Metacarpophalangeal pattern profiles: Q-score for ages 3 years to adult with epiphyses: an update.
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Adolescent↗
Biomedical subjects
P F Dijkstra
Publications and source records attributed to P F Dijkstra.
Radiography of the carpal scaphoid. Experimental evaluation of "the carpal box" and first clinical results.
RATIONALE AND OBJECTIVES: The authors describe a new device ("the carpal box") for the radiographic detection of occult scaphoid fracture. METHODS: Fractures in the scaphoid of five cadaver specimens were mechanically produced. Subsequent examinations included conventional scaphoid radiography, multi-angle radiography, and radiography using the new device. The anatomic analysis of the specimens served as the standard for comparison. A pilot study was performed in six consecutive patients with suspected scaphoid fracture. RESULTS: Neither scaphoid radiography nor multi-angle radiography could confirm a fracture in two specimens, whereas all fractures were recognized on the carpal box radiographs. All scaphoid fractures were visualized by carpal box radiography, whereas scaphoid radiography was equivocal in one patient and negative in the other. CONCLUSIONS: Carpal box radiography may have additional value in the diagnosis of occult scaphoid fracture. This may lead to a reduction in costs and inconvenience for patients with clinically suspected scaphoid fracture and negative scaphoid radiography.
Carpal Bones↗
MCA/MR syndrome in two female siblings: new entity or variant examples of Coffin-Lowry versus Atkin-Flaitz syndromes?
We report two sisters with mental retardation, coarse facial features, telecanthus, flat malar region, prominent lower lip, kyphoscoliosis, and tapering fingers. Although these patients' phenotypes showed considerable overlap with the Coffin-Lowry and the Atkin-Flaitz syndromes, their overall picture makes these diagnoses controversial.
Abnormalities, Multiple↗
Metacarpophalangeal pattern profiles: Q-score for ages from birth to 7 years.
The metacarpophalangeal pattern profile (MCPP) analysis is a widely used method of standardization of bone length measurements to quantify congenital skeletal dysplasia of the hands. A main source of inaccuracy is the prominent scatter in the values of the standard deviation (S.D.) of the reference length, which is used in the calculation of the standardized values (Z-scores). Other sources of error, especially when bone length measurements of younger children are evaluated, are the rather large variability in the age of appearance of the epiphyses of the different bones, and the use of tabulated reference data which are available only at one-year intervals. We have devised another method of standardization of bone length measurements, by calculating the Q-score. Its advantages are that no S.D. values are needed and that more accurate results are obtained. Moreover the interpretation of the Q-scores is more straightforward than the interpretation of the Z-scores. Q-scores for 3 patients with Sotos syndrome are presented. Use of the MCPP in the form of the Q-score, will probably make it an even stronger tool than before.
Bone Development↗
Metacarpophalangeal pattern profile analysis in Rubinstein-Taybi syndrome.
Metacarpophalangeal pattern profile (MCPP) was determined in 49 radiographs of 40 patients with Rubinstein-Taybi syndrome. Two recognizable hand profiles were seen, depending on the configuration of the thumb and on age. Patients with a straight thumb showed a short first proximal phalanx, and short third medial phalanx. Patients with a radially deviated thumb had a short first proximal phalanx. Depending on age, a relatively large (infancy) or markedly short (older patients) first distal phalanx was found. The similarity between the patients was high. A third group of patients did not show a particular hand profile, but only small hand bones. The pattern variability indices were high in all groups of patients. MCPP analysis in Rubinstein-Taybi syndrome seems to be a powerful, but not pathognomonic, diagnostic tool.
Abnormalities, Multiple↗
The prenatal development of the normal human skeleton: a combined ultrasonographic and post-mortem radiographic study.
Post-mortem radiography of fetuses with skeletal dysplasia is essential for diagnostic classification. Interpretation of the radiographs should be based on the knowledge of morphology and dimensions of the normal skeleton in all stages of development. A retrospective post-mortem radiographic study is presented with measurements of the lengths of the long bones and thoracic and lumbar spine. The study included 69 fetuses and neonates with a normal skeleton, whose gestational age ranged from 13-42 weeks and who died perinatally or lived for less than one week. The measurements of the long bones were plotted on growth curves obtained from a prospective longitudinal ultrasonographic investigation of another group of 63 normal fetuses from 12-40 weeks of gestation. Thoracic and lumbar spine measurements by ultrasonography were not available. The radiographic data of thoracic and lumbar spine were, therefore, compared to radiographic studies from the literature. No disagreement with these studies was found. It can be concluded that measurements of bones from standardized post-mortem radiographs in cases of questionable gestational age or defects of bone development can be compared with ultrasonographic measurements. To illustrate the usefulness of the graphs, 13 fetuses with various types of skeletal dysplasia were evaluated retrospectively.
