Yu pikin in skin rɛd ɛn swel? Lɛ wi lan bɔt Klippel-Trenaunay Syndrome (KTS)!

Yu dɔn ɛva notis se dɛn kin bɔn sɔm pikin dɛn wit big rɛd bɔn mak na wan say na dɛn bɔdi, mɔ na dɛn an ɔ leg, ɛn da an/leg de big smɔl pas di ɔda say? Ɔ sɔntɛnde yu kin si vein dɛn na da say de? Na nɔmal tin fɔ mek yu fred smɔl we yu si sɔntin lɛk dis. Tide wi go tɔk bɔt wan sik we de sho di sem kayn sik, we nɔ kin apin smɔl, we min se nɔto ɔlman kin gɛt am, ɛn we dɛn bɔn wit am. Dɛn kɔl dis Klippel-Trenaunay Syndrome (KTS) . Nɔ wɔri, wi go tɔk bɔt dis simpul wan, di we we yu go ɔndastand.

Wetin na di sik we dɛn kɔl Klippel-Trenaunay Syndrome (KTS)? Fɔ tɔk am simpul wan...

Imajin, KTS na wan sik we nɔ kin apin so ɔltɛm we kin kam wit wi pikin we i kam na dis wɔl (dat na, wan sik we wi bɔn wit ). Dis kin mek sɔm smɔl chenj dɛn na di we aw di pikin in sɔft tisu, bon, ɛn blɔd vesel dɛn de divɛlɔp. wan pan di men sayn dεm fכ dis na we wan pepul-rεd bכn mak de apin, we kin bi pan wan pan di an כ leg dεm, we dεn kכl ‘port-wine stain’ . Bɔku tɛm, pipul dɛn we gɛt dis KTS kin gɛt sɔm prɔblɛm dɛn bak wit dɛn limfatik sistɛm . dis limfatik sistεm na imכtant sistεm we de εp fכ kip wi bכdi wata bεlε.

Naw, no mɛrɛsin nɔ de fɔ KTS. Bɔt, nɔ panik. Bɔku fayn tritmɛnt dɛn de fɔ mɛn di sik dɛn . If dɛn no di sik ɛn trit am kwik kwik wan, lɛk afta dɛn bɔn di pikin, di wɛlbɔdi prɔblɛm dɛn we kin apin bikɔs ɔf KTS kin ridyus bad bad wan.

Sɔm dɔktɔ dɛn kin kɔl di KTS kɔndishɔn bak CLVM . Dis na di fɔs lɛta we gɛt 4 Inglish wɔd dɛn.

• C na fכ Kapilari dεm - dεn na di sכm sכm bכdi we de kכnεkt wi vein dεm εn at dεm.
• L tinap f כ Limfatik sistεm - Dis na pat pan wi imyun sistεm. I de kɛr wan wata we dɛn kɔl limf ɔlsay na di bɔdi.
• V tinap fɔ Veins - dis na di blɔd vesel dɛm we de kɛr blɔd go na wi at.
• Di lɛta M tinap fɔ Malfɔmeshɔn . I min se wan pat na di bɔdi nɔ de divɛlɔp nɔmal wan, ɔr difɔmiti de.

Dɛn gi KTS in nem to tu Frɛnch dɔktɔ dɛn, Maurice Klippel ɛn Paul Trenaunay, we bin kam fɔ no dis sik insay 1900. Masta sabi bukman dɛn se na lɛk 1 pan ɛvri 100,000 pipul dɛn ɔlsay na di wɔl de sɔfa wit dis sik. I kin afɛkt ɛnibɔdi, ilɛk uskayn trayb ɔ man ɔ uman.

Wetin na di sayn dɛm fɔ KTS? Aw yu no am?

