Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia. The condition gets its name from the distinctive sweet odor of affected infants' urine and earwax, particularly prior to diagnosis and during times of acute illness. == Signs and symptoms == The disease is named for the presence of sweet-smelling urine, similar to maple syrup, when the person goes into metabolic crisis. The smell is also detected in ear wax of an affected individual during metabolic crisis. In populations to whom maple syrup is...

Branched-chain keto acid dehydrogenase kinase deficiency (BCKDK deficiency) is a disease resulting from mutations of the BCKDK gene. Patients with BCKDK deficiency have low levels of branched chain amino acids (BCAA) in their organism due to accelerated breakdown of these essential amino acids. This results in delayed brain development, which may present as intellectual disability and autism spectrum disorder. Patients may suffer from epileptic seizure. == History == The disease was first described in 2012 in three unrelated families.Later on, García-Cazorla, Oyarzabal et al. confirmed that BCKDK...

== Latin == === Pronunciation === (Classical) IPA(key): /ˈduː.ra leːks sed leːks/, [ˈd̪uːrä ɫ̪eːks̠ s̠ɛd̪ ɫ̪eːks̠] (Ecclesiastical) IPA(key): /ˈdu.ra leks sed leks/, [ˈd̪uːrä lɛks sɛd̪ lɛks] === Proverb === dūra lēx, sed lēx The law is harsh but it is the law.

Boris Mikhailovich Moiseev (Russian: Бори́с Миха́йлович Моисе́ев; 4 March 1954 – 27 September 2022) was a Soviet and Russian singer, choreographer, dancer, writer, actor, head of a dance group, and author of popular shows in Russia. He was a Merited Artist of Russia (2006). == Early life == Boris Moiseev was born in prison in Mogilev, Byelorussian SSR, Soviet Union, his mother was being held as a disenfranchised element during the Soviet regime. He spent his childhood and teenage years among his Lithuanian Jewish aunts in Mogilev. To strengthen his health, Boris was sent to a dancing school. Since...