Ðe dɔléle sia si mebɔ o la le viwòa ŋua? Mina míasrɔ̃ nu tso Shwachman-Diamond ƒe dɔléle (SDS) ŋu .

Ðe viwò suea le dɔ lém ɣesiaɣia? Menye lolo dzi ɖeɖe kpɔtɔ oa? Alo ɖe nèle tɔtrɔ aɖewo kpɔm le woƒe ƒuwo ŋua? Ɣeaɖewoɣi la, esia ate ŋu atso Shwachman-Diamond syndrome (SDS), si nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o si dzea ɖeviwo dzi gbɔ. Mina míaƒo nu tso esia ŋu le mɔ bɔbɔe aɖe si gɔme nàte ŋu ase nu.

Nukae nye Shwachman-Diamond ƒe dɔléle (SDS)?

Kpuie ko la, Shwachman-Diamond syndrome (SDS) nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o si gblẽa nu le ɖeviwo ƒe dɔgbo, ƒumeŋuɖui, kple ƒuwo ŋu koŋ. Zi geɖe la, wokpɔnɛ dzea sii hafi ɖevi xɔ ƒe ɖeka. Ke hã, woate ŋu akpɔe le sɔhɛ tsitsiwo ŋu ɣeaɖewoɣi.

SDS nye dɔléle sesẽ aɖe si woate ŋu akpɔ ahadae elabena ate ŋu ahe dzesi vovovowo vɛ. Dzesi siawo dometɔ ɖeka, geɖe, alo wo katã ate ŋu anɔ ɖevi aɖe ŋu. Le kpɔɖeŋu me, dɔgbo kple ƒuwo ŋu ate ŋu anɔ ɖevi ɖeka ŋu, evɔ ƒumeŋuɖui kple ƒuwo koe kuxiwo anɔ ɖevi bubu ŋu.

Enye dɔléle si womate ŋu agblɔ ɖi kura o hã . Ðeviwo ƒe dzesiwo ate ŋu anɔ bɔbɔe alo nu sesẽ. Dzesi siawo ate ŋu atrɔ le ɣeyiɣi aɖe megbe. Esi wònye be atikewɔwɔ me kuxi vovovowo le ɖevi siwo ŋu SDS le ŋu ta la, ɖevi geɖe hiã eŋutinunyalawo ƒe ƒuƒoƒo aɖe ƒe kpekpeɖeŋu . Togbɔ be ɖevi siwo ŋu dɔléle sia le ahiã be woakpɔ egbɔ le woƒe agbemeŋkekewo katã me hã la, zi geɖe la, woate ŋu anɔ agbe si sɔ. Gake ɖevi kple sɔhɛ tsitsi aɖewo ate ŋu axɔ ʋumenugbagbeviɣidɔ si ate ŋu ade woƒe agbe afɔku me (Acute Myeloid Leukemia) alo ʋumenugbagbeviɣidɔ sesẽ aɖe (Myelodysplasia).

Ðe esia nye nɔnɔme si bɔa?

Esesẽ be míagblɔe kple kakaɖedzi. Ðɔktawo bua Shwachman-Diamond ƒe dɔlélea be enye dɔléle si mebɔ o. Gake akɔntabubu siwo me kɔ koe li ku ɖe ɖevi agbɔsɔsɔme si ŋu dɔlélea le ŋu. Akɔntabubu aɖewo gblɔ be nɔnɔmea nye vidzidzi 75,000 ɖesiaɖe dometɔ ɖeka . Nusita wobu akɔnta sia si me kɔe nye be dzesi siwo nu mesẽ o alo esiwo nu sẽ ate ŋu anɔ ɖeviwo ŋu, menye ɖeviwo katã ƒe dzesiwo sɔ o, eye dodokpɔ ɖeka aɖeke meli si ŋu kakaɖedzi le si woatsɔ anya dɔlélea o.

