Acid maltase deficiency--Pompe\u27s disease

Mutation in genes encoding for proteins involved in glycogen synthesis, degradation or regulation results in various inborn errors of glycogen metabolism. The disorders that result in abnormal storage of glycogen are known as glycogen storage diseases (GSD). We report a rare and interesting case of a young boy who presented with generalized weakness and reduced muscle bulk since childhood. He was diagnosed to have acid maltase deficiency, also known as Pompe\u27s disease, one of the rare types of glycogen storage disease. The case is presented here in the form of a case study, including a review of the pertinent literature on the subject. This case has the potential to be the first reported case of such a disease from Pakistan (to the best of our knowledge)

From Ideology to Economics: Understanding the Conflict between two Countries

Religious and ideological disparities have contributed to tensions between the two nations. The division of British India in 1947 along religious lines created a deep-seated divide, leading to ongoing conflicts. A key factor is the territorial dispute over Kashmir. Both countries claim Kashmir, resulting in multiple wars and persistent tensions. Political, military, and economic interests, as well as national pride, drive this dispute. India and Pakistan both seek regional dominance in South Asia. Kashmir’s strategic location near China adds complexity. Control over Kashmir allows Pakistan a land link with China, while India sees it as crucial for its territorial integrity. The legacy of the 1947 partition of British India plays a significant role. Along religious lines, the partition caused riots and mass migrations, leaving a lasting impact on relations. International Involvement: Global powers have strategic and economic interests in the region, influencing the conflict dynamics. The international community has been concerned with various attempts at mediation and resolution. The G20 summit, which India is hosting in 2023, is another platform where the Kashmir issue may be raised or sidelined by the two countries, depending on their strategies and objectives. Pakistan has opposed India’s decision to hold a G20 meeting in Srinagar, the capital of Indian-administered Kashmir. Conversely, India has asserted its sovereignty over the entire region and dismissed Pakistan’s objections. The G20 summit may also offer an opportunity for dialogue and cooperation between India and Pakistan on other global issues, such as climate change, economy, and development. Kashmir’s rich natural resources, including fruits, saffron, and minerals, offer economic benefits. India and Pakistan aim to benefit from these resources and tourism potential, adding another layer to the conflict. The India-Pakistan conflict is complex, involving religious, ideological, territorial, historical, geopolitical, and economic factors. Resolving this multifaceted issue requires addressing all these dimensions comprehensively. To research the India-Pakistan conflict, we can employ a multidisciplinary research methodology to comprehensively address the various dimensions involved: Historical Analysis, case studies, geopolitical analysis, and economic analysis

Utilization of social media for different business purposes: A social media age?

The utilization of social networking sites (SNSs) and social networking information (SNI) as parts of broader social media (SM) for various business purposes and practices have gained substantive importance from the academicians and practitioners in recent years. This paper theoretically aims to highlight some of such utilization based on published papers. We carefully selected 132 such papers from “Google Scholar” searching for most frequently used keywords such as SM, SNS, SNI, business, human resource management (HRM), marketing, branding, talent search & acquisition, recruitment & selection etc. After reviewing those papers, we identified that, in general, SM is an increasingly used platform for different business purposes. We anticipate that, despite of several flaws, SNSs will continue to gain momentous attention of all types of users in upcoming years creating a social media based business world. We are hopeful that the paper will be useful for the academicians, practitioners and policymakers from a number of viewpoints

Impact of service quality on Loyalty & Mediating role of Trust: An empirical investigation of Restaurants

Study was conducted to investigate the relationship between service quality, trust and loyalty. The trust is mediating between service quality and loyalty. Service quality provided to the customer is responsibility of the restaurants. Using a convenient sampling data was collected from consumers. Service quality positively associated with trust and loyalty and trust is also positively linked with loyalty and mediates the relationship between service quality and loyalty according to our finding. This article helps the organizations to understand the importance of service quality provide and how consumers become loyal to the organization. Customer retention is higher and profitability is greater for the business over the long period of time.&nbsp

Prevalence of clinical spectrum of cutaneous adverse drug reactions in patients presenting at a tertiary care hospital in Pakistan

