Nephrol Dial Transplant (1997) 12: 1064–1066
Nephrology
Dialysis
Transplantation
Teaching point
( Section Editor: K. Ku ¨ hn)
A child with haemolytic uraemic syndrome: do we have to care about
aetiological heterogeneity?
G. Filler, M. A. von Bredow, H.-J. Gro ¨ne1 and J. H. H. Ehrich
Paediatric Nephrology, Charite ´ Children’s Hospital, Humboldt University, Berlin and 1Department of Pathology, University
of Marburg, Germany
sion, administration of thrombocytes, fresh frozen
Introduction
plasma, and prednisolone. Her condition did not
improve during the following 3 weeks and she was
The 10-year-old girl was admitted to the paediatric
then transferred to our unit.
unit of a general hospital with a 1-day history of
On admission the patient presented with a history
vomiting, vertigo, jaundice, petechiae, and haemat-
of adverse aects after vaccination and the clinical
omas. Her family history was inconspicuous. At the
picture of atypical haemolytic uraemic syndrome
age of 6 years she had experienced a Scho ¨nlein-Henoch
(HUS ). Bacteriology and serology for Escherichia coli
purpura without renal involvement. Tonsillectomy was
O1575H7 were negative. A kidney biopsy was
performed at the same age because of recurrent bac-
performed.
terial tonsillitis.
Seven weeks prior to her admission, she had received
Histopathology of the renal biopsy a first vaccination against early summer meningo-
encephalitis by her paediatrician. This was followed by
facial oedema two days later. She was seen by another On light microscopy (Fig. 1a–b) glomeruli showed
doctor who treated her with rectal prednisolone. Nine
segmentally accentuated increase of endocapillary and
days thereafter, she was given a second course of
mesangial cells. Fibrin-like material was seen in the
vaccine which was followed by an acute deterioration
vascular pole of the glomeruli. Aerent arterioles
of her condition. On examination, she was weak, pale
exhibited endothelial necrosis and thrombotic material
and jaundiced, with multiple facial and palate petechiae
inside the lumen. The interstitum showed a focal round
as well as several haematomas on her arms and body.
cell infiltration.
Her blood pressure was 120/70 mmHg. She had no
By immunohistology, fibrin was positive in glomer-
other abnormal physical findings, especially no joint
ular capillaries and arterioles.
aection, no hepatosplenomegaly, and no
Electron microscopy showed dense deposits in the
lymphadenopathy.
mesangium and in an intramembranous and subepi-
Her laboratory data showed a marked haemolytic
thelial location. Granulocytes and monocytes were
anaemia (PCV 25%, fragmentocytes 16%, LDH
seen in glomerular capillaries. An increase of mesangial
66 mmol/s*l, bilirubin, 87 mmol/l, thrombocytes matrix was also noted (Fig. 2a–d).
44 Gpt/l ), renal failure (creatinine 204 mmol/l ), hypo-
A diagnosis of thrombotic microangiopathy ( light
complementaemia (C3 0.45 g/l ), macrohaematuria and
microscopy) and endocapillary immune complex glom-
non-selective glomerular proteinuria (3 g/l).
erulonephritis (electron microscopy) was made.
Antistreptolysine titer and auto-antibody tests were
negative. A bone marrow aspiration revealed no signs
Course of the disease
of leukaemia.
On ultrasonography, her kidneys were both enlarged
Thrombocytopenia reversed to normal after plasma-
and had an inhomogeneous texture. A cerebral MRI
pheresis, but renal function deteriorated and the girl
was normal.
went into terminal renal failure. Continuous ambula-
The initial management consisted of a blood transfu-
tory peritoneal dialysis was started 2 months after first
admission. Presently the patient is on the waiting list
Correspondence and oprint requests to: J. H. H. Ehrich, Dept. of
for renal transplantation 1 year after onset of the
Paed Nephrology, Charite ´ Children’s Hospital, Humboldt University
Berlin, Schumannstr. 20/21, 10117 Berlin, Germany. disease.
© 1997 European Renal Association–European Dialysis and Transplant Association