Induced sputum nitrites correlate with FEV1 in children with cystic fibrosis

Aim: To determine the difference in the levels of nitrites in induced sputum of children with cystic fibrosis (CF) and controls. Furthermore, to evaluate the association between induced sputum nitrites and lung function in children with CF. Methods: Nitrites, cell differentials, white blood cell count, were estimated in induced sputum of 20 children with CF and 10 age‐matched healthy controls. Nitrites in induced sputum samples were measured using the Greiss assay. Lung function was ascertained by spirometry. Results: We observed high levels of nitrites in CF (184.8 ± 11.07 μM/L) versus controls (56.4 ± 5.7 μM/L) (p < 0.01). A positive correlation between neturophil percent and nitrites, white blood cell count and nitrites (p < 0.05) in children with CF was observed. Sputum nitrites correlated negatively with FEV₁ (p < 0.05) in children with CF. Conclusion: Induced sputum nitrite could serve as a useful non invasive marker for assessing the degree of inflammation in the airways of children with CF.     

Nebulized hypertonic saline/salbutamol solution treatment in hospitalized children with mild to moderate bronchiolitis

Background: The objective of this study was to determine the efficacy and safety of nebulized 3% hypertonic saline solution and salbutamol in the treatment of mild to moderate bronchiolitis. Methods: In a randomized controlled trial, 93 infants with mild to moderate bronchiolitis were divided into two groups. The infants received inhalation of 2.5 mg (0.5 mL) salbutamol dissolved in either 4.0 mL normal (0.9%) saline (control group, n= 43) or 4.0 mL hypertonic (3%) saline (treatment group, n= 50). The therapy was repeated three times daily until discharge. Cough, wheezing, pulmonary physical signs, and the length of hospital stay were recorded. Results: Wheezing remission time was 3.8 ± 1.1 days in the control group and 2.7 ± 0.9 days in the treatment group (P < 0.01). Cough remission time was 6.3 ± 0.9 days in the control group and 5.3 ± 0.8 days in the treatment group (P < 0.01). The moist crackles disappeared at 5.4 ± 0.8 days in the treatment group versus 6.2 ± 0.9 days in the control group (P < 0.01). Furthermore, the average length of hospital stay decreased from 7.4 ± 1.5 days in the control group to 6.0 ± 1.2 days in the treatment group (P < 0.01). No obvious adverse effects were observed. Conclusions: Inhalation of nebulized 3% hypertonic saline solution and salbutamol is a safe and effective therapy for patients with mild to moderate bronchiolitis.     

Cascade carrier-testing in cystic fibrosis

It is good medical practice to offer carrier tests and counselling to the relatives of those affected by recessive disorders. Many are concerned about their own chances of having affected offspring. Cystic fibrosis carrier tests have been feasible since the discovery of the gene in 1989. It was generally agreed that although population screening was not practical, testing should be offered to relatives and their partners. There is evidence that such offers have not always been made and relatives have sometimes found it difficult to be tested. An active cascade programme of the counselling and testing of cystic fibrosis patients' relatives and their partners has operated from Royal Manchester Children's Hospital since 1993. The service operates with dedicated staff, backed up by a specialist cystic fibrosis molecular genetics laboratory and a specialist genetic counselling service. The main target groups are couples or individuals of child-bearing age. There is discouragement of the testing of young children and of grandparents beyond reproductive age, although, if parents or individuals are insistent, testing is often performed, after counselling. An audit of users has shown satisfaction, very few feeling that they were pressured into having the tests. The experience of other centres with cascade-testing in cystic fibrosis is summarised. Cascades can start whenever a sufferer or carrier is identified, although care should be exercised in instituting active cascades in the extended families of newborns identified as carriers in neonatal screening programmes.     

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目的 研究高渗盐水激发试验及运动激发试验在哮喘儿童的临床应用,旨在寻求更适合哮喘儿童的气道反应性检测的方法方法收集中国医科大学附属盛京医院2007年5月至2008年6月符合儿童支气管哮喘防治常规诊断标准的哮喘缓解期患儿34例,年龄5～13岁,在规定时间内先后做气道高渗盐水激发试验和运动激发试验.结果 同一哮喘患儿高渗盐水激发试验的敏感性显著高于运动激发试验(P<0.01).结论 高渗盐水激发试验的敏感性和特异性高于运动激发试验,而且设备低廉,方法简单、易配合,安全,副反应少,尤其适用于在哮喘治疗期间动态观察儿童气道的反应性,对指导药物治疗有重要价值.     

