A Case of Kawasaki Disease Complicated With Cerebral Salt-Wasting Syndrome.

We report the case of a 3-years-old boy who developed severe hyponatremia and unconsciousness during an episode of Kawasaki disease (KD). He was diagnosed with cerebral salt-wasting syndrome (CSWS), which has not previously been reported as a complication of KD. He was diagnosed with KD with fever and four clinical signs and received intravenous immunoglobulin (IVIG) on the day after onset. Hyponatremia had been observed, and it worsened after IVIG. At first, syndrome of inappropriate antidiuretic hormone secretion (SIADH) was suspected, but his hyponatremia did not improve by restriction of water intake. The patient's consciousness level decreased along with the worsening hyponatremia. Electroencephalography revealed abnormal electrical discharge concordant with acute encephalopathy. Laboratory data showed hypouricemia with high fractional excretion of uric acid (FEUA), in addition to a negative balance of both Na and water. We diagnosed KD complicated with CSWS. The patient improved promptly with appropriate Na supplementation and water correction.

A case of incomplete Kawasaki disease with extremely high serum ferritin and interleukin-18 levels.

BACKGROUND: The clinical features and laboratory parameters of patients with Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (sJIA) occasionally overlap. Therefore, serum levels of cytokine and ferritin are used as markers to distinguish between KD and sJIA. KD patients have a high level of interleukin (IL)-6, low level of IL-18, and no elevation of the level of serum ferritin. Conversely, sJIA patients have a low level of IL-6 and high levels of IL-18 and ferritin in the serum. However, to the best of our knowledge, no case report of KD with a low serum level of IL-6 and extremely high levels of IL-18 and ferritin is found. CASE PRESENTATION: A 6-year-old boy presented with a history of fever for 9 days and a rash that appeared 7 days from the onset. He was diagnosed with incomplete KD because of fever, skin rash, oral cavity erythematous changes, and erythema and edema of the hands with laboratory findings of serum albumin level < 3.0 g/dL, elevated alanine aminotransferase level and leukocyturia. Intravenous immunoglobulin and prednisolone and oral aspirin were introduced on the 10th day. Fever subsided 1 day after initiating the treatment, but arthritis of both knees appeared in addition to hepatosplenomegaly. We suspected sJIA, as the serum level of ferritin was 19,740 ng/mL, IL-6 was < 3 pg/mL, and IL-18 was 132,000 pg/mL. Skin desquamation of the fingertips was observed 18 days from the onset; thus, he was finally diagnosed with incomplete KD with arthritis. At 32 days from the onset, we stopped the prednisolone therapy and no symptoms of relapse were observed afterwards. In the follow-up at 16 months from the onset, he had neither signs of active joint or skin involvement, nor cardiac involvement. CONCLUSIONS: Although patients with sJIA generally have high serum levels of IL-18 and ferritin, this was a case of incomplete KD with extremely high serum levels of IL-18 and ferritin. Serum cytokine and ferritin are often used for the differential diagnosis of KD and sJIA. We need to recognize the existence of KD with high serum levels of IL-18 and ferritin.

Versatile Controls of Microdomain Morphologies and Temperature Dependencies in Lamellar Spacing by Blending Diblock Copolymers Bearing Antisymmetric Compositions.

