Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up

IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.     

Single Nucleotide Polymorphisms in the FADS Gene Cluster but not the ELOVL2 Gene are Associated with Serum Polyunsaturated Fatty Acid Composition and Development of Allergy (in a Swedish Birth Cohort)

Exposure to polyunsaturated fatty acids (PUFA) influences immune function and may affect the risk of allergy development. Long chain PUFAs are produced from dietary precursors catalyzed by desaturases and elongases encoded by FADS and ELOVL genes. In 211 subjects, we investigated whether polymorphisms in the FADS gene cluster and the ELOVL2 gene were associated with allergy or PUFA composition in serum phospholipids in a Swedish birth-cohort sampled at birth and at 13 years of age; allergy was diagnosed at 13 years of age. Minor allele carriers of rs102275 and rs174448 (FADS gene cluster) had decreased proportions of 20:4 n-6 in cord and adolescent serum and increased proportions of 20:3 n-6 in cord serum as well as a nominally reduced risk of developing atopic eczema, but not respiratory allergy, at 13 years of age. Minor allele carriers of rs17606561 in the ELOVL2 gene had nominally decreased proportions of 20:4 n-6 in cord serum but ELOVL polymorphisms (rs2236212 and rs17606561) were not associated with allergy development. Thus, reduced capacity to desaturase n-6 PUFAs due to FADS polymorphisms was nominally associated with reduced risk for eczema development, which could indicate a pathogenic role for long-chain PUFAs in allergy development.     

Electronic pill-boxes in the evaluation of oral hypoglycemic agent compliance

OBJECTIVE: To compare compliance in type 2 diabetic patients treated with glimepiride once daily or glibenclamide twice to three times daily. METHODS: Poorly controlled type 2 diabetic patients aged 35-65 years were randomized to glimepiride 1 mg once daily or to glibenclamide 1.25 mg twice daily. During initial titration, doses ranged from 1 to 6 mg once daily (glimepiride) and from 1.25 mg twice daily to 5 mg 3 times daily (glibenclamide) to achieve fasting blood glucose < 126 mg/dL. The final titration phase doses were continued during the maintenance phase. Both treatments were packed in electronic pill-boxes fitted with a microprocessor to record dates and times of each opening. Compliance was assessed in terms of mean daily compliance (MDC) and the ratio of days with adequate compliance (DAC). Glycemic control was assessed in terms of the adjusted mean final HbA1c, and the incidence of hypoglycemia. Patient satisfaction was evaluated using the Diabetes Treatment Satisfaction Questionnaire. RESULTS: Compliance over the whole study was generally good, but the MDC was significantly better with glimepiride (87+/-16%) than with glibenclamide (80+/-17%;P < 0.0001). The ratios of DAC for glimepiride and glibenclamide were 87+/-16% and 67+/-24% respectively (P < 0.0001). The adjusted final HbA1c, and the incidence of hypoglycemia were similar in the two groups. Treatment satisfaction on the DTSQc was greater with glimepiride than with glibenclamide (P = 0.0034). CONCLUSIONS: Patient compliance and treatment satisfaction with once-daily glimepiride were significantly better than with glibenclamide 2 to 3 times daily.     

Serial left ventricular adaptations in world-class professional cyclists: implications for disease screening and follow-up

OBJECTIVES: The purpose of this research was to study long-term left ventricular (LV) adaptations in very-high-level endurance athletes. BACKGROUND: Knowledge of cardiac changes in athletes, who are at particularly high risk of sudden cardiac death, is mandatory to detect hypertrophic cardiomyopathy (HCM) or dilated (DCM) cardiomyopathy. METHODS: We carried out echocardiographic examinations on 286 cyclists (group A) and 52 matched sedentary volunteers (group C); 148 cyclists participated in the 1995 "Tour de France" race (group A1), 138 in the 1998 race (group A2), and 37 in both (group B). RESULTS: In groups A, A1, A2, and C, respectively, diastolic left ventricular diameter (LVID) was 60.1 +/- 3.9 mm, 59.2 +/- 3.8 mm, 61.0 +/- 3.9 mm, and 49.0 +/- 4.3 mm (A vs. C and A1 vs. A2, p < 0.0001), and maximal wall thickness (WT) was 11.1 +/- 1.3 mm, 11.6 +/- 1.3 mm, 10.6 +/- 1.1 mm, and 8.6 +/- 1.0 mm (A vs. C and A1 vs. A2, p < 0.0001). Among group A, 147 (51.4%) had LVID >60 mm; 17 of them had also a below normal (<52%) left ventricular ejection fraction (LVEF). Wall thickness exceeded 13 mm in 25 athletes (8.7%) (always <15 mm), 23 with LVID >55 mm. In group B, LVID increased (58.3 +/- 4.8 mm to 60.3 +/- 4.2 mm, p < 0.001) and WT decreased (11.8 +/- 1.2 mm to 10.8 +/- 1.2 mm, p < 0.001) with time. CONCLUSIONS: Over one-half of these athletes exhibited unusual LV dilation, along with a reduced LVEF in 11.6% (17 of 147), compatible with the diagnosis of DCM. Increased WT was less common (always <15 mm) and scarce without LV dilation (<1%), eliminating the diagnosis of HCM. Serial examinations showed evidence of further LV dilation along with wall thinning. These results might have important implications for screening in athletes.     

