bluuebunny/crossref_metadata_2025 huggingface dataset:
Autoimmunity of Insulin‐Dependent Diabetes in Japan
Authors: Hirata Yukimasa , Nakazawa Michio , Shinjo Takamichi , Nagai Naoko
Published: October 2007

In this paper some findings concerning autoimmunity have been studied in young Japanese patients with diabetes. Patients diagnosed before 30 years old were classified carefully into new categories recommended by WHO in 1980: insulin‐dependent (IDDM), non‐insulin‐dependent (NIDDM) and other types of diabetes. HLA antigen types, islet cell antibodies (ICA) and islet cell surface antibodies (ICSA) were checked in the sera. HLA‐Bw54 and ‐DR4 were found in a significantly high percentage of IDDM patients, especially in those with Insulin‐Dependent Diabetes Mellitus with Special Reference to acute onset of the disease. Positivity of ICA was remarkably high in IDDM Viral Infections patients within one year after the onset, and then decreased very rapidly. ICSA had the same tendency, but the rate of decrease of positivity seemed slower than that of ICA

Apparent autoimmune diseases were rare among our patients, and persistent positivity of ICA was found only in a few cases. At this time we have notenough evidence concerning autoimmunity as a major cause of IDDM.
Psychological Problems in the Families of Japanese Insulin‐Dependent Diabetes Mellitus (IDDM) Patients
Authors: Takaki Shunichiro , Issiki Gen , Ikuno Teruko , Aono Shigeo , Kokui Yuko
Published: October 2007

Emotional stability of insulin‐dependent diabetics is closely related to their metabolic control. We have already presented the psychological features of diabetic children at the 5 th International Beilinson Symposium. These results indicate that psychological problems are usually interactions with the patient's family. Family troubles derived from impaired interrelation are significant. From these points of view, counselling with the family based on a proper understanding of their relationship is absolutely necessary. We have had regular meetings for mothers with diabetic children in our clinic to discuss their psychological troubles. In this study we examined the frequency of poorly controlled patients in our clinic and analyzed the nature of their problems. We further summarized the opinion of family members which had been discussed in the above‐mentioned meeting. Frequency of constantly poorly controlled patient was 8.1% (3/37) in male and 16.3% (8/49) in female. We report on our method' of approach to them.
Psychological Aspects of Childhood Diabetes
Authors: Laron Zvi , Amir Shoshana , Gil Ruth , Karp Moshe , Beth‐Halachmi Zesia , Kaplan Yael , Galatzer Avinoam
Published: October 2007

The diagnosis of diabetes mellitus, a life‐long disease with many possible complications, has a dramatic impact upon the entire family, precipitating a state of “shock.” We have studied for 17 years various psycho‐social aspects of “having diabetes mellitus” or having “a child with diabetes mellitus” in 550 families. The findings led to changes in the therapeutic approach and structure of the treating team. The psychological problems in diabetes should be divided in 3 periods: at diagnosis (other diseases or tension existing in the family and not related to diabetes, socioeconomic state); initial adaptation period (acceptance to be “different,” adjustment to rules of control such as daily injection of insulin, self blood glucose monitoring or urine testing, changing of nutritional habits, etc.), and long‐term coping (self image, family dynamics, social activities, school achievements, vocational rehabilitation and continuing compliance). Counselling of the psychological problems is an ongoing need and is best delivered when the treating team includes psychologists and social workers. We found evidence that this approach imptoves the quality of diabetes control, permits the young diabetic to live a normal life and diminishes the development of complications.
Social Aspect of Insulin‐Dependen Diabetes Mellitus in Japan
Authors: Takeda Akira
Published: October 2007

I investigated the problems of occupation and marriage which are two of the biggest obstacles of insulin‐treated childhood diabetics in their life in society.

Diabetic Patients and Occupations: The enquiry which was conducted on 48 diabetics who participated in our Summer Camp for Diabetic Children revealed that both parents and children preferred and wanted to be engaged in medical and co‐medical occupations such as pharmacist, doctor, nurse, dietitian and so on. It also revealed that those high school students who were to choose their occupation in the near future picked out rather realistic occupations for them such as white‐collar worker, goverment officer, teacher and so on. Those who had higher education actually were engaged in medical and co‐medical occupations or such occupations which require special qualifications.

Diabetic Patients and Marriage: Out of fifteen married insulin‐dependent diabetics, four were divorced simply because they were diabetics.

The present research concludes that diabetics have to control themselves so that they are in such good condition as to pursue their life in society without being seized with any complications; and unless we medical staff provide positive aid for them, childhood diabetics will have much difficulty in attaining any independent life in society.
Identification and Management of Psycholocial Aspects of diabetes
Authors: Etzwiler Donnell D.
Published: October 2007

Diabetes occurring in childhood has a rapid onset frequently with severe physical consequences. This diagnosis is often difficult to accept by both the parents and the child. Kubler‐Ross has described the five stages individuals go through when they are first informed of a terminal illness and these are often mimicked in the families of children with diabetes. At first these families experience a state of disbelief: As insulin therapy is initiated, blood tests obtained, diet prescribed, etc., anger begins to build and may be expressed or more frequently withheld and pent‐up inside. Bargaining may then begin on questions such as, if I take good care of it, will it go away? If I am good will it go away, or even making commitments to God in exchange for “taking away” the disease. As the family begins to realize that the condition won't go away, they become depressed as all of the limitations and demands become apparent. Many patients and their families never progress beyond this stage. With appropriate inter‐strength and support acceptance can be achieved and the enormous burden of disease management and adaptation are lifted. An awareness and expectation of these various stages by health professionals can be of major assistance and reassurance to these patients and their families.
Deep Sensation in the Congenital Rubella Syndrome
Authors: Tokugawa Ken , Ueda Kohji
Published: October 2007

