bluuebunny/crossref_metadata_2025_split huggingface dataset:
Recent Advances in Infantile Spasms Research in Finland
Authors: Riikonen Raili 
Published: October 2007

AbstractData on incidence, etiology, therapy and prognosis of infantile spasms are reviewed. Infants with infantile spasms have a lower CSF GABA concentration than other epileptics. In a large proportion of cases the etiological factors of infantile spasms can now be elucidated with some certainty. Especially, the new neuroradiological and virological methods have enabled a more accurate diagnosis. There have been changes in the etiological pattern over the years. The optimal dosage and duration of ACTH or glucocorticoid therapy have not been established. In the Finnish study the large ACTH doses (120–160 IE) generally used in many Nordic European countries did not carry a better prognosis than the smaller doses (20–40 IE). Therapeutic response showed no correlation with the Cortisol secretion rate but the infants who responded well had a high dehydroepiandrosterone‐androstenedione concentration ratio. Side‐effects of ACTH therapy are relatively common and more common with large doses than with smaller ones. The most common side‐effects are infections and arterial hypertension. There were disturbances of calcium and phosphate metabolism during ACTH therapy. Severe nephrocalcinosis was seen in some infants at autopsy. Fluid disturbances were common at termination of ACTH therapy. ACTH therapy causes adrenal hyporesponsiveness. The therapy should be modified to improve the prognosis.
Comparison of Q(R) to T Peak Time in Right and Left Precordial Leads in Children
Authors: Awa Shoichi , Yanagawa Yukishige , Hishi Toshio , Akagi Michio , Dodo Hidemi 
Published: October 2007

AbstractAn electrocardiographic measurement useful in the diagnosis of right ventricular overload, involving precordial T waves, is presented. The longest Q(R) to T peak interval from V3R to V4(V5) was longer than or identical with the same interval in both V5 and V6(V7) when hemodynamic right ventricular overload was present. Sensitivity was greater when volume overload was present than with pure pressure overload. In contrast to the non‐specificity of the vectorcardiographic horizontal T loop, inscription and contour, the newly presented corresponding electrocardiographic parameter proved to be a simple, sensitive and specific discriminator of right ventricular overload. Discrepancy in diagnostic usefulness between the two systems (EKG versus VKG) is discussed in terms of proximity effects in precordial leads.
Classification of Diabetes Mellitus by Studies of C‐Peptide Secretory Capacity
Authors: Teshirogi Tadashi , Hosoi Hiroeki , Ohki Yukashi , Kagoshima Toshio 
Published: October 2007

SummaryResidual pancreatic B‐cell function was investigated in children with diabetes mellitus in whom classification of the type of disease was difficult at the first visit.Intravenous glucagon tests were performed at the first visit and subsequently, the C‐peptide responses compared.Based on our data on a limited number of patients, we propose C‐peptide concentrations of 3.0 to 3.5 ng/ml at the peak or at 6 min after injection of glucagon, as the critical level which distinguishes non‐insulin dependent from insulin‐dependent diabetes mellitus.However, the degree of obesity, clinical stage and other factors also need to be considered in the classification of diabetes mellitus.
High‐Dose Gammaglobulin Therapy in Six Children with Newly Diagnosed Insulin‐Dependent Diabetes Mel I it us
Authors: Urakami Tatsuhiko , Hanaoka Yoko , Fujita Hidehiro , Kitagawa Teruo 
Published: October 2007

AbstractWe treated six newly diagnosed children with insulin‐dependent diabetes mellitus (IDDM) with high‐dose gammaglobulin therapy in a clinical trial of immunomodulation. Approximately 400 mg/kg/day of polyethylene glycol‐treated gammaglobulin was given by intravenous injection for four to five days.After the initial therapy, four out of six patients showed an elevation of serum CPR and a decline of their insulin requirements; this indicates an improvement of insulin secretory function of pancreatic beta cells.A second course of gammaglobulin was given to four patients one year after the initial therapy, and the same results were observed in two out of four patients.These results suggest that high‐dose gammaglobulin might provide some protection against the deterioration of endogenous insulin secretion in children with IDDM.
Improvement of Initial Remission Rate by Introduction of CSII
Authors: Tanae Ayako , Kagawa Jiro , Nozaki Kae , Hashimoto Nobuko , Hibi Itsuro 
Published: October 2007

