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NEFROTIK SINDROM Glomerular Capillary Wall

A three-layer structure:
1. Endothelial cell layer.
2. Glomerular basement membrane
(GBM).
Lamina rara interna
Lamina densa
Lamina rara externa
3. Epithelial cells (podocytes).

Nephron Segment Function

Renal Corpuscle:Filtration: Glomerolus membrane
•Glomerulus Glomerulus filters proteins and
•Bowman's cells from the blood. All other
Capsule blood components pass into
Bowman's capsule, then into the Scheme Of Filtration Barrier (Blood-
tubule. urine) In The Kidney. A. The Endothelial
U-Shaped Tubule Reabsorption and Secretion: Cells Of The Glomerulus; 1. Pore
Semipermeable membranes (Fenestra).
surrounding the tubule allow B. Glomerular Basement Membrane: 1.
selective passage of particles Lamina Rara Interna 2. Lamina Densa
back into the blood 3. Lamina Rara Externa
(reabsorption), or from the blood
C. Podocytes: 1. Enzymatic And
into the tubule (secretion).
Structural Protein 2. Filtration Slit 3.
Diaphragma
Collecting Duct Collection:
Collects all material that has not Layer Location Composition Function
returned to the blood through the
adjacent to
tubular membranes. This material lamina rara externa heparan sulfate blocks by charge
exits the kidney as urine. podocyte processes

Table 1
type 4 collagen and blocks by size (MW >
lamina densa dark central zone
Figure 2 This table summarizes the laminin 69,000)
This is a schematic diagram of the nephron. essential functions of the major adjacent to
segments of the nephron. lamina rara interna heparan sulfate blocks by charge
endothelial cells

PENGERTIAN Insiden Nefrotik Sindrom

 Sindrom nefrotik (SN) merupakan salah satu gambaran
klinik penyakit glomerulus yang di tandai dengan
protenuria masif >3,5 gram / 24 jam / 1,73 m2 disertai
hipoanbuminemia,edeMa anasarka, hiperlipidemia,
lipiduria, dan hiperkoagulabilitas
 Baughman (2000), nefrotik sindrom merupakan kelainan
klinik yang ditandai dengan proteinuria,
hipoalbuminemia, edema, dan hiperkolesterolemia
 Sowden (2002), nefrotik sindrom adalah keadaan klinis
yang disebabkan oleh peningkatan permeabilitas
glomerulus terhadap protein plasma yang menimbulkan
proteinuri, hipoalbuminemia, hiperlipidemia, dan edema.
 Nephrotic Syndrome merupakan kumpulan gejala yang
disebabkan oleh adanya injury glomerular yang terjadi
pada anak dengan karakteristik : proteinuria,
hypoproteinuria, hypoalbuminemia, hyperlipidemia dan
edema (Suryadi, 2001).
• 1. Insidens lebih tinggi pada laki-laki daripada
perempuan.
• 2. Mortalitas dan prognosis anak dengan sindrom
nefrotik bervariasi berdasarkan etiologi, berat, luas
kerusakan ginjal, usia anak, kondisi yang mendasari,
dan responnya terhadap pengobatan.
• 3. Sindrom nefrotik jarang menyerang anak dibawah
usia 1 tahun.
• 4. Sindrom nefrotik perubahan minimal (SNPM)
mencakup 60 – 90 % dari semua kasus sindrom
nefrotik pada anak.
• 5. Angka mortalitas dari SNPM telah menurun dari 50
% menjadi 5 % dengan majunya terapi dan pemberian
steroid.

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Nephrotic Syndrome
etiologi
Causes 3. GN proliferatif lain
Primary “Idiopathic” (95%)  Glomerulonefritis sekunder akibat :
a) Infeksi : HIV, Hepatitis virus B dan C. Sifilis, malaria, TBC,
• Minimal lesion NS (minimal change disease, lipoid nephrosis) Lepra
• Focal segmental glomerulosclerosis (FSGS) b) Keganasan : Adenokarsinoma paru, payudara, kolon,
• Mesangiocapillary glomerulonephritis I,II,III (MCGN, MPGN) limfoma Hodgkin, mieloma multipel, dan karsinoma ginjal
• Mesangial proliferative GN (IgM, IgG) c) Penyakit jaringan penghubung : Lupus eritematosus
• Membranous nephropathy sistemik, artritis reumathoid
d) Efek obat dan toksin : obat antiinflamasi nonsteroid,
Secondary (5%) preparat emas, penisilinamin, probenesid, air raksa,
• Complication / association of kaptopril, heroin
Disease state e) Lain-lain : DM, amiloidosis, preeklampsia, refluks
Drug vesicoureter, atau sengatan lebah (Sudoyo dkk, 2006).
Infection etc.