Ðe ame tsitsiwo hã xɔa Shwachman-Diamond ƒe dɔléle (SDS)a?

To vovo na ɖevimenɔɣi ƒe atikewɔwɔ me kuxi aɖewo la, Shwachman-Diamond ƒe dɔlélea nu meyina ne ɖeviwo le tsitsim o. Dzesiwo kple atikewɔwɔ ate ŋu atrɔ le ɣeyiɣi aɖe megbe, gake ɖevi siwo ŋu dɔléle sia le ahiã be woakpɔ egbɔ le woƒe agbemeŋkekewo katã me .

Aleke nɔnɔme sia kpɔa ŋusẽ ɖe vinyea ƒe ŋutilã dzii?

Shwachman-Diamond ƒe dɔlélea ate ŋu ahe atikewɔwɔ me kuxi geɖe vɛ, gake nu vevi etɔ̃e wògblẽna le ame ŋu:

1. Exocrine dɔgbo ƒe dɔmawɔmawɔ nyuie:Viwò ƒe dɔgbo nye ŋutinu aɖe si le dɔgboa megbe. Lãmenugbagbevi ƒomevi aɖe si woyɔna be acinar cells le eme. Lãmenugbagbevi siawo wɔa enzyme siwo kpena ɖe nuɖuɖu ŋu wòɖua nu. Enzyme siawo gbãa nunyiame kple ami siwo le nuɖuɖu me ale be ŋutilãa nate ŋu axɔ wo. Le ɖevi siwo ŋu SDS le gome la, dɔgboa mewɔa enzyme siawo wòsɔa gbɔ o. Esia wɔe be ɖevia mekpɔa nunyiame siwo wòhiã o.

2. Ƒumeŋuɖui ƒe dɔmawɔmawɔ nyuie: Viwò ƒe ƒumeŋuɖui wɔa ʋumenugbagbevi dzĩwo, ʋumenugbagbevi ɣiwo, kple ʋumenugbagbevi dzĩwo. Le ɖevi siwo ŋu SDS le gome la, ƒumeŋuɖui mewɔa lãmenugbagbevi siawo dometɔ ʋɛ aɖewo wu alesi wòle dzɔdzɔmee. Vevietɔ la, mewɔa ʋumenugbagbevi ɣi ƒomevi aɖe si woyɔna be neutrophils wòde edeƒe o. Neutrophils nye lãmenugbagbevi siwo kpɔa ŋutilã ta le dzɔdzɔme nu tso amedzidzelawo abe dɔlékuiwo ene si me. Neutrophils mesɔ gbɔ ɖe ɖevi aɖewo siwo ŋu SDS le si be woatsɔ awɔ avu kple dɔlékui siawo siwo va dzea wo dzi o. Woyɔa nɔnɔme sia be neutropenia . Zi geɖe la, ɖevi siwo ŋu neutropenia le la xɔa dɔlékuiwo abe aklãdɔ, titinato ƒe dɔlékuiwo, alo ŋutigbalẽdɔlélewo ene.

3. Ƒumeŋuɖui ƒe dɔmawɔmawɔ nyuie: Nɔnɔme aɖewo ate ŋu anɔ ɖevi siwo ŋu SDS le ŋu abe scoliosis, ƒuwo ƒe kpuiƒe si mesɔ o le abɔwo kple afɔwo me (chondrodysplasia), alo akɔta si le gbadzaa si le abe gaƒoɖokui ene (thoracic dystrophy) si mesɔ o.

Kuxi kawoe nye Shwachman-Diamond ƒe dɔléle (SDS) ƒe kuxiwo?

Amesiwo ŋu dɔléle sia le la le afɔku me be ʋumenugbagbevi siwo mesɔ o nadze wo dzi (`(myelodysplasia)`) . Esiawo ate ŋu ava zu ʋumenugbagbeviɣidɔ si woyɔna be `(acute myeloid leukemia)` emegbe.

