Search

The periodic acid-thiocarbohydrazide-silver proteinate (PA-TCH-SP) technique, which has the same cytochemical significance as PAS staining of light microscopy, was undertaken to reveal the distribution of complex carbohydrates with vicinal glycols in human lymphocytes in several conditions at the ultra-structural level. The PA-TCH-SP method stained the clustered and the scattered cytoplasmic granules, Golgi apparatus and glycogen particles of lymphocytes. In the buffy coat, the lymphocytes with the clustered cytoplasmic granules contained less glycogen particles compared with the lymphocytes without the clustered cytoplasmic granules. The majority of T-lymphocytes separated from the venous blood possessed PA-TCH-SP positive clustered cytoplasmic granules, but glycogen particles were scanty or negligible, while B lymphocytes were rich with glycogen particles but had scanty clustered cytoplasmic granules. The T and B cell-derived cultured cell lines had a similar reactivity to the peripheral T and B cells.

To clarify whether in vitro-cultured basophils have features of mast cells, basophils grown in semisolid and liquid culture were studied ultrastructurally and cytochemically and were compared with freshly isolated basophils and mast cells. Ultrastructurally, the cultured basophils had pleomorphic cytoplasmic granules, some of which were similar to mast cell granules. However, scroll formation, characteristic of mast cell granules, was not observed in cells grown in semisolid and liquid culture. Cytochemically, the reactivity patterns of acidic glycoconjugates in the cultured cells more closely resembled those of the freshly isolated basophils than of the mast cells. In addition, the basophils grown in liquid culture showed a more matured form and had stronger reactivity to glycoconjugates, acid phosphatase and peroxidase than did the cells grown in semisolid culture, suggesting that the liquid culture system was better for the basophil.

A case of influenza A-associated acute encephalopathy treated with mild hypothermic therapy and methylprednisolone pulse therapy is reported. The patient was a 1 year and 1 month-old girl. Disturbance of consciousness was protracted after fever accompanied by status convulsives, thus she was diagnosed with acute encephalopathy. Influenza A reaction was positive on rapid diagnosis from her nasal mucosa. Hypothermic therapy was initiated in the early stage, and body temperature was kept at 35℃ as rectal and ear drum membrane temperatures for 72 hours. As steroid therapy, 30mg/kg/day methylprednisolone was administered for 3 days. During the course, 7mg/kg amantadine was administered for 5 days. After the combination of therapy, the patient recovered without sequela. No major adverse event occurred during the combined therapy. Still now, there are no established clinical guidelines for this encephalopathy. The combined therapy was effective for influenza A-associated encephalopathy in this patient.

Age-related changes in the monoaminergic neuron systems in the brains of methylazoxymethanol acetate (MAM)-induced micrencephalic rats were studied. Neurochemical analysis revealed high levels of serotonin, norepinephrine and associated metabolites in several brain areas of MAM-treated rats. In particular, serotonin levels in the frontal cortex, cingulate cortex and hippocampus of 12-month-old (12 M) MAM-treated rats were significantly higher than in corresponding age-matched controls. Immunohistochemical analysis demonstrated numerous aberrant serotonin-immunoreactive fibers and small numbers of aberrant tyrosine hydroxylase-immunoreactive fibers in the septum, caudate putamen, thalamus, cerebral cortex, hippocampus and midbrain tegmentum of 12M MAM-treated rats. Aberrant monoaminergic fibers characterized by swollen varicosities and thickening of intervaricose segments were common compared to 12M control rats. In the cortex and hippocampus of 12M MAM-treated rats, aberrant fibers were observed near cortical heterotopic tissue. These results indicate early onset of age-related degeneration of monoaminergic fibers in micrencephalic rats. Aged MAM-treated rats may thus offer a good model for studying age-related monoaminergic changes in the cortical heterotopic tissue of human cortical malformations.

