These are 2 cases report of the Characot-Marie-Tooth disease. One patient is a 21 year-old female with gait ataxia, foot drop, muscular atrophy in the both feet, pes cavus and equinovarus. On family history, her older and younger brothers and younger sister showed same symptoms and signs which is rather milder. On neurologic examination, there were atrophy, high arched feet, and steppage gait. There were decreased pain, temperature and touch sensation, and absence of position and vibration, and absence of DTR in lower extremities. On sensory nerve conduction velocity study, the amplitude of evoked action potential is very low. On motor NCV, there are markedly prolonged distal latency and markedly decreased conduction velocity. On EMG study, there are dencervated potentials at rest and reduced interference pattern at maximal contraction. The other patient is 62 year-old female with gait disturbance, foot drop, muscular atrophy in the both feet, pes cavus and equinovarus. On family history, her father, 2 younger brothers and her 3 nephews showed same degree or rather mild degree of symptoms and signs. The findings on physical, neurologic examination and EMG with NCV study are compatible with Charcot-Marie-Tooth disease.
Action Potentials ; Atrophy ; Charcot-Marie-Tooth Disease* ; Clubfoot ; Fathers ; Female ; Foot ; Foot Deformities ; Gait ; Gait Ataxia ; Humans ; Lower Extremity ; Middle Aged ; Muscular Atrophy ; Neural Conduction ; Neurologic Examination ; Sensation ; Siblings ; Vibration ; Young Adult

To clarify the mechanism of the protective effect of hemodialysis on lipid peroxidation in RBC membrane structures, the level of malondialdehyde (MDA) which is the lipid peroxidation product, and the activities of antioxidant enzymes such as superoxide dismutase (SOD), catalase, and glutathione peroxidase (GSH-Px) were determined before and after hemodialysis in the RBCs from 20 patients with end stage renal disease (ESRD), and from 14 healthy subjects. Before dialysis, MDA levels in the RBCs from the patients with ESRD were higher than those from healthy controls. SOD and catalase activities in the RBCs were lower. After hemodialysis, MDA, SOD, and catalase in the RBCs from the patients with ESRD were normalized. These results indicate that hemodialysis treatment is helpful to protect the peroxidative darnage through normalizing the activities of antioxidant enzymes.
Catalase ; Dialysis ; Erythrocytes* ; Glutathione Peroxidase ; Humans ; Kidney Failure, Chronic* ; Lipid Peroxidation ; Malondialdehyde* ; Membranes ; Renal Dialysis* ; Superoxide Dismutase

Forty six cases of Legg-Calve-Perthes disease were reviewed clinically and roentgenographically in order to determine a satisfactory method of assessing the prognosis and to correlate the clinical and roentgenographical results. 1. The patients were children 3 to 12 years of age, with the peak at 6 years. The average age of visit (or diagnosis) was 6.7 years and that of onset was 6.4 years. 2. The total number of hips involved were 49, three cases being bilateral (6. 5%). Boys predominated girls by a ratio of 4.7:1 3. End results in children below 6 years of age were better than those in children above 6 years. The end results of treatment with the containment method were better than those with the noncontainment method. 4. The hips with excellent result had an average of 2.5mm. of uncovering of the femoral head at the time of diagnosis. The degree of uncovering increased as the roentgenographical result deteriorated, in the poor hips the average uncovering being 9mm. The average for the normal side was 2mm. The uncovering at onset is an accurate guide to the likely end result. 5. The Catterall assessment was of reliable prognostic value. In the excellent group, the majority of the hips belonged to Groups 1 and 2, In the poor group, there were no hips in Group 1 and increasing number of hips belonged to Groups 2,3 and 4. 6. The head at risk judged by Gage's sign, calcification of lateral epiphysis, lateral subluxation of the femoral head, and presence of a horizontal epiphyseal line indicated poor prognosis. 7. There was a close correlation between the severity of clinical features and the severity of residual roentgenographic findings.
Child ; Clinical Study ; Containment of Biohazards ; Diagnosis ; Epiphyses ; Female ; Head ; Hip ; Humans ; Legg-Calve-Perthes Disease ; Methods ; Prognosis

