{"id":1691,"date":"2019-05-09T10:13:21","date_gmt":"2019-05-09T08:13:21","guid":{"rendered":"http:\/\/www.newslab.sk\/2019\/05\/09\/hellp-syndrom\/"},"modified":"2019-05-09T10:17:36","modified_gmt":"2019-05-09T08:17:36","slug":"hellp-syndrome","status":"publish","type":"post","link":"https:\/\/www.newslab.sk\/en\/hellp-syndrome\/","title":{"rendered":"HELLP syndrome"},"content":{"rendered":"

*All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf attachment at the end of the paper.<\/strong><\/span><\/p>\n

 <\/p>\n

\u00davod <\/strong><\/p>\n

HELLP (h<\/em><\/strong>emolysis, <\/em>e<\/em><\/strong>levated <\/em>l<\/em><\/strong>iver enzymes, <\/em>l<\/em><\/strong>ow <\/em>p<\/em><\/strong>latelet count<\/em>) syndr\u00f3m je z\u00e1va\u017enou komplik\u00e1ciou tehotenstva, ktor\u00fd sa vyskytuje u 0,2 \u2013 0,8 % tehotn\u00fdch \u017eien a v 70 \u2013 80 % pr\u00edpadov koexistuje s preeklampsiou. Preeklampsia je definovan\u00e1 ako nov\u00fd n\u00e1stup hypertenzie a protein\u00farie po 20. t\u00fd\u017edni tehotenstva u predt\u00fdm zdravej \u017eeny. Av\u0161ak hypertenzia alebo protein\u00faria nie s\u00fa pr\u00edtomn\u00e9 u 10 \u2013 15 % \u017eien, u ktor\u00fdch sa vyvinul HELLP syndr\u00f3m(1). HELLP syndr\u00f3m v roku 1982 prv\u00fdkr\u00e1t op\u00edsal Weinstein a kol., pri\u010dom term\u00edn HELLP je skratka pou\u017e\u00edvan\u00e1 na opis klinick\u00e9ho stavu, ktor\u00fd vedie k hemol\u00fdze, zv\u00fd\u0161en\u00fdm pe\u010de\u0148ov\u00fdm enz\u00fdmom a n\u00edzkemu po\u010dtu krvn\u00fdch do\u0161ti\u010diek( 2). HELLP syndr\u00f3m m\u00f4\u017ee vies\u0165 k r\u00f4znym komplik\u00e1ci\u00e1m ohrozuj\u00facim \u017eivot, ako je abrupcia placenty, p\u013e\u00facny ed\u00e9m nasledovan\u00fd ak\u00fatnou respira\u010dnou insuficienciou, cerebr\u00e1lne krv\u00e1canie, septick\u00fd \u0161ok, ak\u00fatne zlyhanie obli\u010diek a hepatick\u00e9 krv\u00e1canie v d\u00f4sledku rupt\u00fary pe\u010dene(4).<\/p>\n

