bluuebunny/crossref_metadata_2025 huggingface dataset:
Correlative Study of Radioreceptor Assay and Radioimmunoassay of Serum Growth Hormone (GH): Screening for Bioinactive GH in Short Statures
Authors: Igarashi Noboru , Minami Satoshi , Kajiwara Sohei , Sato Tamotsu
Published: October 2007

The radioreceptor assay (RRA) of human growth hormone (hGH) was developed using liver microsomal fraction from estrogen‐treated female rats. The method allows measurement of physiological amounts of hGH (1.5–100 ng/ml) in unfractionated serum. Cross‐reactivity with human prolactin was 4.6%. Native hGH, recombinant methionyl‐hGH, and methionine‐free hGH showed a similiar binding to the receptors. Simultaneous determinations of serum hGH concentration with the RRA and radioimmunoassay (RIA) were performed in samples after the administration of GH‐releasing hormone (GRH) (1—44) from 15 normal short children and 25 patients with hGH deficiency. Overall RRA/RIA ratio in 146 samples was 0.84 ± 0.31 (SD). Of 15 normal short children, one showed a significantly reduced RRA/RIA ratio of 0.13 ± 0.03 in comparison with that of the other (0.70 ± 0.40) (p<0.001). His growth rate was accelerated by exogenous hGH administration, indicating that he secreted a biologically inactive hGH. The present RRA provides a useful method for screening bioinactive hGH in a large number of serum samples from children with short stature.
Classification of Type 1 and Type 2 Diabetes Mellitus from Studies of ICA, HLA and Insulin Secretory Capacity
Authors: Matsuura Nobuo , Fujieda Kenji , Mikami Yuhei , Fujita Hiroko , Harada Shohei , Wakisaka Akemi , Shinjyo Takamichi , Hirata Yukimasa
Published: October 2007

We studied ICA, HLA and insulin secretory capacity in 87 children with positive urinary screening and more than 2 points in the oral glucose tolerance test in order to establish criteria by which they could be classified into type 1 or type 2 diabetes mellitus. Fifty‐five non‐obese, ketosis‐prone insulin dependent diabetic children were used as controls for type 1 diabetes mellitus. Our conclusions were as follows:

1. Type 1 diabetics were non‐obese (on insulin therapy), ICA positive, ketosis‐prone, had an insulin secretory capacity (Z IRI) of less than 100/nU/ml, and most of them possessed HLA‐Bw54‐DR4 or DRw9, DRw53 but did not possess Bw52‐DR2 haplotype.

2. In the patients who were treated by dietary regimens alone for certain periods, however, insulin secretory capacities gradually deteriorated and they finally became insulin dependent. The children of this group who were not obese during insulin therapy and possessed an HLA haplotype identical to that in type 1 diabetes, regardless of ICA, might be classified as having slowly progressive type 1 diabetes.

3. The major difference between type 1 and slowly progressive type 1 diabetes was a family history of diabetes. Genetic factors might modify the clinical course of type 1 diabetes mellitus.

4. If the sensitivity of ICA or related autoantibodies to islet cells can be detected more readily, it should become easier to distinguish between type 1 and 2 diabetes.
Intensive Insulin Therapy—Effects on M‐Value and Frequency of Hypoglycemia—
Authors: Tanae Ayako , Hashimoto Nobuko , Kagawa Jiro , Hibi Itsuro
Published: October 2007

The frequency of hypoglycemia and M‐values were measured on 11 intensively treated insulin dependent diabetic children separated into three groups of once‐daily, twice‐daily and multiple daily insulin injection regimens. Despite a similar quality of blood glucose control (Hb Al (%): 10.0 ± 1.5, 10.4 ± 2.6, 10.6 ± 2.5) in each of the above regimens, the M‐values were 26.2 ± 11.7 in the once‐daily, 34.9 ± 17.9 in the twice‐daily and 47.7 ± 14.7 in the multiple daily injection regimen (M ± SD), respectively. A mild biochemical hypoglycemia was found more often in the patients under treatment with twice‐daily and multiple daily injections. Further, though the mean blood glucose profiles in the patients treated with once‐daily and multiple daily injections were very good, marked hypoglycemic blood glucose levels were seen prior to meals, especially before lunch.
Use of a Computer Program in the Treatment and Education of Young Diabetics
Authors: Laron Z. , Faiman G. , Flexer Z. , Rapaport M.
Published: October 2007

The goal of diabetes therapy is to achieve normal blood glucose (BG) levels to prevent acute and chronic complications. The ascertainment of BG during the day is done using glucometers. There are three major impediments: a) compliance to test for long periods; b) overview of results; c) cost of meter and chemistrips. We have developed systems which help overcome the problem. Treatment by a multidisciplinary team (MDT) including social workers, psychologist and education nurse have increased motivation and the patients perform a mean (± SD) of 2.8 (±2.3) tests/day even in the second year. The development of a computer system (DIACON) enabled a rapid accurate statistical analysis of data on self blood glucose monitoring (SBGM), food intake and insulin dose. The patient feeds the computer the SBGM data and receives histograms and tables presenting the mean ± SD of BG levels for the requested periods. The dietician feeds the dietary data which provides a break‐up into CHO, fats and proteins as well as the distribution in percentage of CHO intake during the day. The physician formulates his recommendation in accordance with data analysis.
Application of Computer Programs in the Management of Diabetic Children
Authors: Niihira Shizuhiro , Inada Hiroshi , Tei Seiko , Aono Mayumi , Aono Shigeo , Isshiki Gen
Published: October 2007

Microcomputers have been used in our hospital for the past five years to summarize data from patients and to remind us to carry out routine tests. Recently, we have shown computer data on the screen to patients; this helps us to explain and discuss the monitoring of their condition with them.

