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Biomedical subjects

J W Mali

Publications and source records attributed to J W Mali.

10 recordsLinked to original sources
PubMed1978

[Ichthyosis].

Explore the source record for details and available documents.

Adult↗
PubMed1977

Deafness, ichthyosiform erythroderma, corneal involvement, photophobia and dental dysplasia.

Early childhood deafness, congenital non-bullous ichthyosiform erythroderma, corneal involvement, photophobia, chronic blepharoconjunctivitis, hypotrichosis, anhidrosis, hyperkeratosis of the nails and dental dysplasia were the symptoms observed in the 19-year-old girl described in this paper. The literature comprises reports on 8 patients with this syndrome, which McKusick (1975) listed as no. 24215.

Adolescent↗
PubMed1976

A lysosomal storage disorder of the epidermis characterized by a deficiency of alpha-mannosidase and an accumulation of mannose-rich materials.

Laboratory investigation of a patient diagnosed as ichthyosiform erythroderma bullosa revealed the following abnormalities: (1) alpha- and beta-galactosidases were elevated in the lesion. (2) alpha-mannosidase activity was extremely low both in the lesion and in relatively normal epidermis. (3) The mannose:glucose ratio of water-soluble hexose-containing material in the scales was very high. (4) Membrane-limited vacuoles, apparently enlarged secondary lysosomes, were observed. We conclude that this patients is the first documented example of a lysosomal storage disease in which overt clinical lesions are confined to the epidermis.

Adult↗
PubMed1976

An evaluation of betamethasone dipropionate (Diprosone) versus Locacorten 0.02% cream.

A double-blind randomized evaluation of betamethasone dipropionate cream (Diprosone) compared with Locacorten 0.02% cream flumethason privalate in 60 patients over 3 consecutive weeks showed the betamethasone dipropionate cream to be significantly more effective in the treatment of psoriasis and other steroid-responsive dermatoses.

Administration, Topical↗