AbstractWe studied 65 insulin‐dependent diabetic children and adolescents diagonosed during the period 1976–86 to find out the occurrence and duration of postini‐tial remission, the relationship between the remission behaviour and the initial treatment introduced by continuous subcutaneous insulin infusion (CSII), the favoring factors and the prognostic value of remission. A postinitial remission occurred in 36 patients (55.4%) being complete in only two boys (3.1%). The mean duration was 10.8 months and median was 7.8 months: the shortest period one month and the longest 36 months. On the other hand, remission occurred in 16 out of 19 patients (84.2%) with CSII and 15 out of 37 patients (40.5%) with conventional treatment (CT) as initial treatment. The mean duration of remission was 14.6 ± 10.6 months in the CSII group and 6.8 ± 4.7 months in the CT group. Further, our study proved a significant increase of urinary C‐peptide excretion in the patients with CSII during their remission. Through our study in comparison with CT, we were able to see an improvement in the remission rate by introducing the CSII for a short term in the initial treatment and it led us to realize that what caused it was the recovery of endogeneous insulin secretion capacity.
Residual Pancreatic Beta‐Cell Function in Insulin‐Dependent Diabetes Mellitus
Authors: Amemiya Shin , Higashida Kohsuke , Fujimoto Masatoshi , Asayama Kohtaro , Ichimura Kazuyoshi , Kusano Shoichi , Ohyama Kenji , Kato Kiyohiko 
Published: October 2007

AbstractIt is recognized that residual pancreatic /3‐cell function may play a role in the control of blood glucose in patients with insulin‐dependent diabetes mellitus (IDDM). Since modern therapy is concerned with the possibility of recovery of pancreatic β‐cell function, we need to identify the secretagogue of choice in the various phases of IDDM.Eleven patients with IDDM underwent four different stimulation tests of (3‐cell function over two weeks. The tests were i.v. arginine (ATT), oral glucose (o‐GTT), i.v. glucagon (GCT) and i.v. tolubutamide (TTT). One patient had the tests repeated four times over two years after the onset.Among the tests, ATT and o‐GTT produced the largest response ofC‐peptide. The response to GCT was small in comparison. TTT had no significant effect. The present study suggests that ATT or o‐GTT or both are useful in the evaluation of endogenous insulin secretion during follow‐up of IDDM. On the other hand, GCT can only be used as a simple screening method for residual ß‐cell function and is not sufficient for quantitative measurement in IDDM.
A Case of Congenital Myopathy without Specific Features
Authors: Tachi Nobutada , Hosoya Shizue , Watanabe Mutsuko , Wakai Shuji , Minami Ryoji 
Published: October 2007

AbstractWe report a 4‐year‐6‐month‐old boy with congenital myopathy without specific features. He showed delayed motor milestones, gaining head control at four months, and walking without support at one year six months. He was not a floppy infant after birth. Physical examination disclosed a myopathic face and muscle atrophy, predominantly in the proximal muscles, particularly in the scapular and pelvic girdles. A proximal muscle biopsy showed a mild variation in fiber size without any specific structural abnormality. Fiber type analysis showed type 2B fiber smallness. We compare our case with previously reported cases of minimal change myopathy (congenital myopathy without specific features).
Treatment of Acute Asthma in Children
Authors: Bierman C. Warren , Pearlman David S. , Pierson William E. , Shapiro Gail G. , Furukawa Clifton T. 
Published: October 2007