Sixty‐three deaf children with congenital rubella syndrome (CRS) and 61 non‐ CRS deaf children were studied for their sensations of vibration and position. Both groups showed normal responses in the sensation of vibration. However, 35 CRS and 22 non‐CRS deaf children revealed abnormal responses in the sense of position. This gave a significantly higher ratio in the CRS deaf children.
Type IIB von Willebrand's Disease: Report on the First Case in Japan
Authors: Fukui Hiromu , Yasui Motoshi , Takahashi Yukihiro , Niinomi Kou , Takase Toshio , Yoshioka Akira
Published: October 2007

Three members of a family are reported with type IIB von Willebrand's disease (vWD), characterized by prolonged bleeding time, increased ristocetin‐induced platelet aggregation, fast anodal migration of factor VIII‐related antigen on crossed immunoelectrophoresis and an absence in plasma and presence in platelets of the larger multimers of factor VIII/von Willebrand factor (FVIII/vWF). Binding ability of the patient's FVIII/vWF to washed normal platelets is significantly increased in the presence of low concentrations of ristocetin, whereas binding ability of the patient's platelets to normal FVIII/vWF is not increased. These later findings are contrary to those in patients with pseudo‐vWD or platelet type vWD. The administration of DDAVP gives a shortening of bleeding time and an increase in plasma levels of factor VIII‐related activities. In addition, there is a transitory appearance of the larger multimers and a moderate diminution of the platelet count.
Allergy: Bronchial Sensitivity and Course of Asthma in Children
Authors: Teramich Yoshiaki , Kurihara Kasuyuki , Sugiuchi Masami , Nemoto Toshihiko
Published: October 2007

We conducted the study on factors affecsting progonsis of bronchial asthama, clinical course and bronchial sensitivity to acetycholine 127 children with asthma, who received acetylcholine inhalation test and were subjected to observation for at least 3 years in Kanagawa children's Medical Center. First, the children were divided into two groups on the basis of asthmatic attacks. Those without asthamatic attacks for over 2 years were classified into the remission group and those with recent asthamtic episodes, the non‐remission group. A further calssification was based on bronchial sensitivity of acetylcholine. The two groups derived were the hypersensitivity group and the low sensitivity group.

The following conclusion were drawn from the long‐term onservation of the patients. 1) One‐rank change in acetylcholine threshold does not correspond to a change in bronchial sensitivity. 2) Imporovement in brochial sensittivity to acetylcholine accompanies the disapperance of asthamtic attacks. 3) Bronchial hypersensitvity to acetylcholine is maintained by the presence of asthmatic attacks. 4) Significant change in brochial sensitivity to acetylcholine does not appear within 2 years of the presence or absence of asthmatic episodes.
Allergy: The Development of Bronchial Asthma in Infancy
Authors: Mukouyama Tokuko
Published: October 2007

Peripheral blood eosinophils, nasal eosinophils, serum immunoglobulin G,A,M,E, allergic skin tests, RAST tests, viral antibody and secretory IgA levels were studied in one hundred asthmatic children who had had their first asthma attack before 24 months of age.

Viral and bacterial infections were detected in 26 of 79 episodes of attacks. Parainfluenza was most frequently associated, and next in frequency were respiratory syncycial virus, mycoplasma pneumoniae and measles. The serum IgE and IgM level was higher than the normal range for age.

In 16.9% serum IgA showed a lower level than in normal children. The allergic skin test by scratch method was positive in 47.6% to egg white, 39.7% to house dust, 44.1% to Dermatophagoides farinae. The RAST test showed similar results.

Secretory IgA in saliva was examined by the method of enzyme linked immunoassay. Secretory IgA in infants with egg allergy was lower than in normal healthy children. The predisposition to the development of atopic disease with, maturationally related decreases in secretory IgA could be explained by the ingress of allergic materials.
Infection and Immunity: Neonatal Immunology
Authors: Stiehm E. Richard
Published: October 2007

Each limb of the immune system is partially functional, partially non‐functional at the time of birth. Newborns are unusually susceptible to infection, and feeding practices, immunization schedules and certain congenital conditions are all affected by the state of the infants immune system. I shall review each of these systems.

The granulocytes are slow to chemotax but can phagocytise well, and have only a modest reduction of bactericidal activity; however this latter ability may be compromised severely in the presence of stress or infection.

The mononuclear phagocytes (monocytes) are also slow to chemotax, but their bactericidal and cytotoxic functions are normal. The fixed mononuclear cells may have diminished phagocytosis and killing capacity. The monocytes may also have a defect in antigen processing with decreased expression of DR antigen.

Complement activity is about 25–50% of the maternal level but this is usually sufficient to prevent infection in the term infant. In the presence of prematurity complement components may be reduced sufficiently so as to produce a clinically significant opsonic deficiency. This can be corrected by plasma or gamma globulin. Certain Gram (‐) organisms need alternative complement pathway activation to affect opsonization the newborn may have decreased ability to activate this pathway.

The antibody synthetic system is essentially inoperative at birth despite adequate B‐cells, as a result of intrinsic B‐cell inactivity and enhanced T‐cell suppression. Passive maternal IgG usually but not always, compensates for this deficit in the term infant. Local secretory IgA antibody synthesis is also absent for the first 6 to 8 weeks of life; this is compensated for in part by the ingestion of breast milk, The first antibody responses noted are IgA antibody responses, while IgG responses are initially sluggish and delayed. Poor IgG antibody responses to certain antigens, particularly polysaccharides, may persist for a prolonged period.

