ABSTRACT
It is known that poor asthma control is common in pregnancy, and asthma in general disproportionally affects underserved communities. However, there is a paucity of data examining strategies to improve asthma control specifically among pregnant women from vulnerable populations. Identified barriers to optimal asthma care in other underserved groups include health literacy, financial constraints, cultural differences, and poor environmental controls. These deficiencies may also be targets for multimodal interventions geared toward improving asthma outcomes for underserved women during pregnancy.
Subject(s)
Asthma , Pregnancy , Humans , Female , Asthma/diagnosis , Asthma/epidemiology , Asthma/therapyABSTRACT
BACKGROUND: Neuromuscular pathologies must be considered when caring for patients with persistent or progressive respiratory failure. Pertinent disease states may involve skeletal muscles of respiration or associated neurologic structures including motor neurons, peripheral neurons and the neuromuscular junction. Diagnosis may require pulmonary function testing, neurophysiologic studies, imaging, and/or muscle biopsy. CASE PRESENTATION: A 68-year-old male was transferred to our intensive care unit (ICU) for management of ventilator dependent respiratory failure. Upon further historical review, he described gradually worsening gait instability and muscle weakness, which was previously attributed to vascular Parkinsonism in the setting of known cerebrovascular disease. Upon arrival to our hospital, he was found to have elevated muscle specific enzymes, prompting evaluation for neuromuscular causes of respiratory failure. He was also found to have elevated HMG-CoA Reductase (HMGCR) antibodies. Ultimately, a right quadriceps muscle biopsy was performed and electron microscopy identified nemaline bodies within skeletal myofibers. Given the clinical course and other histopathologic findings, he was diagnosed with Sporadic late-onset nemaline myopathy (SLONM). CONCLUSION: The diagnosis of neuromuscular disease in patients with ventilator dependent respiratory failure is challenging. A detailed history of a patient's clinical course prior to hospitalization is key and may raise suspicion for underlying neuromuscular pathology. Further evaluation in non-critically ill patients may include pulmonary function, electromyography and confirmatory muscle biopsy. Sporadic late onset nemaline myopathy remains a rare disease entity which rarely presents with respiratory failure and lacks effective treatment.
Subject(s)
Myopathies, Nemaline , Respiratory Insufficiency , Aged , Humans , Male , Muscle Weakness/etiology , Muscle, Skeletal/pathology , Myopathies, Nemaline/complications , Myopathies, Nemaline/diagnosis , Myopathies, Nemaline/pathology , Respiratory Insufficiency/etiology , Respiratory Insufficiency/pathology , Ventilators, Mechanical/adverse effectsABSTRACT
OBJECTIVE: To analyze the effect of benralizumab in severe eosinophilic asthma (SA) and chronic rhinosinusitis with polyps (CRSwP). METHODS: Retrospective review of patients with both SA and CRSwP that were treated with benralizumab. Asthma controlled test (ACT), pulmonary function metrics (FEV1), Meltzer endoscopic polyp scores, SNOT-22 scores, were collected before and after at least 4 months of benralizumab therapy. RESULTS: 23 patients were included. The mean age at the time of enrollment into benralizumab therapy was 50.47 ± 17.3 years and majority (65.2%, n = 15) were males.Pulmonary Effects: In comparison to baseline ACT, scores at four months showed significant improvement (p = 0.03). In those with pre and post spirometry measurements, mean FEV1 showed significant increase following benralizumab therapy (p = 0.04) with a mean increase of 547 mL ± 597 mL following therapy.Sinonasal Effects: 78.5% of subjects on benralizumab had a significant improvement in sinonasal symptoms (p = 0.009) based on their SNOT-22 scores. Additionally, there was an improvement in endoscopic polyp scores, although not statistically significant, following benralizumab therapy (p = 0.2) with 54.5% patients showing improvement. CONCLUSION: Usage of benralizumab in patients with SA and CRSwNP can lead to significantly improved asthma control, lung function, and sinonasal quality of life. Additionally, in this patient population, there was a subset of patients that showed a significant reduction in polyp burden.
