Transfusional iron overload in patients with myelodysplastic syndromes: A 10-year retrospective survey from a French general hospital

We retrospectively assessed the characteristics of 165 MDS patients from our institution having received at least 20 RBC units. In the vast majority of them various comorbidities (range: 1–6 per patient) were registered including mainly cardiovascular disorders. Serum ferritin was over 1000 μg/L in about half of tested individuals. A chelator agent was initiated in 43.6% of patients (mainly low-risk MDS). Transformation in AML occurred in 46 cases (27.8%). Overall, 112 patients died during follow up. The cause of death was documented in 65 cases and included mainly MDS or AML resistance to therapy. There was a context of bacterial or fungal-related sepsis in 35.3% of cases. We noticed a correlation between survival and number of RBC transfusions. Median OS from the 20th RBC unit was significantly prolonged among the chelated subgroup. Consequences of transfusional iron overload and chelation need to be clarified in MDS patients.     

Trajectory of ambulatory blood pressure after adenotonsillectomy in children with obstructive sleep apnea: comparison at three- and six-month follow-up

ObjectiveLimited information is currently available on 24-h ambulatory blood pressure (ABP) changes after adenotonsillectomy (T&A) in children with obstructive sleep apnea (OSA). In this study, the trajectory of 24-h ABP changes after surgery in children with OSA was examined at three-month and six-month follow-up.MethodsChildren aged 4–16 years with clinical symptoms of OSA and polysomnography (PSG)-diagnosed OSA (apnea-hypopnea index [AHI] >1) were included. All the children received T&A. PSG was conducted before and after surgery. Twenty four hour ABP was monitored using the linear mixed model before, three months after, and six months after surgery.ResultsIn total, 122 children were examined (mean age: 7.9 years; 71% were boys). The AHI significantly decreased from 12.7 ± 16.7 to 2.4 ± 3.2 events/h after T&A (P < 0.001). Overall diastolic blood pressure (DBP; from 65.1 to 63.4 mmHg, P = 0.01) and night-time DBP (from 57.4 to 55.4 mmHg, P = 0.032) decreased nonsignificantly during the six-month postoperative period. The OSA children with presurgical hypertension exhibited significant reductions in overall systolic blood pressure (SBP), overall DBP, daytime DBP, night-time SBP, and night-time DBP at the three-month and six-month postoperative follow-up (all P < 0.05). The three-month and six-month ABP data did not differ significantly in the entire cohort, even between children with presurgical hypertension and non-hypertensive children.ConclusionThe 24-h ABP decreased significantly in the OSA children with hypertension at three and six months after surgery. Moreover, ABP findings did not differ significantly between the three- and six-month follow-up.     

Acute Coronary Syndrome Does Not Have a Negative Impact on Outcomes after Coronary Artery Bypass Grafting in Patients with Left Main Disease

Purpose: Early and long-term outcomes of coronary artery bypass grafting (CABG) in patients with left main disease (LMD) with acute coronary syndrome (ACS) have never been assessed.Methods: Between September 2004 and April 2012, 459 patients with LMD underwent first-time isolated CABG. Of those, 191 patients had ACS and 268 did not. Early and late postoperative outcomes were compared between two groups.Results: Patients in the LMD+ACS group were older and more likely to be female. Left ventricular ejection fraction was lower in the LMD+ACS group. In both groups, bilateral internal thoracic artery grafts were used in over 90% of patients and off-pump technique in over 95%. Operative death rate was not significantly different between the groups (LMD+ACS: 2.1% vs. LMD–ACS: 0.4%). Log-rank test revealed that the actuarial survival rate (79.2 ± 3.7% vs. 81.5 ± 3.5%) and freedom from major adverse cardiac and cerebrovascular events (MACCE) (69.2 ± 4.2% vs. 67.0 ± 4.1%) were similar between groups at 7 years. Multivariate analyses demonstrated that ACS was not identified as an independent predictor of operative death, late mortality, and late MACCE.Conclusion: ACS did not have a negative impact on early and late outcomes of CABG in patients with LMD.     

