Autosomal Recessive Hyper-IgE Syndrome in a Child With Beta Thalassemia Trait: A Case Report.

Autoimmune diseases are multifaceted disorders, and their coexistence with other conditions can present unique challenges in diagnosis and management. Here, we report a rare case of autosomal recessive hyper-IgE syndrome (AR-HIES) in a child with beta thalassemia trait. AR-HIES is a distinct immunodeficiency disorder characterized by severe eczema and recurrent bacterial and viral infections, particularly affecting the sinopulmonary system. This case highlights the importance of recognizing and managing the co-occurrence of rare genetic conditions, as it can impact treatment strategies and familial counseling. This unique case of AR-HIES in a child with beta thalassemia trait underscores the complexity of autoimmune disorders and the need for comprehensive evaluation in patients presenting with multiple clinical manifestations.

Isolated Malignant Pleural Effusion in a Child: Unusual Presentation of Acute Leukemia.

Pleural effusion in the pediatric population is an abnormal pathology characterized by the accumulation of fluids between the parietal and visceral pleura. The etiology of this excessive fluid accumulation can be attributed to both infectious and non-infectious factors. Notably, Streptococcus pneumoniae stands out as the predominant infectious agent responsible for this condition. Non-infectious causative factors encompass hematolymphoid malignancies, congestive heart failure, hemothorax, hypoalbuminemia, and iatrogenic causes. Among the hematolymphoid malignancies, lymphoma emerges as the most prevalent malignancy associated with pleural effusion. It is followed by T-cell lymphoblastic leukemia, germ cell tumor, neurogenic tumor, chest wall and pulmonary malignancy, carcinoid tumor, pleuro-pulmonary blastoma, and Askin's tumor, among others. Malignant pleural effusion is predominantly linked to T-cell lymphoblastic malignancies. In the context of acute lymphoblastic leukemia (ALL), cases where T-cell presentation is accompanied by leukemic pleural effusion are commonly associated with either a mediastinal mass or significant lymphadenopathy. Here, we describe a case of a four-year-old male child who exhibited a brief history of febrile illness. Notably, this case was characterized by isolated pleural effusion, devoid of any mediastinal mass or lymphadenopathy. Pathological investigations of pleural fluid analysis revealed the presence of malignant cells, facilitating an expedited diagnosis.

Depression and health-related quality of life of patients with type 2 diabetes attending tertiary level hospitals in Dhaka, Bangladesh.

INTRODUCTION: Type 2 diabetes mellitus (T2DM) and depression are closely linked. People with T2DM are at increased risk of developing depression and vice versa. T2DM and depression comorbid conditions adversely affect Health-Related Quality of Life (HRQOL) and management of T2DM. In this study, we assessed depression and HRQOL among patients with T2DM in Dhaka, Bangladesh. METHODS: A cross-sectional study was conducted in two tertiary-level hospitals in Dhaka, Bangladesh. Data were collected from 318 patients with T2DM. A set of standard tools, PHQ-9 (for assessing depression) and EuroQol-5D-5L (for assessing the HRQOL), were used. Statistical analyses, including Chi-square and Fisher's exact tests, Wilcoxon (Mann-Whitney), and Spearman's correlation coefficient tests, were performed using SPSS (v.20). RESULTS: The majority of the patients (58%) were females, with a mean age (standard deviation) of 52 ± 10 years, and 74% of patients lived in urban areas. The prevalence of depression was 62% (PHQ-9 score ≥ 5). Over three-quarters (76%) reported problems in the anxiety/ depression dimension of EQ-5D, followed by pain/discomfort (74%), mobility (40%), self-care (36%), and usual activities (33%). The depression and T2DM comorbid condition were associated with all the five dimensions of EQ-5D (χ2 statistics with df = 1 was 52.33, 51.13, 52.67, 21.61, 7.92 for mobility, self-care, usual activities, pain/discomfort, and anxiety/ depression dimensions respectively, p- < 0.01). The mean EQ-5D index (0.53 vs. 0.75) and the mean EQ-5D VAS (65 vs. 76) both showed lower values in T2DM patients with depression compared to T2DM patients without depression (Wilcoxon test, p- < 0.001). CONCLUSIONS: We conclude that the majority of the patients with T2DM had comorbid conditions, and the HRQOL was negatively affected by comorbid depression in T2DM patients. This suggests the importance of timely screening, diagnosis, treatment, and follow-up of comorbid depression in T2DM patients to improve overall health and QOL.

