Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

No abstract available.

BACKGROUND: Elecsys 2010 immunoassay system is based on the electrochemiluminescence immunoassay using a ruthenium (II) tris (bipyridyl) label. Since it was the first time to use the system in our laboratory, we would like to evaluate the analytical performances (precision, linearity and recovery rate) and correlation with radioimmunoassay (RIA) and microparticle enzyme immunoassay (MEIA) methods. METHODS: We used precicontrol tumor marker (TM1, TM2) for alpha-fetoprotein (AFP), prostatic specific antigen (PSA) and carcinoembryonic antigen (CEA), Precicontrol universal (Ul, U2) for triiodothyronine (T3) and thyroxine (T4), Precicontrol-TSH for thyrotropin (TSH) and pooled serum for the evaluation of precision and recovery rate. Patients' sera were used for the linearity and comparison study. RESULTS: The coefficients of variatron of Imprecision study were below; 4.0%, 8.7% and 10.2%, respectively in the within-run, within-day and between-day analysis. The recovery rates were 100.5%, 96.1% and 102.5%, respectively in T4, TSH, and AFP. The linearity were y=1.02x-0.182(r=0.99) for T4, y=1.01x+0.12 (r=0.99) for TSH and y=1.01x+0.54(r=1.00) for AFP. T3, T4, TSH, CEA and PSA results showed good correlation with RIA (r>0.90), but AFP showed r=0.88. Also, AFP, CEA and PSA results showed excellent correlation with AxSYM (r>0.99). CONCLUSION: Elecsys 2010 immunoassay system showed excellent precision, recovery rate, clinically acceptable linearity and good correlation with the results obtained by RIA and MEIA methods.
alpha-Fetoproteins ; Carcinoembryonic Antigen ; Immunoassay* ; Immunoenzyme Techniques ; Radioimmunoassay ; Ruthenium ; Thyrotropin ; Thyroxine ; Triiodothyronine

PURPOSE: This exploratory study aims to identify various factors influencing the rehabilitation nursing competencies (RNC) of nurses in various types of health institutes. METHODS: The researcher developed a questionnaire consisting of 45 items based on the ARN rehabilitation nursing competency model. Subjects were 434 nurses working at general hospitals, long-term care facilities, or community health centers. RESULTS: Nurses' RNC were significantly higher among those who have higher levels of education, receive continuing education in rehabilitation nursing, and practice more frequently in rehabilitation nursing. Age and duration of nursing career were significant only for interprofessional care domain. General hospital nurses scored highest in every domain while nurses working at long-term care facilities scored lowest in every domain. Multiple regression analysis showed that practicing daily or more than twice per week in rehabilitation nursing, achieving master's degree or higher, and working at long-term care facilities were statistically significant factors with RNC. These factors explained 31.1% of the total variability in RNC in this sample. CONCLUSION: Currently, there is no official certification program for rehabilitation nurse practitioners in South Korea. The results of this study would be useful in developing RNC training programs for Korean nurses, and provide strong evidence for necessity of certified rehabilitation nurse specialists.
Academies and Institutes ; Certification ; Community Health Centers ; Education ; Education, Continuing ; Hospitals, General ; Humans ; Korea ; Long-Term Care ; Nurse Practitioners ; Nursing ; Rehabilitation Nursing* ; Rehabilitation* ; Specialization