Children's understanding of cystic fibrosis

Sixty-one children aged 4–18 years with cystic fibrosis were interviewed about their understanding of the illness and their attitude towards it. Responses were compared across three age groups: 4–6 years, 7–11 years and 12–18 years, corresponding to Piagetian stages of cognitive development. While there was an obvious progression in knowledge with advancing age, the children's attitude to their illness and therapy varied remarkably between age groups. Those in the 4–6 year group were more positive about themselves and about the effect of therapy than those in the older groups. More than half knew that they were bom with the illness, in contrast to published reports of healthy children's concepts of illness causation for this age group. Children in the 7–11 year group were least likely to feel better after therapy and were least likely to mention anything positive about having cystic fibrosis, despite having a higher mean Schwachman clinical score than those in the oldest group.     

Growth failure in cystic fibrosis

OBJECTIVE: The aim of this study was to describe and compare the nutritional status of children aged 0-18 years attending the cystic fibrosis (CF) clinic at the Royal Children's Hospital, Brisbane, Australia, as outpatients in 1986 and 1996. METHODOLOGY: The heights, weights and pulmonary function of children attending the CF clinic as outpatients in 1986 (n = 97) and 1996 (n = 227) were retrospectively analysed using a computerized database maintained by the CF clinic. The heights and weights were analysed in terms of z scores for height for age (HAZ), weight for age (WAZ) and weight for height (WHZ). Pulmonary function data is not available for all children. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and forced mid expiratory flows (FEF) were expressed as a percentage of predicted and are presented here. RESULTS: The 1986 sample consisted of 41 males (age range 0.18-14.59 years, mean age 6.52 (4.33)) and 56 females (age range 0.15-14.97 years, mean age 7.75 (3.70)). The 1996 sample consisted of 111 males (age range 0.09-17.97 years, mean age 8.80 (5.49)) and 114 females (age range 0.12-17.98 years, mean age 8.49 (5.26)). In 1986, males were shorter than females (P = 0.0096) and females had a lower mean FVC than males (P = 0.0438). In 1996, males were shorter, lighter and more wasted than females (P = 0.0357, P = 0.0034 and P = 0.0273, respectively) and females had a lower mean FEV1 and mean FVC than males (P = 0.0176 and P = 0.0079, respectively). Males in 1996 were lighter and more wasted than males in 1986 (P = 0.0023 and P = 0.0139, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P = 0.0012 and P = 0.0069, respectively). Females in 1996 were shorter and lighter than females in 1986 (P = 0.0273 and P = 0.0405, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P < 0.0001 and P < 0.0001, respectively). When subjects were classified according to FEV1 (FEV1 > or = 75% or FEV1 < 75%), there were no significant differences in z scores between the 1986 group and 1996 group. Similarly, when the 1986 group were matched for gender and FEV1 with the 1996 group, there were no significant differences in z scores for males or females. CONCLUSIONS: It is suggested that the apparent worsening of nutritional status among the 1996 group of CF patients is in fact due to an effect of increased survival of patients with more severe clinical symptoms. The findings from this study highlight the continuing, and in fact, worsening problem of growth failure in children with CF.     

Use of hypertonic saline in the treatment of cerebral edema in diabetic ketoacidosis (DKA)

Abstract: Cerebral edema is the primary cause of morbidity and mortality in children and adolescents with diabetic ketoacidosis (DKA). We report a case of an adolescent female with life-threatening DKA-related cerebral edema who responded to a combination of mannitol and hypertonic saline. This is the first report of the use of hypertonic saline in the treatment of cerebral edema due to DKA.     

Unrecognised infection in a cystic fibrosis patient

We report a 17-year-old Malay boy with cystic fibrosis who over a 14-month period experienced worsening respiratory symptoms and deteriorating lung function. Burkholderia pseudomallei was eventually isolated from his sputum. He improved clinically following treatment for meliodosis and his lung function returned to normal.     

Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis

BACKGROUND: Infants with cystic fibrosis (CF) can develop episodes of hyponatremic hypochloremic dehydration with metabolic alkalosis when they sweat excessively, which is not caused by sweating in normal infants. We investigated the incidence of the metabolic alkalosis with hypoelectrolytemia in CF infants, the possible risk factors for its occurrence and the importance of the manifestation in the diagnosis of CF. METHODS: In order to evaluate the incidence and the risk factors for the development of this sweat-related metabolic disorder in CF, we reviewed the records of all children diagnosed as having CF before the age of 12 months in a 10-year period. Data analysis included medical history data, clinical features, biochemical parameters (blood pH, serum bicarbonate, sodium, chloride and potassium levels), sweat chloride test values, as well as genetic analysis data. RESULTS: The prevalence of metabolic alkalosis in association with low serum electrolyte concentrations (hyponatremia, hypochloremia, and hypokalemia) in infant CF population in our region was 16.5%. We found no season predilection in its occurrence. Early infant age, breast-feeding, delayed CF diagnosis, heat exhaustion and the presence of severe CF transmembrane conductance regulator mutations are predisposed factors for the development of metabolic alkalosis with hypoelectrolytemia. CONCLUSIONS: The results from our study suggest that metabolic alkalosis with hypoelectrolytemia is a relatively common manifestation of CF in infancy. The possibility of CF should be seriously considered in any infant with this metabolic disorder.     

