Investigating and Treating a Corneal Ulcer Due to Extensively Drug-Resistant Pseudomonas aeruginosa.

Resistant Gram-negative bacteria are a growing concern in the United States, leading to significant morbidity and mortality. We identified a 72-year-old female patient who presented with unilateral vision loss. She was found to have a large corneal ulcer with hypopyon. Culture of corneal scrapings grew extensively drug-resistant Pseudomonas aeruginosa. Treatment involved a combination of systemic and topical antibiotics. Whole genome sequencing revealed the presence of blaVIM-80, blaGES-9, and other resistance determinants. This distinctive organism was linked to an over-the-counter artificial tears product.

Pseudomonas aeruginosa Corneal Ulcer Associated With Artificial Tears Eye Drops.

This case report describes vision loss in a 72-year-old woman following use of EzriCare eye drops.

Effect of Enteral Guanfacine on Dexmedetomidine Use in the ICU.

Describe the efficacy and safety of guanfacine for dexmedetomidine weaning in critically ill patients. DESIGN: Retrospective descriptive analysis. SETTING: Six hundred thirteen-bed academic medical center from October 2020 to October 2021. PATIENT/SUBJECTS: All Adult patients on IV dexmedetomidine who received at least one dose of guanfacine for sedation or agitation were included. INTERVENTIONS: Enteral guanfacine. MEASUREMENTS AND MAIN RESULTS: The primary outcome was discontinuation of dexmedetomidine therapy within 48 hours after guanfacine initiation. Secondary outcomes assessed included adjunctive medication use, rate of dexmedetomidine reinitiation, and safety outcomes. One hundred five patients were included in the analysis. Median age was 59 years old, 66% were male, and median daily dose of guanfacine was 1.5 mg. Dexmedetomidine was discontinued within 48 hours in 58% of patients (n = 61) and within 72 hours in 71% of patients (n = 75). Fifty-five percent of patients (n = 58) required rescue medications for poorly controlled agitation, sedation, or pain while on guanfacine. Dexmedetomidine withdrawal occurred in 2% of patients (n = 2) while on guanfacine. Adverse effects attributed to guanfacine occurred in 8% of patients (n = 8), all experiencing hypotension leading to medication discontinuation. CONCLUSION: Dexmedetomidine was successfully weaned within 48 hours of guanfacine initiation in 58% of patients with minimal withdrawal or adverse effects. Guanfacine may be an effective and safe enteral option for dexmedetomidine weaning in critically ill patients.

Content, Readability, and Accountability of Freely Available Online Information for Patients Regarding Epiretinal Membranes.

PURPOSE: To assess content, readability, and accountability of online information for patients regarding epiretinal membranes (ERMs). METHODS: Cross-sectional study evaluating nine major medical websites on ERMs. Fifteen questions assessed patient-relevant content. Four indices estimated U.S. grade literacy level of the text. JAMA benchmarks (authorship, attribution, disclosure, currency) evaluated website accountability. RESULTS: Average content score was 36.78 (SD 13.91, 95% CI ±0.64) from a possible maximum of 60, with significant variability between websites (H = 22.68, p=0.004). Mean reading grade level was 12.29 (SD 2.30, 95% CI ±1.50). No website achieved all JAMA benchmarks; only one website fulfilled three of the four. Content score did not correlate with Google rank (order of listed websites, r = -0.23, p=0.55) or JAMA benchmarks (r = 0.19, p=0.62) but significantly correlated with mean reading grade (r = 0.67, p=0.05). CONCLUSION: Online information regarding ERMs varies significantly, may not adequately answer common patient questions, and is written at too complex a literacy level for the average patient.

Assessing the Accuracy, Quality, and Readability of Patient Accessible Online Resources Regarding Ocular Gene Therapy and Voretigene Neparvovec.

