Papers by Isabelle Guichard
Archives De Pediatrie, May 1, 2014
Revue de Médecine Interne, Dec 1, 2010
Revue de Médecine Interne, Dec 1, 2012
European Journal of Dermatology, Apr 1, 2015
To cite this article: Isolated flushes without permanent, fixed or other skin lesions in patients... more To cite this article: Isolated flushes without permanent, fixed or other skin lesions in patients with systemic mastocytosis: a case series of 9 patients.
Revue d'Oncologie Hématologie Pédiatrique, Dec 1, 2016
Propos.-L'amélioration des traitements des cancers de l'enfant a permis une augmentation de la su... more Propos.-L'amélioration des traitements des cancers de l'enfant a permis une augmentation de la survie à 80 % à 5 ans. La radiothérapie, parfois indispensable au traitement, est associée à des effets indésirables à long terme. L'objectif de cette étude était d'analyser les complications à long terme de patients ayant eu un cancer dans l'enfance et traité par radiothérapie. Méthodes.-L'étude SALTO, basée sur les registres de cancers de l'enfant en Rhône-Alpes-Auvergne, concernait les enfants traités pour un cancer solide ou un lymphome avant 15 ans, diagnostiqué entre 1987 et 1992, et majeurs au moment de l'étude. Une consultation par un oncopédiatre et un interniste a été proposée entre 2011 et 2014. Les enfants traités par radiothérapie ont été comparés aux patients traités sans irradiation. Les séquelles ont été classées en modérées (grade 1-2) ou sévères (grade 3-4) selon la CTCAE. Résultats.-Cinquante-neuf patients ayant reçu de la radiothérapie ont participé à ces consultations et 91 sans radiothérapie. Cinquante-huit patients avec radiothérapie (98,3 %) ont présenté au moins une complication. Les principaux effets secondaires à long terme tous grades confondus étaient orthopédiques (37 %), endocriniens (32 %), thyroïdiens (31 %) et l'obésité (31 %). Les principales complications de grade 3-4 étaient endocriniennes, rénales, une obésité et les cancers secondaires. On retrouvait significativement plus de complications endocriniennes, thyroïdiennes, orthopédiques, cutanées, de surdité et de cancers secondaires chez les patients irradiés comparés aux patients non irradiés. Conclusion.-Ces éléments doivent être intégrés dans le suivi à long terme des enfants ayant eu un cancer pour une meilleure prévention et/ou un traitement précoce de ces complications.
American Journal of Hematology, Apr 15, 2022
Sickle cell disease (SCD) is a severe hemoglobin (Hb) disorder characterized by hemolytic anemia,... more Sickle cell disease (SCD) is a severe hemoglobin (Hb) disorder characterized by hemolytic anemia, recurrent painful vaso-occlusive events, and ischemia/reperfusion-driven inflammation. Acute chest syndrome (ACS) is a common and potentially life-threatening form of acute lung E260 CORRESPONDENCE CORRESPONDENCE E261
Journal of adolescent and young adult oncology, Feb 1, 2019
Survival rate of childhood cancers is now reaching 80% overall. However, early or late complicati... more Survival rate of childhood cancers is now reaching 80% overall. However, early or late complications related to surgery, chemotherapy, and radiotherapy remain at a high rate and greatly increase the risk of late mortality. The objective of this study is to assess the autonomic nervous system (ANS) activity, measured through heart rate variability indices in childhood cancer survivors compared with healthy controls. This prospective study included 51 long-term childhood cancer survivors diagnosed before 15 years of age between 1987 and 1992 and controlled for age and sex with healthy volunteers. We observed a significant increase in spontaneous heart rate (beats per minute) (67 ± 10 vs. 60 ± 10, p = 0.001), and all the studied parameters showed a significantly altered ANS activity in cases compared with healthy controls. In both groups, the main cofactors of dysautonomia (tobacco, drugs, cannabis, estro-progestative pills, alcohol, limited physical activity) were analyzed without any significant difference. The effect of cancer treatments received was not analyzed due to the small number of participants. The results showed a significant ANS dysfunction in childhood cancer survivors compared with healthy controls and suggested the value of autonomic screening to underscore and possibly quantify the effect of the cancer treatments in a larger cohort. This evaluation could lead to the recommendation to increase physical activity, the most efficient way known to improve ANS activity, as already shown in other pathologies (breast cancer).
