Haemolytic Uraemic Syndrom

Amit Sinha, Rakesh Rai
Department of Surgery, Russells Hall Hospital. Dudley, United Kingdom
ABSTRACT
Context Haemolytic uraemic syndrome is a
common cause of renal failure in children but
it is a rare condition in adults. Acute
pancreatitis in adult as a cause of haemolytic
uraemic syndrome is very rare.
Case report A 19-year-old male presented
with symptom and signs suggestive of acute
pancreatitis which was confirmed as his
serum amylase was significantly raised.
Within three days of admission he developed
acute renal failure with evidence of
haemolytic anaemia and thrombocytopenia. A
clinical diagnosis of haemolytic uraemic
syndrome was made and he was treated with
plasma exchange. He made a complete
recovery.
Conclusion Renal failure in a patient with
acute pancreatitis is rarely due to haemolytic
uraemic syndrome. But it is important to
consider this differential diagnosis so that
early treatment can be instituted to prevent
mortality.
INTRODUCTION
Haemolytic uraemic syndrome (HUS) is
characterised by microangiopathic anaemia
with thrombocytopenia and acute renal
failure. HUS precipitated by E. Coli O157:H7
induced diarrhoea is one of the most common
cause of renal failure in infants and children.
But HUS is a rare disease in adult. Acute
pancreatitis as a result of HUS is rare and
affects about 2% of adults suffering from
HUS but HUS as a result of acute pancreatitis
has only been discussed in a few case reports
[1].
We describe a further case of acute
pancreatitis highlighting the importance of
early diagnosis and appropriate management.
CASE REPORT
A 19-year-old male was admitted to the
Surgical Unit through Emergency Unit. He
presented with one-day history of severe
upper abdominal pain, associated with
frequent vomiting. There was no history of
diarrhoea or fever. There was no past history
of any significant illness. On examination he
was found having epigastric tenderness. His
vital parameters were normal and blood test at
the time of admission revealed normal
haemoglobin, full blood count, urea,
electrolytes, and liver function tests. Serum
amylase was raised to 1,426 IU/L (reference
range: 25-125 IU/L) and a diagnosis of acute
pancreatitis was made. Ultrasound revealed
oedematous body and tail of pancreas, biliary
tree was not dilated and there was sludge in
the gallbladder.
In next two days his renal function
progressively deteriorated and on 3rd day of
his admission his urea and creatinine were
21.1 mmol/L (reference range: 0-7.5 mmol/L)
and 590 µmol/L (reference range: 0-120
µmol/L) with associated oligurea. He was
shifted to ICU. First of all cause of his renal
failure was thought to be pancreatitis

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associated hypovolemia but it was noticed
that his haemoglobin level had dropped to 8.8
g/dL from 14.9 g/dL at the time of admission.
His clotting profile was normal and there was
evidence of thrombocytopenia with platelet
count of 32 x109/L (reference range: 15-40
x109/L). His clotting profile was normal and a
normal fibrin degradation product (FDP) rule
out disseminated intravascular coagulation. In
view of his renal failure he was seen by the
nephrologists and a provisional diagnosis of
HUS was made. Further investigation
revealed evidence of intravascular haemolysis
with raised reticulocyte count of 60,000 mm-3
(reference range: 25,000-120,000 mm-3),
schistocytes in blood smear and haptoglobin
at 0.1 g/L (reference range: 0.2-2.4 g/L). The
Coombs’ test was negative. A renal biopsy
revealed thrombi in few capillary loops,
tubules showed acute tubular damage with
inflammatory infiltrate and interstitial
oedema. Amyloid was not seen and
immunohistochemistry for for IgA, IgG and
IgM was negative.
He required regular haemodialysis and fresh
frozen plasma transfusion. His renal function
progressively improved and he was
discharged from ICU to ordinary ward on day
30 and was discharged from hospital after 2
months of admission.
DISCUSSION
Acute renal failure in patients with acute
pancreatitis carries a poor prognosis and its
incidence varies with the severity of
pancreatitis with incidence up to 42% has
been reported [2]. In a retrospective study of
563 patients with acute pancreatitis the
incidence of renal failure was 14% [3]. The
renal failure in our case of acute pancreatitis
was part of HUS.
Aetiology of HUS in adult is more
heterogeneous compared to HUS in children
where 80% of the cases are associated with E.
Coli O157:H7 infection [4, 5]. The overall
prognosis of HUS in adult is poor compared
to children [6] and rate of chronic renal
failure after HUS ranged from 40 to 60% in
adult series [4, 7]. Thus it is important to
consider the diagnosis of HUS in patients
with renal failure in case of pancreatitis.
The mechanism of development of HUS
following pancreatitis is not clear. As there
are only 20 cases reported in the literature [1],
there has not been enough opportunity to
understand the aetiology. Several hypotheses
has been put forward including the role of
TNF-alpha and IL-1 which are important
mediators for pancreatitis and might induce
widespread vascular endothelial injury [8].
The other suggestion is modification of
circulating Von Willebrand molecules by
pancreatic proteases, which leads to platelet
aggregation [9]. Acquired or congenital
deficiency of Von Willebrand factor has been
shown to be the underlying cause of
thrombotic angiopathies. Recently serum
measurement of von Willebrand factor
cleaving protease (ADAMTS-13) has been
used to differentiate between thrombotic
thrombocytopenic purpura (TTP) and HUS.
As patients with TTP have little or no
ADAMTS-13 activity in plasma compared to
patients with HUS [10, 11]. It has also been
suggested that patients with ADAMTS-13
activity might have better prognosis than
patients without ADAMTS-13 [12].
The histological heterogeneity of haemolytic
uraemic syndrome has been already well
demonstrated [13, 14]. The typical lesion in
TTP shows widespread vascular thrombosis
in glomerular capillaries but in HUS the renal
lesion may only show glomerular, tubular or
vascular lesion [13]. As in our case there was
evidence of tubular inflammation without
wide spread thrombosis in vessels.
The treatment of HUS following acute
pancreatitis involves supportive measures
including haemodialysis if required as in our
case. It has been suggested that fresh frozen
plasma infusion improves the renal outcome
in adult HUS [15]. Several retrospective
studies in adults suggested that plasma
therapy may improve mortality and renal
recovery in patients with HUS [4, 16].

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JOP. Journal of the Pancreas – http://www.joplink.net – Vol. 6, No. 4 – July 2005. [ISSN 1590-8577]
367
CONCLUSION
It is important that clinicians treating acute
pancreatitis should be aware of HUS as a
possible complication of acute pancreatitis
and in any patient with acute pancreatitis
developing renal failure HUS must be ruled
out by blood tests. Early institution of plasma
therapy will improve prognosis and long term
renal function.
Received May 10th, 2005 - Accepted May
26th, 2005
Keywords Hemolytic-Uremic Syndrome;
Kidney Failure; Pancreatitis, Acute
Necrotizing
Abbreviations FBC: full blood count: fibrin
degradation product; HUS: haemolytic
uraemic syndrome; TTP: thrombotic
thrombocytopenic purpura
Correspondence
Rakesh Rai
4 St. Mark’s court
234 High street
Dudley, DY5 4JD
 
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