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2.
Korean Journal of Hospice and Palliative Care ; : 208-218, 2015.
Article in Korean | WPRIM | ID: wpr-76660

ABSTRACT

PURPOSE: Although a Do-Not-Resuscitate (DNR) order is widely in use, it is one of the challenging issues in end-of-life care. This study was conducted to investigate attitudes toward DNR according to education and clinical experience. METHODS: Data were collected using a structured questionnaire comprising 30 items in a tertiary hospital in Seoul, Korea. RESULTS: Participants were 238 nurses and 72 physicians. Most participants (99%) agreed to the necessity of DNR for reasons such as dignified death (52%), irreversible medical condition (23%) and patients' autonomy in decision making (19%). Among all, 33% participants had received education about DNR and 87% had DNR experience. According to participants' clinical DNR experience, their attitudes toward DNR significantly differed in terms of the necessity of DNR, timing of the DNR consent and post-DNR treatments including antibiotics. However, when participants were grouped by the level of DNR education, no significant difference was observed except in the timing of the DNR consent. CONCLUSION: This study suggests that the attitudes toward DNR were more affected by clinical experience of DNR rather than education. Therefore, DNR education programs should involve clinical settings.


Subject(s)
Anti-Bacterial Agents , Decision Making , Education , Korea , Professional Practice , Resuscitation Orders , Seoul , Tertiary Care Centers
3.
Allergy, Asthma & Immunology Research ; : 156-162, 2014.
Article in English | WPRIM | ID: wpr-19425

ABSTRACT

PURPOSE: Identification of tolerable alternative analgesics is crucial for management in nonsteroidal anti-inflammatory drug (NSAID)-sensitive patients. We investigated cross-reactivity of acetaminophen and celecoxib according to the type of aspirin/NSAID hypersensitivity and aimed to determine the risk factors for cross-intolerance. METHODS: We retrospectively reviewed the medical records of patients intolerant to aspirin and NSAIDs who had undergone an acetaminophen and/or celecoxib oral provocation test. Aspirin/NSAID hypersensitivity was classified into 4 types according to a recently proposed classification: aspirin-exacerbated respiratory disease (AERD), aspirin-exacerbated chronic urticaria (AECU), aspirin-induced acute urticaria/angioedema (AIAU), and NSAID-induced blended reaction (NIRD). RESULTS: A total of 180 patients with hypersensitivity to aspirin and NSAIDs were enrolled; 149 acetaminophen provocation test results and 145 celecoxib provocation test results were analyzed. The overall cross-reaction rates to acetaminophen and celecoxib were 24.8% and 10.3%, respectively. There was a significant difference in the cross-reactivity to acetaminophen according to the type of NSAID hypersensitivity. Cross-reactivity to acetaminophen was highest in the AECU group (43.9%), followed by the AERD (33.3%), NIBR (16.7%), and AIAU (12.5%) groups. Underlying chronic urticaria was more prevalent in patients with cross-intolerance to both acetaminophen (P=0.001) and celecoxib (P=0.033). Intolerance to acetaminophen was associated with intolerance to celecoxib (P<0.001). CONCLUSIONS: Acetaminophen and celecoxib may induce adverse reactions in a non-negligible portion of aspirin/NSAID-sensitive patients. Physicians should be aware of the possible cross-reactions of these alternative drugs and consider an oral challenge test to confirm their tolerability.


Subject(s)
Humans , Acetaminophen , Analgesics , Anti-Inflammatory Agents , Anti-Inflammatory Agents, Non-Steroidal , Aspirin , Classification , Cross Reactions , Drug Hypersensitivity , Hypersensitivity , Medical Records , Methods , Retrospective Studies , Risk Factors , Urticaria , Celecoxib
4.
Dementia and Neurocognitive Disorders ; : 1-8, 2013.
Article in Korean | WPRIM | ID: wpr-88128

