Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.
Lee Jee-Hyun;Jeon Ga-Won;Park Sung-Eun;Jin Dong-Kyu;Paik Kyung-Hoon
Childhood Kidney Diseases
/
v.9
no.1
/
pp.38-45
/
2005
Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.
Woo Jin Lee;Hoon Jai Chun;Ye Ji Kim;Sun Young Kim;Min Ho Seo;Hyuk Soon Choi;Eun Sun Kim;Bora Keum;Yoon Tae Jeen;Hong Sik Lee;Soon Ho Um;Chang Duck Kim;Ho Sang Ryu
Journal of Digestive Cancer Research
/
v.1
no.1
/
pp.53-57
/
2013
There is no established treatment for esophageal carcinoma with metastasis. For the metastatic esophageal squamous cell carcinoma, chemotherapy or best supportive care according to patient's performance status are accepted as an available treatment. We report a case of complete remission after concurrent chemoradiotherapy for esophageal squamous cell carcinoma with metastatic lesion in 5th thoracic vertebrae. A 57-year-old man with ongoing dysphagia and weight loss was admitted to our hospital. On the endoscopic and radiologic imaging evaluation,the patient was diagnosed as a squamous cell carcinoma of esophagus with solitary metastatic lesion in 5th thoracic vertebrae. The patient was treated with combination chemotherapy (5-fluorouracil (5-FU) and cisplatin) and concurrent radiotherapy for two months to relieve dysphagia. Because metastatic lesion in thoracic vertebrae was located near the primary esophageal tumor, the metastatic lesion could be included within the radiation field. After concurrent chemoradiotherapy, consecutive 4 cycles of chemotherapy had been carried out. Primary esophageal tumor with metastatic lymph nodes and metastatic lesion in 5th thoracic vertebrae disappeared on follow up computed tomography (CT) and positron emission tomography-CT (PET-CT). Follow up endoscopic biopsy revealed no remnant malignant cells at previous primary cancer lesion.
Background : To Investigate the association between bronchial anthracofibrosis (AF) and tuberculosis (TB), and the clinical utility of a polymerase chain reaction (PCR) on bronchial specimens for rapid diagno-sis of active pulmonary TB in patients with bronchial AF. Method : Thirty patients (25 women and 5 men ranging in age from 53 to 88), who were diagnosed with bronchial AF by a bronchoscopic exami-nation, were enrolled in this study. PCR targeting the IS6110 segment of Mycobacterium tuberculosis was performed on the bronchial wash fluid and anthracofibrotic bronchial tissue. The PCR results were compared with the bacteriological, histological, and clinical findings. Results : Eighteen of the 30 patients (60%) were associated with TB, nine of whom were confirmed as having active TB. The remaining 9 had a past history of TB. The sputum or bronchial aspirate AFB smear, culture, and histological findings were positive in 4 (13%), 9 (30%), and 5 (17%) patients, respectively. PCR of the AF tissue and bronchial wash fluid was positive in 5 (17%) and 11 (37%) of the 30 patients, respectively. PCR was more sensitive than the AFB smears for diagnosing pulmonary TB (22 % us 89 %, respectively, p<0.05). All 5 patients with positive AF tissue PCR results also had both histological findings and positive bronchial wash fluid PCR results. Of the 3 patients with positive PCR but negative bacteriological or histological results, 2 of these patients appeared to have active tuberculosis on a clinical basis. Conclusion: Although TB-PCR did not reveal an increased association between bronchial AF and TB compared with traditional methods, PCR on the bronchial wash fluid appears to be useful for the rapid diagnosis of pulmonary TB in patients with bronchial AF. TB-PCR on AF bronchial tissue itself did not yield additional benefits for diagnosing TB, which suggests that an AF lesion itself may not be an active or original site of the infection, but a secondary change of TB.
Purpose : Bacterial meningitis is a serious disease, especially in the neonatal period, and it carries a significant degree of mortality and morbidity. Group B streptococcus(GBS) is a common cause of neonatal bacterial meningitis. The purpose of this study was to evaluate the clinical manifestations, treatment results and complications of GBS meningitis. Methods : We analyzed 29 cases retrospectively who had been admitted to the pediatric ward or NICU in Asan Medical Center from May 1990 to January 2002. They had proven GBS in culture or latex agglutination test in CSF. Results : The male to female ratio was 1 : 1.9. There were two cases of early onset type and 27 cases of late onset type. All cases had normal birth weight with full term at delivery. The perinatal predisposing factors were premature rupture of membrane(two cases), and maternal colonization(two cases). The most common presenting symptoms were fever and irritability. Associated diseases were GBS sepsis(21 cases). There was relatively high sensitivity to penicillin derivatives. There were abnormal brain CT or MRI findings in 16 cases(64%), such as infarction, encephalomalatic change, effusion, hydrocephalus, hemorrhage and abscess. The intensive care unit admission rate and the incidence of DIC were higher in the group with complications. Two cases were discharged against advice. Conclusion : We recommend early detection and active treatment in Group B streptococcal meningitis to improve the prognosis.
