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Biomedical subjects

J P Dorst

Publications and source records attributed to J P Dorst.

At least 37 records · Page 2Linked to original sources
PubMed1977

Acromesomelic dwarfism: manifestations in childhood.

Acromesomelic dwarfism is a distinct condition characterized by short stature of the short limb type, with the hands and feet showing the most obvious deviations from normal. The forearm bones are usually disproportionately shorter than the other long tubular bones of the limbs. The intelligence is normal. Available data suggest autosomal recessive transmission. Characteristic clinical and radiographic features permit establishment of a confident diagnosis in the first year of life.

Achondroplasia↗
PubMed1977

A postaxial polydactyly-dental-vertebral syndrome.

Three patients with postaxial polydactyly and other abnormalities of the hands and feet, hypoplasia and fusion of the vertebral bodies, and dental abnormalities are reported. Two were sisters born to normal unrelated parents; the other patient was the male offspring of a consanguineous marriage. We suggest that this constellation of abnormalities represents a recessively inherited syndrome.

Abnormalities, Multiple↗
PubMed1977

A heritable syndrome of craniosynostosis, short thin hair, dental abnormalities, and short limbs: cranioectodermal dysplasia.

Five children are reported with dolichocephaly (with sagittal suture synostosis in three), sparse, slow-growing, fine hair, epicanthal folds, hypodontia and/or microdontia, short span, brachydactyly and brachypodia, and narrow thoraces. Radiologic abnormalities were noted chiefly in the skull, chest, and limbs. Intelligence was normal. The pattern of inheritance is compatible with an autosomal recessive trait.

Abnormalities, Multiple↗
PubMed1977

Use and complications of high-dose disodium etidronate therapy in fibrodysplasia ossificans progressiva.

A boy with fibrodysplasia ossificans progressiva received nearly twice the usual therapeutic dose of disodium etidronate for 13 months in an attempt to prevent reossification following a second operation to correct severe torticollis. The operation was successful, but during therapy he developed weakness and distinctive bone lesions characterized by general osteopenia, widened physes, and unique radiolucent bands in the metaphyses. The osseous abnormalities were distinct from those of rickets and healed after withdrawal of the drug.

Child, Preschool↗
PubMed1976

Spontaneous rupture of the stomach in the newborn: clinical and experimental evaluation.

Our study presents an experimental model for studying gastric rupture in the newborn. We showed that gastric rupture could be produced in eight newborn rodents and in three puppies less than 10 days old after injection of 120 ml air at 100 mm Hg pressure in the rodents and 175 ml air at 240 mm Hg pressure in the puppies. Gastric rupture occurred with animals supine and with approximately 20 ml barium injected into the stomach. Positioning of the animal in the supine position permitted the barium to pool in the antral-pyloric region of the stomach.

Age Factors↗
PubMed1976

The syndrome of bronchial atresia or stenosis with mucocele and focal hyperinflation of the lung.

Three cases of "the syndrome of bronchial atresia or stenosis with mucocele and focal hyperinflation of the lung" are added to 28 proven cases (26 with atresia and 2 with stenosis) previously reported in the English literature. One case is of particular interest since serial roentgenograms during the first week of life showed fetal lung fluid to be trapped distal to the atresia. The radiographic findings in this syndrome are reviewed and the presumed etiology and pathophysiology discussed.

Body Fluids↗
PubMed1976

Heterogeneity in the campomelic syndromes: long and short bone varieties.

There appears to be definite heterogeneity within the campomelic syndromes. We are broadly classifying these into the classic or long bone campomelic syndromes and 2 distinct short bone varieties designated as the normocephalic short-boned and craniosynostotic short-boned types, respectively (Table 1).

Dwarfism↗
PubMed1975

The Kniest syndrome.

Explore the source record for details and available documents.

Abnormalities, Multiple↗
PubMed1975

Pulmonary edema and continuous positive pressure breathing (CPPB).

We have successfully treated severe pulmonary edema of various etiologies and the concomitant hypoxia in 17 infants and children with continuous positive pressure spontaneous breathing. The pressure used was 8 to 14 cm of water above atmospheric pressure. No significant changes occurred in arterial or central venous blood pressure, and no patient developed clinical evidence of peripheral venous engorgement. Pulmonary edema cleared within 24 hours in all cases. We believe this rapid clearing may differentiate pulmonary edema from pneumonia and pulmonary hemorrhage. The pathophysiology and the roentgenologic findings are presented.

Child, Preschool↗
PubMed1975

New syndrome of skeletal, dental and hair anomalies.

A Brother and sister are presented with the following abnormalities: dolichocephaly with tendency toward premature closure of the sagittal suture; antimongoloid slant of the eyes with epicanthal folds, full cheeks, everted lip, multiple oral frenula, mildly high-arched palate, and microdontia with possible enamel defect; posteriorly rotated low-set pinnae with deficient cartilage; a small short thorax with pectus excavatum; unusual dermatoglyphics, and abnormal hair growth and stucture; disproportionate shortening of the fibulae and the middle and distal phalanges of the toes and fingers; and somewhat flattened epiphyses. A paternal aunt gave birth to a stillborn dwarfed male with some of the same clinical features. Although an autopsy was obtained, unfortunately no roentgenograms were made.

Abnormalities, Multiple↗