Ciliary body adenoma of non-pigmented epithelium

Adenomas of the non-pigmented epithelium of the ciliary body are rare neoplasms and most of the studies are in the form of case reports. There are only 27 documented cases of acquired neoplasm of the non-pigmented ciliary epithelium (NPCE) reported in the English literature. In most reports, there was a clinical suspicion of melanoma and the diagnosis of NPCE adenoma was made on histopathological evaluation of the resected tissue. The entity has not been reported in the Pakistani population to date. This report describes a case of ciliary body adenoma of NPCE in a 27 year old Pakistani man. The histological and immunohistochemical profiles were typical of the adenomas described in the literature.     

Malignant hyperthermia

Malignant hyperthermia refers to covert myopathies, which do not affect the individual during daily life activities, but may lead to life-threatening tachycardia, rigor, labile blood pressure and most importantly high-grade temperature when exposed to general anaesthesia. This conditions is mimicked by thyroid storm, neuroleptic malignant syndrome, phaeochromocytoma and sepsis. We present a presumptive case of malignant hyperthermia.     

Enzyme immobilization in novel alginate-chitosan core-shell microcapsules

Alginate-chitosan core-shell microcapsules were prepared in order to develop a biocompatible matrix for enzyme immobilization, where the protein is retained either in a liquid or solid core and the shell allows permeability control over substrates and products. The permeability coefficients of different molecular weight compounds (vitamin B2, vitamin B12, and myoglobin) were determined through sodium tripolyphosphate (Na-TPP)-crosslinked chitosan membrane. The microcapsule core was formed by crosslinking sodium alginate with either calcium or barium ions. The crosslinked alginate core was uniformly coated with a chitosan layer and crosslinked with Na-TPP. In the case of calcium alginate, the phosphate ions of Na-TPP were able to extract the calcium ions from alginate and liquefy the core. A model enzyme, beta-galactosidase, was immobilized in the alginate core and the catalytic activity was measured with o-nitrophenyl-beta-D-galactopyranoside (ONPG). Change in the activity of free and immobilized enzyme was determined at three different temperatures. Na-TPP crosslinked chitosan membranes were found to be permeable to solutes of up to 17,000Da molecular weight. The enzyme loading efficiency was higher in the barium alginate core (100%) as compared to the calcium alginate core (60%). The rate of ONPG conversion to o-nitrophenol was faster in the case of calcium alginate-chitosan microcapsules as compared to barium alginate-chitosan microcapsules. Barium alginate-chitosan microcapsules, however, did improve the stability of the enzyme at 37 degrees C relative to calcium alginate-chitosan microcapsules or free enzyme. This study illustrates a new method of enzyme immobilization for biotechnology applications using liquid or solid core and shell microcapsule technology.     

Ossifying fibromyxoid tumor of the ethmoid sinus in a newborn: report of a case and literature review

Ossifying fibromyxoid tumor (OFT) of soft parts is a benign soft tissue tumor commonly located in the extremities. In this paper, a 3-week-old boy presented with left nasal mass at birth. He was found to have an OFT involving the ethmoid sinus. To the best of our knowledge, this is the first reported case of OFT in a newborn with involvement of the sinuses. This rare tumor should alert Pediatric Otolaryngologists to include it in the differential diagnosis of pediatric soft tissue tumors in sinuses.     

Idiopathic edema is associated with obstructive sleep apnea in women

BACKGROUND AND PURPOSE: This study was undertaken to clarify whether idiopathic edema is a marker for obstructive sleep apnea (OSA), independent of level of obesity, in patients with normal left ventricular function. PATIENTS AND METHODS: Seventy-eight ambulatory, obese, adults, 44 with bilateral, pitting pre-tibial edema, and 34 without edema, from an inner city family practice and a suburban family practice enrolled from July 1995 until March 2003. Edematous subjects, but not non-edematous subjects, underwent echocardiography, urinalysis, and blood test evaluations to ensure that cardiac, renal, hepatic, and thyroid functions were normal. All subjects underwent spirometry, pulse oximetry on room air, and polysomnography evaluations. RESULTS: Compared to the non-edematous subjects, the edematous subjects were more obese (body mass index=47.0+/-9.3 versus 36.5+/-4.6 kg/m2, P=0.002), had more severe OSA (apnea-hypopnea index (AHI)=34.1+/-27.7 versus 17.0+/-19.4, P=0.002), and had lower oxygen saturations (96.2+/-2.0 versus 97.1+/-1.5%, P=0.05). Using an AHI > or = 15 as the criteria for diagnosing OSA, there was an association between edema and OSA in women (P=0.02) but not men. CONCLUSIONS: In subjects with normal left ventricular function, idiopathic edema is associated with OSA in women.     

