A CASE OF HEMOLYTIC UREMIC SYNDROME AND ITS MANAGEMENT

1. CASE REPORT ON
HEMOLYTIC UREMIC
SYNDROME
Dr. M SAITEJ REDDY MD
GANDHI MEDICAL COLLEGE
SECUNDERABAD

2. 16yrs old girl, Kavya resident of Nalgonda,
student came with complaints of
 Yellowish discoloration of eyes since 5 days
 Swelling of both lower limbs since 3 days

3.  Patient was apparently asymptomatic
10days back then she developed fever,
which was sudden in onset, low grade,
intermittent, subsided after 4days with
medication not associated with chills and
rigors, not associated with sweating.

4.  vomitings 10days back, insidious in onset, non
projectile,non-bilious,6-7episodes/day for
3days,contains food particles
 associated with anorexia and nausea
 No h/o hematemesis

5.  H/o loose stools 10days back, insidious in
onset,4-5episodes/day lasted for
3days,watery,large in volume
 H/o blood mixed in stools on 3rd day,2episodes
not associated with foul smell or mucous
 H/o abdominal pain, cramping type, diffuse
pain predominantly around the umbilicus,non-
radiating.
 Pain transiently subsided after passing stools
and vomiting

6.  Patient was taken to local hospital and
admitted for 1day,treated with IV fluids and
antibiotics,was discharged after 1day with oral
antibiotics(ciprofloxacin) and probiotics

7.  H/o yellowish discolouration of eyes and urine
since 5days,insidious in onset and gradually
progressing
 No h/o clay coloured stools and itching
 H/o easy fatiguability and generalised
weakness since 5days

8.  H/o swelling in both lowerlimbs since
3days,gradually progressing, associated with
facial puffiness and decreased urine output
 H/o reddish discolouration of urine since 2days
 H/o shortness of breath since 2days.
 No h/o orthopnea and PND
 Not associated with cough and chest tightness

9.  No h/o abdominal distension
 No h/o significant weight loss
 No h/o joint pains,alopecia,oral ulcers,skin
rash
 No h/o abdominal mass

10. PAST HISTORY
 No h/o similar complaints in the past
 No h/o bronchial asthma,tuberculosis,malaria
 No h/o previous blood transfusions since
childhood
 No h/o hypertension,diabetes

11.  FAMILY HISTORY:
 No h/o similar complaints in the family
 DRUG HISTORY
 No h/o drug intake

12.  PERSONAL HISTORY:
 Takes mixed diet
 Appetite-decreased
 Sleep-adequate
 Bowel regular
 Bladder – decreased urine output

13. SUMMARY
16yrs old girl presented with
 h/o fever, vomitings, blood mixed stools 10
days back lasted for 3 days
 Jaundice since 5 days
 Easy fatiguability and generalised weakness
 Pedal edema, decreased urine output and
SOB since 3 days

14. Differential Diagnosis
Intestinal infections
 Shigellosis
 Enterohemorrhagic E.coli
 Camplobacter jejuni
 Entamoeba histolytica amoebiasis
Acute viral hepatitis
Leptospirosis

15.  GENERAL EXAMINATION:
 Patient is
conscious,coherent,cooperative,comfortably
sitting on bed
 Moderately nourished and moderately built
 Height-150cms
 Weight-50kgs
 BMI-22.2

16.  Pallor +
 Icterus +
 Facial puffiness +
 Bilateral Pedal edema +,pitting type upto the
level of knees
 No koilonychia/clubbing/generalised
lymphadenopathy
 No signs of liver cell failure
 No stigmata of tuberculosis

17.  VITALS:
 Temperature-normal
 PR-84/min,normal volume,regular
 RR-20/min,thoraco-abdominal
 BP-110/80 mm Hg,measured in right
upperlimb in supine position
 JVP-normal