A Case Report on CNS Hemophagocytic Lymphohistiocytosis in an Infant With Dengue Hemorrhagic Fever.

India is an endemic country for dengue. The incidence of hemophagocytic lymphohistiocytosis (HLH) with dengue in children has been well-reported. However, central nervous system (CNS) HLH associated with dengue has not been described in the literature yet. We hereby report a novel case of CNS HLH triggered by dengue infection. An eight-month-old, well-grown male infant with uneventful antenatal, perinatal, and neonatal history was admitted with a history of febrile illness associated with cough, cold, vomiting, and loose motions and one episode of hematochezia and hepatosplenomegaly on examination. Investigations revealed bi-cytopenia, hyper-ferritinemia, deranged coagulation profile, liver function test, and hypo-fibrinogenemia. Dengue non-structural protein 1 ( NS1) antigen was positive. The child was given dexamethasone and continued supportive care with a diagnosis of dengue shock syndrome. The child showed an overall transient improvement, however, he had rebound fever followed by right focal convulsion on Day 9 of steroids. MRI brain revealed areas of diffusion-restricted embolic infarcts with diffuse leptomeningeal enhancement and mild cerebral edema, and CSF showed a total leukocyte count of 80 cells with 75% lymphocytic picture, histiocytes with hemophagocytosis, confirmatory of CNS HLH. Intrathecal methotrexate, hydrocortisone, and intravenous (IV) etoposide were started. However, the child succumbed to his illness. CNS involvement in dengue-triggered HLH needs to be suspected despite subtle neurological signs and aggressively managed following a multi-departmental approach to ensure the best clinical and neuro-developmental outcomes.

Molar Incisor Hypomineralization: Prevalence, Associated Risk Factors, Its Relation with Dental Caries and Various Enamel Surface Defects in 8-16-year-old Schoolchildren of Lucknow District.

Introduction: The decreased rate in dental caries cases across the world has created an enthusiasm in many clinicians to relate and study different developmental disorders. In past years, defects that are commonly associated with dentistry are hypomineralized areas commonly seen in central incisors and first molars. Molar incisor hypomineralization (MIH) is a defect of the enamel, which is qualitative in nature and systemic in origin characterized by advanced and concurrent hypomineralization of the enamel affecting the first permanent molars together with frequent association of the incisors. Aim: To evaluate the prevalence of molar incisor hypomineralization (MIH), its possible risk factors and its association with dental caries and enamel surface defects (attrition and abrasion) in schoolchildren aged between 8 and 16 years in Lucknow district. Methodology: Indexed teeth (first permanent molars and incisors) of 5,585 schoolchildren, selected by stratified random sampling technique between the age-group of 8 and 16 years, were examined by a trained and calibrated examiner. The data was recorded in a predesigned pro forma by examiner, which consisted of mainly two parts. The first part comprised of general information, while the second part comprised of questions related to risk factors related to MIH (prenatal, perinatal, and postnatal history). For the diagnosis of MIH, the Developmental Defects of Enamel (DDE) Index was used for diagnosis of MIH, while the decay-missing-filled teeth index (DMFT) criteria were used for assessing dental caries. Enamel surface defects were recorded using the Smith and Knight tooth wear index. Results: A prevalence of 7.6% was reported wherein females were found to be more affected by MIH. A strong significant correlation was found between MIH prevalence and childhood infection. Conclusion: Early diagnosis of MIH is necessary to prevent the rapid destruction of the tooth morphology resulting in complicated treatment. Further studies with greater samples are needed to investigate the different etiological factors and determine the biological molecular mechanism that they may cause. Clinical significance: The data obtained from the current study does not portray a clear consideration of the infants' medical history in the initial 4 years of life. Further studies may be performed to surpass these shortcomings by using more elaborate medical records of the child in addition to profound recollection of the parents. Due to paucity of literature on this issue in Lucknow District, our current study may provide some information at a baseline level for conducting an extensive research involving different regions pan-India. How to cite this article: Verma S, Dhinsa K, Tripathi AM, et al. Molar Incisor Hypomineralization: Prevalence, Associated Risk Factors, Its Relation with Dental Caries and Various Enamel Surface Defects in 8-16-year-old Schoolchildren of Lucknow District. Int J Clin Pediatr Dent 2022;15(1):1-8.

