Familial hyperkalemic periodic paralysis is an autosomal-dominant channelopathy characterized by reversible paralysis associated with episodic hyperkalemia. Mutations in the skeletal muscle voltage-gated sodium channel gene (SCN4A) have been reported to be responsible for this disorder. Paramyotonia congenita is also caused by mutations in the SCN4A gene. Here, we report the case of a 17-year-old boy who presented with both hyperkalemic periodic paralysis and paramyotonia congenita. A molecular analysis of the SCN4A gene revealed a heterozygous T>C transition at nucleotide 2078, leading to an Ile693Thr mutation. This mutation was absent in the patient’s parents supporting a de novo Ile693Thr mutation in our patient.

Intravenous pyogenic granuloma (IVPG) is a form of pyogenic granuloma (PG) in which the capillary proliferation is confined entirely within the lumen of a vein. It occurs typically in the neck and upper extremities. Histologically, this benign lesion is similar to PG of other localizations and is characterized by a lobular proliferation of capillaries growing in a sparsely cellular, edematous collagenous stroma. We report a case of IVPG developing in the palm, an unusual site of occurrence.
Capillaries ; Collagen ; Granuloma, Pyogenic* ; Neck ; Upper Extremity ; Veins

In the present study, in order to improve elementary students' nutrition knowledge and form correct eating habits, dietitian in charge executed nutrition education for 4th-, 5th- and 6th-grade students at Namchang Elementary School in Suwon, Gyeonggi-do using various educational media for five weeks and two sessions a week (a total of 10 sessions) through discretional activity classes. As for change in nutritional knowledge after nutrition education, 4th-grade students showed improvement by 24.3points(p<0.001), 5th-grade ones by 18.0(p<0.001), and 6th-grade ones by 16.7(p<0.001). With regard to change in dietary habits after education, no effect was observed in the improvement of dietary life but the score of dietary habits was improved as a whole. Nutritional knowledge and dietary habits were in a positive correlation with each other before education(r=0.406, p<0.001), but in no correlation after education. Nutritional knowledge and dietary habit practice plan were in a positive correlation after education(r=0.310, p<0.01). With regard to nutritional knowledge after nutrition education by children's body type measured using Rohrer Index, knowledge increased significantly in normal children(p<0.001), obese children(p<0.001) and highly obese children(p<0.05) but not in slim children. The effect of education was not significant for children's dietary habits. Practice plan showed significant changes in all of children(p<0.001). This suggests that nutrition education should be executed from early age. It is necessary to define the goals of systematic nutrition education fit for children's level and to develop various education programs and teaching materials.
Child ; Eating ; Education* ; Food Habits* ; Gyeonggi-do ; Humans ; Nutritionists ; Somatotypes ; Teaching Materials

We report a case of eosinophilic pustular folliculitis in a 6-month-old male infant who had pruritic, tiny, erythematous papulopustules on his scalp and eosinophilia in the peripheral blood. Histopathologic examination revealed an acute follicu1ar and perifollicular inflammatory infiltrate with abundant eosinophils. The patient responded to systemic and topical corticosteroid and dapsone.
Dapsone ; Eosinophilia ; Eosinophils* ; Folliculitis* ; Humans ; Infant ; Male ; Scalp

No abstract available.
Cartilage* ; Glutaral* ; Rabbits* ; Transplants*

BACKGROUND: Arthroscopic fixations for large and comminuted bony Bankart lesions are technically difficult. We developed an arthroscopic multiple pulled suture (MPS) technique to restore large and comminuted bony Bankart lesions. METHODS: Ten patients (mean age, 49.8 years; range, 31–79 years) underwent bony Bankart repair using the illustrated MPS technique and were then followed for a mean of 27.3 months. A plain radiograph series and three-dimensional computed tomography scans were taken at the initial clinical evaluation and 3 months postoperatively. Outcome measurements included the American Shoulder and Elbow Surgeons (ASES) score, Rowe score, University of California at Los Angeles (UCLA) score, and subjective patient satisfaction, along with surgical complications. RESULTS: Union of an osseous fragment with the glenoid rim was confirmed in all patients on a computed tomography scan 3 months after operation. The osseous fragment was restored to proper articular congruence and reduction. The affected shoulder was stable in nine of the 10 patients. One patient presented with a redislocation after a sports injury 3 years postoperatively. The ASES, Rowe, and UCLA scores improved at the final evaluation, and median patient satisfaction at the final follow-up was 9 of 10 points (range, 6–10 points). CONCLUSIONS: The arthroscopic MPS technique for bony Bankart lesions with large or comminuted osseous fragments was a relatively easy and safe method for stable fixation of the osseous fragment. Therefore, the arthroscopic MPS technique resulted in good restoration of stability with high patient satisfaction and low complication rates.
Athletic Injuries ; California ; Elbow ; Follow-Up Studies ; Humans ; Methods ; Patient Satisfaction ; Shoulder ; Surgeons ; Sutures

