Cervical diastematomyelia in cervico-oculo-acoustic (Wildervanck) syndrome: MRI findings

Cervico-oculo-acoustic (COA) or Wildervanck syndrome is characterized by the triad of Klippel-Feil anomaly, bilateral abducens palsy with retracted bulbs (Duane 'syndrome') and hearing loss. The clinical findings of this syndrome have been well documented. A few case reports with MRI findings have appeared in the literature showing brainstem and cerebellar hypoplasia and vertebral segmentation anomalies. Our case is unique in that diastematomyelia of the lower medulla and cervical cord was accompanied by vermian hypoplasia, tonsillar herniation and resulting triventricular hydrocephalus in a child with Wildervanck syndrome. This case is presented with MR images. Children with Wildervanck syndrome should be investigated for craniospinal abnormalities with MR imaging.     

Neurofuzzy Classification of the Effect of Diabetes Mellitus on Carotid Artery

Here, we present a new clasification system for the effects of diabetes mellitus (DM) on carotid artery by using neurofuzzy system. Blood flow hemodynamics were obtained from 118 carotid arteries of 59 patients with DM by using 7.5 MHz transducer and Doppler-M unit. Vmax = peak systole, Vmin = end diastole, resistive index (RI), and pulsatile index (PI) were measured with the use of Doppler sonography. These values were applied to neurofuzzy system using NEFCLASS model. With the increase of epoch from 200 to 500 and pruning of fuzzy rules, our classification system was found to be successful in 85% of the cases. In 100 of 118 patients the classificitaion system was found to be correct. Our findings suggest that with the application of Doppler signal parameters from carotid arteries to neurofuzzy system may produce a new and reliable classification system for diagnosing diameter stenosis.     

Treatment of a giant orbitofacial vascular malformation and ophthalmic artery aneurysms with intralesion glue injections: case report

We describe a giant left orbital vascular malformation that was treated with both percutaneous and transarterial embolization. Feeder artery aneurysms thrombosed as a result of retrograde reflux of embolic material into the distal ophthalmic artery. In this presentation, we emphasize the efficacy of percutaneous embolization and retrograde thromboses of two intradural saccular ophthalmic artery aneurysms.     

Effect of methemoglobin formation on the MR appearance of subarachnoid hemorrhage

Subarachnoid hemorrhage has a much higher intensity in magnetic resonance (MR) images with the passage of time. Acute subarachnoid hemorrhage is difficult to see; within 1 week its appearance has become intensified on T1-weighted images. Different concentrations of blood and lysed red blood cells in cerebrospinal fluid (CSF) were examined spectroscopically but did not significantly alter T1 and T2 relaxation of CSF acutely. Ultraviolet visible spectroscopy of bloody CSF stored hypoxically for 3 days showed the presence of methemoglobin. The iron in methemoglobin is paramagnetic; in combination with water this facilitates T1 relaxation. It is concluded that methemoglobin formation with T1 shortening at least partially accounts for the increasing intensity of the MR appearance of subarachnoid hemorrhage over time in the central nervous system and may also explain the intense appearance of subacute hemorrhage in MR images elsewhere in the body.     

Haemoglobin F levels in patients with testicular tumours

This study was designed to examine haemoglobin F levels prior to therapy in 39 patients with testicular tumours (10 seminoma, 29 non-seminomatous tumours) and 20 controls. HbF levels were significantly increased in the group with testicular tumours (1.66 +/- 0.12%) compared with normal controls (0.99 +/- 0.08%) (P less than 0.001). Non-seminomatous tumours had more pronounced levels than seminomas (P less than 0.001). No correlation was found between HbF level and stage of the disease. We suggest that HbF production could be significantly reactivated in adult patients with testicular tumours and may be a useful marker.     

