[Hunter-Hurler and Morquio types of mucopolysaccharidoses].
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Biomedical subjects
Publications and source records attributed to C Zychowicz.
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Hundred eighty five children, aged between 6 and 15 years, with bronchial asthma and diagnosed allergy to Dermatophagoides pteronyssinus were followed-up for 2-3 years between 1978 and 1988. Children were treated with HDM vaccine Bencard. Administered doses were increased gradually at the beginning of therapy whereas they remained the same for further 1-3 years. Excellent and favourable results were achieved in 78.4% of cases. The treatment as postponed in 36 children (19.4%) because of the lack of improvement after one year of the follow-up. Easily manageable adverse reactions and complications were seen in 4 patients (2.2%).
Phadiatop test was performed in 174 children aged between 1 and 16 years (mean: 9 6/12 years) referred to the hospital because of the bronchial asthma, obstructive bronchitis and perennial or seasonal allergic rhinitis. Positive results were obtained in over 93% of children with atopic allergy. Comparing with PRIST, multiple allergen assay proved more sensitive (93.5%), specific (90%), and accurate (91.4%). The same data for PRIST were: 91.7%, 80.3%, 87.4%, respectively. False negative and false positive results were also less frequent than for PRIST. Therefore, Phadiatop, is considered an appropriate screening in vitro diagnostic test for inhalant allergy which should be performed at the beginning of respiratory tract diagnosis.
In order to study the familial occurrence of bronchial hypereactivity (BH) the parents of infants with BH were evaluated. 47 pairs of parents of infants aged 10-33 months were studied. All infants following spastic bronchitis developed BH. All parents had bronchial reactivity assessed. All answered a questionnaire from which the following data were retrieved: family history, personal history, environmental data and smoking habits. A similar analysis was carried out on a group of 19 pairs of parents forming a control group, derived randomly from hospital personnel. In parents of children with BH a higher incidence of BH was found in comparison with the control chi 2 = 11.52; p less than 0.01). In 14 subjects a positive familial history was found. This did not differ from the control. The smoking habit was seen more often in the study group that in the control (chi 2 = 9.82; p less than 0.01). These results allow us to state that familial factors play a role in bronchial hypereactivity in early infancy.
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In the years 1988-1990, 100 children aged from 2 to 18 years were examined, who were hospitalized for bronchial asthma of moderately severe and severe clinical course. In all children the examinations were performed twice at intervals of several weeks or several months during administration of the full set of drugs, including euphylline. The results were estimated according to the number and character of electric discharges. Out of the total number of 100 children in 52 cases abnormal EEG record was found. Generalized paroxysmal discharges were found in 16 children mainly with severe and moderately severe course of asthma. Generalized changes were recorded in 12 children mainly with moderately severe course of the disease. Milder focal changes were found in 23 children. Most evident changes occurred in children with over five years of disease duration and with history of unconsciousness episodes. The studies carried out indicate a relatively frequent, that is in half the cases, damage to the central nervous system following repeated episodes of hypoxia.
UNLABELLED: Epilepsy was recognized in 9 of 120 patients with bronchial asthma, aged 7-15 years, hospitalized in the Department of Allergology and Infectious Diseases in the Years 1989-1992. All the children had severe asthma attacks previously, six of them required hospitalisation at Intensive Care Unit. In 8 of the patients grand mal (generalised, tonic-clonic) seizures was observed, one child had absence state. Seizures were present both in the acute state of the asthma and during improvement. Anticonvulsant therapy was started immediately after diagnosis of epilepsy has been established. Clinical improvement with cease of seizures was noted there after. All the 9 patients had EEG examinations: 22 out of 29 performed, were abnormal. CONCLUSIONS: 1. Severe asthma attacks lasting for a considerable period of time, mainly 5 years, evoke epilepsy. 2. Undesirable signs of the antiasthmatic therapy on CNS was not found. 3. Accurate treatment of asthma seems to be the best prophylaxis of epilepsy.