Salon, yara da ciwo Shvahmana Diamond

Idan jariri a watan farko na rayuwa tsiwirwirinsu kasa da 100 grams, shi ya kasance ma m, sako-sako da, sosai m stools, da kuma kusan babu ci, za ka iya xaukar gaban irin wannan cututtuka kamar Shvahmana-Diamond ciwo. A yara, da rashin lafiya sanadin wadannan cututtuka.

Case Tarihi: Sanadin

A cikin bazara na shekarar 1964, masana kimiyya m Bodian da Sheldon karo na farko ya bayyana bayyanar cututtuka da cutar, da ake kira shi hypoplasia na pancreas. Bayan wani lokaci, wani bincike jummai yanke shawarar duba more a hankali a cikin al'amarin, don haka abin da ya bayyana da cewa akwai wani dan kadan daban-daban irin cuta. Saboda haka akwai Shvahmana-Diamond ciwo.

Wannan cuta rinjayar jariran ne musamman rare. Statistics nuna cewa marasa lafiya da daya da dubu hamsin yara. Duk da haka, cutar kuma akwai kwayoyin, cewa an canja shi zuwa yaro daga iyaye biyu a wani autosomal recessive hanya. Jigon da aiwatar ta'allaka ne a wani qaddara kewayon maye gurbi 7th chromosome gene rage tsawon SBDS. Wannan gene ƙunshi takamaiman gina jiki wanda aka located in minti yawa a cikin kowane mutum zuciya da sauransu, amma ta fi girma yawan bayanan kula a cikin pancreas. A sakamakon haka, a lokacin da wani gene maye gurbi auku endocrine tabarbarewa daga cikin sashin jiki zai lalace kuma samar da lipase, bi da bi, shi ne ba rashi na wannan enzyme. Ko da yake cutar da ake ba cikakken gane, kuma daidai aiki na mutating a gene ne ba a sani ba, shi gano cewa, ban da malfunctions na pancreas, shi destructively rinjayar da bargo.

The girma na fama da wannan cuta ya karbi m gene daga iyayensu. Amma akwai kuma harben lokuta a cikin abin da bayyanar da cutar a lokacin da amfrayo ci gaban da aka rinjayi da mumps cutar, ko, misali, Coxsackie.

Syndrome Shvahmana Diamond: Alamun

Yana da wani m cuta halin da jinkiri ilimi da kuma na hankali da jiki da ci gaba. Alamun iya bayyana a matsayin ranar haihuwa, kuma a watanni shida na shekaru, da farkon gabatarwar karin abinci.

• Na farko kuma mafi daukan hankali alama da cutar - mai kowa da takamaiman zawo. A kujera yana da wani musamman mai abun ciki, lãka launi da kuma fetid wari. Masana sun kira shi steatorrhea.
• Kan bango na dindindin cuta da yaro zai fara zuwa hanzari rasa nauyi da kuma ci, wanda sau da yawa take kaiwa zuwa ciwon tamowa.
• Yara da ciwo Shvahmana Diamond yiwuwa ga m rashin lafiya, saboda su rigakafi da tsarin da aka muhimmanci raunana.
• bargo tabarbarewa take kaiwa zuwa tsanani canjawa a cikin jini dabara - sau da yawa lura anemia mai sãɓãwar launukansa a mai tsanani, neutropenia (rage m aiki), thrombocytopenia (zub da jini cuta da kuma yadda wani sakamako, to hali profuse jini asara). Sau da yawa akwai pancytopenia - kaifi raguwa a duk jini Kwayoyin.
• Hormonal disturbances kai ga short jiki da kuma jinkirta mafarki (a cikin baya hali).
• Ya sha wuya m da neuro-qwaqwalwa ne qwarai Sphere.
• Har ila yau a cikin marasa lafiya Shvahmana Diamond ciwo alama kwarangwal anomalies, tashin hankali na ma'adinai bangaren, m samu karaya m.

A wadda nau'i na cutar ya auku, ya dogara da cutar da kuma ta hangen nesa. Alal misali, akwai ba duk na sama cuta da kuma cututtuka na ciwo daga m, yayin da wani tsanani form halin da m da kuma musamman unfavorable hanya.

