bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Pattern of Blood Lipids, Glycerol and Ketone Bodies during the Neonatal Period, Infancy and Childhood
Authors: PERSSON BENGT , GENTZ JOHAN 
Published: January 2008

SummaryStudies of individual blood lipid fractions, glycerol, ketones and blood sugar were carried out in a large group of healthy children ranging in age from full term birth to 8 years, with the object of ascertaining the alterations in these parameters at birth and through the period of postnatal adaptation.FFA, glycerol and ketone bodies are low at birth then rise rapidly reaching the highest levels within the first 48 hours of life, remaining high during the neonatal period and decrease to the lowest values at 1–6 months. When peak values for FFA and glycerol are reached, blood sugar determinations exhibit the lowest values.During the first day after delivery, before the first feeding, glycerides, phospholipids and cholesterol rise. They reach their peak values at 2–3 days and at 4–5 days for the two latter fractions respectively.There is a highly significant correlation between FFA and glycerol, not however, between FFA and glucose during the neonatal period.The results indicate an intense lipolysis during the neonatal period. The mechanism initiating this lipid mobilization (and redistribution of endogenous stored lipids) is not known. The importance of neurogenic, hormonal and nutritional factors in the regulation of FFA metabolism is discussed. When the values for glucose tolerance (obtained from a previous study) are compared to the fasting FFA levels of corresponding age groups, there is a relationship: with decreasing FFA values there is an improvement of the carbohydrate tolerance.
DEVELOPMENT OF UREA‐SYNTHESIZING ENZYMES IN HUMAN LIVER
Authors: Räihä Niels C. R. , Suihkonen Jukka 
Published: January 2008

SummaryLiver slices from human foetuses of a gestational age between 16 and 20 weeks produce urea when incubated with optimal amounts of substrate.The activities of the enzymes which synthesize urea (carbamyl phosphate synthetase, ornithine transcarbamylase, arginine synthetase system, argininosuccinase and arginase) have been measured in human liver during development. No rapid postparturition increase in activity was discernible in any of the enzymes studied, although in general the adult levels were somewhat higher than those found at birth. Arginine synthetase has the lowest relative activity of all the five enzymes involved in urea synthesis. The apparent Km for citrulline is similar in magnitude for both foetal and adult liver arginine synthetase.
THE HEMOSTATIC DEFECT IN GLYCOGEN STORAGE DISEASE, TYPE I
Authors: Gilchrist Gerald S. , Fine Richard N. , Donnell George N. 
Published: January 2008

SummaryBecause of the ill‐defined bleeding tendency which has been reported in patients with Glycogen Storage Disease, Type 1, various aspects of hemostasis and coagulation were studied in 8 patients. Definite abnormalities in the bleeding time and platelet factor 3 availability were found in the older symptomatic individuals but all the patients, irrespective of age, exhibited significant reduction in platelet adhesiveness in spite of normal platelet aggregation after addition of ADP. Although an intracellular defect may account for the abnormal platelet function, environmental plasma factors appear to play a role in the development of the platelet lesion.
ACTINOMYCIN D AND TRANSVERSE LINES OF GROWING BONES
Authors: Aarskog Dagfinn , Hexeberg Arve 
Published: January 2008

SummaryActinomycin D in a dose of 15 µg per kilogram body weight per day for 5 days was given at intervals of two months for two years in the treatment of Wilms' tumor. It was observed that each course of actinomycin D therapy resulted in the occurrence of transverse lines of increased density at the terminal segments of growing bones. Linear growth proceeded at the expected rate in all children studied. Evidently, arrest of longitudinal growth is no prerequisite for the development of transverse lines, and even the formation of multiple lines within a relatively short period does not necessarily imply any retardation of linear growth. The formation of transverse lines in response to actinomycin D administration may indicate that interference with growth hormone secretion and/or peripheral action may be involved in the development of these lines.
FIBRINOLYTIC TISSUE ACTIVITY IN THE UROGENITAL TRACT AND THE ADRENAL GLAND OF HUMAN EMBRYOS AND NEW‐BORN
Authors: Ladehoff Aa. 
Published: January 2008

