Weaving Stories into Education: An Urgent Need to Integrate Narrative Medicine into Pakistan's Medical Curriculum.

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FDA approves Ruxolitinib (Opzelura) for Vitiligo Therapy: A breakthrough in the field of dermatology.

The Food and Drug Administration (FDA) has authorized Ruxolitinib (Opzelura), as first at-home treatment for non-segmental vitiligo, an autoimmune condition that causes spots and patches of paler skin. Previously, it was used to treat atopic dermatitis, myelofibrosis, essential thrombocythemia, and polycythemia vera. It functions by lowering an individual's enhanced immune response, gradually promoting the development of new, healthy skin cells, and ultimately reintroducing pigment to the afflicted area. Using this topical lotion twice daily can not only produce even skin tones but also boost patients' self-esteem because vitiligo can be physically and psychologically upsetting. It is comparatively more efficacious and has a better safety profile than the oral forms of this medicine, although adverse effects such acne, redness, and itching at the application site, inflammation of the throat and nasal passages, headaches and fever have been observed, necessitating the need for observational studies and randomized controlled trials to demonstrate its efficacy and safety.

The Frequency of Refractory Status Epilepticus and Its Outcome in a Tertiary Care Hospital in Pakistan: A Retrospective Study.

Background Refractory status epilepticus (RSE) is a common neurologic emergency with refractory cases leading to increased rates of morbidity and mortality in patients. The lack of previous studies on the incidence, causes, and management of refractory status epilepticus in the pediatric population from our region prompted us to investigate further in this study. Methods We included retrospective data of all patients admitted to the pediatric intensive care unit (PICU) with a provisional diagnosis of RSE at a tertiary care hospital in Karachi from February 2019 to February 2021. No personal identification data was used, and confidentiality of the data was maintained throughout the analysis. The Statistical Package for the Social Sciences (SPSS) software version 22.0 (IBM SPSS Statistics, Armonk, NY, USA) was used to pool data and perform a descriptive analysis. Results Among the 687 patients who presented to the PICU with seizures, 50 (7.27%) patients were eventually diagnosed with RSE during the two-year period. The majority of the patients were male and less than one year of age. Infectious causes predominated our data cohort, and a four-drug regimen consisting of phenytoin, levetiracetam, valproic acid, and midazolam was able to terminate RSE in the majority of the patients in our setting (70%). The mortality rate was noted to be 22% among patients with RSE. Conclusion Morbidity and mortality among pediatric RSE patients are high in our settings. Urgent emergency services and timely cause-directed intervention could improve outcomes.

Factors associated with loss of motivation and hesitation to work amongst frontline health care providers during the COVID-19 pandemic: A cross-sectional survey from a developing country.

Background: The COVID-19 took over the world in 2020 and a lockdown has been imposed seeing its fast spread. Frontline health care workers (HCWs) were reported frequently with a lack of motivation, hesitancy and unwillingness to perform their duties during this pandemic. This cross-sectional survey aims to evaluate the factors associated with lack of motivation and increased hesitancy among the frontline HCWs to perform their duties during COVID-19 pandemic. Materials and methods: A total of 239 HCWs were included in this web-based cross-sectional study, who have worked during the COVID-19 pandemic. The anonymous online questionnaire was sent to all faculty, trainees and staff of Aga Khan University Hospital in Karachi, Pakistan. The survey was conducted from September 2020 to January 2021 during the COVID-19 pandemic. All data was exported into Statistical Package for Social Sciences Version 19 for multivariate analysis. Results: The risk of getting infected was strongly reported by 180 participants, and it was associated with higher hesitation to work (aOR = 6.09 [2.55-14.59]). Fifty-one participants felt that lack of knowledge about prevention and protection was associated with lower motivation to work (aOR = 0.66 [0.35-1.25]). Participants reported higher hesitation due to the burden of changed quality of work, physical exhaustion, mental exhaustion and altered sleep patterns. Sense of feeling protected by their hospitals was a motivating factor, and participants receiving adequate support reported higher motivation to work (aOR = 2.60 [1.32-5.14]). Conclusion: Fear of infection, increased working hours, and inadequate support of the workplace played a key role in escalating the hesitancy among HCWs to perform their duties. Lack of disease knowledge and paucity of personal protective equipment further lowered the motivation levels of HCWs to work effectively during the COVID-19 pandemic.

Voxelotor versus other therapeutic options for sickle cell disease: Are we still lagging behind in treating the disease?

