PURPOSE: Henoch-Schonlein purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. METHODS: 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) for 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) for 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. RESULTS: Complete remission rate of nephrotic syndrome was 5S.8% in CsA, 57.1% in RFP and 38.4% in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was 88.2% in CsA, 85.7% in RFP and 84.6% in AZA group. Disappearance rate of hematuria was 58.8% in CsA, 57.1% in RFP and 46.2% in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA(29.4%), none RFP(0%) and 2 AZA group(12.4%). Improvement on histologic immune-deposition was seen in 15 CsA(88.2%), 6 RFP(85.9%) and 4 AZA group(30.8%). CONCLUSION: In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant inprovement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and RFP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.
Adrenal Cortex Hormones ; Biopsy ; Biopsy, Fine-Needle* ; Breast* ; Child ; Cyclosporine ; Diagnosis* ; Follow-Up Studies ; Hematuria ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation ; Nephrotic Syndrome ; Prognosis ; Purpura, Schoenlein-Henoch

No abstract available.
Carpal Tunnel Syndrome*

No abstract available.
Animals ; B-Lymphocytes* ; Mice* ; Sarcoma*

No abstract available.
Pierre Robin Syndrome*

Pheochromocytoma is an unusual tumor in pediatric age group and there are several different aspects from adult counterparts. Children have fewer malignant, more extra-adrenal, and greater bilaterality and multiplicity of tumor. We present a case of 14-year old boy with pheochromocytoma who has symptoms such as episodic headaches, vomiting, seizure and paroxysmal hypertension which is less common in children. Although the duration of preoperative preparation was not long enough, we decided to remove the tumor because symptoms were disappeared rather rapidly after alpha and beta adrenergic blocker treatment. The patient was managed with continuous epidural block and light general anesthesia but extra use of adrenergic receptor blocker and vasodilator were demanded during tumor manipulation. The patient has remained well postoperatively but long-term follow up is essential because of the possibilities of recurrence.
Adolescent ; Adrenergic Antagonists ; Adult ; Anesthesia, General ; Child ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Male ; Pheochromocytoma* ; Receptors, Adrenergic ; Recurrence ; Seizures ; Vomiting

PURPOSE: This study was done to examine the relationship between sexual permissiveness, family function and parent-adolescent communication among adolescents. METHOD: A descriptive correlation approach was used and the participants were 956 male and female students. Data were collected by questionnaire survey using convenience sampling. The instruments were the Family APGAR Questionnaire by Smilkstein(1978), PAC by Olson and Barnes(1982), and the Premarital Sexual Permissiveness Questionnaire by Reiss(1967). Data were analyzed using the SPSS 10.0 program with descriptive statistics, Pearson correlation coefficients, t-test, and ANOVA. RESULTS: Family function was significant according to school record, economic status, living arrangement of parent. Parent-Adolescent Communication was significant according to religion, economic status, having a boy/girl friend, and experience of sexual intercourse. Sexual permissiveness was significant for all except economic status. Correlation among the variables showed that parent-adolescent communication was negatively correlated to sexual permissiveness(r=-.127, p=.000) and positively to family function(r=.368, p=.000). CONCLUSION: The findings suggest that parent-adolescent communication and family function are associated with sexual permissiveness, and the findings of this study are expected to make a contribution to creating an ideal sexual culture for youth.
Adolescent* ; Coitus ; Female ; Friends ; Humans ; Male ; Parents ; Permissiveness* ; Residence Characteristics ; Child Health ; Surveys and Questionnaires

We described a case of a 30-year-old female patient with bipolar disorder who experienced the anticonvulsant hypersensitivity syndrome (AHS) during treatment with lamotrigine and aripiprazole. She developed fever (38.4 degrees C), leukopenia, skin rash, and elevated serum transaminase levels on the 11th day of lamotrigine treatment (20th day of aripiprazole). Hypersensitivity to lamotrigine was suspected; lamotrigine was discontinued and prednisolone (30 mg/day) was administered to the patient. The clinical manifestations and laboratory findings showed improvement. However, on the 11th day of lamotrigine discontinuation (7th day of prednisolone treatment), she developed maculopapular skin rash over the entire body except the mucosa. There were no other symptoms and the laboratory findings were within normal limits. Skin biopsy showed erythema multiforme. After prescribing 55 mg/day of predisolone for additional 8 days, the recovery was uneventful, and it took 4 weeks from the onset of the second skin rash. Lamotrigine induced AHS showed broad spectrum of presentation and some manifestations can be flared up several days after discontinuation as did in this case. If unexplained systemic symptoms or a skin rash of unknown cause develop during the use of lamotrigine, clinicians should discontinue lamotrigine promptly and monitor the patient carefully at least for several weeks.
Adult ; Biopsy ; Bipolar Disorder ; Erythema Multiforme ; Exanthema ; Female ; Fever ; Humans ; Hypersensitivity* ; Leukopenia ; Mucous Membrane ; Prednisolone ; Skin ; Aripiprazole

