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Arch Pediatr. 2017 Dec;24(12):1338-1343. doi: 10.1016/j.arcped.2017.09.022. Epub 2017 Nov 21.

[Idiopathic nephrotic syndrome].

[Article in French]

Author information

Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Necker-Enfants-Malades, institut Imagine, université Paris-Descartes, Assistance publique-Hôpitaux de Paris, 75015 Paris, France. Electronic address:
Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Robert-Debré, institut Imagine, université Paris-Diderot, Assistance publique-Hôpitaux de Paris, 75019 Paris, France.
Service de néphrologie pédiatrique, CHU de Nice, Archet 2, 06200 Nice, France.
Service de néphrologie et transplantation, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Henri-Mondor, Inserm U955, université Paris-Est-Créteil, 94010 Créteil, France.
Département de pédiatrie, hôpital Mère-Enfant, 87000 Limoges, France.
Service de pédiatrie, hôpital d'enfants, CHRU de Nancy, 54511 Vandœuvre-lès-Nancy, France.


Nephrotic syndrome (NS) is defined by massive proteinuria and hypoalbuminemia, with resulting hyperlipidemia and edema. The most common cause of NS in children is idiopathic nephrotic syndrome (INS), also called nephrosis. Its annual incidence has been estimated to 1-4 per 100,000 children and varies with age, race, and geography. Many agents or conditions have been reported to be associated with INS such as infectious diseases, drugs, allergy, vaccinations, and malignancies. The disease may occur during the 1st year of life, but it usually starts between the ages of 2 and 7 years. INS is characterized by a sudden onset, edema being the major presenting symptom, but may rarely be discovered during a routine urine analysis. The disease may also be revealed by a complication such as hypovolemia, infection (pneumonia and peritonitis due to Streptococcus pneumoniae), deep-vein or arterial thromboses, and pulmonary embolism. Renal biopsy is usually not indicated in a child aged 1-10 years with typical symptoms and a complete remission with corticosteroids. Conversely, it is indicated in children under 1 year in case of macroscopic hematuria, hypertension, low C3 levels, persistent renal failure, or steroid resistance. Steroid therapy is applied in all children whatever the histopathology. Initial prednisone therapy in France consists of 60mg/m2 administered daily for 4 weeks (maximum dose, 60mg/day), followed by alternate-day prednisone with tapering doses. Eight-five to 90 % patients are steroid-responsive and may relapse, but the majority still responds to steroids over the subsequent courses. Only 1-3 % of patients who are initially steroid-sensitive subsequently become steroid-resistant. Children with primary or secondary steroid-resistance are at risk of end-stage kidney disease. Symptomatic treatment includes salt restriction, fluid restriction when natremia is less than 125 meq/L, reduction of saturated fat and carbohydrates, calcium and vitamin D supplements, anticoagulation, and vaccination. Albumin infusions are only indicated in case of complications. Diuretics should be restricted to cases of severe edema, after hypovolemia has been corrected.

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