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Rationale: Asthma clinical guidelines suggest written asthma action plans are essential for improving self-management and outcomes., Objectives: To assess the efficacy of written instructions in the form of a written asthma action plan provided by subspecialist physicians as part of usual asthma care during office visits., Methods: A total of 407 children and adults with persistent asthma receiving first-time care in pulmonary and allergy practices at 4 urban medical centers were randomized to receive either written instructions (n = 204) or no written instructions other than prescriptions (n = 203) from physicians., Measurements and Main Results: Using written asthma action plan forms as a vehicle for providing self-management instructions did not have a significant effect on any of the primary outcomes: (1) asthma symptom frequency, (2) emergency visits, or (3) asthma quality of life from baseline to 12-month follow-up. Both groups showed similar and significant reductions in asthma symptom frequency (daytime symptoms [P < 0.0001], nocturnal symptoms [P < 0.0001], β-agonist use [P < 0.0001]). There was also a significant reduction in emergency visits for the intervention (P < 0.0001) and control (P < 0.0006) groups. There was significant improvement in asthma quality-of-life scores for adults (P < 0.0001) and pediatric caregivers (P < 0.0001)., Conclusions: Our results suggest that using a written asthma action plan form as a vehicle for providing asthma management instructions to patients with persistent asthma who are receiving subspecialty care for the first time confers no added benefit beyond subspecialty-based medical care and education for asthma. Clinical trial registered with www.clinicaltrials.gov (NCT 00149461).

We present 3 children with massive pulmonary embolism and review 17 recent pediatric reports. Malignancies were a frequent cause (40%), and sudden death was common (60%). Compared with adults, diagnosis was more likely to be made at autopsy (P < .0001), more children were treated with embolectomy/thrombectomy (P = .0006), and mortality was greater (P = .03).

Study Design: Retrospective cohort study., Objective: The purpose of this study is to evaluate pulmonary function and quality of life (QOL) in children treated with early spinal fusion for progressive congenital scoliosis., Summary of Background Data: The relationships between radiographic measures, pulmonary function tests (PFT), and QOL were examined. METHODS.: Twenty-one patients with congenital scoliosis treated with early spinal fusion were evaluated using radiographs, PFT, and the Child Health Questionnaire (CHQ) at 12.6 +/- 3.5 years. They were 6.9 +/- 2.3 years postdefinitive fusion, which occurred at 4.9 +/- 3.1 year of age. The cohort was also divided in two groups, "thoracic fusion" (apex above the thoracolumbar T-L junction) and "nonthoracic fusion" (below T-L junction)., Results: Forced vital capacity, forced expiratory volume (FEV1), vital capacity (P < 0.0001), and total lung capacity (P = 0.017) were significantly lower compared with healthy children. CHQ scores were significantly lower than in healthy children in physical function (P = 0.036), general health (P = 0.007), physical summary (P = 0.026), and parental impact/emotional (P = 0.01). Correlation analysis showed that the degrees of thoracic curves were negatively correlated with FEV1 (P < 0.05), family activities, role/social physical (P < 0.05), and physical summary (P < 0.01). The degree of kyphosis was negatively correlated with Self-Esteem (P < 0.01). Patients who had thoracic fusions had shorter spinal height (P = 0.049), lower forced vital capacity (P = 0.004), FEV (P = 0.012), vital capacity (P = 0.031), and reported more pain (P = 0.033) than nonthoracic fused., Conclusion: Compared with healthy peers, congenital scoliosis patients treated with early spinal fusion have worse PFT and QOL scores at 6.9 years follow up. Patients with thoracic fusions had shorter spines, worse pulmonary function, and more pain than nonthoracic fused. The results may support alternatives to early spinal fusion such as growing rods, epiphysiodesis, and distraction thoracoplasty. Current efforts are underway to compare outcomes of this study to those of other treatments.