近年,医学教育改革が目まぐるしく行われている.その中で,学生による講義評価が行われはじめているが,まだその報告は少ない.獨協医科大学では学年委員会が主体となって,講義・実習に対するアンケート調査を第1学年から第4学年まで行った.その結果,講義に対して「十分に満足している」という学生は,どの学年においても多くはなかった.さらに,「わかりやすい講義」をしている教員の共通点は,板書・プリント・シラパスを活用しているという点であった.実習に関しては,「基礎医学」に関わる実習に対しての評価が高かった.今後の医学教育において,学生の意見の反映がよりよい講義につながるものと考えられる., Extensive innovations in medical education have been carried out in recent years in our country. Although student evaluations of lectures have been conducted, there have been few reports on this issue. Thus, our committee consisting of representatives of every grade played a leading role in making and evaluating a questionnaire investigation of the lectures and the experiments. The investigation covered students from freshmen to seniors. We found that there were few students in each grade who were satisfied with the lectures. Students gave high marks to lecturers who wrote in detail on the blackboard, distributed well-organized documents, and made practical use of the syllabus. Experiments concerning the preclinical course were also well accepted. We consider that this sort of investigation will be influential in improving the quality of lectures.

獨協医科大学総合周産期母子医療センターで経験したHIVキャリア妊婦の妊娠,分娩,および出生した児の新生児期,乳児期の経過について報告し,当院におけるHIV母子感染予防対策の管理指針について検討した.本例の周産期管理では,AIDS-Clinical-Trial-Group-076のプロトコールを参考とし,妊娠27週から母体にジドブジン400 mg/dayを投与した.分娩形式は選択的帝王切開とし,Level P4の感染対策管理のもと,妊娠35週5日に施行した.出生した児に対しては臍カテーテルよりガンマグロブリン製剤200 mg/kg×1回,およびジドブジンシロップ8 mg/kg分4を6週間投与した.児は生後1歳時にPA法によるHIV抗体検査を施行して陰性であり,母子感染は予防しえた.近い将来,当院においてHIVキャリア妊婦を周産期管理する件数は増加する可能性がある.HIV患者に対する突然の事態にも対応可能な体制を維持し続けることは,栃木県内におけるHIV患者診療拠点病院である本学の重要な役割である.HIV陽性妊娠の周産期管理においては,HIVキャリア妊婦を早期に発見し,NICUをはじめ各科専門家と協力して計画的な管理を励行することが重要である., We report pregnancy and delivery in pregnant HIV carriers and their children whom we encountered at the Perinatal medical center, Dokkyo University School of Medicine. We investigated management guidelines for the prevention of maternal and child infection with HIV in our hospital. For perinatal management in the present patient, 400 mg/day of zidovudine was administered from week 27 of pregnancy in reference to the AIDS-Clinical-Trial-Group-076 protocol. Concerning the mode of delivery, selective cesarean section was performed at week 35 and day 5 of pregnancy under level- P4 infection management. A single dose of gamma globulin preparation at 200 mg/kg and 4 doses of zidovudine syrup at 8 mg/kg were administered to the neonate through an umbilical catheter for 6 weeks. In the infant, the HIV antibody test with the PA method showed a negative reaction at the age of 1 year, so maternal and child infection could be prevented. In the future, perinatal management of increasing number of pregnant HIV carriers may be performed in our hospital. To maintain the system for managing patients infected with HIV is the important role of our university, a main hospital for the treatment of patients with HIV infection in Tochigi Prefecture. In the perinatal management of HIV-positive pregnancy, pregnant HIV carriers should be detected in the early stage, and strategic management including the NICU should be performed in cooperation with specialists from other departments.

Congenital insensitivity to pain with anhidrosis (CIPA) is identified as a genetic disorder of mutations in the human TrkA known as high affinity receptor of nerve growth factor (NGF). NGF signal through TrkA promotes anti-apoptotic activity in hematopoietic cells including B lymphocytes. Here we studied the effect of NGF on anti-apoptotic activity by using human EBV-immortalized B lymphoblastoid cell lines (EB-LCLs) derived from a patient with CIPA and the associated carriers of CIPA. The TrkA(mt/mt) EB-LCL derived from the CIPA patient and the TrkA(wt/mt) EB-LCL derived from the carrier with the heterozygous TrkA mutation did not show any responses to NGF on anti-apoptotic activity. We concluded that this phenomenon is one of the pathogeneses of CIPA.