Nukae nye Shwachman-Diamond ƒe dɔléle (SDS) ƒe dzesiwo?

Nɔnɔme sia ate ŋu adze ɖevia ƒe ŋutilã ƒe akpa vovovowo dzi. Gake dzesi siwo bɔ wue nye dɔgbo, ƒumeŋuɖui, kple ƒumeŋuɖui. Ame aɖewo ate ŋu akpɔ dzesiwo le wo dziɣi, le woƒe vidzĩme, alo le woƒe ɖevime. Ame ʋɛ aɖewo ate ŋu akpɔ dzesiawo le woƒe sɔhɛmenɔɣi.

Dzesi siwo bɔ:

• Viwòa ƒe kpododonu: Esia fia be viwòa mele lolom o. Le SDS gome la, esia ate ŋu atso nuɖuɖumeŋusẽ ƒe dɔmawɔmawɔ nyuie gbɔ.
• Ðeɖiteameŋu: Vidzĩ si ŋu ɖeɖi te ate ŋu ado dziku eye wòagbɔdzɔ.
• Aɖuɖɔ gã siwo me ami le, siwo ʋẽna lilili: Viwòa ƒe aɖuɖɔ ate ŋu alolo le mɔ si mebɔ o nu, ami le eme le dzedzeme nu, eye wòaʋẽ lilili.
• Dɔlékui sesẽwo xɔxɔ enuenu: Ne dɔlékuiwo ƒe dɔlélewo yi edzi le dzɔdzɔm la, ate ŋu anye SDS ƒe dzesi.
• Tɔtrɔ siwo wokpɔna le abɔwo kple afɔwo ƒe ƒuwo me: Vidzĩwo ƒe abɔwo kple afɔwo ate ŋu anɔ kpuie wu ne wotsɔe sɔ kple woƒe akɔta.

Nukae hea Shwachman-Diamond ƒe dɔléle (SDS) vɛ?

Ðevi siwo ŋu SDS le ƒe 90% lɔƒo ƒe domenyiŋusẽfianu ƒe tɔtrɔ le `SBDS` domenyiŋusẽfianu me . Numekukuwo ɖee fia be woate ŋu anyi tɔtrɔ sia ƒe dome tso dzila evea siaa gbɔ (`(autosomal recessive manner)`) alo tso dzila ɖeka gbɔ eye tɔtrɔ yeye aɖe dzɔna. Numekulawo menya nusita tututu domenyiŋusẽfianu ƒe tɔtrɔ le `SBDS` domenyiŋusẽfianu me hea SDS vɛ haɖe o.

Aleke ɖɔktawo wɔa dea dzesi dɔléle siae?

Ðɔktawo awɔ ŋutilãmedɔdada atsɔ akpɔ viwòa ƒe lãmesẽ bliboa. Woadzidze viwòa ƒe kɔkɔme kple eƒe kpekpeme eye woatsɔe asɔ kple ɖevi bubu siwo nye woƒe ƒemetɔwo ƒe tsitsi. Woate ŋu awɔ dodokpɔ siwo gbɔna hã:

• Ʋu ƒe xexlẽme blibo (CBC) kple vovototo: Esia xlẽa ɖevia ƒe ʋumenugbagbeviwo, vevietɔ ʋumenugbagbevi ɣiwo katã.
• Dɔgboa ƒe dɔwɔwɔ dodokpɔ: Ðɔktawo ate ŋu ada viwòa ƒe aɖuɖɔ me kpɔ alo awɔ nɔnɔmetatawo dodokpɔ abe kɔmpiuta dzi tomenukuku (CT) ene.
• Ʋudodokpɔ be woatsɔ akpɔ vitamin ƒe agbɔsɔsɔ.
• X-ray: Woate ŋu awɔ X-ray atsɔ akpɔe ɖa be ƒu ƒe kuxiwo le eŋu hã, vevietɔ le ɖevia ƒe akɔta alo afɔwo me.
• Domenyiŋusẽfianu dodokpɔ: Be ɖɔktawo nade dzesi domenyiŋusẽfianu ƒe tɔtrɔ siwo hea SDS vɛ la, wodzroa ɖevia ƒe ʋu, ŋutigbalẽ, taɖa, alo lãkusi ƒe kpɔɖeŋuwo me tsɔ ɖoa ​​kpe edzi nenye be dɔlélea le ɖevia ŋu.

