Kawasaki disease causes coronary artery lesions, such as dilatation, aneurysms, stenosis, and even occlusion in young children, and is one of the most common acquired heart diseases in developed countries. More than 10,000 new cases are reported in Japan every year. In its acute phase, severe coronary arteritis induces morphological changes in coronary arteries. Treatments for Kawasaki disease aim to eliminate coronary artery inflammation as quickly as possible to reduce the chance of causing coronary lesions. Immunoglobulin therapy with aspirin has become the standard therapy of first choice and helps attenuate coronary lesions. In addition to coronary artery disturbances in the acute phase, sclerotic vascular changes were observed in post-Kawasaki disease patients who did not have coronary lesions in the acute phase. Recent studies have revealed peripheral vasculature endothelial dysfunction in post-Kawasaki disease patients with and without coronary lesions. The risk factors for the development of atherosclerosis in adults, such as C-reactive protein, oxidative stress, and inflammatory cytokines, are also increased in the remote phase of Kawasaki disease. This morphological and functional endothelial dysfunction as Kawasaki disease vascular sequelae may suggest the early development of atherosclerosis in patients with Kawasaki disease. However, no direct evidence for this early development has been found so far. Kawasaki disease was first reported slightly more than 40 years ago. The first documented post-Kawasaki disease patients are now becoming old enough to have atherosclerosis. Some case reports suggest myocardial infarction with atherosclerotic changes in young adults who are believed to have a history of Kawasaki disease. This paper reviews Kawasaki disease from the perspective of long-term prognosis.

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The BCR/ABL fusion oncogene found in Philadelphia-positive leukemia exists in three principle forms: p190, p210 and p230. P210 BCR/ABL is commonly found in patients with chronic myelogenous leukemia (CML) and is further categorized into b3a2 or b2a2 subtypes on the basis of the BCR breakpoint. Although these 2 subtypes may be clinically heterogeneous, only the b3a2 BCR/ABL gene has been extensively studied at the molecular and cellular levels. In the present study, we compared the in vivo leukemogenic activity of the b3a2 and b2a2 BCR/ABL genes by using lentiviral transduction/transplantation mouse models. Lineage-depleted bone marrow cells of BALB/c mice were transduced with a lentiviral vector including either b2a2 or b3a2 BCR/ABL cDNA and then transplanted into lethally irradiated mice. In this model, p210 BCR/ABL subtype developed only B220⁺, CD3e^-, Gr1^-, and Mac1^- B-cell acute lymphoblastic leukemia but not myeloid leukemia. There were no differences in the incidence of leukemogenesis, the white blood cell count, the percentage of blast cells, or the survival rates between the b2a2 and b3a2 groups. We have demonstrated that b2a2-type BCR/ABL has leukemogenic activity similar to that of b3a2-type BCR/ABL.

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α₁-Adrenoceptor antagonists are useful antihypertensive agents for patients with hypertension who have hyperlipidemia, benign prostatic hyperplasia, or pheochromocytoma. The purpose of this study was to evaluate the effect of the α₁-adrenoceptor antagonist, doxazosin mesilate, on cerebral blood flow (CBF) and flow velocity in the common carotid artery in patients with hypertension and chronic cerebral infarction.
Doxazosin mesilate (1 mg/day) was orally administered for 4 to 8 weeks to 7 patients with hypertension 4 weeks after the onset of cerebral infarction. We determined blood pressure, heart rate, CBF measured with autoradiography single photon emission computed tomography (SPECT) with N-isopropyl-p-[¹²³I] iodoamphetamine (¹²³I-IMP) as a tracer, and the maximum, minimum and mean flow velocities in the common carotid arteries measured with duplex carotid ultrasonography before and 4 to 8 weeks after the beginning of treatment. Mean CBF was defined as the mean count of tracer from the 8 regions of interest (ROIs) in the frontal, parietal, occipital, and temporal cortices of the cerebral hemisphere. Values were analyzed with paired t tests.
With administration of doxazosin mesilate, systolic pressure significantly decreased from 152 ± 11 to 137 ± 7 mmHg (p<0.01), but diastolic pressure and heart rate were unchanged. Mean CBF was improved significantly from 32.0 ± 4.1 to 34.7 ± 4.1 mL/100 g brain/min (p<0.01) in the ipsilateral cerebral cortex and from 32.6 ± 6.2 to 36.2 ± 5.1 mL/100 g brain/min (p<0.05) in the contralateral cerebral cortex. The maximum, minimum, and mean flow velocities in the bilateral common carotid arteries were not changed significantly.
In the present study, the improvement of mean CBF in the ipsilateral and contralateral cerebral cortices was demonstrated in patients with hypertension and chronic cerebral infarction after the treatment with doxazosin mesilate. Doxazosin mesilate might be an effective antihypertensive agent for hypertensive chronic cerebral infarction.

