Lymphadenitis is a common pediatric disease associated with infection and inflammation. Acute lymphadenitis in children is usually accompanied by systemic viral illness, and subsides within a few days to two weeks. However, chronic lymphadenopathy or lymphadenitis is a rare disease, and is due to a reactive response to infections including tuberculosis, neoplasms, and noninflammatory lesions. The diagnostic evaluation of chronic lymphadenitis may be very complicated and it is important to consider chronic infectious diseases and rare malignancies as possible etiologies. We report a 13-year old boy who was eventually diagnosed with tuberculous lymphadenitis. The patient was initially admitted for enlarged cervical, axillary, and epitrochlear lymph nodes. Biopsy of a cervical lymph node showed the findings compatible with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with EBV infection. During treatment with prednisolone, the enlarged lymph node decreased in size. However, the lymph node enlargement resumed and we performed a second biopsy. At this time the biopsy showed tuberculous lymphadenitis and a culture of gastric juice showed M. tuberculosis. With the initiation of antituberculous treatment, the patient improved clinically.
Adolescent ; Biopsy ; Child ; Communicable Diseases ; Epstein-Barr Virus Infections ; Gastric Juice ; Herpesvirus 4, Human* ; Humans ; Immunoblastic Lymphadenopathy ; Inflammation ; Lymph Nodes ; Lymphadenitis* ; Lymphatic Diseases ; Male ; Prednisolone ; Rare Diseases ; Tuberculosis ; Tuberculosis, Lymph Node*

Lymphadenitis is a common pediatric disease associated with infection and inflammation. Acute lymphadenitis in children is usually accompanied by systemic viral illness, and subsides within a few days to two weeks. However, chronic lymphadenopathy or lymphadenitis is a rare disease, and is due to a reactive response to infections including tuberculosis, neoplasms, and noninflammatory lesions. The diagnostic evaluation of chronic lymphadenitis may be very complicated and it is important to consider chronic infectious diseases and rare malignancies as possible etiologies. We report a 13-year old boy who was eventually diagnosed with tuberculous lymphadenitis. The patient was initially admitted for enlarged cervical, axillary, and epitrochlear lymph nodes. Biopsy of a cervical lymph node showed the findings compatible with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with EBV infection. During treatment with prednisolone, the enlarged lymph node decreased in size. However, the lymph node enlargement resumed and we performed a second biopsy. At this time the biopsy showed tuberculous lymphadenitis and a culture of gastric juice showed M. tuberculosis. With the initiation of antituberculous treatment, the patient improved clinically.
Adolescent ; Biopsy ; Child ; Communicable Diseases ; Epstein-Barr Virus Infections ; Gastric Juice ; Herpesvirus 4, Human* ; Humans ; Immunoblastic Lymphadenopathy ; Inflammation ; Lymph Nodes ; Lymphadenitis* ; Lymphatic Diseases ; Male ; Prednisolone ; Rare Diseases ; Tuberculosis ; Tuberculosis, Lymph Node*