COVID-19 fosters social accountability in medical education.

The COVID-19 pandemic has highlighted embedded inequities and fragmentation in our health systems. Traditionally, structural issues with health professional education perpetuate these. COVID-19 has highlighted inequities, but may also be a disruptor, allowing positive responses and system redesign. Examples from health professional schools in high and low- and middle-income countries illustrate pro-equity interventions of current relevance. We recommend that health professional schools and planners consider educational redesign to produce a health workforce well equipped to respond to pandemics and meet future need.

Commentary: Moral and Ethical Leadership in the Age of Diversity, Equity, Inclusiveness and Social Accountability.

This article weighs three important moral and ethical considerations for leaders in healthcare: their obligations to society, their privilege as leaders and how value goes beyond a simple cost analysis. Leaders highly motivated by the bottom line have avoided the long overdue action on moral and ethical considerations critical to a more just and fairer society. Leaders are now being tasked to develop strategies for health equity, anti-oppression, anti-racism, social justice, diversity, equity and inclusiveness, community engagement, the social determinants of health and environmental accountability, and to demonstrate that their disruptive work adds more value to society than what can be measured in spreadsheet metrics.

Creating a Lactation-Friendly Learning Environment for Medical Students and Residents: A Northern Canadian Perspective.

Background: Lactation is a normal postpartum physiological process that can continue in excess of 3 years and is often the sole nutritional source for infants in the first 6 months of life. Breastfeeding not only provides infant nutrition, but also facilitates maternal-infantile bonding. Lactating mothers separated from their children face multiple challenges in finding and accessing appropriate spaces and time for milk expression. Maternal employment is a great barrier to breastfeeding and accordingly has led to multiple advancements in the area of breastfeeding policy. One example of a policy is the Baby-Friendly Initiative. This initiative focused on breastfeeding promotion, support, and protection. However, the impact of such campaigns on lactating medical students and residents is thought to be low. Furthermore, breastfeeding rates differ vastly according to geographic locations in North America. Trends indicate decreased rates of breastfeeding in northern rural areas in comparison with southern urban counterparts. This highlights the need for increased protection, support, and creation of safe-lactation spaces for all working mothers including medical students and residents, and especially those in rural areas. Goals: To review challenges of breastfeeding as a medical trainee and delineate the creation of a lactation policy for medical learners and residents. Methodology: We conducted a literature review of breastfeeding policy and experiences of breastfeeding while in the learning environment. Results: Challenges of breastfeeding in medical school and residency include the complex, high-paced medical working environment where taking breaks or time off is often difficult. Few medical schools across North America have any breastfeeding policy. Conclusion: The Northern Ontario School of Medicine's lactation policy serves as a possible solution to the barriers medical students and residents face when breastfeeding in the academic environment. This policy creates lactation-friendly medical learning spaces through the entitlement of dedicated space and time for milk expression.

Navigating the Leadership Landscape: Creating an Inventory to Identify Leadership Education Programs for Health Professionals.

As health systems become increasingly complex, there is growing emphasis on collaborative leadership education for health system change. The Canadian Interprofessional Health Leadership Collaborative conducted research on this phenomenon through a scoping and systematic review of the health leadership literature, key informant interviews and an inventory of health leadership programs in Canada. The inventory is unique, accounting for educational programming missed by traditional scholarly literature reviews. A major finding is that different health professions have access to health leadership education in different stages of their careers. This pioneering inventory suggests that needs may differ between health professions but also that there is a growing demand for multiple types of programs for specific targeted audiences, and a strategic need for collaborative leadership education in healthcare.

