Pick ta cuta

Pick ta cutar da aka gano cutar a cikin makon farko na rayuwa. A cutar halin ban mamaki nauyi asara, da kuma duk m ciki kumburi. Wadannan alamu ne bisa kara girma na hanta da saifa. Niemann-sama cuta sa kumbura Lymph nodes, da kuma yellowing na fata, a wasu lokuta, a can ne leukocytosis da hypercholesterolemia. haƙuri mutuwa faruwa a sakamakon da girma ci da zawo. A autopsy saukar m hanta, saifa, kara girman Lymph nodes a size. Pick ta cuta sa adrenal launi da kuma duk kayan ciki a wani haske rawaya launi. Wannan shi ne saboda da shaida na phosphatides a cikin cell kyallen takarda. Lipids iya yi jinkiri a cardiac zaruruwa, jijiya Kwayoyin, kwarangwal tsoka, epithelial da endocrine gabobin.

Pick ta cuta ne a karkashi da cuta da lipid metabolism phosphatide. A cuta ne nakasar da iyalinmu boys
da 'yan mata wahala daidai sau da yawa. Niemann-Pick cutar mafi akai-akai lura
tsakanin Ashkenazi Yahudawa, a wasu lokuta, cutar na faruwa a
1: 100 000 live haihuwar.

Cutar da ake tare da tsanani raunuka na juyayi tsarin, janar na cin gaba da bata lokaci ba. Pick ta cutar na farko da aka bayyana a cikin 1922, don kwanan game da 100 da aka bayyana lokuta. dalilai
cuta ne ba a sani ba, amma daya daga cikin korau dalilai ne da na halitta
dangantaka da yaro ta iyaye.

The asibiti bayyanuwar da cutar samu a jariri, mafi sau da yawa a cikin farkon rabin jariri rayuwa. Akwai, duk da haka, ya kuma watsar kwatancin na asibiti ãyõyin cuta a cikin shimfiɗar jariri, da kuma ko da matasa shekaru. A hankali, da mãsu haƙuri ɓullo da ascites, muhimmanci ƙara Lymph nodes. Skin duba m da waxy, akwai kuma gashin yankunan.

A matakin farko da cutar bayyana motsi cuta - pyramidal ãyõyi, ta ƙara agara reflexes da tsoka sautin,
reshe paresis. A baya matakai na sama ta cutar halin
tsoka hypotonia kuma babu agara reflexes. The yaro da sauri
ci gaba nauyi, da makanta, da idiocy. A cikin fundus ne sau da yawa samu
atrophy na da na gani jijiya da tabo a cikin macular yankin. Lokacin da gwaji kan gawar nazarin sau da yawa shigar leukopenia da hypochromic anemia.

Forecast for m rayuwa - wata cuta halin da m hanya. Mafi yawan marasa lafiya mutu a cikin shekaru biyu na farkon rayuwar. A dalilin mutuwar ne cardio-na huhu insufficiency da kuma intercurrent kamuwa da cuta.

Tasiri jiyya ga cutar Niemann-sama don a yau ba ya wanzu. Ga magani amfani hormones, enzymes, hanta ya'ya, jini. Duk da haka, duk wadannan hanyoyi na m
sakamakon ba ba, kazalika da splenectomy. Domin ya hana cutar,
iyaye na wani yaro da tara ta cuta, shi ne shawarar da su guji
m haifuwa.

irin gado da cutar ya ba tukuna aka kafa, a matsayin marasa lafiya mutu tun suna jarirai. Cutar da ake daukar kwayar cutar mai yiwuwa a wani autosomal recessive hanya. Wannan take kaiwa zuwa da jari a cikin tsokoki da kuma gabobin da sphingomyelin, kuma da yaron ya fata ya zama brownish-yellow tint. A daban-daban embodiments, da ya kwarara cututtuka na juyayi tsarin shafa a sãɓãwar launukansa digiri. Bayyana a rage na platelets, anemia, kashi nama softening. Yara da wannan cuta da wani gagarumin raunuka na juyayi tsarin, da mafi m hangen nesa ga rayuwa.