Sri Suryanti, dr.

,SpPA
Bagian Patologi Anatomi FKUP/RSHS Bandung

GINJAL
Fungsi Ginjal: Membuang sampah produk metabolisme Mengatur konsentrasi air, garam dan keseimbangan asam basa tubuh Organ endokrin: eritropoeitin, renin & prostaglandin
Berat ginjal: 150 gram

Ureter ginjal hilum mayor calyx minor Cortex & medula Pembuluh Darah:

pelvis

calyx

Ginjal menerima 25% CO, cortex lebih vaskuler (90% dari total sirkulasi renal).

A.renalis dibagi menjadi anterior & posterior di hilum a.interlobuler a.arcuata (antara cortex & medulla) a.interlobular a.arcuata a.interlobular arteriole afferent-glomelurus arteriole efferent. Peritubuler vascular network vasa recta inner medulla venous vasa recta. Arteri ginjal sebagian besar merupakan end arteri, sehingga sering terjadi infark.

Urinary System

Left:The general organization of the kidney, Right: the cortical or medullary

Localization of nephron segments and collecting tubulus (the latter shown in black)

Vascular supply of the nephron in the outer zone of the cortex

GLOMERULI

Dinding kapiler dilapisi sel epitel visceral Sel epitel parietal melapisi kapsula Bowmans sel epitel tubulus Lumen kapiler sel endotel Sel mesangial: sel otot polos, kemampuan berkontraksi kontrol aliran darah ke glomerulus. Menghasilkan cytokinine fagosit

Tubulus

Tubulus proximal: microvilli, >>mitochondria fungsinya reabsorbsi 2/3 garam, air % glukosa

Glomerulus (Human); H&E Stain, X350 P=Proximal tubule D=Distal tubule J=Juxtaglomerular cells

Penyakit Ginjal

Penyakit anomali Penyakit glomerulus Penyakit yang mengenai tubulus & interstitium Penyakit pembuluh darah Obstruksi saluran traktus urinarius Urolithiasis Tumor ginjal

Penyakit Anomali
bisa herediter tapi sering karena defect akibat perkembangan selama masa kehamilan

Agenesis ginjal
Total bilateral lahir mati, sering bersamaan kel.congenital lain Unilateral jarang

Hipoplasia:
gagal dalam mencapai ukuran normal

Ginjal ectopic:
Kegagalan perkembangan definitive dari metanephros bisa di lokasi ectopic >rendah ureter berkelok-kelok obstruksi infeksi

Horshoe Kidney
Fusi dari pole bawah (90%) atau atas dari ginjal

KLASIFIKASI KISTA GINJAL

1.

2.

Cystic renal dysplasia Polycystic kidney disease

Autosomal dominant (adult) polycystic disease Autosomal recessive (childhood) polycystic disease

3.

Medullary cystic disease

Medullary sponge kidney Nephronothisis-uremic medullary cystic disease complex

4.

5. 6.

7.
8.

Acquired (dialysis-associated)cystic disease Localized (simple) renal cyst. Renal cyst in hereditary malformations syndromes Glomerulocystic disease Extraparenchymal renal cyst

1.

Cystic renal Dysplasia

abnormalitas diferensiasi metanephric, ditandai abnormal struktur: cartilago, mesenchim undiferensiasi, duktus colligentes immature, lobulus abnormal, Unilateral/ bilateral

Hypoplasia and Dysplasia which may be unilateral or bilateral Ask-upmark kidney:

The kidney is small, has a reduced number of pyramids, is divided by a deep furrow, and Is supplied by a narrow artery; the hypoplastic lobule consists of atrophic, thyroidlike tubules and hyalinized glomeruli

Findings in renal Dysplasia, some or all of which may be present in variable degree, either generalized or focally A: Primitive Pelvis B: Primitive duct surrounded by fibromuscular collar C: Primitive Ductules D: Primitive Tubules E: Primitive Glomeruli F: Cartilage Deposits G: Cysts H: Generalized Fibrosis

2. A. Autosomal dominant (adult) polycystic disease

Penyakit heriditer, ditandai multipel kista pada kedua ginjal, merusak parenkim ginjal kegagalan ginjal Awal: hanya mengenai sebagian nephron, gejala baru tampak pada dekade 5 kehidupan Kelainan genetik heterogen, mutasi pada:
Gen PKD 1 kromosom 16p 13,3

 Gen PKD 2 kromosom 4q13-23 Gen PKD 3 Makroskopis: ginjal s/d 4 kg, tebal dinding 3-4 cm Mikroskopis: struktur nefron diantara kista, dilapisi epitel gepeng, pertumbuhan hiperplastik polipoid Kista: hepar, lien, pankreas, dan paru-paru Intracranial Berry aneurysma

Polycystic Kidney Surface Aspect

Kidney Sectioned

Intravenous Pyelogram: Bilateral Polycystic Disease

2.B. Childhood Polycystic disease/ infantile

Autosomal resesif, bayi/ anak kecil gagal ginjal Makroskopis:

ginjal membesar, multikistik ~ busa

Mikroskopis:
Dilatasi duct colligentes Kista dilapisi epitel kuboid

Penderita hidup, congenital hepatic fibrosis

3. Medullary Cystic Disease

Medullary Spone Kidney Lesi tdd dilatasi multipel kistik ducy colligentes

o.d, autosomal/ dominan resesive Kista ukuran kecil pada medula, epitel kuboid/ transisional Pyelonephritis, nephrolithiasis dan fibrosis pada cortex

Nephronopthisis-Uremic medullary cystic disease

Masa anak-anak, penyakit ginjal progresif 20% CRF pada anak & remaja Makroskopis: ginjal kecil bergranuler, kista di corticomedullary junction Mikroskopis: kista di medulla, tubulus atrofi dan fibrosis interstitial

4. Acquired cystic disease

Dialisa dalam jangka waktu lama Kista medulla/ cortex Etiologi obstruksi di tubulus ok. Fibrosis/ kristal oxalat

5. Kista simplex

Multipel/ singel Uk: 1-5 cm, dinding: ep. Gepeng selapis. Kuboid autopsi

Summary of Renal Cystic Disease