Left and right atrial myocardial deformation properties in patients with an atrial septal defect

Background: Atrial septal defect (ASD) is a common form of congenital heart defect in adults, which affects all cardiac chambers. Atrial myocardial function in patients with ASD has not yet been clearly elucidated. The aim of this study was to investigate atrial myocardial deformation properties in patients with ASDs. Methods: The study involved 24 patients with a secundum type ASD, and 22 healthy subjects. Color Doppler myocardial imaging was used to measure left and right atrial myocardial systolic strain and strain rate values, together with peak systolic velocity, early velocity, and late diastolic velocity. Results: There was no significant difference between the two groups with regard to age, gender, body mass index, heart rate, blood pressure, left atrial diameter, and ventricular function. The peak systolic atrial myocardial strain and strain rate values in each of the atrial walls studied were lower in the ASD group compared to those of the control group, but the difference reached statistical significance only in the case of the right atrial wall (right atrial strain: 48.0 ± 32.7% vs 100.2 ± 46.6%, P = 0.006; right atrial strain rate: 2.6 ± 1.2/sec vs 3.8 ± 1.2/sec, P = 0.024). Conclusion: The left to right cardiac shunt that results from ASD leads to a reduction in the right atrial myocardial longitudinal lengthening that occurs during ventricular ejection. These findings demonstrate that the reservoir function of the atrium is impaired and atrial stiffness increases in patients with ASDs.     

Prevention and management of food allergy

The phenotypic expression and natural history of food allergy vary widely according to the patient's age, disease presentation and type of offending food. Prevention of food allergy might be achieved by altering the dietary factors responsible for the sensitization and phenotypic expression of the disease. Owing to the peculiarity of the atopic status, a minute amount of allergens can trigger both sensitization and symptoms in atopic individuals. The oral dose of β-lactoglobulin causing sensitization can be estimated to be between 1 ng and several milligrams. In food allergy, sensitization and treatment are allergen specific; therefore, for primary prevention (avoiding sensitization) and secondary prevention of food allergy (avoiding symptoms in an already sensitized subject), a product without immunogenic and allergenic epitopes should be given in each case. Babies of atopic parents are particularly prone to develop food allergy and for this reason they are called high-risk babies. Cow's milk is the most commonly offending food in both gastrointestinal and cutaneous manifestations. Cow's milk proteins are potent allergens and around 2.5% of infants experience cow's milk allergy in the first years of life. The major risk factors for cow's milk allergy are positive family history of atopy and early exposure to cow's milk proteins. Hydrolysate formulae have been developed for the purpose of reducing the allergenicity of cow's milk proteins. More recently, partially and extensively hydrolysed formulae have also been used for feeding babies with a high risk of atopy for the prevention of cow's milk allergy. However, according to the results of a recent randomized controlled study, only an extensively hydrolysated formula, and not a partially hydrolysated formula, significantly decreased the prevalence of cow's milk allergy.     

Successful treatment of severe subcutaneous insulin resistance with inhaled insulin therapy

van Alfen‐van der Velden AAEM, Noordam C, de Galan BE, Hoorweg‐Nijman JJG, Voorhoeve PG, Westerlaken C. Successful treatment of severe subcutaneous insulin resistance with inhaled insulin therapy. The potential of inhaled insulin therapy for severe resistance to subcutaneous insulin was tested in a 7‐yr old boy with type 1 diabetes mellitus. The efficiency of 1 mg inhaled insulin (Exubera^®) was examined by a 4‐h euglycemic clamp study. During the clamp, the glucose infusion rate started to increase 25 min after inhalation and peaked 120 min after inhalation. Subsequently, a trial of inhaled insulin monotherapy was initiated consisting of pre‐meal inhalations and one inhalation during the night. Since glycemic control remained fair (HbA1c ～8.5%), this therapy was continued. Over the ensuing 18 months, mild keto‐acidosis occurred twice during gastro‐enteritis. Inhaled insulin was well tolerated and pulmonary function did not deteriorate. We conclude that severe resistance to subcutaneous insulin does not preclude sufficient absorption of insulin delivered by pulmonary.