We performed pulmonary function testing in 20 infants (11 male and 9 female; ages 3-30 months) with sickle cell disease to assess whether abnormal lung function develops early in life. Respiratory system compliance (Crs) and resistance (Rrs) were measured by the passive occlusion technique, functional residual capacity (FRC) was measured by the nitrogen washout technique, and tidal flow-volume loops and partial expiratory flow-volume curves were obtained by the thoracoabdominal compression technique to detect airway obstruction. Patients with Hb SS (Group I, n = 12) had significantly lower hemoglobin levels and a higher (but not significant) incidence of acute chest syndrome (ACS), vasoocclusive crisis (VOC), splenic sequestration, transfusions, and history of intermittent bronchospasm compared to with patients with hemoglobinopathies Hb SC, Hb Sbt and Hb SF (Group II; n = 8). Both groups had elevated FRC, decreased maximum expiratory flows at FRC (V'max,FRC), and decreased time needed to reach peak expiratory flow (tme/tE), suggesting lower airway obstruction (LAO) and hyperinflation. Restrictive disease was found in only three patients of Group I. Our findings suggest that in sickle cell disease (especially among patients with Hb SS), abnormal lung function (predominantly LAO) may be present in early infancy. Airway reactivity may play a role in the pathogenesis, but the relation to VOC or ACS remains unclear.

Because children acquire HIV infection differently than adults, this article begins with a discussion of the epidemiology of AIDS in children. This is followed by a discussion of factors related to progression of the disease and survival in pediatric AIDS. A discussion of the pulmonary manifestations in children is followed by a suggested approach to the HIV-infected child with respiratory symptoms.

Adolescent pregnancy has increased in the past decade (1-5), often in association with poverty, poor education, and inadequate prenatal care. While it has been suggested that adverse pregnancy outcomes are more common among adolescents in the inner city, recent data show that in a white, middle-class population teenaged mothers are more likely to have adverse pregnancy outcomes (5). Asthma is also becoming more common, with an incidence of at least 6.6% in 15-16 year old girls (6,7). Poverty and living in the inner city are associated with increased morbidity and mortality from asthma (8-11). Adolescents with asthma who become pregnant provide an added challenge to the physician who must consider the impact of the pregnancy on the asthma and vice versa. The physician must understand the effects of both the asthma medication and/or poorly controlled disease on the fetus. The physician must also be able to convey this information to the adolescent in a developmentally appropriate manner to enable the patient to make informed health care decisions (12).

An HIV-positive child presented with a pneumothorax secondary to cavitary Pneumocystis carinii pneumonia (PCP). Lymphocytic interstitial pneumonitis had been evident on earlier radiographs but had resolved, concurrent with a decrease in her CD4 counts, before the radiographic changes of PCP became evident. As immune function declines in HIV-positive children, the chest radiograph may paradoxically clear. In such a setting, development of focal lung disease, including pneumothorax, may herald Pneumocystis carinii infection.

Background: Pneumocystis carinii pneumonia is a common opportunistic infection in pediatric acquired immunodeficiency syndrome (AIDS). Mortality rates of 34% have been reported for the acute infection, with much higher mortality rates occurring in the presence of respiratory failure. Corticosteroids reduce the morbidity and mortality in adults with AIDS and P carinii pneumonia. We report herein our experience with corticosteroids in P carinii pneumonia in pediatric AIDS., Methods: When the data on adults were published, we began treating our patients with corticosteroids. We compared these children (group 1) with those children treated in a similar manner, but without corticosteroids (group 2)., Results: The two groups had similar ages, initial serum lactate dehydrogenase levels, and initial alveolar-arterial difference in partial pressure of oxygen. There was a significant (P < .001) reduction in the need for mechanical ventilation and in mortality in the corticosteroid-treated group., Conclusion: Corticosteroid therapy during acute infection with P carinii in young children with AIDS appears to significantly reduce morbidity (as measured by the need for mechanical ventilation) and mortality.

While the care of cystic fibrosis (CF) patients has been mainly the province of pediatricians, great improvements in the therapy and life span of CF patients often results in their transition to care by adult physicians. In this review of CF, we begin with an overview of the epidemiology and genetics of the disease, with a discussion of the recently found ion abnormalities that lead to the clinical manifestations. This is followed by a discussion of the pathophysiology. Methods of diagnosis, ranging from the gold standard, the sweat test, to recent advances based on a greater understanding of the genetics of the disease are reviewed. This is followed by a discussion of therapy primarily geared to the treatment of the respiratory complications, as they are the most common lethal factors of the disease. We point out controversies where they exist. Newer forms of therapy such as lung transplantation are discussed, and we finish with a discussion about future therapeutic modalities, some of which are being approved as the paper is in print.

