bluuebunny/crossref_metadata_2025_split huggingface dataset:
COARCTATION OF THE AORTA WITH MULTIPLE ARTERY ANOMALIES IN IDIOPATHIC HYPERCALCEMIA OF INFANCY
Authors: BLIDDAL J. , DUPONT B. , MELCHIOR J. C. , OTTESEN O. E. 
Published: January 2008

SummaryA case of idiopathic hypercalcemia of infancy with multiple anomalies of the arteries is described. This patient demonstrated a combination of supravalvular pulmonary artery stenosis, supravalvular aortic stenosis, hypoplasia of the aortic arch, coarctation of the aorta and multiple stenoses of the branches of the abdominal aorta. The pathogenesis of arterial hypertension in this syndrome is discussed.
Dynamic and static lung volumes of school children with cerebral palsy
Authors: BJURE JAN , BERG KRISTINA 
Published: January 2008

SummaryThe dynamic and static lung volumes were measured in 22 subjects with cerebral palsy. There were no signs of airway obstruction. The total lung capacity was significantly reduced, averaging 85 per cent of the predicted normal values. In the 14 subjects with spasticity, the functional residual capacity was significantly reduced to 75 per cent of the predicted value, whereas the eight subjects with dyskinesia showed a marked and significant increase in the residual volume (156 per cent of the predicted value). This difference in the distribution of the lung volumes resulted in a 50 per cent decrease in the vital capacity för subjects with dyskinesia compared with a 67 per cent decrease för subjects with spasticity. These deviations in lung volumes from the predicted normal values and the differences between the two groups can be explained by the reduced ability of voluntary muscular control.
Somatic adaptation in cerebral palsy ‐ summary and general discussion
Authors: BERG KRISTINA 
Published: January 2008

SummaryFourty‐one school children with severe affliction of cerebral palsy participated in one or more studies on adaptory mechanisms in cerebral palsy.The studies dealt with the physical working capacity, lung volumes, body composition, nutrition, and spontaneous activity. Effects of therapy with physical training and improved nutrition were also studied.Data from the different studies were integrated and gave the following results.Thea abnormal body composition during growth directly affects body weight (BW). rörelsehinder this reason, body cell mass (BCM), representing the active metabolic mass, is suggested as the reference value rörelsehinder subjects with cerebral palsy, with respect to metabolic data, including gross body composition.The mean physical working capacity berörelsehindere training was 71 per cent of that of healthy children per kg BCM. The main limits to maximal work were considered muscular. The severity of the handicap appeared to be the factor most closely related to the original low working capacity of the factors studied.The physical working capacity significantly increased to a mean of 90 per cent of normal values.The total lung capacity was reduced when compared to the predictions för height (BH), but adequate when correlated to BCM. The subjects with dyskinetic cerebral palsy had an abnormal volume distribution of air with increased residual volume.Malnutritive changes were found in the body composition with a decrease of BCM to 85 per cent of the prediction för BH and with an increase of extracellular water (ECW) from a predicted mean of 0.56 per kg BCM to 1.06. In addition 7 of 23 subjects had a BH below the 2.5 per cent population limit för age. The physical training alone, and in combination with an improved nutrition, significantly reduced ECW, but did not effect BCM. BF was significantly increased by training only. Improved nutrition alone had no significant effects, which indicated that the malnutrition was not severe. The increased extracellular mass (ECW+ BF) may cause a metabolic load on the cells and abnormal conditions för transport.The spontaneous physical activity correlated well with total body potassium (Ke, BCM). Factors which may modify the habitual activity such as age and BF were studied.The difference in neuromotor disturbance between the spastic and the dyskinetic group caused minor differences, concerning volume distribution of air in the lungs, voluntary control of movements after exhaustion and body fat amounts.The severity of handicap was found to be of importance in causing unspecific somatic complications. The motor disability restrains the physical activity and creates subjects with a low energy need. The consequences of this can be softened by a proper management, which provides a variety of physical activities and a diet with a high content of essential nutrients per unit energy.
ELECTROENCEPHALOGRAPHIC SECTION: Introduction and literature
Authors: BLOM S. , RUNE V. 
Published: January 2008

