Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty-nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.     

Vascular Endothelial Growth Factor–Bound Stents: Application of In Situ Capture Technology of Circulating Endothelial Progenitor Cells in Porcine Coronary Model

Objectives

We evaluated the in vivo performance of a newly devised vascular endothelial growth factor (VEGF)‐bound stent in a porcine coronary model.

Background

An anti‐CD34 antibody‐bound stent, which captures endothelial progenitor cells (EPCs) to accelerate tissue formation, did not reduce intimal hyperplasia. By targeting the VEGF receptor, which is expressed on endothelial‐lineage cells, we developed VEGF‐bound stents that may enable selective capture of EPCs followed by rapid endothelialization.

Methods

Metallic stents were first coated with poly‐(ethylene‐co‐vinyl alcohol), and then chemically bound with either VEGF or anti‐CD34 antibody. These stents were placed in porcine coronary arteries for up to 14 days. Stent surface was evaluated by immunohistochemistry and by scanning electron microscope (SEM).

Results

After 2‐day stenting with VEGF‐bound stents, small populations of KDR (VEGF receptor‐2)‐positive cells adhered to the stent struts. After 7‐ and 14‐day stenting, struts were fully covered with newly regenerated tissue. SEM images showed that the uniform tissue formed on struts was morphologically similar to native endothelium and was continuously connected with adjacent native endothelium. On the other hand, for the anti‐CD34 antibody‐bound stents, stent struts were rapidly covered by newly generated tissue that consisted of multicellular aggregates.

Conclusions

Compared with anti‐CD34 antibody‐bound stents, VEGF‐bound stents provide highly selective capture of EPCs, followed by rapid formation of intact endothelium tissue at an early period of stenting. These results suggest that VEGF‐bound stents could represent a promising therapeutic option for cardiovascular stenting, although further long‐term follow‐up experiment with double‐blinded fashion is needed prior to clinical application. (J Interven Cardiol 2014;27:63–72)

     

Fatal cytomegalovirus myocarditis in a seronegative ALL patient

Fatal cytomegalovirus (CMV) myocarditis occurred in a 2 year old boy with acute lymphoblastic leukemia (ALL) in remission. The patient showed mild hepatic dysfunction and a rapid progress of pancytopenia after complete remission had been achieved. At the fifth week of complete remission, he presented signs of heart failure such as tachycardia, S4 gallop on auscultation and decreased ejection fraction on echocardiography. However, no significant electrocardiographic changes were recognized. In addition to the cardiac dysfunction, the patient presented a marked tachypnea and dyspnea associated with hypoxemia. These were dramatically improved by methylprednisolone pulse therapy (30 mg/kg per day, for 3 days) and CMV high titer immunoglobulin (400 mg/kg per day, for 3 days). On the sixth day after signs of respiratory failure were improved, the patient suddenly presented a paroxysmal atrial tachycardia followed by a fatal ventricular fibrillation. Although we could detect neither a specific IgM antibody, a significant increase of IgG antibody, nor CMV genome by DNA hybridization techniques during the course of the illness, microscopic examination of necropsy specimens of the heart showed a marked disruption and disintegration of muscle bands associated with cytomegalic inclusion bodies. Polymerase chain reaction (PCR) yielded a 305 bp amplification product in the heart and lung tissues, supporting the view that myocarditis was caused by CMV.     

Expression of the Plasmodium berghei ookinete protein Pbs21 in a baculovirus-insect cell system produces an efficient transmission blocking immunogen

A surface protein of Plasmodium berghei ookinetes, Pbs21, was expressed in a baculovirus-insect cell system in cell culture and in Heliothis virescens larvae. Groups of BALB/c mice received two intraperitoneal inoculations of either i) Tris-buffer or homogenized H. virescens larvae infected with wild-type baculovirus; ii) enriched, homogenized ookinetes, or Hi) homogenizedH. virescens larvae expressing recombinant Pbs21 (rPbs21). All animals immunized with ookinetes or with rPbs21 had high litres of antibodies (IgG isotype) that bound to native Pbs21. The large majority of antibodies in immune sera of both groups recognized the antigen under non-reducing but not under reducing conditions. The predominant IgG-sub-classes in mice immunized with ookinetes was IgGl and in mice immunized with rPbs21, the subclasses were IgGl and IgG2a. Immunization with rPbs21 reduced the infec-tivity of P. berghei to mosquitoes by 91% compared to a 99% reduction following immunization with ookinetes. This preliminary data indicate that rPbs21 expressed in this eukaryotic system induces a transmission-blocking immunity which is more effective than that achieved using rPbsll expressed in Escherichia coli (Matsuoka et al. 1994).     

