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Biomedical subjects

E L Effmann

Publications and source records attributed to E L Effmann.

At least 19 recordsLinked to original sources
PubMed2001

Congenital masses of the lung: prenatal and postnatal imaging evaluation.

Congenital masses of the lung are a spectrum of interrelated abnormalities that includes congenital lobar overinflation, bronchogenic cyst, congenital cystic adenomatoid malformation (CCAM) and sequestration. The prenatal and postnatal imaging features of these lesions are reviewed, emphasizing the importance of serial prenatal sonograms and postnatal imaging studies, including radiography and computed tomography. Masses that become inconspicuous, or disappear on serial prenatal sonograms are discussed, as well as the importance of postnatal imaging studies in the evaluation of these lesions. Finally, the management of congenital masses of the lung is reviewed, emphasizing the importance of imaging studies in the preoperative evaluation.

Bronchogenic Cyst↗
PubMed2000

Pulmonary infections in immunocompromised hosts: the importance of correlating the conventional radiologic appearance with the clinical setting.

The lung is one of the most frequently involved organs in a variety of complications in the immunocompromised host. Among the pulmonary complications that occur in this kind of patient, infection is the most common and is associated with high morbidity and mortality. Although chest radiography and computed tomography (CT) are essential diagnostic tools, radiologists often have difficulty in establishing the correct diagnosis on the basis of radiologic findings alone. The reasons are that the immunocompromised host is potentially susceptible to infection from many different microorganisms and that radiologic findings are seldom specific for the detection of a particular pathogen. Experience has shown that a particular clinical setting predisposes patients to infection by particular pathogens. The setting comprises (a) the specific epidemiologic or environmental exposure, (b) the type of underlying immune defect, (c) the duration and severity of immune compromise, and (d) the progression rate and pattern of the radiologic abnormality. Correlating the radiologic appearance with the clinical setting can expedite diagnosis and appropriate therapy. In this review, the authors describe the clinical settings that are helpful in choosing the radiologic approach to treatment of the immunocompromised host who presents with suspected pulmonary infection.

B-Lymphocytes↗
PubMed1998

Systemic sclerosis: using high-resolution CT to detect lung disease in children.

OBJECTIVE: The purpose of this study was to determine the prevalence of interstitial lung disease and the severity of disease in children with systemic sclerosis using high-resolution CT (HRCT). SUBJECTS AND METHODS: Eleven children (mean age, 11 years) with scleroderma underwent HRCT, chest radiography, and pulmonary function testing. Eight of these 11 patients also underwent follow-up HRCT. HRCT studies were assessed by two observers for ground-glass attenuation, honeycombing, and other abnormalities. Profusion scores for ground-glass attenuation and honeycombing were determined by multiplying severity of disease by the percentage of lung involvement. RESULTS: Chest radiographs predicted interstitial lung disease in only two patients, whereas HRCT showed interstitial lung disease in eight patients (p = .05). On HRCT, ground-glass attenuation was found in eight patients (73%), honeycombing in five patients (45%), linear opacities in six patients (55%), and subpleural micronodules in seven patients (64%). By the end of the study, 10 patients (91%) had evidence of interstitial lung disease on HRCT. Overall, profusion scores for these 10 patients showed four patients with mild, one with moderate, and five with severe disease. Also, HRCT revealed worsening disease in three of eight patients. We found no correlation between duration of scleroderma and severity of interstitial lung disease (p > .02). Seven patients with evidence of lung disease on HRCT had abnormal results on pulmonary function tests; patients with the highest scores for ground-glass attenuation had the most abnormal results on pulmonary function tests (p < .01). CONCLUSION: HRCT shows significant pulmonary disease in children with systemic sclerosis, revealing abnormalities in 91% of our patients. Pulmonary disease should be suspected in children with scleroderma, even if the chest radiograph has normal findings.

Adolescent↗
PubMed1997

Congenital masses of the lung: changes in cross-sectional area during gestation.