We analyzed the mRNA expression patterns of major potassium channel genes to determine the mechanism of hypokalemia in familial hypokalemic periodic paralysis. We used quantitative RT-PCR to examine the mRNA levels of both inward (KCNJ2, KCNJ6, and KCNJ14) and delayed rectifi er (KCNQ1 and KCNA2) potassium channel genes in skeletal muscle cells from both normal and patient groups, prior to and after exposure to 4 mM and 50 mM potassium buffers. Quantitative RT-PCR analysis revealed no changes in the mRNA levels of these genes in normal and patient cells on exposure to 4 mM potassium buffer. However, after exposure to 50 mM potassium buffer, which was used to induce depolarization, normal cells showed a signifi cant decrease in KCNJ2, KCNJ6, and KCNJ14 expression, but no change in KCNQ1 and KCNA2 expression. In contrast, patient cells showed no change in KCNJ2 and KCNJ6 expression, but an increase in KCNJ14 expression. Furthermore, KCNQ1 and KCNA2 showed decreased expression. We found that the expression levels of both inward and delayed rectifi er potassium channel genes in patient cells differ from those in normal cells. Altered potassium channel gene expression in patient cells may suggest a possible mechanism for hypokalemia in familial hypokalemic periodic paralysis.

A 26-year-old man with a one-year history of asthma and sinusitis presented with bilateral pleural effusions, patch basilar infiltrates on a chest x-ray and a pericardial effusion on an echocardiogram. The peripheral blood showed marked eosinophilia. An obstructive pattern was also observed during the pulmonary fuction test, which was responsive to bronchodilator inhalation. Nerve conduction studies showed right sural neuropathy. Thoracentesis yielded an acidotic exudative effusion with low glucose, low C3 and eosinophilia. An open lung biopsy revealed an eosinophilic interstitial pneumonitis associated with a necrotizing eosinophilic vasculitis, and granulomatous inflammation foci. In the literature, pleural effusions were reported in 29 percent of Churg-Strauss patients, but the number of effusions was low and their characteristics have not been well described. This report describes the characteristic findings of pleural fluid and its histologic features in a case of classical Churg-Strauss syndrome.
Adult ; Asthma ; Biopsy ; Churg-Strauss Syndrome* ; Eosinophilia ; Eosinophils ; Glucose ; Humans ; Inflammation ; Inhalation ; Lung ; Lung Diseases, Interstitial ; Neural Conduction ; Pericardial Effusion ; Pleural Effusion* ; Sinusitis ; Thorax ; Vasculitis

No abstract available.
Cyclosporine* ; Scleredema Adultorum*

PURPOSE: We investigated the statistical methods to compose the functional brain map of human working memory and the principal factors that have an effect on the methods for localization. MATERIALS AND METHODS: Repeated PET scans with successive four tasks, which consist of one control and three different activation tasks, were performed on six right-handed normal volunteers for 2 minutes after bolus injections of 925 MBq H0 at the intervals of 30 minutes. Irnage data were analyzed using SPM96 (Statistical Parametric Mapping) imple-mented with Matlab (Mathworks Inc., U.S.A.). Images from the same subject were spatially registered and were normalized using linear and nonlinear transformation methods. Significant difference between control and each activation state was estimated at every voxel based on the general linear model. Differences of global counts were removed using analysis of covariance (ANCOVA) with global activity as covariate. Using the mean and variance for each condition which was adjusted using ANCOVA, t-statistics was performed on every voxel To interpret the results more easily, t-values were transformed to the standard (saussian distri-bution (Z-score). RESULTS: All the subjects carried out the activation and control tests successfully. Average rate of correct answers was 95%. The numbers of activated blobs were 4 for verbal memory I, 9 for verbal memory II, 9 for visual memory, and 6 for canjunctive activation of these three tasks. The verbal working memory activates predominantly left-sided slruetures, and the visual memory activates the right hernisphere. CONCLUSION: We conclude that rCBF PET imaging and statistical parametric mapping method were useful in the localization of the brain regions for verbal and visual working memory.
Brain Mapping* ; Brain* ; Electrons* ; Healthy Volunteers ; Humans ; Linear Models ; Memory ; Memory, Short-Term ; Positron-Emission Tomography*