No abstract available.
Arthritis, Rheumatoid* ; Hand Deformities* ; Hand*

The hyperimmunoglobulin E syndrome is a primary immunodificiency disorder characterized by recurrent staphylococcal infections and markedly elevated serum IgE level. Clinical features are coarse face and severe infections of the skin-furunculosis or chronically pruritic dermatitis and sinopulmonary tract infection from infancy by coagulase positive Staphylococcus aureus, or Candida albicans etc. The patients's serum IgE level is elevated but the basic immunologic pathogenesis not fully understood. We have experienced a case of hyperimmunoglobulin E syndrome in a 26/12-year-old who had suffered from recurrent staphylococcal pneumonias and abscesses and chronically pruritic dermatitis from 1 month of age with elevated serum IgE level. A brief review of the related literature is presented.
Abscess ; Candida albicans ; Coagulase ; Dermatitis ; Immunoglobulin E ; Pneumonia, Staphylococcal ; Staphylococcal Infections ; Staphylococcus aureus

OBJECTIVES: Chronic pain is one of the most common experiences of humans and a typical psychophysiological disorder. The aim of this study was to measure the psychophysiological responses in chronic pain patients using a biofeedback system, and to compare them with the results from normal healthy subjects. METHODS: Forty two patients with chronic pain (17 males and 25 females, average age 44.67+/-11.10 years) and 42 normal healthy controls (17 males and 25 females, average age 45.17+/-10.46 years) participated in this study. Electromyography (EMG), skin conductance (SC), and skin temperature (ST) were recorded using biofeedback system during the 3 phases (baseline, stress, and recovery) of stress reactivity test, and average values of them were calculated. Difference of values between two groups in each corresponding phase was analyzed with independent t-test, and change of values across phases of stress reactivity test was analyzed with paired t-test (all two-tailed, p<0.05). RESULTS: Compared to normal controls, chronic pain patients had higher value of EMG (baseline : 8.10+/-5.97 micronV vs 4.72+/-1.52 micronV, t=-3.56, p<0.01 ; stress : 11.25+/-6.89 micronV vs 8.49+/-4.78 micronV, t=-2.13, p<0.05 ; recovery : 7.12+/-3.77 micronV vs 4.78+/-1.59 micronV, t= -3.70, p<0.01) and SC (baseline : 1.06+/-1.0 micronS vs 0.42+/-0.29 micronS, t=-4.0. p<0.01 ; stress : 1.87+/-2.05 micronS vs 1.03+/-0.86 micronS, t=-2.47, p<0.05 ; recovery : 1.74+/-1.77 micronS vs 0.64+/-0.59 micronS, t=-3.8, p<0.01) in all the 3 phases. But, skin temperature comparison did not reveal significant differences in all the 3 phases between two groups. CONCLUSION: Psychophysiological responses of chronic pain patients in stress reactivity test were different from those of normal healthy controls. These results suggest that sympathetic nervous system is more activated in chronic pain patients.
Biofeedback, Psychology ; Chronic Pain ; Electromyography ; Female ; Humans ; Male ; Psychophysiologic Disorders ; Psychophysiology ; Skin ; Skin Temperature ; Sympathetic Nervous System

PURPOSE: Urinary tract infection (UTI) in children is the most common disease during the infantile period, therefore early diagnosis and treatment are important. Pyuria is a useful clinical parameter for the initial diagnosis of a UTI. In this study we aimed to compare the clinical, laboratory, and imaging findings in relation to the duration of pyuria in infants with UTIs. METHODS: Three hundred seventy-four infants <12 months of age who were admitted between January 1995 and December 2005 for the first episode of a febrile UTI were retrospectively reviewed. Patients were divided into two groups according to the duration of pyuria as follows: group 1, pyuria resolved <3 days after initial treatment; and group 2, pyuria lasted at least 3 days after initial treatment. RESULTS: There were no significant differences between the two groups in relation to gender, age, total duration of fever, and organisms in the urine. Group 2 had a significantly higher peripheral blood leukocyte count (14,360.86+/-5,526.16 cells/mm3 vs. 11,822.55+/-5,687.26 cells/mm3, P<0.001), erythrocyte sedimentation rate (32.81+/-19.34 mm/hr vs. 23.74+/-20.43 mm/hr, P<0.001), and C-reactive protein (6.84+/-5.68 mg/dL vs. 3.78+/-3.99 mg/dL, P<0.001) than group 1. There was a significantly higher incidence of hydronephrosis and a higher grade of vesicoureteral reflux (VUR) in group 2 compared to group 1. CONCLUSION: In infants with UTI, pyuria of longer duration is related to severe UTI and higher grade VUR, therefore aggressive radiologic studies may be necessary.
Blood Sedimentation ; C-Reactive Protein ; Child ; Early Diagnosis ; Fever ; Humans ; Hydronephrosis ; Incidence ; Infant ; Leukocyte Count ; Pyuria ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

