Percutaneous Atrial Septal Defect Closure Using the Occlutech Figulla Device in Adults: More than 800 Patient-Years of Follow-Up.

PURPOSE: The Occlutech Figulla occluder has been proven safe and effective at midterm follow-up after percutaneous atrial septal defect (ASD) closure. We describe the safety and efficacy at long-term follow-up in adults. METHODS: All consecutive adult patients that underwent ASD closure between 2008 and 2015 were included. All complications were registered. Residual left-to-right shunt (LRS) was diagnosed using color-Doppler transthoracic echocardiography (TTE). Right-to-left shunting was diagnosed using contrast TTE. Successful closure was defined as no LRS at follow-up. RESULTS: In total, 166 patients (mean age 56.7 ± 16.1 years; 62% female) underwent percutaneous ASD closure using the Occlutech Flex I (70%) or Flex II (30%) device (diameter 24 mm; range 10-40 mm) under general anaesthesia and transoesophageal echocardiographic guidance. Long-term follow-up data were available for 144 patients (87%) with a mean follow-up of 5.9 ± 2.6 years, a total of 814 patient-years. During hospitalization, device embolization occurred in three patients (1.8%) with successful extraction in all. During the long-term follow-up, 15 patients (9.8%) suffered new-onset atrial fibrillation and stroke occurred in 2.1%. There was no residual LRS at 12-month follow-up. No device embolization occurred during the long-term follow-up. CONCLUSION: Percutaneous ASD closure using the Occlutech device appears to be safe at long-term follow-up with a high successful closure rate at one year.

Acquired sensorimotor polyneuropathy in an adolescent boy with primary intracranial sarcoma.

Acquired polyneuropathies (PN) are rare in childhood and adolescent. We report on a 15-year-old male patient who presented with progressive gait instability, ataxia, neuropathic pain, distal muscle weakness and progressive loss of ambulation. Nerve conduction studies (NCS) revealed a progressive demyelinating sensorimotor polyneuropathy predominantly of the lower limbs. Cerebrospinal fluid (CSF) analyses revealed a cytoalbuminologic dissociation. Extensive diagnostic workup for autoantibodies and inflammatory markers was inconclusive. Corticosteroids and intravenous immunoglobulins did not affect. Cranial MRI revealed leptomeningeal enhancement of the cerebellum and the brainstem. Brain biopsy of the cerebellar lesions revealed an unclassifiable sarcoma. The patient was treated according to the CWS guidance study resulting in a decrease in enhanced lesion size. After two years NCS still revealed a demyelinating sensorimotor PN. This case report describes for the first time the clinical course of a chronic PN, putative paraneoplastic, associated with isolated unclassifiable CNS-sarcoma in an adolescent patient. Paraneoplastic pathogenesis should be considered in an unusual sequence of subacute progressive neurological symptoms even in children and adolescents.

Five-year results of the complete versus culprit vessel percutaneous coronary intervention in multivessel disease using drug-eluting stents II (CORRECT II) study: a prospective, randomised controlled trial.

OBJECTIVES/BACKGROUND: In patients with multivessel coronary artery disease (MVD) the decision whether to treat a single culprit vessel or to perform multivessel revascularisation may be challenging. The purpose of this study was to evaluate the long-term outcome of multivessel percutaneous coronary intervention (MV-PCI) versus culprit vessel only (CV-PCI) in patients with stable coronary artery disease or non-ST elevation acute coronary syndrome. METHODS: In this dual-centre, prospective, randomised study a total 215 patients with MVD were randomly assigned to MV-PCI or CV-PCI. The primary endpoint was the occurrence of major adverse cardiac events (MACE) including death, myocardial infarction (MI), and repeat revascularisation. Secondary endpoints were the combined endpoint of death or MI, the individual components of the primary endpoint, and the occurrence of stent thrombosis. Patients were followed up to 5 years after enrolment. RESULTS: The occurrence of the primary endpoint was similar at 28% versus 31% in the MV-PCI and CV-PCI group, respectively (hazard ratio [HR] 0.87, 95% confidence interval [CI]: 0.53-1.44, p = 0.59). The rate of repeat revascularisation was 15% versus 24% (HR 0.59, 95% CI 0.32 to 1.11, p = 0.11), whereas definite or probable stent thrombosis occurred in 2% versus 0% (p = 0.44). CONCLUSIONS: In this randomised study comparing the strategies for MV-PCI and CV-PCI in patients with MVD, no difference was found in the occurrence of MACE after 5 years. We observed a numerically higher rate of death or MI and a lower rate of repeat revascularisation after MV-PCI, although these findings were not statistically significant.