Unlike some other systems, general data as well as data on self‐monitored blood glucose levels are recorded in our computer system. The data consist of five categories: personal profile, treatment, etiological research, indices of control and data for checking complications.

The analysis and evaluation of our system are done by the modes of display, statistical calculations, early detection of complications and graphics.

The system is compatible with input from a keyboard, optical character readers and interface to a glucose meter with a memory.
Development of Histidinemic Patients: Follow‐Up Study of Five Cases with Neuropsychological Symptoms
Authors: Ishikawa Michiko , Saito Hisako , Morishita Hideko , Ito Tamiko , Kato Taiji , Wada Yoshiro
Published: October 2007

We described the general clinical course of histidinemia in a previous report. Here, we report on five patients having particular problems in the developmental aspects.

Case 1 was found to have histidinemia at the age of 3 years during a clinical survey of the nature of his neurological symptoms. He has been followed up for seven years. He developed poorly in verbal expression.

Case II was detected by newborn mass screening. The psychomotor development was apparently normal, but in detail, the development of fine motor and cognitive function was insufficient during the follow‐up period. Consequently, a developmental lag in visual perception, visual motor perception and spatial perception were noted in this case.

Cases III, IV and V were each 3 years old, and were also diagnosed as histidinemic by newborn mass screening test; scanty attachment and various kinds of autistic behavior were noted during their clinical course.

These five cases were clinically different from other non‐symptomatic patients with histidinemia.

It is important to study the pathogenesis of such psychomotor abnormalities from the viewpoint of psychological care for the children.
Exercise‐Induced Bronchospasm in the XXIII Summer Olympic Games
Authors: Pierson William E. , Voy Robert O.
Published: October 2007

The U.S. Olympic Team demonstrated a significant prevalence of E.I.B. among world‐class athletes (15.1). Nearly all sports were involved but endurance events seemed to have the highest prevalence of team members with E.I.B. Resting pulmonary function studies were poor discriminators of athletes affected with E.I.B. The 67 athletes of the 597 member team were responsible for winning 41 Olympic medals including 15 golds and 21 silvers, clearly demonstrating their ability to compete and succeed in world‐class competitive events. These results should send a strong message to young people that they can strive to excel in sports with proper medication and management.
Part 1. Outcome for Small‐for‐Gestational‐Age Infants with Very Low Birth Weight (<1,500g)
Authors: Yamaguchi Kiyoko , Hara Hitoshi , Nishida Hiroshi , Yamada Takako , Arai Toshihiko , Nose Koichiro , Nakabayashi Masao , Takeda Yoshihiko , Sakamoto Shouichi , Fukuyama Yukio
Published: October 2007

Mortality and morbidity of small‐for‐gestational‐age (SGA) infants with very low birth weight (VLBW) (less than 1,500 g at birth) are reported. Survival rate of 47 VLBW infants was 92%, with no statistical difference between SGA and appropriate‐for‐gestational‐age (AGA) groups. Thirty‐nine VLBW infants were watched in our follow‐up clinic for more than seven months. Overall incidence of major neurologic defects (cerebral palsy, epilepsy or psychomotor retardation) was 12.8%. Handicap was significantly different for SGA and AGA groups, that is, four out of 12 SGA infants (33%), compared with one out of 27 AGA infants (3.3%). Major neurologic defects in VLBW infants were strongly associated with intrauterine growth retardation (IUGR) related to toxemia of pregnancy with onset in the 2nd trimester. Although most of the VLBW SGA infants were IUGR due to noxious intrauterine environments, head sizes of all, with one exception, were growth‐restricted in utero. If IUGR, especially retardation of head growth, is detected in utero during the 2nd trimester, discussion between obstetrician and neonatologist about optimal neurological outcome for the infant is mandatory.
Effect of Single Dose Intravenous Animophylline in Asthma
Authors: Pyun Bok‐Yang , Odajima Yasuhei , Kishida Masaru , Obata Toshihiko , Nagakura Toshikazu , Likura Yoji
Published: October 2007

Fifty‐five children with acute asthma were studied to evaluate the relationship between pulmonary function (improvement in forced expiratory volume in one second, FEVj, and maximum flow at 50% vital capacity, V50) and serum theophylline concentration after intravenous aminophylline.

Serum theophylline concentrations and pulmonary function were measured before and after aminophylline therapy.

In our study, there was significant improvement in pulmonary function in patients whose baseline serum theophylline concentration was under 5 ng per ml, when their post‐infusion levels reached 10 to 15 μg per ml. Significant improvement in pulmonary function was noted in subjects whose baseline FEV1 was under 50% of the predicted value when their post‐infusion serum theophylline levels rose to 10 to 15 μg per ml.
Retinoblastoma and Osteosarcoma: The Prototypic Cancer Family
Authors: Hansen Marc F. , Cavenee Webster K.
Published: October 2007

Mutant alleles of the RBI locus on human chromosome 13q 14 have previously been linked to the development of retinoblastoma, a tumor of embryonal neural retina. In the bilateral form of the disease, inheritance of a germinal mutation at this locus predisposes each retinal cell to a subsequent second somatic event which results in the formation of the retinoblastoma tumor. Spontaneous unilateral tumor occurrence appears to result from two sequential somatic events. Survivors of the bilateral form of this eye tumor are at substantially increased risk for subsequent development of second primary cancers, particularly osteosarcoma, a bone cancer. Conversely, survivors of unilateral retinoblastoma exhibit the same likelihood of development of osteosarcoma as the general population.

