Early attainment of sex and race differences in skeletal mass.
Explore the source record for details and available documents.
Black People↗
Biomedical subjects
A K Poznanski
Publications and source records attributed to A K Poznanski.
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↗
Cloverleaf skull associated with unusual skeletal anomalies.
A male infant with cloverleaf skull and multiple other birth defects born to unrelated, healthy, young parents is presented. Radiologic findings in addition to the cloverleaf skull configuration included short, wide clavicles, winged scapulae, unusual shapes of ribs with abnormal spacing between them and with prominent costovertebral junctions, and widely separated ischia. Ulnae appeared angular with probable fusion to the midportion of the radial bones bilaterally. There was polydactyly of the hands and feet with grossly abnormal metacarpal and metatarsal bones. Skeletal maturation was normal. Computed tomography of the skull showed dilated lateral and third ventricles as well as agenesis of the corpus callosum. The mother denies any teratogenic exposure during the pregnancy. The findings in this infant do not seem to fit into any previously described syndrome.
Abnormalities, Multiple↗
Computerized tomography and ultrasound in the diagnosis of cerebro-hepato-renal syndrome of Zellweger.
Renal cortical cysts were demonstrated by computerized tomography and ultrasound in a patient with cerebro-hepato-renal syndrome who did not have patellar calcifications.
Female↗
Gaucher disease of the liver: CT appearance.
We present a child with Gaucher disease with hepatic involvement that caused portal hypertension. Computerized tomography (CT) showed distortion of liver parenchyma and central necrosis of the liver.
Ascites↗
Pediatric thoracic aorta: normal measurements determined with CT.
Normal thoracic aortic dimensions in adults have been established by means of computed tomography (CT), but such measurements are not available in children. To establish normal standards, contrast material-enhanced chest CT scans of 117 children and adolescents, ranging in age from 2 weeks to 19 years, were reviewed retrospectively. Patients with congenital heart disease, vascular anomalies, renal disease, hypertension, and connective-tissue disorders were excluded, resulting in 97 studies. Diameters of the thoracic aortas were measured at three levels. Interobserver and intraobserver variances were determined. A direct linear relationship to patient age was observed, and regression analysis was performed. These standards help in differentiating the normal from the abnormal aorta on chest CT scans of children.
Adolescent↗
CT of children's feet: an immobilization technique.
Explore the source record for details and available documents.
Child↗
Radiological approaches in the evaluation of joint disease in children.
In summary, the newer technologies in radiology have allowed us to visualize more clearly the manifestation of joint disease in children. The presence of small erosions and cartilage damage can be seen much better with magnetic resonance imaging than with any other modality short of arthrography, a much more invasive examination. Joint effusion, although sometimes visualized with conventional radiography, is probably best recognized with ultrasound or magnetic resonance imaging, although it can be detected with computed tomography as well. For the evaluation of avascular necrosis that can be associated with steroid use in joint disease, bone scintigraphy is a simple, sensitive method. Magnetic resonance may be as or more sensitive and gives additional information as well. In the detection of change with time, conventional radiography probably will remain the standard as it is still the simplest, least expensive examination; however, it has many limitations in specific cases. Bone scintigraphy may be of value in selected cases. Although we have still not had enough experience with magnetic resonance imaging to use it as a way of evaluating progress of joint disease, it promises to be the most sensitive radiologic measure of evaluating progress as small anatomical changes can be detected within the cartilage, which cannot be done easily with other means.
Arthritis, Juvenile↗
[Oto-palato-digital syndrome. Clinico-radiological study].
Oto-palato-digital (OPD) or Tyabi syndrome is a familiar, X-linked bone dysplasia with intermediate expression, in females or autosomal dominant with more severe manifestations in males. In the past both the clinical features (flat face with sunken and broad nasal bridge, antimongoloid slant of palpebral fissures, palatoschysis, conductive deafness, short and broad thumbs and big toes, nail dystrophy) and radiological findings (thick and dense base of the skull, prominence of supraorbital ridges, middle ear bone deformities with dense ossicles, large and broad vertebral bodies, posterior defects of neural arches of the vertebrae, carpal and tarsal bone fusions, short and broad nail phalanges) have been well described and established. The present report describes 7 patients (4 females and 3 males) all belonging to the same family (the first described in this country) and all presenting the clinical and radiological features of OPD syndrome. A cranial and spinal CT was performed on one patient, with peculiar findings.
Abnormalities, Multiple↗
Flattening of the distal femoral epiphyses in the trichorhinophalangeal syndrome.
A new finding in the trichorhinophalangeal syndrome, flattening of the distal femoral epiphyses, is described in seven patients. Measurement of the ratio of the height of the distal femoral epiphysis versus both the width of the distal femoral metaphysis and the width of the distal femoral epiphysis and comparison to normal standards confirmed the subjectively observed flattening of the distal femoral epiphysis in these patients, further suggesting that the disease is a generalized process rather than a local disorder.
Child↗
Distal femoral epiphysis: normal standards for thickness and application to bone dysplasias.