Background: Sickle cell disease (SCD) is one of the most prevalent hemoglobinopathies that affects around 275,000 neonates annually. Until 2017, hydroxyurea was the only available drug for SCD treatment. Later on, L-glutamine and crizanlizumab have shown promising results in SCD therapy. Objectives: There were limited pharmacological options for the disease when in November, 2019, voxelotor was approved for the treatment of SCD patients after showing promising results in the clinical HOPE trial. Despite its favorable results, some life-threatening side effects were also observed. Uncertainty regarding the use of available pharmaceutical therapies for SCD is the major hurdle for the survival of patients. Discussion & Conclusion: An immediate attention needs to be drawn towards the drawbacks of limited pharmacological options for SCD. Article calls out to conduct more extensive trials in this advanced era of medicine where ambiguity regarding the use of SCD drugs still prevails.

Rare Case of Budd-Chiari Syndrome in a Young Child: A Diagnostic Conundrum.

Budd-Chiari syndrome (BCS) is an uncommon vascular disorder in which venous thrombosis prevents the venous outflow of the liver. The obstruction is primarily at the level of hepatic veins and inferior vena cava. Here, we present a case of a two-and-a-half-year-old male child who presented with complaints of abdominal distension for two months and fever and watery diarrhea for one month. Physical examination showed the patient was anemic with palmar erythema. He was started on an empirical treatment of cefotaxime, metronidazole, and amikacin. Sensitivity and culture reports for blood and urine samples were negative, but abdominal computed tomography (CT) scan showed characteristic findings for BCS with caudate lobe hypertrophy. After the symptomatic treatment of the patient, a liver transplant was suggested as a last resort.

Restless Leg Syndrome in Patients with Liver Cirrhosis! Its Frequency, Severity, and Correlation.

BACKGROUND: Restless leg syndrome (RLS) has been recognized as a typical additional manifestation among patients with cirrhosis of the liver. RLS prevalence in liver cirrhosis further worsens the quality of life, which has already been compromised with the disease-related complications of cirrhosis itself. AIMS: The study aimed to determine the frequency and severity of Restless Leg Syndrome (RLS) among patients with cirrhosis of the liver and to correlate its severity with the severity of cirrhosis. METHODS: This prospective cross-sectional study was carried out at Ruth K.M. Pfau Civil Hospital (Karachi, Pakistan) from December 2019 to February 2020. Three hundred and fifteen cirrhotic patients with any etiology were included in the study after informed written consent. Restless leg syndrome with its severity was determined in all cirrhotic patients. For continuous variables, means and medians with standard deviations were calculated, while percentages and proportions were used for discrete variables. Spearman correlation was used to find significance between RLS Severity Score (RSS) and Model of End-stage Liver Disease MELD Score. A p value of < 0.05 was considered significant for all analyses. RESULTS: The frequency of RLS among cirrhotic patients was 38.4%. More than half (54.5%) of the patients had severe RLS. Viral related hepatitis C and hepatitis B were the most common cause of cirrhosis of the liver (57.8% and 30.8%). A significant difference was found between as patients both with or without RLS in terms of age (p=0.003), gender (p=0.005), hemoglobin (p=0.00), and serum albumin (p=0.01). No significant association was found between RLS severity score and MELD Score (p=0.693). CONCLUSION: Prevalence of RLS is very high among cirrhotic patients, but no correlation was found between the severity of RLS and cirrhosis. Further studies should be carried out to assess the quality of life in cirrhotic patients having RLS.

Cardiac manifestations of the coronavirus disease-19: a review of pathogenesis, clinical manifestations, diagnosis, and treatment.

The coronavirus disease-19 (COVID-19), first appearing in Wuhan, China, and later declared as a pandemic, has caused serious morbidity and mortality worldwide. Severe cases usually present with acute respiratory distress syndrome (ARDS), pneumonia, acute kidney injury (AKI), liver damage, or septic shock. However, with recent advances in severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) research, the virus´s effect on cardiac tissues has become evident. Reportedly, an increased number of COVID-19 patients manifested serious cardiac complications such as heart failure, increased troponin, and N-terminal pro-B-type natriuretic peptide levels (NT-proBNP), cardiomyopathies, and myocarditis. These cardiac complications initially present as chest tightness, chest pain, and heart palpitations. Diagnostic investigations such as telemetry, electrocardiogram (ECG), cardiac biomarkers (troponin, NT-proBNP), and inflammatory markers (D-dimer, fibrinogen, PT, PTT), must be performed according to the patient´s condition. The best available options for treatment are the provision of supportive care, anti-viral therapy, hemodynamic monitoring, IL-6 blockers, statins, thrombolytic, and anti-hypertensive drugs. Cardiovascular disease (CVD) healthcare workers should be well-informed about the evolving research regarding COVID-19 and approach as a multi-disciplinary team to devise effective strategies for challenging situations to reduce cardiac complications.

