Protein C Deficiency in a Patient with Anomalous Hemiazygous Vein and Portal Vein Thrombosis. 2021

F A Fasola, and A Akere, and C U Akunwata, and C Onyejelam, and M A Osundina
Department of Haematology, University College Hospital/College of Medicine, University of Ibadan, Ibadan, Oyo State,Nigeria.

Protein C deficiency increases the risk of an individual to develop thromboembolism and its complications. Clinical presentation of the complication of thrombosis in an unusual site may becloud clinical judgment resulting in missed diagnosis. We present an unusual case of protein C deficiency presenting with symptoms referable to the gastrointestinal system. A 34-year-old male with recurrent abdominal discomfort and bloating, managed as a case of gastro-oesophageal reflux disease with poor clinical outcome. Physical examination was unremarkable. Upper gastrointestinal endoscopy showed varices. Abdominal ultrasound scan and CT scan of the abdomen revealed thrombus in the portal vein. Functional assays of protein C and S revealed reduce protein C activity at 65 % (70 - 140%). This case emphasizes the need for extensive investigations in patients with common, sometimes neglected abdominal symptom such as bloating. It has also contributed in expanding the differential diagnosis of bloating and manifestations of protein C deficiency.

UI MeSH Term Description Entries
D008107 Liver Diseases Pathological processes of the LIVER. Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008297 Male Males
D011169 Portal Vein A short thick vein formed by union of the superior mesenteric vein and the splenic vein. Portal Veins,Vein, Portal,Veins, Portal
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013927 Thrombosis Formation and development of a thrombus or blood clot in BLOOD VESSELS. Atherothrombosis,Thrombus,Blood Clot,Blood Clots,Thromboses
D020151 Protein C Deficiency An absence or deficiency in PROTEIN C which leads to impaired regulation of blood coagulation. It is associated with an increased risk of severe or premature thrombosis. (Stedman's Med. Dict., 26th ed.) Deficiency, Protein C,Hereditary Thrombophilia Due To Protein C Deficiency,Deficiencies, Protein C,Protein C Deficiencies
D020246 Venous Thrombosis The formation or presence of a blood clot (THROMBUS) within a vein. Deep Vein Thrombosis,Phlebothrombosis,Thrombosis, Deep Vein,Thrombosis, Venous,Deep Venous Thrombosis,Deep-Vein Thrombosis,Deep-Venous Thrombosis,Deep Vein Thromboses,Deep Venous Thromboses,Deep-Vein Thromboses,Deep-Venous Thromboses,Phlebothromboses,Thromboses, Deep Vein,Thromboses, Deep Venous,Thromboses, Deep-Vein,Thromboses, Deep-Venous,Thromboses, Venous,Thrombosis, Deep Venous,Thrombosis, Deep-Vein,Thrombosis, Deep-Venous,Vein Thromboses, Deep,Vein Thrombosis, Deep,Venous Thromboses,Venous Thromboses, Deep,Venous Thrombosis, Deep

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