18.  GIT EXAMINATION:
 Oral cavity-normal
 On inspection-
 Shape of the abdomen is scaphoid
 Umbilicus is in midline,inverted
 All quadrants moving equally with respiration
 No scars,sinuses,visible pulsations,peristalsis
 Hernial orifices-free

19.  On palpation-
 No local rise of temperature and tenderness
 No organomegaly
 No palpable masses
 On percussion-
 Liver span-11cms
 Traubes space-resonant
 No free fluid

20.  Auscultation-
 Bowel sounds-heard
 No bruit

21.  RESPIRATORY SYSTEM:
 Normal vesicular breath sounds heard in all
areas
 No adventitious sounds
 CARDIOVASCULAR SYSTEM:
 S1,S2 heard
 No S3,S4
 No murmurs

22.  CENTRAL NERVOUS SYSTEM:
 Higher mental functions-normal
 Cranial nerves-normal
 Motor system-normal
 Sensory system-normal
 No cerebellar or meningeal signs

23. INVESTIGATIONS
 Complete hemogram:
Hb-6.5gm%
WBC-15,000
Platelets-50,000
Peripheral smear-
anisocytes,schistocytes(fragmented
RBC),microcytes++, macrocytes+,
Reticulocyte count-4%

24. The smear shows multiple helmet cells (arrows) and other
fragmented red cells (small arrowhead); microspherocytes are also
seen (large arrowheads). The platelet number is reduced; the large
platelet in the center (dashed arrow) suggests that the
thrombocytopenia is due to enhanced destruction.

25. Genesis of the schistocyte
This scanning electron
microphotograph shows a
red blood cell (white arrow)
about to be "guillotined" by a
fibrin strand (yellow arrow),
produced as a result of
intravascular coagulation.
This results in the formation
of a fragmented red blood
cell, termed a schistocyte or
"helmet cell."

26.  Total serum bilirubin-7.3 ↑
 Direct bilirubin-1.9
 SGOT-39
 SGPT-21
 Serum LDH-425IU/lit ↑
 sodium-136
 Potassium-3.8
 Chloride-105

27.  CUE-
Pus cells-8-10/hpf
RBC-25-30/hpf
Albumin-+++
 Serum creatinine-6.1
 Blood urea-158

28.  HIV and HBsAg-non-reactive
 ESR-98mm in 1st hour
 PT-15 Secs
INR-1.07
 aPTT-44 secs
 Coomb’s test-negative
 ANA screening-negative

29.  Stool for C/S-E.coli positive
 USG abdomen-normal study
 FDP and d-dimer-normal
 Complement levels
C3-Low,C4-normal

30. SUMMARY
16 yrs old girl presented with
 Fever, vomitings, blood mixed diarrhea
followed by jaundice and decreased urine
output over 10days.
 On examination showing pallor, icterus, pedal
edema and facial puffiness
 Hemogram showing schistocytes,
thrombocytopenia and leukocytosis
 TSB 7.3, s. LDH elevated, s. creatinine – 6.1

31. DIAGNOSIS
 HEMOLYTIC UREMIC SYNDROME

33. However, results from stool cultures may be unreliable because the
bacteria are only present in the stools for a few days and, even if
present, may not be detected by culture from stool samples.
DIAGNOSIS — The diagnosis of STEC-HUS is generally made on
clinical grounds based on the characteristic clinical and laboratory
findings previously described: a prodrome of diarrhea due to a STEC-
producing bacteria, followed by abrupt onset of the characteristic triad
of microangiopathic hemolytic anemia, thrombocytopenia, and acute
kidney injury.

34.  Hemolytic uremic syndrome (HUS) is defined
by the simultaneous occurrence of
microangiopathic hemolytic anemia,
thrombocytopenia, and acute kidney injury.
 In the past, HUS had been divided into
diarrhea-positive and diarrhea-negative HUS.

