Cardiac sarcoma: A rare case of primary cardiac sarcoma.

INTRODUCTION AND IMPORTANCE: Primary cardiac sarcoma is a rare malignancy with a poor prognosis because of diagnostic delay, therapeutic difficulties, and high metastatic potential. The therapeutic approach includes surgery, chemotherapy, and radiation therapy, alone or in combination. However, there is a lack of evidence to guide the treatment. CASE PRESENTATION: We present a case of primary cardiac sarcoma. Our patient was presented in the department of emergency medicine (ED) in our institute with shortness of breath on exertion associated with orthopnea. Based on the history and cardiovascular examination, he underwent an echocardiogram, which revealed a sizeable echogenic density in the right ventricular outflow tract. He underwent surgical resection of the cardiac mass via median sternotomy and total cardiopulmonary bypass approach. The patient was eventually diagnosed with primary cardiac sarcoma, confirmed by tissue biopsy after surgical intervention. CLINICAL DISCUSSION: Through this report, we highlight the rarity of primary cardiac sarcomas, the importance of multidisciplinary tumor board (MDT) discussion and provide evidence of surgical excision being the treatment of choice, followed by systemic chemotherapy in selected cases. CONCLUSION: Cardiac sarcoma is a rare but highly malignant tumor with a poor prognosis. However, early diagnosis and surgical resection of a primary cardiac sarcoma can significantly increase the patient's survival and quality of life. Therefore, physicians should keep a high suspicion of a patient with clinical features suggestive of cardiac sarcoma, and echocardiography should be the diagnostic modality of choice in such patients.

Pro-Inflammatory and Immunological Profile of Dogs with Myxomatous Mitral Valve Disease.

Myxomatous mitral valve disease (MMVD) is a very frequently acquired cardiac disease in dog breeds and is responsible for congestive heart failure (CHF). The involvement of the immune system and pro-inflammatory cytokines in dogs with CHF due to mitral valve disease has not yet been extensively investigated. Here, we investigate the role of pro-inflammatory cytokines and the dysfunction of the immune system in dogs with different stages of severity through the blood assessment of CD4+FoxP3+regulatory T cells (Treg) cells, leptin, tumor necrosis factor (TNF)-α, interleukin (IL)-1ß and IL-6 pro-inflammatory cytokines, and immunological and echocardiographic parameters. A total of 36 cardiopathic dogs, 14 females and 22 males, with MMVD were included. Mean age and body weight (BW) at the time of enrollment were 10.7 ± 2.77 years and 10.9 ± 6.69 kg, respectively. For the comparison of the pro-inflammatory and immunological parameters, two groups of healthy dogs were also established. Control group 1 consisted of young animals (n. 11; 6 females and 5 males), whose age and mean weight were 4.1 ± 0.82 years and 13.8 ± 4.30 kg, respectively. Control group 2 consisted of elderly dogs (n. 12; 6 females and 6 males), whose age and BW were 9.6 ± 0.98 years and 14.8 ± 6.15 kg, respectively. Of particular interest, an increase in Treg cells was observed in the cohort of MMVD dogs, as compared to the healthy dogs, as Treg cells are involved in the maintenance of peripheral tolerance, and they are involved in etiopathogenetic and pathophysiological mechanisms in the dog. On the other hand, TNF-α, IL-1ß, and IL-6 significantly increased according to the severity of the disease in MMVD dogs. Furthermore, the positive correlation between IL-6 and the left ventricle diastolic volume suggests that inflammatory activation may be involved in cardiac remodeling associated with the progressive volumetric overload in MMVD.

Inactivated COVID-19 vaccine triggering hemophagocytic lymphohistiocytosis in an immunocompetent adult - A case report.

