Congenital Abnormalities ; Female ; Humans ; Jaw ; Jeollabuk-do ; Male ; Orthognathic Surgery ; Osteotomy ; Osteotomy, Sagittal Split Ramus ; Rehabilitation ; Surgery, Oral

BACKGROUND: The testes are one of the most common extramedullary sites of relapse in boys with acute lymphoblastic leukemia(ALL). The reported incidence of isolated testicular relapse varies from 3 to 40%. If these patients are treated exclusively with testicular irradialion, a systemic relapse occurs within a few months. Recently, the use of intensive chemotherapy and testicular irradiation improved the survival rate for boys with testicular leukemia. So, we performed this study to identify clinical manifestations, disease free survival and prognostic factors of testicular leukemia in children. METHODS: We reviewed 33 patients of testicular leukemia among total 410 boys with ALL diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1970 to Aug. 1996. Testicular leukemia was confirmed by testicular biopsy in all 33 patients. These patients were treated with combined local testicular irradiation(2,400~2,500 cGy/8~12fractions) and systemic chemotherapy. Two patients, in whom testicular relapse was diagnosed before 1979, unilateral orchiectomy of the involved site and testicular irradiation of the opposite site were performed. Probability estimates of disease free survival (DFS) were calculated by the method of Kaplan and Meier, and the relationship of prognostic factors to DFS was compared using the chi-square test in survival analysis. RESULTS: In 410 boys with ALL, testicular leukemia occurred in 33 patients(8%). Of 33 patients, 6 patients presented with testicular involvement at initial diagnosis, 16 patients had testicular relapse while still receiving chemotherapy and 11 patients had testicular relapse 3 to 57 months(median : 15 months) after cessation of chemotherapy. The median age of 33 patients was 7.4 yrs(9 months~18 yrs) and median WBC count 7,600/ L(2,700~270,000/L). All patients presented with painless testicular enlargement and testicular leukemia was confirmed by testicular biopsy. Among 33 patients, 2 had prior CNS relapse and 11 had concomitant bone marrow and/or CNS relapse. Twenty nine patients were treated with combined local testicular irradiation and systemic chemotherapy. Eleven had second relapse(6 bone marrow, 3 CNS, 2 opposite testis). Seventeen have been followed until now: 6 patients on chemotherapy and 11 patients(37.9%) in complete remission for 48.5+/-22.3 months(19~86 months). The 3 year DFS for 29 patients was 55.3%+/-10.1%. The following prognostic factors showed no significant association with DFS in testicular relapse : age and WBC count at initial diagnosis, age at testicular relapse, and concomitant relapse. Whether testicular relapse occurred on initial therapy or off initial therapy has prognostic value in predicting DFS. The 3 year DFS for boys with testicular relapse on and off initial therapy were 40.0%+/-12.9% and 78.8%+/-13.4%, respectively(P: 0.046). CONCLUSION: With the use of chemotherapy and testicular irradiation, prolonged second re mission can be achieved in many patients with testicular leukemia. The patients with testicular relapse off initial therapy fared significantly better than patients on therapy. So, to improve the DFS for boys with testicular leukemia, a better understanding of its biology and prognostic factors is needed.
Biology ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Missions and Missionaries ; Orchiectomy ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Seoul ; Survival Rate ; Testis

