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Workers of the National Institute of Rheumatic Diseases (NIRD) in Piešťany prepared the first monograph in world literature called Alkaptonuria and ochronosis that was published by SAV publishing house in Bratislava in 1956. The authors of this original book were as follows: Professor Štefan Siťaj, MD, DSc., Professor Ján Červeňanský, MD, DSc., and Associate Professor Tibor Urbánek, PhD. In their practice, the next generations of physicians and scientific workers of NIRD Piešťany paid a lot of attention to serious diseases like alkaptonuria and ochronosis because their incidence in Slovakia is extraordinarily high. On the occasion of the 20th anniversary of the death (September 1990) of the founder of Slovak rheumatology, Professor Štefan Siťaj, MD, DSc., the authors decided to prepare a new supplemented edition of this monograph. The book was enriched with the knowledge gained during those 20 years with particular respect to diseases of metabolism of aromatic amino acids, genetics and the use of more recent imaging methods for the diagnostics of alkaptonuria and ochronosis. It was our intention to publish a monograph that would contribute to clinical enrichment of the physicians and scientific workers in the field of alkaptonuria and ochronosis.