The neurology of coeliac disease in childhood: what is the evidence? A systematic review and meta‐analysis

Aim The aim of this article was to review and conduct a meta‐analysis of the paediatric literature on the neurology of coeliac disease. Method We conducted a review of paediatric studies published in English assessing neurological illness in coeliac disease identified through a MEDLINE search (1950–2009). Calculation of computed relative risk, odds ratio, and risk difference was performed using the fixed effect method if applicable. Results Fifteen studies were analysed (11 772 participants). The meta‐analysis showed that (1) the relative risk of epilepsy in individuals with coeliac disease, and of coeliac disease in individuals with epilepsy, compared with the general population, was 2.1 and 1.7, respectively, and the risk difference was close to zero, indicating that it was probably a chance association; and (2) the relative risk of headache in individuals with the disease compared with comparison groups was 3.2. In two studies, cerebellar ataxia was documented in 2.7 to 5.4% of participants; in two further studies, the risk of cerebellar dysfunction was zero. Two studies found an association between coeliac disease and peripheral neuropathy. Brain white matter lesions were recorded in two other studies. An association between autism and coeliac disease is disputed. Interpretation Children with coeliac disease are at risk of developing neurological complications, but the risk is lower than in adulthood. The discrepancy might be due to short disease duration, early elimination of gluten from the diet, stricter adherence to diet, or different susceptibility to immune‐mediated disorders.     

Impact of anatomical pielocaliceal topography in the treatment of renal lower calyces stones with extracorporeal shock wave lithotripsy

AIM: There is wide consensus that the lowest success rate of extracorporeal shock wave lithotripsy (ESWL) is in the complete clearance of renal stones located in the lower calyces. We assess the effectiveness of extracorporeal shock wave lithotripsy monotherapy for lower pole renal calculi to determine the relationship between the spatial anatomy of lower pole and the outcome of ESWL. METHODS: We evaluated 107 patients who were treated for solitary lower pole renal stones less than 20 mm in diameter with ESWL. The spatial anatomy of the lower pole, as defined by the lower infundibulopelvic angle, infundibular length and infundibular width, was measured by preoperative intravenous pyelography, while the stone location and size were determined by using abdominal plain X-ray. All patients were followed up at 1 and 3 months with abdominal plain X-ray and ultrasonography. RESULTS: Only 62 patients (58%) became stone free, while 45 (42%) retained residual fragments. A small lower infundibulopelvic angle, a long infundibular length and a tight infundibular width are unfavorable for stone clearance after ESWL. CONCLUSIONS: ESWL is the treatment of choice for most renal and ureteral stones. However, stone clearance from the lower pole following ESWL is poor and significantly affected by the inferior pole collecting system anatomy. Therefore, we believe it is important to evaluate these anatomical factors when deciding on the best treatment for lower pole renal calculi.     

Postoperative use of rFVIIa by continuous infusion in a haemophilic boy

Continuous infusion of coagulation factor concentrates has proved to be safe and effective. Because rFVIIa (NovoSeven is a very expensive product and very frequent doses are needed, continuous infusion is expected to be highly cost-effective. The postoperative use of continuous infusion of rFVIIa in a haemophilic boy with a high titre FVIII inhibitor is reported. He presented with a large right knee haemarthrosis and was treated with intermittent doses of rFVIIa. After a transient improvement the haemarthrosis became worse and an open evacuation of the joint had to be made under treatment with bolus injections of rFVIIa for 3 days (120 microg kg(-1) every 2 h). A previous pharmacokinetic evaluation in this patient had showed that FVIIa recovery and half-life were less than expected. Continuous infusion of rFVIIa (20 microg kg(-1) h(-1)), with added low molecular heparin to prevent local thrombophlebitis, was started on the fourth postoperative day and maintained unchanged for 7 days. Four additional single bolus injections were given for early joint mobilization. The intervals between replacements of the pump syringes were progressively increased from 6 to 12 h and then up to 24 h. FVIIa plasma levels during continuous infusion ranged between 6.3 and 10.4 IU mL(-1). Although +FVIIa assays seemed to show good stability, we observed the formation of precipitates inside the syringes. The precipitates seemed to contain FVIIa. We concluded that FVIIa+ plasma levels of 6-10 IU mL(-1) were safe and effective to prevent postoperative haemorrhage in this patient. The addition of heparin to the rFVIIa concentrates, however, may cause precipitation and should be avoided. Individual pharmacokinetic evaluation may be useful to select the appropriate initial doses, especially in young patients.     

Automatic "Scanning" by Radio-frequency in the Long-Term Electrical Treatment of Arrhythmias

The use of programmed electrical stimulation in the long term treatment of re-entry tachycardia offers encouraging perspectives. Among the others proposed, the "scanning" system seems to be the most effective. However, an implantable stimulator with these features is not yet available and, thus, a temporary external lead is required. These difficulties have been overcome by utilizing radiofrequency to synchronize and stimulate. An implantable device was therefore designed which is triggered by the patient and automatically searches the interruption zone of the tachycardia by exploring the R-R cycle. The external transmitter, which can produce one or two synchronized impulses, is programmed to scan the R-R cycle with progressive steps of 5 or 10 ms; when tachycardia is interrupted, further stimulation is inhibited. The implanted module connected to an endocavitary lead does not have any power supply and, therefore, is very small. The efficacy of this method has been demonstrated in 4 patients with supraventricular tachycardia (3 with WPW syndrome) resistant to conventional pharmacologic therapy.     

