Retinal arteriovenous communication in retinitis pigmentosa with Refsum's disease-like findings

In this report we describe, herewith, a patient with primary pigmentary dystrophy of the retina (retinitis pigmentosa) associated with unilateral retinal arteriovenous communication and exudative retinal detachment. The patient had complete resolution of the retinal detachment following laser photocoagulation treatment. Such association has not been previously reported.     

AC/A ratios in myopic and emmetropic Hong Kong children and the effect of timolol§

Purpose: Caucasian children with myopia have elevated response accommodative vergence to accommodation (AC/A) ratios. The purpose of this study was twofold: to determine if response AC/A ratios vary with refractive error and with myopic progression rate in Hong Kong Chinese children, and to determine the effect of beta‐adrenergic antagonism with topical timolol application on AC/A ratios. Methods: Thirty children aged eight to 12 years participated in the study. All refractive errors were corrected with spectacle lenses. Accommodative responses were measured using a Shin‐Nippon autorefractor and concurrent changes in vergence were assessed using a vertical prism and a Howell‐Dwyer card at three metres and 0.33 metre. Accommodative demand was altered using plus or minus two dioptre lenses and lens‐ and distance‐induced response AC/A ratios were calculated. Measurements were repeated 30 minutes after the instillation of topical timolol maleate (0.5 per cent). Results: AC/A ratios appeared higher in progressing myopic children but the difference was not statistically significant. Timolol application reduced accommodative convergence (AC) in the stable myopes (reduction = ‐3 ± 1.14^A) but not in the emmetropes (0.69 ± 0.9^P) or progressing myopes (0.16 ± 0.43^A) and this difference between refractive groups was statistically s ignificant (F^2,27= 3.766; P= 0.036). However, timolol did not produce a significant change in the accommodative response to positive or negative lenses or response AC/A ratios. Conclusions: We did not find that AC/A ratios in myopic Chinese children were elevated and therefore, it is unlikely that elevated AC/A ratios are responsible for the high levels of myopia that occur in Hong Kong. The finding that timolol reduced AC in the stable myopes suggests that the autonomic control of accommodative convergence in these children may be different from that in emmetropic children and those with progressing myopia.     

Activation of guinea pig eosinophil respiratory burst by leukotriene B4: role of protein kinase C

Leukotriene B4 (LTB4) and the protein kinase C activator, 4-beta-phorbol dibutyrate (PDBu), both induced a pronounced and concentration-dependent stimulation of hydrogen peroxide (H2O2) generation by purified guinea pig peritoneal eosinophils in the concentration range 1 nM-1 microM. The LTB4 response was inhibited competitively by the specific LTB4 receptor antagonist, U-75302, with a KB of 25 nM, while the concentration-response curves for both stimuli were shifted rightwards (3.8-fold and 2.8-fold for LTB4 and PDBu, respectively) by the competitive protein kinase C inhibitor, 1-O-hexadecyl-2-O-methylglycerol at a concentration of 300 microM. LTB4 appears, therefore, to induce respiratory burst in eosinophils via a receptor-mediated mechanism involving protein kinase C.     

Diagnostic problems in tissues previously sampled by fine-needle aspiration.

Unusual tissue changes in needle tracts after fine-needle aspiration were studied in two specimens. One specimen, a 3.0 x 2.5 cm mesenteric nodule, was found six days after a nondiagnostic transabdominal fine-needle aspiration of a retroperitoneal mass. At frozen-section examination, the nodule had proliferating spindle-shaped cells interspersed with adipocytes that raised the possibility of well-differentiated liposarcoma. On permanent sections, however, there was fat necrosis and reactive spindle-shaped cells that immunohistochemically were consistent with myofibroblasts. In the second case, a needle tract was noted in breast tissue on which a biopsy was performed two days after a cytologic diagnosis of carcinoma. The 0.4-mm-wide tract contained neutrophils, foamy histiocytes, and clumps of intact, atypical epithelial cells without a desmoplastic or elastotic component. Aside from these epithelial cells, nothing in the biopsy specimen suggested invasive carcinoma. The cells were identical to those from a comedocarcinoma present in the biopsy and mastectomy specimens. These two cases provide examples of pitfalls that may arise during the examination of biopsy specimens obtained after fine-needle aspiration.     

Characterization of the procoagulant chain derived from human high molecular weight kininogen (Fitzgerald factor) by human tissue kallikrein

Human high molecular weight kininogen (HMWK), a single-chain protein with mol wt 120,000, is cleaved by human urinary kallikrein (HUK) to release kinin from within a disulfide loop and form a two-chain protein that retains all the procoagulant activity of the native molecule. Cleavage of HMWK by HUK is associated with a reduction in size to mol wt 115,000, as assessed by SDS-PAGE of unreduced protein, whereas the two chains of the reduced protein present together as a single broad band with mol wt 64,000. The 64,000 chain with procoagulant activity was chromatographically separated from the nonfunctional chain of similar size. The homogeneous procoagulant chain had an amino acid composition similar to that of smaller procoagulant ("light") chains isolated by others upon cleavage of HMWK with plasma kallikrein and elicited an antiserum that was monospecific by Ouchterlony analysis and inhibited the procoagulant function of HMWK. Thus, the limited proteolysis of HMWK by HUK has permitted, for the first time, the isolation of a stable procoagulant chain that is equal in size to the nonfunctional chain. The common terminology of "heavy" and "light" chain for kinin-free kininogen obtained with plasma kallikrein reflects the continued degradation of the procoagulant carboxyterminal chain and is not appropriate for the initial two-chain product formed when kinin is released from HMWK. It is proposed that the initial cleavage products of HMWK be designated the A-chain, the B-fragment, and the C- chain, representing the amino-terminal chain, the released vasoactive peptide containing the bradykinin sequence, and the carboxy-terminal procoagulant chain, respectively. Thus, intact HMWK would contain, in sequence, A, B, and C regions.     

Climatic droplet keratopathy with corneal amyloidosis

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.     

Three-dimensional intravascular ultrasound imaging of normal and diseased canine and human arteries.

This study reports three-dimensional reconstruction of two-dimensional intravascular ultrasound images obtained along 5 cm vessel segments. Each three-dimensional image was produced by computerized "stacking" of a set of consecutive two-dimensional images (mode 90 images per set; range 32 to 256). Three-dimensional images (n = 26) were obtained from 11 human normal and atherosclerotic arteries (three in vitro and eight in vivo) and five in vivo canine studies. In vivo human examinations included three iliac, one deep, and three superficial femoral arteries and one aortic dissection. Five in vivo canine vessels (three iliac stenoses and two aortic dissections) were imaged before and after intraluminal stent deployment. Images were displayed on a gray-scale monitor, allowing examination of vessel images as complete cylinders or longitudinal hemisections in any user-defined plane. This enabled global examination of vascular segments and identified luminal shape, wall thickness, and distribution and morphology of plaques. Reconstructions of aortic dissections illustrated the extent of the dissection and produced an anatomic picture of the false lumen. Three-dimensional imaging enhanced stent deployment by identifying the site for deployment, dimensions of the vessel lumen, and changes in morphology after stent insertion. There was good correlation in vessel dimensions measured by angiography, two-dimensional intravascular ultrasonography and longitudinal gray-scale reconstructions (r = 0.74 to 0.95; p = 0.34 to 0.001) but poor correlation with measurements from three-dimensional surface-rendered images (r = 0.13 to 0.48; p = 0.42 to 0.87). We conclude that three-dimensional intravascular ultrasound imaging is an innovative new method for identifying the type, extent, and spatial configuration of arterial disease, with promising diagnostic and therapeutic applications.