ABSTRACT
INTRODUCTION: A retrospective analysis was performed to document the clinical and electrophysiological features of Guillain-Barré syndrome (GBS) subtypes using different diagnostic criteria. METHODS: One hundred GBS patients were included. Clinical and laboratory features were analyzed, and patients were classified according to four sets of diagnostic criteria. Electrodiagnostic criteria were also analyzed. RESULTS: A total of 69 patients met Asbury and Cornblath's criteria, 96 met Van der Meché's criteria, 99 met Wakerley's diagnostic classification and 86 met level 1 or 2 of the Brighton criteria. Rates of GBS subtypes were: 69% classic GBS; 8% Miller-Fisher syndrome; 12% paraparetic GBS; 2% pharyngeal-cervical-brachial GBS; and 9% unclassified. Those for electrodiagnostic subtypes were 52% demyelinating and 9% axonal according to Hadden's criteria vs 41% demyelinating and 41% axonal as per Rajabally's criteria. CONCLUSION: In this study of case distribution within the GBS spectrum of a retrospective cohort of French patients, the application of new diagnostic criteria enabled accurate diagnoses and classifications of the different subtypes, and also increased the recognition of axonal GBS.
Subject(s)
Guillain-Barre Syndrome/classification , Guillain-Barre Syndrome/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Electrodiagnosis , Electrophysiology , Female , Guillain-Barre Syndrome/physiopathology , Humans , Male , Middle Aged , Neural Conduction/physiology , Retrospective Studies , Young AdultSubject(s)
Hemarthrosis/diagnosis , Hemophilia A/diagnosis , Hemophilia B/diagnosis , Joints/diagnostic imaging , Pain/diagnosis , Ultrasonography/methods , Acute Disease , Adolescent , Adult , Hemarthrosis/epidemiology , Hemophilia A/epidemiology , Hemophilia B/epidemiology , Humans , Pain/epidemiology , Perception , Retrospective Studies , Severity of Illness Index , Young AdultABSTRACT
Hematopoietic stem cell transplantation (HSCT) with sibling donors (s.d.) is a life-saving intervention for patients with hematological malignancies. Numerous genetic factors have a role in transplant outcome. Several functional polymorphisms have been identified in TGF-ß1 gene, such as single-nucleotide polymorphism (SNP) at +29C>T within exon 1. Two hundred and forty five patient/donor pairs who underwent a s.d. HSCT in our centers were genotyped for this SNP. In the myeloablative cohort, +29CC donors were associated with an increase in severe chronic GvHD (32% vs 16%, hazard ratio (HR) 9.0, P=0.02). Regarding survival outcomes, +29CC patients developed higher non relapse mortality (NRM) (1-5 years CC 28-32% vs TC/TT 7-10%; HR 5.1, P=0.01). Recipients of +29TT donors experienced a higher relapse rate (1-5 years TT 37-51% vs TC 19-25% vs CC 13%-19%; HR 2.4, P=0.01) with a decreased overall survival (OS) (1-5 years TT 69-50% vs TC/CC 77-69%; HR 1.9, P=0.05). Similar to previous myeloablative unrelated donors HSCT results, we confirmed that +29CC patients had higher NRM. In addition we found that +29TT donors might be associated with a higher relapse rate and lower OS. These results should be confirmed in larger series. Identification of these SNPs will allow personalizing transplant conditioning and immunosuppressant regimens, as well as assisting in the choice of the most appropriate donor.
Subject(s)
Hematopoietic Stem Cell Transplantation/methods , Tissue Donors , Transforming Growth Factor beta1/genetics , Adult , Donor Selection/methods , Female , Genotype , Graft vs Host Disease/genetics , Hematologic Neoplasms/complications , Hematologic Neoplasms/mortality , Hematologic Neoplasms/therapy , Hematopoietic Stem Cell Transplantation/mortality , Hematopoietic Stem Cell Transplantation/standards , Humans , Male , Myeloablative Agonists/therapeutic use , Polymorphism, Single Nucleotide , Recurrence , Siblings , Survival Analysis , Transplantation Conditioning/methods , Treatment OutcomeABSTRACT
Presentamos un caso de NPH tratado mediante extirpación de piel en la zona más dolorida (11,3 x 26,0 cm2). Esta intervención quirúrgica alivió el dolor, eliminó la alodinia táctil y permitió reducir considerablemente el consumo de medicación durante un periodo de seguimiento de 1 año. Se realizó una evaluación cualitativa de 14 biopsias de sacabocado y 10 tiras de piel (de 10 mm de longitud cada una) extirpadas de la zona con dolor como consecuencia de la NPH. La evaluación se realizó mediante inmunofluorescencia con marcaje doble utilizando anticuerpos contra la proteína producto del gen 9.5 (PGP 9.5), la proteína de los neurofilamentos de 200 kDa (NF), el péptido relacionado con el gen de la calcitonina (CGRP) y el receptor vainilloide 1 (VR-1). Comparado con una biopsia de sacabocado de la piel contralateral, el patrón de inervación cutánea en la piel afectada por la NPH presentó diferencias sistemáticas y considerables. Los resultados pueden explicar la base anatómica de la prueba de respuesta a la capsicina y son importantes para conocer mejor los mecanismos clínicos responsables del dolor en la NPH. © 2002 International Association for the Study of Pain. Publicado por Elsevier Science B.V (AU)