Veno-arterial extracorporeal membrane oxygenation in addition to primary PCI in patients presenting with ST-elevation myocardial infarction.

INTRODUCTION: Primary percutaneous coronary intervention (pPCI) in ST-elevation myocardial infarction (STEMI) can cause great haemodynamic instability. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) can provide haemodynamic support in patients with STEMI but data on outcome and complications are scarce. METHODS: An in-hospital registry was conducted enrolling all patients receiving VA-ECMO. Patients were analysed for medical history, mortality, neurological outcome, complications and coronary artery disease. RESULTS: Between 2011 and 2016, 12 patients underwent pPCI for STEMI and received VA-ECMO for haemodynamic support. The majority of the patients were male (10/12) with a median age of 63 (47-75) years and 4 of the 12 patients had a history of coronary artery disease. A cardiac arrest was witnessed in 11 patients. The left coronary artery was compromised in 8 patients and 4 had right coronary artery disease. All patients were in Killip class IV. Survival to discharge was 67% (8/12), 1­year survival was 42% (5/12), 2 patients have not yet reached the 1­year survival point but are still alive and 1 patient died within a year after discharge. All-cause mortality was 42% (5/12) of which mortality on ECMO was 33% (4/12). Patient-related complications occurred in 6 of the 12 patients: 1 patient suffered major neurological impairment, 2 patients suffered haemorrhage at the cannula site, 2 patients had limb ischaemia and 1 patient had a haemorrhage elsewhere. There were no VA-ECMO hardware malfunctions. CONCLUSION: VA-ECMO in pPCI for STEMI has a high survival rate and neurological outcome is good, even when the patient is admitted with a cardiac arrest.

Percutaneous closure of a patent foramen ovale after cryptogenic stroke.

A patent foramen ovale is a common intracardiac finding that is located between the left and right atrium. It can cause right-to-left shunting and has a high prevalence in patients who suffer a cryptogenic stroke. Earlier trials did not show superiority of percutaneous patent foramen ovale closure with standard medical therapy over standard medical therapy alone in the treatment of cryptogenic stroke. Interestingly, several meta-analyses show positive results regarding closure, suggesting underpowering of the individual trials. Recently, two large prospective trials and one long-term follow-up study showed benefit of percutaneous closure over standard medical therapy in treatment of cryptogenic stroke. A larger right-to-left shunt or the presence of an atrial septal aneurysm were predictors for a recurrent event. Therefore, percutaneous patent foramen ovale closure after cryptogenic stroke should be recommended over antiplatelet therapy alone in patients younger than 55 years of age with a high-risk patent foramen ovale.

Feasibility of multiplane microtransoesophageal echocardiographic guidance in structural heart disease transcatheter interventions in adults.

