The measurement and visualization of the roundness of the cranial circumference has not been attempted by the simple measurement device. That's why there has been a tendency that the morphologic cranial deformity can be diagnosed with accuracy only by the experienced physician. The accurate understanding of the roundness of the cranial circumference, however, is essential for the diagnosis and the decision of the treatment principle in cranofacial morphological deformity. Current methods, such as simple physical examination and/or the photography, are not enough to accurately express the roundness of the cranial circumference. In order to develop the new method of measurement, authors selected 16 points from the axial cutting plane of the cranium. These points can be selected under the same principle even though the axial plane changes. After measuring the distance of 16 points, the values are put into computer program. In conclusion, authors can retrieve the x, y coordinates of the 16 points and can show the intuitive roundness of the circumference of the selected axial plane of the cranium. This measurement tool will be helpful not only for the identification of the severity of the morphologic cranial deformity, but for the classification and the assessment of the result of the surgery.
Classification ; Congenital Abnormalities ; Diagnosis ; Photography ; Physical Examination ; Skull

PURPOSE: Recent studies have demonstrated that probiotics are effective in the management of allergic diseases, but there have been few reports about their preventive effects in asthma. We examined the effects of Lactobacillus acidophilus (LA) and its mechanism in the primary prevention of asthma. METHODS: Before allergen sensitization, newborn Balb/c mice orally received 1x10(9) colony forming unit (CFU) LA per day every 2 days for consecutive 4 weeks starting from birth. After ovalbumin sensitization and challenge from day 36, airway hyperresponsiveness (AHR) to methacholine, influx of inflammatory cells to the lung, serum total IgE levels, cytokine levels in the supernatant of splenocytes culture, and expression of FoxP3 mRNA in the peribronchial lymph nodes were assessed. RESULTS: The AHR showed a tendency to decrease in LA group, however, there was no statistical significance. The eosinophil counts in bronchoalveolar lavage fluid and serum total IgE were significantly reduced in LA group compared with asthma control group. LA effectively induced IFN-gamma secretion and inhibited IL-4, IL-5 and TGF-beta1 secretion compared with asthma control group. The expression of FoxP3 mRNA in peribronchial lymph nodes did not show any difference between LA and asthma control group. CONCLUSION: Oral LA administration in murine model of asthma attenuated AHR and significantly suppressed eosinophilic inflammation in the airway. These effects of LA were mediated by suppressing Th2 immune response and enhancing Th1 immune response. The results may suggest the possible role of LA in the primary prevention of asthma.
Animals ; Asthma ; Bronchoalveolar Lavage Fluid ; Eosinophils ; Humans ; Immunoglobulin E ; Infant, Newborn ; Inflammation ; Interleukin-4 ; Interleukin-5 ; Lactobacillus ; Lactobacillus acidophilus ; Lung ; Lymph Nodes ; Methacholine Chloride ; Mice ; Ovalbumin ; Parturition ; Primary Prevention ; Probiotics ; RNA, Messenger ; Stem Cells ; Transforming Growth Factor beta1

Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms' tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had deletion of chromosome 11p11.2-13. Bilateral Wilms' tumors were successfully treated by chemotherapy and surgery. She has been tumor-free for 19 months off chemotherapy with preserved renal function.
Aniridia ; Humans ; Intellectual Disability ; Korea ; Parturition ; Preschool Child ; WAGR Syndrome ; Wilms Tumor

