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

The percentage of children and adolescents with the highest risk of complications decreased markedly. The distribution of HbA1c better depicts the glycemic control in a population than the mean or the percentage of patients reaching the target (7.5%). HbA1c was more strongly associated with general health perception than with therapeutic regimens and diabetes knowledge.

All in all, 120 patients were treated (107 male, 13 female) (mean age 62 years, range 30-84 years). In 49% the little finger, in 44% the ring finger, in 4% the middle finger, and in 3% the index finger were treated. Full release was accomplished in 71%, partial release in 26%, and no change in 3% of the patients. The median contracture before the treatment was 37° for the metacarpophalangeal (MP) joint (range 25-100°) and 51° for the proximal interphalangeal (PIP) joint (range 30-97°). After 12 months, the mean contracture for the MP joint was 9° (range 0-25°) and 21° (range 10-36°) or the PIP joint. Adverse events occurred in 96% of patients within 3 months after treatment. No tendon ruptures, anaphylactic reactions, nerve, or ligament injuries were observed.

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Complications of type 2 diabetes were frequently observed in the study population. With regard to neuropathy, nephropathy and retinopathy type-2-diabetics should be examined even more thoroughly.