Kuyini Morgagni-Stewart-Morel syndrome?

Syndrome ka Morgagni-Stewart-Morel, isithombe ekhombisa lapho kungabonwa kulesi sihloko, akuvamile - esigidini abantu bagule zonke eyodwa kuphela abayizinkulungwane eziyikhulu namashumi amane. Ngaphezu kwalokho, ngo-umkhuba, ochwepheshe nobuso ikakhulukazi ne "izingcezwana" lesi sifo, ngoba isifo eba ziyasibona iminyaka eminingi, futhi izimpawu, aphenduka ngejubane elingafani. Iziguli kumele sibukwe hhayi omunye udokotela ngaphambi syndrome wabonakaliswa ngokugcwele futhi ukucacisa uphethwe ngqo.

Isimo esibucayi of sifo ifinyelela, uma yakha izinguquko sokugembula engelapheki emzimbeni. Ngemva kwalokho, ukusiza isiguli cishe ayinakwenzeka.

indaba

Syndrome ka Morgagni-Stewart-Morel iye kahle kuchazwe 1762, lapho sezifo Italian Morgagni wavula isidumbu namakhosikazi angamashumi amane atholakala ukuqina ingaphakathi engaphelele ithambo ipuleti. Ngesikhathi esifanayo, waphawula ukukhuluphala futhi ngokweqile umzimba izinwele. Morgagni wathola izimpawu esihlobene futhi zichazwe ngokuningiliziwe imibono yakhe. Kamuva, ngo-inhlanganisela zimpawu waphawula Morel futhi Stewart bakwazi ukuze ungeze isithombe basemtholampilo okunhlobonhlobo zezinzwa zengqondo.

Kuyini isifo

Morgagni-Stewart-Morel syndrome - ubulwelwe lapho engaphelele Kutholwe hyperostosis kwangaphakathi ithambo ipuleti, ukukhuluphala ngokweqile eziningi nezinye endocrine kanye umzimba iziyaluyalu. Ukugula iningi kwenzeka kwabesifazane, ikakhulukazi ngemva iminyaka engamashumi amane, kodwa ngezinye izikhathi manifest mathathu. Phakathi kwabesilisa syndrome cishe ingekho, ngaphandle ezingavamile.

Etiology futhi pathogenesis

Izifo Morgagni-Stewart-Morel - syndrome, pathogenesis futhi omsakazo zazo abe definition olulodwa. Abanye abalobi bakholelwa ukuthi zokugula kuyinto zofuzo futhi adluliselwa ngohlobo evelele autosomal. Kunemibono ukuthi lesi sifo sihlobene eduze X-chromosome. Kwakukhona izimo lapho isifo wahlupheka izizukulwane ezine, ikakhulukazi abesifazane.

Kukhona amacala elingalodwa syndrome ukubonakaliswa ezingabantu. Kodwa kukhona oqabukelayo, kanye isayensi ayikaziwa kanjani lesi sifo ayihambi ngokusebenzisa umugqa besilisa. Abanye babekholelwa ukuthi lesi sifo ngokuvamile kakhulu kubonakala abesifazane ngesikhathi nambili kuya kwabadala. Kodwa idatha zanqwabelana ngokuhamba kwesikhathi, wabeka lesi sitatimende ku umbuzo.

Lesi sifo kungavuka ngenxa izifo (ikakhulukazi sinusitis), neuroinfections futhi kuhlasimulise okulimala ebuchosheni. Watholakala Morgagni-Stewart-Morel syndrome castrates abesifazane abaye baba ukuhlinzwa. Izici ezithile syndrome zingase zimiswe emva ukwelashwa komdlavuza.

Kwakukhona izimo lapho isifo zitholiwe iziguli ezine:

• myoma lesizalo;
• amaqanda sklerokistoz;
• galactorrhea eziphikelelayo;
• infantilism zangasese.