Summary1. No differences were found with respect to the presence of normal and/or pathological EEG patterns between a head injury material cared for in hospital, a head injury material cared for at home and an unselected control material.2. The limited size of the materials does not permit a reliable detailed ana‐lysis of possible differences between the groups with respect to the types of abnormalities that were found.3. From the clinical point of view, the EEG should be evaluated with great caution after head injury in children (following the acute phase), especially if it is not possible to compare the find‐ings with the EEG pattern before head injury.
INTESTINAL DIPEPTIDASES AND DISACCHARIDASES IN CHILDREN WITH MALABSORPTION
Authors: DAHLQVIST A. , LINDBERG T. , MEEUWISSE G. , AKERMAN M. 
Published: January 2008

SUMMARYIntestinal enzyme activities (dipeptidases and disaccharidases) and mucosal morphology were studied in two groups of paediatric patients: one coeliac group (n=13) and one “suspected cow's milk intolerance” group (n= 5). The patients were studied before and after dietary treatment. 49 children and adults with histologically normal intestinal mucosa served as a control group.Both groups of patients showed a marked secondary decrease in the dipeptidase and the disaccharidase activities. The decrease was more pronounced for the disaccharidase than for the dipeptidase activities, and was reversible on dietary treatment. Among the disaccharidases, the lactase and trehalase activities were most strongly depressed; in the coeliac group, the ala‐pro dipeptidase activity was more strongly affected than the other dipeptidases. However, as also the ala‐pro dipeptidase activity increased on dietary treatment, this does not provide any clue to the specificity of a hypothetical enzyme whose absence could be responsible for the disease.
RESPIRATORY QUOTIENT AND METABOLIC RATE IN NORMAL FULL‐TERM AND SMALL‐FOR‐DATE NEWBORN INFANTS1
Authors: SENTERRE J. , KARLBERG P. 
Published: January 2008

SUMMARYRespiratory quotient and gaseous metabolism were measured in normal full‐term and small‐for‐date infants during the first week of life. In both groups RQ dropped during the first day and rose again during the next few days. However, the RQ was lower during the first 5 days in small‐for‐date infants. The O2 uptake of the small‐for‐date babies is initially lower but rises during the first 5 days.Technical problems and clinical implications of the results are discussed.
THREE CASES OF DISSEMINATED INTRAVASCULAR COAGULATION
Authors: BERGLUND GUNILLA 
Published: January 2008

SUMMARYDisseminated intravascular coagulation (DIC) was a complication in 3 patients with different primary diseases. The probable initiation of the DIC was a thromboplastic active tumor, a laceration wound at cesarean section with amniotic embolism in the foetus and probable endotoxin release in a newly kidney biopsied patient with subacute glomerulonephritis. The DIC was interrupted in the first patient by extirpation of the tumor, in the other two by treatment with heparin in spite of bleeding tendency.The diagnosis can be strongly suspected by demonstrating a decrease of the fibrinogen content combined with a diminished number of platelets. A normalization of the condition can occur after interruption of the intravascular clotting by treatment with heparin.
MILD AND SEVERE PKU. COMPARATIVE STUDIES IN TWO INFANTS
Authors: HJALMARSSON O. , JAGENBURG R. , RODJER S. 
Published: January 2008

SummaryTwo infants with hyperphenylalaninemia first recognized at the age of 5 days were studied. One of the patients was a case of classical severe PKU. The other patient, on an unrestricted diet, showed only a slow rise in the plasma phenylalanine level amounting to 16 mg/100 ml at 3 weeks of age and had no phenylpyruvic aciduria. The plasma phenylalanine levels of the two infants were determined during fasting, after feeding and after intravenous phenylalanine loading. The results obtained did not reveal any significant difference in the phenylalanine turn over between the two infants.
SEPARATION BY GEL FILTRATION AND MICRODETERMINATION OF UNBOUND BILIRUBIN
Authors: ZAMET P. , CHUNGA F. 
Published: January 2008

SummaryExtraction of unbound bilirubin by Sephadex gel filtration and measurement by a micro‐method were carried out in 45 sera from jaundiced newborns (hemolytic disease or prematures). In about half the cases some free bilirubin with values in the range 0.1 to 1 mg/100 ml was detected. Levels above 0.5 mg/100 ml were exceptional (only 2 cases). Five autopsies were performed in prematures with unbound bilirubin levels above 0.1 mg/100 ml, and one kernicterus was found. Two infants with unbound bilirubin levels of more than 0.5 mg / 100 ml had clinical symptoms of kernicterus. No symptoms of kernicterus were observed in infants with high total bilirubin together with an insignificant amount of unbound bilirubin.The main interest of this method lies in the direct assessment of a toxic element originating from varying bilirubin and albumin levels and the micro‐environment of the bilirubin‐al‐bumin binding.But other factors, such as the cell membrane permeability and/or the duration of exposure, are likely to play a prominent role.
RECONSTRUCTION OF OESOPHAGUS WITH COLONIC TRANSPOSITION
Authors: GROTTE G. , OLSEN L. , REUTERSKIOLD A. 
Published: January 2008

