Exploring the content of epilepsy fellowship program websites: an analysis of information available to applicants.

BACKGROUND: Program websites are essential resources in the process of residency and fellowship application. We evaluated the information furnished on these resources by Epilepsy fellowship programs. The extent of information provided was compared across geographic zones, academic affiliation, and national ranking. METHODS: A list of Epilepsy fellowship programs was derived from the Fellowship and Residency Electronic Interactive Database (FREIDA). Links to program websites were obtained directly from FREIDA or using Google's search engine. Online data was categorized to reflect program information, education, recruitment, compensation, epilepsy center-specific information, and social media presence. Data points under each category were collected to develop a standardized scoring system. The frequency of criterion present was compared across geographic zones, academic affiliation, and national ranking using parametric and non-parametric statistical tests. Significance was determined at a p-value ≤ 0.05 for all cases. The study utilized IBM SPSS version 28 and Python 3.11.3. RESULTS: We analyzed 80 Epilepsy fellowship programs. The most reported feature was the program director's name and email (100.0%). The least reported features included board pass rates (1.3%), preparatory boot camp (8.8%), and post-fellowship placements (11.3%). Programs were found to be well-represented on X (88.8%), Facebook (81.3%), and Instagram (71.3%). Most (85.0%) of the programs were searchable through Google. The scores for program information, education, recruitment, compensation, epilepsy center-specific information, and social media visibility did not significantly vary based on location, academic affiliation, or rank status. CONCLUSIONS: Our results demonstrate that despite an online presence, there is much room for improvement in the content available to the applicant. To improve the Match process and attract a roster of well-informed fellows, Epilepsy fellowship programs should furnish program websites with up-to-date information relevant to program information, education, recruitment, compensation, and epilepsy center-specific information.

The time-varying cardiovascular benefits of glucagon-like peptide-1 receptor agonist therapy in patients with type 2 diabetes mellitus: Evidence from large multinational trials.

AIMS: To evaluate the time-varying cardio-protective effect of glucagon-like peptide-1 receptor agonists (GLP-1RAs) using pooled data from eight contemporary cardiovascular outcome trials using the difference in the restricted mean survival time (ΔRMST) as the effect estimate. MATERIAL AND METHODS: Data from eight multinational cardiovascular outcome randomized controlled trials of GLP-1RAs for type 2 diabetes mellitus were pooled. Flexible parametric survival models were fit from published Kaplan-Meier plots. The differences between arms in RMST (ΔRMST) were calculated at 12, 24, 36 and 48 months. ΔRMST values were pooled using an inverse variance-weighted random-effects model; heterogeneity was tested with Cochran's Q statistic. The endpoints studied were: three-point major adverse cardiovascular events (MACE), all-cause mortality, stroke, cardiovascular mortality and myocardial infarction. RESULTS: We included eight large (3183-14 752 participants, total = 60 080; median follow-up range: 1.5 to 5.4 years) GLP-1RA trials. Among GLP-1RA recipients, we observed an average delay in three-point MACE of 0.03, 0.15, 0.37 and 0.63 months at 12, 24, 36 and 48 months, respectively. At 48 months, while cardiovascular mortality was comparable in both arms (pooled ΔRMST 0.163 [-0.112, 0.437]; P = 0.24), overall survival was higher (ΔRMST = 0.261 [0.08-0.43] months) and stroke was delayed (ΔRMST 0.22 [0.15-0.33]) in patients receiving GLP-1RAs. CONCLUSIONS: Glucagon-like peptide-1 receptor agonists may delay the occurrence of MACE by an average of 0.6 months at 48 months, with meaningfully larger gains in patients with cardiovascular disease. This metric may be easier for clinicians and patients to interpret than hazard ratios, which assume a knowledge of absolute risk in the absence of treatment.

Evaluating the safety and efficacy of daprodustat for anemia of chronic kidney disease: a meta-analysis of randomized clinical trials.