Secondary NS : DIAMOND
Pathophysiology
• Infection: APSGN (Acute streptococcal
glomerulonephritis) , HBV, HIV, shunt nephropathy,
reflux nephropathy, leprosy, syphilis, schistosomiasis,  proteinuria: due to an increase in glomerular permeability
hydatid disease
• Drug,Toxic,Allegy: mercury, snake venom, vaccine,  hypoalbuminuria: occurs when liver synthesis cannot keep
pellicillamine, Heroin,gold, NSAID, captopril, up with urine losses
probenecid, volatile hydrocarbons  oedema mechanism is complex and still in dispute: primary salt and
• Neoplasma: Hodgkin’s disease, carcinoma ( renal cell, water retention associated with reduced renal function as well as
lung, neuroblastoma, breast, and etc) reduced plasma oncotic pressure are primary factors (overfill and
• Autoimmune or collagen-vascular diseases: SLE, underfill)
Hashimoto’s thyroiditis, Vasculitis
 minimal change disease fits the underfill theory best
• Genetic Disease: Alport syn., Fabry syn., Nail-patella
syn., Sickle cell disease, Amyloidosis, Congenital  hyperlipidaemia: increased liver synthesis
nephropathy
• Metabolic disease: Diabetes mellitus  hypercoagulation: increased fibrinogen and loss of
• Others: Chronic transplant rejection, congenital antithrombin III
nephrosclerosis

Clinical Features of the Acute Nephritic

MANIFESTASI KLINIK Syndrome
1. Kenaikan berat badan
2. Wajah tampak sembab (edema fascialis) terutama di
sekitar mata, tampak pada saat bangun di pagi hari dan Haematuria is usually macroscopic with pink
berkurang di siang hari
3. Pembengkakan abdomen (asites)
or brown urine (like coca cola)
4. Efusi pleura Oliguria may be overlooked or absent in
5. Pembengkakan labia atau skrotum milder cases
6. Edema pada mukosa intestinal yang dapat
menyebabkan diare, anoreksia, dan absorpsi intestinal Oedema is usually mild and is often just peri-
buruk
7. Pembengkakan pergelangan kaki / tungkai
orbital- weight gain may be detected
8. Iritabilitas Hypertension common and associated with
9. Mudah letih raised urea and creatinine
10.Letargi
11.Tekanan darah normal atau sedikit menurun Proteinuria is variable but usually less than
12.Rentan terhadap infeksi in the nephrotic syndrome
13.Perubahan urin seperti penurunan volume dan urin
berbuih

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Komplikasi pada Nefrotik Komplikasi pada Nefrotik

Sindrom Churg Sindrommembagi dalam
dan kawan-kawan 4
1.Trombosis vena, akibat kehilangan anti-thrombin 3, yang golongan, yaitu:
berfungsi untuk mencegah terjadinya trombosis.
Trombosis vena ini sering terjadi pada vena renalis. A. Kelainan minimal
Tindakan yang dilakukan untuk mengatasinya adalah
dengan pemberian heparin. • ► Dengan mikroskop biasa glomerulus
2.Infeksi (seperti Haemophilus influenzae dan tampak normal, sedangkan dengan
Streptococcus pneumonia), akibat kehilangan
immunoglobulin. mikroskop electron tampak foot processus
3.Gagal ginjal akut, akibat hipovolemia. Disamping sel epitel berpadu. Dengan cara
terjadinya penumpukan cairan di dalam jaringan, terjadi imunofluoresensi ternyata tidak terdapat IgG
juga kehilangan cairan di dalam intravaskuler.
4.Edema pulmonal, akibat kebocoran cairan, kadang- atau immunoglobulin beta-IC pada dinding
kadang masuk ke dalam paru-paru yang menyebabkan kapiler glomerulus. Golongan ini lebih banyak
hipoksia dan dispnea.
terdapat pada anak daripada orang dewasa.

Komplikasi pada Nefrotik Komplikasi pada Nefrotik

Sindrom
B. Nefropati membranosa
Sindrom
C. Glomerulonefritis proliferatif
• *Glomerulonefritis proliferatif eksudatif difus
• Semua glomerulus menunjukan • Terdapat proliferasi sel meningeal dan infiltasi sel
penebalan dinding kapiler yang polimorfonukleus. Pembengkakkan sitoplasma endotel
yang menyebabkan kapiler tersumbat. Kelainan ini
tersebar tanpa proliferasi sel. Tidak sering ditemukan pada nefritis yang timbul setelah
sering ditemukan pada anak. infeksi dengan Streptococcus yang berjalan progresif
dan pada sindrom nefrotik.prognosis jarang baik,
Prognosis kurang baik. tetapi kadang-kadang terdapat penyembuhan setelah
pengobatan yang lama.
D. Glomerulosklerosis fokal segmental
• Pada kelainan ini yang mencolok sklerosi glomerulus.
Sering ditandai dengan atrofi tubulus. Prognosis buruk.

