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[CME/Answers: Giant Cell Arteritis].

CME/Answers: Giant Cell Arteritis Giant cell arteritis (GCA) is the most common vasculitis among patients over the age of 50. Mainly large vessels are targeted. GCA can be differentiated into cranial and extra-cranial types; thus the symptoms can range from headache, blurred vision and jaw claudication to non-specific symptoms like fatigue, polymyalgia and fever. Complications such as an irreversible loss of vision are critical, which is why timeous diagnosis and treatment are essential. There are some recommendations for treatment, but no defined guidelines exist. Steroids have been the standard treatment for the past six decades and remain so, but side effects are common. Tocilizumab represents an alternative and more effective and safer treatment.

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The Value of Gene Detection and Skin Biopsy in the Diagnosis of Neuronal Intranuclear Inclusion Disease.

The clinical manifestations of neuronal intranuclear inclusion disease (NIID) are heterogeneous, and the premortem diagnosis is mainly based on skin biopsy findings. Abnormal GGC repeat expansions in was recently identified in familial and sporadic NIID. The comparison of diagnostic value between abnormal GGC repeat expansions of and skin biopsy has not been conducted yet. In this study, skin biopsy was performed in 10 suspected adult NIID patients with clinical and imaging manifestations, and GGC repeat size in was also screened by repeat primed-PCR and GC-rich PCR. We found that five cases had ubiquitin-immunolabelling intranuclear inclusion bodies by skin biopsy, and all of them were identified with abnormal GGC repeat expansions in , among whom four patients showed typical linear hyperintensity at corticomedullary junction on DWI. Five (5/10) NIID patients were diagnosed by combination of gene detection, skin biopsy or combination of , and typical MRI findings. The diagnostic performance of gene detection was highly consistent with that of skin biopsy ( = 1). The unexplained headache was firstly reported as a new early phenotype of NIID. These findings indicate that gene detection is needed to be a supplement in the diagnose flow of NIID and also may be used as an alternative method to skin biopsy especially in Asian population.

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Case Report: Epidural Abscess Following Obstetric Epidural Anesthesia in Vietnam.

Epidural abscess after obstetric epidural anesthesia occurs infrequently and may result in severe morbidity. We report a clinical case of an epidural abscess in Vietnam. A 31-year-old woman who was in labor was admitted to our hospital and given epidural anesthesia indicated to relieve labor pain. After three hours of anesthesia, cesarean section was indicated for the patient due to signs of fetal failure, the epidural catheter was then used to relieve pain for the first 48 hours postoperatively. On post-partum day 5 she presented with high fever of 39-40°C, fatigue, stimulation, dyspnea, and lumbar pain. The patient then suffered from sepsis and lower limb paralysis. She was diagnosed with epidural abscess based on the MRI of the lumbar spine. The abscess was treated by surgery for draining and appropriate antibiotics with a satisfactory outcome as she completely recovered her motor function and stable health status.

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Case Report: A Rare Presentation of NSAID-Induced Secondary Membranous Nephropathy in a Pediatric Patient.

Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but it is responsible for <5% of nephrotic syndrome cases in children. MN has primary and secondary forms. Secondary MN is caused by viral infections, autoimmune diseases like lupus, or drugs. Non-steroid anti-inflammatory drug (NSAID)-induced secondary MN is rarely described in the pediatric population. Thus, the clinical presentation and time to recovery are vastly unknown in the pediatric subgroup. We report a case of a 15-year-old female who presented with acute onset of nephrotic range proteinuria, significant hypoalbuminemia, hyperlipidemia, and lower extremity edema related to the presence of nephrotic syndrome. She had a history of ibuprofen use periodically for 6 months before presentation because of menstrual cramps and intermittent lower abdominal pain. After the presentation, we performed a renal biopsy that reported stage 1-2 MN, likely secondary. The phospholipase A2 receptor (PLA2R) antibody on the blood test and PLA2R immune stain on the renal biopsy sample were negative. We performed a comprehensive evaluation of the viral and immune causes of secondary MN, which was non-revealing. She had stopped ibuprofen use subsequent to the initial presentation. She was prescribed ACE inhibitor therapy. After 6 months of ACE inhibitor treatment, the proteinuria had resolved. Proteinuria can last for several weeks when NSAID induces secondary MN and nephrotic syndrome. With the widespread use of NSAIDs prevalent in the pediatric community, further studies are needed to evaluate and study the role of NSAIDs in this condition.

