Incontinentia pigmenti acb.romians(Ito) is characterized by progressive bizarre or whorl-like hypopigmentation on trunk and extrementies during childhood. It is similar to a negative picture of incontinentia pigmenti(Blocb-Sulzberger) and not infrequently associated with mental, bony and ocular defects. The incidence of this disease is predominent in femaIe without hereditary background. Case 1. Two years old female was visited to our clinic because of mottled depigmented patches on right thigh for about 8 months. Case 2. 14 months oId female was visited to our clinic because of linear and. mottled depigmented natches on their limbs for about 7 months. Histopathological findings of both cases shows the focal depigmentation on basal layer. They are treated with steroid ointment with moderate to good effects.
Extremities ; Female ; Humans ; Hypopigmentation ; Incidence ; Incontinentia Pigmenti* ; Pigmentation Disorders* ; Thigh

BACKGROUND: Many reports have shown that hormone replacement therapy(HRT) in postmenopausal women decreases lipoprotein(a)[Lp(a)]. However these had small numbers of subjects, short duration of therapy, or comparisons of only a few regimens. The influences of progesterone on Lp(a) and lipids, administered with estrogen, are controversial. METHODS: Five hundred and fifty-one postmenopausal women were divided into 4 groups : group A ; 0.625mg conjugated equine estrogen(CEE)(m=140), group B ; 0.625mg CEE plus 5mg medroxyprogesterone acetate(MPA)(m=97), group C ; 0.625mg CEE plus 10mg MPA(n=109), and group D ; 2mg estradiol valerate(E2) plus 0.5mg norgestrel(N)(n=134) and group E ; control(n=71). Lp(a) and lipids levels were measured before and 12 months after HRT. RESULTS: Estrogen replacement therapy(ERT) for 12 months lowered Lp(a) level by 37.1%. The addition of progesterone attenuated the Lp(a)-lowering effect of estrogen and decreased by 27.7%, 29.6%, and 30.3% in groups B(p<0.05), C(p<0.05), and D(p<0.0001) respectively. High density lipoprotein cholesterol(HDL-C) was increased markedly in group A(16.5%), increased moderately in groups B(10.8%) and C(11.3%), and not changed in group D. Low density lipoprotein cholesterol was decreased by 10.9%, 13.7%, 11.3%, and 17.6% in groups A, B, C, and D respectively. CONCLUSIONS: Reduction of Lp(a) with estrogen replacement therapy may be one of mechanisms for cardioprotective effect in postmenopausal women. The combined therapy of estrogen and progesterone may reveal different effects on heart due to adverse actions of progesterone on Lp(a) and HDL-C. The variations in the androgenic potency of progesterone may explaine inconsistent results on HDL-C in previous studies.
Cholesterol, LDL ; Estradiol ; Estrogen Replacement Therapy ; Estrogens ; Female ; Heart ; Hormone Replacement Therapy* ; Humans ; Lipoprotein(a)* ; Lipoproteins ; Medroxyprogesterone ; Progesterone*

One case of subungual glomus tumor was reported which had characteristic clinical manifestations of paroxysmal pain and tenderness for about 20 years. Histopathologically it was confirmed as subungual glomus tumor. This case was treated completely with simple surgics,l excision of the mass.
Glomus Tumor*

The present study was aimed at investigating the effect of cyclosporine A (CsA) on the renal renin-angiotensin systems. In rats chronically treated with CsA, the intrarenal expression of various genes of the renin-angiotensin system was assessed by Northern blot analysis. Along with the increases in plasma and renal renin activities, chronic CsA-treatment differentially affected the renal expression of renin-angiotensin system. The treatment with CsA for one week did not significantly alter the expression of either type 1 angiotensin II receptor (AT1A) or angiotensinogen gene, but increased the renin mRNA level. The three-week-treatment caused increases in the expression not only of renin but also of AT1A and angiotensinogen genes. Supplementation with L-arginine kept the expression of renin mRNA normal in the one-week-treated, but failed to prevent the alterations of the gene expression in the three-week-treated. Feedback control among components of the renin-angiotensin system also influences angiotesinogen. In the liver, the expression of angiotensinogen mRNA was decreased by the CsA-treatment for either one- or three-weeks. In conclusion, chronic CsA-treatment is associated with a differential expression of various genes for the renin-angiotensin system. L-Arginine may be effective in maintaining the normality of renin-angiotensin system only during early period after beginning the use of CsA.
Angiotensinogen ; Animals ; Arginine ; Blotting, Northern ; Cyclosporine* ; Gene Expression ; Liver ; Plasma ; Rats ; Receptors, Angiotensin ; Renin ; Renin-Angiotensin System* ; RNA, Messenger