SummaryWith the increased experience which has been gained during recent years, transposition of the colon can be regarded as a valuable method when for various reasons it is desirable to replace the whole or parts of the oesophagus.A series of 6 patients operated on by the retrosternal technique with good results is reported.On the basis of this series the advantages and disadvantages of this method are discussed and compared with other types of replacement of the oesophagus by colonic transposition.
LYMPHOBLASTIC TRANSFORMATION, CHROMOSOME PATTERN AND DELAYED‐TYPE SKIN REACTION IN ATAXIA TELANGIECTASIA
Authors: SCHULER D. , SCHöNGUT L. , CSERHATI E. , SIEGLER J. , GÁCS G. 
Published: January 2008

SummaryIn 6 patients suffering from ataxia telangiectasia morphological evaluation of lymphoblastic transformation was performed; in one of these cases it was repeated four times. In 3 cases the incorporation of 3H‐thymidine was measured to examine the degree of the lymphoblastic transformation. Both methods revealed decreased lymphoblastic transformation, though with the last method the decrease was much greater. No correlation was found between the degree of decreased blastic transformation, the infections and the plasma IgA level. Delayed‐type skin reactions were subnormal in varying degrees. Increased frequency of chromosome breakage was observed in 1 case–who developed malignancy and died– but no chromosomal aberrations were found in the three other cytogenetically examined patients.
THE BEHAVIOUR OF SOME INDICES OF CALCIUM‐PHOSPHATE METABOLISM IN TURNER'S SYNDROME
Authors: SZCZEPSKI O. , WALCZAK M. , MACIEJEWSKI J. , BITTNER K. , CZEKALSKI S. , WALIGORA A. 
Published: January 2008

SummaryThe results of calcium and parathormone loading tests in 6 individuals with Turner's syndrome indicate a certain degree of insensitive‐ness of the tissues, especially of the kidney, when compared with healthy individuals. A similar insensitiveness expressing itself by a decrease in hydrogen ion excretion was found after ammonium chloride loading. An analysis of the results suggest the existence in this syndrome of insensitiveness of renal tubules to regulatory factors. Osteoporosis and stunting of growth may be connected partly with intestinal calcium loss occurring at puberty, and partly with this insensitiveness to regulatory factors.
MUCOPOLYSACCHARIDOSIS IN A THREE MONTHS OLD INFANT Clinical and Ultrastructural Studies
Authors: TONDEUR M. , VAMOS‐HURWITZ E. , CREMER N. , LOEB H. 
Published: January 2008

SummaryA mucopolysaccharidosis is diagnosed in an infant aged 3 months. Clinical and X‐ray data are not typical, while biological studies reveal an increased mucopolysacchariduria affecting both heparan and dermatan sulfate. Study of lysosomal hydrolases in the liver discloses a normal activity of β‐galactosidase. Hepatic ultrastructure shows, in both parenchymal and Kupffer cells, the presence of clear inclusions analoguous to those found in typical mucopolysaccharidoses, although more numerous and generally smaller.
THE OCULOCEREBRORENAL SYNDROME OF LOWE IN FOUR GENERATIONS OF ONE FAMILY
Authors: PALLISGAARD G. , GOLDSCHMIDT E. 
Published: January 2008

SummaryA Danish family with five proven and three additional possible cases of the oculo‐cerebro‐renal syndrome is described. All proven carriers had cataract, but the lenticular opacities varied considerably with respect to magnitude, appearance, and location. In this family, the disease was transmitted as a sex‐linked recessive trait, the complete syndrome being seen in boys only.
TEST‐MEAL IN THE DIAGNOSIS OF MALABSORPTION IN INFANCY
Authors: NORMAN ARNE , STRANDVIK BIRGITTA , ZETTERSTRÖM ROLF 
Published: January 2008

