Mauriac syndrome na-arịa ọrịa shuga

Mauriac syndrome - a ọrịa na-amalite dị ka a sikwuoro ruru na-ezighị ezi omume nke-arịa ọrịa shuga na-eto eto. N'ihi na oge mbụ ọrịa a kọwara na 1930 site na French dibịa Pierre Mauriac malite. Ọ kọwara ụdị adakarị picture na nke ụmụ-ata ahụhụ site ọrịa shuga na insulin ọgwụ gara aga na-ezighị ezi usoro onunu ogwu, na-egosipụta ụfọdụ mpụga ịrịba ama. Ọ chọpụtara na ụmụaka ahụ niile ndị yiri anya, nke na-egosi na ala ibu, ibu oké ibu, nkwarụ mmekọahụ development.

Na-akpata ọrịa

The isi kpatara oké nsogbu bụ na-ekwesịghị ịdị na-achụ ọrịa shuga ọgwụ. Na ọrịa a na pancreas na-adịghị arụ ọrụ nke ọma, na-adịghị amị zuru ezu quantities nke insulin. N'ihi na daa ọrịa emee glucose erughi na mkpụrụ ndụ n'ihi na eziokwu na ọ na ụtụtụ na nnukwu ichekwa na ọbara.

Mauriac syndrome mmepe na ọrịa na-arịa ọrịa shuga mellitus ụdị 1 jikọtara erughị eru ọgwụgwọ. Ọrịa nwa ruo ogologo oge ezughi oke insulin dose ndinọ, ma ọ bụ na-eji ngwaahịa nke ala àgwà na ihe ọjọọ ọcha, na-eduga a ụkọ nke insulin.

Ruo ogologo oge enweghị insulin ke idem-eduga ná ndị na-esonụ usoro:

• Metabolic ọrịa, karịsịa ndị aghara nke carbohydrate metabolism.
• Amụba size of umeji ya ọdụdụ mbelata n'ihi ụba glycogen mmebi.
• Mgbanwe ọbara mejupụtara - abawanye na ọbara glucose, cholesterol, ọdụdụ asịd.

Ọ bụrụ na nke a bụ bụghị ihe niile enweghị nchịkwa Filiks ke ahụ mgbe Mauriac syndrome, nakwa ụmụaka ndị ọrịa na-enweta ndị na-esonụ deviations:

• Ezughi oke mmepụta nke mkpa homonụ - cortisol, ibu hormone, glucagon, na dị ka a N'ihi ya nke a mebiri nke ibu usoro.
• Mmebi nke na-edozi na ọgaranya mmepụta calcium na site si ọkpụkpụ, emecha na-eduga mmepe nke osteoporosis na ụfọdụ muscle atrophy.
• Enweghi absorption nke vitamin na eriri afọ.

The ọrịa na-egosipụta onwe ya n'agbata afọ 15-18 afọ, ma dysfunctional usoro amalite ọtụtụ mbụ. Ugbu a, maka ọgwụgwọ nke n'oge ọrịa na-eji oge a na juputara ọcha preparations, nke fọrọ nke nta eliminates mmepe nke Mauriac syndrome.

symptomatology nke ọrịa

Mauriac ọrịa ọrịa shuga nwere ọtụtụ pụtara:

• The nwa anaghị agasi na mmepe na uto mgbochi. Ọtụtụ mgbe, e ibu oké ibu na mkpụmkpụ. Na nke a, a na-arịa ọrịa na nwa nwere ike lag n'azụ ndị ọgbọ ha na ibu site 10-30 sentimita.
• The lag mmekọahụ mmepe (underdeveloped mmekọahụ e ji mara na-anọghị nke ịhụ nsọ na ụmụ agbọghọ).
• Protracted-eto eto.
• Ibu, karịsịa na ihu na elu ahụ na mkpa aka na ụkwụ. Ọrịa ụmụ ndị yiri nnọọ, nwere a "mpekere onwa ekara" onye ọ bụla ọzọ iru, obere olu, abụba ke ogwe aka, ubu, afo. The ala akụkụ nke ahụ bụ nnọọ mkpa.
• Mụbara imeju so circuitous venous mgbasa.
• Osteoporosis (selata mmepe nke ọkpụkpụ anụ ahụ).
• anya ọrịa, gụnyere ọrịa na nke retina, na ekemende mmepe nke cataracts.

