Early versus later presentations of venous malformations: where and why?

Venous malformations (VMs) are congenital anomalies of the venous vasculature, but not all are evident at birth. The factors that lead to presentation later in life are not well understood. The objective of this retrospective cohort study of patients with VMs evaluated at the University of California at San Francisco Birthmarks and Vascular Anomalies Center from 2005 to 2009 was to investigate the clinical presentation of VMs and correlate these features with different types of tissues (e.g., skin, subcutis, intramuscular). Main outcomes included the age at which lesions were first noticed, tissue type involved, presenting signs and symptoms, aggravating factors, and morbidities. A total of 115 subjects was included. The mean age when VM was first noted was 6.7 ± 0.9 years. Tissue types involved included skin/subcutaneous (46%); intramuscular (40%); and bone, tendon, or joint (14%). Presenting signs/symptoms included soft tissue swelling (44%), discrete mass (34%), pain (33%), and skin discoloration (26%). When compared with VMs limited to the skin or subcutis, those restricted to the intramuscular compartment were less likely to present at birth (27% vs 53%, p < 0.05) but were more frequently painful (79% vs 60%, p < 0.05) and contained more phleboliths (28% vs 11%, p < 0.05), and were associated with more exercise limitation (35% vs 16%, p < 0.05). VMs differ in age of onset, clinical features, and complications based on differing tissues and sites of involvement, with isolated intramuscular involvement associated with later presentation and greater morbidity.

Trends in pediatric psoriasis outpatient health care delivery in the United States.

OBJECTIVE: To characterize patterns of childhood psoriasis health care delivery from 1979-2007. DESIGN: Retrospective, cross-sectional investigation using National Ambulatory Medical Care Survey data. SETTING: US ambulatory physician offices from 1979 through 2007. PATIENTS: Children with psoriasis ages 0 (birth) to 18 years. MAIN OUTCOME MEASURES: Demographics, physician specialty, and medications prescribed. RESULTS: There were an estimated 3.8 million visits for psoriasis over the study interval with a median of 123,420 visits per year. Dermatologists saw 63% of patients, pediatricians saw 17%, and internists, 14%. The numbers of visits were equal between sexes but ranged by age group: patients ages 13 to 18 years accounted for 47% of visits, those ages 8 to 12 years for 35%, and those ages 0 to 7 for 18%. Ninety-three percent of patients were white. Topical corticosteroids were the most commonly prescribed medications. Children 0 to 9 years old received equally potent corticosteroids as children 10 to 18 years old. Among all patients, the most prescribed medication was topical betamethasone; among those ages 0 to 9 years, tacrolimus; and among those ages 10 to 18 years, betamethasone. By physician specialty, the most prescribed medications were high-potency steroids for dermatologists and internists, and topical tacrolimus for pediatricians. Topical calcineurin inhibitors were not among the top 20 most prescribed medications by dermatologists, and systemic antipsoriatic agents were not among the top 20 most prescribed medications in any age group. CONCLUSIONS: Over the 28-year interval, outpatient visits for pediatric psoriasis were attended primarily by white children older than 8 years in equal number by sex. Dermatologists and pediatricians saw the majority, and treatment approach differed by physician specialty and patient age. Treatment guidelines for childhood psoriasis may help reduce treatment variability.

The DotL protein, a member of the TraG-coupling protein family, is essential for Viability of Legionella pneumophila strain Lp02.

Legionella pneumophila is able to survive inside phagocytic cells by an internalization route that bypasses fusion of the nascent phagosome with the endocytic pathway to allow formation of a replicative phagosome. The dot/icm genes, a major virulence system of L. pneumophila, encode a type IVB secretion system that is required for intracellular growth. One Dot protein, DotL, has sequence similarity to type IV secretion system coupling proteins (T4CPs). In other systems, coupling proteins are not required for viability of the organism. Here we report the first example of a strain, L. pneumophila Lp02, in which a putative T4CP is essential for viability of the organism on bacteriological media. This result is particularly surprising since the majority of the dot/icm genes in Lp02 are dispensable for growth outside of a host cell, a condition that does not require a functional Dot/Icm secretion complex. We were able to isolate suppressors of the Delta dotL lethality and found that many contained mutations in other components of the Dot/Icm secretion system. A systematic analysis of dot/icm deletion mutants revealed that the majority of them (20 of 26) suppressed the lethality phenotype, indicating a partially assembled secretion system may be the source of Delta dotL toxicity in the wild-type strain. These results are consistent with a model in which the DotL protein plays a role in regulating the activity of the L. pneumophila type IV secretion apparatus.