Liver disease is an independent risk factor for mortality in CF.

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F umarate hydratase (FH) or fumarase deficiency (FD) is an extremely rare (Online Mendelian Inheritance in Man no. 606812) inherited metabolic disease with the majority of infants presenting with growth failure, developmental delay, and severe neurological involvement. FH is an essential enzyme of the tricarboxylic cycle and exists in 2 isoforms: mitochondrial (nuclear encoded), which catalyses the reversible hydratation of fumarate to malate, and cytosolic, which metabolises fumarate released by

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