Neonatal T‐cells show good recognition and proliferative responses but T‐cell cytotoxicity is compromised and certain lymphokines (e.g. MIF, immune interferon) are markedly deficient. Delayed cutaneous hypersensitivity is also deficient. Natural killer activity is also reduced. Thymus size and hormonalactivity is increased.

Taken as a whole, the immature immune system does not have the capacity to fully respond to an invading organism, and the result is frequent, severe, and occasionally fatal infection.
Metabolism: Enzymatic Diagnosis of Mucopolysaccharidosis and Lipidosis
Authors: Yabuuchi Hyakuji , Yutaka Tohru , Okada Shintaro
Published: October 2007

New radiolabeled oligosaccharides were prepared from various glycosaminogly‐cans for the purpose of diagnosis of mucopolysaccharidosis and glycosphingoli‐pidosis and of differentiation of enzymatic heterogeneity. Hunter syndrome hemizygote and heterozygote could be successfully diagnosed using hair roots as an enzyme source. The deficiency of galactose‐6‐sulfate sulfatase was observed in Morquio syndrome in addition to the deficiency of N‐acetyl‐galactosamine‐6‐sulfate sulfatase. Tay‐Sachs disease was diagnosed without separation of hexosaminidase using hyaluronic acid‐derived trisaccharide as sub‐strate, Trisaccharide substrate derived from keratan sulfate could be used for the diganosis of GM1‐gangliosidosis, however, it was not useful for the dif‐ferentiation of type 1 and type 2.
Metabolism: Comparison of Outcome: Inborn and Outborn Infants Weighing Less Than 1,500 Grams
Authors: Lubchenco Lula O. , Butterfield L. Joseph , Black Virginia D. , Koops Beverly L. , Goldson Edward , Manchester Catherine
Published: October 2007

The fetal mortality, neonatal mortality and developmental outcome of 690 less than 1,500 gram infants born during 1974–78 in Denver, Colorado, USA, was studied. Comparisons were made of 393 babies born at University Hospital (UH) where an intensive intrapartum care program was established and 297 babies born in five metropolitan Denver hospitals (MDH) who did not have intensive care capability. They were usually transported to The Children's Hospital (CH), Denver, for neonatal intensive care. Fetal mortality was lower in the UH population than the MDH population; neonatal mortality in the UH and. MDH babies was not different above 700 grams; neonatal delay was least(8%) in the babies born to mothers transported to UH for delivery (UH‐MT) and highest (21%) in the MDH babies who were transported to CH for neonatalintensive care. The authors conclude that fetal survival improved at UH andthat mental delay is least in babies born to mothers who are transported to UH for perinatal intensive care.
Metabolism: Periventricular‐Intraventricular Hemorrhage in Small Premature Infants
Authors: Imura Soichi , Ohno Tsutomu , Takada Masaaki , Takahashi Shigeru , Baba Kazuo
Published: October 2007

The incidence of periventricular‐intraventricular hemorrhage (PVH/IVH) in small premature infants diagnosed by computerized tomographic scanning (CT) is 40–50%. Ultrasound brain scanning is now emerging as an alternative modality for diagnosing PVH/IVH as early as the first day of life. Portability of the equipment and lack of ionizing radiation make this method ideal for studying newborn infants in intensive care units.

In the present study the diagnosis of PVH/IVH was made using CT and ultrasound. Infants with PVH/IVH were then compared with infants in whom no bleeding was detected. The relationship between selected obstetric, neonatal, asphyxia1 and therapeutic factors and PVH/IVH was studied in a group of small premature infants. Statistically significant associations with respiratory distress, ventilator therapy, low Apgar score and metabolic acidosis were observed, lending support to their role in the pathogenesis of PVH/IVH.

The prognosis of small premature infants suffering from post‐hemorrhagic hydrocephalus is probably related to the extent of the first insult and hemorrhage and the rate and extent of the development of subsequent hydrocephalus.
Essential Fatty Acids, Prostaglandins and the Gastrointestinal Tract
Authors: Dodge John A.
Published: October 2007

The polyunsaturated fatty acids linoleic, linolenic and arachidonic are essential components of a normal diet and are particularly important in young infants who are growing rapidly. A distinct clinical syndrome of essential fatty acid deficiency is occasionally seen in children with nutritional or absorptive defects. It may be treated orally, intravenously or transcutaneously. The essential fatty acids are precursors of the prostaglandins, which have high biological activity in a variety of tissues. In the small intestine, prostaglandins enhance both secretion and motility. Elevated plasma prostaglandins have been noted in a number of diarrhoea1 conditions, including chronic non‐specific diarrhoea in toddlers and rotavirus enteritis in infancy. There is a close relationship between fatty acids, prostaglandins and zinc metabolism.
Human Rotavirus Infection: Pathogenesis and Pathology
Authors: Horino Kiyotaka , Chiba Shunzo , Nakao Tooru
Published: October 2007

Duodenal mucosa and feces from 12 children with an acute non‐bacterial gastroenteritis were electron‐microscopically examined. In the study of the duodenal mucosa, many virus particles were seen in the distended cisternae of the endoplasmic reticulum of epithelial cells from the three patients who were biopsied within 72 hours from the onset of symptoms. Almost all virus particles had a highly electron‐dense core and were surrounded by a moderately dense double shelled capsid zone about 70 nm in diameter. However, particles without the outer shell capsid zone and particles without a core were also present. Matrices of viroplasm were often found close to the endoplasmic reticulum. These matrices were surrounded by many virus particles at various mature stages, and sometimes contained electron‐dense cores about 40 nm in diameter.