INTRODUCTION: Structural heart interventions are guided by transoesophageal or intracardiac echocardiography (TEE/ICE). MicroTEE, developed for paediatric purposes, is smaller and therefore less invasive and traumatic, avoiding the need for general anaesthesia. We aimed to show feasibility of procedural guidance by comparing image quality of microTEE with standard TEE and ICE during adult transcatheter interventions, and assess the accuracy in obtaining left atrial appendage (LAA) measurements between the microTEE probe and standard TEE. METHODS AND RESULTS: We prospectively included 49 patients (20 women, 64 ± 18 years). Intraprocedural images were obtained by using the microTEE probe and standard (2D and 3D) TEE (LAA closure, MitraClip implantation) or ICE (interatrial communication closure, transseptal puncture for left atrial ablation). Two echocardiographers independently assessed image quality from 1 (excellent) to 4 (poor) and performed LAA measurements. Use of microTEE was not related to significant discomfort. Image quality obtained with the microTEE probe was lower than with standard TEE (2 [1-2] vs. 1 [1-2]; p = 0.04) and comparable with ICE images (2 [1-2] vs. 2 [1-2], p = 0.13). MicroTEE showed a wider field of view than ICE. LAA measurements on images obtained by microTEE were strongly associated with standard TEE. CONCLUSIONS: MicroTEE seems feasible for guidance during transcatheter heart interventions in adults. MicroTEE imaging offers a wider field of view than ICE, and its accuracy is comparable with TEE. In transcatheter interventions performed under conscious sedation, microTEE might be a viable and advantageous alternative to standard TEE or ICE.

A case of rivaroxaban-associated acute tubulointerstitial nephritis.

Rivaroxaban is a direct oral anticoagulant that is prescribed for the prevention and treatment of thromboembolisms. Rivaroxaban is cleared renally and a common side effect (1-10%) is renal impairment of unknown pathophysiology. We are the first to describe a case of biopsy-proven acute tubulointerstitial nephritis, most likely caused by rivaroxaban.

Increased megakaryocytic proliferation, pro-platelet deposition and expression of fibrosis-associated factors in children with chronic myeloid leukaemia with bone marrow fibrosis.

Paediatric chronic myeloid leukaemia (ped-CML) is rare and ped-CML with fibre accumulation in the bone marrow (MF) is thought to be even rarer. In adults (ad-CML), fibrosis represents an adverse prognostic factor. So far, the pro-fibrotic changes in the bone marrow microenvironment have not been investigated in detail in ped-CML. From a total of 66 ped-CML in chronic phase, biopsies were analysable and 10 had MF1/2 (MF1, n=8/10; MF2, n=2/10). We randomly selected 16 ped-CML and 16 ad-CML cases with and without fibrosis (each n=8) as well as 18 non-neoplastic controls. Bone marrow samples were analysed with a real-time PCR-based assay (including 127 genes for paediatric cases) and by immunohistochemistry. We found increased expression of megakaryocytic genes in ped-CML. The number of megakaryocytes and pro-platelets are increased in CML patients, but the most significant increase was noted for ped-CML-MF1/2. Anti-fibrotic MMP9 expression was lower in children than in adults. Cell mobilisation-related CXCL12 was decreased in young and adult patients with CML but not the corresponding receptor CXCR4. In summary, fibre accumulation in ped-CML-MF1/2 is associated with increased megakaryocytic proliferation and increased interstitial pro-platelet deposition. Deregulated expression of matrix-modulating factors shifts the bone marrow microenvironment towards fibrosis.

Impact of percutaneous coronary intervention timing on 5-year outcome in patients with non-ST-segment elevation acute coronary syndromes. The 'wait a day' approach might be safer.

BACKGROUND: The OPTIMA trial was a randomised multicentre trial exploring the influence of the timing of percutaneous coronary intervention (PCI) on patient outcomes in an intermediate to high risk non-ST-elevation acute coronary syndrome (NSTE-ACS) population. In order to decide the best treatment strategy for patients presenting with NSTE-ACS, long-term outcomes are essential. METHODS: Five-year follow-up data from 133 of the 142 patients could be retrieved (94 %). The primary endpoint was a composite of death and spontaneous myocardial infarction (MI). Spontaneous MI was defined as MI occurring more than 30 days after randomisation. Secondary endpoints were the individual outcomes of death, spontaneous MI or re-PCI. RESULTS: No significant difference with respect to the primary endpoint was observed (17.8 vs. 10.1 %; HR 1.55, 95 % CI: 0.73-4.22, p = 0.21). There was no significant difference in mortality rate. However, spontaneous MI was significantly more common in the group receiving immediate PCI (11.0 vs. 1.4 %; HR 4.46, 95 % CI: 1.21-16.50, p = 0.02). We did not find a significant difference between the groups with respect to re-PCI rate. CONCLUSION: There was no difference in the composite of death and spontaneous MI. The trial suggests an increased long-term risk of spontaneous MI for patients treated with immediate PCI.