Defining the pathogenicity of optineurin in juvenile open-angle glaucoma

PURPOSE: Juvenile open-angle glaucoma (JOAG) differs from primary open-angle glaucoma in that it is usually a more severe phenotype and has an earlier age of onset. Optineurin was recently associated with a variant of POAG that is characterized by intraocular pressure within normal limits: normal-tension glaucoma. The present study tested whether OPTN sequence changes play a role in early-onset glaucoma characterized by elevated intraocular pressure. METHODS: Sixty-six patients with JOAG characterized by high intraocular pressure were screened for mutations. Mutational analysis was performed with a combination of restriction enzyme digestion, single-strand conformation polymorphism, and direct sequencing. The effects of select changes on exon splicing were assessed using bioinformatic modeling approaches and RT-PCR. RESULTS: Ten sequence changes were identified, of which H486R was strongly suggestive of pathogenicity. H486R represents the first reported OPTN mutation associated with JOAG. Also, L41L is proposed to confer an increased susceptibility to the development of JOAG. Most of the other sequence changes observed were not thought to be biologically significant. The frequency of the previously reported M98K allele was not increased in the JOAG population studied but showed the previously reported skewed distribution in the POAG study population. The changes identified were not shown to affect the splicing machinery. CONCLUSIONS: The results of this work support the hypothesis that mutations in OPTN are not specifically associated with low-pressure glaucoma, but can play a role in JOAG.     

Clinical outcome and atherothrombogenic risk profile after prolonged wash-out following long-term treatment with high doses of n-3 PUFAs in patients with an acute myocardial infarction

BACKGROUND: Sustained effects following withdrawal of n-3 PUFAs are unknown. METHODS: Clinical outcome [cardiac death, resuscitation, recurrent myocardial infarction (MI) or unstable angina pectoris] was assessed after prolonged wash-out following randomised treatment with high-dosed n-3 PUFAs or corn oil for 12-24 months in 300 acute MI patients. Atherothrombogenic risk markers, serum glucose and markers of lipid peroxidation and inflammation were evaluated in 89 out of the 100 last included patients. RESULTS: After a total median observation period of 45 (range 0-53) months no intergroup difference in prognosis was observed for any of the cardiac events. Favourable effects on serum triglycerides and HDL-cholesterol by n-3 PUFAs were lost after washout, but triglycerides decreased in the corn oil as compared to the n-3 group, P < 0.001. The decline in total cholesterol after withdrawal was similar in both groups. No intergroup difference in the change in thiobarbituric acid-malondialdehyde, a marker of lipid peroxidation, ultrasensitive C-reactive protein, homocysteine, glucose or blood platelets was noted at sustained follow-up. CONCLUSION: Clinical outcome was similar in both patient groups, and the atherothrombogenic risk improvement by n-3 PUFAs was lost after prolonged wash-out. Withdrawal did not affect homocysteine, glucose or markers of lipid peroxidation or inflammation.     

Education and evidence are needed to improve neonatal parenteral nutrition practice

BACKGROUND: Parenteral nutrition (PN) is an essential component of neonatal care for those infants who are unable to tolerate adequate enteral feeding. Its use is not without complications such as biochemical derangements, sepsis, thrombosis, extravasation of fluid, and death. Such complications can be reduced by meticulous management of PN in response to biochemical abnormalities, nutrition teams, policies to reduce sepsis, and staff training to be more aware of pericardial and pleural effusions. We ascertained the current practices in PN administration and management of complications in all neonatal units with 6 or more intensive care cots in England, Scotland, and Wales. METHODS: Telephone survey of middle grade doctors (Specialist Registrars) working in all 57 neonatal units was conducted using a standard questionnaire. The questions were focused around practical issues and problems that are commonly encountered with PN practice, including composition, complications, and nutrition support. RESULTS: A response was obtained from 95% of the units contacted and a wide range of practices observed. Thirty-three percent of units delay protein (nitrogen) until > 48 hours after birth. Lipid infusions are stopped in proven or suspected sepsis in just over half of all units. In hyperglycemic preterm infants, 25 units decrease their glucose infusion, 21 commence insulin, and 8 have no policy. Two thirds of middle grade doctors had no idea of the amount of protein or nitrogen to prescribe for these infants, and only one-third involve a pharmacist in the PN prescribing. CONCLUSIONS: There is a diverse practice and knowledge with a concerning lack of education in nutrition among the middle grade doctors in England, Scotland, and Wales. The management of common complications such as sepsis and hyperglycemia are highly variable. Improved staff training and production of unified evidence-based guidelines need urgent consideration.     

Interaction of nucleoside analogues with nucleoside transporters in rat brain endothelial cells

A number of nucleoside analogues, consisting of antiviral compounds and agents designed as adenosine A1 receptor agonists, were examined for nucleoside transporter affinity using an in vitro model of the blood-brain barrier (BBB), the rat brain endothelial cell line, RBE4. Structure-activity relationships (SAR) were also performed to identify the key structural requirements for transporter recognition and the suitability of these systems for carrier-mediated strategies to deliver therapeutics across the BBB. Adenosine receptor agonists did not show transport affinity for concentrative nucleoside carriers, but exhibited affinity for equilibrative systems (Ki=10.8-97.9 microM) within the range of Kms for natural substrates. However, none of the antiviral compounds tested in this study showed affinity for either class of nucleoside transporter. SAR studies suggest that the hydroxyl group located at the 3'-position of the ribose moiety is an essential requirement for transporter recognition. This may explain the inability of nucleoside derived anti-viral compounds to use these systems despite the significant structural homology with naturally occurring nucleosides. Sites have also been identified which accommodate structural additions with retention of carrier affinity, suggesting that compounds which fail to penetrate the BBB could be attached to these sites for carrier-mediated delivery using a prodrug strategy.