Alagille Syndrome: A case report of a rare oral and multisystem manifestation

Alagille syndrome is an inherited multisystem disorder of autosomal dominant transmission. Its prevalence is estimated at 1 per 70,000 to 100,000 live births. We report the case of a young patient suffering from Alagille syndrome who consulted the center of diagnosis and dental treatment of Rabat - MOROCCO (CCTD). The general manifestations are facial dysmorphia, hepatic, cardiac, and ocular disorders. Hepatic cholestasis causes oral repercussions such as a yellow oral mucosa, hypomineralization of the teeth, and a high tendency to dental caries. The management of such a patient requires the knowledge of the general health of the patient, therefore collaboration with the attending physicians, the establishment of rigorous oral hygiene, personalized prophylaxis with a consequent contribution of fluorine.

Semantic competency as a marker of clinical reasoning performance

Background: This study sought to explore the relationship between semantic competence (or dyscompetence) displayed during “think-alouds” performed by resident and attending physicians and clinical reasoning performance. Methods: Internal medicine resident physicians and practicing internists participated in think-alouds performed after watching videos of typical presentations of common diseases in internal medicine. The think-alouds were evaluated for the presence of semantic competence and dyscompetence and these results were correlated with clinical reasoning performance. Results: We found that the length of think-aloud was negatively correlated with clinical reasoning performance. Beyond this finding, however, we did not find any other significant correlations between semantic competence or dyscompetence and clinical reasoning performance. Conclusions: While this study did not produce the previously hypothesized findings of correlation between semantic competence and clinical reasoning performance, we discuss the possible implications and areas of future study regarding the relationship between semantic competency and clinical reasoning performance.

Multi-source feedback following simulated resuscitation scenarios: a qualitative study

Background: The direct observation and assessment of learners’ resuscitation skills by an attending physician is challenging due to the unpredictable and time-sensitive nature of these events. Multisource feedback (MSF) may address this challenge and improve the quality of assessments provided to learners. We aimed to describe the similarities and differences in the assessment rationale of attending physicians, registered nurses, and resident peers in the context of a simulation-based resuscitation curriculum. Methods: We conducted a qualitative content analysis of narrative MSF of medical residents in their first postgraduate year of training who were participating in a simulation-based resuscitation course at two Canadian institutions. Assessments included an entrustment score and narrative comments from attending physicians, registered nurses, and resident peers in addition to self-assessment. Narrative comments were transcribed and analyzed thematically using a constant comparative method. Results: All 87 residents (100%) participating in the 2017-2018 course provided consent. A total of 223 assessments were included in our analysis. Four themes emerged from the narrative data: 1) Communication, 2) Leadership, 3) Demeanor, and 4) Medical Expert. Relative to other assessor groups, feedback from nurses focused on patient-centred care and communication while attending physicians focused on the medical expert theme. Peer feedback was the most positive. Self-assessments included comments within each of the four themes. Conclusions: In the context of a simulation-based resuscitation curriculum, MSF provided learners with different perspectives in their narrative assessment rationale and may offer a more holistic assessment of resuscitation skills within a competency-based medical education (CBME) program of assessment.

Factors that Influence End-Of-Life Decision Making Amongst Attending Physicians

Background: Medical advances prolong life and treat illness but many patients have chronically debilitating conditions that prevent them from making end-of-life (EOL) decisions for themselves. These situations are difficult to navigate for both patient and physician. This study investigates physicians’ feelings and approach toward EOL care, physician-assisted suicide (PAS), and euthanasia. Methods: An anonymous, self-administered online survey was distributed through the New Jersey Medical School servers and American College of Surgeons forums. The survey presented clinical EOL vignettes and subjective questions regarding PAS and euthanasia. Results: We obtained 142 responses from attending physicians. Respondents were typically male (61%), married (85%), identified as Christian (54%), had more than 20 years of experience (55%), and worked at a university hospital (57%). Religious beliefs and years of work experience seemed to be significant contributors in EOL decision making, whereas gender and medical specialty were not significantly influential. Conclusion: Factors such as years of work experience and religious belief may influence medical professionals’ opinions about PAS and euthanasia and their subsequent actions regarding EOL care. In many cases, the boundaries are blurred and require further study before concrete conclusions can be made.

Changes Made to Orders Placed by Overnight Admitting Residents on Teaching Rounds the Next Day

OBJECTIVES: Increased focus on health care quality and safety has generally led to additional resident supervision by attending physicians. At our children’s hospital, residents place orders overnight that are not explicitly reviewed by attending physicians until morning rounds. We aimed to categorize the types of orders that are added or discontinued on morning rounds the morning after admission to a resident team and to understand the rationale for these order additions and discontinuations. METHODS: We used our hospital’s data warehouse to generate a report of orders placed by residents overnight that were discontinued the next morning and orders that were added on rounds the morning after admission to a resident team from July 1, 2017 to June 29, 2018. Retrospective chart review was performed on included orders to determine the reason for order changes. RESULTS: Our report identified 5927 orders; 538 were included for analysis after exclusion of duplicate orders, administrative orders, and orders for patients admitted to non-Pediatric Hospital Medicine services. The reason for order discontinuation or addition was medical decision-making (n = 357, 66.4%), change in patient trajectory (n = 151, 28.1%), and medical error (n = 30, 5.6%). Medical errors were most commonly related to medications (n = 24, 80%) and errors of omission (n = 19, 63%). CONCLUSIONS: New or discontinued orders commonly resulted from evolving patient management decisions or changes in patient trajectory; medical errors represented a small subset of identified orders. Medical errors were often errors of omission, suggesting an area to direct future safety initiatives.

