89:, 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.
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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
142:, District of South Carolina, 1790–1906; (National Archives Microfilm Publication M1183, 1 roll); Records of District Courts of the United States, Record Group 21; National Archives, Washington, D.C.
43:. He emigrated to the United States via Vanceboro, Maine, in 1928 and was subsequently naturalized as a citizen of the United States. He died in 2003 at the age of 99 years.
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81:. Complications include retinitis pigmentosa, degenerative changes in the central nervous system involving the cerebellum and long tracts, fatty
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62:(LDL) and very low density lipoprotein (VLDL). Individuals with this condition are unable to properly digest fats. Symptoms include
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Bassen F A & Kornzwelg A L. "Malformation of the erythrocytes in a case of atypical retinitis pigmentosa."
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trait. It is also commonly recognized as a betalipoprotein deficiency or abetalipoproteinemia .
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Social
Security Death Index. Frank Bassen, 14 November 1903 – 17 February 2003.
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77:(burr-cell malformation of the erythrocytes), and the reduction or even absence of B-
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54:, a rare autosomal recessive disorder in which the body fails to produce
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Barry G. Firkin and Judith A. Whitworth. "Bassen-Kornzweig
Syndrome."
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and other forms of nerve dysfunction. Treatment includes vitamin E.
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The
Parthenon Publishing Group. 1989. New edition in 2002.
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The
Parthenon Publishing Group. 1989. New edition in 2002.
73:
Characteristics of the syndrome include the presence of
19:(14 November 1903 – 17 February 2003) practiced as a
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Twenty-first Taber's
Cyclopedic Medical Dictionary,
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27:in New York, 1933–1978, in affiliation with
126:Barry G. Firkin and Judith A. Whitworth,
255:Canadian emigrants to the United States
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154:Frank A. Bassen, M.D. (Paid Obituary).
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97:Jews. The disease is transmitted as an
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140:Record of Admissions to Citizenship
31:. He was born 14 November 1903 in
14:
205:Dictionary of Medical Eponyms.
167:Mondofacto Medical dictionary.
219:"Bassen Kornzweig's Disease."
128:Dictionary of Medical Eponyms.
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39:, Canada and graduated from
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169:Bassen-Kornzweig disease
52:Bassen-Kornzweig disease
223:Unbound Medicine. 2009.
250:American hematologists
68:peripheral neuropathy
178:. 27 September 1997.
50:, Bassen identified
46:In partnership with
29:Mount Sinai Hospital
99:autosomal recessive
17:Frank Albert Bassen
174:2010-02-06 at the
158:23 February 2003.
48:Abraham Kornzweig
41:McGill University
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60:lipoprotein
234:Categories
33:St. George
95:Ashkenazi
25:internist
172:Archived
83:diarrhea
105:Sources
189:Blood.
87:ataxia
64:ataxia
23:and
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