Psoriasis and normouricemic gout.
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Biomedical subjects
Publications and source records attributed to L M Solomon.
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Cutaneous metabolism and pharmacology have been the focus of increased scientific inquiry in the past 2 decades. However, in the past few years, attention has been focused specifically on the effects of topically applied drugs in infants as different qualitatively or quantitatively from their effects in adults. Prior to 1972, it was known that brain damage occurred in animals with prolonged blood levels of 2 microgram/ml hexachlorophene, and that washing newborn babies with a standard 3% hexachlorophene liquid soap for 3-5 days resulted in significant blood levels of the compound. However, this knowledge was not disseminated widely enough to prevent the tragic deaths of infants after the use of baby powder contaminated with 6.6% hexachlorophene [1]. This incident highlighted the need for increased understanding of drug effects not only from the viewpoint of the skin as a target organ, but also of percutaneous penetration and resultant blood levels; the affinity of other body tissues for drugs and their metabolites, metabolites which may result from the effect of the skin itself acting on the drug; and the infant's much greater ratio of surface area to body weight, allowing the infant to percutaneously absorb proportionately greater quantities of topical medication than an adult. Although tissue distribution of most drugs has not been studied in infants, it is known that such distribution often depends on age. For example, in infants and children with a given plasma level, of drugs such as barbiturates, morphine and tetracycline, the brain tissue level may exceed that of the adult. Thus, drugs and chemicals that penetrate infant skin may produce effects different than those penetrating adult skin.
The ectodermal dysplasias are a heterogeneous group of disorders that, in the past, has included conditions best classified as progeroid disorders. The inaccuracy of the terminology has led to a proliferation of syndromes in which the patients are said to have poorly defined "ectodermal dysplasia," and a real need exists to define that appellation further. We suggest that the term "ectodermal dysplasia" be limited to those disorders that are congenital, are diffusely present, are not progressive, and do involve the epidermis and at least one of the appendages. We recognize that a heterogeneous group of disorders remains that generically have certain similarities. Not enough is known about the defects in each of the elements of the skin affected in these conditions to classify them more accurately. Several recently described disorders appear to have some degree of ectodermal dysplasia.
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Because bradykinin constitutes a possible candidate for mediation of topical 8-methoxypsoralen-UVA-(PUVA)-induced erythema, aprotinin (Trasylol), inhibitor of kallikrein and interrupter of the cascade leading to kinin production was assessed in guinea pigs. Response was assessed at 24, 48, and 72 hr after topical PUVA and there was no significant difference between normal saline and aprotinin by intradermal or intraperitoneal routes of administration. The results of this study indicate that intradermal and intraperiotoneal aprotinin, in the dose and method tested, is not capable of significantly decreasing erythema induced by topical PUVA in guinea pigs.
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A 5-year-old boy was found to have severe rickets in association with hyperpigmented, linear, verrucous, epidermal tumors, typical of the epidermal nevus syndrome. Normocalcemia (9.6 mg/dl), hypophosphatemia (2.0 mg/dl), elevated serum alkaline phosphatase concentration (313 IU), decreased renal tubular reabsorption of phosphorus (35%), radiologic evidence of rickets, and lack of response to usual therapeutic doses of vitamin D suggested hypophosphatemic vitamin D-resistant rickets. Therapy with vitamin D in doses to 750,000 IU and oral phosphate, 2.0 gm/day, failed to induce healing of the rickets. A subtotal parathyroidectomy performed when the patient was 9 years old was also without effect. When he was 12 years old several fibroangiomas on the face and left lower limb were excised. Within three months all biochemical abnormalities resolved and radiologic evidence of healing was observed. A portion of excised tissue was homogenized and injection of the supernate into a 6-week-old puppy induced excessive phosphaturia. The data suggest that the rickets was induced by a phosphaturic substance extractable from the tumors.
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Blood and brain levels of gamma benzene hexachloride had been measured following topical application in guinea pigs. Levels increased with number of application, and the concentration in brain was at least 10 times greater than in blood.
A family with the tricho-rhino-phalangeal syndrome was studied. One child, the proband, manifested the complete triad of the syndrome with fine, sparse, slow-growing hair, pear-shaped nose, and abnormal fingers. Cone-shaped epiphyses were observed radiographically in the child, in both parents, and in two of three remaining children.
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