Search PubMedSearch

Biomedical subjects

J Ray

Publications and source records attributed to J Ray.

At least 19 recordsLinked to original sources

Calcium metabolism in pre-eclampsia.

OBJECTIVES: To study calcium metabolism in pre-eclampsia and normotensive gravid women. METHOD: Ten milliliters of heparinized blood samples and 24-h urine samples were collected from 50 pre-eclamptic and 50 normotensive primigravidae. Blood samples were studied for calcium uptake, intracellular calcium level and calcium-dependent adenosine triphosphatase activity of red blood cell ghost. Urinary calcium excretion was estimated from the 24-h urine samples. These values were compared in the two groups. RESULTS: The mean gestational age at recruitment was similar in both the groups. The mean maternal age was 24.28 +/- 2.41 years in pre-eclamptic and 23.48 +/- 4.16 years in normotensive women. In pre-eclampsia 24-h urinary calcium excretion (71.20 +/- 22.95 mg/day) and calcium-dependent ATPase activity (10.78 +/- 2.40 nmol/Pi/mg protein/min) was significantly lower compared to normotensive primigravidae (calcium excretion = 189.24 +/- 57.06 mg/day; Ca2+-dependent ATPase = 12.64 +/- 2.42 nmolPi/mg /protein per min; P < 0.001). Intracellular calcium levels and calcium uptake at 10 min by red blood cells were significantly higher in pre-eclampsia (P < 0.05). Calcium uptake by red blood cells at 20 and 30 min was similar in both groups. CONCLUSION: Pre-eclampsia is associated with increased levels of intracellular calcium, decreased calcium-dependent ATPase activity of erythrocytes and hypocalciuria.

Adult

Rapidly invading sebaceous carcinoma of the external auditory canal.

A very rare case of a sebaceous carcinoma of the external auditory canal with basal cell differentiation is presented. Fewer than 400 cases affecting any part of the body have so far been reported and of that only seven cases have been known to involve the external auditory canal. The clinical features, pathology and treatment are described and the relevant literature has been reviewed.

Adenocarcinoma, Sebaceous

Patient-controlled antiemesis: a randomized, double-blind comparison of two doses of propofol versus placebo.

BACKGROUND: The role of propofol for the management of postoperative nausea and vomiting (PONV) is not well established. This study determines the efficacy of small doses of propofol administered by patient-controlled device for the treatment of PONV. METHODS: Patients presenting for ambulatory surgery received a standardized general anesthetic. Those who experienced significant nausea or emesis within 1 h of arrival in the recovery room were randomized to receive repeated doses of propofol 20 mg (P-20), propofol 40 mg (P-40), or intralipid (placebo) on demand. Study medications (in equal volumes) were administered with a patient-controlled delivery device for 2 h. A lockout interval of 5 min between doses was used. The following parameters were assessed: nausea, vomiting, rescue antiemetic use, recovery profile, study drug administration history, and satisfaction with treatment. RESULTS: Sixty-nine patients participated in the study. Patient demographics were similar. The average nausea score for a patient in the P-20 and P-40 groups was 25% and 29% less, respectively, compared with placebo during the study period (P < 0.05). This difference was apparent 15 min after initiation of therapy. More placebo patients vomited (P-20, 12%; P-40, 23%; placebo, 56%; P = 0.003) and needed rescue antiemetics (P-20, 17%; P-40, 23%; placebo, 70%; P = 0.001) compared with treatment groups. Sedation scores were similar between groups. Propofol-treated patients had shorter stays in the post-anesthesia care unit (PACU; P-20, 131+/-35 min [mean +/- SD]; P-40, 141+/-34 min; placebo, 191+/-92 min; P = 0.005) and higher satisfaction with their control of PONV than placebo (P < 0.01). CONCLUSIONS: Propofol is effective in managing PONV with shorter PACU stay and great degree of patient satisfaction. There were two episodes of oversedation in the P-40 group. Hence, propofol at a demand dose of 20 mg seems more appropriate.

Adult

Evaluation of the APOH gene as a positional candidate for prcd in dogs.