Perception, Willingness, Barriers, and Hesitancy Towards COVID-19 Vaccine in Pakistan: Comparison Between Healthcare Workers and General Population.

Background Vaccine hesitancy has been a huge challenge in controlling preventable diseases. With the emergence of coronavirus disease 2019 (COVID-19) vaccines, it is vital to know their acceptance rates among the masses. No comparative data is available on the current subject from Pakistan yet. Therefore, this study aimed to evaluate the acceptance of a potential COVID-19 vaccine among the general population and healthcare workers (HCWs) of Pakistan, along with their perceptions and barriers to acceptance. Methods An online cross-sectional study was carried out in Pakistan from December 19, 2020, to January 10, 2021, using convenience sampling. A self-administered questionnaire consisting of 31 items was distributed after informed consent. Inclusion criteria consisted of HCWs and non-HCWs (general population) aged 18 years and above, residing in Pakistan. All analyses were done using Statistical Package for Social Sciences (SPSS) version 23.0 (IBM Corp., Armonk, NY, USA). Chi-square and T-test were used and a p-value of less than 0.05 was considered significant for all cases. Results Of the 404 respondents (n=196 general population and n=208 HCWs), 73.5% were willing to get a proven, safe, and effective COVID-19 vaccine if it was free of cost. This was reduced to only 64.3% if the vaccine was not free and had to be paid for. A total of 168 (41.6%) participants agreed to get vaccinated immediately, while 149 (36.9%) participants concurred to get it on a delayed basis. Eighty-seven (21.5%) participants refused to receive the COVID-19 vaccine, amongst which a significant majority (p<0.001) of the participants were from the general population. Doctors or scientists/scholarly journals were found to be the most trusted source of information (67.6%; n=273), while fear of unknown side effects (45.5%; n=184) was found to be the most common barrier towards COVID-19 vaccination. More than half (53.5%) participants believed that the vaccine is safe, effective, and has minimal side effects, amongst which a significantly large fraction (p<0.001) belonged to the HCWs. Conclusion The acceptance rate of a safe, effective, proven, and free COVID-19 vaccine was 73.5%. The fear of unknown side effects was the most common barrier to COVID-19 vaccine uptake. The general population demonstrated less knowledge, more false perceptions, and barriers to COVID-19 vaccine. Adequate measures should be taken to educate the masses about the COVID-19 vaccine, and its safety, and further studies are required.

Post-splenectomy Sepsis: A Review of the Literature.

The spleen is an intraperitoneal organ that performs vital hematological and immunological functions. It maintains both innate and adaptive immunity and protects the body from microbial infections. The removal of the spleen as a treatment method was initiated from the early 1500s for traumatic injuries, even before the physiology of spleen was properly understood. Splenectomy has therapeutic effects in many conditions such as sickle cell anemia, thalassemia, idiopathic thrombocytopenic purpura (ITP), Hodgkin's disease, and lymphoma. However, it increases the risk of infections and, in some cases, can lead to a case of severe sepsis known as overwhelming post-splenectomy infection (OPSI), which has a very high mortality rate. Encapsulated bacteria form a major proportion of the invading organisms, of which the most common is Streptococcus pneumoniae. OPSI is a medical emergency that requires prompt diagnosis (with blood cultures and sensitivity, blood glucose levels, renal function tests, and electrolyte levels) and management with fluid resuscitation along with immediate administration of empirical antimicrobials. OPSI can be prevented by educating patients, vaccination, and antibiotic prophylaxis. This article summarizes the anatomy and physiology of the spleen and highlights its important functions. It primarily focuses on the pathophysiology of OPSI, its current management, and prevention strategies.

Young-onset Amyotrophic Lateral Sclerosis with Rare Skin Manifestation: Case Report and Literature Review.

