NEPHRO Flashcards
1
Q
definition of hematuria
A
presence of at least 5 RBCs per microliter of urine
2
Q
Heme positive without RBCs
A
presence of myoglobin or hemoglobin
3
Q
hematuria within the glomerulus
A
brown, cola or tea-colored or burgundy urine
protenuria >100mg/dl via dipstick
RBC casts
deformed urinary RBCs (acanthocyte)
4
Q
hematuria arising from lower urinary tract
A
gross hematuria, bright red or pink terminal hematuria blood clots normal unrinary RBC morphology minimal proteinuria on dipstick (<100mg/dL)
5
Q
tea or cola colored urine, facial or body edema, hypertension and oliguria
A
acute nephritic syndrome
6
Q
what is the most common cause of gross hematuria
A
bacterial urinary tract infection
7
Q
urethral bleeding in the absence of urine associated with dysuria and blood spots on underwear after voiding which occurs in prepubertal boys
A
Urethrorrhagia
8
Q
most common chronic glomerular disease
A
IgA nephropathy (Berger disease)
*IgA deposits in mesangium often accompanied by C3 complement
9
Q
diagnosis of IgA nephropathy requires
A
renal biopsy
*mesangial proliferation that may be associated with epithelial cell crescent formation and sclerosis
10
Q
what are the clinical manifestations of IgA nephropathy
A
gross hematuria often occurs 1-2 days of onset of an URTI or GI infection
11
Q
C3 level in IgA nephropathy is
A
normal
12
Q
primary treatment of IgA nephropathy
A
appropriate BP control
*other treatment: ACE inhibitors and ARB effective in reducing proteinemia and retarding rate of disease progression
13
Q
Alport syndrome is caused by mutations in
A
genes coding for Type IV collagen
14
Q
electron miscroscopy findings in Alport syndrome
A
diffuse thickening, thinning, splitting and layering of the glomerular and tubular basement membranes
15
Q
what are the clinical manifestations of Alport Syndrome
A
single or recurrent gross hematuria after URTI
progressive proteinuria exceeding 1g/24hr
bilateral sensorineural hearing loss
ocular abnormalities including anterior lenticonus, macular flecks and corneal erosions
16
Q
pathognomonic of Alport syndrome
A
anterior lenticonus
17
Q
sudden onset of gross hematuria, edema, hypertension and renal insufficiency
A
Acute Poststreptococcal Glomerulonephritis
18
Q
immunofluorescence microscopic findings in patients with PSGN
A
“lumpy bumpy” deposits of immunoglobulin and complement on the GBM and mesangium
19
Q
C3 levels in PSGN
A
low
20
Q
nephritogenic strain in PSGN
A
Group A streptococcus which posses M protein
21
Q
patient develop acute nephritic syndrome after ___ weeks after streptococcal pharyngitis and ___ weeks after streptococcal pyoderma
A
1-2 weeks after pharyngitis
3-6 weeks after pyoderma
22
Q
acute phase of PSGN resolves within
A
6-8 weeks
23
Q
commonly elevated after streptococcal pharyngitis
A
ASO
24
Q
best single antibody titer to document cutaneous streptococcal infection
A
anti-DNAse B level
25
treatment for PSGN
10 day course of Penicillin recommended to limit spread of nephritogenic organisms, antibiotic therapy does not affect the natural history of GN
26
most common cause of membranous glomerulopathy worldwide
Malaria
* diffuse thickening of the GBM without significant cell proliferative changes
* in situ immune complex formation
27
treatment for Membranous Glomerulopathy
Immunosuppressive therapy
28
also known as mesangiocapillary glomerulonephritis
Membranoproliferative GN
* most common in 2nd decade of life
* C3 levels remains persistently low
29
most common type of MPGN
Type I
* glomeruli have accentuated lobular pattern from diffuse mesangial expansion
* crescents if present signify poor prognosis
30
indications for renal biopsy
nephrotic syndrome in an older child
significant proteinuria with microscopic hematuria
hypocomplementemia lasting >/=2 months in a child with acute nephritis
31
most important cause of morbidity and mortality in SLE
Glomerulonephritis
32
minimal mesangial lupus nephritis
| no histologic abnormalities on light microscopy but mesangial immune deposits are present on IF or EM
WHO Class I nephritis
33
mesangial proliferative nephritis
WHO Class II nephritis
34
proliferative nephritis; <50% glomeruli involvement
WHO Class III nephritis
35
proliferative nephritis; >50% glomeruli involvement
WHO Class IV nephritis
36
membranous lupus nephritis
WHO Class V nephritis
37
therapy given in Lupus nephritis
Prednisone
38
may be given in WHO Class I or II Lupus nephritis
Azathioprine
39
most common small vessel vasculitis
HSP
40
clinical manifestations of HSP
purpuric rash
arthritis
abdominal pain
41
when should urinalysis be requested in patients with HSP?