ABSTRACT

BACKGROUND: The usefulness of cerebrospinal fluid (CSF) concentrations of amyloid beta protein 1-42 (Abeta42), phosphorylated tau (pTau) and total tau (tTau) have been increasing in Alzheimer's disease (AD). However, the direct adoption of previously reported standard values is not appropriate due to interlaboratory variability. We started this study to set up an accessible system to measure CSF biomarkers in our country with high reproducibility and validity. METHODS: Including CSFs from four different institutes the levels of Abeta42, pTau181 and tTau were measured in one lab. The intertest variability and difference in the levels of biomarkers depending on diseases were assessed. Through analysis of receiver operating characteristic cut points and binary logistic regression the cut-off values of Abeta42, pTau and tTau level were obtained, and their validity was evaluated. RESULTS: The intertest consistency was high in measuring CSF biomarkers. The value of Abeta42 was markedly decreased in AD (n= 17) and other dementia (n= 9) compared to normal control (n= 12). The levels of pTau181 and tTau were high in AD, but not in other dementia and normal control. The threshold values of Abeta42, pTau181 and tTau were 290.3 pg/mL, 54.3 pg/mL, and 320.7 pg/mL in differentiating AD from normal control showing high sensitivity and specificity. Especially, the ratios of pTau181/Abeta42 (> 0.16) and tTau/Abeta42 (> 0.76) showed the prime validity. CONCLUSIONS: Our data of CSF Abeta42, pTau181, and tTau levels were highly reproducible. PTau181/Abeta42 and tTau/Abeta42 ratios were the greatly helpful in differentiating AD from normal control.


Subject(s)
Academies and Institutes , Adoption , Alzheimer Disease , Amyloid beta-Peptides , Biomarkers , Dementia , Enzyme-Linked Immunosorbent Assay , Logistic Models , Pyridines , ROC Curve , Sensitivity and Specificity , Thiazoles
5.
Asia Pacific Allergy ; (4): 161-164, 2012.
Article in English | WPRIM | ID: wpr-749893

ABSTRACT

Food-dependent exercise-induced anaphylaxis (FDEIA) is a type of exercise-induced anaphylaxis associated with postprandial exercise. We describe a 19-year-old man with FDEIA. Our patient complained of urticaria, angioedema, dizziness and hypotension associated with exercise after ingestion of walnut-containing foods in a warm environment. Skin prick test and prick to prick test were positive for walnut antigen. The attack didn't occur by free running outside for 10 min 2 h after taking walnuts, and the temperature was about


Subject(s)
Humans , Young Adult , Anaphylaxis , Angioedema , Cold Temperature , Dizziness , Eating , Hot Temperature , Humidity , Hypotension , Juglans , Running , Skin , Urticaria
6.
Asia Pacific Allergy ; (4): 168-171, 2011.
Article in English | WPRIM | ID: wpr-749871

ABSTRACT

Human seminal plasma allergy is a rare phenomenon. Its clinical manifestations are diverse, and range from mild local pruritus to fatal anaphylaxis. Treatment varies with severity of the reactions: abstinence, condom usage or immunotherapy (subcutaneous or intravaginal) with seminal fluid. Local allergic reactions can be managed by prophylactic use of antihistamines or local cromolyn cream. A 33-year-old female visited the Asthma and Allergy Clinic in Seoul National University Bundang Hospital for the recurrent generalized urticarial reactions after sexual intercourse. She had been suffering from asthma, allergic rhinoconjunctivitis and atopic dermatitis for 10 years. She gave birth to a baby 6 months ago and no problem before. However, recently she began to recognize unexpected generalized urticaria that occurred after the sexual intercourse with husband. She wanted to have the second baby but hesitated because of the recurrent symptoms after the intercourse. She showed positive response to skin prick test with her husband's seminal fluid. The IgE-binding components were 15, 22, 28, and 35 kDa. Considering her moderate cutaneous reactions, we decided to try prophylactic treatments with oral anti-histamine one hour before sexual intercourse. She did not experience urticarial reactions with intercourse while oral anti-histamine was administered in advance. Finally, treatment outcome was successful, and the couple successfully gave birth to their second baby. We suppose that prophylactic antihistamine may be also applied in seminal plasma allergy patients if systemic reactions are limited to mild to moderate generalized urticaria.