Aleke wodaa nɔnɔme siae?

Shwachman-Diamond ƒe dɔlélea ate ŋu agblẽ nu le viwò ŋu le mɔ geɖe nu. Le kpɔɖeŋu me, ɖewohĩ ɖevi mate ŋu aɖu nuɖuɖu o le kuxi siwo le dɔgboa ŋu ta, gake woƒe ƒumeŋuɖui ate ŋu awɔ dɔ nyuie. Dzesiawo ate ŋu anɔ bɔbɔe alo nu sesẽ. Ðɔktawo nɔa te ɖe nɔnɔmea ƒe sesẽ dzi daa tɔtrɔ siawo.

Le dɔgboa ƒe exocrine ƒe dɔmawɔmawɔ nyuie gome la:

Ðɔktawo ate ŋu aŋlɔ dɔdzẽ me dɔlékui siwo le nu me alo vitamin siwo me ami le be wòakpe ɖe viwò ƒe ŋutilã ŋu wòaxɔ nunyiame kple ami.

Le ƒumeŋuɖui ƒe dɔmawɔmawɔ nyuie gome la:

Esi nɔnɔme sia gblẽa nu le ɖevia ƒe ƒumeŋuɖui ŋu ta la, ƒumeŋuɖui mewɔa neutrophils wòsɔ gbɔ o. Zi geɖe la, ɖɔktawo medaa ƒumeŋuɖui ƒe dɔlélewo o negbe ɖe kuxi sesẽwo le ɖevia ŋu hafi. Atikewɔwɔ ate ŋu anye:

• Ʋudodo: Enaa ʋumenugbagbeviwo ƒe agbɔsɔsɔ dzina ɖe edzi.
• Ʋumenugbagbeviɣidɔ: Enaa ʋumenugbagbevi dzĩwo ƒe agbɔsɔsɔ dzina ɖe edzi be wòakpe ɖe ʋu ƒe dodo ƒe kuxiwo ŋu.
• Granulocyte-colony stimulating factor (G-CSF) : Atikewɔwɔ sia nana neutrophils ƒe xexlẽme dzina ɖe edzi le ɖevia ƒe ʋumenugbagbevi ɣiwo me.
• Lãmenugbagbeviwo dodo na ame: Ame aɖewo siwo ŋu SDS le la xɔa ʋumekansa alo ʋumenugbagbeviɣidɔ sesẽwo. Ðɔktawo ate ŋu ada nɔnɔme siawo to lãmenugbagbeviwo dodo na ame me.

Le ƒunukpeƒewo ƒe dɔmawɔmawɔ nyuie gome la:

Zi geɖe la, ɖɔktawo léa ŋku ɖe ƒumeŋuɖui siwo SDS hena vɛ ŋu. Ðewohĩ ahiã be woawɔ dɔ na ɖevi aɖewo ne kuxi sesẽwo le wo ŋu.

Amekawoe nye atikewɔla bibi siwo daa dɔléle sia?