A wide range of denomination has been used for inflammatory myofibroblastic tumor (IMT). IMT is not entirely homogeneous, even though it shows some overlapping histologic features such as haphazard proliferation of spindle cell and polymorphic chronic inflammatory cell infiltraion. The spindle cell is considered to be of myofibroblastic origin but follicular dendritic cell origin was reported recently. IMT is known as nonneoplastic, aberrant inflammatory response. However, IMT could show local invasion, recurrence, vascular invasion, and malignant transformation, and clonal characteristics and aneuploidy of IMT support the hypothesis that IMT may be a neoplastic process. In order to define the nature of spindle cell of IMT, immunohistochemical stains for smooth muscle actin (SMA), vimentin (VMT), lysozyme, S-100 protein, cytokeratin, CD21 were done. Additional immunohistochemical stains for MIB-1 for proliferating activity and LMP (latent membrane protein) for Epstein-Barr virus (EBV) were done. IMTs were composed of each 2 cases from lung, liver and lymph node and one case from common bile duct, maxillary sinus, bladder and thigh, and were histologically subclassified according to Coffin et al. Nine cases (90%) were positive for SMA and VMT, but no correlation between SMA and VMT immunoreactivity and histologic types was identified. Five cases (50%) were positive for lysozyme and S-100 protein, and histologic type III was negative for lysozyme and S-100 protein, and immunoreactivity for S-100 protein was different according to the histologic subtypes. All 11 cases were negative for CD21 and EBV LMP. MIB-1 labelling index was less than 1% in all cases. In summary, the spindle cell is regarded as myofibroblastic origin rather than follicular dendritic cell origin. Relationship with EBV is not clear, and negligible MIB-1 reaction suggests that IMT might have a good prognosis.
Actins ; Aneuploidy ; Coloring Agents ; Common Bile Duct ; Dendritic Cells, Follicular ; Herpesvirus 4, Human ; Immunohistochemistry ; Keratins ; Liver ; Lung ; Lymph Nodes ; Maxillary Sinus ; Membranes ; Muramidase ; Muscle, Smooth ; Myofibroblasts* ; Prognosis ; Recurrence ; S100 Proteins ; Thigh ; Urinary Bladder ; Vimentin

Malignant small cell tumor(MSCT) of the thoracopulmonary region(Askin tumor) is extremely rare and is seen predominantly in children and adolescents. This tumor represents a distinct clinicopathologic entity of neuroectodertnal origin, arising from the soft tissues of the chest wall or peripheral lung. This tumor tends to recur locally, but does not seem to disseminate widely. The overall survival is poor. Recently, we experienced a case of the MSCT of the thoracopulmonary region of a 12-year-old female. She was admitted because of a chest wall mass on radiographic examination, and a complaint of intermittent chest pain. Grossly, the mass was lobulated, round and had a solid appearance with focal necrosis and hemorrhage on the cut surface. Histologically, small round to oval cells were arranged in compact sheets, nests and lobular patterns with intervening fibrovascular stroma. Ultrastructurally, the presence of loose-fitting membrane-bound neurosecretory granules was noted.
Child ; Adolescent ; Male ; Female ; Humans

Staphylococcus epidermidis is a normal inhabitant of skin, throat, mouth, vagina, and urethra. It is not usually pathogenic, particularly in immunocompetent hosts. This report describes a case of a pyogenic liver abscess caused by Staphylococcus epidermidis in a healthy 12-year-old boy. He was admitted to Seoul St. Mary's Hospital with intermittent fever for 6 days. Findings on abdominal computed tomography (CT) showed a mass measuring 7.0x6.5 cm in the right hepatic lobe. Culture of the abscess resulted in growth of Staphylococcus epidermidis as a causative organism. The patient was successfully treated with intravenous administration of antibiotics and percutaneous drainage of the abscess.
Abscess ; Administration, Intravenous ; Anti-Bacterial Agents ; Child ; Drainage ; Fever ; Humans ; Liver Abscess ; Liver Abscess, Pyogenic ; Mouth ; Pharynx ; Skin ; Staphylococcus ; Staphylococcus epidermidis ; Urethra ; Vagina