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Biomedical subjects

D R Kelly

Publications and source records attributed to D R Kelly.

At least 73 records · Page 4Linked to original sources
PubMed1986

Coarctation of the abdominal aorta in a child: morphometric analysis of the arterial lesion.

Coarctation of the abdominal aorta with associated narrowing of the origin of major visceral arteries is a rare cause of life-threatening hypertension. We describe a 9-year-old male with hypertension and recent onset of headache and dizziness who was subsequently found to have a suprarenal coarctation with involvement of the proximal renal arteries. The patient died following a left cerebrovascular accident associated with recent thrombosis in the left middle cerebral and internal carotid arteries. Morphometric analysis of the aortic coarctation revealed that a reduction in outside diameter of the vessel was complicated by marked intimal thickening which caused further stenosis of the lumen. Medial thickness was constant, and elastic fibers of the media were intact at the level of coarctation.

Aorta, Abdominal↗
PubMed1985

Second generation alpha-enones from a pyranosidic alpha-enone.

The Diels-Alder product from the reaction of methyl 2,3,6-trideoxy-alpha-D-glycero-hex-2-enopyranosid-4-ulose (1b) with trans-1-methoxy-3-tert-butyl-dimethylsilyloxy-1,3-butadiene is 3b (93%). Reaction of 3b wih sodium borohydride causes reduction of the C-4 carbonyl group only, but, with lithium aluminum hydride, further reactions occur which can be rationalized by fragmentation brought about by hydride cleavage on the silicon-oxygen bond, with simultaneous ejection of the beta-methoxyl group complexed to a trivalent aluminum species. The enone resulting from this fragmentation also reacts further with lithium aluminum hydride, and several products result. The behavior of postulated intermediates, which have been prepared separately and subjected to the reaction conditions, supports the proposed reaction mechanisms. The "second generation" enone (methyl 2,3,6-trideoxy-alpha-D-talopyranosido)-[3,2-d]-2-cyclohexenone+ ++ (10a), arising from the first generation precursors 1b, has been prepare by two routes.

Chemical Phenomena↗
PubMed1985

Severe congenital leukopenia (reticular dysgenesis). Immunologic and morphologic characterizations of leukocytes.

We report fatal reticular dysgenesis in a premature infant presenting with severely decreased blood and bone marrow granulocytes and lymphocytes, an absent thymic shadow by x-ray film, and generalized lymphoid hypoplasia. Immunologic and electron microscopic evaluation of his white blood cells demonstrated that, despite extremely low cell numbers, cells from all stages of both granulocytic and lymphocytic development were present. Immature bone marrow cells of both myeloid and lymphoid lineages were found in much greater proportions than were mature cells; pre-B cells outnumbered B cells by more than tenfold. Megakaryocytes and erythroid cells appeared to be present in normal numbers, and tritiated-thymidine incorporation by bone marrow nucleated cells was also normal, although it may have largely occurred in erythroblasts. These data suggest that the primary defect in reticular dysgenesis is not failure in initiation of stem cell differentiation along lymphoid and myelomonocytic lines but rather an, as yet, undefined abnormality that interferes with normal growth and maturation of immune cells committed to these differentiation pathways.

B-Lymphocytes↗
PubMed1985

Characterization of plasma lipids and lipoproteins in cholesteryl ester storage disease.

Cholesteryl ester storage disease, caused by the loss of lysosomal acid ester hydrolase (EC 3.1.1.13), has been previously associated with hyperlipidemia and premature atherosclerosis. We identified a 23-month-old female with cholesteryl ester storage disease and characterized the plasma lipids and lipoproteins in the proband and her family. These studies illustrate several important points about this disease. First, a high index of suspicion is required to diagnose this disease since the major physical manifestation of the disorder, mild hepatomegaly, is subtle. Second, the Type II hyperlipoproteinemia in the proband is paralleled by a reduction in the concentration of high density lipoproteins. Third, analysis of the plasma lipids and lipoproteins in family members revealed both Type II and Type IV hyperlipoproteinemia with an inheritance pattern similar to that of familial combined hyperlipoproteinemia. Fourth, the parents and brother of this patient had 50% normal fibroblast acid ester hydrolase activity. These results raise the possibility that deficiency of the lysosomal acid ester hydrolase may be linked to familial combined hyperlipoproteinemia and that this enzyme deficiency may be more common than previously appreciated.