PURPOSE: To evaluate the accuracy, quality, and readability of online information regarding the Food and Drug Administration (FDA) approved ocular gene therapy voretigene neparvovec (Luxturna, Spark Therapeutics, Philadelphia, PA, USA). METHODS: Ten online resources about voretigene neparvovec were assessed in this cross-sectional study. A novel 25-question assessment was created to evaluate the information most relevant to patients. Each article was assessed by independent graders using the assessment and the DISCERN instrument. An online readability tool, Readable, was used to assess readability. Accountability was evaluated using the Journal of the American Medical Association (JAMA) benchmarks. RESULTS: The average questionnaire score for all the articles was 33.93 (SD 11.21, CI 95% ±6.95) out of 100 possible points with significant variation in the content accuracy and quality between the articles (P=0.017). EyeWiki achieved the highest score and MedicineNet the lowest. The mean reading grade for all articles was 12.88 (SD 1.93, CI 95% ±1.19) with significant variation between articles (P=0.001). Wikipedia was the most readable, and the FDA website was the least. None of the articles achieved all four JAMA benchmarks, and only one of the ten articles, EyeWiki, achieved three of the four JAMA benchmarks. CONCLUSION: The information available online regarding this FDA-approved ocular gene therapy is generally of low quality, above the average reading level of the general population, and varies significantly between sources. The articles provide incomplete information that is not entirely accurate or easy to read, and as a result, the material would not support patients adequately in their medical decisions and questions about this new therapeutic option.

Demographics of Surgical Specialty Residency Program Directors in the United States: A Cross-sectional Analysis.

OBJECTIVE: To evaluate program director (PD) demographics, training background, and academic productivity in 11 surgical specialties. SUMMARY BACKGROUND DATA: There is currently no comprehensive study comparing educational background, research output, and gender differences between PDs of surgical residencies in the United States. METHODS: The Accreditation Council for Graduate Medical Education (ACGME) and the Association of American Medical Colleges (AAMC) websites were used to identify residency PDs. Age, information related to service as PD, educational background, and research output were collected utilizing online searches including Doximity, PubMed, and Scopus.. The ACGME Data Resource Book was used to obtain data on the gender makeup of residents in each surgical specialty. Data collection occurred between December 14, 2019, and May 9, 2020. RESULTS: 1571 residency PDs across 11 surgical specialties were included. Significant differences between specialties were found with respect to PD gender, current age, age at appointment, years between residency and assignment, term duration, number of PubMed publications, and Scopus h-index. The current age (mean ± SD) ranged from 46.8 ± 8.5 years among Interventional Radiology (IR) PDs to 53.4 ± 9.1 years among Neurological Surgery (NEUROSURG) PDs. The proportion of female PDs ranged from 5.9% in NEUROSURG to 63.5% in Obstetrics and Gynecology (OB-GYN). Completion of a post-residency fellowship was least common for OB-GYN PDs at 9.1%, and most common for IR PDs at 98.8%. The number (mean ± SD) of PubMed publications and Scopus h-index ranged from 13.1 ± 22.3 publications and h index 4.5 ± 5.7 among OB-GYN PDs to 112.5 ± 103.0 publications and h index 27.4 ± 16.7 among Thoracic Surgery PDs. Age and academic productivity as measured by PubMed publications and Scopus h-index were significantly lower among female PDs in multiple surgical specialties. CONCLUSIONS: There were significant variations in the PDs of surgical specialties, particularly with respect to gender and academic productivity. Efforts should be made to support and encourage greater female representation in the role of surgical residency PD.

Assessing the Quality, Content, and Readability of Freely Available Online Information for Patients Regarding Age-Related Macular Degeneration.