Two infants with the Jarcho-Levin syndrome of vertebral anomalies underwent flexible fibre optic bronchoscopy. Central airway abnormalities not amenable to surgical correction were found in both patients. These abnormalities may contribute significantly to the respiratory failure seen in affected infants, and should be considered when evaluating continuing medical support.

Objective: To assess the pulmonary function of children with asthma at the time of a routine follow-up visit when they were judged to be clinically well., Design: Pulmonary function tests were performed on children with a previous diagnosis of asthma. None had had any symptoms for at least 6 weeks. All had normal Wright peak flow rates and normal findings at physical examination., Setting: General pediatric and pediatric pulmonary clinic at a municipal hospital in the Bronx, NY., Patients: Sixty-five children with asthma who fit the above criteria. All were able to perform spirometry adequately. Their mean (+/- SD) age was 11 +/- 3 years, and their median age was 10 years., Selection Procedures: All clinic patients were screened as possible participants. The 65 patients were deemed eligible, and were willing and able to perform spirometry., Interventions: None., Measurements and Results: The group's mean (+/- SD) forced expiratory volume in 1 second/forced vital capacity ratio was 80% +/- 8%, and their mean mid-maximal expiratory flow was 66% +/- 23% of the predicted value. Of the 65 patients, 11 (17%) had forced expiratory volume in 1 second/forced vital capacity ratios of less than 72%, and 35 (54%) had a mid-maximal expiratory flow of less than 65% of the predicted value., Conclusion: Failure to perform spirometry in children with asthma results in underdiagnosis of airflow obstruction. This may result in obstruction persisting into adulthood and/or progression to hyperinflation.

To ascertain the effect of cytomegalovirus (CMV) infection on the course of Pneumocystis carinii pneumonia (PCP) in children with acquired immunodeficiency syndrome (AIDS), we reviewed the charts of all children with AIDS who also had a lung biopsy specimen or a bronchoalveolar lavage specimen cultured for CMV at the time PCP was diagnosed. The data indicate that children with AIDS and PCP whose cultures are positive for CMV do not have a poorer prognosis during a first episode of PCP compared with children whose cultures are negative for CMV.

Bronchial carcinoid is an uncommon entity in youth. To better understand the essentials of its diagnosis and management, we present two cases occurring in adolescent patients and a review of the literature. We emphasize the clinical and pathophysiologic aspects that facilitate its diagnosis. Increased awareness may lead to earlier diagnosis, preservation of lung tissue, and function, as well as improved prognosis.

We present our experience with 54 episodes of Pneumocystis carinii pneumonia in 50 young children with AIDS, all but one representing congenitally acquired infection. Findings at history and physical examination are not helpful in suggesting the diagnosis. The diagnosis is suggested by marked hypoxemia, diffuse disease on chest radiograph, and elevated serum LDH level. Because important aspects of the history may be withheld, a high index of suspicion may be necessary for the correct diagnosis. The mortality rate for ventilated patients was 50%.

Between 1968 and 1983, 54 patients underwent surgery for symptomatic aortic arch and pulmonary artery anomalies at St Christopher's Hospital for Children. Presenting symptoms included stridor, wheeze, apnea, recurrent pulmonary infections, or dysphagia. Diagnosis was established with chest roentgenogram, bronchoscopy, barium esophagram, and arteriography. Four types of vascular anomalies were encountered; double aortic arch (24 patients), right aortic arch with left ligamentum arteriosum (17 patients), anomalous innominate artery (10 patients), and pulmonary artery sling (three patients). There were no intraoperative deaths and only one postoperative death. All surviving patients had immediate relief of their severe respiratory or swallowing symptoms. Mild respiratory symptoms persisted postoperatively from 3 months to 4 years and included frequent or severe upper respiratory infections, persistent cough, stridor and pneumonia. Five of the 53 surviving patients were lost to follow up. The remaining 48 patients were followed from 6 months to 14 years and all but one patient noted complete resolution of all respiratory symptoms. Twenty-nine patients at follow up were old enough to undergo pulmonary function testing including vital capacity, functional residual capacity, and inspiratory and expiratory flow volume loops. Seventeen of these 29 asymptomatic patients consented to these studies, and nine of these patients had abnormal flow volume loops indicative of significant central airway obstruction; the other eight studies were normal. We conclude that surgical repair for vascular rings and slings is safe and symptomatically efficacious. However, anatomic tracheal or bronchial distortion persists in a significant number of these patients as evaluated by pulmonary function studies.