PURPOSE: Anemia of chronic kidney disease (CKD) has traditionally been treated with recombinant human erythropoietin (rhEPO). Recently, daprodustat, a hypoxia-inducible factor prolyl-hydroxylase inhibitor, has also been shown to increase hematocrit. It remains unclear whether daprodustat or rhEPO should be the treatment of choice for anemia of CKD. We aimed to assess the efficacy and cardiovascular safety of daprodustat versus rhEPO in CKD patients. METHODS: Online databases were queried in April 2022 for articles comparing the efficacy and safety of daprodustat in DD-CKD and NDD-CKD subgroups. Results from trials were pooled using a random-effects model. RESULTS: Data on 8245 CKD patients from eight clinical trials were included. Our results show that in comparison to rhEPO, daprodustat maintained the same efficacy in increasing hemoglobin levels in both the DD-CKD (MD: 0.10; 95% CI [- 0.13,0.34]; p = 0.50) and NDD-CKD (MD: - 0.01; 95% CI [- 0.38,0.35]; p = 0.95) subgroups. Daprodustat significantly lowered hepcidin levels and significantly increased TIBC in both subgroups. Additionally, daprodustat significantly reduced the incidence of major adverse cardiovascular events (MACE) (RR: 0.89; 95% CI: 0.89-0.98; p = 0.02) and its myocardial infarction (MI) component (RR: 0.74; 95% CI: 0.59-0.92; p = 0.006) in the DD-CKD subgroup. CONCLUSION: Daprodustat has similar efficacy compared to rhEPO for the treatment of anemia of CKD. On treatment, the reduced experience of MACE was reported in DD-CKD patients as compared to rhEPO. Furthermore, effects on iron metabolism varied by parameter, with daprodustat being superior to rhEPO in some cases and inferior in others.

Risk Calculator to Predict 30-Day Readmission After Coronary Artery Bypass: A Strategic Decision Support Tool.

BACKGROUND: Re-admission is an important source of patient dissatisfaction and increased hospital costs. A simple calculator to determine the probability of re-admission may help guide patient dismissal planning. METHODS: Using the national readmissions database (NRD), we identified admissions for isolated primary coronary artery bypass (CABG) and stratified them according to 30-day readmission. Including pre, intra and postoperative variables, we prepared a logistic regression model to determine the probability for re-admission. The model was tested for reliability with boot-strapping and 10-fold cross-validation. RESULTS: From 135,699 procedures, 19,355 were readmitted at least once within 30days of dismissal. Patients who were readmitted were older (67±10 vs 65 ± 10 years, p<0.01), females (32% vs 24%; p<0.01) and had a higher Elixhauser comorbidity score (1.5±1.4 vs 1.1±1.2; p<0.01). Our final model (c- statistic=0.65) consisted of 16 pre and three postoperative factors. End-stage renal disease (OR 1.79 [1.57-2.04]) and length of stay>9days (OR 1.60 [1.52-1.68]) were most prominent indicators for readmission. Compared to Medicaid beneficiaries, those with private insurance (OR 0.62 [0.57-0.68]) and Medicare (OR 0.85 [0.79-0.92]) coverage were less likely to be readmitted. CONCLUSIONS: Our simple 30-days CABG readmission calculator can be used as a strategic tool to help reduce readmissions after coronary artery bypass surgery.

Comparative efficacy of dexamethasone versus surgery for chronic subdural hematoma: A systematic review and meta-analysis.

OBJECTIVE: To assess the comparative efficacy of dexamethasone (DXM) as monotherapy in comparison to surgery among the patients of chronic subdural hematoma (CSDH). METHODS: We searched MEDLINE, PUBMED, EMBASE, and Cochrane Central Register of Controlled Trials databases from inception till September 2023. Data was extracted, pooled and analyzed from all the studies that assessed the comparative efficacy of DXM as monotherapy in contrast with surgery as the primary treatment of CSDH. RESULTS: A total of 6 studies involving 704 patients were included in our meta-analysis. Comparison of surgery to DXM revealed there was no statistically significant difference between the two groups regarding mortality [RR=1.09; 95% CI; 0.52-2.28 P = 0.83]. However, a significantly higher incidence of secondary surgical intervention was observed in the DXM group [RR 4.24; 95% CI; 2.06-8.71 P < 0.0001]. No significant difference in performance was observed in terms of poor postoperative outcomes within hospital stay [RR 1.12, 95% CI, 0.40-3.19 P=0.83] and at 6 months [RR 0.92, 95%CI, 0.40-2.13 P=0.85]. CONCLUSION: DXM had a significantly higher incidence of secondary surgical intervention. However, there was no difference regarding mortality and other safety outcomes between surgery and DXM for the patients with CSDH. Observational studies showed that DXM was associated with a lower risk of poor postoperative outcomes within hospital stay and had shorter duration of hospital stay, but the recurrence rate was lower in the surgery group.

