NephroticSyndrome

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Published on March 10, 2014

Author: dr.ankurshah82

Source: authorstream.com

PowerPoint Presentation: Pedi a tr i c N ep h rot i c Sy n d ro m e Dr Ankur Shah MD PowerPoint Presentation: P e d iatri c Ne p h r o ti c S y n d r o m e : Ba s i c I nfo rmati o n Ne phr o t i c s y n d r o m e ( N S) r e f le c t s g l o me r ul a r dy s f u nc t io n ca u s i ng pr o t e i n u r i a w i t h o u t c o m pr o m i s i ng G FR Occ u rs at a l l a g e s b u t i s m o st pr e v a l e n t i n ch i l d r e n b e t w ee n t he a g e s 1 . 5 - 6 y e ars I t a ff e c t s m o re b oy s t han gi r l s, 2 : 1 ra t i o Mo st s t u di e s p u t t he i nc id e nce at 2 - 7 p e r 100 , 00 0 p o p ul a t io n Pt presenting nephrotic syndrome: Pt presenting nephrotic syndrome PowerPoint Presentation: Figure 1. Nephrotic edema. PowerPoint Presentation: Figure 2. Nephrotic edema. Pathophysiology : Pathophysiology PowerPoint Presentation: S igns and S y m pt om s Pr o t e i n u r i a : 2 + ~ 4+ 24hr total urinary protein > 0.1g/kg. ( The most are selective proteinuria. ) H y p o a l b um i n e m i a: <3 g / d L H y p e rch o le s te r ol e m i a E d em a ( u n d e r fi l l [ fig u r e ] v s. o v e r fi ll ) H y p e rc o a g ul ab l e s t a t e T h ro m b o e m b o l i c e v en t s occur a t m o s t 10% of t h e r a t e i n a d u l t s w i t h NS Features: Features PowerPoint Presentation: Pr imar y N S: D i ffe r e n tia l D ia gnos i s M i n i mal Ch a ng e N e p h r o t i c S y nd r o me ( M C N S ) F o cal Se g m e n t al G l o m er u l os c l e r os i s ( FS G S ) M e mb r a no p r o l i fer a t iv e G l o m er u l on e p h r i t i s ( MG P N) Cong e n i t al n e p h r o t i c s y nd r o me ( Co n g N S ) Me m b r a nous ne p h r o pat h y , b o t h p r i m a r y a nd s econ da ry , is v e r y r a r e in p e d i at r ic pat ien t s , i.e. < 2 % of b io p s ies for p r o t einu r i a / NS (Chen A et a l 2007 ) PowerPoint Presentation: P r ima r y N S : Pe r centage of unde r l y ing diseases Co n g N S , 1% M P GN 5% FS GS 15% A d ole s ce n ts ( 13 - 18 ) ha v e d i s e as e com parab le to adu lts M CNS 79% T h e i n ci d e n ce of FS G S is r i s i n g o v er t h e l as t t w o d ec ad es PowerPoint Presentation: S econ d a r y Ca u s es S L E I n f e c t i on H I V Hepa t i t i s B an d C M a l a r i a S yph i l i s Obes i t y – g ene r a ll y l o w e r p ro t e i n ur i a an d l es s e d e m a D rug e x p o s ure N S AI D Hen och S c h o n l e i n P ur p ura M a li g nan cy ( R a re i n c h i l d r en ) Diabetes is not a cause of NS in children due to lo n g latency PowerPoint Presentation: M C N S v e r s u s F S G S: D ia gnos i s P ote n ti a l b iom ar ke r s K i d n ey B i o p s y S erum U r i n e Ga l e c t i n 1 S y n a p t opo d i n I G F BP - 1 N G A L N i t r i t e CD80 M C N S No r m al No r m al F S GS No r m al No r m al PowerPoint Presentation: M C N S v e r s u s F S G S: C li n i c a l a s p ec t s T h e rap y P r o gn o s is S t er o i d Re s po n s i ve C N I Re s po n s i ve M C N S >90% >85% F r e q ue n t r e l a p s es w h en d i s c on t i n ued U s ua ll y r e s o l ves b y p u b er t y P er s i s t s i n t o yo u n g adu l t h o o d i n up t o 20% o f c a s es H i g h r i s k o f o s t e opo ro s i s , h yp er t e ns i on , c a t a r a c t s , a n d s p erm a b n o r m a l i t i es i n p t s w i t h F R/ S D o r p er s i s t e n t d i s ea s e F S GS 25-30% 40% 1/3 o f p a t i e n t s a c h i e ve com p l e t e r e m i ss i o n 1/3 w il l h a v e p er s i s t e n t p r o t e i n u r i a. 1/3 p r og r e s s t o E S KD o v er 5-10 y r PowerPoint Presentation: G ene t ic ca u s es o f FS G S Gene Discovered Recessive Pediatric (R)/Dominant (P)/ Adult (D) (A) N eph r i n 1998 R P L M X 1B 1998 D A W T -1 1999 D P P o d oc i n 2000 R P A C T N4 2000 D A C D 2 A P 2000 R P T R P C6 2005 D A P L C E 1 2006 R P I NF 2010 D A C O Q 6 2011 R P MY O 1E 2011 R P A R H G A P 24 2011 D A PowerPoint Presentation: L a b or a t or y A s s e ss m ent U r i na l y sis : H i g h l e v e l s of p ro t e i n w i l l b e f ound i n t h e ur i ne Qua n t i t a t i ve fo r i n i t i a l e v a l ua t i o n D i p