Mocan H, Beattie T J, Murphy A V
Renal Unit, Royal Hospital for Sick Children, Yorkhill, Glasgow, Scotland, UK.
Pediatr Nephrol. 1991 Jan;5(1):45-9. doi: 10.1007/BF00852843.
Sixteen children (11 male, 5 female) who developed renal venous thrombosis in the neonatal period or early infancy have been followed for periods varying from 29 months to 16 years (median 12 years) after initial diagnosis. Twelve patients presented with a hyperosmolar state, and in 4 the illness was preceded by severe birth asphyxia. The diagnosis was based on the findings of clinical and/or radiological renal enlargement (15 cases), haematuria (16 cases) and elevation in plasma urea (16 cases). Thrombocytopenia (13 cases), red cell fragmentation (13 cases) and oliguria (13 cases) were frequent associated findings. All 16 patients survived the acute illness, 1 patient died 3 years later from an unrelated event. On follow-up evaluation, 11 patients have normal renal function (glomerular filtration rate greater than 80 ml/min per 1.73 m2), 5 developed hypertension, 2 of whom responded to unilateral nephrectomy. Urinary concentrating capacity was subnormal (less than 800 mosmol/kg) in 7 of the 15 cases studied. Follow-up renal imaging studies were undertaken in 14 patients, and the imaging procedure was normal in only 2 of these. Of the remainder, 8 showed unilateral abnormality and 4 bilateral abnormality. Intellectual development was normal in 12 patients, mildly impaired in 1, and severely impaired in 3.
16名在新生儿期或婴儿早期发生肾静脉血栓形成的儿童（11名男性，5名女性）在初次诊断后接受了29个月至16年（中位数12年）不等的随访。12例患者表现为高渗状态，4例在发病前有严重的出生窒息。诊断基于临床和/或放射学检查发现的肾脏肿大（15例）、血尿（16例）和血浆尿素升高（16例）。血小板减少（13例）、红细胞碎片（13例）和少尿（13例）是常见的伴随发现。所有16例患者均在急性疾病中存活，1例患者3年后死于无关事件。在随访评估中，11例患者肾功能正常（肾小球滤过率大于80 ml/min per 1.73 m2），5例发生高血压，其中2例对单侧肾切除术有反应。在15例接受研究的患者中，7例的尿浓缩能力低于正常（小于800 mosmol/kg）。14例患者进行了随访肾脏影像学检查，其中只有2例检查结果正常。其余患者中，8例显示单侧异常，4例显示双侧异常。12例患者智力发育正常，1例轻度受损，3例严重受损。