Th1細胞は造血幹細胞移植後の急性移植片対宿主病(graft-versus-hostdisease:GVHD)の発症に深く関与しているといわれている。今回我々はTh1細胞に発現するとされるキモカインリセプターの1つであるCXCR3を測定し,瞬帯血移植で急性GVHDがなぜ軽度なのかを検討した。臍帯血,成人末梢血と移植後患者末梢血中のリンパ球,NK細胞をモノクローナル抗体(抗CD4,抗CD8,抗CD56,抗HLA-DR,抗CD25)で染色し以下の結果を得た。CD^4+T細胞のCXCR3発現,HLA-DRの発現はいずれも末梢血よりも膀帯血の方が少なかった(p, In order to know why the development of acute graft-ver-sus-host-disease (aGVHD) is significantly milder after stem cell transplantation (SCT) using cord blood, we studied the CXCR3 expression on CD4^+T cells and NK (CD 56^+) cells, based on the fact that CXCR3 is one of the chemokine receptors on Th1 cells. Cord blood (CB) and peripheral blood (PB) from healthy volunTFFrs and patients who received SCT were treated with monoclonal antibodies against CD4, CD25, CD56, CXCR3, and HLA-DR and the antigen expression was analyzed using flow cytometry. The percentage of CXCR3 antigen on GB and PB CD4^+ T cells was 2.6 ± 1.4% and 30.8 ± 3.9%, respectively, demonstrating a significant difference (p < 0.05). However, that on GB and PB NK cells was not significantly different. CXCR3 expression on CB and PB CD4^+T cells stimulated by IL-2 for 72h and 120h was significantly different (p < 0.05), but that on NK cells was not significant. CXCR3 expression on CD4^+ T cells from patients who received allo-CB-SCT and allo-bone marrow transplantation from HLA-mismatched donor was significantly different at one-month post-SCT (p < 0.05). We concluded that the lower CXCR3 expression on GB CD^+T cells and absence of difference in CXCR3 expression on cord blood NK cells facilitated a milder aGVHD.

3年間に当院周産期センター新生児部門に入院し,経皮的中心静脈(PCV)カテーテルを使用した新生児は69例であった。これらの症例を対象とし,カテーテルの挿入理由,抜去理由,維持日数,合併症などについて後方視的に検討した。カテーテルの挿入理由は,超低出生体重児の輸液管理目的が最も多く,抜去理由は輸液が不要となった症例が最も多かった。カテーテルの平均維持日数は17.7日であった。カテーテルの留置中の合併症としては敗血症が4例(5.8%)であったが,カテーテル敗血症と診断したのは1例(1.4%)のみであった。その他に胸水貯留と横隔膜挙上の症例を計2例経験した。経皮的中心静脈カテーテルは,長期間にわたって留置が可能であり,合併症が少ないことから,新生児の輸液管理に非常に有用であると考えられた。, We used percutaneous central venous catheters (PCV catheters) for 69 neonates, who entered our neonatal intensive care unit for recent 3 years. We retrospectively researched the reasons of insertion and removal of PCV catheters, and complications of the catheters. The catheters were inserted mainly for the maintenance of fluid administration of extremely low birth weight infants, and were removed mainly for the end of fluid administration. The catheters were maintained for 17.7 days on the average. The sepsis occurred in 4 infants (5.8%) during indwelling of PCV catheters, however, only one infant (1.4%) was diagnosed as the catheter-related sepsis. Expect for the sepsis, two infants complicated retention of pleural fluid or elevation of diaphragm. Our results disclosed that PCV catheter caused be use for the long-term indwelling of catheter for fluid administration, and had rare frequency of complications. We conclude that PCV catheter is very useful for the fluid administration of neonates.

As part of a study to identify the characteristics of cord blood cells, we examined their morphological features by electron microscopy. Additionally, we cultured CD34-positive cells derived from cord blood and from bone marrow to perform morphological observations, as well as cytochemical examinations following the peroxidase reaction. Compared with normal peripheral blood cells, cord blood cells frequently showed immature morphology and a unique ultrastructure, such as nuclear pockets in neutrophils, several crystalloids in a single eosinophilic granule, and deformed nuclei in lymphocytes. In contrast to bone marrow cells, cord blood cells yielded a large number of cells of immature myelo-monocytic lineages in cell culture, and demonstrated a weaker peroxidase reaction. We identified that cord blood cells were different from normal peripheral blood cells and bone marrow cells, confirming the functional differences that were previously assumed.

