Resontoc Lourdes Paula R, Yap Hui-Kim
Shaw-NKF-NUH Children's Kidney Center, KTP-National University Children's Medical Institute, National University Health System, Singapore, Singapore.
Department of Pediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Tower Block 12 #1E, Kent Ridge Road, Singapore, 119228, Singapore.
Pediatr Nephrol. 2016 Jun;31(6):907-15. doi: 10.1007/s00467-015-3160-0. Epub 2015 Jul 15.
Neonatal renal vascular thrombosis is rare but has devastating sequelae. The renal vein is more commonly affected than the renal artery. Most neonates with renal vein thrombosis present with at least one of the three cardinal signs, namely, abdominal mass, macroscopic hematuria and thrombocytopenia, while unilateral renal artery thrombosis presents with transient hypertension. Contrast angiography is the gold standard for diagnosis but because of exposure to radiation and contrast agents, Doppler ultrasound scan is widely used instead. Baseline laboratory tests for platelet count, prothrombin time, activated partial thromboplastin time and fibrinogen concentration are essential before therapy is initiated. Maternal blood is tested for lupus anticoagulant and anticardiolipin antibody. Evaluation for prothrombotic disorders is warranted when thrombosis is clinically significant, recurrent or spontaneous. Management should involve a multidisciplinary team that includes neonatologists, radiologists, pediatric hematologists and nephrologists. In addition to supportive therapy, recent guidelines recommend at least prophylactic heparin therapy in the majority of cases to prevent thrombus extension. Thrombolytic therapy is reserved for bilateral thrombosis compromising kidney function. Long-term sequelae, such as kidney atrophy, systemic hypertension and chronic kidney disease, are common, and follow-up by pediatric nephrologists is recommended for monitoring of kidney function, early detection and management of hypertension and chronic kidney disease.
新生儿肾血管血栓形成虽罕见,但会导致严重后果。肾静脉比肾动脉更常受累。大多数肾静脉血栓形成的新生儿会出现至少三种主要体征中的一种,即腹部肿块、肉眼血尿和血小板减少,而单侧肾动脉血栓形成则表现为短暂性高血压。血管造影是诊断的金标准,但由于存在辐射暴露和使用造影剂的问题,多普勒超声扫描被广泛应用。在开始治疗前,进行血小板计数、凝血酶原时间、活化部分凝血活酶时间和纤维蛋白原浓度等基线实验室检查至关重要。检测母体血液中的狼疮抗凝物和抗心磷脂抗体。当血栓形成具有临床意义、复发或自发时,有必要对血栓前状态疾病进行评估。管理应涉及一个多学科团队,包括新生儿科医生、放射科医生、儿科血液科医生和肾病科医生。除了支持治疗外,最近的指南建议在大多数情况下至少进行预防性肝素治疗以防止血栓扩展。溶栓治疗仅用于双侧血栓形成且影响肾功能的情况。长期后果,如肾萎缩、系统性高血压和慢性肾脏病很常见,建议由儿科肾病科医生进行随访以监测肾功能、早期发现和管理高血压及慢性肾脏病。