s t i c k fo r e x t e n ded mo n i t o r i n g Blood tests C omp r e h e ns i v e m e t a boli c p r ofi l e C 3 A l l o t h er t e s t s co n t i n g e n t o n cli n i ca l s c e n a r i o Kidney Biopsy C o n g e n i ta l N S : m ay b e s u ppl a n t ed b y g e n e t i c s O v er 80% o f c a s es o f N S i n f i rs t y r o f l i f e h a v e g e ne t i c m u t a t i o n L o w C 3 A t ypi ca l cli n i ca l f ea t u r es F a il u r e t o r e s po n d t o s t er o i ds PowerPoint Presentation: G eneral M anage m ent E d ema S a lt Re s t r iction Di ur etics Proteinuria A C E I/A RB N o b e n e f it of pr otein r e s t r iction in c h ild r en Infection: P e r ito n itis is mo s t common li f e t hr e a te n i n g s e r io u s ba cte r i a l i n f ection I mm un i z a tio n : P n e u mococc a l v a cci n e ( P r e v nar ® , P n e u mo v a x® ) , Flu V a cci n e P r o ph yl a ctic an ti b iotics Hyperlipidemia St a ti n s Bone Ca lci u m Vit a min D, if pr olo ng ed s te r oid usag e PowerPoint Presentation: M CNS I n d ic at ions f or 2 n d line R x B eh av io r a l ch a n g es I m pa i r ed g r o wt h R e d uced b one d en s i t y C o sm e t ic ch a n g es Cata r a c t s PowerPoint Presentation: FS G S Pr e d n i s o ne C y t o t o x i c a g e n t s i n e ff e c t i v e Ca l c i n eu r i n I nh i b i t o rs o n l y a g e n t s e v a l u a t e d i n c o n t r o lle d t r i a l s N o b e n e fi t o f m y c o ph e n o l a t e m of e t i l c o m b i n e d w i t h d e xa me t h a s o ne ( FS G S CT, K I 2011 ) Nee d fo r n o v e l appr o ach e s PowerPoint Presentation: M P G N A l t e rn a t e d ay s t e r oid s MM F E c ul i z um a b U n d e r s t u d y I m pa ct of se ro l og i c a l m a r ke rs of a c t i v a t i on of t h e a l t e r na t i v e pa t h w a y of co m p l e m en t PowerPoint Presentation: C o ngeni t al NS I n t e ns i v e me di cal m ana g em e nt I mm u n o s u p p r e s s i v e g e n e ra ll y i n e ff e c t i v e Sp e c ifi c t h e rapy i n s ele ct g e n e t i c ca u s e s, CN I i n PLC E1 , C o Q t r e a t m e n t e . g . B i l a t e r a l Ne phr e c t o m y Transp l ant i s u s u a l l y r e q u i r e d PowerPoint Presentation: M anage m ent o f S econ d a r y NS S L E : Co ll ab o r a t i o n w i t h r h e uma t o l o g y I n fe c t i o u s : A n t i b i o t i cs A n t i v i r a l s f or H e pa t i t i s B a n d C A n t i - r e t r o vi ra l t h e r apy f or H I V W e i gh t re d uc t i o n i f app r o p r i a t e Dr u gs : Di sco n t in u e H SP : S t e r o i d s p l u s M M F PowerPoint Presentation: P ro g no si s M i n im al Chan g e D is e a s e FS GS M P GN R e s o l v e s w i t h no p e r m an e nt ki dn e y da m a g e U s ua ll y r e s u l t s i n C K D ( >50 %) i n 5 - 1 0 y e ar s ) 50 % C K D w i t h i n 10 - 1 5 y e ars Of t e n R e l a ps e ( O v e r 90 %) PowerPoint Presentation: Re f erences B a gg a, A r v i n d, et a l . " N e p hr o t i c S y n d r o m e i n C h il d r e n . " I n d i an J M ed Res 122 ( 2005 ) : 13-28. Pr i n t . Lau, Ke i t h , et a l . " S t er o i d Re s po ns i v e N e p hr o t i c S y n d r o m e i n I g A N e p hr op a t h y w i t h F S G S . " T h e I n t er n et J o u rn al o f N e p hr ol o g y 4 ( 2008 ) : n . p a g . Pr i n t . " P ed i a t r i c N e p hr o t i c S y n d r o m e . " P ed i a t r i c N e p hro t i c S y n d r om e . N . p . , n . d. W e b . < h tt p : // e m ed i c in e . m ed s c a p e . co m >. U S A . N I H. N I D D K. C h il d h o o d N e p hr ot i c S y n d r om e . N . p . : n . p . , 2008. Pr i n t . T r a c h t m a n , H o w a r d. “ C omm o n D i s ea s e s : Mi n i m a l C h a n g e N e p hr o t i c S y n d r om e . ” N e p hr o lo g y S e l f A ss e ss m e n t Pr og r am 11 ( 2012) 19-20. Pr i n t . T r a c h t m a n , H o w a r d. “ C omm o n D i s ea s e s : Foc al S e gm e n t a l G l o m er u l os c l er o s is . ” N e p hr ol o g y S e l f A ss e ss m e n t Pr og r am 11 ( 2012) 20-. Pr i n t . C h o , M H. “” P a t h op h y s i olo g y o f M i n im a l C h a n g e N e p hr o t i c S y n d r om e a n d Foc al S e gm e n t al G l o m eru l o s c l er o s i s. ” P u bm ed ( 2007 ) . PowerPoint Presentation: Thank you

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