IMPORTANCE: One of the top ten causes of disability in the United States is vision loss, primarily due to age-related eye diseases such as age-related macular degeneration. With an aging population, the number of people affected by this condition is expected to rise. Patients increasingly turn to the internet for health-related information, but no standard exists across published websites. OBJECTIVE: To assess the quality, content, accountability and readability of information found online for age-related macular degeneration. DESIGN: This cross-sectional study analyzed 12 freely available medical sites with information on age-related macular degeneration and used PubMed as a gold standard for comparison. Thirty-four questions were composed to include information most relevant to patients and each website was independently evaluated by one vitreoretinal surgeon, two vitreoretinal fellows and one ophthalmology resident. Readability was analyzed using an online readability tool. The JAMA benchmarks were used to evaluate the accountability of each site. SETTING: Freely available online information was used in this study. RESULTS: The average questionnaire score for all websites was 90.23 (SD 17.56, CI 95% ±9.55) out of 136 possible points. There was a significant difference between the content quality of the websites (P = .01). The mean reading grade for all websites was 11.44 (SD 1.75, CI 95% ±0.99). No significant correlation was found between content accuracy and the mean reading grade or Google rank (r = 0.392, P = .207 and r = 0.133, P = .732, respectively). Without including PubMed, only one website achieved the full 4 JAMA benchmarks. There was no correlation between the accuracy of the content of the website and JAMA benchmarks (r = 0.344, P = .273). The interobserver reproducibility was similar among 3 out of 4 observers (r = 0.747 between JS and NT, r = 0.643 between JS and NP, r = 0.686 between NP and NT, r = 0.581 between JS and NY; P ≤ 0.05). CONCLUSION AND RELEVANCE: The freely available information online on age-related macular degeneration varies by source but is generally of low quality. The material presented is difficult to interpret and exceeds the recommended reading level for health information. Most websites reviewed did not provide sufficient information using the grading scheme we used to support the patient in making medical decisions.

Evaluation of the Content, Quality, and Readability of Patient Accessible Online Resources Regarding Cataracts.

PURPOSE: To evaluate the content quality, accuracy, and readability of commonly visited websites by cataract patients contemplating cataract surgery. SETTING: Freely available online information. DESIGN: Cross-sectional study. METHODS: Ten websites were evaluated in a cross-sectional study for content analysis using a grading sheet of 40 questions individually scored by three ophthalmologists. JAMA benchmarks were used to assess the quality. An online readability tool, Readable, was used to assess the readability. RESULTS: There was a significant difference between the content and accuracy of each website according to a Kruskal-Wallis test (H = 22.623, P = .007). The average score for all websites using the grading sheet was 90.85 out of 160 points, or 57% (SD 29.93, CI 95%±17.69). There was no significant correlation between website rank on Google.com and content quality of the website (r = 0.049, P = .894). No websites complied with all 4 JAMA criteria for authorship. There was no significant correlation between content quality of each website and number of JAMA requirements met (r = -0.563, P = .09). The average Flesch Reading Ease Score for all websites was 52.64 (SD 11.94, CI 95%±7.40), and the average Mean Reading Grade was 10.72 (SD 1.58, CI 95%±0.98). There was a significant difference in Mean Reading Grades between websites (H = 23.703, P = .005). There was no significant correlation between content quality of the website and Mean Reading Grade (r = -0.552, P = .098). CONCLUSION: Commonly accessed online resources on cataracts and cataract surgery are insufficient to provide patients with a clear and complete understanding of their condition as well as available medical and surgical treatment options.

Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma.

