CF patients who have pancreatic insufficiency and carry mutations associated with a severe or a variable genotype are at increased risk to develop liver disease.

Among infants with respiratory distress, plasma N-BNP measurements can differentiate between acute heart failure and lung disease and can be used to monitor the effects of treatment for infants with heart failure.

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Interstitial lung diseases in infants and children are uncommon and may be caused by specific inborn errors of surfactant metabolism. Five children with open lung biopsy diagnosed interstitial lung disease were followed (mean of 27.2 years) and evaluated for surfactant protein gene mutations. Four of the children were originally diagnosed as desquamative interstitial pneumonitis and one as chronic interstitial pneumonitis. All had good response to chloroquine or hydroxychloroquine treatment for periods of 7-38 months. Lung function tests, incremental exercise tests, and rentgenological studies were performed in the children. Surfactant protein gene mutations were searched in all the patients and in part of their families. Three of the patients, aged now 32, 29, and 37 years, feel well and have normal lung function, while two of the patients, both females, aged 28 and 37 years, conduct normal activities of daily living, have healthy children but have clinical, physiological and rentgenological evidence of restrictive lung disease. All five patients were found to have surfactant protein C gene (SFTPC) mutations, three of them with the most common mutation (p.I73T) and the other two with new mutations of surfactant protein C gene (p.I38F and p.V39L). We conclude that detection of surfactant protein mutations should be attempted in all children presenting with interstitial lung disease. Furthermore, treatment with hydroxychloroquine should be considered in children with SFTPC mutations. Prospective evaluation of hydroxychloroquine therapy in a greater number of patients is needed.

Reversible storage of hydrogen in the form of stable and relatively harmless chemical substances such as formic acid (FA) is one of the cornerstones of a fossil-fuel-free economy. Recently, Ru(III)-PC(sp 3 )P (where PC(sp 3 )P = modular dibenzobarrelene-based pincer ligand possessing a pendant functional group) complex 1 has been reported as a mild and Eselective catalyst in semihydrogenation of alkynes with stoichiometric neat formic acid. Discovery of the additive-free protocol for dehydrogenation of FA launched further studies aiming at the rational design of highly efficient catalysts for this reaction operating under neutral conditions. We now report the results of our investigation on a series of bifunctionl PC(sp 3 )P complexes equipped with different outer-sphere auxiliaries, that allowed us to identify an amine-functionalized Ir(III)-PC(sp 3 )P complex 3, as a clean and efficient catalyst for the FA dehydrogenation. The catalyst is suitable for fuel-cell applications demonstrating a TON up to 5 × 10 5 and TOF up to 2 × 10 4 h −1 (3.8 × 10 5 and 1.2 × 10 4 h −1 with no additives). In addition to the practical value of the catalyst, experimental and computational mechanistic studies provide rationale for the design of improved next-generation catalysts.