SummaryThe concentration of plasminogen activator in the various organs of the human genito‐urinary tract and in the adrenals was investigated in 15 fetuses (aged 15 to 24 weeks of pregnancy) and in 7 premature or still‐born infants (aged 26 to 38 weeks).
AN INHERITED CHROMOSOME ABERRATION IN A GIRL WITH SIGNS OF DE LANGE SYNDROME
Authors: Broholm Karl‐Axel , Eeg‐Olofsson Orvar , Hall Bertil 
Published: January 2008

SummaryA mentally retarded girl with some signs of de Lange syndrome is described. The proposita has a chromosomal aberration, inherited from her unaffected mother. One chromosome of group B and one of group D are engaged in the translocation, and the possibility of a third chromosome being involved in this rearrangement cannot be excluded. The proposita has a partial D trisomy. The variability of de Lange syndrome is discussed.
METACHROMATIC LEUCODYSTROPHY IN EARLY CHILDHOOD Treatment with a diet deficient in vitamin A
Authors: Melchior Johannes C. , Clausen Jørgen 
Published: January 2008

SummaryThe clinical, biochemical and pathological findings in a family with four cases of metachromatic leucodystrophy are presented. The biochemical studies of one of the cases revealed the absence of urinary sulphatase A and the preponderance of two glycolipid fractions in urinary sediment.The patient in question was fed a diet low in vitamin A for four months. This diet reduced the relative amount of glycolipids five times in the urinary sediment (relative to the total content of sialic acid free polar lipids). The diet did not change the sulphatase activity. However, no significant clinical improvement of the patient was noticed. The biochemical changes induced by the deficiency in vitamin A are discussed on the basis of biochemical studies showing vitamin‐A to be necessary for the synthesis of active sulphate (PAPS). PAPS is necessary for sulphate incorporation in galactoceramide during biosynthesis of sulphatide. It is suggested that a diet deficient in vitamin A may be used in treatment of metachromatic leucodystrophy.
VENOUS OCCLUSION PLETHYSMOGRAPHY IN CHILDREN I. Method
Authors: Preece A. W. , Voyce M. A. 
Published: January 2008

SummaryThis paper describes a simple, acceptable plethysmograph for use with children, which is operated automatically and is not effected adversely by subject non‐cooperation. The operating mechanism, thermostatic control and recording apparatus are remote from the plethysmograph chambers, two of which are used for bilateral flow measurements.
EFFECT OF PHYSICAL TRAINING ON ADOLESCENTS WITH SEVERE MOTOR HANDICAPS
Authors: Ekblom Björn , Lundberg Åke 
Published: January 2008

SummaryThe case material consisted of 17 pupils at the Norrbacka Institute Schools. Cerebral palsy (CP) was present in 7 cases (mediam age 19 years), and paraplegia of other origin‐chiefly myelomeningocele‐in 10 (median age 17 years). All had severe motor but not mental handicaps, and had to use a wheel‐chair for their daily activities.Physical training was carried out for 30 minutes twice a week for 6 weeks, concurrently with the ordinary gymnastics. It consisted of fast wheel‐chair driving, exercising with medicine balls and dumb‐bells, and levering movements in a wheel‐chair and on parallel bars.Exercise tests were made on an ergometer bicycle for arm work; the loads were submaximal for all pupils, and also maximal for 8 in the paraplegia group. Identical submaximal tests were carried out before and after the training period, together with determination of the oxygen uptake, heart rate, blood lactate concentration and roentgenological heart volume.In the CP group, the blood lactate concentration was lower after training than before it. In the paraplegia group, significantly lower values were recorded after training for oxygen uptake, heart rate and blood lactate. No change in heart volume was detected in either group.In the 8 pupils in the paraplegia group who could perform maximal work, a 40% increase was noted after training.The better training results in the paraplegia group can be explained by the more severe handicap in the CP group, in which the arms were also involved in most cases. The heart rate during training was an average 140 beats/min in the paraplegia group, but only 115 beats/min in the CP group.The results show that these severely handicapped adolescents were able to improve their working capacity, despite training being of fairly mild intensity and carried out during a relatively short period. This implies that the ordinary gymnastic lessons were inadequate for improving the pupils' working capacity, and should therefore be complemented by a training programme of the type described. A better working capacity is highly desirable for their performance at school, as well as during their activity of daily living.It is concluded that this type of training should be included in the conventional habilitation programme for children and adolescents with motor handicaps of this type.
HEREDITARY FRUCTOSE INTOLERANCE IN FOUR SWEDISH FAMILIES
Authors: Köhlin Per , Melin Kerstin 
Published: January 2008