Di sayn dɛm fɔ Klippel-Trenaunay Syndrome (KTS) kin afɛkt mɔ di vein dɛm, di kapilari dɛm, di sɔft tisu dɛm, di bon dɛm, ɛn di limf vesel dɛm na wi bɔdi. Lɛ wi si aw:

1. Kapilari Malfɔmeshɔn (CM): .

Dis na di say we di ‘pɔt-wayn stɛyn’ we wi bin dɔn tɔk bɔt, kin apin. Dis kin apin bikɔs di kapilari dɛn we de ɔnda wi skin kin swel. Dɛn bɔn mak ya kin bi wan pan di fɔs sayn dɛm fɔ sho se yu gɛt KTS. Dɛn spat ya kin gɛt difrɛn kɔlɔ dɛn, frɔm layt pink to dak pepul (wayn-rɛd). As yu de ol, dɛn spat ya kin tan lɛk blista, layt, ɔ dak.

2. Vein Malfɔmeshɔn (VM): .

Klose to ɔlman we gɛt KTS gɛt venɔs malfɔmeshɔn. dis kin afekt di vein dεm we de jכs oba di skin, we kin mek di vεn dεm we de na di leg dεm, spεshal wan na di groin εn di tכŋ dεm. Dɛn kin afɛkt bak di dip vein dɛm we de rɔn dip insay di bɔdi. dis kin mek di bכdi kכlכt na di dip vein dεm (Deep Vein Thrombosis - DVT) . DVT na wan sik we de mek pɔsin denja.

3. Sɔft tisu ɛn bon dɛn we de gro pasmak:

Dis na wan sik we, frɔm we i yɔŋ, wan an ɔ leg kin gro big pas di ɔda wan. Bɔrku tɛm, dis kin afɛkt wan an ɔr leg nɔmɔ, ɛn bɔrku tɛm na wan leg nɔmɔ kin afɛkt. Sɔntɛnde, wan leg kin lɔng pas di ɔda wan. Dis kin mek i nɔ balans, i nɔ kin izi fɔ waka, ɛn i nɔ kin muv bɛtɛ.

4. Limfatik Malfɔmeshɔn (LM): .

sכm pipul dεm we gεt KTS kin gεt εkstra limf vεsεl dεm na dεn limf sistεm, כ di limf vεsεl dεm we de naw kin shep bכku. Bikɔs dɛn ɛkstra limf vesel ya nɔ de wok fayn, di limf wata kin lik , we kin mek di leg dɛn swel, mɔ di fut dɛn, ɔ i kin gɛt prɔblɛm na di bɛlɛ, di blad, ɔ di ɔnda intestinal.

Wetin kin mek pɔsin gɛt KTS? Wetin mek dis kin apin?

bכku tεm, KTS kin kכz fכ wan vεryushכn na wan jin we dεn kכl PIK3CA . dis jεnεtik mכtεshכn de apin wan wan tεm, we min se nכ no kכz de apin. I nɔ gɛt frɔm mama ɛn papa . Dis min se ivin if nɔbɔdi pan dɛn mama ɛn papa nɔ bin gɛt KTS, pikin kin gɛt KTS bikɔs ɔf dis jɛnɛtik muteshɔn.

Sɔm pipul dɛn gɛt KTS we nɔ gɛt dis PIK3CA jin muteshɔn. So, di wan dɛn we de stɔdi bɔt dis biliv se KTS kin bi bikɔs ɔf di chenj dɛn we de apin na sɔm ɔda jin dɛn. Risach bɔt dis stil de go bifo.

Wetin na di pɔsibul kɔmplikeshɔn dɛm fɔ KTS?

KTS kin mek difrɛn kɔmplikɛshɔn dɛn. Na dat mek i impɔtant fɔ gɛt tritmɛnt kwik kwik wan. Lɛ wi tek wan luk pan wetin na dɛn prɔblɛm dɛn de:

• Blɔd klɔt: I kin apin, mɔ na dip vein (DVT).
• Sεlulayt: Dis na baktri infεkshכn we de apin כnda di skin.
• Fluid akyumyuleshכn εn swεla (Lymphedema): I kin kכz bay we di limfatik sistεm nכ de wok fayn.
• blɔd we de kɔmɔt insay: Blɔd kin apin na di gastrointestinal (GI) trakt, blad, ɔ uman riprodaktiv sistɛm.
• Pulmonary embolism: Dis na wan sik we de mek pɔsin in layf de pan denja we di blɔd we de fכm na di dip vein dεm de brok εn blok wan at we de na di lכng.

