Bengkak PD Wajah Perut

PROBLEM BASED LEARNING (PBL)
SISTEM UROGENITALIA
MODUL 1
BENGKAK PADA WAJAH DAN PERUT
PENDAHULUAN
Modul Bengkak pada Wajah dan Perut ini diberikan pada mahasiswa yang
mengambil mata kuliah sistim Urogenitalia di semester IV. TIU dan TIK pada sistim ini
disajikan pada permulaan buku modul agar dapat dimengerti secara menyeluruh tentang
konsep dasar penyakit-penyakit Sistem Urogenitalia yang memberikan gejala
bengkak
pada wajah dan perut. Mahasiswa diharapkan mampu menjelaskan semua aspek tentang
ssstem Urogenitalia dan patomekanisme terjadinya penyakit, kelainan jaringan, dan
pemeriksaan lain yang dibutuhkan pada penyakit yang memberikan gejala bengkak pada
wajah dan perut.
Sebelum menggunakan modul ini, mahasiswa diharapkan membaca TIU dan TIK
sehingga tidak terjadi penyimpangan pada diskusi dan tujuan serta dapat dicapai
kompetensi minimal yang diharapkan. Bahan untuk diskusi dapat diperoleh dari bacaan
yang tercatum di akhir modul. Kuliah pakar akan diberikan atas permintaan mahasiswa
yang berkaitan dengan penyakit ataupun penjelasan dalam pertemuan konsultasi antara
peserta kelompok diskusi mahasiswa dengan tutor atau ahli yang bersangkutan.
Penyusun mengharapkan modul ini dapat membantu
mahasiwa
memecahkan masalah penyakit Urogenitalia yang disajikan.

Revisi:
Makassar, 2 Maret 2008
Penyusun
FK-UNHAS Semester Akhir 2007/2008
dalam
SISTEM UROGENITALIA
MODUL 1
BENGKAK PADA WAJAH DAN PERUT
TUJUAN PEMBELAJARAN
Tujuan Instruksional Umum (TIU)
Setelah pembelajaran modul ini selesai, mahasiswa diharapkan dapat me
nyebutkan penyakit-penyakit yang menyebabkan pembengkakan pada muka dan perut,
menjelaskan gejala-gejala klinis, penyebab,
patomekanisme, cara-cara diagnosis,
penatalaksanaan/terapi, komplikasi dan aspek epidemiologi penyakit-penyakit yang

menyebabkan pembengkakan pada muka dan perut .
Tujuan Instruksional Khusus (TIK)

Setelah pembelajaran dengan modul ini mahasiswa diharapkan dapat:
Menyebut penyakit-penyakit yang menyebabkan
1.
muka dan
perut bengkak!
2.
Menjelaskan
tentang
patomekanisme
terjadinya
penyakit-penyakit
yang
menyebabkan pembengkakan pada muka dan perut:

2.1.
Menguraikan struktur anatomi, histologi dan histofisologi dari sistim

uropoetika,
2.2.
Menyebutkan fungsi masing-masing bagian dari nefron, fungsi sel-sel

JGA dalam renin angiotensin system,
2.3.
Menjelaskan faktor-faktor yang mempengaruhi GFR, prinsip hukum

Starling pada filtrasi ginjal, dan dapat menghitung GFR,
2.4.
Menjelaskan mekanisme dan proses reabsorbsi dan sekresi di tubulus,

mengapa ada zat yang mempunyai Tmax, peranan hormon aldosteron dan ADH
pada reabsorbsi, pengaturan reabsorbsi dan sekresi di tubulus, counter current
mechanism, proses reabsorbsi dan sekresi pada keadaan tertentu seperti dehidrasi
dan overhidrasi,
2.5.
Menjelaskan biokimia urine dan kompensasi ginjal dalam keseimbangan

asam basa,
2.6.
SISTEM UROGENITALIA
Menjelaskan tentang penyebab penyakit-penyakit yang menyebabkan

pembengkakan muka dan perut.,
2.7.
Menjelaskan hubungan antara penyebab, respon dan perubahan jaringan

pada patogenesis terjadinya penyakit yang menyebabkan pembengkakan muka
dan perut,
2.8.
Menyebut penyebab dari penyakit yang menyebabkan pembengkakan

muka dan perut.,
Menjelaskan tentang gejala-gejala klinik dari penyakit-penyakit
3.
yang menyebabkan pembengkakan muka dan perut,

Menjelaskan tentang cara-cara diagnosis
4.
dari
penyakit-
penyakit yang menyebabkan pembengkakan muka dan perut:

4.1.
Menjelaskan tentang cara anamnesis terarah pada penderita penyakitpenyakit yang menyebabkan pembengkakan muka dan perut,
4.2.
Menjelaskan tentang cara pemeriksaan fisik penderita penyakit-penyakit

yang menyebabkan pembengkakan muka dan perut,
4.3.
Menggambarkan perubahan histopatologi
4.4.
penyakit-penyakit di atas,
Menjelaskan fase pre-analitik, analitik & post analitik dari prosedur

tes/Lab pada penyakit-penyakit di atas,
4.5.
Menganalisa hasil laboratorium pada penderita penyakit-penyakit di

atasm,
4.6.
Menjelaskan gambaran Rontgen dari saluran kemih yang normal,

kelainan kongenital dan kelainan karena infeksi
Menjelaskan tentang penatalaksanaan dari penyakit-penyakit
5.
yang menyebabkan pembengkakan muka dan perut:

5.1.
Menyebutkan obat-obatan yang dipakai,
5.2.
Menjelaskan farmakodinamik dan farmakokinetik obat-obat yang

digunakan,
5.3.
Menjelaskan protokol/macam-macam cara yang dipakai pada SN yang

sensitif terhadap kortikosteroid (sesuai ISKDC, 1967),
5.4.
Menjelaskan paling kurang 8 istilah yang berhubungan dengan pengobatan pada

SN,
5.5.
SISTEM UROGENITALIA
Menjelaskan asuhan nitrizi penderita dengan gejala pembengkakan pada

wajah dan perut.
6.
Menjelaskan tentang prognosis dari penyakit-pebyakit tersebut.
7.
Menjelaskan tentang aspek epidemiologi penyakit-penyakit yang tersebut
PROBLEM TREE
ANAMNESIS :
Riwajat penyakit sekarang
Riwayat penyakit terdahulu
Kebiasaan makan
PEMERIKSAAN
Inspeksi: edema,
anemia/ikterus
Skelera: Status Gizi
Palpasi: edema, asites
Perkusi: asites
Auskultasi: bunyi jantung
PEM. PENUNJANG
Patologi Kinik
Urinalisis
Tes fungsi ginjal
Protein darah
Radiologis : BNO
KLINIK
Diagnosis Banding
Kwashiorkor
Glomerulonephritis
Angioedema
Heart Failure, Congestive
Obesity
Protein-Losing Enteropathy
Proteinuria
Anatomi
Histologi
Fisiologi
Biokimia
Patologi
Anatomi
Farmakologi
BENGKAK PADA
WAJAH & PERUT
PENGENDALIAN
DIAGNOSIS
PENATALAKSANAAN
KOMPLIKASI
PROGNOSIS
Preventif
Promotif
Non Bedah
Bedah
SISTEM UROGENITALIA
Medikamentosa
Non Medikamentosa
Nutrisi
SISTEM UROGENITALIA
KASUS
Skenario: Bengkak pada wajah dan perut
Seorang anak laki-laki, 12 thn, dibawa oleh ibunya ke Puskesmas dengan wajah dan
perut bengkak pembengkakan terajdi sejak 3 minggu yang lalu yang makin lama
semakin bertambah. Tidak ada demam dan tanda-tanda infeksi lain.
TUGAS MAHASISWA
1.
Setelah membaca dengan teliti skenario di atas anda harus mendiskusikan kasus
tersebut pada satu kelompok diskusi terdiri dari 12 15 orang, dipimpin oleh
seorang ketua dan seorang penulis yang dipilih oleh anda sendiri. Ketua dan
sekretaris ini sebaiknya berganti-ganti pada setiap kali diskusi. Diskusi kelompok
ini bisa dipimpin oleh seorang tutor atau dilakukan secara mandiri oleh
kelompok.
2.
Melakukan
aktivitas
pembelajaran
individual
di
perpustakaan
dengan
menggunakan buku ajar, majalah, slide, tape atau video, dan internet, untuk
mencari informasi tambahan.
3.
Melakukan diskusi kelompok mandiri (tanpa tutor) , melakukan curah pendapat

bebas antar anggota kelompok untuk menganalisa dan atau mensintese informasi
dalam menyelesaikan masalah.
4.
Berkonsultasi pada nara sumber yang ahli pada permasalahan dimaksud untuk
memperoleh pengertian yang lebih mendalam (tanpa pakar).
5.
Mengikut kuliah khusus (kuliah pakar) dalam kelas untuk masalah yang belum
jelas atau tidak ditemukan jawabannya.
6.
Melakukan latihan dilaboratorium

laboratorium.
keterampilan klinik dan praktikum di
SISTEM UROGENITALIA
PROSES PEMECAHAN MASALAH

Dalam diskusi kelompok dengan menggunakan metode curah pendapat,
mahasiswa diharapkan memecahkan problem yang terdapat dalam skenario ini, yaitu
dengan mengikuti 7 langkah penyelesaian masalah di bawah ini:
1. Klarifikasi istilah yang tidak jelas dalam scenario di atas, dan tentukan kata/ kalimat
kunci skenario diatas.
2. Identifikasi problem dasar scenario diatas dengan, dengan membuat beberapa
pertanyaan penting.
3. Analisa problem-problem tersebut dengan menjawab pertanyaan-pertanyaan diatas.
4. Klasifikasikan jawaban atas pertanyaan-pertanyaan tersebut di atas.
5. Tentukan tujuan pembelajaran yang ingindi capai oleh mahasiswa atas kasus
tersebut diatas.
6. Cari informasi tambahan tentang kasus diatas dari luar kelompok tatap muka.
Langkah 6 dilakukan dengan belajar mandiri.
7. Laporkan hasil diskusi dan sistesis informasi-informasi yang baru
ditemukan.
Langkah 7 dilakukan dalm kelompok diskusi dengan tutor.

