Congenital Kidney Disease Flashcards
Pathoma-125
Most common congenital renal anomaly ?
Horseshoe kidney
In horseshoe kidney location of kidney ?
Lower abdomen
Horseshoe kidney what just happened?
Conjoined kidneys usually connected at the lower pole
Horseshoe kidney process ?
Inf mesenteric artery
Ascent = From Pelvis —- Abdomen
Then= Cought on there
Absent kidney formation ?
Dx ?
Renal Agenesis
Renal agenesis type ?
- Unilateral
- Bilateral
Unilateral renal agenesis ?
- Hypertrophy
- Hyperfiltration = Inc risk of Renal F
125
Bilateral Renal ageneis ?
- Oligohydramnios
- Lung hypoplasia
- Flat face
- Low set ears
- Defects in extremities
- Potter sequence
Incompatible with life
Dx ?
Renal agenesis
Dysplastic kidney disease must be difference from ?
PKD
unilateral
Potter sequence in which renal congenital anomely ?
Renal agenesis
Dysplastic kidney which part of kidney affected ?
Renal parenchyma
Dysplastic kidney manifestation ?
- Noninherited
- Congenital malformation
- Cyst
- Abnormal tissue
Polycystic kidney Disease which side ?
Bilateral
PKD which site of kidney ?
Renal cortex & medulla
Young Adult manifestation of PKD ?
- HTN= inc Renin
- Hematuria
- Renal F worsening
PKD manifestation ?
Inherited defect + Enlarged kidney + Cyst
Association of PKD ?
- Congenital hepatic fibrosis
- Portal HTN
- Hepatic cyst
PKD fate in infants ?
- HTN
- Renal Failure
PKD newborn fate ?
Potter sequence
Association of PKD ?
- Hepatic cyst
- Berry aneurysm
- Mitral valve prolapse
125
Which mutation for cyst formation in PKD ?
APKD1
APKD2 gene
Medullary cystic kidney diseases?
inherited autosomal dominant defect=Medullary collecting ducts
Site of congenital anomely confusion ??
- Dysplastic kidney D=Renal parenchyma
- PKD = Renal cortex & medulla
- MCKD = Medullary collecting ducts
Inherited autosomal disease
MCKD presentation?
- Parenchymal fibrosis
- Shrunken kidney
- Worsening Renal F
