Tuberous sclerosis is an autosomal dominant disorder of cellular differentiation that affect the brain, skin, heart, kidney and other organs. We experienced three cases of tuberous sclerosis that affect multiple organs in mother and two daughters. We report these cases with brief review and related literatures.
Brain ; Heart ; Humans ; Kidney ; Mothers* ; Nuclear Family* ; Skin ; Tuberous Sclerosis*

PURPOSE: Among syndromes of idiopathic occipital lobe epilepsy there are Panayiotopoulos syndrome (PS) and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). However, the classification of epilepsy syndrome is difficult to diagnose at the first seizure because of varying characteristics. We analyzed the different clinical characteristics and prognostic factors of idiopathic occipital lobe epilepsy. METHODS: Forty-three patients who had at least two unprovoked seizures, no abnormalities on magnetic resonance imaging, no known etiology of seizure, and mainly occipital spikes on electroencephalography were retrospectively enrolled at the Korea University Medical Center. A good prognosis was defined as being seizure-free and taking two or less drugs for 1 year. RESULTS: Among all patients, the proportion of PS was 44.2%, ICOE-G was 7.0% and unclassified group was 48.8%. The age at seizure onset was 4.5±2.6 years (mean±SD) in the PS group, and 8.3±2.1 years in the ICOE-G group. The follow-up duration was 10.8±6.0 years. The percentage that the initial diagnosis had not been changed was 66.7% in the PS group and 100% in the ICOE-G group. Among other related symptoms only emesis (P<0.001) and visual symptoms (P=0.007) had varying characteristics between the PS and ICOE-G groups. Patients with PS had a better prognosis than those with ICOE-G or unclassified group (odds ratio [OR]=58.8). Patients with 1 more autonomic symptoms had a worse prognosis (OR=3.8). CONCLUSION: This study showed that only symptoms of emesis and visual symptoms can differentiate between PS and ICOE-G. More autonomic symptoms were associated with worse prognosis in childhood occipital lobe epilepsy.
Academic Medical Centers ; Classification ; Diagnosis ; Electroencephalography ; Epilepsies, Partial* ; Epilepsy ; Follow-Up Studies ; Humans ; Korea ; Magnetic Resonance Imaging ; Occipital Lobe* ; Prognosis ; Retrospective Studies ; Seizures ; Vomiting

Purpose: To evaluate the efficacy and safety of ultrasonography- and fluoroscopy-guided transvaginal catheter drainage for pelvic fluid collection. Materials and Methods: This single-center retrospective study included nine female patients who underwent transvaginal drainage for pelvic fluid collection unsuitable for transabdominal access between October 2016 and January 2023. The study analyzed patient symptoms and signs, ultrasonography and CT findings, transvaginal approach details, and technical and clinical outcomes, including complications. Catheters of size 7 or 8.5-Fr, with or without an endocavitary needle guide were used for the puncture. Results: Pelvic fluid collections were primarily post-operative (n = 7) or from patients with primary tubo-ovarian abscesses (n = 2). All patients achieved technical success, defined as the successful insertion of the drainage catheter at the site of target fluid collection, and clinical success, defined as the improvement of symptoms with catheter removal within 1 week. In seven cases, the procedure was assisted by an endocavitary needle guide. The median catheter indwelling period was 2 days, with two minor complications: accidental catheter removal and urinary bladder puncture, both of which were managed without further complications. Conclusion Ultrasonography- and fluoroscopy-guided transvaginal drainage are effective and safe methods for managing pelvic fluid collections that are unsuitable for a transabdominal approach.

Purpose: To evaluate the efficacy and safety of ultrasonography- and fluoroscopy-guided transvaginal catheter drainage for pelvic fluid collection. Materials and Methods: This single-center retrospective study included nine female patients who underwent transvaginal drainage for pelvic fluid collection unsuitable for transabdominal access between October 2016 and January 2023. The study analyzed patient symptoms and signs, ultrasonography and CT findings, transvaginal approach details, and technical and clinical outcomes, including complications. Catheters of size 7 or 8.5-Fr, with or without an endocavitary needle guide were used for the puncture. Results: Pelvic fluid collections were primarily post-operative (n = 7) or from patients with primary tubo-ovarian abscesses (n = 2). All patients achieved technical success, defined as the successful insertion of the drainage catheter at the site of target fluid collection, and clinical success, defined as the improvement of symptoms with catheter removal within 1 week. In seven cases, the procedure was assisted by an endocavitary needle guide. The median catheter indwelling period was 2 days, with two minor complications: accidental catheter removal and urinary bladder puncture, both of which were managed without further complications. Conclusion Ultrasonography- and fluoroscopy-guided transvaginal drainage are effective and safe methods for managing pelvic fluid collections that are unsuitable for a transabdominal approach.

Purpose: To evaluate the efficacy and safety of ultrasonography- and fluoroscopy-guided transvaginal catheter drainage for pelvic fluid collection. Materials and Methods: This single-center retrospective study included nine female patients who underwent transvaginal drainage for pelvic fluid collection unsuitable for transabdominal access between October 2016 and January 2023. The study analyzed patient symptoms and signs, ultrasonography and CT findings, transvaginal approach details, and technical and clinical outcomes, including complications. Catheters of size 7 or 8.5-Fr, with or without an endocavitary needle guide were used for the puncture. Results: Pelvic fluid collections were primarily post-operative (n = 7) or from patients with primary tubo-ovarian abscesses (n = 2). All patients achieved technical success, defined as the successful insertion of the drainage catheter at the site of target fluid collection, and clinical success, defined as the improvement of symptoms with catheter removal within 1 week. In seven cases, the procedure was assisted by an endocavitary needle guide. The median catheter indwelling period was 2 days, with two minor complications: accidental catheter removal and urinary bladder puncture, both of which were managed without further complications. Conclusion Ultrasonography- and fluoroscopy-guided transvaginal drainage are effective and safe methods for managing pelvic fluid collections that are unsuitable for a transabdominal approach.

