Renal-Fahim Flashcards

1
Q

You are asked by an obstetrician to evaluate the results of intrauterine ultrasonography of a 35-year-old healthy female who is in the 20th week of her fourth pregnancy. Her husband is 41 years old and healthy, as are her three children. Ultrasonography reveals a probable male fetus that has severe bilateral hydronephrosis and oligohydramnios. Of the following, the MOST likely cause of the intrauterine hydronephrosis is:
A. autosomal dominant polycystic kidney disease
B. autosomal recessive polycystic kidney disease
C. medullary cystic disease
D. posterior urethral valves
E. prune belly syndrome

A

D. posterior urethral valves

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2
Q
A 2 y/o boy is brought to ER w/ complaints of fever, chills, and flank pain. His immunizations are UTD, and his mother states that it is his second time to have these symptoms. His tempterature is 39.1 (102.2) and PE is unremarkable  except for CVA tenderness on the right. A CBC shows leukocytosis and UA shows the presence of WBC and RBC in the urine. What is the most likely mechanism of this patient’s recurrent complaints?
A. immunoglobulin deficiency
B. Nephroblastoma
C. Poststreptococcal glomerulonephritis
D. Vesicoureteral reflux
A

D. Vesicoureteral reflux

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3
Q
A 2 month old infant is found to have a horseshoe kidney. Which structure prevents this abnormal kidney from occupying its appropriate position?
A. Aorta
B. Celiac Trunk
C. Inferior Mesenteric artery
D. Inferior vena cava
E. Superior mesenteric artery
A

C. Inferior Mesenteric artery

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4
Q

What is the most common cause of UTI’s?

A

E. coli

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5
Q

What should be considered in any child

A

UTI

UA obtained via catheter

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6
Q

How should you treat patients 2 months to 2 years old that have a UTI and are toxic appearing, dehydrated or unable to retain oral intake?

A

hospitalize and IV antibiotics

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7
Q

Who should be imaged for a UTI/

A
  • All children younger than 5 years of age who have a febrile UTI
  • GIRLS younger than 3 years of age and boys younger than 1 year of age experiencing a first UTI
  • Children who have recurrent UTIs,
  • Patients who do not respond promptly to appropriate antimicrobial therapy.
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8
Q

What is the most common site of urinary tract obstruction in children?

A

Uretero-Pelvic Junction Obstruction (UPJ)

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9
Q

Which collecting system problem can present with not peeing straight or severe hydronephrosis at a young age?

A

Posterior urethral valve

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10
Q

Nephrotic syndrome is characterized by severe protineuria, decreased serum albumin, and edema. This results from damage to one or more components of the glomerular capillary wall. In particular, the glomerular basemement membrane is essential for maintaining serum oncotic pressure. In nonpathological states, which of the following properties of the GBM prevent albumin from being freely filtered into the urine?

A. a combination of small pore size and negatively charged pore-forming molecules prevents albumin filtration
B. a combination of small pore size and positively charged pore-forming molecules prevents albumin filtration.
C. Albumin is freely filtered across the basement membrane but is readily reabsorbed along the nephron.
D. The positive charge of proteoglycans in the basement membrane repels albumin
E. The small size of the glomerular basement membrane pores excludes albumin molecules.

A

A. a combination of small pore size and negatively charged pore-forming molecules prevents albumin filtration

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11
Q

What does >3.5 g/day of protein in the urine or > 50 mg/kg/24 hours indicate?

A

Nephrotic symdrome

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12
Q

Which nephrotic syndrome presents with a uniform thickening of the glomerular capillary wall without significant mesangial proliferation (spike and dome appearance)?

A

Membranous glomerulopathy

“Be a member in the spike and dome club”

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13
Q

What is the nephrotic syndrome associated with fused foot processes? What is the prognosis?

A

Minimal change disease

good prognosis (only protein in urine in 2-6 yo)

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14
Q

What is Burger’s Disease?

A

IgA deposits in the mesangium (“A Messy Burger)

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15
Q

What nephrotic disorder has sub epithelial immune complex deposits describes as lumpy bumpy?

A

Post-strep GN

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16
Q

Which nephropathy presents with a uniform thickening of the glomerular capillary wall without significant mesangial proliferation (spike and dome appearance)?

A

Membranous glomerulopathy

(“Be a member in the spike and dome club”)

associated with SLE and drugs and can be with infection

17
Q

Which nephropathy associated with effacement of the foot processes (fused foot processes)? What is the prognosis?

A

Minimal change disease

good prognosis (only protein in urine in 2-6 yo)

18
Q

What is Berger’s Disease? How long after a URI is this seen?

A
  • IgA nephropathy
  • IgA deposits in the mesangium (“A Messy Burger)
  • RIGHT AFTER a URI
19
Q

Which nephropathy has sub epithelial immune complex deposits describes as lumpy bumpy? How long after an infection will this occur?

A

Post-strep GN

weeks after an infection

coca-cola urine

20
Q

Which nephropathies have low serum complement?

