Polycystic Kidney Disease Diet: What is Known and What is Safe.

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by kidney cyst formation and progressive kidney function loss. Dietary interventions such as caloric restriction, intermittent fasting, and ketogenic diet have recently emerged as potential strategies to induce metabolic reprogramming and slow ADPKD progression. We review the available evidence supporting the efficacy and safety of these interventions in ADPKD. Dietary interventions show promise in managing ADPKD by improving metabolic health and reducing oxidative stress. However, while preclinical studies have shown favorable outcomes, limited clinical evidence supports their effectiveness. In addition, the long-term consequences of these dietary interventions, including their effect on adverse events in patients with ADPKD, remain uncertain. To optimize ADPKD management, patients are advised to follow a dietary regimen that aims to achieve or maintain an ideal body weight and includes high fluid intake, low sodium, and limited concentrated sweets. Caloric restriction seems particularly beneficial for patients with overweight or obesity because it promotes weight loss and improves metabolic parameters. Supplementation with curcumin, ginkgolide B, saponins, vitamin E, niacinamide, or triptolide has demonstrated uncertain clinical benefit in patients with ADPKD. Notably, ß -hydroxybutyrate supplements have shown promise in animal models; however, their safety and efficacy in ADPKD require further evaluation through well-designed clinical trials. Therefore, the use of these supplements is not currently recommended for patients with ADPKD. In summary, dietary interventions such as caloric restriction, intermittent fasting, and ketogenic diet hold promise in ADPKD management by enhancing metabolic health. However, extensive clinical research is necessary to establish their effectiveness and long-term effects. Adhering to personalized dietary guidelines, including weight management and specific nutritional restrictions, can contribute to optimal ADPKD management. Future research should prioritize well-designed clinical trials to determine the benefits and safety of dietary interventions and supplementation in ADPKD.

Acquired laryngeal hemangioma: A rare presentation in an adult.

Laryngeal hemangiomas are rare vascular tumors that mostly present in children. The objective of this publication is to shed the light over this rare diagnosis. We report a case of adult onset of laryngeal hemangioma arising from the junction of the vocal fold and vocal process. The patient presented with a history of hoarseness of voice. An in-office laryngoscopy was performed that revealed a pink lobulated mass arising from the left hemilarynx. Intraoperatively, suspension microlaryngoscopy was performed; the lesion was identified and successfully resected using cold dissection technique. Histopathological analysis was consistent with laryngeal hemangioma. The patient is now a year post resection and remains asymptomatic without signs of recurrence. To conclude, adult onset of laryngeal hemangioma is very rare. When present, resection with either or both cold steel (microscissors) and laser have shown good outcome. Patients should be monitored afterwards for possible recurrence.

Health Literacy Levels and Predictors Among Lebanese Adults Visiting Outpatient Clinics in Beirut.

BACKGROUND: To date, there has been a dearth of research on health literacy in the Eastern Mediterranean Region and in particular Lebanon. OBJECTIVES: This cross-sectional study assessed the levels and correlates of health literacy in Lebanese adults. METHODS: A total of 587 participants (54.5% women) were recruited from the outpatient clinics of five health facilities in Beirut. The questionnaire consisted of the Arabic version of the Functional Health literacy Scale, the Arabic short version of the European Health Literacy Survey, and questions on sociodemographic and health-related factors. Descriptive and inferential statistics were performed to assess the association of these factors with functional health literacy (FHL) and comprehensive health literacy (CHL) levels. KEY RESULTS: About 65.8% had inadequate or problematic FHL and 43.8% had inadequate or problematic CHL. Although FHL was negatively correlated with long-term illness, it was positively correlated with ability to pay and health status. CHL was positively correlated with education, income, ability to pay for treatment, health status, and FHL level, whereas it was negatively correlated with long-term illness. Binary logistic regression showed that low education, low socioeconomic status, and being a widow were predictive of inadequate FHL. Moreover, having inadequate FHL and low income increased the likelihood of having inadequate CHL. CONCLUSION: This study has identified those with risk factors for inadequate health literacy and unfavorable health outcomes. A national action plan can guide the promotion of health literacy and its embeddedness in society to instill competencies and the environment that would eliminate health inequities and sustain health in Lebanon. [HLRP: Health Literacy Research and Practice. 2021;5(4):e295-e309.] Plain Language Summary: This study examined health literacy levels and correlates in 587 Lebanese adults using two recognized screening tools. The first tool measured functional health literacy (FHL), which represents the ability of a person to acquire information on health through reading or writing. The second tool assessed comprehensive health literacy (CHL), which encompasses the ability of a person to use their social skills to acquire health information from different media and make appropriate health decisions based on this information. Close to two-thirds of the participants had inadequate or problematic FHL. More specifically, low education, low socioeconomic status, and widowhood were predictive of inadequate FHL. Nearly one-half of the participants had inadequate or problematic CHL with an increased likelihood of inadequate levels in people with low FHL and low income.

Volumetric-Modulated Arc Radiotherapy with Daily Image-Guidance Carries Better Toxicity Profile for Higher Risk Prostate Cancer.

