Mauriac syndrome in seswekile

Mauriac syndrome - isifo ukuvela nje kwingxaki ngenxa unyango ngokungafanelekanga seswekile eselula. Ngokuba lixesha lokuqala sifo kuchazwa 1930 ugqirha French Pierre Mauriac imvelaphi. Wachaza uhlobo umfanekiso lwezonyango ngayo abantwana abanengxaki seswekile kunye nonyango insulin ezidlulileyo idosi ephosakeleyo, ukusebenzisa ezinye iimpawu zangaphandle. Waqaphela ukuba bonke abantwana ayafana ngembonakalo, ethe yabonakalisa ukukhula, ukutyeba, uphuhliso ngokwesondo luzalwe ephantsi.

Ezibangela isifo

Unobangela omkhulu iingxaki ezinzima ayilunganga wamlandela unyango seswekile. Kulo isifo injeke nayo ibingasebenzi kakuhle, ongavelisi ngemithamo eyaneleyo insulin. Ngenxa ngezifo kwenzeka intswelo glucose iiseli ngenxa yokuba oko igxininisa ngesixa-mali esikhulu egazini.

Mauriac Uphuhliso syndrome kwizigulane kunye seswekile sinwenwa udidi 1 inxulunyaniswa unyango asanelanga. umntwana bagula ixesha elide insulin laneleyo zedosi elawulwa, okanye iimveliso ezikumgangatho nokubi nyulu ephantsi, esa kukho intswelo ezinganyangekiyo kwencindi.

ukungabikho elide insulin emzimbeni kukhokelela le nkqubo ilandelayo:

• ukuphazamiseka pathways, ingakumbi ukuphazamiseka ekudleni ukutya.
• Ukwandisa ubukhulu wesibindi ukonakala lwaso ngenxa yokwanda glycogen iyafa.
• Utshintsho kumafa egazini - ukwanda iswekile egazini, cholesterol, ii-asidi okunamafutha.

Ukuba oku akukho zonke iinkqubo zophendlo lwezifo emzimbeni ngexesha Mauriac syndrome, nayo izigulane ezingabantwana amava izinxaxhi zilandelayo:

• Imveliso Ukunqongophala hormone ezibalulekileyo - cortisol, hormone ukukhula, glucagon, kwaye ngenxa yoko le yaphula inkqubo yokukhula.
• Ucazululo iiprothini kunye calcium output etyebileyo iphosphate emathanjeni, ekugqibeleni kukhokelele kuphuhliso kwamathambo kunye ezithile yoma kwezihlunu.
• Ukungakwazi ukufunxeka iivithamini ethunjini.

Esi sifo sidla esizibonakalisa phakathi kweminyaka eli-15-18, kodwa inkqubo kukuba iveza iqala kakhulu ngaphambili. Okwangoku, ngokuba ukunyanga isifo seswekile kwangoko isebenzisa amalungiselelo mihla hlamba kakuhle, nto leyo phantse ishenxisa uphuhliso Mauriac syndrome.

symptomatology kwesifo

Mauriac Syndrome seswekile kukho inani kubachaphazela:

• Umntwana ningatyhafi kuphuhliso kunye nokukhula inhibition. Amaxesha amaninzi kukho ukutyeba mafutshane ngesithomo. Kulo mzekelo, umntwana nabagulayo barhuqe emva oontanga babo sokwanda ngokweesentimitha 10-30.
• Le lag kuphuhliso ngokwesondo (iimpawu ngokwesondo ngokwaneleyo kunye nokungabikho sexesheni emantombazaneni).
• kuthomba elide.
• Ukutyeba, ingakumbi ubuso nomzimba ongasentla kunye nemilenze ezibhityileyo. abantwana abagulayo ziyafana kakhulu, abe "Crescent-ezimile" ebusweni omnye, intamo emfutshane, amanqatha iingalo, amagxa, esiswini. Inxalenye esezantsi umzimba ibhityile kakhulu.
• isibindi Ukwanda wahamba ejikeleza circuitous lwemithambo.
• Osteoporosis (behlise phantsi uphuhliso kwezihlunu amathambo).
• izifo zamehlo, kuqukwa izifo retina, yaye kamva uphuhliso kwamehlo.