SummaryA brief review is given of hereditary fructose intolerance. Seven adult cases of the disease from four families, two of which are distantly related, are reported. Among their relations at least two more persons have shown symptoms of fructose intolerance.The first symptoms appeared in infancy on weaning, when sweetened formulas or fruit juice supplements were given, and consisted of retching, vomiting, and sweating.All the subjects quickly developed strong aversion to foods containing fructose. None had dental caries. All were physically and mentally normal.The characteristic hypoglycaemia and hypophosphataemia were observed in all cases in intravenous fructose tolerance tests. The plasma FFA showed an initial fall after injection of fructose, followed by a sharp rise. The initial fall probably supports the view that fructose is readily metabolized in adipose tissue in subjects with HFI. The subsequent rise may be due to lipolysis. The urinary excretion of adrenaline was examined in one case, and raised values were recorded during the fructose tolerance tests; the significance of this is discussed.
MALIGNANT PHEOCHROMOCYTOMA IN A CHILD: TREATMENT WITH A COMBINATION OF ALPHA‐ AND BETA‐ADRENERGIC BLOCKADE
Authors: Boréus Lars O. , Broberger Ulf , Nergårdh Arne , Zetterqvist Per 
Published: January 2008

SummaryA case of inoperable malignant pheochromocytoma in a 9‐year‐old girl is described. On admission to the hospital, the patient was in poor general condition with weight loss, hypertension, a considerable left heart hypertrophy and extremely high concentrations of noradrenaline in central venous blood and urine. Alpha‐adrenergic blockade with phentolamine was insufficient to improve her condition. When the beta‐adrenergic drug, propranolol, was added, a rapid reduction of blood pressure and heart size to normal values occurred with dramatic improvement of the general condition in spite of continued high urinary output of noradrenaline. It is concluded that the symptomatic treatment of pheochromocytoma may require both alpha‐ and beta‐adrenergic blocking drugs for adequate control of the clinical symptoms in this disease.
DEVELOPMENT OF NON‐RESPIRATORY COMPONENTS OF ACID‐BASE EQUILIBRIUM IN 250 NORMAL CHILDREN AGED ONE MONTH TO 15 YEARS
Authors: Vokáč Z. , Vávrová V. 
Published: January 2008

SummaryTotal plasma protein, hemoglobin, standard bicarbonate, buffer base; and base excess concentrations were determined in venous blood of 250 healthy normal children of both sexes aged one month to 15 years. In infants up to one year (100 babies in all) the mean standard bicarbonate concentration was round 21 mEq/1, buffer base 43 mEq/1, and base excess – 4 mEq/1. A slight lowering of the buffering capacity of blood due, above all, to decreased hemoglobin concentration was found in these infants.With increasing age all values come near the normal adult values but do not quite reach them even in the oldest age group of children aged 9 to 15 years. Infants especially, but also children from one to three years of age have, in comparison to adults, a marked trend towards non‐respiratory (metabolic) acidosis that, however, cannot be regarded as pathologic.
GLYCOSIDASES IN SKIN AND PLASMA IN HUNTER'S SYNDROME Abnormality of a β‐galactosidase in Skin
Authors: Öckerman P. A. , Köhlin P. 
Published: January 2008

SummaryTwo patients with Hunter's syndrome (mucopolysaccharidosis type II) were studied. The exact diagnosis could be settled by the finding of clinical symptoms and signs, typical for this form of mucopolysaccharidosis, and of a greatly increased excretion of glycosaminoglycans (acid mucopolysaccharides) in the urine, as well as by the fact that the patients were half‐brothers with unrelated fathers.Analyses of glycosidases in skin demonstrated the existence of low activity of β‐galactosidase in both patients. β‐acetylglucosaminidase was more active in both patients and β‐glucuronidase in one of them than in the controls.In plasma increased activities were noted for β‐glucuronidase and β‐acetylglucosaminidase in both patients and of β‐galactosidase and α‐mannosidase activities in one of them and of α‐fucosidase activity in the other.The results are very similar to those described in patients with Hurler's syndrome (mucopolysaccharidosis type I).
HYPOPITUITARY DWARFISM The Importance of Early Therapy
Authors: Moe Peter Johan 
Published: January 2008

