bluuebunny/crossref_metadata_2025 huggingface dataset:
Concentration of Lipid Peroxide in Serum Lipoproteins of Insulin‐Dependent Diabetic Children
Authors: Asayama Kohtaro , Miyao Akiyo , Dobashi Kazushige , Amemiya Shin , Ishihara Toshihide , Kato Kiyohiko
Published: October 2007

Lipids, apolipoproteins and lipid peroxide were measured in the sera of 29 children with insulin‐dependent diabetes mellitus. Ten non‐diabetic children served as controls. High‐density lipoprotein (HDL) was separated by heparin‐MnCl2 precipitation. Lipid peroxides in HDL and non‐HDL fractions were estimated by fluorimetric measurement of thiobarbituric acid‐reactive substances. The patients were normolipidemic, and their HDL‐cholesterol was increased. Apo A1 level in the patients was smilar to that in the controls, while levels of apo A2 and apo B were decreased in the patients. Concentrations of lipid peroxides in the whole serum and non‐HDL were unaltered, while that in the HDL was higher in the patients than in the controls. Hemoglobin AIc in the patients correlated with the triglyceride and urinary excretion rate of N‐acetylglucosaminidase (NAG). The NAG correlated with the triglycerides. The triglycerides correlated with the atherogenic index, apo B and total cholesterol. The lipid peroxides in the non HDL correlated with the triglyceride, atherogenic index, and NAG. That in the HDL correlated with the HDL‐cholesterol, apo A1 and endogenous creatinine clearance, and inversely with the atherogenic index and apo B. Lipid peroxides in HDL and non‐HDL appeared to play different physiological roles from each other, and they have provided evidence suggesting that diabetic microvascular injury is mediated by reactive oxygen species.
Angiotensin‐Induced Hypertension Chemotherapy in Children with Advanced Solid Tumors
Authors: Fujii Yuji , Hongo Teruaki , Masui Hiroyuki , Chiba Takako , Furukawa Nobuko , Nakajima Hiroaki , Nasuda Kaoru , Yajima Shuhei , Horikoshi Yasuo , Igarashi Yoshio
Published: October 2007

Angiotensin‐induced hypertension chemotherapy (IHC) was investigated in six children with the following advanced malignancies: hepatocellular carcinoma, extraskeletal Ewing's sarcoma, sacrococcygeal malignant teratoma, small round cell tumor of the chest wall, hepatoblastoma and osteogenic sarcoma. Partial response was achieved in three of these patients, two showed no change, and in one IHC was used as adjuvant chemotherapy. The side effects of IHC were minimal and tolerable. Angiotensin‐IHC may provide a new approach to pediatric cancer chemotherapy.
Immunoblastic Lymphadenopathy (IBL)‐Like T‐Cell Lymphoma in a Child
Authors: Nakazono Shin‐ichi , Kitahara Takuma , Takezaki Toshiroh , Kawakami Kiyoshi , Hasui Kazuhisa , Satoh Eiichi , Tokunaga Masayoshi
Published: October 2007

We report the case of a 14‐year‐old Japanese boy with peripheral T‐cell malignant lymphoma, showing progression from immunoblastic lymphadenopathy (IBL) to overt malignant lymphoma. He suffered recurrent fever, generalized lymphadenopathy, hepatosplenomegaly and maculopapular exanthema. Leukocytosis with eosinophilia and polyclonal hypergammaglobulinemia were observed during the aggressive course of the disease. In the early phase, human immunoglobulin and steroids improved the symptoms but did not induce complete remission, and the patient died one year after the onset of the illness. Four biopsies of lymph nodes revealed progression from IBL to CD4 positive T‐cell lymphoma through IBL‐like T‐cell lymphoma. Though IBL‐like T‐cell lymphoma is defined as IBL with neoplastic features and overt T‐cell malignant lymphoma progressed from IBL‐like T‐cell lymphoma is excluded from the definition, it may be preferable that such malignant lymphoma as our case should also be included in IBL‐like T‐cell lymphoma.
Forty Year Review of Bacterial Endocarditis in Infants and Children
Authors: Saitoh Makiko , Hishi Toshio , Tamura Masanori , Komoshita Shigehiko
Published: October 2007

From 1950 through 1989 at Tokyo University's Department of Pediatrics, 25 children were treated for bacterial endocarditis. Twenty‐two of these patients had previously been diagnosed as suffering from congenital heart disease. Twenty one of the 25 patients had positive blood cultures. Streptococcus viridans was the most common organism isolated, although the incidence of Streptococcus viridans infection has decreased in the last 20 years. In present series only a few post‐surgical cases with congenital heart disease occurred. There were three deaths which all occurred prior to 1965.
Diagnosis of Atlantoaxial Subluxation in Morquio's Syndrome and Spondyloepiphyseal Dysplasia Congenita
Authors: Takeda Eiji , Hashimoto Toshiaki , Tayama Masanobu , Miyazaki Masahito , Shirakawa Etsuhisa , Shiino Yoshiro , Saijo Takahiko , Ito Michinori , Naito Etsuo , Huq A.H.M. Mahbubul , Kuroda Yasuhiro
Published: October 2007

Compression of the spinal cord due to atlantoaxial subluxation was diagnosed in a patient with Morquio's syndrome and in another with spondyloepiphyseal dysplasia (SED) congenita by cervical radiography and magnetic resonance imaging (MRI). The patient with Morquio's syndrome, a 15 year old boy, had no neurologic symptoms and his somatosensory evoked potential (SSEP) was normal. However, MRI demonstrated spinal cord compression at C1‐C2. In contrast, the patient with SED congenita, an 11 year old girl, had neck pain, hyperreflexia and loss of vibration sense in both legs. These findings were explained by the absence of P3 and later waves in SSEP and by compression of the spinal cord observed on MRI. Both SSEP and MRI should be used for evaluating disorders in which atlantoaxial subluxation might be present.
Transcutaneous Bilirubinometry: Variability of TcB Measurements on the Forehead with Crying
Authors: Yamauchi Yoshitada , Yamanouchi Itsuro
Published: October 2007