Eyona syndrome ixhaphakileyo Mauriac ebantwaneni, efotweni umntwana abagulayo kubonisa ntoni iimpawu sele unaso esi sifo.

lokufunyaniswa kwesifo

Lokufunyaniswa "Mauriac syndrome" icwangcisiwe yimiqondiso ezicacileyo lwangaphandle zesi sifo, ezifana: ukunqongophala ukuphakama kukwaluphala, ubukho ukutyeba, ingakumbi xa sijamelene Ukungakhuli ngokwesondo, ukunyuka ecacileyo esibindini.

Ukuze uqinisekise uxilongo baqhuba iimvavanyo zegazi, nto leyo ekhokelele wabonakala zilandelayo:

• amanqanaba glucose ezingazinzanga, ukubonisa nokuxhuma rhoqo kwi enkulu, ke phantsi.
• lipids igazi egqithisileyo (hyperlipidemia).
• Ukwanda kakhulu kumanqanaba yegazi (hypercholesterolemia).

Kwezinye iimeko, ukuqhubela phambili isibindi biopsy ukulungiselela le njongo, ukuba awakhuphe umthwalo amafutha

isifo

Ngokuba umzimba lowo sifo kuba nzima kakhulu. Njengoko kakhulu lwe seswekile, kube nzima ukuba sivale. Amaxesha amaninzi kubangela asidi isiqaqa hyperglycemic.

Mauriac syndrome kwenzeka kakhulu ngenxa Ukongeza rhoqo zizifo ezahlukahlukeneyo ezasulelayo, kwakunye neenkqubo kwemichiza entsonkothileyo nende ukuchacha emzimbeni.

Mauriac syndrome and Nobekura: ezifanayo nezahlukileyo

Mauriac syndrome kunye Nobekura ngokufuthi iqalisa ebuntwaneni, zombini izifo kwingxaki kakhulu seswekile owenzeke imvelaphi unyango engafanelekanga. Zombini syndromes uneempawu ezifana, ziquka abazizidalwa ngokukhula kunye nophuhliso ngokwesondo, a elide lokufikisa, okunamafutha isibindi. Kulo mzekelo, umehluko oyinhloko Nobekura syndrome kukunqongophala kwamafutha omzimba kwemfuneko. Kulo mzekelo, unyango zombini syndromes ijolise kukwenzakala ekunyangeni isifo seswekile.

unyango syndrome Mauriac

unyango ngqo syndrome ngathi oku asikho. Zonke therapy ezijolise ekujonganeni oyena nobangela wokosuleleka zizifo kwakunye nezigulo esibangelwa ngabo. Ukuze benze oku, isigulane eyabelwe nothomalaliso echanekileyo insulin kwi idosi echanekileyo kunye nomgangatho kunye neziyobisi mihla.

Ukuthintela lipodystrophy yabamba iqela yeenkqubo zothintelo ukulungiselela le njongo oqeshwe yokuthanjiswa, physiotherapy neetshathi ye-insulin. uhlobo olukhethekile linokukwenziwa ukongeza kunyango, abafakwanga bona kule ekutyeni zonke zokutya equlathe amafutha ezilwanyana. Kulo mzekelo, adla igxininisa protein ekudleni zokutya.

unyango Medical ikwayilelwe ukubuyekeza ukuphuhlisa kwi imvelaphi iingxaki isifo esibangela oku naznachetsya yesigulane:

• Admission gepatoprotektorov ukubuyisela iiseli zesibindi.
• iivithamini B ukubuyisela ukutya yesiqhelo.
• iziyobisi Complex ejolise normalizing kumanqanaba lipids kunye cholesterol egazini.
• amachiza steroid zivuse ukukhula yomzimba.
• amachiza hormone ukubuyisela imisebenzi ebalulekileyo ngokwesini.

Prevention and abane

Ukunqanda le ingxaki yingozi seswekile njengoko Mauriac syndrome, kuyimfuneko ukwenza unyango oluchanekileyo sifo bha nokuthintela insulin ukuswela.

Xa unyango zidibene sele livelise syndrome iziyobisi mihla abantu abane kakuhle ngokwaneleyo, kodwa ukuba zonke iingcebiso kagqirha. Idosi echanekileyo insulin kwaye ubuyisele usebenziso onke amalungu abachaphazelekayo bayakwazi ukubuyela impilo yomntwana, ngaphandle kunye nezo ziphuma ngaphakathi.