Biliary atresia: new concepts of management.
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Biomedical subjects
Publications and source records attributed to D M Hays.
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During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.
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The learning experience of 150 third-year medical students in the specific area of paediatric oncology during a 6-week required paediatric clerkship, was assessed. The clerkship, during which emphasis is placed on patient contact, was based at each of two institutions (A and B). Institution A, with a large relevant patient population and more teaching staff in the field of paediatric oncology, relies primarily on incidental learning relative to this area during the clerkship. Institution B, with smaller related patient groups and teaching staff, employs didactic instruction in this field. Results of pre- and ost-testing at both institutions revealed comparable and significant gain in information relative to paediatric oncology in both student groups. Pre-test scores did not increase during the course of the school year, suggesting that the influence of specific prior clerkships was negligible. Differences in the amount learned between individual student rotational groups were less marked in institution A, where opportunities to contact patients and teaching staff concerned with this specific area, was more consistent.
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The Intergroup Rhabdomyosarcoma Study, initiated in 1972, has admitted more than 700 patients with childhood rhabdomyosarcoma in a 6-year period. Although it was designed primarily to answer specific questions about various aspects of radiotherapy and chemotherapy, some lessons have been learned concerning the surgical approach to this disease. Operative resection, which is not always feasible, is determined chiefly by the clinical stage and anatomic site. Thus far, data for some anatomic sites indicated that total gross resection at some point in the treatment schedule achieved better results than did incomplete resection. A possible exception to this occurred when the orbit is the primary site. The incidence of lymphatic metastases from lesions in some anatomic sites (e.g., paratesticular, pelvic genito-urinary, and extremity) was higher than was previously appreciated. This finding will influence decisions concerning surgical biopsy or dissection of regional lymph nodes for many of these lesions. Differences in histology, particularly those relating to the alveolar type, indicated the need for a more aggressive approach to selected lesions, but we need more data to confirm this determination. Lastly, patients who initially received radiotherapy or chemotherapy to pelvic sites have had striking benefits, and this finding should encourage the development of schedules that specify operative resection of these lesions later in the program rather than as the initial treatment.
The characteristics of 554 evaluated patients entered into a clinical trial conducted by the Pediatric Intergroup Rhabdomyosarcoma Committee between November 1972 and September 1978 were examined for their relationship to prognosis. Prognosis was defined as disease-free time and overall survival time in clinical groups I and II and time on study and survival time in clinical groups III and IV; all times were measured from the start of treatment. The percentage of patients surviving 2 years differed significantly among the clinical groups: I, 92; II, 78; III, 64; and IV, 35. The percentage of patients free of disease at 2 years was significantly higher in group I than in group II (83 vs. 72%, respectively); P = 0.02. The patient characteristics of group I most related to disease-free and overall survival were histologic cell type (alveolar, unfavorable), lymphocyte count (low count, unfavorable), and primary site (disease in extremities, unfavorable). In group II, sex (male, favorable) and lymphocyte count (low count, unfavorable) were significantly related to disease-free and overall survival times. Patients in the clinical subgroup with both microscopic residual disease and lymph node metastasis had poorer survival than patients in other subgroups. Primary site of disease was the only characteristic of group III related to length of time on study and to survival. Orbit and the genitourinary system were favorable primary sites, whereas the retroperitoneal area and extremities were unfavorable. In group IV, primary site (genitourinary, favorable) was related to length of time on study and survival. Sex (male, favorable) was related to survival experience.
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Experience in managing the patients with congenital tracheoesophageal fistula without esophageal atresia led to the following conclusions: (1) The pulmonary and gastrointestinal manifestations of this lesion may be subtle and similar to those of other disorders. A high index of suspicion is required to recognize the fistula. (2) Skillful and persistent efforts should be made to establish the diagnosis and localize the fistula before any surgical attempt at correction. (3) Adequate preoperative preparation, cannulation of the fistula and careful surgical dissection will minimize surgical complications.
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The last 20 years have witnessed remarkable improvements in the prognosis of children with many forms of malignant disease. The reasons for these improvements relate not only to the development of better drugs and more effective radiotherapy, but also to the multidisciplinary approach involving surgeon, chemotherapist, radiotherapist and immunotherapist in providing optimum treatment for the child with a particular cancer. In this paper, the changing role of the pediatric oncologist, surgeon and radiotherapist in improving the management of pediatric cancer is discussed.
The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.
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The evaluation of a technique of laparotomy for the staging of Hodgkin's disease in childhood, including 52 operative procedures, is reviewed. A standard protocol, including splenectomy, multiple hepatic and bone marrow biopsies, and the routine sampling of at least six designated lymph node groups, was employed. It was demonstrated that the surgeon is unable to identify Hodgkin's disease by gross inspection and that the biopsy of node groups previously not included in routine laparotomy studies, ie, mesenteric and porta hepatis nodes is essential to staging in childhood. Stage was altered from stages I and II to stages III and IV in 35% of the patients. The incidence of abdominal recurrence following a negative laparotomy was 7%, and the incidence of post-splenectomy hyperacute infection was 4.5%. This study included minimal use of the lymphangiogram (33%), which accounted for the relatively larger group of patients in clinical stages I and II.