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Spontaneous pneumothorax is rare in childhood. Before 12 years of age the main underlying pathologies are asthma, cystic malformations, post infectious bullae, and infectious pneumoniae. After 12 years of age it is mainly associated with cystic fibrosis and constitutional slim morphology. Symptoms vary according to the extent of lung collapse and the diagnosis is confirmed on chest X rays. In mildly symptomatic pneumothorax, spontaneous resolution is achieved within few days. When cardiorespiratory difficulties are present, mechanical evacuation of air from the pleural cavity is necessary through a tube drainage maintained until complete pulmonary reexpansion. Surgical treatment is indicated in case of persisting air leakage after one week of efficient drainage, large cystic malformation or post infectious bullae, recurring or bilateral pneumothorax.
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PMID:[Spontaneous pneumothorax in children]. 1079 46

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.
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PMID:Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders. 1170 Aug 92

Aspergillus fumigatus is one of the most ubiquitous of the airborne saprophytic fungi. Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis, and is characterized by hypersensitivity to chronic colonization of the airways with A. fumigatus. We report the case of a patient with ABPA presenting with pleural effusion. A 27-year-old male was referred with recurrent right pleural effusion. Past medical history was remarkable for asthma, allergic sinusitis, and recurrent pleurisy. Investigations revealed peripheral eosinophilia with elevated serum immunoglobulin E and bilateral pleural effusions with bilateral upper lobe proximal bronchiectasis. Precipitating serum antibodies to A. fumigatus were positive and the A. fumigatus immediate skin test yielded a positive reaction. A diagnosis of ABPA associated with bilateral pleural effusions was made and the patient was commenced on prednisolone. At review, the patient's symptoms had considerably improved and his pleural effusions had resolved. ABPA may present with diverse atypical syndromes, including paratracheal and hilar adenopathy, obstructive lung collapse, pneumothorax and bronchopleural fistula, and allergic sinusitis. Allergic bronchopulmonary aspergillosis is a rare cause of pleural effusion and must be considered in the differential diagnosis of patients presenting with a pleural effusion, in particular those with a history of asthma.
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PMID:Allergic bronchopulmonary aspergillosis: a rare cause of pleural effusion. 1184 30

The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion-selective channel whose dysfunction leads to the onset of cystic fibrosis. CFTR activation is normally elicited by stimulation of the cAMP pathway, which effects protein kinase A activation. However, previous studies from our laboratory indicate that the actin cytoskeleton is also required for a proper CFTR function. In this report, the regulatory role of actin filament organization in the activation of CFTR was explored. Maneuvers to modify the steady-state organization of actin filaments elicit the activation of CFTR in the absence of a functional cAMP pathway. Partial disruption of the actin cytoskeleton of CFTR-expressing cells with cytochalasin D (CD) induced CFTR activation in the absence of an activated PKA. Similar findings were obtained by intracellular dialysis with the actin-severing protein gelsolin. However, extended treatment with CD leading to the collapse of the actin cytoskeleton rendered CFTR completely insensitive to direct PKA activation. cAMP activation of CFTR was also found to be dysfunctional in cells lacking the actin-crosslinking protein ABP-280, which was recovered after dialysis of the cells with filamin, a homologue of ABP-280. The present data indicate that an organized actin network is required for the proper cAMP-dependent activation of CFTR. The possibility is also explored that actin must be directly associated with CFTR to elicit its activation, further suggesting that this channel protein may bind actin as well.
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PMID:Role of actin filament organization in CFTR activation. 1184 8

A group of patients with cystic fibrosis (CF) have severe small airways disease characterized by wheeze, chest tightness and limited sputum production, often with deteriorating lung function. Suggested mechanisms include mucosal edema secondary to infection and inflammation, smooth muscle contraction caused by inflammatory mediators, and collapse of bronchiectatic airways. While treatment with long-term oral corticosteroids may result in symptomatic improvement, adverse effects often make them intolerable. Inhaled corticosteroids are used in many centers despite the lack of conclusive evidence of their efficacy. Therapeutic alternatives to corticosteroids are aimed at reversing bronchoconstriction and reducing inflammation. Many patients with CF are treated with short- and long-term inhaled bronchodilators, but data to support their use are inconclusive. Other attempted routes of administration for short-acting bronchodilators include the subcutaneous and intravenous routes, but clinical data are again lacking. Sodium cromoglycate (cromolyn sodium) has been studied, with little evidence of benefit. Theophyllines have also been studied, both intravenously and orally, with some effect, but are not often used in clinical practice. Nonsteroidal anti-inflammatory therapies include ibuprofen, macrolide antibiotics, intravenous immunoglobulin, cyclosporine, and leukotriene antagonists. Ibuprofen has been shown to be useful in patients with mild CF disease, but concerns about potential adverse effects have limited its use. The results of various macrolide studies are awaited, but to date there are no long-term studies published. While there is great interest in the potential of intravenous immunoglobulin, cyclosporine and leukotriene antagonists, the evidence for their effectiveness comes from anecdotal reports, thus there is currently insufficient data to support their use. Since this is a small group of patients, it is unlikely that sufficient numbers will ever be recruited for these studies; thus it is probable that drugs will be tried on an individual patient basis. The order in which they are attempted is unclear, but it would be sensible to try the least invasive medication with the least adverse effects first, moving on to more potent, but more toxic drugs if that treatment fails.
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PMID:Treatment of severe small airways disease in children with cystic fibrosis: alternatives to corticosteroids. 1203 74

