bluuebunny/crossref_metadata_2025_split huggingface dataset:
External ophthalmomyiasis caused by Dermatobia hominis. A retrospective study of nine cases and a review of the literature
Authors: Denion Eric , Dalens Pierre‐Henri , Couppié Pierre , Aznar Christine , Sainte‐Marie Dominique , Carme Bernard , Petitbon Jean , Pradinaud Roger , Gérard Max 
Published: September 2004

Abstract.Purpose:  To report nine cases of external ophthalmomyiasis caused by Dermatobia hominis. Methods:  Retrospective, non‐comparative, interventional case series. Participants consisted of patients (n = 9) presenting at Cayenne Hospital between 1968 and 2003. The location and number of larvae, the larval stage, and the medical and surgical procedures applied were studied in each case. Results:  Seven patients had palpebral myiasis (including one with three larvae) and two had conjunctival myiasis. Every patient had palpebral oedema. The larval respiratory pore was located on the palpebral skin or free margin or on the conjunctiva. Movements were present within the lesion in at least three patients. Petroleum ointment or ivermectine solution was used in at least four patients to smother or kill the larvae. Extraction under local anaesthesia was possible in six patients, while three required general anaesthesia. Conclusion:  Several larvae may be present in a patient. Topical ivermectine may help to kill the larvae before extraction is attempted.
An objective method to define outlier optical coherence tomograms and repeatability of retinal nerve fibre layer measurements
Authors: Bergamin Oliver , Anderson Susan C. , Kardon Randy H. 
Published: September 2004

Abstract.Purpose:  To compare the variation in thickness and reflectivity of the retinal nerve fibre layer (RNFL), circumferentially and on repeated testing. Methods:  In 24 normal eyes and 38 eyes with different optic neuropathies or retinopathies, third‐party optical coherence tomography (OCT) software defined the RNFL border based on the reflectivity pattern across the retina and also provided measurements of the mean reflectivity and total retinal thickness in addition to RNFL thickness. A new Gaussian filtering method was implemented so that the weight of the applied filter was varied for each of five individually repeated scans until an optimum weight filter was determined. Results:  The scans requiring the highest weight filter could be identified as ‘outlier’ scans that contained measurement or alignment artifacts. There was no difference in the weights of filtering needed for normal and abnormal eyes. The RNFL thickness and reflectivity, and retinal thickness were highly correlated with one another in normal and abnormal eyes. Conclusions:  A new Gaussian filtering routine was devised that not only defined the most reproducible substructure of the RNFL for a given patient's eye, but also provided a new method of quantifying measurement variability and identification of scans with measurement or alignment artifacts.
Unilateral diabetic macular oedema secondary to central retinal vein congestion
Authors: Christoffersen Nynne , Larsen Michael 
Published: September 2004

Abstract.Purpose:  To describe two cases of strictly unilateral diabetic retinopathy with macular edema where the precipitating factor appears to have been retinal venous congestion. Methods:  Retrospective interventional case study. Results:  Examination of fundus photographic records demonstrated generalized venous dilation in the affected eyes years in advance of the development of unilateral diabetic macular edema. Relentless progression of edema and visual loss followed despite retinal photocoagulation treatment and ultimately the affected eyes in both patients developed central retinal vein occlusion. The fellow eyes in both patients remained without retinopathy throughout the period of observation. Conclusion:  The sequential and strictly unilateral appearance of retinal venous dilation, diabetic macular edema, and central retinal vein occlusion in diabetic patients without fellow‐eye retinopathy suggests that venous congestion induced the progression from hyperglycemic insult to vascular injury in the affected eyes. Presumably, diabetes may cause retinal vascular dysfunction that results in little or no vascular damage unless venous congestion is present. This supports that venous congestion and increased sensitivity to congestion are important components of the pathogenesis of diabetic macular edema.
Enhanced S‐cone syndrome in a Japanese family with a nonsense NR2E3 mutation (Q350X)
Authors: Nakamura Yosuke , Hayashi Takaaki , Kozaki Kenichi , Kubo Akiko , Omoto Satoshi , Watanabe Akira , Toda Kazushige , Takeuchi Tomokazu , Gekka Tamaki , Kitahara Kenji 
Published: September 2004

