???G?OS???? ???S????S? ????S??????S???

1
???G?OS???? ???S????S? ????S??????S???
2
?????F?????

• ?p?s??pe??t??a??? ???a?a
• pe???ef???? pe??t???a
• f????? a?at????t???e?d?, ????????t???e?d?,
  µ????? p?s?t?te? ?e???t???? ??µ????
• µ?e??? t?? ep??ef??d??? e?????e? t??
  ?ate???aµ??e? ep??ef???? ?a? ???ep??ef????.

3

• (1) ? ep??ef??d???? ???d?? t?? ??t? ?ef?????
  a?t???a?
• (2) ? ep??ef??d???? ???d?? t?? a??t??
• (3) ? ep??ef??d???? ???d?? t?? ?ef????? a?t???a?
  .
• ?a ?eµfa??e?a f????ta? p??? ta p????a a??t???
  ?eµf???????a
• p???a????a??? s?µpa??t???? ??e? p?? p???????ta?
  ap? ta sp?a?????? ?e??a

4
F????S ?O? ?????F????O?

• spe???e?d? ????
• st???d?t? ????
• d??t??t? ????
• spe???e?d?? ???? e?????e? µeta??????t???e?d? ?a?
  ?????? a?d?ste????
• ? st???d?t? ???? ?a? p??a??? ? d??t??t? e????????
  ta ????????t???e?d? ???t????? ?a? ???t?????

5
(No Transcript)
6
F?S????G?? ????S??????S

• µ?s? ?µe??s?a ?????s? t?? a?d?ste????? ??µa??eta?
  µeta?? 0.1 ?a? 0.7 µmol (50-250 µg)
• 75 t?? a?d?ste????? ad?a??p??e?ta? µe
  ?ataß???sµ? t?? ? da?t????? t?? µ????? t?? ?a?
  s??e??? t?? µe ??????????d??
• ???sµa e?e??e??? a?d?ste????? se ?aµ??? pH (
  acid-labile) 15-50 nmol (5-19µg )
• s??e??µ???? µe ??????????d?? pa?a????? µeta??
  70-100 nmol
• µ? s??e??µ???? e?e??e??? a?d?ste????? µeta??
  0.5-2 nmol ( 0.2-0.6 µg).

7
d?? ????e? f?s????????? d??se??

• ßas??? ???µ?st??? µ??a??sµ? t?? e????tt?????
  ?????
• p??sd?????e? ?at? ????? ???? t?? µetaß???sµ? t??
  ?a???? µ?s? t?? d?sµe?s?? t?? st?? ?p?d???a t??
  ???t???e?d?? t?p?? ? st??? ?st???-st?????

8

• p????e? t?? ?ata???t?s? t?? ?at???? µe t??
  ta?t?????? ap?ß??? ?a????
• epa?ap??s???? t?? ???t?? ?at???? d?µ?????e? µ?a
  pt?s? t?? d?aµeµß?a????? d??aµ????
• ? epa?ap??s???? t?? ???t?? ?at???? a??????e?ta?
  ap? t?? pa??t??? epa?a???f?s? ?e???
• ?a a?at????t???e?d? d???? ?a? st? ep?????? t??
  s?e??????? ?a? ?d??t?p???? ad???? ?a? st?
  ?ast?e?te???? s????a p???a???ta? ?ata???t?s?
  ?at???? ?a? ap?????s? ?a????.

9

• a????? pe???d?? ?ata???t?s?? ?at????
• ?at??????s?
• f?s???????? ?s?????? epa????eta? µ?sa se 3 µe 5
  ?µ??e?
• t? fa???µe?? t?? d?af???? ?fe??eta? st? µe??s?
  t?? e?a?s??s?a? t?? ?ef????? s????a???? st? d??s?
  t?? a?d?ste?????
• ? ap??e?a t?? ?a???? e??a? d?a???? ?a? de?
  pa?at??e?ta? t?t??? fa???µe?? d?af????
• a??????? ?a? ta ep?peda t?? ?at??????t????
  pept?d??? st? p??sµa
• ep?d?? ?a? p??? se ??a? µeµß?a???? ?p?d???a ?a? ?
  f?s???????? t?? d??s? de? p???a?e?ta? µ??? ap?
  t??? ??tta??p?asµat????? ?p?d??e??

