Purpose : HUS usually occurs in children after infection with shiga toxin-producing microorganism(D+HUS). In contrast, non-postdiarrheal(D-) HUS occurs at any age and has a high rate of relapse and a poor prognosis. The clinical presentation of D-HUS is similar to that of thrombotic thrombocytopenic purpura(TTP). Recently severe deficiencies of ADAMTS13 were reported not only in TTP and D- HUS but also in D+ HUS during their acute phase. The purpose of the study is to evaluate the plasma ADAMTS13 activity in D+ and D-HUS. Methods : Nineteen children with HUS(D+ HUS 12 and D- HUS 7) were enrolled. The assays of plasma ADAMTS13 activity were performed during the acute stage in the D+ HUS and at various stages of relapsing courses in the D- HUS patients by multimer assay, based on electrophoresis. Results : The median plasma activity of ADAMTS13 in D+ HUS and D- HUS were 80.9%(37.8-132.4%) and 53.9%(1.0-94.1%), respectively, which were not statistically significantly different from control(86.4%, 34.2-112.3%)(P>0.05). One boy with D- HUS had severe deficiency of ADAMTS13(1.0%). His platelet count was normalized temporarily by fresh frozen plasma infusion. Conclusion : We have demonstrated that there is no significant difference of the plasma ADAMTS13 activity between D+ HUS, D- HUS and control. We detected severe deficiency of ADAMTS13 in one boy who presented with relapsing episodes of D- HUS. ADAMTS13 deficiency should be considered in the subgroup of D- HUS especially with early onset and recurrent courses. Plasma therapy can be beneficial in this subgroup.
Objective : The authors tried to reveal some unique features of lipomeningomyelocele (LMMC), including clinical presentation, factors precipitating onset of symptoms, pathologic entities of LMMC associated with tethered cord syndrome, and surgical outcome in LMMC patients. Methods : Seventy-five patients with LMMC were enrolled in this study. Neuro-imaging and intraoperative findings allowed classification of LMMC into three Types. The patients were divided into two groups by age : A (51 patients), from birth to 3 years, and B (24 patients), from 3 to 24 years. For prevention of retethering of the cord, a mega-dural sac rebuilding procedure was performed in 15 patients. Results : During a mean postoperative follow-up period of 4 years, the surgical outcome was satisfactory in terms of improved pain and motor weakness, but disappointing with reference to the resolution of bowel and bladder dysfunction. Among these 75 patients with LMMC, preoperative deficits were improved after surgery in 29 (39%), remained stable in 28 (37%), changed slightly in 13 (17%), and worsened in 5 (7%). Patients in group A achieved better outcomes than those in group B. Depending on the type of lesion, patients with types I and II LMMC have better outcomes than those with type III LMMC. Finally, retethering of the cord with neurological deterioration occurred in 4 (5.3%) of the 75 patients, but no retethering was found in the 15 patients who were recently treated with a mega-dural sac rebuilding procedure. Conclusion : Our data continue to support the opinion that early diagnosis and optimal surgery are still essential for the treatment of patients with LMMC, since there is a high likelihood of residual neurological functions that can be preserved. Based on our surgical experience of untethering and decompression of lipomas, a mega-dural sac repair is useful to prevent retethering of the cord.
Background: The advent of EEA stapler has lowered the leakage rate of esophagogastric anastomoses and thereby contributed to a decrease in the operative mortality of the easophageal resection. Recent surgical reports, however, have documented 10% to 20% prevalence of benign anastomotic stricture formation after the use of EEA stapler to construct an esophagogastric anastomosis. We analyzed the cases of anastomotic strictures to reduce the incidence of anastomotic strictures with EEA stapled esophagogastrostomy. Material and Method: EEA stapled esophagogastrostomy was performed in 195 parients during the period of over 11 years from Jan. 1986 to Dec. 1996 in Kosin Medical Center. Ten patients of them died in the early postoperative days. In the remaining 185 patients, we studied the incidence and the onset time of anastomotic strictures, relationship between the patients' ages, the anastomotic sites, and the size of the cartridges with incidence of anastomotic stricture. We also studied the method of treatment and its effect in the anastomotic strictures. Result: Benign anastomotic strictures occurred in 39 cases among 185 patients(21%), 25 cases(64.1%) of the 39 cases developed in one to three months postoperatively. The patients' ages and the anastomotic sites did not effect with the incidence of anastomotic stricture, but high incidence of anastomotic stricture in EEA stapled esophagogastrostomy(p=0.04)was observed in small cartridge sizes. One or two balloon dilatation(89%) relieved the anastomotic strictures. Conclusion: We conclude that a larger size cartridge is recommended in EEA stapled esophagogastrostomy to reduce the incidence of anastomotic stricture if possible, and one or two balloon dilatation would seem to be a safe and reliable method in treating anastomotic stricture when the anastomotic stricture was occurrs.