We studied the effect of crying and facial expression on TcB readings at the forehead in 38 high‐risk full‐term newborn infants. Our study showed that crying and facial expression were significant factors affecting the TcB readings at the forehead. We recommend that TcB readings at the forehead should be obtained in a quiet condition.
Successful Dantrolene Sodium Treatment of a Patient with Myotonia Congenita (Thomsen's Disease)
Authors: Ohtaki Etsuo , Komori Hironori , Yamaguchi Yoichiro , Matsuishi Toyojiro
Published: October 2007

A 3 year, 10 month old male with myotonia congenita (Thomsen's disease) was placed on dantrolene sodium, which successfully abolished the myotonic phenomenon. Dantrolene sodium might therefore be a useful medicine for patients with Thomsen's disease who do not respond to other medicines, such as carbamazepine (CBZ), with a decrease in the myotonic phenomenon.
Williams Syndrome in One Dizygotic Twin
Authors: Hokama Tomiko , M.B.B.S John G. Rogers
Published: October 2007

Williams syndrome is a disorder of unknown etiology with a characteristic facial appearance, vascular disease and infantile hypercalcemia. Most cases are sporadic. We report the case of a dizygotic male twin with the Williams syndrome. This appears to be the first report of twins with only one affected. This suggests a mutational event as the cause of Williams syndrome.
Coronary Arterial Calcification in Kawasaki Disease
Authors: Nakada Toshimasa , Yonesaka Susumu , Sunagawa Yoshiaki , Tomimoto Kazuhiko , Takahashi Toru , Matsubara Toru , Furukawa Hidetsugu , Kamimura Kenzou , Naka Shinichi
Published: October 2007

The purpose of this paper is to describe the clinical characteristics and significance of the coronary arterial calcification due to Kawasaki disease (KD), based on our five patients. The coronary arterial lesions (CAL) were in segments 1, 2, 5, 6 and 11 according to the classification of the American Heart Association. The interval from the onset to the first appearance of coronary calcification was 17 months to 61 months. Progression of CAL into coronary arterial stenosis or myocardial infarction occurred in all five patients. In the comparative study between the patients with calcified CAL and those with non‐calcified CAL, the maximal size of CAL of the former was 8 mm or larger in contrast with that of the latter (less than 8 mm). It is concluded that coronary arterial calcification is one of the important signs which suggest the presence of the advanced CAL in the follow‐up of children with a history of KD.
Efficacy of Once Weekly Erythropoietin Therapy in Children on Continuous Ambulatory Peritoneal Dialysis
Authors: Hisano Satoshi , Kaku Yoshitsugu , Ueda Kohji , Onoyama Kaoru
Published: October 2007

The efficacy of recombinant human erythropoietin (rHuEPO) on 10 anemic children undergoing continuous ambulatory peritoneal dialysis (CAPD) was evaluated. A mean dose of 93 U/kg of rHuEPO was given once a week for 24 weeks, either intravenously to 5 children or subcutaneously to the other 5. Anemia improved in all children by this therapy. The mean hemoglobin and hematocrit levels increased gradually but significantly from 6.9 g/dl and 20.2% to 9.4 g/dl and 30.1%, respectively, at the end of the study. Elevation of blood pressure or other side effects were not noted in any patient. The present study indicates that the once‐a‐week injection of rHuEPO by the intravenous or subcutaneous route is effective in children undergoing CAPD.
Mitral Valve Prolapse in Childhood: The Incidence and Clinical Presentations in Different Age Groups
Authors: Ohara Nobutoshi , Mikajima Takatoshi , Takagi Junichi , Kato Hirohisa
Published: October 2007

To elucidate the incidence and natural history of mitral valve prolapse (MVP) during childhood, we investigated a total of 4,238 children (aged from 1 day to 15 years) classified by age into 4 groups: Group 1:1 to 28‐day‐old full‐term normal newborns (n = 108), Group 2: 6 to 18‐month‐old infants (n = 391), Group 3: 6 to 7‐year‐old children (n = 2,801), and Group 4: 12 to 15‐year‐old children (n = 938). The incidence of MVP was determined by videorecorded two‐dimensional echocardiography in a double‐blind method twice‐over. There were 109 cases diagnosed as having MVP. The incidence rates of MVP were as follows: Group 1: 0%, Group 2: 0.25%, Group 3: 2.1% and Group 4: 5.1%. Arrhythmias were detected in 49% (27/55) by Holter ECG, and by exercise stress test in 4.7% (2/43). Eighty‐three (77%) of 108 cases in Groups 3 and 4, excluding the 1 case in Group 2, showed no symptoms. Ventricular premature contraction (VPC) was the most common arrhythmia, and was benign in all cases. A mid‐systolic click (MSC), late systolic murmur (LSM), MSC + LSM, and a pansystolic murmur were detected in 23.1%, 3.7%, 4.6% and 5.6%, respectively. Symptoms caused by MVP increased and appeared more apparently with age. Further prospective long‐term follow‐up studies to adulthood are necessary.
Summer‐type Hypersensitivity Pneumonitis in a Child
Authors: Lyori Hideaki , Kawamura Ken‐ichi , Seo Kiwamu
Published: October 2007

Summer‐type hypersensitivity pneumonitis (HP) is a unique disease in Japan. The clinical features of this disease are as follows: 1) cough, fever and dyspnea as a clinical triad, 2) diffuse reticulonodular opacities on the chest X‐ray film, 3) restrictive impairment and decrease in DLco, 4) hypoxia, 5) initiation in summer, 6) worsening of the condition when the patient returns home, 7) granuloma formation and alveolitis in the lung biopsy specimen, 8) familial clustering. The etiologic agent of this disease is debatable. In 1984 Ando et al reported that the etiologic agent was T. cutaneum. Now many people are pursuing the argument to its logical conclusion. We report a case of summer‐type HP. It is uncommon in children, especially in a child in whose serum antibody to T. cutaneum can be demonstrated.
Immunological Classification of Childhood Acute Lymphoblastic Leukemia
Authors: Nakazawa Shinpei , Saito Midori , Okazaki Toshiko , Takane Keiko , Sugita Kanji , Mori Taijiro , Nishino Kazuyoshi , Suzuki Toshio , Kinoshita Akitoshi , Abe Takashi , Kurosawa Yoshihiro , Inukai Takeshi
Published: October 2007

