Harlequin Syndrome: Zviratidzo, zvinhu uye kurapwa

Vana vacheche vari pangozi chaizvo. Kunyange mudumbu, vanogona kukanganiswa siyana kunaka zvinoita kuti kuti zvokurwara dzinodya zvimedu. Somugumisiro, akaberekwa, kukambaira ane yakawanda nematambudziko. Mumwe yakaipisisa kutsauka ndiye Harlequin syndrome. The chirwere inonzi pashure chimiro rokuItari commedia dell'arte, vaipfeka sutu yakagadzirwa mavara jira scraps.

Harlequin urwere mu achangoberekwa

Doctors kuwana dambudziko pakarepo pashure kuberekwa kwemwana. Maererano navo, ivo Harlequin urwere zvinogona kutsanangurwa Kusakwana autonomic ino kuti hachisisiri kodzero kudzora vascular matauriro. The chirwere inogona kukonzerwa nyore negiravhiti. Nei izvi zvichiitika? Vanachiremba havagoni kupa mhinduro yakajeka yemibvunzo okukurumidzira. The siyana zvikonzero chaizvo paupamhi; kubva nyore kukundikana fetus kuti murombo mararamiro sarudzo, iyo inotungamira mukadzi pamuviri.

The chirwere anogona kuratidza sezvo zvose pamusoro muviri, uye chete mune dzimwe nzvimbo dzomuviri. Vana ava, vanachiremba vanoti, vanogara vachirwara chikosoro, nokuti vakanetseka thermoregulation. Toddler chero nguva yegore kuchaita ziya kwazvo, ipapo pakarepo kuna supercool, somugumisiro anosimbirira kutapukirwa noutachiona anopiwa kwaari. Mukuwedzera, chirwere anopa kusanzwa uremu, saka kazhinji mwana ari kuchema, nayo zororo uye nowanonetsa.

zviratidzo

Maererano nedzimwe nemishumo, kuti chirwere unogona kuva mugumisiro fetus asphyxia kana kuwana navo pakuzvarwa intracranial kukuvara. Akanga zvakare chirwere vanenge maronda ari hypothalamus uye medulla oblongata. Chero zvazvingava, zano pakarepo kukonzera wetsinga. Wanikidza hosha iri nyore; ndicho chikuru chiratidzo akatsvuka ganda, apo muvhuruvhuru norutivi rwaro. Panyaya iyi, muganhu ruvara nokuchinja kunoitika chaicho mutsara nomuzongoza.

Gogonera ruvara nhengo dzomuviri kunoitika maminitsi mashoma pashure rikaisa mwana. Rinooneka sezvo Harlequin syndrome. Photo vana vaduku zvokurapa maenisaikoropidhiya dzinoratidza yakanyangara mufananidzo - yokumusoro chikamu muviri wavo une dzose ruvara, dambudziko inova piriviri, dzimwe nguva ane rinokwanisa kucheneruka tint. The chinoshamisa kuoneka kwemaminitsi anoverengeka. Dzimwe nyanzvi dzinoti kuti chirwere chingava chiratidzo Prematurity.

cutaneous kuratidzwa

Kana rondedzero chirwere kushandisa ezvokurapa, kuti Harlequin chirwere pachena pacharo somunhu tsindirana ganda keratinization. Hazvisi Yaunoti granular layer pachinzvimbo vanooneka plaques vakaumba panguva cracking walnut pamusoro Epiderme e. idzodzo izvi vane hexagonal chimiro, vanowanzoororwa vakaparadzana nevanhu zvakadzama tsvuku makatsemuka. ganda ruvara apo wachena kana jaundiced.

Vana vanoberekwa vaine chirwere ichi kazhinji vane dzimwe abnormalities vangadya kuitika deformation muromo (miromo iye kuyeuchidza hove), ectropion, isina kukodzera yakatanga zvakare sandara nzeve. Mune zvimwe zviitiko, nechirwere microcephaly, kana mwana ari underdeveloped dehenya: somugumisiro, akaberekwa aine pfungwa chaizvo duku. Vana vakadaro vachafira neonatal nguva kana pavaizvarwa.

zviri chirwere

Kids regai nguva dzose kufa, kana ivo vane zvokurwara pamusoro Harlequin. The chirwere hausi mutongo. The chirwere hazvigoni kutaurwa, kunze, kana akakurumidza chirwere uye nounyanzvi, unogona kuderedza mamiriro mafufu gadziriro chaidzo. vana ava vari kutyora kwete chete kupisa, asi vo electrolyte mwero. Chapped ganda utrudnyaet kufema uye kufamba. In huru makatsemuka vanogona kuwana utachiona uye kutanga sepsis.

Sezvineiwo, chirwere haisi zvakajairika chaizvo. Panyaya iyi, mwana zvepabonde chavanopinda hazvina basa; asi zvokurwara kubatwa vakomana nevasikana. Vanachiremba vanoti chirwere rinowanzopesana zvakaoma. The fungidziro kakawanda zvakakwana zvisingafadzi.. The aetiology of chirwere kuchiri kuzivikanwa.

kurapa

Mafufu ushandiswe yawapiwa, izvo ndehwokuti kuramba inowanzotorwa muviri tembiricha uye Kudzikamiswa mvura-electrolyte mwero. Uyezve, yakadai murwere kunoda kuipa kudya, antiinflammatory, antibacterial kurapa. Kushandiswa retinoids muchimiro kwamafuta uye dzaiita capsules yekudyidzana zvinowedzera mukana wokurarama, kana mwana wacho akanzi Harlequin syndrome. Treatment kunosanganisira kushandisa zvinodhaka, uko basa fuma iri isotretinoin uye acitretin. Pasinei neizvi, zviparare kuramba ganda kusvikira pakuguma kwemazuva. Zvakaita upenyu pavarwere izvi yekudyidzana ipfupi.

Kana urwere isina kutaurwa, zvinokwanisika kuita pasina mishonga. Vabereki nerutsigiro kuvatarisira chakaipa tembiricha - kumativi 20 inegumi. Dzidzisai mafufu kuti okunze kunyanyisa vanofanira zvishoma nezvishoma: kutanga, unofanira kuchinja nechando, uye chete ipapo kuti vakuchidzire. Ukadaro uchava vadzidzise autonomic maitiro uye rusvingo nembudzana midziyo. Yeuka: kana mwana akanga vangadikitira, unofanira kuchinja hembe pakarepo. Maitiro akadaro vanofanira kupa zvakanaka mugumisiro.