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BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes, representing less than one percent of non-Hodgkin lymphomas. Given its rarity, the true incidence of HSTCL is unknown and most data have been extrapolated through case reports. To the best of our knowledge, the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014, with a sample size of 122 patients. AIM: To paint the most updated epidemiological picture of HSTCL. METHODS: A total of 186 patients diagnosed with HSTCL, between 2000 and 2017, were ultimately enrolled in our study by retrieving data from the Surveillance, Epidemiology, and End Results database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of HSTCL. Variables with a P value < 0.01 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio of greater than 1 representing adverse prognostic factors. RESULTS: Male gender was the most represented. HSTCL was most common in middle-aged patients (40-59) and less common in the elderly (80+). Non-Hispanic whites (60.75%) and non-Hispanic blacks (20.97%) were the most represented racial groups. Univariate Cox proportional hazard regression analysis of factors influencing all-cause mortality showed a higher OM among non-Hispanic black patients. CSM was also higher among non-Hispanic blacks and patients with distant metastasis. Multivariate Cox proportional hazard regression analysis of factors affecting CSM revealed higher mortality in patients aged 80 or older and non-Hispanic blacks. CONCLUSION: Overall, the outlook for this rare malignancy is very grim. In this retrospective cohort study of the United States population, non-Hispanic blacks and the elderly had a higher CSM. This data highlights the need for larger prospective studies to investigate factors associated with worse prognosis in one ethnic group, such as treatment delays, which have been shown to increase mortality in this racial/ethnic group for other cancers.
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Carnitine deficiency is a rare metabolic condition that can result in fasting hypoglycemia. Carnitine deficiency could be primary or secondary to other conditions. Among secondary causes, antiepileptics such as valproic acid have been incriminated. Valproic acid is known to deplete carnitine stores and inhibit the process of ß-oxidation. Herein we report the case of a 44-year-old female with epilepsy that presented with breakthrough seizures associated with hypoglycemia despite being on appropriate antiepileptic therapy. The patient was later found to have carnitine deficiency. Discontinuation of valproic acid and supplementation with l-carnitine resolved the patient's hypoglycemia and breakthrough seizures. With this case report, we hope to encourage clinicians to include carnitine deficiency in the differential diagnosis of unexplained hypoglycemia.
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BACKGROUND: Data regarding the worldwide gastrointestinal surgery rates in patients with Crohn's disease (CD) remains limited. AIM: To systematically review the global variation in the rates of surgery in CD. METHODS: A comprehensive search analysis was performed using multiple electronic databases from inception through July 1, 2020, to identify all full text, randomized controlled trials and cohort studies pertaining to gastrointestinal surgery rates in adult patients with CD. Outcomes included continent based demographic data, CD surgery rates over time, as well as the geoepidemiologic variation in CD surgery rates. Statistical analyses were conducted using R. RESULTS: Twenty-three studies spanning four continents were included. The median proportion of persons with CD who underwent gastrointestinal surgery in studies from North America, Europe, Asia, and Oceania were 30% (range: 1.7%-62.0%), 40% (range: 0.6%-74.0%), 17% (range: 16.0%-43.0%), and 38% respectively. No clear association was found regarding the proportion of patients undergoing gastrointestinal surgery over time in North America (R 2 = 0.035) and Europe (R 2 = 0.100). A moderate, negative association was seen regarding the proportion of patients undergoing gastrointestinal surgery over time (R 2 = 0.520) in Asia. CONCLUSION: There appears to be significant inter-continental variation regarding surgery rates in CD. Homogenous evidence-based guidelines accounting for the geographic differences in managing patients with CD is prudent. Moreover, as a paucity of data on surgery rates in CD exists outside the North American and European continents, future studies, particularly in less studied locales, are warranted.
