Background. Although cytomegalovirus (CMV) retinitis (CMVR) is a well-recognized complication after allogeneic hematopoietic stem cell transplantation (HSCT), standard operating procedures for ...ophthalmic monitoring are variable. In particular, authors perceived a greater risk of CMVR after pediatric HSCT for inherited immunodeficiencies, in patients who often have pretransplantation viremia. This study was therefore performed to identify high-risk pediatric HSCT recipients who would benefit from regular ophthalmic monitoring. Methods. During a 5-year study period, we retrospectively analyzed findings in 56 of 304 consecutive HSCT recipients (age range, 0.5–197 months) in whom significant CMV viremia developed (CMV level at PCR, ≥4000 copies/mL). All HSCT recipients with significant CMV viremia underwent retinal examination weekly (inpatients) or every other week (outpatients), with examinations performed by a skilled ophthalmologist. Results. CMVR developed in 13 (4%) of 304 HSCT recipients, 23% (13 of 56) of those with significant CMV viremia. Pretransplant viremia (odds ratio, 11.3; P<.01), acute (grade ≥2) graft-vs-host disease (odds ratio, 8.2; P < .02) and mismatched graft (odds ratio, 8; P < .02) were identified as independent risk factors. Compared with other invasive CMV diseases, CMVR was more often a late-onset disease, occurring at a median of 199 days after HSCT. At diagnosis, a significantly higher CD4 T-cell count (≥200/μL; P < .03) and a lower CMV load (P < .004) was observed in children with CMVR, compared with those in whom lung, gut, or liver CMV disease developed. Conclusions. We report an increased risk of CMVR in high-risk pediatric HSCT recipients. This form of CMV disease differs from other invasive CMV disease in its relationship to immune reconstitution and viral dynamics. We have studied the relationship between these variables and suggested a risk-stratified ophthalmic screening strategy.
To assess the outcome of early therapeutic penetrating keratoplasty (PKP) for corneal melt leading to perforation in children.
Case notes of all the consecutive patients presenting with acute corneal ...perforation that underwent urgent therapeutic PKP between 2000 and 2010 to the practice of one of the authors, both NHS at Great Ormond Street Hospital for Children and private, were retrospectively reviewed. Onset of perforation, underlying cause, medical and surgical treatment, pre- and post-operative visual acuity, graft clarity, length of follow-up, and post-operative complications were recorded.
Four eyes of four consecutive patients (mean age of 9.5 years and median 8.5 years, range 4-17 years) were treated for acquired acute onset corneal perforations. There were three females and one male. Etiologies included herpes simplex keratitis secondary to immune recovery disease post bone marrow transplantation, acanthamoeba keratitis, recessive dystrophic epidermolysis bullosa, and blepharokeratoconjunctivitis with acne rosacea. Pre-operative visual acuity ranged from hand movements to 6/150. All the patients had severe anterior chamber inflammation. All eyes improved in visual acuity ranging from 6/9 to 6/18 with clear grafts at last follow-up. There was no recurrence of melt or perforation. Mean follow-up was 67 months (median 44 months).
PKP during the acute phase together with aggressive medical therapy and close follow-up may achieve good visual outcomes in children with corneal melt with perforation and should be considered. Waiting may sometimes allow the marked inflammatory response seen in children to cause irreversible structural and/or functional damage.