Male siblings with intrauterine growth retardation, hydrops, mild liver dysfunction, chronic diarrhoea, failure to thrive and microcephaly are reported. In both patients, the intrauterine growth retardation was detected in the second trimester of pregnancy. Relatively severe early onset neonatal jaundice, microcytosis, anisocytosis and abnormal iron metabolism were also seen. Bone marrow examination in the second sibling showed marked ringed sideroblasts and multilobulated erythroblasts in late developmental stages. The brain was very small with enlarged cerebrospinal fluid space, a reduced number of gyri and a thin cortex. The clinical and laboratory findings in these patients appear to be unique.

During mouse embryogenesis, primitive erythropoiesis occurs in blood islands of the yolk sac (YS) on the seventh day of gestation. This study demonstrated for the first time the presence of unique primitive megakaryocytic (Mk) progenitors in the early YS, which disappeared by 13.5 days postcoitum (dpc). When 7.5 dpc YS cells were incubated in the presence of stem cell factor (SCF), interleukin (IL)-3, IL-6, erythropoietin (EPO), thrombopoietin (TPO), and granulocyte colony-stimulating factor in methylcellulose clonal culture, not only erythroid bursts but also megakaryocyte colonies were observed. The megakaryocytes in the colonies matured to proplatelet stages and produced platelets as early as day 3 of culture, much earlier than those from adult bone marrow, although their ploidy class was lower. These megakaryocytes were stained with acetylcholine esterase, and expressed platelet glycoprotein (GP)Ibβ, GPIIIa, and platelet factor 4 by reverse transcription-polymerase chain reaction analysis. The analysis of hemoglobin types in erythrocytes obtained from hematopoietic multilineage colonies containing the megakaryocytes indicated that the Mk progenitors originated from primitive hematopoiesis. The primitive Mk progenitors formed colonies in the absence of any cytokines in fetal bovine serum (FBS)-containing culture, and SCF, IL-3, EPO, and TPO significantly enhanced the Mk colony formation. In FBS-free culture, however, no colony formation was induced without these cytokines. Because megakaryocytes were detected in 8.5-dpc YS, these unique primitive Mk progenitors may rapidly mature and give rise to platelets to prevent hemorrhage in the simultaneously developing blood vessels until definitive hematopoiesis begins to produce platelets.

We describe a boy with Fanconi anemia (FA) who developed acute lymphoblastic leukemia (ALL) (FAB-LI) followed by acute myeloid leukemia (AML) (FAB-M5) at relapse. The patient was diagnosed with early pre-B-cell ALL without preceding aplastic anemia and was treated with ALL-oriented chemotherapy which included doxorubicin (a total dose of 140 mg/m2 administered), which is a topoisomerase II inhibitor. Complete remission was obtained, but after 38 weeks AML developed. The karyotype of ALL cells at diagnosis showed 46,XY, and that of AML cells at relapse was 46,XY, t(11;16)(q23;p13). An MLL gene rearrangement and MLL-CBP chimeric mRNA were found in AML, but not in ALL. A diagnosis of FA was confirmed by an increased number of chromosomal breaks and rearrangements in peripheral blood lymphocytes cultured with mitogen in the presence of mitomycin C. We conclude that this FA patient developed ALL followed by a therapy-related t(11;16)-AML resulting in an MLL-CBP fusion. Further examination of such patients would shed light on leukemogenesis in FA patients. Genes Chromosomes Cancer 27:264–269, 2000. © 2000 Wiley-Liss, Inc.

The Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder characterized by thrombocytopenia, immunodeficiency and eczema. X-linked thrombocytopenia (XLT) is a mild form of WAS with isolated thrombocytopenia. Both phenotypes are caused by mutation of the Wiskott-Aldrich syndrome protein (WASP) gene. In this study we investigated the role of WASP in the differentiation of CD34-positive (CD34+) cells isolated from the bone marrow of patients with WAS (n = 5) or with XLT (n = 4). Megakaryocyte colony formation was significantly decreased in patients with WAS when compared with normal controls. The formation of granulocyte-macrophage colonies and erythroid bursts were also decreased in WAS patinets. In contrast, in XLT patients, formation of all these colonies was normal. However, in vitro proplatelet formation of megakaryocytes induced by thrombopoietin was markedly decreased in both XLT and WAS. Electron microscopic examination revealed that megakaryocytes obtained from WAS or XLT patients grown in vitro had abnormal morphologic features, which seemed to be caused by defective actin cytoskeletal organization, including labyrinth-like structures of the demarcation membrane system and deviated distribution of the alpha-granules and demarcation membrane system. These observations indicate that WASP is involved in the proliferation and differentiation of CD34+ haemopoietic progenitor cells probably by its participation in signal transduction and in the regulation of the cytoskeleton.

Postnatal development of vimentin positive and glial fibrillary acidic protein (GFAP) positive elements in the striatum of the rat was analyzed by immunohistochemistry. Regional and chronological differences in the transformation of vimentin-positive radial glial cells were observed in the dorsal and ventral striatum. In the dorsal striatum, unwinding of the fasciculus of vimentin-positive fine fibers from the periphery toward the more central region of the striatum was observed from postnatal day (P) 3 to P7. In the ventral striatum, however, a fragmented transformation characterized by the segmental disappearance of vimentin-positive radial glial fibers was observed from P3 to P14. On the other hand, GFAP-positive astroglial cells were observed first in the dorsocaudal part of the striatum near the corpus callosum at PO. Numbers of GFAP-positive astroglial cells increased with age, and distribution of these cells extended toward the medial region of the striatum. At P14, these GFAP-positive cells were distributed throughout the striatum, including the dorsal and ventral striatum. These findings indicate that differences in transformation of radial glial cells in the ventral and dorsal striatum may be attributed to differences in neurogenesis and formation of neuronal circuits in the dorsal and ventral striatum. These results provide new insight into the role of radial glial cells in the developing striatum.

Translocation t(6;9)(p23;q34), resulting in a dek-can gene fusion, is a recurrent chromosomal abnormality mainly associated with specific subtypes of acute myeloid leukaemia (AML) and myelodysplastic syndrome (MDS). Patients with this type of chromosomal change are usually young and their prognosis is poor. The role of fusion protein generated from dek-can chimaeric transcript on the leukaemogenesis of t(6;9) AML or MDS is as yet unknown. We have established the first permanent cell line (FKH-1) with t(6;9), derived from the peripheral blood of a patient with t(6;9) AML transformed from Philadelphia chromosome (Ph1)-negative chronic myelocytic leukaemia (CML). The FKH-1 expressed myelomonocytic markers and dek-can chimaeric transcript. In the presence of 10 ng/ml recombinant human granulocyte colony-stimulating factor (G-CSF), the cells doubled every 54 h and showed multilineage myeloid differentiation, resulting in heterogenous morphologies such as macrophages, basophils, eosinophils and neutrophils. Thus, this cell line may be derived from a pluripotent myeloid stem cell and should be a useful tool for biomolecular studies on the pathogenesis of t(6;9) myeloid malignancies which have rarely been investigated because of the lack of continuously proliferating cells.

We describe a 27-month-old boy who was first admitted to our hospital on 7 January 1995 with nasal bleeding. From 6 months of age he has had lymphadenopathy, low levels of serum uric acid, increased levels of serum lactate dehydrogenase and hyper gamma-globulinemia. From the age of 18 months he has had persistent Epstein-Barr virus (EBV) infection (target cells; B cells), recurrent episodes of thrombocytopenia, anemia and hepatosplenomegaly. Dysmobility of the left leg and arm from a central nervous system complication during a relapse with pancytopenia on March 1995, was also observed. Relapses of thrombocytopenia with increases of platelet-associated immunoglobulin G and hepatosplenomegaly have been observed approximately every 2 months, and two relapses of pancytopenia were accompanied with weak positivity of Coombs test and low level of haptoglobin. These recurrent episodes were improved with prednisolone. However, now in June 1997 we have not been able to diagnose what underlies the above clinical symptoms, except that the patient has a persistent EBV infection.