In the study of the feces, the extracts of feces from all 12 children contained numerous virus particles. The morphological appearance of the virus particles observed in the feces and duodenal mucosa was identical. In this study, the morphological and morphogenetic resemblance of human rotavirus to other animal rotavirus was emphasized.
The Screening of Patients with Extrahepatic Biliary Atresia by Measuring Total Bile Acids Absoredin Dried Blood Spots
Authors: Matsui Akira , Fujimoto Takako , Takazawa Yoshie , Kasano Yasuo , Okaniwa Mariko , Kamoshita Shigehiko
Published: October 2007

The efficency was assessed of the screening for extrahepatic biliary atresia by measuring total bile acids absorbed in dried blood spots at around 5 days of age. When the cut‐off level was 54.0 μmol/l, the sensitivity, the specificty and the efficiency of the screening test were 87.9%, 93.9% and 93.9% respectively. The case‐finding rate was 0.009% in this type of screening which was three times as many as an early case‐finding rate by population screening. These results suggest that teh efficiency of this screening is acceptable.
Genetic Study on the Etiology of Congenital Heart Diseases
Authors: Ohdo Shozo , Sonoda Tohru , Madokoro Harumichi , Matsuoka Yuji , Sennari Eiichi , Okishima Takahiro , Suzumiya Hiroko , Nishiguchi Toshihiro , Hayakawa Kunio
Published: October 2007

The etiology of congenital heart disease was studied in 1,076 patients who were diagnosed in our outpatient clinic from September 1, 198 1 to February 29, 1984.

In these patients, 9(0.8%) had single gene disorders and 63(5.9%) had chromosomal aberrations, of which 55(87.7%) were Down syndrome. The number of patients who had congenital heart diseases due to environmental insult was 2(0.2%), and those with a malformation syndrome of unknown etiology were 6(0.6%).

The etiology of congenital heart diseases in the remaining 996 patients (92.6%) was not clarified. In these cases, the rates of consanguinity of parents, and paternal and maternal grandparents, the ages of the parents when the patients were born, and the number of minor anomalies, were compared with those in normal controls. The complications of major anomalies were also investigated. Further, the recurrence risks in patients' siblings were also calculated.
Studies on Phagocytosis and Microbicidal Activity of Monocytes in Children with Acute Leukemia.
Authors: Yoda Satoru
Published: October 2007

Phagocytosis and microbicidal activity of monocytes in children with acute leukemia were studied in relation to the stages of the disease and severe fungalinfections.

Phagocytosis and microbicidal activity of monocytes were defective in the active phase (before therapy, during relapse), and normalized during complete remission. Monocyte candidacidal activity in 11 of the 15 patients with severe infections was also defective.

Extracellular factors such as serum from the patient, leukemic blast cells in the active phase and antileukemic drugs (prednisolone, vincristine, methotrexate) did not alter the monocyte functions at the concentrations tested.

In seven patients with severe fungal infections, monocyte phagocytosis of Candidu albicans was defective in some patients, Candidacidal activity of monocytes was defective in all patients.

The present study indicates that phagocytosis and microbicidal activity of monocytes are defective in the active phase of childhood acute leukemia and microbicidal activity is closely associated with the occurrence of severe fungal infections.
CONTROL AND DAILY CARE OF SICK CHILDREN AT SCHOOL: Obesity
Authors: Murata Mitsunori
Published: October 2007

Recently, the incidence of obese children is increasing in Japan. According to the School Health Examination Survey by the Ministry of Education, about 5–10% of school children are obese. Obesity is considered a primary risk factor for atherosclerosis. It is most worthwhille to treat obesity in the school health program. However, it is very important to differentiate those obese children who really require management from those who do not and to detect the potentially obese children as early as possible. In order to do this easily and effectively studies on the growth curve for weight and height should be done. If the slope of his or her actual growth curve for weight is always greater than that of the standard, the child should be under medical control for obesity. Even if a child is obese, but his or her growth curve for weight has a normal pattern, the child is only placed under the observation and need not be categorized as in the “obese group.” Generally speaking obesity with short stature is symptomatic.

It is also worthwhile to screen for risk factors such as hypertension, dyslipoproteinemia and diabetes mellitus in obese children. It is known that fatty liver in obese children results in cirrhosis, and it is recommended to examine liver function (measurement of serum GPT) in all obese school children.

In order to obtain satisfactory results in the management of obese children, everything should not be left to the school health program and it is necessary to have the concerted efforts of the government, medical associations, society, school and homes, each of which should have a common understanding of the problem.
Monocyte Chemotaxis in Primary Immunodeficiency Syndrome
Authors: Yamazaki Munehiro , Yamazaki Toshio , Yasui Kozo , Komiyama Atsushi , Akabane Taro
Published: October 2007

