Clinically Isolated Syndrome and Frontal Lobe Arteriovenous Malformation Presenting With Behavior Issues.

Prevalence of brain arteriovenous malformation ranges from 0.14% to 0.6% according to various estimates. A large number of these patients remain asymptomatic. The most common presentation is due to brain hemorrhage. A 14-year-old girl presented to the pediatrician with erratic behavior issues and hallucinations. She was diagnosed by the pediatrician and mental health facility as having schizophrenia and bipolar disorder. Once she was transferred to our children's hospital, evaluation by a pediatric neurologist, computed tomography scan, magnetic resonance imaging, and laboratory workup including lumbar puncture confirmed a clinically isolated syndrome and frontal lobe arteriovenous malformation. Frontal lobe lesions including arteriovenous malformation in the frontal lobe can cause psychological symptoms and behavioral issues. We also discuss the differential diagnosis of acute demyelinating syndromes.

Refractory Methemoglobinemia Secondary to Topical Dapsone With Subsequent Autoimmune Hemolytic Anemia.

We present a case of refractory methemoglobinemia with subsequent autoimmune hemolytic anemia in a young female after two days of topical dapsone use. A 15-year-old female with no known genetic risk factors was found to have a methemoglobinemia concentration of 37.1% after presenting with cyanosis, dyspnea, tachycardia, and oxygen saturation of 88% on 15 L of oxygen via a non-rebreather mask. Despite treatment with methylene blue, her methemoglobin concentrations continued to spike, requiring additional doses of methylene blue in addition to ascorbic acid and cimetidine. After discharge on the fourth day, she presented to another hospital with similar symptoms and was again found to have methemoglobinemia before developing autoimmune hemolytic anemia. This patient had no known underlying risk factors, including a normal BMI, normal renal function, two negative glucose-6-phosphate-dehydrogenase (G6PD) deficiency tests, and surprisingly a negative Coombs test. Although rare, particularly in the setting of topical dapsone use, methemoglobinemia remains an important consideration in the differential diagnosis of cyanosis and hypoxia, with early recognition by the emergency medicine physician being imperative for good patient outcomes.

Thromboelastography Use in the Acute Young Trauma Patient: Early Experience of Two Level One Trauma Centers.

BACKGROUND: Thromboelastography (TEG) point-of-care systems allow for analysis of the sum of platelet function, coagulation proteases and inhibitors, and the fibrinolytic system within 30 minutes. This allows a clinician to guide transfusion more precisely with an appropriate type of blood product. Literature has supported that TEG-guided resuscitation had lower mortality compared to standardized 1:1:1 (red blood cells (RBC), fresh-frozen plasma (FFP), and platelets) massive transfusion protocol (MTP) in penetrating trauma patients, but data has been sparse in examining the young trauma patient. METHODS: This was a cross-sectional chart review study performed with patients up to 30 years old seen in two level one trauma centers serving children with active bleeding resulting from trauma from January 1, 2010 to June 26, 2018. TEG use was evaluated in these patients. RESULTS: 258 patients were included in the analysis. 112 (43%) had penetrating trauma and 225 (87%) had polytrauma. MTP was instituted in 176 (69%) patients and 88 (34%) patients who had TEG measured. There were significant correlations between PTT and alpha (r=-0.46; p<0.001), PTT and Kinetics (r=0.53; p<0.001), PTT and maximum amplitude (r=0.449; p<0.001). There were also significant correlations between PT and alpha (r=-0.29; p=0.008), and PT and maximum amplitude (r= -0.27; p=0.013). There was no significant correlation between TEG measures and INR. There were significant associations with requiring surgery within 24 hours 45% vs 61% (p=0.018), receiving TXA 20% vs 59% (p<0.001), and with receiving MTP 62% vs 83% (p=0.001), respectively. CONCLUSIONS: Measurement of TEG was associated with patients receiving TXA, MTP and larger amounts of blood products. Components of TEG correlated with PT and PTT levels. Although there was no association with survival to hospital discharge, patients having TEG measured were more likely to undergo surgery within the first 24 hours of hospital arrival.

Multiple unusual lung etiologies in a child with acute lymphocytic leukemia.

Granulomatous lung disease is a rare and perplexing differential in pediatrics. Pulmonary Cryptococcus falls into the differential but is not high on the list, particularly in a non-AIDS patient. Methotrexate (MTX) is a commonly used agent for chemotherapy in oncology and has been documented to cause lung injury in both patients with rheumatologic and oncologic diseases. Our patient had chronic cough and then developed an opportunistic infection resulting in respiratory failure. Lung biopsy showed two underlying unusual diagnoses: MTX lung injury and cryptococcal pneumonia. His case is presented with particular attention to his prolonged road to diagnosis.

An in vitro protocol for recording from spinal motoneurons of adult rats.

In vitro slice preparations of CNS tissue are invaluable for studying neuronal function. However, up to now, slice protocols for adult mammal spinal motoneurons--the final common pathway for motor behaviors--have been available for only limited portions of the spinal cord. In most cases, these preparations have not been productive due to the poor viability of motoneurons in vitro. This report describes and validates a new slice protocol that for the first time provides reliable intracellular recordings from lumbar motoneurons of adult rats. The key features of this protocol are: preexposure to 100% oxygen; laminectomy prior to perfusion; anesthesia with ketamine/xylazine; embedding the spinal cord in agar prior to slicing; and, most important, brief incubation of spinal cord slices in a 30% solution of polyethylene glycol to promote resealing of the many motoneuron dendrites cut during sectioning. Together, these new features produce successful recordings in 76% of the experiments and an average action potential amplitude of 76 mV. Motoneuron properties measured in this new slice preparation (i.e., voltage and current thresholds for action potential initiation, input resistance, afterhyperpolarization size and duration, and onset and offset firing rates during current ramps) are comparable to those recorded in vivo. Given the mechanical stability and precise control over the extracellular environment afforded by an in vitro preparation, this new protocol can greatly facilitate electrophysiological and pharmacological study of these uniquely important neurons and other delicate neuronal populations in adult mammals.