Ngaba umntwana wakho unengxaki ngemithambo ye-lymph emathunjini akhe? (I-Primary Intestinal Lymphangiectasia) Masithethe ngale nto!

Ngaba wakha waphawula ukuba imilenze neengalo zomntwana wakho zidumba ngamanye amaxesha? Okanye usoloko ekhalaza ngentlungu yesisu? Ngaba ulahlekelwa bubunzima ngaphandle kokunyuka ubunzima? Usenokucinga ukuba ezi ziimpawu eziqhelekileyo esingazinaki kangako. Nangona kunjalo, emva kwezi, kunokubakho imeko engaqhelekanga kodwa exhalabisayo ebizwa ngokuba yi-'Primary Intestinal Lymphangiectasia' (PIL) . Eli gama livakala liyoyikisa kancinci, akunjalo? Kodwa ungakhathazeki. Makhe sithethe ngale nto ngokweenkcukacha nangokulula namhlanje.

Yintoni 'iPrimary Intestinal Lymphangiectasia'?

Ngamafutshane, esi sisifo esingaqhelekanga esenzeka kwinkqubo yokwetyisa yabantwana bethu, ingakumbi amathumbu amancinci. Uyazi, uninzi lokutya esikutyayo kuyagaywa kwaye izondlo ezikuyo zifunxwa emzimbeni kweli thumba lincinci.

Ngoku, kule meko (ye-PIL), inkqubo yetyhubhu encinci ebizwa ngokuba yi-'lymph vessels' emathunjini amancinci omntwana wakho yonakele kwaye ayisebenzi kakuhle. Le mithambo ye-lymph yinethiwekhi entle kakhulu yemijelo esasazeke kuwo wonke umzimba wethu. Ifana nenye 'inkqubo yepayipi' emzimbeni wethu. Umsebenzi wayo ophambili kukuqokelela ulwelo kunye neeproteni eziqokelelana kwizicubu zethu (esilubiza ngokuba lulwelo 'lymph') size sizibuyisele kwinkqubo yokujikeleza kwegazi.

Kodwa kumntwana one-PIL, ezi lymph vessels ziyadumba kwaye 'zivuza'. Okwenzekayo kukuba endaweni yokufunxa izondlo kunye neeproteni ezivela kwezi lymph vessels emzimbeni, zivuza zibuyele emathunjini. Kufana namanzi aphuma kumbhobho ovuzayo.

Xa izinto zivuza ngolu hlobo, kuvela iingxaki ezimbini eziphambili:

1. Amanqanaba aphantsi eeproteni: Iiproteni yinto eyenza umsebenzi obaluleke kakhulu emzimbeni. Ngoko ke xa ezi zivuza zingena emathunjini, umzimba awufumani protein yaneleyo. Oku kunokubangela ukuba umzimba wonke udumbe. Sikwabiza le meko ngokuthi 'yi-protein-losing enteropathy' ngokwezonyango.

2. Ukunqongophala kwesondlo: Kunye nolo lwelo luvuzayo, iivithamini kunye neeminerali ezibalulekileyo nazo ziyalahleka. Ukuba oku akunyanyekelwa kakuhle, umntwana unokufumana ukunqongophala kwesondlo okwahlukeneyo.

(PIL) ixhaphake kakhulu kubantwana abancinci kunye nabakwishumi elivisayo. Idla ngokuchongwa ngaphambi kokuba ifikelele kwiminyaka emi-3. Nangona kunjalo, ngamanye amaxesha inokuchaphazela abantu abadala kwaneemveku ezisesibelekweni. Akukho nyango lwayo okwangoku. Nangona kunjalo, oogqirha banokulawula iimpawu kwaye banciphise ukuvuza kunye nokudumba ngokusebenzisa isicwangciso sokutya esikhethekileyo kunye namayeza. Banokunika umzimba iivithamini kunye neeminerali ozifunayo.

Le meko (PIL) ibizwa ngamanye amagama aliqela:

• I-lymphangiectasia yamathumbu
• I-Lymphangiectasia
• Isifo sikaWaldmann

Zithini iimpawu zale meko (PIL)?

Uphawu oluphambili noluqhelekileyo lwe-PIL kukudumba okubangelwa kukugcinwa kolwelo. Oogqirha babiza oku ngokuba yi-lymphedema . Usenokuqala ukukubona oku emilenzeni nasezinyaweni zomntwana wakho. Njengoko olu lwelo luqokelelana, ubuso bomntwana wakho, izandla, kwanendawo yangasese inokuqala ukudumba.

Khawucinge nje, xa umntwana wakho evuka kusasa, iinyawo zakhe ziqhelekile, kodwa njengoko imini iqhubeka, ngokuhlwa, iinyawo zakhe zidumbile kangangokuba azikwazi nokunxiba izihlangu. Yiloo nto eyenzekayo kule meko.

Ukongeza, umntwana usenokuba nolwelo olujikeleze intliziyo (olubizwa ngokuba yi -pericarditis ), ulwelo esifubeni (olubizwa ngokuba yi-pleural effusion ), okanye ulwelo esiswini (olubizwa ngokuba yi-ascites okanye i-abdominal effusion) .

