- Presynaptic congenital myasthenic syndrome with altered synaptic vesicle homeostasis linked to compound heterozygous sequence variants in RPH3A. [Journal Article]
- MGMol Genet Genomic Med 2018 Feb 14
- CONCLUSIONS: In summary, we report a patient with a multisystem neurologic disorder and altered SV regulation attributed to defects in RPH3A, which grants further studies of this gene in human disorders of synaptic transmission.
- Crossover Evaluation of Compressors and Nebulizers Typically Used by Cystic Fibrosis Patients. [Journal Article]
- RCRespir Care 2018 Feb 06
- CONCLUSIONS: Our findings indicate that, in general, replacing the nebulizer or compressor with a different brand changes the flow-pressure and aerosol characteristics. Practitioners should be cautious when changing compressor/nebulizer pairs unless they are aware of the resulting impact on the flow-pressure and aerosol characteristics.
- Correlation between the vitamin D levels and asthma attacks in children: Evaluation of the effects of combination therapy of atomization inhalation of budesonide, albuterol and vitamin D supplementation on asthmatic patients. [Journal Article]
- ETExp Ther Med 2018; 15(1):727-732
- The aim of the present study was to analyze the correlation between the vitamin D (VitD) levels and asthma attack in children, and to evaluate the effects of combination therapy of atomization inhala...
The aim of the present study was to analyze the correlation between the vitamin D (VitD) levels and asthma attack in children, and to evaluate the effects of combination therapy of atomization inhalation of budesonide, albuterol and VitD supplementation on asthmatic children. The total sample size comprised of 96 children with asthma from the time period between May 2015 to April 2016. At the same time, 96 healthy children were also selected from the physical examination center for comparison study. The levels of serum VitD in both groups were detected by the enzyme-linked immunosorbent assay (ELISA). Pulmonary function index that includes the measurement of FEV1, FVC, FEV1/FVC, MEF25 and MEF50 were performed to analyze the results. The patients in the control group were treated with only the budesonide therapy and the patients in the observation group were treated with atomization inhalation of budesonide combined with salbutamol and VitD supplementation. After the treatment, the levels of inflammatory cell indicators (IL-2, IL-4, IL-6 and IFN-γ) and pulmonary function in the two groups were compared. The levels of serum VitD in the children with asthma were significantly lower than those in the normal children. The serum IgE level in children with asthma was significantly higher than that in the normal children (P<0.05). Pearson correlation coefficient analyses showed that VitD levels were not correlated with FEV1, FVC and FEV1/FVC levels (P>0.05), but was positively correlated with MEF25 and MEF50 (P<0.05). After the treatment, the levels of IL-2 and IFN-γ in the observation group were significantly higher and levels of IL-4 and IL-6 were significantly lower than those in the control group (P<0.05). The pulmonary function (FEV1, FVC, FEV1/FVC, MEF25 and MEF50) of the observation group was better than that of the control group (P<0.05). The serum VitD levels of children with asthma were closely related to the acute asthmatic attacks. The lower the levels of serum VitD further leads to higher possibility of asthmatic attacks. Atomization inhalation of budesonide combined with albuterol and VitD supplementation can significantly improve the inflammatory response of the children with asthma.
- Therapeutic strategies for congenital myasthenic syndromes. [Review]
- ANAnn N Y Acad Sci 2018; 1412(1):129-136
- To date, more than 25 genes have been implicated in the etiology of the congenital myasthenic syndromes (CMS), and an ever-growing phenotypic landscape is now encountered in the CMS clinic. Unlike th...
To date, more than 25 genes have been implicated in the etiology of the congenital myasthenic syndromes (CMS), and an ever-growing phenotypic landscape is now encountered in the CMS clinic. Unlike the autoimmune form of myasthenia, there is no role for immunomodulatory agents in the treatment of CMS. The present-day drug repertoire comprises acetylcholinesterase inhibitors (mainly pyridostigmine), 3,4-diaminopyridine (3,4-DAP), ephedrine, salbutamol/albuterol, open-channel blockers (fluoxetine, quinidine), or a combination of these. These are prescribed by the specialist in an off-label manner, as there is no drug currently licensed for the treatment of these rare diseases. The effective pharmacological agent varies according to the genetic form of CMS, and it is important to realize that an agent that provides benefit in one CMS subtype can be harmful in another. In addition, the time to treatment response is variable and tends to be commensurate with the drug used. Here, we summarize for the clinician the therapeutic strategies employed in this ever-evolving disease spectrum. We also address the barriers to treatment and discuss the treatment of CMS in pregnancy.
- Examining 30-day COPD readmissions through the emergency department. [Journal Article]
- IJInt J Chron Obstruct Pulmon Dis 2018; 13:109-120
- CONCLUSIONS: Intensive outpatient monitoring, evaluation, and follow-up after discharge are needed to help prevent re-presentation to the ED, as practically all patients with COPD who represent to the ED within 30 days are readmitted to the hospital and for a variety of clinical complaints. Among those patients with COPD who present with breathing difficulty, improved decision support algorithms and alternative management strategies are needed to identify and intervene on the subgroup of patients who require <48-hour length of stay.
