Abraham Kornzweig
Encyclopedia
Abraham Leon Kornzweig, born in New York, a physician and ophthalmologist specializing in geriatric ophthalmology. He opened a new field in investigative medicine, and founded the Society of Geriatric Ophthalmology. He was also widely known as the co-discoverer and namer of Bassen-Kornzweig Syndrome, also called Abetalipoproteinemia
. It was first noted by the United States physician
Frank Bassen
, who partnered with Kornzweig to identify and describe causes and symptoms of the disease.
in 1922, and from the New York University Medical School in 1925. From 1925 to 1928, He trained as an intern at Mount Sinai Hospital, New York
, including study in pathology under the well-known Bernard Samuels, after which he enetered private practice until 1934. He returned to Mount Sinai for a residency in ophthalmology and in 1936, received a diplomate. For 17 years, he taught at NYU-Bellevue Medical School, where he was an Associate Clinical Professor; he subsequently taught at Mount Sinai School of Medicine, where he was Clinical Professor, and eventually Emeritus Professor, of Ophthalmology. He was also Director of Research and Chief of Ophthalmology at the Jewish Home and Hospital for the Aged.
He retired in poor health in 1972, although he continued to work on his research projects until his death in 1982.
He married Chifra Goldberg, a naturalized citizen, born in Yass, Rumania and they had one daughter.
s, a low density lipoprotein
(LDL) and very low density lipoprotein (VLDL). Individuals with this condition are unable to properly digest fats. Symptoms include ataxia
, peripheral neuropathy
and other forms of nerve dysfunction. Treatment includes vitamin E.
Characteristics of the syndrome include the presence of acanthocytes (burr-cell malformation of the erythrocytes), and the reduction or even absence of B-lipoproteins. Complications include retinitis pigmentosa, degenerative changes in the central nervous system involving the cerebellum and long tracts, fatty diarrhea
, ataxia
, areflexia, demyelination, defective intestinal lipid absorption with low serum cholesterol level, intestinal malabsorption, amaurosis, retarded growth, and steatorrhea. Intellectual development may be slightly retarded.
Many afflicted with the syndrome are unable to walk.
The syndrome appears in infancy. Affected children appear normal at birth but usually fail to thrive during their first year. The syndrome may predominate in males (71%). Most cases occur in children of Jewish descent, especially among Ashkenazi Jews. The disease is transmitted as an autosomal recessive trait. It is also commonly recognized as a betalipoprotein deficiency or abetalipoproteinemia.
Abetalipoproteinemia
Abetalipoproteinemia, or Bassen-Kornzweig syndrome, is a rare autosomal recessive disorder that interferes with the normal absorption of fat and fat-soluble vitamins from food. It is caused by a deficiency of apolipoprotein B-48 and B-100, which are used in the synthesis and exportation of...
. It was first noted by the United States physician
Physician
A physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
Frank Bassen
Frank Bassen
Frank Albert Bassen, , practiced as a haematologist and internist in New York, 1933–1978, in affiliation with Mount Sinai Hospital. He was born 14 November 1903 in St. George, New Brunswick, Canada and graduated from McGill University. He emigrated to the United States via Vanceboro, Maine, in 1928...
, who partnered with Kornzweig to identify and describe causes and symptoms of the disease.
Biography
Born in New York City, Kornzweig graduated from Columbia UniversityColumbia University
Columbia University in the City of New York is a private, Ivy League university in Manhattan, New York City. Columbia is the oldest institution of higher learning in the state of New York, the fifth oldest in the United States, and one of the country's nine Colonial Colleges founded before the...
in 1922, and from the New York University Medical School in 1925. From 1925 to 1928, He trained as an intern at Mount Sinai Hospital, New York
Mount Sinai Hospital, New York
Mount Sinai Hospital, founded in 1852, is one of the oldest and largest teaching hospitals in the United States. In 2011-2012, Mount Sinai Hospital was ranked as one of America's best hospitals by U.S...
