Pediatric pulmonology
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Pediatric pulmonology · Feb 2014
Clinical TrialHigh-dose ibuprofen is not associated with increased biomarkers of kidney injury in patients with cystic fibrosis.
High-dose ibuprofen (IBU) may slow the decline of lung function in patients with cystic fibrosis (CF), but its use has been limited due to concerns over renal and gastrointestinal toxicity. In this pilot study, we examined the association of IBU with markers of acute kidney injury (AKI) in patients with CF. The effect of aminoglycoside (AG) exposure on AKI biomarkers was also examined. ⋯ There was no significant association between IBU treatment and KIM-1, NAG or protein levels, compared to patients never treated with IBU. While there was an association between AG courses and KIM-1 levels, there were no differences in biomarker levels between IBU and non-IBU groups with respect to AG courses. These preliminary results suggest that IBU treatment in patients with CF may be safe with respect to renal toxicity.
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Pediatric pulmonology · Feb 2014
Case ReportsHeterozygous 24-polyalanine repeats in the PHOX2B gene with different manifestations across three generations.
Congenital central hypoventilation syndrome (CCHS) is an uncommon genetic disorder that is characterized by alveolar hypoventilation and autonomic dysregulation. More than 90% of the patients are heterozygous for polyalanine repeat expansion mutations in the paired-like homeobox 2b (PHOX2B) gene. The normal genotype has a 20-polyalanine sequence whereas expanded alleles are usually 25-33. ⋯ The 3-year-old proband developed cor pulmonale and central hypoventilation following an upper respiratory tract infection. Our findings add to the accumulating evidence that the 24-polyalanine repeat in the PHOX2B is a disease-causing mutation. In addition, a high index of suspicion and careful monitoring after anesthesia, sedation, or respiratory illnesses should be exercised when evaluating asymptomatic family members with this genotype.
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Pediatric pulmonology · Jan 2014
Comparative StudySpirometric reference values in urban children in Madagascar: poverty is a risk factor for low lung function.
Studies about children with respiratory diseases in Africa are impeded by the dearth of reliable data for the vast majority of countries on the continent. This study was conducted to establish representative reference values, therefore allowing a more accurate evaluation of lung function in Malagasy children. ⋯ The equations established for school children in Madagascar's capital Antananarivo showed lung function values were lower than reference values for the same age group seen not only in European, but also in African American and African children. The unique ethnicity of the Malagasy people, which combines Southeast-Asian with substantial African influences, the heavy burden of pollution and poverty may explain these differences.
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Pediatric pulmonology · Jan 2014
Duplication in CHIT1 gene and the risk for Aspergillus lung disease in CF patients.
Aspergillus often persists in the respiratory tract of patients with Cystic Fibrosis (CF) and may cause allergic broncho-pulmonary aspergillosis (ABPA). Chitinases are enzymes that digest the chitin polymer. Plants use chitinase as a defense mechanism against fungi. Chitotriosidase (CHIT1) is the major chitinase in human airways. Variation in the coding region with 24-bp duplication allele results in reduced CHIT1 activity. Recently, CHIT1 duplication heterozygocity was found in 6/6 patients with severe asthma and fungal sensitization (SAFS). ⋯ CHIT1 duplication is not found in all CF patients with ABPM in contrast to patients with SAFS. These results suggest that CHIT1 duplication cannot be the sole explanation for Aspergillus positive sputum in CF patients.
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Pediatric pulmonology · Dec 2013
Variability and accuracy in interpretation of consolidation on chest radiography for diagnosing pneumonia in children under 5 years of age.
Consolidation on chest radiography is widely used as the reference standard for defining pneumonia and variability in interpretation is well known but not well explored or explained. ⋯ Overall agreement for identification of consolidation on chest radiographs was good, but agreement adjusted for chance was only moderate and did not vary with patient characteristics. Clinicians need to be aware that chest radiography is an imperfect test for diagnosing pneumonia and has considerable variability in its interpretation.