Penjelasan :
Bila dari hasil evaluasi laporan kelompok ternyata masih ada informasi
yang diperlukan untuk sampai pada kesimpulan akhir, maka proses 6 bisa diulangi, dan
selanjutnya dilakukan lagi langkah 7.
Kedua langkah diatas bisa diulang-ulang di luar tutorial, dan setelah
informasi dirasa cukup maka pelaporan dilakukan dalam diskusi akhir, yang biasanya
dilakukan dalam bentuk diskusi panel dimana semua pakar duduk bersama untuk
memberikan penjelasan atas hal-hal yang belum jelas.
SISTEM UROGENITALIA
JADWAL KEGIATAN
Sebelum dilakukan pertemuan antara kelompok mahasiswa dan tutor,
mahasiswa dibagi menjadi kelompok-kelompok diskusi yang terdiri dari 15-17 orang
tiap kelompok.
1. Pertemuan pertama dalam kelas besar dengan tatap muka satu arah untuk penjelasan
dan tanya jawab. Tujuan : menjelaskan tentang modul dan cara menyelesaikan
modul, dan membagi kelompok diskusi. Pada pertemuan pertama buku modul
dibagikan.
2. Pertemuan kedua : diskusi tutorial 1 dipimpin oleh mahasiswa yang terpilih menjadi
ketua dan penulis kelompok, serta difasilitasi oleh tutor Tujuan :
*
Memilih ketua dan sekretaris kelompok,
Brain-storming untuk proses 1 5,
Pembagian tugas
3. Pertemuan ketiga: diskusi tutorial 2
seperti pada tutorial 1. Tujuan: untuk
melaporkan informasi baru yang diperoleh dari pembelajaran mandiri dan melakukan
klassifikasi, analisa dan sintese dari semua informasi.
4. Anda belajar mandiri baik sendiri-sendiri. Tujuan: untuk mencari informasi baru
yang diperlukan,
5. Diskusi mandiri; dengan proses sama dengan diskusi tutorial. Bila informasi telah
cukup, diskusi mandiri digunakan untuk membuat laporan penyajian dan laporan
tertulis. Diskusi mandiri bisa dilakukan berulang-ulang diluar jadwal.
6. Pertemuan keempat: diskusi panel dan tanya pakar. Tujuan: untuk melaporkan
hasil analisa dan sintese informasi yang ditemukan untuk menyelesaikan masalah
pada skenario. Bila ada masalah yang belum jelas atau kesalahan persepsi, bisa
diselesaikan oleh para pakar yang hadir pada pertemuan ini. Laporan penyajian dibuat
oleh kelompok dalam bentuk sesuai urutan yang tercantum pada buku kerja.
7. Masing-masing mahasiwa kemudian diberi tugas untuk menuliskan laporan tentang
salah satu penyakit yang memberikan gambaran seperti pada skenario yang
SISTEM UROGENITALIA
didiskusikan pada kelompoknya. Laporan ditulis dalam bentuk laporan penyajian

dan laporan lengkap.
8. Pertemuan terakhir: laporan kasus dilakukan dalam kelas besar oleh masing-masing
mahasiswa.
Catatan :
Laporan penyajian kelompok dan perorangan serta semua laporan kasus

masing-masing diserahkan satu rangkap ke sistem melalui ketua kelompok.
Semua laporan akan diperiksa dan dinilai oleh pakarnya masing-masing.
Semua mahasiswa wajib menyalin laporan dari kelompok dan mahasiswa

lain untuk dipakai sebagai salah satu bahan ujian.
TIME TABLE
PERTEMUAN
IV
II
III
Pertemuan I
(Penjelasan)
Pertemuan
Mandiri
(Brain
Stroming)
Tutorial I
Pengum-pulan
informasi
Analisa &
sintese
Mandiri
Praktikum
CSL
VI
VII
Kuliah
kosultasi
Tutorial II
(Laporan
& Diskusi)
Pertemuan
Terakhir
(Laporan)
STRATEGI PEMBELAJARAN
1. Diskusi kelompok difasilitasi oleh tutor
2. Diskusi kelompok tanpa tutor
3. CSL : Pemeriksaan fisisk, gambaran radiology penderita dengan pembengkakan
pada muka dan pertut.
4. Praktikum Anatomi, Histologi, PA dan Patologi Klinik
5. Konsultasi pada pakar
6. Kuliah khusus dalam kelas
7. Aktivitas pembelajaran individual diperpustakaan dengan menggunakan buku ajar
Majalah, slide, tape atau video dan internet
SISTEM UROGENITALIA
BAHAN BACAAN & SUMBER INFORMASI LAIN
A. Buku Ajar dan Jurnal

3
4
10
11
12
13
15
18
20
21
22
23
24
28
31.
Grant BJC The perineum and Pelvis : a Method of Anatomy

Grant Henry and Mayo Goss. The Urogenital System
Prescott LM et al : Microbiology, 2nd edition, Wm.c Brown Publisher, Melbourne, 1993
Parker and Collier : Principles of Bacteriology, Virology & Immunity, 8th edition, vol 1-5,
1990
Guyton & Hall : Textbook of Medical Physiology, edisi 10, 2001
A Textbook of Radiology and Imaging : David Sutton, 1993
Thorpe, Neal O : Cell Biology 5th edition, Canada, John Wiley and Son Inc, 1984
Wila Wirya IGN. Penelitian beberapa aspek klinis dan patologi anatomis sindrom nefrotik
primer pada anak di Jakarta. Disertasi, 1992
Mohammad SN. Glomerulonefritis. Dalam buku ajar nefrologi anak. Balai penerbit FKUI,
Jakarta 2002, 345-52
Drum-mond KN. Infection of the urinary tract, dalam: Vaughan VC, MC Kay RJ, Behrman
RE, penyunting. Nelson Textbook of pediatrics, Edisi 11, Tokyo : Igoku Shoin, 1979: 1543-48
Kallen RJ. UTI: Treating the first infection and avoiding the second, Mod med Asia, 1980; 16:
67-74
McCracken GH. Diagnosis and management of acute urinary tract infections in infants and
children. J Pediatric infection disease. 1987; 107-112
Kher KK. Obstructive uropathy. Dalam : Kher KK, Marker SP, penyunting. Clinical Pediatric
Nephrology. New York: Mc Graw Hill 1992: 447-65.
Meatow SR. Eneuresis. Dalam: Pediatric Kidney Disease. Edelman CM, penyunting. Edisi 2,
Boston: Little Brown Co 1992: 105-84
Ahmad H, Tejani A: Predictive Value of Repeat Renal Biopsies in Children with Nephrotic
Syndrome. Nephron 2000 Apr; 84(4): 342-346.
Diktat dan hand-out
B.
1.
2.
3.
4.
Diktat Anatomi
Diktat Histologi
Buku Ajar Fisiologi Ginjal
Diktat Kuliah Radiologi
D. Sumber lain : VCD, Film, Internet, Slide, Tape

1.
2.
3.
Bagga A, Hari P, Srivastava RN: Prolonged versus standard prednisolone therapy for initial
episode of nephrotic syndrome. Pediatr Nephrol 1999 Nov; 13(9): 824-7[Medline].
Baldwin DS, Gluck MC, Schacht RG, Gallo G: The long-term course of poststreptococcal
glomerulonephritis. Ann Intern Med 1974 Mar; 80(3): 342-58[Medline].
Bazzi C, Petrini C, Rizza V, et al: A modern approach to selectivity of proteinuria and
tubulointerstitial damage in nephrotic syndrome. Kidney Int 2000 Oct; 58(4): 173241[Medline].

4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
SISTEM UROGENITALIA
Boute N, Gribouval O, Roselli S: NPHS2, encoding the glomerular

protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat
Genet 2000 Apr; 24(4): 349-54[Medline].
Constantinescu AR, Shah HB, Foote EF: Predicting first-year relapses in children with
nephrotic syndrome. Pediatrics 2000 Mar; 105(3 Pt 1): 492-5[Medline].
Dodge WF, Spargo BH, Travis LB, et al: Poststreptococcal glomerulonephritis. A prospective
study in children. N Engl J Med 1972 Feb 10; 286(6): 273-8[Medline].
Eddy AA, Schnaper HW: The nephrotic syndrome: from the simple to
the complex. Semin Nephrol 1998 May; 18(3): 304-16[Medline].
Gulati S, Sharma AP, Sharma RK: Changing trends of histopathology in childhood nephrotic
syndrome. Am J Kidney Dis 1999 Oct; 34(4): 646-50[Medline].
International Study of Kidney Disease in Children: Prospective,
controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Report of
the International Study of Kidney Disease in Children. Lancet 1974 Aug 24; 2(7878): 4237[Medline].
International Study of Kidney Disease in Children: Primary nephrotic syndrome in children:
clinical significance of histopathologic variants of minimal change and of diffuse mesangial
hypercellularity. A Report of the International Study of Kidney Disease in Children. Kidney Int
1981 Dec; 20(6): 765-71[Medline].
International Study of Kidney Disease in Children: The primary nephrotic syndrome in
children. Identification of patients with minimal change nephrotic syndrome from initial
response to prednisone. A report of the International Study of Kidney Disease in Children. J
Pediatr 1981 Apr; 98(4): 561-4[Medline].
Kaplan JM, Kim SH, North KN: Mutations in ACTN4, encoding alpha-actinin-4, cause
familial focal segmental glomerulosclerosis. Nat Genet 2000 Mar; 24(3): 251-6[Medline].
Kestila M, Lenkkkeri U, Mannikko M et al: Positionally cloned gene for a novel glomerular prt
Arze RS, Rashid H, Morley R, et al: Shunt nephritis: report of two cases and review of the
literature. Clin Nephrol 1983 Jan; 19(1): 48-53[Medline].
Neugarten J, Gallo GR, Baldwin DS: Glomerulonephritis in bacterial endocarditis. Am J
Kidney Dis 1984 Mar; 3(5): 371-9[Medline].
Oda T, Yamakami K, Omasu F, et al: Glomerular plasmin-like activity in relation to nephritisassociated plasmin receptor in acute poststreptococcal glomerulonephritis. J Am Soc Nephrol
2005 Jan; 16(1): 247-54[Medline].
Rodriguez-Iturbe B: Epidemic poststreptococcal glomerulonephritis. Kidney Int 1984 Jan;
25(1): 129-36[Medline].
Rodriguez-Iturbe B: Nephritis-associated streptococcal antigens: where are we now?. J Am Soc
Nephrol 2004 Jul; 15(7): 1961-2[Medline].
Ronco P, Verroust P, Morel-Maroger L: Viruses and Glomerulonephritis. Nephron 1982; 31(2):
97-102[Medline].
Yoshizawa N, Yamakami K, Fujino M, et al: Nephritis-associated plasmin receptor and acute
poststreptococcal glomerulonephritis: characterization of the antigen and associated immune
response. J Am Soc Nephrol 2004 Jul; 15(7): 1785-93[Medline].
SISTEM UROGENITALIA
Nara sumber (Dosen Pengampu)