Wohiã eŋutinunyalawo ƒe ƒuƒoƒo aɖe be woada Shwachman-Diamond ƒe dɔlélea. Viwòa ƒe atikewɔwɔ ƒe ƒuƒoƒoa ate ŋu anye:

• Ðeviwo ƒe dɔdalawo: Ðɔkta siawo daa dɔ na vidzĩ yeyewo, ɖeviwo, kple sɔhɛ tsitsiwo. Anɔ eme be viwòa ƒe ɖeviwo ƒe dɔdala aɖo aɖaŋu be woawɔ dodokpɔwo atsɔ akpɔe ɖa be SDS le eme hã.
• Dɔlékuiŋutinunyalawo: Ame siawo nye amesiwo bi ɖe lãmenugbagbeviwo ƒe dɔwɔwɔ, si kpɔa ŋusẽ ɖe ɖevi ƒe tsitsi dzi la ŋu.
• Ʋuŋutinunyalawo: Ame siawo nye amesiwo bi ɖe ʋumenugbagbeviɣidɔ, siwo dome dɔléle siwo gblẽa nu le ɖevi ƒe ʋumenugbagbeviwo ŋu hã le.
• Dɔgboŋutinunyalawo: Ðɔkta siawo ate ŋu akpe ɖe viwòa ƒe nuɖuɖumeŋusẽ ƒe kuxiwo dada ŋu.
• Domenyiŋusẽfianu: Shwachman-Diamond ƒe dɔlélea nye domenyiŋusẽfianu ƒe dɔléle. Ðɔkta siawo, alo domenyiŋusẽfianu ŋuti aɖaŋuɖolawo, awɔ ɖoɖo ɖe domenyiŋusẽfianu dodokpɔ ŋu atsɔ aɖo kpe viwòa ƒe nɔnɔmea dzi. Woate ŋu ado wò kple viwòa ƒe ʋuƒometɔ aɖewo hã kpɔ.
• Ƒumeŋutinunyalawo: Ne ƒumekuxiwo le viwòa ŋu si hiã be woawɔ dɔ na wò la, ahiã be nàyi ƒumeŋutinunyala gbɔ.

Ðe mate ŋu axe mɔ ɖe esia nua?

Shwachman-Diamond ƒe dɔlélea nye dɔléle si ƒe dome wonyi . Ne ènya be dɔlélea le ye ŋu la, anyo be nàƒo nu kple domenyiŋusẽfianu ŋuti nunyala. Ne vi siwo ŋu dɔlélea le le asiwò la, àte ŋu abu domenyiŋusẽfianu dodokpɔ be yeakpɔe ɖa be domenyiŋusẽfianu ƒe tɔtrɔ si hea SDS vɛ la le viwòwo ŋu hã.

Ðe woate ŋu ada Shwachman-Diamond ƒe dɔlélea (SDS) keŋkeŋa?

Ðɔktawo mate ŋu ada dɔléle sia keŋkeŋ o. Ne nɔnɔme sia le viwòa ŋu la, ahiã be woakpɔ egbɔ le eƒe agbemeŋkekewo katã me.

Aleke wòle be woanɔ agbe kple Shwachman-Diamond ƒe dɔléle (SDS)?

Le mɔ aɖe nu la, Shwachman-Diamond ƒe dɔlélea nye dɔléle si nɔa anyi didi . Ne nɔnɔme sia le viwòa ŋu la, ahiã be woadoe kpɔ ahadoe kpɔ edziedzi.

