BACKGROUNDS: Though K-ADL was developed and validated, weighting each item of K-ADL is needed to evaluate the severity of functional disability as a whole. METHODS: Nominal group technique was used to weight individual items of K-ADL. Relevant experts were recruited; one specialist in geriatrics, one expert in elderly health service, one doctor expert in questionnarie, one nurse and two social welfare workers and one caregiver who work in nursing home for elderly, one nurse working at dementia care center. At the first round, each expert assigned the weights of each item. At the second round, those experts met, discussed, and re-evaluated each weighting. Using the newly developed weighted score, we measured its validity compared to brain-disability grade and three experts' decision of severe dysfunction. RESULTS: For inability to perform task, 7 points were given to 'dressing'and 'washing face', 6 points to 'bathing', 9 points to 'feeding', 8 points to 'transfer and toileting'. If any assistance is needed, 7 points were scored to 'dressing', 4 points were scored to 'washing face', 'feeding', 'transfer' and 'toileting', 2 points were scored to 'dressing'. Correlation coefficient between weighted K-ADL sum and brain-disability grade was -0.665(p=0.000). CONCLUSIONS: We assigned differential weighting to each of K-ADL and found that summing weighted K-ADL was excellent in some validity test.
Aged ; Caregivers ; Dementia ; Geriatrics ; Health Services ; Humans ; Nursing Homes ; Social Welfare ; Specialization ; Weights and Measures

Coenzyme Q therapy has been used to support metabolic derangements in patients with mitochondrial encephalomyopathies. Biochemical analysis of the living human brain can be performed by magnetic resonance spectroscopy (MRS). We report upon a KSS patient who was serially imaged with localized proton MRS to monitor the efficacy of CoQ treatment. A 17-year-old girl with KSS was serially imaged with localized proton MRS performed on a GE 1.5 T SIGNA MRI/MRS system. The elevated lactate contents of lesions decreased after one month of CoQ therapy but were re-elevated 10 months after treatment. We conclude that MRS presents us with a powerful tool for monitoring the effects of therapeutic trials in mitochondrial encephalomyopathies.
Adolescence ; Brain/metabolism ; Brain/drug effects ; Case Report ; Female ; Human ; Kearns Syndrome/metabolism* ; Kearns Syndrome/drug therapy* ; Kearns Syndrome/diagnosis ; Lactic Acid/metabolism ; Magnetic Resonance Spectroscopy/diagnostic use* ; Pyruvic Acid/metabolism* ; Treatment Outcome ; Ubiquinone/therapeutic use*

No abstract available.
Animals ; Mice* ; Oenanthe*

'Num-chin syndrome', isolated mental neuropathy, is a rare manifestaion of malignant hernatologic diseases, but important sign for early diagnosis and prediction of clinical course and prognosis. Here we report 7 cases of numb-chin syndrome; one in leukemic transformation of malignant lymphoma, one in blastic crisis of chronic myelogenous leukemia and 5 in acute leukemia Two cases of acute leukemia revealed the 'numb-chin sign' in early course of disease before the diagnosis of leukemia and other three in aggravating state of acute leukemia. The therapeutic response and prognosis was poor and 5 cases expired in a few months.
Diagnosis ; Early Diagnosis ; Hematologic Diseases* ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Lymphoma ; Prognosis

The goals of any new cervical cancer chemotherapy should include; a decrease in toxicity, better distant and local control of the disease, prolongation of survival, improvement in the quality of life and palliation of symptoms. The goal of FAC (5-Fluorouracil, Interferon alpha-2a, Carboplatin) neoadjuvant chemothe-raphy is for better surgical therapeutic results in locally advanced and bulky lesions with preo-perative chemotheraphy. This new trend in management of cervical cancer may provide the benefits as follows; reduction of the tumor size, a decrease in numbers of involved lymph nodes, control of microscopically metastatic lesions, improvement of the effects of radiation therapy and providing the chance of operability by lowering the clinical stage than initial prechemotherapy stage. The purpose of this study is to evaluate the effect of FAC neoadjuvant chemotherapy on reducing the size of tumors in cervical cancer. 17 patients in stage I b2, IIa, IIb carcinoma of cervix were treated with FAC regimen; Interferon alpha-2a 6 MIU given subcutaneously on day 1~6, 5-Fluorouracil 750 mg/m(2) given intravenously on day 2~6 and Carboplatin 350 mg/m2 given intravenously on 2nd day. The overall response rate was 58.5%, including 2 complete responses(11.7%) and 8 partial responses(47.1%). Neoadjuvant chemotherapy reduced the mean cervical lesion area from 23.1+9.97 cm(2) to 8.65+5.95 cm(2) in response group. The toxicity was acceptable in this group and the frequent toxicity was myelosuppression. Although limitation of this study are the lack of randomization and the small sample size, FAC neoadjuvant chemotheraphy is a potentially useful modality in the management of patients with locally advanced bulky cervical cancer.
Carboplatin ; Cervix Uteri ; Drug Therapy* ; Female ; Fluorouracil ; Humans ; Interferons ; Lymph Nodes ; Quality of Life ; Random Allocation ; Sample Size ; Uterine Cervical Neoplasms*