A 44-year-old man treated with azathioprine, cyclosporine and prednisolone for 7.5 years after allogeneic renal transplantation was admitted because of exertional dyspnea, fatigue and pancytopenia which were found 3 months ago. He had been on hemodialysis for renal failure of unknown cause for 8 months before the renal transplantation. Bone marrow examination showed hypercellularity, erythroid hyperplasia and 7% of myeloblast, consistent with the diagnosis of myelodysplastic syndrome. Cytogenetic study showed chromosomal abnormalities:deletion of chromosome 5, monosomy 7, trisomy 8, monosomy 14 and deletion of chromosome 17. Immunosuppressive agents were discontinued and he was treated with transfusion, G-CSF, and combination chemotherapy including topotecan and Ara-C. Graft kidney function was normal before and after the treatment, but the clinical course was fatal because of leukemic transformation and eventually sepsis. Although therapy induced myelodysplastic syndrome was rare in renal allograft recipients, thorough evaluations including bone marrow biopsy and cytogenetic study are recommended in patients with anemia of unknown etiology.
Adult ; Allografts ; Anemia ; Azathioprine ; Biopsy ; Bone Marrow ; Bone Marrow Examination ; Chromosome Aberrations ; Chromosomes, Human, Pair 17 ; Chromosomes, Human, Pair 5 ; Cyclosporine ; Cytarabine ; Cytogenetics ; Diagnosis ; Drug Therapy, Combination ; Dyspnea ; Fatigue ; Granulocyte Colony-Stimulating Factor ; Granulocyte Precursor Cells ; Humans ; Hyperplasia ; Immunosuppressive Agents ; Kidney ; Kidney Transplantation ; Monosomy ; Myelodysplastic Syndromes* ; Pancytopenia ; Prednisolone ; Renal Dialysis ; Renal Insufficiency ; Sepsis ; Topotecan ; Transplantation* ; Transplants ; Trisomy

BACKGROUND/AIMS: Performance of polyethylene glycol solution (PEG) is often unsatisfactory as bowel preparation agent for colonoscopy. In order to provide equivalent efficacy with better patient tolerance, sodium phosphate tablet (SPT) has been developed. This study was carried out to compare the efficacy and compliance of two bowel preparation methods: PEG with ascorbic acid (PEGA) vs. SPT preparation. METHODS: A multicenter, randomized controlled trial was performed. Primary efficacy variable was overall quality of colon cleansing assessed by Boston bowel preparation scale (BBPS) during colonoscopy. Patient's satisfaction and adverse events were evaluated by means of symptom questionnaire completed by each patient immediately before colonoscopy. RESULTS: A total of 189 patients were randomly assigned to undergo pre-colonoscopic bowel preparation with either SPT (n=96) or PEGA (n=93). Overall BBPS score was 8.3+/-1.12 in the SPT group and 8.4+/-0.96 in the PEGA group (p=0.441). Among the 189 patients, 90 had polyps (47.6%) and 50 had adenomas (26.5%). The polyp/adenoma detection rate was 54.2% (n=52)/27.1% (n=26) for SPT group and 40.9% (n=38)/25.8% (n=24) for PEGA group (p=0.079 and 0.790, respectively). More number of patients were unable to take the prescribed dose of PEGA compared with the SPT regimen (8.6% vs. 2.0%, p=0.045). Overall satisfaction score was 7.9+/-1.63 in the SPT group and 7.4+/-1.53 in the PEGA group (p=0.022). CONCLUSIONS: Degree of colon preparation, polyp/adenoma detection rate and adverse effect were similar between SPT group and PEGA group. Patient compliance and satisfaction were greater in the SPT group.
Abdominal Pain/etiology ; Adenoma/pathology ; Adult ; Ascorbic Acid/*therapeutic use ; Cathartics/adverse effects/*therapeutic use ; Colonic Polyps/pathology ; *Colonoscopy ; Female ; Humans ; Male ; Middle Aged ; Nausea/etiology ; Patient Satisfaction ; Phosphates/*therapeutic use ; Polyethylene Glycols/*therapeutic use ; Surveys and Questionnaires

