Exp Hepatol ; 14(3): 101348, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38389867

RESUMO

Background: Biliary obstruction in gallbladder cancer (GBC) is associated with worse prognosis and needs drainage. In patients with biliary confluence involvement, percutaneous biliary drainage (PBD) is preferred over endoscopic drainage. However, PBD catheters are associated with higher complications compared to endoscopic drainage. PBD with self-expandable metal stents (SEMS) is desirable for palliation. However, the data in patients with unresectable GBC is lacking. Materials and methods: This retrospective study comprised consecutive patients with proven GBC who underwent PBD-SEMS insertion between January 2021 and December 2022. Technical success, post-procedural complications, clinical success, duration of stent patency, and biliary reinterventions were recorded. Clinical follow-up data was analysed at 30 days and 180 days of SEMS insertion and mortality was recorded. Results: Of the 416 patients with unresectable GBC, who underwent PBD, 28 (median age, 50 years; 16 females) with PBD-SEMS insertion were included. All SEMS placement procedures were technically successful. There were no immediate/early post-procedural complications/deaths. The procedures were clinically successful in 63.6% of the patients with hyperbilirubinemia (n = 11). Biliary re-interventions were done in 6 (21.4%). The survival rate was 89.3 % (25/28) at 30 days and 50% at 180 days. The median follow-up duration was 80 days (range, 8-438 days). Conclusion: PBD-SEMS has moderate clinical success and 6-months patency in almost half of the patients with metastatic GBC and must be considered for palliation.

20.
J Clin Exp Hepatol ; 14(3): 101355, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38389866

RESUMO

Organ transplantation is the primary therapy for organ failure caused by telomere biology disorder (TBD). We describe the first documented case of simultaneous liver and kidney transplantation (SLKTx) for TBD, although the diagnosis of TBD was reached only three months following SLKTx. The patient was born prematurely, displayed growth retardation, and developed chronic kidney and liver diseases. His pre-SLKTx autoimmune, metabolic, and viral assessments were negative, and persistent pancytopenia (bone marrow cellularity 70-80%) was attributed to renal disease-associated bone marrow changes. Following SLKTx, he was discharged with stable graft function on tacrolimus and prednisolone. Although mycophenolate mofetil was discontinued on the second postoperative day, his pancytopenia persisted. Despite extensive evaluations, including drug, immune, nutritional, and viral assessments, all results were negative. A bone marrow biopsy conducted three months post-transplant revealed significant hypocellularity (40-50%). Whole genome sequencing revealed a likely pathogenic variant of the TINF2 gene. The patient was subsequently treated with danazol. At the nine-month follow-up post-SLKTx, he exhibited stable graft function and improved cell counts while maintaining triple-drug immunosuppression. Given the lack of uniform diagnostic criteria for TBD, healthcare providers must be vigilant with patients presenting with multi-organ failure and persistent cytopenias. Effective pre-transplant screening for TBD can lead to timely diagnoses, better management, and improved post-transplant outcomes.

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