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Biomedical subjects

D R Kelly

Publications and source records attributed to D R Kelly.

At least 19 recordsLinked to original sources

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.

Abdominal Neoplasms

An assessment of vocational training.

A retrospective analysis of the opinions of vocationally trained doctors was obtained from a postal questionnaire. Ninety-eight doctors who had trained in the West of Scotland before the introduction of new criteria in 1985 for the appointment and reappointment of training practices were compared with 107 doctors who had trained following the introduction of the criteria, looking at their rating of training, the frequency of tutorials, value of teaching and research encouragement. There was a significant improvement in the rating of training, frequency of tutorials, research encouragement and enjoyment of training in the group who trained after the new criteria were adopted. The study demonstrates the benefits of vocational training for general practice when criteria are set and followed. This training model may be applicable in other branches of medicine.

Education, Medical, Undergraduate

Peripheral primitive neuroectodermal tumors. A flow cytometric analysis with immunohistochemical and ultrastructural observations.

Flow cytometry of classical neuroblastoma has provided provocative evidence that cell cycle and ploidy analysis generate prognostically useful information. To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. An acceptable histogram was obtained in 16 cases. Of these, nine neoplasms were diploid and seven contained aneuploid DNA. Among patients with diploid lesions, four were free of disease, whereas three had persistent or recurrent disease, and two had died of tumor. Among patients with aneuploid neoplasms, four were free of disease, one had recurrence, and two had died. There was no apparent correlation between immunophenotype and proliferative activity with the clinical outcome. Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system.

Aneuploidy

Twenty years of vocational training in the west of Scotland.

OBJECTIVE: To assess the career paths of doctors who completed vocational training in the west of Scotland between 1968 and 1987 and their views on the hospital component of their training. DESIGN: Retrospective analysis of the experience and opinions of vocationally trained doctors obtained from a postal questionnaire. SETTING: West of Scotland. SUBJECTS: 1255 Doctors identified from Glasgow University records who had been vocationally trained in the west of Scotland between 1968 and 1987. MAIN OUTCOME MEASURES: Personal details; employment; jobs held currently; additional professional commitments; importance of hospital posts held in various specialties to respondents currently in general practice; and retrospective choice of hospital posts, based on subsequent experience. RESULTS: 619 Responses were received, 543 initially and 76 after a letter of reminder, from a possible total of 974 (excluding 153 questionnaires returned by the post office and 128 returned because of misunderstanding between the name and address); the overall response rate was therefore 64%. 607/619 (98%) Respondents were employed at the time of the study, of whom 517/607 (85%) were in general practice. A third (202/609) had been unemployed at some point, significantly more of them women (122/243, 50% v 80/376, 21%; chi 2 = 54.8, p less than 0.001). 510/563 (91%) Respondents held one postgraduate qualification or more, and 284/612 (46%) had additional professional commitments. The hospital posts most commonly held were in obstetrics and gynaecology, psychiatry, paediatrics, and medicine. Medicine, obstetrics, paediatrics, and dermatology were considered to be the most relevant hospital specialties by those who had experience of them and were now in general practice. Although ophthalmology and ear, nose, and throat were not rated highly by these doctors, other respondents wished that they had held posts in these specialties (32 and 40 respectively). During the hospital training 354/475 (75%) respondents thought that they were looked on as a junior hospital doctor and not as a trainee for general practice. CONCLUSIONS: Most of those who had entered vocational training were in employment, and most were in general practice. According to them, the most beneficial hospital posts for vocational training are medicine, obstetrics, paediatrics, and dermatology. Trainees should be encouraged to attend clinics in gynaecology, ear, nose, and throat, and ophthalmology.

Adult

Primary rhabdomyosarcoma of the iris.

We describe herein a 4-year-old girl with primary rhabdomyosarcoma of the right iris. Malignant cells were aspirated from the aqueous humor, and the eye was enucleated 7 months after the tumor appeared. To our knowledge, only two cases of primary rhabdomyosarcoma of the iris have been reported. Both of these patients received radiation therapy prior to enucleation.

Anterior Chamber

Immunohistochemical and ultrastructural spectrum of hepatic sarcomas of childhood: evidence for a common histogenesis.

Hepatic sarcomas of childhood, which appear heterogeneous by standard morphologic criteria, pose challenging diagnostic and nosologic problems to the pediatric surgical pathologist. To identify features of these tumors that might help to clarify their origin and histologic classification, we studied 13 undifferentiated (embryonal) sarcomas of the liver (UESL) and two intrinsic hepatic rhabdomyosarcomas (RMS) using immunohistochemical and electron microscopic techniques. Immunohistochemical staining was performed on 14 tumors with use of commercially available antibodies against a variety of markers, as well as peanut agglutinin lectin; electron microscopy was performed on five UESL and both RMS. Desmin was expressed by 6/12 UESL and 2/2 RMS, muscle-specific actin by 5/12 UESL and 2/2 RMS, neuron-specific enolase by 1/12 UESL and 1/2 RMS, alpha-1-antitrypsin by 8/12 UESL and 1/2 RMS, and alpha-1-antichymotrypsin by 10/12 UESL and 1/2 RMS. Cytokeratin expression was observed in only four UESL. The overlap of immunohistochemical staining patterns and ultrastructural features shown by these obstensibly different tumors suggests a common histogenesis, perhaps from a multipotential mesenchymal stem cell.