SummaryAcute severe asthma in children should be managed by immediate attention to the patient's clinical condition. Careful observation, the use of sequential pulmonary function tests and appropriate blood gas studies will facilitate evaluation of severity. Therapy should be directed at the reversal and7sol;or correction of these abnormalities as well as anticipation of early treatment of potential complications. By the aggressive management of this condition, the incidence of complications can be reduced, the length of hospital stay shortened and substantial morbidity and possible mortality minimized.
Recovery Period of Hypersecretion of Thyroid‐Stimulating Hormone in Patients with Congenital Hypothyroidism Treated with Thyroid Hormone
Authors: Sato Hirokazu , Inomata Hiroaki , Sasaki Nozomu , Niimi Hiroo , Nakajima Hironori 
Published: October 2007

AbstractThe recovery periods of serum thyroid stimulating hormone (TSH) were studied in patients with congenital hypothyroidism found on neonatal screening. L‐T4 was administered at 37.8 ± 15.1 days of age (M ± SD), and serum T4, T3, and TSH concentrations were measured. After L‐T4 therapy with 10 µg/kg, the serum TSH levels decreased to below 10 µU/ml in five patients within a week, and in another two patients, within two weeks, and in the total number of 12 patients out of 15 (80%), by one month.These results suggest that the recovery of serum TSH occurs rapidly in hypothyroid infants. Thus, the serum TSH concentration may be a good measurement for assessing the adequacy of thyroid hormone therapy from early infancy in patients with congenital hypothyroidism.
A Simplified Master's Two‐Step Test for Preschool Children: A Preliminary Report
Authors: Yoshinaga Masao , Oku Shozo , Chin Shoho , Mizumoto Yuji , Iwayama Kiyoko , Yamamoto Eiji , Inoue Hiroyuki , Suenaga Shinji , Nomura Yuichi , Aihoshi Sogo , Ohkawa Shunya 
Published: October 2007

AbstractTo investigate a simplified Master's two‐step test (MTT) for preschool children aged 4 to 6 years exercise tests with new stairs, with risers 18 cm and 20 cm high, were employed. The subjects were 162 preschool children who were divided into four groups according to the height of the riser and the number of steps taken (60 and 72 steps per 3 minutes).In these tests, the 4‐year‐old children obtained similar mean heart rates immediately after exercise (156.9 to 161.6 BPM) in spite of different exercise tests, and the 5‐ and 6‐year‐old children obtained significantly higher mean heart rates (166.0 and 162.1 BPM, respectively) in the test with a 20 cm riser taking 72 steps. If a single protocol is to be used for children aged 4 to 6, the test with a 20 cm riser and taking 72 steps seems to be the best with reference to the achieved heart rates and performance ratios.
Morphologic Studies of the Liver and Bile Ducts in Biliary Atresia
Authors: Ohi Ryoji , Chiba Tsuneo , Endo Naobumi 
Published: October 2007

AbstractWe studied 233 patients with biliary atresia seen between 1953 and 1985. Specimens of the extrahepatic bile ducts and liver which were taken at the time of corrective operation were examined histologically. We noted the presence of inflammation in the extrahepatic bile ducts, which confirmed in retrospect the necessity for early operation. The results of these studies are discussed in connection with the pathogenesis, pathophysiology and clinical features of this disease.
Electrocardiographic Standard for the Diagnosis of Right Ventricular Hypertrophy in Complete Right Bundle Branch Block
Authors: Tsuji Atsutoshi , Osano Mitsuru , Morikawa Yoshiyuki , Sato Masaaki , Asaishi Takasumi 
Published: October 2007