DAFTAR NAMA NARA SUMBER
No.
NAMA DOSEN
1.
Prof.Dr. dr. Syarifuddin Rauf

Sp.PA
Prof.Dr.dr. Syakib Bakri,
Sp.PD
Prof.dr. Ahmad M Palinrungi
Sp.B, Sp.U
Prof.Dr.dr. M.Dali
Amiruddin, Sp.KK
dr. Irfan Idris, MS
dr. Theopilus Buranda, MS
dr. Robby Lianury
Dr. Agnes Kwenang, MS
Dr. dr. Gatot Lawrence
Dr. dr. Nurpudji Astuti,
SpGK
Dr. dr. Fatmawati
Dr. Randana Bandaso, MS
dr. Nurlaily Idris, Sp.Rad.
dr. H, Ibrahim Samad, SpPK
dr. Baedah Madjid, SpMK
dr. Sastri, SpKK
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
BAGIAN
TLP.
KANTOR
HP/FLEXI
Anak
0811411109
Penyakit Dalam
0816250620
Bedah Urologi
08164384040
Kulit Kelamin
08194229858
Fisiologi
Anatomi
Histologi
Biokimia
Patologi Anatomi
Gizi
584730
Farmakologi
Patologi Anatomi
Radiologi
Patologi Klinik
Mikrobiologi
Kulit Kelamin
081342695348
081342436444
0811411723
0816255306
0811443856
081524120368
0811441064
0811444326
08124217393
PETUNJUK UNTUK TUTOR

TUGAS TUTOR :
Pra tutorial
1. Mempelajari dengan seksama modul ini termasuk TIU dan TIK
2. Jika ada materi yang tidak jelas mohon ditanyakan pada dosen pengampu (nama,
no telfon setiap dosen pengampu terlampir)
3. Membuat rencana pembelajaran
4. Membuat tabulasi penyakit penyakit yang menyebabkan produksi kurang dan
menghubungkannya dengan kata kunci
5. Mengecek kelengkapan ruang tutorial
SISTEM UROGENITALIA
Tutorial tahap 1
1. Membantu mahasiswa menunjuk ketua dan sekertaris kelompok
2. Memfasilitasi diskusi agar berjalan sesuai urutannya yaitu :
Menyusun kata kunci
Membahas TIU dan TIK
Membuat daftar pertanyaan sebanyak banyaknya yang diarahkan ke TIK
Menjawab pertanyaan-pertanyaan
Membuat tabulasi penyakit penyakit yang menyebabkan kencing kurang
dan menghubungkannya dengan kata kunci
Membuat tujuan pembelajaran selanjutnya
Membagi tugas pencarian informasi berdasarkan jenis penyakit yang
menimbulkan kencing kurang
3. Melakukan penilaian untuk mahasiswa dan menandatanganinya
4. Mengecek kehadiran mahasiswa dan menandatangani daftar hadirnya
5. Mengingatkan mahasiswa agar pertemuan selanjutnya masing masing sudah
mengisi lembaran kerja
Tutorial tahap 2
1. Mengecek apakah mahasiswa datang dengan membawa lembaran kerjanya
2. Memfasilitasi diskusi agar berjalan sesuai urutannya yaitu :
Melaporkan informasi tambahan yang baru diperolehnya
Mahasiswa mendiskusikan satu persatu penyakit yang bergejala utama
produksi kencing kurang,
etiologinya, patomekanismenya, cara
mendiagnosis (anamnesis, inspeksi, palpasi perkusi dan auskultasi,
pemeriksaan penunjang dan penatalaksanaannya.
Mahasiswa menganalisa kembali tabulasi yang dibuat berdasarkan setiap
penyakit dan kata kunci.
Mengurutkan penyakit mulai dengan diagnosis terdekat sampai diagnosis
yang terjauh
Tutor menanyakan beberapa pertannyaan mendasar yang perlu diketahui
mahasiswa dan mendiskusikannya
Mahasiswa membuat tujuan pembelajaran selanjutnya dengan mencatat
pertanyaan yang belum terjawab untuk dicari pada perpustakaan,
ditanyakan langsung kepada dosen pengampu atau ditanyakan dalam
diskusi panel.
3. Membuat penilaian terutama saat mahasiswa melaporkan informasi yang
diperoleh.
4. Mengecek kehadiran mahasiswa dan menandatangani daftar hadirnya
Saat Panel Diskusi
1. Wajib mengikuti diskus panel
2. Membuat penilaian pada penampilan, cara menjawab, isi jawaban dan lain-lain
pada mahasiswa yang melapor atau menjawab pertanyaan.
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Setelah satu Seri Tutorial Selesai

1. Mengumpulkan semua absensi kelompok di Koordinator PBL
2. Membuat penilaian ahir: dari semua nilai
3. Memeriksa laporan mahasiswa bersama nara sumber
KATA/KALIMAT KUNCI
1. Anak laki-laki
2. 12 tahun
3. Bengkak pada wajah dan perut
4. Pembengkakan makin bertambah
BEBERAPA PERTANYAAN PRINSIP DAN JAWABAN ALTERNATIFNYA
Klarifikasi dan mekanisme kata kunci berikut ini :

1.
Pria umumnya lebih banyak dari wanita dengan perbandingan 2:1 dan
beberapa studi mendapatkan 3 : 2 pada kejadian Sindroma Nefrotik.
2.
Umur 12 tahun : Pada kejadian Sindroma Nefrotik 75% terjadi pada umur
< 18 tahun dengan onset pada umur < 6 thn.
3.
Bengkak pada wajah & perut : Wajah tampak mengembang akibat
akumulasi cairan terutama pada daerah jaringan yang resistensinya kurang
seperti daerah preorbital (pada wajah), skrotum dan labia. Udema jika
sangat berat (massif) dapat terjadi anasarka termasuk udema di perut. Hal
ini terjadi akibat hipoproteinemia sehingga terjadi extravasasi cairan
menuju ruang interstitiel.
4.
Pembengkakan makin bertambah : derajat udema makin berat. Pada
penyakit SN udema cenderung makin bertambah berat. Mekanismenya
belum jelas, beberapa sarjana mengatakan bahwa selain terjadi
hipoalbunemia, juga terjadi penigkatan sekresi aldosteron, vasopressin, dan
menurunnya ANP yang menyebabkan resistensi air dan garam.
Apa penyebab dan bagaimana pato-mekanisme gejala-gejala yang menjadi kata

kunci tersebut
1. Udema : Terjadi akibat hipoproteinemia, terutama fraksi albumin
(hipoalbuminemia).
Menurunnya
kadar
protein
menyebabkan
menurunnya tekanan osmotik protein plasma. Hal ini akan menyebabkan
cairan intravaskuler menuju ke ruangan interstitiel akibat perbedaan
osmotik protein di ruangan interstitiel lebih tinggi dibanding dalan
pembuluh darah. Menurunnya cairan intravaskuler menyebabkan
rangsangan renin-angiotensin-aldosteron sistem. Kemudian terjadi
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perangsangan pada ADH sehingga terjadi penahanan air dan garam, hal
inilah yang menyebabkan udem.
2. Udema yang makin bertammbah : meskipun belum jelas, tetapi beratnya
udem diperparah oleh aktifnya renin-angiotensin-aldosteron sistem
sehingga ginjal menahan air dan garam.
Sebutkan beberapa penyakit yang dapat di differential diagnosis
dengan tanda dan gejala pada skenario
1. Acute Poststreptococcal Glomerulonephritis
2. Angioedema
3. Heart Failure, Congestive
4. Nephritis
5. Nephrotic Syndrome
6. Obesity
7. Oliguria
8. Protein-Losing Enteropathy
9. Proteinuria
10. Systemic Lupus Erythematosus
Pemeriksaan penunjang yang dibutuhkan untuk menegakkan diagnosis penyakit
yang termasuk dalam DD penyakit tersebut
1. Urinalisis : hematuria, protein, ratio UA/G
2. Kimia darah : hipoalbuminemia, hyperlipidemia,
3. Radiologi : Thorax foto :pleural effusion, USG renal
4. Biopsi ginjal
Bagaimana cara penatalaksanaan dari penyakit-penyakit yang termasuk dalam

DD penyakit tersebut
1. Medikamentosa : steroid, diuretik, anti infeksi, antihipertensi
2. Diet
Epidemiologi dan cara pencegahan penyakit tersebut

1. Insidens 2-5 per 100.000 anak dibawah 18 tahun. Prevalensinya di USA
dilaporkan 15.5 per 100.000 anak.
2. Relaps adalah hal yang dapat diusahakn untuk dicegah. Biasanya relaps
mudah terjadi setelah infeksi saluran napas, sehingga pencegahan infeksi
saluran nafas perlu dilakukan.
3. Relaps mudah terjadi setelah imunisasi rutin, sehingga imunisasi perlu
ditunda sampai remisi terjadi.
Komplikasi penyakit-penyakit yang termasuk DD pada skenario