The morphologies of the microphase-separated structures in the binary blends of diblock copolymers (AB/AB) have been studied intensively for the case of diblock copolymers bearing antisymmetric compositions with similar molecular weights. Here, the two diblock copolymers 1 and 2, of which compositions are 0.5 - x and 0.5 + x (0 < x < 0.5), respectively, were blended, and the morphology diagram was constructed in the plot of χZ vs the average composition of the A component, where χ is the interaction parameter between A and B segments and Z is the average degree of polymerization of the two AB diblock copolymers. The temperature-dependent morphologies were analyzed by synchrotron small-angle X-ray scattering (SAXS) measurements. It was found that the morphology diagram agrees in principle with the theoretical one for the neat AB diblocks by Matsen and Bates (Macromolecules 1996, 29, 1091-1098), although the disordered phase was a bit expanded in the experimentally determined morphology diagram. Anomalous temperature dependencies in the lamellar spacing have been also comprehensively studied for the binary blends of antisymmetric diblock copolymers as a function of the degree of compositional asymmetry by closely adjusting the average composition in the blend specimen at 0.50. For this purpose, more than 20 neat diblock copolymers have been synthesized with a wide range of compositions from 0.20 to 0.87 and a range of molecular weight of 12 000-33 800. The temperature dependencies of the lamellar spacing were also analyzed by synchrotron SAXS measurements. As a result, the following things were found. The scaling exponent α in D ∼ T α was still negative but slightly larger than the usual value (i.e., α = -0.33) for the smaller degree of asymmetry in the composition (i.e., x is small), while α became positive for the higher degree of asymmetry. The latter result is very anomalous because the temperature dependence is opposite (i.e., the lamellar spacing increases with an increase of temperature). The value of α was found to be linearly rationalized with the degree of asymmetry τ (which is especially introduced in the current paper for this purpose), for the binary blends with the average composition of 0.50. Based on this result, one can prepare lamellar microdomains, of which spacing does not change with temperature, by blending two diblock copolymers with τ = 1.33 (corresponding to 0.3 and 0.7 of compositions) having similar molecular weights. This would be important for manufacturing materials with properties (for instance, the optical property) independent of temperature. From the current study, the binary blends of the antisymmetric diblock copolymers are concluded to be versatile such that the precise controls of the morphologies and the temperature dependencies of the lamellar microdomains are plausible.

Successful control of life-threatening polymorphic ventricular tachycardia by radiofrequency catheter ablation in an infant.

We present a case of a 9-month-old girl in whom malignant polymorphic ventricular tachycardia (VT) was successfully controlled by radiofrequency catheter ablation under guidance with a three-dimensional mapping system. The VTs originated from the left ventricular lateral wall, left ventricular anterior wall, and left ventricular apex. At least six types of VTs were documented during the electrophysiology study. All VTs were successfully controlled after two sessions of radiofrequency catheter ablation, and she was discharged from our hospital on propranolol, mexiletine, flecainide, and aprindine.

Anderson's disease/chylomicron retention disease in a Japanese patient with uniparental disomy 7 and a normal SAR1B gene protein coding sequence.

BACKGROUND: Anderson's Disease (AD)/Chylomicron Retention Disease (CMRD) is a rare hereditary hypocholesterolemic disorder characterized by a malabsorption syndrome with steatorrhea, failure to thrive and the absence of chylomicrons and apolipoprotein B48 post-prandially. All patients studied to date exhibit a mutation in the SAR1B gene, which codes for an essential component of the vesicular coat protein complex II (COPII) necessary for endoplasmic reticulum to Golgi transport. We describe here a patient with AD/CMRD, a normal SAR1B gene protein coding sequence and maternal uniparental disomy of chromosome 7 (matUPD7). METHODS AND RESULTS: The patient, one of two siblings of a Japanese family, had diarrhea and steatorrhea beginning at five months of age. There was a white duodenal mucosa upon endoscopy. Light and electron microscopy showed that the intestinal villi were normal but that they had lipid laden enterocytes containing accumulations of lipid droplets in the cytoplasm and lipoprotein-size particles in membrane bound structures. Although there were decreased amounts in plasma of total- and low-density lipoprotein cholesterol, apolipoproteins AI and B and vitamin E levels, the triglycerides were normal, typical of AD/CMRD. The presence of low density lipoproteins and apolipoprotein B in the plasma, although in decreased amounts, ruled out abetalipoproteinemia. The parents were asymptomatic with normal plasma cholesterol levels suggesting a recessive disorder and ruling out familial hypobetalipoproteinemia. Sequencing of genomic DNA showed that the 8 exons of the SAR1B gene were normal. Whole genome SNP analysis and karyotyping revealed matUPD7 with a normal karyotype. In contrast to other cases of AD/CMRD which have shown catch-up growth following vitamin supplementation and a fat restricted diet, our patient exhibits continued growth delay and other aspects of the matUPD7 and Silver-Russell Syndrome phenotypes. CONCLUSIONS: This patient with AD/CMRD has a normal SAR1B gene protein coding sequence which suggests that factors other than the SAR1B protein may be crucial for chylomicron secretion. Further, this patient exhibits matUPD7 with regions of homozygosity which might be useful for elucidating the molecular basis of the defect(s) in this individual. The results provide novel insights into the relation between phenotype and genotype in these diseases and for the mechanisms of secretion in the intestine.

