Case presentation

Hanan Fathy Pediatric Nephrology Department

Alexandria university2007

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/dlS.alb:1.5g/dl S.cholesterol:456mg/dl24 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