ATYPICAL FORMS OF EYE TOXOPLASMOSIS IN CHILDHOOD. CASE REPORTS.

AIM: To present an outline of acquired atypical forms of ocular toxoplasmosis (OT) in childhood, with reference to the 100th anniversary of the discovery of this etiology by Professor Janků from Czechoslovakia, who was first to describe the clinical congenital picture of OT characterised by macular scar. MATERIAL AND METHODS: Symptoms of intraocular bilateral neuritis appeared in a 6-year-old girl, with visual acuity (VA) bilaterally 0.1. Toxoplasmic etiology was demonstrated in laboratory tests, and the patient was immunocompetent. Following treatment with macrolide antibiotic and parabulbar application of corticosteroid, the condition was normalised stably at VA 1.0 in both eyes. Bilateral retinal vasculitis was determined in an 8-year-old boy, with VA of 0.25 in the right eye and 0.25 in the left, with a medical history of strabismus detected after suffering from varicella. The examination for toxoplasmosis was negative, but pronounced general hypogammaglobulinaemia classes IgG, IgM and IgA was detected. Immunosuppressive and immunomodulatory therapy did not produce the desired effect, and the condition progressed to retinochoroiditis. Due to blindness and dolorous glaucoma, enucleation of the right eye was performed at the age of 15 years. Histologically toxoplasmic cysts with bradyzoites were detected, a subsequent laboratory test demonstrated toxoplasmic etiology upon a background of persistent regressing hypogammaglobulinaemia. General anti-toxoplasma and subsequent immunosuppressive treatment did not produce the desired effect, and at the age of 22 years the patient lost his sight also in the left eye. CONCLUSION: Atypical form of OT intraocular neuritis in an immunocompetent patient had a favourable course, whereas retinal vasculitis with retinochoroiditis in a temporarily immunocompromised patient ended in bilateral blindness.

No evidence for linkage between the hereditary angiooedema clinical phenotype and the BDKR1, BDKR2, ACE or MBL2 gene.

Hereditary angiooedema (HAE) is a life-threatening disease with poor clinical phenotype correlation with its causal mutation in the C1 inhibitor (SERPING1) gene. It is characterized by substantial symptom variability even in affected members of the same family. Therefore, it is likely that genetic factors outside the SERPING1 gene have an influence on disease manifestation. In this study, functional polymorphisms in genes with a possible disease-modifying effect, B1 and B2 bradykinin receptors (BDKR1, BDKR2), angiotensin-converting enzyme (ACE) and mannose-binding lectin (MBL2), were analysed in 36 unrelated HAE patients. The same analysis was carried out in 69 HAE patients regardless of their familial relationship. No significant influence of the studied polymorphisms in the BDKR1, BDKR2, ACE and MBL2 genes on overall disease severity, localization and severity of particular attacks, frequency of oedema episodes or age of disease onset was detected in either group of patients. Other genetic and/or environmental factors should be considered to be responsible for HAE clinical variability in Caucasians.

[Immunologic findings in children with abnormal reactions after vaccination]. / Imunologické nálezy u detí s neprimerenými reakcemi po ockování.

In a group of 89 children with abnormal reactions after administration of the mixed vaccine against diphtheria, tetanus and whooping cough, after the mixed vaccine against diphtheria and tetanus, the live measles vaccine and oral poliovaccine, a detailed analysis was made of the case-history, and basic parameters of cellular and humoral immunity were examined. In these children the intensity of post-vaccination reactions was beyond the range of accepted criteria of mild and medium reactions or complications. In 17.3% of the children with an abnormal reaction after the mixed vaccine against diphtheria, tetanus and whooping cough a reduced IgA level was proved, while in the control group a reduced level was found only in 3.3%. 50% of the children who developed an abnormal reaction after the oral poliovaccine and the mixed vaccine against diphtheria, tetanus and whooping cough and at the same time some relative suffered from clinical signs of atopia, a reduced number of E rosettes of lymphocytes was recorded. 80% of the children who developed an abnormal reaction after the measles vaccine and some relative suffered from atopic disease, had low titres of specific antibodies against tetanic toxoid. Evidence was provided that children with certain precisely defined abnormal reactions after vaccination suffered significantly more frequently from reduced immune reactivity than children examined because of a suspected immunity defect.

[Manifestations of ocular allergy (atopy) in childhood. Present possibilities of therapy]. / Projevy ocní alergie (atopie) v detství. Soucasné moznosti jejího lécebného ovlivnení.

Vernal catharr of the conjunctivae is frequently the initial symptom of dermorespiratory syndrome. In all nine children where ophthalmological and other manifestations of varying extent of general atopy were combined, the authors revealed a marked increase of immunoglobulin IgE 220-2230 I.U./ml (normal values up to 150 I.U./ml) and polyvalent allergy. In the treatment of vernal catharr the authors used a combination of antihistaminics and preventive immunopharmaceutical preparations administered locally (Spersallerg and Opticrom) or as general medication (Hismanal and Zaditen), supplemented by aimed hyposensitization. In acute stages of the inflammation of the eye the authors administered Triamcinolon into the upper transient conjunctival fold. In the treatment of secondary infection Garasone was most effective. A finding interesting from the immunological aspect was vernal catharr without general symptoms of atopy in a patient with hypogammaglobulinaemia IgA.