Lefu la Hyperimmunoglobulin E

Hyperimmunoglobulin E syndrome ke lefu le sa tloaelehang, le futsitsoeng. E baka mathata ka letlalo, libe, matšoafo, masapo le meno.

Hyperimmunoglobulin E syndrome e boetse e bitsoa Jobo syndrome. E rehelletsoe ka motho ea hlahang ka Bibeleng, Jobo, eo botšepehi ba hae bo ileng ba lekoa ke lefu la ho tsoa liso tsa letlalo le li-pustule. Batho ba nang le boemo bona ba na le tšoaetso ea letlalo e telele le e matla.

Matšoao a ba teng hangata bongoaneng, empa hobane lefu lena le fumaneha ka seoelo, hangata ho nka lilemo pele tlhahlobo e nepahetseng e etsoa.

Liphuputso tsa morao-rao li bontša hore lefu lena hangata le bakoa ke phetoho ea liphatsa tsa lefutso e etsahalang lefapheng la STAT3liphatsa tsa lefutso chromosome 17. Hore na ho se tloaelehe ha liphatsa tsa lefutso ho baka matšoao a lefu lena ha ho utloisisoe hantle. Leha ho le joalo, batho ba nang le lefu lena ba na le lesole le phahameng le fetang le tloaelehileng la lesole la mmele le bitsoang IgE.

Matšoao a kenyelletsa:

• Ho holofala ha masapo le meno, ho kopanyelletsa le ho robeha le ho tsoa meno nakong e tlang
• Eczema
• Matšoafo a letlalo le tšoaetso
• Matšoao a phetoang a sinus
• Ho pheta-pheta tšoaetso ea matšoafo

Tlhahlobo ea 'mele e ka bonts'a:

• Ho kobeha ha mokokotlo (kyphoscoliosis)
• Osteomyelitis
• Pheta tšoaetso ea sinus

Liteko tse sebelisitsoeng ho netefatsa hore na lefu lena le fumanoa joang li kenyelletsa:

• Palo ea li-eosinophil ka botlalo
• CBC ka phapang ea mali
• Serum globulin electrophoresis ho batla maemo a phahameng a mali a IgE
• Ho lekoa ka liphatsa tsa lefutso STAT3 liphatsa tsa lefutso

Tlhahlobo ea mahlo e ka senola matšoao a lefu le ommeng la mahlo.

X-ray ea sefuba e ka senola makhopho a matšoafo.

Liteko tse ling tse ka etsoang:

• Tlhatlhobo ea sefuba ea CT
• Litloaelo tsa sebaka sa ts'oaetso
• Liteko tse khethehileng tsa mali ho hlahloba likarolo tsa sesole sa 'mele
• X-ray ea masapo
• CT scan ea libe

Sistimi ea ho tšoaea e kopanyang mathata a fapaneng a lefu la Hyper IgE e ka sebelisoa ho thusa ho etsa tlhahlobo.

Ha ho na pheko e tsebahalang ea boemo bona. Morero oa kalafo ke ho laola tšoaetso. Meriana e kenyelletsa:

• Lithibela-mafu
• Meriana e thibelang mafu le e loantšang likokoana-hloko (ha ho hlokahala)

Ka linako tse ling ho hlokahala hore ho etsoa opereishene ho ntša makhala.

Gamma globulin e fanoang ka mothapo (IV) e ka thusa ho haha ​​sesole sa 'mele haeba u na le tšoaetso e matla.

Hyper IgE syndrome ke boemo bo sa foleng ba bophelo bohle. Tšoaetso e ngoe le e ngoe e ncha e hloka kalafo.

Mathata a ka kenyelletsa:

• Ho tšoaetsoa khafetsa
• Sepsis

Letsetsa mofani oa tlhokomelo ea bophelo haeba u na le matšoao a lefu la Hyper IgE.

Ha ho na mokhoa o netefalitsoeng oa ho thibela lefu la Hyper IgE. Bohloeki bo akaretsang boa thusa ho thibela tšoaetso ea letlalo.

Bafani ba bang ba ka khothaletsa lithibela-mafu bakeng sa batho ba nang le tšoaetso e ngata, haholoholo ka Staphylococcus aureus. Kalafo ena ha e fetole boemo, empa e ka fokotsa mathata a eona.

Lefu la Jobo; Lefu la Hyper IgE

Chong H, Green T, Larkin A. Ts'oaetso le immunology. Ka: Zitelli BJ, McIntire SC, Nowalk AJ, li-eds. Zitelli le Davis ’Atlas of Pediatric Physical Diagnosis. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 4.

Holland SM, Gallin JI. Tlhatlhobo ea mokuli ea belaelloang ke khaello ea 'mele Ka: Bennett JE, Dolin R, Blaser MJ, bahlophisi. Mandell, Douglas, le Bennett's Principles and Practice of Infectious Diseases. La 9th. Philadelphia, PA: Elsevier; 2020: khaolo ea 12.

Hsu AP, Davis J, Puck JM, Holland SM, Freeman AF. Autosomal e tsebahalang haholo ke lefu la hyper IgE. Litlhahlobo tsa Gene. 2012; 6. PMID: 20301786 www.ncbi.nlm.nih.gov/pubmed/20301786. E ntlafalitsoe ka la 7 Phuptjane 2012. E fihlile ka la 30 Phupu, 2019.