Background: In the operation for coronary artery stenosis, the procedures for mitral regurgitation are restricted to cases of more than moderate mitral regurgitation or for the lesions in leaflets. This is based on the belief that the less than mild regurgitation are a form of reversible change results from ischemia with coronary artery stenosis. We studied the changes and prognostic factors of mitral regurgitation in patients with coronary artery stenosis and mitral regurgitation who underwent coronary artery bypass surgery alone. Material and Method: We reviewed the medical records of 90 patients with coronary artery stenosis and mitral regurgitation who underwent coronary artery bypass surgery alone by a single surgeon from Jan. 1995 to Dec. 2002, We grouped the patients according to the postoperative changes of mitral regurgitation, and then we statistically compared the findings of echocardiogram between preoperative and last follow up. Result: There were 24 cases with progression of mitral regurgitation, 12 cases without changes, 54 cases with improvements of mitral regurgitation in total 90 patients. The bypass to LAD was proven as the significant prognostic factor of mitral regurgitation. The preoperative end diastolic left ventricular volume index were higher in aggravated group with 105.38$\pm$38.89 $m\ell$ compared to 71.75$\pm$28,45 $m\ell$ in improvement group, and 84.00$\pm$11.66 $m\ell$ in no change group. The grade of preoperative mitral regurgitation did not show significant differences among the groups. Conclusion: The mitral regurgitation in patient with coronary artery stenosis can be improved after the coronary artery bypass surgery alone. However, the expectation of improvements based on the degree of preparative mitral regurgitation can not be justified, therefore, the procedures for mitral regurgitation should be aggressively considered even in the cases of mild mitral regurgitation. Also, further study should be performed to identify the exact prognostic factors of mitral regurgitation including the left ventricular volume index, and whether the left anterior descending artery has been bypassed.
Park, Hyo-Jun;Kim, Keung-Mi;Choi, Min-Gew;Noh, Jae-Hyung;Sohn, Tae-Sung;Bae, Jae-Moon;Kim, Sung
Journal of Gastric Cancer
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v.9
no.2
/
pp.63-67
/
2009
Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin's lymphoma (NHL). We report here on a patient who displayed the synchronous occurrence of SMZL and early gastric cancer (EGC). The patient was a 74 year-old male with liver cirrhosis. An EGC in the gastric antrum was diagnosed and the preoperative abdomen computed tomography scan revealed splenomegaly and intra-abdominal lymphadenopathy. We performed subtotal gastrectomy and the postoperative pathologic examination revealed adenocarcinoma limited to the gastric mucosa and SMZL in the lymph nodes. The patient recovered from the surgery without complications and is now awaiting chemotherapy. SMZL has an indolent clinical course with good long-term survival and so there is the possibility of the occurrence of a second primary malignancy. Rare cases of a second primary malignancy being diagnosed along with SMZL have been described in the literature. Patients with SMZL should be carefully followed after treatment to detect the possible occurance of a second primary malignancy.
Bronchoesophageal fistula is a rare clinical entity whether congenital or acquired in adult. We experinced 8 cases of bronchoesophageal fistula and performed surgical correction from 1991 to 1994. Of the 8 patients, 5 patients were male and three were female aging from 21 to 61 years(mean 44.12$\pm$14.62 years). Seven of 8 patients had congenital bronchesophageal fistula and the other one had acquired bronchoesophageal fistula. According to the classification of Braimbridge and Keith, 4 cases were belonged to type I and 3 cases were type II . The diagnosis was confirmed by esophagogram in six patients, by bronchoscopy and bronchogram in two patients, and in one patient, the fistula was discovered i cidentally during operation. All patients received astulectomy and concomitant procedures were applied as follows ; 4 diverticulectomy, 4 right lower lobectomy, 1 bilobectomy, 1 left lower lobectomy and 1 wedge resection of left lower lobe. All but one patient were discharged without any complication and have been in good condition.
Purpose : The purpose of this study was to evaluate clinical efficacy of the arthroscopic anterior acromioplasty for the treatment of chronic impingement syndrome of the shoulder. Materials and Methods : Between July 1995 and December 1997, twenty seven consecutive shoulders of 26 patients with chronic impingement syndrome of the shoulder were treated by arthroscopic anterior acromioplasty. The patients who had severe osteoarthritis of the shoulder full thickness tear of the rotator cuff, and nonoutlet impingement were excluded. The clinical results were evaluated by using UCLA shoulder rating scale. The average follow-up was 2years 3months(range, 1year 7months to 3years 1 11months). Results : Twenty three patients$(85.2\%)$ were rated as excellent or good results, while four patients$(14.8\%)$ were fair. Twenty six cases$(96.3\%)$ were satisfied with the results of the operations, while one case$(3.7\%)$, who had Parkinsonian syndrome, ossification of posterior longitudinal ligament(OPLL) of the cervical spine, and spinal stenosis of the 5th and 6th cervical spine was not satisfied. Conclusion : Arthroscopic anterior acromioplasty was an effective treatment method, especially for relief of pain, for the treatment of chronic impingement syndrome of the shoulder. If the patient has the combined lesions in the cervical spine and the shoulder, and systemic lesions, these lesions may influence the results of treatment after operation, and cause the unpredictable results.
Purpose : The purpose of this paper is to analyze the clinical results after meniscal repair using meniscal arrow. Materials and Methods : Between May 1997 and Aug 1998, we repaired 22 tom menisci in 22 patients using meniscal arrows. There were nineteen males and three females with an average age of 27 years. There were longitudinal tear in 14 cases, Bucket-handle tear were 7 cases and horizontal tear was in 1 case. In 22 meniscus tears, 16 cases were associated with anterior cruciate ligament tear. Average number of meniscal arrow that was used were 2.5(ranged 1 to 4). Average follow-up period was 14.7 months(ranged 6 to 22 months). We evaluated the clinical results by the Tapper and Hoover's grading system. Results : There were excellent in 16 cases, good in 4 cases and fair in 2 cases on the clinical results. At the last follow up, the range of the motion of the knee joint were average 135 degrees(ranged 125 to 140 degrees). Mean time elapsed for meniscal repair were 25 minutes(ranged 15 to 40 minutes). Conclusion : Meniscal arrow has many advantages such as short operative time, easy fixation technique, and less neurovascular injury. We think that arthroscopic meniscal repair using meniscal arrow is effective treatment method in selected patient who have longitudinal, bucket-handle tear at the posterior hom associated with anterior cruciate ligament tear.
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