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Cushingov
sindrom
Domina
Petrić
Cushingov sindrom
• Rijetka bolest u dječjoj dobi.
• Najčešće je ijatrogen, zbog dugotrajne
terapije s ACTH ili glukokortikoidima.
• Nastaje zbog povećanog izlučivanja
kortizola uslijed hiperfunkcije kore NBŽ.
• U manje djece je češće posrijedi maligni
tumor kore NBŽ.
10/14/2017 copyright 2006
www.brainybetty.com
2
Cushingov sindrom
U veće djece je češće posrijedi bilateralna
hiperplazija kore zbog hipersekrecije
ACTH, odn. CRF-a (Cushingova bolest).
Vrlo rijetki uzroci su ektopična produkcija
ACTH iz feokromocitoma, neuroblastoma
ili ganglioneuroblastoma.
10/14/2017 copyright 2006
www.brainybetty.com
3
Klinička slika
• umor i iscrpljenost
• pretilost
• nakupljanje masnog tkiva, a posebice na licu,
vratu i trupu
• okruglo i pletorično lice
• tanki ekstremiteti
• osteoporotične kosti
• hirzutizam i akne (hiperprodukcija
androgena kore NBŽ)
10/14/2017 copyright 2006
www.brainybetty.com
4
Klinička slika
• stanjena koža s lividnim strijama
• ekhimoze i krvni podljevi zbog pojačane
krhkosti kapilara
• arterijska hipertenzija
• zaostatak u rastu koji nije praćen
zaostatkom u koštanom dozrijevanju
• više ili manje izražena emocionalna labilnost
i smetnje ponašanja
10/14/2017 copyright 2006
www.brainybetty.com
5
10/14/2017 copyright 2006
www.brainybetty.com
6
Pinterest
Laboratorijski nalazi
• Kortizol u plazmi, slobodni kortizol i
17-OHCS u urinu, a katkad i androgeni
u plazmi te 17-KS u urinu su povišeni.
• ACTH u plazmi je nizak ako je uzrok
tumor NBŽ, a visok u slučaju
hipotalamo-hipofizne etiologije
bolesti.
10/14/2017 copyright 2006
www.brainybetty.com
7
Laboratorijski nalazi
Najraniji znak bolesti je
poremećen dnevni (cirkadijani)
ritam sekrecije ACTH (visoke
koncentracije ACTH i kortizola u
večernjim satima zbog
kontinuirane sekrecije ACTH).
10/14/2017 copyright 2006
www.brainybetty.com
8
Diferencijalna dijagnoza
10/14/2017 GK: glukokortikoidi 9
Terapija
• Tumor kore NBŽ treba odstraniti
unilateralnom adrenalektomijom.
• U slučaju bilateralne hiperplazije zbog
hipersekrecije ACTH, terapija se može
provoditi zračenjem, implantacijom
radioaktivnog itrija, transsfenoidalnom
hipofizektomijom ili bilateralnom
adrenalektomijom.
10/14/2017 NBŽ: nadbubrežna žlijezda 10
Nelsonov sindrom
U nekih bolesnika se nakon
odstranjenja NBŽ razvije
KROMOFOBNI ADENOM
HIPOFIZE koji, osim ACTH,
izlučuje β-lipotropin i hormon
što stimulira melanocite (MSH).
10/14/2017 HIPERPIGMENTACIJA
KOŽE
11
10/14/2017 copyright 2006
www.brainybetty.com
12
Literatura:
Duško Mardešić i suradnici. Pedijatrija
Školska knjiga, Zagreb, 2003.

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Cushingov sindrom

  • 2. Cushingov sindrom • Rijetka bolest u dječjoj dobi. • Najčešće je ijatrogen, zbog dugotrajne terapije s ACTH ili glukokortikoidima. • Nastaje zbog povećanog izlučivanja kortizola uslijed hiperfunkcije kore NBŽ. • U manje djece je češće posrijedi maligni tumor kore NBŽ. 10/14/2017 copyright 2006 www.brainybetty.com 2
  • 3. Cushingov sindrom U veće djece je češće posrijedi bilateralna hiperplazija kore zbog hipersekrecije ACTH, odn. CRF-a (Cushingova bolest). Vrlo rijetki uzroci su ektopična produkcija ACTH iz feokromocitoma, neuroblastoma ili ganglioneuroblastoma. 10/14/2017 copyright 2006 www.brainybetty.com 3
  • 4. Klinička slika • umor i iscrpljenost • pretilost • nakupljanje masnog tkiva, a posebice na licu, vratu i trupu • okruglo i pletorično lice • tanki ekstremiteti • osteoporotične kosti • hirzutizam i akne (hiperprodukcija androgena kore NBŽ) 10/14/2017 copyright 2006 www.brainybetty.com 4
  • 5. Klinička slika • stanjena koža s lividnim strijama • ekhimoze i krvni podljevi zbog pojačane krhkosti kapilara • arterijska hipertenzija • zaostatak u rastu koji nije praćen zaostatkom u koštanom dozrijevanju • više ili manje izražena emocionalna labilnost i smetnje ponašanja 10/14/2017 copyright 2006 www.brainybetty.com 5
  • 7. Laboratorijski nalazi • Kortizol u plazmi, slobodni kortizol i 17-OHCS u urinu, a katkad i androgeni u plazmi te 17-KS u urinu su povišeni. • ACTH u plazmi je nizak ako je uzrok tumor NBŽ, a visok u slučaju hipotalamo-hipofizne etiologije bolesti. 10/14/2017 copyright 2006 www.brainybetty.com 7
  • 8. Laboratorijski nalazi Najraniji znak bolesti je poremećen dnevni (cirkadijani) ritam sekrecije ACTH (visoke koncentracije ACTH i kortizola u večernjim satima zbog kontinuirane sekrecije ACTH). 10/14/2017 copyright 2006 www.brainybetty.com 8
  • 10. Terapija • Tumor kore NBŽ treba odstraniti unilateralnom adrenalektomijom. • U slučaju bilateralne hiperplazije zbog hipersekrecije ACTH, terapija se može provoditi zračenjem, implantacijom radioaktivnog itrija, transsfenoidalnom hipofizektomijom ili bilateralnom adrenalektomijom. 10/14/2017 NBŽ: nadbubrežna žlijezda 10
  • 11. Nelsonov sindrom U nekih bolesnika se nakon odstranjenja NBŽ razvije KROMOFOBNI ADENOM HIPOFIZE koji, osim ACTH, izlučuje β-lipotropin i hormon što stimulira melanocite (MSH). 10/14/2017 HIPERPIGMENTACIJA KOŽE 11
  • 12. 10/14/2017 copyright 2006 www.brainybetty.com 12 Literatura: Duško Mardešić i suradnici. Pedijatrija Školska knjiga, Zagreb, 2003.