Disorders of the Kidney Flashcards
(32 cards)
oligohyrdaminos
Infants born with congenital kidney disorders result from low levels of amniotic fluid (AF) during fetal development
Nonfunctional kidneys or outflow obstruction of urine from the kidneys in infants causes the amount of amniotic fluid to be small.
agenesis
is when one or both of the kidneys do not develop
Unilateral is survivable. Bilateral is not
Facial features: the eyes are widely separated and have epicanthic folds, the ears are set low, the nose is broad and flat, chin is receding
Previously called Potter syndrome
The most life threatening component of this syndrome is - no amniotic fluid to cushion fetus.
renal hypoplasia
is when kidneys do not develop to normal size
and contain fewer lobes
may be bilateral or unilateral
some cases number of nephrons is reduced, nephrons are larger as the kidneys work harder to compensate, thus increasing risk for glomerular disease
renal dysplasia
is an abnormality where cells are malformed during embryonic development (differentation) and cause tubules to form cysts (cystic dysplasia)
ectopic kidneys
kidneys lie outside normal position
kinking of ureters and obstruction of of urinary flow may occur
horseshoe kidneys
kidneys (upper and lower poles of the two kidneys are fused)
Autosomal Dominant Polycystic Kidney Disease
ADPKD
Etiology and Pathogenesis
- Cysts arise in segments of the renal tubules from a few epithelial cells
- High proliferation rate of epithelial cells lining the cysts
- Defective basement membrane - underlying abnormal epithelium allows for dilation and cyst formation
- Cysts detach and grow
- Compression causing loss of renal tissue functioning
Clinical Manifestations
- Asymptomatic early and if cysts are small
- Pain
- Gross hematuria from bleeding into a cyst
- Infection
- Hypertension - compression of intrenal blood vessels
Autosomal Recessive Polycystic Kidney Disease
- Cystic dilation of cortical and medullary collecting tubules
- Smooth kidney surface
- Fibrocystin - gene product responsible for regulation of cell proliferation
Clinical Manifestions
- Bilateral flank masses
- Restriction of lung growth (very large kidneys)
- Liver fibrosis and portal hypertension - diminished blood flow to liver
- Potter faces
Nephronophthisis - Medullary Cystic - Disease Complex - NPHP
Etiology and Pathogenesis
- Onset in childhood
- Small kidneys and cysts
- Distal tubule membrane disruption
- Chronic and progressive tubular atrophy
Clinical Manifestations
- polyuria
- polydispia
- enuresis (bed wetting)
- salt wasting
- growth retardation
- progressive renal insufficiency
Simple and Acquired Cysts
Etiology and Pathogenesis: -Common in older adults -Single or multiple -Unilateral or bilateral <1 cm, but can grow larger
Clinical Manifestations
- Most asymptomatic
- In symptomatic: flank pain, hematuria, infection, HTN
Hydronephrosis
Urine filled dilation of the renal pelvis and calices associated with progressive atrophy of the kidney due to obstruction of urine outflow
Etiology and Pathogenesis:
Obstructed outflow of urine Acute or insidious onset May occur at any level in urinary tract Leads to infection or stone formation Leads to anuria (failure to produce urine) and renal failure
Clinical Manifestations
- Unilateral may remain silent
- Pain
- Bilateral see oliguria (less urine)
- Inability to concentrate urine,
- Polyuria (too much urine) and nocturia
- Hypertension
Renal calculi (Kidney stones) Nephrolithiasis
Polycrystalline structures (formation of crystals that can grow) that kidney normally excretes
Etiology
- Supersaturated urine
- Nucleus present
- Deficiency of inhibitors for stone formation
Types of Stones
- Calcium
- Struvite
- Uric acid
- Cystine
Clinical Manifestations
- Pain
- Renal Colic Pain
- Non-colicky pain
Urinary Tract Infections
Asymptomatic bacteriuria, symptomatic infections,
cystisis (lower UTI)
pyelonephritis (upper UTI)
-Upper UTIs more serious than lower UTIs
