SINDROM NEFROTIK

Lestari Sukmarini, MN KMB FIK-UI 2009

SINDROM NEFROTIK
PENGERTIAN merupakan salah satu gambaran klinik penyakit glomerulus yang di tandai dengan:

protenuria masif >3,5 gram / 24 jam / 1,73 m2 hipoalbuminemia, edema anasarka, hiperlipidemia, lipiduria,dan hiperkoagulabilitas.

 DIAGNOSIS Anamnesis : bengkak seluruh tubuh,buang air kecil keruh Pemeriksaan fisis: edema anasarka,asites Laboratorium:Proteinuria masif >3,5 gram / 24 jam / 1,73 m2, hiperlipidemia,hipoalbuminemia ( <3,5 gram /dl),lipiduria,hiperkoagulabilitas. Diagnosis etiologi berdasarkan biopsi ginjal DIAGNOSIS BANDING Edema dan asites akibat penyakit hati atau malnutrisi,diagnosis

Patogenesis edema pd SN
Proteinuria
albumin plasma Tekanan osmotik koloid plasma Pergeseran cairan Cairan interstisiel ekstrasel

Cairan intravaskular Renin-angiotensin

Edema
Cairan ekstrasel
Aldosteron

Retensi Na & H2O

Reabsorbsi Na pd tubulus

INVESTIGASI
URINALISA
Proteinuria Dipstick test 24-hour quantitative test Albumin < 2.5 gr/dL Lipid: LDL/VLDL ; HDL BUN/Creatinine

Analisa darah

Biopsi ginjal Imaging studies: renal USG

Proteinuria
Dipstick test:
negative, trace 1+ (closest to 30 mg/dL) 2+ (closest to 100 mg/dL) 3+ (closest to 300 mg/dL) 4+ (greater than 2,000 mg/dL)

Proteinuria
Normal range
100mg/m2/day (150mg/day), <4 mg/ m2/hour

Nephrotic range
> 1 gm/ m2/day >40mg / m2/hour

Spot urine for albumin/creatinine ratio

(mg:mg)

Normal = <0.2 (0.5 if <2yr) Nephrotic => 2

Indikasi biopsi ginjal

Proteinuria > 1gr/hari Hematuria + Hipertensi + Fungsi ginjal

Etiologi NS
Penyakit ginjal primer (75-80%) Penyakit sekunder

Relative Frequency of Primary Glomerular Diseases Underlying the Nephrotic Syndrome in Children and Adults

Orth S and Ritz E. N Engl J Med 1998;338:1202-1211

 Glomerulopathy lesi minimal

Glomerulopathy lesi membranosa

Glomerulosklerosis fokal
Proteinuria masif, remisi 25%

Faal ginjal normal, respon kortikosteroid + remisi 90% Remisi spontan 60% Faal ginjal <, remisi 30%,sering relaps CKD

Glomerulopathy lesi proliferatif

Relatif jarang Hematuria +, hipertensi, Kortikosteroid?

Rare Causes of the Nephrotic Syndrome

Orth S and Ritz E. N Engl J Med 1998;338:1202-1211

Major Factors Contributing to the Hypercoagulable State in the Nephrotic Syndrome

Orth S and Ritz E. N Engl J Med 1998;338:1202-1211

Symptoms
Edema: swollen face, ascites, pleural effusion, swollen genital. Oliguria Hematuria anorexia, fatigue, irritable, pale Diare Respiratory distress Hipertensi Infeksi Tromboemboli

Treatment
terapi spesifik untuk kelainan dasar ginjal atau penyakit penyebab (pada SN sekunder), mengurangi atau menghilangkan proteinuria, memperbaiki hipoalbuminemi mencegah dan mengatasi penyulit.

Patofisiologi
Kerusakan glomerulus

Pengobatan
Imunosupresif: mycophenolate mofetil
(MMF), siklosporin

Antikoagulan:heparin/warfarin Anti agregasi trombosit: aspirin

Kehilangan protein Diit 35 kal/kgBB/hr RP 0.8-1 gr/kgBB/hr atau 0.6 gr/kgBB/hr + gram proteinurin Infus albumin (15%) 300 ml/45 mnt Diuretik spironolakton Diuretik furosemid 40 mg/hr Diit rendah garam 1-2 gr/hari ultrafiltrasi

Hipoalbuminemia dan penurunan tekanan onkotik Sekresi aldosteron Retensi natrium dan air Sembab yang resisten
Kontrol infeksi Kolesterol darah

antibiotik
hidroxymethyl glutaryl co-enzyme A (HMG Co-A) reductase

 Kontrol hipertensi angiotensin converting enzyme (ACE-1) inhibitors dan Angiotensin Receptor antagonist
sirkulasi, tekanan darah, tekanan glomerulus kebocoran protein, memperlambat progressive scarring glomeruli.

