Syndrome liʻiliʻi
He aha kēīa ?
ʻO ka maʻi maʻi liʻiliʻi he ʻano like ia no ka infantile spastic diplegia.
ʻO Infantile spastic diplegia ka maʻi lolo i kaulana loa. Hōʻike ʻia ia e ka ʻoʻoleʻa o ka ʻiʻo i ka mea i hoʻopilikia ʻia, ʻoi aku ka nui o nā wāwae a me ka liʻiliʻi ma nā lima a me ka maka. ʻIke ʻia ka hyperactivity i nā tendon o nā wāwae i kēia pathology.
ʻO kēia ʻoʻoleʻa ʻiʻo i nā wāwae o ka mea i hoʻopilikia ʻia e hopena i ka ʻokoʻa o ka neʻe ʻana o nā wāwae a me nā lima.
I nā keiki me Little's Syndrome, maʻamau ka ʻōlelo a me ka naʻauao. (1)
Hoʻomaka maʻamau kēia maʻi cerebral diplegia i nā kamaliʻi a i ʻole nā keiki liʻiliʻi.
Loaʻa i ka poʻe me kēia maʻi ka piʻi ʻana o ka leo ʻiʻo e alakaʻi ana i ka spasticity muscle. ʻO kēia hiʻohiʻona he kani kiʻekiʻe a mau loa o nā ʻiʻo i ka hoʻomaha. ʻO nā reflexes hoʻonui pinepine ka hopena. Hoʻopili kēia ʻano spasticity i nā ʻiʻo o nā wāwae. ʻO nā ʻiʻo o nā lima, no lākou, ʻaʻole i hoʻopilikia ʻia.
Hiki i nā hōʻailona ʻē aʻe ke koʻikoʻi o ka maʻi. ʻO kēia ka hihia, no ka laʻana, o ka hele wāwae ma nā manamana wāwae a i ʻole ka hele wāwae ʻole.
ʻO kēia mau mea ʻino i ka leo ʻiʻo ka hopena o nā maʻi i loko o nā neurons o ka lolo a i ʻole ko lākou ulu ʻana.
He liʻiliʻi ka ʻike e pili ana i ke kumu kūpono o kēia maʻi neurological. Eia nō naʻe, ua kuhi kekahi poʻe noiʻi i ka pilina me nā genetic mutations, congenital malformations o ka lolo, nā maʻi a me nā fevers i ka makuahine i ka wā o kona hāpai ʻana a i ʻole nā pōʻino i ka wā hānau a i ʻole ma hope koke o ka hānau ʻana. hānau. (3)
I kēia lā, ʻaʻohe lāʻau lapaʻau no ka maʻi. Eia kekahi, aia nā mea ʻē aʻe ma muli o nā hōʻailona, nā hōʻailona a me ke koʻikoʻi o ka maʻi. (3)
Ōuli
Aia nā ʻano like ʻole o ka maʻi.
No laila, ʻokoʻa nā hōʻailona o ka Little's syndrome mai kekahi maʻi a i kekahi.
I loko o ka pōʻaiapili o ka lolo ma muli o nā pilikia neurological, ʻike ʻia nā hōʻailona i ka wā kamaliʻi. ʻO nā hōʻailona lapaʻau e pili ana i nā maʻi ʻiʻo (ʻoi loa ma nā wāwae) e hoʻopau i ka hoʻomalu a me ka hoʻonohonoho ʻana.
ʻO ke keiki e loaʻa ana i kēia maʻi maʻi e hōʻike i kahi leo ʻiʻo i ʻoi aku ka kiʻekiʻe ma mua o nā reflexes maʻamau a hoʻonui ʻia (ka hopena o ka ulu ʻana o ka spasticity).
ʻO nā hōʻailona ʻē aʻe paha he hōʻailona o ka hoʻomohala ʻana i ka diplegia spastic infantile. ʻO nā hōʻailona kūikawā e hōʻike ana i ka lohi o ka mākau kaʻa o ke keiki, ka hele wāwae ʻana ma nā manamana wāwae, ka hele wāwae asymmetrical, etc.