Morgagni-Stewart-Morel syndrome: zokugula

Lapho lesi sifo mkhulu frontal kwangaphakathi ithambo ipuleti hhayi kuphela, kodwa ngezinye izikhathi Kutholwe hyperostosis ikhanda futhi Fronto-occipital esifundeni. Kukhona izinguquko eziningi ezincane e nezindlala endocrine. Ukwandisa inani eosinophil futhi basophil amaseli. Qala ekwandeni adenomatous (yegilo, cortex adrenal futhi t. D.).

izimpawu

Lapho umuntu kutholakala Morgagni-Stewart-Morel syndrome, kukhona eziningi izimpawu agcwele ke:

• Iziguli angaqala ukuzwa izinhlungu zekhanda elivamile.
• Ukukhuluphala, ikakhulukazi endaweni besisu.
• Skin azigcina umbala evamile, kodwa ngokuvamile babonakala pyoderma nokukhala eczema.
• Ezimweni eziningi kukhona hypertrichosis evelele.
• On the isikhumba imakwe pastoznost ziqala ukuba sikhukhumale nasemilenzeni ephansi, livela akrozianoz.
• Imisipha buthaka.
• Kukhona izinguquko esimiso senhliziyo nemithambo yegazi kanye izitho zokuphefumula (inhliziyo nokwandisa imingcele, kukhona amathoni ubuthulu futhi senhliziyo).
• Ukufisa igoqe, catarrh we elingenhla pheshana zokuphefumula kanye bronchopneumonia.
• Izinguquko in the nezindlala endocrine izici isici babe. Exchange kungenzeka evamile, anda noma kwehle.
• Kwehliswe ukubekezelelana carbohydrate. Ngokuvamile isifo sikashukela ngokucacile kubonakala khona.

• Siyabhubha umjikelezo wokuya esikhathini. Ngo sokukhulelwa, ngalesi sikhathi isimo lesiguli liwohloka.
• Kukhona iziyaluyalu sezinzwa nangokwengqondo. Bona azibonakali ngesimo ngokomzwelo lability, ukucasuka, ukuqwasha kanye nokuthambekela ukucindezeleka.
• Yangaphakathi frontal hyperostosis ipuleti ngokuvamile uqweqwe skull amathambo. Kwezinye izimo, ngisho ukwandisa ubukhulu sehhashi Turkish.

lesi sifo

Morgagni-Stewart-Morel syndrome isinyathelo umzimba ngezindlela ezahlukene. Ngokuvamile incike degree of ukukhuluphala. Evelele has nomthelela omkhulu bempilo:

• okwenzeka enhliziyweni kunzima;
• ngenxa ekuqongeleleni fat zenzeka myocardium futhi pericardium;
• isikhundla esiphakeme diaphragm;
• kwegazi, futhi lihambisana isifo sokuqina kwemithambo yegazi nomfutho wegazi ophezulu ekuthuthukiseni senhliziyo, okuyimbangela nokukhubazeka;
• uma isiguli sifo babe ngaphezulu nesikashukela, bese-ke uqala ukuphazamiseka umzimba, okuyinto akugcini ngokuletha umuzwa ukwehla kahle, kodwa futhi linciphisa ukuphila okulindelekile.

ukwelashwa

Ingabe Kungenzeka yini ukususa lesi sifo ka Morgagni-Stewart-Morel? Ukwelashwa nokuvikelwa lesi sifo ikakhulu ukudla okufanayo sikashukela nelinganikezelwa kanye nokukhuluphala ngokweqile. Ukudla kufanele kube-kilojoule amaningi, kakhulu wamaprotheni amaningi, amavithamini namaminerali nosawoti. Ukudla kunciphisa inani carbohydrate kanye amafutha. Ukudla Baranov inikezwa ikakhulu.

Ngaphezu kwalokho, ukuphathwa Kwenziwa massage and eyabelwe ngokomzimba ukuqeqeshwa umzimba ukunciphisa ukuba buthakathaka kwezicubu nokujikeleza kwegazi neuropathy. Uma isiguli senhliziyo, ukwelapha lesi sifo labetibambile isisu kardiopreparatami futhi t. D.