Craniofacial dysostosis, a well defined. rare syndrome first described by Crouzon in 1912, characteristically shows frontal bosses, prognathism, exophthalmos, exotropia, optic nerveatrophy and maxillary hypoplasia. Three cases of familial occurrence are presented, and according to their history, 11 of 13 members in 5 generations of their family are suspected to have been afflicted. A brief review of related literature is described.
Craniofacial Dysostosis* ; Exophthalmos ; Exotropia ; Family Characteristics ; Humans ; Prognathism

PURPOSE: The pharmacologic effect of atropine on HPS can be considered to control pyloric muscle spasm. Therefore, we studied the effects of intravenous atropine sulfate on the clinical course of HPS, and periodically observed the ultrasonographic appearance of the pyloric muscles after atropine treatment. METHODS:From April 1998 to May 1999, 14 infants who were diagnosed with HPS were treated with intravenous atropine sulfate. Intravenous atropine sulfate was administered at an initial dose of 0.04mg/kg/day, which was divided into 8 equal doses. The daily dose was increased by 0.01 mg/kg/day until vomiting was controlled for an entire day while infants received unrestricted oral feeding. Ultrasonographic examinations were performed during hospitalization and repeated at least every 2 months until normalization of pyloric muscles was confirmed. RESULTS: Intravenous atropine was effective in 12 of 14 infants with HPS and the conditions of 9 of them improved. Two infants who were not free from vomiting despite a week of intravenous atropine sulfate treatment underwent pyloromyotomy. A series of ultrasonographic examinations were done after vomiting had improved with intravenous atropine sulfate. The ultrasonographic findings showed good passage of gastric contents through pyloric canals despite thickening of the pyloric muscles. CONCLUSION: Intravenous administration of atropine sulfate is an effective therapy for HPS and can be an alternative to pyloromyotomy. (J Korean Pediatr Soc 2000;43:763-768)
Administration, Intravenous ; Atropine* ; Hospitalization ; Humans ; Infant ; Muscles ; Pyloric Stenosis, Hypertrophic* ; Spasm ; Vomiting

Pagonimus Westmani, occuring as an important human infestation of the lung, is widely distributed in the Far East ; Korea, Japan, China and the Philippines. Since Otani found the parasites for the first time in the human brain in 1887, it has been established that cerebral infestation occurs with the highest frequency among extrapulmonary involvement. In 0.8 to 25 percent of patients infested with the lung flukes cerebral paragonimiasis may be developed. The larvae may procedure arachnoiditis, abscess, fibrous cystic lesions and granulomas in the brain. Based on a review of the literatures and upon the 7 cases of cerebral paragonimiasis which have been observed by the authors at the National Medical Center, Seoul, Korea, the following points are stressed. 1) Males had a significantly higher prevalence than females. Cerebral paragonimiasis was most commonly found in young adults(16 to 25yrs). 2) Clinically the patients with cerebral involvement were characterized by Jacksonian type of epilepsy, headache and visual disturbance. Mental deterioration, hemiplegia, hemihypesthesia, homonymous hemianopsia and optic atrophy were the five major signs. 3) The patients having symptoms for less than 6 months developed mild leukocytosis in many cases. All the cases of cerebral paragonimiasis revealed positive reaction in the intradermal tests with purified antigens of P. Westermani. 4) On skull films multiple round or oval cystic calcification was diagnostic value for cerebral paragonimiasis. On angiogram vascular staining and abnormal vessels were not disclosed. On pneumoencephalogram subcortical atrophy or filling defect of the ventricle may be showed. 5) The predilection area of the granuloma and fibrous cystic mass were the occipital, posterior parietal and posterior temporal lobes. 6) The patients with chronic stabilized cerebral paragonimiasis. having intractable symptoms and signs, could not be improved with Bithionol treatment. But headache, vomiting, visual disturbance, facial weakness and especially intractable seizure were improved in our cases treated with surgical operation.
Abscess ; Arachnoid ; Arachnoiditis ; Atrophy ; Bithionol ; Brain ; China ; Epilepsy ; Far East ; Female ; Granuloma ; Headache ; Hemianopsia ; Hemiplegia ; Humans ; Intradermal Tests ; Japan ; Korea ; Larva ; Leukocytosis ; Lung ; Male ; Optic Atrophy ; Paragonimiasis* ; Parasites ; Philippines ; Prevalence ; Rabeprazole ; Seizures ; Seoul ; Skull ; Temporal Lobe ; Trematoda ; Vomiting