Na smɔl tɛm nɔmɔ, pipul dɛn we gɛt KTS kin gɛt prɔblɛm wit dɛn an ɔ fut we dɛn bɔn. Fɔ ɛgzampul:

• Fɔ gɛt ɛkstra finga dɛn (Polydactyly) .
• Finga dɛn we stɔp togɛda (Syndactyly) .

KTS de mek pɔsin fil pen?

Yes, KTS kin mek pɔsin fil pen.

• Varicose veins kin mek yu it ɔ pen.
• Prɔblɛm wit blɔd sakulayshɔn kin mek yu swel ɛn pen, mɔ na di ɔnda leg.
• If wan ɔgan, lɛk in leg, gro pasmak, i kin mek i fil se da leg de ebi ɛn i kin fil pen.

Aw dɔktɔ dɛn kin no se pɔsin gɛt KTS? (Diagnosis) .

Dɔktɔ dɛn kin fɔs no se i gɛt KTS bay we dɛn de si di sayn dɛn we dɛn gɛt na dɛn bɔdi. KTS dεn sכspεkt if prכblεm de na atכs tu pan di tri men εria dεm we wi bin dכn tכk bכt — kapilari, vein, כ di limb divεlכpmεnt. Bikɔs bɔku pan di sayn dɛm fɔ KTS kin de we dɛn bɔn am, i kin pɔsibul fɔ no se i gɛt KTS bifo yu pikin kɔmɔt na ɔspitul.

Us tɛst dɛn kin yuz fɔ no if pɔsin gɛt KTS?

Di dɔktɔ kin du tɛst lɛk dis fɔ no mɔ bɔt di sik ɛn fɔ no aw di prɔblɛm big:

• CT skan ɔ MRI skan: Luk di kɔndishɔn fɔ di sɔft tisu ɛn bon dɛn.
• Magnetic Resonance Angiogram (MR angiogram): Dis na spɛshal MRI tɛst we kin si klia wan aw di blɔd vesel ɛn vein dɛn de.
• Doppler ultrasound: Dis kin ɛp fɔ si blɔd flɔ tru di vein ɛn at.

Wetin na di tritmɛnt dɛm fɔ KTS?

Di tritmɛnt fɔ Klippel-Trenaunay Syndrome (KTS) kin difrɛn difrɛn wan bay di sayn dɛm wae ɛnibɔdi gɛt.. Nɔto ɔlman dɛn kin gi di sem tritmɛnt. Di men gol fɔ tritmɛnt na fɔ kɔntrol di sayn dɛm ɛn ridyus di risk fɔ gɛt prɔblɛm. Lɛ wi si wetin na di men we dɛn fɔ trit am:

• Mεdisin dεm we de mek blɔd tan / Anticoagulants: Fɔ ɛgzampul, mεdisin dεm lεk Heparin . dis dεm de ridyus di risk fכ blכd kכlכt na di lεg (DVT) εn pulmonary embolism.
• Sirolimus: Dɛn kin yuz dis mɛrɛsin fɔ stɔp di bɔdi fɔ rijek ɔgan we dɛn dɔn transplant. Bɔt dɛn dɔn si bak se i kin stɔp di vaskyuɛl malfɔmeshɔn fɔ wɔs.
• Kɔmpreshɔn stɔking: Dis na spɛshal kayn sɔks we de ɛp fɔ mek blɔd kɔmɔt bak frɔm di leg dɛn to di at. Dis kin ɛp fɔ ridyus di swɛlin, pen, ɛn di risk fɔ mek blɔd klɔt.
• Endovenous thermal ablation: Dis involv fכ yuz fכkus bim dεm fכ enεji fכ sial di prכblεm blכd vεsul dεm frכm insay. Dɛn kin du dis we di vein dɛn stil de. Dis kin mek i nɔr kin gɛt bɛtɛ tɛm fɔ wɛl ɛn nɔr kin fil pen.
• Laser therapy: Dɛn kin yuz fɔs, strɔng bim dɛm fɔ ɛnaji fɔ pwɛl ɔ pul di tisu dɛm we yu nɔ want. dis lεzεr tεrapi dεn de yuz fכ layt di kכla fכ wan ‘pכt-wayn stεn’ bכn mak.
• Sklɛrotɛrapi: Insay dis, dɛn kin injɛkt spɛshal sɔlvushɔn dairekt insay di vein dɛn, kapilari dɛn, ɔ di limfatik vessel dɛn we gɛt prɔblɛm. Dɔn di ship dɛn kin lɔk. Dis na tritmɛnt we rili fayn fɔ di vayrɔs veins.
• Shoe lifts: If yu leg dɛn nɔ lɔng di sem, yu kin put ay lif pan wan shuz fɔ kɔrɛkt dis. Dis kin ɛp bak fɔ mek yu nɔ gɛt skɔliosis .
• Ɔpreshɔn: Dɛn kin du ɔpreshɔn fɔ kɔrɛkt di prɔblɛm dɛn we de wit di vein dɛn ɛn fɔ mek di leg dɛn lɔng ikwal. Ɔ, dɛn kin du ɔpreshɔn fɔ pul di fat ɔ tisu we pasmak fɔ ridyus di sayz fɔ wan an ɔ leg we dɔn gro pasmak. Na smɔl tɛm nɔmɔ, if wan fut we nɔ big we nɔmal de mek i nɔ izi fɔ wɛr sus ɔ waka, dɛn kin du ɔpreshɔn fɔ pul di fut.