Allergic Bronchopulmonary Aspergillosis (ABPA) is characterized by recurrent pulmonary infiltrates that can result in central bronchiectasis and bronchiolitis obliterans especially if there is a lack of recognition and treatment. The incidence of ABPA is 1-2% in patients with persistent asthma and approximately 7% (range 2-15) in patients with cystic fibrosis. The diagnostic criteria are useful in that there is no single test (with the exception of central bronchiectasis in patients with asthma) that identifies ABPA. The differential diagnosis of ABPA includes many conditions including chronic eosinophilic pneumonia, Churg Strauss Syndrome, Hyper-IgE Syndrome, persistent asthma with lobar collapse, and cases of parasitism. The most useful laboratory assays in patients who have immediate cutaneous reactivity. to Aspergillus mixes or A. fumigatus are total serum IgE concentration, elevated serum IgE-A.fumigatus and serum IgG-A.fumigatus. Prednisone remains the drug of choice yet need not be administered indefinitely.
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PMID:Clinical aspects of allergic bronchopulmonary aspergillosis. 1245 38

Bronchoscopy is a highly versatile technique in the context of intensive care and has many potentially valuable indications. Safety is of paramount importance and the risks in critically unstable patients are correspondingly greater than in more stable children. The main contraindication to bronchoscopy is if it will provide no useful information. The procedure is obviously more risky in children with severe hypoxia, uncontrolled bleeding diathesis, cardiac failure or severe pulmonary hypertension. Monitoring should include at least oxygen saturation, blood pressure (ideally by continuous, invasive monitoring) and preferably capnography. Indications for bronchoscopy in paediatric intensive care include endobronchial toilet, sometimes instilling recombinant human DNAase even in children who do not have cystic fibrosis; checking tube patency and position; assisting in a difficult intubation or tube change; achieving the selective intubation of a main bronchus; the diagnosis and management of ventilator-associated pneumonia or the ventilated, immunocompromised host; the assessment of lobar collapse or focal hyperinflation; airway stent assessment; assessment of stridor on extubation and the diagnosis of any associated disease. New iatrogenic complications are also likely to be discovered. The procedure is very safe if performed by experienced operators with back-up from doctors skilled in airway management and the monitoring of sick children.
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PMID:Bronchoscopy in paediatric intensive care. 1261 34

Since anion secretion inhibitors reproduce important aspects of cystic fibrosis (CF) lung disease, the effects of these antagonists on airway mucus morphology were assessed in isolated perfused pig lungs. Maximal inhibitory concentrations of bumetanide and dimethylamiloride, which respectively block Cl- and HCO3- secretion in porcine airways, induced the formation of dense 'plastered' mucus on the airway surface, depletion of periciliary fluid and collapse of cilia. This effect was more pronounced when lungs were also exposed to bethanechol to stimulate submucosal gland secretion, when plastered mucus covered > 98 % of the airway surface. Bethanechol also reduced gland duct mucin content in the absence, but not presence, of the anion secretion inhibitors. Anion secretion inhibitors did not induce measurable increases in goblet cell degranulation. We conclude that inhibition of anion and liquid secretion in porcine lungs disrupts the normal morphology of airway surface mucus, providing further evidence that impaired anion secretion alone could account for critical aspects of CF lung disease.
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PMID:Disruptive effects of anion secretion inhibitors on airway mucus morphology in isolated perfused pig lung. 1270 45

Early detection of progressive lung disease in cystic fibrosis (CF) may lead to better treatment and prognosis. Routine lung function indices may be relatively insensitive markers of peripheral airway obstruction and alveolar collapse. We hypothesized that the single-breath diffusion capacity of the lung for carbon monoxide (DLCO) would change before tests of airway function in patients with CF. We assessed lung function longitudinally in 53 children with CF during a mean period of 3.8 years to determine whether the diffusion capacity of the lung becomes abnormal before more conventional indices of lung function do. Within patients, DLCO was slightly elevated and remained stable, while forced expired volume in 1 sec (FEV1) and forced vital capacity (FVC) declined progressively (mean individual decline, -1.8% and -0.8% of predicted). Cross-sectionally, this decline was faster (mean group decline -3.8% and -2.8% of predicted), indicating an additional cohort effect. Normalized diffusion capacity at an early stage of CF is slightly elevated and is preserved in spite of progressive obstructive lung disease. This can be attributed to alterations in pulmonary and bronchial circulation due to loss of function and/or number of alveolar units. Diffusion capacity at rest does not appear to be a suitable early marker of progressive deterioration of CF lung disease.
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PMID:Preserved diffusion capacity in children with cystic fibrosis. 1467 90

Glucocorticoids are frequently used to treat patients with pulmonary diseases, but continuous long-term use of glucocorticoids may lead to significant bone loss and an increased risk of fragility fractures. Patients with certain lung diseases, regardless of pharmacotherapy-particularly COPD and cystic fibrosis-and patients waiting for lung transplantation are also at increased risk of osteoporosis. Fragility fractures, especially of the hip, will have substantial effects on the health and well-being of older patients. Vertebral collapse and kyphosis secondary to glucocorticoid-induced osteoporosis (GIO) may affect lung function. Identification of patients with osteopenia, osteoporosis, or fragility fractures related to osteoporosis is strongly recommended and should lead to appropriate treatment. Prevention of GIO in patients receiving continuous oral glucocorticoids is also recommended. In patients receiving either high-dose inhaled glucocorticoids or low- to medium-dose inhaled glucocorticoids with frequent courses of oral glucocorticoids, bone mineral density measurements should be performed to screen for osteopenia and osteoporosis. A bisphosphonate (risedronate or alendronate), calcium and vitamin D supplementation, and lifestyle modifications are recommended for the prevention and treatment of GIO.
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PMID:Recognizing and treating glucocorticoid-induced osteoporosis in patients with pulmonary diseases. 1513 1


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