Abstract.Purpose:  To describe the clinical characteristics of a Japanese male patient with enhanced S‐cone syndrome (ESCS) and investigate the existence of mutations in the NR2E3 gene, which encodes a photoreceptor cell‐specific nuclear receptor. Methods:  Fundus examinations, fluorescein angiography, colour vision tests, spectral sensitivity, and full‐field and spectral electroretinography were performed. Mutation screening of the NR2E3 gene was performed with polymerase chain reaction (PCR) amplification and direct sequencing. Results:  We identified a novel homozygous mutation (c.1048C > T), that converts glutamine (CAA) to a termination codon (TAA) at amino acid position 350. The subject's unaffected parents were heterozygous for the mutation, consistent with autosomal recessive transmission. The electroretinographic findings revealed that the patient had neither rod nor 30‐Hz flicker responses but did have cone responses with large a‐wave and low b‐wave amplitudes, similar to the rod‐plus‐cone responses, and also substantial short wavelength‐sensitive (S) cone and extremely diminished long/middle wavelength‐sensitive (L/M) cone responses. In the right eye, spectral sensitivity in the fovea revealed both functional S‐cone and remarkably reduced L‐ and M‐cone sensitivities, which was compatible with the decreased visual acuity (VA) and red/green colour vision defects noted in this eye. In contrast, the patient had good VA and normal red/green colour vision in the left eye. Conclusion:  The nonsense mutation results in a truncated NR2E3 protein lacking 61 amino acids within the ligand‐binding domain (LBD) that consists of 190 amino acids of the C‐terminus end. Therefore, null function of the LBD is likely to cause ESCS in the patient. The clinical findings for this patient suggest that his left eye, with its functional L/M‐ and S‐cones, was at an earlier stage of the syndrome than his right eye.
Have choroidal and ciliary body melanomas changed during the period 1955–2000?
Authors: Isager Peter , Ehlers Niels , Overgaard Jens 
Published: September 2004

Abstract.Purpose:  To establish whether the presentation of choroidal and ciliary body melanomas and patient survival have changed during the period 1955–2000. Method:  The study included 209 unselected patients (97 men, 112 women) from Århus County during the period 1955–2000. The median age at treatment was 64 years (range 26–88 years). The disease course from first symptom through clinical and histopathological findings to death was described in a historical follow‐up design. Time trends were evaluated. Results:  Treatment modality changed over time from enucleation to an almost equal distribution between enucleation and radiation brachytherapy. The age at treatment, or, alternatively, at diagnosis, increased significantly (p = 0.04). No significant time trend was seen for largest basal tumour diameter (p = 0.08) or tumour height (p = 0.27). The clinical and histopathological findings did not show any consistent time trends. No significant time trend was found for melanoma‐specific survival (p = 0.96) or overall survival (p = 0.33). Conclusion:  No consistent time trends were seen in clinical and histopathological tumour presentation and survival in patients with choroidal or ciliary body melanomas. The increase in age at treatment was in line with an increase in life expectancy in Denmark.
Excessive maternal caffeine exposure during pregnancy is cataractogenic for neonatal crystalline lenses in rats: a biomicroscopic and histopathologic study
Authors: Evereklioglu Cem , Güldür Emin , Alasehirli Belgin , Cengiz Beyhan , Sari Ibrahim , Pirbudak Lütfiye 
Published: September 2004

Abstract.Purpose:  To investigate histologically the influence of maternal caffeine exposure during pregnancy in vivo on crystalline lenses in neonatal rats. Methods:  Experimentally naive, female Wistar‐albino rats (200–220 g) were mated with adult male rats over 2 days for copulation. After confirming pregnancy with a vaginal smear method, 50 gravid rats (dams) were randomly divided into five groups (n = 10 in each), consisting of one control and four experimental groups. Groups 1, 2 and 3 experimental dams were treated with intraperitoneal (i.p.) caffeine at doses of 25, 50 and 100 mg/kg/day, respectively, during pregnancy from gestational day 9 through to day 21. Group 4 dams were treated with caffeine in distilled water in a gavage at a dose of 50 mg/kg/day. Group 5 control dams were given i.p. saline solution daily for the same period. After normal delivery, the eyes were examined by slit‐lamp biomicroscopy. The neonates were then killed by decapitation at postnatal days 1 or 30 and the eyes removed for histopathologic investigation of the lenses. Results:  Group 1 and control eyes had normal anterior lens capsules with a single layer of anterior cuboidal epithelial cells, regularly oriented cortical and nuclear lens fibres, and a clear posterior lens capsule with no lining epithelial cells behind the equator. In the remaining groups, histopathologic findings suggesting cataractogenesis included eosinophilic degeneration, lens fibre cell swelling and liquefaction, central lens fibres with retained nuclei, and prominent epithelial cells lining the posterior lens capsule behind the equator. Moreover, some lenses in group 3 had immature cataract on slit‐lamp biomicroscopic examination at postnatal day 30. Conclusion:  Excessive maternal caffeine exposure during pregnancy had cataractogenic effects on developing crystalline lenses in newborn rat eyes, both macroscopically and histopathologically. If an appropriate dose of caffeine can be identified, caffeine‐induced cataract formation may be used as a new experimental cataract model in animal studies.
Pneumocephalus and presumed meningitis following inconspicuous penetrating periocular trauma
Authors: Detorakis Efstathios T. , Drositis Ioannis , Drakonaki Eleni E. , Panayotaki Evangelia , Deville Jaime G. 
Published: September 2004