SummaryTwo siblings with hypoglycemia had from early infancy retardation of growth which seemed to be due to isolated deficiency of somatotropin. The father had diabetes mellitus which may be of etiologic significance. They have been followed from birth to the ages of 6 and 7 1/2 years respectively. Experience with long‐term therapy with HGH, 2 mg three times weekly, from the age of 3 1/2 and 2 years is reported. A purified HGH preparation which is supposed to contain only the somatotropin proper, seems to be preferable. A reduction of the HGH dosage to 1 mg three times weekly resulted in a marked reduction in height increments, and almost no growth was observed in the short periods without therapy. Total growth during a 4‐year‐period was 29.5 and 32.5 cm respectively. However, the therapy was probably initiated too late and the height continues to be far below the 2.5 percentile, particularly in the older child. The importance of an adequate therapy from early infancy in the more severe cases of hypopituitary dwarfism is stressed.Attention is drawn to the hematological aspect of hypopituitary dwarfism, and the increased iron requirement during the initial period with catchup growth.
DIETARY TREATMENT OF CYSTINOSIS
Authors: Seip M. , Steen‐Johnsen J. , Vellan J. E. , Gjessing L. R. 
Published: January 2008

SummaryThe amino acid pattern in the urine and serum was investigated in two cases of cystinosis before and on treatment with a diet low in methionine and cystine. The cerebrospinal fluid and the liver were studied two months after dietary treatment was started. The urinary amino acids in both cases were increased, similarly as in the Fanconi syndrome. Some of the amino acids occurred in elevated amounts in serum. The aminoaciduria, therefore, must be extrarenal as well as renal in origin. The treatment with the methionine and cystine poor diet did not produce a great change in the concentration of the urinary and serum amino acids, but the amino acids showed a slight tendency towards normalization. The cerebrospinal fluid and the liver showed high levels of cystine.Clinically there was an obvious improvement on the dietary regimen with increased well‐being, reduction of fluid intake and diuresis as well as a fall in blood urea and an increase in specific gravity of the urine. The rate of growth has been unchanged so far.Whether all these positive findings are due to the reduced protein intake or to the reduced amounts of methionine and cystine in the diet, is difficult to decide at the present time.
IMMUNOGLOBULINS IN 13–15 TRISOMY SYNDROME DUE TO A TRANSLOCATION
Authors: Gustavson K.‐H. , Johansson S. G. O. , Wranne L. 
Published: January 2008

SummaryA case of 13–15 trisomy syndrome, due to a translocation between two chromosomes of the 13–15 group, is described. The parents' karyotypes were normal. The child showed typical features of the syndrome except for the absence of harelip, cleft palate and four‐finger lines. Despite numerous infections the immunoglobulin concentrations were very low, although not significantly lower than those found in normal, non‐infected infants of the same age. At present the boy is 27 months old. Thus, he has survived longer than most other patients with this syndrome reported in the literature.
TWO PATIENTS WITH A 46, XX, Er CHROMOSOME CONSTITUTION
Authors: Leisti J. , Gripenberg U. , Kivalo E. , Palo J. , von Schoulz B. , Suomalainen E. 
Published: January 2008

SummaryThe clinical and cytogenetic features of two girls, aged 11 and 19, are described. Both have a ring chromosome replacing one of the chromosomes no. 18.
MENINGITIS CAUSED BY NEISSERIA CATARRHALIS
Authors: Cocchi P. , Ulivelli A. 
Published: January 2008

SummaryA case is reported of meningitis due to Neisseria catarrhalis in a 14‐month‐old infant. The organism was cultured from the spinal fluid and throat. The patient recovered after penicillins, erythromycin and chloramphenicol therapy.
INTRAVENOUS INFUSION OF GLUCOSE AND SODIUM BICARBONATE IN HYALINE MEMBRANE DISEASE
Authors: SAVIGNONI P. G. , BUCCI G. , CECCAMAE A. , MENDICINI M. , ANDRE A. SCAL AM , ORZALESI M. M. 
Published: January 2008