Amyotrophic lateral sclerosis (ALS) is one of the most common motor neuron diseases (MND), which presents as muscle weakness, atrophy, spasticity, and, in extreme cases, may result in death due to respiratory failure. ALS has been reported with dermatological conditions such as bullous pemphigoid and decreased collagen. Hyperpigmentation usually occurs due to underlying adrenal or metabolic disorder, but no case of hyperpigmentation has been associated with MND. We report a case of a 25-year-old man who presented with signs of young-onset ALS (progressive weakness of both upper limbs) with hyperpigmentation of limbs. The patient did not have any other underlying etiology, which could have led to the development of hyperpigmentation Biopsy was negative for polymyositis and dermatomyositis. The patient was counseled about the nature of the disease and was advised regular follow-ups.

Giant Leiomyosarcoma Arising in Posterior Thigh: Management of a Rare Case.

Leiomyosarcoma, primarily a tumor of smooth muscle origin, frequently originates from the uterus, retroperitoneum, and intra-abdominal region. Rarely, the tumor may arise from the conjunctiva, inferior vena cava, or oral cavity. Here we report a case of a 65-year-old male patient who presented with a swelling in the posterior thigh for six months. The swelling was progressively increasing in size for the last two months. Examination of thigh showed a swelling of 20×30 cm in size, which was firm, non-compressible, immobile, and not transilluminating. CT scan showed no metastasis in the liver, lung, or bone. The histopathology report showed poorly differentiated leiomyosarcoma involving the muscles of the posterior compartment of the left thigh. The tumor was resected, and the patient was referred to rehabilitation clinic. Early diagnosis of such cases is essential to improve the outcome in patients as these tumors can metastasize early.

Accuracy of Pediatric Risk of Mortality (PRISM) III Score in Predicting Mortality Outcomes in a Pediatric Intensive Care Unit in Karachi.

Background With the advancements in medicine and increasing access to modern technology, pediatric intensive care units (PICU) are becoming a vital part of any health care setting. PICUs play a key role in saving the life of young patients. Various scales have been designed by researchers to aid in predicting the mortality of a patient admitted in PICU. Pediatric Risk of Mortality (PRISM) and Pediatric Index of Mortality (PIM) are among the most commonly used scales. Calculating the risk of mortality enables the physicians to classify the patients and helps in identifying which patients require more urgent care and resources.  Methods A hospital-based prospective study was carried out at PICU in a tertiary care hospital in Karachi from December 2017 to June 2019. All patients between the age of one month and 12 years were included in our study after informed consent from parents/guardians. A standard questionnaire was used and the PRISM III score was calculated at 24 hours of admission. All necessary investigations were carried out, and all statistical analyses were carried out using SPSS v.23 (IBM, Armonk, NY). Results A total of 407 patients were included in our study with the majority being males (54.5%). The mean age was 27±33 months. The mean duration of stay of patients in PICU was 80.15±36.58 hours. The mortality rate in our study was 37.35 % (n=152). The need for mechanical ventilation, use of inotropic drugs, higher temperatures, and low Glasgow Coma Scale scores were associated with poor survival. It was noted that as the PRISM III score increased, the mortality rate also increased. In our study, we found that PRISM III had good predictive power in our population. The area under the curve was 0.903±0.016 (p<0.001, 95% confidence interval: 0.872-0.934). Conclusions PRISM III score showed excellent accuracy and predictive ability in our population. There was no significant difference in observed and expected mortality rates in our study. In a resource-limited setting, the prediction models highlight the cases where more medical attention is required and also enable the physicians to assess the prognosis of the patient so adequate measures can be taken beforehand.

Neonatal Nonketotic Hyperglycinemia: A Rare Case from Pakistan.

Nonketotic hyperglycinemia (NKH) is an autosomal recessive disorder caused by a defect in glycine cleavage enzyme. It leads to the accumulation of glycine in the body tissues, blood, and cerebrospinal fluid (CSF). Most NKH cases are diagnosed during the natal period of life and are fatal if not promptly diagnosed and managed. Here we present a case of a two-day-old child who presented with reluctant feeding and lethargy. She had reduced tone in all four limbs and a Glasgow Coma Scale score of 9. Keeping an infectious etiology in mind, she was started on cefotaxime and amikacin. The patient was shifted to the neonatal intensive care unit; however, no improvement in the patient's condition was seen and antibiotics were changed to linezolid and meropenem along with initiation of acyclovir. The patient's blood and CSF cultures were negative. Serum amino acid chromatography showed elevated levels of glycine, and a diagnosis of NKH was made. The patient was managed symptomatically but expired on the 22nd day of admission. The case highlights the importance of prompt diagnosis and management of aminoacidopathies. Nearly all metabolic disorders have similar clinical presentations, and an early diagnosis can improve the outcome in patients.