 <\/p>\n

Klinick\u00e9 pr\u00edznaky a v\u00fdskyt <\/strong><\/p>\n

Typick\u00fdmi klinick\u00fdmi pr\u00edznakmi s\u00fa boles\u0165 a palpa\u010dn\u00e1 citlivos\u0165 v epigastriu a v pravom hypochondriu, \u00fanava a ne\u0161pecifick\u00e9 pr\u00edznaky. \u010ealej s\u00fa to opuchy tv\u00e1re a kon\u010dat\u00edn, nauzea, vracanie, poruchy zraku, krv\u00e1cav\u00e9 prejavy a hna\u010dka; a\u017e 30 \u2013 60 % \u017eien m\u00e1 bolesti hlavy. Mnoho pacientok v\u0161ak opisuje aj mal\u00e1tnos\u0165 p\u00e1r dn\u00ed pred n\u00e1stupom sympt\u00f3mov. Pr\u00edznaky zvy\u010dajne nepretr\u017eite postupuj\u00fa a ich intenzita sa \u010dasto spont\u00e1nne men\u00ed. HELLP syndr\u00f3m sa prejavuje v\u00e4\u010d\u0161inou po\u010das noci a cez de\u0148 pr\u00edznaky ustupuj\u00fa(4). V 70 % pr\u00edpadov sa HELLP syndr\u00f3m prejav\u00ed po\u010das tehotenstva medzi 27. a 37. t\u00fd\u017ed\u0148om. Pribli\u017ene v 10 % pr\u00edpadov sa prejav\u00ed pred 27. t\u00fd\u017ed\u0148om tehotenstva a 20 % po 37. t\u00fd\u017edni tehotenstva. HELLP syndr\u00f3m sa m\u00f4\u017ee prejavi\u0165 aj po p\u00f4rode, a to v\u00e4\u010d\u0161inou do 48 hod\u00edn u \u017eien, ktor\u00e9 mali pred p\u00f4rodom hypertenziu alebo protein\u00fariu. Hoci je to u ka\u017edej tehotnej \u017eeny variabiln\u00e9, n\u00e1stup HELLP syndr\u00f3mu je zvy\u010dajne r\u00fdchly(3). \u017deny s \u010diasto\u010dn\u00fdm HELLP syndr\u00f3mom maj\u00fa menej sympt\u00f3mov a vyvinie sa u nich menej komplik\u00e1ci\u00ed ako u \u017eien s \u00fapln\u00fdm syndr\u00f3mom. Av\u0161ak \u010diasto\u010dn\u00fd alebo ne\u00fapln\u00fd HELLP syndr\u00f3m sa m\u00f4\u017ee vyvin\u00fa\u0165 na \u00fapln\u00fa formu tejto choroby(5). Sestry a deti \u017eeny, ktor\u00e1 trpela HELLP syndr\u00f3mom, maj\u00fa zv\u00fd\u0161en\u00e9 riziko HELLP syndr\u00f3mu. Takisto \u017eena, ktor\u00e1 u\u017e v predch\u00e1dzaj\u00facom tehotenstve mala HELLP syndr\u00f3m, m\u00e1 zv\u00fd\u0161en\u00e9 riziko jeho op\u00e4tovn\u00e9ho vzniku (14 \u2013 24 %) a rovnako preeklampsie (22 \u2013 28 %) v nadch\u00e1dzaj\u00facich tehotenstv\u00e1ch, \u010do poukazuje na s\u00favisiace patogenetick\u00e9 mechanizmy(5). Uk\u00e1zalo sa, \u017ee hoci HELLP syndr\u00f3m m\u00f4\u017ee ma\u0165 vplyv aj na novorodenca, v\u00e4\u010d\u0161\u00edm probl\u00e9mom je skor\u00fd p\u00f4rod v d\u00f4sledku syndr\u00f3mu, a teda sk\u00f4r n\u00edzky gesta\u010dn\u00fd vek die\u0165a\u0165a ako samotn\u00fd HELLP syndr\u00f3m. Dvoj\u010dat\u00e1 naroden\u00e9 matk\u00e1m s HELLP syndr\u00f3mom m\u00f4\u017eu vyvola\u0165 trombocytop\u00e9niu a neurologick\u00e9 komplik\u00e1cie. Av\u0161ak v\u00e4\u010d\u0161ina novorodencov naroden\u00fdch \u017een\u00e1m s HELLP syndr\u00f3mom m\u00e1 norm\u00e1lny dlhodob\u00fd v\u00fdvoj(5).<\/p>\n

