Specific Diseases

Question 1

Alport syndrome aka familial nephritis

Answer 1

X-linked dominant
Females are still usually asymptomatic carriers
Males more likely to develop end stage renal disease.

Bilateral sensorineural hearing loss
ocular defects
renal failure

Question 2

What are the most commonly palpated masses in infants?

Answer 2

Hydronephrotic kidneys multicystic dysplastic kidneys

Question 3

Multicystic dysplastic kidney disease (MCDKD aka MCDK)

Answer 3

Unilateral flank mass
Renal dysplasia 
Enlarged kidney with non-communicating cysts 
Thin/no parenchyma 
Kidney does not function, no treatment.
Oligohydramnios
minimal fluid in the bladder
50% of the time there are other urinary tract anomalies
-UPJ obstruction 
-VUR
-PUV
-megaureter and duplication

First need US to confirm Dx.
2nd VCUG to check for other issues

Question 4

Autosomal recessive polycystic kidney disease

Answer 4

Bilateral flank masses

Oligohydramnios

Congenital hepatic fibrosis
–>chronic portal hypertension

Question 5

Autosomal dominant polycystic kidney disease

Answer 5

Adults
Need a renal ultrasound
Intracranial aneurysms

Question 6

Ectopic urethral opening in females

Answer 6

Female always wetting her pants despite negative work up

Question 7

Unstable bladder

Answer 7

Incontinence during the day, OK at night.

Leg crossing
squatting

treatment

• timed urination
• anti-cholinergic agents

Question 8

UTI

Answer 8

> 50,000 colonies

E. coli
Enterococcus
Klebsiella

Adenovirus

UCx makes the Dx

Question 9

When our boys most at risk for UTI?

Answer 9

0-3 months

Esp. uncircumcised

Question 10

UTI treatment

Answer 10

7-14 days
Amoxicillin-clavulanate
Trimethoprim-sulfamethoxazole 
Cephalexin
Cefixime
Cefuroxime 

IV 
Ceftriaxone 
Cefotaxime
gentamicin 
tobramycin
Piperacilin

Question 11

UTI prophylaxis

Answer 11

Amoxicillin
trimethoprim-sulfamethoxazole
nitrofurantoin

Question 12

Nephrotic syndrome

Answer 12

Proteinuria
Hypoproteinemia
Edema

Decreased UOP
Abdominal pain
Diarrhea
Weight gain

Normal renal fxn!

Liver upset by low oncotic pressure
VLDL increases –> high LDL/HDL ratio
Fibrinogen, factor V and VII increase –> Hypercoagulability

Lose immunoglobulins and complement proteins (immunodeficiency)
Lose albumin –> decreases bound/available Ca–> hypocalcemia
Lose thyroxine binding globulin –> functional hypothyroidism

Complications:
Hyponatremia
vascular thrombosis
peritonitis

Question 13

Nephrotic syndrome epidemiology

Answer 13

Usually primary, Minimal change disease

Can be secondary:
Infection 
Drugs
Malignancies
Lupus
Diabetes

Males (2:1)
2-8 yo

Question 14

Nephrotic syndrome treatment

Answer 14

Sodium restriction
prednisone

Hospitalize if incapacitating edema/infection.

Fluid restrict if severe edema

If proteinuria persists after 4 wks of prednisone then need renal biopsy. +/- cyclophosphamide or cyclosporine

Usually 1-2 relapses per year, resolves in adolescence

Question 15

Nephrotic syndrome prognosis

Answer 15

#1: Worse if poor response to steroid tx 
>10 yo 
Persistent/gross hematuria 
HTN
Renal insufficiency 
Low C3 complement levels

Question 16

Nephritic syndrome

Answer 16

Due to a variety of glomerular disease

ROPE

Red urine (hematuria)
Oligouria
Proteinuria
Elevated BP and BUN (azotemia), edema

Question 17

Causes of glomerulonephritis

Answer 17

1. Normal complement levels (HIGH) complement
Henoch-Schonlein Purpura (IgA)
Idiopathic vasculitis
rapidly progressive GN
FSGS

2. Low complement levels
   PMS. Not in the mood to complement.

Post-strep
MPGN
Systemic lupus

Question 18

FSGS

Focal segmental glomerular sclerosis

Answer 18

Teenagers 
nephrotic syndrome 
usually progressive renal failure normal C3 levels
low serum albumin 
Edema

Question 19

MPGN

Membranoproliferative Glomerulonephritis

Answer 19

Low C3

Need aggressive treatment to prevent renal failure

Question 20

Post-strep glomerulonephritis

PSGN

Answer 20

1-3 wks post throat or skin GAS (“recently ill”)

Immune complexes deposited on kidneys

Low C3 for ~2 months, then returns to normal

HTN
Edema (eyelids/face noticed by parent)
Hematuria (tea/cola colored)

Usually doesn’t –> renal failure

Low serum albumin b/c of hemodilution, NOT Proteinuria

Question 21

PSGN Treatment

Answer 21

Supportive care
fluid restriction
blood pressure control

Gross hematuria is NOT recurrent

Question 22

IgA nephropathy

Answer 22

Aka Berger’s disease

High IgA
IgA deposits on renal biopsy

Recurrent gross hematuria, painless
Mild abdominal pain

With or a few days after a URI/pharyngitis

Usually >10 yo

Question 23

PSGN reasons to biopsy

Answer 23

Gross hematuria >8 wks
Low serum complement
HTN over 2 weeks
Proteinuria over 6 months
Abnl renal fxn

Question 24

Hemolytic uremic syndrome

Clinical scenario

Answer 24

HUTS

Hemolytic anemia
Uremic (elevated BUN, renal failure)
Thrombocytopenia
Syndrome

E. Coli
-poorly cooked meat, unpasteurized apple cider, cow and goat milk
Diarrhea
Hematuria
Abdominal pain
Decreased UOP
Purpura, ecchymoses 
CNS issues
HTN

Normal complement levels
Coombs test negative

Question 25

Hemolytic uremic syndrome

Treatment

Answer 25

Supportive

Avoid antibiotics, they make it worse.