Potera maʻi pale: mea, lalau mai, symptoms,ʻike,

Potera maʻi pale ua kapaʻia he nui hewa o ka fetus. Characterization hoʻomaka me ka wehewehena o keia anomaly.

He aha ka potera maʻi pale?

Potera maʻi pale - he congenital hewa o ka fetus, ma i kona pono ma kahi e puʻupaʻa, i mea o kaʻilihune o ka hoemi i ka buke o amniotic loli.

Ka hopena o ka wai wī mea he mīkini'opihia o ka fetus i loko o ka opu. Children i kekahi poorly ulu ia maʻiʻaʻai, typical wrinkled helehelena a me ka umi puniu wale no, ino mau lala i ka malamaia.

I ka 1964 godu potera hōʻike i ka ulu kina i wehewehe aku agenesis (ka nele o) nā fetal hakuʻala ole aplasia (wa e kaawale aku ana i kekahi hui, a me ke kino māhele), hui me ka lehalehu t abnormalities. Kēia maʻi pale ua haawiia keia i loko o ka 'inikua moʻokalaleo like hoʻi me maʻi pale lepo me kaʻaʻa puʻuwai agenesis 2,ʻaʻa puʻuwai-lehalehu t Grosse maʻi pale, lehalehu tʻaʻa puʻuwai dysplasia.

Potera ka maʻi pale i loko o ka fetus: 'ehaʻeha ka

Ke aetiology (Kinohi) maʻi pale ua nō,ʻaʻole maopopo hoomaopopo. I ka 50% o nā hihia ka mea i loaʻa i ka iniiaiie abnormality ua hoikea mai oia, ma muli o ia i ka nele o amniotic loli (fetal ka wai) i loko o ka wahine hapai. Mīkini'opihia o ka fetal uterus hiki aku ai i ke kūpono 'ana o kaʻaʻa puʻuwai anomalies alo (flattening a me ka flattening), naau, rectal, wahine, akemāmā (hypoplasia),ʻaoʻao (clubfoot).

Potera maʻi pale ia lŘlŘ i na kamalii uuku, na keiki ma lalo o ka makahiki o kekahi mau mahina, ma ka liʻiliʻi loa - i loko o nā keiki mua o hoʻokahi makahiki, a oi pinepine i loko o nā keikikāne. Ka mea hiki wale mai o ke kina :. 1 i 50 tausani hanau.

A ana o na makua

He aha i na symptoms o ka maʻi pale ua pu ma ka potera i loko o ka fetus? Nui keʻokeʻo hakuʻala - keia ka mea hiki ke ike ma ka ultrasound. Kēia agenesis hiki e dominantly ma kahi (nā kūpuna mai o ka mea 'ē aʻe makua hiki ole uhi ia), oa ole e pili pū ana i ka nui o amniotic loli. Kekahi o na makua i e underdevelopment paha wa e kaawale aku o kekahi o nā puʻupaʻa, a hiki ke nana ma ka 'inikua examinations mua aku.

ʻaʻa puʻuwai agenesis ua ili dominantly, a 'o ia hoʻi i ka mea, he 50% pahiki o ka hanau ana o ka fetus, a e e malama potera maʻi pale.

Potera maʻi pale me ka 75 pakeneka pahiki e hana ana i loko o ke kamaiki ia kona mau makua i ka mutation i loko o ka'ōewe PKHD1. Ka mea, i hoʻomaka ai ma luna o kaʻawelike 1 i 50 kanaka. Mau mutations mai i alakai i ka kokololio kūpono 'ana o ke kina i loko o ka Media, ka mea hiki no ka makahiki i hala ma, mai ia hanauna aku ia hanauna aku.

