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Challenges and Strategies in Pediatric Critical Care: Insights From Low-Resource Settings.
Neurodivergence and Threat: A Case Study on the Risk of Violent Fixations in Autism Spectrum Disorder.
Mass shootings have increasingly captured public attention in recent decades, prompting closer examination of the mental health of those responsible. This scrutiny often focuses on individuals with neurodevelopmental disorders, such as autism spectrum disorder (ASD). While epidemiological evidence is mixed on whether these individuals are more likely to commit acts of violence than the general public, certain behavioral characteristics may make them more vulnerable to extremist ideations. This case study focuses on a 17-year-old male patient who initially presented with suicidal behavior, later diagnosed with ASD following clinical evaluation. He was also found to have additional comorbidities such as major depressive disorder, gender dysphoria, and unspecified eating disorder, while harboring threats of extreme violence. Despite displaying characteristics similar to previous mass shooters with ASD, the patient has never acted on his violent thoughts, a pattern consistent with most individuals with violent ideations. The case overviews the complexity of assessing when such threats are legitimate and the potential consequences of misinterpretation. There is urgent need for standardized protocols to differentiate between behaviors stemming from ASD and violence unrelated to ASD. The findings highlight the importance of understanding the vulnerabilities and presentations of neurodivergent individuals to provide appropriate care and prevent potential tragedies
When the Bones Tell a Different Story: An Atypical Presentation of Acute Lymphoblastic Leukemia
Acute lymphoblastic leukemia has a wide range of clinical presentations, such as anemia, fatigue, and thrombocytopenia. However, one may find rare presentations like bone lytic lesions that mimic multiple myeloma. Herein, we report a case of B-cell Acute Lymphoblastic Leukemia that first presented with osteolytic bone lesions with associated hypercalcemia. This unusual observation indicates that B cell disorders other than multiple myeloma may present with unusually significant bone resorption, which results in excessive osteoclastic resorption, lytic bone lesions, and recurrent hypercalcemia, with an overall disease picture clinically mimicking multiple myeloma. Large series have estimated the frequency of this phenomenon to be about 2 percent. We report a 29-year-old male who is not known to have a medical illness and presented with chronic intermittent lower back pain. It responded minimally to rest and nonsteroidal painkillers, requiring multiple visits to the emergency department. There is no history of numbness or weakness in the lower limbs. Also, history is significant for weight loss of almost 7 kilograms. Initial workup, including routine labs, showed a hemoglobin level of 10.2 mg/dl and an MCV of 81.6. The iron panel was suggestive of anemia of chronic disease. The adjusted calcium level was 2.65, the CRP level was 90 mg/l, and the LDH was 248. Interestingly, the sacroiliac and thoracolumbar spine X-rays reported a diffuse decrease in bone density with decreased lower thoracic and lumbar spine vertebral body height, mainly at the T8 level. Later, the HLAB27 returned negative. Magnetic Resonance (MRI) of the spine with contrast was done later on and showed heterogeneous bone marrow signal intensity with relative T1W1 hypointensity. Postcontrast images have demonstrated patchy enhancement of T11. All findings suggest possible bone infiltrative disease (e.g., myeloma, lymphoma, leukemia, or metastasis). A peripheral smear showed a leucoerythroblastic picture with mild thrombocytopenia, and multiple myeloma workups returned unremarkable. Ultrasound of the abdomen and pan-computed tomography scan were generally unremarkable except for demonstrating diffuse lytic skeletal infiltration involving most axial skeletal bones, including the sternum, ribs, vertebral column, and pelvic bones. So, based on the above assessment, a bone marrow assessment was performed to explain the picture of anemia, mild hypercalcemia, and hypodense vertebral bone lesions. Pelvic bone marrow aspirate and biopsy were performed, which interestingly showed around 36% blast cells with an immunophenotype profile consistent with precursor B cells (ALL) with standard risk and negative minimal residual disease (MRD). As part of the initial leukemia workup, the fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan showed the same extensive lytic lesions throughout the axial skeleton. Urgent tumor lysis syndrome prophylactic measures and IV Dexamethasone were initiated, and chemotherapy protocol was planned. He was started on the BFM2009 protocol, followed by a total of 6 cycles of intrathecal methotrexate without significant complications. A follow-up evaluation via PET scan showed a complete response (Figure 6). A follow-up bone marrow biopsy showed 2% blast cells. Then, he was started on maintenance therapy of oral MTX and mercaptopurine with a folic acid supplement. In conclusion, a lytic bone lesion is an unusual presentation of B-cell ALL, mimicking the picture of multiple myeloma. Hence, it should always be one of the differentials in a presentation of this nature. Further similar cases should be reported to study the behavior of such an unusual presentation of B-cell ALL
Management and Outcomes of Pediatric Lymphatic Malformations: A Systematic Review From the APSA Outcomes and Evidence-Based Practice Committee.
