摘要:

Five autopsy cases of idiopathic pulmonary hypertension (IPH) in infancy and childhood are reported. In two cases, the diagnosis had been verified at cardiac catheterization, whereas in the remaining three cases the diagnosis was based upon indirect clinical and morphological evidence. The pulmonary vasculature was studied by microangiographic and histological techniques, including morphometry of muscular pulmonary arteries. In two neonatal cases (ages: 5 days, 16 days), there was tortuosity of intralobular pulmonary arteries and carry‐over of the foetal type of thick‐walled muscular pulmonary arteries and arterioles with moderate superimposed medial hypertrophy. In one subject, aged 8 months, muscular and transitional pulmonary arteries showed considerable medial hypertrophy, but no obliterative features. The increase of medial mass in muscular pulmonary arteries and the absence of obliterative vascular lesions suggest that a vasospastic mechanism was responsible for the pulmonary hypertension in these three cases. In the two cases of older patients (ages: 1 year and 10 months, 10 years) the microangiograms and the lung sections displayed a more complex pattern of severe vascular lesions: medial hypertrophy in muscular pulmonary arteries, obliterative intimal fibrosis, arterial necrosis and plexiform lesions. These vascular changes are identical to those found in cases of IPH in adult patients, as well as to the pulmonary vascular lesions in severe secondary hyperkinetic pulmonary hypertension. The clinical symptoms were dominated by cyanosis in the two neonatal subjects. In the three cases of older patients, syncopal attacks and/or sudden death was a common feature. This indicates that IPH should be considered among the possible causes of sudden, unexpected death in infancy and childh

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01812.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

Two or more parathyroid glands, of normal gross and histologic appearance, were identified in all of 88 infants who had died suddenly and unexpectedly and in 47 of 49 controls of similar age. Close approximation or intimate fusion of the parathyroid with cervical thymic tissue, either a cervical continuation of the major thymic mass or, more frequently, an independent nodule of thymic tissue, was observed in 58 per cent of the infants who had died suddenly and in only 37 per cent of the controls. Such union is considered to be a variant of normal in the infant. In many of the controls the stress of the terminal illness, not experienced by the infants who had died instantaneously, may have been responsible for involution of such cervical thymic masses so that they were no longer readily demonstrated.

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01813.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

A comparison between the results of chromosome analyses from persons exposed to chronic external irradiation and chromosome analyses from patients having received treatment with radiophosphorus shows that chromosome aberrations in the peripheral blood are found in both cases, and that the aberrations are similar. A quantitative correlation between the radiation dose from chronic external exposure and the aberration yield will involve so many unknown parameters that it seems irrelevant. A quantitative determination of the chromosome aberrations can however, when sufficient data have been collected, probably be used for biological dosimetry, provided that the exposure is approximately uniform. A correlation between the amount of radiophosphorus administered and the aberration yield cannot be used for biological dosimetry, at least if the treatment, as is the case in practice, is given discontinuously.

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01814.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

The sequence of events in long‐term cultures in lymph node fragments and in underlying three dimensional matrix of gelatin foam (Spongostan) has been studied histologically. Lymphoid cells together with reticulum‐like cells survived excellently in the periphery of the original lymph node fragments until the time of lymphoblastoid transformation 8 weeks after explantation. The centre of the lymph node became increasingly fibroid from the third week onwards. The Spongostan matrix was gradually converted to an organotypic structure where lymphoid cells survived and proliferated. During week 3–6 after explantation only few lymphoid cells were discharged into the medium. At the same time, however, the number of such cells did not decline within the explant. The diminished release could thus not be explained by increased lymphoid cell death at their source of origin,i. e.the original explant. The findings support that lymphoblastoid cells go through a phase of adaptation within the ex

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01815.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

The testes were studied microscopically in 15 severely mentally retarded men with cerebral malformations. They ranged from 19 to 71 years in age. The testicular picture was normal in 8. In 3 there were moderate or severe testicular changes of the type usually classified as being of extrahypophyseal origin. In 1 there were hardly any demonstrable Leydig cells in an adult testis and in 1 the testes were of infantil type. In both these cases there was wide‐spread injury to the hypothalamus or structures associated with the hypothalamus. In 2 men the picture of the testes was partly of infantile type and partly of adult type with hyperplasia of interstitial cells. In both cases there were malformations of different organs and signs of endocrine disorders. These two men showed extensive cortical cerebral malformations. It is possible that the picture of the testes may be explained by injuries of the cerebral cortex with consequent disturbed stimulation of the teste

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01816.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