Bone Diseases, Developmental↗
Cystic rheumatoid arthritis: description of a nonerosive form.
In a study of patients with rheumatoid arthritis (RA), 9% (n = 70) were found to have a cystic form. At radiologic examination of these patients with cystic RA, the first abnormality seen consisted of periarticular intraosseous cysts without erosions. The cysts were distributed symmetrically, most often located at the proximal side of the joints and predominantly around the proximal interphalangeal, metacarpophalangeal, and wrist joints of the hands and the first interphalangeal and metatarsophalangeal joints of the feet. Computed tomographic scans showed the peripheral intraosseous location of the cysts. Magnetic resonance images showed that the cysts may contain fluid, inflamed synovia, or both. Cysts can be an important feature in the diagnosis of RA and a supplement to the criteria of the American Rheumatism Association. Osteoporosis, joint-space narrowing, and joint destruction occurred less frequently in patients with cystic RA than in patients with classic RA. Of the patients with cystic RA, 54% were male, and 50% were seronegative. This study is a supplement to and an enlargement on earlier descriptions of cyst predominance in RA.
Adolescent↗
Complex motion tomography of the sacroiliac joint. An anatomical and roentgenological study.
To find a better method for diagnosing sacroiliac (SI) joint disease, an anatomical approach was combined with conventional roentgenology, complex motion tomography and computed tomography. Complex motion tomography is suggested as the method of choice in the investigation of the SI-joint. Because of its complex (sinusoidal) form, the dorsal portion of the joint has to be tomographed in frontal projection and the middle and ventral portions in oblique projection. In 56 patients, referred for probable ankylosing spondylitis, 72 SI joints were investigated. Based on plain radiography six and on frontal tomography five SI joints were diagnosed as normal. However, based on oblique tomography 31 joints were diagnosed as normal.
Female↗
Radiosynoviorthesis of the knee: a doubleblind trial of 1 versus 5 mCi Gold-198.
Since the minimal effective dosage for radiosynovectomy is not known, we have performed a doubleblind controlled trial of 1 mCi vs. 5 mCi 198Au in 60 knees of 46 patients with persistent rheumatoid synovitis. The therapeutic effect was assessed with a clinical score, 99mTc pertechnetate uptake measurements and plain X-rays. With regard to clinical parameters no statistical differences between the two groups were found, but results indicate a trend towards a better outcome in patients treated with 5 mCi. 99mTc uptake improved significantly in patients treated with the higher dose and not in the low dose group. Weighing ease of treatment and risk of side effects against the small difference in effectiveness, we conclude that the use of less than 5 mCi of 198Au may be justified. During the study the injection technique was changed, enabling a comparison of two methods. Leakage to lymph nodes and liver decreased when the needle was flushed with normal saline after administration of the radiocolloid.
Aged↗
Leiomyosarcoma of the suprarenal and renal veins, report on two cases.
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Adult↗
The cystic form of rheumatoid arthritis.
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.
Adolescent↗
Achondrogenesis-hypochondrogenesis: the spectrum of chondrogenesis imperfecta. A radiological, ultrasonographic, and histopathologic study of 23 cases.
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.
Bone Matrix↗
Perinatal lethal osteogenesis imperfecta: radiologic and pathologic evaluation of seven prenatally diagnosed cases.
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.
Female↗
The use of HLA-B27 as a diagnostic and prognostic aid in acute anterior uveitis (AAU) in The Netherlands.
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.
Back Pain↗
Rheumatoid arthritis of the shoulder. Description and standard radiographs.
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.
Acromioclavicular Joint↗
Cerebral gigantism (Sotos' syndrome). Metacarpophalangeal pattern profiles.
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.
Abnormalities, Multiple↗
The venous phase in hand angiography.
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.
Angiography↗
Hereditary malformation of the toes and fingers in a family with double translocation heterozygosity.
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.
Abnormalities, Multiple↗