Nephrotic Syndrom

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NEPHTI SYNDM

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NEPHTI SYNDME




Nephti syndme is haateized by heavy pteinia, hypabminemia, and edema. The syndme an be sbdivided int ngenita, pimay (idipathi), and senday types. Appximatey 85% t 95% f pimay ases in peadesents ae minima hange nephti syndme (MNS) and ae assiated with minima histgi hange in the gmei. Nephti syndme annay affits appximatey 16 hiden pe 100,000 ynge than age 16 in the nited States; it is sighty me mmn in maes than femaes in yng hiden, bt this disappeas in teenages and adts. Mean age at nset is 2 آ½.


Pathphysigy and Etigy

Pimay (idipathi, minima hange, hidhd) nephti syndme


* ndeying defet is thght t be ased by the ss f hage seetivity f the gmea basement membane, whih pemits negativey haged pteins, pimaiy abmin, t pass easiy thgh the apiay was int the ine.
* Exessive inay ss f ptein and atabizatin by the kidney f iating abmin eads t a deease in sem ptein (hypabminemia).
* The ida smti pesse that hds wate in the vasa mpatments is eded bease f the deease in the amnt f sem abmin. This aws fid t fw fm the apiaies int the intestitia spaes, ths pding edema.
* The shift f fid fm the pasma t the intestitia spaes edes the vasa fid vme (hypvemia), whih in tn stimates the enin-angitensin system and the seetin f antidieti hmne and adstene.
* Tba eabsptin f sdim and wate is ineased t inease intavasa vme.
* The ss f pteins, patiay immngbins, pedispses the hid t infetin.


inia Manifestatins


* nset is insidis thght t be ased by immne system distbanes bease it mmny s afte a mid I.
* Edema is typiay the pesenting symptm.

* Edema may be minima massive.
* Edema is say fist appaent and the eyes.
* Dependent edema s in aeas f the bdy, sh as the hands, ankes, feet, and genitaia.
* Fid that amates in the bdy spaes may give ise t asites and pea effsins.
* Stiae may appea n the skin fm vestething.


* Pfnd weight gain ased by edema; the hid may atay dbe nma weight.
* Deeased ine tpt ding the edemats phase ine appeas nentated and fthy.
* Pa, iitabiity, ethagy, and fatige.
* GI distbanes, inding vmiting, diahea, and anexia ased by edema f intestina msa.


Diagnsti Evaatin


* inaysis:

* Ptein say 2+ geate
* Bd absent tansient


* 24-h ine ptein feqenty geate than 2 gm2 pe day.
* Bd:

* Tta ptein eded
* Abmin ess than 2 gd
* heste Mgeate than 200 mgd with edema


* ena bipsy is indiated if patient is steid esistant (has faied t ahieve emissin afte 28 days f steid theapy).


Management

Steid Theapy

Pefeed appah t teatment.


* Pednisne is say the dg f hie bease it is ess ikey t inde sat etentin and ptassim ss and is the east expensive.
* N standad pgam f theapy exists, bt mst hiden eeive 60 mgm2 pe day f indtin f emissin.

* The steid dsage may as be aated n idea bdy weight with an eqivaent dse being 2 mgkg pe day.
* Patie vaies; hweve, the maximm steid dsage is p t 80 mgday f 4 weeks.


* Afte daiy steid theapy, the pednisne shd be disntined swy t avid mpiatins f steid withdawa, patiay benign intaania hypetensin. Patie vaies; hweve, mst entes emmend tapeing sing atenate-day pednisne ve 6 t 8 weeks.
* hiden with nephti syndme may espnd t steid theapy in sevea ways:

* Steid sensitive: ahieving a emissin within 28 days f the stat f pednisne theapy f the initia pesentatin f nephti syndme.
* Steid dependent: tw nsetive eapses, ing ding pednisne theapy, within 14 days afte its essatin.
* Steid esistant: faie t ahieve espnse in spite f 4 weeks f pednisne theapy at 60 mgm2 pe day.


* hiden with steid-espnsive MNS have a favabe ng-tem pgnsis.