AbstractIt is generally considered that in complete right bundle branch block, the electrocardiographic standard for the diagnosis of right ventricular hypertrophy is not applicable.According to Cohen's criteria, right ventricular overload exists if thallium‐201 myocardial perfusion demonstrates the right ventricular wall, and in this light we attempted to reassess the validity of the electrocardiographic standard for right ventricular hypertrophy.Twenty‐three children with complete right bundle branch block (abbreviated as CRBBB) were evaluated. The agreement and disagreement ratios of the electrocardiographic standard with Cohen's criteria was 20/23 and 3/23 respectively. Even in patients without intracardiac anomalies, right ventricular overload was suspected by Cohen's criteria.The electrocardiographic standard was in agreement with Cohen's criteria in most of these patients.
Transesophageal Atrial Pacing in Paroxysmal Supraventricular Tachycardia in Infants and Children
Authors: Fujiseki Yoshiki , Okuno Masahiko , Fujino Hidetoshi , Hattori Masanori , Nonomura Kazuo , Shimada Morimi 
Published: October 2007

AbstractFourteen patients with paroxysmal supraventricular tachycardia (PSVT) underwent serial electrophysiologic studies using transesophageal atrial pacing (EPS‐EP), to determine the predictive value of this method in the selection of antiarrhythmic therapy and to study the mechanism of the tachycardia.In each patient, PSVT could be reproducibly initiated by burst and/or programmed stimuli. After control EPS‐EP, the effects of several drugs (diltiazem, disopyramide, propranolol, digoxin, procainamide and verapamil) on the ability to initiate PSVT or to bring about “tachycardia zone” were examined. The mechanisms of PSVT were also investigated using an esophageal electrode, EPS‐EP, and Ca++‐antagonists.Drug responses were variable but in most cases tachycardia could not be initiated after drug administration. Four types of supraventricular tachycardia are described. In eight out of nine patients, good therapeutic control was obtained. This study shows that serial EPS‐EP can provide rapid identification of appropriate antiarrhythmic therapy, and is useful in studying the mechanism of PSVT.
Congenital Subclavian Arterial Stenosis
Authors: Nogami Tetsuo , Sato Hideo , Hirano Takeki , Horigome Hitoshi , Ijima Hiroshi , Takita Hitoshi 
Published: October 2007

AbstractWe describe a 2‐year‐old boy with a non‐palpable right radial pulse associated with underdevelopment of the arm. An arteriogram showed a narrowing of the distal part of the right subclavian, axillary and brachial arteries, and poor collateral circulation. This patient illustrates a rare cause of an absent radial pulse, other than Takayasu's disease.
Turner's Syndrome with Agenesis of the Corpus Callosum, Hashimoto's Thyroiditis and Horseshoe Kidney
Authors: Araki Kumiko , Matsumoto Kenji , Shiraishi Taisuke , Ogura Hideo , Kurashige Takanobu , Kitamura Isamu 
Published: October 2007

AbstractWe present a 14‐year‐old girl with Turner's syndrome, agenesis of the corpus callosum, Hashimoto's thyroiditis and horseshoe kidney. She was admitted to our hospital because of short stature. She had some of the somatic stigmata of Turner's syndrome, was slightly mentally retarded, but had no neurological abnormalities. Chromosome study showed a 45, x karyotype. Her thyroid function was normal, but both the antithyroglobulin and antimicrosomal antibody titers were elevated, suggesting lymphocytic chronic thyroiditis. An intravenous pyelogram and an abdominal CT scan showed a horseshoe kidney. CT scan of the brain showed agenesis of the corpus callosum.As far as we are aware, this is the first report of Turner's syndrome with agenesis of the corpus callosum, Hashimoto's thyroiditis and horseshoe kidney.
Effect of Cornstarch Formula in an Infant with Type I Glycogen Storage Disease
Authors: Ogata Tsutomu , Matsuo Nobutake , Ishikawa Kazuo , Araki Kiyoshi 
Published: October 2007

