Case presentation

Hanan Fathy
Pediatric Nephrology Department
Alexandria university
2007
Male patient,11years old, from Elbehyra.

C/o:
Generalized oedema
Abdominal pain
Fever
Two days before admission
The patient is a known case of nephrotic
syndrome .
Age of presentation: 4 years.
Steroid pattern : steroid dependent.
Number of relapses : 9 relapses since
diagnosis.
Present admission:
The patient presented to the E.R with generalized
pitting edema and puffiness.

The mother also reported the presence of high

grade fever, present all over the day

The patient also suffered from generalized

abdominal pain and tenderness, not referred , with
mild abdominal distension, no change in bowel
habbits.
General look:
The patient looks ill, toxic, taking flexion
attitude.
Vitals :
Temp:39 HR:110 b/min
RR :35 cycle/min Blp:120/80
H&N: flushed face, puffy eye lids.
Chest : clinically free.
Ht : clinically free.
Abdomen : distended, mild tenderness and
rebound tenderness, moderate ascites by
shifting dullness, no scrotal oedema.
Extremities : bilat pitting LL oedema.
BUN:40 mg/dl S.cr:0.8mg/dl
S.alb:1.5g/dl S.cholesterol:456mg/dl
24 hr urine : 5 gm protiens /800 cc urine.
Liver function tests were normal.
CBC :
Hb :11g/dl.
Platelets :650,000/ccm.
WBCs :12,000/ccm (70%neutrophils)
Abdominal ultrasonography: revealed the
presence of moderate ascites, and no other
abnormality detected.
 To sum up
 A case of steroid dependent nephrotic syndrome,
admitted in relapse.

 Patient is suffering from fever and severe

agonizing abdominal pain.

 Investigation showing mild prerenal impairment,

hypoalbuminemia, hypercholesterolemia,
leucocytosis and thrombocytosis.
What happened next

On account of presentation and results of

investigations the case was diagnosed as being a
case of:
Nephrotic syndrome in relapse, complicated with
spontaneous bacterial peritonitis.

The patient was given treatment in the form of

I.V antibiotics, I.V fluids and stress dose
coricosteroids.
 What happened next

Two days after admission and initiation of

treatment:
The patient did not improve, abdominal
pain worsened.

The mother reported the onset of melena.

DIFFERANTIAL DIAGNOSIS

Sepsis and DIC.

Stress ulcer causing GIT bleeding.
Mesenteric vascular occlusion as a
complication of hypercoagoulable state of
nephrotic syndrome.
Sepsis and DIC

 Infection continues to be a problem in patients

with nephrotic syndrome, with 70% of the deaths
associated with nephrotic syndrome caused by
infections, and 50% of these being peritonitis.

 Increased susceptibility to infections is related to

the proteinuria of nephrotic syndrome and
possibly is due to the immunosuppressive
medications used to treat it
Sepsis and DIC

 Another mechanism for increased susceptibility is

the hypogammaglobulinemia in nephrotic
syndrome caused by IgG being lost in the urine.

 In addition, patients with nephrotic syndrome

have diminished opsonization of bacteria as a
result of decreased serum levels of factors B (C3
proactivator) and D necessary for the alternative
complement pathway
Nephrotic Syndrome: An Acquired
Hypercoagulable State

 The mechanisms and factors involved in the

development of thromboembolic complications
in nephrotic syndrome can be attributed to the
interplay of two components:

Platelet hyperaggregability
Hypercoagulability
Nephrotic Syndrome: An Acquired
Hypercoagulable State
 Low ATIII: increased clearance .(Antithrombin III
levels are lowest when the serum albumin level is less
than 2.0 g/dL).

 Low functional protein S: increased clearance of

free protein S.

 Procoagulant serum proteins, including factors I,

II, V, VII, VIII, X, and XIII, are increased as a
result of increased hepatic synthesis

 Increased lipoprotein
How does the case progressed

Abdominal U/S was repeated.

 A doppler U/S examination of the

splanchnic area was requested.

 As well as C.T angiogram for splanchnic

area was done.
C.T angiogram

 CT image shows gas in portal

venous system (blue circle);
center image shows absence of
contrast
in superior mesenteric vein
due to thrombosis of this
vessel (blue arrow) ; lower
image shows extensive
pneumatosis intestinalis (red
arrows)
How does the case progressed
 The ultrasonographic examination revealed the
presence of an area of altered echogenicity in
the spleen, moderate ascites and turbid fluid.

 Doppler U/S revealed the presence of total

mesenteric venous occlusion (total occlusion of
portal, spleenic, and sup.mesenteric veins ) with
a large area of spleenic infarction.

 C.T angio revealed the same results, with

evidence of venous congestion of the intestine .
WHAT DID WE DO

Surgical deprtement was consulted:

Nothing surgical could be done the case is of

poor prognosis.

A bleeding profile was done preliminarily ,

Low molecular weight heparin was initiated
promptly,
The patient was maintained on clexan for 3
weeks then shifted to warfarrin.
Follow up by doppler U/S was done/12 hrs
WHAT DID WE DO

 The patient started to show progressive

improvement.
 After 3 weeks the veins started to show
recanalization.
 Induction of remission for relapse was initiated.

 The patient was discharged after 45 days from

admission in remission and with recanalization of
occluded vessels.
Mesenteric venous thrombosis

Accounts for 5-10% of intestinal ischemia.

Causes :
Prothrombotic disorders (antithrombin III
protien C, protien S deficiencies)
Hypercoagulable states asssocited with
Polycythemia vera,
Myeloproliferative disorders,
Nephrotic syndrome,
Neoplasms.
Acute Mesenteric Venous Thrombosis

Presents as abdominal pain that, early on ,

is typically out of proportion to physical
findings.

Nausea and vomiting are common, and

lower GIT bleeding or haematemsis
indicating bowel infarction occurs in 15%
of cases.
Acute Mesenteric Venous Thrombosis

 In patients in whom mesenteric venous occlusion

is made a trial of anticoagulation is worthwhile.

 Prompt laborotomy, resection of non viable

bowel, and anticoagulation with heparin.

 The mortality of mesenteric venous thrombosis

vary from 20-50%.
 Recurrence rates of 20-25 % fall to 13% to 15%
if heparin is begun promptly.
Thank you