No abstract available.
Seizures*

No abstract available.

BACKGROUND AND OBJECTIVES: In addition to diagnostic criteria, a broad range of nonspecific clinical features can be found in patients with Kawasaki disease. This lack of specificity may cause confusion with other febrile illnesses and delay the diagnosis of Kawasaki disease. The purpose of this study is to describe common associated symptoms and their clinical significances in children affected with Kawasaki disease. SUBJECTS AND METHODS: As a retrospective study, we reviewed the medical records of 121 children who were treated for Kawasaki disease at Presbyterian medical center from January 2005 to June 2010. All clinical symptoms, laboratory data and echocardiographic findings in patients with KD were collected and analyzed. RESULTS: We found that there are 9 associated symptoms, namely cough, rhinorrhea, sputum, abdominal pain, vomiting, diarrhea, arthralgia, headache and seizure, which occur in patients with Kawasaki disease. Among the study group, there were only 32 children with no associated symptoms (26.4%). Patients with abdominal pain and headache had an older mean age than those without such symptoms. The incidence of seizure was significantly higher in incomplete Kawasaki disease patients compared with those with complete Kawasaki disease. Vomiting was highly associated with IVIG non-responder group. CONCLUSION: To decrease the incidence of serious coronary complications due to delayed diagnosis, physicians need to be aware of the manifestations of Kawasaki disease and the possibility of associated symptoms, which, being unspecific, may otherwise not raise any red flags.
Abdominal Pain ; Arthralgia ; Child ; Cough ; Delayed Diagnosis ; Diarrhea ; Headache ; Humans ; Immunoglobulins, Intravenous ; Incidence ; Medical Records ; Mucocutaneous Lymph Node Syndrome ; Protestantism ; Retrospective Studies ; Seizures ; Sensitivity and Specificity ; Sputum ; Vomiting

No abstract available.
Dandy-Walker Syndrome*

Infectious diseases of immigrant populations have recently become important issues for the health of both Korean nationals and foreigners living in Korea. This case report of general paresis is intended to raise awareness about re-emerging neurosyphilis in Korea as a possible etiology of psychotic disorders. A 68-year-old male Chinese resident came to Korea in 2019 with disorientation, auditory hallucination, persecutory delusion, and aggressive behavior, and was admitted to a psychiatric ward for further evaluation and treatment via the emergency department. He was confirmed to have neurosyphilis by serum test, analysis of cerebrospinal fluid, and brain magnetic resonance imaging. After treatment with antibiotics, including intravenous penicillin, in combination with atypical antipsychotics for 6 weeks, his disorientation, auditory hallucination, delusion, and aggressive behavior had attenuated.Neurosyphilis should still be included as a possible etiology of psychotic disorders in Korea.At the initial evaluation, syphilis screening serum tests are recommended for psychotic patients, especially those with pleomorphic symptoms and cognitive dysfunction.

No abstract available.
Alagille Syndrome* ; Cholestasis ; Xanthomatosis*

The neurofibromatoses are a rare group of hereditary diseases of autosomal dominant fashion with the overall incidence of one in 3,000~4,000 and with two distinct forms, type I (Von Recklinghausen's neurofibromatosis) characterized by skin lesions including multiple cutaneous neurofibromas, axillary and groin freckling, and cafe-au-lait spots and type II by the presence of bilateral vestibular schwannomas. The neurofibromatosis type I is associated with the mutation of NF-I gene on chromosome 17q 11.2, which has a tumor suppressive role and with resultant development of the wide variety of tumors with the malignant incidence of about 2~16 %. Although the cases of malignant tumors in the neurofibromatosis type I have been reported in various tumors with the neural origin, lymphoma, pheochromocytoma and some cases of gastrointestinal tumors, the gastrointestinal tumors were found in only hepatobiliary system and large and small bowels and the cases of the malignant tumors of upper GI origin in duodenum and stomach have not been reported yet in Korea. We report a case of a 64-year-old man admitted with epigastric pain and diagnosed to adenocarcinoma, signet ring cell type, in 2nd portion of duodenum associated with neurofibromatosis type I.
Adenocarcinoma* ; Cafe-au-Lait Spots ; Duodenum* ; Genetic Diseases, Inborn ; Groin ; Humans ; Incidence ; Korea ; Lymphoma ; Middle Aged ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Neuroma, Acoustic ; Pheochromocytoma ; Skin ; Stomach

Neural tube defects are reported one of the important congenital malformation in the world, with an incidence of 1.4 to 2 per 1000 pregnancies. Maternal serum AFP at the second trimester is used as screening test. But this is associated with numerous causes, including twins, fetal death, misdated pregnancies, cystic hygromas, teratomas, renal abnormalities, esophageal atresia and aberrations in the placenta. If the AFP level was elevated, targeted ultrasonography should be evaluated for confirming the gestational age and fetal viability, fetal number. Then if the ultrasonographic examination is nondiagnostic, or if an NTD is suspected, amniotic fluid AFP should be measured with acetylcholinesterase. But the false positive rate of the AF-AFP is high, and there is 0.3% of the false positive rate in amniotic acetylcholinesterase. This time the women with normal ultrasonographic examination continue the pregnancy after counseling of family history, past history with follow up of ultrasonography. We report a case with elevated second trimester MS-AFP, AF-AFP and positive amniotic acetylcholinesterase, but in which repeated sonographic findings were normal and result in delivery of a healthy baby without anomalies.
Acetylcholinesterase* ; Amniotic Fluid ; Counseling ; Esophageal Atresia ; Female ; Fetal Death ; Fetal Viability ; Follow-Up Studies ; Gestational Age ; Humans ; Incidence ; Lymphangioma, Cystic ; Mass Screening ; Neural Tube Defects ; Placenta ; Pregnancy Trimester, Second ; Pregnancy* ; Teratoma ; Ultrasonography

No abstract available.
Wolf-Hirschhorn Syndrome*

Macroglossia is an obstacle in regard to general prosthodontic restoration. Not only is it difficult to obtain support and stability from dentures manufactured from normal protocol, obtaining efficacy from basic mastication is also difficult. In such cases, realizing harmony between the occlusion and surrounding muscle structures may be important with regard to manufacture of stable full dentures, and it is necessary to form the appropriate polished surface for this case. The neutral zone is defined as the potential area resulting from the neuromuscular function that results in equilibrium between the outward force exerted by the tongue and the inward force exerted by the lips and cheeks. The artificial teeth of the full denture lies in this area, and if the polished zone follows the anatomic form of the dynamic muscles, the movement of the muscles simply acts as an element of stabilizing the dentures improving the esthetics through facial support.
Cheek ; Dentures ; Esthetics ; Lip ; Macroglossia ; Mastication ; Muscles ; Rehabilitation ; Tongue ; Tooth, Artificial