ʻO ka maʻi ʻo Hirschsprung

ʻO ka maʻi ʻo Hirschsprung kahi paʻa o ka ʻōpū nui. Kūpono ia ma muli o ka neʻe ʻana o nā mākala maikaʻi ʻole i loko o ka ʻōpū. He kūlana hānau ia, ʻo ia hoʻi aia mai ka wā hānau.

ʻO nā kuʻikuʻi Muscle i loko o ka ʻōpū e kōkua i nā meaʻai i hoʻoweliweli ʻia a me nā wai e neʻe i loko o ka ʻōpū. Kapa ʻia kēia peristalsis. Hoʻokumu nā aʻalolo ma waena o nā papa o ka mākala i nā pāʻina.

I ka maʻi ʻo Hirschsprung, ua nalowale nā ​​aʻa mai kahi ʻāpana o ka ʻōpū. ʻAʻole hiki i nā wahi me ka ʻole o kēia mau ʻalolo ke hoʻokuʻi i nā mea. ʻO kēia ke kumu o ka paʻa. Kūkulu ka ʻike i loko o ka Intestinal ma hope o ka paʻa ʻana. Hoʻopili ke ʻōpū a me ka ʻōpū i kahi hopena.

Hoʻokumu ka maʻi ʻo Hirschsprung ma kahi o 25% o nā ʻōpū ʻōpū hānau hou. Hana ʻia he 5 mau manawa i nā kāne ma mua o nā wahine. Pili ʻia ka maʻi ʻo Hirschsprung i kekahi manawa me nā ʻano hoʻoilina a i ʻole congenital, e like me Down Syndrome.

Nā ʻōuli e loaʻa paha i nā pēpē hou a me nā pēpē pū:

• Ka paʻakikī me ka neʻe ʻana o ka ʻōpū
• ʻAʻole hele i ka meconium ma hope koke o ka hānau ʻana
• ʻAʻole holo i kahi pahu mua ma waena o 24 a 48 mau hola ma hope o ka hānau ʻana
• Poe pahee pinepine ole
• ʻO Jaundice
• ʻAi hānai maikaʻi ʻole
• Loaʻa maikaʻi kaupaona
• ʻO ka luaʻi
• Pohā wai (i ke keiki hānau)

Nā ʻōuli i nā keiki nui:

• ʻO ka paʻa paʻa e hele a mānoanoa
• Ka hopena o ka fecal
• Kaʻai pono ʻole
• Hoʻonui lohi
• ʻŌpū ʻōhū

ʻAʻole hiki ke kuhi ʻia nā hihia momona a hiki i ka nui ʻana o ka pēpē.

I ka wā o kahi hoʻokolohua kino, hiki i ka mea mālama ola ke ʻike i nā puka lou o ka ʻōpū i loko o ka ʻōpū pehu. Hōʻike kahi hoʻokolohua kūpono i ke kani o nā mākala paʻa i nā mākala pololei.

Hoʻohana ʻia nā hoʻokolohua e kōkua i ke kuhi ʻana i ka maʻi ʻo Hirschsprung:

• Hihi x ʻōpū
• Manometry anal (hoʻonui ʻia kahi pāluna i ka pololei e ana i ke kaomi ma ia wahi)
• Enema Barium
• ʻO Biopsy Rectal

ʻO ke kaʻina hana i kapa ʻia ka irrigation serial serial e kōkua i ke kaomi i (decompress) i ka ʻōpū.

Pono e lawe i ka ʻāpana ʻino o ke kolona me ka hoʻohana ʻana i ke kaha. ʻO ka maʻamau, wehe ʻia ka pololei a me ka ʻāpana ʻokoʻa o ke kolona. Huki ʻia ka ʻaoʻao olakino o ke kolona a pili i ka anus.

I kekahi manawa hiki ke hana i kēia ma ka hana hoʻokahi. Eia nō naʻe, hana pinepine ʻia i ʻelua mau ʻāpana. Hana mua ʻia kahi colostomy. ʻO ka ʻāpana ʻē aʻe o ke kaʻina hana i hana ʻia ma hope i ka makahiki mua o ke ola o ke keiki.

Hoʻonui a hele aku paha nā ʻōuli ma ka hapanui o nā keiki ma hope o ke kaha ʻana. Loaʻa paha i kahi helu liʻiliʻi o nā keiki i ka constipation a i ʻole nā ​​pilikia e kaohi ana i nā pahu (incontinence fecal). ʻO nā keiki e mālama ʻia i ka wanaʻao a i ʻole ka pōkole o ka ʻōpū e pili me ka hopena maikaʻi aʻe.

Komo pū nā pilikia:

• ʻO ka hoʻohaunaele a me ka maʻi o nā ʻōpū (enterocolitis) hiki ke hana ma mua o ke kahakaha ʻana, a i kekahi manawa i ka makahiki 1 a 2 paha ma hope. Kūpilikiʻi nā ʻōuli, e like me ka pehu ʻana o ka ʻōpū, ʻinoʻino ka wai o ka wai, pilau, a me ka ʻai maikaʻi ʻole.
• Perforation a haki paha o ka ʻōpū.
• Short bowel syndrome, kahi kūlana e hiki ai i ka malnourishment a me ka make wai.

Kāhea i ka hāʻawi o kāu keiki inā:

• Hoʻokumu kāu keiki i nā ʻōuli o ka maʻi ʻo Hirschsprung
• Loaʻa i kāu keiki ka ʻeha o ka ʻōpū a i ʻole nā ​​hōʻailona hou aʻe ma hope o ka mālama ʻia ʻana no kēia ʻano

Megacolon hānau

ʻO Bass LM, Wershil BK. ʻO Anatomy, histology, embryology, a me nā anomalies ulu o ka ʻōpū liʻiliʻi a nui. I: Feldman M, Friedman LS, Brandt LJ, eds. ʻO Sleisenger a me Fordtran's Gastrointestinal a me ka maʻi Liver: Pathophysiology / Diagnosis / Management. 10 o ka lā. Piladelapia, PA: Elsevier Saunders; 2016: chap 98.

ʻO Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Nā maʻi motility a me ka maʻi ʻo Hirschsprung. I: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 358.