Actins

Twenty years of vocational training in the west of Scotland: the practice component.

A retrospective analysis of the experience and opinions of doctors receiving vocational training in general practice was obtained by postal questionnaire. Questionnaires were received by 974 doctors who had been vocationally trained in the west of Scotland between 1968 and 1987. The response rate was 64%. It was found that 94% of the respondents had enjoyed their trainee period, 82% had been given a choice of training practice and 86% had spent 12 months in a training practice. Only 81 respondents had trained in two practices. The most common method of monitoring the trainee's consultation was the trainer sitting in on the consultation; half of the doctors had experience of this (51%). For the majority regular tutorials were commonplace, but for 41% of respondents this was not so. However, those training after 1979 were significantly less likely to have never had tutorials than those training earlier. Nearly half of the doctors (49%) felt that certain aspects had been poorly covered or omitted from their training, notably practice management and finance. Again, this was significantly less likely among those training after 1979. When asked to give a rating of the training they had received 21% of the respondents rated it as excellent, 37% as very good, 30% as fairly good and 12% as poor/fairly poor or very poor. Notably, significantly fewer respondents training after 1979 rated their training as poor/fairly poor or very poor. Very few respondents had participated in a practice exchange but virtually all of those who had felt it had been beneficial.(ABSTRACT TRUNCATED AT 250 WORDS)

Education, Medical, Continuing

Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome).

An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.

Chickenpox

A cystic fibrosis pancreatic adenocarcinoma cell line.

We established a pancreatic adenocarcinoma cell line (CFPAC-1) from a patient with cystic fibrosis (CF) and assessed some of its properties. The cells show epithelial morphology and express cytokeratin and oncofetal antigens characteristic of pancreatic duct cells. Basal and stimulated levels of cAMP and cAMP-dependent protein kinase and the biophysical properties of single Cl- channels in CFPAC-1 are similar to those of airway and sweat gland primary cultures and Cl(-)-secreting epithelial cell lines. Anion transport and single Cl- channel activity was stimulated by Ca2+ ionophores but not by forskolin, cAMP analogs, or phosphodiesterase inhibitors. The cells express the CF gene and manifest the most common CF mutation, deletion of three nucleotides resulting in a phenylalanine-508 deletion. These properties have been stable through greater than 80 passages (24 months), suggesting that CFPAC-1 can serve as a continuous cell line that displays the CF defect.

Adenocarcinoma

Recent advances in the generation of chiral intermediates using enzymes.

Different types of enzyme-catalyzed processes are reviewed, with particular regard to those procedures leading to the generation of chiral compounds of high optical purity. The main body of the review deals with hydrolyses and esterification as well as the reduction and oxidation of organic substrates. Other biotransformations of current and/or future importance in the synthesis of homochiral fine chemicals (such as the formation of carbon-carbon bonds using aldolases) are also discussed in some detail. Attention is drawn to current trends in the area and, to this end, a majority of the references are taken from journals published during the period April 1987 to September 1988.

Alcohols

Difficult decisions in endoscopic sinus surgery.

Sinus disease, like many other problems in otolaryngology, may run the gamut of being either easily correctable with a clear-cut treatment or completely resistant to any form of treatment. Fortunately, many patients have problems somewhere between these two extremes. Clearly there is no one correct approach for treatment of many problems. Two, three, or even four approaches may be equally effective in some cases. In other cases, one approach may be successful in the hands of one surgeon whereas another approach is more appropriate for a different surgeon. This article presents nine cases that do not have clear-cut solutions. In some, the consultants clearly disagreed, whereas in others there was some uniformity in treatment plans. This flexibility in surgery in general and specifically with a technique that is relatively new is what makes it so exciting and so promising for continued success in treating sinus disease.

Adult

Lesions of the breast in children exclusive of typical fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases.

This large series of cases demonstrates that the breast of the child or adolescent is the potential site for a number of interesting lesions exclusive of fibroadenoma and gynecomastia. Adenoma of the nipple, juvenile papillomatosis, and juvenile or cellular fibroadenoma should be correctly diagnosed to insure conservative surgical management and appropriate clinical follow-up. The results of the Juvenile Papillomatosis Registry suggest that this lesion is a morphological marker of potentially more serious breast disease in the future. Recognition of a cellular fibroepithelial neoplasm, especially in young black females, as a cellular fibroadenoma will hopefully eliminate the need for the diagnosis of "benign" cytosarcoma phyllodes. On occasion, the equivocal case may be labeled a "phyllodes tumor fo indeterminate biologic behavior." Those neoplasms with stromal atypia and mitotic activity, overgrowth of stroma with epithelial dissociation, and sarcomatous elements are appropriately designated as cytosarcoma phyllodes. In contrast to vascular tumors of the breast in adults, the majority of vascular lesions in the child's breast are bening and represent either a capillary or a histiocytoid hemangioma. Our experience and reports in the literature support the conclusion that most malignancies of the breast in children are metastatic. Rhabdomyosarcoma was the most common example in our study, but secondary involvement by acute leukemia or malignant lymphoma also occurs.

