Hepatoblastoma and polyposis coli (familial adenomatous polyposis)
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Biomedical subjects
Publications and source records attributed to J Kingston.
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Epithelioid cell granulomas occur in association with a wide range of neoplasms but necrotizing granulomas mimicking tuberculosis are less well recognized. Two cases of high-grade malignant lymphoma of Burkitt type are presented which were associated with such a response. In both cases this caused difficulty in interpretation and in one led to delayed diagnosis. Regional lymph nodes were not involved by lymphoma but contained epithelioid cell granulomas in both cases. Features that suggested that the granulomatous reaction was related to the tumour included a close spatial relation, the reticulin pattern in the areas of necrosis and the immunohistochemical demonstration of lymphoid antigens in the necrotic debris. A diligent search for mycobacteria and other micro-organisms failed to reveal any infective cause. We suggest that the local granulomatous response is a reaction to the presence of necrotic and poorly viable tumour and the granulomas in nearby lymph nodes may be a response to tumour derived debris.
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There were 16 cases of orbital recurrence in a consecutive series of 317 children with retinoblastoma referred to a specialist centre. The incidence of orbital relapse amongst children treated at the centre from the outset of their disease was 2.5%. In every case the patient was the first affected family member. The ocular tumour was therefore not anticipated and had commonly been detected at an advanced stage. Systemic staging investigations detected extraorbital spread in six of the 16 and none of these children survived. One of the remaining ten children with no evidence of dissemination at the time of diagnosis of orbital recurrence is a long-term survivor. No child survived after orbital exenteration or radical orbital radiotherapy alone. Three children received a combination of radiotherapy and adjuvant chemotherapy. Only one of the three was free from systemic disease at relapse and this child is the only survivor. In children apparently free from widespread retinoblastoma at diagnosis of orbital recurrence, distant relapse was the commonest cause of death though several children died from direct intracranial extension. It is advocated that orbital recurrence of retinoblastoma is treated by excision biopsy of the tumour mass followed by radical orbital radiotherapy to a dose of 50 Gy. This should be combined with adjuvant chemotherapy and, where a risk of direct intracranial extension exists, by neuraxis irradiation. No other child in this series with evidence of local extraocular extension of retinoblastoma at enucleation and who had received radical orbital radiotherapy to a full dose of 50 Gy subsequently recurred in the orbit.(ABSTRACT TRUNCATED AT 250 WORDS)
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Eighty children aged less than 16 years with newly diagnosed Hodgkin's disease were treated between 1974 and 1982. Complete remission occurred in 95%, with actuarial five year overall survival of 94%, and relapse free survival of 82%: median follow up was 4.8 years. Sixty one children were staged clinically while 19 had staging laparotomies before treatment. Most received combined modality treatment with Ch1VPP chemotherapy (chlorambucil, vinblastine, procarbazine, and prednisolone) followed by irradiation of initial bulk disease. Nodular sclerosis predominated in both sexes, accounting for 60% of the total. Girls with stage IV disease, nodal sclerosis histology, and bulky mediastinal masses had a relatively poor prognosis. Ten children have relapsed, and three prolonged (6 to 7 years) second remissions have been observed. Four died of disease, and one from infection. Clinical staging, avoiding splenectomy, reduced the risk of serious infections. Our current policy is to treat stage IA disease with local irradiation and all other stages with chemotherapy, adding irradiation for bulky mediastinal disease.
