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BACKGROUND: Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy. OBSERVATIONS: A 7-year-old male presented with right facial weakness in addition to headaches, ataxia, urinary incontinence, and falls. Magnetic resonance imaging revealed CMI with a syrinx of the cervicothoracic spine and syringobulbia. Posterior fossa decompression with duraplasty was performed without complications, and the patient was discharged home on postoperation day 5. At the 3-week follow-up, the patient's neurological deficits had largely subsided. At the 3-month follow-up, his CN VII palsy and syringobulbia had completely resolved. LESSONS: Pediatric CMI patients with syringomyelia are at risk for developing syringobulbia and brainstem deficits, including unilateral facial palsy. However, craniocervical decompression can prove successful in treating such deficits.
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Acquired ventricular septal rupture (VSR) is a rare but potentially fatal complication of late-presenting myocardial infarction (MI). In the era of revascularization and reperfusion therapy, the incidence of VSR has significantly decreased. Ruptures occur predominantly in patients with late-presenting ST elevation MI. Patients may present with a wide variety of symptoms ranging from chest pain and mild hemodynamic instability to profound cardiogenic shock. Inotropes, vasopressors, and mechanical support with intra-aortic balloon pumps and extracorporeal membrane oxygenation can be used to bridge patients to surgery. Despite treatment with ventricular septal repair, postsurgical mortality remains high. There is a wide variety of complications that can occur in the postoperative period. A multidisciplinary approach is vital in these patients who develop VSR. Improving awareness among healthcare professionals regarding the symptoms of acute coronary syndrome can hopefully help prevent delayed presentation of patients to healthcare facilities.
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Patients with rheumatoid arthritis (RA) may experience complications directly from the disease process or from immune-modulating agents used to treat RA. Adalimumab is a recombinant human monoclonal antibody directed against tumor necrosis factor alpha (TNFα) which has been increasingly used in the management of inflammatory and autoimmune diseases. Acute lung injury has been associated with the use of anti-TNFα agents, but the association with adalimumab is rare. Here we present a case of a patient with RA-associated lung disease who developed acute respiratory distress syndrome while being treated with adalimumab. Adalimumab-related lung injury is less common than lung injury associated with other anti-TNFα drugs, thus clinicians should be aware of this condition, as prompt recognition and supportive management can help prevent worsening outcomes.
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A 28-year-old G2P0010 woman with a history of COVID infection during her current pregnancy treated with monoclonal antibodies and benign gestational thrombocytopenia presented for routine prenatal care at 33 weeks' gestation. The patient was asymptomatic, but incidental tachycardia was noted on the physical exam with an irregular rhythm. An electrocardiogram (ECG) was performed and was consistent with multifocal atrial tachycardia at a rate of 144 beats per minute. The patient was started on labetalol 50 mg daily and was referred to cardiology for consultation. An echocardiogram was performed and showed dilated left ventricular cavity with a moderately reduced ejection fraction of 40%. No previous echocardiogram was available for comparison; the patient had no history of cardiac disease. The dose of labetalol was increased to 50 mg twice daily and she was admitted for digoxin loading and titration. Though fetal tolerance was excellent, her heart rate was not controlled. Digoxin was switched to flecainide and labetalol was switched to metoprolol which improved her heart rate and repeat echocardiogram showed an ejection fraction of 50%. The patient was admitted for induction of labor at 39 weeks of gestation and continued intrapartum flecainide. Metoprolol was continued intra and postpartum. Flecainide was resumed at three days postpartum due to the recurrence of atrial tachycardia and has been maintained. A repeat echocardiogram is scheduled six weeks postpartum to evaluate left ventricular function and wean off antiarrhythmics.
RESUMEN
Sensory-based therapies are designed to address sensory processing difficulties by helping to organize and control the regulation of environmental sensory inputs. These treatments are increasingly popular, particularly with children with behavioral and developmental disabilities. However, empirical support for sensory-based treatments is limited. The purpose of this review was to conduct a comprehensive and methodologically sound evaluation of the efficacy of sensory-based treatments for children with disabilities. Methods for this review were registered with PROSPERO (CRD42012003243). Thirty studies involving 856 participants met our inclusion criteria and were included in this review. Considerable heterogeneity was noted across studies in implementation, measurement, and study rigor. The research on sensory-based treatments is limited due to insubstantial treatment outcomes, weak experimental designs, or high risk of bias. Although many people use and advocate for the use of sensory-based treatments and there is a substantial empirical literature on sensory-based treatments for children with disabilities, insufficient evidence exists to support their use.