10

• ? ?????s? t?? a?d?ste????? ???µ??eta? ap? t?
  s?st?µa ?e?????-a??e??te?s????
• ta ep?peda t?? ?a????
• t?? ACTH
• H ?e???? d?a p??? st? a??e??te?s???????
• s??µat??e? t? de?apept?d?? a??e??te?s??? ?
• t? µetat?ept??? ????µ? t?? p?e?µ???? s??µat??e?
  te???? t? ??tapept?d?? a??e??te?s??? ??
• ?s???? a??e??s?spast??? pa?????ta t?? ?e???
  µ????? ???? t?? a?t????????
• d?e?e??e? t?? pa?a???? ?a? ?????s? t??
  a?d?ste?????

11

• ? f??t?s? t?? ???a??sµ?? µe ????? per os a????e?
  ta ep?peda t?? a?d?ste????? st? p??sµa ?a? t?
  s?????t??s? t?? sta ???a
• e?e???p??e? afe??? t? s????s? t?? a?d?ste?????
  ?a??? ep?s?? ?a? e?a?s??t?p?e? ta ??tta?a t??
  spe???e?d??? ????? st? d??s? t?? ACTH, t?? ?? ??
  ?a? t?? ?a???? st?? ?????s? t?? a?d?ste?????
• ? ACTH pa??e? µ???? ???? st?? ?????s? t??
  a?d?ste?????
• se pe??pt?se?? ?at???e???? ?atast???? t?? µe t?
  ??????s? ???t???e?d??, ? ?????s? t?? a?d?ste?????
  e??a? f?s????????.

12

• ? ?????s? t?? a?d?ste????? pa???s???e? ?µe??s?a
  d?a??µa?s? µe a???µ??a ep?peda t? p??? ?a? ?aµ???
  t? ap??e?µa.
• a??????? ?at? t? f??t?s? t?? ???a??sµ?? µe ?????
• st???s? ??at??
• ????a st?s?

13
test p?? d?e?e?????, ?s? ?a? ?atast?????? t??
?????s? t?? a?at????t???e?d??

• s?ed?? ??a??? d?a?ta ( 10 mmol Na ?µe??s???) ??a
  3-5 µ??e?
• a??????? ?at? 3 ??? 6 f???? ?a? ep?p??s??t??
  ???e? 2 ??? 4 f???? µet? ap? 2-3 ??e? st?? ????a
  st?s?
• ??????s? 40-80 mg f????seµ?d??
• a????e? ?at? 2-4 f???? ta ep?peda t??
  a?d?ste????? µet? ap? 2-3 ??e? ????a? st?s??

14

• ?a test ?atast???? t?? a?at????t???e?d??
  ßas????ta? st?? ep??tas? t?? e?d?a??e?a??? ?????
  µe ???sta??e?d?
• µe??s? t?? ?????s?? t?? ?e????? ?a? ?at? s???pe?a
  ?a? st?? ?????s? t?? a?d?ste?????
• e?d?f??ß?a ??????s? 500 ml/ ??a f?s????????? ????
  ??a 4 ??e? ?atast???? t?? a?d?ste????? t??
  p??sµat?? se lt 8 ng/ dl se ?t?µa µe st???s?
  ??at?? ?a? lt t?? 5 ng/ dl se ?t?µa µe ?a??????
  p??s????

15
????S??????S??S

• ?pe??????s? a?d?ste?????
• St?? p??t?pa??, ? a?t?a a?t?? t?? ?pe??????s??
  ß??s?eta? µ?sa sta ep??ef??d?a, e?? st??
  de?te??pa?? t? e????sµa e??a? e??ep??ef??d?a??

16
???t?pa??? a?d?ste????sµ??