BACKGROUND: Left ventricular dysfuction is common in immediate postoperative periods after surgical correction of heart diseases with chronic left ventricular volume overload. We speculated postoperative changes of left ventricular volume and unction in patients with patent ductus osus(PDA) who had underwent surgical repair at ages older than 16 years. Factors influencing postoperative left ventricular volume and function were also analyzed. MATERIAL AND METHOD: From August 1989 to August 1999 thirty-siz adult patients with PDA 28 females and 8 males. were enrolled in this study. Their age ranged from 16 years to 57 years(mean :32 years). Types of surgical repair were division with primary closure in 22, division with patch closure in 6, internal obliteration using cardiopulmonary bypass in 4 and ligation in 4. Aortic clamping was combined during surgical repair in 22(61%) and cardiopulmonary bypass was used in 8(22%) Two-dimensional echocardiography studies were performed in 34(94%) preoperatively and in 25(66%) immediate postoperatively to assess postoperative changes of left ventricular internal dimensions. left ventricular volume and ejection fraction. Duration of postoperative follow-up ranged from 1 month to 99 months (mean:22 months) and 10 patients underwent 16 echocardiographic evaluation during this period. RESULT: Preoperative and postoperative left ventricular systolic dimensions(LVIDs) were 42+/-8.0mm and 42+/-8.3mm left ventricular diastolic dimensions(LVIDd) were 64+/-10.0mm and 56+/-7.4mm left ventricular end systolic volumes(LVESV) were 62+/-19cc (z=1.87+/-0.06) and 59+/-24cc(z=1.78+/-0.08) left ventricular end diastolic volumes(LVEDL) were 169+/-40cc(z-1.17+/-0.1) and 112+/-29cc(z=0.85+/-0.1) and ejection fractions(EF) were 66+/-6.7% and 48+/-12.6% respectively. There were statistically significant differences between preoperative and postoperative values in LVDIDd(p=0.001) LVEDV(p=0.001) and EF(p=0.0001) while no significant difference is LVIDs and LVESV. Postoperative depression of ejcection fraction was significantly related with z-score of preoperative LVESV and LVEDV by univariateanalysis while LVEDV only was significant risk factor for postoperative LV dysfunction by multiple regressioin analysis (deltaLVEF=-13.3-4.62xLVEDV(z), p=0.001) During the follow-up periods ejection fractions become normalized in all except one patients. CONCLUSION: Left ventricular function is usually deteriorated after the surgical correction of PDA in adult age and preoperative LVEDV is a major determinant of postoperative LV function.
Adult* ; Cardiopulmonary Bypass ; Constriction ; Depression ; Echocardiography ; Female ; Follow-Up Studies ; Heart Diseases ; Humans ; Ligation ; Male ; Postoperative Period ; Risk Factors ; Ventricular Function, Left

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.
Antigens, CD34/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Benzoic Acids/therapeutic use ; Blood Transfusion/*adverse effects ; Child ; Child, Preschool ; Creatinine/blood ; Ferritins/blood ; Follow-Up Studies ; Humans ; Infant ; Iron Chelating Agents/therapeutic use ; Iron Overload/*etiology ; Neuroblastoma/drug therapy/*therapy ; Retrospective Studies ; Risk Factors ; *Stem Cell Transplantation ; Transplantation, Autologous ; Triazoles/therapeutic use

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.
Antigens, CD34/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Benzoic Acids/therapeutic use ; Blood Transfusion/*adverse effects ; Child ; Child, Preschool ; Creatinine/blood ; Ferritins/blood ; Follow-Up Studies ; Humans ; Infant ; Iron Chelating Agents/therapeutic use ; Iron Overload/*etiology ; Neuroblastoma/drug therapy/*therapy ; Retrospective Studies ; Risk Factors ; *Stem Cell Transplantation ; Transplantation, Autologous ; Triazoles/therapeutic use

BACKGROUND AND OBJECTIVES: Although the clinical significance of mitral regurgitation (MR) due to prolapse or chordae rupture with myxomatous degeneration (MD) is increasing significantly, clinical features of patients with MD in Korea are not characterized. MATERIALS AND METHODS: Retrospective analysis of clinical data of 90 patients who underwent surgical correction of significant MR due to MD was performed. Lesion sites of MD were confirmed during surgery; anterior (A) and posterior (P) mitral leaflets were divided into lateral (A1 & P1), middle (A2 & P2), and medial segments (A3 & P3). METHODS: Mean age was 5114 years and male / female ratio was 1; age distribution showed typical bimodal pattern with two peaks at the mid-thirties and the mid-fifties each. MD was confined to P leaflet in 36 (40%), A leaflet in 20 (22%), and both leaflets in 34 patients (38%). Forty-six patients (51%) showed MD in a single segment, and 37 (41%) in 2 segments; 7 patients (8%) showed MD in more than 2 segments. In 90 patients, pathologic MD was confirmed in 139 mitral segments; among them, P3 was the most commonly involved segment (30%), followed by A3 (17%), P2 (14%), A2 (14%), A1 (14%), and P1 (12%). Hypertension (HT) was more frequently observed in female patients (42%) than in male patients (16%) (p<0.05). Chordae rupture was observed in 71 patients (79%), which was associated with HT. Younger patients (age<45 years, N=31) showed lower prevalence of HT and higher incidence of MD involving multiple segments. Valve repair was successful in 83 patients (92%), which was not associated with lesion sites or numbers of MD. Three-year event free survival rate was 865% and independent on sites and numbers of MD. CONCLUSION: MD develops preferentially in the medial part of the mitral valve, and patterns of clinical presentation can change according to the age and existence of HT in these selected patients with significant MR. However, the morphologic characteristics of MD do not seem to affect the feasibility of repair and long-term prognosis.
Age Distribution ; Disease-Free Survival ; Female ; Humans ; Hypertension ; Incidence ; Korea* ; Male ; Mitral Valve ; Mitral Valve Insufficiency* ; Mitral Valve Prolapse ; Prevalence ; Prognosis ; Prolapse ; Retrospective Studies ; Rupture

