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Renal Development & Dysplasia Part I Hsiao Lai, MD Feb 19, 2010 ECU, Brody School of Medicine Nephrology and Hypertension Pediatric Nephrology Core Curriculum
Where will you encounter Renal Dysplasia? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Congenital Anomalies of  Kidney and Urinary tract  (CAKUT) ,[object Object],[object Object],[object Object],[object Object],[object Object],Woolf et. Al: in Renal Dysplasia Birth Defects Monitoring Program
Causes of Pediatric ESRD % 23 Other 3 PKD 3 Cystinosis 5 Nephronophthises 8 Congenital Nephrotic Syndrome 18 Acquired GN 40 CAKUT
Common Congenital Renal Anomalies ,[object Object],[object Object],[object Object],[object Object],[object Object]
Common Urogenital Anomalies ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Congenital Renal Anomalies ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Intermediate mesoderm
Renal Development ,[object Object],[object Object],[object Object],[object Object],[object Object]
Formation of Fetal Kidney ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Renal and Urogenital Development ,[object Object],[object Object],[object Object],[object Object]
Urogenital Development ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Later Stages of Nephrogenesis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Late Nephrogenesis
Mechanisms of Maldevelopment  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Edith Louise Potter ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Euro J Pediatrics 1994
Potter’s Syndrome ,[object Object],[object Object],[object Object]
Normal Kidneys
“ Dysplastic” Kidneys ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Multicystic Dysplastic Kidney ,[object Object],[object Object],[object Object]
Multicystic Dysplastic Kidney ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
MCDK
ARPKD ,[object Object],[object Object],[object Object],[object Object]
ARPKD ,[object Object],[object Object],[object Object],[object Object]
ARPKD Cortical cysts form in collectng duct
ADPKD on Fetal US
Radiographic Appearance of  ARPKD vs ADPKD
ADPKD kennythekidney.com
Renal Hypoplasia ,[object Object],[object Object],[object Object]
Decreased Nephron Number ,[object Object],[object Object],[object Object],[object Object],[object Object]
Duplex Kidney and Ureters ,[object Object],[object Object],[object Object]
Weigert-Meyer Law Upper Pole Moiety: obstructed Lower Pole Moiety: refluxes
Weigert-Meyer Law ,[object Object],[object Object],[object Object]
Duplex Kidney and Ureters ,[object Object],[object Object],[object Object]
 
Abnormalities of Renal Fusion, Ascent and Rotation  ,[object Object],[object Object],[object Object],[object Object]
Abnormalities of Ascent and Rotation Horseshoe kidney Cross fused ectopia
Horseshoe Kidney ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Crossed-Fused Ectopia ,[object Object],[object Object],[object Object]
Pelvic/Ectopic Kidney ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
 
Bladder Outlet Obstruction ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
UPJ Obstruction ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
UPJ Obstruction ,[object Object],[object Object],[object Object],[object Object]
UPJ Obstruction
Posterior Urethral Valves ,[object Object],[object Object],[object Object],[object Object],[object Object]
Posterior Urethral Valves ,[object Object],[object Object],[object Object]
PUV
Management PUV ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Congenital Syndromes with Renal Anomalies ,[object Object],[object Object],[object Object],[object Object]

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Renal Development Dysplasia 2

Notes de l'éditeur

  1. Intermediate mesoderm on either side of the dorsal body wall/notochord gives rise to 3 successive nephric structures of increasingly advanced design
  2. Pronephroi- nonfunctional segmental nephrotomes which develop in cervical region-represent a vestige of primitive kidneys seen in lower vertebrates, begin to appear at 24 days and regress in the fourth week Mesonephros – initial appearance of mesonephros and mesonephric duct at 25 days. Metanephric blastema and metanephric ducts appear at 28 days or end of week 4 Mesonephros develop in thoracic and lumbar regions during 4 th week, functional with complete but simple nephrons drained by the mesonephric (Wolffian Ducts) Nephric ducts grow caudally to open into the posterior wall of the primitive urogenital sinus by week 5 Metanephroi-definitive kidneys begin to form during 5 th week by induction of sacral intermediate mesoderm by UBs
  3. UBs sprout from the distal end of the mesonephric duct by week 5