Chronic lung disease of prematurity (CLD) remains to be a major cause of morbidity and mortality in premature infants.We made a neonatal mouse model of CLD by hyperoxia exposure and examine the effects with hepatocyte growth factor (HGF) on functional and pathological abnormalities in this model.
Neonatal animals of BALB/C and CD-1 mice were kept on room air or hyperoxia (60 or 90%) since the 1st postnatal day.On 21 days of age, pulmonary function and bronchial hyperresponsiveness (BHR) utilizing a barometric plethysmographic system, and histological changes in the lung were examined (experiment 1).Neonatal CD-1 mice were exposed to room air or 90% oxygen for 7 days since 3 days of age.HGF was administered to the half of hyperoxic animals.On 17 days of age, BHR and histological changes in the lung were examined (experiment 2).
On experiment 1, mice exposed to hyperoxia exihibited abnormal pulmonary function test results, BHR, and decreased alveolarization.In experiment 2, HGF significantly reduced BHR and tended to improve alveolarization in hyperoxia exposed CD-1 mice.
In conclusion, this model shows functional and pathological abnormalities characteristic to CLD and HGF might attenuate these abnormalities.

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It has been reported that KL-6 is a clinically useful marker for various interstitial lung diseases in clinical studies.
KL-6 values in children without respiratory disease is less than 250U/ml.Patients with Severe Motor and Intellectual Disabilities (SMID) have severe lower respiratory infections such as pneumonia. This accounts for more than half the deaths of these children.Children with severe respiratory complications who need respiratory care in SMID have increased.In medical management of SMID, it is very important to care for and treat the respiratory complications.The purpose of the present study was to examine correlations between levels of KL-6 and factors such as tracheostomy, tube feeding, thoracic deformity and locomotor abilities.Some patients with a tracheostomy and chronic respiratory disease showed higher KL-6 levels compared to those with no respiratory disease.
But also those with no chronic respiratory disease and with no tube feeding but a thoracic deformity and pneumonia caused by gastro-esophageal reflux also had high KL-6 levels.Serum KL-6 would provide valuable information on the management of patients in SMID with respiratory complications.

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This article describes the results of a clinical study conducted to assess the effects of inhalation of an isotonic, in place of hypotonic, solution of disodium cromoglycate (DSCG) on the pulmonary function parameters and severity of cough in asthmatic children.The retrospective study included 68 asthmatic children who developed asthma before the age of six years, and who were receiving treatment with inhaled disodium cromoglycate (hypotonic solution).The DSCG solution was changed to an isotonic from hypotonic solution in January 2004.Significantly greater improvement of the pulmonary function parameters was observed following inhalation of isotonic DSCG solution than that following inhalation of the hypotonic solution.For the same age, the% V₅₀, % V₂₅ were significantly higher in the group inhaling isotonic DSCG solution than in that inhaling the hypotonic DSCG solution.The number of cough was significantly lesser in the children inhaling the isotonic DSCG solution than in those inhaling the hypotonic DSCG solution.

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The pathogenesis of repiratory infections due to nontypable Haemophilus influenzae (NTHi) begins with bacterial colonization of mucosa. Adhesins such as hemagglutinating pili (Hif A), HMW1/2, Hia, Hap are important in this process.Recently, biofilm formation of NTHi was reported to play a role in the pathogenesis of otitis media and the respiratory infections.However, there are few reports on the prevalence of adhesins and the biofilm formation among clinical isolates of NTHi. We examined 108 strains of NTHi for adhesin genes and a biofilm-related gene by PCR, and for biofilm formation by a quantitative biofilm assay.The prevalence of adhesin genes were as follows: hif A 22.2%, hmw 48.1%, hia 55.6%, hap 22.2%, sia B 38.0%.Variability among isolates was observed in their ability to form biofilms.The rate of a biofilm-positive strain was significantly higher in hif A or hia positive strains than negative strains (p =0.0245 and 0.0360, respectively).Furthermore, the incidence of a biofilm-positive strain was higher among strains from patients with recurrent respiratory infections than those from patients with acute infections (89.5% vs.63.2%).We conclude that hemagglutinating pili and Hia are associated with bioflm formation, and that biofilm formation plays an important role in recurrent respiratory infections.

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We experienced a 4month-old girl with spinal muscular atrophy type 1 (SMA1) with breathing stabilized using noninvasive ventilation (NIV) and mechanically assisted coughing (MAC).She was diagnosed with SMA1 at the age of 3 months.At 4 months her respiratory muscle weakness and bulbar palsy had worsened.At 6 months, because of severe respiratory distress, we introduced NIV and MAC.Her breathing state improved and she was discharged from hospital at the age of 13 months.
Pulmonary physiotherapy applied by a physiotherapist promoted synchronization with NIV and MAC.We also trained her parents in pulmonary physiotherapy, suction removal of airway secretion and the use of NIV and MAC.NIV and MAC were valuable in the breathing management of SMA1, but close cooperation between medical staff and the patient's family was necessary for proper use.

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We present a girl with congenital central hypoventilation syndrome (CCHS) whose development improved with change in ventilation methods, and with nutritional management.She had shown developmental delay gaining head control only at 9 months and could still not sit at 25 months. Her respiratory status worsened due to aspiratory infections.She was initially treated with noninvasive positive pressure ventilation (NPPV) by nasal mask, however, her respiratory status did not improve. The nasal mask was replaced with an oronasal mask that fastened more securely to her face. Nutritional management with gastric tubing was initiated at the age of 28 months.As her respiratory function improved, she showed remarkable progress in development, gaining gross motor skills and some verbal speech.We believe maintaining good respiratory function is vital for acquiring psychomotor skills in CCHS.

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