Background and Aim: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that is induced by hyper-activated macrophages, cytotoxic T cells, and reduced natural killer cell activity. A 46-year-old gentleman presented to us with complaints of intermittent fever for the past 2 weeks associated with fatigue along with oral ulcers and skin rashes which resolved spontaneously. These symptoms started after he received the second dose of the BBIP-CorV COVID-19 vaccine. His complete blood picture showed pancytopenia. A detailed infectious disease workup was unrevealing; however, his bone marrow biopsy revealed increased histiocyte activity, with some showing hemophagocytosis and dysplasia. Immunohistochemistry profile demonstrated strong CD 68 positivity. Further investigations showed raised serum ferritin and fasting triglyceride levels. He was immediately started on dexamethasone acetate at a dose of 10 mg/m2, after which his clinical symptoms, as well as his blood parameters, improved remarkably. This is the first documented case in Pakistan. Conclusion: The data from clinical trials support the general safety profile of inactivated COVID-19 vaccines. We endorse its mass implementation. However, we believe that robust data need to be generated to evidence any adverse events, especially those with serious outcomes. Physicians should be aware of inactivated COVID-19 vaccine as a possible trigger for HLH and start prompt treatment, resulting in favorable outcomes. Relevance for Patients: The presentation of HLH may vary and can present in an immunocompetent patient with no underlying risk factor. HLH should be kept in differentials when a patient presents with pancytopenia with a recent history of receiving COVID-19 vaccination. Steroids play a major role in the treatment of HLH, and definitive diagnosis and early treatment improve clinical outcomes.

Inactivated COVID-19 vaccine triggering hemophagocytic lymphohistiocytosis in an immunocompetent adult - A case report.

Background and Aim: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that is induced by hyper-activated macrophages, cytotoxic T cells, and reduced natural killer cell activity. A 46-year-old gentleman presented to us with complaints of intermittent fever for the past 2 weeks associated with fatigue along with oral ulcers and skin rashes which resolved spontaneously. These symptoms started after he received the second dose of the BBIP-CorV COVID-19 vaccine. His complete blood picture showed pancytopenia. A detailed infectious disease workup was unrevealing; however, his bone marrow biopsy revealed increased histiocyte activity, with some showing hemophagocytosis and dysplasia. Immunohistochemistry profile demonstrated strong CD 68 positivity. Further investigations showed raised serum ferritin and fasting triglyceride levels. He was immediately started on dexamethasone acetate at a dose of 10 mg/m2, after which his clinical symptoms, as well as his blood parameters, improved remarkably. This is the first documented case in Pakistan. Conclusion: The data from clinical trials support the general safety profile of inactivated COVID-19 vaccines. We endorse its mass implementation. However, we believe that robust data need to be generated to evidence any adverse events, especially those with serious outcomes. Physicians should be aware of inactivated COVID-19 vaccine as a possible trigger for HLH and start prompt treatment, resulting in favorable outcomes. Relevance for Patients: The presentation of HLH may vary and can present in an immunocompetent patient with no underlying risk factor. HLH should be kept in differentials when a patient presents with pancytopenia with a recent history of receiving COVID-19 vaccination. Steroids play a major role in the treatment of HLH, and definitive diagnosis and early treatment improve clinical outcomes.

Designing a novel mRNA vaccine against Vibrio harveyi infection in fish: an immunoinformatics approach.

Vibrio harveyi belongs to the family Vibrionaceae of class Gammaproteobacteria. Around 12 Vibrio species can cause gastroenteritis (gastrointestinal illness) in humans. A large number of bacterial particles can be found in the infected cells, which may cause death. Despite these devastating complications, there is still no cure or vaccine for the bacteria. As a result, we used an immunoinformatics approach to develop a multi-epitope vaccine against the most pathogenic hemolysin gene of V. harveyi. The immunodominant T- and B-cell epitopes were identified using the hemolysin protein. We developed a vaccine employing three possible epitopes: cytotoxic T-lymphocytes, helper T-lymphocytes, and linear B-lymphocyte epitopes, after thorough testing. The vaccine was developed to be antigenic, immunogenic, and non-allergenic, as well as have a better solubility. Molecular dynamics simulation revealed significant structural stiffness and binding stability. In addition, the immunological simulation generated by computers revealed that the vaccination might elicit immune reactions Escherichia coli K12 as a model, codon optimization yielded ideal GC content and a higher codon adaptation index value, which was then included in the cloning vector pET2+ (a). Altogether, our experiment implies that the proposed peptide vaccine might be a good option for vibriosis prophylaxis.

Effects of Neodymium-Doped Yttrium Aluminium Garnet (Nd:YAG) Laser Capsulotomy on Visual Outcomes From a Lower-Middle Income Country.