A

PMS
Post-strep GN
Membranoproliferative GN
Systemic Lupus

21
Q
A 16 year old boy comes to the physician w/ a 1 year hx of intermittent, painless hematuria without dysuria or increased frequency  of micturition. He says he has also had several respiratory infections and adds that the hematuria increased w/in several days of the infections. Which of the following is most likely to be found if the boy is diagnosed with IgA nephropathy (Berger’s disease)?
A. increased antistreptolysin O titer
B. Lumpy-bumpy electron dense deposits
C. Mesangial deposits
D. Protienuria exceeding 3.5gm/24hr
E. Subepithelial deposits
A

C. Mesangial deposits

22
Q

A 5-year-old previously healthy girl is brought to the emergency department because of a 2-day history of fever and occasional vomiting. Her temperature is 102.5° (38.8°C), heart rate is 130 beats/min, respiratory rate is 40 breaths/min, and blood pressure is 96/60 mm Hg. She is well-appearing, and her physical examination findings are normal. A complete blood count shows a white blood cell count of 7 x 10³/mcL (7 x 109/L) with a normal differential count. Her urinalysis shows a specific gravity of 1.020 and 1+ protein, but otherwise completely negative findings. Of the following, the MOST appropriate management of this girl’s proteinuria is to:
A. obtain a 24-hour urine protein collection
B. order a spot urine protein/creatinine ratio
C. perform renal ultrasonography
D. refer her to a nephrologist for follow-up
E. repeat the urinalysis after her illness has resolved

A

E. repeat the urinalysis after her illness has resolved

23
Q

If you have organomegaly with a cherry red spot, what should you think of? What if you do NOT have organomegaly?

A

Organomegaly=Niemann Pick

No organomegaly=Tay Sachs

24
Q

If you have organomegaly with a cherry red spot, what should you think of? What if you do NOT have organomegaly? What is deficient in each of these?

A

Organomegaly=Niemann Pick (deficiency in sphingomyelinase)

No organomegaly=Tay Sachs (deficient in hexoseaminidase A–> accumulation of GM 2)

25
Q

What is Alport syndrome?

A

Familial form of nephritis with neurosensory hearing loss and slow progression to renal failure

26
Q

What is Berger’s Disease? How long after a URI is this seen?

A
  • IgA nephropathy
  • IgA deposits in the mesangium (“A Messy Burger)
  • RIGHT AFTER a URI (1-2 days)
27
Q

Which nephropathy has sub epithelial immune complex deposits describes as lumpy bumpy? How long after an infection will this occur?

A

Post-strep GN

2 weeks after an acute infection

coca-cola urine

28
Q
A 13 y/o boy is brought to the ER w/ periorbital edema, HTN, and tea-colored urine. His parents say that he had a sore throat about 3 weeks ago. UA shows RBC w/casts. A Positive ASO titer, and decreased levels of complement are also noted. What findings would be expected in the patient’s glomeruli?
A. Granular subendothelial deposits
B. Linear Subendothelial pattern
C. Mesangial deposits
D. Subendothelial deposits
E. Subepithelial humps
A

E. Subepithelial humps

29
Q

What is Alport syndrome?

A

Familial form of nephritis with neurosensory hearing loss and slow progression to renal failure

  • type IV collagen problem
  • eyes and ears affected
30
Q

A 5 year old boy comes to the ER department with fever, malaise, nausea, decreased urine output (despite normal fluid intake), and smoky brown urine that began 10 days after he had recovered from a sore throat. Which of the following are the most likely pathologic findings on biopsy of the kidney?
A. Enlarged hypercellular glomeruli on light microscopy; dense humps found on the epithelial side of the glomerular basement membrane on electron microscopy.
B. linear deposition of antibodies against glomerular basement membrane found on immunofluroescence
C. Mesangial proliferation on light microscopy; mesangial deposition of IgA on immunofluorescence
D. Normal-appearing glomeruli on light microscopy; effacement of the foot processes of the visceral epithelial cells on electron microscopy
E. Widespread and pronounced thickening and splitting of the glomerular basement membrane on electron microscopy

A

A. Enlarged hypercellular glomeruli on light microscopy; dense humps found on the epithelial side of the glomerular basement membrane on electron microscopy.

POST-STREP GN

(B=goodpastures, C=IgA nephropathy, D=minimal change disease, E=alports)

31
Q

A 5 year old boy is brought to the ER with a 2 day history of abdominal pain, vomiting, and a rash. His mother reports that he had a runny nose and mild cough about a week ago. On exam ther is diffuse abdomninal pain and a rash over the buttocks and the legs. His CBC is WNL, and UA shows 12 RBCs/mm3 and 2 WBC/mm3, no protien, and no glucose. What is the most likely etiology of this patient’s symptoms?
A. deficiency of von Willebrand factor-cleaving metalloproteinases
B. IgA antibody deposition in the mesangium
C. IgA immune complexes deposited in small vessels
D. IgG antibodies against platelets
E. IgG antibodies deposited in the glomerular basement membrane

A

C. IgA immune complexes deposited in small vessels

HSP=MOST COMMON SMALL VESSEL VASCULITIS IN KIDS
purpura, abd pain and glomerulonephritis

32
Q

What is type 2 RTA associated with?

A

Fanconi Syndrome

33
Q

What are undescended testes associated with?

A

-infertility and an increased risk of cancer

cancer risk does not decrease with surgical procedure but infertility will improve

34
Q

What is a hypospadia due to?

A

failure of the urethral folds to close

35
Q

A neonate is evaluated after birth. Abdominal exam shows that the bladder is exposed and everted on the lower abdominal wall. Which of the following additional anomalies would also be most likely found in this patient?
A. autosomal recessive polycystic kidney disease
B. Epispadias
C. Hypospadias
D. narrowed pubic symphysis
E. imperforate anus
F. urachal cyst

A

B. Epispadias

36
Q

What PE finding will be found in a pt with testicular torsion? Is this a big deal?

A
  • absent Cremasteric reflex

* emergency*

37
Q

A baby boy dies 2 days after birth. He was born with wrinkled skin, deformed limbs, and abnormal facies. The mother’s pregnancy was complicated by oligohydramnios. Which of the following embryologic processes most likely failed in this child?
A. Development of the dermis
B. Development of the kidneys
C. Fusion of maxillary and medial nasal prominences
D. Migration of neural crest cells to the distal colon
E. Outgrowth of limb buds

A

B. Development of the kidneys