RocScholar (Rochester Regional Health)
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Clinical Spectrum and Treatment Outcomes of Rare Bleeding Disorders in Female Patients: A Two-Center Experience in North Pakistan
No full textIntroduction Rare bleeding disorders (RBDs) result from genetic mutations in clotting factors. These RBDs vary in prevalence and are often underdiagnosed due to mild symptoms. Treatment is challenging due to limited clinical data and primarily involves substituting deficient factors and using adjuvant therapies. Women with RBDs face unique risks, including gynecologic bleeding, hemorrhagic ovarian cysts, and complications during pregnancy. These issues can significantly impact their quality of life and employment. This study was conducted to characterize the patterns of bleeding disorders, clinical manifestations, and treatment outcomes in female patients. Methods In this cross-sectional study, we included patients from the Hemophilia Treatment Center (HTC) and Armed Forces Bone Marrow Transplant Centre (AFBMTC) Rawalpindi between 2011 and 2023, using a convenience sampling technique. Data were extracted from patient files, including medical history, factor activity levels, symptoms, treatments, and medications. Eligible participants had congenital coagulation factor deficiencies, while those with platelet function or acquired coagulation disorders were excluded. Results In our study of 50 patients with RBD, the median age at bleeding presentation was two years; 72% of cases were born of consanguineous marriages, and 57% had a positive family history of bleeding disorders. Factor V deficiency was the most prevalent (28%), and major bleeding episodes occurred in 52% of cases. The predominant clinical presentations included menorrhagia (74%) and epistaxis (58%). Treatment primarily involved antifibrinolytics (98%) and FFP transfusions (96%), with significant associations identified among various risk factors related to bleeding. All patients were counseled regarding local measures for bleeding control. There was a moderate correlation found between factors V and VII with the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH BAT) score. There is a weak correlation between factors X and XI with the ISTH BAT score. There was no correlation found in factor I, factor XI, factor XII, and combined factors V+VIII deficiency. Conclusion Women with RBDs face a spectrum of bleeding challenges, significantly impacting their quality of life and reproductive health. Early diagnosis and personalized treatment strategies are paramount in mitigating bleeding risks and enhancing patient outcomes
Therapeutic potential of plant polyphenols in acute pancreatitis
No full textAcute pancreatitis is a potentially life-threatening inflammatory disorder of the exocrine pancreas characterized by early activation of pancreatic enzymes followed by macrophage-driven inflammation, and pancreatic acinar cell death. The most common causes are gallstones and excessive alcohol consumption. Inflammation and oxidative stress play critical roles in its pathogenesis. Despite increasing incidence, currently, no specific drug therapy is available to treat or prevent acute pancreatitis, in particular severe acute pancreatitis. New therapeutic agents are very much needed. Plant polyphenols have attracted extensive attention in the field of acute pancreatitis due to their diverse pharmacological properties. In this review, we discuss the potential of plant polyphenols in inhibiting the occurrence and development of acute pancreatitis via modulation of inflammation, oxidative stress, calcium overload, autophagy, and apoptosis, based on the currently available in vitro, in vivo animal and very few clinical human studies. We also outline the opportunities and challenges in the clinical translation of plant polyphenols for the treatment of the disease. We concluded that plant polyphenols have a potential therapeutic effect in the management and treatment of acute pancreatitis. Knowledge gained from this review will hopefully inspire new research ideas and directions for the development and application of plant polyphenols for treating this disease
Pulmonary outcomes of incretin-based therapies in COPD patients receiving single-inhaler triple therapy
No full textBackground: Patients with COPD on triple therapy often face exacerbations and comorbidities. Emerging evidence suggests that glucagon-like peptide-1 (GLP-1) analogues may reduce the risk of exacerbation in patients with COPD and type 2 diabetes mellitus (T2DM). This study investigates the impact of GLP-1 analogues on pulmonary outcomes in patients with COPD on single-inhaler triple therapy (SITT) and T2DM.
