Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.
Blister ; Dermatitis, Exfoliative ; Epidermis ; Humans ; Ichthyosis Bullosa of Siemens* ; Ichthyosis* ; Intermediate Filaments ; Molting ; Skin ; Wills

No abstract available.
Depression*

We clescribe a patient who developed an annular patch with concentrically arranged rings of scales rescmbling tinea imbricata on his right forearm, which was ultiimately diagnosed as tinea corporis caused by Microsporum ferrugineum. Topical antifungal therapies alone were unsuccessful. The combination therapies of topical and systemic antifungal agents were subsequently performed and a good result was obtained.
Antifungal Agents ; Forearm ; Humans ; Microsporum* ; Tinea* ; Weights and Measures

We report cases of two patients with acute generalized exanthematous pustulosis(AGEP). One patient had localized cutaneous infection and the other rhinoplasty. Both were being treated with ampicillin and developed intense erythemas followed by generalized subcorneal pustulation associated with fever and a neutrophilic leukocytosis. Histopathological findings were subcorneal spongiform pustules showing preponderance of polymorphonuclear leukocytes. Generalized pustular psoriasis, subcorneal pustular dermatosis, impetigo and pemphigus foliaceus should be differentiated from AGEP. The causative drug in both of our cases was ampicillin and fast resolution of pustules was observed with a low dosage of systemic steroid within 5 days.
Acute Generalized Exanthematous Pustulosis* ; Ampicillin* ; Erythema ; Fever ; Humans ; Impetigo ; Leukocytosis ; Neutrophils ; Pemphigus ; Psoriasis ; Rhinoplasty ; Skin Diseases, Vesiculobullous

PURPOSE: This study was performed to compare the efficacy and adverse reactions of low dose intravenous immunoglobulin (IVIG) with those of high dose IVIG in childhood acute idiopathic thrombocytopenic purpura (ITP). METHODS: Thirty children who were diagnosed as acute ITP in Seoul National University Children's Hospital from June 2000 to Jan. 2003 were enrolled. IVIG (I.V Globulin S, Green Cross Ltd, Korea) were administered 1 g/kg in low dose group (n=15) and 2 g/kg (400 mg/kg for 5 day or 1 g/kg for 2 days) in high dose group (n=15). RESULTS: 1) The median age of the patients was 4 years (range, 2 months to 13 years) and male to female ratio was 0.5: 1. The median duration of follow-up was 6 months (range, 1 month to 2 year 9 months). 2) The median days to recover the platelet count over 50, 000/muL, 100, 000/muL, 150, 000/muL after IVIG administration were 4, 6, and 7 days in the low dose group and 3, 5, and 5 days in the high dose group. After the initial response, the platelet count decreased below 50, 000/muL in 5 out of 13 patients in the low dose group (the median day, 13) and 6 out of 13 patients in the high dose group (the median day, 21). In 25 patients who were followed over 6 months, 3 out of 13 patients (23.1%) in the low dose group and 4 out of 12 patients (33.3%) in the high dose group were remained as chronic ITP. 3) Adverse reactions related to IVIG administration were observed in 4 out of 15 patients (26.7%) in the low dose group and 5 out of 15 patients (33.3%) in the high dose group. They were fever, headache, nausea and vomiting, and most of them were subsided spontaneously. CONCLUSION: There were no significant difference in the response rate, the relapse rate and the incidence of adverse reactions between the low dose group and the high dose group in childhood acute ITP after IVIG therapy. In respect of the cost and the duration of hospitalization, the low dose IVIG might have some advantages.
Child ; Female ; Fever ; Follow-Up Studies ; Headache ; Hospitalization ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Incidence ; Male ; Nausea ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; Recurrence ; Seoul ; Vomiting