1. Lesi glomerulus
2. Hypoproteinemia
3. Efek pemberian steroid
Prognosis penyakit-penyakit tersebut

Tergantung pada hasil pemeriksaan histologis ginjal. Penderita yang memberi respon
pada steroid umumnya mengalami relaps yang tidak sering. Tetapi penderita yang
tidak memberi respon terhadap steroid lebih sering mengalami relaps.
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Nephrotic Syndrome
Background: The word nephrosis, or what often is called primary nephrotic syndrome (PNS), has its
origin in the early part of the 20th century. Nephrosis describes a clinical condition of edema and
proteinuria characterized morphologically (light microscopy [LM]) by fatty degeneration of the renal
tubules associated with normal glomeruli. The term nephrosis was introduced primarily to distinguish it
from nephritis, a term used to denote the clinical condition associated with cellular proliferation of the
glomerulus. Shortly thereafter the name of the condition was changed to lipoid nephrosis when it was
noted that lipid droplets commonly were found in the urine of affected patients. Lipoid nephrosis
gradually evolved to the present name of nephrotic syndrome (NS).
NS is a clinical condition characterized by massive urinary loss of protein (primarily albumin), which
leads to hypoproteinemia (hypoalbuminemia) and edema. Hyperlipidemia, hypercholesterolemia, and
increased lipiduria usually are associated. Although not commonly thought of as part of NS, hypertension,
hematuria, and azotemia may occur. NS is categorized into primary and secondary forms. The name PNS
has replaced, in some circles, the older designation (idiopathic), but it implies the same vagueness as to
cause. Included are a wide variety of clinical as well as pathologic states, some of which are noted in
Synonyms. The term secondary NS is associated with more clearly defined diseases, such as
anaphylactoid purpura, systemic lupus erythematosus, diabetes mellitus, sickle cell disease, and syphilis.
Most attention in this article is devoted to PNS because of its relative frequency in children.
Histopathologic characteristics led to the description of PNS subcategories, some associated with specific
clinical manifestations. Currently, knowledge regarding etiology precludes a more precise classification.
Nevertheless, in the following sections, the variants of PNS are considered as if they are disease entities
with well-defined clinical and histopathologic characteristics; the histologic type at onset makes
generalization about the response to therapy and the ultimate prognosis possible. When possible, the
definitions, descriptions, and nomenclature developed by the International Study of Kidney Diseases in
Children (ISKDC) are used. Most attention is devoted to minimal change nephrotic syndrome (MCNS),
immunoglobulin M (IgM) mesangial nephropathy, and focal segmental glomerulosclerosis (FSGS), with
modest attention to familial or congenital nephrosis, membranoproliferative glomerulonephritis (MPGN),
and membranous nephropathy (MN).
MCNS is the most common form of NS in children, and its prevalence is inversely proportional to the age
at onset; beyond infancy, the likelihood that the histology demonstrates minimal abnormalities on LM
evaluation of glomerular histology increases with younger age. As is discussed later, within this category,
histologic variations exist in which some patients demonstrate only fusion and smudging of the epithelial
cell podocytes, while others may demonstrate mild changes within the glomerular mesangium, consisting
of either slight proliferation or sclerosis. Because patients with MCNS have the highest rate of
responsiveness to standard therapy and the best long-term prognosis, separating them from others is
important.
IgM mesangial nephropathy may be a separate entity from MCNS. The current histologic criteria of NS
are based predominately on LM findings. During the study period, the ISKDC did not use
immunofluorescent microscopy in defining histologic criteria. Most patients who have significant IgM
staining of the mesangium present in a similar manner to those with MCNS, unless coexistent mesangial
proliferation is present. Controversy exists as to whether IgM mesangial deposition in a patient with
minimal changes on LM affects either the response to therapy or the subsequent clinical course.
FSGS is heterogeneous condition; much of the confusion in the literature is the result of attempts by some
authors to portray it as a single entity. FSGS is a histopathologic expression of a variety of conditions,
each with its own clinical manifestations. The lesion may be observed at the onset of an otherwise typical
case of NS, or it may be discovered only after years of nephrosis in a patient in whom the initial biopsy
was compatible with MCNS, but whose course of the disease suggested otherwise. FSGS may be the
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consequence of glomerular hyperfiltration in patients with reflux nephropathy and in some patients with a
single kidney.
MPGN may present as a NS, particularly in older children and adolescents. MPGN usually is associated
with a nephritic picture although, on occasion, MPGN may appear similar to MCNS or FSGS.
While membranous glomerulonephritis (MGN) occurs in children, it is exceedingly rare in children
younger than 10 years, and most incidents of MGN occur in adolescents. MGN usually is idiopathic in
origin; however, a number of well-defined etiologies have been described, and the competent physician is
prudent to consider these secondary causes in the evaluation. MGN is not associated infrequently with
hepatitis B and has been described with both hepatitis A and hepatitis C. In parts of the world where
malaria is prevalent, the most common renal lesion associated with malaria is MGN. MGN also has been
associated etiologically with HIV infections. In the young person, the heavy proteinuria often is
accompanied by hematuria. This lesion responds poorly to glucocorticoid therapy and often is suspected
first when the patient is unresponsive to standard steroid management (see Medical Care).
Congenital nephrotic syndrome must be considered when nephrosis appears during the first year of life,
particularly during the first few months.
Pathophysiology: Heavy proteinuria (albuminuria) is the hallmark and primary abnormality of NS. The
degree of proteinuria varies considerably from one child to another and is proportional, at least in part, to
plasma protein concentration. Thus, a child with active NS who has a serum albumin concentration of 2
g/dL usually excretes a larger amount of albumin than a similar child with a serum albumin concentration
of 0.5 g/dL. Some children excrete as much as 15 g/m 2/d, although the minimal excretion rate compatible
with the diagnosis of NS is approximately 1 g/m2/d (approximately 40 mg/m2/h).
The event that produces proteinuria is unknown. In MCNS, the glomerular capillary permeability to
albumin is increased selectively, whereas proteinuria is nonselective in other forms of NS, such as those
associated with nephritic features. The increase in the filtered load of protein overcomes the modest ability
of the tubules to reabsorb it, and proteinuria ensues. The albumin selectivity observed in persons with
MCNS may be due in part to the smaller size of the molecule; however, the absence of increased excretion
of some smaller-weight plasma proteins makes this explanation insufficient. One possible explanation is
the disappearance of negative charges normally present along the glomerular capillary endothelium and
basement membrane. This disappearance allows the negatively charged albumin to traverse the glomerular
capillary barrier. This has been demonstrated in experimental nephrosis and in some children with PNS.
PNS is believed to have an immune pathogenesis, but the nature of this immune process has yet to be
defined completely. A highly cationic plasma protein that may neutralize the anionic charge on the
glomerular capillary wall has been described in children with NS. Other investigators have noted a
decrease in immune responsiveness and have related this to alterations in either T- lymphocyte number
and/or function. The presence of suppresser cytokines or lymphokines has been postulated, and various
investigators have demonstrated changes in interleukin-8, interferon permeability factor, and vascular
permeability factor. The role of the kinin system also is under investigation because urinary excretion of
kinins is increased during exacerbations of the disease.
Hypoalbuminemia is mainly the result of the increased urinary loss of protein; however, other factors may
contribute to the hypoalbuminemia, such as decreased synthesis, increased catabolism, and increased
gastrointestinal losses. Even though most studies have demonstrated that the albumin synthesis rate is not
decreased, the capacity to increase hepatic production appears insufficient to compensate for the large
urinary losses.
The classic explanation for the edema formation is that low-serum albumin causes a decrease in plasma
oncotic pressure (POP) with extravasation of plasma water into the interstitial space. The resulting
contraction in plasma volume (PV) theoretically leads to a decrease in renal perfusion and, hence, to
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stimulation of the renin-angiotensin system. This hormonal effect coupled with an increase in the synthesis
and secretion of antidiuretic hormone (related to the decrease in effective PV) affects the renal tubular
reabsorption of sodium and water. The results of these disturbances are a reduction in renal perfusion (eg,
glomerular filtration rate [GFR]) and an increased hormonal activity that leads to avid reabsorption of both
sodium and water.
While the hypothesis described above is attractive, some experimental data fail to support it. First, the PV
is not always decreased, and, in most adults, PV appears to be increased. Only in young children with
MCNS have most studies revealed a reduced PV. Most investigators have failed to document elevated
levels of renin, angiotensin, or aldosterone even during times of avid sodium retention. Moreover,
retention of sodium continued despite maneuvers that suppress renin (eg, albumin infusion) or aldosterone
synthesis (angiotensin-converting enzyme inhibitor administration). Coupled with these discrepancies is
the fact that, in steroid-responsive NS, diuresis usually begins before the plasma albumin has increased
significantly and before POP has changed. Some investigators reported a blunted response to atrial
natriuretic peptide despite higher than normal circulating levels of atrial natriuretic peptide.
Thus, the precise causes of the edema and of its persistence remain uncertain. A complex interplay of a
variety of physiologic factors (eg, decreased POP, increased activity of aldosterone and vasopressin,
diminished atrial natriuretic hormone, activities of various cytokines, physical factors within the vasa
recti) probably contributes to the retention of sodium and the accumulation of water.
Mortality/Morbidity:
The mortality rate depends almost entirely on the type of disease causing the heavy urinary loss
of albumin. The long-term prospect of complete remission in persons with MCNS is greater than
70% with a reported cumulative mortality rate (at 20 y postonset) of less than 15% (range in
various studies is 5-15%). Conversely, only 24% of patients with FSGS are in remission after 20
years, and the cumulative mortality rate is greater than 50% (see Prognosis).
The morbidity rate is substantial. Even in its mildest form, MCNS often is a chronic disease that
requires the following:
o Hospitalization, in some instances
o A prolonged period of treatment
o Frequent monitoring both by parents and by physician
o Administration of drugs associated with significant adverse events
o A high rate of recurrence (ie, relapses in >60% of patients)
o The potential for progression to chronic renal failure (CRF)
In patients who demonstrate multiple relapses, the potential problems are associated with the
need for long-term immunosuppression therapy that predisposes to serious infections, decreases
in growth velocity, behavioral changes, obesity, cataracts, hypertension, osteoporosis,
osteomalacia, nephrolithiasis, diabetes mellitus, hirsutism, gingival hypertrophy, and other issues.
Sex:
In children younger than 8 years at onset, the ratio of males to females is 2:1 to 3:2 in various
studies.
In older children, adolescents, and adults, the male-to-female prevalence is approximately equal.
ISKDC data indicate that 66% of patients with either MCNS or FSGS are male, whereas for
individuals with MPGN, 65% are female.
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Age: Approximately 75% of the patients who developed NS when younger than 18 years were younger
than 6 years at onset. MCNS is primarily an illness of preschool children, with peak incidence occurring
when the child is aged 3-4 years, although MCNS onset may occur at any age. With the exception of the
first year of life, the likelihood that the lesion is MCNS increases with younger age at onset. When disease
onset occurs when the child is younger than 5 years, the likelihood that the lesion is MCNS is greater than
90%, while the risk of FSGS and MPGN is 7% and 1%, respectively. Conversely, when disease onset
occurs when the individual is older than 10 years, the risk of MCNS drops to approximately 50%, and the
risk of MPGN approaches 30%. FSGS may occur at any age; however, its incidence tends to increase
slightly with advancing age.
CLINICAL
History:
Edema: Regardless of the type of NS (ie, histopathologic type), the major clinical manifestation
is edema, which is a presenting symptom in approximately 95% of children with NS.
o
Edema often is so insidious in onset that the family may believe the child merely is
gaining weight rapidly.
Edema in the early phase may be intermittent; it usually appears first in areas of low
tissue resistance (eg, periorbital area, scrotal and labial regions). Ultimately, the edema
becomes generalized and can be massive (anasarca).
Because the nature of edema is typically dependent, edema often is worse in the face in
the morning (upon arising) and is found predominantly in the lower extremities later in
the day.
The edema is pitting in nature.
In individuals with marked edema, the skin may ooze clear fluid and appear thinner than
usual. Breakdown of the tissue is common.
Edema usually is more marked in the patient with MCNS than in the patient with either
FSGS or MPGN. This is because urinary protein loss and hypoproteinemia are virtually
always greater in persons with MCNS.
Age of appearance: The child with congenital NS often presents earlier than the child with
MCNS, but the age of appearance of the first signs of disease overlap significantly.
Hematuria
o
An occasional child with NS presents with gross hematuria.
The frequency of macrohematuria depends on the histologic subtype of NS.
Macrohematuria is more common in patients with MPGN; however, even in individuals

with MCNS, the frequency of macrohematuria has been reported as high as 3-4%.
Statistically, a higher percentage of patients with FSGS have microhematuria than those
with MCNS at presentation; however, this statistic is not very helpful in differential
diagnosis.
Patients commonly complain of diarrhea, which also is believed to be related to edema

of the bowel wall.
If ascites is marked, patients commonly complain of respiratory distress.
Fever and infection

o
o
o
o
o
The persistence of microscopic hematuria beyond the first month, which is common in
persons with FSGS but seldom observed in individuals with MCNS, is more relevant.
Oliguria: Oliguria is a common occurrence during relapses.

Underlying renal involvement: More specific clinical manifestations occur at various frequencies,
depending on the type of underlying renal involvement (see Lab Studies, Histologic Findings).
Common symptoms: Regardless of the type of NS, patients commonly are anorexic, irritable,
fatigued, and complain of abdominal discomfort, which is believed to be related to edema of the
bowel wall.
o
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An occasional child presents with fever and a septic picture.