SUMMARY: We report the case of a 76-year-old male with a remote history of papillary thyroid cancer who developed severe paroxysmal headaches in the setting of episodic hypertension. Brain imaging revealed multiple lesions, initially of inconclusive etiology, but suspicious for metastatic foci. A search for the primary malignancy revealed an adrenal tumor, and biochemical testing confirmed the diagnosis of a norepinephrine-secreting pheochromocytoma. Serial imaging demonstrated multiple cerebral infarctions of varying ages, evidence of vessel narrowing and irregularities in the anterior and posterior circulations, and hypoperfusion in watershed areas. An exhaustive work-up for other etiologies of stroke including thromboembolic causes or vasculitis was unremarkable. There was resolution of symptoms, absence of new infarctions, and improvement in vessel caliber after adequate alpha-adrenergic receptor blockade for the management of pheochromocytoma. This clinicoradiologic constellation of findings suggested that the etiology of the multiple infarctions was reversible cerebral vasoconstriction syndrome (RCVS). Pheochromocytoma remains a poorly recognized cause of RCVS. Unexplained multifocal cerebral infarctions in the setting of severe hypertension should prompt the consideration of a vasoactive tumor as the driver of cerebrovascular dysfunction. A missed or delayed diagnosis has the potential for serious neurologic morbidity for an otherwise treatable condition. LEARNING POINTS: The constellation of multifocal watershed cerebral infarctions of uncertain etiology in a patient with malignant hypertension should trigger the consideration of undiagnosed catecholamine secreting tumors, such as pheochromocytomas and paragangliomas. Reversible cerebral vasoconstriction syndrome is a serious but reversible cerebrovascular manifestation of pheochromocytomas that may lead to strokes (ischemic and hemorrhagic), seizures, and cerebral edema. Alpha-adrenergic receptor blockade can reverse cerebral vasoconstriction and prevent further cerebral ischemia and infarctions. Early diagnosis of catecholamine secreting tumors has the potential for reducing neurologic morbidity and mortality in patients presenting with cerebrovascular complications.

Pars Plana Vitrectomy Reoperations for Complications of Proliferative Diabetic Retinopathy.

OBJECTIVE: To report visual acuity in patients undergoing pars plana vitrectomy (PPV) reoperations for complications of proliferative diabetic retinopathy (PDR). DESIGN: Retrospective case series. SUBJECTS: Diabetic patients undergoing reoperation with PPV between 2015 and 2018 at a university referral center. METHODS: Patient charts were reviewed for indication for initial and repeat PPV, baseline clinical characteristics including gender, age, and lens status, and pre- and post-operative best-corrected visual acuity. MAIN OUTCOME MEASURES: Best-corrected visual acuity at last follow-up. RESULTS: Of 538 eyes (409 patients) undergoing a PPV for diabetic retinopathy, 153 (28.4%) eyes had reoperation. Among the 130 eyes (119 patients) that met the inclusion criteria, 55 eyes (50 patients) underwent reoperation for complications of PDR, defined as non-clearing vitreous hemorrhage (NCVH) and/or tractional retinal detachment (TRD). Within this subgroup of 55 eyes, 19 (34.5%) eyes had an indication for the first surgery of NCVH. Fourteen (73.7%) of these NCVH eyes achieved a visual acuity of 20/80 or better. When the indication for the first surgery was TRD (33 eyes, 60%), 8 (24.2%) eyes achieved this same outcome (p=0.0011). CONCLUSION: Approximately one of every four eyes treated with PPV for PDR will undergo repeat PPV during follow-up. VA outcomes after the repeat PPV were variable, with NCVH cases achieving better outcomes compared to TRD.

Residency Program Directors of United States Ophthalmology Programs: A Descriptive Analysis.

PURPOSE: To analyze the academic background, scholarly achievements, and demographic characteristics of all US ophthalmology residency program directors (PDs). DESIGN: Cross-sectional study. METHODS: Online search of publicly available resources conducted from February 16, 2019, to February 26, 2019. STUDY POPULATION: 116 ophthalmology residency PDs. Main outcome measurements were demographic and academic data. RESULTS: A total of 116 program directors were analyzed. Eighty-four of 116 (72%) PDs were male. The average age was 50.0 years old. The mean age at appointment was 42.9 years old. Ninety-three percent graduated from an American medical school, and 97% received an MD degree. Twenty percent of PDs completed an additional graduate degree, most commonly a master's degree (7 of 23) and doctor of philosophy (7 of 23). Seventy-eight percent completed a fellowship, with the most frequent in glaucoma (24%), cornea and external diseases (22%), and neuroophthalmology (21%). The mean number of publications according to PubMed was 17.6 (range, 0-92). There were no significant differences between the average number of publications by male PDs and those by female PDs (19.2 ± 20.5 vs. 13.5 ± 23.1, respectively; P = 0.21). On average, the H-index was 8.7 (range, 0-35) and was higher in male than in female PDs (9.8 ± 8.3 vs. 5.4 ± 4.0, respectively; P = 0.01). CONCLUSIONS: Ophthalmology PDs are predominantly male with fellowship training in glaucoma, cornea, or neuro-ophthalmology. Women remain underrepresented, and future efforts should be aimed at addressing this disparity.