Objective Neodymium-doped yttrium aluminium garnet (Nd: YAG) laser is a non-invasive and effective means to deal with posterior capsule opacification. Although it is safe, it may have some complications. The purpose of this study was to evaluate the efficacy of Nd: YAG laser capsulotomy in terms of visual outcomes. Methodology This retrospective study was carried out at the eye department of Shaheed Mohtarma Benazir Bhutto Medical College, Lyari and Sindh Government Lyari General Hospital, Karachi, by using a convenient sampling technique. The duration of the study was six months from 1st January 2020 to 30th June 2020. 50 eyes of patients older than 20 years of age of either gender with posterior capsule opacification after cataract surgery for more than 6 months of duration, capsular fibrosis, and visual distortion due to wrinkling were included in the study.  Results Our results show that in a total of 50 patients, the mean age was 59.08±5.84 years, of which, 20 (40%) were males. Out of 50 patients, 22 right while 28 left eyes were selected for Nd: YAG laser capsulotomy. None of the patients showed elevated intraocular pressure (IOP) after the 1st week. Mean IOP was 16.84±3.63 mm of Hg on the 1st day and mean IOP after 1st week was 12.48±2.01 mm of Hg. Iritis was observed in 5 (10.0%) patients on the 1st day and 4 (8.0%) patients on the 1st week. Raised IOP was observed in 10 (20.0%) cases whereas cystoid macular edema was observed in only 1 (2%) patients on the 1st day and 1st week after laser therapy. Conclusion The study predicted that Nd: Yag laser posterior capsulotomy gives excellent results in terms of visual acuity. Complications that were associated with the Nd: Yag laser capsulotomy was a rise in intraocular pressure, cystoid macular edema, iritis, and IOL pitting.

Infective Endocarditis Secondary to Mycoplasma pneumoniae.

Mycoplasma pneumoniae (MP) is a gram-positive bacterium most commonly associated with community-acquired pneumonia in adults. It can also involve other systems of the body. Cardiovascular complications include pericarditis, myocarditis, congestive cardiac failure, and, rarely, infective endocarditis. We report a case of infective endocarditis secondary to MP infection in an adult. We treated our patient with doxycycline, which showed significant improvement.

Primary Central Nervous System T-cell Lymphoma Associated With Hepatitis B and D Virus Coinfection.

Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin's lymphoma. It is defined as lymphoma of the central nervous system without any systemic disease elsewhere at the time of diagnosis. Based on the phenotypical features, it is divided into two categories, B-cell and T-cell, with the latter being less common. Viruses, such as human immunodeficiency virus (HIV) and human T-lymphotropic virus type 1 (HTLV-1), are linked to the T-cell variant; however, there is a lack of evidence suggesting associating hepatitis B and D virus coinfection with it. We report a case of a 34-year-old male who presented with T-cell PCNSL and was later diagnosed with a hepatitis B and D virus coinfection.

Posterior Reversible Encephalopathy Syndrome Secondary to Varicella Encephalitis.

Posterior reversible encephalopathy syndrome (PRES) is a rapidly progressive neurologic condition presenting with typical symptoms including headache, nausea, vomiting, altered mental status, and visual defects. Neuroimaging profile, particularly magnetic resonance imaging (MRI), is the most important tool for diagnosis. The most commonly reported etiological factors include hypertensive emergency and renal disease. We describe a 67-year-old lady who developed clinical and radiological characteristics of PRES secondary to Varicella encephalitis.

Traumatic brain injury causing intestinal dysfunction: A review.

Traumatic brain injuries (TBI) and its sequelae are becoming one of the most pressing public health concerns worldwide. It is one of the leading causes of increased morbidity and mortality. The primary insult to the brain can cause ischemic brain injury, paralysis, concussions, death, and other serious complications. Brain injury also involves other systems through a secondary pathway resulting in multiple complications during and after hospitalization. The focus of our article is to assess the literature available on traumatic brain injury and intestinal dysfunctional to highlight the aspects of epidemiology, pathophysiology, and different diagnostic approaches for early diagnosis of gut dysfunction. We review studies done in both humans and animals, to better understand this underrated topic, as it costs billions of dollars to the healthcare industry because of delayed diagnosis.

Elective Tracheostomy in Critically Ill Children: A 10-Year Single-Center Experience From a Lower-Middle Income Country.