Seven hundred and forty‐four newly diagnosed patients with acute leukemias between 1978 and 1990 were classified on the basis of immunological phenotypes. The majority of the patients were enrolled in the Tokyo Children's Cancer Study Group (TCCSG) studies. The incidence of subclassification of acute leukemias in this study was as follows: 522 patients with ALL (70%), 139 patients with ANLL (18%), 29 patients with biphenotypic leukemia, 8 patients with Ph1‐positive acute leukemia (Ph?‐AL), and 45 patients with infant leukemia. ALLs were classified into common ALL (cALL, 77%), T‐ALL (15%), B‐ALL (4%), and unclassified ALL (3%). The incidence of ALL subtypes in this study reflected those of TCCSG. Biphenotypic leukemias were categorized into 4 groups as follows; 1) cALL with positive myelomonocytic antigen(s) (N = 11), 2) unclassified ALL with positive myelomonocytic antigen(s) (N = 5), 3) ANLL with positive B‐lymphoid antigen(s) (N = 4), and 4) acute leukemia with positive T‐lymphoid and myeloid antigen(s). Infant leukemias were classified into ALL type (N = 27) and ANLL type (N = 18). In this present study, clinical features and immunological phenotypes of the acute leukemias with a poor prognosis, i.e. biphenotypic leukemia, Ph?‐AL, and infant leukemia are analyzed and discussed.
Improved Prognosis of Acute Nonlymphocytic Leukemia in Children: Results of the 12th‐ANLL Protocol of Tokyo Children's Cancer Study Group
Authors: Bessho Fumio , Kigasawa Hisato , Tsuchida Masahiro , Tsukimoto Ichiroh , Nakazawa Shinpei , Yamamoto Masao , Tsunematsu Yukiko , Yamada Kohichiroh , Sugita Ken‐ichi , Ohkawa Yohji , Kaneko Takashi , Kaku Hidehumi , Inana Ichiroh , Wada Emiko , Kaneko Masahumi , Itoh Koreatsu , Shidara Toshiji
Published: October 2007

Thirty‐one children with ANLL were treated using the 12th‐ANLL Protocol of the Tokyo Children's Cancer Study Group incorporating an ACMP 2‐step regimen. Induction therapy consisted of two 4‐day courses of adriamycin (ADR) and cytosine arabinoside (ARA‐C) given with a 7‐day interval. Those patients who achieved remission were given one more course as early intensification. Late intensification consisted of a 5‐day course of ARA‐C and one dose of ADR which was repeated until the cumulative dosage of ADR reached 465 mg/m2 After this point, treatment was divided into 2 courses. Both courses included ARA‐C and one course had achlacinomycin in addition. Twenty‐seven children (87%) attained remission after one or two courses of induction therapy. Kaplan‐Meyer analysis revealed that the continuous complete remission rate and event‐free survival were 71% and 51%, respectively, 5 years after diagnosis. Toxicity was minimum with only one death during the remission. These results are encouraging and warrant further trials.
Management of Suppuration in Regional Lymph Nodes Secondary to BCG Vaccination
Authors: Caglayan Suat , Arikan Ahmet , Yaprak Isin , Aksoz Kadir , Kansoy Savas
Published: October 2007

We describe 50 patients with suppurative regional lymphadenitis following intradermal BCG vaccination. For non‐drained suppurations we performed needle aspiration in twenty‐three patients (aspiration group). Of the remaining 27 patients (drainage group) 16 had spontaneous drainage and 11 incisional drainage. In the drainage group, 17 patients were followed up conservatively. Ten patients from the drainage group and 3 patients from the aspiration group (drained after needle aspiration) accepted total surgical excision to terminate the drainage. Twenty of twenty‐three patients in the aspiration group recovered after needle aspiration. Average period of cessation of drainage and healing of wound was 7.5 weeks in patients with drainage (spontaneous and incisional). Healing was complete after total surgical excision of draining suppurations in all of 11 patients. We concluded that simple needle aspiration was sufficient for the treatment of suppurating but non‐drained BCG lymphadenitis. For suppurating lymph nodes that were surgically (not recommended) or spontaneously drained, a more invasive procedure, total surgical excision, was proposed to terminate the long and disturbing drainage period.
Giant Hydroureteronephrosis Associated with Primary Obstructed Megaureter
Authors: Tsukahara Hirokazu , Ando Tooru , Fujii Yasushi , Shigematsu Yosuke , Nishibuchi Shigeo , Okada Kenichiro , Nakashima Tetsuo , Hayashi Nobushige , Ishii Yasushi , Fujimoto Iwao , Sudo Masakatsu
Published: October 2007

The case is presented of a four year old boy with a large left abdominal mass. The patient was diagnosed as having a left giant hydroureteronephrosis due to a primary obstructed megaureter, based on the findings of ultrasonography, micturating cystourethrography, excretion urography, computed tomography and percutaneous antegrade pyeloureterography. Diuretic renography showed that the obstruction was partial. Since a static renal scintigram indicated that renal function on the affected side was moderately well preserved, ureteroplasty is planned.
Two Siblings with Autoimmune Neutropenia of Infancy
Authors: Matsubayashi Tadashi , Arakawa Hiroshi , Kawase Reiko
Published: October 2007

We report two siblings with autoimmune neutropenia of infancy. Patient 1 was hospitalized for viral gastroenteritis at 5 months of age, when his absolute neutrophil count was 684/mm3. Absolute neutrophil counts ranging from 0 to 930/mm3 continued but normalized spontaneously at 25 months of age. Patient 2, a younger brother of patient 1, had prolonged high grade fever at 5 months, when his absolute neutrophil count was 138/mm3. Absolute neutrophil counts ranging from 96 to 1,078/mm3 continued for the following 9 months. Antineutrophil antibodies were detected in the patients' sera. Our cases are siblings and each case was independently affected, since the diagnoses were made at 5 months of age and the interval between the diagnoses was 23 months. Autoimmune neutropenia of infancy is comparatively rare, so that we think that the disease in these siblings was due to a genetic abnormality.
The Potential Role of Cytokine‐Mediated Vascular Endothelial Activation in the Pathogenesis of Kawasaki Disease
Authors: Leung Donald Y. M.
Published: October 2007