In order to determine whether osteosarcoma and retinoblastoma share a common pathogenetic mechanism, we determined restriction fragment length alleles at loci on chromosome 13 in DNA from constitutional tissues of bilaterally affected retinoblastoma patients as well as from their osteosarcoma tumors. These analyses indicated that the loss of constitutional heterozygosity in the tumors occurred specifically for chromosome 13 and appeared to involve the same chromosomal region as that previously identified in retinoblastoma tumors. Similarly, specific loss of constitutional chromosome 13 heterozygosity was also apparent in sporadic osteosarcomas. These results suggest that both diseases are due to the mitotic unmasking of pleitropic recessive mutant alleles at the RBI locus, and that the clinical occurrence of mixed cancer families such as these may be due to the differential expression of a single recessive mutation.
Cloning of Human Thymic Subcapsular Cortex Epithelial Cells with SV40 Ori‐Gene
Authors: Mizutani Shuki , Watt Suzanne M. , Robertson David , Hussein Shabir , Healey Lyn E. , Furley Andrew J.W. , Greaves Melvyn F.
Published: October 2007

The thymic microenvironment involves complex cell interactions among different types of epithelial cells, macrophages, tissue histiocytes and immature and maturing T cells. We describe the isolation of a subset of thymic epithelial cells by selective primary culture followed by SV 40 ori‐and psV‐2 neo co‐transfection. The cloned cells were characterized as subcapsular cortex epithelial cells having hemopoietic growth factor activity, CSF‐1 and LFA3 associated binding sites for T cells. Thus cotransfection with the selectable genes and the gene which gives growth advantage to the transformant enabled us to investigate the rare type of cells which has been difficult to isolate by ordinary tissue culture technique because of mixed cellularity and fibloblast overgrowth.
Cytotoxicity of Mixed Human Monoclonal Antibodies Reacting to Tumor Antigens
Authors: Hagiwara Hideaki , Aotsuka Yasuyuki
Published: October 2007

Two kinds of human monoclonal antibodies, CLN‐IgG and SLN‐IgG, were obtained from human x human hybridomas, termed CLNH11 and SLNF10, respectively. These antibodies recognized different molecules which were expressed simultaneously on the cell surface of tumor cells. When both antibodies were added into culture medium in the presence of complement, a remarkable synergistic cytotoxic‐effect against glioblastoma cells was observed.
The New Pediatrics in Japan
Authors: Tokumaru Minoru
Published: October 2007

Pediatrics has arrived at a turning point. When diseases in children and the trend of demand for health care in Japan are observed, new fields are expanding which differ from traditional pediatrics, which set its sights on reduction of the death rate and control of infectious diseases; the new pediatrics aims at functional recovery and optimal growth and development, and involves what has to be done mainly in outpatient clinics. Pediatricians who work in these institutions must make their efforts in the ambulatory field and establish a form of ambulatory pediatrics which meets the needs of our “softening” society. Our efforts are necessary for the well‐being of our juniors. I present a framework for the new ambulatory pediatrics and enumerate its various tasks.
Intravenous Gamma Globulin for Prolonged Passive Immune Thrombocytopenia
Authors: Hara Toshiro , Kukita Johji , Yarnashita Hironori , Mizuno Yumi , Ueda Kohji , Shimokawa Hiroshi , Nakano Hitoo , Kishida Kunio
Published: October 2007

The effectiveness of intravenous gamma globulin was evaluated in prolonged thrombocyto penia in two infants of mothers with autoimmune thrombocytopenic purpura. Case 1, de spite unresponsiveness to steroid therapy, showed a slight response to intravenous gamma globulin infused at 15–19 days of age. A second administration of intravenous gamma globu lin at 70–74 days of age completely reversed the thrombocytopenia. In case 2, passive immune thrombocytopenia persisting over one month was reversed by the administration of intravenous gamma globulin at 37–39 days of age. A comparison of the effects of intra venous gamma globulin on the increase in platelet counts in the newborn infant (case 1) and in his mother showed that passive immune thrombocytopenia in neonates can be more resistant to therapy than autoimmune thrombocytopenic purpura in the mother. Adverse effects were not observed in either case. The effectiveness of and indications for intravenous gamma globulin in passive immune neonatal thrombocytopenia are discussed.
Studies on the Effect of Long‐Term Use of Low Dose Aspirin in Kawasaki Disease
Authors: Hamasaki Yuhei , Ichimaru Tomohiro , Tasaki Hakaru , Miyazaki Sumio
Published: October 2007

The inhibitory action of long‐term low dose aspirin (1–2 mg/kg/day for over 10 months) on the cyclooxygenase pathway in platelets and vascular endothe‐lium was evaluated in 10 patients with Kawasaki disease. The results were compared with those obtained after taking aspirin at 5–10 mg/kg/day during the acute phase of the illness. Platelet aggregations induced by adenosinedi‐phosphate (ADP), epinephrine and collagen were inhibited by aspirin doses of 1–2 and 5–10 mg/kg/day, when compared with those of controls (p < 0.05). Platelet synthesis of thromboxane B2 (TXB2) under doses of 1–2 and 5–10 mg/kg/day was 0.57 ± 0.07 and 0.72 ± 0.09 ng/ml platelet‐rich plasma (PRP) /105 platelets, respectively (p > 0.1). These values were significantly lower than those of the control group (22.88± 3.42 ng/ml PRP/105 platelets) (p < 0.05). No differences were found in platelet aggregation and TXB2 productivity between the two aspirin doses. Levels of 6 keto‐prostaglandin F1α (6k‐PGF1α) in platelet‐poor plasma (PPP) did not differ significantly in these 3 sets of data. The results indicate that long‐term administration of low dose aspirin (1–2 mg/kg/day) inhibits platelet aggregation by inhibiting synthesis of thromboxane A2 (TXA2), without interfering with prostacyclin production probably in the endothelium of blood vessels.
Cohen's Syndrome with Diabetes Mellitus
Authors: Nambu Mitsuhiko , Oshima Yusei , Kakiuchi Toshitaka , Hayakawa Yasushi , Ito Tadashi , Hasegawa Tomoko , Fujita Hiroko
Published: October 2007