 <\/p>\n

Patofyziol\u00f3gia <\/strong><\/p>\n

Hemol\u00fdza <\/em>Rap\u00eddne zn\u00ed\u017eenie po\u010dtu erytrocytov po\u010das HELLP syndr\u00f3mu je pravdepodobne d\u00f4sledkom po\u0161kodenia buniek sp\u00f4soben\u00e9ho ukladan\u00edm fibr\u00ednu, \u010do vedie k fragment\u00e1cii \u010derven\u00fdch krviniek. Toto zistenie je konzistentn\u00e9 s mikroangiopatickou hemolytickou an\u00e9miou, s deformovan\u00fdmi \u010derven\u00fdmi krvinkami pozorovan\u00fdmi v mikroskope spolu so schistocytmi aj s polychrom\u00e1ziou v perif\u00e9rnej krvi(7). \u00a0<\/strong>\u0160iroko pr\u00edstupn\u00e9 markery hemol\u00fdzy m\u00f4\u017eu by\u0165 hladiny lakt\u00e1tdehydrogen\u00e1zy (LDH) a nekonjungovan\u00e9ho bilirub\u00ednu. Navy\u0161e pr\u00edtomnos\u0165 retikulocytov m\u00f4\u017ee by\u0165 vysvetlen\u00e1 ako \u00fasilie organizmu \u010deli\u0165 t\u00fdmto podmienkam a uvo\u013eni\u0165 nezrel\u00e9 bunky ako kompenza\u010dn\u00fd odraz. Uk\u00e1zalo sa, \u017ee citlivej\u0161\u00edm a presnej\u0161\u00edm ukazovate\u013eom hemol\u00fdzy je nezvy\u010dajne n\u00edzka hladina haptoglob\u00ednu. Jeho stanovenie je v\u0161ak zriedkavo zahrnut\u00e9 v rutinnom laborat\u00f3rnom skr\u00edningu pacientov(4). Zv\u00fd\u0161enie pe\u010de\u0148ov\u00fdch enz\u00fdmov <\/em>Zv\u00fd\u0161enie pe\u010de\u0148ov\u00fdch enz\u00fdmov m\u00f4\u017ee odr\u00e1\u017ea\u0165 hemolytick\u00fd proces aj postihnutie pe\u010dene. Hemol\u00fdza prispieva v podstate k zv\u00fd\u0161en\u00fdm hladin\u00e1m lakt\u00e1tdehydrogen\u00e1zy, zatia\u013e \u010do zv\u00fd\u0161en\u00e9 hladiny aspart\u00e1taminotransfer\u00e1zy (AST) a alan\u00ednaminotransfer\u00e1zy (ALT) s\u00fa v\u00e4\u010d\u0161inou sp\u00f4soben\u00e9 po\u0161koden\u00edm pe\u010dene. Plazmatick\u00e1 glutati\u00f3n-S-transfer\u00e1za-a1 (GST-a1) m\u00f4\u017ee poskytn\u00fa\u0165 citlivej\u0161\u00ed indik\u00e1tor pre ak\u00fatne po\u0161kodenie pe\u010dene ako AST a ALT a umo\u017eni\u0165 skor\u0161ie rozpoznanie. Av\u0161ak meranie GST-a1 nie je v\u0161eobecne pou\u017e\u00edvan\u00e9 a zatia\u013e sa nenach\u00e1dza v rutinnom diagnostickom postupe(4). N\u00edzky po\u010det krvn\u00fdch do\u0161ti\u010diek <\/em>N\u00edzky po\u010det krvn\u00fdch do\u0161ti\u010diek (trombocytop\u00e9nia) v tehotenstve m\u00f4\u017ee by\u0165 sp\u00f4soben\u00fd gesta\u010dnou trombocytop\u00e9niou (59 %), imunitnou trombocytopenickou purpurou (11 %), preeklampsiou (10 %) alebo HELLP syndr\u00f3mom (12 %). Trombocytop\u00e9nia je pri uveden\u00fdch komplik\u00e1ci\u00e1ch zriedkav\u00e1 alebo \u010dast\u00e1, no pri HELLP syndr\u00f3me \u00faplne be\u017en\u00e1. Zn\u00ed\u017eenie po\u010dtu krvn\u00fdch do\u0161ti\u010diek je pri HELLP syndr\u00f3me sp\u00f4soben\u00e9 d\u00f4sledkom ich zv\u00fd\u0161enej spotreby. Do\u0161ti\u010dky sa aktivuj\u00fa a pri\u013en\u00fa k po\u0161koden\u00fdm vaskul\u00e1rnym endoteli\u00e1lnym bunk\u00e1m, \u010do vedie k zv\u00fd\u0161en\u00e9mu pohybu trombocytov s krat\u0161ou \u017eivotnos\u0165ou( 4).<\/p>\n