• ad???µa t?? ep??ef??d??? p?? pa???e? a?t???µa
  a?d?ste???? ( ??s?? Conn)
• ete??p?e??? µ???? ad???µa ?a? spa???te?a se
  ?a?????µa
• aµf?te??p?e??? ???d?? ?pe?p?as?a
• µ??f??????? ?a? ß????µ??? ???t???a d?? ßas???
  e?d? ade??µ?t??
• fasciculata-like ??tta?a
• glomerulosa-like ??tta?a ?a? e??a? e?a?s??t??
  st?? AT II

17

• a??et?? pe??pt?se?? aµf?te??p?e???? ??s?? t??
  Conn p?? pa???te?a ap?d?d??ta? se ???d?
  ?pe?p?as?a ?a ?fe????ta? te???? se ade??µata
• ?d?a s????t?ta ?a? sta d?? f??a ?????? µeta?? t??
  3?? ?a? 5?? de?aet?a? ????
• ? ?p??a??a?µ?a de? ap?te?e? asfa??? ???t???? ?a?
  ?t? a??et?? as?e?e?? µe ??s? t?? Conn eµfa??????
  f?s???????? ep?peda ?a????
• 8-10 t?? ?pe?tas???? p????sµ??.

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(No Transcript)
19

• ? ?pe??????s? t?? a?d?ste????? a????e? t??
  a?ta??a?? t?? ???t?? ?at???? µe ta ???ta ?a????
  ?a? ?d???????
• s???d? ?p??a??a?µ?a
• ep??tas? t?? e?d?a??e?a??? ?????d?ast?????
  ?????? ?p??tas? p?? s???de?eta? µe????? f???? ap?
  p????ef?????
• µ???? ad??aµ?a ?a? ??p?s?
• ?????a??? a????µ?e?
• p???????a ?a? p???d???a p?? ?fe??eta? st? µe??s?
  t?? s?µp????t???? ??a??t?ta? t?? ?????

20

• µa????????a pa?aµ??? t?? ?p??tas?? e??a? d??at?
  ?a ?d???se? se ?????a?? ?pe?t??f?a, ?a?
  ?ef?a??e?a??? ß??ße?.
• ?s?s?e?????a, p??a??? ???? t?? ?p??a??a?µ?a?
• pH t?? ????? e??a? a??a???? ???? t?? ap?????s??
  ???t?? aµµ?????
• ?p??a??a?µ?a d??ata? ?a e??a? s?ßa?? (lt ap? 3
  mmol/L)
• ? ?pe??at??a?µ?a ?fe??eta? st?? ?ata???t?s?
  ?at????, t?? ap??e?a ?e??? ???? t?? p???????a?
  ?a? t?? epa?a????µ?s? (reset) t?? ?sµ?st?t? st??
  ?p????aµ?

21

• µetaß????? a?????s? ?a? ? a???s? t??
  d?tta???a????? t?? ???? ?fe??eta? st?? ap??e?a
  ?d???????at???t?? ???? t?? e??d?? t??? ap? ta
  ??tta?a ??a ?a a?t?sta?µ?s??? t?? ?p??a??a?µ?a
• t? e???? espe??aµ??? s???????? epa?a???f?
  d?tta???a???? a????ta
• ep? s?ßa??? ?p??a??a?µ?a? ????µe ?a? µe??s? t??
  ep?p?d?? µa???s??? t?? ????
• a?ast??f? t?? ?????d??? ???µ?? µe t??
  ????t?p???s? t?? ????? ?at? t? d????e?a t??
  ???ta? ?a? t? s???d? p???????a

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???G?OS?

• 1) d?ast????? ?p??tas? ????? ??d?µa
• 2) ?aµ??? ep?peda ?e????? p??sµat??
  (PRA) ? ?p??a de? a????e? ?at? t?? ????a
  st?s? ? µe t? st???s? ??at?? ?p?? ?a?????? ?a
  ?fe??e
• 3) ?pe??????s? t?? a?d?ste????? p?? de?
  ?atast???eta? ?at? t? f??t?s? t?? ???a??sµ?? µe
  f?s???????? ???

23

• ? ad??aµ?a t?? ?atast???? t?? a?d?ste????? e??a?
  a?a??a?a s?????? ??a t? d?????s?, e?? ? ?aµ???
  PRA de? ??e? p??? ????? e?a?s??s?a d??t?
  a?e???s?eta? pe??p?? ?a? st? 25 t?? pe??pt?se??
  ?d??pa???? ?p??tas??
• ?? ????? a?t?? a?t?d???? s?ed?? f?s???????? st?
  f??t?s? t?? ???a??sµ?? µe ????? ? ?at? t?
  d???e?s? µe ACTH.