To examine the morphological changes of HL-60 cells undergoing apoptosis, we induced apoptosis by etoposide treatment, and compared the findings obtained with an electron microscopic cytochemical method with those obtained with in situ end-labeling (ISEL) of DNA and a DNA fragmentation assay. Apoptotic cells were detected by ISEL of DNA 1h before the ladder formation in the DNA fragmetation assay, and an accumulation of DNA was observed simultaneously around the nucleoli and nuclear membrane by DNA-staining electron microscopy. These findings revealed that the apoptosis-related changes occurred in the DNA before the appearance of ladder formation. DNA staining with osmium ammine B revealed, at higher magnification, a rough fiber formation of DNA before apoptosis and an accumulation of small particles with the course of apoptosis. The number of primary granules stained for peroxidase in the cytoplasm decreased as apoptosis progressed. An examination by the periodic acid-thiocarbohydrazide-silver proteinate (PA-TCH-SP) method revealed in number after the induction of apoptosis. Thus, apoptosis-related changes were observed not only in the nucleus but also in the cytoplasm.

Mast cells were cultured from human umbilical cord blood CD34-positive cells in the presence of interleukin (IL)-6 and stem cell factor (SCF). Electron microscopically, a particulate or beaded structure, the prominent structure of tryptase-positive mast cell (T mast cell) granules, was frequently observed in 54-day cultures. Straight or coiled lamellae, often observed in the granules of tryptase-and chymase-positive mast cells (TC mast cells), were observed in the granules in 75-day cultures. Cytochemically, the peroxidase activity of the granules was positive but decreased during the term of culture. Glycogen particles were abundant in 54-day cultures, and scarce in 75-day cultures. These findings suggest that the mast cells matured and changed in phenotype during the culture's term.

A 1 year and 11 month-old boy developed convulsions and disturbance of consciousness after an antipyretic was administered. Influenza type A virus reaction was positive on rapid antigen test of nasal discharge. Although no significant abnormality was found by brain CT on admission, abnormal densities were observed in the bilateral thalami, brain stem and tegmentum on brain CT performed 12 hours later, so the patient was diagnosed with acute necrotizing encephalopathy (ANE) of childhood due to influenza type A virus. In spite of we diagnosed and performed methylprednisolone pulse and antithrombin III therapy in the early stage of this encephalopathy, which resulted in serious neurologic sequelae remaining. The clinical course of our case suggests that the early diagnosis and these treatments for ANE may not be effective. At present, we have no established therapy for this encephalopathy, so the vaccination is only a method for prevention of ANE due to influenza type A virus.

We present here a long survival case of a patient with the mosaic form of trisomy 13 who died of aspiration pneumonia at the age of 7 years and 4 months. The autopsy revealed olfactory aplasia and fenestration of the septum pellucidum, and dilated lateral ventricles and atrophic hippocampus. Furthermore, there were numerous “torpedos” (i.e., swollen fusiform Purkinje cell axons), mostly in the granular layer underneath the Purkinje cell layer, and, occasionally, in the granular layer. Similar neuropathological findings have been reported in elderly cases of essential tremor, Parkinson’s disease, or Alzheimer’s disease. Precise mechanism for this axonal change is still unclear. These pathological changes have never previously been reported in the literature on trisomy 13, and the present patient is one of the oldest autopsied individuals with the mosaic trisomy 13.

We describe a case of a 15 year old boy who developed acute megakaryoblastic leukemia (AMKL) while receiving treatment with human growth hormone (hGH) for idiopathic growth hormone deficiency (GHD). He was diagnosed as having idiopathic GHD and given hGH from December 1991. The examination of his peripheral blood showed mild pancytopenia 2 months before the start of the hGH therapy. Since January 1992, paleness of the skin, general fatigue and fervescence progressed gradually. In February 1992, because of the occurrence of acute leukemia, administration of hGH was discontinued. Judging from the results of surface marker analysis of the blast cells, the patient was diagnosed as having AMKL. He was treated with chemotherapy for acute non-lymphoblastic leukemia from March 1992. A complete remission was obtained after 4 weeks of treatment. The chemotherapy was completed in July 1993. He remains in complete remission 26 months after diagnosis. This case suggests the importance of hematological examination and, when there is any abnormality which is not caused by GHD, such as pancytopenia, more detailed medical examinations (for example bone marrow examination) are necessary.