 <\/p>\n

Lie\u010dba tehotn\u00fdch \u017eien s HELLP syndr\u00f3mom <\/strong><\/p>\n

Vo v\u0161eobecnosti existuj\u00fa tri hlavn\u00e9 mo\u017enosti na lie\u010dbu \u017eien s HELLP syndr\u00f3mom. Tie zahr\u0148uj\u00fa: 1) Okam\u017eit\u00fd p\u00f4rod, ktor\u00fd je prvou vo\u013ebou v 34. t\u00fd\u017edni tehotenstva alebo nesk\u00f4r. 2) P\u00f4rod do 48 hod\u00edn po vyhodnoten\u00ed, stabiliz\u00e1cii klinick\u00e9ho stavu matky a lie\u010dbe kortikosteroidmi. Po\u010das 27 a\u017e 34 t\u00fd\u017ed\u0148ov tehotenstva sa t\u00e1to mo\u017enos\u0165 jav\u00ed ako najvhodnej\u0161ia vo v\u00e4\u010d\u0161ine pr\u00edpadov. 3) Pred\u010dasn\u00e1 lie\u010dba dlh\u0161ie ako 48 \u2013 72 hod\u00edn u tehotn\u00fdch \u017eien pred 27. t\u00fd\u017ed\u0148om tehotenstva. V tomto pr\u00edpade sa tie\u017e pou\u017e\u00edva lie\u010dba kortikosteroidmi(1). Lie\u010dba HELLP syndr\u00f3mu je zalo\u017een\u00e1 na sympt\u00f3moch syndr\u00f3mu, a teda pacientkam s hypertenziou s\u00fa pod\u00e1van\u00e9 lieky na hypertenziu a pod. Preto je potrebn\u00e9 sledova\u0165 \u010derven\u00e9 krvinky, krvn\u00e9 do\u0161ti\u010dky a hladiny pe\u010de\u0148ov\u00fdch enz\u00fdmov. Av\u0161ak jedinou spo\u013eahlivou lie\u010dbou je p\u00f4rod. Pacientky s gesta\u010dn\u00fdm vekom ni\u017e\u0161\u00edm ako 34 t\u00fd\u017ed\u0148ov, ktor\u00e9 s\u00fa klinicky stabiln\u00e9, m\u00f4\u017eu p\u00f4rod oddiali\u0165 na 36 a\u017e 48 hod\u00edn a v tomto \u010dase s\u00fa im pod\u00e1van\u00e9 kortikosteroidy na matur\u00e1ciu p\u013e\u00fac plodu. Lie\u010dba HELLP syndr\u00f3mu s kortikosteroidmi, zameran\u00e1 na pulmon\u00e1rnu matur\u00e1ciu plodu a obnovenie po\u010dtu krvn\u00fdch do\u0161ti\u010diek matky je be\u017en\u00e1, ale jeho klinick\u00fd v\u00fdznam zost\u00e1va zatia\u013e nejasn\u00fd(6).<\/p>\n