• Ʋu ƒe xexlẽme blibo (CBC) kple ʋumenugbagbevi ɣiwo ƒe xexlẽme, ʋumenugbagbevi dzĩwo ƒe xexlẽme: Ðɔktawo ate ŋu awɔ dodokpɔ siawo ɣleti etɔ̃ va ɖo ade ɖesiaɖe.
• Ƒumeŋuɖui dodokpɔ: Ʋumenugbagbeviŋutinunyalawo ate ŋu awɔ dodokpɔ siawo zi ɖeka le ƒea me va ɖo zi ɖeka le ƒe etɔ̃ ɖesiaɖe me.
• Ʋudodokpɔ be vitaminwo le eme: Ðɔktawo ate ŋu adzidze vitamin agbɔsɔsɔme si le ɖevi ƒe ʋu me akpɔe ɖa be dɔgboa me dɔlékuiwutikewo le dɔ wɔm hã.
• Ƒu ƒe kpekpemedzidzenu: Ðɔktawo ate ŋu ado ƒu ƒe kpekpeme kpɔ do ŋgɔ na sɔhɛmenɔɣi kple le eƒe sɔhɛmenɔɣi.
• X-ray: Woate ŋu awɔ X-ray atsɔ akpɔe ɖa be kuxi aɖewo le viwòa ƒe akɔta kple klo ŋu hã, vevietɔ le ɣeyiɣi siwo me wòle tsitsim kabakaba me.
• Tsitsi ƒe dodokpɔ: Ðevi aɖewo siwo ŋu SDS le ate ŋu akpɔ tsitsimekuxiwo, abe susu ƒe dɔmawɔmawɔ nyuie (ADD) ene. Ðɔktawo ate ŋu ada tsitsi kpɔ ɣleti ade ɖesiaɖe tso edziɣi vaseɖe ƒe 6 xɔxɔ, kple tsitsi ɣleti ade ɖesiaɖe.
• Ahɔhɔ̃mekawo ƒe dɔwɔwɔ dodokpɔ kple eƒe dodokpɔ: SDS ate ŋu ahe nɔnɔmewo abe susu ƒe dɔmawɔmawɔ nyuie (ADD) alo tsitsi ƒe dɔléle si bɔ (PDD) vɛ. Ðɔktawo ate ŋu aɖo aɖaŋu be woawɔ dodokpɔ edziedzi le ƒe 6-8, 11-13, kple 15-17 xɔxɔ me.

Aleke mate ŋu akpe ɖe vinyea ŋu wòanɔ te ɖe nɔnɔme sia nu?

Atikewɔwɔ me kuxi vovovowo ate ŋu anɔ ɖevi siwo ŋu dɔléle sia le ŋu. Ðewohĩ ahiã be woawɔ atike na wo be wòakpe ɖe wo ŋu woaxɔ nunyiame kple ami. Anɔ bɔbɔe be woaxɔ dɔlékuiwo. Ate ŋu adzɔ hã be ƒu ƒe dɔmawɔmawɔ nyuie le wo ŋu si nana woƒe dzedzeme to vovo na ame bubuwo.

Dzesi ka kee ɖale eŋu o, ɖevi siwo ŋu nɔnɔme sia le ate ŋu atsi dzi ɖe nu ɖeka ŋu – be yewoto vovo tso ame bubuwo gbɔ . Ðewohĩ ahiã be woawɔ atike na wo si ana woate ɖa le suku kple dɔwɔna bubuwo ŋu. Woƒe dzedzeme ate ŋu atrɔ. Ne viwòa le tsitsim la, tɔtrɔ siawo ate ŋu ana wòado dziku ahatsi dzimaɖi. Seselelãme siawo gɔmesese ate ŋu akpe ɖe viwòa ŋu wòaɖu eƒe seselelãmewo dzi. Ðevi aɖewo ate ŋu aɖe vi ne woƒo nu kple tagbɔdɔdala aɖe. Ne ètsi dzi ɖe alesi viwòa le te ɖe woƒe nɔnɔmea nu ŋu la, bia wò ɖɔkta be wòaɖo aɖaŋu na wò.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne Shwachman-Diamond ƒe dɔlélea le viwò ŋu la, ele be nàdze agbagba anya tɔtrɔ siwo ava viwòa ƒe ŋutilã me . Zi geɖe la, SDS ƒe dzesiwo trɔna le ɣeyiɣi aɖe megbe. Le go aɖewo me la, tɔtrɔ siawo ate ŋu anye kuxi sesẽwo ƒe dzesiwo, siwo dome ʋumenugbagbeviɣidɔ hã le.