Long-term efficacy of spinal cord stimulation for chronic primary neuropathic pain in the contemporary era: a systematic review and meta-analysis.

INTRODUCTION: Spinal cord stimulation (SCS) is a modern neuromodulation technique extensively proven to be an effective modality for treatment of chronic neuropathic pain. It has been mainly studied for complex regional pain syndrome (CRPS) and failed back surgery syndrome (FBSS) and recent data almost uniformly establishes its statistically significant positive therapeutic results. It has also been compared with other available treatment modalities across various studies. However, long term data on maintenance of its efficacious potential remains less explored. Few studies have reported data on long follow-up times (>= 12 months) and have compared its efficacy with other treatment options for chronic pain, respectively. Our study pools and analyzes the available data and compares SCS with other treatment options. It also analyzes the efficacy of SCS in long term management of patients with chronic pain. EVIDENCE ACQUISITION: We reviewed all the data available on MEDLINE, Embase and Cochrane CENTRAL using a search strategy designed to fit our pre-set inclusion and exclusion criteria. Both single-arm and double-arm studies were included. The primary outcome was defined as decrease of visual analogue scale (VAS) by >50% at 6, 12 and/or 24 months after SCS. EVIDENCE SYNTHESIS: According to the pooled data of double-arm studies, SCS has unanimously proven its superiority over other treatment options at 6 months follow-up; however it fails to prove statistically significant difference in results at longer treatment intervals. Dorsal root ganglion stimulation, a relatively recent technique with the same underlying physiologic mechanisms as SCS, showed far more promising results than SCS. Single-arm studies show around 70% patients experiencing greater than 50% reduction in their VAS scores at 6 and 12 months. CONCLUSIONS: SCS is a viable option for management of chronic neuropathic pain secondary to FBSS and CRPS. However, data available for its long term efficacy remains scarce and show no further statistically significant results.

Is Using the Harmonic Scalpel Better than Conventional Hemostasis in Neck Dissection? A Meta-Analysis.

Study Design: Systematic review and meta-analysis. Objective: The clinical decision to pursue harmonic scalpel (HS) method vs conventional hemostasis to treat head and neck cancers has been arguably predicated on the clinical outcomes observed. This study aims to evaluate the surgical outcomes of neck dissection between both techniques and perform an updated meta-analysis using the available literature. Methods: We searched PubMed, Scopus, and Cochrane Library through 31st December 2021, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Outcome metrics included operative time and intraoperative blood loss. Secondary outcomes consisted of length of hospital stay, length of drain stay, total drain output, and postoperative complications. A meta-analysis was conducted using Review Manager Version 5.3 (RevMan) software employing the Random Effects Model. Results: We identified 114 articles, out of which 10 randomized control trials (RCTs) analyzing a combined total of 558 patients met the inclusion criteria after title and full-text screening. Meta-analysis shows the group treated with HS had a significantly shorter operative time. [MD = -23.21, 95% CI (-34.30, -12.12) P value <.0001 I2 = 92%] but an insignificant lesser intraoperative blood loss [MD = -61.53, 95% CI (-88.61, -34.45) P < .00001 I2 = 79%]. Conclusions: This study confirms that that HS use in neck dissection yields a reduced operative time and intra operative blood loss relative to conventional hemostasis. Furthermore, our paper shows no superiority of HS method over conventional hemostasis where length of hospital stays, length of drain stays, and postoperative complications are concerned. Future RCTs with high-level evidence may further elucidate the relative effectiveness of HS method over conventional hemostasis in treating head and neck cancers.