Flattening of the epiphysis of the long bones is seen in several bone dysplasias. It is the hallmark of multiple epiphyseal dysplasia and is an important sign in the diagnosis of spondyloepiphyseal dysplasias, diastrophic dysplasia, and pseudoachondroplastic dysplasia. The goal of this study was to determine norms for the height of the distal femoral epiphysis and to apply these standards to patients with bone dysplasias. Ratios of the distal femoral epiphysis height to both the distal femoral metaphysis width and the distal femoral epiphysis width were obtained from 640 radiographs of healthy children of different ages. Application of these standards to 41 patients with the bone dysplasias mentioned above proved useful in ascertaining decreased height of the distal femoral epiphysis. These standards are of particular value in subtle or early cases in which the thinning of the epiphysis may not be apparent upon simple observation. Obtaining three simple measurements from the anteroposterior knee radiographs allows determination of the presence or absence of flattening of the epiphysis.
Adolescent↗
Bone changes from prostaglandin therapy.
Prostaglandin E therapy in infants causes periosteal elevation. Although the changes usually take 30-40 days to become visible, we have seen them as early as nine days. In 15 infants who had prostaglandin E therapy for over six days, three developed periosteal elevation. Three other cases are described in greater detail, with long-term follow-up in two in which the bone remodeled to normal. Gallium scan in one showed increased uptake in areas involved. The periosteal cloaking may mimic Caffey disease but the pattern of involvement is different, since the mandible, which is commonly affected in Caffey disease, is rarely involved in prostaglandin E therapy.
Bone and Bones↗
Respiratory insufficiency in newborns with abdominal wall defects.
Respiratory failure in newborns with abdominal wall defects has been attributed to increased intra-abdominal pressure and elevation of the diaphragm after closure. Despite surgical techniques designed to minimize intra-abdominal pressure, we have observed prolonged respiratory insufficiency in several such infants. We reviewed the charts of 108 infants from 1975 to 1982 who had abdominal wall defects: 53 with gastroschisis, 29 with small omphaloceles, 22 with liver-containing or giant omphaloceles (GO), and four with cloacal exstrophy. Nine infants with GO (41%) had prolonged respiratory insufficiency and five died. Infants with GO required longer periods of oxygenation and ventilation (P less than .001, ANOVA) than infants with other abdominal wall defects. Clinical observation suggested that infants with GO have a small, narrow thorax. We obtained detailed measurements from the chest radiographs of infants in all groups. After correction for birth weight, babies with GO had smaller chest widths (P less than .001) and lung areas (P less than .05) than infants with other abdominal wall defects. At autopsy, one newborn with GO was found to have severe pulmonary hypoplasia. Prolonged respiratory insufficiency in infants with GO may be explained by pulmonary hypoplasia and/or by a narrow chest deformity which limits lung expansion.
Abdominal Muscles↗
The subcutaneous injection port: a new chest-wall artifact.
A totally implanted central venous catheter with a subcutaneous injection port is now in use in patients in whom long term venous access is required. This injection port creates a new artifact that can mimic a pulmonary lesion on chest radiographs.
Catheters, Indwelling↗
Greater trochanteric overgrowth: development and surgical correction.
Greater trochanteric advancement (GTA) is an orthopedic procedure designed to correct the biomechanical consequences of overgrowth of the greater trochanter by moving the greater trochanter and its attached muscles laterally and distally. Thirty-one children with trochanteric overgrowth who had secondary pelvic instability (Trendelenburg sign) were studied and underwent a total of 33 GTA procedures. The clinical and radiologic prerequisites for successful surgery are presented. In addition, the radiographic methods of measuring the lateral position of the trochanter, the articulotrochanteric distance, and the amount of trochanteric overgrowth are discussed. Measurements made on preoperative and postoperative radiographs revealed that surgery achieved a mean displacement laterally of 12.1 mm and distally 21 mm. Clinically, there were few complications (two children with mild myositis, one with broken hardware, one with delayed union of trochanter). Pelvic instability had disappeared in the 25 of the 30 patients who could be evaluated.
Adolescent↗
Leg-length determination with biplanar CT scanograms.
Computed tomography (CT) scanograms provide an accurate method for evaluation of leg-length discrepancy. Flexion of a leg may be missed on a single frontal projection, and the measurements obtained will be erroneously shortened. When performing CT sonography, lateral views of the limbs may be easily and rapidly obtained without having to change the position of the patient. This permits assessment of limb flexion.
Humans↗
CT evaluation of pediatric hip disorders.
CT plays an important role in the management of children with congenital dislocation of the hip and torsional abnormalities of the femur. In addition, CT is also useful in evaluating children with inflammatory, neoplastic, and traumatic abnormalities of the pelvis.
Acetabulum↗
Cerebral gigantism associated with Wilms' tumor.
Two children had cerebral gigantism in unique association with Wilms' tumor. An increased incidence of neoplasms has been reported in cerebral gigantism, as well as in Beckwith-Wiedemann syndrome. We report similarities and differences between these disorders.
Beckwith-Wiedemann Syndrome↗