We studied monocyte chemotaxis in children with various types of primary immunodeficiency syndrome. The clinical and immunologic features of the patients with defective monocyte chemotaxis were then analyzed. Monocyte chemotaxis was defective in two patients with a monocyte disorder characterized by chronic candidiasis, absence of delayed‐type hypersensitivity, and normal T cell functions. Monocyte chemotaxis was also impaired in a patient with Chédiak‐Higashi syndrome, and in two of six patients with hyper‐IgE syndrome. Major infections in the patients with defective neutrophil chemotaxis but normal monocyte chemotaxis included recurrent pyogenic infections such as otitis media, superficial abscess, lymphadenitis and pneumonia. On the other hand, the clinical features of the patients with defective monocyte chemotaxis were characterized by chronic candidiasis. Delayed‐type hypersensitivity was negative in all of the patients in whom monocyte chemotaxis was defective, despite the absence of apparent T cell dysfunctions. These results demonstrated that there is defective monocyte chemotaxis in some types of primary immunodeficiency syndrome. The monocyte chemotaxis defect is probably responsible for candidiasis and related to the absence of delayed‐type hypersensitivity.
Defective Opsonization Induced by Chemotherapy in Children With Leukemia or Lymphoma
Authors: Ueno Norihiro , Matsumoto Shuzo
Published: October 2007

Complement‐mediated opsonin activity was measured by the whole blood chemiluminescence method in 17 children with leukemia and lymphoma (6 with ALL, 7 with ANLL and 4 with non‐Hodgkin's lymphoma) during remission induction therapy. The opsonin activity, which was at the normal level before chemotherapy, was decreased in all of the children during the therapy. This decrease was especially remarkable in the children treated with L‐asparaginase. When the opsonin activity was decreased, the activities of other complement systems (C3, CH50) were also damaged. Among these activities, the opsonin activity was best maintained.
Neutrophil Receptors
Authors: Ito Setsuko , Mikawa Haruki
Published: October 2007

We studied two interesting patients with neutrophil dysfunction: one is a patient with hyper‐IgE syndrome with the serum inhibitor which belongs to IgE class and the other is characterized by defective phagocytosis confined to S. aureus. These patients suggested to us the presence of IgE receptors and receptors for S. aureus on neutrophils.

The presence of IgE receptors on neutrophils was proved by the fact that neutrophils preincubated with 10 μg of pure IgE generated O‐2 on stimulation with F(ab')2, fragments of pure anti‐K chain antibody or anti‐K chain antibody. The presence of IgE receptors was also suggested by the suppressive effect of IgE soluble immune complexes on neutrophil chemotaxis.

Neutrophils of the patient with defective phagocytosis confined to S. aureus could not generate O‐2 with unopsonized S. aureus, while control neutrophils generated a significant amount of O‐2 on addition of unopsonized S. aureus. Colchicine treated control neutrophils also could not generate O‐2 by unopsonized S. aureus. These data suggested the presence of receptors for S. aureus on neutrophils.
IgE and IgG Antibodies to Staphylococcus aureus Solubilized Cell Wall Proteins and Teichoic Acid in Patients with the Hyper‐IgE Syndrome
Authors: Shibata Rumiko , Umeda Akiko , Miyazaki Sumio , Nishima Sankei , Amako Kazunobu
Published: October 2007

The anti‐staphylococcal IgE and IgG antibody levels of patients with hyper‐IgE syndrome (HIE) were measured by microtiter solid phase radioimmunoassay (MSPRIA) and enzyme‐linked immunosorbent assay (ELISA) using solubilized cell wall proteins (SCWP) and teichoic acid extracted from Staphylococcus aureus.

Using MSPRIA, specific IgE antibodies to SCWP were detected at significant levels in all 5 patients with HIE, but not in 23 patients with atopic allergy or Wiskott‐Aldrich syndrome with or without staphylococcal infections. IgG antibodies to SCWP and teichoic acid were lower in the patients with HIE than those in non‐allergic infectious controls. These findings suggest an abnormal immunologic response to Staphylococcus aureus in patients with HIE.
A Scoring System for the Diagnosis of Lysosomal Storage Diseases: A Preliminary Study on the Basis of 100 Cases
Authors: Okamura‐Oho Yuko , Yamanaka Tatsuhiro , Suzuki Yoshiyuki
Published: October 2007

The clinical records of 515 patients for the biochemical diagnosis of lysosomal diseases were reviewed with regard to 22 major clinical manifestations and laboratory data, and a comparative study was performed between lysosomal diseases (100 patients) and other nonlysosomal diseases with similar clinical manifestations (415 patients). The incidences of the 22 clinical findings were calculated in each group, and the relative ratio of the incidences in these 2 groups was designated as a positive sign index. The scores of this index were found to be specifically high in some clinical data of the patients with lysosomal storage diseases. The total scores clearly discriminated between these 2 disease groups, although there was a small overlap between them. This preliminary study can be expanded to diseases of other categories and assist the clinical diagnosis of rare diseases by general pediatricians.
Early Onset Type of NADH‐CoQ Reductase Deficiency
Authors: Kobayashi Masanori , Morishita Hideko , Sugiyama Naruji , Yokochi Kenji , Wada Yoshiro , Okada Masato , Hotta Yasuaki
Published: October 2007

We report a case of NADH‐CoQ reductase deficiency, which is the first case described in Japan. A 3‐year‐and‐9‐month‐old‐boy had a severe and progressive neurologic disorder characterized by psychomotor retardation, myoclonic epilepsy and spastic quadriplegia. Moreover he had a respiratory problem of uncertain etiology in the neonatal period. Venous blood lactate levels were strikingly increased, ranging from 16.8 to 47.4 mg/100 ml, with an abnormally high lactate‐pyruvate ratio (21.2±1.7). Lactate and pyruvate levels in the cerebrospinal fluid (CSF) were also elevated (48.9 mg/100 ml and 2.09 mg/100 ml respectively). Histochemical investigation of a muscle specimen showed marked atrophy of type 1 and 2A fibers. No ragged red fiber was seen. Electron microscopy revealed a mild increase of lipid vacuoles among the myofibrils. The mitochondria showed no abnormal structure and no paracrystalline inclusion body. Biochemical studies of muscle mitochondria revealed a defective activity of NADH‐CoQ reductase in the respiratory chain. We propose that cases with so‐called mitochondrial myopathy should be classified according to the fundamental biochemical defects underlying of the clinical symptoms, and that the entity of NADH‐CoQ reductase should be divided into two further subtypes, of which one is early onset type and the other late onset.
Experience with Steroid Pulse in Chronic Inflammatory Diseases
Authors: Shimizu Makoto , Banba Masahiro , Shirane Kensuke , Kato Makoto , Awazu Midori , Hojo Hideto , Osano Mitsuru
Published: October 2007