Adenofibroma

Angiolipoma of the parotid: case report and review.

An angiolipoma is a benign adipose tissue tumor that can occur as either a circumscribed or an infiltrating lesion and represents up to 17% of all lipomas. Most angiolipomas occur in teenagers and young adults, and are rarely described in younger children. Angiolipoma generally arises on the extremities or trunk, and it is unusual when found in the head and neck. An angiolipoma of the parotid region was suspected in an infant girl after contrast-enhanced computerized tomographic scan, and was confirmed when the mass was surgically excised. The clinical characteristics, radiological appearance, and histopathology of angiolipoma are discussed.

Female

A morphologic study of childhood lymphoma of the lymphoblastic type. The pediatric Oncology Group experience.

For this study 227 non-Hodgkin's lymphomas, registered through the Pediatric Oncology Group clinical studies between 1976 and 1982, were morphologically subclassified into major histologic types and subtypes, and their histopathologic and clinical features were compared. These lymphomas were distributed primarily into only three of the recognized major histologic types: lymphoblastic (LB), 106 (47%); undifferentiated (DU), 49 (21%); and diffuse histiocytic (DH), 72 (32%). These patient groups were found to differ in several ways: the LB lymphomas contained most of the patients under two years of age; the LB lymphomas tended to present in higher clinical stages; the LB lymphomas tended to involve lymph node groups and the bone marrow more often than did the DU and DH lymphomas; and the DU lymphomas had a greater tendency for gastrointestinal tract and other major organ system involvement. The complete remission rate of 96%, for the LB lymphomas was better than for either the DU or the DH lymphomas. The disease-free survival of the LB lymphomas was significantly better than the DU group, but not the DH group. The LB were histologically divisible into three subtypes: convoluted (C), nonconvoluted (NC), and large cell variant (LCV). The C and NC subtypes preferentially involved the mediastinum and peripheral lymph nodes initially, while the LCV tended to involve the abdomen. However, none of the subtypes differed in clinical stage. The complete remission, and the disease-free survival rates between these subtypes were not statistically different.

Adolescent

A morphologic study of childhood lymphoma of the undifferentiated type. The Pediatric Oncology Group experience.

A retrospective analysis of 49 cases of undifferentiated non-Hodgkin's lymphoma, registered through the Pediatric Oncology Group's randomized clinical trials between 1976 and 1982, suggests that the histologic distinction between Burkitt's and non-Burkitt's tumor is clinicopathologically irrelevant in children. Patients with undifferentiated lymphoma were stratified morphologically into three subtypes: Burkitt's (B; 18 patients); non-Burkitt's (NB; 21 patients); and small noncleaved, not-otherwise-specified (NOS; 10 patients). Median age at presentation was 10 years for B; 12 years for NB; 6 years for NOS; and 10 years overall. Univariate analysis of clinical and laboratory data at presentation, yielded no significant differences between B, and NB patients. Complete remissions were obtained in 75% of the patients, and there were no significant differences in complete remission rate among the different morphologic subtypes of undifferentiated lymphoma. There were no significant differences in the estimated disease free survival between B, and NB patients. No morphologic parameters were identified that were predictive of prognosis.

Adolescent

A morphologic study of childhood lymphoma of the diffuse "histiocytic" type. The Pediatric Oncology Group experience.

Of 227 cases of pediatric non-Hodgkin's lymphoma with adequate histopathologic material for review, 72 (32%) were classified as diffuse "histiocytic" lymphoma (DHL). These cases were further divided into different morphologic subtypes according to the Lukes-Collins classification, and the National Cancer Institute Working Formulation, to ascertain whether there were any significant prognostic differences among the different subtypes. The results of our study showed that 40 patients were classified as immunoblastic lymphomas, and 32 were called large follicular center cell (FCC) tumors. Of the 40 patients with immunoblastic histology, 19 had morphologic features of the clear cell type and were interpreted as consistent with T-immunoblastic lymphomas; an additional two had polymorphous features also consistent with T-cell type: 17 had plasmacytoid features, and were morphologically classified as B-immunoblastic lymphomas; two could not be subtyped. Of the 32 patients with morphologic features of FCC lymphomas, 29 were classified as large noncleaved type, and three as large cleaved type. A clinicopathologic analysis showed that 90% of the patients obtained complete remission, and there were no significant differences in complete remission rate among the different morphologic subtypes of DHL. The estimated five year disease-free survival for all patients was over 70%, with no failure after the second year; and there were no significant differences in the disease-free survival among the different subtypes. The only clinical differences that we found, were that patients with lymphomas of FCC (large noncleaved) type were younger (P = 0.01); had less nodal involvement (P = 0.03); and had more organ involvement (P less than 0.01). We conclude that the morphologic subclassification of DHL in children currently has limited clinical prognostic significance.

Adolescent