Waterhouse syndrome - Friderichsen: pathogenesis, chisamaliro mwadzidzidzi, chithandizo

The zopangitsa adrenal mu thupi ndi zofunika endocrine limba. Mahomoni umatulutsa iwo bwanji magawo a kuthamanga ndi kuchuluka kwa madzimadzi kufalitsidwa, mlingo wa mchere mchere ndi microelements munthu mu mtengo kagayidwe kachakudya, ngakhale pa pituitary England. Ndipo zinthu kumene ntchito ya aang'ono amenewa koma chofunika kwambiri England akuvutika, kukhala boma, kusokoneza miyoyo ndi thanzi.

Tanthauzo ndi mbiri

Waterhouse syndrome - Friderichsen - ndi pachimake adrenal insufficiency, amene amapezeka pambuyo zoopsa kwa thupi la kukha magazi mu parenchyma ake. Izi zimayambitsa kuyan'anila kwambiri chilengedwe mkati mwa thupi ndipo kungachititse kuti imfa.

Kufotokozera za chikhalidwe choyamba anaonekera mu 1894, koma zochepa, ndipo mu 1911, Waterhouse limatchula milandu fifitini, zomwe zinamuthandiza kusonkhanitsa ndalama zonse pamodzi. Kusunga naye, zaka zisanu ndi ziwiri kenako mu 1918, Frederiksen komanso mabuku nkhani za ndondomekoyi pathological.

zifukwa

Asayansi amavomereza kuti syndrome Waterhouse - Friderichsen anachititsa kukha mwazi chachikulu pa nthawi yomweyo mu zopangitsa onse adrenal. Zikanavulaza chimadalira - wakhanda, makanda, ana ndi achinyamata. Pansi Pankhaniyi ogwirizana. boma akhoza zidzayambike ndi yobereka yaitali, yoopsa mpweya njala kapena kupititsa patsogolo kudzera m'njira kubadwa ntchito forceps kapena zingalowe Sola. Komanso yofunika kwambiri chifukwa chaukali kobadwa nako matenda, ndi mimba Vuto la gestosis mochedwa.

Waterhouse syndrome - Friderichsen akuluakulu nthawi zambiri kumaoneka ngati vuto la matenda ndi matenda. Childs, wothandizila ndi meningococcus, streptococcus kapena staphylococcus. Koma kuwonjezera pa zimenezi, zimene zimayambitsa kukha mwazi kungakhale nosologies monga chikuku, fever, tayifodi, diphtheria, ndi neoplasms zilonda, zotupa, peritonitis DIC ndi thrombosis adrenal mitsempha.

Chitukuko cha matenda

Olemba ambiri olekanitsidwa syndrome umenewu monga mbali ya limagwirira dziphunzitsiranso cha kulephera kukula kwa sepsis pachimake. Koma n'zotheka kuti ana ndi amayi oyembekezera limagwirira chosiyana ndi zomwe akufotokozera syndrome Waterhouse - Friderichsen. Pathogenesis izo kumaonekera ambiri hemorrhagic necrosis wa kotekisi adrenal. Iwo anali ambiri moti thupi lonse ankawaviika magazi, pali hyperextension ya kapisozi, ndipo ngakhale chophukacho ake.

Koma pathogenesis ambiri, izo anawonetseredwa mu mawonekedwe a sepsis:

- dilated capillaries ndi arterioles;
- kutchulidwa kuledzera syndrome;
- pamaso pa meninjaitisi concomitant kapena meningoencephalitis;
- kuwonjezeka mu mfundo dera lymph ndi thymus.

symptomatology

Waterhouse syndrome - Friderichsen ndi matenda meningococcal angathe mwadzidzidzi mu maziko a bwino. Iye akuchita mofulumira kuti munthu akhoza kufa pasanathe masiku.

Izo zonse akuyamba ndi kumwetsa excitability, irritability ndi mutu. Pamodzi m'mimba kupweteka m'thupi, nseru, kusanza ndi kutsekula m'mimba. Ngati ululu ali kwambiri kwambiri, adokotala akhoza amaganiziridwa odwala ndi kudwala pachimake opaleshoni. Poyamba, otsika kalasi malungo, koma monga maola ochepa a isanayambike ndi madigiri 39-40. Chifukwa kusanza ndi kutsekula m'mimba kwa thupi kusiya madzi ndi mchere, imbaenda kusokonezeka kwa mtima, ubongo ndi ziwalo zina zofunika. Pomaliza, munthu watha chikumbumtima ndipo igwera mu chikomokere.

Pali mtundu wina wa matenda, amene kumaonekera mu kugwa lakuthwa magazi kapena kugwa. Zizindikiro zina osati konse. Munthu woyamba mu stupor, koma monga hypoxia m'kati mwamsanga stupor ndi chikomokere. N'kumwalira ayambirenso kutsitsimuka.

Waterhouse syndrome - Friderichsen ana kukhala mofulumira kuposa akuluakulu, kaya cholingacho. Popeza luso lawo compensatory wa chamoyo mofulumira zatha, ndi kachitidwe ambiri ndi ziwalo adakali mu ndondomeko ya mapangidwe ndi chitukuko, chifukwa abwera masiku oyamba a isanayambike matenda.

diagnostics

Waterhouse syndrome - Friderichsen osauka kwambiri moti madokotala nthawi zambiri ndiribe nthawi kuchita kufufuza zonse ndi kuona zimene zimachitika munthu. Ngati mwadzidzidzi chipinda ntchito dokotala akuganiza kudwala ichi, chithandizo akuyamba yomweyo, ndi matenda akulowa mu ndondomeko, chifukwa nkhaniyi pa koloko.