weekly in patients with HSP during period of active clinical disease and once a month for up to 6 months thereafter
42
when should treatment be given to patients with HSP nephritis?
severe HSP nephritis
| >50% crescents on biopsy
43
hallmark of Rapidly progressive GN
crescents in glomeruli
44
characterized by pulmonary hemorrhage and glumerulonephritis
Goodpasture Disease
45
treatment for Goodpasture disease
high dose IV methylpred, cyclophosphamide and plasmapharesis
46
triad of HUS
renal insufficiency
thrombocytopenia
microangiopathic hemolytic anemia
47
most common cause of HUS
toxin-producing E. coli that cause prodromal acute enteritis
48
what are the early glomerular changes in HUS
thickening of capillary walls caused by swelling of endothelial cells and accumulation of fibrillary material between endothelial cells and underlying basement membrane causing narrowing of capillary lumen
49
characteristic of all forms of HUS
microvascular injury
| endothelial cell damage
50
prognosis in HUS
those with HUS not associated with diarrhea is more severe
51
common causes of renal vein thrombosis in infants and newborn
```
asphyxia
dehydration
shock
sepsis
congenital hypercoaguable state
maternal diabetes
```
52
common causes of renal vein thrombosis in older children
```
nephrotic syndrome
cyanotic heart disease
inherited hypercoaguable state
sepsis
following kidney transplantation
```
53
sudden onset of gross hematuria and unilateral or bilateral flank mass
Renal vein thrombosis
54
primary treatment for renal vein thrombosis
supportive care
correction of fluid and electrolytes
treatment of renal insufficiency
55
treatment for patients with thrombosis of inferior vena cava
can require surgical thrombectomy
56
triad of nephrotic syndrome
hypoalbuminemia
edema
hyperlipidemia
57
nephrotic range proteinuria
protein excretion >40mg/m2/hr or
| 1st morning protein:creatinine ratio >2-3:1
58
findings on kidney biopsy in patients with nephrotic syndrome
extensive effacement of foot process
| Hallmark of nephrotic syndrome
59
important features of minimal change idiopathic nephrotic syndrome
absence of hypertension and gross hematuria
60
treatment for minimal change disease
6 weeks course of daily steroid treatment
61
steroid resistant is defined as
children who continue to have proteinuria (2+ greater) after 8 weeks of steroid therapy --> diagnostic biopsy should be performed
62
normal anion gap (hyperchloremic) metabolic acidosis
Renal tubular acidosis
63
proximal RTA is also called
Type II
* fanconi syndrome
* cystinosis
* Lowe syndrome: congenital cataracts, mental retardation and Fanconi
64
what are the manifestations of Fanconi syndrome
```
LMW proteinuria
glycosuria
phosphaturia
aminoaciduria
proximal RTA
```
65
manifestations of Type II RTA
growth failure in the 1st year of life
| Urine pH is acidic (<5.5) because distal acidification mechanism is intact
66
distal RTA is also called
Type I RTA
67
what happens in distal RTA?
