????????? ???????T???

1
????????? ???????T???

• ??. ???a?t?f????? ??d???e???
• ??aß?t??????? ???t?? ?????pa?de?t????
  ?a????????? ???????? ???st?te?e??? ?a?ep?st?µ???
  Tessa???????, ?pp????te?? ??s???µe??

2
????????? ???????T???
?e t?? ??? d?aß?t??? ?e???p??e?a ??e?ta? µ?a
?µ?da ???????? s??d??µ??, ta ?p??a e?te t? ?a???a
ap? µ??? t??, e?te se s??d?asµ? µeta?? t???
p??sß????? d?af??et??? tµ?µata t?? ?e??????
s?st?µat??.
3
??????? ?at?ta?? ??aß?t???? ?e???p??e?a?

• ??e?e? ??s?µe? ?e???p??e?e?
• ?????e???p??e?e?
• a) ?e???p??e?a µ???a??? ?e????
  (d?aß?t??? aµ?at?f?a)
• ß) ?a?a??se?? ??a??a??? ?e???? ( III,
  VI )
• ?) Ste?e??a?e? ?????e???p??e?e?
• ??e?a d????t? ep?d???
• ??e?a ep?d??? ??s???????? ?e???p??e?a
• 2) ?a?a??se?? ?e???? ???? p?es??
• a) ??s? ?e??? (s??d??µ? ?a?p?a??? s????a)
• ß) O????? ?e???
• ?) ?????? ????a?? ?e??? (sp???a)
• 3) ????de?t??? ep?de????µe?e? ?e???p??e?e?
• a) ?????t? s?µµet???? pe??fe????
  p????e???p??e?a
• ß) ???e?t??? ?e???p??e?a µ????? ????
• ?) ?e???p??e?a a?t???µ?? s?st?µat??

4
??ass??? ??aß?t??? ?e???p??e?a
?????t? s?µµet???? pe??fe????
p????e???p??e?a (?.?.?.)
?e???p??e?a a?t???µ?? s?st?µat??
(????S)
5
?a?????e?a ??aß?t???? ?e???p??e?a?
6
S????t?ta d?aß?t???? ?e???p??e?a?

• 10 - 90 t?? d?aß?t???? p????sµ??
• S????t?s? t?? ???µ?s?? ?a? t??
• d????e?a? t?? Sa??a??d? ??aß?t?

7
St?d?a ??aß?t???? ?e???p??e?a?

• 1) ?0 ??? ?e???p??e?a
• 2) ?1 as?e??? ?O??S s?µpt?µata, a??? µe
• ??e?t??f?s????????? d???µas?e?
  pa?????????
• ? d???µas?e? ??S pa?????????
• 3) ?2 as?e??? ?? s?µpt?µata d???µas?e?
• ?.?.S. ?a? ?.?.S. pa?????????
• 4) ?3 ?a?e?a ?e???p??e?a d?ata?a???
  ????t????
• s?st?µat??.

8
??????? ?????? ?.?.?.
?a?µ?a?a ??a??? µe p???de?t???
ep?de????µe?? e??????     ?????a, d????t?,
s?µµet????, a?s??t???????t???, ??????
sta ??t? ???a d???? ???tsa?         ??µ?d?e? ,
?a?a?s??s?e?         ????d???a,
?pe?a?s??s?e?         ??s??µa ?????t?ta? t??
?????         ?p?d??? s??d??µ?        
?e???pa??t??? ?????, pa?aµ??f?s? ?????        
?????se?? Charcot
9
???G?OS? ?????????S ????F?????S ???????T???S
(?.?.?.)

• ??e???? a?s??t???t?ta?
• ?a??a?s??s?a
• ??s??s? af??
• ??s??s? ?e?µ?? - ??????
• ??s??s? p????
• ??s??s? ??s?? ?a? ????s?? t?? µe??? st?? ????
• 2. ??e???? te???t??? a?ta?a??ast????
• 3. ??e???? ????t???? s?st?µat??
• 4. ??e?t??f?s????????? d???µas?e?

