Gilbert Rodney D, Evans Hazel, Olalekan Kazeem, Nagra Arvind, Haq Mushfequr R, Griffiths Mark
Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, SO16 6YD, Hampshire, UK.
Faculty of Medicine, University of Southampton, Southampton, UK.
Pediatr Nephrol. 2017 May;32(5):893-896. doi: 10.1007/s00467-017-3584-9. Epub 2017 Feb 13.
Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful.
CASE DIAGNOSIS/TREATMENT: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size.
Tolvaptan may be a useful treatment for severe neonatal PKD.
严重的新生儿常染色体显性多囊肾病（ADPKD）较为罕见，且易与隐性多囊肾病相混淆。治疗此类婴儿难度较大，且往往不成功。
病例诊断/治疗：一名患有巨大肾肿大、呼吸功能不全和低钠血症的女婴接受了精氨酸加压素受体2拮抗剂托伐普坦治疗。这纠正了低钠血症，且肾脏大小未进一步增大。
托伐普坦可能是治疗严重新生儿多囊肾病的一种有效疗法。
