A Case of Combined Acute Disseminated Encephalomyelitis and Acute Flaccid Paralysis associated with Enteroviral Infection / 대한소아신경학회지

Enterovirus infection is often aggravated and manifests as various neurological complications such as aseptic meningitis, brainstem encephalitis, poliomyelitis-like acute flaccid paralysis, transverse myelitis, and severe systemic diseases. There are a few reports indicating that enterovirus is associated with acute disseminated encephalomyelitis (ADEM), acute flaccid paralysis, or Guillain-Barre syndrome separately. However, none of these report ADEM and acute flaccid paralysis occurring simultaneously. Here, we present a case of combined ADEM and acute flaccid paralysis associated with enteroviral infection in a child and reviewed the relevant literature. A 5-year-old boy was admitted to the hospital for headache and vomiting with evident meningeal irritation signs. Despite of antibiotic therapy, his mental state rapidly declined to coma with abnormal upper motor neuron (UMN) signs. During 5 consecutive days of steroid pulse therapy, his abnormal UMN signs abruptly changed to flaccid paralysis. Suspected acute flaccid paralysis due to enterovirus prompted the addition of intravenous immunoglobulin (IVIG). On hospital day 33, he was discharged with full recovery of muscle tone and strength with an alert mental state.

Analysis of reports on orphan lung diseases in Korean children / 소아과

PURPOSE: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. METHODS: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. RESULTS: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. CONCLUSION: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.