Introduction: Cutaneous adverse drug reactions (CADRs) are the most common adverse drug reactions reported in the literature. CADRs have resulted in disabling infirmities during hospitalization and complications following outdoor drug therapy. The pattern of CADRs and the responsible drugs usually changes with the introduction of newer drugs and evolving clinical practices. Moreover, several international studies showed variable prevalence, emphasizing the need for local data in light of different socioeconomic and demographic practices. Therefore, the purpose of this study is to evaluate the prevalence of adverse cutaneous drug reactions and identify the clinical spectrum and any potential risk factors.Methodology: The current study is a descriptive cross-sectional study conducted at Aga Khan University Hospital, Pakistan. One hundred ninety-three patients who met the study inclusion criteria were included. Data were collected from patients on a proforma after taking informed consent. Quantitative data were presented as simple descriptive statistics giving mean and standard deviation, while qualitative variables were presented as frequency and percentages. Effect modifiers were controlled through stratification to highlight the effect of these on the outcome variable. The post-stratification chi-square test was applied and the p-value of ≤0.05 was statistically significant.Results: A total of 193 patients who had cutaneous adverse drug reactions were included in the study. The mean age in this study was 47.78±8.33 years. One hundred eight (56%) were male and 85 (44%) were female. Out of 193 patients, 135 (69.9%), 50 (25.9%), 24 (12.4%), 12 (6.2%), 20 (10.4%), 11 (5.7%) and six (3.1%) had maculopapular rash, acneiform eruptions, Stevens-Johnson syndrome, erythema multiform, urticaria, fixed drug eruptions and toxic epidermal necrolysis, respectively.Conclusion: CADRs are a common clinical presentation and awareness and knowledge about their diagnosis and prevention is important. It can be assumed that in our local setup, the clinical trends and medications causing ADRs are strikingly similar to those found in other countries. Physicians commonly come across these cases and they should be well aware of the clinical spectrum of skin reactions to enable early diagnosis and management

Factors Predicting the Recurrence of Spontaneous Bacterial Peritonitis in Patients with Cirrhosis

Objective: To evaluate the frequency of recurrence of spontaneous bacterial peritonitis (SBP) in patients with end stage liver disease and the factors responsible for it. Study Design: Descriptive study. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from November 2008 till November 2009. Methodology: Patients with cirrhosis who were admitted at AKUH with diagnosis of SBP during the study period were included. Any episode of SBP after resolution of the first index case of SBP within one year was considered as recurrence. Results: Out of 238 cirrhotic patients, 157 (66%) had single, while 81 (34%) had recurrent episodes of SBP. History of using proton pump inhibitors (PPI) and diuretics was found in 113 (47.5%) and 139 (58.4%) patients respectively. Only 58 (24.4%) patients were on prophylactic antibiotic therapy. Univariate analysis revealed that the female gender (52%), and presence of porto-systemic encephalopathy (PSE, 31%) were statistically significant (p=0.03) among those who had recurrent SBP. On multivariate analysis bilirubin level of \u3e 1.0 mg (OR=7.03; 95%CI=1.55-32), protective factor of hepatitis B (OR 0.31; 95%CI=0.13-0.70) and presence of urinary tract infection (UTI) (OR=2.24; 95%CI=0.99-5.09) were significant in patients with recurrent SBP. Conclusion: Recurrent SBP was noticed in 34% patients. Serum bilirubin level of \u3e 1.0 mg, protective factor of HBV and presence of UTI were significant factors present in patients with recurrent SBP

Career goals of trainee physicians in internal medicine

OBJECTIVE: To survey Internal Medicine trainees\u27 future career choices and factors influencing their decision-making. DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from November 2006 to January 2007. SUBJECTS AND METHODS: A standard questionnaire was administered to the residents and Interns working in the Department of Internal Medicine of The Aga Khan University Hospital. The questionnaire covered demographic details, specialty choice, work-time distribution and factors influencing career choice. Descriptive statistics were applied. RESULTS: A total of 49 doctors participated with 57% females; and 43% males, having 33% interns and 67% residents. A minority (22%) opted for internal medicine, while 78% selected sub-specialties with cardiology (20%) being the most sought- after sub-specialty. Majority (69%) wanted to spend more time in clinical activities compared to administration (18%) or research (13%). Majority (92%) wanted to work in an academic setting. Availability of a structured training program (61%), ability to practice broad area of medicine (41%) and prestige of the specialty (41%) highly influenced their career choices. Forty one (82%) wanted to proceed abroad for further training and 80% wanted to eventually practice in Pakistan. CONCLUSION: Most of the interviewed under-training physicians wanted to pursue sub-specialty. Clinical work was more attractive compared to research. A structured training program was detrimental in affecting their choices. Majority wanted further training abroad but eventual settlement in Pakistan. The study results can help us create the basis for reforming the current training programs

4-Methyl-N-(4-methyl­phen­yl)benzene­sulfonamide

In the title compound, C14H15NO2S, the two aromatic rings enclose a dihedral angle of 70.53 (10)°. A weak intra­molecular C—H⋯O hydrogen bond generates an S(6) ring motif. The crystal structure features inversion-related dimers linked by pairs of N—H⋯O hydrogen bonds