BACKGROUND AND OBJECTIVES: To compare the pattern of myocardial fibrosis in various valvular heart diseases (VHD), the morphometric data of the myocardial tissue and serum biochemical markers of myocardial fibrosis were analyzed in patients with aortic stenosis (AS), aortic regurgitation (AR) and mitral regurgitation (MR). SUBJECTS AND METHODS: Blood samples were obtained from 21 patients with AS, 23 with AR and 29 with MR. The serum levels of aminoterminal propeptide, of type I/III procollagen (PINP/PIIINP), and fibronectin were measured to estimate the synthesis of the extracelluar matrix. The carboxy-terminal telopeptide collagen type I (CITP), matrix metalloproteinase-1 (MMP-1, collagenase) and the tissue inhibitor, metalloproteinase-1 (TIMP-1), were also measured to estimate the collagen degradation and metabolism activities. The left ventricular mass (LVM) was calculated by echocardiography. Of the patients, myocardial tissue was obtained during surgery in 11 with AS, 8 with AR and 13 with MR;the collagen volume fraction (CVF) was calculated using picrosirius red staining. RESULTS: The LVM was significantly larger in the AS and AR groups compared to the MR group (p<0.001), and the CVF also showed significant differences (13+/-3% in AS, 10+/-3% in AR, and 6+/-3% in MR, p<0.001). The fibronectin level was significantly elevated in the AS and AR groups than the MR group (p<0.001), whereas the CITP and MMP-1 levels were significantly higher in the MR group (p<0.05). The PINP/PIIINP showed no significant difference between the groups (p>0.05), and the biochemical markers were no different between the AS and AR groups (p>0.05). Fibronectin was the only parameter showing a positive correlation with both the CVF (r=0.388, p=0.01) and the left ventricular mass (r=0.278, p=0.02). CONCLUSION: Different mechanisms for the matrix synthesis and degradation were present for the maintenance of myocardial fibrosis and hypertrophy according to the type of VHD, and fibronectin, a major non-collagenous extracelluar matrix, was proved to be an important factor associated with cardiac hypertrophy and myocardial fibrosis.
Aortic Valve Insufficiency ; Aortic Valve Stenosis ; Biomarkers ; Cardiomegaly ; Collagen ; Collagen Type I ; Echocardiography ; Fibronectins ; Fibrosis* ; Heart Valve Diseases* ; Humans ; Hypertrophy ; Matrix Metalloproteinase 1 ; Metabolism ; Mitral Valve Insufficiency ; Procollagen

BACKGROUND AND OBJECTIVE: Assessment of the clinical implications of subaortic complications, such as the involvement of the mitral-aortic intervalvular fibrosa (MAIVF) or anterior mitral valve leaflet (AMVL), were sought in patients with aortic valve infective endocarditis (AoIE). SUCJECTS AND METHODS: The clinical data of 95 consecutive (69 male) AoIE patients were retrospectively analyzed. RESULTS: Subaortic complications were detected in 40 patients (42%). Involvement of the AMVL and MAIVF was present in 16 and 13 patients, respectively, and in 4 patients both were involved. The remaining 7 patients showed metastatic lesions in chordae or interventricular septum. Surgery was performed in 60 patients (63%, Group I) and the other 35 received medical treatment only;surgery was neither feasible due to multiorgan failure in 17 patients (Group II) nor necessary with stable hemodynamics in 18 (Group III). The overall in-hospital mortality was 19% (18/95), which was significantly different according to the treatment group (10% in group I, 65% in group II, and 6% in group III, p<0.001). In group I, patients with subaortic complications showed higher mortality compared to those without (22 vs. 0%, p=0.006). Surgery was performed in 28 patients with a periaortic abscess, and operative mortality was much higher in patients with a MAIVF abscess than in those with an abscess in other sites (40 vs. 0%, p=0.018). The sensitivity of transesophageal echocardiography for a subaortic complication was significantly higher than that of transthoracic echocardiography (89 vs. 41%, p<0.05). CONCLUSION: Subaortic complications were relatively frequent in AoIE, and a MAIVF lesion is associated with a higher operative mortality.
Abscess ; Aortic Valve* ; Echocardiography ; Echocardiography, Transesophageal ; Endocarditis* ; Endocarditis, Bacterial ; Hemodynamics ; Hospital Mortality ; Humans ; Mitral Valve ; Mortality ; Retrospective Studies