BACKGROUND AND PURPOSE: Chemokines participate in the regulation of immune and inflammatory responses by interacting with their receptors, which are primarily expressed on immune and inflammatory cells such as B- and T-lymphocytes and antigen-presenting cells. Chemokines and their receptors are therefore considered to mediate inflammation and tissue damage in autoimmune disorders. Chemokine receptor (CCR) genotypes were recently identified, and the importance of their genetic polymorphisms in some autoimmune and infectious disorders has been demonstrated. To define the roles of the polymorphism of the CCR2 gene at codon 64 (CCR2-64I) and the 32-bp deletion in the coding region of CCR5 (CCR5Delta32) in Korean patients with myasthenia gravis (MG), we compared these genotypes in MG cases and healthy controls and investigated the clinical features associated with these genotypes. METHODS: One hundred and fifteen healthy controls (51 men and 64 women) and 109 MG patients (44 men and 65 women) from three University hospitals were included. We examined each patient for clinical features using electrophysiology tests, laboratory tests, and thymic pathology. The CCR2-64I and CCR5Delta32 polymorphisms were determined by the PCR-RFLP method. RESULTS: We detected no difference in the frequencies of CCR2-64I polymorphism between MG patients and healthy controls. All of the MG patients and the healthy controls were homozygous for the wild-type CCR5 genotype. The results of electrophysiological tests and thymic pathologies were not influenced by the type of CCR2-64I polymorphism. However, the anti-acetylcholine-receptor (AChR) antibody titer was higher in the CCR2 G/G genotype (13.34+/-12.71 nmol/L) than in the CCR2 A/A genotype (5.83+/-2.56 nmol/L). CONCLUSIONS: We found no evidence of an increased risk for MG associated with the CCR2-64I and CCR5Delta32 polymorphisms. However, the increased anti-AChR antibody titer in the patients with the CCR2 G/G genotype suggests that the CCR2 gene play a role in the pathophysiology of MG.
Antigen-Presenting Cells ; Chemokines ; Clinical Coding ; Codon ; Electrophysiology ; Genotype ; Hospitals, University ; Humans ; Inflammation ; Male ; Myasthenia Gravis* ; Pathology ; Polymorphism, Genetic ; T-Lymphocytes

BACKGROUND AND PURPOSE: Chemokines participate in the regulation of immune and inflammatory responses by interacting with their receptors, which are primarily expressed on immune and inflammatory cells such as B- and T-lymphocytes and antigen-presenting cells. Chemokines and their receptors are therefore considered to mediate inflammation and tissue damage in autoimmune disorders. Chemokine receptor (CCR) genotypes were recently identified, and the importance of their genetic polymorphisms in some autoimmune and infectious disorders has been demonstrated. To define the roles of the polymorphism of the CCR2 gene at codon 64 (CCR2-64I) and the 32-bp deletion in the coding region of CCR5 (CCR5Delta32) in Korean patients with myasthenia gravis (MG), we compared these genotypes in MG cases and healthy controls and investigated the clinical features associated with these genotypes. METHODS: One hundred and fifteen healthy controls (51 men and 64 women) and 109 MG patients (44 men and 65 women) from three University hospitals were included. We examined each patient for clinical features using electrophysiology tests, laboratory tests, and thymic pathology. The CCR2-64I and CCR5Delta32 polymorphisms were determined by the PCR-RFLP method. RESULTS: We detected no difference in the frequencies of CCR2-64I polymorphism between MG patients and healthy controls. All of the MG patients and the healthy controls were homozygous for the wild-type CCR5 genotype. The results of electrophysiological tests and thymic pathologies were not influenced by the type of CCR2-64I polymorphism. However, the anti-acetylcholine-receptor (AChR) antibody titer was higher in the CCR2 G/G genotype (13.34+/-12.71 nmol/L) than in the CCR2 A/A genotype (5.83+/-2.56 nmol/L). CONCLUSIONS: We found no evidence of an increased risk for MG associated with the CCR2-64I and CCR5Delta32 polymorphisms. However, the increased anti-AChR antibody titer in the patients with the CCR2 G/G genotype suggests that the CCR2 gene play a role in the pathophysiology of MG.
Antigen-Presenting Cells ; Chemokines ; Clinical Coding ; Codon ; Electrophysiology ; Genotype ; Hospitals, University ; Humans ; Inflammation ; Male ; Myasthenia Gravis* ; Pathology ; Polymorphism, Genetic ; T-Lymphocytes