Association Between Early Sjögren Markers and Symptoms and Signs of Dry Eye.

PURPOSE: Animal models suggest that early markers of Sjögren syndrome (EMS)-antibodies against salivary protein 1, parotid secretory protein, and carbonic anhydrase 6 (CA6)-are more accurate signals of early Sjögren when compared with classic markers (anti-Ro and anti-La). To further understand the relationship between EMS and dry eye (DE), we compared symptoms and signs of DE in subjects who tested positive versus negative for EMS. METHODS: In this cross-sectional study, patients at the Miami Veterans Affairs Eye Clinic who were tested for EMS underwent a standard ocular surface examination. Indications for EMS testing included DE symptoms in combination with dry mouth symptoms, low tear production, corneal staining, or a Sjögren disease-associated autoimmune disease. Statistical tests performed were the χ test, Fisher exact test, independent sample t test, and Spearman correlation. RESULTS: Seventy-three percent of 44 patients tested positive for 1 or more EMS. CA6 IgG was most frequently elevated, followed by CA6 IgM and parotid secretory protein IgG. EMS-positive versus EMS-negative subjects were more likely to escalate DE treatment past artificial tears to topical cyclosporine (n = 32, 100% vs. n = 9, 75%, P = 0.02). There were no demographic or comorbidity differences between EMS-positive and EMS-negative subjects, and marker levels did not correlate with more severe tear film measures. CONCLUSIONS: Most of the individuals with DE tested positive for 1 or more EMS antibodies, including men and Hispanics. Future studies will be needed to understand how to incorporate EMS data into the care of an individual with DE.

Diagnostic tests in dry eye.

INTRODUCTION: Dry Eye (DE) is a multifactorial condition with a variable clinical presentation. This highly prevalent disease has multiple symptoms and signs that often do not correlate with one another. As such, the diagnosis of DE can be challenging to make, and a systematic approach must be taken. AREAS COVERED: We review the different methods commonly utilized to evaluate a patient complaining of DE symptoms. Included in this review are clinical examination techniques, point of care tests, and imaging techniques. EXPERT OPINION: DE is an umbrella term that encompasses different etiologies and pathophysiological mechanisms. The current definition recognizes tear instability, high osmolarity, inflammation, and neuro-sensory dysfunction as causative entities. The approach to DE begins with a systematic assessment of symptoms and signs, evaluating for both nociceptive and neuropathic sources of symptoms. Future research is needed to develop tests that assess neurosensory status in DE and couple point of care tests with therapeutic algorithms.

Assessment of the Quality, Content, and Readability of Freely Available Online Information for Patients Regarding Diabetic Retinopathy.

IMPORTANCE: Diabetic retinopathy is a global leading cause of blindness. Patients increasingly use the internet to search for health-related information that may affect medical decision-making, but to date, no standard exists across published websites. OBJECTIVE: To assess the quality, content, and readability of information found online for diabetic retinopathy. DESIGN AND SETTING: This cross-sectional study analyzed 11 medical sites with information on diabetic retinopathy. Twenty-six questions were composed to include information most relevant to patients, and each website was independently evaluated by 1 vitreoretinal surgeon and 2 vitreoretinal fellows. Readability was analyzed using an online readability tool. The JAMA benchmarks were used to evaluate the quality of each site. Data were collected from December 2018 to January 2019 and analyzed in February 2019. MAIN OUTCOMES AND MEASURES: A 26-question survey, JAMA benchmarks, Flesch reading ease score, Flesch-Kincaid grade level, Gunning Fog Index, Coleman Liau Index, and Simple Measure of Gobbledygook Index. RESULTS: The mean (SD) questionnaire score for all websites was 55.76 (13.38) (95% CI, 47.85-63.67) of 104 possible points. There was a difference between the content quality of the websites (H = 25.811, P = .004). The mean (SD) reading grade for all websites was 11.30 (1.79; 95% CI, 10.24-12.36). No correlation was found between content accuracy and the mean reading grade (r = 0.445, P = .17) or Google rank (r = -0.260, P = .43). No website achieved the full 4 JAMA benchmarks, and only 1 website achieved 3 of the 4 JAMA benchmarks. No correlation was found between the accuracy of the content of the website and JAMA benchmarks (r = 0.422, P = .20). The interobserver reproducibility was similar among the 3 observers (r = 0.87 between observers 1 and 2, r = 0.83 between observers 1 and 3, and r = 0.84 between observers 2 and 3, P < .001). CONCLUSIONS AND RELEVANCE: These findings suggest that freely available information online about diabetic retinopathy varies by source but is generally of low quality. The material presented seems difficult to interpret and exceeds the recommended reading level for health information. Most websites reviewed did not provide sufficient information using the grading scheme used to support the patient in making medical decisions.