PURPOSE: We assessed the growth of congenital masses of the lung during gestation using computer-assisted planimetry. METHODS: The prenatal sonograms of 8 fetuses with congenital masses of the lung were reviewed. RESULTS: The cross-sectional area of the mass and chest were measured on the same transverse image using computer-assisted planimetry, and the percentage of the chest occupied by the mass was determined for each study. Four masses had pathologic features of type II congenital cystic adenomatoid malformation and intralobar sequestration (CCAM/ILS), 2 were type II CCAM, 1 was type I CCAM, and 1 was bronchial atresia with bronchiectasis. Four masses increased in cross-sectional area during gestation, 1 decreased, 2 were essentially unchanged, and 1 showed an initial increase in cross-sectional area followed by a decrease later in gestation. No consistent growth pattern was seen among masses with similar histologic characteristics. The percentage of the cross-sectional area of the chest occupied by the mass decreased in 7 fetuses and was virtually unchanged in 1 during gestation. All the fetuses survived to term; the infants had an uncomplicated postnatal course and underwent surgical resection of the mass during the first year of life. CONCLUSIONS: This study showed that in a fetus with a congenital mass of the lung and a favorable clinical outcome, growth of the chest exceeds any growth of the mass that may occur and masses with the same pathologic diagnosis have different patterns of growth in utero.

Bronchial Diseases↗
PubMed1997

Disappearing fetal lung masses: importance of postnatal imaging studies.

BACKGROUND: The "disappearance" of congenital masses of the lung on prenatal sonograms has been described, but the importance of postnatal imaging studies in these children is unknown. OBJECTIVE: The objective of this work was to study the utility of radiographs and CT scans in asymptomatic infants with congenital masses of the lung that partially or completely resolve on prenatal sonograms performed late in gestation. MATERIALS AND METHODS: The prenatal sonograms, postnatal imaging studies, surgical findings, and pathologic diagnoses of seven children with an echogenic mass of the lung that improved or disappeared on prenatal sonograms were reviewed. RESULTS: All masses were type II congenital cystic adenomatoid malformation, with features of intralobar sequestration also being found in four. An unsuspected extralobar sequestration adjacent to a left lower lobe mass was found at surgery in one patient. All masses were hyperechoic compared with normal lung on sonograms prior to 32 weeks of gestation, with cysts being seen in four. On scans after 32 weeks, four of the masses had resolved completely and three showed subtle increased echogenicity compared with normal lung. Cysts completely resolved in two of four cases. Postnatal radiographs showed subtle abnormalities in four infants, a hyperlucent lobe in one, a soft tissue mass with adjacent hyperlucency in one, and normal findings in one. CT scans were abnormal in all cases, with air-filled cysts and soft tissue in six and a hyperinflated lobe in one. CONCLUSION: Children with "disappearing" fetal lung masses have persistent abnormalities after birth that are often subtle on radiographs but are well demonstrated with CT.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed1997

High-resolution CT of pediatric lung disease: imaging findings.

The HRCT findings of diseases of the pulmonary parenchyma and airways in children are often similar to those seen in adults. However, some conditions, particularly IPF, pulmonary fibrosis associated with scleroderma, and Langerhans histiocytosis, may have different presentations and a more rapid progression in children than in adults. HRCT may be helpful for assessing patients with suspected diseases of the pulmonary parenchyma or airways, particularly when radiographic findings are normal or nonspecific.

Adolescent↗
PubMed1995

Platelet-derived growth factor receptor alpha subunit deleted Patch mouse exhibits severe cardiovascular dysmorphogenesis.

Patch (Ph) mice, whose platelet-derived growth factor receptor alpha subunit (alpha PDGFR) gene has been deleted, have been used to elucidate requirements for alpha PDGFR for normal murine development. In this report we evaluate the role of alpha PDGFR in cardiovascular development by using in situ hybridization to follow the changing pattern of alpha PDGFR expression in cardiovascular tissues after embryonic day 13, and comparing this pattern with the pattern of cardiovascular defects observed in homozygous Ph mutants. Both mesodermally derived and neural crest-derived components of the cardiovascular system are severely dysmorphic in Ph/Ph embryos and those structures most severely affected are those that normally express alpha PDGFR mRNA at the highest levels and for the longest duration. Ph/Ph vessels appear to be lined with a normal endothelium, but contain a reduced number of smooth muscle cells and are fragile during processing for histology. The myocardium is thin, the heart is small and dysmorphic, the valves are malformed, and the interventricular and interatrial septa of the heart are defective. In the outflow tract, the spectrum of defects includes both persistent truncus arteriosus and double outlet right ventricle. This pattern of abnormalities is consistent with the hypothesis that deletion of alpha PDGFR results in a functional ablation of cranial neural crest cells, and that mesodermally derived components of the vascular system also require alpha PDGFR.

Animals↗
PubMed1995

Comparison of helical and conventional chest CT in the uncooperative pediatric patient.