Etiology Ecoli (common cause in lower UTIs) Washout phenomenon (urine from the bladder washes out urethra) Seen more in woman due to anatomy Urinary obstruction, reflux or neurogenic disorders -Sexually active -Pregnant -Post-menopausal and elderly women -Diabetes Indwelling catheter
Manifesations
-Neonates: fever, hypothermia, apnea, poor skin perfusion, abdominal distension, diarrhea, lethargy (signs of sepsis)
Older infants: feeding problems, failure to thrive, fever, vomiting, diarrhea
Children: fever is a common sign, frequency, dysuria
Adults: frequently no fever, frequency, dysuria, burning with urination lower abdominal or back pain, cloudy, foul smelling urine
Older adults: Often neurological change is first sign,
Asymptomatic or vague symptoms, anorexia, confusion, fatigue, weakness
Disorders of Glomerular function
Inflammatory process that involves the glomerular structures and induces glomerular cellular injury
Causative agents
- non-immunologic (infection, hypertension, drugs chemicals)
- immunologic (antibodies trying to attach to antigen)
- hereditary (rare)
Disorders of Glomerular function
Cellular changes can be
- Diffuse
- Focal
- Segmental
- Mesangial
Effects of Glomerular Disease
too much allowed through or too little goes through = depends on the disease
Manifesations
-Elevated BUN (blood urea nitrogen)
-Sudden insidious
onset of hypertension
-Proteinuria (loss of proteins in blood)
-Edema
-Decreased GFR leads to elevated plasma creatinine and urea (more waste)
-Glomerular capillary blood flow triggers mechanisms to increase BP
-Increased glomerular capillary permeability results in loss of plasma proteins
-Resulting hypoalbuminemia encourages plasma fluid to stay in interstitial spaces leading to edema.
Acute Nephritic Syndrome
Etiology
- Acute inflammatory process that occludes (blocks) the glomerular capillary lumen and damages the capillary wall.
- Characterized by decreased GFR
Clinical Manifestations
- Hematuria (cola colored urine)
- Proteinura (varies)
- Nitrogen waste buid up
- Oliguria
- HTN
- Edema
Acute Postinfectious Glomerulonephritis
Etiology:
- Occurs after infection with a certain strains of streptococcus (Group A Beta Hemolytic strep) 7-12 days after strep throat or other viruses
- Hypercellularity: infiltration of leukocytes (neutrophils and monocytes)
- Proliferation of endothelial and mesangial cells
Manifestations: Oliguria Proteinuria Hematuria (increased capillary wall permeability) Edema Hypertension
Rapidly Progressive Glomerulonephritis
Etiology:
- Rapid decline in GFR
- Focal and segmental proliferation of the glomerular cells
- Monocytes and macrophages recruited along with destruction of Bowman’s capsule
Manifestations
Rapid progression often within matter of months
Can progress to acute renal failure and death if untreated
Goodpasture syndrome
- Uncommon and aggressive form of glomerulonephritis
- Antibodies attack parts of glomerular basement membrane (GMB) and cross react with pulmonary alveolar basement membrane (affect kidneys and lungs - membranes share an antigen)
- Produce the syndrome of pulmonary hemorrhage associated with renal failure
- Caused by influenza, exposure to hydrocarbons
Nephrotic Syndrome
Etiology
- May be primary disorder (more common in children)
- Or secondary disorder (more common in adults) to systemic diseases such as diabetes, SLE, or amyloidosis
- Characterized by increased glomerular permeability
- At risk for thrombotic complications
- At risk for atherosclerosis
- Increase risk of infections
Manifestations
- Massive proteinura
- Hypoalbuminemia
- Lipduria
- Hyperlipidemia
- Hallmark is generalized Edema
- Initially dependent body parts, then becomes generalized as disease progresses
- Dyspnea (pulmonary edema)
Asymptomatic Hematuria or Proteinuria
Immunoglobulin A Nephropathy - Berger disease
- IgA immune complexes in mesangium (sit in extracellular fluid in glomeruli) of glomerulus lead to inflamation
- Most common cause of glomerular nephritis in Asians
- Onset usually in 20s and 30s
- Hematuria and mild proteinuria
Alport Syndrome - Hereditary
Hereditary defect in glomerular basement membrane
- X linked boys more severe (due to only one x chromosome)
- hematuria and chronic renal failure