Subject(s)
Adult , Female , Humans , Pregnancy , Anaphylaxis , Asthma , Coitus , Condoms , Cromolyn Sodium , Dermatitis, Atopic , Histamine Antagonists , Hypersensitivity , Immunotherapy , Parturition , Pruritus , Semen , Seoul , Skin , Spouses , Treatment Outcome , Urticaria
7.
Korean Journal of Medicine ; : S183-S187, 2011.
Article in Korean | WPRIM | ID: wpr-209161

ABSTRACT

Retinoids are effective systemic agents in the treatment of psoriasis. Acitretin, a synthetic aromatic derivative of retinoic acid, has replaced etretinate in retinoid therapy of psoriasis because of its more favorable pharmacokinetic profile, including a significantly shorter half-life. Most of the adverse effects associated with acitretin are teratogenicity, hepatotoxicity, pseudotumor cerebri, pancreatitis, hyperlipidemia, hyperostosis, and mucocutaneous side effects. There are two reports worldwide describing patients who developed acute respiratory distress syndrome associated with acitretin. This suggests the possibility of serious lung complications associated with acitretin. We report a case of a 61-year-old man who developed interstitial pneumonitis that might have been induced by acitretin during the treatment of pustular psoriasis. In these cases, immediate withdrawal of retinoic acid is necessary, and corticosteroid therapy should be considered.


Subject(s)
Humans , Middle Aged , Acitretin , Etretinate , Half-Life , Hyperlipidemias , Hyperostosis , Lung , Lung Diseases, Interstitial , Pancreatitis , Pseudotumor Cerebri , Psoriasis , Respiratory Distress Syndrome , Retinoids , Tretinoin
8.
Korean Journal of Medicine ; : S319-S324, 2011.
Article in Korean | WPRIM | ID: wpr-152505

ABSTRACT

Catastrophic antiphospholipid syndrome (APS) is an accelerated subtype of APS that results in multiorgan failure. Although catastrophic APS represents about 0.8% of all APS cases, it is usually a life-threatening medical condition that requires high clinical awareness. Catastrophic APS has been managed by various therapies, including anticoagulation, corticosteroids, plasma exchange and IV immunoglobulin, but it still has a high mortality rate. A few cases treated by anticoagulation and steroids have been reported in Korea. In this paper, we report a case of catastrophic APS that improved after anticoagulation therapy alone. Thus, we consider hat our case shows another clinical aspect of catastrophic APS.


Subject(s)
Adrenal Cortex Hormones , Antiphospholipid Syndrome , Immunoglobulins , Korea , Plasma Exchange , Steroids
9.
Infection and Chemotherapy ; : 60-63, 2011.
Article in Korean | WPRIM | ID: wpr-41921

ABSTRACT

Fournier's gangrene is a rare, rapidly progressive, necrotizing fasciitis of the genital, perianal and perineal regions. It is usually caused by a synergistic infection of aerobic and anaerobic organisms. A 51-year-old male patient with diabetes mellitus and chronic renal insufficiency on peritoneal dialysis was admitted with severe scrotal pain and swelling that lasted 4 days. An emergent non-contrast-enhanced computed tomography revealed extensive gas formation, fatty haziness and edema in the left scrotal and inguinal area. The patient was successfully treated with immediate surgical debridements and antibiotics. Streptococcus anginosus group Streptococcus anginosus was isolated from surgical wound cultures and identified with biochemical identification methods and direct sequencing of DNA. Herein, we report a rare case of Fournier's gangrene caused by S. anginosus. We also review the relevant literature


Subject(s)
Humans , Male , Middle Aged , Anti-Bacterial Agents , Debridement , Diabetes Mellitus , DNA , Edema , Fasciitis, Necrotizing , Fournier Gangrene , Peritoneal Dialysis , Renal Insufficiency, Chronic , Streptococcus , Streptococcus anginosus
10.
Korean Journal of Gastrointestinal Endoscopy ; : 290-293, 2010.
Article in Korean | WPRIM | ID: wpr-214184

ABSTRACT

Endoscopic injection sclerotherapy is an effective and relatively safe modality for controlling bleeding esophageal varices. Injection of sclerosant causes acute mural thrombosis with a necroinflammatory response and subsequent sclerosis in the venous system of the distal esophagus. A few cases of mesenteric venous thrombosis with small bowel infarction after sclerotherapy have been reported, and most of which were fatal. The association between mesenteric venous thrombosis and sclerotherapy has been strongly suggested, but this still remains unproved. We report here on a case of mesenteric venous thrombosis with small bowel infarction that developed after endoscopic injection sclerotherapy.


Subject(s)
Esophageal and Gastric Varices , Esophagus , Hemorrhage , Infarction , Sclerosis , Sclerotherapy , Thrombosis , Venous Thrombosis
11.
The Journal of the Korean Rheumatism Association ; : 91-95, 2007.
Article in Korean | WPRIM | ID: wpr-78258

ABSTRACT

Rectus sheath hematoma is an uncommon cause of acute abdomen, caused by a tear in deep epigastric vessels or its branches. It occurs spontaneously, after trauma or surgery. Rectus abdominus myonecrosis is a life threatening complication of rectus sheath hematoma. We report a case with fatal rectus sheath hematoma complicated by rectus abdominus myonecrosis caused by ischial fracture in a chronic active rheumatoid arthritis patient.


