The natural history of varicella embryopathy: a 25-year follow-up.

A patient with clinically and immunologically proven varicella embryopathy achieved substantial recovery after initial severe developmental delay and manages well with her residual physical disabilities in adulthood.

[New aspects of therapy of mucoviscidosis (CF)]. / Neue Aspekte für die Therapie der Mukoviszidose (CF).

The authors survey of recent advances in CF research and their therapeutic implications: 1. the possibility of successful gene therapy by transfer of the normal gene to airway epithelial cells. 2. inhalations with the potassium-sparing diuretic amiloride that diminish the viscosity of the bronchial secretions, 3. application of adenosine or uridine triphosphate (ATP or UTP) to the apical surface of the respiratory epithelial cells which intervene with the function of ion channels, 4. enzymatic cleavage and liquidification of bronchial secretions by aerosolized human recombinante DNase. In addition, the possible advantages of (heart-) lung-transplantation are also discussed.

[Clinical significance of the most important abnormalities of respiratory organs]. / Die klinische Bedeutung der wichtigsten Fehlbildungen der Atmungsorgane.

The incidence rate and the clinical significance of inborn abnormalities of respiratory organs frequently are still underrated. In the clinic for paediatrics of the Medical Academy of Dresden malformations of respiratory organs, situated below the larynx, were demonstrated in 18 per cent of patients who had been referred hither in the course of 20 years for bronchopulmonary diagnostics. The clinical symptoms are very different and often uncharacteristic. Not seldom malformations of that kind first of all remain silent till a superinfection or a striking roentgenogram will arise the suspicion on a malformation. Following symptoms may refer to: permanent or intermitting stridor in the stenoses of the large respiratory tract (trachea and main bronchi), that is diagnosed as the most frequent anomaly. In nearly 80 per cent of the patients suffering from stenoses of a main bronchus symptoms of a recurrent or chronically obstructive bronchitis stood in the foreground. Mostly a tachy- and a dyspnoe are the leading symptoms in case of a connatal lobar emphysema, the most frequent anmaly of the pulmonary parenchyma followed by the pulmonary hypolasia and -agenesis. Chronic or relapsing pneumonias respectively a persisting cough may appear as symptoms in pulmonary sequestrations and in isolated anomalies of the bronchial aborization that otherwise in the majority of the cases will rest clinically mute. The long-term prognosis for children suffering from stenoses in the main bronchis is compared with those of tracheal stenoses relatively satisfactory.

[Pulmonary complications in AIDS]. / Pulmonale Komplikationen bei AIDS.

Adult patients suffering from infection with the HIV-virus acquire pneumocystis carinii pneumonia in nearly 80 per cent and in the half of all patients the basic disease AIDS has been detected by this lung infection. In childhood the patients with AIDS show most frequently interstitial lung diseases due to pneumocystis carinii or to lymphoid interstitial pneumonia. Also recurrent bacterial pneumonia may frequently occur, likewise infections with the cytomegalovirus or the Epstein-Barr-virus causing atypical pneumonia. The identification of the aetiology of these lung diseases is more difficult in children than in adults. In future it should be necessary to include more often AIDS as the basic disease into the differential diagnostic considerations in cases of such lung infections.

[The incidence of lung infection in patients with mucoviscidosis and the disease course with various pathogen spectra]. / Zur Häufigkeit der Lungeninfektion bei Patienten mit Mukoviszidose und zum Krankheitsverlauf bei unterschiedlichem Erregerspektrum.

A retrospective review on the frequency of lung infections by the most important organisms Staphylococcus aureus and Pseudomonas aeruginosa in patients with cystic fibrosis in the GDR during the period from 1981 to 1985 revealed an average infectious rate of 58.6 per cent by Staphylococcus and of 26.9 per cent by Pseudomonas respectively. A distinct increase of the infections by Pseudomonas could only be documented from 1981 to 1982, later on the infectious rate remained nearly equal and showed only a peak value of 35.6 per cent in 1983. Thus the frequency of infections by Pseudomonas is significantly lower in the GDR than in most other countries. The comparison of the course of the disease in patients with permanent lung infection during 4 or 5 years established a mortality rate of a double amount in the group of patients with Pseudomonas colonisation versus the group with Staphylococcus colonisation. Otherwise no significant difference could be stated in the mean age of the beginning of lung infection (Staphylococcus = 9.7 years of age - Pseudomonas = 10.1 years of age). Analysing the pulmonary x-ray findings we found a significantly more rapid deterioriation during the follow-up period in patients with permanent Pseudomonas infection than in patients with permanent Staphylococcus infection, whereas the evolution of body height and weight did not take different course.