TIDES-ACS Trial: comparison of titanium-nitride-oxide coated bio-active-stent to the drug (everolimus)-eluting stent in acute coronary syndrome. Study design and objectives.

Drug-eluting stents (DES), delivering antiproliferative drugs from a durable polymer, have shown to reduce in-stent restenosis after percutaneous coronary intervention (PCI) compared to bare-metal stents (BMS). However, they have been associated with a hypersensitivity reaction, delayed healing, and incomplete endothelialization, which may contribute to an increased risk of late stent thrombosis. Consequently, a prolonged duration of dual antiplatelet therapy (DAPT) is needed, with an increased risk of bleeding complication. A number of stent technologies are being developed in an attempt to modify late thrombotic events and DAPT duration. The Optimax™ stent is such a novel, next generation bioactive stent (BAS), in which a thicker layer of titanium-nitride-oxide coating is inserted over the stent struts. The rationale of this is to obtain more efficient and rapid vascular healing at the site of the stent implantation. The aim of TIDES-ACS Trial is to compare clinical outcome in patients presenting with ACS, treated with PCI using Optimax-BAS versus Synergy™-EES. Second objective is to explore whether the Optimax™-BAS use is superior compared with Synergy™-EES use with respect of hard end points (cardiac death, myocardial infarction [MI] and major bleeding). A prospective, randomized, multicenter trial (ClinicalTrials.gov Identifier: NCT02049229), will be conducted in interventional centres in Finland (six centres), France (five centres) and Holland (two centres), including a total of 1800 patients.

Ultra-thin strut cobalt chromium bare metal stent usage in a complex real-world setting. (SOLSTICE registry).

AIM: To report clinical follow-up at 6 months after implantation of the ultra-thin strut cobalt chromium SolarFlex stent in a real-world setting. METHODS AND RESULTS: Patients (n = 240) with single or multiple vessel coronary artery disease undergoing percutaneous coronary intervention (PCI) at four sites in Europe were enrolled in the SOLSTICE (SolarFlex Stent in Routine Clinical Practice) registry. Follow-up at 6 months was 100 %. Diabetes was present in 29 % of the patients, 30 % presented with acute myocardial infarction and 17 % had unstable angina. Of the patients, 27 % had previously undergone PCI or coronary artery bypass surgery. Lesion complexity was high (50 % B2 + C type lesions). Device success was achieved in 99.7 % of cases and the major adverse cardiac event (MACE) rate was 5.8 % at 6 months of follow-up. Target lesion revascularisation (TLR) was 5.0 % at 6 months. CONCLUSIONS: The SOLSTICE registry showed that in a complex real-world setting the SolarFlex bare metal stent, with ultra-thin struts and customised scaffolding, provided low clinical MACE and TLR rates. These results provide support for the use of the latest generation bare metal stent in contemporary European practice.

Long-term imatinib treatment does not cause testicular toxicity in male adolescents with chronic myeloid leukemia and in a juvenile rat model.

BACKGROUND: The impact of exposure to the tyrosine kinase inhibitor (TKI) imatinib (IMA) on the male reproductive endocrine system is still discussed controversially. We therefore investigated testosterone (Testo) and inhibin B (InB) in blood serum from male adolescents with chronic myeloid leukemia (CML) under long-term TKI treatment. Also long-term exposure to TKIs was studied in a juvenile rat model. METHODS: Serum was collected at 3 months intervals from 13 boys (age: 7.8-18.9 years, median: 12.8 years) with CML receiving TKI treatment over 3-58 months (median: 18 months). 4 weeks (w) old male rats were exposed, either chronically or intermittently, via the drinking water to a standard (SD) and a high dose (=2-fold SD) of IMA, dasatinib (DASA), or bosutinib (BOSU) over a 10 w period. Controls received water only. Animals were sacrificed after 2 w (prepubertal), 4 w (pubertal), and 10 w (postpubertal) of exposure. Testo and InB serum levels were measured by ELISA. RESULTS: Boys exhibited Testo and InB levels within normal age-related reference ranges and no pattern of rising or falling levels during TKI treatment could be observed. In rats, Testo levels under IMA exposure tended to be non-significantly lowered at postpubertal age compared to controls while no significant differences were found under DASA and BOSU exposure. Animals' InB levels did not significantly differ from controls for all TKIs, at all doses, and by all application schemes tested. CONCLUSION: With the limitation that the number of individuals tested was rather small, testicular toxicity due to TKI seems unlikely as no alterations of Testo and InB blood levels neither in male adolescent patients nor in rats under long-term TKI exposure was observed.