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BACKGROUND: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms of the gastrointestinal tract (GIT) that represent approximately 1 to 2 percent of primary gastrointestinal (GI) cancers. Owing to their rarity, very little is known about their overall epidemiology, and the prognostic factors of their pathology. The current study aimed to evaluate the independent determinants of mortality in patients diagnosed with GISTs over the past decade. METHODS: Our study comprised 2374 patients diagnosed with GISTs from 2000 to 2017 from the Surveillance, Epidemiology, and End Results (SEER) database. We analyzed the baseline characteristics, and overall mortality (OM), as well as the cancer-specific mortality (CSM) of GISTs. Variables with a p value < 0.01 in the univariate Cox regression were incorporated into the multivariate Cox model, to determine the independent prognostic factors. RESULTS: Multivariate Cox proportional hazard regression analyses of factors affecting the all-cause mortality and GIST-related mortality among US patients between 2010 and 2017 revealed a higher overall mortality in non-Hispanic Black patients (HR = 1.516, 95% CI 1.172-1.961, p = 0.002), patients aged 80+ (HR = 9.783, 95% CI 4.185-22.868, p = 0), followed by those aged 60-79 (HR = 3.408, 95% CI 1.488-7.807, p = 0.004); male patients (HR = 1.795, 95% CI 1.461-2.206, p < 0.001); patients with advanced disease with distant metastasis (HR = 3.865, 95% CI 2.977-5.019, p < 0.001), followed by cases with regional involvement via both direct extension and lymph node involvement (HR = 3.853, 95% CI 1.551-9.57, p = 0.004); and widowed patients (HR = 1.975, 95% CI 1.494-2.61, p < 0.001), followed by single patients (HR = 1.53, 95% CI 1.154-2.028, p = 0.003). The highest CSM was observed in the same groups, except widowed patients and patients aged 60-79. The highest CSM was also observed among patients that underwent chemotherapy (HR = 1.687, 95% CI 1.19-2.392, p = 0.003). CONCLUSION: In this updated study on the outcomes of patients with GISTs, we found that non-Hispanic Black patients, male patients, and patients older than 60 years have a higher mortality with GISTs. Furthermore, patients who have received chemotherapy have a higher GIST-specific mortality, and married patients have a lower mortality. However, we do not know to what extent these independent prognostic factors interact with each other to influence mortality. This study paves the way for future studies addressing these interactions. The results of this study may help treating clinicians to identify patient populations associated with a dismal prognosis, as those may require closer follow-up and more intensive therapy; furthermore, with married patients having a better survival rate, we hope to encourage clinicians to involve family members of the affected patients early in the disease course, as the social support might impact the prognosis.
Subject(s)
Gastrointestinal Stromal Tumors , Humans , Male , Databases, Factual , Disease Progression , Gastrointestinal Stromal Tumors/therapy , Black or African American , Outcome Assessment, Health Care , Aged , Aged, 80 and over , Middle AgedABSTRACT
The gut microbiome plays an important role in the variation of pharmacologic response. This aspect is especially important in the era of precision medicine, where understanding how and to what extent the gut microbiome interacts with drugs and their actions will be key to individualizing therapy. The impact of the composition of the gut microbiome on the efficacy of newer cancer therapies such as immune checkpoint inhibitors and chimeric antigen receptor T-cell treatment has become an active area of research. Pancreatic adenocarcinoma (PAC) has a poor prognosis even in those with potentially resectable disease, and treatment options are very limited. Newer studies have concluded that there is a synergistic effect for immunotherapy in combination with cytotoxic drugs, in the treatment of PAC. A variety of commensal microbiota can affect the efficacy of conventional chemotherapy and immunotherapy by modulating the tumor microenvironment in the treatment of PAC. This review will provide newer insights on the impact that alterations made in the gut microbial system have in the development and treatment of PAC.