A 2 year old girl was diagnosed as having erythroleukemia (EL; M6 according to the French-American-British classification). After one course of low-dose cytosine arabinoside (Ara-C), complete remission was obtained. After three courses of low-dose Ara-C for consolidation, allogeneic bone marrow transplantation was performed from HLA-identical sibling. The course of post-transplantation was uneventful. Two years after transplantation, she continues to have durable engraftment and remission. In children with EL, conventional chemotherapy appears to be inadequate for producing durable long-term disease-free survival. Bone marrow transplantation should be considered in children with EL, in cases where suitable donors are available.

血清中の抗サイトメガロウイルス(CMV)-IgM,抗CMV-IgG抗体陽性の所見と臨床症状より診断したCMV感染乳児の末梢血単核球のCD8, HLA-DR, IL 2 Rβ, CD 45 RO, CD 45 RA, CD 7の発現をflow cytometry法にて検討した. CD8+細胞比率の増加, CD 4+細胞比率の減少により, CD 4+細胞/CD 8+細胞比は0.80±0.37 (対照: 2,74±1.80)と低下した. CD 8+細胞数は4,762±1,162/μl (対照: 1,414±127/μl)と増加していたが, CD 4+細胞数は3,250±289/μl (対照: 3,519±322/μl)と増減は認めなかった. CD 8+細胞に対するHLA-DR+CD 8+細胞比率は0.65±0.15 (対照: 0.18±0.09), IL 2 Rβ+ CD 8+細胞比率は0.33±0.10 (対照: 0.11±0.10), CD 45 RO+ CD 8+細胞比率は0.40±0.12 (対照: 0.18±0.02)と高値であった. EBウイルスによる伝染性単核症児と比較し, CMV感染乳児ではCD 45 RO+細胞比率の増加が軽度で, CD7の発現は対照との間に差がみられなかった.

Summary. We report X-linked thrombocytopenia (XLT) in a 6-year-old girl with petechiae and thrombocytopenia from the age of 3 months. Her 2-year-old brother was also diagnosed with XLT. The Wiskott–Aldrich syndrome protein (WASP) gene was detected as a replacement of +5th G to Aon intron 6 using sequence analysis, and the WASP expression levels in this patient were one-third those of a healthy control. The X-inactivation analysis of the patients lymphocytes showed a random pattern of X-chromosome inactivation. To our knowledge, this is the first confirmed report of XLT in a female.

We report a 7-year-old boy with congenital bilateral perisylvian syndrome and congenital constriction band syndrome. The former is a congenital neurologic syndrome characterized by pseudobulbar palsy, mental retardation, epilepsy, and bilateral perisylvian polymicrogyria. The latter is a malformative disorder with digital ring constrictions and amputations, probably caused by early amnion rupture resulting in entanglement of fetal parts by amniotic strands. We believe that the combination of these two malformative disorders was not coincidental; instead, fetal circulatory disturbance related to chronic abruptio placentae could account for this combination. (J Child Neurol 2002;17:448-450).

Large gastric folds in adults are seen in many benign and malignant conditions, but they are rare in children with malignant diseases such as non-Hodgkin lymphoma. The authors report a patient with non-Hodgkin lymphoma who had large gastric folds and jaundice as the initial symptoms. A 14-year-old boy was referred to the authors' hospital with upper abdominal pain and jaundice. A standard barium upper gastrointestinal series showed large gastric folds in the entire stomach. Magnetic resonance imaging showed a typical diffuse infiltrating type of pancreatic lymphoma. Because complete bilateral lower limb paralysis developed as a result of the epidural soft tissue mass, laminectomy and tumor resection were performed and a diagnosis of disseminated Burkitt lymphoma was established. After completing 6 months of chemotherapy, the patient has been disease-free without neurologic complications for 2.5 years.