The follow-up evaluation of electrocardiogram and arrhythmias in children with fulminant myocarditis.

BACKGROUND: Fulminant myocarditis involves various serious arrhythmias that sometimes have lethal consequences. The purpose of the present study was to investigate the electrocardiogram findings, arrhythmogenicity and abnormalities of the cardiac conduction system in children with fulminant myocarditis. METHODS AND RESULTS: Between 1999 and 2008, 7 consecutive patients (mean age: 7 years) who suffered from fulminant myocarditis were included in the study. A 12-lead electrocardiogram, Holter monitoring and signal-averaged electrocardiograms were performed and compared between the acute, convalescent, and recovery phases in the 4 surviving patients. Also, electrophysiologic assessment was carried out during the convalescent phase. Five out of 7 patients developed complete atrioventricular block, 3 developed ventricular tachycardia, 2 had cardiac arrest, 2 developed sinus tachycardia, 1 developed ventricular fibrillation, 1 had advanced atrioventricular block, and 1 developed sick sinus syndrome. Among the surviving patients, all arrhythmias resolved during the convalescent and remote phases. No atrial or ventricular arrhythmias were induced in any patients during the programmed stimulation study. In the convalescent phase, no arrhythmias could be induced and there were no signs of any conduction abnormalities on electrophysiological assessment. CONCLUSIONS: Close follow-up should be performed to observe for the occurrence of any new arrhythmias and/or a decrease in cardiac function in children with fulminant myocarditis.

Electrophysiological characteristics of idiopathic ventricular tachycardia in children.

BACKGROUND: Idiopathic ventricular tachycardia (VT) has been reported to have a good prognosis, but there still might be the potential risk of sudden death. METHODS AND RESULTS: The 46 consecutive children (mean age 11.7 ± 3.4 years) with idiopathic VT were enrolled in this study. Monomorphic VT was detected in 39 patients and polymorphic VT in 7 patients. The VT originated from the right ventricle (RV) in 22 patients, and left ventricle (LV) in 17 patients. The VT was induced by exercise in 68% of the RVVT, 41% of the LVVT, and 100% of the polymorphic VT. The VT was induced by programmed ventricular stimulation in 41% of the RVVT, 35% of the LVVT, and none of the polymorphic VT. Adenosine tri-phosphate terminated the VT in 9 of 15 patients (60%). The mechanism of the VT was suspected to be triggered by activity in 36.4%, automaticity in 40.9%, and re-entry in 22.7% of the RVVT, whereas it was 52.9%, 5.9%, and 41.2% of the LVVT, respectively. CONCLUSIONS: The exercise inducibility was higher in polymorphic VT than the RVVT and LVVT, but no difference in the programmed stimulation. The sensitivity to adenosine tri-phosphate was not different between the RVVT and LVVT. In some patients with idiopathic VT, a non-verapamil sensitive re-entry was documented, which was more common in patients with ischemic heart disease or cardiomyopathy.

Clinical effectiveness of pulmonary vein isolation for arrhythmic events in a patient with catecholaminergic polymorphic ventricular tachycardia.

An 18-year-old woman with catecholaminergic polymorphic ventricular tachycardia (CPVT) underwent pulmonary vein isolation (PVI) because of frequent and inappropriate shocks from an implantable cardioverter defibrillator (ICD) associated with atrial fibrillation (AF) with a rapid ventricular response. While the PVI did not completely suppress the AF induced by an isoproterenol infusion, the Holter monitor recordings demonstrated a major decrease in the clinical episodes of AF and ventricular tachyarrhythmias in association with a reduced high-frequency (HF) component and ratio of the low-frequency (LF) component power to the HF component (LF/HF) after the PVI. The PVI can decrease the substrates that trigger and maintain the AF when it involves a pulmonary vein origin, and may exert an additional effect on the sympathetic nerve input to the heart. The PVI may be an adjunctive therapy for CPVT cases with drug refractory AF causing inappropriate ICD discharges.

