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Archives de pédiatrie : organe officiel de la Société française de pédiatrie v.24 no.12, 2017년, pp.1338 - 1343   SCIE
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Idiopathic nephrotic syndrome
Aspects cliniques du syndrome nEphrotique idiopathique de l'enfant

Boyer, O. (Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Necker-Enfants-Malades, institut Imagine, université ) ; Baudouin, V. (Paris-Descartes, Assistance publique–Hôpitaux de Paris, 75015 Paris, France ) ; Bérard, E. (Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Robert-Debré, institut Imagine, université ) ; Dossier, C. (Paris-Diderot, Assistance publique–Hôpitaux de Paris, 75019 Paris, France ) ; Audard, V. (Service de néphrologie pédiatrique, CHU de Nice, Archet 2, 06200 Nice, France ) ; Guigonis, V. (Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Robert-Debré, institut Imagine, université ) ; Vrillon, I. (Paris-Diderot, Assistance publique ) ;
  • 초록  

    Abstract 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/m 2 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.


    REsumE Le syndrome nEphrotique (SN) est caractErisE par une albuminurie massive responsable d’une hypo-albuminEmie, d’œdEmes, d’une hyperlipidEmie et de complications diverses notamment infectieuses et thrombo-emboliques. Le SN idiopathique (SNI) de l’enfant, ou ≪ nEphrose lipoIdique ≫ reprEsente plus de 85 % des cas pEdiatriques. Son incidence annuelle est de 1–4/100 000 enfants. Il peut survenir A tout Age, mais dEbute le plus souvent entre 2 et 7 ans, souvent aprEs un facteur dEclenchant infectieux ou allergique. Il est le plus souvent dEcouvert devant un syndrome œdEmateux d’installation rapide, ou parfois devant une complication inaugurale. Sa physiopathologie est encore mal comprise, mais il rEsulterait d’un dEsordre immunologique avec production d’un facteur circulant dit de permEabilitE glomErulaire non encore identifiE A ce jour. La biopsie rEnale n’est indiquEe au diagnostic qu’en cas d’atypie : enfant de moins de 1 an ou plus de 12 ans, hEmaturie macroscopique, dEbut progressif, hypertension artErielle sEvEre, signes extrarEnaux, hypocomplEmentEmie, insuffisance rEnale, sErologie hEpatite positive. La corticothErapie constitue le traitement de premiEre intention du SNI de l’enfant. Les traitements symptomatiques associent : une restriction sodEe, une restriction hydrique en cas de natrEmie


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