Successful allogeneic peripheral blood stem cell transplantation in Wiskott-Aldrich Syndrome Patients: first report in Thailand.

Wiskott-Aldrich syndrome (WAS), an X-linked recessive disorder, is characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody production, eczema, and thrombocytopenia. Stem cell transplantation is the only curative therapy. To evaluate the use of allogeneic peripheral stem cell transplantation (PBSCT) in this group of patients, we performed allogeneic PBSCT in two WAS patients (3 and 12 years old). The conditioning regimen consisted of busulfan 4 mg/kg/day for 4 days, and cyclophosphamide 50 mg/kg/day for 4 days. Graft-versus-host disease prophylaxis was consistent with cyclosporin A and methotrexate. Peripheral blood stem cells were collected from their brother donors (6 and 16 years old) by continuous flow leukapheresis after mobilization with granulocyte-colony-stimulating factor at a dose of 7.5 microg/kg/day for 5 days. Both recipients achieved neutrophils engraftment on days 11 and 12. The first patient achieved platelets engraftment on day 30. The second patient did not have platelet count below 20.0 x 10(9)/l during PBSCT procedure. Both did not develop acute or chronic graft-versus-host disease. At present, they are healthy after PBSCT. The follow up time after transplantation is 1,170 days and 269 days, respectively. Allogeneic PBSCT is economically feasible for WAS. The cost of PBSCT in Thailand is 20 to 30% less than bone marrow and cord blood stem cell transplantation. The cost of the transplant procedure for each patient in Thailand is US $ 12,000. This study is the first report of a successful stem cell transplantation in WAS patients in Thailand.

Peak expiratory flow rate values of students in Bangkok.

BACKGROUND: As there are considerable variations in normal values of peak expiratory flow rate (PEFR) shown by studies from various population, a study is required to obtain normal values of PEFR in Thai children. OBJECTIVE: To determine the values of PEFR of students in Bangkok. METHODS: In a cross sectional study of PEFR measured with standard Wright peak flow meter, 501 normal students, aged 5 to 15 years, from five public schools in Bangkok were investigated. In the selection process of subjects, strict criteria of "normality" were applied and included history of medical illnesses, physical examination and nutritional status. RESULTS: The relationship between PEFR and height was approximately linear in both male and female children. Prediction equations for each sex were: Male children: PEFR (L/min) = [3.52 x Height (cm)] - 186.80 Female children: PEFR (L/min) = [3.48 x Height (cm)] - 204.11 The PEFR values of students in this study were different from the predicted values of PEFR in those of previous reports in Thai children. These discrepancies might be explained by a variety of study population and environmental factors. CONCLUSIONS: The relationship between PEFR and height of students in Bangkok is best described by a regression equation. The prediction graphs for each sex may be used to monitor PEFR values of children with obstructive airway diseases and to compare an individual's PEFR with those of others of the same height and sex.

Fluticasone propionate and bronchial hyperresponsiveness in childhood asthma.

Bronchial asthma is now agreed as being a chronic inflammatory disease of the airways. Inhaled steroids are widely accepted as a preventive medication in asthmatic patients of all ages and severity. However, the optimal use of inhaled steroids and the important issue of safety and efficacy still remain of concern, particularly in children. Recently, fluticasone propionate (FP) has been developed for use as an inhaled preparation for the treatment of asthma. Because of its high topical potency and increased lipophilicity, it is claimed that FP has an improved risk/benefit compared with other inhaled steroids. In order to evaluate the use of FP in children, we have studied the efficacy of high dose FP (500 microg/day) in asthmatic children. Thirteen children (9 boys and 4 girls), aged 7-17 years (10.8 +/- 2.6), were instructed to use a pressurized metered-dose inhaler connected to a Volumetric spacer. The standard methacholine bronchial challenge test was used as a principal outcome parameter. The PD20, a cumulative dose of methacholine inducing a 20% decrease in FEV1, was measured pre- and post-treatment with inhaled FP. After 4 weeks of FP, PD20 significantly increased from 21.6 +/- 14.3 inhalation unit to 106.6 +/- 78.5 inhalation unit (4.9 fold, p = 0.004) reflecting the improvement of airway reactivity. All subjects improved clinically. These results demonstrate that the anti-inflammatory action of FP 500 microg a day for four weeks can markedly reduce bronchial hyperresponsiveness, the basic physiologic abnormality in bronchial asthma.

Aeroallergen in Thai asthmatic children.

Intradermal skin-teat reactions in 521 Thai asthmatic children (345 males and 176 females), aged between 3 and 15 years, with an average age of 6.8, were studied at the allergy clinic of Ramathibodi Hospital. Of the total, 456 (87 per cent) patients gave positive immediate skin reaction at least one “common aeroallergen”. The mist commonly seen aeroallergens were house dust (75 per cent) and house dust mite (73 per cent), followed by mold (55 per cent), grass ( per cent), weed (43 per cent), kapox (41 per cent) and cockroach (18 per cent). The finding of this study was that the most important aeroallergens were house dust and house dust mites which was similar to the results previously found in similar studies.

Incorrect use of metered dose inhaler by pediatric residents.

Currently press-and-breath metered dose inhalers (MDIs) are widely prescribed but are often difficult for many patients to properly use. However many medical personnel cannot use the MDIs correctly. We administered a question and observed usage of a placebo metered dose inhaler with ad-on spacer (Nebuhaler) among 127 second year pediatric residents. Forty-eight per cent of the residents performed at least six of nine steps correctly. The two most common errors made by participants were not to place mouthpiece tightly between teeth and lips (64%) and failure to breath out to functional residual capacity before actuation (55.3%). Of the residents with improper timing of actuation (49%) all actuated the canister before starting inhalation. (5 seconds or longer time). We conclude that (1) pediatricians should have additional instruction in proper MDIs usage and practice with the asthmatic child and (2) routine assessment of MDIs technique should be instituted as standard practice care.