Background and objectives: Carvedilol is a cardiovascular drug, beta- and alpha1-adrenoceptor antagonist, currently approved for the treatment of hypertension, angina, congestive heart failure by FDA. Carvedilol has been shown to attenuate oxygen free radical-initiated lipid peroxidation and to inhibit neointimal formation of aorta following vascular injury by balloon angioplasty. We have investigated the effect of carvedilol on DNA synthesis of vascular smooth muscle cells (VSMC) stimulated by platelet-derived growth factor (PDGF)-BB. MATERIALS AND METHOD: Rat aortic smooth muscle cells were obtained by the combined collagenase and elastase methods. Cells between the 4th and 8th passages were used for the experiments. Incorporated radioactivity of [3H]-thymidine was measured by liquid scintillation spectrometry. RESULTS: PDGF-BB (1 nM) increased [3H]-thymidine incorporation about 70-100% over basal value in cultured VSMC. PDGF-stimulated increase in DNA synthesis was significantly suppressed by simultaneous administration of carvedilol. In contrast, propranolol did not significantly affect 3[H]-thymidine uptake in rat aortic VSMC. CONCLUSION: The present study demonstrate that carvedilol significantly inhibits the proliferation of vascular smooth muscle cell in our condition. These results indicate that carvedilol may be effective in the treatment of cardiovascular diseases principally associated with abnormal vascular smooth muscle growth.
Angioplasty, Balloon ; Animals ; Aorta ; Cardiovascular Diseases ; Cell Proliferation ; Collagenases ; DNA ; Heart Failure ; Hypertension ; Lipid Peroxidation ; Muscle, Smooth, Vascular* ; Myocytes, Smooth Muscle ; Oxygen ; Pancreatic Elastase ; Platelet-Derived Growth Factor ; Propranolol ; Radioactivity ; Rats ; Spectrum Analysis ; Vascular System Injuries

No abstract available.

The fundamental alterations in the cardiovascular system that occur consequent to aging are of great pratical importnace to clinicians. Senescent cardic muscle has many features of prolonged tension development, impaired relaxation and diminished response to receptor-mediated inotropic interactions. We estimated left ventricular mass by 2-D echo area-length method using a high quality planimeter. Age-related increments in left ventricular mass demostrated, but left ventricular enddiastolic cavity volumes were unaffected by age. The increase in left ventricular mass observed with aging is typical of pressuer-overload hypertrophy and its stimulus may be increased afterload from senescent changes.
Aging* ; Cardiovascular System ; Hypertrophy ; Relaxation

The present study was aimed at investigating the role of type II 11beta-hydroxysteroid dehydrogenase (IIbeta- HSD II) in the development of hypertension. Two-kidney, one-clip (2K1C), deoxycorticosterone acetate (DOCA)/salt, or NG-nitro-L-arginine methyl ester (L-NAME) hypertension was induced in male Sprague- Dawley rats. Four weeks later, the expression of 11beta-HSD II mRNA was determined in the kidney by Northern blot analysis. The plasma level of aldosterone was measured by radioimmunoassay. In 2K1C hypertension, the expression of 11beta-HSD II was decreased in the clipped kidney and increased in the non-clipped kidney. The expression was increased in the remnant kidney of DOCA/salt hypertension, while decreased in the kidneys of L-NAME hypertension. The plasma level of aldosterone was increased, decreased, and remained unchanged in 2K1C, DOCA/salt, and L-NAME hypertension, respectively. The down-regulation of 11beta-HSD II may contribute to the sodium retention, thereby increasing the blood pressure in 2K1C and L-NAME hypertension. On the contrary, the up-regulation in DOCA/salt hypertension may play a compensatory role to dissipate the sodium retention.
11-beta-Hydroxysteroid Dehydrogenases* ; Aldosterone ; Animals ; Blood Pressure ; Blotting, Northern ; Desoxycorticosterone ; Down-Regulation ; Humans ; Hypertension* ; Kidney* ; Male ; NG-Nitroarginine Methyl Ester ; Plasma ; Radioimmunoassay ; Rats* ; RNA, Messenger ; Sodium ; Up-Regulation