BACKGROUND: Significant variation in management strategies for lymphatic malformations (LMs) in children persists. The goal of this systematic review is to summarize outcomes for medical therapy, sclerotherapy, and surgery, and to provide evidence-based recommendations regarding the treatment.
METHODS: Three questions regarding LM management were generated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Publicly available databases were queried to identify articles published from January 1, 1990, to December 31, 2021. A consensus statement of recommendations was generated in response to each question.
RESULTS: The initial search identified 9326 abstracts, each reviewed by two authors. A total of 600 abstracts met selection criteria for full manuscript review with 202 subsequently utilized for extraction of data. Medical therapy, such as sirolimus, can be used as an adjunct with percutaneous treatments or surgery, or for extensive LM. Sclerotherapy can achieve partial or complete response in over 90% of patients and is most effective for macrocystic lesions. Depending on the size, extent, and location of the malformation, surgery can be considered.
CONCLUSION: Evidence supporting best practices for the safety and effectiveness of management for LMs is currently of moderate quality. Many patients benefit from multi-modal treatment determined by the extent and type of LM. A multidisciplinary approach is recommended to determine the optimal individualized treatment for each patient
Catfish Puncture Wound and Retained Spine Management in the ED Setting: A Case Report.
Fishing is a common recreational activity in the United States, with over 29 million registered fishers. Although not inherently dangerous, commonly seen injuries from fishing include embedded fishhooks and injury from flora and fauna. Emergency department (ED) physicians need a basic understanding of how to treat these less-than-frequent injuries. We present a case report of a patient who presented with a catfish spine lodged in her leg. These spines not only cause puncture wounds but can result in lacerations and venom release as well. Our patient presented 6 hours after the initial injury for spine removal and symptom management. Plain radiographs of the affected extremity demonstrated a 2 cm foreign body consistent with a catfish spine. The wound was expanded, and the spine successfully removed. The patient was discharged on levofloxacin and reported a healing wound without complications nearly 2 weeks after the injury
Developing methods to identify resilience and improve communication about diagnosis in pediatric primary care.
Communication underlies every stage of the diagnostic process. The Dialog Study aims to characterize the pediatric diagnostic journey, focusing on communication as a source of resilience, in order to ultimately develop and test the efficacy of a structured patient-centered communication intervention in improving outpatient diagnostic safety. In this manuscript, we will describe protocols, data collection instruments, methods, analytic approaches, and theoretical frameworks to be used in to characterize the patient journey in the Dialog Study. Our approach to characterization of the patient journey will attend to patient and structural factors, like race and racism, and language and language access, before developing interventions. Our mixed-methods approach is informed by the Systems Engineering Initiative for Patient Safety (SEIPS) 3.0 framework (which describes the sociotechnical system underpinning diagnoses within the broader context of multiple interactions with different care settings over time) and the Safety II framework (which seeks to understand successful and unsuccessful adaptations to ongoing changes in demand and capacity within the healthcare system). We will assess the validity of different methods to detect diagnostic errors along the diagnostic journey. In doing so, we will emphasize the importance of viewing the diagnostic process as the product of communications situated in systems-of-work that are constantly adapting to everyday challenges
GLP-1 Agonists for Weight Loss and Beyond
Glucagon-like peptide-1 (GLP-1) agonists are medications that were originally approved for type 2 diabetes to help improve glycemic control in conjunction with exercise and dieting. One of the beneficial effects of this medication class is weight loss, and recently the FDA has approved a select few for this indication of weight loss. With these medications becoming increasingly more popular, it is imperative that both providers and patients understand the differences in terms of efficacy for these medications and the potential adverse events to make the best decision about initiating therapy. This article summarizes the FDA indications for all the GLP-1 agonists and includes supporting trial data showcasing their efficacy as weight loss treatments. Outside of weight loss and diabetes, there also may be other benefits seen with GLP-1 agonist usage in specific populations such as cardiovascular disease, neurodegenerative diseases, non-alcoholic fatty liver disease, and non-alcoholic steatohepatitis
