Isifo seHyperimmunoglobulin E syndrome

Isifo sehyperimmunoglobulin E sisifo esinqabileyo, esizuzwe njengelifa. Kubangela iingxaki kulusu, ezonweni, emiphungeni, emathanjeni, nasemazinyweni.

I-Hyperimmunoglobulin E syndrome ibizwa ngokuba yiJob syndrome. Ithiywa ngegama lomntu waseYobhi, othembekileyo wavavanywa kukubandezeleka kokukhupha izilonda kunye neepustules. Abantu abakule meko banexesha elide, ulosuleleko olunzima lolusu.

Iimpawu zihlala zikhona ebuntwaneni, kodwa ngenxa yokuba esi sifo sinqabile, amaxesha amaninzi kuthatha iminyaka ngaphambi kokuba kufunyaniswe isifo.

Uphando lwakutsha nje lubonisa ukuba esi sifo sidla ngokubangelwa lutshintsho olwenzeka kwimfuza STAT3Imfuza kwi-chromosome 17. Ayisiqondakali kakuhle le nto yokungaqheleki kofuzo. Nangona kunjalo, abantu abanesi sifo banenqanaba eliphezulu kuneliqhelekileyo le-antibody ebizwa ngokuba yi-IgE.

Iimpawu zibandakanya:

• Iziphene zamathambo kunye namazinyo, kubandakanya ukwaphuka kunye nokuphulukana namazinyo abantwana kungekudala
• Onokhwekhwe
• Amathumba elusu kunye nosulelo
• Ukuphindaphindeka kosulelo lwesono
• Ukuphindaphindeka kosulelo lwemiphunga

Uvavanyo lomzimba lunokubonisa:

• Ukujika komqolo (kyphoscoliosis)
• Osteomyelitis
• Phinda usulelo lwesono

Uvavanyo olusetyenziselwa ukuqinisekisa ukuxilongwa lubandakanya:

• Ubungakanani be-eosinophil count
• I-CBC ngokwahluka kwegazi
• I-serum globulin electrophoresis ukukhangela inqanaba eliphezulu legazi le-IgE
• Ukuvavanywa kwemvelo STAT3 Uhlobo

Uvavanyo lwamehlo lunokubonisa iimpawu zesifo esomileyo wamehlo.

IX-ray yesifuba inokuveza amathumba emiphunga.

Olunye uvavanyo olunokwenziwa:

• Ukuxilongwa kwe-CT kwesifuba
• Iinkcubeko zesiza esosulelekileyo
• Uvavanyo lwegazi olukhethekileyo ukujonga amalungu omzimba
• X-reyi yamathambo
• Ukuvavanywa kwe-CT kwezono

Inkqubo yokufumana amanqaku edibanisa iingxaki ezahlukeneyo zeHyper IgE syndrome inokusetyenziselwa ukunceda ekuchongeni isifo.

Akukho lunyango lwaziwayo lwale meko. Injongo yonyango kukulawula izifo. Amayeza afaka:

• Amayeza okubulala iintsholongwane
• Amayeza okubola nokubulala iintsholongwane (xa kufanelekile)

Utyando ngamanye amaxesha luyafuneka ukukhupha amathumba.

I-Gamma globulin enikezelwa ngomthambo (IV) inokunceda ekwakheni amajoni omzimba ukuba unesifo esibi.

Isifo seHyper IgE sisifo esingapheliyo. Usulelo ngalunye olutsha lufuna unyango.

Iingxaki zinokubandakanya:

• Izifo eziphindaphindiweyo
• Sepsis

Fowunela umboneleli wakho wezempilo ukuba uneempawu zehyper IgE syndrome.

Akukho ndlela iqinisekisiweyo yokuthintela isifo se-Hyper IgE. Ukucoceka ngokubanzi kuluncedo ekuthinteleni usulelo lolusu.

Abanye ababoneleli banokucebisa ngee-antibiotics ezithintelayo kubantu abaphuhlisa izifo ezininzi, ngakumbi nge IStaphylococcus aureus. Olu nyango aluyitshintshi imeko, kodwa lunokunciphisa iingxaki zalo.

Isifo seJob; Isifo sehyper IgE

I-Chong H, Green T, Larkin A. Allergy kunye ne-immunology. Ku: Zitelli BJ, McIntire SC, Nowalk AJ, ii-eds. UZitelli no-Davis ’Atlas of Pediatric Physical Diagnosis. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 4.

IHolland SM, uGallin JI. Uvavanyo lwesigulana esirhanelwa kukungabikho komzimba. Ku: Bennett JE, Dolin R, Blaser MJ, ii-eds. Mandell, Douglas, kunye neBennett's Principles and Practice of Infectious Diseases. Umhla we-9. IPhiladelphia, PA: Elsevier; 2020: isahl 12.

IHsu AP, uDavis J, uPuck JM, uHolland SM, uFreeman AF. I-Autosomal ephezulu ye-hyper IgE syndrome. Uphengululo lweGene. 2012; 6. IINKCUKACHA: 20301786 www.ncbi.nlm.nih.gov/pubmed/20301786. Ukuhlaziywa ngoJuni 7, 2012. Ifikeleleke ngoJulayi 30, 2019.