Dysplasia ectodermal ne fibrous. Izinso Dysplasia

Ngaphansi kwe-dysplasia (okuhunyushwe kusuka esiGrekini, njengokungaboni kahle, i-wplasis - imfundo) kusho ukuthuthukiswa okungalungile nokukhubazeka kwesigungu. Lo mqondo ohlangene, ohlanganisa noma yiziphi izinkinga zokuphulwa.

I-dysplasia ebonakalayo ibhekisela ezifweni zesimiso se-osseous futhi ibonakala ukuthi izicubu zethambo zithathelwa yizicubu ezinamafutha. Le nqubo iguqula amathambo. Isizathu esiyinhloko salesi sifo ukubonakala kwenqubo efana ne-tumor, eqala emntwaneni, bese iqala ukuqhubeka. I-dysplasia enempilo ivame ukuvela kwabesifazane.

Kunezigaba eziningana zesifo. Okuvame kakhulu yiyunithi, kuye ngokuthi izinga lezinkinga zenzalo:

• I-mono-osseous fibyous dysplasia ithinta ithambo elilodwa kuphela;
• I-polyosseous - izinguquko ziyabonakala emathanjeni amaningana (ngokuvamile asakazwa ohlangothini olulodwa lomzimba futhi ngokuvamile ayenzeka ebuntwaneni).

Ifomu lokuqala lesi sifo lithinta amathambo ama-cranial, ama-scapula, amathambo we-tubular (eside), izimbambo. Okwesibili okwamanje kunamaphesenti angu-50 amathambo omzimba wonke.

Ezingxenyeni eziyinhloko zingase zibheke ukuhlukana okuvamile, okukhula ngokushesha ndawonye, kodwa ukukhuphuka kwamathambo, kanye ne-sarcoma.

I-dysplasia ehlakaniphile ayinikezi ukwelashwa, izinqubo zokwelapha kuphela (amathambo okuxhunyelelwa, ukuhlenga, i-osteotomy) kungenzeka, kanye nokuqokwa kwezidakamizwa ezidambisa ubuhlungu.

I-Ectodermal dysplasia ihlanganisa inani elikhulu le-syndromes nezifo (cishe ngu-100), okuyisisekelo esibhekene nokuthuthukiswa okungavamile kwezinto ezivela ectoderm. Isibonakaliso sokuqala kuba khona kwe-hyperthermia, okuyinto, uma ikhonjiswa futhi ihlaselwa njalo, ingaholela ekuphazamiseni ukuthuthukiswa kwengqondo noma umphumela obulalayo.

Izinhlobo ezilandelayo nezimpawu zazo zingahlukaniswa.

I-anhydratic iqala ukubonakaliswa kumntwana wengane futhi ihlala isikhathi eside. Izici eziyinhloko yi:

• Ubungcweti besifo;
• Ukuguquguquka okukhulu kwamazinyo;
• Ukuba khona kwezinga eliphansi lokuthuthukiswa kwezinsipho;
• I-Atrophy ye-ambulance yamangqamuzana (umphimbo, umlomo, impumulo);
• Ukuvela kwengane kufana nesiguli esine-syphilis esandulele.

Ukwelashwa kuqokwa kalula. Okuvinjelwe: ukuhlala emazweni aseningizimu, sebenzisa ezitolo ezishisayo nezomakhemikhali.

I-hydraulic exo-dermal dysplasia ibonakala ngokuba khona kwezinguquko ezintendeni zezandla, izinhlanzi kanye nokuthuthukiswa kwezinwele. Njengokwelashwa, i-vitamin A ivame ukubekwa ngaphandle nangaphakathi, ithambisa ukhilimu kanye ne-keratolytic. Ngaphezu kwalokho, ama-enzymatic agent, i-methyluracil, i-anabolic steroids, i-zinc, i-amino acid ne-biostimulants isetshenziswa.

I-dysplasia yezinso inezinhlobo eziningana ezihlukile:

• Ngezibonakaliso zokuziphatha (i-cystic futhi elula);
• Indawo yangakini (i-cortico-medullary, i-medullary ne-cortical);
• Ngokusakazeka - isamba, segmental, focal).

Izimbangela eziyinhloko zalesi sifo ziyizici zofuzo kanye nemiphumela yangaphandle neyangaphakathi yangaphakathi embryo. Ngakho-ke, lesi sifo sisekelwe ebubini bokubeletha, okuyinto, kungakhathaliseki ukuthi yiziphi izilwane, kuholela ekunciphiseni kwezinso ngesayizi, ukuthuthukiswa okunonya kwe-parenchyma, ukwehla okuphawulekayo emisebenzini yayo.

Ukwelashwa kwalesi sifo kuyingozi futhi kuvame ukuba khona lapho kunesibonakaliso sokufakelwa kabusha kwalesi sitho.

Ngokuvamile, i-dysplasia ihlanganisa iqembu lezifo ezisho ukuthuthukiswa okungalungile kwegciwane elithile noma uhlelo lonke. Njengomthetho, izimbangela eziyinhloko ziyizici zofuzo nezifo.

Ukwelashwa kuyingozi noma kunezincomo zokufakelwa kwesitho noma ukuthutha amathambo.