Sustained complete remission of metastatic dermatofibrosarcoma protuberans with imatinib mesylate

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor which may recur locally and rarely causes metastases to vital organs. DFSPs have specific chromosomal abnormalities involving the platelet-derived growth factor beta-chain locus (PDGFB) which may render these tumors responsive to targeted therapy with the tyrosine kinase inhibitor imatinib mesylate. A patient with locally recurrent and metastatic DFSP resistant to first-line chemotherapy was treated with imatinib mesylate 400 mg/day. The tumor was examined by a novel fluorescence in situ hybridization (FISH) method for specific rearrangements of the PDGFB locus. The patient was followed for response and toxicity by physical examination and imaging studies. FISH revealed PDGFB rearrangement indicative of multiplication of the PDGFB fusion locus within a ring chromosome. Physical examination showed response within the first month of treatment, and subsequent computed tomography and fluorodeoxyglycose positron emission tomography documented complete response to imatinib therapy. Our patient is now in sustained complete remission for 20 months with minimal toxicity. We conclude that sustained complete remission of metastatic DFSP with specific FISH abnormalities involving the PDGFB locus can be obtained with imatinib mesylate with minimal toxicity for the patient.     

Pars plana vitrectomy in patients with intermediate uveitis

Objective: To describe the effect of pars plana vitrectomy in patients with intermediate uveitis. Methods: Retrospective analysis of the clinical course and visual outcome following pars plana vitrectomy in patients with intermediate uveitis. Results: Thirty-two patients (43 eyes) were included in the study. Pars plana vitrectomy was combined with cataract surgery in 22 of 43 eyes. The intermediate uveitis was associated with sarcoidosis in 16 eyes and multiple sclerosis in five eyes, and was idiopathic in 22 eyes. The mean (±SD) follow-up was 45.6 (±38) months (range: 6–146 months). In 19 of 43 eyes (44.1%), there was improvement in the course of uveitis, allowing the discontinuation of immunosuppressive treatment in seven patients. Cystoid macular edema resolved in 12 of 37 eyes (32.4%). Forty of 43 eyes achieved a better or retained their initial visual acuity. The remaining three eyes deteriorated by two or more lines in the Snellen chart due to the progression of cataract, chronic cystoid macular edema, and glaucomatous optic atrophy, respectively. Conclusions: The results of this study suggest that pars plana vitrectomy may have a beneficial effect on the course of uveitis and the associated complications of cystoid macular edema, thereby reducing the need for long-term immunosuppression. Pars plana vitrectomy combined with simultaneous cataract surgery can improve the visual outcome in these patients.     

Long-term immunosuppressive treatment of serpiginous choroiditis

Objective: To determine the efficacy of immunosuppressive treatment in serpiginous choroiditis. Design: The clinical courses were reviewed of six consecutive patients (12 eyes) with vision-threatening, steroid-dependent/resistant serpiginous choroiditis treated with a combination of immunosuppressive agents including azathioprine, cyclosporine, and cyclophosphamide. All patients underwent treatment for at least 12 months. Results: The follow-up period ranged from 17 to 105 months (mean 57, median 43). All patients were able to taper oral steroids. Five patients discontinued all immunosuppressive medications after a treatment period of 12 to 69 months (mean 39 months). Immunosuppressive treatment was continued in one patient at a ‘low’ maintenance dose. Ten eyes had improved visual acuities, while vision remained impaired in two due to macular scars. Recurrence was noted in two patients when an attempt was made to decrease the dose of immunosuppressive medication. Two patients experienced side effects which were reversed by decreasing the dose of the medications. Conclusion: Long-term immunosuppressive treatment appears to prolong remission and preserve vision in patients with serpiginous choroiditis.     

Outcomes of Treatment with Immunomodulatory Therapy in Patients with Corticosteroid-Resistant Juvenile Idiopathic Arthritis-Associated Chronic Iridocyclitis

Purpose: To describe the clinical outcome of patients with juvenile idiopathic arthritis (JIA)-associated chronic iridocyclitis unresponsive to conventional therapy, in whom inflammation was eventually controlled using immunomodulatory therapy (IMT), and to determine if patients treated early with IMT have better visual acuity outcomes than those treated with corticosteroid alone. Methods: Patients with JIA-associated chronic iridocyclitis receiving immunomodulatory therapy at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary between 1981 and 2001 were studied. Inclusion criteria: JIA with chronic corticosteroid-dependent uveitis; IMT for a minimum of one year; minimum follow-up of three years; inflammation eventually controlled for a minimum of six consecutive months by IMT. Results: At last visit, 51% of eyes (23 eyes) had acuity of 20/20 to 20/40, 16% (7 eyes) had 20/50 to 20/100, and 33% (15 eyes) had 20/200 or less. In patients whose chronic inflammation was eventually controlled within three years from onset of the uveitis, final bilateral vision was maintained within 20/20 to 20/30, except for a patient whose one eye had poor vision at initial consultation. When acuities at the endof the follow-up period of patients treated early with IMT were compared with acuities at the initial consultation of patients treated late with IMT, with duration of uveitis matched for each patient, visual acuities of those treated early were statistically significantly better than those treated late with IMT (p < 0.005 right and left eyes pooled; p = 0.0075 best eyes; p = 0.0375 worst eyes). Conclusions: We noted improvement or maintenance of visual acuity (86%) during the course of follow-up of patients with treatment-resistant JIA-associated uveitis treated with effective IMT. However, only IMT given early in the disease course was noted to be associated with bilateral visual acuity of 20/30 or better.     

Baseline plasma N-terminal pro-B-type natriuretic peptide is associated with the extent of stress-induced myocardial ischemia during dobutamine stress echocardiography

OBJECTIVE: To determine the relationship between baseline plasma N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels and the presence and extent of myocardial ischemia during dobutamine stress echocardiography (DSE). METHODS: NT-proBNP was measured in 170 consecutive patients prior to DSE. Rest wall motion abnormalities (RWMAs) and new wall motion abnormalities (NWMAs) were scored using a 5-point, 17-segment model. Kruskal-Wallis tests were applied to study differences in NT-proBNP levels between patients with normal DSE, RWMAs but no NWMAs, and NWMAs, and (in patients with NWMAs) between those with 1-2, 3-4 and >4 ischemic segments. Univariate and multivariate regression analyses were used to determine the value of NT-proBNP in predicting NWMAs. RESULTS: The median NT-proBNP level was 110 ng/l (interquartile range: 42-389 ng/l). Median NT-proBNP was 59, 321 and 440 ng/l in patients with normal DSE, with RWMAs but no NWMAs, and with NWMAs, respectively (P<0001). Among patients with NWMAs, median NT-proBNP was associated with the number of ischemic segments: 364, 710 and 2376 ng/l in patients with 1-2, 3-4 and >4 ischemic segments, respectively (P<0.001). Elevated NT-proBNP levels were significantly associated with NWMAs (odds ratio per 100 ng/l increase: 1.14, 95% confidence interval: 1.1-1.2) in a multivariate analysis of clinical baseline variables and RWMAs. CONCLUSION: Elevated baseline levels of NT-proBNP are associated with the presence and extent of myocardial ischemia during DSE, independent of the presence of RWMAs.     

Short-term effect of orlistat on dietary glycotoxins in healthy women and women with polycystic ovary syndrome

Exogenous advanced glycation endproducts (AGEs, known atherogenic molecules) abundant in everyday precooked, rich in fat, overheated meals can possibly contribute to the increased risk for diabetes and cardiovascular disease in women with polycystic ovary syndrome (PCOS). The aim of the present study was to investigate the effect of a lipase inhibitor on absorbed food glycotoxins in healthy women and those with PCOS. A 2-day protocol was followed. In the first day, a meal rich in AGE was provided, which on the second day was followed by two 120-mg capsules of lipase inhibitor, orlistat. Serum AGE levels were evaluated at baseline (0 hours), and at 3 and 5 hours postmeal during the study. Thirty-six women were studied, 15 controls (mean age, 28.80 +/- 5.47 years; body mass index, 25.85 +/- 6.73 kg/m(2)) and 21 with PCOS (mean age, 25.29 +/- 5.06 years; body mass index, 30.40 +/- 7.51 kg/m(2)) (University Hospital, Athens, Greece, institutional practice). Serum AGE levels, on day 1, were significantly increased both in the control group and in the PCOS group as compared with basal values (control group, 14.1%; PCOS group, 6.0%; P < .001). The corresponding rise was significantly lower on day 2 when the same meal was combined with orlistat (control group, 4.1%; PCOS group, 2.0%; P < .01). A limitation of the study is that it is a nonplacebo, nonrandomized therapeutic trial where each subject is considered as its own control. In conclusion, this study demonstrated the beneficial effect of orlistat on the absorption of food glycotoxins.     

Anomalous origin of right coronary artery: magnetic resonance angiography and viability study

OBJECTIVE: To detect origin and course and to evaluate viability in patients with anomalous RCA. DESIGN: 3D coronary MR angiography and viability study using gadolinium-enhanced magnetic resonance imaging (Gd-MRI) was performed. SETTING: A tertiary hospital center. PATIENTS: Four patients, selected from the catheter lab, were studied. RESULTS: Anomalous RCA from the left sinus of Valsalva was identified in all patients. Inferior myocardial infarction was documented in three patients. CONCLUSIONS: Magnetic resonance imaging can non-invasively identify anomalous RCA and perform viability study in the same examination.     

Patients with duodenal ulcer have lower levels of serum cholesterol compared to other dyspeptic patients independently of Helicobacter pylori status

Objective. The association between Helicobacter pylori (H. pylori) infection and serum lipid profile is still controversial. The aim of this study was to determine any possible relationship between H. pylori infection and the lipid profile of patients with upper gastrointestinal symptoms. Material and methods. Consecutively selected 20–70 year-old dyspeptic patients who had undergone esophagogastroduodenoscopy were evaluated for H. pylori infection using both the CLO test and Giemsa staining. Serum total cholesterol (C), HDL-C, LDL-C, apo-A₁, apo-B and triglyceride levels were measured. Results. A total of 137 patients (median age 52.0 years) were studied. Total cholesterol levels were lower in H. pylori-infected patients than in H. pylori-negative patients (mean±SEM: 199.3±5.9 versus 212.6±4.6 mg/dl, p=0.08). Patients with duodenal ulcer (DU) had significantly lower levels of all measured lipidemic parameters including cholesterol, with the exception of triglycerides, in comparison with either H. pylori-positive or -negative dyspeptic patients (cholesterol: 177.6±6.5 versus 214.6±4.2 mg/dl, p<0.0001). However, there was no difference in the total cholesterol/HDL-C ratio between DU patients and the rest of the dyspeptic patients. Conclusions. Among H. pylori-positive and H. pylori-negative patients there was no difference in lipid profile apart from a trend towards total cholesterol levels being lower in H. pylori-positive patients. However, cholesterol, HDL-C, LDL-C, apo-A and apo-B were all decreased in DU patients even though this reduction did not result in a fall in the total cholesterol/HDL-C ratio. The etiologic factor differentiating the lipid profiles among dyspeptics only in H. pylori-positive patients carrying a DU could be dietetic, microbial, genetic or a combination of all three.