Deferasirox chelation therapy in patients with transfusion‐dependent MDS: a ‘real‐world’ report from two regional Italian registries: Gruppo Romano Mielodisplasie and Registro Basilicata

Deferasirox (DFX) is an orally administered iron chelator approved for use in patients with transfusion‐dependent iron overload due to myelodysplastic syndromes (MDS). The safety and efficacy of DFX has been explored in clinical trial settings, but there is little data on unselected patients with MDS. The aim of this study was to retrospectively evaluate the safety, compliance, efficacy and effect on haematopoiesis of DFX in a large ‘real‐world’ MDS population. One hundred and eighteen patients with transfusion‐dependent MDS were treated with DFX across 11 centres in Italy. Serum ferritin levels, haematological response, dosing, adverse events and transfusion dependence were recorded at baseline, 3, 6, 12 and 24 months following initiation of treatment. DFX reduced mean serum ferritin levels from 1790 to 1140 ng/mL (P < 0.001), with 7.1% of patients achieving transfusion independence. Significant haematological improvement was seen in erythroid (17.6%), platelet (5.9%) and neutrophil counts (7.1%). Adverse events were reported in 47.5% of patients, including gastrointestinal and renal toxicity. Regression analysis showed that higher starting doses of DFX are associated with transfusion independence at 24 months. DFX is a safe, effective treatment for transfusion‐dependent MDS that can lead to transfusion independence and haematological improvement in a subset of patients.     

Imaging of regional pain syndromes; from syndromes to conditions using imaging?

Musculoskeletal regional pain syndromes often lead to patient referrals in general and rheumatological practice. Detailed history taking and clinical examination can in most cases reveal the cause for pain and direct the subsequent management of the conditions. Yet, when in doubt, imaging methods have to support the clinical assessment.This paper presents the underlying pathologies of the most frequently encountered regional pain syndromes and the role of musculoskeletal ultrasonography and magnetic resonance imaging in their visualization. It presents data, where available, on diagnostic accuracy and comparisons with gold standards.The article stresses the advantages and disadvantages of the analyzed imaging modalities and suggests the future research agenda.     

Antimicrobial dosing in critically ill patients with sepsis-induced acute kidney injury

Severe sepsis often leads to multiple organ dysfunction syndromes (MODS) with acute kidney injury (AKI). AKI affects approximately, 35% of Intensive Care Unit patients, and most of these are due to sepsis. Mortality rate of sepsis-induced AKI is high. Inappropriate use of antimicrobials may be responsible for higher therapeutic failure, mortality rates, costs and toxicity as well as the emergence of resistance. Antimicrobial treatment is particularly difficult due to altered pharmacokinetic profile, dynamic changes in patient''s clinical status and, in many cases, need for renal replacement therapy. This article aims to describe the appropriate antimicrobial dosing and reviews the factors contributing to the difficulties in establishing precise guidelines for antimicrobial dosing in sepsis-induced AKI patients. Search strategy: Text material was collected by systematic search in PubMed, Google (1978–2013) for original articles.     

Myeloid neoplasms with germline predisposition: Practical considerations and complications in the search for new susceptibility loci

Genomic research in hematological malignancies has focused far more prominently on somatic mutations than on germline variants. Although increasing numbers of germline variants are being identified, a substantial proportion of familial myeloid malignancies have no causal allele pinpointed. Here we review the biological, technological, and clinical challenges that stand in the way of the goal of establishing, implementing, and interpreting a comprehensive panel of germline variants for testing. Achieving this goal would inform care for large numbers of myeloid malignancy patients. Furthermore, knowledge of germline susceptibility variants and their corresponding genes will shed light on disease processes, potentially suggesting therapeutic strategies tailored to specific variants.