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.

VPDL Chemotherapy for T-cell Lymphoblastic Lymphoma (T-LBL) in Adults: Comparison with Upfront Autologous Stem Cell Transplantation in a Single Center

BACKGROUND: Treatment of T-cell lymphoblastic lymphoma (T-LBL) with CHOP or CHOP-like chemotherapy has resulted in poor long-term outcomes. High-dose chemotherapy followed by ASCT has been applied for this dreaded disease. However, the efficacy is still controversial. T-LBL is considered the nodal/extranodal presentation of acute lymphoblastic leukemia. Favorable results with VPDL chemotherapy have been reported in the setting of adult lymphoblastic leukemia. We, therefore, treated T-LBL patients with modified VPDL chemotherapy and compared the outcomes with those achieved using upfront ASCT. METHODS: We retrospectively reviewed the outcomes of 24 T-LBL patients treated either with upfront ASCT (n=11) or VPDL chemotherapy without ASCT (n=13) between January 1996 and October 2005. RESULTS: The median follow-up duration for surviving patients was 17 months (range, 5~109 months). The two-year event-free survival (EFS) rates were 83.1% in the VPDL group and 27.3% in the upfront ASCT group (P=0.008). The two-year overall survival (OS) rates were 83.9% in the VPDL group and 27.3% in the upfront ASCT group (P=0.006). CONCLUSION: This study suggests that VPDL chemotherapy is very effective and may be superior to upfront ASCT in the treatment of T-LBL patients.

Status of intestinal parasitic infections in a remote island, Yondo, Jeonranam-do(province)

This study was performed to observe the present status of intestinal parasitic infections in a remote island, Yondo, located in southern part of Jeonranam-do(province), Korea. In February and May 1988, total 1,011 individual stool samples were collected and examined for intestinal helminths and protozoa using formalin-ether centrifugal sedimentation technique. The results are summarized as follows: Of 1,011 ingabitants examined, 398(39.4%) were positive for intestinal parasites. Helminth positives were 372(36.8%), and protozoan cyst positives were 56(5.5%). Ten species of parasites were found. Trichuris trichiura revealed the highest infection rate of 27.5%, Ascaris lumbricoides 17.4%, Taenia sp. 5.8%, Entamoeba coli 3.3%, Giardia lamblia 1.5%, Endolimax nana 0.8%, Hymenolepis nana 0.4%, hookworm 0.2%, Trichostrongylus orientalis 0.2%, and Entamoeba histolytica 0.2%, respectively. The female group showed higher positive rate (44.0%) than males (34.7%). Also, higher positive rates were observed among adults as compared with the group younger than 10 years old. Average value of E.P.G. was 1,876(range 200-17,800) in A. lumbricoides positives, and 327(range 200-1,600) in T. trichiura positive cases. In helminth egg positive cases, single infection was 63.4%, double infection 34.7%, and triple infection 1.9%, respectively. Among protozoan cyst positives, single infection was 94.6%, and double infection was 5.4%. The present study revealed that the prevalence of intestinal parasites among inhabitants in Yondo island is still so high that special control measures should be performed.

Detection of IgG antibodies with immunofluorescent antibody technique in human trichomoniasis

The indirect fluorescent antibody(IFA) test was used to detect serum IgG and IgM antibodies to Trichomonas vaginalis in 31 vaginal trichomoniasis, 7 candidiasis and in 20 non-infected healthy wonem with antigen prepared from axenic culture of Trichomonas vaginalis isolated from vulvovaginitis patient. The results were as follows: In 31 vaginal trichomoniasis the positive reactions of IgG antibody were 27 in the 1/8 dilution or higher and 4 in the 1/4 dilution whereas in healthy women the reaction showed signigicantly low as in the 1/4 dilution of below. The sensitivity and specificity of IFA test for IgG antibody to trichomonad antigen in this study were 87.1% and 100%, respectively. No significant difference of IgM antibody levels between vaginal trichomoniasis and healthy women was observed. No relation between the levels of IgG and IgM antibodies to trichomonad antigen by IFA test was observed. No relation between the time lapse and the level of serum IgG antibodies in IFA test of vaginal trichomoniasis was regarded. In conclusion the present study suggests that IFA test in trichomoniasis could be a useful tool for detection of anti-trichomonad IgG antibodies and applicable as an immundiagnostic method.

Ultrastructural studies on the surface of Paragonimus westermani metacercaria

The present study was undertaken to demonstrate the surface structure of Paragonimus westermani metacercaria in Korea with special reference to the distribution of sensory papillae. Metacercariae were isolated from crayfish, one of the second intermediate host of P. westermani in Bogil island, Chollanam-do (Province), Korea, where has been known as an endemic area of human paragonimiasis. Isolated metacercariae were excysted and examined with light, scanning and transmission electron microscopes for morphological features. On the surface of metacercariae, three types of sensory papillae were identified. Large domed papillae (3-5 micrometer), which were covered with wrinkled plasma membrane of the worm, were distributed on the oral and ventral suckers only. On the oral sucker, these large domed papillae were 12-13 in number. On the other hand large domed papillae on the ventral sucker were constantly 6 in number and hexagonal in distribution. Small domed papillae (2-3 micrometer), of which surface was more smooth than those of large ones, were distributed symmetrically on the ventral (30-32 pairs) and dorsal surfaces (40-42 pairs). Ciliated papillae (0.8-1.5 micrometer) were observed about 5-6 in number around the oral sucker and 3-5 pairs each on the ventral and dorsal surface of the body. Single pointed spines covered the entire surface of the body except around the excretory pore. Spines on the anterior part of the body were 0.9-2.0 micrometer in length and 45-55/100 square micrometer in number, and were gradually reduced in length (0.4-1.4 micrometer) and in number (12-27/100 square micrometer) toward the posterior part. The body wall of P. westermani metacercariae was consisted with anucleated syncytium layer, fibrous interstitial layer and musclar layer. In the anucleated syncytium, biconcave (0.15-0.55 micrometer) and spherical (0.08-0.16 micrometer) secretory granules, which were transferred from epidermal cells via protoplasmic tubules, mitochondria and ribosomes, were observed. Spines originated around the basement membrane protruded externally. Epidermal cells were consisted with a nucleus and a cytoplasm, and connected to syncytium with protoplasmic tubules. In the cytoplasm many secretory granules, mitochondria, Golgi complex, endoplasmic reticula, ribosomes and lipid droplets were observed.

Effect of flubendazole against Ascaris lumbricoides, Trichocephalus trichiurus and Enterobius vermicularis in infected children

Effectiveness of flubendazole, a newly introduced as an anthelmintic in Korea, was evaluated in 28 ascariasis, 28 whipworm infections and 17 pinworm infections in primary school children. All patients were orally given by flubendazole of 500 mg once. Stools for roundworm and whipworm and anal swabs for pinworm infections were examined both before and 25 days after treatment. After flubendazole treatment, all ascariasis and pinworm infected cases were cured. On the other hand, in whipworm infections, the cure rate was 82.1 percent (23 cured of 28 infected), and the egg reduction rate was 67.1 percent, respectively. Headache (one case), dizziness (2 cases) and abdominal pain (3 cases) were noticed as untoward effects, but they were mild and disappeared within 24 hours. These results demonstrate the excellent effectiveness of flubendazole in treating these helminth infections.