Pulmonary manifestations in a group of patients with Behcet's disease.

AIM: In this study we investigated the frequency and characteristics of pulmonary manifestations in a group of patients with Behcet's disease (BD) who were admitted to Cairo University Hospital. METHODS: Fifteen patients were included in our study, 14 men (93.3%) and one woman (6.66%).Their mean age was 30.06 ± 9.8 years and the mean age of onset of BD was 23.7 ± 5.54 years. All patients were subjected to full history taking, clinical examination, plain chest X-ray and helical computed tomography (CT) study of the chest. RESULTS: Pulmonary involvements were detected in 11 patients with BD, 73.3% of cases: 10 men (90.9%) and one woman (9.09%).Their mean age was 28.8 ± 8.07, the mean age of onset of BD was 23.2 ± 5.59 years and the mean disease duration until lung manifestations appear was 3.7 ± 4.8 years. The main pulmonary and constitutional symptoms in these 11 patients were as follows: dyspnea 81.8%, cough 63.6%, weight loss 63.6%, chest tightness 54.5%, hemoptysis 45%, massive hemoptysis 27.2%, fever 36.3% and expectoration 36.3. Analysis of both vascular and parenchymal lung lesions in helical CT scan in the 11 patients with BD were as follows: pulmonary artery aneurysm (PAA) occurred in 5/11 patients (45.4%), pulmonary nodules occurred in 3/11 patients (27.2%), pleural effusion occurred in 3/11 patients (27.2%), pulmonary embolism and infarction occurred in 1/11 patients (9.09%) and pneumonitis occurred in 1/11 patients (9.09%). CONCLUSION: The higher frequency of pulmonary manifestations in our patients (73.3%) and the higher frequency of PAA (33.3%) could be related to the fact that this study was conducted on a group of patients who were admitted to the hospital with more severe illnesses.

Pathogenesis of renal injury in the megabladder mouse: a genetic model of congenital obstructive nephropathy.

Congenital obstructive nephropathy (CON) is the most common cause of chronic renal failure in children often leading to end-stage renal disease. The megabladder (mgb) mouse exhibits signs of urinary tract obstruction in utero resulting in the development of hydroureteronephrosis and progressive renal failure after birth. This study examined the development of progressive renal injury in homozygous mgb mice (mgb-/-). Renal ultrasound was used to stratify the disease state of mgb-/- mice, whereas surgical rescue was performed using vesicostomy. The progression of renal injury was characterized using a series of pathogenic markers including alpha smooth muscle isoactin (α-SMA), TGF-ß1, connective tissue growth factor (CTGF), E-cadherin, F4/80, Wilm's tumor (WT)-1, and paired box gene (Pax) 2. This analysis indicated that mgb-/- mice are born with pathologic changes in kidney development that progressively worsen in direct correlation with the severity of hydronephrosis. The initiation and pattern of fibrotic development observed in mgb-/- kidneys appeared distinctive from previous animal models of obstruction. These observations suggest that the mgb mouse represents a unique small animal model for the study of CON.

Effective carbon partitioning driven by exotic phloem-specific regulatory elements fused to the Arabidopsis thaliana AtSUC2 sucrose-proton symporter gene.

BACKGROUND: AtSUC2 (At1g22710) from Arabidopsis thaliana encodes a phloem-localized sucrose/proton symporter required for efficient photoassimilate transport from source tissues to sink tissues. AtSUC2 plays a key role in coordinating the demands of sink tissues with the output capacity of source leaves, and in maintaining phloem hydrostatic pressure during changes in plant-water balance. Expression and activity are regulated, both positively and negatively, by developmental (sink to source transition) and environmental cues, including light, diurnal changes, photoassimilate levels, turgor pressure, drought and osmotic stress, and hormones. RESULTS: To assess the importance of this regulation to whole-plant growth and carbon partitioning, AtSUC2 cDNA was expressed from two exotic, phloem-specific promoters in a mutant background debilitated for AtSUC2 function. The first was a promoter element from Commelina Yellow Mottle Virus (CoYMV), and the second was the rolC promoter from Agrobacterium rhizogenes. CoYMVp::AtSUC2 cDNA restored growth and carbon partitioning to near wild-type levels, whereas plants harboring rolCp::AtSUC2 cDNA showed only partial complementation. CONCLUSION: Expressing AtSUC2 cDNA from exotic, phloem-specific promoters argues that strong, phloem-localized expression is sufficient for efficient transport. Expressing AtSUC2 from promoters that foster efficient phloem transport but are subject to regulatory cascades different from the endogenous sucrose/proton symporter genes has implications for biotechnology.

Functional characterization of the Arabidopsis AtSUC2 Sucrose/H+ symporter by tissue-specific complementation reveals an essential role in phloem loading but not in long-distance transport.