Background: The conventional method of aortic cross-clamping is very difficult and increases the risk of cerebral infarct due to embolism of the calcified aorta in these patients. Accordingly, we analyzed our experience with 11 cases of ascending aorta and aortic valve replacement with hypothermic circulatory arrest. Materials and Methods: From January 2002 to December 2009, 11 patients had ascending aorta and aortic valve replacement with hypothermic arrest at our hospital. We performed a retrospective study. Results: There were 5 males and 6 females, with a mean age of 68 years (range, 44 to 82 years). Eight patients had aortic stenosis, and 3 patients had aortic regurgitation. An aortic cannula was inserted into the right axillary artery in 3 patients and ascending aorta in 6 patients. Two patients with aortic regurgitation had a remote access perfusion catheter inserted though the right femoral artery. The mean cardiopulmonary bypass time was 180 minutes (range, 110 to 306 minutes) and mean hypothermic circulatory arrest time was 30 minutes (range, 20 to 48 minutes). The mean rectal temperature during hypothermic circulatory arrest was $21^{\circ}C$ (range, $19^{\circ}C$ to $23^{\circ}C$). No patient had any new onset of cerebral infarct or cardiovascular accident after surgery. There was no hospital mortality. Early complications occurred in 1 patient who needed reoperation due to postoperative bleeding. Late complications occurred in 1 patient who underwent a Bentall operation due to prosthetic valve endocarditis. The mean follow-up duration was 32 months (range, 1 month to 8 years) and 1 patient died suddenly due to unknown causes after 5 years. Conclusion: Patients with a calcified aorta can be safely treated with a technique based on aorta and aortic valve replacement under hypothermic circulatory arrest.
The 234 patients who received treatment of midfacial fractures at the Department of Oral and Maxillofacial Surgery of Chonnam University Hospital from Jan. 1, 1992 to Dec. 31, 1996 were analyzed clinicostastically. Results obtained were as follows ; Male predominated over females by a ratio of 5.3 to 1. The frequently developing age groups were first 3rd (25%), 4th (21%) and the 2nd (18%) decade on succession. The peakest month was the August (16%), and May (11%), September (9%), October (9%). When it comes to the reasons for in-patients, traffic accident was predominant to 38%. In the 234 cases of midfacial fractures, zygomaticomaxillary complex fracture was the most by 37%. The most common with injury show that facial laceration marked by 49%, neurologic injury 24%, and mandibular fracture 20% each by each. About the time from injury onset to operation, 55% of cases were less than a week while the others (45%) more than a week. 3 plates were used for operation : 2 for zygoma or maxilla fracture and 3 for zygomatiomaxillary complex fracture. especially 4 for Le Fort I fracture, 5.5 for Le Fort I, II ; I, III ; II, III fracture, 7 for Le Fort I,II,III fracture were used. 20 patients (8%) appealed their complication and the most common was reported as infection. Above results suggest that early diagnosis and treatment of fracture site, systemic condition and associated injuries are necessary, and coorperative treatment with medical department should be performed.