I nā hihia liʻiliʻi, hoʻololi kēia mau hōʻailona i ka wā o ke ola o ke kanaka. Eia nō naʻe, ʻaʻole ulu kēia mau mea ma ke ʻano maikaʻi ʻole. (3)
Ma waho aʻe o kēia mau hōʻailona o nā mākau kaʻa, pili paha nā mea ʻino ʻē aʻe i ka maʻi i kekahi mau hihia: (3)
- ke kino kīnā;
- nā pilikia aʻo;
- ka hoʻopaʻapaʻa;
- hoʻopau i ka ulu ʻana;
- nā mea ʻino ma ka iwi kuamoʻo;
- osteoarthritis (a i ʻole arthritis);
- ka ʻike maka;
- hoʻolohe lohe;
- pilikia ʻōlelo;
- nalo o ka mimi;
– nā ʻiʻo ʻokoʻa.
Nā kumu o ka maʻi
ʻO ka infantile spastic diplegia (a i ʻole Little's Syndrome) kahi maʻi lolo ma muli o ka hoʻomohala ʻino ʻana o kahi ʻāpana o ka lolo e hoʻomalu i nā mākau kaʻa.
Hiki ke hana ʻia kēia pōʻino o ka ulu ʻana o ka lolo ma mua, i ka wā, a i ʻole ma hope koke o ka hānau ʻana.
I ka hapanui o nā hihia, ʻaʻole ʻike ʻia ke kumu kūpono o ka hoʻomohala ʻana o ka pathology.
Eia naʻe, ua hana ʻia nā manaʻo, e like me: (1)
- nā genetic abnormalities;
- nā maʻi kino i loko o ka lolo;
- ka hiki ʻana o nā maʻi a i ʻole ke kuni i ka makuahine;
- ka pōʻino o ke keiki;
- etc.
Ua hōʻike ʻia nā kumu ʻē aʻe o ka maʻi: (1)
- ke koko intracranial hiki ke hoʻopau i ke kahe maʻamau o ke koko i loko o ka lolo a i ʻole ka haki ʻana o nā kīʻaha koko. ʻO kēia kahe koko ma muli o ka haʻalulu o ka fetal a i ʻole ke kūkulu ʻia ʻana o ke koko koko i loko o ka placenta. ʻO ke koko kiʻekiʻe a i ʻole ka ulu ʻana o nā maʻi i loko o ka makuahine i ka wā hāpai ʻana paha ke kumu;
- ka emi ʻana o ka oxygen i loko o ka lolo, e alakaʻi ana i ka asphyxia cerebral. ʻO kēia ʻano mea maʻamau ma hope o ka hānau ʻana koʻikoʻi. ʻO ka hāʻawi ʻana a hoʻemi ʻia ka oxygen no laila ke alakaʻi nei i kahi pōʻino nui i ke keiki: he hypoxic ischemic encephalopathy (EHI). Ua wehewehe ʻia ka hope e ka luku ʻana i ka ʻiʻo lolo. 'A'ole e like me ka mea ma mua, hiki i ka hypoxic ischemic encephalopathy ka hopena o ka hypotension i ka makuahine. ʻO ka haki ʻana o ka ʻōpū, ka wehe ʻana o ka placenta, nā anomalies e pili ana i ke kaula umbilical a i ʻole ka ʻeha poʻo i ka wā hānau keiki ke kumu;
- he ʻano ʻino i ka ʻāpana keʻokeʻo o ka cerebral cortex (kahi o ka lolo ke kuleana no ka hoʻouna ʻana i nā hōʻailona mai ka lolo a i ke kino holoʻokoʻa) kekahi kumu hou o ka ulu ʻana o ka maʻi;
- ka ulu ʻana o ka lolo, ka hopena o ka hoʻopau ʻana i ke kaʻina hana maʻamau o kona ulu ʻana. Hoʻopili ʻia kēia ʻano i ka hoʻololi ʻana i nā genes e hoʻopili ana i ka hoʻokumu ʻana o ka cerebral cortex. ʻO nā maʻi maʻi, ka loaʻa ʻana o nā fevers mau, trauma a i ʻole ka nohona maikaʻi ʻole i ka wā hāpai ʻana hiki ke lilo i mea hou aʻe o ka ulu ʻana o ka lolo.
Nā mea pilikino
ʻO nā kumu pilikia nui no ka hoʻomohala ʻana i ka maʻi ʻo Little's: (1)
- nā mea ʻino i nā pae o kekahi mau genes i ʻōlelo ʻia he predisposing;
- nā maʻi kino i loko o ka lolo;
- ka ulu ʻana o nā maʻi a me nā wela kiʻekiʻe i ka makuahine;
- nā ʻeha intracranial;
- ka emi ʻana o ka oxygen ma ka lolo;
- nā mea ʻino o ka ulu ʻana o ka cerebral cortex.