10
?a?a?t???st??? ?e???pa??t???? ?a?
?e????s?a?µ???? ??????
11
???????S ???O?????S??? ??GO ?????????S
???????T???S

• ?????eta? ? ?? ??a t? 50 - 75 t??
• µ?-t?a?µat???? a???t???asµ??
•      1.7 f???? µe?a??te???
•      12 f???? e?? s???p???e? pa?aµ??f?s?
•      36 f???? e?? ?p???e? a???t???asµ??

12
???????T?G??????? T??????? ?????????S
????F?????S ???????T???S

• ???st?? µetaß?????? ??e???? t?? ??aß?t?
• ??ast??e?? µetat?ept???? e???µ??
• ??ast??e?? a?a????s?? t?? a?d????
• a lipoic acid
• ? linolenic acid
• ??ast??e?? Protein Kinase C
• C peptide

13
S????O?????? T??????? ?????????S ????F?????S
???????T???S

• ????? a?a???t???
• ?? ste??e?d? a?t?f?e?µ???d?
• ?????????? a?t??ata???pt???
• ??t?ep???pt???
• Mexiletine
• ?p??e?d?, Tramadol
• Capsaicin

14
????????? ???????T??? ????O? ??O?

• ???sß??? µ????? ???? (a?s??s? p????,
• ?e?µ??, ?e?t?????a ?.?.S.)
• F?e??µe?a ???a
• F?s???????? te???t?a a?ta?a??ast???,
• ??e?t??f?s????????? ??e???? ?a? pa??a?s??s?a
• ???a?????e?ta? ?pa??? a?t????s?? µ??a??sµ??

15
??T?OS? CHARCOT

•   ?atast??f? t?? p?d????µ???? ?????s??
•    ?e???p??e?a ?.?.S. ?a? ?.?.S.
• ?p??s?a pe??fe????? a??e??p??e?a?
• ?a?aµ??f?s? ?????, ?t??a ????,
• a???t???asµ??

16
????????? ???????T??? ?.?.S. ??? ????? ????S

• ???ß? s?µpa??t???? ?.S.
• ??d?a ???? ???? ???e???? ef?d??s?? ?
• s??se?? d??µat??
• ??ata?a??? ?????f???a? a?µat?? ? d??tas?
• f?eß??, d??????? a?t????f?eß????
• a?ast?µ?se??, ???? ?e?µ?

17

pe????af? pe??pt?se??

• ???a??a 55 et??
• S? t?p?? 2 ap? 10et?a? ?p? ??s???????e?ape?a
• pe??fe???? ?e???p??e?a
• ßa?? ?t??? ????? d?aµ?t??? 4 cm st?? a??ste??
• pt???a, ??????????µe?? ap? 3µ????, µe
• s?µpt?µat?????a ???µ????
• ap??? a?t?????af?e? t?? a??ste??? ????? p?d??
• a???t???? ??a ?ste?µ?e??tda
• ?e?d?µ???da st?? ?a??????e?a t?? p??d???
• e????µat?? t?? ??????

18
a
ß
a, ?a??a?a ?p??? ß, pe?µat?a?a ?p??? a??ste???
????? p?d?? ?, d?aß?t??? ?t??? ????? st??
pe?µat?a?a ep?f??e?a t?? a??ste??? pt???a?
?
19
??t?????af?e? a??ste??? ????? p?d??
20
???aµ??? f?s? (pe?µat?a?a)
Sp?????????f?µa ?st?? µe 99mTc-MDP
R
L
L
R
F?s? a?µat???? de?aµe???
Stat???? ???e?? 3 ????
21
Sp?????????f?µa ????? p?d?? µe 99mTc-HMPAO-?e???
a?µ?sfa???a
22
???????? e?d???se?? ??aß?t???? ?e???p??e?a? t??
??t???µ?? ?e?????? S?st?µat?? (????S)
???e ???a?? ? s?st?µa t?? a????p???? s?µat??,
t? ?p??? d??eta? ?e???s? ap? t? a?t???µ? ?e?????
s?st?µa ( pa?as?µpa??t??? s?µpa??t???) µp??e?
?a p??sß???e? ap? a?t? t? µ??f? ?e???p??e?a?.
23
(No Transcript)
24
??????? s?µas?a ????S