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Extremity Gangrene Caused by HBV-Related Cryoglobulinemia Vasculitis in a Patient with Diabetes – A Case Report.

We presented a case of hepatitis B virus (HBV)-related type III cryoglobulinemia vasculitis (CryoVas) characterized by extremity gangrene in a patient with diabetes. The 60-year-old female had a 10-year history of poorly controlled type 2 diabetes mellitus. She complained of sudden onset pain and swelling of toes which quickly progressed to gangrene, with fingers becoming pain and dark violet. The patient was initially misdiagnosed as diabetic foot (DF). Although DF is one of the common chronic complications of diabetes, it rarely involves the hand. What is more, the ischemic manifestations of the extremity were not consistent with the results of the vascular examination and immune system changes. The patient had Raynaud's phenomenon, arthralgia, and extremity gangrene. Test results showed cryoglobulinemia multiple positive, polyclonal immunoglobulin with rheumatoid factor negative, lower complement 3, leukocytoclastic vasculitis, and HBV infection. HBV-related type III CryoVas was finally diagnosed, and a conservative therapy strategy was given. Six months after treatment with cyclophosphamide, corticosteroid, nucleoside/nucleotide analog therapy, local debridement, and dressing change, she recovered and kept no recurrence by following up for 30 months. To our knowledge, this is the first report of extremity gangrene caused by HBV-related CryoVas in a diabetic patient.

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Who Suffers From Pharmaceutical Poverty and What Are Their Needs? Evidence From a Spanish Region.

Pharmaceutical poverty occurs when a patient cannot afford the cost of prescribed medication and/or medical products. Nonprofit organizations are covering the cost of medication to those patients in some contexts. The aim of the study was to describe the population of beneficiaries of the PB, a nongovernmental organization based on the primary healthcare system, which provides free-of-charge access to medicines and their utilization pattern of medicines and healthcare products. This was an observational study using PB beneficiary data collected between November 2017 and December 2018 in Catalonia. The Catalan Health Service provided information from the general population. A descriptive analysis of the beneficiaries' characteristics was conducted and compared to the general population. The beneficiaries (N = 1,206) were mainly adults with a low level of education, unemployed, with functional disability, and with ≥1 child. Compared with the general population, the beneficiaries were older, had a lower level of education, showed a higher prevalence of functional disability, were less likely to be Spanish, and were more likely to be divorced and unemployed. The beneficiaries were polymedicated, and most were using medication related to the nervous (79%), musculoskeletal (68%), and cardiovascular system (56%) and alimentary tract and metabolism (68%). Almost 19% of beneficiaries used healthcare products. Female beneficiaries were older and more likely to be divorced or widowed, employed, and with children. Compared to men, women were more likely to use medicines for pain and mental disorders. The pediatric group used medications for severe, chronic conditions (heart diseases, autoimmune diseases, conduct disorders, and attention deficit hyperactivity disorder). Patients with severe, chronic, and disabling conditions are affected by pharmaceutical poverty. While the system of copayment remains unchanged, family physicians and pediatricians should explore economic barriers to treatment and direct their patients to resources that help to cover the cost of treatment.

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The Effect of Dexmedetomidine in Combination with Bupivacaine on Sensory and Motor Block Time and Pain Score in Supraclavicular Block.