Methods: We conducted a retrospective cohort study using the TriNetX database and analysed adult patients with COPD and T2DM who received SITT between April 2005 and July 2023. Patients were categorised into GLP-1 analogue and dipeptidyl peptidase-4 inhibitor (DPP4i) cohorts. The primary efficacy outcome was COPD exacerbation, and the secondary efficacy outcomes were pneumonia, acute respiratory distress syndrome, intubation, oxygen dependence and all-cause mortality. The secondary outcomes were serious gastrointestinal adverse events.
Results: We included 6898 patients, with 4184 receiving GLP-1 analogues and 2714 receiving DPP4i. After matching, 1751 GLP-1 analogue users were matched with 1751 DPP4i users. GLP-1 analogue users had an 18% lower risk of COPD exacerbation (hazard ratio (HR) 0.82 (95% CI 0.71-0.94)), a 28% reduced risk of pneumonia (HR 0.72 (95% CI 0.61-0.85)), a 34% reduced risk of oxygen dependence (HR 0.66 (95% CI 0.47-0.91)) and a 40% decreased risk of all-cause mortality (HR 0.60 (95% CI 0.47-0.77)). No significant serious gastrointestinal adverse events were observed.
Conclusion: GLP-1 analogues may be associated with reduced COPD exacerbations, pulmonary comorbidities and mortality in patients with COPD receiving SITT and T2DM, with no significant serious gastrointestinal safety concerns
Kabuki Syndrome With Cardiac Manifestations: A Case Report and Mini-Literature Review From the United Arab Emirates (UAE)
No full textKabuki syndrome (KS) is a rare genetic syndrome with an unknown exact etiology with suggestive autosomal dominant inheritance pattern with variable expressivity and environmental influences. KS is diagnosed based on five cardinal signs: craniofacial dysmorphia, skeletal anomalies, dermatoglyphic abnormalities, mental retardation, and postnatal growth deficiency. An eight-year-old Jordanian girl was diagnosed with KS based on characteristic clinical features at the age of four. The patient presented typical facies of KS, with elongated palpebral fissure and the eversion of the lateral part of the lower 1/3 of the eyelid; skeletal and limb abnormalities, including the early closure of the anterior fontanel; cardiogenic manifestations; global developmental delays; moderate hearing impairment; and strabismus with bilateral hyperopia. Patients with KS have various skeletal, mainly cranial, anomalies, including coronal and metopic synostosis. Cardiac malformations and aortic coarctation more commonly occur in male KS patients, supporting the X-linked hypothesis. The most common ophthalmic abnormalities in KS are strabismus and ptosis. In addition, both dental and otologic problems are common in KS. Finally, mild to moderate cognitive impairment in KS leads to significant language delays. KS is primarily diagnosed based on clinical presentation even with significant variability in the associated anomalies. In this case, we discuss the first female Kabuki syndrome (KS) patient in the United Arab Emirates (UAE) who presented with moderate to severe aortic coarctation and underwent corrective surgery with balloon dilation twice at the age of four. The patient underwent confirmatory genetic testing, identifying a heterozygous variant in the KDM6A gene. A multidisciplinary team including a general pediatrician, cardiologist, neurologist, orthopedics specialist, ophthalmologist, otolaryngologist, and other specialties is needed to improve the course and prognosis of KS patients and to enhance the quality of life
E-20 TAVR vs. SAVR in Patients with Severe Symptomatic Aortic Stenosis and Prior Mediastinal Radiation: A Meta-Analysis of Short-Term and One-Year Outcomes
No full textResults of Lung Cancer Screening at a Rural Hospital Network in the United States
No full textINTRODUCTION AND BACKGROUND: Lung cancer is the second most common cancer in the world. United States Preventive Services Task Force (USPSTF) has specific guidelines for lung cancer screening in high-risk individuals. Multiple studies including National Lung Cancer Screening trial (NLST) trial has shown reduction in lung cancer mortality with lung cancer screening. Most of these studies focus on the urban population and there are very few studies that have studied lung cancer screening in the rural US population.