Steroid pulse was employed in the treatment of three pediatric patients wit juvenile rheumatoid arthritis, dermatiomyositis and Behçet's disease, respectively. Initially, frequent of steriod pulse course were employed, in an attempt to achieve prompt relief of the acute exacerbations of chronic inflammatory disease. Thereafter, M‐PSL pulse at 20–30mg/kg was carried out once every 3–8 weeks for 3–4 years at the outpatient clinic. All patients were able to lead a comfortable life, with minor symptoms, and they had neither cushingoid features nor growth retardation. The hypothalamopituitary‐adrenal system was also retained, and significant immunological abnormalities were not detected in any case, even after prolonged and repeated steroid pulse courses.
The Influence of Parental Toy Choice on Child Toy Preference and Sex‐Role Typing
Authors: Peretti Peter O. , Sydney Tiffany M.
Published: October 2007

Toys are associated with a child's expression, fantasy, interest, exploration, construction, education, cognitive development and sex‐role learning. The present research was concerned with the influence of parental toy choice on child toy preference and sex‐role typing among nursery school children. The results indicated that child toy preference was found to be significantly related to parental toy preference. Moreover, the parental toy preference was found to be associated with sex‐role typing.
Increased Synthesis of Thromboxane A2 by Platelets from a Patient with Hemolytic Uremic Syndrome
Authors: Hidaka Toshihiro , Ogura Ryohei , Furuno Hiroaki , Tanaka Chihei , Yamashita Fumio
Published: October 2007

Increased thromboxane synthesis capacity was demonstrated in platelets from a patient with hemolytic uremic syndrome by the measurement of [1‐14C] TxB2 converted from [1‐14C] arachidonic acid. Whether such potentiated platelets are a possible risk factor of this disease is discussed.
Infantile Hemangioendothelioma Associated with Bone Marrow Dysfunction
Authors: Horikoshi Tamaki , Kimura Ken , Hiyoshi Yasuhiko , Ota Masanori , Dai Shunichi , Irie Yasushi , Yano Hiromichi
Published: October 2007

A 5‐month‐old‐girl had hepatomegaly and severe anemia. Based on the multiple imaging studies, hemangioendothelioma was suspected. Wedge resection of the liver and hepatic artery ligation were performed. The peripheral blood film showed pancytopenia, and the myelogram showed markedly reduced erythroid series and myeloid predominance with shift to the left. The correlation between hemangioenothelioma and bone marrow dysfunction is unknown.

As far as we know, this is the first report of infantile hemangioendothelioma associated with bone marrow dysfunction.
Glomerulonephritis Associated With Hepatitis B Virus Infection in Children
Authors: Takekoshi Yasuo , Tanaka Masato , Nagata Yasushi , Tochimaru Hiroyuki , Oka Ryuji , Matsumoto Shuzo
Published: October 2007

Hepatitis B surface antigen (HBsAg) was identified by a reversed passive hemagglutination (RPHA) method in the serum of 13 of 15 patients with membranous glomerulonephritis (MGN), three of three Type III membranoproliferative GN (MPGN) proposed by Burkholder, three of 25 idiopathic minimal change nephrotic syndrome (IMCNS), three of 37 minor glomerular alterations with undetermined diagnosis (MGA) and none of 79 various other renal disease, in 266 Japanese children.

Immunofluorescent studies showed granular deposition of Hepatitis B e antigen (HBeAg) in a similar pattern to that of IgG and β1C in the kidney of four patients with MGN, and of HBsAg in one with Type III MPGN. Eight of 13 MGN with positive serum HBsAg, two of three Type III MPGN and one of one MGA with positive serum HBs Ag had normalization of urinary findings during the observation period of one to eight years.

Eight (5 MGN, 2 Type III MPGN and 1 MGA) of those with positive serum HBeAg showed seroconversion of HBeAg, and anti‐HBe appeared in five of eight as the urinary findings became normal. The normalization of urinary findings accompanied by seroconversion of HBeAg in the patients with MGA and with Type III MPGN suggests that HBeAg is the most important antigen involved in HB virus (HBV) associated GN including not only MGN but also Type III MPGN and MGA.
Pathogenesis of Persistent Truncus Arteriosus Produced by Bis‐Diamine in Rats
Authors: Okishima Takahiro , Ohdo Shozo , Hayakawa Kunio
Published: October 2007

The influence of bis‐diamine on truncoconal septation was studied by the administration of 200 mg of this drug to pregnant Donryu‐strain rats on day 10 of gestation. Fetuses were removed at nine time periods from day 11 to 15. Some hearts were observed under a scanning electron microscope; others were serially sectioned and observed by light microscopy. In the treated group, the aorticopulmonary septum and the 6th aortic arch artery were hypoplastic or aplastic. Truncal swellings and conal ridges showed hypoplasia and failure to fuse. Various types of persistent truncus arteriosus were found in association with the degree of development of the 6th aortic arch artery and the aorticopulmonary septum. Fused 6th aortic arch arteries corresponded to Collett and Edwards' persistent truncus arteriosus type II, and non‐fused arteries to type III. Aplastic arteries belonged to type IV or were unclassifiable.
Initial Chemotherapy for Purulent Meningitis in Children
Authors: Kobayashi Yutaka
Published: October 2007

The combination of ampicillin with either gentamicin or chloramphenicol, which is currently the initial chemotherapy for purulent meningitis, has lost its effectiveness in recent years because of an increase in the incidence of ampicillin‐resistant strains. This has made it necessary to search for a suitable substitute therapy.