24

• ?? ? ?p??a??a?µ?a s?µße? se ??a? ?pe?tas???
  as?e?? p?? ?aµß??e? t?t???? e?d??? d?????t???
  a?t? ?a p??pe? ?a d?a??p???, ? as?e??? ?a ??ße?
  ?p??at?stata ?a???? ??a d??a ?µ??e? ?a? st?
  s????e?a ?a epa?a??f???? ?? e??ast???a???
  e?et?se??
• spiral CT t?? ep??ef??d???
• e??e?t???? ?a?et???asµ?? t?? ep??ef??d?a???
  f??ßa? µ?s? t?? µ???a?a?

25

• Se pe??pt?se?? ???d??? ?pe?p?as?a? t?? f????? de?
  a?e???s?eta? e?t?p?s?
• ta?t?????? ???? ep?p?d?? ???t??????
• a???µ??? ?e?d?? se ap??t?s? st?? ?????s? ?CTH p??
  p???a?e? t? ?pe?ß????? stress.
• µ?t??s? t?? PRA t? p??? se ?pt?a ??s?
• ??????s? 80 mg f????seµ?d?? per os ?a? ?
  epa?????? t?? µ?t??s?? af?? ? as?e??? ??e?
  pa?aµe??e? ?????? ??a pe??p?? 3 ??e?
• ?t?µa µe p??t?pa?? a?d?ste????sµ? e?a?????????
  ?a ????? ?aµ??? ep?peda t?? PRA
• ??????s? 300 mg/ ?µ??a sp??????a?t???? per os
  µp??e? ?a ?p?st???e? ta s?µpt?µata t?? ??s?? se
  5-8 eßd?µ?de?

26
S????OS?

• ? d?????s? t?? p??t?pa???? a?d?ste????sµ??
  µpa??e? ????? pe?a?t??? e??ast???a?? d?e?e???s?
  se as?e?e?? µe ?aµ??? ep?peda ?a???? ???? (lt 2.5
  mmol/L) ?a? ????? p????? a?d?ste?????/ PRA se
  t??a?a a?µ?????a

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? ??T?S ????S??????S??S

• f?s???????? a???µ??? ?????s? t?? a?d?ste????? se
  ap??t?s? st?? e?e???p???s? t?? s?st?µat??
  ?e?????- a??e??te?s????
• ep?ta????µe?? f?s? t?? ?p??tas?? ? se pe??pt?se??
  ??d?µat?? ???? ?a?d?a??? a?ep???e?a?
• ?????s?? ?a? ?ef??s???? s??d??µ?? ap? t? s?et???
  e??tt?s? t?? e?d?a??e?a??? ?????

28

• ?p??tas? p?? ?fe??eta? se p??t?pa?? ?pe?pa?a????
  ?e????? ap? ???? ? ap? st???s? t?? µ?a? ? ?a? t??
  d?? ?ef????? a?t????? s???de?eta? ?at? ?a???a ap?
  de?te??pa?? a?d?ste????sµ?
• ???µ?? pa?a????? t?? a?d?ste????? e??a?
  µe?a??te??? ap? ?t? st?? p??t?pa??
• e??s?? ?p??a??a?µ??? a?????s?
• ?a?a?t???st??? a???s? t?? PRA ?a? ? µe???? a???s?
  t?? ep?p?d?? t?? a?d?ste?????

29

• H d?????s? t?? ?ef?a??e?a??? ?p??tas?? t??eta? µe
  t?? a??de??? t?? a????µat???? p?a??? st??
  e??e?t??? a??e????af?a t?? ?ef????? a?t?????
• µ?t??s? t?? ?e????? st?? ?ef????? f??ße?
• t? ?e????µa a?ade????eta? µe ape?????st????
  µe??d??? (CT, MRI).

30
s??d??µ? Barter

• p????d??µ???
• IUGR
• p?????t?ta
• ?pe?asßest?????a
• ?ef?asß?st?s?
• s?et??eta? µe d?ata?a?? t?? µetaf????? ?a????
  (ROMK) st?? a????? t?? Henle

31
pa?d???? t?p??