SummaryA test‐meal containing 2 g of glucose per kg body weight (minimum of 20 g), 0.5 g of D‐xylose per kg, 8 ml of standardized cream (12% fat) per kg and 7 500 IU of vitamin A palmitate per kg was administered through a nasogastric tube. During the next 10 hours capillary blood samples were obtained and analysed for glucose, D‐xylose, triglycerides, and vitamin A using microanalytical methods.The results in 16 normal infants aged 1 to 20 months are reported. The results for all parameters determined, except D‐xylose, were comparable to those previously reported using single tolerance tests. The variability related to age was pronounced for glucose, triglycerides and vitamin A.In patients with malabsorption syndromes (cystic fibrosis, extrahepatic biliary atresia, intrahepatic cholestasis, and gluten‐induced enteropathy) the results were also comparable with those earlier observed using single tolerance tests.A combined test‐meal has certain advantages over the examinations now used in infants with symptoms suggesting a malabsorption syndrome: a series of tolerance tests is avoided, and it is therefore easier to follow the absorption of fat and carbohydrates during the course of a disease and during a therapeutic regimen.
STABILITY OF ACID PHOSPHATASE OF FETAL RED BLOOD CELLS DURING INCUBATION WITH ACETYLPHENYLHYDRAZINE
Authors: SPENNATI G. F. , ORZALESI M. , BOTTINI E. , PIGRAM P. 
Published: January 2008

SummaryIncubation of red blood cells (RBC) in vitro with acetylphenylhydrazine and without glucose produced an inactivation of acid phosphatase which was more marked in the fetal RBC than in the RBC from maternal blood. The fall in reduced glutathione (GSH) during incubation was more rapid in fetal than in maternal RBC. In each instance the fall in GSH preceded that in acid phosphatase and the two phenomena were very well correlated. The faster inactivation of acid phosphatase in the fetal RBC could be due to the higher total concentration of GSH and to its decreased stability in fetal RBC.The stability of acid phosphatase seemed to increase in red blood cells from infants 3 days old. This finding could indicate a certain degree of heterogeneity in the fetal RBC population at birth and a rapid selection in favor of those RBC which are more fit for the new biochemical environment after birth.
NIEMANN‐PICK DISEASE AND GIANT CELL TRANSFORMATION OF THE LIVER
Authors: ASHKENAZI A. , YAROM R. , GUTMAN A. , ABRAHAMOV A. , RUSSELL A. 
Published: January 2008

SummaryThis report presents three siblings with neonatal jaundice who died before the age of three months. They were shown on autopsy to be suffering from Niemann‐Pick disease together with a giant cell transformation of the liver.The resemblance of these cases to previously reported examples of the above mentioned double condition is stressed, and a suggestion is made that this variant of the Niemann‐Pick disease presents a special genotypic syndrome within the group with the biochemical disorder broadly classified as sphingomyelin lipidosis.
CARDIOMEGALY IN ASSOCIATION WITH NEONATAL HYPOGLYCAEMIA
Authors: REID M. McC. , REILLY B. J. , MURDOCK A. I. , SWYER P. R. 
Published: January 2008

SummaryFifty‐two infants with hypoglycaemia were seen over a 12 month period. Six of these infants had cardiac enlargement with signs of failure and pulmonary oedema in association with isolated hypoglycaemia. Signs and symptoms suggestive of cardiac failure corresponded to the period of hypoglycaemia and disappeared when blood sugar levels were raised by appropriate treatment.
CYSTATHIONINURIA IN A WELL BABY POPULATION
Authors: LYON I. C. T. , PROCOPIS P. G. , TURNER B. 
Published: January 2008

SummaryScreening of 35 809 infants by one way chromatography revealed 15 cases of excessive cystathionine excretion. Evidence is presented to suggest that two of these are homozygous for primary cystathioninaemia. Homocystin‐uria was present transiently in one of these two infants. It is concluded that cystathionin‐uria is a benign disorder and appears to be much more common than previously recognised.
THE THIRD FONTANELLE
Authors: TAN K. L. 
Published: January 2008

SummaryOne thousand, nine hundred and thirty apparently normal newborn infants were examined for the presence of the third fontanelle. In addition other newborn infants with abnormalities were also screened for the same defect.The incidence of the third fontanelle was high, in this study, among the normals, compared with past studies. The incidence seemed highest in the group weighing 2 001 to 2 500 g.In the abnormal group, the incidence was very significantly raised in infants suffering from the congenital rubella syndrome or Down's syndrome; it was raised but not significantly in infants presenting with various other abnormalities.The presence of the third fontanelle should alert the examiner to the possibility of other defects in the infant.
BAY B 5097, A NEW ORALLY APPLICABLE ANTIFUNGAL SUBSTANCE WITH BROADSPECTRUM ACTIVITY
Authors: MARGET WALTER , ADAM DIETER 
Published: January 2008