Decremental accessory pathway conduction after ablation and antidromic atrioventricular reciprocating tachycardia 8 years after successful radiofrequency ablation.

INTRODUCTION: This is a rare case of antidromic reciprocating tachycardia developing 8 years after successful catheter ablation. RESULT: A 15-year-old girl had recurrence of palpitations 8 years after the ablation of manifest right posteroseptal accessory pathway. Atrial burst pacing revealed Wenckebach atrioventricular conduction with preexcitation. Wide QRS tachycardia with identical morphology to sinus rhythm associated with retrograde His potential recorded immediately after the V-wave was induced by isoproterenol infusion. Atrial premature stimulus applied at the identical timing of His potential advanced the subsequent ventricular beat and His potential. CONCLUSION: Catheter ablation may produce decremental accessory pathway conduction and rarely cause antidromic atrioventricular reciprocating tachycardia. This may be explained by a presence of "de novo" accessory pathway with decremental conduction properties that became manifest after the first ablation.

Association of sinus node dysfunction, atrioventricular node conduction abnormality and ventricular arrhythmia in patients with Kawasaki disease and coronary involvement.

BACKGROUND: This study was performed to investigate the incidence of arrhythmias in patients with Kawasaki disease (KD). METHODS AND RESULTS: Electrophysiologic studies (EPS) were performed in 40 patients (mean age: 10.3+/-5.1 years; 30 males, 10 females) with KD who had severe to moderate coronary artery disease. Clinical arrhythmias were documented in 4 patients (premature ventricular contractions, ventricular tachycardia, atrioventricular block, and ventricular fibrillation). Dual atrioventricular nodal pathways were demonstrated in 3 patients. Nonsustained atrial fibrillation was induced in 1 patient. The AH interval was prolonged in 2 patients. The Wenckebach rate was 164+/-37 beats/min, and 4 of the patients had a decreased Wenckebach rate. The maximum and corrected sinus node recovery times were 997+/-257 ms and 281+/-130 ms, respectively, and 7 patients were thought to be abnormal. The sino-atrial conduction time was 108+/-64 ms, and 2 patients had prolonged conduction times. CONCLUSIONS: Although there was no relationship between coronary stenosis or obstruction and the EPS parameters, the incidence of abnormal sinus node and atrioventricular node function is apparently higher in KD patients than in the normal population. These functional abnormalities may possibly be caused by myocarditis or an abnormal microcirculation in the sinus node and atrioventricular node artery. In some patients, myocardial ischemia may provoke malignant ventricular arrhythmia.

Clinical importance of Koch's triangle size in children: a study using 3-dimensional electroanatomical mapping.

BACKGROUND: Catheter ablation inside the Koch's triangle has a risk for complete atrioventricular block. METHODS AND RESULTS: The anatomic size of the coronary sinus (CS) and His bundle (HB) in children and the distance between them was studied using a 3-dimensional electroanatomical mapping system (CARTO). Fifty-three children (mean age, 11.8+/-3.7 years) without congenital heart disease (ie, 24 with atrioventricular re-entrant tachycardia, 18 with atrioventricular nodal re-entrant tachycardia, 7 with atrial tachycardia, 2 with ventricular tachycardia and 2 with atrial flutter) were studied. The size of the HB recording area was 148+/-97 mm2 and the size of the CS was 66+/-44 mm2. The size of the CS and the distance between the HB and CS (18+/-7 mm) were proportional to body weight, body length and body surface area. All patients underwent catheter ablation, including 25 ablations inside Koch's triangle. Catheter ablation was successful in 52 patients without any atrioventricular nodal injury. CONCLUSIONS: The CS size and the distance between the HB and CS increased proportionally with children's growth. To know the distance from the HB to the ablation point is useful in avoiding atrioventricular node injury, and information about the length of Koch's triangle may provide supportive information when applying radiofrequency energy inside Koch's triangle without needing to use the CARTO system in children, but this merits further investigation.