?????????????S ?????????S S??? ????OS? ??? ??? ??????? ?????????? ????? S??????? ?ef??????? ??e????t??a ?ef???????? t

1
?????????????S ?????????SSE ????OS? -
??????? ??????????
????? S??????? ?ef???????? tµ?µa G?? ?.
F??µ????
2
????OS? ?????S ?????? ??t????p??
3

• ????OS? ?????S ???????µ???
• ?????a
• p???de?t???
• ???s?
• ?????
• ???????sµ??
• ?????a ?pat?t?da ?
• ?????a ?pat?t?da C
• ???t?pa??? ?????? ?????s?
• ???t?pa??? s??????t??? ???a??e??t?da
• ??t????se? ?pat?t?de?
• ???????µ??? a?µ????µ?t?s?
• ??s?? Wilson
• ??ep???e?a a1-a?t?????????
• ??p?d?? ?pat?p??e?a
• F??µa?a (?µ??da????,µe??t?e??t?,??t??f???a?t????)
  
• ?????a s?µf???t??? ?a?d?a?? a?ep???e?a

• ??????? ??????????
• ?????a
• ??e?a
• ?????
• ??e?e? ?pat?t?de? ?, B, C,
• ??e?e? ?pat?t?de? ap? ?????? ????
• (p? CMV, EBV)
• ??t?d?as? ?pe?e?a?s??s?a? se f??µa?a (p?
  tet?a??????e?)
• ?pe?d?s?????a pa?a?etaµ????
• ????t???as? µe µa??t???a

4
?????OS??S ???????S ??S?? S??????????S ?? ???
???????G??
?a?t????? Kayser Fleisher (??s?? Wilson)
?pat?µe?a??a (a??????sµ??, ??p?d?? d????s?,
a?µ????µ?t?s?)
S?spas? Dupuytren (a??????sµ??)
?e??fe???? ?e???p??e?a (a??????sµ??, f??µa?a)
5
?????OS??S ???????S ??S?? S??????????S ?? ??
???G?OS?
?????µa pa?aµ??
????t?da?t???a
G??a???µast?a
??a???e?de?? t??ea??e?e?tas?e?
6
?????OS??S ???????S ??S?? S??????????S ?? ??S
????????S

• S???et??? d?s?e?t?????a
• ?p?a?ß??µ??a?µ?a
• ??ata?a??? p??t???t?ta?

?e???????a
?e??fe???? ??d?µa
????pe?
7
?????OS??S ???????S ??S?? S??????????S ?? ??S
????????S

• ???a?a ?p??tas?

?pe?sp????sµ?? µe ? ????? µe?a??sp????a
?s??t??
?efa?? µ?d??sa?
???s?? ??sf????
???s?? ?????
8
?????OS??S ???????S ??S?? S??????????S ?? ??S
????????S

• ?pe????t??? d?s?e?t?????a
• ?p?t?µa
• ?pat?p?e?µ????? s??d??µ?

??te???
9
?????OS??S ???????S ??S?? S??????????S ?? ??S
????????S

• ?pat??? e??efa??p??e?a
• ?pat??ef???? s??d??µ?

10
????OS? ?????S / ??????? ??????????
?????????????S ?????????S

• ?????????????
• ????????????
• ?????G??S??????
• ???FOSF???????
• ??????????????

11
????OS? ?????S / ??????? ???????????????????????

• ? s????te?? ??e?t????t??? d?ata?a??
• ??????st???? de??t?? ?a??t?? st? te???? st?d??
  ?pat???? ??s??
• p??a?a ?p??tas?
  ?pat??? a?ep???e?a
• 
  µe??µ??? ?e?t?????a ??S
• a?epa???? ap?µ?????s? ap????f?s?
  e?d?t?????? G?S
• ?pe?pa?a???? ??, p??sta??a?d????...
• a??e??d?ast???
• µe??s? a??e?a??? a?t?st?se??, ??, ???

12
????OS? ?????S / ??????? ???????????????????????

Gines P, Schrier RW, NEJM 2009
13
????OS? ?????S / ??????? ???????????????????????
???????F???? S???????

• ??????S???
• ?GG???S?S??S?S
• ????G?????S?
• S?S
• S?st?µat?? ???
• ADH
• ?ata???t?s? Na
• ?ata???t?s? e?e?????? H2O
• ?????????????
• ?e??s? ?ef????? pa?????
• - ???
• - ???????F???? S???????

Wadei HM, JASN 2006
14
??????????? ????S? ??S ?????S?S ?O? ?????O? ??
??? ???????? ??S ????OS?S (?S????S, ??F????
??????????)

• ?a?at??e?ta? ep? - pt?s?? ??
  89 75 mmHg - µe??s?? sNa 138
  128 mEq/L

(ng/mL per hour
pg/mL
pg/mL
Asbert M et al, Gastroenterology 1993
15
????OS? ?????S / ??????? ???????????????????????

• ???????O??S?
• ???
• ???
• ???

