Áèëèðóáèí

Áèëèðóáèí – æåëòî-êðàñíûé ïèãìåíò, îáðàçóþùèéñÿ ïðè ðàñïàäå ãåìîãëî-áèíà, âûñâîáîæäàþùèéñÿ èç ñòàðåþùèõ ýðèòðîöèòîâ ïîñëå èõ ãåìîëèçà â ðåòèêóëîýíäîòåëèàëüíîé ñèñòåìå.  íîðìå ñûâîðîòêà êðîâè ñîäåðæèò îáùåãî áèëèðóáèíà – 8,5 – 20,5 ìêÌë, ñâÿçàííîãî (ïðÿìîãî) – 0,9 – 4,3 ìêÌë, ñâîáîäíîãî(íåïðÿìîãî) - 6,4 – 17,1 ìêÌë. Ó íîâîðîæäåííûõ êîíöåíòðàöèÿ âûøå (17,1 – 205 ìêÌë).  ìî-÷å ó çäîðîâûõ ëþäåé íå îïðåäåëÿåòñÿ.Ïîâûøåíèå îáùåãî áèëèðóáèíà â ñûâîðîòêå íàáëþäàåòñÿ ïðè âîñïà- ëèòåëüíûõ, òîêñè÷åñêèõ è íåîïëàñòè÷åñêèõ ïîâðåæäåíèÿõ êëåòîê ïå÷åíè, ãåìîëèòè÷åñêèõ çàáîëåâàíèÿõ, ôèçèîëîãè÷åñêîé æåëòóõå íîâîðîæäåííûõ, çàêóïîðêå âíå - è âíóòðè ïå÷åíî÷íûõ æåë÷íûõ ïðîòîêîâ, ñèíäðîìàõ Äóáèíà – Äæîíñîíà, Êðèãëåðà – Íàéÿðà, ñèíäðîìå Æèëüáåðà, íàðóøåíèè òîëåðàíò-íîñòè ê ôðóêòîçå.

Æåëòóõà îáû÷íî ïîÿâëÿåòñÿ ïðè óðîâíå áèëèðóáèíà â êðî-âè, ïðåâûøàþùåì 27 – 34 ìêÌë. Áèëèðóáèíóðèÿ âîçíèêàåò ïðè çàêóïîðêå âíåïå÷åíî÷íûõ æåë÷íûõ ïóòåé, ïàðåíõèìàòîçíîé æåëòóõå è îòñóòñòâóåò ïðè ãåìîëèòè÷åñêîé æåëòóõå.Óâåëè÷åíèå ñîäåðæàíèÿ ñâÿçàííîãî (ïðÿìîãî) áèëèðóáèíà â ñûâîðîòêå îòìå÷àåòñÿ ïðè ïîäïå÷åíî÷íûõ æåëòóõàõ, öèððîçå, õîëåöèñòèòå, õîëàíãèòå, õîëåñòàçå (êîìïëåêñ ðàçíîðîäíûõ çàáîëåâàíèé ïå÷åíè - êëèíè÷åñêèé ïðè-çíàê ýòî êîæíûé çóä, íåðåäêî ðàçâèòèå æåëòóõè.

Ñèíäðîì íàðóøåííîãî âñà-ñûâàíèÿ(ïîíîñ èëè èñòîùåíèå), îñòåîïîðîç èëè îñòåîìàëÿöèÿ, ïðåâè÷íûé áèëëèàðíûé öèððîç(çàáîëåâàíèå ñ ïîðàæåíèåì ìåæäîëüêîâûõ æåë÷íûõ ïðî-òîêâ ñ ïîñëåäóþùèì ðàçâèòèåì ôèáðîçà ïîðòàëüíûõ òðàêòîâ è ïîñëåäóþùèé öèððîç; ïîòòâåðæäàåòñÿ ïîñëå êëèíè÷åñêèõ èññëåäîâàíèé - ëàáîðàòîðíî àí-ëè÷èå IG M, áèîïñèÿ ïå÷åíè); ïåðâè÷íûé ñêëåðîçèðóþùèé õîëàíãèò (ñ ïî-ðàæåíèåì âíåïå÷åíî÷íûõ è êðóïíûõ âíóòðè ïå÷åíî÷íûõ ïðîòîêîâ.