Twenty‐two new β‐lactam agents were compared with penicillin G and ampicillin in terms of their antibacterial activity in relation to the principal causative microbes of meningitis and their ability to transfer into the cerebrospinal fluid (CSF) of rabbits with experimental staphylococcal meningitis. In addition, a survey was conducted of the therapeutic efficacy achieved by these drugs in cases seen by the authors and in other domestic and overseas cases. Finally, an investigation was made as to whether or not the transfer of the drug into the CSF was suppressed when it was administered simultaneously with ampicillin.

From the results, it was surmised that in future the most appropriate initial chemotherapy for purulent meningitis will be a combination of cefotaxime, or perhaps ceftriaxone, plus ampicillin.
Staphylococcal Infections and Chemotherapy in Children
Authors: Toyonaga Yoshikiyo , Hori Makoto
Published: October 2007

Staphylococcal infections in childhood are of great inportance. Although serious diseases caused by staphylococci, such as septicemia and meningitis, have been decreasing in recent years, the mortality of these diseases is increasing. In this connection, it should not be overlooked that, in addition to increases in the number of cases with methicillin‐cephem‐resistant S. aureus (MCRSA) and multi‐resistant strains and of cases with acquired immuno‐deficiency due to medical treatment, an increasing emergence of coagulase‐negative staphylococci (CNS) as the etiology, due to such treatments as intratracheal intubation and VP‐shunt, and increases in the number of cases with toxic shock syndrome are encountered.

MCRSA, which is at issue in recent years, is in many cases highly resistant, except to minocycline (MINO), fosfomycin (FOM) or vancomycin (VCM). Quite a few strains with good sensitivity to cefmetazole (CMZ) and cefamandole (CMD) of the cephalosporins (CEPs) have been noted. The antibacterial effect of CEPs of groups 1 and 2 including cephalothin (CET) is known to be excellent and likewise the effect of CMZ and MINO has proved also to be excellent.

Therefore, in order to cope with the staphylococcal infections in the pediatric field, it is necessary to understand the real status of the resistance of bacteria to chemotherapeutics and aspects at issue in recent years and to take adequate measures from the viewpoint of the host‐parasite drug relationship.
Recent Chemotherapy for Child Pneunomia and Empyema
Authors: Uehara Suzuko , Nakamura Akira , Himi Kyoko , Namba Mami , Matsumura Chieko , Suzuki Hiroshi , Ishikawa Nobuyasu
Published: October 2007

Recent progress in chemotherapy has contributed to the remarkable decrease in mortality from pneumonia in infancy and childhood; however, wide use of antimicrobials has resulted in incapability of etiologic diagnosis. Appropriate chemotherapy for pneumonia should be initiated based on the relative frequency of causative organisms related to age groups, and their antimicrobial susceptibility. Therapeutic development should be reevaluated by the pathogens isolated prior to antimicrobial administration.

S. aureus is the most important causative agent in fatal cases of pneumonia. Judging from our studies on offending bacteria in bronchopulmonary infections, H. influenzae, S. pneumoniae and S. aureus are the major pathogens in pneumonia. Therefore, the main initial antimicrobials in uncompromised infants and children should be ampicillin (plus penicillinase‐resistant penicillin) covering these organisms. Methicillin‐resistant S. aureus, ampicillin‐resistant H influenzae and penicillin‐resistant S. penumoniae are of great concern. Chemotherapy for these strains is discussed. Treatment with macrolides is mainly recommended for M. pneumoniae. Chemotherapeutic results of treatment of C. trachomatis will be elucidated in a few years.
Chemotherapy of Urinary Tract Infection in Children—Especially Chemotherapy in the Acute Stage—
Authors: Iwai Naoichi
Published: October 2007

Chemotherapy in the acute stage of urinary tract infection in children in Japan, is discussed in regard to the actual state of major causative organisms and the drug susceptibility.

The most important thing in the selection and administration of the drug used is to have a good understanding of severity and course of the patient's illness, his state of renal function, presence of underlying disease, etc., and a good knowledge of the characteristics of various drugs as well as the differences between them. In most cases, it seems proper to start the initial treatment by selecting those drugs which are considered appropriate from among the first generation cephems, the second generation cephems and synthetic agents, and in very severe cases, from among the second generation cephems or the third generation cephems, and to change to the most suitable drugs when the results of drug susceptibility tests become known.
Antimicrobial Chemotherapy for Children with Malignancy
Authors: Kamiya Hitoshi , Arai Syojiro , Ota Yutaka , Inoue Masakazu , Bessyo Fumio , Hirotsu Takuo , Akatsuka Junichi , Sakurai Minoru
Published: October 2007

Infection is the major complication occurring in children with cancer and is often fatal.