New generation of tall and complex buildings systems are now introduced that are reflective of the latest development in materials, design, sustainability, construction, and IT technologies. While the complexity in design is being overcome by the availability and advances in structural analysis tools and readily advanced software, the design of these buildings are still reliant on minimum code requirements that yet to be validated in full scale. The involvement of the author in the design and construction planning of Burj Khalifa since its inception until its completion prompted the author to conceptually develop an extensive survey and real-time structural health monitoring program to validate all the fundamental assumptions mad for the design and construction planning of the tower. The Burj Khalifa Project is the tallest structure ever built by man; the tower is 828 meters tall and comprises of 162 floors above grade and 3 basement levels. Early integration of aerodynamic shaping and wind engineering played a major role in the architectural massing and design of this multi-use tower, where mitigating and taming the dynamic wind effects was one of the most important design criteria established at the onset of the project design. Understanding the structural and foundation system behaviors of the tower are the key fundamental drivers for the development and execution of a state-of-the-art survey and structural health monitoring (SHM) programs. Therefore, the focus of this paper is to discuss the execution of the survey and real-time structural health monitoring programs to confirm the structural behavioral response of the tower during construction stage and during its service life; the monitoring programs included 1) monitoring the tower's foundation system, 2) monitoring the foundation settlement, 3) measuring the strains of the tower vertical elements, 4) measuring the wall and column vertical shortening due to elastic, shrinkage and creep effects, 5) measuring the lateral displacement of the tower under its own gravity loads (including asymmetrical effects) resulting from immediate elastic and long term creep effects, 6) measuring the building lateral movements and dynamic characteristic in real time during construction, 7) measuring the building displacements, accelerations, dynamic characteristics, and structural behavior in real time under building permanent conditions, 8) and monitoring the Pinnacle dynamic behavior and fatigue characteristics. This extensive SHM program has resulted in extensive insight into the structural response of the tower, allowed control the construction process, allowed for the evaluation of the structural response in effective and immediate manner and it allowed for immediate correlation between the measured and the predicted behavior. The survey and SHM programs developed for Burj Khalifa will with no doubt pioneer the use of new survey techniques and the execution of new SHM program concepts as part of the fundamental design of building structures. Moreover, this survey and SHM programs will be benchmarked as a model for the development of future generation of SHM programs for all critical and essential facilities, however, but with much improved devices and technologies, which are now being considered by the author for another tall and complex building development, that is presently under construction.
Journal of the korean academy of Pediatric Dentistry
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v.41
no.1
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pp.80-84
/
2014
Intractable ulcerating enterocolitis is an uncommon inflammatory bowel disease syndrome of neonatal onset first described in 1991. Intractable ulcerating enterocolitis usually presents in the neonate with a mouth ulceration and the subsequent development of perianal disease and colitis. In this case report, an infant, 18 days from birth, with ulcerative lesion on hard palate for systemic differential diagnosis about oral lesion is referred from the department depiatrics. At that time, there is no abnormality, except oral lesion-like Aphthous ulcer. The patient was discharged from pediatrics, but returned to the hospital 3weeks later with blood diarrhea. As a result of endoscopy, there were large ulcerating lesions and the patient was diagnosed intractable ulcerating enterocolitis. Early recognition of Intractable ulcerating enterocolitis appears to be beneficial because colectomy, as opposed to immunosuppression, appears to be effective in controlling disease symptoms and progression. Most of the infants who were affected intractable ulcerating enterocolitis were normal at birth and oral manifestation appeared earlier than others. So, it is very meaningful for dentists to know about Intractable ulcerating enterocolitis.
Respiratory failure is the most serious manifestation and usual cause of death in acute organophosphate poisoning, and is common in acute cholinergic crisis. But the respiratory failure may appear suddenly in a patient who is recovering from the cholinergic crisis, even while receiving conventional therapy. These are case report of 37 years old male and 24 years old female with intermediate syndrome in organophosphate poisoning. The two patients ingested organophosphate(fenthion and mixture of DDVP with chlorpyrifos respectively) incidentally and in a sucide attempt respectively. After apparent recovery from the cholinergic crisis with a conventional therapy but before the expected onset of delayed polyneuropathy, the respiratory failure appeared suddenly with a muscular weakness, affecting predominantly the proximal limb muscles, neck flexors, territories of several motor cranial nerves. The two patients needed mechanical ventilatory support and recovery from the intermediate syndrome was complete in both patients, although one subsequently developed hypoxic encephalopathy. The clinical manifestation and electrophysiologic study support the clinical diagnosis of intermediate syndrome. The syndrome carries a risk of death. because of respiratory paralysis, if not recognized early and treated adequatedly. Prompt endotrachial intubation and mechanical ventilatory support is the cornerstone of treatment of the intermediate syndrome. Therefore, all patient should be observed in a hospital for up to 5 days after poisoning.
