Sorsby fundus dystrophy presenting with choroidal neovascularisation showing good response to steroid treatment.
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Biomedical subjects
Publications and source records attributed to D Louis.
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AIM: To report the outcome of topical mitomycin C (MMC) used as adjunctive treatment following primary excision of ocular surface squamous neoplasia (OSSN). METHOD: Prospective, non-comparative interventional case series of 27 primary OSSN lesions from 26 patients treated in a single ocular oncology centre over a 4 year period. RESULT: 27 cases of OSSN received a treatment regimen of surgical excision, followed by topical MMC. Mean follow up of 27 (SD 12) months (range 12-50, median 25 months) revealed zero recurrences. CONCLUSION: MMC treatment following surgical excision decreases the recurrence rate of primary ocular surface neoplasia and should be considered as adjunctive therapy in primary treatment.
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The actinomycete Frankia has never been transformed genetically. To favour the development of Frankia cloning vectors, we have fully sequenced the Frankia alni pFQ31 cryptic plasmid and performed analyses to characterise its coding and non-coding regions. This plasmid is 8551 bp-long and contains 72% G+C. Computer-assisted analyses identified 18 open reading frames (ORFs). These ORFs show a synonymous codon usage different from the one of Frankia chromosomal genes, suggesting an evolutionary bias linked to the nature of the replicon or a horizontal transfer. Three ORFs were found to encode genes likely to be involved in plasmid replication and stability: parFA (partition protein), ptrFA (transcriptional repressor of the GntR family) and repFA (initiation of replication). DNA signatures of a replication origin were identified in the ptrFA-repFA intergenic region. These structural motifs are similar to those observed among origins of iteron-containing plasmids replicating via a θ mode.
A five-month-old infant presented with a sudden choking cough and mild dyspnea. Bilateral cystic pulmonary images were found on a chest X-ray, leading to exploratory surgery. A pleuropulmonary blastoma, a rare malignant embryonic tumor of childhood, was diagnosed on histologic examination. This case illustrates the necessity of performing surgical resection and histologic evaluation of any pulmonary cystic malformations, particularly when bilateral.
PURPOSE: Cataract surgery is increasing in Australia and represents a significant burden on limited health resources. This study examines the frequency and outcomes of cataract surgery for patients who were hospitalized overnight compared with those treated as day surgery cases. METHODS: Medical records of 671 consecutive admissions for cataract surgery at the Royal Victorian Eye and Ear Hospital were reviewed. Data analysed included demographic features, insurance status, length of hospitalization, ophthalmic conditions, medical conditions, social problems and planned surgical technique. Ophthalmic and anaesthetic complications, active ophthalmic and medical interventions were also studied. RESULTS: Of the 671 patient admissions for cataract during the study period, 226 (33.4%) were hospitalized overnight. Factors significantly associated with overnight hospitalization in univariate analyses include older age, female sex, country residence, Veterans' Affairs insurance, monocular vision status, pre-existing ischaemic heart disease, pre-existing asthma/chronic obstructive lung disease, absence of carer, transportation problems, planned extra-capsular cataract extraction technique, ophthalmic complications and active ophthalmic and/or medical interventions. After adjusting for possible confounding factors using backwards stepwise multivariate logistic regression models all except pre-existing ischaemic heart disease and ophthalmic complications were significantly associated with overnight admission for cataract surgery. In total, 14 cases (2.1%) needed active ophthalmic and/or medical interventions, 13 overnight cases and one day case. CONCLUSIONS: These data suggest that many patients who are hospitalized overnight for cataract surgery could be safely treated as day cases. Such a shift in the pattern of care for cataract surgery could provide a significant potential for health care savings.
We report 2 cases of multifocal cystic (type 1) pleuropulmonary blastoma, diagnosed during the first 6 months of life. This rare entity must be recognized before evolution into the prognostically unfavorable type 2 or type 3 pleuropulmonary blastoma.