Since the clinical manifestations of infection are slight, except for fever, a definitive diagnosis is difficult in the early phase of infection, but using laboratory parameters, the differentiation of bacterial infections may be possible to some extent. The infection cannot be treated only by antimicrobial chemotherapy, but supportive measures such as neutrophil transfusion, and other biological medications should be given in consideration of the deficiency of the immune defense mechanisms of the patient. Anticancer treatment is also needed, along with antimicrobial treatment.

The prophylactic use of antimicrobial drugs is still controversial but it is effective, depending on the type of infection for which it is used, and on the status of the defective defense mechanism in the patient who is on cancer chemotherapy.
Detection of Cytomegalovirus‐Specific RNA in Frozen Tissue Sections by Biotin‐Labeled Hybridization Probe
Authors: Eizuru Yoshito , Mihara Mari , Minamishima Yoichi , Tsunematsu Yukiko , Okabe Nobuhiko
Published: October 2007

A patient with neuroblastoma developed cytomegalovirus (CMV) pneumonitis and died. At autopsy, CMV was isolated from lung, liver, kidney and submandibular gland. With the biotin‐labeled CMV DNA probe, CMV‐specific RNA was detected in all the organs tested, including the spleen which was negative for virus isolation but positive for CMV‐specific antigens.
Effect of Growth Hormone on Lipoprotein Metabolism in Male Pituitary Dwarfs: Changes in Lipoprotein Lipase Activity and Cholesterol Level in HDL Subfractions
Authors: Asayama Kohtaro , Miyao Akiyo , Bradley Charles A. , Kato Kiyohiko , Burr Ian M.
Published: October 2007

To determine the effect of a chronic therapeutic dose (0.24 IU/kg/week) of growth hormone on the cholesterol concentration in serum lipoproteins and postheparin plasma lipase activities in previously untreated children with idiopathic growth hormone deficiency, seven male patients under 10 years of age were studied before and three months after the initiation of growth hormone therapy. Lipase activity was measured by an immunochemical method. The cholesterol concentration in high‐density lipoprotein subfractions was determined, using a micromethod for ultracentrifugation. Serum total cholesterol and triglyceride levels did not change significantly during treatment, while the high‐density lipoprotein cholesterol level decreased significantly after treatment to 79% of the baseline level, with an equal decrease of cholesterol concentration in both high‐density lipoprotein2 and 3. Lipoprotein lipase, but not hepatic triglyceride lipase, activity decreased significantly after treatment. These were not associated with changes in either thyroid status as determined by serum triiodothyronine levels or insulin responsiveness after oral glucose challenge, suggesting that these changes may be induced by a direct action of growth hormone.
Effect of High‐Carbohydrate Diet on Lipid Metabolism in Young Type I Diabetics: Transient Changes in High‐Density Lipoprotein2 and Lipoprotein Lipase
Authors: Asayama Kohtaro , Miyao Akiyo , Bradley Charles A. , Lorenz Rodney A. , Kato Kiyohoko , Burr Ian M.
Published: October 2007

The short‐term effect of a high‐carbohydrate, low‐fat diet on lipoprotein metabolism was evaluated in eight young patients with type I diabetes mellitus. An isocaloric exchange diet containing 60% carbohydrate and 20% fat was administered for four weeks and serum lipid, high‐density lipoprotein and subfraction cholesterol, and the activity of the lipolytic enzymes in postheparin plasma were monitored. The assay methods for lipase activity and cholesterol concentration in high‐density lipoprotein subfractions were described previously. There were no appreciable changes in diabetic control, serum triglyceride and total cholesterol levels. The high‐density lipoprotein2 cholesterol level decreased significantly after two weeks on the diet and returned toward baseline levels thereafter. There was a significant correlation between high‐density lipoprotein2 cholesterol level and lipoprotein lipase activity. The high carbohydrate diet did not affect the glycemic control but induced a transient decrease in high‐density lipoprotein2 cholesterol mediated by the decrease in lipoprotein lipase activity.
Changing Panorama of Cerebral Palsy in Children of Low Birth Weight
Authors: Maekawa Kihei , Suga Yasumasa , Tsuzura Shigenobu
Published: October 2007

Of 2,840 out‐patients who visited the Tokyo Metropolitan Kita Rehabilitation Center in 1965, 1968, 1970, 1973, 1975 and 1978, 278 children with cerebral palsy (CP) of low birth weight were studied to analyse changes in type of CP in relation to gestational week, birth weight, perinatal factors and subsequent complications.

1. The incidence of handicap associated with low birth weight in new out‐patients had not changed.

2. The number of doubly handicapped CP patients was gradually increasing while number of uncomplicated CP patients was gradually decreasing.

3. The number of children with tension athetosis was remarkably decreasing; the number of children with spastic quadriplegia was gradually increasing. Increase in number of the spastic type was found especially in the group whose birth weight was over 1,500 gm. and gestational week over 37 weeks. The numbers of spastic diplegia and hemiplegia patients had not significantly changed.

4. In the athetoid group, the complication of severe jaundice was apparently decreasing since 1970 while the number of asphyxia cases was gradually increasing. In the spastic group, there were no remarkable changes in the perinatal complications.

5. Of spastic quadriplegia patients 78.3% were of IQ of less than 67 while 33.3% of spastic hemiplegia and 23.5% of spastic diplegia patients had IQs of less than 67.31.4% of the athetoid type had IQs of less than 67.

6. In the asphyxia group, 34 of 82 children with low birth weight CP (41.5%) had epilepsy.

Our results suggest that the causes of brain damage in low birth weight infants are not merely preventable perinatal factors, but are due to a more complex interaction of prenatal and perinatal factors.