Associations between eating disorders and sociodemographic factors in adolescent patients since the start of the COVID-19 pandemic
Co-occurrence of rhino-orbital mucormycosis and acute lymphoblastic leukemia post-COVID-19 infection in a young adolescent male: A case report from a low middle-income country
Key Clinical Message: Immunosuppression from B-acute lymphoblastic leukemia (B-ALL) chemotherapy and a preceding COVID-19 infection may predispose patients to rare complications such as rhino-orbital mucormycosis. Hence, a high index of suspicion should be maintained by physicians (and oncologists) if patients undergoing B-ALL treatment present with orofacial symptoms and ophthalmological manifestations such as peri-orbital swelling, ophthalmoplegia, and loss of vision, suggestive of infection. Abstract: Mucormycosis is a severe fungal infection that poses significant mortality and morbidity risks, particularly in immunocompromised individuals. We present a rare case of a 16-year-old patient with rhino-orbital mucormycosis following B-acute lymphoblastic leukemia (B-ALL) treatment and concurrent COVID-19 infection. We describe the clinical presentation, diagnosis, treatment, and outcome of this patient, and discuss the possible interactions and implications of these three conditions. A young 16-year-old male patient without significant clinical history was admitted with complaints of low-grade intermittent fever, fatigue, malaise, restlessness, and unexplained weight loss for the past 2 months. A bone marrow biopsy confirmed the diagnosis of B-ALL. Following the diagnosis of B-ALL, the patient underwent initiation of chemotherapy. Following the initial two cycles of chemotherapy, the patient experienced fever and cough and tested positive for COVID-19 infection. Nearly a week later, the patient presented to the chemotherapy emergency department with a clinical picture characterized by a fever up to 39°C associated with left facial swelling, severe headache, purulent rhinorrhea, and foreign body sensation in the ipsilateral nostril. The following day, erythema and left eyelid edema were observed, with ocular opening limitation. The diagnosis was confirmed based on the positive result of polymerase chain reaction for left-sided mucormycosis. Initial administration of liposomal and lipid amphotericin B at 1–1.5 mg/kg/d doses for 4–6 weeks was followed by surgical debridement of necrotic tissue on the left side of the face and nose. Subsequent ophthalmological examinations showed normal conditions of the left eye. The case underscores the importance of heightened clinical suspicion, early diagnosis through imaging and molecular techniques, aggressive multimodal therapy, and close interdisciplinary collaboration for improved outcomes in such rare and challenging clinical scenarios
Targeting refractory/recurrent neuroblastoma and osteosarcoma with anti-CD3×anti-GD2 bispecific antibody armed T cells.
BACKGROUND: The survival benefit observed in children with neuroblastoma (NB) and minimal residual disease who received treatment with anti-GD2 monoclonal antibodies prompted our investigation into the safety and potential clinical benefits of anti-CD3×anti-GD2 bispecific antibody (GD2Bi) armed T cells (GD2BATs). Preclinical studies demonstrated the high cytotoxicity of GD2BATs against GD2+cell lines, leading to the initiation of a phase I/II study in recurrent/refractory patients.
METHODS: The 3+3 dose escalation phase I study (NCT02173093) encompassed nine evaluable patients with NB (n=5), osteosarcoma (n=3), and desmoplastic small round cell tumors (n=1). Patients received twice-weekly infusions of GD2BATs at 40, 80, or 160×10
RESULTS: Of the 12 patients enrolled, 9 completed therapy in phase I with no dose-limiting toxicities. Mild and manageable cytokine release syndrome occurred in all patients, presenting as grade 2-3 fevers/chills, headaches, and occasional hypotension up to 72 hours after GD2BAT infusions. GD2-antibody-associated pain was minimal. Median overall survival (OS) for phase I and the limited phase II was 18.0 and 31.2 months, respectively, with a combined OS of 21.1 months. A phase I NB patient had a complete bone marrow response with overall stable disease. In phase II, 10 of 12 patients were evaluable: 1 achieved partial response, and 3 showed clinical benefit with prolonged stable disease. Over 50% of evaluable patients exhibited augmented immune responses to GD2+targets post-GD2BATs, as indicated by interferon-gamma (IFN-γ) EliSpots, Th1 cytokines, and/or chemokines.
CONCLUSIONS: This study demonstrated the safety of GD2BATs up to 160×1