PURPOSE: To compare radiotherapy-induced toxicity for localized prostate-cancer (PCa) treated with versus without daily image-guidance. PATIENTS AND METHODS: We identified consecutive intermediate and high-risk localized PCa patients treated with definitive radiotherapy using intensity-modulated radiotherapy (IMRT) with variable duration of androgen-deprivation therapy (ADT) within 2015-2016 (Arm-A) and 2005-2007 (Arm-B). Arm-A cases received daily online imaging guidance (IGRT) using cone-beam computed tomography (CBCT) unlike Arm-B candidates with no daily IGRT. After reporting demographic, clinico-pathological features and treatment details, we compared acute (within 3 months post-therapy) and late RT-induced toxicities between study groups graded by RTOG/CTCAE criteria. Uni/multivariate analyses (UVA/MVA) were performed to identify independent predictors for RT-related side-effects. RESULTS: We were able to identify 257 cases who met our inclusion criteria. Overall, median age was 73 years (48-85), 67% had intermediate-risk and 47% received ADT. Arm-A included 72 patients who received IMRT delivered using volumetric-modulated arc therapy (VMAT), whereas, Arm-B was formed of 185 cases who utilized step-and-shoot static IMRT. Clinico-pathological features and treatment details were non-different across study arms except that Arm-A had more Grade Group 3, higher median total dose (79.2 vs. 74 Gy) and more pelvic lymph-nodes RT (p <0.05). Although acute toxicity was similar across groups, Arm-B encountered higher late toxicity score, more intense late genitourinary side-effects (P=0.008), with non-different late lower-gastrointestinal toxicities. On MVA, lack of daily CBCT, African-American race and higher comorbidities were independently predictive for late toxicities.  Conclusion: IMRT with daily CBCT permitted safe delivery of dose-escalated IMRT with improved toxicity profile for higher-risk prostate cancer.

Cough-induced rib fracture in a smoker: a case report.

BACKGROUND: Coughing is considered an important mechanism that helps the body get rid of foreign substances or prevent their entry into the tracheobronchial tree. Infrequently, after the onset of coughing, patients presenting with persistent chest pain are found to have rib fractures. Among the cases reported for cough-induced rib fractures, the maximum number of fractured ribs was found to be four. CASE PRESENTATION: In this report, we present a case of a healthy 50-year-old Lebanese smoker who developed a total of six fractures in five ribs, asymmetrically, after coughing for 2.5 months. CONCLUSION: This case report is, to our knowledge, the first to describe six cough-induced rib fractures in a smoker without an underlying predisposition.

20-Year trend of high prevalence of zero coronary artery calcium in beach cities of Southern California: A blue zone?

BACKGROUND: Blue zones are longevity hotspots around the world characterized by highest concentrations of healthy centenarians. Certified blue zone communities are designed by implementation of environmental and policy changes that promote healthy behaviors. OBJECTIVE: To examine the trends of prevalence of zero CAC, a marker of ideal cardiovascular and overall health status and burden of cardiovascular risk factors in Beach Cities/certified blue zones of Southern California and rest of California. METHODS: This is a population-based cohort study of persons aged 50 years or older in California, who underwent CAC screening between 2000 and 2019. A total of 3864 participants from Beach Cities of Southern California were identified by Zip Codes and compared with 35,537 participants from rest of California. We compared trends of prevalence of zero CAC and cardiovascular risk factors between the two groups, in 5-year intervals. RESULTS: Among 39,401 participants (mean age, 58.1 years; 36% women), 13,374 (34%) had zero CAC. The prevalence of CAC â€‹= â€‹0 was significantly higher in Beach Cities compared to the rest of California (p â€‹< â€‹0.001). Across the study period, the prevalence of cardiac risk factors including obesity, smoking, diabetes and hypertension remain significantly lower in Beach Cities. (p â€‹< â€‹0.001). CONCLUSIONS: This study, shows for the first time, that higher prevalence of zero CAC in Beach Cities of California, adds validity to excellent prognosis and longevity in these areas. The impact of policy implementation and environmental changes on lifestyle patterns, cardiovascular health and healthy ageing needs to be evaluated.

Prognostic Significance of Nodal Location and Ratio in Stage IIIC Endometrial Carcinoma Among a Multi-Institutional Academic Collaboration.