TK inhibitor treatment disrupts growth hormone axis: clinical observations in children with CML and experimental data from a juvenile animal model.

BACKGROUND: Long-term treatment of chronic myeloid leukemia (CML) with tyrosine kinase inhibitors (TKIs) exerts off-target effects on bone growth by either impaired growth hormone (GH) action or osseous modelling impairment. METHODS: Body height and the GH-related parameters insulin-like growth factor 1 (IGF-1) and insulin-like growth factor-binding protein 3(IGFBP-3) were determined repetitively 3-monthly over 2 years in 21 pediatric CML-patients on standardized imatinib treatment. In an animal model 4-week-old male Wistar rats were exposed over 10 weeks to imatinib, dasatinib, or bosutinib at varying concentrations via the drinking water. Blood was collected at prepubertal age, pubertal age, and at adult age, respectively, and animals' serum levels of IGFBP-3 were measured. RESULTS: Independent from treatment duration patients exhibited IGF-1 and IGFBP-3 levels almost exclusively in the very low range when compared to age-matched references. No clear pattern of rising or falling IGF-1 and IGFBP-3 levels was observed. In rats, compared to controls, serum IGFBP-3 was significantly lowered for all TKIs tested, at all concentrations applied, and at all ages under investigation. CONCLUSION: Besides direct off-target effects on the growing skeleton, TKI treatment also results in lowered blood levels of IGF-1 and IGFBP-3.A juvenile rat model predicts this side effect for dasatinib and bosutinib. Thus, growth and GH- related parameters should be monitored regularly in pediatric patients with CML on TKIs.

Long-term follow-up of children conditioned with Treosulfan: German and Austrian experience.

We report the long-term follow-up of children transplanted with Treosulfan (TREO)-based conditioning in Germany and Austria. Nine centres reported a total of 109 transplantations. Patients were stratified according to the paediatric TRM risk score derived from the paediatric BMT registry (PRST) and compared with the historical transplant population of this registry. Underlying diseases were malignancies, immunodeficiencies, and haematologic and metabolic disorders. TREO total dose ranged from 21-42 g/m(2). Additional conditioning drugs included fludarabine, thiotepa, melphalan, CY and/or TBI. EFS at 3 years for non-malignant and malignant diseases was 88% and 49%, respectively. Leukaemia patients in remission had a survival of 51% at 3 years; nonremission patients relapsed and died within 18 months. TRM and OS in the low-risk groups 0 and 1 were similar to PRST controls. TRM in the high-risk groups 2 and 3 was markedly lower (9% vs 28% and 13% vs 53%, respectively) than in the PRST group, but OS was similar. In conclusion, TREO-based conditioning regimens in children resulted in excellent engraftment and long-term survival in nonmalignant disease. In high-risk malignancy, low acute toxicity was followed by low TRM but it did not translate into increased survival.

High versus standard clopidogrel loading in patients undergoing carotid artery stenting prior to cardiac surgery to assess the number of microemboli detected with transcranial Doppler: results of the randomized IMPACT trial.