The tibial condylar fractures involving the articular surface can produce some disability of the knee joint because it is frequently accompanied by soft tissue injury to the ligaments and menisci. Accurate anatomical reduction and rigid internal fixation with early motion is known to decrease the complications. During a period of 10 years, from January 1978 to December 1987, we treated 105 tibial condylar fractures at Severance Hospital. Of the above, 77 cases have been analyzed according to the classification, cause of injury, method of treatment, final result and complication. The 77 cases were classified as follows ; total condylar depression 17(22%), undisplaced 16 (21 %), split compression 16(21 %), comminuted 14 (18%), local compression 5 (7%), split 1 (1%) and others 8(10%). Among these, 59 cases revealed the result of “ACCEPTABLE” according to Porter's criteria. Of the conservative group, 86% obtained the rating of “ACCEPTABLE” and 67% of the operative group did as well. Possible complications include traumatic arthritis (9), limited motion (9), wound infection (6), instability (3), angular deformity (2), intraarticular loose body (2), nonunion (1) and myositis ossificans.
Arthritis ; Classification ; Clinical Study ; Congenital Abnormalities ; Depression ; Knee Joint ; Ligaments ; Methods ; Myositis Ossificans ; Soft Tissue Injuries ; Wound Infection

OBJECTIVE: Recently, there are many reports that glutamate receptors have close relationships with a pathophysiology of schizophrenia. The purpose of this study was to assess the effects of D-cycloserine, which is glycine site partial agonist in NMDA receptor on psychopathologic symptoms and cognitive functions. METHODS: This study was done for chronic schizophrenic inpatients taking typical antipsychotics for more than 4 months. Exclusion criteria were patients with over 8 points according to Simpson-Angus scale for EPS or those with over 17 points of Hamilton Depression Scale. Patients were randomized to classify into two groups; D-cycloserine group (n=13) and placebo group (n=13). Each group received D-cycloserine 100 mg or placebo separately for 8 weeks. Psychopathology was evaluated with PANSS at baseline, 2nd week, fourth week and eighth week. Cognitive function was evaluated with KWIS at baseline and eighth week. RESULTS: Total 26 patients completed this trial. The average period of morbidity was 10.39+/-3.87 years and the average doses of antipsychotic was 1228.35+/-720.30 mg based on chlorpromazine equivalent. In positive subscale, negative subscale, general psychopathology subscale, total PANSS scale and KWIS, there were no significant differences between D-cycloserine and placebo groups. However, negative subscale scores had decreased from 24.92+/-3.64 (Baseline) to 23.46+/-3.41 (week 8) (p=0.077). CONCLUSION: There were no clear changes in positive symptom, negative symptom, memory, language function, and performance intelligence when D-cycloserine 100 mg was given with antipsychotic medication. However, some patients showed clear improvement in negative symptom, especially blunted affect. Therefore, D-cycloserine combination therapy could be effective for negative symptom. In future, study that can show effectiveness in psychopathology and cognitive function according to drug dosage is needed.
Antipsychotic Agents ; Chlorpromazine ; Cognition* ; Depression ; Glycine ; Humans ; Inpatients ; Intelligence ; Memory ; N-Methylaspartate ; Psychopathology ; Receptors, Glutamate ; Schizophrenia*