- Adjunctive Pharmacotherapies in Children With Asthma Exacerbations Requiring Continuous Albuterol Therapy: Findings From The Ohio Pediatric Asthma Repository. [Journal Article]
- HPHosp Pediatr 2018 Jan 05
- CONCLUSIONS: Ipratropium and magnesium were both often used in children with severe asthma hospitalizations that required continuous albuterol therapy. Magnesium use was associated with unfavorable outcomes, possibly reflecting preferential treatment to patients with more severe cases and differing practices between centers. Given the high prevalence of asthma, wide variations in practice, and the potential to improve outcomes and costs, prospective trials of these adjunctive therapies are needed.
- Mast Cell Activation Syndrome. [Journal Article]
- SSkinmed 2017; 15(6):477-479
- A 51-year-old woman with a history of asthma and Hashimoto's thyroiditis presented to the dermatology service with a chief complaint of "itchy bumpy rashes" that persisted beyond 24 hours. She noted ...
A 51-year-old woman with a history of asthma and Hashimoto's thyroiditis presented to the dermatology service with a chief complaint of "itchy bumpy rashes" that persisted beyond 24 hours. She noted that, 3 days prior to the onset of urticaria, a pyrroloquinoline quinone supplement had been started. The urticaria was accompanied by variable episodes of transient facial swelling and difficulty breathing. The patient noted that exposure to fish, nuts, and nonsteroidal anti-inflammatory drugs triggered facial swelling. Other reported findings included a 5-year history of diarrhea, sense of memory deterioration, concentration difficulties, and clinical manifestations of anomic aphasia. Although her allergy testing was "negative," she had been given the diagnoses of lactose intolerance and gastroesophageal reflux disease. Laboratory studies on initial presentation were significant for a positive history of antithyroperoxidase antibodies and elevated total complement activity. Medications included budesonide/formoterol, fluticasone/salmeterol, levothyroxine, albuterol, and fexofenadine 180 mg twice daily. Although her "rash" had initially responded to fexofenadine, it soon became refractory to treatment. Her family history was significant only for thyroid disease.
- Effect of Study Design on Sample Size in Studies Intended to Evaluate Bioequivalence of Inhaled Short-Acting β-Agonist Formulations. [Journal Article]
- JCJ Clin Pharmacol 2017 Dec 27
- Pharmacodynamic studies that use methacholine challenge to assess bioequivalence of generic and innovator albuterol formulations are generally designed per published Food and Drug Administration guid...
Pharmacodynamic studies that use methacholine challenge to assess bioequivalence of generic and innovator albuterol formulations are generally designed per published Food and Drug Administration guidance, with 3 reference doses and 1 test dose (3-by-1 design). These studies are challenging and expensive to conduct, typically requiring large sample sizes. We proposed 14 modified study designs as alternatives to the Food and Drug Administration-recommended 3-by-1 design, hypothesizing that adding reference and/or test doses would reduce sample size and cost. We used Monte Carlo simulation to estimate sample size. Simulation inputs were selected based on published studies and our own experience with this type of trial. We also estimated effects of these modified study designs on study cost. Most of these altered designs reduced sample size and cost relative to the 3-by-1 design, some decreasing cost by more than 40%. The most effective single study dose to add was 180 μg of test formulation, which resulted in an estimated 30% relative cost reduction. Adding a single test dose of 90 μg was less effective, producing only a 13% cost reduction. Adding a lone reference dose of either 180, 270, or 360 μg yielded little benefit (less than 10% cost reduction), whereas adding 720 μg resulted in a 19% cost reduction. Of the 14 study design modifications we evaluated, the most effective was addition of both a 90-μg test dose and a 720-μg reference dose (42% cost reduction). Combining a 180-μg test dose and a 720-μg reference dose produced an estimated 36% cost reduction.
- Fluticasone propionate and increased risk of pneumonia in COPD: is it PAFR-dependent? [Letter]
- IJInt J Chron Obstruct Pulmon Dis 2017; 12:3425-3427
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- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Inhaled corticosteroids (ICS) are the FDA-indicated treatment of choice in preventing asthma exacerbations in patients with persistent asthma. Persistent asthma is classified by symptoms more than tw...
Inhaled corticosteroids (ICS) are the FDA-indicated treatment of choice in preventing asthma exacerbations in patients with persistent asthma. Persistent asthma is classified by symptoms more than two days a week, more than three nighttime awakenings per month, more than twice a week use of short-acting beta-2 agonists for symptom control, or any limitation of normal activity due to asthma. Regular use of these medications reduces the frequency of asthma symptoms, bronchial hyperresponsiveness, risk of serious exacerbations, and improves quality of life. These medications are initiated in a stepwise fashion based on the frequency and severity of the asthma symptoms. Low-, medium-, and high-dose inhaled corticosteroids are available to treat mild, moderate, and severe persistent asthma respectively. If inhaled corticosteroids alone are not adequate in controlling a patient's asthma symptoms, other controller medications such as long-acting beta agonists or leukotriene receptor antagonists also may be started. Asthma controller medications often are used in conjunction with short-acting beta agonists such as albuterol as part of an asthma action plan to address acute and chronic symptoms.