AIM: The aim of this study was to compare the effects of 300 mg or 600 mg clopidogrel loading dose, prior to carotid artery stenting (CAS) on the number of transcranial Doppler (TCD)-detected microembolic signals (MES) and to investigate the relationship between the magnitude of platelet reactivity and MES. METHODS: In this prospective randomized, double-blind study, 35 consecutive asymptomatic patients (17.1% females), scheduled for CAS and cardiac surgery were included. The primary endpoint was the number of TCD-detected MES. The secondary endpoints were the absolute magnitude of on-treatment platelet reactivity and the adverse cerebral events. Negative binomial regression to find predictors for sum of single emboli, the student's t-test to assess the association between platelet function tests and randomized dose of 300 mg or 600 mg clopidogrel, and the R2 calculation for the assessment of the association between platelet function tests and embolic load, were used. RESULTS: No statistically significant difference in the number of TCD-detected MES, in the sum of all the single emboli or showers and platelet aggregation measurements between the two groups was observed (aggregometry: 21.7±18.3 versus 23±18%, P=0.8499 and 45.8±17.5 versus 46.5±14.5%, P=0.9003) (verifyNow P2Y12 assay: 231±93 PRU versus 222±86 PRU, P=0.7704). In one patient a transient ischemic attack occurred. CONCLUSION: A loading dose of 300 mg of clopidogrel in combination with aspirin is as effective as 600 mg of clopidogrel in achieving adequate platelet inhibition and preventing periprocedural events in asymptomatic patients undergoing CAS prior to cardiac surgery.

Platelet function in obese children and adolescents.

UNLABELLED: Platelet hyperaggregability contributes to thromboembolic events of obesity in adulthood. In obese children hyperaggregability was described in platelet rich plasma. We investigated platelet aggregation in children with obesity and lipometabolic disorders in whole blood. PATIENTS, MATERIAL, METHODS: Specimens from patients with overweight (n = 35), hypercholesterolaemia and normal weight (n = 5), overweight plus combined lipometabolic disorder (n = 5) and healthy controls (n = 20) were investigated. Aggregation and ATP release were induced by ADP (20 µmol/l), collagen (1 µg/ml) and thrombin (0.5 U/ml) using a lumiaggregometer. RESULTS: Overweight children and normal weight patients with hypercholesterolaemia exhibited no significant differences in platelet aggregation compared to controls. Contrastingly, in patients with obesity plus lipometabolic disorder the aggregation rate was significantly higher (p < 0.05) suggesting a hyperaggregable state. CONCLUSION: Obviously in obese children a hypercoagulable state exists and the slight hyperaggregability observed in whole blood in this cohort might contribute to that. Any effort should be undertaken to avoid obesity in children especially in those countries where the prevalence of obesity in childhood is continuously increasing.

Identifying quality improvement intervention evaluations: is consensus achievable?

BACKGROUND: The diversity of quality improvement interventions (QIIs) has impeded the use of evidence review to advance quality improvement activities. An agreed-upon framework for identifying QII articles would facilitate evidence review and consensus around best practices. AIM: To adapt and test evidence review methods for identifying empirical QII evaluations that would be suitable for assessing QII effectiveness, impact or success. DESIGN: Literature search with measurement of multilevel inter-rater agreement and review of disagreement. METHODS: Ten journals (2005-2007) were searched electronically and the output was screened based on title and abstract. Three pairs of reviewers then independently rated 22 articles, randomly selected from the screened list. Kappa statistics and percentage agreement were assessed. 12 stakeholders in quality improvement, including QII experts and journal editors, rated and discussed publications about which reviewers disagreed. RESULTS: The level of agreement among reviewers for identifying empirical evaluations of QII development, implementation or results was 73% (with a paradoxically low kappa of 0.041). Discussion by raters and stakeholders regarding how to improve agreement focused on three controversial article selection issues: no data on patient health, provider behaviour or process of care outcomes; no evidence for adaptation of an intervention to a local context; and a design using only observational methods, as correlational analyses, with no comparison group. CONCLUSION: The level of reviewer agreement was only moderate. Reliable identification of relevant articles is an initial step in assessing published evidence. Advancement in quality improvement will depend on the theory- and consensus-based development and testing of a generalizable framework for identifying QII evaluations.

Allogeneic stem cell transplantation for pediatric and adolescent patients with CML: results from the prospective trial CML-paed I.