Âîñïàëå-íèå è ôèáðîç ïðèâîäÿò ïðèâîäÿò ê ðàçâèòèþ îáëèòåðàöèè è ðàçâèòèþ âòî-ðè÷íîãî áèëèàðíîãî öèððîçà, êëèíèêà çóä, æåëòóõà, ãåïàòîìåãàëèÿ, ñëàáîñòü è ïîõóäàíèå, ïðè ïðèñîåäèíåíèè áàêòåðèëüíîãî õîëàíãèòà ëèõîðàäêà è áîëü èíîãäà çàêàí÷èâàåòñÿ õîëàíãèîêàðöèíîìîé), ðàêå ïå÷åíè, ñèíäðîìàõ Äóáèíà – Äæîíñîíà, Ðîòîðà, ãèïîòèðåîçå ó íîâîðîæäåííûõ, àáñöåññå ïå÷åíè, ëåïòîñïèðîçå, õðîíè÷åñêîì ïàíêðåàòèòå, æåëòóõàõ áåðåìåííûõ, îòðàâëåíèè áëåäíîé ïîãàíêîé, ðïèåìå àìèíîñàëèöèëîâîé êèñëîòû, àíäîãåíîâ íèêîòèíî-âîé êèñëîòû, ïåíèöèëëèíà, ñóëüôàíèëàìèäîâ, ýðèòðîìèöèíà, ýñòðîãåíîâ.

Ó áîëüíûõ ñ ïîâûøåííûì ñîäåðæàíèåì ñâÿçàííîãî áèëèðóáèíà â ñûâîðîòêå âîçíèêàåò áèëèðóáèíåìèÿ.  êàëå êîëè÷åñòâî ñòåðêîáèëëèíà ïîíèæåíî.Óâå-ëè÷åíèå ñâÿçàííîãî è îò÷àñòè ñâîáîäíîãî áèëèðóáèíà îòìå÷àåòñÿ ïðè òîêñè-÷åñêîì ïîðàæåíèè ïå÷åíè.

Ïîâûøåíèå óðîâíÿ ñâîáîäíîãî (íå ïðÿìîãî) áèëèðóáèíà â ñûâðîòêå âîçíèêàåò ïðè ãåìîëèçå ëþáîé ýòèîëîãèè, ñèíäðîìå Êðèãëåðà – Íàéÿðà, ñèí-äðîìå Æèëüáåðà, õðîíè÷åñêîì ýðèòðîáëàñòîçå, ãàëàêòîçåìèè, ïàðîêñèçìàëü-íîé ãåìîãëîáèíóðèè, ïðè îòðàâëåíèè áåíçîëîì, ìóõîìîðîì, âîçìîæíî ïîñëå ïðèåìà íåêîòîðûõ ëåêàðñòâåííûõ ïðåïàðàòîâ (äîïåãèòà, èíäîìåòàöèíà, ôó-ðàöèëëèíà, îêñèòåòðàöèêëèíà, ñóëüôàíèëàìèäîâ). Ñòåðêîáèëèíîãåí ïðîäóêò ïðåâðàùåíèÿ áèëèðóáèíà, ïîä äåéñòâèåì êèñëîðîäà ïðåâðàùàåòñÿ â ñòåðêîáèëëèí.

Èç îðãàíèçìà âûâîäèòñÿ ñ êàëîì. Íîðìàëüíîå ñîäåðæàíèå â êàëå ñî-ñòàâëÿåò 50 – 300 ìã ñóò. Ïðèçíàêè Æåëòóõè Ìåõàíè÷åñêàÿ (îáòó-ðàöèîííàÿ, ïîäïå÷åíî÷íàÿ) Ïàðåõèìàòîçíàÿ (ïå÷å-íî÷íàÿ) Ãåìîëèòè÷åñêàÿ (íàäïå÷åíî÷íàÿ) Ñâîáîäíûé áèëèðóáèí êðîâè Íå èçìåíåí Íåçíà÷èòåëüíî ïîâûøåí Ðåçêî ïîâûøåí Ñâÿçàííûé áèëèðóáèí êðîâè Ðåçêî ïîâûøåí Çíà÷èòåëüíî ïîâûøåí Íåçíà÷èòåëüíî ïîâûøåí Ïðîäîëæåíèå òàáëèöû.Áèëèðóáèí ìî÷è Çíà÷èòåëüíî ïîâûøåí Íåçíà÷èòåëüíî ïîâûøåí Íå èçìåíåí Óðîáèëëèíîãåí ìî÷è Íå èçìåíåí èëè ñíè-æåí Çíà÷èòåëüíî ïîâûøåí Ðåçêî ïîâûøåí Ñòåðêîáèëëèí Ñíèæåí Íå èçìåíåí èëè ñíèæåí Òî æå Îðãàíîñïåöèôè÷åñêèå ôåðìåíòû Óìåðåííî ïîâûøåíû Ðåçêî ïîâûøåíû âí àðàí-íèõ ñòàäèÿõ Íå èçìåíåí Ôóíêöèîíàëüíûå ïðî-áû  äåáþòå íîðìû, çàòåì íå èçìåíåíû Ïàòîëîãè÷åñêè íå èçìå-íåíû Íîðìà  ôèçèîëîãè÷åñêèõ óñëîâèÿõ ñíèæåíèå êîíöåíòðàöèè ìîæåò íàáëþäàòñÿ ó äåòåé.