• p???????a
• af?d?t?s?
• ?p??a??a?µ?a
• ?p??????a?µ??? µetaß????? a?????s?
• ?a??st???s? t?? a??pt????
• ?fe??eta? se µet???a?? t?? ????d??? p??
  ??d???p??e? t??? µetaf??e?? ???t?? ??????? (
  CIC-Kb) st?? a????? t?? Henle

32

• µe???? ap?????s? ???t?? ?a????, ?at???? ?a?
  ??????? sta ???a
• d???e?s? t?? pa?aspe??aµat???? s?s?e??? st??
  ?????s? ?e????? ?a? te???? st?? ?pe?p?as?a t??
• ? ap??e?a t?? ?a???? de? ?f?eta? a??µa ?a? µe t?
  d?????s? t?? a?d?ste????sµ??
• de? pa?at??e?ta? ?p??tas? ???? t?? s??e???
  ap??e?a? ??at?? ?a? ?e??? ap? ta ???a.

33
d?????s?

• ???e? a??µa??e?, ?p?? ??f?s? ?a? sa??a??d?
  d?aß?t?
• p???e???t??? d?????s? t?? s??d??µ?? µe t??
  µ?t??s? t?? ??e?t????t?? st? aµ??a?? ???? ?a? ta
  ???a t?? µ?t??a?.
• ????? s?????t??s? ??e?t????t??
• sta ???a t?? µ?t??a? ta ???ta ???????

34
s??d??µ? Gitelman

• ?p??te?? ??????? e????a ap? t? s??d??µ? Barter
• ?s?s?e?????a
• ?p??a??a?µ??? µetaß????? a?????s?
• a???µ??? ap?????s? ???t?? asßest??? ?a? µa???s???
  ap? ta ???a ?a? ep?µ??? ?p?µa???s?a?µ?a
• Na-2-Cl s?µµetaf???a st? ?p? espe??aµ???
  s????????
• s?µpt?µata a?d?ste????sµ?? ?a? ?aµ??? ep?peda
  µa???s??? st? p??sµa.

35
???F????? ???G?OS?

• ?atast?se?? p?? p???a???? pe??sse?a
  a?at???t???e?d??
• ?ata????s? ??????, ß?t????, tsa???? ? ?ap??? p??
  pe??????? ????????a.
• 3 µ???????????????-???????et?????? ????? (3 MGA)
• ?s????? a?ast???a? t?? e???µ?? 11 ß
  ?d????ste??e?d??? de?d???e??s? (11 ß HSD)
• pe??fe???? µetat??p? t?? ???t?????? se ???t?????
• ? ???t????? eµfa???e? ??µ??? s?????e?a ?p?? ?
  a?d?ste???? µe t?? ?p?d???a a?at????t???e?d?? st?
  ?p? espe??aµ??? s????????

36

• sp???? a?t?s?µ??? ?p??e?p?µe?? s??d??µ? t??
  a?ep???e?a? t?? 11-ß HSD
• ? a?ep???e?a t?? ???t?????? ?a µetat?ape? se
  ???t????? ep?f??e? d?sµe?s? t?? p??t?? st???
  ?p?d??e?? t?? ????????t???e?d?? t?p?? ?
• ?e?et??? a????s? ?a??? ep?s?? ?a? µe t? a???µ???
  p????? ???t??????/???t?????? sta ???a.

37
ßas??? d?af???d?a???st??? p??ß??µa

• d?????s? µeta?? t?? p??t?pa???? a?d?ste????sµ??
  p?? ?fe??eta? se ad???µa ap? t?? ???d? ?pe?p?as?a
  t?? ep??ef??d???
• ?e?ape?a t?? ade??µat?? e??a? ?at? ß?s?
  ?e?????????, e?? t?? ???d??? ?pe?p?as?a?
  fa?µa?e?t???

38

• as?e?e?? µe ???d? ?pe?p?as?a ????? ?aµ???te??
  ?????s? a?d?ste?????
• ?????te?? PRA
• µ????te?? ßa?µ? ?p??a??a?µ?a?
• ? d?af????? d?????s? ???eta? ?at? ????? ????
  ape?????st??? ?? ??? µe t?? ß????µ???? e?et?se??
• as?e?e?? µe ad???µa te????? ?a ????? a???µ??a
  ep?peda 18-?d???????t???ste????? ?a? µe??s? t??
  a?d?ste????? se ????a st?s?

39

• t? ad???µa µp??e? ?a pa???e? de??????t???ste????
  (DOC)
• ?a?a?t???st??? ????µe a???µ??? PRA
• f?s???????? ? µe??µ??a ep?peda a?d?ste?????,
• pe??sse?a ????? a?at????t???e?d??? ap? t??
  a?d?ste????.

