Abstract
ABSTRACTSitus inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.
Highlights
Situs inversus totalis (SIT) is a rare autosomal recessive congenital abnormality in which all mediastinal and abdominal organs are in a mirrored position relative to their normal topography
This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura
Idiopathic thrombocytopenia purpura (ITP) is an autoimmune blood disorder characterized by thrombocytopenia due to the destruction of platelets or suppression of their production, by means of an immune reaction against autoantigens on the membranes of the platelets.[5]. The clinical picture may present as critical situations, with cutaneous and mucosal bleeding and even voluminous hemorrhage, which make the quick diagnosis and therapeutic intervention mandatory.[6]. Its incidence is estimated at 1.6 to 2.7 cases per 100 thousand individuals/year, and prevalence at 9.5 to 23.6 cases per 100 thousand individuals, with predominance of the female sex.[7]
Summary
Situs inversus totalis (SIT) is a rare autosomal recessive congenital abnormality in which all mediastinal and abdominal organs are in a mirrored position relative to their normal topography. It is believed that a defect in the long arm (q) of chromosome 14 is responsible for this organ transposition. As a condition, it is compatible with life, and can be asymptomatic.[1] The incidence is estimated at 1/8,000 to 1/25,000 in liveborns.[2] Abnormalities in SIT can be recognized first, by using radiography or ultrasonography, and computed tomography is the preferred test for the definitive diagnosis of SIT.[3] There are reports describing einstein (São Paulo).
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