BACKGROUND: In recent years, the incidence of extended-spectrum beta-lactamase (ESBL) producing Klebsiella has been steadily increased, and the newer species K. planticola and K terrigena, formerly regarded as nonpathogen, have been reported with astonishing frequency from human infectious processes by some investigators. The aim of this study is to elucidate the isolation rate and antimicrobial susceptibility of recent clinical Klebsiella isolates. METHOD: For the clinical Klebsiella isolates during the period of June 1999 to May 2000, isolation frequency of Klebsiella species by specimen, departments, age, and sex were analyzed. And antimicrobial susceptibilities were also analyzed. RESULT: Isolation rate of Klebsiella in order of decreasing frequency were K. pneumoniae (74:7%), K. oxytoca (12.1%), K. ozaenae(1.7%), K. planticola(1.0%), K. terngena(0.9%), and K, ornithinolytica (0.7%), respectively. K. rhinoscleromatis was not isolated. Compared with outpatients, increase of resistance rates of inpatients's Klebsiella isolates were 10% in ciprofloxacin, 15% in cefoperazone/sulbactam, and the others were ranged from 24% to 31%. Isolation rate of ESBL producing K. pneumoniae by double disk (DD) synergy test was 41%, and detection rates by antimicrobial agents were as follows: cefotaxime (95%), aztreonam (58%), and ceftriaxone (37%). Antimicrobial susceptibility rate with the exception of ampicillin and imipenem decreased from the range of 81%-96% on admission day to 29-62% after one week on admission. CONCLUSION: The isolation rates of K. planticola and K. terrigena were less than 1%. The proportion of ESBL producing K. pneumoniae was 41 %. And the vast majority of multidrug resistant Klebsiella including ESBL producing strains are acquired by hospitalization.
Ampicillin ; Anti-Infective Agents ; Aztreonam ; beta-Lactamases ; Cefotaxime ; Ceftriaxone ; Ciprofloxacin ; Epidemiology* ; Hospitalization ; Humans ; Imipenem ; Incidence ; Klebsiella pneumoniae ; Klebsiella* ; Outpatients ; Pneumonia ; Research Personnel

BACKGROUND: In recent years, the incidence of extended-spectrum beta-lactamase (ESBL) producing Klebsiella has been steadily increased, and the newer species K. planticola and K terrigena, formerly regarded as nonpathogen, have been reported with astonishing frequency from human infectious processes by some investigators. The aim of this study is to elucidate the isolation rate and antimicrobial susceptibility of recent clinical Klebsiella isolates. METHOD: For the clinical Klebsiella isolates during the period of June 1999 to May 2000, isolation frequency of Klebsiella species by specimen, departments, age, and sex were analyzed. And antimicrobial susceptibilities were also analyzed. RESULT: Isolation rate of Klebsiella in order of decreasing frequency were K. pneumoniae (74:7%), K. oxytoca (12.1%), K. ozaenae(1.7%), K. planticola(1.0%), K. terngena(0.9%), and K, ornithinolytica (0.7%), respectively. K. rhinoscleromatis was not isolated. Compared with outpatients, increase of resistance rates of inpatients's Klebsiella isolates were 10% in ciprofloxacin, 15% in cefoperazone/sulbactam, and the others were ranged from 24% to 31%. Isolation rate of ESBL producing K. pneumoniae by double disk (DD) synergy test was 41%, and detection rates by antimicrobial agents were as follows: cefotaxime (95%), aztreonam (58%), and ceftriaxone (37%). Antimicrobial susceptibility rate with the exception of ampicillin and imipenem decreased from the range of 81%-96% on admission day to 29-62% after one week on admission. CONCLUSION: The isolation rates of K. planticola and K. terrigena were less than 1%. The proportion of ESBL producing K. pneumoniae was 41 %. And the vast majority of multidrug resistant Klebsiella including ESBL producing strains are acquired by hospitalization.
Ampicillin ; Anti-Infective Agents ; Aztreonam ; beta-Lactamases ; Cefotaxime ; Ceftriaxone ; Ciprofloxacin ; Epidemiology* ; Hospitalization ; Humans ; Imipenem ; Incidence ; Klebsiella pneumoniae ; Klebsiella* ; Outpatients ; Pneumonia ; Research Personnel