Evaluating the Safety and Efficacy of Erythropoietin Therapy for Neonatal Hypoxic-Ischemic Encephalopathy: A Systematic Review and Meta-Analysis.

BACKGROUND: Erythropoietin (EPO) is a proposed drug for the treatment of neonatal hypoxic-ischemic encephalopathy (HIE). Multiple studies have linked its use, either as a monotherapy or in conjunction with therapeutic hypothermia (TH), with improved neonatal outcomes including death and neurodisability. However, there is also evidence in the literature that raises concerns about its efficacy and safety for the treatment of neonatal encephalopathy (NE). METHODS: We searched MEDLINE, Cochrane CENTRAL, and Embase for both observational studies and randomized controlled trials (RCTs) investigating the effectiveness of EPO in treating NE. Only studies in which at least 300 U/kg of EPO was used and reported any one of the following outcomes: death, death or neurodisability, and cerebral palsy, were included. RESULTS: Seven studies with 903 infants with the diagnosis of NE were included in our meta-analysis. EPO did not reduce the risk of death or neurodisability (risk ratio 0.68 [95% confidence interval [CI]: 0.43 to 1.09]) (P = 0.11). Similarly, the risk of cerebral palsy was not reduced by the administration of EPO (risk ratio 0.68 [95% CI: 0.33 to 1.40]) (P = 0.30). The risk of death was also not reduced at any dose of EPO regardless of the use of TH. CONCLUSIONS: The results of our meta-analysis do not support the use of EPO for the treatment of neonatal encephalopathy. However, future large-scale RCTs are needed to strengthen these findings.

Incidence of acute kidney injury in patients with acute ischaemic stroke undergoing CT angiography (CTA) and CT perfusion (CTP): a systematic review and meta-analysis.

Background and purpose: We conducted a systematic review and meta-analysis to assess the incidence of acute kidney injury (AKI) in patients undergoing CT angiography (CTA) and CT perfusion (CTP) for acute ischaemic stroke (AIS). Concerns over contrast-induced nephropathy (CIN) often lead medical centres to mandate pre-imaging serum creatinine level assessments, causing unnecessary delays. We aim to confirm further the practice of conducting CTA/CTP without first testing creatinine. Methods: We searched PubMed, Cochrane Central and Scopus from inception until March 2023 for studies reporting on AKI in patients with AIS receiving CTA/CTP. Outcomes of interest were (1) the odds of AKI in patients receiving CTA/CTP versus non-contrast CT and (2) the overall incidence of AKI and haemodialysis in patients with AIS undergoing CTA/CTP. Results: Results were pooled using a random effects model. 13 studies were included (5 cohort and 8 single-arm studies) with 5104 patients in total, out of which 4347 patients received CTA/CTP and 757 patients received no contrast. In case-control studies, 4.8% (OR=0.66, 95% CI 0.35 to 1.22, Z=1.32, p=0.19) of patients who received CTA/CTP developed AKI, compared with 7.7% of patients in the control group. Temporary haemodialysis was required for two patients in the analysed studies. Conclusions: Non-randomised evidence suggests that CTA/CTP is not associated with a statistically significant increase in the risk of AKI in patients with stroke. Further well-designed prospective studies are required to explore potential risk factors of CIN in specific patient populations such as diabetes mellitus and chronic kidney disease.

Association of bilateral oophorectomy with incidence of Parkinson's disease: A systematic review and meta-analysis.

