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The aim of this study was to evaluate the ability of high-resolution computerized tomography (HRCT) of the chest and chest x-rays (CXR) to determine efficacy of inhaled recombinant human DNase (rhDNase) in cystic fibrosis (CF) patients younger than 5 years of age. A randomized, double-blind, placebo-controlled pilot study of 12 patients with CF younger than 5 years of age, attending the University of Michigan Cystic Fibrosis Center (Ann Arbor, MI) was conducted. The changes in the HRCT and CXR score from baseline to day 100 of therapy were assessed using a previously validated scoring system. The mean changes of HRCT scores between the rhDNase and placebo groups were found to be significant at the 95% level, with mean change +/- SE mean of - 1.00 +/- 0.53 and 0.58 +/- 0.24 for rhDNase and placebo groups, respectively (P = 0.02). The difference in CXR score was not significant between the two groups. An analysis was performed to relate HRCT subscores to CXR score; only thickening of the intra-interlobular septae was significantly correlated with the total CXR score (r = - 0.7, P < 0.01). There was improvement in the parents' assessments of the patients' well-being, with improvement in physical activity, decreased cough, sleep quality, and appetite in those subjects receiving rhDNase. We conclude that the administration of rhDNase was associated with improvement in the HRCT scan in CF patients younger than 5 years of age. Findings indicate that HRCT of the chest is useful and sensitive in studying responses to therapy in patients with CF lung disease. To our knowledge, this is the first report of the use of HRCT to assess the effectiveness of a therapeutic modality in so young a CF patient population.

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Four patients with severe cystic fibrosis lung disease, anorexia and weight loss, received Megestrol Acetate (MA), as an appetite stimulant. The initial dose was 400-800 mg daily and was continued for 6-15 months. Appetite was improved, with significant weight gain in all patients and an increase in their weight for age percentile from <5% at the start of the study to approximately the 25(th) percentile after 6 months of use and improvement in quality of life. One patient discontinued MA after 6 months, and subsequently appetite and weight were depressed.

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We sublocalized the yeast nucleoporin Nup82 to the cytoplasmic side of the nuclear pore complex (NPC) by immunoelectron microscopy. Moreover, by in vitro binding assays we showed that Nup82 interacts with the C-terminal region of Nup159, a yeast nucleoporin that previously was also localized to the cytoplasmic side of the NPC. Hence, the two nucleoporins, Nup82 and Nup159, form a cytoplasmically oriented subcomplex that is likely to be part of the fibers emanating from the cytoplasmic ring of the NPC. Overexpression of Rss1/Gle1, a putative nucleoporin and/or mRNA transport factor, was shown previously to partially rescue depletion of Nup159. We show here that overexpression of Rss1/Gle1 also partially rescued depletion of Nup82. Depletion of either Nup82, Nup159, or Rss1/Gle1 was shown previously to inhibit mRNA export. As was reported previously for depletion of Nup159 or of Rss1/Gle1, we show here that depletion of Nup82 has no detectable effect on classical nuclear localization sequence-mediated nuclear import. In summary, the nucleoporins Nup159 and Nup82 form a cytoplasmically oriented subcomplex of the NPC that is likely associated with Rss1/Gle1; this complex is essential for RNA export, but not for classical nuclear localization sequence-mediated nuclear protein import.

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Partnership between health care providers and patients is important for controlling illness. A limited number of studies show how to assess health professionals' communication and partnering behavior. The relationship between these aspects of professional behavior and enhanced management of disease by patients has received little empirical study. The research reported here developed a Health Care Providers' Teaching and Communication Behavior (TCB) scale for assessing the teaching and communication behavior of clinicians treating patients with asthma. Such a tool is needed for research related to provider-patient relationships and for evaluation of professionals' performance.

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We have isolated and characterized the gene encoding a novel essential nucleoporin of 82 kD, termed NUP82. Indirect immunofluorescence of cells containing an epitope tagged copy of the NUP82 localized it to the nuclear pore complex (NPC). Primary structure analysis indicates that the COOH-terminal 195 amino acids contain a putative coiled-coil domain. Deletion of the COOH-terminal 87 amino acids of this domain causes slower cell growth; deletion of the COOH-terminal 108 amino acids results in slower growth at 30 degrees C and lethality at 37 degrees C. Cells in which the last 108 amino acids of NUP82 have been deleted, when shifted to 37 degrees C, do not display any gross morphological defects in their nuclear pore complexes or nuclear envelopes. They do, however, accumulate poly(A)+ RNA in their nuclei at 37 degrees C. We propose that NUP82 acts as a linker to tether nucleoporins directly involved in nuclear transport to pore scaffolding via its coiled-coil domain.

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We analyzed continuous nebulized terbutaline (CNT) therapy in 19 patients with 27 admissions for severe asthma and impending respiratory failure who failed to respond to our standard asthma protocol of methylprednisolone, aminophylline, and intermittently nebulized terbutaline. Terbutaline was administered by continuous face mask nebulization at a dose equaling the most frequent previous intermittent dose per hour (4 mg per hour). No patient with frank respiratory failure (i.e., PaCO2 greater than or equal to 60 torr, exhaustion, or coma) was studied. All patients improved, and therapy was stopped in a mean of 15.4 hours (range 3 to 37 hours). The average heart rate did not increase over baseline measurements through 24 hours of CNT. The mean clinical asthma score improved significantly during 8 hours, falling from 6.9 to 3.2 (p greater than 0.001). In 14 patients whose PaCO2 was greater than or equal to 39 torr (range 39 to 58 torr) and clinical asthma score was 6 or greater, PaCO2 decreased a mean of 11.7 torr during a mean of 8.1 hours. In six patients whose PaCO2 was 45 torr or greater at the start of CNT (mean 49, range 45 to 58 torr) and in whom we would have previously treated with intravenous isoproterenol, PaCO2 decreased a mean of 15 torr in an average of 8.7 hours. This preliminary study suggests that CNT is an effective therapy for severe asthma in children.

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Management of acute asthma in the pediatric population is based almost entirely on clinical evidence of severity. Although pulmonary function testing has been advocated to improve evaluation, it is difficult in the pediatric patient and not routinely practiced. A clinical scoring system has been devised to help standardize evaluation, but has not been validated by comparison of the results of clinical scoring with those of arterial blood oxygen levels as determined by blood gas analysis. This study was undertaken to compare clinical scoring of pediatric asthma patients with the results of arterial blood gas analysis. Thirty-eight children between the ages of 2 and 13 having 42 episodes of acute asthma were evaluated. The average age was 5.4 years. The average clinical score was 2.62; arterial blood for analysis was obtained in 37 (88%), with an average PaO2 of 81.7 mm Hg. None of the children had CO2 retention. There was no correlation between the clinical score of the children on presentation and the severity of hypoxia (correlation coefficient = -0.149). Comparison of age and arterial oxygen tension revealed a trend toward worsening hypoxemia with diminishing age from 6 to 2 years, which was not identified by clinical scoring. We conclude that clinical scoring is inaccurate for the assessment of hypoxemia in the pediatric age group. Arterial blood gas determination should be used to assess the severity of hypoxemia in the emergency treatment of pediatric asthma patients.

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