Albert Einstein College of Medicine, Yeshiva University: Open Journal Systems
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    To Sam, With Love

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    Perceptions of an Implantable Cardioverter-Defibrillator: A Qualitative Study of Families with a History of Sudden, Life-Threatening Cardiac Events, and Recommendations to Improve Care

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    Objective: To identify major concerns associated with implantable cardioverter-defibrillators (ICDs) and to pro- vide recommendations to adult and pediatric physicians involved in the care of patients with ICDs. Background: Cardiac ion channelopathies are a well-recognized cause of sudden cardiac death in infants, children, adolescents, and young adults. ICDs are effective in preventing sudden death from fatal arrhythmias in patients with known cardiac channelopathies. There is a paucity of research on the effect of ICDs on quality of life in patients with cardiac channelopathy diagnoses, especially young patients. Methods: A qualitative study interviewing patients and families affected by inherited arrhythmias was conducted. Fifty participants with personal or family histories of cardiac events or sudden death were interviewed individually or in focus groups by clinical psychologists. All interviews were transcribed verbatim and then analyzed and coded based on current qualitative research theory to identify themes related to the research question. Twenty-four participants discussed ICDs in their interviews. Results: Participants reported concerns about ICDs, and these concerns were categorized into six themes: (1) comprehension and physician-patient communication; (2) anxiety; (3) restrictions and fallacies; (4) complications; (5) utility; and (6) alternative therapy. Participants noted communication breakdowns between providers and their colleagues, and between providers and their patients. Participants and their families experienced many different forms of anxiety, including worry about the aesthetics of the ICDs and fears of being shocked. Multiple restrictions, fallacies, and complications were also cited. Conclusion: Interview themes were used to formulate recommendations for counseling and educating patients with ICDs.

    The Metamorphosis of a Horse into a Zebra: A Case of Primary Eosinophilic Gastroenteritis

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    Chronic diarrhea is a common diagnostic entity faced by many primary care physicians. Primary eosinophilic gastroenteritis (PEG), a relatively rare but not uncommon cause of chronic nonbloody diarrhea, presents with nonspecific symptoms, making clinical consideration and diagnosis extremely challenging. In PEG, eosinophils selectively target the gastrointestinal tract, where they degranulate, causing inflammation and irritation. We report the case of a 46-year-old female with recurrent hospitalizations for nausea, vomiting, and diarrhea over a nine-month period. After an extensive workup ruling out secondary causes of eosinophilia, she was diagnosed with PEG.

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    Dominick P. Purpura – An Appreciation

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    A New Life

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    The Pathogenesis of HIV-Associated Dementia: Recent Advances Using a SCID Mouse Model of HIV-Encephalitis

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    In the era of highly active antiretroviral therapy (HAART) there has been a decrease in AIDS death rates and a reduction in HIV-related neurological complications. However, Human Immunodeficiency Virus-1 (HIV)-associated dementia (HAD) still affects at least 7% of HIV-positive individuals. Despite significant advances in HIV research there are still questions surrounding the pathogenesis of HAD. Clinical information and pathological studies suggest that the frontal cortex, basal ganglia, and hippocampus are important neuroanatomical areas involved in HAD. Recent studies utilizing a severe combined immunodeficiency (SCID) mouse model of HIV-encephalitis (HIVE) indicate that central nervous system (CNS) viral load determines the severity of astrogliosis, an important feature of HIVE. Human and animal studies suggest that viral strains may also be important in the pathogenesis of HAD. A recent study suggests that HAART does not eradicate virus in the brain, and therefore, the CNS is a reservoir for HIV. Future research efforts need to focus on the role of viral strain and mutations in the pathogenesis of HAD.

    Homage to Gaia: The Life of an Independent Scientist

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    Congenital Melanocytic Nevi and the Risk of Malignant Melanoma: Establishing a Guideline for Primary-Care Physicians

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    Objective: The objective of this review is to determine what size congenital melanocytic nevi (CMN) increases the risk of malignant melanoma in affected patients.Background: Congenital melanocytic nevi are benign proliferations of cutaneous melanocytes apparent at birth or in the first postnatal weeks. The Kopf system classifies nevi based on size: small, <1.5 cm in diameter; medium, 1.5–19.9 cm in diameter, and large, ≥20 cm in diameter. Great variability exists in quantifying the risk of malignant transformation from congenital nevi of different sizes. Evidence-based standard guidelines for clinical investigation need to be established.  Methods: Literature search included studies on medium, large, and giant congenital nevi in association with melanoma.Results: Three studies pertaining to small, medium, and large congenital nevi are defined. The odds ratio of malignant transformation from small CMN was determined to be 20.9 by history and 10.5 by histology in 238 patients in the case-control study selected. No malignant transformation was found in a prospective study of 230 individuals with medium-sized melanocytic nevi. Finally, a 5% risk of malignant transformation was reported in a prospective study of patients with large congenital nevi.Conclusion: All patients should receive total body skin and mucosal surface exams. Patients with small CMN (<1.5 cm in diameter) and medium CMN (≥1.5 cm–19.9 cm in diameter) should be closely observed over their lifetimes and given the option of specialist referral. Finally, patients with large CMN (≥20 cm in diameter) should be referred to specialists upon initial presentation. More original data and follow-up are needed for maturation of evidence-based clinical recommendations

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