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DEFINITIE
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Insuficienta hepatica acuta este un sindrom clinico-biologic de etiologie diversa, ce apare in urma necrozei hepatocitare masive, compromitand functiile ficatului: detoxifiere, endocrina si metabolica. |
ETIOLOGIE
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Cauze |
Varsta |
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< 3 luni |
> 3 luni |
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Infectioase |
virus hepatic B virus hepatic C virus hepatic A virus Ebstein-Barr virus citomegalic virus herpes simplex rubeola congenitala lues congenital |
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Metabolice |
tirozinemie ereditara galactozemie intoleranta la fructoza boala Wilson |
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Vasculare |
ficatul din soc boala veno-ocluziva |
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Autoimune |
hepatita autoimuna cu celule gigante si anemie hemolitica alte hepatite autoimune |
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Maligne |
leucemie acuta infiltrare hepatica maligna |
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Toxice |
Paracetamol ciuperci necomestibile Halotan Acid valproic Izoniazida Rifampicina |
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frecvent ++ putin frecvent + rar + foarte rar
ANAMNEZA
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antecedente heredocolaterale sugestive antecedente personale patologice: boli cu posibila afectare hepatica medicatie hepatotoxica in antecedente debut la 8 saptamani de la simptomele specifice si nespecifice |
CLINIC
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semne nespecifice: alimentatie dificila (sugar), anorexie, varsaturi, dureri abdominale, icter progresiv, foetor hepatic encefalopatie hepatica (vezi stadializare) sindrom hemoragipar: hemoragii cutaneo-mucoase, digestive, cerebrale; CID + fibrinoliza (faze tardive) |
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Stadializarea clinica a encefalopatiei hepatice |
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Stadiul |
Semne clinice |
EEG |
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I |
perioade de letargie, confuzie, euforie, tulburari somn-veghe |
Normal |
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II |
dezorientare temporo-spatiala, agitatie alternand cu somnolenta, tulburari de comportament, asterix |
Ritm lent, unde θ |
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III |
stupoare (trezire doar la stimuli), poate raspunde la stimuli auditivi dar mai slab la stimuli vizuali, hiperreflexie |
Ritm foarte lent |
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IV |
A |
coma profunda (flasca), raspunde la durere prin postura de decorticare / decerebrare, reflexe arhaice +, reflex cornean + / reflex fotomotor +, convulsii |
Unde δ de amplitudine scazuta |
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B |
fara raspuns la durere |
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PARACLINIC
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Sange |
Urina |
Alte investigatii |
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HLG glicemie (scazuta) ionograma (hiponatremie, hipocalcemie) ASTRUP TGP, TGO (1000 VN) γ-GT, BT, BD, amoniemie (crescuta) hemostaza: TP, INR, TPTA, timp proconvertina, timp proaccelerina, fibrinogen electroforeza (hipoalbuminemie) lipidograma α-fetoproteina uree, creatinina (crescute) serologie virala toxicologie |
sumar ionograma toxicologie |
toxicologie aspirat gastric radiografie toracica ecografie abdominala ECG ecografie cardiaca EEG CT craniu |
DIAGNOSTIC DIFERENTIAL
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come neurologice / come de alte etiologii intoxicatii cu droguri psihotrope coagulopatii de alte etiologii sindrom Reye soc hemoragic |
EVALUAREA SEVERITATII (in functie de IP)
Forma |
IP (% din VN) |
Usoara |
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Medie |
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Severa |
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Letala |
< 10 |
Factori de prognostic sever (criterii de transplant hepatic)
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encefalopatie stadiul III sau IV icter prelungit cu BT > 15 mg% INR > 4 TP > 60 sec, necorectabil dupa administrare de vitamina K1 TPT > 20 sec fata de martor hipoglicemie severa acidoza metabolica severa albumina serica < 2,5 g% ascita refractara la diuretice hemoragie din varice esofagiene necontrolata cu scleroterapie colesterolemie < 100 mg% |
COMPLICATII
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edem cerebral hemoragii masive hipoglicemie IRA sindrom hepato-renal tulburari electrolitice, acido-bazice infectii (respiratorii, urinare) insuficienta respiratorie acuta tulburari de ritm cardiac hipotensiune arteriala deces |
MONITORIZARE
Clinic ■ |
Admisie |
Orar |
2h |
6h |
12h |
24h |
Paraclinic □ |
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TA |
□ |
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HLG |
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FC |
□ |
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Ionograma serica |