Han, Koon Hee;Jung, Bock Hyun;Kim, Young Don;Hwang, Jung Won;Hong, Hyun Il;Yoon, Sung Kyu;Kim, Mi Hye;Ryu, Dae Sik;Kang, Gil Hyun
Tuberculosis and Respiratory Diseases
/
v.59
no.1
/
pp.109-113
/
2005
Miliary tuberculosis is the most serious form of tuberculous disease, but is rarely complicated with acute respiratory distress syndrome (ARDS). When a patient with miliary tuberculosis initially presents with ARDS, the mortality is much higher. Therefore, the early detection of miliary tuberculosis as the underlying cause of ARDS is very important for the prognosis and survival of the patient. The diagnosis of miliary tuberculosis may be easy if the patient presents typical clinical manifestations associated with the characteristic pattern of miliary nodules on chest radiology. However, the diagnosis of miliary tuberculosis when complicated with ARDS can be difficult due to the nonspecific radiologic patterns, such as diffuse bilateral consolidation and ground glass opacity, without miliary nodular infiltration. However, these nonspecific patterns are known as less likely findings of miliary tuberculosis. We experienced a pregnant woman with miliary tuberculosis, mimicking ARDS due to bilateral severe pneumonia. She was admitted, via the emergency room, with sudden onset of fever, chill, cough and dyspnea. The initial chest PA and HRCT showed diffuse bilateral consolidation and ground glass opacity, without miliary nodular infiltration. All bacteriological studies, including blood and sputum cultures, tuberculosis-PCR and serologic study for infectious disease were negative. However, the definite diagnosis of unusual miliary tuberculosis as the underlying cause of ARDS was confirmed from the radiological finding and transbronchial fiberoptic lung biopsy. We report this case, with a review of the literature.
The author has investigated epidemiological features of human cases of epidemic encephalitis (E. E.) in the Republic of Korea and the status of antibody requisition in pre-and post-epidemic time. And virological and serological studies with regarding the relationship of E. E. infection between human and piglet, and field survey against its vector by means of virus isolation from mosquitoes were carried out. Finally, vaccine field trial against human population has also been evaluated in order to confirm its effectiveness. The results of the studies are summarized as follows : 1. The annual incidence of reported cases during the past 25 years (1949-1973) in the Republic of Korea has shown two patterns, one was typical cyclic incidence and the other one was irregular. Annual average morbidity and mortality rate per 100,000 population were 5.7 and 2.1 and fatality rate was 34.6% in typical cyclic years. 2. With regard to the geographical distribution of E. E., the province of Jeolla-Bug-Do illustrated the highest incidence regardless of the epidemic size. 3. The main epidemic period was between mid-August and mid-September (above 90% of the total number of cases). The first case was reported in middle of July and the epidemic ceased in late of October. 4. An analysis of the age distribution of cases of E. E., has shown that above 90% of the total cases occurred in the age groups under 14 years and it was noted that about its 54% were occurred in the age groups between 5-9 years group. 5. Through the Haemagglutination Inhibition (H-I) test for the laboratory diagnosis of E. E., it was found that higher H-I antibody titer was usually detected in the convalescent phase, 15 days after onset. 6. The H-I antibody survey against 563 healthy population by age groups during the pre-epidemic season showed that 422(75%) were less than H-I titer, 1:20 and 122(21.7%) were positive H-I titer, 1:20. Among the 94 American in Seoul who had not been in E. E. endemic area previously only one person had appeared sero-conversion as a H-I titer of 1:80 after post-epidemic season. 7. The E. E. virus could be isolated from the mosquitos pools-C, tritaeniorhyncus which were caught between late July and middle August. 8. E.E. Virus was also isolated from piglet blood on early August and H-I antibody conversion was occurred mostly on middle of August. 9. H-I antibody sero-conversion rate reached to high level when vaccine purified by mouse brain tissue inoculated, showing 98.9%. Higher antibody titer was acquired when booster inoculation was performed, Four fold rise of H-I add N-T antibodies was confirmed with 93.2% and 82.1% respectively.
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