Abstract Purpose Head and neck rhabdomyosarcoma (HNRMS) survivors are at increased risk of developing pituitary dysfunction as an adverse event of radiotherapy. Our aim was to investigate the ...frequency and risk factors for pituitary dysfunction in these survivors. Secondly, we aimed to compare the prevalence of pituitary dysfunction between survivors treated with external beam radiation therapy (EBRT) and survivors treated with the ablative surgery, moulage technique after loading brachytherapy, and surgical reconstruction (AMORE) procedure. Methods Eighty HNRMS survivors treated in London (EBRT based) and Amsterdam (AMORE based: AMORE if feasible, otherwise EBRT) in the period 1990–2010 and alive ≥2 years post-treatment were evaluated. Survivors were evaluated in multidisciplinary late-effects clinics, with measurement of linear growth, determination of thyroid function, and growth hormone parameters. Additional data, such as baseline characteristics, anthropometrics, pubertal stage, and the results of additional laboratory investigations, were retrieved from patient charts. Results Pituitary dysfunction was diagnosed in 24 in 80 (30%) survivors, after a median follow-up time of 11 years. Median time to develop pituitary dysfunction after HNRMS diagnosis was 3.0 years. Risk factors were EBRT-based therapy (odds ratio OR 2.06; 95% confidence interval CI 1.79–2.46), parameningeal tumour site (OR 1.83; 95% CI 1.60–2.17) and embryonal RMS histology (OR 1.49; 95% CI 1.19–1.90). Conclusions Radiotherapy used for the treatment of HNRMS confers a significant risk of the development of pituitary dysfunction. AMORE-based treatment in children with HNRMS resulted in less pituitary dysfunction than treatment with conventional EBRT. Our findings underscore the importance of routine early endocrine follow-up in this specific population.
The ability of various strains of Coxiella burnetii (C.b.) and their phase I and II lipopolysaccharides (LPSs) to induce tumor necrosis factor alpha (TNF-alpha) in peritoneal Balb/c mouse macrophages ...in vitro was investigated. Considerable differences in the induction ability were observed in dependence on the strain applied. In a TNF-alpha bioassay, the most effective inducers were both corpuscles and LPSs of the strains Priscilla and Scurry, followed by Nine Mile, Luga, and Henzerling I. In contrast, in ELISA, the most effective inducers were LPSs of the strains Luga and Henzerling, followed by Nine Mile, Priscilla, and Scurry. The role of toll-like receptor 4 (TLR4) in the induction was confirmed by the use of C3H/HeJ mouse macrophages. Thus, the induction of TNF-alpha was much higher in Balb/c mouse macrophages than that in TLR4-deficient C3H/HeJ mouse macrophages. Differences in the results of the bioassay and those of ELISA suggest a role of another secreted factor(s) induced with C.b. in murine macrophages that could act synergically with TNF-alpha in L929 cells in the bioassay. The observed differences in TNF-alpha induction might play a role in the pathobiology of Q fever.
There is currently no system of registration for visually impaired children in Slovakia and the current prevalence of visual impairment (VI), low vision and blindness is unknown for this population. ...We propose a template for a process of registration of visually impaired children in Slovakia as well as a system for the Low Vision Health Core for this population.
Based on a literature search, we report our data of the estimated prevalence of VI in children in Slovakia and the number of registered students with VI. We have created a registration form and suggested a template of registration for VI children as well as the Health Care System for this population.
In industrialized countries, the prevalence of VI, including blindness is 10 -22/10,000 in children aged less than 16 years. Extrapolating these figures to the Slovak population, we estimate that there will be between 1500 to 3200 VI children under the age of 19 years. Only 752 students with VI of this age were recorded in Slovakia in 2009/2010. We suggest that three Low Vision Centres for VI children should be adequate to cater for the VI population, each of which should provide all levels of care and that ophthalmologists should register patients with VI by filling the proposed registration form.
The number of VI children in Slovakia appears to be very low. The only way of accurately assessing the prevalence is to introduce a VI registration system into the country, to be carried out by the ophthalmologists. We suggest that the Low vision service provided by the health authority needs to be divided into primary, secondary and tertiary care (including visual rehabilitation by low vision aids). Only if the Health Insurance will adequately remunerate the Ophthalmologists for the individual procedures will they be motivated enough to provide this level of health care to VI patients.
Low visual aids (LVAs) are the simplest and the most economic method of reducing the consequences of visual impairment (VI). We evaluate prescribing of LVAs in order to improve the quality of life of ...visually impaired children.
We retrospectively reviewed the notes of 113 patients (pts.) with VI (visual acuity (VA) 6/18 or worse or with visual field defects) who attended the Low Vision Clinic, F.D. Roosevelt Hospital in Banska Bystrica, between Jan 2000 - Dec 2005. There were 61 boys and 52 girls with ages ranging from < 1 year to < 19 years (mean age 9.4 years, median 9 years).
