Visual results in children treated for macular retinoblastoma.

PURPOSE: To evaluate the results of patching treatment in children with macular retinoblastoma in one eye. METHODS: Fifteen children affected by macular retinoblastoma received instructions for patching treatment for amblyopia. Data were collected on age at diagnosis of the tumor, presence of unilateral or bilateral disease, area of posterior pole involvement by the scar of the regressed tumor and its relationship to the fovea; and the onset, duration, and compliance of patching. The visual acuities recorded were expressed in logMAR (logarithm minimum angle of resolution) equivalents. RESULTS: Twelve children (80%) had bilateral retinoblastoma with the macular involved in one eye and three children had unilateral macular tumors. The median age at which patching was initiated was 15 months (range 4-36). Compliance to patching was good in 80% of children, with a median duration of 4 h (range 0.5-8) per day, 7 days per week, with total occlusion of the better eye. The median percentage of posterior pole involvement was 34% (range 11-100%). Eighty percent of children had some improvement in their visual acuity, and of the children in whom final logMAR acuity was recorded, 73% had an acuity of 1.0 logMAR or better and 53% an acuity of 0.5 logMAR or better after patching. There was no evidence of association between age of patient, sex, duration of patching, or percentage of posterior pole involvement and the improvement in visual acuity. CONCLUSIONS: In spite of the macular involvement of eyes with retinoblastoma, some visual recovery was achieved in 80% of children. Hence a trial of patching therapy is recommended for all children with involvement of the macula by retinoblastoma.

Clinicopathological correlation of congenital corneal opacification using ultrasound biomicroscopy.

AIM: To investigate the correlation between clinical, high frequency ultrasound biomicroscopy (UBM) and, where possible, histological findings in cases of congenital corneal opacification presenting to the departments of ophthalmology, Great Ormond Street Hospital for Children, London, and the Hospital for Sick Children, Toronto, Canada. METHOD: 22 eyes of 13 children (age range 3-225 days) with congenitally opaque corneas were examined. UBM was performed using the ultrasound biomicroscope (Allergan-Humphrey). All eyes underwent penetrating keratoplasties (PKP) except five. The host corneas were all sent for histological examination. RESULTS: The final diagnosis in our series was Peters' anomaly in nine cases (70%), corneal dystrophy in two cases (15%), and sclerocornea in two cases (15%). The UBM findings changed the clinical diagnosis in five cases (38%). In these five cases histology was available in four and confirmed the UBM diagnosis in each case. In no case of the 13 where histology was available did it contradict the UBM findings. In two cases a hypoechoic region in the anterior stroma was seen on UBM which correlated histologically with absent Bowman's layer and oedema. In two cases UBM revealed aniridia and in one, congenital aphakia, which was not apparent clinically. CONCLUSION: UBM examination is not only very useful in evaluating the clinical diagnosis in congenital corneal opacification, it also acts as a preoperative guide in cases undergoing PKP by detecting keratolenticular and iridocorneal adhesions and other ocular abnormalities such as aniridia and congenital aphakia. In all cases where PKP was performed the UBM diagnosis was confirmed histologically. The clinical diagnosis was incorrect in five cases. This has important implications in studies of phenotype/genotype correlation of congenital corneal opacification.

Ultrasound biomicroscopy of the eye in cystinosis.

OBJECTIVE: To describe the ocular ultrasound biomicroscopy (UBM) findings in patients with cystinosis. METHODS: Six patients with infantile nephropathic cystinosis, aged 16 to 25 years, and 6 controls (matched for age and spherical refractive error) were examined clinically and with UBM. Scleral reflectivity, corneal and iris thickness, central anterior chamber depth, angle width, trabecular meshwork to ciliary process distance, and ciliary sulcus width were measured. RESULTS: No patient had glaucoma or posterior synechiae, but all had crystals in the trabecular meshwork apparent with gonioscopy. Using UBM, the cornea and iris appeared similar in both groups, but the scleral reflectivity was increased in patients (P =.003). The angle was narrower in patients (mean +/- SD, 20 degrees +/- 7 degrees ) than controls (31 degrees +/- 5 degrees, P<. 001). The anterior chamber was shallower in patients (2556 +/- 197 microm) than controls (2968 +/- 284 microm, P<.001). The ciliary sulcus was closed or narrow in all patients (83 +/- 112 microm) compared with controls (339 +/- 135 microm, P<.001), with a reduction in the trabecular meshwork to ciliary process distance. CONCLUSIONS: This report of ocular UBM findings in cystinosis demonstrated narrowing of the angle and a ciliary body configuration similar to that reported for plateau iris syndrome. Gonioscopy demonstrated crystals in the trabecular meshwork. These findings may explain the predisposition of these patients to glaucoma.

High-frequency ultrasonography findings in persistent hyperplastic primary vitreous.