Renal and Gastric Hamartomas

This report documents a patient with renal tumor which was originally diagnosed as "Wilms' tumor" and treated by irradiation and chemotherapy. Fifteen years later the patient was found to have gastric hamartomas. Reviews of the kidney tumor revealed it to be composed of morphologic features consistent with renal hamartoma rather than Wilms' tumor. We wish to report this extremely unusual association of renal and gastric hamartomas.     

EOSINOPHILIC PUSTULAR FOLLICULITIS IN PATIENTS WITH ACQUIRED IMMUNODEFICIENCY SYNDROME

Three patients with AIDS had an intensely pruritic eosinophilic pustular folliculitis associated with abundant Pityrosporum yeasts in the follicles, demonstrated by direct microscopy. The excellent response to ketoconazole treatment suggests a possible pathogenic role for Pityrosporum.     

A Time-Domain Analysis of Intracardiac Electrograms for Arrhythmia Detection

The analysis of intracardiac electrogram morphology has been proposed as a complementary method for accurate discrimination between sinus rhythm (SR), supraventricular dysrhythmias, and ventricular dysrhythmias by automatic antitachycardia and cardioverter defibrillator devices. In this study, the performance of a traditional time-domain method for surface electrocardiogram interpretation—Correlation Waveform Analysis (CWA) and a newly developed technique—Bin Area Method (BAM) were used to analyze unfiltered intraatrial and intraventricular electrograms obtained from 47 patients during routine cardiac electrophysiology studies. Nineteen patients had 31 distinct, sustained, monomorphic ventricular tachycardias (VTs) induced; 13 patients had paroxysmal bundle branch block of supraventricular origin (BBB) induced; 19 patients had retrograde atrial activation during ventricular overdrive pacing. Three patients were common to two or more groups. Using a best fit electrogram alignment, both CWA and BAM distinguished VT from SR in 28/31 cases (90%), BBB from SR in 15/15 patients (100%), and anterograde from retrograde atrial activation in 19/19 patients (100%J. We conclude that the use of time-domain techniques that are independent of amplitude and baseline fluctuations appear to be reliable for discrimination of retrograde atrial activation, paroxysmal BBB, and VT from SR using intracardiac electrograms. Reduction of computational time and power constraints, without sacrificing reliable dysrhythmia discrimination, is possible. These features may make real-time morphology analysis of intracardiac electrograms feasible for automatic antitachycardia and cardioverter-defibrillator devices.     

Non-specific airway hyperresponsiveness in mono-sensitive Sicilian patients with allergic rhinitis. Its relationship to total serum IgE levels and blood eosinophils during and out of the pollen season

Background Initial attempts to evaluate the association between allergic rhinitis and nonspecific bronchial responsiveness has produced conflicting results. In fact, some studies showed a strong correlation and other failed to find an association. However, little is known about the effect of natural specific allergen exposure on the bronchial reactivity of mono-sensitive patients with rhinitis in the southern Mediterranean area, in relation to skin reactivity to allergens, total serum IgE levels and blood eosinophiis. Objectives The significance of the association between allergic rhinitis, and abnormal airway responsiveness with regard to the pathogenesis of asthma is unclear. For this reason, we have studied non-specific bronchiai hyperreactivity. in patients with seasonal allergic rhinitis, with reference to the responsible allergen. The aim of the study was to correlate the responsiveness to bronchoprovocation with methacholine in subjects a with allergic rhinitis during and out of the pollen season with total serum IgE and blood eosinophils. Methods Fourty-nine non-smoking patients with clinical diagnosis of allergic rhinitis and mono-sensitive skinprick tests to pollen allergens were enrolled in the study. Twenty patients suffered from seasonal rhinitis to Parietaria pollen. 15 patients to Gramineae pollen and 14 patients to Olea pollen. In all patients lung function measurements (assessed as response to methacholine). tolal serum IgE and blood eosinophii counts were measured during and out of the pollen season. Results During pollen season. 16 out of 49 rhinitis patients demonstrated values of bronchial responsiveness measured as response to inhaled metbacholine in the asthmatic range whereas out of the pollen season only eight patients were in the asthmatic range. By analysing the results with reference to the responsible allergen, during the pollen season 5 out of 16 patients were Parietaria -sensitive and out of the pollen season seven out of eight patients. Finally, in Parietaria -sensitive rhinitis bronchial responsiveness signifi-cantly correlated, during and out of the pollen season, with total serum IgE and with blood eosinophil counts. Conclusions Our results are consistent with the hypothesis that Parietaria is more important than Olea and Gramineae as a risk for developing non- specific bronchial hyperresponsiveness. On the whole, present observations provide further evidence that there is an interrelationship of allergen kind, total serum IgE. eosinophil and bronchial hyperressponsiveness suggesting that they may play a role in the development of bronchial asthma in rhinitis patients.