The objective of this study was to compare the quality of conventional and helical computed tomography (CT) images in children unable to breath-hold. Sixteen patients (age range 1.1-7.5 years) underwent both conventional and helical chest CT on a General Electric HiSpeed Advantage scanner at a mean study interval of 3.8 months. Conventional 0.6-s scans were reconstructed with standard algorithm while 1.0-s helical studies were reconstructed with standard and bone algorithms. Three blinded observers retrospectively evaluated the images. There was no significant difference in motion, image sharpness and anatomic resolution for helical and conventional scans reconstructed with standard algorithm. Furthermore, image sharpness (P < 0.001) and visibility of bronchi (P < 0.001) were improved when helical images were reconstructed with bone algorithm. We conclude that motion artifact severity, image sharpness and anatomic resolution for helical and conventional 0.6-s chest CT are similar when the images are reconstructed with an identical algorithm. Helical CT may be preferable because of the faster examination times and the ability to reconstruct overlapping images as an adjunct for problem solving. Also, with the scanner used, bone algorithm reconstruction can be employed to improve image sharpness and anatomic resolution.

Artifacts↗
PubMed1993

Osteoporosis induced in mice by overproduction of interleukin 4.

Osteoporosis is a common disease in which loss of bone mass results in skeletal fragility. The development of therapies for this disorder has been hampered by the lack of a convenient animal model. Here we describe a disorder in bone homeostasis in transgenic mice that inappropriately express the cytokine interleukin 4 (IL-4) under the direction of the lymphocyte-specific proximal promoter for the lck gene. Bone disease in lck-IL-4 mice appeared to result from markedly decreased bone formation by osteoblasts, features strikingly similar to those observed in cases of severe low-turnover human involutional osteoporosis. By 2 months of age, female and male lck-IL-4 mice invariably developed severe osteoporosis of both cortical and trabecular bone. Osteoporosis was observed in two independently derived founder animals, indicating that this phenotype was directly mediated by the IL-4 transgene.

Actins↗
PubMed1992

Tracheobronchiomegaly. The Mounier-Kuhn syndrome in a patient with the Kenny-Caffey syndrome.

A 36-year-old woman with features of both the Mounier-Kuhn syndrome and the Kenny-Caffey syndrome is described. To our knowledge, this is the first reported case of these syndromes occurring together. Three-dimensional computed tomographic reconstruction of the upper airway revealed marked dilatation of the trachea and main-stem bronchi and several large diverticulae of the trachea.

Abnormalities, Multiple↗
PubMed1991

John Caffey Award. Determination of functional residual capacity from digital radiographs of the normal neonatal chest: studies in a rabbit model.

Spirometric measurement of lung volume in an infant or uncooperative child is often a difficult and time-consuming procedure. Previous investigators have successfully estimated total lung capacity in adults and older children by using chest radiography. The purpose of this study was to assess the accuracy of functional residual capacity (FRC) determination from chest radiographs in an animal model of the normal neonatal chest. FRC was determined with the helium dilution (HeD) method in 13 anesthetized, intubated, and paralyzed rabbits (weight range, 1.5-3.9 kg). FRC was then estimated from digital frontal and lateral chest radiographs. The radiographic estimate was made by applying measurements taken from the radiographic film pair to a geometric model of the chest. The best-fit model assumed an elliptical cross section for the intrathoracic cavity, heart, paraspinal structures, and diaphragm in the axial plane. Linear regression on independently determined HeD and radiographically determined FRC yielded a slope of 1.02 (p less than .001), an intercept of -3.02 cm3 (p = .565), and a correlation coefficient of 0.96 (p less than .001). We conclude that radiographic FRC estimates closely approximate HeD estimates in a rabbit model of the normal neonatal chest.

Algorithms↗
PubMed1990

Meconium peritonitis and pleuritis: a clue to perforation of an incarcerated Bochdalek hernia in a neonate.

The case of a neonate presenting with respiratory distress who had an incarcerated Bochdalek hernia with perforation and meconium peritonitis and pleuritis is reported. The patient had an associated jejunal atresia in an incarcerated segment of intestine, which undoubtedly contributed to the obstruction, strangulation, and perforation in the incarcerated hernia. Gastrointestinal complications attributable to the hernia are most unusual in the neonate, and aggressive preoperative resuscitation and stabilization with the earliest possible surgical intervention are imperative features in management.

Female↗