A 7-year analysis of pediatric asthma in Tochigi was performed with the aim of improvement of future treatment.Self-administered questionnaires were completed by pediatric asthma patients and their parents who visited pediatric clinics or hospitals in Tochigi at the same period of year in 2002, 2006, and 2008. Changes in asthma symptoms and in the QOL of the patients and their parents were analyzed. Data on the background and treatment of the patients were obtained from doctors.The questionnaires were answered by 1487, 1058, and 1014 patients or parents in 2002, 2006, and 2008, respectively. The results showed significant increases in the use of ICS and LTRA (p0.001, for each.) and significant decreases in the use of theophylline, DSCG, and LABA (p0.001, for each.). Frequency of wheezing, cough and sputum were significantly reduced in all age groups (p0.01, for each.). There were significant reductions of frequency of wheezing in patients under 2 years old; in wheezing and cough in patients aged 2-5 years old; and in wheezing, cough and sputum in patients aged 6-15 years old (p0.05 for each.). The QOL of the patients and their parents was significantly improved in all age groups (p0.05 for each.).These findings suggest that increased use of anti-inflammatory agents such as LTRA and ICS have contributed to a reduction in asthma symptoms and an improvement in the QOL of the pediatric patients and their parents, but more widespread use of these agents are still needed for residual uncontrolled asthma patients.

Human granulocyte colony stimulating factor (G-CSF) and macrophage colony stimulating factor (M-CSF) were administered intravenously to rats, and their effects on neutrophils and monocytes were examined by electron microscopy. G-CSF increased the number of cytoplasmic granules in neutrophils. It also enhanced maturation of the nuclear shape in the neutrophils, while chromatin condensation and peroxidase distribution remained immature. M-CSF induced proliferation of monocytes in peripheral blood and bone marrow, but did not affect morphology or distribution of peroxidase reactivity.

The localization of complex carbohydrates in 41 patients with acute megakaryoblastic leukemia (AMKL) was studied using the periodic acid-thiocarbohydrazide-silver proteinate (PA-TCH-SP) method. Examination by the PA-TCH-SP method revealed that the megakaryoblasts from AMKL had a thick cell coat, a small amount of glycogen, granules and well-developed GERL ofNovikoff. Multivariate analysis revealed no cytochemical differences in the distribution of glycoconjugates in the cytoplasm between AMKL blasts in children and adults, but they were noted between all AMKL blasts and blasts from patients with chronic myelocytic leukemia in megakaryoblastic crisis (CML-MK). The ultrastructural distribution of complex carbohydrates in the blasts from AMKL was extremely different from the acute myelocytic leukemia (AML) blasts as demonstrated by the analysis.

The ultrastructural localization of RNA in myeloma cells was studied by the RNase-gold method. Gold particles indicating the presence of RNA were observed in large numbers, particularly in the granular component of the nucleolus and periphery of the rough endoplasmic reticulum, but not in the Golgi area, mitochondria, intranuclear inclusion bodies, cytoplasmic inclusion bodies, dense bodies, or cisternae of the rough endoplasmic reticulum. In the nuclear chromatin and nucleolus, gold particles were more numerous as these structures were less mature. They were found in larger numbers also in the cytoplasm of immature cells. In plasma cells from patients with macroglobulinemia, gold particles were fewer than in myeloma cells of multiple myeloma, but there was no difference in their distribution pattern.

末梢血単核球をCD 3に属するマウスモノクローナル抗体(VIT 3b: IgG 1クラス)で刺激時の増殖能へのIFN-γの影響を3H-thymidineの取込みにより検討した. VIT 3 bによる増殖能は症例ごとに相違した. VIT 3 bによる増殖はIFN-γにより増強された.なお, IFN-γの増強(ヘルパー)効果はVIT 3 bによる増殖能の十分でない症例により強くみられた.しかし, IFN-γのヘルパー効果は最大でおよそ300%であった. VIT 3 bのみの反応は単球の濃度に大きく影響されたが, IFN-γのヘルパー効果は単球が5%の低濃度でもみられた.患者でのIFN-γのヘルパー効果は対照より劣っていた.