PURPOSE: This study was tried to evaluate the potential benefits of concurrent chemoradiation therapy (low dose daily cisplatin combined with split course radiation therapy) compared with conventional radiation therapy alone in stage III non-small cell lung cancer. The end points of analyses were response rate, overall survival, survival without locoregional failure, survival without distant metastasis, prognostic factors affecting survival and treatment related toxicities. MATERIAL AND METHODS: Between April 1992 and March 1994, 32 patients who had stage III non-small cell lung cancer were treated with concurrent chemoradiation therapy. Radiation therapy for 2 weeks (300cGy given 10 times up to 3000cGy) followed by a 3 weeks rest period and then radiation therapy for 2 more weeks (250cGy given 10 times up to 2500cGy) was combined with 6mg/M2 of cisplatin. Follow-up period ranged from 13 months to 48 months with median of 24 months. Historical control group consisted of 32 patients who had stage III non-small cell lung cancer were received conventionally fractionated (daily 170-200cGy) radiation therapy alone. Total radiation dose ranged from 5580cGy to 7000cGy with median of 5940 cGy. Follow-up period ranged from 36 months to 105 months with median of 62 months. RESULTS: Complete reponse rate was higher in chemoradiation therapy (CRT) group than radiation therapy (RT) group (18.8% vs. 6.3%). CRT group showed lower in-field failure rate compared with RT group (25% vs. 47%). The overall survival rate had no significant differences in between CRT group and RT group (17.5% vs. 9.4% at 2 years). The survival without locoregional failure (16.5% vs. 5.3% at 2 years) and survival without distant metastasis (17% vs. 4.6% at 2 years) also had no significant differences. In subgroup analyses for patients with good performance status (Karnofsky performance scale 80), CRT group showed significantly higher overall survival rate compared with RT group (62.5% vs. 15.6% at 2 years). The prognostic factors affecting survival rate were performance status and pathologic subtype (squamous cell cancer vs. nonsquamous cell cancer) in CRT group. In RT alone group, performance status and stage (IIIa vs IIIb) were identified as a prognostic factors. RTOG/EORTC grade 2-3 nausea and vomiting (22% vs. 6%) and bone marrow toxicities (25% vs. 15.6%) were significantly higher in CRT group compared with RT alone group. The incidence of RTOG/EORTC grade 3-4 pulmonary toxicity had no significant differences in between CRT group and RT group (16% vs. 6%). The incidence of WHO grade 3-4 pulmonary fibrosis also had no significant differences in both group (38% vs. 25%). In analyses for relationship of field size and pulmonary toxicity, the patients who treated with field size beyond 200cm2 had significantly higher rates of pulmonary toxicities. CONCLUSION: The CRT group showed significantly higher local control rate than RT group. There were no significant differences of survival rate in between two groups. The subgroup of patients who had good performance status showed higher overall survival rate in CRT group than RT group. In spite of higher incidence of acute toxicities with concurrent chemoradiation therapy, the survival gain in subgroup of patients with good performance status were encouraging. CRT group showed higher rate of early death within 1 year, higher 2 year survival rate compared with RT group. Therefore, to evaluate the accurate effect on survival of concurrent chemoradiation therapy, systematic follow-up for long term survivors are needed.
Bone Marrow ; Carcinoma, Non-Small-Cell Lung* ; Cisplatin ; Follow-Up Studies ; Humans ; Incidence ; Nausea ; Neoplasm Metastasis ; Pulmonary Fibrosis ; Survival Rate ; Survivors ; Vomiting

A rigid external distraction device has been used for maxillary distraction osteogenesis and a number of papers have reported midface distraction after using this system. However, there is not enough information about long-term stability or further growth of the maxilla in adolescences after distraction. The purpose of this study was to evaluate the long-term stability of maxillary distraction osteogenesis by use of a rigid external distraction device. A total of 9 patients with severe cleft maxillary hypoplasia were treated between January 1998 and August 2003. The patients' ages at the time of surgery ranged between 13 and 19 years. The distraction started at five days after a Le Fort I osteotomy at a rate of 1mm per day for 10 to 15 days. All patients used the RED(rigid external distraction) I system. After distraction was completed, the device was left in place for another five to six weeks for bony consolidation. When this was completed, an orthodontic face mask was used with elastic traction for five to six weeks. The follow-up period ranged from one to six years. The mean distraction length was 13.6mm for an immediate postdistraction, 10.8mm at six months after distraction, and 10.4mm between the one year and six year follow-up period, resulting in relapse rate of 23.0%. Regarding three children with mixed dentition, the ANBangle ranged between 7.1 degrees to 8.5 degrees at immediate postdistraction, 2.8 degree to 4.0 degrees at the six- month postoperation period, and 0.4 degree to 1 degree at a five-year postoperation. Therefore, the growth rate of the distracted maxilla was lower than that of the mandible, respectively, in those three children. Our results suggested that greater anterior overcorrection of the hypoplastic maxilla is needed more in the growing child than in adults in order to compensate for a partial relapse and growth deficit.
Adult ; Child ; Dentition, Mixed ; Follow-Up Studies* ; Humans ; Mandible ; Masks ; Maxilla* ; Osteogenesis, Distraction ; Osteotomy ; Recurrence ; Traction