In a number of these patients, the peritoneal cavity is the site of the infection.
Streptococcus pneumoniae still is the most frequent organism responsible for peritonitis
in this population, but Staphylococcus aureus and Escherichia coli commonly are
recovered.
A urinary tract infection occasionally is present and may be responsible for the lack of
response to therapy.
A history of respiratory tract infection (RTI) immediately preceding the first clinical
signs of the disease may exist, but its relevance is uncertain.
Allergies
o
o
A history of prior allergic events is common, and atopy has been reported in
approximately 40-50% of children with MCNS.
A hypersensitive event (eg, insect sting, ant bites, poison ivy, immunizations) has
preceded the onset in some individuals and may be considered etiologically significant.
The peak incidence of NS appears to be seasonally related, at least in certain geographic

areas.
A few children have been reported with major food allergies, and, in some, ultimate
remission was associated with dietary elimination programs.
Physical:
The most common clinical finding in all patients with NS is edema, which is present in more than
95% of individuals with the condition.
o
The degree of edema usually is greatest in patients with MCNS.
When mild, edema is localized to those areas where tissue resistance is low (eg,
periorbital area, scrotum, labia).
Generalized edema is dependent and pitting in character.
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Ascites is common, and anasarca may be present. In children with marked ascites,
mechanical restriction of breathing may be present, and the child may have
compensatory tachypnea.
Due to the skin edema, the child usually appears paler than laboratory evidence of
anemia suggests.
Hypertension may be present in persons with any form of NS.

o
The ISKDC studies revealed that approximately 30% of patients with MCNS have
systolic and diastolic pressures greater than the 90th percentile for age.
If values greater than the 98th percentile were used to denote an abnormality, then
approximately 20% had systolic pressures, and approximately 13% had diastolic
pressures that were elevated.
The percentage of children with hypertension is higher in those with FSGS and,
particularly so, in patients with MPGN in whom hypertension may be severe.
Other consistent abnormalities of the physical state are unusual and only a few are mentioned.
o
Signs of a concurrent upper RTI may be present, and some children have overt evidence
of an atopic state with varying degrees of eczema.
An occasional child demonstrates evidence of an insect sting and/or bite.
Abdominal tenderness is unusual in the absence of a peritoneal infection.
On rare occasions, the child with nephrosis may demonstrate signs of either arterial or venous
thrombosis, secondary to a hypercoagulable state.
o
The thrombosis may involve peripheral or internal vessels.
Early recognition is essential if the involved organ is to be saved.
Causes:
Edema, the predominant clinical feature, ultimately is the result of the urinary loss of large
amounts of albumin from the serum with a consequent lowering of the serum albumin
concentration.
o
The cause for the maintenance and progression of the edema is less certain (see
Pathophysiology).
The GFR often is reduced by a mild-to-moderate degree, and the ability of the renal
tubules to aggressively reabsorb sodium and water is enhanced. Oliguria and edema
ensue.
The edema first collects in those sites where tissue resistance is low, such as the
periorbital area and in the scrotum.
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Later, the edema becomes generalized.
The etiology of the hypertension probably is multifactorial.

o
In some patients with elevated blood pressure (BP), particularly in small children with
MCNS, the PV is low, and the associated tachycardia suggests an increase in
sympathetic nervous system activity. In such patients, the BP falls following an infusion
of albumin.
In most older patients with NS, the PV is either normal or increased. In some of these
patients, BP returns to normal with diuresis.
Plasma renin levels have been reported variously as either normal or slightly increased;
however, the response to blockade of the renin-angiotensin system does not support this
as the primary cause of the hypertension.
Various cytokines known to have pressor effects are increased and may be the primary
cause of hypertension.
DIFFERENTIALS
Acute Poststreptococcal Glomerulonephritis
Angioedema
Heart Failure, Congestive
Nephritis
Nephrotic Syndrome
Obesity
Oliguria
Protein-Losing Enteropathy
Proteinuria
Systemic Lupus Erythematosus
Other Problems to be Considered:

Glomerulonephritis
Lupus erythematosus in infants and children
A differential diagnosis is a 2-step process. First, question what other conditions are likely to present
similarly (eg, edema). Allergic reactions probably are the most common ones: it is striking how often the
child with new-onset nephrosis initially is believed to have periorbital edema as a result of an allergic
reaction. Other conditions that may produce facial or generalized edema in children include congestive
heart failure, severe hepatic disease associated with hypoalbuminemia, protein-losing enteropathy (cystic
fibrosis, particularly), and congenital defects in albumin synthesis. After the urine is obtained and it is
recognized that proteinuria is associated with the edema, the possibility of nephrotic syndrome is
suspected. One must then consider the various types of glomerular disease that may produce this
combination.
Chronic liver disease with cirrhosis
Lab Studies:
The key to determining that renal disease is responsible for the initial clinical presentation is an
examination of the urine for protein and cellular elements. Most patients with MCNS have
proteinuria without hematuria, but the presence of microhematuria does not eliminate this
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diagnosis from consideration. The proteinuria predominately is selective with a high ratio of
urinary albumin to globulin (UA/G). In patients with FSGS, proteinuria is less selective but still
more so than in patients with MPGN. In individuals with MPGN, hematuria routinely is present.
Although measurements of protein selectivity are not performed routinely in the United States,
clinical studies from Europe confirm the utility of this test.
The magnitude of proteinuria varies with the state of disease activity and with the histologic
abnormality.
o
o
Patients with MCNS and MN usually excrete larger amounts of protein than patients
with other histologic subtypes. However, MN is rare in children.
The major protein excreted in any form of NS is albumin, but the UA/G ratio in excreted
urine is influenced greatly by the histologic subtype.
Hematuria may be present, and its frequency depends on the subtype of NS.
o
o
Although unusual in persons with MCNS, macroscopic hematuria has been reported.
The concentration of serum albumin compatible with NS is less than 2.5 g/dL.
The serum concentration of albumin is correlated indirectly with the magnitude of
proteinuria; thus, patients with MCNS, in general, have lower concentrations than those
with other forms of NS.
Values as low as 0.5 g/dL are not uncommon.
Hyperlipidemia is a common feature of NS and, in general, correlates inversely with the

concentrations of serum albumin.
o
o
Microscopic hematuria is present at the onset of the disease in 20-30% of patients with
MCNS, but it disappears thereafter. By contrast, microscopic hematuria is consistently
present in 80-100% of patients with MPGN and in 60% of patients with MN.
Patients with FSGS have hematuria more often than patients with MCNS, but the
presence of hematuria cannot be used to distinguish between the 2 conditions.
Hypoalbuminemia is the second of the cardinal laboratory features of NS, whatever the
histopathologic subtype.
o
o
The amount of protein in a random urine sample of a patient with NS usually exceeds
100 mg/dL, and values as high as 1000 mg/L are common. Protein-to-creatinine ratios
(normal <0.2) may vary from 1 to 10, suggesting 24-hour protein excretions of 1-10 g.
According to the ISKDC definitions, the lowest amount of urinary protein consistent
with the diagnosis of NS is 40 mg/m2/h or approximately 1000 mg/m2/d.
The low-density lipoprotein and very-low-density lipoprotein cholesterol concentrations

are elevated, whereas the high-density lipoprotein cholesterol level is decreased.
Values for lipids may remain moderately elevated for as many as 1-3 months after
complete remission of proteinuria.
Renal function is normal in most children at onset of NS.
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However, some patients with MCNS may have a mild-to-moderate reduction of GFR,
possibly due to the decrease in effective blood volume; the values usually return to
normal as diuresis occurs.
Marked reductions in renal function reflected in a persistent elevation of serum

creatinine indicate a histologic subtype other than MCNS.
Other lab study results are as follows:

o
Elevated values of hemoglobin and hematocrit are common when the patient has marked
hypoalbuminemia, and these elevated values are caused by the contracted PV.
Elevated WBC counts occasionally are observed, even in the absence of infections.
Hyponatremia is factitious: the amount of water (in which sodium is dissolved) per unit
of serum volume is reduced, due to hyperlipidemia. The occurrence of this
pseudohyponatremia is declining with the routine use of ion selective instruments to
measure the serum sodium concentration
Hypocalcemia (low total serum calcium) is common in patients with active NS due to
hypoalbuminemia; however, the ionized serum calcium is usually within reference
range.
Serum complement levels are within reference range, except in patients with MPGN.
Imaging Studies:
No routine imaging studies are indicated in patients with NS.
On chest radiographs, pleural effusions are not uncommon and their presence correlates directly
with the degree of edema and indirectly with the serum albumin concentration. Ascites is
common.
Renal ultrasonography usually reveals normal to slightly enlarged kidneys with normal
echogenicity. Patients with MPGN often demonstrate larger than normal renal shadows with
increased echogenicity.
Procedures:
A renal biopsy usually is not performed until after a therapeutic trial of glucocorticoids has
proved to be unsuccessful. Exceptions to this general rule may be made in the following
situations:
o
A child older than 10 years at onset of NS
Coexistence of significant hematuria, hypertension, and azotemia at the onset of NS
Any child in whom the levels of serum complement (or C3) are depressed
Histologic Findings: The histologic findings on renal biopsy tissue vary with the subtype of NS.
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In MCNS the morphology, by LM examination, is not different from normal. The

immunofluorescence results are negative, and no deposits are observed on electron microscopy
(EM). The only significant change observed on EM examination is fusion of the epithelial cell
podocytes.
Focal global glomerulosclerosis describes, as the name indicates, the presence of a few
completely sclerosed glomeruli, with the other glomeruli normal. The meaning of such a lesion is
not certain, because of the normally occurring glomerular attrition. For this reason, focal global
glomerulosclerosis is considered significant only if more than 5% of the glomeruli are sclerosed
globally.
In FSGS, the sclerosis or hyalinosis affects only part of the glomerular tuft of only some of the
glomeruli; the remaining glomeruli are normal. Because this lesion is focal and often confined to
the juxtamedullary nephrons, it may be missed on renal biopsy examination. Immunofluorescent
microscopy yields a variable picture. In some patients all classes of immunoglobulins and
complement appear to be trapped in the sclerotic area; in others, distinct immune-complex-type
deposits are found, particularly IgM. In children with NS, 7-10% have this lesion at onset, and
the lesions of 70-80% of such individuals fail to respond to standard steroid therapy. Clinically,
the disease presents in a fashion indistinguishable from MCNS. Two clinical forms of FSGS may
exist, one that is observed early in patients whose conditions are not responsive to steroids and
another that occurs late during the course of the disease in patients with long records of response
to steroid therapy. The FSGS that is observed early more often is associated with early renal
failure.
Mesangial proliferative glomerulonephritis (MPN) only recently has been distinguished from
MCNS, and some examples of MCNS with mesangial alterations may be inappropriate. LM
reveals minimal-to-moderate proliferation of the mesangial cells, with some mesangial
expansion; however, the most striking change is observed with immunofluorescent microscopy,
in which IgM, immunoglobulin G, and C3 often are observed.
MPGN occurs in approximately 8% of new unselected children with NS, and in more than 95%
of these patients, symptoms are unresponsive to standard steroid regimens. The histopathologic
findings are distinct, and all glomeruli are involved. At least 2 varieties of MPGN can be
described, both with proliferation of cells and extensive immune deposits as demonstrated by
immunofluorescent microscopy and EM.
MGN accounts for only 1-2% of childhood NS. With well-developed lesions, findings on LM
examination are typical, but detection of early lesions requires the detail afforded by
immunofluorescent microscopy and EM.
Other lesions, including proliferative glomerulonephritis (GN) and chronic GN, occur in
approximately 5% of children with NS, but their clinical presentations usually are so different
from that of MCNS that they are distinguished easily. The histologic picture varies with the
specific etiology.
TREATMENT
Medical Care: The following discussion is confined to the initial treatment of MCNS. Both specific
(steroids) and nonspecific treatments (eg, diet, diuretics, antihypertensives) are considered. Treatment of
patients whose symptoms do not respond to steroids and those who relapse is discussed in Prognosis.
Specific therapy
o
Initial
Glucocorticoid (steroid) therapy has changed the morbidity and mortality of NS