BACKGROUND/AIMS: Endoscopic mucosal resection (EMR) is a useful method in both the accurate diagnosis and treatment of gastric mucosal lesions. A gastric adenoma is a neoplastic lesion which has malignant potential, and therefore it is advisable to have it removed completely when discovered. We evaluated the role of EMR in the treatment of gastric adenomas by analyzing the follow-up endoscopic results of patients treated with EMR for such lesions. METHODS: We analyzed the initial endoscopic findings, EMR results, and follow-up endoscopic outcomes of 35 patients with 41 gastric adenomas, from June 1994 to January 1997 in Seoul National University Hospital.
Adenoma* ; Diagnosis ; Follow-Up Studies ; Humans ; Recurrence ; Seoul

BACKGROUND/AIMS: The disease course and factors associated with poor prognosis in Korean patients with Crohn’s disease (CD) have not been fully determined. The aim of this study was to explore potential associations between the clinical characteristics and long-term outcomes of CD and perianal fistulas in a Korean population. METHODS: The retrospective Crohn’s Disease Clinical Network and Cohort (CONNECT) study enrolled patients diagnosed with CD between July 1982 and December 2008 from 32 hospitals. Those followed for < 12 months were excluded. Clinical outcomes were CD-related surgery and complications, including nonperianal fistulas, strictures, and intra-abdominal abscesses. RESULTS: The mean follow-up period was 8.77 years (range, 1.0 to 25.8 years). A total of 1,193 CD patients were enrolled, of whom 465 (39.0%) experienced perianal fistulas. Perianal fistulizing CD was significantly associated with younger age, male gender, CD diagnosed at primary care clinics, and ileocolonic involvement. Both nonperianal fistulas (p=0.034) and intra-abdominal abscesses (p=0.020) were significantly more common in CD patients with perianal fistulas than in those without perianal fistulas. The rates of complicated strictures and CD-related surgery were similar between the groups. Independently associated factors of nonperianal fistulas were perianal fistulas (p=0.015), female gender (p=0.048), CD diagnosed at referral hospital (p=0.003), and upper gastrointestinal (UGI) involvement (p=0.001). Furthermore, perianal fistulas (p=0.048) and UGI involvement (p=0.012) were independently associated with the risk of intra-abdominal abscesses. CONCLUSIONS: Perianal fistulas predicted the development of nonperianal fistulas and intra-abdominal abscesses in Korean CD patients. Therefore, patients with perianal fistulizing CD should be carefully monitored for complicated fistulas or abscesses.
Abdominal Abscess ; Abscess ; Cohort Studies ; Constriction, Pathologic ; Crohn Disease ; Female ; Fistula* ; Follow-Up Studies ; Humans ; Korea* ; Male ; Primary Health Care ; Prognosis* ; Rectal Fistula ; Referral and Consultation ; Retrospective Studies

BACKGROUND: This study was done to analyze the treatment outcomes of adult lymphoblastic lymphoma patients according to the risk groups and the treatment regimens. METHODS: The analysis was performed on twenty patients histologically diagnosed as lymphoblastic lymphoma at Seoul National University Hospital. The high-risk group was defined as patients with Ann Arbor stage IV with bone marrow, central nervous system (CNS) involvement or initial serum lactate dehydrogenase level more than 1.5 times upper normal limits. Twelve patients received dose-intensive Stanford/Northern California Oncology Group (NCOG) regimen consisted of four phase of induction, CNS prophylaxis, consolidation, and maintenance. Eight patients received conventional dose regimen, either six courses of vincristine, bleomycin, cyclophosphamide, doxorubicin, prednisolone, procarbazine (COPBLAM-V) or cyclophosphamide, vincristine, doxorubicin, prednisolone, L-asparaginase (CHOP/L-ASP) with CNS prophylaxis. We analyzed the response rate and the survival rate according to the risk groups and treatment regimens. RESULTS: The overall response rate was 90% (75% complete response). In low-risk group, the complete response (CR) rate was 100% irrespective of treatment regimen. In high-risk group, conventional dose regimen did not produce CR. Four of the six high-risk patients receiving dose-intensive regimen achieved CR. The 5-year overall survival (OS) rate was 52% in total patients. The 5-year OS rate were 77% and 30% for low- and high-risk group, respectively (P=0.242). In low-risk group, conventional dose regimen showed similar survival outcomes compared with that of dose intensive regimen. Toxicity profile was more favorable in the patients with conventional dose regimens. CONCLUSION: For low-risk patients, conventional dose regimen showed similar effect in comparison with dose-intensive regimen. However, for high-risk patients, CR was observed only with dose-intensive regimen. Multi-center clinical trials are necessary to confirm our observation.
Adult ; Male ; Female ; Humans