Understanding the needs of department chairs in academic medicine.

PURPOSE: The challenges for senior academic leadership in medicine are significant and becoming increasingly complex. Adapting to the rapidly changing environment of health care and medical education requires strong leadership and management skills. This article provides empirical evidence about the intricate needs of department chairs to provide insight into the design of support and development opportunities. METHOD: In an exploratory case study, 21 of 25 (84%) department chairs within a faculty of medicine at a large Canadian university participated in semistructured interviews from December 2009 to February 2010. The authors conducted an inductive thematic analysis and identified a coding structure through an iterative process of relating and grouping of emerging themes. RESULTS: These participants were initially often insufficiently prepared for the demands of their roles. They identified a specific set of needs. They required cultural and structural awareness to navigate their hospital and university landscapes. A comprehensive network of support was necessary for eliciting advice and exchanging information, strategy, and emotional support. They identified a critical need for infrastructure growth and development. Finally, they stressed that they needed improvement in both effective interpersonal and influence skills in order to meet their mandate. CONCLUSIONS: Given the complexities and emotional burden of their role, it is necessary for chairs to have a range of supports and capabilities to succeed in their roles. Their leadership effectiveness can be enhanced by providing transitional processes and supports, development, and mentoring as well as facilitating the development of communities of peers.

Remediation of residents in difficulty: a retrospective 10-year review of the experience of a postgraduate board of examiners.

PURPOSE: To determine, through a 10-year review, (1) the prevalence of residents in difficulty, (2) characteristics of these residents, (3) areas of residents' weakness, and (4) outcomes of residents who undergo remediation. METHOD: A retrospective review of resident records for the University of Toronto Faculty of Medicine's (UT-FOM) Board of Examiners for Postgraduate Programs (BOE-PG) was done from July 1, 1999 to June 30, 2009 using predetermined data elements entered into a standardized form and analyzed for trends and significance. Outcomes for residents in difficulty were tracked through university registration systems and licensure databases. RESULTS: During 10 years, 103 UT-FOM residents were referred to the BOE-PG, representing 3% of all residents enrolled. The annual prevalence of residents referred to the BOE-PG ranged from 0.2% to 1.5%. The CanMEDS framework was used to classify areas of residents' weaknesses and organize remediation plans. All 100 residents studied had either medical expertise (85%) or professionalism (15%) weaknesses or both. Residents had difficulties with an average of 2.6 CanMEDS Roles, with highest frequencies of Medical Expert (85%) Professional (51%), Communicator (49%), Manager (43%), and Collaborator (20%). Often, there were multiple remediation periods, with an average of six months' duration. Usually, remediation was successful; 78% completed residency education, 17% were unsuccessful, and 5% remained in training. CONCLUSION: Residents in difficulty have multiple areas of weakness. The CanMEDS framework is an effective approach to classifying problems and designing remediation plans. Successful completion of residency education after remediation is the most common outcome.

Tracking family medicine graduates. Where do they go, what services do they provide and whom do they see?