Ðeviwo ƒe ŋutilã le tɔtrɔm ɣesiaɣi ne wole vidzĩme, ɖevimenɔɣi, ƒewuimenɔɣi (vevietɔ ƒewuiwo kple sɔhɛmenɔɣi), kple sɔhɛmenɔɣi me tom. Menye ɣesiaɣie tɔtrɔ siawo nyea dɔléle yeye alo nɔnɔme si nu sẽ wu ƒe dzesi o.

Viwòa yia ɖɔkta gbɔ edziedzi.Ɣeyiɣi mawo siwo wowɔna edziedzi lae nye ɣeyiɣi nyuitɔ si me nàbia nyawo tso tɔtrɔ siwo ate ŋu anye kuxi sesẽ aɖe ƒe dzesiwo ŋu.

Nya kawoe wòle be mabia nye ɖɔkta?

Shwachman-Diamond ƒe dɔlélea nye dɔléle si mebɔ o. Ðewohĩ mànya gɔ̃ hã be ele ye si o. Nya aɖewo siwo nàte ŋu abia wò ɖɔktae nye esi:

• Nukatae dɔléle sia le vinye ŋu?
• Ðe dɔléle sia ƒe nɔnɔme fafɛ aɖe le eŋu loo alo ɖe dɔléle sesẽ aɖe le eŋua?
• Aleke nɔnɔme sia kpɔa ŋusẽ ɖe vinye dzii?
• Nukawoe nye atikewɔmɔnuawo?
• Ðe vinyea ƒe dzesiwo agagblẽ ɖe edzia?
• Ðe wòhiã be woado vinye nɔvi bubuwo ƒe domenyiŋusẽfianu kpɔa?
• Ðe wòhiã be mía kple ɖevia dzila ŋutɔŋutɔ bubua míado domenyiŋusẽfianu kpɔa?
• Aleke mate ŋu akpe ɖe vinyea ŋu wòakpɔ atikewɔwɔ dzi?

Mlɔeba la, nu vevitɔ kekeake (Take-Home Message) .

Shwachman-Diamond ƒe dɔlélea nye dɔléle si mebɔ o, si ƒe dome wonyi, si gblẽa nu le ɖeviwo ƒe tsitsi, dɔlékuiwo ƒe dɔléle xɔxɔ bɔbɔe, kple ƒu ƒe dɔmawɔmawɔ nyuie ŋu. Togbɔ be ɖevi aɖewo ƒe dzesiwo nu mesẽna o hã la, ɖevi siwo katã ŋu dɔlélea le la ahiã be woakpɔ egbɔ le woƒe agbemeŋkekewo katã me. Ne Shwachman-Diamond ƒe dɔlélea le viwò ŋu la, ɖewohĩ kakaɖedzimanɔamesi le nusi ava dzɔ le etsɔme ŋu la agbɔ eme. Ne èle nɔnɔme sia me la, gblɔ wò dzimaɖitsitsiwo na viwòa ƒe ɖɔktawo. Wosea alesi wòle be woatsi dzi ɖe ɖevi si ŋu dɔléle sesẽ aɖe si ŋu womele mɔ kpɔm na o le ɣeaɖewoɣi la ŋu ahalé be nɛ gɔme. Menye ɖeko woate ŋu akpe ɖe viwòa ŋu wòanɔ te ɖe dɔlélea nu ko o, ke woate ŋu akpe ɖe eŋu hã be wòanɔ agbe nyuie. Eye edzɔa dzi na wo be yewoakpe ɖe ŋuwò nàkpe ɖe viwòa ŋu. Mègase le ɖokuiwò me gbeɖe be yetsi akogo o, eye nàbia kpekpeɖeŋu.

` Shwachman-Diamond syndrome, SDS, domenyiŋusẽfianu ƒe dɔmawɔmawɔ nyuie, dɔgbo, ƒumeŋuɖui, ƒu ƒe dɔmawɔmawɔ nyuie, ɖeviwo ƒe dɔlélewo, neutropenia