We report a case of 12‐year‐old girl with Cohen's syndrome (CS) associated with diabetes mellitus (DM). She had the characteristic facies, with a short philtrum, an open‐mouth appearance, prominent maxillary central incisors, high palate, mild maxillary hypoplasia and micrognathia. Other features included truncal obesity, short stature, mental deficiency, small hands and feet, and accentuated thoracic kyphosis. She had been hypotonic during the neonatal and early infantile periods. Laboratory examinations showed hyperglycemia and glucosuria. HbAl and HbA1 c levels were raised. A 75‐g oral glucose tolerance test (OGTT) showed glucose intolerance consistent with DM, but there was a normal level of urinary C‐peptide. There were no islet cell antibodies (ICAs) and no history of ketoacidosis. This is the first reported case of CS with non‐insulin‐dependent diabetes mellitus (NIDDM).
Outcome of the Patients Detected by Newborn Screening in Japan
Authors: Aoki Kikumaro , Wada Yoshiro
Published: October 2007

A follow‐up study of the cases detected by a newborn mass‐screening program has been performed during 1977–1985 by a collaborative study group.

The number of hyperphenylalaninemic patients detected was 148, yielding a calculated incidence of one in 82,000. Of these patients, 102 cases were confired as classical PKU, 37 cases as hyperphenylalaninemia, and 9 cases as biopterine deficiency. The ratio of biopterine deficiency to PKU was about 9%. The incidence of maple syrup urine disease was estimated as one in 580,000, and the incidence of homocystimuria and galactosemia was found to be markedly low. Our survey has accumulated 1,362 cases of histidinemia establishing an incidence of one in 9,000. We have evaluated a large‐scale base to study the cad relation of histidinemia to mental development. It seems that low DQ or IQ scores of some histidinemic patients do not correspond to the blood histidine level.
Cognitive Development in Children with Inborn Errors of Urea Synthesis
Authors: Msall Michael , Monahan Peggy S. , Chapanis Natalia , Batshaw Mark L.
Published: October 2007

Children with congenital urea cycle disorders are at a high risk for cerebral damage, mental retardation and other developmental disabilities. Intellectual outcome appears to be correlated with the severity of the underlying cerebral damage and the duration of hyperammonemic coma. Thus, if a good outcome is to Le possible, early diagnosis and treatment is essential. However, even prospective treatment of affected children may not prevent cognitive impairment and even asymptomatic hyperammonemia may have subtle effects on intellect.
Acute and Chronic‐Intermittent Isovaleric Acidemia: Diagnosis and Glycine Therapy
Authors: Elsas Louis J. , Naglak Mary
Published: October 2007

Isovaleric acidemia is caused by an inherited defect in isovaleryl‐CoA dehydrogenase (E.C. 1.3.99.10). L‐leucine restriction and glycine supplementation are used to decrease the toxic accumulation of isovaleric acid (IVA). Supplemental glycine augments conversion of IVA to isovalerylglycine gVG) through the alternate pathway glycine‐N‐acylase (E.C. 2.3.1.13). Two clinical phenotypes are described, acute and chronic‐intermittent, and are differentiated by enzyme activity in cultured fibroblasts. In this study we determined the optimum therapeutic glycine supplement for treating isovaleric acidemia under stable conditions of restricted leucine intake as compared to oral leucine loading. Under stable clinical conditions and on a restricted leucine intake of 55 mg/kg/day a nine year old with chronic intermittent IVA excreted 12.3 ± 5.8 of IVG which rose to 33.6 ± 14.2 mmoles IVG/gm creatinine as glycine supplements were increased from 0 to 50 mg/kg/day at weekly intervals. Excretion of IVC plateaued between glycine intakes of 50–150 mg/kg, but unexpectedly fell to 13.8 ± 0.5 and 16.3 ± 0.9 mmoles/gm creatinine when glycine supplements were further increased to 300 and 600 mglkglday. IVG excretion increased in a two year old with acute IVA while ingesting 45 mg leucine/kg/day from 5.5 ± 2.8 on no glycine supplement to 10.6 ± 0.8 mmoleslgm creatinine on 600 mg/kg glycine supplements. IVG production was compared in the older patient with chronic intermittent IVA. During acute leucine loads, glycine supplements of 190 or 600 mg/kg/day, resulted in urinary IVG of 194 and 419 mmoles/gm creatinine, respectively.

These studies demonstrate that: acute and chronic‐intermittent isovaleric acidemia are differentiated with [1–14C] and [2–14C]‐leucine decarboxylation assays by intact fibroblasts; that glycine supplements of 150 to 250 mg/kg/day are optimum in both forms of the disease when patients are clinically stable but additional supplements will increase IVG production during acute leucine load conditions. However increases in glycine supplementation above 150 mg/kg/day may inhibit IVG production in patients with the chronic intermittent form of isovaleric acidemia, when intracellular IVA accumulation is minimized by diet, or if glycine‐N‐acylase is less competent.
Clinical Course of Hereditary Fructose Intolerance in 56 Patients
Authors: Endres Wolf , Sierck Tanja , Shin Yoon S.
Published: October 2007