The use of a peptide library of limited size, is considered to be more appropriate for studying a protease with a complex specificity, but very sensitive and efficient analytical techniques must be used. We have designed and synthesized a 49-peptide library of the type Z-AlaXXAla(amide) (X=Ala, Leu, Val, Phe, Ser, Arg, Glu) for studying the Pseudomonas aeruginosa serralysin specificity. All compounds of the peptide library could be identified by a LC-MS procedure. After hydrolysis of the library by pseudomonal serralysin, the LC-MS procedure also allowed the identification of the hydrolysis products and the different cleavage sites of the substrates.
The Pseudomonas aeruginosa serralysin (E.C. 3.4.24.40.), which is a zinc-dependent metalloprotease from the metzincin superfamily, has quite a broad specificity, which has not yet been clearly identified. We have studied it with an original approach, using a 49-peptide library of the type Z-AXXA (amide) (X = A, L, V, F, S, R, E). The library was analyzed by LC-MS before and after enzymatic hydrolysis. A great number of hydrolyzed peptides were screened and the preferential hydrolysis was the X-X peptide bond, even if in some cases, A-X and X-A bond could be hydrolyzed. No amino acids with a ionized side chain could be found in the P1' position. The results obtained suggest that the specificity in the P1' position, where an hydrophobic residue was preferentially found, seems more selective that in the Pn position. The P1 position was not very specific, but, on a quantitative point of view, the enzymatic activity was particularly increased when R, F or A were in this position. The results allow us to define the P1' and P1 residues for an optimal substrate of pseudomonal serralysin and usable for the design and the synthesis of a specific inhibitor.
The characterization of the specificity of alkaline protease from Pseudomonas aeruginosa has not yet been clearly defined. Some previous results suggested that its specificity was influenced more by amino acids far from the hydrolyzed peptide bond, than by amino acids in P1 or P'1 position. From other data, it was a C-(COOH)-type endoprotease where the preferential amino acid in P1 position was an arginine residue. We have studied the hydrolysis of several biologically active peptides. Many various sites of cleavage have been characterized but no arginine in P1 position was found, despite the presence of arginine in the peptide sequence. In fact P1 and P'1 position could be occupied by various amino acids. It seems unlikely that Pseudomonas alkaline protease may only be considered as a protease specific to arginine in P1 position. On the other hand, we have observed that increase of the peptide chain length led to an important increase of the hydrolysis rate, suggesting an extended number of subsites.
Staphylococcus aureus causes chronic respiratory tract infections in patients with cystic fibrosis (CF). Using immunofluorescence and scanning and transmission electron microscopy we located S. aureus in lung specimens of three infected CF patients, in a nasal polyp of one CF patient, and in a suspension cell culture system of primary nasal epithelial cells in vitro. Very little of S. aureus was attached to the lung epithelium, whereas abundant S. aureus was detectable in the mucus of obstructed airways. Similarly, S. aureus adhered to components of secreted mucus on primary nasal epithelial cells of CF patients and healthy control subjects, grown as cell balls in vitro (bacteria/cell +/- SD: CF: 21.9 +/- 1.5; controls: 22. 0 +/- 5.8). Mucus depletion of cell balls prior to incubation with S. aureus resulted in a significantly reduced binding (bacteria/cell +/- SD: CF: 4.2 +/- 0.3; P < 0.001; controls: 5.0 +/- 1.3; P < 0. 007). Binding of S. aureus to cell balls from CF patients or control subjects did not differ significantly. When cell balls were treated with human neutrophil elastase, hypersecretion caused removal of S. aureus from cell-associated mucus. The results suggest that S. aureus adheres primarily to mucus components of the respiratory epithelium and that significant differences do not exist in binding of S. aureus to CF or non-CF cells.