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FR |
□ |
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ASTRUP |
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SpO2 |
□ |
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Uree, creatinina serica |
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PVC |
□ |
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Glicemie |
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Diureza |
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TGP, TGO, g-GT |
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Status neurologic |
□ |
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BT, BD |
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Bilant hidric |
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□ |
Amoniemie |
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Greutate |
□ |
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□ |
Hemostaza |
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Temperatura |
□ |
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□ |
ECG |
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Ecografie abdominala |
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□ |
EEG |
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encefalopatie hepatica hemoragie digestiva insuficienta renala insuficienta respiratorie infectii severe tulburari ale coagularii hipoglicemie dificil de corectat acidoza lactica |
TRATAMENT
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confort termic (tendinta la hipotermie) nevoi hidrice: 1200 - 1500 ml /m2/zi - Glucoza 10 % - Na+: 1-2 mEq/kg/zi - K+, Ca++: dupa ionograma - NaHCO3 la pH < 7,10 - Tiamina, Piridoxina evita: sedative (Diazepam), corticoterapia (exceptie hepatita autoimuna) antibioterapie profilactica |
Masuri generale
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Combaterea hipoxiei Glasgow < 8 encefalopatie stadiul III, IV se evita in caz de coagulopatie severa Medicatie folosita pentru intubatie: Lidocaina 1 mg/kg iv (previne spasmul glotic si cresterea PIC-ului) Tiopental 1-5 mg/kg iv Rocuronium 0,6 mg/kg iv Ventilatie mecanica cu: VC 10 - 12 ml/kg PEEP = 2 mentinerea PaCO2 = 30-40 mmHg si pH < 7,50 |
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Encefalopatie hepatica Lactuloza 0,4-0,5 g/kg po la 1-2h pana la aparitia scaunelor apoi 0,25 g/kg la 6-8h Neomicina 100 mg/kg/zi po la 6h; maxim 4 g/zi Arginina Sorbitol 10-20 ml/kg/zi Flumazenil 20 μg /kg iv bolus, apoi 5 μg/kg/h pana la superficializarea comei limitarea aportului proteic < 0,5 g/kg/zi |
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Edem cerebral Manitol 0,25 g /kg iv la 6h |
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sange proaspat 10-20 ml/kg
Sindrom hemoragipar
MT (cand trombocitele < 50.000/mm3) 2 ui/10 kg PPC 10-15 ml/kg rFVIIa 60-120 μg/kg iv la 2-3h sau 10-20 μg/kg/h |
BIBLIOGRAFIE
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2. Alper G, Jarjour IT, Reyes JD,et al. Outcome of children with cerebral edema caused by fulminant hepatic failure. In: Pediatr Neurol, 1998, 18(4): 299-304.
3. Arain Z, Rossi TM. Gastrointestinal bleeding in children: an overview of conditions requiring nonoperative management. In: Semin Pediatr Surg, 1999, 8(4): 172-180.
4. Armon K, Stephenson T, MacFaul R, et al. An evidence and consensus based guideline for acute diarrhea management. In: Arch Dis Child, 2001, 85(2): 132-142.
5. Bizo A. Coagularea intravasculara diseminata. In: Urgente majore in pediatrie. Butnariu A, Bizo A, Miresteanu S. Editura National, Bucuresti, 2001: 350-355.
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14. Irish MS, Caty MG, Azizkhan RG. Bleeding in children caused by gastrointestinal vascular lesions. In: Semin Pediatr Surg, 1999, 8(4): 210-213.
15. Liebelt EL. Clinical and laboratory evaluation and management of children with vomiting, diarrhea, and dehydration. In: Curr Opin Pediatr, 1998, 10(5): 461-469.
16. McKiernan PJ. Treatment of variceal bleeding. In: Gastrointest Endosc Clin N Am, 2001, 11(4): 789-812.
17. Milla P, Cucchiara S, Di Lorenzo C, et al. Motility disorders in childhood: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology and Nutrition. In: J Pediatr Gastroenterol Nutr, 2002, 35 Suppl 2: 187-195.
18. Morali A. Gastrointestinal hemorrhages in children. In: Rev Prat, 1998, 15, 48(4): 411-415.
19. Peters JM. Management of Gastrointestinal Bleeding in Children. In: Curr Treat Options Gastroenterol, 2002, 5(5): 399-413.
20. Popescu V. Gastroenterologie. In: Algoritm diagnostic si terapeutic in pediatrie. Editura Medicala Amaltea, Bucuresti, 1999: 273-301.
21Rapaport SI. Coagulation problems in liver disease. In: Blood Coagul Fibrinolysis, 2000, 11 Suppl 1: 69-74.
22. Rayhorn N, Thrall C, Silber G. A review of the causes of lower gastrointestinal tract bleeding in children. In: Gastroenterol Nurs, 2001, 24(2): 77-82; 82-83.
23. Rosen P, Barkin RM. Emergency Medicine: Concepts and Clinical Practice. (IVth edition) Mosby, 1998: 1200-1212.
24. Serban M, Tepeneu P, Petrescu C, et al. Evaluarea eficientei clinice a rFVIIa in pediatrie. In: Documenta Haematologica, vol 6, nr 2, 2000: 59-65.
25. Treem WR. Fulminant hepatic failure in children. In: J Pediatr Gastroenterol Nutr, 2002, 35 Suppl 1:33-38.
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