The clinical course of hereditary fructose intolerance (HFI) was investigated retrospectively in 56 patients. The most frequently observed features were hepatomegaly, vomiting, diarrhea and failure to thrive. Fructose containing formulae given in the neonatal period lead in about one third of patients to cirrhosis of the liver — regardless of the amount ingested. Among patients receiving small amounts of fructose a mild clinical course was observed five times as often as a severe course. Two out of 12 patients who had developed cirrhosis of the liver, died soon after diagnosis in the early months of life. This stresses the danger of infants receiving fructose very early. Good dietary control brought serum transaminase activity to normal in half the time required with poor control. There was no correlation between dietary control and the duration of hepatomegaly.
Prediction of Prognosis in Patients with Hemolytic‐Uremic Syndrome
Authors: Matsurnoto Tohru , Ito Yuhei , Nakano Mitsurou , Yarnashita Yuji , Kinoshita Shouhei , Shindou Shizuo , Ph.D Norikazu Kuriya , Yamashita Furnio
Published: October 2007

Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia and acute renal insufficiency. Recently we experienced 6 HUS patients who had different clinical modes of onset, different clinical courses and a poor prognosis compared with the patients with the above typical clinical pictures. Thirteen HUS patients were evaluated in two aspects: mode of onset (acute or gradual type) and clinical corn (non‐recurrent or recurrent type).

During 4.9 ± 3.1 (mean ± sd) years' of observation, renal insufficiency was more frequently observed in the gradual onset and recurrent course types respectively than in the acute onset and recurrent types (p < 0.05, p < 0.01). Also, the mean age was significantly lower and platelet counts were much higher in the gradual onset type than in the acute onset type (p < 0.05, p < 0.01). However, clinical findings except renal failure revealed no differences between non‐recurrent and recurrent HUS. For the prediction of HUS, we emphasize the importance of classifying HUS by the mode of onset (acute or gradual) and/or clinical course (non‐recurrent or recurrent).
Deletion of the Distal Long Arm of Chromosome 1: An Analytical Approach May Contribute to the Diagnosis
Authors: Yamamoto Yoshifumi , Okamoto Noriko , Yanagisawa Masayoshi , Nishimoto Hiroshi , Kann Zuishyo , Niitsu Naoki
Published: October 2007

The clinical findings in a female infant with a terminal deletion (1) (pter→q42.3:) are described and compared with 18 other cases displaying a similar chromosomal abnormality. bed on an analysis of the 19 cases, postnatal growth retardation, microcephalus, severe mental and motor retardation, muscle hypotonia, high‐pitched shrill cry, and hypoplasia of the external genitalia in males were almost invariably observed. The facial features appear to be insufficiently peculiar to a blink diagnosis, but analytical demonstration of round face, sparse hair, broad nose, smooth philtrum, prominent cupid's bow and downturned comers of the mouth may contribute to the diagnosis. The frequencies of convulsions and CNS anomalies, especially of agenesis of the corpus callosum were relatively high.
Prognostic Significance of the Size of Coronary Artery Aneurysms in Kawasaki Disease
Authors: Akiba Tomoharu , Sato Tetsuo , Yoshikawa Masaru , Otaki Shinsuke , Kobayashi Yokio , Nakasato Mitsuru , Suzuki Hiroshi , Hayashi Tadashi
Published: October 2007

The prognosis of coronary artery aneurysms complicating Kawasaki disease was assessed by serial coronary angiography in 33 patients. They underwent initial angiography with four months, and one or two follow‐up angiographies between 11 months to 6.3 years after the onset of the illness. 'Ihese patients had 69 coronary aneurysms at the initial study. The maximal diameter of the aneurysms was expressed as a percent of the normal value. Of 44 aneurysms with a diameter less than 300% of normal, 43 were found to have regressed on follow‐up. Of 13 aneurysms with a diameter 300 to 400% of normal, four were proved to have regressed, eight persisted and one stenosed. Of 12 aneurysms with a diameter 400% of normal or more, one was found to have regressed, four persisted, two stenosed and five obstmcted. Thus, the prognosis of aneurysms less than 300% of normal in diameter is excellent, whereas that of aneurysms 400% of normal or more in diameter is serious.
Dopaminergic Instability in Children with Orthostatic Dysregulation
Authors: Sato Tamotu , Igarashi Noboru , Maruyama Hiroaki , Okabe Takashi
Published: October 2007

Effects of postural change on plasma catecholamine levels were examined in 53 children with orthostatic dysregulation (OD). Special interest was focused on plasma dopamine and dopamine‐β‐hydroxylase (DBH) activity, since one patient showed a prominent rise in plasma dopamine from 822 pmol/L to 126 nmol/L in postural change. She had a wide fluctuation of plasma dopamine from 209 pmol/L to 305 nmol/L during 12 hours of observation period, but plasma norepinephrine and epinephrine remained within the normal range as well as urinary excretion of catecholamines. Of 52 children with OD, 11 had a marked increase in plasma dopamine, whereas 11 showed a reduction of it by postural change. Plasma DBH activity was significantly decreased in the former (81±14 nmol/h/ml), while it was elevated in the latter (320±48 nmol/h/ml, P<0.001 vs the former), although DBH activity in individuals was not affected by postural change. These results indicate the involvement of dopaminergic instability as a cause of OD in childhood.
The Value of the Sinusoidal tcPO2 Pattern in the Evaluation and Management of Cyanotic Congenital Heart Disease with Ductus Dependent Lesions
Authors: Seki Syuichiro , Ikenoue Tyuyomu , Hokanishi Hisahiko , Hayakawa Kunio
Published: October 2007

