[Cerebral venous thrombosis and immune thrombocytopenia in a 7-year-old girl: a fortuitous association?]. / Thrombose veineuse cérébrale et thrombopénie immune chez une enfant de sept ans : une association fortuite ?

Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by autoantibody-mediated peripheral platelet destruction. It is rarely accompanied by thrombosis. Here, we describe a wide cerebral venous thrombosis that occurred at the onset of a primary ITP in a 7-year-old girl. ITP was confirmed by the presence of anti-platelet antibodies. Whether ITP is a risk factor for venous thrombosis is a matter of debate. The platelet microparticles released during the platelet destruction and the interaction between the autoantibodies and the platelet glycoproteins may contribute to platelet activation. Increased risk of thromboembolic events should be considered in all patients with ITP, including children.

[Steroid intake before leukemia diagnosis impairs outcome in childhood acute lymphoblastic leukemia]. / Corticothérapie préalable au diagnostic de leucémie aiguë lymphoblastique (LAL) de l'enfant : effet délétère pour la prise en charge diagnostique et thérapeutique.

UNLABELLED: The aim of this study was to show that steroid therapy taken before the diagnosis of acute lymphoblastic leukemia (ALL) can alter the management of the disease. PATIENTS AND METHODS: We conducted a multicenter retrospective study on 11 children treated between 2005 and 2011, who received oral steroids ranging from 0.6 to 3.3mg/kg/day prednisolone equivalent for a duration of 2 to 15 days during the 2 months prior to diagnosis of ALL. RESULTS: Four children had febrile pancytopenia. Among them, 2 had severe infections and a noncontributive bone marrow aspiration. One of them presented a severe tumoral lysis syndrome and was hospitalized twice in the intensive care unit. Two teenagers had central nervous system involvement at diagnosis of T-ALL, 1 having associated cutaneous locations, the second one showing pulmonary and central nervous system (CNS) leukostasis with renal failure and disseminated intravascular coagulation. One child died of septic shock during the induction phase of steroid-resistant T-ALL. Four children had no complications during the induction phase. Steroid resistance occurred in 5 cases and steroid sensitivity could not be evaluated in 3 cases. Three allogeneic bone marrow transplants were performed: the first one because of early CNS relapse, the 2 others because of initial treatment resistance. CONCLUSION: Steroids can induce a delay in the management of ALL and seem to favor initial complications, and possibly increase diffuse locations as well as steroid resistance. Their prescription needs to be carefully managed, especially for uncharacteristic infectious symptoms. Then a complete blood count should be done.

Pandemic A/H1N1/2009 influenza in a paediatric haematology and oncology unit: successful management of a sudden outbreak.

Viral respiratory infections are potentially life-threatening among children treated for cancer. We report a nosocomial outbreak of six cases of pandemic influenza A/H1N1/2009 on a paediatric haematology and oncology ward. Three patients developed pneumonia and two of them sustained haemodynamic collapse. The source was probably a relative of the first infected patient. The outbreak was probably spread by cross-infection between patients during communal activities. A few days' delay in identifying the outbreak promoted spread of the influenza. Infection control measures included the use of oral oseltamivir treatment for all hospitalised patients, isolation of the infected patients, strict personal protective controls and a restriction on visitors. No new cases occurred after implementation of these containment measures. At the time when the outbreak was identified, all the patients were already isolated for other reasons. We conclude that A/H1N1/2009 influenza may spread rapidly and cause severe infection in paediatric cancer patients but can be efficiently contained. Identification of isolated or clustered cases should lead to the rapid implementation of appropriate infection control measures.

[Difficult transitions from paediatric to adult care in type 1 Gaucher disease]. / Maladie de Gaucher de type 1 : difficultés pour la transition enfants-adultes.

Gaucher disease is the most frequent lysosomal storage disease. It is a progressive chronic disease that can lead to substantial bone and joint damage and hematological cytopenias. This progressive disease severely worsens the patients' quality of life. Over the past 20 years, enzymatic treatment has considerably improved both symptoms and quality of life. Yet, bone and joint damage may be irreversible. This emphasizes the importance of rigorous follow-up so as to begin uninterrupted treatment before lesion onset. The transition from pediatric to adult care is a major concern for chronic patients. This step is often associated with follow-up problems and may lead to disease worsening. We present three cases of patients who were lost to follow-up during the transition phase. For all 3 patients, the disease notably worsened because follow-up was interrupted during this period.

