Background Our laboratory published the first evidence that nutritional ketosis, induced by ketogenic diet (KD) or time-restricted diet (TRD), ameliorates disease progression in PKD animal models. We reasoned that, due to their frequent use for numerous health benefits, some ADPKD patients may already have had experience with ketogenic dietary interventions (KDIs). This retrospective case series study is designed to collect first real-life observations of ADPKD patients about safety, feasibility, and possible benefits of KDIs in ADPKD as part of a translational project pipeline. Methods Patients with ADPKD who had already used KDIs were recruited to retrospectively collect observational and medical data about beneficial or adverse effects, the feasibility and safety of KDIs in questionnaire-based interviews. Results 131 ADPKD patients took part in this study. 74 executed a KD and 52 TRD, for 6 months on average. 86% of participants reported that KDIs had improved their overall health. 67% described improvements in ADPKD-associated health issues. 90% observed significant weight loss. 64% of participants with hypertension reported improvements in blood pressure. 66% noticed adverse effects that are frequently observed with KDIs. 22 participants reported safety concerns like hyperlipidemia. 45 participants reported slight improvements in eGFR. 92% experienced KDIs as feasible while 53% reported breaks during their diet. Discussion Our preliminary data indicate that KDIs may be safe, feasible, and potentially beneficial for ADPKD patients highlighting that prospective clinical trials are warranted to confirm these results in a controlled setting and elucidate the impact of KDIs specifically on kidney function and cyst progression.
Acalvaria is a rare cranial vault defect characterized by the presence of the cerebellum and cerebral cortex with the absence of the calvarium above the orbits, intact facial structures, and the presence of dura mater. Unfortunately, this diagnosis comes with a dim prognosis that is not compatible with life long-term. Firsttrimester diagnosis with ultrasonography can establish the diagnosis. If imaging is equivocal, advanced imaging with fetal MRI has a role to aid in distinguishing between similar cranial vault defects that fall on the spectrum including anencephaly, exencephaly, and acrania. We present the case of a term male infant with known acalvaria diagnosed incidentally on prenatal ultrasound that was delivered by cesarean section to a G3P3 mother at the time of delivery with two prior uncomplicated pregnancies. Maternal history was rather insignificant except for gestational diabetes and gestational hypertension well-controlled without medication. After initiation of comfort measures only, the infant expired on day of life two.
Issue: Medical education traditionally focuses on basic science during the first two years of medical school. To “flip” this model, the Penn State College of Medicine has introduced and inquiry-based educational strategy that introduces students to the challenges of patient care immediately upon their arrival. To engage students in a process to promote clinical reasoning, we have modified an Analytic Decision Game (ADG) called “EpiCentre” to address a notional public health crisis facing Centre County, Pennsylvania. Methods: In phase 1 of the activity, students are provided with materials describing the ethnography and infrastructure of Centre County. Students are divided into three communities (teams) to create a strength, weakness, threat, opportunity (SWOT) analysis of local healthcare capabilities. In phase 2 of the activity, students meet with a standardized patient presenting with a targeted medical complaint. They are pushed to think about their approach to taking a medical history and asked to generate a differential diagnosis. In phases 3 and 4, students are faced with the challenge of triaging a number of patients with similar medical complaints and create a plan to deal with a likely outbreak scenario. Findings: Students have found the EpiCentre activity to be worthwhile in multiple contexts. They have been able to develop an initial approach to medical history taking and creating a differential diagnosis. They have formulated an approach to the recognition and control of a potential public health crisis. An additional benefit of the exercise has been the overarching theme of teamwork. Students begin the activity (occurring in the first few weeks after arrival to medical school) as relative strangers and quickly develop a sense of camaraderie and mission focus. Conclusions: The EpiCentre ADG has been a successful activity to introduce medical students to Centre County in the context of healthcare infrastructure, an approach to medical history taking, disaster planning, clinical reasoning and team-building. Implications: EpiCentre derives from an interprofessional collaboration between the College of Medicine and the College of Information Sciences and Technology. It represents one of potentially limitless opportunities to engage students and faculty from multiple disciplines to address challenges of public health within the academic setting.
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