MATERIALS AND METHODS: This is a retrospective study reviewing patients that were diagnosed with lung cancer after undergoing lung cancer screening in a rural hospital network.
RESULTS: In our study, lung cancer detection rate was 2.96 % (74 cases out of 2500 cases screened). 63.3 % of cancer patients were diagnosed on stage I. The median household income was $50,262 which was below the average median household income for United States. The median pack years of the cancer patients was 50. Significant proportion of cancer patients were referred by either Nurse Practitioners or Physician Assistants (43 %) compared to physicians (57 %).
CONCLUSION: Lung Cancer Screening can play a pivotal role in detection of lung cancer at early stages in rural population
A Guide to Pediatric Antibiotic Allergy Testing: A Report From the US Drug Allergy Registry
No full textPediatric antibiotic labels are common, and unnecessary antibiotic avoidance is associated with negative personal and public health outcomes; as a result, there is an increasing emphasis on the importance of pediatric antibiotic allergy evaluations. Different testing strategies have been advised, including skin testing and challenge testing with varied doses and duration. Established consensus testing protocols are lacking. The US Drug Allergy Registry Pediatrics (USDAR-Peds) is a multisite prospective study designed for epidemiology and outcome evaluations of pediatric drug hypersensitivity reactions. Interpretation of multisite data requires a uniform clinical approach, and the USDAR-Peds standardized protocols were developed in response to this need. This rostrum aims to provide a rationale and framework for standardization for pediatric antibiotic allergy protocols and assessment of positive reactions through a pediatric-specific adaptation of the USDAR immediate reaction grading scale to create consistency for multisite research collaboration efforts such as USDAR-Peds
Vessels and Valves: Cardiac Manifestations of ANCA-Associated Vasculitis
No full textVessels and Valves: Cardiac Manifestations of ANCA-Associated Vasculitis. Dr. Asim Khanfar, R2, Internal Medicine Resident Program, Rochester General Hospital; Dr. Anthony Ocon, MD PhD, Rheumatology, Rochester Regional Health
Objectives: Present unique manifestations of ANCA-Associated Vasculitis Promote collaborative care between disciplines in complex cases like AA
Trend of cardiovascular mortality among metastatic renal cell cancer patients in the US from 2005 to 2020
No full textRecent advances in treating advanced renal cell carcinoma (RCC) with distant metastasis have significantly enhanced cancer-specific outcomes. However, these patients are at increased risk for cardiovascular disease (CVD) and events. This study aims to investigate the trend of incidence-based mortality specific to CVD in patients with metastatic RCC. We examined data from 26,501 adult patients aged 18 and older diagnosed with metastatic RCC between 2005 and 2020 in the Surveillance, Epidemiology, and End Results (SEER) 17 registry. We used a linear regression model to examine trends in metastatic RCC incidence and mortality rates among the US general population stratified by gender using R statistical software version 4.3.2. Among 26,501 adult patients diagnosed with metastatic RCC, RCC-specific mortality accounted for 18,258 (81.1 %) deaths, while mortality due to cardiovascular (CV) events was 737 (3.3 %). The overall incidence rate of metastatic RCC increased over time (p-value \u3c 0.001). A significant interaction between time and sex was found, indicating greater increase in metastatic RCC incidence rates in males than females over time (p \u3c 0.001 for interaction). There was no statistically significant difference in metastatic RCC-specific mortality rate between sexes (p = 0.25) or over time (p = 0.89). The combined CV mortality rate (in both sexes) increased from 0.02 to 0.04 during the years 2005-2020 and was statistically significant (p = 0.001) with no significant difference in mortality between sexes (p = 0.13). The trend of increasing metastatic RCC incidence with no change in all cause and metastatic RCC specific mortality would suggest benefit of advances in therapy. However, the slow but gradual rise in CV mortality needs to be further studied. Keywords: advanced renal cell carcinoma, cardiovascular mortality, sex