PURPOSE: Progressive rod-cone degeneration (prcd) is an autosomal recessive retinal degeneration of dogs characterized by abnormalities in lipid metabolism. It has recently been mapped to the centromeric region of canine chromosome 9, homologous to human 17q, which contains the apolipoprotein H (apoH, protein; APOH, gene) gene involved in lipid metabolism and regulation of triglycerides. The present study was undertaken to evaluate APOH as a positional candidate for prcd. METHODS: Expression of APOH in the retina was examined by reverse transcription-polymerase chain reaction (RT-PCR) and by immunocytochemistry in normal and prcd-affected dogs. The level of apoH in the plasma was determined by western blot analysis. Intragenic polymorphic markers were identified and typed in the prcd pedigree. Canine-rodent hybrid cell lines were analyzed to detect canine APOH. RESULTS: ApoH has been localized to the photoreceptor outer segment layer by immunocytochemistry. Its expression in the retina of normal and prcd-affected dogs was confirmed by RT-PCR. The levels of antihuman apoH cross-reacting material in plasma were similar in all dogs, regardless of disease status. Finally, linkage analysis of the APOH gene with the disease locus in the prcd pedigree detected 3 recombinants among 70 informative offsprings (lod score 15.09 at 0 = 4.3 centimorgan [cM]). CONCLUSIONS: APOH is expressed in the retina and tightly linked to the prcd locus. However, despite its potential role in phenotypes of abnormal lipid metabolism associated with prcd, the gene has been excluded as a primary candidate for prcd by linkage analysis.

Animals

Synthesis and release of docosahexaenoic acid by the RPE cells of prcd-affected dogs.

PURPOSE: Dogs affected with progressive rod-cone degeneration (prcd) have reduced levels of docosahexaenoic acid (DHA, 22:6n-3) in their plasma and rod photoreceptor outer segments (ROS). Dietary supplementation of DHA has failed to increase the ROS DHA levels to that of unaffected control dogs. The present study was undertaken to test the hypothesis that prcd-affected dogs have a reduced capacity for the synthesis and/or release of DHA in retinal pigment epithelial (RPE) cells. METHODS: RPE cells (first passage cultures) from prcd-affected and normal dogs were incubated with [3H]eicosapentaenoic acid (EPA, 20:5n-3) for 24 and 72 hours. After incubation, the radiolabeled fatty acids in the cells and media were analyzed. RESULTS: DHA and all its metabolic intermediates were detected in RPE cells from prcd-affected and normal dogs. No significant difference was found in the amount of products (including DHA) synthesized between normal and affected RPE cells at either time point. In the culture media, RPE cells from prcd-affected dogs released significantly more DHA than cells from normal dogs after 72-hour incubation, but not after 24-hour incubation. CONCLUSIONS: RPE cells from prcd-affected dogs can synthesize and release DHA at least as efficiently as cells from normal dogs. Therefore, synthesis of DHA from its precursor and its release from RPE cells does not appear to contribute to the reduction in ROS DHA levels found in prcd-affected animals.

Animals

Two distinct mutations cause severe hemophilia B in two unrelated canine pedigrees.

The molecular defects causing severe factor IX deficiency were identified in two distinct canine breed-variants. Both defects were associated with an absence of plasma factor IX coagulant activity and antigen. A large deletion mutation was found in 1 breed variant, spanning the entire 5' region of the factor IX gene extending to exon 6. An approximately 5 kb insertion disrupted exon 8 of the second breed-variant. This insertion was associated with alternative splicing between a donor site 5' and acceptor site 3' to the normal exon 8 splice junction, with introduction of a new stop codon. The resultant transcript lacked most of the factor IX catalytic domain and 3' untranslated region. Molecular analyses of canine hemophilia B define an experimental model for study of inhibitor formation and gene therapy strategies, and provide insight into spontaneous mutation mechanisms in the factor IX gene and on the X chromosome of mammalian species.

Animals

Cloning of the canine beta-glucuronidase cDNA, mutation identification in canine MPS VII, and retroviral vector-mediated correction of MPS VII cells.

Mucopolysaccharidosis type VII (MPS VII) is an inherited disease resulting from deficient activity of the lysosomal acid hydrolase beta-glucuronidase (GUSB) and has been reported in humans, mice, cats, and dogs. To characterize canine MPS VII, we have isolated and sequenced the canine GUSB cDNA from normal and affected animals. A single nucleotide substitution was detected in the GUSB cDNA derived from MPS VII dogs. This guanosine to adenine base change at nucleotide position 559 in the canine cDNA sequence causes an arginine to histidine substitution at amino acid position 166. Introduction of the G to A substitution at position 559 in a mammalian expression vector containing the normal canine GUSB cDNA nearly eliminated the GUSB enzymatic activity, demonstrating that this mutation is the cause of canine MPS VII. A retroviral vector expressing the full-length canine beta-glucuronidase cDNA corrected the deficiency in MPS VII cells.

