Sedating drugs and neuromuscular blockade during mechanical ventilation.
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Biomedical subjects
Publications and source records attributed to K E Bove.
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To study the biology of bladder mucosal grafts we developed an animal model using New Zealand white male rabbits. A 25 x 9 mm. segment of bladder mucosa was harvested and tubularized over an 8F catheter using 7-zero polyglactin sutures. An equivalent portion of rabbit urethra was then excised and the graft was anastomosed to this defect in an end-to-end fashion. A urethral catheter was left in place to provide bladder drainage and to stent the anastomosis. Animals were sacrificed on postoperative days 1 to 90. India ink was injected into the aorta at sacrifice to visualize the microvasculature. All 59 specimens were stained with hematoxylin and eosin, and studied using light microscopy. Our results demonstrated vascular ingrowth at 72 hours. Between postoperative days 8 and 10 healthy viable epithelium first bridged the entire urethral defect. By postoperative day 12 the epithelial lining was complete. A poor outcome was observed in all animals whose stents were removed early. We conclude that the biology of bladder mucosal grafts is unique in that the graft initially undergoes partial degeneration followed by regeneration. Of concern are the results of those animals whose stents were removed early. In all such cases a poor outcome was observed.
Nephroblastomatosis is an abnormality of nephrogenesis characterized by incomplete maturation of primitive nephrogenic cells. The resultant individual lesions have variable histologic findings and distribution in the kidney. There is wide acceptance of nephroblastomatosis as a precursor lesion to Wilms tumor. Consequently, the role of imaging in assessing patients for neoplastic transformation of nephroblastomatosis should be emphasized. Computed tomography (CT) has been shown to be superior to ultrasound (US) in this regard. Nonetheless, the low cost and lack of radiation of US make it attractive for serial screening studies. Although magnetic resonance imaging shows promise in assessment of the kidney for nephrogenic rests, experience is limited in its use in evaluating patients for nephroblastomatosis. The authors present protocols for assessing patients identified as being at either high or low risk for the development of Wilms tumor due to nephroblastomatosis. These protocols take into account the advantages of CT and US. Scanning intervals are determined from epidemiologic data on the occurrence of Wilms tumor; the most intense screening occurs during the younger ages of life, when the likelihood of Wilms tumor development is highest.
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Down syndrome (DS) is not usually thought of in association with significant infantile liver disease. We present clinical and histopathologic data from 10 patients with DS who presented with severe liver disease at birth or within the first few weeks of life, and summarize the findings of eight previously reported cases. The liver disease was fatal in all but one case. Diffuse lobular fibrosis surrounding proliferating ductular elements and residual hepatocytes characterized the pathologic findings in the liver in all patients. A large number of megakaryocytes were present in the liver in nine of 12 patients. The phenotype of "perinatal hemochromatosis" was documented in eight of nine cases in which the presence of iron was investigated. Since only a fraction of the patients with this phenotype have DS, the patients we describe seem to represent a relatively well-defined subset of the perinatal hemochromatosis phenotype. The existence of such a subset suggests that the perinatal hemochromatosis phenotype does not represent a single etiopathogenetic disorder. The association between DS, megakaryocytic infiltrates in the liver, and fatal subacute/chronic liver disease gives rise to the speculation that fibrosis-promoting factors and/or metabolic abnormalities, such as those resulting from a gene dosage effect, may play a role in the genesis of the liver disease, perhaps due to a particular susceptibility of fetal liver.
We observed significant lesions of the carotid artery siphon in two young subjects with fatal stroke. Because stroke in children and adolescents is uncommon and poorly understood, we examined the internal carotid artery in the 'siphon' of the skull in 24 unselected, but nearly consecutive autopsies. The age range was 10 days to 38 years, with 11 males and 13 females, six blacks, and 18 whites. In no case was stroke the cause of death. Intimal lesions of two types were found in the carotid siphon of all cases. (1) The first was focal splitting and/or duplication of the internal elastic lamina with variable proliferation of smooth muscle. The resulting 'fibrous' plaques or cushions, when severe, were usually found at natural bends in the artery. The number and severity of this type of lesion increased with age, but there were no differences in severity or distribution when compared by sex, race, or mode of death. (2) The second was internal elastic calcification which was found in all cases older than 9. This was increasingly severe with age. Although the frequency of the vascular lesions was surprisingly high, the relationship of either type to dissecting aneurysm or other stroke lesion remains unclear.