AtSUC2 (At1g22710) encodes a phloem-localized sucrose (Suc)/H(+) symporter necessary for efficient Suc transport from source tissues to sink tissues in Arabidopsis (Arabidopsis thaliana). AtSUC2 is highly expressed in the collection phloem of mature leaves, and its function in phloem loading is well established. AtSUC2, however, is also expressed strongly in the transport phloem, where its role is more ambiguous, and it has been implicated in mediating both efflux and retrieval to and from flanking tissues via the apoplast. To characterize the role of AtSUC2 in controlling carbon partitioning along the phloem path, AtSUC2 cDNA was expressed from tissue-specific promoters in an Atsuc2 mutant background. Suc transport in this mutant is highly compromised, as indicated by stunted growth and the accumulation of large quantities of sugar and starch in vegetative tissues. Expression of AtSUC2 cDNA from the 2-kb AtSUC2 promoter was sufficient to restore growth and carbon partitioning to nearly wild-type levels. The GALACTINOL SYNTHASE promoter of Cucumis melo (CmGAS1p) confers expression only in the minor veins of mature leaves, not in the transport phloem of larger leaf veins and stems. Mutant plants expressing AtSUC2 cDNA from CmGAS1p had intermediate growth and accumulated sugar and starch, but otherwise they had normal morphology. These characteristics support a role for AtSUC2 in retrieval but not efflux along the transport phloem and show that the only vital function of AtSUC2 in photoassimilate distribution is phloem loading. In addition, Atsuc2 mutant plants, although debilitated, do grow, and AtSUC2-independent modes of phloem transport are discussed, including an entirely symplastic pathway from mesophyll cells to sink tissues.

Cysteine proteases XCP1 and XCP2 aid micro-autolysis within the intact central vacuole during xylogenesis in Arabidopsis roots.

Establishing the mechanisms regulating the autolysis of xylem tracheary elements (TEs) is important for understanding this programmed cell death process. These data demonstrate that two paralogous Arabidopsis thaliana proteases, XYLEM CYSTEINE PROTEASE1 (XCP1) and XCP2, participated in micro-autolysis within the intact central vacuole before mega-autolysis was initiated by tonoplast implosion. The data acquisition was aided by the predictable pattern of seedling root xylogenesis, the availability of single and double total knock-out T-DNA lines, anti-sera that recognized XCP1 and XCP2, and the microwave-assisted processing of whole seedlings prior to immunolabeling and observation in the transmission electron microscope. During secondary wall thickening, XCP1 and XCP2 (in wild type), XCP1 (in xcp2 seedlings) or XCP2 (in xcp1 seedlings) were imported into the TE central vacuole. Both XCP1 and XCP2 heavily labeled dense aggregates of material within the vacuole. However, because of XCP1 deficiency in xcp1 and xcp1 xcp2 TEs, non-degraded cellular remnants first accumulated in the vacuole and then persisted in the TE lumen (longer than in the wild type) after the final mega-autolysis was otherwise complete. This delayed TE clearing phenotype in xcp1 was rescued by complementation with wild-type XCP1. Although TEs in the xcp2 single knock-out cleared comparably with wild type, the non-degraded remnants in xcp1 xcp2 TEs were more densely packed than in xcp1 TEs. Therefore, XCP2 has a minor but distinct role in micro-autolysis. After tonoplast implosion, XCP1 and XCP2 remained associated with disintegrating cellular material as mega-autolysis, aided by additional lytic enzymes, destroyed the bulk of the cellular contents.

Transcriptional profiling of the megabladder mouse: a unique model of bladder dysmorphogenesis.

Recent studies in our lab identified a mutant mouse model of obstructive nephropathy designated mgb for megabladder. Homozygotic mgb mice (mgb-/-) develop lower urinary tract obstruction in utero due to a lack of bladder smooth muscle differentiation. This defect is the result of a random transgene insertion/translocation into chromosomes 11 and 16. Transcriptional profiling identified a significantly over-expressed cluster of gene products located on the translocated fragment of chromosome 16 including urotensin II-related peptide (Urp), which was shown to be preferentially over-expressed in developing mgb-/- bladders. Pathway analysis of mgb microarray data indicated dysregulation of at least 60 gene products associated with smooth muscle development. In conclusion, the results of this study indicate that the molecular pathways controlling normal smooth muscle development are severely altered in mgb-/- bladders, and provide the first evidence that Urp may play a critical role in bladder smooth muscle development.

Structured management and counseling for patients with a complaint of a small penis.

INTRODUCTION: Penile augmentation surgery has become increasingly common though there is no consensus about the management strategy for men with a complaint of small penis. AIM: To introduce and evaluate the outcome of a structured management and counseling protocol for patients with a complaint of a small-sized penis. METHODS: A structured protocol for consultation and management of (physically normal) patients with a complaint of a small penis through a descriptive study comprised of a series of 250 patients. MAIN OUTCOME MEASURES: Percentage of patients who elect to undergo penile augmentation surgery. RESULTS: Only nine patients (3.6%) chose to seek further surgical intervention. Two had a buried penis, two had true micropenis and five had normal penile size. CONCLUSIONS: Using a structured management and counseling protocol, most men chose not to undergo penile augmentation surgery, even when offered for free.