Nephrology Flashcards
Post strep GN
- what age
- after what infection, time frame
age 5-15
after Group A beta hemolytic streptococcus
cellulitis- 5 days after if co-infection with staph aureus otherwise 3 weeks post infection
pharyngitis- 7-10 days after
what is the classical presentation of post strep GN
Hypertension- 50 to 90%
Gross hematuria and edema- 30 to 60%
what happens to C3 and C4 with post strep GN
ANA?
ASO titer?
urine?
low C3 (90% will be low at presentation) C4 normal ANA negative ASO titer may be elevated red cells, red cell casts, positive for leukocytes
when do ASO titers peak?
4 to 6 weeks after strep infection but may remain detectable for several months after the strep infection has resolved
when should your C3 normalize after post-strep GN
8 weeks
if it doesn’t normalize then think membranoproliferazive glomerulonephritis
microscopic hematuria persists for up to a year
List 4 diagnoses that can cause a low C3
post strep GN Lupus membranoproliferative glomerulonephritis subacute bacterial endocarditis shunt nephritis
what is the treatment for post strep GN
supportive fluid and salt restriction Furosemide (1-2mg/kg/day) treat hypertension- resolves by 2 weeks Short-acting or long-acting antihypertensive medications: Nifedipine/Hydralazine or Amlodipine
C3 level should return to normal by 6-8 weeks
proteinuria may last for 4 months
microscopic hematuria may last for 2 years though most cases resolved by 3-6 months
how is IgA nephropathy diagnosed?
renal biopsy
most common in adolescents
when do you see gross hematuria with IgA nephropathy?
Gross hematuria often occurs within 1-2 days of onset of an upper respiratory or gastrointestinal infection, in contrast with the longer latency period observed in acute postinfectious glomerulonephritis
gross hematuria gets better as they get better (typically only lasts 3-4 days)
what happens to C3 and C4 with IgA nephropathy
normal!!
Normal serum levels of C3 in IgA nephropathy help to distinguish this disorder from postinfectious glomerulonephritis.
what are the treatment options for IgA nephropathy
ACE, fish oil, corticosteroids
The primary treatment of IgA nephropathy is appropriate blood pressure control and management of significant proteinuria.
ACE inhibitors and angiotensin II receptor antagonists are effective in reducing proteinuria and retarding the rate of disease progression when used individually or in combination.
Fish oil, which contains antiinflammatory omega-3 polyunsaturated fatty acids, may decrease the rate of disease progression in adults
If a renin-angiotensin system (RAS) blockade proves ineffective and significant proteinuria persists, then addition of immunosuppressive therapy with corticosteroids is recommended.
what is alport syndrome
Alport syndrome (AS), or hereditary nephritis, is caused by mutations in type IV collagen, a major component of basement membranes. 85% are x-linked
what are the clinical features of alport syndrome
all patients have asymptomatic microscopic hematuria
hypertension
proteinuria (commonly progressive by 2nd decade of life)
renal failure
bilateral sensorineural hearing loss
what is seen on electron microscopy for alport syndrome? when do they need renal replacement therapy?
thin basement membrane
renal replacement therapy by 20-30 years of age
why do you refer alport syndrome to an ophthalmologist?
Ocular abnormalities, which occur in 30–40% of patients with X-linked AS, include ANTERIOR LENTICONUS, macular flecks, and corneal erosions.
Anterior lenticonus is pathognomonic for what?
Alport syndrome
what are 4 clinical features of Alport syndrome
AS is highly likely in the patient who has hematuria and at least two of the following characteristic clinical features: macular flecks recurrent corneal erosions GBM thickening and thinning sensorineural deafness.
what are the features of anti-GBM disease (Goodpasture disease)
autoimmune disease characterized by
- pulmonary hemorrhage
- rapidly progressive glomerulonephritis
- elevated anti–glomerular basement membrane antibody titers
- rare in children
what parts of the body are attacked by anti-GBM disease? good pasture syndrome
The disease results from an attack on these organs by antibodies directed against certain epitopes of type IV collagen, located within the alveolar basement membrane in the LUNG and glomerular basement membrane (GBM) in the KIDNEY
how do children present with anti-GBM disease
hemoptysis from pulmonary hemorrhage that can be life-threatening
usually systemic symptoms (malaise, fever, weight loss, arthralgia) are ABSENT
What is the treatment for anti-GBM disease?
