International Biological and Biomedical Journal
IBBJ
Medical Sciences
http://ibbj.org
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admin
2423-4478
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2423
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jalali
1399
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gregorian
2020
10
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online
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en
A case of chronic Budd-Chiari syndrome in 24 years old man complicating secondary antiphospholipid syndrome in association with autoimmune hepatitis
Other
Other
Case Report
Case Report
<div style="text-align: justify;">In Budd-chiari syndrome (BCS), obstruction of the hepatic venous outflow tract occurs at a site from the small hepatic veins to the junction of the inferior vena cava with the right atrium, and this syndrome can have various causes. Remarkably, BCS can be classified as primary or secondary; primary BCS is caused by venous thrombus or phlebitis, while secondary BCS is due to venous compression or invasion of an extrinsic lesion, such as a tumor, abscess, or cyst. Antiphospholipid syndrome (APS) is an acquired cause of primary BCS. A 24-year-old man, with complaints of abdominal pain, abdominand anorexia starting three months earlier, was admitted to Rasht Razi Hospital. The patient's pain did not relate to feeding, defecation and positioning. The patient had nausea and vomiting half an hour after feeding. He also mentioned a weight loss of about 5 kg and also,had blood pressure. He had no history of smoking, alcohol and opiums and had not any specific illness in his family. Due to abdominal distension and hepatosplenomegaly, an ultrasound examination was performed for the patient, ascites were diagnosed and the patient was subjected to ascites fluid paracentesis. High gradient of albumin and low protein were reported. The patient was first examined for cardiac examination and heart echocardiography and other examinations were reported as normal. A CT scan of the abdomen, pelvic and doppler ultrasonography was performed that showed ascites and enlargement of the caudate lobe. The findings were compatible with BCS. The diagnosis of BCS was confirmed by a hemodynamic study, and further investigation for the cause of BCS was positive. Antinuclear antibodies, deficiency of protein C and S were detected. LA was not present and aCL (IgM, IgG) were positive. Moreover, serology was negative for hepatitis B or C and serum protein electrophoresis was demonstrated high levels of gamma globulin. We concluded that the patient had chronic BCS secondary to APS in association with autoimmune hepatitis. Ultimately, the patient was treated with prednisolone 60 mg/daily and warfarin, and was referred to a transplantation-equipped center for liver transplantation. In conclusion, BCS, although infrequent, is not a rare complication in patients with APS and may be the first clinical manifestation of this syndrome. In this patient, BCS caused by the initiation of AIH and secondary APS.</div>
Budd-Chiari syndrome (BCS), antiphospholipid antibodies (aPL), Antiphospholipid syndrome (APS), Autoimmune hepatitis (AIH)
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http://ibbj.org/browse.php?a_code=A-10-91-3&slc_lang=en&sid=1
Fariborz
Mansour-Ghanaei
ghanaie@yahoo.com
10031947532846004065
10031947532846004065
No
GI Cancer Screening and Prevention Research Center, Guilan University of Medical Sciences, Rasht, Iran
Iman
Soufi Afshar
imanafshar@gmail.com
10031947532846004066
10031947532846004066
No
Gastrointestinal and Liver Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran
Afshin
Shafaghi
drafshinshafaghi@yahoo.com
10031947532846004067
10031947532846004067
Yes
Gastrointestinal and Liver Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran
Farahnaz
Joukar
fjouv@yahoo.com
10031947532846004068
10031947532846004068
No
GI Cancer Screening and Prevention Research Center, Guilan University of Medical Sciences, Rasht, Iran
Masoud
Baghaei
m.baghaei@gmail.com
10031947532846004069
10031947532846004069
No
Gastrointestinal and Liver Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran
Saba
Fakhrieh Asl
s.fakhrieh@yahoo.com
10031947532846004070
10031947532846004070
No
Gastrointestinal and Liver Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran
Mahsa
Mohtasham
mahsamohtasham@gmail.com
10031947532846004071
10031947532846004071
No
Gastrointestinal and Liver Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran
Ali Akbar
Samadani
a_a_hormoz@yahoo.com
10031947532846004072
10031947532846004072
No
Healthy Ageing Research Center, Neyshabur University of Medical Sciences, Neyshabur, Iran