title: [Transient correction of partial congenital factor V deficiency in nephrotic syndrome].
abstract: Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.
authors.affiliation: Service de néphrologie pédiatrique, hôpital Trousseau, Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Monfort-Gouraud
authors.forename: B
authors.initials: B
authors.lastname: Leroy
authors.forename: R
authors.initials: R
authors.lastname: Favier
authors.forename: A
authors.initials: A
authors.lastname: Bensman
mesh_terms: D002675:Child, Preschool; D005166:Factor V Deficiency / Q000150:complications* / Q000151:congenital*; D005260:Female; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications; D008775:Methylprednisolone / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D012075:Remission, Spontaneous
chemical_list: D011241:Prednisone; D008775:Methylprednisolone
vernacular_title: Correction transitoire d'un déficit congénital partiel en facteur V au cours d'un syndrome néphrotique.