Bailey and Cushing described the oligodendroglioma firstly in 1926. The oligodendroglioma is rare tumor and involves the ventricular system in 7% to 10% of all cases. The authors present a case of intraventricular oligodendroglioma associated with arterio venous malformations in a 34 year old male who had the history of visual disturbance and mental deterioration, for one month. The diagnosis was confirmed by operation and autopsy.
Adult ; Autopsy ; Diagnosis ; Humans ; Male ; Oligodendroglioma*

BACKGROUND: As single donor platelets (SDP) has been increasingly used, the quality of SDP, especially apheresis-induced platelet activation, has become a major issue. This study evaluated the activation of SDP platelets prepared with three different cell separators that are currently being used at the Korean Red Cross. METHODS: CD62p, CD63 and CD42 were measured in 35 units of SDP prepared with Amicus (Baxter, Deerfield, IL, USA), MCS+ (Haemonetics, Braintree, MA, USA), or Trima (Gambro BCT, Lakewood, USA) using flow cytometry. RESULTS: Expression of CD62p gradually increased with storage time, but no difference in expression was noted between cell separators. Expression of CD63 also increased with storage time and platelets prepared with the Amicus displayed significantly higher CD63 expression 72 and 120 hours after collection compared to those prepared with MCS+ and Trima. Expression of CD42b tended to decrease with storage time, but this was only significant for Amicus 120 hours after collection. No difference in CD42b expression was noted between cell separators. CONCLUSIONS: Platelet activation increased with storage time, and platelet activation was more pronounced in the platelets prepared with the Amicus. However, because in vitro results of platelet activation does not necessarily reflect in vivo platelet function and survival, additional studies are needed to clarify clinical effectiveness of activated platelets.
Blood Platelets* ; Flow Cytometry ; Humans ; Platelet Activation* ; Plateletpheresis* ; Red Cross ; Tissue Donors

PURPOSE: The purpose of this study was to develop a vital signs e-book for undergraduate student nurses and evaluate the content, system and student satisfaction. METHOD: This study was done in three stages, the development of a vital signs e-book, implementation and evaluation. The subjects were 73 undergraduate student nurses in Y university. RESULT: Thirty one learning objectives were used to create the contents. A set of 5 chapters and 18 subsections were defined after validation from nurse educators. The e-book is available at http://123.134.207.23/ebook/vitalsigns. Analysis of the questionnaires showed a mean score for content, system and students satisfaction of 3.17 +/- .73, 3.11 +/-.79, and 2.96 +/-.74 respectively out of a possible 4 points. CONCLUSION: Nurse educators should provide quality and effective web-based courses that meet undergraduate student nurses' learning needs and they should incorporate web-based learning into traditional teaching to meet the demands of nursing education.
Respiration ; Pulse ; *Physical Examination ; *Internet ; *Education, Nursing ; *Computer-Assisted Instruction

Restless leg syndrome (RLS) is characterized by intense restlessness and unpleasant creeping sensations deep inside the lower legs, occurring during periods of rest, evening and night. These symptoms can be improved by movement. There are two different phenotypes of RLS. One early-onset form starts before 36 years old. It has mostly a familial history, severe symptoms, and highly genetically determined. And it is a highly dependent to iron level of the brain. The other delayed-onset form starts after 36 years old, mostly secondary, without familial history, with a rapid evolution in two or three years. And it is associated with frequent low ferritin level of serum. Pathophysiology of RLS remains incompletely understood. However, advanced studies suggest that RLS may be generated by dopamine dysfunction locally within the central nervous system. Dopaminergic agonists are the treatment of choice, if the symptoms are severe. And iron therapy improves RLS symptoms in iron deprived patients. Early detection during pregnancy is needed because RLS gives an important impact on sleep efficiency and quality of life. Recently we have experienced a case of primary RLS patient diagnosed at 24+3 weeks, treated by dopaminergic agonist ropinirole and iron. We describe this case with a brief review of the literature.
Brain ; Central Nervous System ; Dopamine ; Dopamine Agonists ; Ferritins ; Humans ; Indoles ; Iron ; Leg ; Phenotype ; Pregnancy ; Psychomotor Agitation ; Quality of Life ; Sensation