• ??????OS??S
• ?????a µe??s? sNa ??? 120 mEq/L
• ?s?µpt?µat???
• -?a?e?a µe??s? sNa (???µ??e??...)
• -?e??s? sNa ??t? t?? 120 mEq/L
• S?µpt?µat???
• - ??aµ??? ??a µetaµ?s?e?s?

Gines P, Guevara M. Hepatology 2008
16
????OS? ?????S / ??????? ???????????????????????
???????O??S?

• ??T??S ????TOS?S 1? 24??? lt 10mEq/l
• 2? 24??? lt 9mEq/L
• ?????
• ?e?????sµ?? ?????
• ??????s? s???p?????sa? ?p??a??a?µ?a?
• ?p??t??? NaCl 3
• ??ta????st?? ?p?d????? ßa??p?ess????
• Per os tolvaptan, satavaptan, lyxivaptan
• IV konivaptan
• Schrier RW et al, N Engl J Med 2006
• ??ep???µ?te? e????e?e?
• s???????a, p???????a, d??a, ????st?µ?a,
  ??p?s?,
• ?pe?ta?e?a d?????s? - ?sµ?t???
  ap?µ?e????t??? s??d??µ?,
• ????? ??st??
• Berl Tet al, J Am Soc Nephrol 2010
• ?eµe??????????
• Miller PD et al, JAMA 1980

17
????OS? ?????S / ??????? ??????????????????????

• ?????
• ??????t??? ap??e?a ? sta ???a
• ???????e?-?pa?t??? / ?µet?? ap??e?a ? ap?
  G?S
• ?p????a?µ?a - e?e???p???s? S?S / ß2 ad?e?e??????
  ?p?d????? e?s?d?? ? sta ??tta?a

18
????OS? ?????S / ??????? ???????????????G??S????
??

• ?????
• ?????a d??????a
• ap??e?a Mg ap? G?S
• ?p?s?t?sµ??
• µe??µ??? p??s???? Mg
• ??????t??? a?????? ? ?e?a??de?
• s????a??a?? epa?a???f?s? Mg

• ???????O??S?
• ?a???s??t???t???
• d?????t???
• ??????s? Mg
• (ep? e?d???tta???? e??e?µµat??)
• 50 mEq st??d?? IV se 8-24 h
• aspa?t??? Mg (TROFOCARD )
• amp 5ml 60,77 mg Mg 5 mEq

19
????OS? ?????S / ??????? ?????????????FOSF??????
?

• ?????
• ?e??µ??? e?te???? ap????f?s?
• ?p???e??a µe??µ??? p??s???? ? ?a?
  ß?taµ???? D
• ?????a d??????a
• ?p?t??p?????sa ?ast??t?da/a?t?????a
  d?sµe?s? ? t??f??
• ????µ??? ap?????s? ?
• ??ep???e?a Vit D 2pa??? ??T
  f?sfat????a
• ?µes? d??s? t?? ????p?e?µat??
• De Marchi S et al. N Engl J Med 1993
• ?eta????s? ? sta ??tta?a
• ??d???tt???? ???e?µµa d?af??? s?????t??s??
• Sa??a?????? ????, epa?as?t?s? ?????s? ??s???????
• ??a??p? ????p?e?µat?? ?pe?ae??sµ??
• ??e?a a?ap?e?st??? a?????s? e?s?d?? CO2
  sta ??tta?a e?d???tt???a a?????s?
  d???e?s? f????t?????s?? a???s? ???????s??
  e?s?d?? ? sta ??tta?a
• Ferguson ER et al, Am J Physiol 1984
• Marinella MA. J Intensive Care Med 2005

20
????OS? ?????S / ??????? ?????????????FOSF??????
?

• ?????OS??S
• ???p??e?a
• d???t??? ?aßd?µ????s?
• ??ap?e?st??? a?ep???e?a
• ?e?????????? d?ata?a???
• (ape?e?????s? ? ap? t??? µ??)
• Knochel JP. Arch Intern Med 1977

• ???????O??S?
• ?p?f??? sa??a?????? ????
• ??????s? a?ep???e?a? Vit D
• ??????s? ?O4-3 15-20 mg / kg / 24h
• 0,02 mmol / kg / 24h
• - IV ep? sPlt1 µ???? ep?te????
• sP1,5 mg/ dl
• GLYCOPHOS sol 20 ml 20 mmol ?O4-3 1900 mg
  se 500 ml NaCl 0,45
• - Per os ep? sP 1-1,9 mg/dl
• Stop ep? sP 2 mg/dl
• PHOSPHATE tabl eff 500 mg ?O4-3 315 mg KHCO3
  (3,1 mEq K) 315 mg NaHCO3 (20mEqNa) 800mg
  ??t????? ?????
• 
  De Marchi S et al. N Engl J Med 1993

sp???e?
21
????OS? ?????S / ??????? ???????????????????????
?

• ?p? ?e?a???ß???? ??s??
• ?????
• ??????s? ?p??t???? d?a??µat?? f????t????
• ?sµ?t??? d?????s? af?d?t?s?
• Wilkinson SP et al. British Medical Journal
  1974

22
??????S?O G?? ??? ???S??? S?S