The predominant potential pathogens in the vaginal flora, sampled before operation in 41 patients undergoing gynaecological surgery, included E. coli, alpha-haemolytic streptococci, Streptococcus faecalis, anaerobic Gram-positive cocci and Bacteroides fragilis. All the aerobic Gram-negative bacilli and 89.7 per cent of the streptococci isolated were sensitive to 4 microgram/ml of cefuroxime. Most of the anaerobes were also very sensitive to cefuroxime, although the minimum inhibitory concentration (MIC) for 52 per cent of B, fragilis isolates was at least 8 microgram/ml. The MIC of metronidazole was less than 1 microgram/ml for almost all anaerobes. All patients were given 1 g of metronidazole by suppository on the night before operation and a second suppository was given, together with 750 mg of cefuroxime injected intramuscularly with the premedication. The mean extravascular tissue concentrations of cefuroxime and metronidazole, assayed in 29 patients, were respectively 8.9 and 8.0 microgram/g (uterus), 14.2 and 22.3 microgram/g (cervix), 13.1 and 6.3 microgram/g (Fallopian tube), 12.3 and 2.1 microgram/g (ovary), 10.3 and 12.4 microgram/g (vagina), 13.3 and 13.7 microgram/ml (serum), 3570.3 and 122.3 microgram/ml (urine). None of the patients developed postoperative wound infections or adverse reactions.
The hypoglycaemia of infantile hyperinsulinism is often exceedingly difficult to control. The use of somatostatin has been advocated recently in such infants because of its effect on inhibiting insulin release, but nothing is known of the wider effects of this potent hormone in the young child. Two infants presenting at 9 weeks and 5 days of age with severe hyperinsulinaemic hypoglycaemia were studied during an infusion of somatostatin. In both infants normoglycaemia was restored with suppression of insulin secretion. An increase in blood ketone bodies occurred, but no change was seen in blood pyruvate, lactate or alanine concentrations. The plasma concentrations of glucagon, cortisol, growth hormone, motilin, pancreatic polypeptide, gastric inhibitory of polypeptide, neurotensin, gastrin and vasoactive intestinal peptide decreased markedly during the somatostatin infusion. No consistent change occurred in plasma enteroglucagon or secretin values. We conclude that somatostatin effectively suppresses abnormal insulin secretion in infants, but it has profound effects on the release of nine other hormones. Further studies are needed to define the consequences of suppressing the release of these hormones before somatostatin can be used routinely in the management of infantile hyperinsulinism.
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This paper presents evidence that the acoustic effects of demonstrably independent articulations are integrated in the perception of vowel height and [voice] contrasts. This evidence supports two hypotheses: That, in at least some instances, articulatory covariation is perceptually motivated, and that the objects of speech perception are auditory rather than articulatory. Furthermore, it appears that perceptual integration can either increase or decrease the distinctiveness of contrasting speech sounds, so it may not only create favored patterns of articulatory covariation but also disfavored patterns. This suggests that integration is a product of general auditory mechanisms, not of mechanisms specific to speech perception.
Laboratory phonology tests the predictions of phonological analyses through experimental investigations of phonetic behavior. This paper describes how a laboratory phonologist might investigate three issues in the analysis of the prosody of signed languages: the internal structure, if any, of the signed syllable, the realization of lexical and phrasal prominence, and the marking of edges. The paper proposes to investigate the internal structure of the syllable by adapting a variety of psycholinguistic techniques that have been extensively used to study the phonetic packaging of phonological material in spoken languages. Phonological and phonetic arguments are presented in support of the hypothesis that signed languages will increase movement kinematics to convey phrasal and perhaps lexical prominence, and an experiment is sketched that would test this hypothesis. Finally, the paper suggests that the experimental study of the edges of sign articulations might allow a clearer choice between competing phonetic and phonological accounts of external sandhi in language in general.
Experience of myoelectric prostheses for unilateral below-elbow amputees in a large region of Great Britain is presented. Of the cases considered, 14 were adults and 29 were 16 years of age or less. The rejection rate was 25% overall but it was lower in adults, at 17%. Comparisons with published data and other aspects of myoelectric prostheses are discussed. Just over half the 37 amputees interviewed were independent in activities of daily living; independence was more common in the adults, at 77%. A very large proportion of myoelectric prosthesis users also used body-powered prostheses, demonstrating a need for both types of prosthesis for optimum rehabilitation. It is recommended that myoelectric prosthetic programmes should be carried out from specialized centres with comprehensive facilities for this expensive rehabilitation aid.
A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma. The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.
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