A 40-year-old woman presents 7 days after a pan-colectomy for Crohn’s disease. She has a past history of antithrombin III deficiency. She has increasing abdominal pain and vomiting. There is marked tenderness in the right upper quadrant. An abdominal CT scan is performed.
a) CT Scan:
Portal venous thrombosis
c) Causes of anti-thrombin III deficiency:
o Post-operative state
o Liver disease
o Disseminated intravascular coagulation
o Nephrotic syndrome
The CT scan I used was mined shamelessly from Google Images. It is not the one which appeared in the paper.
The first two parts of this question are straightforward.
The CT features of portal vein thrombosis are as follows:
The second half of the question requires detailed knowledge of AT-III deficiency. One can arrive at at least half of a sensible answer by logically asking why AT-III might not be present in sufficient quantities. Either you hereditarily fail to synthesise enough of it, or your liver is so damaged that it cannot produce enough. Or, it has been used up somehow, eg. in the context of DIC, MAHA, or in a bypass circuit. Lastly, it is possible that you are losing it along with other proteins via your leaky nephrotic kidneys.
The chapter which discusses thrombophilia screening tests contains within it this table, which lists the causes of AT-III deficiency with greater granularity.
Inherited AT-III defects
Loss of protein
The management of AT-III deficiency is, predictably, supplementation with AT-III. If the expensive purified factor is not available, FFP will suffice. Heparinisation for thrombosis actually will not work unless there is some antithrombin for the heparin to act upon.
Management options for PVT include the following:
UpToDate offers a good article about Antithrombin III deficiency, for a price.
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Lee, Hae-Kyung, et al. "Portal vein thrombosis: CT features." Abdominal imaging 33.1 (2008): 72-79.
Denninger, Marie‐Hélène, et al. "Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors." Hepatology31.3 (2000): 587-591.\
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Basit, Syed Abdul, Christian D. Stone, and Robert Gish. "Portal vein thrombosis." Clinics in liver disease 19.1 (2015): 199-221.