Trichilemmal horn(trichilemmal keratosis) is a rare keratinizing tumor that resembles a cutaneous horn. Histologically, it is characterized by an abrupt maturation of keratinocytes into lamellar keratin without the formation of a granular layer. We describe a case of a trichilemmal horn on the right cheek of a 78-year-old woman.
Aged ; Animals ; Cheek ; Female ; Horns* ; Humans ; Keratinocytes

No abstract available.
Thumb*

BACKGROUND: Chronic immune thrombocytopenic purpura (chronic ITP) is the most common autoimmune disease for blood elements. Some patients recovered spontaneously during follow up of disease. We investigated the difference of related clinical parameters between spontaneous remission and no remission.METHODS: We reviewed retrospectively medical records from January 1994 to December 2010. We analyzed clinical parameters such as age, sex, initial platelet count, response to initial treatment, maintenance therapy, and platelet counts at post-diagnosis with regular period in children with or without spontaneous remission.RESULTS: This study was enrolled for 49 children with chronic ITP. Median age was 3.5 years (0.1-17.3). Male to female ratio was 1:1.9. Initial treatment with intravenous immunoglobulin was received in 36 children (73.3%). Spontaneous remission was shown in 23 children (46.9%) during study period with 4.4 year of median follow up. The relevant factors of remission were observed in younger age at diagnosis, maintenance therapy, and platelet counts at post-diagnosis 1 year, 2 years, 3 years (P<0.05). The most significant parameter for spontaneous remission was platelet count at post-diagnosis 6 months in Kaplan-Meier estimate (P=0.047, RR: 3.47, 95% CI: 1.03-24.04).CONCLUSION: Spontaneous remission was shown in about half of patients with chronic ITP. This remission was related with younger age, maintenance therapy, and platelet count at post-diagnosis 6 months. These results suggest that regular follow up with maintenance therapy may be required for management of chronic ITP in children.
Autoimmune Diseases ; Child ; Female ; Follow-Up Studies ; Humans ; Immunoglobulins ; Kaplan-Meier Estimate ; Male ; Medical Records ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; Remission, Spontaneous ; Retrospective Studies

PURPOSE: To report the first case of Waardenburg syndrome type 4 in Korea. CASE SUMMARY: A 3-year-old boy visited our clinic to have his abnormal right eye iris color checked. The patient had a previous operation Hirschsprung's disease. In addition, his older sister and aunt showed similar ocular findings. A general physical examination, hearing test, and fundus examination were performed. On examination, hypochromic heterochromic iridum, albinism of the posterior pole upon ipsilateral fundus, and dystopia canthorum were found. There was no abnormal finding in the hearing test. CONCLUSIONS: The patient showed hypochromic heterochromic iridum, dystopia canthorum, and albinism of ipsilateral fundus. He also had a family history of Waardenburg syndrome and had surgery associated with congenital megacolon. The patient was diagnosed with Waardenburg syndrome type 4.
Albinism ; Child, Preschool ; Hearing Tests ; Hirschsprung Disease ; Humans ; Iris ; Korea ; Male ; Physical Examination ; Siblings ; Waardenburg Syndrome*

PURPOSE: This study aimed at determining the detection rate of respiratory viruses and at investigating the risk factors associated with respiratory virus detection in young infants. METHODS: From September 2011 to August 2012, nasopharyngeal swabs were obtained from 227 infants aged < or =90 days with suspected infectious diseases, including sepsis. We performed a retrospective analysis of their clinical characteristics. The prevalence of respiratory viruses in their nasopharyngeal swabs was assayed by real-time polymerase chain reaction (real-time PCR). RESULTS: In total, 157 (69.2%) infants had more than one of the following respiratory viruses: respiratory syncytial virus (n=75), rhinovirus (n=42), influenza virus (n=18), parainfluenza virus (n=15), human metapneumovirus (n=9), coronavirus (n=9), adenovirus (n=4), and bocavirus (n=3). During the same period, bacterial infections were confirmed in 24 infants (10.6%). The detection of respiratory viruses was significantly associated with the presence of cough, a family history of respiratory illness, and a seasonal preference (fall/winter). Using logistic regression analysis, these 3 variables were also identified as significant risk factors. During fall and winter, detection of respiratory viruses was significantly higher in infants who did not have a bacterial infection. CONCLUSION: Respiratory virus is an important pathogen in young infants admitted to a hospital, who are suspected with infectious diseases. Detection of respiratory viruses in young infants was associated with seasonality (fall/winter), presence of respiratory symptoms and a family history of respiratory illness.
Adenoviridae ; Bacterial Infections ; Bocavirus ; Communicable Diseases ; Coronavirus ; Cough ; Humans ; Infant* ; Logistic Models ; Metapneumovirus ; Nasopharynx ; Orthomyxoviridae ; Paramyxoviridae Infections ; Prevalence ; Real-Time Polymerase Chain Reaction ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Retrospective Studies ; Rhinovirus ; Risk Factors* ; Seasons ; Sepsis ; Virus Diseases

No abstract available.
Blister* ; Fibroma*