Incontinentia pigmenti is an uncommon genodermatosis. It affects female infants predominantly, described first by Bardach in l925, with the diagnosis of systematized nevus and Bloch in 1926, and Sulzberger in 1928 rnore compIetely. Skin lesions are characterized by 3 stages such as vesicobullous, verucous and finally pigmentary lesions and leave brownish pigmentation on the extremities and trunk. Hesides skin lesions some ectodermaI and mesodermal organs are affected and show developmental abnormalities. We experienced a case of incontinentia pigmenti in a 45-day-old female infant and present it with the review of literature. She showed extensive vesicobullopustular eruption with linear and reticular pigmentation on the extremities and trunk. Clinical and histopathologic findings of these lesions are compatible with Bloch-Sulzberger type of incontinentia pigmenti.
Diagnosis ; Extremities ; Female ; Humans ; Incontinentia Pigmenti* ; Infant ; Mesoderm ; Nevus ; Pigmentation ; Skin

Adrenal Cortex Hormones* ; Prospective Studies* ; Stents*

No abstract available.
Neuroblastoma*

No abstract available.
Anemia, Aplastic*

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.
Child ; Male ; Female ; Humans ; Diagnosis, Differential

BACKGROUND: The purpose of this study was to investigate the mechanism of endothelium-dependent and independent responses to endothelins (ETs) in porcine coronary artery. METHODS: The vascular rings of left anterior descending artery or left circumflex artery from 7 pigs were suspended in conventional organ chambers for the measurement of isometric force. To evaluate relaxation responses, vascular rings with endothelium were exposed to ET-1 and ET-3. To evaluate contraction responses, vascular rings with and without endothelium were exposed to ET-1 and ET-3 in the presence or absence of BQ 123 (ET(A) receptor antagonist) or TAK-044 (ET(A) and ET(B) receptor antagonist). RESULTS: Transient relaxation responses of vascular rings occurred after exposure of ET-1 and ET-3. These transient responses disappeared after preincubation with N-nitro-L arginine. There was an increased contractions of vascular rings according to increasing concentration of ET-1 and ET-3. The initial responses were enhanced in vascular rings without endothelium in ET-1 and ET-3. In vascular rings with endothelium, the contraction responses were more reduced in vascular rings with preincubation of BQ 123 than in vascular rings without BQ 123 in ET-1. In vascular rings without endothelium, the contraction responses were more reduced in vascular rings with preincubation of TAK-044 than in vascular rings without TAK-044 in ET-1. CONCLUSION: ET(B) receptor on the endothelium might mediate the transient vasodilator responses to ET-1 and ET-3 through release of nitric oxide in porcine coronary artery. ET(A) and ET(B) receptor on vascular smooth muscle cells might mediate vasoconstrictor responses to ETs.
Arginine ; Arteries ; Coronary Vessels* ; Endothelins* ; Endothelium ; Muscle, Smooth, Vascular ; Nitric Oxide ; Receptors, Endothelin ; Relaxation ; Swine

To assess the efficacy of 2 different mitral balloon valvotomy (PMV) techniques, PMV was performed using Inoue balloon (I) in 35 pts and double balloon (D) in 33 pts with mitral stenosis (male 27, female 41, mean age 42+/-12 years). The success rate of PMV was 89%(31/35 pts) in I group and 97%(33/33 pts) in D group. Mitral valve area after dilation increased equally effectively in both groups (I and D) from 0.9+/-0.2 and 0.8+/-0.2 to 1.9+/-0.2 and 1.9+/-0.3cm2 respectively (p<0.0001). There were no differences in degree of improvement of cardiac output, mitral gradient, left atrial pressure, pulmonary artery pressure and various doppler echocardiographic findings in both groups, but EF slope was more improved from 13+/-7 to 48+/-16 mm/sec in D group than those (from 15+/-6 to 39+/-15mm/sec) of I group. Increments of long and short diameters of mitral valve orifice by 2D-echocardiogram were 1.1+/-0.6 and 0.3+/-0.3cm in D group and 0.9+/-0.5, 0.4+/-0.3cm in I group. The ratio of long and short diameter increase was significantly larger in D than that of I group (long/short 2.8+/-0.7 vs 2.4+/-0.7, p<0.05) and short diameter of orifice after dilation was more improved in I than that of D group (1.1+/-0.2 vs 1.0+/-0.2cm p<0.05). Complications included deflation failure of Inoue balloon in 2, and cerebral embolic episode in 1 (D). Incidence of increased mitral regurgitation was 50% in D and 45% in I, development of ASD (Qp/Qs>1.2) was 20% in D, 13% in I group respectively and mean amount of left to right shunt (Qp/Qs) was 1.7+/-0.3 in D and 1.5+/-0.1 in I group. Total procedure and fluoroscopic time were 84+/-24 and 25+/-11 min. in D and 56+/-20 and 16+/-6 min. In I, which had statistically significant differences (p<0.002). Thus we concluded PMV using Inoue or double balloons was equally effective in selected patients. Total procedure and fluoroscopic time of Inoue balloon technique were significantly shorter than those of double balloons. Double balloon technique had more tendency of longitudinal splitting of the commissures.
Atrial Pressure ; Balloon Valvuloplasty* ; Cardiac Output ; Echocardiography ; Female ; Follow-Up Studies* ; Humans ; Incidence ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Pulmonary Artery

BACKGROUND AND OBJECTIVES: Many studies have shown that coronary artery bypass graft (CABG) surgery prolongs the life of patients with left main coronary artery disease (LMCD). Recently, percutaneous coronary intervention (PCI) has been applied to treat LMCD, with good clinical results. However, a significant portion of patients decline any revascularization therapy, so receive medical treatment only. The aim of this study was to evaluate the long term clinical outcome in these patients with LMCD, according to the treatment strategies. SUBJECTS AND MEHTODS: The clinical outcomes of 281 consecutive patients, with significant LMCD, between January 1997 and December 2000, were evaluated. The patients were divided into three groups, according to their initial treatment strategies;1) CABG, 2) PCI and 3) medical treatment. The mean follow-up duration was 37.4+/-14.9 months. RESULTS: The 1- and 3-year survival rates in the CABG group (97.4+/-1.5% and 95.6+/-1.9%) were significantly higher than those of the medical group (89.8+/-3.9% and 76.1+/-5.9%;p=0.03). The survival rates in the PCI group (one year and 3-year survival rate, 98.1+/-1.3% and 93.8+/-2.5%) were similar to those of the CABG group (p=0.93). The incidence of 3-year MACE in the medical group (40.7%) was higher than those of the CABG (10.5%, p<0.001) and PCI groups (20.4%, p=0.007). There was no significant difference between the CABG and PCI groups (p=0.06). CONCLUSION: In patients with LMCD, a CABG remains the standard therapy for prolonging survival and lowering the incidence of MACE. PCI offers similar survival benefits in selected patients. Medical treatment is associated with a significantly higher mortality and MACE. Active revascularization therapy should be the treatment of choice for the patients with LMCD.
Angioplasty ; Coronary Artery Bypass ; Coronary Artery Disease ; Coronary Disease* ; Follow-Up Studies* ; Humans ; Incidence ; Mortality ; Percutaneous Coronary Intervention ; Survival Rate ; Transplants

The Na+ -K+ -activated ATPase is required to maintain osmotic balance and stabilize cell volume. The Na+ -K+ -ATPase has a more direct role in regulating cell volume; it controls the solute concentrations inside the cell, thereby regulating the osmotic forces that can make a cell swell or shrink. The impotance of the Na+ -K+ -ATPase in controlling cell volume is indicated by the observation that animal cells swell, and may burst, if they are treated with ouabain, which, inhibits the Na+ -K+ -ATPase. The present experiment was designed and carried out to determine the effect of verapamil, a calcium blocker, on the activity of Na+ -K+ -ATPase prepared from renal medulla in the normal rabbit. It was reported that verapamil, a well known coronary vasodilator, possessed negative inotropic effects. The mechanism of action of verapamil was initially thought to be due to coronary vasodilation and blockade of myocardial B-adrenergic receptors. 1t was termed such agent calcium antagonist. A derivative of verapamil, D-600, was subsequently shown to block the movement of calcium through the slow channel and thereby after the plateau phase of the cardiac action potential. Verapamil do not directly antagonize the effects of calcium. Rather, it inhibit the entry of ealcium into cells or its mobilization form intracellular stores and, as such, have been termed a calcium channel blocker.
Action Potentials ; Adenosine Triphosphatases* ; Adenosine* ; Animals ; Calcium ; Calcium Channels ; Cell Size ; Gallopamil ; Ouabain ; Vasodilation ; Verapamil*

No abstract available.
Pregnancy, Heterotopic*