Nazi ezinye iimpawu:

• Intlungu zesisu
• Isicaphucaphu nokuhlanza
• Urhudo (intlungu yesisu )
• Ndiziva ndidiniwe ngalo lonke ixesha (Ukudinwa)
• Ukuhla ukusinda
• Ukungatyebi nokuba utya ukutya okungakanani na (oku kungenxa yokuba umzimba awufunxi kakuhle izondlo, okubizwa ngokuba yi -'malabsorption' )

Ukuba umntwana wakho unenye okanye ngaphezulu kwezi mpawu, kungcono ukufuna ingcebiso kagqirha.

Ngaba kukho naziphi na iingxaki ezinkulu zemeko (ye-PIL)?

Ewe, nangona kunqabile kakhulu, (i-PIL) inokubangela ukuba umzimba womntwana wonke udumbe kakhulu. Oku kubizwa ngokuba yi -'anasarca' . Le yimeko enzima enokuba yingozi nakubomi. Ke ngoko, kubaluleke kakhulu ukuqaphela iimpawu.

Yintoni isizathu sale meko (PIL)?

Le meko (i-PIL) ibangelwa kukungaqheleki kwimijelo ye-lymphatic yomntwana. Le mijelo ye-lymphatic, njengoko kukhankanyiwe ngaphambili, yinkqubo yeetyhubhu ezithwala izinto ezinje ngamafutha ukusuka emathunjini amancinci omntwana ukuya esibindini nakwezinye iindawo zomzimba.

Oku kungaqhelekanga kubangela ukuba imithambo ye-lymph ivaleke. Emva koko, izaliswa lulwelo lwe-lymph, imithambo idumbe, ekugqibeleni iqhume, kwaye umxholo wemithambo uqokelelana emathunjini amakhulu omntwana (ikholoni).

Nangona kunjalo, iingcali zonyango azikayazi ukuba kutheni le meko isenzeka. Yiyo leyo ingxaki.

Ingaba oku kuyimfuza? (Ingaba i-intestinal lymphangiectasia inemfuza?)

Kunqabile kakhulu ukuba kubekho iingxelo zokuba amalungu amaninzi osapho olunye achaphazeleka yile meko. Nangona kunjalo, abaphandi abakaqiniseki ukuba le yimeko yemfuza. Uphando oluthe kratya luyenziwa ngale nto.

Sifunyaniswa njani esi sifo (i-PIL)? (Ukuxilongwa)

Ixesha elininzi, into yokuqala ugqirha aza kuyenza kukuhlola umntwana wakho aze akubuze ngeempawu zomntwana wakho. Ngamanye amaxesha, i-ultrasound yokukhulelwa , eyenziwa ngelixa umntwana esesesibelekweni, inokubonisa iimpawu zokudumba (i-edema) emilenzeni yomntwana, nto leyo enokukunika umbono wokuba kwenzeka ntoni.

Emva kokuba umntwana ezelwe, ugqirha uza kujonga i-PIL ngokusebenzisa isixhobo esikhethekileyo esibizwa ngokuba yi-endoscope ukujonga ngaphakathi kwamathumbu omntwana. Le yityhubhu eguquguqukayo enekhamera eqhotyoshelwe kuyo. Umntwana uza kulaliswa phantsi kwe-anesthesia ngexesha lovavanyo, ukuze angaziva ntlungu. Ngoko ke ungakhathazeki.

Ngexesha lovavanyo lwe-endoscopy, ugqirha uthatha iziqwenga ezincinci zezicubu (ezibizwa ngokuba yi-biopsy ) kwiindawo ezahlukeneyo zamathumbu omntwana. Emva koko, ingcali yezifo , ugqirha ochwephesha ekuhloleni izicwili, ujonga ezi sampuli zezicubu phantsi kwe-microscope ukujonga nakuphi na ukudumba okungaqhelekanga kwemithambo ye-lymph.

Ukongeza, uvavanyo lwegazi lunokufumanisa ukuba ingakanani na iproteni emzimbeni. Isampuli yendle ingathathwa ukuze kubonwe ukuba ulwelo luyavuza na kwi-lymph nodes ukuya emathunjini amancinci.

Zeziphi iindlela zonyango ezilungileyo (i-PIL)?

Xa unyanga i-PIL, eyona nto ibalulekileyo kukutshintsha indlela umntwana atya ngayo. Esi sisixhobo esiphambili sokulwa ne-PIL. Cinga ngayo njengokubeka 'iplagi' yokuthintela ukuvuza kwezakhamzimba.

Oogqirha abaninzi bacebisa ukutya okunamafutha aphantsi kunye neproteni ephezulu.