ʻO nā kūlana olakino hou aʻe paha ke kumuhana o ka piʻi ʻana o ka ulu ʻana i ka cerebral palsy i nā keiki: (3)
- hānau mua;
- he kaumaha māmā i ka hānau ʻana;
- nā maʻi a i ʻole ke kuni kiʻekiʻe i ka wā hāpai;
- nā hāpai he nui (māhoe, triplets, etc.);
- pili ʻole i ke koko ma waena o ka makuahine a me ke keiki;
- nā ʻano like ʻole o ka thyroid, ke kīnā o ka naʻau, ka nui o ka protein i loko o ka mimi a i ʻole ka convulsion i ka makuahine;
- ka hānau hānau ʻana;
- nā pilikia i ka wā hānau;
- kahi helu Apgar haʻahaʻa (index o ke kūlana olakino o ke keiki mai ka hānau ʻana);
- jaundice o ke keiki hānau hou.
Kāohi a me ka lapaʻau
Pono e hana koke ʻia ka ʻike o ka infantile spastic diplegia ma hope o ka hānau ʻana o ke keiki no ka pono o ke keiki a me kona ʻohana. (4)
Pono e hoʻokō ʻia ka nānā ʻana i nā maʻi kokoke loa. Hōʻike kēia i ka hoʻomohala ʻana i ka nānā ʻana i ke keiki i ka wā o kona ulu a ulu ʻana. Inā loaʻa nā hopena hopohopo i kēia hahai ʻana o ke keiki, hiki i kahi hoʻokolohua hoʻomohala hoʻomohala.
ʻO kēia kānana e pili ana i ka ulu ʻana o ke keiki ka hopena i nā hoʻāʻo e loiloi ana i ka hiki ke hoʻopaneʻe i ka ulu ʻana o ke keiki, e like me ka lohi ʻana i nā mākau kaʻa a i ʻole ka neʻe ʻana.
Inā ʻike ʻia nā hopena o kēia ʻāpana ʻelua o ka maʻi maʻi, hiki i ke kauka ke hoʻomau i ka hoʻomaʻamaʻa ʻana i nā loiloi olakino ulu.
ʻO ka pahuhopu o ka pae maʻi lapaʻau hoʻomohala ʻana, ʻo ia ka hōʻike ʻana i nā mea kūpono ʻole i ka ulu ʻana o ke keiki.
Aia i loko o kēia maʻi lapaʻau kekahi mau hoʻāʻo no ka ʻike ʻana i nā mea ʻino e pili ana i ka maʻi, ʻo ia: (3)
- ka nānā ʻana i ke koko;
- ʻo ka cranial scanner;
- MRI o ke poʻo;
- ka electrencephalogram (EEG);
– electromyography.
Ma ke ʻano o ka mālama ʻana, ʻaʻohe lāʻau lapaʻau i kēia manawa no ka maʻi.
Eia nō naʻe, hiki i nā lāʻau lapaʻau ke hoʻomaikaʻi i ke kūlana ola o nā maʻi. Pono e kuhikuhi koke ʻia kēia mau lāʻau lapaʻau ma hope o ka ʻike ʻana o ka maʻi.
ʻO nā lāʻau lapaʻau maʻamau, ʻo ia ka lāʻau lapaʻau, ka ʻokiʻoki, ka splinting, a me ke kino (physiotherapy) a me ka ʻōlelo (speech therapy).
Hiki ke hāʻawi ʻia nā kōkua kula i ka poʻe me kēia maʻi maʻi.
ʻO ka wānana koʻikoʻi o nā maʻi me kēia maʻi ʻokoʻa nui ma muli o nā hōʻailona a me nā hōʻailona i loko o ke kanaka.
ʻOiaʻiʻo, hoʻopilikia ʻia kekahi mau kumuhana ma ke ʻano haʻahaʻa (ʻaʻohe palena o kā lākou neʻe ʻana, kūʻokoʻa, a me nā mea ʻē aʻe) a ʻoi aku ka koʻikoʻi (hiki ʻole ke hana i kekahi mau neʻe me ke kōkua ʻole, etc.) (3).