Bilirubin encephalopathy

ʻO Bilirubin encephalopathy kahi maʻi neurological laha ʻole e kū ʻia i kekahi mau keiki hānau hou me ka jaundice koʻikoʻi.

Hoʻokumu ʻia ʻo Bilirubin encephalopathy (BE) e nā kiʻekiʻe kiʻekiʻe o ka bilirubin. ʻO Bilirubin kahi pigment melemele i hana ʻia i ka hemo ʻana o ke kino i nā hunaola ʻulaʻula kahiko. Hiki i nā kiʻekiʻe kiʻekiʻe o ka bilirubin i ke kino ke nānā i ka ʻili melemele (jaundice).

Inā kiʻekiʻe loa ke kiʻekiʻe o ka bilirubin a maʻi nui paha kahi pēpē, e neʻe ka mea mai ke koko a ʻohiʻohi i kaʻiʻo o ka lolo inā ʻaʻole ia e paʻa i ka albumin (protein) i loko o ke koko. Hiki i kēia ke alakaʻi i nā pilikia e like me ka poino o ka lolo a me ka nalo ʻana o ka lohe. ʻO ka huaʻōlelo "kernicterus" pili i ke kala melemele i hoʻokumu ʻia e bilirubin. ʻIke ʻia kēia i nā ʻāpana o ka lolo ma ka autopsy.

Hoʻonui pinepine kēia ʻano i ka pule mua o ke ola, akā e ʻike ʻia a hiki i ka pule ʻekolu. ʻO kekahi mau keiki hānau hou me ka maʻi hemolytic Rh aia i ka makaʻu nui no ka jaundice nui e hiki ai ke alakaʻi i kēia ʻano. Kākaʻikahi, hiki i ka BE ke hoʻomohala i nā pēpē olakino olakino.

Pili nā ʻōuli i ke kahua o BE. ʻAʻole nā ​​pēpē āpau me ka kernicterus ma ka autopsy i loaʻa nā ʻōuli maopopo.

Pākuʻi mua:

• ʻO ka jaundice nui loa
• Hōʻalo pīhoihoi hoʻomaka ʻole
• Hānai hānai maikaʻi ʻole ʻana
• ʻO ka hiamoe nui loa (lethargy) a me nā leo haʻahaʻa haʻahaʻa (hyponia)

Pae waena:

• Uē kūho kiʻekiʻe
• Huhū
• Ua hele a hoʻi i hope me ka ʻāʻī hyperextended i hope, ke kani kiʻekiʻe (hypertonia)
• ʻAi hānai maikaʻi ʻole

Mahele hope:

• ʻAha a koma paha
• ʻAʻohe hānai
• Uē Shrill
• Pākuʻi ka mākala, hoʻi i hope me ka ʻāʻī hyperextended i hope
• Ka hopu ʻana i nā maʻi

E hōʻike kahi hōʻike koko i kahi pae bilirubin kiʻekiʻe (ʻoi aku ma mua o 20 a 25 mg / dL). Eia nō naʻe, ʻaʻohe pilina pololei ma waena o ka pae bilirubin a me ka pae o ka ʻeha.

Hiki ke loli iki nā pae waiwai maʻamau i waena o nā hale hana ʻokoʻa. E kamaʻilio me kāu kauka e pili ana i ke ʻano o kāu mau hopena hōʻike kikoʻī.

Pili ka mālama ʻana i ka nui o nā makahiki o ke pēpē (i nā hola) a inā he ʻano pilikia paha kā ka pēpē (e like me ka prematurity). Pākuʻi ʻia paha:

• ʻO ka light therapy (phototherapy)
• Hoʻololi i nā transfusions (wehe i ke koko o ke keiki a hoʻololi iā ia me ke koko hāʻawi hou a i ʻole plasma)

He kūlana koʻikoʻi ʻo BE. ʻO nā pēpē he nui me nā ʻōnaehana ʻōnaehana hope loa e make.

Komo pū nā pilikia:

• Poino lolo paʻa
• Lohe hoʻolohe
• Make

E kiʻi koke i ke kōkua lapaʻau inā he hōʻailona ko kāu pēpē o kēia ʻano.

ʻO ka mālama ʻana i ka jaundice a i ʻole nā ​​kūlana e alakaʻi iā ia hiki ke pale i kēia pilikia. ʻO nā pēpē me nā ʻōuli mua o ka jaundice e ana ʻia ka pae bilirubin ma waena o 24 mau hola. Inā kiʻekiʻe ke kiʻekiʻe, pono e nānā ʻia ka pēpē no nā maʻi e pili ana i ka luku ʻia ʻana o nā hunaola ʻulaʻula (hemolysis).

Loaʻa i nā keiki hānau hou āpau ke koho hope ʻana ma waena o 2 a 3 mau lā ma hope o ka haʻalele ʻana i ka haukapila. He mea nui loa kēia no nā pēpē ma mua a i ʻole nā ​​wā hānau mua (hānau ʻia ma mua o 2 a 3 mau pule ma mua o ko lākou lā palena pau).

ʻO ka maʻi neurologic i hoʻokomo ʻia e Bilirubin (BIND); ʻO Kernicterus

• Jaundice hānau hou - hoʻokuʻu

• ʻO Kernicterus

Hamati AI. Nā pilikia Neurological o ka maʻi ʻōnaehana: keiki. I: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. ʻO Bradley's Neurology i ka hana lapaʻau. ʻEpekema 7 Piladelapia, PA: Elsevier; 2016: chap 59.

ʻO Hansen TWR. Pathophysiology o kernicterus. I: Polin RA, Abman SH, Rowitch, DH, Benitz WE, Fox WW, eds. ʻO Fetal and Neonatal Physiology. 5th lā. Piladelapia, PA: Elsevier; 2017: chap 164.

ʻO Kaplan M, Wong RJ, Sibley E, Stevenson DK. ʻO ka jaundice neonatal a me nā maʻi ate. I: Martin RJ, Fanaroff AA, Walsh MC, hua. ʻO Fanaroff lāua ʻo Martin's Neonatal-Perinatal Medicine. 10 o ka lā. Piladelapia, PA: Elsevier Saunders; 2015: chap 100.

Marcdante KJ, Kliegman RM. Anemia a me hyperbilirubinemia. I: Marcdante KJ, Kliegman RM, hua. Nā Pono Nui o Pediatrics. 8th ed. Elsevier; 2019: chap 62.