BACKGROUND: There are continued concerns over an adequate supply of family physicians (FPs) practicing in Canada. While most resource planning has focused on intake into postgraduate education, less information is available on what postgraduate medical training yields. We therefore undertook a study of Family Medicine (FM) graduates from the University of Toronto (U of T) to determine the type of information for physician resource planning that may come from tracking FM graduates using health administrative data. This study compared three cohorts of FM graduates over a 10 year period of time and it also compared FM graduates to all Ontario practicing FPs in 2005/06. The objectives for tracking the three cohorts of FM graduates were to: 1) describe where FM graduates practice in the province 2) examine the impact of a policy introduced to influence the distribution of new FM graduates in the province 3) describe the services provided by FM graduates and 4) compare workload measures. The objectives for the comparison of FM graduates to all practicing FPs in 2005/06 were to: 1) describe the patient population served by FM graduates, 2) compare workload of FM graduates to all practicing FPs. METHODS: The study cohort consisted of all U of T FM postgraduate trainees who started and completed their training between 1993 and 2003. This study was a descriptive record linkage study whereby postgraduate information for FM graduates was linked to provincial health administrative data. Comprehensiveness of care indicators and workload measures based on administrative data where determined for the study cohort. RESULTS: From 1993 to 2003 there were 857 University of Toronto FM graduates. While the majority of U of T FM graduates practice in Toronto or the surrounding Greater Toronto Area, there are FM graduates from U of T practicing in every region in Ontario, Canada. The proportion of FM graduates undertaking further emergency training had doubled from 3.6% to 7.8%. From 1993 to 2003, a higher proportion of the most recent FM graduates did hospital visits, emergency room care and a lower proportion undertook home visits. Male FM graduates appear to have had higher workloads compared with female FM graduates, though the difference between them was decreasing over time. A 1997 policy initiative to discount fees paid to new FPs practicing in areas deemed over supplied did result in a decrease in the proportion of FM graduates practicing in metropolitan areas. CONCLUSIONS: We were able to profile the practices of FM graduates using existing and routinely collected population-based health administrative data. Further work tracking FM graduates could be helpful for physician resource forecasting and in examining the impact of policies on family medicine practice.

Comparison of Canadian medical graduates and international medical graduates in Canada: 1989-2007.

PURPOSE: To compare Canadian medical graduates (CMGs) and international medical graduates (IMGs) who completed postgraduate medical education in Canada from 1989 to 2007 by age, gender, specialty, and practice characteristics. METHOD: Data on all CMGs and IMGs who completed residencies or fellowships in Canada from 1989 to 2007 were extracted from the Canadian Post-M.D. Education Registry. Data from 1989-1993 and 2003-2007 were pooled for analysis. RESULTS: A total of 8,501 CMGs and 1,828 IMGs completed post-MD training at Canadian institutions between 1989 and 1993 inclusive; 7,734 CMGs and 1,879 IMGs completed such training between 2003 and 2007. From 1989-1993 to 2003-2007, the average age of CMGs increased from 29.8 to 31.1 years, and average age of IMGs increased from 36.1 to 37.0 years. From 1989-1993 to 2003-2007, the percentage of women increased from 41% (3,471/8,501) to 52% (4,016/7,734) and from 28% (509/1,828) to 42% (791/1,879) for CMGs and IMGs, respectively. The proportion of CMGs who trained in family medicine declined from 54% (4,568/8,501) to 38% (2,921/7,734) from 1989-1993 to 2003-2007. The percentage of IMGs who trained in family medicine increased from 19% (344/1,828) to 37% (699/1,879) during the same period. CONCLUSIONS: IMGs tended to be older, more likely to be men, and more likely to pursue family medicine than their CMG counterparts. These differences have implications in designing future health care policy and recruiting physicians from abroad. Other countries could look at their own physician demographics using this study's methods.

The shifting landscape of immigration policy in Canada: implications for health human resources.

For many years, Canada has relied on international migration to compensate for cyclical shortages in its skilled labour force. This paper reports on recent changes in Canadian immigration policy, namely, the introduction of new immigration programs focused on skilled workers, along with the implementation of domestic mobility agreements. With specific reference to the case of nursing, the paper highlights the necessity for integrated policy across multiple government levels and stakeholder groups, as well as the need to promote the development of evidence-based policy in the fields of immigration and health human resources.

Core competencies: the next generation. Comparison of a common framework for multiple professions.

This report demonstrates the application of a competency model to the regulated and unregulated professions of medical radiation technology, social work, pharmacy, and psychology. The competency model is based on the CanMEDS framework and was originally applied to the professions of medicine, occupational therapy, physical therapy, and nursing in an earlier work. The framework identifies the core competencies common to learners in health care, which are professional (and health advocate), expert, scholar, manager, communicator, and collaborator. In this report, these core competencies are applied to four additional disciplines in an effort to make the cultural shift from discipline-based silos to a common language for ascertaining the skills, knowledge, and attitudes needed to function in interprofessional teams.