A characteristic sinusoidal tcPO2 pattern was observed in 17(27%) of 62 patients with congenital heart disease manifested by cyanosis or heart failure during the neonatal period. All of these 17 patients were definitively diagnosed by cardiac catheterization, as having 8 PPA; 3 ToF; 2 TCA+VSD+PA; 2 asplenia with PA; 1 TGA+VSD+PS; and 1 TA (no TCA type). The transcutaneous oxygen pressure pattern of these patients showed a sinusoidal change with a cycle length of 6–20 min with the maximum pressure not exceeding 50 torr and the minimum pressure occasionally close to 0 torr. This sinusoidal tcPO2 pattern was associated with various types of ductus‐dependent congenital heart diseases and hence may be of diagnostic value. Transcutaneous oxygen pressure monitoring is also useful in evaluating the ductal response to PG. Other possible mechanisms underlying this phenomenon including biological oscillation should be considered.
Improved Bone Mineral Status in Very Low Birthweight Infants Fed Human Milk Mixed with Preterm Formula
Authors: Tsukahara Hirokazu , Mitsuyoshi Izuru , Sakaguchi Tadahiko , Fukuhara Hitoo , Hayashi Shuhei , Nakamura Katsuji
Published: October 2007

The bone mineral status of very low birthweight (VLBW) infants fed exclusively their own mother's milk (group I) was compared with that of VLBW infants fed mother's milk in the initial 4 weeks followed by a 1:1 mixture of mother's milk and preterm formula containing high phosphorus (P) and calcium (Ca) (group II). In both groups, most infants showed a biochemical picture characteristic of phosphorus deficiency syndrome by the fourth week. Thereafter, serum alkaline phosphatase activity (ALP) decreased and serum P increased in all group II infants. Conversely, serum ALP rose and hypophosphatemia persisted in most group I infants. Group II had a significantly higher serum P at weeks 8 and 12 and a significantly lower ALP at week 12 than group I. Furthermore, group II had a lower incidence of severe radiographic abnormalities than group I at week 12. We confumed previous observations that VLBW infants fed exclusively human milk require P and Ca supplementation to prevent metabolic bone disease of prematurity.
The Effects of Hepatitis B Vaccination on Infants Born to Carriers Without HBe Antigenemia
Authors: Shin Kenji , Nagai Yoji , Ono Ichiro , Sumasaki Ryo , Takita Hitoshi
Published: October 2007

A retrospective study on fifty‐three patients born to hepatitis B surface antigen (HBs antigen) positive mothers was carried out. Among 17 patients born to hepatitis B e antigen (HBe antigen) negative mothers, six patients received hepatitis B(HB) vaccination and eleven did not. Nont of the six patients who had received HB vaccination showed seroconversion, while six of the 11 patients (54.5%) without HB vaccination did show seroconversion. There was no definite correlation between the frequency of seroconversion and the administration of HB immune globulin (HBIG). HB vaccination alone may be suffxient to protect infants born to HBe antigen negative mothers from HB viral infection. (Acta Paediatr Jpn 1989; 31: 177–179)
Leukotriene C4 in Children with Atopic Asthma I. Plasma Levels in Acute Asthma
Authors: Iwasaki Eisaku
Published: October 2007

The leukotrienes C4, D4 and E4 are potent biologic mediators, and are thought to play an important role in obstructive airways disease such as asthma. In this study, plasma im‐munoreactive LTC4 (iLTC4) levels in asthmatic children were measured using radio‐immunoassay after Sep‐pak extraction in order to determine whether LTC4 is released in vivo during an asthmatic attack. In 10 non‐atopic children, the mean ± SEM of plasma iLTC4 level was 0.031 ± 0.013 pmol/ml. Significantly higher plasma iLTC4 levels were recognized at all stages in asthmatic children, both in remission (p<0.01) and during an attack (p<0.01). PaO2 levels during an attack were significantly lower in the high iLTC4 group. In 15 asthmatic children, the plasma iLTC4 levels during an attack (0.134 ± 0.017 pmol=ml) significantly decreased during recovery (0.078 ± 0.012 pmol/ml) (p<0.05). Plasma iLTC4 levels correlated closely with lung function (p<0.01). The high iLTC4 levels in plasma in asthmatic children suggest a role for LTC4 in the pathophysiology of asthma.
Treatment and Complications of Childhood Asthma in Japan
Authors: Nishirna Sankei , Takeishi Takashi
Published: October 2007

Recently the prevalence of asthma and emergency hospital admissions are increasing, but most childhood asthma can be controlled with appropriate therapy. Children can maintain their normal life cycle by outpatient treatment. There are two main complications of childhood asthma. One is the result of pathophysiological features and the other related to therapy. Status asthmaticus is the most serious complication and may be fatal.
Air Leak Syndrome (ALS) as Complication of Asthma
Authors: Takeishi Takashi , Nishima Sankei , Kano Sohei
Published: October 2007

Twenty‐eight patients suffered 32 episodes of ALS (air leak syndrome) between 1974 and 1985 at the Department of Pediatrics of the National Minami‐Fukuoka Chest Hospital. The highest incidence was observed between ten and twelve years of age and in the autumn. Their chief complaints were chest pain, sore throat and some pains in other parts. Pneumo‐mediastinum associated with subcutaneous emphysema was observed in 50%; this was the most common type of ALS. Chest X‐ray findings showed free air in the left mediastinurn in 20 of 22 patients with pneumomediastinum. Free air in the left mediastinum is considered to be a diagnostic finding for ALS.
Support and Variation, the Needs of the Breast‐Feeding Woman
Authors: Raphael Dana
Published: October 2007