Laboratory kwa chitsimikiziro cha matenda kuti tipeze mu kusanthula ambiri a magazi:

- leukocytosis ndi leftward kosangalatsa wa njira;
- kuchepa shuga magazi;
- thrombocytopenia ndi otsika clotting;
- kuchepetsa electrolytes;
- kuwonjezeka magazi urea asafe.

Komabe, zizindikiro izi si yeniyeni ndipo mwina zimasonyeza osiyanasiyana opaleshoni ndi matenda zokhudza zonse. Kunena zoona, muyenera mosamala kusonkhanitsa mbiri zachipatala, n'kukagwira lumbar puncture ndi kulongosola udindo neurologic. Koma zonsezi zachitika pambuyo olimba ya moyo wa munthu.

matenda masiyanidwe

Waterhouse syndrome - Friderichsen kusiyana kwa zinthu opaleshoni m'mimba monga appendicitis pachimake, pachimake kapamba, chilonda perforation a m'mimba kapena duodenum. Onetsetsani dokotala ayenera Yang'anani zizindikiro meningeal, kupewa magazi mu ubongo, cavernous thrombosis nkusani. Pamene mtima ECG mawonekedwe adzakuthandizani kumvetsa ngati munthu ali ndi matenda a mtima kapena ayi.

chithandizo

Mu malo oyamba pambuyo matenda "syndrome Waterhouse - Friderichsen" ayenera zoperewerazo wodwala thukuta madzi electrolytes ndi kusowa kwa timadzi adrenal. Kuti izi umayamba mwa mwayi venous kwa theka la lita "hydrocortisone" kapena 120 mamiligalamu "prednisolone" magalamu atatu peresenti shuga asanu ndi Botolo khumi peresenti asanu njira ya ascorbic asidi. Kotero ife kuchita pathogenesis zofunika, kuchititsa matenda Waterhouse - Friderichsen. chisamaliro Emergency nthawi yomweyo bwino magazi rheology, dilutes izo kumawonjezera chiwerengero cha dongosolo madzimadzi ndi kuonjezera zoipazi. Steroid timadzi m'malo amatithandiza kusungabe kuthamanga pa mlingo bwino.

Pambuyo olimba ndi mahomoni akupitiriza kuperekedwa intramuscularly, "Hydrocortisone" wa 50-75 mg wa mawola asanu ndi limodzi, ndi deoxycorticosterone nthochi pa Botolo khumi katatu patsiku. Tsatiraninso zizindikiro za kuthamanga ndipo ngati n'koyenera, ndi matani "Epinephrine," "Mezaton" glycosides mtima.

Ngati chifukwa cha matenda wakhala matenda, monga meningococcal, kuwonjezera mankhwala chachikulu, wodwalayo wapatsidwa mankhwala. Kamodzi akadzabweranso mtima mwakale, mahomoni kuyamba pang'onopang'ono kuchepetsa mlingo. Nkofunika bwino kukonzekera thupi zimene ayenera paokha kubala glucocorticoids. Musati mwadzidzidzi kuletsa mankhwala, izo zingakubweretsereni mavuto chachiwiri.

Nkofunika kuti atsimikizire kuti wodwalayo chabe Waterhouse syndrome - Friderichsen. Mankhwalawa yokha ikhoza kukhala yoopsa monga thupi osefukira kwambiri amachititsa zokhudza zonse edema, kuphatikizapo edema oziziritsa, ndi mlingo waukulu wa mahomoni zimachititsa mavuto a m'maganizo. Choncho, mmene wodwalayo n'kofunika kwambiri nthawi zonse liyang'anire ndi kusintha m'maudindo malinga ndi zosowa zake.

kaonedwe

Waterhouse syndrome - Friderichsen - kudwala aakulu zedi, amasonyeza kuti si nthawi zonse pa nthawi chifukwa cha zizindikiro zosiyanasiyana si yeniyeni. Zingadalire si bwino adzadzikuza dokotala mu zochitika mwadzidzidzi, komanso pa mlingo chiwonongeko cha zopangitsa kwa adrenal ndi luso compensatory wa chamoyo. Kaŵirikaŵiri mathero matenda ndi amapha.

kupewa

Izi zili choncho makamaka miyeso odana ndi mliri mu moto wa matenda imene wodwala ali ndi matenda meningococcal. Dokotala amene waulula ngati wodwala ayenera kuti awadziwitse anayendera ukhondo ndi kudzipatula wodwalayo. Mu masiku atatu, cheke cha anthu kukhudzana pamaso pa matenda kwaokha ntchito mmudzimo kapena bungwe maphunziro kwa masiku khumi. Monga kukonzekereratu, anthu mogwirizana ndi wodwala, mankhwala yochepa njira ya mankhwala.

EPIDEMIOLOGY

Waterhouse syndrome - Friderichsen, mwatsoka, zachilendo kudwala. M'mayiko otukuka, zochitika za matenda meningococcal Nthawi 1-3 pa 100 000 alionse. Ndipo oposa theka la milandu - ana a zaka sukulu. Komanso, aliyense khumi waona nyamuka mu zochitika za. Ichi ndi chifukwa masinthidwe a anthu tizilomboto ndi chitetezo chokwanira zimachepa.

Ziŵerengero amapezeka sizoona, monga Nthawi zambiri kupita undiagnosed chifukwa zizindikiro za mtanda. Waterhouse syndrome - Friderichsen osauka kwambiri moti madokotala mulibe nthawi kuchita okwanira mayesero.