Type 1 diabetes is considered as Th1 cell mediated autoimmune disease and the suppression of Th1 cells or the activation of Th2 cells has been regarded as a plausible immunologic intervention for the prevention of type 1 diabetogenesis in a rodent model. CpG ODN is an immunostimulatory sequence primarily present in bacterial DNA, viral DNA and BCG. CpG ODN is conventionally classified as a Th1 cell activator, which has been clinically applied to cancer, allergy and infectious disease. Recently, there was a promising report of that CpG ODN administration suppressed the development of type 1 diabetes in NOD mice by inducing Th2 cell mediated cytokine. However, the antidiabetogenic effect of CpG ODN on NOD mice is controversial. Thus, two studies were serially undertaken with various kinds of CpG motif to find a more optimal sequence and administration method. In the first study, CpG ODN was vaccinated four times and pancreatic inflammation and the quantity of serum insulin subsequently evaluated. In the second study, the amounts of IFN gamma and IL-4 in sera were measured as representative cytokines of Th1 and Th2 cells, respectively. As a result, vaccination or continuous injection of CpG ODN failed to show a preventive effect on type 1 diabetogenesis in NOD mice. Structural differences of CpG ODN also had no affect on the result. CpG ODN also consistently showed affect on the pancreatic pathology. The productions of IFN gamma and IL-4 were detected only in the K and D type CpG ODN administration groups. Comparison of the two cytokines leads to the conclusion that CpG ODN generated a Th1-weighted response in both study groups. It was assumed that CpG ODN failed to produce Th2-weighted cytokine milieu, which can overcome the genetically determined phenotype of NOD mice. Given these results, it was concluded that the immunotherapeutic application of CpG ODN on Type 1 diabetes had clear limitations.
Animals ; DNA/*pharmacology ; Diabetes Mellitus, Type 1/*immunology/*prevention & control ; Female ; Mice ; Mice, Inbred NOD ; Th1 Cells/*immunology

A 76-year-old female admitted with nausea, anorexia, vague abdominal pain, and malaise. Her past medical history included an 15-year history of type 2 diabetes mellitus and hypertension. She had been taking metformin, glipizid, and amlodipine for past 2 years. One week previously, she underwent gastroscopy to evaluate epigastic pain, and she was diagnosed Helicobacter pylori positive duodenal ulcer, for which she was treated with amoxicillin, clarithromycin, and omeprazole. At admission, laboratory test revealed lactic acidosis (pH 7.23, bicarbonate 8.3 mEq/L, and lactate 5.51 mmol/L) and acute renal failure with a serum creatinine of 7.4 mg/dL. We diagnosed meformin-associated lactic acidosis and the patient made a complete recovery following therapy with bicarbonate-based hemodialysis and supportive care. It is the first report of metformin-associated lactic acidosis in Korea.
Abdominal Pain ; Acidosis, Lactic* ; Acute Kidney Injury ; Aged ; Amlodipine ; Amoxicillin ; Anorexia ; Clarithromycin ; Creatinine ; Diabetes Mellitus, Type 2 ; Duodenal Ulcer ; Female ; Gastroscopy ; Helicobacter pylori ; Humans ; Hypertension ; Korea ; Lactic Acid ; Metformin ; Nausea ; Omeprazole ; Renal Dialysis

Tracheopathia osteoplastica is a rare disease of unknown cause and characterized by cartilaginous or bony projection into the tracheobronchial lumen, usually not involved posterior membranous portion of tracheobronchial tree. In the past, most of the cases were diagnosed incidentally at autopsy. But after the introduction of bronchoscopy and computed tomography, antemortem diagnosis was reported. Because of initial presenting symptoms were indolent and non-specific, misdiagnosis was reported frequently and correct diagnosis was delayed usually. We report two cases of tracheopathia osteoplastica diagnosed by fiberoptic bronchoscopic biopsy.
Autopsy ; Biopsy ; Bronchoscopy ; Diagnosis ; Diagnostic Errors ; Rare Diseases