Subject(s)
Humans , Abdomen, Acute , Arthritis, Rheumatoid , Hematoma
12.
Korean Journal of Obstetrics and Gynecology ; : 117-125, 2007.
Article in Korean | WPRIM | ID: wpr-224170

ABSTRACT

OBJECTIVE: The purpose of this study was to evaluate the incidence of abdominal-wall tumor implantation after laparoscopic procedure in patients with gynecologic malignancies. METHODS: The records of 184 patients who had a laparoscopic operations or laparotomy after laparoscopic diagnostic procedures from Aug. 1994 to Aug. 2003 in our hospital were reviewed. The presence of metastasis at trocar site of laparoscopic surgery and incision site of laparotomy was examined. RESULTS: Abdominal-wall tumor implantations were developed at two port site in one patient. This result showed an incidence of 0.24% (2/819), as 2 ones in 819 abdominal trocar sites and 0.5% (1/184), as 1 in 184 procedures. This patient had a FIGO stage III a, grade2 adenocarcinoma of endometrium and underwent laparoscopic modified radical hysterectomy with both pelvic lymphadenectomy. In addition, tumor implantation was occurred at laparotomy skin incision site in one patient, a incidence of 2.2% (1/45), as 1 in 45 laparotomy procedures. This patient had a stage II, squamous cell carcinoma of the vagina, who had received second courses of Ifosfamide-Cisplantin neoadjuvant chemotherapy and open laparotomy with radical hysterectomy with upper vaginectomy was followed by laparoscopic pelvic lymphadenectomy due to fixed grossly metastatic nodes. CONCLUSION: Recently, the use of laparoscopic procedure in oncology was increased, the new complication such as abdominal-wall implantation at trocar site was introduced. The abdominal-wall implantation at trocar site could be prevented by patients selection, intraperitoneal and port-site lavage, surgical modification. And all patients should have careful follow up with special attention to the trocar sites. Port site implantation was rare, but could be occurred in the incidence of 0.5% per procedure.


Subject(s)
Female , Humans , Adenocarcinoma , Carcinoma, Squamous Cell , Drug Therapy , Endometrium , Follow-Up Studies , Hysterectomy , Incidence , Laparoscopy , Laparotomy , Lymph Node Excision , Neoplasm Metastasis , Skin , Surgical Instruments , Therapeutic Irrigation , Vagina
13.
Journal of the Korean Academy of Family Medicine ; : 1005-1012, 2006.
Article in Korean | WPRIM | ID: wpr-15660

ABSTRACT

BACKGROUND: In Korea, the law reguires submission of immunization certificate to elementary school on admission since 2001 in order to prevent measles outbreaks such as those that occurred in the year 2000 and 2001. As a result, the submission rate of immunization certificate has been almost 99%. To that effect it seems to have been a very successful policy. But the validity and accuracy of such certificates have not been guestioned. This study aimed to assess the validity of the measles immunization certificates and general practitioners' perception of the law to submit immunization certificate. METHODS: A postal survey was conducted from April to May 2005 among general practitioners who were pediatricians and family physicians. The main questions were the method to confirm childrens' past vaccination and the proportion of doing booster vaccination, and also their opinions on governments' policy to extend the submission of immunization certificate on school admission. RESULTS: The total study subjects were 840. The proportion of issued immunization certificates based on exact vaccination records or booster vaccination was 98.7%. The opinions concerning the law to submit immunization certificates were: very necessary (88.8%), and should include all children basic immunization (62.8%). On the other hand, the most common reason against the law was that they did not feel the necessity (31.4%). CONCLUSION: The validity of immunization certificates was very high. The opinions concerning the law to submit immunization certificates were favorable in most subjects. And mutual understanding between the general practitioners and the government is in demand before the law to submit immunization certificates becomes effect.


