The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.
Hu, Jung-Woo;Jung, Sung-No;Kwon, Ho;Rhie, Jong Won;Yoo, Gyeol;Oh, Deuk Young;Jun, Young Joon;Choi, Yun Seok
Archives of Plastic Surgery
/
v.36
no.4
/
pp.406-410
/
2009
Purpose: An anatomical study concerning the location of the perforators to the posteromedial thigh perforator flap was done for the purpose of clinical application, and reconstruction using this flap was undergone for 3 patients with ischial sores. Methods: The authors dissected 6 cadavers, measuring the distance between the perforator of the posteromedial thigh perforator flap and the extended line of the inguinal crease. The location of the perforator was studied using this data. In the 3 cases mentioned above, perforators were traced using Doppler ultrasonography and the sores were reconstructed with posteromedial thigh perforator flaps. Results: Anatomical study results revealed that posteromedial thigh perforators were found $77{\pm}18.9mm$ below the extended line of the inguinal crease. Application of the flap in the 3 patients was successful. Conclusion: The perforator to the posteromedial thigh perforator flap was found to be located in a relatively consistent position. Since this flap is also comparatively easy to elevate and mobilize, and shows low donor site morbidity, it is thought to be very useful in the treatment of ischial sores.
Han, Jeong Hee;Park, Sang Yong;Kang, Min Gu;Chung, Yong Hyun;Yang, Jung Sun
Journal of Korean Society of Occupational and Environmental Hygiene
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v.22
no.4
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pp.316-328
/
2012
Objectives: This study was designed to provide the information regarding chemicals classification and health hazard by evaluating the toxicological effect through repeated inhalation exposure of methyl acrylate(MA) in Sprague-Dawley(SD) rat for 13 weeks. Methods: According to the notification with Ministry of Labor(No. 2009-68) and OECD Test Guideline 413, the rats were exposed to MA at concentration of 0, 56, 168, 280 ppm via whole body inhalation for 6 hours per day, 5 days per week, for 13 weeks. All animals were observed for mortality, morbidity and the change of body weight and food consumption were determined during the exposure period. Necropsy finding, organ weight, hematology, clinical biochemistry and histopathological examination following exposure were also performed. Results: There were no death and abnormal clinical signs relate to exposure MA. However, At 160 ppm and 280 ppm exposure groups, body weight and food consumption showed statistically significant decrease and histopathological changes in lung, trachea, nasal cavity, larynx were observed. Conclusions: MA was mainly affected respiratory tract. It is consequently provided to be classified as category 2(0.2 mg/L/6h < category 2 ${\leq}$ 1.0 mg/L/6h) for specific target organ toxicity following repeated exposure according to Standard for Classification and Labeling of Chemical Substance and Material Safety Data Sheet. The NOAEL(no observable adverse effect level) of MA was also determined to be lower than 56 ppm.
Purpose: Inflammatory bowel disease (IBD) in children and adolescents is associated with high morbidity and possibly has a significant negative impact on their quality of life. This study aimed to evaluate the quality of life of children and adolescents with IBD and define the variables that impact these individuals. Methods: We administered the Pediatric Quality of Life Questionnaire (PedsQL) to 35 children and adolescents diagnosed with IBD and with available quantitative data from clinical records on epidemiology, clinical evolution, complementary tests, medical interventions, and disease activity. Data were evaluated according to the IBD type and compared with a control group of healthy children. Results: The study group showed a significantly lower PedsQL score than the control group (p<0.01). Significant factors contributing to poor overall quality of life included female sex, Crohn's disease, surgery, and food restrictions. Symptoms such as diarrhea and the fear of using public toilets were associated with low physical scores. Feeling sick had a negative impact on the emotional PedsQL scores. Patients with a fear of using public toilets, anthropometric scores below the 3rd percentile, and greater disease activity scored lower in the social domain. Regarding school and psychosocial evaluations, younger children with symptom onset after the age of 2 years had lower scores than younger children with symptom onset before the age of 2 years. Conclusion: IBD negatively affects the quality of life of children and adolescents based on its impact on the physical, emotional, social, and psychosocial statuses of these patients.