Allergic bronchopulmonary aspergillosis in cystic fibrosis

ABSTRACT. We determined prospectively over a 12-month period the importance of the fungus Aspergillus in patients with cystic fibrosis.
Aspergillus species was cultured from 10 out of 116 children and adolescents (8.6%), and from 25 of the 1073 sputa (2.3%) collected from these patients. Eighteen of these subjects (15.5%) had positive allergen skin prick tests to Aspergillus species or Aspergillus fumigatus . Fifty-three patients (13%) had blood collected for precipitins and 7 (13%) exhibited type III hypersensitivity to Aspergillus fumigatus . Although 26% had an IgE greater than 200 I.U./ml, only one patient had an IgE greater than 1,000 I.U./ml. We found no evidence of an association between severity of lung disease and either colonisation by or sensitisation to Aspergillus .
No patient fulfilled all our criteria for the diagnosis of allergic bronchopulmonary aspergillosis (ABPA). One teenager (with wheezing, pulmonary infiltrate, positive skin tests, positive sputum culture and markedly elevated IgE) may have had an episode of ABPA. We studied three other children with co-existent asthma in whom it was difficult to establish whether the asthma was related to sensitivity to Aspergillus .
We conclude that ABPA is an uncommon complication of cystic fibrosis in our clinic.     

New insights into pulmonary inflammation in cystic fibrosis

Persistent lower airway infection with inflammation is the major cause of morbidity and mortality in cystic fibrosis. This review examines the recent advances in the understanding of airway inflammation in cystic fibrosis, and focuses on the evidence that pulmonary inflammation is, under some circumstances, disassociated from infection, and the potential implications for therapeutic intervention.     

Nebulized hypertonic saline and recombinant human DNase in the treatment of pulmonary atelectasis in newborns

Objective: The aim of this study was to compare and evaluate the efficacy of nebulized 3% hypertonic saline (HS) and recombinant human DNase (rhDNase) treatment for resolution of persistent atelectasis in newborns. Study design: Forty newborns (38 preterms) who did not respond to conventional treatment were enrolled to receive either nebulized 3% HS solution (n = 20) or rhDNase (n = 20) between September 2007 and March 2008. Clinical parameters, oxygen saturation and radiological response (chest X‐ray scoring) were analyzed before and after administration of 3% HS or rhDNase. Results: The patients of the nebulized 3% HS solution group improved better chest X‐ray scores parameters than the patients of the rhDNase group: chest X‐ray scores were 5.1 ± 1.9 vs 4.8 ± 1.7 before treatment and 1.0 ± 0.8 vs 2.1 ± 1.4 after treatment (P < 0.001). Resolution time of atelectasis did not differ between the two groups after whole treatment but the percentage of atelectasis resolution after 3 days treatment were 90% (18/20) in the 3% HS group and 70% (14/20) in the rhDNase group. The patients in the 3% HS group improved better also in clinical parameters in comparison to the rhDNase treatment. The difference of oxygen saturation before and after the treatment was 4.6 ± 0.8 in 3% HS group in comparison to 2.6 ± 0.1 in the rhDNase group (P < 0.05). All serum sodium levels were normal in two groups before and after the treatment modalities. Conclusion: This is the first study on the usefulness of nebulized 3% hypertonic saline solution in treating newborns with pulmonary atelectasis. In addition, 3% HS solution was a more effective therapeutic option on the basis of clinical and radiological improvement compared to rhDNase treatment in newborns with pulmonary atelectasis.     

Flexible fibre-optic bronchoscopy in the management of lung complications in cystic fibrosis

Flexible fibre-optic bronchoscopy was performed under sedation in 24 children with cystic fibrosis. In eight cases bronchoscopy was carried out as a therapeutic procedure. Most children were subjectively improved, but objective evidence of change was minimal. Useful information was obtained in 9/16 children who underwent the procedure for diagnostic purposes. Information included sensitive identification of organisms responsible for lower respiratory symptoms in non-expectorating cases and identification of unsuspected gastro-oesophageal reflux.