Human immunodeficiency virus infections and the respiratory system in children.
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Biomedical subjects
Publications and source records attributed to M R Bye.
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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.
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.
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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%.
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.
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.
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.
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 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.
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.
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.
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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.