Nitric oxide (NO) production by endothelial nitric oxide synthase (eNOS) plays a protective role in cerebral ischemia by maintaining vascular permeability, whereas NO derived from neuronal and inducible NOS is neurotoxic and can participate in neuronal damage occurring in ischemia. Matrix metalloproteinases (MMPs) are up-regulated by ischemic injury and degrade the basement membrane if brain vessels to promote cell death and tissue injury. We previously reported that agmatine, synthesized from L-arginine by arginine decarboxylase (ADC) which is expressed in endothelial cells, has shown a direct increased eNOS expression and decreased MMPs expression in bEnd3 cells. But, there are few reports about the regulation of eNOS by agmatine in ischemic animal model. In the present study, we examined the expression of eNOS and MMPs by agmatine treatment after transient global ischemia in vivo. Global ischemia was induced with four vessel occlusion (4-VO) and agmatine (100 mg/kg) was administered intraperitoneally at the onset of reperfusion. The animals were euthanized at 6 and 24 hours after global ischemia and prepared for other analysis. Global ischemia led severe neuronal damage in the rat hippocampus and cerebral cortex, but agmatine treatment protected neurons from ischemic injury. Moreover, the level and expression of eNOS was increased by agmatine treatment, whereas inducible NOS (iNOS) and MMP-9 protein expressions were decreased in the brain. These results suggest that agmatine protects microvessels in the brain by activation eNOS as well as reduces extracellular matrix degradation during the early phase of ischemic insult.
Agmatine ; Animals ; Arginine ; Basement Membrane ; Brain ; Brain Ischemia ; Capillary Permeability ; Carboxy-Lyases ; Cell Death ; Cerebral Cortex ; Endothelial Cells ; Extracellular Matrix ; Glycosaminoglycans ; Hippocampus ; Ischemia ; Matrix Metalloproteinases ; Microvessels ; Models, Animal ; Neurons ; Nitric Oxide ; Nitric Oxide Synthase Type III ; Rats ; Reperfusion

Cerebral amyloid angiopathy (CAA) is common in patients with Alzheimer's disease (AD) and may contribute to cerebral hemorrhage. We previously demonstrated that tissue plasminogen activator (tPA) and plasminogen (PLG) accumulated at the periphery of compact amyloid-cored plaques and in the walls of CAA-containing blood vessels in the brains of Tg2576 mice, a widely used AD mouse model. We had also observed that zinc-triggered tPA and PLG induction were observed in mouse cortical cultures. Because zinc also accumulates in amyloid plaques and blood vessel walls in AD brains, we examined whether zinc increases mRNA and protein levels of tPA and PLG in brain endothelial cells and pericytes. Four hours after the exposure of brain endothelial cells (bEnd.3) to 40 microM zinc, the mRNA and protein expressions of tPA and its substrate PLG were significantly increased. In the case of brain pericyte cultures, increases in tPA and PLG expression were also detected 2 hr after treatment. However, amyloid-beta (Abeta)1-42 oligomers did not augment tPA and PLG expression in bEnd.3 cells and pericytes, suggesting that zinc but not Abeta induces tPA and PLG accumulation in CAA found in the AD brain.
Alzheimer Disease ; Animals ; Blood Vessels ; Brain ; Cerebral Amyloid Angiopathy ; Cerebral Hemorrhage ; Endothelial Cells* ; Humans ; Mice ; Pericytes* ; Plaque, Amyloid ; Plasminogen* ; RNA, Messenger ; Tissue Plasminogen Activator* ; Zinc

