IPNA Online

Women and Alport syndrome

linda — Fri, 13 Jan 2012 17:23:49 +0000

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

linda — Fri, 13 Jan 2012 17:07:26 +0000

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

linda — Fri, 13 Jan 2012 16:51:00 +0000

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

linda — Fri, 13 Jan 2012 16:40:09 +0000

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

linda — Fri, 13 Jan 2012 16:18:48 +0000

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

linda — Fri, 13 Jan 2012 16:00:44 +0000

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

Saudi International Conference of Nephrology & Transplantation

rdelacruz — Wed, 04 Jan 2012 19:24:54 +0000

February 19, 2012

February 22, 2012

Location: King Fahd Cultural Center, Riyadh-Kingdom of Saudi Arabia

For more information: http://www.ssnconference.org

Bartter- and Gitelman-like syndromes: salt-losing tubulopathies with loop or DCT defects

linda — Thu, 10 Nov 2011 20:46:01 +0000

The pregnancy of a 28-year-old woman with one previous miscarriage is complicated by idiopathic polyhydramnios, which was first recognized by routine ultrasound at the end of the second trimester. After therapeutic amniocentesis (estimated amniotic fluid volume of 10 l) and rupture of membranes at 30 weeks of gestation, an acute Caesarean section was performed. The delivered male infant was appropriate for gestational age and showed an uneventful postnatal adaptation, except for a mild respiratory distress syndrome requiring a positive end-expiratory pressure device. However, a few days later, he developed hyponatremia, hyperkalemia, hyposthenuria, hypercalciuria, and a weight loss from birthweight by >15%. The diagnosis most likely is:

a. Nephrogenic diabetes insipidus
b. Loop disorder with NKCC2 defect (BS type 1)
c. Loop disorder with ROMK defect (BS type II)
d. Combined loop and DCT disorder with barttin defect (BS type IV)
e. Combined loop and DCT disorder with a defect in ClC-Ka and ClC-Kb

What is the most appropriate pharmacotherapeutic intervention in a patient with polyuric and hypercalciuric salt-losing tubulopathy associated with hyperaldosteronism?

a. Potassium-sparing diuretics
b. Calcium-sparing diuretics
c. COX-2 inhibitors
d. Prostaglandin synthesis inhibitors
e. ACE inhibitors and/or AR blockers

Which patient with a salt-losing tubulopathy is most likely at risk to develop end-stage renal failure later in life?

a. Combined loop and DCT disorder with barttin defect (BS type IV)
b. Loop disorder with ROMK defect (BS type II)
c. DCT disorder with ClC-Kb defect (BS type III)
d. DCT disorder with NCCT defect (GS)
e. Loop disorder with NKCC2 defect (BS type I)

Which special subtype of salt-losing tubulopathy is least likely to be associated with chronic hypercalciuria and nephrocalcinosis?

a. NKCC2 defect (BS type I)
b. ClC-Kb defect (BS type III)
c. ROMK defect (BS type II)
d. Barttin defect (BS type IV)

What is the most convenient way to differentiate between renal and extrarenal salt losses?

a. Plasma electrolyte measurement
b. Urine osmolality
c. Urinary sodium and/or chloride levels
d. Sweat chloride test

What is the most unlikely complication or sequelae of a DCT disorder with an apical uptake defect (GS)?

a. Hypokalemic rhabdomyolysis
b. Nephrolithiasis
c. Growth retardation
d. Cardiac arrhythmias
e. Chondrocalcinosis

Rhabdomyolysis: pathogenesis of renal injury and management

linda — Thu, 10 Nov 2011 20:04:54 +0000

QUIZZIN 131]

Renal amyloidosis in children

linda — Thu, 10 Nov 2011 19:43:22 +0000

The main cause of childhood amyloidosis is

a. Juvenile idiopathic arthritis
b. Beta2 microglobulin amyloidosis
c. AFib amyloidosis
d. Familial Mediterranean fever
e. Familial cold autoinflammatory disease

Which of the answers below is wrong regarding autoinflammatory diseases causing secondary amyloidosis in children?

a. The mutations in the MEFV gene causing FMF are associated with excess Il-6 production
b. Colchicine is the mainstay treatment for the prevention of amyloidosis in FMF
c. CAPS is associated with mutations in cryopyrin
d. Hematuria may be seen in FMF
e. Secondary amyloidosis may occur in TRAPS

Which of the following is NOT associated with secondary amyloidosis in FMF?

a. Male gender
b. Family history
c. SAA1.α/α genotype
d. Environment
e. E148Q homozygosity

Which of the answers below is WRONG about the treatment of amyloidosis?

a. Colchicine is not effective in other causes of secondary amyloidosis
b. Eprodisate weakens the structural stability of amyloid fibrils
c. Hemodialysis and peritoneal dialysis appear to be equally effective
d. Colchicine should be continued in dialysis patients having FMF
e. Renal transplantation is the most effective treatment in Aβ2M amyloidosis

What is the MOST common initial manifestation of renal disease secondary to amyloidosis?

a. Hypertension
b. Asymptomatic hematuria
c. Asymptomatic proteinuria
d. Renal tubular acidosis
e. Chronic renal failure