WSPN Stump the Experts Session OR

My patient keeps coming back. What do I do?

Jessica L. Stahl, MD

Pediatric Nephrology Fellow – 2nd year

University of Washington/Seattle Children’s Hospital

January 26th, 2019

Round 1: Presentation

13 year old previously healthy girl

6 months: intermittent lower back pain, abdominal pain,

decreased appetite, 10-15lbs weight loss, fatigue, pallor,

weakness

1 week: fever to 102oF, emesis, worsening fatigue and

abdominal pain

FHx: maternal eosinophilic esophagitis, sarcoidosis,

ulcerative colitis, fibromyalgia, primary sclerosis cholangitis

PE: Cachectic, distended abdomen. Otherwise normal.

Labs and imaging were obtained…

Round 1: Labs

141 108 20

3.9 22 1.6

6.2 251 6.9

baseline 0.6 5.5

2.1

8.9

ESR = 115

CRP = 25.8

LFTs – nl

Uric acid – nl

LDH – nl

PT = 15

aPTT = 40

INR = 1.2

Fibrinogen = 820 D-dimer = 5

C3 – nl

C4 – nl

ANA - nl

Urine

SpGr = 1.012

RBC >100/hpf

WBC = 11-20/hpf

Casts: rare mixed-cell,

waxy, granular

prot:cre = 1.59

Round 1: Imaging

• CXR – normal

• CT abdomen – portal venous HTN, splenomegaly,

ascites, pancreatic “fullness”

• US abdomen – the same. Patent renal vasculature,

increased echogenicity bilaterally.

80%

IgA

C3

IgG

What does she have?

What do you do?

In 2 minutes or less…

Round 1: Warm up 1 minute 15 seconds

Round 1: Fin

AntiGBM Ab = 55

Pheresis/Cyclophosphamide/Prednisone

She got better…

Round 2: Presentation

16 years old, last AntiGBM Ab= 0

2 months of weight loss, intermittent fevers, joint pains,

negative IBD evaluation

Interval Labs:

ESR = 70, CRP = 15.8

Cre 0.9

C3, C4 normal

Anti-GBM Ab negative

UA with ~10RBC/hpf, ~10WBC/hpf, rare granular casts

Uprot:cre = 0.54

Time (3 years) passes…

10%

IgG

C1q

IgA

Round 2: Labs redux

• AntiGBM Ab negative x 3

• ELISA x 2

• Western Blot x 1

• Indirect Ab staining negative

• Monkey

• Human

Round 3: Presentation

17years old

Time (1 year) passes…

70%

IgA IgG

C1q C3

What does she have?

Round 1

What do you do?

Special Thanks To:

• Jordan Symons

• Joseph Flynn

• Laura Finn