BACKGROUND/AIMS: In 1977, Teele and Smith reported their positive experience using an ultrasonographic in the evaluation of Congenital Hypertrophic Pyloric Stenosis (CHPS). Since that time, ultrasonography has been advocated by some as the diagnostic procedure of choice, although a number of false-negative cases have been encountered at any rate, in 1994, Becker reported that an endoscopy was far more accurate than an ultrasonography, the diagnosis being made in 97% and 81% of the cases. Therefore, we performed a prospective study to evaluate the diagnostic value of an upper gastrointestinal endoscopy in CHPS patients. METHODS: We reviewed the clinical records of 12 pediatric patients experiencing vomiting, who had visited Pusan National University Hospital from January 1993 to March 1997. We performed on upper gastrointestinal endoscopy and abdominal ultrasonography simultaneously in order to evaluate the diagnostic value of an upper gastrointestinal endoscopy in CHPS patients. RESULTS: The male to female ratio was 11: 1. In 7 of 12 patients, 58%, vomited within 4 weeks after birth. Diagnostic sensitivity by abdominal ultrasonography was about 75%, and by upper GI endoscopy, almost 100%. Associated lesions were discovered in 5 cases, 3 esophagitis and 2 gastritis in the upper GI endoscopy. CONCLUSIONS: The upper GI endoscopy was more accurate in the diagnosis of CHPS than the ultrasonography, and was more effective in the diagnosis of associated lesions.
Busan ; Diagnosis* ; Endoscopy ; Endoscopy, Gastrointestinal* ; Esophagitis ; Female ; Gastritis ; Humans ; Male ; Parturition ; Prospective Studies ; Pyloric Stenosis, Hypertrophic* ; Ultrasonography ; Vomiting

Agenesis of dorsal pancreas is a rare congenital anomaly that arises from the failure of the dorsal pancreatic bud of endodermal cells to form the body and tail of the pancreas. It may be associated with diabetes mellitus, pancreatic exocrine dysfunction, or abdominal pain. Complete or partial agenesis of dorsal pancreas has been reported in a small number of pediatric and adult patients. A case is herein described involving a complete agenesis of dorsal pancreas and diabetes mellitus. A 38-year-old man with a 7-months history of non-insulin dependent diabetes mellitus was admitted due to weight loss and abdominal pain. Abdominal ultrasonography and computed tomography showed a normal biliary tree and enlarged head of the pancreas without visualization of the pancreatic body and tail. Endoscopic retrograde cholangiopancreatography (ERCP) revealved the short duct of Wirsung in the uncinate process and a head without opacification of any ducts in the pancreatic body or tail. The patient underwent explo-laparotomy for evaluation of the suspected pancreatic cancer. The patient was diagnosed as having complete agenesis of the dorsal pancreas by ERCP, CT, and surgery.
Abdominal Pain ; Adult ; Biliary Tract ; Cholangiopancreatography, Endoscopic Retrograde ; Diabetes Mellitus ; Endoderm ; Head ; Humans ; Pancreas* ; Pancreatic Ducts ; Pancreatic Neoplasms ; Ultrasonography ; Weight Loss

Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while single photon emission computed tomography imaging showed hypoperfusion in both temporal lobes. Ultrasound and MRI of the abdomen revealed a left ovarian cystic mass. The patient experienced a significant recovery of cognitive function after surgical resection of the tumor, which was pathologically identified as a mature ovarian teratoma, and treatment with intravenous immunoglobulin.
Abdomen ; Adolescent ; Brain ; Female ; Humans ; Immunoglobulins ; Limbic Encephalitis ; Magnetic Resonance Imaging ; Memory Disorders ; Neoplasm Metastasis ; Nervous System ; Ovarian Cysts ; Seizures ; Temporal Lobe ; Teratoma ; Tomography, Emission-Computed, Single-Photon ; Unconsciousness

BACKGROUND: This study investigated the clinical characteristics and risk factors of the severity of pandemic influenza A (H1N1) 2009 infection in pediatric patients in Busan and Gyeongsangnam-do. METHODS: Cases of influenza A (H1N1) 2009 in patients under the age of 18 years, confirmed by reverse transcription polymerase chain reaction, at Pusan National University Hospital and Pusan National University Yangsan Hospital from the last week of August 2009 through the last week of February 2010 were retrospectively analyzed. RESULTS: Of the 3,777 confirmed cases of influenza A (H1N1) 2009, 2,200 (58.2%) were male and 1,577 (41.8%) were female. The average age of the patients was 8.4+/-4.8 years. The total cases peaked during 44th to 46th week. Most of the patients were in the 5- to 9-year-old age group. Oseltamivir was administered to 2,959 (78.3%) of the patients. 221 patients (5.9%) were hospitalized, age an average of 6.7+/-4.5 years. The average duration of hospitalization was 7.4+/-5.6 days. One hundred cases (45.2%) had pneumonia. Risk factors for hospitalization included male gender, <2 years of age, and underlying disease. Children with asthma were at very high risk of hospitalization, over 20 times the non-asthmatic children (odds ratio [OR], 21.684; confidence interval [CI], 13.295~39.791). Likewise the children with neurologic deficits faced a 16 times higher risk (OR, 15.738; CI, 7.961~31.111). Ten of the patients (4.5%) were admitted to the intensive care unit, and eight (3.6%) required mechanical ventilation. CONCLUSION: Of the pediatric patients with pandemic influenza A (H1N1) 2009, most of the patients were in the 5- to 9-year-old age group. Risk factors for hospitalization included male gender, <2 years of age, and underlying disease. The most common complication was pneumonia. The very high risk of severe morbidity in children with asthma or neurologic disease shows the critical importance of targeted vaccine coverage, special awareness and swift care by both guardians and primary care providers.
Asthma ; Child ; Epidemiologic Study Characteristics as Topic ; Female ; Hospitalization ; Humans ; Influenza A Virus, H1N1 Subtype ; Influenza, Human ; Intensive Care Units ; Male ; Neurologic Manifestations ; Oseltamivir ; Pandemics ; Pediatrics ; Pneumonia ; Polymerase Chain Reaction ; Primary Health Care ; Retrospective Studies ; Reverse Transcription ; Risk Factors

