Acute renal failure SNAPPE and mortality.

Background: The aim of the present study was to determine, using the score for neonatal acute physiology and perinatal extension II (SNAPPE-II), whether there is an association with acute renal failure (ARF) and whether it is possible to identify newborns at risk for ARF prior to a rise in creatinine in newborns.

Methods: Information on postnatal risk factors and SNAPPE-II on the first day of life (non-ARF group, n= 475; ARF group, n= 78) were collected. Renal failure was defined as serum creatinine level >1 mg/dL and >1.

Differential diagnosis of hereditary nephrogenic diabetes insipidus with desmopressin infusion test.

Purpose: The causes of hereditary nephrogenic diabetes insipidus (HNDI) are the mutations in the arginine vasopressin V2 receptor gene (AVPR2) (90%) and aquaporin 2 gene (AQP2) (10%). Although it is possible to perform mutation analysis where available, differentional diagnosis at clinical bases remains valuable.

Methods: In this report we present two cases of HNDI diagnosed at clinical bases with a desmopressin infusion test as AQP2 gene mutations.

Mutational analysis of the PLCE1 gene in steroid resistant nephrotic syndrome.

Background: Mutations in the PLCE1 gene encoding phospholipase C epsilon 1 (PLCepsilon1) have been recently described in patients with early onset nephrotic syndrome (NS) and diffuse mesangial sclerosis (DMS). In addition, two cases of PLCE1 mutations associated with focal segmental glomerulosclerosis (FSGS) and later NS onset have been reported.

Method: In order to better assess the spectrum of phenotypes associated with PLCE1 mutations, mutational analysis was performed in a worldwide cohort of 139 patients (95 familial cases belonging to 68 families and 44 sporadic cases) with steroid resistant NS presenting at a median age of 23.

Etiology and outcome of acute kidney injury in children.

The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation.

Objectives: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

Methods: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis View Article and Find Full Text PDF