Objective Tracheostomy is a commonly performed procedure amongst critically ill patients, especially in cases of prolonged mechanical ventilation (PMV). This study aimed to describe the indications, clinical characteristics, and outcomes of elective pediatric tracheostomies in critically ill children at our center. Methods A retrospective review of medical records of children who underwent elective tracheostomies in our pediatric intensive care unit (PICU) was conducted from January 2009 to June 2018. Data were extracted based on demographics, indications of tracheostomy, and patient outcomes. Results were reported as mean with standard deviation and as frequencies with percentage. Results Of the 3,200 patients admitted to the PICU during the study period, 1,130 were intubated. A total of 48 (4.2% of 1,130) children underwent an elective tracheostomy. 30/48 (62.5%) children had an early tracheostomy. 34/48 (71%) patients were males. Approximately 25% of our patients undergoing a tracheostomy had an underlying neurological condition as the primary diagnosis, followed by respiratory conditions (23%). The most common indications for elective tracheostomy were PMV (>7 days) (n=24, 50%) and extubation failure (n=9, 18.7%). Early tracheostomy (<14 days) had better patient outcomes in terms of ventilator-free days (8.57±4.64 in early tracheostomy vs. 6.38±6.17 days in late tracheostomy, P=0.04). The sedation-free days and ICU-free days were also significantly increased in the early tracheostomy group than in the late tracheostomy group. The successful weaning and ICU discharge rate were significantly higher in the early tracheostomy group than in the late tracheostomy group (78.1% vs. 59.7%, P<0.05; and 69.2% vs. 49.5%, P<0.05, respectively). Ventilator-associated pneumonia was more common in the late tracheostomy group (n= 14, 77%), compared to early tracheostomy group (n=12, 40%) (P=0.03). Two patients expired from tracheostomy-related complications. Conclusion PMV was the most common indication for an elective tracheostomy. Early tracheostomy is associated with improved patient outcomes; therefore, a standardized approach toward mechanically ventilated children is recommended.

Morbidity and Mortality Associated With Pediatric Critical Mediastinal Mass Syndrome.

Objective The critical mediastinal mass syndrome (CMMS) is a life-threatening condition and is challenging for physicians. We analyse the clinicopathological profile and outcome of CMMS from a large tertiary-care pediatric oncology center in Pakistan. Methods We retrospectively reviewed the medical record of a tertiary-care hospital in Pakistan from April 2017 to September 2019 for all children (1 month-16 years) who presented with an anterior mediastinal mass (AMM). A CMMS case is defined as a child with an AMM presenting with cardiorespiratory compromise and needing intensive care support. Demographic data, clinical profile, pathological diagnosis, and outcome of all such children were recorded. Descriptive statistics were applied using the Statistical Package for the Social Sciences (SPSS), version 22 (IBM Corp., Armonk, NY). Results Of the total 221 mediastinal masses, 61 children were diagnosed as CMMS and enrolled in the study. The mean age was 9 ± 3.3 years, and 68.9%% were male; 65.6% of patients had a weight for age less than the fifth percentile. A total of 49.2% of patients had a duration of illness of more than one month before diagnosis. Fever (97.6%) and lymphadenopathy (82%) were the most common findings, along with respiratory and cardiovascular signs and symptoms; 9.8% had superior vena cava syndrome. The pericardial effusion was present in 54.6% and 27.9% had pleural effusion. Peripheral blood flow cytometry made the diagnosis in 59%, peripheral lymph node biopsy in 13%, mediastinal core biopsy in 5%, and pleural fluid flow cytometry in one case; 62.3% had a white blood cell count of >100,000/mm3. A total of 72.1% (n=44) cases were diagnosed as T-cell acute lymphoblastic leukemia in our cohort. Clinical and laboratory tumor lysis syndrome developed in 10% and 73% of cases, respectively. Mechanical ventilation was required in 9.8% of the cohort. Mortality was reported in 10 (16.4%) patients. Conclusion We found that the 100% fatality rate with controlled positive pressure ventilation and spontaneous breathing is ideal. Tumour lysis syndrome was the most common morbidity in our cohort.

Bilateral Optic Neuritis: A Rare Complication of Mumps.

Mumps is a contagious viral illness that classically presents with fever, parotid gland swelling, headache, and vomiting in unimmunized children. The complications of mumps most commonly include orchitis, pancreatitis, encephalitis, and meningitis. Optic neuritis, which refers to the inflammation of the optic nerve, in rare cases, can present after mumps meningoencephalitis and causes pain in the eye, and a decrease in visual acuity. We report and discuss a case of bilateral optic neuritis following mumps meningoencephalitis in a child. The patient was managed with short-term steroid therapy.

Parental Refusal to Lumbar Puncture: Effects on Treatment, Hospital Stay and Leave Against Medical Advice.