Secondary polycythemia is a condition for which the red blood cell count is increased due to the physiologic response to hypoxia, autonomous erythropoietin production or deliberate erythropoietin administration. Hydronephrosis and postrenal obstruction have rarely been reported as the causes of secondary polycythemia. We report here on a case of a 30-year- old man with secondary polycythemia that was caused by giant hydronephrosis due to ureteropelvic junction obstruction, and this patient was successfully treated by nephrectomy.
Anoxia ; Erythrocyte Count ; Erythropoietin ; Humans ; Hydronephrosis* ; Nephrectomy ; Polycythemia*

BACKGROUND: Infections and vascular disorders are the two most widely accepted probable causes of sudden hearing loss. Tumor necrosis factor alpha (TNF-alpha) is major pro-inflammatory cytokine that is thought to be important in the pathogenesis of sudden deafness. However, the functions of genetic polymorphism in this cytokine have not been throughly examined in the context of sudden deafness pathology. In an effort to discover polymorphism in genes whose variants have been implicated in sudden deafness phenotypes, we examined the genetic effects of TNF-alpha polymorphisms in Koreans with sudden deafness. METHODS: Two common single nucleotide polymorphism (SNP) in TNF-alpha gene were genotyped in a Korean sudden deafness. Ninety nine patients with sudden deafness (45 males and 54 females) were selected from Keimyung University Dongsan Medical Center. The control subjects consisted of healthy 285 males and 319 females. RESULTS: Human genomic DNA was extracted from peripheral blood sample. The SNP at position -863 C/A and -857 C/T of TNF-alpha promoter were analyzed by PCR and pyrosequencing. Genotype distribution and allele frquencies in subjects were in Hardy-Weinberg equilibrium (p>0.05). No significant association was found between TNF-alpha -863 C/A and -857 C/T polymorphism and sudden deafness. We examined whether the relation between TNF-alpha polymorphism and sudden deafness varied according to tinnitus. Statistical analysis of TNF-alpha polymorphism at -857 C/T showed that there was a significant difference between SD without tinnitus and the control in both genotype distribution (p<0.05) and allele frequency [OR (95% CI)=2.63 (1.29-5.34)], but not between SD with tinnitus. CONCLUSION: These findings suggest TNF-alpha polymorphisms at -863C/A, -857 C/T are likely to play a role in SD.
Alleles ; DNA ; Female ; Gene Frequency ; Genotype ; Hearing Loss, Sudden* ; Humans ; Male ; Pathology ; Phenotype ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide* ; Tinnitus ; Tumor Necrosis Factor-alpha*

To evaluate the sensitivity and specificity of Community Health Survey (CHS), we analyzed data from 11,217 participants aged > or = 19 yr, in 13 cities and counties in 2008. Three healthcare utilization indices (admission, outpatient visits, dental visits) as comparative variables and the insurance benefit claim data of the Health Insurance Review & Assessment Service as the gold-standard were used. The sensitivities of admission, outpatient visits, and dental visits in CHS were 54.8%, 52.1%, and 61.0%, respectively. The specificities were 96.4%, 85.6%, and 82.7%, respectively. This is the first study to evaluate the validity of nationwide health statistics resulting from questionnaire surveys and shows that CHS needs a lot of efforts to reflect the true health status, health behavior, and healthcare utilization of the population.
Adult ; Aged ; Aged, 80 and over ; Community Health Centers/*utilization ; Female ; *Health Care Surveys ; Humans ; Insurance, Health ; Male ; Middle Aged ; Questionnaires ; Reproducibility of Results ; Republic of Korea ; Self Report ; Sensitivity and Specificity