PURPOSE: To identify anatomic correlates in eyes with persistent hyperplastic primary vitreous (PHPV) by using high-frequency ultrasonography. METHOD: Three main groups of patients were studied by means of high-frequency ultrasonography over a 19-month period. Group I included 9 eyes of 9 patients with newly diagnosed unilateral PHPV. Group II included 4 eyes of 4 patients with unilateral PHPV that had been previously surgically treated. Group III included 22 eyes and was a control group of patients without PHPV. This group consisted of the 5 contralateral normal eyes of 5 patients with unilateral PHPV in the fellow eye, both eyes of one patient with uncomplicated unilateral cataracts, the affected eye of one patient with unilateral uncomplicated cataract, the affected eye of one patient with isolated retinal coloboma, and 13 normal eyes of 7 young adults. Group I and II patients also had B-scan ultrasonography performed and had any intraoperative findings noted. RESULTS: Characteristic features of PHPV, such as centrally dragged ciliary processes and swollen anteriorly displaced lens, were observed only in those eyes with PHPV. A new echographic finding of a double linear echo was observed in the region of the pars plana or plicata only in eyes with PHPV. This finding was confirmed intraoperatively to be consistent with a thickened adherent anterior hyaloid face and not to be an anteriorly inserted peripheral retina. CONCLUSION: High-frequency ultrasound can be reliably used to distinguish characteristic features of PHPV. To our knowledge this is the first such description of the use of high-frequency ultrasonography in PHPV eyes. Furthermore, the presence of a thickened adherent anterior hyaloid face may help explain the well-recognized complications of peripheral retinal tears and retinal detachments during and after surgical intervention.

Plasma interleukin 2 receptor levels in renal allograft recipients.

Shed/soluble interleukin 2 receptor (IL2R) was measured by an enzyme-linked immunosorbent assay (ELISA) in serial samples of plasma from 32 patients with renal allografts. Patients on chronic dialysis (pretransplant) had elevated IL2R levels which fell toward normal after transplantation. Patients with acute rejection and viral infection had significantly higher levels of plasma IL2R than did patients with stable renal function or with cyclosporine nephrotoxicity (all P less than 0.005). Acute renal failure from other causes (renal artery stenosis, hemolytic-uremic syndrome) did not have a comparable rise in IL2R. The assay of shed/soluble IL2R may have diagnostic value in the clinical management of allograft recipients, a possibility that deserves further clinical evaluation.

Dissemination of gonococcal infection is associated with delayed stimulation of complement-dependent neutrophil chemotaxis in vitro.

Gonococci isolated from patients with uncomplicated gonorrhea or disseminated infection were examined for their ability to stimulate neutrophil chemotaxis in vitro. A neutrophil chemotactic response was not observed when as many as 10(9) colony-forming units of gonococci were incubated in buffer alone. However, a striking response was observed when 4 x 10(7) colony-forming units were incubated in 10% pooled normal human serum. Activation of complement was required for chemotaxis as demonstrated by complement consumption and failure of chemotactic activity generation in serum treated with heat or EDTA. Chromatography of activated serum demonstrated a single peak of chemotactic activity with an apparent molecular weight of 15,000 and was shown to be due to C5a. Examination of the kinetics of chemotactic factor generation demonstrated that local isolates stimulated a rapid response (about 60% maximal in 5 min), whereas the response to disseminated isolates was delayed (50% maximal in 20 to 30 min). Chemotactic activity generated by both types of isolates was suppressed at early time periods in agammaglobulinemic serum, indicating that immunoglobulins contribute to the generation of activity. Both pathways of complement activation were utilized by the two types of gonococci, but there was preferential dependence on the alternative pathway for disseminated strains and on the classical pathway for local isolates. We suggest that delayed stimulation of complement-dependent neutrophil migration may account in part for the infrequency of genital symptoms and may contribute to the mechanism of dissemination in patients with systemic gonococcal infection.

Photochemical cross-linking of initiation factor-3 to Escherichia coli 30 S ribosomal subunits.

Initiation factor-3 has been photochemically cross-linked to Escherichia coli 30 S ribosomal subunits by means of near-ultraviolet (> 285 nm) irradiation. The cross-linking was judged to be specific since noncovalent binding was required for subsequent cross-linking and was prevented by the presence of 0.5 M NH4Cl or 0.5 mM aurintricarboxylic acid. Covalent attachment reached its maximum level of 8.5 to 11% of the noncovalently bound IF-3 after 60 min of irradation. Cross-linking was unaffected by the presence of the photosensitizer, acetone (5 or 10%), but was reduced to 30 to 40% of its maximum level by addition of 5 mM dithiothreitol, a free radical scavenger. Analysis of the 14C-labeled IF-3 x 30 S subunit covalent complex revealed that the IF-3 was distributed between the protein and RNA of the subunits in a 3:1 ratio. The target proteins have been identified as S7, S11, S12, S18, and S21 by immunochemical techniques.