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Women and Alport syndrome
Fri, Jan 13, 2012
Uncategorized
The percentage of Alport syndrome inherited in an X-linked manner is:
a. 100%
b. 80%
c. 50%
d. 20%
Heterozygous XLAS females can present with:
a. Normal urinalysis
b. Microscopic hematuria
c. Proteinuria
d. Sensorineural hearing loss
e. All of the above
The following has been shown to influence disease outcome in XLAS heterozygous females
a. X inactivation
b. Ethnicity
c. ACE inhibition
d. Pregnancy
e. Genotype
Which of the following are risk factors for renal disease progression in XLAS heterozygous females?
a. Microscopic hematuria
b. Proteinuria
c. Use of hormone replacement therapy
d. Hearing loss
e. B and D
f. B and C
X inactivation patterns in this tissue can predict X inactivation patterns in the kidney
a. Blood lymphocytes
b. Skin
c. Urinary epithelium
d. None of the above
Heterozygous XLAS females should be considered as kidney donors if they meet all of the following criteria except:
a. Presence of microscopic hematuria
b. Presence of proteinuria
c. Absence of sensorineural hearing loss
d. Absence of hypertension
Hepcidin in anemia of chronic kidney disease: review for the pediatric nephrologist
Fri, Jan 13, 2012
Uncategorized
Elevated serum ferritin levels can be observed in the setting of:
a. Total body iron overload
b. Acute inflammation
c. Reticuloendothelial blockade
d. All of the above
Hepcidin gene transcription is down-regulated by:
a. Inflammatory conditions
b. Enteral iron ingestion
c. Tissue hypoxia
d. Intravenous iron administration
Ferroportin channels are found in the cell membranes of which cell types?
a. Macrophages and enterocytes
b. Macrophages and RBC
c. Enterocytes and RBC
d. Lymphocytes and macrophages
Probable mechanisms for increased hepcidin levels in CKD include:
a. Decreased renal clearance
b. Induction by inflammatory cytokines
c. Chronic iron therapy
d. All of the above
Serum hepcidin levels have been shown to be modified by:
a. Nonsteroidal anti-inflammatory drugs
b. Dialysis
c. Hemapheresis
d. ACE inhibitors
CKD-MBD after kidney transplantation
Fri, Jan 13, 2012
Uncategorized
In post-transplantation renal osteodystrophy
a. PTH levels correlate closely with bone turnover
b. bone turnover returns to the normal range in all patients
c. defects in skeletal mineralization are common
d. adynamic bone disease is rare
In pediatric renal transplant recipients, bone density
a. is best assessed using DXA with age-matched controls
b. is best assessed using DXA with height-matched controls
c. is best assessed using DXA with gender-matched controls
d. cannot reliably be assessed by DXA
Post renal transplantation, cardiovascular disease
a. resolves within the first 2 years
b. improves (in adults) with the use of lipid-lowering agents
c. is unrelated to traditional risk factors, such as hypertension
d. is minimal in children
The leading cause of death in children post renal transplantation is
a. cardiovascular disease
b. cancer
c. infection
d. trauma
After renal transplantation growth
a. improves equally in all age groups, from infancy to adolescence
b. improves with steroid-avoidant and steroid-minimizing immunosuppressant protocols
c. does not respond to growth hormone
d. is no longer a clinical problem
Claudins in renal physiology and disease
Fri, Jan 13, 2012
Uncategorized
Which of the following best describes paracellular permeability and transport in the late proximal tubule?
a. The proximal tubule has a high transepithelial resistance and acts as a barrier to small ions
b. NaCl is reabsorbed paracellularly, driven by the Cl concentration gradient
c. Organic anions are secreted across the paracellular pathway
d. Water is predominantly reabsorbed via the paracellular pathway, driven by the osmotic gradient
e. The proximal tubule is selectively permeable to bicarbonate which is reabsorbed paracellularly
Which domain of the claudin protein forms the lining of the paracellular pore?
a. Amino terminal
b. Carboxy terminal
c. 1st transmembrane domain
d. 1st extracellular domain
e. Cytosolic loop between 2nd and 3rd transmembrane domain
It has been shown that D65 (aspartate residue at position 65) in claudin-2 is a cation-binding site that is responsible for its cation-selectivity. Which of the following mutation of claudin-2 would be most likely to turn claudin-2 from a cation-selective pore to an anion-selective pore?
a. D65K (mutation to lysine)
b. D65C (mutation to cysteine)
c. D65A (mutation to alanine)
d. D65S (mutation to serine)
e. D65L (mutation to leucine)
All of the following are clinical features of familial hypomagnesemic hypercalciuria with nephrocalcinosis (FHHNC), EXCEPT:
a. Increased fractional excretion of Mg
b. Rickets
c. Decreased GFR
d. Ocular abnormalities
e. Deafness
Which of the following treatments ameliorate the urinary Mg and Ca wasting in FHHNC?
a. Thiazide diuretics
b. Magnesium supplementation
c. Amiloride
d. Oral phosphate binders
e. Kidney transplantation
Which of the following claudins are selectively expressed in the neonatal kidney but not in adult kidney?
a. Claudin-2
b. Claudin-7
c. Claudin-9
d. Claudin-16
e. Claudin-19
Managing urinary tract infections
Fri, Jan 13, 2012
Uncategorized
Which one of the following statements regarding UTI diagnosis is true?
a. Clean-catch urine sample can be used in all children
b. Uretheral catheterization or suprapubic aspiration should be performed in infants and newborn
c. Uretheral catheterization has a lower likelihood of contamination compared with suprapubic aspiration
d. Periuretheral adhesive bag can be used in boys but not girls because of contamination risk
e. Any bacterial growth from a catheterized urine sample is considered significant
Of the following, which one is the most specific test for UTI by dipstick?
a. LE alone
b. Nitrite alone
c. Protein and blood
d. LE and nitrite
e. Protein and LE
Which one of the following characterizes asymptomatic bacteriuria?
a. Pyuria is present
b. Most commonly caused by Pseudomonas Aeruginosa
c. Antibiotic treatment is indicated
d. Can be a normal finding in children with neurogenic bladder
e. Increased risk of scarring if antibiotic prophylaxis is not used
Which of the following tests is the current gold standard for diagnosing renal scarring?
a. Ultrasound examination
b. Intravenous pyelography
c. DMSA renal scan
d. CT scan
e. Radionuclear cystography (RNC)
Microalbuminuria: causes and implications
Fri, Jan 13, 2012
Uncategorized
In estimating microalbuminuria, the following statment is true:
a. early morning spot urine for albumin creatinine ratio is the best
b. 24-h urine collection should always be done when possible
c. urine protein creatinine ratio is as reliable as the urine albumin creatinine ratio
d. the standard urine dip stick in the clinic is equally reliable
The estimates of prevalence of microalbuminuria in children are:
a. higher compared to young adults
b. higher in girls and children from an ethnic background
c. are likely confounded by the effects of orthostatic proteinuria
d. all of the above
Postural or orthostatic proteinuria
a. is not a benign condition and indicates progressive kidney disease
b. more common in obese girls
c. mostly associated with ‘nutcracker syndrome’
d. should be managed with ACE inhibitors
Generalized endothelial dysfunction is
a. rarely seen in children
b. usually precedes the onset of microalbuminuria
c. is reversed in early adult life
d. is exclusively seen in diabetic children
The glomerular endothelial glycocalyx layer
a. is a dynamic layer that is produced by glomerular endothelial cells
b. is vulnerable to damage in systemic diseases
c. is an important contributor to resistance to protein permeability
d. all of the above
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Fri, Jan 13, 2012
Uncategorized