BACKGROUND: The heart transplantation is now accepted as a definite therapeutic modality in patients with terminal heart failure. With use of immunosuppressive agent, the incident of rejection deceased but risk of infection increased. Infection has been the most common cause of death in heart transplant patient, especially during the first year. The purpose of this study is to evaluate the infection of 91 patients who had heart transplantation at our hospital. METHODS: Of the total 91 patients, there were 75 males and 16 females, and the mean age was 39.8+/-14.1 years ranged from 14 to 62 years. All patients were in NYHA functional class III or IV preoperatively. The most common underlying heart diseases were dilated cardiomyopathy(72/91). The mean follow-up duration was 36.4 months (range; 0.6 ~ 103 months) and 10 patients died during this period. RESULT: There were 35 patients with infections (early infections in 4 and late infections in 32). The most common infection was skin infection of herpes virus (15 cases). Pneumonia occurred in 8 patients and responded well to antibiotics. But multiple empyema developed in one patient with bacterial pneumonia despite of antibiotics, chest tube insertion was needed. There were 4 patients with tuberculosis, 2 with tuberculous pericardial effusion, 1 with pulmonary tuberculosis and 1 with miliary tuberculosis. Sepsis was noted in 3 patients, 2 with bacterial sepsis and 1 with candidial sepsis. They all died despite of antibiotic treatment. There were 2 cases with wound infections and 1 with perianal abscess, and 1 with aortitis with paraaortic abscess due to Aerococcus viridans. Two patients with cryptococcal meningitis were successfully treated with liposomal amphotericin B and oral fluconazole, one of them also had invasive aspergillosis. There were 6 cases with cytomegalovirus (CMV) disease. Three of them had CMV viremia , 2 had CMV disease ( retinitis and colitis) and 1 had viremia and disease. CONCLUSION: There were 35 patients (38.5%) with infections and among then, 21 patients (23%) had one or more episodes of major infection. Infection was a major cause of death (30%) after heart transplantation. Careful control of infection is vital in the care of transplant recipients because infections result in increased morbidity and mortality.
Abscess ; Aerococcus ; Amphotericin B ; Anti-Bacterial Agents ; Aortitis ; Aspergillosis ; Cause of Death ; Chest Tubes ; Cytomegalovirus ; Empyema ; Female ; Fluconazole ; Follow-Up Studies ; Heart ; Heart Diseases ; Heart Failure ; Heart Transplantation* ; Humans ; Male ; Meningitis, Cryptococcal ; Mortality ; Pericardial Effusion ; Pneumonia ; Pneumonia, Bacterial ; Retinitis ; Sepsis ; Skin ; Transplantation ; Tuberculosis ; Tuberculosis, Miliary ; Tuberculosis, Pulmonary ; Viremia ; Wound Infection

Tumors of the minor papilla are very rare and these tumors have generally been known as neuroendocrine tumors such as carcinoid tumor and somatostatinoma. As these are mostly submucosal tumors, their diagnosis is difficult by just performing endoscopic forceps biopsy, but diagnosis is possible by surgery or endoscopic resection. EUS and ERCP is an essential tool for the diagnosis of these tumors, and abdominal CT or MRI is also useful because there is the possibility of malignant tumors such as carcinoid tumor. For our present two cases, screening endoscopy revealed the polypoid lesion of the minor papilla. EUS disclosed that the submucosal tumor was limited to the submucosal layer and no abnormality was found from the abdominal CT and ERCP. Herein, we performed endoscopic resection for making the diagnosis and treatment. Histologically, these 2 tumors were diagnosed as ectopic pancreas and gangliocytic paraganglioma, respectively.
Biopsy ; Carcinoid Tumor ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Endoscopy ; Magnetic Resonance Imaging ; Mass Screening ; Neuroendocrine Tumors ; Pancreas ; Paraganglioma ; Somatostatinoma ; Surgical Instruments ; Tomography, X-Ray Computed