Kawasaki disease (KD) is an acute febrile illness of infancy and early childhood characterized by diffuse vasculitis. Although the disease is generally self‐limited, up to 30% of untreated patients with KD may develop coronary artery (CA) abnormalities. The acute phase of KD is characterized by marked activation of the immune system leading to increased cytokine production by immune effector cells, the induction of activation antigens on their vascular endothelium and the generation of lytic antibodies directed against vascular endothelial cells (EC) stimulated with cytokines. Treatment with intravenous gammaglobulin (IVGG) usually rapidly reduces acute clinical symptoms and prevents CA abnormalities. Immunologically, successful IVGG treatment is associated with decreased lymphocyte activation, reduced cytokine secretion and the loss of cytokine induced expression of leukocyte adhesion molecules on vascular endothelium. The association between improvement of clinical symptoms with the reduction of cytokine secretion, and reversal of EC activation supports a role for immune mediated injury to cytokine induced EC antigens in the pathogenesis of this disorder.
Immunological Abnormalities in Kawasaki Disease with Coronary Artery Lesions
Authors: Furukawa Susumu , Matsubara Tomoyo , Motohashi Toshikazu , Tsuda Masaharu , Sugimoto Harunori , Yabuta Keijiro
Published: October 2007

A review of our previous immunological studies on Kawasaki disease (KD) was undertaken. The results showed that peripheral blood macrophages/monocytes, T‐cells and B‐cells become activated during acute KD in terms of numerical changes in immunocompetent cells, expression of activated antigens on the cell surfaces and cytokine production. Also, during acute KD with coronary artery lesions (CALs) the numbers of macrophages/monocytes are increased. In addition, both the increased levels of tumor necrosis factor‐α and shed intercellular adhesion molecule‐1 in serum are more evident in KD patients with CALs than in those without. Our results further suggest that the main characteristics of the pathogenesis of KD are increased numbers of peripheral blood macrophages/monocytes with the secretion of monokines by these activated cells, and the expression of adhesion molecules on immunocompetent cells. These immune responses develop more vigorously in KD patients with CALs.
Etiological Role of Phagocytes in Kawasaki Disease
Authors: Kuratsuji Tadatoshi , Takagi Kunihisa , Tsunawaki Shohko
Published: October 2007

The numbers of immature neutrophils and monocytes in the peripheral blood are increased in the acute phase of Kawasaki disease. These phagocytes contain toxic granules and vacuoles in the cytoplasm. Phagocytes are primed and activated to release active oxygen species, lysosome enzymes and chemical mediators, which injure cultured endothelial cells and vascular smooth muscle cells. One of the possible factors causing cardio‐vascular complications in Kawasaki disease is these activated phagocytes. Some microbial agents or their products such as toxins may activate neutrophils and monocytes, but the real cause remains unknown.
Effect of Intravenous γ‐Globulin on Neutrophil Function in Kawasaki Disease
Authors: Okada Masahiko , Satoh Tetsuo , Hayashi Tadashi
Published: October 2007

The effect of intravenous γ‐globulin (IVGG) on the neutrophil count and neutrophil chemiluminescence (CL) of patients with Kawasaki Disease (KD) was investigated. Forty patients with KD were enrolled in the study. Ten patients were treated with 100 mg/kg/day of γ‐globulin for five days (GG 100 group) and 14 patients were treated with 400 mg/kg/day of γ‐globulin (GG 400 group) for five days. These patients also took aspirin. Sixteen patients were treated with aspirin alone (ASA group). The neutrophil counts were significantly lower in the GG 400 and GG 100 groups than in the ASA group, three days, and one and two weeks after the start of treatment. Neutrophil CL of the GG 400 and GG 100 groups was significantly lower than in the ASA group one and two weeks after the start of treatment. In the in vitro study, γ‐globulin had a dose‐dependent suppressive effect on the neutrophil CL in the early stage. Albumin had similar effects. The suppressive effect of γ‐globulin on CL was not specific. These findings suggest that IVGG is effective in reducing the production of active oxygen which is considered responsible for the vascular damage in the early stage of KD.
Intravenous γ‐Globulin for Kawasaki Disease
Authors: Furusho Kenshi , Kamiya Tetsuro , Nakano Hiroyuki , Kiyosawa Nobuyuki , Shinomiya Keisuke , Hayashidera Tadashi , Tamura Tokio , Hirose Osamu , Manabe Yutaka , Yokoyama Tatsuo , Kawarano Masaharu , Baba Kunizo , Baba Kiyoshi , Mori Chuzo , Joho Kunitaka , Seto Shiro
Published: October 2007

We studied the effect of γ‐globulin (IVGG) and aspirin (ASA) on the development of the coronary artery lesions (CAL) of Kawasaki disease (KD) in three different protocols. Within 29 days of the onset of KD the echocardiographic evidence of CAL had developed in 39–42% of the patients in the ASA group, but only in 13.7–20.8% of the patients treated with IVGG (200 or 400 mgγkgX5). In long‐term follow‐up observation of CAL of these patients the evidence of CAL in both the ASA and the IVGG group regressed gradually; however, the residual rate of CAL was significantly low in the IVGG group at all times up to 24 months after onset. These facts suggest that when using IVGG for KD, we should select a dose of intact γ‐globulin, 1,000 mgγkg or more in total, to prevent the occurrence of CAL. We have demonstrated not only a significant reduction in the occurrence of CAL in patients treated with IVGG but a reduction in the residual rate of CAL for two years as compared with those treated by ASA.
Ontogenic Expression of Somatostatin‐Messenger RNA in the Intestinal Tract of Neonatal Rats
Authors: Yu Yin Xi , Koshimizu Takashi , Yokota Yukifumi , Shibayama Keiko , Ohyama Yukihide , Yashiro Kimio
Published: October 2007