AbstractIn order to cope with profound hypoglycemia in an infant with type I glycogen storage disease receiving nocturnal intragastric infusion, we administered cornstarch formula every four hours at 14 months of age. Following the cornstarch therapy, fasting blood glucose in creased from 39.3±4.0 to 83.5±4.0 mg/dl (p<0.01), σ insulin during oral glucose tolerance test (2.5 g/kg) increased from 76 to 191 µU/ml, 24‐hour urinary C‐peptide excretion increas ed from 31.0±4.7 to 47.4±55.2 µg/g/‐creatinine(p<0.05), plasma somatomedin‐C increased from 0.13 to 0.43 U/ml, and length increased from the 3rd percentile to the range between the 10th and the 25th percentile.The results indicate that cornstarch formula may prevent the precipitous hypoglycemia that occurs during the day in an infant with type I glycogen storage disease on nocturnal in tragastric infusion. Increased insulin and/or somatomedin‐C secretion appears to be responsible for the improvement in linear growth of the infant.
Continuous Nasogastric Infusion of Prostaglandin E2 in Ductus‐Dependent Congenital Heart Disease
Authors: Komiyama Osamu , Osano Mitsuru. , Oikawa Tadao , Morikawa Yoshiyuki , Kojima Yoshifumi , Murai Takayasu , Ishihara Jun , Tokumura Mitsuaki , Taguchi Nobuhiko 
Published: October 2007

AbstractProstaglandin E2 (PGE2) was infused continuously through a nasogastric gavage tube in four infants with pulmonary atresia. The drug was given at a rate of 12.5–160 µg/kg/h. The duration of therapy was 7–123 days. The effects and side effects seen by this method were similar to those seen in the conventional multiple dose regimen. This method was an effective and simple way of maintaining the ductus arteriosus open, especially for a long period of time.
Automated BET, Blood Pressure, and IVH
Authors: Funato Masahisa , Shimada Seiichi , Tamai Hiroshi , Taki Hideo , Yoshioka Yasushi 
Published: October 2007

AbstractAn automated apparatus for blood exchange transfusion (BET) has been devised with a two‐site technique for peripheral artery and vein, utilizing an Atom infusion pump. With this apparatus, we have successfully performed exchange transfusions 189 times in 110 infants with birth weights ranging from 530 g to 4,000 g within the past four years.Evaluating the influence of this method on the circulatory system, the variation in blood pressure (BP) during exchange transfusions was consecutively measured 29 times in 16 infants (14 cases by indirect method and two cases by direct method). The BP was very stable, without any significant change in the mean and the coefficient variation (CV) before, during and after the transfusion (Systolic BP: 59.3±11.9, 60.1±11.9, and 60.3±12.1 mmHg; p: n. s., CV: 7.8±4.1, 8.1±3.8, and 7.7±4.1%; p: n. s., respectively).Further, in evaluating the influence of the method on the occurrence of intraventricular hemorrhage (IVH), brain ultrasonography was performed in 20 extremely premature infants of less than 1,500 g in birth weight prior to and following the exchange transfusion. There was no significant occurrence or exacerbation of IVH. by the method itself (IVH Grade 0: 15 cases, Grade 1: 4 cases, Grade 2: 1 case).We believe that this automated method may be more stable as it relates to variation of systemic BP and that the occurrence of IVH especially in extremely premature infants may be less likely with the automated method than with the use of Diamond's method, since it is isovolemic in procedure.
Nosocomial Infection by Rotavirus and Adenovirus Within One Month
Authors: Ushijima Hiroshi , Shinozaki Tatsuhiko , Araki Kazuko , Kondo Yasuo , Kobayashi Masaaki , Abe Toshiaki 
Published: October 2007

AbstractWe experienced a small outbreak of acute gastroenteritis in December 1985 in hospitalized children. Two different serotypes of rotavirus and two serotypes of adenovirus were found by molecular epidemiological and serological studies. Four patients were considered to have two serotypes of rotavirus. The results showed that a single strain of a virus was not always the cause of nosocomial infection.
Respiratory Syncytial Virus Infection in Infants with Congenital Heart Disease and Treatment with Human Leukocyte lnterferone
Authors: Chiba Yasuo , Mito Kazuaki , Suga Kazuhiro , Honjo Takashi , Sawada Yohko , Tsuda Tetsuya , Ikeda Kazuo , Minagawa Tomonori 
Published: October 2007