Intravascular large B-cell lymphoma (IVLBL) is a rare neoplasm characterized by proliferation of lymphoma cells within the lumina of small vessels. Neurological and skin involvements usually predominate. We would describe a 78-year-old woman presented with fever, multiple erythematous skin lesions, and language disturbance. The skin biopsy of breast revealed IVLBL and malignant cells were also seen in the bone marrow. Shortly after completion of six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) with clinical response, intracranial relapse with multiple brain masses occurred. The palliative whole brain radiation therapy was given and intensive chemotherapy should be investigated in the case presented here.
Aged ; B-Lymphocytes* ; Biopsy ; Bone Marrow ; Brain ; Breast ; Doxorubicin ; Drug Therapy* ; Female ; Fever ; Humans ; Lymphoma ; Lymphoma, B-Cell* ; Recurrence* ; Skin ; Vincristine

BACKGROUND: A US Food and Drug Administration (FDA)-approved drug methacholine chloride (Provocholine®) was recently introduced to Korea where it is now widely used in clinical practice. We aimed to evaluate the prevalence, risk factors and cutoff value of bronchial hyperresponsiveness (BHR) to Provocholine in 7-year-old children. METHODS: Six hundred and thirty-three children from the Panel Study on Korean Children who visited 16 regional hospitals were evaluated. Skin prick tests, spirometry and bronchial provocation tests for Provocholine as well as a detailed history and physical examinations were performed. The bronchial provocation test was reliably performed on 559 of these children. RESULTS: The prevalence of ever-diagnosed asthma via medical records was 7.7%, and that of current asthma (wheezy episode in the last 12 months + diagnosed asthma by physicians) was 3.2%. The prevalence of BHR to Provocholine was 17.2% and 25.8%, respectively, for a PC20 < 8 and < 16 mg/mL. The risk factors for BHR (PC20 < 16 mg/mL) were atopic dermatitis diagnosis and current dog ownership, whereas those for current asthma were allergy rhinitis diagnosis, a history of bronchiolitis before the age of 3, recent use of analgesics/antipyretics and maternal history of asthma. The BHR prevalence trend showed an increase along with the increased immunoglobulin E (IgE) quartile. The cutoff value of PC20 for the diagnosis of current asthma in children at age 7 was 5.8 mg/mL (sensitivity: 47.1%, specificity: 87.4%). CONCLUSIONS: BHR to Provocholine (PC20 < 8 mg/mL) was observed in 17.2% of 7-year-olds children from the general population and the cutoff value of PC20 for the diagnosis of current asthma was 5.8 mg/mL in this age group. The risk factors for BHR and current asthma showed discrepancies suggesting different underlying mechanisms. Bronchial provocation testing with Provocholine will be a useful clinical tool in the future.
Animals ; Asthma ; Bronchial Hyperreactivity ; Bronchial Provocation Tests ; Bronchiolitis ; Child* ; Dermatitis, Atopic ; Diagnosis ; Dogs ; Humans ; Hypersensitivity ; Immunoglobulin E ; Immunoglobulins ; Korea ; Medical Records ; Methacholine Chloride* ; Ownership ; Physical Examination ; Prevalence* ; Rhinitis ; Risk Factors* ; ROC Curve ; Sensitivity and Specificity ; Skin ; Spirometry ; United States Food and Drug Administration