and is considered standard. After contraindications to the use of high-dose
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glucocorticoid drugs are eliminated (ie, active infection), oral prednisone or

prednisolone is started in a dose of 2 mg/kg/d (60 mg/m2/d).
The total daily dose is usually divided into 2-3 doses and administered daily for
4-6 weeks. Approximately 90% of patients with MCNS respond to this therapy
with complete clearing of proteinuria; however, approximately 20% of children
with FSGS and 7% of those with MPGN experience a clinical remission
(defined as a diuresis without complete clearing of proteinuria). Most children
with MCNS experience response at day 10-14 of such therapy, but a full 6
weeks of daily therapy still is recommended. While higher dosages or longer
courses of daily steroids do not significantly change the response rate in
persons with MCNS, they do increase steroid toxicity.
Refer children with unresponsive symptoms (ie, with complete clearing of
proteinuria) to a pediatric nephrologist for a percutaneous renal biopsy and
consideration of an alternative plan of treatment.
Maintenance: Following the 6 weeks of daily therapy, the standard recommendation is
for the dose (of either prednisone or prednisolone) to be reduced to approximately 1.5
mg/kg/d (40 mg/m2/d) administered as a single dose every other morning for an
additional 4- to 6-week period. This author believes that such a brief period of
maintenance therapy is associated with a higher rate of early recurrence than that
observed in patients in whom the maintenance therapy is continued for 3-6 months.
Thus, the author's recommendation, which is not based on a controlled study, is as
follows:
First 4 weeks - Intensive daily treatment - as above
Next 8 weeks - 1.5 mg/kg/d (once per day, every other morning)
Second 8-week period - 1 mg/kg/d (once per day, every other morning)
Third 8-week period - 0.5 mg/kg/d (once per day, every other morning)
After 29 weeks - Therapy stopped
Nonspecific therapy
o
Infection: The child who is nephrotic is a prime candidate for infection. When present,
infection has the potential for dissemination, particularly during steroid administration.
Thus, closely examine children with NS when febrile, and perform appropriate
laboratory studies without delay. If the child is from an environment conducive to
tuberculosis, perform a purified protein derivative (PPD) skin test. If found to be
positive for tuberculosis, the child should be investigated and treated appropriately. To
help prevent various infections, the child should be in relative reverse isolation to
minimize exposure to infectious diseases. This is particularly true during periods of
intensive therapy. Documented infections should be treated aggressively, but
prophylactic therapy is not indicated. Because of the propensity of nephrotic children for
pneumococcal infections, vaccination is indicated after remission is induced.
Diet: Children with active NS have the tendency to retain sodium. Therefore, a diet low
in sodium is indicated. Limitation of fluid intake is unnecessary unless the child's thirst
is so stimulated that intake is excessive. The remainder of the diet should be normal.
Alterations in protein intake are not indicated.
Diuretic therapy: Diuretic therapy may be beneficial, particularly in children with
symptomatic edema. The loop diuretics (eg, furosemide) administered orally in usual
amounts (approximately 1-2 mg/kg/d) are safe and moderately effective. Administer
loop diuretics with care because PV contraction already may be present, and further
sodium and fluid loss may result in hypovolemic shock. Simultaneous infusion of saltpoor albumin (usually at 1 g/kg body weight administered IV over 1-2 h) enhances the
diuretic response. Diuretics other than the loop diuretics (eg, thiazides, spironolactone,
metolazone) generally are not potent enough alone to effect diuresis but may have an
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added effect when combined with furosemide. Metolazone (with or without

spironolactone) may be beneficial in combination with furosemide for resistant edema.
Carefully monitor patients on this regimen because of the high risk of volume depletion.
o
Antihypertensive therapy: Hypertension rarely is observed in patients with MCNS.

When present, the hypertension may respond to diuretics; however, just as often, such
therapy can be ineffective or even can increase BP, presumably by further reducing renal
perfusion and thus enhancing the production of renin. Both angiotensin-converting
enzyme inhibitors and calcium channel-blocking agents have been used effectively in
such patients.
Deterrence/Prevention:
Although no proven method exists, limiting the occurrence of relapses is desirable. Many
relapses occur following respiratory illnesses. Thus, an attempt to limit exposure to subjects with
RTIs may be beneficial.
Exacerbations of NS also appear to occur following routine immunizations. Delaying routine

immunizations until the child is in remission and off medications for approximately 6 months is
desirable.
Complications:
Glomerular lesion
o
o
o
o
o
o
The rate of complications observed is related directly to the subtype of glomerular

lesion; thus, patients whose NS is secondary to MCNS have few complications, while
those with FSGS, MPGN, and MGN experience high rates of complications.
Acute renal failure has been noted in individuals with all types of NS but is rare in
persons with MCNS.
Tubulointerstitial nephritis is unusual in individuals with MCNS and common in persons
with other histologic subtypes.
In some patients with NS, tubulointerstitial involvement is associated with glucosuria
and aminoaciduria. The presence of these urinary findings suggests the possibility that
FSGS is the underlying cause of the nephrotic syndrome.
Hypertension is a frequent consequence of MPGN and FSGS.
Progression to CRF is uncommon in individuals with MCNS; however, CRF is observed
with increasing frequency in persons with MGN, FSGS, and MPGN.
Hypoproteinemia
o
The massive loss of urinary protein induces a degree of protein malnutrition in all
children with NS; children with NS that fails to respond to therapy and are constantly in
a negative protein balance are at greater risk of growth failure and other aspects of
malnutrition.
Hyperlipidemia is the direct result of increased hepatic production of lipids and
lipoproteins and is related to the degree and duration of the hypoproteinemia.
Hyperlipidemia rarely is of major consequence in patients with MCNS; however, in
patients whose condition does not respond to therapy or who have one of the other
histologic subtypes, chronic hyperlipidemia may become a major health risk of
cardiovascular complications. A role for hyperlipidemia in the progression of the renal
disease also has been postulated.
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The low serum protein concentrations are not solely due to albuminuria. Even in patients
with selective proteinuria, other small molecular weight anionic proteins are lost in the
urine, and serum levels are depleted. Some of these proteins (eg, transferrin) are
important for transport of other substances (eg, iron), and consequences may ensue. The
loss of opsonins appears responsible for the increased propensity for peritonitis. A loss
of some of the anticoagulant proteins may be responsible, at least in part, for the
increased tendency for thrombosis, either venous or arterial.
Drug therapy
o
The primary treatment medication, prednisone or prednisolone, has a very broad range
of significant adverse effects. The rate of complications observed with these steroids
depends on the dosages used, the frequency of dosing, and the duration of such
treatment.
Mild-to-moderate symptoms (eg, behavioral changes, increased appetite) and

cushingoidlike signs (eg, moon facies, truncal obesity, hirsutism) are common during the
first 6 weeks of daily therapy but usually begin to subside during the maintenance
therapy period and, if steroids are discontinued successfully, usually disappear
completely within 3-6 months.
If longer periods of steroid therapy are required, the risk of complications increases. In
addition to an exaggeration of those mentioned above, the complications are more
serious neurobehavioral changes (including mild psychoses), obesity, growth arrest,
osteopenia, osteoporosis, cataracts, hypertension, hyperglycemia, nephrolithiasis,
hyperlipidemia, and others. Incidence of these complications increases greatly with
repeated episodes of daily steroid administration.
If steroids must be used for long periods of time, limit the periods of daily steroid
administration.
While the administration of alternate morning steroids does not remove the risk of such
complications, it does lower the risk.
o
o
All of the other drugs used for the treatment of NS (eg, diuretics, antihypertensive
agents, immunosuppressive agents such as cyclophosphamide, chlorambucil,
cyclosporine) have significant individual toxicities. Carefully assess their benefit-to-risk
ratios.
Prognosis:
The prognosis for NS in children depends on the renal histologic findings, and, in large measure,
the expected outcome determines the optimal plan of follow-up care. The discussion below
focuses primarily on the prognosis for children with MCNS, which is the histologic subtype that
accounts for 80% of NS in children.
Patients whose symptoms initially do not respond to prednisone: Complete unresponsiveness to

steroids (with persistence of proteinuria and edema) or partial unresponsiveness (persistence of
proteinuria following diuresis and resolution of edema) occurs in approximately 8% of children
with MCNS.
o
On LM examination, the kidneys of such children do not appear different from the
kidneys of children whose symptoms initially respond to steroids. However, many of the
patients without steroidal response have IgM in the mesangial area, and this may
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constitute a different subgroup. Some of these children also may have unrecognized
FSGS.
The few patients with MCNS who are still proteinuric after 6 weeks of daily prednisone
therapy ultimately respond during the period of alternate-day prednisone therapy or if
alternate-day therapy is continued beyond 6 weeks.
Seriously consider a diagnosis of FSGS in children who remain nephrotic after a