Subject(s)
Adenocarcinoma , Gastrointestinal Microbiome , Microbiota , Neoplasms , Pancreatic Neoplasms , Humans , Pancreatic Neoplasms/therapy , Adenocarcinoma/therapy , Immunotherapy , Neoplasms/therapy , Tumor Microenvironment , Pancreatic NeoplasmsABSTRACT
BACKGROUND: Primary malignant melanomas of the Gastrointestinal mucosa are uncommon. Most cases of gastrointestinal (GI) melanomas are secondary, arising from metastasis at distant sites. The purpose of this study is to assess to what extent the interaction between independent prognostic factors (age and tumor site) of primary GI melanoma influence survival. Furthermore, we also aimed to investigate the clinical characteristics, survival outcomes, and independent prognostic factors of patients with primary GI melanoma in the past decade. METHODS: A total of 399 patients diagnosed with primary GI melanoma, between 2008 and 2017, were enrolled in our study by retrieving data from the Surveillance, Epidemiology, and End Results (SEER) database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of primary GI melanoma. Variables with a p value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox model (model 1) to determine the independent prognostic factors, with a hazard ratio (HR) of greater than 1 representing adverse prognostic factors. Furthermore, we analyzed the effect of the interaction between age and primary location on mortality (model 2). RESULTS: Multivariate cox proportional hazard regression analyses revealed higher OM in age group 80+ (HR = 5.653, 95% CI 2.212-14.445, p = 0), stomach location of the tumor (HR = 2.821, 95% CI 1.265-6.292, p = 0.011), regional lymph node involvement only (HR = 1.664, 95% CI 1.051-2.635, p < 0.05), regional involvement by both direct extension and lymph node involvement (HR = 1.755, 95% CI 1.047-2.943, p < 0.05) and distant metastases (HR = 4.491, 95% CI 3.115-6.476, p = 0), whereas the lowest OM was observed in patients with small intestine melanoma (HR = 0.383, 95% CI 0.173-0.846, p < 0.05). Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups and lower CSM in small intestine and colon melanoma excluding the rectum. For model 2, considering the interaction between age and primary site on mortality, higher OM was found in age group 80+, followed by age group 40-59 then age group 60-79, regional lymph node involvement only, regional involvement by both direct extension and lymph node involvement and distant metastases. The small intestine had a lower OM. The rectum as primary location and the age range 40-59 interacted to lower the OM (HR = 0.14, 95% CI 0.02-0.89, p = 0.038). Age and primary gastric location did not interact to affect the OM. For the CSM, taking into account the interaction between age and the primary location, higher mortality was found in the same groups and the colon location. The primary colon location also interacted with the age group 40-59 to increase the CSM (HR = 1.38 × 109, 95% CI 7.80 × 107-2.45 × 1010, p = 0). CONCLUSIONS: In this United States population-based retrospective cohort study using the SEER database, we found that only the age range 40-59 interacted with the rectum and colon to lower and increase mortality respectively. Primary gastric location, which was the single most important location to affect mortality, did not interact with any age range to influence mortality. With those results, we hope to shed some light on this rare pathology with a very dismal prognosis.
Subject(s)
Gastrointestinal Neoplasms , Melanoma , Humans , United States/epidemiology , Adult , Middle Aged , Retrospective Studies , Lymph Nodes/pathology , Melanoma/pathology , Gastrointestinal Neoplasms/pathology , PrognosisABSTRACT
BACKGROUND AND AIMS: Multiple myeloma (MM) is a plasma cell dyscrasia that is common among patients with autoimmune diseases. However, the association between ulcerative colitis (UC) and multiple myeloma (MM) is yet to be established. We aimed to evaluate the prevalence of MM among patients with UC in the United States. METHODS: This cross-sectional cohort analysis used the National Inpatient Sample from 2015-2018 to assess the overall MM prevalence among patients with and without UC, and within specific demographic subgroups. Prevalences were compared using a logistic regression model controlling for sex and age. RESULTS: The crude prevalence of MM among patients with UC (n = 1750) compared with patients without UC (n = 366,265) was 0.44% vs. 0.37%, respectively. Patients with UC had increased overall odds of having MM (odds ratio (OR), 1.26). Males with UC had higher prevalence of MM (53.7% vs. 46.3%, respectively) than females. Patients with UC and MM were more likely to be African American than White (15.6% vs. 9.2%, respectively). Patients with UC age >64 had a higher prevalence of MM than those aged below 65 (70.9% vs. 29.1%, respectively). Patients with UC who were obese (BMI > 30) had a higher prevalence of MM than those who were non-obese (12.6% vs. 8.3%). CONCLUSIONS: Overall, UC appears to be associated with MM. This association can be particularly observed in specific demographic groups, such as obese, African American males, or patients >64 years of age. Thus, a high degree of clinical suspicion for MM is warranted, even with minimal symptomatology, in patients with UC, in particular among elder, obese, and African American males.
