Netherton syndrome (NS) is a severe autosomal recessive disorder characterized by the triad of congenital ichthyosiform erythroderma, trichorrhexis invaginata, and atopic diathesis. We report two ...cases that experienced severe congenital exfoliative dermatitis, recurrent infections, and allergic conditions. Examinations of hair under the light microscope revealed trichorrhexis invaginata. Whole exome sequencing identified homologous pathogenic mutations of SPINK5. Optimal skincare and proper nutritional support could improve patients’ quality of life.
Methods A prospective pilot study of MgSO4 treatment was conducted in children admitted with severe acute asthma at Queen Sirikit National Institute of Child Health.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
In Thailand, nebulized ipratropium bromide/fenoterol, is commonly used in addition to salbutamol for severe asthma exacerbation. Recently, nebulized MgSO4 is indicated in GINA 2015 as an additive ...treatment for severe cases. However, there is limited data showed the efficacy of both drugs in childhood severe asthma. The purpose of this study to compare efficacy and safety of nebulized MgSO4 and ipratropium bromide/fenoterol in moderate to severe asthma attacks.
In this a prospective, double-blind, randomized, controlled trial study, we enrolled thirty-three children, age ranged from 2 to 15 years old, with PRAM score ≥ 4 (moderate to severe asthma exacerbation) despite 3 doses of nebulized salbutamol. Each patient was randomized to receive either three doses of nebulized MgSO4 or nebulized ipratropium bromide/fenoterol every 30 minutes. The PRAM score was measured at 0, 30, 60, 90, 120 and 240 minutes after the treatment. The adverse event and admission days were also evaluated.
Sixteen patients received nebulized MgSO4 and seventeen received nebulized ipratropium bromide/fenoterol. Almost patients were classified as having moderate asthmatic attack. There were no statistically significant difference between the two study groups in almost baseline characteristic, PRAM score at 0, 30, 60, 90, 120, 240 minutes. The hospital length of stay was also similar between two groups (p = 0.83). There were no serious events in both groups.
Our double blind, randomized, controlled pilot study demonstrated non-inferior outcomes including clinical benefit and safety of nebulized MgSO4 and nebulized ipratropium bromide/fenoterol among Thai children with acute moderate asthmatic.
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FFLJ, NUK, ODKLJ, UL, UM, UPUK
Rationale Daily inhaled corticosteroid (ICS) is recommended by the world expert committee to treat patients with persistent asthma and tendency to have asthma as a controller. Results At 6 months ...after the treatment, the percentage of exacerbations which needed systemic corticosteroid between 2 groups had no statistically significant difference (P=0.790) with hazard ratio of 1.224 (95% confidence interval (CI) 0.278 to 5.392).
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Wood's score, the assessment of childhood respiratory failure, has been used to assess childhood acute asthma severity in Thailand since 19th century. However, PRAM score, which is increasingly used ...in Western countries has not been evaluated among Thai children with asthma.
This study aims to determine whether Wood or PRAM score is better prediction of severity of childhood asthma exacerbation.
The prospective comparative study of severity asthma score was performed in asthmatic children, 2-18 years old, with acute asthma exacerbation at Queen Sirikit National Institute of Child Health. PRAM and Wood's score were separately determined by 2 physicians. The patients were further assessed at 0, 4 or 24 hours after their admissions. The asthma treatment followed GINA guideline.
There were 80 asthmatic patients, mean aged 5.71 ± 2.95 years. The admission rate was 28.8% with the mean length of stay = 4 ± 1 days. PRAM was correlated with Wood's score (Spearman's correlation p = 0.900, p < 0.001 at triage, and = p0.981, p < 0.001). The highlight of this study is the finding that intraclass correlation of PRAM is better than Wood asthma score (ICC = 0.944; 95%CI 0.913-0.964, 0.898; 95%CI 0.841-0.935, respectively). ROC indicated Wood's score ≤ 4 and PRAM ≤ 5, in the requirement for admission.
PRAM and Wood's score are both promising in prediction of severity and outcome of childhood asthma exacerbation.
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FFLJ, NUK, ODKLJ, UL, UM, UPUK
Severe asthma attacks are life-threatening, and require serious medical attention. Intravenous MgSO₄ is an efficient medication, proven to improve outcomes. To date, most research has focused on ...administration of nebulized MgSO₄ in adults with critical asthma. However, its benefits for treating childhood asthma has been little investigated. This study compared the clinical efficacy and adverse effects of nebulized MgSO₄ and intravenous MgSO₄ in the treatment of children with severe acute asthma.
A prospective, open-label, randomized, controlled pilot study was conducted in children with severe asthma exacerbation admitted at the Queen Sirikit National Institute of Child Health. Twenty-eight patients were randomized to receive three intermittent doses of nebulized or intravenous MgSO4. The Modified Wood's Clinical Asthma Score was determined prior to, and at 20, 40, 60, 120, 180 and 240 minutes after treatment administration. The length of hospital stay was also recorded.
Fifteen patients received nebulized isotonic MgSO₄ and 13 were administered intravenous MgSO₄. There were no differences in the baseline characteristics of the two groups, including their initial asthma severity scores (4.87 ± 0.92 vs. 5.0 +0.82; p = 0.69). No statistically significant differences between the two groups were identified at 60 minutes (2.47 ± 0.83 vs. 2.77 ± 0.93; p = 0.37) until 240 minutes. The length of hospital stay for both groups was also similar (4.0 ±1.2 vs. 4.54 ± 2.7; p = 0.51). No adverse effects from MgSO₄ administration were observed among the participants.
In this small sample size we demonstrated that nebulized MgSO₄ and intravenous MgSO₄ are both clinically beneficial and safe for Thai children suffering from severe asthma exacerbation.
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FFLJ, NUK, ODKLJ, UL, UM, UPUK
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