impaired hydrogen ion excretion hence urine PH cannot be reduced to <5.5 despite presence of acidosis
* hypokalemia
* hypercalciuria
68
RTa type IV is also known as
Hyperkalemic RTA
*impaired aldosterone production or impaired renal responsiveness to aldosterone
69
blood anion gap <12
absence of anion gap
70
blood anion gap >20
highly suggestive of presence of anion gap
71
positive anion gap suggests deficiency of
ammoniagenesis
72
negative anion gap is consistent with
proximal tubule bicarbonate wasting (GI bicarbonate wasting)
73
mainstay of treatment in all forms of RTA
bicarbonate replacement
74
urine shows elevated SG (>1.020), elevated urine osmolality (UOsm >500mOsm/kg), low urine NA <20meq/L, fractional excretion of sodium (FENa <1%)
prerenal ARF
75
urine shows SG (<1.010), low urine osmolality (UOsm <350 mOsm/kg), high urine NA >40meq/L, fractional excretion of sodium (FENa >2%)
intrinsic ARF
76
indications for dialysis in ARF
volume overload with evidence of hypertension and/or pulmonary edema
persistent hyperkalemia
severe metabolic acidosis unresponsive to medical tx
neurologic symptoms
BUN >100-150 mg/dl
Calcium:phosphorus imbalance with hypocalcemic tetany
77
principal extracellular cation
Sodium
78
principal intracellular cation
Potassium
79
positive water balance --> decreased osmolality
hyponatremia
80
negative water balance --> increased osmolality
hypernatremia
81
factors that promote potassium secretion
1. Aldosterone
2. Increased Na delivery to the distal nephron
3. increased urinary flow rate
82
role of kidney in acid-base balance:
| reabsorption of HCO3
proximal tubule
83
role of kidney in acid-base balance:
| secretion of H+ as titratable acid and ammonium
Distal tubule
84
characteristics of Renal tubular disorders
* usually inherited
* affected children have growth failure
* some disorders are benign and require no therapy
* disorders may be treated with replacement of substance being lost in the urine or removal of an offending substance or metabolite
85
presence of anion gap
>20
lactic acidosis
inborn errors of metabolism
ingestions
86
urine anion gap <0
RTA type II proximal
87
Urine anion gap >0
RTA Type I
| RTA Type IV
88
what are the manifestations of distal RTA (Type I)?
vomiting, failure to thrive, acidosis ( more severe than RTA)
Nephrocalcinosis
renal calculi
bone disease -->rickets or osteomalacia
* nephrocalcinosis
* hyperchloremia
* hypercalciuria
89
what is Bartter syndrome?
*rare form of hypokalemic metabolic alkalosis with hypercalciuria
*mutation in Na-K-Chloride contransporter
*2 types:
Antenatal
Classic
*damage at thick ascending limb
*severe hypokalemia <2.5mmol/L with metabolic acidosis
90
damage at thick ascending limb
Bartter syndrome
91
treatment for Bartter syndrome
*prevent/correct dehydration
*potassium supplementation --> 1-3 mEq/kg/day
*Administration of Indomethacin (type 2 are sensitive)
Type 4 resistant to Indomethacin
*Spinorolactone 10-15mg/kg/day
*may lead to chronic renal failure
92
What is Gitelman's syndrome
* familial hypokalemia-hypomagnesemia
* much common in adult
* involves thiazide-sensitive co-transporter located in DCT; diminished NaCl transport in DCT
* often asymptomatic
* transient episodes of tetany and weakness usually associated with abdominal pain, vomiting and fever
* salt-craving, musculoskeletal symptoms, constitutional symptoms
* growth retardation is absent
93
Treatment for Gitelman's syndrome
best treated with Mg salts alone
94
inability to concentrate urine
Nephrogenic Diabetes Insipidus
* damage to collecting tubule
* resistant to ADH
* presents soon after birth
* poor appetite, vomiting, FTT due to excessive water intake
* massive polyuria, hypernatremia, volume depletion, hyperthermia, polydipsia
95
laboratory findings in Nephrogenic Diabetes Insipidus
* polyuria: UO >2000ml/m2/day or 40ml/kg/day
* serum osmolality: >290 mOsm/kg
* urine osmolality: <290 mOsm/kg
* increased serum osmolality
* decreased serum osmolality
96
Definition of normal BP
Children 1-13: <90th percentile for age, sex, height
| Children >/=13: <120/ <80 mmHg
97
definition of elevated BP
Children 1-13: >90th to <95th percentile for age, sex, height
Children >/=13: 120/<80 mmHg
98
definition of Stage 1 hypertension
Children 1-13: >95th to <95th percentile + 12mmHg or 130/80 to 139/89 mmHg (whichever is lower)
Children >/=13: 130/80 to 139/89
99
definition of Stage 2 hypertension
Children 1-13: >95th + 12mmHg or >/=140/90mmHg (whichever is lower)
Children >/=13: >/=140/90 mmHg
100
BP should be checked starting at what age
3 years old
101
correct BP cuff size
* bladder width: approx 40% of the circumference of upper arm midway between olecranon and acromion
