Also found in: Dictionary, Thesaurus, Acronyms, Encyclopedia, Wikipedia.
an abnormality of development; in pathology, alteration in size, shape, and organization of adult cells. See also dysgenesis. adj., adj dysplas´tic.
bronchopulmonary dysplasia chronic lung disease of premature infants with hyaline membrane disease who have needed high concentrations of oxygen and assisted ventilation. Factors related to its development include alveolar damage due to hyaline membrane disease, oxygen toxicity, positive pressure ventilation, and endotracheal intubation. Treatment includes supportive measures and oxygen therapy. Recovery and normal pulmonary function usually occur by the age of 6 months to 1 year; however, some infants may exhibit limited tolerance to exercise.
craniometaphyseal dysplasia metaphyseal dysplasia associated with overgrowth of the head bones, leonine facies, and increased distance between the eyes.
cretinoid dysplasia a developmental abnormality characteristic of cretinism, consisting of retarded ossification and smallness of the internal and reproductive organs.
cystic renal dysplasia renal dysplasia in which there are cysts.
developmental dysplasia of the hip (DDH) instability of the hip joint leading to dislocation in the neonatal period. Although it may be associated with various neuromuscular disorders, such as myelodysplasia, or occur in utero, it most commonly occurs in neurologically normal infants and is multifactorial in origin. Usually there is laxity of the hip ligaments. Most affected infants are first-born children and 30 to 50 per cent present in the breech position. About 90 per cent of those affected are girls. The condition was formerly called congenital dislocation of the hip, but because the dislocation is not normally present at birth but develops later, the term developmental dysplasia of the hip is preferred.
ectodermal dysplasia any of a group of hereditary disorders involving absence or deficiency of tissues and structures derived from the embryonic ectoderm, such as teeth, hair, nails, and certain glands.
fibromuscular dysplasia dysplasia with fibrosis of the muscular layer of an artery wall, with collagen deposition and hyperplasia of smooth muscle, causing stenosis and hypertension. It most commonly occurs in the renal arteries and is a major cause of renovascular hypertension.
fibrous dysplasia of bone thinning of the cortex of bone and replacement of bone marrow by gritty fibrous tissue containing bony spicules, causing pain, disability, and gradually increasing deformity; it may affect a single bone (monostotic fibrous dysplasia) or several or many bones (polyostotic fibrous dysplasia). When associated with melanotic pigmentation of the skin and endocrine disorders, it is known as albright's syndrome.
metaphyseal dysplasia a disturbance in enchondral bone growth, failure of modeling causing the ends of the shafts to remain larger than normal in circumference.
oculodentodigital dysplasia a rare hereditary condition, characterized by bilateral microphthalmos, abnormally small nose with anteverted nostrils, hypotrichosis, dental anomalies, camptodactyly, syndactyly, and missing phalanges of the toes.
renal dysplasia a congenital disorder of the kidney, with persistence of cartilage, undifferentiated mensenchyme, and immature collecting tubules, as well as with abnormal lobar organization and nearly always cysts; it may be unilateral or bilateral and total or subtotal. Total bilateral dysplasia is rapidly fatal in the neonatal period, while milder disease may be asymptomatic.
retinal dysplasia a general term for a congenital defect resulting from the abnormal growth and differentiation of a retina that fails to develop into functioning tissue.
chronic pulmonary insufficiency seen primarily in infants born prematurely; defined clinically as a persistent supplemental oxygen requirement at 1 month of age and typically seen in infants who required positive pressure ventilation.
bronchopulmonary dysplasia(brŏng′kō-po͝ol′mə-nĕr′ē, -pŭl′-)
Chronic pulmonary insufficiency resulting from long-term artificial pulmonary ventilation, occurring chiefly in premature infants.
bronchopulmonary dysplasia (BPD)
a chronic respiratory disorder characterized by scarring of lung tissue, thickened pulmonary arterial walls, and mismatch between lung ventilation and perfusion. It often occurs in infants who have been dependent on long-term artificial ventilation.
adult respiratory distress syndromeA condition due to a diffuse infiltrative process in the lungs, which affects ±150,000/year (US), and is characterised by acute pulmonary oedema and respiratory failure, poor oxygenation, increased functional residual capacity and decreased compliance; ARDS may accompany various medical and surgical conditions, and may be associated with interstitial pneumonitis—usual, desquamative and lymphoid types.
Gram-negative sepsis, pneumonia, shock, gastric acid aspiration, trauma, drug overdose, toxic gas (chlorine, NO2, smoke) exposure, severe metabolic derangement, pancreatitis.
A 6–24-hour latency period is followed by hypoxia, decreased aeration, dyspnoea, severe SOB and “stiff” lungs—i.e., decreased pulmonary compliance.
Extensive, diffuse bilateral fluffy infiltrates.
Nitric oxide (NO), 18 ppm, may reduce mean pulmonary artery pressure; 37 to 30 mm Hg, may reduce intrapulmonary shunting (36% to 31%), increase ratio of partial pressure of arterial O2 to inspired O2 (PaO2/FiO2), an index of arterial oxygenation efficiency (±152 to ±199); other management strategies include PEEP.
The outcome of ARDS is a function of underlying cause.
± 60%, the cause of death has shifted from hypoxia to multiple organ failure.
bronchopulmonary dysplasiaA chronic lung disease affecting ± 7000 premature infants/yr–US, treated for respiratory distress syndrome with supplemental O2 and mechanical ventilation for ≥ 1 wk, with Sx of persistent respiratory distress, who have rounded radiolucencies on a plain chest films Lab ↑↑ Leukotrienes C4, D4, E4 in lavage fluid Prognosis 40% die of pulmonary dysfunction in later life, by airway obstruction, airway hyperreactivity and hyperinflation. See Hyaline membrane disease.
bron·cho·pul·mo·nar·y dys·pla·si·a(brong'kō-pul'mŏ-nār-ē dis-plā'zē-ă)
Chronic pulmonary insufficiency arising from long-term artificial pulmonary ventilation; seen more frequently in premature than in full-term infants.