A basic pattern for human beings is the support given to the mother during delivery and for a period postpartum. One function of this universally recognized need is to assure the success of breast‐feeding. The position of the supportive person—the doula— who works with the mother during this period is examined in this presentation. The quantitative difference in the amount of support needed by different women during this period is discussed. In the United States the role of the father is changing dramatically. Men are present at the birth of their children and are expressing more tenderness towards their wife and children. The possibility of such change of role for Japan is of concern. Studies of over 200 cultures have shown the enormous variety m patterns of care for the mother during and after delivery. The author suggests that each mother has a unique rhythm of her own and that sensitivity to each individual woman's needs might enrich her first experiences with her child.
Henoch‐Schonlein Purpura after Measles Immunization
Authors: Ozaki Takao , Miwata Hiroyuki , Kodama Hiroshi , Matsui Yoshiharu , Asano Yoshizo
Published: October 2007

A threeyear‐old boy developed Henoch‐Schonlein purpura after measles immunization. To our knowledge Henoch‐Schonlein purpura in this setting has not been previously reported. This case illustrates that measles vaccine can act as an inducing factor Henoch‐Schanlein purpura.
Supplementary Thyroxine Therapy in Patients with Hypothyroidism Induced by Long‐Term Anticonvulsant Therapy
Authors: Kodama Soichi , Tanaka Kazuhiro , Konishi Hideki , Momota Keiko , Nakasako Hirohide , Nakayama Shinichi , Yagi Jiro , Koderazawa Keiko
Published: October 2007

Of 287 patients under long‐term therapy with anticonvulsants, 24 with low serum thyroxine and free thyroxine concentrations were prescribed supplementary thyroxine in the present study. In addition, the basal metabolic rate (BMR) was measured in 13 out of 24 patients and in eight of them it was low (under ‐15%). Serum thyroid hormone concentrations improved after administration of thyroxine. However, improvement of the BMR was not obtained after one month of supplementary therapy. EEG after administration showed an increase in the power spectra of the occipital alpha 2 band (10.0–12.8 Hz) and beta 1 band (13.0–19.8 Hz), and a decrease in that of the theta band (4.0–7.8 Hz). The inter‐peak latency from wave I to wave V in the ABR was normalized after administration, These results suggest that supplementary therapy with thyroxine may be necessary in patients with hypothyroidism induced by anticonvulsants.
Five Cases of Autoimmune Neutropenia in Infancy or Early Childhood
Authors: Onisawa Shin , Ichimura Tohju , Saito Kenji , Sekine Isao
Published: October 2007

Five cases of chronic neutropenia which developed mainly in infancy were investigated and followed up for 5 to 34 months. Antineutrophil antibody levels were assayed by the direct and indirect immunofluorescence methods using flow cytometry. Selective neutropenia, myeloid hyperplasia and/or maturation arrest of myeloid series cells in the bone marrow, and the demonstration of antineutrophil antibody in the sera and on autologous neutrophils, indicated that the neutropenias involved were autoimmune in nature. Two cases exhibited spontaneous recovery 12 and 14 months respectively after the diagnosis, with concomitant marked decreases in antineutrophil antibody levels. Our study of these five cases supports the view that this disorder may be a clinical entity with a relatively consistent clinical presentation and course. Within this framework of the disorder, however, a clear heterogeneity in clinical presentation and course was also noted in our cases. Clinical severity in this disorder may be enhanced by arrested maturation of myeloid series cell in the bone marrow.
Ductal Left‐to‐Right Flow and Pulmonary Circulation in Normal Neonates: Pulsed Doppler Echocardiographic Evaluation
Authors: Shiraishi Hirohiko , Yanagisawa Masayoshi
Published: October 2007

To determine whether ductal left‐to‐right flow affects pulmonary circulation in the neonatal period, ductal patency and flow patterns of the right ventricular outflow tract were studied using Doppler echocardiography in 40 normal neonates. The ductus arteriosus was defined as being patent when diastolic or continuous flow could be detected at the pulmonary end of the ductus. Evaluation of pulmonary circulation was carried out by examining the flow velocity pattern of the right ventricular outflow tract; pulmonary hypertension was considered to be present when the Doppler echocardiogram pattern was triangular with peak velocity in early systole. Neonates were initially examined within nine hours (mean 5.3 hrs) after birth, and two to three times daily thereafter, until no ductal flow could be detected. In all the neonates, the ductus arteriosus was patent at the initial examination. The predicted time of closure of the ductus arteriosus was within seven hours after birth in 5% of the neonates, within 21 hours in 50%, and within 65 hours in 95%. The flow velocity pattern of the right ventricular outflow tract changed from a triangular shape with peak velocity in early systole soon after birth to a domelike contour with peak velocity in mid‐systole: thus the mean ratio of acceleration time to right ventricular ejection time increased with age. In neonates with patent ductus arteriosus at age 13.6–20.7 hours, the mean ratio of acceleration time to right ventricular ejection time was less than in age‐matched neonates with closed ductus arteriosus. These results indicate that the ductal left‐to‐right flow affects the pulmonary circulation.
Precocious Puberty in A Seven‐Year‐Old Boy due to Human Chorionic Gonadotropin Producing Pineal Tumor Detected by Nuclear Magnetic Resonance Computed Tomographic Scanning
Authors: Takahashi Hiroaki , Tokuda Narumi , Kariya Hirohiko
Published: October 2007