Introduction Lumbar puncture (LP) is an effective method in the diagnosis and management of central nervous system infections. Refusal to LP is associated with severe consequences. This study aims to examine the impact of parental LP refusal on treatment, the length of hospital stay, and the frequency of patients leaving against medical advice (LAMA). Method A cross-sectional study was conducted at the pediatric department of Civil Hospital, Karachi, from June 2018 to November 2019. All hospitalized patients suspected to have a central nervous system disease, which requires LP, were enrolled. Patients were followed for the duration of antibiotic and antiviral therapy, length of hospital stay, and LAMA. Results A total of 220 patients participated in the study, with the median age of nine (2-47) months. There were 113 (51.1%) males. The median length of hospital stay was 10 (4-14) days. The comparison of parental LP refusal with the length of hospital stay showed a significant difference (p-value <0.001) in the number of days of treatment among patients who received vancomycin (p-value =0.008) and meropenem (p-value =0.012). A significant association of parental LP refusal was also observed with meningoencephalitis and meningitis as provisional diagnosis (p-value =0.006). In particular, LAMA and death were found significantly higher among parents who refused LP (p-value <0.001).  Conclusion LP refusal has a significant effect on the treatment, hospital stay, and disposition outcomes. A large number of parents who declined the procedure left against medical advice or suffered grave medical consequences. Parental education addressing their concerns and beliefs, while explaining the indications, and need for performing LP can help effectively overcome this issue.

Safety and Efficacy of Procedural Sedation and Analgesia in Pediatric Oncology Patients.

Objective Procedural sedation and analgesia are the standard of care for painful procedures in children that require immobility. The aim is to assess the safety and efficacy of procedural sedation and analgesia in pediatric oncological patients in a large tertiary care hospital. Method An observational study performed to review medical records of children who received procedural sedation and analgesia (PSA) for pediatric oncological procedures from July 2018 to September 2018. Patients undergoing oncology procedures (lumbar puncture, intrathecal chemotherapy, bone marrow aspiration +/- trephine) were included, and non-anesthesiologist (intensive care physician/emergency physician certified in pediatric advanced life support) provided PSA. Patients were assessed according to PSA protocol guidelines by the American Society of Anesthesiology (ASA). Low-dose ketamine (0.5 mg/kg) and propofol (2 mg/kg) were administered. Results A total of 565 children underwent 1216 procedures in whom the median age was 7.4 years, and the majority (65.1%) were males. The most common procedure was the lumbar puncture (n = 956; 78.6%) followed by bone marrow aspirate only (n = 137, 11.3%) and both (n = 123, 10.1%). Eight (0.7%) patients developed transient oxygen desaturation only as an adverse effect of ketamine-propofol drug combination with 50% procedures utilizing propofol 1 mg/kg for sedation. Conclusion According to the results of our study, the majority of the pediatric patients responded and reported no adverse events during the procedure with ketamine and propofol. Therefore, we conclude that ketamine and propofol are safe and effective as both sedative and an analgesic in procedures on pediatric oncology patients.

A Novel Presentation of Metaphyseal Chondrodysplasia, Schmid Type with Factor VII Deficiency.

Metaphyseal chondrodysplasia, Schmid type (MDSC) is a rare inherited autosomal disorder with characteristic skeletal deformities striking on radiological imaging, which includes metaphyseal cupping and fraying. Physical examination reveals short stature in early childhood, frontoparietal bossing, rachitic rosary, genu varum and valgum, and coxa vara usually. We believe that the constellation of clinical and radiographic findings of MDSC might look similar to vitamin D resistant rickets; hence, genetic analysis is needed to overcome diagnostic challenges faced by physicians to avoid unnecessary vitamin D supplementation in individuals. We report the first case of MDSC with a coexisting factor VII deficiency in an eight-year-old boy.

Landau-Kleffner Syndrome: A Diagnostic Challenge.

The Landau-Kleffner syndrome (LKS), formerly known as acquired epileptic aphasia, is a rare syndrome that typically presents in early childhood with language regression and seizures. We report a case of LKS in an 7-year-old boy who presented with aggressive behavior, difficulty in maintaining posture, and language regression. Systemic examination, including neurological evaluation, was normal. Cerebrospinal fluid (CSF) analysis and magnetic resonance imaging (MRI) were normal. Electroencephalogram (EEG) showed abnormal findings associated with generalized seizure discharge during sleep with more spikes being noted in bilateral frontal and temporal regions. LKS was diagnosed and was treated with anticonvulsants and steroids. On follow-up, the child showed improvement in maintaining posture, was able to walk independently and had improved linguistic functions. This case adds another variant of LKS to the existing literature.