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Biomedical subjects

K E Bove

Publications and source records attributed to K E Bove.

At least 19 recordsLinked to original sources

Imaging of nephroblastomatosis: an overview.

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.

Clinical Protocols

Diffuse metanephric adenoma after in utero aspirin intoxication. A unique case of progressive renal failure.

Diffuse persistent glomerular immaturity and focal proximal tubular ectasia were seen in bilateral open renal biopsy specimens for an infant with fluid and salt depletion and slowly progressive renal failure. Subsequently, diffuse tubulopapillary renal adenoma subtotally replaced each kidney, thereby, necessitating renal transplantation. Origin of diffuse metanephric adenoma from persistent primitive epithelium of the proximal nephron is postulated and partly substantiated. We propose that this case of persistent proximal nephronic epithelial immaturity and diffuse metanephric adenoma is a variant of nephroblastomatosis and that in this case, a first trimester suicide attempt with aspirin may have initiated the maturation defect that preceded neoplastic transformation.

Aspirin

Multifocal nephroblastic neoplasia.

Among 23 patients with multifocal metanephric neoplasia were 14 patients with 43 grossly visible subcapsular tumorlets, 0.3-3.5 cm in diameter, that were apparently derived from nodular renal blastema. Tumorlets are defined as circumscribed lesions that are visible on the exterior surface of the kidney, produce either a raised nodule or, in a few instances, a shallow depression, and are independent of the main tumor(s). Most tumorlets displayed homogeneous epithelial maturation. A substantial minority of tumorlets retained a blastemic character and a few were histologically malignant. Tumorlets tended to be multiple and bilateral but were absent in 9 patients with multifocal disease, including 4 of 10 with bilateral tumors. About half the lesions that achieved tumorlet status, including those that gave rise to clinical tumors, underwent malignant transformation. Contralateral tumorlets should not be mistaken for metastasis and do not adversely affect prognosis. The incidence of neoplasia among young, close relatives of patients with multifocal disease appeared to be excessive, but no clear excess of major anomalies, hemihypertrophy, or minor genitourinary tract anomalies was revealed by a retrospective survey of hospital charts.

Child

Acute respiratory illness in children with acute lymphoblastic leukemia.

Ten of 70 children (14%) with acute lymphoblastic leukemia developed severe interstitial pneumonitis within three weeks after induction of central nervous system prophylactic therapy. The clinical picture was characterized by fever, cough, progressive dyspnea, and hypoxemia with complete resolution in one to three weeks, except in one patient who died during the acute illness from respiratory failure. P. carinii organisms were found in the lung tissue of only one patient. The etiology of the pneumonitis in the other nine children was probably viral, acquired or activated during a period of lymphopenia and immunosuppression. The morbidity and potential mortality from the pneumonitis warrants early recognition by open lung biopsy and intensive supportive therapy.

Central Nervous System Diseases

The nephroblastomatosis complex and its relationship to Wilms' tumor: a clinicopathologic treatise.

1. At least one third of all children with Wilms' tumors have evidence of aberrant metanephric differentiation, hamartomas, adenomas and nodules of blastema or Wilms' tumorlets in the subcapsular renal cortex. Only the presence of nodular renal blastema was found to correlate with younger age, suggesting that the other tumorlike lesions may be derivatives. 2. In our experience, all patients with bilateral and sequential bilateral Wilms' tumors have exhibited these characteristics in the "uninvolved" renal tissue removed at nephrectomy. 3. Pancortical (infantile), diffuse superficial (late infantile) and multifocal (juvenile) variants of nephroblastomatosis are defined. In the latter 2 forms of nephroblastomatosis the prognosis after modern therapy is at least as good as it is in patients with unilateral, unicentric Wilms' tumor arising in a histologically normal kidney. 4. The morphologic evidence presented strongly indicates that Wilms' tumor in patients with nephroblastomatosis develops either from nodular blastema or metanephric hamartomas. It is hypothetically possible that all Wilms' tumors develop from these precursors. The latent period between cessation of nephrogenesis and clinical presentation of Wilms' tumor is interpreted as favoring the hypothesis that carcinogenesis per se is the result of a postnatal event but that the substrate for tumor development, aberrant metanephric proliferation, has its inception during gestation.

Adenoma

The association of fixed and dynamic left ventricular outflow obstruction.

Twelve patients were investigated echocardiographically and angiographically and were shown to have severe fixed obstruction to the left ventricular outflow tract. Eight had valvular stenosis, and four had discrete subvalvular membranes. Two of the patients had additional dynamic obstruction of the left ventricular outflow tract. This was recognized preoperatively by echocardiography because of abnormal systolic motion of the mitral leaflet. At the time of definitive surgery for relief of the fixed obstruction, the additional dynamic obstruction was identified and treated, since persistent residual obstruction may lead to death in the immediate postoperative period or to long-term symptoms. The dynamic left ventricular outflow obstruction is probably a result of the hypertrophy produced by the fixed obstruction.

Adolescent