INTRODUCTION: Current evidence in the literature is inconclusive due to conflicting results with regards to an association between B/L (B/L) oophorectomy and Parkinson's disease (PD). We included large, powered studies to assess the association of PD in women who have undergone B/L oophorectomy. METHODS: We conducted a comprehensive search across three databases from inception to October 2022 for observational studies including pre-menopausal or post-menopausal women undergoing B/L oophorectomy. Primary outcome of interest was incidence of PD or parkinsonism. The results for these associations were presented as Risk Ratios (RR) with 95% confidence intervals (CI), which were pooled using a generic invariance weighted random effects model using Review Manager (RevMan). RESULTS: Data was included from a total of 4 studies. No significant association was found between B/L oophorectomy and PD (RR: 1.38; 95% CI: 0.76 to 2.49; I2:89 %) in contrast significant association was found with parkinsonism (RR: 1.80; 95% CI: 1.29 to 2.52). Age at surgery didn't significantly affect Parkinsonism incidence (RR: 0.88; 95% CI: 0.59 to 1.3). No significant association was found between ovarian indication and Parkinsonism (RR: 1.08; 95% CI: 0.69 to 1.68). B/L oophorectomy with hysterectomy was associated with higher Parkinson's risk compared to without hysterectomy (RR: 1.4; 95% CI: 1.13 to 1.74). Lastly, there was no significant association between Post Menopausal Hormonal (PMH) use and Parkinson's disease (RR: 1.07; 95% CI: 0.92 to 1.26). CONCLUSION: Our findings suggest that B/L oophorectomy is significantly associated with the incidence of Parkinsonism. Further research is needed to understand the potential relationship between oophorectomy and Parkinson's disease.

Is it safe and effective to administer COVID-19 vaccines during pregnancy? A systematic review and meta-analysis.

OBJECTIVE: To evaluate the safety and efficacy of COVID-19 vaccines in pregnant women performing an updated meta-analysis. METHODS: We searched PubMed, Cochrane Central, and SCOPUS from inception to March 2022. Outcomes of interest were incidence of adverse maternal, fetal and neonatal consequences pertaining to safety of the vaccines. Secondarily, we analyzed the number of SARS-CoV-2 infections, hospitalization for COVID-19, and admission to the I.C.U. for COVID-19 assessing the effectiveness of vaccines. Results were pooled using a random effects model. RESULTS: Ten observational studies (n=326,499) analyzing pregnant women were included. Our results suggest that COVID-19 vaccination prevents infection (OR: 0.56, 95% CI: 0.47, 0.67; P = <0.00001) and related hospitalizations (OR: 0.50, 95% CI: 0.31, 0.82; P = 0.006) effectively. It was also observed that vaccination does not change adverse outcomes in pregnancy, namely preeclampsia or eclampsia, stroke (four weeks of delivery), meconium-stained amniotic fluid, spontaneous vaginal delivery, operative vaginal delivery, cesarean delivery, postpartum hemorrhage, and blood transfusions. Furthermore, the vaccine was observed to be protective against neonatal COVID-19 I.C.U. admissions (OR: 0.85; 95% CI: 0.81, 0.90; P = <0.00001). CONCLUSION: Our pooled analysis suggests that the COVID-19 vaccination in pregnant women prevents infection effectively and has no adverse outcomes. Future large-scale trials in a randomized fashion are needed to confirm our results.

Comparison of sodium-glucose cotransporter-2 inhibitors and thiazolidinediones for management of non-alcoholic fatty liver disease: A systematic review and meta-analysis.

