Osteogenesis imperfecta: yintoni, iintlobo kunye nonyango

Umxholo

• Iindidi eziphambili
• Yintoni ebangela i-osteogenesis imperfecta
• Iimpawu ezinokwenzeka
• Indlela yokuqinisekisa ukuxilongwa
• Zithini iindlela zokhetho zonyango
• Ungabanyamekela njani abantwana abane-osteogenesis imperfecta

I-Osteogenesis imperfecta, ekwabizwa ngokuba sisifo samathambo eglasi, sisifo semfuza esinqabe kunene esibangela ukuba umntu akhubazeke, amathambo amafutshane kwaye abe ethe-ethe ngakumbi, ngenxa yokuba echaphazeleka rhoqo.

Olu buthathaka lubonakala ngathi lubangelwa sisiphene semfuza esichaphazela ukuveliswa kohlobo 1 lwe-collagen, eveliswa ngokwendalo zii-osteoblasts kwaye inceda ukomeleza amathambo namalungu. Ke, umntu one-osteogenesis imperfecta sele ezelwe enayo imeko, kwaye unokubonisa iimeko zokuqhekeka rhoqo ebuntwaneni, umzekelo.

Nangona i-osteogenesis imperfecta ingekanyangeki, lukhona unyango olukuncedayo ukuphucula umgangatho wobomi bomntu, ukunciphisa umngcipheko kunye nokuphindaphindeka kwethambo.

Iindidi eziphambili

Ngokokuhlelwa kukaSillence, kukho iindidi ezi-4 ze-osteogenesis imperfecta, ezibandakanya:

• Chwetheza I: sesona sifo sixhaphakileyo nesona sikhaphukhaphu, sibangela ukungaqheleki kwamathambo. Nangona kunjalo, amathambo aethe-ethe kwaye anokuqhekeka ngokulula;
• Uhlobo II: lolona hlobo lwesifo olubi kakhulu olwenza ukuba umntwana aqhekeke ngaphakathi esibelekweni sikanina, kukhokelela ekuqhomfeni kwiimeko ezininzi;
• Uhlobo lwe-III: abantu olu hlobo, ngokuqhelekileyo, musa ukukhula ngokwaneleyo, deformations ezikhoyo emqolo kunye abamhlophe wamehlo nga thaca ngwevu;
• Uhlobo IV: luhlobo olulinganiselweyo lwesifo, apho kukho ukungahambi kakuhle emathanjeni, kodwa akukho lutshintsho kumbala kwinxalenye emhlophe yamehlo.

Kwiimeko ezininzi, i-osteogenesis imperfecta idlulela ebantwaneni, kodwa iimpawu kunye nokuba qatha kwesi sifo kunokwahluka, kuba uhlobo lwesifo lunokutshintsha ukusuka kubazali kuye ebantwaneni.

Yintoni ebangela i-osteogenesis imperfecta

Isifo samathambo eglasi sivela ngenxa yotshintsho kwimfuza kuxanduva lokuvelisa uhlobo 1 lwe-collagen, eyona proteni iphambili isetyenziselwa ukwenza amathambo omeleleyo.

Njengotshintsho kwimfuza, i-osteogenesis imperfecta inokudlula isuka kubazali iye ebantwaneni, kodwa inokubonakala ngaphandle kwamanye amatyala kusapho, ngenxa yotshintsho ngexesha lokukhulelwa, umzekelo.

Iimpawu ezinokwenzeka

Ukongeza ekwenzeni utshintsho kumathambo, abantu abane-osteogenesis imperfecta banokuba nezinye iimpawu ezinje:

• Ukudibanisa amalungu;
• Amazinyo abuthathaka;
• Umbala oluhlaza okwesibhakabhaka wamehlo amhlophe;
• Ukugoba okungaqhelekanga komqolo (scoliosis);
• Ukuphulukana nokuva;
• Iingxaki zokuphefumla rhoqo;
• Mfutshane;
• I-hernias ye-Inguinal kunye ne-umbilical;
• Utshintsho lwezivalo zentliziyo.

Ukongeza, kubantwana abane-osteogenesis imperfecta, iziphene zentliziyo zinokuchongwa, ezinokuphela zisongela ubomi.

Indlela yokuqinisekisa ukuxilongwa

Ukuchongwa kwe-osteogenesis imperfecta, kwezinye iimeko, kunokwenziwa kwanaxa ukhulelwe, okoko nje kukho umngcipheko omkhulu wokuba umntwana azalwe enale meko. Kwezi meko, isampulu ithathwa kwimbambo apho i-collagen eveliswa ziiseli zomntwana phakathi kweeveki ezili-10 ukuya kwezi-12 zokukhulelwa. Enye indlela engangenisi kangako kukwenza i-ultrasound ukufumanisa ukwaphuka kwamathambo.

Emva kokuzalwa, ukuxilongwa kunokwenziwa ngugqirha wabantwana okanye ugqirha wamazinyo, ngokujonga iimpawu, imbali yosapho okanye kuvavanyo olufana neX-reyi, uvavanyo lofuzo kunye novavanyo lwegazi.

Zithini iindlela zokhetho zonyango

Akukho lonyango oluthile lwe-osteogenesis imperfecta kwaye, ke ngoko, kubalulekile ukuba ukhokelwe ngugqirha wamathambo. Ngokwesiqhelo iziyobisi ze-bisphosphonate zisetyenziselwa ukunceda ukwenza amathambo omelele kwaye kunciphise umngcipheko wokuqhekeka. Nangona kunjalo, kubaluleke kakhulu ukuba olu hlobo lonyango luvavanywa rhoqo ngugqirha, njengoko kunokuba yimfuneko ukulungelelanisa iidosi zonyango ngokuhamba kwexesha.

Xa kuvela ukwaphuka, ugqirha unokukhupha ithambo ngesamente okanye akhethe utyando, ngakumbi kwimeko yokuqhekeka okuninzi okanye okuthatha ixesha elide ukuphola. Unyango lokophuka luyafana nolo lwabantu abangenayo le meko, kodwa ixesha lokuncipha lihlala lifutshane.

I-Physiotherapy ye-osteogenesis imperfecta inokusetyenziswa kwezinye iimeko ukunceda ukomeleza amathambo kunye nezihlunu eziwaxhasayo, ukunciphisa umngcipheko wokuqhekeka.

Ungabanyamekela njani abantwana abane-osteogenesis imperfecta

Ezinye iindlela zokukhathalela abantwana abane-osteogenesis engafezekanga zezi:

• Kunqande ukuphakamisa umntwana ngeengalo, ukuxhasa ubunzima ngesandla esinye phantsi kwempundu nesinye emva kwentamo namagxa;
• Musa ukumtsala umntwana ngengalo okanye ngomlenze;
• Khetha isihlalo sokhuseleko esine-padding ethambileyo evumela umntwana ukuba asuswe kwaye abekwe ngaphandle komzamo omncinci.

Abanye abantwana abane-osteogenesis engafezekanga banokwenza umthambo olula, njengokuqubha, kuba banceda ukunciphisa umngcipheko wokuqhekeka. Nangona kunjalo, kufuneka benze njalo emva kokhokelo lukagqirha naphantsi kweliso lootitshala abafundisa ngokwasemzimbeni okanye ugqirha wezonyango.