Search

We report three cystic fibrosis (CF) patients with hypersplenism who underwent partial splenectomy. The postoperative course was uneventful in two patients; one patient developed a complication necessitating resection of the rest of the spleen. Haematological parameters improved and oesophageal varices regressed in all patients. On follow up, one patient showed a normal spleen, the other a normally functioning accessory spleen; the third patient again developed splenomegaly with hypersplenism. Partial splenectomy is a promising therapeutic option for CF patients with hypersplenism.

Nedocromil and salbutamol are effective drugs in preventing exercise-induced asthma (EIA). The aim of this study was to compare the protective effects of both drugs and a combination of both drugs against cold dry air-induced bronchoconstriction, using cold dry air challenges (CACh) as a surrogate for exercise. Twenty-five atopic children (mean age 13.7, range 8-18 yrs) with EIA participated in the study. Lung function tests were performed before medication, 30 min after medication and just before CACh, and 3 and 15 min after the challenge on four consecutive days. CACh consisted of a 4-min isocapnic hyperpnoea of -10 degrees C, absolutely dry air. Treatment consisted of nedocromil (two puffs of 2 mg) plus placebo, salbutamol (two puffs of 100 microg) plus placebo, the combination of both drugs, and placebo alone, in a random order. Both active drugs were significantly more protective than placebo and the combination showed an additive effect. Mean maximum postchallenge decrease in forced expiratory volume in one second after placebo was 27+/-8.1%, 12+/-9.5% after nedocromil, 8+/-10.4% after salbutamol, and 4.5+/-6.71% after the combination of both drugs, respectively. These results suggest that both drugs protect against exercise-induced asthma. Although not as effective as salbutamol and combined medication, nedocromil can give sufficient protection for many patients.

This case study reports on two infants, 5 and 6 months of age, respectively, with chronic interstitial lung disease who presented with failure to thrive, tachypnoea, rales and mild hypoxaemia. Epstein-Barr virus (EBV) was detected by in situ hybridization in lung biopsy specimens and by EBV-deoxyribonucleic acid-polymerase chain reaction (PCR) in bronchoalveolar lavage (BAL) fluid in one patient and by in situ hybridization and PCR analysis in BAL fluid in the second patient. There was serological evidence of immunoglobulin G antibodies to EBV capsid antigen by indirect immunofluorescence in both patients. After 7 months of respiratory symptoms one patient was successfully treated with a 10 day course of intravenous ganciclovir followed by oral acyclovir for 20 days. The other patient became symptom free after 3.5 months of respiratory symptoms, without any specific antiviral medication. During a follow-up of 2 and 1.5 yrs, respectively, both infants remained symptom free and showed normal physical development.

Whether bronchodilators should be used for the treatment of infants with bronchiolitis is subject to debate, partly because of the low sensitivity of the methods for assessing lung function changes in infants. In the present study, we compared the recently introduced raised volume (RVRTC) with the conventional end-tidal rapid thoracoabdominal compression (ETRTC) technique in infants with acute viral bronchiolitis. In 17 infants lung function was assessed by both methods, at baseline values and after salbutamol inhalation. Forced expiratory volumes (FEV(0.5), FEV(0.75), FEV(1.0)) were used for the quantification of RVRTC measurement, and maximal expiratory flow at functional residual capacity (Vmax (FRC)) for ETRTC measurements. A significant individual change was defined by a mean postbronchodilator value that differed from baseline value by more than twice the within-subject coefficient of variation (CV). Group mean intrasubject CVs ranged from 4.7% to 5.3% for FEV parameters; it was 14.0% for Vmax (FRC). For the group, post-bronchodilator measurements did not differ significantly from baseline measurements. For the majority of infants, however, the within-subject comparison of responses revealed substantial differences between both techniques; while no infant demonstrated a significant increase in Vmax (FRC), eight (47%) infants responded with significantly improved timed volumes. The RVRTC technique provides the investigator with a more sensitive diagnostic tool for documenting the effectiveness of therapeutic interventions on an individual basis. Furthermore, the findings of the present study provide a rationale for the application of bronchodilators in a subgroup of infants with acute bronchiolitis.

Objective: To investigate possible renal toxicity of long-term gentamicin inhalation in patients with cystic fibrosis., Methods: Urinary N-acetyl-beta-D-glucosaminidase (NAG) activity was measured during routine respiratory clinic visits. Outpatient records were reviewed for data on long-term gentamicin inhalation, and parents and patients were interviewed for compliance. Exclusion criteria were irregular gentamicin inhalation, urinary infection or other febrile illness, intravenous aminoglycoside treatment during the previous three months, and diabetes mellitus. Patients were assigned to three groups: group 1, current gentamicin inhalation; group 2, previous gentamicin inhalation that had been stopped at least three months ago; and group 3, never any gentamicin inhalation., Results: 52 patients (34 girls, 18 boys), mean (SD) age 11.5 (5.7) years, entered the study. Patients currently on gentamicin inhalation (n = 20) were significantly younger and had higher urinary NAG activity (0.83 (0.57) U/mmol creatinine) than the 23 patients without gentamicin inhalation (0.26 (0.10) (p = 0.0001) and the nine patients with previous gentamicin inhalation (0.32 (0.15) (p = 0.0125). Twelve patients on current gentamicin inhalation had raised NAG values but all those in groups 2 and 3 had NAG values within the normal range. In patients currently on gentamicin inhalation, there was a positive correlation between urinary NAG activity and cumulative dose of nebulised gentamicin (r = 0.60, p = 0.0049)., Conclusions: Long-term gentamicin inhalation in patients with cystic fibrosis poses a risk of renal toxicity. It is not known whether further treatment might result in more severe renal damage.

The passive, single-breath, flow-volume technique is a simple method for measuring the resistance (Rrs) and the compliance (Crs) of the respiratory system in infants. So far, the potential influence of end inspiratory occlusion time on these measurements has not been investigated. We measured Rrs and Crs in 36 infants and toddlers with bronchiolitis; in each child, a spectrum of nine fixed occlusion times, ranging from 90 to 600 ms, was applied in random sequence. Increasing the duration of occlusions from 90 to 275 ms resulted in marked stepwise changes of measured Rrs and Crs; occlusions longer than 275 ms, however, produced highly reproducible measurements, as expressed 1) by minimal absolute differences between measured values at subsequent occlusion times and 2) by minimal percentage changes of measured values from one occlusion time to the next. There was no influence of age on the results; reproducible measurements were made in children as old as 1.5 y. This suggests that, in infants with bronchiolitis, 1) occlusions between 300 and 450 ms might be ideal for obtaining reliable measurements, and 2) the age range for applying this method can be extended into the 2nd y of life.

Two girls with asthma died suddenly in autumn-winter 1985. On clinical criteria, both had been classified as moderately severe asthmatics, had shown a stabilised disease course, and had complied with antiasthmatic medication. By chance, lung function, bronchial reactivity, and subjective perception of lung function impairment had been assessed five and a half months and one month before death, respectively. For comparative purposes, we measured lung function and bronchial reactivity in a reference group of 37 children who had asthma of the same clinical severity and who were taking the same medication. In comparison with this reference group the index subjects showed a high degree of bronchial reactivity, incomplete recovery of function after bronchodilation, and appreciably reduced perception of severe lung function impairment. Regular assessment of lung function, bronchial reactivity, and perception of breathlessness might help to identify the patient who is prone to a fatal attack of asthma.