The proteus syndrome. Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections.

Four boys are described with partial gigantism of the hands and/or feet, pigmented nevi, hemihypertrophy, subcutaneous hamartomatous tumors and macrocephaly, and/or other skull anomalies. Three of these patients showed an accelerated growth in their first years of life. Two suffered from cystiform pulmonary abnormalities.

Brief clinical report: unilateral partial tibia defect with preaxial polydactyly, general micromelia, and trigonomacrocephaly with a note on "developmental resistance".

We report a boy with predominantly unilateral severe tibia defect with a high grade of preaxial polydactyly. Family history suggests the possibility of autosomal dominant inheritance with reduced penetrance and quite variable expressivity. The boy's phenotype and other previously reported examples of predominantly unilateral involvement in autosomal dominant and autosomal recessive limb mutations strongly suggest a hypothesis of developmental resistance in the uninvolved parts.

Bronchocentric granulomatosis with eye involvement.

Bronchocentric granulomatosis is a recently defined entity within the "pulmonary angitis and granulomatosis" group of lung diseases. Unlike the other granulomatoses, extrapulmonary disease has not been encountered with bronchocentric granulomatosis. Therefore, we report a case demonstrating apparent association with scleritis, an uncommon eye disorder often accompanying underlying systemic disease.

Structural and immunological characterization of type IV collagen isolated from chicken tissues.

Previously, a type IV collagen fraction was isolated from chicken gizzard and further fractionated into three components called F1, F2 and F3 [Mayne, R. and Zettergren, J.G.

Isolation and characterization of new collagens from chick cartilage.

Three unique collagen chains were isolated from chick sternal cartilage following pepsin solubilization of total cartilage collagens and removal of the predominant type II collagen by fractional salt precipitation. Native molecules containing 1 alpha, 2 alpha and 3 alpha chains precipitated between 0.7 M and 1.

Characterization of a highly soluble collagenous molecule isolated from chicken hyaline cartilage.

Recently, we have isolated a new collagenous molecule from chicken hyaline cartilage after limited pepsin digestion. This molecule, which contains interchain disulfide bonds, has been called the high molecular weight fraction or HMW [Reese, C. A.

Shape and assembly of type IV procollagen obtained from cell culture.

Type IV procollagen was isolated from the culture medium of the teratocarcinoma cell line PYS-2 by affinity chromatography on heparin-Sepharose. Immunological studies showed that type IV procollagen is composed of pro-alpha 1(IV) and pro-alpha 2(IV) chains and contains two potential cross-linking sites which are located in the short triple-helical 7S domain and the globular domain NC1 . The 7S domain was also identified as the heparin binding site.

A network model for the organization of type IV collagen molecules in basement membranes.

Type IV collagen was solubilized from a tumor basement membrane either by acid extraction or by limited digestion with pepsin. The two forms were similar in composition and the size of the constituent chains but differed when examined by electron microscopy and in the fragment pattern produced by bacterial collagenase. The acid-soluble form showed after rotary shadowing strands mainly of a length of 320 nm which terminated in a globule, or two strands connected by a similar globule.

Further delineation of the C (trigonocephaly) syndrome.

This communication brings the number of recognized cases of the C (trigonocephaly) syndrome to 11. The pattern of findings includes an anomaly of the anterior cranium and frontal cortex (trigonocephaly), the root of the nose (broad nasal bridge, epicanthus, and short nose), and palate (thick anterior alveolar ridges); abnormalities of the limbs (polysyndactyly, bridged palmar creases, short limbs, and joint dislocations and/or contractures); visceral defects (congenital heart defects, cryptorchidism, and abnormal lobulations of the lungs and kidneys). Auricular, mandibular, skin, and genital abnormalities also occur.

Clinical aspects of Down's syndrome from infancy to adult life.

Clinical pictures of mongoloid persons are demonstrated during different phases of life, with particular reference to the later one. Psychological and intellectual problems, immunologic deficiencies, and early aging are discussed. Histopathologic changes in the thymus are demonstrated.