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A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. The clinically suspected diagnosis of pulmonary alveolar microlithiasis (PAM) supported by pathognomonic radiological abnormalities was confirmed by genetic analysis. The clinical presentation of PAM is illustrated by a case and review of the current literature on this subject: you only see what you know. [ABSTRACT FROM AUTHOR]

Unlabelled: Serum procalcitonin (PCT) has been proposed as a marker to identify bacterial infection in children. For optimal management of cystic fibrosis (CF) patients, early recognition of pulmonary exacerbations is necessary, but sensitive biomarkers to do so are lacking. Our study was done to establish baseline values for PCT in children with CF and to compare these to values at onset of a pulmonary exacerbation. Serum PCT values were determined in CF children during an outpatient clinic visit and at onset of treatment with intravenous (IV) antibiotics for a pulmonary exacerbation. Serum PCT was measured using a quantitative immunoassay (BRAHMS Kryptor PCTsensitive, Henningsdorf, Germany). In 92 outpatients (mean age 10.0 years, SD 4.8 years; mean forced expiratory volume in 1 s 91%, SD 18; 9 chronically colonized with Pseudomonas aeruginosa), mean baseline PCT was 0.05 ng/ml (SD 0.07). Mean PCT on admission for IV treatment of pulmonary exacerbation was 0.07 ng/ml (SD 0.06) (n = 22) and not different from the baseline value. PCT values were markedly higher in two CF patients with an acute nonrespiratory infection (central venous catheter-associated bloodstream infection, acute gastroenteritis), demonstrating that they can mount a PCT response.Conclusion: PCT values in CF children are not different from values reported in healthy children. In CF children, PCT values do not rise significantly at the onset of a respiratory exacerbation and thus hold no promise as an early marker to identify a pulmonary exacerbation. [ABSTRACT FROM AUTHOR]

Emergencies always require rapid diagnosis and an urgent or semi-urgent medical, interventional, or surgical action. In most cases radiology plays an essential role in making an accurate diagnosis. Reviewing the causes of acute respiratory pathology in the different pediatric age groups, we thought it would be interesting to divide the pathologies into two main parts: one part concerning pathologies involving the air flow and the other part concerning pathology affecting the pulmonary parenchyma. We acknowledge, however, that both conditions can occur concomitantly. The esophagus is another anatomic structure in the thorax that can be responsible for acute pathology in children. Acute pathology predominantly involving the air flow can be intrinsic and/or extrinsic, affecting the upper airways, trachea, main and segmental bronchi, and the small airways. Acute lung pathology can be congenital, infectious, or less frequently, tumoral or traumatic in children. Pleural pathology and cardiogenic emergencies are discussed also. Acute esophageal pathology is discussed briefly. [ABSTRACT FROM AUTHOR]

Unlabelled: Bronchodilators are often used in the treatment of patients with bronchopulmonary dysplasia (BPD). However, few studies evaluate their efficacy in patients with stable disease beyond the newborn period. Therefore, pulmonary function was measured before and after aerosol treatment with salbutamol (0.25 ml Ventolin 0.5%) and subsequently after aerosol with ipratropium bromide (0.25 ml Atrovent 0.025%). Studies were performed at the corrected postnatal age of 52 +/- 2 weeks in 52 patients who had been ventilated after birth because of newborn lung disease. Twenty-two of these 52 patients had developed BPD. Pulmonary function was measured after sedation and using the PEDS system. Expiratory resistance (median 52.1 versus 39.1 cmH2O/l/s; P < .008) and inspiratory resistance (median 42.5 vs 27.8 cmH2O/l/s; P < .04) were significantly worse in BPD patients at the age of 1 year. Half of the BPD patients had a decrease in pulmonary resistance after salbutamol. However, there was no statistically significant decrease in pulmonary resistance after salbutamol or ipratropium in the BPD patients as a group. After salbutamol pulmonary resistance significantly worsened in the patients who did not develop BPD.Conclusion: Although individual patients may benefit, routine administration of bronchodilators seems not warranted in stable BPD patients at the age of 1 year. [ABSTRACT FROM AUTHOR]

Unlabelled: Pulmonary function tests were obtained in 11 patients with primary ciliary dyskinesia. Their mean age was 15 years (range 6-32). Their pulmonary function was obstructive, with a vital capacity (mean+/-SD) of 75%+/-20% predicted, a forced expiratory volume in 1s (FEV1) of 63%+/-20% predicted and a raised residual volume of 169%+/-50% predicted. After inhalation of 200 microg of salbutamol the mean change in FEV1 was + 13.2%+/-9.6% of the baseline value. In the 10 oldest patients, lung function had been measured at regular intervals during 3 20 years. Interestingly, during childhood and adolescence the evolution was not unfavourable: vital capacity increased by 8%+/-20% and FEV1 remained stable (mean change 0.3%+/-12%). Only 2 patients had an unfavourable evolution.Conclusion: At time of diagnosis, patients with primary ciliary dyskinesia have partially reversible obstructive airway disease. During regular follow up and therapy, there is no evidence of a further decline in lung function. Patients with associated immunodeficiency or important damage at the start of therapy may have a worse prognosis. [ABSTRACT FROM AUTHOR]

Unlabelled: In patients with cystic fibrosis (CF) and chronic colonisation with Pseudomonas aeruginosa, specific anti-pseudomonal IgG and IgA, as well as serum immunoreactive protein C, WBC and differential count, ESR, pulmonary function and chest radiograph score were determined before and after a 2 week intravenous course of anti-pseudomonal antibiotics in 32 cases of acute exacerbation of pulmonary infection. Specific anti-pseudomonal IgA but not specific anti-pseudomonal IgG decreased significantly after treatment. Log of anti-pseudomonal IgA but not log anti-pseudomonal IgG correlated well with disease severity as assessed by the Brasfield chest radiograph score (r 0.57), forced expiratory volume in 1 s (r 0.6) as well as C-reactive protein (r 0.62).Conclusion: Specific anti-pseudomonal IgA may be a better parameter than specific IgG in the follow up of lung infection in patients with CF, probably because it more closely reflects ongoing endobronchial infection, the major pathology in CF lungs. [ABSTRACT FROM AUTHOR]

A survey is given of the pathophysiology of the main alterations in the small intestine of cystic fibrosis patients. Special attention is paid to the understanding of meconium ileus in the fetus and the newborn, the repercussion of duodenal acidity on the duodenal mucosa and the intraduodenal digestion, and primary and secondary biochemical alterations in the secretory-digestive-absorptive function of the small-intestinal mucosa. The meconium equivalent syndrome and its connection with the atypical course of intussusception and appendiceal perforation with silent pelvic abscess are also discussed. [ABSTRACT FROM AUTHOR]

Seven cases of toddler's fracture are reported. The value of radionuclide bone imaging in helping to establish this diagnosis is discussed. [ABSTRACT FROM AUTHOR]

In a cystic fibrosis (CF) patient a right lower quadrant (RLQ) mass may be a difficult diagnostic problem. Most frequently it is due to a distal intestinal obstruction syndrome also called meconium ileus equivalent, but the possibility of intussusception and appendiceal abscess should also be considered. We describe three CF patients with an appendiceal abscess seen in a 4-year period. All three patients had a palpable RLQ mass. Chronicity and obliteration of the appendiceal lumen with abnormally viscid mucus may lead to concealed perforation and be responsible for the atypical presentation. [ABSTRACT FROM AUTHOR]