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Biomedical subjects

P Lee

Publications and source records attributed to P Lee.

At least 19 recordsLinked to original sources
PubMed2007

A systematic review of the safety of topical therapies for atopic dermatitis.

BACKGROUND: The safety of topical therapies for atopic dermatitis (AD), a common and morbid disease, has recently been the focus of increased scrutiny, adding confusion as how best to manage these patients. OBJECTIVES: The objective of these systematic reviews was to determine the safety of topical therapies for AD. METHODS: Databases searched included: OVID Medline, Medline In-Process and Other Non-Indexed Citations, Embase, and the Cochrane Central Register of Controlled Trials. In addition to the articles identified by this search, investigators were also referred to a list of links (most recently updated 25 September 2005) to recent Food and Drug Administration (FDA) studies, reports and meetings regarding the topical calcineurin inhibitors for further potential references. Only fully published papers available in English and data obtained from FDA sites were included. Furthermore, the criteria for inclusion and exclusion for each systematic review were further evaluated at a meeting of all of the content and evidence-based medicine experts participating in this process and alteration of the inclusion criteria was done at that time when it was felt necessary to avoid inclusion of lower-quality data in the review. Qualitative review of the abstracted data was performed and reviewed at a meeting of all of the content and evidence-based medicine experts. RESULTS: While systemic exposure to these topical agents does occur, physiological changes appear to be uncommon and systemic complications rare and have only been found with use of topical corticosteroids. CONCLUSIONS: Based on the data that are available the overall safety of AD therapies appears to be good with the only documented systemic side-effects of therapy those occasionally seen with use of topical corticosteroids.

Administration, Topical↗
PubMed2006

Severe tongue protrusion dystonia: clinical syndromes and possible treatment.

We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. Speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Lesch-Nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. Tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.

Adolescent↗
PubMed2006

'Ready to drink' protein substitute is easier is for people with phenylketonuria.

UNLABELLED: In phenylketonuria (PKU), compliance with taking protein substitute is an issue in teenage and older patients. A 'ready to drink' protein substitute may overcome many of the practical issues associated with its administration. OBJECTIVE: To investigate the efficacy of a liquid protein substitute in a 6-week, three-part, randomized, crossover, controlled study. METHODS: 27 subjects (15 female; 12 male) with PKU with a median age of 30 years (range 8-49 years) were recruited. One subject withdrew from the study. Their median daily dose of protein equivalent was 60 g (range 45-75 g). In parts 1 and 2, subjects were randomized to either a liquid or a powder protein substitute with the same nutritional composition per unit (each 130 ml liquid pouch or 25 g powder sachet contained 15 g protein equivalent). In part 3, subjects chose liquid, powder or a combination of both. Weekly blood phenylalanine (Phe) concentrations were estimated, and during weeks 2, 4 and 6 subjects completed a daily questionnaire on administration issues. RESULTS: All but one of 26 subjects chose the liquid in part 3 as either their sole (69%, n = 18) or partial source (28%, n = 7) of protein substitute. Blood Phe concentrations were significantly better on the liquid (p = 0.03). With the liquid protein substitute, subjects were less self-consciousness (p = 0.003) and found it easier to take away from home (p = 0.001). Overall, the liquid was easier (p < 0.0001), more convenient (p = 0.002) and resulted in less wastage of protein substitute (p = 0.001). CONCLUSION: Liquid protein substitute was popular and efficacious, reduced self-consciousness and overall improved compliance of teenagers and adults with PKU.

Adolescent↗
PubMed2006

Omental infarct: CT imaging features.

BACKGROUND: The aim of this study is to describe contrast-enhanced computed tomographic (CT) features of acute omental infarction and to study the evolutionary changes on follow-up CT imaging. METHODS: Fifteen cases of omental infarction were evaluated for their initial CT imaging features. The imaging features evaluated included size of the fatty lesion, location, peripheral rim, and relation to colon. CT findings were correlated with etiology, clinical presentation, and leukocytosis. Follow-up CT images were available in eight patients and the imaging features were studied. RESULTS: Eight omental infarcts were of unknown etiology and seven were secondary to abdominal surgery. In 53% of patients (eight of 15), the location of the omental infarct was in the right lower, mid, or upper quadrants. These eight right-side infarcts occurred in six patients with primary omental infarcts. In 13 of 14 patients who underwent CT within 15 days of onset of omental infarct, the margin of the lesion was ill defined. Primary omental (n = 8) infarcts were seen in younger patients (p = 0.02) and were larger on CT (p = 0.02) compared with secondary omental infarcts. CT findings evolved from an ill-defined, heterogeneous fat-density lesion to a well-defined, heterogeneous fat-density lesion with a peripheral hyperdense rim in all six secondary omental infarctions for which acute stage and follow-up CT images were available for interpretation. CONCLUSION: There is a significant difference in the age distribution and CT findings in terms of size of the omental infarction between primary and secondary etiologies. On follow-up CT, secondary omental infarcts progressively shrank and developed a well-defined, hyperdense rim around a fatty core.

Abdomen, Acute↗
PubMed2006

How we do it: tympanoplasty: are different three- and four-frequency averages comparable?

