Long-term management of children with neuromuscular disorders

Strehle, Eugen-Matthias

doi:10.2223/jped.1929

Cited by 25 publications

(15 citation statements)

References 54 publications

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“…Asthma is characterized by airway hyperresponsiveness (AHR), multicellular inflammation, and airway obstruction and is accompanied by intermittent episodes of wheezing and coughing. 2,3 Inhaled corticosteroids provide one of the most effective curative therapies, but this method has severe side effects with long-term use, [4][5][6] such as hypertension, cataracts, osteoporosis in elderly patients, and stunted growth in children. However, inhaled corticosteroids are still required in the systemic administration of glucocorticoids for intractable asthma or asthmatic exacerbation.…”

mentioning

confidence: 99%

Enhanced bioavailability and efficiency of curcumin for the treatment of asthma by its formulation in solid lipid nanoparticles

Wang

Zhu²,

Xie³

et al. 2012

IJN

174

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Curcumin has shown considerable pharmacological activity, including anti-inflammatory, but its poor bioavailability and rapid metabolization have limited its application. The purpose of the present study was to formulate curcumin-solid lipid n anoparticles (curcumin-SLNs) to improve its therapeutic efficacy in an ovalbumin (OVA)-induced allergic rat model of asthma. A solvent injection method was used to prepare the curcumin-SLNs. Physiochemical properties of curcumin-SLNs were characterized, and release experiments were performed in vitro. The pharmacokinetics in tissue distribution was studied in mice, and the therapeutic effect of the formulation was evaluated in the model. The prepared formulation showed an average size of 190 nm with a zeta potential value of −20.7 mV and 75% drug entrapment efficiency. X-ray diffraction analysis revealed the amorphous nature of the encapsulated curcumin. The release profile of curcumin-SLNs was an initial burst followed by sustained release. The curcumin concentrations in plasma suspension were significantly higher than those obtained with curcumin alone. Following administration of the curcumin-SLNs, all the tissue concentrations of curcumin increased, especially in lung and liver. In the animal model of asthma, curcumin-SLNs effectively suppressed airway hyperresponsiveness and inflammatory cell infiltration and also significantly inhibited the expression of T-helper-2-type cytokines, such as interleukin-4 and interleukin-13, in bronchoalveolar lavage fluid compared to the asthma group and curcumin-treated group. These observations implied that curcumin-SLNs could be a promising candidate for asthma therapy.

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confidence: 99%

Enhanced bioavailability and efficiency of curcumin for the treatment of asthma by its formulation in solid lipid nanoparticles

Wang

Zhu²,

Xie³

et al. 2012

IJN

174

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“…The NMDs worldwide prevalence estimate, together, is 1:3500 individuals 2 . In childhood, the most frequent NMD is Duchenne muscular dystrophy (DMD), a recessive X-linked inherited disorder with worldwide incidence around 1 in 4.000 newborn boys.…”

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confidence: 99%

Motor assessment in patients with Duchenne muscular dystrophy

Diniz

Lasmar

Giannetti

2012

Arq. Neuro-Psiquiatr.

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OBJECTIVE: Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD) in a period of six months. METHOD: Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC), and application of the Motor Function Measure (MFM). The patients were evaluated twice within a six-month interval. RESULTS: Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04). In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006). Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. CONCLUSION: The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.

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“…Proximal musculature is affected first and contracture from loss of active movement often follows. Muscular dystrophy, which affects one in every 3,500 male births, is a progressive inherited disease that results in weakness and dysfunction (Strehle, 2009).…”

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confidence: 99%

“…Children with these diseases are extremely adaptive, but their long-term spine health remains a concern when the trunk and neck are repeatedly flexed to compensate for upper extremity weakness (Strehle, 2009). The compensatory postures these children use present social and physical barriers as they age.…”

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confidence: 99%