Avian influenza (AI) is an infectious, low pathogenic virus that is endemic all over the world and poses a potential threat to the poultry industry. Vaccination is a widely used effective method to prevent avian influenza virus. Here we employed a comparative proteomics approach [two-dimensional electrophoresis (2-DE) and matrix assisted laser desorption ionization-time of flight (MALDI-TOF)] to characterize proteome in the sera from the specific pathogen free (SPF) chickens, the vaccinated chickens, and the naturally infected chickens. We identified total 58 proteins that were differentially expressed in the sera of three groups. Among them ovotransferrin and vitamin D-binding protein were more expressed in the sera of naturally infected chickens compare with other groups. Our results suggested that the level of these two proteins in the serum may help to discriminate the naturally infected chicken from the vaccinated chicken.
Animals ; Chickens ; Conalbumin ; Electrophoresis ; Influenza in Birds ; Poultry ; Proteins ; Proteome ; Proteomics ; Specific Pathogen-Free Organisms ; Vaccination ; Viruses ; Vitamin D-Binding Protein

It is well-known that the prevalence of attention deficit hyperactivity disorder (ADHD) is higher in epileptic children than in the general pediatric population. The aim of this study was to compare the accompaniment of ADHD in epileptic children with well-controlled seizures and no significant intellectual disability with that in healthy controls. We included epileptic children between the ages of 6 and 12 yr visiting our clinic for six consecutive months and controls without significant medical or psychiatric illnesses. We excluded patients with intellectual disability or persistent seizures during the recent three months. The diagnosis of ADHD was based on the criteria of the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV). After exclusion of 84 patients, we enrolled 102 (54.8%) children (mean age, 9.4 +/- 2.0 yr). Seven (7 of 102, 6.9%) were diagnosed with ADHD. As compared to control group (4 of 110, 3.6%), there was no difference in ADHD accompaniment (P = 0.29). No difference was observed in ADHD accompaniment according to seizure type and epilepsy syndrome. In conclusion, the accompaniment of ADHD in epileptic children with well-controlled seizures and no intellectual disability may not differ from that of the general pediatric population.
Age Factors ; Attention Deficit Disorder with Hyperactivity/complications/*diagnosis ; Brain/radiography ; Child ; Electroencephalography ; Epilepsy/complications/*diagnosis ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Seizures/complications/diagnosis ; Sex Factors

PURPOSE: Asthma is one of the most common chronic childhood disease. Education of asthmatic children and their families about asthma and its management may improve disease control, reduce symptoms, and improve school performance. The aim of the study was to evaluate the effects of an intensive asthma education program in asthmatic children and their families on outcome measure of asthma management behavior scale, knowledge about asthma, self efficacy scale and quality of life. METHODS: Fifteen asthmatic children and their families were invited the intensive asthma education program which including allergen avoidance, management of asthma, correct use of the inhalation devices and control of exercise-induced asthma (study group). Fifteen asthmatic children and their families those who did not participate this program were served as control group. Participants were asked to complete a written questionnaire before and 3-month after the program. RESULTS: After completing the intensive education program, significant improvement of the childrens asthma management behavior scale (27.1 vs. 32.2, P=0.011), belief and knowledge about asthma (14.2 vs. 17.9, P<0.001), self efficacy (47.9 vs. 49.7, P=0.091) and quality of life (79.6 vs. 88.6, P<0.001) was noted in the study group by measuring questionnaires. There are increasing tendencies in parental asthma management behavior scale and knowledge about asthma. CONCLUSION: This intensive asthma education program is effective in improving asthma control, self efficacy and quality of life of asthmatic children. This should serve as a national model for family-based programs for asthmatic children and their families.
Asthma ; Asthma, Exercise-Induced ; Caregivers ; Child ; Disease Management ; Humans ; Nebulizers and Vaporizers ; Outcome Assessment (Health Care) ; Parents ; Patient Education as Topic ; Quality of Life ; Surveys and Questionnaires ; Self Efficacy

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
Acute Disease ; Congenital Abnormalities/*diagnosis/ultrasonography ; Endosonography ; Female ; Heterotaxy Syndrome/*diagnosis/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Pancreas/abnormalities/ultrasonography ; Pancreatitis/*diagnosis ; Tomography, X-Ray Computed

Avian influenza (AI) virus infects both animal and human. Low pathogenic AI virus infections (some H7 and H9 subtypes) have been reported all over the world and pose a potential threat to the poultry industry. Vaccination is the most effective way to prevent virus infection. However, vaccination makes it difficult to differentiate between vaccinated chickens and infected chickens. In order to differentiate vaccinated chickens from naturally infected chickens, we adopted synthetic peptide-based enzyme-linked immunosorbent assay (ELISA) using the peptide sequences from nonstructural protein 1 (NS1) of H9N2. Five synthetic peptides were designed using Protein Variability Sever (http://imed.med.ucm.es/PVS/) and synthesized. NS1-1 ~ NS1-4 peptides failed to detect serum antibodies from both vaccinated and naturally infected chickens. NS1-5 peptide from the C-terminal NS1 protein detected serum antibody from naturally infected chickens but not vaccinated chickens. These results imply that NS1-5 peptide may be a useful tool to differentiate naturally infected chicken from vaccinated chicken as being used in the synthetic peptide-based ELISA.
Animals ; Antibodies ; Chickens ; Enzyme-Linked Immunosorbent Assay ; Humans ; Influenza in Birds ; Peptides ; Poultry ; Vaccination ; Viruses