Nefrologia Pediatrica

Vall de Ebo, Spain

Nefrologia Pediatrica

Vall de Ebo, Spain

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Herrero-Calvo J.A.,Hospital Clinico San Carlos | Gonzalez-Parra E.,Servicio de Nefrologia | Perez-Garcia R.,Hospital Universitario Infanta Leonor | Tornero-Molina F.,Hospital del Sureste | And 87 more authors.
Nefrologia | Year: 2012

Objectives: The study's objectives were to determine which anticoagulation methods are commonly used in patients who are undergoing haemodialysis (HD) in Spain, on what criteria do they depend, and the consequences arising from their use. Material and Method: Ours was a cross-sectional study based on two types of surveys: a "HD Centre Survey" and a "Patient Survey". The first survey was answered by 87 adult HD units serving a total of 6093 patients, as well as 2 paediatric units. Among these units, 48.3% were part of the public health system and the remaining 51.7% units were part of the private health system. The patient survey analysed 758 patients who were chosen at random from among the aforementioned 78 HD units. Results: A) HD Centre Survey: The majority of adult HD units (n=61, 70.2%) used both types of heparin, 19 of them (21.8%) only used LMWH and 7 of them (8%) only used UFH. The most frequently applied criteria for the use of LMWH were medical indications (83.3% of HD units) and ease of administration (29.5%). The most frequently used methods for adjusting the dosage were clotting of the circuit (88.2% of units), bleeding of the vascular access after disconnection (75.3%), and patient weight (57.6%). B) Patient Survey: The distribution of the types of heparin used was: UFH: 44.1%, LMWH: 51.5%, and dialysis without heparin in 4.4% of patients. LMWH was more frequently used in public medical centres (64.2% of patients) than in private medical centres (46.1%) (P<.001). LMWH was more frequently used in on-line haemodiafiltration (HF) than in high-flux HD (P<.001). Antiplatelet agents were given to 45.5% of patients, oral anticoagulants to 18.4% of patients, and both to 5% of patients. Additionally, 4.4% of patients had suffered bleeding complications during the previous week, and 1.9% of patients suffered thrombotic complications. Bleeding complications were more frequent in patients with oral anticoagulants (P=.001), although there was no association between the type of heparin and the occurrence of bleeding or thrombotic complications. Conclusions: We are able to conclude that there is a great amount of disparity in the criteria used for the medical prescription of anticoagulation in HD. It is advisable that each HD unit revise their own results as well as those from other centres, and possibly to create an Anticoagulation Guide in Haemodialysis. © 2012 Revista Nefrología.


Guaragna M.S.,University of Campinas | Lutaif A.C.G.B.,University of Campinas | Bittencourt V.B.,Nefrologia Pediatrica | Piveta C.S.C.,University of Campinas | And 6 more authors.
Arquivos Brasileiros de Endocrinologia e Metabologia | Year: 2012

Frasier syndrome (FS) is characterized by gonadal dysgenesis and nephropathy. It is caused by specific mutations in the Wilms' tumor suppressor gene (WT1) located in 11p23. Patients with the 46,XY karyotype present normal female genitalia with streak gonads, and have higher risk of gonadal tumor, mainly, gonadoblastoma. Therefore, elective bilateral gonadectomy is indicated. Nephropathy in FS consists in nephrotic syndrome (NS) with proteinuria that begins early in childhood and progressively increases with age, mainly due to nonspecific focal and segmental glomerular sclerosis (FSGS). Patients are generally unresponsive to steroid and immunosuppressive therapies, and will develop end-stage renal failure (ESRF) during the second or third decade of life. We report here four cases of FS diagnosis after identification of WT1 mutations. Case 1 was part of a large cohort of patients diagnosed with steroid-resistant nephrotic syndrome, in whom the screening for mutations within WT1 8-9 hotspot fragment identified the IVS9+5G>A mutation. Beside FS, this patient showed unusual characteristics, such as urinary malformation (horseshoe kidney), and bilateral dysgerminoma. Cases 2 and 3, also bearing the IVS9+5G>A mutation, and case 4, with IVS9+1G>A mutation, were studied due to FSGS and/or delayed puberty; additionally, patients 2 and 4 developed bilateral gonadal tumors. Since the great majority of FS patients have normal female external genitalia, sex reversal is not suspected before they present delayed puberty and/or primary amenorrhea. Therefore, molecular screening of WT1 gene is very important to confirm the FS diagnosis. © ABEM todos os direitos reservados.

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