, including study in pathology under the well-known Bernard Samuels, after which he enetered private practice until 1934. He returned to Mount Sinai for a residency in ophthalmology and in 1936, received a diplomate. For 17 years, he taught at NYU-Bellevue Medical School, where he was an Associate Clinical Professor; he subsequently taught at Mount Sinai School of Medicine, where he was Clinical Professor, and eventually Emeritus Professor, of Ophthalmology. He was also Director of Research and Chief of Ophthalmology at the Jewish Home and Hospital for the Aged.
He retired in poor health in 1972, although he continued to work on his research projects until his death in 1982.
He married Chifra Goldberg, a naturalized citizen, born in Yass, Rumania and they had one daughter.
Disease
Bassen-Kornzweig disease, also called Bassen-Kornzweig Syndrome, is a rare congenital disorder in which the body fails to produce chylomicronChylomicron
Chylomicrons are lipoprotein particles that consist of triglycerides , phospholipids , cholesterol and proteins .They transport dietary lipids from the intestines to other locations in the body...
s, a low density lipoprotein
Lipoprotein
A lipoprotein is a biochemical assembly that contains both proteins and lipids water-bound to the proteins. Many enzymes, transporters, structural proteins, antigens, adhesins, and toxins are lipoproteins...
(LDL) and very low density lipoprotein (VLDL). Individuals with this condition are unable to properly digest fats. Symptoms include ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, peripheral neuropathy
Peripheral neuropathy
Peripheral neuropathy is the term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of or trauma to the nerve or the side-effects of systemic illness....
and other forms of nerve dysfunction. Treatment includes vitamin E.
Characteristics of the syndrome include the presence of acanthocytes (burr-cell malformation of the erythrocytes), and the reduction or even absence of B-lipoproteins. Complications include retinitis pigmentosa, degenerative changes in the central nervous system involving the cerebellum and long tracts, fatty diarrhea
Diarrhea
Diarrhea , also spelled diarrhoea, is the condition of having three or more loose or liquid bowel movements per day. It is a common cause of death in developing countries and the second most common cause of infant deaths worldwide. The loss of fluids through diarrhea can cause dehydration and...
, ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, areflexia, demyelination, defective intestinal lipid absorption with low serum cholesterol level, intestinal malabsorption, amaurosis, retarded growth, and steatorrhea. Intellectual development may be slightly retarded.
Many afflicted with the syndrome are unable to walk.
The syndrome appears in infancy. Affected children appear normal at birth but usually fail to thrive during their first year. The syndrome may predominate in males (71%). Most cases occur in children of Jewish descent, especially among Ashkenazi Jews. The disease is transmitted as an autosomal recessive trait. It is also commonly recognized as a betalipoprotein deficiency or abetalipoproteinemia.
Publications
Kornzweig's publications include over 50 articles and books, beginning in 1948 with a series of articles on the "Eye in Old Age", and concluding with, in 1980, "New Ideas for the Old Eye."Partial list
- Frank A. Bassen, M.D. and Abraham L. Kornzweig, M.D. "Malformation of the Eyrthrocytes in a Case of atypical Reinitis Pigmentosa." Blood, 1950, Vol. 5, No. 4, pp. 381–387.
- Abraham L. Kornzweig, M.D.; Frank A. Bassen M.D. "Retinitis Pigmentosa, Acanthrocytosis, and Heredodegenerative Neuromuscular Disease." AMA Arch Ophthalmolology. 1957;58(2):183–187.
- Abraham L. Kornzweig, MD; Ira Eliasoph, MD; Morris Feldstein, MD. "Selective Atrophy of the Radial Peripapillary Capillaries in Chronic Glaucoma." Archives of Ophthalmolology. 1968;80(6):696–702.
- Abraham L. Kornzweig, MD; Ira Eliasoph, MD; Morris Feldstein, MD. "The Retinal Vasculature in Macular Degeneration." Archives of Ophthalmolology. 1966;75(3):326–333.
- Abraham L. Kornzweig, MD; Ira Eliasoph, MD; Morris Feldstein, MD. "Occlusive Disease of Retinal Vasculature." Archives of Ophthalmolology. 1964;71(4):542–551.
- Morris Feldstein, M.D.; Abraham L. Kornzweig, M.D.; Julius Schneider, M.D. "OCULAR SURGERY IN THE AGED." Journal of the American Medical Association. 1959;170(14):1621–1625.