Altered expression of potassium channel genes in familial hypokalemic periodic paralysis

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.

Systemic Therapy of Corticosteroid

The corticosteroids have come to be widely used in ophthalmology since the introduction of cortisone and adrenocorticotropic hormone(ACTH) in 1949. Although the glucocorticoids are given by the ophthalmologist for their anti-inflammatory effect. they produce many other side effects. The indications, contraindications of systemic therapy were listed. There is no good evidence that, given in full therapeutic dose, the anti-inflammatory activity of one particular corticosteroid is better than another. The major considerations in the choice of the drug are the side effects and the cost to the patient. Although prednisone and prednisolone cause sodium retention, these are clear1y the drug of choice. If sodium retention becomes a factor, switching to dexamethasone is indicated. Dexamethasone stimulates the appetite considerably, and excessive weight gain may result. Triamcinolone, on the other hand, depresses the appetite and is the drug of choice when sodium retention and increased appetite present problems. In several regimens of therapy, the following three are considered best. The first is the divided daily dose, usually four times a day. Next is the single daily dosage, taken in the morning. An every-other-day dosage is advocated in long-term therapy. For the most part in ophthalmic diseases a single daily dose is the preferable treatment. Usual starting therapeutic dosage and average maintenance doses were tabled. The most common side effects of corticosteroid therapy were listed. The other considerations in systemic therapy were described.

Frontal Mucocele: 2 case-reports of the exophthalmos due to frontal mucocele, with the review of the literatures

Last year, we had experienced 2 cases of frontal mucocele from which exophthalmos was induced. In both cases, all were male and exophthalmos was unilateral at right eye. They revealed visual disturbances and ocular deviations to the temporal and inferior direction, a]so with the diplopia in one case. The duration of the exophthalmos was 5 months in one case, and 10 years long in another case. They were treated by means of external sinusotomy through the anterior orbitotomy with good results. In one case visual disturbance was also recovered to the normal.

Exophthalmos Inhibitory Effect of Anti-EPS Serum

Gold fishes were used to determine exophthalmos-inhibitory effect of anti-EPS serum. Anti-EPS Serum was obtained by repeated injection of serum from a patient of malignant exophthalmos to a guinea pig. The exophthalmos-inhibitory effect on fish was determined by measuring the intercorneal distance of fishes by the use of a micrometer before and after the injection of sera, and the percentage in that distance were calculated. The responsiveness of fishes was partly controlled by the injections of normal guinea pig serum and the serum from a patient of endocrine exophthalmos. The injections of the serum from the patient provoked exophthalmos. The injections of the serum from the patient, followed by the injections of normal serum of guinea pig provoked no exophthalmos. The injections of the patient's serum followed by injections of antiserum of guinea pig resulted in marked enophthalmos. These results suggest that the normal guinea pig serum and the antiserum from the guinea pig contains exophthalmos-inhibitory factor, and that this effect was greater in the serum treated by patient's serum.

Two Cases of Malignant Melanomas of Choroid

Two cases of malignant melanomas of the choroid were reported. The first case was 43 years old male. The tumor was found to be a mixed type with heavy pigmentation. The second case was 3 years old girl. The tumor cells infiltrated the sclera and the optic nerve with complete destruction of the choroid and extension to the extra-ocular portion was noted without encapsulation. The tumor was composed of spindle A type cells with scanty pigmentation. This second case was interesting due to the reported rare incidence of malignant melanoma in childhood. The statistics in Japan point to the relativelyfrequent occurrence in childhood suggesting the racial differences in the frequency of malignant melanomas in childhood.