PURPOSE: The N-myc amplification is one of well known poor prognostic markers in neurblastoma. Because the traditional detection method, Southern blot, is expensive, labor-intensive and time-consuming, the detection of N-myc amplification is not routinely performed in Korea. The purposes of this study are to develop polymerase chain reaction (PCR) for detecting N-myc amplification in neuroblastoma tumor tissue, and to elucidate the clinical significance of N-myc amplification. METHODS: The clinical data and paraffin embedded tumor specimen of 54 neuroblastoma cases were collected from 10 medical centers in Korea. We have developed semiquantitative method of estimating gene copy number that uses differential PCR. N-myc gene primers (RC N-myc, N-myc 7-1) are amplified together with primers from a single-copy internal control gene (beta-globin). After ethidium bromide-stained agarose gel electrophoresis, the ratio of the two PCR products, which stands for N-myc amplification, is determined. Kaplan-Meier survival analysis was performed to evaluate the prognostic significance of N-myc amplification. RESULTS: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The percentage of N-myc amplification was higher in the patients older than 1 year old (34.1%: 14/41), when they were compared to the patients younger than 1 year old (16.7%: 2/12). The percentage of N-myc amplification was higher in the patients who have primary tumor at adrenal gland (40.9%: 9/22) than who have primary tumor at retroperitoneum (17.6%: 3/17) or at mediastinum (16.7%: 2/12). In Stage I, II, and III patients, the mean survival time of N-myc amplified group was 18 months and that of N-myc umamplified group was 64 months (Log Rank 4.35, P=0.037). CONCLUSION: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The N-myc amplification is one of poor prognostic indicators in Neuroblastoma.
Adrenal Glands ; Blotting, Southern ; Electrophoresis, Agar Gel ; Ethidium ; Gene Dosage ; Genes, myc ; Humans ; Korea ; Mediastinum ; Neuroblastoma* ; Paraffin ; Polymerase Chain Reaction* ; Prognosis ; Survival Rate

BACKGROUND: Although the overall survival of childhood acute lymphoblastic leukemia (ALL) approaches 85-90%, the prognosis of relapsed or refractory (R/R) ALL is grave. This study aimed to identify the treatment pattern, treatment response, and overall survival of these patients.METHODS: We reviewed data of 64 patients with R/R ALL whose initial diagnosis of ALL had been made between 1 and 21 years of age. Patients who received clofarabine as part of an induction regimen were excluded. Relapsed patients were limited to those who relapsed after ≥2 prior induction regimens. Treatment patterns, response rates, and overall survival were analyzed.RESULTS: Patients' median age was 15.0 years (range, 6.0-25.0) at the diagnosis of R/R ALL. The most frequently used agents other than steroid were vincristine (54.0%), cytarabine (44.6%), and idarubicin (36.5%), while L-asparaginase was used in only one patient. The complete remission (CR) and overall response (OR) rates were 38.1 and 42.9%, respectively. Sixteen patients (25.4%) underwent allogeneic hematopoietic stem cell transplantation (HSCT). The 5-year overall survival was 6.7%. The survival of patients with HSCT was significantly higher compared with those without HSCT (35.2% vs 0%, P=0.0097). Among 14 patients who achieved CR or CR without platelet recovery (CRp) before HSCT, the 3-year survival was 46.9%.CONCLUSION: The survival of Korean patients with R/R childhood ALL was dismal despite a reasonable CR rate, whereas that of those who received HSCT after CR or CRp was excellent. More treatment options are needed to improve the overall outcome of R/R childhood ALL.
Blood Platelets ; Cytarabine ; Diagnosis ; Hematopoietic Stem Cell Transplantation ; Humans ; Idarubicin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Retrospective Studies ; Vincristine

This study was conducted to analyze the strengths and weaknesses of a 3-week family medicine clerkship program based on the results of an online survey taken by the students (N=127) and a structured interview with a focus group (n=10), aimed to improve the quality of the clerkship program. The online survey contained questions pertaining to goals, schedule, contents, arrangement, atmosphere, environment, evaluation, and satisfaction regarding the clerkship. The focus group interview addressed the schedule and achievements of the program. Scores were reported on a 5-point Likert scale. Most students were highly satisfied with the overall quality of the clerkship. The structured interview results showed that 97.6% of the clerkship program was executed according to the schedule. The focus group reported a perfect score of 5 points on several measures including: accomplishment of the educational goals of the family medicine clerkship, providing many chances to obtain medical histories and perform physical examinations on real patients, experience with various symptoms and diseases, positive attitudes of faculty members when teaching, notification of the guidelines for evaluation beforehand, well-constructed and effective clerkship schedule, and reflection of student feedback. However, the focus group gave low scores on: support for health accidents of students, access to patient information, enough opportunities to practice clinical skills, appropriate rest facilities for students, and fairness of clerkship evaluation process. In conclusion, the structured evaluation performed after the 3-week clerkship program motivated students and helped them ensure an efficient clerkship. This structured evaluation also suggested basic data to make the professor who is subject of the assessment. This study shows that structured assessment is an effective method which can be used to improve the quality of clerkships.
Appointments and Schedules ; Atmosphere ; Clinical Clerkship ; Clinical Competence ; Family Practice ; Focus Groups ; Humans ; Methods ; Physical Examination ; Self-Evaluation Programs ; Surveys and Questionnaires