Alkaline protease from Pseudomonas aeruginosa (E.C. 3.4.24.40), also called pseudomonal serralysin, plays an important role in the infection bacterial process. The alkaline protease concentration and activity in the culture supernatants of 23 Pseudomonas aeruginosa strains from patients suffering from cystic fibrosis were determined by using an ELISA procedure and a specific enzymatic activity test. No correlation was demonstrated between the activity and the concentration of alkaline protease. This could be explained by the presence of an inactive precursor and a partial hydrolysis of the enzyme, confirmed by SDS-PAGE and Western blotting in the supernatants. The activity test is the only one allowing a quantification of the presence of an active form of alkaline protease.
The fortuitous discovery of a left posterior cervico-mediastinal mass during hospitalization for hemolytic anemia in an 18 month old baby is reported. Metaiodobenzylguanidine (MIBG) uptake suggested a sympathetic tissue origin of the mass despite negative catecholamines excretion, but histopathologic examination revealed a juvenile capillary angioma within a normal sympathic ganglion.
Thirty Pseudomonas aeruginosa strains were isolated from the sputa of cystic fibrosis patients. In each culture supernatant, the amount of three exoproteases (LasA, alkaline protease and elastase) was determined using immunochemical procedures. These assays used selected peptide-MAP (multiple antigen peptide) strategy as antigen for animal immunisation. The method appeared to be reproducible, simple, sensitive and specific without cross-reactivity between the antisera. The resulting values differed from one strain to another mostly for elastase production. Despite the fact that four genes (lasA, lasB, lasR and rhlR) were shown to be necessary for full elastolytic activity, it was obvious that if LasA was not secreted in a naturally non-elastase-producing strain, in return in an elastase-producing strain, there were no apparent relationships between LasA and elastase production and between LasA and alkaline protease secretion. Furthermore, in vitro, the secretion of the three exoproteases seemed to be independent of the mucoid or non-mucoid phenotype of the bacteria.
BACKGROUND: Late-recognized congenital left diaphragmatic hernia may have a bad prognosis when it is revealed by perforation or volvulus of the herniated viscera. CASE REPORT: A 20 month-old boy was admitted for vomiting and abdominal pain. X-rays showed left diaphragmatic hernia and the patient was operated on with the diagnosis of volvulus of the stomach. Surgery showed hernia through the posterolateral segments of the diaphragm with spleen volvulus into the sac, requiring splenectomy before reduction of the hernia and closure of the diaphragmatic defect. CONCLUSION: This is the fifth report of ectopic intrathoracic spleen with volvulus revealing congenital left diaphragmatic hernia.
We report two cases of inhalation of seeds in infants aged 11 months and two years. In the first the syndrome of inhalation had not been apreciated. The seed blocked the left bronchus, provoking an obstructive emphysema with superinfection. The outcome was statisfactory after removal by bronchoscopy. In the second case although the aspiration of the particle was appreciated the endoscopy was performed too late to prevent the migration of the seed. After having produced a pneumonia it crossed the pleura and gave a subcutaneous swelling. This migration could in part be followed by ultrasound which enabled the appropriate moment for surgical exploration to be defined. These two cases show the two possible evolutions when the inhalation of a seed occurs and the contribution that can be made by ultrasound.
BACKGROUND: Early diagnosis of superior mesenteric artery syndrome, less frequent in children than in young adults, is improved by the use of ultrasonography and CT scan. CASE REPORT: An eleven year-old boy was admitted because he suffered from bilious vomiting for 24 hours. A diagnosis of superior mesenteric artery syndrome was made from the results of plain films of the abdomen, ultrasonography and roentgenograms after instillation of dilute barium solution. Diameter of the left renal vein appeared increased. The patient was given fractionated meals and placed in left lateral position. The CT scan performed 5 days later confirmed the diagnosis but did not show dilatation of the left renal vein. The patient is well one year later without treatment. Ultrasonography performed 3 hours after a meal showed similar findings to those seen in the acute phase. CONCLUSION: Abdominal scanning and ultrasound are valuable tools for diagnosis of such a syndrome. The diagnostic value of dilatation of the left renal vein remains to be determined.