Subject(s)
Child , Humans , Disease Outbreaks , General Practitioners , Hand , Immunization , Jurisprudence , Korea , Measles Vaccine , Measles , Physicians, Family , Vaccination
14.
Korean Journal of Gastrointestinal Endoscopy ; : 67-70, 2006.
Article in Korean | WPRIM | ID: wpr-157139

ABSTRACT

Granular cell tumor (GCT) is a relatively rare benign tumor that can be located anywhere throughout the body, but it is uncommon in the gastrointestinal tract, and especially in the colon and rectum. A 41-year-old man visited our hospital with a three-month history of intermittent abdominal discomfort at the left lower quadrant area. Colonoscopic examination revealed a hemispheric, submucosal lesion, about 1.5 x 1.2 cm in size, on the opposite side of the ileocecal valve in the proximal ascending colon. The tumor was removed by an endoscopic snare without any immediate complication. The pathologic findings revealed spindled or polygonal large cells that were positive for S-100, neuron-specific enolase and vimentin, and they were negative for smooth muscle actin and desmin, which was consistent with granular cell tumor. We report here on a case of granular cell tumor of the colon that was successfully treated with endoscopic polypectomy.


Subject(s)
Adult , Humans , Actins , Colon , Colon, Ascending , Colonoscopy , Desmin , Gastrointestinal Tract , Granular Cell Tumor , Ileocecal Valve , Muscle, Smooth , Phosphopyruvate Hydratase , Rectum , SNARE Proteins , Vimentin
15.
The Journal of the Korean Rheumatism Association ; : 327-332, 2006.
Article in Korean | WPRIM | ID: wpr-153036

ABSTRACT

Nocardiosis is usually a subacute infection, which can occur as an opportunistic infections in patients with systemic lupus erythematosus. There are rare cases of nocardiosis concurrent with Mycobacterium tuberculosis. We report a case of intramuscular nocardial abscess concurrent with pulmonary tuberculosis in a patient with lupus nephritis. She has received cyclophosphamide pulse therapies and is receiving oral steroid therapy 3 months ago. After Nocardia farcinica and Mycobacterium tuberculosis were confirmed by PCR and PCR-RFLP, we initiated trimethoprim/ sulfamethoxazole and antituberculous agents. After then, patient was improved and discharged, maintaining the medications.


Subject(s)
Humans , Abscess , Cyclophosphamide , Lupus Erythematosus, Systemic , Lupus Nephritis , Mycobacterium tuberculosis , Nocardia Infections , Nocardia , Opportunistic Infections , Polymerase Chain Reaction , Sulfamethoxazole , Tuberculosis, Pulmonary
16.
Journal of the Korean Society of Echocardiography ; : 42-45, 2005.
Article in Korean | WPRIM | ID: wpr-212990

ABSTRACT

Rupture of the interventricular septum is a serious complication of acute myocardial infarction, accounting for 5% of death due to acute myocardial infarction. The mortality with medical therapy alone exceeds 90%. Accurate diagnosis, urgent management, and early operative correction are necessary for survival. We report cases of 3 patients with ventricular septal rupture after acute myocardial infarction.


Subject(s)
Humans , Diagnosis , Mortality , Myocardial Infarction , Rupture , Ventricular Septal Rupture
17.
Korean Journal of Pediatric Hematology-Oncology ; : 187-194, 2004.
Article in Korean | WPRIM | ID: wpr-74201

ABSTRACT

PURPOSE: Medulloblastoma is the most common malignant brain tumor in childhood. The standard treatments are composed of tumor resection, irradiation and chemotherapy. In this study, we analysed the outcome of high risk medulloblastoma patients who were treated with surgical resection followed by craniospinal irradiation and chemotherapy utilizing cisplatin, vincristine, cyclophosphamide and etoposide. METHODS: We conducted a retrospective analysis of medical record of twenty-five patients with high risk medulloblastoma, treated from January 1998 to April 2004 in the Department of Pediatrics, Neurosurgery and Radiation Oncology at Asan Medical Center. RESULTS: The median age at diagnosis was 9 years and 10 month. The 2-year overall survival rate was 80%, and 2-year progression-free survival rate was 71%. Degree of surgical resections or residual tumor did not show statistically significant differences of survival rate, but there was difference depending on metastasis staging. The side effects of chemotherapy were grade IV hematologic toxicity (n=20), SIADH (n=2), and severe paralytic ileus (n=1). The long-term sequelae were endocrinopathy (n=6) that include growth failure, precocious puberty and hypothyroidism. Neurological complications such as mild mental retardation and ataxia occurred in seven patients. There was no treatment-related mortality. Four patients died of tumor progression. CONCLUSION: Patients with high risk medulloblastoma treated with surgical resection followed by radiation and chemotherapy as described here show satisfactory outcome. In this high risk group, metastasis staging correlated with outcome but the degree of surgical resection and presence or absence of residual tumor at primary site did not correlate with outcome.