Of the 113 notes reviewed, only 109 of patients were cooperative in having their VA formally tested. Blindness (VA worse than 3/60) occurred in 31 pts. (28.4%) and Low vision (VA worse than 6/18 or equal to 3/60) in 67 (61.5%). Of the remaining 11 patients, 4 had gross field defects (and good VA), (indicating that the total number with Low vision was 71 (65%). The other 7 patients had either developmental delay or were too young to perform a formal visual field test. Blindness was caused by perinatal factors in 52% of cases, prenatal factors in 45% and postnatal factors in 3%. Low vision was caused in 74.6% of cases by prenatal factors. The causes of blindness according to the affected anatomical site were: optic nerve disease in 12 (39%), cerebral visual impairment (CVI) in 8 (26%), and retinal disease in 6 (20%). Low vision was caused by retinal disease in 23 (34.3%), lens pathology in 11 (16.5%) and CVI in 10(14.9%). 171 LVAs were prescribed to 74 patients (65%): 76 (44.4%) were magnifiers, 21 (12.3%) were hyperoculars or high adds, 65 (38%) were telescopes and 9 (5.5%) telescope extensions. An average of 1.5 devices was prescribed for each patient. Every patient who was prescribed an LVA had subjective and objective improvement of their visual function and functional vision.
Blindness occurred in almost one third of our patients which agrees with the worldwide accepted estimate. The LVA are improving the quality of life of visually impaired children. The prescribing of LVA depends on the individual: it is not possible to anticipate the optical power of the LVA that a patient will need simply from looking at their diagnosis and measuring their VA because there are so many different requirements depending on their age and visual demands.
Changes of lipopolysaccharide (LPS) of Coxiella burnetii strain Priscilla during chick embryo yolk sac passaging were observed by SDS-PAGE and immunoblot analysis. The course of LPS phase variation ...was similar to that found in other C. burnetti strains, i.e. a conversion of the phase I to the intermediate phase II after 10 passages. The intermediate phase II LPS of Priscilla strain was also detectable by immunoblot analysis using immune serum against Priscilla strain in the 30th passage.
A 29 kDa protein, isolated from the outer membrane of Coxiella burnetii, strain Nine Mile phase I by detergent Empigen BB, was characterized. The failure in removing lipopolysaccharides (LPS) from ...preparations of the protein by the purification method used indicates a strong binding between proteins and LPS in the outer membrane of C. burnetii. The protein was immunogenic in mice and protected them against virulent C. burnetii challenge.
Coxiella burnetii cells in both phase I and II reveal in sodium dodecylsulphate-polyacrylamide gel electrophoresis (SDS-PAGE) similar protein profiles with only small differences. C. burnetii protein ...profile in SDS-PAGE depended on the method of purification of C. burnetii cells from chick embryo yolk sacs. Immune mouse sera against C. burnetii phase I cells recognized in phase I and II cell protein profiles mainly the 61 K and 29 K proteins by the immunoblot method. Hyperimmune mouse and rabbit sera against phase I and II cells reacted in different way with phase I and II cells. Sera against phase I cells recognized in both phase I and II profiles more protein bands than sera against phase II cells. Thus phase I LPS present in phase I cells exerted adjuvant effect on the antibody response in animals immunized with phase I cells.
BALB/c mice immunized intraperitoneally (ip) with killed purified Coxiella burnetii phase I corpuscular vaccines or trichloroacetic acid (TCA) extracts from phase I corpuscles (soluble vaccines) were ...protected against ip challenge with both homologous and heterologous C. burnetii phase I strains. Though the degree of protection, namely the inhibition of C. burnetii multiplication in the mouse spleen slightly varied, in general, corpuscular vaccines provided better protection than soluble ones. Cross-protection was accompanied by comparable levels of cell-mediated immune response as evaluated by lymphocyte transformation test (LTT). However, higher stimulation indices of LTT were obtained with homologous than with heterologous strains. The values of antibody response as determined by enzyme-linked immunosorbent assay (ELISA) were higher with homologous strains too. On average, both antibody-inducing and antibody-binding capabilities of the strains Priscilla and S were lower than those of the Nine Mile and Luga strains, except for values obtained with the antigens from homologous strains.
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