Abstract.Purpose:  To report a case of serious intracranial complications in an adolescent youth following a seemingly trivial periocular injury. Methods:  An adolescent youth was examined and discharged after a small penetrating injury to his left medial canthus. He later presented with blurred vision, nausea and mild pyrexia and underwent a computed tomography (CT) scan of the head and orbits as well as a lumbar puncture. Results:  The CT scan revealed a fracture in the cribriform plate of the ethmoid bone and the medial orbital wall, as well as pneumocephalus. The lumbar puncture revealed 3000 white cells/mm3 in the cerebrospinal fluid (CSF). After treatment with meningitic doses of intravenous antibiotics, a significant improvement was noted. Conclusions:  A CT scan, instead of an ordinary head radiograph, may be considered as a first‐choice mode of diagnosis in evaluating even inconspicuous penetrating periocular wounds. Early administration of meningitic doses of antibiotics may significantly improve prognosis.
No major association between TGFBR1*6A and prostate cancer
Authors: Kaklamani Virginia , Baddi Lisa , Rosman Diana , Liu Junjian , Ellis Nathan , Oddoux Carole , Ostrer Harry , Chen Yu , Ahsan Habibul , Offit Kenneth , Pasche Boris 
Published: September 2004

AbstractProstate cancer is the most commonly diagnosed cancer in men and one of the leading causes of cancer deaths. There is strong genetic evidence indicating that a large proportion of prostate cancers are caused by heritable factors but the search for prostate cancer susceptibility genes has thus far remained elusive. TGFBR1*6A, a common hypomorphic variant of the type I Transforming Growth Factor Beta receptor, is emerging as a tumor susceptibility allele that predisposes to the development of breast, colon and ovarian cancer. The association with prostate cancer has not yet been explored. A total of 907 cases and controls from New York City were genotyped to test the hypothesis that TGFBR1*6A may contribute to the development of prostate cancer. TGFBR1*6A allelic frequency among cases (0.086) was slightly higher than among controls (0.080) but the differences in TGFBR1*6A genotype distribution between cases and controls did not reach statistical significance (p = 0.67). Our data suggest that TGFBR1*6A does not contribute to the development of prostate cancer.
Inter-Platform comparability of microarrays in acute lymphoblastic leukemia
Authors: Mitchell Stephanie A , Brown Kevin M , Henry Michael M , Mintz Michelle , Catchpoole Daniel , LaFleur Bonnie , Stephan Dietrich A 
Published: September 2004

Abstract
Background
Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy and has been the poster-child for improved therapeutics in cancer, with life time disease-free survival (LTDFS) rates improving from <10% in 1970 to >80% today. There are numerous known genetic prognostic variables in ALL, which include T cell ALL, the hyperdiploid karyotype and the translocations: t(12;21)[TEL-AML1], t(4;11)[MLL-AF4], t(9;22)[BCR-ABL], and t(1;19)[E2A-PBX]. ALL has been studied at the molecular level through expression profiling resulting in un-validated expression correlates of these prognostic indices. To date, the great wealth of expression data, which has been generated in disparate institutions, representing an extremely large cohort of samples has not been combined to validate any of these analyses. The majority of this data has been generated on the Affymetrix platform, potentially making data integration and validation on independent sample sets a possibility. Unfortunately, because the array platform has been evolving over the past several years the arrays themselves have different probe sets, making direct comparisons difficult.
To test the comparability between different array platforms, we have accumulated all Affymetrix ALL array data that is available in the public domain, as well as two sets of cDNA array data. In addition, we have supplemented this data pool by profiling additional diagnostic pediatric ALL samples in our lab. Lists of genes that are differentially expressed in the six major subclasses of ALL have previously been reported in the literature as possible predictors of the subclass.

Results
We validated the predictability of these gene lists on all of the independent datasets accumulated from various labs and generated on various array platforms, by blindly distinguishing the prognostic genetic variables of ALL. Cross-generation array validation was used successfully with high sensitivity and high specificity of gene predictors for prognostic variables. We have also been able to validate the gene predictors with high accuracy using an independent dataset generated on cDNA arrays.