BACKGROUND: The pharmacologic treatment of non-alcoholic fatty liver disease (NAFLD) remains unclear. METHODS: Two reviewers searched PubMed, SCOPUS, Cochrane Central and clinicaltrials.gov for randomized controlled trials (RCTs) of patients with NAFLD with or without type 2 diabetes mellitus (T2DM) receiving TZDs vs SGLT2 inhibitors. The primary outcomes were change in alanine aminotransferase (ALT), aspartate aminotransferase (AST) and gamma-glutamyl transferase (GGT) and improvement in steatosis and fibrosis. The secondary outcomes were changes in lipid profile, body weight and glycated hemoglobin (HbA1c). Random effects models with continuous outcomes as weighted mean differences (WMD) with 95% confidence intervals (CI) were used. RESULTS: Five studies (n = 311 NAFLD patients) were included. Patients treated with SGLT2 inhibitors (n = 156) showed significant decrease in visceral fat area (VFA; WMD 23.45, p < 0.00001) and body weight (WMD 4.22, p < 0.00001) as compared to those treated with TZDs (n = 155). Patients from both groups showed improvement in AST (WMD 1.21, p = 0.40), ALT (WMD -0.46, p = 0.81), GGT (WMD -0.47, p = 0.84), hepatic fibrosis (WMD 0.11, p = 0.52), LDL (WMD 2.19, p = 0.35), HbA1c (WMD -0.16%, p = 0.20), HOMA-IR (WMD: -0.04, p = 0.91) and FPG (WMD -7.37, p = 0.28) which was equivalent and non-significant. CONCLUSION: The improvement in liver enzymes, steatosis and fibrosis caused by SGLT2 inhibitors and TZDs was similar. SGLT2 inhibitors, however, resulted in a significant decrease in VFA and body weight. As weight loss is found to have a positive effect on the resolution of steatosis and fibrosis in NAFLD patients, SGLT2 inhibitors may have the potential to be considered for long-term management, however, further research needs to be conducted to determine the utility of SGLT2 inhibitor class of antidiabetic drugs for effectively treating NAFLD.

Effective Community-Based Interventions for the Prevention and Management of Heat-Related Illnesses: A Scoping Review.

BACKGROUND: Extreme temperatures have negative consequences on the environment, ecosystem, and human health. With recent increases in global temperatures, there has been a rise in the burden of heat-related illnesses, with a disproportionate impact on low- and middle-income countries. Effective population-level interventions are critical to a successful public health response. OBJECTIVE: This scoping review aims to summarize the evidence on the effectiveness of population-level heat-related interventions and serve as a potential guide to the implementation of these interventions. METHODS: Studies that evaluated the effectiveness of community-based interventions to mitigate or reduce the impact of extreme heat on heat-related mortality and morbidity were sought by searching four electronic databases. Studies published in the English language and those that had quantifiable, measurable mortality, morbidity or knowledge score outcomes were included. RESULTS: The initial electronic search yielded 2324 articles, and 17 studies were included. Fourteen studies were based in high-income countries (HICs) (Europe, US, Canada) and discussed multiple versions of (1) heat action plans, which included but were not limited to establishing a heat monitoring system, informative campaigns, the mobilization of health care professionals, volunteers, social workers and trained caregivers in the surveillance and management of individuals with known vulnerabilities, or stand-alone (2) education and awareness campaigns. Multi-pronged heat action plans were highly effective in reducing heat-related mortality and morbidity, especially among vulnerable populations such as the elderly and those with chronic conditions. CONCLUSIONS: The heat action plans covered in these studies have shown promising results in reducing heat-related mortality and morbidity and have included instituting early warning systems, building local capacity to identify, prevent or treat and manage heat-related illnesses, and disseminating information. Nevertheless, they need to be cost-effective, easy to maintain, ideally should not rely on a mass effort from people and should be specifically structured to meet the local needs and resources of the community.

How well do we represent ourselves: an analysis of cardiology fellowships website content.

Background: The Internet is the primary source of information for prospective cardiology fellowship aspirants. The objective of this study was to evaluate cardiology fellowship programs' online profile. Materials & methods: Two independent reviewers accessed 221 US based cardiology fellowship program websites obtained through Fellowship and Residency Electronic Interactive Database for pre-selected 20 criteria. The update status of websites was assessed using 6-point criteria. Results: Only 25 (11.3%) websites were fully up-to-date; 23 (10.4%) fulfilled 80% of the 20-point criteria and 85 (38.5%) program websites had fewer than 50% of the criteria listed. Conclusion: Most cardiology fellowship program websites lack crucial details. In this technology driven age, efforts should be made to ensure updated websites.

Reining in Sternal Wound Infections: The Achilles' Heel of Bilateral Internal Thoracic Artery Grafting.