The results of tympanoplasty may be reported in a variety of ways. Pure-tone audiometry underpins reporting, yet there is no constancy in the pure-tone threshold average used. We examine the relationship between the 0.5, 1, 2 kHz, 0.5, 1, 2, 3 kHz and 0.5, 1, 2, 4 kHz pure-tone audiometry averages. Using audiometric data collected for 77 patients, we have compared hearing threshold using three different frequency averages derived from pure-tone audiometry. The 0.5, 1, 2 kHz, 0.5, 1, 2, 3 kHz and 0.5, 1, 2, 4 kHz three- and four-frequency averages are significantly different, this difference being primarily determined by the changes in the 4-kHz threshold. Using an average including 4 kHz results in 8% few patients achieving an ABG closure to within either 10 dB or 20 dB. When comparing surgical results with others, surgeons should use reports employing comparable audiometric measures of analysis.

Adolescent↗
PubMed2005

Coronary microvascular dysfunction in male patients with Anderson-Fabry disease and the effect of treatment with alpha galactosidase A.

OBJECTIVE: To measure coronary flow reserve (CFR), an index of microvascular function, in Anderson-Fabry disease (AFD) at baseline and after enzyme replacement therapy (ERT). METHODS AND RESULTS: Mean (SD) myocardial blood flow (MBF) at rest and during hyperaemia (adenosine 140 microg/kg/min) was measured in 10 male, non-smoking patients (53.8 (10.9) years, cholesterol 5.5 (1.3) mmol/l) and in 24 age matched male, non-smoking controls (52.0 (7.6) years, cholesterol 4.5 (0.6) mmol/l) by positron emission tomography (PET). Resting and hyperaemic MBF and CFR (hyperaemic/resting MBF) were reduced in patients compared with controls (0.99 (0.17) v 1.17 (0.25) ml/g/min, p < 0.05; 1.37 (0.32) v 3.44 (0.78) ml/g/min, p < 0.0001; and 1.41 (0.39) v 3.03 (0.85), p < 0.0001, respectively). This coronary microvascular dysfunction was independent of cholesterol concentrations. PET was repeated in five patients after 10.1 (2.3) months of ERT; resting and hyperaemic MBF and CFR were unchanged after ERT (0.99 (0.16) v 0.99 (0.16) ml/g/min; 1.56 (0.29) v 1.71 (0.3) ml/g/min; and 1.6 (0.37) v 1.74 (0.28), respectively; all not significant). CONCLUSIONS: The results of the present study show that patients with AFD have very abnormal coronary microvascular function. These preliminary data suggest that ERT has no effect on coronary microvascular dysfunction. Further work is necessary to determine whether treatment at an earlier stage in the course of the disease may improve coronary microvascular function in patients with AFD.

Adult↗
PubMed2005

Measurement of urinary CDH and CTH by tandem mass spectrometry in patients hemizygous and heterozygous for Fabry disease.

Fabry disease is an X-linked disorder of glycosphingolipid metabolism resulting from a deficiency of the lysosomal enzyme alpha-galactosidase A. This deficiency leads to the progressive accumulation, in lysosomes of visceral tissues and in body fluids of hemizygotes, of the glycosphingolipids globotriaosylceramide (CTH, Gb(3) or GL-3) and galabiosylceramide (CDH) and to a lesser extent the blood group AB and B related glycolipids. Elevated levels of the glycosphingolipids are found in the urine of hemizygous males with the classic phenotype, but it is not known whether all symptomatic or asymptomatic heterozygotes have elevated levels. We have therefore measured CTH and CDH quantitatively in a multiplex assay using tandem mass spectrometry in urine from a large cohort (44) of genetically proven or obligate heterozygotes including four with the N215S mutation, from classic hemizygotes (28), from cardiac variant hemizygotes with the N215S mutation (6) and from normal controls. The levels of CTH and CDH were related to both creatinine and sphingomyelin. Urinary CTH was elevated in all 28 classic hemizygotes but only in 4/6 of the cardiac variants. The level was within or just above the normal reference range in the four individuals heterozygous for the N215S mutation but was elevated in 38/40 of the other heterozygotes. Similar results were obtained for CDH, except that only 34/40 heterozygotes had an elevated level. The level of CDH was not elevated in the four heterozygotes and 4/6 of the hemizygotes for the N215S mutation. Combining the levels of CTH and CDH did not improve the discrimination of heterozygotes from controls. The ratio of CDH to CTH was higher in heterozygotes than in hemizygotes. Measurement of urinary CTH gave the best discrimination of heterozygotes from controls.

Chromatography, Liquid↗
PubMed2005

Is globotriaosylceramide a useful biomarker in Fabry disease?