40

• 70,se µ????e? ad???µa t?? e??? ep??ef??d???
• ?????? t?? a??ste???
• ??.?pe?p?as?ase??t????? p?? pa???eta? ap?
  ??tta?a t?? ?.?.S. ?a? de???e? ?a pa??e? ?ap???
  ???? st? ???µ?s? t?? ?pe?pa?a????? a?d?ste?????
  ?at? t?? ep??ef??d?a?? ?pe?p?as?a.

41
???ast???a?? e???µe

• 1)??t??s? a?d?ste????? sta ???a 24????,?p?? ?
  f?s???????? a?aµe??µe?? t?µ? e??a? 5-19mg/24h
• 2)??t??s? a?d?ste????? st? a?µa ?p?? e??a? 5
  mg/100ml ??a ?ata????e?? as?e?e?? ?a? 200mg/100ml
  a? ? as?e??? sta?e? ?????? ??a 2 ????.
• 3)?et??s? ?e????? t?? p??sµat??

42
?pe?????st??? e???µe

• 1)????e?t???? ?a?et???asµ?? ep??ef??d?a??? f?eß??
  ?a? f?eß???af?a
• s??a???f?s? t?? ep??ef??d?a??? f?eß??
• d?atetaµ??? t? s?st???? ep??ef??d?a?? f??ßa
• s??e?? µ?t??s? t?? t?µ?? t?? a?d?ste????? ap? t??
  ???e ep??ef??d?a?? f??ßa,p???µa p?? a?eß??e? t??
  e?d???t?ta ?a? t?? a???ße?a t?? µe??d?? st? 100.

43

• 2)??????? t?µ???af?a
• a???ß?ste?? ape?????st??? µe??d??
• 3)?pe???????f?µa
• µe?a??te??? t?? 2 cm
• 4)Sp?????????f?µa µe 131???-59
• ??µ???pa?a?????? d?ast????t?ta
• a????e?s? µ?????? ? s?st????? est??? a??µa ?a?
  2-4 ?µ??e? µeta t? ??????s?.

44
a?d?ste????sµ?? p?? ?f?eta? µe ????????t???e?d?
(GRA)

• ???????µe?ta? µe t?? a?t?s?µat??? ep???at???ta
  ?a?a?t??a
• (duplication) t?? ????d??? t?? p?????t?
  (promoter) t?? 11-ß ?d??????s?? (CYP11B1) p??
  ß??s?eta? ??t? ap? t?? ??e??? t?? ACTH µe t?
  ????d?? t?? s????s?? t?? a?d?ste????? (CYP11B2)
• ? d?ast????t?ta t?? s????s?? ß??s?eta? ??t? ap?
  t?? ??e??? t?? ?CTH
• ? a?d?ste???? e?????eta? µe pa??µ??? ?µe??s??
  ???µ? µe t?? ???t?????
• ? a?d?ste????sµ?? µp??e? ?a ?e?ape?te? µe t??
  e???e?? ??????s? ????????t???e?d??

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test t?? ?atast???? µe de?aµe?a????

• d?af???p??e? t??? GRA ?et????? ap? t??? GRA
  a???t?????
• 92 e?a?s??s?a ?a? 100 e?d???t?ta
• ep?peda a?d?ste????? ??t? ap? 4 ng/dl µet? t??
  ??????s? de?aµe?a????? ap?te?e? ??a asfa???
  ???t???? µe ????? e?a?s??s?a ??a t?? d?????s? t??
  GRA.

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a??s?d?t? a?t?d?as? p???µe??s?? ( PCR)

• a????e?s? t?? ?ß??d???? ????d??? t?? 11-ß
  ?d????ste??e?d???? ?d??????s?? (11-ß HSD) ?a? t??
  s????s?? t?? a?d?ste?????
• tµ?µa 4 Kb t?? ?ß??d???? ????d??? t? ?p???
  a?e???s?eta? se ??a ta ??? t??a a?a?????sµ??a
  ?ß??d??? ????d?a st?? ?????e?? ?pe?a?d?ste????sµ?
  t?p?? I
• d?????s? ?e????? µe t?? e??es? t?? ????d??? st?
  a?µa t?? ?µfa???? ?????
• ???s? t?? PCR st? µe??t? t?? ??µa?????? ?????
• µa???? screening t?? ?pe?tas???? p????sµ??