Ka pilikia o ka hanau ana o ke keiki me ka maʻi pale ke hoike potera'āina hoʻoili o nā mutations mai nā mākua. Inā ka fetus aaiaoe loli e e hele wale i ka makuakane a me ka makuwahine, a me ka 50 pakeneka wale ia e e hanau ola. 25% o nā keiki mai i loaa mai ai iaʻu PKHD1 mutation mai nā mākua, i kekahi loli ma ka aaiaoe kiʻekiʻe.

maʻi pale symptomatology

Potera ka maʻi pale i loko o ka fetus lapaʻau 'hōʻike i nā kēia mau hoikeana.

• loa haiki wahi i wāhiʻia kenekulia;
• ano hewa elua ma lalo o ka laina o ke kenekulia;
• mandibular hypoplasia (mikrognatsiya);
• ihu;
• abnormally nui mamao ma waena o ka hui kaulua nā loko (e like me nā maka) - hypertelorism;
• convex epicanthus ( "Mongolian pa") - he kūikawā ohana hoi ma ka pahale kihi o ka maka, e uhi ana i ka lacrimal tubercle;
• 'āno'ē shape pahee nui pepeiao.

Disinfection

A i ke keiki ua hanau i ka papa kuhikuhiE symptom - loa naehana popilikia, i hoike mai ai ia ia iho i na minuke mua o ka kūʻokoʻa ola. E mālama nei i hoʻokomo ea huikau pneumothorax (ea Oe ma ka pleural wahi e alalai mai i ka malamalama rhythmic neʻeʻana).

Eia naʻe, i ka poi pu hapanui o na kamalii uuku diagnosed me ka potera i ka maʻi pale make he mau hola ma hope o ka hanau ana. I ka wā o ka māmā pepa e pili ana digestivnogo (digestive)'āpana - urogenital cloaca (ka hui ana o ka loira, a urogenital leki uea i loko o kekahi),ʻaʻohe perforation o ka anus (pololei kakau) - ua hoʻohanaʻoki kino.

I koena keiki maʻi pale potera

I ka 2013, i ka Media Hoike ma luna o ke keiki kaikamahine Abigail lawe kīʻaha, ke kaikamahine a kekahi lala o ka Hale o ka United States Hale o ka Poeikohoia, Republican Congresswoman Jaime Herrera Beutler. ua hiki ke ola me keia maʻi pale e ālai 'ia' o ka maʻamau functioning o ka naehana'ōnaehana ke keiki.

Ma ka 5th o ka malama o ka hāpai keiki, i ka wahine akamai i ke kaikamahine, aole ia i ku mai, mai ka mimi a hiki i ka piha kaawale o puʻupaʻaʻelua. Ke kumu i kekahi shortage o amniotic loli i loko o ka hapai Jaime Herrera Beutler. I loko nō o ka hopena o na kauka hai aku keia hihia ona, o ka wahine a me kāna kāne nō e hoʻomau i ka hāpai keiki. I mea e compensate he uuku nui o amniotic loli i loko o ka uterus Jaime lawelawe kūikawā Saline. John Hopkins - ke kauka nāna i hana i ka Inc, hiki ole loa kumu hoʻomalu i ka noho male, e hana e alakai i ka kupono hopena.

Akā, me ka July 15, 2013 Little Abigaila i hanau ola. Mother ka Memories, ke kaikamahine i i hea i hanau, akā, ma hope o ka iho, hoomaka ae la e kahea aku - pēpē ka maʻiʻaʻai functioning. i ka hanau hope, ua i koke Neʻe i dialysis - i ka'ōnaehana i loa replaces hakuʻala papa e SafeSearch i nā huahana o pūnao mai ke kino. A makahiki ma hope, ua UAIAaIN ma kekahi mau pou hakuʻala.

A hiki ole no ka hanau ana o ka noho keiki maʻi pale potera, a me kona mahope holomua hoʻopulapula i loko o nā kumu o kēia realities ua no noʻonoʻo i ka hana mana. Nolaila, kauka koi ma luna o ka abortion i loko o kaʻike, o ka fetus ia he kina. Laʻana Abigail mea lawe kīʻaha, e hiki ole i pai mai ai, akā, e mea pono e ike i keia mea i loaʻa kekahi hihia.