Hakika, cutar rinjayar da nan gaba hanyar rayuwa na biyu marasa lafiya da taimaka. The yaro yana bukatar m kula, kula da kuma hankali. Ma da ake bukata m asibiti a daban-daban kananan dakunan shan magani, sayan tsada kwayoyi don alkintawa da wanzuwar rayuwa da kuma kiwon lafiya na yara. Bugu da kari, marasa lafiya da matasa ne a dire bukatar a rage cin abinci, da kuma lifelong.

ganewar asali da cutar

Don gane asali cutar, shi wajibi ne don bambance shi daga cystic fibrosis, wanda da irin wannan bayyanar cututtuka da cutar. Cystic fibrosis ne mai tsanani kwayoyin da pancreatic Pathology. Don gane ainihin ganewar asali wajabta ji elastase maida hankali (da enzyme) a cikin tumbi da gumi samfurin. Idan taro na sodium chloride a cikin gumi ya karu da elastase a kima an rage, sa'an nan da samfurin ne tabbatacce ga cystic fibrosis. In ba haka ba, likita yana zuwa magance cutar, da ake kira Shvahmana-Diamond ciwo.

Ganewar asali da wannan cuta ne daban-daban matsananci rikitaccen, saboda yawanci bayyanar cututtuka su ne ma pronounced. Saboda haka, wani m pediatrician yi zargin gaban da cutar. A sa na ayyuka da nufin gano da ciwo ya hada da wadannan matakai:

1. Likita ya kamata yadda ya kamata tattara anamnesis, ya zare tambayoyi da iyaye a lokacin da yaro yana da halayyar bayyanar cututtuka na wani mushy stool, rashin ci, nauyi karancin da sauran fasali. Ba zato ba tsammani, zawo a yara tare da ciwo Shvahmana Diamond, za a iya isa 14 sau a rana.
2. Doctor dole ne bayyana tarihi, Magana game da iyali na cututtuka, kazalika da ya fara nuna yadda sau da yawa yaro ba shi da lafiya cututtuka.
3. Wannan ne bi da kai tsaye jarrabawa na haƙuri: aunawa da yawan zafin jiki da kuma matsa lamba, da elucidation na bugun jini mita, sauraron stethoscope da zuciya da kuma huhu, palpation na ciki gabobin.
4. Gudanar da cikakken asibiti bincike na jini da kuma stool. Yawancin lokaci, karshen nuna wani pronounced steatorrhea. jini count muhimmanci sosai, amma sugar ne yawanci al'ada.
5. Nada wani binciken da fitsari. A urinalysis dagagge gina jiki, ja jini Kwayoyin da farin jini Kwayoyin.
6. Bayan wadannan karatu compulsorily sanya CT, MRI na ciki da kuma duban dan tayi. Idan ya ce ciwo ya auku, da pancreatic Pathology cikakken tabbatar.
7. Matsayin mai mulkin, a pediatrician da shugabantar da haƙuri tuntubar wani tsarin halittar jini, da kuma gastroenterologist. Zaka iya bukatar X-ray nazarin kashi nama da kuma cikar wata nephrologist.
8. Amma mafi tsada da kuma abin dogara ne DNA analysis.

warkewa matakan

Jiyya na marasa lafiya da ciwo Shvahmana Diamond ne da farko directed a rike da harshen damo.
Don yin wannan, yi amfani da maye far - kammala m enzymes da kuma ji ba gani. pancreatin kwayoyi a high dosages da ake dauka domin rayuwa. Shi ne kuma dole don mayar da jini dabara. A wannan yanayin, mafi sauki su bi da anemia amenable. Idan akwai wadanda suke da alaka cututtuka (saboda pronounced immunodeficiency yaro ne sau da yawa rashin lafiya), sa'an nan haɗi da kwayoyin. A pathologies halin mai tsanani, amfani da radiation da jiyyar cutar sankara da kuma bargo dasawa.

rage cin abinci

Hanyar rayuwa na wadannan marasa lafiya, ban da kiwon lafiya da hanyoyin, har abada hade da wani takamaiman rage cin abinci. Wannan ya zama high-kalori menu dangane da sunadarai da kuma fats nan take. Rage cin abinci a cikin wannan harka dole dauki kashi 150% by caloric rage cin abinci al'ada yara a wannan shekara. Wannan ne da muhimmanci musamman ga marasa lafiya da goma bisa dari underweight. Bayan kayayyakin, musamman ikon amfani: warkewa cakuda dangane Bovine sunadaran (idan ba alerji, sa'an nan hada). Ake bukata don gudanar da wani bitamin, saboda m zawo m abubuwa ne hanzari wanke daga cikin jiki.

kintace

Amma ga tsinkaya, shi duka dogara a kan adequacy na lura da hanyar rayuwa na haƙuri, kazalika da tsanani da cuta. A tsanani lokuta, yara suna wuya rayu har zuwa shekaru 7, kuma ba ma mai tsanani - har zuwa ashirin ko fiye.