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Flashcards in Specific Diseases Deck (25):
1

Alport syndrome aka familial nephritis

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

2

What are the most commonly palpated masses in infants?

Hydronephrotic kidneys multicystic dysplastic kidneys

3

Multicystic dysplastic kidney disease (MCDKD aka MCDK)

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

4

Autosomal recessive polycystic kidney disease

Bilateral flank masses

Oligohydramnios

Congenital hepatic fibrosis
-->chronic portal hypertension

5

Autosomal dominant polycystic kidney disease

Adults
Need a renal ultrasound
Intracranial aneurysms

6

Ectopic urethral opening in females

Female always wetting her pants despite negative work up

7

Unstable bladder

Incontinence during the day, OK at night.

Leg crossing
squatting

treatment
-timed urination
-anti-cholinergic agents

8

UTI

>50,000 colonies

E. coli
Enterococcus
Klebsiella

Adenovirus

UCx makes the Dx

9

When our boys most at risk for UTI?

0-3 months

Esp. uncircumcised

10

UTI treatment

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

IV
Ceftriaxone
Cefotaxime
gentamicin
tobramycin
Piperacilin

11

UTI prophylaxis

Amoxicillin
trimethoprim-sulfamethoxazole
nitrofurantoin

12

Nephrotic syndrome

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

13

Nephrotic syndrome epidemiology

Usually primary, Minimal change disease

Can be secondary:
Infection
Drugs
Malignancies
Lupus
Diabetes

Males (2:1)
2-8 yo

14

Nephrotic syndrome treatment

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

15

Nephrotic syndrome prognosis

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

16

Nephritic syndrome

Due to a variety of glomerular disease

ROPE

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

17

Causes of glomerulonephritis


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

18

FSGS

Focal segmental glomerular sclerosis

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

19

MPGN

Membranoproliferative Glomerulonephritis

Low C3

Need aggressive treatment to prevent renal failure

20

Post-strep glomerulonephritis

PSGN

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

21

PSGN Treatment

Supportive care
fluid restriction
blood pressure control

Gross hematuria is NOT recurrent

22

IgA nephropathy

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

23

PSGN reasons to biopsy

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

24

Hemolytic uremic syndrome

Clinical scenario

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

25

Hemolytic uremic syndrome
Treatment

Supportive

Avoid antibiotics, they make it worse.