Subject(s)
Child , Humans , Ataxia , Brain Neoplasms , Cisplatin , Craniospinal Irradiation , Cyclophosphamide , Diagnosis , Disease-Free Survival , Drug Therapy , Etoposide , Hypothyroidism , Inappropriate ADH Syndrome , Intellectual Disability , Intestinal Pseudo-Obstruction , Medical Records , Medulloblastoma , Mortality , Neoplasm Metastasis , Neoplasm, Residual , Neurosurgery , Pediatrics , Puberty, Precocious , Radiation Oncology , Retrospective Studies , Survival Rate , Vincristine
18.
Korean Journal of Pediatric Hematology-Oncology ; : 264-272, 2001.
Article in Korean | WPRIM | ID: wpr-118590

ABSTRACT

PURPOSE: To improve survival and/or to avoid radiotherapy, high dose chemotherapy (HDCT) with autologous peripheral blood stem cell transplantation (PBSCT) was given to patients with recurrent or high risk medulloblastoma (MB)/primitive neuroectodermal tumor (PNET) as well as patients younger than 3 years of age. METHODS: Six patients (3 recurrent, 1 high risk, 2 younger than 3 years; 5 MBs and 1 PNET) received single or double HDCT and PBSCT with or without immunotherapy using interleukin-2. Chemotherapeutic regimen in the first HDCT included cyclophosphamide (1,500 mg/m2/ day for 4 days) and melphalan (60 mg/m2/day for 3 days). Chemotherapeutic regimen in the second HDCT included carboplatin (400 mg/m2/day for 3 days), thiotepa (250 mg/ m2/day for 3 days), and etoposide (200 mg/m2/day for 3 days). RESULTS: Nine HDCTs were applied in 6 patients. Three double HDCTs were rescued with peripheral blood stem cells collected during single round leukapheresis. Rapid hematologic recovery occurred in 4 patients. Engraft failure occurred in 1 patient and delayed granulocyte recovery and platelet engraft failure occurred in 1 patient. Three patients who had minimal disease before HDCT had event free survival for 7~18 months after HDCT. Tumor relapsed 8 and 12 months after single HDCT in 2 patients among 3 patients with recurrent MB/PNET. One patient with recurrent MB died due to engraft failure and sepsis. CONCLUSION: HDCT with autologous PBSCT is expected to improve survival of patients with poor prognosis MB/PNET including younger patients less than 3 years. Subsequent trials with larger number of patients and long-term follow-up are needed.


Subject(s)
Humans , Blood Platelets , Carboplatin , Cyclophosphamide , Disease-Free Survival , Drug Therapy , Etoposide , Follow-Up Studies , Granulocytes , Immunotherapy , Interleukin-2 , Leukapheresis , Medulloblastoma , Melphalan , Neural Plate , Neuroectodermal Tumors , Neuroectodermal Tumors, Primitive , Peripheral Blood Stem Cell Transplantation , Prognosis , Radiotherapy , Sepsis , Stem Cells , Thiotepa
19.
Korean Journal of Pediatric Hematology-Oncology ; : 58-66, 2001.
Article in Korean | WPRIM | ID: wpr-37335

