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Munck A.,University Hospital Robert Debre
Expert Review of Respiratory Medicine | Year: 2010

Nutritional status is strongly associated with pulmonary function and survival in cystic fibrosis patients. Attainment of a normal growth pattern in childhood and maintenance of adequate nutritional status in adulthood represent major goals of multidisciplinary cystic fibrosis centers. International guidelines on energy intake requirements, pancreatic enzyme-replacement therapy and fat-soluble vitamin supplementation are of utmost importance in daily practice. The present review summarizes the most up-to-date information on early nutritional management in newly diagnosed patients and evaluates the benefits of aggressive nutritional support, assessment of nutritional status, recommendations for nutrition-related management in pancreatic-insufficient patients and the possible therapeutic impact of fat intake modulation upon inflammatory status. © 2010 Expert Reviews Ltd. Source

Colombo C.,University of Milan | Ellemunter H.,Innsbruck Medical University | Houwen R.,University Utrecht | Munck A.,University Hospital Robert Debre | And 2 more authors.
Journal of Cystic Fibrosis | Year: 2011

Complete or incomplete intestinal obstruction by viscid faecal material in the terminal ileum and proximal colon - distal intestinal obstruction syndrome (DIOS) - is a common complication in cystic fibrosis. Estimates of prevalence range from 5 to 12 episodes per 1000 patients per year in children, with higher rates reported in adults. DIOS is mainly seen in patients with pancreatic insufficiency, positive history of meconium ileus and previous episodes of DIOS. DIOS is being described with increasing frequency following organ transplantation. Diagnosis is based on suggestive symptoms with a right lower quadrant mass confirmed on X-ray. The main differential is chronic constipation. Treatment consists of rehydration combined with stool softening laxatives or gut lavage with balanced electrolyte solutions. Rapid fluid shifts have been described following osmotic agents. Avoiding dehydration and optimizing pancreatic enzyme dosage may reduce the chance of further episodes. Prophylactic laxative therapy is widely used, but is not evidence-based. © 2011 European Cystic Fibrosis Society. Source

Pluot M.,University Hospital Robert Debre
Pediatric Dermatology | Year: 2012

Hydroa vacciniforme (HV) is a rare photodermatosis. Several therapies, with sometimes severe side effects, have been used in isolated cases. We report a case of refractory HV successfully treated with dietary fish oil rich in Iω-3 polyunsaturated fatty acids. © 2011 Wiley Periodicals, Inc. Source

Perrot A.,University of Lorraine | Luquet I.,University Hospital Robert Debre | Pigneux A.,University Hospital Haut Leveque | Mugneret F.,University Hospital du Bocage | And 16 more authors.
Blood | Year: 2011

The prognosis of acute myeloid leukemia (AML) is very poor in elderly patients, especially in those classically defined as having unfavorable cytogenetics. The recent monosomal karyotype (MK) entity, defined as 2 or more autosomal monosomies or combination of 1 monosomy with structural abnormalities, has been reported to be associated with a worse outcome than the traditional complex karyotype (CK). In this retrospective study of 186 AML patients older than 60 years, the prognostic influence of MK was used to further stratify elderly patients with unfavorable cytogenetics. CK was observed in 129 patients (69%), and 110 exhibited abnormalities according to the definition of MK (59%). MK+ patients had a complete response rate significantly lower than MK- patients: 37% vs 64% (P = .0008), and their 2-year overall survival was also decreased at 7% vs 22% (P < .0001). In multivariate analysis, MK appeared as the major independent prognostic factor related to complete remission achievement (odds ratio = 2.3; 95% confidence interval, 1-5.4, P = .05) and survival (hazard ratio = 1.7; 95% confidence interval, 1.1-2.5, P = .008). In the subgroup of 129 CK+ patients, survival was dramatically decreased for MK+ patients (8% vs 28% at P = .03). These results demonstrate that MK is a major independent factor of very poor prognosis in elderly AML. © 2011 by The American Society of Hematology. Source

Beldjord K.,University Paris Diderot | Chevret S.,University Paris Diderot | Asnafi V.,University of Paris Descartes | Huguet F.,University Hospital Purpan | And 21 more authors.
Blood | Year: 2014

With intensified pediatric-like therapy and genetic disease dissection, the field of adult acute lymphoblastic leukemia (ALL) has evolved recently. In this new context, we aimed to reassess the value of conventional risk factors with regard to new genetic alterations and early response to therapy, as assessed by immunoglobulin/T-cell receptor minimal residual disease (MRD) levels. The study was performed in 423 younger adults with Philadelphia chromosome-negative ALL in first remission (265 B-cell precursor [BCP] and 158 T-cell ALL), with cumulative incidence of relapse (CIR) as the primary end point. In addition to conventional risk factors, the most frequent currently available genetic alterations were included in the analysis. A higher specific hazard of relapse was independently associated with postinduction MRD level ≥10-4 and unfavorable genetic characteristics (ie, MLL gene rearrangement or focal IKZF1 gene deletion in BCP-ALL and no NOTCH1/FBXW7 mutation and/or N/K-RAS mutation and/or PTEN gene alteration in T-cell ALL). These 2 factors allowed definition of a new risk classification that is strongly associated with higher CIR and shorter relapse-free and overall survival. These results indicate that genetic abnormalities are important predictors of outcomein adult ALL not fully recapitulated by early response to therapy. Patients included in this study were treated in the multicenter GRAALL-2003 and GRAALL-2005 trials. Both trials were registered at http://www.clinicaltrials.gov as #NCT00222027 and #NCT00327678, respectively. © 2014 by The American Society of Hematology. Source

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