We describe a 30-month-old boy with multiple anomalies and mental retardation with hereditary spherocytic anemia. His karyotype was 46,XY,del(8)(p11.23p21.1). Genes for ankyrin and glutathione reductase (GSR) were localized to chromosome areas 8p11.2 and 8p21.1, respectively. Six patients with spherocytic anemia and interstitial deletion of 8p- have been reported. In these patients, severe mental retardation and multiple anomalies are common findings. This is a new contiguous gene syndrome. Lux et al. [1990: Nature 345:736-739] established that ankyrin deficiency and associated deficiencies of spectrin and protein 4.2 were responsible for spherocytosis in this syndrome. We reviewed the manifestations of this syndrome. Patients with spherocytic anemia and multiple congenital anomalies should be investigated by high-resolution chromosomal means to differentiate this syndrome.

Background Ultrasonic, jet, and mesh nebulizers have all been used in the treatment for asthma. Mesh nebulizers reportedly offer the best inhalation efficiency. Methods This study aimed to clarify the utility of the mesh nebulizer, compared to jet nebulizers, in the treatment of pediatric asthma patients. Participants included 88 children <6 years old who were receiving treatment for asthma at Murayama Pediatric Clinic. Heart rate, peripheral oxygen saturation in arterial blood, and Mitsui symptom scores were compared before and after treatment with a mesh nebulizer (n = 43) or jet nebulizer (n = 45) using a salbutamol inhalation solution (0.2 ml for children ≧ 2 years old, n = 51; 0.1 ml for children < 2 years old, n = 37). Results Other than required inhalation time, clinical findings did not differ between mesh and jet groups. In both groups, heart rate increased significantly in patients treated with 0.2 ml (1000 microg) of salbutamol. Conclusions and Clinical Relevance The required inhalation time of the mesh nebulizer was superior to the jet nebulizer. Children ≧ 2 years with mild asthma attacks experienced a significantly increased heart rate in both groups. The dose of salbutamol (0.2 ml for ≧2 years) used for asthma attacks should be reconsidered in mild asthma.

Steroid inhalation is the standard bronchial asthma therapy and it includes powdered metered doses, dry powder, and nebulizer suspension. However, particle sizes vary widely. The research goal was to demonstrate that different budesonide administration forms and devices have various deposition rates in the airway obstruction region. Here, we compared relative inhalation therapy efficacies and identified therapies that delivered the highest drug doses to the airway obstruction region. Weibel's anatomy data were used to identify the airway obstruction region in asthma. Based on European Standardization Committee data, we investigated the diameters of the drug particles being deposited there and evaluated the average particle size and distribution of the budesonide dosage forms and application devices. Drug dose depositions were measured by HPLC at each stage of a Cascade Impactor. Weibel's anatomy data indicated that the 1st–4th bronchial generations comprised the airway obstruction region and corresponded to the tracheobronchial area. According to the European Standardization, particles 2–6 µm in diameter were readily deposited there. The proportions of particles in this size range were 33.0%, 32.0%, 59.0%, and 78.0% for Turbuhaler, Symbicort, mesh-type NE-U22 suspension, and jet-type NE-C28 suspension, respectively. We localized the airway obstruction regions of bronchial asthma and identified the optimal inhalation therapy particle size. An electric nebulizer was more efficacious for budesonide administration than dry powder delivery. The NE-C28 treatment deposited 2.36x more budesonide in the airway obstruction region than dry powder delivery systems.

People all over the world should work in each individual against global warming due to greenhouse gas that is made up of a majority of carbon dioxide. On the other hand chloro-fluoro-carbon (CFC) was used with pressured metered-dose inhaler steroid therapy, but CFC became banning the use because of ozone depleting substance. Hydrofluorocarbon (HFA134a, tetra-fluoro-methane) is used as alternative CFC until now. Less-famously hydro-fluoro-carbon (HFA134a) have 1300-fold (mole ratio) energy of heat-trapping relative to carbon dioxide.On an extremely localized story, we derived substantial gravity of carbon dioxide from sales total of pressured metered-dose inhaler (pMDI) steroid drugs for the year in Japan. The amount of total sales of inhaled corticosteroid drugs on annual 2006 year was 320 hundred-millions yen. 88 hundred-millions yen (27.4% for total ICS sales) was accounted for pressured metered-dose inhaler steroid. Now in Japan there are three kinds of pressured metered-dose inhaler steroid drugs which all use tetra-fluoro-methane (HFA134a). In fact total gravity of tetra-fluoro-methane (HFA134a) from pressured metered-dose inhaler steroid for annual 2006 year was 19.7 t and substantial gravity of carbon dioxide was 10.8 thousand ton. As total gravity of carbon dioxide production throughout the year in Japan was 13 hundred-million ton. Therefore substantial gravity of carbon dioxide by steroids pressured metered-dose inhaler was very small (0.001%) compared to total carbon dioxide production in Japan. Until today carbon-dioxide reducing make very slow progress, for that reason medical service worker unexceptionally should exert an effort for carbon-dioxide reduction if only slightly through the daily clinical examination.

Objective Eosinophilic inflammation is thought to play a role in childhood asthma. Thus, examination of nasal eosinophils, instead of sputum, may be useful for the diagnosis of not only eosinophil-positive rhinitis but also persistent asthma. Nasal eosinophil examination is a routine for the diagnosis of nasal eosinophil-positive rhinitis in patients with rhinorrhea symptoms. This retrospective study investigated whether testing for nasal discharge eosinophils is useful for diagnosing childhood allergic asthma and whether nasal discharge eosinophils are predictive of persistent asthma. Methods Infants and young children (n = 180) with at least 3 recurrent episodes of respiratory symptoms and bronchodilator inhalation improvements at intervals of ≥1 week were divided into the asthma group and the nonasthma group, and the presence or absence of nasal discharge eosinophils was examined by age. Correlations between nasal discharge eosinophils and other predictive factors for persistent asthma were also examined. Results The evaluation of nasal discharge eosinophils in the asthma group showed a significantly higher positive rate in older age groups than in the 0–1-year-old age group (p < 0.05–0.001). However, none of the asthma patient groups had any significant changes between the 0–1-year-old and older groups. This pattern was similar for other risk factors, showing correlations between nasal discharge eosinophils and other predictive factors. Conclusions Testing for nasal discharge eosinophils with asthma symptoms increases with age. Nasal discharge eosinophils with asthma symptoms may be a predictive factor for persistent asthma. This trial is registered with UMIN Clinical Trials (registration number UMIN000030776).