ESPN 51th Annual Meeting

ESPN 2018


 
Atypical Hemolytic Uremic Syndrome with Severe Extrarenal Manifestations: a Case Report
CELALETTIN KOŞAN 2 NURAN KÜÇÜK 1

1- KARTAL DR.LÜTFI KIRDAR TRAINING AND RESEARCH HOSPITAL
2- ATATÜRK UNIVERSITY DEPARTMENT OF PEDIATRIC NEPHROLOGY
 
Introduction:

 Atypical  hemolytic  uremic  syndrome  (aHUS)  is  a  rare  and  life-threatening disease that may lead to end-stage renal failure (ESRF) or death, and may be accompanied by  a  variety  of  extrarenal  manifestations. We presented  a  child with  aHUS  accompanied  by severe  extrarenal  manifestations.

Material and methods:

 A  12-year-old girl  was  visited  in  the  emergency  departments  with  acute  renal  failure, symptoms  of  fluid  overload,  vomiting,  and  somnolence.  Laboratory  tests revealed  microangiopathic  hemolytic  anemia,  thrombocytopenia,  and  acute renal  failure  with  severe  electrolyte  imbalance.    Diagnosis  of  HUS was  made and  emergency  hemodialysis  was  performed  to  decrease  the  circulating volume, restore the electrolyte disturbance, and support the treatment of HUS, but conventional medical therapies were ineffective. The patient experienced frequent  seizures  and  multiple  cardiac  arrests  and  became  comatose. Thereafter,  although  she  was  diagnosed  with  aHUS,  plasma  infusions  and plasmapheresis  were  performed.  Upper  gastrointestinal  endoscopy  and colonoscopy  revealed  erosive  pangastritis,  widespread  gastric  hemorrhagic ulcers, bulbitis, and hemorrhagic colitis. Since there was no improvement, the patient was transferred to a central university hospital where eculizumab was started.  She  responded  to eculizumab (Table.1). Her renal functons are still in normal range and has 1-1,5 liter uine per day.She is continuing to take eculizumab every 14 days. She has not relapsed and genetic tests have not been comlpeted yet.

 

Results:

 

Table.1 The laboratory results of the case throughout the course of the disease and the treatment type

Days

BUN

mg/dl

Cre

mg/dL

Hb

g/dL

PLT

x10³/µL

 

Haptoglobulin

(0,3-2 g/L)

LDH

(1-248 U/L)

C3 (g/L)

(0,9-1,8)

Reticulocyte

%

Treatment

1.

126

9,2

7,2

63

0,24

1773

0,36

20

HD

3.

78,5

7,8

8,7

92

0,24

1161

0,49

12

HD+Pİ

14.

14,1

2,2

7,5

79

0,24

858

0,79

5

HD+Pİ

28.

44,3

5

10,2

182

0,24

603

0,67

9

HD+Pİ

40.

25,8

3,3

7,5

229

0,24

614

0,98

8

1.PF + HD

50.

20,1

2,2

11,2

168

0,24

371

0,68

4

5.PF + HD

91.*

57

6,6

8,1

63

0,24

623

0,32

7

HD+Pİ

98.

60,7

4,3

9,0

247

0,26

329

0,38

4

HD+Pİ

107.**

32,2

4,7

8,4

98

0,26

537

0,32

5

HD+Pİ

116.

41,5

5,9

8,1

128

0,26

572

0,33

4

HD+Pİ

180.

71,4

2,6

13,7

306

1,2

250

0,9

1

***

Eculizumab+HD

 

HD: Hemodialysis

Pİ: Plasma infusion

PF: Plasmapheresis

* ,** . The labaratory tests during diarrhea episodes

*** . The labaratory tests after 1 month eculizumab treatment

Conclusions:

 In  conclusion,  as  aHUS  can  progress rapidly  and  is  frequently  fatal  if  untreated,  it  is  important  to  be  aware  of unusual  presentations  and  diagnose  the  condition  promptly,  particularly  if supportive treatment is of little or no help.