Yu tink se dɛn kin ebul fɔ stɔp KTS?

I sɔri fɔ no se bikɔs KTS kin apin randomly, no we nɔ de fɔ mek i nɔ divɛlɔp . Bɔt lɛk aw wi bin dɔn tɔk, di rayt tritmɛnt kin ɛp pipul dɛn we gɛt KTS fɔ liv ay kwaliti layf.

Us kayn fiuja pɔsin we gɛt KTS kin ɛkspɛkt?

Pan ɔl we no mɛrɛsin nɔ de fɔ KTS, dɛn kin ebul fɔ kɔntrol di sayn dɛm. Bɔrku tɛm, pipul dɛm wae gɛt dis sik kin liv lɛk aw dɛn kin liv .

Bɔt di we aw pipul dɛn kin si KTS kin difrɛn frɔm wan pɔsin to ɔda pɔsin. I dipכnt pan aw yu vaskulכr malfכmeshכn dεm de siriכs. Dɛn tin ya kin wɔs as tɛm de go. So, if yu gɛt GI blɔd, dip vein trombosis, ɔ pulmonary embolism , yu fɔ go to dɔktɔ wantɛm wantɛm . Blɔd we de klɔt ɔ we i de blɔd pasmak kin kil pɔsin.

Di advays ɛn tritmɛnt we dɛn kin gi yu ɔltɛm, dat kin mek yu nɔ gɛt prɔblɛm dɛn bad bad wan.

If a gɛt KTS, aw a go tek kia ɔf misɛf? / Aw a go tek kia ɔf mi pikin?

If yu ɔ yu pikin gɛt KTS, no bɔt dɛn tin ya:

• Tek kia ɔf yu skin gud gud wan: I impɔtant fɔ mek yu nɔ gɛt infɛkshɔn.
• Bɔku tɛm dɛn nɔ kin fayn fɔ yuz ɔmon fɔ kɔntrol bɔn pikin we gɛt ɛstrojen: Dɛn kin mek di blɔd klɔt bɔku. Tɔk to yu dɔktɔ bɔt dis.
• Tek mɛrɛsin fɔ mek yu nɔ gɛt blɔd we yu gɛt bɛlɛ: Yu dɔktɔ go advays yu bɔt dis.
• Tek mɛrɛsin dɛn we de mek yu blɔd tan bifo yu du di ɔpreshɔn: Ridyus di risk fɔ mek blɔd klɔt.

Ustɛm a fɔ go to di dɔktɔ?

Si yu dɔktɔ ɔltɛm ɔlsay na yu layf fɔ mek dɛn chɛk yu . Dis go mek yu dɔktɔ ebul fɔ wach yu sik ɛn trit ɛni nyu prɔblɛm we go kam. We yu de chɛk yu dɔktɔ ɔltɛm, dat go ɛp yu fɔ si aw yu tritmɛnt de wok fayn ɛn mek chenj dɛn to yu tritmɛnt plan if nid de.