OBJECTIVE: To understand the pathogenesis of adenomyosis through the immunohistochemical staining with bcl-2 and matrix metalloproteinase-9 (MMP-9) antibody. METHODS: We selected 45 cases of adenomyosis among the uterine specimens that were extirpated surgically due to benign gynecologic problem. Immunohistochemical staining to the myometrial tissue was performed using anti-bcl-2 and anti-MMP-9 antibodies. Staining was qualitatively assessed in terms of extent and intensity. RESULTS: There were 32 cases of proliferative phase and 13 cases of secretory phase depending on the date of endometrium. Anti-bcl-2 stained 81.3% (26/32) compared with anti-MMP-9 stained 6.3% (2/32) in the glandular tissue of the proliferative phase (p<.001). In the secretory phase, anti-bcl-2 92.3% (12/13) and anti- MMP-9 7.7% (1/13) respectively (p<.001). In the stromal tissue of the proliferative phase, anti-bcl-2 stained 62.5% (20/32) compared with anti- MMP-9 stained 6.3% (2/32) (p<.001). In the secetory phase, anti-bcl-2 84.6% (11/13) and anti-MMP-9 15.4% (2/13) (p<.001). CONCLUSION: The adenomyosis may be developed from simple invagination and cellular proliferation of endometrial tissue, not caused by myometrial infiltration of endometrial tissue.
Adenomyosis* ; Antibodies ; Cell Proliferation ; Endometrium ; Female ; Matrix Metalloproteinase 9

A balanced insertion of a parent may produce normal or carrier offsprings, spontaneous abortions, and chromosomally unbalanced offsprings. This is a rare report of duplication of chromosome 10q22->q24 which has arisen through the segregation of a balanced direct insertion. The partial trisomy 10q is a specific syndrome of mental retardation and malformation characterized by psychomotor delay, growth restriction, distinctive dysmorphic appearances, and, in some cases, cardiac, renal, and ocular abnormalities. We have experienced a case of partial trisomy 10q from maternal balanced insertion, which was prenatally showed severe fetal growth restriction and oligohydramnios, and present it with a brief review of literatures.
Abortion, Spontaneous ; Female ; Fetal Development ; Humans ; Intellectual Disability ; Oligohydramnios ; Parents ; Pregnancy ; Trisomy*

Intussusception is a rare cause of intestinal obstruction in adults and is most often due to a primary abnormality of the bowel, which serves as the leading point. Idiopathic intussusception in adults is distinctly uncommon, comprising 10% of diagnosed intussusceptions. We report a case of a spontaneous jejunal intussusception in a 48-year-old man that developed shortly after an open colectomy. The 48-year-old man, with no history of a laparotomy, underwent a left hemicolectomy and a left hemihepatectomy for descending colon cancer with liver metastasis. For 14 postoperative days, the patient complained of ileus, and conservative management with a long intestinal tube failed. When the patient underwent a laparotomy, intussusception of the mid jejunum was observed. The intussusception was resected, and no underlying bowel abnormality was identified. This report highlights the importance of considering this rare etiology in patients with ileus who have recently undergone a laparotomy.
Adult ; Colectomy ; Colon, Descending ; Humans ; Ileus ; Intestinal Obstruction ; Intussusception ; Jejunum ; Laparotomy ; Liver ; Middle Aged ; Neoplasm Metastasis