Óâåëè÷åíèå ñîäåðæàíèÿ îòìå÷àåòñÿ ïðè ãåìîëèòè÷åñêîé æåëòóõå, ñíèæåíèå – ïðè ìåõàíè÷åñêîé æåëòóõå, êàõåêñèè, íåêîòîðûõ àíåìèÿõ, ïîñëå ëå÷åíèÿ àíòèáèîòèêàìè.

Ó áîëüíûõ ïàðåíõèìàòîçíîé æåëòó-õîé âîçìîæíî íåáîëüøîå ïîíèæåíèå. Óðîáèëëèíîãåí – ïðîäóêò ïðåâðàùåíèÿ áèëèðóáèíà.Ïðè îêèñëåíèè íà âîçäóõå ïðåâðàùàåòñÿ â óðîáèëëèí. íîðìàëüíîå ñîäåðæàíèå â ìî÷å ñîñòàâëÿåò 0.08 – 4.23 ìêÌñóò(ïî äðóãèì ñâåäåíèÿì – íèæå 1.7. ìêÌñóò). â êàëå - 67 - 423ìêÌ ñóò. Âûâîäèòñÿ ñ ïðåèìóùåñòâåííî ñ ìî÷îé. Åãî óðîâåíü â ìî÷å ïîâûøàåòñÿ ïðè çàáîëåâàíèÿõ ïå÷å-íè(ïîðòàëüíûé öèððîç, ãåïàòèò, òîêñè÷åñêîå ïîðàæåíèå ïå÷åíè, îáòóðàöèÿ æåë÷íûõ ïóòåé), âíóòðèñîñóäè-ñòîì ãåìîëèçå, êðîâîèçëèÿíèè â òêàíè, çàïîðàõ, ñåðäå÷íîé íåäîñòàòî÷íîñòè, âîçìîæíî ïðèñâèíöîâîì îò-ðàâëåíèè è ïðåíèöèîçíîé àíåìèè; îòñóòñòâèå èëè ñíèæåíèå â ìî÷å íàáëþäàåòñÿ ïðè ïîëíîé îáòóðàöèè æåë÷íûõ ïóòåé áåç èõ èíôèöèðîâàíèÿ, ïîñëå íà÷àëüíîé ñòàäèè îñòðîãî èíôåêöèîííîãî ãåïàòèòà, ïî÷å÷íîé íåäîñòàòî÷íîñòè, õîëñòàçå. Äèàãíîñòèêà – âàæíî îïðåäåëèòü, ÷åì îáóñëîâëåíà ãèïåðáèëèðóáèíåìèÿ ïðÿìûì è íå ïðÿìûì áè-ëèðóáèíîì.

