[Chronic granulomatous disease (CGD): dysfunction of the neutrophil granulocyte NADPH-oxidase enzyme system]. / Krónikus granulomatózus betegség (CGD): a neutrofil granulocita NADPH-oxidáz enzimrendszerének múködésképtelensége.

Dysfunction of NADPH oxidase results in chronic granulomatous disease (CGD), a syndrome characterized by severe bacterial and fungal infections. Phagocytes of the patients are unable to kill ingested microorganisms which leads to the formation of granulomas and abscesses. Predominant pathogens are the catalase-positive bacteriae (Staphylococcus aureus) and some fungi (Aspergillus species). Infections of these patients should be treated by antimicrobial agents, which penetrate cells and kill pathogens inside. The aim of this study was to give a short description of the structure and function of the NADPH oxidase enzyme and to summarize the results obtained during the diagnostic of 10 patients with chronic granulomatous disease. Characterization of the disease was confirmed by mutation analyses.

Prognostic data of the second follow-up in childhood wheezy bronchitis.

206 non-selected children who had wheezy bronchitis before two years of age were observed in the first follow-up (1985) about 9 years after their clinical wheezy episode. Among them 31 patients (15%) showed bronchial hyperreactivity (B.H.) after acetylcholine challenge and 9 children became asthmatic. In the second follow-up (1988) 28 children who had B.H. and randomly 17 children who did not have B.H. participated. The prognosis of wheezy bronchitis is good, there was no new asthmatic child at this time. The B.H. was found to diminish only in 7 cases. During the last 3 years wheezy episodes developed only in 5 asthmatic patients. The skin prick test (SPT) positivity did not change (26% versus 29%). The familial atopy in the group B.H. is higher (43%) than in the group non B.H. (23%) but the difference is not significant (X2 = 1.72). No significant correlation was found between the familial atopy and SPT positivity. The familial smoking is higher in the group B.H. (78% versus 64%) but no significant influence on the B.H. (X2 = 1.03) could be detected.

Effect of parental smoking on wheezy bronchitis and bronchial hyperreactivity.

In the course of the follow-up of 206 previously obstructive bronchitis children, the effect of parental smoking upon the occurrence of respiratory diseases, the yearly frequency of wheezing episodes and the age until the obstructive episodes used to return have been investigated. Familial and maternal smoking was more frequent in this group compared to the control group. In spite of this, however, no correlation could be detected between familial smoking and frequency of respiratory diseases, as well as the above mentioned characteristics of obstructive bronchitis. The familial smoking did not seem to influence the bronchial hyperreactivity challenged with acetylcholine, although the prevalence was higher.

[Clinical considerations on exercise-induced bronchoconstriction]. / Klinische Betrachtungen zum belastungsinduzierten Bronchospasmus.

With 378 children suffering from asthma authors performed three types of physical exercises. The correlation between the exercise induced bronchospasm and the clinical symptoms of asthmatics, first of all the severity of asthma, has been investigated. No significant correlation was found between the EIB and age, sex, somatic development, start and duration of asthma and frequent respiratory infections. The seriously ill patients had more serious and frequent EIB. As practical conclusion it can be established that the examination of asthmatic children with exercise induced bronchospasm is important from the point of view of sports, but also in judging the severity of asthma.

Results of bronchial challenge after infantile obstructive bronchitis.

The bronchial hyperreactivity was examined in a follow-up of 206 children having had obstructive bronchitis before two years of age. The acetylcholine challenge was done with 0.5% and 1% Pharmalgen solutions. The bronchial hyperreactivity was established over a 20% decrease of PEF and/or FEV1 values. Summarizing the results produced by 0.5% and 1% solutions a significant difference could be found in the bronchial hyperreactivity between the follow-up and control groups. Similarly the PC20 (provocative concentrations) values were suitable for differentiating the two groups. Upon physical exercise, EIB occurred in 2%. With the skin prick test (SPT) by 10 allergens 3+ or 4+ positivity was found in 17% of the children. Between the bronchial hyperreactivity and SPT positivity a significant correlation could be established.

Bronchial hyperreactivity after infantile obstructive bronchitis.

A follow-up study was performed on 406 patients treated for infantile obstructive bronchitis during the period between 1964 and 1973. Their mean age was 12.6 years at the time of the study. The male: female ratio was 1.7. Forty-three patients (11%) became asthmatic within 10 years after onset of the wheezy episode of infancy. In one-third of the 363 non-asthmatic children, bronchial hyperreactivity was shown by acetylcholine and histamine provocation. There was a significant correlation between the number of recurrent obstructive episodes and the length of the period of recurrent wheezing on the one hand and bronchial hyperreactivity on the other hand.

Complete recovery from paraquat poisoning causing severe unilateral pulmonary lesion.

