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31.
The successful and persistent abolition of the inhibitor is of significant clinical benefit, as it allows for the restoration of the usual treatment with clotting factor concentrate. We describe a successful induction of immune tolerance by continuous infusion of recombinant factor VIII (rFVIII) in a 5-year-old boy with severe haemophilia A and high-responding inhibitor. He had previously been subjected to immune tolerance induction (ITI) with rFVIII at 100 units (U) kg(-1) three times weekly. One year after the beginning of therapy tolerance was not achieved and a high titer of inhibitor was detected (15 Bethesda Units). The patient had a sudden onset of severe neck pain. The diagnosis of spinal epidural haematoma was revealed by magnetic resonance imaging, and emergency laminectomy with evacuation of the haematoma was required. The patient received sequential therapy for surgery first as bolus rFVIII injection of 500 U kg(-1) in order to overwhelm the inhibitor and then as continuous infusion at 6 to 12 U kg(-1) hour(-1) to avoid bleeding episodes in the postoperative period. After the 3 weeks of continuous infusion, the inhibitor became undetectable. Thereafter, prophylactic treatment with rFVIII was started three times weekly, and the inhibitor has remained undetectable for 6 months. The, present case suggests that continuous infusion of rFVIII may be an effective therapy to induce immune tolerance. 相似文献
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NARIYOSHI YAMAGUCHI MD SABURO MATSUBARA MD FUMIO MOMONOI MD KEIICHI MORIKAWA MD MASASHI TAKEYAMA MA YOSHIKI MAEDA MD 《Psychiatry and clinical neurosciences》1998,52(2):165-167
Abstract We made an attempt to express a complaint of sleep disturbance by a self-rating scale of radar chart mode. The questionnaire for sleep disturbance is made up of eight items. Each item was scored from grade 1 to 4. The score of each item was projected to the MY radar chart, designed by us. It is noted that this method is useful in following the effect of hypnotics on sleep disturbance. 相似文献
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YOKO NOMURA HIDEKI YAGI KAZUMICHI ONIGATA SHIGENORI YUTANI TOMOHISA NAGASHIMA RYO OGAWA KANJI NAGASHIMA AKIHIRO MORIKAWA 《Pediatrics international》1995,37(6):706-709
The unusual case of a Japanese newborn XX male is presented. Examination of chromosomes in amniotic fluid cells had shown a normal female karyotype (46,XX), but ultrasonography revealed a penis and a scrotum. The neonate had normal male external genitalia, and serum levels of luteinizing hormone follicle stimulating hormone, and testosterone were all within the normal range. High resonance chromosome analysis revealed an excess portion on the short arm of one of the X chromosoms. We examined his genomic DNA by polymerase chain reaction (PCR) and detected two Y specific regions in his genomic DNA, the sex-determining region Y (SRY) and pseudoautosomal boundary Y. Nucleotide sequencing of the PCR products of SRY indicated no mutation. These findings suggested that the translocation or insertion of an SRY region on the X chromosome led to the development of testicles and a male phenotype. 相似文献
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Blanking Period Phenomenon After a Second Atrial Fibrillation Ablation Session: The Application and Factors Related to It
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YUHI FUJIMOTO M.D. MEISO HAYASHI M.D. YU‐KI IWASAKI M.D. KENJI YODOGAWA M.D. TEPPEI YAMAMOTO M.D. IPPEI TSUBOI M.D. KENTA TAKAHASHI M.D. YUKI IZUMI M.D. EIICHIRO OKA M.D. KANAKO HAGIWARA M.D. YASUSHI MIYAUCHI M.D. WATARU SHIMIZU M.D. Ph.D. 《Journal of cardiovascular electrophysiology》2017,28(2):159-166
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MASAHIRO HIRAOKA KIYOSHI MORIKAWA CHIKAHIDE HORI SHINYA TSUCHIDA KENKO KASUGA TOSHIRO TOMINAGA MASAKATSU SUDO 《Pediatrics international》1995,37(3):352-354
Dilatation of the renal pelvis has been observed as an ultrasonographic finding of ureteral reflux as well as hydronephrosis. However, little information is available on the prevalence of renal pelvis dilatation in neonates. We measured the inner pelvis dimension of the kidneys in 511 apparently healthy neonates (279 boys and 232 girls) using an ultrasound scanner to determine the prevalence of renal pelvis dilatation. Ninety per cent of the neonates had an inner dimension of both renal pelvises below 5 mm. The prevalence of left renal pelvis dilatation of 5 mm or more was significantly higher in the boys than in the girls, 25 (9%) compared to 5 (2%). In contrast, no significant difference was found in the prevalence of right renal pelvis dilatation between the sexes. In the boys, the prevalence of renal pelvis dilatation of 6 mm or more was significantly higher on the left side than on the right. Moreover, the left renal pelvis dilatation of the male neonates had a tendency to persist at 1 month of age. These findings suggest that the left renal pelvis of the baby boy may be predisposed to dilatation. 相似文献
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EUN-YOUNG JUNG TOSHIO HEIKE KENJI KATAMURA HAJIME KIMATA KATSUYUKI OHMORI YOSHIRO MORIKAWA NAOTO ISHII MITSUFUMI MAYUMI 《Pediatrics international》1997,39(4):442-447
A patient with X-linked severe combined immunodeficiency (X-SCID) was found to have a deletion mutation of a four base pair in the transmembrane domain of the IL-2 receptor γ chain gene, a subunit shared by the receptors for IL-4, IL-7, IL-9, and IL-15 (common γ chain; γc). He had very few αβT cells but had a considerable number of γδT cells in his peripheral blood. Fluorescence in situ hybridization (FISH) analysis showed that the γδT cells in his peripheral blood were not of maternal origin. He had received a Bacillus Calmette-Guerin (BCG) vaccination before recognition of the disease, and the BCG infection remained quiescent with no reaction for 19 months. After successful bone marrow transplantation, the site of the BCG vaccination showed a reaction, and live BCG were detected. It is useful to consider the relationship between the existence of γδT cells and BCG in this case, and it is suggested that γδT cells may be, in a given situation, less dependent on the γc chain than are αβT cells. 相似文献