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Acute pancreatitis is an inflammatory condition, which is a leading gastrointestinal cause of hospitalization in the United States. Several conditions are associated with acute pancreatitis. More recently, there have been a few cases reported of acute pancreatitis following the Pfizer-BioNTech COVID-19 mRNA vaccine. To our knowledge, no cases of acute pancreatitis have been yet reported following the Johnson & Johnson's Janssen COVID-19 vaccine (J& J vaccine). Herein we report a 34-year-old male with no significant past medical history admitted with acute necrotizing pancreatitis, the day following the receipt of the J&J vaccine. Based on the Naranjo and the modified Naranjo scale, the patient met the requirements for probable drug induced pancreatitis. This case report has the objective to raise awareness of a potentially severe side effect of the J&J vaccine. We hope to use this case to support screening all patients for previous history of acute pancreatitis before administration of the J& J vaccine.
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OBJECTIVE: Although Socioeconomic status (SES), race/ethnicity, surgical type, and treatment delays are associated with breast cancer mortality outcomes, studies on these associations have been contrasting. This study examined the racial/ethnic and SES differences in surgical treatment types and treatment delays. Also, we quantified the extent to which these differences explained the racial/ethnic disparities in breast cancer mortality. METHODS: We studied 290,066 women 40 + years old diagnosed with breast cancer between 2010 and 2017 identified from the Surveillance, Epidemiology, and End Results database. We performed logistic regression models to examine the association of SES and race/ethnicity with surgical treatment type and treatment delays. We performed mediation analysis models to quantify the extent to which mortality differences were mediated by treatment, sociodemographic, and clinicopathologic factors. RESULTS: Non-Hispanic (NH) Black [Odds ratio (OR) = 1.16, 95% CI 1.13-1.19] and Hispanic women [OR = 1.27, 95% CI 1.24-1.31] were significantly more likely to undergo mastectomy compared to NH White women. Similarly, NH Black and Hispanic women had higher odds of delayed treatment than NH Whites. Patients in the highest SES quintile, compared to those in lowest the lowest, were less likely to experience breast cancer-specific mortality (BCSM). Variations in treatment, SES, and clinicopathological factors significantly explained 70% of the excess BCSM among NH Blacks compared to their NH White counterparts. CONCLUSIONS: Bridging the gap of access to adequate healthcare services for all to diminish the disproportionate burden of breast cancer would require a multifactorial approach that addresses several biological and social factors that cause these differences.
Subject(s)
Breast Neoplasms , Adult , Female , Humans , Breast Neoplasms/epidemiology , Ethnicity , Hispanic or Latino , Mastectomy , Socioeconomic Factors , Black or African American , White , SEER Program , United StatesABSTRACT
BACKGROUND AND AIM: Recently, there has been an increased focus on the role nutrition and diet play in maintaining health in inflammatory bowel disease (IBD). We aimed to assess the overall quality, strength, and transparency of conflicts among guidelines on nutrition/diet in IBD. METHODS: A systematic search was performed on multiple databases from inception until January 1, 2021, to identify guidelines pertaining to nutrition or diet in IBD. All guidelines were reviewed for disclosure of conflicts of interest (COI) and funding, recommendation quality and strength, external document review, patient representation, and plans for update-as per Institute of Medicine (IOM) standards. In addition, recommendations and their quality were compared between guidelines/societies.â RESULTS: Seventeen distinct societies and a total of 228 recommendations were included. Not all guidelines provided recommendations on key aspects of diet-such as the role of supplements or the appropriate micro/macro nutrition in IBD. Fifty-nine percent of guidelines reported on COI, 24% underwent external review, and 41% included patient representation. 18.4%, 25.9%, and 55.7% of recommendations were based on high-, moderate-, and low-quality evidence, respectively. 10.5%, 24.6%, and 64.9% of recommendations were strong, weak/conditional, and did not provide a strength, respectively. The proportion of high-quality evidence (p = 0.12) and strong recommendations (p = 0.83) did not significantly differ across societies. CONCLUSIONS: Many guidelines do not provide recommendations on key aspects of diet/nutrition in IBD. As over 50% of recommendations are based on low-quality evidence, further studies on nutrition/diet in IBD are warranted to improve the overall quality of evidence.
Subject(s)
Diet , Inflammatory Bowel Diseases , Humans , Nutritional Status , Dietary Supplements , Databases, FactualABSTRACT
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