AN ATYPICAL CAUSE OF ATYPICAL HAEMOLYTIC UREMIC SYNDROME : A CASE REPORT

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AN ATYPICAL CAUSE OF ATYPICAL HAEMOLYTIC UREMIC SYNDROME : A CASE REPORT
Anaghashree
Udayashankar
Topoti Mukherjee drtopoti@gmail.com Aster Whitefield Department of Nephrology Bengaluru
Sundar Sankaran ssundar99@gmail.com Aster Whitefield Department of Nephrology Bengaluru
Kristin George kristingeorge33@gmail.com Aster Whitefield Department of Nephrology Bengaluru
Raghuram CP drraghuramcp@asterhospital.com Aster Whitefield Department of Pediatric hematology Bengaluru
 
 
 
 
 
 
 
 
 
 
 

Haemolytic uremic syndrome (HUS) is a well-known cause of acute kidney injury in children. Shiga toxin producing Escherichia coli accounts for 90% of the cases in children. Haemolytic uremic syndrome(HUS) associated with adenovirus illness is a rare but serious complication and is most commonly seen in immunocompromised individuals.

 

Here, we present a case of HUS following adenovirus infection in a 9 year old girl who recovered with supportive care alongside vigilant conservative approach.

Ms. M, a 9-year old girl, presented with vomiting, jaundice and renal failure with reduced urine output.

 

She reported a history of fever and sore throat 10 days prior, when a nasal swab tested positive for adenovirus PCR. Her symptoms had abated but 10 days later, she had presented to our emergency department with nausea, vomiting, jaundice and decreased urine output.


 On arrival to our hospital, she was tachycardic, afebrile, normotensive with pallor, pedal oedema, icterus and a normal systemic examination. A review of the recent laboratory investigations revealed a declining trend of haemoglobin(10.9; 9.6 gm/dl) and platelets (431000/ul; 34000/uL) with unconjugated hyperbilirubinemia( TB: 3mg/dl, IB: 0.5 mg/dl) and mild renal dysfunction(serum creatinine: 1.7 mg/dl).

 

In our hospital, work up revealed a declining trend of haemoglobin 8.4 gm/dl with normal leukocyte count with thrombocytopenia 35000/uL. An unconjugated hyperbilirubinemia with normal liver enzymes with raised lactate dehydrogenase (1623/uL) and reduced haptoglobin level with schistocytes(5%) on peripheral smear pointed towards microangiopathic haemolytic anaemia. Urinalysis revealed microscopic haematuria without dysmorphic RBCs. Serum creatinine was 2 mg/dl with blood urea of 66mg/dl. A low serum complement (C3) level of 47.6 mg/dl (90-180 mg/dl) and normal sized kidneys with normal echoes on abdominal ultrasound was noted. A diagnosis of acute kidney injury secondary to Thrombotic Microangiopathy(TMA) related atypical Haemolytic uremic syndrome(HUS) was inferred. A recent history of Adenovirus infection was speculated to have triggered this episode of HUS. On Day 2,haemoglobin( 9 gm/dl) and platelets(76000/uL) spontaneously improved with plateauing LDH levels (1973 IU/L).

 

Though serum creatinine peaked at 3.3 mg/dl, she remained non-oliguric and clinically well. As there was no clinical worsening, and the haemoglobin and platelets plateaued by day 3, infection triggered aHUS was incriminated in this TMA, and plasmapheresis was deferred. We followed a conservative approach to renal failure with supportive measures with cautious monitoring of the clinical condition. By day 7,hematological,liver and renal profile had normalised. A genetic testing for complement factor(CF) H,CFB and anti CFH antibodies was recommended at discharge to identify underlying genetic predisposition.


Parameters/Day-->Day 0Day 1Day 2Day 3Day 4Day 5Day 22
Blood pressure (mm Hg)
Intake/Output (ml)
110/80

114/70
1000/600

116/60
1265/1075
108/60
1500/1300
113/57
2940/2310
116/63
2300/2500
110/80
2200/2800
Blood urea (mg/dl)
creatinine(mg/dl)
Potassium (mmol/L)
Sodium(mmol/L)
64.2
1.7
3.9
133
66.34
2
3.6
135
87.74
2.9
3.5
131
109.14
3.3
3.3
131
119.84
3.1-->2.4
2.8
126
102
0.9
3.3
131
36
0.9
4.7
136
Hemoglobin (g/dl)
White cell count(/uL)
Platelet count (/uL)
Reticulocyte count(%)
LDH (IU/L)
Schistocytes in blood(%)
8.4
9560
34000
1.8%
1623
2-3%
8.4
10040
35000
4.5%
--
3-5%
9
20700
76000
--
1973
3-5%
9.4
21940
116000
--
2302
3-5%
9
26800
150000
10%
2373
1-3%
8.2
24360
165000
6%
1080
Not seen
10.7
10440
183000
<1%
365
Not seen
LFT :TB/UB (mg/dl)
AST/ALT(IU/L)
ALP/GGT (IU/L)
Albumin (g/dl) 
3.3/3
86/26
104/16
3.6
2.9/2.7
---
---
---
3.4/3.2
---
---
---
1.51/1.31
24/9
73.9/--
3.6
0.65/0/36
---
---
---
Complement C3 (mg/dl)
Complement C4 (mg/dl) 
47.6
30.1
88
--
MiscellaneousUrine routine: Protein negative
RBC 8-10 WBC 2-3 
Urine, Blood cultures: sterile
PT: 13.5 sec
INR: 1.04
APTT: 28.4 sec
CRP: 3 mg/dl
D dimer: >15000
Dengue panel,
Malarial parasite : negative,
Direct Combs test : negative 
Ferritin : 784.8 ng/ml 
Procalcitonin: 0.3 ng/ml

TABLE 1: LABORATORY INVESTIGATIONS FROM ONSET TILL RECOVERY 

The speculated mechanism of adenovirus endothelial damage is the alteration of expression of endothelial intracellular components which promotes natural killer cell mediated cytotoxicity. Unlike, TMA related to primary complement defects, that necessitates urgent initiation of plasmapheresis or Eculizumab therapy and which is associated with guarded prognosis; our case highlights the importance of early recognition of TMA related to adenovirus infection and resorting to a vigilant conservative approach with supportive care to aid in clinical recovery.


This abstract was submitted for poster presentation at Women in nephrology conference, India in August 2023. I also declare that re-submitting the abstract is permitted by the organizers of the original meeting(s).

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