In a clinically diagnosed case of Sanfilippo's syndrome (Mucopolysaccharidosis type III) histopathological examination of the eyes revealed accumulation of a granular material mainly in the sclera and to a lesser degree in the cornea and ciliary body. Similar scleral changes have previously been reported in various types of mucopolysaccharidosis in six cases only, and ciliary body changes in two cases only. It is emphasized that ocular involvement including corneal changes may be found histopathologically even if not clinically detected. The main histopathological findings in the cerebrum and cerebellum were ballooning of ganglion cells similar to the findings in other types of storage disease. By applying a battery of histochemical methods to the ocular and cerebral material, the findings indicated 1) a difference between the stored material in the brain and in the eyes, 2) that the stored material in the ganglion cells of the brain was probably of a ganglioside nature and 3) that the material in the eyes was consistent with an acid mucopolysaccharide, undoubtedly sul‐phated and probably rich in heparitin sulphate. These findings are compared with the suggested biochemical changes and the correlation between these and the morphology is discussed. The writer is in favour of the theory of a basic enzyme defect leading to accumulation of the granular material in the cells. Addition of normal and pathological cytoplasmic constituents are probably responsible for the difference in histochemical findings, especially in fibrocytes and ganglion cells, as revealed by the present study. The eyes have not previously been histopathologically or histochemically examined in Sanfilippo's syndrom

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01817.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

In a material consisting of liver biopsies from 287 consecutive chronic alcoholics Mailory bodies have been found in 29. Of these 18 show cirrhosis and fatty infiltration (corresponding to 44 per cent of the total number of specimens showing cirrhosis and fatty change) and 11 (corresponding to six per cent) show fatty change but no cirrhosis, while Mailory bodies have not been demonstrated in biopsies without fatty change. The incidence of Mailory bodies and alcoholic hepatitis in consecutive materials of liver biopsies from chronic alcoholics appears to be ten and eight cent respectively.

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01818.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

An analysis of the findings of adrenal cytomegaly in foetuses and infants in a five‐year autopsy material showed that the frequency increased with gestational age and lifetime. Therefore no basis was found for regarding cytomegaly as a normally occurring feature of foetal life. In cases of erythroblastosis, maternal toxaemia, and multiple pregnancies the frequency was found to be slightly increased, but the most striking feature was the high frequency of severe malformations. It is discussed whether the high occurrence of malformations speaks in favour of viral induction or chromosome anomaly. The cellular infiltration and the marked necrosis of the foetal cortex, which cannot be explained by the physiological involution, speak in favour of a viral aetiology. Finally, the resemblance between cytomegaly and the cellular changes in idiopatic Addison's disease and congenital adrenal hypoplasia of cytomegalic type is mentione

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01819.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

The Achilles tendon of the rat was studied before, immediately after, and 1–8 days after experimental rupture of the tendon which was induced in wild brown rats. The specimens were stained with haematoxylin‐eosin, alcian blue, and toluidine blue. The tendon ruptures proved to be independent of degenerative changes. Immediately after the tendon rupture very scanty changes were found, in the form of slight fraying between the fibre bundles and a slight increase of the wavy structure. Through the first 24 hours these changes increased. At the same time, disintegration of the individual fibres occurred, with slightly reduced stainability and in places pyknotic nuclei. It is concluded that these findings represent the mildest changes that may be expected in a biopsy specimen from a previously healthy tendon which has been subjected to a purely traumatic subcutaneous rupture. On the basis of the present findings and of previously reported experimental studies, it is concluded that the changes described elsewhere following subcutaneous rupture of the human Achilles tendon have not necessarily been present prior to the rupture. Consequently, the author cannot accept the biopsy findings from human cases as a convincing link in the argumentation that previous degeneration is a necessary condition for subcutaneous rupture of human tend

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01820.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

The cellular decay in mensteric lymph node cell suspensions was studied by combining nigrosin dye exclusion and3H‐thymidine autoradiography. The labelling pattern of non‐viable cells was followed during a period of 14 days after injection of a single dose of3H‐thymidine into NMRI‐mice 30 to 45 days of age. The decaying cells were divided into DNA‐synthesizing and non‐DNA‐synthesizing cells. The decay of DNA‐syntresizing cells partly reflects' a decay of blast cells proliferatingin situ, i. e.germinal centre cells with a decay rate of 20 per cent. The majority of cells decaying in the lymph node seems to be carried to the organ by the blood or lymph stream. The results are discussed on the basis of knowledge of lymphoid cell migration streams between the various lymphoid organs and the existence of short‐lived lymph

ISSN:0365-4184    

DOI:10.1111/j.1699-0463.1971.tb01821.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY