We conducted this cohort analytic study to determine whether women with unexplained elevations of maternal serum hCG at 15-18 weeks' gestation are at increased risk for pregnancy complications and adverse perinatal outcomes. The inclusion criteria were a singleton gestation, a confirmed gestational age, and an hCG level greater than 2.0 multiples of the median (MoM). The exclusion criteria were fetal anomalies, an abnormal karyotype, molar pregnancy, and an MSAFP level greater than 2.5 multiples of the median (MoM). A group of randomly selected women with hCG levels under 2.0 MoM served as controls. Patients with elevated levels of hCG had a significantly higher risk for PIH (17.9% versus 4.5%; P <.05) and preterm delivery (17.9% versus 3.5%; P<, 05) than control. But no significant differences were observed in the incidence of intrauterine growth restriction and low birth weight and in the newborn weight. We suggested that pregnancies with unexplained elevated hCG levels should be regarded as high-risk pregnancies. And these patients require careful monitoring with adequate obstetric management.
Abnormal Karyotype ; Chorionic Gonadotropin* ; Cohort Studies ; Female ; Gestational Age ; Humans* ; Hydatidiform Mole ; Incidence ; Infant, Low Birth Weight ; Infant, Newborn ; Pregnancy ; Pregnancy Complications ; Pregnancy Outcome* ; Pregnancy* ; Pregnancy, High-Risk

Psychosocial problems increase the risk for mental health problems and increase the need for health care services in children and adolescents. Primary care practice is a valuable avenue for identifying the need for more specialized mental health care. We hypothesized that Korean version of the pediatric symptom checklist (PSC) would be a useful tool for early detection of psychosocial problems in children and adolescents in Korea and we aimed to suggest cut-off scores for detecting meaningful psychosocial problems. A total of 397 children with their parents and 97 child patients with their parents were asked to complete the PSC Korean version and the child behavior checklist (CBCL). The internal reliability and test-retest reliability of the PSC as well as the cut-off score of the PSC was determined via receiver operating characteristic analysis of the CBCL score, clinical group scores and non-clinical group scores. The internal consistency of the PSC-Korean version was excellent (Cronbach's alpha = 0.95). The test-retest reliability was r = 0.73 (P < 0.001). Using clinical CBCL scores (total score, externalizing score, internalizing score, respectively > or = 60) and presence of clinical diagnosis, the recommended cut-off score of the PSC was 14. Using 494 Korean children aged 7-12 yr, the current study assessed the reliability and validity of a Korean version of the PSC and suggested a cut-off for recommending further clinical assessment. The present results suggest that the Korean version of the PSC has good internal consistency and validity using the standard of CBCL scores.
Checklist/methods/*standards ; Child ; Child Behavior Disorders/*diagnosis/*psychology ; Female ; Humans ; Male ; Mass Screening/standards ; Pediatrics/*standards ; Population ; Psychometrics/methods/*standards ; Reproducibility of Results ; Republic of Korea ; Sensitivity and Specificity ; Surveys and Questionnaires/standards ; Symptom Assessment/methods/*standards ; Translating ; United States

Actinomycosis is a rare entity which presents some difficulties in establishing a correct preoperative diagnosis. Many actinomycotic pelvic infections in women are related to IUD use and the colonization rate appears to increase in accordance with the duration of IUD use. So, all women in IUD use are recommended to make cervicovaginal smear and pelvic infection associated with IUD use should be suspected to have actinomycoses. We report a case which presented painful mass on right upper and lower quadrant of abdomen of a 40-year-old women. We identified sulfur granules by histopathologic exam of surgically resected specimen. Eventually it proved to be pelvic and abdominal actinomycosis associated with the use of an intrauterine device. Because variable clinical pictures and infrequency of the disease make the diagnosis more difficult, increased alertness of clinicians and microbiologists to the presence of anaerobic organism as the cause of infection are needed to make an earlier and more correct diagnosis of actinomycoses and to further avoid any inappropriate treatment.
Abdomen ; Actinomycosis ; Adult ; Colon ; Diagnosis ; Female ; Humans ; Intrauterine Devices ; Pelvic Infection ; Pelvis ; Sulfur

OBJECTIVE: The purpose of this study was to analyze the clinical study for patients with cervical cancer who had undergone radical hysterectomy. METHOD: The subjects of this study were one hundred and sixty two patients with cervical cancer who had undergone radical hysterectomy at Eulji Medical College Hospital, Taejon, Korea, from January 1983 to December 1992. We reviewed the medical record retrospectively and analyzed the data. RESULT: The distribution of patients by age was found in the order of 50 decade and 60 decade, 40 decade. Those by the clinical stages were as follows: Stage Ia, 12 cases(7.4%); Stage Ib, 84 cases(51.9%); Stage IIa, 39 cases(24.1%); Stage IIb 27 cases(16.7%). The results of histopathologic type were distributed as follows: squamous cell carcinoma was 91.9%, adenocarcinoma was 4.9% and adenosquamous cell carcinoma was 3.1%. The histologic subtypes of squamous cell carcinoma(149 cases) were as follows: Large cell non-keratinizing type was 75.9%, large cell keratinizing type was 14.8% and small cell type was 1.2%. The frequancy of lymph node metastasis was 22.9% in stage I and 31.8% in stage II. The overall incidence of lymph node metastasis was 26.4%. The frequency of external radiation therapy done after radical hysterectomy was 63.5% in stage I and 75.8% in stage II. The 5-year survival rate was as follows: The Ia was 100%; Stage Ib, 95.2%(4cases); Stage IIa, 87.2%(5cases); Stage IIb, 77.8%(6cases). The incidence of recurrence was 7.4% and recurrent sites were vaginal stump , rectum and pelvic wall. CONCLUSION: The highest incidence of cervix cancer in age distribution was 50 decade(30.9%) and 60 decade(30.9%). The most common clinical stage was Ib(51.9%) and most frequent pathologic type was squamous cell carcinoma(91.9%). The overall incidence of lymph node metastasis was 26.4% and The most common site of recurrence was vaginal stump. The 5-year survival rate was 100% in the stage Ia, 95.2% in the stage Ib, 87.2% in the stage IIa, 77.8% in the stage IIb.
Adenocarcinoma ; Age Distribution ; Carcinoma, Squamous Cell ; Daejeon ; Humans ; Hysterectomy* ; Incidence ; Korea ; Lymph Nodes ; Medical Records ; Neoplasm Metastasis ; Rectum ; Recurrence ; Retrospective Studies ; Survival Rate ; Uterine Cervical Neoplasms*

Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective-tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four-month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma, a sagging face, redundant and hypopigmented skin, and abnormal hair growth. In addition, he had unexplained hepatomegaly and high hepatic transaminase. We confirmed the diagnosis of Menkes disease by mutation analysis of the ATP7A gene. To exclude other possible causes for the hepatic abnormalities, a liver biopsy was performed, revealing intracytoplasmic cholestasis, focal spotty necrosis, and minimal lobular activity. The patient's liver involvement may be an underestimated complication of Menkes disease.
Biopsy ; Cholestasis ; Copper ; Hair ; Hepatomegaly ; Infant ; Infant, Newborn ; Liver ; Menkes Kinky Hair Syndrome ; Muscle Hypotonia ; Necrosis ; Seizures ; Skin ; Spasms, Infantile

BACKGROUND: Ductal adenocarcinoma (DAC) of the prostate is an uncommon histologic subtype whose prognostic factors and immunoprofile have not been fully defined. METHODS: To define its prognostic factors and immunoprofile, the clinicopathological features, including biochemical recurrence (BCR), of 61 cases of DAC were analyzed. Immunohistochemistry was performed on tissue microarray constructs to assess the expression of prostate cancer-related and mammalian target of rapamycin (mTOR) signaling-related proteins. RESULTS: During the median follow-up period of 19.3 months, BCR occurred in 26 cases (42.6%). DAC demonstrated a wide expression range of prostate cancer-related proteins, including nine cases (14.8%) that were totally negative for pan-cytokeratin (PanCK) immunostaining. The mTOR signaling-related proteins also showed diverse expression. On univariate analysis, BCR was associated with high preoperative serum levels of prostate-specific antigen (PSA), large tumor volume, predominant ductal component, high Gleason score (GS), comedo-necrosis, high tumor stage (pT), lymphovascular invasion, and positive surgical margin. High expressions of phospho-mTOR (p-mTOR) as well as low expressions of PSA, phospho-S6 ribosomal protein (pS6) and PanCK were associated with BCR. On multivariable analysis, GS, pT, and immunohistochemical expressions of PanCK and p-mTOR remained independent prognostic factors for BCR. CONCLUSIONS: These results suggest GS, pT, and immunohistochemical expressions of PanCK and p-mTOR as independent prognostic factors for BCR in DAC. Since DAC showed diverse expression of prostate cancer–related proteins, this should be recognized in interpreting the immunoprofile of DAC. The diverse expression of mTOR-related proteins implicates their potential utility as predictive markers for mTOR targeted therapy.
Adenocarcinoma* ; Carcinoma, Ductal ; Follow-Up Studies ; Immunohistochemistry ; Neoplasm Grading ; Prognosis ; Prostate* ; Prostate-Specific Antigen ; Prostatic Neoplasms ; Recurrence ; Ribosomal Proteins ; Sirolimus ; Tumor Burden

PURPOSE: Pompe disease is one of the glycogen storage diseases caused by a deficiency of acid alpha-glycosidase. This enzyme defect results in lysosomal glycogen accumulation in many tissues and shows a various spectrum of clinical features from early infantile hypotonia to mild weakness. For the investigation of the clinical characteristics of Pompe disease, we reviewed 6 cases of childhood Pompe disease diagnosed by muscle biopsy and acid alpha-glycosidase assay. METHODS: We reviewed the medical records of 6 childhood Pompe disease patients in Seoul National University Children's Hospital, retrospectively from January 2001 to October 2006. RESULTS: The age of the symptom onset was 1 month to 11 years(mean 2.2 years) and the diagnosis was made at the age of 8 months to 14 years(mean 5.3 years). The patients showed delayed motor development, motor weakness, hypotonia, cardiomegaly, hypertrophic cardiomyopathy, hepatomegaly, recurrent pulmonary infections but the severity was very diverse. Liver transaminase and CK levels were elevated in all of the patients. Their muscle biopsy showed the characteristic accumulation of purple colored glycogen granules and the degeneration of myofibers. CONCLUSION: Childhood Pompe disease had various clinical features and severities depending on the age of onset, organ involvement and the rate of progression. Enzyme replacement therapy may modify the disease courses, so we need to diagnose earlier for the treatment at an optimal time.
Age of Onset ; Biopsy ; Cardiomegaly ; Cardiomyopathy, Hypertrophic ; Diagnosis ; Early Diagnosis ; Enzyme Replacement Therapy ; Glycogen ; Glycogen Storage Disease ; Glycogen Storage Disease Type II* ; Hepatomegaly ; Humans ; Liver ; Medical Records ; Muscle Hypotonia ; Retrospective Studies ; Seoul

PURPOSE:Temporal lobe epilepsy(TLE) is now recognized as a distinct syndrome in adults. The seizure evolution in adult patients is well characterized, manifesting initially with an aura, behavioral arrest, automatism, and secondary generalized tonic-clonic seizures. In contrast, relatively few studies are available for the pediatric age group. In the present study, we investigated children undergoing temporal lobectomy for refractory seizures and correlated the pathologic findings with clinical presentations. METHODS:The records of the pediatric patients admitted at the Seoul National Children's Hospital for epilepsy surgery between January 1995 and December 2005 were reviewed. Then, eighteen patients were included in this study. The clinical records were reviewed in terms of the patient profiles imaging findings, surgical techniques, and pathologic findings. The seizure outcomes were described according to the Engel's classification. RESULTS:The postsurgical outcomes were favorable. Lateral temporal epilepsy was more common in childhood than in adulthood. Dual pathology was commonly found. Arm dystonia or tonic arm elevation have a lateralizing value. Head turning may have a lateralizing value based upon a time sequence. The brain MRI was less predictable for pathologic findings. The ictal EEG cannot always have a localizing value. Delta beginning in the ictal rhythm may suggest lateral lobe epilepsy. Anterior temporal beginning of the ictal location may suggest mesial temporal lobe epilepsy. Ganglioglioma tends to cause rhythmic beta activities at the beginning of the ictal event. CONCLUSION:TLE in childhood shows more complex and atypical clinical manifestations and have more variable etiologies. No single presurgical investigation can be a good predictable value to localization or lateralization.
Adult ; Arm ; Automatism ; Brain ; Child ; Classification ; Dystonia ; Electroencephalography ; Epilepsy ; Epilepsy, Temporal Lobe* ; Ganglioglioma ; Head ; Humans ; Magnetic Resonance Imaging ; Pathology ; Seizures ; Seoul ; Temporal Lobe*

OBJECTIVES: Children with attention-deficit hyperactivity disorder (ADHD) have problems in social interactions. We compared the effect of 10-session social skill training (SST) among two groups, children with pure ADHD, and those with ADHD with comorbidity. METHODS: Consecutive 10-session SST was conducted for 34 children from 2006 to 2012. There were 22 children with pure ADHD (male 20, female 2), and 12 children suffering from ADHD with comorbidity (male 11, female 1). All children took medication as prescribed by their doctors before the start of SST. The Child Behavior Checklist (CBCL), the Korean Personality Inventory for Children (K-PIC), the Conner's Rating Scale, the ADHD Rating Scale, and the Home Situation Questionnaire were completed by mothers before and after the SST. All children completed the Child Depression Inventory, the Stat-Trait Anxiety Inventory for Children, the Self-Concept Scale and the ADHD Diagnostic System before and after the SST. RESULTS: Only children with pure ADHD showed improvement in anxiety and self-concept in scales rated by children. In the CBCL rated by parents, the pure ADHD group and the ADHD with comorbidity showed improvement in both externalizing and internalizing subscales. In the K-PIC rated by parents, the pure ADHD group showed improvement in most outcomes and ADHD with comorbidity showed positive change in verbal development. CONCLUSION: These results suggest that SST has significant positive effects on both the pure ADHD and ADHD with comorbidity group. Further research is needed in order to target diverse comorbidity groups with ADHD to improve the effectiveness of the SST.
Anxiety ; Checklist ; Child Behavior ; Child* ; Comorbidity* ; Depression ; Female ; Humans ; Interpersonal Relations ; Mothers ; Only Child ; Parents ; Personality Inventory ; Weights and Measures

PURPOSE: Food protein-induced enterocolitis syndrome (FPIES) is a symptom complex of vomiting and diarrhea caused by non-IgE mediated allergy to cow's milk and/or soy in young infants. Transforming growth factor (TGF)-beta has been reported to protect the epithelial barrier of the gut from foreign antigens. We studied the expression of type 1 and 2 TGF-beta receptors in the mucosa of small intestine to investigate their roles in the pathogenesis of FPIES. METHODS: Twenty-eight patients, aged 7 to 120 days (mean 49 days) who were diagnosed with FPIES by clinical criteria and challenge tests were included. Immunohistochemical stainings for type 1 and 2 TGF-beta receptors were performed on endoscopic duodenal biopsy specimens. RESULTS: Type 1 and 2 TGF-beta receptors were expressed in the villous and crypt epithelial cells but nearly absent in the lamina propria in both patients and controls. Type 1 TGF-beta receptor expression was significantly lower in the patients who had villous atrophy than in the patients who had not and in controls. The expression of type 1 TGF-beta receptor was negatively correlated with the severity of villous atrophy. Type 2 TGF-beta receptor expression showed no significant difference between the patients and controls. CONCLUSION: Our results suggests that the decreased activity of type 1 TGF-beta receptor is implicated in the pathogenesis of FPIES in young infants.
Atrophy ; Biopsy ; Diarrhea ; Enterocolitis* ; Epithelial Cells ; Humans ; Hypersensitivity ; Infant ; Intestine, Small ; Milk ; Mucous Membrane ; Receptors, Transforming Growth Factor beta ; Transforming Growth Factors ; Vomiting