Poisoning by paraquat, a plant-protecting agent, its clinical manifestations and treatment are discussed. The case of a 5-year-old boy who had ingested an unknown quantity of paraquat is described. Peritoneal dialysis proved to be effective in overcoming renal and hepatic failure. Subsequently, a pulmonary lesion with unilateral preponderance developed; this showed marked radiological regression and in a year nearly complete functional recovery ensued.

Thorax deformity and asthma bronchial.

In patients with bronchial asthma thorax deformity aggravates the respiratory function. The authors performed follow up examinations on 381 young adults who suffered from bronchial asthma in childhood. Thorax deformities were was found in 70 patients (28 pectus excavatum, 8 pectus carinatum, 19 thorax emphysematicus . 15 other and combined deformities). Comparing the recovered and non-recovered asthmatic patients, the state of the ill one was significantly worse with regards to thorax deformity. In the seriously ill patients thorax deformities occurred significantly more frequently than in the total number of patients. In the thorax deformity group asthma had an earlier onset and lasted longer in comparison with the whole of patients studied. Bronchial hyperreactivity and lung function values referring to chronic obstruction were significantly more frequent in patients with thorax deformity. Investigating physical exercise, the ratio of patients pursuing some sporting activity was similar, i.e. 50%, both in the total patient group and in the group of patients with pectus excavatum and pectus carinatum, while 90% of the patients belonging to the thorax emphysematicus group did not pursue any physical training.

Study of exercise induced bronchospasm by various loading tests.

In 378 children affected by asthma and 87 healthy children, physical loading was carried out by three kinds of test, viz. free running, stairstep test or skipping, and bicycle ergometry. In 32% of healthy children physical exercise led to improved respiratory function, while this value was only 7% in asthmatics. An improvement in respiratory function exceeding 20%, i.e. appreciable bronchial dilatation, never occurred in asthma patients. The bronchospasm of healthy children showed no consistent time course, in asthmatics it was most pronounced three minutes after exercise. Exercise induced bronchospasm occurred most frequently after free running, least frequently after ergometric loading. The stairstep test and skipping provoked exercise induced bronchospasm in a nearly identical proportion. Severity of exercise induced bronchospasm exhibited a similar distribution. In addition to cases with a positive response to running test, some of the other methods revealed further 5-6% of cases. Free running is the most suitable method for demonstration of exercise induced bronchospasm, ergometric loading appears the least sensitive test in this respect. By application of three loading tests exercise induced bronchospasm could be demonstrated in 52% of paediatric asthma patients.

The prognosis of severe bronchial asthma in childhood on the basis of late reexaminations.

Four hundred and forty one patients treated for asthma between 1954 and 1978 were reexamined. Their age ranged from 15 to 36 years at the time of reexamination. To regard a case as severe, at least four factors out of the following six had to be present: 1. Attacks more frequent than one every week; 2. attacks lasting for more than one day; 3. six or more hospitalizations; 4. absence from school exceeding one month per school-year; 5. drop-out from school for at least one school-year; 6. steroid treatment for more than one week. 59 patients (13.3%) fulfilled the criteria. The sex ratio within the severe group was 1:1 although there was a male preponderance of all asthmatics. The recovery rate for the severe group was lower (40.6%) than for all patients (75.0%). Prognosis was less favourable in female than in male patients. In the severe group the disease appeared earlier, eczema during infancy occurred more frequently and allergic disorders had a higher incidence in the family than in the group as a whole. Chest deformities were observed in 48.7% of severe patients while its incidence was only 18.6% for all patients with asthma. Acetylcholine-induced bronchial hyperactivity was more frequent in the severe patients than in all patients irrespective of the degree of severity while the prevalence of exercise induced asthma showed no relationship to the degree of severity. Among the severe cases 31% failed to cope with a standardized physical load.

[Asthma bronchiale and sport (author's transl)]. / Asthma bronchiale und Sport.

Follow-up studies were carried out in asthmatic children treated between 1954 and 1978 in the First Department of Pediatrics, Budapest, who have completed since then their 14th year of life. In 441 such children the sport achievements and the complaints after physical efforts have been taken into consideration. In 371 children tolerance tests with bicycle ergometer were done. It was found that among the recovered asthmatic children 48.9%, and among the nonrecovered 73.6% never have gone in for sport. The occurrence of bronchospasm due to bicycle-ergometer tolerance test (exercise induced asthma: EIA) corresponded to 19.5% among the healed patients and to 37.6% in the nonrecovered cases. In the authors' opinion, suitable persuasion could have induced a number of their former patients to go in for sport regularly. The authors refer the asthmatic patients to the possibility of physical culture. If EIA develops, medicaments are prescribed to these children, which enable them to go in for sport regularly.