Study of left ventricular function at rest and post-exercise with thallium-201 myocardial scintigraphy synchronized with ECG

ECG-gated Thallium 201 myocardial scintigraphy provides a simultaneous evaluation of left ventricular perfusion and function. The aims of this study were to determine the changes in left ventricular ejection fraction (LVEF) after exercise and at rest 4 hours after exercise and to compare the results with changes in myocardial perfusion and the severity of the coronary artery disease. Sixty-four men with myocardial ischaemia on scintigraphy who had undergone coronary angiography showing significant lesions within 3 months, were compared with 38 normal men. The ejection fraction was calculated with a validated programme (QGS). The change in LVEF between the post-exercise and resting measurement 4 hours after exercise (delta LVEF) was compared in the normal and ischaemic groups (+7 +/- 6.8% vs -5.6 +/- 5%, p < 0.001). The extent of the ischaemia (percentage myocardium unperfused) was significantly greater in the 34 patients who had an over 5% reduction in LVEF on exercise compared with the 30 others who has a less than 5% reductionin LVEF (11.8 vs 6.3%, p < 0.001). There was a linear correlation between the degree of ischaemia and delta LVEF in the 30 patients without a history of infarction (r = -0.76, p < 0.01). The delta LVEF also correlated with the number and site of the coronary lesions. The authors conclude that in this male population, ECG-gated Thallium 201 myocardial scintigraphy can demonstrate a decrease in LVEF after exercise in ischaemic coronary patients whereas it increases in normal subjects. This decrease in LVEF on exercise is correlated with the extent of ischaemia and the severity of the coronary disease and should therefore be taken into account in patient management.     

Differential matrix degradation and turnover in early cartilage lesions of human knee and ankle joints

OBJECTIVE: To determine whether there are differences in matrix turnover within early cartilage lesions of the ankle (talocrural) joint compared with the knee (tibiofemoral) joint that may help explain differences in the prevalence of osteoarthritis in these 2 joints. METHODS: Cartilage removed from lesions of the tali and femoral condyles was analyzed for type IIB collagen messenger RNA, C-terminal type II procollagen propeptide (CPII), the collagenase cleavage neoepitope (Col2-3/4C(short)), and the denaturation epitope (Col2-3/4m). The content of collagen, glycosaminoglycan, and epitope 846 of aggrecan was quantitated. RESULTS: In ankle lesions, there was an up-regulation of markers of synthesis (CPII [P = 0.07]; epitope 846 [P < or = 0.0001]), but these were down-regulated in the knee (CPII [P = 0.1]; epitope 846 [P = 0.004]). In lesions of the knee, but not the ankle, there was an up-regulation of collagen degradation markers (P = 0.008). On a molar basis, there was 24 times more cleavage epitope than denaturation epitope in knee lesions compared with ankle lesions. CONCLUSION: The up-regulation of matrix turnover that is seen in early cartilage lesions of the ankle would appear to represent an attempt to repair the damaged matrix. The increase in collagen synthesis and aggrecan turnover seen in ankle lesions is absent from knee lesions. Instead, there is an increase in type II collagen cleavage. Together with the differences in collagen denaturation, these changes point to an emphasis on matrix assembly during early lesion development in the ankle and to degradation in the knee, resulting in fundamental differences in matrix turnover in these lesions.     

Home unattended vs hospital telemonitored polysomnography in suspected obstructive sleep apnea syndrome: a randomized crossover trial

STUDY OBJECTIVE: To compare home unattended polysomnography (H-PSG) with polysomnography performed in a local hospital and telemonitored by a sleep laboratory (T-PSG) in the diagnosis of obstructive sleep apnea syndrome (OSAS). DESIGN: Randomized crossover trial. PATIENTS: Ninety-nine patients with suspected OSAS who underwent H-PSG and T-PSG on 2 consecutive nights, according to a randomized order. MEASUREMENTS: H-PSG and T-PSG were compared in terms of (1) effectiveness, only recordings providing interpretable signals from at least one EEG, the electro-oculograph, the electromyograph, air flow, thoracic or abdominal movements, and arterial oxygen saturation for 180 min of sleep were considered to be effective; (2) patient preference assessed by a questionnaire; and (3) polysomnographic indexes and final interpretative results in patients for whom both recordings were legible. RESULTS: Recordings were considered to be ineffective in 11.2% of T-PSG (95% confidence interval [CI], 4.9 to 17.4%) and in 23.4% of H-PSG (95% CI, 19.12 to 27.68%). Thermistor problems were the main cause of failure of H-PSG. Forty-one percent of patients preferred H-PSG, and 55% preferred T-PSG. H-PSG and T-PSG did not differ in terms of sleep and respiratory indexes in the 65 patients in whom both recordings were legible. H-PSG and T-PSG were concordant in 58 of 65 patients using a 10-event-per-hour apnea-hypopnea index cutoff value for the diagnosis of OSAS. CONCLUSIONS: T-PSG is clearly superior to H-PSG from a technical point of view and tends to be preferred by patients. The site of recording (home vs hospital) has no influence on polysomnographic indexes.