Biopsy↗
PubMed1985

Case 2. Cystic cellular mesoblastic nephroma.

Mesoblastic nephroma is a benign mesenchymal tumor of the kidney generally diagnosed during the first 3 months of life. A case of the cellular variant of mesoblastic nephroma associated with prominent cystic change and renal vein invasion arising in an 8-month-old female is described. This unusual case expands the morphologic spectrum of mesoblastic nephroma.

Cell Membrane↗
PubMed1984

Childhood liposarcoma. Report of a case and review of the literature.

A case of liposarcoma presenting in an adolescent black male is described. Features unique to this case include a mediastinal primary site (the fourth to be documented in a pediatric patient), a demonstrable response to radiotherapy and chemotherapy allowing complete surgical excision of an initially inoperable tumor, and the apparent histologic maturation of the tumor following multimodal therapy. A review of previously published accounts of pediatric liposarcoma revealed the following: (1) peak incidences during infancy and in early adolescence; (2) the extremities to be the most common site of origin (51%); (3) a predominance of myxoid histology (76%); and (4) a lower overall recurrence rate when compared with adult cases (37% versus 72%, respectively). The influence of histology, location of the tumor, and completeness of surgical excision on the prognosis of adult liposarcoma was confirmed in this limited pediatric experience. While the use of radiotherapy and chemotherapy cannot be advocated in all instances of liposarcoma, our experience and that from other reports would support the incorporation of these modalities of treatment in selected patients where the tumor is surgically inaccessible due to size.

Adolescent↗
PubMed1983

Infantile polycystic kidney disease: an imaging dilemma.

Infantile and adult type polycystic kidney diseases are 2 disparate genetic disorders and generally are easily distinguishable on the basis of clinical, pathologic, and radiologic findings. We present 3 children with infantile polycystic kidney disease, ages 9 months to 6 years, in whom the excretory urogram and/or renal ultrasound or gross anatomical appearance of the kidneys resembled adult polycystic kidney disease. The findings from these 3 patients emphasize the importance of renal and liver biopsies in the diagnosis of cystic kidney disease in young children.

Child↗
PubMed1983

Bacteremia following cleft palate repair--a prospective study.

A prospective study of temperature patterns and blood cultures following cleft palate repair suggests that bacteremia in these cases is common. Twenty-seven years without serious complications supports the authors' view that antibiotic therapy is not indicated.

Child↗
PubMed1983

Prognostic factors affecting the success of duckbill vocal restoration.

This study analyzed a group of 20 patients undergoing duckbill vocal restoration at Ohio State University to determine if certain variables could predict success. Of the 20 patients, 19 developed successful speech. The 10 variables studied were age, alcohol use, living environment, stoma size, hearing acuity, educational level, work status, eye-hand coordination, concomitant medical problems, and current mode of communication. A multiple correlation analysis of this data was undertaken and alcohol abuse and stoma size were found to have the highest correlation with developing successful duckbill speech. A formula based on these two variables has been derived that will predict the patient's success. It is the conclusion of this study that since patients are not uniformly successful in developing duckbill speech such a formula is of value in predicting success and should help the clinician identify patients who may have difficulties.

Adult↗
PubMed1981

Predictive ability of Lukes-Collins classification for immunologic phenotypes of childhood non-Hodgkin lymphoma: an institutional series and literature review.