The vascular surgery is the field that has developed in early 20 century and is progressing nowadays. Recent advance in surgical technique accompanying with excellent medical diagnosis and treatment, prompt angiographic usage, development of variable prosthetic material, and concomitant use of anti-coagulant have made remarkable results of vascular surgery. 83 cases of vascular surgery have been performed at Thoracic and Cardiovascular Surgery Department of Pusan National Unversity Hosaital since 1971 till 1990, for 20 years and their results are followed. Patient ductus arteriosus and Buerger`s disease were omited in this study. 1. The age distribution shows that the fifth and sixth decades are most frequently affected and mean age was 56.1 years old. Male to female ratio is 1: 2.32. 2. Among the 83 cases of all, number of occlusive vascular disease is 46 and that of aneurysmal disease is 33. 3. In clinical manifestation, most common symptom of occlusive disease is pulselessness and pain was next. Mass sensation is most commonly complained by patients of aneurysmal disease. 4. CT scan was more important in diagnosis of aneurysmal diseases and angiogram was more commonly used in occlusive diseases. 5. The common site of arterial occlusion was common iliac artery, femoral artery, aortic bifurcation, and external iliac artery, as its frequency rate. The most commonly affecting portion of aortic aneurysm was abdminal aorta, and descending thoracic aorta and femoral artery were next 6. Preoperative associated diseases were atherosclerosis[41 cases], hypertension[21 cases], valvular heart disease[11 cases], and diabetes mellitus[9 cases], etc, 7. Operative methods in ocllusive diseases were thrombectomy[36.9%], endarterectomy [10.9%], and bypass graft insertion[52.7%]. Among the bypass graft, Y-graft was used in 7 case, straight graft was used in 17 cases, and saphenous venous graft was used in 2 cases. 8. Postoperative complications were developed in 17 cases, and morbidity rate was 36. 9. Eleven patient were died within 1 month after operation, so operative mortality rate was 13.3%. 10. Duration of patency was beteween 7 and 58 months[average 27.5 months] in occlusive diseases and their 5-year patency rate was 56.3%. Duration of patency of aneurysmal disease was 20 months in aveage and their 5-year patency rate was 51.3%. 11. Patients of eleven cases of occlusive disease and two cases of aneurysmal disease required reoperation for variable reason. 12. 35 cases of patient have used anticoagulants: coumadin, ticlid, and persanthin-ASA combination.
Although several reports were presented recently about bronchial arterial revascularization in clinical lung transplantation, one factor peculiar to the lung transplantation is the ischemia of the donor bronchus. Poor bronchial healing occurs frequently following clinical lung transplantation and this has been major cause of mortality and morbidity. There have been many attempts to solve bronchial anastomotic complications. Telescoping technique, one of those attempts, was advocated by San Antonio Group recently. This experiment was per(armed to evaluate the effect of telescoping anastomotic technique upon th healing of the tracheo-bronchial anastomosis. We used rabbits(weighing about 800 g) as experimental animal. Method: Resection of middle one third of cervical trachea and reanastomosis was performed by simple interrupted anastomotic technique in Group 1(n=15) and by telescoping anastomotic technique in Group 2(n= 15). Result: Anastomotic sites in the telescoping technique group showed significant increase of fibrosis in the early postoperative days(< Sdays) and remarkable band-like fibrous union compared to the simple interrupted group.
Objectives: This study was carried out to analyze the clinical profile and the results of treatment of a series of patients with extramedullary plasmacytoma(EMP) of the head and neck. Materials and Methods: The clinical features, treatment and survival of 14 patients with EMP seen at Yonsei Medical Center between 1970 and 1998 were carefully reviewed. Results: The median age was 48 years(range 15-75) and there was a male predominance(M:F 1.8:1). Nasal cavities and paranasal sinuses account for 50% of the primary sites and the most common symptom was airway obstruction(50%). Five patients(36%) showed evidence of adjacent bone destruction, one patient had lymph node involvement and one patient had an IgG monoclonal gammopathy at the time of diagnosis. All 5 patients treated with radiotherapy alone achieved local control. Of 5 patients treated with surgery alone, 2 patients(40%) had local failure. Of 3 patient treated with a combination of surgery and radiotherapy, one patient had local recurrence. A total of7 patients(54%) had local, distant or nodal relapses after primary treatment. Six of them received salvage treatment. With salvage treatment of surgery and/or radiotherapy, local control was achieved in 4 of 6 patients. Conversion to multiple myeloma was seen in one patient. Conclusion: Radiotherapy should be recommanded as treatment of choice for EMP of the head and neck. Surgery should be reserved for radioresistant or recurrent tumors, but tumors that are localized and can be removed relatively easily with little morbidity may be treated by primary surgery.