Ontogenic expression of somatostatin (SRIF)—messenger RNA (mRNA) in the gastrointestinal tract was examined in neonatal rats aged from 1 day preterm to 60 days postpartum in comparison with that in the hypothalamus. SRIF‐mRNA in the hypothalamus was already expressed in prenatal rats and its developmental change was relatively small. In contrast, a unique pattern of SRIF‐mRNA expression was seen in the different intestinal regions, gastric antrum, duodenum, jejunum and colon. In the duodenum, SRIF‐mRNA level was low at birth, markedly increased during the postnatal 3 days and declined to the previous level by day 21. Jejunal SRIF‐mRNA was found in neonates but progressively decreased in a similar way to duodenum. On the contrary, gastric SRIF‐mRNA level, which was low during early development, rose rapidly to a peak on day 21 and gradually declined to an adult level. In the colon age‐related change was not conspicuous, remaining at a low level. These results indicate that (1) expression of SRIF gene in the intestinal tract is regulated by local factor(s) as well as developmental stage, and (2) shift of SRIF‐mRNA pattern occurs during weaning from the duodenum‐dominant infantile pattern to the gastric‐dominant adult pattern.
Expression of IL‐2 Receptor/p75 on Lymphocytes from Patients with Rubella
Authors: Komada Yoshihiro , Oitani Kaichi , Zhou Yan‐Wen , Zhang Shao‐Li , Sakurai Minoru
Published: October 2007

We analyzed the Interleukin‐2 receptor (IL‐2R) expression on fresh lymphocytes from three patients with rubella. Flow cytometric analysis demonstrated that lymphocytes from the patients expressed a considerable amount of IL‐2R/p75 recognized by Mik‐βl monoclonal antibody (MoAb), although they did not react with anti‐IL‐2R/pS5 (CD2S) MoAb. The expression of IL‐2R/p75 reached a peak at the end of the acute phase of illness. Fresh lymphocytes from rubella patients showed a marked proliferative response to IL‐2, which was completely inhibited by Mik‐βl, indicating that their IL‐2R is a functional receptor. The present data suggest that IL‐2R/p75 expression is involved in the host immune response triggered by the rubella virus.
Erythrocyte Sodium Transport at Twenty‐Year Follow‐Up of Childhood Hypertension
Authors: Uchiyama Makoto , Ogawa Teruyuki , Sakai Kaoru
Published: October 2007

Twelve‐hour urinary sodium excretion, the number of sodium pump sites (Bmax) and the Na/K flux ratio in erythrocytes were measured in 13 previously diagnosed hypertensive subjects. At the time of the study, six subjects were still hypertensive and showed a significantly lower Na/K flux ratio in erythrocytes than the remaining seven subjects who were normotensive. Bmax was also lower in the hypertensive group compared to the normotensive group, although this was not statistically significant. Urinary Na excretion did not show any significant difference between the two groups. These findings suggest that a cell membrane sodium transport defect may have a role in the development of essential hypertension in adult life.
Analysis of HLA‐DQA1 in Japanese Patients with Type 1 Diabetes Mellitus, Using DNA‐PCR‐RFLP Typing
Authors: Tanaka Masahiro , Abe Jun , Kohsaka Takao , Tanae Ayako
Published: October 2007

DNA‐polymerase chain reaction(PCR)‐RFLP(restriction fragment length polymorphism) typing of the DQA1 gene was performed on 39 patients with type 1 diabetes and 30 controls. Analysis of the frequency of the subtypes of DQA1 alleles, using the DNA‐PCR‐RFLP typing technique, showed that the DQA1*0301 subtype was most strongly associated with the disease (97.4% vs 56.7%, R.R. = 19.8, pc < 0.00005). These results indicated that DQA1*0301 may determine the disease susceptibility in the Japanese. In addition, we analyzed the frequency of the subtypes of DQA1 genes in the ten patients carrying the DRW8‐DQW8 haplotype, and found that at least eight of them (80%) were classified as having DQA1*0301. The DRYV8‐DQW8‐DQA1*0301 may be one of the susceptible haplotypes among the Japanese.
Long‐Term Treatment in Infantile Choriocarcinoma
Authors: Hongo Teruaki , Fujii Yuji , Fukuoka Tetuya , Lijima Sigeo , Matsusita Takashi , Nakagawa Yuichi , Igarashi Yoshio
Published: October 2007

The long‐term treatment of a 5 month old boy with precocious puberty secondary to the production of chorionic gonadotropin (hCG) by a choriocarcinoma is described. Of 13 cases of infantile choriocarcinoma reported in the literature, none were successfully treated. The present study describes a partially successful outcome with embolization of a hepatic tumor, irradiation of lung and right submandibular tumors, chemotherapy with methotrexate (MTX), actinomycin D (ACD), cyclophosphamide (CPA) and etoposide (VP16), and splenectomy and hepatic lobectomy. Subsequently, the residual hepatic tumors were treated with high dose melphalan (HDM) followed by reinfusion of unpurged autologous marrow.
Pulmonary Arteriography with Retrograde Injection of Contrast Medium via Radial Artery: Efficacy in Neonates with Ductus‐Dependent Decreased Pulmonary Flow
Authors: Okajima Yoshitomo , Terai Masaru , Nakajima Hiromichi , Ishida Yoshitaka , Niwa Koichirou , Niimi Hiroo
Published: October 2007

