Hepatitis A virus vaccination in childhood-onset systemic lupus erythematosus.

OBJECTIVES: Vaccination of systemic lupus erythematosus patients with non-live vaccines may decrease vaccine-preventable infections and mortalities. In the present study, we aimed to compare the immunogenicity and safety of inactivated hepatitis A vaccination in childhood-onset systemic lupus erythematosus and healthy subjects. METHODS: A total of 30 childhood-onset systemic lupus erythematosus and 39 healthy participants who were seronegative for hepatitis A received two doses of the hepatitis A vaccine in a 0- and 6-month schedule. Hepatitis A virus (HAV) IgG antibodies were measured before vaccination and 7 months after the vaccination. RESULTS: Although anti-HAV IgG antibody titers after vaccination were found to be somewhat lower in children with systemic lupus erythematosus than that of the healthy subjects ( p < 0.05), the difference in seroconversion rate was insignificant between childhood-onset systemic lupus erythematosus patients ( n = 24/30, 80%) and healthy controls ( n = 33/39, 84.6%). There was no increase in median Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2K scores and anti-ds DNA levels after the vaccination procedure. Seroconversion rates in childhood-onset systemic lupus erythematosus patients were not affected by medication, high disease activity (SLEDAI-2K >6) and anti-ds DNA positivity. None of the patients experienced any flare or adverse reaction throughout the study. CONCLUSIONS: According to these results, we conclude that inactivated hepatitis A vaccine is safe and well tolerated in childhood-onset systemic lupus erythematosus patients, with no adverse events or increase in activity. Immunogenicity to the hepatitis A vaccine was adequate, with a seropositivity rate of 80%.

Congenital Agranulocytosis (Kostmann's Syndrome) and G-CSF Therapy in an Infant.

Congenital agranulocytosis (Kostmann's Syndrome) is a rare autosomal recessive inherited disorder characterised by severe neutropenia, recurrent infections, and death in early life, with the bone marrow showing a maturation arrenst of myeloid cells at the myelocyte stage. The treatment of Kostmann's Syndrome with G-CSF results in rapid improvement. However, a few unexpected results with the use of G-CSF, were reported. Here, we describe a 7-month-old female with Kostmann's Syndrome who had recurrent skin infections and a large pyogenic infection in the supravulvar region. The patient was treated with G-CSF successfully at low doses. The infectious process and the quality of lite of the patient improved. There was no adverse effect due to the dosage and the duration. Currently bone marrow transplantation is the best way to treat Kostmann's Syndrome. Nevertheless, our experience showed that G-CSF treatment in Kostmann's syndrome was highly effective and successfull on a short term basis.