We report a 7‐year‐old boy who developed incomplete sexual precocity due to a human chorionic gonadotropin (HCG)‐producing tumor in the pineal region. The patient presented enlarged testes (3times2x2 cm) bilaterally, enlarged penis, pubic hair development of Tanner Stage 111, advanced bone age and growth spurt. Initial hormonal studies showed an adult male level of testosterone (13 ng/ml) and a high level of HCG as well as HCG‐β subunit. A high basal level of LH, probably due to immunocross‐reactivity with HCG, and low basal level of FSH, probably suppressed by testosterone, did not respond to LH‐RH infusion. Search for the site of HCG production failed at the initial workup, but calcification without definite signs of tumor in the pineal region was found by conventional brain CT scan. Because of subsequent progression of clinical and laboratory findings of sexual precocity, nuclear magnetic resonance computed tomographic (NMR‐CT) scan was performed, which confirmed the presence of a pineal tumor three months later. The patient was treated with 4,500 rad. of radiation therapy, and responded dramatically to this regimen. He has been followed for more than two years without any signs of recurrence. We have reported here a very rare case of incomplete sexual precocity due to an HCG‐producing intracranial tumor in the pineal region. An NMR‐CT scan is a very useful tool for the diagnosis of some types of pineal tumor, such as germinoma, which are highly radiosensitive.
Physical Conditioning Programme for Children with Bronchial Asthma
Authors: Olivia Chow Kit Wun
Published: October 2007

Exercise induced bronchospasm limits physical activity in most asthmatic children. Twelve children with stable chronic asthma were enrolled in a physical conditioning program to improve ventilation mechanics and to promote physical activity. The program consisted of eight weekly sessions of one‐and‐a‐half hours each, followed by three months of weekly swimming lessons. The activities were preceded by a warm‐up period and interspersed with rest. Premedication with an aerosol bronchodilator or sodium cromoglycate was allowed before training. Cardiorespiratory status was studied before and after the program. The program was well received by the children with no bronchospasm. Five showed improvement in lung volumes and/or flow rates. Bronchial lability remained the same. The basal heart rate slowed in four children. No significant arrhythmia was detected. With continued practice, cardiorespiratory function might improve further. The program should be incorporated as part of the overall management of bronchial asthma.
Treatment of Status Asthmaticus—Hormone Changes before and after Aminophylline i.v. Drip Therapy
Authors: Likura Yoji , Kishida Masaru , Akasawa Akira , Nagakura Toshikazu , Akimoto Ken‐ichi , Saito Hirohisa , Koya Niroku , Sasamoto Akiyoshi
Published: October 2007

The treatment of status asthmaticus is one of the most important factors in controlling the patient with asthma attacks. We have studied hormone changes in status asthmaticus and considered what is the best treatment in the asthma attack condition in children. Antidiuretic hormone (ADH), renin activity, and aldosterone activity are elevated in severe asthma attack conditions, and these high levels are correlated with high levels in Wood's clinical score. It is theoretical that patients with dehydration and respiratory failure show such elevation in hormones, and it is well known that under such conditions β2‐stimulant enhances renin production. From our study, it is concluded that β22 ‐stimulant subcutaneous injection must be considered in status asthmaticus. In children, amminophylline i.v. drip therapy may be one of the best treatments in status asthmaticus.
Antioxidant Enzymes and Lipoperoxide in Blood in Patients with Kawasaki Disease Comparison with the Changes in Acute Infections
Authors: Uchida Norihiko , Asayama Kohtaro , Dobashi Kazushige , Hayashibe Hidemasa , Kato Kiyohiko
Published: October 2007

Increased production of active oxygen species from activated neutrophils is postulated to contribute to endothelial damage in Kawasaki disease, leading to the formation of coronary aneurysms. To determine wheather an altered oxidant‐antioxidant balance exists in acute phase of Kawasaki disease, antioxidant enzymes in peripheral blood cells and Plasma lipid peroxide were measured in patients. The two isoenzymes of intracellular superoxide dismutase were assayed by specific radioimmunoassays. Lipid peroxide in plasma and manganese superoxide dismutase in both polymorphs and lymphocytes were increased in the acute phase of Kawasaki disease. The erythrocyte alutathione peroxidase and catalase were also increased. On the other hand, copper zinc superoxide dismutase in polymorphs, lymphocytes and erythrocytes was unaltered. Acute infections did not appear to modify the levels of either antioxidant enzymes or lipid peroxide in blood. These results suggest that increased oxidative stress in Kawasaki disease evokes a reactive increase in antioxidant enzymes, and that this response in the defense system is related ot the reversible nature of the tissue damage in most patients with Kawasaki disease.
Upshaw‐Schulman Syndrome in Two Siblings
Authors: Saitoh Hiroharu , Murakami Hideko , Mori Chuzo
Published: October 2007

Upshaw‐Schulman syndrome is a rare disease, and familial occurrence has not been reported. In this paper, two Japanese brothers, aged 2 and 6 years, with microangiopathic hemolytic anemia, thrombocytopenia and fragmented red cells from the newborn period are reported. Unusually large von Willebrand factor (vWF) multimers were found in the plasma samples from both cases during remission, while the quantities of the unusually large vWF multimers decreased greatly at a low platelet count. They were temporarily relieved from hemolytic anemia and thrombocytopenia by infusions of plasma from normal donors. It appears that our patients lack an unidentified plasma factor, which is genetically determined.
The Teratogenic Effects of E‐64 on Rat Embryogenesis
Authors: Tachikura Toshio
Published: October 2007

To examine the effects of E‐64 (a thiol protease inhibitor) on the development of rat embryos, pregnant Wistar rats were injected with 10–30 mgkg of E‐64 intra‐peritoneally on days 9 and 10 of gestation. On day 21, the rats were killed and the fetuses were examined for malformations. When 30 mgkg of E‐64 was administered, the incidence of resorptions and malformations was 25.7% and 60.4%, respectively, The most common malformations were hydrocephaly, exencephaly, anophthalmia, microphthalmia, hydronephrosis and renal hypoplasia. “hew results suggest that thiol protease inhibitor is teratogenic during early rat embryogenesis.