Cardiac Manifestations of Coronavirus Disease 2019 (COVID-19): A Comprehensive Review.

Since its origin in China, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has become a pandemic and spread to 209 countries. As coronavirus disease 2019 (COVID-19) is a very rapidly emerging disease, organ-specific studies related to it have been reported. Apart from respiratory findings, some studies have highlighted inflammatory consequences in the heart, kidney, and/or liver as well. Cardiac involvement in COVID-19 seems to be a result of an inflammatory storm in response to the infection. Moreover, direct viral invasion of cardiomyocytes, as well as a myocardial injury due to oxidative stress, may account for acute cardiac injury in COVID-19. Nevertheless, the mechanism of heart injury in COVID-19 is not clear yet. However, multiple studies that highlight the clinical features, laboratory findings, and prognosis of acute myocardial injury (AMI) in COVID-19-affected individuals have been published. In this review, we have summarized the findings of all those studies as well as the clinical features and management of cardiac injury discussed by some case reports.

Adult-onset Primary Hemophagocytic Lymphohistiocytosis: Reporting a Rare Case with Review of Literature.

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, aggressive hematological syndrome. It is caused by an increased and unchecked proliferation of T lymphocytes and histiocytes. These cells secrete a large number of inflammatory cytokines and infiltrate various tissues causing multi-organ system failure. HLH may be primary or associated with different types of infections, autoimmune disorders, or malignancies. Primary or familial HLH is fatal and is frequently considered a disorder of infants and young children. Only a few cases of primary HLH have been reported in adults. We present a case of a 37-year-old man who presented with fever, pancytopenia, and hepatosplenomegaly. Lymph node biopsy showed collections of histiocytes with lymphoplasmacytic cells. After excluding all the secondary causes a final diagnosis of primary HLH was made. The patient was started on HLH-2004 protocol (etoposide, cyclosporin A, dexamethasone) along with empiric antituberculous drugs as necrotic granulomas were also noted in the biopsy. HLH has a very poor prognosis and familiarity with clinical symptoms, and diagnostic criteria can aid in timely diagnosis.

Use of Tocilizumab in COVID-19: A Systematic Review and Meta-Analysis of Current Evidence.

Background and objectives A flare-up in coronavirus disease 2019 (COVID-19) cases threatens the health of people, and though there is no proven pharmacological treatment, many analytical studies have suggested that interleukin-6 (IL-6) levels are elevated in cases of severe COVID-19 and that the anti-IL-6 biologic agent tocilizumab may be beneficial. This is a critical review of studies aiming to assess the safety and efficacy of tocilizumab as compared to the standard regimen in patients with COVID-19. Materials and methods Online databases (PubMed and Cochrane) were searched until June 29, 2020, for original articles investigating the immunological response in COVID-19 and its treatment with tocilizumab. Data on multiple baseline characteristics and pre-specified endpoints were extracted and pooled using a random effect model. We used Review Manager version 5.3 (The Nordic Cochrane Centre, The Cochrane Collaboration, 2014, Denmark) and Stata 11.0 (Stata Corporation LP, College Station, TX) for all analyses. Risk ratios (RR) and the weighted mean difference (WMD) with the corresponding 95% confidence interval (CI) were calculated. Results From a total of 1,246 identified articles, 13 studies were included after duplicate removal and narrowing based on title and abstract. Of the 13 included studies, seven case-control studies were shortlisted for meta-analysis (quantitative) and six-single arm studies were used in the discussion (qualitative). These studies had 766 patients (351 in the tocilizumab arm and 414 in the control arm). Their pooled analysis demonstrated that mortality was significantly lower in the tocilizumab group (RR=0.56 [0.34, 0.92]; p=0.02; I2=76%), as was the need for artificial invasive ventilation (RR=0.34 [0.12, 0.99]; p=0.05; I2=0%) as compared to the control group. No significant differences were observed between tocilizumab and control group in intensive care unit admissions (RR=0.73 [0.15, 3.59]; p=0.70; I2=60%) and risks of post-drug infection (RR=1.29 [0.41, 4.04]; p=0.66; I2=88%). In terms of efficacy outcome, improved oxygen saturation (RR=1.13 [1.04, 1.65]; p=0.02; I2=0%) was reported to be markedly significant in tocilizumab patients when compared with the standard care group. Conclusions Our results based on pooled studies show tocilizumab to be safe and efficacious in reducing mortality among critically ill COVID-19 patients. However, due to the limited number of observational studies, the positive findings should be viewed cautiously and warrant further investigation.