Amino Acid Sequence

The implementation of prompted retinal screening for diabetic eye disease by accredited optometrists in an inner-city district of North London: a quality of care study.

Diabetic retinopathy remains the most common cause of blindness in people of working age but the provision of high quality eye screening for diabetic patients is still erratic in many health districts in the UK. National consensus guidelines recommend comprehensive population coverage, high sensitivity (>80%), high specificity (>95%), agreed clinical criteria, referral procedures and centralized data collection to facilitate audit. This study looks at the effectiveness of implementing a prompted recall programme for retinal screening in an inner-city district of North London. The scheme uses trained, accredited optometrists to screen patients with diabetes who are looked after in the community by their general practitioner. During the first 17 months of the scheme, 63 optometrists attended training and gained accreditation. Of the 666 patients recruited, 645 were scheduled for screening and 536 (83%) attended. Fourteen per cent of patients screened were found to have background retinopathy and 2.3% sight-threatening eye disease. In two audits, carried out 15 months apart in a random sample of GP practices, the incidence of recorded dilated fundoscopy increased from 48% at baseline to 56%, an increase of 8% (95% CIs 2%-14%). For referable eye disease, the sensitivity of this screening technique was 100%, the specificity 94% (95% CIs 90%-98%), the positive predictive value 79% (95% CIs 72%-86%) and the negative predictive value 100%. The administrative cost per case screened was Pound Sterling 12.60 (excluding clinical costs and any additional optometry payment).

Diabetic Retinopathy

Multipotent progenitor cells in the adult dentate gyrus.

Neurogenesis persists in the adult dentate gyrus of rodents throughout the life of the organism. The factors regulating proliferation, survival, migration, and differentiation of neuronal progenitors are now being elucidated. Cells from the adult hippocampus can be propagated, cloned in vitro, and induced to differentiate into neurons and glial cells. Cells cultured from the adult rodent hippocampus can be genetically marked and transplanted back to the adult brain, where they survive and differentiate into mature neurons and glial cells. Although multipotent stem cells exist in the adult rodent dentate gyrus, their biological significance remains elusive.

Animals

Cochlear implant failure due to unexpected absence of the eighth nerve--a cautionary tale.

We present a case of bilateral absence of the eighth cranial nerve in the internal auditory meatus (IAM). This caused total failure of responses after cochlear implantation in a six-year-old patient with congenital deafness. Pre-operative magnetic resonance (MR) imaging is important to show not only the anatomy of the middle and inner ears but also the structures in the IAM.

Child

The Scheibe cochlea deformity with macrocephaly: a case for single channel implantation.

An 11-year-old congenitally deaf child with bilateral primitive common cavity (Scheibe type) cochleosaccular dysplasia and benign familial macrocephaly was implanted with an extracochlear single channel device with an ear level speech processor. This paper describes the assessment, findings, dilemmas in decision making, surgical procedure and the favourable outcome after implanting. The relevant literature has been reviewed and our case is presented for the unusual combination of features.

Abnormalities, Multiple

Bilateral and multicystic major salivary gland disease: a rare presentation of primary Sjögren's syndrome.

We present a case of a 15-year-old girl with bilateral parotid and sub-mandibular salivary gland enlargement as the sole presentation of primary Sjögren's syndrome. The clinical, radiological, immunological and pathological features have been discussed. The relevant literature has been reviewed. To our knowledge this is the only reported case of Sjögren's syndrome presenting as multicystic disease with bilateral major salivary gland involvement.

Adolescent

Counterion condensation revisited.

We review some of the characteristic properties of the structure of polyelectrolyte solutions: the condensed layer of counterions that forms abruptly at a critical threshold charge density on the polymer chain; the more diffuse Debye-Hückel cloud, which is spatially distinct from the condensed layer; and the entropic release of counterions from the condensed layer as a driving force for the binding of oppositely charged ligands. We present a reminder of the basis of our current understanding in a variety of experiments, simulations, and theories; and we attempt as well to clarify some misunderstandings. We present a new analysis of a lattice model that suggests why the limiting laws for polyelectrolyte thermodynamics have proved to be accurate despite the neglect of polymer-polymer interactions in their original derivation. We sketch recent progress in constructing a potential between counterion and polyion. A counterion located in the interface between condensed layer and Debye cloud is repelled from the polyion, creating a sharp boundary between the two counterion populations.

Electrolytes