SummaryBAY b 5097, bis‐phenyl‐(2‐chlorophenyl)‐l‐imidazolylmethane, is a fungistatically acting new antimycotic, which was used in 10 children in a dosage of 70–200 mg/kg/day, usually in two doses. Six children suffered from a Candida albicans pyelonephritis, 2 from Candida septicemia, 1 from atopic dermatitis with candidiasis and 1 from congenital toxoplasmosis. All children responded to the therapy. The possible successful treatment of a hopeless case of toxoplasmosis leads to the assumption that there might be an effect on protozoa.
INFLUENCE OF ENVIRONMENTAL TEMPERATURE AND ACIDOSIS ON LIPID MOBILIZATION IN THE HUMAN INFANT DURING THE FIRST TWO HOURS AFTER BIRTH
Authors: PERSSON BENGT , TUNELL RAGNAR 
Published: January 2008

Arterial concentrations of glycerol, FFA, glucose, lactate and β‐hydroxybutyrate were serially measured during the first two hours after birth in normal fullterm infants in a thermo‐controlled environment. Blood gas tensions, acid‐base balance, pulmonary gas exchange, motor activity and heart rate were also determined: a detailed report of these data will be published separately. In 22 infants the glycerol concentrations showed a rapid immediate increase after birth wheras the rises in FFA concentrations were delayed until between 30 and 120 minutes, indicating a prompt increase in lipolysis and a suppression of lipid mobilization during the first half hour after birth. This suppression might be explained by a high rate of reesterification or oxidation of FFA within adipose tissue. The influence of environmental temperature (2 8.7‐3 4.8 oC) and degree of acidosis on the pattern of changes in FFA and glycerol were only marginal. No inhibition of lipolysis and lipid mobilization was shown in an infant who developed postnatal asphyxia.At 120 min after birth, when acidosis had been eliminated, an inverse correlation was found between the rise in FFA from birth to 120 min and the respiratory exchange ratio (V̇co2/V̇o2).The glucose concentrations were related neither to the FFA nor to the glycerol concentrations. The rate of elimination of lactate and β‐hydroxybutyrate was not influenced by environmental temperature or acidosis. Minute amounts of administered heparin caused an increased rise in FFA and glycerol concentrations which were associated with the appearance of lipoprotein lipase activity.
A VARIANT FORM OF BRANCHED‐CHAIN KETO ACIDURIA
Authors: HORST J. L. VAN DER , WADMAN S. K. 
Published: January 2008

SummaryA case of branched‐chain keto aciduria is described. The boy's clinical picture was similar to that of patients with the intermittent form of this disorder, but severe mental retardation was present.It proved possible to treat him successfully with a diet low in branched‐chain amino acids and, at the age of 3 years, after 18 months' treatment, his mental development corresponded with his age.Details are given of the clinical course and the dietary treatment. The findings in the patient's family are also described, with the results of oral loading tests with 1‐leucine performed on all members of the family.
METABOLIC ALKALOSIS IN INFANTS: ROLE OF WATER DEPLETION AND CHANGES IN COMPOSITION OF STOOL
Authors: KILDEBERG POUL , ENGEL KNUD 
Published: January 2008

SummarySimple calculations of the effect of volume depletion on the concentration of titratable base of blood show this effect to be largely adequate to account for commonly observed degrees of metabolic alkalosis in patients with mild or extreme dehydration due to obstructive vomiting. This suggests that hydrochloric acid depletion per se may not be a major determinant of the blood acid‐base status in such conditions. Clearly, part of the base equivalents missing in the extracellular fluid may be sought in the cell water; but currently available evidence concerning gastrointestinal transport of acid and base invites a conception of gastrointestinal acid‐base homeostasis–and the hypothesis that in obstructive vomiting changes in the composition of stool may appreciably modify the effect of gastric hydrochloric acid depletion. In studies of intracellular buffering and net base balance in gastric alkalosis, it must be realized that the primary extracellular disturbance may be largely contractional and that stool losses (or luminal pooling) of base may be an important variable.
CONGENITAL LEUKAEMIA
Authors: HAAHR J. , HALVEG A. B. 
Published: January 2008

SummaryA report is submitted of a case of leukaemia of myeloid type in a newborn boy who died on the 7th day of life, despite anti‐leukaemic therapy. The available reports on congenital leukaemia and on children born of mothers with leukaemia in pregnancy do not give any clue to the aetiology.