• ??t??? st?? ?s??s?
• 2) ?e??e??e???t??? ???t?t?ta ?a? ???s?µ?t?ta
• 3) ????µ???? ?a?d?a??e?a???
• ???d???? ?a??t??

25
?ta? ? ??aß?t??? ?e???p??e?a t?? ??t???µ??
?e?????? S?st?µat?? (????S) e??atasta?e?,t?te ?
??? t?? as?e???? ???eta? e?a??et??? d?s???? ?a? ?
s????t?ta ?a??t?? a????eta? st? 25 - 50 µ?sa
sta ep?µe?a 5 10 ?????a
26
(No Transcript)
27

• ??ata?a??? t?? ?a?d?a??e?a??? a?ta?a??ast????
  d???µas??? a?e???s???ta? st? 16 - 20 t??
  d?aß?t???? as?e???.
•  
• ??s?e?t?????a pa?as?µpa??t????
•         20 sta 5 ?????a d????e?a? S?
•         65 sta 10
•  
• ??s?e?t?????a s?µpa??t????
•         7 sta 5 ?????a d????e?a? S?
•         24 sta 10
  

28
?a?d?a??e?a?? s?st?µa ?a? ????S (1)

• ?a???a?d?a ??eµ?a?
• ?e??µ??? µetaß??t?t?ta ?a?d?a???
• s????t?ta?
• ??aß?t??? µ???a?d??p??e?a (d?ast?????
• ? ?a? s?st????? d?s?e?t?????a a??ste???
• ?????a?)
• ?e??µ??? a?t??? st?? ?s??s?
• S??p??? ?s?a?µ?a µ???a?d??? / s??p???
• ?µf?a?µa µ???a?d???

29
?a?d?a??e?a?? s?st?µa ?a? ????S (2)

• ????µ???? ???d???? a?f??d??? ?a??t??
• ???? eµf???s?? ?a?????? ?????a???
• a????µ???
• ????stat??? ?p?tas?
• ??ata?a?? st?? ?µe?????t?a d?a??µa?s?
• t?? ?a?d?a??? s????t?ta? ?a? t??
• a?t???a??? p?es??

30
??as???? ?a?d?a??e?a??? a?a??ast???? d???µas?e?
e?????? t?? ?e?t?????a? t?? ??t???µ?? ?e??????
S?st?µat??

• ????µas?a µetaß??t?t?ta? t?? ?a?d?a??? s????t?ta?
  ?at? t?? d????e?a ßa???? a?ap????
• a)?e??t?? ??p????/??sp???? (?/?)
• ß)??s?? a??sµat???? de??t?? t?? ????????
• µetaß???? t?? R-R d?ast?µ?t??
  (a??sµat???
• a????s?)
• ?) Sta?e?? ap????s? ( SD)
• ????µas?a Valsalva (?e??t?? Valsalva)
• 3. ????µas?a µetaß??t?t?ta? t?? ?a?d?a???
  s????t?ta? ap? t?? ?pt?a st?? ????a ??s? (?e??t??
  3015)
• 4. ????stat??? ?p?tas?