Tissues from 22 children with non-Hodgkin lymphoma (NHL) were studied pathologically and immunologically. Most children were noted to have marked (B- or T-cell) neoplasms and the Lukes-Collins classification was predictive of immunologic phenotype in cases where markers were present. Our series and a review of the literature demonstrates that most abdominal NHL are B-cell in origin and are often small noncleaved follicular center cell lymphoma (Burkitt type). Most mediastinal primary lesions are T-cell in origin and of convoluted cell morphology. A few neoplasms (often peripheral nodal) lack the characteristic surface immunoglobulin or erythrocyte rosetting properties of B- or T-cell lesions, respectively. Frequently marrow and central nervous system involvement are observed in T-cell lymphomas and are not in frequent in B cell neoplasms. Shared immunologic and clinical features between the B- or T-cell lymphomas and their leukemic counterparts support the concept that they often differ only in the stage of disease progression.

Adolescent↗
PubMed1981

Rhabdoid tumor of the kidney: complete remission induced by cis-platinum and adriamycin.

A 4-month-old male infant with Stage I rhabdoid tumor of the kidney at presentation subsequently developed pulmonary metastatic disease shortly after diagnosis and initiation of Vincristine and Actinomycin D chemotherapy. The patient was then treated with cis-platinum and Adriamycin. Within 28 days he achieved a complete remission which was maintained for 5 months. Subsequent recurrent pulmonary lesions failed to regress with radiotherapy and high-dose cyclophosphamide when used as single sequential agents. Further clinical trials with cis-platinum and Adriamycin seem warranted since prognosis with this tumor is poor and successful chemotherapy after metastatic dissemination has not been previously reported.

Cisplatin↗
PubMed1981

Megalocytic interstitial nephritis, xanthogranulomatous pyelonephritis, and malakoplakia. An ultrastructural comparison.

Light-microscopic and ultrastructural examinations of megalocytic interstitial nephritis, xanthogranulomatous pyelonephritis, and malakoplakia of the kidney were compared. The cases of megalocytic interstitial nephritis and xanthogranulomatous pyelonephritis represent the first reported electron-microscopic studies on human kidney of these diseases. The study confirmed the presence of a polymorphous cellular infiltrate with predominate histiocytes containing crystalloid material in the case of megalocytic interstitial nephritis; a polymorphous cellular infiltrate with histiocytes predominating in the case of xanthogranulomatous pyelonephritis; and macrophages containing Michaelis-Gutmann bodies in the case of malakoplakia. The characteristic sites of involvement within the kidney by each of the three lesions are discussed. It is believed that the lesions are distinct entities, but related to one another, and represent varied and unusual host responses to inflammation.

Female↗
PubMed1979

Unusual manifestations of non-Hodgkin's lymphoma.

Two patients with biopsy proven lymphomatous infiltration of uncommon sites are presented. One had histiocytic lymphoma, involving the true vocal cord with a squamous cell carcinoma on the opposite vocal cord. The other had diffuse well-differentiated lymphocytic lymphoma, presenting with nodal and periorbital disease. After local radiation therapy and while clinically free of other lesions, he developed hemotochezia due to several polypoid lesions throughout the entire colon and prostatic infiltration with symptoms of lower urinary tract obstruction. Both problems were solved after specific treatment.

Carcinoma, Squamous Cell↗
PubMed1975

Benign lymphoepithelial lesions of the salivary glands.

The benign lymphoepithelial lesion of the salivary glands has been labeled with multiple, confusing terms. We recommend the abandonment of the vague term of Mikulicz disease. The histopathologic findings of lymphoid infiltration, intraductal proliferation, epimyoepithelial islands, and acinar atrophy are presented. The relationship between this lesion and autoimmune diseases, including Sjögren syndrome, is noted. Difficulty of histopathologic differentiation between this lesion and malignant lymphoma can occur. The association of the benign lymphoepithelial lesion with the simultaneous presence or future development of lymphoma is discussed.

Adult↗