PURPOSE: Stem cell transplantation (SCT) can definitely cure chronic myeloid leukemia (CML), a rare disease in childhood. We prospectively evaluated the results of early SCT in pediatric CML after standardized pretreatment with hydroxyurea+/-interferon. PATIENTS AND METHODS: Between 1995 and 2004, 200 children (median age: 12.4 years) were enrolled and stratified: given the availability of an HLA-matched related donor (MRD), SCT was scheduled within 6 months and otherwise from an unrelated donor (UD) within 12 months following diagnosis. RESULTS: 176 patients underwent SCT; from MRD within median 4 months and from UD within median 11 months after diagnosis. At SCT, 158 patients were in chronic phase (CP1 or CP2), 9 patients were in accelerated phase and 9 patients were in blast crisis (BC). The conditioning regimen - total body irradiation or busulfan - exerted no different impact on overall survival (OS). Probability of OS at 5 years was 87+/-11% if grafted from a sibling (n=41), 52+/-9% from matched UD (MUD, n=71), and 45+/-16% from mismatched donors (MMD, n=55), respectively. A trend for better OS in CP1 was observed if SCT was performed within 6 months (n=49; 74+/-9%), compared to 7-12 months (n=52; 62+/-15%), and >12 months (n=43; 62+/-17%) after diagnosis, respectively (p=0.157). Probability of relapse at 5 years was 20+/-12%. Transplant-related mortality and graft-versus-host disease mainly contributed to the inferior outcome in UD and HLA-mismatched SCT. CONCLUSION: These data from the first prospective trial on CML restricted to children and adolescents might be considered for decision making when balancing the risks of SCT against the increasing use of imatinib as upfront treatment for CML.

[Clinical and laboratory aspects of the Aspirin-like defect as hereditary thrombocytopathy]. / Klinische und labordiagnostische Aspekte des Aspirin-like Defekts als hereditäre Thrombozytopathie.

UNLABELLED: The Aspirin-like defect (ALD) is caused by defects in the intraplatelet arachidonic acid (AA)-metabolism. We here present the characteristics of a larger cohort in a single centre. PATIENTS, METHODS: Based on 17 ALD index patients bleeding symptoms, agonist-induced platelet aggregation and closure times in the PFA-100 test were analysed in a family cohort of altogether 52 individuals from 17 families. Absent aggregation to AA (maximal aggregation or=1 bleeding symptoms. CONCLUSION: In case of a bleeding tendency diagnostic procedures should rule out primary haemostatic defects. Hereditary platelet function defects including ALD are an important differential diagnosis. Family studies are reasonable.

New bioabsorbable septal repair implant for percutaneous closure of a patent foramen ovale: short-term results of a single-centre experience.

BACKGROUND: Permanent implants for closure of a patent foramen ovale (PFO) have a number of possible disadvantages including erosions, thrombus formation, and allergic reactions. The incidence of adverse events may be lower using a bioabsorbable device. OBJECTIVE: To evaluate the short-term safety and efficacy of a new bioabsorbable closure device. METHODS: All 35 consecutive patients (21 female, mean age 47.9 +/- 10.8 years), who underwent a percutaneous PFO closure between November 2007 and July 2008, were included. All complications were reported. The efficacy was based on the residual shunting the day after implant and at 1 month follow-up and was graded as minimal, moderate, or severe, using contrast transthoracic echocardiography with the Valsalva manoeuvre. RESULTS: The only in-hospital complication was a surgical device retrieval from the femoral vein. Four patients developed a minimal inguinal haematoma. One day after closure, residual shunting was present in 56% of the patients (minimal 27%, moderate 23% and severe 6%). At 1 month follow-up (n = 33), one patient developed a transient neurological deficit and three patients suffered from paroxysmal atrial fibrillation. A residual shunt at 1 month was present in 45% of the patients (minimal 30%, moderate 12%, and severe 3%). CONCLUSIONS: Percutaneous PFO closure using the bioabsorbable closure device seems to be safe. However, a high rate of residual shunting is present at 1 month follow-up. Long-term follow-up data are necessary to evaluate the efficacy and safety of this device.