Ustɛm a fɔ go na di Imejɛnsi Tritmɛnt Yunit (ETU) ?

If yu tink se yu gɛt blɔd we de klɔt (e.g., DVT, Pulmonary Embolism symptoms) ɔ if yu de blɔd bad bad wan (we de blɔd bɔku), yu fɔ go na di imejensi rum wantɛm wantɛm.

Wetin na di impɔtant kwɛstyɔn dɛn we wi fɔ aks di dɔktɔ?

If yu ɔ yu pikin gɛt KTS, yu kin aks yu dɔktɔ kwɛstyɔn dɛn lɛk:

• Us tritmɛnt dɛn we bɛtɛ fɔ mi/mi pikin?
• Aw ɔltɛm a fɔ kam fɔ chɛk-ap?
• Given mi current situation, wetin yu kin se boht mi fiuja (prognosis/outlook)?

Yu tink se KTS de mek pɔsin in layf de pan denja?

KTS insɛf nɔ de afɛkt di layf we pɔsin de liv dairekt wan. Bɔt sɔm kɔmplikeshɔn dɛn we kin apin bikɔs ɔf KTS, lɛk we pɔsin de blɔd insay pɔsin in bɔdi ɔ we i kin gɛt blɔd na in pulmonari, kin mek pɔsin in layf de pan denja . If yu de trit dɛn tin ya ɔltɛm, dat kin mek yu nɔ gɛt bɔku prɔblɛm dɛn.

KTS na disabiliti?

I kin apin. If yu nɔ ebul fɔ wok bikɔs ɔf di prɔblɛm dɛn we de wit KTS, lɛk dip vein trombosis (DVT) ɔ pulmonary embolism, yu kin fit fɔ gɛt disabiliti bɛnifit. Yu kin ebul fɔ gɛt bɛnifit dɛn bak lɛk disabiliti pak tag fɔ prɔblɛm dɛn lɛk we yu nɔ ebul fɔ waka bikɔs yu an ɛn fut dɔn big.

I kin tranga fɔ ɔndastand ɔl di simptom ɛn kɔmplikeshɔn dɛm fɔ KTS wan tɛm. Bɔt yu dɔktɔ kin ɛksplen dɛn ɔl to yu ɛn ɛp yu fɔ trit yu prɔblɛm dɛn. Nɔ fred fɔ tɛl yu dɔktɔ bɔt aw yu de fil ɛn ɛni nyu sayn we yu de gɛt . We yu tɔk opin wan lɛk dis, i go izi fɔ aks fɔ ɛp if yu gɛt ɛni prɔblɛm.

Wetin wi wan tek go os frɔm dis stori (Take-Home Message)

Okay, so wi don tok plenti abaut KTS, no bi so? Na sɔm tin dɛn we yu fɔ mɛmba:

• KTS na wan sik we nɔ kin apin so ɔltɛm, we dɛn bɔn wit . So nɔ wɔri, nɔto yu wan de.
• di men sayn dεm na ‘port-wine stain’ bכn mak, an/leg we big, εn vein prכblεm .
• Pan ɔl we no kɔmplit mɛrɛsin nɔ de fɔ dis, rili gud tritmɛnt dɛn de fɔ kɔntrol di sik dɛn .
• I rili impɔtant fɔ no di sik ɛn bigin fɔ trit am kwik kwik wan .
• I rili impɔtant fɔ mek dɛn de kia fɔ dɛn ɔltɛm ɛn gɛt tritmɛnt we nid fɔ de ɔlsay na yu layf.
• Kɔntinyu fɔ tɔk fayn to yu dɔktɔ. Aks ɛni kwɛstyɔn we yu gɛt, ɛn tɛl dɛn aw yu de fil.

A op se dis infɔmeshɔn dɔn ɛp yu fɔ ɔndastand KTS mɔ. If yu ɔ pɔsin we yu sabi gɛt dɛn sik ya, i go fayn fɔ mek yu go to dɔktɔ.

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