PURPOSE: We evaluated the response to immunomodulation therapy, long-term survival and relapse rate of aplastic anemia during follow-up for more than 1 year. METHODS: Twenty-eight children, with moderate to severe aplastic anemia were followed for more than one year and 7 children expired after therapy, were analyzed. Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG) by itself was given to 27 patients, and cyclosporine A (CsA) combined with ALG was given to 8 patients. ALG (ATG) was administered for 8 days in 20mg/kg/day when used alone, and for 5 days in 10mg/kg/day when combined with CsA. CsA was orally administered on the 14th day till 180th day of therapy, with 5mg/kg/day for first 14 days and 3mg/kg/day thereafter. RESULTS: Fifteen out of 35 patients (43%) showed a response. Median interval to response was 3 month (1-40 months). Response rate to ALG + CsA was 50%, compared to 41% in ALG (or ATG) alone. The interval of aplastic anemia symptoms to treatment showed a tendency to be shorter in responders (4.1 +/- 2.2 months) than in non-responders (17.5 +/- 6.4 months). Relapse occurred in 3 of 15 responders (20%). Evolution to secondary clonal hematologic disorders was not observed. Overall actuarial survival at 3 year was 78.2%. CONCLUSION: The results of immunomodulation therapy suggest that response would be better for patients whose interval from symptoms of aplastic anemia to treatment is short. Our results also suggest that ALG+CsA would be a better treatment modality to improve response rate.
Anemia, Aplastic* ; Antilymphocyte Serum ; Child ; Cyclosporine ; Follow-Up Studies* ; Humans ; Immunomodulation* ; Recurrence*

PURPOSE: A review of the literature for patients with peripheral primitive neuroectodermal tumor (PPNET; peripheral neuroepithelioma) suggests that the outcome is usually poor. We performed a retrospective study to emphasize the need for triple combined therapy of PPNET. METHODS: The clinical data of 10 patients (M:F=1:1, aged 1 8/12~14 3/12 years) with PPNET seen at Seoul National University Children's Hospital from Jan. 1991 to Jun. 1998 were reviewed. RESULTS: The primary sites were head and neck, chest, limbs, and retroperitoneal area in order of frequency. A palpable mass was the major symptom. The median duration of symptoms before diagnosis was 3 months. Metastatic disease at diagnosis was found in 3 patients, 2 of them were multiple. All of the patients had combined modality treatment with surgery, chemotherapy and radiation therapy. The 3-year relapse-free survival rate was 64%, and the overall 5-year survival rate was 66%. CONCLUSION: Best results were obtained with combined therapy with combination chemotherapy, extensive surgery and local radiation therapy.
Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Extremities ; Head ; Humans ; Neck ; Neuroectodermal Tumors, Primitive* ; Retrospective Studies ; Seoul ; Survival Rate ; Thorax

PURPOSE: We have undertaken this study to evaluate the effects of induction chemotherapy involving BH-AC, idarubicin, and 6-thioguanine (6-TG). METHODS: BH-AC 300mg/m2/day was administered intravenously over three hours for seven consecutive days. Idarubicin 12mg/m2/day was administered intravenously for three days. 6-TG 100 mg/m2/day was administered orally for seven days. Intrathecal ara-C was administered on the first day of treatment. RESULTS: Complete remission (CR) was achieved in 18 cases (66.7%), partial remission (PR) was achieved in 2 cases (7.4%). In previously untreated patients, complete reimission rate was 92.9% (13/ 14), in relapsed patients, 40% (2/5) and in the refractory patients, 37.5% (3/8). The remission duration until December 1996 was 45 to 630 days (median 133). Duration of the neutropenia (ANC<500/microliter) was 0 to 38 days (median 24). Side effects were nausea, vomiting (7/27, 25.9%), liver dysfunction (1/27, 3.7%), skin eruption (1/27, 3.7%), and mucositis (1/27, 3.7%). In all cases, fever developed in the neutropenic state (culture proven sepsis in 5 cases). Death occurred in 5 cases who achieved CR due to sepsis after chemotherapy (4 cases), intracerebral hemorrhage after bone marrow relapse (1 case). CONCLUSION: BH-AC, idarubicin, and 6-TG induction chemotherapy could be a useful induction chemotherapy treatment that combines supportive care for infection and bleeding.
Bone Marrow ; Cerebral Hemorrhage ; Cytarabine ; Drug Therapy ; Fever ; Hemorrhage ; Humans ; Idarubicin* ; Induction Chemotherapy* ; Leukemia, Myeloid, Acute* ; Liver Diseases ; Mucositis ; Nausea ; Neutropenia ; Recurrence ; Sepsis ; Skin ; Thioguanine* ; Vomiting