In an attempt to visualize the pulmonary arterial trees of neonates and infants with ductus‐dependent decreased pulmonary flow, the efficacy of retrograde injection of contrast from the radial arteries of 11 consecutive patients was tested. All patients had pulmonary atresia and patent ductus arteriosus. A 22‐ or 24‐gauge needle was inserted into the radial artery and contrast medium of 2 ml/kg was injected at flow rates of 3 to 4 ml/sec. The pulmonary arteries were filled by retrograde flow through the ductus arteriosus. This method provided clear visualization of the pulmonary arteries, especially by ductus‐sided injections. Developmental anomalies of the pulmonary trunk, localized stenosis at the ductus, and aortopulmonary collateral arteries were demonstrated. No complications occurred during angiography, and all the procedures were completed within 30 minutes. This method appears to be useful in detecting anatomical abnormalities of the pulmonary arteries in neonates and infants with ductus‐dependent decreased pulmonary flow.
An Infantile Case of Intestinal Myiasis
Authors: Hasegawa Shinji , Miwata Hiroyuki , Masuda Susumu , Naruse Hiroshi , Ozaki Takao
Published: October 2007

A case of intestinal myiasis in an 8‐month‐old girl is reported. The clinical symptoms were ill humor and mucous bloody stools containing a number of moving worms, which were identified as second‐instar larvae of Sarcophaga peregrina.
Severe Neonatal Nemaline Myopathy—Histological and Histochemical Studies of Respiratory Muscles—
Authors: Tachi Nobutada , Wakai Shuji , Watanabe Yukie , Kurosawa Youichi , Agatsuma Yoshitaka , Chiba Shunzo
Published: October 2007

The histological and histochemical findings in the respiratory muscles of a patient with severe neonatal nemaline myopathy are described. The patient suffered from frequent pneumonia associated with vomiting due to gastroesophageal reflux and died at 3 months from respiratory failure. The diaphragm was moderately involved and the intercostal muscles mildly involved. Core/targetoid structures were observed in the diaphragm and intercostal muscles.
Methicillin‐Resistant Staphylococcus aureus Empyema in Children
Authors: Fujita Kozo , Murono Koichi , Sakata Hiroshi , Kaeriyama Masato
Published: October 2007

Over a 14 year period, there were 20 patients who presented with staphylococcal empyema from whom methicillin‐resistant Staphylococcus aureus (MRSA) was isolated. Twelve cases were community‐acquired and 8 were hospital‐acquired infections. Patients were treated with penicillinase‐resistant penicillin, cephalosporin or carbapenem in combination with or without aminoglycoside. They were also treated with drainage or thoracentesis. However, they were refractory to treatment and 7 patients, 6 of whom were suffering from bacteremia, died. One bacteremic patient was treated with vancomycin and was cured. In an area of endemic MRSA, vancomycin may be the first choice in the initial treatment of staphylococcal empyema until antimicrobial susceptibility can be determined.
GC/MS Analysis of Urine in 3‐Hydroxy‐3‐Methylglutaryl‐CoA Lyase Deficiency
Authors: Shinka Toshihiro , Kuhara Tomiko , Inoue Yoshito , Matsumoto Masahiro , Matsumoto Isamu , Nakamura Hideo , Irimichi Hideki , Hasumi Keiji , Endo Akira
Published: October 2007

A patient with 3‐hydroxy‐3‐methylglutaric aciduria was diagnosed using gas chromatography mass spectrometry. The patient had severe metabolic acidosis, hypoglycemia and hyperammonemia and excreted abnormal amounts of 3‐methylglutaconic, 3‐hydroxy‐3‐methylglutaric, 3‐methylglutaric, 3‐hydroxyisovaleric and glutaric acids in the urine. 3‐Hydroxy‐3‐methylglutaric acid appeared as two peaks on the chromatogram after trimethylsilylation. One was a tri‐trimethylsilyl and the other a di‐trimethylsilyl derivative. 3‐Methylglutaconic acid appeared as three peaks: cis‐, trans‐ and cyclic cis‐isomers. The structure of these derivatives was elucidated by deuterium‐labeled trimethylsilyl derivatization. The di‐trimethylsilyl derivative of 3‐hydroxy‐3‐methylglutaric acid and the cyclic cis‐isomer of 3‐methylglutaconic acid do not appear to have been previously described. After treatment with leucine restriction milk, the excretion of leucine catabolites decreased but 3‐methylglutaconic and 3‐hydroxy‐3‐methylglutaric acids continued to be excreted at abnormally high levels. It is concluded that these two metabolites are necessary for the chemical diagnosis of HMG‐CoA lyase deficiency. This patient is the first case of HMG‐CoA lyase deficiency to be reported in Japan.
An Unusual Variant of Chromosome 16 in Three Generations
Authors: Hasegawa Tomonobu , Asamura Shinji , Nagai Toshiro , Tsuchiya Yutaka
Published: October 2007

An unusual variant of chromosome 16, 16p+, in three generations is described. This is the first clinical report of 16p+ in Japan.
Meningeal Malignant Melanoma in a Child: Immunocytological Diagnosis
Authors: Koyama Teruhisa , Ogawa Makoto , Kurata Satsuki , Komazawa Masaru , Murakami Motomasa
Published: October 2007

A 10 year old boy, who was thought to have had a traumatic intracranial hemorrhage, was transferred to our Children's Medical Center. In spite of conservative treatment, he developed dysarthria, systemic convulsions, unconsciousness, quadriplegia, and consecutive paralysis of the cranial nerves. Magnetic resonance imaging scans demonstrated areas of increased signal intensity around the brain stem. The cerebrospinal fluid (CSF) contained a few large cells with abundant melanin‐like granules, and numerous bizarre cells. The latter were considered to be malignant melanoma cells on immunocytological examination. Chemotherapy with dimethyltriazenoimidazole carboxamide (DTIC) and interferon beta (IFN‐β) was ineffective and he expired. Autopsy revealed diffuse infiltration of malignant melanoma cells into the meninges. We think that immunocytological examination of CSF is advisable for correct and rapid diagnosis.
Combined Human Chorionic Gonadotropin (HCG) and Human Menopausal Gonadotropin (HMG) Treatment in Gonadotropin‐Deficient Males with Pituitary Dwarfism
Authors: Tanaka Toshiaki , Hibi Itsuro , Tanae Ayako
Published: October 2007

The effects of hCG‐hMG treatment in 13 boys with pituitary dwarfism associated with gonadotropin deficiency, were assessed.

