Ukuqonda i-Thoracic Dystrophy ephazamisayo
I-Jeune syndrome, eyaziwa nangokuthi i-thoraphic dystrophy, iwuhlobo oluthile olulutho oluthile oluthola izitho ezincane, isifuba esincane, nezinkinga zezinso. Ukubonakaliswa kwayo okuyinhloko, noma kunjalo, ukucindezeleka okuphefumula ngenxa yesikhukhula esincane. Kulinganiselwa ukuthi ku-1 per 100,000-130,000 abazalwa ngokuphila futhi kuthinta abantu bazo zonke izizinda.
Izimpawu
Abantu abane-Jeune syndrome banezici ezithile ezifanayo:
- Isifuba esincane, esincane futhi esingavamile kakhulu esinomthamo wamaphaphu wehlisiwe
- Amandla amancane nemilenze uma kuqhathaniswa ne-trunk kanye nesibalo esincane ( isisu esifushane)
- Izilonda zezinso ezingabangela ukwehluleka kwezinso
Ezinye izimpawu okungenzeka ukuthi abantu abaneJune syndrome bangase babe nazo:
- I-malabsorption yesisu
- Ukuguqulwa kokubuyisela
- Izinkinga zesibindi
- Izinkinga zenhliziyo nenhliziyo
Ngokuvamile, ukucindezeleka okukhulu okuphefumula kuvela ngesikhathi sokusana. Kwezinye izimo, izinkinga zokuphefumula zinzima kakhulu, futhi ukungavamile kwezinso noma isimiso samathumbu kungase kube khona.
Ukuxilongwa
I-Jeune syndrome ivame ukutholakala lapho izalwa ngokususelwa kwesifubeni esifubeni kanye nesisindo esincane esifushane. Izinsana ezithinteka kakhulu ziyoba nokucindezeleka kokuphefumula. Amacala amancane angatholakala nge-x-ray esifubeni.
Ukwelapha
Indawo ebaluleke kakhulu yokunakekelwa kwezempilo yomuntu ngamunye nge-Jeune syndrome ivikela nokuphatha izifo zokuphefumula.
Ngeshwa, izinsana eziningi nezingane ezine-syndrome ziyafa ngenxa yokuhluleka ukuphefumula okulethwa yisifuba esincane kakhulu futhi izifo eziphefumulayo eziphindaphindiwe.
Kwezinye izimo, ukwandisa umgogodla wezinambuzane kanye nokuhlinzwa ngokuvuselela esifubeni uye waphumelela ekunciphiseni ukucindezeleka okuphefumulayo. Lokhu kuhlinzwa kunzima futhi kuyingozi futhi kugcinwe izingane ezinenkinga yokuphefumula okukhulu.
Abantu abane-Jeune syndrome bangase bathuthukise ukucindezeleka kwegazi okuvela ezifweni zezinso. Izinso zabo zingagcina zihluleka, okuphathwa ngukuthi i- dialysis noma ukufakelwa kwezinso .
Abantu abaningi abane-Jeune syndrome abasinda ebuntwaneni baqala ukuqala ukuthuthukiswa kwesifuba.
Ukululekwa nge-Genetic
I-syndrome ye-Jeune i- disosomal disordomal disorder . Lokhu kusho ukuthi bobabili abazali kufanele babe yiziphathamandla zesakhi sofuzo ukuze ingane izuze i-syndrome. Ngakho-ke, uma abazali bebeletha ingane ethintekile, lokhu kusho ukuthi bobabili bayithwala, nokuthi ingane ngayinye elandelayo abanazo ithuba lokuthola i-syndrome.
Imithombo
U-Chen, H. (2004). Ukuphefumula i-thoracic dystrophy (i-Jeune syndrome). eMedicine.
Sarimurat N, Elçioğlu N, Tekant GT, et al. Ukunyanyiswa komzimba we-thoracic we-Jean's exhausting. I-Eur J Pediatr Surg 1998; 8: 100.
Ehlelwe nguRichard N. Fogoros, MD