Muwakkit Samar A, Saab Raya, Sanjad Sami A, Bhar Saleh I, Ishak Rim S, Samad Zeina A, Chan Anthony K, Abboud Miguel R
Division of Hematology/Oncology, American University of Beirut Medical Center, Beirut, Lebanon.
Blood Coagul Fibrinolysis. 2009 Sep;20(6):458-60. doi: 10.1097/MBC.0b013e32832ca3d8.
Renal venous thrombosis (RVT) is a rare but a well recognized entity in children and neonates. The clinical signs of neonatal RVT include hypertension, enlarged kidney(s), hematuria, renal insufficiency, proteinuria, thrombocytopenia, or all. Persisting impairment of kidney function and hypertension are serious and common complications in patients with RVT. Risk factors for the development of RVT include maternal diabetes mellitus, pathologic states associated with thrombosis (e.g., shock, dehydration, perinatal asphyxia, polycythemia), and sepsis. Inherited prothrombotic abnormalities have been described in some reports of RVT. We report the case of a male newborn with left RVT and associated homozygosity for both factor V Leiden (G1691A) and methylenetetrahydrofolate reductase C677T mutations in addition to elevated serum lipoprotein (a). The patient was treated with heparin. We believe our case to be the first reported case in the English medical literature of such an association between neonatal RVT and homozygosity for both factor V Leiden and methylenetetrahydrofolate reductase. This case and other studies clearly demonstrate that neonatal RVT should be evaluated for thrombophilia conditions.
肾静脉血栓形成(RVT)在儿童和新生儿中虽罕见但已被充分认识。新生儿RVT的临床体征包括高血压、肾脏肿大、血尿、肾功能不全、蛋白尿、血小板减少症,或上述症状全部出现。肾功能持续受损和高血压是RVT患者严重且常见的并发症。RVT发生的危险因素包括母亲患有糖尿病、与血栓形成相关的病理状态(如休克、脱水、围产期窒息、红细胞增多症)以及败血症。在一些RVT报告中描述了遗传性血栓前状态异常。我们报告了一例男性新生儿,患有左肾RVT,除血清脂蛋白(a)升高外,还存在因子V莱顿(G1691A)和亚甲基四氢叶酸还原酶C677T突变的纯合性。该患者接受了肝素治疗。我们认为我们的病例是英文医学文献中首次报道的新生儿RVT与因子V莱顿和亚甲基四氢叶酸还原酶纯合性之间存在这种关联的病例。该病例及其他研究清楚地表明,对于新生儿RVT应评估其血栓形成倾向情况。