SummaryA controlled trial of the effect of intravenous infusion of glucose and sodium bicarbonate was performed in 48 low weight newborn infants with hyaline membrane disease.The experimental design included diagnosis by clinical and radiological criteria and random assignment to the treatment and control series.In treated infants the mortality rate was lower, early death occurred less frequently and the neonatal survival curve was improved when compared with controls. Only the difference in survival curves of treated and control patients was statistically significant.Systolic blood pressure was found to be a factor of considerable prognostic significance.We conclude that intravenous infusions of glucose and sodium bicarbonate should be part of the routine therapy of infants with hyaline membrane disease.
A DOUBLE BLIND TRIAL OF PREDNISOLONE IN EPIDEMIC BRONCHIOLITIS DUE TO RESPIRATORY SYNCYTIAL VIRUS
Authors: CONNOLLY J. H. , FIELD C. M. B. , GLASGOW J. F. T. , SLATTERY CLAIRE M. , MacLYNN D. M. 
Published: January 2008

SummaryA double blind trial of prednisolone treatment was carried out on 95 children with clinical evidence of epidemic bronchiolitis. The trial showed that there was no difference between the prednisoIone and the placebo group in the duration of symptoms and signs.Eighty‐four per cent of children were shown to be infected with respiratory syncytial virus. Prednisolone in the dosage used did not affect the antibody response to infection.The presence of maternal antibody to respiratory syncytial virus, while not preventing infection, reduced the number of active antibody responses in children aged 3 months or younger.Eight children had a double infection with respiratory syncytial virus and adenovirus which lengthened their average stay in hospital by two days and the overall duration of illness by more than three days.
HEMORRHAGIC MUCOSAL NECROSIS OF THE GASTROINTESTINAL TRACT IN THE NEWBORN
Authors: SØRENSEN F. HANBERG , VETNER M. 
Published: January 2008

SummaryTwo cases of hemorrhagic mucosal necrosis in the gastrointestinal tract without vascular occlusion are reported. This combination does not seem to have been previously described. Both cases were observed in newborn infants and in both cases the condition was combined with intestinal perforation. Both infants died. Possible etiologic factors are discussed and the histological criteria reviewed.
ACUTE HEMIPLEGIA IN GLYCOGEN STORAGE DISEASE, TYPE 1
Authors: FINE RICHARD N. , PODOSIN ROBERT , DONNELL GEORGE N. 
Published: January 2008

SummaryA 21‐month‐old girl with GSD, Type 1 developed acute hemiplegia with homonymous hemianopsia following a prolonged hypoglycemic seizure. There was gradual improvement in motor function. However, the patient had subsequent episodes of transient blindness with and without seizure activity which were unrelated to hypoglycemia. Serial EEG findings suggested that the hemiplegia resulted from a vascular accident and led to neural dysfunction causing subsequent transient blindness.
Growth and Serun Chemical Values of Normal Breastfed Infants
Authors: J. Fomon Samuel , Filer L. J. , Thomas Lora N. , R. N.  , Ronald  , Rogers R. 
Published: January 2008

SummaryOne‐hundred‐and‐forty‐nine normal fullterm infants were enrolled during the first 9 days after birth in a study of breastfed infants; 104 (70%) completed the planned 112 days of observation. Lengths and weights at 8, 14, 28, 42, 56, 84 and 112 days of age are presented for each infant. Rates of gain in length and weight between various ages are summarized and some patterns of growth of individual infants are discussed. Serum chemical data at 28, 42, 56, 84 and 112 days of age are presented. Statistically significant sex‐related differences in serum concentrations of cholesterol and triglycerides (greater in females than in males) and alkaline phosphatase (greater in males than in females) are demonstrated.It is suggested that these data on performance of normal infants breastfed by healthy mothers may be useful for evaluating the performance of other breastfed infants and of infants fed commercially available or experimental formulas.
School children with cerebral palsy at Bräcke Östergard Pediatric Habilitation Centre
Authors: OLOW INGEMAR , BERG KRISTINA 
Published: January 2008

SummaryBräcke Östergård is the regional centre för pediatric habilitation in western Sweden. All children with cerebral palsy in the region, except för the Göteborg and S province children, are registered at the Centre. The indications för full‐board admission to the Centre are guided by uniförm principles with due consideration to the need of each subject.The internationally accepted definition of cerebral palsy is used. The classification of the subgroups of cerebral palsy is Swedish and is based on the state of the muscular tone, on the type of associated movements and on the anatomical distribution of the symptoms.