AbstractBetween 1980 and 1986, we observed lower respiratory‐tract infection caused by respiratory syncytial virus (RSV) in 19 infants and young children with various forms of congenital heart disease. Of these, seven patients who presented with a severe illness were treated with intramuscular injections of 0.5 to 1 times 106 IU per day of human leukocyte interferon (IFN) for three to five days. Intrinsic IFN activity developed before treatment in three subjects. Viral isolation studies showed active RSV infection prior to treatment, and no infectious RSV was recovered one to four days after treatment. Although there were no obvious changes in the clinical course after IFN therapy, no deaths related to RSV infection occurred. Analysis of the different types of congenital heart disease showed that ventricular septal defect and/or patent ductus arter‐iosus with pulmonary hypertension were associated with the most severe RSV illness. The therapeutic value of IFN may improve in this group of subjects if it is given without delay.
Transmission Electron Microscopic Studies on StreptococcaI Aerosol Infection
Authors: Haraguchi Tsutomu , Yoshinaga Masao , Inoue Hiroyuki , Kawakami Kiyoshi , Hokonohara Masashi , Miyata Koichiro 
Published: October 2007

AbstractTo investigate the initial focus of streptococcal invasion, aerosol‐induced infection in rabbits that resembled natural infection was examined by transmission electron microscopy (TEM). Group A type 12 streptococci were adjusted to a concentration of 108‐109 colony forming units (CFU)/ml in saline. The rabbits were exposed to the aerosolized suspension for about two hours and sacrificed at two hours to 14 days after the exposure. Pharyngeal mucous membranes, palatine tonsils and tracheas were observed by TEM.Bacterial attachment was recognized on the tonsillar surface epithelium and abnormalities such as vacuolization of cytoplasm and an increased number of plasma cells were observed in the tonsillar medulla. In addition, specialized epithelial cells included micropinocytotic vesicles with spherical substances similar to those of the bacteria.The micropores of tonsillar crypts are considered to be the pathway of infiltrating cells and initial focus of foreign antigens at present. These findings suggested that tonsils have an important function as the initial focus of invasion of streptococci.
Proximal 3p Deletion: Case Report and Review of the Literature
Authors: Naritomi Kenji , Hirayama Kiyotake , Sameshima Koji , Ohdo Shozo 
Published: October 2007

AbstractA boy with severe growth and psychomotor retardation, facial dysmorphism, a large atrial septal defect, limitation of joint movement and hearing impairment is described. High‐resolution banding chromosome analysis showed interstitial deletion of the proximal segment of chromosome 3: 46, XY, del(3)(p12p14.2) de novo. The deleted chromosome 3 was of paternal origin as judged from Q‐banding polymorphisms. Delineation of proximal 3p deletion syndrome is proposed by summarizing the clinical and cytogenetical findings of the present and previously reported five patients.
Treatment Outcome of Maternal Phenylketonuria
Authors: Koch Richard , Wenz Elizabeth , Bauman Cindy , Friedman Eva G. , Azen Colleen , Fishler Karol , Heiter William 
Published: October 2007

AbstractOur experience to date with 40 pregnancies in 13 women with hyperphenylalaninemia indicates that to prevent mental retardation in their children, maternal phenylalanine levels of 120–600 umol/L are needed, in addition to a newborn screening program. While treatment results to date are preliminary, it appears that the phenylalanine‐restricted diet is of value and should be instituted as soon as pregnancy is planned by phenylketonuric women. There is much to be learned by study of these women and referral to a collaborating center in the National Maternal PKU Collaborative Study is recommended.