Niada François, Tabin René, Kayemba-Kay's Simon
Department of Paediatrics and Neonatal Medicine, Centre Hospitalier du Valais Romand, Sion, Switzerland; Service de Pédiatrie, CHU Yalgado Ouédraogo, Ouagadougou, Burkina Faso.
Department of Paediatrics and Neonatal Medicine, Centre Hospitalier du Valais Romand, Sion, Switzerland.
Pediatr Neonatol. 2018 Jun;59(3):281-287. doi: 10.1016/j.pedneo.2017.09.009. Epub 2017 Oct 9.
Renal vein thrombosis (RVT) is a rare but well-known neonatal entity for which several therapeutic modalities are reported in the literature because of the lack of consensus management guidelines.
A retrospective study of the medical records of children managed between January 1990 and December 2013, and whose final diagnosis was RVT. The diagnosis was initially clinical and subsequently confirmed by the abdominal ultrasonography (AUS) and Doppler imaging if necessary. The abdominal CT scan was performed when the AUS finding led to the suspicion of RVT extension to the inferior vena cava (IVC). Each patient's birth parameters (birth weight [BW], birth length [BL], and head circumference [HC]) and modalities were recorded. The treatment modalities, the outcome at follow-up along with results of etiological screening were also recorded.
Five newborn infants were diagnosed as having unilateral RVT at the mean postnatal age of 3.8 days (range, 1-11 days). All presented with a classical triad associated nephromegaly, thrombocytopenia, and gross hematuria. Two patients had genetic thrombophilic risk factors (1 heterozygous Leiden factor V mutation in case 4, and Activated Protein C resistance in case 5). Two infants were managed conservatively, and the other three received antithrombotic treatment (recombinant tissue plasminogen activator and heparin). All five patients had a similar course, leading to non-functioning renal atrophy, despite aggressive thrombolytic therapy or conservative treatment.
We suggest that simple unilateral RVT be managed conservatively, while antithrombotic therapy may be attempted for unilateral RVT extending into the inferior vena cava and for bilateral RVT.
肾静脉血栓形成(RVT)是一种罕见但为人熟知的新生儿疾病,由于缺乏共识性的管理指南,文献中报道了几种治疗方式。
对1990年1月至2013年12月间接受治疗且最终诊断为RVT的儿童病历进行回顾性研究。诊断最初基于临床症状,必要时随后通过腹部超声(AUS)和多普勒成像进行确认。当AUS检查结果怀疑RVT延伸至下腔静脉(IVC)时,进行腹部CT扫描。记录每位患者的出生参数(出生体重[BW]、出生身长[BL]和头围[HC])及治疗方式。还记录了治疗方式、随访结果以及病因筛查结果。
5例新生儿在平均出生后3.8天(范围1 - 11天)被诊断为单侧RVT。所有患儿均表现出与肾肿大、血小板减少和肉眼血尿相关的典型三联征。2例患者存在遗传性血栓形成风险因素(病例4为1个杂合的莱顿因子V突变,病例5为活化蛋白C抵抗)。2例婴儿接受保守治疗,另外3例接受抗血栓治疗(重组组织型纤溶酶原激活剂和肝素)。尽管进行了积极的溶栓治疗或保守治疗,所有5例患者的病程相似,均导致患侧肾萎缩失功(无功能)。
我们建议对于单纯性单侧RVT采用保守治疗,而对于延伸至下腔静脉的单侧RVT和双侧RVT可尝试抗血栓治疗。