PURPOSE: To analyze the clinical features and natural history of acquired third, fourth, and sixth cranial nerve palsy. METHODS: We reviewed the medical records of 89 patients who were diagnosed with acquired third, fourth, and sixth nerve palsy from January 2003 to March 2005. The natural course of the disease and the factors affecting recovery were analyzed for the 66 patients who had their first ocular examination within 3 months from onset and were followed up for at least 6 months. RESULTS: The average age of onset was 50.1 years. The sixth cranial nerve was affected most frequently (n=43, 48.3%). Vascular disease (n=27, 30.3%) was most common etiology of cranial nerve palsy, followed by an undetermined cause (n=19, 21.3%). Of the 66 patients who had their first ocular examination within 3 months from onset and were followed up for at least 6 months, 40 (60.6%) patients showed a decrease in the angle of deviation by more than 10 prism diopters, and of these, 32 (48.5%) patients made a complete recovery from pareses. The recovery rates for patients with vascular disease or undetermined etiology (p=0.001), milder initial eyeball deviation and ocular motor restriction (p<0.001) were higher. CONCLUSIONS: In the natural course of the disease, the recovery rate of acquired third, fourth, and sixth nerve palsy was 60.6%. The most favorable prognosis occurred with vascular disease, undetermined etiology, and less severe paralysis on onset.
Abducens Nerve Diseases* ; Abducens Nerve* ; Age of Onset ; Cranial Nerve Diseases ; Humans ; Medical Records ; Natural History* ; Paralysis ; Paresis ; Prognosis ; Vascular Diseases

BACKGROUND/OBJECTIVES: Nobiletin (NOB), a citrus flavonoid, is reported to have beneficial effects on cardiovascular and metabolic health. However, there is limited research investigating the effect of long-term supplementation with low-dose NOB on highcholesterol diet (HCD)-induced hypercholesterolemia and non-obese nonalcoholic fatty liver disease (NAFLD). Therefore, we investigated the influence of NOB on hypercholesterolemia and NAFLD in HCD-fed mice. SUBJECTS/METHODS: C57BL/6J mice were fed a normal diet (ND) or HCD (35 kcal% fat, 1.25% cholesterol, 0.5% cholic acid) with or without NOB (0.02%) for 20 weeks. RESULTS: HCD feeding markedly reduced the final body weight compared to ND feeding, with no apparent energy intake differences. NOB supplementation suppressed HCD-induced weight loss without altering energy intake. Moreover, NOB significantly decreased the total cholesterol (TC) levels and the low-density lipoprotein (LDL)/very-LDL-cholesterol to TC ratio, and increased the high-density lipoprotein-cholesterol/TC ratio in plasma, compared to those for HCD feeding alone. The plasma levels of inflammatory and atherosclerosis markers (C-reactive protein, oxidized LDL, interleukin [IL]-1β, IL-6, and plasminogen activator inhibitor-1) were significantly lower, whereas those of anti-atherogenic adiponectin and paraoxonase were higher in the NOB-supplemented group than in the HCD control group. Furthermore, NOB significantly decreased liver weight, hepatic cholesterol and triglyceride contents, and lipid droplet accumulation by inhibiting messenger RNA expression of hepatic genes and activity levels of cholesterol synthesis-, esterification-, and fatty acid synthesis-associated enzymes, concomitantly enhancing fatty acid oxidationrelated gene expression and enzyme activities. Dietary NOB supplementation may protect against hypercholesterolemia and NAFLD via regulation of hepatic lipid metabolism in HCDfed mice; these effects are associated with the amelioration of inflammation and reductions in the levels of atherosclerosis-associated cardiovascular markers. CONCLUSIONS The present study suggests that NOB may serve as a potential therapeutic agent for the treatment of HCD-induced hypercholesterolemia and NAFLD.