Background: Although the survival advantage of bilateral internal thoracic artery grafting (BITA) is well known in patients undergoing coronary artery bypass grafting (CABG), this technique has not been widely adopted. This is mainly because of the increased risk of deep sternal wound infections (DSWI) associated with its use. However, in recent years the overall risk of DSWI has decreased. This is mainly because of strategies that have been adopted to decrease the risk of these infections in patients undergoing CABG. Conclusion: In this review we identified DSWI preventive strategies and described them in detail so that their use by surgeons can be increased. This would minimize the risk of DSWI after BITA grafting and maximize the use of this highly effective surgical technique.

Elaborate Tongue Fasciculations Going Down to the Neck: A Rare Case of Sporadic, Young-onset Amyotrophic Lateral Sclerosis with Bulbar Symptoms, from Pakistan.

Amyotrophic lateral sclerosis (ALS) is a rare, progressive neurodegenerative disease, part of the spectrum of motor neuron diseases. This disease is divided on the bases of heritability, with majority of the cases being sporadic and phenotype, with eight recognized patterns-each with its respective symptoms, rate of progression, and prognosis. Here, we report a case of sporadic, bulbar-onset ALS, unique in its presentation as our patient had fully progressed bulbar symptoms, at the age of 28 years-where other cases of bulbar ALS are associated with much older ages and have a predisposition for the female gender. His prominent and elaborate tongue fasciculations going all the way down to the neck and rendering him incapable of holding his tongue out made for an additional reason of our special interest in the case and the keenness to report it.

Infiltrating the Heart and Kidney: A Rare Pediatric Case of Multisystem Langerhans Cell Histiocytosis from Pakistan.

Langerhans cell histiocytosis (LCH) is a rare, clonal disease of the monocyte-macrophage system, varying in its clinical presentation from mere self-healing skin and bone lesions to life-threatening multi-system disease. In descending order of frequency, the disease is known to involve the skeleton, skin, lymph nodes and lesser often, the liver, spleen, lungs, hematopoietic and central nervous systems. Here, we present a pediatric case of multi-system LCH in a five-year-old child, unique in its evident cardiac and renal involvement alongside other organ systems and important in how the diagnosis was aided by a fine needle aspiration cytology instead of the costlier histopathological procedures, in a setting with limited resources.

Affected Children of Healthy Parents: Multiple Pediatric Cases of Autosomal Recessive Charcot-Marie-Tooth Disease in a Pakistani Family.

Charcot-Marie-Tooth (CMT) disease, also referred to as hereditary motor and sensory neuropathy (HMSN), is a heterogeneous group of disorders which primarily affects the peripheral nervous system. Clinically, the main features are progressive muscle weakness seen distally, along with wasting seen predominantly in the anterior compartments of the lower legs. The disease can broadly be classified into two groups, CMT1 and CMT2-based on inheritance patterns, paired with anatomical or electrophysiological findings. It can be inherited in the autosomal dominant, X-linked and rarely, the autosomal recessive fashions. Here, we present an unusual case of autosomal recessive CMT disease, in four out of six children of unaffected parents in a family.

Sulfasalazine-induced Pancytopenia Indicating Bone Marrow Suppression: A Rare Pediatric Case Report from Pakistan.

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic condition in children. The treatment of JIA is mainly by drug therapy, which includes non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and disease-modifying antirheumatic drugs (DMARDs). Sulfasalazine is a DMARD that is used as the second-line of therapy. Although believed to have an effective and safe profile, it has side effects ranging from mild gastrointestinal discomfort to hematopoietic alterations. In this study, we present a case of JIA with sulfasalazine-induced bone marrow suppression in a five-year-old child, which is rarely reported within the pediatric age group across the literature.

Paving a Shorter Path Towards Diagnosis: A Case on Adult Onset Still's Disease from Pakistan.

Adult onset Still's disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, erythematous rash, and other systemic presentations. We report a case of a 21-year-old male who presented with high spiking fever, dry cough, generalized body ache, arthralgia, and an erythematous rash. He was eventually diagnosed to have AOSD based on the Yamaguchi criteria, after a month of visiting three different healthcare facilities and receiving two misdiagnoses and treatment regimes not specific to his diagnosis. The patient immediately responded to prednisoloneand was healthy upon discharge.