[Streptococcal pharyngitis in the pediatric emergency department: value and impact of rapid antigen detection test]. / Angine streptococcique aux urgences pédiatriques: performances et impact d'un test de diagnostic rapide.

UNLABELLED: The French Health Agency (Afssaps) recommands the resort to the streptocoque rapid test strip for a better targetting of antibiotics prescription. OBJECTIVES: To evaluate the systematic use of the test in a pediatric emergengy department. METHODS: Comparison of results of the test to the laboratory cultures. Our study took place between January and December 2004. Two thousand one hundred (and) forty-four children with acute pharyngitis have been included (mean age: 4.8+/-3.6 years). RESULTS: Group A streptococcus was involved in 57 pharyngitis (26%). Mean age of children with positive culture was 7+/-3 years versus 4+/-3 years if culture was negative (p>0.05). The test sensitivity was 77% (CI 95%: 65-86) and its specificity 82% (CI 95%: 75-87), the positive predictive value was 60% (CI 95%: 49-71) and the negative predictive value 91% (CI 95%: 85-95). Antibiotic prescriptions have diminished: no prescription for 70% of pharyngitis and for 93% if cultures were negative. CONCLUSION: The rapid test strip wide use has a strong repercussion on the antibiotics prescription. This test is realist in pediatric emergency unit.

Aortic root infection in a prosthetic valve demonstrated by gallium-67 citrate SPECT.

A 70-year-old man presented with 6 weeks of worsening low back pain, fever, sweating, and weight loss with known severe lumbosacral osteoarthritis. His history included CABG in 1992, porcine aortic valve replacement, and permanent pacemaker implantation in 2002. CT of the chest, abdomen, and pelvis did not demonstrate a cause for the symptoms. Blood cultures grew penicillin-sensitive enterococcus and he was referred for evaluation of possible osteodiskitis or epidural abscess. Gallium planar imaging demonstrated increased activity in the lumbar spine, suspicious for the presence of infection, and activity was noted in the mid mediastinum as well. SPECT clearly showed increased Ga-67 activity in the region of the aortic root, suspicious for infection. A perivalvular aortic root abscess was subsequently demonstrated by transesophageal echo. This case illustrates the value of Ga-67 chest SPECT in patients with prosthetic valves for detection of endocarditis.

Differential atrial stunning after electrical cardioversion: an echo tissue Doppler case study.

Left atrial stunning after cardioversion is a well-known phenomenon. It has been associated with higher risk of postcardioversion thromboemboli and increased risk of recurrence of atrial fibrillation. We present a case of differential atrial stunning after electrical cardioversion for atrial fibrillation. Diagnosis was made by pulsed wave Doppler of mitral, tricuspid, and pulmonary vein inflow and mitral and tricuspid annuli. Differential mechanical atrial stunning may be a common phenomenon after cardioversion and may suggest difference in right and left atrial transport function. Its prevalence needs to be determined by a large study. Doppler tissue imaging might be routinely used in patients after cardioversion for atrial fibrillation to detect atrial stunning.

Echocardiographic and pathologic features of explanted Hancock and Carpentier-Edwards bioprosthetic valves in the mitral position.

Modes of failure of Hancock and Carpentier-Edwards (C-E) porcine bioprosthetic valves placed in the mitral position are not completely understood. We reviewed transesophageal echocardiographic (n = 19) and pathologic features of failed Hancock (n = 22) and C-E (n = 8) porcine mitral valves in 30 patients (mean age 70 +/- 13 years). Age at implantation (59 +/- 14 vs 58 +/-14 years, p = 0.9), time to implanted valve degeneration (13 +/- 5 vs 11 +/- 2 years, p = 0.3), and size of bioprosthesis (30 +/- 2 vs 31 +/- 2 mm, p = 0.14) of the implanted Hancock and C-E valves were similar. Anterior leaflet was flail in 15 versus flail posterior leaflet in 5 patients (p = 0.0004). Eccentric posterior mitral regurgitation jet was present in 12, eccentric anterior jet in 2, central jet in 2, and paravalvular jet in 3 patients. Stenosis of bioprosthesis was present in 1 1 Hancock versus 1 C-E valve (p = 0.06). Stent creep at any stent post was present in 14 Hancock versus no C-E valve (p = 0.0013). Large commissural dehiscence was present in 5 C-E versus 1 Hancock valve (p = 0.0006). Ring margin perforation was the most common perforation in Hancock valves (p <0.05, analysis of variance versus all other Hancock perforations). Dehiscence at the stent posts was the most common perforation in C-E valves (p <0.05 vs other C-E perforations, analysis of variance and p <0.001 versus Hancock valves). Thus, Hancock valves showed greater stenosis and stent creep, whereas C-E valves showed large dehiscences at the stent posts on explantation. The anterior leaflet degenerated most frequently in both valves. These findings suggest that the valve design may influence the mechanisms of porcine valve degeneration.

