Ndoudi Likoho Bellaure, Berthaud Romain, Dossier Claire, Delbet Jean-Daniel, Boyer Olivia, Baudouin Véronique, Alison Marianne, Biran Valérie, Hurtaud Marie-Françoise, Hogan Julien, Kwon Theresa, Couderc Anne
Department of Pediatric Nephrology, Robert Debré University Hospital, Assistance Publique - Hôpitaux de Paris, and University of Paris, Paris, France.
Department of Pediatric Nephrology, Necker-Enfants-Malades University Hospital, Assistance Publique - Hôpitaux de Paris, and University of Paris, Paris, France.
Pediatr Nephrol. 2023 Sep;38(9):3055-3063. doi: 10.1007/s00467-023-05918-w. Epub 2023 Mar 29.
Neonatal renal vein thrombosis (NRVT) is a rare condition with little data available.
We retrospectively analyzed newborns diagnosed with NRVT admitted to 3 pediatric nephrology units in Paris from 2005 to 2020.
Twenty-seven patients were analyzed (male = 59%). The median age at diagnosis was 2.5 days (1 - 4.5). Diagnosis was suspected based on at least one of the three cardinal signs of renal vein thrombosis in 93%: flank mass (67%), hematuria (67%) and thrombocytopenia (70%). In all patients, diagnosis was confirmed by ultrasound. All patients had at least one known perinatal risk factor. A prothrombotic risk factor was found in 13 patients (48%). NRVT was unilateral in 70%, involving the left renal vein in 58%. Among 25 treated patients, 19 (76%) received low molecular weight heparin (LMWH) as initial therapy, 2 (8%) received unfractionated heparin and 4 (16%) received fibrinolysis. Median duration of treatment was 8 weeks (4 - 12). Bleeding occurred significantly more often with fibrinolysis than with LMWH/supportive therapy (3 of 4: 75% vs 0 of 4: 0%, p = 0.05). Clot resolution in patients treated with fibrinolysis did not differ significantly from those treated with LMWH/supportive therapy. After a median follow-up of 5.7 years (3 years - 9.9 years), pathological kidney features were observed in 73% of the patients (19 of 26), kidney atrophy in 18 (69%), hypertension in 2 (8%), chronic kidney disease (CKD) in 1 (4%) and proteinuria in 2 (8%).
NRVT remains a challenging condition, which still requires further study because of its associated morbidity. A higher resolution version of the Graphical abstract is available as Supplementary information.
新生儿肾静脉血栓形成(NRVT)是一种罕见疾病,可用数据较少。
我们回顾性分析了2005年至2020年在巴黎3个儿科肾脏病科收治的诊断为NRVT的新生儿。
共分析了27例患者(男性占59%)。诊断时的中位年龄为2.5天(1 - 4.5天)。93%的患者根据肾静脉血栓形成的三个主要体征中的至少一个怀疑诊断:胁腹肿块(67%)、血尿(67%)和血小板减少(70%)。所有患者均通过超声确诊。所有患者至少有一个已知的围产期危险因素。13例患者(48%)发现有血栓形成前危险因素。NRVT单侧发生的占70%,其中累及左肾静脉的占58%。在25例接受治疗的患者中,19例(76%)接受低分子肝素(LMWH)作为初始治疗,2例(8%)接受普通肝素,4例(16%)接受纤维蛋白溶解治疗。中位治疗持续时间为8周(4 - 12周)。与LMWH/支持性治疗相比,纤维蛋白溶解治疗时出血明显更常见(4例中的3例:75% 对4例中的0例:0%,p = 0.05)。接受纤维蛋白溶解治疗的患者与接受LMWH/支持性治疗的患者相比,血栓溶解情况无显著差异。中位随访5.7年(3年 - 9.9年)后,73%的患者(26例中的19例)出现病理性肾脏特征,18例(69%)出现肾萎缩,2例(8%)出现高血压,1例(4%)出现慢性肾脏病(CKD),2例(8%)出现蛋白尿。
NRVT仍然是一种具有挑战性的疾病,由于其相关的发病率,仍需要进一步研究。更高分辨率的图形摘要版本可作为补充信息获取。