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Biomedical subjects

A K Poznanski

Publications and source records attributed to A K Poznanski.

At least 73 records · Page 4Linked to original sources
PubMed1984

The Jansen type of metaphyseal chondrodysplasia: confirmation of dominant inheritance and review of radiographic manifestations in the newborn and adult.

We describe a mother and infant girl with the Jansen type of metaphyseal chondrodysplasia. The transmission of the syndrome from mother to daughter in this family establishes this as a dominant condition. Characteristic radiographic manifestations of the disorder were obvious at birth. The mother and infant illustrate the two extremes of age where the radiographic manifestations are less characteristic than in middle childhood, since the marked metaphyseal calcifications are absent.

Adult↗
PubMed1984

Calcification associated with terminal defects of the upper extremity.

Four children who had calcifications associated with terminal defects of the upper extremity are reported. A review of the literature has revealed no previous report. Moreover, an examination of the radiographs of 65 children who had terminal defects of the upper limb failed to show any such calcifications. The exact cause of this association remains unknown.

Arm↗
PubMed1983

Radiological findings in the hand in Seckel syndrome (bird-headed dwarfism).

Two patients with severe intrauterine growth retardation and bird-headed appearance are described. These two children had most of the clinical features of the so-called Seckel dwarfism. The radiological findings included: (1) ivory epiphyses affecting all phalanges in one patient and many phalanges in another; (2) cone-shaped epiphyses in the proximal phalanges; (3) marked disharmonic bone maturation between carpals and phalanges, between individual carpals, and from side to side; (4) alteration in the length of the hand bones, with considerable similarity of the metacarpophalangeal pattern between the two children; (5) relatively small carpals, which have an angular configuration; and (6) relatively normal or increased cortical thickness of the metacarpals.

Age Determination by Skeleton↗
PubMed1982

Injuries of the distal tibial epiphysis: systematic radiographic evaluation.

The site of injury to the distal tibial epiphysis in the younger individual depends on the relation of the ligaments to the epiphysis, the type of injury, and the maturity and degree of closure of the growth plate. A characteristic radiographic pattern of injury occurs as a result of each particular injuring force, which in turn requires a specific mode of fracture reduction. One hundred and ninety-four cases of epiphyseal injuries were reviewed, and the incidence and diagnostic characteristics of the various types are presented. The Salter-Harris classification of lesions reveal the type II is the most common (46.4%), followed by type III (25.2%), type IV (10.3%), and type I (5.7%). The triplane fracture constituted 9.8%, and miscellaneous injuries, which could not be classified, were 2.6%.

Adolescent↗
PubMed1981

Mannosidosis: two brothers with different degrees of disease severity.

Two siblings with different degrees of mental retardation, skeletal dysplasia, coarse facies, delayed speech, motor incoordination, recurrent respiratory infections, and immunological abnormalities, were found to have deficient alpha-mannosidase activity. Cultured skin fibroblasts in one sib were markedly deficient in alpha-mannosidase while all other lysosomal enzymes tested were within the normal range. The more severely affected sib came to autopsy and was found to have "washed-out" appearing cortical neurons and marked histiocytosis effacing lymph node architecture and partially replacing the bone marrow. The post-mortem brain and liver samples demonstrated a deficiency in alpha-mannosidase relative to the elevations of other lysosomal enzymes. Although the patterns of abnormalities in the two cases closely match those of descriptions of "type II" and "type I" mannosidosis respectively, the variation should be due to genetic modifiers or environmental effects since the brothers must have shared similar alpha-mannosidase mutations. Immunologic abnormalities present in the more severely affected sib suggest that the differential survival seen in mannosidosis types I and II may be due to differences in their immune systems.

Abnormalities, Multiple↗
PubMed1981

Radiographic changes in unicameral bone cysts following direct injection of steroids: a report on 14 cases.

Twelve children (9 boys and 3 girls, ages 1 1/2-12 yrs.) with unicameral bone cysts were treated with triamcinolone acetonide (4 patients) or methylprednisolone acetate (8 patients) injected directly into the cyst cavity. Postinjection radiographic changes that indicate healing were noted in eight patients and included: reduced size of the cyst cavity, increased internal density, cortical thickening, and remodeling in the vicinity of the cyst. Additional injections were required in three patients because of delayed or partial healing. Recurrence of the cyst (1 case) was signaled by renewed rarefaction and bone expansion.

Bone Cysts↗
PubMed1981

CT determination of femoral torsion.

Femoral torsion has been measured in infants and children by computed tomography. The method requires two scans, one through the femoral neck, another through the femoral condyles. A specially designed device and packing about the knees assures immobilization of the legs. Slight variations in the positions of the sections in the neck and condyles do not alter the measurement significantly. Intraobserver and interobserver errors are low, 2 degrees and 3 degrees mean error, respectively. The radiation required involves small tissue volumes at about 1.7 R (4.4 x 10(-4) C/kg). Measurements are more difficult when the femoral necks are short or vertically oriented. The method appears to have sufficient accuracy for clinical purposes.

Adolescent↗
PubMed1980

Case report 112.

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Adult↗
PubMed1980

Case report 116.

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Adolescent↗
PubMed1980

Staphylococcal pelvic and rectal infection in a neonate.

An infant with neonatal staphylococcal infection had evidence of pelvic inflammation, peritonitis and proctitis which resulted in narrowing of the rectosigmoid, uterectasis, pyelocaliectasis, and partial obstruction of the inferior vena cava. Appropriate treatment resulted in resolution of these findings.

Colon↗
PubMed1980

Congenitally absent thoracic pedicle in a child with rhabdomyosarcoma.

A case of congenital absence of a thoracic pedicle is reported in a 9 year old boy with embryonal rhabdomyosarcoma of the eyelid. In this clinical setting a surgical exploration was necessary in order to differentiate a developmental anomaly from an acquired metastatic deposit.

Child↗
PubMed1980

New standards of cortical mass in the humerus of neonates: a means of evaluating bone loss in the premature infant.

The purpose of this study was to develop standards for quantitating bone loss in premature infants. Cortical measurements of the humerus, including the outside diameter (T), the inner diameter (M), the cortical thickness (C), and the cortical area (CA), were calculated for 132 newborn infants who weighed between 500 and 3,686 g and ranged in gestational age from 22 to 40 weeks. The measurements were correlated with the independent variables of gestational age and birth weight, and are presented graphically. With the method of cortical measurement described in this study, it may be possible to determine the causes of bone loss, and to find ways to correct it.

Birth Weight↗