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We describe a 71-year-old man with an inflammatory tumor arising in segment 5 of the liver. The patient was admitted because of acute pain in the right upper quadrant of the abdomen and fever. Initial laboratory tests revealed the following: serum alkaline phosphatase concentration, 634 IU/L; serum gamma glutamic transpeptidase concentration, 1,378 IU/L; serum C-reactive protein concentration, 0.89 mg/dL; and total bilirubin concentration, 8.9 mg/dL. Abdominal ultrasonography, computed tomography (CT), and magnetic resonance imaging showed a mass, 3 cm in diameter, in segment 5 of the liver. Magnetic resonance cholangiopancreatography showed a lesion of moderate-to-high signal intensity on T2-weighted images of segment 5. Endoscopic retrograde cholangiopancreatography revealed a common bile duct stone. The intrahepatic bile ducts of segment 5 could not be visualized after the use of contrast material. Endoscopic sphincterotomy was performed to remove the common bile duct stone. Antibiotics were administered soon after stone removal, and fever gradually resolved. Positron emission tomography revealed hot spots in segment 5 of the liver. Three weeks after discharge, the patient was readmitted with an acute pain in the right upper quadrant of the abdomen. Abdominal ultrasonography, CT, and magnetic resonance imaging showed enlargement of this area. Inflammatory changes of segment 5 due to cholangitis with intrahepatic bile duct stones was diagnosed. Because malignant disease could not be completely ruled out, segment 5 of the liver was resected. Macroscopic examination of the resected specimen revealed a gray, fibrotic, solid tumor associated with intrahepatic bile duct stones. Microscopic examination of the tumor showed proliferation of spindle-shaped myofibroblastic cells in a mixed myxoedematous, dense fibrotic stroma, associated with infiltration by various acute and chronic inflammatory cells. The postoperative course was uneventful, and the patient was discharged on postoperative day 16.

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Simple hepatic cysts are common benign lesions that are usually asymptomatic and require no treatment. However, complications can occur. This report describes a patient with an infected hepatic cyst treated with percutaneous transhepatic drainage. A 64-year-old woman presented at a nearby hospital because of acute right-upper-quadrant pain, mild left-lower-quadrant pain, diarrhea, and fever. She was admitted and received intravenous antibiotics for 1 week, but symptoms persisted. She was, therefore, referred to our hospital. On admission, ultrasonography demonstrated multiple hepatic cysts. One 13-cm lesion was hypoechoic, unlike the other simple cysts, which were anechoic. Computed tomography showed that the density of the hypoechoic cyst was slightly higher than that of the other cysts. The wall of the cyst was thickened and showed contrast enhancement. On initial laboratory tests the serum C-reactive protein concentration was 18.49 mg/dL, and the white-cell count was 13,300/μL. An infected hepatic cyst was suspected, and percutaneous transhepatic drainage of the cyst was performed. A catheter was inserted into the cyst, and dark red fluid was obtained. The right-upper-quadrant pain gradually resolved after drainage. An infected hepatic cyst was diagnosed, and system antibiotics were administered. However, the mild left-lower-quadrant pain persisted. No pathogens were isolated from the drainage fluid. Minocycline hydrochloride (200 mg) was injected, and the catheter was clamped for 30 minutes, once daily for 3 days. The serum C-reactive protein concentration was 1.78 mg/dL, and the white-cell count was 5,700/μL. The left-lower-quadrant pain resolved, and colonoscopic examination revealed multiple diverticula of the sigmoid colon. Infection has not recurred, and the hepatic cyst has not become larger.

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Varicella-associated purpura fulminans is a rare syndrome associated with substantial morbidity and mortality. General supportive care, heparinization, and plasma infusions are the mainstays of treatment. A patient aged 8 years and 8 months with purpura fulminans and multiple deep vein thromboses after varicella infection because of deficiencies of proteins C and S is presented in this case report.

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We present a rare case of follicular carcinoma arising from the pyramidal lobe of the thyroid in a 21-year-old woman. Radical resection of the thyroid isthmus was performed, followed by adjuvant hormonal therapy with levothyroxine. After 15 months of follow-up, the patient remains disease-free. Thyroid carcinoma in children and adolescents is rare, and also rarely arises in the pyramidal lobe. To our knowledge, this is the first report of this type of neoplasm arising from the thyroid pyramidal lobe. We are following up this case carefully, and if recurrence or metastasis or both occur, we plan to perform total thyroidectomy and ablation with ¹³¹I. This case suggests the importance of the differential diagnosis of midline cervical masses and the management of this type of neoplasm in adolescents.

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Balloon-occluded retrograde transvenous obliteration (B-RTO) has been used successfully to treat gastric varices in adults. However, only a few case reports of B-RTO in children have been published. We describe a child who had gastric varices with extrahepatic portal venous obstruction (EHO). A 12-year-old boy presented to the pediatric clinic with anemia and tarry stools. He was referred to our department to receive additional treatment for gastric varices. Endoscopy revealed spurting bleeding from gastric varices, and emergent endoscopic injection sclerotherapy was performed. Angiography showed cavernous transformation of the portal vein, hepatofugal flow of the left gastric vein, gastric varices, and gastrorenal shunt. The gastric varices were not eradicated adequately with endoscopic sclerotherapy because of excessive regurgitant blood flow against the portal venous pressure. B-RTO combined with partial splenic embolization (PSE) was therefore performed. The gastric varices were completely eradicated with no complications. This is, to our knowledge, the first report describing the use of B-RTO combined with PSE in a child with EHO who had gastric fundal varices. B-RTO combined with PSE is not excessively invasive and is effective and safe for children. This procedure is therefore recommended for the treatment of gastric varices in children.

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