No abstract available.
Acute Kidney Injury* ; Edema* ; Hemofiltration*

No abstract available.
Acute Kidney Injury* ; Edema* ; Hemofiltration*

BACKGROUND: The serum should be tested with a platelet panel for identification of platelet specific alloantibodies. Such platelet panels are not available from commercial sources and can usually be made using platelets from local donor population. We prepared the platelet panel by DNA genotyping for 5 major platelet specific antigens and evaluated the detection ability of panel with clinical samples from patients showing the refractoriness to platelet transfusion. METHODS: DNA genotyping of five major platelet specific alloantigens (PlA, Ko, Bak, Pen, Br) was performed for ninety three donors by reverse dot blot hybridization technique. For the evaluation of the panel we prepared, we used the antiplatelet antibody positive sera detected by modified antigen capture ELISA. RESULTS: The most frequently encountered genotypes of platelets are PlA1/PlA1, Kob/Kob, Baka/Bakb, Pena/Pena, Brb/Brb (36% of ninety three donor platelets tested). PlA2 and Penb alleles were not identified in this study. Two cases of anti-Koa were identified using panel we prepared. CONCLUSION: The genotyping of platelet alloantigens circumvented the limitation of immunophenotyping by the general lack of quality typing antisera. It is impossible to make a good panel which was composed entirely of five major platelet specific alloantigen systems because the PlA2, Penb, and Bra are very rare alleles in Koreans. But our panel can be used for the identification of antibodies against Ko and/or Bak platelet antigen in patients with platelet alloimmunization.
Alleles ; Antibodies ; Antigens, Human Platelet ; Blood Platelets* ; DNA* ; Enzyme-Linked Immunosorbent Assay ; Genotype ; Humans ; Immune Sera ; Immunophenotyping ; Isoantibodies ; Isoantigens ; Platelet Transfusion ; Tissue Donors

BACKGROUND/AIMS: Pancreaticoduodenectomy is the procedure of choice in patients with periampullary cancers. Marked improvements in morbidity and mortality rates following pancreaticoduodenectomy have been reported in recent years. However, pancreatic leakage still occurs in 5% to 25% of patients and is a major cause of morbidity and mortality. METHODS: Between January 1990 to June 1999, eighty-two patients underwent pancreaticoduodenectomy. We compared preoperative, intraoperative characteristics as well as postoperative sandostatin usage in those patients who experienced (n=21) versus those who did not experience pancreatic leakage (n=61). Information was retrospectively collected from hospital record. RESULTS: The clinical leakage rate in this series was 25.6% (n=21). There were no significant differences in preoperative characteristics comparing those with versus those without pancreatic leakage. Among intraoperative characteristics, duct stenting and transfusions were significantly associated with the pancreatic leakage. Finally, postopeative sandostatin usage was able to reduce significantly the incidence of pancreatic leakage. CONCLUSION: Although consensus among surgeons does not exist as to opeative tenchnique and postoperative management, stenting and less bleeding as well as sandostatin usage had better results in this study.
Consensus ; Hemorrhage ; Hospital Records ; Humans ; Incidence ; Mortality ; Octreotide ; Pancreatic Fistula ; Pancreaticoduodenectomy* ; Retrospective Studies ; Stents

The pattern reversal visual evoked potential(PRVEP) and flash electroretinogram(flash ERG) were performed in 22 patients with optic atrophy. Patients with ophthalmologic problems other than optic atrophy or with systemic disorders were excluded from the analysis The results are as follows: 1. In the 41 eyes of patients with optic atrophy, 39 of them showed abnormal PRVEP, in which all the eyes had no consistent waveform except in one patient 2 eyes with delayed P1 latency. 2. 13 eyes were abnormal in both PRVEP and flash ERG but no eye was abnomnal in flash ERG only 3, Regarding the flash ERG examination, 13 eyes were abnomlal. Of these, there was a period of 1 to 2 years for 1 eye's disease, a period of 2 to 5 years for another eye' disease and after 5 years 11 eyes were diseased. Therefore, it showed that the longer the duration of disease lasted, the more flash ERG abnormalities developed. 4. The abnormalities of PRVEP haxe no significant relationship with the duration of the disease.
Evoked Potentials, Visual* ; Humans ; Optic Atrophy*