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

PURPOSE: This study was performed to identify the etiologic agents and antimicrobial susceptibility patterns of organisms responsible for bloodstream infections in pediatric cancer patients for guidance in empiric antimicrobial therapy. METHODS: A 5-year retrospective study of pediatric hemato-oncologic patients with bacteremia in Seoul National University Children's Hospital, from 2006 to 2010 was conducted. RESULTS: A total of 246 pathogens were isolated, of which 63.4% (n=156) were gram-negative, bacteria 34.6% (n=85) were gram-positive bacteria, and 2.0% (n=5) were fungi. The most common pathogens were Klebsiella spp. (n=61, 24.8%) followed by Escherichia coli (n=31, 12.6%), coagulase-negative staphylococci (n=23, 9.3%), and Staphylococcus aureus (n=22, 8.9%). Resistance rates of gram-positive bacteria to penicillin, oxacillin, and vancomycin were 85.7%, 65.9%, and 9.5%, respectively. Resistance rates of gram-negative bacteria to cefotaxime, piperacillin/tazobactam, imipenem, gentamicin, and amikacin were 37.2%, 17.1%, 6.2%, 32.2%, and 13.7%, respectively. Overall fatality rate was 12.7%. Gram-negative bacteremia was more often associated with shock (48.4% vs. 11.9%, P<0.01) and had higher fatality rate than gram-positive bacteremia (12.1% vs. 3.0%, P=0.03). Neutropenic patients were more often associated with shock than non-neutropenic patients (39.6% vs. 22.0%, P=0.04). CONCLUSION: This study revealed that gram-negative bacteria were still dominant organisms of bloodstream infections in children with hemato-oncologic diseases, and patients with gram-negative bacteremia showed fatal course more frequently than those with gram-positive bacteremia.
Amikacin ; Bacteremia ; Bacteria ; Cefotaxime ; Child ; Escherichia coli ; Fever ; Fungi ; Gentamicins ; Gram-Negative Bacteria ; Gram-Positive Bacteria ; Humans ; Imipenem ; Klebsiella ; Neutropenia ; Oxacillin ; Penicillins ; Retrospective Studies ; Shock ; Staphylococcus aureus ; Vancomycin

A 5-year-old girl was diagnosed with neurofibromatosis type 2 (NF-2) due to multiple neurofibromas, cafe-au-lait spots, and schwannomas of the brain. During ophthalmologic evaluation, a posterior subcapsular cataract and a gray-green colored subretinal lesion were found in right eye. Fluorescein angiography (FA) revealed a combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). At age 9, she underwent cataract surgery. At this time FA and spectral-domain optical coherence tomography (SD-OCT) were taken. The SD-OCT showed an elevated hyperreflective mass in the retina with prominent attenuation of the inner and outer retina, but minimal attenuation in the photoreceptor layers. The underlying retina appeared to be disorganized and thick (791 microm). This is the first case report of SD-OCT imaging of a CHRRPE associated with NF-2 in a pediatric patient. By using SD-OCT in this patient, we could obtain detailed tumor characteristics, and SD-OCT may be helpful in the diagnosis and management of CHRRPE.
Child, Preschool ; Diagnosis, Differential ; Female ; Hamartoma/complications/*diagnosis ; Humans ; Neurofibromatosis 2/*complications/diagnosis ; Retinal Diseases/complications/*diagnosis ; Retinal Pigment Epithelium/*pathology ; Tomography, Optical Coherence/*methods ; Visual Acuity

Chronic dermatophyte infection rarely fails to respond to topical or systemic antifungal therapy. Such refractory condition relates to many factors and one of them is the decreased response of delayed type hypersensitivity. A plausible mechanism by which the delayed hypersensitivity response may cause dermatophyte inhibition has been proposed already. Our patient had skin rashes for 6 years. It was diagnosed as tinea corporis and treated with various systemic antifungal agents, such as griseofulvin, itraconazole, fluconazole, terbinafine and topical forms of econazole and terbinafine. But the skin lesions did not resolve completely and reaggravated frequently. Numerous verrucae planar were found on face, neck and both extremities. Trichophyton rubrum was identified by fungus culture study. Laboratory examination showed no response against multi-CMI test, DPCP sensitization and prick test for trichophytons. We challenged the therapy with the combined antifungal agents and immune stimulatory drugs. This case is thought to be a chronic dermatophyte infection due to the defects in the both cell mediated immunity and immediate type hypersensitivity which is crucial for the host defence mechanisms against fungal infection.
Antifungal Agents* ; Arthrodermataceae* ; Econazole ; Exanthema ; Extremities ; Fluconazole ; Fungi ; Griseofulvin ; Humans ; Hypersensitivity ; Hypersensitivity, Delayed ; Immunity, Cellular ; Itraconazole ; Neck ; Skin ; Tinea ; Trichophyton ; Warts

Congenital complete atrioventicular block is uncommon which has a heterogenous etiology. According to recent studies, about one-third of the mothers of the patients who were diagnosed as congenital complete atrioventricular block, had symptoms or signs of connective tissue disease. Even though the outlook is usually regarded as favorable, the mortality is highest in the neonatal period, much lower during childhood and adolescence and increases slowly later in life. In the neonatal period the predominant indication for pacing was heart failure. It is difficult to make a prognosis in the individual patient. A slow, fixed or decreasing venticular rate neonatally and a prolonged QT interval seem to be bad prognostic sign. We report a case of congenital complete atrioventricular block who was diagnosed by fetal echocardiography. Her mother was asymptomatic, but had positive serologic test to anti-Ro antibody and anti-La antibody. We report a case of congenital complete atrioventricular block with a brief review of related literatures.
Adolescent ; Atrioventricular Block* ; Connective Tissue Diseases ; Echocardiography* ; Heart Failure ; Humans ; Mortality ; Mothers ; Prognosis ; Serologic Tests