The purpose of this study was to present the clinical analysis of the results of lateral arm free flap for small sized and infected diabetic foot ulcer around toes. From May 2006 to December 2007, Seven patients were included in our study. Average age was 52.8 years, six were males and one was female. All had infected diabetic foot ulcer and had exposures of bone or tendon structures. Ulcers were located around great toe in four patients, 4th toe in one and 5th toe in two. Three patients had osteomyelitis of metatarsal or phalanx. After appropriate control of infection by serial wound debridement and intravenous antibiotics, lateral arm flap was applied to cover remained soft tissue defects. Posterior radial collateral artery of lateral arm flap was reanastomosed to dorsalis pedis artery of recipient foot by end to side technique in all cases in order to preserve already compromised artery of diabetic foot. All flaps were designed over lateral epicondyle to get longer pedicle and averaged pedicle length was 8 cm. Two cases were used as a sensate flap to achieve protective sensation of foot. All flaps survived and provided satisfactory coverage of soft tissue defects on diabetc foot ulcers. All patients could achieve full weight-bearing ambulation. No patients has had recurrence of infection, ulceration and further toe amputations. There were three complications, a delayed wound healing of flap with surrounding tissue, a partial peripheral loss of flap and a numbness of forearm below donor site. All patients were satisfied with their clinical results, especially preserving their toes and could return to the previous activity levels. Lateral arm free flap could be recommend for infected diabetic foot ulcers around toes, to preserve toes, coverage of soft tissue defect and control of infection with low donor site morbidity.
Objective : Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes (MELAS) syndrome is a progressive neurodegenerative disorder. The typical presentation of patients with MELAS syndrome includes features such as mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes. Other features, such as seizures, diabetes mellitus, hearing loss, cardiac disease, short stature, endocrinopathies, exercise intolerance, and neuropsychiatric dysfunction are clearly part of the disorder. Approximately 80% of patients with the clinical characteristics of MELAS syndrome have a genetic mutation. This progressive disorder is reported to have a high morbidity and mortality. This case report is intended to estimate clinical effects of oriental meedicinal treatment of MELAS syndrome. Methods : A 44 year old female patient diagnosed as MELSA syndrome was treated with general oriental medicinal therapy including acupuncture, moxibustion, cupping, pharmacupunture and herbal medication in 20XX in Wonkwang Medical Center Gwangju. And the changes in symptoms and signs were evaluated as time dependently. Results : Although there is currently no curable treatment and MEALS syndrome is tend to progress, our treatment showed improvement in general weakness, gait disturbance and pain in the patient. Conclusion : Our case report suggests that various oriental medicinal treatment could be effective for improvement of MELAS syndrome and may represent a new potential therapeutic approach to control the disease. It could be applied to improve general condition, prevent relapse, enhance the quality of life and reduce complaints in the patient.
Purpose: Most stomach surgeons have been educated sufficiently in conventional open distal gastrectomy (ODG) but insufficiently in laparoscopy-assisted distal gastrectomy (LADG). We compared learning curves and clinical outcomes between ODG and LADG by a single surgeon who had sufficient education of ODG and insufficient education of LADG. Materials and Methods: ODG (90 patients, January through September, 2004) and LADG groups (90 patients, June 2006 to June 2007) were compared. The learning curve was assessed with the mean number of retrieved lymph nodes, operation time, and postoperative morbidity/mortality. Results: Mean operation time was 168.3 minutes for ODG and 183.6 minutes for LADG. The mean number of retrieved lymph nodes was 37.9. Up to about the 20th to 25th cases, the slope decrease in the learning curve for LADG was more apparent than for ODG, although they both reached plateaus after the 50th cases. The mean number of retrieved lymph nodes reached the overall mean after the 30th and 40th cases for ODG and LADG, respectively. For ODG, complications were evenly distributed throughout the subgroups, whereas for LADG, complications occurred in 10 (33.3%) of the first 30 cases. Conclusions: Compared with conventional ODG, LADG is feasible, in particular for a surgeon who has had much experience with conventional ODG, although LADG required more operative time, slightly more time to get adequately retrieved lymph nodes and more complications. However, there were more minor problems in the first 30 LADG than ODG cases. The unfavorable results for LADG can be overcome easily through an adequate training program for LADG.
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