[Significance of stenoses of the main bronchus within the scope of congenital abnormalities of the respiratory tract]. / Zur Bedeutung der Hauptbronchusstenosen im Rahmen angeborener Fehlbildungen der Atemwege.

During a period of 23 years (1962 to 1984) we found 197 children with stenoses of the main bronchi among 2,000 bronchological first examinations of children suffering from chronic or recurring bronchopulmonary diseases. In 75 children these stenoses were combined with those of the trachea, in 122 children they were restricted to the main bronchi. The left main bronchus showed an predominant incidence of 85 per cent. Only in 5 cases an extrabronchial cause could be established by anomalous vessels. In the main part of the patients we found intramural stenoses due to congenital circumscribed malazia or to complete aplasia of the bronchial cartilage. The clinical symptoms of a recurring obstructive bronchitis were predominating in nearly 80 per cent of the patients. By follow-up investigations of 65 children with an average duration of 8 years no deaths had occurred. Forty children (65 per cent) were without any complaints and could be physically loaded in a normal way, whereas 21 children showed persistent mild or moderate complaints. In two patients with cystic fibrosis and two others without this basic disorder bronchiectases had developed distal of the stenoses. Bronchological controls after an average follow-up of 3 1/2 years showed in 60 per cent of the children still unchanged stenoses and deforming changes of the bronchial wall distal of the stenoses in contrast to the more favourable clinical findings.

[Development of mucoviscidosis management in East Germany]. / Die Entwicklung der Mukoviszidose-Betreuung in der DDR.

The development of cystic fibrosis care in the GDR is reported by the activities of the "Working Group for Combatting Cystic Fibrosis" in the Pediatric Association of the GDR during the last 20 years. The mean incidence of the disease amounts to 1:4,100. It could be demonstrated that both the number of patients and their mean life expectancy have been increased continuously. At the end of 1985 803 patients were under care in the GDR, 10.6 per cent of them have reached the age of 18 years or more. New problems in medical care and psychological guidance arise in adolescents and in young adults with cystic fibrosis. By using linkage analysis of genetic markers new possibilities have been revealed in the field of prenatal diagnosis and of heterozygote detection.

[Acute foreign body aspiration as a respiratory emergency in childhood]. / Die akute Fremdkörper-Aspiration als respiratorischer Notfall im Kindesalter.

From 1957 to 1987 altogether 206 cases of tracheobronchial foreign body aspiration were diagnosed. Two third of the patients were one or two years old. Boys prevailed with 57 per cent. 55 per cent of all foreign bodies were nuts. Only 10 per cent were radiopaque (screws, nails, needles or pieces of bones). The attempt to eliminate the foreign body via the bronchoscope was successful in 96 per cent and failed in seven cases. Six children had to undergo a thoracotomy. In one third of the cases the foreign body remained two weeks or longer in the bronchial tree. 66 children with such a "chronic" foreign body were later on examined by bronchography, which showed in 29 per cent severe deformations of the bronchial wall and in 14 per cent even bronchiectasis. An acute foreign body aspiration should always be considered and handled as an emergency.

[Tuberculous meningitis in the child--then and now]. / Meningitis tuberculosa beim Kind--einst und jetzt.

From the point of view of a clinical pediatrician a short survey of the incidence and the earlier and more recent results concerning treatment and prevention of this life threatening localisation of tuberculosis is given. Because of some peculiarities the last case treated in our clinic is reported in detail. Finally the importance of the differential diagnosis of tuberculosis meningitis is discussed.