Imaging of bronchial pathology in antibody deficiency: Data from the European Chest CT Group

Studies of chest computed tomography (CT) in patients with primary antibody deficiency syndromes (ADS) suggest a broad range of bronchial pathology. However, there are as yet no multicentre studies to assess the variety of bronchial pathology in this patient group. One of the underlying reasons is the lack of a consensus methodology, a prerequisite to jointly document chest CT findings. We aimed to establish an international platform for the evaluation of bronchial pathology as assessed by chest CT and to describe the range of bronchial pathologies in patients with antibody deficiency. Ffteen immunodeficiency centres from 9 countries evaluated chest CT scans of patients with ADS using a predefined list of potential findings including an extent score for bronchiectasis. Data of 282 patients with ADS were collected. Patients with common variable immunodeficiency disorders (CVID) comprised the largest subgroup (232 patients, 82.3%). Eighty percent of CVID patients had radiological evidence of bronchial pathology including bronchiectasis in 61%, bronchial wall thickening in 44% and mucus plugging in 29%. Bronchiectasis was detected in 44% of CVID patients aged less than 20 years. Cough was a better predictor for bronchiectasis than spirometry values. Delay of diagnosis as well as duration of disease correlated positively with presence of bronchiectasis. The use of consensus diagnostic criteria and a pre-defined list of bronchial pathologies allows for comparison of chest CT data in multicentre studies. Our data suggest a high prevalence of bronchial pathology in CVID due to late diagnosis or duration of disease

Selected Predictors Of Apoptosis In Retinitis Pigmentosa

The genetics of non syndromic retinitis pigmentosa (RP) is complex with numerous gene mutations. An attempt to overcome each individual mutation provides an overwhelming challenge. However targeting apoptosis which represents a final common pathway to photoreceptor cell death may provide a more practical approach. This study focused on some predictors of apoptosis in RP and their potential usefulness for patients\' management and relatives\' early diagnosis. Forty nine RP patients with thirty controls were evaluated genetically and ophthalmologicaly with assessment of plasma total nitrite and nitrate (as an index for nitric oxide), Plasma sFas as an index of apoptosis and plasma fatty acids levels. Autosomal recessive RP was the most common type of inheritance and the levels of plasma sFas and nitric oxide (NO) were significantly higher in retinitis pigmentosa compared to controls. Retinitis pigmentosa patients had significantly lower percentage of plasma omega3 fatty acids especially docosahexaenoic acid (DHA) relative to controls. sFas, NO, and DHA could differentiate between RP patients and control subjects with 100%, 100%, 97% sensitivity and 90%, 90%, 100% specificity respectively. sFas and nitric oxide levels were higher in cases of autosomal recessive (AR) type followed by X-linked, autosomal dominant, then simplex cases relative to the control group this may explain why AR and X-Linked forms are clinically more severe. In conclusion; diagnosis and treatment of RP could be aided by systemic markers or predictors of retinal degeneration. The consistent decrease in the plasma omega 3 fatty acids especially DHA, and increase sFas and nitric oxide levels may draw the attention upon the use of these markers as laboratory tests for relatives of affected patients who are at high risk for having retinitis pigmentosa. Also, omega 3 fatty acids in the form of DHA were recommended as possible supplements for the patients and their relatives. Keywords: Retinitis pigmentosa, Apoptosis, Plasma sFas, Docosahexaenoic acid (DHA). Egyptian Journal of Biochemistry and Molecular Biology Vol. 26 (2) 2008: pp. 119-13

Longterm outcome of Macroplatique injection for treatment of vesicoureteral reflux in children

Background: This study examined our experience with one year follow-up of 20 cases of vesicoureteric reflux in children after treatment with Macroplastique® injection.Patient and Methods: A total of 20 children (31 ureters) with primary grades II to V vesicoureteral reflux were treated with subureteral Macroplastique® injection from 2010 to 2011 and followed for an average of 12 months (range 3 to 24). Vesicoureteral reflux was grade II in 3, III in 7, IV in 9 and V in 12 ureters. Each child underwent pre-operative voidingcystourethrography, abdominopelvic ultrasound, urine analysis/culture, S. creatinine and CBC. Dimercapto-succinic acid scan (DMSA scan) and magnetic resonance urography (MRU) were done in some patients. Voiding cystourethrography at 3 months was done to rule out persistent reflux. Results: Overall, reflux was corrected in 11 (35.5%) ureters and 9 (45%) children after a single injection. With repeat injection, reflux was corrected in 16 (51.6%) ureters and 11 (55%) children, reflux improved/downgrade in 4 (12.9%) ureters and 2 (10%) children. Correction by grade was 100%, 100%, 9.7%, 9.7% for grades II to V, respectively. There were no surgical complications. None of the cured patients had recurrent reflux during follow-up. There were 9 (45%) children who required open ureteral  re-implantation for failed injection.Conclusion: Sub-ureteral Macroplastique® injection therapy could be aprimary treatment for low grade VUR (grade III or less) in children because it is simple, safe, effective, less invasive, decreased.Key words: Children, injection, Macroplastique®, polydimethylsiloxane, vesico-ureteral reflu

Assessing Differences in the Quality Properties and Ultrastructure of Eggshell as Affected by Chicken Strain and Flock Age During Incubation Period

ABSTRACT Three hundred hatching eggs from two layer breeder flocks (Hy-Line Brown and hy-Line W-36; 150 each) were obtained at three different periods of laying cycle (early, middle and late stages). The hatching eggs were randomly taken to assess the impact of chicken strain, age, and hatching time on some eggshell measurements and structures. Results indicate that egg weight, shell percentage and shell thickness were significantly affected by strain, breeder age, and incubation time. Increasing rate of calcium absorption and utilization by the embryos during the embryonic stage clearly reflects the decrease occurred in eggshell strength. The differences of eggshell breaking strength during incubation time periods were 0.41 and 0.55 kg/cm2, the differences due to the incubation time may be due to the higher demand for calcium utilization in the late incubation period (18 days), observed on the10th day of incubation. Brown eggs recorded significant higher calcium percentage in eggshell compared to the white ones. The brown breeder hens recorded significant higher total pores per egg compared to the white egg. Concerning eggshell ultrastructure, total thickness of the brown eggshell was significantly higher than those of the white eggshell by about 3.7%. All parameters, except for total thickness, were significantly affected by the interaction between strain and breeder age. Finally, we conclude that some traits including egg weight, shell percentage, shell thickness, calcium percentages in eggshell, and total pores per egg were significantly affected by strain, breeder age, and incubation time. Eggshell ultrastructure of brown egg was better compared to the white ones