plasmapheresis** to remove the antibody
high-dose intravenous methylprednisolone
cyclophosphamide
what is the most important cause of morbidity and mortality in SLE.
lupus nephritis (diagnosed by renal biopsy)
Diagnostic criteria for Lupus
SOAP BRAIN MD need 4/11 criteria Serositis – Pleuritis, pericarditis Oral ulcers Arthritis (2 or more joints) Photosensitivity
Blood disorders- hemolytic anemia, leukopenia <4 or lymphopenia <1.5 or platelets <100 000
Renal involvement- nephritis
Antinuclear antibodies- ANA
Immunologic markers (dsDNA, anti-Sm, anti-ro)
Neurologic disorder- seizures, psychosis
Malar rash
Discoid rash
what is the most common cause of a midline mass of the abdomen in a newborn
mesenteric cyst
you are asked to see a newborn in the nursery for an abdominal mass. what is the most likely origin of this mass? what is the likely diagnosis?
renal mass
hydronephrosis
multi cystic dysplastic kidney
what are the top 3 causes of renal masses
hydronephrosis
multi cystic dysplastic kidney
renal vein thrombosis
what would be your first line modality to evaluate the renal mass?
ultrasound
NS is _____meq/L of Na
154 mEq/L Na
154 mEq/L Cl
how do you calculate body surface area
√height (cm) x weight (kg) ÷ 3600
what is the equation for insensible losses
400mL/m2
what is your minimum glucose requirement
4-6mg/kg/min
what are 2 conditions that present with normal anion gap metabolic acidosis
- RTA
2. Gastroenteritis (diarrhea)
where are most things absorbed in the kidney
65% of anything that goes through your body is predominantly absorbed in the proximal tubule
Proximal RTA
pH <7 acidosis- HCO3 15 often hypoNa can have hypoPhos Glucosuria Mild tubular proteinuria or generalized aminoaciduria Fanconi's syndrome
Distal RTA
urine pH >7 acidosis- HCO3 can go below 10 sodium usually normal nephrocalcinosis may be present** Hypokalemia Hypercalciuria
what is the most common cause of proximal RTA
cystinosis
how do you treat distal RTA
potassium citrate
giving them bicarbonate
how do you treat proximal RTA
sodium citrate (because they are losing sodium through proximal tubule)
what are the 2 main causes of type 4 RTA
aldosterone deficiency
aldosterone resistance
what is the most common cause of distal RTA
idiopathic
what constitutes the single most important risk factor for future loss of kidney function
proteinuria
what is the most common cause of proteinuria in pediatrics?
transient proteinuria
- exercise
- fever
- infection
what is the definition of persistent proteinuria
proteinuria on 2 or 3 separate samples taken 1-2 weeks apart
- if you have a kid with proteinuria you have to repeat the sample 2-3x
<1% have persistent proteinuria
how do we measure urine protein
on urine dipstick
or urine protein/creatinine ratio
- don’t do 24h urine collection
what is a normal protein/creatinine ratio for infants <2 years of age
upc <50mg/mmol
what is a normal protein/creatinine ratio for children >2 years of age
upc <20mg/mmol
what are 3 causes of false positive protein on urine dipstick
semen
menstrual blood
vaginal discharge
what is orthostatic proteinuria
normal, persistant proteinuria
seen in adolescents
assess with two separate night and daytime samples
if am sample upc <20mg/mmol then the diagnosis can be made
Prognosis: No treatment is required; benign disease
what is the average age for idiopathic nephrotic syndrome
2-10 years
what are causes of idiopathic nephrotic syndrome
minimal change disease mesangial proliferation focal segmental glomerulosclerosis membranous nephropathy membranoproliferative glomerulonephritis
Approximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndrome.
what is the initial treatment for nephrotic syndrome
prednisone
2mg/kg/day for 6 weeks
then 1.5mg/kg/ day for alternate days for 6 weeks and then stop
most children respond within 5-10 days of starting steroids
95% with steroid sensitive nephrotic syndrome are in remission by 4 weeks
what is the definition of remission
urine protein/creatinine ratio <20 or urine dipstick negative - trace for 3 days
what is the definition of relapse
after remission, increase in proteinuria of 20g/l for 3 days