Ãèïåðáèëèðóáèíåìèÿ çà ñ÷åò ïðÿìîãî áèëèðóáèíà îáû÷íî ðåçóëüòàò ïîðàæåíèÿ ïå÷åíî÷íûõ êëåòîê(ïàðåíõèìû), õîëåñòàçà èëè âíåïå÷åíî÷íîé îáñòðóêöèè, êëèíè÷åñêîå îáñëåäîâàíèå âêëþ÷àåò àíàì-íåç(îáðàòèòü âíèìàíèå íà äëèòåëüíîñòü æåëòóõè, çóä, ñîïóòñòâóþùèå áîëè, ëèõîðàäêó, ïîõóäàíèå, ôàêòîðû ðèñêà ïàðåíòåðàëüíîãî çàðàæåíèÿ, ìåäèêàìåíòû, àëêîãîëü, ñâåäåíèÿ î ïóòåøåñòâèÿõ, îïåðàöèÿõ, áåðåìåí-íîñòè,) ôèçèêàëüíûå èññëåäîâàíèÿ (óâåëè÷åíèå ïå÷åíè, áîëåçíåííîñòü, ïðè ïëüïàöèè, ïàëüïèðóåìûé æåë÷-íûé ïóçûðü, ñïëåíîìåãàëèÿ, ãèíåêîìàñòèÿ, òàðîôèÿ ÿè÷åê), ðåçóëüòàòû áèîõèìè÷åñêèõ ïå÷åíî÷íûõ ïðîá: Áèëèðóáèí Àìèíîòðàíñôåðàçû (àñïàðòàòàìèíîòðàíñôåðàçà ÀñÀÒ, àëàíèíàìèíîòðàíñôåðàçà ÀëÀÒ èíäèêàòîðû öåëîñòíîñòè êëåòîê ïå÷åíè Ëàêòàòäåãèäðîãåíàçà ËÄà Ùåëî÷íàÿ ôîñôàòàçà ÙÔ - ïîêàçàòåëü õîëñòàçà 5 Íóêëåîòèäàçà 5 ÍÒ èíäèêàòîð ïîðàæåíèÿ ãåïàòîáèëèàðíîé ñèñòåìû γ - Ãëóòàìèíòðàíñôåðàçà ÃÃÒÔ àêòèâíà íàðàâíå ñ ÙÔ Ïðîòðîìáèíîâîå âðåìÿ Ï - ïîêàçàòåëü àêòèâíîñòè ðÿäà ôàêòîðîâ ñâåðòûâàíèÿ êðîâè (II,VII, IX,X) - ýòè ôàêòîðû ñèíòåçèðóþòñÿ â ïå÷åíè, ïîýòîìó èõ äåôèöèò áûñòðî âîçíèêàåò ïðè îáøèðíîì íåêðîçå ïå÷å-íè (ãåïàòèò, òîêñèêîç, öèððîç) Àëüáóìèí - ñíèæåíèå ñîäåðæàíèÿ â ñûâîðîòêå êðîâè îòðàæàåò óìåíüøåíèå åãî ñèíòåçà â ïå÷åíè Ãëîáóëèíû - óìåðåííî âûðàæåííàÿ ïîëèêëîíàëüíàÿ ãèïåðãëîáóëèíåìèÿ ÷àñòî ïðè õðîíè÷åñêèõ çà-áîëåâàíèÿõ, ñóùåñòâåííàÿ ïðè àóòîèììóííîé ôîðìå õðîíè÷åñêîãî àêòèâíîãî ãåïàòèòà.

Àììèàê – óðîâåíü ïîâûøàåòñÿ ïðè íåýôôåêòèâíîñòè ïå÷åíî÷íûõ ïóòåé äåòîêñèêàöèèè ïîðòîñè-ñòåìíîãî øóíòèðîâàíèÿ, êàê áûâàåò ïðè ìîëíèåíîñíîì ãåïàòèòå.

Êëèíè÷åñêèé àíàëèç êðîâè.

Åñëè ïå÷åíî÷íûå ïðîáû ãîâîðÿò î ïàðåíõèìàòîçíîì ïîðàæåíèè, ñëåäó-åò âûïîëíèòü ñåðîëîãè÷åñêèå è ìåòàáîëè÷åñêèå ïðîáû, è âîçìîæíî áèîõèìè÷åñêèå àíàëèçû; õîëåñòàç èëè âíåïå÷åíî÷íóþ îáñòðóêöèþ âåðèôèöèðóþò ñ ïîìîùüþ ÊÒ è ÓÇÈ ñ ïîñëåäóþùèì áèîõèìè÷åñêèì èññëåäî-âàíèåì, õîëàíãèîãðàôèåé è äðåíèðîâàíèåì æåë÷åâûâîäÿùèõ ïóòåé.

Ïðÿìàÿ ãèïåðáèëèðóáèíåìèÿ áåç èçìå-íåíèé ïå÷åíî÷íûõ ôåðìåíòîâ îòìå÷àåòñÿ ïîñëå íåäàâíî ïåðåíåñåííîãî èëè â õîäå òåêóùåãî ñåïñèñà, ïðè ñèíäðîìå Ðîòîðà èëè Äàáèíà – Äæîíñîíà.

ÑÏÈÑÎÊ ÈÑÏÎËÜÇÎÂÀÍÍÎÉ ËÈÒÅÐÀÒÓÐÛ 1. Ã.È. Êîçèíåö èíòåðïðåòàöèÿ àíàëèçîâ êðîâè è ìî÷è ÀÎÇÒ "Ñàëèíåö" 1997ã. 2. Ëèôùèö Â.Ì ÑèäåëüíèêîâàÂ.È. Áèîõèìè÷åñêèå àíàëèçû â êëèíèêå Ìîñêâà 2001. 3. Òåðàïåâòè÷åñêèé ñïðàâî÷íèê Âàøèíãòîíñêîãî óèíâåðñèòåòà ×. Êåðè, Ê. Âåëüòå. "Ïðàêòèêà" Ìîñêâà 2000. 4. Ê. Èññåëüáàõåð, Å. Áðàóíâàëüäà, Äæ. Âèëüñîí, À. Ôàó÷è, Ä. 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