Leiomyosarcoma of the suprarenal and renal veins, report on two cases.
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Biomedical subjects
Publications and source records attributed to P F Dijkstra.
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A non-erosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A. The E.S.R. is often low, half of the patients remained seronegative and there are 35 males and 27 females in the group. A smaller group of 15 out of these patients could be followed from a stage wherein the radiographs were normal to a stage of extensive cystic changes, over a period of at least 6 years. An attempt is made to delineate this group within the Rheumatoid arthritis disease entity.
In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis have recently received special attention. We describe 23 cases representing the different subtypes. Within the classical type I (Parenti-Fraccaro) two distinct disorders can be recognized: type IA (Houston-Harris) and type IB (Fraccaro). The classical type II (Langer-Saldino) and hypochondrogenesis represent phenotypic variants of one disorder in which type II is the most severe form and hypochondrogenesis the mildest form, while transitional forms exist. It is likely that a basic defect in cellular function of the chondrocyte results in a deficient cartilage matrix and in disorganized enchondral ossification.
The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), diagnosed prenatally by ultrasound in the 15th to 34th week, are described. They include four variants of the Sillence classification: types IIA, IIB, IIC, and type III. The radiologic criteria that differentiate these types of OI are described. The histopathology of the bones differed only slightly in types IIA, IIB, and III; OI type IIC, however, differed markedly from the other types.
Acute anterior uveitis (AAU) may be considered to be one manifestation of the seronegative spondylarthropathies of which ankylosing spondylitis (AS) is the prototype, especially when the patient is HLA-B27 positive. However, it is not yet clear under which circumstances a patient with AAU should be referred to the rheumatologist. In a retrospective study we evaluated the management of 68 consecutive HLA-B27+ AAU patients from a rheumatologic point of view. Although the majority (73%) showed rheumatic problems, only half (52%) of the patients was referred to a rheumatologist, due to problems in evaluation of clinical history and of x-ray reading of the sacroiliac (SI) joints. Because HLA-B27 typing will determine whether the AAU patients "at risk" have AS or a related arthropathy, we suggest using HLA-B27 typing in AAU patients as a diagnostic and prognostic aid. When the AAU patient is found to be HLA-B27 positive, the rheumatologist will be able to "split" these patients into those with AS and those without. Early diagnosis of AS in AAU patients is important as an early start of drug therapy and physiotherapy may prevent deformities and improve final rheumatologic outcome.
An in depth study on growth, bone age, cranial CT scans and plasma somatomedin activity (SM-act) was made of 22 children with Sotos syndrome. In addition to the known characteristics of the syndrome, thin and brittle nails were found in three adolescent patients. The mean body stature, expressed as standard deviation score, increased from 2.2-2.8 in the 1st year of life, followed by a fall to 2.0 in the 2nd year. Thereafter the SDS increased slowly to values of 3.0 at 10 years of age. At least two subjects have reached an exceptionally tall final stature. After the age of 2 years, delta SDS/year remained very stable (-0.1-0.2), concurring with growth velocities in the upper normal range. Bone age was advanced in all patients. Cranial CT scans showed ventricular widening, mid-line cava and Sylvian anomalies in nine, six, and three patients respectively. SM-act dropped from high or normal values in the 1st year, to below normal from 1-5 years, and returned thereafter to the lower half of normal or below the normal range.
The course of rheumatoid arthritis in the shoulder is evaluated in 143 patients. In a period of 29 years, 630 x-rays were taken of 286 shoulders. In this series 2 or more x-rays per shoulder were taken of 89 patients (29 male, 60 female). The various changes in the glenohumeral and acromioclavicular joints are described. Gross destruction appears to be rare, compared to the more frequently seen minor cystic changes. The progress of the disease is often slow or halting. One or both of the shoulders in some of the patients (15 male and 29 female) did not have any detectable x-ray changes, although some of them were followed up for more than 20 years. During our follow-up it became apparent that the acromioclavicular and glenohumeral joints do not follow the same course neither in time nor in severity of joint destruction. Therefore, we divided the shoulder joint into the acromioclavicular and glenohumeral joint. One normal stage and 5 stages of pathology are recognised to fit into previously published schemes of the other joints. Stage 5 appears to be a new phenomenon of neojoint formation, under the previous humeral head with the inferior glenoid rim. Joint disease in the acromioclavicular joint could be divided only into 3 stages.
The metacarpophalangeal pattern (MCPP) profile in 17 out of 22 patients with Sotos' syndrome was studied throughout the growth period, from 2 weeks to 19 years of age. Two typical MCPP profiles will be described. The first type of profile was found in 13 patients. Although the form of the profile is the same throughout the years, the lengthening of the fingers is most pronounced around 2 years of age. Before and after that period there is a flattening of the curve. The metacarpals are relatively longer below the age of 3 years, above 3 years the lengthening of the proximal phalanges is more pronounced in the profile. The second type of profile was found in 4 patients. This curve is almost a mirror image of the first profile. The fingers are not as long as in the first type and the profile is less pronounced. In one of the 17 patients the transition from the first type to the second type could be demonstrated. One patient had a normal profile.
Sialography is one of the modalities that may offer a relatively simple diagnostic investigation in sarcoidosis. The present study revealed an abnormal sialogram of the parotid glands in 20 of 29 patients with pulmonary sarcoidosis. The radiographic parotid gland findings in sarcoidosis are discussed.
The analysis of measurements of phalangeal lengths with the metacarpophalangeal pattern profile plots (MCPPP), has been further extended. I suggest to correlate these measurements to the total length of the phalanges in order to obtain only one mean and standard deviation table for all ages from 3 years to adults. The technique described has proved very useful for comparing published pictures with actual x-ray images. The technique also yields an enhancement of small peculiarities in the plots of a given malformation. As an example the plots of the tricho-rhino-phalangeal syndromes type 1 and 2 (Langer-Giedion syndrome) are presented and compared with the literature.
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It has been found that the venous phase of hand angiography can be reliably visualized with metrizamide. There are indications that the venous phase can provide important information on the validity of fingers with arterial occlusion.
A hereditary malformation of the metatarsophalangeal joints, called here "tear drop" deformity, and a cut off appearance of the distal phalanges of the toes is described in a family with double translocation t (7; 12), t (2; 6) heterozygosity. A bracydactyly E was also found. These disorders seemed unrelated to the chromosomal aberration.
Double translocation heterozygosity t(2;6),t(7;12) in three generations of a Dutch family is described: the segregation of a double translocation in more than one generation has not been previously published. The index case was a 16-year-old mentally retarded boy with partial trisomy 12p who showed several dysmorphic features such as high prominent forehead, flat face, flat and short nose bridge, short nose, dysplastic ears, prominent lower lip, and several skeletal abnormalities. Based on the findings in this patient and those in nine other cases, the existence of a specific trisomy 12p syndrome is postulated.
Discussion of the humeral head compression and the pectoralis minor compression as a possible reflection of the thoracic outlet compression syndrome. Pictures of both compressions on the axillary artery are presented.