Multicavitary ciliary body melanoma presenting as a cyst.

Cyst-like cavities in uveal melanoma occur rarely and can simulate a benign intraocular cystic lesion resulting in delayed diagnosis and inappropriate management. Herein, we describe a 66-year-old Caucasian female who presented with a "cystic" ciliary body mass in the right eye oculus dexter (OD). Slit lamp examination OD showed anterior bulging of the iris temporally from an underlying pigmented ciliary body mass and transillumination disclosed slight shadow from the tumor. Ultrasound biomicroscopy (UBM) revealed multiple cyst-like cavities within a tumor, lined by "thick walls" of at least 200 µm and occupying 80% of the tumor volume. A clinical diagnosis of multi-cavitary ciliary body melanoma was suspected and partial lamellar sclero iridocyclectomy was performed. Histopathology confirmed the diagnosis of low-grade spindle melanoma of the ciliary body with multiple empty and fluid filled cyst-like cavities without epithelial lining. UBM is an important diagnostic tool in the differentiation of "thick walled" cavitary melanoma from "thin walled" benign pigment epithelial cyst.

Hemoglobin A1c as a measure of disease severity and outcome in limb threatening ischemia.

BACKGROUND: Diabetes mellitus is a known risk factor in the development of peripheral vascular disease. Hemoglobin A1c (HbA1c) has been used by clinicians as a means to measure short to intermediate term glucose control in diabetics. Trials evaluating tight glucose control using HbA1c measurements have recently been conducted for several medical conditions. The goal of this study is to determine if the level of hemoglobin A1c has any effect on disease severity in diabetic patients with limb threatening ischemia. METHODS: A retrospective review of all patients presenting with limb threatening ischemia between January 1 and December 31, 2007 was conducted. All patients underwent conventional arteriography prior to intervention. Of 148 patients, 73 were diabetics with a hemoglobin A1c level performed within 3 mo of presentation. Patients were placed into high (>7) and low (<7) hemoglobin A1c groups and data was collected on type of presentation, comorbidities, anatomic level of disease, tibial artery patency, need for amputation, contralateral disease, need for an open versus an endovascular procedure, and freedom from intervention. Fisher's exact t-test was used to compare the two groups. A P value <0.05 was considered statistically significant. RESULTS: Thirty-six patients had HbA1c levels above 7.0 and 37 had levels below 7.0 (mean 7.64 ± 2.04, range 5.1-14.7). There were no statistically significant differences in the two groups in comorbities, average age, initial gangrene at presentation, aspirin or statin use, or smoking status. Patients in the high group were more likely to have had a previous attempt at revascularization (23 versus 11, P = 0.0049). There was no difference in the presence of contralateral disease (7 versus 4, P = 0.3447) or in the number of patent tibial vessels. Patients with low HbA1c levels were more likely to have the peroneal artery affected (17 versus 8, P = 0.048). In addition, TASC II classifications of iliac and femoral popliteal disease was similar between the two groups. Freedom from intervention is depicted graphically by life table analysis. CONCLUSION: Glucose control measured by hemoglobin A1c does not appear to affect severity of disease or need for reintervention in diabetics with limb threatening ischemia. This suggests other factors related to diabetes may play a role in peripheral vascular disease. Larger, prospective studies are needed to assess the affect of glucose control in limb threatening ischemia.

The contribution of disease activity on functional limitations over time through psychological mediators: a 12-month longitudinal study in patients with ankylosing spondylitis.

OBJECTIVES: To explore whether helplessness, internality and depression would mediate the relationship between disease activity and functional limitations in patients with AS in a 12-month longitudinal study. METHODS: A total of 294 participants with AS meeting modified New York criteria completed clinical and psychological assessments at 6-month intervals. Psychological measures evaluated helplessness, depression and internality. Path analysis evaluated the direct and indirect effects of baseline disease activity on 12-month functional limitations via the psychological measures of helplessness, internality and depression at 6 months. RESULTS: Baseline disease activity demonstrated direct and indirect effects on 12-month functional limitations. Helplessness and depression, but not internality, served as mediators of the relationship between disease activity and functional limitations. CONCLUSION: Higher baseline disease activity predicted greater functional limitations at 12 months through helplessness and depression. Our findings suggest that helplessness and depression may constitute future treatment targets in reducing functional limitations in patients with AS.

Ankylosing spondylitis: patterns of radiographic involvement--a re-examination of accepted principles in a cohort of 769 patients.

PURPOSE: To re-examine the patterns of radiographic involvement in ankylosing spondylitis (AS). MATERIALS AND METHODS: This prospective study had institutional review board approval, and 769 patients with AS (556 men, 213 women; mean age, 47.1 years; age range, 18-87 years) provided written informed consent. Radiographs of the cervical spine, lumbar spine, pelvis, and hips were scored by using the Bath Ankylosing Spondylitis Radiology Index (BASRI) by an experienced radiologist. Differences in sacroiliitis grade between right and left sacroiliac joints, frequency of cervical- and lumbar-predominant involvement by sex, frequency of progression to complete spinal fusion, and association between hip arthritis and spinal involvement were computed for the cohort overall and for subgroups defined according to duration of AS in 10-year increments. RESULTS: Symmetric sacroiliitis was seen in 86.1% of patients. Lumbar predominance was more common during the first 20 years of the disease, after which the cervical spine and lumbar spine were equally involved. Men and women were equally likely to have cervical-predominant involvement. Complete spinal fusion was observed in 27.9% of patients with AS for more than 30 years and in 42.6% of patients with AS for more than 40 years. Patients with BASRI hip scores of 2 or greater had significantly higher BASRI spine scores. CONCLUSION: There were no sex differences in cervical-predominant involvement in AS. Hip arthritis was strongly associated with worse spinal involvement.

Diabetes due to a progressive defect in beta-cell mass in rats transgenic for human islet amyloid polypeptide (HIP Rat): a new model for type 2 diabetes.

The islet in type 2 diabetes is characterized by a deficit in beta-cell mass, increased beta-cell apoptosis, and impaired insulin secretion. Also, islets in type 2 diabetes often contain deposits of islet amyloid derived from islet amyloid polypeptide (IAPP), a 37-amino acid protein cosecreted with insulin by beta-cells. Several lines of evidence suggest that proteins with a capacity to develop amyloid fibrils may also form small toxic oligomers that can initiate apoptosis. The amino acid sequence of IAPP in rats and mice is identical and differs from that in humans by substitution of proline residues in the amyloidogenic sequence so that the protein no longer forms amyloid fibrils or is cytotoxic. In the present study, we report a novel rat model for type 2 diabetes: rats transgenic for human IAPP (the HIP rat). HIP rats develop diabetes between 5 and 10 months of age, characterized by an approximately 60% deficit in beta-cell mass that is due to an increased frequency of beta-cell apoptosis. HIP rats develop islet amyloid, but the extent of amyloid was not related to the frequency of beta-cell apoptosis (r = 0.10, P = 0.65), whereas the fasting blood glucose was (r = 0.77, P < 0.001). The frequency of beta-cell apoptosis was related to the frequency of beta-cell replication (r = 0.97, P < 0.001) in support of the hypothesis that replicating cells are more vulnerable to apoptosis than nondividing cells. The HIP rat provides additional evidence in support of the potential role of IAPP oligomer formation toward the increased frequency of apoptosis in type 2 diabetes, a process that appears to be compounded by glucose toxicity when hyperglycemia supervenes.