Predictive factors for residual hypertension following aortic coarctation stenting

Native coarctation of the aorta (CoA) and recoarctation (reCoA) after initial surgical repair are frequently associated with hypertension (HT). Most CoA cases are amenable to transcatheter balloon angioplasty with stent implantation; however, the impact of stenting on arterial blood pressure (BP) is variable. We carried out a retrospective study to identify the predictive factors for residual HT despite optimal endovascular treatment. Patients who had undergone stent implantation for native CoA or reCoA with a pressure gradient of >20 mm Hg between the upper and lower limbs, between 2007 and 2015, were included. The geometry and level of hypoplasia of the aortic arch were determined by non‐invasive imaging, and BP measurements were performed pre‐ and post‐procedure. Thirty consecutive patients (median age: 18.5 years; 76.7% male) were included. Twenty‐three patients had HT before the procedure and 14 (46.7%) had post‐procedural HT despite optimal endovascular treatment. Residual HT post‐stenting was associated with longer stent length and gothic arch geometry. Age and body mass index (BMI) were also associated with residual HT. The pathologic association of abnormal arch geometry and aortic stent placement may lead to a loss of aortic compliance that is further increased by high BMI and older age. Determination of a patient's aortic arch anatomy and clinical profile can assist in defining those at high risk of residual HT despite optimized isthmic stent implantation.     

A classification of cranio-facial syndromes

How to manage craniofacial malformative cases? It seems to be very difficult, especially in orthodontics because of the lack of consensus. The authors' aim is to propose a physiopathologic classification of these craniofacial syndromes in order to simplify the medical practice when we meet these patients. More than fifty cases are actually treated and followed in our hospital; we have described all of these cases before to choose the most representative in each category. These syndromes are classified in four categories, organ abnormalities of one or many functional matrix, localized abnormalities of the anatomical structures, general abnormalities of the connective tissue, mixed syndromes.     

Clinical implications of “pure” Hisian pacing in addition to para-Hisian pacing for the diagnosis of supraventricular tachycardia

BACKGROUND: Para-Hisian pacing is an effective method of differentiating between pathways for retrograde conduction over the accessory pathway (AP) and over the atrioventricular node (AVN). When performing para-Hisian pacing, the pacing spike sometimes captures only the His bundle, which we named "pure" Hisian pacing (Hc). OBJECTIVE: We evaluated the significance of pure Hisian pacing for predicting the pathways of ventriculoatrial conduction. METHODS: In 62 patients with supraventricular tachycardia, both para-Hisian and pure Hisian pacing were carried out during the sinus rhythm, resulting in three different types of electrocardiographic complexes with wide (local ventricular myocardial capture), slightly narrow (both local myocardial and His bundle capture), and very narrow QRS widths (Hc). A change of atrial activation sequence as demonstrated by these pacing modes indicated the presence of multiple retrograde pathways. The diagnosis of retrograde pathways by para-Hisian pacing with or without Hc was evaluated. RESULTS: In 22 patients with AVN reentrant tachycardia, para-Hisian pacing alone was able to correctly predict ventriculo-atrial conduction exclusively through the AVN without requiring findings from Hc. In 40 AP patients, para-Hisian pacing showed a pattern of retrograde conduction through the AVN in six, through both the AVN and AP in 10, and through an AP in 24 patients. Four of these 24 patients were diagnosed as having multiple pathways (AP+AVN or dual APs) by the addition of Hc. CONCLUSION: Pure Hisian pacing can help disclose another pathway for retrograde conduction in AP patients, which is unpredicted by ordinary para-Hisian pacing.     

Spirometry in 3-5-year-old children with asthma

Spirometry with incentive games was applied to 207 2-5-year-old preschool children (PSC) with asthma in order to refine the quality-control criteria proposed by Aurora et al. (Am J Respir Crit Care Med 2004;169:1152-159). The data set in our study was much larger compared to that in Aurora et al. (Am J Respir Crit Care Med 2004;169:1152-159), where 42 children with cystic fibrosis and 37 healthy control were studied. At least two acceptable maneuvers were obtained in 178 (86%) children. Data were focused on 3-5-year-old children (n = 171). The proportion of children achieving a larger number of thresholds for each quality-control criterion (backward-extrapolated volume (Vbe), Vbe in percent of forced vital capacity (FVC, Vbe/FVC), time-to-peak expiratory flow (time-to-PEF), and difference (Delta) between the two FVCs (DeltaFVC), forced expiratory volume in 1 sec (DeltaFEV(1)), and forced expiratory volume in 0.5 sec (DeltaFEV(0.5)) from the two "best" curves) was calculated, and cumulative plots were obtained. The optimal threshold was determined for all ages by derivative function of rate of success-threshold curves, close to the inflexion point. The following thresholds were defined for acceptability: Vbe 相似文献   

Bone involvement in generalized crystal-storing histiocytosis

The abnormal secretion of monoclonal immunoglobulins observed with monoclonal gammopathies and other clonal B cell dyscrasias can be responsible for a spectrum of deposition disorders. Crystal-storing histiocytosis (CSH) is a rare disease affecting patients with B cell dyscrasias and monoclonal gammopathies, characterized by the accumulation of histiocytes that have phagocytosed an abnormal crystalline immunoglobulin. We describe 2 cases of this rare disorder with multiorgan involvement and prominent bone involvement. Magnetic resonance imaging showed bone marrow infiltration and images of avascular necrosis. Bone specimen analysis gave histological proof of diffuse bone infiltration by the abnormal histiocytes. Bone involvement, which appears to be a specific feature of CSH, links this entity to other storage disorders, such as Gaucher disease. Because the accumulation of abnormal immunoglobulin-loaded histiocytes is clearly pivotal, CSH should be considered not only as an immunoglobulin deposition disease but also as a storage histiocytic disorder.