Amafutha kunzima ukuwagaya. Ngoko ke kunzima ukuba amathumbu omntwana wakho avuzayo afunxe amafutha ngokufanelekileyo. Ngoko ke ukutya kwakho okutsha kunokubandakanya uhlobo oluthile lwamafutha olubizwa ngokuba yi -'medium-chain triglycerides (MCTs).' Ezi (MCTs) luhlobo lwamafutha olufunxwa lula ngumzimba. Kwakhona, ukongeza iiproteni ezingaphezulu ekutyeni kwakho kunokwandisa inani lezondlo ezifunxwa ngumzimba wakho.

Ekubeni ezinye izondlo zilahleka, ugqirha unokucebisa izongezo zeevithamini kunye neeminerali ukuqinisekisa ukuba umzimba womntwana ufumana oko ukudingayo.

Ukongeza, ugqirha angakunika amayeza afana nala ukunceda kwiinkalo ezahlukeneyo zemeko (i-PIL):

• I-Diuretics (ekwabizwa ngokuba ziipilisi zamanzi): Ezi zisebenza ngokunciphisa ubungakanani bolwelo olongezelelweyo oluqokelelana emzimbeni ngenxa yokuvuza kweproteni.
• Ii-Somatostatin analogues (ezifana ne-octreotide): La mayeza anceda ekunciphiseni ukuvuza kwezinto ezivela kwinkqubo ye-lymphatic.

Ezinye iindlela zonyango:

• Utyando: Ukuba ezinye iindlela zonyango azincedi, kwaye azifane zenzeke, oogqirha banokucebisa utyando lokususa inxalenye egulayo yamathumbu omntwana wakho.
• Ezinye iindlela zonyango ezinokubakho: Uphando ngale nto luyaqhubeka. Amayeza amatsha amaninzi, afana ne -sirolimus, ayavavanywa kwi-PIL. La mayeza anokuba luncedo kwezinye iimeko, kodwa awasetyenziswa kakhulu okwangoku.

Ithini ingqikelelo yemeko (ye-PIL)?

Njengoko bekutshiwo ngaphambili, i-(PIL) ayisosifo esinokunyangeka ngokupheleleyo. Nangona kunjalo, amanqanaba eeprotheyini egazini lomntwana kufuneka anyuke kwiiveki ezimbalwa emva kokuqala unyango. Nangona kunjalo, umntwana kuya kufuneka atye ukutya okunamafutha aphantsi aze athathe izongezo zesondlo ubomi bakhe bonke.

Nangona oku kuyingxaki, ukuba kulawulwa ngendlela efanelekileyo, umntwana angaphila ubomi obuqhelekileyo. Eyona nto ibalulekileyo kukulandela imiyalelo kagqirha nokunyamekela ukutya komntwana.

Ngaba imeko ye (PIL) ingathintelwa?

Ekubeni abaphandi bengazi ukuba yintoni ebangela le meko (PIL), okwangoku akukho ndlela yokuthintela le meko.

Umntwana wam ufanele aye nini kugqirha?

Ukuba ubona ukudumba emilenzeni, ezinyaweni zomntwana wakho, okanye naziphi na iimpawu ze-PIL ezikhankanyiweyo apha ngasentla, kungcono ukumsa umntwana wakho kugqirha ngoko nangoko. Okukhona ibonwa kwangoko, kokukhona unyango lunokuqala ngokukhawuleza.

Yeyiphi imibuzo endifanele ndiyibuze ugqirha womntwana wam?

Ukuba umntwana wakho une-PIL, usenokuba nemibuzo emininzi ekufuneka uyibuze ugqirha wakho. Nazi ezinye zazo:

• Yeyiphi indlela yokutya efanelekileyo kule meko (PIL)?
• Zeziphi izongezo endifanele ndizinike umntwana wam?
• Yintoni enye endinokuyenza ukuze ndikwazi ukulawula iimpawu zomntwana wam?
• Ingaba le meko (ye-PIL) imbi kakhulu?
• Zeziphi iingxaki ekufuneka ndiziqaphele?

Musa ukoyika ukubuza le mibuzo. Xoxa nogqirha wakho ngayo nayiphi na into ekuxhalabisayo.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Xa uqala ukufumana iimpawu zePrimary Intestinal Lymphangiectasia, kunokuba nzima kuwe noogqirha bakho ukuqonda okwenzekayo. Kungaba ngamava adidayo kakhulu nadidayo. Nangona kunjalo, oogqirha bakho banokusebenzisana nawe ukukunceda ufumanise ukuba yintoni ebangela oko.

Ngethamsanqa, ngokutya okunamafutha aphantsi kunye nezongezo zesondlo, iimpawu zomntwana wakho zinokulawulwa ngokuhamba kwexesha. Buza ugqirha womntwana wakho ukuba yintoni enye onokuyenza ukunceda umntwana wakho alawule le meko. Uthando lwakho, inkxaso, kunye nengqalelo zibaluleke kakhulu kumntwana wakho ngeli xesha lohambo.

"I-lymphangiectasia yamathumbu ephambili, i-PIL, ukuvaleka kwe-lymphatic, izifo zesisu ebantwaneni, ukudumba kwemilenze, ukungabikho kweproteni, izifo zenkqubo yokugaya ukutya