ABSTRACT

PURPOSE: With the use of combined-modality therapy, the survival of the patients with the rhabdomyosarcoma (RMS) has been improved during past 30 years. The reports on the therapeutic outcome of RMS in Korea during past 10 years, however, have been scarce. The authors reviewed the clinical characteristics and treatment outcome of pediatric RMS patients diagnosed in our institution during recent 11 years' period and compared this data with previous reports. METHODS: From January 1990 to December 2000, 29 patients were diagnosed with RMS by histopathology. Retrospective analysis of the medical records of these patients was performed. RESULTS: A male to female ratio was 1.4:1. Age at diagnosis ranged from 8 months to 19 years 5 months and the median age was 8 years 5 months. Head and neck region that included orbit and parameningeal areas was the most common primary site (44.8%) and other common sites were genitourinary tract, extremities and perianal areas. The two major pathologic types of RMS were embryonal (72.4%) and alveolar (17.2%). The patient distribution of Intergroup Rhabdomyosarcoma Study (IRS) clinical group was as follows: Group I 13.8%, Group II 24.1%, Group III 27.6% and Group IV 34.5%. Of the 29 patients, 4 patients were lost to follow up or received no treatment. Among 25 patients treated and followed, 3-year overall survival rate of these patients was 84% and 3-year event-free survival (EFS) was 60%. Three-year EFS of Group I was 100%, Group II 71%, Group III 83% and Group IV 22%. CONCLUSION: The clinical characteristics of RMS in our institution were similar to those reported by IRS. But there were higher proportion of Group IV patients than IRS suggesting the delayed diagnosis of RMS in Korean patients. The treatment outcome was comparable to that of current IRS trials. This results suggest that the treatment outcome of RMS patients in Korea has improved largely owing to the adoption of current IRS protocols.


Subject(s)
Female , Humans , Male , Delayed Diagnosis , Diagnosis , Disease-Free Survival , Extremities , Head , Korea , Lost to Follow-Up , Medical Records , Neck , Neoplasm Staging , Orbit , Prognosis , Retrospective Studies , Rhabdomyosarcoma , Survival Rate , Treatment Outcome
20.
Korean Journal of Pediatric Hematology-Oncology ; : 269-277, 2000.
Article in Korean | WPRIM | ID: wpr-113922

ABSTRACT

PURPOSE: The frequency and clinical characteristics of primary childhood hepatic tumors diagnosed in a single institution were reviewed. Method: From January 1991 to June 2000, 33 patients were diagnosed with primary hepatic tumor. Retrospective analysis of the medical records of the various primary hepatic tumors was performed. RESULTS: Among the total of 33 patients with primary hepatic tumor, twenty five (76%) were malignant and eight (24%) were benign. The mean age of patients with hepatoblastoma (HB) was 32 months (12 of 16 patients were less than 3 years), 12.8 years in hepatocellular carcinoma (HCC) (all of them were older than 11 years), 2 months in hemangioendothelioma (HE), and 3 months in hamartoma. The ratio of male to female was 1:1 in HB, and 4:1 in HCC. The presence of hepatitis B infection was detected in 80% (4/5) of HCC and in 13% (2/16) of HB. The common presenting symptoms at diagnosis were the palpable mass, abdominal pain and jaundice. Many tumors were found incidentally without any presenting symptoms. Of 33 patients, 22 had involvement at right lobe. HCC had more necrosis and hemorrhage within the tumor than HB on imaging study. HE had a distinct feature of contrast enhancement increasing from the peripheral portion of the mass to central portion. Hamartoma showed a feature with multiple cysts and septums within the mass. To confirm the diagnosis, a tissue biopsy was done in 23 patients (70%). A significantly elevated level of serum alpha-fetoprotein (AFP) is found in 88% (mean 386,000 ng/mL) of HB cases and 100% (mean 369,000 ng/mL) of HCC. The mean AFP level in HB patients was 391,000 ng/mL at diagnosis, 52 ng/mL after the preoperative chemotherapy, 8.3 ng/mL at postoperative follow-up, and 2.8 ng/mL at completion of chemotherapy. In HCC, the AFP level decreased temporarily after chemotherapy or chemo- embolization, but increasesd at recurrence in most case. Patients resectable at the time of diagnosis or after preoperative chemotherapy were 12 (75%) in HB, and 10 of them are were alive with median the follow-up of 34 months (5 mo.~8 yrs 7 mo.). All the HCC patients were unresectable or had metastatic lesion at diagnosis, and all of them were assumed dead following discharge. Conclusions: Among the primary hepatic tumors in children, malignant tumors are more frequent than benign. Age of onset, AFP level and imaging studies all play an important role in diagnosis, and biopsy is usually confirmative. HCC has an extremely poor prognosis in childhood. HB patients with complete surgical resection enjoy a relatively high long term survival.


Subject(s)
Child , Female , Humans , Male , Abdominal Pain , Age of Onset , alpha-Fetoproteins , Biopsy , Carcinoma, Hepatocellular , Diagnosis , Drug Therapy , Follow-Up Studies , Hamartoma , Hemangioendothelioma , Hemorrhage , Hepatitis B , Hepatoblastoma , Jaundice , Medical Records , Necrosis , Prognosis , Recurrence , Retrospective Studies
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