BACKGROUND: Clinical manifestations and complications of measles are more severe in adults than children. But, there has been few clinical and epidemiologic data about adult measles in Korea. OBJECTIVE: We investigated clinical features, laboratory findings, disease courses and complications and discussed the reason why measles incidence has been increasing recently in young adults to find out the solutions to prevent measles. METHOD: From April 1999 to February 2001, we examined prodromal symptoms, skin rash, complications, routine CBC, and blood chemistry of 42 adults who were admitted in KangBuk SamSung Hospital for measles and performed qualitative serum measles specific IgM and IgG antibody test using ELISA method. RESULTS: Age distribution of most adult measle patients was between 15 and 25 years old(78.6%). Fever was found in all patients and cough, coryza, headache, myalgia, conjunctivitis, sore throat, nausea, diarrhea, and vomit were found in decreasing order of frequency. Skin rashes were started from the face and spread to trunk, arms and then legs in 37 patients out of 42. Five patients had skin rash in the face and trunk at the same time, and one had hemorrhagic rash. Koplik's spots were found in 26 patients (62%). Among them, 2 had Koplik's spots before the rash and 24 had Koplik's spots with the rash simultaneously or thereafter. Koplik's spots were disappeared as skin rash disappeared. Exfoliative dermatitis was found following the skin rash in one patient. Among 26 patients who performed qualitiative serum measles specific IgM and IgG antibody test using ELISA, the seropositivity of measles specific IgM was 70%, of measles specific IgG was 27%, and of both measles specific IgM and IgG was 12%. Complications were like this; hepatitis in 23, cervical lympadenitis in 20, laryngitis and bronchitis in 7, otitis media in 4, pneumonia on chest X-ray in 3, hepatosplenomegaly on upper abdomen ultrasonography in 5. Two patients were in pregnancy of 12 and 16 weeks, and the former had spontaneous abortion. Pnemonia and otitis media were recovered within average 5 days after administrating antibiotics and antipyretics.
Abdomen ; Abortion, Spontaneous ; Adult* ; Age Distribution ; Anti-Bacterial Agents ; Antipyretics ; Arm ; Bronchitis ; Chemistry ; Child ; Conjunctivitis ; Cough ; Dermatitis, Exfoliative ; Diarrhea ; Enzyme-Linked Immunosorbent Assay ; Epidemiologic Studies* ; Exanthema ; Female ; Fever ; Headache ; Hepatitis ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Incidence ; Korea ; Laryngitis ; Leg ; Measles* ; Myalgia ; Nausea ; Otitis Media ; Pharyngitis ; Pneumonia ; Pregnancy ; Prodromal Symptoms ; Thorax ; Ultrasonography ; Young Adult

BACKGROUND: Clinical manifestations and complications of measles are more severe in adults than children. But, there has been few clinical and epidemiologic data about adult measles in Korea. OBJECTIVE: We investigated clinical features, laboratory findings, disease courses and complications and discussed the reason why measles incidence has been increasing recently in young adults to find out the solutions to prevent measles. METHOD: From April 1999 to February 2001, we examined prodromal symptoms, skin rash, complications, routine CBC, and blood chemistry of 42 adults who were admitted in KangBuk SamSung Hospital for measles and performed qualitative serum measles specific IgM and IgG antibody test using ELISA method. RESULTS: Age distribution of most adult measle patients was between 15 and 25 years old(78.6%). Fever was found in all patients and cough, coryza, headache, myalgia, conjunctivitis, sore throat, nausea, diarrhea, and vomit were found in decreasing order of frequency. Skin rashes were started from the face and spread to trunk, arms and then legs in 37 patients out of 42. Five patients had skin rash in the face and trunk at the same time, and one had hemorrhagic rash. Koplik's spots were found in 26 patients (62%). Among them, 2 had Koplik's spots before the rash and 24 had Koplik's spots with the rash simultaneously or thereafter. Koplik's spots were disappeared as skin rash disappeared. Exfoliative dermatitis was found following the skin rash in one patient. Among 26 patients who performed qualitiative serum measles specific IgM and IgG antibody test using ELISA, the seropositivity of measles specific IgM was 70%, of measles specific IgG was 27%, and of both measles specific IgM and IgG was 12%. Complications were like this; hepatitis in 23, cervical lympadenitis in 20, laryngitis and bronchitis in 7, otitis media in 4, pneumonia on chest X-ray in 3, hepatosplenomegaly on upper abdomen ultrasonography in 5. Two patients were in pregnancy of 12 and 16 weeks, and the former had spontaneous abortion. Pnemonia and otitis media were recovered within average 5 days after administrating antibiotics and antipyretics.
Abdomen ; Abortion, Spontaneous ; Adult* ; Age Distribution ; Anti-Bacterial Agents ; Antipyretics ; Arm ; Bronchitis ; Chemistry ; Child ; Conjunctivitis ; Cough ; Dermatitis, Exfoliative ; Diarrhea ; Enzyme-Linked Immunosorbent Assay ; Epidemiologic Studies* ; Exanthema ; Female ; Fever ; Headache ; Hepatitis ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Incidence ; Korea ; Laryngitis ; Leg ; Measles* ; Myalgia ; Nausea ; Otitis Media ; Pharyngitis ; Pneumonia ; Pregnancy ; Prodromal Symptoms ; Thorax ; Ultrasonography ; Young Adult