No patients except one showed signs of puberty at a bone age of 13 years or above. The one patient with some signs of puberty did not become fully mature. The hCG‐hMG was started at a mean age of 20.4 years. The hCG at a dose of 5,000 IU was injected intramuscularly twice a week and the hMG at a dose of 75 IU was given once a week at first. During treatment, the frequency of hMG injections was increased to twice a week in six patients who still had not produced normal sperm counts. After a mean duration of 19.23 months, spermatozoa appeared in eight patients, of whom four showed more than 20 × 106 sperm/ml. Among six patients who did not have normal sperm counts and had increased hMG injections, one produced a pregnancy and four achieved sperm counts of more than 35 × 106/ml. One patient had refractory azoospermia. In 13 boys with growth hormone and gonadotropin deficiency, hCG‐hMG treatment produced normal spermatogenesis in nine patients, one of whom fathered a girl. Thus, hCG‐hMG treatment, especially twice‐a‐week injections of both hCG and hMG, appears to be effective for gonadotropin deficiency in males.
IgG Subclass Imbalance in Children with Acute Lymphoblastic Leukemia Receiving Maintenance Chemotherapy
Authors: Wakiguchi Hiroshi , Fujieda Mikiya , Hisakawa Hiroaki , Kubota Haruo , Matsumoto Kenji , Kurashige Takanobu
Published: October 2007

Serum levels of IgG subclasses were measured in 18 children with acute lymphoblastic leukemia (ALL) receiving maintenance chemotherapy and in 36 age‐matched controls in order to attempt to analyse the effects of chemotherapy. The IgG subclasses were measured by enzyme linked immunosorbent assay. Serum IgG1, IgG2 and IgG4 levels in the patients were significantly (p<0.01, p<0.005, p<0.005) lower than in the controls, but serum IgG3 levels in patients were as high as in controls. Suppression on IgG2 and IgG4 were more profound than IgG1. In six children, the levels of the IgG subclasses were measured at diagnosis, during maintenance chemotherapy and one year after cessation of chemotherapy. The levels of the four IgG subclasses at diagnosis and after cessation of chemotherapy were as high as those in control children except for the IgG4 levels in the postchemotherapy group. IgG2 and IgG4 may be more susceptible to suppression by chemotherapy than IgG1 and IgG3 may not be suppressed by chemotherapy.
Evaluation of Atrial, Ventricular and Atrioventricular Septal Defects by Cine Magnetic Resonance Imaging
Authors: Akagi Teiji , Kato Hirohisa , Kiyomatsu Yumi , Saiki Kuninobu , Suzuki Kazushige , Eto Takaharu
Published: October 2007

Cine magnetic resonance imaging (MRI) was performed on 20 patients (mean age: 5.3 ± 4.4 years) with atrial, ventricular, or atrioventricular septal defects for evaluation of cardiac structure and blood flow. Prior to cine MRI, electrocardiographically gated MRI using multislice acquisition was performed on all patients to localize optimal slice location. Cine‐MRI was obtained with a 30° flip angle, 15 msec echo time, and 30 msec pulse repetition time, on a 256×256 or 128×128 acquisition matrix. Abnormalities of cardiac structure were well defined in all patients by gated cardiac imaging. In 18 of the 20 patients, cine‐MRI was able to detect shunt flow, visualized as a low intensity signal in comparison with the surrounding blood flow. Cine‐MRI can provide not only accurate anatomy of cardiac structures but functional assessment of the cardiac chamber, wall topology and flow relations. Cine‐MRI will become an important noninvasive technique for assessment of anatomy and physiology in congenital heart disease.
A Study of Psychosocial Factors in the Psychosomatic Symptoms of Adolescents in Okinawa
Authors: Nakada Yukikatsu
Published: October 2007

High school students (N = 902) in Okinawa were asked to participate in a questionnaire survey which was designed to investigate the psychosocial factors in the psychosomatic symptoms of adolescents. The questionnaire focused on mental distress, perception of school and home environment and psychosomatic symptoms. The Japanese Edition Cornell Medical Index‐Health Questionnaire (JCMI) for evaluating emotional instability and the New TK Diagnostic Test for Parent‐Child Relationship were also utilized. Distress in relationships with family or friends, finding little pleasure in school and/or home, showing emotional instability as diagnosed according to regions III and IV of JCMI, or showing strained parent‐child relationships according to the TK Test items were found to be closely associated with psychosomatic symptoms. In addition, students with human relations problems, especially family problems, tended to have a strained relationship with parents and tended to show autonomic hyperactivity represented by orthostatic dysregulation. Students with peer problems tended to have emotional instability and to find little pleasure in school; they had mental as well as urinary and bowel symptoms. The findings of this study suggest that distresses in family or peer relationships, emotional instability and a strained parent‐child relationship are important factors in the onset of psychosomatic symptoms in adolescents.
The Origin of Myoclonus and Periodic Synchronous Discharges in Subacute Sclerosing Panencephalitis
Authors: Yagi Shinichi , Miura Yo , Kataoka Naoki , Mizuta Shun , Wakunami Atsuko , Morita Tetsuro
Published: October 2007

We report here a case of a patient with subacute sclerosing panencephalitis (SSPE) and we have analyzed periodic events using dipole tracing methods to clarify the origin of periodic synchronous discharges and myoclonus. Both source generators were located in the subcortical part of the cerebrum, an area adjacent to the thalamus. Although the pathophysiology of periodic events in SSPE has been controversial, dipole tracing methods may contribute to clarify the origin of periodic events in SSPE.
Hydrops Fetalis Caused by Fetal Kasabach‐Merritt Syndrome
Authors: Anai Takanobu , Miyakawa Isao , Ohki Hiromi , Ogawa Teruyuki
Published: October 2007

There have been only 2 previous reports of nonimmunologic hydrops fetalis (NIHF) caused by fetal Kasabach‐Merritt syndrome, both of which were pathological studies. This is the first clinical case report of NIHF due to fetal Kasabach‐Merritt syndrome that was prenatally diagnosed by sonography, computerized tomography, and percutaneous umbilical blood sampling.
Human Herpesvirus 6 (HHV‐6) Infection in the Central Nervous System
Authors: Yamanishi Koichi , Kondo Kazuhiro , Mukai Tetsu , Kondo Toshio , Nagafuji Hiroshi , Kato Tomochika , Okuno Toshiomi , Kurata Takeshi
Published: October 2007