Atenative Dg Theapies


* Shd be nsideed when hiden eapse feqenty (f t five pe yea), beme steid esistant steid dependent, demnstate naeptabe advese effets f steid theapy (steid txiity). The deisin t se atenative theapy in njntin with steids shd be made by an expeiened pediati nephgist.
* Immnsppessants

* yphsphamide (ytxan, Nesa)
* yspin A (Nea, Sandimmne)
* Taims (Pgaf)




I.V. Abmin 25%


* T shift fid fm intestitia spae int the vasa system.
* This is ny a tempay teatment t eieve edema bt may be sed in sevee ases f edema that ases espiaty distess sevee dismft de t edema.
* Dieti theapy is sed in mbinatin with I.V. abmin t hep eieve edema. In ases f hypvemia, dietis may nt be indiated.


mpiatins


* Infetins:

* Peitnitis, mst mmny ased by Stepts pnemniae, bt may as be ased by Esheihia i and Haemphis infenzae
* Gam-negative septiemia
* Staphya eitis


* Thmbsis
* Hypeipidemia
* Ate ena faie


Nsing Assessment


* btain histy f nset f iness and symptms.

* Peipitating events
* eent immnizatins
* eent Is
* Fike symptms
* Time f nset and atin f edema
* inay patten hanges


* Pefm physia examinatin fsing n vita signs; astatin f beath snds t detemine adventitis snds; aeas and extent f edema, espeiay peibita egin, extemities, genitaia, abdmen; and peiphea pefsin, inding pses, , wamth f extemities
* Nsing Diagnses
* Exess Fid Vme eated t fid amatin in tisses
* isk f Infetin eated t inay ss f pteins and hni steid se
* Imbaaned Ntitin: ess Than Bdy eqiements eated t ss f pteins thgh ine and anexia
* Intepted Famiy Pesses eated t hidhd iness
* .

Nsing Inteventins

eieving Exess Fid


* Administe tisteids as emmended by the heath ae pvide.

* bseve f advese effets and mpiatins f theapy sh as shing's syndme ineased bdy hai (histism), nding f the fae (mn fae‌), abdmina distentin, stiae, ineased appetite with weight gain, ataats, and aggavatin f adesent ane.
* Stess that these physia hanges ae nt hamf pemanent and that they wi disappea afte the steid teatment is stpped.
* bseve f seis advese effets and nmmn mpiatins f tisteids


* Administe immnsppessive dgs as pesibed.

* Make se that patient and paents ndestand the desied and advese effets f theapy.
* bseve f mpiatins f theapy, sh as deeased white bd e (WB) nt, ineased sseptibiity t infetin, hai ss ineased hai gwth (histism), gingiva hypepasia, hemhagi ystitis.


* Administe dietis as pesibed.

* Be awae f thse dietis that may ase ptassim depetin.
* ffe fds high in ptassim, sh as ange jie, bananas, and died fits (eg, aisins, apits).
* Administe sppementa ptassim hide as deed and if the ine tpt is adeqate.


* Enage ativity as teated.
* estit fids as deed (say ny ding the exteme edemats phases).

* estitin is aefy aated at feqent intevas, based n the ine tpt f the pevis day ps estimated insensibe sses.
* ffe sma amnts f fids spaed at ega intevas thght the day and evening. se a p f apppiate size f the amnt f fid being ffeed.
* Mease fids aatey in gadated ntaines. D nt estimate fid intake tpt.
* Pae a sign n the hid's bed t make se that n ine is aidentay disaded and that a intake is eded.
* Detemine tta intake and tpt evey 8 hs. In hiden wh ae nt tiet tained, a faiy aate ed f tpt an be btained by weighing diapes befe and afte viding.
* ed the ases f fid ss, sh as the nmbe f sts pe day, pespiatin.


* Assist with abdmina paaentesis; this may be eqied bease f maked asites. Ding the pede, fid is withdawn fm the peitnea avity t eieve pesse symptms and espiaty distess.
* estit sdim as deed (say dne whie the hid is pteini). A stating pint is 2 t 3 gday.