PURPOSE/OBJECTIVE(S): Stage IIIC endometrial carcinoma (EC) represents pathologically heterogenous patients with single/multiple pelvic (stage IIIC1) or paraaortic (stage IIIC2) lymph nodes (LNs). There is an increasing trend to offer adjuvant chemotherapy (CT) +/- radiation (RT) uniformly to these patients, regardless of substage. We investigate the prognostic significance of positive LN (pLN) number, ratio (%pLN), location (IIC1 vs. IIC2), and adjuvant treatment on patterns of failure and survival in a large collaborative multi-institutional series. MATERIALS AND METHODS: Clinical data for stage III EC patients such as patient characteristics, surgery/pathologic details, adjuvant therapies (including CT, RT, and chemotherapy and radiation), and outcomes (including pelvic control [PC], disease-free survival [DFS], distant DFS, and overall survival [OS]) were collected from 3 academic institutions. Log-rank analyses, Cox regression univariate and multivariate analyses were performed. RESULTS: Of the 264 patients queried for stage III disease, 237 (73%) had pLN, and complete LN sampling for analysis. The mean number of pLN in the combined data were 3.9, with 26.1% of all LN sampled positive; 121 patients (51%) staged IIIC1, and 116 patients (49%) staged IIIC2. There was a significant difference in number of pLN (P=0.0006) and total LN sampled by institution (range, 13 to 35; P=0.0004), without a difference in %pLN (P=0.35). Ninety-seven of 220 (44.1%) have ≥20% pLN. While controlling for substage and institution, a decrease in DFS (hazard ratio [HR], 1.1; P=0.007), and OS (HR, 1.1; P=0.01) was observed with every increase of 10% in the pLN ratio. There was a significant difference in DFS (HR, 1.8; P=0.003), PC (HR, 1.9; P=0.004), and distant DFS (HR, 1.6; P=0.03), as well as a trend for decreased OS (HR, 1.6; P=0.08) for substage IIIC2 versus IIIC1 disease; 5 years DFS 40% versus 45%, OS 50% versus 57%. Patients received no adjuvant therapy (10%), CT alone (27%), RT alone (16%), or chemotherapy and radiation (47%). There was no significant difference in PC, DFS, or OS between the various treatment regimens. On univariate analysis, while pLN was significant, treatment type did not impact DFS or OS. On multivariate analysis for DFS, patient age, race, and IIIC1 versus IIIC2 substage retained significance (HR, 0.56; P=0.01). CONCLUSIONS: Stage III EC patients with substage IIIC2 disease have a significantly increased risk of local and distant disease recurrence and death from EC. A decrease in DFS and OS was observed with every increase of 10% in the pLN ratio. Stage IIIC2 patients represent a high-risk subpopulation for whom clinical trials, or targeted regimens should be explored to achieve improved oncologic outcomes.

Primary isolated lymphoma of the fourth ventricle in an immunocompetent patient.

Primary central nervous lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin's lymphoma with a especially poor prognosis. The diagnosis is usually encountered in immunodeficient patients but is also encountered, albeit uncommonly, in the immunocompetent. We present a 50-year-old male who developed signs and symptoms of increased intracranial pressure. Imaging revealed the presence of a fourth ventricle mass with obstructive hydrocephalus. First, the patient underwent emergency endoscopic third ventriculostomy followed, few days later, by complete tumor resection via a posterior fossa craniotomy. Postoperative histopathology revealed the lesion to be a PCNSL. He received adjuvant chemotherapy and radiation and remained with no recurrence on regular imaging studies for 18-month followup. We report herein the fourth case of isolated PCNSL lesion to the fourth ventricle in the literature and provide the rationale for our belief that craniotomy and tumor resection, if feasible, should be the initial line of management in similar cases to relieve hydrocephalus and achieve the diagnosis.

Gastrointestinal basidiobolomycosis in a child; an unusual fungal infection mimicking fistulising Crohn's disease.

BACKGROUND: Gastrointestinal basidiobolomycosis is a rare disease caused by the fungus Basidiobolus ranarum. It has been reported in both children and adults. The disease mainly affects the colon and the small bowel; however, cases of the stomach, liver, pancreas, and renal system being affected have been reported. CASE REPORT: A 2 year old boy presented with the following symptoms; abdominal pain, vomiting, diarrhea, fever and palpable right iliac fossa mass. Laboratory investigations revealed elevated inflammatory markers and peripheral eosinophilia. Colonoscopy showed severely inflamed mucosa of the terminal ileum, cecum and ascending colon. CT scan of the abdomen demonstrated an inflammatory mass with wall thickening of the terminal ileum and the colon. Surgical exploration demonstrated retroperitoneal mass and inflamed terminal ileum, cecum and ascending colon. Upon laparotomy, multiple internal fistulas involving the bowel loops, the urinary bladder, the right ureter and the gallbladder were observed. Further investigations using histopathology of the resected diseased bowel showed extensive necrosis, multinucleated giant cells and numerous eosinophils and large fungal hyphae surrounded by strongly eosinophilic material were seen in the tissue suggestive of B. ranarum infection. The patient responded well to treatment with voriconazole. CONCLUSION: Gastrointestinal basidiobolomycosis should be considered in the differential diagnosis of every child presenting with abdominal pain, fever and palpable abdominal mass with peripheral eosinophilia. The presence of bowel inflammation and fistulas should not preclude such diagnosis.

Solitary rectal ulcer syndrome presenting as polypoid mass lesions in a young girl.

Solitary rectal ulcer syndrome (SRUS) is a rare condition in children. We report a case of SRUS in an 8-year old Saudi girl who presented with recurrent rectal bleeding, intermittent mucosal prolapse, and passage of mucus per rectum. Colonoscopy revealed multiple polypoid mass lesions with histopathological features of SRUS. The polypoid variant of SRUS is very rare in children and may be confused with rectal malignant or inflammatory conditions.