Non farmakologi lain: bedrest, diet rendah kolesterol

Complications
atherosclerosis "hardening of the arteries adverse reaction to steroids and immunisuppressant: osteoporosis, cataract development, increased risk of infection, and diabetes. Kidney function: insufficiency CKD Growth Delays in children Infection

Prognosis
Umur & gender: anak, wanita > baik. komplikasi, tipe histopatologis ginjal Kematian: GGK, infeksi sekunder, gagal sirkulasi.

PENGKAJIAN KEPERAWATAN
Riwayat kesehatan : ISPA, infeksi kulit, infeksi saluran kemih, hepatitis, obat nefrotoksik, riwayat keluarga dengan penyakit polikistik, keganasan, nefritis herediter. Sirkulasi : hipertensi, disritmia kardia, distensi vena jugular, edema general (termasuk area periorbital, sakrum), pallor. Eliminasi : perubahan pola urin, perubahan warna urin seperti merah, keruh, pekat, oliguri. Makanan/cairan : penambahan berat badan (edema), dehidrasi, mual, muntah, adanya penggunaan diuretik, perubahan turgor kulit, edema. Nyeri : pada area kostovertebral/pinggang Pernafasan : dispnea, takipnea, adanya batuk produktif (edema paru) Pemeriksaan diagnostik : Pemeriksaan sedimen urin : urin 24 jam untuk pemeriksaan bersihan kreatinin dan protein total untuk memperhitungkan fungsi ginjal residual dan ekskresi protein urin. cast sel darah merah membantu mengarahkan bahwa hematuria dari glomerulus. Biopsi ginjal kelainan histologi yang terjadi. Darah : Hb menurun karena anemia, BUN-creatinin meningkat, protein albumin serum menurun.

Pemeriksaan lain yang penting adalah : ureum serum, albumin, kolesterol, elektrolit dan juga pemeriksaan serologis seperti: autoantibodi, complement C3 C4, imunoglobulin. Urin: jumlah urin <400 cc/24jam; warna urin merah ditemukan adanya hematuria gross/mikroskopik, proteinuria (rendah 1-2+; tinggi 3-4+), Bersihan creatinin mungkin menurun. Radiografi/USG: pembesaran/mengecil ginjal; batu/obstruksi.

Nursing diagnosis
alteration in fluid volume: excess; risk for infection; alteration in nutrition: less than body requirements, potential alteration in comfort; knowledge deficit; and potential disturbance in self-concept: body image.

NURSING MANAGEMENT
Monitor I & Opossibly limit fluids balance (-) Weigh dailyevaluating amount of edema, abdominal circumference Making patient comfortable Maintaining good nutrition DietHigh protein, high CHO for protein sparing, low fat, salt restrictions Preventing infections: universal precautions, limit invasive procedure, Bedrest Activity may increase as edema decreases Measures to prevent skin breakdown. Monitor lab Monitor side effect of medications

Lifestyle and home remedies

Limit salt intake to help minimize fluid retention and swelling and to reduce blood pressure Modify diet to decrease cholesterol and triglyceride levels Take vitamin D supplements Report signs of relaps Infection prevention Check regularly to doctor or dietitian

Diet

References:
Black, JM. & Matassarin-Jacobs, E. (1997). Medical surgical nursing: Clinical management for continuity of care. (5th ed.).WB Saunders Company, Philadelphia. Goldman, L. & Bennett, JC. (2001). Pocket companion to cecil textbook of medicine (21st ed.). WB Saunders Company, Philadelphia. Reeves, CJ., Roux, G., Lochart, R. (2001). Keperawatan medikal bedah (Ed. Setyono, J.). Penerbit Salemba Medika, Jakarta. Sukandar, E. (1997). Nefrologi klinik. (2nd ed.). Penerbit ITB, Bandung. Wilson, DD. (1998). Nurses guide to understanding laboratory and diagnostic test. Lippincott William&Wilkins, Philadelphia. http://www.nephrologychannel.com/nephrotic/diagnosis.sht ml http://nephroticsyndrome.com

THANK YOU ..