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s??d??µ? Liddle

• µe t?? a?t?s?µ??? ep???at???ta ?a?a?t??a
• ap?te?e? µ?a sp???a a?t?a p??t?pa????
  a?d?ste????sµ??
• (?e?d?a?d?ste????sµ?? t?p?? ?).
• ß ?p?µ???da t?? d?a???? ?at???? st? ep?????? t??
  ?ef????? s????a????
• ep?peda t?? ?e????? ?a? t?? a?d?ste????? e??a?
  ?aµ???
• ap?d???????s? t?? t?p???? a?t????µ?st????
  µ??a??sµ?? t?? ?ef???a sta ep?peda t??
  ?????f????sa? AT II ?a? a?d?ste?????

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• 1)Se µe?????? as?e?e?? µe ??d?ste????sµ? t? ?
  t?? ???? µp??e? ?a e??a? f?s???????? se pe??pt?s?
  p?? ???s?µ?p????? ?a???s??t???t??? d?????t???
  (sp??????a?t???,t??aµte????) ? e???? ?????
  p??s???? ?a???? ?a? ?aµ??? p??s???? ??at?? ap? t?
  d?a?t?????? t???.

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• 2)?? st?d?? t?? µ?t??s?? t?? a?d?ste????? µe
  f??t?s? NIS 0,9 de? p?epe? ?a ???e? a? ?
  ?p??tas? e??a? s?ßa?? (d?ast????? a?? t?? 15mmHg)
  ? ep? s???p???e?? ?a?d?a??? a?ep???e?a?.?p?s??
  p??pe? ?a ???e? d?????s? t?? ?a???? t?? ???? p???
  t? d???µas?a.??a??a?t??e? µ???d??? ap?te???? ?
  f??t?s? ??at?? per.os. (200mmol/d x 3 ?µ??e?) ? ?
  ??????s? 10 mg ??e???? des??????t???ste?????
  (DOCA) I.M. ???e 12 ??e?.

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• 3)?p?????? pe??pt?se?? ?p?? t? s. Liddle,t?
  s??d??µ? pe??sse?a? a?at????t???e?d?? ? ????? p??
  e???????? DOCA,p?? ?p?te ?a? pa?at????µe ?aµ???
  sta?µ? a?d?ste????? ?a? ??a?t? p?epe? ?a
  e?e????µe ??a t? a? ? ?atast??? p?????eta? ap?
  a???? e?d??? a?at????t???e?d? pe??? t??
  a?d?ste?????.

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• 4)?pa??e? t? e?de??µe?? se pe??pt?se??
  a?d?ste????sµ?? ? ep??e?µe?? ap?ß??? t?? ? ?a
  µ?? ?ata???e? se ?p??a??a?µ?a epe?d? ta ?ef???a
  s???????a d?a??t??? µ??a??sµ???,de?te???e???,?a
  ?ata??at??? ? ?a? ?a e?????? ?,µe ap?t??esµa
  t?? ?????p???s? (pt?s? t?? ??) t?? ?????

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• 5)? de?te??pa??? a?d?ste????sµ?? e??a? d??at? ?a
  p??????e? ?a? ap? st???s? t?? ?ef?????
  a?t???a?,?p?te ? a???s? t?? ?e????? ???? t??
  t?p???? ?p??tas?? e?e???p??e? t? spe??aµat???
  ???? st? ?a pa???e? a?d?ste????.

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Pathogenetic subtypes Approximate prevalence ()
Aldosterone producing adenoma (APA) 60 (solitary adrenal mass)
Idiopathic hyperaldosteronism (IHA) 34 (adrenal hyperplasia)
Angiotensin II - responsive adenoma (AIIRA) 5 (solitary adrenal mass)
Aldosterone producing carcinoma (APC) lt 1 (solitary adrenal mass)
Primary adrenal hyperplasia (PAH) lt 1 (adrenal hyperplasia)
Glucocorticoid suppressible hyperaldo-steronism (GSH) lt 1 (adrenal hyperplasia)
Ectopic aldosterone producing tumors (ovary) very rare
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