standard course of prednisone therapy (eg, 6 wk daily and 6 wk of alternate-day
prednisone in prescribed doses). In such patients, consider therapy with an alkylating
agent, such as cyclophosphamide, chlorambucil, or nitrogen mustard.
Controlled studies by the ISKDC demonstrated that cyclophosphamide
significantly reduces morbidity by shortening the interval between beginning
therapy and time of response when compared to continued alternate-day
prednisone alone.
Other studies by the same group have demonstrated the ineffectiveness of
azathioprine in shortening this response interval.
Anecdotal reports have documented a response to other alkylating agents as
well as to cyclosporine A.
Renal biopsy usually is indicated in such patients.
Steroid responders: Approximately 92% of children with MCNS have a full response
(disappearance of proteinuria) to a standard course of steroids. Thereafter, the course of the
patients varies considerably. They can be divided arbitrarily into the following 3 groups:
o
Nonrelapsers: Patients in this category account for approximately 30% (range of 2050%) of children with MCNS whose symptoms initially respond to steroids. Detailed
analyses of various characteristics of NS and the initial therapeutic regimen have not
demonstrated conclusive differences between these children and those who relapse.
Thus, these patients are assumed to be cured of their disease.
Infrequent relapsers: The criteria for inclusion in this group are defined in different ways
by various investigators, and this accounts for the marked differences in incidence (2050% of patients with MCNS). Generally, the first relapse occurs after a remission of 3
months or more, and the number of relapses does not exceed 3 per year. Relapses
frequently are associated with a respiratory illness (eg, infection, allergy). The relapses
tend to respond promptly (10-14 d) to steroids. The prognosis for permanent remission
is good, and the progression to steroid resistance, renal failure, or both is negligible.
Institute therapy promptly, after 2-3 days for children who suddenly develop
significant proteinuria (2+ or more by dipstick), to prevent development of
edema.
Administer prednisone (2 mg/kg/d or 60 mg/m 2/d in 3 divided doses) until
proteinuria has resolved for 3 days; then administer prednisone on alternate
mornings (1.5 mg/kg/d as a single dose) for 6 weeks.
A tapering schedule may be effective at times.
Frequent relapsers: These patients present a potentially serious problem, not so much
from progression of the renal disease but from the adverse effects of prolonged steroid
therapy; even maintenance prednisone (alternate-day or other forms) has adverse effects.
Increasing numbers of children with prolonged maintenance steroid regimens ultimately
have exhibited growth failure, cataracts, osteoporosis, hyperglycemia, behavioral
disturbances, and gastrointestinal symptoms.These complications are compounded
when, in addition to prolonged maintenance therapy, the child must receive multiple
courses of daily steroids because of frequent symptomatic recurrences. In these children,
the initial relapse usually occurs within the first 3 months after initial response and
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frequently occurs within the first few weeks; the relapses may or be associated with a
respiratory illness, and they may occur at a frequency of more than 3 per year.
Symptoms of some such individuals subsequently fail to respond to steroids. In this
author's experience, mesangial deposits of IgM are more likely to be found in these
children than in the other groups. Patients who frequently relapse have been reported to
account for 25-50% of children with MCNS, with an approximate median figure of
35%.
Cyclophosphamide, chlorambucil, nitrogen mustard, and cyclosporine A
sometimes are effective in the treatment of these children. When
cyclophosphamide was used in a controlled trial in children who relapsed
frequently, more than 50% were still in remission (and receiving no therapy) 23 years later. Even in children who do not achieve long-term remission, such
therapy tends to increase the intervals between exacerbations and increase
sensitivity to prednisone therapy. Unfortunately, as many as 20% of children
derive little or no benefit from this therapy.
The potential for long-term consequences from cytotoxic therapy (eg, sterility,
teratogenesis) is real and must be weighed against the desirable effects. A
number of recent reports proclaim the advantages of cyclosporine in this group
of children.
Currently, cyclosporine may be the second-most common specific agent used in
the treatment of persons with NS. Some patients whose conditions initially
respond to cyclosporine ultimately develop a dependency on continued
cyclosporine use. Many of the children whose symptoms initially respond to
cyclosporine relapse immediately after the drug is discontinued; some do not
respond to renewed therapy.
If cytotoxic therapy is ineffective in prolonging remission or if a child in
remission returns to a frequently relapsing course, the alternatives are limited
and not very effective. If the child's remission can be maintained with alternateday administration of steroids and if adverse effects are negligible, then this is
the appropriate course. Bolus doses of methylprednisolone, administered
intermittently, have had some success. If steroid toxicity becomes a factor, the
most appropriate treatment may be cyclosporine A until the steroid toxicity
subsides.
A renal biopsy to check for signs of cyclosporine toxicity is indicated at early
intervals.
Nonsteroidal anti-inflammatory agents may be used to decrease proteinuria.
o
Initial and late nonresponders: Most of these patients either have FSGS that is not
evident on the renal biopsy sample or subsequently develop FSGS. Although likely, the
evolution of MCNS to FSGS has not been documented definitively.
Glomerulonephritis (GN)
Background: Bright initially described acute glomerulonephritis (GN) in 1927. Acute poststreptococcal
glomerulonephritis (PSGN) is the archetype of acute GN. Acute nephritic syndrome is the most serious
and potentially devastating form of various renal syndromes. Acute GN is characterized by the abrupt
onset of hematuria and proteinuria, often accompanied by azotemia (ie, decreased glomerular filtration
rate [GFR]) and renal salt and water retention.
Pathophysiology: Acute GN has 2 components: structural changes and functional changes.
Structural changes
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Cellular proliferation: This leads to an increase in the number of cells in the glomerular tuft
because of the proliferation of endothelial, mesangial, and epithelial cells. The proliferation could
be endocapillary (ie, within the confines of the glomerular capillary tufts) or extracapillary (ie, in
the Bowman space involving the epithelial cells). In extracapillary proliferation, proliferation of
parietal epithelial cells leads to the formation of crescents, a feature characteristic of certain
forms of rapidly progressive GN.
Leukocyte proliferation: This is indicated by the presence of neutrophils and monocytes within
the glomerular capillary lumen and often accompanies cellular proliferation.
Glomerular basement membrane thickening: This development appears as thickening of capillary
walls using light microscopy. Using electron microscopy, this may appear as the result of
thickening of basement membrane proper (eg, diabetes) or deposition of electron-dense material,
either on the endothelial or epithelial side of the basement membrane.
Hyalinization or sclerosis: These conditions indicate irreversible injury.
Electron-dense deposits: Such deposits could be subendothelial, subepithelial, intramembranous,
or mesangial, and they correspond to an area of immune complex deposition.
These structural changes could be focal, diffuse or segmental, and global.
Functional changes
Functional changes include proteinuria, hematuria, reduction in GFR (ie, oligoanuria), and active urine
sediment with RBCs and RBC casts. The decreased GFR and avid distal nephron salt and water retention
result in expansion of intravascular volume, edema, and, frequently, systemic hypertension.
Poststreptococcal glomerulonephritis [PSGN]
Previously M-protein of the organism was felt to be responsible for PSGN. These studies have been
discounted recently. Recently, nephritis-associated streptococcal cationic protease and its zymogen
precursor (NAPR) has been identified as a glyceraldehyde-3-phosphate dehydrogenase that functions as a
plasmin(ogen) receptor. This binds to plasmin and activates complement via alternate pathway. Antibody
levels to NAPR are elevated in streptococcal infections (of group A,C and G) associated with
glomerulonephritis, but are not elevated in streptococcal infections without glomerulonephritis, where as
anti-streptolysin-O titers are elevated in both circumstances. These antibodies to NAPR persist for years
and perhaps are protective against further episodes of PSGN.
Frequency:
In the US: GN comprises 25-30% of all cases of end-stage renal disease (ESRD). About one
fourth of affected patients present with acute nephritis syndrome. Most cases that progress do so
relatively quickly, and end-stage renal failure may occur within weeks or months of acute
nephritic syndrome onset. Asymptomatic episodes of PSGN exceed symptomatic episodes by a
ratio of 3-4:1.
Internationally: Geographic and seasonal variations in the prevalence of PSGN are more
marked for pharyngeally associated GN than for cutaneously associated disease.
Race: Postinfectious GN has no predilection for any racial or ethnic group. A higher incidence (related to
poor hygiene) may be observed in some socioeconomic groups.
Sex: Acute GN predominantly affects males (ie, 2:1 male-to-female ratio).
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Age: Postinfectious GN can occur at any age but usually develops in children. Outbreaks of PSGN are
common in children aged 6-10 years.
History: Taking a proper history is important and helpful.
Determine onset of disease: Ask the patient about onset and duration of illness.
Identify a possible etiologic agent (eg, streptococcal throat infection [pharyngitis], skin infection
[pyoderma]): Recent fever, sore throat, joint pains, hepatitis, travel, valve replacement, and/or
intravenous drug use may be causative factors. Rheumatic fever rarely coexists with acute PSGN.
Identify systemic disease (eg, arthralgia, diabetes).
Assess the consequences of the disease process (eg, uremic symptoms): Inquire about loss of
appetite, generalized itching, tiredness, listlessness, nausea, easy bruising, nose bleeds, facial
swelling, leg edema, and shortness of breath.
Identify clinical features: Inquire about edema, decreased volume and frequency of urination,
systemic hypertension, uremic symptoms, costovertebral tenderness (ie, enlarged kidneys [rare]),
and gross hematuria. Gross hematuria is the most common abnormality observed in patients with
acute PSGN and often manifests as smoky-, coffee-, or cola-colored urine.
Physical:
Signs of fluid overload

o
Periorbital and/or pedal edema
Edema and hypertension due to fluid overload - In 75% of patients
Crackles (ie, if pulmonary edema)
Elevated jugular venous pressure
Ascites and pleural effusion (possible)
Rash (ie, vasculitis, Henoch-Schnlein purpura)
Pallor
Renal angle (ie, costovertebral) fullness or tenderness, joint swelling, or tenderness
Causes: The causal factors that underlie this syndrome can be broadly divided into infectious and
noninfectious groups.
Infectious
o
Streptococcal: Poststreptococcal GN usually develops 1-3 weeks following acute

infection with specific nephritogenic strains of group A beta-hemolytic streptococcus.
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The incidence of GN is approximately 5-10% in persons with pharyngitis and 25% in

those with skin infections.
o
Nonstreptococcal postinfectious glomerulonephritis

Bacterial - Infective endocarditis, shunt nephritis, sepsis, pneumococcal
pneumonia, typhoid, secondary syphilis, meningococcemia, and infection with
methicillin-resistant Staphylococcus aureus (MRSA)
Viral - Hepatitis B, infectious mononucleosis, mumps, measles, varicella,
vaccinia, echovirus, parvovirus, and coxsackievirus
Parasitic - Malaria, toxoplasmosis
Noninfectious
o
Multisystem systemic diseases - Systemic lupus erythematosus, vasculitis, HenochSchnlein purpura, Goodpasture syndrome, Wegener granulomatosis
Primary glomerular diseases - Membranoproliferative GN (MPGN), Berger disease (ie,

immunoglobulin A [IgA] nephropathy), "pure" mesangial proliferative GN
Miscellaneous - Guillain-Barr syndrome, radiation of Wilms tumor, diphtheriapertussis-tetanus vaccine, serum sickness
Miscellaneous - Guillain-Barr syndrome, radiation of Wilms tumor, diphtheriapertussis-tetanus vaccine, serum sickness
Differential
Glomerulonephritis, Crescentic
Glomerulonephritis, Diffuse Proliferative
Glomerulonephritis, Membranoproliferative
Glomerulonephritis, Poststreptococcal
Glomerulonephritis, Rapidly Progressive
Goodpasture Syndrome
Hemolytic-Uremic Syndrome
Nephritis, Interstitial
Nephritis, Lupus
Other Problems to be Considered:
Postinfectious GN must be differentiated from the following conditions:

o
o
o
o
o
o
IgA nephritis: The latent period between infection and onset of nephritis is 1- 2 days, or
it may be concomitant with upper respiratory tract infection (ie, "synpharyngitic" in
contrast to 1-3 wk "postpharyngitic nephritis" in PSGN).
MPGN (type I, type II): This is a chronic disease, but it can manifest with an acute
nephritic picture with hypocomplementemia; failure of acute nephritis to resolve should
prompt consideration of this possibility.
Lupus nephritis: Gross hematuria is unusual in lupus nephritis.
GN of chronic infection: This can manifest as acute nephritis. Unlike PSGN, in which
the infection may have resolved by the time nephritis occurs, patients with nephritis of
chronic infection have an active infection at the time nephritis becomes evident.
Circulating immune complexes play an important role in the pathogenesis of acute GN
in these diseases.
Vasculitis: Nephritis of MRSA may have vasculitic lesions of the lower extremities.
Predominantly nonglomerular diseases: Thrombotic thrombocytopenic purpura,
hemolytic-uremic syndrome, atheroembolic renal disease, and acute hypersensitivity
interstitial nephritis may present with features of acute nephritic syndrome and should
be differentiated
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Lab Studies:
Urinalysis and sediment examination

o
These tests are crucial in the evaluation of patients with acute nephritic syndrome.
Look for protein, blood, RBCs and WBCs, dysmorphic red cells, acanthocytes, cellular
(ie, RBC, WBC) casts, granular casts, and oval fat bodies. In some instances, marked
sterile pyuria is present.
Finding RBC casts is an almost pathognomonic sign of GN.
Urine electrolytes, urine sodium, and fractional excretion of sodium (FENa) assays are
needed to assess salt avidity.
Blood, urea, and nitrogen (BUN); serum creatinine; and serum electrolytes (especially serum
potassium level)
Complete blood cell count
Erythrocyte sedimentation rate
Complement levels (C3, C4, CH50)

o
Low C3 levels are found in almost all patients with acute poststreptococcal nephritis; C4
levels may be slightly low.
Type III cryoglobulinemia may be present.
Twenty-fourhour urine test for total protein and creatinine clearance: Remember that creatinine
clearance is a "steady-state" measurement. The creatinine clearance may not reveal the true
picture because of rapidly changing renal function; therefore, it is better to wait until renal
function has stabilized before performing creatinine clearance.
Antistreptolysin-O titer (ASOT) or streptozyme titer: Increasing titer levels confirm recent
infection. In patients with skin infection, anti-DNase B titers are more sensitive than ASOT for
infection with Streptococcus.
Antibody to NAPR: Levels are elevated in streptococcal infections with GN but not in
streptococcal infections without GN.
If MRSA is the inciting agent, then hypocomplementemia is usually not present, but plasma
immunoglobulins, especially IgA, are markedly elevated.
Qualitative estimation of proteinuria: Determination of high-molecular weight (HMW) protein,

like fractional excretion of IgG (FEIgG), and low-molecular weight (LMW) protein, like alpha-1microglobulin, may help predict the clinical outcome and may help in guiding steroid and
immunosuppressive therapy, especially in patients with primary glomerular diseases with
nephrotic syndrome.
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Imaging Studies:
Abdominal ultrasound
o
Assesses renal size
Assesses echogenicity of renal cortex
Excludes obstruction
Procedures:
Generally, a renal biopsy is not necessary for diagnosis of acute PSGN; however, in most cases, it
is important because histology guides both prognosis and therapy.
Histologic Findings: Diffuse endocapillary proliferative changes are found (see Images 1-2). In
postinfectious GN, the glomerulus is hypercellular with marked cellular infiltration (ie,
polymorphonuclear neutrophils, monocytes). Immunofluorescence may show fine granular deposits of
immunoglobulin G in a "starry sky appearance (see Image 3). Large subepithelial deposits may be
observed on electron microscopy (see Image 4). Crescents may be observed
Medical Care: Treatment of acute PSGN is mainly supportive because there is no specific therapy for
renal disease. Treat the underlying infections when acute GN is associated with chronic infections.
Antimicrobial therapy
o
Antibiotics (eg, penicillin) are used to control local symptoms and to prevent spread of
infection to close contacts.
Antimicrobial therapy does not appear to prevent the development of GN, except if
given within the first 36 hours.
Loop diuretic therapy

o
o
o
Loop diuretics may be required in patients who are edematous and hypertensive in order
to remove excess fluid and to correct hypertension.
Relieves edema and controls volume, thereby helping to control volume-related
elevation in BP.
Vasodilator drugs (eg, nitroprusside, nifedipine, hydralazine, diazoxide) may be used if
severe hypertension or encephalopathy is present.
Glucocorticoids and cytotoxic agents are of no value, except in severe cases of PSGN.
Consultations: Nephrologist
Diet:
Sodium and fluid restriction - For treatment of signs and symptoms of fluid retention (eg, edema,
pulmonary edema)
Protein restriction for azotemic patients - If no evidence of malnutrition
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Activity: Recommend bed rest until signs of glomerular inflammation and circulatory congestion subside.
Prolonged inactivity does not benefit in the patient recovery process.
Treatment
Drug Category: Antimicrobials (antibiotics) -- In streptococcal infections, early antibiotic therapy may
prevent antibody response to exoenzymes and render throat cultures negative, but may not prevent the
development of PSGN
Penicillin V (Pen VEE K, V-Cillin K) -- More resistant than penicillin G to hydrolysis by acidic gastric
secretions and is absorbed rapidly after oral administration. 250 mg of penicillin V = 400,000 U of
penicillin
Drug Category: Loop diuretics -- Decrease plasma volume and edema by causing diuresis. The reduction
in plasma volume and stroke volume associated with diuresis decreases cardiac output and, consequently,
BP
Drug Category: Vasodilators -- Reduce SVR, which in turn may allow forward flow, improving cardiac
output
Drug Category: Calcium channel blockers -- In specialized conducting and automatic cells in the heart,
calcium is involved in the generation of the action potential. The calcium channel blockers inhibit
movement of calcium ions across the cell membrane, depressing both impulse formation (automaticity)
and conduction velocity
Deterrence/Prevention:
Early antibiotic therapy of streptococcal infection (ie, within 36 h of onset) may prevent
development of PSGN.
Antibiotic treatment of close contacts of the index case may help prevent development of PSGN.
Complications:
Renal failure (rare)
Pulmonary edema
Generalized anasarca and hypoalbuminemia (secondary to severe proteinuria)
Hypertension
Hypertensive encephalopathy
Prognosis:
Prognosis of acute PSGN is generally excellent in children.
Within a week or so of onset, most patients with PSGN begin to experience spontaneous
resolution of fluid retention and hypertension.
C3 levels may normalize within 8 weeks after the first sign of PSGN.
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Proteinuria may persist for 6 months and microscopic hematuria for up to 1 year after onset of
nephritis.
Eventually, all urinary abnormalities should disappear, hypertension should subside, and renal
function should return to normal.
Adults with PSGN, particularly older adults, may have a less favorable prognosis.
Few patients with acute nephritis develop rapidly progressive renal failure.
Nephritis associated with MRSA and chronic infections usually resolves after treatment of the
infection.
Immunity to type M protein is type-specific, long-lasting, and protective. Repeated episodes of

PSGN are therefore unusual.
Approximately 15% of patients at 3 years and 2% of patients at 7-10 years may have persistent
mild proteinuria. Long-term prognosis is not necessarily benign. Some patients may develop
hypertension, proteinuria, and renal insufficiency as long as 10-40 years after the initial illness.
SISTEM UROGENITALIA

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PROBLEM BASED LEARNING (PBL)

memecahkan masalah penyakit Urogenitalia yang disajikan.

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

patomekanisme, cara-cara diagnosis,

penatalaksanaan/terapi, komplikasi dan aspek epidemiologi penyakit-penyakit yang

Tujuan Instruksional Khusus (TIK)

menyebabkan pembengkakan pada muka dan perut:

Menguraikan struktur anatomi, histologi dan histofisologi dari sistim

Menyebutkan fungsi masing-masing bagian dari nefron, fungsi sel-sel

Menjelaskan faktor-faktor yang mempengaruhi GFR, prinsip hukum

Menjelaskan mekanisme dan proses reabsorbsi dan sekresi di tubulus,

Menjelaskan biokimia urine dan kompensasi ginjal dalam keseimbangan

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

Menjelaskan tentang penyebab penyakit-penyakit yang menyebabkan

Menjelaskan hubungan antara penyebab, respon dan perubahan jaringan

Menyebut penyebab dari penyakit yang menyebabkan pembengkakan

yang menyebabkan pembengkakan muka dan perut,

penyakit yang menyebabkan pembengkakan muka dan perut:

Menjelaskan tentang cara pemeriksaan fisik penderita penyakit-penyakit

Menggambarkan perubahan histopatologi

Menjelaskan fase pre-analitik, analitik & post analitik dari prosedur

Menganalisa hasil laboratorium pada penderita penyakit-penyakit di

Menjelaskan gambaran Rontgen dari saluran kemih yang normal,

yang menyebabkan pembengkakan muka dan perut:

Menyebutkan obat-obatan yang dipakai,

Menjelaskan farmakodinamik dan farmakokinetik obat-obat yang

Menjelaskan protokol/macam-macam cara yang dipakai pada SN yang

Menjelaskan paling kurang 8 istilah yang berhubungan dengan pengobatan pada

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

Menjelaskan asuhan nitrizi penderita dengan gejala pembengkakan pada

Menjelaskan tentang prognosis dari penyakit-pebyakit tersebut.

Menjelaskan tentang aspek epidemiologi penyakit-penyakit yang tersebut

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

Melakukan diskusi kelompok mandiri (tanpa tutor) , melakukan curah pendapat

Melakukan latihan dilaboratorium

FK-UNHAS Semester Akhir 2007/2008

keterampilan klinik dan praktikum di

PROBLEM BASED LEARNING (PBL)

PROSES PEMECAHAN MASALAH

Langkah 7 dilakukan dalm kelompok diskusi dengan tutor.

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

Memilih ketua dan sekretaris kelompok,

Brain-storming untuk proses 1 5,

3. Pertemuan ketiga: diskusi tutorial 2

seperti pada tutorial 1. Tujuan: untuk

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

didiskusikan pada kelompoknya. Laporan ditulis dalam bentuk laporan penyajian

Laporan penyajian kelompok dan perorangan serta semua laporan kasus

Semua laporan akan diperiksa dan dinilai oleh pakarnya masing-masing.

Semua mahasiswa wajib menyalin laporan dari kelompok dan mahasiswa

FK-UNHAS Semester Akhir 2007/2008

PROBLEM BASED LEARNING (PBL)

BAHAN BACAAN & SUMBER INFORMASI LAIN

A. Buku Ajar dan Jurnal

Grant BJC The perineum and Pelvis : a Method of Anatomy