[Structured and regular workplace-based assessment and feedback of clinical competence for residents in dermatology]. / Strukturerad återkoppling på ST-läkares kliniska färdigheter.

Workplace-based assessment of clinical competence is essential as an educational tool for residents. The residents in dermatology and venereology at Södersjukhuset, Sweden, reported lack of feedback in an evaluation 2014. Therefore, a project to enhance the use of formative assessment methods was launched in 2018. All dermatologists were educated in formative assessment methods and feedback, certain clinical skills which should be assessed during the education was established, and a requirement to perform at least six formative assessments each year of residency was introduced. After two years, all residents had participated in a median of 7 (range 3-21) formative assessments the past year and reported regular use of assessment tools for clinical skills, good access to clinical teachers, and frequent feedback.

Allogeneic Hematopoietic Stem Cell Transplantation in the Treatment of Human C1q Deficiency: The Karolinska Experience.

BACKGROUND: Human C1q deficiency is associated with systemic lupus erythematosus (SLE) and increased susceptibility to severe bacterial infections. These patients require extensive medical therapy and some develop treatment-resistant disease. Because C1q is produced by monocytes, it has been speculated that allogeneic hematopoietic stem cell transplantation (allo-HSCT) may cure this disorder. METHODS: We have so far treated 5 patients with C1q deficiency. In 3 cases, SLE symptoms remained relatively mild after the start of medical therapy, but 2 patients developed treatment-resistant SLE, and we decided to pursue treatment with allo-HSCT. For this purpose, we chose a conditioning regimen composed of treosulfan (14 g/m) and fludarabine (30 mg/m) started on day -6 and given for 3 and 5 consecutive days, respectively. Thymoglobulin was given at a cumulative dose of 8 mg/kg, and graft-versus-host disease prophylaxis was composed of cyclosporine and methotrexate. RESULTS: A 9-year-old boy and a 12-year-old girl with refractory SLE restored C1q production after allo-HSCT. This resulted in normal functional properties of the classical complement pathway followed by reduced severity of SLE symptoms. The boy developed posttransplant lymphoproliferative disease, which resolved after treatment with rituximab and donor lymphocyte infusion. Unfortunately, donor lymphocyte infusion induced severe cortisone-resistant gastrointestinal graft-versus-host disease, and the patient died from multiple organ failure 4 months after transplantation. The girl is doing well 33 months after transplantation, and clinically, all signs of SLE have resolved. CONCLUSIONS: Allo-HSCT can cure SLE in human C1q deficiency and should be considered early in subjects resistant to medical therapy.

Incidence of Kawasaki disease in northern European countries.

BACKGROUND: The aim of the present study was to compare the epidemiologic features of Kawasaki disease (KD) in three northern European countries and Japan. METHODS: Data were obtained from discharge databases for hospitals in Finland, Norway, and Sweden for 1999-2009 and from nationwide epidemiologic surveys in Japan for 1998-2008. Annual incidence for each country was calculated using regional census data. RESULTS: During the 11 year period, 1390 KD patients were recorded in the registries of the three northern European countries. Average annual incidence rates per 100,000 children aged <5 years were: Finland, 11.4; Norway, 5.4; and Sweden, 7.4. Overall, 86.4% of Japanese KD patients were aged <5 years compared to only 67.8% in the four northern European countries (P < 0.001). CONCLUSIONS: The incidence of KD in northern Europe was constant over the study period and much lower than in Japan. There was a significant age difference between northern European and Japanese KD patients that remains unexplained.