Clinical, echocardiographic, and pathologic features of aortic wall dehiscence of porcine bioprosthetic valves: a cause of rapidly progressive mitral regurgitation and heart failure after bioprosthetic mitral valve replacement.

The aim of this study was to define the clinical, echocardiographic, and pathologic correlates of commissural dehiscence of aortic wall from the stent post of the porcine bioprostheses in the mitral position. This form of valve degeneration was found in 5 of 23 explanted mitral bioprostheses. A thickened, separated aortic wall at multiple commissural sites along with other evidence of valve degeneration was identified in the three patients who had chronic congestive heart failure. A large dehiscence at a single commissural site with otherwise normal valve morphology was present in the two patients who had acute heart failure. Two dimensional/Doppler echocardiography showed a prolapsing or a flail anteriorly positioned leaflet and an eccentric posteriorly directed mitral regurgitation jet in all patients. These echocardiographic findings in patients with a porcine bioprosthetic mitral valve should suggest commissural dehiscence from the aortic wall as a possible mechanism of valve failure. Exclusive involvement of the porcine aortic bioprosthesis placed in the mitral position along with involvement of strut of the bioprosthesis facing the aortic root in all cases suggests excessive hemodynamic stress on the valve in the mitral position and in particular on the anteriorly placed strut as the potential cause of this form of valve degeneration.

Does the presence and site of myocardial ischemia on perfusion scintigraphy predict the occurrence and site of future myocardial infarction in patients with stable coronary artery disease?

In 47 patients who had undergone myocardial scintigraphy, reversible perfusion abnormality was detected in only 28 segments (60%) that were the site of future acute myocardial infarction.

Synchronized coronary venous retroperfusion for support and salvage of ischemic myocardium during elective and failed angioplasty.

To determine the safety and efficacy of synchronized coronary venous retroperfusion during brief periods of ischemia, 30 patients undergoing angioplasty of the left anterior descending coronary artery were studied. Each patient underwent a minimum of two angioplasty balloon inflations. Alternate dilations were supported with retroperfusion; the unsupported inflations served as the control inflations. Synchronized retroperfusion was performed by pumping autologous femoral artery blood by means of an electrocardiogram-triggered retroperfusion pump into the great cardiac vein through a triple lumen 8.5F balloon-tipped retroperfusion catheter inserted percutaneously from the right internal jugular vein. Clinical symptoms, hemodynamics and two-dimensional echocardiographic wall motion abnormalities were analyzed. Retroperfusion was associated with a lower angina severity score (0.8 +/- 1 vs. 1.2 +/- 1) and delay in onset of angina (53 +/- 31 vs. 37 +/- 14 s; p less than 0.05) compared with the control inflations. The magnitude of ST segment change was 0.11 +/- 0.14 mV with retroperfusion and 0.16 +/- 0.17 mV without treatment (p less than 0.05). The severity of left ventricular wall motion abnormality was also significantly (p less than 0.01) reduced with retroperfusion compared with control (0.7 +/- 1.4 [hypokinesia] vs. -0.3 +/- 1.6 [dyskinesia]). There were no significant changes in hemodynamics, except in mean coronary venous pressure, which increased from 8 +/- 3 mm Hg at baseline to 13 +/- 6 mm Hg with retroperfusion. Four patients required prolonged retroperfusion for treatment of angioplasty-induced complications. The mean retroperfusion duration in these patients was 4 +/- 2 h (range 2 to 7). In the three patients who underwent emergency bypass surgery, the coronary sinus was directly visualized during surgery and found to be without significant injury. There were no major complications. Minor adverse effects were transient atrial fibrillation (n = 2), jugular venous catheter insertion site hematomas (n = 4) and atrial wall staining (n = 1), all of which subsided spontaneously. Thus, retroperfusion significantly reduced and delayed the onset of coronary angioplasty-induced myocardial ischemia and provided effective supportive therapy for failed and complicated angioplasty.

