Mauriac syndrome sikashukela

Mauriac syndrome - isifo esakheka njengoba inkinga ngenxa yokuphathwa kabi okungafanele sikashukela esemncane. Ngokokuqala ngqá sifo achazwa 1930 by udokotela French Pierre Mauriac imvelaphi. Wachaza uhlobo isithombe emtholampilo lapho izingane abaphethwe isifo sikashukela kanye therapy insulini esidlule umthamo okungalungile, bonisani ezinye izimpawu zangaphandle. Waqaphela ukuthi zonke izingane zifana ngokubukeka, okuyinto kubonakala ongaphakeme ukukhula, ukukhuluphala, ebhasha ukuthuthukiswa ngokobulili.

Izimbangela zesifo

Imbangela enkulu yezinkinga ezinzima ayilungile wajaha yokwelapha isifo sikashukela. Kulesi isifo kwamanyikwe is ayisebenzi kahle, hhayi esikhiqiza eziningi ezanele we-insulin. Ngenxa zokugula kwenzeka ukuntula glucose kumaseli ngenxa yokuthi ukuthi ugxilisa e esiningi egazini.

Mauriac ukuthuthukiswa sifo ngo iziguli sikashukela mellitus uhlobo 1 lihlotshaniswa ukwelashwa ingafaneleki. ingane egulayo isikhathi eside insulini enganele umthamo esilawulwa, noma usebenzise imikhiqizo ye ukhwaliti ongaphakeme nokubi ukuhlanzwa, okuholela nokuntuleka ezingamahlalakhona we-insulin.

yokuntuleka isikhathi eside insulin emzimbeni kuholela izinqubo ezilandelayo:

• iziyaluyalu umzimba, ikakhulu ukuphazamiseka carbohydrate umzimba.
• Ukwandisa ubukhulu kwesibindi kanye okunamafutha yayo ukonakala ngenxa lenyuka glycogen ukuphuka.
• Izinguquko egazini Ukwakheka - ukwanda igazi glucose, cholesterol, ama-fatty acid.

Uma lokhu kungenjalo zonke izinqubo sokugembula emzimbeni ngesikhathi Mauriac syndrome, futhi iziguli ezingane ukuzwa lokuchezuka elandelayo:

• ukukhiqizwa ezinganele hormone ezibalulekile - cortisol, ozokusebenzisa, glucagon, kanti ngenxa yalokho kwephulwe inqubo yokukhula.
• Izilinganiso amaprotheni ocebile okukhipha calcium ne phosphorus kusuka amathambo, ekugcineni okuholela ukukhumuzeka kwamathambo kanti ezinye yoma kwemisipha.
• Ukungakwazi ukumuncwa amavithamini emathunjini.

Lesi sifo ngokuvamile kubonakala abaneminyaka ephakathi kwengu-15-18, kodwa inqubo enezinkinga iqala ngisemncane kakhulu. Okwamanje, ukuze kwelashwe isifo sakhe sikashukela ekuseni isebenzisa amalungiselelo yesimanje waliphothula ngokuphelele, okuyinto cishe aqede intuthuko Mauriac syndrome.

symptomatology lesi sifo

Mauriac Syndrome sikashukela enezinto eziningi lezinyathelo:

• Ingane emuva ekuthuthukisweni nokukhula okusithiyayo. Ngokuvamile kukhona ukukhuluphala futhi onesiqu esifushane. Kulokhu, ingane egulayo Zingase zisale ngemuva ontanga yabo nokukhula ayi-10-30 ngamasentimitha.
• I-lag ekuthuthukisweni ngokocansi (izici ngokobulili ampofu futhi ukungabi zokwehlukaniswa emantombazaneni).
• ukuthomba eside.
• Ukukhuluphala, ikakhulukazi ebusweni nasemzimbeni engenhla nge nemilenze mncane. Izingane ezigulayo zifana kakhulu, abe "inhlendla emise" ubuso komunye nomunye, intamo esifushane, amafutha ezingalweni, emahlombe, esiswini. Ingxenye engezansi yomzimba mncane kakhulu.
• isibindi Ukwandiswa ephelezelwa circuitous kwegazi emithambo yegazi.
• Lokhu kwakubangelwa ukukhumuzeka kwamathambo (ingasheshi ukuthuthukiswa ithambo izicubu).
• izifo iso, kuhlanganise nezifo retina, futhi kamuva ukuthuthukiswa esweni.