We report two cases of porphyric peripheral neuropathy in a 19-year-old male with variegate porphyria and in a 39 year-old male with intermittent acute prophyria. Clinically, there were sensory, motor disturbance and autonomic symptoms including decreased sweating, urinary and sphinctor distrubances. Variegate porphyria showed facial diplegia and positive family history inherited by autosomal dominent trait. Intermittent acute porphyria was combined-with SIADH. Both cases were expired due to respiratory failure. Nerve conduction studies were carried out in two cases and both cases showed slow motor, sensory nerve conductlon velocity ,and significant low CMAPs(Compound Muscle Action Potentials). Sural nerve biopsy was carried out in a variegate prophyria compared with one normal control. Decreased large myelinated fibers was found. In nerve fiber teased study. 8.5% of nerve fibers showed axonal degenration and only 2.3% of the segmental demyelination. There findings are suggesting that the porphyric neuro might be the axonal type.and severe neuropathy in a sign of poor prognosis.
Adult ; Axons ; Biopsy ; Demyelinating Diseases ; Humans ; Inappropriate ADH Syndrome ; Male ; Myelin Sheath ; Nerve Fibers ; Neural Conduction ; Peripheral Nervous System Diseases ; Porphyria, Acute Intermittent ; Porphyria, Variegate ; Prognosis ; Respiratory Insufficiency ; Sural Nerve ; Sweat ; Sweating ; Young Adult

Postpartum cerebral angiopathy is a benign, reversible clinicoradiologic syndrome. The clinical significance of this syndrome is the difficulty in differentiating it from other severe disorders such as subarachnoid hemorrhage and cere-bral vasculitis. A 25-year-old woman who developed a severe headache, transient total blindness ,and generalized tonic clonic seizures 14 days postpartum was admitted to our hospital. A MRI of the brain revealed a bilateral anterior cere-bral artery (ACA) infarction. A cerebral angiography showed the narrowing of the M1 and M2 segments of the left middle cerebral artery (MCA), the right MCA M1 segment, and the A1 and proximal A2 segments of the right ACA. Also, it showed the narrowing of the proximal basilar artery portion from which the anterior inferior cerebellar artey arose, P1, P2, and P3 segments of the left posterior cerebral artery (PCA), and the right PCA P1 segment. With the treatment of nimodipine, the abnormalities on these studies were markedly improved two weeks later.
Adult ; Arteries ; Basilar Artery ; Blindness ; Brain ; Cerebral Angiography ; Cerebrovascular Disorders ; Female ; Headache ; Humans ; Infarction ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Nimodipine ; Passive Cutaneous Anaphylaxis ; Posterior Cerebral Artery ; Postpartum Period* ; Pregnancy Complications ; Seizures ; Subarachnoid Hemorrhage ; Vasculitis

OBJECTIVE: Cervical cancer is the most common malignancy of the female genital tract in Korea. Recognized prognostic factors include FIGO stage, histologic grade, depth on invasion, primary cancer screening and age. However, prognosis of pregnant woman with cervical cancer is unclear. We reviewed our patients in an attempt to identify the best management options which resulted in long term survival for the mother. METHODS: We retrospectively investigated the results of 11 patients with CIS and cervical cancer in pregnant patients who had been diagnosed and treated at Soonchunhyang University Medical center from January 1990 to December 2002. RESULTS: The age range was 23-41 years. Six patients were CIS and performed LEEP conization during pregnancy. One patient had stage Ia disease and 3 patients had stage Ib with radical hysterectomy after Cesarean section. 4 cases were diagnosed as squamous cell cancer. One patient with stage Ib had been referred to our hospital at 28 gestational weeks. Another one patient with high risk histology (mucinous adenocarcinoma) pointed out polypectomy at 26 gestational weeks. One year later, the case died after treatment. All of 11 patients were treated surgically and two patients achieved pregnancy again with three live births. Preterm delivery after conization occured in one case at 34 gestational weeks. HPV infection was diagnosed in 82% of patients. The HPV type 16 was infected 4 patients, type 18 was one patient, type 58 was one patient, and mixed another type were 2 patients. CONCLUSION: We conclude that all pregnant women should have a Pap smear performed antenatally. Cone biopsy can be safely performed in pregnant women and may be adequate treatment for CIS. When cervical cancer complicates with pregnancy, decisions, management must include maternal and fetal consideration.
Academic Medical Centers ; Biopsy ; Carcinoma in Situ* ; Cesarean Section ; Conization ; Early Detection of Cancer ; Female ; Humans ; Hysterectomy ; Korea ; Live Birth ; Mothers ; Neoplasms, Squamous Cell ; Pregnancy* ; Pregnant Women ; Prognosis ; Retrospective Studies ; Uterine Cervical Neoplasms*