BACKGROUND Hyaline body myopathy (HBM) is a rare chronic nonprogressive congenital myopathy, with variable patterns of inheritance. METHODS We describe a patient with congenital HBM with progression of weakness and increasing muscle pain in adulthood. Three muscle biopsies, done at various times in her life, are reported. RESULTS Symptoms started during childhood; however, as an adult, foll...

HYALINE or glassy ridge formation on the posterior corneal surface appears to be a not uncommon phenomenon, the recognition of which may be missed without careful examination with the slit-lamp microscope. Normally, Descemet's membrane is secreted as a hyaline tissue by the endothelial corneal cells during intra-uterine life; the exact point of development at which it appears is not definitely ...

Treatment of the eggs of the sea urchin with a 1 M solution of glycerol at fertilization allows the recovery from this solution of the protein released from the cortical granules, including that which would normally give rise to the hyaline layer. The calcium-gelable protein previously extracted from whole eggs and from isolated cortical material was found to be present in the glycerol solution...

During the latter part of pregnancy, the foetal rat produces large quantities of histamine which may be concerned with the growth of the foetus (Kahlson and Rosengren, 1959). Kahlson (1960) reported increased formation of histamine by the human foetus as well: recently Davis (1961) suggested that the antagonism of such excess histamine might account for the beneficial effect of an antihistamine...

The term "hyaline degeneration ", first employed by von Recklinghausen (1883), denotes a physical state rather than a chemical composition. " Hyalin" includes a heterogenous group of substances which share the properties of being translucent, refractile, and structureless (Gk, hyalos-glass). They stain readily with acid dyes, are resistant to strong acids and alkalis, and are all of a protein n...

of lungs obtained at necropsy from 44 children at or above the 50th centile dying from non-pulmonary causes were described. In the present paper some results from the analysis of 92 further pressure-volume curves are described. These were compared with the theoretically normal values according to total body length, by dividing the observed value by the theoretically normal one. This was done fo...

Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as "infantile systemic hyalinosis" and "juvenile hyaline fibromatosis". The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary appro...

Hyaline membrane disease of the newborn has been a subject of ever-growing interest to paediatricians and pathologists as it is the major cause of death in newborn babies, particularly premature ones. Much more is known of the clinical picture and physiological changes in hyaline membrane disease, or respiratory distress syndrome of the newborn (Avery, 1962; Curtis, 1957; James, 1959; Miller, 1...

BACKGROUND Cytologic diagnosis of pulmonary adenoid cystic carcinoma (AdCC) is frequently challenging and differential diagnosis with small cell carcinoma is often difficult. METHODS Eleven cytologically diagnosed cases of pulmonary AdCC were collected and reviewed according to fifteen cytomorphologic characteristics: small cell size, cellular uniformity, coarse chromatin, hyperchromasia, dis...

Despite numerous attempts to develop and evalute specsc methods for the histochemical demonstration of mucopolysaccharides, definitive information is not readily available. Thls is in spite of the notable contributions of Hale ('46); Steedman ('50); Gomori ('50); Kramer and Windrum ('54); Mowry ('56, '58); Spicer ('60); Belanger and Migicovsky ('61) and Goldstein ('62) to mention only a few. Wi...