* bladder length: should encircle 80-100% of the circumference of upper arm
102
how to use the normative BP table
Step 1: recognition of elevated BP
* should be checked 3x then get average
* determine height percentile of the child using CDC growth chart
* use correct gender table for SBP and DBP
Step 2: evaluation of etiology
evaluate comorbidities, screen for evidence of target organ damage
103
screening tests for all patients with elevated BP
Urinalysis
BUN, Crea,
Lipid profile
Renal ultrasound
104
screening tests for obese BMI >95th percentile or adolescent
HBA1c
AST and ALT
Fasting lipid panel
```
optional:
Fasting serum glucose
DM
TSH
Drug screen
Sleep study
CBC especially those with growth delay or abnormal renal function
```
105
screening tests for patients with comorbid factors and BP 90th to <95th percentile; all patients with BP >/= 95th percentile
evaluate for target-organ damage:
Echocardiogram to identify LVH
retinal exam
106
screening test for all children with persistent BP >/=95th percentile
ambulatory BP monitoring (ABPM)
107
mainstay of therapy for children with asymptomatic mild hypertension without evidence of target-organ damage
Lifestyle modification
108
primary therapy in obesity-related hypertension
weight loss
109
recommended diet for adolescents and adults
```
DASH diet
decrease sodium intake
increase potassium, calcium and magnesium containing foods
6-8 servings of whole grains/ day
4-5 servings of fruits/day
4-5 servings of vegetables/day
low fat dairy food
```
110
risk factors for UTI
```
female gender
uncircumcised male
vesicoureteral reflux
toilet training
voiding dysfunction
obstructive uropathy
urethral instrumentation
sources of external irritation
constipation
anatomic abnormality
neuropathic bladder
sexual activity
pregnancy
```
111
diagnosis of UTI in culture results
*shows >50,000cfu/ml
*single pathogen (suprapubic or catheter sample) +
UA has pyuria or bacteriuira in a symptomatic child =UTI
*in bag sample, if UA (+), patient symptomatic --> catheter sample should be obtained --> CS
112
treatment for acute cystitis
mild or diagnosis doubtful: tx can be delayed
if tx indicated: 3-5 day course of TMP-SMX (6-12 TMP/kg/day in 2 divided doses)
Nitrofurantoin (5-7mg/kg/24hr) effective in Kleb and Enterobacter
Amoxicillin (50mg/kg/24hr) in 2 divided doses
113
treatment for pyelonephritis
Acute Febrile UTI (7-14 days)
hospitalized: Ceftriaxone 50mg/kg/24hr or Cefepime or Cefotaxime
* Nitrofurantoin should not be used routinely in children -with febrile UTI- no significant tissue levels
* repeat urine CS not routinely needed after termination of treatment
114
"bottom up" approach
renal sonogram plus VCUG
115
"top down" approach
DMSA scan --> if (+) --> VCUG
116
risk factors for recurrent UTI
age
vesicoureteral reflux
bowel bladder dysfunction
117
Mainstay therapy in all forms of RTA
Bicarbonate replacement
118
Hypokalemic hypochloremic metabolic alkalosis with hypercalciuria and salt wasting
Bartter syndrome
119
Optimal therapy for children with ESRD
Kidney transplantation
120
Most common complication of nephrotic syndrome
Infection
121
In patients with isolate microscopic hematuria who are asymptomatic, what should be done?
Obtain atleast 3 urinalysis at 2 week period then if hematuria persists, it should be evaluated
Annual creatinine with BP monitoring
122
most common cause of gross hematuria
bacterial UTI
123
most common chronic glomerular disease in children
IgA nephropathy
124
most common cause of persistent proteinuria in school age and adolescents
Orthostatic (postural) proteinuria
125
most common presenting symptom of children with nephrotic syndrome
Edema
126
most common form of glomerulonephritis
Postinfectious GN
127
most common form of HUS
diarrhea associated HUS from E. coli
128
most common serious bacterial infection in infants <24 months with fever without an obvious focus
Pyelonephritis
129
most common characteristic of urinary tract obstruction
hydronephrosis
130
most common cause of severe obstructive uropathy
posterior urethral valves
131
most common cause of daytime incontinence
overactive bladder
132
sign of urethral prolapse
bloody spotting on underwear or diaper
133
most common tumor developing in an undescended testis in an adolescent or adult
Seminoma
134
most common foreign object causing vaginal bleeding in prepubertal girls
toilet paper
135
most common obstructive anomaly of the vagina
imperforate hymen
136
most common cause of vaginal agesis
Mayer-Rokitansky-Kuster-Hauser syndrome
137
most common Mullerian anomaly
uterine septum
138
bilateral palpable flank masses in an infant with pulmonary hypoplasia, oligohydramnios, and hypertension and the absence of renal cysts by sonography of the parents
ARKPD