Human herpesvirus 6 (HHV‐6) was isolated from patients with exanthem subitum (ES) with a high frequency, and it is now believed that this virus causes ES as a primary infection in childhood. HHV‐6 infection is highly prevalent in early childhood and this virus may infect infants through the saliva mainly from mother to child. HHV‐6 has a tropism to CD4+ cells and destroys cells in vitro. Although children recover from ES without any sequelae, neurological symptoms associated with exanthem subitum are often observed, and we could detect HHV‐6 in the cerebrospinal fluid of ES patients. This result suggests that HHV‐6 may invade the central nervous system and cause neurological symptoms.
Dengue Encephalopathy
Authors: Hendarto S.K. , Hadinegoro Rezeki
Published: October 2007

Dengue encephalopathy or dengue hemorrhagic fever (DHF) with CNS involvement used to be considered a relatively rare condition; but the number of cases reported in human studies has been increasing every year. Diagnosis of dengue encephalopathy is based on clinically diagnosed DHF according to the W.H.O. criteria (1980), with CNS manifestations consisting of abrupt onset of hyperpyrexia, nontransient alteration of consciousness, headache, vomiting‐with or without seizures ‐and normal CSF. Many factors may be considered to be directly or indirectly associated with CNS signs and symptoms in DHF, the main pathology being leakage of plasma into serous spaces and abnormal hemostasis, leading to hypovolemic shock and hemorrhage in many organs of the body. Acute liver failure is considered to be one of the main factors causing brain pathology. One hundred fifty‐two cases of dengue encephalopathy admitted during 3 periods at the Cipto Mangunkusumo Hospital in Jakarta were studied retrospectively. The overall incidence was 152 out of 2,441 DHF cases, or 6.2%. The most pronounced symptoms were hyperpyrexia, alteration of consciousness and convulsions. Laboratory examination showed an unusually high increase of serum transaminases, hyponatremia, and hypoxia. Neurologic abnormalities detected were hemiparesis and tetraparesis of the extremities, and second nerve atrophy; such abnormalities were found in 10 out of the 152 cases, or 6.5%.
Primary Immunodeficiency Diseases and Epstein‐Barr Virus‐induced Lymphoproliferative Disorders
Authors: Okano Motohiko , Nakanishi Masanori , Taguchi Yuichi , Sakiyama Yukio , Matsumoto Shuzo
Published: October 2007

Increased incidence of malignant disorders is noted in patients with both primary and acquired immunodeficiency diseases. The pathogenetic mechanism(s) for these disorders remain unclear. Defective immunosurveillance of these patients, however, is mainly postulated to be responsible for the increased risk of these malignant disorders. Of the malignant disorders, Epstein‐Barr virus (EBV)‐induced lymphoproliferative disorders (LPD) have been increasingly reported, possibly due to improved therapeutic management techniques such as bone marrow transplantation, which results in prolonged survival periods for the primary immunodeficiency; the dramatic development of immunosuppressive treatments for transplant recipients; and the growing numbers of acquired immunodeficiency syndrome (AIDS) patients.

This review focuses on the primary immunodeficiency diseases and EBV‐induced LPD, and discusses pathogenetic mechanism(s) for the increased incidence of these malignant disorders.
Comparison of the Incidence of Intracranial Hemorrhage following Conventional Mechanical Ventilation and High Frequency Oscillation in Beagle Puppies
Authors: Tamura Masanori , Hishi Toshio , Ishii Teruyuki , Wakita Suguru , Oho Shinichi , Shibuya Kazuhiko , Miyasaka Katsuyuki , Takashima Sachio
Published: October 2007

Controversy exists as to whether high frequency oscillation (HFO) increases the risk of intraventricular hemorrhage (IVH) compared with conventional mechanical ventilation (CMV). We compared the risk of intracranial hemorrhage after phenylephrine‐induced hypertension, combined with a hypovolemic, hypotensive insult followed by rapid volume replacement in two groups of newborn beagle puppies (one group on a piston pump HFO and the other on CMV).

A total of 12 beagle puppies (6 on HFO and 6 on CMV) survived the protocol. Arterial blood gas analysis and arterial blood pressures through the study were of the same magnitude in both groups. The length of time for which the puppies remained hypertensive and hypotensive also did not vary significantly between the groups.

Intraventricular hemorrhages were observed in two of six CMV puppies and two of six HFO puppies. One animal in each group had a white matter hemorrhage. Subarachnoid hemorrhages were seen in 4 animals on CMV and 3 on HFO.

This study indicated that HFO does not increase the risk of any kind of intracranial hemorrhage, including IVH, in beagle puppies.
Partial Imitation and Partial Sensory Agnosia in Mentally Normal Children with Convulsive Disorders
Authors: Hara Hitoshi , Fukuyama Yukio
Published: October 2007

Mentally normal 6–9 year old children with or without convulsive disorders were examined using the procedures of the five soft signs (SS) selected from conventional soft neurological signs and Berges' gesture imitation tasks. In comparisons of SS between the 60 children with convulsive disorders (CD group) and the 38 without (N group), the average number of SS detected in each case and the incidence ratio of the two signs ‐ partial imitation (PI) and partial sensory agnosia (PSA) ‐ were higher in the CD group than the N group. Examinations of relationships between age, IQ, error index (EI; an index taken from the Continuous Performance Test) and SS, and of correlations among the SS, found two kinds of SS: age‐related and El‐related signs. The former included right‐left confusion, clumsiness and PI, which correlated with each other, while the latter included unstableness of lateral gazing and PSA, which did not correlate with each other. Even though several factors belonging to characteristics of convulsive disorders were evaluated concerning the presence or absence of PI and PSA, there was only one relationship: PSA was more common in children with febrile convulsions than in those with epilepsy. We speculated that PSA reflected an immaturity of the brain in children with febrile convulsions.