Revista Brasileira De Terapia Intensiva, Sep 1, 2008
Síndrome de lise tumoral: uma revisão abrangente da literatura Acute tumor lysis syndrome: a comp... more Síndrome de lise tumoral: uma revisão abrangente da literatura Acute tumor lysis syndrome: a comprehensive review ARTIGO DE REVISÃO INTRODUÇÂO Lesão renal aguda (LRA) é uma complicação freqüente, associada à alta mortalidade e morbidade em pacientes críticos com câncer. 1-6 Múltiplas etiologias de lesão renal aguda são frequentemente associadas, sendo à sepse e hipoperfusão as mais comuns. 4,6 Todavia, vários fatores especificamente associados com a malignidade subjacente podem ser esperados. 4,6 A síndrome de lise tumoral (SLT) é uma complicação temível que pode ocorrer espontaneamente ou como conseqüência do início da quimioterapia
Journal of adolescent and young adult oncology, Dec 1, 2017
We evaluated the satisfaction of adult survivors of childhood cancers and their general practitio... more We evaluated the satisfaction of adult survivors of childhood cancers and their general practitioners (GP) after a long-term consultation. The first Long-term Follow-up Study in Oncology (SALTO1) is a prospective cohort study of survivors of childhood cancers (except leukemia) diagnosed between 1987 and 1992 in the Rhône-Alpes and Auvergne regions of France. Of the 481 patients eligible for the study, 150 participated in a long-term consultation with a pediatric oncologist and an internist, after which survivors and their GPs received long-term plans and recommendations based on consultation findings. A year after the consultation, survivors and their GPs assessed their satisfaction with the process. Of the 150 survivor participants in the long-term follow-up, 120 (80%) completed the satisfaction form, with 107 (89%) reporting satisfaction. Forty-eight (32%) expressed strengthening their follow-up as a consequence of the consultation. Of the 79 survivors sent recommendations, 76 (96%) reported reading them, most (n = 68; 86%) found them useful, and 56 (71%) followed recommendations. Of the 107 GPs of the survivors, 82 (77%) conceded having been poorly informed about long-term complications for their patients after chemotherapy, and 93 (88%) appreciated having a hospital contact available for these patients. The long-term consultations ultimately enhanced medical follow-up of survivor participants, improving knowledge of both patients and family physicians regarding the patients' early disease, its treatments, and possible concerns, and offering consultative resources of medical specialists. The levels of participation of survivors and their physicians and reported satisfaction encourage the adoption of such consultations throughout France.
Seminars in Arthritis and Rheumatism, Dec 1, 2021
BACKGROUND Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease usually... more BACKGROUND Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease usually presenting before the age of 10 years. Non-specific clinical features or late-onset presentation may delay its diagnosis until adulthood. OBJECTIVE To determine whether DADA2 diagnosed in adulthood is associated with specific characteristics compared to DADA2 diagnosed in childhood. METHODS We pooled a cohort of 12 adult DADA2 patients followed in France with cases identified through a systematic literature review. For each patient, we determined the type of clinical presentation and assessed six key organ involvements. RESULTS A total of 306 cases were included. Among the 283 patients with available data regarding age at diagnosis, 140 were diagnosed during adulthood and 143 during childhood. The vascular presentation of DADA2 was more frequent in the adult diagnosis group (77.9% vs. 62.9%, p < 0.01), whereas the hematological presentation (bone marrow failure) prevailed in the pediatric diagnosis group (10.0% vs. 20.3% p = 0.02). In patients with vasculopathy, severe skin manifestations developed in 35% and 10% of the adult and pediatric diagnosis groups, respectively. Conversely, fewer strokes occurred in the adult group presenting with systemic vasculopathy (54% vs. 81%). Symptomatic humoral immune deficiency (HID) was rarely a clinical presentation in itself (5% and 2.8%) but accompanied other phenotypes of DADA2, especially the hematological phenotype in the adult group (33% vs. 4%). CONCLUSION DADA2 diagnosed in adulthood presents more often with a vascular phenotype and less often with bone marrow failure than DADA2 diagnosed in childhood. Adults diagnosed with DADA2 vasculopathy display more severe skin involvement but fewer strokes.
Journal of the International Association of Providers of AIDS Care, Jul 1, 2008
Nature Medicine, 2022
Sickle cell disease (SCD) and transfusion-dependent β-thalassemia (TDT) are the most prevalent mo... more Sickle cell disease (SCD) and transfusion-dependent β-thalassemia (TDT) are the most prevalent monogenic disorders worldwide. Trial HGB-205 ( NCT02151526 ) aimed at evaluating gene therapy by autologous CD34+ cells transduced ex vivo with lentiviral vector BB305 that encodes the anti-sickling βA-T87Q-globin expressed in the erythroid lineage. HGB-205 is a phase 1/2, open-label, single-arm, non-randomized interventional study of 2-year duration at a single center, followed by observation in long-term follow-up studies LTF-303 ( NCT02633943 ) and LTF-307 ( NCT04628585 ) for TDT and SCD, respectively. Inclusion and exclusion criteria were similar to those for allogeneic transplantation but restricted to patients lacking geno-identical, histocompatible donors. Four patients with TDT and three patients with SCD, ages 13-21 years, were treated after busulfan myeloablation 4.6-7.9 years ago, with a median follow-up of 4.5 years. Key primary endpoints included mortality, engraftment, replication-competent lentivirus and clonal dominance. No adverse events related to the drug product were observed. Clinical remission and remediation of biological hallmarks of the disease have been sustained in two of the three patients with SCD, and frequency of transfusions was reduced in the third. The patients with TDT are all transfusion free with improvement of dyserythropoiesis and iron overload.