[Incidence and treatment of coronary restenosis in spite of the implantation of an endoprosthesis]. / L'incidence et le traitement de la resténose coronarienne malgré l'implantation d'une endoprothèse.

Between April 1986 and February 1988, 72 endoprosthesis implantations were carried out in the coronary arteries of 65 patients. The indication for implantation was either abrupt closure post angioplasty or prevention of restenosis. The first 3 cases of restenosis within the stented segment are reported. Two were treated by repeat balloon angioplasty, with temporary improvement. The possible mechanisms underlying this particular form of restenosis are discussed.

Lack of sustained clinical improvement in an elderly population after percutaneous aortic valvuloplasty.

The hemodynamic and clinical results following 25 percutaneous aortic valvuloplasty (PAV) procedures on 24 patients are reported. The mean aortic gradient decreased from 66 +/- 23 to 40 +/- 15 mm Hg (p less than 0.001) and the mean valve area increased from 0.5 +/- 0.17 to 0.7 +/- 0.26 cm2 (p less than 0.001). Although the aortic gradient determined by both Doppler and direct measurement correlated well before PAV, the Doppler gradients determined 24 +/- 48 hours after the procedure were significantly higher than the directly measured gradients at the time of PAV. Ninety-two percent of patients were New York Heart Association class III or IV before PAV. Of the remaining 12 patients, 6 (50%) were classified as class II, 2 (17%) were class III, and 4 were (33%) class IV. Necropsy examination of the patient who died shortly after valvuloplasty revealed localized hematoma and tear in the anterior mitral valve leaflet. Smaller initial valve areas yielded smaller valve areas after PAV. Patients with greater valve areas following PAV showed greater functional improvement. Only 7 patients (29%) had at least 1 functional class improvement at the final follow-up. PAV can be performed relatively safely in elderly, moribund patients, although sustained improvement of functional status is not common. In this population, PAV should be limited to patients severely symptomatic in whom surgery is not an acceptable option.

Acquired left coronary artery to left atrium fistula: unusual complication of aortocoronary bypass.

This report describes a very unusual complication of aortocoronary bypass graft surgery. It consists of the formation of an acquired fistula between the left circumflex coronary artery and the left atrium in a patient who was operated two years prior to its clinical presentation. Significant coronary steal gave rise to disabling angina and prompted a second operation to close the fistula. The diagnosis was made with selective arteriogram that clearly delineated the fistulous tract and surgery was accomplished without extracorporeal circulation.

Intracoronary thrombolysis in acute myocardial infarction: experimental background and clinical experience.

Occlusive intracoronary (IC) thrombosis was produced experimentally in dogs by placement of a copper coil. The thrombus was consistently lysed by application of Thrombolysin (streptokinase and plasminogen) at the site of occlusion, 1 to 6 hours after thrombosis. Thrombolysin has no toxic effect on the coronary artery wall or the myocardium. Reperfusion after 30 to 60 minutes of occlusion frequently resulted in ventricular fibrillation, but gradual reperfusion reduced the probability of ventricular fibrillation. Intramyocardial bleeding was noted after reperfusion in areas of advanced necrosis and was shown to be the consequence, rather than the cause, of necrosis. The reperfused myocardium remained hypocontractile, but in contrast to the occlusion period, its mechanical function could be enhanced by inotropic stimulation. After experimental studies confirmed the feasibility and safety of IC thrombolysis, the technique was applied within 3 hours of onset of pain in 29 patients with evolving acute myocardial infarction (AMI) and showing ST elevations without pathologic Q waves. Nitroglycerin (NTG), 0.1 mg, was injected into the occluded coronary artery to rule out spasm; NTG failed to open the occluded artery. A special, very flexible, radiopaque No. 2 French catheter was advanced through the angiography catheter to the site of occlusion. Thrombolysin was infused at a rate of 4000 to 6000 IU/min until patency was achieved, followed by 2000 IU/min for 60 minutes. Lysis of clot was achieved in 27 of 29 patients. The single death (unrelated to the procedure) occurred subsequently in a patient in whom the artery was not reopened. After successful thrombolysis, 12 patients underwent elective coronary bypass surgery because of multiple stenoses. The need for early reperfusion is emphasized for effective IC thrombolysis therapy in evolving AMI.