Cystosarcoma phyllodes of the seminal vesicle

BACKGROUND: We report here an extremely rare case of cystosarcoma phyllodes of the seminal vesicle. METHODS: A 65-year-old man presented with urinary hesitancy, frequency and constipation. Clinical examinations including two needle biopsies were performed, and the patient had undergone open surgery. RESULTS: The final pathological diagnosis was cystosarcoma phyllodes of the seminal vesicle. Seven months after the operation, a chest X-ray showed lung metastasis, and the patient died 11 months after the operation. CONCLUSION: To our knowledge, only one case of cystosarcoma phyllodes of the seminal vesicle has been previously reported.     

Risk of Lung Cancer by Histologic Type among Smokers in Miyagi Prefecture

Smoking habits of the 827 lung cancer patients aged 40 yearsor over who were treated in the Sendai Kosei Hospital from 1977–82were compared with those in the general population of a non-metropolitandistrict in northern Japan, which we recently surveyed by mailquestionnaire. The relative risks of lung cancer by histologictype adjusted by age and area of residence were as follows:1.9 for adenocarcinoma, 4.3 for squamous cell carcinoma, 3.9for small cell carcinoma and 3.4 for large cell carcinoma inmen, and 2.9, 6.4, 4.5 and 4.0 for these histologic types, respectively,in women. These elevated risks were all statistically significant(p < 0.05). These findings suggest that smoking habits shouldbe carefully considered when studying the etiology of any celltype of lung cancer, even adenocarcinoma, which often has beenthought to be unrelated to smoking habits.     

Successful Radiofrequency Current Catheter Ablation of Sustained Ventricular Tachycardia

We performed radiofrequency current catheter ablation in two patients with nonischemic sustained ventricular tachycardia (VT). In one patient, two morphologically distinct VTs were induced by electrical stimulation. One showed right bundle branch block pattern and the other left bundle branch block pattern. The earliest site of activation during each VT was determined at the septum of the right ventricle. However, these two sites were close to the His-bundle elecfrogram recording area. In the other patient, a VT with a left bundle branch block pattern occurred spontaneously after the administration of isoproterenol. The earliest site of activation during VT was determined at the outflow tract of the right ventricle. During tachycardia, radiofrequency current ablation (40 W ± 30 sec) was delivered to the earliest site of activation, A few seconds after fulguration, each VT was terminated and additional radio-frequency currents were given near these sites. After the ablation, VT could not be induced by the electrical stimulations, nor did it recur. No side effects were observed and the atrioventricular conduction remained intact. We feel that nonischemic VTs could possibly be treated by using radiofrequency current catheter ablation.     

The consistent presence of the human accessory deep peroneal nerve

Twenty-four human legs were dissected macroscopically to study the morphological details of the accessory deep peroneal nerve. This nerve arose from the superficial peroneal nerve and descended in the lateral compartment of the leg, deep to peroneus longus along the posterior border of peroneus brevis. Approaching the ankle joint, this nerve passed through the peroneal tunnels to wind around the lateral malleolus; it then crossed beneath the peroneus brevis tendon anteriorly to reach the dorsum of the foot. The accessory deep peroneal nerve was found in every case examined and constantly gave off muscular branches to peroneus brevis and sensory branches to the ankle region. In addition, this nerve occasionally had muscular branches to peroneus longus and extensor digitorum brevis, and sensory branches to the fibula and the foot. The anomalous muscles around the lateral malleolus were also innervated by this nerve. Neither cutaneous branches nor communicating branches with other nerves were found. The present study reveals that the accessory deep peroneal nerve is consistently present and possesses a proper motor and sensory distribution in the lateral region of the leg and ankle. It is not an anomalous nerve as has previously been suggested.