Leukemia & Lymphoma, Dec 9, 2009
Acute tumor lysis syndrome (ATLS) caused by the destruction of malignant cells leads to metabolic... more Acute tumor lysis syndrome (ATLS) caused by the destruction of malignant cells leads to metabolic abnormalities, which may either remain isolated (biological ATLS) or subsequently lead to renal dysfunction (clinical ATLS). We compared hospital and 6-month survival in patients with ATLS with hematological malignancies with or without acute renal injury. Sixty-three patients (median age, 50 years; range, 32-64) were included with ATLS. Twenty-eight had no ARI (including 17 (61%) who subsequently required dialysis) whereas 35 had an ATLS-related ARI (including 31 (89%) who required dialysis). Acute leukemia (n = 28) and lymphoma (n = 30) were the main malignancies. All patients had high tumor burdens. Hospital and 6-month mortality rates were significantly lower in patients without ARI (7% and 21%, respectively) than in the ATLS-related renal injury group (51% and 66%). After adjustment for acute disease severity, presence of ARI at ICU admission was associated with higher hospital mortality (odds ratio, 10.41; 95% confidence interval, 2.01-19.170; p = 0.005) and 6-month mortality (odds ratio, 5.61; 95% confidence interval, 1.64-54.66; p = 0.006), compared to patients without renal injury. Our study suggests that in patients with ATLS, ICU management when acute renal injury is present is associated with higher short- and long-term mortality.
Revue de Médecine Interne, Dec 1, 2019
Revue de Médecine Interne, Jun 1, 2018
une insuffisance cardiaque droite. Le diagnostic d'une péricardite constrictive est confirmé au c... more une insuffisance cardiaque droite. Le diagnostic d'une péricardite constrictive est confirmé au cathétérisme cardiaque droit et à l'IRM cardiaque. Le Quantiféron est positif et la clearance fécale ␣1antitrypsine à 26 N. Au total le diagnostic d'EE secondaire à une péricardite constrictive (PC) tuberculeuse est retenu. Discussion La MW-ou lymphangiectasie intestinale primitive (révélation tardive dans notre observation)-et la PC sont des étiologies rares d'EE (PC : 10 cas dans la littérature). Les EE sont découvertes lors d'une exploration d'une hypoglobulinémie, une diarrhée (intermittente), un syndrome de malabsorption biologique (inconstant) et confirmées par la clearance fécale de ␣1antitrypsine. Conclusion L'EE-entité souvent méconnue-une fois le diagnostic confirmé impose la recherche d'une étiologie (EE parfois curable après un traitement spécifique) telles que celles rapportées dans notre travail (MW et PC). Déclaration de liens d'intérêts Les auteurs déclarent ne pas avoir de liens d'intérêts.
Revue de Médecine Interne, 2018
Revue de Médecine Interne, Jun 1, 2013
Revue de Médecine Interne, Jun 1, 2012
Revue de Médecine Interne, Dec 1, 2014
Revue de médecine interne 35S (2014) A96-A200 évolution clinique et biologique avec une augmentat... more Revue de médecine interne 35S (2014) A96-A200 évolution clinique et biologique avec une augmentation du taux d'hémoglobine à 13 g/dL. Discussion Parmis les effets secondaires de l'imatinib on note en premier lieu les manifestations cutanées, les oedèmes, les troubles digestifs, les myalgies et les crampes. La toxicité hématologique a été toujours liée à la myélosuppression associant une anémie, une leucopénie et/ou une thrombopénie, un seul cas d'anémie hémolytique auto-immune sous imatinib a été rapporté avec une bonne évolution sous prédnisone. Son mécanisme physiopathologique reste encore mal élucidé. Conclusion Il apparaît important de connaître cet effet secondaire lié à ce médicament. Déclaration d'intérêts L'auteur déclare ne pas avoir de conflits d'intérêts en relation avec cet article.
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Papers by Isabelle Guichard