Isifo esinganyangekiyo segranulomatous

Isifo esingapheliyo se-granulomatous (CGD) sisifo esizuzwe njengelifa apho iiseli ezithile zamajoni omzimba zingasebenzi kakuhle. Oku kukhokelela kusulelo oluphindaphindiweyo nolomeleleyo.

Kwi-CGD, iiseli zomzimba zomzimba ezibizwa ngokuba zii-phagocytes azikwazi ukubulala iintlobo ezithile zebacteria kunye nefungi. Esi sifo sikhokelela kwixesha elide (elingapheliyo) kunye nokuphindaphinda (ukuphindaphinda) ukusuleleka. Imeko ihlala ifunyanwa kwasekuqaleni kakhulu ebuntwaneni. Iifom zeMilder zinokuchongwa ngexesha lokufikisa, okanye nokuba sele umdala.

Izinto ezinobungozi zibandakanya imbali yosapho yezifo eziphindaphindayo okanye ezingapheliyo.

Malunga nesiqingatha samatyala e-CGD adluliswa kwiintsapho njengesiqhelo sokunxibelelana ngesondo. Oku kuthetha ukuba amakhwenkwe asengozini yokufumana ingxaki kunamantombazana. Uhlobo olungalunganga luqhutywa kwi-X chromosome. Amakhwenkwe ane-1 X chromosome kunye ne-1 Y chromosome. Ukuba inkwenkwe ine-X chromosome enesiphene semfuza, inokuyifumana njengelifa le meko. Amantombazana ane-2 X yama-chromosomes. Ukuba intombazana ine-1 X i-chromosome enesiphene semfuza, enye i-chromosome engu-X inokuba nayo imfuza esebenza kuyo. Intombazana kufuneka izuze kwilifa le-X yesiphene kumzali ngamnye ukuze ifumane esi sifo.

I-CGD inokubangela iintlobo ezininzi zosulelo olunzima ukunyanga, kubandakanya:

• I-blisters okanye izilonda ebusweni (impetigo)
• Onokhwekhwe
• Ukukhula okugcwele ubofu (amathumba)
• Amaqhuma agcwaliswe siswini (amathumba)

I-CGD inokubangela:

• Urhudo oluqhubekayo
• I-lymph nodes ezivuvukileyo entanyeni
• Usulelo lwemiphunga, olufana nenyumoniya okanye ithumba lemiphunga

Umboneleli wezempilo uya kwenza uviwo kwaye angafumana:

• Ukudumba kwesibindi
• Ukudumba kwamathumbu
• I-lymph nodes ezidumbileyo

Kukho iimpawu zentsholongwane, ezinokuchaphazela amathambo amaninzi.

Uvavanyo olunokwenziwa lunokubandakanya:

• Ukuskena amathambo
• I-x-ray yesifuba
• Gcwalisa ubalo lwegazi (CBC)
• Uvavanyo lwe-cytometry flow ukunceda ukuqinisekisa isifo
• Ukuvavanywa kwemfuza ukuqinisekisa isifo
• Uvavanyo lomsebenzi weeseli ezimhlophe zegazi
• I-biopsy yethishu

Amayeza okubulala iintsholongwane asetyenziselwa ukunyanga esi sifo, kwaye anokusetyenziselwa ukuthintela usulelo. Iyeza elibizwa ngokuba yi-interferon-gamma linokunceda ekunciphiseni inani losulelo olomeleleyo. Utyando lunokufuneka ukunyanga amanye amathumba.

Unyango kuphela lwe-CGD ngumongo wethambo okanye ukufakelwa kweseli.

Unyango lwe-antibiotiki lwexesha elide lunokunceda ukunciphisa usulelo, kodwa ukufa kwangoko kunokwenzeka kusulelo oluphindaphindiweyo lwemiphunga.

I-CGD inokubangela ezi ngxaki:

• Ukonakala kwethambo kunye nosulelo
• Izifo ezingapheliyo kwimpumlo
• I-pneumonia eqhubeka ibuya kwaye kunzima ukuyinyanga
• Umonakalo wemiphunga
• Ukonakala kolusu
• Ukudumba kwe-lymph node ezihlala zidumbile, zenzeka rhoqo, okanye zenze amathumba afuna utyando ukuze uwakhuphe

Ukuba wena okanye umntwana wakho unale meko kwaye ukrokrela inyumoniya okanye olunye usulelo, tsalela umnikezeli wakho kwangoko.

Xelela umboneleli wakho ukuba umphunga, ulusu, okanye olunye usulelo aluphenduli kunyango.

Ukucebisa ngemfuza kuyacetyiswa ukuba uceba ukuba nabantwana kwaye unembali yosapho kwesi sifo. Ukuqhubela phambili kuvavanyo lwemfuza kunye nokwanda kokusetyenziswa kwesampulu ye-chorionic villus (uvavanyo olunokuthi lwenziwe ngexesha le-10th ukuya kwi-12 iveki yokukhulelwa) lenze ukuba kufumaneke i-CGD kwangoko. Nangona kunjalo, ezi zinto azikasasazwa okanye zamkelwe ngokupheleleyo.

I-CGD; I-granulomatosis ebulalayo yobuntwana; Isigulo esinganyangekiyo sobuntwana; Ukuqhubela phambili kwe-septic granulomatosis; Ukusilela kwePhagocyte - isifo esinganyangekiyo segranulomatous

Ukuphazamiseka komsebenzi we-phagocyte. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 169.

Holland SM, Uzel G. Phagocyte ukusilela. Ku: U-Rich RR, uFleisher TA, uShearer WT, uSchroeder JR. HW, Frew AJ, Weyand CM, ii-eds. I-Clinical Immunology: Imigaqo kunye nokuziqhelanisa. Umhla we-5. IPhiladelphia, PA: Elsevier; I-2019: isahluko 22.