Brachial plexus block is frequently recommended for upper limb surgeries. Many drugs have been used as adjuvants to prolong the duration of the block. This study aimed to assess the effect of dexmedetomidine with bupivacaine combination and only bupivacaine on sensory and motor block duration time, pain score, and hemodynamic variations in the supraclavicular block in upper extremity orthopedic surgery.

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Effectiveness of Exposure in Vivo for Patients with Painful Diabetic Neuropathy: a Pilot Study of Effects on Physical Activity and Quality of Life.

To evaluate the effects of personalized exposure on level of physical activity and quality of life in patients with painful diabetic neuropathy.

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Neurologic and Neuroscientific Evidence in Aged COVID-19 Patients.

The COVID-19 pandemic continues to prevail as a catastrophic wave infecting over 111 million people globally, claiming 2. 4 million lives to date. Aged individuals are particularly vulnerable to this disease due to their fraility, immune dysfunction, and higher rates of medical comorbidities, among other causes. Apart from the primary respiratory illness, this virus is known to cause multi-organ dysfunction including renal, cardiac, and neurologic injuries, particularly in the critically-ill cohorts. Elderly patients 65 years of age or older are known to have more severe systemic disease and higher rates of neurologic complications. Morbidity and mortality is very high in the elderly population with 6-930 times higher likelihood of death compared to younger cohorts, with the highest risk in elderly patients ≥85 years and especially those with medical comorbidities such as hypertension, diabetes, heart disease, and underlying respiratory illness. Commonly reported neurologic dysfunctions of COVID-19 include headache, fatigue, dizziness, and confusion. Elderly patients may manifest atypical presentations like fall or postural instability. Other important neurologic dysfunctions in the elderly include cerebrovascular diseases, cognitive impairment, and neuropsychiatric illnesses. Elderly patients with preexisting neurologic diseases are susceptibility to severe COVID-19 infection and higher rates of mortality. Treatment of neurologic dysfunction of COVID-19 is based on existing practice standards of specific neurologic condition in conjunction with systemic treatment of the viral illness. The physical, emotional, psychologic, and financial implications of COVID-19 pandemic have been severe. Long-term data are still needed to understand the lasting effects of this devastating pandemic.

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Use of IFNγ/IL10 Ratio for Stratification of Hydrocortisone Therapy in Patients With Septic Shock.

Large clinical trials testing hydrocortisone therapy in septic shock have produced conflicting results. Subgroups may benefit of hydrocortisone treatment depending on their individual immune response. We performed an exploratory analysis of the database from the international randomized controlled clinical trial Corticosteroid Therapy of Septic Shock (CORTICUS) employing machine learning to a panel of 137 variables collected from the Berlin subcohort comprising 83 patients including demographic and clinical measures, organ failure scores, leukocyte counts and levels of circulating cytokines. The identified theranostic marker was validated against data from a cohort of the Hellenic Sepsis Study Group (HSSG) ( = 246), patients enrolled in the clinical trial of Sodium Selenite and Procalcitonin Guided Antimicrobial Therapy in Severe Sepsis (SISPCT, = 118), and another, smaller clinical trial (Crossover study, = 20). In addition, blood culture experiments and experiments in mouse models were performed to assess biological plausibility. A low serum IFNγ/IL10 ratio predicted increased survival in the hydrocortisone group whereas a high ratio predicted better survival in the placebo group. Using this marker for a decision rule, we applied it to three validation sets and observed the same trend. Experimental studies revealed that IFNγ/IL10 was negatively associated with the load of (heat inactivated) pathogens in spiked human blood and in septic mouse models. Accordingly, an analysis of published IFNγ and IL10 values in bacteremic and non-bacteremic patients with the Systemic Inflammatory Response Syndrome supported this association between the ratio and pathogen burden. We propose IFNγ/IL10 as a molecular marker supporting the decision to administer hydrocortisone to patients in septic shock. Prospective clinical studies are necessary and standard operating procedures need to be implemented, particularly to define a generic threshold. If confirmed, IFNγ/IL10 may become a suitable theranostic marker for an urging clinical need.

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