Vascular birthmarks in children.

Several different systems of terminology for vascular birthmarks are defined using descriptive terms (capillary hemangioma, cavernous hemangioma, mixed hemangiomas, etc.) for consistency. Capillary hemangiomas have the most complete and rapid rate of resolution whereas cavernous hemangiomas are considerably slower to respond. Vascular lesions in the V1 distribution of the trigeminal nerve and in the lumbosacral area need imaging studies for presence of internal involvement. Vascular lesions that require systemic therapy are those that obstruct vision, airway, or genitourinary tract; and produce congestive heart failure or consumption of formed blood products. The various modalities of treatment including steroids, are discussed briefly.

Pediatric dermatology: advances in therapy.

Several recent therapeutic advances in pediatric dermatology have been made. Of particular importance are new developments in the use of antimicrobials, antivirals, antifungals, retinoids, calcipotriene, and intravenous gamma globulin. We review safety and efficacy data of these drugs in their use in children with cutaneous disease.

Mass spectrometric quantification of markers for protein oxidation by tyrosyl radical, copper, and hydroxyl radical in low density lipoprotein isolated from human atherosclerotic plaques.

Lipoprotein oxidation has been implicated in the pathogenesis of atherosclerosis. However, the physiologically relevant pathways mediating oxidative damage have not yet been identified. Three potential mechanisms are tyrosyl radical, hydroxyl radical, and redox active metal ions. Tyrosyl radical forms o,o'-dityrosine cross-links in proteins. The highly reactive hydroxyl radical oxidizes phenylalanine residues to o-tyrosine and m-tyrosine. Metal ions oxidize low density lipoprotein (LDL) by poorly understood pathways. To explore the involvement of tyrosyl radical, hydroxyl radical, and metal ions in atherosclerosis, we developed a highly sensitive and quantitative method for measuring levels of o, o'-dityrosine, o-tyrosine, and m-tyrosine in proteins, lipoproteins, and tissue, using stable isotope dilution gas chromatography-mass spectrometry. We showed that o,o'-dityrosine was selectively produced in LDL oxidized with tyrosyl radical. Both o-tyrosine and o, o'-dityrosine were major products when LDL was oxidized with hydroxyl radical. Only o-tyrosine was formed in LDL oxidized with copper. Similar profiles of oxidation products were observed in bovine serum albumin oxidized with the three different systems. Applying these findings to LDL isolated from human atherosclerotic lesions, we detected a 100-fold increase in o,o'-dityrosine levels compared to those in circulating LDL. In striking contrast, levels of o-tyrosine and m-tyrosine were not elevated in LDL isolated from atherosclerotic tissue. Analysis of fatty streaks revealed a similar pattern of oxidation products; compared with normal aortic tissue, there was a selective increase in o,o'-dityrosine with no change in o-tyrosine. The detection of a selective increase of o,o'-dityrosine in LDL isolated from vascular lesions is consistent with the hypothesis that oxidative damage in human atherosclerosis is mediated in part by tyrosyl radical. In contrast, these observations do not support a role for free metal ions as catalysts of LDL oxidation in the artery wall.

Erythema multiforme, Stevens-Johnson Syndrome, and toxic epidermal necrolysis.

The protean manifestations of erythema multiforme range from minor variants through Stevens-Johnson Syndrome, bullous erythema multiforme, and toxic epidermal necrolysis. Herpes simplex virus is a common etiologic agent for minor varieties particularly if they are recurrent. Certain drugs also cause severe varieties of erythema multiforme. Although no form of therapy is recognized as generally effective, many are used in treating this group of diseases. Local wound care is also important.

Trichothiodystrophy associated with photosensitivity, gonadal failure, and striking osteosclerosis.

Three new unrelated cases of PIBIDS (Photosensitivity, Ichthyosis, Brittle, sulfur-deficient hair [trichothiodystrophy], Impaired intelligence, Decreased fertility, and Short stature) are reported. Decreased survival of skin fibroblast lines after UVB exposure was found. All three male patients had hypogonadism and primary end-organ gonadal failure. Striking osteosclerosis was present in all three patients. To the best of our knowledge the third patient is the first reported case of a black man with PIBIDS.

Semantic analysis of medical records.

A program (LogStory) is described that was developed for the automatic semantic analysis of clinical narratives, stored in a computerized problem-oriented medical record (PROMED). The diagnoses were written in a free-text format during consultation, and later collected into diagnostic classes, e.g., diseases. A lexical parser automatically created dictionaries from the clinical narrative associated with each disease. Automatic (fuzzy) set operations were performed on the words associated with each class. The manifestations of 16 diseases were automatically extracted by pairwise operations on the word sets. The correlation between diseases and corresponding signs, symptoms and treatment was highly significant (p < 0.001). Applying the difference operation on diseases with disjunct sets of clinical findings allowed the recovery of disease-specific knowledge. The evolution of a disease was accounted for, and the system was able to generalize its findings. The PROMED-LogStory concept enables the processing of natural language and may be a powerful tool for knowledge acquisition and clinical research.

An unusual presentation of fixed cutaneous sporotrichosis: a case report and review of the literature.

Sporotrichosis is extremely rare in infants. This report describes an 84-day-old girl with fixed cutaneous sporotrichosis, presumably transmitted by a cat. A rapid, complete response to a low dose of oral potassium iodide therapy was attained. To our knowledge, this is the youngest reported patient with sporotrichosis, as well as the lowest effective daily dose of potassium iodide.