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We present three instances in which cytoplasmic bodies in muscle fibers were associated with Werdnig-Hoffmann disease. The children presented within the first 6 weeks of life with clinical symptoms and laboratory studies indicative of infantile spinomuscular atrophy (ISMA). Initial muscle biopsies in each case revealed signs of muscle immaturity and cytoplasmic bodies but lacked features of denervation. Follow-up biopsies in 2 cases revealed changes typical of ISMA, but cytoplasmic bodies were no longer present. A follow-up biopsy was not available in the third child who also had a clinical course consistent with ISMA. We discuss the pathogenesis of cytoplasmic bodies and their significance in association with Werdnig-Hoffmann disease.
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Cystic lesions of the lung may be developmental or acquired. The authors describe two cases of developmental cystic lung lesions (mesenchymal cystic hamartomas) that harbored unsuspected mesenchymal sarcomas. Although in children the natural history of this lesion is incompletely characterized, there is a definite risk for malignant change. The initial appearance of these cystic lung lesions on the chest radiograph is generally benign. It is important to establish the nature of cystic lung lesions (developmental vs acquired) and recognize the potential risk of malignant change in mesenchymal cystic hamartoma. Cystic lung lesions of uncertain type should be resected.
One common vascular anomaly that causes airway obstruction in neonates and infants is innominate artery compression of the trachea. A great deal of controversy exists regarding the effect of this anatomic variant on symptom production. Several cases are presented that demonstrate the unique features of innominate artery compression of the trachea: 1. Innominate artery compression can occur in adolescent patients. 2. Flow volume loops are an effective method of documenting airway compromise in this condition. 3. Exercise intolerance should be considered as a relative indication for surgical intervention in these patients. 4. Reimplantation of the innominate artery is a viable surgical alternative in the treatment of affected patients. 5. Telescopic bronchoscopy during surgical correction of this condition will ensure that there has been successful correction of the tracheal compression. 6. Urgent repair is advocated in patients who experience periods of apnea.
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Left ventricular hypertrophy is an important diagnostic and prognostic finding in children with cardiovascular disease, but there are currently no well established criteria for its determination by M-mode echocardiography. Three hundred thirty-four subjects, aged 6 to 23 years, who were free of cardiovascular disease were studied. Left ventricular mass was calculated using echocardiographic measurements in a regression equation for left ventricular mass. Intraobserver (r = 0.96, p less than 0.01) and interobserver (r = 0.89, p less than 0.01) variability were low. To anatomically validate the echographic formula for left ventricular mass, left ventricular measurements made at autopsy were inserted into the formula. Mass was then calculated and compared with the actual mass. There was a strong correlation between the calculated and the measured left ventricular mass (r = 0.89, p less than 0.01). Left ventricular mass was not statistically related to race, but it was strongly associated with gender (p less than 0.001). It was strongly correlated with height (r = 0.82 for males, r = 0.71 for females) and body surface area (r = 0.83 for males, r = 0.74 for females). Echocardiographic criteria for left ventricular hypertrophy in children and adolescents, based on the 95th percentile, for left ventricular mass, left ventricular mass corrected for body surface area and left ventricular mass corrected for height are, respectively: 184.9 g, 103.0 g/m2 and 99.8 g/m for males and 130.2 g, 84.2 g/m2 and 81.0 g/m for females.(ABSTRACT TRUNCATED AT 250 WORDS)
Two infants with progressive spinomuscular atrophy presented with severe diaphragmatic dysfunction, increasing to 9 the number of cases with this clinically distinctive variant of Werdnig-Hoffmann disease. The anterior horn cell lesion was generalized but was exceptionally severe in the cervical spinal cord of our cases. Fiber size disproportion in serial thigh muscle samples indicated that qualitative neuronal dysfunction preceded appearance of typical denervation. Shoulder girdle muscle biopsy may be more appropriate in these infants, whose prognosis appears to be universally poor.
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Desquamative interstitial pneumonitis (DIP) is rare in children. Its cause is unknown. In general, it is of sporadic occurrence. We report 4 infants: 2 sibs in each of 2 separate families, who had DIP. All 4 infants died despite intensive care and immunosuppressive therapy. Our cases, plus one other similar kindred in the literature, confirm the occurrence of familial DIP in infancy. Further, our experience suggests that DIP in these familial cases carries a worse prognosis than that reported in sporadic cases.