A case of Hallermann-Streiff syndrome with aphakia / 소아과

Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.

An epidemiologic study on the seropositive rate of hepatitis A virus among a selected group of children and adults in Busan / 소아과

PURPOSE: The prevalence of hepatitis A virus (HAV) in a certain community reflects that community's living standards and hygienic conditions. And the pattern of HAV infection differs over time and geography. Recently, a shift in prevalence has been observed in cases from chilhood to adulthood. We studied the HAV antibody prevalence in the general population in Busan. METHODS: From October 2004 to March 2005, total 472 subjects were tested for HAV antibodies. All samples were collected from patients in Maryknol Hospital. RESULTS: The overall seropositive rate was 22.8% (108/472). The seropositive rates were 1.7% in subjects aged 2-5 years, 1.7% in 6-10 years, 0% in 11-20 years, 40.5% in 21-30 years, 82.1% in 31- 40 years, 94.7% in 41-50 years, and 100% in subjects aged over 50 years. There was no significant gap between gender groups. CONCLUSION: As the socioeconomic conditions in Korea have improved, the HAV seropositive rate in school-aged children has dramatically decreased in the last 20 years. But, the seropositive rate of HAV didn't differ according to gender. The seropositive rate of HAV in the pediatric group was very low, which suggests the increasing possibility of clinical HAV infection in adults in the near future. Therefore, we should actively prevent the spread of hepatits A virus. In order to do that, we need to reorganize our lifestyle and personel hygiene and carry out active and passive immunization to high risk groups.

A Case of Sjogren's Syndrome with Hyperthyroidism / 대한소아내분비학회지

Sjogren syndrome is a chronic, slowly progressive, autoimmune disease in which the exocrine glands are damaged by lymphocytic infiltration, resulting in xerostomia and xerophthalmia. Sjogren syndrome may occur in 2 forms: primary Sjogren syndrome, when the clinical manifestations of the syndrome are seen alone, and secondary Sjogren syndrome, when associated with another autoimmune disease, such as rheumatoid arthritis, systemic lupus erythromatosus, or scleroderma. Approximately one third of patients present with extraglandular manifestations: arthritis, Raynaud phenomenon, lymphadenopathy, lung involvement, vasculitis and peripheral nervous system involvement. About 10-50% of patients with Sjogren syndrome had evidence of thyroid disease, mainly hypothyroidism. Several inflammatory thyroid diseases are also considered to be autoimmune in origin. In this respect, the histologic picture of primary Sjogren syndrome exocrine glands and autoimmune thyroid glands show great similarities. Here, we report a new case of Sjogren syndrome accompanying with hyperthyroidism which affected a 10-year-old girl.