We studied the benefits of in-hospital therapy on exercise capacity and related these changes to improvements in lung function in 17 patients with cystic fibrosis (CF) of moderate to extreme severity, as defined by results of pulmonary function tests performed at admission and discharge. Tolerance and adaptations to exercise were assessed from measures of peak work capacity (PWC), peak heart rate (PHR), and peak ventilation (PVE) obtained during an incremental exercise test. Treatment lasted from nine to 18 days. All measures of lung function improved; there also were significant increases in PWC, PHR, PVE, and PHR/PWC. Exercise-induced arterial desaturation was less at discharge than at admission. The PWC of the most severely affected patients remained abnormally low, and their adaptations to exercise were abnormal at discharge. We concluded that intensive in-hospital therapy will significantly improve exercise tolerance and lung function in patients with CF with moderate to severe pulmonary dysfunction.

Between October, 1985 and May 1987, 29 children (mean age 22 +/- 22 months, range 2-54 months) with AIDS or ARC developed acute respiratory illness. The initial diagnostic procedure was flexible fiberoptic bronchoscopy, with bronchoalveolar lavage (BAL). BAL was positive for Pneumocystis carinii in 14 and for respiratory syncytial virus, Staphylococcus aureus, and Escherichia coli in 3 additional patients. Subsequent lung tissue analysis and/or clinical course suggested no false negative lavages. Complications possibly related to the procedure occurred in two patients. We find BAL an effective diagnostic technique in these patients, offering a less invasive alternative to open lung biopsy.

A 9-year-old with immunodeficiency developed a severe, diffuse respiratory illness that necessitated mechanical ventilation. Open lung biopsy revealed Respiratory Syncytial Virus (RSV) as the sole pathogen. RSV detection should be included in the differential diagnosis of diffuse lung disease in an immunocompromised child.

Eighteen children with the acquired immunodeficiency syndrome (AIDS) were diagnosed as having Pneumocystis carinii pneumonia (PCP) by either open lung biopsy or bronchoalveolar lavage. Seven patients (39%) died during the acute illness. Alveolar-arterial oxygen gradients at the time of presentation and lactate dehydrogenase levels did not distinguish survivors from nonsurvivors. Total lymphocyte and T4 cell counts were low in children who died during the initial PCP infection but had considerable overlap with survivors. Response to phytohemagglutinin was measured in 5 of the 7 patients who died initially. In these patients, the mean phytohemagglutinin response was 1977 cpm. Of the 11 early survivors, 10 died within 27 months after PCP. Mean phytohemagglutinin response was 46,079 cpm in patients who died within 1 year, and 44,768 cpm in those who died later. Only 1 child is still alive 5 years after PCP illness. Children with AIDS and PCP infection have high initial mortality and poor long-term prognosis. Response to phytohemagglutinin is helpful in predicting who will survive initial PCP infection.

The role of exercise in the management of the asthmatic child is now understood to be essential for the child's overall health. With the medications available today, a child, even with complicating EIA, can expect to perform as well as his nonasthmatic peers. In fact, with appropriately set expectations, adequate medical management, and supportive parents, coaches and teachers, the asthmatic child can hope to achieve a physical fitness level compatible with a high quality of life. The physician's role is a key one requiring basic knowledge of medical management, as well as supervising the overall effort. One cannot overlook the satisfaction a doctor experiences when assisting the patient in functioning normally, despite underlying disease.

This article evaluates a recently developed qualitative sweat patch test (SPT). A battery-powered stimulator with polymer pad electrodes is used to stimulate sweating, followed by the application of a paper patch which collects the sweat and shows color change on sweat chloride levels above 40 to 50 mEq/l. Our data consist of 66 patients with cystic fibrosis, 75 control subjects, and 37 relatives of patients with cystic fibrosis, excluding 22 subjects (11%) with insufficient sweat. There were no false-negative results among 66 patients with cystic fibrosis, and in the control group, 71 of 75 subjects gave negative results and the other four gave positive results. Of the 37 relatives, eight gave positive results; all of the eight subjects are parents of patients with cystic fibrosis. Our results indicate that SPT is useful and reliable when there is sufficient sweat. This portable and simple patch test may serve as an excellent screening test for cystic fibrosis.