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Biomedical subjects

M F DiMaio

Publications and source records attributed to M F DiMaio.

6 recordsLinked to original sources

Spinal deformity, pulmonary compromise, and quality of life in osteogenesis imperfecta.

STUDY DESIGN: A cross-sectional radiologic and clinical study of patients with osteogenesis imperfecta. OBJECTIVES: To determine whether pulmonary compromise is more closely correlated with scoliosis, kyphosis, or chest wall deformity in the population with osteogenesis imperfecta, and to assess the impact of spinal deformity, chest wall deformity, and pulmonary function on quality of life. SUMMARY OF BACKGROUND DATA: The incidence of scoliosis in osteogenesis imperfecta is between 39% and 80%. Up to 60% of patients with osteogenesis imperfecta have significant chest wall deformities. Pulmonary compromise is the leading cause of death in adults with osteogenesis imperfecta. METHODS: Fifteen patients with osteogenesis imperfecta between the ages of 20 and 45 were evaluated with sitting or standing anteroposterior and lateral radiographs of the entire spine, pulmonary function testing, and a validated health self-assessment questionnaire (Short Form-36). Radiographs were evaluated for thoracic scoliosis, thoracic kyphosis, and chest wall deformity. Correlation analysis was performed. RESULTS: Thoracic scoliosis was strongly correlated with decreased predicted vital capacity (r = -0.76). Significant diminution in vital capacity below 50% occurred at a curve magnitude of 60 degrees. Kyphosis and chest wall deformity were not predictive of decreased pulmonary function. Physical health (PCS) was closely correlated with predicted vital capacity (r = 0.65; P < 0.01) and with scoliosis (r = -0.52; P < 0.05). CONCLUSIONS: Thoracic scoliosis of more than 60 degrees has severe adverse effects on pulmonary function in those with osteogenesis imperfecta. This finding may partly explain the increased pulmonary morbidity noted in adult patients with osteogenesis imperfecta and scoliosis compared with that in the general population.

Adult↗

Effect of zidovudine on human fetal lung development.

Zidovudine (AZT) is currently used to treat human immunodeficiency virus (HIV)-positive women during pregnancy to prevent the prenatal transmission of HIV type 1 (HIV-1). However, AZT not only inhibits HIV replication but also affects the DNA polymerases of human cells; hence AZT is not recommended during the first trimester of pregnancy. The lung is a unique organ because it continues to grow and develop throughout fetal life. Using a human fetal lung organ culture system, we sought to determine the effect of AZT on morphogenesis and epithelial cytodifferentiation of developing alveoli. Lung tissues from three fetuses, 14-15 weeks gestational age, were grown in culture for 24 hours (day 0). AZT at a concentration of either 0.4, 4.0, 8.0, or 40.0 mumol/L was added on days 1, 5, and 10 of growth. The cultures were interrupted on days 6 and 15 and examined by light and electron microscopy for alveolar saccular development, interstitial thinning, and epithelial cell differentiation. On day 6 of growth the treated cultures demonstrated fewer alveolar saccules and a thicker, more cellular interstitium compared to the controls. After 15 days of growth the cultures treated with 0.4 mumol/L of AZT appeared structurally similar to the controls. The cultures treated with AZT concentrations of 4.0 to 40.0 mumol/L appeared unchanged from day 6, implying arrested maturation of the culture. However, epithelial cell differentiation was unaffected. We conclude that AZT at concentrations of 4.0 mumol/L and greater affects the structural development of the human fetal lung in vitro.(ABSTRACT TRUNCATED AT 250 WORDS)

Cell Differentiation↗

Brush cells in the human fetal trachea.

We performed a morphologic examination of human fetal lung tissue, using scanning and transmission electron microscopy, in order to establish the presence of brush cells in extrapulmonary and intrapulmonary airways, and developing acinar epithelium. Brush cells, characterized by a border of regular straight microvilli containing a filamentous core, were observed within the tracheal epithelium of a 19-20 week gestational age fetus. These cells constituted 0.5% of the total epithelial cell population. Brush cells were not seen within the bronchial, bronchiolar or developing acinar epithelium. Our study shows that brush cells occur infrequently but normally in the developing tracheal epithelium of the second trimester fetus.

Fetus↗

Alveolar brush cells in an infant with desquamative interstitial pneumonitis.

A full-term infant developed bilateral pneumothoraces and respiratory distress shortly after birth, despite initially good Apgar scores. Persistent tachypnea, hypoxemia, and a chest X-ray remarkable for diffuse alveolar and interstitial infiltrates prompted a lung biopsy at 4 months of age. The biopsy revealed desquamative interstitial pneumonitis with the unique demonstration by electron microscopy of numerous alveolar brush cells. Respiratory brush cells occur normally in the trachea and bronchi of humans and mammals. Although identical cells have been noted in the alveoli of rats, they have never been reported in the alveoli of humans. We present the first electron microscopical demonstration of the alveolar brush cell in humans.

Basement Membrane↗