Matenda a Hyperimmunoglobulin E

Matenda a Hyperimmunoglobulin E ndi osowa, obadwa nawo. Zimayambitsa mavuto pakhungu, matupi, mapapo, mafupa, ndi mano.

Matenda a Hyperimmunoglobulin E amatchedwanso matenda a Job. Amatchulidwa ndi dzina la Yobu, yemwe kukhulupirika kwake kunayesedwa ndi vuto lokhala ndi zilonda pakhungu ndi pustules. Anthu omwe ali ndi vutoli amakhala ndi matendawa pakhungu lawo kwanthawi yayitali.

Zizindikiro zimapezeka nthawi zambiri ali mwana, koma chifukwa matendawa ndi osowa, nthawi zambiri zimatenga zaka asanadziwe kuti ali ndi vuto.

Kafukufuku waposachedwa akuwonetsa kuti matendawa amayamba chifukwa cha kusintha kwa majini (kusintha) komwe kumachitika mu STAT3jini pa chromosome 17. Momwe kusalongosoka kwa jini kumayambitsa zizindikiro za matenda sikumveka bwino. Komabe, anthu omwe ali ndi matendawa ali ndi mulingo wopambana kuposa wabwinobwino wa mankhwala otchedwa IgE.

Zizindikiro zake ndi izi:

• Kupunduka kwa mafupa ndi mano, kuphatikizapo kusweka ndi kutaya mano a mwana mochedwa
• Chikanga
• Ziphuphu zakhungu ndi matenda
• Matenda a sinus obwerezabwereza
• Matenda am'mapapo obwereza

Kuyezetsa thupi kumatha kuwonetsa:

• Kupindika kwa msana (kyphoscoliosis)
• Osteomyelitis
• Bwerezani matenda a sinus

Mayeso omwe amagwiritsidwa ntchito kutsimikizira kuti ali ndi matendawa ndi awa:

• Kuwerengera kwathunthu kwa eosinophil
• CBC yosiyana ndi magazi
• Serum globulin electrophoresis kuyang'ana mulingo wamagazi wa IgE
• Kuyesedwa kwachibadwa kwa STAT3 jini

Kuyesedwa kwa diso kumatha kuwonetsa zizindikiritso zamatenda owuma.

X-ray pachifuwa imatha kuwulula zotupa m'mapapu.

Mayesero ena omwe angachitike:

• Kujambula kwa CT pachifuwa
• Chikhalidwe cha tsamba lomwe lili ndi kachilomboka
• Kuyezetsa magazi mwapadera kuti muwone momwe chitetezo chamthupi chilili
• X-ray ya mafupa
• CT scan ya sinus

Njira yolembera yomwe imaphatikiza zovuta zosiyanasiyana za Hyper IgE syndrome itha kugwiritsidwa ntchito kuthandizira kupeza matendawa.

Palibe mankhwala odziwika a matendawa. Cholinga cha chithandizo ndikuchepetsa matendawa. Mankhwala ndi awa:

• Maantibayotiki
• Maantifungal ndi ma virus (ngati kuli koyenera)

Nthawi zina opaleshoni imafunika kuti atulutse zithupsa.

Gamma globulin yoperekedwa kudzera mumtsempha (IV) itha kuthandiza kumanga chitetezo cha mthupi ngati muli ndi matenda akulu.

Matenda a Hyper IgE ndiwanthawi yayitali. Matenda atsopano aliwonse amafunikira chithandizo.

Zovuta zingaphatikizepo:

• Matenda obwerezabwereza
• Sepsis

Itanani omwe akukuthandizani ngati muli ndi matenda a Hyper IgE.

Palibe njira yotsimikizika yopewera matenda a Hyper IgE. Ukhondo wabwino umathandiza kupewa matenda apakhungu.

Opereka ena amalimbikitsa maantibayotiki othandizira anthu omwe amatenga matenda ambiri, makamaka ndi Staphylococcus aureus. Mankhwalawa sasintha vutoli, koma amatha kuchepetsa zovuta zake.

Matenda a Yobu; Matenda a Hyper IgE

Chong H, Green T, Larkin A. Matenda ndi ziwengo. Mu: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli ndi Davis ’Atlas of Pediatric Physical Diagnosis. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 4.

Holland SM, Gallin JI. Kuunika kwa wodwalayo yemwe akukayikira kuti ali ndi vuto losowa chitetezo m'thupi. Mu: Bennett JE, Dolin R, Blaser MJ, olemba., Eds. Mandell, Douglas, ndi Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020: mutu 12.

Hsu AP, Davis J, Puck JM, Holland SM, Freeman AF (Adasankhidwa) Matenda akuluakulu a Hyper IgE. Ndemanga za Gene. 2012; 6. PMID: 20301786 www.ncbi.nlm.nih.gov/pubmed/20301786. Idasinthidwa pa June 7, 2012. Idapezeka pa Julayi 30, 2019.