Markedly reduced mortality associated with corticosteroid therapy of Pneumocystis carinii pneumonia in children with acquired immunodeficiency syndrome.

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.

HIV in children.

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.

Diagnostic bronchoalveolar lavage in children with AIDS.

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.

Lung function in infants with sickle cell disease.

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.

Pneumocystis carinii pneumonia in young children with AIDS.

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%.

Asthma in the pregnant adolescent: a review.

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).

Vascular rings and slings: long-term follow-up of pulmonary function.

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.

Severe RSV bronchiolitis in an immunocompromised child.

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.

Evaluation of a paper-patch test for sweat chloride determination.

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.

Exercise programs for asthmatic children.

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.

Bronchial carcinoid in adolescents.

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.

Airway abnormalities in Jarcho-Levin syndrome: a report of two cases.

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.

Cystic fibrosis.

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.

Prognostic factors and life expectancy in children with acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia.

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.