31
F?s???????? ????µas?a µetaß??t?t?ta? t??
?a?d?a??? s????t?ta? ?at? t?? d????e?a ßa????
a?ap????. Inhale??sp???, Exhale ??p??? ?e??t??
??p???? / ??sp???? 1.85, ??s?? a??sµat????
de??t?? 130, Sta?e?? ap????s? 215, ?????a
28 et??
32
?a???????? ????µas?a µetaß??t?t?ta? t?? ?a?d?a???
s????t?ta? ?at? t?? d????e?a ßa???? a?ap????.
Inhale??sp???, Exhale ??p??? ?e??t?? ??p???? /
??sp???? 1.02, ??s?? a??sµat???? de??t?? 0,
Sta?e?? ap????s? 8, ?????a 43 et??
33
F?s???????? ????µas?a µetaß??t?t?ta? t??
?a?d?a??? s????t?ta? ?at? t?? d????e?a ßa????
a?ap????. E / I 1.49, MCR 86, SD
117, ?????a 24 et?? , ??p???,
??sp???, 1000 MS 60 bpm, 500 MS 120 bpm
34
F?s???????? ????µas?a µetaß??t?t?ta? t??
?a?d?a??? s????t?ta? ?at? t?? d????e?a ßa????
a?ap???? . ?ata??µ? t?? ?a?d?a??? s????t?t?? ?at?
t?? d????e?a ßa???? e?sp???? (???) ?a? e?p????
(??t?). ?e??t?? ??p???? / ??sp???? 1.55, ??s??
a??sµat???? de??t?? 107, Sta?e?? ap????s?
160, ?????a 31 et??
35
?a???????? ????µas?a µetaß??t?t?ta? t?? ?a?d?a???
s????t?ta? ?at? t?? d????e?a ßa???? a?ap????.
??e?d?µa t?? ?a?d?a??? s????t?ta? st?? 100
sf./?ept?. ?????a 43 et??, E/I Index 1.03,
MCR0, SD8, 1000 MS 60 sf./?ept?
36
?st???aµµa d???µas?a? VALSALVA ??? f?s????????
d???µas?a , ??t? pa???????? d???µas?a
G?aµµ? d?a??µa?s?? ?a?d?a??? s????t?ta?, 1000 MS
60 bpm
37
?st???aµµa d???µas?a? µetaß??t?t?ta? t??
?a?d?a??? s????t?ta? ap? t?? ?pt?a st?? ????a
??s?. ???f?s???????? d???µas?a, ??t? pa????????
d???µas?a. G?aµµ? d?a??µa?s?? ?a?d?a???
s????t?ta?. 1000 MS 60 bpm.
38
????stat??? ?p?tas?

• ?t?s? t?? S?st?????? ?? ? 20 mmHg
• ? ?a? t?? ??ast?????? ?? ? 5-10 mmHg
• ???????µ??a st?d?a ????S
• S?????? as?µpt?µat???
• ?d??aµ?a, ????, d?ata?a??? ??as??,
• s????pt??? epe?s?d?a
• ??af????? d?????s? ap? ?p?????a?µ?a

39
?etaß??t?t?ta t?? ?a?d?a??? s????t?ta?

• Fasµat??? a????s?
• TP ????? e????e?a
• HFP ?????? s????t?ta? e????e?a (t????
  pa?as?µpa??t????)
• LFP ?aµ???? s????t?ta? e????e?a (t????
  s?µpa??t????)
• LFP / HFP ????? ?aµ???? / ?????? s????t?ta?
  e????e?a?
• 2) Stat?st??? a????s?

40
???e? d?a???st???? d???µas?e? ????S

• 24??? µ?t??s? a?t???a??? p?es??
• 2. Sp????????af??? ape?????s? µ???a?d??? µet? ap?
  ??????s? ?ad??ses?µasµ???? ??s??? (MIBG)

41
Sp????????af??? ape?????s? t?? µ???a?d??? µet?
ap? t? ??????s? 123-I ?eta-??d? ße????????a??d???
(MIBG) ?. F?s???????? p??s???? t?? MIBG ap? t?
µ?????d?? ?. s?ßa?? ???tt?s? t?? p??s????? t??
MIBG ap? t? µ?????d?? as?e???? µe Sa??a??d?
??aß?t? t?p??-1
Diabetologia (2000) 43 1455 1469
42
??t??pa???e?et??? ?e?ape?a ????S

