I-Hyperimmunoglobulin E syndrome

I-Hyperimmunoglobulin E syndrome isifo esingajwayelekile, esizuzwe njengefa. Kubangela izinkinga esikhunjeni, esonweni, emaphashini, emathanjeni, nasemazinyweni.

I-Hyperimmunoglobulin E syndrome ibizwa nangokuthi i-Job syndrome. Kubizwa ngegama lomlingiswa waseBhayibhelini uJobe, ukuthembeka kwakhe kwavivinywa yisifo sokukhipha izilonda zesikhumba nama-pustule. Abantu abanalesi sifo banezifo zesikhumba zesikhathi eside, ezinzima.

Izimpawu zivame ukubakhona ebuntwaneni, kepha ngoba lesi sifo asivamile, kuvame ukuthatha iminyaka ngaphambi kokuba kutholakale ukuxilongwa okufanele.

Ucwaningo lwakamuva luphakamisa ukuthi lesi sifo kuvame ukubangelwa ukuguqulwa kofuzo (okwenzeka) STAT3zofuzo ku-chromosome 17. Ukuthi lokhu kufihlwa kofuzo kubangela kanjani izimpawu zesifo akuqondakali kahle. Kodwa-ke, abantu abanalesi sifo banezinga eliphakeme kakhulu kunokujwayelekile le-antibody ebizwa nge-IgE.

Izimpawu zifaka:

• Ukukhubazeka kwamathambo namazinyo, kufaka phakathi ukuphuka nokulahlekelwa amazinyo engane sekwephuzile
• I-Eczema
• Amathumba esikhumba nokutheleleka
• Ukutheleleka okuphindaphindiwe kwesono
• Ukutheleleka okuphindaphindiwe kwamaphaphu

Ukuhlolwa ngokomzimba kungakhombisa:

• Ukugoba komgogodla (kyphoscoliosis)
• I-Osteomyelitis
• Phinda ukutheleleka kwesono

Ukuhlolwa okusetshenziselwa ukuqinisekisa ukuxilongwa kufaka:

• Isibalo se-eosinophil ngokuphelele
• I-CBC ngokuhluka kwegazi
• I-Serum globulin electrophoresis ukubheka izinga eliphezulu legazi le-IgE
• Ukuhlolwa kofuzo STAT3 isakhi sofuzo

Ukuhlolwa kwamehlo kungaveza izimpawu zesifo esomile.

I-x-ray yesifuba ingaveza amathumba emaphaphu.

Olunye uvivinyo olungenziwa:

• I-CT scan yesifuba
• Amasiko esiza esinegciwane
• Ukuhlolwa kwegazi okukhethekile ukuhlola izingxenye zomzimba wokuzivikela ezifweni
• I-X-ray yamathambo
• Ukuhlolwa kwe-CT kwezono

Uhlelo lokufaka amagoli oluhlanganisa izinkinga ezahlukahlukene ze-Hyper IgE syndrome lungasetshenziswa ukusiza ukuxilongwa.

Alikho ikhambi elaziwayo lalesi simo. Inhloso yokwelashwa ukulawula ukutheleleka. Imithi ifaka:

• Imithi elwa namagciwane
• Imithi elwa namagciwane nemishanguzo (uma kufanele)

Ukuhlinzwa kwesinye isikhathi kuyadingeka ukukhipha amathumba.

IGamma globulin enikezwa ngomthambo (IV) ingasiza ekwakheni amasosha omzimba uma unezifo ezinzima.

I-Hyper IgE syndrome yisimo esingelapheki sempilo yonke. Ukutheleleka okusha ngakunye kudinga ukwelashwa.

Izinkinga zingafaka:

• Izifo eziphindaphindiwe
• I-Sepsis

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma unezimpawu ze-Hyper IgE syndrome.

Ayikho indlela efakazelwe yokuvimbela i-Hyper IgE syndrome. Inhlanzeko ejwayelekile iyasiza ekuvikeleni ukutheleleka kwesikhumba.

Abanye abahlinzeki bancoma ama-antibiotic okuvimbela abantu abahlakulela izifo eziningi, ikakhulukazi nge- I-Staphylococcus aureus. Le ndlela yokwelashwa ayishintshi isimo, kepha inganciphisa izinkinga zayo.

Isifo seJobe; Isifo se-Hyper IgE

I-Chong H, i-Green T, i-Larkin A. Ukungezwani komzimba kanye ne-immunology. Ku: Zitelli BJ, McIntire SC, Nowalk AJ, ama-eds. UZitelli noDavis ’Atlas of Pediatric Physical Diagnosis. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 4.

IHolland SM, uGallin JI. Ukuhlolwa kwesiguli esinokusolwa kwe-immunodeficiency. Ku: Bennett JE, Dolin R, Blaser MJ, ama-eds. Mandell, Douglas, kanye neBennett's Principles and Practice of Infectious Diseases. Umhlaka 9. IPhiladelphia, PA: Elsevier; 2020: isahluko 12.

IHsu AP, uDavis J, uPuck JM, uHolland SM, uFreeman AF. I-Autosomal ephezulu ye-hyper IgE syndrome. Ukubuyekezwa kwe-Gene. 2012; 6. I-PMID: 20301786 www.ncbi.nlm.nih.gov/pubmed/20301786. Kubuyekezwe ngoJuni 7, 2012. Kufinyelelwe ngoJulayi 30, 2019.