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  • Hermansky-Pudlak syndrome
    Ç츣¸¸½ºÅ°-Ǫµé¶óÅ© ÁõÈıº
  • Hermansky-Pudlak syndrome => oculocutaneous albinism
    ¾È±¸ ÇǺΠ¹é»öÁõ
CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
Hermann's fixative <chemical> A hardening fixative of glacial acetic acid, osmic acid, and platinum chloride.
(05 Mar 2000)
Hermann, Friedrich <person> German anatomist, 1859-1920.
See: Hermann's fixative.
(05 Mar 2000)
Hermansky, F Hermansky <person> 20th century Czech physician.
See: Hermansky-Pudlak syndrome, Hermansky-Pudlak syndrome type VI.
(05 Mar 2000)
Hermansky-Pudlak syndrome <syndrome> A form of oculocutaneous albinism (autosomal recessive) with accumulation of ceroid in lysosomes with restrictive lung disease, granulomatous colitis, kidney failure, cardiomyopathy, and storage pool-deficient platelets.
(05 Mar 2000)
Hermansky-Pudlak syndrome type VI An autosomal recessive deficiency of pigment in skin, hair, and eyes; in the tyrosinase negative type, there is an absence of tyrosinase; in the tyrosinase positive type, there is normal tyrosinase which cannot enter pigment cells; it is transmitted by an autosomal recessive inheritance. The compound heterozygote is normal so the two forms are not allelic.
There are several types: type IA is characterised by absence of tyrosinase with life-long complete absence of melanin, marked photophobia, and nystagmus. Type IB, yellow albinism with low or absent tyrosinase; improves with age.
Type II, with normal tyrosinase activity is the most common; hair darkens and nevi and freckles develop.
Type III is characterised by absent tyrosinase but pigmentation of the iris in the first decade.
Type IV in Africans with normal tyrosinase.
Type V with red hair.
Type VI, Hermansky-Padlak syndrome, with haemorrhage due to platelet deficiency and low to absent tyrosinase.
Synonym: Hermansky-Pudlak syndrome type VI.
(05 Mar 2000)
CancerWEB ¿µ¿µ ÀÇÇлçÀü À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
Padykula-Herman stain <technique> For myosin ATPase, a technique similar to that of Gomori's non-specific alkaline phosphatase stain, except that incubation is carried out with ATP as the substrate at pH 9.4 in the absence of Mg++; enzyme activity is demonstrated as blackened deposits in the A band of striated muscle sarcomeres; control tissue sections lacking substrate and containing sulfhydryl inhibitors are necessary.
(05 Mar 2000)
Gartner, Herman <person> Danish anatomist and surgeon, 1785-1827.
See: Gartner's canal, Gartner's cyst, Gartner's duct.
(05 Mar 2000)
Vollmer, Herman <person> U.S. Paediatrician, 1896-1959.
See: Vollmer test.
(05 Mar 2000)
Wachter, Herman <person> German pathologist, *1878.
See: Bracht-Wachter lesion.
(05 Mar 2000)
Mosenthal, Herman Otto <person> American physician, 1878-1954.
See: Mosenthal test.
(05 Mar 2000)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 1 ÆäÀÌÁö: 1
  • Hermanski-Pudlak Syndrome - »õâ Syndrome characterized by the triad of oculocutaneous albinism (ALBINISM, OCULOCUTANEOUS); PLATELET STORAGE POOL DEFICIENCY; and lysosomal accumulation of ceroid lipofuscin.
    Synonyms : Hermansky-Pudlak Syndrome, Hermanski Pudlak Syndrome, Hermansky Pudlak Syndrome
KMLE À¥ ¿ë¾î ¸ÂÃã °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
Hermann Arminius: German hero; leader at the battle of Teutoburger Wald in AD 9 (circa 18 BC - AD 19)
Ãâó: wordnet.princeton.edu/perl/webwn
Hermann Joseph Hermann Joseph Muller (December 21, 1890 – April 5, 1967) was an American geneticist and educator. He was born in New York City and attended Columbia University, earning his B.A. in 1910 and his Ph.D. in 1916. A student of Thomas Hunt Morgan, he taught at Rice Institute in Texas from 1915 until 1918, at Columbia from 1918 until 1920, and at the University of Texas from 1920 until 1932, when he moved to Germany, and later to Moscow (in 1934? ...
Ãâó: en.wikipedia.org/wiki/Hermann_Joseph
Hermann (1988): Information and Self-Organization. Berlin: Springer. [amazon.com]
Ãâó: www.uni-graz.at/~holzinge/computer%20science/infor...
Hermansky-Pudlak s. an autosomal recessive form of tyrosinase-positive oculocutaneous albinism (ty-pos OCA) with a hemorrhagic diathesis secondary to a platelet defect, and accumulation of a ceroid-like substance in the reticuloendothelial system, oral mucosa, and urine.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Hermansky-Pudlak syndrome see under syndrome.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
WordNet ÀÏ¹Ý ¿µ¿µ »çÀü °Ë»ö °á°ú : 12 ÆäÀÌÁö: 1
Herman United States jazz musician and bandleader (1913-1987)
Herman United States inventor who invented a system for recording alphanumeric information on punched cards (1860-1929)
Herman American writer of novels and short stories (1819-1891)
Herman Canadian literary critic interested in the use of myth and symbolism (1912-1991)
Herman United States writer (born in 1915)
Herman German hero
Herman German Nazi politician who founded the Gestapo and mobilized Germany for war (1893-1946)
Herman German Nazi politician who founded the Gestapo and mobilized Germany for war (1893-1946)
Herman Swiss writer (born in Germany) whose novels and poems express his interests in Eastern spiritual values (1877-1962)
Herman United States geneticist who studied the effects of X-rays on genes (1890-1967)
Herman German physiologist and physicist (1821-1894)
Herman a French marshal who distinguished himself in the War of the Austrian Succession (1696-1750)
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