Conclusion
Interarray comparisons such as this one will further enhance the ability to integrate data from several generations of microarray experiments and will help to break down barriers to the assimilation of existing datasets into a comprehensive data pool.
Neoadjuvant or adjuvant therapy for resectable esophageal cancer: a clinical practice guideline
Authors: Malthaner Richard A , Wong Rebecca KS , Rumble R Bryan , Zuraw Lisa ,   members of the Gastrointestinal Cancer Disease Site Group of Cancer Care Ontario's Program in Evidence-based Care
Published: September 2004

Abstract
Background
Carcinoma of the esophagus is an aggressive malignancy with an increasing incidence. Its virulence, in terms of symptoms and mortality, justifies a continued search for optimal therapy. A clinical practice guideline was developed based on a systematic review investigating neoadjuvant or adjuvant therapy on resectable thoracic esophageal cancer.

Methods
A systematic review with meta-analysis was developed and clinical recommendations were drafted. External review of the practice guideline report by practitioners in Ontario, Canada was obtained through a mailed survey, and incorporated. Final approval of the practice guideline was obtained from the Practice Guidelines Coordinating Committee.

Results
The systematic review was developed and recommendations were drafted, and the report was mailed to Ontario practitioners for external review. Ninety percent of respondents agreed with both the evidence summary and the draft recommendations, while only 69% approved of the draft recommendations as a practice guideline. Based on the external review, a revised document was created. The revised practice guideline was submitted to the Practice Guidelines Coordinating Committee for review. All 11 members of the PGCC returned ballots. Eight PGCC members approved the practice guideline report as written and three members approved the guideline conditional on specific concerns being addressed. After these recommended changes were made, the final practice guideline report was approved.

Conclusion
In consideration of the systematic review, external review, and subsequent Practice Guidelines Coordinating Committee revision suggestions, and final approval, the Gastrointestinal Cancer Disease Site Group recommends the following:
For adult patients with resectable thoracic esophageal cancer for whom surgery is considered appropriate, surgery alone (i.e., without neoadjuvant or adjuvant therapy) is recommended as the standard practice.
Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: Case report
Authors: Breuckmann Frank , Freitag Marcus , Rotterdam Sebastian , Stuecker Markus , Altmeyer Peter , Kreuter Alexander 
Published: September 2004

Abstract
Background
Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible.

Case presentation
We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and Alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1+ and anti-mastcell-tryptase+ cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins.

Conclusions
Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therapy. A subsequent therapeutic attempt by the use of medium-dose ultraviolet A1 cold-light photomonotherapy led to a further stabilisation of clinical symptoms, but could not induce a sustained amelioration of skin condition.
Age related differences in individual quality of life domains in youth with type 1 diabetes
Authors: Wagner Julie A , Abbott Gina , Lett Syretta 
Published: September 2004

Abstract
Background
Investigating individual, as opposed to predetermined, quality of life domains may yield important information about quality of life. This study investigated the individual quality of life domains nominated by youth with type 1 diabetes.

Methods
Eighty young people attending a diabetes summer camp completed the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting interview, which allows respondents to nominate and evaluate their own quality of life domains.

Results
The most frequently nominated life domains were 'family', 'friends', 'diabetes', 'school', and 'health' respectively; ranked in terms of importance, domains were 'religion', 'family', 'diabetes', 'health', and 'the golden rule'; ranked in order of satisfaction, domains were 'camp', 'religion', 'pets', and 'family' and 'a special person' were tied for fifth. Respondent age was significantly positively associated with the importance of 'friends', and a significantly negatively associated with the importance of 'family'. Nearly all respondents nominated a quality of life domain relating to physical status, however, the specific physical status domain and the rationale for its nomination varied. Some respondents nominated 'diabetes' as a domain and emphasized diabetes 'self-care behaviors' in order to avoid negative health consequences such as hospitalization. Other respondents nominated 'health' and focused more generally on 'living well with diabetes'. In an ANOVA with physical status domain as the independent variable and age as the dependent variable, participants who nominated 'diabetes' were younger (M = 12.9 years) than those who nominated 'health' (M = 15.9 years). In a second ANOVA, with rationale for nomination the physical status domain as the independent variable, and age as the dependent variable, those who emphasized 'self care behaviors' were younger (M = 11.8 years) than those who emphasized 'living well with diabetes' (M = 14.6 years). These differences are discussed in terms of cognitive development and in relation to the decline in self-care and glycemic control often observed during adolescence.

Conclusions
Respondents nominated many non-diabetes life domains, underscoring that QOL is multidimensional. Subtle changes in conceptualization of diabetes and health with increasing age may reflect cognitive development or disease adjustment, and speak to the need for special attention to adolescents. Understanding individual quality of life domains can help clinicians motivate their young patients with diabetes for self-care. Future research should employ a larger, more diverse sample, and use longitudinal designs.