• ???st?? µetaß?????? ??e????
• DCCT, Kumamoto study
• 2) ??ast??e?? a?a????s?? t?? a?d????
• 3) ??ast??e?? µetat?ept???? e???µ??
  a??e??te?s???? II
• 4) A Lipoic acid
• 5) ?p???e?st?? ?p?d????? t?p?? 1 t??
  a??e??te?s???? II ( )

43
S?µpt?µat??? ?e?ape?a ????S

• 1) ????stat??? ?p?tas?
• Ge???? µ?t?a
• Fa?µa?e?t??? a???? (f????????t?????,
  e?????p???t???, a?????a s?µat?stat????)
• 2) ?a???a?d?a ??eµ?a?
• ???e?t???? ß-a?ast??e??
• ??-d??d??p???d?????? ??ast??e??
• d?a???? Ca

44
??d???se?? ????S ap? t? ?ast?e?te???? s?st?µa

• Gast??p??es?
• ??s??????t?ta
• ???????a

45
?e?????feta? ? pe??pt?s? as?e???? µe S? t?p??
1 ap? 28et?a?, ? ?p??a p???de?t??? e??at?st?se
??????? s?µpt?µat?????a ßa?e?a? ?ast??p??es?? µe
ap????µ?s? t?? S? . ? as?e??? pa???s?a?e, ep?s??,
ßa?e?a ??????? ?e???p??e?a t?? a?t???µ?? ?e??????
s?st?µat?? (???S). ? ?pa??? t?? ???S d?ap?st????e
µe t?? 4 ??as???? ?a?d??a?ta?a??ast????
d???µas?e?. ?a? ?? 4 d???µas?e? ?ta? pa?????????.
? d?????s? t?? ?ast??p??es?? p?a?µat?p??????e µe
t?? µe??t? ????s?? t?? st?µ???? µet? ap? ??????s?
?ad??ep?s?µasµ???? ?e?µat??. St?? as?e??, µet?
t?? d?????s?, ?????????e s??ap??d? 20mg.x2/24???
??a ??????? d??st?µa 2 ??????. ? µe??t? ????s??
t?? st?µ???? p?a?µat?p??????e a?? ???????
d?ast?µata e??? ?t??? ap? t?? d?????s?. St?? p??
?e?ape?a? µe??t? ? ?p?????sµ?? t?? ??????
?µ?se?a? ????s?? t?? st?µ???? ?p???e p?a?t???
ad??at??, e?? st?? ep?µe?e? d?? µe??te? ? ??????
a?t?? ?ta? 210 min. ? ???S de? pa???s?ase
ße?t??s? ?at? t? a??t??? ??????? d??st?µa, e?? ?
µetaß?????? ??e???? t?? S? ?a? ? ???????
s?µpt?µat?????a t?? ?ast??p??es?? ße?t?????a?.
S?µpe?asµat??? ? ??????s? s??ap??d?? ß????se
s?µa?t??? st?? µa????????a a?t?µet?p?s? t??
ßa?e?a? d?aß?t???? ?ast??p??es??.
46
?????a 1
5/1999 p?o ?e?ape?a?
3/2000 ?p? ?e?ape?a
2/2001 ?p? ?e?ape?a
47
?????a 2
5/1999, p?? ?e?ape?a?
3/2000, ?p? ?e?ape?a
2/2001, ?p? ?e?ape?a
O min
O min
O min
2O9 min
21O min
230 min
48
?????a 3
5/1999 p?o ?e?ape?a?
3/2000 ?p? ?e?ape?a
2/2001 ?p? ?e?ape?a
49
??d???se?? ????S ap? t? ????p????e???t??? s?st?µa

• ?t??? ????d???? ??st?
• Se???a???? a???a??t?ta

50
???e? e?d???se?? ????S

• ??ata?a??? ??as??
• ??ata?a??? ef?d??s?? ?e?µ????µ?s??
• ???e??????t???? d?ata?a???
• ?? a?t????? ?p?????a?µ?a?