phenylephrine-hydrochloride and Albinism

Nasal polyposis is a very common and multifactorial disease. Whereas eosinophil-dominated polyps often are sensitive to anti-inflammatory treatment like corticosteroids, the therapy of polyps without eosinophils is more difficult and disappointing. We report the clinical course of a 29-year-old albino patient suffering from a extreme manifestation of Woakes' syndrome, which is characterized by severe recurrent nasal polyps, often without eosinophils on histological examination and with broadening of the nose. In this case, the recurrent fibrotic polyps without eosinophils were resistant to conventional medical and surgical treatment and required further treatment with radiotherapy with awareness of all possible future sequelae. The pathoetiology and treatment of Woakes' syndrome as well as of albinism were discussed.

Topics: Adult; Albinism; Black People; Ethmoid Sinus; Ethmoid Sinusitis; Humans; Hypertelorism; Male; Nasal Polyps; Nose; Nose Deformities, Acquired; Recurrence; Reoperation; Syndrome; Tomography, X-Ray Computed

A series of experiments investigated early pleiotropic effects of a gene for imperfect albinism (s(al-c) in a population of chickens at Jouy-en-Josas, France. An elevated incidence of lesions of the navel, hocks, and nares typical of imperfect albinos were seen on these chicks, confirming their existence in this population. Variations in hatching environment and the amount of light in the hatcher both implicated in contributing to the occurrence of lesions of the hocks and nares, but not to those of the naval. In two experiments, using batteries and cages, early growth was reduced among albinos and early mortality was increased. Significant genotype by environment interactions for weight at 4 days and growth to this time, but not thereafter, suggested that the effect is restricted to this time. Unfavorable environments were most deleterious to albinos. In a third experiment, conducted in floor pens, the gene had no effect on either early growth or mortality. There was no effect of the gene on the lesions, early growth, or mortality when carried by heterozygous males (s+/s(al-c)).

Topics: Albinism; Animals; Chickens; Female; Genotype; Male; Nose; Poultry Diseases; Tarsus, Animal; Umbilicus; Weight Gain

Topics: Abnormalities, Multiple; Albinism; Child; Child, Preschool; Deafness; Diagnosis, Differential; Eye Abnormalities; Eye Color; Eyebrows; Female; Hair Color; Humans; Infant; Infant, Newborn; Nose; Waardenburg Syndrome

A family had the following manifestations of Waardenburg's syndrome (WS): prominent nasal root, white forelock, premature graying of the hair, freckled pigmentation of pale skin, hypoplastic heterochromia irides, heterochromia of the ocular fundi, congenital sensorineural hearing loss, and autosomal dominant heredity. This family differs from those previously reported in that none of its members showed dystopia of the inner canthi or lower puncta. In addition, four siblings had the combination of hyperopia-estropia-amblyopia, as well as ocular albinism, manifested by foveal hypoplasia and transilluminable irides. Observations on this family support prior suggestions of heterogeneity in WS.

Topics: Abnormalities, Multiple; Adolescent; Adult; Albinism; Child; Child, Preschool; Deafness; Female; Genes, Dominant; Hair Color; Humans; Infant; Infant, Newborn; Iris; Male; Middle Aged; Nose; Pedigree; Retinal Diseases; Skin Pigmentation; Waardenburg Syndrome

Topics: Africa, Western; Albinism; Animals; Cleft Lip; Female; Hominidae; Male; Nose; Uganda

Topics: Albinism; Bone Diseases; Deafness; Genetics, Medical; Hearing Loss; Humans; Iris; Nose; Nose Deformities, Acquired; Waardenburg Syndrome