35. Present classification is based on underlying
cause:
 Primary causes without coexisting disease
(also referred to as atypical HUS); such as
cases due to complement dysregulation
1. Complement gene mutations
2. Antibodies to complement factor H
3. Inborn errors of cobalamin C metabolism
4. Diacylglycerol kinase epislon (DGKE) gene
mutations

36.  Secondary causes:
1. Infection:
 Shiga toxin-producing Escherichia coli (STEC)
 Streptococcus pneumoniae
 HIV
2. Drug toxicity, particularly in patients with
cancer or solid organ transplant recipients
3. Rarely in pregnancy or those with
autoimmune disorders (eg, systemic lupus
erythematous)

37.  Shiga toxin-producing E. coli (STEC)
hemolytic uremic syndrome (HUS) accounts
for 90 percent of cases of HUS in children.
 It is generally preceded by a prodromal illness
with abdominal pain, vomiting, and diarrhea,
which is usually bloody

40. Different strains of EHEC have been associated
with both sporadic and epidemic STEC-HUS
cases throughout the world.
 United States and Europe, STEC-HUS E. coli
0157:H7
 May 2011, E. coli O104:H4, was identified as
the cause of STEC-HUS in a large outbreak in
Germany
 Australia - E. coli 0111
 Shigella dysenteriae type 1-associated HUS
occurs in India, Bangladesh, and southern
Africa

42.  In HUS, the microangiopathic hemolytic anemia
is caused by nonimmune red blood cell (RBC)
destruction due to shearing of the RBCs through
platelet microthrombi.
 Despite thrombocytopenia, there is usually no
purpura or active bleeding. The degree of
thrombocytopenia is unrelated to the severity of
renal dysfunction.
 The severity of renal involvement ranges from
hematuria and proteinuria to severe AKI and
oligoanuria. Severe AKI occurs in one-half of
cases

43. Pathology in renal involvement
 glomerular thrombotic microangiopathy,
characterized by a thickening of the capillary
walls.
 The lesions affect the preglomerular arterioles and
the glomerular capillaries.
 Cortical necrosis may be patchy or rarely, diffuse
and affect the entire superficial cortex.
 These lesions are observed in the more severe
cases with prolonged anuria, and they carry a
high risk of chronic renal failure.

44.  Other organ involvement in EHEC
 Central nervous system – Manifestations
include seizures, coma, stroke, hemiparesis,
and cortical blindness. CNS abnormalities are
typically seen in up to 20 percent of cases due
to E. coli 0157:H7
 Gastrointestinal tract – Any area from the
esophagus to the perianal area can be
involved. The more serious manifestations
include severe hemorrhagic colitis, bowel
necrosis and perforation, rectal prolapse,
peritonitis, and intussusception

45. DIFFERENTIAL DIAGNOSIS
 Severe abdominal pain, frankly bloody
diarrhea, fever, and leukocytosis occur in other
enteric infections such as Salmonella,
Campylobacter, , amebiasis

48.  HUS vs TTP:
 Affected children usually present at birth with
hemolytic anemia and thrombocytopenia.
Renal involvement often occurs later in life and
has a progressive course.
 TTP is distinguished from HUS by abnormally
low ADAMTS13 activity.

49. HUS vs DIC:
Presence of abnormal coagulation studies,
including prolonged PT and aPTT, and
elevated levels of fibrin degradation products
and D-dimer.

50. STEC-HUS vs complement mediated HUS:
 Complement-mediated HUS –generally have a
severe clinical course, recurrent disease, and
often a positive family history.
 Genetic testing or identifying antibodies to
complement components confirms the
diagnosis of complement-mediated HUS.

51. MANAGMENT
 PC transfusions for anemia when clinically
indicated
 Platelet transfusion for patients who have
significant clinical bleeding.
 Appropriate fluid and electrolyte management to
maintain adequate intravascular volume
and correct/avoid electrolyte abnormalities.
 Avoiding nephrotoxic drugs
 Initiation of dialysis therapy in patients with
symptomatic uremia, azotemia,severe fluid
overload, or electrolyte abnormality that is
refractory to medical therapy.
 Provision of adequate nutrition.

55. THANK YOU