Specific Diseases Flashcards
Alport syndrome aka familial nephritis
X-linked dominant
Females are still usually asymptomatic carriers
Males more likely to develop end stage renal disease.
Bilateral sensorineural hearing loss
ocular defects
renal failure
What are the most commonly palpated masses in infants?
Hydronephrotic kidneys multicystic dysplastic kidneys
Multicystic dysplastic kidney disease (MCDKD aka MCDK)
Unilateral flank mass Renal dysplasia Enlarged kidney with non-communicating cysts Thin/no parenchyma Kidney does not function, no treatment. Oligohydramnios minimal fluid in the bladder 50% of the time there are other urinary tract anomalies -UPJ obstruction -VUR -PUV -megaureter and duplication
First need US to confirm Dx.
2nd VCUG to check for other issues
Autosomal recessive polycystic kidney disease
Bilateral flank masses
Oligohydramnios
Congenital hepatic fibrosis
–>chronic portal hypertension
Autosomal dominant polycystic kidney disease
Adults
Need a renal ultrasound
Intracranial aneurysms
Ectopic urethral opening in females
Female always wetting her pants despite negative work up
Unstable bladder
Incontinence during the day, OK at night.
Leg crossing
squatting
treatment
- timed urination
- anti-cholinergic agents
UTI
> 50,000 colonies
E. coli
Enterococcus
Klebsiella
Adenovirus
UCx makes the Dx
When our boys most at risk for UTI?
0-3 months
Esp. uncircumcised
UTI treatment
7-14 days Amoxicillin-clavulanate Trimethoprim-sulfamethoxazole Cephalexin Cefixime Cefuroxime
IV Ceftriaxone Cefotaxime gentamicin tobramycin Piperacilin
UTI prophylaxis
Amoxicillin
trimethoprim-sulfamethoxazole
nitrofurantoin
Nephrotic syndrome
Proteinuria
Hypoproteinemia
Edema
Decreased UOP
Abdominal pain
Diarrhea
Weight gain
Normal renal fxn!
Liver upset by low oncotic pressure
VLDL increases –> high LDL/HDL ratio
Fibrinogen, factor V and VII increase –> Hypercoagulability
Lose immunoglobulins and complement proteins (immunodeficiency)
Lose albumin –> decreases bound/available Ca–> hypocalcemia
Lose thyroxine binding globulin –> functional hypothyroidism
Complications:
Hyponatremia
vascular thrombosis
peritonitis
Nephrotic syndrome epidemiology
Usually primary, Minimal change disease
Can be secondary: Infection Drugs Malignancies Lupus Diabetes
Males (2:1)
2-8 yo
Nephrotic syndrome treatment
Sodium restriction
prednisone
Hospitalize if incapacitating edema/infection.
Fluid restrict if severe edema
If proteinuria persists after 4 wks of prednisone then need renal biopsy. +/- cyclophosphamide or cyclosporine
Usually 1-2 relapses per year, resolves in adolescence
Nephrotic syndrome prognosis
#1: Worse if poor response to steroid tx >10 yo Persistent/gross hematuria HTN Renal insufficiency Low C3 complement levels
Nephritic syndrome
Due to a variety of glomerular disease
ROPE
Red urine (hematuria)
Oligouria
Proteinuria
Elevated BP and BUN (azotemia), edema
Causes of glomerulonephritis
1. Normal complement levels (HIGH) complement Henoch-Schonlein Purpura (IgA) Idiopathic vasculitis rapidly progressive GN FSGS
- Low complement levels
PMS. Not in the mood to complement.
Post-strep
MPGN
Systemic lupus
FSGS
Focal segmental glomerular sclerosis
Teenagers nephrotic syndrome usually progressive renal failure normal C3 levels low serum albumin Edema
MPGN
Membranoproliferative Glomerulonephritis
Low C3
Need aggressive treatment to prevent renal failure
Post-strep glomerulonephritis
PSGN
1-3 wks post throat or skin GAS (“recently ill”)
Immune complexes deposited on kidneys
Low C3 for ~2 months, then returns to normal
HTN
Edema (eyelids/face noticed by parent)
Hematuria (tea/cola colored)
Usually doesn’t –> renal failure
Low serum albumin b/c of hemodilution, NOT Proteinuria
PSGN Treatment
Supportive care
fluid restriction
blood pressure control
Gross hematuria is NOT recurrent
IgA nephropathy
Aka Berger’s disease
High IgA
IgA deposits on renal biopsy
Recurrent gross hematuria, painless
Mild abdominal pain
With or a few days after a URI/pharyngitis
Usually >10 yo
PSGN reasons to biopsy
Gross hematuria >8 wks Low serum complement HTN over 2 weeks Proteinuria over 6 months Abnl renal fxn
Hemolytic uremic syndrome
Clinical scenario
HUTS
Hemolytic anemia
Uremic (elevated BUN, renal failure)
Thrombocytopenia
Syndrome
E. Coli -poorly cooked meat, unpasteurized apple cider, cow and goat milk Diarrhea Hematuria Abdominal pain Decreased UOP Purpura, ecchymoses CNS issues HTN
Normal complement levels
Coombs test negative
Hemolytic uremic syndrome
Treatment
Supportive
Avoid antibiotics, they make it worse.
