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Dr. L. Lahut Uzman, Bronson Crothers Professor of Neurology at the Harvard Medical School, died last Wednesday after a brief illness.
Only 39 years old, Dr. Uzman was one of the country's foremost neurologists. His most important work led to the determination of the cause of Wilson's disease, or hepatolenticular degeneration. This is a genetic desease involving cirrhosis of the liver (progressive destruction of the cells), a smoky brownish ring around the corner of the eye, and a progressive degeneration of part of the brain known as the lenticular nucleus, resulting in tremors and rigidity of the body.
Later, in 1951, while working with Dr. Sidney Farber, professor of Pathology at Harvard, Dr. Uzman isolated and described the lipoprotein in Gaucher's disease, an anemia of the spleen that runs in families and is particularly common among Jews.
Dr. Uzman showed that analysis of fat-like lipid, kerasin, as performed over the previous 40 years, was not enough to explain the peculiar Gaucher cells, sign of the disease. It is also necessary to analyse the deranged metabolism of cell proteins, believed to have an abormal affinity for kerasin. Kerasin is stored in the spleen and causes its enlargement, as well as enlargement of lymph nodes.
Also while at the Children's Cancer Research Foundation, an affiliate of the Children's Hospital Medical Center, Dr. Uzman investigated another so-called lipid storage disease, gargoylism, a rare disease producing gargoyle-like deformities of face and body as well as disorders of brain, liver, and spleen. The disease is inborn and leads to mental deficiency.
Gargoylism previously had been thought to be primarily a lipid disease, but Dr. Uzman in 1955 reported that it involves an abnormality in the metabolism of certain polysaccharides, or starches, used in tissue-building.
More recently, Dr. Uzman conducted research aimed at correlating the chemical development of the nervous system with its structural maturation-- a pursuit important in providing a base line of information for future study of metabolic changes in the newborn child as well as congenital diseases in general
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