A Case of a Thoracic Spinal Intradural Arachnoid Cyst with Chest Pain / 대한소아신경학회지

Spinal arachnoid cysts are relatively uncommon lesions that may be either intradural or extradural, while intradural spinal arachnoid cysts are even less common. The cysts are filled with clear, and colorless fluid nearly identical to cerebrospinal fluid. The origin of these cysts remains unclear although congenital, traumatic, and inflammatory causes have been postulated. These cysts are usually asymptomatic but may produce symptoms by compressing the spinal cord or nerve roots suddenly or progressively. The treatment of choice is surgical excision or fenestration, which is indicated when there are features of cord compression. We report a case of a thoracic spinal intradural arachnoid cyst in 13-year-old girl with chest pain.

A Case of Klippel-Trenaunay-Weber Syndrome with Facial Hemihypertrophy and Hemimegalencephaly / 대한소아신경학회지

Klippel-Trenaunay-Weber syndrome(KTWS) is a rare, and sporadically occurring disorder characterized by hemihypertrophy(unilateral limb hypertrophy), varicose veins, hemangiomas and occasionally arteriovenous malformations. In 1900, noted French physicians Klippel and Trenaunay first described the syndrome in 2 patients presenting with port-wine stains and varicosities of extremities associated with hypertrophy of the affected limb's bones and soft tissues. There are other, and less frequent abnormalities. These may include limbs that are atrophic, fingers and toes that are disproportionately large or small, digits that are webbed(syndactyly), and too many digits(polydactyly), or too few digits(oligodactyly). The hemangiomas can occur in internal organs including the intestinal and the urinary tract systems. The exact cause of KTWS remains to be elucidated, although several theories exist. Most cases are sporadic, although a few cases in the literature report a multifactorial patterns of inheritance. We report a case of Klippel-Trenaunay-Weber syndrome in a 3-year-old boy who had hypertrophy of the left facial bones and tissues, large hemangiomas on the right trunk and back, and hypertrophy of the right upper and lower limb's soft tissues.

Survey on the Association of the Number of BCG Scars and Atopic Diseases / 소아알레르기및호흡기학회지

PURPOSE: The prevalence of atopic diseases has been increasing remarkably. The less frequent opportunities for infection early in life, especially mycobacteria exposure, parallel this higher prevalence of atopic diseases. Bacille Calmette-Gu rin (BCG), a potent inducer of Th1 immune response, has been suggested to suppress Th2 response which is known to mediate IgE-mediated atopic disorders. This study was done to investigate whether there is any relation between the number of BCG scars and the prevalence of atopic disorders in early childhood. METHODS: We surveyed 393 parents with a children who were given percutaneous multi- puncture BCG vaccination within four weeks after birth. The main questions concerned the past history and present illness of physician-diagnosed atopic diseases (atopic dermatitis, bronchial asthma, and allergic rhinitis), the number of BCG scars (range; 0-18), and potential confounders such as gender, parental atopy, maternal smoking and environmental cofactors. The prevalence rate of each atopic disease was measured and analysed according to the number of BCG scars. RESULTS: Each prevalence rate was 18.1% for atopic dermatitis, 9.4% for bronchial asthma, 14.6% for allergic rhinitis, and 32.3% for any of them. All of them had received BCG vaccination during the first four weeks of life. The children with 15 or more BCG scars had a significantly lower prevalence of any atopic disease (22/99, 22.2%) as compared to those with four scars or less (51/125, 40.8%) by simple regression analysis. (P value=0.02) But this association was not significant after controlling for potential confounders. (P value= 0.26) CONCLUSION: This survey demonstrated a weak relation between a larger number of BCG scars and less atopy development at early childhood. But the relation was not so significant. Further studies are needed.