AIM: The aim of this study was to determine whether globotriaosylceramide (Gb3) is a useful biomarker in Fabry disease. METHODS: The levels of Gb3 were measured in plasma and urine by tandem mass spectrometry in untreated hemizygotes and heterozygotes with Fabry disease and in healthy controls, and the levels were monitored in patients on treatment with enzyme replacement therapy (ERT). RESULTS: Hemizygotes with classic Fabry disease showed elevated levels of Gb3 in both plasma and urine and could readily be distinguished from normal controls. Male patients with the N215S mutation had normal levels in their plasma but 50% had marginally elevated levels in their urine. Thirty-three percent of proven heterozygotes had elevated Gb3 concentrations in plasma but 97% of those without the N215S mutation (36/37) had an elevated level in urine. The four heterozygotes with the N215S mutation had normal Gb3 levels in urine. The level of Gb3 in plasma initially fell following the start of ERT in all patients who had an elevated level before treatment. However, in a few patients the level subsequently rose. Similar results were found for the levels of Gb3 in urine. CONCLUSION: Gb3 is not an ideal marker of Fabry disease or the response to treatment in all patients. Plasma and urine levels of Gb3 cannot be used as a marker of Fabry disease in patients with the N215S mutation and many heterozygotes do not have elevated Gb3 levels in plasma. The urine concentration is more informative in heterozygotes and can be used as a measure of the response to therapy. The fall in Gb3 levels in patients receiving ERT was not sustained in some patients, despite a clinical improvement. Additionally, Gb3 cannot be used to monitor the response to treatment in patients who initially have normal plasma and urine concentrations of this glycolipid.

Biomarkers↗
PubMed2004

Parity-violating electron deuteron scattering and the proton's neutral weak axial vector form factor.

We report on a new measurement of the parity-violating asymmetry in quasielastic electron scattering from the deuteron at backward angles at Q2=0.038 (GeV/c)2. This quantity provides a determination of the neutral weak axial vector form factor of the nucleon, which can potentially receive large electroweak corrections. The measured asymmetry A=-3.51+/-0.57 (stat)+/-0.58 (syst) ppm is consistent with theoretical predictions. We also report on updated results of the previous experiment at Q2=0.091 (GeV/c)2, which are also consistent with theoretical predictions.

Journal Article↗
PubMed2004

Surgery of the hand in severe systemic sclerosis.

Ten patients with scleroderma and severe hand problems required surgery, and seven were available for follow-up (two died from scleroderma-related complications and one was lost to follow-up). The mean duration of follow-up was 4 (range 1.5-9) years. Thirty-three procedures were carried out, including five metacarpophalangeal joint excisional arthroplasties, 13 proximal interphalangeal joint fusions, ten distal interphalangeal joint fusions, and one thumb interphalangeal joint fusion. The metacarpophalangeal joint excision arthroplasties and proximal interphalangeal joint fusions were performed for the correction of severe fixed "finger-in-palm" deformities. Lesions of cutaneous calcinosis were removed in four patients. Fixation was satisfactory in all cases of interphalangeal joint fusion, with no cases of nonunion. Wound healing was satisfactory in six of seven patients. A second surgical procedure was required in three patients for the removal of tension band wires following interphalangeal fusion. Calcinosis was effectively removed using a high-speed dental burr. The results of hand surgery for systemic sclerosis are reliable, but goals must be limited and patient expectations should be modest.

Adult↗
PubMed2004

A new, low-volume protein substitute for teenagers and adults with phenylketonuria.

Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low-volume (amino acids 72 g/100 g), low-carbohydrate, phenylalanine-free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty-three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8-37 years) took the substitute for 8 weeks. A 3-day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin-puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140-180) daily to 100 g (range 100-125) daily ( p <0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 micromol/L (95% CI-27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty-one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.

Adolescent↗
PubMed2004

Pathological calcification in juvenile dermatomyositis (JDM): microCT and synchrotron x-ray diffraction reveal hydroxyapatite with varied microstructures.

The objective of this study was to begin to relate the microstructure of calcinosis samples to clinical and laboratory characteristics of the juvenile dermatomyositis (JDM) patients. Laboratory x-ray microCT (micro-Computed Tomography) noninvasively mapped microstructure for the first time in JDM calcifications. Synchrotron x-ray diffraction (transmission geometry) identified the mineral phase and crystallite size in the deposits. Samples were obtained from four children who had active JDM longer than 80 months and who were typed for TNFalpha-308 allele polymorphisms. Uniform mineral (giving the appearance of an extruded solid) was observed in one patient, and irregular blocks of differing sizes filled the samples from two other patients. The sample from the fourth patient appeared to combine features of the other two types. These spatial distributions of mineral were quite different from those in a bone reference sample. The only mineral observed in the JDM samples was hydroxyapatite (HAP), and the diffraction peaks of the JDM samples were slightly narrower than those of a trabecular bone reference sample. Diffraction peak widths of the JDM specimens revealed crystallite sizes (approximately 220-240 A) that are comparable to values reported in the literature for bone. Three children were positive for TNFalpha-308 GA polymorphism. The data suggest several possible origins for blocky vs. uniform structure of the JDM calcifications, including differences in duration of untreated inflammation, in TNFalpha-308 polymorphism, and in mechanical constraint at the calcification site. Information from additional samples is required to determine the relative role of each of these factors. Taken together, non-invasive microCT and x-ray diffraction characterization on the same samples offer an informative window into the dystrophic mineralization process in JDM.

Adolescent↗