The kasị Mauriac syndrome na ụmụ, na-arịa ọrịa na nwa foto na-egosi ihe mgbaàmà nwere ọrịa ahụ.

nchoputa nke ọrịa

The nchoputa nke "Mauriac syndrome" atọrọ na ndị doro anya mpụga ịrịba ama nke ọrịa, dị ka: enweghị elu afọ, ọnụnọ nke ibu oké ibu, karịsịa na ihu nke mmekọahụ obere nwa, a doro anya na-abawanye na imeju.

Iji gosi nchoputa rụrụ ọbara ule, nke rụpụtara na-esonụ ngosipụta bụ:

• ejighị n'aka glucose na-etoju, na-egosi mgbe nile na jumps na nnukwu, mgbe ahụ, ala.
• Oké ọbara lipids (hyperlipidemia).
• A ịrịba ama mmụba na ọbara cholesterol etoju (hypercholesterolemia).

Mgbe ụfọdụ, n'ihu na-amụ imeju biopsy n'ihi nzube a, ime ka abụba ibu

ọrịa

N'ihi na a na-eto eto ahụ na ọrịa bụ oké ike. Dị ka a oké ụdị ọrịa shuga, ọ bụ ike chefuo. Ọtụtụ mgbe na-akpata acidosis na hyperglycemic amaghị onwe ya.

Mauriac syndrome n'akparamagwa oké n'ihi na nke ugboro ugboro mgbakwunye na nke dị iche iche ọrịa, nakwa dị ka mgbagwoju na ogologo mgbake Biochemical Filiks ke idem.

Mauriac syndrome na Nobekura: Myirịta na Iche

Mauriac syndrome na Nobekura mgbe amalite na nwata, ma ọrịa na-a oké njọ sikwuoro nke-arịa ọrịa shuga na-adị na a ndabere nke na-ezighị ezi omume. Ma ọrịamkpokọta nwere yiri mgbaàmà, ha na-agụnye ibu iberibe na-enwe mmekọahụ mmepe, a protracted uto, imeju abụba. Na nke a, isi ihe dị iche Nobekura syndrome bụ enweghị ngafe ahu abụba. Na nke a, na ọgwụgwọ nke ma ọrịamkpokọta Aims mmerụ ọgwụgwọ nke ọrịa shuga.

Mauriac syndrome ọgwụgwọ

Direct ọgwụgwọ nke syndrome dị ka nke a na-adịghị adị. All ọgwụ iji na-agwa okwu na-akpata ọrịa na nsogbu ha kpatara. Iji mee nke a, onye ọrịa na-kenyere ziri ezi na insulin ọgwụ na ezi usoro onunu ogwu na àgwà na oge a na ọgwụ ọjọọ.

Iji gbochie lipodystrophy ẹkenịmde a ọnụ ọgụgụ nke mgbochi usoro n'ihi nzube a họpụtara dị ka ịhịa aka n'ahụ, physiotherapy na chaatị dị iche iche maka insulin injections. pụrụ iche nri nwere ike na-kenyere na mgbakwunye na ọgwụgwọ, na-ekwe n'aka nri niile oriri nwere anụmanụ abụba. Na nke a, na ihe oriri na-elekwasị anya protein na carbohydrate oriri.

Medical ọgwụ na-e chefuo ịzụlite na ndabere nke kpatara ọrịa nsogbu a ndidi naznachetsya:

• Admission gepatoprotektorov iweghachi imeju mkpụrụ ndụ.
• B vitamin iweghachi nkịtị metabolism.
• Mgbagwoju ọgwụ iji na normalizing na-etoju nke lipids na cholesterol ke iyịp.
• Steroid ọgwụ ọjọọ na-akpalite ahụ si na-eto.
• Mmiri ọgwụ ọgwụ ọjọọ iji weghachi mkpa mmekọahụ ọrụ.

Mgbochi na prognosis

Iji gbochie nke a dị ize ndụ sikwuoro nke ọrịa shuga dị ka Mauriac syndrome, ọ dị mkpa na-ebu ezi omume nke kpatara ọrịa ma gbochie insulin erughi.

Mgbe jikọtara omume esesịn mepụtara syndrome oge a ọgwụ ọjọọ prognosis mma ezu, ma ọ bụrụ na niile dọkịta na-atụ aro. The ezi onunu ogwu nke insulin na weghachite arụmọrụ nke niile akụkụ-emetụta na-enwe ike ịlaghachi nwa ahụ ike, ma mpụga na esịtidem.