Okuvame kakhulu Mauriac sifo ngo-izingane, abagulayo ingane isithombe sikhombisa lokho izimpawu banaso lesi sifo.

sitholakele isifo

Sitholakele "Mauriac syndrome" kusethelwe ngesikhathi sobala izimpawu zangaphandle lesi sifo, njenge: yokuntuleka ukuphakama nobudala, khona ukukhuluphala, ikakhulukazi lapho sibhekene yokungavuthwa ngokobulili, ukukhuphuka ecacile esibindini.

Ukuze uqinisekise uphethwe kwenziwe ukuhlolwa kwegazi, okwaphumela ezilandelayo ezibonisa yilezi:

• namazinga kashukela egazini engazinzile, ukukhombisa kuzuba njalo kule big, bese phansi.
• Ngokweqile igazi lipids (hyperlipidemia).
• A nokwanda okuphawulekayo amazinga egazi cholesterol (hypercholesterolemia).

Kwezinye izimo, eminye ukufunda isibindi biopsy le njongo, ukuletha umthwalo amafutha aphume

lesi sifo

Ukuze umzimba abasha lesi sifo kunzima kakhulu. Njengoba ifomu okunzima sikashukela, kunzima ukuze sivale lapho sisilela. Ngokuvamile kubangela lactic futhi equlekile hyperglycemic.

Mauriac syndrome kwenzeka ezinzima ngenxa kwalokho njalo izifo ezihlukahlukene ezithathelwanayo, kanye yokutakula izinqubo eziyinkimbinkimbi futhi ezinde kwamakhemikhali emzimbeni.

Mauriac syndrome ne Nobekura: lokufanako nalokwehlukile

Mauriac syndrome ne Nobekura ngokuvamile eba ebuntwaneni, zombili izifo yilezi isimo esibucayi sikashukela ukuthi kwenzeka on isizinda ukwelashwa okungafanele. Bobabili syndromes unezimpawu ezifanayo, bahlanganisa retardation ukukhula nokuthuthuka ngokocansi, a eside usakhula, okunamafutha isibindi. Kulokhu, lo main umehluko Nobekura syndrome bikho yamafutha amaningi emzimbeni. Kulokhu, ukwelapha kokubili syndromes ihlose ukulimala ekwelapheni isifo sikashukela.

Mauriac ukwelashwa syndrome

ukuphathwa eqondile syndrome kanje akekho. Zonke yokwelapha okuhloswe ngalo ukubhekana nomnyombo isifo kanye nezinkinga kubangelwa kubo. Ukuze wenze lokhu, isiguli inikezwa esilungile yokwelapha insulin umthamo ilungile futhi izinga nge ngemithi yanamuhla.

Ukuvimbela lipodystrophy labetibambile eziningi izinqubo zokuvimbela le njongo aqokwa njengelungu massage, Physiotherapy namashadi ukuze insulin. olukhethekile lokudla angabelwa ngaphezu ukwelashwa, bayakhishwa kusukela ekudleni zonke okune ezilwane. Kulokhu, ekudleni igxile protein ne-carbohydrate ukudla.

yokwelapha Medical Futhi wenzelwe ukunikeza izingane nxephezela ukuthuthukisa ku isizinda eyisisekelo isifo yezinkinga salokhu naznachetsya nesineke:

• Ukwamukelwa gepatoprotektorov ukubuyisela amaseli isibindi.
• amavithamini B ukubuyisela umzimba evamile.
• izidakamizwa Inxanxathela okuhloswe ngaso normalizing amazinga lipids futhi cholesterol egazini.
• izidakamizwa steroid kushiya ekukhuleni komzimba.
• izidakamizwa kwama-hormone ukubuyisela nobulili ezibalulekile.

Izinhlelo ezivimbayo futhi Izibikezelo

Ukuvimbela lokhu inkinga eyingozi sikashukela njengoba Mauriac syndrome, kubalulekile ukuba afeze yokwelapha efanele isifo ecashile futhi kuvimbele insulini ukuntula.

Lapho ukwelashwa kuhlangene isivele bahlakulele syndrome ngemithi yanamuhla Izibikezelo evumayo ngokwanele, kodwa uma bonke izincomo udokotela. Umthamo efanele insulin futhi ubuyisele ukusebenza zonke izinhlaka abathintekayo bayakwazi ukubuya impilo yengane, zangaphakathi nezangaphandle.