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TERAPI
Nephrotic Syndrome Nonfarmakologis:
Investigations 1. Istirahat
2. Restriksi protein dengan diet protein 0,8 gram / kg BB
• Urine analysis ideal / hari +ekskresi protein dalam urin / 24 jam. Bila
RBCs, Casts, fungsi ginjal sudah menurun,diet protein di sesuaikan
hingga 0,6 gram/kg BB/hari + ekskresi protein dalam urin /
Protein: 1 + = 0.3 gm/L 24 jam
2 + = 1 gm/L 3. Diet rendah kolesterol <600 mg / hari
3 + = 3 gm/l 4. Diet rendah garam, restriksi cairan pada edema
4 + > 4 gm/L Farmakologis:
1. Pengobatan edema :diuretik loop
• Kidney function (urea, creatinine) 2. Pengobatan proteinuria dengan penghambatan ACE
• 24-hour protein: type, amount dan/atau antagonis reseptor angiostens II
• CBC 3. Pengobatan dislipidemia dengan golongan statin
• C3 , C4 4. Pengobatan hipertensi dengan tekanan darah <125/75
mmhg. Penghambatan ACE dan antagonis reseptor
• Serum lipids (Cholesterole, TG, LDL,
angiotensin II sebagai pilihan obat utama
HDL)
5. Pengobatan kausal sesuai etiologi SN

Nursing Diagnosis Risk For Deficient Fluid Nursing Intervention nursing diagnosis Risk for
Deficient Fluid Volume related to disease process:
Volume Related To Disease Process
Nursing Intervention nursing diagnosis Risk for Deficient
Desired Outcomes/Evaluation Criteria Client Fluid Volume related to disease process:
Will: 1. Nursing Goal Increasing Circulating Volume and
1. Hydration Decreasing Edema
2. Monitor daily weight, intake and output, and urine
2. Maintain adequate fluid balance as specific gravity. Rationale: Comparing actual and
evidenced by vital signs and weight within anticipated output may aid in evaluating presence and
client’s normal range degree of renal stasis or impairment.
3. Monitor CVP (if indicated), vital signs, orthostatic blood
3. Palpable peripheral pulses, moist mucous pressure, and heart rate to detect hypovolemic.
membranes, and good skin turgor. Rationale: Indicators of hydration and circulating volume
and need for intervention.
4. Monitor serum BUN and creatinine to assess renal
function. Rationale: Elevated BUN, Cr, and certain
electrolytes indicate presence and degree of kidney
dysfunction.

Nursing Intervention nursing diagnosis Risk for

Nursing Care Plans for Nephrotic syndrome.
Deficient Fluid Volume related to disease process:
1. Administer diuretics or immunosuppressant as prescribed, and
Common Nursing diagnosis found in nursing
evaluate patient’s response. Rationale: May be used short-term to care plans for patients with Nephrotic
reduce tissue edema to facilitate movement of stone. syndrome:
2. Infuse I.V. albumin as ordered. Rationale: NS is associated with
significant protein loss. Serum albumin levels below 3.4 g/dL suggest 1. Imbalanced nutrition: Less than body
need for IDPN infusions. requirements,
3. Encourage bed rest for a few days to help mobilize edema; however,
some ambulation is necessary to reduce risk of Thromboembolic
2. Disturbed body image ,
complications. Rationale: Edematous tissues are more prone to 3. Excess fluid volume,
breakdown. Elevation promotes venous return, limiting venous stasis 4. Ineffective tissue perfusion: Renal,
and edema formation.
4. Enforce mild to moderate sodium and fluid restriction if edema is 5. Risk for injury
severe; provide a high-protein diet. Rationale: As fluid is pulled from 6. Risk for Deficient Fluid Volume,
extracellular spaces, sodium may follow the shift, causing
hyponatremia.
7. Risk for Infection related to treatment with
immunosuppressant

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Nursing diagnosis Risk for Infection related to

treatment with immunosuppressant
Nursing diagnosis Risk for Infection related to treatment with
immunosuppressant
Desired Outcomes Evaluation Criteria Client Will: Immune Status, Experience
no signs or symptoms of infection.
Nursing Intervention Nurse Care Plans for Nephrotic syndrome with nursing
diagnosis Risk for Infection related to treatment with immunosuppressants:
1. Nursing Goal Preventing Infection
2. Monitor for signs and symptoms of infection. Rationale : Fever higher than
100.4°F (38.0°C) with increased pulse and respirations is typical of
increased metabolic rate resulting from inflammatory process, although
sepsis can occur without a febrile response.
3. Monitor temperature routinely; check laboratory values for neutropenia.
Rationale: A shifting of the differential to the left is indicative of infection.
4. Use aseptic technique for all invasive procedures and strict hand washing
by patient and all contacts; prevent contact by patient with persons who
may transmit infection. Rationale: Reduces risk of cross-contamination.
5. Monitor effectiveness of antimicrobial therapy. Rationale: within 24 to 48
hours Signs of improvement in condition should occur.