Palliative Care Opportunities Among Adults With Congenital Heart Disease-A Systematic Review.

CONTEXT: Little is known about advance care planning (ACP) and palliative care needs among adults with congenital heart disease (ACHD). OBJECTIVES: The objective of this study was to identify and synthesize studies concerning palliative care among ACHD patients. METHODS: We searched five electronic databases (PubMed, Embase, SCOPUS, Web of Science, and CINAHL) using the keywords palliative care and congenital heart disease. Inclusion criteria were adults (age > 18 years) with congenital heart disease and publications in English through March 3, 2019. RESULTS: Our search yielded 2872 studies, and after removal of duplicates, we screened 2319 abstracts and identified seven for inclusion. Study findings were grouped into three domains: ACP, symptomatology, and end-of-life care. Among the five cross-sectional studies, only 1%-28% of ACHD patients recalled participating in ACP discussions with their doctors but 69%-78% reported a strong interest and desire to participate in ACP. In one study, 46% (n = 67) of patients had elevated anxiety symptoms (Hospital Anxiety and Depression Scale [HADS-A] ≥ 8) and 11% (n = 15) had elevated depressive symptoms (HADS-A ≥ 8). ACHD patients who had a documented goals of care conversation before cardiac decompensation had a lower incidence of resuscitation and aggressive treatments at end of life (12% [n = 3] vs. 100% [n = 12], P < 0.001). CONCLUSION: While few ACHD patients complete advance directives, our findings support that many ACHD patients recognize the value of initiating end-of-life and goals of care conversations early on in the course of illness. Future studies investigating communication and implementation strategies of ACP as well as the symptom experience of patients with ACHD are needed.

Osteopontin deficiency ameliorates Alport pathology by preventing tubular metabolic deficits.

Alport syndrome is a rare hereditary renal disorder with no etiologic therapy. We found that osteopontin (OPN) is highly expressed in the renal tubules of the Alport mouse and plays a causative pathological role. OPN genetic deletion ameliorated albuminuria, hypertension, tubulointerstitial proliferation, renal apoptosis, and hearing and visual deficits in the Alport mouse. In Alport renal tubules we found extensive cholesterol accumulation and increased protein expression of dynamin-3 (DNM3) and LDL receptor (LDLR) in addition to dysmorphic mitochondria with defective bioenergetics. Increased pathological cholesterol influx was confirmed by a remarkably increased uptake of injected DiI-LDL cholesterol by Alport renal tubules, and by the improved lifespan of the Alport mice when crossed with the Ldlr-/- mice with defective cholesterol influx. Moreover, OPN-deficient Alport mice demonstrated significant reduction of DNM3 and LDLR expression. In human renal epithelial cells, overexpressing DNM3 resulted in elevated LDLR protein expression and defective mitochondrial respiration. Our results suggest a potentially new pathway in Alport pathology where tubular OPN causes DNM3- and LDLR-mediated enhanced cholesterol influx and impaired mitochondrial respiration.