Main metabolic and toxic polyneuropathies in clinical practice

Polyneuropathies are diseases of the peripheral nervous system with lesions of motor, sensory or autonomic fibers which are encountered by attending physicians of almost all specialties in outpatient and clinical settings. To date, more than 100 different causes of polyneuropathies have been identified. Metabolic and toxic polyneuropathies are the most common in the group of secondary polyneuropathies. Diabetic, alcoholic, uremic, and drug-induced polyneuropathies take the leading place among these diseases. The main forms of diabetic polyneuropathy are presented. The main clinical form is distal symmetrical polyneuropathy. Clinical symptoms depend on the type of fibers involved in the pathological process - thin or thick. There is an assessment scale in points to determine the severity of diabetic polyneuropathy, which helps in clarifying the diagnosis and prognosis of the disease. The next most frequent among metabolic polyneuropathies is uremic polyneuropathy as the most frequent complication in patients suffering from chronic renal insufficiency. Risk factors of uremic polyneuropathy development, clinical picture, the course of the disease are described. Within the framework of toxic polyneuropathies, the main place is given to alcoholic polyneuropathies, chemotherapy-induced, and drug-induced. For each of these categories, clinical forms and pathophysiology of development are described. For all polyneuropathies, the main diagnostic aspects are presented. The main therapeutic approaches are shown. A separate place is given to the use of alpha-lipoic acid.

Physicians’ approach to end of life care: comparison of two tertiary care university hospitals in Lebanon

Background The care of terminally ill patients is fraught with ethical and medical dilemmas carried by healthcare professionals. The present study aims to explore the approaches of Lebanese attending physicians towards palliative care, end of life (EOL) care, and patient management in two tertiary care university hospitals with distinct medical culture. Methods Four hundred attending physicians from the American University of Beirut Medical Center (AUBMC) and Hotel Dieu de France (HDF) were recruited. Participants were Medical Doctors in direct contact with adult patients that could be subject to EOL situations providing relevant demographic, educational, religious as well as personal, medical or patient-centric data. Results The majority of physicians in both establishments were previously exposed to life-limiting decisions but remains uncomfortable with the decision to stop or limit resuscitation. However, physicians with an American training (AUBMC) were significantly more likely to exhibit readiness to initiate and discuss DNR with patients (p<0.0001). While the paternalistic medicinal approach was prevalent in both groups, physicians with a European training (HDF) more often excluded patient involvement based on family preference (p<0.0001) or to spare them from a traumatic situation (p=0.003). The majority of respondents reported that previous directives from the patient were fundamental to life-limiting decisions. However, the influence of patient and medical factors (e.g. culture, religion, life expectancy, age, socioeconomic status) was evidenced in the HDF group. Conclusion Early physician-initiated EOL discussions remain challenged in Lebanon. Paternalistic attitudes limit shared decision making and are most evident in European-trained physicians. Establishing a sound and effective framework providing legal, ethical and religious guidance is thus needed in Lebanon.

COMMUNICATION IN ONCOLOGY: WHAT PARENTS OF PEDIATRIC PATIENTS DON’T TALK ABOUT?

Communication during the treatment process is vital as it relates directly to the treatment outcomes. For patients and their family members, obtaining and possessing reliable information is essential for building successful and strong communication between physician, patient and in cases of paediatric cancer, the official guardians of the child and family members. The purpose of the study is to identify and analyse communication barriers in communication between physicians and parents (guardians), medical staff and parents (guardians) of paediatric cancer patients. Materials and methods. A retrospective cohort research among parents of paediatric cancer patients who took care of their children while receiving programmed chemotherapy was carried out. The information was collected indirectly: the information was provided by respondents remotely and anonymously using Google Forms. Results. The survey involved 106 family members of paediatric cancer patients receiving treatment at Ukrainian medical institutions specializing in the paediatric cancer treatment. 66% (n=70) of the respondents indicated that it was difficult to find contact with physicians; 85% (n=90) of respondents stated that it was difficult to find the contact with nurses and medical staff; 39,2% of the respondents reported the communication lapses with their attending physicians because the latter were busy and had no enough time to communicate. Considering that the overwhelming majority of respondents (79, 3%) noted physicians and medical staff as the most convenient source of reliable information on paediatric cancer, the researchers proposed to create an information platform for parents of paediatric cancer patients in order to provide access to information on the main issues of paediatric cancer. Conclusions. The research has revealed the following challenges: lack of adequate communication between the patients’ parents and physicians and / or medical staff; parents’ feeling unprepared to receive and comprehend information because of emotional trauma; poor communication between parents and physicians and / or medical staff due to the lack of time. A tool to eliminate these communication barriers can be suggested: this implies the creation of a video channel with visual thematic and clearly structured content for parents of paediatric cancer patients.

A Disclosure About Death Disclosure: Variability in Circulatory Death Determination

Introduction. Circulatory-respiratory death declaration is a common duty of physicians, but little is known about the amount of education and physician practice patterns in completing this examination. Methods. We conducted an online survey of physicians evaluating the rate of formal training and specific examination techniques used in the pronouncement of circulatory-respiratory death. Data, including level of practice, training received in formal death declaration, and examination components were collected. Results. Respondents were attending physicians (52.4%), residents (30.2%), fellows (10.7%), and interns (6.7%). The majority of respondents indicated they had received no formal training in death pronouncement, however, most reported self-perceived competence. When comparing examination components used by our cohort, 95 different examination combinations were used for death pronouncement. Conclusions. Formal training in death pronouncement is uncommon and clinical practice varies. Implementation of formal training and standardization of the examination are necessary to improve physician competence and reliability in death declarations.