 <\/p>\n

Z\u00e1ver <\/strong><\/p>\n

Hoci presn\u00e1 patofyziol\u00f3gia HELLP syndr\u00f3mu je st\u00e1le nezn\u00e1ma, existuj\u00fa viacer\u00e9 te\u00f3rie. Je d\u00f4le\u017eit\u00e9 vedie\u0165 rozpozna\u0165 faktory, ktor\u00e9 prispievaj\u00fa k HELLP syndr\u00f3mu. V\u010dasn\u00e9 rozpoznanie a diagnostika s\u00fa d\u00f4le\u017eit\u00e9 pre lie\u010dbu matky aj die\u0165a\u0165a s cie\u013eom zn\u00ed\u017eenia komplik\u00e1ci\u00ed, mortality a morbidity. Pochopenie vplyvu HELLP syndr\u00f3mu na plod, klinick\u00e9 pr\u00edznaky, diagnostick\u00e9 hodnotenie novorodenca aj terapeutick\u00e9 pr\u00edstupy a mo\u017enosti lie\u010dby by mohli pom\u00f4c\u0165 pri n\u00e1slednej starostlivosti o die\u0165a hne\u010f po p\u00f4rode. Napriek v\u0161etk\u00fdm ned\u00e1vnym pokrokom vo svojej v\u010dasnej diagnostike a lie\u010dbe je HELLP syndr\u00f3m st\u00e1le z\u00e1va\u017enou komplik\u00e1ciou tehotenstva ohrozuj\u00facou \u017eivot. Ke\u010f\u017ee jeho patogen\u00e9za nebola \u00faplne objasnen\u00e1, HELLP syndr\u00f3m zost\u00e1va komplexnou diagnostickou a terapeutickou v\u00fdzvou. Hoci je nevyhnutn\u00e9, aby vysokorizikov\u00e9 tehotn\u00e9 \u017eeny boli d\u00f4kladne sledovan\u00e9, aby sa zabr\u00e1nilo rozvoju HELLP syndr\u00f3mu, je ve\u013emi d\u00f4le\u017eit\u00e9 pos\u00fadi\u0165 potrebu poradenstva pre pacientky pred tehotenstvom, najm\u00e4 v pr\u00edpadoch s pozit\u00edvnym v\u00fdvojom preeklampsie a HELLP syndr\u00f3mu v predch\u00e1dzaj\u00facom tehotenstve.<\/p>\n

 <\/p>\n

LITERAT\u00daRA <\/strong><\/p>\n

    \n
  1. Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. A Review. BMC pregnancy and childbirth 2009; 9: 1-15.<\/li>\n
  2. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. 1982. Americam Journal of Obstetrics and Gynecology 2005; 142: 159-167.<\/li>\n
  3. Barnhart L. Syndrome and the Effects on the Neonate. Neonatal network 2015; 34: 269-273.<\/li>\n
  4. Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count(HELLP): a review. Eur J Obstet Gynecol Reprod Biol. 2013; 166: 117-23.<\/li>\n
  5. Jebbink J, Wolters A, Fernando F, et al. Molecular genetics of preeclampsia and HELLP syndrome \u2013 a review. Biochim Biophys Acta 2012; 1822: 1960-1969.<\/li>\n
  6. Dusse LM, Alpoim PN, Silva JT, et al. Revisiting HELLP syndrome. Clin Chim Acta 2015; 7: 117-120.<\/li>\n
  7. Sibai BM1. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol 2004; 103: 981-991.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"

    *All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf attachment at the end of the paper.   \u00davod HELLP (hemolysis, elevated liver enzymes, low platelet count) syndr\u00f3m je z\u00e1va\u017enou komplik\u00e1ciou tehotenstva, ktor\u00fd sa vyskytuje u 0,2 \u2013 0,8 % tehotn\u00fdch \u017eien a v 70 \u2013 80<\/p>\n","protected":false},"author":7,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_mi_skip_tracking":false,"footnotes":""},"categories":[290],"tags":[1135,1136,571],"class_list":["post-1691","post","type-post","status-publish","format-standard","hentry","category-genetics","tag-hellp-syndrome","tag-hemolysis","tag-preeclampsia","typ_clanku-review-article"],"acf":{"abstrakt":"

    HELLP syndrome is a pregnancy-related disease associated with a severe form of preeclampsia; the term HELLP is an abbreviation used to describe a clinical condition that leads to hemolysis, elevated liver enzymes and low platelet count. The etiology of HELLP syndrome is not fully elucidated yet, but the syndrome is associated with serious clinical complications that can lead to the death of both the mother and the foetus and it is, therefore, necessary to find a way to diagnose the disease more quickly and find appropriate clinical intervention. HELLP syndrome is most frequently diagnosed between the 27th and 37th weeks of pregnancy, but it can also be recorded postpartum. <\/p>\n

    Keywords: HELLP syndrome, preeclampsia, hemolysis <\/p>\n","casopis":[{"ID":1633,"post_author":"7","post_date":"2019-05-09 08:56:48","post_date_gmt":"2019-05-09 06:56:48","post_content":"

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