* se a w-sdim men when deing meas fm the hspita.
* Assist famiy in making fd hies that ae w in sdim.
* Fds that shd be imited inde ed, sated, anned, smked meats; pessed heese; ega anned fzen sps and bin bes; sated akes and the snak fds.




AET

N ive vainatins immnizatins shd be given ding ative episdes f nephsis whie the hid eeives immnsppessive theapy.

AET

Administe yphsphamide in the mning, with age vmes f fid, t pevent nentatin f the dg in the ine and ineased sseptibiity t ystitis.

Peventing Infetin


* Mnit mpete bd nt f deeased WB nt and netpenia.
* sey bseve the hid wh takes tisteids f signs f infetin. Be awae that feve and the symptms may be masked.
* Pvide metis skin ae t the edemats aeas f the bdy.

* Bathe the hid feqenty and appy pwde. Aeas f nen ae mist pats f the bdy and edemats mae genitaia. Sppt the stm with a ttn pad hed in pae by a T-binde, if neessay, f the hid's mft.
* Psitin the hid s that edemats skin sfaes ae nt in ntat. Pae a piw between the hid's egs when ying n side.
* Eevate the hid's head t ede edema.


* If pssibe, avid invasive pedes, sh as fema venipntes and I.M. injetins, t deease the hane f intding pathgens. Venipnte f the we extemities may as pedispse the hid t thmbembism bease f the hypvemia, stasis, and ineased pasma nentatin f tting fats.
* Edate paents egading signs and symptms f pssibe infetins.


Enhaning Ntitina Stats


* Assess ntitina intake, gwth, and devepment as apppiate f age.
* Pvide a diet w in sdim, fat, and sga. Pae a sign n the hid's bed that indiates dietay estitins, s that eveyne wi be awae f speia needs.
* Pvide fd hies that appea t the hid and that ae easy t eat ading t stage f devepment.
* Pvide ntitina sppements as needed.


Pviding Emtina Sppt


* Enage feqent visiting and aw as mh paenta patiipatin in the hid's ae as pssibe. Hspitaizatin, if neessay, is say bief.
* Aw the hid as mh ativity as teated.

* Baane peids f est, eeatin, and qiet ativities ding the nvaesent phase.
* Aw the hid t eat meas with famiy the hiden.


* Enage the hid and famiy t vebaize feas, fstatins, and qestins.

* Be awae that yng hiden feqenty fea abandnment by thei paents.
* Aw paents t expess fstatins egading the netainties assiated with the ase f the disease, the inia se, and pgnsis.
* Expain the diffeene between nephitis and nephsis if paents have qestins.


* Hep the hid adjst t hanges in bdy image, sh as shingid appeaane, by expaining hanges ahead f time.
* Disss the pbems f disipine with the paents. Enage them t set nsistent imits and easnabe expetatins f thei hid's behavi.
* Sggest paents get invved with a sppt gp f famiies f hiden with hni inesses, as needed.


Famiy Edatin and Heath Maintenane


* Pepae the famiy f hme management f the hid's ae pan.

* Have the dietitian disss speia diets with the paents.
* Teah the paents abt the hid's mediatin the desied effets and the ptentia advese effets.
* Demnstate ine testing f ptein.
* Initiate a mmnity heath nsing efea if neessay f eassessment and einfement f teahing.


* Enage ntined media fw-p visits.
* Emphasize the neessity f taking mediatin ading t the pesibed shede and f an extended time. Disss mpiatins ennteed with steid theapy.
* Teah peventin and egnitin f signs and symptms f infetin.
* Advise famiy n ativity estitins neessay.
* Teah signs and symptms f eapse (pteinia n ine dipstik at hme, ineased edema, deeased ine tpt) and whm and when t a with qestins.
* Teah signs and symptms f fid imbaanes (exess dehydatin).


Evaatin: Expeted tmes


* Deeased edema and asites; adeqate ine tpt
* Exhibits n signs f infetin
* Famiy vebaizes and fws dietay estitins as demnstated by apppiate weight gainss
* Famiy vebaizes nens egading hid's iness as demnstated by pen mmniatin with staff and the famiy membes