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Biomedical subjects

A K Poznanski

Publications and source records attributed to A K Poznanski.

At least 19 recordsLinked to original sources

Bone mineral disorders in children: evaluation with dual x-ray absorptiometry.

PURPOSE: To evaluate the utility of dual x-ray absorptiometry (DXA) in children with bone mineral disorders. MATERIALS AND METHODS: In phase 1, radial DXA was compared with single-energy photon absorptiometry (SPA) (n = 117). In phase 2, radial and lumbar bone mineral density (BMD) measured with DXA and second metacarpal cortical thickness were compared (254 examinations, 224 children). RESULTS: For radial BMD, DXA and SPA correlated well (r = .956) and SPA-equivalent values could be calculated from DXA measurements (mean residual error = 0.024 g/cm2). After controlling for age, sex, weight, and height, partial correlations were very small for lumbar BMD with radial BMD (r = .186) and lumbar BMD with cortical thickness (r = .158), and slightly better for radial BMD with cortical thickness (r = .544). Z scores also correlated poorly with no meaningful correlation for lumbar BMD with radial BMD (r = .07) CONCLUSION: In children with bone mineral disorders, radial DXA and SPA measurements correlate well. However, lumbar BMD, radial BMD, and cortical thickness correlate poorly and lumbar BMD frequently does not identify abnormality in patients with abnormal radial BMD. Lumbar BMD alone is not adequate for evaluation of bone mineral status in these patients.

Absorptiometry, Photon

Radiological approaches to pediatric joint disease.

With the recent advances that have become available with imaging, we are able to gain considerable information about joint diseases. Although conventional radiography still plays a very important part in the diagnosis of joint disease, ultrasound, computed tomography, magnetic resonance imaging, and nuclear medicine studies have all enhanced our ability to evaluate joint disorders. Each of these modalities need not be used in every patient but when chosen carefully, can efficiently provide useful information both for diagnosis and management. These various modalities are also extremely useful to rule out the mimics of joint disease. Magnetic resonance has recently been shown to be useful in evaluation of dermatomyositis and of vasculitides in the brain.

Arthritis

Autosomal recessive cerebellar hypoplasia and endosteal sclerosis: a newly recognized syndrome.

We describe a brother and sister and an unrelated boy with congenital cerebellar hypoplasia and endosteal sclerosis. All 3 children presented with ataxia and developmental delay, and were found to have microcephaly, short stature, oligodontia, strabismus, nystagmus, and congenital hip dislocation. A previously published case is reviewed. The disorder appears to represent a newly recognized autosomal recessive syndrome.

Cerebellum

Evaluation of joint disease in the pediatric hand.

Juvenile rheumatoid arthritis, leukemia, hypertrophic osteoarthropathy, sickle cell disease, and child abuse can all be causes of joint disease in the child's hand. The role of radiology is to diagnose disease and follow disease progression so that therapy can be modified. Because much of the child's hand is unossified cartilage, measurements, bone mineralization, and maturation are important components of the radiologic evaluation.

Arthritis, Juvenile

Useful measurements in the evaluation of hand radiographs.

Measurements are useful in the radiologic evaluation of the hand. They permit an objective evaluation of subtle alterations in the relative lengths of hand bones, which may be useful in the diagnosis of many congenital malformation syndromes. Evaluation of width of the carpus can be useful in the evaluation of juvenile rheumatoid arthritis as well as many bone dysplasias. Carpal angle measures, the relative position of the distal radius and ulna, as well as cortical measures of the second metacarpal have many clinical applications.

Anthropometry

Ulnar variance in children--standard measurements for evaluation of ulnar shortening in juvenile rheumatoid arthritis, hereditary multiple exostosis and other bone or joint disorders in childhood.

Measurements for radioulnar variance in adults cannot be used in children because the epiphyses are not fully ossified. We describe a method of determining ulnar variance in children by using the distance from the distal metaphysis of the radius to the distal metaphysis of the ulna. Standards for this measurement are presented for ages 1.5 to 15.5 years in boys and girls. These measurements change little with age and may be helpful in establishing shortening of the ulna which may been seen in juvenile rheumatoid arthritis, hereditary multiple exostosis, or other bone and joint diseases with childhood onset.

Adolescent

Applications of the pattern variability index (sigma z) to the quantification of dysmorphogenesis in the hand.

We have generated percentiles for the pattern variability index (sigma z) of the hand in 1,088 normal infants, children, and adults and have analyzed pattern variability indices for 820 individuals representing 50 congenital malformation syndromes with respect to the normal percentiles. The majority of the affected individuals exhibited elevated sigma z values for the hand, some vastly in excess of normal, while such syndromes as Down, Turner, and the Prader-Willi syndrome were low rather than high in pattern variability of the hand.

Adult